Encyclopedia of Clinical Neuropsychology

Encyclopedia of Clinical Neuropsychology Jeffrey S. Kreutzer John DeLuca Bruce Caplan Editors Encyclopedia of Clinic...

Author: Jeffrey Kreutzer | John DeLuca | Bruce Caplan

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Encyclopedia of Clinical Neuropsychology

Jeffrey S. Kreutzer John DeLuca Bruce Caplan Editors

Encyclopedia of Clinical Neuropsychology With 199 Figures and 139 Tables

Editors Jeffrey S. Kreutzer, PhD, ABPP, FACRM Rosa Schwarz Cifu Professor of Physical Medicine and Rehabilitation, and Professor of Neurosurgery, and Psychiatry Virginia Commonwealth University – Medical Center Department of Physical Medicine and Rehabilitation VCU P.O. Box 980542 Richmond, Virginia 23298-0542 USA [email protected]

Bruce Caplan, PhD, ABPP Independent Practice 564 M.O.B. East, 100 E. Lancaster Ave. Wynnewood, PA 19096 USA [email protected]

John DeLuca, PhD, ABPP Vice President of Research Kessler Foundation Research Center 1199 Pleasant Valley Way West Orange, NJ 07052 USA and Professor of Physical Medicine and Rehabilitation, and Neurology and Neuroscience University of Medicine and Dentistry of New Jersey – New Jersey Medical School [email protected]

ISBN 978-0-387-79947-6

e-ISBN 978-0-387-79948-3

Print and electronic bundle ISBN 978-0-387-79949-0 DOI 10.1007/978-0-387-79948-3 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2010933970 © Springer ScienceþBusiness Media, LLC 2011 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer ScienceþBusiness Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Product liability: The publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature. Printed on acid-free paper Springer is part of Springer ScienceþBusiness Media (www.springer.com)

We dedicate the Encyclopedia of Clinical Neuropsychology to our teachers and mentors, the people who taught, supported, and inspired us to choose and follow careers in the field of clinical neuropsychology. David Michael Scott, in graduate school, first helped me appreciate the importance of learning about the brain and nervous system. Alexander Manning taught me about the brain and standardized neuropsychological assessment. Donald Kausch taught me how to administer and interpret the Halstead Reitan and tests designed by Arthur Benton. My internship supervisors, Muriel Lezak, Larry Binder, Diane Howieson, Richard Erickson, Orin Bolstad, David Shaw, and Julian Taplin taught me so much about neuropsychology, how to work with families, and inspired me on to a career in the field. Jeffrey T. Barth, Ronald Ruff, and Harvey Levin, colleagues I worked with on the Traumatic Coma Data Bank project, helped me learn neuropsychological research methods, brain injury, and how to be patient and tenacious. Mitchell Rosenthal, Paul Wehman, and Henry Stonnington taught me about rehabilitation, teamwork, hope, and how to be practical. JSK As an undergraduate, Martin Hahn lit the fire within me regarding an interest in science, which launched my pursuit for advanced education in brain-behavior relations. Dick Burright taught me about critical thinking in science, through a lot of hard work. Peter Donovick guided me through graduate school, even when the path ahead seemed unclear, and was the primary reason I discovered my ultimate career path in human neuropsychology. Keith Cicerone rounded out my skills and provided me with the finishing touches in my education and training and helped me carve out my particular niche in neuropsychology, which included both research and clinical activities. Joel DeLisa provided me with the fertile environment I needed to launch my career in clinical neuropsychology, particularly by supporting a research environment based on my own interests, approach, and skills, allowing me to pursue a career first dreamed as an undergraduate. Lastly and perhaps most importantly, I dedicate this work to all of my post-doctoral and pre-doctoral trainees, too numerous to list, who have challenged me professionally and personally, and by far have had the most influence on the success in my career. I hope that I have had but a fraction of an influence on theirs. JDL To: Marcel Kinsbourne, who gets the credit (and blame) for awakening my interest in neuroscience in general and neuropsychology in particular and exposing me to world-class intellects; Leonard Diller, from whom I learned the joy of immersion in – and struggle to master – both the historical and contemporary neuropsychology and rehabilitation literature; Charles Gibson, who gave me (in retrospect, perhaps unwisely) an inordinate amount of professional freedom in my first real job; Mitchell Rosenthal, who embodied the lesson I learned from my father, Jerome Caplan, (‘‘Be kind, because everyone you meet is fighting a hard battle’’) and encouraged me to do the same; and listed last, but most important, my multifaceted partner, Judy Shechter, my ‘‘intellectual boomerang’’ colleague and constant source of entertainment. BC

Acknowledgement The conceptualization, compilation, and production of the Encyclopedia of Clinical Neuropsychology spanned more than four years. We set out to develop a uniquely comprehensive, authoritative, indispensable reference work, and we are hopeful that our goal has been achieved. We owe an immeasurable debt to the many people who supported us through the course of the project. Foremost, we are grateful to our families for their enthusiastic support, encouragement, and patience. We are indebted to our cadre of esteemed Associate Editors for helping to develop their sections, recruit contributors, and ensure the presence of consistently high quality entries. We express our appreciation to the brilliant group of authors whose efforts form the core of our project. We are immensely indebted to the superb Springer major reference works team including Janice Stern, Anil Chandy, Lydia Mueller, and Oona Schmid who taught us, encouraged us, kept us organized and on track, and helped us every step of the way. We are also grateful to our students, patients, and their families from whom we learned much about facing challenges and the value of being practical. Jeffrey S. Kreutzer John DeLuca Bruce Caplan

Preface It is doubtful that there is a more rapidly evolving psychological specialty than clinical neuropsychology. Every day, clinicians are challenged to help patients with a widening variety of cognition-compromising disorders including traumatic brain injury, vascular conditions, brain tumors, developmental disabilities, psychiatric disturbances, and neurodegenerative disorders. Some practitioners serve pediatric populations, others treat the elderly, and many serve general adult populations. Some patients have progressive disorders, while others can achieve substantial improvement over time. Assessment is typically the starting point, with clinicians addressing a myriad of referral questions, which may relate to the patient’s ability to work, return to school, manage personal affairs, drive, live independently, or be considered eligible for disability benefits. Increasingly, clinicians are involved in civil and forensic proceedings, contributing to decisions about responsibility, competence, and entitlement to damages for injury. In fulfilling its clinical mandates, clinical neuropsychology relies strongly on its research base. As a hybrid of cognitive psychology, neuroscience and clinical psychology, clinical neuropsychology investigations are at the forefront of translational research in brain-behavior relations. The future of both clinical practice and research lies with our trainees at all levels — undergraduate, doctoral and post-doctoral. Easily accessible and frequently updated knowledge in clinical neuropsychology provides the foundation for the education and training of our future clinical neuropsychologists. A fundamental aim of this work has been to provide such a resource and, with the online version, to permit revision and expansion as the field evolves. Most neuropsychological reference books focus primarily on assessment, diagnosis, functional neuroanatomy, and descriptions of various disease entities and their higher cortical consequences. To date, none has been encyclopedic in format. We see it as a mark of the maturity of the field that such a multi-volume publication is now warranted. Clinicians, patients, family members, researchers and students all recognize that evaluation and diagnosis is only a starting point for the treatment and restoration process. Few would be satisfied with an end-product consisting only of a diagnosis and/or description of the patient’s cognitive topography. During the past decade, treatment services have proliferated, and neuropsychologists have been in the forefront of these developments because of their special training and experience. Neuropsychological clinicians now provide a variety of services in addition to assessment including psychological counseling, neurobehavioral management, cognitive rehabilitation, family intervention, and vocational rehabilitation in hospitals and community-based settings. In view of this expanded scope of contemporary practice, we envisioned an encyclopedia containing information pertinent to these activities. This encyclopedia will serve as a unified, comprehensive reference for professionals involved in the diagnosis, evaluation, and rehabilitation of children and adults with neuropsychological disorders. It will also provide students and scientists with the breadth of knowledge needed to build a scientific basis for interventions and treatment for patients. We hope Encyclopedia of Clinical Neuropsychology is the first place readers turn for factual, relevant, and comprehensive information to aid in delivering the highest quality services. September 2010 Jeffrey S. Kreutzer John DeLuca Bruce Caplan

Editors Jeffrey S. Kreutzer, PhD, ABPP, FACRM Rosa Schwarz Cifu Professor of Physical Medicine and Rehabilitation, and Professor of Neurosurgery, and Psychiatry Virginia Commonwealth University – Medical Center Department of Physical Medicine and Rehabilitation VCU P.O. Box 980542 Richmond, Virginia 23298-0542 USA [email protected] John DeLuca, PhD, ABPP Vice President of Research Kessler Foundation Research Center 1199 Pleasant Valley Way West Orange, NJ 07052 USA and Professor of Physical Medicine and Rehabilitation, and Neurology and Neuroscience University of Medicine and Dentistry of New Jersey – New Jersey Medical School [email protected] Bruce Caplan, PhD, ABPP Independent Practice 564 M.O.B. East, 100 E. Lancaster Ave. Wynnewood, PA 19096 USA [email protected]

Associate Editors Cristy Akins Mercy Family Center 110 Vetrans Memorial Blvd Metarie, LA 70005 USA [email protected] Carol L. Armstrong The Children’s Hospital of Philadelphia Neuro-Oncology/Neuropsychology 3535 Market Street, Ste. 1409-1410 Philadelphia, Pennsylvania USA [email protected] Shane S. Bush Long Island Neuropsychology, P.C. 290 Hawkins Avenue, Suite B Lake Ronkonkoma, NY 11779 USA [email protected] Tamara Bushnik Rusk Institute for Rehabilitation Medicine NYU Langone Medical Center 400 East 34th Street, RR115A New York, NY 10016 USA [email protected] Gordon Chelune Center of Alzheimer’s Care, Imaging and Research University of Utah 650 Komas Dr., Ste 106A Salt Lake City, UT 84108 USA [email protected] Nancy D. Chiaravalloti Department of Physical Medicine and Rehabilitation UMDNJ-New Jersey Medical School 1199 Pleasant Valley Way West Orange, NJ 7052 USA [email protected]

Ronald A. Cohen Department of Psychiatry and Human Behavior The Miriam Hospital Brown University 164 Summit Ave Providence, RI 2906 USA [email protected] John C. Courtney Department of Psychology Children’s Hospital of New Orleans 200 Henry Clay Avenue New Orleans, LA 70118 USA [email protected] Rik Carl D’Amato University of Macau Santa Clara Valley Medical Center Faculty of Social Sciences and Humanities 229 Tai Fung Building Taipa, Macau SAR China [email protected] Roberta DePompei University of Akron Department of Speech Language, Pathology and Audiology Akron, OH 44325-3001 USA [email protected] Janet E. Farmer University of Missouri-Columbia Thompson Center for Autism and Neurodevelopmental Disorders 300 Portland Street, Suite 110 Columbia, MO 65211 USA [email protected]

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Associate Editors

Robert G. Frank College of Public Health Kent State University P. O. Box 5190 Kent, OH 44242-0001 USA [email protected] Michael Franzen Allegheny Neuropsychiatric Institute Allegheny General Hospital 4 Allegheny Center Pittsburgh, PA 15212 USA [email protected] Robert L. Heilbronner Chicago Neuropsychology Group 333 N. Michigan Avenue, #1801 Chicago, IL 60601 USA [email protected] [email protected] Susan K. Johnson Department of Psychology University of North Carolina At Charlotte 9201 University City Blvd. Charlotte, NC 28223-0001 USA [email protected] Douglas I. Katz Boston University School of Medicine Braintree Rehabilitation Hospital 250 Pond Street Braintree, MA 2184 USA [email protected] Stephanie A. Kolakowsky-Hayner Director, Rehabilitation Research Santa Clara Valley Medical Center Rehabilitation Research Center 751 South Bascom Ave. San Jose, CA 95128 USA [email protected]

James F. Malec Rehabilitation Hospital of Indiana 4141 Shore Drive Indianapolis, IN 46254 USA [email protected]

Paul Malloy The Warren Alpert Medical School of Brown University Butler Hospital 345 Blackstone Blvd. Providence, RI 2906 USA [email protected]

John E. Mendoza SE LA Veterans Healthcare System Department of Psychiatry and Neurology Tulane University Medical Center 3928 S. Inwood Ave. New Orleans, LA 70131 USA [email protected]

Randall E. Merchant Virginia Commonwealth University Medical Center Box 980709 MCV Station Richmond, VA 23298-0709 USA [email protected]

Sarah A. Raskin Department of Psychology and Neuroscience Program Trinity College Hartford, CT 6106 USA [email protected]

Stephanie Reid-Arndt School of Health Professions - Health Psychology Ellis Fischel Cancer Center University of Missouri-Columbia Columbia, MO 65211 USA [email protected]

Associate Editors

Elliot J. Roth Feinberg School of Medicine Physical Medicine and Rehabilitation Northwestern University 345 E. Superior Chicago, IL 60611 USA [email protected] [email protected] Bruce Rybarczyk Department of Psychology Virginia Commonwealth University Box 842018 Richmond, VA 23284-2018 USA [email protected] Anthony Y. Stringer Department of Rehabilitation Medicine Emory University 1441 Clifton Road NE Atlanta, GA 30322 USA [email protected]

Lyn Turkstra University of Wisconsin, Madison 7225 Medical Sciences Center 1300 University Avenue Madison, WI 53706-1532 USA [email protected] Nathan D. Zasler Concussion Care Centre of Virginia, Ltd. 3721 Westerre Parkway, Suite B Richmond, VA 23233 USA [email protected]

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List of Contributors GALYA ABDRAKHMANOVA Department of Pharmacology Virginia Commonwealth University 1112 E. Clay Street, P.O. Box 980524 Richmond, VA 23298-0565 USA [email protected] THOMAS M. ACHENBACH University of Vermont 2 Colchester Ave. Burlington, VT 05405-0134 USA [email protected] RUSSELL ADAMS Department of Psychiatry and Behavioral Science University of Oklahoma Health Sciences Center P.O. Box 26901 Oklahoma City, OK 73190 USA [email protected] CRISTY AKINS Mercy Family Center 110 Vetrans Memorial Blvd Metarie, LA 70005 USA [email protected] AMY ALDERSON Emory University/Rehabilitation Medicine 1441 Clifton Road Atlanta, GA 30322 USA [email protected] DANIEL N. ALLEN Department of Psychology University of Nevada Las Vegas Box 455030; 4505 Maryl and Parkway Las Vegas, NV 89154-5030 USA [email protected]

BRITTANY J. ALLEN Department of Health Psychology, DC 116.88 University of Missouri, Columbia One Hospital Drive Columbia, MO 65212 USA [email protected]

JASON VAN ALLEN Clinical Child Psychology Graduate Program University of Kansas 1000 Sunnyside Ave Lawrence, KS 66045 USA [email protected]

KARIN ALTERESCU Neuropsycholgy Program Queens College and The Graduate Center of the City University of New York Flushing, NY 11367 USA [email protected]

AKSHAY AMARANENI Department of Rehabilitation Medicine Emory University Atlanta, GA 30322 USA [email protected]

MELISSA AMICK Department of Psychiatry and Human Behavior Brown University Providence, RI 02912 USA and Department of Medical Rehabilitation Memorial Hospital of Rhode Island 111 Brewster Street Pawtucket, RI 02860 USA [email protected]

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List of Contributors

HEATHER ANDERSON Department of Neurology University of Kansas School of Medicine 3599 Rainbow Blvd., MS 2012 Kansas City, KS 66160 USA [email protected]

AMY J. ARMSTRONG Department of Rehabilitation Counseling Virginia Commonwealth University P.O. Box 980330 Richmond, VA 23298 USA [email protected]

STEVEN W. ANDERSON University of Iowa Hospitals and Clinics 0080-C RCP, 200 Hawkins Drive Iowa City, Iowa 52242 USA [email protected]

GLENN S. ASHKANAZI Department of Clinical and Health Psychology University of Florida-College of Public Health and Health Professions P.O. Box 100165 Gainesville, FL 32610-0165 USA [email protected]

KEVIN M. ANTSHEL Department of Psychiatry and Behavioral Sciences Upstate Medical University 750 East Adams Street Syracuse, NY 13210 USA and State University of New York - Upstate Medical University 1752 Greenspoint Court Syracuse Mount Pleasant, SC 29466 USA [email protected]

JENNIFER ANN NISKALA APPS Department of Psychiatry & Behavioral Medicine Children’s Hospital of Wisconsin/Medical College of Wisconsin 9000 W Wisconsin Ave Ste B510 Milwaukee, WI 53226 USA [email protected]

CAROL L. ARMSTRONG The Children’s Hospital of Philadelphia Neuro-Oncology/Neuropsychology 3535 Market Street, Ste. 1409-1410 Philadelphia, PA 19104 USA [email protected]

STEPHANIE ASSURAS Neuropsychology Program Queens College and The Graduate Center of the City University of New York Flushing, NY 11367 USA [email protected] JANE AUSTIN Department of Psychology William Paterson University 300 Pompton Road Wayne, NJ 7470 USA [email protected] BRADLEY AXELROD John D. Dingell VA Medical Center Psychology Section 4646 John R Street Detroit, MI 48201 USA [email protected] GLEN P. AYLWARD SIU School of Medicine-Pediatrics P.O. Box 19658 Springfield, IL 62794-9658 USA [email protected]


SAMANTHA BACKHAUS Neuropsychology Rehabilitation Hospital of Indiana 4141 Shore Dr. Indianapolis, IN 46254 USA [email protected] SANDRA BANKS Department of Psychiatry Allegheny General Hospital Four Allegheny Center Pittsburgh, PA 15212-5234 USA [email protected] JAMES H. BANÕS Department of Physical Medicine and Rehabilitation University of Alabama at Birmingham 619 19th Street South; SRC 530 Birmingham, AL 35249-7330 USA [email protected] RUSSELL BARKLEY State University of New York - Upstate Medical University 1752 Greenspoint Ct. Mt. Pleasant, SC 29466 USA [email protected] MARK S. BARON Neurology Virginia Commonwealth University Southeast/Richmond Veterans Affairs Parkinson’s Disease Research, Education and Clinical Center (PADRECC) Box 980599 Richmond, VA USA [email protected] IDA SUE BARON Director of Neuropsychology Inova Fairfax Hospital for Children Falls Church, VA 10116 Weatherwood Ct. Potomac, MD 20854 USA [email protected]

ERIKA M. BARON Rusk Institute of Rehabilitative Medicine Psychology Service Pediatrics New York University Langone Medical Center 550 First Avenue New York, NY 10016 USA [email protected] WILLIAM B. BARR New York University School of Medicine Medicical Center, Comprehensive Epilepsy Center 403 East 34th Street, EPC - 4th Floor New York, NY 10016 USA [email protected] RUSSELL M. BAUER Department of Clinical and Health Psychology University of Florida P.O. Box 100165 Health Science Center Gainesville, FL 32610-0165 USA [email protected] JESSICA BEAN Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected] PE´LAGIE M. BEESON Department of Speech, Language, & Hearing Sciences The University of Arizona Tucson, Arizona 85721-0071 USA [email protected] JAY BEHEL Department of Behavioral Sciences Rush University Medical Center 1653 W. Congress Parkway Chicago, IL 60612 USA [email protected]

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STACY BELKONEN Department of Rehab Medicine Mount Sinai School of Medicine 5 East 98th Street New York, NY 10029 USA [email protected]

JOHN BIGBEE Anatomy and Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA 23284 USA [email protected]

BRIAN D. BELL Department of Neurology University of Wisconsin 600 Highland Ave. Madison, WI 53792 USA [email protected]

ERIN D. BIGLER Department of Psychology Brigham Young University 1001 SWKT, P.O. Box 25543 Provo, UT 84602-5543 USA [email protected]

ANDREW BELL Department of Anatomy and Neurobiology Virginia Commonwealth University 1101 East Marshall Street Richmond, VA 23298-0709 USA [email protected]

NATALIE C. BLEVINS Department of Psychiatry Adult Psychiatry Clinic and Study Center Indiana Universtiy Hospital 550 N. University Blvd. Ste 3124 Indianapolis, IN 46202 USA [email protected]

H. ALLISON BENDER Neuropsychology Queens College CUNY 65-30 Kissena Blvd Flushing, NY 11367 USA and New York University Langone Medical Center 403 East 34th Street New York, NY 10016 USA [email protected] DANIEL B. BERCH Child Development and Behavior Branch National Institute of Child Health and Human Development, NIH 6100 Executive Blvd., Room 4B05 Bethesda, MD 20892-7510 USA and Curry School of Education University of Virginia Charlottesville, VA 22904–4260 USA [email protected]

MICHELLE L. BLOCK Anatomy and Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA 23284 USA [email protected] DOUG BODIN Department of Pediatrics Nationwide Children’s Hospital and The Ohio State University 700 Children’s Drive Columbus, OH 43205 USA [email protected] ANGELA M. BODLING Center for Health Care Quality University of Missouri—Columbia One Hospital Drive Columbia, MO 65212 USA [email protected]


ROBERT BOLAND Department of Psychiatry and Human Behavior The Warren Alpert Medical School of Brown University Butler Hospital Blackstone Blvd. Providence, RI 2906 USA [email protected]

JOHN G. BORKOWSKI Department of Psychology University of Notre Dame 118 Haggar Hall Notre Dame, IN 46556 USA [email protected]

JOAN C. BOROD Neuropsychology Program Queens College and The Graduate Center of the City University of New York 6530 Kissena Blvd. Flushing, NY 11367 USA and Mount Sinai School of Medicine One Gustave L. Levy Place New York, NY 10029-6574 USA [email protected]

BETH BOROSH Cognitive/Behavioral Neurology Center Northwestern Feinberg School of Medicine 675 N. Street Clair, Galter 20-100 Chicago, IL 60611 USA [email protected]

DAWN E. BOUMAN Medical Psychology and Neuropsychology Drake Center 151 W. Galbraith Road Cincinnati, OH 45216-1096 USA [email protected]

ISABELLE BOURDEAU Research Centre CHUM, Hoˆtel-Dieu 3850, rue Saint-Urbain Montreál, QC H2W 1T7 Canada [email protected] ALYSSA BRAATEN Emory University/Rehabilitation Medicine 1441 Clifton Road, Room 210 Atlanta, GA 30322 USA [email protected] LISA A. BRENNER VISN 19 MIRECC 1055 Clermont Street Denver, CO 80220 USA [email protected] ANDREW BRODBELT Consultant Neurosurgeon The Walton Centre for Neurology and Neurosurgery Lower Lane Liverpool L9 7LJ UK [email protected] JOHN BROWN Medical College of Georgia 1120 15th Street Augusta, GA 30912 USA [email protected] MARGARET BROWN Mount Sinai School of Medicine 272 West 107th Street, Apt. 7A New York, NY 10025 USA [email protected] SARAH S. CHRISTMAN BUCKINGHAM Department of Communication Sciences and Disorders The University of Oklahoma Health Sciences Center 825 NE 14th Street, P.O. Box 26901 Oklahoma City, OK 73126-0901 USA [email protected]

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HUGH W. BUCKINGHAM Sciences & Disorders and Interdepartmental Program in Linguistics Louisiana State University 136B Coates Hall Baton Rouge, LA 70803-2606 USA [email protected]

MERYL A. BUTTERS University of Pittsburgh School of Medicine, WPIC 3811 O’Hara Street Pittsburgh, PA 15213 USA [email protected]

JEFFREY M. BURNS Department of Neurology University of Kansas School of Medicine 3901 Rainbow Boulevard Kansas City, KS 66160 USA [email protected]

DEBORAH A. CAHN-WEINER UCSF Epilepsy Center University of California 400 Parnassus Avenue San Francisco, CA 94143-0138 USA [email protected]

THOMAS G. BURNS Neuropsychology Children’s Healthcare of Atlanta 1001 Johnson Ferry Road NE Atlanta, GA 30342 USA [email protected]

CHARLES D. CALLAHAN Memorial Medical Center 701 N. 1st Street Springfield, IL 62781 USA [email protected]

SHANE S. BUSH Long Island Neuropsychology, P.C 290 Hawkins Avenue, Suite B Lake Ronkonkoma, NY 11779 USA [email protected] TAMARA BUSHNIK Rusk Institute for Rehabilitation Medicine NYU Langone Medical Center 400 East 34th Street, RR115A New York, NY 10016 USA [email protected] MELISSA BUTTARO Department of Psychiatry Brown University, The Miriam Hospital 164 Summit Ave Providence, RI 2906 USA [email protected]

BRUCE CAPLAN Independent Practice 564 M.O.B. East 100 E. Lancaster Ave. Wynnewood, PA 19096 USA [email protected]

NOELLE E. CARLOZZI Outcomes & Assessment Research Laboratory Kessler Foundation Research Center 1199 Pleasant Valley Way West Orange, NJ 7052 USA [email protected]

HELEN M. CARMINE ReMed Paoli, PA USA


DOMINIC A. CARONE University Hospital – Neuropsychology Assessment Program SUNY Upstate Medical University 750 East Adams Street Syracuse, NY 13210 USA [email protected]

JENNIFER CASS Department of Pediatrics Nationwide Children’s Hospital and The Ohio State University 700 Children’s Drive Columbus, OH 43205 USA [email protected]

AMIRAM CATZ DEPARTMENT OF SPINAL LOEWENSTEIN REHABILITATION HOSPITAL 278 ACHVZA STREET RAANANA 43100 ISRAEL AND

TEL-AVIV UNIVERSITY TEL-AVIV ISRAEL [email protected]

COLBY CHLEBOWSKI Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected] WOON CHOW Anatomy & Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA USA [email protected] SHAWN E. CHRIST University of Missouri 25 McAlester Hall Columbia, MO 65211-2500 USA [email protected] SEVERN B. CHURN Neurology Virginia Commonwealth University Box 980599, MCV Station Richmond, VA 23298-0599 USA [email protected]

JESSICA CHAIKEN Media and Public Education Manager National Rehabilitation Information Center (NARIC) 8201 Corporate Drieve, Suite 600 Landover, MD 20785 USA [email protected]

ANGELA HEIN CICCIA Case Western Reserve University Department of Communication Sciences 11206 Euclid Avenue Room 410 Cleveland, OH 44106-7154 USA [email protected]

SANDY SUT IENG CHEANG University of Macau Department of Psychology Av. Padre Toma´s Pereira Taipa, Macau SAR China [email protected]

URAINA CLARK The Warren Alpert Medical School of Brown University The Miriam Hospital Neuropsychology, The CORO Center, 3rd Floor 1 Hoppin Street, Suite 317 Providence, RI 2903 USA [email protected]

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MARY CLARK University of Missouri 300 Portland Street, Suite 110 Columbia, MO 65211 USA [email protected] ELAINE CLARK Department of Educational Psychology University of Utah 1705 Campus Center Drive, #327 Salt Lake City, UT 84112-9255 USA [email protected] RONALD A. COHEN Department of Psychiatry and Human Behavior The Miriam Hospital Brown University 164 Summit Ave Providence, RI 2906 USA [email protected] MORRIS J. COHEN Neurology, Pediatrics & Psychiatry Director, Pediatric Neuropsychology, Medical College of Georgia and BT-2601 Children’s Medical Center 1446 Harper Street Augusta, Georgia 30912 USA [email protected] RAY COLELLO Anatomy & Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA USA [email protected] GRACE COMBS Applied Psychology and Counselor Education Department of Psychology, FSL University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected]

ADAM CONLEY Virginia Commonwealth University Medical Center Richmond, VA 23284 USA [email protected] W. CARL COOLEY Medical Director Center for Medical Home Improvement Crotched Mountain Foundation and Rehabilitation Center 1 Verney Drive Concord, NH 3047 USA [email protected] PATRICK COPPENS SUNY Plattsburgh Communication Disorders and Sciences 101 Broad Street Plattsburgh, NY 12901 USA [email protected] STEPHEN CORREIA Neuropsychology Butler Hospital Veterans Affairs Medical Center Warren Alpert Medical School of Brown University 345 Blackstone Blvd. Providence, RI 2906 USA [email protected] JOYCE A. CORSICA Department of Behavioral Sciences Rush University Medical Center 1653 W. Congress Parkway Chicago, IL 60612 USA [email protected] H. BRANCH COSLETT Department of Neurology University of Pennsylvania, HUP 3400 Spruce Street Philadelphia, PA 19104 USA [email protected]


JOHN C. COURTNEY Department of Psychology Children’s Hospital of New Orleans 200 Henry Clay Avenue New Orleans, LA 70118 USA [email protected]

DAVID R. COX Neuropsychology & Rehabilitation Consultants, PC 600 Market Street Suite 301 Chapel Hill, NC 27516 USA [email protected]

LAURA CRAMER-BERNESS Department of psychology William Paterson University 300 Pompton Road Wayne, NJ 7470 USA [email protected]

JUDY CREIGHTON Neuropsychology Program Queens College and The Graduate Center of the City University of New York Flushing, NY USA [email protected]

ANTHONY CUVO Center for Autism Spectrum Disorders Southern Illinois University, Mail Code 6607 Carbondale, LL 62901 USA [email protected] RIK CARL D’AMATO University of Macau Santa Clara Valley Medical Center Faculty of Social Sciences and Humanities 229 Tai Fung Building Taipa, Macau SAR China [email protected] KRISTEN DAMS-O’CONNOR Mount Sinai School of Medicine Department of Rehabilitation Medicine 5 East 98th Street Rm B-14 New York, NY 10029-6574 USA kristen.dams-o’[email protected] ANDREW S. DAVIS Department of Educational Psychology Ball State University Teachers College Room 524 Muncie, IN 47306 USA [email protected]

JACQUELINE L. CUNNINGHAM The Children’s Hospital of Philadelphia Department of Psychology, CSH 021 34th Street and Civic Center Blvd. Philadelphia, PA 19104-4399 USA [email protected]

SCOTT L. DECKER Counseling and Psychological Services Georgia State University P.O. Box 3980 Atlanta, GA 30302-3980 USA [email protected]

SEAN CUNNINGHAM Department of Educational Psychology University of Utah 1705 Campus Center Drive, #327 Salt Lake City, UT 84112-9255 USA [email protected]

NICK A. DEFILIPPIS Georgia School of Professional Psychology Argosy University 980 Hammond Drive NE Bldg. 2, Suite 100 Atlanta, GA 30328 USA [email protected]

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KATHLEEN DEIDRICK Department of Health Psychology Thompson Center for Autism and Neurodevelopmental Disorders University of Missouri-Columbia 300 Portland Street, Suite 110 Columbia, MO 65202 USA [email protected] DEAN C. DELIS University of California San Diego School of Medicine, San Diego Veterans Affairs Healthcare System SDVAMC, 3350 La Jolla Village Drive La Jolla, CA 92161 USA [email protected] JOHN DELUCA Neuropsychology and Neuroscience Laboratory Kessler Foundation Research Center 1199 Pleasant Valley Way West Orange, NJ 07052 USA [email protected] GEORGE J. DEMAKIS Department of Psychology University of North Carolina Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected] THESLEE JOY DEPIERO Boston University School of Medicine Braintree Rehabilitation Hospital 250 Pond Street Boston, MA 2184 USA [email protected] ROBERTA DEPOMPEI University of Akron Department of Speech Language, Pathology and Audiology Akron, OH 44325-3001 USA [email protected]

BRUCE J. DIAMOND Department of Psychology William Paterson University 300 Pompton Road Wayne, NJ 07470 USA [email protected] AIMEE DIETZ Communication Sciences and Disorders University of Cincinnati Hastings and Williams French Building 3202 Eden Avenue (Mail Location 0379) Cincinnati, OH 45267-0379 USA [email protected] MARCEL DIJKERS Mount Sinai School of Medicine One Gustave Levy Place, Box 1240 New York, NY 10029-6574 USA [email protected] CARL B. DODRILL Department of Neurology University of Washington School of Medicine 4488 West Mercer Way Seattle, WA 98040 USA [email protected] PETER DODZIK Clinical Psychology & Behavioral Sciences American School of Professional PsychologySchaumburg Argosy University Schaumburg Campus 999 Plaza Drive, Suite 800 Schaumburg, IL 60173 USA [email protected] JACOBUS DONDERS Mary Free Bed Rehabilitation Hospital 235 Wealthy SE Grand Rapids, MI 49503-5299 USA [email protected]


KERRY DONNELLY VA WNY Healthcare System University of Buffalo (SUNY) Behavioral Health Careline (116B) 3495 Bailey Avenue Buffalo, NY 14215 USA [email protected] LAUREN R. DOWELL Laboratory for Neurocognitive and Imaging Research Kennedy Krieger Institute 1750 E. Broadway, 3rd Floor Baltimore, MD 21205 USA [email protected]

JEFF DUPREE Anatomy & Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA USA [email protected]

MOIRA C. DUX Rosalind Franklin School of Medicine University of Maryland Medical Center/Baltimore VA 226 S. Ann Street Baltimore, MD 21231 USA [email protected]

LINDSEY DUCA Spinal Cord Injury Clinic VA Palo Alto Health Care System 3801 Miranda Ave. (128) Palo Alto, CA 94304 USA [email protected]

NATASHA K. EADDY Neurorehabilitation Specialists Baylor College of Medicine Brain Injury and Stroke Program Fellow Houston, TX USA [email protected]

ALEKSEY DUMER Queens College and The Graduate Center of the City University of New York NSB A340 6530 Kissena Blvd. Flushing, NY 11367 USA [email protected]

ANGELA EASTVOLD Department of Psychology University of Utah Salt Lake City, UT 84112-0251 USA [email protected]

MARY DUNKLE National Organization for Rare Disorders (NORD) 55 Kenosia Avenue, P.O. Box 1968 Danbury, CT 06813-1968 USA [email protected] KARI DUNNING Department of Rehabilitation Sciences University of Cincinnati P.O. Box 670394 Cincinnati, OH 45267-0394 USA [email protected]

DAWN M. EHDE Department of Rehabilitation Medicine University of Washington Seattle, WA 98195 USA [email protected]

ERIN E. EMERY Department of Behavioral Sciences Rush University Medical Center 1653 W. Congress Parkway Chicago, IL 60612 USA [email protected]

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ALLISON S. EVANS Department of Pediatrics/Deptartment of Psychiatry and Human Behavior Memorial Hospital of RI Neurodevelopmental Center 555 Prospect Street Pawtucket, RI 2860 USA [email protected]

DANIEL ERIK EVERHART Department of Psychology Eastern Carolina University Rawl Bldg, East 5th Street Greenville, NC 27858 USA [email protected]

NATHAN EWIGMAN Department of Clinical and Health Psychology University of Florida Gainesville, FL 32611 USA [email protected]

NATHALIE DE FABRIQUE Cook County Department of Corrections 750 N. Dearborn Street, #1504 Chicago, IL 60610 USA [email protected]

JOSEPH E. FAIR Brigham Young University 2062 Dakota Ave Provo, UT 84606 USA [email protected]

JAELYN R. FARRIS Department of Psychology University of Notre Dame Notre Dame, IN 46556 USA [email protected]

AMANDA FAULHABER William Paterson University Department of Psychology, Program in Clinical & Counseling Psychology 300 Pompton Road Wayne, NJ 07470 USA [email protected] DEBORAH A. FEIN University of Connecticut 406 Babbidge Raod, Unit 1020 Storrs, CT 06269-1020 USA [email protected] LEILANI FELICIANO Department of Psychology University of Colorado at Colorado Springs Colorado Springs, CO USA [email protected] WARREN L. FELTON Neurology Virginia Commonwealth University Medical Center Box 980599 Richmond, VA USA [email protected] ERIC M. FINE University of California, San Diego School of Medicine San Diego Veterans Affairs Healthcare System 1616 9th Ave. Apt. #23 La Jolla, CA 92101 USA [email protected] JESSICA FISH Medical Research Council Cognition and Brain Sciences Unit 15 Chaucer Road Cambridge, CB2 7EF UK [email protected]


JULIE TESTA FLAADA 2431 Wilshire Lane NE Rochester, MN 55906 USA [email protected]

Winthrop University Hospital State University of New York, Stony Brook School of Medicine Mineola, NY USA [email protected]

JENNIFER FLEMING School of Health and Rehabilitation Sciences The University of Queensland St Lucia, Brisbane, Queensland 4072 Australia [email protected]

HE´LE`NE FORGET Universite´ du Que´bec en Outaouais De´partement de psychoe´ducation et de psychologie Gatineau, QC Canada [email protected]

FAYE VAN DER FLUIT University of Wisconsin-Milwaukee Department of Psychology, Gerland Hall P.O. Box 413 Milwaukee, WI 53201-0413 USA [email protected]

JAMES R. FLYNN Department of Politics The University of Otago P.O. Box 56 Dunedin New Zealand [email protected]

KRISTIN JOAN FLYNN PETERS Department of Health Psychology University of Missouri Health Care, School of Health Professions One Hospital Dr., DC 116.88 Columbia, MO 65212 USA [email protected]

NANCY S. FOLDI Psychology Program Queens College and The Graduate Center of the City University of New York 65-30 Kissena Blvd Flushing, NY 11367 USA and

BONNY J. FORREST San Diego Center for Children 311 4th Avenue Suite 609 San Diego, CA 92111 USA [email protected] MICHAEL A. FOX Anatomy & Neurobiology Virginia Commonwealth University Medical Center Box 980709 Richmond, VA USA [email protected] LISA M. FOX Rusk Institute of Rehabilitative Medicine NYU Langone Medical Center, Psychology Department 400 E. 34th Street New York, NY 10016 USA [email protected] LAURA L. FRAKEY Memorial Hospital of Rhode Island and Alpert Medical School of Brown University Pawtucket, RI USA [email protected] ROBERT G. FRANK College of Public Health Kent State University P.O. Box 5190 Kent, OH 44242-0001 USA [email protected]

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MICHAEL FRANZEN Allegheny Neuropsychiatric Institute Allegheny General Hospital 4 Allegheny Center Pittsburgh, PA 15212 USA [email protected]

SARAH FREEMAN San Jose Unified School District 210 Tyler Ave. San Jose, CA 95117 USA [email protected]

KATHLEEN L. FUCHS Department of Neurology University of Virginia Health System P.O. Box 800394 Charlottesville, VA 22908-0394 USA [email protected]

PAMELA G. GARN-NUNN Professor of Speech-Language Pathology University of Akron Room 181, Polsky Building, 225 South Main Street Akron, OH 44325-3001 USA [email protected] KELLI WILLIAMS GARY PM&R Neuropsychology and Rehab Psychology Services Virginia Commonwealth University VCU Health Systems/MCV Hospitals and Physicians 1200 E. Broad Street, Room 3-102, Box 980542 Richmond, VA 23298 USA [email protected] BRANDON E. GAVETT Department of Neurology Boston University School of Medicine Boston, MA 02118-2526 USA [email protected]

TERISA GABRIELSON Department of Educational Psychology University of Utah 1705 Campus Center Drive, #327 Salt Lake City, UT 84112-9255 USA [email protected]

HELEN M. GENOVA Neuropsychology and Neuroscience Laboratory Kessler Foundation Research Center 300 Executive Drive, Suite 010 West Orange, NJ 7052 USA [email protected]

SHERRI GALLAGHER Flagstaff Unified School District 2910 N. Prescott Road Flagstaff, AZ 86001 USA [email protected]

GLEN GETZ Department of Psychiatry Allegheny General Hospital Four Allegheny Center Pittsburgh, PA 15212 USA [email protected]

FRANK J. GALLO University of Wisconsin-Milwaukee Department of Psychology P.O. Box 413 Milwaukee, WI USA [email protected]

GERARD A. GIOIA George Washington University School of Medicine Children’s National Medical Center 14801 Physician’s Lane, Suite 173 Rockville, MD 20850 USA [email protected]


ELIZABETH LOUISE GLISKY Department of Psychology University of Arizona, 1503 East University Blvd/ P.O. Box 210068 Tucson, AZ 85721 USA [email protected]

EMILIE GODWIN Virginia Commonwealth University 1223 East Marshall Street Richmond, VA 23298-0542 USA [email protected]

GARY GOLDBERG Virginia Commonwealth University School of Medicine/ Medical College of Virginia Richmond, VA USA [email protected]

BRAM GOLDSTEIN Hoag Hospital Cancer Center Department of Gynecologic Oncology 351 Hospital Road, Ste. 507 Newport Beach, CA 92663 USA [email protected] ASSAWIN GONGVATANA Neuropsychology Brown University The Miriam Hospital, Coro Bldg. 3-West One Hoppin Street Providence, RI 02906 USA [email protected] DANIEL GOOD Brigham Young University 395 North 100 East Provo, UT 84062 USA [email protected]

MYRON GOLDBERG Department of Rehabilitation Medicine University of Washington Medical Center 1959 NE Pacific Street, Box 356490 Seattle, WA 98195-6450 USA [email protected]

ROBERT M. GORDON Rusk Institute of Rehabilitation Medicine New York University Langone Medical Center 400 East 34th Street, Room 507A-RR New York, NY 10016 USA [email protected]

DIANE CORDRY GOLDEN Association of Assistive Technology Act Programs P.O. Box 32 Delmar, NY 12054 USA [email protected]

KIMBERLY A. GORGENS Graduate School of Professional Psychology University of Denver, MSC 4104 2450 South Vine Street, MSC 4101 Denver, CO 80208 USA [email protected]

CHARLES J. GOLDEN Center for Psychological Studies Nova Southeastern University 3301 College Avenue Fort Lauderdale, FL 33314 USA [email protected]

JANET GRACE Medical Rehabilitation Memorial Hospital of RI 111 Brewster Street Pawtucket, RI 2860 USA [email protected]

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MARTIN R. GRAF Department of Neurosurgery Virginia Commonwealth University Medical Center P.O. Box 980631 Richmond, VA 29298-0631 USA [email protected]

AUDREY H. GUTHERIE Rehabilitation Research & Development Center of Excellence Atlanta Veterans Administration Medical Center 1670 Clairmont Road Decatur, GA 30033 USA [email protected]

LORI GRAFTON Carolinas Rehabilitation Carolinas HealthCare System Charlotte, NC 28232-2861 USA [email protected]

KARL HABERLANDT Department of Psychology Trinity College 300 Summit Street Hartford, CT 6119 USA [email protected]

MICHAEL R. GREHER National Jewish Health and University of Colorado Denver School of Medicine Denver, CO USA [email protected]

MAUREEN GRISSOM University of Missouri, Department of Health Psychology MU Thompson Center for Autism and Neurodevelopmental Disorders 300 Portland Street Suite 110 Columbia, MO 65211 USA [email protected]

ELIZABETH STANNARD GROMISCH Trinity College 1005 Smith Ridge Road Hartford, CT 6840 USA [email protected]

WILLIAM GUIDO Anatomy & Neurobiology Virginia Commonwealth University Medical Center Box 980709 Richmond, VA USA [email protected]

MARTIN HAHN Department of Biology William Paterson University Wayne, NJ 7470 USA [email protected] KATHRINE HAK Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected] MARLA J. HAMBERGER New York Presbyterian Columbia Comprehensive Epilepsy Center The Neurological Institute Columbia University 710 West 168 Street, 7th floor New York, NY 10032 USA [email protected] FLORA HAMMOND Brain Injury Program Director/Research Director Carolinas Rehabilitation 1100 Blythe Blvd Charlotte, NC 28203 USA [email protected]


BENJAMIN M. HAMPSTEAD Emory University/Rehabilitation Medicine, Atlanta VAMC RR&D CoE 1441 Clifton Road Suite 150 Atlanta, GA 30322 USA [email protected]

JANNA L. HARRIS Hoglund Brain Imaging Center University of Kansas Medical Center 3901 Rainbow Blvd. Mail Stop 1052 Kansas City, KS 66160 USA [email protected]

ERIC S. HART University of Missouri Center for Health Care Quality Clinical Support and Education Building Columbia, MO 65212 USA [email protected]

TRISHA HAY Hoglund Brain Imaging Center University of Kansas Medical Center 3901 Rainbow Blvd Kansas City, KS 66160 USA [email protected]

AMY HEFFELFINGER Associate Professor of Neurology Medical College of Wisconsin 9200 W. Wisconsin Ave Milwaukee, WI 53226 USA [email protected]

ROBERT L. HEILBRONNER Chicago Neuropsychology Group 333 N. Michigan Avenue, #1801 Chicago, IL 60601 USA [email protected] [email protected]

KENNETH M. HEILMAN Department of Neurology University of Florida College of Medicine The Malcom Randall Veterans Affairs Hospital Box 100236 Gainesville, FL 32610 USA [email protected] NATHAN HENNINGER Department of Pediatrics Nationwide Children’s Hospital College of Medicine, Ohio State University 700 Children’s Drive Columbus, OH 43205 USA [email protected] MARY HIBBARD Rusk Institute of Rehabilitation Medicine New York, NY 10016 USA [email protected] YVONNE HINDES Division of Applied Psychology Faculty of Education, University of Calgary 2500 University Drive N.W Calgary, AB T2N 1N4 Canada [email protected] MERRILL HISCOCK Department of Psychology University of Houston Houston, TX 77204-5022 USA [email protected] ELISE K. HODGES Department of Psychiatry University of Michigan Health System Neuropsychology Division 2101 Commonwealth, Suite C Ann Arbor, MI 48105 USA [email protected]

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ANNA DEPOLD HOHLER Boston University Medical Center 720 Harrison Avenue, Suite 707 Boston, MA 2118 USA [email protected]

BRADLEY J. HUFFORD Neuropsychology Rehabilitation Hospital of Indiana 4141 Shore Drive Indianapolis, IN 46254 USA [email protected]

TRACEY HOLLINGSWORTH Nationwide Children’s Hospital Developmental Assessment Program 187 W. Schrock Road Columbus, OH 43081 USA [email protected]

JOEL W. HUGHES Department of Psychology Kent State University 228 Kent Hall Kent, OH 44242-0001 USA [email protected]

KARIN F. HOTH National Jewish Medical and Research Center National Jewish Health Denver, CO USA [email protected]

DAVID HULAC Division of Counseling and Psychology in Education University of South Dakota 414 E. Clark Street Vermillion, SD 57069 USA [email protected]

MARIANNE HRABOK Department of Psychology University of Victoria P.O. Box 3050, STN CSC Victoria, BC V8W 3P5 Canada [email protected]

EDWARD E. HUNTER Department of Psychiatry and Behavioral Sciences University of Kansas Medical Center 3901 Rainbow Boulevard Kansas City, KS 66160 USA [email protected]

LEESA V. HUANG Department of Psychology-0234 California State University 400 West First Street Chico, CA 95928-9924 USA [email protected]

SCOTT J. HUNTER Department of Psychiatry & Behavioral Neuroscience University of Chicago 5841 S Maryland Ave., MC 3077 Chicago, IL 60637 USA [email protected] [email protected]

DAWN H. HUBER Pediatric Neuropsychological Services, LLC 1829 S. Kentwood, Suite 108 Springfield, MO 65804 USA [email protected]

KAREN HUX Special Education and Communication Disorders University of Nebraska – Lincoln 318N Barkley Memorial Center Lincoln, NE 68583-0738 USA [email protected]


SUMMER IBARRA Rehabilitation Hospital of Indiana 4141 Shore Drive Indianapolis, IN 46254 USA [email protected] FARZIN IRANI Psychiatry University of Pennsylvania 3400 Spruce street, 10 Gates Philadelphia, PA 19104 USA [email protected] CINDY B. IVANHOE Neurorehabilitation Specialists Baylor College of Medicine The Institute for Rehabilitation and Research 1333 Moursund Avenue, D110 Houston, TX 77030 USA [email protected]

MATTHEW JACOBS Deparment of Psychology Pennsylvannia State University 111 Moore Building University Park, PA 16802 USA [email protected] LISA A. JACOBSON Department of Neuropsychology Kennedy Krieger Institute Johns Hopkins University School of Medicine 1750 East Fairmount Ave. Baltimore, MD 21231 USA [email protected] KELLY M. JANKE University of Wisconsin-Milwaukee Department of Psychology P.O. Box 413 Milwaukee, WI 53201-0413 USA [email protected]

GRANT L. IVERSON Department of Psychiatry University of British Columbia British Columbia Mental Health & Addictions 2255 Wesbrook Mall Vancouver, BC V6T 2A1 Canada [email protected]

NICHOLAS JASINSKI Division of Neuropsychology Henry Ford Health System 1 Ford Place Detroit, MI 48202 USA [email protected]

COLLEEN E. JACKSON Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected]

BETH A. JERSKEY Department of Psychiatry and Human Behavior Alpert Medical School of Brown University Butler Hospital Blackstone Blvd. Providence, RI 2906 USA [email protected]

KIMBERLE M. JACOBS Department of Anatomy and Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA 23298-0709 USA [email protected]

CHASMAN JESSE Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected]

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AMITABH JHA TBIMS National Data and Statistical Center Craig Hospital 3425 South Clarkson Street Englewood, CO 80113 USA [email protected]

MI-YEOUNG JO Private Practice 15353 Valerio Street Van Nuys, CA 91406 USA [email protected]

SUSAN K. JOHNSON Department of Psychology University of North Carolina at Charlotte 9201 University City Blvd. Charlotte, NC 28223-0001 USA [email protected]

JULENE K. JOHNSON UCSF Epilepsy Center University of California 400 Parnassus Avenue San Francisco, CA 94143-0138 USA [email protected]

JUDY A. JOHNSON Pasadena Independent School District 29731 Sullivan Oaks Drive Pasadena, TX 77386 USA [email protected]

NANCY JOHNSON Cognitive/Behavioral Neurology Center Northwestern Feinburg School of Medicine 675 N. Street Clair, Galter 20-100 Chicago, IL 60611 USA [email protected]

KRISTIN L. JOHNSON Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected] ERIN JOYCE Pacific Graduate School of Psychology–Stanford Doctor of Psychology Consortium Spinal Cord Injury Clinic VA Palo Alto Health Care System 3801 Miranda Ave. (128) Palo Alto, CA 94304 USA [email protected] AARON N. JUNI Neuropsychology and Rehabilitation Psychology Department of Physical Medicine & Rehabilitation The Johns Hopkins School of Medicine 600 North Wolfe Street/Phipps 174 Baltimore, MD 21287 USA [email protected] STEPHEN M. KANNE Thompson Center for Autism and Neurodevelopmental Disorders University of Missouri 300 Portland, Suite 110 Columbia, MO 65211 USA [email protected] RICHARD F. KAPLAN Department of Psychiatry (MC-2103) UConn Health Center 263 Farmington Ave Farmington, CT 06030-2103 USA [email protected] PAUL E. KAPLAN Capitol Clinical Neuroscience 104 Summer Shade Court Folsom, CA 95630-1565 USA [email protected]


EDITH KAPLAN Department of Psychology Suffolk University 26 Laconia Street, P.O. Box 476 Boston, MA 02420-0005 USA [email protected] NARINDER KAPUR Neuropsychology Department Addenbrooke’s Hospital R3 Neurosciences, Box 83 Cambridge, CB2 0QQ UK [email protected] STELLA KARANTZOULIS Neuropsychology Program NYU Langone Medical Center City University New York, NY USA [email protected] DOUGLAS I. KATZ Boston University School of Medicine Braintree Rehabilitation Hospital 250 Pond Street Boston, MA 2184 USA [email protected] MICHAEL KAUFMAN Department of Neurology Carolinas Medical Center 1010 Edgehill Road North Charlotte, NC 28207-1885 USA [email protected] JACOB KEAN Department of Physical Medicine and Rehabilitation Indiana University School of Medicine 200 S. Jordan Avenue Indianapolis, IN 47405 USA [email protected]

SALLY L. KEMP University of Missouri 1328 Secluded Woods Drive Columbia, MO 56020 USA [email protected] KIMBERLY A. KERNS Department of Psychology University of Victoria Victoria, BC V8W 3P5 Canada [email protected] FARY KHAN Department of Medicine University of Melbourne and the Royal Melbourne Hospital Bldg 21, Royal Park Campus Parkville, VA, VIC 3152 Australia [email protected] SO HYUN KIM University of Michigan Autism and Communication Disorders Center (UMACC) 2236 East Hall Ann Arbor, MI 48109-0406 USA [email protected] TRICIA Z. KING Georgia State University Department of Psychology 140 Decatur Street, Suite 1151 Atlanta, GA 30303 USA [email protected] JENNIFER SUE KLEINER Department of Psychology University of Arkansas for Medical Sciences Blandford Physician Center Suite 410, 4301 West Markham Street, #568 Little Rock, AR 72205 USA [email protected]

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BONITA P. KLEIN-TASMAN Department of Psychology University of Wisconsin-Milwaukee 2441 E. Hartford Ave. Milwaukee, WI 53211 USA [email protected]

KATE KRIVAL Speech Pathology, School of Health Sciences Kent State University A111 Music and Speech Bldg Kent, OH 44242 USA [email protected]

STEPHANIE A. KOLAKOWSKY-HAYNER Director, Rehabilitation Research Santa Clara Valley Medical Center Rehabilitation Research Center 751 South Bascom Ave. San Jose, CA 95128 USA [email protected]

LAUREN B. KRUPP Department of Neuropsychology Research Stony Brook University SUNY Stony Brook Stony Brook, NY 11794 USA [email protected]

ELIZABETH KOZORA Department of Medicine National Jewish Medical, and Research Center National Jewish Health 1400 Jackson Street Denver, CO 80208 USA [email protected]

JOEL H. KRAMER UCSF Memory and Aging Center UCSF Med Ctr, 0984-8AC 350 Parnassus Ave, Suite 706 San Francisco, CA 94143 USA [email protected]

MATTHEW KRAYBILL Department of Psychology University of Utah Salt Lake City, UT 84112-0251 USA [email protected]

DENISE KRCH Kessler Foundation Research Center West Orange, NJ USA [email protected]

BRAD KUROWSKI Cincinnati Children’s Hospital Medical Center University of Pittsburgh Cincinnati, OH USA [email protected]

MATTHEW M. KURTZ Department of Psychology Wesleyan University Judd Hall 314 Middletown, CT 6459 USA [email protected]

MONICA KURYLO Department of Rehabilitation Medicine University of Kansas Medical Center 3901 Rainbow Blvd Kansas City, KS 66160 USA [email protected]

CHRISTINA KWASNICA Barrow Neurological Institute 222 W Thomas Road Ste 212 Phoenix, AZ 85013 USA [email protected]


DAVID LACHAR University of Texas Houston Health Science Center 1300 Moursund Houston, TX 77030 USA [email protected] SUSAN LADLEY-O’BRIEN University of Colorado Health Sciences Center Department of Physical Medicine an Denver Health Medical Center 777 Bannock Street #0113 Denver, CO 80204 USA Susan.Ladley-O’[email protected] GINETTE LAFLECHE Memory Disorders Research Center VA Boston Healthcare System and Boston University School of Medicine 150 S. Huntington Ave. (151A) Boston, MA 2130 USA [email protected] AUDREY LAFRENAYE Department of Anatomy and Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA 23298-0709 USA [email protected]

GUDRUN LANGE Department of Radiology University of Medicine & Denistry of New Jersey Pain and Fatigue Study Center, UMDNJ-New Jersey Medical School 30 Bergen Street, ADMC 1618 Newark, NJ 07103 USA [email protected] KAREN G. LANGER Rusk Institute of Rehabilitation Medicine NYU Langone Medical Center Department of Psychology 400 E. 34th Street, RR-515 Flushing, NY 10016 USA [email protected] MICHAEL J. LARSON Brigham Young University 3032 E. 1530 S. Provo, UT 84660 USA [email protected] JENNIFER C. GIDLEY LARSON Department of Psychology University of Utah Salt Lake City, UT 84112-0251 USA [email protected]

SARAH K. LAGEMAN Division of Neuropsychology and Behavioral Health Department of Rehabilitation Medicine Emory University 1441 Clifton Road NE Atlanta, GA 30322 USA [email protected]

THOMAS M. LAUDATE Boston University Brigham and Women’s Hospital 648 Beacon Street, 2nd Floor Boston, MA 02215-2013 USA [email protected]

RAEL T. LANGE British Columbia Mental Health and Addiction Services University of British Columbia PHSA Research and Networks Suite 201, 601 West Broadway Vancouver, BC V5Z 4C2 Canada [email protected]

RONALD M. LAZAR Cerebrovascular Division/Department of Neurology Neurological Institute of New York Columbia University Medical Center 710 West 168th Street New York, NY 10032 USA [email protected]

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VICTORIA M. LEAVITT Kessler Foundation Research Center 1468 Midland Ave., apt 1B West Orange, NJ 10708 USA [email protected]

SOPHIE LEBRECHT Brown University Visual Neuroscience Laboratory Waterman Street Providence, RI 02903 USA [email protected]

SING LEE Department of Psychiatry The Chinese University of Hong Kong 7A, Block E, Staff Quarters, Prince of Wales Hospital Shatin, HKSAR, PRC China [email protected]

ANDREA M. LEE University of Manitoba 1702-72 Donald Street Winnipeg, MB R3C 1L7 Canada [email protected]

GEORGE LEICHNETZ Virginia Commonwealth University Richmond, VA USA [email protected]

HOYLE LEIGH Department of Psychiatry University of California, San Francisco 155 N. Fresno Street Fresno, CA 93701 USA [email protected]

JEANNIE LENGENFELDER Kessler Foundation Research Center West Orange, NJ 07052 USA [email protected]

MARCUS PONCE DE LEON Chief, Neurology Service William Beaumont Army Medical Center 5005 N. Piedras Street El Paso, Texas 79920-5001 USA [email protected]

KANGMIN D. LEE Department of Neurosurgery Virginia Commonwealth University Box 980631 Richmond, VA USA [email protected]

TERRY LEVITT Independent Practice 1324 College Drive Saskatoon, Saskatchewan S7N 0W5 Canada [email protected]

STACIE A. LEFFARD Rehabilitation Psychology and Neuropsychology Physical Medicine & Rehabilitation, University of Michigan 325 E. Eisenhower Parkway Ann Arbor, MI 48108 USA [email protected]

ALLEN N. LEWIS Department of Rehabilitation Counseling School of Allied Health Professions Virginia Commonwealth University P.O. Box 980330 Richmond, VA 23298 USA [email protected]


PAMELA H. LEWIS Department of Rehabilitation Counseling School of Allied Health Professions, Virginia Commonwealth University 980330 Richmond, VA 23298-0330 USA [email protected]

DAVID J. LIBON Department of Neurology Drexel University, College of Medicine New College Building, Mail Stop 423, 245 North 15th Street Philadelphia, PA 19102 USA [email protected]

DEBBIE LICHESKY American Academy of Pediatrics Elk Grane Village, IL USA

MARY BETH LINDSAY Department of Educational Psychology University of Utah 1705 Campus Center Drive, #327 Salt Lake City, UT 84112-9255 USA [email protected]

CASSIE LINDSTROM Dept of Psychology UNC-Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected]

DONAEC LOCKE Psychiatry and Psychology Mayo Clinic 13400 East Shea Blvd Scottsdale, AZ 85259 USA [email protected]

CHRIS LOFTIS National Council for Community Behavioral Healthcare STG International 1527 N. Van Dorn Street Alexandria, VA 22304 USA [email protected] KENNETH J. LOGAN Department of Communication Sciences & Disorders University of Florida P.O. Box 117420, 343 Dauer Hall Gainesville, FL 32611-7420 USA [email protected] CATRINA C. LOOTENS Department of Pediatrics University of Kansas Medical Center, MS 4004, G005 Miller 3901 Rainbow Blvd. Kansas City, KS 66160-7330 USA [email protected] EDUARDO LOPEZ Associate Medical Director/Clinical Services Center for Head Injuries JFK Johnson Rehabilitation Institute 65 James Street Edison, NJ 8818 USA [email protected] CATHERINE LORD Autism and Communication Disorders Center (UMACC) University of Michigan 300 North Ingalls, 10th Floor Ann Arbor, MI 48109-0406 USA [email protected] JANIS LORMAN The University of Akron School of Speech–Language Pathology and Audiology Room 181, Polsky Building, 225 South Main Street Akron, OH 44325-3001 USA [email protected]

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N. G. LOUISA Department of Rehabilitation Medicine Royal Melbourne Hospital Parkville, Victoria Australia [email protected]

and Johns Hopkins University School of Medicine Baltimore, MD 21205 USA [email protected]

STEPHEN D. LUKE National Dissemination Center for Children with Disabilities (NICHCY) Washington, DC USA [email protected]

BRI MAKOFSKE Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected]

KRISTINE LUNDGREN Department of Communication Sciences and Disorders University of North Carolina at Greensboro 323 Ferguson Building P.O. Box 26170 Greensboro, NC 27402 USA [email protected]

JAMES F. MALEC Rehabilitation Hospital of Indiana 4141 Shore Drive Indianapolis, IN 46254 USA [email protected]

JON G. LYON 6344 Hillsandwood Road Mazomanie, WI 53560 USA [email protected]

AMIT MALHOTRA Kaiser Permanente Medical Center 280 West MacArthur Boulevard Oakland, CA 94611-5693 USA [email protected]

DONALD E. LYTLE Department of Psychology California State University 400 West First Street Chico, CA 95928-0234 USA [email protected]

PAUL MALLOY The Warren Alpert Medical School of Brown University Butler Hospital 345 Blackstone Blvd. Providence, RI 2906 USA [email protected]

ANNA MACKAY-BRANDT Department of Psychiatry and Human Behavior Brown University Medical School 78 Dana Street Providence, RI 2906 USA [email protected]

WILLIAM VICTOR MALOY The Virginia Institute of Pastoral Care 2000 Bremo Road, Suite 105 Richmond VA 23226 USA [email protected]

E. MARK MAHONE Department of Neuropsychology Kennedy Krieger Institute 1750 E. Fairmount Avenue Baltimore, MD 21231 USA

CARLYE G. MANNA Neuropsychology Program New York State Psychiatric Institute New York, NY USA [email protected]


ASHLEY DE MARCHENA Department of Psychology University of Connecticut 406 Babbidge Road Storrs, CT 06269-1020 USA [email protected]

JEANNE W. MCALLISTER Center for Medical Home Improvement Crotched Mountain 18 Low Avenue Concord, NH 3301 USA [email protected]

BERNICE A. MARCOPULOS Department of Psychiatry and Neurobehavioral Sciences University of Virginia, Director, Neuropsychology Lab Western State Hospital Box 2500 Charlottesville, VA 24402-2500 USA [email protected]

DAVID MCCABE Queens College and The Graduate Center of the City University of New York Department of Psychology 65-30 Kissena Blvd. Flushing, NY 11367 USA [email protected]

CHRISTINA R. MARMAROU Neurosurgery Virginia Commonwealth University Box 980631 Richmond, VA USA [email protected]

REBECCA MCCARTNEY Emory University/Rehabilitation Medicine 1441 Clifton Road NE Atlanta, GA 30322 USA [email protected]

GUIDO MASCIALINO Department of Rehab Medicine Mount Sinai School of Medicine 5 East 98th Street New York, NY 10029 USA [email protected]

DALENE MCCLOSKEY Centennial Board of Cooperative Educational Services 16473 Longs Peak Road Greeley, CO 80631 USA [email protected]

MICAH O. MAZUREK Thompson Center for Autism and Neurodevelopmental Disorders University of Missouri 300 Portland, Suite 110 Columbia, MO 65211 USA [email protected]

ERICA MCCONNELL University of Northern Colorado 2250 Ironton Street Greeley, CO 80010 USA [email protected]

MICHE´LE M. M. MAZZOCCO Johns Hopkins University School of Medicine Kennedy Krieger Institute 707 North Broadway Baltimore, MD 21211 USA [email protected]

MICHAEL A. MCCREA Executive Director Neuroscience Center 721 American Avenue, Suite 501 Waukesha, WI 53188 USA [email protected]

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JACINTA MCELLIGOTT National Rehabilitation Hospital Rochestown Avenue Dun Laoghaire, CO Dublin Ireland [email protected]

MELISSA J. MCGINN Anatomy & Neurobiology Virginia Commonwealth University School of Medicine Box 980709 Richmond, VA USA [email protected]

DAVID E. MCINTOSH Ball State University Department of Special Education, Teachers College Room 722 Muncie, IN 47306 USA [email protected]

MIECHELLE MCKELVEY Department of Communication Disorders COE B141, University of Nebraska Kearney Kearney, NE 68849 USA [email protected]

NICOLE C. R. MCLAUGHLIN Butler Hospital Alpert Medical School of Brown University 345 Blackstone Blvd Providence, RI 02906 USA [email protected]

BRIAN T. MCMAHON Department of Rehabilitation Counseling Virginia Commonwealth University P.O. Box 980330 Richmond, VA 23298 USA [email protected]

LEMMIETTA MCNEILLY Chief Staff Officer Speech-Language Pathology, American SpeechLanguage-Hearing Association 2200 Research Boulevard, Rockville, MD 20850-3289 USA [email protected] RORY MCQUISTON Anatomy & Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA USA [email protected] LINDA MCWHORTER Department of Psychology University of North Carolina at Charlotte 9201 University City Blvd North Carolina Charlotte, NC 28223 USA [email protected] MARY-ELLEN MEADOWS Division of Cognitive and Behavioral Neurology Brigham and Women’s Hospital 221 Longwood Ave Boston, MA 2115 USA [email protected] MICHAEL S. MEGA Cognitive Assessment Clinic Providence Brain Institute, Providence Health System 9427 SW Barnes Road, Suite 595 Portland, OR 97225 USA [email protected] STEPHEN S. MEHARG Center for Memory and Learning 945 – 11th Ave Suite A Longview, WA 98632 USA [email protected]


JOHN E. MENDOZA SE LA Veterans Healthcare System Department of Psychiatry and Neurology Tulane University Medical Center 3928 S. Inwood Ave. New Orleans, LA 70131 USA [email protected]

JOHN E. MEYERS Private Practice Neuropsychology Schofield Barracks, Concussion Clinic 94-553 Alapoai Street # 162 Mililani, HI 96789 USA [email protected]

MARK MENNEMEIER Neurobiology and Developmental Sciences University of Arkansas for Medical Sciences 4301 W Markham Slot 826 Little Rock, AR 72205-7199 USA [email protected]

DAVID MICHALEC Division of Psychology Ohio State University Nationwide Children’s Hospital Developmental Assessment Program 187 W. Schrock Road Columbus, OH 43081 USA [email protected]

RANDALL E. MERCHANT Virginia Commonwealth University Medical Center Box 980709 MCV Station Richmond, VA 23298-0709 USA [email protected] BRAD MERKER Henry Ford Health Systems 1 Ford Place, 1E Detroit MI 48202 USA [email protected] GARY B. MESIBOV University of North Carolina at Chapel Hill CB 7180, 310 Medical School Wing E Chapel Hill, NC 27599-7180 USA [email protected] TIMOTHY VAN METER Virginia Commonwealth University Richmond, VA USA [email protected] LINDA MEYER Communication Services Woodrow Wilson Rehabilitation Center P.O. Box 1500 Fishersville, VA 22939-1500 USA [email protected]

ERIC N. MILLER UCLA Psychology Clinic 2191 Franz Hall Los Angeles, CA 90095 USA [email protected] ETHAN MOITRA Drexel University Department of Psychology 509 Windwood Place Morgantown, WV 26505 USA [email protected] DORIS S. MOK Department of Psychology Faculty of Social Sciences and Humanities University of Macau Av. Padre Toma´s Pereira Taipa, Macau SAR China [email protected] ANNA BACON MOORE Department of Rehabilitation Medicine, Division of Neuropsychology Emory University School of Medicine 1441 Clifton Road Suite 150 Atlanta, GA 30322 USA [email protected]

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AMY C. MOORS Villanova University Department of Psychology 800 Lancaster Ave Villanova, PA 19085 USA [email protected] LISA MORAN Department of Psychology Nationwide Children’s Hospital 700 Children’s Drive Columbus, OH 43205 USA [email protected] JOSEPH E. MOSLEY Department of psychology William Paterson University 300 Pompton Road Wayne, NJ 7470 USA [email protected] MARGARET MOULT Olin Neuropsychiatry Research Center Institute of Living 400 Washington Street Hartford, CT 6106 USA [email protected] MARY PAT MURPHY MSN, CRRN Paoli, PA USA SUZANNE MUSIL Rush University Medical Center Chicago, IL USA [email protected] [email protected] SYLVIE NAAR-KING UHC 6d5, 4201 St. Antoine Detroit, MI 48201 USA [email protected]

LUBA NAKHUTINA New York University Langone Medical Center Queens College and The Graduate Center of The City University of New York, Room NSB-318 65-30 Kissena Blvd Flushing, NY 11367 USA [email protected] AARON P. NELSON Division of Cogntive and Behavioral Neurology Brigham and Women’s Hospital Bosten University 221 Longwood Ave Boston, MA 2115 USA [email protected] CHRISTINA NESSLER Aphasia/Apraxia Research Program VA Salt Lake City Healthcare System 500 Foothill Drive, 151-A Salt Lake City, UT 84148 USA [email protected] ADRIAN NESTOR Department of Cognitive and Linguistic Sciences Brown University P.O. 1978 Providence RI 02906 USA [email protected] PAUL NEWMAN Department of Medical Psychology and Neuropsychology Drake Center 151 West Galbraith Road Cincinnati, OH 45216-1096 USA [email protected] CHRISTINE MAGUTH NEZU Department of Psychology Drexel University–Hahnemann Campus Mail Stop 515, 245 N 15th Street Philadelphia, PA 19102-1192 USA [email protected]


JANET P. NIEMEIER Department of Neuropsychology and Rehabilitation Psychology Virginia Commonwealth University, School of Medicine P.O. Box 980661 Richmond, VA 23298 USA [email protected] C. MICHAEL NINA Department of Psychology William Paterson University 300 Pompton Road Wayne, NJ 7470 USA [email protected] VIRGINIA A. NORRIS Spinal Cord Injury Clinic VA Palo Alto Health Care System 3801 Miranda Ave. (128) Palo Alto CA 94304 USA [email protected] OLGA NOSKIN Department of Neurology The Neurological Institute of New York Columbia University College of Physicians and Surgeons 710 W 168th Street, NI-6 New York, NY 11032 USA [email protected]

JONATHAN A. OLER Department of Psychiatry University of Wisconsin, 6001 Research Park Blvd Madison, WI 53719 USA [email protected] KATHLEEN O’TOOLE Children’s Healthcare of Atlanta Atlanta, GA USA kathleen.o’[email protected] ROHAN PALMER Institute for Behavioral Genetics University of Colorado at Boulder 447 UCB Boulder, CO 80309-0447 USA [email protected] CHRISTINA A. PALMESE Department of Neurology Beth Israel Medical Center 10 Union Square East, Suite 5D New York, NY 10003 USA [email protected]

THOMAS A. NOVACK Department of Psychiatry and Behavioral Neurobiology University of Alabama at Birmingham 619 19th Street S Birmingham, AL 35249-7330 USA [email protected]

JUHI PANDEY Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA and The Children’s Hospital of Philadelphia Philadelphia, PA USA [email protected]

THOMAS OAKLAND Department of Educational Psychology College of Education University of Florida 1410 Norman Hall Gainesville, FL USA [email protected]

BO CARLOS PANG Department of Economics Faculty of Social Sciences and Humanities University of Macau Av. Padre Toma´s Pereira Taipa, Macau SAR China [email protected]

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KATHRYN V. PAPP Department of Psychology The University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected]

RICK PARENTE Department of Psychology Towson University Towson, MD USA [email protected]

MATTHEW R. PARRY Virginia Commonwealth University Richmond, VA USA [email protected]

JANET PATTERSON Department of Communicartive Sciences and Disorders California State University East Bay 25800 Carlos Bee Blvd. Hayward, CA 94542 USA [email protected]

SHELLEY PELLETIER Board Certified in School Psychology Shoreline Pediatric Neuropsychology Services, LLC 954 Middlesex Turnpike, A2 Old Saybrook, CT 6475 USA [email protected]

KENNETH PERRINE Northeast Regional Epilepsy Group 104-20 Queens Blvd., Apt. 10C Hackensack, NJ 11375 USA [email protected]

AMY PETERMAN Department of Psychology University of North Carolina at Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected]

JO ANN PETRIE Brigham Young University Provo, UT USA [email protected]

LEADELLE PHELPS University at Buffalo, State University of New York 427 Baldy Hall Buffalo, NY 14260 USA [email protected]

KRISTIN D. PHILLIPS Medical College of Wisconsin-Milwaukee Department of Neurology, Division of Neuropsychology 9200 W. Wisconsin ave Milwaukee, WI 53226 USA [email protected]

LINDA L. PHILLIPS Anatomy & Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA USA [email protected]

WADE PICKREN Ryerson University Department of Psychology, American Psychological Association 350 Victoria Street Toronto, ON ON M5B 2K3 Canada [email protected]


ERIC E. PIERSON Educational Psychology Ball State University 2000 W. University Ave. Muncie, IN 47306 USA [email protected]

VICTOR R. PREEDY Nutritional Sciences Division King’s College London 150 Stamford Street London, SE1 9NH UK [email protected]

IRENE PIRYATINSKY Butler Hospital and Alpert Medical School of Brown University 345 Blackstone Blvd Providence, RI 2906 USA [email protected]

ANDREW PRESTON Department of Pediatrics Neurodevelopmental Center/ Memorial Hospital of Rhode Island and Warren Alpert Medical School of Brown University 555 Prospect Street Pawtucket, RI 2860 USA [email protected]

KENNETH PODELL Division of Neuropsychology Henry Ford Health Systems 1 Ford Place, Ste. 1 E Detroit, MI 48202-3450 USA [email protected] DONNA POLELLE Department of Commication Sciences and Disorders Saint Xavier University 3700 W 103rd Street Chicago, IL 60655 USA [email protected] MATTHEW R. POWELL Clinical Neuropsychologist Behavioral Medicine Center Waukesha Memorial Hospital, Neuroscience Center 721 American Avenue, Suite 501 Waukesha, WI 53188 USA [email protected] TIFFANY L. POWELL Department of Neurosurgery Virginia Commonwealth University Box 980631 Richmond, VA USA [email protected]

MICHELLE ANN PROSJE University of Florida 2036 NW 36th Street Gainesville, FL 32605 USA [email protected] ADELE S. RAADE Adjunct Assistant Professor Boston University Department of Speech Language, & Hearing Sciences 635 Commonwealth Avenue Boston, MA 2215 USA [email protected] VANESSA L. RAMOS Department of Psychology Nationwide Children’s Hospital 700 Children’s Drive Columbus, OH 43205 USA [email protected] KATE D. RANDALL Psychology Univesity of Victoria Victoria, BC Canada [email protected]

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STEVEN Z. RAPCSAK Neurology Service (1-27) Neurology Section, Southern Arizona VA Health Care System, Department of Neurology, University of Arizona 3601 S 6th Ave Tucson, AZ 85723 USA [email protected] SARAH A. RASKIN Department of Psychology and Neuroscience Program Trinity College Hartford, CT 6106 USA [email protected] JOSEPH F. RATH Rusk Institute of Rehabilitation Medicine NYU Langone Medical Center, Department of Psychology 400 East 34th Street New York, NY 10016 USA [email protected] HOLLY RAU Department of Psychology University of Utah Salt Lake City, UT 84112-0251 USA [email protected]

SHERYL REMINGER Psychology Department University of Illinois at Springfield Springfield, IL 62703 USA [email protected]

KATHRYN K. REVA University of Northern Colorado 51 W 69th Street Apt 4D New York, NY 10023 USA [email protected]

JOSE A. REY College of Pharmacy Nova Southeastern University 3200 South University Dr. Ft. Lauderdale, FL 33328 USA [email protected]

CECIL R. REYNOLDS Texas A&M Universuty 704 Harrington Tower College Station, TX 77843-4225 USA [email protected]

ANASTASIA RAYMER Professor of Early Childhood, Speech Pathology and Special Education Old Dominion University 110 Child Study Center Norfolk, VA 23529-0136 USA [email protected]

JILL B. RICH Department of Psychology York University 4700 Keele Street Toronto, ON M3J 1P3 Canada [email protected]

CHRISTINE REID Department of Rehabilitation Counseling Virginia Commonwealth University P.O. Box 980330 Richmond, VA 23298 USA [email protected]

ROBERT RIDER Drexel University Department of Psychology PSA Building, 3141 Chestnut Street Philadelphia, PA 19104 USA [email protected]


GIULIA RIGHI Brown University Visual Neuroscience Laboratory Waterman Street Providence, RI 02903 USA [email protected]

CAROLE ROTH Otolaryngology Clinic, Speech Division Naval Medical Center 34520 Bob Wilson Drive San Diego, CA 92134-2200 USA [email protected]

DIANA L. ROBINS Department of Psychology Georgia State University Department of Psychology P.O. Box 5010 Atlanta, GA 30302-5010 USA [email protected]

ELLIOT J. ROTH Feinberg School of Medicine Physical Medicine and Rehabilitation Northwestern University 345 E. Superior Chicago, IL 60611 USA [email protected] [email protected]

DANIEL E. ROHE Mayo Clinic 200 First Street Southwest Rochester, MN 55905 USA [email protected] MARYELLEN ROMERO Assistant Professor of Psychiatry Department of Psychiatry and Neurology Tulane University Health Sciences Center 1440 Canal Street, TB-53 New Orleans, LA 70112 USA [email protected] KATHERINE A. ROOF Department of Psychology University of North Carolina at Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected] JON ROSE Spinal Cord Injury Clinic Veterans Affairs Palo Alto Healthcare System 3801 Miranda Ave. (128) Palo Alto, CA 94304 USA [email protected]

LINDA ROWLEY Waisman Center Family Village University of Madison 1500 Highland Avenue Madison, WI 53705-2280 USA [email protected] DONALD ROYALL The University of Texas Health Center at San Antonio 7703 Floyd Curl Dr, Mail Code 7792 San Antonio, TX 78229-3900 USA [email protected] SHAHAL ROZENBLATT Advanced Psychological Assessment 50 Karl Avenue, Suite 104 P. C. Smithtown, NY 11787 USA [email protected] ALEXANDRA RUDD-BARNARD Rusk Institute of Rehabilitative Medicine Psychology New York University Langone Medical Center Service Psych InPat 550 First Avenue New York, NY 10016 USA [email protected]

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RONALD RUFF San Francisco Clinical Neurosciences & University of California San Francisco San Francisco Clinical Neurosci 909 Hyde Street, #620 San Francisco, CA 94109 USA [email protected]

JESSICA SOMERVILLE RUFFOLO Neuropsychology Clinic The Miriam Hospital The Coro Center, Suite 317 Providence, RI 2903 USA [email protected]

BETH RUSH Psychiatry and Psychology Mayo Clinic Davis 4-N, 4500 San Pablo Road Jacksonville, FL 32224 USA [email protected]

MICHELE RUSIN Emory University/Rehabilitation Medicine 1776 Briarcliff Road NE Atlanta, GA 30306 USA [email protected]

CATHY RYDELL American Academy of Neurology 1080 Montreal Avenue Saint Paul, MN 55116 USA [email protected]

BONNIE C. SACHS Department of Psychology & Psychiatry Mayo Clinic College of Medicine 4500 San Pablo Road Jacksonville, FL 32224 USA [email protected]

AMANDA L. SACKS Department of Rehab Medicine Mount Sinai Medical Center 5 East 98th Street New York, NY 10029 USA [email protected]

DONALD H. SAKLOFSKE Division of Applied Psychology Faculty of Education, University of Calgary 2500 University Drive NW Calgary, AB T2N 1N4 Canada [email protected]

JULIA RUTENBERG Emory University/Rehabilitation Medicine Atlanta VAMC RR&D CoE Atlanta, GA USA [email protected]

STEPHEN P. SALLOWAY Butler Hospital Alpert Medical School of Brown University 345 Blackstone Blvd Providence, RI 2906 USA [email protected]

BRUCE RYBARCZYK Department of Psychology Virginia Commonwealth University Box 842018 Richmond, VA 23284-2018 USA [email protected]

JEFFREY SAMUELS North Broward Medical Center Inpatient Rehabilitation Unit 1 West Sample Road Deerfield Beach, FL 33064 USA [email protected]


MARK A. SANDBERG Independent Practice Community Re-entry Program St. Charles Hospital 50 Karl Ave., Suite 104 Smithtown, NY 11787 USA [email protected] MARLA SANZONE Independent Practice, Loyola College of Maryland 104-A Annapolis Street Annapolis, MD 21401 USA [email protected] LYNN A. SCHAEFER Department of Physical Medicine and Rehabilitation Nassau University Medical Center 2201 Hempstead Turnpike East Meadow, NY 11554 USA [email protected] GERTINA J. VAN SCHALKWYK Department of Psychology Faculty of Social Sciences & Humanities (FSH), University of Macau Av. Padre Tomas Pereira Taipa, Macau SAR China [email protected] PHILIP SCHATZ Saint Joseph’s University Department of Psychology Post Hall #222 Philadelphia, PA 19131 USA [email protected] MIKE R. SCHOENBERG Associate Professor Department of Psychiatry and Behavioral Sciences, University of South Florida College of Medicine 3515 E. Fletcher Ave Tampa, FL 33613 USA [email protected]

AARON SCHRADER Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected] JILLIAN SCHUH Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected] CHRISTIAN SCHUTTE John D. Dingell VA Medical Center Psychology Section (11MHPS) 4646 John R Detroit, MI 48201-1916 USA [email protected] KERRI SCORPIO Neuropsychology Program Queens College and The Graduate Center of the City University of New York 6530 Kissena Blvd. Flushing, NY 11367 USA [email protected] DANIEL L. SEGAL Department of Psychology University of Colorado at Colorado Springs 1420 Austin Bluffs Parkway Colorado Springs, CO 80933 USA [email protected] ROBIN SEKERAK Waikato District Health Board PB 3200 Hamilton 2100 New Zealand [email protected]

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SVETLANA SEROVA Neuropsychology Postdoctoral Fellow Department of Rehabilitation Medicine, Mount Sinai School of Medicine One Gustave L. Levy Place, Box 1240 New York, NY 10029 USA [email protected]

LAURA SHANK Rehabilitation Psychology and Neuropsychology Physical Medicine & Rehabilitation University of Michigan 325 E. Eisenhower Parkway Ann Arbor, MI 48108 USA [email protected]

CASEY R. SHANNON University of Northern Colorado Greeley, CO USA [email protected]

ANUJ SHARMA Virginia Commonwealth University School of Medicine Richmond, VA USA [email protected]

SALLY E. SHAYWITZ Department of Pediatrics Yale University School of Medicine P.O. Box 208064 New Haven, CT 6520 USA [email protected]

BENNETT A. SHAYWITZ Yale University School of Medicine P.O. Box 208064 New Haven, CT 6520 USA [email protected]

VICTORIA SHEA Division TEACCH Carolina Institute on Developmental Disabilities University of North Carolina at Chapel Hill Chapel Hill, NC USA [email protected]

JUDITH A. SHECHTER 100 East Lancaster Avenue Suite 564 East Wynnewood, PA 19096 USA [email protected]

TAMARA GOLDMAN SHER Institute of Psychology Illinois Institute of Technology 3105 S. Dearborn St. Chicago, IL 60616 USA [email protected]

ELISABETH M. S. SHERMAN Alberta Children’s Hospital University of Calgary Calgary, AB Canada [email protected]

CHERYL L. SHIGAKI Department of Health Psychology University of Missouri One Hospital Drive, DC046.46 Columbia, MO 65212 USA [email protected]

GERALD SHOWALTER Department of Psychiatry and Neurobehavioral Sciences University of Virginia School of Medicine Charlottesville, VA 22908-0203 USA [email protected]


SEEMA SHROFF Anatomy & Neurobiology Virginia Commonwealth University Box 980709 Richmond, VA USA [email protected]

DAVID H. KEUNG SHUM Griffith University School of Psychology, Mt Gravatt Campus Griffith University Nathan Brisbane, Queensland 4111 Australia [email protected]

LINDA SHUSTER West Virginia University P.O. Box 6122 Morgantown, WV 26506 USA [email protected]

SUE ANN SISTO School of Health Technology and Management Stony Brook University 1500 Stony Brook Road Stony Brook, NY 11794-6018 USA [email protected]

BETH SLOMINE 707 North Broadway Baltimore, MD 21205 USA [email protected]

AUDREY SMERBECK School and Educational Psychology University at Buffalo The State University of New York Buffalo, NY 14260-1000 USA [email protected]

MARIAN L. SMITH Via Christi Hospital Pittsburg Mt. Carmel Via Christi Behavioral Health Crossroads Counseling Center 200 E. Centennial Avenue, suite 13 Pittsburg, KS 66762 USA [email protected]

JILL SNYDER Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected]

MCKAY MOORE SOHLBERG Communication Disorders and Sciences University of Oregon 5284 University of Oregon Eugene, OR 97403 USA [email protected]

SARA S. SPARROW 94, Linsley Lake Road North Branford, CT 6171 USA and Yale University Child Study Center 230 South Frontage Road New Haven, CT 06471 USA [email protected]

FERRINNE SPECTOR Psychology McMaster University 1280 Main Street West Hamilton, ON L8S4K1 Canada [email protected]

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APRIL SPIVACK Department of Psychology University of North Carolina - Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected]

ANTHONY Y. STRINGER Department of Rehabilitation Medicine Emory University 1441 Clifton Road NE Atlanta, GA 30322 USA [email protected]

BETH SPRINGATE Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected]

RENEÉ STUCKY Health Psychology PM&R Rusk Rehab Center Columbia, MO 65211 USA [email protected]

SUSAN STEFFANI CCC-SLP California State University, Chico, Department of Communication Sciences and Disorders 400 West 1st Street Chico, CA 95929-330 USA [email protected]

TARYN M. STEJSKAL Department of Physical Medicine and Rehabilitation Virginia Commonwealth University Medical Center Virginia, VA USA [email protected]

LAUREN STUTTS Department of Clinical and Health Psychology University of Florida Gainesville, FL 32611 USA [email protected] YANA SUCHY Department of Psychology University of Utah 380 S. 1530 E., Rm. 502 Salt Lake City, UT 84112-0251 USA [email protected]

WILLIAM STIERS Johns Hopkins University School of Medicine 5601 Loch Raven Boulevard, Suite 406 Baltimore, MD 21239 USA [email protected]

JAMES F. SUMOWSKI Neuropsychology and Neuroscience Kessler Medical Rehabilitation Research and Education Center Kessler Foundation Research Center 1199 Pleasant Valley Way West Orange, NJ 7052 USA [email protected]

ESTHER STRAUSS Department of Psychology University of Victoria P.O. Box 3050 Victoria, BC V8W 3P5 Canada [email protected]

DONG SUN Department of Neurosurgery Virginia Commonwealth University Medical Center P.O. Box 980631 MCV Campus Richmond, VA 23298 USA [email protected]


ZOE¨ SWAINE Department of Clinical and Health Psychology University of Florida Gainesville, FL 32611 USA [email protected] JOAN SWEARER Department of Neurology University of Massachusetts Medical School 55 Lake Avenue North Worcester, MA 01655 USA [email protected] LAWRENCE H. SWEET Department of Psychiatry and Human Behavior Brown University, Butler Hospital 345 Blackstone Blvd. Providence, RI 2906 USA [email protected] RUSSELL H. SWERDLOW University of Kansas School of Medicine Landon Center on Aging, MS 2012 3901 Rainbow Blvd Kansas City, KS 66160 USA [email protected] MICHAEL J. TARR Department of Cognitive and Linguistic Sciences and Brain Science Program Brown University Waterman Street Providence, RI 2903 USA [email protected] ELLA B. TEAGUE Neuropsychology program Queens College and The Graduate Center The City University of New York 65-30 Kissena Blvd Flushing, NY 11367 USA [email protected]

RICHARD TEMPLE Clinical Operations CORE Health Care 400 US Hwy 290 West, Bldg B Ste. 205 Dripping Springs, TX 78620 USA [email protected] CLAIRE THOMAS-DUCKWITZ University of Northern Colorado 1040 Blue Spruce Drive Greeley, CO 80538 USA [email protected] JENNIFER TINKER Department of Psychology Drexel University 3315 Market Street, 14-308 Philadelphia, PA 19104 USA [email protected] MICHELLE M. TIPTON-BURTON Physical Medicine and Rehabilitation Santa Clara Valley Medical Center 751 South Bascom Avenue San Jose, CA 95128 USA [email protected] JEFFREY B. TITUS Pediatric Neuropsychologist Washington University School of Medicine St. Louis Children’s Hospital One Children’s Place, 3S-32 St. Louis, MO 63110 USA [email protected] TERI A. TODD Department of Kinesiology California State University, Chico 400 West 1st Street Chico, CA 95929-330 USA [email protected]

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ALEXANDER I. TRO¨STER Department of Neurology (CB 7025) University of North Carolina School Medicine 3114 Bioinformatics Building Chapel Hill, NC 27599-7025 USA [email protected] NAM TRAN Neurosurgery Virginia Commonwealth University Medical Center Box 980631 Richmond, VA USA [email protected] KATHERINE TREIBER Santa Clara Valley Medical Center Utah State University Logan, UT 84322 USA and University of Massachusetts Medical School Worcester, MA USA [email protected] ANGELA K. TROYER Division of Psychology Baycrest Centre for Geriatric Care 3560 Bathurst Street Toronto, ON M6A 2E1 Canada [email protected]

LYN TURKSTRA University of Wisconsin-Madison 7225 Medical Sciences Center 1300 University Avenue Madison, WI 53706-1532 USA [email protected]

GARY TYE Neurosurgery Virginia Commonwealth University Box 980631 Richmond, VA USA [email protected]

KATHERINE TYSON Department of Psychology University of Connecticut 406 Babbidge Road, Unit 1020 Storrs, CT 6269 USA [email protected]

JAMIE VANNICE Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected]

THEODORE TSAOUSIDES Department of Rehab Medicine Brain Injury Research Mount Sinai School of Medicine 5 East 98th Street, Room B-15 New york, NY 10029 USA [email protected]

TODD VAN WIEREN Disability Support Services Indiana University of Pennsylvania Indiana, PA USA [email protected]

JOANN T. TSCHANZ Utah State University Center for Epidemiologic Studies 4450 Old Main Hill Logan, UT 84322-4440 USA [email protected]

REBECCA VAURIO Kennedy Krieger Institute 707 North Broadway Baltimore, MD 21205 USA [email protected]


JENNIFER VENEGAS Department of Educational Psychology University of Utah 1705 Campus Center Drive, #327 Salt Lake City, UT 84112-9255 USA [email protected] MIEKE VERFAELLIE Memory Disorders Research Center 151A VA Boston Healthcare System and Bosten University School of Medicine 150 South Huntington Ave Boston, MA 2130 USA [email protected]

FRED R. VOLKMAR Yale University 230 South Frontage Road New Haven, CT 06520-7900 USA [email protected] SCOTT VOTA Neurology Virginia Commonwealth University Box 980599 Richmond, VA USA [email protected]

JEAN VETTEL Brown University Campus Box 1978 Providence, RI 2912 USA [email protected]

GEORGE C. WAGNER Department of Psychology Rutgers University 152 Freylinghuysen Road Piscataway New Brunswick, NJ 8854 USA [email protected]

CHAD D. VICKERY Neuropsychology Department Methodist Rehabilitation Center 1350 E. Woodrow Wilson Jackson, MS 39216 USA [email protected]

CHRISTOPHER WAGNER Department of Rehabilitation Counseling Virginia Commonwealth University P.O. Box 980330 Richmond, VA 23298 USA [email protected]

MICHAEL R. VILLANUEVA Department of Psychology University of North Carolina-Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected]

NATALIE WAHMHOFF Department of Educational Psychology University of Utah 1705 Campus Center Drive, #327 Salt Lake City, UT 84112-9255 USA [email protected]

MARTIN A. VOLKER School and Educational Psychology University at Buffalo The State University of New York Buffalo, NY 14260-1000 USA [email protected]

JULIE L. WAMBAUGH Veterans Affairs Salt Lake City Healthcare System and University of Utah 151 A 500 Foothill Blvd. Salt Lake City, UT 84148 USA [email protected]

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SETH WARSCHAUSKY Department of Physical Medicine and Rehab University of Michigan 325 East Eisenhower Ann Arbor, MI 48108 USA [email protected] ADAM B. WARSHOWSKY Clinical Neuropsychologist, Dual/SCI Unit Mount Sinai Medical Center, Shepherd Center 2020 Peachtree Road Atlanta, GA 30309 USA [email protected] AMANDA WAXMAN Neuropsychology Program Queens College of City University of New York 564 Amsterdam Avenue Apt. 3C New York, NY 10024 USA [email protected] NADIA WEBB Department of Psychology Children’s Hospital of New Orleans 200 Henry Clay Avenue New Orleans, LA 70118 USA [email protected] CHRISTINE J. WEBER-MIHAILA Neuropsychologist Northeast Regional Epilepsy Group 104 East 40th Street, Suite 607 New York, NY 10016 USA [email protected] [email protected] STEPHEN T. WEGENER Division of Rehabilitation Psychology and Neuropsychology Department of Physical Medicine and Rehabilitation The Johns Hopkins School of Medicine 600 North Wolfe Street, Phipps 174 Baltimore, MD 21287 USA [email protected]

JOHN D. WESTBROOK National Center for the Dissemination of Disability Research (NCDDR) SEDL 4700 Mueller Blvd. Austin, TX 78723 USA [email protected] MICHAEL WESTERVELD Medical Psychology Associates Florida Hospital 5165 Adanson Street, Suite 200 Orlando, FL 32804 USA [email protected] HOLLY JAMES WESTERVELT Clinical Neuropsychologist Neuropsychology Program Rhode Island Hospital Alpert Medical School of Brown University 593 Eddy Street, POB 430 Providence, RI 2903 USA [email protected] MARNIE J. WESTON Center for Health Care Quality University of Missouri-Columbia One Hospital Drive Columbia, MO 65212 USA [email protected] KRISTINE B. WHIGHAM Licensed Psychologist Department of Neuropsychology Children’s Healthcare of Atlanta 1001 Johnson Ferry Road, NE Atlanta, GA 30342 USA [email protected] GALE G. WHITENECK Craig Hospital 3425 S. Clarkson Street Englewood, CO 80113 USA [email protected]


JOHN WHYTE Department of Rehabilitation Medicine Thomas Jefferson University Moss Rehabilitation Research Institute Albert Einstein Healthcare Network 60 E. Township Line Road Elkins Park PA 19027 USA [email protected] ROBERT G. WILL University of Edinburgh Edinburgh UK [email protected] GAVIN WILLIAMS Senior Physiotherapist Epworth Rehabilitation Centre Epworth Hospital 29 Erin Street Richmond Melbourne, Vic 3121 Australia [email protected] BRENDA WILSON Department of Communication Disorders and Sciences Eastern Illinois University 600 Lincoln Ave Charleston, IL 61920-3099 USA [email protected] JILL WINEGARDNER Northern California Programs Learning Services 10855 DeBruin Way Gilroy, CA 95020 USA and Princess of Wales Hospital Ely, Cambridgeshire UK [email protected] DEBORAH WITSKEN University of Minnesota Medical School 2020 Garfield Ave, Apt. 7 Minneapolis, MN 55405 USA

and University of North Colorado Greeley, CO USA [email protected] ERICKA WODKA Center for Autism and Related Disorders Kennedy Krieger Institute 3901 Greenspring Avenue Baltimore, MD 21211 USA [email protected] THOMAS R. WODUSHEK Center for Neurorehabilitation Services, PC 1045 Robertson Street Fort Collins, CO 80524-3926 USA [email protected] JENNIFER WOEHR Department of Neurology Mount Sinai School of Medicine One Gustave L. Levy Place, Box 1139 New York, NY 10029 USA [email protected] EDISON WONG Center for Pain and Medical Rehab 33 Electric Avenue, Suite B03 Fitchburg MA 01420 USA [email protected] MICHAEL S. WORDEN Department of Neuroscience Brown University 185 Meeting Street Box G-LN Providence, RI 2912 USA [email protected] JERRY WRIGHT Rehabilitation Research Center Santa Clara Valley Medical Center 751 S. Bascom Avenue San Jose, CA 95128 USA [email protected]

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FAN WU Department of Psychology Faculty of Social Sciences and Humanities University of Macau Av. Padre Tomas Pereira Taipa, Macau SAR China [email protected] GLENN WYLIE Neuropsychology and Neuroscience Laboratory Kessler Medical Rehabilitation Research and Education Center Kessler Foundation 1199 Pleasant Valley Way West Orange, NJ 7052 USA [email protected] KEITH O. YEATES Department of Psychology Nationwide Children’s Hospital 700 Children’s Drive Columbus, OH 43205 USA [email protected] ANGELA YI Department of Rehab Medicine Mount Sinai School of Medicine 5 East 98th Street New York, NY 10029 USA [email protected]

OSBORN H. ZACHARY Behavioural Health Service Line Harry S. Truman Memorial Veteran’s Hospital Columbia, MO 65201 USA [email protected]

CHRISTINA ZAFIRIS Applied Psychology and Counselor Education University of Northern Colorado McKee 248, Box 131 Greeley, CO 80631 USA [email protected]

ROSS ZAFONTE Spaulding rehabilitation Hospital Harvard Medical School 125 Nashua Street Boston, MA 2114 USA [email protected]

NATHAN D. ZASLER Concussion Care Centre of Virginia, Ltd. 3721 Westerre Parkway, Suite B Richmond, Virginia 23233 USA [email protected]

BRIAN YOCHIM Department of Psychology University of Colorado at Colorado Springs 1420 Austin Bluffs Parkway Colorado Springs, CO 80933 USA [email protected]

ISLAM ZAYDAN Neurology Virginia Commonwealth University Box 980599 Richmond, VA USA [email protected]

MICHELE L. ZACCARIO Rusk Institute New York University Langone Medical Center Pace University 339 East 28th Street New York, NY 10016 USA [email protected]

FADEL ZEIDAN Department of Psychology UNC Charlotte 9201 University City Blvd Charlotte, NC 28223 USA [email protected]


DENNIS J. ZGALJARDIC Department of Neuropsychology Transitional Learning Center at Galveston 1528, Postoffice Street Galveston, TX 77550 USA [email protected]

MIRIAM ZICHLIN Aging and Dementia Research Center NYU School of Medicine 550 First Ave. MHL 310 New York, NY 10016 USA [email protected]

ZHENG ZHOU Department of Psychology St. John’s University Queens, NY 11439 USA [email protected]

MOLLY E. ZIMMERMAN Albert Einstein College of Medicine 1165 Morris Park Ave Rousso Bldg Rm 310 Bronx, NY 10461 USA [email protected]

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A 2 2 Table ▶ Contingency Table

2 & 7 Test ▶ Ruff 2&7 Selective Attention Test

3MS ▶ Modified Mini-Mental State Examination

7-Item BBS-3P ▶ Berg Balance Scale

15 Item Test ▶ Rey 15 Item Test

504 Plan M. J. H OLCOMB 1, DAVID E. M ACINTOSH 2 1 Ball State University Muncie, IN, USA 2 Ball State University Muncie, IN, USA

5-HTP ▶ L-Tryptophan

5-Hydroxytryptophan ▶ L-Tryptophan

6MWD ▶ Six-Minute Walk Test

6MWT ▶ Six-Minute Walk Test

Definition A 504 Plan refers to Section 504 of the Rehabilitation Act of 1973 (Public Law 93-112) and the Americans with Disabilities Act of 1990 (Public Law 101-336), which makes it illegal to exclude anyone from a federally funded program or activity based on a disability. Section 504, a federal civil rights law, specifically prohibits discrimination against individuals with disabilities, within any school system or other recipient of federal financial assistance. The definition of recipient is a broad one, as it can include not only schools but also states (including their Departments of Education) or counties, agencies, institutions, or other organizations that benefit from Federal funds, directly or indirectly.

Current Knowledge A 504 plan documents accommodations for qualified students which will allow them to have opportunities similar

Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan (eds.), Encyclopedia of Clinical Neuropsychology, DOI 10.1007/978-0-387-79948-3, # Springer Science+Business Media LLC 2011

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AACN Practice Guidelines

to those of their peers. An Individualized Education Plan (IEP) is not a 504 plan because IEPs only cover an inclusive list of students with disabilities. A 504 plan covers a far wider range of conditions, including both those that actually limit one or more major life activities (the criterion for disability under IDEA) and those that do not limit a major life activity but are perceived as limiting by the recipient of funding. Thus, individuals who are not eligible for special education services under IDEA may nonetheless be eligible for accommodations under Section 504. While both laws require provision of a free appropriate public education, a comprehensive evaluation is not required to obtain services under the provisions of Section 504. While IDEA provides for comprehensive evaluation at the expense of the school district, this is not the case for services requested under Section 504. In sum, the purpose of 504 legislation is to level the playing field for those who don’t require the significant level of accommodation and/or assistance needed by those who meet criteria for an IEP under IDEA. Examples of conditions that may qualify for 504 services include asthma, diabetes, eating disorders, ADHD, depression, and conduct disorder.

Cross References ▶ Accommodations ▶ Americans with Disabilities Act (1990) ▶ IDEA ▶ Rehabilitation Act of 1973

References and Readings Smith, T. E. C., & Patton, J. R. (1998). Section 504 and the public schools. Austin: TXL Pro-Ed.

AACN Practice Guidelines R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Practice development; Practice guidelines

Historical Background The American Board of Clinical Neuropsychology (ABCN) is a specialty board within the American Board of Professional Psychology (ABPP). For those seeking board certification in clinical neuropsychology, ABCN is the board responsible for overseeing the examination process. The American Academy of Clinical Neuropsychology (AACN) is the organization for those awarded board certification by the ABCN. In 2007, AACN produced the first set of practice guidelines, which were intended to ‘‘. . .facilitate the continued systematic growth of the profession of clinical neuropsychology, and to help assure a high level of professional practice.’’

Current Knowledge Given the recent growth of clinical neuropsychology, coupled with the American Psychological Association’s focus on Evidence-Based Practice, the AACN established (AACN, 2007) guidelines for the practice of neuropsychological assessment and consultation. The guidelines are intended to provide standards for competence and professional conduct within the practice of neuropsychology by describing the ‘‘most desirable and highest level of professional conduct’’ for clinical neuropsychologists providing clinical neuropsychology services. It is important to note that the guidelines are fully compatible with the current APA (2002) Ethical Principles of Psychologists and Code of Conduct (EPPCC) as well as the Criteria for Practice Guideline Development and Evaluation (2002) and Determination and Documentation of the Need for Practice Guidelines (2005). The AACN practice guidelines include recommendations for the practice of clinical neuropsychology and they are not to be regarded as mandatory standards. The guidelines detail consideration of ethical and clinical issues as well as specific methods and procedures for the practice of neuropsychology. There are several major areas of emphasis in the guidelines. They include: (1) Definitions; (2) purpose and scope; (3) education and training; (4) work settings; (5) ethical and clinical issues (e.g., informed consent, patient issues in third party assessments, test security; underserved populations/cultural issues; and (6) methods and procedures (e.g., review of records, measurement procedures, test administration and scoring, and interpretation).

AAMD Adaptive Behavior Scales

References and Readings American Psychological Association. (2002). Criteria for practice guideline development and evaluation. American Psychologist, 57, 1048–1051. American Psychological Association. (2002) Ethical principles of psychologists and code of conduct. American Psychologist, 57, 1060–1073. American Psychological Association. (2005). Determination and documentation of the need for practice guidelines. American Psychologist, 60, 976–978. Committee on Ethical Guidelines for Forensic Psychologists. (1991). Specialty guidelines for forensic psychologists. Law and Human Behavior, 15, 655–665. The AACN practice guidelines can be found on the AACN’s Web site (www.theaacn.org) and are also published in the AACN’s journal: The Clinical Neuropsychologist, 21, 209–231.

AAMD ABS: 2 ▶ AAMD Adaptive Behavior Scales

AAMD Adaptive Behavior Scales C RISTA A. H OPP 1, I DA S UE B ARON 2 1 Inova Fairfax Hospital for Children 2 Inova Fairfax Hospital for Children Falls Church, VA, USA

Synonyms AAMD ABS: 2; AAMR ABS-RC: 2; AAMR ABS-S: 2

Description The American Association for Mental Deficiency Adaptive Behavior Scales (AAMD ABS) is a revised edition (1993) of the original assessments that were published in 1969. The American Association for Mental Retardation (AAMR) (formerly known as the American Association for Mental Deficiency) has changed its name to American Association on Intellectual and Developmental Disabilities (AAIDD). Therefore, intellectual disabilities have replaced mental retardation as the terminology of choice. The behavior scales have been published in two versions, the Adaptive Behavior Scales-Residential and Community,

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2nd edition (ABS-RC: 2) and the Adaptive Behavior Scales-School, 2nd edition (ABS-S: 2). Current versions are a comprehensive compilation of the past versions. These assessments seek to develop an estimate of adaptive behaviors in two scales defined with personal independence and maladaptive behaviors in individuals with intellectual disabilities. Items are rated with a yes/no response, on a 0–3 scale, or by frequency. Historically, the ABS-RC: 2 was used in institutions, but it is now also used in community settings, whereas the ABS-S: 2 was designed for use in school settings. For both the ABS-RC: 2 and the ABS-S: 2, the assessment can be administered by either of two approaches. In one method, the assessment is completed by a professional or paraprofessional trained to use the scales. In the second method, the assessment is administered by someone familiar with the individual being evaluated. Interpretation of results must be completed by an individual with formal training in psychometrics and these scales. The ABS-S: 2 enables an appraisal of an individual’s ability to cope with challenges they encounter in their school, and aids in the diagnosis of intellectual disabilities at ages 3–21. There are nine subscales in the first part of the assessment, measuring personal independence and responsibility of daily living: independent functioning, physical development, economic activity, language development, numbers and time, prevocational/vocational activity, self-direction, responsibility, and socialization. The second part of the assessment, which addresses behavioral domains, consists of seven subscales: social behavior, conformity, trustworthiness, stereotyped and hyperactive behavior, self-abusive behavior, social engagement, and disturbing interpersonal behavior. The ABS-S: 2 was normed on 2,074 students with intellectual disabilities and 1,254 of their peers without intellectual disabilities. Administration takes place in an interview format with either a parent or teacher and may vary from 20 min to 2 h, dependent on the rater. Scoring is completed by hand. Raw scores are converted into percentiles, standard scores, and age equivalents for each subdomain. Five factors can be derived: Personal selfsufficiency, community self-sufficiency, personal social responsibility, social adjustment, and personal adjustment. Percentiles, factor standard scores, and age equivalents are then reported based on factor scores. The ABS-RC: 2 is also useful for the assessment of personal development and social behavior in individuals with intellectual disabilities, but it has been developed for individuals aged 18–79. Like the ABS-S: 2, the assessment has two parts, but there are more subscales in each part.

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AAMD Adaptive Behavior Scales

The first part has ten subscales: independent functioning, physical development, economic activity, language development, numbers and time, domestic activity, prevocational/ vocational activity, self-direction, responsibility, and socialization. The second part contains eight subscales: social behavior, conformity, trustworthiness, stereotyped and hyperactive behavior, sexual behavior, self-abusive behavior, social engagement, and disturbing interpersonal behavior. The ABS-RC: 2 was normed on a sample of 4,000 adults with intellectual disabilities, and administration times vary between 15 and 40 min, depending on the informant’s knowledge of the individual being assessed. Raw scores are recorded and then converted to standard scores and percentiles. The subscales yield the same five-factor scales as the ABS-S: 2.

Historical Background The AAMD first published the ABS in 1969 in response to the definition of mental retardation that was enlarged in 1959 to include adaptive behavior. The ABS-S: 2, first published in 1969 by Nihira, Foster, Shellhaas, and Leland, was revised and standardized in 1974 by Lambert, Windmiller, and Cole and again in 1981 by Lambert and Windmiller. The second and current edition was published in 1993. The ABS-RC:2 were also first published in 1969 by Nihira, Foster, Shellhaas, and Leland. It was revised in 1974, and again in 1993. The goals of the revisions have been to improve the reliability of the interviewer in differentiating between individuals with intellectual disabilities who are institutionalized and those living in the community. Previously, these individuals had been classified at different adaptive behavior levels according to the AAIDD.

adaptive behavior as measured in Part II was not related to the Vineland Adaptive Behavior Scale and Adaptive Behavior Inventory (ABI), other measures of maladaptive behaviors.

Clinical Uses The ABS: 2 assesses the status of individuals with intellectual disability, emotional maladjustment, autism, or developmental disability. It enables a professional to assess strengths and weaknesses of an individual in adaptive areas, document progress, and assess the effectiveness of intervention/school programs. The manual cautions that the examiner should interview a significant informant or the instrument should be administered by that significant informant. If an informant is unable to provide needed information, then another informant needs to be interviewed. Whereas the ABS is a standard assessment used in determining adaptive and maladaptive behavior, its psychometric properties are limited, especially compared to other measures such as the Vineland Adaptive Behavior Scales. Whereas a strength of the ABS-S: 2 is that it was normed on students with and without intellectual disabilities, the ABS-RC: 2’s standard scores and percentile ranks were not compared to individuals without intellectual disabilities. Therefore, this assessment may not meet the criteria to make a diagnosis of mental retardation according to the AAMR requirements.

Cross References ▶ Vineland Adaptive Behavior Scales

Psychometric Data References and Readings The authors of the ABS-S: 2 report three types of reliability: internal consistency, stability, and interscorer. Internal consistency is reported to range from 0.79 to 0.98, while measures of stability range from 0.82 to 0.97. For Part I, interscorer reliability ranges from 0.95 to 0.98 whereas it is 0.96 to 0.99 for Part II. Authors report criterion validity in Part 1 moderately correlated with the ABS and the Vineland Adaptive Behavior Scales, although Part II was not significantly related to either (Lyman, 2007). The ABS-RC: 2 reports an internal consistency ranging from 0.81 to 0.97. Concerning discriminant validity,

Aiken, L. (1996). Assessment of intellectual functioning. Switzerland: Burkhauser. Bracken, B., & Nagle, R. (2007). Psychoeducational assessment of preschool children. New York: Routledge. Hogg, J., & Langa, A. (2005). Assessing Adults with Intellectual Disabilities. Malden, MA: Blackwell. Lyman, W. (2008). Test review In N. Lambert, K. Nihira, & H. Lel (1993). AAMR Adaptive behavior scales: school. Assessment for Affective Intervention, 33, 55–57. Reynolds, C., & Fletcher-Janzen, E. (2007). Encyclopedia of special education, a reference for the education of children, adolescents, and adults with disabilities and other exceptional individuals (3rd ed., Vol. 1). Hoboken, NJ: Wiley.

Abbreviated Injury Scale

AAMR ABS-RC: 2 ▶ AAMD Adaptive Behavior Scales

AAMR ABS-S: 2 ▶ AAMD Adaptive Behavior Scales

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Abbreviated Injury Scale E DISON WONG Center for Pain & Medical Rehab Fitchburg, MA, USA

Synonyms Organ injury scale

Definition

ABAS ▶ Adaptive Behavior Assessment System – Second Edition

Abasia D OUGLAS I. K ATZ Braintree Rehabiltation Hospital Braintree, MA, USA Boston University School of Medicine Boston, MA, USA

The Abbreviated Injury Scale (AIS) is an anatomical scoring system first introduced in 1969. It has been revised and updated against survival data so that it now provides a reasonably accurate way of ranking the severity of injury. Injuries are ranked on a scale of 1–6, with 1 being minor, 5 severe, and 6 an unsurvivable injury (Table 1). This represents the ‘‘threat to life’’ associated with an injury and is not meant to represent a comprehensive measure of severity. The AIS is not a linear scale, in that the difference between AIS1 and AIS2 is not the same as that between AIS4 and AIS5. Organ Injury Scales of the American Association for the Surgery of Trauma are mapped to the AIS score for calculation of the Injury Severity Score.

Definition Current Knowledge This refers to an inability to walk. Abasia may be caused by a variety of conditions including weakness, spasticity, cerebellar incoordination, and movement disorders of various types.

Cross References ▶ Ataxia ▶ Spastic Gait

ABAS-II ▶ Adaptive Behavior Assessment System – Second Edition

The latest incarnation of the AIS score is the 2005 revision. AIS is monitored by a scaling committee of the Association for the Advancement of Automotive

Abbreviated Injury Scale. Table 1 AIS scores and their definition of injury severity AIS Score

Injury

1

Minor

2

Moderate

3

Serious

4

Severe

5

Critical

6

Unsurvivable

5

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6

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Ability Focused

Medicine and has been adopted by the American Association for the Surgery of Trauma since its publication in the Journal of Trauma in 1985.

ablation is too destructive to neighboring tissues. Even with sophisticated neurosurgical techniques, ablation of any type in the nervous system may still produce unwanted motor, sensory, or cognitive-behavioral impairments.

References and Readings

Cross References

Copes, W. S., Sacco, W. J., Champion, H. R., & Bain, L. W. (1989). Progress in characterizing anatomic injury. Proceedings of the 33rd Annual Meeting of the Association for the Advancement of Automotive Medicine, pp. 205–218. Greenspan, L., McClellan, B. A., & Greig, H. (1985). Abbreviated injury scale and injury severity score: A scoring chart. The Journal of Trauma, 25, 60–64. Moore, E. E., Shackford, S. R., Pachter, H. L., McAninch, J. W. Browner, B. D., Champion, H. R., et al. (1989). Organ injury scaling: Spleen, liver, and kidney. The Journal of Trauma, 29, 1664–6. Yentis, S. M., Hirsch, N. P., & Smith, G. B. (2004). Anaesthesia and intensive care A-Z. New York: Butterworth & Heinemann.

▶ Commissurotomy ▶ Craniotomy ▶ Gamma Knife ▶ Hemispherectomy ▶ Lobectomy ▶ Lobotomy ▶ Pallidotomy ▶ Prefrontal Lobotomy ▶ Radiosurgery ▶ Temporal Lobectomy


Ability Focused ▶ Flexible Battery

Ablation E DISON WONG Center for Pain and Medical Rehab Fitchburg, MA, USA

Synonyms Resection

Krayenbuhl, H., Wyss, O. A., & Yasargil, M. G. (1961). Bilateral thalamotomy and pallidotomy as treatment for bilateral parkinsonism. Journal of Neurosurgery, 18, 429–444. Lord, S. M., & Bogduk, N. (2002). Radiofrequency procedures in chronic pain. Best Practice & Research. Clinical Anaesthesiology, 16, 597–617. Lunsford, L. D., Flickinger, J. C., & Steiner, L. (1988). The gamma knife. JAMA, 259, 2544. Shah, R. V., Ericksen, J. J., & Lacerte, M. (2003). Interventions in chronic pain management. 2. New frontiers: Invasive nonsurgical interventions. Archives Physical Medicine and Rehabilitation, 84, S39–44.

Abnormal Brain Growth ▶ Microcephaly

Definition Ablation is the removal or destruction of an anatomical structure by means of surgery, disease, or other physical or energetic process. Ablation is employed as a treatment of various medical conditions and includes recent advances in technology. Surgical ablation of neuronal pathways to the globus pallidus or thalamus has been used historically to treat parkinsonism. Interventional pain experts use radiofrequency ablation of nerves in the spine to treat chronic back pain. Gamma radiation or ‘‘gamma knife surgery’’ is used to excise brain tumors when traditional surgical

Abnormal Walking ▶ Gait Disorders

Aboulia ▶ Abulia

Absence Epilepsy

ABS ▶ Agitated Behavior Scale

Absence Epilepsy J EFFREY B. T ITUS 1,2 , R EBECCA K ANIVE 1 M ICHAEL M ORRISSEY 1 1 St. Louis Children’s Hospital St. Louis, MO, USA 2 Washington University School of Medicine St. Louis, MO, USA

Synonyms Petit mal epilepsy; Psychomotor seizures; Pyknoleptic petit mal (childhood absence epilepsy)

Definition Absence epilepsy is a form of idiopathic generalized epilepsy that is characterized by seizures that involve sudden arrest in activity, awareness, and responsiveness, and may include some mild motor features. Typical absence seizures usually last less than 10 s and end as abruptly as they start. Patients have no recollection of the event and often return immediately to their previous activity with little or no post-ictal alterations in functioning. Generalized spike-and-wave discharges on EEG are required for the diagnosis and are strongly correlated with the clinical events.

Categorization

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an underestimation. It has been suggested that JAE may be as common as juvenile myoclonic epilepsy (JME), though this has not been well-established. CAE is typically considered to be more common in females. CAE is associated with a strong family history of seizures. There is strong concordance among identical twins, and multiple genes likely account for transmission. Siblings of patients with CAE have about a 10% chance of having seizures, and about one-third of patients with CAE have a family member with epilepsy. Nevertheless, the causal influences of CAE are believed to be multifactorial, depending on both genetic and nongenetic factors. Causal factors in JAE have not been well-studied but may be similar to what is found in CAE.

Natural History, Prognostic Factors, Outcomes Typical age of onset in CAE is between 3 and 8 years, but rare cases of onset prior to 3 years of age have been reported. Onset of JAE is considered to be between 10 and 17 years. Because onset of CAE has been reported in cases as old as 10 or 11 years, there is clear overlap between CAE and JAE. EEG and clinical findings are often useful in differentiating CAE from JAE in older children and younger adolescents. It is unusual for a child to exhibit features of CAE after the age of 11 years. Outcomes in CAE and JAE are generally favorable. Most patients with CAE experience remission of seizures by mid-adolescence, with only a small proportion experiencing absence seizures into adulthood. About 40% of patients with CAE also exhibit generalized tonic–clonic seizures. They often emerge around the time of puberty, are relatively easy to control, and more commonly persist into adulthood than absence seizures. Tonic–clonic seizures Absence Epilepsy. Table 1 Clinical features of CAE and JAE CAE

JAE

Incidence

2–8% (of children with epilepsy)

Unknown

Age of onset

3–8 years

10–17 years

Epidemiology

Seizure frequency

Multiple per day

One or fewer per day

Incidence reports of absence epilepsy range from 49 to 98 per 100,000. Among children with epilepsy, 2–8% have been estimated to have CAE. The incidence of JAE has not been well-studied. Estimates suggest that JAE accounts for up to 20% of absence epilepsy cases; however, this may be

Response to treatment

Good

Good

Seizure freedom

Expected

Less common

Treatment duration

Through mid-adolescence

Often through adulthood

Childhood absence epilepsy (CAE). Juvenile absence epilepsy (JAE).

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Absence Epilepsy

are more common in JAE and occur in about 80–90% of cases. Some patients with JAE also exhibit myoclonic seizures, but they are typically mild and infrequent. While most patients with CAE become seizure-free in adolescence, seizure outcome in JAE is not well known. CAE is considered to be a benign childhood epilepsy because of relatively good seizure control and functional outcomes. Seizure control is less common in JAE, but functional outcomes may be similar. Further research is needed to examine this. Tonic–clonic seizures are believed to be a marker for poorer seizure outcome in both CAE and JAE. Functional outcomes in CAE are thought to be most heavily influenced by psychosocial factors, such as family adjustment, support systems, educational attitudes, and stigma toward the condition. Cognitive and/or behavioral side effects from antiepileptic drug (AED) therapy may also limit outcomes.

Neuropsychology and Psychology of Absence Epilepsy Cognitive functioning in CAE is traditionally considered ‘‘benign,’’ because children typically present with normal intelligence and exhibit no significant impairments in functional outcomes. However, more recent research has found evidence that patients with CAE are prone to having cognitive deficits and psychosocial problems, and they are more likely to receive special education services and display low academic achievement. While patients with poor seizure control exhibit the greatest difficulties, cognitive and behavioral problems are also experienced by patients with good seizure control. Unfortunately, limited information is known about cognitive and psychological functioning in JAE. Patients with CAE do not have a characteristic cognitive profile. Cognitive difficulties have been reported in multiple domains, including attention, memory, and visual-spatial processing. A recent study by Caplan et al. (2008) revealed the presence of subtle cognitive impairments in children with CAE. When compared with controls, they found that children with CAE (ages 6.7–11.2 years) had significantly lower intelligence, as measured by the Wechsler Intelligence Scale for Children – Revised/Third Edition. While, as a group, children with CAE performed in the average range, they were below the performance of a control group. Similar differences were noted on verbal and visual intellectual tasks. The difference in performance IQ (PIQ) was less robust, but still significant, between children with CAE and controls. Among their sample of 69 children with CAE, 27% demonstrated

overall intelligence at least one standard deviation below the mean. Similar rates were found for VIQ and PIQ. Their spoken language quotient (SLQ), as measured by various versions of the Test of Language Development, was average, but it was also lower than controls. A high percentage of children with CAE performed at least one standard deviation below the mean on language measures. In addition to finding a higher rate of cognitive limitations, Caplan et al. (2008) confirmed that children with CAE also experience emotional and behavioral comorbidities. Among the 69 children with CAE in their sample, 30% had a diagnosis of attention-deficit/hyperactivity disorder (ADHD), with 52% of those children diagnosed as ADHD-inattentive type. Moreover, about 29% of their samples were diagnosed with a form of internalizing psychopathology. Among those children, 75% were diagnosed with anxiety, 20% with depression, and 5% with both anxiety and depression. After controlling for IQ and demographic variables, children with CAE were found to have significantly higher ratings on scales of the Child Behavior Checklist (CBCL) that assess attention problems, somatic problems, social problems, withdrawal, and thought problems. The authors discovered that children with lower intelligence had greater social problems, and females in the CAE sample were almost six times more likely to be diagnosed with an anxiety disorder. In addition, children with CAE were more likely to be diagnosed with ADHD or anxiety if they had more frequent seizures or a longer duration of illness.

Evaluation Children and adolescents with CAE and JAE typically present with no focal neurological abnormalities on examination. The presence of absence seizures is a defining feature of absence epilepsy, and hyperventilation or light stimulation can be highly effective at eliciting an event. In CAE, absence seizures occur multiple times per day, but, in JAE, they are more rare and may only occur once per day. Absence seizures can be either typical or atypical, and discrimination between the two types is usually done off of EEG findings. While typical absence seizures are characterized by clearly delineated episodes of activity arrest and impaired consciousness for less than 10 s, atypical absence seizures are associated with less abrupt onset and termination, and they may more commonly involve various semiological phenomena. Atypical absence seizures often last for more than 10 s and cannot be elicited by hyperventilation or light stimulation. Tonic

Absence Seizure

seizures are also frequently present in children with atypical absence seizures. Typical absence seizures can be subdivided into simple and complex. Simple typical absence seizures constitute about 90% of cases and may involve only minor motor mannerisms (e.g., mild eyelid fluttering). Patients with complex typical absence seizures display more involvement of motor features, such as automatisms or decreased or increased muscle tone. Loss of consciousness may also be longer. Complex partial seizures can often mimic absence seizures, particularly when their expression is limited. Typical absence seizures can be distinguished from complex partial seizures because they are briefer, more frequent, and have no post-ictal impairment. EEG characteristics and the presence of various seizure types often distinguish atypical absence seizures from complex partial epilepsy. When considering the presence of absence seizures, it is important to consider whether the episodes can be accounted for by variations in attention. This is especially important when considering the high rate of attention problems in children with epilepsy. Attempting to determine the degree of responsiveness during the episodes often helps with making the differential diagnosis; however, this can be difficult to determine when episodes are very brief. Moreover, it is not uncommon for patients to have both absence seizures and attention problems. Therefore, a child’s ability to respond during an episode cannot be used to rule-out the presence of absence seizures. Sometimes a neuropsychological assessment can be helpful in differentiating between absence seizures and episodes of inattention. If the examiner has experience with absence seizures, the neuropsychological assessment can provide multiple hours of one-on-one observation and interaction that might provide opportunities to observe the episodes and attempt to elicit responses. This can also be helpful if mental fatigue tends to elicit more events. On EEG, absence seizures are characterized by paroxysmal bursts of high amplitude 3–4 Hz spike and slow waves that are superimposed on a normal background. The bursts vary in length (3–10 s), and the clinical absence is time-locked to the burst period. This activity (clinical and electrographic) can be provoked during a routine EEG recording using the hyperventilation activation procedure.

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Ethosuximide has also been recommended and may be more appropriate for younger patients. In rare cases of more difficulty in controlling seizures, polytherapy may be needed. In patients with CAE, a seizure-free period of 2 years is often recommended prior to discontinuation of therapy; however, this should be determined on a case-bycase basis. Patients with JAE will require longer treatment and may continue on AEDs indefinitely. In adolescent patients, it is important to educate about the increased risk of seizures with poor medication compliance, alcohol consumption, or sleep deprivation.

Cross References ▶ Petit Mal Seizure ▶ Juvenile Myoclonic Epilepsy (JME)

References and Readings Aicardi, J. (1998). Diseases of the nervous system in childhood. London: Mac Keith. Berkovic, S. F., & Benbadis, S. (2001). Childhood and juvenile absence epilepsy. In E. Wyllie (Ed.), The treatment of epilepsy: Principles and practice (3rd ed. pp. 485–490). Philadelphia, PA: Lippincott Williams & Wilkins. Caplan, R., Siddarth, P., Stahl, L., Lanphier, E., Vona, P., Gurbani, S., Koh, S., Sankar, R., & Shields, W. D. (2008). Childhood absence epilepsy: Behavioral, cognitive, and linguistic comorbidities. Epilepsia, 49(11), 1838–1846.

Absence Seizure K ENNETH P ERRINE Northeast Regional Epilepsy Group Hackensack, NJ, USA Weill-Cornell College of Medicine New York, NY, USA

Synonyms Petit mal seizure; Psychomotor seizures

Definition Treatment Response to AED therapy in CAE and JAE is good, and valproic acid is often considered the drug of first choice.

An absence (usually pronounced with a French accent as ‘‘ab-SAWNS’’) seizure is a type of generalized seizure caused by a large burst of electrical discharges that

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Abstract Reasoning

begins in broad, bilateral brain regions simultaneously (as opposed to a partial seizure). During an absence seizure, the patient will lose interaction with the environment, stare blankly (‘‘zone out’’), and perhaps blink the eyes. There is no true loss of consciousness or motor functions. The seizure is typically short in duration (only several seconds), and patients often resume their ongoing activity without realizing even that they had a seizure (but will be amnestic for anything occurring during the episode). There are no postictal problems after the end of the seizure. Although no first aid is required, the patient should be protected from doing anything dangerous during the episode (e.g., cooking, crossing the street) but the episodes are often so brief that intervention is difficult.

Current Knowledge The cause of absence seizures is unknown. Patients with absence seizures typically have no positive neuroimaging findings, but usually have bursts of 3-per-s bilaterally synchronous spike/wave epileptiform activity on a routine EEG (even when not having a seizure). Absence seizures can be differentiated clinically from complex partial seizures, in which there is a similar disruption of consciousness and ‘‘zoning out,’’ by the duration of the episode. Absence seizures last only a few seconds, while complex partial seizures usually last 1–1.5 min. Absence seizures typically begin in childhood, respond well to medication, and often remit spontaneously by adulthood. Common medications for absence seizures include divalproex/ valproate sodium (Depakote), ethosuximide (Zarontin), and lamotrigine (Lamictal). Although the frequency of absence seizures can approach dozens per day, only mild (at worst) neuropsychological deficits are typically shown if the absence episodes occur without other seizure types. They do not have a dramatic impact on academic performance. However, absence seizures may occur with other seizure types in serious disorders such as Lennox-Gastaut syndrome, in which case there is considerable cognitive dysfunction and a worse prognosis.

Cross References ▶ Epilepsy

References and Readings Engel, J., & Pedley, T. A. (Eds.). (2008). Epilepsy: A comprehensive textbook (2nd ed.). New York: Lippincott Williams & Wilkins. www.epilepsyfoundation.org

Abstract Reasoning DAVID H ULAC University of South Dakota Vermillion, SD, USA

Synonyms Logical reasoning

Definition The neuropsychological construct of abstract reasoning refers to an individual’s ability to recognize patterns and relationships of theoretical or intangible ideas. Abstract reasoning is contrary to concrete reasoning whereby an individual recognizes patterns in information obtained through the immediate senses. When thinking abstractly, an individual must analyze and synthesize information without the aid of empirical information. Frequently, abstract reasoning requires an individual to apply concrete information to other scenarios that may not directly relate to that person’s experience. Abstract reasoning is most closely related to rational thought as opposed to empirical thought. While using deductive reasoning, a purely rational thinker does not look to determine the accuracy of a premise, but seeks only to understand the relationship between the premises. An example of deductive reasoning, which requires abstract reasoning, may go like this: 1. Premise 1: Egypt is located in South America. 2. Premise 2: The Sphinx lies in Egypt. 3. Conclusion: The Sphinx is located in South America. Empirically and concretely, it is obvious that Egypt is not in South America, but in Africa. To complete the syllogism, however, the thinker must ignore the concrete distortion, and instead focus on the two premises and understand if the conclusion logically flows. Common measures of abstract reasoning include the Similarities, Picture Concepts, and Matrix Reasoning subtests of the Wechsler scales. During a mental status exam, abstract reasoning is measured by asking a subject to describe the meanings of proverbs or to describe word similarities. Abstract reasoning, most commonly understood as being a function of the left hemisphere of the brain, is a precursor for using and understanding language and

Academic Ability

mathematics. Individuals who struggle with abstract reasoning benefit when an instructor uses examples to make the concept more concrete. Frequently, children with learning disabilities have difficulty with these abstract subjects, but achieve greater success in courses with more concrete subject matters such as social studies and science.

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Cross References ▶ Action-Intentional Disorders ▶ Adynamia ▶ Avolition

References and Readings References and Readings Goldstein, G. (2004). Abstract reasoning and problem solving in adults. In M. Hersen (Ed.), Comprehensive handbook of psychological assessment, Vol. 1: Intellectual and neuropsychological assessment (pp. 293–308). Hoboken, NJ: Wiley.

Abulia I RENE P IRYATINSKY Butler Hospital and Alpert Medical School of Brown University Providence, RI, USA

Synonyms Aboulia; Apathy; Athymia; Loss of psychic self-activation; Psychic akinesia

Definition Abulia refers to a lack of will, drive, or initiative. The word is derived from the Greek ‘‘abουlίa,’’ meaning ‘‘non-will.’’ It should be distinguished from an inability to actually perform the activity due to cognitive or physical disability. Abulia is manifested by the lack of motivation, spontaneity, and initiation. Some research indicates that abulia occurs because of malfunction of the brain’s dopaminedependent circuitry, especially bilateral lesions in the medial frontal lobes, basal ganglia, and their connections. The following criteria have been suggested for the diagnosis of abulia: (1) decreased spontaneity in activity and speech; (2) prolonged latency in responding to queries, directions, and other stimuli; and (3) reduced ability to persist with a task.

Berrios, G. E., & Grli, M. (1995). Abulia and impulsiveness revisited: A conceptual history. Acta Psychiatrica Scandinavica, 92(3), 161–167. Caplan, L. R., Schmahmann, J. D., Kase, C. S., Feldmann, E., Baquis, G., Greenberg, J. P., et al. (1990). Caudate infarcts. Archives of neurology, 47(2), 133–143. Drubach, D. A., Zeilig, G., Perez, J., Peralta, L., & Makley, M. (1995). Treatment of abulia with carbidopa/levadopa. Journal of Neurologic Rehabilitation, 9, 151–155. Egnelborghs, S., Marien, M. A., Pickut, B. A., Verstraeten, M. A., & De Deyn, P. P. (2000). Loss of psychic self-activation after paramedian bithalamic infarction. Stroke, 31, 1762–1765. Forstl, H., & Sahakian, B. A. (1991). A psychiatric presentation of abulia: Three cases of frontal lobe ischaemia and atrophy. Journal of the Royal Society of Medicine, 84, 89–91. Kumral, E., Evyapan, D., & Balkir, K. (1999). Acute caudate vascular lesions. Stroke, 30, 100–108. Laplande, D. N. A., Sauron, B., de Billy, A., & Dubois, B. (1992). Lesions of the basal ganglia due to disulfiram neurotoxicity. Journal of Neurology, Neurosurgery & Psychiatry, 55, 925–929. Litvan, I., Paulsen, J. S., Mega, M. S., & Cummings, J. L. (1998). Neuropsychiatric assessment of patients with hyperkinetic and hypokinetic movement disorders. Archives of Neurology, 55, 1313–1319. Powell, J. H., Al-Adawi, S., Morgan, J., & Greenwood, R. J. (1996). Motivation deficits after brain injury: Effects of bromocriptine in 11 patients. Journal of Neurology, Neurosurgery & Psychiatry, 60, 416–421.

Abusive Head Trauma ▶ Shaken Baby Syndrome (SBS)

ACA ▶ Anterior Cerebral Artery

Academic Ability ▶ Academic Competency

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Academic Competency

Academic Competency T ODD VAN W IEREN Indiana University of Pennsylvania Indiana, PA, USA

Synonyms Academic ability; Academic performance; Educational productivity

Definition The multidimensional characteristics of a learner – including skills, attitudes, and behaviors – that factor into their academic success. These characteristics can be separated and considered in one of two primary domains: academic skills or academic enablers (DiPerna & Elliot, 2000; Elliot & DiPerna, 2002). Academic skills are both the basic and complex skills (e.g., reading, writing, calculating, and critical thinking) needed to access and interact with content-specific knowledge. Academic enablers, however, are the attitudes and behaviors (e.g., interpersonal skills, motivation, study skills, and engagement) that a learner needs in order to take advantage of education.

Ma, L., Phelps, E., Lerner, J. V., & Lerner, R. M. (2009). Academic competence for adolescents who bully and who are bullied. The Journal of Early Adolescence, 29(6), 862–897. Shapiro, E. S. (2008). From research to practice: promoting academic competence for underserved students. School Psychology Review, 37(1), 46–51.

Academic Performance ▶ Academic Competency

Academic Skills C HRISTINA Z AFIRIS University of Northern Colorado Greeley, CO, USA

Definition

▶ Academic Skills ▶ Learning

Academic skills refer to a student’s ability to perform ageappropriate school activities related to writing, reading, and mathematical problem-solving. Additionally, academic skills refer to the information learned which is relevant to school success. Having solid academic skills improves academic progress throughout one’s school experience. Many of the academic skills a child learns are acquired in the school setting. However, pre-academic skills may be obtained in the child’s environment prior to the start of formal schooling. This may be achieved by exposure to mathematics (such as adding and subtracting objects at home), coloring, and reading with and to the child.


Cross References

Edl, H. M., Jones, M. H., & Estell, D. B. (2008). Ethnicity and english proficiency: Teacher perceptions of academic and interpersonal competence in European American and Latino students. School Psychology Review, 37(1), 38–45. Elliot, S. N., & DiPerna, J. C. (2002). Assessing the academic competence of college students: Validation of a self-report measure of skills and enablers. Journal of Postsecondary Education and Disability, 15(2), 87–100. DiPerna, J. C., & Elliot, S. N. (2000). The academic competence evaluation scales (ACES college). San Antonio, TX: The Psychological Association. Hutto, L. (2009). Measuring academic competence in college students: a review of research and instruments. Saarbru¨cken Germany: VDM Verlag.

▶ Academic Competency ▶ Educational Testing ▶ Learning ▶ Reading

Cross References

References and Readings Burchinal, M. R., Peisner-Feinberg, E., Pianta, R., & Howes, C. (2002). Development of academic skills from preschool through second grade: Family and classroom predictors of developmental trajectories. Journal of School Psychology, 40(5), 415–436.

Acalculia Christian, K., Morrison, F. J., & Bryant, F. B. (1998). Predicting kindergarten academic skills: Interactions among child care, maternal education, and family literacy environments. Early Childhood Research Quarterly, 13(3), 501–521. Shapiro, E. S. (2004). Academic skills problems: Direct assessment and intervention (3rd ed.). New York: Guilford Press.

Acalculia N ATALIE WAHMHOFF, E LAINE C LARK University of Utah Salt Lake City, UT, USA

Synonyms Acquired dyscalculia; Dyscalculia; Mathematics disability

Definition Acalculia, most simply, is the inability to perform mathematical tasks. These difficulties can stem from other deficits or can exist independently. Acalculia deficits can be global or selective and manifest in a wide variety of number processing and calculation abilities.

Categorization Generally, authors have agreed on two major distinctions: primary and secondary acalculia (Growth-Marnat, 2000). Primary acalculia occurs when mathematical deficits are fundamental and are present independently of other deficits. Deficits in primary acalculia include poor estimation, number comparison abilities, and difficulty understanding procedural rules and numerical signs. In primary acalculia, these deficits will exist regardless of whether tasks are presented in an oral or written format (Adila & Rosselli, 2002). The secondary acalculias are due to primary deficits in other areas. Aphasic acalculia occurs in patients with Wernicke’s and Broca’s aphasia. Patients with Broca’s aphasia have problems when translating word representations of numbers (three hundred and forty-five) to their numeral form (345). They may also read numbers with morphological errors (15 is read as 50) (Ardila & Rosselli, 2002; Basso, Burgio, & Caporali, 2000). When the secondary acalculia stems from Wernicke’s aphasia, deficits are more severe. Reading and writing of numbers often have semantic errors, and poor verbal memory often impacts the calculation abilities of these patients (Grafman & Rickart, 2000).

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Alexic acalculia is the inability to read number and correlations with the inability to read text. People with this type of acalculia may focus only on beginning digits (538 is read as 53). For those with alexic acalculia, mental calculation abilities exceed written calculation abilities (Ardila & Rosselli, 2002). Agraphic acalculia is the inability to write numbers. Like aphasic acalculia, agraphic acalculia correlates with Broca’s and Wernicke’s aphasia. In Broca’s aphasia, acalculia deficits manifest as omissions, substitutions, and order reversal. In Wernicke’s aphasia, difficulties are especially evident when required to write quantities when they are orally dictated. Those with Wernicke’s aphasia also tend to make paralexias and paragraphias (Ardila & Rosselli, 2002; Growth-Marnat, 2000). Frontal acalculia deficits occur in conjunction with attention difficulties, perseveration, and impairment of more complex math concepts (Dehaene, Cohen, & Changeux, 1998). Difficulties are most apparent with multistep operations, algorithms, and when planning is required. While complex concepts are difficult for patients with frontal acalculia, more basic math concepts are usually maintained (Ardila & Rosselli, 2002). Spatial acalculia impacts written mathematical tasks more than mental math tasks. A difficulty with writing numbers is quite apparent in these cases and manifest in several ways. Writing on only one side of the page, inability to write numbers in a straight line, and general disorganization are some of the deficits that impact math performance (Basso, Burgio, & Caporali, 2000). Patients with spatial acalculia often forget where to place remainders and carried numbers, despite understanding the basic division and multiplication functions. Math procedure signs are often undetected or switched (add instead of subtract).

Epidemiology Acalculia can result from stroke, tumors, and trauma. It is also seen in patients with degenerative dementia (Ardila & Rosselli, 2002).

Prognostic Factors and Outcomes There is noted variability in prognosis for acalculia, ranging from no recovery to full recovery. For primary acalculia, improvement is limited. In the case of secondary acalculias, recovery from the primary deficit, such as aphasia, alexia, and agraphia, occur, the corresponding acalculia deficits tend to improve as well.

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Neuropsychology and Psychology of Acalculia Primary acalculia is associated with left posterior parietal lesions. More specifically, damage to the left angular and supramarginal gyri occurs with primary acalculia (Grafman & Rickart, 2000). It is suggested that there are separate neuropathways for rote number knowledge and semantic number knowledge. Neuroimaging techniques reveal that several brain areas are active when performing calculations and also that the pattern differs according to what type of calculation is done (Dehaene, Cohen, & Changeux, 1998). This occurs to the many abilities that calculation often requires, including verbal, spatial, executive functioning, and memory. The areas most associated with calculation are the upper cortical surface and anterior aspect of the left middle frontal gyrus, the bilateral supramarginal and angular gyrus, the left dorsolateral prefrontal and premotor cortices, Broca’s area, inferior parietal and left parietal cortex, and the inferior occipitotempral regions (Ardila & Rosselli, 2002). It is important to keep in mind that damage to the right hemisphere and the frontal lobes also impact the occurrence of acalculia, especially when it is a secondary acalculia.

Evaluation The arithmetic section of the Wide Range Achievement Test (WRAT) has often been used to test operational skills. The Key Math, which is designed for children and adolescents, tests more targeted and specific abilities that are suggested for an acalculia assessment (Grafman & Rickart, 2000). Many authors have suggested experimental batteries that target specific functions and include error analysis. These batteries often assess skills in the following areas: number recognition, number writing, number transcoding, quantification, magnitude estimation, basic arithmetic operations, calculation fact verification, multicolumn calculations, magnitude comparison, fractions, algebra, and numeric knowledge. When possible, these skills should be assessed in both written and oral form (Ardila & Rosselli, 2002; Grafman & Rickart, 2000).

Treatment Some authors have suggested beginning rehabilitation with an error analysis if it was not completed during the

assessment. This will provide explicit areas to target during rehabilitation (Grafman & Rickart, 2000). Long-term rehabilitation programs should begin simply and progressively work toward more complex tasks. With secondary acalculia, focusing rehabilitation on the primary deficit may significantly improve the secondary acalculia deficits (Ardila & Rosselli, 2002).

Cross References ▶ Agraphia ▶ Alexia ▶ Aphasia ▶ Gerstmann’s Syndrome ▶ Spatial Dyscalculia

References and Readings Ardila, A., & Rosselli, M. (2002). Acalculia and dyscalculia. Neuropsychology Review, 12, 179–231. Ardila, A., Matute, E., & Inozemtseva, O. (2003). Progressive agraphia, acalculia, and anomia: a single-case report. Applied Neuropsychology, 10, 205–214. Basso, A., Burgio, F., & Caporali, A. (2000). Acalculia, aphasia, and spatial disorders in left and right brain-damaged patient. Cortex, 36, 265–280. Dehaene, S., Cohen, L., & Changeux, J. P. (1998). Neuronal network models of acalculia and prefrontal deficits. In R. W. Parks, D. S. Levine, & D. L. Long (Eds.), Fundamentals of neural network modeling: neuropsychology and cognitive neuroscience (pp. 233–255). Cambridge, MA, USA: MIT. Grafman, J., & Rickart, T. (2000). Acalculia. In M. J. Farah & T. E., Fienberg (Eds.), Patient based approaches to cognitive neurosciences: issues in clinical and cognitive neuropsychology. Cambridge, Massachusetts: MIT. Growth-Marnat, G. (Ed.). (2000). Neuropsychological assessment in clinical practice. New York: Wiley. Scruggs, T. E. & Mastropieri, M. A. (2000). Acalculia. In Encyclopedia of special education (2nd ed., Vol. 1, p. 27). New York: Wiley.

ACC ▶ Anterior Cingulate Cortex

Accelerated Hypertension ▶ Hypertensive Encephalopathy

Accessory Cuneate Nucleus

Acceleration Injury B ETH R USH Mayo Clinic Jacksonville, FL, USA

Synonyms Acceleration–deceleration injury

Definition Traumatic injury to the brain resulting from high-speed acceleration of the brain within the skull cavity in the direction of inertial force.

Current Knowledge During acceleration injury, movement of the head is unrestricted. One of the most common scenarios resulting in acceleration injury is a high-speed motor vehicle accident. Primary brain injury results from brain tissue and brain structures compressing against one another in the force of inertia. This may result in bruising, hemorrhage, and shearing of the underlying tensile strength of white matter connections deep within the brain. Secondary injury may occur hours or even days after the inciting traumatic event. Secondary effects of injury can include decreased cerebral blood flow, edema, hemorrhage, increased intracranial pressure, and biochemical changes that may cause excitotoxicity and more extensive damage to the surrounding brain structures and their associated connections. Theoretical models of linear acceleration injury now address the heterogeneity of effects that can result from such biomechanical injuries. Although diffuse brain damage may result from this type of injury, a key factor that predicts the extent of damage following acceleration injury is the area of initial impact. Given that the structure and projection pathways of the brain have varying densities and tensile strengths within different regions of the brain, the point of impact is most likely the key in determining the extent of damage that takes place and the likelihood and course of recovery that is possible following injury. Patients sustaining acceleration injury may experience headache, photophobia, phonophobia, nausea, and dizziness immediately following injury onset. On neuropsychological evaluation, patients with acceleration injuries are

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more likely to demonstrate a diffuse, rather than focal, profile of cognitive impairment when cognitive impairment is present. The lateralization of cognitive impairment that is typically observed in focal brain injury is relatively uncommon following acceleration injury. A diffuse profile of cognitive impairment in acceleration injury is due to the disruption of white matter tracts that are responsible for efficiency and coordination of communication between functional brain injuries. As such, a patient with acceleration injury may demonstrate cognitive slowing, executive dysfunction, and problems with simple and complex attention as a consequence of his/her brain injury.

Cross References ▶ Biomechanics of Injury ▶ Deceleration Injury ▶ Diffuse Axonal Injury

References and Readings Bayly, P. V., Cohen, T. S., Leister, E. P., Ajo, D., Leuthardt, E. C., Genin, G. M. (2005). Acceleration-induced deformation of the human brain. Journal of Neurotrauma, 22(8), 845–856. Sabet, A. A., Christoforou, E., Zatlin, B., Genin, G. M., & Bayly, P. V. (2008). Deformation of the human brain induced by mild angular head acceleration. Journal of Biomechanics, 41(2), 307–315.

Acceleration–deceleration Injury ▶ Acceleration Injury ▶ Deceleration Injury

Accessory Cuneate Nucleus J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Synonyms Lateral cuneate nucleus

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Accident Claims

Definition

Definition

Nucleus in the dorsolateral portion of the medulla that receives sensory information likely from touch, pressure, and stretch receptors in the upper extremities. It gives rise to the cuneocerebellar tract which enters the cerebellum via the inferior cerebellar peduncle. The accessory cuneate nucleus is thought to be the equivalent of the dorsal nucleus of Clarke in the lumbar, thoracic, and lower cervical cord which is the source of the dorsal spinocerebellar tract. These nuclei and tracts provide unconscious (as opposed to ‘‘conscious’’) sensory feedback to the cerebellum in its regulation of individual muscles. Lesions of this nucleus might be expected to produce cerebellar type symptoms of the ipsilateral upper extremity (i.e., ataxia/incoordination of movement), but it is relatively small and isolated lesions are likely to be extremely rare.

In order to provide students with disabilities the free, appropriate public education mandated by IDEA 2004 and Section 504 of the Rehabilitation Act of 1973, changes typically must be made to a child’s educational curriculum or environment. These accommodations include changes in the method of presentation of material, classroom seating location, availability of an interpreter for those with hearing impairment, response format, testing time allowed, setting, or other reasonable steps that do not significantly alter the content of educational material or the validity of tests. To be eligible to receive accommodations, students must be identified as having a disability consistent with the guidelines presented in IDEA 2004 or Section 504 of the Rehabilitation Act of 1973. Accommodations may also be required in the workplace under the Americans with Disabilities Act. These could include installation of a ramp to permit wheelchair access, flexible working hours, or provision of TTY machines.

Accident Claims ▶ Personal Injury

Cross References ▶ 504 Plan, Americans with Disabilities Act

Accident Neurosis ▶ Compensation Neurosis

References and Readings Education, 34 C.F.R. }104. Individuals with Disabilities Education Improvement Act of 2004, 20 U.S. C. } 1400 et seq. Rehabilitation Act, 29 U.S.C. } 794.

Accommodations J ACOB T. LUTZ 1, DAVID E. M C I NTOSH 2 1 Bell State University Muncie, IN, USA 2 Bell State University Muncie, IN, USA

Synonyms Reasonable accommodations

Accumbens Nucleus ▶ Nucleus Accumbens

Acetylaspartic Acid ▶ N-Acetyl Aspartate

Acetylcholine

Acetylcholine J OA NN T. T SCHANZ 1, K ATHERINE T REIBER 2 1 Utah State University Logan, UT, USA 2 University of Massachusetts Medical School Worcester, MA, USA

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important role in the contraction of skeletal muscles. Studies also suggest a role in cortical arousal, REM sleep, and cognitive functions such as attention, learning, and memory. Its presence in cardiac and smooth muscles, organs, and salivary, tear, and sweat glands affect autonomic functions (Feldman et al., 1997).

Current Knowledge Definition Acetylcholine has been identified as a neurotransmitter substance since the mid-1920s. It is the neurotransmitter substance present at the neuromuscular junction and also innervates structures of the parasympathetic and sympathetic nervous systems (Feldman, Meyer, & Quenzer, 1997; Iversen, Iversen, Bloom, & Roth, 2009). In the brain, cholinergic neurons have a wide distribution. Projections emanate from the basal forebrain in the medial septal nucleus and terminate in the hippocampus and limbic cortex. Among other areas receiving cholinergic input are the neocortex, olfactory bulbs, amygdala, neostriatum (caudate nucleus and putamen), the hypothalamus, and various regions in the brain stem (Feldman et al., 1997). Acetylcholine is synthesized from the precursors Acetyl CoA and choline in a chemical reaction involving the catalytic enzyme, choline acetyltransferase (ChAT). The presence of this enzyme has been used as a marker to locate cholinergic neurons. Acetylcholine degradation (the primary mode of removal from synapses) is accomplished by the activity of a group of enzymes known as cholinesterases. Acetylcholinesterase is the primary enzyme that breaks down acetylcholine in the synapse. Thus, to enhance cholinergic function, a number of substances have been developed that inhibit the activity of this enzyme (Iversen et al., 2009). Based on differences in the agonists that stimulate cholinergic receptors, two receptor subtypes have been identified, nicotinic and muscarinic. Nicotinic receptors are stimulated by nicotine, are excitatory, and show a rapid response to stimulation. Muscarinic receptors are stimulated by muscarine, have either excitatory or inhibitory effects, and show a slower response to stimulation. Further subtypes exist within the nicotinic and muscarinic classes (Feldman et al., 1997; Iversen et al., 2009). Acetylcholine is involved in a number of behavioral processes. As a neurotransmitter substance at the neuromuscular junction, it acts on motor neurons of the spinal cord and cranial motor nerve nuclei, playing an

Applications Dysfunction in the cholinergic system has been implicated in a number of clinical conditions including Alzheimer’s disease (AD), diffuse Lewy body dementia (Londos, Brun, Gustafson, & Passant, 2003), Huntington’s disease, and myasthenia gravis (Iversen et al., 2009). Recent work also suggests a reduction in cholinergic activity in Parkinson’s disease that may appear relatively early in the course of the condition (Shimada et al., 2009). Acetylchoinesterase inhibitors are used in the palliative treatment of AD and myasthenia gravis. Cholinergic or anticholinergic compounds are also used as a muscle relaxant for surgery, treatment of parkinsonism, glaucoma, urinary retention, and in nonclinical applications such as insecticides in agriculture and neurotoxins (and their antidotes) in warfare (Feldman et al., 1997; Iversen et al., 2009). Much research is being conducted to develop agents with greater receptor subtype specificity to better address clinical conditions.

Cross References ▶ Alzheimer’s Disease ▶ Anticholinesterase Inhibitors ▶ Cholinesterase Inhibitors

References and Readings Feldman, R. S., Meyer, J. S., & Quenzer, L. F. (1997). Acetylcholine. In Principles of neuropsychoparhmacology (pp. 246–249). Sunderland, MA: Sinauer Associates. Iversen, L. L., Iversen, S. D., Bloom, F. E., & Roth, R. H. (2009). Acetylcholine. In Introduction to neuropsychopharmacology (pp. 128–149). New York: Oxford University Press. Londos, E., Brun, A., Gustafson, L., & Passant, U. (2003). Lewy body dementia. Clinical challenges in diagnosis and management. In K. Iqbal & B. Winblad (Eds.), Alzheimer’s disease and related

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disorders: Research advances (pp. 133–142). Bucharest, Romania: Ana Asian International Academy of Aging. Shimada, H., Hirano, S., Shinotoh, H., Aotsuka, A., Sato, K., Tanaka, N., et al. (2009). Mapping of brain acetylcholinesterase alterations in Lewy body disease by PET. Neurology, 73, 273–278.

(Bartels & Zeki, 2000), patients lose the ability to perceive color, and therefore experience the world as varying shades of gray. This disorder is termed cerebral achromatopsia. The loss of color vision in these patients cannot be explained by the photoreceptors typically damaged or absent in patients with other types of color blindness.

Acetylcholinergic System Categorization ▶ Cholinergic System

Acetylcholinesterase Inhibitors ▶ Anticholinesterase Inhibitors ▶ Cholinesterase Inhibitors

ACHE Inhibitors ▶ Anticholinesterase Inhibitors

Cerebral achromatopsia results from bilateral damage to the V4/V4a region of the color center. If patients experience complete ablation of V4, they lose color vision in their entire visual field. However, if patients experience unilateral damage to V4, hemi-achromatopsia ensues, where patients only lose color vision in the contralateral half of their visual field. In less extreme cases, known as dyschromatopsia, patients lose the ability to perceive selective colors and/or color constancy. These neuropsychological disorders, which are the result of damage to the cerebral cortex, should not be confused with congenital achromatopsia, which occurs as a malfunction of the cone photoreceptors.

Epidemiology

AchEIs ▶ Anticholinesterase Inhibitors ▶ Cholinesterase Inhibitors

Achromatopsia S OPHIE L EBRECHT, M ICHAEL J. TARR Brown University Providence, RI, USA

Synonyms Acquired achromatopsial; Color agnosia; Color blindness; Cortical color blindness

Short Description or Definition Following damage to the ventral medial region of the occipital lobe, known as the ‘‘color center’’ of the brain

Cerebral achromatopsia arises following brain damage to V4/V4a located in the ventral medial region of the occipital lobe, typically caused by a tumor, a hemorrhage, or some sort of brain trauma. Due to the low incidence rate of cerebral achromatopsia, it is difficult to provide a reliable estimate of its prevalence. However, it seems safe to say that it is extremely rare. A review of the documented cases showed that of the 27 cases reported, 3 patients recovered, 3 partially recovered, and 21 showed no recovery (Bartels & Zeki, 2000).

Natural History, Prognostic Factors, Outcomes The first cases of cerebral achromatopsia were reported by Verrey (1888). In response to these patients, Verrey introduced the concept of a ‘‘color center’’ in the brain. Continued research confirmed the existence of a cortical region devoted to color processing. Almost a century later, Meadows demonstrated a correlation between the cortical regions sensitive to color, and the damaged cortical regions in achromatopsic patients (Meadows, 1974).

Acoustic Aphasia

Neuropsychology and Psychology of Achromatopsia The region of damage in the visual field of achromotopsic patients, V4/V4a, is organized retinotopically; therefore, damage to a particular region of V4 results in a loss of color vision at the corresponding location in the visual field. For example, if damage to V4 occurs in the left hemisphere, the patient will lose color vision in the right half of their visual field. Because V4 is located in the vicinity of the fusiform gyrus and the lingual gyrus, known to process faces (Kanwisher et al., 1997), the comorbity between achromatopsia and prospoagnosia is extremely high (Bouvier & Engel, 2006). In addition, patients with achromatopsia also have a higher incidence of spatial and shape deficits. It has been noted that patients with complete achromatopsia cannot even imagine color, which means they cannot dream in color or use color during mental imagery. This absence of color vision often leaves patients with no appetite for foods, which appear gray, and no desire for intimacy, as flesh appears gray. An insightful case study of a color-blind painter describes these experiences in detail (Sacks, 1995).

Evaluation Cerebral achromatopsia can be diagnosed using a range of color vision tests. The simplest test is an explicit colornaming task that requires patients to name the color of individual flash cards. The most common test for color blindness is the Ishihara plates test. These plates contain isoluminant colored dots of varying sizes that together create the perception of a number embedded in noise. In order to perceive the number, patients must be able to distinguish between the different colored dots. Another widely-used test is the Farnsworth-Maunsell 100 Hue test, in which patients are required to order colored caps based on gradual shifts in hue from light to dark. Patients with color blindness are unable to perform this task. Rarely, a diagnosis is made using a Nagel Anomaloscope. This apparatus is typically used to determine whether a patient is a monochromat or a diachromat; however, some experimenters/practitioners use it in the study of cerebral achromatopsia.

Treatment There is a period of spontaneous recovery for neurovisual lesions, which typically lasts 3 months post-lesion, but can

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occur for up to a year. With regard to the treatment and diagnosis of cerebral achromatopsia, experimenters report that some patients are not conscious of the absence of color vision. This phenomenon has been explained by the ablation of a color module leaving patients without even the concept of color post-lesion. This symptom of achromatopsia should be noted when addressing patients, because pushing a patient to describe a condition they are not aware of could be distressing for the patient.

Cross References ▶ Scotoma

References and Readings Bartels, A., & Zeki, S. (2000). The architecture of the color centre in the human visual brain: New results and a review. The European Journal of Neuroscience, 12(1), 172–193. Bouvier, S. E., & Engel, S. A. (2006). Behavioral deficits and cortical damage loci in cerebral achromatopsia. Cerebral Cortex (New York, N.Y.: 1991), 16(2), 183–191. Kanwisher, N., McDermott, J., & Chun, M. M. (1997). The fusiform face area: A module in human extrastriate cortex specialized for face perception. Journal of Neuroscience, 17(11), 4302–4311. Meadows, J. C. (1974). Disturbed perception of colors associated with localized cerebral lesions. Brain: A Journal of Neurology, 97(4), 615–632. Sacks, O. W. (1995). An anthropologist on mars: Seven paradoxical tales. New York: Vintage Books. Verrey, D. (1888). He´miachromatopsie Droite Absolue. Conversation Partielle De La Perception Lumineuse Et Des Formes. Ancien Kyste He´morrhagique De La Partie Infe´rieure Du Lobe Occipital Gauche. Archives d’ophtalmologie, 8, 289–300. Werner, J. S., & Chalupa, L. M. (2004). The visual neurosciences. Cambridge, MA: MIT Press.

ACoA ▶ Anterior Communicating Artery

Acoustic Aphasia ▶ Pure Word Deafness

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Acoustic Neuroma

Acoustic Neuroma E THAN M OITRA Drexel University Morgantown, WV, USA

Synonyms Neurolemmoma; Vestibular schwannoma

Definition A benign tumor of the Schwann cells occurring near the cerebellopontine angle of the brain stem. Typically, it arises from the vestibulocochlear or eighth cranial nerve, which connects the brain to the inner ear. It is commonly associated with neurofibromatosis type 2 and often occurs bilaterally. Tumor growth is usually slow and may result in some hearing loss or deafness, tinnitus, vertigo, and vestibular dysfunction. Most acoustic neuromas are diagnosed in patients between the ages 30 and 60. Etiology is unknown. Treatment options include radiosurgery and microsurgical removal.

Acoustic Neuroma. Figure 2 Courtesy Carol Armstrong. Children’s Hospital of Philadelphia and the University of Pennsylvania Medical School, Department of Neurology

Cross References ▶ Radiosurgery ▶ Radiotherapy

References and Readings Jørgensen, B. G., & Pedersen, C. B. (1994). Acoustic neuroma. Follow-up of 78 patients. Clinical Otolaryngology, 19, 478–484.

Acquired Achromatopsial ▶ Achromatopsia

Acoustic Neuroma. Figure 1 Courtesy Carol Armstrong. Children’s Hospital of Philadelphia and the University of Pennsylvania Medical School, Department of Neurology

Acquired Dyscalculia ▶ Acalculia

Acquired Immunodeficiency Syndrome (AIDS)

Acquired Epileptic Aphasia ▶ Landau–Kleffner Syndrome

Acquired Immunodeficiency Syndrome (AIDS) C. M ICHAEL N INA William Paterson University Wayne, NJ, USA

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pandemic has killed approximately 25 million people worldwide. UNAIDS, a joint program of the United Nations and the World Health Organization, estimates that globally, in 2007, 33.2 million people lived with HIV, 2.5 million became newly infected, and 2.1 million died from AIDS. In North America alone, 1.3 million lived with HIV, 46,000 became newly infected, and 21,000 died from AIDS; and approximately, 500,000 have already died from AIDS.

Categorization Differentiation between a diagnosis of HIV or AIDS depends on CD4+ T cell count and presence of opportunistic infections.

Short Description or Definition Acquired immunodeficiency syndrome or AIDS is a disease caused by infection with the human immunodeficiency virus or HIV. HIV is a viral pathogen that attacks CD4+ T cells (thymus originating lymphocyte cells with cluster determinant 4 + surface receptor sites) of the human body’s immune system. These CD4+ T cells (also called T4 or T helper cells) play a central signaling role in the human immune response. In addition, HIV also causes damage to the central nervous system. The exact cause of this damage is unclear at this time, but it is believed to be caused either by the ‘‘Trojan horse’’ model or neuroinflammation model. In the Trojan horse model, immune system cells known as macrophages conceal and convey HIV into the brain, where they can disrupt supportive brain cells such as astrocytes and microglia. In the neuroinflammation model, the body’s over stimulated immune system causes an increased production of CD14+ CD16+ monocytes which flood the brain, causing inflammation and damage to brain cells and structures. AIDS is the name given to the end stage of HIV infection when the body’s ability to fight off microorganisms is compromised, resulting in debilitating or fatal diseases, which are known as ‘‘opportunistic infections.’’ An individual with HIV infection receives a formal diagnosis of AIDS when the individual has at least one opportunistic infection or when the individual’s CD4+ T cell count is below 200 per mm3 of blood (normal count is typically 500–1,500 per mm3). In the absence of anti-HIV or antiretroviral drug therapy, progression to AIDS can take an average of 8–12 years for adults and adolescents, and 3 years from birth in prenatally infected children. A quarter of a century after the first deaths from AIDS were identified, the AIDS

Etiology/Epidemiology In 1981, the US Centers for Disease Control and Prevention (CDC) began receiving reports about unusual cases of Pneumocystis carinii pneumonia (PCP) and Kaposi’s sarcoma in young gay men and PCP in injection drug users. These diseases were not typically seen in individuals with healthy immune systems. In early 1982, similar disease patterns were seen in blood transfusion recipients, hemophiliacs, and heterosexual partners of those already infected. In late 1982, the CDC officially named this disease pattern as acquired immune deficiency syndrome or AIDS. In 1984, a previously unknown human retrovirus was discovered in the blood of individuals with AIDS by teams in the US and France. In 1986, the retrovirus was named as HIV. Retroviruses have an RNA (ribonucleic acid) genome, and use an enzyme called reverse transcriptase to convert their RNA into DNA (deoxyribonucleic acid), in order to Acquired Immunodeficiency Syndrome (AIDS). Table 1 Differentiation between human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome (AIDS) in individuals infected with HIV Symptom

Diagnosis

CD4+ T cell count of 200 or higher per mm3/blood

HIV infection

CD4+ T cell count below 200 per mm3/blood

AIDS

Presence of one or more opportunistic infection

AIDS

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replicate, which is done in the nucleus of infected cells. HIV is a member of the lentivirus group of retroviruses which also includes simian immunodeficiency virus. Lentiviruses typically have longer incubation periods and greater genetic complexity than other retroviruses. In 1985, a second strain of the virus was discovered, which was designated as HIV-2. The original strain of the virus was designated as HIV-1. HIV-1 is much more common throughout the world, while HIV-2 is more common in certain parts of Africa alone. Also, HIV-2 appears to be milder than HIV-1, with a slower progression to AIDS. Since its establishment in humans, HIV-1 has undergone mutation of its genome and there are now three groups of HIV-1. How HIV is transmitted tends to vary worldwide depending upon the geographic region. In the United States, approximately 45% of current cases of HIV infection were obtained through male–male sexual contact (men who have sex with men or MSM), 22% were through injecting drug users (IDU), and 5% were through individuals who were both MSM and IDU. Approximately, 27% of cases were through male–female sexual contact. Transmission rates have been changing though, with new cases of infection in older white MSM decreasing. Transmission rates have been increasing in African–American and Latino MSM and younger white MSM due to increases in high-risk sexual practices; approximately, 50% of new cases are in African–American MSM. Rates of transmission are also increasing in women, primarily due to heterosexual contact with MSM and IDU. In Africa, transmission is primarily due to male–female sexual contact. In Eastern Europe, transmission is primarily in IDU or male–female sexual contact. In Southeast Asia, transmission is primarily through contact with commercial sex workers.

Natural History, Prognostic Factors, Outcomes HIV is not transmitted through casual contact, such as touching. It can be transmitted when the bodily fluids of infected individuals – primarily blood, semen, vaginal fluid, or breast milk – comes into contact with the bloodstream or mucosal tissues of uninfected individuals. Transmission can occur through: 1. Unprotected sexual contact (anal, vaginal, or oral) with an individual infected with HIV 2. Sharing needles or syringes with HIV-infected individuals

3. Transfusion of infected blood or other bodily incorporation of infected blood 4. A fetus or infant exposed to HIV before or during birth or through breast feeding The natural progression of HIV infection can be divided into three stages: primary infection, clinical latency, and symptomatic disease stage. The symptomatic disease stage is further divided into early and late stages, with AIDS being equated with the late-symptomatic disease stage. After a person is initially infected with HIV, a primary or acute infection stage commences, in which HIV replicates up to ten billion copies of itself daily; high levels of HIV in the blood or viraemia is evident. Approximately, 2–4 weeks after exposure, nearly 70% of those newly infected will experience an acute illness, which has symptoms similar to influenza or mononucleosis, including fever, fatigue, muscle weakness, headache, ocular pain, sensitivity to light, sore throat, diarrhea, and lymphadenopathy. This illness is due to the temporary reduction of CD4+ T cells; it lasts for approximately 2 weeks and then resolves spontaneously. It is during this stage that the individual typically first begins to produce antibodies to HIV, which is designated as seroconversion. Serological testing of blood can reliably detect HIV antibodies 2–6 months after seroconversion. Testing typically begins with an enzyme-linked immunosorbent assay (ELISA) or test that looks for antibodies to HIV. A second positive ELISA is needed in order to confirm the result. This would then be followed by the Western Blot Procedure to confirm the presence of at least two specific HIV antigen groups. A diagnosis of HIV infection is given after a positive Western Blot test follows two positive ELISA tests. If HIV is confirmed, additional tests for plasma viral RNA (viral load) and CD4+ T cell counts are then typically completed, in order to assess the state of the immune system and disease prognosis. Higher viral load counts are typically related to faster disease progression. Lower CD4+ T cell counts are typically related to greater clinical vulnerabilities. After the acute illness disappears, the individual enters the clinical latency stage in which symptoms are typically absent, other than possibly chronic lymphadenopathy. This stage lasts an average of 10 years. During the clinical latency stage, HIV continues to replicate and attack CD4+ T cells, which in turn continues to counter attack. As the immune system becomes more compromised, individuals eventually enter the early symptomatic disease stage, when a variety of symptoms begin to manifest, including lymphadenopathy, lack of energy, diarrhea,


unintentional weight loss, chronic low-grade fever and sweats, frequent rashes or fungal infections, headaches, or short-term memory loss. Finally, individuals enter the late stage of the symptomatic disease stage or AIDS when the person has at least one opportunistic infection or when the individual’s CD4+ T cell count is below 200 per mm3 of blood. The most common opportunistic infections are PCP, Kaposi’s sarcoma, HIV wasting syndrome, and HIVencephalopathy (also known as dementia due to HIV disease or AIDS dementia complex).

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Acquired Immunodeficiency Syndrome (AIDS). Table 2 HIV dementia symptoms Behavioral difficulties Depression Apathy, anhedonia, social withdrawal Personality changes, including spontaneous sudden and strong emotions Cognitive difficulties Confusion Short-term memory lapses Loss of concentration

Psychological and Neuropsychological Correlates of HIV Infection

Motor difficulties Lack of muscular coordination Tremors

As HIV infection progresses, various psychological and neuropsychological complications involving both the central as well as peripheral nervous systems can become evident. During primary infection, reports of headaches and aseptic meningitis are common. During the clinical latency stage, an acute inflammatory demyelinating neuropathy (similar to Guillain-Barre syndrome; characterized by progressive muscle weakness) can occasionally develop. During the early symptomatic disease stage, peripheral neuropathy is common. This is characterized by spontaneous pain (dysesthesia), pain due to light touches or changes in temperature (hyperesthesia), and weakness and wasting in arms/legs (distal atrophy). It is during the late symptomatic disease stage or AIDS that most major neuropsychological complications develop, and can include: 1. HIV encephalopathy (HIV dementia) 2. Opportunistic infections (a) Viral (Cytomegalovirus; Herpes Simplex I and II; Herpes Zoster; JC virus, a polyomavirus or papovavirus which causes PML [progressive multifocal leukoencephalopathy]) (b) Fungal/Protozoan (Toxoplasmosis, Cryptococcus, Candida, Mycobacterium) 3. Lymphomas (a) Primary central nervous system lymphomas (b) Systemic (metastatic) lymphomas. (The most common systemic lymphomas are: Hodgkin’s; immunoblastic; Burkitt’s; and non-Hodgkin’s, which is particularly prevalent.) HIV encephalopathy is the term used to describe the pathological features of encephalitis of the brain due to HIV, while HIV dementia (also known as AIDS dementia complex) is used to describe the clinical syndrome. This

Muscle weakness Loss of balance

syndrome is characterized by behavioral, cognitive, and motor declines and difficulties (Table 2). Initial symptoms typically manifest as cognitive difficulties (loss of concentration and mild deficits in memory) with motor and behavioral difficulties frequently occurring. (This early stage is often labeled as HIV-associated minor cognitive motor disorder.) Later symptoms include partial paralysis, incontinence, and severe cognitive impairment. Death usually occurs within 1–6 months after onset of severe symptoms. Individuals who are coinfected with hepatitis C or were IDU, typically display worse symptoms faster. As HIV-infected individuals live longer, it is estimated that 50–75% of all patients with AIDS will evidence some form of HIV dementia. While HIV can be present in any part of the brain, HIV is particularly common in the basal ganglia and central white matter (and to a lesser extent in neocortical gray matter, the brainstem, and the cerebellum) in individuals not receiving antiretroviral therapy or highly active antiretroviral therapy (HAART) (see below). In individuals on HAART, there is evidence of greater inflammation in the hippocampus and surrounding entorhinal and temporal cortex.

Treatment While there is no cure or vaccine for HIV or AIDS at this time, there are currently four different classes of

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antiretroviral drugs that interfere with the ability of HIV to replicate: reverse transcriptase inhibitors (nucleoside and non-nucleoside types); protease inhibitors; entry/fusion inhibitors; and integrase inhibitors. In 1987, the US Food and Drug Administration (FDA) approved Azidothymidine (AZT, also known as Zidovudine), the first nucleoside-reverse transcriptase inhibitor (NRTIs). Saquinavir, the first protease inhibitor was approved in 1995. Nevirapine, the first non-nucleoside-reverse transcriptase inhibitor was approved in 1996. Enfuvirtide, the first fusion inhibitor was approved in 2003. Maraviroc, the first entry inhibitor, and Reltegravir, the first integrase inhibitor, were approved in 2007. In 1996, combination drug therapy or HAART began. Three or more drugs are used in combination in order to counter the development of drug resistance by HIV. Strict adherence to medication intake schedules is required. Not only is this schedule difficult to follow for many individuals, HAART often produces unpleasant and toxic side effects, including stomach problems and lipodystrophy. If followed correctly, HAART typically and drastically reduces viral load, often to undetectable levels in the blood, which allows the immune system to rebound. Antiretroviral drug therapy and treatments for opportunistic infections have greatly increased life expectancy of those with HIV infection, but due to the presence of HIV in cells that remain out of reach of antiretroviral drugs, eradication of HIV from the human body is unattainable at this time.

UNAIDS. (2007). UNAIDS Annual Report 2007: Knowing your epidemic. Retrieved June 15, 2008 from http://data.unaids.org/pub/Report/ 2008/jc1535_annual_report07_en.pdf Weeks, B. S., & Alcamo, I. E. (2006). AIDS: The biological basis (4th ed.). Sudbury, MA: Jones and Bartlett Publishers.

Acquisition of Knowledge ▶ Learning

Action Tremor A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Synonyms Intention Tremors

Definition Cross References ▶ Dementia ▶ Encephalitis ▶ Meningitis

Action tremor is a rhythmic, oscillatory, and involuntary movement of the limb that is seen with movement of an extremity. It may be seen in isolation with a cerebellar lesion or associated with other tremor types such as the postural tremor of essential tremor or the rest tremor of Parkinson’s disease.


Cross References

Bartlett, J., & Finkbeiner, A. (2006). The guide to living with HIV infection, developed at the John Hopkins AIDS clinic (6th ed.). Baltimore: Johns Hopkins University Press. Portegies, P., & Berger, J. (Eds.). (2007). HIV/AIDS and the nervous system: Handbook of clinical neurology. Amsterdam: Elsevier. Pratt, R. (2003). HIV & AIDS: A foundation for nursing and healthcare practice. London: Arnold Publishers. Sande, M., & Volberding, P. (Eds.). (1999). The medical management of AIDS (6th ed.). Philadelphia: Saunders. Stine, G. (2005). AIDS update 2005. San Francisco: Pearson/Benjamin Cummings.

▶ Essential Tremor ▶ Parkinson’s Disease

References and Readings Fahn, S., & Jankovic, J. (Eds.). (2007). Tremors: Diagnosis and treatment. In Movement disorders (pp. 451–479). Philadelphia: Churchill Livingstone Elsevier.

Action-Intentional Disorders

Action-Intentional Disorders K ENNETH M. H EILMAN The Malcom Randall Veterans Affairs Hospital Randall Veteran’s Affairs Medical Center Gainesville, FL, USA

Synonyms Abulia; Akinesis; Hypokinesis; Motor impersistence (These terms are not fully synonymous with actionintentional disorders, but comprise important elements of the syndrome and are often used when describing specific these elements.)

Definition In the absence of weakness, patients can have a disability with initiating (akinesia, hypokinesia, abulia) or sustaining actions (impersistence), inhibiting irrelevant actions (defective response inhibition), and stopping an action when the task has been completed (motor perseveration).

Current Concepts The motor system allows humans to interact with their environment and alter themselves as well as others. The human corticospinal motor system together with the motor units and muscles can mediate an almost infinite number of movements and thus the human motor system needs to be guided by at least two major types of programs: praxic and intentional. The praxic programs provide the corticospinal system with the knowledge of how to make skilled movements (spatial and temporal aspects of movements) and the intentional programs provide the corticospinal system with information about when to move. In this section, we will discuss disorders of the intentional, or ‘‘when,’’ systems. When interacting with environmental stimuli or the self, there are four ‘‘when’’ questions that must be addressed: these are (1) when to move, (2) when to persist at a movement or movements, (3) when to end a movement or a series of movements, and (4) when not to move. The inability to initiate a movement in the absence of a corticospinal or motor unit injury is called akinesia. Some patients are able to move after a delay and we call this hypokinesia. Motor impersistence is when a patient cannot sustain a movement

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or a series of movements that are needed to complete a task. The inability to stop a movement or an action program when it is no longer required is called motor perseveration and the inability to withhold a response to a sensory stimulus is called defective response inhibition. These motor intentional disorders are parallel to disorders of sensory attention, akinesia being akin to unawareness, impersistence being the motor parallel of decreased vigilance, motor perseveration being parallel to failures of extinction or habituation, and defective response inhibition being similar to distractibility. There are also cognitive defects that mirror four types of intentional motor disorders mention above, but these will not be discussed here. In the next section, we briefly describe each of these intentional disorders, including subtypes of each category and in the final section we briefly discuss the possible pathophysiology.

Clinical Manifestations Akinesia An organism might fail to initiate a movement for many reasons, but comprehension, attentional, perceptual, sensory, and motor disorders that lead to a failure of movement initiation should not be termed akinesia. In contrast to these disorders, akinesia is caused by a failure of the systems that are responsible for activating the motor system. There are three methods by which akinesia can be distinguished from extreme weakness. Certain forms of akinesia are present under certain sets of circumstances and absent in others. Thus, using the behavioral method, if it can be demonstrated that a patient makes movements in one set of circumstances (e.g., a motionless left hand is brought over to the right side of the body and the patient is able to now move this hand) and not in the other, this failure to move is related to an akinesia. If the akinesia is not limited to a set of circumstances then the clinician may have to depend on brain imaging, or physiological techniques such as magnetic stimulation of the motor cortex to demonstrate that the brain lesion did not involve the motor system and thus the failure to move is not caused by weakness. There are at least three subtypes of akinesia: (1) Body part: Akinesia may involve the eyes, the neck and head, a limb, or the total body; (2) Action space: Akinesia of the limbs, eyes, or head may depend on where in space the body part is moved or in what direction it is moved.

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The former is called spatial akinesia (e.g., a hand that does not move in left hemispace, but does move in right bodycentered hemispace) and the latter is called directional akinesia (e.g., a horizontal gaze palsy where patients cannot move their eyes to the left); (3) Stimulus–response conditions: Some patients, such as those with Parkinson’s disease, are impaired in spontaneously initiating a movement, but in response to a stimulus often have no trouble initiating a movement. We call this endogenously evoked akinesia (endo-evoked). Patients who fail to move to an imperative stimulus but will move spontaneously we call exogenously evoked akinesia. A patient may have both exoevoked and endo-evoked akinesia, which we term mixed or global akinesia.

with various body parts including the limbs, eyes, neck, eyelids (e.g., keep your eyes closed for until I tell you to open them), jaw, and tongue. Patients can even demonstrate impersistence in activities such as walking. Like akinesia, it may also be directional (e.g., inability to maintain leftward gaze) or hemispatial (inability to maintain dorsiflexion of the wrist in left space with the left arm, but able to do so in right space).

Defective Response Inhibition

Patients with sensory extinction are able to detect single stimuli on either side of their body, but when presented with two stimuli one on each side of their body they remain unaware of contralesional stimuli. Motor extinction is a form of akinesia or hypokinesia where a patient who is without sensory extinction is asked to respond by moving the hand (or hands) that was (were) touched. The examiner then delivers stimuli to the right, left, and both hands and patients with motor extinction are aware that both hands have been touched, but either fail to lift the contralesional hand to simultaneous stimuli or lift it after a delay.

Not all stimuli require a response and sometimes a response might interfere with goal-oriented behavior. Defective response inhibition is defined as responding when no response of that body part is required. It can be seen in a variety of body parts and might also be directional and perhaps hemispatial. There are several forms of defective response inhibition. One means of testing for this disorder is to use the crossed response task. A blindfolded patient is instructed to raise the hand opposite to that touched. Patients with defective response inhibition will often raise the touched hand first. This type of defective response inhibition may be termed motor (limb or eye directional) response disinhibition. These can be either contralesional or bilateral. The eye directional defective response inhibition has also been called a visual grasp. There are some patients, however, who have a perceptual disorder and when stimulated on one side (e.g., left hand) feel that they were stimulated on the other (e.g., right hand). This phenomenon is called allesthesia and it should not be confused with defective crossed response inhibition. Patients with defective response inhibition may also fail on the types of go–no-go tasks described by Luria. For example, the patient may be instructed to put up two fingers when the examiner puts up one finger and to put up no fingers if the examiner puts up two fingers. If the patient mimics the examiner such that when the examiner puts up one finger, the patient puts up one finger and when the examiner puts up two fingers, the patient puts up two fingers, the patient has echopraxia.

Motor Impersistence

Motor Perseveration

The inability to sustain a motor act or a series of motor acts that are required to complete a goal is called motor impersistence. Like akinesia, impersistence can be associated

When a patient incorrectly repeats a prior response or when a patient continues to perform the same act when the goal of the act has been completed, it is called

Hypokinesia A milder defect in the intentional motor (‘‘when’’) systems might not induce a total inability to initiate a response (i.e., akinesia), but rather these patients’ intentional deficit might be manifested by a delay in initiating a response. We call this delay hypokinesia. The hypokinesias may also be subtyped into body part (e.g., limb or eyes) and action space (e.g., directional and hemispatial).

Motor Extinction

Activa®

motor perseveration. In one type of motor perseveration, when the task requirements have changed the patient is unable to switch to a different motor program and incorrectly repeats the movements. Luria (1965) calls this inertia of program action and Sandson and Albert (1987) call this recurrent perseveration. In the second type, the patient continues to perform movements even though the task is completed. Luria (1965) called this efferent perseveration; however, Sandson and Albert (1987) call this continuous perseveration.

Pathophysiology of Intentional Disorders Intentional motor disorders are often associated with bilateral hemispheric lesions, but when these disorders are caused by a unilateral hemispheric lesion they are more commonly associated with right than left-hemisphere lesions. The intentional disorders that have been reported to be induced by primarily right-hemisphere lesions include akinesia (e.g., left-sided limbs, leftward arm movements, and even left horizontal gaze), hypokinesia (slowed reaction times), motor impersistence of the left-sided limbs, left-sided gaze), and motor (continuous) perseveration. Many of the intentional defects associated with righthemisphere dysfunction, however, are not just limited to the left limbs. For example, patients with a right-hemisphere lesion are more often abulic, have slowed reaction times of their right hand, and have motor impersistence of eye closure. These clinical studies suggest that the right hemisphere may be dominant for intentional control of the motor systems. Studies with normal subjects provide further evidence for right-hemisphere intentional dominance. The anatomic and physiological basis for this dominance is not entirely understood. Studies of patients with focal lesions and studies of monkeys suggest that the frontal lobes may play a critical role in mediating intentional activity. The most important areas of the frontal lobes appear to be the medial and lateral frontal lobes. The frontal cortex has strong projections to the striatum. The lateral portion of the frontal lobe projects to the caudate. The premotor cortex projects to the putamen and the cingulate gyrus projects to the ventral striatum. The striatum projects to the pars reticularis of the substantia nigra and the globus pallidus. The globus pallidus projects to specific thalamic nuclei and these thalamic nuclei project back to the frontal cortex. Just as injury of the frontal lobes can induce intentional

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deficits, injuries, or diseases that injure the basal ganglia, the substantia nigra (e.g., Parkinson’s disease), portions of the thalamus, as well as the white matter connections can also induce intentional deficits.

Future Directions Disorders of intention have received considerably less neuroscientific study than have disorders of sensory selective attention. There is a need for additional experimental and clinical neuropsychological studies of these disorders. Furthermore, assessment batteries are needed that will facilitate the assessment of the subtypes of motor intention disturbances and which may provide additional quantitative data for experimental analysis and normative comparison between patient groups and health individuals.

Cross References ▶ Attention ▶ Directional Hypokinesis ▶ Impersistence ▶ Neglect Syndrome

References and Readings Heilman, K. M., Valenstein, E, Rothi, L. J. G., & Watson, R. T. (2004). Intentional motor disorders and apraxia. In W. G. Bradley, R. B. Daroff, G. M. Fenichel, & J. Jankovic (Eds.), Neurology in clinical practice: Principles of diagnosis and management. (pp. 117–130). Phila Penn: Butterworth Heineman. Heilman, K. M., Watson, R. T., & Valenstein, E. (2003). Neglect and related disorders. In K. M. Heilman, & E. Valenstein (Eds.), Clinical neuropsychology, (4th ed., pp. 296–346). New York: Oxford University Press. Heilman, K. M. (2004). Intentional neglect. Frontiers in Bioscience, 9, 694–705. Luria, A. R. (1965). Two kinds of motor perseveration in massive injury to the frontal lobes. Brain, 88, 1–10. Sandson, J., & Albert, M. L. (1987). Varieties of perseveration. Neuropsychologia, 22, 715–732.

Activa® ▶ Deep Brain Stimulator (Parkinsons)

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Active Limb Activation

Active Limb Activation S ARAH A. R ASKIN Trinity College Hartford, CT, USA

Synonyms Limb activation

Active Memory ▶ Short-Term Memory

Activities of Daily Living (ADL) A NGELA K. T ROYER Baycrest Centre for Geriatric Care Toronto, Ontario, Canada

Definition Active limb activation is a rehabilitation technique for individuals with unilateral neglect. In a series of studies, Robertson and North (1992, 1993, 1994) and others (Mattingly, Robertson, & Driver, 1998) have demonstrated that moving the upper or lower extremity on the affected side can reduce neglect symptoms. The effect is seen only with active movement, as opposed to passive movement, and only when the limb is moved in the effected hemispace. However, the limb need not be observed visually. It should be noted that the effect has not been demonstrated universally (e.g., Brown, Walker, Gray, & Findlay, 1999).

Cross References ▶ Attention Training ▶ Behavioral Inattention Test ▶ Cognitive Rehabilitation ▶ Neglect Syndrome

References and Readings Brown, V., Walker, R., Gray, C., & Findlay, J. (1999). Limb activation and the rehabilitation of unilateral neglect: Evidence of task-specific effects. Neurocase, 5, 129–142. Mattingly, J., Robertson, I., & Driver, J. (1998). Modulation of covert visual attention by hand movement: Evidence from parietal extinction after right hemisphere damage. Neurocase, 4, 245–253. Robertson, I., & North, N. (1992). Spatio-motor cueing in unilateral left neglect: The role of hemispace, hand and motor activation. Neuropsychologia, 30, 553–563. Robertson, I., & North, N. (1993). Active and passive activation of left limbs: Influence on visual and sensory neglect. Neuropsychologia, 31, 293–300. Roberson, I., & North, N. (1994). One hand is better than two: Motor extinction of left hand advantage in unilateral neglect. Neuropsychologia, 32, 1–11.

Synonyms Adaptive functions; Functional abilities

Definition Activities of daily living (ADLs) are self-care activities that are important for health maintenance and independent living. ADLs comprise a broad spectrum of activities, traditionally classified as basic and instrumental ADLs (BADLs and IADLs, respectively). BADLs, also called physical or self-maintenance ADLs, are life-sustaining self-care activities such as feeding, grooming, bathing, dressing, toileting, and ambulation. IADLs are more complex activities that are necessary for independent living, such as using the telephone, preparing meals, shopping, managing finances, taking medications, arranging appointments, and driving. These activities are important for participating in one’s usual work, social, or leisure roles.

Historical Background The evolution of the concept of ADLs is reflected in the development of instruments to measure these abilities (McDowell & Newell, 1996). Measures of BADLs were first developed in the 1940s and 1950s, primarily out of the needs to assess fitness for military duty in World War II and to determine the required levels of care for institutionalized older adults and those with chronic illnesses. These early measures include the PULSES profile, the Barthel Index, and the Katz Index of ADL, among others. Later, in the 1960s and 1970s, there was increased interest in caring for older and disabled individuals in the community, and this spawned the need for tools to

Activities of Daily Living (ADL)

measure IADLs that are important for independent living. Some of the first of these measures were Lawton and Brody’s IADL Scale and the Disability Interview Schedule.

Current Knowledge ADLs are of interest across various health disciplines. Current knowledge in this area is based on research conducted by psychologists, occupational therapists, nurses, psychiatrists, neurologists, and social workers, among others.

Relevance to Neuropsychology For the neuropsychologist, an understanding of the patient’s level of independence in ADLs, and in particular IADLs, is of interest for several reasons. The diagnosis of a number of cognitive and mental disorders requires an appraisal of the patient’s functional ability (American Psychiatric Association, 2000). For example, impairment in adaptive or functional ability is a diagnostic criterion for mental retardation and for schizophrenia. Impaired daily functioning is also required for the diagnosis of dementia and is one of the defining differences between dementia (in which IADLs are impaired) and mild cognitive impairment (in which IADLs are intact or minimally affected). Increasingly, the evaluation of daily functioning is also used to identify appropriate treatments for cognitive and mental disorders. In particular, an important part of determining the effectiveness of behavioral or pharmacological interventions is measuring the impact of the intervention on the patient’s daily functional ability, in addition to cognitive or affective outcomes.

Assessment of ADLs Assessment of ADLs can be accomplished in a number of ways. Real-world observation of the patient in his or her own home provides relevant, objective information about daily function. However, this method is obviously time and labor intensive, and there are practical limits to the number of behaviors that can be observed within a given time period. An alternative is the use of performance-based measures, which require the patient to complete functional tasks – such as preparing a meal, using the telephone, or making personal financial transactions – that are presented in a standardized way in the laboratory or clinic. A number of such instruments have been developed to measure single or multiple functional domains. Tests

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include the Direct Assessment of Functional Status, the Independent Living Scales, the Structured Assessment of Independent Living Skills, the Medication Management Abilities Assessment, and many others. The use of questionnaires administered either on paper or by interview allows the sampling of a large number of behaviors in a short period of time. Self-report questionnaires may be appropriate for use with cognitively-normal or mildly impaired populations. In the evaluation of dementia and other cognitive disorders, however, selfreported abilities may be difficult to interpret because of disease-related decreases in self-awareness. The use of informant-based questionnaires avoids this limitation, although informants can also be biased in their reports and may not always be available. Nevertheless, this is one of the most common methods for measuring IADLs, and a large number of informant-based questionnaires exist, such as the Lawton-Brody IADL Scale, the Bristol ADL Scale, and the ADL questionnaire. The choice of which particular method of assessment to be used will depend, in addition to practical considerations such as time, on the purpose of the assessment. Real-word observations and performance-based measures provide information about what the person is capable of doing. Questionnaires, on the other hand, measure what the individual is actually doing in his or her dayto-day life.

Future Directions Although there are a large number of relevant instruments that have been developed to assess ADLs, they vary in terms of how well their psychometric properties have been characterized. Systematic literature reviews (e.g., Moore, Palmer, Patterson, & Jeste, 2007; Sikkes, de Lange-de Klerk, Pijnenburg, Scheltens, & Uitdehaag, 2009) indicate that, for many of these measures, there is a need for better theoretical justification of the content of the instrument, additional information about test validity and reliability, indication of what constitutes a meaningful change over time, information about the relation between test performance and actual real-world functioning, and the development of comprehensive normative data.

Cross References ▶ Adaptive Behavior ▶ Basic Activities of Daily Living (B-ADL) ▶ Functional Status

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Activities of Daily Living Questionnaire

▶ Instrumental Activities of Daily Living (I-ADL) ▶ Lawton-Brody iADL Scale

References and Readings American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: Author. Law, M., Baum, C., & Dunn, W. (Eds.). (2001). Measuring occupational performance: Supporting best practice in occupational therapy. Thorofare, NJ: Slack. Lawton, M. P., & Brody, E. M. (1969). Assessment of older people: Selfmaintaining and instrumental activities of daily living. Gerontologist, 9, 179–186. McDowell, I., & Newell, C. (1996). Measuring health: A guide to rating scales and questionnaires (2nd ed.). New York: Oxford. Moore, D. J., Palmer, B. W., Patterson, T. L., & Jeste, D. V. (2007). A review of performance-based measures of functional living skills. Journal of Psychiatric Research, 41, 97–118. Sikkes, S. A. M., de Lange-de Klerk, E. S. M., Pijnenburg, Y. A. L., Scheltens, P., & Uitdehaag, B. M. J. (2009). A systematic review of Instrumental Activities of Daily Living scales in dementia: room for improvement. Journal of Neurology, Neurosurgery, and Psychiatry, 80, 7–12. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests (3rd ed.). New York: Oxford.

Activities of Daily Living Questionnaire J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms ADLQ

Description The activities of daily living questionnaire (ADLQ) was developed to measure the functional abilities of people with dementia. It is an informant-rated questionnaire and should be completed by the patient’s primary caregiver. It consists of 28 items covering both basic and instrumental activities of daily living, organized into six subscales:

self-care activities; household care; employment and recreation; shopping and money; travel; and communication. The informant rates the subject’s competence in each area according to a set of four descriptions of different competence levels; scores range from 0 to 3 where higher scores indicate greater impairment. A fifth response option, ‘‘don’t know/has never done’’ is also available, and if this option is selected, the item is excluded from scoring. Scores from individual items are summed (with adjustment for any items marked ‘‘don’t know/has never done’’) to form subscale scores and then transformed to a percentage impairment total score. Scores of 0–33% are classified as no/mild impairment, those of 34–66% as moderate impairment, and those of 67–100% as severe impairment.

Historical Background The first reported use of the ADLQ was in a longitudinal study looking at cognitive test performance and daily functioning in patients with Alzheimer’s disease (Locascio, Growdon, & Corkin, 1995). However, the development and psychometric properties of the measure were first reported in Johnson, Barion, Rademaker, Rehkemper, and Weintraub (2004). Since then, a Chinese version has been developed and evaluated (ADLQ-CV; Chu & Chung, 2008), and it has been used in several studies involving people with non-Alzheimer’s dementia.

Psychometric Data Johnson et al. (2004) collected ADLQ data from the primary caregivers of 140 people with dementia of various types (Alzheimer’s disease, vascular/mixed, and frontotemporal/primary progressive aphasia). The scale was completed twice, with a 1 year interval between completions. Evidence of convergent validity was in the form of correlations with global severity ratings (clinical dementia rating r = 0.5 and 0.55 for first/second ratings, respectively; MMSE r = 0.42 and 0.38 for first and second ratings, respectively). Further evidence of its validity came from the finding that scores declined significantly over the year-long interval between testings, as would be expected in people with degenerative conditions. A subgroup of 28 participants took part in a test–retest reliability study, with a 2–8 week interval between testings (mean 25.6 days, SD 12.2). Correlations between first and second ratings for the six subscales were high, between 0.86 and 0.92, with the exception of the employment subscale, which correlated at 0.65. Kappa scores for 25% of scale

Activity Restrictions, Limitations

items were 0.42–0.60 (classified as ‘‘moderate’’), for 54% of scale items were 0.61–0.80 (classified as ‘‘good’’), and for 21% of scale items 0.81–1.0 (classified as ‘‘very good’’). The validity of the ADLQ was investigated via correlations between 29 participants’ scores on the ADLQ and the record of independent living (RIL), another ADL measure. In line with Johnson et al.’s predictions, there were significant correlations between the ADLQ and the ‘‘activities’’ and ‘‘communication’’ subscales of the RIL, but not the ‘‘behavior’’ subscale of the RIL. Chu and Chung (2008) conducted a study examining the psychometric properties of a Chinese translation of the ADLQ (ADLQ-CV), with 125 caregivers of people with moderate Alzheimer’s disease. The ADLQ-CV was shown to have good internal consistency (a = 0.81), test–retest reliability at a 2-week interval (intra-class correlation (ICC) ¼ 0.998), and inter-rater reliability (ICC ¼ 0.997, for primary and secondary caregiver ratings). Correlations with the disability assessment for dementia were strong (r = 0.92), suggesting that it is a valid measure. A factor analysis also confirmed that the ADLQ-CV has a six-factor structure, following the six proposed subscales.

Clinical Uses The ADLQ may be used to assist in the diagnosis of dementia, in decision making regarding necessary intervention and/or assistance, and in monitoring change over time or in response to treatment.

Cross References ▶ Alzheimer’s Disease Cooperative Study ADL Scale ▶ Bristol Activities of Daily Living Scale ▶ Disability Assessment for Dementia ▶ Lawton-Brody ADL Scale

References and Readings Chu, T. K. C., & Chung, J. C. C. (2008). Psychometric evaluation of the Chinese version of the activities of daily living questionnaire (ADLQ-CV). International Psychogeriatrics, 20, 1251–1261. Johnson, N., Barion, A., Rademaker, A., Rehkemper, G., & Weintraub, S. (2004). The activities of daily living questionnaire: A validation study in patients with dementia. Alzheimer’s Disease and Associated Disorders, 18, 223–230. Locascio, J. J., Growdon, J. H., & Corkin, S. (1995). Cognitive test performance in detecting, staging, and tracking Alzheimer’s disease. Archives of Neurology, 52(11), 1087–1099.

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Activity Restrictions, Limitations B RIAN YOCHIM University of Colorado at Colorado Springs Colorado Springs, CO, USA

Definition This idea refers to restrictions prescribed by clinicians who treat patients with recent strokes, head injuries, or other neurological conditions, after a neurological event has left the patient with deficits in important areas of functioning. Patients are often restricted from driving, cooking, managing finances, or completing other instrumental activities of daily living after a neurological event. The activities of focus must be tailored to the patient and can range from restrictions in playing professional sports to restrictions in managing small amounts of cash.

Current Knowledge Rehabilitation professionals encounter patients whose injuries have left them with deficits both in physical and cognitive realms. Strokes and traumatic brain injuries can cause physical impairments in walking, swallowing, use of an arm and/or leg, communication, and other important skills. Injuries can also lead to cognitive deficits in memory, executive functioning, social functioning, language, visuospatial skills, attention, and/or processing speed. These basic deficits in turn lead to impaired functioning in everyday life. Rehabilitation professionals must assess patients’ abilities to complete these daily activities and often must place restrictions on what activities patients can continue to complete. If patients are deemed to be unable to drive, for example, clinicians must follow appropriate legal and ethical channels to protect the patient and public. These limitations in activities can lead to difficulties in adjustment for the patient, which can sometimes result in depressed mood and other affective symptoms. This notion is related to the Activity Restriction Model of Depressed Affect (Williamson & Shaffer, 2000), which has been studied as one etiology of depressive symptoms among older adults.

Cross References ▶ Instrumental Activities of Daily Living (IADLs) ▶ Recommendation

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Activity Therapy

References and Readings Greenwood, R. J., Barnes, M. P., McMillan, T. M., & Ward, C. D. (Eds.). (2003). Handbook of neurological rehabilitation (2nd ed.). New York: Psychology Press. Mills, V. M., Cassidy, J. W., & Katz, D. I. (Eds.). (1997). Neurologic rehabilitation: A guide to diagnosis, prognosis, and treatment planning. Malden, MA: Blackwell Science. Williamson, G. M. & Shaffer, D. R. (2000). The activity restriction model of depressed affect: Antecedents and consequences of restricted normal activities. In G. M. Williamson, D. R. Shaffer, & P. A. Parmelee (Eds.), Physical illness and depression in older adults: A handbook of theory, research, and practice. New York: Kluwer Academic/Plenum Publishers.

Activity Therapy

require voluntariness and instead the actus reus is viewed in light of the severity of the offense.

Cross References ▶ Insanity ▶ Insanity Defense ▶ Mens Rea

References and Readings Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (1997). Psychological evaluations for the courts: A handbook for mental health professionals and lawyers. New York: Guilford.

▶ Recreational Therapy

Acute Brain Failure Actus Reus

▶ Delirium

M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

Acute Brain Syndrome Definition Actus reus is Latin for ‘‘guilty act.’’ Under most circumstances, a crime consists of at least two factors. The first factor is the physical conduct or act associated with the crime, which is known as the ‘‘actus reus.’’ In order for an individual to be convicted of a crime, it must be demonstrated beyond a reasonable doubt, that the defendant committed the physical act of the crime, or the ‘‘actus reus.’’ However, it must concurrently be established that the defendant also possessed ‘‘mens reas,’’ which translates to ‘‘guilty mind’’ referring to the mental element of the crime. Thus, a conviction necessitates, beyond reasonable doubt, establishment of an illegal act coupled with a particular mental state (e.g., intent, knowledge, recklessness, or negligence). Description of the actus reus is typically classified into one of three categories: commissions, omissions, and/or commonwealth. Commission refers to an affirmative act; omission refers to a failure to act; and commonwealth refers to a state of affairs, or circumstances. Commissions and omissions necessitate causation; commonwealth does not always

▶ Metabolic Encephalopathy

Acute Cerebrovascular Attack ▶ Stroke

Acute Confusional State ▶ Delirium ▶ Metabolic Encephalopathy

Acute Coronary Syndrome ▶ Myocardial Infarction

Acute Lymphoblastic Leukemia

Acute Encephalopathy ▶ Delirium ▶ Toxic-Metabolic Encephalopathy

Acute Febrile Polyneuritis ▶ Guillain–Barre´ Syndrome

Acute Infective Polyneuritis ▶ Guillain–Barre´ Syndrome

Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) ▶ Guillain–Barre´ Syndrome

Acute Lymphoblastic Leukemia J ACQUELINE L. C UNNINGHAM The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms ALL

Definition Acute lymphoblastic leukemia (ALL) is a form of cancer of the white blood cells (leukocytes). ALL is the most common type of childhood leukemia, and is distinguished from chronic lymphoblastic leukemia (CLL) and acute myeloid (or myelogenous) leukemia, which are more prevalent in adults.

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Current Knowledge Symptoms ALL is characterized by the rapid proliferation of immature blood cells (lymphoblasts), which crowd out mature, functional cells. It is associated with the enlargement of lymphoid tissue in areas including the lymph nodes, spleen, bone marrow, and lungs, and with increased lymphocytic cells circulating in blood and in various tissues and organs. Persons afflicted will experience weakness and fatigue, anemia, unexplained fever and infections, weight loss, or loss of appetite.

Pathophysiology Cancer, including ALL, is caused by damage to DNA.

Treatment The earlier the ALL is detected, the more effective is its treatment. The goal is to induce a lasting remission, considered to be a prevalence of less than 5% of lymphoblasts in bone marrow. Advances made in the ability to match the genetic properties of the blast cells to treatment options, in association with the availability of new drugs and improvements made in bone marrow and stem cell transplantation, have changed the prognosis for ALL from a zero to a 75% survival rate over the past 40 years. Most (if not all) patients with a childhood history of ALL have brain atrophy. Whereas atrophy is associated with treatment-effects of cranial irradiation therapy and intrathecal chemotherapy (usually methotrexate), it can also occur as a result of the condition, itself, rather than as an outcome of treatment, as it appears to cause atrophy of the brain, which is not specific to certain brain tissues (Lucy Rorke, MD, personal communication). Nonetheless, the strongest detrimental impacts on cognition are attributable to treatment-effects and their damaging influence on the biological substrates of core neurocognitive abilities, including executive functions and information processing. Such impacts disrupt the secondary abilities, i.e., those that are acquired and knowledge-based. The main approaches to alleviating neurocognitive effects of treatment include cognitive remediation, pharmacology, and ecological alterations in the classroom.

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Acute Myelogenous Leukemia

Cross References ▶ Acute Myelogenous Leukemia ▶ Leukemia ▶ Neoplasms

References and Readings Butler, R. W., & Mulhern, R. K. (2005). Neurocognitive interventions for children and adolescents surviving cancer. Journal of Pediatric Psychology, 30, 65–78. Crosley, C. J., Rorke, L. B., Evans, A., & Nigro, M. (1978). Central nervous system lesions in childhood leukemia. Neurology, 28, 678–685. Prassopoulos, P., Carouras, D., Golfinopoulos, S., Evlogias, N., Theodoropoulos, V., & Panagiotou, J. (1996). Quantitative assessment of cerebral atrophy during and after treatment in children with acute lymphoblastic leukemia. Investigational Radiology, 12, 749–754. Pui, Ching-Hon (2003). Treatment of acute leukemias: New directions for clinical research. New York: Humana Press.

Acute Myelogenous Leukemia J ACQUELINE L. C UNNINGHAM The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms Acute myeloid leukemia; AML

Definition Acute myelogenous leukemia (AML) is a form of cancer of the white blood cells (leukocytes). It is a relatively rare cancer that occurs more commonly in adults than in children, with more men affected than women. The median age at diagnosis is 63 years.

out mature, functional cells. In AML, the cell type is granuloid, whose cancerous change disrupts its normal ability to form red cells, some types of white cells, and platelets. Resulting symptoms are anemia, easy bruising and bleeding, and disruption to the body’s ability to resist infection. Impaired cognition and fatigue are also strongly associated with AML. Whereas impairments in these areas have been attributed to effects of chemotherapy, recent research by Meyers, Albitar, and Estey (2005) has identified differing cytokine levels present prior to chemotherapy as also contributing to these symptoms.

Pathophysiology The malignant cell in AML is the myeloblast, a mutated and immature cell in the granulocytic series, which undergoes combinations with other mutations, to produce a leukemic clone of cells. Because the process contributes to much diversity and heterogeneity in cell differentiation, the diagnosis of AML can be challenging. It remains important, however, since the chromosomal structure of the leukemic cells is the disease’s most critical prognostic factor.

Treatment Treatment in AML consists primarily of chemotherapy, with the goal of achieving remission. Without postremission (consolidation) therapy, almost all patients eventually relapse. Neurocognitive and neuropsychiatric symptoms are highly prevalent in patients with cancer and cause significant impairments in their ability to function. Whereas such impairments are known to be associated with aggressive cancer treatment, they are additionally attributed to biologic mechanisms underlying the cancer itself. Recent research (Meyers et al., 2005) on AML has made linkages between cytokineimmunologic activation and factors including cognitive functioning, significant fatigue, and quality of life in AML patients studied prior to the initiation of treatment.

Current Knowledge Cross References Symptoms Acute forms of leukemia are characterized by the rapid proliferation of immature blood cells which rapidly crowd

▶ Acute Lymphoblastic Leukemia ▶ Leukemia ▶ Neoplasms

Acute Respiratory Distress Syndrome

References and Readings Meyers, C. A., Albitar, M., & Estey, E. (2005). Cognitive impairment, fatigue, and cytokine levels in patients with acute myelogenous leukemia or myelodysplastic syndrome. Cancer, 104, 788–793. Pui, C.-H. (2003). Treatment of acute leukemias: New directions for clinical research. New York: Humana Press.

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Acute Respiratory Distress Syndrome D ONA EC LOCKE Mayo Clinic Scottsdale, AZ, USA

Synonyms

Acute Myeloid Leukemia

Adult respiratory distress syndrome; Respiratory distress syndrome

▶ Acute Myelogenous Leukemia

Definition

Acute Radiation Somnolence J ACQUELINE L. C UNNINGHAM The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Acute respiratory distress syndrome (ARDS) is the presence of pulmonary edema in the absence of volume overload or depressed left ventricular function, and is characterized by the development of sudden breathlessness within hours to days of an inciting event. ARDS is not a specific disease; instead, it is a type of severe, acute lung dysfunction that is associated with a variety of diseases and trauma.

Definition Acute radiation somnolence is a relatively transient and benign effect of cranial irradiation. It is manifested as sleepiness occurring during irradiation used to treat brain tumors. It occurs in both children and adults and usually affects daily functioning during the course of treatment. Although it is self-limiting, and resolves with medication and with the termination of irradiation, symptoms can be upsetting to patients. Nursing intervention which focuses on preparation through counseling and education serves to alleviate distress. Acute radiation somnolence is usually treated with steroids.

Cross References ▶ Radiation Oncology ▶ Radiotherapy

References and Readings Brady, L. W., Heilmann, H. P., Molls, M., & Schlegel, W. (2006). New techniques in radiation oncology. New York: Springer.

Historical Background In the past, ARDS signified adult respiratory distress syndrome to separate this from infant respiratory distress syndrome seen in premature infants. However, this type of pulmonary edema can also occur in children, so ARDS has gradually evolved to mean acute rather than adult.

Current Knowledge ARDS typically develops within 12–48 h after the inciting event, although, in rare instances, it may take up to a few days. Persons developing ARDS are critically ill, often with multi-system organ failure. It is a life-threatening condition; therefore, hospitalization is required for prompt management. ARDS is associated with severe and diffuse injury to the alveolar-capillary membrane (the air sacs and small blood vessels) of the lungs. Fluid accumulates in some alveoli of the lungs, while some other alveoli collapse. This alveolar damage impedes the exchange of oxygen and carbon dioxide, which leads to a reduced concentration of oxygen in the blood. Low levels of oxygen in the blood cause damage to other vital organs of the body such as the kidneys.

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Acute Respiratory Distress Syndrome

The 1994 American–European Consensus Committee defines ARDS as the acute onset of bilateral infiltrates on chest radiography, a partial pressure of arterial oxygen (PaO2) to fraction of inspired oxygen (FIO2) ratio of less than 200 mmHg and a pulmonary artery occlusion pressure of less than 18, or the absence of clinical evidence of left arterial hypertension. The mortality rate is approximately 30–40%. Death usually results from multi-system organ failure rather than lung failure alone. Causes: A number of clinical conditions are associated with the development of ARDS.

Sepsis and the systemic inflammatory response syndrome (SIRS) are the most common conditions associated with the development of ARDS. Severe traumatic injury (especially multiple fractures), severe head injury, and pulmonary contusion are strongly associated with the development of ARDS. In traumatic injury, factures of the long bones can cause ARDS through fat embolism. In severe brain injury, ARDS is thought to develop owing to a sudden discharge of the sympathetic nervous system, which then leads to acute pulmonary hypertension and injury to the pulmonary capillary bed. In pulmonary contusions, ARDS develops through direct trauma to the lung. Multiple blood transfusions are an independent risk factor for ARDS. The risk is independent of the reason for the transfusion or the coexistence of trauma. The incidence of ARDS increases with the number of units of blood transfused. If the patient has pre-existing abnormal liver functioning or a coagulation abnormality, the risk is further increased. Near drowning can be another cause of ARDS. Development of ARDS is slightly more common with saltwater than with fresh-water. Aspiration leads to an osmotic gradient that favors movement of water into airspaces of the lung. Aspiration may be visible with chest radiography, although the chest radiograph may be normal early in the course of the disease. Smoke inhalation is another possible cause of ARDS. Smoke inhalation causes lung tissue damage from direct heat, toxic chemicals, and particulate matter carried into the lung. Patients with smoke inhalation initially may be asymptomatic, but patients with airway burns, exposure to toxic fumes, or exposure to carbon monoxide should be monitored closely for the development of ARDS, even if the symptoms are initially absent. Overdoses of narcotics, tricyclic antidepressants, and other sedatives have been associated with the development of ARDS. Overdoses of tricyclic antidepressants

are the most common. This risk is independent of the risk from concurrent aspiration. Medical Treatment for ARDS:

People with ARDS require hospitalization and treatment in an intensive care unit. There is no specific treatment for ARDS, but rather, treatment is primarily supportive using a mechanical respirator and supplemental oxygen. Diuretics can be given to eliminate fluid from the lungs. However, fluids are often given via IV to provide nutrition and prevent dehydration, but fluids must be carefully monitored to avoid fluid accumulation in the lungs. Antibiotic therapy may be administered to treat infection, which is often the underlying cause of ARDS. Corticosteroids may sometimes be given late in the process of ARDS or if the patient is in shock. If the patient is in shock, drugs to counteract low blood pressure caused by shock may be administered. If the patient is experiencing anxiety, this can be treated with anti-anxiety medications.

Respiratory therapists may see these patients to provide inhaled drugs to decrease inflammation and provide respiratory comfort. Because of the acute and medically serious nature of ARDS, it would be unlikely for neuropsychological exam to be requested when a person is acutely ill with ARDS. Mortality with ARDS is 30–40% and the person would typically be treated in an Intensive Care Unit. If the person survives, outpatient neuropsychological evaluation could be requested and results may show memory deficits related to the hypoxia as well as neuropsychological deficits related to the underlying medical cause for ARDS (e.g., severe TBI, near drowning, sepsis, medication overdose).

Cross References ▶ Anoxia ▶ Hypoxia

References and Readings Bernard, G. R., Artigas, A., Brigham, K. L., Charlet, J., Falke, K., Hudson, L, Lamy M., Legall, J. R., Morris, A., & Spragg, R. (1994). Report of the American-European consensus conference on ARDS: Definitions, mechanisms, relevant outcomes and clinical trial coordination. Intensive Care Medicine, 20, 225–232.

Adaptive Behavior Assessment System – Second Edition

ADA ▶ American’s with Disabilities Act of 1990

Adaptation ▶ Tachyphylaxis

Adaptive Behavior Assessment System – Second Edition T HOMAS OAKLAND University of Florida Gainesville, FL, USA

Synonyms ABAS; ABAS-II

Description The Adaptive Behavior Assessment System – Second Edition (ABAS-II; Harrison & Oakland, 2003) provides an assessment of adaptive behavior and skills for persons from birth through age 89. Five forms are available: parent/primary caregiver form (for ages 0–5), teacher/ day-care provider form (for ages 2–5), parent form (for ages 5–21), teacher form (for ages 5–21), and an adult form (for ages 16–89). Its standardization sample is large (>4,000) and representative of US data from 1999 to 2000 with respect to gender, race/ethnicity, and parental education, and it is proportional to individuals with disabilities. Forms are available in French-Canadian and Spanish. The scales have been adapted for use in Sweden and Taiwan, with plans for extensions to the Czech Republic, Denmark, Germany, Romania, and Spain.

Historic Background The ABAS (Harrison & Oakland, 2000) preceded the development of the ABAS-II. The ABAS was developed

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to be a measure of adaptive behavior consistent with current definitions (e.g., those promulgated by the American Psychiatric Association’s (2000) Diagnostic and Statistical Manual of Mental Disorders and the American Association on Intellectual and Developmental Disabilities’ (AAIDD, 1992) models of adaptive behavior) that underscored the importance of ten skill areas: communication, community use, functional academics, health and safety, home or school living, leisure, selfcare, self-direction, social, and work skills. The ABAS norm groups were large and included persons 5 through 89. The ABAS was revised shortly after its publication in response to two issues: a need for the downward extension of the ABAS for younger children and a change in the concept of adaptive behavior embodied in AAIDD’s 2002 definition, one that emphasized the importance of three domains (e.g., conceptual, social, and practical). The ABAS-II is the only scale of adaptive behavior consistent with models of adaptive behavior advocated by the AAIDD’s 1992 and 2002 definitions and the American Psychiatric Association’s (2000) Diagnostic and Statistical Manual of Mental Disorders. Scaled scores for 11 adaptive skill areas are provided (Table 1). Ten skill area scores combine to produce standard scores in their respective domains: conceptual (communication, functional academics, and self-direction), social (social skills and leisure), and practical (self-care, home or school living, community use, health and safety, and work for adults); motor skills are assessed for young children. A General Adaptive Composite Score is derived from the skill scores.

Item Data All items are scored on a four-point scale: 0 (cannot perform the behavior), 1 (can perform the behavior yet does not), 2 (performs the behavior sometimes), and 4 (performs the behavior most or all of the time). This feature is consistent with the World Health Organization’s International Classification of Functioning (Mpofu & Oakland, 2010) effort to distinguish activities and performance. Respondents may indicate that they guessed. Data from subtests with more than three guesses should not be used. The ABAS-II’s scoring and reporting system informs clinicians of interventions likely to promote the development of selected behaviors associated with critical items.

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Adaptive Behavior Assessment System – Second Edition. Table 1 Adaptive skills and three adaptive domains Adaptive skills Communication

Speech, language, and listening skills needed for communication with other people, including vocabulary, responding to questions, and conversation skills

Community use

Skills needed for functioning in the community, including use of community resources, shopping skills, and getting around in the community

Functional academics

Basic reading, writing, mathematics, and other academic skills needed for daily, independent functioning, including telling time, measurement, as well as writing notes and letters

Home living

Skills needed for basic care of a home or living setting, including cleaning, straightening, property maintenance and repairs, as well as food preparation and performing chores

Health and safety Skills needed for protection of health and to respond to illness and injury, including following safety rules, using medicines, and showing caution Leisure

Skills needed for engaging in and planning leisure and recreational activities, including playing with others, engaging in recreation at home, and following rules in games

Self-care

Skills needed for personal care including eating, dressing, bathing, toileting, grooming, and hygiene

Self-direction

Skills needed for independence, responsibility, and self-control, including starting and completing tasks, keeping a schedule, following time limits, following directions, and making choices

Social

Skills needed to interact socially and get along with other people, including having friends, showing and recognizing emotions, assisting others, and using manners

Work

Skills needed for successful functioning and holding a part-time or full-time job in a work setting, including completing work tasks, working with supervisors, and following a work schedule

Motor skillsa

Basic fine and gross motor skills needed for locomotion, manipulation of the environment, and the development of more complex activities such as sports, including sitting, pulling up to a standing position, walking, fine motor control, and kicking

Three domains and associated skill areas Conceptual

Includes communication, functional academics, self-direction, and health and safety skills

Practical

Includes social skills and leisure skills

Social

Includes self-care, home/school living, community use, health and safety, and work skills

a

Although fine and gross motor development is not included as one of the ten skills identified by the American Association on Intellectual and Developmental Disabilities, it is included in some scales of adaptive behavior.

Psychometric Data Scaled scores generally range from 40 to 120. Consistent with all measures of adaptive behavior, the ABAS-II is more sensitive to the assessment of adaptive behavior and skills at the lower than the higher ranges. Cut scores are not provided by disability category; instead, reliance is placed on diagnostic standards established by state and national authorities. The ABAS-II demonstrates suitable psychometric qualities. Internal consistency is high, with reliability coefficients of 0.85–0.99 for the General Adaptive Composite, three adaptive behavior domains, and skill areas. Test–retest reliability coefficients are in the 0.80s and 0.90s for the General Adaptive Composite, three domains, and skill areas (Harrison & Oakland, 2003). Inter-rater reliability coefficients (e.g.,

between teachers, day-care providers, and parents) range from the 0.60s to the 0.80s for the skill areas and are in the 0.90s for the General Adaptive Composite. Its construct validity is strong as displayed through factor analyses (Harrison & Oakland, 2003; Wei, Oakland, & Algina, 2008). Its concurrent validity with the Vineland Adaptive Behavior Scales – Classroom Edition’s Adaptive Behavior Composite is high, r = 0.82 (Harrison & Oakland, 2003). See reviews by Burns (2005), Meikamp and Suppa (2005), and Rust and Wallace (2004) for additional details.

Clinical Uses Measures of adaptive behavior have been most important in assessment of persons with mental retardation (now

Addiction

referred to as intellectual disabilities by AAIDD). The ABAS-II is useful in this diagnosis as well as in intervention planning and monitoring for this and other disorders. The ABAS-II also may assist in promoting an understanding of the impact on a person’s daily life activities of other disorders (e.g., those often diagnosed first during infancy or early childhood include autism, disorders of attention, communication, conduct, elimination, feeding and eating, learning, motor skills, and pervasive developmental disorders; Harman, Smith-Bonahue, & Oakland, 2009; Oakland & Harrison, 2008). The ABAS-II is useful with children and adolescents who display disorders including attention deficit/hyperactivity, acquired brain injury, auditory or visual impairment, autism, developmental delays, emotional/behavioral disorders, learning disabilities, and physical impairments (Ditterline, Banner, Oakland, & Becton 2008; Harrison & Oakland, 2003; Oakland & Harrison, 2008). Adults diagnosed with such disorders as anxiety, acute stress or adjustment disorder, bipolar disorder, depression, mood disorders, psychosis, Parkinson’s, postpartum depression, substance abuse, schizophrenia, and sleep disturbance may display impairments in their functional daily living skills. Older adults diagnosed with Alzheimer’s type dementia and other cognitive and neuropsychological disorders with late-life onset often display impairments in their functional daily living skills. Although data from the ABAS-II may not be crucial in the diagnosis of some of these disorders, ABAS-II data will promote an understanding of their impact on daily living skills. The ABAS-II is used in the assessment of mental retardation among death row inmates in light of the 2002 US Supreme Court Atkins decision (Olley & Cox, 2008).

of support (10th ed.). Washington, DC: American Association on Mental Retardation. American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: American Association on Mental Retardation. Burns, M. K. (2005). Review of the adaptive behavior assessment system – second edition. In R. Spies & B. Plake (Eds.), The sixteenth mental measurements yearbook. Lincoln, NE: Buros Institute of Mental Measurements. Ditterline, J., Banner, D., Oakland, T., & Becton, D. (2008). Adaptive behavior profiles of students with disabilities. Journal of Applied School Psychology, 24, 191–208. Harman, J., Smith-Bonahue, T., & Oakland, T. (2009). Assessment of adaptive behavior development in young children. In E. Mpofu & T. Oakland (Eds.). Rehabilitation and Health Assessment: Applying ICF Guidelines. New York: Springer. Harrison, P. & Oakland, T. (2000). Adaptive behavior assessment system. San Antonio, TX: Harcourt Assessment. Harrison, P. & Oakland, T. (2003). Adaptive behavior assessment system – second edition. San Antonio, TX: Harcourt Assessment. Meikamp, J., & Suppa, C. H. (2005). Review of the adaptive behavior assessment system – second edition. In R. Spies & B. Plake (Eds.), The sixteenth mental measurements yearbook. Lincoln, NE: Buros Institute of Mental Measurements. Mpofu, E. & Oakland, T. (2010). Assessment in rehabilitation and health. Upper Saddle River, NJ: Merrill. Oakland, T. & Harrison, P. (2008). Adaptive behavior assessment system-II: Behavior assessment system-II: Clinical use and interpretation. New York: Elsevier Olley, J. G., & Cox, A. (2008). Assessment of adaptive behavior in adult forensic cases: The use of the ABAS-II. In T. Oakland, & P. Harrison, (Eds.), Adaptive behavior assessment system-II: Clinical use and interpretation. Boston: Elsevier Rust, J. O. & Wallace, M. A. (2004). Test review: Adaptive behavior assessment system – second edition. Journal of Psychoeducational Assessment, 22, 367–373. Wei, Y., Oakland, T., & Algina, J. (2008). Multigroup confirmatory factor analysis for the parent form, ages 5–21, of the adaptive behavior assessment system-II. American Journal on Mental Retardation. 113, 178–186.

Adaptive Functions Cross References ▶ Activities of Daily Living ▶ Activity Restrictions and Limitations ▶ Adaptive Behavior ▶ Intellectual Disabilities

▶ Activities of Daily Living (ADL)

ADD ▶ Attention Deficit, Hyperactivity Disorder ▶ Minimal Brain Dysfunction

References American Association on Intellectual and Developmental Disabilities. (1992). Definitions, classifications, and systems of supports (9th ed.). Washington, DC: American Association on Mental Retardation. American Association on Intellectual and Developmental Disabilities. (2002). Mental retardation: Definition, classification, and systems

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Addiction ▶ Substance Abuse Disorders

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Adelaide Activities Index

Adelaide Activities Index ▶ Frenchay Activity Index

Adenoma E THAN M OITRA Drexel University Morgantown, WV, USA

ADHD, Combined ▶ Attention Deficit, Hyperactivity Disorder ▶ Minimal Brain Dysfunction

ADHD, Predominantly Hyperactive-impulsive Type ▶ Attention Deficit, Hyperactivity Disorder ▶ Minimal Brain Dysfunction

Definition A benign tumor of glandular origin. There are three types of adenomas: tubular (most common; tube-like structure), villous (least common; most likely to become cancerous; ruffled structure), and tubulovillous (blend of tubular and villous structures). Adenomas do not metastasize, though they can develop into malignancies known as adenocarcinomas. The tumor may occur throughout the endocrine system, including the pituitary gland. Pituitary adenomas occur at a much higher incidence in adults than in children. Because their invasiveness is local, they are almost always benign and can be difficult to detect. There is the secreting and the nonsecreting type. Clinical symptoms come from the endocrine dysfunction or from mass effect, and include headaches, hypopituitarism, and visual loss (caused by compression in the optic chiasm). Treatment of pituitary adenomas includes correction of electrolyte dysfunction, replacement of pituitary hormones, surgical resection, and radiotherapy.

Cross References ▶ Pituitary Adenoma

ADHD, Predominantly Inattentive Type ▶ Attention Deficit, Hyperactivity Disorder ▶ Minimal Brain Dysfunction

ADI-R ▶ Autism Diagnostic Interview, Revised

ADLQ ▶ Activities of Daily Living Questionnaire

Admissibility M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

References and Readings Mazzaferri, E. L., & Saaman, N. A. (Eds.) (1993). Endocrine tumors. Boston: Blackwell Scientific Publications.

ADHD ▶ Attention Deficit, Hyperactivity Disorder ▶ Minimal Brain Dysfunction

Definition Admissibility of evidence refers to any testimonial, documentary material, or other form of tangible evidence that can be considered by the trier of fact, most typically a judge or a jury, in the context of a judicial or administrative proceeding. In order for evidence to be admissible, it must be relevant, non-prejudicial, and possess some indicia of reliability. For example, if

Adoption Studies

evidence consists of a witness testimonial, it must be established that the witness is credible and that he/she has knowledge of that which he/she is declaring. For neuropsychologists, a central issue is the admissibility of one’s data and opinions. Rules 401, 402, and 702–705 from Article VII of the Federal Rules of Evidence (FRE) relate to ‘‘Opinions & Expert Testimony.’’ Perhaps of most relevance to psychologists is rule FRE 702 which states, ‘‘If scientific, technical, or other specialized knowledge will assist the trier of fact to understand the evidence or to determine a fact in issue, a witness qualified as an expert by knowledge, skill, experience, training or education, may testify thereto in the form of an opinion or otherwise.’’ In other words, the expert should possess some form of knowledge that a typical judge or juror would not be expected to know or understand. Rule 703 states, ‘‘The facts or data in the particular case upon which an expert bases an opinion or inference may be those perceived by or made known to the expert at or before the hearing. If of a type reasonably relied upon by experts in the particular field in forming opinions or inferences upon the subject, the facts or data need not be admissible in evidence in order for the opinion or the inference to be admitted. Facts or data that are otherwise inadmissible shall not be disclosed to the jury by the proponent of the opinion or inference unless the court determines that their probative value in assisting the jury to evaluate the expert’s opinion substantially outweighs their prejudicial effect.’’ Several important cases have addressed the admissibility of scientific testimony. In the case of Frye v. United States (1923), the Frye standard was established which stated that: only scientific methods and concepts with ‘‘general acceptance’’ within a particular field are admissible. In the more recent case of Daubert v. Merrell Dow (1993), it was determined that scientific testimony has to abide by two criteria, the testimony must be: (a) scientifically valid and (b) relevant to the case at hand.

Cross References ▶ Daubert v. Merrell Dow Pharmaceuticals (1993)

References and Readings A complete list of the Federal Rules of Evidence is available at: http:// judiciary.house.gov/media/pdfs/printers/108th/evid2004.pdf.

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Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan, & J. Ricker (Eds.). Textbook of clinical neuropsychology. New York: Taylor & Francis. Jenkins v. United States, 307 F. 2d 637 (1962). Kaufmann, P. M. (2008). Admissibility of neuropsychological evidence in criminal cases: Competency, insanity, culpability, and mitigation. In R. Denney, & J. Sullivan (Eds.). Clinical neuropsychology in the criminal forensic setting. New York: Guilford.

Admissibility of Psychological Evidence ▶ Jenkins v. U.S. (1962)

Admissibility of Psychological/ Neuropsychological Evidence ▶ Baxter v. Temple (2005)

Adoption Studies R OHAN PALMER 1, M ARTIN H AHN 2 1 University of Colorado Boulder, CO, USA 2 William Paterson University Wayne, NJ, USA

Definition Adoption studies typically compare pairs of persons, e.g., adopted child and adoptive mother or adopted child and biological mother to assess genetic and environmental influences on behavior.

Current Knowledge Design Familial resemblance of behaviors is due to genetic and/or common familial environmental influences. Adoption studies provide a direct test of the role of both factors. This is possible by drawing comparisons between families that share genetic and environmental influences and

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families that share only genetic or environmental factors. Adoption creates two types of families. The ‘‘genetic family’’ consists of pairs of genetically related individuals who do not share a common family environment (e.g., biological parent and adopted-away child). The similarity between these pairs of relatives provides a direct estimate of genetic effects on behaviors. The second type family is the ‘‘environmental family,’’ which is made up of pairs of individuals who are not genetically related but who share a common family environment (e. g., adoptive parent and adopted child). The similarity between pairs of relatives from an ‘‘environmental family’’ indicates the presence of environmental influences on behavior. Adoption studies utilize either parent–offspring pairs or sibling pairs. Because data on biological parents and siblings of adoptees are sometimes rare, comparison between ‘‘genetic-plusenvironmental’’ families (i.e., intact families) and adoptive families also provides evidence of genetic and environmental influences.

Relevance to Neuropsychology The Colorado Adoption Project has been collecting longitudinal data on biological and adoptive parents and their biological or adopted children for over 30 years (Petrill, Plomin, DeFries, & Hewitt, 2003). In one set of analyses from that project reported by Plomin, Fulker, Corley, and DeFries (1997), parent–offspring correlations were calculated for children aged 3–16 years. The results of the analyses show increasing correlations across those ages between biological parents and their adopted-away children on such special cognitive abilities as verbal skills and perceptual speed. Correlations between adoptive parents and adopted children remained about zero across those ages. The authors interpret the results to indicate that heritability increases for those special cognitive abilities with age and that the role of shared environment is low or nonexistent. Today, adoption study data are used to assess the genetic and environmental influence on a variety of clinical outcomes that include drug addiction (Young, Rhee, Stallings, Corley, & Hewitt, 2006) and age of sexual initiation (Bricker et al., 2006), to name a few.

References and Readings Bricker, J. B., Stallings, M. C., Corley, R. P., Wadsworth, S. J., Bryan, A., Timberlake, D. S., et al. (2006). Genetic and environmental influences on age at sexual initiation in the Colorado adoption project. Behavior Genetics, 36, 820–832. Petrill, S. A., Plomin, R., DeFries, J. C., & Hewitt, J. K. (2003). Nature, nurture, and the transition to early adolescence. Oxford: Oxford University Press. Plomin, R., Fulker, D. W., Corley, R., & DeFries, J. C. (1997). Nature, nurture, and cognitive development from 1 to 16 years: A parentoffspring adoption study. Psychological Science, 8, 442–447. Young, S. E., Rhee, S. H., Stallings, M. C., Corley, R. P., & Hewitt, J. K. (2006). Genetic and environmental vulnerabilities underlying adolescent substance use and problem use: General or specific? Behavior Genetics, 36, 603–615.

ADOS ▶ Autism Diagnostic Observation Schedule

Adrenal Hormones ▶ Minimal Brain Dysfunction

Adrenaline ▶ Epinephrine

Adrenergic Agonists ▶ Catecholamines

Adrenocorticotropic Hormone DAVID J. L IBON Drexel University, College of Medicine Philadelphia, PA, USA

Definition Cross References ▶ Twin Studies

Adrenocorticotropic hormone (ACTH) is produced by the anterior pituitary gland and is a component of the

Advanced Progressive Matrices

hypothalamic-pituitary-adrenal axis. The release of ACTH is associated with the biological response to stress. The production of ACTH from the pituitary gland stimulates the adrenal glands to produce cortisol. The ACTH stimulation test is a common procedure used to assess the integrity of the adrenal glands. This test is used to identify a number of medical conditions including adrenal insufficiency, Addison’s disease, and related medical conditions (Melmed & Kleinberg, 2008).

Cross References ▶ Hypothalamus

References and Readings Melmed, S., & Kleinberg, D. (2008). Anterior pituitary. In H. M. Kronenberg, S. Melmed, K. S. Polonsky, & P. R. Larsen (Eds.), Williams textbook of endocrinology (11th edn.). Philadelphia, PA: Saunders Elsevier.

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Description First developed in the 1940s as an additional form of the Raven’s progressive matrices, the advanced progressive matrices (APM) were developed to test intellectual efficiency in people with greater than average intellectual ability, and to differentiate clearly between people of superior ability. A nonverbal test of inductive reasoning, the APM contains 48 items, presented as one set of 12 (Set I), and another of 36 (Set II). As in the standard version of the test (SPM), items are presented in black ink on a white background, and become increasingly difficult as progress is made through each set. Although it is an untimed task, some clinicians administer the APM under time constraints. Set II can be used without a time limit to assess the examinee’s total reasoning capacity. In this case, the examinee would first be shown the problems of Set I as examples to explain the principles of the test, and would then be given approximately 1 h to complete the task. Alternately, Set I can be given as a short practice test followed by Set II as a speed test. In this case, 40 min is the time limit most commonly given for Set II.

Historical Background

Adult Respiratory Distress Syndrome ▶ Acute Respiratory Distress Syndrome

Advanced MS ▶ Secondary-Progressive Multiple Sclerosis

The APM was designed in the 1940s to assess nonverbal abstract conceptualization skills of individuals for whom the standard version was too easy; that is, those achieving a raw score of 50 or above on the SPM. For children over 10 years of age with high intellectual functioning, the APM may be the appropriate version to ensure an adequate ceiling (Mills, Ablard, & Brody, 1993). For additional information about the historical background of the original test, please refer to the entry for Raven’s Progressive Matrices.

Psychometric Data

Advanced Progressive Matrices V ICTORIA M. L EAVITT Kessler Foundation Research Center West Orange, NJ, USA

Synonyms APM

Norms for adolescents (ages 12–16.5) and adults (18–68+; Sets I and II) for untimed (ages 12–70+) and timed (ages 17–28) versions are provided for North America (Raven, Raven Court, 1998).The reliability of the test is considered good, with high internal consistency of APM Set II, and split-half reliability coefficients varying between 0.83 and 0.87 (Strauss, Sherman, & Spreen, 2006). Set I, as it has only 12 items, yields lower figures. Reliability of the original 48-item version was found to be high for adults and children aged 11.5 years+ (>0.80); for younger

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children, it was only reasonably reliable (0.76). Overall, Set II scores increased by three points on retest (Raven et al., 1998).

Clinical Uses The SPM and CPM have been found to be sensitive to a variety of neurological and neuropsychiatric conditions (▶ Raven’s Progressive Matrices). The APM, designed for use with higher functioning individuals, may be more appropriately employed for assessing an individual’s capacity for decision-making or strategic planning at the management level in the workplace or in a higher education setting.

Cross References ▶ Colored Progressive Matrices ▶ Raven’s Progressive Matrices ▶ Standard Progressive Matrices

References and Readings Mills, C. J., Ablard, K. E., & Brody, L. E. (1993). The Raven’s progressive matrices: Its usefulness for identifying gifted/talented students. Roeper Review, 15, 183–186. Raven, J. C. (1965, 1994). Advanced progressive matrices sets I and II. Oxford: Oxford Psychologists Press. Raven, J., Raven, J. C., & Court, J. H. (1996). Progressive matrices: A perceptual test of intelligence. Individual form. Oxford: Oxford Psychologists Press. (Original work published 1938) Raven, J., Raven, J. C., & Court, J. H. (1998). Raven manual: Section 4. Advanced progressive matrices. Oxford: Oxford Psychologists Press Ltd. Raven, J., Raven, J. C., & Court, J. H. (2003). Manual for Raven’s progressive matrices and vocabulary scales. Section 1: General overview. San Antonio, TX: Harcourt Assessment. Strauss, E., Sherman, E. M. S., Spreen, O. (Eds.). (2006). A compendium of neuropsychological tests (3rd ed.). NY: Oxford University Press.

Advocacy A MY J. A RMSTRONG Virginia Commonwealth University Richmond, VA, USA

Synonyms Advocate; Support

Definition The process of supporting or acting on behalf of a cause; facilitating equal community access and participation of individuals or groups that have typically been socially and/or economically marginalized. There are several types of advocacy to include: Systems Advocacy: the process in which any system (public, private, community based) is made more responsive to the needs of the individual served by the system. This process may include increasing awareness of services and resources available within a community; identifying unmet needs of individuals; identifying existing barriers that impede access to community services and resources; developing strategies to eliminate legislative, regulatory, social and economic barriers that may impede access to one’s community supports and resources. Individual Advocacy: the process of increasing awareness of unmet needs and procuring rights or benefits on behalf of another individual or group of individuals. Self-Advocacy: the process of empowering an individual to rely upon him or herself to make his/her own choices and decisions in order to direct the course of his/her life. The People First movement of the 1970s was a progenitor of self-advocacy as a civil rights movement. The independent living movement also fostered self-advocacy and provided a foundation for self-advocacy activism.

Cross References ▶ Americans with Disabilities Act ▶ Independent Living

References and Readings Dell Orto, A. E., & Marinelli, R. P. (Eds.) (1995). Encyclopedia of disability and rehabilitation. New York: MacMillian Publishing. Test, D., Fowler, C. H., Wood, W. M., Brewer, D. M., & Eddy, S. (2005). Conceptual framework of self-advocacy for students with disabilities. Remedial and Special Education, 26, 43–54. Wehmeyer, M. L. (2004). Self-determination and the empowerment of people with disabilities. American Rehabilitation, 28, 22–29.

Advocate ▶ Advocacy

Affective Disorder

Adynamia I RENE S HULOVA -P IRYATINSKY Butler Hospital Providence, RI, USA

Synonyms Asthenia

Definition Adynamia refers to a general weakness and lack of energy evident through lack of verbal or overt behavior due to a disease or neurological conditions. It can manifest as lethargy, loss of strength, weakness in extremities, and difficulty initiating activities or completing tasks. Adynamia can be observed after trauma to the frontal lobes, multiple sclerosis, and other conditions. In language, verbal adynamia (lack of spontaneity of speech) is seen with lesions of the medial frontal lobes and refers to difficulty in initiation and maintenance of language output.

Cross References ▶ Abulia ▶ Apathy ▶ Transcortical Motor Aphasia

Definition Affect is the display and experiencing of emotion. It includes positive dimensions such as joy, interest, and contentment, as well as negative dimensions of emotion such as disgust, fear, and anger. Affect is a very rapid response to internal (e.g., thoughts, memory) or external stimuli (e.g., other people). It is different from mood, in that it is more momentary and observable by others, whereas mood is longer-lasting and constitutes a symptom that patients may report (e.g., depression). Affect can be observed from facial expression, gestures, posture, and speech (e.g., word choice, tone, rate).

Cross References ▶ Affective Disorder ▶ Emotions ▶ Mood Disorder

References and Readings Batson, C. D., Shaw, L. L., & Oleson, K. C. (1992). Differentiating affect, mood and emotion: Toward functionally-based conceptual distinctions. Emotion. Newbury Park, CA: Sage. Blechman, E. A. (1990). Moods, affect, and emotions. Hillsdale, NJ: Lawrence Erlbaum Associates. Ekman, P. (1993). An argument for basic emotion. Cognition and Emotion, 6, 169–200.

References and Readings Berrios, G. E. (2008). Classic text no. 76: ‘Asthenia’ by A. Dechambre (1865). History of Psychiatry, 19(4), 490–501. Caplan, D. (1987). Neurolinguistics and linguistic aphasiology: An introduction. New York: Cambridge University Press.

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Affect Display ▶ Affect

Affective Disorder

J OEL W. H UGHES Kent State University Kent, OH, USA

J OEL W. H UGHES Kent State University Kent, OH, USA

Synonyms

Synonyms

Affect display

Emotional disorder; Mood disorder

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Affective Disorder

Short Description or Definition Affective disorder is a mental disorder predominantly characterized by altered mood that results in a significant impairment in social, occupational, or other important area of functioning. Affective disorders include depressive disorders such as major depressive disorder, minor depressive disorder, and dysthymia, as well as manic disorders such as bipolar disorder and cyclothymic disorder. Affective disorders may be primary or caused by medical conditions or substances.

Categorization Mania and depression seem to anchor the ends of an emotional and behavioral continuum, an observation that dates from ancient times. In Hippocrates’ humoral theory, mania resulted from an excess of yellow bile, and depression to an excess of black bile. In the early twentieth century, German psychiatrist Emil Kraepelin described affective disorders as belonging to a manic–depressive form of psychosis, which he differentiated from dementia praecox. The term ‘‘manic depression’’ was replaced by more contemporary language, including major depressive disorder and bipolar disorder in the twentieth century, and, for example, major depressive disorder was first incorporated into the third edition of the Diagnostic and Statistical Manual (DSM-III). Depressive disorders include major depressive disorder and dysthymic disorder. Diagnosis of major depressive disorder is made on the basis of symptoms, as there is no physiological test that reliably diagnoses depression. Major depressive disorder requires at least 2 weeks of depressed mood and/or loss of interest in usually pleasurable activities (anhedonia). In addition, at least four of the following seven symptoms must also be present; significant weight gain or loss or appetite changes, sleep disturbance (e.g., early morning awakening with difficulty returning to sleep), observable disturbances in psychomotor speed (increased or diminished), loss of energy or excessive fatigue, feelings of low self-worth or inappropriate guilt, cognitive changes such as the subjective experience of difficulty concentrating, and thinking about or planning suicide. Dysthymia is similar to major depressive disorder, although the depression must be chronic (i.e., two or more years of depressed mood), and during the first 2 years of the dysthymia, there must not have been an episode of major depression or a period of longer than 2 months with no symptoms.

Bipolar disorder is diagnosed when there is a ‘‘manic’’ mood disturbance characterized by markedly expansive, elevated, or irritable mood, lasting at least 1 week. The mood disturbance must be accompanied by additional symptoms such as grandiosity, excessive risky behavior such as sexual behavior or irresponsible spending, and decreased need for sleep. A ‘‘mixed’’ episode denotes mood disturbances that are characterized by both manic and depressive symptoms. A ‘‘hypomanic’’ episode is a less-pronounced elevation of mood that would not qualify as a true manic episode. Bipolar disorders follow a course in which periods of elevated mood alternate with periods of depression, and are categorized according to the nature of these episodes. For example, Bipolar I involves alternating manic and depressive episodes; in Bipolar II, there are alternating hypomanic and depressive episodes; cyclothymic disorder involves alternating hypomanic and depressive episodes that do not meet full criteria for major depression.

Epidemiology Affective disorders are very common. At any one time, approximately 10% of the adult population, or nearly 20 million Americans, have a depressive illness. Rates of depression are even higher in patients with comorbid medical conditions, and, for example, about 30% of patients with cardiac disease have clinically significant depression. Bipolar disorder is much less common than unipolar depression, occurring in between 2% and 4% of the population (including Bipolar I, Bipolar II, and cyclothymic disorder). While depression is twice as common among women as men, bipolar is equally common in men and women.

Natural History, Prognostic Factors, and Outcomes Affective disorders often start in adolescence. For example, the onset of Bipolar disorder is typically 15–24 years of age. However, the most likely ages for a first major depressive episode are 30–40 years of age. Depressive disorders often remit spontaneously, but recurrence is common, and about 15% of individuals experiencing an initial major depressive episode will develop chronic recurrent depression. The bipolar disorders are highly heritable, and research continues to determine genetic risk markers for bipolar disorder. Brain imaging studies also suggest that a broad risk for unstable moods may underlie

Affective Spectrum Disorders

bipolar disorder, but more research is warranted. The causes of depression are not fully understood, but appear to involve the interaction of genetic and environmental factors such as stress and disruptions in interpersonal relationships. Thus, in addition to female gender, risk factors for depression include severe life stress such as traumatic events and loss of significant relationships. Depression is associated with shorter life expectancy from suicide and other causes of death. For example, depression increases risk of cardiac disease, as well as risk of mortality among individuals with cardiac disease.

Neuropsychology and Psychology of Affective Disorder Depression is common in neurological conditions such as stroke and traumatic brain injury (Robinson, 2006; Rosenthal, Christensen, & Ross, 1998). Even without an obvious neurologic insult, individuals with alterations in executive control, memory, and emotion regulation are at increased risk for depression. Furthermore, individuals with depression often show neuropsychological deficits in the absence of neurological conditions. The neuropsychological deficits specified in the diagnostic criteria for depression include difficulty concentrating and making decisions. Thus, depressed patients often exhibit deficits in executive control, memory, and processing speed. For bipolar disorder, distractibility is typically present, as well as impaired decision making reflected in the criterion relating to distractibility of excessive involvement in activities that present significant risk of negative consequences. Current neuropsychological theories of depression emphasize the frontal lobes and basal ganglia, including abnormalities in neural circuitry involving the prefrontal cortex, mesiotemporal cortex, striatum, amygdala, and thalamus (Chamberlain & Sahakain, 2006). These areas may also be implicated in bipolar disorder, as they appear to underlie mood symptoms and treatment effects.

Evaluation Assessment of affective disorders focuses on self-report instruments and clinical interviews. Neuropsychological testing may reveal deficits in executive function, attention psychomotor slowing, and biases in the processing of emotional stimuli. Specifically, depressed individuals

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have exaggerated responses to negative feedback, including rumination. Neuropsychological evaluations in depression and bipolar disorder are used frequently in research, as tests with broad clinical utility in the context of assessing or treating affective disorders have not been widely disseminated.

Treatment Depression is often treated with medication and/or psychotherapy. A large number of medications are available to treat depression, including selective serotonin reuptake inhibitors, which typically have relatively milder side effects and lower risks than older drugs such as monoamine oxidase inhibitors. Treatment of bipolar disorders requires pharmacotherapy. In contrast to major depressive disorder, bipolar cannot be successfully treated by psychotherapy alone.

Cross References ▶ Depressive Disorder

References and Readings Allen, L. B., McHugh, R. K., & Barlow, D. H. (2008). Emotional disorders: A unified protocol. In D. H. Barlow (Ed.), Clinical handbook of psychological disorders: A step-by-step treatment manual (4th ed.) (pp. 216–249). New York: Guilford Press. American Psychiatric Association (2000). Diagnostic and statistical manual of mental disorders (4th ed.). Text Revision. Washington, DC: American Psychiatric Association. Chamberlain, S. R., & Sahakain, B. J. (2006). The neuropsychology of mood disorders. Current Psychiatry Reports, 8, 458–463. Clark, L., Chamberlain, S. R., & Sahakian, B. J. (2009). Neurocognitive mechanisms in depression: Implications for treatment. Annual Review of Neuroscience, 32, 57–74. Robinson, R. (2006). The neuropsychiatry of stroke (2nd ed.). New York: Cambridge University Press. Rosenthal, M., Christensen, B., & Ross, T. (1998). Depression following traumatic brain injury. Archives of Physical Medicine and Rehabilitation, 79, 90–103.

Affective Spectrum Disorders ▶ Unexplained Illness

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Afferent

Afferent J OHN B IGBEE Virginia Commonwealth University Richmond, VA, USA

Synonyms

Cross References ▶ Lemniscal System

References and Readings Luria, A. L. (1976). The working brain: An introduction to neuropsychology. New York: Perseus Books Group.

Sensory

Age Decrements Definition Afferent is an anatomical term that indicates functional directionality. In nervous tissue, afferent is often used synonymously with sensory information when it refers to nerves carrying impulses from peripheral receptors toward the central nervous system. Afferent can also be used in general to refer to any connection coming into a structure within the nervous system. The opposite direction of conduction is efferent.

Afferent Paresis M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

Synonyms Age-associated cognitive decline

Definition The concept of age decrements in neuropsychology refers to a decline in cognitive performance due to normal aging rather than due to an extraneous or internal event that is known to negatively affect cognitive performance, such as a traumatic brain injury, stroke, psychiatric symptoms, and extensive drug use history.

Current Knowledge Definition A deficit in the ability to perform voluntary movements due to loss of kinesthetic feedback. The primary and secondary motor cortices have extensive inputs from the somatosensory areas in the parietal lobes. Following lesions to this latter area, particularly the post-central gyrus or to the lemniscal system which provides proprioceptive information to it, motor difficulties may be observed either in the limbs or in speech production. Although the muscles involved in such activities are not weak per se, the loss of sensory information results in a disruption of motor control and an imprecise excitation of muscle groups required to execute specific, voluntary fine-motor responses.

Variability in the performance of aging individuals adds complexity to the determination of specific age decrements on neuropsychological tests. It is generally thought that individuals are more likely to retain ‘‘crystallized’’ knowledge (e.g., that which is practiced, overlearned, and skill-based) than ‘‘fluid’’ knowledge (e.g., problemsolving). As there are factors that heighten the risk for age decrements, protective factors may counteract the risk. For instance, higher levels of education and positive health status may slow down the rate of cognitive decline that would otherwise occur with increasing age. One concept that illustrates age decrements is AgeAssociated Memory Impairment (AAMI), which pertains to age-related decline in performance specifically in terms of memory.

Ageusia

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Cross References

Current Knowledge

▶ Cognitive Reserve ▶ Memory Impairment

Agenesis can result from various etiologies, including genetic predisposition, chromosomal abnormalities, or intrauterine trauma, such as infection. When present, this condition is commonly associated with other neuroanatomical anomalies, metabolic disturbances, and/or neurobehavioral deficits. The latter might include mental retardation, seizures, motor deficits, and psychiatric disturbances. However, some patients may be relatively asymptomatic, the callosal defect being discovered only serendipitously late in life. The latter is more likely to occur when the agenesis is not accompanied by other neurological or metabolic defects. Whereas ‘‘disconnection syndromes’’ are routinely present following surgical commissurotomy for intractable epilepsy, they are generally not present with agenesis.

References and Readings Lezak, M. D. (2004). Neuropsychological assessment (4th Ed.). New York: Oxford University Press.

Age Equivalent ▶ Mental Age


Age-associated Cognitive Decline ▶ Age Decrements ▶ Mild Cognitive Impairment

Age-Associated Memory Impairment (AAMI)

Aicardi, J., Chevrie, J.-J., & Baraton, J. (1987). Agenesis of the corpus callosum. In P. J. Vinken, G. W. Bruyn, & H. L. Klawans (Eds.), Handbook of clinical neurology (Vol.50, pp. 149–173). New York: Elsevier. Marszal, E., Jamroz, E., Pilch, J., Kluczewska, E., Jablecka, H., & Krawczyk, R. (2000). Agenesis of the corpus callosum: Clinical description and etiology. Journal of Child Neurology, 15, 401–405. Zaidel, E., & Iacoboni, M. (2003). The parallel brain: The cognitive neuroscience of the corpus callosum. Cambridge, MA: MIT Press.

▶ Benign Senescent Forgetfulness

Ageusia Agenesis of Corpus Callosum J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition A developmental defect in which either all or part of the corpus callosum fails to develop.

M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Definition Ageusia is the loss of the sense of taste. The disorder should be distinguished from a disruption in the ability to perceive flavor, which requires a combination of olfactory, gustatory, and somatosensory functions. Frequently, complaints of ageusia are often explained by olfactory

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Aggravating Factors

dysfunction rather than a disruption in taste perception, per se. The majority of taste receptors (buds) are located on the tongue and this information is carried by the VIIth (anterior two thirds) and IXth (posterior third) cranial nerves, with other taste receptors (cranial nerve X) located in other regions of the mouth and throat. These taste fibers enter the solitary nucleus (rostral portion) in the upper medulla and from there second-order neurons travel to the ventral posterior medial nuclei of the thalamus. Thalamic projections carrying this gustatory information then project to the post-central gyrus in the region of the parietal operculum and to the underlying insular cortex where the sensation of taste is likely experienced. Lesions of the VIIth nerve can result in loss of taste in the ipsilateral anterior two thirds of the tongue which is more readily assessable to clinical testing than lesions of the IXth or Xth nerves. However, total loss of taste (ageusia) is seldom seen as a result of structural lesions because of the multiple and bilateral pathways involved. Ageusia (or hypogeusia) is more likely to result from more systemic problems such as treatments for cancer (radiation, chemotherapy), certain types of influenza, diabetes, or certain medications. Taste acuity (hypogeusia) can decline with age and may contribute to the anorexia and weight loss often seen in elderly persons. The prognosis in acquired ageusia is often correlated directly with the expected course of the illness or injury causing the dysfunction.

Cross References ▶ Taste

Aggravating Factors R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition Refers to any relevant circumstances in correspondence with the evidence presented during the trial that, from the perspective of the jurors, makes the harshest penalty appropriate. By contrast, mitigating factors refer to evidence regarding the defendant’s character or circumstances related to the crime that would provide foundation for a juror to vote for a lesser sentence.

Historical Background In 1972, the U.S. Supreme Court considered the death penalty to be a cruel and an unusual punishment because the manner in which capital sentences were decided in Georgia was capricious (Furman v. Georgia, 1972). This decision discontinued death penalty litigation in the USA at that time because none of the states had a system that was substantially different. In 1976 (Gregg v. Georgia), the Court accepted as constitutional Georgia’s rewrite of their statute which included a capital sentencing process that required presentation before a judge or jury of aggravating and mitigating factors. It required at least one or ten specified aggravating circumstances to be established beyond reasonable doubt to impose the death penalty. Some examples include: whether the crime (murder) was particularly cruel and atrocious, if more than one victim was murdered, whether the murder occurred during the commission of a felony, etc.


Current Knowledge

Cerf-Ducastel, Van de Moortele, P.-F., MacLeod, P. Le Bihan, D., & Faurion, A. (2001). Interaction of gustatory and lingual somatosensory perceptions at the cortical level in the human: A functional magnetic resonance imaging study. Chemical Senses, May 1, 26(4), 371–383. Doty, R. L., & Kimmelman, C. P. (1992). Lesser20R.P. Smell and taste and their disorders. In A. K. Asbury, G. M. McKhann, & W. I. McDonald (Eds.), Diseases of the nervous system (2nd ed., pp. 390–403). Philadelphia, PA: W.B. Saunders. Wilson-Pauwek, L., Akesson, E., & Stewart, P. (1988). Cranial nerves: Anatomy and clinical comments. Philadelphia, PA: B.C. Decker.

Laws regarding how aggravating or mitigating factors should be weighed by jurors vary based on state laws. Neuropsychological assessments in death penalty cases typically focus on mitigating factors, such as neuropsychological or neurobehavioral impairments, as there is an increased body of evidence demonstrating a preponderance of neurocognitive deficits in violent criminals. Neuropsychological assessment with respect to aggravating factors is less common and typically addresses increased risk of future dangerousness.

Agitated Behavior Scale

Cross References ▶ Mitigating Factors

References and Readings Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Furman v. Georgia, 408 U.S. 238 (1972). Gregg v. Georgia, 49 L.Ed.2d. 859 (1976). Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts: A Handbook for mental health professionals and lawyers. (3rd ed.). New York: Guilford Press.

Agitated Behavior Scale DANIEL N. A LLEN University of Nevada Las Vegas, Nevada, USA

Synonyms ABS

Description The agitated behavior scale (ABS) was designed to evaluate agitation and other problematic behaviors that commonly occur during the acute recovery phase following traumatic brain injury (Corrigan, 1989). The ABS is composed of 14 items that represent a number of commonly occurring problematic behaviors such as short attention span, impulsivity, uncooperativeness, violence, and angry outbursts. Information that assists in completing the ABS, including descriptions of the behaviors and ratings for each item, as well as examples, is available with the author (Corrigan). Each item is rated on a 1–4-point scale based on intensity of the behavior or frequency of its occurrence. Additionally, when assigning ratings, the degree to which the behavior interferes with functional behavior is also considered. If the behavior is absent a rating of 1 is assigned. When the behavior is present a rating of 2 or greater is assigned, with a rating of 4 indicating that the behavior is present to an extreme degree. A total score is derived by summing across all 14 items (range 14–56) with scores less than 22 in the normal range, scores of 22–28 indicating mild agitation,

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29–35 moderate agitation, and 35–56 severe agitation. Subscale scores can also be calculated for disinhibition, aggression, and lability although it appears that ABS primarily measures a single construct (Bogner et al., 2000), so that the total score may be most appropriate when interpreting test results.

Current Knowledge The ABS is often used to perform serial assessments to track changes in agitation that occur as a natural part of the recovery process and as a result of treatment. Although designed with traumatic brain injury in mind, the ABS has also been used to assess agitation in other populations, such as patients with progressive dementia (Corrigan, Bogner, and Tabloski, 1996; Tabloski, McKinnon-Howe, and Remington, 1995). No differences have been found between males and females with brain injury on the total score or the subscale scores (Kadyan et al., 2004). Internal consistency estimates range from 0.74 to 0.92 (Bogner et al., 1999; Corrigan, 1989), with interrater reliability of 0.92 for the total score, and with comparable reliabilities of 0.90, 0.91, and 0.73 for the disinhibition, aggression, and lability scores, respectively. Subscale to total score correlations range from 0.43 to 0.55. The construct validity of the ABS has been supported by factor-analytic studies that demonstrated the presence of three factors representing disinhibition, aggression, and lability (Corrigan and Bogner, 1994). ABS scores account for a substantial portion of the variance (from 36% to 62%) in independent observations of agitation (Corrigan, 1989) and are able to predict changes in cognition (Corrigan and Mysiw, 1988), which provides additional support for its validity. Thus, there is evidence that the ABS is a highly practical measure with sound psychometric properties that allow for serial assessment of agitation in populations with brain injury.

Cross References ▶ Post-traumatic Confusional State ▶ Traumatic Brain Injury

References and Readings Bogner, J. A., Corrigan, J. D., Bode, R. K., & Heinemann, A. W. (2000). Rating scale analysis of the Agitated Behavior Scale. Journal of Head Trauma Rehabilitation, 15, 656–659.

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Bogner, J. A., Corrigan, J. D., Stange, M., & Rabold, D. (1999). Reliability of the Agitated Behavior Scale. Journal of Head Trauma Rehabilitation, 14, 91–96. Corrigan, J. D. (1989). Development of a scale for assessment of agitation following traumatic brain injury. Journal of Clinical and Experimental Neuropsychology, 11, 261–277. Corrigan, J. D. & Bogner J. A. (1994). Factor structure of the Agitated Behavior Scale. Journal of Clinical and Experimental Neuropsychology, 16, 386–392. Corrigan, J. D. & Mysiw, W. J. (1988). Agitation following traumatic head injury: equivocal evidence for a discrete stage of cognitive recovery. Archives of Physical Medicine and Rehabiltation, 69, 487–492. Kadyan, V., Mysiw, W. J., Bogner, J. A., Corrigan, J. D., Fugate, L. P., & Clinchot, D. M. (2004). Gender differences in agitation after traumatic brain injury. American Journal of Physical Medicine & Rehabilitation, 83, 747–752.

Agitation PAUL D. N EWMAN Drake Center Cincinnati, OH, USA

Synonyms Posttraumatic agitation

Definition Agitation is an excess of one or more behaviors that occur during the course of delirium when cognition is impaired. The behaviors most often in excess during agitation include aggression, akathisia, disinhibition, and/or emotional lability. Specific examples of agitated behavior may include pacing, hand wringing, pulling at tubes or restraints, inappropriate verbalizations, excessive crying or laughter, etc. Agitation is often conceptualized to result from an inability to cope with overstimulation. Stimulation may be internal (e.g., pain or hallucinations) or external (e.g., noise, light, or conversation). One’s ability to cope with stimulation may be viewed as a threshold. Adverse changes to the brain’s typical functioning have the potential to lower this threshold. Thus, individuals with traumatic brain injury or dementia may become agitated at lower levels of stimulation than noninjured individuals.

Current Knowledge There was no consensus on the definition of agitation within the greater health-care profession for many years. Clinicians in neuro-rehabilitation were using the term in the early 1980s to describe a pattern of behavior observed during recovery from traumatic brain injury. The development of the Agitated Behavior Scale by Corrigan and associates in the late 1980s to measure this brain-injuryrelated behavior led to a more refined definition of the term. The term is not limited to just traumatic brain injury as agitation can manifest in any setting in which an individual experiences delirium and impaired cognition (e.g., dementia). The importance of the concept of agitation and its measurement was vital to the establishment of the now accepted viewpoint that recovery from agitation is preceded by improvement in cognition. Or conversely, interventions that decrease arousal and/or cognition can lead to a worsening of agitation.

Cross References ▶ Agitated Behavior Scale ▶ Behavior Management ▶ Deescalation ▶ Dementia ▶ Frustration Tolerance ▶ Post-traumatic Confusional State ▶ Traumatic Brain Injury

References and Readings Corrigan, J. D. (1989). Development of a scale for assessment of agitation following traumatic brain injury. Journal of Clinical and Experimental Neuropsychology, 69, 261–277. Sandel, M. E., & Bysiw, W. J. (1996). The agitated brain injured patient. Part 1: Definitions, differential diagnosis, and assessment. Archives of Physical Medicine and Rehabilitation, 77, 617–623. Smith, M., Gardner, L. A., Hall, G. R., & Buckwalter, K. C. (2004). History, development, and future of the progressively lowered stress threshold: A conceptual model for dementia care. Journal of the American Geriatric Society, 52, 1755–1760.

Agnogenic Medial Arteriopathy ▶ Cadasil

Agrammatic Speech

Agnosia A NASTASIA R AYMER Old Dominion University Norfolk, VA, USA

Definition Agnosia is a failure to recognize a sensory stimulus that is not attributable to dysfunction of peripheral sensory mechanisms or to other cognitive impairments associated with brain damage (Bauer & Demery, 2003). Agnosia is often described as a percept that is ‘‘stripped of its meaning.’’ The individual can respond to the presence of the stimulus, but has difficulty processing the perceptual information in sufficient detail to make sense of and meaningfully recognize it. The stimulus can be recognized through other sensory modalities.

Cross References ▶ Apperceptive Visual Agnosia ▶ Associative Visual Agnosia ▶ Auditory Agnosia ▶ Pure Word Deafness ▶ Tactile Agnosia ▶ Visual Object Agnosia

References and Readings Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed., pp. 236–295). New York: Oxford University Press. Farah, M. J. (1990). Visual agnosia. Cambridge, MA: MIT Press. Feinberg, T. E., Rothi, L. J. G., & Heilman, K. M. (1986). Multimodal agnosia after unilateral left hemisphere lesion. Neurology, 36, 864–867. Riddoch, M. J., & Humphreys, G. W. (2001). Object recognition. In B. Rapp (Ed.), The handbook of cognitive neuropsychology (pp. 45–74). Philadelphia, PA: Psychology Press.

Current Knowledge Agnosia can occur in any perceptual modality, though it is most commonly reported to affect the visual modality (Farah, 1990). Multi-modality forms of agnosia also have been described (Feinberg, Rothi, and Heilman, 1986). Lesions associated with agnosia will vary across sensory modalities, usually affecting bilateral postRolandic cortical sensory regions or disconnecting incoming pathways from one hemisphere to the other (Bauer & Demery, 2003). Different forms of agnosia have been described that depend upon how much incoming information can be processed. Some forms (e.g., apperceptive agnosia) are associated with disruption at early stages of perceptual processing. The person cannot copy or match an incoming percept to a like stimulus and may make perceptual confusions, yet can conjure up some perceptual information from memory (e.g., visual imagery tasks) or answer questions about perceptual attributes of a stimulus. In other forms of agnosia (e.g., associative agnosia), the person can copy or match percepts, but is not able to conjure up information about perceptual characteristics of a stimulus from memory and also has difficulty appreciating the meaningfulness of a percept, its category, context, associated objects and actions. In either case, accurate processing through that perceptual modality is disrupted.

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Agonist ▶ Receptor Spectrum

Agonist Spectrum ▶ Receptor Spectrum

Agrammatic Aphasia ▶ Agrammatism

Agrammatic Speech ▶ Telegraphic Speech

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Agrammatism LYN T URKSTRA 1, C YNTHIA K. T HOMPSON 2 1 University of Wisconsin-Madison Madison, WI, USA 2 Northwestern University Evanston, IL, USA

Synonyms Agrammatic aphasia

Definition Agrammatism refers to language production that is lacking in grammatical structures. The basic signs of agrammatism are short phrase length, simplified syntax, errors and omissions of main verbs, and omission or substitution of grammatical morphemes such as plural markers or functors (Saffran, Berndt, & Schwartz, 1989). There may also be errors in tense, number, and gender, and difficulty in producing sentences with movement of grammatical elements, such as passive sentences, Wh- questions, and complex sentences (Benedet, Christiansen, & Goodglass, 1998; Caplan & Hanna, 1998; Goodglass, 1997; Faroqi-Shah & Thompson, 2004). Spoken and written production typically shows similar error patterns. Typically, individuals with agrammatic aphasia also show impaired comprehension of grammatical structures, particularly noncanonical semantically reversible sentences (e.g., ‘‘the boy was kicked by the horse’’; Berndt, Mitchum, & Haendiges, 1996; Caramazza & Zurif, 1976).

Historical Background Historically, agrammatism was thought of as a syndrome typically associated with nonfluent aphasia (Goodglass, 1997). More recent studies (e.g., Dick et al., 2001) have shown that features of agrammatism are present in the production of many individuals with various forms of aphasia, as well as in normal speakers under stressful conditions, and agrammatism is not attributable to any single site of lesion.

Some authors have argued that agrammatism reflects an underlying impairment in language representation and/or processing (Grodzinsky 1986, 1990, 1995; Zurif, Swinney, Prather, Solomon, & Bushell, 1993), while others contend that they represent the speaker’s strategic adaptation to an underlying language processing impairment that is not specific to grammar (Kolk & Heeschen, 1990; also see discussion in Beeke, Wilkinson & Maxim, 2007). Consistent with the processing deficit view, individuals with agrammatic aphasia show problems computing syntactic structures in real time (Dickey, Choy, & Thompson, 2007; Swinney, Prather, & Love, 2000; but see Blumstein et al., 1998) and also may have deficits that impact both production and comprehension, although not always the same structures (Dickey, Milman, & Thompson, 2008). Also, the structures that typically are impaired in agrammatic aphasia are similar across many languages. In support of the adaptation view, there is evidence that the grammatical structures used by individuals with agrammatic aphasia vary as a function of the task. For example, individuals with agrammatic aphasia may produce more complex sentences on standardized language tests, in which grammatical completeness is the focus, than in conversational interactions, in which the message and interaction are the focus and the communication partners are co-constructing a dialog (Beeke, Maxim, & Wilkinson, 2008). There is evidence of treatment efficacy for interventions aimed improvement of underlying representation/ processing impairments and deficits in adaptation. Verb as Core (Loverso, Prescott, & Selinger, 1986), Mapping Therapy (Schwartz, Saffran, Fink, & Myers, 1994), and Treatment of Underlying Forms (TUF; Thompson, Shapiro, Kiran, & Sobecks, 2003; Thompson, 2008) focus treatment on verbs and verb argument structure, training patients to map form to meaning in both simple and complex sentences. Notably, TUF results in strong generalization from complex to simple structures by controlling the lexical and syntactic variables of sentences trained (see Thompson & Shapiro, 2007, for review). Various approaches to treatment of grammatical morphology, such as deficits in verb tense and agreement, also have been shown to be efficacious (Faroqi-Shah, 2008; Friedmann, Wenkert-Olenik, & Gil, 2000; Mitchum & Berndt, 1994; Weinrich, Boser, & McCall, 1999).

Current Knowledge

Cross References

The underlying mechanisms of agrammatism have been debated in the literature over the past several decades.

▶ Aphasia ▶ Grammar

Agraphia

▶ Nonfluent Aphasia ▶ Paragrammatism ▶ Syntax ▶ Telegraphic Speech

References and Readings Beeke, S., Maxim, J., & Wilkinson, R. (2008). Rethinking agrammatism: Factors affecting the form of language elicited via clinical test procedures. Clinical Linguistics and Phonetics, 22(4–5), 317–323. Beeke, S., Wilkinson, R., & Maxim, J. (2007). Individual variation in agrammatism: A single case study of the influence of interaction. International Journal of Language and Communication Disorders, 42(6), 629–647. Benedet, M. J., Christiansen, J. A., & Goodglass, H. (1998). A crosslinguistic study of grammatical morphology in Spanish- and English-speaking agrammatic patients. Cortex, 34(3), 309–336. Berndt, R. S., Mitchum, C. C., & Haendiges, A. N. (1996). Comprehension of reversible sentences in ‘‘agrammatism’’: A meta-analysis. Cognition, 58(3), 289–308. Blumstein, S. E., Byma, G., Kurowski, K., Hourihan, J., Brown, T., & Hutchinson, A. (1998). On-line processing of filler-gap construction in aphasia. Brain and Language, 61, 149–168. Caplan, D., & Hanna, J. E. (1998). Sentence production by aphasic patients in a constrained task. Brain and Language, 63(2), 184–218. Caramazza, A., & Zurif, E. B. (1976). Dissociation of algorithmic and heuristic processes in language comprehension: Evidence from aphasia. Brain and Language, 3(4), 572–582. Dick, F., Bates, E., Wulfeck, B., Utman, J. A., Dronkers, N., & Gernsbacher, M. A. (2001). Language deficits, localization, and grammar: Evidence for a distributive model of language breakdown in aphasic patients and neurologically intact individuals. Psychological Review, 108(4), 759–788. Dickey, M. W., Choy, J., & Thompson, C. K. (2007). Real-time comprehension of wh-movement in apahsia: Evidence from eyetracking while listening. Brain and Language, 100, 1–22. Dickey, M. W., Milman, L. H., & Thompson, C. K. (2008). Judgment of functional morphology in agrammatic aphasia. Journal of Neurolinguistics, 21(1), 35–65. Faroqi-Shah, Y., & Thompson, C. K. (2004). Semantic, lexical, and phonological influences on the production of verb inflections in agrammatic aphasia. Brain and Language, 89(3), 484–498. Faroqi-Shah, Y. (2008). A comparison of two theoretically-driven treatments of verb inflections in agrammatic aphasia. Neuropsychologia, 46, 3088–3100. Friedmann, N., Wenkert-Olenik, D., & Gil, M. (2000). From theory to practice: Treatment of agrammatic production in hebrew based on the tree pruning hypothesis. Journal of Neurolinguistics, 13, 250–254. Grodzinsky, Y. (1986). Language deficits and syntactic theory. Brain and Language, 27, 135–159. Grodzinsky, Y. (1990). Theoretical perspectives on language deficits. Cambridge, MA: MIT Press. Grodzinsky, Y. (1995). A restrictive theory of agrammatic comprehension. Brain and Language, 50, 27–51. Goodglass, H. (1997). Agrammatism in aphasiology. Clinical Neuroscience, 4(2), 51–56. Kolk, H. H. J., & Heeschen C. (1990). Adaptation symptoms and impairment symptoms in Broca’s aphasia. Aphasiology, 4, 221–231.

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Loverso, F. L., Prescott, T. E., & Selinger, M. (1986). Cuing verbs: A treatment strategy for aphasic adults. Journal of Rehabilitation Research, 25, 47–60. Mitchum, C., & Berndt, R. (1994). Verb retrieval and sentence construction: Effects of targeted intervention. In M. J. Riddoch & G. Humphreys (Eds.), Cognitive neuropsychology and cognitive rehabilitation. Hove, Sussex: Erlbaum. Saffran, E. M., Berndt, R. S., & Schwartz, M. F. (1989). The quantitative analysis of agrammatic production: Procedure and data. Brain and Language, 37(3), 440–479. Schwartz, M. F., Saffran, E. M., Fink, R. B., & Myers, J. L. (1994). Mapping therapy: A treatment programme for agrammatism. Aphasiology, 8, 19–54. Swinney, D., Prather, P., & Love, T. (2000). The time course of lexical access and the role of context: Converging evidence from normal and aphasic processing. In Y. Grodzinsky, L. P. Shapiro, & D. Swinney (Eds.), Language and the brain: Representation and processing. New York: Academic Press. Thompson, C. K., & Shapiro, L. P. (2007). Complexity in treatment of syntactic deficits. American Journal of Speech and Language Pathology, 16, 30–42. Thompson, C. K., Shapiro, L. P., Kiran, S., & Sobecks, J. (2003). The role of syntactic complexity in treatment of sentence deficits in agrammatic aphasia: The complexity account of treatment efficacy (CATE). Journal of Speech, Language and Hearing Research, 42, 690–707. Weinrich, M., Boser, K. I., & McCall, D. (1999). Representation of linguistic rules in the brain: Evidence from training an aphasic patient to produce past tense verb morphology. Brain and Language, 70, 144–158. Zurif, E., Swinney, D., Prather, P., Solomon, J., & Bushell, C. (1993). Online analysis of syntactic processing in Broca’s and Wernicke’s aphasia. Brain and Language, 45, 448–464.

Agranular (Motor Cortex) ▶ Primary Cortex

Agraphia P E´ LAGIE M. B EESON 1,2 , S TEVEN Z. R APCSAK 1,2,3 1 Department of Speech, Language, & Hearing Sciences 2 Department of Neurology 3 Southern VA Health Care System, The University of Arizona Tucson, AZ, USA

Synonyms Written language disorders

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Short Description or Definition

Epidemiology

Agraphia is the term applied to acquired disorders of spelling or writing caused by neurological damage in individuals with normal premorbid literacy skills. There are several different agraphia profiles that variously result from impairments of spelling knowledge, sound-to-letter correspondences, letter-shape information, or motor control for handwriting. Although agraphia can occur in relative isolation, it often co-occurs with acquired impairments of reading (alexia) and spoken language (aphasia).

Agraphia is commonly observed following damage to the language-dominant left hemisphere. Although it is most frequently caused by stroke, agraphia can follow any kind of focal damage to the brain regions critical for implementing the various cognitive operations necessary for normal spelling and writing. Agraphia is also observed in individuals with neurodegenerative disorders, including those with primary progressive aphasia/semantic dementia or Alzheimer’s disease. The specific agraphia profile reflects the region of cortical damage or atrophy.

Categorization Several distinct forms of acquired agraphia occur that reflect specific combinations of impaired and preserved spelling and writing abilities following damage to certain brain regions. Spelling difficulties can result from damage to central linguistic processes supported by the languagedominant hemisphere in a manner analogous to acquired impairments of reading (▶ alexia). Agraphia can also result from disruption of peripheral processing components that guide the selection and production of appropriate letter shapes. Common central agraphia syndromes

Phonological agraphia refers to an impaired ability to manipulate the sound system of the language (phonology) which manifests as a disproportionate difficulty with the spelling of nonwords (e.g., flig, merber) compared with real words. Deep agraphia is characterized by a marked impairment of spelling ability for nonwords, as seen in phonological agraphia, but with the additional hallmark feature of semantic errors (e.g., car for vehicle). Surface agraphia (also called lexical agraphia) is characterized by relatively preserved ability to spell nonwords and regularly spelled words in the face of marked impairment of spelling words with irregular sound–letter correspondences, such as choir. Common peripheral agraphia syndromes

Allographic agraphia is an impairment of written spelling due to errors in letter selection. Apraxic agraphia is an impairment of the selection and implementation of graphic motor programs necessary to move the hand to form letter shapes. Micrographia is the production of abnormally small letters due to defective control of the force, speed, and amplitude of handwriting movements.

Natural History, Prognostic Factors, Outcomes The prognosis for recovery from agraphia depends on the etiology of the lesion and the extent of the underlying brain damage. Agraphia following stroke or traumatic brain injury tends to show some spontaneous recovery in the first months after brain damage occurs, but residual impairments often persist. Additional improvements may be achieved with behavioral treatment directed toward strengthening the weakened cognitive processes that support spelling or motor control for writing. In individuals with neurodegenerative disorders, progressive worsening of the spelling impairment is observed along with the gradual deterioration of other language and cognitive functions.

Neuropsychology and Psychology of Agraphia Written words are typically produced in response to activation of a concept in the semantic system. The motivation to write a word may be driven by the desire to convey a message, or in response to an auditory stimulus, as in the context of writing a word to dictation. As depicted in the cognitive model of single-word processing in Fig. 1, the word meaning (semantics) and the phonological word form (phonology) both provide access to spelling knowledge (orthography). In literate adults, the spellings of familiar words are easily recalled as whole words from one’s spelling vocabulary (i.e., orthographic lexicon). In contrast to this lexical approach, spellings can be assembled on the basis of the knowledge of sound-toletter correspondences using a sublexical processing strategy as depicted in Fig. 1. A sublexical approach is often employed when one is unsure about the spelling of a

Agraphia

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Orthography

Phonology Lexical Words

Words

Spoken word

Auditory analysis

Written word

Sublexical Phonemes

Letters

Motor speech programs

Graphic motor programs

Speech

Writing

Visual analysis

Agraphia. Figure 1 A cognitive model indicating the component processes involved in spelling and writing

word, or when required to spell an unfamiliar word or a nonword, such as glope. Spelling via sound–letter correspondences is likely to yield correct responses for regularly spelled words, such as drive, but over-reliance on the sublexical route will result in phonologically plausible errors for irregularly spelled words, such as kwire for choir. Thus, according to a dual-route model as depicted in Fig. 1, only the lexical route can deliver correct spellings for irregularly spelled words. The final stages of writing require translation of abstract spelling knowledge into letter shapes and selection and implementation of the graphic motor programs for the appropriate handwriting movements. The various agraphia syndromes reflect specific impairments to these component processes necessary for spelling and writing.

Phonological/Deep Agraphia Phonological agraphia is characterized by difficulty in the generation of spellings on the basis of sound-to-letter correspondences. This problem is particularly evident during clinical evaluation when an individual is asked to generate plausible spellings for nonwords. The disproportionate difficulty in spelling nonwords compared to familiar words gives rise to an exaggerated lexicality effect (Henry, Beeson, Stark, & Rapcsak, 2007; Rapcsak et al., 2009). According to a dual-route model (Fig. 1), poor nonword spelling in phonological agraphia is attributable

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to damage to the sublexical route, while the better preserved real-word spelling by these patients reflects the residual functional capacity of the lexical and semantic routes. There is evidence to suggest that phonological agraphia reflects a central impairment of phonological processing ability that is also apparent on reading tasks; however, the spelling impairment is typically of greater severity due to the fact that spelling is a harder task than reading (Rapcsak et al., 2009). Although spelling accuracy for words (both regular and irregular) is better preserved than spelling of nonwords, performance is often degraded to some extent relative to premorbid performance. Due to the reliance on lexical processing with limited sublexical input, real word spelling is typically influenced by lexicalsemantic variables such as word frequency (high > low), imageability (concrete > abstract), and grammatical class (nouns > verbs > functors). Deep agraphia includes all of the characteristic features of phonological agraphia, but it is distinguished from the latter by the production of semantic errors (e.g., husband written as wife). In essence, deep agraphia can be considered a more severe form of phonological agraphia. Like phonological/deep alexia, phonological/deep agraphia is typically encountered in patients with aphasia syndromes characterized by phonological impairment including Broca’s, conduction, and Wernicke’s aphasia. In such cases, there is damage to a network of perisylvian cortical regions involved in speech production/perception and phonological processing including Broca’s area,

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precentral gyrus, insula, Wernicke’s area, and supramarginal gyrus (Fig. 2). The contribution of these regions to phonological processing skills is evident from lesion studies, but also in functional imaging studies of healthy individuals when they perform a variety of written and spoken language tasks requiring phonological processing (Jobard et al., 2003; Vigneau et al., 2006; Rapcsak et al., 2009). In individuals with deep agraphia, the left hemisphere damage tends to be more extensive than that associated with phonological agraphia, and it has been hypothesized that the right hemisphere may be responsible for the characteristic deep agraphia profile (Rapcsak, Beeson, & Rubens, 1991).

Surface Agraphia Surface agraphia is characterized by difficulty in spelling irregular words, which contain atypical sound-to-letter correspondences. Regular words are spelled with significantly better accuracy, thus yielding a regularity effect. Nonword spelling is relatively preserved. In a manner analogous to surface alexia, a dual-route theory attributes surface agraphia to dysfunction of the lexical spelling route (Fig. 1). Specifically, it has been suggested that the spelling disorder results from damage to the orthographic lexicon (Rapcsak & Beeson, 2004). The loss of word-specific orthographic knowledge prompts reliance on a sublexical phoneme–grapheme conversion strategy that produces phonologically plausible regularization errors on irregular words, a finding that is most pronounced on low frequency items (e.g., yot for yacht). Surface agraphia may also result from damage to central semantic

representations as observed in individuals with semantic dementia (Graham, Patterson, & Hodges, 2000). The reduction in the ability to process lexical-semantic information in such individuals results in overreliance on sublexical spelling procedures and regularization errors. As expected, it is not uncommon to observe co-occurance of surface alexia and agraphia in individuals with semantic dementia (Graham et al., 2000). Surface agraphia, like surface alexia, is typically associated with extrasylvian brain pathology (Fig. 2). Focal lesions that give rise to surface agraphia have been documented in the left inferior occipito-temporal cortex (Rapcsak & Beeson, 2004). This region includes a portion of the fusiform gyrus known as the visual word form area that has been shown to be engaged in healthy adults during reading (Cohen et al., 2002) and spelling tasks (Beeson et al., 2003) and may represent the neural substrate of the orthographic lexicon. Surface agraphia has also been described following focal damage to posterior middle/inferior temporal gyrus and angular gyrus (Rapcsak & Beeson, 2002) and in patients with left anterior temporal lobe atrophy (Graham et al., 2000). In these cases, the spelling deficit may reflect damage to a distributed extrasylvian cortical network involved in semantic processing (Fig. 2).

Allographic Agraphia Allographic agraphia refers to a disturbance of the ability to activate or select appropriate letter shapes for the abstract orthographic representations generated by central spelling routes. This impairment of handwriting

Graphomotor control

Phonology

Semantics Orthography

Agraphia. Figure 2 Cortical regions involved in spelling and writing

Agraphia

is characterized by letter selection errors that often include the substitution of physically similar letter forms (e.g., b for h). The allographic difficulty may be specific to letter case (upper vs. lower) or style (print vs. cursive). When allographic agraphia occurs in isolation, oral spelling is preserved, as well as the ability to correctly arrange component letters that make up a word (i.e., anagram spelling) and typing. Allographic agraphia is often associated with damage to left temporo-parietooccipital regions.

Apraxic Agraphia Apraxic agraphia is characterized by poor letter formation in handwriting that is not attributable to allographic disorder or sensorimotor, cerebellar, or basal ganglia dysfunction. The difficulty arises at the level of motor programming for the skilled movements of the hand so that the spatiotemporal aspects of writing are disturbed. Individual letters are often difficult to recognize, and may simply appear to be meaningless scrawls. Lesions associated with apraxic agraphia have been noted in the hemisphere contralateral to the dominant hand. Thus, in right-handed individuals, the damage typically involves the left superior parietal lobe in the region of the intraparietal sulcus, the dorsolateral premotor cortex just anterior to primary motor cortex for the hand, or the supplementary motor area (Fig. 2).

Nonapraxic Disorders of Motor Execution In addition to apraxic agraphia, there are several additional disorders of motor execution that affect the ability to form legible letter shapes. These writing difficulties include disturbances of the regulation of movement force, speech, and amplitude. Micrographia (the production of small letters with reduced legibility) is a common example that is associated with the basal ganglia pathology in Parkinson disease. Cerebellar pathology can also result in poor handwriting due to irregular and disjointed hand movements. Handwriting difficulty is also associated with damage to primary sensorimotor cortex and/or associated corticospinal tracts that cause hemiparesis of the dominant hand. When the hemiparesis is marked, individuals typically shift to writing with the nondominant hand. Improvement in graphomotor control of the nondominant hand is apparent with practice and often provides a fully functional substitute; however, the automaticity of motor movements is rarely comparable to the premorbidly dominant hand.

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Evaluation Evaluation of individuals with acquired agraphia is structured so that the status of all the relevant component processes involved in spelling and writing are examined. Controlled word lists for such assessment can be found in the literature (e.g., Beeson & Henry, 2008) or in commercially available test batteries (e.g., Kay, Lesser, & Coltheart, 1992). A comprehensive battery should include regularly and irregularly spelled words as well as nonwords. The evaluation should allow the clinician to identify the nature of the functional impairment and to locate the level of breakdown with reference to a cognitive model of normal spelling. It is equally important to document relatively spared abilities and the use of compensatory strategies by the patient, as this information is helpful in planning treatment.

Treatment Several behavioral treatment approaches have shown positive outcomes in the rehabilitation of agraphia (for a recent review see Beeson & Henry, 2008). In general, treatment is directed toward strengthening impaired processes and training the use of compensatory strategies necessary to bypass the functional deficit. Because written spelling tasks inherently involve reading, behavioral treatments for spelling can also serve to strengthen reading. However, given that spelling is often significantly more impaired than reading, it is not uncommon to address spelling at a lexical level while treating reading at the text level (Beeson & Rapcsak, 2006).

Cross References ▶ Alexia ▶ Aphasia ▶ Phonological/Deep Alexia ▶ Pure Alexia ▶ Surface Alexia

References and Readings Beeson, P. M., & Henry, M. L. (2008). Comprehension and production of written language. In. R. Chapey (Ed.), Language intervention strategies in adult aphasia (5th ed., pp. 654–688). Baltimore, MD: Wolters Kluwer/Lippincott, Williams & Wilkins.

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Beeson, P. M., & Rapcsak, S. Z. (2002). Clinical diagnosis and treatment of spelling disorders. In A. E. Hillis (Ed.), Handbook on adult language disorders: Integrating cognitive neuropsychology, neurology, and rehabilitation (pp. 101–120). Philadelphia: Psychology Press. Beeson, P. M., & Rapcsak, S. Z. (2006). Treatment of alexia and agraphia. In J. H. Noseworthy (Ed.), Neurological Therapeutics: Principles and Practice (2nd ed., pp. 3045–3060). London: Martin Dunitz. Beeson, P. M., Rapcsak, S. Z., Plante, E., Chargualaf, J., Chung, A., Johnson, S. C., et al. (2003). The neural substrates of writing: A functional magnetic resonance imaging study. Aphasiology, 17, 647–665. Cohen, L., Lehe´ricy, S., Chochon, F., Lemer, C., Rivaud, S., & Dehaene, S. (2002). Language-specific tuning of visual cortex? Functional properties of the Visual Word Form Area. Brain, 125, 1054–1069. Graham, N. L., Patterson, K., & Hodges, J. R. (2000). The impact of semantic memory impairment on spelling: evidence from semantic dementia. Neuropsychologia, 38, 143–163. Henry, M. L. Beeson, P. M., Stark, A. J., & Rapcsak, S. Z. (2007). The role of left perisylvian cortical regions in spelling. Brain and Language, 100, 44–52. Jobard, G., Crivello, F., & Tzourio-Mazoyer, N. (2003). Evaluation of the dual route theory of reading: A metaanalysis of 35 neuroimaging studies. NeuroImage, 20, 693–712. Kay, J., Lesser, R., & Coltheart, M. (1992). Psycholinguistic assessments of language processing in Aphasia (PALPA). East Sussex, England: Lawrence Erlbaum Associates. Rapcsak, S. Z., & Beeson, P. M. (2000). Agraphia. In L. J. G. Rothi, B. Crosson, & S. Nadeau (Eds.), Aphasia and language: Theory and practice (pp. 184–220). New York: Guilford. Rapcsak, S. Z., & Beeson, P. M. (2004). The role of left posterior inferior temporal cortex in spelling. Neurology, 62, 2221–2229. Rapcsak, S. Z., Beeson, P. M., Henry, M. L., Leyden, A., Kim, E. S., Rising, K., et al. (2009). Phonological dyslexia and dysgraphia: cognitive mechanisms and neural substrates. Cortex, 45(5), 575–591. Rapcsak, S. Z., Beeson, P. M., & Rubens, A. B. (1991). Writing with the right hemisphere. Brain and Language, 41, 510–530. Tainturier, M.-J., & Rapp, B. (2001). The spelling process. In B. Rapp (Ed.), The handbook of cognitive neuropsychology (pp. 233–262). Philadelphia: Psychology Press. Vigneau, M., Beaucousin, V., Herve´, P. Y., Duffau, H., Crivello, F., Houde´, O., et al. (2006). Meta-analyzing left hemisphere language areas: phonology, semantics, and sentence processing. NeuroImage, 30, 1414–1432.

(weight), or resistance to pressure, with difficulties in perceiving size or shape is referred to as amorphognosia. While perhaps seeming a bit artificial, according to Bauer and Demery (2003), the distinction between ahylognosia and amorphognosia apparently traces back to 1935 when a French neurologist, Delay, divided astereognosis into two subtypes of deficits: amorphognosia, which was defined as a difficulty in recognizing the size or shape of an object by touch, and ahylognosia, which was described as a failure to differentiate the ‘‘molecular qualities’’ of an object, such as its density, weight, thermal conductivity, or roughness. Delay also defined a third type of astereognosis, tactile asymboly, which was characterized as the inability to identify an object by touch in the absence of amorphognosia and ahylognosia. These same distinctions were followed by Critchley (1969) and continue to be used by more recent authors (Bauer & Demery, 2003). Hecaen and Albert (1978) in their book, Human Neuropsychology, attempted to explain these distinctions by suggesting that ahylognosia was ‘‘the loss of the capacity to differentiate structural components of objects, resulted from impairment of intensity analyzers.’’ By contrast, amorphognosia, was thought to reflect ‘‘the loss of the capacity to differentiate forms, resulted from impairment of the analyzers of extent.’’ Because determining any of these qualities requires discriminatory judgments, in the absence of more elementary tactual defects, such disturbances suggest pathology involving the somatosensory areas of the parietal lobe.

Cross References ▶ Amorphognosis ▶ Astereognosis ▶ Tactile Agnosia

Ahylognosia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition Inability to determine by touch alone certain physical properties of an object such as its texture, density

References and Readings Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman, & E. Valenstein (Eds.), Clinical Neuropsychology, (4th ed., pp. 236–295). New York: Oxford University Press. Critchley, M. (1969). The parietal lobes. New York: Hafner Publishing Co. Delay, J. (1935). Les astereognosis. Pathologie due Toucher, Clinque, Physiologie, Topographie. Paris: Masson. Hecaen, H., & Albert, M. L. (1978). Human neuropsychology (Chapter 6, Disorders of somesthesis and somatognosis). New York: Wiley.

Akelaitis, Andrew John Edward (‘‘A.J.’’) (1904–1955)

Akathisia A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Synonyms Restlessness

Akelaitis, Andrew John Edward (‘‘A.J.’’) (1904–1955) M ICHAEL J. L ARSON , J OSEPH E. FAIR Brigham Young University Provo, UT, USA

Major Appointments

Definition Akathisia is a syndrome characterized by unpleasant sensations of inner restlessness that manifests itself with an inability to sit still or remain motionless.

Current Knowledge It is most often seen as a side effect of medications, mainly neuroleptic antipsychotics. Patients may have difficulty describing their symptoms, leading to a misdiagnosis of anxiety and worsening of the condition upon treatment with neuroleptic antipsychotic agents. Several medications have been used to treat the condition, including benztropine and beta-blocking agents. Withdrawal of the offending agent is often most effective. It may be seen with Parkinson’s disease.

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Dr. A.J. Akelaitis began his career as an assistant professor in the Department of Medicine, Division of Psychiatry, at the University of Rochester School of Medicine and Dentistry. At the same time, he also held appointments at the clinics of the Strong Memorial and Rochester Municipal Hospitals in Rochester, New York. He left these appointments to serve in the Navy during World War II. Following his service in the war, Dr. Akelaitis worked as an Assistant Professor of Neurology at the New York Medical College and Assistant Professor of Clinical Medicine in Neurology at Cornell University Medical College. He also served as the attending neuropsychiatrist at Mount Vernon (New York) Hospital and on the staff of the Bellevue Hospital and the New York Hospital.

Major Honors and Awards

Dr. Akelaitis was a Fellow of the American Psychiatric Association. He was specialty certified by the American Board of Psychiatry and Neurology and held membership appointments in the American Medical Association, the New York State Medical Society, the New York Society for Clinical Psychiatry, and the New York Neurological Society.

Cross References ▶ Parkinson’s Disease ▶ Tardive Dyskinesia

Landmark Clinical, Scientific, and Professional Contributions

References and Readings Kumar, A., & Calne, D. (2004). Approach to the patient with a movement disorder and overview of movement disorders. In R. L. Watts, & W. C. Koller (Eds.), Movement disorders (2nd ed., p. 9). New York: McGraw-Hill.

Dr. A.J. Akelaitis is best known for his observations of patients who underwent sectioning of the corpus callosum (i.e., ‘‘split-brain’’ patients). Beginning in the late 1930s, the neurosurgeon Dr. William P. van Wagenen pioneered surgical sectioning of the corpus callosum for the treatment of intractable epilepsy (Mathews, Linskey, & Binder, 2008). Dr. Akelaitis worked closely with Dr. van Wagenen and performed

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pre and postoperative tests of cognitive and neurological functioning on many of these individuals. According to Akelaitis’ reports, patients who underwent callosotomy surgery largely did not show lasting changes in cognitive, intellectual, or motor functioning, although their seizure activity was consistently alleviated. For nearly two decades, Akelaitis’ reports of largely normal functioning after callosotomy perpetuated the generally accepted belief that sectioning the corpus callosum did not impact cognitive or motor functioning in humans. Despite his reports of few neurological changes following callosotomy, Akelaitis noted periodic cases with hemiplegia and praxic disturbances. He was slow, however, to include the sectioning of the corpus callosum in his explanations for these changes; rather, he attributed the symptoms to unintended operative damage to adjacent cortical areas. In some cases, postoperative symptoms were seen as exacerbations of precallosotomy characteristics or were attributed to preexisting and/or postoperative psychological or behavioral factors. Further, many of the symptoms observed by Akelaitis were transient and consequently not considered to be conclusively linked with callosal sectioning (Sauerwein & Lassonde, 1996). Several factors most likely influenced Akelaitis’ reports of minimal neurological changes following callosotomy surgery. First, the majority of patients Akelaitis observed did not have complete callosotomies, nor were neurosurgical procedures well standardized at the time. Of the 28 patients he studied, only one third were reported to have undergone ‘‘complete’’ callosal sectioning, with the remainder ‘‘nearly complete’’ or ‘‘partial’’ sectioning (Bogen, 1995). The patients with only partially sectioned callosal fibers undoubtedly continued to have interhemispheric transmission, thereby contributing to Akelaitis’ findings of generally intact functioning. Next, emerging research at the time reported no cognitive changes following sectioning of the corpus callosum. For example, Walter Dandy stated in 1936 that when ‘‘the corpus callosum is split longitudinally. . . no symptoms follow its division. This simple experiment at once disposes of the extravagant claims to the functions of the corpus callosum’’ (see Zaidel, Iacoboni, Zaidel, & Bogen, 2003). Finally, Akelaitis lacked the technologies, such as the tachistoscope used by his successors, to present stimuli to one visual field. Such technology would possibly have given him insight into the specialization of the two hemispheres and interhemispheric transfer of information via the corpus callosum (Mathews, Linskey, & Binder, 2008).

Despite his contributions as one of the first individuals to study neurological functioning following callosotomy, Akelaitis has been criticized for employing insensitive or inadequate testing procedures. However, reviews of his cases have confirmed that his patients did exhibit what are now considered typical symptoms, although his explanations for these manifestations, while consistent with much of the research of the time, were often inadequate (Sauerwein & Lassonde, 1996). In the 1950s and 1960s, researchers including Roger Sperry, Michael Gazzaniga, Norman Geschwind, Edith Kaplan, and Joseph Bogen began to publish articles involving callosotomies in animals and humans, which contradicted many of Akelaitis’ findings. This sparked renewed interest in the function of the corpus callosum and eventually earned Sperry the Nobel Prize in 1981. Through the course of his short career, Dr. Akelaitis made significant contributions toward research on the corpus callosum and advanced the treatment of intractable epilepsy. He also published articles regarding the psychiatric aspects of myxedema (severe hypothyroidism), hereditary and vascular cerebral atrophy, lead encephalopathy, acute demyelinating processes (multiple sclerosis), and Pick’s disease.

Short Biography Andrew John (‘‘A.J.’’) Akelaitis was born in Baltimore, Maryland, on July 11, 1904. He studied medicine at Johns Hopkins University and received his M.D. in 1929. In the early 1930s, he practiced clinical neurology in Rochester, New York. He subsequently became an Assistant Professor of Medicine at the University of Rochester School of Medicine and Dentistry. Dr. Akelaitis joined the Navy during World War II where he served with distinction at the rank of Commander. He married the former Victoria Chesno. The couple had one son, Andrew, and a daughter, Lillian. Akelaitis died at the New York Hospital on November 24, 1955 at the young age of 51.

Cross References ▶ Corpus Callosum ▶ Epilepsy ▶ Split Brain

Akinetic Mutism

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Definition

Akelaitis, A. J. (1941). Psychobiological studies following section of the corpus callosum: A preliminary report. American Journal of Psychiatry, 97, 1147–1157. Akelaitis, A. J. (1941). Studies on the corpus callosum. II. The higher visual functions in each homonymous field following complete section of the corpus callosum. Archives of Neurology and Psychiatry, 45, 788–796. Akelaitis, A. J., Risteen, W. A., Herren, R. Y., & Van Wagenen, W. P. (1942). Studies on the corpus callosum. III. A contribution to the study of dyspraxia and apraxia following partial and complete section of the corpus callosum. Archives of Neurology and Psychiatry, 47, 971–1008. Akelaitis, A. J. (1944). Studies on the corpus callosum. IV. Diagonistic dyspraxia in epileptics following partial and complete section of the corpus callosum. American Journal of Psychiatry, 101, 594–599. Akelaitis, A. J. (1944). Study on gnosis, praxis, and language following section of corpus callosum and anterior commisure. Journal of Neurosurgery, 1, 94–102. Bogen, J. (1995). Some historical aspects of callosotomy for epilepsy. In A. G. Reeves & D. W. Roberts (Eds.), Epilepsy and the corpus callosum 2 (pp. 107–121). New York: Plenum Press. Gazzaniga, M. S. (1995). Principles of human brain organization derived from split brain studies. Neuron, 14, 217–228. Gazzaniga, M. S. (2005). Forty-five years of split-brain research and still going strong. Nature Reviews: Neuroscience, 6, 653–659. Mathews, S., Linskey, M., & Binder, D. (2008). William P. van Wagenen and the first corpus callosotomies for epilepsy. Journal of Neurosurgery, 108, 608–613. Sauerwein, H. C., & Lassonde, M. (1996). Akelaitis’ investigations of the first split-brain patients. In C. Code, C.-W. Wallesh, Y. Joanette, & A. R. Lecours (Eds.), Classic cases in neuropsychology (pp. 305–317). Hove, East Sussex: Psychology Press. Zaidel, E., Iacoboni, M., Zaidel, D., & Bogen, J. (2003). The callosal syndromes. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (pp. 347–403). New York: Oxford University Press.

Akinesis is an absence or paucity of movement, resulting from an abnormal motor control. It is a problem that may occur in Parkinson’s disease when patients develop freezing or inability to initiate movement. It may also occur as a result of a paralyzed muscle, such as with an anesthetic nerve block.

Akinesia ▶ Akinesis

Akinesis D OUGLAS I. K ATZ Braintree Rehabilitation Hospital Braintree, MA, USA Boston University School of Medicine Boston, MA, USA

Cross References ▶ Action-Intentional Disorders ▶ Akinetic Mutism ▶ Bradykinesia ▶ Parkinson’s Disease

Akinetic ▶ Akinesis

Akinetic Mutism M ICHAEL S. M EGA Brain Institute Providence Health System Portland, OR, USA

Synonyms A spectrum of motivational impairment has abulia at one end and akinetic mutism at the other. Coma vigil is not akinetic mutism; it arises when a comatose patient regains the sleep-wake cycle, eyes open during the day and closed during sleep at night, usually after 2 weeks of a brain lesion that produces irreversible coma. Coma vigil is also referred to as a persistent vegatative state. When brain lesions disconnect all descending motor output but preserve conscious awareness the patient is said to be locked in. In akinetic mutism, patients still respond to their internal and external environment – and thus are not in coma, and they are not locked in since they can accomplish motor output, given sufficient motivation.

Short Description or Definition Synonyms Akinesia; Akinetic

The fully formed akinetic mute state usually results from bilateral anterior cingulate lesions (Fig. 1). Patients are

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Akinetic Mutism. Figure 1 Arrows show the left greater than right anterior cingulate lesions due to bilateral anterior cerebral artery (ACA) ischemic stroke. Bilateral ACA lesions usually result in death due to loss of all limbic motivational input to prefrontal cortex

profoundly apathetic, incontinent, and akinetic. They do not initiate eating or drinking and if speech occurs, it is restricted to terse responses. They seem awake, visually tracking objects, but displaying no emotions – even during painful circumstances, they remain indifferent. The akinetic mute state also results from bilateral subcortical paramedian diencephalic and midbrain lesions possibly affecting the ascending reticular core, medial forebrain bundles, and isolated bilateral globus pallidus lesions.

Categorization When anterior cingulate lesions are bilateral, limbic, cognitive, and motor activation is disrupted producing profound akinetic mutism. Loss of ascending input from the reticular core, due to bilateral lesions of the medial forebrain bundle, may also produce akinetic mutism. Rarely are complete bilateral lesions seen in humans, more frequently partial circuit disruption results in a graded loss in motivation depending upon which circuit is damaged. Five frontal-subcortical circuits have been named according to their function or cortical site of origin: the motor circuit, originating in the supplementary motor

area, and the oculomotor circuit, originating in the frontal eye fields, are dedicated to motor function. The dorsolateral prefrontal, lateral orbitofrontal, and anterior cingulate circuits support executive cognitive functions, personality, and motivation, respectively (Mega & Cummings, 1994). Each of the five circuits has the same member structures: the frontal lobe, striatum, globus pallidus, substantia nigra, and thalamus. There is a progressive spatial compaction of the circuits as they travel through the basal ganglia. A lesion anywhere along the path of a circuit will produce the same clinical result but only in the globus pallidus interna are all the frontal-subcortical circuits in such a compact spatial volume that a relatively small lesion can have profound effects.

Epidemiology Akinetic mutism is exceedingly rare when permanent, since a bilateral lesion is necessary and usually results in death. Unilateral anterior cerebral artery (ACA) strokes are the usual cause of transient akinetic mutism, but ACA strokes only make up 1% of all cerebral vascular lesions.

Akinetic Mutism

Natural History, Prognostic Factors, and Outcomes The natural history of akinetic mutism, when it arises from a unilateral lesion, is usually a 2-week period of gradual improvement from the fully formed syndrome to near-complete recovery presumably enabled by contralateral limbic activation gaining access to deafferented networks. The outcome from bilateral lesions is usually death, given no ability for cross-hemispheric motivation. Thus, prognosis will rely upon neuroimaging documenting the extent of the lesion.

Neuropsychology and Psychology Extracingulate connections support a segregation of the cingulate into functional subregions (for a complete discussion of these circuits, ▶ Cingulate Gyrus). Paralleling the general distinction between posterior granular sensory cortices and anterior agranular executive cortices, the anterior cingulate can be considered an executive region for affective motivation and cognition, while the posterior cingulate, with its prominent granular layer IV receiving sensory input, is engaged in visuospatial and memory processing. The interconnections between the anterior and posterior cingulate allow for regulatory control by the anterior executive effector regions over posterior sensory processing and reciprocal modulation of that regulatory input by the posterior cingulate. Three anterior effector regions include a visceral effector region inferior to the genu of the corpus callosum encompassing area 25, the anterior subcallosal portions of 24a–b, and 32; a cognitive effector region that includes most of the supracallosal area 24, and areas 24a0 –b0 and 320 ; and a skeletomotor effector region within the depths of the cingulate sulcus, that includes areas 24c0 /23c on the ventral bank, with 24c0 g and 6c on the dorsal bank. These three cingulate effector regions integrate ascending input concerning the internal milieu of the organism with visceral motor systems, cognitive-attentional networks, and skeletomotor centers to produce the affective motivation necessary for the organism’s engagement in the environment. Circumscribed lesions in humans are rarely confined to one region of the cingulate. With an anterior lesion, the cognitive, skeletomotor, and visceral effector regions are often affected. Bilateral lesions result in an akinetic mute state. The loss of spontaneous motor activity results when the lesion involves the supplementary motor area and the

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skeletomotor effector region. When these two motor regions are spared, motor activity will be normal but the patient will demonstrate profound indifference, docility, and the loss of motivation to engage in a task. They can be led by the examiner to engage in a task but will fail to selfgenerate sustained directed attention. They lack cognitive motivation. The role of the anterior cingulate as a cognitive effector is appreciated within the realm of language. Language, a cognitive function, is distinguished from the motor function of speech. Transcortical motor aphasia (TCMA) is the usual result of left anterior medial or anterior dorsolateral prefrontal lesions. The classic syndrome of TCMA is initial mutism that resolves in days to weeks, yielding a syndrome featuring delayed initiation of brief utterances without impaired articulation, excellent repetition, inappropriate word selection, agrammatism, and poor comprehension of complex syntax. Activation of dorsolateral prefrontal cortices enabling language and speech arises from two sources: the anterior cingulate and the supplementary motor area (with the cingulate skeletomotor region). When the executive prefrontal cortex (areas 9, 10, and 46) is disrupted, cognitive language deficits are prominent (TCMA, type I); when motor neurons in area 4, devoted to the speech apparatus, are disconnected from their activation, speech hesitancy and impoverished output ensues (TCMA, type II). These two functional realms are separable and can be disconnected anywhere along two pathways. Direct damage to the supplementary motor area or its outflow to the motor cortex traveling in the anterior superior paraventricular white matter will produce TCMA type II. Direct damage to the anterior cingulate, its outflow to areas 9, 10, and 46, or to the caudate – via the subcallosal fasciculus, just inferior to the frontal horn of the lateral ventricle – will disrupt frontal-subcortical circuits involved in motivation and executive cognitive function. The initial muteness has been described by a patient after recovery from an anterior cingulate/supplementary motor infarction as a loss of the ‘‘will’’ to reply to her examiners, because she had ‘‘nothing to say,’’ her ‘‘mind was empty,’’ and ‘‘nothing mattered’’ (Damasio & Van Hoesen, 1983). The loss of will to initiate a motor function results from supplementary motor or cingulate skeletomotor region damage, while poor initiation of a cognitive process results from lesions in supracallosal cingulate areas. Loss of emotional vigilance ranging from flattened affect to neglect can be produced by surgery in this region. Anterior cingulate lesions in monkeys – difficult subjects

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in which to evaluate subtle behavioral changes – produce either no observable change or result in a transient stupor with ensuing lethargy, tameness, disturbed intraspecies social behavior, and decreased pain sensitivity (Pribram & Fulton, 1954). Removal of the anterior cingulate (areas 24 and 32) in humans (cingulectomy) has been employed as a treatment for epilepsy, psychiatric, and pain disorders. The cingulum bundle has also been the site of surgical lesions (cingulumotomy when only the bundle is transected, or cingulotomy when cingulate cortex is also removed) to treat psychiatric and pain disorders. The cingulum contains the efferents and afferents of the cingulate to the hippocampus, basal forebrain, amygdala, and all cortical areas, as well as fibers of passage between hippocampus and prefrontal cortex, and from the median raphe to the dorsal hippocampus. Surgical ablation of the anterior portion (sparing fibers relevant to memory function) is most successful when treating aggression, extreme anxiety, obsessive–compulsive behaviors, and severe pain. Psychotic symptoms show only a temporary response. The only prospective long-term follow-up of patients undergoing supracallosal anterior cingulotomy for the treatment of medically refractory obsessive–compulsive disorder revealed a clear response in 28% and a partial response in 17% (Baer, Rauch, Ballantine, Martuza, Cosgrove, Cassem, et al., 1995). Including the subcallosal anterior cingulate/medial orbital cortex may provide the best result in treating the refractory obsessive–compulsive patient (Hay, Sachdev, Cumming, Smith, Lee, Kitchener, et al., 1993) due to the elimination of the visceromotor aspects of the disorder. Postsurgical personality changes are subtle after the acute attentional disorder resolves. Although formal cognitive testing is unaltered, affect is flattened. Motivation for previous enjoyments, such as reading, hobbies, and even spectator sports, is lost (Tow & Whitty, 1953); subtle changes that reflect the loss of higher cognitive motivation. The three anterior cingulate regions, by virtue of the distinct functional systems they coordinate, are the conduits through which limbic motivation can activate feeling, thought, and movement – partial lesions produce partial aspects of the akinetic mute state depending upon their location. Subcortical lesions can also produce the fully formed syndrome. Carbon monoxide poisoning with resultant apathy and placidity was described in a patient with a ventral pallidal lesion who also had hypoperfusion on single photon emission computed tomography (SPECT) predominately in the cingulate bilaterally (Mori, Yamashita, Takauchi, & Kondo, 1996). Hypometabolism on 18 F-fluorodeoxyglucose positron emission tomography

(FDG-PET) in frontal cortex has also resulted from pallidal lesions (Laplane, Levasseur, Pillon, Dubois, Baulac, Mazoyer, et al., 1989) disconnecting their cortical targets. Yet, when pallidal lesions result from carbon monoxide poisoning, microscopic cortical lesions may contribute to the functional imaging abnormalities. Ventral extension of a pallidal lesion appears to disconnect the anterior cingulate circuit, in nonhuman primates and humans (Mega & Cohenour, 1997), from limbic drive. Bilateral paramedian or anterior thalamic lesions (Nagaratnam, Nagaratnam, Ng, & Diu, 2004), caudate (Grunsfeld & Login, 2006), or putamen (Ure, Faccio, Videla, Caccuri, Giudice, Ollari, et al., 1998) lesions will also disrupt the anterior cingulate frontal-subcortical circuit.

Evaluation Evaluation of the patient suspected of suffering from akinetic mutism is to first rule out other causes of possible unresponsiveness. Documenting the response to first verbal stimuli, and then sensory stimuli, will provide evidence for or against coma. Patients in coma will not respond to internal (e.g., hunger) or external (e.g., pain) stimuli. All patients who survive the myriad of insults producing coma will regain the sleep-wake cycle and will eventually open their eyes spontaneously. They are then described as being in a persistent vegetative state. The locked-in patient will blink to command and can be taught to use blinking as a form of communication. The patient with akinetic mutism will respond to stimuli but will not initiate an unprovoked response. When any patient with limited response is encountered, a brain imaging study is required in their evaluation.

Treatment Time is the best treatment for unilateral lesions producing akinetic mutism since after the acute phase of the lesion (4–6 weeks) the patients usually recover limbic activation from unaffected regions. When subcortical lesions destroy ascending dopaminergic fibers in the medial forebrain bundle, patients may respond to dopaminergic agonist (Psarros, Zouros, & Coimbra, 2003), or paradoxically antagonists of the D2 receptor (Brefel-Courbon et al., 2007) and GABA activation (Spiegel, Casella, Callender, & Dhadwal, 2008), perhaps due to blocking feedback-loop inhibition.

Alcohol Abuse

Cross References

Alcohol Abuse

▶ Abulia ▶ Amotivation ▶ Apathy ▶ Cingulate Gyrus

N ATE E WIGMAN University of Florida Gainesville, FL, USA


Synonyms

Baer, L., Rauch, S. L., Ballantine, H. T., Martuza, R., Cosgrove, R., Cassem, E., et al. (1995). Cingulotomy for intractable obsessivecompulsive disorder: prospective long-term follow-up of 18 patients. Archives of General Psychiatry, 52, 384–392. Brefel-Courbon, C., Payoux, P., Ory, F., Sommet, A., Slaoui, T., Raboyeau, G., et al. (2007). Clinical and imaging evidence of zolpidem effect in hypoxic encephalopathy. Annals of Neurology, 62(1), 102–105. Damasio, A. R., & Van Hoesen, G. W. (1983). Focal lesions of the limbic frontal lobe. In K. M. Heilman & P. Satz (Eds.), Neuropsychology of human emotion (pp. 85–110). New York: Guilford. Grunsfeld, A. A., & Login, I. S. (2006). Abulia following penetrating brain injury during endoscopic sinus surgery with disruption of the anterior cingulate circuit: case report. BMC Neurology, 6, 4. Hay, P., Sachdev, P., Cumming, S., Smith, J. S., Lee, T., Kitchener, P., et al. (1993). Treatment of obsessive-compulsive disorder by psychosurgery. Acta Psychiatrica Scandinavica, 87, 197–207. Laplane, D., Levasseur, M., Pillon, B., Dubois, B., Baulac, M., Mazoyer, B., et al. (1989). Obsessive-compulsive and other behavioural changes with bilateral basal ganglia lesions. Brain: A Journal of Neurology, 112, 699–725. Mega, M. S., & Cohenour, R. C. (1997). Akinetic mutism: a disconnection of frontal-subcortical circuits. Neurology, Neuropsychology, and Behavioral Neurology, 10, 254–259. Mega, M. S., & Cummings, J. L. (1994). Frontal subcortical circuits and neuropsychiatric disorders. Journal of Neuropsychiatry and Clinical Neurosciences, 6, 358–370. Mori, E., Yamashita, H., Takauchi, S., & Kondo, K. (1996). Isolated athymhormia following hypoxic bilateral pallidal lesions. Behavioral Neurology, 9, 17–23. Nagaratnam, N., Nagaratnam, K., Ng, K., & Diu, P. (2004). Akinetic mutism following stroke. Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia, 11(1), 25–30. Pribram, K. H., & Fulton, J. F. (1954). An experimental critique of the effects of anterior cingulate ablations in monkey. Brain: A Journal of Neurology, 77, 34–44. Psarros, T., Zouros, A., & Coimbra, C. (2003). Bromocriptine-responsive akinetic mutism following endoscopy for ventricular neurocysticercosis. Case report and review of the literature. Journal of Neurosurgery, 99(2), 397–401. Spiegel, D. R., Casella, D. P., Callender, D. M., & Dhadwal, N. (2008). Treatment of akinetic mutism with intramuscular olanzapine: a case series. Journal of Neuropsychiatry and Clinical Neurosciences, 20(1), 93–95. Tow, P. M., & Whitty, C. W. M. (1953). Personality changes after operations of the cingulate gyrus in man. Journal of Neurology, Neurosurgery, and Psychiatry, 16, 186–193. Ure, J., Faccio, E., Videla, H., Caccuri, R., Giudice, F., Ollari, J., et al. (1998). Akinetic mutism: a report of three cases. Acta Neurologica Scandinavica, 98(6), 439–444.

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Alcoholism; Binge drinking; Excessive alcohol use

Short Description or Definition Alcohol abuse refers to a ‘‘maladaptive pattern of alcohol [use] leading to clinically significant impairment or distress.’’ The DSM-IV Criteria for alcohol abuse are

DSM-IV-TR Criteria for Alcohol Abuse 1. A maladaptive pattern of alcohol abuse leading to clinically significant impairment or distress, as manifested by one or more of the following, occurring within a 12-month period:

Recurrent alcohol use resulting in failure to fulfill major role obligations at work, school, or home (e.g., repeated absences or poor work performance related to substance use; substance-related absences, suspensions, or expulsions from school; or neglect of children or household). Recurrent alcohol use in situations in which it is physically hazardous (e.g., driving an automobile or operating a machine). Recurrent alcohol-related legal problems (e.g., arrests for alcohol-related disorderly conduct). Continued alcohol use despite persistent or recurrent social or interpersonal problems caused or exacerbated by the effects of the alcohol (e.g., arguments with spouse about consequences of intoxication or physical fights).

2. These symptoms must never have met the criteria for alcohol dependence. Although alcohol abuse is diagnosed primarily by observed or reported impairment and distress related to alcohol use, the Dietary Guidelines for Americans recommends no more than one drink per day for women and two drinks per day for men (USDA, 2005).

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Categorization In the DSM-IV-TR, alcohol abuse is differentiated from alcohol dependence in that the former consists of drinking that impairs functioning without withdrawal symptoms and is thus diagnosed only when dependence is not present (Hasin, Van Rossem, McCloud, & Endicott, 1997). An alcohol abuser may continue to drink despite awareness of the potential negative physical, social, and legal consequences.

Epidemiology Alcohol abuse is associated with diseases of the liver, hypertension, neurological damage, and cardiac diseases such as heart failure. In 2000, alcohol abuse was responsible for 85,000 deaths in the U.S. National data suggest that the prevalence of DSM-IV-TR alcohol abuse (not including alcohol dependence) was 4.65% in 2001–2002. At that time, alcohol abuse was more common among men, younger respondents, and Whites. From 1991–1992 to 2001–2002, the prevalence of alcohol abuse increased, especially among young African American and Hispanics and in both men and women (Grant et al., 2004). It appears that alcohol abuse is generally more severe with earlier onset in age of alcohol use (Grant, Stinson, & Harford, 2001). Results from a national survey suggest that close to one fifth of adolescents and adults engaged in binge drinking one or more times within the last 30 days (US DHHS, 2002).

Natural History, Prognostic Factors, Outcomes In The Natural History of Alcoholism Revisited, George Vaillant (1995) described alcohol dependence as a condition of gradual onset over 5–15 years of continuous alcohol abuse. He found that the average age of onset was 29 years among a cohort of delinquent youth and 41 among a higher educated group. In the cohorts that Vaillant (1995) studied, the prevalence of alcoholism increased until age 40 and then declined at a rate of 2–3% per year thereafter. Potential risk factors for alcohol abuse in adolescence and early adulthood include being in areas of high availability and accessibility, sensation seeking and low harm-avoidance in youth, family history of alcohol abuse, liberal family attitude toward alcohol use, lack of family closeness, and early behavioral problems

(Hawkins, Catalano, & Miller, 1992). Another risk factor appears to be comorbid mental disorders. Epidemiological data suggest that 37% of people who have an alcohol disorder also have another mental disorder (Regier et al., 1990), emphasizing the importance of mental and behavior health screening. In terms of prognostic factors, Vaillant (1995) suggests that those who achieve ‘‘longterm sobriety usually [are characterized by] (1) a less harmful, substitute dependency; (2) new relationships; (3) sources of inspiration and hope; and (4) experiencing negative consequences of drinking.’’ In Vaillant (1995) delinquent youth cohort, by age 70, 54% had already died, 32% were abstinent, 12% were still abusing alcohol, and 1% were controlled drinkers (i.e., drinking but not abusing).

Neuropsychology and Psychology of Alcohol Abuse In a review of the literature of neuropsychological deficits in chronic alcohol abusers, Chelune and Parker (1981) found patterns of neurological damage such as cerebral atrophy, ventricular enlargement, and decreased cerebral blood flow. Approximately 10% of chronic alcohol abusers have neurocognitive deficits commensurate with diagnoses of alcohol-related amnesia or dementia. A large portion of those without diagnosable neurocognitive deficits still evince disturbed neuropsychological performance (Rourke & Grant, 2009). Alcoholics generally function in the average to above average range on IQ tests with consistently lower performance IQ (PIQ) scores relative to verbal IQ (VIQ). Their PIQ scores are similar to those of persons with brain damage, whereas VIQ scores are comparable with those of normal controls (Chelune & Parker, 1981; Rourke & Grant, 2009). However, this discrepancy is not diagnostic of alcoholism. Within the Wechsler subtests, Block Design appears to be the most frequently impaired relative to normal controls in all studies reviewed. Block Design impairment has been cited as an effective discriminator between alcoholics and non-alcoholics. Object Assembly and Digit Symbol were also impaired relative to normal controls in more than 3/4 of the studies. Other tests that have revealed impairment in alcoholics include the Category Test, Wisconsin Card Sorting Test, Raven’s Progressive, Shipley–Hartford Abstract Age, and other tests of abstract thinking. Alcoholics also generally perform poorly on Part B of the Trail Making Test relative to matched control groups (Chelune & Parker, 1981).

Alcohol Abuse

Overall, the most consistently impaired neuropsychological domains include verbal and nonverbal learning and perceptual-motor skills. More broadly, most reviews conclude that abstraction-executive abilities are impaired among alcohol abusers (Rourke & Grant, 2009). Despite the consistency of these neuropsychological findings, many of the samples from these studies are recently detoxified adults. Grant and Adams (2009) point out that neuropsychological recovery typically occurs following the first year – and perhaps more – of detoxification. Although the exact mechanisms of these neuropsychological deficits are not known, some of the major hypotheses attempting to explain these deficits have been (Chelune & Parker, 1981): 1. Chronic alcohol abuse results in premature aging of the brain. 2. Chronic alcohol abuse leads to global generalized CNS dysfunction. 3. Chronic alcohol abuse differentially disrupts the right hemisphere of the brain. 4. Chronic alcohol abuse exerts its detrimental effect on the anterior-basal regions of the brain. 5. Chronic alcohol abuse produces a generalized CNS impairment that is particularly disruptive of the fronto-parietal association areas of the brain. More recent neural hypotheses of the mechanisms of neuropsychological deficits include reduced regional blood flow to the frontal lobes, reduction in metabolites (e.g., NAA) that indicate lack of neuronal integrity, frontal-striatal and cerebellar dysfunction manifesting as loss of dendritic arbor (Rourke & Grant, 2009). Grant and Adams (2009) note that molecular mechanisms of the influence of chronic alcohol abuse on neuropsychological functioning are largely unknown.

Evaluation A common screening tool for alcohol abuse is the CAGE questionnaire (Ewing, 1984; see An even briefer CAGE Questionnaire, Table B).The CAGE is highly effective at identifying problem drinkers among adults (Bernadt, 1982). Two ‘‘yes’’ responses on the CAGE indicate that the respondent should be investigated further. The questionnaire asks the following questions:

Have you ever felt you needed to Cut down on your drinking? Have people Annoyed you by criticizing your drinking?

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Have you ever felt Guilty about drinking? Have you ever felt you needed a drink first thing in the morning (Eye-opener) to steady your nerves or to get rid of a hangover?

Other brief assessments for alcohol abuse include the POSIT and CRAFFT for adolescents (Knight, Sherritt, Harris, Gates, & Chang, 2003), the Michigan Alcoholism Screen Test (MAST) for adults (Magruder-Habib, Stevens, & Alling, 1993), and the AUDIT-C for both adults and adolescents (Bush et al., 1998). According to Fiellin, Reid, and O’Connor (2000), the CAGE and the AUDIT are the superior screening instruments in primary care settings compared with other alcohol abuse screeners and other clinical methods. The CAGE is superior at detecting diagnosable abuse and dependence and the AUDIT is superior at detecting at risk and harmful drinking (Fiellin et al., 2000).

Treatment Treatment ranges from support groups to rehabilitation centers. Treatments of alcohol abuse appear to be largely psychosocial. In a systematic review, brief psychosocial interventions among primary care patients were found to be effective at reducing alcohol consumption (Kaner et al., 2007). Although well-known support groups such as Alcoholic Anonymous (AA) have been helpful to many people and likely constitute the most accessible form of treatment, evidence has not supported AA’s effectiveness at reducing alcohol problems (Ferri, Amato, & Davoli, 2006). Medical treatments of alcohol abuse focus on reducing craving. Naltrexone (Chick et al., 2000) and Acomprosate (Garbutt, West, Carey, Lohr, & Crews, 1999) have been found to be effective at reducing craving. However, most medications are aimed at dependence, not abuse symptoms.

Cross References ▶ Alcohol Brain Syndrome ▶ Alcohol Dependence ▶ Blood Alcohol Level ▶ Fetal Alcohol Syndrome ▶ Michigan Alcoholism Screen Test ▶ Substance Abuse ▶ Wernicke-Korsakoff ’s Syndrome

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References and Readings Bernadt, M. W. (1982). Comparison of questionnaire and laboratory tests in the detection of excessive drinking and alcoholism. Lancet, 6, 325–328. Chelune, G. J., & Parker, J. B. (1981). Neuropsychological deficits associated with chronic alcohol abuse. Clinical Psychology Review, 1, 181–195. Chick, J., Anton, R., Checinski, K., Croop, R., Drummond, D. C., Farmer, R. et al. (2000). A multicentre, randomized, double-blind, placebo-controlled trial of naltrexone in the treatment of alcohol dependence or abuse. Alcohol, 35, 587–593. Ewing, J. A. (1984). Detecting alcoholism: The CAGE questionnaire. JAMA, 252, 1905–1907. Ferri, M. M. F., Amato L., & Davoli M. (2006). Alcoholics anonymous and other 12-step programmes for alcohol dependence. Cochrane Database of Systematic Reviews, Issue 3, Art. No.: CD005032. doi: 10.1002/14651858.CD005032.pub2 Fiellin, D. A., Reid, M. C., & O’Connor, P. G. (2000). Screening for alcohol problems in primary care: A systematic review. Archives of Internal Medicine, 160(13), 1977–1989. Garbutt, J. C., West, S. L., Carey, T. S., Lohr, K. N., & Crews, F. T. (1999). Pharmacological treatment of alcohol dependence: A review of the evidence. JAMA, 281, 1318–1325. Grant, B. F., Dawson, D. A., Stinson, F. S., Chou, S. P., Dufour, M. C., & Pickering, R. P. (2004). The 12-month prevalence and trends in DSM-IV alcohol abuse and dependence: United States, 1991–1992 and 2001–2002. Drug and Alcohol Dependence, 74, 223234. Grant, I., Adams K. M. (Eds.) (2009). Neuropsychological assessment of neuropsychiatric disorders (3rd ed., pp. 127–158.). New York: Oxford University Press. Grant, B. F., Stinson, F. S., & Harford, T. C. (2001). Age at onset of alcohol use and DSM-IV alcohol abuse and dependence: A 12-year followup. Journal of Substance Abuse, 13, 493–504. Hasin, D. S., Van Rossem, R., McCloud, S., & Endicott, J. (1997). Alcohol dependence and abuse diagnoses: Validity in a community sample of heavy drinkers. Alcoholism, Clinical and Experimental Research, 21, 213–219. Hawkins, J. D., Catalano, R. F., & Miller, J. Y. (1992). Risk and protective factors for alcohol and other drug problems in adolescence and early adulthood: Implications for substance abuse prevention. Psychological Bulletin, 112, 64–105. Knight, J. R., Sherritt, L., Harris, S. K., Gates, E. C., & Chang, G. (2003). Validity of brief alcohol screening tests among adolescents: A comparison of the AUDIT, POSIT, CAGE, and CRAFFT. Alcoholism, Clinical and Experimental Research, 27, 67–73. Magruder-Habib, K., Stevens, H. A., & Alling, W. C. (1993). Relative performance of the MAST, VAST, and CAGE versus DSM-III-R criteria for alcohol dependence. Journal of Clinical Epidemiology, 46, 435–441. Regier, D. A., Farmer, M. E., Rae, D. S., Locke, B. Z., Keith, S. J., Judd, L. L., et al. (1990). Comorbidity of mental disorders with alcohol and other drug abuse. Results from the epidemiologic catchment area (ECA) study. JAMA, 264, 2511–2518. Rourke, S. B., & Grant, I. (2009). The neurobehavioral correlates of alcoholism. In I. Grant & K. M. Adams (Eds.), Neuropsychological assessment of neuropsychiatric and neuromedical disorders (3rd ed., pp. 398–454). New York: Oxford University Press. U.S. Department of Health and Human Services. Substance Abuse and Mental Health Services Administration(US DHHS). (2002). Results

from the 2001 national household survey on drug abuse: Volume I. Summary of national findings (Office of Applied Studies, NHSDA Series H-17 ed.) (BKD461, SMA 02–3758). Washington, DC: U.S. Government Printing Office. Retrieved March 14, 2009, from the World Wide Web: http://www.oas.samhsa.gov/nhsda/2k1nhsda/ vol1/Chapter3.htm United States Department of Agriculture and United States Department of Health and Human Services (USDA). (2005). Dietary guidelines for Americans: Chap. 9 – Alcoholic beverages (pp. 43–46). Washington, DC: US Government Printing Office. Vaillant, G. E. (1995). The natural history of alcoholism revisited. Cambridge, MA: Harvard University Press.

Alcohol Addiction ▶ Alcoholism

Alcohol Amnesic Disorder ▶ Korsakoff ’s Syndrome

Alcohol Dependence G LENN S. A SHKANAZI University of Florida-College of Public Health and Health Professions Gainesville, FL, USA

Synonyms Alcoholism

Definition As described in DSM-IV, alcohol dependence is a set of symptoms encompassing dysfunction in cognitive, behavioral, and physiological domains caused by continued alcohol use. A pattern of repeated alcohol ingestion exists, resulting in increasing amounts consumed in order to obtain the desired effect (i.e., tolerance) and characteristic symptoms if use is suddenly suspended (i.e., withdrawal). There is a perceived loss of control over drinking, exhibited by repeated failed attempts to decrease or quit drinking. Individuals may spend increasing amounts of time

Alcoholic Brain Syndrome

in drinking-related behaviors without being able to stop, despite being aware that drinking is causing, or exacerbating, psychological or medical problems. Cognitive consequences can include memory loss, difficulty performing familiar tasks, poor or impaired judgment, and problems with language.

Cross References ▶ Alcohol Abuse ▶ Alcohol Dementia ▶ Alcoholic Brain Syndrome ▶ Substance Abuse ▶ Substance Abuse Disorders ▶ Wernicke–Korsakoff Syndrome

References and Readings American Psychiatric Association (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: American Psychiatric Association.

Alcoholic Amnestic Disorder ▶ Wernicke-Korsakoff Syndrome

Alcoholic Brain Syndrome G LENN S. A SHKANAZI University of Florida-College of Public Health and Health Professions Gainesville, FL, USA

Synonyms Alcoholic dementia; Alcoholic hallucinosis; Delirium tremens; Korsakoff ’s syndrome; Wernicke–Korsakoff syndrome

Short Description or Definition ‘‘Alcoholic brain syndrome’’ is a collection of several syndromes associated with the acute or chronic use of

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alcohol, resulting in significant impairment on normal brain functioning (APA Dictionary of Psychology, 2007).

Categorization As mentioned in the definition, alcoholic brain syndrome encompasses several syndromes. 1. Alcohol withdrawal delirium: A reversible condition that develops after cessation of chronic, extreme alcohol intake. Symptoms include disturbed consciousness (e.g., disruption in attention/concentration), disruption in memory, orientation, and language beyond what would be expected from typical alcohol withdrawal. 2. Alcohol-induced persisting dementia: A chronic condition that includes multiple cognitive deficits as a result of prolonged alcohol abuse. Cognitive areas generally impaired include memory, speech, motor/ sensory functions and executive functions. Global impairment in intellectual functioning evolves gradually over time. 3. Alcohol-induced persisting amnestic disorder: A persistent disturbance in memory functioning caused by chronic alcohol abuse. Memory impairment is severe enough to cause significant disturbance in occupational or social functioning. 4. Wernicke’s encephalopathy (WE): A syndrome resulting from chronic alcoholism leading to nutritional deficiency (i.e., Vitamin B1 [Thiamine] and characterized by acute confusion, ataxia, sluggish pupillary reflexes, and nystagmus and memory deficits). The syndrome can result in coma or death. Lesions are centered in the midbrain, cerebellum, and diencephalon. 5. Korsakoff ’s syndrome: This condition often follows episodes of WE. Thiamine deficiency, as a result of chronic, severe alcohol abuse, leads to a dense anterograde and retrograde amnesia. Patients with Korsakoff ’s syndrome can store information for only a few seconds before they forget it. The resulting amnesia is thought to be due to damage in the mammillary bodies, anterior or dorsomedial nuclei (or both) of the thalamus. Another common feature is confabulation, in which the patient recounts detailed and convincing memories for events that have never happened. 6. Alcohol-induced psychotic disorder: A condition involving the presence of delusions and/or hallucinations due to the physiological effects of alcohol.

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Epidemiology Up to 2 million alcoholics have developed permanent and debilitating conditions that require lifetime custodial care. A number of factors influence how and to what extent alcohol affects the brain. These include the age at which the person started drinking, duration of drinking, amount of alcohol consumed, drinking style/pattern, patient’s age, education, genetic background, family history of alcoholism, neuropsychiatric risk factors (e.g., prenatal alcohol exposure), and general health status. Studies comparing men and women’s sensitivity to alcohol-induced brain damage have not been conclusive. Poor nutrition has been a major contributor to the development of alcohol-induced brain damage. Up to 80% of alcoholics have a deficiency in thiamine (i.e., Vitamin B1). This vitamin is an essential nutrient required by all tissues including the brain. Some of these people will progress to WE. Approximately 80–90% of alcoholics with Wernicke’s develop Korsakoff ’s psychosis, which is more prevalent in men aged 45–65. Women who develop this condition tend to do so at a younger age (i.e., 35–55).

Natural History, Prognostic Factors, and Outcomes WE is a medical emergency and requires immediate treatment, as it can lead to death in approximately 20% of untreated cases. Symptoms can develop within hours and can be easily missed as many mimic intoxication. If treatment is given in time, usually through the administration of thiamine, progression of symptoms can be slowed or stopped. Ocular abnormalities usually recover within a few days to a few weeks, but ataxia takes 1–2 months longer to resolve. The acute confusion/delirium usually improves within 1–2 days after the treatment but may take 1–3 months to completely clear. If treatment is not provided, then irreversible brain damage, or even death, is possible. Of those who survive, approximately 85% develop Korsakoff ’s syndrome. However, not every person who develops Korsakoff ’s syndrome has a previous episode of Wernicke’s. Some will develop Korsakoff ’s gradually with either no known history or brief episodes of Wernicke’s. Some patients are initially comatose or semiconscious and only when the acute disorder has resolved is the underlying Korsakoff ’s syndrome manifest. These patients are still susceptible to developing Wernicke’s, especially if drinking were to continue.

Loss of some cognitive functions including memory in Korsakoff ’s syndrome may be permanent. Once the patient has developed Korsakoff ’s, the treatment strategies are not clear. However, it is important for patients to remain abstinent from alcohol. Depending on the degree of memory and executive function impairment, and availability of family support, patients with Korsakoff ’s may require long-term custodial care.

Neuropsychology and Psychology of Alcoholic Brain Syndrome The classic symptom in Korsakoff ’s syndrome is the inability to form new memories (i.e., anterograde amnesia). However, patients also demonstrate significant deficits in their ability to recall incidents or events from their own past as well (i.e., episodic memory). Memory for facts, concepts, and language (i.e., semantic memory) is variable while perceptual-motor memory is thought to be preserved. The inability to recall previously learned information (i.e., retrograde amnesia) can often extend back 20–30 years in a person’s life with Korsakoff ’s patients. Generally, a temporal gradient exists such that memories from the more distant past are recalled better than the more recent ones. The basis of this extensive retrograde amnesia is still a matter of great controversy. These patients are typically younger than most patients presenting to dementia services and because they often present as initially confused, with concomitant frontal lobe pathology, they are more likely to demonstrate aggressive, agitated behaviors and anxiety. Those with irreversible brain damage are unlikely to be able to live alone but also typically lack available social services. These patients often have a difficult time maintaining social and familial relationships and live isolated lives.

Evaluation For patients who meet the DSM-IV criteria for WE or Korsakoff ’s syndrome, neuropsychological assessment is useful for documenting functions that are impaired, the severity of impairment, and the prognostic factors involved in determining the patient’s ability to manage daily life either independently or with assistance. However, it is preferable for the neuropsychological assessment to occur when the patient has been abstinent from alcohol for a long enough period of time to insure that the acute symptoms of alcohol withdrawal have subsided.

Alcoholism

Treatment The primary treatment option for patients experiencing alcoholic brain syndrome is to stop drinking and remain abstinent. Without additional alcohol exposure, the recovery from the delirium caused by alcohol is usually good. This is obviously the first treatment to be utilized. As mentioned above, thiamine deficiency is an important contributor to alcohol-related brain damage; therefore, Vitamin B1 supplementation is necessary. Initially, the vitamins can be given intravenously or intramuscularly followed by oral administration. WE responds well to high-dose vitamins, and such treatment can prevent the occurrence of severe, chronic Korsakoff’s syndrome. Secondarily, nutritional counseling to promote a vitamin-rich and balanced diet is also part of this initial treatment protocol, especially for longer-term recovery and prevention.

Cross References ▶ Alcoholism ▶ Amnesia ▶ Anterograde Amnesia ▶ Dementia ▶ Encephalopathy ▶ Episodic Memory ▶ Korsakoff ’s Syndrome ▶ Organic Brain Syndrome ▶ Retrograde Amnesia ▶ Semantic Memory ▶ Substance Abuse

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Alcoholic Dementia ▶ Alcoholic Brain Syndrome

Alcoholic Hallucinosis ▶ Alcoholic Brain Syndrome

Alcoholic Polyneuropathy ▶ Korsakoff ’s Syndrome

Alcoholic Psychosis ▶ Korsakoff ’s Syndrome

Alcoholism G LENN S. A SHKANAZI University of Florida-College of Public Health and Health Professions Gainesville, FL, USA


Synonyms

Kopelman, M., Thomson, A., Guerrini, I., & Marshall, E. (2009). The Korsakoff Syndrome: Clinical aspects, psychology and treatment. Alcohol & Alcoholism, 44(2), 148–154. Martin, P., Singleton, C., & Hiller-Sturmhofel, S. (2003). The role of thiamine deficiency in alcoholic brain disease. Alcohol Research & Health, 27(2), 134–142. Oscar-Berman, M., & Marinkovic, K. (2003). Alcoholism and the brain: An overview. Alcohol Research & Health, 27(2), 125–133. Parsons, O. (1996). Alcohol abuse and alcoholism. In R. Adams, O. Parsons, J. Culbertson, & S. Nixon (Eds.), Neuropsychology for clinical practice: Etiology, assessment, and treatment of common neurological disorders. Washington, DC: American Psychological Association. Rourke, S., & Grant, I. (2009). The neurobehavioral correlates of alcoholism. In I. Grant & K. M. Adams (Eds.), Neuropsychological assessment of neuropsychiatric and neuromedical disorders (3rd ed.). New York, NY: Oxford University Press. White, A. (2003). What happened? Alcohol, memory blackouts, and the brain. Alcohol Research & Health, 27(2), 186–196.

Alcohol abuse; Alcohol addiction; Alcohol dependence; Problem drinking; Substance abuse

Definition The term ‘‘alcoholism’’ has a variety of definitions. For some, it is a disease that makes a person dependent on alcohol, causes an obsession with alcohol and inability to control how much they drink even though their drinking causes serious problems in their relationships, health, work, and finances. Others do not define it as a ‘‘disease’’ per se but rather a ‘‘condition,’’ behavioral in nature, which results in continued consumption of alcohol despite health problems and negative social consequences. For some, the definition must include the concepts of

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addiction and physiological withdrawal mechanisms, while for others, these are consequences of drinking. It is common for laypeople to equate any kind of excessive drinking with alcoholism. Those in the mental health fields see that disorders related to alcohol use lie along a continuum of severity that may include physical dependency/withdrawal (i.e., alcohol dependence) or may involve impaired drinking habits that lead to health or social problems/consequences but without dependency/ withdrawal (i.e., alcohol abuse). According to the APA Dictionary of Psychology, alcoholism is the popular term for ‘‘alcohol dependence.’’

Historical Background The term ‘‘alcoholism’’ was first used in 1849 by a physician, Magus Haas, to describe the systematic adverse effects of alcohol overconsumption. In the USA, it became a popular term in the 1930s as a result of the growth of Alcoholics Anonymous (AA). Previously, society viewed those who drank to excess as immoral, weak of character, and irresponsible. Society’s response was punishment and removal of overconsumers from sober society to protect the community. With the rise of AA, and their publication (i.e., the ‘‘Big Book’’), the view of alcoholism changed from character flaw to medical disease. AA viewed alcoholism as a physical allergy to alcohol accompanied by an obsession with drinking. This organization began to dispel the previously held beliefs that alcoholics were unemployable, destitute, and isolated individuals by demonstrating that some highly respected people who had been alcohol dependent had eventually overcome their disorder and went on to lead productive lives.

Epidemiology The epidemiology of alcoholism can be confusing and contradictory, depending on the definition being utilized and the measurement tool. The generally accepted overall rate of occurrence of alcoholism in the USA is 10%. The U.S. National Longitudinal Alcohol Epidemiologic Study concluded that alcoholism is prevalent in 20% of adult hospital inpatients and in 17% of community-based primary care practices. A 1985 U.S. National Hospital Survey found that 528,000 patients were discharged from hospitals with a primary diagnosis of substance abuse, and for 81% (428,000), alcohol was the abused substance. According to a 2001 survey conducted by the National Institute on Alcohol Abuse and Alcoholism (NIAAA) in the USA, approximately 48% of adults (aged 12 or older)

reported being current drinkers of alcohol (approximately 109 million). That number drops to 44% when the age is 18 or older. Approximately 20% of persons aged 12 or older participated in binge drinking at least once in 30 days prior to the 2001 survey. ‘‘Heavy drinking’’ was reported by 5.7% of the 12 or older population (12.9 million). The highest prevalence for both binge and heavy drinking was for those in the 18–25 age groups with the peak rate occurring at age 21. Studies have found those who begin drinking at an earlier age are at higher risk to develop dependency. Those Americans who wait till age 21 are 4 times less likely to become dependent than those who begin drinking before the age of 15 (i.e., 40% who start before age 15 develop dependency on alcohol at some point in their lives). The risk for developing dependency declines with age, as the prevalence rate for alcoholism in those persons greater than 65 years old is 3%. There are other nonage risk factors as well. Those with lower education and lower socioeconomic status are also at higher risk. There are also gender differences as men are at minimum 2.5 times more likely to be defined as ‘‘alcoholic’’ as women; however, the proportion of female alcoholics is increasing. White, non-Hispanic, individuals are more likely to develop alcoholism than AfricanAmericans. The risk for Hispanics is generally the same as Whites. Alcoholism is estimated to be the third leading cause of preventable death in the USA (after smoking and obesity). In the USA, 85,000 deaths are attributable to alcohol each year at a cost of $185 billion. The NIAAA estimates that intoxication is present in 30–60% of homicides, 22% of suicides, 33–50% of automobile accidents, 67% of drownings, and 70–80% of fire-related deaths. More than 50% of American adults have a close family member who has or has had alcoholism. Approximately one in four children younger than 18 in the USA is exposed to alcohol abuse or alcohol dependence in their family. Internationally, the World Health Organization estimates that there are 140 million people worldwide that are alcohol dependent and they account for 3.5% of the total cases of disease worldwide, which is a higher rate than tobacco or illicit drugs.

Current Knowledge Causes There is no identifiable single cause of alcoholism. Scientists believe that a myriad of factors play a role in the development of alcoholism.

Alcoholism

1. Genetics: Previous twin and adoption studies have demonstrated that genes play an important role in the development of alcoholism. Researchers found that identical twins (i.e., identical genes) have a higher concordance rate for drinking behavior than fraternal twins. Other studies have cast some doubt on these twin studies by suggesting the environment of identical twins is more alike than fraternal twins, thus suggesting a weakening of the argument in favor of genes. In the adoption studies, researchers found that whether reared by biologic or adoptive parents, the sons of males with alcohol problems are 4 times more likely to have alcohol problems than sons of persons who are not. In either case, epidemiologic studies indicate that alcoholism tends to run in families. Alcoholics are 6 times more likely than nonalcoholics to have blood relatives who are alcohol dependent. In summary, a person’s genetic makeup can predispose them to alcoholism or not. 2. Peer influence: Social networks that include heavy drinkers and alcohol abusers increase an individual’s risk for alcoholism. 3. Cultural influence: Cultures that include well-established taboos against drunkenness and rules regarding drinking have lower alcoholism rates than those who do not. 4. Psychiatric conditions: Certain psychiatric diagnoses increase the risk of alcoholism. These include ADHD, panic disorder, schizophrenia, and antisocial personality disorder.

Screening There are a variety of measures for alcoholism including the following: 1. CAGE: The CAGE is named for the four questions asked of a patient before any questions regarding quantities drank are asked. a Have you ever felt the need to Cut down on your drinking? b Have people Annoyed you by criticizing your drinking? c Have you ever felt Guilty about drinking? d Have you ever felt you needed a drink in the morning to steady your nerves or get rid of a hangover? (Eye-opener) The CAGE has been extensively validated. Those who answer ‘‘YES’’ to two or more questions are 7 times

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more likely to be alcohol dependent. It is not an adequate measure by itself but can alert a health-care provider to probe further. Another weakness is that it tends to be less reliable with populations with lower alcoholism rates (e.g., elderly) and does not identify ‘‘hazardous drinking.’’ 2. Alcohol Use Disorders Identification Test (AUDIT): The AUDIT can detect both hazardous drinking and alcohol abuse. It does not need to be administered face to face like the CAGE. It was developed by the World Health Organization and yields scores for consumption, dependency, and alcohol-related problems. 3. Alcohol Dependence Data Questionnaire: More sensitive than the CAGE and can distinguish abuse versus dependence.

Diagnosis Health-care providers most often use the Diagnostic Statistical Manual of the Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR) criterion for alcohol dependence. The diagnosis requires three of the following criteria: 1. Maladaptive pattern of the use leading to impairment/ distress as manifested by three or more of the below occurring in the same 12-month period. Tolerance Withdrawal Drink more frequently or in larger amounts than intended Persistent desire to drink or unsuccessful efforts to cut down or control use Great deal of time spent in acquiring/using alcohol or recovering from its effects Important social, occupational, or recreational activities given up or reduced because of alcohol Drinking continues despite knowledge of persistent or recurrent physiological, or psychological problems caused or exacerbated by drinking

Treatment There are several well-accepted avenues of treatment. 1. Psychosocial: Studies have shown that simple, brief interventions can be effective in those not severely alcohol dependent. One of those getting an extensive trial has been ‘‘Motivation Interviewing’’ based on

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Prochaska’s Five Stages of Change Model. A summary of the treatment approach is as follows: Precontemplation – Patient expresses no interest or need for change. The health-care professional’s options are limited. They can point to discrepancies between the patient’s goals and behavior and recommend 2 weeks of abstinence. Contemplation – Patient expresses ambivalence or skepticism about change. The provider should work to influence them in direction of change, provide information about the dangers of alcohol abuse, and recommend an abstinence trial. Preparation – Patient accepts need for change and makes plans to accomplish changed drinking goal. Action – Patient recognizes problem in drinking behavior and takes observable steps to decrease alcohol use. Professional reinforces decision for change and may introduce self-help groups and/ or medications. Maintenance – Patient and professional work together to maintain change and prevent relapse.

2. Medications: The most common medications in the treatment of alcoholism are: Disulfiram (Antabuse) – Prevents the elimination of acetaldehyde, which is a by-product of alcohol metabolism. Results in unpleasant side effects in persons still drinking including nausea, dizziness, headache, flushing, vomiting, heart palpitations, and sudden drop in blood pressure. Disulfiram needs to be taken daily to be effective. However, in at least one large clinical trial it did not increase abstinence. Naltrexone (ReVia) – May work by blocking the positive effects felt from drinking by blocking opiate receptors in the brain thereby decreasing craving for alcohol. Clinical studies have found a modest decrease in relapse (12–20%). This drug has an unknown cause of action. Acamprosate (Campral) – Used to maintain abstinence once alcoholics have stopped drinking. Thought to work by stabilizing the chemical balance in the brain. In clinical trials, the one year abstinence rates have been 18% and 12% at two years.

afford a private hospital or private psychiatrist could only find help in state hospitals, jails, or churches. AA was the first self-directed approach toward treatment. The AA treatment model includes self-help groups, utilizing psychological principles organized in small local community groups. The ‘‘12 steps’’ of AA encourage confrontation of denial, admission of powerlessness over alcohol, and strives for people to atone for harm caused by their behavior while drinking. It encourages its members to live ethically with a reliance on a ‘‘higher power.’’ It is this sense of AA as a ‘‘religion’’ that has led to nonreligious selfhelp groups including rational recovery, LifeRing, and SOS.

Future Directions The following are areas needing continued study: 1. Genetic research – current and future studies are looking at individuals with a family history of alcoholism to pinpoint the location of genes that influence vulnerability to alcoholism. This line of study will assist in the early identification of individuals at risk and of new, gene-based treatment approaches. 2. Treatment approaches – The NIAAA has been funding a study called ‘‘Project MATCH’’ whose goal is to identify variables important in predicting outcome based on patient characteristics and treatment design. 3. Medications – Naltrexone was the first drug approved by the FDA in 45 years to help alcoholics stay sober following detoxification. More research is needed.

Cross References ▶ Alcoholic Brain Syndrome ▶ Fetal Alcohol Syndrome ▶ Korsakoff ’s Syndrome ▶ Michigan Alcoholism Screening Test ▶ Motivational Interviewing ▶ Substance Abuse Disorders ▶ Twin Studies ▶ Wernicke–Korsakoff ’s Syndrome

References and Readings 3. Self-help groups: Perhaps the best-known organization involving alcoholism is AA. Until the mid-1930s in the USA, alcohol-dependent persons who could not

National Institute on Alcohol Abuse and Alcoholism. Etiology and natural history of alcoholism. URL Accessed on June 1, 2009 (http://pubs.

Alexia niaaa.nih.gov/publications/social/module2etiology&naturalhistory/ module2.html). National Institute on Alcohol Abuse and Alcoholism. Helping patients who drink too much: A clinician’s guide. URL Accessed on June 1, 2009 (http://www.niaaa.nih.gov/Publications/EducationTrainingMaterials/guide.htm). Schuckit, M. (2000). Drug and alcohol abuse: A clinical guide to diagnosis and treatment. New York, NY: Kluwer Academic/Plenum Publishers. U.S. Department of Health and Human Services and SAMHSAs National Clearinghouse for Alcohol and Drug Information. Accessed URL on June 1, 2009 (http://ncadistore.samhsa.gov/catalog/facts.aspx? topic=3).

Alertness C HRIS L OFTIS STG International Alexandria, VA, USA

Synonyms Awareness; Consciousness; Watchfulness

Definition A state of being mentally perceptive and responsive to external stimuli. A ‘‘readiness to respond’’ that can be detected by Electroencephalography (EEG). Alertness is susceptible to fatigue; maintaining a constant level of alertness is difficult, particularly for monotonous tasks demanding continuous attention. Stimulants such as nicotine, caffeine, and amphetamines can temporally boost alertness. Diminished alertness is often associated with the physiological response of yawning, which may boost the alertness of the brain. Impaired alertness is a common symptom of a number of conditions, including narcolepsy, attention deficit disorder, traumatic brain injury, chronic fatigue syndrome, depression, Addison’s disease, and sleep deprivation.

Cross References ▶ Alertness ▶ Electroencephalography

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Alexia S TEVEN Z. R APCSAK , P E´ LAGIE M. B EESON The University of Arizona Tucson, AZ, USA

Short Description or Definition The term alexia is applied to acquired disorders of reading produced by neurological injury in individuals with normal premorbid literacy skills. Clinically, patients with alexia have difficulty in recognizing, pronouncing, or comprehending written words. Although alexia can occur in relative isolation, it is more frequently encountered in the context of spoken language dysfunction or aphasia. Most individuals with alexia have concommitant spelling impairment or agraphia, suggesting that reading and spelling rely on shared cognitive representations and neural substrates. Acquired alexia needs to be distinguished from developmental dyslexia reflecting a failure to attain normal reading skills.

Categorization Alexia is not a single clinical entity. Instead, there are several distinct forms of alexia characterized by specific combinations of impaired and preserved reading abilities and associated with unique lesion profiles. The three most commonly encountered alexia syndromes include pure alexia/letter-by-letter reading, phonological/deep alexia, and surface alexia. In order to understand the neuropsychological mechanisms underlying different subtypes of alexia, it is important to briefly review the cognitive processes involved in normal reading. Reading is a complex cognitive skill that requires rapid visual discrimination of letters and words, as well as the ability to link information about visual word forms (orthography) with knowledge about word sounds (phonology) and word meanings (semantics). According to an influential dual-route model of reading (Coltheart, Rastle, Perry, Langdon, & Ziegler, 2001), perceptual processing of written words begins with visual feature analysis and letter shape detection (Fig. 1). Following the letter identification stage, the model postulates two distinct procedures or processing routes for deriving phonology from print. The lexical route requires the activation of memory representations of written word forms stored in the orthographic lexicon, followed by

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Orthography Lexical

Words

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Alexia. Figure 1 A cognitive model indicating the component processes involved in reading

the retrieval of the corresponding spoken word forms from the phonological lexicon. The lexical route is normally used to read familiar words and can support the processing of both regular words that have predictable spelling–sound relationships (e.g., spring) and irregular words that contain atypical letter–sound or grapheme– phoneme mappings (e.g., choir). By contrast, the sublexical route operates on units smaller than the whole word and is thought to rely on the serial conversion of individual graphemes to the corresponding phonemes. The sublexical route is essential for accurate reading of unfamiliar words or nonwords (e.g., nace) because these novel items, by definition, do not have preexisting representations in the orthographic or phonological lexicon. The sublexical route can also be used to generate plausible pronunciations for regular words that strictly obey spelling–sound conversion rules. However, processing irregular words by the sublexical procedure results in regularization errors (e.g., have read to rhyme with save). Thus, according to dual-route theory, only the lexical reading route can deliver a correct response to irregular words. Note that the model depicted in Fig. 1 also includes an indirect route from orthography to phonology via the semantic system. The activation of word meanings by this semantic reading route is critical for

written word comprehension. However, whether semantic mediation is also normally required for accurate oral reading of familiar words is a topic of controversy (Coltheart et al., 2001; Plaut, McClelland, Seidenberg, & Patterson, 1996; Woollams, Lambon Ralph, Plaut, & Patterson, 2007). In summary, skilled reading depends on interactions between visual/orthographic processing, phonology, and semantics. Damage to these functional domains or the disruption of the transfer of information between the cognitive/brain systems that support these operations results in alexia.

Epidemiology Alexia is commonly observed in right-handed individuals following damage to the language-dominant left hemisphere. Although it is most frequently caused by stroke, alexia can follow any kind of focal injury (e.g., trauma, tumor) to the brain regions critical for implementing the various cognitive operations necessary for normal reading. Alexia is also often seen in the setting of neurodegenerative disorders, especially in patients with primary progressive aphasia/semantic dementia or Alzheimer’s disease. In general, the specific alexia profile is determined

Alexia

not so much by the etiology of the brain damage than by the location of the responsible lesions.

Natural History, Prognostic Factors, Outcomes The prognosis for recovery from alexia depends both on the etiology of the lesion and the extent of the underlying brain damage. Alexia following stroke tends to show some spontaneous recovery over time, but patients with extensive brain damage may never regain useful reading function and typically stop reading for pleasure. In individuals with neurodegenerative disorders, progressive worsening of the reading impairment is observed along with the gradual deterioration of other language and cognitive functions.

Neuropsychology and Psychology of Alexia Pure Alexia/Letter-By-Letter Reading In pure alexia, the rapid visual identification of familiar words that characterizes normal skilled reading is disrupted. Reading is slow and laborious, often relying on a serial letter-naming strategy known as ‘‘letter-by-letter’’ reading. Typically, there is a monotonic increase in reading latencies as a function of the number of letters in the word, giving rise to an abnormal word length effect that is considered the hallmark feature of the syndrome. Varying degrees of letter identification difficulty are present and visual reading errors are common (e.g., chain – charm). Collectively, these behavioral observations suggest that visual processing impairment plays a critical role in the pathogenesis of pure alexia (Behrmann, Plaut, & Nelson, 1998). Although the reading disorder may be unaccompanied by significant aphasia or agraphia, many patients with pure alexia demonstrate concommitant anomia and spelling impairment (Rapcsak & Beeson, 2004). Furthermore, patients often perform poorly on nonreading tasks that require fine-grained visual discrimination, suggesting that the reading impairment is part of a more general visual processing deficit (Behrmann et al., 1998). Within the framework of the cognitive model depicted in Fig. 1, pure alexia is attributable to dysfunction at the visual feature analysis and/or letter identification stages of reading, or it may be produced by damage to the orthographic lexicon. Damage to any of these visual processing components would be expected to interfere with the rapid perceptual identification of familiar

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orthographic word forms and result in an abnormal word length effect in oral reading. Pure alexia/letter-by-letter reading is most commonly seen following left inferior occipito-temporal damage caused by posterior cerebral artery strokes. It has been proposed that the critical lesions degrade or disrupt visual input to the visual word-form area (VWFA) or directly damage the VWFA itself (Cohen et al., 2003; Epelbaum et al., 2008). The VWFA is consistently activated in functional imaging studies of reading in normal individuals and has been localized to the mid-lateral portions of the left fusiform gyrus (BA37) (Cohen et al., 2002; Jobard, Crivello, & Tzourio-Mazoyer, 2003) (Fig. 2). The VWFA receives converging input from bilateral posterior occipital areas (BA17,18/19) involved in visual feature analysis and letter shape detection and it integrates this information into larger perceptual units corresponding to whole words (Fig. 2). Activation of the VWFA is sensitive to the orthographic familiarity of the letter string, consistent with the notion that this cortical region may constitute the neural substrate of the orthographic lexicon. The orthographic codes computed by the VWFA are subsequently transmitted to cortical systems involved in the phonological and semantic components of reading (Fig. 3). Importantly, it has been shown that spelling familiar words also activates the VWFA (Beeson et al., 2003). These observations confirm the central role for the VWFA in orthographic processing and support the view that the same orthographic lexical representations mediate reading and spelling. Consistent with this hypothesis, patients with damage to the VWFA are likely to show evidence of reading and spelling impairment attributable to the loss of word-specific orthographic representations (Rapcsak & Beeson, 2004).

Phonological/Deep Alexia Phonological alexia is characterized by a disproportionate difficulty in processing nonwords compared with familiar words, giving rise to an exaggerated lexicality effect in reading (Crisp & Lambon Ralph, 2006; Patterson & Lambon Ralph, 1999; Rapcsak et al., 2009). Attempts to read nonwords often result in real word responses known as lexicalization errors (e.g., nace – name). Although in phonological alexia reading of familiar words (both regular and irregular) is relatively preserved, performance is typically influenced by lexical-semantic variables including word frequency (high>low), imageability (concrete>abstract), and grammatical class (nouns>verbs > functors). Deep alexia includes all the

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Alexia. Figure 2 Location of the visual word form area (VWFA) (indicated by green circle) as determined by functional neuroimaging studies of reading. This region receives input from bilateral posterior occipital visual areas (shown in purple). Arrow indicates callosal transfer of information initially processed by right visual cortex

Phonology Visual analysis

Semantics

Orthography

Alexia. Figure 3 Cortical regions involved in reading

Alexia

characteristic features of phonological alexia, but it is distinguished from the latter by the production of prominent semantic reading errors (e.g., boy – son) (Coltheart, Patterson, & Marshall, 1980). Although phonological and deep alexia were originally considered separate entities, there is now much evidence to suggest that the difference between these syndromes is quantitative rather than qualitative. Thus, phonological and deep alexia are more appropriately considered as points along a continuum, with the latter representing a more severe version of the former (Crisp & Lambon Ralph, 2006; Rapcsak et al., 2009). Phonological alexia is typically encountered in patients with aphasia syndromes characterized by phonological impairment (i.e., Broca’s, conduction, Wernicke’s). Furthermore, it has been shown that most patients with phonological alexia demonstrate prominent deficits and increased lexicality effects in spoken language tasks that require the manipulation and maintenance of sublexical phonological information (e.g., repetition, rhyme judgments, phoneme segmentation and blending), and also that such non-orthographic measures of phonological ability correlate with and are predictive of reading performance (Crisp & Lambon Ralph, 2006; Rapcsak et al., 2009). These observations suggest that the written and spoken language impairments in phonological alexia have a common origin and are merely different manifestations of a central or modality-independent phonological deficit (Crisp & Lambon Ralph, 2006; Patterson & Lambon Ralph, 1999; Rapcsak et al., 2009). Consistent with this view, the reading disorder in phonological alexia is usually accompanied by a qualitatively similar spelling impairment (phonological agraphia) (Rapcsak et al., 2009). According to dual-route models (Fig. 1), poor nonword reading in phonological alexia is attributable to damage to the sublexical route, while the relatively preserved real word reading performance of these patients reflects the residual functional capacity of the lexical and semantic routes. The general phonological impairment observed in the vast majority of patients suggests that the most common site of damage may be at the level of the phoneme units (with additional damage to the phonological lexicon in more severe cases), as these phonological processing components are shared between written and spoken language tasks. Phonological alexia is most often associated with damage to a network of perisylvian cortical regions involved in speech production/perception and phonological processing in general. Components of this distributed phonological system include posterior-inferior frontal gyrus/Broca’s area (BA44/45), precentral gyrus (BA4/6),

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insula, superior temporal gyrus/Wernicke’s area (BA22), and supramarginal gyrus (BA40) (Rapcsak et al., 2009). Consistent with the phonological deficit hypothesis, there is an excellent neuroanatomical correspondence between the location of the lesions that produce phonological alexia and the location of the perisylvian cortical areas that show activation in normal individuals during a variety of written and spoken language tasks requiring phonological processing (Jobard et al., 2003; Rapcsak et al., 2009; Vigneau et al., 2006). As predicted by the continuum model, there is considerable overlap between the perisylvian lesion profiles of patients with phonological and deep alexia, although the damage in deep alexia tends to be more extensive. In fact, the massive destruction of left-hemisphere language areas in deep alexia has lead to the hypothesis that reading performance in these patients may be mediated by the intact right hemisphere (Coltheart et al., 1980).

Surface Alexia In surface alexia the main difficulty involves reading irregular words, especially when these items are of low frequency. Regular words of comparable frequency are processed more efficiently, and the discrepancy in performance between words with predictable versus atypical spelling–sound relationships is reflected by an increased regularity effect in reading. Nonword reading is typically preserved. According to dual-route theory, surface alexia is attributable to dysfunction of the lexical reading route (Fig. 1). Specifically, it has been suggested that the reading disorder in some cases may result from damage to the orthographic lexicon (Coltheart et al., 2001; Patterson, Marshall, & Coltheart, 1985). Due to the loss of wordspecific orthographic knowledge, patients with this type of deficit will be forced to rely on a sublexical grapheme– phoneme conversion strategy that produces phonologically plausible regularization errors on irregular words. Low-frequency irregular words are especially vulnerable because the activation of representations in the orthographic lexicon is normally modulated by word frequency and the relative refractoriness of low-frequency items may be further exaggerated by the brain damage. Consistent with the notion that reading and spelling rely on shared orthographic representations, patients with surface alexia following damage to the orthographic lexicon show similar difficulty in spelling irregular words (surface agraphia) (Patterson et al., 1985; Rapcsak & Beeson, 2004). Alternatively, surface alexia may result from damage to central semantic representations (Woollams et al.,

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2007). Specifically, it has been proposed that accurate oral reading of low-frequency irregular words normally requires additional support from the semantic reading route and cannot be mediated efficiently by pathways that rely on direct transcoding between orthographic and phonological representations (Plaut et al., 1996). With the degradation of semantic knowledge, the relative inadequacy of non-semantic reading routes is revealed and manifests itself as surface alexia. Consistent with the semantic deficit hypothesis, many patients with surface alexia perform poorly on verbal and nonverbal cognitive tasks requiring semantic processing (e.g., picture naming, verbal fluency, spoken word and picture comprehension). Furthermore, the severity of the semantic impairment on these nonreading tasks has been shown to correlate with reading accuracy for low-frequency irregular words (Woollams et al., 2007). The proposed central semantic deficit may also explain the frequent co-occurrence of surface alexia and surface agraphia (Graham, Patterson, & Hodges, 2000). In contrast to the strong association between perisylvian damage and phonological alexia, surface alexia is typically encountered in the setting of extrasylvian brain pathology. Although uncommon in patients with stroke, surface alexia has been described in individuals with left temporo-parietal lesions centered on posterior middle/ inferior temporal gyrus and angular gyrus (BA20/21,37/ 39), and also following inferior occipito-temporal lesions that involved the VWFA (Rapcsak & Beeson, 2004; Vanier & Caplan, 1985). As expected, patients with surface alexia following VWFA damage also showed evidence of visual processing impairment and features of pure alexia/letterby-letter reading (Rapcsak & Beeson, 2004). A particularly dramatic and pure form of surface alexia is consistently observed in patients with semantic dementia (SD) (Woollams et al., 2007). SD is a subtype of primary progressive aphasia/frontotemporal dementia in which the neurodegenerative process has a predilection for left anterior and inferolateral temporal cortex, including the temporal pole, middle/inferior temporal gyri, and anterior fusiform gyrus (BA38,20/21) (Galton et al., 2001; Mummery et al., 2000). Surface alexia has also been described in patients with Alzheimer’s disease (Patterson, Graham, & Hodges, 1994) and is likely to reflect the frequent involvement of left temporo-parietal cortex by the disease process. Although distributed over a large anatomical area, the disparate extrasylvian lesion sites in surface alexia seem to have in common the potential for disrupting either lexical orthographic or semantic processing. Specifically, in patients with VWFA involvement the reading disorder

may reflect damage to the orthographic lexicon resulting in the loss of word-specific orthographic knowledge. By contrast, in patients with anterior temporal lobe lesions, and possibly also in patients with posterior temporoparietal damage, surface alexia may be attributable to the degradation of central semantic representations. The latter hypothesis is supported by functional imaging studies of semantic processing in normal individuals that have shown activation of a large-scale left-hemisphere extrasylvian cortical network that included both anterior temporal lobe and posterior temporo-parietal sites (Vigneau et al., 2006; Binder, Desai, Graves, & Conant, 2009) (Fig. 3).

Evaluation In evaluating patients with alexia it is important to assess the status of all the relevant component processes involved in reading (Fig. 1). A comprehensive battery should include tests of letter and word recognition, as well as measures of oral reading and reading comprehension. The evaluation should allow the clinician to identify the nature of the functional impairment and to locate the level of breakdown with reference to a cognitive model of normal reading. It is equally important to document relatively spared reading abilities and the use of compensatory strategies by the patient, as this information may be helpful in planning treatment. The assessment of alexia is best accomplished by the use of commercially available reading batteries (e.g., Kay, Lesser, & Coltheart, 1992).

Treatment A variety of behavioral treatment approaches have shown positive outcomes in the rehabilitation of alexia. In general, treatment is directed toward strengthening the impaired reading procedure/route or it encourages the use of compensatory strategies to bypass the functional deficit (for a review, see Beeson & Rapcsak, 2006).

Cross References ▶ Agraphia ▶ Aphasia ▶ Dyslexia ▶ Phonological/Deep Agraphia ▶ Surface Agraphia

Alexithymia

References and Readings Beeson, P. M., & Rapcsak, S. Z. (2006). Treatment of alexia and agraphia. In J. H. Noseworthy (Ed.), Neurological therapeutics: Principles and practice (2nd ed., pp. 3045–3060), London: Martin Dunitz. Beeson, P. M., Rapcsak, S. Z., Plante, E., Chargualaf, J., Chung, A., Johnson, S. C., et al. (2003). The neural substrates of writing: A functional magnetic resonance imaging study. Aphasiology, 17, 647–665. Behrmann, M., Plaut, D. C., & Nelson, J. (1998). A literature review and new data supporting an interactive account of letter-by-letter reading. Cognitive Neuropsychology, 15, 7–51. Binder, J. R., Desai, R. H., Graves, W. W., & Conant, L. L. (2009). Where is the semantic system? A critical review and meta-analysis of 120 functional neuroimaging studies. Cerebral Cortex, 19, 2767–2796. Cohen, L., Lehe´ricy, S., Chochon, F., Lemer, C., Rivaud, S., & Dehaene, S. (2002). Language-specific tuning of visual cortex? Functional properties of the visual word form area. Brain, 125, 1054–1069. Cohen, L., Martinaud, O., Lemer, C., Lehe´ricy, S., Samson, Y., Obadia, M., et al. (2003). Visual word recognition in the left and right hemispheres: Anatomical and functional correlates of peripheral alexias. Cerebral Cortex, 13, 1313–1333. Coltheart, M., Patterson, K., & Marshall, J. C. (1980). Deep dyslexia. London: Routledge & Kegan Paul. Coltheart, M., Rastle, K., Perry, C., Langdon, R., & Ziegler, J. (2001). DRC: A dual route cascaded model of visual word recognition and reading aloud. Psychological Review, 108, 204–256. Crisp, J., & Lambon Ralph, M. A. (2006). Unlocking the nature of the phonological-deep dyslexia continuum: The keys to reading aloud are in phonology ad semantics. Journal of Cognitive Neuroscience, 18, 348–362. Epelbaum, S., Pinel, P., Gaillard, R., Delmaire, C., Perrin, M., Dupont, S., et al. (2008). Pure alexia as a disconnection syndrome: New diffusion imaging evidence for an old concept. Cortex, 44, 962–974. Galton, C. J., Patterson, K., Graham, K., Lambon Ralph, M. A., Williams, G., Antoun, N., et al. (2001). Differing patterns of temporal atrophy in Alzheimer’s disease and semantic dementia. Neurology, 57, 216–225. Graham, N. L., Patterson, K., & Hodges, J. R. (2000). The impact of semantic memory impairment on spelling: Evidence from semantic dementia. Neuropsychologia, 38, 143–163. Jobard, G., Crivello, F., & Tzourio-Mazoyer, N. (2003). Evaluation of the dual route theory of reading: A metaanalysis of 35 neuroimaging studies. NeuroImage, 20, 693–712. Kay, J., Lesser, R., & Coltheart, M. (1992). Psycholinguistic assessments of language processing in aphasia (PALPA). East Sussex, England: Lawrence Erlbaum Associates. Mummery, C. J., Patterson, K., Price, C. J., Ashburner, J., Frackowiak, R. S. J., & Hodges, J. R. (2000). A voxel-based morphometry study of semantic dementia: Relationship between temporal lobe atrophy and semantic memory. Annals of Neurology, 47, 36–45. Patterson, K., Graham, N., & Hodges, J. R. (1994). Reading in dementia of the Alzheimer type: A preserved ability? Neuropsychology, 8, 835–407. Patterson, K., & Lambon Ralph, M. A. (1999). Selective disorders of reading? Current Opinion in Neurobiology, 9, 235–239. Patterson, K. E., Marshall, J. C., & Coltheart, M. (1985). Surface dyslexia: Neuropsychological and cognitive studies of phonological reading. London: Lawrence Erlbaum.

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Plaut, D. C., McClelland, J. L., Seidenberg, M. S., & Patterson, K. (1996). Understanding normal and impaired word reading: Computational principles in quasi-regular domains. Psychological Review, 103, 56–115. Rapcsak, S. Z., & Beeson, P. M. (2004). The role of left posterior inferior temporal cortex in spelling. Neurology, 62, 2221–2229. Rapcsak, S. Z., Beeson, P. M., Henry, M. L., Leyden, A., Kim, E. S., Rising, K., et al. (2009). Phonological dyslexia and dysgraphia: Cognitive mechanisms and neural substrates. Cortex, 45(5), 575–591. Vanier, M., & Caplan, D. (1985). CT correlates of surface dyslexia. In K. E. Patterson, J. C. Marshall, & M. Coltheart (Eds.), Surface dyslexia: Neuropsychological and cognitive studies of phonological reading (pp. 511–525). London: Lawrence Erlbaum. Vigneau, M., Beaucousin, V., Herve´, P. Y., Duffau, H., Crivello, F., Houde´, O., et al. (2006). Meta-analyzing left hemisphere language areas: Phonology, semantics, and sentence processing. NeuroImage, 30, 1414–1432. Woollams, A., Lambon Ralph, M. A., Plaut, D. C., & Patterson, K. (2007). SD-squared: On the association between semantic dementia and surface dyslexia. Psychological Review, 114, 316–339.

Alexia Without Agraphia ▶ Alexia

Alexithymia J OEL W. H UGHES Kent State University Kent, OH, USA

Definition A deficit in apprehending, experiencing, and describing emotions, including difficulty in perceiving and understanding the feelings of others. In particular, difficulty in distinguishing between emotions and bodily sensations that indicate emotional arousal.

Current Knowledge The term ‘‘alexithymia’’ was coined by the late psychiatrist Peter Sifneos to describe patients who could not find the appropriate words to describe their emotional states. Literally meaning ‘‘without words for emotions’’ in Sifneos’ native Greek, Alexithymia is a trait that overlaps

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with a number of medical and psychiatric disorders. Alexithymia is associated with somatic complaints such as headaches, lower back pain, irritable bowel syndrome, and fibromyalgia. It is also associated with psychiatric conditions such as anorexia nervosa, autism spectrum disorders including Asperger’s, major depressive disorder, panic disorder, posttraumatic stress disorder, and substance abuse.

Cross References ▶ Emotional Intelligence

References and Readings Sifneos, Peter E. Alexithymia: Past and present. The American Journal of Psychiatry, 153, 137–142. Taylor, Graeme J; Bagby, R. Michael and Parker, James DA (1997). Disorders of Affect Regulation: Alexithymia in Medical and Psychiatric Illness. Cambridge: Cambridge University Press. ISBN 052145610X. Taylor GJ, & Taylor HS (1997). Alexithymia. In M. McCallum & W.E. Piper (Eds.) Psychological mindedness: A contemporary understanding. Munich: Lawrence Erlbaum Associates.

Short Description or Definition Alien hand syndrome (AHS) is a relatively rare manifestation of damage to specific brain regions involved in voluntary movement. The core observation is the patient report that one of his/her hands is displaying purposeful, coordinated, and goal-directed behavior over which the patient feels he/she has no voluntary control. The patient fails to recognize the action of one of his hands as his own. The hand, effectively, appears to manifest a ‘‘will of its own.’’ This unique involuntary movement disorder is characterized by coordinated, well-organized, and clearly goal-directed limb movements that would otherwise be indistinguishable from normal voluntary movement. This definition excludes disordered, non-purposeful, and dyskinetic movements associated with other involuntary movement disorders such as chorea, athetosis, hemiballism, and myoclonus. The alien hand can be engaged in performing a specific goal-directed task or the purposeful use of an external object. Distinguishing this condition from asomatognosia, there is typically normal awareness and recognition of the limb reported by the patient. However, the patient perceives a lack of self-agency (‘‘I am not doing that. . .’’) with regard to the observed behavior of the limb, but displays intact ‘‘ownership’’ (‘‘. . .even though I know this is my hand’’).

Categorization

‘‘Alice in Wonderland’’ Syndrome ▶ Metamorphopsia

Three variants of AHS have been described, each with unique behavioral manifestations and neuroanatomical correlations. These variants include the frontal, callosal, and posterior forms.

Frontal Form

Alien Hand Syndrome G ARY G OLDBERG , M ATTHEW E. G OODWIN Virginia Commonwealth University School of Medicine/ Medical College of Virginia Richmond, VA, USA

Synonyms Anarchic hand; Callosal apraxia; Diagnostic dyspraxia; Dr. Strangelove syndrome; Intermanual conflict; Magnetic apraxia; Wayward hand

Neuroanatomy The most common variant is the ‘‘frontal’’ form. It is associated with damage to the medial surface of the cerebral hemisphere in the frontal region. This variant has been described in cerebral infarction in the territory of the anterior cerebral artery, with tumors involving the medial surface of the cerebral hemisphere, and in other conditions affecting the function of the medial frontal lobe region. When the region of injury extends posteriorly to involve the medial aspect of the prefrontal gyrus associated with the primary motor cortex (PMC), the patient may present with crural hemiparesis, with greater weakness in the leg as compared to the arm. This

Alien Hand Syndrome

presentation corresponds to the topographical organization of the PMC with control of lower limb movement located more medially than the areas that control the upper limb. The frontal variant is seen with involvement of the medial aspect of the premotor cortex anterior to PMC including the supplementary motor area (SMA) and anterior cingulate cortex (ACC). In functional activation studies, the medial frontal cortex has also been found to activate spontaneously with complex purposeful movements and with internal imaging of voluntary movement, suggesting that it may serve as a higher level system that modulates the activation of PMC in accordance with volitional aspects of the performance. The readiness potential that precedes an overt voluntary movement by over 1,000 ms arises through activation of the anteromedial frontal cortex, suggesting that excitation of this region precedes the appearance of the overt movement and activation of the PMC. Activation of the ACC is involved in intentional suppression of prepotent responses as tested with the Stroop test. These areas may serve as a higherlevel system modulating the activation of PMC in accordance with the volitional aspects of the performance.

Clinical Presentation Behaviors seen frequently with the frontal variant include involuntary, visually driven reaching and grasping onto objects, an inability to voluntarily release these objects, and utilization behavior in which the presence of a frequently encountered object such as a comb or a toothbrush elicits behavior in which the object may be put to use independent of the social context. A grasp reflex to tactile stimulation is often present in the affected hand. The patient may wake themselves up from sleep by grasping and pulling their own body parts. Patients may show a prepotent tendency to be drawn toward external objects. They also may demonstrate alien-associated sexual self-stimulation or involuntary fondling of another’s body, a great source of public embarrassment (Ong Hai and Odderson, 2000). Interestingly, while the patient clearly manifests purposeful involuntary coordinated behaviors in the affected limb, when they attempt to willfully move the limb, this is effortful and difficult. Voluntary movement in the affected limb is often hypokinetic and hypometric with greater activation of the axial and proximal limb muscles compared to the distal muscles controlling the wrist and fingers, even though these muscles are readily activated in the alien movements. Generally, these alien behaviors appear in the hand contralateral to the damaged hemisphere regardless of hemispheric dominance. When the dominant hemisphere is damaged, in addition to alien hand behavior in the

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nondominant hand, they may experience difficulty with the initiation of spontaneous speech while being able to follow verbal commands and repeat phrases without difficulty. These findings are consistent with a transcortical motor aphasia that affects spontaneous verbalization and production of propositional language more than repetition and responsive language. Alternatively, this could be understood as an inability to initiate spontaneous verbal output. The patient may thus be viewed as partially mute due to the relative akinesia seen with medial frontal cortex injury.

Callosal Form Neuroanatomy The ‘‘callosal’’ variant is seen with an isolated lesion of the corpus callosum. The voluntary motor systems of the two hemispheres are isolated from each other due to lost interhemispheric communication. This variant has been described most frequently as a transient condition following callosotomy. It may also be seen following infarction or tumors selectively involving this structure.

Clinical Presentation In the ‘‘callosal’’ variant of AHS, the appearance of ‘‘intermanual conflict’’ or ‘‘self-oppositional’’ behaviors is the predominant feature. Grasping behaviors and externally driven reaching movements seen in the frontal variant are notably less prominent. When there is a major disconnection between the two hemispheres resulting from callosal injury, the language-linked dominant hemisphere agent that maintains its primary control over the contralateral dominant limb effectively loses its direct and linked control over the separate ‘‘agent’’ based in the nondominant hemisphere (and, thus, the nondominant limb), which had been previously responsive and ‘‘obedient’’ to the dominant agent. The possibility of purposeful action in the nondominant limb occurring outside of the realm of influence of the dominant agent thus can occur. In the callosal variant, the problematic alien hand is consistently the nondominant hand, while the dominant hand is the identified ‘‘good’’ controlled hand. The patient may express frustration and bewilderment at the conflicting and disruptive behavior of the alien hand whose motivations remain inaccessible to consciousness. There may be an attentional component that modulates the appearance of these episodes of self-oppositional behavior since intermanual conflict is observed more frequently when the patient is fatigued, stressed, or is engaged in effortful multitasking activity. Occasionally,

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rather than acting in a contradictory manner, the two hands are observed to be engaged in two different and entirely unrelated activities as if being guided by completely separate and independent intentions. In a dramatic example of this behavior, one patient was observed to initiate smoking a cigarette by pulling the cigarette out of the package and placing it in her mouth with the controlled dominant hand followed by the alien nondominant hand, rather than beginning to light the cigarette, suddenly reaching up, pulling it out of the her mouth, and throwing it across the room. Astonished, the patient reasoned that perhaps the alien hand was not in favor of her smoking! The callosal and frontal variants are often seen in combination with a corresponding overlap of observed behaviors. For example, following cerebral infarction in the territory of the anterior cerebral artery, there may be ischemic injury to both the medial frontal lobe and the corpus callosum. In this circumstance, there may be both visually directed reaching and grasping alien behaviors in the limb contralateral to the area of injury as well as episodes of intermanual conflict. However, a clear differentiation between apparent intermanual conflict due to attempts to restrain alien behaviors associated with the frontal variant (e.g., as in the case of ‘‘self-grasping’’ described below), and true intermanual conflict, in which the two hands are directed toward independently contradictory purposes, may be difficult to differentiate.

from objects approaching the hand in distinct contrast to the reaching and grasping behaviors that are seen in the frontal variant. The alien hand may assume a characteristic posture of fully extended digits with the palmar surface retreating from environmental objects, an observation that has been labeled an ‘‘instinctive avoidance reaction’’ by Denny-Brown and has also been referred to as the ‘‘parietal hand.’’ At times, grasping behaviors can also be observed with the posterior variant. Alien hand behavior has also been reported in association with subcortical thalamic infarction. In addition to having been observed in the context of stroke, tumors and surgical sectioning of the corpus callosum, alien hand behavior has been described in association with a number of progressive neurodegenerative disorders including corticobasal degeneration, multiple sclerosis, spongiform encephalopathy, and Alzheimer’s disease. When AHS appears with these progressive encephalopathies, it is usually accompanied by various forms of motor apraxia, along with multiple additional cognitive disturbances characteristic of the particular condition.

Epidemiology While there are no epidemiologic studies of the occurrence of AHS variants in association with acquired brain damage, it can be assumed that this is a relatively rare but striking manifestation of neurologic pathology.

Posterior or ‘‘Sensory’’ Form Neuroanatomy The third identified variant of AHS is the ‘‘posterior’’ or ‘‘sensory’’ form, which appears most often with a parietal or parieto-occipital focus of circumscribed damage. As in the frontal variant, the alien behavior appears in the hand contralateral to the damaged hemisphere.

Clinical Presentation In the patient with the posterior variant, the movement of the affected alien limb is typically less organized and often has an ataxic instability particularly with visually guided reaching. The limb also may show proprioceptive sensory impairment with hypesthesia, so that kinesthetic impairment limits the monitoring of limb position. Visual field deficits as well as hemi-inattention may be seen on the same side as the alien hand. In this variant, the limb may be observed to lift up off of support surfaces involuntarily and ‘‘levitate’’ in the air seemingly to avoid contact with support surfaces. It may also be seen to withdraw

Pathophysiology and Prognosis Adapting the concept developed by Derek Denny-Brown regarding positive and negative cortical tropisms based in the parietal lobe and frontal lobes (Denny-Brown, 1956, 1966), respectively, a heuristic model has been proposed. In this model, there are two separable but interactive components of an intrahemispheric premotor intentional system that modulate the output of the PMC of the hemisphere and its direct influence over the spinal motor nuclei innervating the muscles of the contralateral distal upper limb (Goldberg and Bloom, 1990). The first component is a posterolateral premotor system (PLPS) based in the posterior parietal region that is involved in generating movements of the contralateral arm and hand that are directed toward external objects and are responsive to externally sensed contingencies. The second component is an anteromedial premotor system (AMPS) based in the medial frontal region that is involved in generating movements in the contralateral

Alien Hand Syndrome

upper limb that are directed by an internal action plan and driven by an anticipatory model of future contingencies. It presumably is also involved in activating withdrawal movements that pull the limb back and away from external stimuli. It also functions to withhold action directly responsive to surrounding objects through inhibitory influence over the PLPS. These two systems are proposed to be in a metastable balance through mutually inhibitory influence. Together, these two hemispheric agency systems form an integrated intrahemispheric agency system. Furthermore, each intrahemispheric agency system has the capability of acting autonomously in its control over the contralateral limb, although overall unitary control by a conscious agent is maintained through interhemispheric communication between these systems via the corpus callosum at the cortical level and other interhemispheric

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commissures linking the two cerebral hemispheres at the subcortical level. Thus, conscious human agency can be thought of as emerging through the linked and coordinated action of at least four major premotor systems, two in each hemisphere. The overall general configuration of this heuristic model is shown in Fig. 1. It is proposed that AHS, in its different variants described above, appears due to damage either to the corpus callosum in the callosal variant (Fig. 2), the AMPS of either hemisphere in the frontal variant (Figs. 3 and 4), or to the PLPS of either hemisphere in the posterior variant (Figs. 5 and 6). The common factor in these anomalous conditions is the relative sparing of the PMC region controlling the contralesional alien hand, while the premotor regions involved in the intentional selection of action and the

Alien Hand Syndrome. Figure 1. Heuristic model for understanding alien hand syndrome (AHS). Abbreviations: RH, Right Hemisphere; LH, Left Hemisphere; CC, Corpus Callosum; PMC, Primary Motor Cortex; AMPS, Anteromedial Premotor System; PLPS, Posterolateral Premotor System. This view is shown looking down from above the vertex with the face located at the top of the drawing and the back of the head noted at the bottom of the drawing, the left side to the left and the right side to the right of the diagram. The open bidirectional arrow between the AMPS and the PLPS indicates an interaction characterized by mutually interactive inhibition creating a complementary metastable control of the contralateral hand. Solid arrows indicate facilitatory connections or connections that maintain synchrony and coherence between the connected structures. Output from PMC is directed primarily to the contralateral limb with some less potent ipsilateral projections illustrated by a dotted line. See text for further detail. Note that the left hemisphere is stippled in the diagram designating this as the dominant hemisphere for most individuals in correspondence with a dominant right hand

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Alien Hand Syndrome. Figure 2. The callosal variant of AHS. Theoretical explanatory model for the alien behaviors observed in callosal damage. In this instance, there are findings consistent with callosal apraxia in addition to intermanual conflict associated with the complete separation of the two intrahemispheric premotor intentional control systems. The limbs appear to be operated by two relatively autonomous control systems. The intentional premotor system in the dominant hemisphere is linked to the language system while that of the nondominant hemisphere is separated from it. The dominant hand is understood as connected to self while the nondominant hand is not. The alien hand in this variant is the nondominant hand. This is indicated by the stippled overlay on the left nondominant hand

inhibition of automatic behaviors in response to external factors are impaired. A recent fMRI study of cortical activation patterns associated with alien and non-alien movement has demonstrated that alien movement is in fact characterized by isolated activation of PMC without concomitant activation of intrahemispheric premotor regions, while voluntary behavior includes the activation of PMC in concert with activation of intrahemispheric premotor regions (Assal, Schwartz, & Vuilleumier, 2007).

Neuropsychology and Psychology of AHS The presence of AHS can cause the patient significant psychological distress as the hand seems to possess the capability for acting autonomously, independent of their

conscious voluntary control. The patient may become fearful that they will be held accountable for consequences of an action of the alien hand over which they do not feel control. The patient may display ‘‘auto-criticism’’ complaining that the alien hand is not doing what it has been ‘‘told to do’’ and is therefore characterized as disobedient, wayward, or ‘‘evil.’’ They may even physically strike the alien hand with the controlled hand as a ‘‘punishment’’ intended to discourage its wayward behavior, or constrain the movement of the alien hand by grasping tightly onto it with the controlled hand (‘‘self-grasping’’). They may verbally address and instruct the hand as if it were an unruly child acting autonomously and in need of correction. Conversely, they may respond to these contrary actions with amusement. Given the predicament created, the patient may develop depersonalization and dissociate themselves from the unintended actions of the hand. They often choose to

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Alien Hand Syndrome. Figure 3. The nondominant frontal variant of AHS. Theoretical explanatory model for the alien behaviors observed in the frontal variant associated with damage to the AMPS of the nondominant hemisphere. In this case, the contralesional nondominant hand develops alien hand findings due to the release by disinhibition of the reaching and grasping behaviors driven from the dominant PLPS

Alien Hand Syndrome. Figure 4. The dominant frontal variant of AHS. Theoretical explanatory model for the alien behaviors observed in the frontal variant associated with damage to the AMPS of the dominant hemisphere. In this case, the contralesional dominant hand develops alien hand findings due to the release by disinhibition of the reaching and grasping behaviors driven from the dominant PLPS. In addition, spontaneous expressive language initiation is impaired due to the role of the AMPS of the dominant hemisphere in the initiation of verbal output

Alien Hand Syndrome. Figure 5. The nondominant posterior variant of AHS. Theoretical explanatory model for the alien behaviors observed in the posterior variant associated with damage to the PLPS of the nondominant hemisphere. In this case, the contralesional nondominant hand develops alien hand findings due to the release by disinhibition of behaviors driven from the nondominant AMPS

Alien Hand Syndrome. Figure 6. The dominant posterior variant of AHS. Theoretical explanatory model for the alien behaviors observed in the posterior variant associated with damage to the PLPS of the dominant hemisphere. In this case, the contralesional dominant hand develops alien hand findings due to the release by disinhibition of behaviors driven from the dominant AMPS

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identify an external ‘‘alien’’ source for the voluntary control of the hand, or assign a distinct personality to the hand as a way of seeking a satisfactory narrative to explain this perplexing situation. From a psychological perspective, it is helpful to counsel the patient regarding the organic basis of their problem and provide assurance that there is a rational explanation for their concerns and that there is evidence that these problems can be treated and may gradually improve over time. In AHS, different regions of the brain are able to command purposeful limb movements, without generating the conscious feeling of self-control over these movements. There is thus a dissociation between the actual execution of the physical movements of the limb and the process that produces an internal sense of voluntary control over the movements. This latter process, impaired in AHS, normally produces the conscious sensation that movement is being internally initiated and produced by an active self. Presumably, this process differentiates between ‘‘re-afference’’ (i.e., the return of kinesthetic sensation from the self-generated ‘‘active’’ limb movement) and ‘‘ex-afference’’ (i.e., kinesthetic sensation generated from an externally produced ‘‘passive’’ limb movement). It may do this by giving rise to a parallel output signal from motor regions, a so-called ‘‘efference copy.’’ The efference copy is then translated into a corollary discharge, which conveys the expected re-afferent sensory response from the commanded movement. The corollary discharge can then be used in somatosensory cortex to distinguish re-afference from ex-afference and thus differentiate a self-produced active movement from a movement resulting from external forces. AHS may thus involve impaired production and transmission of either an efference copy or a corollary discharge signal.

Evaluation Evaluation of the patient with AHS involves careful observation of limb movement in various naturalistic contexts, along with reports from the patient regarding their sense of control over these movements. The relative dependence of movement on external context should be evaluated through assessment for utilization behaviors elicited by the presentation of external objects commonly encountered in daily activities. A phenomenologic approach to assessing and documenting the motor behavior and linking it to introspective report from the patient is essential. Not only should the verbal reports of the patient be noted, but also the associated affect. The limb should be

evaluated for evidence of a grasp reflex with both tactile and visual stimulation. The ability to release objects that have been grasped should also be assessed. Evaluation for callosal apraxia and impairment of interhemispheric transfer of information should be included. When the posterior variant of AHS is suspected, a visual field assessment and somatosensory examination of the affected limb should be completed as well as assessment for hemi-inattention. Evidence of a tendency to withdraw the limb from tactile and visual stimulation should also be elicited and noted.

Treatment There is no definitive specific treatment for AHS but a number of different rehabilitative approaches have been described. Furthermore, in the presence of unilateral damage within a single cerebral hemisphere, there is often a gradual reduction in the frequency of alien behaviors observed over time and a gradual restoration of voluntary control over the affected hand. This suggests that neuroplasticity in the bihemispheric and subcortical brain systems involved in voluntary movement production can serve to reestablish functional connection between the executive production process and the internal self-generation and volitional registration process. Exactly how this may occur is not well understood but could involve a reorganization within residual elements of the intrahemispheric premotor systems both at the cortical and subcortical levels. In addition, some degree of expanded participation of the intact ipsilateral hemisphere may be involved in the recovery process by extending ipsilateral motor projections. Different strategies can be used to reduce the interference of the alien hand behavior in the ongoing coherent controlled actions being performed by the patient. In the frontal variant, an object such as a cane can be placed in the grip of the alien hand so that it does not reach out to grasp onto other objects, thus impeding the patient’s forward progress during walking. In another approach, voluntary control of the limb is developed by training the patient to perform a specific task with the alien limb, such as moving the alien hand to contact a specific object or a highly salient environmental target. Through training to enhance volitional control, the patient can effectively override the alien behavior when it occurs. Recognizing that alien behaviors in the frontal variant are often sustained by tactile input, another approach involves simultaneously ‘‘muffling’’ the actions of the alien hand and limiting sensory feedback by placing it in a restrictive ‘‘cloak’’ such as a specialized soft foam hand orthosis or,

Allele

alternatively, an everyday oven mitt. Of course, this then limits the degree to which the hand can engage in functional goals. It may also be possible to develop improved participation of ipsilateral hemispheric premotor mechanisms by engaging the patient in coordinated bimanual activities that necessitate cooperative coordination mechanisms within residual intact components of the motor control system in both hemispheres.

Cross References ▶ Anterior Cingulate ▶ Apraxia ▶ Corpus Callosum ▶ Environmental Dependency ▶ Movement Disorder ▶ Utilization Behavior

References and Readings Assal, F., Schwartz, S., & Vuilleumier, P. (2007). Moving with or without will: Functional neural correlates of alien hand syndrome. Annals of Neurology, 62, 301–306. Biran, I., & Chatterjee, A. (2004). Alien hand syndrome. Archives of Neurology, 61, 292–294. Denny-Brown, D. (1956). Positive and negative aspects of cerebral cortical functions. North Carolina Medical Journal, 17, 295–303. Denny-Brown, D. (1966). The cerebral control of movement. Liverpool: Liverpool University Press. Frith, C. D., Blakemore, S.-J., & Wolpert, D. M. (2000). Abnormalities in the awareness and control of action. Philosophical Transactions of the Royal Society of London, 355, 1771–1788. Giovanetti, T., Buxbaum, L. J., Biran, I., & Chatterjee, A. (2005). Reduced endogenous control in alien hand syndrome: Evidence from naturalistic action. Neuropsychologia, 43, 75–88. Goldberg, G., & Bloom, K. K. (1990). The alien hand sign. Localization, lateralization, and recovery. American Journal of Physical Medicine and Rehabilitation, 69, 228–238. Goldberg, G. (1992). Premotor systems, attention to action and behavioural choice. In J. Kien, C. McCrohan, & W. Winlow (Eds.), Neurobiology of motor programme selection. New approaches to mechanisms of behavioural choice (pp. 225–249). Oxford: Pergamon. Ong Hai, B. G., & Odderson, I. R. (2000). Involuntary masturbation as a manifestation of stroke-related alien hand syndrome. Archives of Physical Medicine and Rehabilitation, 79, 395–398. Pack, B. C., Stewart, K. J., Diamond, P. T., & Gate, S. D. (2002). Posteriorvariant alien hand syndrome: Clinical features and response to rehabilitation. Disability and Rehabilitation, 24, 817–818. Scepkowski, L. A., & Cronin-Golomb, A. (2003). The alien hand: Cases, categorizations, and anatomical correlates. Behavioral and Cognitive Neuroscience Reviews, 2, 261–277. Sumner, P., & Husain, M. (2008). At the edge of consciousness: Automatic motor activation and voluntary control. Neuroscientist, 14, 474–486.

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ALL ▶ Acute Lymphoblastic Leukemia

Allele J OHN D E LUCA Kessler Foundation Research Center West Orange, NJ, USA

Definition Allele is an alternate form of a gene, which is the basic unit of inheritance. A gene is located at a particular site on the chromosome, and can have several alleles for that locus. For example, A, B, and O are different alleles for the ABO blood-type marker locus of a gene. Alleles greatly influence the expression of physical and behavioral phenotypes or traits such as eye color. For instance, the apolipoprotein E (APoE) gene is a well-known risk factor for developing Alzheimer’s disease. The APoE gene has three common alleles: epsilon 2, epsilon 3, and epsilon 4. There is some evidence that carriers of the APoE epsilon 4 allele are at a greater risk for the development of Alzheimer’s disease. In contrast, the APoE epsilon 3 allele has been suggested as a ‘‘protective’’ factor in the development of Alzheimer’s disease (Plomin, Defries, Craig, & McGuffin, 2003).

Cross References ▶ Alzheimer’s Disease ▶ Apolipoprotein E (ApoE) ▶ Chromosome ▶ Deoxyribonucleic Acid (DNA) ▶ Gene ▶ Phenotype

References and Readings Plomin, R., Defries, J. C., Craig, W., & McGuffin, P. (2003). Behavioral genetics in the postgenomic era. Washington, DC: American Psychological Association.

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Allesthesia

Allesthesia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition Misperception of the location of a stimulus. Although it can occur in other modalities, it is most commonly elicited by tactile stimulation and is often seen in the presence of other symptoms of unilateral asomatognosia. If a tactual stimulus is applied to the side of the body contralateral to a hemispheric lesion, the allesthetic patient may perceive the nature of the stimulus correctly but identify it as being applied to the comparable area on the opposite (unaffected) side of the body. In some instances the stimulus may be perceived as being on the same side of the body to which it was applied, but displaced significantly from the point of the actual stimulation (usually toward the midline). When present, this phenomenon likely results from post-rolandic (parietal) lesions of the right rather than the left hemisphere. More rarely it has been associated with brainstem lesions.

Current Knowledge Allokinesia is often associated with neglect syndromes, usually involving damage to the right hemisphere. It is the motor counterpart of alloesthesia. Typically, a patient moves the right limb in response to a request to move the left limb or moves towards the right, away from the neglected side, when asked to move toward the neglected side. In animal models, the phenomena has been associated with frontal, arcuate gyrus lesions (Heilman, Valenstein, Day, & Watson, 1995) and disconnections of frontal and posterior parietal cortices (Burcham, Corwin, Stoll, & Reep, 1997).

Cross References ▶ Allesthesia ▶ Neglect Syndrome

References and Readings Burcham, K. J., Corwin, J. V., Stoll, M. L., & Reep, R. L. (1997). Disconnection of medial agranular and posterior parietal cortex produces multimodal neglect in rats. Behavioural Brain Research, 86(1), 41–47. Heilman, K. M., Valenstein, E., Day, A., & Watson, R. (1995). Frontal lobe neglect in monkeys. Neurology, 45(6), 1205–1210.

Cross References ▶ Asomatognosia

Alpha Rhythm Allokinesia D OUGLAS I. K ATZ Boston University School of Medicine Boston, MA, USA

C INDY B. I VANHOE , N ATASHA K. E ADDY Baylor College of Medicine Houston, TX, USA

Synonyms Alpha waves; Berger’s waves

Definition Definition This phenomenon refers to a motor response in the wrong limb, contralateral to the requested side, sometimes opposite to the direction requested.

Electromagnetic oscillations in the frequency range of 8–12 Hz arising from synchronous and coherent electrical

Alprazolam

activity of the thalamic pacemaker cells in the human brain. Also called Berger’s wave.

Current Knowledge Alpha waves are believed to arise from the white matter of the occipital lobes. They increase during periods of relaxation with eyes closed. Alpha waves are thought to represent activity in the visual cortex and are associated with feelings of calmness and relaxation. Alpha waves increase when eyes are closed and during meditation and are associated with creativity and mental coordination.

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Alprazolam J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Alprazolam

Brand Name Xanax, Xanax XR

References and Readings Bragatti, J. A., De Moura Cordova, N., Rossato, R., & Bianchin, M. M. (2007). Alpha coma and locked-in syndrome. Journal of Clinical Neurophysiology, 24(3), 308. Min, B. K., Busch, N. A., Debener, S., Kranczioch, C., Hansimayr, S., Engel, A. K., et al. (2007). The best of both worlds: Phase reset of human EEG alpha activity and additive power contribute to ERP generation. International Journal of Psychophysiology, 65(1), 58–68.

Alpha Waves ▶ Alpha Rhythm

Class Benzodiazepine

Proposed Mechanism(s) of Action Binds to benzodiazepine receptors at the GABA-A ligandgated channel, thus allowing for neuronal hyperpolarization. Benzodiazepines enhance the inhibitory action of GABA via boosted chloride conductance.

Indication Generalized Anxiety and Panic Disorders

Off Label Use

Alphabetic Principle ▶ Phonics

Other anxiety disorders, irritable bowel syndrome, insomnia, adjunctive treatment in mania and psychosis, premenstrual dysphoric disorder.

Side Effects

Alpha-Synuclein Inclusions ▶ Lewy Bodies

Serious Respiratory depression, hepatic dysfunction (rare), renal dysfunction and blood dyscrasias, grand mal seizures

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ALS

Common Sedation, fatigue, depression, dizziness, memory problems, disinhibition, confusion, ataxia, slurred speech

References and Readings Physicians’ Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson PDR. Stahl, S. M. (2007). Essential psychopharmacology: The prescriber’s guide (2nd ed.). New York, NY: Cambridge University Press.

Additional Information Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

ALS ▶ Anterolateral System

Altered Testing Procedures ▶ Modified Testing

Alternate Forms ▶ Polymorphism

Alternate Test Forms K YLE E. F ERGUSON 1, G RANT L. I VERSON 2 1 University of British Columbia Vancouver, BC, Canada 2 University of British Columbia & British Columbia Mental Health & Addiction Services Vancouver, BC, Canada

Synonyms Equivalent forms; Parallel forms

Definition

ALSFRS ▶ Amyotrophic Lateral Sclerosis Functional Rating Scale

ALSFRS-R ▶ Amyotrophic Lateral Sclerosis Functional Rating Scale

Alterations ▶ Polymorphism

Altered ▶ Transgenic

Alternate test forms are designed to avoid or reduce content- or item-specific practice effects that are associated with repeated administrations of the same neuropsychological test(s) (Benedict & Zgaljardic, 1998). Examination of the manuals for many intellectual and neuropsychological tests illustrate that practice effects are common, especially over brief retest intervals (e.g., days or weeks). Regarding test construction, alternate test forms should include the same number of items, and the items should be of equivalent difficulty. Moreover, the test instructions, time limits, examples, and format should be identical to the original instrument developed during standardization, to reduce measurement error (Jackson, 2009). Of course, measurement error can never be eliminated. For example, content-sampling error and time-sampling error – inherent in all test–retest paradigms – are always concerns in developing alternate test forms (Strauss, Sherman, & Spreen, 2006). Additionally, alternate test forms cannot control other factors such as positive carry-over effect (i.e., developing better test-taking strategies), familiarity with the testing context (i.e., novelty

Alternate Test Forms

effects), performance anxiety, and regression to the mean, among others (Benedict & Zgaljardic, 1998; Busch, Chelune, & Suchy, 2006; Salinsky, Storzbach, Dodrill, & Binder, 2001). This might, to some extent, explain why some studies show that alternate test forms reduce or eliminate practice effects, whereas other studies do not.

Current Knowledge Alternate test forms are developed by administering an equivalent test – comprising items of similar difficulty – to the same group of examinees or normative sample, shortly before or after being administered the original test form. Scores from the two forms are then correlated (This is called alternate form reliability, or equivalent or parallel form reliability), which yields a reliability coefficient – otherwise known as the coefficient of equivalence. If the original and alternate test forms are truly equivalent, then there would be (theoretically) a one-to-one correspondence between the two sets of scores (Petersen, 2008). Moreover, their means and variances would also be very similar. Therefore, the coefficient of equivalence should be high (i.e., >0.80; Sattler, 2001). Of course, though they appear similar, the two forms are often not of equivalent difficulty, or otherwise parallel. Thus, in the absence of employing special empirical procedures like test equating, which ‘‘fine-tune the test construction process’’ (Petersen, 2008, p. 99), the two forms cannot be used interchangeably. Test equating refers to a class of statistical concepts and procedures that adjust for differences in difficulty level on alternate test forms (Please note that these procedures adjust for differences in test difficulty, not differences in content (see Kolen & Brennan, 2004)), so that the forms can be used interchangeably (see Kolen & Brennan, 2004, pp. 2–3, for a discussion of this procedure; White & Stern, 2003). Test equating establishes, empirically, ‘‘a relationship between raw scores on two test forms that can then be used to express the scores on one form in terms of the scores on the other form’’ (Petersen, Kolen, & Hoover, 1989, p. 242; see also Dorans & Holland, 2000; Petersen, 2008). Common types of test equating are Item Response Theory (IRT), linear, and equipercentile (Ormea, Reeb, & Riouxc, 2001). The Neuropsychological Assessment Battery (Stern & White, 2003), Hopkins Verbal Learning Test-Revised (Brandt & Benedict, 2001), Brief Visuospatial Memory Test-Revised (Benedict, 2001), and Wide Range Achievement Test-Fourth Edition (Wilkinson & Robertson, 2006) are several examples of tests (or test batteries) that

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provide alternate test forms. With the above caveats in mind, alternate test forms can be useful in serial neuropsychological evaluations.

Cross References ▶ Item Response Theory ▶ Reliable Change Index ▶ Test Construction ▶ Test Reliability and Validity

References and Readings Benedict, R. H., & Zgaljardic, D. J. (1998). Practice effects during repeated administrations of memory tests with and without alternate forms. Journal of Clinical and Experimental Neuropsychology, 20(3), 339–352. Benedict, R. H. B. (2001). Brief visuospatial memory test - revised. Odessa, FL: Psychological Assessment Resources. Brandt, J., & Benedict, R. H. B. (2001). Hopkins verbal learning test-revised. Odessa, FL: Psychological Assessment Resources. Busch, R. M., Chelune, G. J., & Suchy, Y. (2006). Using norms in neuropsychological assessment. In D. K. Attix & K. A. Welsh-Bohmer (Eds.), Geriatric neuropsychology: Assessment and intervention (pp. 133–157). New York: Guilford. Dorans, N. J., & Holland, P. W. (2000). Population invariance and equitability of tests: Basic theory and the linear case. Journal of Educational Measurement, 37, 281–306. Jackson, S. L. (2009). Research methods and statistics: A critical thinking approach (3rd ed.). Belmont, CA: Wadsworth Cengage Learning. Kolen, M. J., & Brennan, R. L. (2004). Test equating, scaling, and linking: Methods and practices (2nd ed.). New York: Springer. Ormea, D., Reeb, M. J., & Riouxc, P. (2001). Premorbid IQ estimates from a multiple aptitude test battery: Regression vs. equating. Archives of Clinical Neuropsychology, 16, 679–688. Petersen, N. S. (2008). A discussion of population invariance of equating. Applied Psychological Measurement, 32, 98–101. Petersen, N. S., Kolen, M. J., & Hoover, H. D. (1989). Scaling, norming, and equating. In R. L. Linn (Ed.), Educational measurement (3rd ed., pp. 221–262). New York: Macmillan. Salinsky, M. C., Storzbach, D., Dodrill, C. B., & Binder, L. M. (2001). Test-retest bias, reliability, and regression equations for neuropsychological measures repeated over a 12–16-week period. Journal of the International Neuropsychological Society, 7(5), 597–605. Sattler, J. M. (2001). Assessment of children: Cognitive applications (4th ed.). San Diego: Jerome M. Sattler. Stern, R. A., & White, T. (2003). Neuropsychological assessment battery. Lutz, FL: Psychological Assessment Resources. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests: Administration, norms, and commentary (3rd ed.). New York: Oxford University Press. White, T., & Stern, R. A. (2003). Neuropsychological assessment battery: Psychometric and technical manual. Lutz, FL: Psychological Assessment Resources. Wilkinson, G. S., & Robertson, G. J. (2006). Wide range achievement test (4th ed.). Lutz, FL: Psychological Assessment Resources.

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Alzheimer, Alois (1864–1915)

Alzheimer, Alois (1864–1915) K ATHERINE S. M C C LELLAN 1, A NNA B ACON M OORE 2 1 Atlanta Veterans Affairs Medical Center Decatur, GA, USA 2 Emory University School of Medicine Atlanta, GA, USA

Major Appointments

Intern – Mental Asylum at Frankfurt am Main, 1888– 1895 Senior Physician – Mental Asylum at Frankfurt am Main, 1895–1903 Researcher – Royal Psychiatric Clinic and District Mental Asylum, Munich, 1903–1912 Assistant Professor – Ludwig-Maximilian University, Munich, 1904–1912 Chief Physician – Royal Psychiatric Clinic and District Mental Asylum, Munich, 1906–1909 Professor of Psychiatry – Psychiatry Clinic of Silesian Friedrich-Wilhelm University, Breslau, 1912–1915


Extraordinary Professor, Ludwig-Maximilian University (1909) Geheimer Ministerialrat (Cabinet Councillor) (1915)


Alois Alzheimer was both an excellent clinician and a notable researcher. He is best remembered for being the first to definitively describe the symptoms and cerebral lesions of the disease now known as Alzheimer’s Disease. Nonetheless, his contributions to science and medicine did not begin, nor do they end, there. He was one of the leaders of the movement to implement the nonrestraint principle (explained more fully below) in asylums. His neurohistological work advanced the idea that psychiatric diseases were biological in origin. And, through his roles as both doctor and scientist, he contributed to our understanding of a variety of conditions such as cerebral atherosclerosis, alcoholism, and general paresis.

Short Biography In the German municipality Marktbreit, Alois Alzheimer was born on June 14, 1864 to Eduard and Theresia Alzheimer. Eduard, a Royal Notary, provided his family with a comfortable upbringing. Although Alois had only an older brother when he was born, six more siblings followed him. Alois spent the first four years of his education at Catholic school in Marktbreit, until his family left the area to find a new home with superior educational opportunities for the children. The family’s chosen residence was in Aschaffenburg, and in 1874, Alois moved there in order to study at the Royal Humanistic Gymnasium. Alois completed his high-school degree in 1883 with excellent grades. He then decided to study medicine because of his aptitude and fondness for the natural sciences, as well as a sense of duty to mankind. He enrolled at the Royal Friedrich Wilhelm University in Berlin for the 1883–1884 winter semester. In his psychiatry lecture there, he learned of John Conolly’s nonrestraint principle. Also called open treatment, the nonrestraint principle proposed the novel view that the mentally ill should be treated with a minimal amount of physical constraint. Although Berlin was the medical capital of Germany, Alois disliked Berlin and its distance from his family. Therefore, he was transferred to the University of Wu¨rzburg (Lower Franconia, Germany), where his older brother was studying. As an aside, due to the influence of his older brother, Alois joined and later held several officer positions in the Franconian Corps. His histology professor, Alfred von Ko¨lliker, gave him his first experience with microscopes and staining techniques, which lead to his passion for forensic psychiatry. In the fall of the following year, Alois left to spend his winter semester at the Eberhard Karls University of Tu¨bingen. He returned in 1887 to the Wu¨rzburg Anatomical Institute’s department of microscopy to write his doctoral thesis, ‘‘On the Earwax Glands.’’ The intricate figures he presented in the paper, as in all his papers, were proof of how scrupulously he conducted his research and clinical work. With the completion of his thesis, Alois Alzheimer received his doctor of medicine degree. He passed the state medical examination and was awarded a license to practice medicine in 1888. Shortly thereafter, he became a personal physician to a mentally ill woman and traveled with her for five months. Emil Sioli, the director of the Municipal Asylum for the Insane and Epileptic in Frankfurt am Main had advertized for an intern, specifically hoping for a competent doctor


who was also adept with a microscope. Upon his return, the 24-year old Dr. Alzheimer was hired immediately. Dr. Franz Nissl also was hired as senior physician for the asylum. Nissl not only became one of Alzheimer’s closest friends, but also taught him a powerful staining technique for highlighting neuronal cell bodies (the Nissl stain), that helped Alzheimer achieve success in his histological studies. Sioli’s main goal for the asylum was to fully employ the nonrestraint principle. Alzheimer was particularly skilled at gaining the trust of patients through conversation, and he often documented these conversations. The dialogues often were central to diagnosing a patient, and even more so to research. His talent in clinical interviewing was such that clinicians who later read his notes had sufficient information to evaluate his opinions and to make their own diagnoses. Alzheimer drew on his microscopy and forensic psychiatry training, to do histological investigations into the physical origins of psychiatric disorder. In Frankfurt, his topics of study included epilepsy, senile dementia, criminal minds, and a variety of psychoses. He established himself as a well-rounded physician by publishing papers on a wide variety of topics. Aside from his duties as a physician and researcher, he also appeared as an expert before courts and presented at many scientific meetings. While at Frankfurt, Alzheimer became an expert on general paresis, which later became the subject of his postdoctoral thesis. In Algeria, a personal physician who had been traveling with a man suffering from general paresis sent a telegram to Alzheimer in 1892 to request that he treat the worsening patient. Alzheimer obliged and went to North Africa. He intended to bring the patient back to his hospital in Germany, but the patient died before reaching Germany, leaving his wife, Cecilie, a widow. Alzheimer and Cecilie became close friends, and eventually the widow asked him to marry her. They were married in April 1894 in the registry office of Frankfurt. Because Cecilie was Jewish, she had to convert to Catholicism before the two could be married by the church in February 1895. On March 10, 1895, their first child, Gertrud, was born, and Dr. Nissl was chosen to be her godfather. But Nissl soon moved to work with Emil Kraepelin in Heidelberg. Nissl’s departure created room for Alzheimer to be promoted to senior physician within Sioli’s asylum. Also that year, to lessen the overcrowding of the main hospital, a new branch asylum opened. With this addition, Sioli and Alzheimer furthered their goal of fully implementing the nonrestraint principle by instituting duration baths rather than isolation. The asylum became known as a revolutionary clinic, and it elevated the

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reputations of all its doctors. But above all, in 1901, Alzheimer met the patient who would immortalize his name: Auguste D. Auguste had been admitted to the asylum because of delusional and excessively forgetful behavior. Although at admission she was disoriented, anxious, and suspicious, over time she became unruly and disruptive. Alzheimer was particularly intrigued by her case for the duration of her stay in the hospital. Alzheimer’s second child, Hans, was born in 1896, and his third, Maria, was born in 1900. However, the lavish lifestyle he had lived with Cecilie ended when she died in February 1901. Alzheimer’s sister, Elisabeth, took over his household. Though she was strict, she became an integral part of the family. Without Cecilie, Alzheimer no longer had a reason to stay in Frankfurt. After his application to be director of a regional asylum was rejected, he joined Nissl in Heidelberg in 1903 and went to work for Emil Kraepelin. The group he joined there was an international team of researchers. Later that same year, Kraepelin was named director of the Royal Psychiatric Clinic and the District Mental Asylum in Munich. Alzheimer followed him, but was not paid in Munich due to the lack of a position for him, and also his desire to manage his own time. Despite his absence from Frankfurt, Alzheimer still received updates on Auguste D. By this point, Alzheimer’s thesis on general paresis was finished, but because he moved twice in such a short time, he had not yet turned it in. Alzheimer submitted his postdoctoral thesis to the Ludwig-Maximilian University in Munich with the hopes of gaining associate professorship. In it, he published not only his clinical dialogues, but also his postmortem histological findings. With this paper, he asserted that histological examinations could definitively show the presence of general paresis. Until then, few doctors suspected that syphilis was a cause of general paresis, but shortly thereafter the link between the two was found. His work was surpassed by the discovery of a way to diagnose syphilis, without resorting to autopsies. In August 1904, he joined the university’s medical faculty. Because of his experience at remodeling the Frankfurt clinic, Alzheimer was fundamental in finishing the plans for the new Munich clinic. He furnished his anatomic laboratory with the best equipment and the brightest students – many of whom went on to make great contributions to science, including Ugo Cerletti – electrical shocks to generate convulsions, Hans Gerhard Creutzfeldt and Alfons Jakob – Creutzfeldt-Jakob disease, Frederic Lewy – Lewy bodies, and others. Alzheimer was made

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chief physician in 1906, a paid position, but also one that took away much of his time in the laboratory. Two topics that consumed Alzheimer in Munich were psychiatric symptoms resulting from pathological anatomy and classification of mental illnesses by etiology. The latter faced much opposition from the scientific community. Yet, the most opposition he ever faced was his presentation of the Auguste D. case. Auguste D. had always fascinated Alzheimer. He had paid special attention to her, taking copious notes about their conversations. When he moved away, he still received updates about her condition, which worsened progressively until her death. When Auguste D. died in 1906, her files, brain, and spinal cord were sent to Munich. Alzheimer, along with his student Gaetano Perusini, immediately began examining the case. In Tu¨bingen, Alzheimer presented her case in a lecture entitled ‘‘On a Peculiar Severe Disease Process of the Cerebral Cortex,’’ in which he described the lesions (now known to be neurofibullary tangles) that he believed caused Auguste’s symptoms. Based on records from the time, his peers did not bother to ask questions, nor were there any comments about the lecture in the minutes. He later published the entire lecture, but still it received little attention. He then tasked Perusini to find more patients, similar to Auguste D. in the clinic. Perusini found four cases and published an article entitled ‘‘On Clinically and Histologically Peculiar Mental Illnesses in Advanced Age.’’ Another student of Alzheimer’s, Francesco Bonfiglio found another case of presenile dementia, and also published on the disease. Spurred by Bonfiglio’s paper, Kraepelin included a section on ‘‘Alzheimer’s Disease,’’ in the 1910 edition of his text book Clinical Psychiatry. This publication is acknowledged as the origin of the term. Alzheimer himself never referred to it as ‘‘Alzheimer’s Disease,’’ though he had later publications on the disease. Alzheimer decided to resign his post as chief physician in order to devote more time to research, specifically traveling to study epilepsy. Although he was no longer employed by Kraepelin, Alzheimer undertook the responsibilities of coeditor of Kraepelin’s Journal of Complete Neurology and Psychiatry. Recognition for Alzheimer and the disease carrying his name began to spread. In 1912, the Silesian FriedrichWilhelm University in Breslau asked him to join their faculty as a full professor of psychiatry. During the move to Breslau, Alzheimer fell ill, but nevertheless assumed his duties with vivacity. His patients and coworkers, including Georg Stertz, Ottfried Fo¨rster, and Ludwig Mann, took notice of his kind, yet authoritative presence. In 1913, his health forced him to visit a private clinic.

Though he returned to work, his health had not improved. This did not impede his ability to make significant contributions to science: in 1913 he found the syphilis pathogen in the central nervous system of a patient with general paresis. After a long illness, Alois Alzheimer died on December 19, 1915 from a heart condition and kidney failure. Though no one immediately took over his pursuit of an understanding of Alzheimer’s disease, people recommenced research on Alzheimer’s disease cases in the 1950s. Studies of the disease began in earnest after Martin Roth’s assertion in the 1960s that Alzheimer’s disease was the most common cause of senile dementia. In the 1970s, Robert Katzman further propelled the surge of interest in Alzheimer’s disease by stating that it was one of the most widespread diseases. Since then, the amount of research on Alzheimer’s disease has increased exponentially, resulting in multiple foundations and centers devoted solely to the disease that Alois Alzheimer’s colleagues considered trivial.

Cross References ▶ Alzheimer’s Dementia ▶ Alzheimer’s Disease ▶ Paresis

References and Readings Engstrom, E. (2007). Researching dementia in imperial Germany: Alois Alzheimer and the economies of psychiatric practice. Culture, Medicine, and Psychiatry, 31, 405–413. Graeber, M., Ko¨sel, S., Egensperger, R., Banati, R., Mu¨ller, U., Bise, K., et al. (1997). Rediscovery of the case described by Alois Alzheimer in 1911: Historical and molecular genetic analysis. Neurogenetics, 1, 73, 80. Lage, J. (2006). 100 years of Alzheimer’s disease (1906–2006). Journal of Alzheimer’s Disease, 9, 15–26. Maurer, K., & Maurer, U. (1998). Alzheimer: The life of a physician and the career of a disease. New York: Columbia University Press. Morris, R., & Salmon, D. (2007). The centennial of Alzheimer’s disease ¨ ber eine eigenartige Erkankung der and the publication of ‘‘U Hirnrinde’’ by Aloïs Alzheimer. Cortex, 43, 821–825. Small, D., & Cappai, R. (2006). Alois Alzheimer and Alzheimer’s disease: A centennial perspective. Journal of Neurochemistry, 99, 708–710. Snyder, P., & Pearn, A. (2007). Historical note on Darwin’s consideration of early-onset dementia in older persons, thirty-six years before Alzheimer’s initial case report. Alzheimer’s and Dementia, 3, 137–142. Zilka, N., & Novak, M. (2006). The tangled story of Alois Alzheimer. Bratisl Lek Listy, 107, 343–345.

Alzheimer’s Dementia

Alzheimer’s Dementia J OA NN T. T SCHANZ , A ARON A NDERSEN Utah State University Logan, UT, USA

Synonyms Alzheimer’s disease; Early-onset Alzheimer’s disease; Familial Alzheimer’s disease; Senile dementia of the Alzheimer’s type

Short Description or Definition One of the leading causes of dementia in late-life, Alzheimer’s disease (AD), is a progressive neurodegenerative disorder characterized by a gradual onset and progressive course, affecting memory and other cognitive domains. For a diagnosis, the cognitive impairments of AD must not occur exclusively in the context of a delirium, and must be of sufficient severity to cause impairment in social or occupational functioning. Diagnoses of AD (Possible or Probable AD) are based on the history and presentation of clinical symptoms, evidence of cognitive impairment, and the exclusion of other causes of dementia such as stroke, metabolic disorders, or other conditions that may account for the cognitive impairment. A diagnosis of Definite AD is based upon postmortem neuropathological analysis and is made when there are sufficient numbers of senile plaques and neurofibrillary tangles in specific brain regions.

Categorization AD may be categorized according to age of onset, family history, or presenting clinical features. Age categories distinguish between senile and pre-senile onset (onset before age 65). Classifications based on family history (familial AD vs. sporadic AD) distinguish AD forms that show high heritability. Familial AD is rare, generally of pre-senile onset, and has been associated with mutations in the APP gene on chromosome 21, Presenilin 1 gene on chromosome 14, and Presenilin 2 gene on chromosome 1 (Hardy, 2003). Its transmission resembles an autosomal dominant pattern (Morris & Nagy, 2004).

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AD has also been classified according to the clinical presentation of symptoms. Its most common presentation involves early and significant memory impairment. Variants to this presentation have been reported in the literature, and they include a visual (posterior) form with significant impairment in higher-level processing of visual stimuli, an aphasic form with significant language involvement, and a frontal form with prominent impairment of executive functions. At autopsy, these variants usually exhibit AD neuropathology in brain regions typically involved in the specific neuropsychological domain (Grabowski & Damasio, 2004).

Epidemiology Prevalence and Incidence. AD is the most common cause of dementia in late-life, accounting for 50–70% of all cases (Malaspina Corcoran, Schobel, & Hamilton, 2008). Current estimates suggest that 4.5 million individuals suffer from AD in the US, and projections based on population trends suggest an increase to 13.2 million by 2050 (U.S. Department of Health and Human Services, 2006). The overall prevalence of AD is about 5–6% in individuals aged 65 years or older in North America, and doubles approximately every 5 years after the age of 60. Estimates suggest a prevalence of 1% at age 60, 16% between ages 80 to 85, and 26 to 45% for those above age 85. Incidence rates also exhibit an age-related increase. Studies report differing patterns of AD prevalence and incidence at the upper end of the lifespan, with some reporting a plateau at very old ages (age 90 or 100; Mendez & Cummings, 2003). Risk Factors. Increasing age is among the strongest risk factor for AD. Other risk factors include the ε4 allele of the Apolipoprotein E (APOE) gene, positive family history (also in sporadic AD), low education (possibly due to less neural reserve), female gender (even after accounting for differential survival), and history of head trauma and vascular factors such as high cholesterol and high blood pressure. Some risk factors occurring earlier in the lifespan affect AD risk. Studies suggest that high blood pressure or high serum cholesterol in midlife increases the risk of AD later in life. Although inconsistent, some studies report that treatment with antihypertensive medications or cholesterol lowing agents reduces the risk for AD (Soininen, Kivipelto, Laakso, & Hiltunen, 2003). Recent studies have also examined the role of insulin resistance and diabetes in AD risk. Among potential ‘‘protective’’ factors, data from epidemiological studies suggest a lower

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risk of AD among women receiving hormone replacement therapy. However, a large randomized clinical trial of estrogen and estrogen + progesterone in elderly women suggested an increase in all-cause dementia in those receiving the combination hormone treatment. Thus, hormone therapy is not recommended for cognitive health (Malaspina et al., 2008). Other factors under active investigation are diet, nutrients and nutrient supplements such as antioxidant vitamins, omega 3 fatty acid, medications such as non-steroidal anti-inflammatory agents, and lifestyle practices such as physical activity and cognitive and social engagement.

symptom onset range from 2 to 20 years. The mean survival has been reported as approximately 10 years, but some studies have reported considerably shorter duration of 3 years. More rapid rate of disease progression has been associated with early, prominent language impairment, frontal features, and extrapyramidal signs (Mendez & Cummings, 2003).

Neuropsychology and Psychology of Alzheimer’s Dementia Neuropsychological Deficits

Natural History, Prognostic Factors, Outcomes The clinical course of AD is usually one of a gradual onset of symptoms with progressive decline. Many scientists believe the disease process starts in the brain decades before overt symptoms emerge. A preclinical phase, characterized primarily by episodic memory deficits, heralds the onset of symptoms. This stage, also referred to as mild cognitive impairment (MCI), lasts approximately 1–3 years. Progression to dementia is characterized by increasing severity of cognitive impairment with severe memory deficits, visuospatial impairment, and other perceptual disturbances. Language impairment begins with mild naming difficulties and circumlocutory speech, but progresses to include comprehension deficits. Apraxia (difficulty performing learned motor tasks in the absence of impairment in primary motor or sensory functions) and impaired executive functions and computational ability are also apparent. Behavioral changes are common with indifference, irritability, and sadness, progressing to delusions and, in some individuals, more severe psychiatric disturbances such as hallucinations and agitation. In end stages, there is severe deterioration of all cognitive functions, speech is generally unintelligible, and motor rigidity and urinary and fecal incontinence are present. Death may occur as the result of other causes such as pneumonia or infections (Mendez & Cummings, 2003). On postmortem exam, the brain is characterized by generalized atrophy and sulcal and ventricular enlargement. Figure 1a displays gross atrophy of an AD brain compared with a brain from a cognitively normal elderly individual. Figure 2 displays a coronal section of an AD brain at the level of the hippocampus. The duration of the entire disease course from MCI to death is highly variable. Survival estimates from

The neuropsychology of AD follows the clinical progression. In early stages, memory is almost always involved, with specific deficits in learning new information. Remote memory such as memory for autobiographical or other knowledge-based systems (semantic memory) is relatively unaffected. In early stages, standardized testing with word lists may reveal relative preservation of immediate or working memory, but impairment in delayed recall. There is usually some benefit from cuing or recognition procedures. With progression, cuing is no longer helpful, and remote recall is affected. Implicit memory may be relatively spared as patients show evidence of learning on priming and procedural motor tasks. Orientation to time and place is also affected in AD (Knopman & Selnes, 2003). Language impairments progress from mild anomia and word finding difficulties in early stages, to include impairment in comprehension and writing. Errors in speech (paraphasias) become more common, and word substitutions become progressively less related to the target words. Repetition of speech may be relatively unaffected until late in the disease course (Knopman & Selnes, 2003; Mendez & Cummings, 2003). Tests of verbal fluency and confrontation naming are especially sensitive to early changes in language. Visuospatial disturbances may be subtle or nonexistent in the earliest stages of AD. In moderate and severe stages, impairment may be evident on figure copying tasks or judgment of line orientation (Knopman & Selnes, 2003). Figure 3 displays characteristic examples of visuoconstructional impairment in four representative patients with AD. Impaired abstract reasoning, sustained attention, planning, judgment, and problem solving may characterize impairment in executive functions. Deficits in executive functions may be demonstrated on tests of verbal fluency, trailmaking, and set shifting. Tests such as the Rey Complex


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b Alzheimer’s Dementia. Figure 1 (a) and (b) display the brains from a cognitively normal elderly individual and an individual who suffered from advanced AD, respectively. Note the severe atrophy apparent in the AD brain (Photo courtesy of Christine Hulette, M.D., Bryan Alzheimer Disease Research Center, Duke University. Reproduced with permission from Elsevier Limited)

figure and clock drawing may also elicit impairment in executive functions with poor planning and execution of the tasks. Deficits in working memory may be evident on tasks requiring mental manipulation or divided attention (Knopman & Selnes, 2003). Other neurocognitive aspects of AD include apraxia and anosognosia. In mild AD, deficits in praxis are not common but emerge later in the disease course. Assessment of

apraxia may involve pantomiming the execution of a task. Anosognosia or an unawareness of disability is quite common (Knopman & Selnes, 2003). Standardized assessment approaches are few. Some approaches rely on clinical observation, noting a discrepancy between self-report of cognitive impairment and test performance, or a discrepancy between caregiver and patient report of impairment.

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Alzheimer’s Dementia. Figure 2 Display of the atrophy in AD in this coronal section including the hippocampi. Note the dilated lateral ventricles and loss of inferior temporal mass (Photo courtesy of Steven S. Chin, M.D., Ph.D., University of Utah Health Sciences Center)

Alzheimer’s Dementia. Figure 3 Display of the visuoconstructional impairments in the drawings of four individuals with Possible or Probable AD. The stimulus is the left-most figure

Behavioral Symptoms Behavioral changes are extremely common in AD, with nearly all individuals exhibiting at least one symptom at some point over the disease course. Among the most common of these changes is apathy, characterized by a lack of interest and indifference. Anxiety, irritability, and depression are also common, as are delusions. Some patients may exhibit hallucinations, and particularly challenging for caregivers and family are disruptive behaviors such as agitation and aggression. The course of behavioral symptoms is variable, with severe episodes alternating with milder ones, raising questions about environmental triggers. Noting the co-occurrence of one or more behavioral disturbances, some scientists believe these symptoms are better conceptualized as behavioral syndromes, with implications for underlying brain pathology. Several questionnaires are available for assessing behavioral symptoms, ranging from a single symptom questionnaire to larger inventories of multiple symptoms.

Assessment of behavioral symptoms is particularly important in an AD evaluation as their presence may suggest other causes of dementia.

Evaluation A through clinical work-up is important for diagnosing AD or determining the etiology of dementia. Critical elements of an evaluation include a detailed clinical history and mental status and physical exams. Due to inaccurate reporting by patients, interview with a reliable informant is necessary. Laboratory, neuroimaging, and neuropsychological testing are important to exclude other causes of dementia. Laboratory testing may include a blood count, routine chemistries, thyroid function, and B12 levels. Neuroimaging with MRI or CT may reveal generalized cerebral atrophy with associated sulcal widening and ventricular enlargement. In early stages of the disorder, the brain may appear normal on MRI/CT. PET


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ICMRGIc (normalized to Pons) 0.0 0.0

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1.0 2.0 3.0 Z-score

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Alzheimer’s Dementia. Figure. 4. Seventy-four year old control subject with normal cognition. The top row shows normal brain metabolic activity and the bottom row shows very few regions of hypometabolism. The areas of significant hypometabolism indicated in the medial views are due to this individual having enlarged lateral ventricles relative to normative subjects. Figures 4–6 These images are processed FDG-PET images obtained from elderly subjects. The images have been processed using Neurostat sterotactic surface projections to illustrate the changes of the brain in Alzheimer’s disease. Subject scans are shown in two rows in each figure, depicting projections onto six surfaces: R-lateral, L-lateral, R-medial, L-medial, Superior and Inferior. The top row in each figure displays regional glucose metabolism with ‘‘cooler’’ colors (purple, blue) reflecting areas of hypometabolism. The bottom row in each figure displays relative glucose metabolism for each participant as compared with a normative sample of 27 cognitively normal elderly individuals. In this bottom series, the images display the statistical significance, expressed as Z-scores, of the hypometabolism when compared to those of the normative sample. The brighter colors (red, white) represent areas of significant hypometabolism and the cooler colors of blues and purples represent relatively normal brain metabolism (All photographs courtesy of Norman L. Foster, M.D. and Angela Y. Wang, Ph.D., Center for Alzheimer’s Care, Imaging and Research, University of Utah)

Alzheimer’s Dementia. Figure. 5. Sixty year old subject clinically diagnosed with MCI. The top row shows symmetric decreases in metabolic activity in both hemispheres of the brain. Abnormalities are primarily in the parietal lobe (shown in the R-lateral and L-lateral views) and the posterior cingulate cortex (shown in the R-medial and L-medial views), as seen in the green regions. The bottom row confirms that these regions (green, yellow and red areas) are indeed significantly (Z-scores 2.5) hypometabolic. This pattern is a distinguishing feature of AD seen in FDG-PET studies (All photographs courtesy of Norman L. Foster, M.D. and Angela Y. Wang, Ph.D., Center for Alzheimer’s Care, Imaging and Research, University of Utah)

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Alzheimer’s Dementia. Figure. 6. Seventy-two year old subject clinically diagnosed with AD. This subject shows an even greater and more widely distributed decrease in glucose metabolism. Parietal and temporal lobes and posterior cingulate cortex (green and blue region in the top row) are affected. The statistically significant changes in metabolic pattern (red and white regions in the lower row) are much greater than the MCI case (All photographs courtesy of Norman L. Foster, M.D. and Angela Y. Wang, Ph. D., Center for Alzheimer’s Care, Imaging and Research, University of Utah)

imaging is a more sensitive technique for detecting changes in brain function in early stages. Reduced glucose metabolism, usually in the temporo–parietal and posterior cingulate regions, is a consistent pattern in early AD. Figures 4 through 6 display the pattern of glucose hypometabolism in MCI and AD compared with a cognitively normal elderly individual. Neuropsychological testing is important to establish the degree of cognitive impairment and to identify patterns that may be suggestive of specific dementing illnesses. Additional tests such as sampling cerebrospinal fluid for tau and amyloid-B42 assays may be helpful as supplemental procedures in complex cases (Mendez & Cummings, 2003).

Treatment Treatment for AD is palliative, with medications and therapies providing symptom management. Medications most commonly used are cholinesterase inhibitors that functionally address the cholinergic deficit of AD by blocking the activity of the acetylcholine degrading enzyme, acetylcholinesterase. These medications are modestly effective, and patients and families may observe an improvement in some cognitive and behavioral symptoms. However, the medications do not modify the trajectory of disease progression. In general, cholinesterase inhibitors are welltolerated. The use of the first FDA-approved drug of this class, tacrine, however, is rarely administered now because of risk of liver toxicity. Other medications include donepezil, rivastigmine, and galantamine. Side effects include gastrointestinal symptoms such as diarrhea, nausea, and

vomiting (Orgogozo, 2003). Memantine, an NMDA glutamate receptor blocker, has been approved for use in moderate and severe AD. This drug is believed to be effective by reducing neuronal excitotoxicity. Other treatments include the use of psychotropic medications (such as antidepressant and antipsychotic medications) to address the behavioral or neuropsychiatric symptoms. Cognitive rehabilitation may be attempted early in the disease course while patients are still able to participate. Psychoeducation, behavioral techniques, music therapy, and caregiver support and interventions are also important elements of clinical care.

Cross References ▶ Alois Alzheimer ▶ Aricept (Donepezil) ▶ Cholinesterase Inhibitors ▶ Dementia ▶ Neurofibrillary Tangles ▶ Senile Dementia ▶ Senile Plaques

References and Readings Grabowski, T. J., & Damasio, A. R. (2004). Definition, clinical features and neuroanatomical basis of dementia. In M. M. Esiri, V. M.-Y. Lee, & J. Q. Trojanowski (Eds.), The neuropathology of dementia (2nd ed., pp. 1–33). Cambridge, UK: Cambridge University Press. Hardy, J. (2003). The genetics of Alzheimer’s disease. In K. Iqbal & B. Winblad (Eds.), Alzheimer’s disease and related disorders: research

Alzheimer’s Disease advances (pp. 151–153). Bucharest, Romania: Ana Asian International Academy of Aging. Knopman, D., & Selnes, O. (2003). Neuropsychology of dementia. In K. M. Heilman & E. Valenstein’s (Eds.), Clinical neuropsychology (4th ed., pp. 574–616). New York: Oxford University Press. Malaspina, D., Corcoran, C., Schobel, S., & Hamilton, S. P. (2008). Epidemiological and genetic aspects of neuropsychiatric disorders. In S. C. Yudofsky & R. E. Hales’ (Eds.), Neuropsychiatry and behavioral neurosciences (5th ed., pp. 301–362). Washington, DC: American Psychiatric Association Press. Mendez, M. F., & Cummings, J. L. (2003). Dementia a clinical approach (3rd ed.). Philadelphia: Butterworth. Morris, J. H., & Nagy, Z. (2004). Alzheimer’s disease. In M. M. Esiri, V. M.-Y. Lee, & J. Q. Trojanowski (Eds.), The neuropathology of dementia (2nd ed., pp. 161–206). Cambridge, UK: Cambridge University Press. Orgogozo, J.-M. (2003). Treatment of Alzheimer’s disease with cholinesterase inhibitors. An update on currently used drugs. In K. Iqbal & B. Winblad (Eds.), Alzheimer’s disease and related disorders: Research advances (pp. 663–675). Bucharest, Romania: Ana Asian International Academy of Aging. Soininen, H., Kivipelto, M., Laakso, M., & Hiltunen, M. (2003). Genetics, molecular epidemiology and cardiovascular risk factors of Alzheimer’s disease. In K. Iqbal & B. Winblad (Eds.), Alzheimer’s disease and related disorders: Research advances (pp. 53–62). Bucharest, Romania: Ana Asian International Academy of Aging. U.S. Department of Health and Human Services. (2006). Journey to discovery. 2005–2006 Progress report on Alzheimer’s disease. Washington, DC: U.S. Department of Health and Human Services.

Alzheimer’s Disease RUSSELL H. S WERDLOW, H EATHER A NDERSON J EFFREY M. B URNS University of Kansas School of Medicine Kansas City, KS, USA

Definition A neurodegenerative disease of the brain characterized clinically by insidious, chronic, and progressive cognitive decline, and histologically by cerebral accumulations of the proteins beta amyloid (plaques) and tau (tangles).

Historical Background In 1902, a woman called Auguste D. came under the care of Dr. Alois Alzheimer, then at the University of Frankfurt. The patient manifested changes in behavior and cognition. Her clinical course was characterized by

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progressive paranoia, delusional thinking, disorientation, and poor memory. She was institutionalized for the last 3 years of her life. Upon her death, Alzheimer analyzed her brain using a silver stain, and described both extracellular and intracellular protein accumulations. The extracellular protein accumulations were termed plaques and the intraneuronal protein accumulations were called tangles. Alzheimer presented the results of this autopsy in 1906. Several other similar cases of relatively ‘‘presenile’’ (i.e., arbitrarily defined as an onset prior to age 55–65) clinical dementia associated with plaques and tangles were noted by Alzheimer and others over the next 4 years. In 1910, Alzheimer’s departmental chair, Emil Kraepelin, published a textbook covering the fields of neurology and psychiatry, and referred to patients with presenile dementia, plaques, and tangles as having ‘‘Alzheimer’s disease.’’ Concurrently, other investigators, such as Oscar Fischer, also reported plaque presence in elderly demented individuals. These individuals were older than those with ‘‘presenile’’ dementia (i.e., generally older than age 55–65). As the commonality of progressive dementia in the elderly was well recognized, the presence of plaques in elderly demented individuals was felt to represent a normal phenomenon. Such individuals were not diagnosed with Alzheimer’s disease. Instead, cognitive decline in elderly adults was attributed to normal aging or other poorly described conditions, such as ‘‘hardening of the arteries.’’ As a result, Alzheimer’s disease remained relatively uncommon for a number of subsequent decades. In the 1960s, investigators began comparing elderly demented subjects to those diagnosed with ‘‘presenile’’ Alzheimer’s disease. Notable similarities were observed regarding the clinical course (chronic and progressive), the clinical features (cognitive decline that featured evolution of an amnestic state, followed by behavioral changes), and histopathology (plaques and tangles). By the 1970s, the number of demented elderly was growing fast as demographic shifts in the aging population combined with increased recognition of the syndrome. At this point, the original definition of Alzheimer’s disease (as described by Alzheimer and named by Kraepelin) was expanded to account for all dementing individuals with plaques and tangles, although some separation of these groups was envisioned. Those meeting the original criteria of plaque and tangle dementia in presenile adults were designated as having dementia of the Alzheimer type (DAT), while the previously unconsidered elderly cases were designated as having senile dementia of the Alzheimer type (SDAT). With increasing recognition of the problem, Alzheimer’s disease very quickly became

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incredibly common, as well as a Western civilization health priority. In the USA, the 1980s saw the establishment of federally funded Alzheimer’s disease research centers, which began to systematically study the clinical course of this progressive dementia, mostly in the common SDAT form. Academic research began to unravel the chemical makeup of plaques and tangles. Investigations into patterns and causes of neurodegeneration were performed. This advancing knowledge enhanced the ability of clinicians to diagnose Alzheimer’s disease at increasingly subtle stages, as well as the ability to pharmacologically intervene to achieve partial, temporary symptomatic benefit in at least some individuals.

Current Knowledge Scientific Perspective The plaques seen in persons with Alzheimer’s disease contain several aggregated proteins. The major constituent is a protein called amyloid beta (Ab). ‘‘Beta’’ is a chemical term that specifies a certain pattern of protein folding. ‘‘Amyloid’’ is a general term that refers to proteins that give a particular appearance when exposed to a particular type of stain, Congo red. The beta amyloid, or Ab, found in the brains of Alzheimer’s disease patients derives from a particular protein called the amyloid precursor protein (APP). In the human brain, the APP is 695 amino acids long. It is a transmembrane protein. One end (the carboxy end) is found inside neurons, in the cytoplasm. The other end (the amino end) extends outside the cell. In between the cytoplasmic and extracellular portions is a stretch that runs through the membrane. The normal function of APP is not well known. APP is digested by different enzymes, which cut the protein at different points. An enzyme complex called the beta secretase (BACE) cuts APP in its extracellular portion. An enzyme or group of enzymes referred to as the alpha secretase cuts APP in its intramembrane segment. The gamma secretase cuts APP twice, both times in its intramembrane segment. Both of the gamma secretase cuts occur closer to the carboxy end of the APP than the alpha secretase cut. Different cutting combinations generate various APP by-products. Cutting of an APP by beta and gamma secretases generates a 38–43 amino acid stretch, and this stretch tends to assume a beta folding conformation and has the features of an amyloid protein (i.e., birefringence under the microscope when stained with Congo red). The

40 and 42 amino acid-long variants of Ab predominate in plaques, and are often designated Ab40 and Ab42. Ab42 seems to be particularly important to the formation of the amyloid plaques of Alzheimer’s disease, probably because this version of the protein is quite insoluble. When Ab accumulations begin to form in brain, they are not associated with disrupted cell elements and are called ‘‘diffuse plaques.’’ Another type of more evolved plaque can also be found in Alzheimer’s disease patients, in which Ab becomes condensed at the center of the plaque, and the vicinity of the plaque is associated with disrupted cell elements such as degenerating axons and dendrites. As axons and dendrites are collectively called ‘‘neurites,’’ this type of plaque is called a ‘‘neuritic plaque.’’ The tangles of Alzheimer’s disease are found primarily in neurons. Under the microscope tangles have a fibrous quality to them, and hence tangles in Alzheimer’s disease are referred to as ‘‘neurofibrillary tangles.’’ Neurofibrillary tangles consist of a protein called tau. Normally, tau is found in association with microtubules, which act as a skeleton, or ‘‘cytoskeleton’’ supporting the cellular structure. The function of tau appears to be the stabilization of these microtubules. Like many proteins, after its production tau is modified by the addition and subtraction of phosphate groups on certain amino acids, especially serine and threonine. During embryonic development, tau is heavily phosphorylated, but during youth and early adulthood this heavily phosphorylated pattern is rare if at all seen. In Alzheimer’s disease, though, tau again takes on a heavily phosphorylated pattern, which is felt to reflect an abnormal physiologic event and is referred to as tau ‘‘hyperphosphorylation.’’ Hyperphosphorylated tau molecules begin to pair off, a process called ‘‘dimerization.’’ Hyperphosphorylated tau dimers, also called ‘‘paired helical filaments,’’ are quite insoluble and begin to aggregate with each other. This aggregation, typically visible extending from cell bodies into axons, comprises the neurofibrillary tangle. As impressive as this advancing understanding of plaque and tangle composition is, recognizing what constitutes these aggregations does not address why they form. In this regard, genetic studies of DAT subjects who inherit the disorder in an autosomal dominant fashion have had a large impact. Several hundred such families have been documented. In these families the disease affects about 50% of each generation, with typical onset occurring in the 3rd, 4th, 5th, or 6th decades. A small number of these families have demonstrable mutations in the gene that encodes the APP. This gene is located on chromosome 21, the same chromosome that is present in excess in Down’s syndrome. Down’s syndrome patients


invariably accumulate Ab plaques in their 5th decade. A somewhat larger number of these families have mutations in the gene that encodes a protein called presenilin 1. This gene is found in chromosome 14. Presenilin 1 protein constitutes part of the gamma secretase complex. A smaller number of families have mutation of a related gene on chromosome 1, which encodes a related protein, presenilin 2. Presenilin 2 can also participate in formation of the gamma secretase. Mutations in the genes that encode APP, presenilin 1, and presenilin 2 all enhance the production of Ab42. This has lent support to the ‘‘amyloid cascade hypothesis,’’ which posits as Ab42 is generated it begins to interfere with neuronal function, kill neurons, and generate the other histologic features seen in Alzheimer’s disease. While the logic underlying this hypothesis is obvious, it is important to keep in mind it assumes the very small subset of early-onset, autosomal dominant Alzheimer’s disease (which accounts for far less than 1% of those affected) have a similar if not identical etiology to the common sporadic, late-onset cases that constitute the vast majority. In those subjects, what initiates Ab42 production remains an open area of debate. Conceivably, population diversity in genes that contribute to APP production or processing could cause Ab42 to appear. Environmental factors could lead to Ab42 formation. Also, a variety of age-related factors promote Ab42 formation. Other factors are recognized to play a role in Alzheimer’s disease, and where these factors fit into or what they tell us about the etiologic hierarchy of the disease is unclear. One factor relates to the APOE gene on chromosome 19. The APOE gene shows population variability due to the presence of two polymorphic positions. The common APOE variants are the e2, e3, and e4 forms. The APOE e4 form is over represented in those with Alzheimer’s disease, where it seems to move up the age of presentation in those destined to develop the disorder. Mitochondrial function is also altered in Alzheimer’s disease, and these alterations are not limited to the brain.

Diagnostic Perspective Dementia is defined as cognitive decline that has advanced to that point it interferes with activities of daily living. While dementia has many different etiologies, Alzheimer’s disease is the most common cause of dementia, accounting for 50–60% of dementia verified by neuropathological examination of the brain at autopsy. The clinical diagnosis (i.e., diagnosis in life) of Alzheimer’s disease is made in patients who have progressive dementia

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with no other systemic or brain diseases that could account for the progressive cognitive decline. A diagnosis of ‘‘definite Alzheimer’s disease’’ can only be diagnosed at autopsy by the presence of plaques and tangles (although in some schemas tangles are not requisite) in an individual with a clinical history suggestive of dementia. The presence of plaques and tangles in typical brain regions (mesial temporal, parietal, and inferior frontal structures) is quite common in elderly persons with the clinical syndrome of Alzheimer’s disease. As a result of the high prevalence of Alzheimer’s disease with advancing age (at least one commonly quoted study estimates approximately half of those over the age of 85 have it), the specificity of the clinical diagnosis is high. Recognition of how common Alzheimer’s disease is in later life has also served to enhance clinician awareness, thus improving sensitivity of the diagnosis. In the hands of an experienced physician, clinical diagnostic accuracy is excellent. Criteria originally designed to facilitate identification of subjects for clinical trials have helped to standardize clinical diagnostic approaches. These criteria, such as those proposed by the National Institute of Neurologic, Communicative Disorders, and Stroke (NINCDS) and the Alzheimer’s Disease and Related Disorders Association (ADRDA) in the 1980s emphasize the importance of establishing that a progressive dementia exists in a patient. Two basic approaches are commonly used toward this end. One is to demonstrate a pattern of cognitive domain strengths and weaknesses that reliably suggest decline from a previous level of cognitive function has emerged. For example, defective memory retention in the presence of another defective cognitive domain (language, executive function, visuospatial function, and praxis) in an elderly patient with cognitive complaints and an otherwise unremarkable physical exam is strongly suggestive of Alzheimer’s disease. The other approach focuses more on defining the degree and nature of emerging declines in daily living activities. This latter technique focuses extensively on collateral history obtained from family members or friends of the patient. The diagnosis is made primarily through clinical impression, although that impression is influenced by a small set of recommended laboratory and imaging tests. These tests are serologic (vitamin B12 level, thyroid function tests, electrolytes with renal and hepatic indices, and a blood cell count) and structural (brain imaging by either computed tomography or magnetic resonance imaging) in nature. As currently used, they mostly serve to rule out the presence of concomitant pathologies that can interfere with cognition. Although this has contributed to the view that the Alzheimer’s disease diagnosis is one of exclusion,

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it should be noted that certain patterns of cognitive decline elicited by clinical history or demonstrable by neuropsychological testing are so typical of Alzheimer’s disease they can be used to support a diagnosis of inclusion. It is important to note, though, that at the time of this writing PET and APOE genotyping are not commonly used in the diagnosis of Alzheimer’s disease and cannot by themselves establish a diagnosis of Alzheimer’s disease.

Treatment Perspective Although Alzheimer’s disease is currently neither reversible nor curable, it is possible to treat its symptoms. The first approved treatment for Alzheimer’s disease was tacrine, a cholinesterase inhibitor. This drug increased levels of brain acetylcholine by antagonizing its synaptic degradation. Increasing brain cholinergic tone was identified as a pharmacologic target because Alzheimer’s disease patients show a profound loss of acetylcholine due to degeneration of cholinergic neurons in the basal forebrain. Safer cholinesterase inhibitors (donepezil, rivastigmine, and galantamine) have since superseded tacrine. In addition to inhibiting acetylcholinesterase, rivastigmine also inhibits buytrylcholinesterases that also hydrolyze acetylcholine, and galantamine is an allosteric modulator of acetylcholine nicotinic receptors. Each agent shows a similar overall degree of efficacy, although the individual with Alzheimer’s disease may respond to or tolerate one drug better than the other. Treatment cohorts followed for 12 weeks to 3 years indicate that as a group, those started on cholinesterase inhibitors tend to perform and appear slightly improved compared to their immediate pretreatment baseline. This improvement appears detectable for 6–12 months. By 12 months, though, treatment groups return to their pretreatment performance as ascertained by cognitive testing, clinical impression, and caregiver impression. Beyond 12 months, patients continuously decline below their pretreatment baseline, although for at least the next several years patients appear to perform better on cognitive testing than would otherwise be expected. The clinical meaningfulness of this sustained benefit has fueled considerable debate. Benefits have been observed on measures of cognitive ability, functional ability, behavior, and caregiver stress. At the time of this writing, memantine is the only non-cholinesterase inhibitor specifically approved for the treatment of Alzheimer’s disease. Under in vitro conditions, memantine blocks a cation channel associated with the NMDA type of glutamate-activated ionotropic receptors. Whether or not this is its primary mechanism

of action in Alzheimer’s disease has been questioned. In any case, cohorts of patients with moderate or severe Alzheimer’s disease, when randomized to memantine, perform better on measures of cognitive and functional performance than do concurrent placebo treatment groups. In severe Alzheimer’s disease, the magnitude of observed benefit is similar to that obtained with donepezil. Memantine and donepezil have been studied in combination with each other. Subjects with mini-mental state exam scores of 5–14, who were already on donepezil, did better as a group when memantine was added to their treatment regimen than when placebo was added. Demonstrable benefits in mild Alzheimer’s disease are lacking and thus the role of memantine in the mild stages of Alzheimer’s disease is not clear. A single study concluded high-dose vitamin E (2000 iu each day) might slightly slow decline in Alzheimer’s disease patients. More recent general evidence, though, suggests taking more than 400 iu of vitamin E on a daily basis increases overall mortality. The marginality of any vitamin E benefit, in conjunction with safety concerns, has reduced enthusiasm for the use of vitamin E in Alzheimer’s disease. Although a variety of other prescription medications (estrogens, statins), nonprescription medications (nonsteroidal anti-inflammatories), and nutraceuticals (gingko biloba) have been considered for the treatment of Alzheimer’s disease, published data to date on all other treatment options has been at worst negative and at best insufficient to earn regulatory approval. Other drug categories are commonly used to treat targeted symptoms associated with Alzheimer’s disease. For instance, antipsychotic medications are often used to treat agitated behavior. Some studies do show efficacy in this regard, although other studies have argued the limited behavioral benefits antipsychotics may confer is canceled out by increased morbidity.

Future Directions Scientific Perspective In the short term, considerable effort will be directed at additional studies of Ab dynamics and homeostasis. Research will focus on the toxicities of different degrees of Ab aggregation (especially oligomers, defined as short, soluble polymers of amyloid), cellular mechanisms of Ab disposal, and tissue-level mechanisms of Ab disposal. Research over the longer term will need to address the fact that the predominant etiologic hypothesis, the amyloid cascade hypothesis, cannot yet explain why Ab


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homeostasis changes in most of those affected or how Ab might give rise to other aspects of Alzheimer’s disease pathology. It is possible the amyloid cascade hypothesis will prove valid in those with early onset, autosomal dominant Alzheimer’s disease caused by mutations of the genes encoding APP, presenilin 1, and presenilin 2 proteins, but not the late-onset cases (the vast majority). Disproving the amyloid cascade hypothesis in the late-onset cases will likely require two events. First, interventions that attempt to treat Alzheimer’s disease by targeting Ab will need to show absent or limited efficacy. Second, other hypotheses better able to explain the overall Alzheimer’s clinical and pathological big picture will need to demonstrate viability and durability.

amyloid plaques and neurofibrillary tangles can be administered intravenously, and the degree of brain ligand retention measured using PET. This approach can provide an estimate of an individual patient’s plaque burden. Development of techniques such as this will increasingly render the diagnosis of Alzheimer’s disease one of inclusion. Even so, this technology may, like others, turn out to serve best as an adjunct to the clinical diagnosis as opposed to the principal determinant of the diagnosis. The reason for this is that a substantial percentage of nondemented individuals have relatively high plaque burdens. The significance of increased plaque burden in nondemented individuals will need to be determined with prospective long-term studies.

Diagnostic Perspective

Treatment Perspective

Because it will likely prove easier in the future to prevent neurodegeneration rather than reverse it, the ability to render an early, accurate diagnosis is crucial. Also, the ability to treat the disease (either symptomatically or disease modifying) increases the importance of early diagnosis. A confluence of neuropsychologic/clinical longitudinal studies performed in conjunction with careful histopathologic correlation has already allowed a syndrome called mild cognitive impairment (MCI) to be defined. MCI is known to represent a precursor of the Alzheimer syndrome in the majority of those diagnosed with it, and in more than half the MCI syndrome simply represents early Alzheimer’s disease. There is an emerging consensus that the line between ‘‘normal’’ age related cognitive decline and clinically excessive cognitive decline, at least on an etiologic level, is a blurry one. Accordingly, by the time MCI is diagnosable in many individuals, substantial irreversible brain change has occurred. Techniques and technologies for pushing the limits of the diagnosis to stages that precede MCI are therefore needed. Most development toward this end focuses on the study of potential ‘‘biomarkers.’’ Biomarkers can be entities detectable in extractable tissues, such as blood or cerebrospinal fluid (CSF). For example, CSF tau levels increase in Alzheimer’s disease, while CSF Ab levels decline. When used in conjunction with fluorodeoxyglucose PET, which shows the brain’s ability to consume glucose, investigators have been able to develop algorithms that predict future cognitive decline in elderly adults with MCI, and even in individuals before they manifest cognitive complaints. Biomarkers can also be demonstrated in vivo. For instance, ligands that bind amyloid plaques or both

None of the treatments approved for use in Alzheimer’s disease are approved for use in MCI, although available data argue cholinesterase inhibition (at least with donepezil) may provide a marginal benefit. Such a benefit would not be surprising, especially if MCI represents very early Alzheimer’s disease in most people. Over a decade of experience with symptomatic treatment has made it abundantly clear that disease-modifying treatments are required. Most current approaches toward disease modification are targeted to Ab homeostasis. Inhibition of its production (gamma secretase inhibitors and modifiers), its targeted removal (active and passive immunization approaches), prevention of its aggregation, and enhancement of enzymatic degradation are all under active pursuit. To date, a phase II Ab vaccination trial (AN1792) was halted when several of the subjects developed encephalitis. Other data obtained through this trial suggest the approach was successful in reducing cerebral amyloid plaques. However, the most extensive published clinical data from AN1792 indicate that one year after vaccination, the rate of cognitive decline was similar to (unchanged from or only very slightly reduced from) the rate of decline shown by the placebo group of that trial. A phase III trial of tramiprosate, which retards Ab aggregation, was negative. A phase III trial of a gamma secretase modifying agent (R-flurbiprofen) is underway. Phase III trials of agents intended to humorally remove Ab are scheduled. If attacking Ab fails to meaningfully benefit Alzheimer’s disease patients, the validity of the amyloid cascade hypothesis in late-onset, sporadic Alzheimer’s disease will be called into question. If this happens, new models for drug design will be needed. Currently, mice expressing a

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mutant APP transgene, sometimes in conjunction with other mutant human transgenes, serve as the gold standard for preclinical testing of potential Alzheimer’s disease treatments.

Cross References ▶ Alzheimer’s Dementia ▶ Memory Impairment ▶ Mental Status Examination ▶ Mini Mental State Exam ▶ Neurobehavioral Cognitive Status Examination ▶ Senile Dementia

References and Readings Amaducci, L. A., Rocca, W. A., & Schoenberg, B. S. (1986). Origin of the distinction between Alzheimer’s disease and senile dementia: how history can clarify nosology. Neurology, 36, 1497–1499. Blacker, D., & Tanzi, R. E. (1998). The genetics of Alzheimer disease: current status and future prospects. Archives of Neurology, 55, 294–296. Blessed, G., Tomlinson, B., & Roth, M. (1968). The association between quantitative measures of dementia and of senile change in the cerebral grey matter of elderly subjects. The British Journal of Psychiatry, 114, 797–811. Campion, D., Dumanchin, C., Hannequin, D., Dubois, B., Belliard, S., Puel, M., et al. (1999). Early-onset autosomal dominant Alzheimer disease: prevalence, genetic heterogeneity, and mutation spectrum. The American Journal of Human Genetics, 65, 664–670. Corder, E. H., Saunders, A. M., Strittmatter, W. J., Schmechel, D. E., Gaskell, P. C., Small, G. W., et al. (1993). Gene dose of apolipoprotein E type 4 allele and the risk of Alzheimer’s disease in late onset families. Science, 261, 921–923. De Leon, M. J., & Klunk, W. (2005). Biomarkers for the early diagnosis of Alzheimer’s disease. Lancet Neurology, 5, 198–199. Evans, D. A., Funkenstein, H. H., Albert, M. S., Scherr, P. A., Cook, N. R., Chown, M. J., et al. (1989). Prevalence of Alzheimer’s disease in a community population of older persons. Higher than previously reported. JAMA, 262, 2551–2556. Gearing, M., Mirra, S. S., Hedreen, J. C., Sumi, S. M., Hansen, L. A., & Heyman, A. (1995). The Consortium to establish a registry for Alzheimer’s disease (CERAD). Part X. Neuropathology confirmation of the clinical diagnosis of Alzheimer’s disease. Neurology, 45, 461–466. Gilman, S., Koller, M., Black, R. S., Jenkins, L., Griffith, S. G., Fox, N. C., et al. (2005). Clinical effects of Abeta immunization (AN1792) in patients with AD in an interrupted trial. Neurology, 64, 1553–1562. Hardy, J., & Allsop, D. (1991). Amyloid deposition as the central event in the aetiology of Alzheimer’s disease. Trends in Pharmacological Sciences, 12, 383–388. Hardy, J. A., & Higgins, G. A.(1992). Alzheimer’s disease: the amyloid cascade hypothesis. Science, 256, 184–185. Katzman, R. (1976). The prevalence and malignancy of Alzheimer’s disease: a major killer. Archives of Neurology, 33, 217–218.

Khachaturian, Z. S. (1985). Diagnosis of Alzheimer’s disease. Archives of Neurology, 42, 1097–1106. Klunk, W. E., Engler, H., Nordberg, A., Wang, Y., Blomqvist, G., Holt, D. P., et al. (2004). Imaging brain amyloid in Alzheimer’s disease with Pittsburgh Compound-B. Annals of Neurology, 55, 306–319. Mayeux, R., Saunders, A. M., Shea, S., Mirra, S., Evans, D., Roses, A. D., et al. (1998). Utility of the apolipoprotein E genotype in the diagnosis of Alzheimer’s disease. Alzheimer’s Disease Centers Consortium on Apolipoprotein E and Alzheimer’s Disease. The New England Journal of Medicine, 338, 506–511. McKhann, G., Drachman, D., Folstein, M., Katzman, R., Price, D., & Stadlan, E. M. (1984). Clinical diagnosis of Alzheimer’s disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer’s Disease. Neurology, 34, 939–944. Morris, J. C. (2006). Mild cognitive impairment is early-stage Alzheimer disease: time to revise diagnostic criteria. Archives of Neurology, 63, 15–16. Mosconi, L., De Santi, S., Li, J., Tsui, W. H., Li, Y., Boppana, M., et al. (2007). Hippocampal hypometabolism predicts cognitive decline from normal aging. Neurobiol Aging. doi:10.1016/j. neurobiolaging.2006.12.008. National Institute on Aging, and Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer’s Disease. (1997). Consensus recommendations for the postmortem diagnosis of Alzheimer’s disease. Neurobiology of Aging, 18(4 Suppl.), S1–2. Petersen, R. C., Smith, G. E., Waring, S. C., Ivnik, R. J., Tangalos, E. G., & Kokmen, E. (1999). Mild cognitive impairment: clinical characterization and outcome. Archives of Neurology, 56, 303–308. Petersen, R. C., Thomas, R. G., Grundman, M, Bennett, D., Doody, R., Ferris, S., et al. (2005) Vitamin E and donepezil for the treatment of mild cognitive impairment. The New England Journal of Medicine, 352, 2379–2388. Ronald and Nancy Reagan Research Institute of the Alzheimer’s Association and the National Institute on AgingWork Group. (1998). Consensus report of the work group on: molecular and biochemical markers of Alzheimer’s disease. Neurobiology of Aging, 19, 109–116. Sano, M., Ernesto, C., Thomas, R. G., Klauber, M. R., Schafer, K., Grundman, M., et al. (1997). A controlled trial of selegiline, alpha-tocopherol, or both as treatment for Alzheimer’s disease. The Alzheimer’s Disease Cooperative Study. The New England Journal of Medicine, 336, 1216–1222. Scheuner, D., Eckman, C., Jensen, M., Song, X., Citron, M., Suzuki, N., et al. (1996). Secreted amyloid beta-protein similar to that in the senile plaques of Alzheimer’s disease is increased in vivo by the presenilin 1 and 2 and APP mutations linked to familial Alzheimer’s disease. Nature Medicine, 2, 864–870. Schneider, L. S., Tariot, P. N., Dagerman, K. S., Davis, S. M., Hsiao, J. K., et al. (2006). Effectiveness of atypical antipsychotic drugs in patients with Alzheimer’s disease. The New England Journal of Medicine, 355, 1525–1538. Snowdon, D. A., Kemper, S. J., Mortimer, J. A., Greiner, L. H., Wekstein, D. R., & Markesbery, W. R. (1996). Linguistic ability in early life and cognitive function and Alzheimer’s disease in late life. Findings from the Nun Study. JAMA, 275, 528–532. Swerdlow, R. H. (2007). Is aging part of Alzheimer’s disease, or is Alzheimer’s disease part of aging? Neurobiology of Aging, 28, 1465–1480.

Alzheimer’s Disease Cooperative Study ADL Scale

Alzheimer’s Disease Cooperative Study ADL Scale J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms Alzheimer’s disease co-operative study ADL scale for mild cognitive impairment (ADCS-ADL-MCI); Alzheimer’s disease co-operative study ADL scale for severe impairment (ADCS-ADL-sev).

Description The ADCS-ADL assesses the competence of patients with Alzheimer’s Disease (AD) in basic and instrumental activities of daily living (ADLs). It can be completed by a caregiver in questionnaire format, or administered by a clinician/researcher as a structured interview with a caregiver. All responses should relate to the 4 weeks prior to the time of rating. The six basic ADL items each take an ADL (e.g., eating) and provide descriptions of level of competence, with the rater selecting the most appropriate option (e.g., ate without physical help and used a knife; used a fork or spoon but not a knife; used fingers to eat; was usually fed by someone else). The 16 instrumental ADL items follow the format ‘‘In the past 4 weeks, did s/he use the telephone,’’ with the response options of yes/no/don’t know. If the response is ‘‘yes,’’ a rating is then made regarding his/her competence according to a set of descriptions tailored to that activity (e.g., for the telephone item, whether the person looked up phone numbers and made calls, made calls only to well-known numbers without referring to a directory, made calls only to well-known numbers using a telephone directory, answered the phone but did not make calls, or only spoke when put on the line). Adapted versions of the scale suitable for people with MCI (ADCSMCI-ADL) and moderate-severe AD (ADCS-ADL-sev) have also been developed. Scores on the 24-item ADCS-ADL range from 0 to 78, those on the 18-item ADCS-MCI-ADL range from 0 to 57, and on the 19-item ADCS-ADL-sev from 0 to 54, where higher scores reflect greater competence (see section ‘‘Psychometric

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Data’’ for further details). The entire instrument takes 15–30 min to administer.

Historical Background The ADCS is a United States-based initiative that aims to conduct research informing the prevention and treatment of AD, as well as developing measures for use in people with AD, particularly in clinical trials. The ADCS-ADL was the first ADL scale to be developed for use specifically in clinical trials with people with AD across the range of severity. The 23 items in the standard version were selected from a pool of 45 items based upon a stringent set of psychometric criteria (see Section ‘‘Psychometric Data’’). Using the same criteria, Galasko et al. (2005) developed a version of the ADCS-ADL for more severely impaired participants, which is known as the ADCS-ADL-sev, and a version for people with MCI has also been developed (ADCS-MCI-ADL, Perneczky et al., 2006). The ADCS-ADL has been used in a variety of clinical trials.

Psychometric Data Galasko et al. (1997) selected the items for the ADCS-ADL from a pool of 45 items thought to be relevant to the target population on the basis of existing scales and clinical experience. To determine which ADLs were most suitable for inclusion, the 45-item version was administered at baseline, 6 months and 12 months later to 64 elderly controls and 242 people with AD, stratified by MMSE score at baseline assessment. Half of participants were additionally assessed at 1 and 2 months postbaseline. An item was included in the final measure if it fit the criteria that it: was performed either premorbidly or at baseline by >90% of participants (showing it was applicable to the target group), had a kappa agreement statistic at 1–2 months of >0.4 (indicating good test-retest reliability), had a significant correlation with MMSE score (indicating appropriate scaling and validity), and showed decline over 12 months in at least 20% of participants (indicating validity and sensitivity to change). Galasko et al. (2005) used the same criteria in the development of the ADCS-ADL-sev, based on longitudinal data of 145 patients with Mini-Mental State Examination (MMSE) scores between 0 and 15. Galasko et al. reported good test-retest reliability (baseline-1 month r = 0.94, baseline-2 months r = 0.89,

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Alzheimer’s Disease Co-operative Study ADL Scale for Mild Cognitive Impairment (ADCS-ADL-MCI)

month1–month2 r = 0.94), and there was evidence of convergent validity based upon the strong correlation between ADCS-ADL-sev and other global impairment measures (ADCS-ADL-sev – MMSE r = 0.64; ADCSADL-sev – Severe Impairment Battery r = 0.71). The mean score on first test was 25.4 (SD 12.7, maximum obtainable 54), with a mean decline of 5.6 points (SD 7.5) over 6 months and 10.3 points (SD 10.3) over 12 months. Perneczky et al. (2006) have found that the ADCSMCI-ADL scale can discriminate people with MCI from control participants (a cut-off score of 52 gives sensitivity of 0.89 and specificity of 0.97).

Clinical Uses The ADCS-ADL and its variants are the only ADL scales designed with AD specifically in mind, and can provide a fairly detailed assessment of competence in a variety of ADLs. Galasko et al. (2005) state that the measure takes too long to administer for it to be widely adopted in clinical practice, but it would be useful in intervention studies, and the ADL-sev in particular where the severity of the disorder may render measures such as the MMSE unsuitable due to floor effects. The careful selection of items for the ADCS-ADL suggests that they are eminently suitable for use in clinical trials. Perneczky et al. (2006) found that even patients with a diagnosis of Mild Cognitive Impairment exhibit deficits in instrumental ADLs on the ADCS-ADL-MCI, and that scores can successfully discriminate patients with MCI from healthy controls; as such, results from this scale may be useful in forming an MCI diagnosis.

Galasko, D., Schmitt, F., Thomas, R., Jin, S., Bennett, D., & Ferris, S. (2005). Detailed assessment of activities of daily living in moderate to severe Alzheimer’s disease. Journal of the International Neuropsychological Society, 11, 446–453. Perneczky, R., Pohl, C., Sorg, C., Hartmann, J., Komossa, K., Alexopoulos, P., et al. (2006). Complex activities of daily living in mild cognitive impairment: Conceptual and diagnostic issues. Age and Ageing, 35, 240–245.

Alzheimer’s Disease Co-operative Study ADL Scale for Mild Cognitive Impairment (ADCSADL-MCI) ▶ Alzheimer’s Disease Cooperative Study ADL Scale

Alzheimer’s Disease Co-operative Study ADL Scale for Severe Impairment (ADCS-ADL-sev) ▶ Alzheimer’s Disease Cooperative Study ADL Scale

Amantadine ▶ Symmetril (Amantadine)

Cross References

Ambidexterity

▶ Bristol Activities of Daily Living Scale ▶ Disability Assessment for Dementia ▶ Lawton–Brody iADL Scale ▶ The Activities of Daily Living Questionnaire

J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA


Definition

Galasko, D., Bennett, D., Sano, M., Ernesto, C., Thomas, R., Grundman, M., et al. (1997). An inventory to assess activities of daily living for clinical trials in Alzheimer’s disease. The Alzheimer’s disease Cooperative Study. Alzheimer’s Disease and Associated Disorders, 11(S2), S33–S39.

Ambidexterity is the tendency for one to be more or less equally proficient in carrying out complex or skilled motor tasks with either the right or the left hand. While complete ambidexterity is relatively rare, mixed

American Academy of Clinical Neuropsychology (AACN)

proficiencies or preferences are not uncommon, with men more frequently demonstrating such mixed preferences than women. Tan (1988) found that approximately 66% of the population was noted to express a strong right-handed preference, while a little more than 3% were predominately left handed. The remaining 30% evidenced mixed hand preferences. As noted elsewhere in this volume, handedness is a common, but not the only measure of what is referred to as ‘‘cerebral dominance.’’ Another of the more frequent indices of dominance is language, which is typically organized primarily in the left hemisphere. While in the majority of non-brain-injured individuals, the control of both complex motor skills and language functions rest within the left hemisphere, this may not always be the case, particularly for those who are either left handed or ambidextrous. It has been shown that while right hemisphere dominance for language is quite rare in right-handers, it could approach 30% in strong left-handers. Individuals who are ambidextrous or whose parents are left handed tend to fall somewhere in between these two groups with regard to the hemispheric localization of language. Furthermore, the localization of language may not be an all-or-none phenomena. While one hemisphere may be more predominant, language functions may be mediated to some extent by both hemispheres. Individuals with mixed or anomalous dominance, including those who were ambidextrous, tend to have a greater incidence of at least some degree of bilateral representation of language. In the event of unilateral strokes, such individuals may evidence less severe residual aphasic deficits when compared to patients with strongly lateralized language when that hemisphere is affected.

Cross References ▶ Anomalous Dominance ▶ Dominance (Cerebral)

References and Readings Benson, D. F., & Geschwind, N. (1985). Aphasia and related disorders: A clinical approach. In M. Mesulam (Ed.), Principles of behavioral neurology (pp. 193–238). Philadelphia: F.A. Davis Co. Knecht, S., Drager, B., Deppe, M., Bode, L., Lohmann, H., Floel, A., et al. (2000). Handedness and hemispheric language dominance in healthy humans. Brain, 123, 2512–2518. Pieniadz, J. M., Naeser, M. A., Koff, E., & Levine, H. L. (1983). CT scan hemispheric asymmetry measurements in stroke cases with global aphasia: Atypical asymmetries associated with improved recovery. Cortex, 19, 371–391.

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Pujol, J., Deus, J., Losilla, J. M., & Capdevila, A. (1999). Cerebral lateralization of language in normal left-handed people studied by functional MRI. Neurology, 52, 1038–1043. ¨ . (1988). The distribution of hand preference in normal men and Tan, U women. International Journal of Neuroscience, 41, 35–55.

Ambiguous Personality Assessment ▶ Projective Technique

American Academy of Clinical Neuropsychology (AACN) R EBECCA M C C ARTNEY Emory University/Rehabilitation Medicine Atlanta, GA, USA

Membership American Academy of Clinical Neuropsychology (AACN) is an organization for psychologists who have achieved board certification in the specialty of Clinical Neuropsychology, under the American Board of Clinical Neuropsychology (ABCN). Membership in the Academy consists of three classes: Active, Senior, and Affiliate. Active members are elected from among psychologists who have been certified in clinical neuropsychology by the ABCN in affiliation with the American Board of Professional Psychology (ABPP). Senior members are elected from among Active members who have been Academy members, for a period of no less than the five preceding years, are age 65 or older or disabled, and are fully retired from the active practice of clinical neuropsychology. They continue to be listed in the membership directory of the academy, and they continue to receive any newsletters distributed to Academy members. Senior members have no financial obligations to the Academy and are allowed to continue to subscribe to any journal available through the Academy. At the time of this publication, there were 367 active senior members in the United States and 20 members in Canada. Affiliate members are elected from among all others who are intellectually interested in the purposes of the Academy and wish to participate in its non-voting activities. All members are provided with a subscription to The Clinical Neuropsychologist, access to the AACN Clinical Discussion Email List, and discounted fees to meetings and workshops.

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American Academy of Neurology (AAN)

Presidents of the Academy include Byron P. Rourke, (1995–1996), Wilfred van Gorp (1996–2002), Catherine A. Mateer (2002–2004), Robert L. Mapou (2004–2006), Jerry J. Sweet (2006–2008).

Major Areas or Mission Statement AACN’s stated mission is to maintain the standards of Clinical Neuropsychology through support of the board certification process of ABCN. The Academy holds the following objectives: (1) Support for the principles, policies, and practices that seek the attainment of the best in clinical neuropsychological patient care. (2) The pursuit of excellence in psychological education, especially as it concerns the clinical neuropsychological sciences. (3) The pursuit of high standards in the practice of clinical neuropsychology and support of the credentialing activities of the ABCN. (4) Support for the quest of scientific knowledge by support for research in neuropsychology and related fields. (5) The communication of scientific and scholarly information through continuing education (CE), scientific meetings, and publications. (6) Provision for communication with other groups and representation for clinical neuropsychological opinion to best achieve and preserve the purposes of the Academy.

Cross References ▶ American Board of Clinical Neuropsychology (ABCN) ▶ International Neuropsychological Society ▶ National Academy of Neuropsychology

References and Readings Boake, C. (2008). Clinical neuropsychology. Professional Psychology: Research and Practice, 39(2), 234–239. Boake, C., & Bieliauskas, L. A. (2007). Development of clinical neuropsychology as a psychological specialty: A timeline of major events. The ABPP Specialist, 26, 42–43. Yeates, K. O., & Bieliauskas, L. A. (2004). The American Board of Clinical Neuropsychology and American Academy of Clinical Neuropsychology: Milestones past and present. The Clinical Neuropsychologist, 18, 489–493.

American Academy of Neurology (AAN) C ATHERINE M. RYDELL American Academy of Neurology Saint Paul, MN, USA

Landmark Contributions

Address (and URL)

AACN was founded in 1996. The first appointed president was Byron Rouke, Ph.D. and the first elected president was Wilfred Van Gorp, Ph.D. AACN cosponsored the Houston Conference on Specialty Education and Training in Clinical Neuropsychology in 1997. This conference was a national consensus conference of neuropsychological organizations held with the purpose of establishing training guidelines for clinical neuropsychology. The Houston Conference guidelines have since become the model for most programs offering formal training in clinical neuropsychology. AACN held its first annual conference in 2003. During that same year, The Clinical Neuropsychologist became AACN’s official journal. In 2007, AACN began on-line Continuing Education (CE) programs.

American Academy of Neurology 1080 Montreal Avenue Saint Paul, Minnesota 55116 www.aan.com (800) 879-1960 (US) (651) 695-2717 (international) (651) 361-4800 (fax)

Major Activities AACN hosts one conference each year. This conference is open to both members and nonmembers. The official journal published by AACN is The Clinical Neuropsychologist.

Membership The American Academy of Neurology (AAN), established in 1948, is an international professional association of more than 21,000 neurologists and neuroscience professionals dedicated to providing the best possible care for patients with neurological disorders. The AAN is strongly committed to its mission of ensuring the maintenance of the principles and standards set forth in the AAN mission statement. Approximately 22,000 members reside in the USA and 4,000 are international members. Membership includes clinicians, academicians, researchers, business administrators, residents, fellows, and medical students.

American Academy of Neurology (AAN)

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Major Areas or Mission Statement

Major Activities

The vision of the AAN is to be indispensable to its members. The mission of the AAN is to promote the highest-quality neurologic care and enhance member career satisfaction. To accomplish these purposes, the AAN has established the following organizations to support its membership:

Physician Education and Lifelong Learning

The American Academy of Neurology Foundation (AAN Foundation), established in 1993, raises funds to support clinical research in neurologic disorders. AAN Enterprises, Inc. (AEI), a for-profit subsidiary of the AAN, was formed in 1999 by the AAN to develop new sources of revenue to pay for state-of-the-art products and services for its membership. The American Academy of Neurology Professional Association (AANPA) was established in 2007 and includes all AAN members. The AANPA created a political action committee, BrainPAC, to represent the interests of USA neurologists in Washington, DC.

Landmark Contributions The AAN was founded in 1948 by A. B. Baker, MD, chair of the neurology department of the University of Minnesota, in response to the difficulties of one of his residents, Joseph Resch, in finding a society that he could join to continue his education and network with fellow neurologists. Baker was aided by Adolph L. Sahs, MD, of the University of Iowa; Francis M. Forster, of Jefferson Medical Hospital in Philadelphia; and Russell DeJong, MD, of the University of Michigan. Baker served as the first Academy president, and Forster and Sahs later had terms as president. DeJong was the founding editor-inchief of the journal Neurology®, which began publication in 1951. The AAN had 52 founding members. The establishment of the Academy, coupled with the increased need for neurologists due to World War II, helped elevate the status of neurology as a practice distinct from psychiatry. In 1947, there were between 300 and 325 physicians in the USA who designated themselves as primary neurologists, and there were 32 residency positions available nationwide. By 1970, there were 2,727 primary neurologists and some 700 residents in training. By the end of 2007, there were more than 16,000 neurologists in the USA. Currently, nearly 2,200 residents have memberships with the AAN.

The AAN’s Annual Meeting is one of the largest gatherings of neurology professionals in the world. Held each spring, the event attracts nearly 13,000 clinicians, academicians, researchers, exhibitors, and media representatives to share the latest in neurology science and education. The AAN also offers members three-day regional conferences in the fall of each year, and occasional workshops. Education activities and programs are structured to support the ongoing development of neurology professionals from medical students to accomplished clinicians and scientists.

Science and Research The Annual Meeting is a leading forum for sharing the latest developments in science and research, as is the weekly peer-reviewed journal Neurology®. AAN scientific awards, presented at the Annual Meeting, honor outstanding achievements in neurology, from aspiring medical students to veteran researchers. Through the AAN foundation, the AAN provides support to young researchers through more than a dozen clinical research training fellowships, enabling them to pursue research initiatives and helping them to secure academic appointments and future fundings.

Clinical Practice The AAN develops clinical practice guidelines to assist its members in clinical decision making related to the prevention, diagnosis, treatment, and prognosis of neurologic disorders. Each guideline makes specific practice recommendations based upon a rigorous and comprehensive evaluation of all available scientific data. The AAN also develops position statements on a variety of ethical issues to help guide neurologists and others in decision making. Members also rely on the AAN for the latest information on coding, reimbursement, quality initiatives, patient safety, and practice management issues.

Advocacy To help foster changes in health care that will benefit patients and enhance the practice of neurology, the AAN presents advocacy training opportunities for members

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through the Donald M. Palatucci Advocacy Leadership Forum, and the Kenneth M. Viste, Jr., MD, Neurology Public Policy Fellowship. Members also participate in the annual Neurology on the Hill visits to the USA Capitol in Washington, DC. The AANPA’s BrainPAC political action committee also is instrumental in representing neurology’s interests on the federal level and supporting federal legislators who support the profession and patients with neurologic disorders.

Publishing AAN Enterprises, Inc., has four highly successful publications published by Lippincott Williams and Wilkins. The weekly journal Neurology® is the most widely read peerreviewed neurology journal in North America. Neurology Today®, published biweekly, leads all other neurology tabloids in readership. Neurology Now®, a bimonthly patient-oriented magazine available in AAN member offices, currently has about 256,000 subscribers. Continuum: Lifelong Learning in Neurology®, the AAN’s bimonthly continuing education monograph, is recognized by the American Board of Psychiatry and Neurology as a key tool for maintenance of certification. AEI also publishes the monthly member magazine AANnews, which focuses on AAN activities, events, and services; a book series for patient and their families on treating and living with neurologic disorders; and textbooks geared toward professionals.

Cross References ▶ Neuropsychiatry

References and Readings Visit the AAN online at www.aan.com.

American Academy of Pediatrics D EBBIE L INCHESKY American Academy of Pediatrics Elk Grove Village, IL, USA

Membership The American Academy of Pediatrics (AAP) has approximately 60,000 members in the USA, Canada, Mexico, and

many other countries. Members include pediatricians, pediatric medical subspecialists, and pediatric surgical specialists. More than 34,000 members are board-certified and called Fellows of the American Academy of Pediatrics (FAAP).

Major Areas or Mission Statement The AAP is committed to the attainment of optimal physical, mental, and social health and well-being for all infants, children, adolescents, and young adults.

Landmark Contributions The AAP was founded in June 1930 by 35 pediatricians who met in Detroit in response to the need for an independent pediatric forum to address children’s needs. When the AAP was established, the idea that children have special developmental and health needs was a new one. Preventive health practices now associated with child care – such as immunizations and regular health exams – were only just beginning to change the custom of treating children as ‘‘miniature adults.’’

Major Activities One of the AAP’s major activities is to further the professional education of its members. Continuing education courses, annual scientific meetings, seminars, publications and statements from committees, councils, and sections form the basis of a continuing postgraduate educational program. More than 30 committees develop many of the AAP’s positions and programs. Committees have interests as varied as injury and poison prevention, disabled children, sports medicine, nutrition, and child health financing. The AAP currently has six councils and 48 sections consisting of more than 41,500 members with interest in specialized areas of pediatrics. This includes a section for resident physicians with more than 9,000 members. Sections and councils present educational programs for both their members and the general membership of the AAP in order to highlight current research and practical knowledge in their respective subspecialties. The AAP publishes Pediatrics, its monthly scientific journal; Pediatrics in Review, its continuing education journal; and its membership news magazine, AAP News.

American Board of Clinical Neuropsychology (ABCN)

It also publishes manuals on such topics as infectious diseases and school health. In its public education efforts, the AAP produces patient education brochures and a series of child care books written by AAP members. The AAP executes original research in social, economic, and behavioral areas and promotes funding of research. It maintains a Washington, DC office to ensure that children’s health needs are taken into consideration as legislation and public policy are developed. The AAP’s state advocacy staff provides assistance to chapters, promoting issues such as child safety legislation and Medicaid policies that increase access to care for low-income children.

American Board of Clinical Neuropsychology (ABCN) M ICHAEL W ESTERVELD 1, K EITH O. Y EATES 2 1 Florida Hospital Orlando, FL, USA 2 Nationwide Children’s Hospital Columbus, OH, USA

Address (and URL) The American Board of Clinical Neuropsychology (ABCN) is an organization that awards board certification to practicing clinical neuropsychologists. It is a member of the American Board of Professional Psychology (ABPP). Information about ABCN can be obtained from the ABCN web site (www.theabcn.org) and also at the ABPP web site (www.abpp.org). Mail correspondence for ABCN can be directed to: Department of Psychiatry (F6248, MCHC-6) University of Michigan Health System 1500 East Medical Center Drive, SPC 5295 Ann Arbor, MI 48109-5295

Membership As of May, 2010 ABCN had awarded 748 diplomas. Diplomates from throughout the USA, District of Columbia, and Canada are represented among the ranks of ABCN. Awarding of the ABCN diplomate is based primarily on clinical knowledge and skill, as demonstrated throughout the examination process which includes a written examination, practice sample review,

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and oral examination. Because the diploma is based on peer review of clinical competency, the majority of ABCN diplomates are active clinicians. However, many also engage in clinical and basic science research, teaching, and a wide range of other professional activities. The ABCN Board of Directors consists of 15 members elected by diplomates in good standing. The term of office is 5 years. Officers of the Board (President, Vice President, Secretary, Treasurer) are elected by the Board from among active elected directors. Elected Board members may serve no more than two consecutive terms. In addition to elected Board members, there is an examination chairperson, selected by the Board for a term of 5 years.

Major Areas or Mission Statement According to the ABCN bylaws, the organization exists to develop and maintain procedures to examine the qualifications of candidates for board certification in Clinical Neuropsychology, to conduct the examinations and award certificates to qualified candidates, to maintain a registry of certificate holders, and to serve the public welfare by identifying practitioners who have obtained advanced education and training in clinical neuropsychology and demonstrated the ability to apply such skills in a competent manner.

Landmark Contributions ABCN was incorporated in 1981. After the findings of the joint Division 40-INS task force on Education, Accreditation, and Credentialing in 1981 (published in 19845 and republished in the first issue of The Clinical Neuropsychologist in 19877) established requisite education and training experiences, the need for a means of identifying welltrained and competent practitioners was recognized. A planning group (Linas Bieliauskas, Louis Costa, Edith Kaplan, Muriel Lezak, Charles Matthews, Steven Mattis, Manfred Meier, and Paul Satz) incorporated ABCN in Minneapolis in 1981. The organization was formed with the intention of affiliating with the ABPP, a unifying governing body for independently incorporated specialty examining boards akin to the ABMS for medical specialties. After the first examinations were completed in 1983, ABCN formally affiliated with ABPP (also in 1983) and the first ABPP–ABCN diplomas were awarded in 1984. The first President of ABCN was Manfred Meier, who served from 1983 until 1991.

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ABCN was initially established as an organization solely charged with awarding diplomas to applicants successfully demonstrating competency through the examination process. In 1988, it became a membership organization and began charging dues so that resources for further development of the organization could be built. This included creation and maintenance of a written examination in consultation with Professional Examination Services (PES). After years of development and pilot testing to assure validity and reliability of the written examination, in 1993, ABCN began to require that new candidates pass the written examination prior to submitting practice samples. The written examination is regularly reviewed for content updates to remove outdated items and assure that advances in clinical practice and knowledge in the field are reflected in the examination. The American Academy of Clinical Neuropsychology (AACN), an organization originally comprised of ABCN diplomates, was formed in 1996. ABPP had received legal advice that there was potential for conflict of interest in the roles of credentialing bodies that also engaged in advocacy. As a result, the academy was formed to fulfill the advocacy and professional development role. AACN has grown significantly and now includes an affiliate member category for neuropsychologists who have not yet received their ABCN diploma, and for affiliated professionals who are not neuropsychologists. Although ABPP has recently received a different legal opinion that allowed member boards to once again merge with their academies, ABCN and AACN have grown and function well in their complementary roles and at this time have no plans to merge. In 1997, a landmark conference regarding education and training for clinical neuropsychologists was held in Houston (the ‘‘Houston Conference on Specialty Education and Training in Clinical Neuorpsychology’’). Attending the conference were representatives from each of the professional neuropsychology organizations, and the proceedings were published in 1998. In 2002, the ABCN Board of Directors voted to adopt the Houston Conference training guidelines as requisite training to be eligible for the ABCN diplomate. Candidates who received their degrees after January 1, 2005 are expected to have had training and experience consistent with the guidelines laid out in the Houston Conference proceedings. In 2007, ABCN began to consider subspecialization within the field, and address examination and recognition of special competencies, such as pediatric neuropsychology. At that time, ABPP did not have a model for subspecialization, and worked with ABCN to develop a framework to address issues such as overlap with other

boards and recognition of special competencies of existing board members. As a result, the Pediatric Special Interest group was formed, and held the first meeting in 2009 during the AACN Conference. Table 1 presents a timeline summary of major landmarks for ABCN.

Major Activities ABCN’s primary activities are developing, maintaining, and conducting the examination. The examination process consists of four distinct steps. First, the education and training experiences of the applicant are reviewed, initially at the ABPP central office, where the application is examined for graduate training, internship, and licensure status. Applications are then forwarded to ABCN for review of advanced specialty training. Any practicing clinical neuropsychologist with a doctoral-level degree who possesses a valid license to practice psychology is eligible to

American Board of Clinical Neuropsychology (ABCN). Table 1 Timeline for major ABCN milestones 1981 ABCN incorporated in Minnesota 1983 First set of examinations completed 1983 Formal affiliation between ABCN and ABPP established 1984 First ABCN/ABPP diplomates awarded 1988 ABCN bylaws revised to create membership organization 1989 ABCN designated Specialty Council in Clinical Neuropsychology by ABPP 1993 Written examination formally instituted 2002 AACN establishes mentoring program to promote board certification through ABCN 2002 ABCN votes to adopt Houston Conference guidelines for eligibility for board certification, beginning in 2005 2002 Written examination updated to reflect Houston Conference guidelines 2004 500th ABCN diploma awarded 2004 BRAIN Website and Listserv group formed 2005 Houston Conference education and training requirements implemented 2007 Committee to study subspecialization formed 2009 700th diploma awarded 2009 Pediatric Neuropsychology special interest group formed


apply. Beginning in 2005, applicants for the ABCN diploma are expected to complete training consistent with the Houston Conference on Specialty Education and Training in Clinical Neuropsychology. This includes coursework in the areas outlined in the Houston Conference, and completion of a formal 2-year postdoctoral residency program in Clinical Neuropsychology. However, recognizing that the field has evolved, applications from candidates who obtained their graduate training prior to implementation of the Houston Conference standards are evaluated according to the standards in place at the time their degree was granted, provided they can demonstrate that the pertinent requirements were met during their training (see www.theabcn.org for detailed requirement listings). Candidates who are respecializing in neuropsychology, or who recently completed respecialization programs, are expected to have education and training experiences consistent with the requirements in place at the time of their respecialization, not the date of their original degree. Once an applicant’s credentials have been reviewed and accepted, the next step in the examination process is a written examination. The written examination consists of 100 multiple-choice questions that cover a range of topics in neuropsychology. It is intended to evaluate the candidate’s breadth of knowledge and to assure that they have the foundational knowledge necessary for competent practice in clinical neuropsychology. It is administered at major conferences, including the AACN conference, National Academy of Neuropsychology (NAN) annual meeting, International Neuropsychology Society (INS) North American Meeting, and the American Psychological Association (APA) meeting. It was developed and is maintained in association with PES. Once a candidate has passed the written examination, the next step is submission of a practice sample consisting of two typical cases in the candidate’s practice. The practice samples are reviewed by at least three independent, board certified neuropsychologists. Following acceptance of the practice sample, the candidate is invited to sit for the oral examination that consists of three parts – practice sample, fact-finding, and ethics/professional development. The practice sample section of the orals provides the candidate an opportunity to discuss their practice as applied to the specific cases they submitted. The cases also serve as a starting point leading to more in-depth discussion of differential diagnosis, and general information about the nature of the disorder in the case and related conditions. The fact-finding section of the oral examination is an opportunity for the candidate to demonstrate clinical skills. The candidate is

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presented with a brief description of a case, and is asked to inquire about background history, test data, and related medical information to arrive at a clinical diagnosis and conclusion. Along the way, the candidate may be asked about their rationale for test selection, their differential diagnostic considerations, and how test results may support or otherwise aid in diagnosis and treatment planning. The professional and ethical portion of the examination is an opportunity for the candidate to demonstrate knowledge of important ethical considerations in the practice of neuropsychology, as well as discuss important issues for the field. A comprehensive overview of the examination process was published in 2008 (Armstrong, et al., 2008). Currently, ABCN conducts written examinations at four major conferences each year: International Neuropsychology Society (INS North American Meeting) American Academy of Clinical Neuropsychology (AACN) American Psychological Association (APA) National Academy of Neuropsychology (NAN) Oral examinations are conducted twice annually in Chicago, Illinois, hosted by Rush University Medical Center. One examination is conducted each autumn (usually late October, or early November) and the other in the spring (usually early May). The AACN holds an annual conference for continuing education, professional development, and furthering the growth of the profession through advocacy. The meeting is held annually in June.

Cross References ▶ American Academy of Clinical Neuropsychology (AACN) ▶ American Board of Professional Psychology (ABPP) ▶ American Psychological Association (APA) ▶ International Neuropsychology Association (INS) ▶ Meier, Manfred John (1929–2006) ▶ National Academy of Neuropsychology (NAN)

References and Readings Armstrong, K., Beebe, D. W., Hilsabeck, R. C., & Kirkwood, M. W. (2008). Board certification in clinical neuropsychology: A guide to becoming ABPP/ABCN certified without sacrificing your sanity. Oxford Press.

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American Board of Pediatric Neuropsychology

Bieliauskas, L. A., & Matthews, C. G. (1987). American Board of Clinical Neuropsychology: Policies and procedures. The Clinical Neuropsychologist, 1, 21–28. Bieliauskas, L. A., & Matthews, C. G. (1990). American Board of Clinical Neuropsychology Update, 1990. The Clinical Neuropsychologist, 4, 337–343. Bieliauskas, L. A., & Matthews, C. G. (1997). The American Board of Clinical Neuropsychology, 1996 update: Facts, data, and information for potential candidates. The Clinical Neuropsychologist, 11, 222–225. Hannay, H. J., Bieliauskas, L., Crosson, B. A., Hammeke, T. A., Hamsher, K. deS., & Koffler, S. (Eds.). (1998). Proceedings of the Houston Conference on specialty education and training in clinical neuorpsychology. Archives of Clinical Neuropsychology, 13, 157–250. Ivnik, R. J., Haaland, K. Y., & Bieliauskas, L. A. (2000). American Board of Clinical Neuropsychology special presentation. The Clinical Neuropsychologist, 14, 261–268. Report of the Division 40/INS Joint Task Force on Education, Accreditation, and Credentialing (1984). Division 40 Newsletter, Vol. 2, no. 2, pp. 3–8. Reports of the ins - division 40 task force on education, accreditation, and credentialing (1987). The Clinical Neuropsychologist, 1(1), 29–34. Yeates, K. O., & Bieliauskas, L. A. (2004). The American Board of Clinical Neuropsychology and American Academy of Clinical Neuropsychology: Milestones past and present. The Clinical Neuropsychologist, 18, 489–493.

training in pediatric neuropsychology (from graduate school to continuing education), written examination, a practice sample submission, and an oral examination. The ABPdN does not have a ‘‘grand fathering’’ policy, and thus, all existing board members were required to complete all new phases of the examination process to ensure equality of standards among boarded members. As of early 2010, 111 neuropsychologists have submitted applications to ABPdN and 75 members have passed the ABPdN examination process. At present, there are 57 active and five emeritus members of the board from 21 states, Canada, and Puerto Rico.

Major Areas or Mission Statement Board certification in pediatric neuropsychology serves to assist consumers by offering supportive evidence of the competence of the pediatric neuropsychologists. The ABPdN is the only board certification organization with the sole purpose of examining and certifying competence in pediatric neuropsychology.


American Board of Pediatric Neuropsychology P ETER D ODZIK American School of Professional Psychology-Schaumburg Schaumburg, IL, USA

Membership The American Board of Pediatric Neuropsychology (ABPdN) was developed in 1996 by a coalition of clinical practitioners, representing institutions hiring pediatric neuropsychologists. The original group conceived the board to advance their belief that a unique interplay exists between neurodevelopmental issues and neuropsychological assessment that requires special sets of expertise not readily assessed by the then existing boarding entities. Following discussion with colleagues who were members of medical practice and psychology boards, the coalition elected to establish an independent certifying authority. The examination process evolved into a comprehensive and multilevel process that includes a written application including clinical case vignettes used to determine decision-making strategies of the applicant, scope of practice and a thorough assessment of organized

Members of ABPdN practice in a variety of settings including universities, teaching hospitals, general hospitals, hospital trauma centers, private practices, rehabilitation facilities, stroke centers, memory disorder centers, group practices, and child development centers. Current members hold academic affiliations at over 40 colleges and universities. Several members have developed tests commonly used in the practice of pediatric and general neuropsychology. Member accomplishments include past president of APA Division 40, current and past presidents of four State Psychology Boards, past president of National Academy of Neuropsychology, past and present editor of Archives of Clinical Neuropsychology, past editor of Journal of School Psychology, and the owner/moderator of PEDS-NPSY, a pediatric list-serve with over 1,600 members.

Major Activities The ABPdN is the board-certifying arm of the American Academy of Pediatric Neuropsychology (AAPN), which is devoted to training and promotion of the field of pediatric neuropsychology. The AAPN, in affiliation with the American College of Professional Neuropsychology, holds an annual conference each spring with topics related to the field of pediatric neuropsychology.


The primary activity of ABPdN is conducting the board certification process. Board examination through the ABPdN involves several stages. The format of the ABPdN’s examination processes has been constant since the examinations held in 2004, but the procedures continue to be reviewed and amended. The purpose of the ABPdN examination process is to ensure that the examinee has demonstrated competency to practice pediatric neuropsychology. The specific stages are discussed below and more detail can be obtained from the ABPdN web site (Beljan, Bos, Courtney, & Dodzik, 2006). The overall pass rate for each stage of the examination process is between 73% and 81%.

Credential Review Minimum training and education standards include completion of a doctoral degree from a regionally accredited program in applied psychology that was, at the time the degree was granted, accredited by the APA, CPA, or was listed in the publication Doctoral Psychology Programs Meeting Designation Criteria (ASPPB National Register designation committee, 2008). Membership in the National Register of Health Service Providers in Psychology, the Canadian Register of Health Service Providers, or those holding the Certificate of Professional Qualification qualify as meeting the doctoral requirements for membership. Licensure or certification at the independent practice level as a psychologist in the state, province, or territory in which the psychologist actively practices is also required. The applicant must be practicing as a pediatric neuropsychologist and must have completed an Association of Psychology Postdoctoral and Internship Center (APPIC) or APA accredited internship that included a documented rotation or concentration in neuropsychology, and 2 years of postdoctoral supervised experience in neuropsychology, at least 50% of that being pediatric-oriented. In addition, each applicant reviewed by the Board must provide the following: 1. Education a) Undergraduate degree transcript b) Graduate degree transcript c) Internship verification contact information d) Postdoctoral residency verification contact information e) Postdoctoral fellowship verification contact information (if applicable) f) Detailed description of training in pediatric neuropsychology (narrative)

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2. Continuing education a) Verification of CEUs in pediatric neuropsychology for the past 3 years 3. Clinical work a) Clinical appointment verification contact information b) Breakdown of clinical practice by age, disorders, and ethnic background c) Completion of clinical vignettes 4. Educational appointment (if applicable) a) Academic institution verification contact information The application is first reviewed by the Examination Chair for completion and accuracy of documents and licensure status. The application is then reviewed by a panel of three reviewers. A passing score by two of the three reviewers is required to move to the next stage of the examination. Each reviewer evaluates the application for consistent and thorough training in pediatric neuropsychology at multiple levels of training.

Practice Sample The purpose of the practice sample is to determine the applicant’s clinical knowledge. While the written examination was designed to assess content-specific knowledge with regard to pediatric neuropsychology, the practice sample allows the board to evaluate the day-to-day skills of the applicant. To that end, the sample should reflect a typical patient seen in the applicant’s clinical practice. Practice samples may include assessment or intervention techniques. After an application is reviewed and the candidate is determined to be board-eligible, they will then be invited to provide a practice sample that reflects their typical work in pediatric neuropsychology. Prior to taking the objective and oral examination, the candidate must prepare and tender a written sample of an original pediatric neuropsychological examination performed solely by the candidate. Appropriate samples may also include case analysis/interventions and supervision sessions.

Written Examination The third step is the written exam, a 100 question, multiplechoice instrument designed and constructed by other pediatric neuropsychologists whose purpose is to assess the candidate’s breadth of knowledge in pediatric neuropsychology. The questions were first assessed for face validity, clustered for content area, rank-ordered, deleted or refined,

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reanalyzed, debated, approved, and then compiled into a larger item pool for random selection by domain each year. A passing score of 70% is required. Each exam includes the following basic core areas:

Psychometrics Pediatric Neurosciences Psychological and Neurological Development Neuropsychological and Neurological Diagnostics Ethics and Legal Issues Research Design Review for Clinical Application Intervention Techniques Consultation and Supervisory Practices

Oral Examination This part of the examination process is comprised of a review of the candidate’s practice sample, the nature and application of neuropsychological knowledge to their current practice, appreciation for ethical issues and obligations, and a review of the candidate’s views and philosophy on pediatric neuropsychology. The oral examination also includes a mock case review, in which the candidate is given information about a fictional case, and they develop and articulate their working hypothesis. The oral examination is intended to be a collegial opportunity for the reviewers to validate the candidate’s ability to ‘‘think on their feet’’ and discern their preparation and readiness for board certification. The first portion of the oral examination permits the examination team to consider the scope of the candidate’s body of training and how they practice pediatric neuropsychology (e.g., acute care, rehabilitation, outpatient, assessment, and/or treatment) so that the fact-finding and practice sample review can be conducted in the most relevant fashion. This section is broken into two parts: Part I: The examinee will explain their background.

The examinee will provide a history of their educational and professional background. Special consideration should be given to their pediatric neuropsychological training and background. The examinee will explain their current role as a pediatric neuropsychologist and the issues their typical clientele present.

Part II: The examinee will demonstrate pertinent knowledge of practical pediatric neuropsychology. The next segment of the oral examination allows the candidate to present the material in their practice sample and to provide an overview of the history, evaluation process, and outcome of the case. The examiners evaluate their

ability to articulate the major findings and their rationale. Candidates discuss their rationale in such areas as: (1) Test selection (if applicable): psychometric properties, test validity/reliability, limitations for use, and exclusion of all competing diagnoses. (2) Test interpretation (if applicable): alternate interpretations of findings, conflict resolution within the data, discussion of strengths and weaknesses, and environmental and cultural factors. (3) Diagnostic conclusions: alternate diagnosis, ultimate understanding of neuropathology, prognosis, progression, lateralizing/localizing effects, pathognomic signs, causality, environmental conditions, and effects on neural development. (4) Recommendations and treatment planning: best practices for treatment, availability, prognosis, funding, delivery options, cost/benefit analysis, iatrogenic outcomes, parental compliance/agreement, and ethical issues. (5) Consultation and supervision (if applicable): best practices for communication of data, delivery options, supervisee needs/relationships, and rapport/therapeutic relationship. This process is intended to be collegial and the examiners endeavor to be sensitive to the different and yet equally viable approaches within pediatric neuropsychology. The purpose is to ascertain the Candidate’s logic and thought processes and to allow them to demonstrate these skills. During the ethics segment, there is discussion of one or two standardized vignettes, and the candidate is expected to present relevant comments on the ethical dilemmas, thoughtfully weighing them in the light of the APA ethics principles, professional practice standards, and relevant statutes.

Cross References ▶ American Psychological Association (APA), Division 40 ▶ National Academy of Neuropsychology (NAN)

References and Readings ASPPB National Register designation committee (2008). Retrieved October 1, 2009 from http://www.nationalregister.org/designate_ stsearch.html Beljan, P., Bos, J., Courtney, J., & Dodzik, P. (2006). Preparation guide for examination and certification by the American Board of Pediatric Neuropsychology. Retrieved from http://abpdn.org/docs/studyguide.pdf For additional information please see the web site at www.abpdn.org.

American Board of Professional Psychology (ABPP)

American Board of Professional Psychology (ABPP) C HRISTINE M AGUTH N EZU Drexel University – Hahnemann Campus Philadelphia, PA, USA

Membership The American Board of Professional Psychology (ABPP) has 3,074 currently active board-certified specialists in membership. As a national-in-scope credentialing organization in professional psychology, its membership is comprised doctoral-level psychologists who provide professional services and consultation and are licensed to practice psychology in the jurisdiction in which they practice. Completion of a doctoral degree, completion of a qualified internship, relevant postdoctoral experience, and relevant jurisdictional licensure as a psychologist are the minimum prerequisites for approval to take an ABPP board certification exam.

Major Areas or Mission Statement The American Board of Professional Psychology (ABPP) is a national-in-scope credentialing organization that has been awarding board certification in professional psychology specialties for over 60 years (Bent, Packard & Goldberg, 1999; Finch, Simon & Nezu, 2006; Packard & Reyes, 2003). ABPP describes the value of its credential as one that ‘‘provides peer and public recognition of demonstrated competence in an approved specialty area in professional psychology’’ (American Board of Professional Psychology, 2008). Moreover, ABPP board certification is increasingly associated with greater opportunities for career growth, including employment opportunities, practice mobility between jurisdictions, and financial compensation (American Board of Professional Psychology; Sweet, Nelson & Moberg, 2006). ABPP is currently a unique and unitary umbrella organization with multiple specialty boards that include cognitive-behavioral, clinical, clinical child and adolescent, clinical health, clinical neuropsychology, counseling, couples and family, forensic, group, school, rehabilitation, organizational, business, and consulting, and psychoanalysis. Many professional psychologists seek dual certifications that reflect the full scope of

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their specialties. Examples of these might include clinical and cognitive-behavioral, clinical neuropsychology and rehabilitation, or counseling and group. For a licensed psychologist to be ‘‘board eligible,’’ each of the 13 boards require that he or she meets both generic and specialty eligibility criteria concerning education, professional training, and licensure in the jurisdiction where professional services are provided. Once an individual’s credentials are reviewed and approved, the individual seeking board certification moves to the next phase of their candidacy process. In clinical neuropsychology and forensic specialties, this necessitates passing a written examination. In all other specialties, the candidates are not required to take a written exam, and may move directly to the final phases in the process. For all specialties, this includes first submitting a professional practice sample. After the practice sample is approved, the oral examination (final phase) is typically scheduled. Specialty boards may also provide a ‘‘senior option’’ regarding practice samples submitted by candidates with at least 15 years of experience post licensure who may submit samples of their professional work such as publications, treatment manuals, program manuals, or a comprehensive summary of their professional practice, to satisfy the requirements of a professional practice sample. With regard to both practice samples and oral exams, the candidate’s competency is assessed across various domains. These competency domains may be functional in nature, and include the day-to-day activities of specialty practice, such as assessment, intervention, and/or consultation that are informed by a scientific literature base. They also include foundational competencies, such as ethics, individual and cultural diversity, and interpersonal competence, which cut across all of a specialist’s other activities. The competency model upon which ABPP board certification is based, draws from several important sources such as the APA-sponsored Competencies Conference in 2002 and resulting Task Force on Assessment of Competence in Professional Psychology (Kaslow et al., 2007), and a review of competency assessment models developed both within (e.g., Assessment of Competence Workgroup from Competencies Conference – Roberts, Borden, Christiansen, & Lopez, 2005; Leigh et al, 2007) and outside (e.g., American Council for Graduate Medical Education and American Board of Medical Specialties, 2000) of the profession of psychology. There is a strong consensus among many professional psychologists that the American Board of Professional Psychology represents a high degree of integrity regarding

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specialty board certification and serves as a gold standard for demonstration of specialty competency in professional psychology.

For interested applicants, it contains application instructions as well as other helpful information. The organization will publish its first book, Becoming Board Certified by the American Board of Professional Psychology (ABPP) in 2009.

Landmark Contributions The origins of ABPP can be traced back to its establishment in 1947 as the American Board of Professional Examiners in Psychology (Bent et al., 1999). The intention of the original board was to ensure that individuals were qualified to perform the professional service activities associated with clinical and counseling psychology. However, as professional psychology expanded its scope and depth, the organization changed its name to the American Board of Professional Psychology to reflect the expansion of specialization activities that were emerging for professional psychologists. As a result, the number of its affiliated specialty boards and associated academies has grown from 3 to 13, reflecting this professional expansion and the breadth of specialties that have emerged over that past 5 decades (Finch et al., 2006; Packard & Reyes, 2003).

Major Activities Each of the psychology specialty boards under the ABPP umbrella has an elected trustee who participates as a member of the ABPP Board of Trustees as the overall governance group of the ABPP. Each specialty board assumes the responsibility for developing and carrying out the ABPP specialty examinations. The ABPP central office, under the management of a full-time Executive Officer, executes important day-to-day functions for all of the 13 specialty boards. These include generic candidacy verification of applicants, budget maintenance and accounting responsibilities, record keeping, development and maintenance of an ABPP Directory, development and editing responsibility for the ABPP website, monitoring the organization relative to ethical/legal issues, planning of conference and governance activities, and general administrative support. The primary publication of the organization, The Specialist, is published twice annually and available to all members in both electronic and printed format. The organization website (www.ABPP.org) contains important information regarding the mission, governance, and organizational documents. For the public, the website contains listings of board-certified specialists across specialties and practice jurisdictions.

Cross References ▶ American Academy of Clinical Neuropsychology (AACN) ▶ American Board of Clinical Neuropsychology (ABCN) ▶ American Board of Rehabilitation Psychology (ABRP)

References and Readings American Council for Graduate Medical Education and American Board of Medical Specialties (2000). Toolbox of assessment methods. Chicago, IL: American Council for Graduate Medical Education and American Board of Medical Specialties. American Board of Professional Psychology (2008). Retrieved June 25, 2008, from http://www.abpp.org Bent, R. J., Packard, R. E., & Goldberg, R. W. (1999). The American board of professional psychology. Professional Psychology: Research and Practice, 30, 65–73. Datillio, F. M. (2002). Board certification in psychology: Is it really necessary? Professional Psychology: Research and Practice, 33, 54–57. Finch, A. J., Simon, N. P., & Nezu, C. M. (2006). The future of clinical psychology: Board certification. Clinical Psychology: Science and Practice, 13, 254–257. Kaslow, N. J., Rubin, N. J., Bebeau, M. J., Leigh, I. W., Lichtenberg, J. W., Nelson, P. D., Portnoy, S. M., & Smith, I. L. (2007). Guiding principles and recommendations for the assessment of competence. Professional Psychology: Research and Practice, 38, 441–451. Leigh, I. W., Smith, I. L., Bebeau, M. J., Lichtenberg, J. W., Nelson, P. D., Portnoy, S., Rubin, N. J., & Kaslow, N. J. (2007). Competency assessment models. Professional Psychology: Research & Practice, 38, 463–473. Nezu, C. M., Finch, A. J., & Simon, N. P. (Eds.) (2009, in press), Becoming board certified by the American board of professional psychology (ABPP). New York: Oxford University Press. Packard, T., & Reyes, C. J. (2003). Specialty certification in professional psychology. In M. J. Prinstein & M. D. Patterson (Eds.), The portable mentor: Expert guide to a successful career in psychology (pp. 191– 208). New York: Plenum. Roberts, M. C., Borden, K. A., Christiansen, M. D., & Lopez, S. J. (2005). Fostering a culture shift: Assessment of competence in the education and careers of professional psychologists. Professional Psychology: Research and Practice, 36, 355–361. Sweet, J. J., Nelson, N. W., & Moberg, P. J. (2006). The TCN/AACN 2005 ‘‘Salary Survey’’: Professional practices, beliefs, and incomes of U.S. Neurophysiologists. The Clinical Neuropsychologist, 20, 325–364.

American Board of Professional Neuropsychology (ABN)

American Board of Professional Neuropsychology (ABN) J OHN E. M EYERS Private Practice, Neuropsychology Mililani, Hawaii, USA

Membership The American Board of Professional Neuropsychology (ABN) comprises 350 (as of 2010) neuropsychologists who have doctoral degrees, and they are licensed as psychologists and have completed the ABN diplomate examination process. ABN was established in 1982 by a group of clinical neuropsychologists, all of whom were diplomates of the American Board of Professional Psychology (ABPP), to provide peer regulation of the practice of professional neuropsychology. The process of obtaining the ABN diplomate is a dynamic one which has changed over the years and is expected to evolve as the field of neuropsychology evolves. Initially, in addition to obtaining a doctoral degree, licensure as a psychologist, and completing a number of years of postdoctoral experience in neuropsychology, early applicants were required to show evidence of specialized training in neuropsychology and to provide supervisory evaluations of their competency in professional neuropsychology. Between 1982 and 1985, following a review of credentials and supervisory evaluations, work samples were required. These were graded by multiple examiners on a pass/fail basis. Individuals who passed this final step were awarded a diplomate. Individuals who did not pass evaluation were allowed to apply for a ‘‘Certificate in Professional Neuropsychology,’’ indicating that they had some training in neuropsychology but not sufficient to be awarded diplomate status. This was initially intended as an interim credential as part of the process of obtaining a diplomate. After 1985, this process was abandoned as increasing numbers of neuropsychology training programs became available. In February of 1989, the ABN was reorganized and the bylaws were modified. An annual dues structure was instituted and ABN became a membership organization whose only credential is a diplomate. This newly established organization mandated continuing education for active membership. It was required that all those who had a ‘‘Certificate in Professional Neuropsychology’’ complete

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the diplomate process to maintain membership. At this time, an oral examination and essay examination were added to the case study reviews, and all previous members were allowed the opportunity to undergo the expanded examination process. Those who successfully completed the process, including the new oral examination, were given full diplomate status in ABN. After 1991, those who did not successfully complete the additional oral examination were no longer listed as diplomates through ABN. The oral examination included three 1 h sessions dealing with ethics, the work sample, and general knowledge. ABN no longer required letters of competency from supervisors but instead required letters of recommendation from other neuropsychologists. In 2004, the diplomate evaluation process was again reevaluated and work began on substituting a multiplechoice general knowledge examination for the oral examination on the same subject. This process took several years to complete, and as of January 1, 2009, all applicants were required to complete the multiple-choice written examination; the essay examination was dropped in favor of the multiple-choice exam. In 2008, the original acronym for ABN was changed from ABPN to ABN to avoid confusion with the American Board of Psychiatry and Neurology. The current examination procedure includes: 1. 2. 3. 4. 5.

Review of credentials and letters of recommendation A 100-question multiple-choice examination A case study-work samples review A 1-h ethics oral examination and A 1-h work style oral examination

The multiple-choice written examination covers areas of general knowledge based on the recommended guidelines of the Houston Conference. The ethics examination addresses ethical situations and current ethical dilemmas, and the work style examination covers clinical vignettes and clinical decision-making.

Major Areas or Mission Statement ABN recognizes and encourages the pursuit of excellence in the practice of clinical neuropsychology. ABN’s primary objective is the establishment of professional standards of expertise for the practice of clinical neuropsychology. Through its credentialing and examination processes and its continuing education requirement, the ABN offers to the medical community, the public, and to individuals

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who have a need for applied neuropsychological services, a process whereby competent professional neuropsychologists can be identified. To achieve the standards set forth by the ABN for competent professional practice of neuropsychology, the following outcome objectives have been developed:

Validate the skills of clinical practitioners Identify competent practitioners Provide public information about professional neuropsychology Document the maintenance of competence of professional neuropsychology practitioners with continuing education requirements Provide individuals, organizations, and agencies who use neuropsychology services with a referral directory of ABN diplomates

Recognition by ABN signifies to the public and to other health professionals a high level of competency in applied neuropsychology. The ABN does not ascribe to any specific theoretical framework. While recognizing the importance and contribution of a graduate education in neuropsychology and subsequent specialty training, the ABN believes that the critical element in the practice of professional neuropsychology is the application of that training to client issues and needs.


locations throughout the country. A workshop on the ABN examination process is held at least once a year. Individual candidate mentoring is offered throughout the year.

References and Readings http://www.neuropsychologyboard.org/ Bennett, T. L., Horton, A. M., Jr. & Elliott, R. W. (1999). American Board of Professional Neuropsychology (ABPN). Bulletin of the National Academy of Neuropsychology, 14, 7–9. Elliott, R. W., & Horton, A. M., Jr. (1994). Philosophy of the American Board of Professional Neuropsychology. Bulletin of the National Academy of Neuropsychology, 11, 14–15. Elliott, R. W., & Horton, A. M., Jr. (1995). History and current status of the American Board of Professional Neuropsychology. The Independent Practitioner, 15, 175–177. Goldstein, G. (2001). Board certification in clinical neuropsychology: Some history, facts and opinions. Journal of Forensic Neuropsychology, 2, 57–65. Horton, A. M. Jr., Crown, B. M., & Reynolds, C. R. (2001). American Board of Professional Neuropsychology: An Update-2001. Journal of Forensic Neuropsychology, 2, 67–78.

American Board of Rehabilitation Psychology DANIEL E. R OHE Mayo Clinic Rochester, Minnesota

‘‘Applied Neuropsychology,’’ a peer reviewed edited journal, is the official journal of the ABN.

Membership Major Activities ABN holds annual board of directors’ meetings in the spring and at the National Academy of Neuropsychology (NAN) conference. Associated with ABN is the American College of Professional Neuropsychology (ACPN) whose purpose is to provide continuing education programs in neuropsychology. The ACPN is approved by the American Psychological Association to provide continuing education programs. Every year, ACPN offers continuing education at an annual conference and at general membership meetings held in conjunction with other neuropsychological or psychological organizations. Twice a year, the board of directors and committee chairs meet to organize ABN’s professional activities. ABN candidate examinations and examiner training workshops are held a minimum of twice a year at

The American Board of Rehabilitation Psychology (ABRP) is one of 13-member boards of the American Board of Professional Psychology (ABPP). The ABRP consists of 135 (as of 2010) doctoral-level psychologists who are primarily engaged in provision of clinical services to individuals and their families affected by a wide range of disabilities and chronic health conditions including brain injury, spinal cord injury, amputations, chronic pain, multiple sclerosis, cancer, and sensory impairment such as blindness and deafness. In addition to clinical services, the majority of the members also engage in research, teaching, and administration of rehabilitation programs. Rehabilitation psychologists are also involved in interdisciplinary teamwork with other medical and rehabilitation providers. Rehabilitation psychologists who are boarded in the specialty reside in 30 states and Canada.

American Board of Rehabilitation Psychology

Major Areas or Mission Statement The mission of the ABRP is to protect the public and enhance the quality of health care by certifying rehabilitation psychologists who demonstrate the knowledge, skills, and attitudes essential to maximize quality of life for individuals with disabilities and chronic illness. The vision of the ABRP is that all psychologists practicing in rehabilitation will be boarded in the specialty. Psychologists who obtain the diplomate in rehabilitation psychology must meet the generic requirements for specialty certification by the ABPP that include a doctoral degree in psychology from an accredited degree program and licensure as a psychologist for independent practice in the USA or Canada. The ABRP-specific eligibility requirements include: completion of a recognized internship program and 2 years of supervised practice in rehabilitation psychology. In addition, the candidate must have completed at least 3 years of experience in rehabilitation psychology. Given the diverse training experiences of rehabilitation psychologists, the credential review includes significant reliance on the ratings of supervisors (two required) and the endorsement of colleagues and peers (two required). The candidate then submits a twopart practice sample (typically two case reports) that is evaluated by three ABRP examiners. Finally, the candidate completes an oral examination on: two clinical vignettes, their practice sample, and an ethics examination. The entire examination process is designed to ensure that each candidate demonstrates the foundational and the functional competencies required of the diplomate in rehabilitation psychology. The foundational competencies fall in four domains: interpersonal interactions, individual and cultural diversity, ethical and legal foundations, and professional identification. The functional competencies encompass science base and application, assessment, intervention, consultation, and consumer protection.

Landmark Contributions The primary contribution of ABRP is providing the opportunity for psychologists who are dedicated to the health and welfare of individuals with disabilities and chronic illness to be certified as rehabilitation psychologists. The ABRP began as a Credentials Committee within the Division of Rehabilitation Psychology in 1993. This committee met throughout 1993 and 1994 and incorporated as the American Board of Rehabilitation Psychology in 1995. On December 4, 1994 they established bylaws and elected officers: Richard Cox (president,

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1994–2000), Bernard Brucker (vice-president), Mitchell Rosenthal (secretary), Daniel Rohe (treasurer). The members at large were: Bruce Caplan, David Cox, Harry Parker, Anthony Ricci, James Whelan, and Mary Willmuth. Subsequent board presidents have been Mitchell Rosenthal (2000–2004), Bernard Brucker (2004–2008), and Daniel Rohe (current president). The second major contribution is the crafting of an organization that reflected the values of the professionals who created it. The ABRP devised an innovative examination process that is user-friendly, collegial, competencybased, and affirming of the candidate. The ABRP was the first board to devise a proactive mentoring program that has a credentialed colleague personally guide the applicant through each step of the process. The third major contribution is cosponsorship of the annual Rehabilitation Psychology meeting with the Division of Rehabilitation Psychology that began in 1999. The annual meeting has become an institutionalized opportunity for leaders in the field to meet, present research, and promote the specialty to new students.

Major Activities The major activity of ABRP is cosponsorship of the Annual Conference of Rehabilitation Psychology with Division 22 of the American Psychological Association. This conference occurs the last weekend of February and provides the opportunity to earn continuing education credits. The conference provides ABRP sponsored educational sessions that explain the process of attaining the diplomate in rehabilitation psychology to interested candidates. The conference features nationally recognized leaders in the field of rehabilitation psychology. The ABRP board works in tandem with the American Academy of Rehabilitation Psychology (AARP). The AARP is a separate organization with overlapping board membership with the ABRP board. The AARP contributes the operational support required for organizing the Annual Conference of Rehabilitation Psychology.

Cross References ▶ American Board of Professional Psychology (ABPP) ▶ American Psychological Association (APA), Division 22 ▶ Rehabilitation Psychology

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Frank, R., Rosenthal, M., & Caplan, B. (Eds.). (2009). Handbook of rehabilitation psychology (2nd ed.). Washington, DC: American Psychological Association. Nezu, C., Finch, A., Jr., & Simon, N. (Eds.). (2009). Becoming board certified by the American board of professional psychology. New York, NY: Oxford University Press, Inc.

In addition to the continuing education benefit, ACPN also has an official quarterly journal, Applied Neuropsychology, which is dedicated to the presentation of practitioner-based scholarly research. Diplomates of the ABN who are in good standing are automatically Fellows of ACPN and may use the acronym FACPN on their signature line. Members of other neuropsychological organizations may also join the ACPN as Affiliate members and receive a subscription to Applied Neuropsychology, and participate in ACPN continuing education programs.

American College of Professional Neuropsychology (ACPN) J OHN E. M EYERS Private Practice, Neuropsychology Mililani, Hawaii, USA

Address (and URL) The American College Of Professional Neuropsychology (ACPN) c/o Michael Raymond, Ph.D., ABN Executive Director for ABN John Heinz Institute of Rehabilitation Medicine, Neuropsychology Services 150 Mundy Street Wilkes-Barre. PA 18702 Tel.: (570)826-3771 http://www.neuropsychologyboard.org/

Major Activities ACPN is accredited by the American Psychological Association to sponsor continuing education for psychologists. ACPN has two general meetings a year. One meeting, National Academy of Neuropsychology (NAN) annual conference, a continuing education breakfast, is typically held at the fall. The second yearly meeting is a multiday conference, usually held in the spring. This is a much larger conference with multiple speakers, presentations, and a poster session highlighting recent clinically relevant studies and papers.

Cross References ▶ American Board of Professional Neuropsychology (ABN)

Membership The American College of Professional Neuropsychology (ACPN) is a membership organization formed on September 1, 1995 that is composed of 350 (2009) Neuropsychologists who have doctoral degrees, are licensed as psychologists, and have completed the Diplomate examination process.

American Congress of Rehabilitation Medicine M ARCEL P. J. M. D IJKERS Mount Sinai School of Medicine New York, NY, USA

Membership Major Areas or Mission Statement The academic arm of the American Board of Professional Neuropsychology (ABN) is the ACPN. The mission of the ACPN is to promote and provide the highest levels of services related to professional neuropsychology, for the benefit of the public and the profession.

Membership is about 800, consisting of clinicians and nonclinicians with an interest in medical rehabilitation research, and training in medicine, psychology, occupational and physical therapy, nursing, speech and language pathology, political science, etc. Medical rehabilitation concerns restoration of function for individuals who as a

American Congress of Rehabilitation Medicine

result of stroke, traumatic brain injury, spinal cord injury, amputation, and other disorders have impairments and activity limitations that are primarily physical in nature, but often also include cognitive and behavioral deficits; it is to be distinguished from psychiatric rehabilitation, addictions rehabilitation, etc., although there is overlap in methods and sometimes clientele. Members share an interest in rehabilitation research, and the translation of research-based knowledge into formats that are of use to medical rehabilitation clinicians. About 70 members are located outside the USA, especially in Canada.

Mission Statement ‘‘The mission of the American Congress of Rehabilitation Medicine is to enhance the lives of persons living with disabilities through a multidisciplinary approach to rehabilitation, and to promote rehabilitation research and its application in clinical practice’’ (About ACRM, 2008). ‘‘The American Congress of Rehabilitation Medicine serves people with disabling conditions by promoting rehabilitation research and facilitating information dissemination and the transfer of technology. We value rehabilitation research that promotes health, independence, productivity, and quality of life for people with disabling conditions. We are committed to research that is relevant to consumers, educates providers to deliver best practices, and supports advocacy efforts that ensure adequate public funding for our research endeavors’’ (About ACRM, 2008). ‘‘To develop and implement our vision, ACRM will seek the involvement of rehabilitation professionals, including clinicians, senior level service managers, administrators, educators, and researchers. We will call upon the leaders in rehabilitation to identify current best practices and best providers at all levels of care. We will disseminate this information to the field at our regional and national meetings, through directed position papers, and in our journal, Archives of Physical Medicine and Rehabilitation’’ (About ACRM, 2008).

Landmark Contributions The American Congress of Rehabilitation Medicine was established in 1923 as the American College of Radiology and Physiotherapy, a professional organization of physicians who had a clinical interest in diagnostic and therapeutic radiology, as well as the therapeutic application of electricity and other physical therapies (About ACRM,

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2008). Reflecting the ongoing differentiation between radiologists and what (much later) would be called physiatrists, the name was changed to American Congress of Physical Therapy in 1925. To emphasize its link to medicine rather than allied health, the organization renamed itself American Congress of Physical Medicine in 1944. While World War I had given rise to the development of rehabilitation, the involvement of physicians had been limited – rehabilitation was centered on the vocational rehabilitation of discharged servicemen. During and after World War II, however, a number of physicians became specialists in rehabilitation and started to apply methods they had used with servicemen to the treatment of civilians with amputations, spinal cord injury, stroke, and developmental disabilities such as cerebral palsy. To avoid the creation of a separate organization involving physicians with very similar interests and therapeutic regimens, a ‘‘shotgun marriage’’ between physiatrists and rehabilitation physicians was acknowledged in 1952 with expansion of the name of the organization to American Congress of Physical Medicine and Rehabilitation (Zeiter, 1954). In the 1960s, the Congress opened its membership to nonphysician rehabilitation professionals, first only those holding a doctoral degree (1965), then also to nurses and therapists with an (earned) master’s degree (Anonymous, 1998). To acknowledge the diminishing emphasis on physical medicine, the Congress changed its name again, to American Congress of Rehabilitation Medicine, in 1966. ACRM accepted rehabilitation professionals with a bachelor’s degree as members starting in 1986. The first nonphysician to become president of the organization took office in 1977; neuropsychologists who have served as president include Leonard Diller, Mitchell Rosenthal, and Wayne Gordon. In recent years, ACRM has redefined itself as an organization focusing on rehabilitation science, with strong interest in both generating knowledge through research and knowledge translation to bring research results to the clinic in a format that practitioners can use (Hart, 1997; Heinemann, 2006; Wilkerson, 2004). It now is primarily a group of creators, transmitters, and consumers of research-based rehabilitation knowledge, both those with clinical training (physicians, occupational and physical therapists, psychologists, etc.) and those without (engineers, political scientists, etc.), bound by the conviction that collaboration of disciplines is the best way to solve the problems inherent in disablement and the rehabilitation of persons with impairment, activity limitations, and participation restrictions. The insignia of the organization still reflects ACRM’s roots in physical

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medicine, including the traditional symbols for the four elements: water, earth, fire, and air.

Major Activities ACRM communicates with its members through its scientific journal (the Archives of Physical Medicine and Rehabilitation – APM&R), a newsletter (Rehabilitation Outlook) and weekly E-news, an electronic digest of time-sensitive news. An annual scientific meeting of 3–4 days, often held jointly with other scientific and professional organizations, brings together members and nonmembers to discuss research findings, research methods, and issues relevant to the funding, implementation, and dissemination of rehabilitation research. A number of standing committees offer members an opportunity to work on issues of special interest. Current committees include the International Committee (focusing on the communications between US and foreign rehabilitation research specialists), the Clinical Practice Committee (dealing with issues of evidence-based practice and related matters), and the Involving Consumers in Rehabilitation Research Committee. The Early Career Committee aims to assist individuals new to rehabilitation research in mastering the scientific, administrative, and personal aspects of a career in rehabilitation research. Over the years, a number of interdisciplinary special interest groups (ISIGs) have existed under the aegis of ACRM; current groups include ISIGs focused on spinal cord injury, stroke, the measurement of participation, and traumatic brain injury. The Brain Injury ISIG (BI-ISIG) grew out of the ACRM Head Injury Task Force, first called together in 1979. The BI-ISIG, which attracts large numbers of psychologists and especially neuropsychologists, has played a crucial role in the development of services for individuals with traumatic brain injury (TBI) in the United States. A definition of mild TBI often used in the literature emerged from the work of this group (American-Congress-ofRehabilitation-Medicine.-Head-Injury-InterdisciplinarySpecial-Interest-Group, 1993). The Journal of Head Trauma Rehabilitation (JHTR) was founded by a physician (Sheldon Berrol) and a psychologist (Mitchell Rosenthal) who were active in the BI-ISIG, as well as involved with the fledgling National Head Trauma Foundation, now the Brain Injury Association of America. There is significant overlap between the BI-ISIG membership and both the Editorial Board of JHTR and the leadership of the TBI Model Systems of Care (demonstration and research

grant programs supported by the National Institute on Disability and Rehabilitation Research since 1987). There also is considerable overlap between the membership of the BI-ISIG and Divisions 22 (Rehabilitation Psychology) and 40 (Clinical Neuropsychology) of the American Psychological Association. The BI-ISIG publishes a newsletter, Moving Ahead. Intense collaboration in research and clinical care occurs among the BI-ISIG members, who have their own task forces and come together in an additional annual meeting. APM&R began in 1920 as the Journal of Radiology, the private property of a Dr. Albert A. Tyler (Cole, 1999). The journal changed its name to the Archives of Physical Therapy, X-ray, Radium, in 1926; in 1930, Dr. Tyler gave the journal to ACRM (then still named the American Congress of Physical Therapy) as a ‘‘debt-free, unencumbered gift.’’ The later changes in the name of the journal parallel the changes in the name of its owner. It became the Archives of Physical Therapy in 1938, the Archives of Physical Medicine in 1945; in 1953, the journal became the Archives of Physical Medicine and Rehabilitation, the name it still has (Nelson, 1969). However, the content has shifted gradually from emphasis on physical medicine, with a fairly low research basis, to an accent on rehabilitation as carried out by all disciplines that play a role in medical rehabilitation. It now is almost exclusively a research journal, with non-US contributions constituting over half the contents (Dijkers, 2009). The journal probably gives the best indication of the role of neuropsychology in rehabilitation settings, and of neuropsychologists in ACRM. The first paper with neuropsycholog* in its title or abstract was published in 1975. Almost 200 have been published since, but they did not become an annual presence until 1984. The number now averages ten a year. In scanning the contributions of neuropsychologists to APM&R, a number of characteristics of neuropsychology in rehabilitation stand out:

Many of these papers are coauthored with representatives of other disciplines, especially physicians. Several straddle neuropsychology and rehabilitation psychology, reflecting the fact that in many rehabilitation programs psychologists need to wear multiple hats. The focus, especially in recent years, is as much on treatment as on diagnosis, with cognitive rehabilitation for TBI and other diagnostic groups most prominent. A great variety of diagnostic groups have been studied, including those with peripheral vascular disease amputations, post-polio fatigue, multiple sclerosis,

American Psychological Association (APA)

sickle-cell disease, progressive supranuclear palsy, myotonic muscular dystrophy, and spinal cord injury. However, over the years and especially recently, stroke and TBI have been the etiologies of disability that rehabilitation neuropsychologists have most often been concerned with.

Wilkerson, D. L. (2004). Individual, science, and society: ACRM’s mission and the body politic. Archives of Physical Medicine and Rehabilitation, 85(4), 527–530. Zeiter, W. J. (1954). The history of the American Congress of Physical Medicine and Rehabilitation. Archives of Physical Medicine and Rehabilitation, 35(11), 683–688.

While the American Congress of Rehabilitation Medicine is not an organization of psychologists, let alone neuropsychologists, it is safe to say that it has played a key role in the development of neuropsychology for medical rehabilitation patients in the United States. In the foreseeable future, it probably will continue to be the forum in which these specialists, especially those who are interested in research, interact with nurses, speech/language pathologists, neuroscientists, and other specialties that contribute to rehabilitation.

▶ Weschler’s Adult Reading test


WADE P ICKREN Ryerson University Toronto, ON, Canada

About ACRM. (2008). Retrieved August 25, 2008, from http://www.acrm. org/about/index.cfm American-Congress-of-Rehabilitation-Medicine.-Head-Injury-Interdisciplinary-Special-Interest-Group. (1993). Definition of mild traumatic brain injury. Journal of Head Trauma Rehabilitation, 8(3), 86–87. Anonymous. (1998). Development of the American Congress of Rehabilitation Medicine into a multidisciplinary professional society: Final report of the Professional Development Committee, 1969–1972. Archives of Physical Medicine and Rehabilitation, 79(12 Suppl. 2), 4–12. Cole, T. M. (1999). ACRM presidential address. In the clothing of challenge. American Congress of Rehabilitation Medicine. Archives of Physical Medicine and Rehabilitation, 80(2), 127–129. Dijkers, M. P. (2009). International Collaboration and Communication in Rehabilitation Research. Archives of Physical Medicine and Rehabilitation, 90(5), 711–716. Hart, K. A. (1997). Rehabilitation research: The new focus of the American Congress of Rehabilitation Medicine. Archives of Physical Medicine and Rehabilitation, 78(12), 1287–1289. Heinemann, A. W. (2006). ACRM’s evolving mission: Opportunities to promote rehabilitation research. Archives of Physical Medicine and Rehabilitation, 87(2), 157–159. Kottke, F. J., & Knapp, M. E. (1988). The development of physiatry before 1950. Archives of Physical Medicine and Rehabilitation, 69 Spec No, 4–14. Krusen, F. H. (1969). Historical development in physical medicine and rehabilitation during the last forty years. Walter J. Zeiter Lecture. Archives of Physical Medicine and Rehabilitation, 50(1), 1–5. Nelson, P. A. (1969). History of the Archives – A journal of ideas and ideals. Archives of Physical Medicine and Rehabilitation, 50(7), 367–405. Rusk, H. A. (1969). The growth and development of rehabilitation medicine. Archives of Physical Medicine and Rehabilitation, 50(8), 463–466.

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American National Adult Reading Test (ANART)


Address and URL 750 First Street NE, Washington, DC 20002-4242 (www. apa.org)

Membership 150,000 as of 2010

Major Areas or Mission Statement The mission of the APA is to advance the creation, communication, and application of psychological knowledge to benefit society and improve people’s lives.

Landmark Contributions The American Psychological Association (APA) was founded in 1892 by a small group of men interested in what was called ‘‘the new psychology.’’ Its founding at this particular time can best be understood as part of the large number of changes occurring in the USA at that time. The emergence of a number of what are now standard academic disciplines, psychology, economics, political

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science, biochemistry, physiology, in the last 2 decades of the nineteenth century was part of a reorganization of American knowledge production, reflecting a division of intellectual labor similar to the division of manufactory labor. Like its fellow disciplines, the new psychology grew and prospered as it responded to the needs of American society. Within the modern university system that emerged after the U.S. Civil War, the new disciplines quickly developed advanced degrees that provided credentials, which served to validate the discipline’s members as experts in their special field. This occurred in parallel with the progressive movement in politics, which called for a more efficient, less corrupt, social order. The synergism of these two developments, specialized expertise and rationalized government, helped create the demand for trained personnel to fill the new professional niches created by the demands for a more efficient society. Psychology was one of the most successful of the new disciplines to make itself useful for the social management of an increasingly complex and diversified society. In July 1892, G. Stanley Hall (1844–1924) met with a small group of men to discuss the possibility of organizing a psychological association. Although the details of the meeting are not known, the group elected 31 individuals, including themselves, to membership, with Hall as the first President. The first meeting of the new American Psychological Association (APA) was held in December 1892 at the University of Pennsylvania. The basic governance of the APA at this time was consisted of a small council with an executive committee. This plan remained in effect until the reorganization of APA during World War II. Membership growth of the APA was modest over the first 50 years of its existence. From 31 members in 1892, there were 125 members in 1899, 308 in 1916, 530 in 1930, and 664 in 1940. In 1926, a new class of nonvoting membership was formed, associate, and most of the growth occurred in that class after 1926, so that there were 2,079 associate members in 1940. Many of these associates were individuals doing practical or applied work in psychology and who also belonged to one of the applied associations that emerged in this time. Realizing that the growth of applied psychology represented a potential threat to its preeminence, the leaders of APA sought to reorganize the association during World War II. Under this reorganization plan, the APA merged with other psychological organizations and created divisions to represent special fields of interest. There were initially 17 divisions (19 were proposed). The result was an

association that was much more broadly based than before the War and that was organized around an increasingly diffuse conceptualization of psychology. Now, the association’s scope included professional practice and the promotion of human welfare, as well as the practice of the science of psychology. This flexibility in scope has remained to the present time, as new challenges and demands have arisen. Psychology boomed after the end of World War II, with the greatest increase in membership coming between 1945 and 1970. This was due to intense interest in the field, especially in the domains of clinical and applied psychology, among returning serviceman, many of whom saw the great need for better psychological services firsthand during the war. Institutional or structural factors that facilitated this growth included the GI Bill, the new Veterans Administration Clinical Psychology training program, and the creation of the National Institute of Mental Health. For the first time, psychology was a field, both science and practice, that was richly funded for training and research. This was, as one scholar termed it, The Golden Age of Psychology. The rapid and incredible growth in APA’s membership reflected this trends, as membership grew 630% from 1945 to 1970, from 4,183 members (1945) to 30,839 (1970). By comparison, from 1970 to 2000, APA membership grew to 88,500, with another 70,500 affiliates. Part of what facilitated this growth was the new divisional structure of the APA that grew out of the reorganization plan during World War II. Now, members could join a special interest group within APA and find other like-minded members. Of course, this also facilitated the fractionation of psychology and pushed the field away from any sense of unity that it may have held prior to the war. Nineteen divisions were approved in 1944, with the two most numerous being clinical and personnel (now counseling). This reflected the sectional structure of the American Association of Applied Psychology (AAAP, f. 1937), which had emerged in 1937 as the chief rival to the APA and had been the chief reason for the reorganization. Because the Psychometric Society (Division 4) decided not to join and after Division 11, Abnormal Psychology and Psychotherapy, merged with Division 12, Clinical Psychology, the number of active divisions was reduced to 17. Growth in the number of divisions was slow until the 1960s, only three more were added, in part because many of the older members, then in leadership positions, were quite resistant to increasing the number of divisions. The growth in the number of divisions since the 1960s has been consistent, with 54 divisions now part of the APA structure. Many of the newer divisions reflect the growth


of particular practice areas, for example, Division 50, Addictions. However, there has also been growth in special interest areas that belie any simple science/practice dichotomy, for example, Society for the Psychology of Women, Society for the History of Psychology, International Psychology, Media Psychology, or the Study of Men and Masculinity.

Major Activities The effect on APA governance of the divisional structure and the growth of state and provincial psychological organizations has been marked. As mentioned, prior to World War II, APA’s governance structure was a small council with an executive committee. After the reorganization and the end of the war, the Council of Representatives has grown in number to accommodate representation from each division and from state and provincial psychological associations, thus making governance somewhat unwieldy. Various plans have been tried over the years to ensure a voice for each of the areas and interests groups in psychology on the council and it remains a dynamic situation. One result of the growth of professional psychology, especially clinical and counseling psychology, on governance has been the increase in the representation of professional interests, for example, licensing, specializations, etc., in the deliberations of the council. At times, this has led to tension between the representatives of psychological science and those whose main commitment is to advancing professional practice. In historical retrospect, it seems clear that this tension was inherent in the reorganization of APA, as the association reflected developments in the field. As a membership organization, APA has often been perceived as inadequately representing one or more its constituencies. It has been the case, more often than not, that the resulting tension was resolved and the unhappy parties remained within the association. However, there have also been more serious disagreements that have resulted in new organizations being formed. In the late 1950s, a group of experimental psychologists grew unhappy with what they perceived as APA’s drift from scientific psychology. By the end of 1959, this group formed the Psychonomic Society in order, they asserted, to foster psychology as a science without a need to attend to professional issues. The Psychonomic Society remains a very viable and valuable organization of scientists to the present moment; many of its members remained APA members, as well. A more serious division occurred in

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the mid- to late 1980s, as tensions between those who wanted APA to remain a primarily scientific organization and those who sought a greater emphasis by the association on professional practice rose to a boil. A proposed reorganization plan was defeated by a vote of the membership and almost immediately a large group of dissident psychological scientists, including former APA Presidents, left the APA to form what is now the Association for Psychological Science (APS). Still, after a period of struggle, both organizations are strong, stable representatives of psychology, with many psychologists belonging to both associations. One result of the split that led to the formation of APS is that professional interests have grown stronger within APA. As the number of psychologists devoted to professional practice grew and gained greater influence in the APA governance structure, a new unit was established in the APA Central Office. The Office of Professional Practice was created in the mid-1980s with a mandate to focus on applied practice activities, especially the promotion of health-care practice. To finance the expansion of activities, a special assessment was levied on psychologists licensed for health-care practice. With this money, the office was able to engage in consultation, technical assistance, and legal and legislative assistance for professionals. The office also began to work closely with state associations to enhance practice issues and support efforts relevant to legislation in state legislatures. Within a few years, the range of activities led to the need to create the Practice Directorate within APA. Since that time, the Practice Directorate has played the important roles of handling all practice-related programs and has been responsible for the coordination of practice efforts in legal and legislative arenas. The special assessment and the Practice Directorate represented a special moment in APA’s history in that they enhanced the power of clinical and professional practice both within and without APA. Even so, APA has maintained a commitment to the promotion of psychological science. It publishes more than 40 peer-reviewed scientific journals. Internally, in the APA Central Office, this is represented by the Science Directorate. Since the late 1980s, the Central Office has been reorganized to better represent the diverse constituencies of the membership. Beginning with the formation of the Practice Directorate in the late 1980s, other Directorates were formed in the hope that the interests of all the membership would be better represented. As of 2009, there were the Practice, Education, Science, and Public Interest Directorates. From a historical perspective, it is too soon to determine whether this approach represents

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an advance for the association or a further balkanization of the field. APA remains the world’s largest membership organization of psychologists. It has a fascinating past, marked by growth, conflict, and increasing diversification.

Cross References ▶ Advocacy; Entries 77–86 (excluding 84); Entries 376, 377 ▶ American Psychological Association Division 22 ▶ American Psychological Association Division 40

References and Readings Dewsbury, D. A. (1997). On the evolution of divisions. American Psychologist, 52, 733–741. Evans, R. B., Sexton, V. S., & Cadwallader, T. C. (Eds.). (1992). The American Psychological Association: A historical perspective. Washington, DC: American Psychological Association. Fernberger, S. W. (1932). The American Psychological Association: A historical summary, 1892–1930. Psychological Bulletin, 29, 1–89. Guthrie, R. V. (1998). Even the rat was white: A historical view of psychology. Boston: Allyn and Bacon. Pickren, W. E., & Schneider, S. F. (Eds.). (2005). Psychology and the National Institute of Mental Health: A historical analysis of science, practice, and policy. Washington, DC: APA Books.

American Psychological Association (APA), Division 22 W ILLIAM S TIERS Johns Hopkins University School of Medicine Baltimore, MD, USA

Membership The American Psychological Association (APA) Division 22 – Rehabilitation Psychology is composed of over 1,111 (2009) psychologists who provide clinical services (91%), teach (65%), conduct research (41%), manage rehabilitation programs (37%), and perform other activities too. They work in hospitals and clinics (40%), in university, college, medical school (27%), and other settings, and are also in independent practice (28%).

Major Areas or Mission Statement The Division of Rehabilitation Psychology works to unite psychologists and others interested in the prevention and rehabilitation of disability and chronic illness. Rehabilitation Psychology Practice is a specialty within the domain of professional healthcare psychology, which applies psychological knowledge and skills on behalf of individuals with disabilities and chronic health conditions in order to maximize their health and welfare, independence and choice, functional abilities, and role participation. Such disabilities include spinal cord injury, brain injury, stroke, amputations, burns, work-related injuries, multiple traumatic injuries, chronic pain, cancer, heart disease, multiple sclerosis, neuromuscular disorders, AIDS, developmental disorders, psychiatric impairment, substance abuse, impairments in sensory functioning, and other physical, mental and/or emotional impairments. The broad field of Rehabilitation Psychology also includes rehabilitation program development and administration, research, teaching, public education and development of policies for injury prevention and health promotion, and advocacy for persons with disabilities and chronic health conditions.

Landmark Contributions 1. Rehabilitation psychologists have worked in medical settings as part of teams of healthcare professionals for more than half a century, long before psychologists were regularly involved in other healthcare settings. 2. Division 22 was established in 1958, one of the earlier divisions in APA. 3. Division 22 members conducted the initial research on individual, interpersonal, and social changes related to changes in appearance and physical capacity, as well as the social psychology of stereotyping and prejudice faced by persons with disability. 4. Division 22 members were among the pioneers helping psychology understand the world of work, how the same can be affected by impairment and disability, and issues about vocational rehabilitation. 5. Rehabilitation psychologists have developed the principles of cognitive rehabilitation, and have served as leaders in the federal model systems programs for traumatic brain injury, spinal cord injury, and burns. 6. Board Certification in Rehabilitation Psychology was established in 1997.

American Psychological Association (APA), Division 40

Major Activities The journal Rehabilitation Psychology is published quarterly by the APA. Division 22, in conjunction with the American Board of Rehabilitation Psychology, holds an annual conference in the spring.

Cross References ▶ American Psychological Association (APA) ▶ Rehabilitation Psychology

References and Readings American Psychological Association. (2008). A closer look at Division 22: A growing field meets the challenges of war. Monitor on Psychology, 38(8), 54–55. Frank, R., Rosenthal, M., & Caplan, B. (Eds.). (2009). Handbook of rehabilitation psychology (2nd ed.). Washington, DC: American Psychological Association. Larson, P., & Sachs, P. (2000). A history of Division 22. In D. A. Dewsbury (Ed.), Unification through division: Histories of the divisions of the American Psychological Association (Vol. 5, pp. 33–58). Washington, DC: American Psychological Association.

American Psychological Association (APA), Division 40 W ILLIAM B. B ARR New York University School of Medicine New York, USA

Membership The Division of Clinical Neuropsychology (Division 40) is one of 56 specialty divisions recognized by the American Psychological Association (APA). Since its inception, it has become one of APA’s largest and most active divisions. In its nearly 30 years, membership has grown from 433 psychologists to its current numbership of 5,315, which currently makes it the second largest of all APA divisions behind only the Independent Practice Division (Division 42). The division’s representation to the APA council has grown over

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the years from its initial one representative to the current allotment of four seats. This trend coincides with Division 40’s increasing influence within APA and increasing recognition of neuropsychology as a clinical specialty. Eligibility for membership is based on the criteria required for Associate, Member, or Fellow status in the APA. Additional requirements include demonstrated interest in the field of neuropsychology and its scientific development, public dissemination, and/or clinical applications. All members of the division have rights and privileges to hold office and serve on division committees, vote in regular elections, attend various meetings of the division, and receive publications of the division. Information for joining Division 40 can be obtained on the division’s website at http://www.div40.org/membership.html. APA statistics indicate that the majority of Division 40 members are women (55%). Ethnic minority members constitute 8% of the membership, consistent with larger APA trends. Approximately, 80% of the division memberships have Ph.D. in clinical psychology or a related field. Nearly half (42%) of the members work in independent settings. Most other members work in medical schools, hospitals, and university settings. Many combine their work in institutional and private-practice settings. Membership surveys have indicated that psychologists in Division 40 spend a substantially larger amount of time (>40%) in assessment activities than other APA members (6 cm in diameter) may require endovascular embolization as an adjunct to surgical intervention (Zhao et al., 2005).

Mechanisms Cognitive improvements have been attributed to improved cerebral blood flow and reduction of hypoperfusion (Lantz & Meyers, 2008). Surgery may result in severe neuropsychological complications for some patients, including executive dysfunction and aphasia (Lantz & Meyers, 2008; Zhao et al., 2005).

Neuropsychological and Psychological Outcomes The actual occurrence of cognitive deficits in AVM is difficult to determine (Lantz & Meyers, 2008) because much of the data have been pooled from patients with ruptured and unruptured AVMs. Patients with AVM may exhibit below normal performance on tests of intelligence, attention, and memory (Lantz & Meyers, 2008). Some research has demonstrated postsurgical improvement in patient’s neuropsychological functioning, including better performance on tasks requiring executive function (Lantz & Meyers, 2008). Recently, researchers have demonstrated brain reorganization of language function in patients with AVM by using selective Wada testing (intracarotid amobarbital sodium and xylocaine procedure), magnetic resonance imaging, and functional magnetic resonance imaging (Lantz & Meyers, 2008). Other research has demonstrated structural reorganization involving the motor cortex (Lantz & Meyers, 2008). Patients with AVM are more likely to report developmental learning disorders than patients with tumors or aneurysms, with AVM patients reporting four times the rate of learning disability compared to the normal population (Lantz & Meyers, 2008; Lazar et al., 1999). This finding may suggest that disorders of learning and intellectual function may serve as a marker for early cerebral dysfunction in patients with AVMs (Lazar et al., 1999).

Cross References ▶ Anterior Communicating Artery ▶ Gamma Knife ▶ Intracarotid Sodium Amytal Test ▶ Radiosurgery, Stereotactic Radiation Therapy ▶ Shunts ▶ Wada Test

References and Readings Al-Shahi, R., & Warlow, C. (2001). A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain, 124, 1900–1926. Aminoff, M. J., Greenberg, D. A. & Simon, R. P. (2005). Clinical neurology. New York: McGraw-Hill. Frosch, M. P., Anthony, D. C., & De Girolami, U. (2005). The central nervous system. In V. Kumar, A. K. Abbas, & N. Fausto (Eds.), Pathologic basis of disease (pp. 1347–1420). Philadelphia: Elsevier. Lantz, E. R., & Meyers, P. M. (2008). Neuropsychological effects of brain arteriovenous malformations. Neuropsychology Review, 18, 167–177. Lazar, R. M., Connaire, K., Marshall, R. S., Pile-Spellman, J., Hacein-Bey, L., Solomon, R. A., et al. (1999). Developmental deficits in adult patients with arteriovenous malformations. Archives of Neurology, 56, 103–106.

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Moftakhar, P., Hauptman, J. S., Malkasian, D., & Martin, N. A. (2009). Cerebral arteriovenous malformations. Part 1: cellular and molecular biology. Neurosurgical Focus, 26(5), 1–15. Ropper, A. H., Brown, R. H., Adams, R. D., & Victor, M. (2005). Adams & Victor’s principles of neurology. New York: McGraw-Hill. Stein, B. M., & Wolpertson, S. M. (1980). Arteriovenous malformations of the brain. I: Current concepts and treatments. Archives of Neurology, 37, 69–75. Warlow, C. (2001). Stroke, transient ischemic attacks, and intracranial venous thrombosis. In M. Donaghy (Ed.), Brain’s diseases of the nervous system (pp. 775–896). New York: Oxford University Press. Webster’s new explorer medical dictionary (New Ed.). (2006). Springfield, MA: Merriam-Webster. Zhao, J., Wang, S., Li, J., Qi, W., Sui, D., & Zhao, Y. (2005). Clinical characteristics and surgical results of patients with cerebral arteriovenous malformations. Surgical Neurology, 63, 156–161.

Arteriovenous Malformations

Cross References ▶ Apraxia of Speech ▶ Articulation Disorder ▶ Ataxia ▶ Dysarthria ▶ Dystonia ▶ Phoneme ▶ Phonics ▶ Speech-Language Pathology

References and Reading Hulit, L. M., & Howard, M. R. (2005). Born to talk: an introduction to speech and language development (4th ed.). Boston: Pearson A & B. Plante, E., & Beeson, P. M. (2008). Communication and communication disorders: a clinical introduction (3rd ed.). Boston: Pearson A&B.

▶ Vascular Malformations

Articulation Disorders Arteritis ▶ Vasculitis

Articulation J ANET PATTERSON California State University East Bay Hayward, CA, USA

Definition Articulation is (1) the juncture between bones or cartilages in the skeleton of a vertebrate; (2) the movement pattern and relationship of oral structures such as the tongue and lips, to produce the sounds of speech. Articulation develops gradually and consistently across children of all cultures, and the earliest sounds made by infants are undifferentiated. As a child matures and motor control becomes increasingly well-coordinated, the child’s speech becomes intelligible within the linguistic community. Articulation is evaluated through tests of single sounds and words, and in contexts such as oral reading and conversation. Articulation disorders can disrupt speech intelligibility temporarily or for extended periods of time.

PAMELA G. G ARN -N UNN University of Akron Akron, OH, USA

Short Description or Definition An articulation disorder is a failure to acquire a speech sound or sounds of a particular language by the expected normative age due to some type of motoric problem. Speech sound errors in articulation disorders include Substitutions: replacing a standard speech sound with a different standard speech sound e.g., Wabbit for rabbit, thpoon for spoon, or bery for very. Omission: omission of a standard speech sound e.g., ba for bat, gin for green. (Widespread omissions often indicate a phonological disorder, however.) Distortions: replacement of a standard speech sound by a nonstandard sound e.g., s in soup sounds ‘‘slushy.’’ Additions: addition of a sound or syllable e.g., cart for car or chiminey for chimney. Additions are the least commonly occurring type of articulation error. While some of these sound changes are common for toddlers and early preschoolers, children should master the speech sounds of English by the age of 8. NOTE: However, individuals whose sound substitutions or omissions reflect a dialectic variation or acquisition of

Articulation Disorders

English as a second language are not considered to have an articulation disorder.

Categorization

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Articulation Disorders. Table 1 By age 3: p m h w b (emerging between ages 1½ and 3) By age 3 ½:

k g d t f y (girls only) (emerging between ages 2 and 3 ½)

By age 4: y (boys)

An articulation disorder is a type of speech sound disorder. It is associated with a motoric inability to produce a speech sound or sounds (rather than a failure to acquire the speech sound rules of a particular language) by the expected normative age. (▶ Phonological Disorder.) Articulation and phonological disorders can co-occur.

Epidemiology Incidence figures are available for speech sound disorders, of which articulation disorders are one type. Figures cited for preschoolers are 8–9% with approximately 5% still demonstrating a speech sound disorder by first grade. Incidence of speech sound disorders in children is higher than in adults. Some children will outgrow their errors, while others will require treatment from a speech-language pathologist to develop understandable speech (see Evaluation). During the process of speech development, articulation disorders occur more often in children with

Genetic syndromes such as Down syndrome or other syndromes associated with cognitive delays Childhood apraxia of speech Neurological disorders such as cerebral palsy Orofacial anomalies such as cleft palate Myofunctional disorders (sometimes referred to as tongue thrust disorders)

In some cases, no definitive etiological factor will be found. For school-aged children, articulation disorders can be a continuation of an earlier phonological or articulation problem or the result of some type of neurological injury. Similarly, in adults, a speech sound disorder can consist of residual errors of an earlier disorder or a new disorder due to a variety of neurological causes. ▶ Dysarthria and ▶ apraxia for further information on these adult causes of speech sound disorders.

Natural History, Prognostic Factors, Outcomes A child’s acquisition of the speech sounds is a gradual process. Correct articulation can depend not only on

By age 5: s-blends (emerging between ages 3½ and 5) By age 5 ½:

v

By age 6: sh ch j (girls) By age 7: sh ch j (boys) th (as in that) By age 8: r s ng l z th (as in thumb) zh (as in measure)

motor skills and perceptual development but also the sound make-up and length of a word. Nevertheless, research indicates that the following sounds should be mastered by the ages indicated:

Vowels: should all be acquired by age 3 with the exception of the er sound in words like bird and hammer. Consonants: these represent ages of mastery; prior to these ages, correct production will vary

For children who do not meet these milestones, testing and possible treatment by a certified speech-language pathologist is required. A person who continues to exhibit articulation errors past age 8 should also be evaluated unless their sound usage is characteristic of a dialect or first language.

Neuropsychology and Psychology of Disorder In some cases, an articulation disorder may be associated with damage to the central or peripheral nervous system. Specific neurological correlates usually are not found except in cases of dysarthria or apraxia. When misunderstood, some children may react by refusing to speak or withdrawing from others. Children who are unable to communicate due to an articulation disorder can become frustrated and act out because they cannot make basic needs and wants known. The child’s family can also become frustrated at their inability to communicate with their child. However, this type of behavior is much more likely to occur in conjunction with phonological disorders rather than articulation disorders. For adults with speech sound disorders due to apraxia or dysarthria, the effect on communication will depend on the number of sounds affected, the degree of speech understandability, and the patient’s reaction to the communication problem.

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Evaluation Evaluation of articulation disorders is designed to determine: 1. Existence of a problem 2. Nature of the problem (sounds in error, patterns intelligibility) 3. Possible etiology(ies) of the problem, e.g., structural problem or neurological disorder 4. Probable course of treatment 5. Prognosis To meet these goals, the following components should be included in an evaluation for speech sound disorders: 1. 2. 3. 4. 5. 6.

Case history Hearing screening Oral mechanism evaluation Phonemic sound-by-position tests Language testing Other tests as appropriate

Treatment For patients with simple articulation disorders, a traditional, phonetic approach can be successful. ▶ Phonological Disorders and ▶ Articulation Disorders for more information on treatment.

ASEBA ▶ Child Behavior Checklist

ASHA-FACS ▶ American Speech-Language-Hearing Association Functional Assessment of Communication Skills for Adults

Ashworth Spasticity Scale (and Modified Version) K ARI D UNNING University of Cincinnati Cincinnati, OH, USA

Synonyms AS; MAS

Description Cross References ▶ Apraxia ▶ Articulation Disorder ▶ Dysarthria ▶ Phonological Disorder ▶ Phonology

References and Readings American Speech-Language-Hearing Association. (2007). Speech sound disorders: Articulation and phonological disorders, from www.asha. org/public/speech/disorders/SpeechSoundDisorders. American Speech-Language-Hearing Association. (2008). Incidence and prevalence of communication disorders and hearing loss in children (2008 Ed.), from www.asha.org/members/research/reports/children.

AS ▶ Ashworth Spasticity Scale (and Modified Version)

The Ashworth Scale (AS) and Modified Ashworth Scale (MAS) measure spasticity. During the administration of both AS (Ashworth, 1964) and MAS (Bohannon & Smith, 1987), the examiner passively moves the joint being tested and rates the perceived level of resistance in the muscle groups opposing the movement. Both scales are singleitem measures ranging from 0 to 4, where 0 indicates no increase in muscle tone and 4 indicates that the affected part is rigid in flexion or extension. The AS is considered an ordinal scale, whereas the MAS is considered a nominal scale due to ambiguity created by the addition of the 1þ grade between 1 and 2 (Pandyan, Johnson, Price, Curless, Barnes, & Rodgers, 1999).

Historical Background The AS was first described by Ashworth in 1964 (Ashworth, 1964) and was subsequently modified with the addition of a 1þ grade by Bohannon in 1987 with the intent to increase sensitivity (Bohannon & Smith, 1987). However,

ASIA Impairment Scale

this addition may have decreased the reliability of the MAS for heavier limbs (see below) (Ansari, Naghdi, Arab, & Jalaie, 2008; Pandyan et al., 1999; Platz, Eickhof, Nuyens, & Vuadens, 2005).

Psychometric Data Inter- and intra-rater reliability of the AS and MAS show wide variation (Pandyan et al., 1999; Platz et al., 2005), which cannot be attributed to any one factor (Platz et al., 2005), although some evidence suggests that the interrater reliability of the MAS is lower for heavier limbs (Ansari et al., 2008; Pandyan et al., 1999; Platz et al., 2005). Both scales have demonstrated responsiveness to treatment (Platz et al., 2005). Spasticity is characterized by an involuntary muscle activity (Pandyan et al., 2005) and has been traditionally defined as a velocity-dependent increase in muscle tone due to a hyperactive stretch reflex (Lance, 1980). The construct validity of the AS and MAS as spasticity assessments is inadequate because they do not address velocity dependence. Rather, these scales measure passive resistance to movement (hypertonia), which is influenced by spasticity but also altered by biomechanical factors unrelated to involuntary muscle activation (Fleuren, Voerman, & Erren-Wolters, 2009; Pandyan et al., 1999; Platz et al., 2005). Thus, AS and MAS scores are only moderately associated with reflexes (Platz et al., 2005) and electromyographic assessments (Fleuren et al., 2009; Pandyan et al., 1999; Platz et al., 2005) and more strongly associated with objective measures of resistance (Fleuren et al., 2009; Pandyan et al., 1999; Platz et al., 2005).

Clinical Uses

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References and Readings Ansari, N. N., Naghdi, S., Arab, T. K., & Jalaie, S. (2008). The interrater and intrarater reliability of the Modified Ashworth Scale in the assessment of muscle spasticity: Limb and muscle group effect. NeuroRehabilitation, 23(3), 231–237. Ashworth, B. (1964). Preliminary trial of carisoprodol in multiple sclerosis. Practitioner, 192, 540–542. Bohannon, R. W., & Smith, M. B. (1987). Interrater reliability of a modified Ashworth scale of muscle spasticity. Physical Therapy, 67(2), 206–207. Fleuren, J. F., Voerman, G. E., Erren-Wolters, C. V., et al. (2009). Stop using the Ashworth Scale for the assessment of spasticity. Journal of Neurology, Neurosurgery, and Psychiatry, 81(2), 46–52. Lance, J. W. (1980). Symposium synopsis. In R. G. Feldman, R. R. Young, & W. P. Koella (Eds.), Spasticity: Disordered motor control (pp. 485–494). Chicago, IL: Year Book Medical Publishers. Pandyan, A. D., Gregoric, M., Barnes, M. P., et al. (2005). Spasticity: Clinical perceptions, neurological realities and meaningful measurement. Disability and Rehabilitation, 27(1–2), 2–6. Pandyan, A., Johnson, G., Price, C., Curless, R., Barnes, M., & Rodgers, H. (1999). A review of the properties and limitations of the Ashworth and modified Ashworth Scales as measures of spasticity. Clinical Rehabilitation, 13(5), 373–383. Platz, T., Eickhof, C., Nuyens, G., & Vuadens, P. (2005). Clinical scales for the assessment of spasticity, associated phenomena, and function: A systematic review of the literature. Disability and Rehabilitation, 27(1–2), 7–18.

ASIA (American Spinal Injury Association) Exam ▶ ASIA Impairment Scale


Despite the fact that the AS and MAS are actually only valid assessments of hypertonia (Fleuren et al., 2009; Pandyan et al., 1999; Platz et al., 2005), these scales are the most commonly used clinical tools to assess spasticity (Pandyan et al., 1999; Platz et al., 2005). Both scales have been used to describe treatment response for persons with a wide range of upper motor neuron disorders, including traumatic brain injury, stroke, multiple sclerosis, cerebral palsy, and spinal cord injury (Platz et al., 2005).

A MITABH J HA Craig Hospital Englewood, CO, USA

Cross References

Description

▶ Severe Brain Injury ▶ Spinal Cord Injury

The International Standards for Neurological Classification of SCI (ISNCSCI) is a widely accepted and readily

Synonyms ASIA (American Spinal Injury Association) exam; Frankel scale; International standards for the neurological classification of spinal cord injury

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administered guide to document neurological function after spinal cord injury (SCI) and is intended to be a standard for measuring neurological outcomes in both clinical and research settings. Briefly, these standards utilize a two-step process consisting of a specific neurological examination followed by a classification procedure based on the results of the exam. The systematic neurological examination assesses sensory and motor function of each spinal segmental level. Sensation of light touch and pinprick (PP) stimuli is scored as 0 for absent, 1 for impaired, and 2 for normal. Motor function is scored on a scale of 0 for total paralysis to 5 for normal strength. All 28 dermatomes are tested bilaterally for sensory function, and ten key muscles are tested bilaterally for motor function, yielding sensory/light touch (LT) and sensory/pinprick (PP) summed scores ranging from 0 to 112 and motor summed scores ranging from 0 to 100. The neurological level is assigned as the lowest level with normal neurological function. In

addition, the ASIA Impairment Scale (AIS) grade classification, an indicator of injury ‘‘completeness’’ similar to the Frankel scale, is assigned based on this information. AIS A denotes a complete injury with no sensory or motor function below the level of injury. Incomplete injuries are graded as AIS B if there is sensory but no motor function below the injury level, AIS C if there is some motor sparing, AIS D for substantial motor sparing, and AIS E for normal neurological examination (Fig. 1).

Historical Background ISNCSCI has been used extensively in clinical practice and research since 1982. The standards and accompanying reference manual have undergone sequential revisions, most recently in 2000 and 2003, respectively.

Patient Name_ ___________________________________ Examiner Name _________________________________ Date/Timeof Exam ___________________

STANDARD NEUROLOGICAL CLASSIFICATION OF SPINAL CORD INJURY (scoring on reverse side)

(distal phalanx of middle finger) (little finger)

Comments:

REV 03/06

ASIA Impairment Scale. Figure 1 International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI)

Asomatognosia

Psychometric Data Published studies have found total motor score ICCs from 0.98 to 0.99 for intra-rater reliability and 0.97 for interrater reliability. Total sensory scores intra-rater reliability has ranged from 0.76 to 0.98, and 0.88 to 0.96 for interrater. One study reported agreement on individual muscles with Kappas ranging from 0.3 to 0.89 for each myotome, 0.02 to 0.83 for dermatome assessment using pinprick, and 0.17 to 1 when assessed using light touch.

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Asomatognosia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Synonyms Disturbance of body schema

Clinical Uses

Definition

The exam is used to document sensory and motor function after SCI. It has been used to diagnose SCI, as an outcome measure in studies to treat spinal cord pathology, as well as a tool to predict outcomes such as independence with activities of daily living, employment, life satisfaction, and life expectancy.

Disturbance in the normal awareness of one’s own body, typically characterized by one or more of the following symptoms: (1) a tendency to ignore or neglect one side of the body, (2) a failure to recognize or difficulty in identifying a specific part of the body (usually a limb or part of a limb), (3) difficulty in differentiating the right from the left side of the body, or (4) recognizing an impairment in a part of the body (anosognosia).

Cross References ▶ Sensorimotor Assessment ▶ Spinal Cord Injury

References and Reading American Spinal Injury Association. (2002). International standards for neurological classification of spinal cord injury (Revised 2002). Chicago: American Spinal Injury Association. American Spinal Injury Association. (2003). Reference manual for the international standards for neurological classification of spinal cord injury (Revised 2003). Chicago: American Spinal Injury Association. Furlan, J. C., Fehlings, M. G., Tator, C. H., & Davis, A. M. (2008). Motor and sensory assessment of patients in clinical trials for pharmacological therapy of acute spinal cord injury: Psychometric properties of the ASIA standards. Journal of Neurotrauma, 25(11), 1273–1301. Kirshblum, S. C., Memmo, P., Kim, N., Campagnolo D., & Millis, S. (2002). Comparison of the revised 2000 American spinal injury association classification standards with the 1996 guidelines. American Journal of Physical Medicine & Rehabilitation, 81(7), 502–505. Mulcahey, M. J., Gaughan, J., Betz, R. R., & Johansen, K. J. (2007). The international standards for neurological classification of spinal cord injury: Reliability of data when applied to children and youths. Spinal Cord, 45(6), 452–459.

Current Knowledge Asomatognosia most commonly results from acute or subacute brain lesions and may affect one or both sides of the body. Unilateral neglect generally involves an entire side of the body, more commonly the left. This might be reflected in a failure to shave the affected side of the face, putting a glove only on one hand, or reduced use of the involved limb for certain activities, even though it is physically capable of doing so. If a limb is paralyzed, the patient may either deny or minimize the impairment (anosognosia), or may even deny ownership of the affected limb. If the affected side or a part of the body is stimulated, the individual may report that the homologous area on the intact side was touched (allesthesia). Patients may also have difficulty localizing or identifying parts of their own body (autotopagnosia). This is most commonly expressed as difficulty naming or identifying individual fingers (especially the three middle digits) either of their own hands or those of others (finger agnosia). This deficit is usually expressed bilaterally. Right–left disorientation is generally also considered a form of asomatognosia. Here, the individual has difficulty reliably identifying the right

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and left sides of his or her own body or those of the examiner. Although asomatognosia strictly refers to impaired awareness or attention to parts of one’s own body, personal neglect often extends into extrapersonal space. Thus, a patient may fail to attend to visual or auditory stimuli on the affected side, despite intact visual fields or the fact that auditory stimuli enter both ears. This can be very disconcerting for family members if they are not made aware of these phenomena, perhaps believing the patient is purposely ignoring their presence. Unilateral neglect or anosognosia type disorders are most commonly found, following acute lesions (such as strokes) of the right hemisphere. Although improvement is typically seen over time, subtle degrees of deficit may persist indefinitely. By contrast, those deficits that present bilaterally (such as finger agnosia and right–left disorientation) are usually the result of posterior left-hemispheric lesions.

Cross References ▶ Allesthesia ▶ Anosognosia ▶ Autotopagnosia ▶ Finger Agnosia ▶ Right–Left Disorientation

Asperger’s Disorder S TEPHEN M. K ANNE , M ICAH O. M AZUREK Thompson Center for Autism and Neurodevelopmental Disorders Columbia, MO, USA

Synonyms Asperger syndrome

Short Description or Definition Asperger’s disorder is a neurodevelopmental disorder that is associated with impairment in social relatedness and repetitive or restricted behaviors and interests. Social difficulties that are characteristic of Asperger’s disorder include nonverbal aspects of social interaction (e.g., eye contact, gestures, and facial expressions) as well as social and emotional reciprocity (e.g., sharing interests, taking turns, demonstrating empathy). Behaviorally, individuals with Asperger’s often exhibit intense and narrow circumscribed interests, insistence on sameness or routine, and behavioral rigidity (American Psychiatric Association, 1994). While overall level of intellectual functioning (i.e., IQ) is not impaired in individuals with Asperger’s disorder, their cognition is often compromised in other areas such as executive functioning (see Neuropsychology and Psychology of Asperger’s Disorder below).

References and Readings Hecaen, H., & Albert, M. L. (1978). Human neuropsychology (pp. 303– 330). New York: Wiley. Heilman, K. M., Watson, R. T., & Valenstein, E. (2003). Neglect and related disorders. In K. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (pp. 296–346). New York: Oxford University Press. Kortte, K. B., & Wegener, S. T. (2004). Denial of illness in medical rehabilitation populations: Theory, research and definitions. Rehabilitation Psychology, 49, 187–199. Prigatano, G. P., & Schacter, D. L. (1991). Awareness of deficit after brain injury: Clinical and theoretical issues. New York: Oxford University Press.

Asperger Syndrome ▶ Asperger’s Disorder

Categorization Asperger’s disorder is currently classified in the DSM-IV as one of five separate pervasive developmental disorders (which also include autistic disorder, Rett’s disorder, childhood disintegrative disorder, and pervasive developmental disorder NOS). Asperger’s disorder is a fairly recent addition to the DSM, first appearing only in the latest version, the DSM-IV (1994). According to the DSM-IV, to meet criteria for Asperger’s disorder, an individual must demonstrate impairment in social interaction (exhibiting at least two out of four possible symptoms) and restricted and repetitive patterns of behaviors or interests (exhibiting at least one out of four possible symptoms). In addition, an individual must not have a history of developmental delays in language, cognition, or adaptive functioning. Of note, the criteria

Asperger’s Disorder

for Asperger’s disorder are identical to those for autistic disorder (i.e., autism) in the areas of social impairment and restricted and repetitive behavior. However, autistic disorder requires an additional criterion of impairment in communication (i.e., delays in language development, impairment in conversation, stereotyped language, or lack of pretend play). Additionally, there is not a requirement in the criteria for autistic disorder that cognitive, language, and adaptive development fall within the normal range in childhood (as is the case in Asperger’s disorder) (American Psychiatric Association, 1994).

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Prevalence estimates have varied widely from 0.3/1,000 to 6/1,000 (see Mattila et al., 2007 for review). Based on a review of the literature, Fombonne (2003, 2005) estimated the prevalence rate for Asperger’s disorder to be approximately 2/10,000. Such wide variations in prevalence rates are likely due to differences in diagnostic procedures and operational definitions used in each study. In fact, recent rates from the same study varied from 1.6/1,000 to 2.9/1,000 depending on the specific criteria used for diagnosis (Mattila et al., 2007). In terms of sex differences, males are overrepresented in Asperger’s disorders, with an estimated sex ratio of 4:1 (see Schopler, Mesibov, & Kunce, 1998 for review).

a result, Wing (1981) published an influential paper reintroducing Asperger’s original ideas and arguing for broadening the definition of autism to include Asperger’s disorder on the autism continuum. Eventually, a separate diagnosis of Asperger’s disorder was added to the fourth edition of the DSM (1994). Since that time, debate has continued as to whether or not Asperger’s disorder should remain a separate diagnosis from autism. The prevailing current view is that Asperger’s disorder and autism are not distinctly different, and that Asperger’s disorder may simply represent the milder end of the autism spectrum. As a result, Asperger’s disorder is often used synonymously with the term ‘‘high-functioning autism’’ (which typically refers to individuals meeting criteria for Autistic Disorder whose IQ levels are above 70). With regard to developmental course, Asperger’s disorder is generally diagnosed much later than autistic disorder, with an average age of diagnosis being 11 years (possibly due to the lack of early developmental delays). It follows a continuous course throughout the lifespan, although for some individuals symptoms remit as a result of early intervention (see Frith, 2004 for review). Research into prognostic factors and outcome in Asperger’s disorder is sparse, particularly since it has only been recognized as an official diagnosis for little over a decade; however, IQ and language ability have been found to be strong predictors of outcomes in autism spectrum disorders in general.

Natural History, Prognostic Factors, Outcomes

Neuropsychology and Psychology of Asperger’s Disorder

Asperger’s disorder takes its name from the Austrian physician, Hans Asperger, whose 1944 paper on ‘‘autistic psychopathy’’ described a group of children who showed deficits in social behaviors, insistence on sameness, a lack of nonverbal communication, repetitive movements, and average intelligence. Asperger (in 1944) and Leo Kanner (in 1943), although unaware of one another’s work, were the first to describe this cluster of symptoms. While Leo Kanner’s seminal work describing autistic behaviors was the subject of much discussion and resulted in the eventual inclusion of autism in the DSM (in 1980), Asperger’s paper did not receive wide attention after publication and was not translated into English until 1991 (see Frith, 1991). After the appearance of autism in the DSM-III, it became apparent that there was a group of individuals who did not meet the criteria for the narrowly defined definition of infantile autism, but who demonstrated deficits in social interaction and repetitive behaviors. As

By definition, social interactions are impaired in Asperger’s disorder. However, the underlying processes by which social interactions are disrupted have been the source of recent attention. First, there is clear evidence that individuals with Asperger’s disorder have impairments in their ability to understand complex emotions and a resulting inability to recognize and empathize with others’ feelings. Individuals with Asperger’s disorder (as well as autistic disorder), also have impairments in what is known as ‘‘theory of mind.’’ As such, they have difficulty automatically attributing mental states to others. Although there are no formal criteria concerning communication skills for Asperger’s disorder, clinical and research accounts highlight the presence of social communication difficulties. Specifically, difficulties with pragmatic language and difficulties with turn-taking in conversation are common (see Klin, Volkmar, & Sparrow, 2000 for review).

Epidemiology

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Asperger’s Disorder. Table 1 DSM-IV criteria for Asperger’s disorder Social impairment (2 or more)

Restricted and repetitive behavior (1 or more)

Lack of delays in

1. Impaired nonverbal behavior

1. Abnormal and intense preoccupation with stereotyped or restricted interest

Language

2. Impaired peer relationships

2. Inflexible and nonfunctional routines or rituals

Cognitive development

3. Lack of seeking to share enjoyment, interests, or achievements

3. Stereotyped and repetitive motor mannerisms

Self-help skills

4. Lack of social or emotional reciprocity

4. Preoccupation with parts of objects

Adaptive behavior (other than social) Curiosity about the environment

Source: From Diagnostic and Statistical Manual of Mental Disorders, fourth edition, by American Psychiatric Association, 1994, Washington, DC

Asperger’s disorder is also associated with cognitive features that affect functioning outside the social domain. Studies have shown that individuals with Asperger’s disorder have very uneven cognitive profiles. One explanation for this common finding is that these individuals have ‘‘weak central coherence.’’ That is, they are more likely to process information as discrete units rather than processing them as a unified whole. There is some evidence that bottom-up processing occurs without accompanying top-down control. As a result, high levels of details are perceived, while global information may be missed (see Frith, 2004). Studies have also shown consistent deficits in overall executive function among individuals with Asperger’s disorder (as is also the case in autistic disorder). Specifically, poor performance has been shown on both the Wisconsin Card Sorting Test and the Tower of Hanoi. Particular deficits have been noted in the ability to shift response set and in overall planning. Consistent with this, individuals with Asperger’s disorder are often described as having difficulty adjusting to changes in routine or task demands, and as having a strong need for sameness (see Klin et al., 2000). Some studies, including Wing’s (1981) original description, have found significantly higher verbal IQ scores than performance IQ scores among individuals with Asperger’s disorder (the reverse of which is typically found in autistic disorder). Motor clumsiness has also been observed among children with Asperger’s disorder since Hans Asperger’s original paper, although it has never been a part of the formal diagnostic criteria (see Frith, 1991). As a result, researchers have been interested in potential similarities between Asperger’s disorder and nonverbal learning disorders (NVLD) or right hemispheric dysfunction. These profiles are marked by relative strengths at rote verbal skills, with deficits in social understanding and motor coordination. While there is a

great deal of overlap among these conditions, empirical findings have been equivocal. Some studies have found visual-spatial impairments (with strengths in Verbal IQ) in Asperger’s disorder, while others have not demonstrated this pattern (see Klin et al., 2000). Further work with more stringent diagnostic criteria is needed in this area.

Coexisting Conditions In addition to the core symptoms, Asperger’s disorder may also be accompanied by co-occurring disorders. Studies have shown that a large percentage of children with Asperger’s disorder also exhibit problems with attention and impulse control (similar to those found in ADHD). However, the DSM-IV prevents an additional diagnosis of ADHD when there is an existing pervasive developmental disorder diagnosis. In adolescence and adulthood, case studies indicate relatively high rates of depression, anxiety, and bipolar disorder among individuals with Asperger’s disorder (see Ghaziuddin, 2002 for review). Recent evidence has also demonstrated that individuals with Asperger disorder have significant adaptive impairments as well (see Saulnier & Kim, 2007).

Evaluation Diagnostic assessment of Asperger’s disorder is best conducted using multiple methods and observers. Due to the complexity of the disorder, and its effects on broad areas of functioning, interdisciplinary assessment is recommended. First, because Asperger’s is a neurodevelopmental disorder, parent report of early history and development, as well as structured observations of current behavior, are essential. Currently, the two ‘‘gold-standard’’


tools for diagnosis of autism spectrum disorders are the Autism Diagnostic Interview – Revised (ADI-R) and the Autism Directed Observation Schedule (ADOS). These are the most widely studied measures in the field, and reliability and validity have been well established. The ADI-R is a comprehensive interview that assesses past and current functioning in the areas of communication, social interaction, and restricted or repetitive behavior. The Autism Directed Observation Schedule (ADOS) is another diagnostic tool that allows for clinic-based observations across various structured activity- and conversationbased interactions. Despite their advantages, however, neither tool was designed to measure Asperger’s disorder specifically, or to differentiate between Asperger’s disorder and other PDDs. A number of other scales have been developed to assess Asperger’s disorder, but systematic research is lacking as of yet (see Matson & Boisjoli, 2008; Mesibov, Shea, & Adams, 2001 for review). In addition to assessing the core symptoms of Asperger’s disorder, assessment should focus on cognitive, adaptive, and communication skills. General measures of intelligence, such as the Wechsler Scales and the Stanford Binet Intelligence Scales are useful in assessing overall functioning as well as particular strengths and weaknesses. Additionally, it is helpful to include measures of visualspatial and visual-motor processing, particularly since these areas are typically weaker in Asperger’s disorder. Given common deficits in executive function and attention, neuropsychological assessment of these functions is recommended. Measures of social communication and pragmatic language, and adaptive skills, also add information to the clinical picture and help inform intervention recommendations (see Klin et al., 2000 for review).

Treatment There is, as of yet, no available treatment that provides a ‘‘cure’’ for the core impairments of Asperger’s disorder. However, there are a number of interventions that target specific symptoms. In addressing social deficits in Asperger’s disorder, there have been several promising studies of social competence interventions among children and adolescents with Asperger’s disorder and autism. Such interventions can be delivered in educational or outpatient clinic-based settings, and typically include cognitive and behavioral components (including direct instruction, modelling skills, and skills practice) (see Klin et al., 2000). Educationally, students with Asperger’s disorder often benefit from modifications and supports provided through special education. Although services vary widely

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based on the region, they may range from specialized schools designed to serve students with Asperger’s disorder to modifications within general education classrooms. Some students may get benefit and support from paraprofessional aides in the classroom, while others may require only slight academic modifications. Most students with Asperger’s disorder benefit greatly from communication interventions aimed at improving pragmatic and social skills (see Klin et al., 2000; Mesibov et al., 2001 for review). Family support, parent training, and instruction on behavior management strategies can also be helpful when disruptive behaviors accompany the clinical picture. For adolescents and adults, there are emerging data showing that both individual and group-based cognitive behavioral therapy are promising in the treatment of co-occurring symptoms of depression and anxiety. Individual work with counsellors or mental health professionals could also focus on social and communication skills training as well as bolstering adaptive functioning. In addition, medications may be prescribed to treat associated symptoms (particularly inattention, depression, and anxiety).

Cross References ▶ Autistic Disorder ▶ Nonverbal Learning Disabilities ▶ Pervasive Developmental Disorder NOS

References and Readings American Psychiatric Association. (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: American Psychiatric Publishing, Inc. Fombonne, E. (2005). The changing epidemiology of autism. Journal of Applied Research in Intellectual Disabilities, 18, 281–294. Fombonne, E., & Tidmarsh, L. (2003). Epidemiologic data on Asperger disorder. Child and Adolescent Psychiatric Clinics of North America, 12, 15–21. Frith, U. (Ed.). (1991). Autism and Asperger syndrome. Cambridge: Cambridge University Press. Frith, U. (2004). Emanuel Miller lecture: Confusions and controversies about Asperger syndrome. Journal of Child Psychology and Psychiatry, 45, 672–686. Ghaziuddin, M. (2002). Asperger syndrome: Associated psychiatric and medical conditions. Focus on Autism and Other Developmental Disabilities, 17, 138–144. Klin, A., Volkmar, F. R., & Sparrow, S. (Eds.). (2000). Asperger syndrome. New York, NY: The Guilford Press. Matson, J. L., & Boisjoli, J. A. (2008). Strategies for assessing Asperger’s syndrome: A critical review of data based methods. Research in Autism Spectrum Disorders, 2, 237–248.

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Mattila, M., Kielinen, M., Jussila, K., Linna, S., Bloigu, R., Ebeling, H., et al. (2007). An epidemiological and diagnostic study of Asperger syndrome according to four sets of diagnostic criteria. Journal of the American Academy of Child & Adolescent Psychiatry, 46, 636–646. McLaughlin-Cheng, E. (1998). Asperger syndrome and autism: A literature review and meta-analysis. Focus on Autism and Other Developmental Disabilities, 13, 234–245. Mesibov, G. B., Shea, V., & Adams, L. W. (2001). Understanding Asperger syndrome and high-functioning autism. New York, NY: Kluwer Academic/Plenum Publishers. Saulnier, C. A., & Klin, A. (2007). Brief report: Social and communication abilities and disabilities in higher functioning individuals with autism and Asperger syndrome. Journal of Autism and Developmental Disorders, 37(4), 788–793. Schopler, E., Mesibov, G. B., & Kunce, L. J. (Eds.). (1998). Asperger syndrome or high-functioning autism? New York, NY: Plenum Press. Wing, L. (1981). Asperger’s syndrome: A clinical account. Psychological Medicine, 11, 115–130.

Assessment of Consent

responsibilities, interpersonal relationships, community life, education, employment, and recreation (classified as social roles). The long form includes 31 subsections, essentially covering the listed domains with a greater degree of specificity. Each item is rated on a 4-point ‘‘level of accomplishment’’ scale (with an additional option to state ‘‘not applicable’’), a 5-point ‘‘level of satisfaction’’ scale, as well as a rating regarding the type and level of assistance required (i.e., no assistance, assistive device, adaptation, human assistance). A score for each item is obtained with reference to a scoring template included in the manual, grading according to the level of difficulty and level of assistance. Item scores range from 0 (not accomplished) to 9 (performed with no difficulty and no assistance), with mid-scale examples being 3 (performed with difficulty and human assistance), and 6 (performed with difficulty and technical aid or adaptation). Scores can then be weighted by the number of applicable activities to obtain domain-level scores, or a simple formula can be used to obtain an overall score.

▶ Informed Consent


Assessment of Life Habits (LIFE-H) J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms The abbreviation LIFE-H is consistent, but version numbers are often appended (e.g., LIFE-H 1.0, 2.0, 3.0, 3.1)

Description The Assessment of Life Habits (LIFE-H) is a self-report measure of social participation of people with disabilities. The original version of the scale consisted of 298 items; later versions have reduced the number of items to 240 (version 3.0). Various short forms are also available (55–77 items), with the most recent being the 77-item version 3.1. The long form is said to take between 20 and 120 min to complete, and the short form, 20–60 min. In the short form (version 3.1), items are organized into 12 categories: nutrition, fitness, personal care, communication, housing, mobility (classified as activities of regular living) and

Noreau, Fougeyrollas, and Tremblay (2005) stated that the LIFE-H was developed to assess social participation in people with disabilities, regardless of the nature of those disabilities, and based upon the Disability Creation Process model, which views handicap as ‘‘the situational result of the interaction of two causal dimensions: the characteristics of the individual and those of the environment.’’ Version 2.0 of the scale was developed following a content validity study that involved 12 experts in rehabilitation medicine evaluating the scale (in terms of clarity and pertinence of content, classifications used in the measurement scales, etc.); modifications included reversing the scoring of the accomplishment section such that higher scores reflected the competence in the activity. Version 3.0 incorporated a greater number of items within particular domains and added additional filter questions to some sections (e.g., if you are not currently employed, skip to section x). Version 3.1 is a short form based upon version 3.0.

Psychometric Data Fougeyrollas et al. (1998) reported that the LIFE-H v1.0 demonstrated acceptable internal consistency in adults and children (Intra-class Correlation (ICCs) > 0.5 for each life habit), and good test–retest reliability in children and adults with spinal cord injury (ICC children r = 0.73,

Assessment of Motor Process Skills

and adults r = 0.74). Inter-rater reliability was examined in a group of 20 stroke patients (Beaulieu et al., 1996; Cited in Noreau et al., 2002), with ICCs for 6/12 ‘‘accomplishment’’ ratings of life habits above 0.6, and 10/12 ‘‘satisfaction’’ ratings for life habits above 0.6. Similar findings have been reported for inter-rater reliability of LIFE-H scores for people with physical disabilities, with ICCs of r > 0.75 for 7/10 categories, and r = 0.89 for the whole scale (Noreau et al., 2004). Several studies have examined the predictive validity of the LIFE-H. Desrosiers et al. (2003) presented evidence of the convergent validity of the LIFE-H in the form of high correlations with the Functional Autonomy Measurement System (SMAF), and moderate correlations with the Functional Independence Measure (FIM). Further, LIFE-H scores were lower in stroke patients than neurologically healthy controls. A comprehensive review of the psychometric properties of the LIFE-H is available online (http:// www.medicine.mcgill.ca/Strokengine-assess/module_lifeh_ indepth-en.html#section3).

Clinical Uses The LIFE-H is available in Dutch, English, and French versions. Adapted forms suitable for use with children aged 0–4 and 5–13 are available (for which a proxy respondent is required). The LIFE-H has been used to evaluate social participation in many patient groups, including children with cerebral palsy, adults with Spinal Cord Injury, Traumatic Brain Injury, and stroke.

Cross References ▶ Functional Autonomy Measurement System ▶ Functional Independence Measure

References and Readings More information is available on http://www.medicine.mcgill.ca/ Strokengine-assess/module_lifeh_indepth-en.html#section3. Accessed 12 May 2009. Desrosiers, J., Rochette, A., Noreau, L., Bravo, G., He´bert, R., & Boutin, C. (Sept.–Oct. 2003). Comparison of two functional independence scales with a participation measure in post-stroke rehabilitation. Archives of Gerontology and Geriatrics, 37(2), 157–172. Desrosiers, J., Noreau, L., Rochette, A., Bravo, G., & Boutin, C. (2002). Predictors of handicap situations following post-stroke rehabilitation. Disability & Rehabilitation, 24(15), 774–785. Fougeyrollas, P., Noreau, L., Bergeron, H., Cloutier, R., Dion, S. A., & St-Michel, G. (1998). Social consequences of long term impairments

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and disabilities: Conceptual approach and assessment of handicap. International Journal of Rehabilitation Research, 21(2), 127–141. Noreau, L., Desrosiers, J., Robichaud, L., Fougeyrollas, P., Rochette, A., & Viscogliosi, C. (March 18, 2004). Measuring social participation: Reliability of the LIFE-H in older adults with disabilities. Disability & Rehabilitation, 26(6), 346–352. Noreau, L., Fougeyrollas, P., & Tremblay, J. (2005). The measure of life habits (LIFE-H) user’s manual. RIPPH. http://www.ripph.qc.ca/? rub2=4&rub=15&lang=en. Accessed 23 Apr 2010. Noreau, L., Fougeyrollas, P., & Vincent, C. (2002). The LIFE-H: Assessment of the quality of social participation. Technology and Disability, 14(3), 113–118. Rochette, A., Desrosiers, J., & Noreau, L. (2001). Association between personal and environmental factors and the occurrence of handicap situations following a stroke. Disability & Rehabilitation, 23(13), 559–569.

Assessment of Motor Process Skills K ELLI W ILLIAMS G ARY Virginia Commonwealth University Richmond, VA, USA

Synonyms AMPS

Description The Assessment of Motor Process Skills (AMPS) is a standardized observational assessment widely used by occupational therapists to measure the quality of performance in activities of daily living (ADL) of persons across the age spectrum beginning at 3 years. Specifically, the AMPS tests functions that relate to purposeful, goal-oriented daily life tasks that a person wants, needs, and is expected to perform; it does not evaluate neuromuscular, biomechanical, cognitive, and psychosocial impairments (Fisher, 2006). The current version of the assessment contains 83 calibrated ADL tasks that permit evaluation of 36 skills (16 motor, 20 process); AMPS-trained raters must observe two or more specific tasks in 10–20 min increments. A multi-perspective approach is used to rate each task by observing various motor and process skills in terms of physical effort, efficiency, safety, and independence. The 16 motor skills reflect the ability to use body positions, obtain and hold objects, move self and objects, and sustain performance during ADL task performance. The 20 process skills

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Assisted Living

pertain to sustaining performance, applying knowledge, temporal organization, organizing space and objects, and adapting performance. Scores are based on observation of the client from certified raters. Motor and process skills are rated simultaneously utilizing a 4-point ordinal criterion referenced rating scale with the highest score denoting competent performance, followed by questionable, inefficient, and markedly inefficient performance. AMPS computer scoring software converts ordinal raw scores of easy skill items for persons of low ability and hard skill items for persons of high ability along a single common equal-interval linear scale (Fisher, 1994).

Historical Background The genesis of the AMPS is found in the psychiatric assessment of clients with schizophrenia and depression in Halifax, Canada (Fisher & Bernspa˚ng, 2007). In 1994, the basic idea was further developed and a specific tool was standardized by Anne G. Fisher, ScD, OTR and colleagues from the Division of Occupational Therapy, Umea˚ University in Umea˚, Sweden. Currently, the AMPS is used in at least 20 countries. In 2006, the most recent version (sixth version) was published to increase applicability across populations, diagnoses, disabilities, cultural background, nationality, and age groups by adding additional tasks (Fisher, 2006).

Psychometric Data The AMPS has robust psychometric properties. Interrater and intrarater reliability are high with 95% of calibrator raters demonstrating goodness-of-fit to the many-faceted Rasch model. Test–retest reliability is high on a diagnostically heterogeneous sample of older adults with r = 0.90 to 0.91 for AMPS process scale and motor scale, respectively (Fisher, 2006). Studies have found good validity of the AMPS when applied to groups of different racial, ethnic, and cultural backgrounds, across gender, and with multiple diagnoses.

Clinical Uses Fisher (2006) states: ‘‘the AMPS provides occupational therapy practitioners with a powerful and sensitive tool that can assist with planning effective ADL interventions and documenting change.’’ Because of the AMPS’ unique and innovative design, occupational performance is evaluated based on the familiarity and relevance of the

tasks to the client’s daily life needs. Therefore, the environment should be naturalistic and approximate the conditions in which the client can comfortably perform tasks. Settings for AMPS observation can vary based on the space available and can include fully equipped clinic kitchens, laundry rooms, outdoor, and the client’s own room in the hospital or nursing home. The primary advantage of the AMPS is that it can be used with persons of virtually any age, diagnosis, or disability. However, the scope and breadth of evidence for AMPS use is limited in psychiatric, neurologic, and pediatric settings. Geriatric settings have offered the most research evidence for those with cognitive impairments and dementias, followed by a sizable proportion of research for people with learning disabilities (Hitch, 2007).

Cross References ▶ Activities of Daily Living ▶ Instrumental Activities of Daily Living ▶ Occupational Therapy

References and Readings Fisher, A. G. (1994). Development of a functional assessment that adjusts ability measures for task simplicity and rater leniency. In M. Wilson (Ed.), Objective measurement: Theory into practice (Vol 2, pp. 145– 175). Norwood, NJ: Ablex. Fisher, A. G. (2006). Assessment of motor and process skills. Vol. 1: Development, standardization, and administration manual (6th ed.). Fort Collins, CO: Three Star. Fisher, A. G., & Bernspa˚ng, B. (2007). Response to: A critique of the Assessment of Motor and Process Skills (AMPS) in mental health practice. Mental Health Occupational Therapy, 12, 10–11. (Available from http://www.ampsintl.com/documents/MHOT%20March% 202007.pdf) Hitch, D. (2007). A reply from Danielle Hitch to the Fisher and Bernspa˚ng response to: A critique of the Assessment of Motor and Process Skills (AMPS) in mental health practice. Mental Health Occupational Therapy, 12, 14. (Available from http://www.ampsintl. com/documents/MHOT%20March%202007.pdf)

Assisted Living J AY B EHEL Rush University Medical Center Chicago, IL, USA

Synonyms Domiciliary care; Residential care

Assistive Technology

Definition

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Assisted living is a care arrangement that provides supervision and assistance to individuals who are unable to live independently but do not require the level of care provided in conventional nursing homes.

D IANE C ORDRY G OLDEN Association of Assistive Technology Act Programs Delmar, NY, USA

Current Knowledge

Definition

Assisted living arrangements may take place in structured assisted living facilities, small group homes, or an individual’s own home or the home of a family member. These arrangements have as their goal the preservation of a degree of autonomy and privacy at home or in a homelike setting. When sited in one’s home, assistance may be provided by a combination of paid caregivers, family members, and other paid or unpaid assistants to help with housekeeping, laundry, cooking, and transportation. Assistance provided may include supervision for safety, medication management, meal preparation, and accompaniment and assistance during community-based activities. Assisted living facilities may also offer social activities and specialized services for individuals with cognitive impairment. Basic Activities of Daily Living (BADL) such as hands-on bathing, dressing, and feeding are usually not considered as a part of an assisted living arrangement as the consistent need for such basic care is often seen as an indication that nursing home or a homebased parallel thereof is the more appropriate level of care. Assisted living typically is not covered by private insurance or Medicare, and access to such care may be limited by an individual’s finances. Moreover, there is a considerable variability in how care facilities, clinicians, and professional literature define and discuss assisted living. Consequently, the appropriate role of assisted living in the continuum of care remains unclear, and refinement and redefinition of this role likely will be ongoing for some time.

Assistive technology (AT) is a term used to refer to both AT devices and AT services. A formal, legal definition of AT devices and services was first published in the Technology-Related Assistance for Individuals with Disabilities Act of 1988 as follows:

Cross References ▶ Life Care Planning

References and Readings Stone, R. I., & Reinhard, S. C. (2007). The place of assisted living in longterm care and related service systems. Gerontologist, 47(Spec. No. 3), 23–32.

" assistive technology device means any item, piece of

equipment, or product system, whether acquired commercially, modified, or customized, that is used to increase, maintain, or improve functional capabilities of individuals with disabilities " assistive technology service means any service that direct-

ly assists an individual with a disability in the selection, acquisition, or use of an assistive technology device

AT devices include a vast array of items such as wheelchairs, eyeglasses, hearing aids, Braille printers, electronic note-takers and organizers, augmentative communication systems, text-to-speech software, speech synthesizers, adaptive keyboards, alternative pointing devices, voice recognition software, aids for daily living, etc. AT services include evaluation/assessment services, selecting, fitting, customizing, and repairing devices, delivering training and technical assistance supports, and coordinating funding and other necessary interventions to support device acquisition and use. The definition of AT devices and services has remained unchanged through numerous reauthorizations of the Assistive Technology Act and has been adopted in other statutes, such as the Individuals with Disabilities Education Act. The same definition has also been used in promulgating federal rules, such as the Electronic and Information Technology Accessibility Standards developed pursuant to Section 508 of the Rehabilitation Act (http://www.accessboard.gov/sec508/standards.htm).

Historical Background A precise history of AT is difficult to depict because of the diversity of devices and services included in the definition

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of AT. The history of hearing aids can be traced back to Alexander Graham Bell’s pioneering work on development of the telephone. Modern wheelchairs are patterned after the first folding, tubular steel wheelchair developed in the 1930s; while the first dedicated wheelchair (called an invalids chair) is thought to have been invented 4 centuries ago for Phillip II of Spain. Some devices were developed as AT and evolved into mainstream technology. For example, in 1948 the National Bureau of Standards developed specifications for a low-cost reliable talking-book machine for the blind that became the tape recorder. Conversely, some items developed as mainstream technology became AT such as voice recognition software originally developed for dictation that is used by individuals with motor disabilities who are unable to use a keyboard for computer access. In recent years, technology use has become more commonplace for everyone. Similarly, AT use is now more frequent across the disability spectrum, addressing deficits in hearing, vision, motor, social, organizational, cognitive, speech, language, information processing, etc. Especially critical today is the use of information technology (IT), including telecommunications. IT use is now critical to success in education, employment, independent living, and community integration and AT is the interface that makes IT accessible (http://www.albritton.us/AThistory.html).

Rationale or Underlying Theory Today, AT intervention is rooted in the disability rights movement and self-determination efforts of individuals with disabilities and their advocates. These initiatives helped to delineate the difference between the medical/ rehabilitation and independent living models of intervention for individuals with disabilities. The medical model identifies a physical or mental impairment or lack of certain skills and treatment is delivered to remediate the deficit(s). With the medical model the locus of the problem lies with the individual and the goal is to ‘‘fix’’ the individual in some way through professional treatment. Under the independent living model the problem is defined as a lack of supports and accommodations, inaccessibility, and/or autonomy – the problem lies with the environment or the interaction with the environment, rather than within the person. In this model, AT plays a major role in addressing/ameliorating interaction difficulties, typically without overtly attempting to ‘‘fix’’ the disability itself (DeJong, 1979; Pelka, 1997).

Goals and Objectives AT goals and objectives begin with a primary focus on ameliorating and/or compensating for a specific functional deficit. For example, electronic organizers can be used to address memory or information processing problems; text-to-speech software can be used to address reading deficits; augmentative communication systems can be used to address communication limitations, etc. In most cases, secondary goals are also targeted for outcomes including increasing academic success, fostering gainful employment, supporting independent community living, decreasing inappropriate behaviors, etc. With expanding legal mandates for integration of individuals with disabilities into all societal settings (Individuals with Disabilities Education Act, Section 504 of the Rehabilitation Act, and the Americans with Disabilities Act), AT goals and objectives continue to expand into new outcome areas.

Treatment Participants AT is an appropriate intervention option to consider when functional limitations are encountered. Candidacy for AT is not limited by age, disability diagnosis, or severity/combination of deficits. There are no prerequisites for AT consideration and AT should not be relegated to a ‘‘last resort’’ intervention after all other interventions have been tried and abandoned. AT can address a variety of human functions and is frequently grouped into areas such as vision, hearing, communication, daily living, computer access, learning/ cognition, environmental adaptations, mobility/seating/ positioning, vehicle modifications, and recreation/leisure. For almost all functional limitations, there is a range of AT intervention that can be considered as a treatment option (Cook & Hussey, 2001).

Treatment Procedures Consideration for AT begins with assessment by a qualified team of individuals who are knowledgeable about the individual, their strengths and limitations and the range of potential AT options available to address the individual’s functional needs. Best practice includes conducting structured device trials with various AT devices in the environment(s) in which the individual will be using the technology (e.g., home, school, work, community, etc.). This allows for comparative analysis of different device


features and functions to determine which best addresses the individual’s needs. Once AT has been acquired for an individual, training and support must be provided for the user, their family, and other critical individuals such as teachers, therapists, etc. More complex AT (computer-based software applications, assistive listening systems, augmentative communication devices, etc.) frequently requires significant investment of time and resources in initial programming, fitting, and set-up, in addition to training on device use (Galvin & Scherer, 1996).

Efficacy Information Efficacy research on AT includes basic documentation of changes in functional skill areas (those the AT is intended to address) and potential secondary improvements in academic, social, behavioral, and other areas. Much of this efficacy is self-evident and is reported by the AT users themselves (Scherer, 1993). No discussion of AT efficacy would be complete without addressing the issues of device abandonment and cost/benefit. For many types of AT, consumer discontinuing use of the device after acquisition has been a historic problem (Wessels, Dijcks, Soede, Gelderblom, & De Witte, 2003). Factors shown to mitigate abandonment include active consumer and family involvement in the selection and implementation of AT and the relative advantage of AT within the array of intervention options available (Alper & Raharinirina, 2006; Riemer-Reiss & Wacker, 2000). As technology continues to improve, the problem of device abandonment steadily abates. Today the greater challenge is in justifying the cost/benefit of AT to secure funding from private insurance, local, state, federal, and other funding sources. Some types of AT, such as durable medical equipment, have a longer history of cost/benefit data including prevention of secondary disabilities making funding more readily available. Other types of AT, such as electronic organizers used to remediate/compensate for cognitive limitations, are relatively new with little cost/benefit data making funding difficult to secure (Gillette & DePompei, 2004; Hart, Buchhofer, & Vaccaro, 2004).

Outcome Measurement The field of AT outcomes is quite young with most published work emerging in the 1990s. Two of the first focused articles on evaluating AT outcomes posed the questions ‘‘Are we ready to answer the tough questions?’’

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and ‘‘Do we understand the commitment?’’ (DeRuyter, 1995; Trachtmann, 1994). In these articles, the authors postulated that stakeholders and AT providers must be prepared to show how their devices/services make a difference in the lives of individuals who receive an AT intervention. Today, outcome measurement is occurring in all AT service areas (medicine, education, employment/vocational rehabilitation, and independent living) through a variety of interdisciplinary activities. Some are driven by policy needs, in particular, accountability for public dollars spent on AT (e.g., Medicare, Medicaid, special education, vocational rehabilitation, etc.) and justification for private insurance expenditures on AT. Others are driven by an overarching goal of quality service delivery and continuous program improvement. The most direct outcome measure for AT intervention is demonstration of functional skills, independence, wellbeing, and quality of life. Administration of standardized assessments in the aided condition (using AT) can be useful in measuring outcomes in discreet skill areas (e.g., auditory discrimination, memory, expressive communication, etc.). In addition, some global assessment of AT outcomes can be helpful such as the Psychosocial Impact of Assistive Devices Scales (PIADS) (Jutai & Day, 2002), the Quebec User Evaluation of Satisfaction with Assistive Technology (QUEST) (Demers, Weiss-Lambrou, & Ska, 1996), and similar instruments. A number of online resources are also available with extensive data on AT outcome tools and research such as the Adaptive Technology Resource Center (http://atrc.utoronto.ca/index.php? option=com_content&task=view&id=175&Itemid=69), the Assistive Technology Outcomes Measurement System (ATOMS) Project (www.uwm.edu/CHS/atoms), the Consortium for Assistive Technology Outcomes Research (www.atoutcomes.org), and the Quality Indicators for Assistive Technology Services (www.qiat.org).

Qualifications of Treatment Providers AT intervention can be provided by an extensive list of professionals, usually specialists in the type of the AT provided. For example, hearing aids are typically provided by audiologists or hearing instrument dispensers, eyeglasses by optometrists and ophthalmologists, etc. However, as the AT becomes less ‘‘prescriptive’’ in nature, the range of providers expands. Electronic note-takers and organizers can be provided by a whole host of providers, special educators, rehabilitation counselors, behavior therapists, occupational therapists, AT practitioners, etc.

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The Rehabilitation Engineering and Assistive Technology Association of North America (RESNA) administers a certification program for Assistive Technology Professionals (ATPs) with associated standards of practice (http://www. resna.org/assets/240_standardsofpracticefinal1.pdf).

Association Areas M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Cross References

Synonyms

▶ Americans with Disabilities Act of 1990 ▶ Augmentative and Alternative Communication ▶ Independent Living Centers ▶ Individuals with Disabilities Education Act ▶ Section 504 of the Rehabilitation Act of 1973

Association cortex

References and Readings Alper, S., & Raharinirina, S. (2006). Assistive technology for individuals with disabilities: A review and synthesis of literature. Journal of Special Education Technology, 21(2), 47–64. Cook, A., & Hussey, S. (2001). Assistive technologies: Principles and practice (2nd ed.). California: Mosby Year-Book, Inc. DeJong, G. (1979). Independent living: From social movement to analytic paradigm. Archives of Physical Medicine and Rehabilitation, 60, 435–446. Demers, L., Weiss-Lambrou, R., & Ska, B. (1996). Development of the Quebec user evaluation of satisfaction with assistive technology (QUEST). Assistive Technology, 8, 3–13. DeRuyter, F. (1995). Evaluating outcomes in assistive technology: Do we understand the commitment? Assistive Technology, 7(1), 3–16. Galvin, J. C., & Scherer, M. J. (1996). Evaluating, selecting and using appropriate assistive technology. Gaithersburg, MD: Aspen Publishers, Inc. Gillette, Y., & DePompei, R. (2004). The potential of electronic organizers as a tool in the cognitive rehabilitation of young people. NeuroRehabilitation, 19(3), 233–243. Hart, T., Buchhofer, R., & Vaccaro, M. (2004). Portable electronic devices as memory and organizational aids after traumatic brain injury: A consumer survey study. Journal of Head Trauma Rehabilitation, 19(5), 351–365. Jutai, J., & Day, H. (2002). Psychosocial impact of assistive devices scale (PIADS). Technology and Disability, 14(3), 107–111. Pelka, F. (1997). ABC-CLIO companion to the disability rights movement. Santa Barbara, CA: ABC-CLIO, Inc. Riemer-Reiss, M. L., & Wacker, R. R. (2000). Factors associated with assistive technology discontinuance among individuals with disabilities. Journal of Rehabilitation, 66(3), 44–50. Scherer, M. J. (1993). Living in the state of stuck: How technology impacts the lives of people with disabilities. Cambridge, MA: Brookline Books. Trachtmann, L. (1994). Outcome measures: Are we ready to answer the tough questions? Assistive Technology, 6, 91–92. Wessels, R., Dijcks, B., Soede, M., Gelderblom, G. J., & De Witte, L. (2003). Non-use of provided assistive technology devices, a literature overview. Technology and Disability, 15(4), 231–238.

Definition It is recognized that the brain is neither holistic nor rigidly localized with respect to cognitive functions. However, higher-order cognitive capabilities depend on specialized regions within the brain that process, link or integrate elementary or new, as well as stored information into increasingly complex wholes. Such regions are termed association areas and are thought to be the neuroanatomical substrate for such higher functions as memory, emotion, perception, language, spatial and problem-solving skills, as well as the planning and execution of behavioral responses. Three major association areas are recognized: (1) Frontal association cortices, as the name implies, are located in the more anterior aspects of the frontal lobes and include dorsolateral, orbitofrontal, and premotor areas. While various feedback loops are likely involved including those from the posterior and limbic association areas, conceptually, the initial decisions and planning regarding executive or motor responses to a given situation are generally thought to flow from the prefrontal (most anterior) cortices to the premotor cortex that organizes, coordinates, and sequences the actions essential to the successful completion of the response. From there, commands are believed to be forwarded to the primary motor area (precentral gyrus) that then actually executes the motor response. The orbitofrontal cortex is shared with limbic association cortex (see below) that underscores the importance of the integration of emotion, memory, and behavior. Lesions to prefrontal association cortices often affect self-monitoring, planning, and executive functions, including behavioral inhibition. (2) The limbic association cortex includes ventromedial frontal lobe, medial parietal lobe, temporal pole, and cingulate and parahippocampal areas. Integration of information from the hypothalamus, other limbic or paralimbic structures, and secondary sensory

Association for Postdoctoral Programs in Clinical Neuropsychology (APPCN)

association areas is received and projected to other areas of the cortex, including the prefrontal cortex discussed above, again permitting the integration of emotions, cognition and perceptions, and memory. Dysfunction is often expressed as emotional/behavioral dysregulation and memory impairment. (3) The locations of the parieto-temporal-occipital association cortices are described by their names and are typically divided into secondary and tertiary areas. The former are unimodal in nature (respond more or less exclusively to a single sensory modality) and lie adjacent to their respective primary cortical sensory projection areas. They are thought to be responsible for further integrating and processing sensory input into potentially meaningful percepts. Hence, lesions of these secondary association areas will commonly result in modality-specific perceptual disturbances or agnosias. By contrast, the tertiary or heteromodal association areas receive input from all sensory modalities. Because of this and their central location, they are sometimes referred to as the PTO (parietal-temporal-occipital) cortex. Because of their crossed or multimodal inputs, these latter areas, which are very highly developed in man, are thought to represent the foundations for higher-order conceptual and intellectual abilities, including abstraction, language, and visual-spatial and mathematical problem solving, any or all of which can be adversely affected by lesions to these areas.

Cross References ▶ Association Pathways ▶ Heteromodal Cortex ▶ Secondary Cortex ▶ Unimodal Cortex

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Association Cortex ▶ Association Areas ▶ Homotypic Cortex

Association for Postdoctoral Programs in Clinical Neuropsychology (APPCN) J ACOBUS D ONDERS Mary Free Bed Rehabilitation Hospital Grand Rapids, MI, USA

Address (and URL) APPCN P.O. Box 69037, Pleasant Ridge, MI 48069 www.appcn.org

Membership The Association of Postdoctoral Programs in Clinical Neuropsychology (APPCN) is an organization of approximately 50 member programs that offer comprehensive, integrated postdoctoral residencies.

Major Areas or Mission Statement The mission of APPCN is to foster the provision of advanced specialty education and training to promote the competencies that are necessary for practice in the specialty of clinical neuropsychology (Boake, Yeates, & Donders, 2002).

References and Readings Landmark Contributions Kupermann, I. (1991). Localization of higher cognitive and affective functions: The Association cortices. In E. R. Kandel, J. H. Schwartz, & T. M. Jessel (Eds.), Principles of neural science (3rd ed., pp. 823– 838). East Norwalk, CT: Appleton & Lange. Mendoza, J. E., & Foundas, A. F. (2008). Clinical neuroanatomy: a behavioral approach. New York: Springer. Mesulam, M.-M. (2000). Principles of behavioral and cognitive neurology. New York: Oxford University Press. Pandya, D. N., & Seltzer, B. (1982). Association areas of the cerebral cortex. Trends in Neurosciences, 5, 385–390. Pandya, D. N., & Yeterian, E. H. (2003). Cerebral cortex: architecture and connections. In Encyclopedia of the neurological sciences (pp. 594– 604). USA: Elsevier Science.

Formally incorporated in 1992, APPCN contributed to the Houston Conference, which established that completion of two years of formal postdoctoral residency training is a uniform requirement for entry into the professional practice of clinical neuropsychology (Hannay, Bieliauskas, Crosson, Hammeke, Hamsher, & Koffler, 1998). In more recent years, APPCN has been a key representative to interorganizational groups like the Clinical Neuropsychology Synarchy, and the Inter-Organizational Summit on Education and Training.

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Association for Postdoctoral Programs in Clinical Neuropsychology (APPCN)

Major Activities APPCN is not an accrediting organization, a role which is left to the Commission on Accreditation of the American Psychological Association (APA). However, a growing number of the APPCN members are currently accredited by APA as a postdoctoral specialty program in clinical neuropsychology. Details about individual APPCN member programs, including focus on adult v. pediatric neuropsychology, accreditation status, primary diagnostic groups served, and other characteristics, are available on the APPCN website. The major standards for program membership in APPCN include the following: (1) the duration of training is for a minimum of 2 years, or for an equivalent time on no less than a half-time basis, at a fixed site with regular, on-site supervision; (2) the program includes an organized and integrated combination of at least 50% clinical service, at least 10% didactic/educational activities, and at least 10% research or other scholarly activities; and (3) the program director is board-certified in Clinical Neuropsychology through the American Board of Professional Psychology (ABBP–CN). One of the major accomplishments of APPCN is the development and implementation, in collaboration with National Matching Services, of a computerized system for matching of applicants for postdoctoral residency training in clinical neuropsychology to programs that offer such training. This electronic system, instituted in 2001, is the most fair to applicants, and the most efficient for programs, with all APPCN programs that have open positions in any given year taking part in this electronic match. Postdoctoral programs that are not members of APPCN are also allowed to participate as long as they meet APPCN standards #1 and #2 above, and if they agree to respect all other conditions of the match, including prohibition of pre-emptive offers, and adherence to the binding nature of the match outcomes. Further details are available at http://www.natmatch.com/appcnmat/. APPCN is dedicated to education of aspiring neurop sychologists about what it takes to become a competitive candidate for postdoctoral residency training. For this purpose, several educational seminars are offered on a regular basis, some in collaboration with other organizations, such as Division 40 (▶ Clinical Neuropsychology) of the APA and the Association of Neuropsychology Students in Training (ANST). Over the past several years, APPCN program directors have also consistently provided a ‘‘special topic presentation’’ about postdoctoral residency training at the annual meeting of the National Academy of Neuropsychology (NAN).

APPCN has also strived to make the process of education and evaluation as part of postdoctoral residency training in clinical neuropsychology more standardized. For this purpose, a 50-item written examination has been developed by APPCN to be used with residents who are near completion of their first postdoctoral training year, to evaluate their knowledge of major content areas like functional neuroanatomy, adult and pediatric syndromes, psychometrics, etc. This exam is not intended to give residents a ‘‘grade’’; rather, it is to be used as an educational tool, to identify relative strengths and weaknesses in the residents’ working knowledge base, so that the relative lacunae can be addressed during the subsequent training year. During that second year, APPCN member programs also have the opportunity to start preparing residents for board certification, by means of ethics vignettes and mock oral ‘‘fact finding’’ case materials that are very similar in format and level of difficulty to those used by ABPP–CN. Finally, the most recent initiative of APPCN has involved advocacy with the United States Department of Veterans Affairs for the development of more postdoctoral training programs in clinical neuropsychology, primarily because of the high number of traumatic brain injuries among US service personnel involved in the combat in Iraq. When more than a dozen of these training programs became available in the Fall of 2007, APPCN contacted the programs, provided mentoring as needed, and waived their first-time participation fee in the electronic match. APPCN has also continued to offer assistance to new programs as they seek specialty accreditation through the APA. APPCN will continue to embrace additional organized and integrated postdoctoral training programs in clinical neuropsychology.

Cross References ▶ American Board of Clinical Neuropsychology (ABCN) ▶ American Board of Professional Psychology (ABPP) ▶ American Psychological Association (APA)

References and Readings Boake, C., Yeates, K. O., & Donders, J. (2002). Association of Postdoctoral Programs in Clinical Neuropsychology: update and new directions. The Clinical Neuropsychologist, 16, 1–6. Hannay, H. J., Bieliauskas, L., Crosson, B. A., Hammeke, T. A., Hamsher, K., & Koffler, S. P. (1998). The Houston Conference on specialty education and training in clinical neuropsychology. Archives of Clinical Neuropsychology, 13, 157–250.

Associative Aphasia

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Cross References

Association Pathways

▶ Commissures, Cerebral ▶ Projection Pathways


Associational Fibers

Definition Fiber pathways that lie within the cerebrum that connect one part of the cerebral cortex with another within the same hemisphere. Association pathways are thus contrasted with commissures that generally interconnect homologous areas of the two halves of the brain, and projection pathways that are fiber tracts interconnecting cortical and subcortical structures. They may be very long (typically termed ‘‘fasciculi’’) or very short. The latter may consist of ‘‘U’’-shaped fibers connecting one gyrus with an adjacent one or horizontal connections within a gyrus itself (e.g., bands of Baillarger). These various pathways allow different areas of the brain to communicate with one another. Some of the major association pathways are shown in Fig. 1.

M ELISSA J. M C G INN Virginia Commonwealth University School of Medicine Richmond, VA, USA

Synonyms Arcuate fibers

Definition Associational fibers are white matter fibers that connect various cortical regions within the same cerebral hemisphere. Being the most prevalent type of neuronal tracts found in the cortex, associational fibers permit bidirectional communication between different cortical areas, allowing the cortex to function as a coordinated whole. Associational fibers arise from cortical layer II/III pyramidal neurons and can be classified as either short associational fibers, which connect adjacent gyri within the same lobe, or long associational fibers, interconnecting more distant regions located in different lobes. The major long associational fibers tracts in the brain include the superior longitudinal fasciculus, arcuate fasciculus, uncinate fasciculus, and cingulum.

Cross References ▶ Arcuate Fasciculus ▶ Association Pathways ▶ Cerebral Cortex ▶ Cingulum ▶ Superior Longitudinal Fasciculus ▶ White Matter

U U SLF

AF arcuate fasciculus IOF inf. occiptofrontal fasc. SLF sup. longitudinal fasc. U U-fibers UF uncinate fasciculus

Association Pathways. Figure 1

AF IOF UF

Associative Aphasia ▶ Conduction Aphasia

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Associative Memory

Associative Memory ▶ Paired-Associate Learning

Associative Visual Agnosia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition Regardless of modality, an associative agnosia implies that although perception is intact, the particular stimulus has no meaning (‘‘associative’’ value) to the individual. The stimulus can neither be named nor linked to other personal or sensory experiences. Hence, associative visual agnosia refers to the inability to identify or categorize a visually presented stimulus despite adequate visual perception.

Current Knowledge Individuals with this disorder should be able to match the visual stimulus to a sample and copy or draw what is seen, thus distinguishing associative from apperceptive visual agnosia. In the latter condition, visual object recognition is also impaired, but primarily as a result of a disturbance of perception. In addition to having difficulty naming visually presented objects, a patient suffering from associative visual agnosia would likely be unable to describe their use or purpose, or indicate to which category of objects they may belong. However, in pure visual associative agnosia, identification should be possible if the patient were allowed to hold the object(s) (tactile recognition). An associative visual agnosia may differentially affect recognition of objects, words, colors, or faces. In visual agnosia for words (also known as pure alexia or pure word blindness), visual word recognition is impaired. But the individual may be able to ‘‘read’’ if allowed to trace the letters with a finger, thus permitting tactile or kinesthetic recognition of individual letters. In associative color agnosia, the individual may be able to match colors, but neither name them nor identify objects with which they might be associated (such as cherries or apples for the color red). Facial agnosia (prosopagnosia) is a bit complex in that one may differentiate the inability to make discriminations among unfamiliar faces (thought to be more of a

perceptual problem) from an inability to recognize familiar faces (generally considered an associative problem). Thus, in the latter instance, while the patient might be able to match the face or picture of a familiar person to one within an array of pictures, he would not be able to identify the face or the picture as that of his wife, his daughter, or other famous person with whom he might be familiar. While the specific lesions causing specific associative visual agnosias are not well defined, they are generally thought to represent a disconnection type syndrome involving the temporal, occipital, and/or parietal regions of the left hemisphere with some disruption of fiber pathways or connections between the unimodal (visual) and heteromodal cortices.

Cross References ▶ Alexia ▶ Apperceptive Visual Agnosia ▶ Color Agnosia ▶ Color Anomia ▶ Disconnection Syndrome ▶ Heteromodal Cortex ▶ Prosopagnosia ▶ Unimodal Cortex

References and Readings Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman & E. Valenstein (Eds.), Clinical neuropsychology, (4th ed., pp. 236– 295). New York: Oxford University Press. DeRenzi, E., & Spinnler, H. (1966). Visual recognition in patients with unilateral cerebral disease. Journal of Nervous and Mental Disease, 142, 513–525. DeRenzi, E., Scotti, G., & Spinnler, H. (1969). Perceptual and associative disorders of visual recognition. Relationship to the side of the cerebral lesion. Neurology, 19, 634–642.

Astasia-Abasia D OUGLAS I. K ATZ Braintree Rehabilitation Hospital Braintree, MA, USA Boston University School of Medicine Boston, MA, USA

Synonyms Blocq’s disease

Astereognosis

Definition An inability to stand and walk in a normal and coordinated manner. Astasia means inability to maintain standing and abasia refers to impaired coordination of gait. The term is usually applied to unusual, often bizarre patterns of gait and stance that appear to have no neuropathophysiologic basis. Conversion disorder is frequently the underlying cause. Patients may sway in a staggering, unstable manner, often catching themselves before falling. This syndrome is also referred to as Blocq’s disease.

Cross References ▶ Abasia ▶ Gait Disorders ▶ Psychogenic Disorder

References and Readings Morris, J.G., Mark de Moore, G., Herberstein, M. (2006). Psychogenic Gait: An example of Deceptive Signaling. In: C.R. Cloninger, & M. Hallett (Eds.), Psychogenic movement disorders: neurology and neuropsychiatry. Philadelphia: Lippincott Williams & Wilkins. Okun, M.S., & Koehler, P.J. (2007). Paul Blocq and (psychogenic) astasia abasia. Movement Disorder, 22, 1373–1378.

Astereognosis M ELISSA A MICK VA Boston Healthcare System Boston, MA, USA

Synonyms Object agnosia; Tactile agnosia

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also called morphognosia, reflect impairments in recognizing the physical features of the object (e.g., weight or texture). Secondary recognition deficits reflect a specific impairment in object recognition with spared primary recognition (for review see De Renzi, 1982).

Epidemiology Astereognosis can be common after stroke with one report indicating that up to 90% of patients demonstrate astereognosis (Connell, Lincoln, & Radford, 2008). Damage to the cortical regions important for haptic input integration can cause astereognosis. This disorder, therefore, is common and can occur in the presence of many neurological disorders including brain (e.g., Knecht, Kunesch, & Schnitzler, 1996), or spinal cord tumors (Lesoin, Rousseaux, Martin, Petit, & Jomin, 1986), and traumatic brain injury (Hom & Reitan, 1982).

Natural History, Prognostic Factors, Outcomes Connell et al. (2008) followed 58 stroke survivors over a period of 6 months (baseline, 2, 4, and 6 months) and at each time period participants completed the Nottingham Sensory Assessment (see below). Stereognosis significantly improved during the observation period, with the greatest changes occurring within the first 4 months (baseline relative to 4-month performance). Regression analyses indicated that stroke severity and motor performance of the upper limb were predictive of the presence of impaired stereognosis at the baseline assessment.

Neuropsychology and Psychology of Astereognosis

Short Description or Definition Astereognosis is defined as the inability to identify objects through touch without visual input.

Categorization Astereognosis has been subdivided into primary and secondary recognition deficits. Primary recognition deficits,

Astereognosis can occur after injury to either the left or right hemisphere. A specialized role for the right hemisphere in stereognosis has been proposed, however this finding has not been consistently observed (for review see Zaidel, 1998 and De Renzi, 1982). Initially astereognosis was thought to be due to damage to the primary somatosensory cortex; however, posterior parietal lesions have also been associated with this impairment (Knecht et al., 1996).

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Evaluation

Cross References

Astereognosis is often examined with non-standardized methods. In the typical neurological examination, astereognosis is assessed by asking the patient to identify an object through touch without visual input. Common objects used for identification can include coins, keys, paper clips, or screws. For patients with hemiparesis, the examiner may manipulate the patient’s hand to assist in object identification. Standardized assessments of astereognosis do exist. The Tactile Form Recognition Test from the Halstead–Reitan Neuropsychological Test Battery (Reitan & Wolfson, 1993) requires participants to manipulate a flat plastic shape with one hand obscured from vision, while the other hand points to the same shape mounted on a board with three other potential distractors. In the Benton Stereognosis Test (Benton, 1969), 10 cards with fine grain, sandpaper figures pasted on top are felt by the participant out of view. The participant has 30 s to explore the card and 45 s to respond. Responses are made by pointing to the corresponding line drawing mounted in full view of the respondent. The Nottingham Sensory Assessment includes an assessment of astereognosis (Gaubert & Mockett, 2000). In this task the participant is blindfolded and asked to name the object placed in their hand. Presentation of the objects is time limited. Objects to be identified include coins, comb, sponge, pencil, scissors, a cup, and a glass. Responses are scored on a scale of 0-2 depending upon the quality of the verbal response.

▶ Ahylognosia ▶ Amorphognosis ▶ Parietal Lobe ▶ Somatosensory Cortex ▶ Stereognosis ▶ Tactile Agnosia ▶ Tactile Form Recognition ▶ Tactual Performance Test

Treatment Astereognosis has been observed to spontaneously improve over time (Connell et al., 2008). One study has found that stereognosis improves following systematic hand retraining in stroke survivors who were at least 2 years post stroke. Yekutiel & Guttman (1993) had 25 participants receive three, 45-min hand-retraining sessions weekly for a period of 6 weeks. The therapy was customized for each participant but everyone received education to improve insight about their impairment and exercises were intended to be appropriately challenging, designed to promote self-efficacy, used vision and the less affected hand to aid sensory function, and provided frequent breaks and novel stimuli. Unlike the control group, the patient group showed a statistically significant improvement on the stereognosis assessment. These findings suggest that functional gains through therapy can occur in the years following stroke.

References and Readings Benton, A. L. (1969). Stereognosis test; Manual of instructions. Neuropsychology Laboratory, Department of Psychology, University of Victoria. De Renzi, E. (1982). Disorders of space exploration and cognition. New York: Wiley. Connell, L. A., Lincoln, N. B., & Radford, K. A. (2008). Somatosensory impairment after stroke: Frequency of different deficits and their recovery. Clinical Rehabilitation, 22, 758–767. Gaubert, C. S., & Mockett, S. P. (2000). Inter-rater reliability of the Nottingham method of stereognosis assessment. Clinical Rehabilitation 14(2), 153–159. Hom, J., & Reitan, R. M. (1982). Effect of lateralized cerebral damage upon contralateral and ipsilateral sensorimotor performances. Journal of Clinical Neuropsychology 4(3), 249–268. Knecht, S., Kunesch, E., & Schnitzler, A. (1996). Parallel and serial processing of haptic information in man: Effects of parietal lesions on sensorimotor hand function. Neuropsychologia, 34, 669–687. Lesoin, F., Rousseaux, M., Martin, H. J., Petit, H., & Jomin, M. J. (1986). Astereognosis and amyotrophy of the hand with neurinoma of the second cervical nerve root. Neurology, 233, 57–58. Lincoln, N. B., Crow, J. L., Jackson, J. M., Waters, G. R., Adams, S. A., & Hodgson, P. (1991). The unreliability of sensory assessment. Clinical Rehabilitation, 5, 273–282. Reitan, R. M., & Wolfson, D. (1993). The Hasltead-Reitan neuropsychological test battery: Theory and clinical interpretation. Tucson, AZ: Neuropsychology Press. Yekutiel, M., & Guttman, E. J. (1993). A controlled trial of the retraining of the sensory function of the hand in stroke patients. Journal of Neurology, Neurosurgery, and Psychiatry, 56, 241–244. Zaidel, E. (1998). Stereognosis in the chronic split brain: Hemispheric differences, ipsilateral control and sensory integration across the midline. Neuropsychologia, 36, 1033–1047.

Asthenia ▶ Adynamia

Asymmetry

Astrocytoma

Asymmetry

R OBERT R IDER Drexel University Philadelphia, PA, USA

M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Definition

Synonyms

Astrocytomas are the most frequently diagnosed tumors, are usually slow-growing, and may develop a cystic component. Arising in astrocytic cells anywhere throughout the central nervous system, they may occur in any age group, but are most frequently diagnosed in middle-aged males. The highest incidence of brain stem astrocytomas is found in children. Grading systems focus on the degree of resemblance to normal astrocytes, with higher grades associated with more rapid growth and greater likelihood of metastasis. Three common types of astrocytomas are: low-grade astrocytomas, which are often benign and tend to occur in the cerebellum (especially in children) but may also occur in the cerebrum in adults; anaplastic astrocytomas, which are malignant; glioblastoma multiforme, which are thought to arise from astrocytomas and are the most malignant. The specific symptoms associated with astrocytomas depend on the region of the CNS that is affected.

Hemispheric specialization

Cross References ▶ Fibrillary Astrocytoma ▶ Oligoastrocytoma ▶ Pilocytic Astrocytoma ▶ Xanthroastrocytoma

References and Readings Louis, D., Ohgaki, H., Cavenee, W., & Wiestler, O. (2007). WHO classification of tumours of the central nervous system (4th ed.). World Health Organization.

Astrocytosis ▶ Gliosis

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Definition Asymmetry is the discordance between the right and left sides of the brain in respect to structure and/or function.

Current Knowledge Although not initially linked to brain asymmetry, the first behavioral asymmetry that was likely noted was the superiority of motor skills exhibited by one hand, most commonly the right, over the other. The next real breakthrough with regard to asymmetry is generally thought to have occurred in the nineteenth century with the discovery that acquired language deficits (aphasia) were typically associated with lesions of the left hemisphere. Since then, other asymmetries, both functional and structural, have been identified with regard to the two cerebral hemispheres.

Structural Asymmetries Structural asymmetries of the brain were first noted around the beginning of the twentieth century, but it was not until the late 1960s that these were first strongly correlated with functional differences between the hemispheres. In a study of 100 postmortem brains, Geschwind and Levitsky (1968) noticed that the planum temporale, located in the temporal operculum, was larger in 65% of the brains studied as compared with only 11% in which the right was larger. They concluded that this difference was likely related to the left hemisphere’s association with the production of language in most individuals. Subsequent studies have demonstrated that this asymmetry can be shown to present even prior to birth, reinforcing the genetic predisposition to left-hemispheric dominance for language. Since the advent of more sophisticated imaging techniques that allow for large-scale in vivo studies of the brain, other structural differences have been documented. The inferior frontal gyrus in the left hemisphere,

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corresponding to Broca’s area, has been shown to be more highly developed on the left side for most individuals. The gyri and sulci associated with the motor strip (Brodmann’s area 4) are more prominent in the left hemisphere of right-handers. Fairly consistent differences in the lateral fissure have been found, with the posterior ascending ramus of this sulcus making a more abrupt upward turn in the right hemisphere as compared with the left. This would suggest likely differences in the distribution of the supramarginal and angular gyri in the inferior parietal lobules of the two hemispheres. Even on a more microlevel, differences in the size and organization of individual cells or cell columns have been identified in the two hemispheres. It is reasonable to speculate that some structural differences likely relate to functional differences between the two hemispheres, particularly behaviors such as language and handedness. However, functional asymmetries have either been demonstrated or are suspected well beyond those which can currently be explained by structural differences. The following represent a sampling of some of the functional differences that have been observed.

Functional Asymmetries It has been well established that language expression and comprehension are normally mediated primarily, if not exclusively, by the left hemisphere, even among left-handers. However, the right hemisphere has also been shown to play an important role in communication. Verbal communication is not just about using words in sentences or paragraphs; emotional tone or nuances of the speaker often convey important meaning. In some communications, such as those with a sarcastic intent, the real message is carried by the tone rather than by the words, which, if interpreted literally, might actually convey a very different message. The ability to use as well as interpret these emotional components of speech, known as prosody, is primarily mediated by the right hemisphere; damage to this side of the brain may produce various forms of aprosodia. With regard to using or interpreting the language of others, the right hemisphere is also believed to play an important role in identifying the central theme or point of the discourse of others and being able to stay on point when speaking or writing. It appears to be important in appreciating verbal (as well as nonverbal) humor and in detecting meaning from the differential inflections given to individual words in speech. In addition to words, numbers also have their own symbolic meaning. Hence, as might be expected, the

ability to use numbers is thought to be a function normally carried out by the left hemisphere, the disturbance of which following a lesion to the left hemisphere may be defined as acalculia (dyscalculia). However, most complex arithmetical operations also have a spatial component. For example, precise alignment of rows and columns of numbers is critical in mathematical operations, whether completed mentally or on paper. These spatial relations can be disturbed following right-hemispheric lesions, resulting in what has been termed spatial dyscalculia. It is known that the hemisphere contralateral to the hand being used to carry out some motor tasks is immediately responsible for the execution of these movements. However, the motor programs or engrams for overlearned motor skills are believed to reside in the left hemisphere, certainly for the vast majority of right-handers, as well as many left-handers. Thus, any lesion that either directly interferes with those engrams or the ability of that information to reach the premotor cortex of either hemisphere can result in an impaired performance, especially if the individual is asked to demonstrate the action in the absence of the actual object. This latter condition is referred to as an ideomotor apraxia. Perceptual abilities appear to be differentially distributed between the two hemispheres. It has already been noted that the left hemisphere is normally the leading hemisphere in interpreting verbal (semantic) information, while the right appears to be better adapted to processing certain types of emotional cues. It seems that the right hemisphere is also the more proficient in processing many types of visual-spatial or visual-gestalt information. Thus, the right hemisphere has been found to be generally superior in carrying out certain constructional tasks, making judgments regarding the orientation of lines in space, in making discriminations regarding unfamiliar faces, and in recognizing familiar tunes or environmental sounds. On the other hand, the left hemisphere appears to be the leading hemisphere when it comes to perception of certain aspects of one’s own body. Problems of right–left orientation and difficulty recognizing individual fingers of one’s hands (finger agnosia) are typically associated with lesions of the left inferior parietal lobule. Functional MRI studies have demonstrated consistent activation of right hemisphere structures during tests of vigilance and directed attention. However, divided attention tasks have been shown to selectively activate left prefrontal cortex. PET imaging studies have demonstrated increased blood flow in the right prefrontal and superior parietal cortex during tasks requiring sustained attention, regardless of the type of stimulus (verbal, visual, etc.) or where it is introduced (left vs. right).

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Differences between the two hemispheres have also been demonstrated in learning and memory tasks and other cognitive domains. Of the two, the left hemisphere has been more strongly associated with learning verbal information. While many studies have shown that the right hemisphere is perhaps better at learning certain ‘‘nonverbal’’ or ‘‘visual-spatial’’ type information, the findings are generally less robust compared to the left hemisphere and verbal memory. One frequent explanation for this is that when faced with any memory task, humans have a natural tendency to try to verbally encode the stimulus, thus bringing the left hemisphere into play. It has also been suggested that the two hemispheres play different roles in attention. The much more frequent association of disorders such as unilateral neglect and anosognosia with right hemispheric lesions have led to the hypothesis that while, as might be expected, the left hemisphere attends to the right side of space (both personal and extrapersonal), the right hemisphere focuses on both right and left space. Finally, the association of the right hemisphere and emotional expression would appear to go beyond the affective intonations of speech as described above. It is commonly observed, both by health-care professionals as well as the spouses and other family members of persons with brain injury that individuals with right hemisphere lesions often behave differently than those with left-sided lesions. While the latter seem to remain emotionally attached, even if that emotion is often one of anger, frustration, or sadness, right hemispherically damaged patients are more likely to be described as apathetic, indifferent, emotionally flat, both in terms of their verbal and facial expressions and their interpersonal relationships.

Davidson, R. J., & Hugdahl, K. (Eds.). (1996). Brain asymmetry. Cambridge, MA: Bradford Books. Gazzaniga, M. S. (2000). The new cognitive neurosciences. Boston: MIT Press. Gazzaniga, M. S., Ivry, R. B., & Mangun, G. R. (2002). Cognitive neuroscience: The biology of the mind. New York: W.W. Norton & Company. Geschwind, N., & Levitsky, W. (1968). Left-right asymmetry in temporal speech region. Science, 161, 186–187. Good, C. D., Johnsrude, I., Ashburner, J., Henson, R. N. A., Friston, K. J., & Frackowiak, R. S. J. (2001). Cerebral asymmetry and the effects of sex and handedness on brain structure: A voxel-based morphometric analysis of 465 normal adult human brains. Neuroimage, 14, 685–700. Kinsbourne, M. (Ed.). (1978). Asymmetrical function of the brain. New York: Cambridge University Press. Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach. New York: Springer. Ross, E. (2000). Affective prosody and the aprosodias. In M. Mesulam (Ed.), Principles of behavioral and cognitive neurology. New York: Oxford University Press. Walsh, K. (1994). Neuropsychology: A clinical approach. New York: Churchill Livingstone.

Cross References

Definition

▶ Anosognosia ▶ Directed Attention ▶ Dominance, Cerebral ▶ Ideomotor Apraxia ▶ Language ▶ Unilateral Neglect ▶ Wada Test

Ataxia describes a lack of coordination while performing voluntary movements. It is associated with damage to the cerebellum or its afferent or efferent pathways. It may appear as clumsiness, inaccuracy, or instability. It may affect any part of the body. When ataxia affects the arms and hands, it may cause tremor due to overcorrection of inaccurate movements. It may produce dysmetria or an inability to gauge distance correctly. It may cause pastpointing when an attempted reach overshoots the target. It may also cause dysdiadochokinesia or poor performance of regular, repeated movements. Cerebellar injury may contribute to nystagmus, hyper and hypometric saccades, scanning speech, titubation, and difficulties with gait and balance.

References and Readings Corballis, P. M. (2003). Visuospatial processing and the right-hemisphere interpreter. Brain and Cognition, 53, 171–176. Davidson, R. J. (1992). Emotion and affective style: Hemispheric substrates. Psychological Science, 3, 39–43.

Ataxia A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Synonyms Clumsiness

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Current Knowledge

Neuropsychology

There are a number of different types of damage to the cerebellum. These range from fixed damage (e.g., stroke, trauma, hypoxic injury) to chemical and metabolic, and degenerative. Cerebellar injury related to vitamin deficiency (e.g., E, B12, and thiamine) may be reversible and should be identified and treated. Metabolic diseases such as Hartnup’s, Refsum’s, and the mitochondrial disorders are less treatable. A deficiency of Coenzyme Q10 has been described in patients with cerebellar ataxia, usually with childhood onset and often associated with seizures. The symptoms may respond to Coenzyme Q10 treatment. There are a number of hereditary cerebellar ataxias. Most of the autosomal recessive and autosomal dominant ataxias have no treatments. An exception is vitamin E deficiency, which is an autosomal recessive disorder. It is due to a mutation in the gene coding for the alpha tocopherol transfer protein located on the long arm of chromosome 8. It is characterized by childhood onset of ataxia, dysarthria, areflexia, proprioceptive deficits, extensor plantar responses, and skeletal deformities. The episodic ataxias, which are inherited in an autosomal dominant fashion, may also have some symptomatic treatment regimens. Episodic Ataxia Type 1 is related to a chromosome 12 mutation in the potassium channel gene. Clinically, the disease manifests with episodes of ataxia lasting seconds to minutes. Patients may also suffer from myokymia during and between attacks of ataxia. The ataxia may be induced by startle or exercise. Episodic Ataxia Type 2 is related to a defect located on chromosome 19. It is due to a mutation in a voltage-dependent calcium channel. Clinically, patients present with nystagmus. Attacks last minutes to hours and may be induced by a change in posture. Patients may also complain of vertigo. As the disease progresses, ataxia becomes permanent.

Cerebellar syndromes may be associated with cognitive slowing. Evaluation includes a detailed neurological examination, magnetic resonance imaging, and laboratory investigation for reversible or genetic causes. Treatment depends on the underlying insult.

Cross References ▶ Cerebellum ▶ Dysdiadochokinesia

References and Readings Gilman, S. (2004). Clinical features and treatment of cerebellar disorders. In R. L. Watts & W. C. Koller (Eds.), Movement disorders (2nd ed., pp. 723–736). New York: McGraw-Hill.

Atheromatous Plaque ▶ Atherosclerosis

Atherosclerosis E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Arteriosclerotic vascular disease or ASVD; Atheromatous plaque; Hardening of the arteries

Epidemiology Definition Ataxia is a common sign associated with inherited, acquired, toxic, and traumatic events.

Natural History The genetic syndromes that are associated with ataxia tend to be progressive. Individuals with static insults such as strokes or trauma may show improvement in function over time.

Atherosclerosis is the progressive pathological process of buildup of plaque inside the blood vessels, resulting in blockage of blood flow through the vessels.

Current Knowledge The plaque that causes atherosclerosis is comprised of fatty substances, cholesterol, cells, calcium, and fibrin, a

Atkins v. Virginia

stringy material found normally in the blood to help clot the blood. The plaque formation process stimulates the cells of the artery wall to produce substances that then accumulate in the vessel wall. Fat builds up within these cells and around them, and they form connective tissue and calcium. The artery wall thickens, the artery’s diameter is reduced, and blood flow and oxygen delivery are decreased. Plaques can rupture or crack open, causing the sudden formation of a blood clot (thrombosis). Atherosclerosis can cause angina or myocardial infarction if it blocks the blood flow in the coronary arteries that supply the heart muscle, stroke if it blocks the carotid arteries that supply the brain, kidney disease if it blocks the renal arteries or gangrene possibly leading to amputation if it blocks the peripheral arteries that supply the limbs. Atherosclerosis may be asymptomatic for many years. Risk factors for this disease have been well studied. It is thought that atherosclerosis is caused by a response to damage to the endothelium from high cholesterol, high blood pressure, and cigarette smoking. A person who has all three of these risk factors is eight times more likely to develop atherosclerosis than is a person who has none. Physical inactivity, diabetes, and obesity are also risk factors for atherosclerosis. Heredity, advancing age, and racial background are less-significant risk factors. Treatment options include lifestyle changes, use of lipid-lowering and other drugs, angioplasty, and various surgical procedures, depending on the location of the plaque. Many lifestyle changes that prevent disease progression also prevent the onset of the disease; these include a low-fat, low-cholesterol diet, weight loss, exercise, blood pressure control, diabetes management, and smoking cessation.

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References and Readings Stary, H. C., Chandler, A. B., Dinsmore, R. E., Fuster, V., Glagov, S., Insull, W., et al. (1995). A definition of advanced types of atherosclerotic lesions and a histological classification of atherosclerosis: A report from the Committee on Vascular Lesions of the Council on Arteriosclerosis, American Heart Association. Circulation, 92, 1355–1374.

Atherosclerotic Dementia ▶ Vascular Dementia

Atherosclerotic Heart Disease ▶ Coronary Disease

Atherothrombotic Brain Infarction ▶ Ischemic Stroke

Athymia ▶ Abulia

Atkins v. Virginia Cross References ▶ Angioplasty ▶ Anticoagulation ▶ Antiplatelet Therapy ▶ Atherosclerosis ▶ Cerebrovascular Disease ▶ Cholesterol ▶ Coronary Disease ▶ Ischemic Stroke ▶ Myocardial Infarction ▶ Peripheral Vascular Disease ▶ Stent ▶ Thrombolysis ▶ Thrombosis

R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Mental retardation defense

Historical Background Daryl Atkins and William Jones abducted Eric Nesbitt from a convenience store and after finding only $60 in

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his wallet, Atkins and Jones used Nesbitt’s vehicle to drive to an ATM and forced him to withdraw $200. Thereafter, Atkins and Jones drove Nesbitt to an isolated location where he was shot eight times and subsequently died. Atkins and Jones were quickly tracked down by police and in custody, it was determined that Jones’ story claiming that Atkins pulled the trigger was more coherent than Atkins’ story implicating Jones as the shooter. Thus, Atkins was charged and convicted of abduction, armed robbery, and capital murder. This took place despite the results of an IQ test completed by a clinical psychologist, in which Atkins’ score of 59 placed him in the mildly mentally retarded range. Nonetheless, he was sentenced to death. The Supreme Court of Virginia was in agreement with the judgment of the trial court and the appeal was taken to the U.S. Supreme Court. In July of 2002, the U.S. Supreme Court reversed the judgment of the trial court and the Supreme Court of Virginia and referred the case back to the sentencing court to render a sentence other than the death penalty. The U.S. Supreme Court ruled that the punishment was excessive and thus prohibited by the eighth Amendment as cruel and unusual if it is not ‘‘graduated and proportioned to the offense’’. An excessive judgment is judged by current societal standards. Thus, society’s standards of decency, albeit subject to change, must prove that they are influenced by ‘‘objective factors to the maximum possible extent.’’ Furthermore, it was ruled that mental retardation does not preclude a person’s capability to discriminate right from wrong, though mental retardation does lead to a diminished capacities to process and comprehend information, reduces communication abilities, and decreases one’s ability to learn from mistakes and experiences, reason logically, inhibit impulses, and understand the emotions and behaviors of others. The U.S. Supreme Court concluded that mentally retarded individuals are not exempt from criminal sanctions, though a decrease in personal culpability is warranted. Thus, due to the conclusions that the purposes of retribution and deterrence are not accomplished in the execution of mentally retarded individuals, coupled with the increased risk that the death penalty will be imposed erroneously; the U.S. Supreme Court ruled that the eighth Amendment precludes execution of mentally retarded persons. Despite the court’s ruling, in July of 2005 a Virginia jury determined that Atkins was intelligent enough to be executed due to the fact that another IQ score had been recorded at above 70. Moreover, the prosecution claimed that his poor performance in school was related to use of alcohol and drugs and that earlier assessments of his IQ

were ‘‘tainted’’. Thus, Atkins was set to be executed on 2 December 2005. However, the decision was recently reversed again by the Virginia Supreme Court, as a result of state procedural grounds.

Current Knowledge Forensic psychological and neuropsychological assessments of mentally retarded individuals being considered for the death penalty are highly important. Specifically, the U.S. Supreme Court did not specify the degree of mental retardation required to circumvent the death penalty and instead left such determinations up to the discretion of the states. It is important to note that sub-average intelligence alone does not warrant a label of mental retardation. Impairments in adaptive functioning and evidence of mental retardation prior to the age of 18 years are needed for a diagnostic determination of mental retardation.

Cross References ▶ Intellectual Disabilities ▶ Intelligence

References and Readings Atkins v. Virginia, 153 L. Ed 2d 335 (2002). Cunnningham, M. D., & Goldstein, A. M. (2003). Sentencing determinations in death penalty cases. In A. Goldstein (Ed.). Forensic psychology (Vol 11). Handbook of psychology. New Jersey: Wiley. Denney R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.). Forensic neuropsychology: a scientific approach. New York: Oxford University Press. Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts: A handbook for mental health professionals and lawyers (3rd ed.). New York: Guilford Press.

Atomoxetine J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Atomoxetine

Atrophy

Brand Name Strattera

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Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.com: 8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Class Norepinephrine reuptake inhibitor

Proposed Mechanism(s) of Action Inhibits the presynaptic reuptake of norepinephrine and theoretically increases dopamine in the prefrontal cortex via the same mechanism.

Indication Attention Deficit Hyperactivity Disorder.

Atrophy J OA NN T. T SCHANZ Utah State University Logan, UT, USA

Synonyms Degenerative; Wasting

Definition

Off Label Use Treatment resistant depression and anxiety.

Atrophy refers to loss of cells of any tissue. In the brain, atrophy refers to a loss of neurons that may be generalized (e.g., diffuse atrophy) or focal, reflecting

Side Effects Serious Increased cardiac rate, potential hypertension, orthostatic hypotension, rare liver damage, potential for induction of mania, and suicidal ideation.

Common Sedation in children, decreased appetite, dry mouth, constipation, nausea, vomiting, dysmenorrhea, erectile dysfunction, and impaired libido.


Additional Information Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com

Atrophy. Figure 1 Display of diffuse atrophy of the cerebral hemispheres. Note the shrunken gyri and prominent, widened sulci (Photo courtesy of Steven S. Chin, M.D., Ph.D., University of Utah Health Sciences Center)

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circumscribed regional cell loss. With atrophy, there is also a corresponding loss of neural connections (synapses). Features of atrophy include sulcal widening, shrunken gyri, and enlarged ventricles. Focal atrophy may occur as a result of trauma or cerebrovascular lesions, for example. Generalized atrophy may occur with neurodegenerative conditions such as Alzheimer’s disease. Atrophy may be viewed on gross inspection of the brain postmortem or antemortem with structural imaging techniques such as MRI or CT scan. Figure 1 displays diffuse brain atrophy of the cerebral hemispheres viewed from the top.

adequate training of an attendant, and financial issues including paying a competitive wage to personal attendants. Funding sources of attendant care may include private resources such as: Health Insurance; Auto Insurance; and Worker’s Compensation; or public resources such as: Medicaid; Department of Vocational Rehabilitation; Department of Veterans Affairs; Crime Victims Compensation and/or other State-funded programs.

Cross References ▶ Assisted Living

Attendant Care A MY J. A RMSTRONG Virginia Commonwealth University Richmond, VA, USA

Definition Attendant care involves the provision of services to assist individuals with mental and/or physical disabilities in the performance and/or conduct of activities of daily living in order to maximize community inclusion and independent living. The intent of attendant care is to promote independence, participation, and quality of life of the individual while also preventing medical problems. Typically, the individual receiving attendant services is unable to perform such tasks independently, or may perform them with great difficulty. Attendant services include, but are not limited to activities such as: bathing, dressing, feeding, toileting, transferring, mobility, cooking, cleaning, laundering, cognitive assistance and monitoring. Services may also relate to sustaining health such as dispensing medications etc. Attendant care may be provided by a family member such as a spouse, partner, sibling or parent, or by a hired employee. Typically, attendant care is provided by persons who have been trained to provide the service/s within the home and/or community. The independent living model of attendant care contends that individuals with disabilities should be empowered, to the highest degree possible, to recruit, screen and hire, train and terminate their respective personal attendants, thereby ensuring self-determination and choice. Additional considerations related to the provision of attendant care include where and how to locate and access quality service providers,

References and Readings Rodriguez-Banister, K. (2006). The Personal Care Attendant Guide: The Art of Finding, Keeping, or Being One. New York: Demos Medical Publishing.

Attention R ONALD A. C OHEN Brown University Providence, RI, USA

Synonyms Concentration; Focus; Vigilance

Definition Cognitive processes that enable the selection of, focus on, and sustained processing of information. The object of attention can either be environmental stimuli actively being processed by sensory systems, or associative information and response alternatives generated by ongoing cognitive activity.

Historical Background Attention is subjectively self-evident to all people, and terms that referred to attention-type experiences have been described by philosophers through the ages. The concept of attention is strongly linked in the philosophy

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to the nature of consciousness, self-awareness, and most theories of the ‘‘mind.’’ Accordingly, attention has been the subject of psychological inquiry from the beginning of this scientific discipline. The writings of William James captured this fact, as evident from this well-known excerpt from his Principles of Psychology (1898). " Everyone knows what attention is. It is the taking posses-

sion by the mind, in clear and vivid form, of one out of what seem several simultaneously possible objects or trains of thought. Focalization, concentration, of consciousness are of its essence. It implies withdrawal from some things in order to deal effectively with others

While written over 100 years ago, this description very succinctly captures essential aspects of the phenomena of attention, and remains apropos even today. The understanding of cognitive, behavioral, and neuropsychological bases, influences, and effects of attention have dramatically evolved since the time of William James. Yet, the underlying subjective and behavioral experiences characterized by James and the other psychologists of his time remain largely consistent with current phenomenology of attention. Different types of attention were described, such as directed, divided, focused, sustained, selective, and volitional attention, many of which continue to be used to describe the varieties of attentional experience. The primary limitation of these early efforts was the lack of experimentation that would have enabled operationalizing of these constructs and understanding of the processes underlying them. Following the initial efforts of early psychologists to study attention from perspectives of structuralism and functionalism, a rather long period ensued dominated by behaviorism during which cognitive processes, like attention, were largely viewed as outside of the realm of empirical psychological inquiry. Attention was considered to be a construct that could be explained by more basic behavioral principles, such as discrimination learning, cue dominance, anticipation, and expectation. Classical conditioning theory provided an essential framework for behavioral analysis of attention, as the orienting response to novel stimuli, and its subsequent habituation, provided the behavioral and neural building blocks for simple forms of attention, in the absence of long-term memory formation (i.e., conditioning). The concepts of motivation and drive which played a major role in neobehaviorism, also helped to bridge attention theory and learning principles. Information theory (Shannon & Weaver, 1949), which evolved out of technological advances in radio communication and also radar detection during World War II, was a major impetus for the subsequent re-emergence of

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cognitive science. In particular, the application of information processing models and signal detection methods to the study of communication led to a resurgence of research interest in attention. This is not surprising considering the fact that signal detection and selection, the basic elements of almost all theories of selective attention, are also central to information and communication theory. This approach emphasized the probabilistic nature of information detection and selectivity, a conceptual departure from earlier psychophysical methods used to study perception. The application of information processing approaches to the study of attention was a logical step, as one of the primary problems for any communication or information processing system is reducing the total amount of incoming signal to manageable levels to enable subsequent processing of this information. Broadbent (1958) proposed the first formal model of selective attention based on the information processing theory. He maintained that attention occurs because there is an information ‘‘bottleneck’’ as the large quantity of environmental information that is available during parallel processing is subject to channel capacity limitations later in the stream of processing due to serial processing constraints. Broadbent posited that the primary requirement for attention to occur was a filtering process (as shown in Fig. 1) that occurred soon after sensory registration and served to separate relevant from irrelevant signals in order to enable meaningful information to be available for subsequent serial processing through limited capacity information channels. This model presumed a somewhat passive system by which this filtering occurred, with selection driven by the salience of the stimuli themselves. However, the nature of this filtering process was not fully operationalized in this initial model. In the years that followed, a number of variations on this model of attention were proposed by other investigators working in the newly emerging field of cognitive psychology. Most notably, Treisman proposed an attenuation theory of attention, which was similar to Broadbent’s model in which he postulated a single process that occurred early in the information processing stream, soon after sensory registration (Treisman & Gelade, 1980). According to attenuation theory, selective attention requires distinguishing between messages on the basis of their physical characteristics, such as location, intensity, and pitch, as well as content. In this model of attention, stimuli naturally differ in their threshold for activating awareness of a stimulus, and the process of attention effectively decreases (i.e., attenuates) the strength (e.g., loudness) of irrelevant stimuli. This attenuation process was considered to occur in conjunction with a feature integration process that enabled

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Messages

Sensory store

Filter

Detector

To memory

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Attention. Figure 1 Model depicting filtering process as proposed by Broadbent (1958)

perceptual experience. This line of research was noteworthy for the use of dichotic listening paradigms in which attention is divided between the two ears, and information must be selected from one of the two channels of input. The bottleneck models proposed by Broadbent and Treisman proposed that selection occurs at a very early stage of processing soon after sensory registration, thereby linking attention squarely with sensory selection. Essentially, selective attention filters inhibits focus on information occurring in the unattended ear in dichotic listening experiments before semantic analysis and other cognitive processes have time to occur. Other investigators (e.g., Deutch and Deutch (1963) argued that attention is strongly influenced by the response demands of a situation and that it likely occurred at a later stage of processing, and that in reality both ears analyze incoming information semantically, though response demands creates a bias toward one ear over the other. This led to heated debates in the 1960s and 1970s over the location of the bottleneck. While considerable experimental evidence indicated that early sensory selection occurs prior to a point in time when semantic information has been processed, there is also other paradigms that demonstrate that in most situations selection is greatly influenced by semantics and the response requirements that exist. Subsequent researchers took this a step further by demonstrating that capacity limitations constrain attention and the intensity of attentional focus that is possible at any given point in time. Kahenman’s (1983) capacity theory of attention proposed that people’s capacity for attentional focus is not static, but instead varies as a function of factors such as the reward characteristics of the task, arousal level, and other biological determinants. This theory of attention was extremely important in that it brought to the forefront the fact that attention should not be conceptualized in purely mechanical terms as was the case in early attention models based solely on information processing theory. Rather attention needed to be viewed in the context of the biological factors that drive it. This helped to catalyze an emphasis on the study of focused attention, a shift that coincided with information coming from psychophysiological studies that showed

linkages between arousal, activation, and effort in the control of attention (Pribram & McGuinness, 1979). A large body of cognitive studies of attention followed this pioneering work. Several of these are particularly important in a historical context. Posner (1979) made an important distinction between overt and covert shifts of attention that occur in the context of visual selective attention. Overt attention is characterized by the act of intentionally directing attention (i.e., looking) toward a stimulus, whereas covert attention occurs without intention when focus is drawn to a particular stimulus or location, typically as a result of cues or other types of information of which the person may have little conscious awareness. Posner also refined the use of chronometric methods to demonstrate the costs associated with these attention shifts. His research also made a distinction between two primary processes, selection and focus, that were necessary to account attention’s intensity and spatial distribution. Shiffrin and Schneider (1979) conducted seminal studies that distinguished automatic from controlled attention. They varied the number of targets to be detected and the consistency of target location based on either fixed or variable memory demands. By creating greater variability in task characteristics, subjects could not rely on memory to facilitate performance, which slowed their response time. Under these conditions, automaticity was no longer feasible. Besides demonstrating the distinction between automatic and controlled attention, these findings also illustrated the relationship between attention and memory, and set the stage for a long line of research examining working memory in relationship to attention.

Neuropsychological Models and Frameworks Over the past 2 decades, research efforts have been directed at organizing these varieties of attention into coherent frameworks. Furthermore, researchers have proposed neuropsychological models of attention that seek to characterize the functional neuroanatomic systems involved in attention, the processes for which these systems are

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responsible, and also how these functional brain areas interact. The models described below are not meant to be an exhaustive review of the literature in this regard, but rather highlights some of the key elements of current theoretical frameworks and the extent to which there is consistency across models. Alan Mirsky provided one of the first neuropsychological frameworks to account for what he described as the ‘‘elements’’ of attention. This framework proposed five elements of attention: (1) selection, (2) focus, (3) execute, (4) switch, and (5) sustain. This theoretical framework was derived from factor analyses of neuropsychological test results obtained from a large sample from his clinical practice. Cohen (1993) proposed a similar component process framework of attention that hypothesized four primary components of attention (Fig. 2): (1) sensory selective attention; (2) response intention, selection, and control; (3) capacity-focus; and (4) sustained attention. A primary goal of Cohen’s model was to include components reflecting similar levels of analysis. The components of this framework were also derived from factor analysis of clinical neuropsychological data with efforts made to retain only the minimum number of factors necessary to account for maximum variance in the data, with an effort to make conservative interpretation of the component processes associated with each factor. Each component was hypothesized to be a function of other more basic subcomponent processes. This model posits that these four components of attention are not completely orthogonal or functionally independent, but instead rather share common component subprocesses, processes depending on the task at hand. A simplified version of the model is shown. In everyday situations, attention depends on the interaction of all four of these component processes. However, for some tasks, the primary demand may be for sustained attention or vigilance, whereas for another task it may be

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efficient use of available attention capacity and the intensity of focus. Similarly, some tasks that are weighted more demand for sensory selective attention, while others place greater demand on response intention and selection. In other words, while these four components need to be accounted for in explaining attention across all situations, particular tasks may require minimal demands for sustained attention, but intense demands on capacity and focus. Validation efforts directed at this framework have shown the principal factors to be highly reliable, internally consistent, and valid with respect to their weighting relative to specific brain disorders and conditions. For example, patients with attention-deficit disorder have greatest impairment on tasks requiring sustained attention, whereas patients with diminished speed of processing have greatest problems on tasks requiring capacity and focus. It is noteworthy that the analyses conducted by both Mirsky and Cohen yielded very similar factor structures and validity data, providing strong evidence that four to five primary components processes exist that account for most varieties of attention. These attentional component processes are described in greater detail below.

Selective Attention A fundamental aspect of all attentional processes is that it is selective. Attention enables the selective deployment of cognitive resources for the processing of information from either the external environment or internal cognitive processes or associative representations. Attention also requires a shift from less salient information. Processes that enable or facilitate the selection of salient information for further cognitive processing are collectively referred to as selective attention (Treisman, 1969; Triesman & Geffen, 1967). Individuals are constantly flooded with an infinite number of signals from both outside and within. By reducing

Attention. Figure 2 Simplified neuropsychological model of the components of attention

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the amount of information that will receive additional processing, attention constrains incoming information to the individual’s available capacity at a given point in time, thereby keeping the level of information to be processed at a manageable level. While selective attention is necessary and beneficial for cognitive function, there are costs associated with selectively attending. By attending to a particular stimulus, the likelihood of detecting other potentially relevant stimuli or choosing an alternative response strategy is reduced. Optimal selective attention depends on the system being flexible and adaptive, with the capacity to select and focus on certain stimuli, but then to shift to other stimuli or cognitive processing when task conditions change. Selective attention thereby serves as a gating mechanism for the flow of information processing and the control of behavior.

Response Selection and Control Attention has traditionally been viewed as a process closely related to perceptual processing. It prepares the individual for sensory intake, perceptual analysis, and integration with other cognitive processes. Yet, there are many situations in which attention is not directed at incoming sensory information, but rather selection response alternatives, and the control of responding once a selection has been made. Even when a task primarily requires selective attention, there are usually coexisting response demands. While sensory selection may be automatically elicited by the occurrence of salient external stimuli, more often than not the act of attending is linked to a planned, goal-directed course of action. In this regard, attention and responding are directed to obtain information that will optimize behavior. The processes associated with response selection and control range from simple behavioral orienting, such as turning one’s head in response to a sound source to more complex cognitive processes involving intention, planning, and decision making. Response selection and control form the basis for what humans typically experience as volitional action. Before responding, individuals generate a large number of response alternatives. These response alternatives are evaluated prior to making an actual motor response, leading to response bias, that is, the probability of selecting specific responses. The attentional processes involved in response selection and control are related to a broader class of cognitive processes, commonly referred to as executive functions (Fuster, 1989; Luria, 1966). Several processes associated with response generation underlie executive control: intention, selection, initiation, inhibition, facilitation, and

switching. Not only do these processes account for the control of simple motor responses, they also provide the foundation for more complex cognitive processes, such as planning, problem solving, and decision making, as well as conceptual processes such as categorization, organization, and abstraction. Executive control is strongly dependent on the actions of prefrontal-subcortical systems. Executive control is dependent on the ability of the system to act with intention, to initiate responding, to inhibit responding based on new information, and to efficiently shift from one response alternative to another in accordance with changing environmental demands.

Focused Attention and Capacity Limitations Attention is also characterized by having intensity and by the extent to which it is allocated in either a focal or diffuse manner. The intensity of attentional focus is a function of both situational and task demands and organismic factors, such as motivation and drive. Focused attention is constrained by capacity limitations (Kahneman, 1973) that limit the intensity of focus that is possible on a moment-by-moment basis. Attentional capacity is influenced by both structural and energetic factors (Cohen, 1993). Energetic capacity limitations tend to be state dependent and reflect the changing energetic conditions of the brain, including motivation, and the incentives to attend that are present in the situation. Structural factors tend to be more stable and dependent on each person’s intrinsic information processing capacity. Factors that influence structural capacity include the processing speed capacity that is a function of the integrity of neural transmission, memory encoding, storage and retrieval limitations, and temporal–spatial processing dynamics that vary across people. Given these factors that limit attentional capacity, focused attention varies relative to the cognitive demands and type of information to be processed, and situational incentive. Focused attention can occur relative to either sensory selective attention, or intention and response selection, and in fact often involves the coordination of sensory and response selection. Such coordination is quite effortful (Pribram & McGuinness, 1975). Arousal and activation vary as a function of the existing demands for focused attention, with greater activation occurring when there is more utilization of available capacity because of the need to focus.

Automatic Versus Controlled Attention An important distinction exists between automatic and controlled attentional processing (Schneider & Shiffrin, 1977;

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Hasher & Zacks, 1979). Automaticity occurs most commonly in the context of sensory selective attention, particularly for tasks requiring simple detection of a target from a set of stimuli, and also on tests of attention span. With automaticity, there is usually relatively little demand placed on attentional capacity, and often attention can occur without much awareness or subjective effort (e.g., attending to other cars while driving on an empty highway). Automaticity can be interfered with increase in size and complexity of the environment to be attended to. Spatial selective attention is particularly well suited for automatic attentional processing since visual information typically occurs in parallel with a vast array of information reaching the brain almost instantaneously. Automaticity is more difficult to achieve for tasks that require sequential cognitive operations, though some degree of automaticity may be attainable through practice. Controlled attention is typically required for tasks in which there are working memory demands, or other requirement of other cognitive processes, such as memory encoding and retrieval, rapid processing speed, or executive control. The demand for focused intensity varies as a function of requirements for controlled attention that exist for a particular task. Generally, response intention, selection, and control are not very amenable to automatic attentional processing, as behavioral responding typically requires complex motor sequencing with executive control demands. However, the fact people are able to perform certain tasks such as typing or playing a musical instrument with considerable automaticity illustrates that automaticity is attainable for welllearned motor programs.

Sustained Attention Attention varies as a function of the temporal dynamics of the task to be performed and the situation, and all humans experience some degree of performance variability, particularly when long periods of sustained performance are required. Sustained attention refers to processes that enable the maintenance of performance over time. Compared to other cognitive processes, such as language and visual perception, attention is inherently variable by necessity, as it must be responsive to changing stimulus conditions, task demands, and motivational and energetic states. Problems with sustained attention commonly occur on tasks requiring attentional persistence for long durations when there are high levels of demand for effortful processing. All people have limits in their capacity for sustained attention. Sitting in a 1-hour lecture is not a problem for most bright college students, but even the

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brightest students would encounter tremendous difficulties sustaining their focus for a lecture that lasted 12 consecutive hours. Vigilance refers to sustained attention directed toward specific targets, in which a state of readiness is required to detect and respond to stimuli occurring at variable and often infrequent intervals (e.g., Colquhoun & Baddeley, 1967; Corcoran et al., 1977). Detecting rare targets with lengthy intervals between responses can be difficult. This type of sustained attention is quite common in everyday life. For example, a watchman may spend the entire night attending to the possibility of an intruder without this event ever occurring. Attention to low-frequency events has different processing requirements than responding to highfrequency events and, for many people, is more difficult. Vigilance and sustained attention are under the influence of sustained motivational level, boredom, and fatigue, which are sensitive to the dynamics of temporal tasks.

Current Clinical and Experimental Evidence Neuropsychological Studies Twenty years ago the clinical and experimental neuropsychological literature on impairments of attention associated with neurological and psychiatric disorders affecting the brain was quite limited. Much of the neuropsychological focus on attention was on the assessment of attention span in the context of psychometric analysis of performance on tests such as digit span. This probably reflected the fact that adequate attention was once viewed more as a necessary condition for other cognitive functions to occur optimally, but not particularly important in its own right in considerations of brain–behavior relationships. This attitude has changed dramatically, and attention is now widely regarded as a critical cognitive process that reflects not only the interface between both the external environment and internal cognitive functions, but also moment-by-moment information processing. A literature review conducted about 2 decades ago revealed less than 500 studies focused on the neuropsychology of attention. Recent literature reviews suggest that this number is now over 40, 000. This increase in interest in attention reflects the fact that: (1) attention disturbances are one of the most common by-products of brain, (2) attention is closely tied to the human experiences of consciousness, awareness, and cognitive control, (3) major advances have occurred in the methodology for studying and assessing attention, and (4) for a number of reasons, there has been an increased societal interest in

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attention disturbances, perhaps in part because of the intense information processing demands and pace of life that people now experience. Impairments of attention may be either specific or nonspecific. Specific impairments occur when only aspect of attention is affected. Often this occurs when impaired attention is directly associated with a particular type of cognitive operation, such as spatial processing. These impairments are often associated with focal brain disturbances affecting specific cortical or subcortical systems necessary for the cognitive operation. Nonspecific disturbances of attention are much more common, often occurring due to disorders that affect arousal, motivation, or other factors that reduce attentional capacity, and as a result of more diffused nonlocalized brain disorders. Both types of attentional disturbance provide insights into the cognitive processes of attention and the brain mechanisms that underlie these processes. Though localized lesions provide the best vehicle for analysis of the role of specific brain structures in attentional control, nonspecific attentional impairments illustrate the influence of metabolic and neurotransmitter abnormalities on information processing rate, arousal, and other energetic and structural factors that may affect attentional capacity and focus. Attentional capacity is a direct function of level of consciousness, making this an essential part of the clinical assessment of attention. Levels of consciousness range from normal states of alertness and awareness to coma. A brief summary of the attentional disturbances associated with several common neurological and psychiatric conditions is provided below. For more detailed consideration see Cohen (1993).

Stroke and Neglect Syndrome Unilateral stroke affecting the nondominant cortex often causes hemi-neglect syndrome, perhaps the most well known and dramatic form of attention disturbance. The defining feature of neglect syndrome is the failure to attend to, respond to, or be aware of stimuli on one side of space. Many variants of neglect syndrome may be observed clinically. Most patients with neglect exhibit impairments of sensory selective attention, although some may have primary problems with hemi-spatial response selection and control. Experimental investigations have confirmed the role of attention in hemi-neglect syndrome. Manipulation of attentional parameters demonstrates that symptoms of hemi-neglect change as attentional demands are modified (Kaplan et al., 1989). Regardless of which attentional process is most affected, all patients with neglect syndrome have a fundamental disorder involving the spatial distribution and allocation of attention.

Alzheimer’s and Neurodegenerative Dementias Attention disturbance is usually not described as a primary feature of Alzheimer’s disease (AD) and historically tended to be viewed as one cognitive function that was largely spared. This conclusion is misleading. While patients with early-stage AD typically do not show overt symptoms of severe inattention, they frequently have marked difficulty with focused attention and executive control, particularly when tasks required controlled attentional processing. This reflects a distinction between performance on tests of simple and complex attention, as conclusions about spared attention in AD have often been based on the observation of preserved attention span on tests such as digit span. Patients with early AD are often usually alert, energetic, and able to maintain their general focus on the assessment process. Yet, most will have considerable difficulty on tasks requiring focused and divided attention, suppression of interference (e.g., Stroop), information processing speed and efficiency (e.g., Symbol Coding), and working memory. As the disease progresses, performance becomes impaired on most tasks requiring effortful attentional processing. Pervasive disturbance eventually develops affecting all aspects of attention, including self-awareness.

Multiple Sclerosis Multiple sclerosis (MS), one of the most common neurologic in young adults, often affects learning, memory, and executive control. Given the fact that the disease affects the myelin of the white matter, deficits in these areas are often strongly associated with attentional impairments and slowed inefficient information processing. Attentional capacity is typically reduced with performance decrements usually evident under conditions of increased informational load. Fatigue is the most common of all symptoms in MS and is associated not only with motor effort but also with attending to and performing cognitive tasks. Patients with MS experience difficulty maintaining consistent effort on tasks.

HIV Similar to MS, HIV-infected patients who have not developed severe AIDS dementia typically show primary impairments in the areas of psychomotor and information processing speed, focused and sustained attention, and executive functioning. This reflects the fact that when not adequately treated, HIV tended to initially have greatest effects on subcortical systems, including the basal ganglia.

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Closed Head Injury The most common effects of closed head trauma are diffuse axonal damage due to shearing forces and frontal lobe disturbances. Consequently, attention and executive dysfunction are among the most common associated cognitive problems. Persistent distractibility, poor concentration, apathy, and fatigability are prominent sequelae. Deficits of arousal and poor performance on measures of selective, focused, divided and sustained attention, processing speed, and executive functioning tend to occur, which may contribute to associated learning and memory retrieval problems as well.

Epilepsy Transient changes in the level and quality of consciousness, common in seizure disorder, typically cause marked alterations in attention around the time of the seizure. During the time between seizures, patients with epilepsy may have greater problems than healthy individuals on tests of focused and divided attention. These deficits appear to be related in part to slowed speed of processing and its effects on attentional capacity. Pharmacological effects associated with anticonvulsant therapy likely contribute in part to these attentional effects.

Metabolic Disturbances Factors that affect the metabolic function often cause delirium, or more subtle alterations in attention and arousal. Accordingly, metabolic disturbance is one of the most common reasons for transient alterations in attention among people without other neurological or psychiatric illness. Metabolic disturbances that affect the brain can be the result of a wide variety of factors, including drug effects and systemic illnesses, such as liver and kidney disease, and diabetes.

Psychiatric Disorders Difficulties with focused and sustained attention are extremely common among patients with psychiatric disorders, including affective disorders (major depression and bipolar disturbance) and schizophrenia. Severe anxiety states can also interfere with attention. A strong relationship exists between expenditure of effort and performance on tests of attention and other demanding cognitive functions for patients with major affective disorders. Impairments tend to be somewhat proportional to severity of depression, with performance improving when the depression resolves. Diminished attentional capacity is particularly evident on tasks that require psychomotor speed, attentional focus, and effortful demands for mental control. Abnormal attention is also a central feature of schizophrenia, as filtering of

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irrelevant stimuli and thoughts has long been considered to be a major element of the disorder, which has been linked to the dopamine system. Schizophrenics often encounter difficulties on tests of sensory selective attention because of their susceptibility to distraction. Slowed reaction time and processing speed also contribute to problems with attentional capacity, and both focused and sustained attention (Nuechterlein, 1977).

Attention-Deficit Disorder (ADD) A developmental disorder of attention, ADD is the most widely recognized of all attention disturbance. Problems with sustained attention and distractibility are key features of the disorder, along with hyperactivity among a subset of children. While there is general agreement regarding the existence of ADD, there continues to be considerable debate about its manifestations and pathophysiology, particularly in light of the fact that ADD tends to occur along with other comorbid conditions.

Primate Studies Understanding of the neural substrates of attention was greatly enhanced by the use of neurophysiological methods in primates. The value of these studies is that they provided directed recording of electrical activity from brain areas implicated in attention both by past clinical studies of patients with neurological disorders and also ablation studies involving laboratory animals. In the 1970s, Robert Wurtz, Michael Goldberg and their colleagues (1982) began electrophysiological studies from the brain of monkeys trained on specific attention paradigms. The earliest of these studies showed that the superior colliculus exhibits increased firing rates during visual attention, providing the first direct evidence of the involvement of a neural area in this process. Subsequently, a large number of studies were conducted that showed the contribution of other brain regions, particularly in posterior visual areas to specific aspects of visual selective attention. This work both confirmed the role of areas like the inferior parietal cortex that had been suspected of being involved in visual selective attention based on studies of hemi-neglect syndrome. Over time, there has been increasing emphasis on the role of frontal brain systems in relationship to these posterior brain areas. There is now a large body of research on this topic, supporting to general conclusions about selective attention: (1) Visual selective attention is controlled by multiple interacting brain areas that comprise a functional system. (2) Selective attention involves not only

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posterior visual brain areas, but also frontal–striatal systems that provide executive response control. Active investigation continues using primate models with particular emphasis on source analysis of how particular types of neurons are tuned to optimize attention to particular types of signals. This research has been instrumental in characterizing the functional brain systems governing attention in humans.

Functional Neuroimaging Attention was one of the first cognitive functions to be demonstrated through the use of functional imaging methods, such as functional MRI and PET. The fact that attentional parameters can be easily manipulated in the context of the scanner and that attention reflects the moment-bymoment information processing of the brain makes it very conducive to study through functional brain imaging. These efforts have largely confirmed the involvement of inferior parietal and frontal brain systems in attention, with studies showing the relative contribution of specific areas in selective, focused, and sustained attention. This is a rapidly growing area of neuropsychological inquiry. To date, results of functional neuroimaging experiments have largely supported evidence from earlier cognitive, neuropsychological, psychophysiological, and primate studies with respect to the neural substrates of attention.

Clinical Assessment Considerations Although an essential cognitive process, attention is more difficult to directly observe or measure than other cognitive functions like language, visual perception, or memory. Attention fluctuates in accordance with changes in task demands and the processing capacity of the patient over time. Unlike other cognitive functions, performance may be quite different across different points in time, and it is this variability that in fact defines attention. Attention is often situation specific. This accounts for why some children with ADD perform well in a controlled laboratory setting, despite reports of gross problems with inattention in school or the home. Unlike most other cognitive processes, attention primarily serves a facilitative function. Attention enhances or inhibits perception, memory, motor output, and executive functions, including problem solving. Yet, attention is always measured as a function of performance on tasks that also loads on one or more of these other cognitive domains. Therefore, pure tests of attention do not exist,

and attention usually must be assessed within the context of performance on tasks that load on one or more these other domains. Attentional performance is often assessed as derived measure obtained by comparing performance across tasks that control for key attentional parameters (e.g., target–distractor ratio). Absolute performance often provides less informative measures of performance inconsistencies in the assessment of attention. For example, how performance varies as a function of time, spatial characteristics, or memory load provides more information about attentional dynamics than simply considering total errors on a visual detection task. Since attention is not the by-product of a unitary process, or a single sensory modality, it cannot be adequately assessed on the basis of findings from one specific test. For example, conclusions based on digit span performance are misguided. Attentional assessment requires a multifactorial approach. The specific attention measures used in an evaluation depends on the overall level of functioning of the particular patient. For patients with global cognitive dysfunction, it may be difficult to use certain tasks that require overly complex responses. For patients with relatively high overall cognitive abilities, tasks should be chosen that require multiple component processes. If the patient is able to perform well on these tasks, then severe attention disturbance involving specific attentional component processes can be ruled out. The Stroop and TrailMaking tests are examples of tasks that require multiple attentional processes. If impairments are found on such tasks, then more extensive testing of specific component processes can be conducted. When possible, efforts should be made to use tasks that incorporate signal detection methods, even when not evaluating sensory selective attention per se. This methodology provides the best means of accurately summarizing performance relative to all types of possible errors, and easily integrates with response time measures. Attentional parameters that should be considered. A thorough assessment of attention should be based on analysis of data from a comprehensive battery of attentional tests that sample underlying component processes (Cohen, 1993). Accordingly, tasks should be used that are differentially sensitive to the following attentional parameters: (1) spatial characteristics, (2) temporal dynamics, (3) memory demands, (4) processing speed requirements, (5) perceptual complexity, (6) demands for response sequencing and control, (7) cognitive complexity of the task, (8) effort required to complete task, and (9) task salience, relevance, and reward value. While multifactor neuropsychological assessment provides the best means of evaluating attentional

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impairments, a comprehensive attentional evaluation may not be feasible in everyday clinical practice, because of time constraints, the patient’s overall severity of cognitive impairment, or the fact that other cognitive functions must be assessed in greater detail because of the referral questions. Consequently, clinicians should be aware of the information that can be obtained from different levels of attentional assessment. A few primary tests of attention should be included in all neuropsychological evaluations. The continuous performance test (CPT) paradigm provides an excellent measure for assessing sustained attention and other related indices. Tests of focused and selective attention are also now widely available. Several attention batteries have also been developed that may facilitate the comprehensive assessment of the elements of attention.

Future Directions Real-time functional brain-imaging methods will enhance the ability of neuropsychologists in the future to assess moment-by-moment variations in attention associated with task performance. There continues to be the need to attentional batteries that are theoretically coherent and provide assessment of the component processes that govern attention.

Cross References ▶ Attention-Deficit Disorder ▶ Automaticity ▶ Consciousness ▶ Directed Attention ▶ Divided Attention ▶ Effort ▶ Focused Attention ▶ Habituation ▶ Hemi-Inattention Syndrome ▶ Intention ▶ Orienting Response ▶ Selective Attention ▶ Sustained Attention

References and Readings Cohen, R. A. (1993) Neuropsychology of Attention. New York: Plenum. Cohen, R. A., Meadows, M. E., Kaplan, R. F., & Wilkinson, H. (1994) Habituation and sensitization of the OR following bilateral cingulate damage. Neuropsychologia, 32(5), 609–617.

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Colquhoun, W. P., & Baddeley, A. D. (1967). Influence of signal probability during pretraining on vigilance decrement. Journal of Experimental Psychology, 73, 153–155. Desimone, R., & Gross, C. G. (1979). Visual areas in the temporal cortex of the macaque. Brain Research, 178, 363–380. Deutsch, J. A., & Deutsch, D. (1963) Attention: Some theoretical considerations. Psychological Review, 70, 80–90. Fuster, J. M. (l989). The prefrontal cortex: Anatomy, physiology, and neuropsychology of the frontal lobe. New York: Raven. Goldberg, M. E., & Bushnell, M. D. (l981). Behavioral enhancement of visual response in monkey cerebral cortex. II. Modulation in frontal eye fields specifically related to saccades. Journal of Neurophysiology, 46, 783–787. Hasher, L., & Zacks, R. T. (1979). Automatic and effortful processes in memory. Journal of Experimental Psychology: General, 108, 356–388. Heilman, K. M., Watson, R. T., & Valenstein, E. (l993). Neglect and related disorders. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (3rd ed., pp. 279–336). New York: Oxford University Press. Heilman, K. M., Bowers, D., Coslett, H. B., & Watson, R. T. (l983). Directional hypokinesia in neglect. Neurology, 2(33), 104. Heilman, K. M., Watson, R. T., Valenstein, E., & Goldberg, M. E. (1988). Attention: Behavior and neural mechanisms. Attention, 11, 461–481. James, W. (1890). The principles of psychology. (Vol. 1., pp. 403–404). New York: Henry Holt. Kahneman, D. (1973). Attention and effort. Englewood Cliffs, NJ: Prentice-Hall. Kahneman, D., & Treisman, A. (1984). Changing views of attention and automaticity. In R. Parasuraman & D. R. Davies (Eds.), Varieties of attention. New York: Academic. Kaplan, R. F., Verfaellie, M., DeWitt, L. D., & Caplan, L. R. (1990). Effects of changes in stimulus contingency on visual extinction. Neurology, 40(8), 1299–1301. Mattingley, J. B., Bradshaw, J. L., Bradshaw, J. A., & Nettleton, N. C. (1994). Residual rightward attentional bias after apparent recovery from right hemisphere damage: Implications for a multi-component model of neglect. Journal of Neurology, Neurosurgery & Psychiatry, 57(5), 597–604. Mesulam, M. A. (1981). A cortical network for directed attention and unilateral neglect. Archives of Neurology, 10, 304–325. Parasuraman, R. (1984). Sustained attention in detection and discrimination. In R. Parasuraman & D. R. Davies (Eds.), Varieties of attention. (pp. 243–289). New York: Academic. Pardo, J. V., Fox, P. T., Raichle, M. E. (1991). Localization of a human system for sustained attention by positron emission tomography. Nature, 349(6304), 61–64. Posner, M. I., Walker, J. A., Friedrich, F. A., & Rafal, R. D. (l987). How do the parietal lobes direct covert attention? Neuropsychologia, 25(lA), 135–145. Posner, M. I., & Cohen, Y. (1984). Facilitation and inhibition in shifts of visual attention. In H. Bouma & D. Bowhuis (Eds.), Attention and performance X. Hillsdale, NJ: Erlbaum. Pribram, K. H., & McGuinness, D. (1975). Arousal, activation, and effort in the control of attention. Psychological Review, 82, 116–149. Schneider, W., & Shiffrin, R. M. (1977). Controlled and automatic human information processing: I. Detection, search, and attention. Psychological Review, 84, 1–66. Shannon, C. E., & Weaver, W. (1949). The mathematical theory of communication. Urbana, Illinois: The University of Illinois Press. Treisman, A., & Gelade, G. (1980). A feature-integration theory of attention. Cognitive Psychology, 12, 97–136.

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Verfaellie, M., Bowers, D., & Heilman, K. M. (1988). Attentional factors in the occurrence of stimulus-response compatibility effects. Neuropsychologia, 26, 435–444. Watson, R. T., Heilman, K. M., Cauthen, J. C., & King, F. A. (1973). Neglect after cingulotomy. Neurology, 23, 1003–1007. Wurtz, R. H., Goldberg, M. E., Robinson, D. L. (1982). Brain mechanisms of visual attention. Scientific American, 246(6), 124–135.

Attention Deficit Disorder ▶ Attention Deficit, Hyperactivity Disorder

Attention Deficit, Hyperactivity Disorder K EVIN M. A NTSHEL , R USSELL B ARKLEY State University of New York - Upstate Medical University NY, Mount Pleasant, SC, USA

Synonyms ADD; ADHD; ADHD, combined; ADHD, predominantly hyperactive-impulsive type; ADHD, predominantly inattentive type; Attention deficit disorder; Hyperkinetic disorder

Short Description or Definition Attention deficit/hyperactivity disorder (ADHD) is characterized by developmentally inappropriate degrees of inattention, impulsiveness, and/or hyperactivity that most often arise in early to middle childhood, result in functional impairment across multiple domains of daily life activities, and remain relatively persistent over time.

Categorization DSM-IV-TR defines three ADHD subtypes: Predominantly Inattentive type (ADHD-I), Predominantly Hyperactive/Impulsive type (ADHD-H/I) and Combined type (ADHD-C). ADHD-C is the most prevalent subtype in clinically-referred samples yet the true population prevalence of ADHD-C and ADHD-I is likely comparable, each

accounting for roughly half of the ADHD cases. ADHDH/I is far less common, is most often observed in preschool and early elementary school aged children, and is probably just the earlier developmental stage to the Ctype in many instances. In general, hyperactive-impulsive symptoms decline more steeply as children age (although feelings of restlessness may persist), but inattentive symptoms remain relatively constant. Children with ADHD-H/I and ADHD-C are at higher risk for conduct problems. Youth with ADHD-I are at higher risk for learning disorders, anxiety and possibly depression. While some argue that ADHD-I is a distinct disorder from ADHD-C and ADHD-HI, others have not found consistent differences between the subtypes on neuropsychological or laboratory measures. Though diagnosed as a categorical disorder, ADHD may actually represent an extreme end along a normal continuum for the traits of attention, inhibition and the regulation of motor activity.

Epidemiology The population prevalence of ADHD is estimated to be 3–9% of school-age children and 4–5% of adults. ADHD is more prevalent in males and those with chronic health problems, family dysfunction, low socioeconomic status, presence of a developmental impairment, or urban living. ADHD is a worldwide disorder found in most countries with rates similar to if not higher than those found in North America. Differences across ethnic groups within the North America are sometimes found but seem to be more a function of social class than ethnicity.

Natural History, Prognostic Factors, Outcomes The syndrome of attention difficulties, impulsive behavior and overactivity has been known since the late 1700s and certainly since the early 1900s. Numerous attempts have been made at definition and nomenclature, including Strauss syndrome, minimal brain dysfunction or damage, hyperkinetic child syndrome (or hyperkinesis), and attention deficit disorder with and without hyperactivity. ADHD can exist without other psychiatric disorders in 25–30% of ADHD cases (MTA Collaborative Group, 1999) but is more often associated with co-morbidity. Oppositional defiant disorder (45–65%) is the most common psychiatric co-morbidity in ADHD. As many as half of these oppositional children will progress to early onset

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conduct disorder such as lying, stealing, fighting and otherwise violating the rights of others. Major depressive disorder (20–30%) and anxiety disorders (10–30%) are also relatively common co-morbid conditions in pediatric ADHD. Longitudinal research following children with ADHD into adulthood suggests that 50–86% of children with ADHD continue to show impairing symptoms as they age. The fact that some children do not continue to have an ADHD diagnosis may be due in part to the finding that ADHD symptoms decline as a function of age in typically developing populations. However, it may also simply reflect that DSM symptoms and symptom thresholds may be developmentally inappropriate and too restrictive, respectively, to be applied to outside of childhood. For example, DSM-IV inattentive symptoms are more common in adolescents than DSM-IV hyperactive/impulsive symptoms. While this may infer that inattention persists at higher levels than hyperactivity/impulsivity, it may also simply reflect the developmental insensitivity of the DSM-IV symptoms. Genetics also appear to be a large factor in those who continue to demonstrate clinically significant ADHD post-childhood versus those whose symptoms are in remission. For example, prevalence rates of ADHD among the relatives of children with persistent ADHD are significantly higher than rates in relatives of children with remitted ADHD. In addition, a history of major depressive disorder before age 13 is a predictor of the syndromic persistence of ADHD into adolescence as is having a below average IQ. By definition, individuals with ADHD need to be functionally impaired in two or more domains of major life activities. In children, academic, social and family functioning domains are the most frequently impaired (MTA Collaborative Group, 1999). Educational impairments including academic underachievement and learning disabilities are well documented in the pediatric ADHD literature. In adolescents and young adults, academic impairments continue to persist; young adults with ADHD completed fewer years of education, with nearly 1/4 failing to complete high school. Others have similarly reported that relatively few young adults with ADHD attempt college (20%) and even fewer graduate (5%) from college. Compared to children with ADHD who are followed into adulthood, clinically diagnosed adults with ADHD appear to have higher intellectual levels, have graduated from high school and have at least attempted college. In addition to educational impairments, impairments in domains such as occupational, dating/marital relations, financial management, driving, child-rearing, managing a

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household and maintaining health are also consistently reported in adults with ADHD. For example, employer ratings are lower for adults with ADHD and adults with ADHD have more part-time employment and change jobs more often. There are some data to suggest that ADHD is more functionally impairing than most other outpatient psychiatric disorders in these domains. While the relationship between ADHD symptoms and impairment in children with ADHD is modest (r = 0.3), these relationships may be more robust in adults (r = 0.7).

Neuropsychology and Psychology of ADHD A meta-analysis suggested that children with ADHD have an IQ about nine points lower than typically developing peers. Similar data have been reported in adults with ADHD. Lower performance on the WAIS-III Arithmetic and Digit Symbol may account for a substantial portion of the IQ differences noted between adults with ADHD and community controls. Controlled processing deficits are commonly observed in pediatric ADHD. Children with ADHD perform less well on laboratory tasks that assess vigilance, motoric inhibition, organization, planning, complex problem solving, and verbal learning and memory. Adults with ADHD are impaired on these same cognitive domains. Both children and adults with ADHD perform less well on tasks that require vigilance, or the ability to sustain attention. There is also some evidence that ‘‘rare target’’ paradigms (few targets, many non-targets) such as the Gordon Diagnostic System appear to be more difficult for adults with ADHD than those paradigms with higher signal probabilities. Response inhibition has been hypothesized to play a central role in pediatric ADHD. Continuous performance test (CPT) commission errors are a common laboratory measure of this construct. Unlike attention deficits (which seem to emerge more reliably in rare target CPT’s), response inhibition deficits emerge more reliably in higher signal probabilities such as the Conners CPT. Several studies have reported that adults with ADHD make more errors of commission on high signal CPT’s relative to both clinical and community control participants. Executive functioning deficits are present in both pediatric and adult ADHD. Thus, it is surprising that performance on one of the most well established tests of executive functioning, the Wisconsin Card Sorting Test is not impaired in adults with ADHD. Multiple studies have failed to report a

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significant difference between adults with ADHD and community controls on WCST categories completed and number of errors, both perseverative and non-perseverative. Verbal fluency is impaired in adult ADHD. The most widely used verbal fluency task in adult ADHD populations has been the Controlled Oral Word Association Test. Multiple studies have reported significant differences between community controls and adults with ADHD. Given the importance of attention and working memory to memory encoding and storage, it is not surprising that adults with ADHD have been demonstrated to have memory deficits. They also appear to have more difficulty managing auditory/verbal information relative to visual information. Although differences emerge between adults with ADHD and community controls on the WAIS-III Digit Span, the effect size of the differences are much larger on the California Verbal Learning Test. For example, adults with ADHD perform less well on overall rates of learning, recall, recognition and semantic clustering. The weaker performance on the semantic clustering index may indicate failure to adopt a strategy.

Evaluation The American Academy of Child and Adolescent Psychiatry (Pliszka, 2007) has established guidelines for the assessment and treatment of ADHD. No neurological, genetic, neuropsychological or behavioral tests have sufficient positive and negative predictive power to accurately classify ADHD cases with sufficient success to recommend them for clinical diagnosis. Clinical diagnosis is based largely on careful history taking, use of structured interviews containing DSM-IV criteria for ADHD and related disorders, and the expert knowledge of the clinician in the differential diagnosis among childhood mental disorders. Paramount in the evaluative process is the time to listen to parental concerns, probe for details concerning nature, onset, and course, elaborate the specific impairments resulting from these concerns, and place them in the larger framework of the clinical taxonomy of mental disorders. The clinical interview is then supplemented with the use of parent and teacher behavior rating scales to assess developmental deviance of symptoms, screening of intelligence and academic achievement skills by standardized testing, brief observation of the child during unstructured and structured activities, contact with school personnel concerning classroom functioning and compilation of prior school and mental health records available on the child. Other sources of information essential for the diagnostic process are behavioral rating scales or checklists on

which normative data are available. These include ‘‘broad band’’ questionnaires, such as the Behavioral Assessment System for Children – 2nd edition or Child Behavior Checklist for screening the major dimensions of childhood psychopathology (e.g. anxiety, depression, attention, hyperactivity, aggression, etc.). ‘‘Narrow band’’ questionnaires specifically evaluate the symptoms of ADHD as set forth in DSM-IV. Rating scales can reliably, validly and efficiently measure DSM-IV-based ADHD symptoms. Some examples of instruments demonstrating appropriate psychometric properties with a strong normative base include the ADHD Rating Scale IVand the Conners Rating Scales – 3rd edition. A number of specific tests have been devised to provide objective measures of a subject’s vigilance and impulse control, such as the Gordon Diagnostic System, Conners Continuous Performance Test or the Test of Variables of Attention, among others. Research suggests, that these tests are not especially accurate at classifying children as ADHD; while the presence of abnormal scores on such tests indicates the presence of a disorder in as many as 90% of children who perform poorly, such scores cannot indicate the specific disorder present. Moreover, the ecological validity of these tests is low thus precluding the ability to predict from the test scores how the child will function in more natural settings, such as home and school. These tests are therefore not recommended for routine diagnostic evaluations of children with ADHD, although they may be used in clinics specializing in ADHD as part of research or drug trials. More useful information is likely to be obtained from the parent and teacher rating scales discussed above.

Treatment Treatment for ADHD in children typically involves three components: parent and child education and support, classroom accommodations, and medication. Substantial evidence exists to show that training parents in child behavior management skills can be of significant benefit in the reduction of parent–child conflict and improvement in child success within the home (MTA Collaborative Group, 1999). The school setting frequently requires adjustment to meet the special needs of the child with ADHD. School interventions often include alterations to the curriculum and work load to better mesh with the limited attention, persistence, and disorganization of the child with ADHD; increases in sources of positive reinforcement for work productivity; occasional use of immediate and systematic negative consequences for disruptive or inappropriate behavior; implementation of a daily


school behavior report card (the ratings on which are linked to a home token economy). The mainstay of treatment for many children with ADHD is medication, frequently psychostimulants. Three classes of medication appear to be useful for management of ADHD, these being: psychostimulants (methylphenidate, amphetamines), noradrenergic reuptake inhibitors (atomoxetine), and antihypertensive medications (clonidine, guanfacine). Stimulant medications, especially extended release formulations, are a front-line management strategy in pediatric ADHD; approximately 70% of children with ADHD will show an efficacious response to stimulant medications such as Methylphenidate or mixed-salt amphetamines. The side effects of stimulants are fairly benign, short-lived, dose related, and often managed through dose or timing adjustments, or by switching to a different delivery system or stimulant. Atomoxetine is a nonstimulant approved for management of ADHD. Atomoxetine is an exclusive noradrenergic reuptake inhibitor and is the first drug indicated for ADHD that is not a Schedule II controlled substance with low potential for abuse, making it more convenient than the stimulants for sampling, prescribing, and titrating. Clonidine and guanfacine are alpha2-noradrenergic agonists that have some effectiveness for the management of hyperactive-impulsive ADHD symptomatology. They are also considered ‘‘off-label’’ for treatment of ADHD as they have not as yet been specifically approved by the FDA for treatment of ADHD. An extended release form of guanfacine received FDA approval in 2009. In adults, stimulant medications are effective in approximately 70% of individuals with ADHD. Atomoxetine may be particularly effective for adults with ADHD and co-morbid depression or for those with a co-morbid substance use disorder. Managing psychiatric co-morbidity is a significant component of pediatric ADHD. The same dictum appears central to managing ADHD in adults. While ‘‘uncomplicated’’ ADHD exists in about 25% of the adults with ADHD, most adults with ADHD have significant psychiatric co-morbidity that requires clinical attention and management. One aspect in which the psychiatric comorbidity is evident in treatment strategies is pharmacotherapy. Although the evidence for the efficacy of polypharmacy is limited at this time, multiple researchers have asserted that polypharmacy may be more likely in adult ADHD than pediatric ADHD. Similar to pediatric ADHD, a psychosocial treatment component is typically recommended in adult ADHD.

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What constitutes the psychosocial component, however, appears to be somewhat different in adult ADHD relative to pediatric ADHD. For example, neither cognitive behavioral therapy (CBT) nor cognitive therapy has much research support in pediatric ADHD. Nonetheless, there are some data to suggest that CBT may be more efficacious in adults with ADHD. For example, in the adult ADHD literature, there is some evidence that CBT is efficacious for reducing functional impairments in adults concurrently treated with stimulants.

Cross References ▶ Americans with Disabilities Act ▶ Attention; Attention/Executive Functions ▶ Atomoxetine; Behavior Assessment System for Children (BASC) ▶ Cognitive Behavioral Therapy ▶ Conners 3rd edition ▶ Continuous Performance tests ▶ D-amphetamine ▶ Executive Functioning ▶ Individuals with Disabilities Education Act ▶ Learning Disability ▶ Metacognition ▶ Methylphenidate ▶ Section 504 of the Rehabilitation Act of 1973 ▶ Stimulants ▶ Working Memory

References and Readings Barkley, R. A. (2006). Attention deficit hyperactivity disorder: A handbook for diagnosis and treatment (3rd ed.). New York: Guilford Press. Barkley, R. A., & Murphy, K. R. (2006). Attention deficit hyperactivity disorder: A clinical workbook (3rd ed.). New York: Guilford. MTA Collaborative Group. (1999). A 14-month randomized clinical trial of treatment strategies for attention-deficit/hyperactivity disorder. The MTA Cooperative Group. Multimodal treatment study of children with ADHD. Archives of General Psychiatry, 56 (12), 1073–1086. Nigg, J. (2006). What causes ADHD: Understanding what goes wrong and why. New York: Guilford. The ADHD Report, a bimonthly newsletter for clinicians edited by Dr Barkley with contributions from leading clinicians and researchers. Guilford Publications, New York. Pliszka, S. (2007). Practice parameter for the assessment and treatment of children and adolescents with attention-deficit/hyperactivity disorder. Journal of the American Academy of Child and Adolescent Psychiatry, 46(7), 894–921.

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Attention Network Test (ANT)

Attention Network Test (ANT) M ICHAEL S. WORDEN Brown University Providence, RI, USA

Attention is often subdivided by researchers into a number of separate systems. Although there is certainly some interaction between them, these systems play different roles in terms of their effect on information processing and the control of behavior. Further, there is evidence that different attentional systems are associated with different, largely nonoverlapping brain regions and rely to a large extent on different neurotransmitter systems. One such framework advanced by Michael Posner and colleagues defines three separate attention systems or networks: alerting, orienting, and executive control. The alerting system is responsible for helping the organism reach and maintain an alert state. This state, which is separate from arousal, is characterized by a readiness to perceive and process incoming stimuli. The alerting system has been associated with superior parietal, right frontal, and thalamic brain regions and the norepinephrine neurotransmitter system. The orienting system is responsible for selecting and giving preference to specific sensory information, often in terms of spatial location. Attentional orienting in space may be done overtly by, for example, moving the head or covertly, that is, without moving the eyes or head. For example, a football player might look down the field with his eyes while attending covertly to the location and movements of other players in his peripheral vision. Attended items are generally processed faster and more accurately than nonattended items. Brain areas that have been linked to the orienting system include areas of the parietal cortex and the frontal eye fields, and the cholinergic neurotransmitter system appears to play an important role. The executive attention system is involved in monitoring one’s performance in the context of current task demands and providing control signals that help other systems adapt to changing contexts and conflicting information. Executive attention is especially important for detecting and responding to situations in which there is stimulus–response conflict. Such conflict arises when two or more stimuli or two or more aspects of the same stimulus are associated with different behavioral responses. A common example is the Stroop task in which subjects are presented with printed words and they must name the color of the ink in which the word

is printed (e.g., red ink) when the word itself spells out a different color (e.g., BLUE). Brain areas implicated in the executive attention system include frontal midline regions such as the anterior cingulate cortex and the lateral prefrontal cortex. The neurotransmitter dopamine is important in the functioning of this network. The Attention Network Task (ANT) was developed by Jin Fan, Michael Posner, and colleagues at the Sackler Institute for Developmental Psychobiology. Using subtractive methodology, the ANT is designed to assess each of these three attentional networks using a single reactiontime paradigm. The fundamental task of the participant is simple. On each trial, the participant looks at a small fixation cross in the center of a computer screen and a small arrow, called the target, is briefly displayed either above or below the fixation. The participant is required to respond by pressing one of two buttons as quickly and accurately as possible indicating whether the arrow is pointing to the left or right. On some presentations, the target is preceded by a briefly presented cue stimulus while on other trials it is presented with no advanced warning. These cues are either predictive or non-predictive. Orienting cues are presented either above or below the central fixation and indicate the location at which the upcoming arrow will be shown. Non-orienting cues are presented either at the center of the screen or else both above and below fixation simultaneously. Both types of cues indicate that the target is about to appear but only orienting cues provide information regarding the location of the impending target. Finally, in some cases, the target arrow is flanked on either side by other stimuli. These flanking stimuli may be arrows pointing in the same direction as the target (called congruent trials) or they may be arrows pointing in the opposite direction as the target (called incongruent trials). The efficiency of the three attention networks may be assessed independently for each participant by use of the subtractive method. Both reaction time and accuracy may be examined. To assess the alerting network, scores from trials in which no cue was presented are compared to scores from trials in which there was a cue presented. The difference in mean reaction times between these two trial types constitutes an efficiency score for the alerting network and is indicative of the extent to which the alerting network was able to use the information provided by the cue to improve behavioral performance. In a similar manner, an efficiency score for the orienting network may be derived by subtracting mean scores from trials with orienting cues from the scores for trials with non-orienting cues. Both of these trial types include a cue so there should be no difference in terms of a

Attention Training

contribution from the alerting network. The difference in scores measures the degree to which the orienting system could take advantage of the predictive cues to orient to a specific spatial location. In the case of the non-orienting cues, the participant could not predict whether the target would appear above or below the fixation point and therefore could not improve performance by orienting to one or the other spatial location. An efficiency score is derived for the executive attention system by comparing scores on trials with congruent flankers to trials with incongruent flankers. Subjects will tend to be slower and less accurate for incongruent trials than for congruent trials, and the size of these differences indicates the extent to which the individual is able to suppress conflicting response tendencies. A number of intriguing findings have come from studies that have utilized the ANT. Supporting the notion that the three attention networks assessed by the ANT constitute independent systems, a large-scale study of over 200 individuals found that there was very little correlation in efficiency scores among the three networks. In other words, the particular score of an individual on any one of the three attention networks does not tend to predict that individual’s scores on the other two networks. A high efficiency score for the alerting network, for example, does not suggest what one’s scores are likely to be for either the orienting or executive attention networks. Using electroencephalographic (EEG) recordings from the surface of the scalp, it was found that each of these three attention systems is associated with distinct patterns of neural oscillations. A number of variations on the original ANT have been developed to address specific questions and for the study of special populations. For example, child-friendly versions of the ANT that use cartoon pictures of fish instead of arrows have been used to study the development of attention systems. Attentional deficits are a hallmark of many psychiatric and neurological disorders. The ANT has been used to assess the relative impact of many disorders on the different attention systems and to help distinguish between or establish subtypes of particular disorders. Among others, variations on the ANT have proven useful in the study of attention deficit hyperactivity disorder, Alzheimer’s disease, autism, borderline personality disorder, traumatic brain injury, substance abuse, and schizophrenia.

References and Readings Fan, J., McCandliss, B. D., Sommer, T., Raz, A., & Posner, M. I. (2002). Testing the efficiency and independence of attentional networks. Journal of Cognitive Neuroscience, 14(3), 340–347.

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Fan, J., McCandliss, B. D., Fossella, J., Flombaum, J. I., & Posner, M. I. (2005). The activation of attentional networks. Neuroimage, 26(2), 471–479. http://www.sacklerinstitute.org/users/jin.fan/ Posner, M. I., & Rothbart, M. K. (2007). Research on attention networks as a model for the integration of psychological science. Annual Review of Psychology, 58, 1–23.

Attention Process Training ▶ Attention Training

Attention Span ▶ Span of Apprehension

Attention Training M C K AY M OORE S OHLBERG University of Oregon Eugene, OR, USA

Synonyms Attention process training, direct attention training, process training

Definition Attention training is based on the premise that attentional abilities can be improved by activating particular aspects of attention through a stimulus drill approach. The repeated stimulation of attentional systems via graded attention exercises is hypothesized to facilitate changes in attentional functioning. Most attention training programs assume that aspects of cognition can be isolated and discretely targeted with training exercises.

Current Knowledge The aspects of attention that are trained vary widely among interventions and frequently depend upon a theoretical model of attention. Attention models,

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regardless of their operational framework, appear to include functions related to sustaining attention over time (vigilance), capacity for information, shifting attention, speed of processing, and screening out distractions. Some attention efficacy studies evaluate attention interventions that focus on particular attention components such as reaction time and sustained attention for visual information (e.g., Ponsford and Kinsella, 1988). Other efficacy studies use attention training programs that include hierarchical tasks to address a continuum of attention components from basic sustained attention to more complex mental control (e.g., Park, Proulx, & Towers, 1999; Sohlberg, McLaughlin, Pavese, & Heidrich, 2001). Evidence supports the effectiveness of attention training beyond the effects of nonspecific cognitive stimulation for patients with traumatic brain injury or stroke during the postacute phase of recovery and rehabilitation (Butler, 2008; Cicerone et al., 2000). Evidence-based practice guidelines for attention training were generated from examination of the intervention research literature (Sohlberg et al., 2003). Analysis of nine Class I and Class II studies suggested that certain aspects of attention training are helpful in improving attention performance in some adults with traumatic brain injury. Treatment parameters found to influence positive outcomes included high frequency of attention training, combining attention training with metacognitive training (e.g, self monitoring and strategy training), and individualizing training to match the client’s attention profile. The effects of attention training may be relatively small or taskspecific, and the research encourages clinicians to actively facilitate and monitor the impact of attention training on functional, everyday activities.

Cross References ▶ Attention ▶ Attention/Executive Functions ▶ Neuropsychological Rehabilitation ▶ Plasticity ▶ Process Training

References and Readings Butler, R. W., Copeland, D. R., Fairclough, D. L., Mulhern, R. K., Katz, E. R., Kazak, A. E. et al. (2008). A multicenter, randomized clinical trial of a cognitive remediation program for childhood survivors of a pediatric malignancy. Journal of Consulting and Clinical Psychology, 76(3), 367–378.

Cicerone, K. D., Dahlberg, C., Kamar, K., Langenbahn, D. M., Malec, J. F., Bergquist, T. F. et al. (2000). Evidence-based cognitive rehabilitation: Recommendations for clinical practice. Archives of Physical Medicine & Rehabilitation, 81, 316–321. Galbiati, S., Recla, M., & Pastore, V. (2009). Attention remediation following traumatic brain injury in childhood and adolescence. Neuropsychology, 23(1), 40–49. Park, N. W., & Ingles, J. L. (2001). Effectiveness of attention rehabilitation after acquired brain injury: A meta-analysis. Neuropsychology, 15, 199–210. Park, N. W., Proulx, G., & Towers, W. M. (1999). Evaluation of the attention process training programme. Neuropsychological Rehabilitation, 9, 135–154. Sohlberg, M. M., Avery, J., Kennedy, M., Ylvisaker, M., Coelho, C., Turkstra, L., & Yorkston, K. (2003). Practice guidelines for direct attention training. Journal of Medical Speech Language Pathology, 11(3), xix–xxxix. Sohlberg, M. M., McLaughlin, K. A., Pavese, A., Heidrich, A., & Posner, M. (2001). Evaluation of attention process training and brain injury education in persons with acquired brain injury. Journal of Clinical and Experimental Neuropsychology, 22, 656–676.

Attentional Response Bias R ONALD A. C OHEN Brown University Providence, RI, USA

Definition Attentional response bias refers to the tendency to respond to the targets of attention on a particular task or in a given context. This tendency is often operationalized using signal detection theory based on possible error types that can occur; that is, either misses (errors of omission) or false positive (errors of commission). The proportion of these types of errors can be expressed as an index corrected relative to the total number of errors of all types made (b: beta). A person who makes a much larger number of errors of commission than errors of omission (misses) is exhibiting a response bias toward responding by indicating they detected the target even when it was not presented, which in many cases reflects excessive impulsivity or inhibitory control problems. Conversely, a tendency to miss targets, but to rarely make false positive efforts, suggests that the patient may be trying hard to never make an error, which in turn results in not responding when they should. This is often observed in patients who lack motivation, are depressed, or who experience problems because of slowed processing speed. In healthy individuals, response bias can be influenced by

Atypical Teratoid/Rhabdoid Tumor (AT/RT)

instructional set, reward, or other factors that shift the likelihood of responding or not responding.

Cross References

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References and Readings Larrabee, G. (2005). Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Stern, B. H., & Brown, J. (2007). Litigating brain injuries. New York: Thomson Reuters.

▶ Continuous Performance Test ▶ Signal Detection Theory

Atypical Antipsychotic Attention-Deficit/Hyperactivity Disorder (ADHD)

▶ Neuroleptics

▶ Minimal Brain Dysfunction

Atypical Autism Attorney M OIRA D UX Rosalind Franklin School of Medicine Baltimore, MD, USA

Definition An attorney is defined as one who is legally appointed on another’s behalf. An attorney-at-law is an individual who has achieved the necessary educational requirements (J.D.) and is licensed to practice law by the highest court of a state or some other form of jurisdiction. In civil cases (e.g., personal injury, medical malpractice), there are plaintiff and defense attorneys. The plaintiff attorney represents the injured party (e.g., plaintiff) in an action against the party they allege to be responsible for the damages; the defense attorney represents the defendant (e.g., insurance company, hospital, and doctor). In criminal matters, there are prosecution and defense attorneys. The prosecuting attorney represents the party (e.g., Federal, State, or local government) who has accused and wants to convict the offender of some type of criminal action (e.g., murder, assault). The defense attorney represents the party (e.g., defendant) who has been accused of committing the crime.

Cross References ▶ Litigation

▶ Pervasive Developmental Disorder NOS

Atypical Teratoid/Rhabdoid Tumor (AT/RT) J ENNIFER T INKER Drexel University Philadelphia, PA, USA

Definition Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare, highly malignant tumor of early childhood, most commonly diagnosed in infants who are less than 2 years. First described by Rorke and colleagues in 1987 (Lefkowitz, Rorke, & Packer, 1987), the AT/RT received its designation because of its complex histological components. Prognosis is extremely poor with a median survival of 6–11 months. Over half of AT/RTs identified are located within the posterior fossa (brainstem, cerebellum, and predominantly the cerebello-pontine angle) (Rorke, Packer, & Biegel, 1996). Clinical presentation varies largely by tumor location and size. Infants, in particular, may present with nonspecific symptoms, including lethargy and failure to thrive. Older children (>3 years of age) may demonstrate more specific problems, including head tilt, cranial nerve palsy, headache, and hemiplegia (Rorke & Biegel, 2000). Often histologically confused with PNET/ medulloblastoma.

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Cross References ▶ Medulloblastoma ▶ Primitive Neuroectodermal Tumor

References and Readings Lefkowitz, I. B., Rorke, L. B., & Packer, R. J. (1987). Atypical teratoid tumor of infancy: definition of an entity. Annals of Neurology, 22, 56–65. Rorke, L. B., & Biegel, J. A. (2000). Atypical teratoid/rhabdoid tumour. In P. Kleihues & W. K. Cavenee (Eds.), World health organization classification of tumours. Pathology & genetics. tumours of the nervous system (pp. 145–148). Lyons, France: IARC Press. Rorke, L. B., Packer, R. J., & Biegel, J. A. (1996). Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. Journal of Neurosurgery, 85, 56–65.

Atypicals (antipsychotics) ▶ Antipsychotics

Auditory Agnosia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Synonyms Auditory-sound agnosia; Auditory-verbal agnosia; Pure word deafness

Definition Rare condition in which sounds, although heard, are not properly interpreted and thus have little or no meaning for the patient.

condition, appreciation of certain aspects of musical sounds might also be compromised (amusia). For this syndrome to be diagnosed, other higher order deficits that might more readily explain the deficit (such as aphasic disorder) should be ruled out. In auditory-verbal agnosia there is impairment of one’s ability to process, interpret, or comprehend speech sounds or spoken language. Patients may report that it is like hearing someone speaking in a foreign language. Reading, writing, and speaking are intact, although speaking may be slightly problematic due to the distortions in auditory feedback heard as speech is attempted. In auditory-verbal agnosia (pure word deafness), the ability to match nonspeech or ‘‘environmental’’ sounds (e.g., a barking dog, the ringing of a bell, or a train whistle) to corresponding pictures may remain intact. Conversely, one may have difficulties identifying or matching nonspeech sounds, while retaining the ability to process and interpret spoken language. Some degree of impairment in one’s ability to recognize musical sounds is commonly, but not invariably, present in these disorders. Select patients may have difficulty recognizing familiar tunes or melodies, while retaining their ability to produce them spontaneously. Others may be impaired at matching tones, rhythms, or timbre, for example, identifying the sound of a particular musical instrument. Auditory agnosia is thought to result from either (1) unilateral or bilateral lesions of the unimodal (secondary) auditory association cortex in the middle portions of the superior temporal gyrus and/or (2) a disconnection syndrome involving the primary auditory cortex (Heschl’s gyrus) of one hemisphere and the subcortical projections from the opposite hemisphere to the unimodal auditory association cortex on that same side. Such lesions would allow elementary sounds to be heard (as one or both of Heschl’s gyri are intact), but would produce impaired higher level processing due to damage or inaccessibility to the unimodal cortex. With critically placed bilateral lesions of the superior temporal gyri, an agnosia for all types of complex auditory input may be present. Auditory-verbal agnosia is more likely to result from left-sided lesions as described above, while auditory agnosia for nonspeech sounds is more likely to be associated with right hemispheric lesions. Agnosia for musical sounds may also be differentially affected, but in an even less consistent manner.

Current Knowledge When present, auditory agnosia, is usually primarily limited to impaired recognition of either language sounds or nonlanguage (environmental) sounds. The former is known as auditory-verbal agnosia or pure word deafness. No commonly used term is applied to the latter. In either

Cross References ▶ Agnosia ▶ Amusia ▶ Disconnection Syndromes

Auditory Discrimination

References and Readings Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed., pp. 236–295). New York: Oxford University Press.

Auditory and Visual Evoked Potentials ▶ Event-Related Paradigms

Auditory Brainstem Response Audiometry ▶ Brainstem Auditory Evoked Responses

Auditory Brainstem Responses (ABR) ▶ Brainstem Auditory Evoked Responses

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Heschl’s gyrus. The primary auditory cortex receives direct input from the medial geniculate nuclei of the thalamus, which it is thought to process auditory input at a very basic level with little, if any, distinction between the right and left hemispheres. The secondary auditory cortex (primarily Brodmann’s area 22) surrounds the primary cortex and, for the most part, is located in the lateral portion of the superior temporal gyrus. The posterior portion of this secondary cortex in the left hemisphere constitutes Wernicke’s area. These secondary cortices are thought to be responsible for the further refinement of auditory input, organizing it into meaningful or potentially meaningful percepts. Lesions of Wernicke’s area (left hemisphere) are associated with severe comprehension and other language-related deficits, whereas comparable lesions in the right hemisphere may be associated with difficulty recognizing or interpreting nonlanguage sounds. Such lesions in the right hemisphere might help account for the inability of some patients to comprehend or interpret the emotional tones or inflections in spoken language, which may convey more meaning than the actual words themselves (i.e., receptive aprosodia).

Cross References ▶ Aprosodia ▶ Auditory Agnosia ▶ Homotypic Cortex ▶ Idiotypic Cortex

Auditory Cortex J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition That portion of the cerebral cortex devoted exclusively to the processing of input from the medial geniculate nuclei (auditory information).

Auditory Discrimination B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms Current Knowledge Located in the superior portion of the temporal lobe of each hemisphere, the auditory cortex consists of both primary (idiotypic) and secondary (unimodal homotypic) cortices. The former is located in the temporal operculum (Brodmann’s area 41 and part of 42) and is referred to as

Auditory processing

Definition Auditory discrimination is the ability to recognize differences in phonemes (the smallest unit of sound in a

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language), including the ability to identify words and sounds that are similar and those that are different. Auditory discrimination tests are performed to measure a person’s phonological awareness, such as the ability to focus on and manipulate phonemes within spoken words. Impaired auditory discrimination should be addressed early in child development as it is pertinent to learning and development.

Cross References ▶ Language ▶ Phonological Disorders ▶ Phonology

References and Readings Gordon-Brannan, M. E., & Weiss, C. E. (2008). Clinical management of articulatory and phonologic disorders (3rd ed.). Philadelphia, PA: Lippincott Williams & Wilkins. Moller, A. R. (2000). Hearing: Its physiology and pathophysiology. San Diego, CA: Academic Press. Warren, R. M. (1999). Auditory perception: A new synthesis (2nd ed.). New York: Cambridge University Press. Warren, R. M. (2008). Auditory perception: An analysis and synthesis (3rd ed.). New York: Cambridge University Press.

Auditory Evoked Response (AER) ▶ Brainstem Auditory Evoked Responses

Current Knowledge From the Outer Ear to the Cochlear Nuclei Sound is transmitted through the air as longitudinal waves, enters the outer ear, and vibrates the tympanic membrane. The three ossicles (malleus, incus, and stapes) amplify and transmit these vibrations to the oval window, producing waves in the scala vestibuli, a fluid-filled compartment within the coil-shaped cochlea of the inner ear. These fluid waves distort the stiff basilar membrane. Residing on the membrane, hair cells within the organ of Corti transduce the minute movements of the membrane into the graded release of glutamate onto the peripheral processes of bipolar afferent fibers, whose cell bodies are located in the spiral ganglion. The central processes exit the base of the cochlea, form the auditory trunk of the vestibulocochlear nerve (eighth cranial nerve, CN VIII), and project ipsilaterally to the ventral and dorsal cochlear nuclei in the brainstem.

From the Cochlear Nuclei to the Superior Olivary Nuclei Fibers from the dorsal cochlear nucleus decussate to the contralateral inferior colliculus via the lateral lemniscus. Fibers from the ventral cochlear nuclei project ipsilaterally to the superior olivary nucleus and also decussate via the trapezoid body to the contralateral superior olivary nucleus. This circuit provides temporal and intensity differences in the horizontal plane between right and left ear to aid in sound source localization. Because of the bilateral nature of these afferent projections, central lesions rarely result in total unilateral hearing loss.

Auditory Pathway WOON C HOW Virginia Commonwealth University Richmond, VA, USA

Definition The auditory neural pathway in the central nervous system transmits and processes sound signals from the ear to the cortex. The configuration of the pathway is multisynaptic and bilaterally projecting.

From the Superior Olivary Nuclei to the Medial Geniculate Nuclei Afferent fibers from the superior olivary nuclei merge with other audition-associated ascending fibers and project via the lateral lemniscus to the inferior colliculus. The inferior colliculus receives bilateral inputs from almost all audition-related nuclei and acts as a nearly obligatory relay in the ascending auditory pathway. It is here that horizontally oriented and vertically oriented sound source localization data is fully and finally integrated. Ascending fibers from the inferior colliculus project ipsilaterally to

Auditory Processing

the last subcortical relay station, the medial geniculate nucleus. Located in the posteroinferior portion of the thalamus, the medial geniculate nucleus is a relay between the inferior colliculus in the brainstem and the auditory cortex. The medial geniculate nucleus plays a role in directing and maintaining attention.

From the Medial Geniculate Nuclei to Heschl’s Gyri Outputs from the medial geniculate nucleus project via the internal capsule to the ipsilateral primary auditory cortex located in the posterior portion of the superior temporal gyrus of Heschl. At the cortical level, detected sound is finally perceived. Bilateral lesions of the auditory cortex remove the conscious perception of sounds, but because of extensive subcortical processing, an individual may still react reflexively to a sound without actually ‘‘hearing’’ it.

Tonotopic Mapping One idea of note is tonotopy, which is the spatial arrangement of where particular frequencies of sound are relayed and processed within the auditory system. In the cochlea, high frequency sounds are detected by hair cells at the base and low frequency sounds at the apex. This tonotopic organization is preserved systematically all the way up to the primary auditory cortex, where higher frequency sounds are mapped to a more medial location on the superior temporal gyrus whereas lower frequency sounds are mapped to a more anterolateral location.

Cross References ▶ Auditory Cortex ▶ Auditory System ▶ Cochlea ▶ Cochlear Nuclei (Dorsal and Ventral) ▶ Heschl’s Gyrus ▶ Inferior Colliculi ▶ Internal Capsule ▶ Lateral Lemniscus ▶ Medial Geniculate Nuclei ▶ Trapezoid Body ▶ Vestibulocochlear Nerve

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Auditory Perceptual Disorder (APD) ▶ Central Auditory Processing Disorder

Auditory Processing S COTT L. D ECKER , J ESSICA A. C ARBONI Georgia State University Atlanta, GA, USA

Definition Auditory processing is a term used to describe the process in which sound waves are transduced into neurological impulses and decoded by the primary auditory cortex in the temporal lobe of the brain. Further processing involves general sound detection and language sounds. Object vibration causes molecules of air surrounding it to condense and pull apart, producing waves that travel away from the object. Receptor cells within our ears will be stimulated if the vibration ranges between approximately 30 and 20,000 times per second (Carlson, 2007). These waves will then be perceived as sound. There are three dimensions of sound: pitch, loudness, and timbre. The pitch of an auditory stimulus is determined by the frequency of vibration or cycle per second (Hertz). Intensity of sound is a function of loudness, whereas vigorous vibrations of an object produce more intense sound waves thus producing louder sounds. Information regarding the nature of sound is produced through timbre. Our ears are able to detect stimuli, determine the spatial location of those stimuli, and recognize the identity of such stimuli.

Cross References ▶ Auditory Discrimination ▶ Auditory Pathway

References and Readings Carlson, N. R. (2007). Physiology of behavior (8th ed.). Boston: Pearson.

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Auditory Processing Disorder (APD) ▶ Central Auditory Processing Disorder

Auditory Sensory Memory ▶ Echoic Memory

Auditory Spatial Processing ▶ Spatial Processing

Auditory System M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Structure The structure and function of the human auditory system was first postulated by the physicist George Ohm more than 100 years ago. Dr. Ohm theorized that the auditory system’s main function was to translate complex sound material into highly specialized vibratory signals that could then be processed in the brain and recoded as recognizable entities. At a very basic level, the auditory system might be considered as being composed of three primary structures and their interconnections. The first of these is the ear, which itself is typically divided into three components. The outer or external ear is that which is visible, the pinna and the auditory meatus or ear canal which terminates at the tympanic membrane or ear drum. Next is the middle ear which primarily consists of a linked series of three small bones, the malleus, incus, and stapes, which act together as a system of levers. The former is attached to the tympanic membrane and the latter to the oval window of the inner ear. The middle ear is connected to the oral cavity by the eustachian tube which allows for

equalization of pressure on either side of the tympanic membrane. The semicircular canals, the vestibule, and the cochlea comprise the inner ear. The first two constitute the end organs for the vestibular system, whereas the cochlea and organ of Corti contained within it represent the origin of the nerve impulses that eventually are translated into sounds. The second set of structures in the auditory system is the brainstem nuclei associated with hearing. The dorsal and ventral cochlear nuclei located in the region of the pontine–medullary junction represent the origin of the second-order auditory fibers. Next in line are the superior olivary nuclei, which lie in the pons and are the first nuclear group to receive auditory input from both ears. The next and final major nucleus concerned with hearing in the brain stem is the inferior colliculus, a paired structure in the dorsal portion of the midbrain. The brain itself might be considered the third portion of the auditory system. The two most critical structures here are the medial geniculates of the thalamus, and Heschl’s gyri (Brodmann’s area 41) which lie in the temporal operculum (within the lateral fissure) of each hemisphere. It is this last structure, in conjunction with its adjacent secondary auditory cortices, which is responsible for processing the auditory input into meaningful information. Finally, there are the major pathways that interconnect these various structures. The auditory portion of the vestibulo-cochlear nerve (CN VIII) is the first-order neuronal pathway in the auditory system. It has its origins in the organ of Corti and terminates in the dorsal and ventral cochlear nuclei. The acoustic stria (dorsal, ventral, and intermediate) form the second-order neurons of the auditory system. What is important to note is that while most fibers cross the midline, some remain ipsilateral, thus at a very early stage there is bilateral input from each ear. The trapezoid body of the pons represents one such major crossing of these auditory fibers (primarily those from the ventral acoustic stria). Most of these second-order fibers synapse in the superior olivary nuclei, although some proceed directly to the inferior colliculi. The lateral lemniscus, again consisting of both crossed and uncrossed fibers, interconnects the superior olivary nuclei with the inferior colliculi. From there, the brachium of the inferior colliculi carries auditory signals to the medial geniculates which, in turn, project ipsilaterally to the primary auditory cortices. It should be noted that, due to the arrangement of the auditory pathways, by the time these signals reach the cortex they are derived from both ears, with approximately 60% coming from the contralateral ear and 40% from the ipsilateral one.

Auditory System

Function Joseph Fourier, a French mathematician, identified the physical and mathematical properties of sound waves and described the transformation of such stimuli into frequency, amplitude, and phase, which govern discrete elements of sound such as loudness and pitch. In its raw, unprocessed state, sound exists in the form of vibration that results in alterations in the pressure of the air in the immediate environment. These alterations in pressure take the form of waves that have a specific frequency or combination of frequencies as well as intensity. The frequency of the sound wave, as measured by hertz (Hz) is the major determinant of the pitch of the resulting sound, experienced by the listener as high or low. The amplitude of the wave, or its height, is the major determinant of the loudness of the resulting sound, measured in decibels (dB). The human ear is capable of capturing sound over a considerable frequency range, approximately 20–20,000 Hz. The transduction of sound waves into the perception of sound is complex. Vibrations entering the external auditory meatus strike the tympanic membrane, causing it to vibrate. This vibration is transferred directly to the ossicles; first the malleus, which is attached to the tympanic membrane, followed by the incus and then the stapes which sets the oval window of the inner ear in motion. The vibration is then picked up by the fluids (perilymph and endolymph) of the cochlea, first setting the perilymph of the scala vestibuli and then the endolymph of the organ of Corti and basilar membrane upon which its rests, and finally the perilymph of the scala tympani from where it is dissipated via the round window of the inner ear. In the course of this activity, the basilar membrane is differentially affected depending on the frequency of the waves causing the hair cells along its length to be stimulated, initiating patterns of nerve impulses that correspond to the particular pitch. This very discrete information is picked up by the auditory nerve (CN VIII) in the form of bioelectrical nerve impulses, which then are propagated through the various pathways described above until eventually reaching the cerebral cortex where they are eventually interpreted as speech or other sounds.

Illness Damage to any part of the auditory system, from cerumen (wax) in the auditory canal to bilateral cortical lesions (exceedingly rare) can result in hearing deficits. Because of the multiple crossings and ipsilateral connections within the system, hearing loss which is confined to one ear

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normally implies damage no higher than the cochlear nuclei. Pure word deafness (intact hearing with the inability to understand spoken language without other major aphasic deficits) can result from a relatively rare occurrence of damage to Heschl’s gyrus on the left and a dissociation of input to the secondary association areas from the non-dominant hemisphere. Damage at intermediate levels may result in poor localization of sounds. Aside from ponto-medullary strokes, unilateral (or bilateral) hearing loss is most commonly the result of damage to the middle or inner ear or the nerves that emanate from the latter. Any of many causes could be the problem, from prolonged exposure to loud noises, trauma, infections, medications, and, of course, simply aging. While formal assessments of hearing loss are best left to audiologists, a gross assessment of hearing acuity is important to better understand why a particular patient may be having difficulty either on examination of mental status or coping at home or on the job. Given a hearing loss, neurologists will often try to differentiate its particular nature. Two types of peripheral hearing loss (i.e., not due to a lesion of the brain stem or above) are typically identified, conductive and sensorineural. The former, which is generally more amenable to treatment, is a result of a problem with the external or middle ear, while the latter implies damage to the inner ear. These can often be distinguished by a couple of procedures using a tuning fork (preferably 512 Hz). In the first, the ability of the patient to hear the vibration is tested by comparing air to bone conduction (Rinne test). Here the base of the vibrating tuning fork is applied to the mastoid process just behind the ear. When the sound is said to have dissipated, the ends of the fork are immediately moved near the auditory canal (air conduction). If the problem is in the middle ear, the sound will not be heard. Conversely, if the sound is heard better via air than bone conduction, a sensorineural (inner ear) deficit is suspected. It should be noted that for normals, air conduction will be superior to bone conduction, but one should be looking for relative differences in acuity, not absolute auditory thresholds as the latter will likely lowered in the affected ear. A second procedure is to press the base of the tuning fork on the middle of the forehead. If there is a sensorineural loss, the sound will be localized to the unaffected ear, while it will be localized (sound louder) in the affected ear in a conductive hearing loss. This latter procedure is referred to as the Weber test. Another common problem associated with hearing is tinnitus, a buzzing, ringing, or other repetitive noxious sound in one or both ears. It can be relatively brief or chronic. If the latter, it can be very disturbing to the patient. The causes can be multiple, including certain

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drugs (e.g., aspirin), aftereffects of exposure to loud noises, infections, or occasionally may represent the initial symptoms of a more serious condition such as a brainstem tumor. Unfortunately, treatment options for this condition are quite limited. Having background noise, such as music, is often helpful.

Cross References ▶ Aphonia ▶ Auditory Cortex ▶ Auditory Pathway ▶ Cochlea ▶ Pure Word Deafness ▶ Tinnitus ▶ Weber Test

References and Readings Bradley, W. G., Daroff, R. B., Fenichel, G. M., & Jankovic, J. (2004). Neurology in clinical practice: Principles of diagnosis and management – Fourth Edition. Philadelphia, PA: Butterworth-Heineman. Wilson-Pauwek, L., Akesson, E. J., Stewart, P. A., & Spacey, S. D. (2002). Cranial nerves in health and disease. Hamilton, ONT: B.C. Decker, Inc.

Auditory Verbal Learning J UDY C REIGHTON 1, H. A LLISON B ENDER 2 S TEPHANIE A SSURAS 1, J ENNIFER WOEHR 3 J OAN C. B OROD 1,3 , N ANCY S. F OLDI 1,4 1 Queens College and The Graduate Center of the City University of New York Flushing, NY, USA 2 New York University Langone Medical Center New York, NY, USA 3 Mount Sinai School of Medicine New York, NY, USA 4 State University of New York, Stony Brook School of Medicine Mineola, NY, USA

Description An auditory verbal learning task typically requires individuals to hear a list of items, learn those items, and recall

and/or recognize them at a later time. These tasks assess acquisition and retrieval components of memory, including encoding, learning characteristics, storage, consolidation over short- or long-time intervals, and subsequent access to the information either by free retrieval or recognition. The nature of the test composition, the instructions to the individual, and the scoring dictate what conclusions are drawn from the task. Auditory verbal learning tasks (AVLTs) are used in both clinical and research settings, and are a hallmark of memory assessment. Recent versions of these tests include the California Verbal Learning Test-II (CVLT-II; Delis, Kaplan, Kramer, & Ober, 2000), Hopkins Verbal Learning Test-R (HVLT-R; Brandt & Benedict, 2001), World Health Organization/UCLA Auditory Verbal Learning Test (WHO/UCLA AVLT; Maj et al., 1993), Rey Auditory Verbal Learning Test (RAVLT; Schmidt, 1996), Neuropsychological Assessment Battery (NAB; Stern & White, 2003), and List Learning in the Center to Establish Registry for Alzheimer’s Disease (CERAD; Morris et al., 1989; Welsh, Butters, Hughes, Mohs, & Heyman, 1991). Other commonly used list-learning tasks (e.g., ADASCOG; Rosen, Mohs, & Davis, 1984), like the Free and Cued Selective Reminder Task (FCSRT; Buschke, Sliwinski, Kuslansky, Katz, Verghese, & Lipton, 2006; Grober, Merling, Heimlich, & Lipton, 1997), are not solely auditory, adding visual words or picture presentations of items. List-learning tasks share many design features. The number of words in the to-be-learned list (e.g., 12–16 items) has been designed to exceed the typical vigilance span of seven items and to stress learning demands. The learning phase is the presentation of the list across several trials. Following each trial, the examinee is asked to recall as many items as possible. Once the learning is complete, a short time interval elapses, usually including interference tasks designed to prevent rehearsal of the list items. The examinee is then asked to recall items from the list, constituting a short term recall. After another longer interval, again containing some distraction, a recall is requested. These short- and long-term retrieval assessments capture the examinee’s ability to store, consolidate, and maintain information, as well as to retrieve it on command. Tests can include a subsequent multiple- or forced-choice task to facilitate access, capturing the items that were encoded but could not be accessed on free retrieval. Despite commonalities, instruments vary in content and administration. List construction on some tests (e.g., ▶ CVLT-II, ▶ HVLT-R, WHO/UCLA ▶ AVLT, and


▶ NAB) incorporates semantic categories, whereas others do not (e.g., ▶ RAVLT, ▶ CERAD, and ADAS-COG). Examinees are not initially informed of these categories in order to determine whether they can exploit semantic information to their advantage and to facilitate their ability to organize, encode, and learn more items. To ensure and document that any particular item is fully encoded with semantic knowledge, the FCSRT adopts an alternate use of semantic organization by cueing examinees with the semantic category while the word is being learned and by prompting the category immediately afterward. Another variation among tests is the use of interference lists. For instance, after the initial learning phase of the target lists in the CVLT-II and NAB, an alternate list is administered, designed to share some but not all of the semantic categories of the target list. This helps to address susceptibility to interference and source learning. Other tests (e.g., ▶ HVLT-R and FCSRT) do not have prescribed interference tasks, although other word-list or naming tasks should not be used during the delay. Delayed recall is commonly tested after a 20–30min interval. There are various methods to assess the recognition memory of the target list. Examinees can identify target-list words among a list of distracters (CVLT-II, HVLT-R, and NAB) or identify targets in forced- (CVLT-II) or multiple-choice (FCSRT) recognition trials. While most tasks use recognition after short(NAB) or long-term (CVLT-II and HVLT-R) free recall, one test (ADAS-COG) relies exclusively on the immediate recognition to assess learning. AVLTs reveal significant information about learning and memory processes. The multiple trial exposure generates an individual’s learning curve, indicating whether information learned on an earlier trial is maintained and appended with new information. Learning characteristics, particularly serial position effects (i.e., primacy and recency effects), provide insight about how learning occurs. For instance, the dual storage model (Raaijmakers & Shiffrin, 1981) suggests that items from primacy and middle regions of the lists are thought to reflect longterm storage, whereas recent items remain in immediate working memory. The position of an item in the list, known as the distinctiveness feature, can also aid in later recall, with the distinct first or last items having preference. Susceptibility to proactive and/or retroactive interference of the interference list or shared semantic categories of target items are analyzed. Long-term retention of verbal information can be parsed into storage and retrieval components via examination of free-recall versus recognition scores.

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Historical Background Word lists have been used to assess learning and memory for over a century. Ebbinghaus (1885) was early to observe and describe the serial position phenomenon. Eduoard Clapare`de (Boake, 2000) assessed learning and memory in his work on child pedagogy in 1916 using a 15-item word list, which was adapted by Andre´ Rey to develop the RAVLT in 1941. The RAVLT, first published in France, has been adapted since its development and modified for use in multiple languages. In 1996, the RAVLT manual (Schmidt, 1996) was published, providing standard instructions on administration, scoring, and interpretation. The original CVLT, published in 1987 (Delis, Kramer, Kaplan, & Ober, 1987), incorporated semantic categories, scorings of learning characteristics, use of semantic strategy, and contrast measures of learned and retrieved items. The CVLT-II revision (Delis et al., 2000), published in 2000, adopted new norms. The CVLT–Children’s Version (Delis, Kramer, Kaplan, & Ober, 1994) is appropriate for children in the age group of 5–16 years. The HVLT (Brandt, 1991) was introduced with six alternate forms designed for longitudinal, repeated testing, and the revised version (Brandt & Benedict, 2001) incorporated delayed recall and recognition trials. The newest list-learning task from the NAB (Stern & White, 2003) has been normed on individuals up to 97 years of age. The WHO/UCLA AVLT was designed by the WHO in 1993 to better evaluate examinees worldwide (Maj et al., 1993). When designing this instrument, the authors were careful to select words that were familiar across multiple cultures. Similar to the CVLT-II, the words comprising the WHO/UCLA AVLT can be classified into categories. However, the categories of the WHO/UCLA AVLT are universally familiar (i.e., body parts, animals, tools, household objects, and transportation vehicles). Of note, the WHO/UCLA AVLT is a component of the Neuropsychological Screening Battery for Hispanics (Ponton et al., 1996).

Psychometric Data Normative data for AVLTs span an age range of 7–97 years. Groups used in normative data include healthy adults, men, women, and Hispanic individuals. Stratified norms according to age, sex, ethnicity, educational level, and region of the country (e.g., Northeast) are also available. Many tests (e.g., ▶ CVLT-II and ▶ CERAD) have been normed for European, Asian, and Southeast Asian groups.

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Direct translation of word lists into other languages should be avoided, as words may have been selected to accommodate word frequency within a language or appropriateness within a culture. Some AVLTs are targeted for certain populations using shorter versions and more age-appropriate word lists (e.g., ▶ CVLT-C). Shorter forms have also been adopted for more impaired individuals (e.g., ▶ CVLT-II, Short Form; Delis et al., 2000). Verbal learning tests are used to evaluate memory performance over time. Test–retest reliability using the same version of the test has been evaluated for 1-month as well as 1-year intervals. Many tests have adopted alternate or multiple forms. Whereas alternate versions can reduce practice effects, there is evidence that these effects are not completely eliminated (Houx et al., 2002) due to procedural learning. Therefore, examiners must be very cognizant of practice effects. Even after 2 years, healthy older adults showed practice effects on the CVLT-II (Blasi, Zehnder, Berres, Taylor, Spiegel, & Monsch, 2009). Construct validity of adult and child versions of the CVLT in assessing learning and memory has been examined in several studies. Attention span, learning efficiency, delayed recall, and inaccurate recall represent one example of a four-factor model that was identified by a factor analysis in a sample of children with traumatic brain injury (Mottram & Donders, 2005). Variability across studies may be a result of methodological differences, demographic differences, severity of patient population, and/or time elapsed between disease onset and assessment. In terms of inter-test relationships, tests are not interchangeable, as word lists and administrations vary significantly. However, the RAVLT correlates moderately with the CVLT-II. The CVLT-II is now incorporated into the Wechsler Memory Scale – IV as a valid component of the memory indices. The HVLT-R also shows a high correlation with the CVLT-II, although it may be more appropriate for greater disease severity.

Clinical Uses Verbal learning tests are ubiquitous in assessments of memory-impaired populations. Amnesic disorders, degenerative dementias (including Alzheimer’s, Parkinson’s, and Huntington’s disease), temporal lobe epilepsy, traumatic brain injury, depression, and focal stroke are diseases that have not only benefited from these tests, but also have promoted refinements and further development to extend our knowledge of the neuropsychological underpinnings of memory function. These instruments evaluate both quantitative and procedural aspects of

verbal learning, providing detailed information about an individual’s capacity to acquire, consolidate, store, and retrieve information, thus revealing significant aspects of the learning and retrieval processes. Learning characteristics, particularly primary and recency serial position effects (Foldi, Brickman, Schaefer, & Knutelska, 2003), are highly informative in differentiating clinical populations and provide insight about how learning is occurring. A detailed step-by-step exploration of each individual’s learning process enables the clinician or researcher to identify specific areas of vulnerability and can guide intervention strategies. Auditory verbal learning tasks have been instrumental in assessing change in memory over time. The auditory verbal learning paradigm has been very sensitive in discriminating between healthy and memory-impaired groups. A vast literature demonstrates the sensitivity of these tests to the detection of prodromal Alzheimer’s disease.

Cross References ▶ California Verbal Learning Test-II ▶ California Verbal Learning Test-II, Children’s Version ▶ Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) ▶ Hopkins Verbal Learning Test – Revised ▶ Memory ▶ Neuropsychological Assessment Battery ▶ Rey Auditory Verbal Learning Test

References and Readings Blasi, S., Zehnder, A. E., Berres, M., Taylor, K. I., Spiegel, R., & Monsch, A. U. (2009). Norms for change in episodic memory as a prerequisite for the diagnosis of mild cognitive impairment (MCI). Neuropsychology, 23, 189–200. Boake, C. (2000). Edouard Clapare`de and the Auditory Verbal Learning Test. Journal of Clinical and Experimental Neuropsychology, 22, 286–292. Brandt, J. (1991). The Hopkins Verbal Learning Test: Development of a new memory test with six equivalent forms. The Clinical Neuropsychologist, 5, 125–142. Brandt, J., & Benedict, R. (2001). Hopkins Verbal Learning Test-Revised. Lutz, FL: Psychological Assessment Resources. Buschke, H., Sliwinski, M. J., Kuslansky, G., Katz, M., Verghese, J., & Lipton, R. B. (2006). Retention weighted recall improves discrimination of Alzheimer’s disease. Journal of the International Neuropsychological Society, 12, 436–440. Delis, D. C., Kaplan, E., Kramer, J. H., & Ober, B. A. (2000). California Verbal Learning Test – II (2nd ed.). San Antonio, TX: The Psychological Corporation.

Augmentative and Alternative Communication (AAC) Delis, D. C., Kramer, J., Kaplan, E., & Ober, B. A. (1994). California Verbal Learning Test – Children’s Version. San Antonio, TX: Pearson. Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (1987). California Verbal Learning Test: Adult Version. New York: The Psychological Corporation. Ebbinghaus, H. (1885). Memory: A contribution to experimental psychology (H. A. Ruger & C. E. Bussenenues, Trans., 1913). New York: Teachers College, Columbia University. Foldi, N. S., Brickman, A. M., Schaefer, L. A., & Knutelska, M. E. (2003). Distinct serial position profiles and neuropsychological measures differentiate late life depression from normal aging and Alzheimer’s disease. Psychiatry Research, 120, 71–84. Grober, E., Merling, A., Heimlich, T., & Lipton, R. B. (1997). Free and cued selective reminding and selective reminding in the elderly. Journal of Clinical and Experimental Neuropsychology, 19, 643–654. Houx, P. J., Shepherd, J., Blauw, G. J., Murphy, M. B., Ford, I., Bollen, E. L., et al. (2002). Testing cognitive function in elderly populations: The PROSPER study. Journal of Neurology, Neurosurgery, and Psychiatry, 73, 385–389. Maj, M., D’Elia, L., Satz, P., Janssen, R., Zaudig, M., Uchiyama, C., et al. (1993). Evaluation of two new neuropsychological tests designed to minimize cultural bias in the assessment of HIV-1 seropositive persons: AWHO study. Archives of Clinical Neuropsychology, 8, 123–135. Morris, J. C., Heyman, A., Mohs, R. C., Hughes, J. P., van Belle, G., Fillenbaum, G., et al. (1989). The Consortium to Establish a Registry for Alzheimer’s Disease (CERAD). Part I. Clinical and neuropsychological assessment of Alzheimer’s disease. Neurology, 39, 1159–1165. Mottram, L., & Donders, J. (2005). Construct validity of the California Verbal Learning Test-Children’s Version (CVLT-C) after pediatric traumatic brain injury. Psychological Assessment, 17, 212–217. Ponton, M. O., Satz, P., Herrera, L., Ortiz, F., Urrutia, C. P., Young, R., et al. (1996). Normative data stratified by age and education for the Neuropsychological Screening Battery for Hispanics (NeSBHIS): Initial report. Journal of the International Neuropsychological Society, 2, 96–104. Raaijmakers, J. G. W., & Shiffrin, R. M. (1981). Search of associative memory. Psychological Review, 88, 93–134. Rosen, W. G., Mohs, R. C., & Davis, K. L. (1984). A new rating scale for Alzheimer’s disease. American Journal of Psychiatry, 141, 1356–1364. Schmidt, M. (1996). Rey Auditory Verbal Learning Test: A handbook. Los Angeles: Western Psychological Services. Stern, R. A., & White, T. (2003). Neuropsychological Assessment Battery. Lutz, FL: Psychologial Assessment Resources. Welsh, K., Butters, N., Hughes, J., Mohs, R., & Heyman, A. (1991). Detection of abnormal memory decline in mild cases of Alzheimer’s disease using CERAD neuropsychological measures. Archives of Neurology, 48, 278–281.

Auditory-Sound Agnosia ▶ Auditory Agnosia

Auditory-Verbal Agnosia ▶ Auditory Agnosia

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Augmentative and Alternative Communication (AAC) D IANE C ORDRY G OLDEN Association of Assistive Technology Act Programs Delmar, NY, USA

Definition Augmentative and alternative communication (AAC) is a set of procedures and processes by which an individual’s communication skills can be maximized for functional and effective communication. AAC approaches supplement or replace natural speech with aided options that incorporate the use of some type of device ranging from simple picture communication systems to complex speech generating devices and/or unaided options that involve only the individual’s body, such as sign language. AAC may be used to augment understanding as well as written or oral expression. A ‘‘multimodal’’ approach that includes both devices and unaided strategies may be most effective in meeting the individual’s communication needs.

Historical Background Prior to about 1970, AAC was not a widely accepted intervention technique and could even be described as contraindicated in the professional literature. At that time, it was thought that the act of vocal production was a critical building block of human language development. As a result, interventionists believed that providing an alternative to speech production would deter speech (and thus language) development because the child would choose to use the ‘‘easier’’ alternative mechanism (Bates, 1976; Fourcin, 1975). In the late 1960s and in the 1970s, research evidence indicated speech was actually a secondary component to language function and that robust receptive and expressive language could be developed without vocal production. In many cases, the use of alternatives to speech production was found to actually support (and in no way deter) vocal production (Schlosser & Wendt, 2008; Silverman, 1980; Zangari, Lloyd, & Vicker, 1994). In 1980, the American Speech-Language-Hearing Association (ASHA) established an Ad Hoc Committee on Communication Processes and Nonspeaking Persons that developed a position statement outlining the concept of AAC as a set of intervention techniques using a variety of

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symbol sets and communication interaction behaviors. This became the framework for the field of AAC today. At about the same time, new computer technologies were exploding onto the scene creating previously unimaginable opportunities for AAC device development. The Trace Research and Development Center, part of the College of Engineering at the University of WisconsinMadison, was formed in 1971 to address the communication needs of people with severe disabilities who were nonspeaking. The Center was an early leader and innovator in the augmentative communication field and pioneered development of electronic communication aids in the 1970s and 1980s that became prototypes for the speech generating devices (SGDs) of today (Vanderheiden, 1978; Vanderheiden & Grilley, 1976).

Rationale or Underlying Theory Today the widespread acceptance of AAC is a valid intervention option to develop viable means of effective communication for any individual with limited ‘‘natural’’ speech, as well as to enhance comprehension for those who are not hearing impaired, but who have difficulty understanding spoken language. In 1992, the National Joint Committee for the Communication needs of Persons with Severe Disabilities issued a Communication Bill of Rights that unequivocally states, ‘‘All persons, regardless of the extent or severity of their disabilities, have a basic right to affect, through communication, the conditions of their own existence.’’ A set of 12 specific rights are described in the Bill of Rights including, ‘‘the right to have access at all times to any needed augmentative and alternative communication devices and other assistive devices and to have those devices in good working order.’’

Goals and Objectives Most of the early history of AAC focused on individuals with neuromotor impairments that limited oral-motor skills such as cerebral palsy and amyotrophic lateral sclerosis. However, with the passage of landmark legislation such as the Education of Handicapped Children’s Act (P.L. 94–142), Section 504 of the Rehabilitation Act, and later the Americans with Disabilities Act, individuals with all types of disabilities have become more integrated into education, employment, community living, and society in general. This change created a widespread need for individuals with all types of disabilities to have an effective, functional communication system. More complex and efficient AAC

systems have been developed to meet this need with some specifically focused on individuals with neuropsychological disabilities such as autism (Glennen & DeCoste, 1997). While the core goal of AAC is to provide effective communication, related objectives can include decreasing problem behaviors and increasing successful education, employment, and community living outcomes.

Treatment Participants Early in the history of AAC, two misconceptions thrived regarding candidacy for AAC – that a set of prerequisite skills (usually cognitive and motor) was required before AAC could be considered and AAC should only be implemented after all traditional forms of speech therapy had failed. Both have been proven unsubstantiated as many successful AAC users have severe motor and/or cognitive impairments, and research has shown no justification for waiting to implement AAC as it can support speech development (Shane & Bashir, 1980). As a result, candidacy for AAC is not limited by age, disability diagnosis or prerequisite cognitive or motor skills. Individuals who can benefit from AAC may be of all ages, including infants and toddlers with disabilities, and may have diagnoses including apraxia, dysarthria, aphasia, autism, ALS, cerebral palsy, multiple sclerosis, Parkinsons, mental retardation, etc.

Treatment Procedures The challenges for those providing intervention are maintaining current knowledge of the vast array of available AAC device options, appropriately matching the skill sets of individuals with disabilities to available AAC systems, securing funding to acquire the system, improving communication opportunities and environments, and providing supports sufficient to ensure effective use of the system. Consideration for aided AAC intervention begins with assessment by a qualified team of individuals who are knowledgeable about the individual and his other strengths and limitations especially in the areas of speech, language, and motor skills. One or more team members should be knowledgeable about the range of potential AAC alternatives available and those that are viable options to meet the individual’s communication needs. Appropriate practice in assessment includes conducting structured device trials with various AAC devices in the environment(s) in which the individual will be using the technology (e.g., home, school, community, etc.). This allows for comparative analysis of different device

Augmentative and Alternative Communication (AAC)

features and functions to determine which best address the individual’s functional communication needs. The result of an AAC assessment is identification of the system features appropriate for an individual including specification of device input features (selection techniques), message characteristics, and output features. Once an appropriate AAC system has been acquired, training and support for the user, their family, and others (e.g., teachers, therapists, etc.) must be implemented. More complex AAC systems frequently require initial programming and device setup as part of user support services. Short and long-term communication goals should be developed using the AAC system and therapy services implemented to support goal achievement (Beukleman, Garrett, & Yorkston, 2007; Beukelman & Mirenda, 2005; www.aac-rerc.com).

Efficacy Information Efficacy research on AAC ranges from observation of changes in functional communication skills to potential secondary improvements in academic, social, behavioral, and other areas. For individuals with limited or no functional communication, an AAC system that delivers basic communication ability can be deemed effective by self-verification of communication occurring (FriedOken & Bersani, 2000). Beyond this basic gauge of AAC efficacy, research has been done on a variety of specific outcomes such as decreasing problem behaviors through the use of AAC (Vaughn & Horner, 1995), enhancing the rate of AAC communication, (Venkatagiri, 1993), and even nuances such as speech synthesizer intelligibility (Mirenda & Beukelman, 1990) all in an effort to support AAC efficacy. Seminal work on AAC efficacy done by Ralf Schlosser (2003) addressed a wide range of AAC efficacy issues including the role of AAC in facilitating or hindering natural speech development, literacy development in AAC users, and the effects of speech output (use of speech generating devices). In 2001, Medicare began coverage of speech generating devices (SGDs) based on acceptance of AAC efficacy research. Since then, a number of private insurance carriers have followed suit and now cover SGDs as do most state Medicaid programs.

Outcome Measurement The most direct outcome measure for AAC intervention is demonstration of effective and efficient communication.

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Any number of standardized communication measures can be used to document communication efficacy using the AAC system. In addition, the Adaptive Technology Resource Center had identified a variety of outcome measurement tools that focus on the functional efficiency and functional gains achieved through the use of assistive technologies including AAC, see http://atrc.utoronto.ca/ index.php?option = com_content&task = view&id = 178&Itemid = 69.

Qualifications of Treatment Providers AAC intervention is typically provided by speechlanguage pathologists (SLPs) who are licensed by states as health care providers, educated at the graduate level in the study of human communication, its development and its disorders. Medicare requires an SLP who provides AAC assessments or treatment to hold the Certificate of Clinical Competence (CCC) in speechlanguage pathology from the American SpeechLanguage-Hearing Association. In addition to SLPs, some other types of professional providers may be members of the intervention team providing AAC services, especially in the educational environment, e. g., special educators, occupational therapists, assistive technology practioners, etc. (ASHA, 2002; ASHA, 2004; ASHA, 2005).

Cross References ▶ Articulation ▶ Articulation Disorder ▶ Assistive Technology ▶ Speech ▶ Speech/Communication Disabilities ▶ Speech-Language Pathology ▶ Speech-Language Therapy ▶ Speech Sound Disorders

References and Readings American Speech-Language-Hearing Association. (1981). Position statement on non-speech communication. ASHA, 23, 577–581. American Speech-Language-Hearing Association. (2002). Augmentative and alternative communication: Knowledge and skills for service delivery [Knowledge and Skills]. Retrieved from www.asha.org/policy

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American Speech-Language-Hearing Association. (2004). Roles and responsibilities of speech-language pathologists with respect to augmentative and alternative communication: Technical report [Technical Report]. Retrieved from www.asha.org/policy American Speech-Language-Hearing Association. (2005). Roles and responsibilities of speech-language pathologists with respect to augmentative and alternative communication: Position statement [Position Statement]. Retrieved from www.asha.org/policy Bates, E. (1976). Language and context: The acquisition of pragmatics. New York: Academic Press. Beukleman, D., Garrett, K., & Yorkston, K. (2007). Augmentative and alternative communication strategies for adults with acute or chronic medical conditions. Baltimore: Paul H. Brookes Publishing Company. Beukelman, D., & Mirenda, P. (2005). Augmentative and alternative communication: Management of severe communication disorders in children and adults (3rd ed.). Baltimore: Paul H. Brookes Publishing Company. Fourcin, A. J. (1975). Language development in the absence of expressive speech. In E. H. Lenneberg & E. Lenneberg (Eds.), Foundations of language development (Vol. II). New York: Academic Press. Fried-Oken, M., & Bersani, H. (2000). Speaking up and spelling it out: Personal essays on augmentative and alternative communication. Baltimore: Paul H. Brookes Publishing Company. Glennen, S. L., & DeCoste, D. C. (1997). Handbook of augmentative and alternative communication. San Diego: Singular Publishing Group. Mirenda, P., & Beukelman, D. (1990). A comparison of intelligibility among the natural speech and seven speech synthesizers with listeners from three age groups. Augmentative and Alternative Communication, 6, 61–68. National Joint Committee for the Communication Needs of Persons with Severe Disabilities. (1992). Guidelines for meeting the communication needs of persons with severe disabilities. ASHA, 34(Suppl. 7), 1–8. Retrieved from www.asha.org/policy or www.asha.org/njc Schlosser, R. W. (2003). The efficacy of augmentative and alternative communication: Toward evidence-based practice. San Diego: Academic Press. Schlosser, R. W., & Wendt, O. (2008). Effects of augmentative and alternative communication intervention on speech production in children with autism: A systematic review. American Journal of Speech-Language Pathology, 17, 212–230. Shane, H., & Bashir, A. S. (1980). Election criteria for the adoption of an augmentative communication system: Preliminary considerations. Journal of Speech and Hearing Disorders, 45, 408–414. Silverman, F. (1980). Communication for the speechless. Englewood Cliffs, NJ: Prentice-Hall. Vanderheiden, G. C. (1978). Non-vocal communication resource book. Baltimore: University Park Press. Vanderheiden, G. C., & Grilley, K. (1976). Non-vocal communication techniques and aids for the severely physically handicapped. Austin, TX: Pro-Ed. Vaughn, B., & Horner, R. (1995). Effects of concrete versus verbal choice systems on problem behavior. Augmentative and Alternative Communication, 11, 89–92. Venkatagiri, H. S. (1993). Efficiency of lexical prediction as a communication acceleration technique. Augmentative and Alternative Communication, 9, 161–167. Zangari, C., Lloyd, L. L., & Vicker, B. (1994). Augmentative and alternative communication: A historic perspective. Augmentative and Alternative Communication, 10, 27–59.

Aura K ENNETH P ERRINE Northeast Regional Epilepsy Group Hackensack, NJ, USA Weill-Cornell College of Medicine New York, NY, USA

Definition An aura is a paroxysmal episode that occurs before several types of neurologic events. It is a type of warning heralding the onset of the ictal event such as a migraine or an epileptic seizure. Auras usually last longer in migraines (up to minutes) than in seizures (typically several seconds). Episodes longer in duration or more remote from the ictus in both migraine and seizures are called prodromes. In both disorders, auras can represent any disruption of neurologic function, the specific phenomena of which arise from the localization of their onset in the brain.

Current Knowledge Auras can consist of disruptions or activations of primary sensory modalities (touch, hearing, smell, taste, vision), including paresthesias (somatosensory hallucinations that feel like tingling or ‘‘pins and needles’’) or numbness, unformed (noises, distortions) or formed (voices, songs, commercial jingles) auditory hallucinations or transient deafness, and olfactory or gustatory hallucinations (usually noxious, such as burned rubber). Visual hallucinations are especially common in migraine, and can include loss of vision such as blind spots or hemianopsia (loss of vision on one side), positive phenomena such as ‘‘scintillating scotoma’’ (flickering spots of light that may begin centrally and extend to arcs of flickering white or colored lights), or zig-zag or other geometric lines or patterns. Formed visual hallucinations can occur (especially in epilepsy), which are usually described as animals or cartoon characters. Visual distortions can also occur, such as macropsia/micropsia (seeing objects larger/smaller) and telescopia/micropsia (seeing objects farther away/closer). In epilepsy, more complex somatosensory auras can occur, such as a rising epigastric sensation (feeling the stomach rising up to the mouth) or ineffable ‘‘feelings’’ that the patient cannot elucidate. Complex experiential psychic auras can also occur (especially in epilepsy), such as de´ja` vu, out-of-body experiences, depersonalization,

Autism Diagnostic Interview, Revised

derealization, bizarre perceptual phenomena, etc. Psychological symptoms such as anxiety, panic, and fear are also common. In migraines, auras are thought to be caused by the vascular phenomena causing the headache. Of note, migraine auras can occur without any subsequent headache. In epilepsy, auras are actually simple partial seizures produced by epileptiform electrical discharges that affect one brain region alone; they do not disrupt consciousness, and can be recalled by the patient after the ictus.

Cross References ▶ Epilepsy ▶ Partial Seizures

References and Readings Allan Ropper, A. H., & Samuels, M. (2009). Adams and Victor’s principles of neurology (9th ed.). New York: McGraw-Hill. Engel, J., & Pedley, T. A. (Eds.). (2008). Epilepsy: A comprehensive textbook (2nd ed.). New York: Lippincott Williams & Wilkins. www.epilepsyfoundation.org

Authoritative Reference ▶ Learned Treatise

Autism ▶ Autistic Disorders

Autism Diagnostic Interview, Revised S O H YUN K IM , C ATHERINE L ORD University of Michigan Autism and Communication Disorders Center (UMACC) Ann Arbor, MI, USA

Synonyms ADI-R

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Description The current edition of the Autism Diagnostic InterviewRevised (ADI-R; Rutter, Le Couteur, & Lord, 2003) is a standardized, semistructured, and investigator-based interview for parents or caregivers of individuals with autism. It provides a diagnostic algorithm for the ICD-10 definition of autism (World Health Organization [WHO], 1992) and DSM-IV (American Psychiatric Association [APA], 1993). The interview is appropriate for the diagnostic assessment of any person within the age range extending from early childhood to adult life, provided that they have a non-verbal mental age above 2 years. The ADI-R includes 93 items in three domains of functioning – language/communication, reciprocal social interactions, and restricted, repetitive, and stereotyped behaviors and interests, as well as other aspects of behaviors. Up to 42 of the interview items are systematically combined to produce a formal, diagnostic algorithm for autism as specified by the authors, or a general diagnosis of autism spectrum disorders (ASD) as used in several collaborative studies (Risi et al., 2006). All items in the ADI-R are coded in terms of whether the behavior is ‘‘currently’’ occurring, and whether it ‘‘ever’’ occurred, or occurred during a specifically defined period in preschool years. The diagnostic algorithm is based on the ‘‘ever/most abnormal’’ codes in preschool years, but current scores can be used to facilitate a clinical diagnosis. Most of the ADI-R pertains to behaviors that are rare in individuals who do not have ASD and/or who do not have profound intellectual disabilities. Thus, numerical estimates of the typical scores of general population have not been obtained. Researchers have used scores in the domains or overall as estimates of severity of autistic symptoms. However, the validity of this approach has not been directly tested. Scores have been published for many research populations but not yet systematically dimensionalized.

Historical Background The WPS Edition of the ADI-R (2003) is a modified version of the 1994 version (Lord, Rutter, & Le Couteur, 1994), which was based on the original Autism Diagnostic Interview (ADI; Le Couteur et al., 1989). The 1994 version was somewhat shorter than the original in order to make the interview more appropriate for clinical, as well as research, usage. The diagnostic algorithm developed for the 1994 version remains unaltered (apart from minor changes in age cutoff).

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Autism Diagnostic Interview, Revised

Psychometric Data Psychometric properties for the original ADI were provided for a carefully selected, blindly interviewed and coded, sample of 16 autistic and 16 mentally handicapped children and adults covering a range of IQs and chronological ages. Inter-rater reliability was assessed for a sample of ten children with autism and ten without, with multirater kappas ranging from 0.55 to 0.94 for each item and intraclass correlations above 0.94 for all subdomain and domain scores. The majority of individual items showed good discriminative validity showing diagnostic differences across autistic and mentally handicapped groups (Le Courteur et al., 1989). Psychometric properties for the current ADI-R were provided for a carefully selected, blindly interviewed and coded, sample of 25 autistic and 25 mentally handicapped children ranged in chronological age from 36 to 59 months, with mental ages ranging from 21 to 74 months. Inter-rater reliability was assessed, with multirater kappas ranging from 0.63 to 0.89 for each item and intraclass correlations above 0.92 for all subdomain and domain scores. Following the initial standardization study of the ADI-R, a further study was undertaken of a separate sample of 53 children with autism and 41 nonautistic children with mental handicap or language impairments (Lord et al., 1993). Inter-rater reliability was as high as the initial study, with multirater kappas ranging from 0.62 to 0.96 for individual items. Test-retest reliability was very high, with all coefficients being in the 0.93–0.97 range. Majority of individual items showed good discriminative validity showing diagnostic differences across autistic and mentally handicapped groups (see Lord et al., 1994). The algorithm cutoffs were determined by identifying the point within each area that yielded the best combination of sensitivity and specificity both exceeding 0.90.

Clinical Uses The ADI-R offers a profile of a child in different areas including language/communication, reciprocal social interactions, and restricted, repetitive, and stereotyped behaviors and interests based on the parents’ detailed descriptions of the history and behaviors of the child. It can provide a comprehensive description of a child both currently and in earlier ages, but must be used in conjunction with observations and/or direct testing in making a diagnosis of ASD. The ADI-R can provide a useful structure to obtain history and understand a parent’s perspective on their children’s symptoms

associated with ASD, but requires approximately two hours to administer and substantial practice to do so reliably. The Diagnostic Algorithms are sets of rules that allow classification of patterns of behavior according to whether or not they meet the current DSM-IV or ICD-10 diagnostic criteria of autism and nonautistic ASD. One caveat for clinical users is that they should be aware that diagnostic algorithm result and a true clinical diagnosis are not the same. Clinical diagnosis is based on multiple sources of information, including direct observation. Thus, even though the ADI-R provides broader contexts including the information about history or functioning of a child than observations, ADI-R alone cannot be used to make a complete standard diagnosis. Current Behavior Algorithms can be used to assess the participant’s current behavior. This can be used in clinical settings to assess changes brought about by intervention or changes reflecting increasing developmental maturity or changing life circumstances. The ADI-R should only be used by appropriately experienced clinicians. Interviewers must be familiar with the concepts of ASD and relevant behaviors. Training workshops and videotapes are available to help clinicians understand the scoring and administration of the ADI-R.

Cross References ▶ Autism Diagnostic Observation Schedule ▶ Autistic Disorder ▶ Childhood Autism Rating Scales ▶ Modified Checklist for Autism in Toddlers (M-CHAT) also CHAT

References and Readings American Psychiatric Association. (1993). Options book for DSM-IV. Washington, DC: Author. DiLavore, P., Lord, C., & Rutter, M. (1995). The pre-linguistic autism diagnostic observation schedule (PL-ADOS). Journal of Autism and Developmental Disorders, 25, 355–379. Le Couteur, A., Rutter, M., Lord, C., Rios, P., Robertson, S., Holdgrafer, M., et al. (1989). Autism diagnostic interview: A semistructured interview for parents and caregivers of autistic persons. Journal of Autism and Developmental Disorders, 19, 363–387. Lord, C., Rutter, M., & Le Couteur, A. (1994). Autism diagnostic interview-revised: A revised version of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders. Journal of Autism and Developmental Disorders, 24(5), 659–685. Risi, S., Lord, C., Gotham, K., Corsello, C., Chrysler, C., Szatmari, P., et al. (2006). Combining information from multiple sources in the

Autism Diagnostic Observation Schedule diagnosis of autism spectrum disorders. Journal of the American Academy of Child and Adolescent Psychiatry, 45, 1094–1103. Rutter, M., Le Couteur, A., & Lord, C. (2003). Autism diagnostic interview-revised. Los Angeles: Western Psychological Services. World Health Organization. (1992). The ICD-IO classification of mental and behavioral disorders: Clinical descriptions and diagnostic guidelines. Geneva: Author.

Autism Diagnostic Observation Schedule S O H YUN K IM , C ATHERINE L ORD University of Michigan Autism and Communication Disorders Center (UMACC) Ann Arbor, MI, USA

Synonyms ADOS

Description The Autism Diagnostic Observation Schedule (ADOS; Lord, Rutter, DiLavore, & Risi, 2001) is a semistructured, standardized assessment of communication, social interaction, and play or imaginative use of materials for individuals who have been referred because of possible autism spectrum disorders (ASD). As part of the schedule, planned social occasions, referred to as ‘‘presses’’ (Lord, Rutter, Goode, & Heemsbergen, 1989) are created in which a range of social initiations and responses is likely to appear. In the same way, communication opportunities are designed to elicit a range of interchanges. Play situations are included to allow observation of a range of imaginative activities and social role-play. A variety of structured activities and materials, and less structured interactions, provide standard contexts within the ADOS in which the social, communicative, and other behaviors relevant to the understanding of ASD are observed. The ADOS consists of four modules. Each module is appropriate for children and adults at different developmental and language levels, ranging from no expressive or receptive use of words, to fluent, complex language in an adult. Only one module, lasting about 30 minutes, is administered to any individual at a given point of time. In the ADOS, the examiner uses the module that best matches the expressive language skills of the individual child or adult in

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order to make judgments about social and communicative abilities as independent as possible from the effects of absolute level of language delay. Each module has its own protocol, which contains a schedule of activities designed for use with children or adults at particular developmental and language levels. Recently, the Toddler Module of the ADOS was developed for use in children between 12 and 30 months of age in addition to the original four modules (Lord, Luyster, Gotham, & Guthrie, in press). Module 1 is intended for children who do not use spontaneous phrase speech consistently. It consists of 10 activities with 29 accompanying ratings. Module 2 is intended for children with some flexible phrase speech who are not verbally fluent. It consists of 14 activities with 28 accompanying ratings. Module 3 provides 13 activities and 28 ratings. It is intended for verbally fluent children for whom playing with toys is age-appropriate. The operational definition of verbal fluency is the spontaneous, flexible use of sentences with multiple clauses that describe logical connections within a sentence. It requires the ability to talk about objects or events not immediately present. Module 4 contains the socioemotional questions, along with interview items about daily living and additional tasks. It is intended for verbally fluent adults and for adolescents who are not interested in playing with toys such as action figures (usually over 12–16 years). This module consists of 10–15 activities with 31 accompanying ratings. The difference between Modules 3 and 4 lies primarily in whether information about social communication is acquired during play or through a conversational interview. It is important to note that adolescents or adults may feel uncomfortable when presented with the toys for young children that are available in modules 1 and 2; suggestions for modifying the earlier modules to be appropriate for older children or adults who are less verbal are available from the authors. In addition to the four modules, the Toddler Module is intended for children between 12 and 30 months of age who should have a nonverbal age equivalent of at least 12 months and be walking independently. It consists of 11 activities with 41 accompanying ratings (Lord et al., in press). The ADOS provides the diagnostic algorithms that are sets of rules that allow classification of autism or ASD. Separate diagnostic algorithms for each module can be generated using subsets of items in each module. Items and the thresholds for the classification of autism and of ASD in the algorithms differ for each module. However, the general principles and procedures for computation are the same across modules and similar to the DSM-IV (American Psychiatric Association, 1993) and ICD-10 (World Health Organization, 1992). The algorithms for

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Module 1, 2, and 3 were recently revised from the previous algorithms (Gotham, Risi, Pickles, & Lord, 2007). Reflecting recent research, the revised algorithms now consist of two new domains, Social Affect and Restricted, Repetitive Behaviors, combined to one score to which thresholds are applied, resulting in generally improved predictive validity compared to the previous algorithms. The module 1 consists of no words and some words algorithms by language level. The module 2 includes ‘‘Younger than 5’’ and ‘‘Greater or Equal to 5’’ algorithms by chronological age. Module 3 includes a single algorithm. All items appearing on the new algorithms contribute to a single score with two classification thresholds, one for autism and another for ASD. There are the Toddler Module algorithms for children between 12 and 30 months, who do not have phrase speech; once children have developed phrase speech, they should be administered module 2. Since differential diagnosis can be challenging especially in toddlers, the toddler module algorithms generate range of concern (little-or-no concern; mild-to-moderate concern; moderate-to-severe concern) rather than strict classifications. Most of the ADOS pertains to behaviors that are rare in individuals who do not have ASD and/or who do not have profound intellectual disabilities. Thus, numerical estimates of the typical scores of general population have not been obtained. Scores from module 1 to 3 have now been calibrated for children with ASD to yield a standard severity score based on a large sample (see below; Gotham, Pickles, & Lord, in press).

Historical Background In its present form, the current WPS Edition of the ADOS (Lord et al., 2001) is a combination of two similar diagnostic instruments: the 1989 version of the ADOS (Lord et al., 1989) and the Pre-Linguistic ADOS (PL-ADOS; DiLavore, Lord, and Rutter, 1995). The ADOS was first introduced in the 1980s as a method of standardizing direct observations of social behavior, communication, and play in children suspected of having autism. It was intended to be administered to children between the ages of 5 and 12, who had expressive language skills at least at the 3-year-old level. It was proposed as a complementary instrument to the Autism Diagnostic Interview (ADI; Le Couteur et al., 1989), an investigator-based parent or caregiver interview that yielded a description of history, as well as current functioning, in areas of development related to autism. Because children under age five constitute the bulk of referrals for a first diagnosis of autism,

there was a need to extend the age and verbal limits of the ADOS to be appropriate for younger and nonverbal children. The PL-ADOS was then created based on the growing interest in using the instruments in clinical settings, which addressed the concerns of parents and fit the abilities of children functioning at infant and toddler levels. As a result, it included more flexible, briefer activities and greater use of play materials for nonverbal young children that served as a downward extension for the ADOS, rather than a replacement. The PL-ADOS was effective in discriminating 2–5-year-old-children with autism from children with non-autism spectrum developmental delays (DiLavore et al., 1995). However, it tended to be underinclusive for children with autism who had some expressive language. Thus, a tool was required to address the needs of children who fell between the PL-ADOS and ADOS in language skills. Furthermore, the ADOS consisted primarily of activities intended for school-age children. Additional or alternative tasks were needed for adolescents and adults. The current edition of the ADOS was designed in response to these factors. The current ADOS differs from the preceding instruments in a way that it is aimed at providing standard contexts for the observation of behavior for a broader developmental and age range of individuals suspected of having autism. Thus, the current ADOS includes additional items developed for verbally fluent, high-functioning adolescents and adults as well as younger and nonverbal children. However, even though this updated version of the ADOS did indeed extend the usefulness of the original ADOS below a language level of 3 years, research has indicated that it remained of limited value for children with nonverbal mental age below 16 months. Thus, a standardized diagnostic measure applicable for infant and young toddlers was also needed for early identification (Gotham et al., 2007). The recent development of the Toddler Module of the ADOS reflects this need for the measure to be applicable to very young children from 12 to 30 months. The original algorithms included two domains, social interaction and communication. Recently, Gotham et al. (2007) revised algorithms for module 1, 2, and 3, and the existing social and communication domains were merged, and the domain of Restricted Repetitive Behaviors (RRB) was newly included. The revised algorithms resulted in increased specificity and sensitivity proving increased diagnostic validity compared to the previous algorithms. Furthermore, even though the inclusion of the RRB domain did not improve predictive value of the ADOS in differentiating individuals with autism from those with pervasive developmental disorder – not otherwise specified (PDD-NOS; also referred as ASD), it

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aided in distinguishing PDD-NOS from non-spectrum cases.

Psychometric Data Psychometric properties for the original ADOS were provided for a carefully selected, blindly interviewed and coded, sample of 223 children and adults with autistic disorder (autism), pervasive developmental disorder – not otherwise specified (PDD-NOS) or non-spectrum (NS) diagnoses. Inter-rater reliability was assessed, with mean multirater kappas of all items for each module ranging from .65 to .78 and intraclass correlations above .82 for all subdomain and domain scores. Test-retest reliability varied by subdomain ranging from .59 to .82. In the original sample, the ADOS algorithms generally achieved 94% correct classification. The exceptions were the ASD versus non-spectrum (NS) module 2 specificity of 87% and module 3 sensitivity of 90%, and the PDD-NOS versus NS Module 2 specificity of 88% and sensitivity of 89% and module 3 sensitivity of 80% (Lord et al., 2001). Psychometric properties for the newly revised algorithms were provided for a sample of 1,139 different participants. The revised algorithms resulted in increased specificity in classifying non-autism ASD in lower functioning populations, evidenced by the 12–31% increase in specificity for children without any words (depending on nonverbal mental age) and the modest gain in specificity for older children who have not progressed beyond phrase speech. During module 1, no words improved in each diagnostic comparison (e.g., from the sensitivity of 19% to 50% for children with nonverbal mental age of 15); the specificity of both module 2 groups improved for non-autism ASD versus NS (e.g., from 77% to 83% for children greater or equal to 5). For autism versus non-spectrum and for ASD versus NS, the new and old algorithms performed approximately equally well in terms of sensitivity. For non-autism ASD versus nonspectrum, sensitivity of the new algorithm was somewhat lower in module 1, no words (as was necessary to raise specificity; e.g., 100% in old algorithm versus 97% in new algorithm for children under nonverbal mental age under 15), but it showed improvement from the old algorithm in the higher-functioning modules 1 (AUT versus NS; from 88% to 97%) and module 2 (ASD versus NS; from 76% to 84%) cells. Inter-rater reliability on the ADOS was monitored through joint administration and scoring by two different examiners for at least 1 in 10 cases and, in some cases, through scoring of videotapes. Agreement remained at greater than 85% (Gotham et al., 2007).

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Psychometric properties for the Toddler Module were provided for a sample of 182 different participants. The sensitivity of each algorithm ranged from 83% to 91% and specificity from 86% to 94%. Inter-rater item and domain reliability was greater than .71, and inter-rater algorithm reliability ranged from .60 to .90. Intraclass correlations ranged from .74 to .99 for all algorithm domains and total scores (Lord et al., in press).

Clinical Uses Use of the ADOS is related to the examiner’s clinical skills and experience with the instrument. Examiners need to be sufficiently familiar with the ratings and the activities so that they can focus their attention on observation of the individual being assessed, rather than on administration of tasks. The examiner should have sufficient practice in observation of ASD symptoms and scoring of the ADOS items, as well as in administering the activities. Examiners are encouraged to attend workshops, use videotapes, or work with colleagues to obtain inter-rater reliability before administering the ADOS for clinical or research purposes (Lord et al., 2001). Examiners should note that the Toddler Module and module 1 are always administered with parents or caregivers in the room, which provides an opportunity to show a parent, examples of behaviors that define ASD, and get information from a parent about the validity of the child’s behaviors during the testing session. Because the ADOS consists of codings made from a single observation, it does not include information about history or functioning in other contexts. This means that the ADOS alone cannot be used to make a complete standard diagnosis, but used in conjunction with other testing. Lord and her colleagues suggested several strategies that clinicians or researchers may take to measure how behaviors of individual may have changed over time by using the ADOS item and domain scores (Lord et al., 2000). If an individual has been administered the same module more than once, raw scores on individual items and on algorithm domains can be compared. If an individual has changed modules, raw scores on items that remain constant across modules (about two thirds of each contiguous module) can be compared, yet comparison of raw domain scores is not meaningful. However, the ADOS calibrated scores recently developed by Gotham et al. (in press) can be used in this case to compare assessments across modules and time. The calibrated scores have more uniform distributions across age- and language-groups compared to raw totals, which make it possible to compare children’s scores longitudinally across

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distinct algorithms. Thus, calibrated scores can be useful in clinical settings to test treatment responsiveness and other clinical outcomes in individuals with ASD. In addition, it was suggested that more detailed coding of communication samples or particular behaviors (e.g., pragmatics, sentence structure, gestures) may also be carried out from videotapes of the ADOS. Other observational coding schemes that address specific aspects of behavior in more detail may also be applied using the ADOS as a way of obtaining a discrete sample of behavior in standard contexts. Often, clinicians carrying out diagnostic assessments may wish to make programming suggestions for parents/caregivers, therapists, or teachers. Many of the activities and codes of the earlier modules have fairly straightforward implications both for how to teach an individual child and for the content of appropriate goals. For example, module 1 provides opportunities for children to make requests in a number of circumstances, including requests for action (i.e., the examiner to blow a balloon), requests for food, requests to continue a social game, and requests for an object or activation of that object (i.e., operating a bubble gun). Noting how children make requests and in what circumstances they are most easily able to communicate their interest or needs, allows the clinician to create goals to teach new request behaviors and to help the children generalize existing behaviors across contexts. Generating programming goals from modules 3 and 4 may be somewhat more complex, because fewer codes describe specific behaviors that may be usefully taught in a direct fashion. Realizing the degree to which adults with autism have limited insight into the nature of social relationships, or having the opportunity to observe adolescents describing the emotions of the main characters in a story, can be helpful in representing the strengths they may have and difficulties they experience in social interaction.

DiLavore, P., Lord, C., & Rutter, M. (1995). Pre-linguistic autism diagnostic observation schedule (PL-ADOS). Journal of Austism and Developmental Disorders, 25, 355–379. Gotham, K., Pickles, A., & Lord, C. (in press). Standardizing ADOS scores for a measure of severity in autism spectrum disorders. Journal of Autism and Developmental Disorders. Gotham, K., Risi, S., Pickles, A., & Lord, C. (2007). The autism diagnostic observation schedule: Revised algorithms for improved diagnostic validity. Journal of Autism and Developmental Disorders, 37, 613–627. Le Couteur, A., Rutter, M., Lord, C., Rios, P., Robertson, S., Holdgrafer, M., et al. (1989). Autism diagnostic interview: A semistructured interview for parents and caregivers of autistic persons. Journal of Autism and Developmental Disorders, 19, 363–387. Lord, C. E., Luyster, R., Gotham, K., & Guthrie, W. J. (in press). Autism diagnostic observation schedule – toddler module. Los Angeles, CA: Western Psychological Services. Lord, C., Risi, S., Lambrecht, L., Cook, E. H., Leventhal, B. L., DiLavore, P., et al. (2000). The autism diagnostic observation schedule–generic: A standard measure of social and communication deficits associated with the spectrum of autism. Journal of Autism and Developmental Disorders, 30(3), 205–223. Lord, C., Rutter, M., DiLavore, P. C., & Risi, S. (2001). Autism diagnostic observation schedule. Los Angeles, CA: Western Psychological Services. Lord, C., Rutter, M., Goode, S., & Heemsbergen, J. (1989). Autism diagnostic observation schedule: A standardized observation of communicative and social behavior. Journal of Autism and Developmental Disorders, 19(2), 185–212. Lord, C., Rutter, M., & Le Couteur, A. L. (1994). Autism diagnostic interview-revised: A revised version of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders. Journal of Autism and Developmental Disorders, 24(5), 659–685. World Health Organization. (1992). The ICD-IO classification of mental and behavioral disorders: Clinical descriptions and diagnostic guidelines. Geneva, Switzerland: Author.

Autism Spectrum Disorder ▶ Pervasive Developmental Disorder NOS

Cross References ▶ Autism Diagnostic Interview-Revised (ADI-R) ▶ Autistic Disorder ▶ Childhood Autism Rating Scales ▶ Modified Checklist for Autism in Toddlers (M-CHAT) also CHAT

References and Readings American Psychiatric Association. (1993). Options book for DSM-IV. Washington, DC: Author.

Autistic Disorder F RED R. VOLKMAR Yale University New Haven, CT, USA

Synonyms Childhood autism; Infantile autism; Kanner’s syndrome

Autistic Disorder

Short Description/Definition Autistic disorder is a neurodevelopmental condition characterized by marked problems in social interaction, communication/play, and a set of unusual behaviors related to difficulties in tolerating change in the environment. The condition is of early onset. In most cases, it appears to be congenital, but perhaps in 20% of cases, a period of normal development is observed. The condition always appears before 3 years of age.

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Rates of autistic disorder are typically three to four times higher in boys than in girls. The nature of this gender difference remains unclear, but speculation has centered on lower thresholds for expression of the condition in boys. An early impression of increased rates in better educated families appears to have been due to referral bias and has not been supported by later work.


Categorization Autism was first described by Leo Kanner in 1943 (Kanner, 1943). Early controversy centered around the idea that autism might be a form of schizophrenia, but several lines of evidence suggest this is not the case. Changes in approaches to the definition of autism have occurred over time. Currently, both the American Psychiatric Association (DSM-IV-TR) (World Health Organization, 2000) and International (ICD-10) (World Health Organization, 1994) categorization systems define autistic disorder in essentially the same way. Autistic disorder is one or a group of conditions referred to as the pervasive developmental disorders (PDD). Other conditions in that class include Aspeger’s disorder (in which marked social deficits are observed but some aspects of language are relatively preserved); Rett’s disorder (a condition largely confined to girls and characterized by marked deterioration in motor, cognitive, and communicative skills); childhood disintegrative disorder (a rare condition where at least 2 years of normal development precedes the emergence of an ‘autistic like’ illness); and PDD-NOS (not otherwise specified) – a term reserved for cases exhibiting some features of autism but not the full syndrome.

Epidemiology A number of epidemiological studies have been undertaken around the world. Their interpretation is complicated by methodological differences including case finding and definitions used. The earliest studies reported rates on the order of 1 in 2,000 children, but more recent work suggests that a figure of 1 in 800–1,000 children is probably more accurate; the broader PDD spectrum is much more ambiguously defined and probably affects as many as 1 in 150 children (Fombonne, 2005). Much debate has centered on whether autism is increasing in frequency, but this issue remains unclear despite better methods of case detection and greater public awareness (Fombonne, 2005).

Issues of diagnosis can be complex in infants as not all required features may be exhibited until around age 3 (Lord & Venter, 1992). After that time, diagnostic agreement increases substantially. By school age, autistic children become more sociable and may make significant academic gains although behavioral difficulties are prominent. During adolescence, some children make substantial gains and others lose skills. There is increased risk for development of epilepsy throughout the developmental period, with peak frequencies of new onset of epilepsy in early childhood and adolescence (Volkmar & Nelson, 1990). The first studies of long-term outcome in children with autism were relatively pessimistic with only 2–3% of cases being able to achieve adult independence and selfsufficiency. Several factors appear to significantly improve prognosis: cases are now detected at early ages (when intervention may be more effective (National Research Council, 2001), and in many countries, educational services are now mandated). It appears that at least 20% or more of children with autism are capable of self-sufficiency in adulthood with at least another 15–20% able to be largely independent (Howlin, 2005). Major predictors of long-term outcome include nonverbal cognitive ability and the capacity to use language to communicate only around age 5. Adaptive abilities (the ability to cope with real world situations) are also important particularly as the person becomes older.

Neuropsychology and Psychology of Autistic Disorder The first attempts to develop psychological models of autism centered around the notion that experiential factors might be involved. As evidence of brain involvement accumulated, theories shifted to focus on neurocognitvie and brain-based mechanisms.

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Several neurocognitive models/theories have been proposed. One approach posits difficulties in executive functioning skills; this model would account for some of the problems with shifting set and perseveration typical of individuals with autism (Ozonoff et al., 2005). However, deficits in these areas are not specific to autism and are not strongly related to the extent of social vulnerability. Another approach has focused on difficulties in what is termed ‘Central Coherence’ or the capacity to integrate information into coherent or meaningful wholes (Happe, 2005). This model centers on problems resulting from difficulties in selective attention and appreciation of social meaning. Another approach posits difficulties in understanding and empathizing with others (Baron-Cohen, 1989). This Theory of Mind hypothesis has been very productive for research. It presumes that difficulties arise as a result of an inability to understanding feelings, intention, and social meaning. Weaknesses of this approach include the fact that more able individuals with autism can solve usual theory of mind type problems; a second problem arises because many of the first features of autism appear before usual theory of mind skills are established in typically developing infants. A relatively newer approach, Enactive Mind, has attempted a synthesis of insights from studies of social cognitive information processing in autism with normal developmental perspectives (Klin, Jones et al., 2003) (Fig. 1). A focus on specific brain mechanisms was suggested by high rates of epilepsy and various neurological signs and symptoms (e.g., persistence of ‘primitive’ reflexes, delayed development of hand dominance, etc.). A range of abnormalities has been found in post-mortem studies. Lesion studies, e.g., of the amygdala or hippocampus, have produced some behaviors in monkeys similar to some of those seen in autism (Bachevalier, 1996). Other studies have focused on abnormalities in the cerebellum and overall brain size which appears to be increased in autism (Courchesne et al., 2004). Other approaches have focused on specific neuropsychological processes. For example, Scultz and colleagues (Schultz et al., 2000) used fMRI techniques to demonstrate that more cognitively able individuals with autism process faces differently than typical controls; essentially they fail to activate the fusiform ‘face area’. This observation is of interest given a large body of experimental work on differences in face processing in autism. Another work, e.g., using eye tracking technology, has revealed marked differences in scanning of the environment during social situations with more able individuals with autism tending to focus on the lower half of the face or objects, thus losing a considerable

Autistic Disorder. Figure 1 Visual focus of an autistic man and a normal comparison subject showing a film clip of a conversation. Typically developing person (top line) goes back and forth between the eyes in viewing a social scene, a high functioning person with autism goes back and forth between the mouths of the speakers. Reprinted with permission from Klin, Jones, Schultz, Volkmar, and Cohen (2002)

amount of social-affective information (Klin, Jones et al., 2002). Beginning with the first twin studies of autism in the late 1970s a considerable body of work has strongly implicated genetic factors in the pathogenesis of autism. There are significantly increased rates of autism in identical twins and a higher risk in siblings both of autism and a range of other developmental problems. It appears that multiple genes are involved and several candidate genes are now being studied (Rutter, 2005).

Evaluation Evaluation of the child with autism typically involves the efforts of members of several different disciplines – psychology, speech-language pathology, medicine, occupational and physical therapy, and special education. Goals for evaluation include clarification of the diagnosis and establishment of patterns of strengths/weakness that have implications for programming. Medical evaluations are indicated to look for conditions like Fragile X syndrome and seizures sometimes associated with autism (Volkmar et al., 1999).

Treatment Over the past decade a considerable body of work on intervention has become available. In its influential 2001

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review a panel from the US National Research Council systematically evaluated ten treatment programs for younger children with autism. Although differing in some respects these programs shared many similarities including intensive individualized programs and structured teaching. Many, although not all, of these programs make extensive use of applied behavior analytic principles to teach basic skills which can then be expanded. Increasing social and communication abilities are important goals. Psychotherapy is not a mainstay of treatment but is sometimes used in older and more able individuals but is to be problem-focused in nature. Drug treatments can be helpful relative to certain symptoms (e.g., agitation, stereotyped mannerisms) but do not address the core social deficit (Scahill & Martin, 2005).

Cross References ▶ Adaptive Behavior ▶ ADI ▶ ADOS ▶ Applied Behavior Analysis ▶ Asperger Disorder ▶ Behavior Modification ▶ Epilepsy ▶ Executive Functioning ▶ Intellectual Disability ▶ TEACCH ▶ Vineland II

References and Readings American Psychiatric Association. (2000). Diagnostic and statistical manual (4th ed., Text Rev.). Washington, DC: APA Press. Bachevalier, J. (1996). Brief report: Medial temporal lobe and autism: A putative animal model in primates. Journal of Autism and Developmental Disorders, 26(2), 217–220. Baron-Cohen, S. (1989). The theory of mind hypothesis of autism: A reply to Boucher [comment]. The British Journal of Disorders of Communication, 24(2), 199–200. Courchesne, E., Redcay, E., Kennedy, D. P. (2004). The autistic brain: Birth through adulthood. Current Opinion in Neurology, 17(4), 489–496. Fombonne, E. (2005). Epidemiological studies of pervasive developmental disorders. In F. R. Volkmar, A. Klin, R. Paul, & D. J. Cohen (Eds.), Handbook of autism and pervasive developmental disorders (Vol. 1, pp. 42–69). Hoboken, NJ: Wiley. Happe, F. (2005). The weak central coherence account of autism. In F. R. Volkmar, A. Klin, R. Paul, & D. J. Cohen (Eds.), Handbook of autism and pervasive developmental disorders (Vol. 1, pp. 640–649). Hoboken, NJ: Wiley. Howlin, P. (2005). Outcomes in autism spectrum disorders. In F. R. Volkmar, A. Klin, R. Paul, & D. J. Cohen (Eds.), Handbook of autism and pervasive developmental disorders (Vol. 2, pp. 201–222). Hoboken, NJ: Wiley.

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Kanner, L. (1943). Autistic disturbances of affective contact. Nervous Child, 2, 217–250. Klin, A., Jones, W., Schultz, R., Volkmar, R., & Cohen, D. (2002). Visual fixation patterns during viewing of naturalistic social situations as predictors of social competence in individuals with autism. Archives of General Psychiatry, 59(9), 809–816. Klin, A., Jones, W., Schultz, R., & Volkmar, F. (2003). The enactive mind, or from actions to cognition: Lessons from autism. Philosophical Transactions of the Royal Society of London, Series B: Biological Sciences, 358(1430), 345–360. Klin, A., Jones, W., Schultz, R., Volkmar, F., & Cohen, D. (2002). Defining and quantifying the social phenotype in autism. American Journal of Psychiatry, 159, 895–908. Lord, C., & Venter, A. (1992). Outcome and follow-up studies of highfunctioning autistic individuals. In E. Schopler & G. B. Mesibov (Eds.), High-functioning individuals with autism current issues in autism (Vol. xviii, pp. 187–199; 316). Plenum: NY. National Research Council. (2001). Educating young children with autism. Washington, DC: National Academy Press. Ozonoff, S., South, M., & Provencal, S. (2005). Executive functions. In F. R. Volkmar, A. Klin, R. Paul, & D. J. Cohen (Eds.), Handbook of autism and pervasive developmental disorders (3rd edn, pp. 606–27). New York: Wiley. Rutter, M. (2005). Genetic influences and autism. In F. R. Volkmar, A. Klin, R. Paul, & D. J. Cohen (Eds.), Handbook of autism and pervasive developmental disorders (Vol. 1, pp. 425–452). Hoboken, NJ: Wiley. Scahill, L., & Martin, A. (2005). Psychopharmacology. In F. R. Volkmar, A. Klin, R. Paul, & D. J. Cohen (Eds.), Handbook of autism and pervasive developmental disorders (Vol. 2, pp. 1102–1122). Hoboken, NJ: Wiley. Schultz, R. T., Gauthier, I., Klin, A., Fulbright, R. K., Anderson, A. W., Volkmar, F. (2000). Abnormal ventral temporal cortical activity during face discrimination among individuals with autism and Asperger syndrome. Archives of General Psychiatry, 57(4), 331–340. Volkmar, F., Cook, E. Jr., Pomeroy, J., Realmuto, G., Tanguay, P. (1999). Summary of the practice parameters for the assessment and treatment of children, adolescents, and adults with autism and other pervasive developmental disorders. Journal of the American Academy of Child and Adolescent Psychiatry, 38(12), 1611–1616. Volkmar, F. R., & Nelson, D. S. (1990). Seizure disorders in autism. Journal of the American Academy of Child and Adolescent Psychiatry, 29(1), 127–129. World Health Organization. (1994). Diagnostic criteria for research. Geneva: World Health Organization.

Autobiographical Memory K ATHERINE T YSON University of Connecticut Storrs, CT, USA

Synonyms Memory; Personal memory; Recollective memory

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Levin, Benton, and Grossman (1982) are sometimes credited with originating the term AM. However, AM research dates back to Francis Galton’s 1879 study of his own recall of events in his personal past. Galton sampled his own episodic memories by finding associations between words and events from his past and dating those events. In 1974, Crovitz and Schiffman modified Galton’s technique to create what became a widely used method for studying AM (see Rubin, 1999). Their revised technique involved asking participants to think of memories associated with words presented to them. In 1983, Nigro and Neisser pioneered studies examining the effect of point of view on AM in their studies of field memories (viewed from the same viewpoint as originally experienced) and observer memories (viewed from an observer’s perspective). They found that older memories were more often viewed from the observer point of view than recent memories (see Rubin, 1999). Current research has spanned a variety of areas, including examining AM’s functional mechanisms, studying psychopathology’s potential effects on AM, and investigating AM’s neuropsychological underpinnings through imaging studies.

problems as they examine lessons from past events and think about how future events and behavior might turn out. As a cultural mechanism, in particular, AM helps not only to shape the individual self, but also to provide the individual with a sense of identity in relation to a wider community (Bluck, 2003). In Euro–American societies, AM appears to emerge around three-and-a-half years of age, as adults begin to share memories with their children. Some cultural differences have emerged in the nature of AM. Asian societies, for example, may report fewer AMs and have fewer and later memories from earlier childhood compared to Euro–Americans. Whereas collective identity may be the focus in these societies, Americans tend to focus on creating a unique identity; AM is essential to this development of a self. In its role of aiding self-development, AM may also drive positive perceptions of self and emotion regulation. Research by Wilson and Ross (Bluck, 2003) has shown that remembering positive events often brings about a positive mood. Furthermore, the third person, observer’s perspective from which people often remember negative life events gives them distance from those events, which may also promote well-being. Sharing of AMs with others may aid emotion regulation. As a facilitator of social interaction AM helps people to reflect on and share recollections of the past with one another. Fivush et al. (Bluck, 2003) have suggested that in mother–child relationships, sharing of AMs may help children to learn how to deal with and express emotions, particularly negative ones. In its directive function, AM guides current behavior and functioning. Both everyday and traumatic memories guide people’s present decisions and actions.

Current Knowledge

Methods in AM Research

Phenomenology

Several methods have been developed for studying AM. Given its complexity and the lack of consensus about how AM works, no single method has emerged as a gold standard; rather, these methods are often used in combination. Open-ended methods include the word-cue method, similar to Galton’s original method. Participants are presented with cue words or other stimuli and asked to think of memories. Typically, after the cued portion of such a study, the researcher will ask the participants to date the memories. This method has tended to provide consistent results, lending support to expected findings: childhood amnesia of early life memories, retention of the

Autobiographical memory (AM) is the memory of events or information involving the self. Researchers generally conceptualize AM as episodic, as opposed to semantic. AMs are temporally defined (e.g., by the date of the remembered event) and involve a sense of ‘‘recollection or reliving’’ of the original event (Greenberg & Rubin, 2003).


Much of the recent research on AM has concentrated on AM’s function. Pillemer (1992) posited that AM has three basic functions: self-related, communicative (social), and directive (planning for present and future events) (Bluck, 2003). In serving the self, AM helps people to develop a sense of coherence and continuity in defining who they are through memories. In its communicative role, AM provides the content of conversations and facilitates building intimacy in social relationships. Sharing of AMs can also inform and teach others about the sharer’s world. As a directive tool, AM can help people solve


memories of the most recent two decades, and for people over age 40, an increase in memories about adolescence and early adulthood (Wenzel & Rubin, 2005). This cueing method is most commonly applied using the Autobiographical Memory Test, first described in 1986 by Williams and Broadbent (Wenzel & Rubin, 2005). A second open-ended method involves simply asking a participant about his or her life. A more structured approach, the involuntary-memory-diary method, asks participants to keep a diary record of involuntary AMs as they occur. Some other methods for delving into AM are more closed-ended. The Autobiographical Memory Interview, developed by Kopelman, Wilson, and Baddeley, was designed to be used with neuropsychological patients (Wenzel & Rubin, 2003). The interview asks for specific kinds of memories from specific time periods. The participant is not provided a choice as to the types of memories he or she will share. In the diary recall method, participants record events and subsequently receive a memory test for these events. Unlike the other methods, the diary recall method can provide some measure of the accuracy of memories. A final method, the questionnaire method, asks participants to report on a series of properties of AMs; this method is often used in conjunction with other approaches. Recently, Sutin and Robins (2007) have developed The Memory Experiences Questionnaire, a measure designed to assess a comprehensive range of dimensions of AM. In developing methods to study such a multifaceted phenomenon as AM, researchers focus on several variables of interest. These variables include: whether the memory is general or specific, latency to retrieve a memory, number of omissions (i.e., when a person does not present a specific personal memory for certain stimuli presented), age of memories, and affective tone. Studies have shown that the last of these variables, affective tone, can potentially differentiate AMs of people with psychopathology from AMs of people without psychopathology (Dalgleish & Brewin, 2007).

AM and Psychopathology In studying the relationship between psychopathology and AM, researchers have found that certain aspects of AM in clinical populations do differ from those of healthy populations. Evidence suggests that AM in patients with suicidal ideation, current or past depression, and trauma history may be overgeneral compared to the specific memories that healthy individuals recall (Hermans, Raes, Philippot, & Kremers, 2006). For example, a depressed patient might report a memory of going to lunch

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with her mother on Tuesdays, as opposed to remembering a specific one of those Tuesday lunches. Research also indicates that trauma and non-trauma memories differ substantially in clinical, but not in healthy populations (Dalgleish & Brewin, 2007). While involuntary memory may be enhanced in some clinical populations, voluntary memory is often fragmented, incomplete, and disorganized, particularly in people with a trauma history. PET and fMRI studies have suggested that the retrieval of trauma memories in PTSD patients is characterized by increased activity of limbic and paralimbic areas, including the amygdala. Additionally, researchers have found deactivation of the medial prefrontal areas and Broca’s area and decreased hippocampal activity in PTSD patients when they processed emotional, rather than neutral material. In looking at depressed individuals, studies have suggested that cues reflecting personal characteristics are more likely to promote a shift to processing of information within the long-term view of the self, increasing the likelihood that self-related semantic information will be provided in response to cues on the Autobiographical Memory Test. In a different, but related line of research, euthymic individuals with a history of depression and patients with a borderline personality disorder retrieved less specific AMs in response to cue words that matched highly endorsed attitudes or schema. This finding suggests that an impaired retrieval of specific memories may be the result of certain cues activating generic, higher-order mental representations in people with both psychopathology histories and present diagnoses (Dalgleish & Brewin, 2007; Hermans et al., 2006). Generally dysfunctional attitudes, whether in an individual exhibiting psychopathology or in a healthy individual, could play a part in an individual’s inability to retrieve specific memories.

Neuropsychology Although researchers have studied the neuropsychology of AM specifically, knowledge about the neuropsychology of memory in general is much more substantial. By looking at the broad memory literature, researchers have been able to make some claims about the neuropsychology of AM and to suggest areas for future study. AM appears to be distributed throughout the brain. Retrieval of personally experienced events has been linked to medial temporal lobe, visual cortex, posterior parietal midline, and prefrontal cortex activity (Daselaar et al., 2008; Greenberg & Rubin, 2003). AM’s emotional and sensory components may involve still other brain areas.

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Daselaar et al. (2008) have worked to map the time course of AM, including emotional and reliving aspects of AM. Through functional magnetic resonance imaging (fMRI), Daselaar et al. examined both initial accessing of memories and subsequent elaboration of the memories. As a person first began to recall a personal memory, hippocampal, retrosplenial, and medial and right prefrontal cortex activation occurred (Daselaar et al., 2008). As participants rated memories, brain areas associated with emotion and sensory function were activated, including the amygdala and hippocampus for initial emotion ratings, and visual cortex and ventromedial and inferior prefrontal cortex for reliving ratings. This line of research underlines AM’s dynamic involvement of multiple brain areas. The visual, auditory, and olfactory systems all appear to be potential parts of the broad AM system. Research has demonstrated that visual imagery is central to AM, especially when considering long-term visual memories (Greenberg & Rubin, 2003). The medial-temporal lobes and the frontal lobes have been implicated in case study research examining visual imagery’s role in AM. Auditory imagery may also be involved in AM, but so far studies have not shown autobiographical amnesias to be related specifically to damage of the auditory cortices. As with visual imagery, further research may provide more information on this aspect of AM. In addition to its sensory dimensions, AM also seems to be closely related to language. However, with only one exception, semantic dementia, AM impairment does not seem to be related to language-related neuropsychological impairments (Greenberg & Rubin, 2003). In semantic dementia, patients display better recall for recent memories than for older ones. A patient with semantic dementia experiences loss of AM, as well as a loss of the ability to maintain semantic memories in storage.

Future Directions Future research on AM should concentrate on defining and specifying AM both behaviorally and through neuropsychological techniques, including functional and structural imaging. Continuing to consider and revise a conceptual model of AM, such as Pillemer’s, is important to providing researchers a better, more definite way to conceptualize AM. Looking at AM cross-culturally may help to determine whether this type of memory plays distinct roles in different cultures and societies. Individual differences in AM are also an important area for study, given researchers’ focus on AM as important to the formation of self. In research on psychopathology,

looking at schema activation and overgeneral AMs may be important to understanding why clinical populations remember AMs differently from nonclinical populations. Taking a closer look at the neuropsychology of AM will be necessary as we find better ways to define AM behaviorally. Correlating behavioral changes with the brain changes we can see through imaging will be a significant area for future study so that we develop a clearer idea of what structures make up the AM circuitry in the brain.

Cross References ▶ Declarative Memory ▶ Episodic Memory ▶ False Memory ▶ Forgetting ▶ Memory Impairment ▶ Remote Memory ▶ Working Memory

References and Readings Bluck, S. (Ed.). (2003). Autobiographical memory: Exploring its functions in everyday life [Special issue]. Memory, 11. Dalgleish, T., & Brewin, C. R. (Eds.). (2007). Autobiographical memory and emotional disorder [Special issue]. Memory, 15(3). Daselaar, S. M., Rice, H. J., Greenberg, D. L., Cabeza, R., LaBar, K. S., & Rubin, D. C. (2008). The spatiotemporal dynamics of autobiographical memory: Neural correlates of recall, emotional intensity, and reliving. Cerebral Cortex, 18, 217–229. Greenberg, D. L., & Rubin, D. C. (2003). The neuropsychology of autobiographical memory [Special issue]. Cortex, 39, 687–728. Hermans, D., Raes, F., Philippot, P., & Kremers, I. (Eds.). (2006). Autobiographical memory specificity and psychopathology. New York: Psychology Press. Levin, H. S., Benton, A. L., & Grossman, R. G. (1982). Neurobehavioral consequences of closed head injury. New York: Oxford University Press. Rubin, D. C. (Ed.). (1999). Remembering our past: Studies in autobiographical memory. New York: Cambridge University Press. Sutin, A. R., & Robins, R. W. (2007). Phenomenology of autobiographical memories: The memory experiences questionnaire. Memory, 15(4), 390–411. Wenzel, A., & Rubin, D. C. (Eds.). (2005). Cognitive methods and their application to clinical research. Washington, DC: American Psychological Association.

Autoimmune Disorders ▶ Myasthenia Gravis

Automated Neuropsychological Assessment Metrics

Autoimmune Thyroiditis ▶ Hypothyroidism

Automated Interpretation ▶ Test Interpretations: Computer Based

Automated Neuropsychological Assessment Metrics S UMMER I BARRA Rehabilitation Hospital of Indiana Indianapolis, IN, USA

Synonyms ANAM

Definition The Automated Neuropsychological Assessment Metrics (ANAM) is a computer-based battery of tests designed to measure an individual’s neurocognitive skills including areas such as sustained attention, processing speed, working memory, and visuospatial ability. It consists of 31 test modules as well as forms for recording demographic information (see Fig. 1; Center for the Study of Human Operator Performance; Reeves, Winter, Bleiberg, & Kane, 2007). The entire battery of tests can be administered or the administrator can elect to customize a subset of the

ANAM executive module 2-Choice reaction time Matching to sample Simple reaction time Manikin Pursuit tracking Complex reaction time Digit reaction time

Participant information Code substitution Mathematical processing Tapping Switching Stroop test 4-Choice reaction time Visual vigilance

Automated Neuropsychological Assessment Metrics. Figure 1

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ANAM tests in order to assess more specific areas of functioning (Vincent et al., 2008). The most recent version of ANAM uses a Windows platform and can be found at the Center for the Study of Human Operator Performance at the University of Oklahoma (Jones, Loe, Krach, Rager, & Jones, 2008). Administration time can range from a few minutes for a single subtest to 90þ minutes for the entire battery (U.S. Army Medical Department, 2009). Scoring for the ANAM is computer generated. Scores for ANAM subtests can be calculated in a variety of ways, including the percentage of correct responses (accuracy score), mean response time for accurate responses (MS), and the ratio of accuracy and speed or number of correct responses per minute (throughput score) (Jones et al., 2008).

Historical Background The ANAM was originally developed by the US Department of Defense as a means of monitoring changes in human performance when encountering environmental challenges, but has now become a common assessment instrument for use in several clinical populations (Kane, Roebuck-Spencer, Short, Kabat, & Wilken, 2007) and research applications (Vincent et al., 2008). The current ANAM is the result of 30þ years of research and is directly linked to older standardized test batteries, including the Unified Tri-Service Cognitive Performance Assessment Battery (Reeves et al., 2007).

Current Knowledge Various combinations of ANAM subtests have been employed to investigate neurocognitive changes and impairment in medical and neurological conditions including acquired brain injury, multiple sclerosis, Parkinson’s disease, systemic lupus erythematosus (Kane et al., 2007),

Sleepiness scale Logical reasoning Memory search Procedural reaction time Tower puzzle Spatial processing – delayed Relative judgment Unstable tracking

Mood scale Matching grids Running memory CPT Spatial processing Standard CPT Grammatical reasoning Symbolic reaction time Dual task

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migraine headache (Roebuck-Spencer, Sun, Cernich, Farmer, & Bleiberg, 2007) and Alzheimer’s dementia (Levinson, Reeves, Watson, & Harrison, 2005). Currently, in an effort to better identify the occurrence of traumatic brain injury (TBI), the ANAM is also being used by the US military to establish a baseline of neurocognitive functioning prior to deployment for all Service members (U.S. Army Medical Department, 2009). Data from over nine studies suggest that varying combinations of ANAM subtest batteries are sensitive and specific in detecting neurocognitive change among individuals with neurological disorders (Kane et al., 2007). Common uses include screening and triage, monitoring of disease progression, and detection of treatment and medication effects (Kane et al., 2007). Other uses of the ANAM include evaluation of cognitive functioning in determining fitness for duty, neurotoxicology, human factors engineering, and various fields of medicine such as aerospace, undersea, military operations, and sports (Reeves et al., 2007).

Advantages The ANAM has been noted as an ideal instrument for assessing change in neurocognition (Roebuck-Spencer et al., 2007). Through randomization of stimuli, practice effects are minimized across numerous testing sessions (Roebuck-Spencer et al., 2007). Further, subtle changes in response time can be more precisely detected as compared to manual calculation of response time (Roebuck-Spencer et al., 2007). Other advantages include time efficiency and cost-effectiveness, both of which are helpful when attempting to triage large numbers of patients (Kane et al., 2007).

for accurate responses (MS), and the ratio of accuracy and speed or number of correct responses per minute (throughput score) (Jones et al., 2008).

Psychometrics The subtests of the ANAM were selected from previously established assessment instruments (e.g., Walter Reed Performance Assessment Battery, the Air Force Criterion Task Set, Navy Performance Evaluation Tests for Environmental Research) with research supporting their respective sensitivity, reliability, and validity (Reeves et al., 2007). As researchers have attempted to gather psychometric data for the current ANAM, many have included only subsets of the subtests in their studies rather than the entire ANAM battery. Various combinations of ANAM subtests have been shown to be both sensitive and specific in detecting cognitive changes in a number of neurological conditions (Kane et al., 2007). Through the process of multiple baseline administrations, test–retest reliabilities across several ANAM subtest throughput scores ranged from 0.50 to 0.91 with 9 of the 10 estimates 20,000 mossy fibers and only one climbing fiber. Parallel fibers are the long axons of granule cells that pass dorsally through the granule and Purkinje cell layers to reach the molecular layer of the cerebellar cortex, where they bifurcate and run parallel to the long axis of the folium. Parallel fibers excite a row of Purkinje cells and in addition to a few basket cells that in turn will inhibit distant Purkinje cells outside the field of excitation. The aminergic fibers originate in the Raphe nuclei and possess serotonergic input, modulating the granule and molecular layers. This category of fibers also includes those originating from the locus ceruleus possessing noradrenergic input and terminating in all three cortical layers.

Cerebral Amyloid Angiopathy

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The cerebellum receives input from all areas of the central and peripheral nervous systems. Continuous flow of information from the spinal centers and cortical areas are integrated in the cerebellar cortex. The cerebellar output then guides the precision of different cerebral functions namely equilibrium, planned voluntary movements, and muscle tone. The cerebellar modulation of motor control is executed through its inhibitory output to the motor cortex and the descending motor tracts. Guided by visual, proprioceptive, and vestibular spinal input, the cerebellum compares the intended force needed to execute a planned voluntary movement with the appropriate muscle power needed to execute it. It then modulates the tone of the agonist and antagonist muscles through inhibitory input to the motor cortex, the pyramidal and extrapyramidal tracts, aiming at the execution in a precise manner. While this voluntary movement is executed, maintenance of equilibrium regardless of movement or body position is achieved through the cerebellar output to the antigravity muscles and the vestibular centers. Eye movements are also maintained during body movement via extensive connections with the occulomotor nuclei in the brain stem. More recent data has shown that the cerebellum is involved in cognitive, behavioral, and emotional processing, including executive control, attention, working memory, learning, language, pain, and emotion (Strick et al., 2009).

Albus, J. S. (1971). A theory of cerebellar function. Mathematical Biosciences, 10, 25–61. Miller, N., & Newman, N. (2005). Walsh and Hoyt’s clinical neuroophthalmology (6th ed.). Philadelphia, Pennsylvania: Lippincott Williams and Wilkins. Ramnani, N. (2006). The primate cortico-cerebellar system: anatomy and function Nature Reviews Neuroscience, 7, 511–522. Ropper, A., & Brown, R. (2005). Adams and Victor’s principles of neurology (11th ed.). USA: McGraw-Hill Inc. Strick, P. L., Dum, R. P., & Fiez, J. A. (2009). Cerebellum and nonmotor function. Annual Review Neuroscience, 32, 413–434.

Lesions Damage to the cerebellar center or to either its inflow or outflow tracts leads to loss of cerebellar fine tuning modulation on different cerebral functions. Vestibulocerebellar lesions can result in disequilibrium, nystagmus, abnormal gait, and recurrent falls. Truncal ataxia and scanning speech are seen with spinocerebellar lesions. Patients with corticocerebellar lesions can display signs of dysmetria, asynergia (decomposition of the voluntary movement), hypotonia, dysdiadochokinesia, and intention tremors.

Cross References ▶ Ataxia ▶ Dysdiadochokinesia ▶ Glutamate ▶ Nystagmus ▶ Proprioception

Cerebral Amyloid Angiopathy E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition Cerebral amyloid angiopathy is a syndrome characterized by recurrent spontaneous lobar cerebral hemorrhages of various sizes and in various locations. Each hemorrhage may be asymptomatic or may cause all of the symptoms of lobar hemorrhages resulting from increased intracranial pressure, including severe headache, seizure, stiff neck, and vomiting; altered consciousness; paralysis or weakness and sensory loss; cognitive and language dysfunction, often leading to dementia after multiple episodes.

Current Knowledge The pathological process that causes this disease is the deposition of a protein, beta-amyloid, in the walls of the arteries of the brain. Interestingly, this protein is identical to the one found in high quantities in the brains of patients with Alzheimer’s disease. The incidence of cerebral amyloid angiopathy is difficult to estimate, but is known to increase with advancing age. It is thought to account for 15% of all intracerebral hemorrhages in patients over 60 years and up to one-half of lobar intracerebral hemorrhages in patients older than 70, totaling about 20 per 100,000 per year in that group. Diagnosis is usually made based on the clinical presentation and imaging of recurrent spontaneous lobar hemorrhages, with no other predisposing problems, usually associated with progressive decline in function, and most often associated with dementia. The recurrences can occur simultaneously,

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clustered in time, or separated by years. The definitive diagnosis, based on pathological findings, is most typically made postmortem. Treatment is usually supportive, consisting of observation, symptom relief, and rehabilitation.

Cross References ▶ Hemorrhagic Stroke ▶ Lobar Hemorrhage

References and Readings Kinnecom, C., Lev, M. H., Wendell, L., et al. (2007). Course of cerebral amyloid angiopathy-related inflammation. Neurology, 68, 1411–1416.

normal, but magnetic resonance imaging of the brain is abnormal in more than 90% of patients. However, the pattern of abnormal findings is not specific. Cerebrospinal fluid analysis usually reveals elevations in total protein level or white blood cell count. Angiography has a low sensitivity and low specificity. Treatment usually includes cyclophosphamide and prednisone.

Cross References ▶ Lupus Cerebritis ▶ Moyamoya Disease ▶ Vasculitis ▶ Vasospasm


Cerebral Angiitis E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Birnbaum, J., & Hellmann, D. B. (2009). Primary angiitis of the central nervous system. Archives of Neurology, 66, 704–709. Calabrese, L. H., Duna, G. F., & Lie, J. T. (1997). Vasculitis in the central nervous system. Arthritis & Rheumatism, 40, 1189–1201. Younger, D. S. (2004). Vasculitis of the nervous system. Current Opinion in Neurology, 17, 317–336.

Synonyms Cerebral vasculitis

Definition Cerebral angiitis or cerebral vasculitis is a relatively rare disease, characterized by inflammation of the blood vessels inside and leading to the brain. It may be caused either by a primary disease of the blood vessel walls producing inflammation or as a secondary phenomenon resulting from a systemic inflammatory disease such as systemic lupus erythematosus or certain infections.

Current Knowledge Angiitis that is confined to the brain is relatively uncommon, and is called primary angiitis of the central nervous system (PACNS), isolated CNS vasculitis, primary CNS vasculitis, or granulomatous angiitis of the nervous system. It usually affects small- and medium-sized cerebral blood vessels, but does not involve blood vessels outside of the CNS. Headache and encephalopathy are the most frequent symptoms. Stroke occurs in about 20% of patients. Blood tests reflecting inflammation are usually

Cerebral Artery ▶ Anterior Cerebral Artery ▶ Internal Carotid Artery ▶ Middle Cerebral Artery ▶ Posterior Cerebral Artery

Cerebral Autoregulation ▶ Cerebral Blood Flow

Cerebral Blood Flow E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Cerebral autoregulation; Cerebral perfusion pressure

Cerebral Cortex

Definition Cerebral blood flow is the amount of blood that goes through the arterial tree in the brain in a given amount of time.

K IMBERLE M. J ACOBS Virginia Commonwealth University Richmond, VA, USA

Synonyms

In adults, cerebral blood flow is typically 750 ml per minute, or about 50 ml per 100 grams of brain tissue per minute. This amount is equivalent to about 15% of the total cardiac output. Cerebral blood flow is highly regulated, through ‘‘autoregulation,’’ in order to meet the metabolic demands of the functioning brain. If it is too high, it can cause elevated intracranial pressure, which will compress and damage brain tissue. If it is too low, it will fail to meet the demands of the brain, resulting in cerebral ischemia if blood flow is less than 20 ml per 100 grams of brain tissue per minute and in cerebral infarction if blood flow is less than 10 ml per 100 grams of brain tissue per minute. Cerebral blood flow is affected by blood viscosity, blood vessel size, intracranial pressure level, and systemic blood pressure.

Cerebrum surface; Cortex

▶ Atherosclerosis ▶ Diffusion-Weighted Imaging ▶ Ischemic Penumbra ▶ Ischemic Stroke ▶ Perfusion-Weighted Imaging ▶ Transcranial Doppler Ultrasonography ▶ Vasospasm

References and Readings Aaslid, R., Lindegaard, K. F., Sorteberg, W., & Nornes, H. (1989). Cerebral autoregulation dynamics in humans. Stroke, 20, 45–52. Kandel, E. R., Schwartz, J. H., & Jessell, T. M. (2000). Principles of neural science (4th ed., p. 1305). New York: McGraw-Hill.

Definition The cerebral cortex is a structure lying on the outer surface of the vertebrate cerebrum that is responsible for consciousness and higher brain functions.

Historical Background The cerebral cortex is a structure lying on the outer surface of the vertebrate cerebrum that is responsible for consciousness and higher brain functions, including sensory perception, voluntary movement, language, reasoning, memory, and planning. Cerebral comes from the Latin word cerebrum, meaning brain. Cortex comes from the Latin word for bark, which is typically an outer layer or covering. In large mammals, this structure is folded forming ridges known as gyri and grooves known as sulci. Gyri and sulci normally form in the same relative locations from one individual to another. This folding increases the cortical surface area while allowing for constraints on skull circumference. Abnormal folding of the cortex is associated with neurological deficits. Absent or reduced folding in humans is known as lissencephaly (smooth brain) and is associated with mental retardation and epilepsy (Leventer, Mills, & Dobyns, 2000). The abnormality of small regions of increased folding is known as polymicrogyria (many small ridges), and can also be associated with developmental delay and epilepsy (Piao et al., 2005).

Current Knowledge

Cerebral Cavernous Malformation (CCM) ▶ Angioma, Cavernous Angioma

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Structurally, the cerebral cortex can be divided into four lobes named after the overlying cranial bones: frontal, parietal, temporal, and occipital. Prominent sulci define the borders of these lobes. Primary functions of the lobes

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can be ascribed as follows: occipital: vision; parietal: sense of touch (somatosensation) and body position; frontal: planning of action and control of movement; and temporal: hearing, visual identification, and memory. These lobes are present in each of the two hemispheres (left and right). The cerebral cortex can also be divided on the basis of phylogeny into archicortex, paleocortex, and neocortex (oldest to newest). Archicortex consists of the hippocampus, which is associated with the acquisition of memories. Paleocortex is primarily associated with the function of olfaction. Only mammals have neocortex. In humans, the majority of the cerebral cortex is made up of neocortex. In addition, as a percentage of total brain tissue, humans have more neocortex than other species (see for example, human relative to rat, Swanson, 1995). This is unique to the neocortex, since evolution has not increased the size of other brain structures, such as the cerebellum (Clark, Mitra, & Wang, 2001). This increase in proportion is due to increased surface area, and not to a change in the thickness of the cortex, which is from 1–3 mm thick in all species. Cortex is made up of gray matter, where cell bodies predominate, and white matter that consists primarily of myelinated axons. All cortex is laminated, but the gray matter of neocortex has six layers (Fig. 1), while that of the older archi-and paleocortices has three layers. Cortical layers are differentiated based on their cellular components. The basic components and lamination of cortex are consistent across phylogeny. Within neocortex, the layers are given names that represent the predominant neuronal cell type. The outermost layer (identified by Roman numeral I), contains mainly dendrites and axons and is called the molecular layer. Layer II is called the external granule layer and consists of small, spherical cells. Layer III primarily contains small to medium pyramidal neurons and is called the external pyramidal layer. Layer IV contains spiny stellate neurons and is called the internal granule cell layer. Layer V contains large pyramidal neurons and is called the internal pyramidal layer. Layer VI has a variety of morphological cell types and is therefore called the polymorphic cell layer. These layers have differential functions that are consistent across different neocortical areas (Fig. 2). Layer I is a modulatory region and receives input from higher order cortical regions. Layers II and III perform intracortical processing, receiving input from the deeper layer IV, as well as from adjacent layers II and III and from the homologous cortical region in the opposite hemisphere. Layer IV is the major input layer and receives specific thalamocortical afferents in sensory areas of cortex. Layer V is the major cortical output, for

Cerebral Cortex. Figure 1 Cortical Lamination. Cresyl Violet stained coronal section from rat, showing neocortex above hippocampus (archicortex). Neocortical layers are indicated by Roman numerals. Layer V is commonly divided into subparts with layer Va containing callosal projection neurons, and Vb containing the largest pyramidal neurons that project to spinal cord and other subcortical locations. Within this section of neocortex, slight differences in cytoarchitectonics can be seen between the somatosensory cortex (Brodmann’s area 3) to the right and motor cortex (Brodmann’s area 4) to the left. Most obvious is the lack of a clear layer IV within motor cortex. WM neocortical white matter; s.o. stratum oriens; s.p. stratum pyramidale; s.r. stratum radiatum, all of the CA1 region of the hippocampus

instance sending the result of motor cortical processing to the motor neurons of the spinal cord. Layer VI provides a return feedback to the thalamus. Different functional regions of cortex are considered to have primary, secondary, and association components. In sensory cortex, the primary cortical area is the region that first receives information about that sense from the periphery (traveling by way of the thalamus). The secondary cortical area is considered ‘‘higher order’’ because the input it receives is the result of cortical processing from the primary cortical area. Association cortex receives input from several different cortical regions. The general function of the layers is maintained across cortical regions; however, there are slight changes in cell size and packing density (cytoarchitecture) from one

Cerebral Cortex

I II Callosal

III

IV

Callosal

V

VI Modulatory DA NE 5-HT ACh

Specific Thalamic Afferents

To Spinal Cord Superior Colliculus

Nonspecific Thalamic Afferents

Cerebral Cortex. Figure 2 Diagram of typical excitatory neuronal cell types and connections of neocortex. Layer I, the modulatory cell layer, contains the tufts of deeper lying pyramidal neurons, nonspecific thalamic afferents, and input from brainstem modulatory transmitter systems. Layer II contains primarily small granule cells. Layer III has small pyramidal neurons that perform intracortical processing, sending their axons horizontally within layer III. Layer IV contains spiny stellate neurons that send their output to layer III. Specific thalamic afferents make excitatory synapses within layer IV on the spiny stellate cells as well as on the apical dendrites of deeper lying pyramidal neurons. These specific thalamic afferents have a smaller projection to layer VI, near the border with layer V. Layer V is the major output layer for the cortex, and contains medium and large pyramidal neurons. Layer VI contains neurons with a variety of shapes. Note that inhibitory cells that make up 20% of the neurons in the neocortex are not shown here. Modulatory: Modulatory neurotransmitters; DA: Dopamine; NE: Norepinephrine; 5-HT: Serotonin; and ACh: Acetylcholine

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cortical area to another. These cytoarchitectonic differences were used by Korbinian Brodmann in 1909 to draw boundaries presumed to identify functionally different cortical areas. Perpendicular to the plane of the cortical layers are functional modules called cortical columns. The idea that a column of cortex represents a fundamental processing unit was brought to light by Vernon Mountcastle of Johns Hopkins University (Mountcastle, 1957). Within a column, neurons tend to have similar response properties. For instance, within somatosensory cortex, the neurons within a column have similar receptive fields (area of the receptive surface that causes the neuron to fire action potentials). Neurons of different columns have nonoverlapping receptive fields. The result of evolution then has been to add cortical columns or additional processing units. Head trauma, stroke, and tumor may all result in lesions of the cerebral cortex. The function lost will be dependent on the location of the lesion. For example, lesion of Brodmann’s area 17 will result in loss of vision, while lesion of Brodmann’s area 3 will result in some loss of somatosensation including touch and pain discrimination. Damage to Brodmann’s area 4 will result in loss of motor function. Lesions of the frontal cortex can cause severe personality changes. Memory loss is typically associated with cortical lesions. The inability to speak occurs after destruction of Broca’s area in the ventral portion of the frontal lobe, typically in the left hemisphere (Brodmann’s areas 44 and 45). Incoherent speech or ‘‘word salad’’ results from the destruction of Wernicke’s area in the upper portion of the temporal lobe (part of Brodmann’s area 22).

Cross References ▶ Association Cortex ▶ Auditory Cortex ▶ Brodmann’s Areas of the Cortex ▶ Heteromodal Cortex ▶ Homotypic Cortex ▶ Neocortex ▶ Prefrontal Cortex ▶ Primary Cortex ▶ Secondary Cortex ▶ Somatosensory Cortex ▶ Striate Cortex ▶ Tertiary Cortex ▶ Unimodal Cortex ▶ Visual Cortex

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Cerebral Dominance

References and Readings Clark, D. A., Mitra, P. P., & Wang, S. S. (2001). Scalable architecture in mammalian brains. Nature, 411, 189–193. Jones, E. G., & Peters, A. (Eds.). (1990). Cerebral cortex: Volume 8A: Comparative structure and evolution of the cerebral cortex, part I. New York: Springer. Jones, E. G., & Peters, A. (Eds.). (1990). Cerebral cortex: Volume 8B: Comparative structure and evolution of the cerebral cortex, part II. New York: Springer. Leventer, R. J., Mills, P. L., & Dobyns, W. B. (2000). X-linked malformations of cortical development. American Journal of Medical Genetics, 97, 213–220. Mountcastle, V. B. (1957). Modality and topographic properties of single neurons in cat’s somatic sensory cortex. Journal of Neurophysiology, 20, 408–434. Piao, X., Chang, B. S., Bodell, A., Woods, K., Benzeev, B., Topcu, M., et al. (2005). Genotype-Phenotype analysis of human frontoparietal polymicrogyria syndromes. Annals of Neurology, 58(5), 680–687. Rasmussen, W., & Penfield, T. (1957). The cerebral cortex of man: A clinical study of localization of function. New York: Macmillan Company. Swanson, L. W. (1995). Mapping the human brain: Past, present, and future. Trends in Neuroscience, 418, 471–474.

Cerebral Dominance

Vasogenic edema involves a disruption in the blood– brain barrier with leakage of fluid from the intravascular space. In cytotoxic edema, the blood–brain barrier is intact, and there is an increase in the intracellular fluid compartment.

Cross References ▶ Cerebral Perfusion Pressure ▶ Intracranial Pressure

References and Readings Beaumont, A., Marmarou, A., & Ward, J. D. (2001). Intracranial Hypertension Mechanisms and Management. In D. G. McClone (Ed.), Pediatric Neurosurgery (pp. 619–633). Philadelphia: W. B. Saunders. Greenberg, M. S. (1997). Handbook of Neurosurgery. Lakeland, FL: Greenberg Graphics. Rosenblum, W. I. (2007). Cytotoxic edema: monitoring its magnitude and contribution to brain swelling. J Neuropathol Exp Neurol, 66(9), 771–778.

Cerebral Embolism

▶ Hemispheric Specialization E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Cerebral Edema G ARY T YE 1, J OHN B ROWN 2 1 Virginia Commonwealth University Richmond, VA, USA 2 Medical College of Georgia Augusta, GA, USA

Synonyms Cytotoxic edema; Vasogenic edema

Synonyms Embolic stroke

Definition A cerebral embolism is a blood clot (thrombus) that starts from the heart or blood vessel where the clot originates and stops in an artery that leads to or rests within the brain. The result is occlusion of the vessel and obstruction of the flow of oxygen and blood to the brain tissue supplied by that artery.

Definition Current Knowledge Cerebral edema is an increase in the water content of the brain that leads to brain swelling. It may be divided into two broad categories: vasogenic and cytotoxic.

Cerebral embolisms cause about 15–20% of all strokes and about one-quarter of all ischemic strokes. It occurs most

Cerebral Palsy

frequently in patients who have known heart disease, including atrial fibrillation and other arrhythmias, valve disease, ‘‘mural thrombus’’ (a blood clot sitting in the left ventricle of the heart), or other conditions. It causes symptoms similar to those of thrombotic strokes, but the presentations of embolic strokes tend to be more abrupt and dramatic. These can include sudden onset of hemiplegia, sensory loss, facial weakness, cognitive deficits, or speech disturbance. Seizures or headaches are relatively common in embolic strokes, and both of these symptoms are relatively rare in ischemic strokes. In addition, there may be multiple diffuse simultaneous neurological findings, which may result from multiple simultaneous emboli, known as ‘‘showers of emboli.’’ Usually, management requires addressing the cardiac condition and preventing subsequent emboli by using anticoagulants, in addition to the treatment of the cerebral infarction and its neurological consequences.

▶ Frontal Lobe ▶ Occipital Lobe ▶ Parietal Lobe ▶ Temporal Lobe

Cerebral Hemorrhage ▶ Hemorrhagic Stroke

Cerebral Infarction ▶ Ischemic Stroke

Cerebral Leukencephalopathy ▶ Periventricular Leukomalacia

Cerebral Malformation References and Readings DiTullio, M. R., & Homma, S. (2002). Mechanisms of cardioembolic stroke. Current Cardiology Reports, 4, 141–148. Fuster, V., Rydeń, L. E., Cannom, D. S., Crijns, H. J., Curtis, A. B., & Ellenbogen, K. A. (2006). ACC/AHA/ESC 2006 Guidelines for the Management of Patients with Atrial Fibrillation: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Revise the 2001 Guidelines for the Management of Patients With Atrial Fibrillation): Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation, 114, e257–e354. Hart, R. G., Pearce, L. A., & Aguilar, M. I. (2007). Meta-analysis: antithrombotic therapy to prevent stroke in patients who have nonvalvular atrial fibrillation. Annals of Internal Medicine, 146, 857–867.

Cerebral Evoked Potentials ▶ Event-Related Potentials

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Cerebral Hemisphere

Cross References ▶ Anticoagulation ▶ Echocardiogram ▶ Infarction ▶ Ischemic Stroke ▶ Myocardial Infarction ▶ Thrombosis ▶ Warfarin

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▶ Arteriovenous Malformation (AVM)

Cerebral Microvasculature ▶ Blood-Brain Barrier

Cerebral Palsy K ATHLEEN K EELY M C C ANN D EIDRICK University of Missouri-Columbia Columbia, MO, USA

Synonyms Static encephalopathy

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Cerebral Palsy

Short Description or Definition As defined by the International Workshop on the Definition and Classification of Cerebral Palsy, Cerebral Palsy (CP) is: " a group of permanent disorders of the development of

movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems (Rosenbaum et al., 2007).

Categorization Classification schemes are critical to providers attempting to describe the disorder, measure change in function, and provide prognostic information (Rosenbaum et al., 2007). Historically, classification has focused on two features: (a) tone and (b) body part involvement (Menkes & Sarnat, 2000). Table 1 for a summary. Spastic forms of CP are more common, with spastic diplegia being the most common (Menkes & Sarnat, 2000). However, there is disagreement regarding classification schemes, largely due to poor reliability (Accardo & Hoon, 2008). Recent attempts to improve classification schemes include: (a) development of standardized examination procedures and diagnostic algorithms and (b) use of the Gross Motor Functional Classification System (GMFCS), which focuses on functional mobility without identifying abnormalities of tone or affected limbs (Rosenbaum et al., 2007).

Epidemiology Prevalence rates vary from 1.3 to 3/1,000 and are stable across country of origin (Clark & Hankins, 2003). Preterm

birth raises the risk of CP, with a recent study reporting a diagnosis of cerebral palsy among 20% of children born at or before 27 weeks of gestation versus diagnosis of 5–6% of children born between 28 and 31 weeks (Ancel et al., 2006). Reviewers note that overall rates of CP are climbing, while rates of CP among full-term infants have remained stable (1.1/1,000). This suggests that increases are largely due to greater survival of preterm infants (Mukherjee & Gaebler-Spira, 2007).

Natural History, Prognostic Factors, and Outcomes Etiology varies by birth status (preterm or full-term) and type of CP (Menkes & Sarnat, 2000). For preterm infants, the most common causes of CP are intraventricular hemorrhage and/or periventricular leukomalacia. Risk factors for CP in full term infants include prenatal infections, anoxic or ischemic injuries, genetic syndromes, brain malformations, or stroke. Atypical CP with athetoid movements is typically caused by basal ganglia damage secondary to hyperbilirubinema (Mukherjee & Gaebler-Spira, 2007). A variety of symptoms are associated with CP, which vary by severity and type of CP (Menkes & Sarnat, 2000; Mukherjee & Gaebler-Spira, 2007). See Table 2 for a summary.

Neuropsychology and Psychology of Cerebral Palsy As many as 30–50% of children with CP may have a diagnosis of mental retardation, with increased incidence for children with quadriplegia, more severe motor deficits, full-term birth, and/or a coexisting seizure disorder (Menkes & Sarnat, 2000; Mukherjee & Gaebler-Spira, 2007). Estimates of intellectual functioning can be difficult to obtain, as apraxic speech, visual difficulties, and

Cerebral Palsy. Table 1 Traditional categorization of cerebral palsy Type

Description

Spastic cerebral palsy

Increased muscle tone with movement

Spastic quadriplegia

Spasticity of upper and lower limbs

Spastic diplegia

Greater involvement of the legs than the arms

Spastic hemiparesis

Greater involvement of one side of the body (more often the right); greater impairment of the arm than the leg

Extrapyramidal cerebral palsy

Involuntary and abnormal muscle movements: dystonia (fluctuating tone and abnormal body postures) and/or athetoid movements (writhing movements in the extremities)

Hypotonic cerebral palsy

Persistent, low muscle tone

Mixed and atypical cerebral palsy

Mixture of spasticity and extrapyramidal symptoms

Cerebral Palsy

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Cerebral Palsy. Table 2 Symptoms associated with CP Domain

Symptoms

Secondary muscular and orthopedic symptoms

Delayed development of adaptive motor skills

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Gait abnormalities Oral-motor difficulties and problems with speaking and drooling Contracture (shortening of the muscle) Bone deformities (e.g., hip subluxation/dislocation) Scoliosis Osteoporosis Reduced limb growth

Neurologic symptoms

Seizure disorder

Sensory

Visual problems Homonomous hemianopsia (in spastic hemiplegia) Strabismus Nystagmus Visual sequela related to prematurity Tactile/perceptual deficits Stereognosis Poor two-point discrimination (in spastic hemiplegia) Neglect of affected side of the body (in spastic hemiplegia) Hearing loss

Feeding/gastrointestinal

Dysphagia and aspiration Malnutrition requiring gastrostomy Gastroesophageal reflux Constipation Incontinence or difficulty voiding

Dental

Malocclusion

Pain and fatigue

Pain associated with primary (e.g., spasticity and contractures) and secondary (e.g., constipation) disease processes

Poor tooth enamel

Pain related to medical procedures Fatigue secondary to poor mobility

fine motor deficits can limit participation in traditional tests (Fennell & Dikel, 2001). Little is known about cognitive functioning among children with CP who do not have a diagnosis of mental retardation, reports of deficits in learning (specifically arithmetic), visual-perceptual processing (particularly in spastic diplegia), working memory, and executive functioning are emerging (Blondis, 2004; Jenks et al., 2007). The empirical literature regarding mental health in children with CP is sparse. Overall, studies of children with physical disabilities suggest that difficulties with adjustment are atypical, with the exception of poor selfconcept in areas directly impacted by the physical

disability (e.g., attractiveness, social interaction, athletics, academics) (Miyahara & Cratty, 2004). Adolescent girls may be at particularly increased risk for low self-concept (Shields, Murdoch, Loy, Dodd, & Taylor, 2006). Studies of Quality of Life (QOL) suggest lower QOL for children with CP, particularly in areas associated with CP and its sequella (e.g., academics, social interaction) and most notably for children with quadriplegia/more severe CP (Livingston, Rosenbaum, Russell, & Palisano, 2007). Transition to adulthood is understudied, but issues regarding reduced involvement in age-appropriate social activities and roles, employment, and leisure activities are of concern (Liptak, 2008).

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Cerebral Palsy

Evaluation Given the broad variability in etiology and the sparse knowledge base regarding neuropsychological functioning in CP, evaluations should be tailored to the child and situation. Prior to the evaluation, medical evaluations clarifying the child’s vision and hearing will be critical. Assessment measures should be chosen carefully to avoid underestimates of function due to motor or verbal output problems (e.g., use of nonverbal tests of intelligence that minimize requirements for motor output) (Fennell & Dikel, 2001). In children with marked communication deficits, parents and educators may be interested in possible methods for alternative communication (e.g., picture exchange systems, complex computerized devices), a question which can be addressed through a multi-disciplinary evaluation including a psychologist, speech–language pathologist, and occupational therapist. For children who are less impaired, evaluations should include a broad overview of neuropsychological functions with attention to executive functioning, visual-perceptual processing, and academics. Assessments should include formal or informal evaluation of pain, fatigue, and psychological and behavioral functioning.

Treatment Multidisciplinary treatment is considered the standard of care for children with CP (Braddom, 2007). Children are involved in medical and therapeutic treatments designed to decrease spasticity and increase function (e.g., botox injections, baclofen pump, surgical interventions, splinting and casting, physical and occupational therapies, early intervention services). Although promising, treatment efficacy is unknown for many of these interventions (Tupper, 2007). Interventions developed from a rehabilitation psychology perspective are integral to the treatment plan, including behaviorally-based treatments to increase function and assessment and intervention of pain and fatigue. Constraint-induced movement therapy (CIMT) is a promising approach that attempts to overcome learned non-use of the affected limb and to promote use of the affected limb through brain re-organization (Hoare, Wasiak, Imms, & Carey, 2007). Patients are restrained from using the unaffected limb through a sling or glove for 2–3 weeks and behavioral techniques (e.g., shaping, massed practice, scaffolding, and positive reinforcement) are used to encourage use of the affected limb. Also promising are behavioral treatments that address problems with drooling (Van der Burg, Didden,

Jongerius, & Rotteveel, 2007). Pain can be life-limiting and may be underrecognized due to communication deficits and atypical pain responses in children with CP (Houlihan, O’Donnell, Conaway, & Stevenson, 2004). Adaptation of common self-report pain assessment scales and/or use of measures that have been developed for children who have communication problems may help the team to adequately evaluate and treat pain (Hadden & von Baeyer, 2005). Fatigue due to increased energy expenditure during daily tasks can also impact function and should be addressed (Berrin et al., 2007).

Cross References ▶ Assistive Technology ▶ Augmentative and Alternative Communication ▶ Constraint Induced Therapy ▶ Encephalopathy ▶ Hemiparesis ▶ Hemiplegia ▶ Interdisciplinary Team Rehabilitation ▶ Periventricular Leukomalacia ▶ Prematurity and Low Birthweight

References and Readings Accardo, P. J., & Hoon, A. H., Jr. (2008). The challenge of cerebral palsy classification: The ELGAN study. Journal of Pediatrics, 153, 451–452. Ancel, P.-Y., Livinec, F., Larroque, B., Marret, S., Arnaud, C., Pierrat, V., et al. (2006). Cerebral palsy among very preterm children in relation to gestational age and neonatal ultrasound abnormalities: The EPIPAGE cohort study. Pediatrics, 117, 828–835. Berrin, S. J., Malcarne, V. L., Varni, J. W., Burwinkle, T. M., Sherman, S. A., Artavia, K., et al. (2007). Pain, fatigue, and school functioning in children with cerebral palsy: A path-analytic model. Journal of Pediatric Psychology, 32(3), 330–337. Blondis, T. A. (2004). Neurodevelopmental motor disorders: Cerebral palsy and neuromuscular diseases. In D. Dewey & D. E. Tupper (Eds.), Developmental motor disorders: A neuropsychological approach (pp. 113–136). New York: Guilford Press. Braddom, R. L. (Ed.). (2007). Physical medicine and rehabilitation (3rd ed.). Philadelphia: Elsevier, Inc. Clark, S. L., & Hankins, G. D. V. (2003). Temporal and demographic trends in cerebral palsy – fact and fiction. American Journal of Obstetrics and Gynecology, 188, 628–633. Fennell, E. B., & Dikel, T. N. (2001). Cognitive and neuropsychological functioning in children with cerebral palsy. Journal of Child Neurology, 16, 58–63. Hadden, K. L., & von Baeyer, C. L. (2005). Global and specific behavioral measures of pain in children With Cerebral Palsy. Clinical Journal of Pain, 21(2), 140–146. Hoare, B. J., Wasiak, J., Imms, C., & Carey, L. (2007). Constraint-induced movement therapy in the treatment of the upper limb in children

Cerebral Ventricles with hemiplegic cerebral palsy. Cochrane Database of Systematic Reviews (2), CD004149. Houlihan, C. M., O’Donnell, M., Conaway, M., & Stevenson, R. D. (2004). Bodily pain and health-related quality of life in children with cerebral palsy. Developmental Medicine and Child Neurology, 46(5), 305–310. Jenks, K. M., de Moor, J., van Lieshout, E. C. D. M., Maathuis, K. G. B., Keus, I., & Gorter, J. W. (2007). The effect of cerebral palsy on arithmetic accuracy is mediated by working memory, intelligence, early numeracy, and instruction time. Developmental Neuropsychology, 32, 861–879. Liptak, G. S. (2008). Health and well being of adults with cerebral palsy. Current Opinion in Neurology, 21, 136–142. Livingston, M. H., Rosenbaum, P. L., Russell, D. J., & Palisano, R. J. (2007). Quality of life among adolescents with cerebral palsy: What does the literature tell us? Developmental Medicine and Child Neurology, 49(3), 225–231. Menkes, J. H., & Sarnat, H. B. (2000). Perinatal asphyxia and trauma. In J. H. Menkes & H. B. Sarnat (Eds.), Child neurology (6th ed., pp. 401–466). Philadelphia: Lippincott Williams & Wilkins. Miyahara, M., & Cratty, B. J. (2004). Psychosocial functions of children and adolescents with movement disorders. In D. Dewey & D. E. Tupper (Eds.), Developmental motor disorders: A neuropsychological perspective (pp. 427–442). New York: Guilford. Mukherjee, S., & Gaebler-Spira, D. J. (2007). Cerebral palsy. In R. L. Braddom (Ed.), Physical medicine and rehabilitation (3rd ed., pp. 1243–1267). Philadelphia: Elsevier. Rosenbaum, P., Paneth, N., Leviton, A., Goldstein, M., Bax, M., Damiano, D., et al. (2007). A report: The definition and classification of cerebral palsy April 2006. Developmental Medicine and Child Neurology – Supplementum, 109, 8–14. Shields, N., Murdoch, A., Loy, Y., Dodd, K. J., & Taylor, N. F. (2006). A systematic review of the self-concept of children with cerebral palsy compared with children without disability. Developmental Medicine and Child Neurology, 48, 151–157. Tupper, D. E. (2007). Management of children with disorders of motor control. In S. J. Hunter & J. Donders (Eds.), Pediatric neuropsychological intervention: A critical review of science and practice (pp. 338–365). Cambridge, UK: Cambridge University Press. Van der Burg, J. J. W., Didden, R., Jongerius, P. H., & Rotteveel, J. J. (2007). Behavioral treatment of drooling: A methodological critique of the literature with clinical guidelines and suggestions for future research. Behavior Modification, 31, 573–594.

Cross References ▶ Cerebral Blood Flow ▶ Intracranial Pressure

References and Readings Diringer, M. N., & Axelrod, Y. (2007). Hemodynamic manipulation in the neuro-intensive care unit: cerebral perfusion pressure therapy in head injury and hemodynamic augmentation for cerebral vasospasm. Current Opinion in Critical Care, 13(2), 156–162. Wright, W. L. (2007). Multimodal monitoring in the ICU: when could it be useful? Journal of the Neurological Sciences, 261(1–2), 10–15.

Cerebral Seizures ▶ Epilepsy

Cerebral Thrombophlebitis ▶ Central Venous Thrombosis

Cerebral Vasculitis

Cerebral Perfusion Pressure

Definition Cerebral perfusion pressure (CPP) is the net pressure of flow of blood to the brain, which is the difference between

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the mean arterial pressure (MAP) and the intracranial pressure (ICP).

▶ Cerebral Angiitis

G ARY T YE 1, J OHN B ROWN 2 1 Virginia Commonwealth University Richmond, VA, USA 2 Medical College of Georgia Augusta, GA, USA

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Cerebral Venous Thrombosis ▶ Central Venous Thrombosis

Cerebral Ventricles ▶ Ventricles

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Cerebrovascular Accident (CVA)

Cerebrovascular Accident (CVA) ▶ Stroke

Cerebrovascular Dementia

injury is irreversible, this causes a completed ‘‘stroke,’’ which is defined as the neurological manifestations of disease of blood vessels of the brain. Other forms of cerebrovascular disease also can occur. Inflammation of blood vessel walls (‘‘vasculitis’’), bleeding into the cerebral vessel walls (‘‘dissection’’), and hemorrhage, or extravasation of blood outside of the vessels themselves and into the brain tissue, also can cause brain damage. These can give rise to strokes.

▶ Multi-infarct Dementia

Cross References

Cerebrovascular Disease E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition Cerebrovascular disease refers to the group of conditions characterized by disease of the blood vessels that supply blood to the brain. It can occur in the blood vessels that lead to the brain or in the blood vessels inside the brain. While it usually presents with symptoms of a stroke (the group of clinical manifestations of cerebrovascular disease), it can be asymptomatic, in which case it is usually detected either by physical examination or selected imaging techniques. The term ‘‘cerebrovascular accident’’ (or CVA) is incorrect and should be avoided, as there is nothing accidental about a stroke. The term ‘‘cerebrovascular disease’’ is a more general term than is ‘‘stroke,’’ because ‘‘cerebrovascular disease’’ includes asymptomatic or subclinical disease, in addition to the clinically manifest strokes. Most cerebrovascular disease is obstructive in nature, caused by atherosclerotic plaques that line the blood vessel walls and block the blood flow. If the blockage is only partial, and is not severe enough to impair brain function, then the disease remains asymptomatic. However, if the obstruction is severe enough to reduce blood supply to the extent that brain injury occurs, then symptoms suggesting a stroke ensue. If these symptoms are temporary and completely reversed, the phenomenon is known as a ‘‘transient ischemic attack.’’ If the brain

▶ Atherosclerosis ▶ Cerebral Angiitis ▶ Cerebral Embolism ▶ Dissection ▶ Hemorrhagic Stroke ▶ Infarction ▶ Intracerebral Hemorrhage ▶ Ischemic Stroke ▶ Stroke ▶ Subarachnoid Hemorrhage ▶ Thrombosis ▶ Transcranial Doppler Ultrasonography ▶ Vascular Dementia ▶ Vascular Malformation ▶ Vasculitis

References and Readings Wolf, P. A., & Grotta, J. C. (2000). Cerebrovascular disease. Circulation. 102, IV-75.

Cerebrum Surface ▶ Cerebral Cortex

CES-D ▶ Center for Epidemiological Studies–Depression

Chapple v. Ganger

CFL Test ▶ Controlled Oral Word Association Test ▶ F-A-S Test ▶ Verbal Fluency

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involved, especially neuropsychologists. These types of challenges question the scientific basis of one or more of the expert’s methods, measures, or conclusions. Challenges to specific methods are brought under Frye v. United States (1923) and Daubert v. Merrell Dow (1993) rulings.

Cross References

CGY

▶ Daubert v. Merrell Dow

▶ Centigray


Chandler Exterminators v. Morris (1992) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition The testimony of neuropsychologists is commonly challenged by defense attorneys in cases relating to inferences of subtle brain changes associated with neurotoxic brain injury. In the case of Schudel v. General Electric (1995), plaintiffs accepted neuropsychological evidence for brain damage caused by organic solvents and polychlorinated biphenyls (PCBs). However, the federal appeals court from the Ninth Circuit ruled that neuropsychological testimony is limited only to damages and cannot determine physical causation. The court opined that determination of causation is relegated to medical doctors (MDs) or left to the discretion of the jury to make connections between neurocognitive deficits presented and exposure to toxins. In the case of Chandler Exterminators v. Morris (1992), the Georgia Supreme Court ruled in favor of the trial court’s decision to prohibit neuropsychological testimony that proposed a link between neurotoxicants and impaired neuropsychological test scores. In response to this case, Georgia legislature wrote a new law permitting neuropsychologists to provide testimony related to causation of brain injuries in Georgia. Challenges to specific neuropsychological tests and test batteries occur with some degree of frequency, and they should be taken seriously by all parties

Chandler Exterminators Inc. v. Morris, 200 Ga. App. 816 (1992). Schudel v. General Electric, 120 F. 3d 991 (1995).

Chapple v. Ganger R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Historical Background In the cause of Daubert v. Merrell Dow (1993), it was ruled that for scientific testimony to be admissible, it has to be: (a) scientifically valid, and (b) relevant to the case at hand. The court provided a list of guidelines intended to aid in the determination of scientific validity (e.g., peer reviewed, fasifiability, acceptable error rate, etc.). The Daubert ruling along with subsequent related rulings (e.g., General Electric v. Joiner, 1997, Kumho Tire v. Carmichael, 1999), generated significant debate among psychologists and neuropsychologists, and many other disciplines. Specifically, Reed (1996) viewed the Daubert ruling to necessitate the utilization of commercially available fixed batteries only, such as the Halsteid–Reitan Battery. However, most neuropsychologists employ a flexible battery approach; thus, contradicting Reed’s assertions implying that most neuropsychologists would not be suited for involvement in forensic work. In support of his conclusion, Reed referenced the case of Chapple v. Ganger (1998), a brain injury claim. Review of the judge’s written decision in Chapple v. Ganger outlined that all

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neuropsychological testimony (even partial HRB protocols from two other neuropsychologists) was admitted into evidence and the fact that the judge had placed more emphasis on testimony from a fixed battery advocate was completely unrelated to the determination of the test battery. Indeed, there was no Daubert challenge to a flexible test battery approach. In this particular case, only the testimony of a vocational specialist who conducted no testing and provided no evidence in the form of a peerreviewed study to support his claims, was subject to a Daubert hearing from the defense.

References and Readings Chapple v. Ganger, 851 F. Supp. 1481, E.D. of Washington (1994). Daubert v. Merrell Dow Pharmaceuticals, 509 U.S. 579 (1993). General Electric co. v. Joiner, 522 U.S. 136 (1997). Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney-neuropsychologists relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Kumho Tire Co. v. Carmichael, 526 U.S. 137 (1999). Reed, J. E. (1996). Fixed versus flexible neuropsychological test batteries under the Daubert standard for the admissibility of scientific evidence. Behavioral Sciences and the Law, 14, 315–322.

Charles Bonnet Syndrome M ELISSA B UTTARO Brown University Providence, RI, USA

Synonyms/Associated Terms Bonnet syndrome

Categorization The images associated with CBS are often rich in detail, and their clarity frequently contrasts sharply with sufferers’ blurred perception of real objects (Menon, Rahman, Menon, & Dutton, 2003). They are sometimes referred to as ‘‘pseudo-hallucinations’’ to indicate that the person experiencing them is aware that the images are not real. Hallucinations may vary greatly in terms of color, clarity, movement, and bizarreness (Plummer, Kleinitz, Vroomen, & Watts, 2007). Nevertheless, common themes and figures have been described, including humans and animals, extended landscapes, and ornate structures (Plummer et al., 2007). Ffytche and Howard (1999) classified their patients’ hallucinations into eight categories (Table 1). While the clinical validity of this classification system has not yet been established, it does share some similarities with known functional and anatomical networks within the visual association cortex (Plummer et al., 2007).

Epidemiology 1. Prevalence: Once considered rare, CBS is becoming increasingly more common (Rovner, 2006). The larger number of reported CBS cases may be related to the growing population of older adults and the more common occurrence of visual disorders such as agerelated macular degeneration, glaucoma, and cataracts (Rovner, 2006). It has also been argued that past prevalence estimates of CBS were spuriously low due to a

Charles Bonnet Syndrome. Table 1 Hallucination Category Description Tessellopsia

Regular, overlapping patterns


Hyperchromatopsia

Hyperintense, vivid, brilliant colors

Charles Bonnet syndrome (CBS) is characterized by the following features (Eperjesi & Akbarali, 2004):

Prosopometamorphopsia Facial distortions

1. The presence of well-formed, complex, repetitive or persistent visual hallucinations 2. Full or partial retention of insight into the unreal nature of the hallucinations 3. Absence of hallucinations in other sensory modalities (e.g., auditory, olfactory) 4. Absence of delusions

Dendropsia

Branching forms

Perseveration

True percept that persists after the individual looks away

Illusory visual spread

Spread of a non-hallucinated pattern

Polyopia

Multiple copies of a percept

Micropsia/macropsia

Miniaturized/‘‘larger than life’’ images


general lack of awareness of the syndrome in the medical community, as well as patients’ reluctance to disclose their hallucinatory symptoms for fear of being labeled psychotic or demented (Plummer et al., 2007). One estimate suggests that the prevalence of complex visual hallucinations in patients with visual impairment is between 11% and 15% (Menon et al., 2003). 2. Age of onset: CBS may occur at any age, but it is more common in the elderly. Average age of onset tends to be in the 70s and 80s (Plummer et al., 2007). The increased prevalence of CBS in older adults is likely related to the greater incidence of sudden visual loss and/or isolation in this age group (Menon et al., 2003).

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Charles Bonnet Syndrome. Table 2 Factors Favoring the Recurrence of Hallucinations Dimly lit conditions States of drowsiness Physical and social isolation

Charles Bonnet Syndrome. Table 3 Factors that May Help Relieve Hallucinations Rapid blinking Sustained eye closure

Natural History, Prognostic Factors, Outcomes Historical background: Charles Bonnet syndrome is named after the eminent Swiss philosopher and naturalist who first described this phenomenon in 1760 (Hedges, 2007). In a book entitled Essai Analytique sur les Faculties de L’Ame [Analytical Essays Concerning the Faculties of the Mind], Charles Bonnet described how his cognitively intact, 89-year-old grandfather with failing eyesight began to experience well-formed visual hallucinations, which he was aware were not actually physically present. Interestingly, Charles Bonnet began to have similar experiences later in his own life. At the age of 22, he began to experience severe eye pain and progressively worsening loss of vision that made it difficult for him to use a microscope, and he ultimately turned to more abstract philosophical pursuits and theoretical questions in biology. In his retirement, he experienced formed visual hallucinations associated with many of the common attributes of the syndrome that shares his name, including blindness, intact cognition, and occurrence in quiet and reflective settings. Another native of Geneva, George de Morsier, proposed in 1967 that visual hallucinations in older men without mental deficiency be designated the syndrome of Charles Bonnet. Current thinking/prognostic factors: The question of whether visual impairment is necessary for the development of CBS has been the matter of debate. Some argue that CBS is almost invariably associated with impaired vision, and it may occur whenever sensory input to the brain is decreased sufficiently to allow release phenomena. Other researchers report that visual dysfunction, though common, is not mandatory for diagnosis, and note that CBS has been found in individuals with intact vision (Terao & Collinson, 2000).

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Diversionary activities Limiting exposure to dim lighting Walking away Looking at or approaching images

It has been argued that CBS is more commonly associated with higher degrees of visual impairment, and with bilateral as opposed to unilateral ocular pathology (Menon et al., 2003). It has also been suggested that it is not the specific lesion site or the severity of impairment, but rather the rate of development of the visual impairment that best predicts CBS (Plummer et al., 2007). That is, hallucinations may be more common in the context of sudden or unexpected decrease in visual function (Menon et al., 2003). Outcomes: The course of CBS can be unpredictable. While the onset is generally sudden, it may also be gradual (Menon et al., 2003). Hallucinations can last from seconds to hours, or even days. Clustering of episodes across days or weeks is not unusual. Three patterns of the syndrome have been described (Menon et al., 2003). The episodic pattern, characterized by hallucinations that happen over a period of days to months and then permanently cease, is reportedly the least common. In the periodic pattern, phases of hallucinatory activity alternate with phases of remission. The continuous pattern, as its name suggests, is characterized by unremitting hallucinations (i.e., no hallucination-free intervals) (Menon et al., 2003). Overall, the duration of CBS can extend from days to years, and spontaneous recurrence after a symptom-free interval is possible. Interestingly, some sufferers have reported permanent remissions of CBS in conjunction with ongoing visual decline (Plummer et al., 2007).

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Neuropsychology and Psychology of Charles Bonnet Syndrome The exact pathophysiology of CBS is unclear. Although Charles Bonnet suggested that the primary pathology was restricted to the eye, subsequent research has suggested that complex hallucinations can occur in the context of visual impairment secondary to pathology anywhere along the central visual pathway, from the orbit to the occipital cortex (Menon et al., 2003). A leading hypothesis is that complex visual hallucinations result from deafferentation of the visual association cortex following lesions among the central visual pathway. Age-related macular degeneration is a commonly cited cause of CBS, but other ocular pathologies include glaucoma, central retinal artery occlusion, and optic neuritis (Plummer et al., 2007). Extraocular and central visual axis pathologies associated with CBS include lesions of the pituitary and optic chiasm, meningioma, and occipital stroke.

Evaluation The evaluation of CBS should begin with a clinical interview, which should be approached carefully in light of sufferers’ frequent reluctance to disclose hallucinatory experiences. The clinician should assess the nature of the hallucinations, the modalities in which they occur, the presence of delusions, and the patient’s insight. It is important to note that insight into the illusory nature of the hallucinations may not occur immediately; in fact, there may be a period of initial deception, especially if the perceived images are not uncommon and fit realistically into the patient’s surroundings (Menon et al., 2003). Referrals to an ophthalmologist, low-vision specialist, and neuropsychiatrist may be helpful. A key issue for clinical neuropsychologists is the differential diagnosis of CBS from other causes of visual hallucinations. Conditions belonging in the differential include migraine, occipital seizures, peduncular hallucinosis (usually from rostral brainstem infarct), drug-induced states, psychiatric disease, delirium, and dementia. In particular, CBS may occur in the early stages of dementia with Lewy bodies (DLB), and as cognitive function declines, insight about the unreal nature of the hallucinations vanishes (Terao & Collinson, 2000). Some have suggested the term Charles Bonnet Plus or CBS plus to describe visual hallucinations that occur in the presence of a neuropsychiatric disorder (Eperjesi & Akbarali, 2004; Menon et al., 2003).

Treatment Treatment for CBS may not always be necessary, since visual hallucinations often resolve spontaneously, either in response to improvement or further deterioration of visual function (Menon et al., 2003). In addition, many patients are not distressed by their hallucinations and may even enjoy them. However, if hallucinations are frequent or distressing, the following treatment options are available: 1. Optimizing visual acuity: Patients should initially be referred to a low-vision specialist, who may be able to reduce or alleviate hallucinations by optimizing visual function (e.g., via prescription eyeglasses or visual aids). If appropriate, the patient might be considered for surgery (e.g., cataract surgery or neurosurgical procedures). Several reports indicate that improvement of visual function, either spontaneously or by intervention, can effectively decrease or even eliminate hallucinations (Menon et al., 2003). 2. Supportive treatment: A key component in the management of CBS is supportive. Patients may derive comfort from sympathetic explanations that their hallucinations are not uncommon, are not necessarily a marker of psychiatric disease, and may represent a release phenomenon in the context of visual impairment. Using the analogy of ‘‘phantom visions,’’ similar to a phantom limb syndrome, may be helpful (Rovner, 2006). 3. Psychotherapeutic strategies: Psychotherapeutic techniques used for phantom limb pain, including distraction, hypnosis, relaxation training, and cognitive restructuring can help reduce the unpleasant effects of intrusive and upsetting visual hallucinations (Menon et al., 2003). Support or psychoeducational groups are useful settings in which sufferers can meet, obtain reassurance, and be given advice about specific techniques for reducing hallucinations (Eperjesi & Akbarali, 2004). 4. Behavioral/environmental modifications: Approaches such as rapid eye blinking, sustained eye closure, minimizing fatigue and stress, and engaging in distracting activities (e.g., listening to the radio and attending to household chores) may help reduce hallucinations. Limiting exposure to dim lighting (e.g., by increasing lighting in the home in the evening) and taking steps to reduce glare may also be helpful. Looking directly at the images, attempting to approach them, and conversing with them have also been reported to stop hallucinations (Menon et al., 2003).

CHART Short Form

Since solitude and loneliness, particularly during the evening hours, tend to heighten hallucinations, strengthening social networks and increasing the amount of time spent interacting with others may be useful (Plummer et al., 2007). 5. Pharmacological interventions: Referral to a specialist for pharmacological therapy may be helpful. A few case studies have reported efficacy of pharmacological agents in alleviating symptoms, such as sodium valproate, olanzapine, and carbamazepine (Plummer et al., 2007). Use of other medications (e.g., risperidone, gabapentin, and diazepam) has also been described (Eperjesi & Akbarali, 2004). 6. Follow-up: Since some cases of CBS do go on to develop dementia, it is recommended that clinicians follow patients with complex visual hallucinations carefully over time (Menon et al., 2003). Though most patients experience no practical problems associated with CBS, continuous visual hallucinations can interfere with navigation and driving, and patients’ ability to perform daily activities safely should be monitored over time (Menon et al., 2003).

Cross References ▶ Dementia with Lewy Bodies ▶ Macropsia ▶ Micropsia ▶ Visual Hallucinations

References and Readings Eperjesi, F., & Akbarali, N. (2004). Rehabilitation in Charles Bonnet syndrome: A review of treatment options. Clinical and Experimental Optometry, 87(3), 149–152. Ffytche, D. H., & Howard, R. J. (1999). The perceptual consequences of visual loss: ‘Positive’ pathologies of vision. Brain, 122(Pt 7), 1247–1260. Hedges, T. R. (2007). Charles Bonnet, his life, and his syndrome. Survey of Ophthalmology, 52(1), 111–114. Menon, G. J., Rahman, I., Menon, S. J., & Dutton, G. N. (2003). Complex visual hallucinations in the visually impaired: The Charles Bonnet Syndrome. Survey of Ophthalmology, 48(1), 58–72. Plummer, C., Kleinitz, A., Vroomen, P., & Watts, R. (2007). Of Roman chariots and goats in overcoats: The syndrome of Charles Bonnet. Journal of Clinical Neuroscience, 14(8), 709–714. Rovner, B. W. (2006). The Charles Bonnet syndrome: A review of recent research. Current Opinion in Ophthalmology, 17(3), 275–277. Terao, T., & Collinson, S. (2000). Charles Bonnet syndrome and dementia. Lancet, 355(9221), 2168.

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CHART ▶ Craig Handicap Assessment and Reporting Technique

C CHART Short Form G ALE G. W HITENECK Craig Hospital Englewood, CO, USA

Synonyms CHART-SF; Craig handicap assessment and reporting technique (CHART) short form

Definition The Craig Handicap Assessment and Reporting Technique Short Form (CHART-SF) is a 19-item measure of handicap or level of societal participation. Released in 1998, the CHART-SF is the short version of the 32-item CHART instrument designed to provide a simple, objective measure of the degree to which impairments and disabilities result in handicaps (societal participation limitations) for adolescents and adults (15 years and older) in the years after initial rehabilitation. Like its precursor, the CHART-SF includes six subscales (physical independence, cognitive independence, mobility, occupation, social integration, and economic independence), which closely reflect the disablement model developed by the World Health Organization, published in 1980 and revised in 2001. Each subscale contains from 2 to 5 questions, which together quantify the extent to which individuals fulfill various social roles. CHART-SF focuses on objective, observable criteria that are easily quantifiable and unlikely to be open to subjective interpretation. Each of the domains or subscales of the instrument have a maximum score of 100 points, which is considered the level of performance typical of the average non-disabled person. High subscale scores indicate less handicap, or higher social and community participation. Although originally developed for use with persons with spinal cord injury, the CHART and the CHART-SF have proven to be appropriate measures of societal participation that can be used with individuals having a

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range of physical or cognitive impairments. The CHARTSF was designed to be administered by interview, either in person or by telephone and takes approximately 5–7 min to administer. There is no set time period for administering the CHART-SF; however, it is recommended that multiple measurements be taken over the course of a person’s lifetime to assess the changes with adaptation to the disability and to gain insight into changes in participation, which may occur over time. The 19-item CHART-SF with subscales closely approximating the subscale scores for the CHART long form is recommended for those populations for whom time is at a minimum.

CHEIs ▶ Cholinesterase Inhibitors

Chelation B RUCE J. D IAMOND, A MANDA FAULHABER William Paterson University Wayne, NJ, USA

Synonyms Current Knowledge In an effort to reduce the number of items in the original CHART, a short form was developed. A multidimensional analysis was performed which showed that fewer variables were needed to obtain CHART scores. Regression analyses were performed on each subscale with the dependent measure being the scale score and the variables contributing to the subscale acting as the predictor variables. All CHART subscale scores could be reduced by fewer questions to reach 90% explained variance except Economic Self-Sufficiency, which using the main variables could only explain 45%.

Cross References ▶ Craig Handicap Assessment and Reporting Technique

EDTA therapy

Short Description or Definition Chelation therapy has been used in allopathic and in complementary and alternative (CAM) forms of medicine. Claims that chelation therapy with ethylene diamine tetraacetic acid (EDTA) is an effective technique for controlling and treating cardiovascular disease are not supported by systematic reviews of the literature (Ernst, Pittler, Stevinson, White & Eisenber, 2001; Seely, Wu & Mills, 2005). However, Chelation therapy does appear to be highly effective and is the treatment of choice in treating heavy metal poisoning (Ernst et al., 2001). Recent research also suggests that Chelation therapy may have applications in the treatment of malaria (Mabeza, Lovevsky, Gordeuk & Weiss, 1999). The therapy involves the intravenous administration of EDTA, which binds ions in the blood, and is often used in combination with vitamins, trace elements, and iron supplements.

References and Readings Whiteneck, G., Brooks, C. A., Charlifue, S., Gerhart, K. A., Mellick, D., Overholser, D., et al. (1998). Guide for use of the CHART: Craig handicap assessment and reporting technique. www.craighospital. org/Research/CHART December 28, 2009.

CHART-SF ▶ CHART Short Form ▶ Craig Handicap Assessment and Reporting Technique

Categorization While Chelation therapy has been used for treating lead poisoning and vascular occlusive disease, oral chelation therapy with the a-ketohydroxypyridine chelator 1,2-dimethyl3-hydroxypyrid-4-one (L1, INN/BAN: deferiprone) has also been used in iron- and aluminum-overloaded patients. A number of iron(III) chelators have also shown antimalarial activity in vitro with the proposed mechanism of activity involving the withholding of iron from metabolic pathways of the intra-erythrocytic parasite and the formation of toxic complexes with iron.

Chemical AIDS

History Chelation therapy began in the early 1950s and was primarily used to treat metal poisoning of the blood. However, both allopathic and CAM practitioners have claimed that the technique can be used to reverse the arteriosclerotic disease process (e.g., peripheral arterial occlusive disease (PAOD)).

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levels (Kontoghiorghes, 1995). Toxic side effects include six cases of reversible agranulocytosis, 0–30% incidence of transient musculoskeletal and joint pains, 0–6% of gastric intolerance, and 0–2% zinc deficiency. In the treatment of malaria, iron chelation therapy with desferrioxamine, which is the only compound of this nature that is available for use in humans, has shown clinical activity in both uncomplicated and severe malaria in humans (Mabeza et al., 1999).

Evaluation Studies examining the efficacy of Chelation therapy have shown mixed results. In one authoritative systematic review, it was stated that proponents of chelation therapy adhere to pathophysiological models of arteriosclerosis, which are inconsistent with current knowledge and practice (Ernst et al., 2001). In a systematic review of chelation therapy in the treatment of malaria, it was concluded that when used via oral administration, its efficacy and low cost make it more accessible than desferrioxamine for the majority of patients needing iron chelation (Mabeza et al., 1999).

Adverse Side Effects In one systematic review, adverse effects were characterized as rare but cases of hypocalcemia and a single case of increased creatinine was noted in a patient on the EDTA intervention (Seely, Wu & Mills, 2005). Moreover, it was emphasized that if the treatment is used in lieu of proven therapies, indirect harm to the patient could result. In another systematic review, it was reported that EDTA treatment may be associated with life-threatening adverse effects, such as hypocalcemia and severe kidney damage, in addition to prolonged bleeding and respiratory distress (Ernst et al., 2001).

Treatment and Mechanisms Typically, Chelation therapy is administered in multiple sessions with each treatment lasting for over an hour. The putative mechanism underlying chelation therapy is the binding of ions in the blood by EDTA. The therapy is, generally, considered to be effective in heavy metal poisoning. In CAM applications, it has been used as an alternative to bypass surgery, based on the idea that chelation therapy removes harmful plaque build-up in the arteries (unblocking arteriosclerotic arteries) and helps prevent strokes. The mechanism is believed to involve the extraction of calcium out of arteriosclerotic plaques via the chelating mechanism. In a systematic review of randomized, placebo-controlled, double-blind trials, it was concluded that Chelation therapy for PAOD is not superior to placebo, that it is associated with considerable risks and costs, and that it should now be considered obsolete (Ernst et al., 2001). However, it should also be noted that using oral chelation therapy, in doses ranging from 55 to 100 mg kg–1 of L1 (a-ketohydroxypyridine chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1, INN/ BAN: deferiprone)), a majority of iron-loaded patients showed urinary iron excretion levels greater than those accumulating from transfusions (15–35 mg d–1) and also reductions in serum ferritin and liver iron to near normal

Cross References ▶ Lead Exposure

References and Readings Ernst, E., Pittler, M. H., Stevinson, C., White, A., & Eisenber, D. (Eds.) (2001). The desktop guide to complementary and alternative medicine: An evidence based approach. Amsterdam: Elsevier Health Sciences. Kontoghiorghes, G. J. (1995). New concepts of iron and aluminum chelation therapy with oral L1 (deferiprone) and other chelators: A review. Analyst, 120, 845–851. Mabeza, G. F., Loyevsky, M., Gordeuk, V. R., Weiss, G. (1999). Iron chelation therapy for Malaria: A review. Pharmacology & Therapeutics, 81, 53–75. Seely, D. M., Wu, P., & Mills, E. J. (2005). EDTA Chelation therapy for cardiovascular disease: A systematic review. BMC Cardiovascular Disorders, 5, 32–38.

Chemical AIDS ▶ Multiple Chemical Sensitivity

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Chemical Hypersensitivity Syndrome

Chemical Hypersensitivity Syndrome ▶ Multiple Chemical Sensitivity

Chemotherapy B RAM G OLDSTEIN Hoag Hospital Cancer Center Newport Beach, CA, USA

Synonyms Systemic therapy

Definition Chemotherapy is a systemic treatment for cancer, comprising cytoxic agents that target cancer cells. Normally, cells develop and die in an orderly and determined fashion. However, when cancer manifests itself, the cells intractably divide and proliferate. Chemotherapy drugs target these cancer cells by destroying them before they continually multiply and divide. In particular, chemotherapy interferes with or impairs the targeted molecules (e.g., DNA, proteins) during designated cellular stages, such as synthesis or mitosis. The majority of chemotherapy drugs damage or interfere with the replication of DNA and/or RNA, and are used to treat several malignancies, particularly brain tumors, lymphomas, and leukemias. Some of the chemotherapies include: alkylating agents (such as cisplatin, carboplatin, cyclophosphamide, and temozolomide) to treat brain tumors, lymphomas, and leukemias; nitrosoureas (e.g., carmustine and lomustine) are indicated for the treatment of brain tumors and lymphomas; antimetabolites (such as methotrexate) to treat leukemias; anthracycline and related drugs (e.g., doxorubicin), which have toxic effects on the heart; topoisomerase inhibitors (such as topotecan, irinotecan, and etoposide); mitotic inhibitors (e.g., vinblastine and vincristine), which can cause peripheral nerve damage; and corticosteroid hormones, which can be used to kill or slow the growth of cancer cells. There are other chemotherapies that are excluded from these categories, namely L-asparaginase, -hydroxyurea, and

-thalidomide. The specific manner in which chemotherapy achieves the intended effect is contingent upon the particular drug(s) employed. However, cytotoxicity usually occurs when the cell attempts to divide and before the repair occurs (Chabner & Longo, 2004). The probability of the intended effect on the targeted molecules reflects the appropriate concentration of drugs, amount or dose and timing of drug administration. Drug absorption, distribution, and penetration are also significant factors inherent in the efficacy of chemotherapy. The precise drug dosage can be complicated because if the amount is too low, it may be ineffective against the tumor. Conversely, if the dosage is too high, patients may suffer from excessive toxicity. Since chemotherapy damages healthy cells during the therapeutic process, the treatment is associated with several harmful side effects, such as myelosuppression. For example, bone marrow, which produces white blood cells, red blood cells, and blood platelets, can be damaged during chemotherapy treatment. In particular, white blood cells and platelets frequently drop transiently after chemotherapy, so that patients are at increased risk for infection and bleeding during and post chemotherapy. Many chemotherapy patients also suffer from nausea and vomiting because the drugs irritate the stomach lining and bowel. Certain chemotherapy drugs also cause alopecia, or hair loss. This condition results from the chemotherapy agent adversely affecting the growth of hair cells, causing them to become brittle and eventually break. Several chemotherapies also result in anorexia, severe loss of appetite, and significant weight loss. Fatigue, diarrhea, and constipation are also very common side effects from cancer and chemotherapy. Additionally, specific chemotherapy agents can cause stomatitis, a condition that results in sores manifesting inside the mouth or throat. Chemotherapy is most often given intravenously, whereby a thin needle is inserted into a patient’s vein on the hand or lower arm. Intravenous chemotherapy can also be delivered through catheters, ports, and pumps. The treatment is frequently given in cycles (i.e., specified treatment periods) that reflect alternating rest periods. This is necessary because patients require substantial relief to permit the body to recuperate, build healthy new cells, and restore strength. Treatment regimens may be given daily, weekly, or monthly and are based upon a drug’s efficacy or toxicity. Chemotherapy is usually administered before (neo-adjuvant) surgery or post (adjuvant) surgery. Neoadjuvant chemotherapy is intended to decrease the primary tumor’s size. This potentially mitigates the harmful effects of surgery or radiotherapy and enhances the efficacy of chemotherapy.

Chemotherapy

Adjuvant chemotherapy may also reduce the probability of tumor resistance to drug therapy in the event of disease recurrence (Chabner & Longo, 2004). Furthermore, adjuvant chemotherapy is effective at destroying residual cancer cells that have spread to distal parts of the body (i.e., metastasis), particularly because rapidly proliferating lesions are very amenable to treatment. Palliative chemotherapy is indicated when the curative potential is very low and the primary goals are to decrease the patient’s tumor burden and prolong life expectancy.

Current Knowledge Recent neuropsychological research has indicated that chemotherapy can also adversely impact cognitive functioning, both short-term and delayed. In particular, neuropsychological research studies have provided evidence discussing the impact of chemotherapy on attention, memory, and concentration (Armstrong, Gyato, Awadalla, Lustig, & Tochner, 2004). Consequently, many of these chemotherapy-induced cognitive impairments can significantly impair patients’ daily activities, such as working, being involved in a committed relationship, and attending to personal responsibilities. Research has suggested that many of these impairments are temporary but some may be more long-term, or even permanent. The cognitive effects are not uniform, and the severity appears to reflect a higher concentration and/or larger dose of chemotherapy. Most of the different types of chemotherapy drugs damage or interfere with the replication of DNA and/or RNA, and are used to treat several malignancies, particularly brain tumors, lymphomas, and leukemias. Some of the chemotherapies include: alkylating agents (such as cisplatin, carboplatin, cyclophosphamide, and temozolomide) to treat brain tumors, lymphomas, and leukemias; nitrosoureas (e.g., carmustine and lomustine) to treat brain tumors and lymphomas; antimetabolites to treat leukemias (such as methotrexate); anthracycline and related drugs (e.g., doxorubicin), which have toxic effects on the heart; topoisomerase inhibitors (such as topotecan, irinotecan, and etoposide); mitotic inhibitors (e.g., vinblastine and vincristine), which can cause peripheral nerve damage; and corticosteroid hormones, which can be used to kill or slow the growth of cancer cells. There are other chemotherapies that are excluded from these categories, such as L-asparaginase, -hydroxyurea, and -thalidomide. The specific manner in which chemotherapy achieves the intended effect is contingent upon the particular drug(s) employed. However, cytotoxicity usually occurs when

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the cell attempts to divide and before repair occurs (Chabner & Longo, 2004). The probability of the intended effect on the targeted molecules reflects the appropriate concentration of drugs, amount or dose and timing of drug administration. Drug absorption, distribution, and penetration are also significant factors inherent in the efficacy of chemotherapy. The precise drug dosage can be complicated because if the amount is too low, it may be ineffective against the tumor. Conversely, if the dosage is too high, patients may suffer from excessive toxicity. Since chemotherapy damages healthy cells during the therapeutic process, the treatment is associated with several harmful side effects, such as myelosuppression. For example, bone marrow, which produces white blood cells, red blood cells, and blood platelets, can be damaged during chemotherapy treatment. In particular, white blood cells and platelets frequently drop transiently after chemotherapy, so that patients are at increased risk for infection and bleeding during and after chemotherapy. Many chemotherapy patients also suffer from nausea and vomiting because the drugs irritate the stomach lining and bowel. Certain chemotherapy drugs also cause alopecia or hair loss. This condition results from the chemotherapy agent adversely affecting the growth of hair cells, causing them to become brittle and eventually break. Several chemotherapies also result in anorexia, severe loss of appetite, and significant weight loss. Fatigue, diarrhea, and constipation are also very common side effects from cancer and chemotherapy. Additionally, specific chemotherapy agents can cause stomatitis, a condition that results in sores manifesting inside the mouth or throat. Chemotherapy is most often given intravenously, involving a thin needle, which is inserted into a patient’s vein on the hand or lower arm. Intravenous chemotherapy can also be delivered through catheters, ports, and pumps. The treatment is frequently given in cycles that reflect specified treatment periods which are alternated with rest periods. This is necessary because patients require substantial relief to permit the body to recuperate, build healthy new cells, and restore strength. Treatment regimens may be given daily, weekly, or monthly and are based upon a drug’s efficacy or toxicity. Chemotherapy is usually administered before (neo-adjuvant) surgery or post (adjuvant) surgery. Neo-adjuvant chemotherapy is intended to decrease the primary tumor’s size. This potentially mitigates the harmful effects of surgery or radiotherapy and enhances the efficacy of chemotherapy. Adjuvant chemotherapy is employed when there is scant evidence of residual disease, but there is an increased

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risk of cancer recurrence. Adjuvant chemotherapy may also reduce the probability of tumor resistance to drug therapy in the event of disease recurrence (Chabner & Longo, 2004). Furthermore, adjuvant chemotherapy is effective at destroying residual cancer cells that have spread to distal parts of the body (i.e., metastasis), particularly since rapidly proliferating lesions are very amenable to treatment. Palliative chemotherapy is indicated when the curative potential is very low and the primary goals are to decrease the patient’s tumor burden and prolong life expectancy.

Cross References ▶ Systemic Therapy

References and Readings Armstrong, C. L., Gyato, K., Awadalla, A. W., Lustig, R., & Tochner, Z. A. (2004). A critical review of the clinical effects of therapeutic irradiation damage to the brain: the roots of controversy. Neuropsychology Review, 14, 65–86. Chabner, B. A., & Longo, D. L. (2004). Cancer chemotherapy and biotherapy: Principles and practice. Hagerstown, MD: Lippincott, Williams & Wilkins.

Chief Sensory Nucleus of V

cuneatus and gracilis in the medulla, which mediate similar input from the trunk and extremities. It gives rise to trigeminothalamic fibers, which terminate in the ventral posterior medial nucleus of the thalamus.

Current Knowledge Because of its size and density, it is rare for brainstem lesions to be isolated to a single nucleus or pathway. Theoretically, lesions which involve this nucleus might most readily be distinguished on a routine neurological exam by changes (asymmetries) in two-point discrimination on the ipsilateral face. In practice, however, such lesions are likely to involve other brainstem nuclei and pathways, including the adjacent motor nucleus of V, spinal trigeminal tract and/or nucleus, spinal thalamic tracts, lateral portions of the medial lemniscus, and middle cerebellar peduncles resulting in ipsilateral muscle weakness of the jaw muscles, ipsilateral changes in pain and temperature in the face and diminished or abolished corneal reflex, contralateral loss of pain and temperature in the extremities, diminished or loss of proprioception, stereognosis, and vibration in the contralateral extremities (leg > arm), and ipsilateral cerebellar signs.

References and Readings Gilman, S., & Newman, S. W. (2003). Manter and Gatz’s essentials of clinical neuroanatomy and neurophysiology. Philadelphia: F.A. Davis. Wilson-Pauwek, L., Akesson, E. J., Stewart, P. A., & Spacey, S. D. (2002). Cranial nerves in health and disease. Hamilton, ON: B.C. Decker.


Child Behavior Checklist Synonyms Principal sensory nucleus of the trigeminal nerve; Principal sensory nucleus of V

Definition

T HOMAS M. A CHENBACH University of Vermont Burlington, VT, USA

Synonyms ASEBA; CBCL

Nucleus responsible for proprioceptive feedback from the muscles of facial expression, stereognosis or fine tactual discrimination, and vibratory sensations from the face. Located in the dorsolateral pons just medial to the middle cerebellar peduncle and inferior to the superior cerebellar peduncle, it is the functional equivalent of the nuclei

Description The Achenbach System of Empirically Based Assessment (ASEBA) comprises a family of forms for rating

Child Behavior Checklist

behavioral/emotional problems and adaptive characteristics. For ages 1½ to 90þ years, developmentally appropriate forms are designed to be completed by collaterals who know the person who is being assessed. These forms include versions of the Child Behavior Checklist (CBCL), completed by parent figures for 1½- to 5-yearolds and for 6- to 18-year-olds; the Caregiver-Teacher Report Form (C-TRF) for ages 1½–5, completed by daycare providers and preschool teachers; the Teacher’s Report Form (TRF) for ages 6–18, completed by teachers and other school personnel; the Adult Behavior Checklist (ABCL) for ages 18–59, completed by spouses, partners, family members, friends, therapists, and other collaterals; and the Older Adult Behavior Checklist (OABCL) for ages 60 and older, completed by caregivers as well as by collaterals. The ASEBA also includes parallel forms completed by the people being assessed, including the Youth Self-Report (YSR) for ages 11–18, the Adult Self-Report (ASR) for ages 18–59, and the Older Adult Self-Report (OASR) for ages 60 and older. The collateral and self-report forms assess functioning in everyday contexts over periods of 2–6 months. In addition to the collateral and self-report forms, other ASEBA forms are designed for rating behavior observed in specific situations. These forms include the Direct Observation Form (DOF), which is completed by observers who rate two or more 10-min samples of children’s behavior observed in classrooms and other group settings; the Semistructured Clinical Interview for Children and Adolescents (SCICA), which provides an interview protocol and a rating form completed by the

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interviewer who administers the SCICA to 6- to 18-yearolds and the Test Observation Form (TOF), which test examiners use to rate the behavior observed during the administration of individual ability and achievement tests to 2- to 18-year-olds. Table 1 summarizes the ASEBA forms, ages covered, who completes the forms, and references to manuals for each form.

Normed Profiles Scores obtained from all ASEBA forms are displayed on profiles in relation to norms that are based on distributions of scale scores obtained by large samples of peers. For the collateral and self-report forms for ages 1½ to 90þ years, norms are based on a US national probability sample of people who had not received mental health or substance abuse services in the preceding 12 months. For the CBCL/6–18, TRF, and YSR, norms are provided for many cultures in addition to the USA, as detailed later (Achenbach & Rescorla, 2007a, b). Multicultural norms for the CBCL/1½–5 and C-TRF were released in 2010. For the DOF, SCICA, and TOF, norms are based on ratings of children observed in the contexts for which these instruments are designed. Each profile displays an individual’s scale scores in terms of standard scores (T scores) and percentiles based on the normative sample of that individual’s peers, as rated by a particular type of informant (e.g., parent, teacher, self). The profiles also display demarcations between the normal range, borderline clinical range, and clinical range on each scale. Figure 1 illustrates a profile

Child Behavior Checklist. Table 1 ASEBA assessment instruments Instrument

Ages

Completed by

Reference

CBCL/1½–5

1½–5

Parent figures

Achenbach and Rescorla (2000)

C-TRF

1½–5

Daycare providers, preschool teachers


CBCL/6–18

6–18

Parent figures

Achenbach and Rescorla (2001, 2007a)

TRF

6–18

Teachers


YSR

11–18

Youths


TOF

2–18

Psychological examiner

McConaughy and Achenbach (2004)

DOF

6–11

Observer


SCICA

6–18

Interviewer


ASR

18–59

Adults


ABCL

18–59

Collaterals


OASR

60

Older adults

Achenbach, Newhouse, and Rescorla (2004)

OABCL

60

Collaterals

Achenbach, Newhouse, and Rescorla (2004)

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80

S C O R E

14.Cries

29.Fears

30.FearSchool

31.FearDoBad

32.Perfect

33.Unloved

35.Worthless

45.Nervous

50.Fearful

52.Guilty

71.SelfConsc

91.ThinkSuic

112.Worries

1

0

0

0

0

2

2

2

0

0

1

2

2

Anxious/ depressed 12 70-C >97

2

2

0

0

2

2

2

2

111.Withdrawn

103.Sad

102.LacksEnergy

75.Shy

69.Secretive

65.Won’tTalk

42.PreferAlone

5.EnjoysLittle

Withdrawn/ depressed 12 83-C >97

0

0

1

0

0

1

0

1

1

2

56g.Vomit 0

1

1

2

1

2

2

0

1

2

2

79.SpeechProb

64.PreferYoung

62.Clumsy

48.NotLiked

38.Teased

36.GetsHurt

34.OutToGet

27.Jealous

25.NotGetAlong

12.Lonely

11.Dependent

Social problems 14 80-C >97

2

2

1

0

2

0

2

0

2

1

0

2

100.SleepProb

85.StrangeIdeas

84.StrangeBehav

83.StoresUp

76.SleepsLess

70.SeesThings

66.RepeatsActs

58.PicksSkin

46.Twitch

40.HearsThings

18.HarmSelf

9.MindOff

Thought problems 14 77-C >97

2

1

1

1

2

0

2

0

0

B = Borderline clinical range; C = Clinical range

56f.Stomach

56e.SkinProb

56d.EyeProb

56c.Nausea

56b.Headaches

56a.Aches

54.Tired

51.Dizzy

47.Nightm ares

Somatic complaints 6 64 92

Date filled: 01/08/2001 Birth date: 03/03/1986 Agency: CMHC Verified: Scanned

95.Temper 97.Threaten 104.Loud

2 0 0

105.UsesDrugs 1

94.Teases

0

101.Truant 0

89.Suspicious

87.MoodChang

86.Stubborn

68.Screams

57.Attacks

37.Fights

23.DisobeySchl

22.DisobeyHome

21.DestroyOther

20.DestroyOwn

19.DemAtten

16.Mean

3.Argues

2

2

1

0

0

1

1

1

0

1

0

1

2

99.Tobacco

96.ThinksSex

90.Swears

82.StealsOther

81.StealsHome

72.SetsFires

67.RunAway

63.PreferOlder

43.LieCheat

39.BadFriends

28.BreaksRules

26.NoGuilt

2.Alcohol

Aggressive behavior 14 67-B 96

0

1

1

0

0

1

0

0

0

0

0

0

1

Rule-breaking behavior 5 56 73

Broken lines = Borderline clinical range

78.Inattentive

61.PoorSchool

41.Impulsive

17.Daydream

13.Confused

10.SitStill

8.Concentrate

4.FailsToFinish

1.ActsYoung

Attention problems 9 63 90

Externalizing

Informant: Self Relationship: Self

N O R M A L

C L I N I C A L

Child Behavior Checklist. Figure 1 Syndrome profile scored from the Youth Self-Report completed by 15-year-old Wayne Webster (From Achenbach & Rescorla, 2001, p. 33)

Copyright 2001 T.M. Achenbach

Total score T score Percentile

≤50

55

60

65

70

75

85

T

90

95

Internalizing

Gender: Male Age: 15

YSR/11–18 - Syndrome scale scores for Boys (2001 version)

C

100

ID: 2301251405–003 Name: Wayne webster Clinician: Dr. Barrett

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of syndrome scales scored from the YSR completed by 15-year-old Wayne Webster (not his real name).

Scales on Which ASEBA Instruments Are Scored ASEBA problem items are scored on scales for syndromes derived empirically via exploratory factor analyses (EFAs) and confirmatory factor analyses (CFAs). These empirically derived syndromes reflect patterns of problems found to co-occur in ratings by each kind of informant. In addition to the syndrome scales, each form is scored on DSM-oriented scales constructed by having experts from many cultures select ASEBA problem items that are very consistent with particular diagnostic categories of the American Psychiatric Association’s (1994) Diagnostic and Statistical Manual-Fourth Edition (DSMIV). Like the syndrome scales, the DSM-oriented scales are displayed on profiles in terms of T scores, percentiles, and normal, borderline clinical, and clinical ranges. Most forms are also scored on scales comprising critical items that are of particular concern to clinicians. The collateral and self-report forms are additionally scored on scales for favorable characteristics, such as competence, adaptive functioning, and personal strengths. The particular items and scales are geared to the developmental level of people being assessed and to the informants’ knowledge of people being assessed. For example, parents of 6- to 18-year-olds provide data regarding their children’s involvement in sports, nonsports activities, organizations, jobs and chores, friendships, and relationships with parents, siblings, and peers. Teachers provide data on children’s academic performance and adaptive characteristics at school. For adults, data are requested regarding friendships, relations with spouse or partner, children, job, and enrolment in educational programs. Only the items relevant to the adult being assessed are scored. For example, adults who lack a spouse or partner, children, job, or enrolment in educational programs are not scored on those items. Adult forms also have normed scales for substance use.

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McConaughy, & Howell, 1987). Consequently, professionals who work with children recognize the need to obtain reports from multiple informants. Meta-analyses of correlations between collateral and self-reports of adult psychopathology have also revealed only modest correlations that argue for using multi-informant data to assess adults (Achenbach, Krukowski, Dumenci, & Ivanova, 2005). Because each informant may provide valid and useful information that differs from what other informants provide, data from multiple informants should be compared. Software for scoring ASEBA forms facilitates crossinformant comparisons by printing scores obtained from parallel forms on parallel profiles. In addition, it prints side-by-side comparisons of ratings by up to eight informants on all problem items that have counterparts on forms completed by different informants. It also prints Q correlations that measure the degree of agreement between each pair of informants and compares them with Q correlations between pairs of informants in large reference samples. An especially useful kind of comparison between informants’ reports is illustrated in Fig. 2. This is a comparison between syndromes scored from the YSR completed by Wayne Webster, CBCLs completed by Wayne’s parents, and TRFs completed by three of Wayne’s teachers. For each syndrome, such as the Anxious/Depressed syndrome shown in the upper left-hand corner, the bars reflect the magnitude of standard scores (T scores) obtained from ratings by each kind of informant. Because the T scores are based on ratings by each kind of informant for a normative sample of children, the height of the bar indicates the level of the problems reported by a particular kind of informant compared to problems reported by that kind of informant for a normative sample of children. For example, the leftmost bars indicate that the Anxious/Depressed syndrome scores obtained from CBCL ratings by Wayne’s parents are above the top broken line compared to parents’ CBCL ratings of a normative sample of adolescent boys. As scores above the top broken line are in the clinical range, the CBCL bars indicate that Wayne’s parents reported more problems of this syndrome than were reported by parents of 97% of boys in the normative sample.

Cross-Informant Comparisons Multicultural Norms Meta-analyses have revealed that correlations between parent, teacher, and self-reports of children’s problems are typically only low to moderate (Achenbach,

Norms obtained in one society may not be generalizable to other societies. To determine the degree of

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68–B TRF4

61 TRF5

63 TRF6

70–C TRF4

65–B TRF5

69–B TRF6

73–C CBC1

69–B CBC1

82–C CBC1

73–C CBC2

66–B CBC2

70–C CBC2

79–B TRF4 74–C TRF5

74–C TRF6

70–C TRF4 60 TRF5

66–B TRF6

Aggressive behavior

77–C YSR3

Thought problems

83–C YSR3

71–C CBC1

58 CBC1

76–C CBC2

61 CBC2

Informant name Carmen Hernandez Charles Dwyer

50 TRF4

58 TRF5

62 TRF6

63 YSR3

68–B TRF4

65–B TRF5

64 TRF6

Attention problems

64 YSR3

Somatic complaints

Relationship Classroom teacher {F} Classroom teacher {M}

Date 04/11/2001 04/12/2001

Comparison date: 04/13/2001

56 YSR3

63 TRF4

53 TRF5

53 TRF6

67–B YSR3

82–C TRF4

65–B TRF5

67–B TRF6

Child Behavior Checklist. Figure 2 Cross-informant comparisons of syndrome scores for Wayne Webster (From Achenbach & Rescorla, 2001, p. 39)

≤50

60

70

{F} = Female {M} = Male

Rule-breaking behavior

80–C YSR3

Social problems

70–C YSR3

Age 15 15

Birth date: 03/03/1986 Eval ID 005 006

80

62 CBC2

69–B CBC2

72–C CBC2

Form TRF5 TRF6

Withdrawn/depressed

Date 04/04/2001 04/05/2001 04/08/2001 04/10/2001

Gender: Male Relationship Biological mother Biological father Self Classroom teacher {M}

B = Borderline clinical range; C = Clinical range Broken lines = Borderline clinical range

57 CBC1

69–B CBC1

72–C CBC1

Informant name Alice N. Webster Ralph F. Webster Self George jackson

Anxious/depressed

Age 15 15 15 15

90

100

≤50

60

70

80

90

100

≤50

60

70

80

90

100

Eval ID 001 002 003 004

Cross-informant comparison - CBCL/TRF/YSR Syndrome scale T Scores (2001 version)

Name: Wayne webster

C

Form CBC1 CBC2 YSR3 TRF4

ID: 2301251405

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generalizability across societies, the same assessment instruments must be administered to large representative samples of people in different societies. This has been done with ASEBA instruments in many societies. CFAs of CBCL, TRF, and YSR data from many societies support the generalizability of syndromes that were initially derived from US samples. Comparisons of scale scores show that the distributions of CBCL, TRF, and YSR scores in many societies approximate those obtained in the USA. However, some societies have substantially lower or higher mean scores. To take account of societal differences in scale scores, separate sets of norms have been constructed for the societies obtaining relatively low scores, societies obtaining intermediate scores, and societies obtaining relatively high scores. Because parent, teacher, and self-ratings often yield different scores, the multicultural CBCL, TRF, and YSR norms were constructed separately. For some societies, problem scores obtained from one kind of informant are relatively low, while scores obtained from another kind of informant are intermediate or high. For example, CBCL and TRF problem scores obtained from Japanese parents and teachers are in the low range, whereas YSR scores obtained from self-ratings by Japanese youths are in the intermediate range. To enable practitioners and researchers to compare CBCL, TRF, and YSR scores with culturally appropriate norms, the scoring software provides options for displaying problem scale scores in relation to norms for low-scoring, intermediate-scoring, and high-scoring societies. For example, CBCL and TRF scores for a Japanese youth would typically be displayed in relation to norms for low-scoring societies. However, the youth’s YSR scores would be displayed in relation to norms for intermediate-scoring societies. If the Japanese youth lived in the USA and attended an American school, the TRF scores would be displayed in relation to US norms for teachers’ ratings. If the youth’s parents were well acculturated to the USA, the CBCL scores could be displayed in relation to US norms and also in relation to Japanese norms to see whether the scores were clinically deviant according to either set of norms.

Historical Background The ASEBA stems from Achenbach’s (1966) factoranalytic derivation of syndromes of child and adolescent psychopathology. Since then, over 4 decades of research and practical experience have produced ASEBA instruments for ages 1½ to 90þ years. Achenbach (2009) documents the historical development of ASEBA research,

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instruments, theory, and applications, as well as directions in which the ASEBA is now moving. Translations are available in 85 languages. Over 6,500 publications by some 9,000 authors report use of the ASEBA in 80 cultural groups and societies (Be´rube´ & Achenbach, 2010). ASEBA instruments are available in paper and Internet-based electronic versions in many countries around the world for practical assessment in clinical, educational, forensic, and other services, as well as for research on countless topics, such as genetics, medical conditions, outcome evaluations, epidemiology, development, diagnosis, and multicultural comparisons. Because the ASEBA’s conceptual framework is open ended and generative, it continues to advance in multiple directions (Achenbach, 2009).

Psychometric Data Table 2 summarizes psychometric data for all ASEBA instruments in terms of mean alphas, test-retest reliability, and the percentage of variance in ASEBA scale scores accounted for by clinical referral status, after partialing out demographic effects. Many additional psychometric findings – including goodness of fit obtained from CFAs in diverse samples – are reported in ASEBA manuals and in refereed publications listed by Be´rube´ and Achenbach (2010).

Clinical Uses ASEBA instruments have numerous clinical uses. Be´rube´ and Achenbach (2010) list publications reporting use of the ASEBA in relation to over 150 medical conditions. Some 600 publications report use of the ASEBA for evaluating treatments and outcomes for many kinds of psychopathology and other problems. ASEBA instruments can be used at many stages of clinical processes, including screening to identify needs for help, documentation of problems and adaptive functioning for use in clinical referrals, and intake assessment on which to base treatment decisions. During the course of treatment, ASEBA instruments are useful for determining whether goals are being met. Following the treatment, ASEBA instruments can be readministered to evaluate outcomes and subsequent functioning. At any point, ASEBA instruments can be used to assess behavioral/ emotional concomitants of neuropsychological and medical disorders. The availability of similar ASEBA instruments for children and adults facilitates family

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Child Behavior Checklist. Table 2 Summary of ASEBA psychometric data Alphaa Instrument

Narrow

Reliabilityb Broad

Narrow

Validityc Broad

Narrow

Broad

CBCL/1½–5

0.76

0.92

0.82

0.89

11

17

C-TRF

0.80

0.94

0.78

0.85

13

20

CBCL/6–18

0.83

0.94

0.88

0.92

24

32

TRF

0.86

0.94

0.84

0.90

15

20

YSR

0.78

0.92

0.78

0.85

10

14

TOF

0.82

0.90

0.76

0.84

9

14

DOF

0.68

0.79

0.51

0.72

7

20

SCICA

0.70

0.84

0.75

0.80

9

16

ASR

0.78

0.93

0.84

0.91

10

14

ABCL

0.80

0.94

0.84

0.88

6

8

OASR

0.80

0.96

0.86

0.95

13

20

OABCL

0.83

0.97

0.94

0.95

19

29

Narrow, mean for syndrome and DSM-oriented scales; broad, mean for internalizing, externalizing, and total problems. Data are from manuals listed in the Further Reading. a Cronbach’s coefficient alpha for the internal consistency of scales. b Pearson rs for test-retest reliability over 8- to 16-day intervals. SCICA rs are between ratings by different interviewers who interviewed children over intervals averaging 12 days. c Percentage of variance accounted for by clinical referral status (referred vs. nonreferred) in multiple regressions of referral status on ASEBA scale scores with demographic variables partialed out.

assessment, as well as close coordination between interventions for parents and their children.

References and Readings Achenbach, T. M. (1966). The classification of children’s psychiatric symptoms: A factor-analytic study. Psychological Monographs, 80, (No. 615). Achenbach, T. M. (2009). The Achenbach system of empirically based assessment (ASEBA): Development, findings, theory, and applications. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. Achenbach, T. M., Krukowski, R. A., Dumenci, L., & Ivanova, M. Y. (2005). Assessment of adult psychopathology: Meta-analyses and implications of cross-informant correlations. Psychological Bulletin, 131, 361–382. Achenbach, T. M., McConaughy, S. H., & Howell, C. T. (1987). Child/ adolescent behavioral and emotional problems: Implications of cross-informant correlations for situational specificity. Psychological Bulletin, 101, 213–232. Achenbach, T. M., Newhouse, P. A., & Rescorla, L. A. (2004). Manual for the ASEBA older adult forms & profiles. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. Achenbach, T. M., & Rescorla, L. A. (2000). Manual for the ASEBA preschool forms & profiles. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. Achenbach, T. M., & Rescorla, L. A. (2001). Manual for the ASEBA schoolage forms & profiles. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families.

Achenbach, T. M., & Rescorla, L. A. (2003). Manual for the ASEBA adult forms & profiles. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. Achenbach, T. M., & Rescorla, L. A. (2007a). Multicultural supplement to the manual for the ASEBA school-age forms & profiles. Burlington, VT: University of Vermont Research Center for Children, Youth, and Families. Achenbach, T. M., & Rescorla, L. A. (2007b). Multicultural understanding of child and adolescent psychopathology: Implications for mental health assessment. New York: Guilford Press. Achenbach, T. M., & Reescorla, L. A. (2010). Multicultural guide to the manual for the ASEBA preschool forms & profilies. Burlington, Vermont, University of Vermont Research Center for Children, Youth, and Families. American Psychiatric Association (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: Author. Be´rube´, R. L., & Achenbach, T. M. (2010). Bibliography of published studies using the Achenbach system of empirically based assessment (ASEBA). Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. McConaughy, S. H., & Achenbach, T. M. (2001). Manual for the semistructured clinical interview for children and adolescents (2nd ed.). Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. McConaughy, S. H., & Achenbach, T. M. (2004). Manual for the test observation form for ages 2–18. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families. McConaughy, S. H., & Achenbach, T. M. (2009). Manual for the ASEBA direct observation form for ages 6–11. Burlington, VT: University of Vermont, Research Center for Children, Youth, and Families.

Childhood Autism Rating Scales

Childhood Autism ▶ Autistic Disorder

Childhood Autism Rating Scales M AUREEN G RISSOM University of Missouri Columbia, MO, USA

Synonyms CARS

Description The childhood autism rating scale (CARS) is a 15 item measure intended to assist in distinguishing children with autism spectrum disorder (ASD) from children with other types of delays. It is an observational scale in which each item is rated from 1 (within normal limits) to 4 (severely abnormal) and ratings include consideration of ‘‘peculiarity, frequency, and duration’’ of the behavior rated (Schopler, Reichler, & Renner, 1988). It yields a total score ranging from 15 to 60. Scores of 30–36.5 suggest mild to moderate Autism and 37–60 suggest severe Autism. However, when used with adolescents and adults, the cut-off has been decreased to 28 (Schopler et al., 1988). The CARS was initially developed using a sample of 1,606 children, approximately three quarters of whom were male. Sixty seven percent of the sample was white, 30% African American, and 3% was of other racial descent. Within the male and female samples, the age distribution was similar with 56% age 5 years or younger, 32% between ages 6 and 10 years, and 11% age over 10 years. Seventy one percent of the sample had an IQ (as determined by one of various measures) below 70, 17% with an IQ between 70 and 84, and 13% with an IQ above 84 (Schopler et al., 1988).

Historical Background The CARS was developed at the University of North Carolina at Chapel Hill and was used to evaluate children referred to the TEACCH program. The CARS has been in use since 1971 (Reichler & Schopler, 1971) at which point it was called the childhood psychosis rating scale (CPRS)

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and the CARS was published as an appendix to the 1980 article by Schopler, Reichler, DeVellis, and Daly. It was originally intended for use by trained diagnosticians making ratings based on observation; however, the measure has been expanded for use by others (e.g., physicians, speech–language pathologists, special educators, and audiologists) as well as to include information from other sources such as parent report and classroom observations. It is important to note that the CARS was developed prior to the DSM-IV concept of Autism as a spectrum of disorders (Magyar & Pandolfi, 2008).

Psychometric Data The CARS Manual (1988) describes high internal consistency reliability (alpha = 0.94) and an average interrater reliability of 0.71 for two independent raters across 280 cases. Test–retest reliability for 91 cases rated 1 year apart by two separate raters was 0.88. The sensitivity of the CARS was 100% for a sample of 54 children with autistic disorder in a study by Rellini et al. (2003). However, there is concern that the CARS may overdiagnose children with cognitive impairments as having autism with lower IQ scores correlating with higher CARS scores (Perry, Condillac, Freeman, Dunn-Geier, & Belair, 2005). In addition, sensitivity in the Rellini et al. study was not as great for distinguishing children with Asperger’s disorder and pervasive developmental disorder-not otherwise specified (PDD-NOS) from children with attention-deficit/hyperactivity disorder (ADHD) and language delay. Despite the fact that the CARS was initially published during the time of the DSM-III, the CARS has shown good agreement with clinical diagnosis with the DSM-IV/ICD-10 criteria (Perry, Condillac, Freeman, Dunn-Geier, & Belair, 2005). Several studies have looked at the relations between the CARS and other measures intended to assist in the diagnostic process. In one study, the CARS and the Autism Diagnostic Interview-Revised (ADI-R) were found to correlate 91.8% for cases in which a child received a diagnosis of Autism and 44.4% for those in which an Autism diagnosis was not given (Pilowsky, Yirmiya, Shulman, & Dover, 1998). More recently, Saemundsen, Magnusson, Smari, and Sigurdardottir (2003) found a correlation of 0.81 between the CARS (having a total score greater than 30) and the ADI-R (meeting cut-offs in all three domains) in a study that included 54 children. Several factor analyses of the 15 items comprising the CARS have been conducted. Magyar and Pandolfi (2007) found that four components: (1) a social component, (2) a negative emotionality construct, (3) a

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Childhood Epileptic Encephalopathy with Diffuse Slow Spike-and-waves

sensory and stereotypy construct, and (4) an ‘‘activity level and consistency of intellectual response’’ component, accounted for 57% of the variance in ratings.

Rating Scale (CARS). Journal of Autism and Developmental Disorders, 10, 91–103. Schopler, E., Reichler, R. J., & Renner B. (1988). The Childhood Autism Rating Scale (CARS). Los Angeles, CA: Western Psychological Services.

Clinical Uses The CARS can be used as part of multimodal approach to the diagnosis of ASD that ‘‘includes observation of the child, caregiver interview, assessment of developmental levels, detailed developmental history, and screening for associated disorders such as Fragile X’’ (Magyar & Pandolfi, 2007, p. 1787).

Childhood Epileptic Encephalopathy with Diffuse Slow Spike-and-waves ▶ Lennox–Gastaut Syndrome

Cross References ▶ Autism Diagnostic Interview-Revised ▶ Asperger’s Disorder ▶ Attention-Deficit/Hyperactivity Disorder ▶ Autism Diagnostic Observation Schedule ▶ Autistic Disorder ▶ Pervasive Developmental Disorder Not Otherwise Specified ▶ TEACCH

References and Readings Magyar, C. I., & Pandolfi, V. (2007). Factor structure evaluation of the Childhood Autism Rating Scale. Journal of Autism and Developmental Disorders, 37, 1787–1794. Perry, A., Condillac, R. A., Freeman, N. L., Dunn-Geier, J., & Belair, J. (2005). Multi-site Study of the Childhood Autism Rating Scale (CARS) in Five Clinical Groups of Young Children. Journal of Autism and Developmental Disorders, 35, 625–634. Pilowsky, T., Yirmiya, N., Shulman, C., & Dover, R. (1998). The Autism Diagnostic Interview-Revised and the Childhood Autism Rating Scale: Differences between diagnostic systems and comparison between genders. Journal of Autism and Development Disorders, 28, 143–151. Reichler, R. J. & Schopler, E. (1971). Observations on the nature of human relatedness. Journal of Autism and Childhood Schizophrenia, 1, 283–296. Rellini, E., Tortolani, D., Trillo, S., Carbone, S., & Montecchi, F. (2004). Childhood Autism Rating Scale (CARS) and Autism Behavior Checklist (ABC) Correspondence and Conflicts with DSM-IV Criteria in Diagnosis of Autism. Journal of Autism and Developmental Disorders, 34, 703–708. Saemundsen, E., Magnusson, P., Smari, J., & Sigurdardottir, S. (2003). Autism Diagnostic Interview-Revised and the Childhood Autism Rating Scale: Convergence and discrepancy in diagnosing Autism. Journal of Autism and Developmental Disorders, 33, 319–328. Schopler, E., Reichler, R. J., DeVellis, R. F., & Daly, K. (1980). Toward objective classification of childhood autism: Childhood Autism

Childhood or Adolescent Brain Injury ▶ Pediatric Traumatic Brain Injury

Children’s Category Test VANESSA L. R AMOS , K EITH O. Y EATES Nationwide Children’s Hospital Columbus, OH, USA

Synonyms CCT

Description The Children’s Category Test (CCT) is an abbreviated version of the original Halstead Category Test (HCT; Reitan & Wolfson, 1992). The CCT is an individually administered instrument designed to measure nonverbal learning and memory, concept formation, and problemsolving abilities. The CCT consists of two levels. Level 1 is given to children aged 5–8 and consists of five subtests and 80 items. Level 2 is given to children aged 9–16 and consists of six subtests and 83 items. The child’s task is to identify the single conceptual rule underlying the items in

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each subtest. The last subtest on both levels requires the child to remember and reapply the conceptual rules from previous subtests. The CCT was normed on a stratified representative sample of 920 children in 12 age groups ranging from 5 years to 16 years, 11 months. Administration requires approximately 15–20 min. The raw score is the total number of errors (CCT Total), which is converted into an age-normed T-score (M = 50, SD = 10). The CCT is easy to administer and score.

Historical Background The CCT was developed in an effort to provide an efficient and well-normed children’s version of the HCT, which is well documented to be sensitive to cerebral impairment (Reiten & Wolfson, 1992), and the previous children’s versions of the Category Test (Reiten & Wolfson, 1993). Concerns about lengthy administration times and expensive, bulky equipment led to the development of various short forms of these tests (e.g., Short Category Test Booklet Format), with the most comprehensive of these efforts resulting in the CCT (Boll, 1993). The CCT and California Verbal Learning Test-Children’s Version (CVLT-C) were standardized and normed on the same population.

Psychometric Data Median internal consistency reliability for the CCT Total score is 0.88 for Level 1 and 0.86 for Level 2, with average standard errors of measurement of 3.46 and 3.74, respectively (Boll, 1993). The separate age-level values and averaged coefficients indicate that the CCT possesses a high degree of internal consistency across ages. The manual reports a variety of studies demonstrating that the CCT consistently and significantly correlates with other measures of achievement or cognitive ability, such as the WISC-R and CVLT-C.

Clinical Uses The CCT is a widely used test of the ability to solve problems by developing and modifying strategies of responding to various visual designs and patterns (Nesbit-Greene & Donders, 2002). The CCT does not require demonstration of acquired skills, ability, or knowledge, and eliminates potential confounding variables because it can be used with children with motor

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deficits or speech/language difficulties (Boll, 1993). The CCT can provide insights regarding a child’s cognitive abilities and learning strategies in terms of difficulties in memory or shifting between conceptual ideas (MacNeil Horton, 1996). However, a poor score on the CCT does not necessarily indicate a neurologically based disorder; rather it indicates a disruption in mental processing (Boll, 1993). Studies of the CCT’s sensitivity to brain dysfunction indicate that the measure is not consistently sensitive to structural brain damage or neurodevelopment disorders in children (Bello, Allen, & Mayfield, 2008; Donders, 1996). Results from studies examining the sensitivity of the CCT to various forms of brain dysfunction suggest that the CCT assesses multiple dimensions of problem solving, rather than a general construct of abstraction (Nesbit-Greene & Donders, 2002), which has led some to caution against relying on the total error score as the sole index of brain dysfunction (Allen, Knatz, & Mayfield, 2006; Bello et al., 2008). The use of the CCT with braininjured children may be hampered by the fact that the overall T-score is based on six different subtests that differ in their sensitivity to the severity of the injury (NesbitGreene & Donders, 2002). Another problem with using the CCT with brain-injured children is that it is untimed and children are provided with corrective feedback throughout the test administration, and these features may compensate for any deficits in processing speed or executive function that the child may be experiencing (Donders & NesbitGreene, 2004). Overall, studies investigating the use of the CCT with brain-injured children suggest that caution is needed in interpreting the results, and that it should be supplemented with psychometric data from additional neuropsychological tests (Donders & Nesbit-Greene, 2004; Moore, Donders, & Thompson, 2004).

Cross References ▶ Concept Learning ▶ Delis-Kaplan Executive Function System ▶ Halstead-Reitan Neuropsychological Test Battery ▶ Memory (Including Memory Impairment) ▶ Nonverbal Learning Disabilities ▶ Problem Solving

References and Readings Allen, D. N., Knatz, D. T., & Mayfield, J. (2006). Validity of the children’s category test-level 1 in a clinical sample of heterogeneous forms of brain dysfunction. Archives of Clinical Neuropsychology, 21, 711–720.

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Bello, D. T., Allen, D. A., & Mayfield, J. (2008). Sensitivity of the children’s category test level 2 to brain dysfunction. Archives of Clinical Neuropsychology, 23, 329–339. Boll, T. (1993). Children’s category test. San Antonio, TX: Psychological Corporation. Donders, J. (1996). Validity of short forms of the intermediate Halstead category test in children with traumatic brain injury. Archives of Clinical Neuropsychology, 11, 131–137. Donders, J., & Nesbit-Greene, K. (2004). Predictors of neuropsychological test performance after pediatric traumatic brain injury. Assessment, 11(4), 275–284. MacNeil Horton, A. (1996). Book and test reviews. Archives of Clinical Neuropsychology, 11, 171–173. Moore, B. A., Donders, J., & Thompson, E. H. (2004). Validity of the children’s category test-level 1 after pediatric traumatic brain injury. Archives of Neuropsychology, 19, 1–9. Nesbit-Greene, K., & Donders, J. (2002). Latent structure of the children’s category test after pediatric traumatic head injury. Journal of Clinical and Experimental Neuropsychology, 24(2), 194–199. Reiten, R. M., & Wolfson, D. (1992). Neuropsychological evaluation of older children. South Tucson, AZ: Neuropsychology Press. Reiten, R. M., & Wolfson, D. (1993). The Halstead-Reitan neuropsychological test battery: Theory and clinical interpretation (2nd ed.). South Tucson, AZ: Neuropsychology Press.

Children’s Memory Scale M ORRIS J. C OHEN Medical College of Georgia Augusta, Georgia, USA

Synonyms CMS

Description The Children’s Memory Scale (CMS), published in 1997, provides a comprehensive assessment of learning and memory in children and adolescents of ages 5 through 16 years. The CMS is individually administered and designed to be used as part of a standard psychological or neuropsychological evaluation. It assesses declarative learning and memory functions across three domains: Auditory/Verbal, Visual/Nonverbal, and Attention/Concentration (working memory). Each domain contains two core subtests and one supplemental subtest. The subtests comprising the Attention/Concentration domain provide measures of attention and working memory. Each subtest in the Auditory/ Verbal and Visual/Nonverbal domains provide measures

of both immediate and delayed (30 min) recall. Each Auditory/Verbal subtest also provides a measure of recognition recall. After administration of the core subtests (Table 1), the examiner can derive eight index scores: Attention/Concentration, Verbal Immediate, Verbal Delayed, Delayed Recognition, Visual Immediate, Visual Delayed, Learning, and General Memory (Fig. 1). The learning Index is derived using subtest scores from the Auditory/Verbal (Word Pairs) and the Visual/Nonverbal (Dot Locations) domains. The General Memory Index is a measure of global memory functioning and is generated using both the immediate and delayed memory indexes from the Auditory/Verbal and Visual/Nonverbal domains. The immediate portion of the core battery takes approximately 30–40 min to administer, with an additional 10–20 min required for administration of the delayed recognition sections. Two record forms are provided for students of 5–8 and 9–16 years. Scoring tables in the manual or computer software allow for conversion of raw scores to scaled scores (mean = 10; SD = 3) at the individual subtest level and standard scores (mean = 100; SD = 15) at the index level. Table 1 provides a brief description of the core subtests.

Historical Background Development of the CMS began in 1985 to provide clinicians with a comprehensive, well-standardized, individually administered instrument that would assess the important processes involved in learning and memory within the pediatric population. Prior to this, traditional psychological evaluation of children with neurological and neurodevelopmental disorders included tests of intelligence, achievement, and behavior/emotional functioning with little if any attention paid to the child’s ability to learn and remember new information. This was the case despite the fact that most referrals were in some way related to the student’s inability to learn and remember school-related content. As a result, the CMS was developed with five goals in mind: 1. The development of an instrument that was consistent with current theoretical models of learning and memory. 2. The development of an instrument that was sensitive to developmental changes over time. 3. To evaluate the relationship between memory and intelligence and provide the clinician with a mechanism to meaningfully evaluate discrepancies between IQ and learning/memory performance.

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Children’s Memory Scale. Table 1 Description of CMS core index and subtest components Core Index Verbal Memory Indexes (Immediate, Delayed, and Recognition)

Core Subtest Stories

Subtest Description Two stories (age-dependent 5–8-, 9–12-, and 13–16-year olds) are read. Immediately after presentation of each story, the child is asked to repeat as much of the story as can be remembered. In the delayed portion, the child retells the stories and then answers questions about the stories (recognition recall).

Word Pairs A list of 10 or 14 (age-dependent 5–8-year olds; 9–16-year olds) related and unrelated word pairs are read; thereafter the stem is read and the child recalls the associate. Three learning trials are administered followed by a free recall. In the delayed portion, the child is asked to recall the word pairs spontaneously followed by a recognition section. Visual/Non-Verbal Memory Indexes (Immediate and Delayed)

Attention/Concentration Index

Dot Locations

The child is shown an array of dots (blue) located within a rectangle in a stimulus book. This page is removed and the client is asked to replicate the spatial location of the dots by placing chips on a 3 4 or 4 4 rectangular grid (depending on age 6 dots for 5–8-year olds; 8 for dots 9–16-year olds). Three learning trials are administered followed by presentation of a distractor array (red dots) after which an immediate recall trial is presented. In the delayed portion, the child is asked to reproduce the original blue dot array.

Faces

The child is presented with a series of 12 (5–8-year olds) or 16 (9–16-year olds) pictured human faces one at a time. In the immediate and delayed recall sections, the child is asked to identify the stimulus faces from a different set of foils (36 or 48 colored photos).

Numbers

A digit span forwards and backwards task (similar to the WISC-III subtest).

Sequences The client is asked to mentally sequence or manipulate information as quickly as possible. The 12 items include such tasks as reciting numbers, days of the week and months in forward and reverse order, and counting by 2s, 4s, and 6s. Scoring is based upon accuracy and speed.

4. The inclusion of a diversified selection of clinically and educationally relevant tasks that would allow clinicians to indentify and characterize learning and memory disorders in children and help them to design remedial and compensatory programs based upon the child’s performance. 5. The development of an instrument that could be successfully administered within a standardized testing situation and also be child friendly. As such, the CMS focused upon the assessment of declarative memory with no attempt made to formally evaluate procedural memory, which involves skill learning and classical conditioning. Further, the CMS was unable to provide measures of long-term memory beyond 30 min due to the time restriction of a traditional assessment and the logistical problems inherent in the reevaluation of the standardization sample over a longer time interval.

Psychometric Data The CMS was standardized on a representative US sample of 1,000 children. The sample was stratified according to age (10 age groups ranging in age from 5 to 16 years; 100 per age group), sex (equal number of males and females in each age group), race/ethnicity (White, African American, Hispanic, and other), geographic region (northeast, north central, south, west), and parent education level (five categories ranging from 45; 3) permanent psychiatric exclusions include lifetime diagnoses of bipolar affective disorders, schizophrenia of any subtype, delusional disorders of any subtype, dementias of any subtype, organic brain disorders, and alcohol or substance abuse within 2 years before onset of the fatiguing illness, any past or current diagnosis of major depressive disorder with psychotic or melancholic features, anorexia nervosa, or bulimia. If none of the exclusions apply, using the 1994 case definition, CFS is diagnosed based on the following criteria: (1) fatigue is of new or definite onset, not due to exertion, not relieved by rest, and must result in substantial reductions in previous levels of educational, occupational, social, or personal activities, (2) fatigue is of at least 6 months duration, and (3) concurrent with at least four of the following eight symptoms that could not have predated the onset of fatigue: impairment in short-term memory or concentration severe enough to result in substantial reductions in previous levels of educational, occupational, social, or personal activities, painful lymph nodes in front or back of the neck or under the arms, sore throat, muscle pain, pain in more than one joint without accompanying redness or swelling, headaches of a new type, unrefreshing sleep, postexertional malaise lasting

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more than 24 h. These symptoms are nonspecific and variable in both nature and severity over time. They were selected on the basis of consensus clinical opinion and were not identified empirically.

Categorization Only a small percentage of patients complaining of fatigue will be categorized as having CFS. Most patients either have prolonged fatigue, defined as self-reported, persistent fatigue of 1 month or longer, or chronic fatigue defined as self-reported persistent or relapsing fatigue of 6 or more consecutive months (Fukuda et al., 1994). Other conditions of unexplained etiology with similar symptom profiles are often comorbid with CFS and can include Fibromyalgia Syndrome (FMS), Temporo-Mandibular Syndrome (TMS), Irritable Bowel Syndrome (IBS), Multiple Chemical Sensitivity (MCS), Gulf War Syndrome (GWS), Major Depressive Disorder (MDD), and anxiety disorders.

Epidemiology In the United States, CFS occurs in up to about 0.5% of the general population. It is most commonly found in middle-aged women and is most common in Latinos, followed by African Americans, and Whites. The illness affects women (predominantly between the ages of 40 and 59) more often than men (Reyes et al., 2003). In general, the expression of the syndrome is not genderspecific (Buchwald, Pearlman, Kith, & Schmaling, 1994). Chronic Fatigue Syndrome can also occur in children and adolescents. While gender distribution is similar to that of adults, prevalence rates are significantly lower.

Natural History, Prognostic Factors, Outcomes Disorders with similar symptom profiles have been described for at least two centuries and have been known under a variety of names including neurasthenia, Akureyri disease, Epstein Barr Syndrome, and chronic mononucleosis. Although many hypotheses exist about the causes for CFS, the etiology of the condition is still unclear. Some believe that CFS is a latent form of depression and anxiety disorder, while others view the syndrome as a sleep disorder, attribute it to endocrine dysfunction or abnormalities within the central nervous system (CNS). Abnormalities identified in individuals with CFS include Hypothalamic–Pituitary–Adrenal Axis

dysfunction, cortisol dysregulation, small white matter lesions in the frontal regions of the brain, orthostatic and cognitive dysfunction. Most abnormalities are found in CFS patients who do not suffer from comorbid psychiatric disorder, most commonly depression. Treatments, especially cognitive behavioral and graded exercise treatments, enhance the prognosis for improvement. If untreated, complete recovery from CFS is rare.

Neuropsychology and Psychology of Chronic Fatigue Syndrome CFS patients typically complain of difficulties with concentration, memory, and thinking, yet neuropsychological testing does not generally confirm the reported cognitive dysfunction. Available data suggest that the main cognitive deficit in individuals with CFS is slowed information processing, which can affect memory as well as executive function. Depression is a very common comorbid condition (Tiersky, Johnson, Lange, Natelson, & DeLuca, 1997). Neuroimaging data increasingly provide evidence for decreased cerebral blood flow and functional activation of brain areas suggesting increased cognitive effort (Lange, Wang, Deluca, & Natelson, 1998; Lange et al., 2005).

Evaluation A neuropsychological testing battery for individuals with CFS should include measures of overall current and premorbid cognitive function (i.e., Wechsler Test of Adult Reading, Wechsler Adult Intelligence Scale III/IV), simple and complex attention as well as information processing and working memory (i.e., Continuous Performance Test, Gordon, Trails, Paced Auditory Serial Addition Test), executive function (i.e., subtests of Delis–Kaplan Executive Function System including verbal fluency, towers test; Wisconsin Card Sort Test), memory (i.e., Wechsler Memory Scale III/IV, California Verbal Learning Test II, Rey Osterrieth Complex Figure Test), language function (i.e., Boston Naming Test), visual– perceptual function (i.e., Judgment of Line Orientation, Hooper), and motor function (i.e., grip strength, finger tapping, pegboard). It is also recommended to test the level of motivation and effort expanded during neuropsychological testing as well as emotional functioning to improve interpretability of test results. When an individual is diagnosed with CFS, it may be desirable to evaluate the intensity and severity of symptoms associated with CFS using self-report

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questionnaires. To assess fatigue intensity, several measures have been used including the Chalder Fatigue Scale and the Krupp Fatigue Scale; both have acceptable psychometric properties. The Chalder Fatigue Scale is a 14item instrument with a 4-choice format and separates mental and physical fatigue (Chalder et al., 1993). The Krupp Fatigue Severity Scale includes nine items rated on 7-point scales and is sensitive to different aspects and gradations of fatigue severity. Most items in the Krupp scale are related to behavioral consequences of fatigue (Krupp, LaRocca, Muir-Nash, & Steinberg, 1989). The Checklist Individual Strength (CIS) is a 20-item inventory with four subscales commonly used to measure fatigue severity, concentration, reduced motivation, and physical activity. The CIS focuses on fatigue over the preceding 2 weeks (Vercoulen, Swanink, Fennis, Galama, Van der Meer, & Bleijenberg, 1994). Another commonly used instrument to measure fatigue severity is the Multidimensional Fatigue Inventory, also a 20-item questionnaire providing scores for severity of general fatigue, physical fatigue, mental fatigue, as well as reduced motivation and activity (Smets, Garssen, Bonke, & De Haes, 1995). Another symptom that might warrant additional investigation is pain. Five of the eight CFS-defining symptoms reflect pain (headaches of a new type, pattern, or severity, muscle pain, and multi-joint pain without swelling or redness, sore throat, tender cervical/axillary lymph nodes). In many cases, pain may be the primary determinant of disability for some individuals with CFS. The McGill Pain Questionnaire (MPQ) is a well-validated questionnaire that can be used to further characterize pain or follow the course of pain in CFS (Melzack, 1975). The MPQ includes four components: (1) a human figure drawing on which patients are asked to mark the location of their pain; (2) a series of adjectives divided into groups from which patients identify their experience by circling word descriptors; (3) questions about prior pain experience, pain location, and information on the use of pain medication; and (4) a present pain intensity index.

Treatment Effective treatment needs to be tailored to each individual diagnosed with CFS and often consists of a combination of behavioral, pharmacological, and physical interventions. Behavior modification or cognitive restructuring are two cognitive behavioral therapy (CBT) approaches used to treat CFS. Using this modality, reductions in the effect of fatigue on functional ability and quality of life have been shown in CFS (Kroenke, Taylor-Vaisey,

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Dietrich, & Oxman, 2000). Medication management of fatigue is in its infancy; presently, there is no FDA approved drug for fatigue. Over the counter medications offer a first line of therapeutic possibilities. Stronger medications, including stimulants and antidepressants, require a physician’s prescription. In general, medications used to treat depression, anxiety, and pain are very often used for the pharmacologic treatment of CFS. It is important to recognize that fatigue is not solely due to disease processes, but can occur as an indirect consequence of decreased physical activity and conditioning. Graded aerobic exercise training is a safe and effective treatment for CFS and has been shown to improve quality of life. The primary goal of exercise is to avoid the spiral of deconditioning that is common in most fatiguing diseases.

Cross References ▶ Epstein Barr Syndrome ▶ Gulf War Syndrome ▶ Neurasthenia ▶ Unexplained Illness

References and Readings Buchwald, D., Pearlman, T., Kith, P., & Schmaling, K. (1994). Gender differences in patients with chronic fatigue syndrome. Journal of General International Medicine, 9, 397–401. Chalder, T., Berelowitz, G., Pawlikowska, T., Watts, L., Wessely, S., Wright, D., et al. (1993). Development of a fatigue scale. Journal of Psychosomatic Research, 37, 147–153. Carruthers, B. M., Jain, A. K., De Meirleir, K. L., Peterson, D. L., Klimas, N. G., Lerner, A. M. et al. (2003). Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome: clinical Working Case Definition, Deagnostic and Treatment Protocols. Journal of Chronic Fatigue Syndrome, 11, 7–115. DeLuca, J. (2008). Fatigue as a window to the brain. London, UK: MIT Press. Fukuda, K., Straus, S. E., Hickie, I., Sharpe, M. C., Dobbins, J. G., & Komaroff, A. (1994). The chronic fatigue syndrome: a comprehensive approach to its definition and study. International Chronic Fatigue Syndrome Study Group. Annals of Internal Medicine, 121, 953–959. Holmes, G. P., Kaplan, J. E., Gantz, N. M., Komaroff, A. L., Schonberger, L. B., Straus, S. E., et al. (1988). Chronic fatigue syndrome: a working case definition. Annals of Internal Medicine, 108, 387–389. Jason, L. A., Bell, D. S., Rowe, M. D., van Hoof, E. L. S. J. K., Lapp, C., et al. (2006). A pediatric case definition for myalgic encephalomyelitis and chronic fatigue syndrome. Journal of Chronic Fatigue Syndrome, 13, 1–44. Kroenke, K., Taylor-Vaisey, A., Dietrich, A. J., & Oxman, T. E. (2000). Interventions to improve provider diagnosis and treatment of mental disorders in primary care. A critical review of the literature. Psychosomatics, 41, 39–52.

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Krupp, L. B., LaRocca, N. G., Muir-Nash, J., & Steinberg, A. D. (1989). The fatigue severity scale. Application to patients with multiple sclerosis and systemic lupus erythematosus. Archives of Neurology, 46, 1121–1123. Lange, G., Steffener, J., Cook, D. B., Bly, B. M., Christodoulou, C., Liu, W. C., et al. (2005). Objective evidence of cognitive complaints in Chronic Fatigue Syndrome: a BOLD fMRI study of verbal working memory. Neuroimage, 26, 513–524. Lange, G., Wang, S., Deluca, J., & Natelson, B. H. (1998). Neuroimaging in chronic fatigue syndrome. American Journal of Medicine, 105, 50S–53S. Melzack, R. (1975). The McGill pain questionnaire: major properties and scoring methods. Pain, 1, 277–299. Reyes, M., Nisenbaum, R., Hoaglin, D. C., Unger, E. R., Emmons, C., Randall, B., et al. (2003). Prevalence and incidence of chronic fatigue syndrome in Wichita, Kansas. Archives of Internal Medicine, 163, 1530–1536. Sharpe, M. C., Archard, L. C., Banatvala, J. E., Borysiewicz, L. K., Clare, A. W., David, A., et al. (1991). A report–chronic fatigue syndrome: guidelines for research. Journal of the Royal Society of Medicine, 84, 118–121. Smets, E. M., Garssen, B., Bonke, B., & De Haes, J. C. (1995). The Multidimensional Fatigue Inventory (MFI) psychometric qualities of an instrument to assess fatigue. Journal of Psychosomatic Research, 39, 315–325. Tiersky, L. A., Johnson, S. K., Lange, G., Natelson, B. H., & DeLuca, J. (1997). Neuropsychology of chronic fatigue syndrome: a critical review. Journal of Clinical and Experimental Neuropsychology, 19, 560–586. Vercoulen, J. H., Swanink, C. M., Fennis, J. F., Galama, J. M., Van der Meer, J. W., & Bleijenberg, G. (1994). Dimensional assessment of chronic fatigue syndrome. Journal of Psychosomatic Research, 38, 383–392. Wessely, S. (1995). The epidemiology of chronic fatigue syndrome. Epidemiological Review, 17, 139–151.

Short Description or Definition The Global Initiative for Chronic Obstructive Lung Disease (GOLD) Workshop defines COPD as ‘‘a disease state characterized by airflow limitation that is not fully reversible. The airflow limitation is usually both progressive and associated with an abnormal inflammatory response of the lungs to noxious particles or gases.’’ The GOLD committee was organized by the World Health Organization (WHO) and the US National Heart Lung and Blood Institute (NHLBI). Additional information about definitions, diagnosis, treatment, and research can be found at www.gold copd.com.

Categorization COPD has traditionally been defined by severity of airflow limitation. Indeed, the GOLD COPD classification system relies on measures of airflow limitation to stratify COPD severity (i.e., Forced Expiratory Volume in 1 Second (FEV1) and Forced Expiratory Volume in 1 Second/Forced Vital Capacity(FEV1/FVC ratio)). This staging system is currently the primary determinant of treatment guidelines. Heterogenity in the pathophysiology underlying COPD has been increasingly recognized over the past decade including chronic bronchitis, peripheral airways disease, and emphysema (Friedlander, Lynch, Dyar, & Bowler, 2007). At times, asthma has also been included in grouping of patients with COPD.

Chronic Multisymptom Illnesses ▶ Unexplained Illness

Chronic Obstructive Pulmonary Disease E LIZABETH KOZORA , K ARIN F. H OTH National Jewish Health Denver, CO, USA

Synonyms Chronic bronchitis; COPD; Emphysema

Epidemiology COPD is the fourth leading cause of death in United States. Worldwide mortality from COPD is predicted to increase owing to the increased tobacco consumption in third world countries, and COPD mortality among women is increasing faster than mortality among men (Anthonisen & Manfred, 2004). The third National Health and Nutrition Examination Survey, conducted between 1988 and 1994, demonstrated that between 5% and 8% of the US population had COPD as defined by physiological parameters (NHANES III). The worldwide prevalence of COPD in 1990 was estimated by the WHO/ World Global Burden of Disease Study to be 9.34 per 1,000 in men and 7.33 per 1,000 in women. In the US, COPD death rates are low among people under the age of 45, but increase with age.

Chronic Obstructive Pulmonary Disease

Natural History, Prognostic Factors, Outcomes Risk factors for COPD include both environmental and host-related variables. Cigarette smoke has been identified as the most important external risk factor, followed by pipe and cigar smoke, occupational dusts and chemicals, history of severe childhood respiratory infections, HIV infection, outdoor pollution, and IV drug use. Host-related factors include airways hyper-responsiveness, genetic factors, severe hereditary alpha1-antitrypsin deficiency, low birth weight, and maternal cigarette smoking during gestation (Anthonisen & Manfred, 2004).

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in daily activity, increased disease severity, and impaired health status (Hopkins & Bigler, 2001; McSweeny & Labuhn, 1996).

C Evaluation Neuropsychological evaluation for cognitive problems associated with COPD typically involves measurement of attention, learning and memory, reasoning and executive functioning skills, visuomotor speed, and visuoperceptual function. Psychological evaluation typically involves measures of depression, anxiety, and health-related quality of life.

Neuropsychology and Psychology of COPD Treatment Empirical studies have documented neuropsychological (i.e., cognitive) deficits in patients with chronic airway obstruction and COPD. Two of the largest studies conducted in the 1980s were the Nocturnal Oxygen Therapy Trial (NOTT) and the Intermittent Positive Pressure Breathing Trial (Prigatano & Levin, 1988). The pattern and extent of cognitive dysfunction reported in COPD vary across patients and appear to be associated with disease severity. In COPD patients with moderate to severe hypoxemia, deficits have been identified in simple motor movement and overall strength, perceptualmotor integration, abstract reasoning, attention to auditory stimuli, learning and memory, and language skills. In patients with mild hypoxemia, impairments in higher cerebral functioning include abstract reasoning, auditory and visual attention, verbal and nonverbal learning and recall, and reasoning and motor skills (Kozora et al., 2008). There is some evidence that cognitive impairment has an independent impact on patients’ daily functioning, medical regimen adherence, and quality of life, although results have been mixed (McSweeny & Labuhn, 1996). Psychological changes and emotional distress have also been noted in COPD patients. To date, depression and anxiety are the most commonly observed psychological problems in COPD (Hynninen, Breitve, Wiborg, Pallesen, & Nordhus, 2005) with estimates of the prevalence of depression ranging from approximately 25% to 50%. Some of the discrepancies in prevalence estimates across studies may be related to differences in the method used to assess depression. In addition to emotional distress, multiple studies have demonstrated poor quality of life in patients with COPD, which has been associated with restrictions

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Research to date suggests that a variety of therapeuticapproaches utilized in COPD patients (including oxygen therapy, comprehensive rehabilitation programs, and surgical techniques) improve psychological and cognitive functioning. Long-term (greater than 6 months) use of oxygen therapy improves cognitive performance in COPD, probably due to direct effects of improved oxygen delivery to the central nervous system (Prigatano & Levin, 1988). Early studies reported improvement in visual memory, verbal memory, and motor speed among the COPD subjects following 6 months of continuous oxygen therapy. Large multisite studies have also demonstrated benefits of oxygen for cognitive function in COPD. For example, in the Nocturnal Oxygen Therapy Trial, COPD patients receiving continuous oxygen therapy for 12 months experienced greater improvements in cognitive performance than did patients receiving only nocturnal oxygen therapy. There is also evidence suggesting that comprehensive multidisciplinary rehabilitation programs can improve cognitive functioning and psychological status in emphysema patients. Comprehensive rehabilitation programs for treatment of COPD are well established and typically include assessment, education, instruction on respiration, psychosocial support, and exercise training with the goal of restoring patients to the highest level of independent function (Make, 2004). As noted in a recent review article (Kozora et al., 2008), Emery and colleagues first reported in 1991 improved complex attention in COPD patients following a 30-day exercise rehabilitation program that included instructional/educational components, psychosocial counseling, and stress reduction. In a

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subsequent study published in 1998, they reported improvement in verbal fluency and reduction in symptoms of anxiety and depression in a group participating in exercise, stress reduction, and education programs when compared to a control group participating in stress reduction and education treatment only. This finding highlighted the utility of the exercise component toward improved cognitive and psychological functions. In 2002, Kozora and colleagues also reported improvement in visual attention and semantic fluency, among COPD patients following a 3-week comprehensive rehabilitation program when compared to untreated COPD and healthy control subjects similar in age, education, and gender. This program included exercise, educational, instructional, and psychosocial components. In addition to cognitive changes, significant improvement in depressive symptoms was reported. Together, these studies indicate improved cognitive performance and psychological status following comprehensive rehabilitation with an exercise component. Many studies have also reported increased quality of life following comprehensive rehabilitation (Make, 2004). Finally, studies have suggested some improvement in cognition for moderate to severe COPD patients following lung volume reduction surgery (LVRS). In an ancillary study of the National Emphysema Treatment Trial (NETT), Kozora and colleagues (Kozora et al., 2008) examined neuropsychological and psychological functioning of patients receiving LVRS when compared to patients receiving only medical therapy (MT). The LVRS group showed significant improvement compared to the MT group at 6 months on a measure of psychomotor speed, delayed recall for verbal information, and trends toward improved sequential thinking, and psychomotor speed and naming to confrontation. LVRS patients also experienced significant reduction in depression at 6 months, as reflected in total Beck Depression Inventory score. There was no direct evidence that improved cognition in LVRS was related to improved physical capacity (workload and 6 min walk) or pulmonary function.

Cross References ▶ Anoxia

References and Readings Anthonisen, N. R., & Manfred, J. (2004). Epidemiology of chronic obstructive pulmonary disease. In J. D. Crapo, J. Glassroth,

J. B. Karlinsky, & T. E. King (Eds.), Baum’s textbook of pulmonary diseases (7th ed.). Philadelphia: Lippincott, Williams & Wilkins. Friedlander, A. L., Lynch, D., Dyar, L. A., & Bowler, R. P. (2007). Phenotypes of chronic obstructive pulmonary disease. COPD: Journal of Chronic Obstructive Pulmonary Disease, 4(4), 355–384. Hopkins, R. O., & Bigler, E. D. (2001). Pulmonary disorders. In R. E. Tarter, M. Butters, & S. R. Beers (Eds.), Medical neuropsychology (2nd ed., pp. 247–266). New York: Kluwer/Plenum. Hynninen, K. M., Breitve, M. H., Wiborg, A. B., Pallesen, S., & Nordhus, I. H. (2005). Psychological characteristics of patients with chronic obstructive pulmonary disease: A review. Journal of Psychosomatic Research, 59(6), 429–443. Kozora, E., Emery, C., Kaplan, R. M., Wamboldt, F. S., Zhang, L., & Make, B. J. (2008). Cognitive and psychological issues in emphysema. Proceedings of the ATS, 5(4), 556–560. Make, B. (2004). Pulmonary rehabilitation. In J. D. Crapo, J. Glassroth, J. B. Karlinsky, & T. E. King (Eds.), Baum’s textbook of pulmonary diseases (7th ed., pp. 289–307). Philadelphia: Lippincott Williams and Wilkins. McSweeny, A. J., & Labuhn, K. T. (1996). The relationship of neuropsychological functioning to health-related quality of life in systemic medical disease: The example of chronic obstructive pulmonary disease. In I. Grant & K. M. Adams (Eds.), Neuropsychological assessment of neuropsychiatric disorders (pp. 577–602). New York: Oxford University Press. Prigatano, G. P., & Levin, D. C. (1988). Pulmonary system. In R. E. Tarter, D. H. Van Theiel, & K. L. Edwards (Eds.), Medical neuropsychology (pp. 11–26). New York: Plenum.

Chronic Organic Brain Syndrome ▶ Organic Brain Syndrome

Chronic Progressive Multiple Sclerosis ▶ Primary Progressive Multiple Sclerosis

Chronic Widespread Pain Disorder ▶ Fibromyalgia

Cingulate Cortex ▶ Cingulate Gyrus

Cingulate Gyrus

Cingulate Gyrus M ICHAEL M EGA Providence Brain Institute Portland, OR, USA

Synonyms Cingulate cortex; Subcallosal; Subgenual

Structure The cingulate gyrus, first named by Burdach in 1822, was combined with the anterior olfactory region and hippocampus by Broca to form the ring of olfactory processing he termed the grand lobe limbique. Anatomic studies revealed extensive connections between the anterior thalamus, known to be associated with the hippocampus and hypothalamus, and the cingulate. In 1937, Papez combined these anatomic results with the clinical reports of emotional disturbances following lesions to the cingulate and other limbic structures to propose a mechanism of emotion based on a limbic circuit. For Papez, the integration of internal feelings and emotional responsiveness with the functions of the lateral cerebral cortex occurred in the cingulate. The limbic circuit of Papez however did not find anatomical evidence to support the closing connection of the cingulate to the hippocampus until 1975, when Shipley and Sørensen documented that the presubiculum, which receives a dense cingulate outflow, projects heavily to layer III of the entorhinal cortex – the origin of the perforant pathway into hippocampal pyramidal cells. Understanding the function of the cingulate in the integration of internal feelings and emotional responsiveness with movement and thought begins with an appreciation of its cytoarchitecture. Brodmann’s original cytoarchitectonic separation of anterior and posterior cingulate cortex into areas 24 and 23 based upon the presence of agranular cortex in area 24 contrasted with the granular cortex of area 23 has been refined by more accurate studies of the progressive cytoarchitectonic elaboration in the ventral to dorsal direction. Two main centers of isocortical development can be traced phylogenetically and through cytoarchitectonic progression. These two developmental trends, termed paralimbic belts, are transitional cortical zones from less-differentiated allocortex to more-differentiated isocortex with two functional centers:

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the more rostral olfactory piriform paleocortex unites the orbitofrontal, insular, and temporopolar regions, while the more caudal archicortex of the hippocampus provides the nidus for developmental spread through parahippocampal and entorhinal regions into the posterior cingulate. Both paralimbic belts reflect a different emphasis within the cingulate (Fig. 1). The orbitofrontal-centered belt processes the internal affective state of the organism. The more recent hippocampal-centered belt is the externally directed evaluative arm of the limbic system. Both work in concert, enabling the selection of environmental stimuli based on the internal relevance those stimuli have for the organism. Although both areas 24 and 32 are part of the hippocampal belt, the more rostral connections of area 24 contrast with the more caudal sensory connections of area 23, distinguishing the anterior executive from the posterior evaluative cingulate. Appreciating that the major reciprocal connections between the orbitofrontal trends are with the anterior cingulate, while the hippocampal trends are with the posterior cingulate, will assist the understanding of cingulate function. Three anterior effector regions and a posterior processing region also emerge through a review of the efferent and afferent connections of the cingulate. The three anterior regions include a visceral effector region inferior to the genu of the corpus callosum encompassing area 25, the anterior subcallosal portions of 24a–b, and 32; a cognitive effector region, which includes most of the supra-callosal area 24, and areas 24a’–b’ and 32’; and a skeletomotor effector region within the depths of the cingulate sulcus that includes areas 24c’/23c on the ventral bank, with 24c’g and 6c on the dorsal bank. These three cingulate effector regions integrate ascending input concerning the internal milieu of the organism with visceral motor systems, cognitive-attentional networks, and skeletomotor

Cingulate Gyrus. Figure 1 The Orbiotfrontal (red) and hippocampal (blue) paralimbic trends

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centers to produce the affective motivation necessary for the organism’s engagement in the environment. The posterior sensory processing region (areas 23a–b and 29/30) assists in the memory and processing of environmental stimuli targeted as relevant to the organism based on their motivational valence. The following sections will explore the connections of these four cingulate regions (Figs. 2 and 3) and their function as observed from electrical stimulation, imaging, and clinical data.

Function Visceral effector region. Major reciprocal connections with the visceral effector region are with the basal and accessory basal amygdala, medial orbitofrontal cortex areas 11, 12, and 13, anterior superior temporal pole area 38, and the anterior ventral claustrum. Major nonreciprocal projections from the visceral effector region target the parasympathetic nucleus of the solitary tract, the sympathetic thoracic intermediolateral column, the dorsal motor

Cingulate Gyrus. Figure 2 Cytoarchitectonic divisions of the cingulate and adjacent areas

nucleus of the vagus, and the nucleus accumbens/olfactory tubercle of the ventral striatum. Brain areas that have reciprocal connections with the visceral effector region, except the claustrum that supplies auditory input, also influence visceral function when stimulated. This results from their amygdalar connections that convey the visceral state of the organism to paralimbic areas. The orbitofrontal cortex mediates empathic, civil, and socially appropriate behavior. Rostral auditory association cortex in the superior temporal area also provides auditory information to the visceral effector region. No visual information has direct access to the subcallosal area. The dorsolateral prefrontal lobe, functioning as an ‘‘executive processor,’’ provides nonreciprocal input from areas 9 and 46 to the subcallosal anterior cingulate region. Executive functions permit an organized behavioral response to solve a complex problem. This includes the activation of remote memories, self-direction, and independence from environmental contingencies, shifting and maintaining behavioral sets appropriately, generating motor programs, and using verbal skills to guide behavior. Dorsolateral prefrontal efferents into the subcallosal cingulate provide feedback inhibition on the basic drives of hunger, aggression, and reproductive urges. Table 1 for a summary of these anatomic connections. Subcallosal cingulate processing modulates visceral output from brain-stem sympathetic and parasympathetic centers. Access to the basal ganglia, via the ventral striatum, allows processing of the internal milieu of the organism to influence the skeletal motor system as well. This visceral motor network encompasses the bulk of the orbitofrontalcentered paralimbic belt dedicated to assessing the emotional valence of objects based upon internal motivating drives. Patients with lesions in this area are often

Cingulate Gyrus. Table 1 Major reciprocal connections and nonreciprocal targets for the visceral effector region of the cingulate cortex Reciprocal connections Basal and accessory basal amygdala Medial orbitofrontal areas 11, 12, and 13 Superior temporal pole area 38 Anterior ventral claustrum Nonreciprocal targets Cingulate Gyrus. Figure 3 The four functional divisions of the cingulate. The visceral effector region (1), the cognitive effector region (2), the skeletomotor effector region (3), and the posterior cingulate (4)

Parasympathetic nucleus of solitary tract Sympathetic intermediolateral column Dorsal motor nucleus of the vagus Nucleus accumbens and olfactory tubercle

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disinhibited with irritability, lability, tactlessness, and fatuous euphoria. Patients act upon visceral drives without regard to social decorum. Cognitive effector region. Major reciprocal connections with the cognitive effector region are with the basal amygdala, prefrontal areas 8, 9, 10, and 46, caudal orbitofrontal area 12, inferior temporal pole area 38, rostral insula, anterior parahippocampal areas 35 and 36, and the anterior medial claustrum. Areas reciprocally connected to the cognitive effector region share a general similarity with the subcallosal anterior region underscoring their common membership in the orbitofrontal paralimbic belt. The cognitive effector region is more developed in its cytoarchitecture, than the subcallosal cingulate, and thus has stronger connections with more phylogenetically recent neocortex of dorsolateral prefrontal areas 8, 9, 10, and 46 devoted to executive function. The amygdala provides internal affective input to the supracallosal anterior cingulate. The distribution of amygdala efferents delineates the dorsal boundary of the cingulate as a functional system. Auditory input arises from the anterior medial claustrum as well as a minor link with the auditory association area of the superior temporal gyrus. The rostral insula and anterior parahippocampal areas provide additional reciprocal connections with the cognitive effector region not associated with the subcallosal region. Rostral insular cortex is a transitional paralimbic region that integrates visceral alimentary input with olfactory and gustatory afferents. Connections with the anterior parahippocampal areas 35 and 36 allow the supracallosal cingulate to influence multimodal sensory afferents entering the hippocampus. Major nonreciprocal projections of the supracallosal anterior cingulate include the auditory association cortex of anterior superior temporal area 22, allowing the cognitive effector region to influence language and the access of semantic stores. The posterior parietal area 7a and the dorsomedial head of the caudate are also targets. Parietal area 7a is the sensory component in the extrapersonal attentional network linked with the dorsolateral prefrontal ‘‘executive system.’’ The head of the caudate is also a target of this executive prefrontal cortex. Cingulate input to the caudate assists in the initiation of vocalization behavior as well as executive function. Emotional vocalizations occurring during stimulation in monkeys requires intact cingulate efferents to the periaqueductal gray that produce similar behaviors when stimulated. The most caudal amygdalar projections to 24c, extending into anterior 24c’, innervate a face representation region that may have direct connections with the facial nucleus in the pons. Efferents to the dorsomedial pons provide cingulate influence on the

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reticular activating system and its control over arousal. Table 2 for a summary of these anatomic connections. Skeletomotor effector region. Major reciprocal connections with the skeletomotor effector region are with the primary and supplementary/premotor motor cortex areas 4 and 6, prefrontal areas 8, 9, and 46, parietal areas 1, 2, 3a, 5, and 7b, and caudal insula. Major nonreciprocal projections from the skeletomotor area target the lateral putamen, spinal cord, ventromedial parvocellular division of the red nucleus, and the ventrolateral pontine gray matter. Primary motor cortex has very limited input. The medial supplementary, lateral premotor, and cingulate skeletal motor regions are the only forebrain inputs to the primary motor cortex. The skeletomotor region in the banks of the cingulate sulcus conveys limbic influence to the medial supplementary, lateral premotor, and primary motor cortex. Frontal eye fields in area 8 also share reciprocal connections with the skeletal motor effector region. Areas 9 and 46 of the dorsolateral prefrontal cortex contribute executive input to the limbic motor system. Thus, executive and limbic systems gain access to primary motor area 4 indirectly. The cingulate skeletal motor region receives the greatest outflow from executive prefrontal cortex than all other motor cortices underscoring its influence over goal-directed behavior. Sensory-motor parietal areas 1, 2, 3a, and 5 also have reciprocal connections with the skeletal motor center within the banks of the cingulate sulcus. The rostral parietal area 7b has a strong relationship with the premotor cortex, while the granular cortex of the caudal

Cingulate Gyrus. Table 2 Major reciprocal connections and nonreciprocal targets for the cognitive effector region of the cingulate cortex Reciprocal connections Basal amygdale Prefrontal areas 8, 9, 10, and 46 Caudal orbitofrontal cortex area 12 Inferior temporal pole area 38 Rostral insula Anterior parahippocampal areas 35 and 36 Anterior medial claustrum Nonreciprocal targets Anterior superior temporal area 22 Parietal area 7a Dorsomedial head and body of caudate Periaqueductal gray Dorsomedial pontine gray matter

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insula is a somatosensory limbic region. More corticospinal neurons are found in the cingulate than are found in the supplementary motor cortex, and the cingulate has about 40% of the amount found in primary motor cortex. Table 3 for a summary of these anatomic connections. Sensory processing region. Major reciprocal connections with the sensory processing region are with caudal parietal area 7a, frontal eye fields area 8, posterior perirhinal area 35, presubiculum, posterior parahippocampal area 36, prefrontal area 46, and the ventral caudal claustrum. Major nonreciprocal projections from the sensory processing region target the dorsal caudate, posterior superior temporal gyrus area 22, and orbitofrontal area 11. These regions are shown in Table 4. Cingulate Gyrus. Table 3 Major reciprocal connections and nonreciprocal targets for the skeletomotor effector region of the cingulate cortex Reciprocal connections Primary motor area 4 Supplementary motor area 6 Prefrontal areas 8, 9, and 46 Parietal areas 1, 2, 3a, 5, and 7b Caudal insula Nonreciprocal targets Lateral putamen Spinal cord Red nucleus Ventrolateral pontine gray matter

Cingulate Gyrus. Table 4 Major reciprocal connections and nonreciprocal targets for the sensory processing region of the cingulate cortex Reciprocal connections Caudal parietal area 7 Frontal eye fields area 8 Prefrontal area 46 Posterior parahippocampal areas 35 and 36 Presubiculum Ventral caudal claustrum Nonreciprocal targets Dorsal caudate Posterior superior temporal area 22 Orbitofrontal area 11

The posterior granular sensory cortices are distinguished from the anterior agranular executive cortices. The posterior cingulate, with its prominent granular layer IV, is dedicated to visuospatial and memory processing. Major reciprocal connections are with the dorsal visual system of the inferior parietal lobe dedicated to spatial processing and with the frontal eye fields in area 8. Reciprocal connections with lateral prefrontal area 46 allow an interaction between executive and sensory/mnemonic processing, which may mediate perceptual working memory tasks. Posterior parahippocampal and perirhinal areas 36 and 35, as well as the presubiculum, are reciprocally connected to the sensory processing region of the posterior cingulate. These connections modulate the multimodal efferents entering the entorhinal layer III cells that form the perforant pathway into the hippocampus. Feedback from these areas to the cingulate provides highly processed sensory information, and the ventral visual system involved in feature analysis can influence the posterior cingulate through these connections. Although in cats dorsal caudal claustrum is related to visual processing, while the ventral caudal claustrum receives auditory input, it is possible that in primates visual information may reach the posterior cingulate via the ventral caudal claustrum. The nonreciprocal targets of the posterior sensory processing region include the dorsal caudate which also receives input from area 7a of the caudal inferior parietal lobe. This shared connection between the dorsal head of the caudate and the dorsal visual system of area 7a supports the role of the posterior cingulate in visual attention. Output to posterior superior temporal area 22 will influence auditory association cortex. Limited efferents to the rostral portion of area 11 provide the only overlap with the orbitofrontal-centered belt. Electrical stimulation of the cingulate. Electrical stimulation studies of the cingulate in humans and animals are difficult to interpret because differing techniques have been used in these investigations. With varying intensity, time course, and location of stimulation, it is not surprising that a spectrum of results is noted. Despite technical variations, stimulation of the anterior cingulate in humans regularly produces visceral motor and affective changes, speech alterations, and automatic motor behaviors (Meyer, McElhaney, Martin, & McGraw, 1973). In contrast to inhibitory responses elicited by the stimulation of primary motor cortex, which cannot be controlled, respiratory arrest from cingulate stimulation can be overcome volitionally (Penfield & Jasper, 1954). Automatic behaviors noted include unilateral and bilateral movements and repetitive ‘‘tic like’’ movements of the hands,

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lips, or tongue. These movements can also be consciously suppressed; implicating the cingulate as an ‘‘unconscious’’ effector supports its role in the pathophysiology of obsessive–compulsive disorder (behaviors that respond well to cingulotomy). Fear, pleasure, agitation, euphoria, and a sense of well being – affective phenomena also common after limbic stimulation – have been reported (Meyer, McElhaney, Martin, & McGraw, 1973). Involuntary vocalizations and speech arrests are less common in humans than in animals with stimulation of areas 32, 24, and the rostral part of 25 (Vogt & Barbas, 1988). Functional activation of the cingulate. In functional activation studies, the cognitive effector region of the anterior cingulate is activated when sustained attention to novel tasks is required. Tasks spanning motor, language, memory, and visuospatial paradigms all produce supracallosal anterior cingulate activation. When memory encoding is combined with a motor task demanding sustained divided attention (Fletcher et al., 1995), only the anterior cingulate is activated due to the sustained vigilance demanded by dividing effort between the two tasks. When motivation to master a task is no longer required, and accurate performance of a task becomes routine, the anterior cingulate returns to a baseline activity level (Raichle et al., 1994). The acquisition of novel cognitive strategies requires the ‘‘dynamic vigilance’’ of the supracallosal cingulate, but with practice the motivation required to entrain new cognitive networks to a novel task is no longer necessary. A distinction between motivation and attention is important. A task is still attended to and completed correctly after the motivating influence of the supracallosal cingulate has initiated the acquisition of an efficient cognitive routine. Through the activation of the anterior supracallosal cingulate limbic motivation directs the selection of the best cognitive strategy among many competing contingencies. Thus, activation studies using varied tasks consistently activate the cognitive effector region in normals motivated to succeed in whatever task is given them. The contribution to an extrapersonal attentional network – involving direct links between the anterior cingulate, dorsolateral executive frontal area, and the inferior parietal lobule – provided by the cognitive effector region is the motivation to engage in a cognitive challenge. Functional imaging has also confirmed the role of the skeletomotor effector region in the preparation of motor output and motor learning. When a motor task is only imagined, the cingulate cortex inferior and anterior to the supplementary motor area (dorsal bank of the cingulate sulcus) shows significant activation. During the acquisition of procedural learning in a rotory pursuit task, the

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cingulate skeletomotor region is also activated (Grafton, Woods, & Tyszka, 1994).

Illness Structural and functional abnormalities. Seizures originating in the anterior cingulate can alter visceral activity, produce involuntary skeletomotor output, result in disturbed attention, and cause interictal behavior abnormalities. The severity and specific abnormality will depend upon the location of the seizure focus and ensuing damage that affects interictal brain function. A diverse assortment of atonic, absence, speech arrest, autonomic, and complex partial seizures with secondary generalization have been described. Inaccessibility of the medial hemisphere to surface electrode recording is the greatest obstacle to the elucidation of cingulate seizures. In a study involving 36 cases (Mazars, 1970), depth electrodes revealed near-instantaneous bilateral spread to the frontal poles when the focus was in the anterior cingulate; posterior foci spread to the contralateral cingulate within seconds, followed by involvement of the convexities with generalized tonic–clonic seizures. Emotional stress often precipitated the seizures. Psychoses and episodic rage were common interictal behavioral abnormalities that responded to the removal of the anterior cingulate and occasionally the frontal pole as well. Consciousness may be altered and automatisms can be voluntarily inhibited or integrated with ongoing movements (Geier et al., 1977). An 11-year-old girl who initially had atonic seizures at age 30 months was reported to develop complex partial seizures with blinking, lip smacking, automatisms, and humming (Levin & Duchowny, 1991). An obsessive– compulsive disorder developed over a 5-year period, and by age 8, she became preoccupied with Satan and her personal hygiene. Seizure focus, recorded from depth electrodes, was in the right anterior cingulate. The patient’s behavioral abnormalities responded well to a 4-cm ablation of the right anterior cingulate. Another case of a right anterior cingulate focus with accompanying behavioral abnormalities has been described (Devinsky, Morrell, & Vogt, 1995). One year after mild head trauma, a 42-year-old male developed, over a 15-year period, sociopathic behavior and complex partial seizures unresponsive to medical treatment. Seizures were usually nocturnal and frequent (10–20 per night), with stereotypic motor output: facial contortions, tongue thrusting, a strangulated yell, flexion of the neck and trunk, bilateral extremity extension, and thrashing

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with preserved consciousness. Occasionally, generalization to tonic–clonic seizures developed with loss of consciousness. Irritability, disinhibition, and sexual deviancy were behavioral complications in a police officer who was dismissed from the force because of brutality and the use of confiscated drugs. Surface and sphenoidal electroencephalogram showed rhythmic bifrontal theta. Magnetic resonance imaging (MRI) and [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET) were essentially normal, but depth electrodes revealed a right cingulate focus which spreads to the ipsilateral orbitofrontal area and contralateral anterior cingulate in 300 ms. Resection of the right cingulate and anterior corpus callosum relieved 90% of the spells with only brief axial flexion being the residual seizures. The behavioral abnormalities were reported to improve with the patient married and employed as a fast-food restaurant manager. Both stimulation and seizure activity can discharge the functional centers of the cingulate to produce a visceral effect, a cognitive or behavioral change, and a speech or motor output. Appreciating the functional centers within the cingulate assists the interpretation of the signs and symptoms exhibited when it discharges. The interictal behavioral abnormalities of anterior cingulate epilepsy reflects the dysfunction of limbic networks which, if affecting infracallosal and orbitofrontal cortex, will result in visceral motor disturbances and disinhibition with socially inappropriate behavior. Obsessive– compulsive features may occur with dysfunction of the cognitive component of the supracallosal cingulate. This abnormal ‘‘dynamic vigilance’’ exerted by the cognitive effector region in obsessive–compulsive disorders can occur from a well-circumscribed seizure focus in this region (Levin & Duchowny, 1991) and is relieved by surgical ablation of this region or its outflow (Tow & Whitty, 1953). Focal lesions and syndromes. Well-circumscribed lesions in humans are rarely confined to one region of the cingulate. With an anterior lesion, both the cognitive, skeletomotor and visceral effector regions are often affected. Bilateral lesions result in an akinetic mute state (▶ Akinetic Mutism). Patients are profoundly apathetic. Rarely moving, and incontinent, they eat and drink only when fed, and if speech occurs it is limited to monosyllabic responses to questioning. Patients appear awake with eyes tracking objects. Displaying no emotions, even when in pain, patients show complete indifference to their circumstance. Transient akinetic mutism with similar features occurs with unilateral lesions. The akinetic mute state can also result from bilateral paramedian diencephalic and midbrain

lesions, possibly affecting the ascending reticular core. Failure of response inhibition on go-no-go tests is the major neuropsychological deficit in the patient with an anterior medial frontal damage. The loss of spontaneous motor activity results when the lesion involves the supplementary motor area and the skeletomotor effector region. When these two motor regions are spared, motor activity will be normal but the patient will demonstrate profound indifference, docility, and the loss of motivation to engage in a task. They can be led by the examiner to engage in a task but will fail to self-generate sustained directed attention. They lack cognitive motivation. The role of the anterior cingulate as a cognitive effector is appreciated within the realm of language. Language, a cognitive function, is distinguished from the motor function of speech. Transcortical motor aphasia (TCMA) is the usual result of left anterior medial or anterior dorsolateral prefrontal lesions. The classic syndrome of TCMA is initial mutism that resolves in days to weeks, yielding a syndrome featuring delayed initiation of brief utterances without impaired articulation, excellent repetition, inappropriate word selection, agrammatism, and poor comprehension of complex syntax. Activation of dorsolateral prefrontal cortices enabling language and speech arises from two sources: the anterior cingulate and the supplementary motor area (with the cingulate skeletomotor effector region). When the executive prefrontal cortex (areas 9, 10, and 46) is disrupted, cognitive language deficits are prominent (TCMA, type I); when motor neurons in area 4, devoted to the speech apparatus, are disconnected from their activation, speech hesitancy and impoverished output ensues (TCMA, type II). These two functional realms are separable and can be disconnected anywhere along two pathways. Direct damage to the supplementary motor area or its efferent pathway to the motor cortex traveling in the anterior superior paraventricular white matter will produce TCMA type II. Direct damage to the anterior cingulate, its outflow to areas 9, 10, and 46, or to the caudate – via the subcallosal fasciculus, just inferior to the frontal horn of the lateral ventricle – will disrupt frontal-subcortical circuits involved in motivation and executive cognitive functions. The initial muteness has been described by a patient after recovering from an anterior cingulate/supplementary motor infarction as a loss of the ‘‘will’’ to reply to her examiners, because she had ‘‘nothing to say,’’ her ‘‘mind was empty,’’ and ‘‘nothing mattered’’ (▶ Akinetic Mutism). The cingulum bundle has also been the site of surgical lesions (cingulumotomy, or cingulotomy when cingulate cortex is also removed) to treat psychiatric and pain disorders. The cingulum contains the efferents and afferents

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of the cingulate to the hippocampus, basal forebrain, amygdala, and all cortical areas, as well as fibers of passage between hippocampus and prefrontal cortex, and from the median raphe´ to the dorsal hippocampus. Surgical ablation of the anterior portion (sparing fibers relevant to memory function) is most successful when treating aggression, extreme anxiety, obsessive–compulsive behaviors, and severe pain. Psychotic symptoms show only a temporary response. The three anterior cingulate regions, by virtue of the distinct functional systems they access, are the conduits through which limbic motivation can activate feeling, thought, and movement. Lesions of the posterior cingulate disrupt memory function in animals and humans. The closing link in the circuit of Papez, from the anterior thalamic efferents traveling through the posterior cingulum to areas 32 and 29/30, is the cingulate projection sent to the presubiculum. Anterior cingulotomy will not disrupt this memory circuit but rarely pathologic lesions will extend into, and beyond, the posterior cingulate. If the lesion extends inferior to the splenium of the corpus callosum, it may also disrupt the fornix, thus disconnecting the efferents from the hippocampus to the diencephalon. If the lesion extends posteriorly, it may damage the supracommissural portion of the hippocampus – the gyrus fasciolaris and the fasciola cinerea. A large leftsided lesion that extended beyond the posterior cingulate into the fornix and supracommissural hippocampus after the surgical repair of an arteriovenous malformation resulted in a persistent amnesia (Cramon & Schuri, 1992). Disruption of septo-hippocampal pathways in the cingulum and fornix were thought by the authors to play a significant role in the patient’s clinical deficit, but other important components of Papez’s circuit had clearly been damaged. A rare lesion restricted to the left posterior cingulate, the cingulum, and the splenium of the corpus callosum (but possibly sparing the fornix) resulted in a severe amnesia after the repair of an arteriovenous malformation (Valenstein et al., 1987). The analysis of rare circumscribed lesion in humans cannot determine if the posterior cingulate cortex, rather than the cingulum or neighboring members of Papez’s circuit, result in amnesia when lesioned due to the location of fiber pathways to the hippocampus that are buried in the posterior cingulate. Excitotoxic lesions in animals that destroy neurons but spare fibers of passage can clarify this issue. Based upon posterior cingulate cortical lesions, using the selective cytotoxin quisqualic acid (Sutherland & Hoesing, 1993), results in animal studies reveal that area 29 neurons are necessary for the acquisition and retention of spatial and nonspatial memory. Furthermore, the posterior cingulate acts in concert with the anterior thalamus

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and the hippocampus during encoding and may also be important in the storage of long-term information. Synthesizing cytoarchitectonic refinements, nonhuman primate tracer studies, clinical-behavioral correlation data, and functional neuroimaging results have been refined – but have not significantly added to – the basic description of cingulate function offered by Papez: " It is thus evident that the afferent pathways from the recep-

tor organs split at the thalamic level into three routes, each conducting a stream of impulses of special importance. One route conducts impulses through the dorsal thalamus and the internal capsule to the corpus striatum. This route represents ‘the stream of movement.’ The second conducts impulses from the thalamus through the internal capsule to the lateral cerebral cortex. This route represents ‘the stream of thought.’ The third conducts a set of concomitant impulses through the ventral thalamus to the hypothalamus and by way of the mamillary body and the anterior thalamic nuclei to the gyrus cinguli, in the medial wall of the cerebral hemisphere. This route represents ‘the stream of feeling.’ In this way, the sensory excitations which reach the lateral cortex through the internal capsule receive their emotional coloring from the concurrent processes of hypothalamic origin which irradiate them from the gyrus cinguli. (Papez, 1937)

References and Readings Devinsky, O., Morrell, M. J., & Vogt, B. A. (1995). Contributions of anterior cingulate cortex to behavior. Brain, 118, 279–306. Fletcher, P. C., Firth, C. D., Grasby, P. M., Shallice, T., Frackowiak, R. S. J., & Dolan, R. J. (1995). Brain systems for encoding and retrieval of auditory-verbal memory. An in vivo study in humans. Brain, 118, 401–416. Geier, S., Bancaud, J., Talairach, J., Bonis, A., Szikla, G., & Enjelvin, M. (1977). The seizures of frontal lobe epilepsy. A study of clinical manifestations. Neurology, 27, 951–958. Grafton, S. T., Woods, R. P., & Tyszka, M. (1994). Functional imaging of procedural motor learning: Relating cerebral blood flow with individual subject performance. Human Brain Mapping, 1, 221–234. Levin, B., & Duchowny, M. (1991). Childhood obsessive-compulsive disorder and cingulate epilepsy. Biological Psychiatry, 30, 1049–1055. Mazars, G. (1970). Criteria for identifying cingulate epilepsies. Epilepsia, 11, 41–47. Meyer, G., McElhaney, M., Martin, W., & McGraw, C. P. (1973). Stereotactic cingulotomy with results of acute stimulation and serial psychological testing. In L. V. Laitinen & K. E. Livingston (Eds.), Surgical approaches in psychiatry (pp. 39–58). Baltimore: University Park Press. Papez, J. W. (1937). A proposed mechanism of emotion. Archives of Neurology and Psychiatry, 38, 725–733. Penfield, W., & Jasper, H. (1954). Epilepsy and the functional anatomy of the human brain. Boston: Little Brown.

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Raichle, M. E., Fiez, J. A., Videen, T. O., MacLeod, A.-M. K., Pardo, J. V., Fox, P. T., et al. (1994). Practice-related changes in human brain functional anatomy during nonmotor learning. Cerebral Cortex, 4, 8–26. Sutherland, R. J., & Hoesing, J. M. (1993). Posterior cingulate cortex and spatial memory; a microlimnology analysis. In B. A. Vogt & M. Gabriel (Eds.), Neurobiology of cingulate cortex and limbic thalamus: A comprehensive handbook (pp. 461–477). Boston: Birkhaüser. Tow, P. M., & Whitty, C. W. M. (1953). Personality changes after operations of the cingulate gyrus in man. Journal of Neurology Neurosurgery & Psychiatry, 16, 186–193. Valenstein, E., Bowers, D., Verfaellie, M., Heilman, K. M., Day, A., & Watson, R. T. (1987). Retrosplenial amnesia. Brain, 110, 1631–1646. Vogt, B. A., & Barbas, H. (1988). Structure and connections of the cingulate vocalization region in the rhesus monkey. In J. D. Newman (Eds.), The physiological control of mammalian vocalization (pp. 203–225). New York: Plenum Press. Von Cramon, D. Y., & Schuri, U. (1992). The septo-hippocampal pathways and their relevance to human memory: A case report. Cortex, 28, 411–422.

Cingulum I RENE P IRYATINSKY Butler Hospital and Alpert Medical School of Brown University Providence, RI, USA

Definition The cingulum is a collection of white matter fibers projecting from the cingulate gyrus to the entorhinal cortex in the brain, allowing for communication between components of the limbic system. The cingulum connects the medial temporal lobe with the posterior cingulate gyrus. Diffusion tensor imaging studies have reported cingulum bundle disruption in mild cognitive impairment and Alzheimer’s disease. In addition, some research indicates that in Alzheimer’s disease, the posterior cingulate cortex hypofunction is due to the indirect effect of the degeneration of cingulum fibers secondary to medial temporal lobe atrophy.

Cross References

Fellgiebel, A., Mu¨ller, M. J., Wille, P., Dellani, P. R., Scheurich, A., Schmidt, L. G., et al. (2005). Color-coded diffusion-tensor-imaging of posterior cingulate fiber tracts in mild cognitive impairment. Neurobiology of Aging, 26(8), 1193–1198. Firbank, M. J., Blamire, A. M., Krishnan, M. S., Teodorczuk, A., English, P., Gholkar, A., et al. (2007). Atrophy is associated with posterior cingulate white matter disruption in dementia with Lewy bodies and Alzheimer’s disease. NeuroImage, 36(1), 1–7. Rose, S. E., McMahon, K. L., Janke, A. L., O’Dowd, B., de Zubicaray, G., Strudwick, M. W., et al. (2006). Diffusion indices on magnetic resonance imaging and neuropsychological performance in amnestic mild cognitive impairment. Journal of Neurology, Neurosurgery & Psychiatry, 77(10), 1122–2112. Wakana, S., Jiang, H., Nagae-Poetscher, L. M., van Zijl, P. C. M., & Mori, S. (2004). Fiber tract-based atlas of human white matter anatomy. Radiology, 23(1), 77–87. Zhang, Y., Schuff, N., Jahng, G. H., Bayne, W., Mori, S., Schad, L., et al. (2007). Diffusion tensor imaging of cingulum fibers in mild cognitive impairment and Alzheimer disease. Neurology, 68(1), 13–19.

CIQ ▶ Community Integration Questionnaire

Circadian Clock ▶ Circadian Rhythms

Circadian Rhythms B RUCE J. D IAMOND William Paterson University Wayne, NJ, USA

Synonyms Biological cycles; Biorhythms; Circadian clock

▶ Cingulate Gyrus

Short Description or Definition References and Readings Catani, M., Howard, R. J., Pajevic, S., & Jones, D. K. (2002). Virtual in vivo interactive dissection of white matter fasciculi in the human brain. NeuroImage, 17(1), 77–94.

The pervasive characteristics of living organisms are rhythmic biological and behavioral changes, which are expressed at varying levels of organization ranging from basic cellular to the highly complex level. These rhythms

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are generally referred to as circadian rhythms and they organize a variety of behaviors, including sleep, which is characterized by a 90-min Rapid Eye Movement (REM) cycle and the wakefulness–sleep cycle, which is organized around an approximate 24-h cycle (Carlson, 1999).

Developmental factors may also mediate some of the variations in circadian rhythms. For example, the aging process is often accompanied by changes in circadian rhythms that may affect sleep and place older adults at a higher risk for sleep disturbance (Lee-Chiong, 2005).

Categorization

History and Impact

The core body temperature rhythm and the sleep–wake cycle are among the most extensively studied of the human biorhythms (Waterhouse & DeCoursey, 2004b) with the 24-h circadian rhythms represented most prominently in the research literature. This is because these rhythms impact daily life (e.g., work, school, medical– psychological status) and are of relatively brief duration, so they are amenable to study (Clark, 2005). There is also a developmental progression in how rhythmicity is expressed from birth to old age (Waterhouse & DeCoursey, 2004b). There are a number of biological rhythms characterized by different time periods (Table 1).

Endogenous biological clocks were demonstrated over 200 years ago (Kolb & Wishaw, 2005) and entrainment was identified as the most important property for determining the phase relationship of a circadian clock (Johnson, Elliot & Foster, 2004). Circadian rhythms may confer an adaptive value on an organism, since the organism can anticipate environmental changes (Clark, 2005), and physiological functions synchronized with the time of day are associated with enhanced efficiency (Quigg, 2006). Disruption in circadian rhythms can adversely impact individuals involved in shift and night work, who suffer jet lag or whose schedules are temporally irregular. The impact is more profound when task demands involve vigilance and reaction time (Costa, 1999) and are, therefore, more vulnerable to the effects of fatigue. Attentional regulation over both incoming information and outgoing responses may also be vulnerable to time of day effects. Disturbances in sleep–wake cycles and biological rhythms have been associated with affective disorders including depression.

Mechanisms Circadian rhythms are modulated by both internal clocks (e.g., pacemakers) and external triggers that can entrain biorhythms, acting as zeitgebers (e.g., changes in illumination). The superchiasmic nucleus (SCN) of the hypothalamus has been identified as the principal biological clock (Carlson, 1999) based on lesion studies and day– night changes in activity levels. The mechanisms mediating communication and synchronization between neurons appear to be chemical in nature. Both the SCN and the pineal gland exert an influence on seasonal rhythms with SCN-induced melatonin secretion involved in synchronizing circadian rhythms (Carlson, 1999).

Circadian Rhythms. Table 1 Biological rhythms Biological rhythms

Time period

Activity

Infradian

Period of less than 24 h

Eating behaviors

Circadian

Period of about 24 h

Sleep–wake

Ultradian

Period of about 28 days

Human female menstrual cycle

Circannual

Yearly

Migratory birds

Evaluation There are large differences in circadian cycles between younger and older adults. Clinicians should assess the quantitative and qualitative nature of cognitive decline in the elderly with attention to circadian influences (with the elderly tending to be morning oriented) (Hasher, Goldstein & May, 2005). In evaluating clients for possible disruptions in circadian rhythms, clinicians would be well advised to determine if clients engage in shift work, have experienced jet lag or changes in schedule, or exhibit fatigue, sleep disorders, excessive daytime drowsiness, or decrements in job-related performance (Costa, 1999).

Treatment While the circadian clock of healthy humans is entrained to a period of about 24 h, the precise timing varies on an

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Circle of Friends

intraindividual and interindividual basis. At a certain point, these deviations may be considered clinical abnormalities (i.e., seasonal affective disorder). In fact, seasonal affective disorder has been treated using phototherapy, which involves the use of light boxes, whereby a patient is exposed to light each winter morning or evening and the circadian rhythm becomes entrained in ways that induce circadian phase shifts (Kolb & Wishaw, 2005). Individuals who make substantial changes to their sleep– wake cycles during night work or after trans-meridian travel could be helped using a chronobiological treatment. The treatment would involve exposure to appropriate circadian synchronizers and basing therapy on phase response curves for light and melatonin (Waterhouse & DeCoursey, 2004a). By making accommodations in the timing of work-related activities (Hasher et al., 2005), performance efficiency can be improved and fatigue decreased, thus enhancing health and safety (Costa, 1999). And in treating patients with bipolar disorder, psychosocial therapies should identify areas of vulnerability including unhealthy circadian rhythms (Newman, 2006).

Cross References ▶ Fatigue ▶ Sleep Disturbance

References and Readings Carlson, N. R. (1999). Foundations of Physiological Psychology (4th edn.). Boston: Allyn & Bacon. Clark, A. V. (Ed.). (2005). Causes, role and influence of mood states. Hauppauge, NY: Nova Biomedical Books. Costa, G. (1999). Fatigue and biological rhythms. In D. J. Garland, J. A. Wise, & D. V. Hopkin (Eds.), Handbook of aviation human factors (pp. 235–255). Mahwah, NJ: Lawrence Erlbaum. Hasher, L., Goldstein, D., & May, C. P. (2005). It’s about time: Circadian rhythms, memory, and aging. In C. & Izawa N. Ohta (Eds.), Human learning and memory: Advances in theory and application: The 4th Tsukuba international conference on memory (pp. 199–217). Mahwah, NJ: Lawrence Erlbaum. Johnson, C. H., Elliot, J., & Foster, R. (2004). Fundamental properties of circadian rhythms. In J. C. Dunlap, J. J. Loros, & P. J. DeCoursey (Eds.), Chronobiology: Biological timekeeping (pp. 67–105). Sunderland, MA: Sinauer Associates. Kolb, B., & Wishaw, I. Q. (2005). An introduction to brain and behavior. New York: W. H. Freeman. Lee-Chiong, T. L. (2005). Sleep: A comprehensive handbook. Hoboken, NJ: Wiley. Newman, C. F. (2006). Bipolar disorder. In F. Andrasik (Ed.), Comprehensive handbook of personality and psychopathology: Vol. 2: Adult psychopathology (pp. 244–261). Hoboken, NJ: Wiley.

Quigg, M. (2006). Circadian rhythms: Problems with the body clock. In N. F. Watson & B. V. Vaughn (Eds.), Clinicians guide to sleep disorders (pp. 277–304). Philadelphia: Taylor & Francis. Waterhouse, J. M., & DeCoursey, P. J. (2004a). Human circadian organization. In J. C. Dunlap, J. J. Loros, & P. J. DeCoursey (Eds.), Chronobiology: Biological timekeeping (pp. 291–323). Sunderland, MA: Sinauer. Waterhouse, J. M., & DeCoursey, P. J. (2004b). The relevance of circadian rhythms for human welfare. In J. C. Dunlap, J. J. Loros, & P. J. DeCoursey (Eds.), Chronobiology: Biological timekeeping (pp. 325–356). Sunderland, MA: Sinauer.

Circle of Friends ▶ Circles of Support

Circle of Willis E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition The circle of Willis is the anatomical name given to the formation of arteries at the base of the brain that contribute the overwhelming majority of blood supply to the brain.

Current Knowledge The circle of Willis is formed by the connections between the predominantly horizontal branches that derive from the middle cerebral arteries anteriorly and from the basilar artery posteriorly. The right and left middle cerebral arteries each give off an anterior cerebral artery (forming the anterolateral borders of the circle of Willis), which goes forward to supply blood to the frontal lobe. These anterior cerebral arteries are connected to each other by the anterior communicating artery, which forms the front of the circle. Posteriorly, the basilar artery bifurcates into the right and left posterior cerebral arteries, which supply the occipital and posterior temporal lobes and the cerebellum, forming the posterior

Circles of Support

border of the circle. Each posterior cerebral artery is connected to the middle cerebral artery on its corresponding side by a posterior communicating artery, forming the posterolateral borders of the circle. There are several clinical implications of the circle pattern of these arteries. Perhaps most importantly, because of the interconnectedness of the arteries that result from this circle format, if one of the main arteries is occluded, the distal smaller arteries that it supplies can potentially receive blood from the other arteries that make up the circle, a phenomenon known as collateral circulation. This helps to prevent cerebral ischemia and stroke. The circle of Willis also is a common site for cerebral aneurysms, with the greatest numbers involving the anterior communicating artery, posterior communicating arteries, and middle cerebral arteries.

Cross References ▶ Anterior Cerebral Artery ▶ Anterior Communicating Artery ▶ Basilar Artery ▶ Internal Carotid Artery ▶ Middle Cerebral Artery ▶ Posterior Cerebral Artery ▶ Posterior Communicating Artery ▶ Vertebrobasilar System

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personal goals. It is one of many tools addressing life planning from a functional or strategic assessment approach known as person-centered planning. Personcentered planning replaces more traditional assessment approaches associated with the medical model of services. Circles of support originated in Canada and have experienced widespread use in North America. Circles view people as individuals and assist them to attain selfdetermination focusing upon empowerment and not dependence of the individual. It is capacity oriented and identifies strengths, preferences, likes, and dislikes of the individual. The circle will also identify support needs in order to achieve a particular goal. The focus person leads the process and decides who will participate in the circle and the direction which the planning will take. Typically, a facilitator is selected from within the circle to help energize the group. The first circle is the circle of intimacy and includes the people most intimate in the focus person’s life. The second circle, the circle of friendship, includes good friends and close relatives. The third circle, the circle of participation includes the people and organizations the focus person is involved with. The fourth circle is the circle of exchange and includes those that are paid to be in the focus person’s life. Members are not paid to be involved in a circle of support, but are involved in the focus person’s life in some capacity. Members use their skills, knowledge, and networks to help the focus person accomplish goals. The circle develops and monitors the plan, making sure that it is current with the wishes of the focus person. It is as ongoing process that is dynamic.

Circles of Support A MY J. A RMSTRONG Virginia Commonwealth University Richmond, VA, USA

Cross References ▶ Inclusion ▶ Medical Model

Synonyms Circle of friends

Definition A circle of support is a group of people that forms a community around a specific individual (focus person) with significant disabilities to assist him or her to achieve

References and Readings Falvey, M. A., Forest, M., Pearpoint, J., & Rosenberg, R. (1994). All my life’s a circle. Using the tools: Circles, MAP’s and PATH. Toronto, Canada: Inclusion Press. O’Brien, C. L., & O’Brien, J. (2000). The origins of person-centered planning: A community of practice perspective. Retrieved from http://thechp.syr.edu/PCP_History.pdf O’Brien, J., & Lyle O’Brien, C., (Eds.). (1998). A little book about person centered planning. Toronto: Inclusion Press.

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Circumduction

Circumduction T HESLEE J OY D E P IERO Boston University School of Medicine Braintree, MA, USA

Synonyms Spastic gait

Definition Circumduction describes the movement of the leg of a person with hemiplegia, hemiparesis or paraplegia, paraparesis. Lesions of the pyramidal tract cause more weakness in the flexors of the leg than the extensors (hip and knee flexors and ankle dorsiflexors). Because of this weakness, the foot cannot be raised from the floor, and the leg cannot be advanced in a straight line forward, as it would do normally. The leg moves away from the trunk, then it is advanced toward it during walking, in a circular pattern. The medial side of the shoe scrapes the floor, causing excessive wear in that area.

are often used by persons with aphasia when having difficulty recalling or retrieving a word. In place of the target word, a description of the word is used. Circumlocutions, or ‘‘substitutions of object description (e.g., snow/ soft, white/cold) and instrumental function (e.g., watch/ knowing the hour) can be observed in aphasic output’’ (Benson & Ardila, 1996; p. 53). They occur frequently with a posterior (sensory) aphasia. Circumlocutions can represent a positive symptom of anomia in which, upon failure to retrieve a word, the subject talks around the word by defining it, describing a referent, or even making sound effects. Pointing to his wrist, a patient might say, ‘‘I wear it right here, and I tell time with it; mine goes tick, tick.’’ The use of circumlocutions ‘‘is indicative of intact semantic activation and a general capacity to retrieve lexical forms’’ (Davis, p. 109).

Cross References ▶ Anomia ▶ Aphasia

References and Readings Cross References ▶ Hemiparesis ▶ Hemiplegia ▶ Pyramidal Tract

References and Readings Victor, M., & Ropper, A. H. (2001). Adams and victor’s principals of neurology (7th ed.). New York: McGraw-Hill.

Circumlocution C AROLE R. R OTH Naval Medical Center San Diego, CA, USA

Definition The use of an unnecessarily large number of words to express an idea. Evasion in speech. Circumlocutions

Benson, D. F., & Ardila, A. (1996). Aphasia: A clinical perspective. Chapter 4, Linguistic analysis of aphasia, 46–60. New York: Oxford University Press. Davis, G. A. (2000). Aphasiology: Disorders and clinical practice. Chap. 1, Introduction to acquired language disorders, 1–19 (p. 7); Chap. 5, Investigating symptoms and syndromes, (92–119). Needham Heights, MA: Allyn & Bacon.

Circumstantiality R OBERT F RANK Kent State University Kent, OH, USA

Definition Circumstantiality is circuitous thinking and speech that digresses from the essential point. It differs from tangentiality in which the individual ultimately fails to address the main idea. In circumsatantiality, the main point is never lost but may be ‘‘clouded’’ and its appearance

Civil Law

delayed by excess and repeated material. Circumstantial thinking is a characteristic of thought disorders.

Cross References ▶ Tangentiality

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Off Label Use Premenstrual dysphoric disorder, obsessive compulsive disorder, panic disorder, generalized anxiety disorder, posttraumatic stress disorder, and social anxiety disorder.

Side Effects Serious

Circumventricular Organ ▶ Subfornical Organ

Citalopram J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Seizures, mania, and suicidal ideation (all rare).

Common Sexual dysfunction, gastrointestinal upset, insomnia, sedation, tremor, headache, dizziness, sweating, bruising and very rare bleeding, rare hyponatremia, and a potential for SIADH (syndrome of inappropriate antidiuretic hormone secretion).

References and Readings Generic Name Citalopram

Physicians’ Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson PDR. Stahl, S. M. (2007). Essential psychopharmacology: The prescriber’s guide (2nd ed.). New York, NY: Cambridge University Press.

Brand Name

Additional Information Celexa

Class Selective Serotonin Reuptake Inhibitor

Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Proposed Mechanism(s) of Action Citalopram blocks the presynaptic serotonin reuptake pump and desensitizes serotonin receptors, theoretically increases serotonin neurotransmission, and is a mild antihistamine.

Indication Depression.

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Civil Action ▶ Litigation

Civil Law ▶ Personal Injury

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Civil Litigation

Civil Litigation M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

Definition Civil law is a division of the law that deals primarily with disputes between individuals and/or organizations, in which some form of compensation may be awarded to the victim. Typically, civil law involves filing of a lawsuit by a private party, called ‘‘the plaintiff.’’ Civil litigation commonly involves hearing related to disputes regarding torts, contracts, probate of wills, trusts, property, administrative law, commercial law as well as a plethora of other matters related to private parties and organizations. Civil litigation primarily aims to correct an injustice, uphold an agreement, or settle a dispute. If compensation is awarded to the victim, then the person/organization responsible for the injustice is responsible for covering the compensation. In civil litigation, the burden of proof is usually placed on the plaintiff, though exceptions do exist. Punishment related to civil litigation is typically limited to reimbursement for loses incurred by the plaintiff as a result of actions/inactions committed by the defendant. Incarceration is not a punishment rendered via civil litigation. Civil litigation and criminal litigation are not mutually exclusive entities. For example, an individual involved in a criminal case may seek compensation in civil court. For neuropsychologists, civil litigation typically involves determination of causation, damages, and disability in disputes in which neuropsychological functioning is of relevance (e.g., personal injury, medical malpractice).

References and Readings Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Psychology Press. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney-neuropsychologists relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts (3rd ed.). New York: Guilford Press.

CJD ▶ Creutzfeldt-Jakob Disease

Classical Model of Aphasia ▶ Wernicke–Lichtheim Model of Aphasia

Classical Test Theory M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Classical test theory is the body of concepts and methods that have formed the basis for psychological assessment. Classical test theory posits that observed scores are the additive function of true scores and error terms. True scores are the ideal value of a construct in a particular person or situation. The error term is the effect of factors extraneous to the construct of interest. Error terms are assumed to be independent of (or uncorrelated with) the true scores. Analysis of the reliability of a score can be accomplished by manipulating factors thought to be influencing the error term. For example, in order to examine the effect of factors related to time or instance of measurement, a test might be administered to the same individuals on two different occasions. The relation between the two observed scores, determined by calculating a correlation coefficient or by performing an analysis of variance, helps to estimate the magnitude of the error term and the proportion of the observed score that is likely to be true score.

Cross References ▶ Item Response Theory ▶ Reliability ▶ Sources of Error

Clinical Dementia Rating


Cross References

Embretson, S. E. (Ed.). (2010). Measuring psychological constructs: Advances in model-based approaches. Washington, DC: American Psychological Association. Lord, F. M., & Novick, M. R. (2008). Statistical theories of mental test scores. New York: Information Age Pub Inc.

▶ Beyond Reasonable Doubt ▶ Burden of Proof ▶ Preponderance of Evidence

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Clear and Convincing Evidence

Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts: A handbook for mental health professionals and lawyers (3rd ed.). New York: Guilford Press.


Client Generated Index (CGI) Definition The burden of proof is the obligation to shift the assumed conclusion away from an oppositional opinion to one’s own position: it may only be fulfilled by evidence. Under the Latin maxim, necessitas probandi incumbit ei qui agit, the general rule is that ‘‘the necessity of proof lies with he who complains.’’ The burden of proof, therefore, usually lies with the party making the claim. The exception to this rule is when a prima facie case has been made. He who does not carry the burden of proof carries the benefit of assumption, meaning he needs no evidence to support his claim. Fulfilling the burden of proof effectively captures the benefit of assumption, passing the burden of proof off to another party. Clear and convincing evidence is a burden of proof required of a plaintiff for him to win the lawsuit. This standard is higher than mere preponderance of the evidence. Proof of fraud, for example, usually requires clear and convincing evidence. Clear and convincing evidence is the higher level of burden of persuasion and is most often employed in civil litigation. To prove something by ‘‘clear and convincing evidence,’’ the party with the burden of proof must convince the trier of fact that it is substantially more likely than not that the thing is in fact true. This is a lesser requirement than ‘‘proof beyond a reasonable doubt,’’ which requires that the trier of fact be close to certain of the truth of the matter asserted, but a stricter requirement than proof by ‘‘preponderance of the evidence,’’ which merely requires that the matter asserted seem more likely true than not.

▶ Patient Generated Index

Clinical Dementia Rating J ING E E TAN 1, E STHER S TRAUSS 1, E LISABETH M. S. S HERMAN 2 1 University of Victoria Victoria, BC, Canada 2 Alberta Children’s Hospital, University of Calgary Calgary, AB, Canada

Synonyms CDR

Description The Clinical Dementia Rating (CDR; Hughes, Berg, Danziger, Coben, & Martin, 1982) is a semi-structured, clinician-rated interview widely used to stage the progression of dementia using information provided by the patient and an informant. A global CDR score is generated to stage the severity of dementia. It is based on ratings of the patient’s functioning in six domains commonly affected in Alzheimer’s disease (AD): memory, orientation, judgment and problem solving, community affairs, home and hobbies, and personal care. The CDR rates only

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impairments due to cognitive deficits rather than to physical disability. Based solely on clinical information obtained from the patient and informant, and without reference to psychometric performance, a box score describing the level of impairment is generated for each domain. The box score ranges from 0 to 3, representing ‘‘none’’ to ‘‘severe’’ impairment. Using a scoring algorithm, one of five possible stages is then derived from the individual box scores as follows: CDR = 0 (no dementia); CDR = 0.5 (questionable dementia); CDR = 1 (mild dementia); CDR = 2 (moderate dementia); CDR = 3 (severe dementia). Although not part of the original protocol, CDR = 4 (profound) and CDR = 5 (terminal) can also be used to classify the later stages of dementia. An alternative method that generates a total score (range 0–18) from the sum of boxes (CDR-SB) has also been used for quantification purposes in longitudinal studies (e.g., Cortes et al., 2008). The CDR takes about 90 min to administer. The CDR has been used in clinical practice and multicenter clinical trials, as well as in cross-cultural dementia studies around the world. The CDR protocol is available in over 60 languages and dialects, including English, French, Spanish, Italian, Portuguese, Dutch, Czech, Bulgarian, Russian, Tagalog, Afrikaans, Greek, German, Hebrew, Indian dialects, Chinese dialects, Japanese, and Korean. These translations can be downloaded free of cost on the CDR Web site (http://alzheimer.wustl.edu/cdr/ default.htm). An online training video on the use of the CDR is available free for individual users, and takes about 8–9 h to complete. Detailed scoring algorithms, including ‘‘tie-break’’ rules, and an online scoring worksheet are available on the CDR Web site.

Historical Background The CDR was originally developed at the Washington University School of Medicine in 1979 to evaluate the progression of AD (Hughes et al., 1982). The original protocol has evolved somewhat over the years, with box descriptors updated to sharpen the distinction between severity levels within each domain, and new scoring rules added to resolve scoring ambiguity (Morris, 1993). Alternative scoring methods have also been suggested to improve scoring accuracy (Gelb & St. Laurent, 1993). An extension of this scale to include CDR = 4 (profound) and CDR = 5 (terminal) to classify the later stages of dementia (Dooneief, Marder, Tang, & Stern, 1996) among nursing home elderly has been proposed. As well, another method

that uses a total score generated from the sum of boxes (CDR-SB) has gained popularity to quantify progression of AD in longitudinal clinical trials of experimental therapies (e.g., Petersen et al., 2005).

Psychometric Data Interrater Reliability Most of the psychometric studies on the CDR have focused on interrater reliability in multicenter clinical trials. These studies have concluded that experience using the CDR increases reliability estimates, although adequately trained inexperienced raters may also demonstrate a high level of agreement (kappa = 0.83 or higher; Schafer et al., 2004; Tractenberg, Schafer, & Morris, 2001). The CDR also shows good reliability among raters of various qualifications. There were no major differences in reliability among physicians, nurses, PhDs, social workers, psychometrists, or research assistant raters (85% for non-MDs and 82% for MDs; Oremus, Perrault, Demers, & Wolfson, 2000). Kappas between physician raters range from 0.75 to 0.94 for the six individual domain scores and CDR-SB score. Kappas between nurses, or between nurses and physicians, range from 0.66 to 0.77 (Oremus et al., 2000).

Construct Validity Evidence for construct validity of the CDR appears solid. In the original study, the CDR had strong correlations with the Blessed Dementia Scale (BDS; r = 0.74) and the Pfeiffer Short Portable Mental Status Questionnaire (SPMSQ; r = 0.84) among individuals with CDR ratings between no dementia and very mild dementia (Hughes et al., 1982). Correlations with various cognitive measures ranged from small to large in community-dwelling samples (Folstein’s Mini-Mental State Examination = 0.33; BDS = 0.74; SPMSQ = 0.84). Similar correlations were found with neuropsychological measures such as the CERAD Boston Naming Test, list learning, and verbal fluency (Oremus et al., 2000). In terms of neuropathology, CDR = 0.5 (a proxy for mild cognitive impairment; MCI) has been associated with multiple pathological signs including those related to AD, Lewy body dementia, or vascular dementia, as well as nonspecific pathology (Saito & Murayama, 2007). Further, increased microglia activation, an inflammatory biomarker of AD, was seen with advancing CDR


stages (Xiang, Haroutunian, Ho, Purohit, & Pasinetti, 2006). A negative association was also found between the CDR-SB score and glucose metabolism in the right posterior cingulate gyrus (Perneczky, Hartmann, Grimmer, Drzezga, & Kurz, 2007).

Predictive Validity There is evidence that individuals with higher CDR-SB scores are more likely to develop dementia in the future (Lynch et al., 2006). In addition, CDR scores predict survival in individuals with suspected dementia. Using survival as outcome, the median survival was 1 year for CDR = 5, 2 years for CDR = 4, 2.5 years for CDR = 3, 3 years for CDR = 2, and 3.5 years for CDR = 1 (Dooneief et al., 1996). Use of the CDR as a screening tool for dementia revealed a sensitivity of 92% and specificity of 94% for mild dementia in a community sample of adults older than age 75 (Juva et al., 1995).

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populations (Lim, Chin, Lam, Lim, & Sahadevan, 2005; Senanarong, Chen, & Orgogozo, 2006), although empirical validation of various translations is needed. The CDR presents several advantages over psychometric tests. First, it is an assessment option for patients who are illiterate and/or have limited English language proficiency. Moreover, it can also be used in the presence of aphasia, a condition common among patients with dementing disorders. Lastly, the administration of the CDR does not require a standardized set of instructions, but is dependent upon a set of guidelines. As such, the CDR is easily adapted for cross-cultural use (e.g., Lim et al., 2005). A disadvantage of the CDR is that it is somewhat lengthy to administer.

Cross References ▶ Alzheimer’s Disease ▶ CERAD ▶ Dementia

Clinical Uses References and Readings The original focus of the CDR was to assess communitydwelling older adults, since its anchor points probe for examples of one’s engagement with the home and community. However, it has also been adapted for use in chronic long-term care facilities (Marin et al., 2001). The global CDR is widely used primarily for the staging of AD. It has also been used to stage other dementing disorders such as Parkinson’s disease and frontotemporal dementia. In recent years, CDR = 0.5 has been used to characterize MCI; however, there is evidence that a large proportion (29.7%) of individuals at CDR = 0.5 also meet ICD-10 criteria for mild dementia (Lynch et al., 2006). In terms of longitudinal studies of AD progression, both the global CDR and the CDR-SB appear to be useful for tracking cognitive changes over a 2–3 year period (e.g., Cortes et al., 2008; Meguro et al., 2004). Of the two scoring methods, the CDR-SB is more commonly used in clinical drug trials because it was found to be sensitive to changes within 12 months following baseline measurement in donepezil drug trials, whereas the global CDR was not (e.g., Petersen et al., 2005). There is also evidence that the CDR-SB is more useful than the global CDR in distinguishing mild cognitive deficits from dementia (Lynch et al., 2006). In addition to western populations, use of the CDR has also been accepted as an appropriate comprehensive measure for studies of dementia patients in Asian

Cortes, F., Nourhashe´mi, F., Gue´rin, O., Cantet, C., Gillette-Guyonnet, S., Andrieu, S., et al. (2008). Prognosis of Alzheimer’s disease today: A two-year prospective study in 686 patients from the REAL-FR study. Alzheimer’s and Dementia, 4(1), 22–29. Dooneief, G., Marder, K., Tang, M. X., & Stern, Y. (1996). The Clinical Dementia Rating scale: community-based validation of ‘profound’ and ‘terminal’ stages. Neurology, 46, 1746–1749. Gelb, D. J., & St. Laurent, R. T. (1993). Alternative calculation of the global clinical dementia rating. Alzheimer Disease & Associated Disorders, 7(4), 202–11. Hughes, C. P., Berg, L., Danziger, W. L., Coben, L. A., & Martin, R. L. (1982). A new clinical scale for the staging of dementia. British Journal of Psychiatry, 140, 566–572. Juva, K., Sulkava, R., Erkinjuntti, T., & Ylikoski, R. (1995). Usefulness of the Clinical Dementia Rating scale in screening for dementia. International Psychogeriatrics, 7(1), 17–24. Lim, W. S., Chin, J. J., Lam, C. K., Lim, P. P. J., & Sahadevan, S. (2005). Clinical Dementia Rating: experience of a multi-racial Asian population. Alzheimer Disease and Associated Disorders, 19(3), 135–142. Lynch, C., Walsh, C., Blanco, A., Moran, M., Coen, R., Walsh, J., et al. (2006). The clinical dementia rating sum of box score in mild dementia. Dementia and Geriatric Cognitive Disorders, 21(1), 40–43. Marin, D., Flynn, S., Mare, M., Lantz, M., Hsu, M., Laurans, M., et al. (2001). Reliability and validity of a chronic care facility adaptation of the Clinical Dementia Rating scale. International Journal of Geriatric Psychiatry, 16(8), 745–750. Meguro, K., Shimada, M., Yamaguchi, S., Sano, I., Inagaki, H., Matsushita, M., et al. (2004). Neuropsychosocial features of very mild Alzheimer’s Disease (CDR 0.5) and progression to dementia

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in a community: The Tajiri Project. Journal of Geriatric Psychiatry and Neurology, 17(4), 183–189. Morris, J. C. (1993). The clinical dementia rating (CDR): Current version and scoring rules. Neurology, 43(11), 2412–2414. Oremus, M., Perrault, A., Demers, L., & Wolfson, C. (2000). A review of outcome measurement instruments in Alzheimer’s disease drug trials: Psychometric properties of global scales. Journal of Geriatric Psychiatry and Neurology, 13(4), 197–205. Perneczky, R., Hartmann, J., Grimmer, T., Drzezga, A., & Kurz, A. (2007). Cerebral metabolic correlates of the clinical dementia rating scale in mild cognitive impairment. Journal Of Geriatric Psychiatry And Neurology, 20(2), 84–88. Petersen, R. C., Thomas, R. G., Grundman, M., Bennett, D., Doody, R., Ferris, S., et al. (2005). Vitamin E and Donepezil for the treatment of Mild Cognitive Impairment. New England Journal of Medicine, 352(23), 2379–2388. Saito, Y., & Murayama, S. (2007). Neuropathology of mild cognitive impairment. Neuropathology, 27(6), 578–584. Schafer, K., Tractenberg, R., Sano, M., Mackell, J., Thomas, R., Gamst, A., et al. (2004). Reliability of monitoring the clinical dementia rating in multicenter clinical trials. Alzheimer Disease & Associated Disorders, 18(4), 219–222. Senanarong, V., Chen, C., & Orgogozo, J. (2006). Third AsiaPacific Regional Meeting of the International Working Group on Harmonization of Dementia Drug Guidelines: Meeting Report Summary. Alzheimer Disease & Associated Disorders, 20(4), 311–312. Tractenberg, R., Schafer, K., & Morris, J. (2001). Interobserver disagreements on clinical dementia rating assessment: Interpretation and implications for training. Alzheimer Disease & Associated Disorders, 15(3), 155–161. Xiang, Z., Haroutunian, V., Ho, L., Purohit, D., & Pasinetti, G. M. (2006). Microglia activation in the brain as inflammatory biomarker of Alzheimer’s disease neuropathology and clinical dementia. Disease Markers, 22(1–2), 95–102.

Clinical Depression ▶ Major Depression

Clinical Interview K IMBERLY A. G ORGENS University of Denver Denver, CO, USA

Synonyms Diagnostic interview; Intake; Intake interview; Unstructured clinical interview

Definition A skillfully conducted clinical interview is the cornerstone of psychological assessment. This interaction, typically a face-to-face meeting that lasts between 30 min and 2 h, generates a tremendous amount of data for the clinician via both observation and direct questioning. Information obtained through observation during the clinical interview is considered qualitative or descriptive, and can include impressions about cognition, attention, orientation, language, sensorimotor deficits, affect, insight, attitude toward assessment, acculturation, hygiene, interpersonal relations, and coping mechanisms, among other variables. In addition, the verbal exchange between patient and clinician yields information about current life circumstances, including the reason for referral and history of the presenting problem, as well as an account of developmental/medical/ family history, educational and occupational achievement, legal problems, sociocultural/religious considerations, substance abuse, and other relevant psychiatric issues. Together with test data and collateral information, interview material is an invaluable tool for clinical hypothesis formulation and testing, as well as treatment planning.

Current Knowledge

Clinical Extender ▶ Psychometrician

Clinical Importance ▶ Clinical Significance

There are three types of clinical interview, reflecting the degree to which the content and questions are scripted: structured, semi-structured, and unstructured. A structured interview (e.g., the Structured Clinical Interview for DSM-IV-Clinical Version [SCID-I]; First, Gibbon, Spitzer, & Williams, 2001), like any standardized assessment tool, gathers specific data that allows clinicians to make comparisons between client and normative group function. Criticisms of structured clinical interviews include a frustration with lengthy questionnaires and the missed

Clinical Neuropsychology

opportunity for meaningful dialogue (Maruish, 2008). An unstructured clinical interview, on the other hand, is principally reliant on clinical skill for direction. An optimal unstructured clinical interview involves moving from broad content areas to more specific ones, from openended to more directive questions seeking yes/no responses. While the goals of clinical interviewing remain much the same regardless of format, critics have argued that bias is more easily introduced into unstructured clinical interviews than standardized approaches. A hybrid, the semi-structured clinical interview, offers many of the benefits of its structured and unstructured counterparts, with breadth and depth chief among them. The semi-structured format ensures that all areas of potential clinical concern are assessed, while affording the clinician the flexibility to dictate the degree of attention each content area receives. In addition, the semi-structured clinical interview can be altered to accommodate disabilities, it can be abbreviated to meet the needs of the client (e.g., fatigue) or the setting (e.g., bedside assessment), and it can be amended to include additional lines of inquiry. As well as yielding information regarding patient history and current functioning, a clinical interview offers opportunities to build rapport and foster a working alliance. In addition to promoting satisfaction with the assessment process, the development of rapport may promote compliance from a reluctant patient and provide a foundation for follow-up discussions and interventions that may be indicated. Also, the dialogue during a clinical interview allows a clinician to provide important patient education. The initial conversation may explicitly address confidentiality, insurance/fee setting, the nature and purpose of the examination, the intended use of assessment data, and a summary of follow-up plans including feedback sessions (Lezak, Howieson, & Lorig, 2004). Despite routine use in clinical practice, there is considerable debate about the reliability of clinical interviews. Many believe the variability in questioning undermines the utility of the tool itself. Some research has suggested that unstructured clinical interviews often fail to detect psychiatric and comorbid conditions (Zimmerman & Mattia, 1999). Other researchers find ‘‘empirical rigor’’ in skillful clinical interviewing (Shedler, 2002, p. 429). This argument notwithstanding, the clinical interview remains a staple assessment tool, as fallible or effectual as the clinician.

Cross References ▶ Behavioral Assessment ▶ Mental Status Examination

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▶ Referral Question ▶ Self-Report Measures ▶ Structured Clinical Interview for DSM-IV (SCID-I) ▶ Structured Interview of Reported Symptoms (SIRS)

C References and Readings First, M., Gibbon, M., Spitzer, R. L., & Williams, J. B. W. (2001). User’s guide for the Structured Clinical Interview for DSM-IV Axis I disorders. New York: Biometrics Research. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment, (4th ed.). New York, NY: Oxford University Press. Maruish, M. M. (2008). The clinical interview. In R. P. Archer & S. R. Smith (Eds.), Personality assessment. New York: Routledge. Rogers, R. (2001). Diagnostic and structured interviewing. A handbook for psychologists. New York: Guilford Press. Sbordone, R. J. (2000). The assessment interview in clinical neuropsychology. In G. Groth-Marnat (Ed.), Neuropsychological assessment in clinical practice (pp. 94–126). New York: Wiley. Shedler, J. (2002). A new language for psychoanalytic diagnosis. Journal of the American Psychoanalytic Association, 50, 429–456. Zimmerman, M. (1994). Interview Guide for Evaluating DSM-IV Psychiatric Disorders and the Mental Status Examination. East Greenwich, RI: Psych Products Press. Zimmerman, M., & Mattia, J. I. (1999). Psychiatric diagnosis in clinical practice: Is comorbidity being missed? Comprehensive Psychiatry, 40(3), 182–191.

Clinical Neuropsychology A NTHONY Y. S TRINGER Emory University Atlanta, GA, USA

Definition Clinical neuropsychology is a specialty within psychology that applies the science of brain-behavior relations to the assessment, diagnosis, treatment, and rehabilitation of patients across the life span with neurological, medical, neurodevelopmental, psychiatric, or other cognitive and learning disorders (Barth et al., 2003). The American Psychological Association (APA) defines a clinical neuropsychologist as ‘‘a professional psychologist who applies principles of assessment and intervention based upon the scientific study of human behavior as it relates to normal and abnormal functioning of the nervous system’’ (APA, 1989, pp. 22).

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Clinical neuropsychology is recognized as a specialty by APA, the American Board of Professional Psychology, and the Canadian Psychological Association. Though there is no agreed upon date for the emergence of the field, clinical neuropsychology began to be recognized as a distinct professional discipline following a 1948 symposium at the APA annual meeting appropriately entitled ‘‘Neuropsychology.’’ In this symposium, Hans-Lukas Teuber described procedures he and Morris Bender developed to study the behavioral effects of penetrating missile wounds to the brain (Benton, 1987). Clinical neuropsychology had many forerunners. In the 1880s, Sir Francis Galton opened a laboratory in London where for a few pennies, people could take tests of visual acuity, reaction time, and various psychophysical abilities. A cousin of Charles Darwin, Galton’s ideas about eugenics have made him a historically controversial figure. Nonetheless, factor analytic statistical methods grew out of his work and became critical for the development and validation of mental ability tests. His work was put to practical use in early twentieth-century France when psychologists Alfred Binet and Theódore Simon developed intellectual tests to identify and place children in need of special education. World War I also stimulated interest in his work in the USA, as the military sought tests that could efficiently identify the strengths and weaknesses of large numbers of military recruits. As many as one million soldiers underwent mental ability testing during World War I (Hartman, 1991). Just after World War I, the German American psychologist Shepherd Ivory Franz (1919) published detailed descriptions of tests of tactile sensation, motor coordination, praxis, language, attention, memory, visuospatial perception, reasoning, and intelligence. By 1924, hand dynamometers, finger tapping keys, motor steadiness tests, form perception boards, and tests of color vision, vibration sense, attention, and memory were available (Hartman, 1991). Though these tests were quickly adopted by researchers (see Neuropsychology, Science of), clinical application came a few decades later. Franz advocated for their clinical use in a series of lectures to the Government Hospital for the Insane, starting in 1910 (Hartman, 1991) and in 1904 was instrumental in establishing a psychological laboratory at Mclean Hospital in Boston. This was the first such laboratory in a hospital setting in the USA In 1935, the experimental psychologist Ward Halstead opened a laboratory at the University of Chicago for the psychological study of neurology and neurosurgery patients. Adopting techniques used to test animals in ablation studies, Halstead designed a series of tests

intended to measure what he termed ‘‘biological intelligence.’’ The influence of Galton’s statistical methods is seen in Halstead’s factor analysis of his test battery. Halstead proposed a four-factor theory of biological intelligence in his 1947 book Brain and Intelligence. Halstead’s book was severely criticized by contemporary scholars, and ultimately had little influence on theories of intelligence (Hartman, 1991). Halstead’s test battery, however, did prove highly influential. Ralph Reitan, a student of Halstead, established a laboratory at the University of Indiana Medical Center in 1950. Over the course of several decades, Reitan expanded upon Halstead’s initial battery, adding procedures for detecting aphasia and sensory perceptual impairments and for comparing performances of the two sides of the body. Reitan developed adaptations of the original adult battery for children and adolescents, collected normative data, and used discriminant function analysis to validate the ability of the various batteries to discriminate brain damaged from neurologically healthy individuals. The Halstead–Reitan Neuropsychological Test Battery set a standard of excellence for test development in neuropsychology and for a time was the most widely used approach in clinical neuropsychological assessment. Reitan’s students were also prolific (Reed, 1985). They include Hallgrim Kløve who studied with Reitan in the 1950s and then established a laboratory at the University of Wisconsin. Another student, Homer Reed, directed the Neuropsychology Laboratory at the New England Medical Center in Boston where he concentrated on use of the battery with pediatric patients. Phillip Rennick, who did fellowship training with Reitan, went on to establish a laboratory at the former (now defunct) Lafayette Clinic in Detroit, and developed a repeatable neuropsychological battery for situations in which serial testing is needed. Just as World War I provided an impetus for the development of psychological ability tests, World War II stimulated the development of neuropsychological assessment and treatment methods because of the large number of veterans who returned having survived penetrating missile wounds to the brain. Reitan’s early work involved the examination of brain-injured World War II veterans. This population also provided the impetus for HansLukas Teuber’s work in the USA and Aleksandr Romanovich Luria’s work in Russia. Teuber immigrated from Germany and served as a noncommissioned naval officer before establishing the Psychophysiological Laboratory with the neurologist Morris Bender at New York University (Weinstein, 1985). Besides being the focal point for numerous seminal studies, Teuber’s laboratory was the incubator for many


neuropsychologists who went on to make important contributions including Joseph Altman, Lila Ghent, Rita Rudel, Josephine Semmes, and Sidney Weinstein. Meanwhile, working with Russian World War II veterans at the Burdenko Neurosurgical Institute in Moscow, Luria developed a richly qualitative approach to neuropsychological assessment that was in stark contrast to the quantitative and normatively based test batteries that were in use in the USA Luria also was an exponent of neuropsychological approaches to rehabilitation and to use of pharmacologic agents to enhance recovery of function (Gualtieri, 1988). Besides having cognitive disorders, many injured World War II veterans were aphasic, sparking neuropsychological interest in language and the brain. Kurt Goldstein’s (▶ Goldstein, Kurt) book Language and Language Disorders, published in 1948, combined his theories about abstracting ability with neurology’s classic anatomico-clinical syndrome approach to aphasia. The numbers of aphasic veterans available for study along with Goldstein’s influential book attracted experimental neuropsychologists and psycholinguists to the study of language disorders (Goodglass, 1985). Aphasia research centers began to appear in the USA, among them being a laboratory established by Arthur Benton. In 1948, the year of Teuber’s seminal APA presentation, Arthur Benton accepted an appointment as Professor of Psychology at the University of Iowa, and by 1950 established a neuropsychological testing unit at the University of Iowa Hospitals (Hamsher, 1985). There, Benton and his students and colleagues conducted normative studies, examining the effects of age, gender, and education. They also developed a variety of tests for use in studying what had previously been vaguely defined clinical disorders such as the Gerstmann syndrome (▶ Gerstmann Syndrome). Over the next 2 decades, Benton’s laboratory was home to numerous neuropsychology pioneers including Max Fogel, Donald Shankweiler, Kerry deS. Hamsher, Nils Varney, Scott Lindgren, Otfried Spreen, and Harvey Levin (Hamsher, 1985). Benton’s laboratory conducted important studies of aphasia using control group designs, psychological test construction methods, statistical analysis, and psycholinguistic theory. The behavioral neurologist Norman Geschwind, along with his colleagues Davis Howes and Harold Goodglass, established an aphasiology center at the Boston Veterans Administration Hospital, continuing the more rigorous neuropsychological approach to language disorders. The Boston VA became a major center for neuropsychological training and research with Dr. Edith Kaplan famously serving there as ‘‘mother’’ to a generation of

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neuropsychology practitioners and researchers. Dr. Kaplan and her associates in Boston developed the process-approach to neuropsychological assessment, recognizing that tests are complex and multifactorial and that patients can take different paths to the same test score. According to this approach, only by systematically analyzing the process(es) by which patients arrive at their responses, often by parsing a test into fine-grained components, can a neuropsychologist truly understand what aspect of brain functioning is compromised. Activity was not confined to North America. Clinical neuropsychology got its South American start when C. Mendilaharsu and S. Acevedo de Mendilaharsu established the first South American neuropsychological laboratory in 1958 at the Neurological Institute in Montevideo, Uruguay (Ardila, 1990). Despite sometimes challenging economic conditions, South American neuropsychologists conducted investigations of constructional ability, dementia, and language. The field spread to Mexico, Peru, Columbia, Chile, Argentina, Brazil, Honduras, Nicaragua, and elsewhere. Clinical neuropsychology as a profession has become increasingly organized since its inception. The International Neuropsychological Society (INS), the National Academy of Neuropsychology (NAN), and the APA Division of Clinical Neuropsychology (Division 40) are the most well-known professional neuropsychological organizations within the USA. Founded in 1967, INS has a membership that exceeds 3,500. NAN, formed in 1974, includes more than 3,000 members. Division 40 of the American Psychological Association, incorporated in 1980, is the most recent of these professional organizations, with a membership of over 4,000 neuropsychologists. Although smaller in membership, neuropsychologists have formed similar professional organizations throughout Europe, Asia, South America, Australia, and Africa (see, for example, Jodzio, 1998; Nihashi, 1998; Preilowski, 1997). Formal courses and organized programs of instruction in clinical neuropsychology began to appear in the 1960s, with one of the first offered in the Biological Psychology Doctoral Program at the University of Oklahoma Health Sciences Center (Parsons, 1991). Quality and content of instruction varied from program to program as they multiplied in the 1970s. In 1979, Dr. Manfred Meier initiated an effort to establish standards of training and competence for neuropsychologists in the USA. This effort culminated first in the establishment of the American Board of Clinical Neuropsychology (ABCN) in 1981. A year later, the American Board of Professional Neuropsychology (ABN) was established

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with a similar goal of establishing standards of professional expertise in neuropsychology. These two certification bodies continue to function autonomously, though ABCN affiliated with the American Board of Professional Psychology (ABPP) in 1984, joining other boards that conduct peer review of the credentials, knowledge, and practice of clinical psychologists seeking subspecialty certification in the USA. In 1987, APA Division 40 published guidelines for doctoral, internship, and postdoctoral training in neuropsychology and a year later formally adopted the definition of a clinical neuropsychologist quoted above. Included in this definition (but not quoted) were explicit training, supervision, licensing, and peer review requirements that must be met by neuropsychologists. APA also recognized attainment of the ABCN/ABPP diploma as ‘‘the clearest evidence of competence as a Clinical Neuropsychologist’’ (APA Division 40, 1989). As of 2010, over 500 practicing clinicians have attained ABPP/ABCN diplomate status in neuropsychology and over 350 neuropsychologists had attained ABN certification. Though ABCN and ABN initially differed in their examination procedures, currently both boards require a peer review of credentials, a multiple-choice written exam, and a 1 h ethics oral exam. The two boards differ, however, in other aspects of their oral examination. In particular, ABCN, but not ABN, conducts an oral ‘‘factfinding’’ examination in which candidates solicit information about a neuropsychological patient in order to arrive at a diagnostic formulation and clinical recommendations. Nonetheless, the two boards have grown more similar since their inception, and have discussed merger, but these efforts have been unsuccessful. One milestone likely to occur in the USA in the twenty-first century is the achievement of widespread ABCN or ABN diplomacy among those identifying neuropsychology as their primary area of specialization. This milestone would signal the professional and organizational maturity of clinical neuropsychology.

Cross References ▶ American Board of Clinical Neuropsychology (ABCN) ▶ American Board of Professional Neuropsychology ▶ American Psychological Association (APA), Division 40 ▶ Benton, Arthur (1909–2006) ▶ Forensic Neuropsychologist ▶ Forensic Neuropsychology ▶ Geschwind, Norman (1926–1984) ▶ Halstead, Ward (1908–1968)

▶ International Neuropsychological Society ▶ Luria, Alexander Romanovich (1902–1977) ▶ National Academy of Neuropsychology ▶ Neuropsychiatry ▶ Neuropsychology, Science of ▶ Reitan, Ralph (1922– ) ▶ Teuber, Hans-Lukas (1916–1977)

References and Readings American Psychological Association Division 40 (1989). Definition of a clinical neuropsychologist. The Clinical Neuropsychologist, 3, 22. Ardila, A. (1990). Neuropsychology in Latin America. The Clinical Neuropsychologist, 4, 121–132. Barth, J. T., Pliskin, N., Axelrod, B., Faust, D., Fisher, J., Harley, J. P., et al. (2003). Introduction to the NAN 2001 definition of a clinical neuropsychologist. Archives of Clinical Neuropsychology, 18, 551–555. Benton, A. (1987). Evolution of a clinical specialty. In K. M. Adams, & B. P. Rourke (Eds.), The TCN guide to professional practice in clinical neuropsychology (pp. 1–4). Amsterdam: Swets & Zeitlinger. Goldstein, K. (1948). Language and language disorders. Orlando, FL: Grune & Stratton. Goodglass, H. (1985). Aphasiology in the United States. International Journal of Neuroscience, 25, 307–311. Groth-Marnat, G. (2000). Introduction to neuropsychological assessment. In G. Groth-Marnat (Ed.), Neuropsychological assessment in clinical practice: A guide to test interpretation and integration (pp. 3–25). New York, NY: Wiley. Gualtieri, C. T. (1988). Pharmacotherapy and the neurobehavioural sequelae of traumatic brain injury. Brain Injury, 2, 101–129. Hamsher, K. D. (1985). The Iowa Group. International Journal of Neuroscience, 25, 295–305. Hartman, D. E. (1991). Reply to Reitan: Unexamined premises and the evolution of clinical neuropsychology. Archives of Clinical Neuropsychology, 6, 147–165. Jodzio, K. (1998, Spring). Neuropsychology in Poland: Past and present. International Neuropsychological Society Liaison Committee Newsletter, 5, 1–3. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). The practice of neuropsychological assessment. In Neuropsychological assessment (4th ed., pp. 3–14). New York, NY: Oxford University Press. Nihashi, N. (1998, Spring). Neuropsychology in Japan. International Neuropsychological Society Liaison Committee Newsletter, 5, 1–3. Parsons, O. A. (1991). Clinical neuropsychology 1970–1990: A personal view. Archives of Clinical Neuropsychology, 6, 105–111. Preilowski, B. (1997). Establishing clinical neuropsychology in Germany: Scientific, professional, and legal issues. Neuropsychology Review, 7, 187–199. Reed, J. (1985). The contributions of Ward Halstead, Ralph Reitan and their associates. International Journal of Neuroscience, 25, 289–291. Stringer, A. Y., & Cooley, E. L. (2002). Neuropsychology: A twentiethcentury science. In A. Y. Stringer, E. L. Cooley, & A.-L. Christensen (Eds.), Pathways to prominence in neuropsychology: Reflections of twentieth century pioneers (pp. 3–26). New York, NY: Psychology Press. Weinstein, S. (1985). The influence of Hans-Lukas Teuber and the psychophysiological laboratory on the establishment and development of neuropsychology. International Journal of Neuroscience, 25, 277–288.

Clinical Practice Guidelines

Clinical Practice Guidelines A NDREA M. L EE University of Manitoba Winnipeg, Manitoba, Canada

Synonyms Practice guidelines

Definition According to the Institute of Medicine’s 1990 report, Clinical Practice Guidelines: Directions for a New Program, clinical practice guidelines are ‘‘systematically developed statements to assist practitioner and patient decisions about appropriate health care for specific clinical circumstances.’’

Historical Background Recommendations for appropriate care have been found in ancient writings (IOM, 1992). Modern guidelines have been developed by professional organizations for over 50 years. It was only in the 1990s, however, that the systematic, evidence-based guidelines began to appear with any regularity. In November 1989, the Agency for Health Care Policy and Research (AHCPR) was created with, among other responsibilities, a mandate to develop, disseminate, and evaluate clinical practice guidelines. The AHCPR then enlisted the Institute of Medicine (IOM) for advice. The result was the 1990 report generated by the IOM, called Clinical Practice Guidelines: Directions for a New Program, that aimed to encourage standardization and consistency in the development of guidelines.

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guide the allocation of health care resources, and (5) reduce the risk of liability for negligent care (IOM, 1992). In addition to patients and their families, health care providers, and health care institutions, others such as payers, health care benefit providers, and public policy makers have a stake in clinical practice guidelines. Legislators, regulators, and health care purchasers take an interest in studying them to assist them in making decisions that control health care costs. It is assumed that if the most appropriate care is administered, better health outcomes will be achieved and health care costs will be lowered. Although some guidelines could likely achieve such hopes for decreased health care costs, other guidelines may not. The Agency for Healthcare Research and Quality (AHRQ), formerly known as the AHCPR, created a central public resource for evidenced-based clinical practice guidelines called the National Guideline Clearinghouse (NGC) that can be accessed at http://www. guideline.gov/ For a clinical practice guideline to be included on the NGC, the following criteria must be met: (1) The guideline must contain systematically developed recommendations, strategies, or information that assist in the decision making of appropriate health care in specific clinical situations by healthcare providers and patients; (2) the guideline must be produced by a medical specialty association, professional societies, public or private organizations, government agencies, or health care organizations or plans; (3) a documentation should be produced that verifies that a systematic literature search and review of scientific literature was performed during the guideline development; (4) the guidelines should have been reviewed or produced within 5 years and should be available in print or electronic format in the English language (National Guideline Clearinghouse, 2009). These criteria ensure minimum quality of guidelines submitted to the NGC.

Future Directions Current Knowledge The five major purposes of clinical practice guidelines are to: (1) aid in clinical decision-making by patients and health care providers, (2) educate individuals or groups, (3) assess and ensure quality of care, (4)

An ongoing challenge is the implementation of clinical practice guidelines and ensuring their application by health care providers. Factors such as information overload, habitual practice patterns, fears of malpractice, and a lack of economic incentives create barriers to guideline application and each must be addressed on an individual and systemic level.

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Cross References ▶ AACN Practice Guidelines

References and Readings IOM. (1990). In M. J. Field & K. N. Lohr (Eds.), Clinical Practice guidelines: Directions for a new program. Washington, DC: National Academy Press. IOM. (1992). In M. J. Field & K. N. Lohr (Eds.), Guidelines for clinical practice: From development to use. Washington, DC: National Academy Press. National Guideline Clearinghouse. (2009). Inclusion criteria. Retrieved October 13, 2009, from the National Guideline Clearinghouse Web site: http://www.guideline.gov/submit/inclusion.aspx

Clinical Relevance ▶ Clinical Significance

Clinical Significance M ONICA K URYLO 1, J ASON VAN A LLEN 2 1 The University of Kansas Medical Center Kansas, KS, USA 2 University of Kansas Lawrence, KS, USA

Synonyms Clinical importance; Clinical relevance

Definition Clinical significance is a perceived, valued, and functionally relevant discrepancy in symptoms/abilities that reflects an important change in functioning. This can involve either an improvement (usually as a result of treatment or intervention) or a decline (typically due progression of illness or disorder) as measured by symptoms or impairment level. The relevance and value of this to difference may be determined by the client, mental health professional, and/or the client’s significant other(s).

Clinical significance also refers to a static condition of import – for example, a functionally relevant discrepancy between cognitive abilities in different domains (e.g., language vs. visual-perceptual abilities). The term can also refer to the level of distress or impairment related to psychological symptoms as criteria for a DSM-IV TR disorder diagnosis. While clinical significance may be supported by statistically significant differences on quantitative measures of functioning, statistical significance cannot be equated with clinical significance.

Cross References ▶ Functional Assessment ▶ Premorbid Functioning ▶ Quality of Life ▶ Reliable Change Index ▶ Response to Intervention ▶ Statistical Significance

References and Readings American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (Rev. 4th ed.). Washington, DC: American Psychiatric Association. Jacobson, N. S., Roberts, L. J., Berns, S. B., & McGlinchey, J. B. (1999). Methods for defining and determining the clinical significance of treatment effects: Description, application, and alternatives. Journal of Consulting and Clinical Psychology, 67, 300–307. Jacobson, N. S., & Truax, P. (1991). Clinical significance: A statistical approach to defining meaningful change in psychotherapy research. Journal of Consulting and Clinical Psychology, 59, 12–19. Kazdin, A. E. (1999). The meanings and measurement of clinical significance. Journal of Consulting and Clinical Psychology, 67, 332–339. Kazdin, A. E. (2003). Clinical significance: Measuring whether interventions make a difference. In A. E. Kazdin (Ed.), Methodological issues and strategies in clinical research (3rd ed., pp. 691–710). Washington, DC: American Psychological Association. Spitzer, R. L., & Wakefield, J. C. (1999). DSM-IV diagnostic criterion for clinical significance: Does it help solve the false positives problem? American Journal of Psychiatry, 156, 1856–1864.

Clinical Target Volume ▶ Involved Field Radiotherapy

Clock Drawing

Clock Drawing DAVID J. L IBON 1, E DITH K APLAN 2, R OD S WENSON 3, DANA L. P ENNEY 4 1 Drexel University College of Medicine Philadelphia, PA, USA 2 Suffolk University Boston, MA, USA 3 North Dakota School of Medicine Fargo, ND, USA 4 The Lahey Clinic Burlington, MA, USA

Description Clock Drawing Test (CDT) is a widely used and popular neuropsychological test. Rubin, Barr, and Burton (2005) reported that the CDT appears in the top 40 tests most commonly used by neuropsychologists. The CDT is often considered to be a visuoconstructional test. Modern versions of the CDT usually contain at least two parts – clock drawing to command and clock drawing to copy. In the command condition, patients are presented with a blank sheet of paper and are asked to ‘‘draw the face of a clock showing the numbers and the two hands set for ten after eleven.’’ In the copy condition, a pre-drawn model of a clock with numbers and hands set for 10 after 11 is presented, and the patient is asked to copy the model. Clock drawing with hands set for ten after eleven is an innovation introduced by Edith Kaplan (Kaplan, 1988, 1990). As described below, other versions of the CDT ask the patient to set the hands for other times. Also, some versions of the CDT include a clock subtest in which patients are presented with pre-drawn clock faces with and without numbers and/or hands and are asked to either draw clock hands to designate specific times or read the specified time (Borod, Goodglass, & Kaplan, 1980; Leach, Kaplan, Reweilak, Richards, & Proulx, 2000; Tuokko, Hadjustavropoulos, Miller, & Beattie, 1992).

Historical Background Since the late 1980s, a profusion of research using the CDT as an assessment tool for dementia has emerged. However, some of the more interesting historical roots of the CDT came from research with aphasic patients. Henry Head, in his magnum opus Aphasia and Kindred

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Disorders of Speech (1926), assessed aphasic patients using a wide number of drawing tests including ‘‘the clock test’’ (p. 214) to assess deficits in understanding and executing complex propositional speech and deficits associated with symbol formation. In describing propositional speech and symbolic formation deficits associated with aphasia, Head (1926) wrote ‘‘any act of mental expression, which demands symbolic formulation, tends to be defective and the higher its propositional value the greater the difficulty it will present’’ (p. 212). Head (1926) provided many examples of aphasic patients who demonstrated striking impairment in executing the propositional command to set the clock hands for a specified time. For example, Head described an aphasic patient who was able to set clock hands ‘‘correctly at 3:40,’’ but not when he was told to place the hands at ‘‘20 minutes to 4.’’ For this latter test condition (‘‘20 minutes to 4’’), Head commented that his patient appeared ‘‘doubtful of the meaning of the words 20 minutes to.’’ Other researchers have used the CDT to assess deficits in symbolic formation in neurologic patients (Mayer-Gross, 1935; McFie & Zangwill, 1960; Van Horst, 1934). Classically trained neurologists often associate the CDT as a means to assess constructional apraxia. Kleist (1912, cited in Benton & Tranel, 1993) described constructional apraxia as deficits in formative activities necessary to assemble parts into a meaningful whole. For Kleist, the essential defect in constructional apraxia was the ineffective translation of visuoperceptual information into an effective motor act. In this sense, the concepts of Kleist are consistent with the constructs Head (1926) used to understand impaired clock drawings produced by aphasic patients. Interestingly, Kleist tended to associate constructional apraxia with lesions in the left posterior cortex. This is in stark contrast to the anecdotal point of view that tends to automatically associate defective clock drawing (or any other impaired figure copying test for that matter) with a right parietal lesion. While it is certainly true that patients with right parietal lesions often produce very spatially impaired clock drawings, it is naive to automatically associate impaired clock drawing with either a lesion in any single brain region, or as a measure of any single cognitive operation. As described below, defective clock drawings to command and copy can be associated with a wide array of neurologic lesions and underlying cognitive disorders. Many time settings have been used in the CDT. Kaplan (1988, 1990) recommends using ‘‘ten after eleven.’’ First, clock setting for ten after eleven requires the patient to disambiguate a complex propositional command. Second, since the numbers ‘‘10’’ and ‘‘11’’ are on the clock,

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Clock Drawing

patients need to resist the temptation to be pulled to the numbers ‘‘10’’ and ‘‘11.’’ Thus, clock setting to ten after eleven tends to elicit a variety of stimulus-bound errors. In a survey of neuropsychologists and neurologists described by Freedman, Leach, Kaplan, Shulman, and Delis (1994), other commonly used clock settings include ‘‘20 after 8’’ and ‘‘3 o’clock.’’

Psychometric Data Some scoring guidelines for the CDT can be found as part of the Boston Diagnostic Aphasia Examination supplementary language tests (BDAE; Goodglass & Kaplan, 1972, 1983; Goodglass, Kaplan, & Baresi, 2001). In the original BDAE (with the rakishly purple cover [see Holland’s forward to the third edition of the BDAE, 2001]), the CDT test was one of several tests believed to be sensitive to parietal lobe injury. As described in the BDAE corpus, clock drawing is one of six figures where patients are asked to draw to command and copy (i.e., a clock with hands set for 10 after 11, a daisy, an elephant, a red cross, a three-dimensional cube, and a house). For the clock drawing portion of the test, a three-point scoring system was described awarding a point for an approximately circular clock face, symmetry of number placement, and correctness of numbers. No scoring for the representation of the clock hands was suggested. Normative data for the entire figure drawing test (range 0–13 for the separate command and copy test conditions) is provided. Separate normative data for the CDT is not included. Additional normative information is provided by Borod, Goodglass, and Kaplan (1980). In this report, norms are also provided for two additional clock assessment procedures – clock setting with numbers and clock setting without numbers. In both tests, the patient is presented with pre-drawn clock faces with and without numbers and are asked to draw the hands to read 1:00, 3:00, 9:15, and 7:30. Performance is assessed using a 12-point scoring system. Borod and colleagues (1980) described both age and educational effects for this clock assessment procedure. Other researchers have commented on the effects of education on clock drawing test performance (Marcopulos, McLean, & Giuliano, 1997). The Kaplan-Baycrest Neuropsychological Survey (KBNS; Leach et al., 2000) contains a comprehensive clock test consisting of five parts: (1) clock drawing to command where patients are asked to draw the face of clock put in all the numbers and set the hands for 10 after 11, (2) a clock drawing to copy condition where

patients are presented with a blank page and asked to draw a clock with numbers and set the hands for ten after eleven, (3) a pre-drawn clock face where patients are asked to put in all the numbers and set the hands for 20 after 8, (4) a clock reading test with hands, but without numbers where patients are asked to identify a specified time, and (5) a clock reading test with numbers and hands where patients are, again, asked for the specified time. For clock drawing to command and copy, behavior related to the drawing of the clock face, the numbers, and hands, and the representation of the clock hands originating from the center of the clock are each scored using a 13-point scoring system. The pre-drawn clock face condition is scored using an 11-point scoring system. Thus, for this portion of the test scores have a 0–37 point range. Each clock reading subtest contains six test stimuli. Performance on the three clock drawing test conditions are combined with a score measuring the copy of a complex figure for a combined age-corrected scale score. The two clock reading subtests are scored separately (range 0–6). For these two tests, age-related percentile cut scores are provided. Freedman, Leach, Kaplan, Shulman, and Delis (1994) described a very comprehensive clock scoring system using a variety of clock drawing conditions and clock settings. Normative data was collected and grouped by decade from age 20 to 80þ. Separate scales were developed to assess the drawing of the clock face, the drawing of the numbers, the presence and drawing of the clock hands, and the degree to which the clock hands emanated from the center of the clock face. Base rates for a wide range of clock drawing behavior are provided. These data show that certain errors occur more frequently with age. For example, for clock setting using ‘‘ten after eleven,’’ the representation of the clock hands tends to be differentially affected by age. As noted above, a wide number of clock drawing procedures have been reported (Lezak, Howison, & Loring, 2004); however, most researchers follow Kaplan’s (1988, 1990) suggestion and ask patients to set the hands for ‘‘ten after eleven’’ (Freedman et al., 1994). It is important to understand that many CDT scoring procedures are essentially atheoretical, that is, the administration and scoring procedures were devised with an eye toward sensitivity to brain damage or neurological insult rather than to assess for deficits involving specific cognitive constructs. Recent research, particularly in using the CDT as part of a dementia evaluation, suggests that the command and copy conditions are related to different underlying cognitive mechanisms (Cosentino, Jefferson, Chute, Kaplan, & Libon, 2004; Libon, Swenson,

Clock Drawing

Barnoski, & Sands, 1993; Libon, Malamut, Swenson, & Cloud, 1996; Rouleau, Salmon, Butters, Kennedy, & McGuire, 1992). Equally important, different results are obtained depending on test instruction (see Cosentino et al., 2004 for a review). These considerations are critically important when the task at hand is to differentiate between say, dementia subtypes.

Clinical Uses Focal lesions – Clock drawing has not been extensively studied in non-dementia, focal lesioned patients. Nonetheless, specific patterns of deficits can be associated with specific neurologic lesions. Freedman et al. (1994) and Kaplan (1988, 1990) provide some instructive exemplars. For example, patients with left posterior brain lesion resulting in a Wernicke’s aphasia often present with language comprehension deficits. While these patients may demonstrate general understanding of the clock drawing instructions, numbers may be omitted entirely with hatch marks used as substitutes (Freedman et al., 1994). Patients with a left anterior lesion presenting with a Broca’s aphasia often have difficulty in understanding functor words such as ‘‘to’’ and ‘‘after.’’ These patients, therefore, may be apt to draw the clock hands pointing to the numbers ‘‘10’’ and ‘‘11.’’ Further assessment is required to see if this kind of error is caused by a language-related deficit or represents an executive deficit. Patients with left hemisphere lesions might initiate their drawing on the left side of the clock, that is, on the side contralateral to their intact right hemisphere. Thus, numbers may be written correctly but in a counterclockwise direction (Freedman et al., 1994). Interesting dissociations can be found in clock drawings to command versus copy in focal lesion patients. Kaplan (1990) provides several instructive examples. An analysis of clock drawings produced by a patient with a right parietal lesion demonstrates differential impairment in the copy versus the command test conditions. In the copy test condition, many numbers were omitted on the left side of the drawing. The clock drawing to command did not demonstrate this behavior and was generally intact compared to the copy test condition. Kaplan (1988) demonstrated the opposite profile in a patient with a right temporal lesion. Here, there was differential impairment in the command condition. For this patient, the clock drawing to copy was generally intact. For the right parietal lesioned patient, the differential impairment in the copy condition likely reflected a deficit involving visually mediated neglect of left hemi-space. For the left temporal lobe patient, the visuospatial impairment seen

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in the command condition may be due to a deficit in visuospatial memory. Tranel, Rudrauf, Vianna, and Damasio (2008) administered the CDT to a large group of focal lesioned patients. Imaging studies found errors on the CDT were associated with right parietal (supramarginal gyrus) and left inferior frontal-parietal opercular brain damage. These researchers also noted that visuospatial errors were predominant in patients with right hemisphere damage, whereas time-setting errors were predominant in patients with left hemisphere lesions. Dementia – As noted above, since the late 1980s, there has been a plethora of research demonstrating the value of the clock drawing test as both a screening test for dementia as well as a means of investigating cognitive constructs that may differentiate between dementia subtypes (see Cosentino et al., 2004 for a review). Rouleau et al. (1992, 1996) examined patients with Alzheimer’s disease (VaD) and Huntington’s disease (HD) administering a clock drawing test to command and copy with hands set for ten after eleven. An analysis of errors proved effective in differentiating between dementia subtypes. AD patients made more conceptual errors while HD patients produced more graphomotor errors. These authors speculated that semantic knowledge deficits might underlie the deficits produced on the CDT by AD patients whereas executive dysfunction might underlie the errors produced by HD patients. When the command and copy conditions were compared, AD, but not HD patients improved from the command to copy test conditions. Rouleau’s research was the impetus for a series of studies conducted by Libon and colleagues (Cosentino et al., 2004; Libon et al., 1993, 1996) that examined differences on the CDT between patients with AD and vascular dementia (VaD). In their original study, Libon et al. (1993) found no difference in errors between AD and VaD patients in the command condition. However, similar to Rouleau, AD patients improved, that is, made fewer errors than VaD patients in the copy condition. These findings were replicated in a second study (Libon et al., 1996), that is, AD patients generally improved from the command to copy test conditions compared to VaD patients. Cosentino et al. (2004) grouped dementia patients diagnosed clinically with either AD or VaD on the basis of MRI white matter alterations (MRI-WMA). These groups were compared to dementia patients with Parkinson’s disease (PD). Patients presenting with minimal to mild MRI-WMA continued to improve from the command to copy test conditions, that is, produce fewer errors in the copy versus the condition test conditions, compared to patients with moderate to severe MRI-WMA and PD patients. Errors produced the copy condition were

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correlated with poor performance on executive tests. Errors produced in the command condition were correlated with overall dementia severity and tests related to semantic knowledge. Cahn-Weiner (2003) correlated CDT performance with MRI measures of atrophy and found that impaired CDT performance was attributable to impairment in multiple cognitive domains but was primarily related to volume loss involving the right temporal cortex. Taken as a whole, this research suggests that different cognitive constructs underlie impaired clock drawing in patient with cortical versus subcortical dementia.

Cross References ▶ Constructional Apraxia

References and Readings Benton, A., & Tranel, D. (1993). Visuoperceptual, visuospatial, and visuoconstructional disorders. In K. M. Heilman & E. Valenstien, (Eds.), Clinical neuropsychology (3rd ed.). New York: Oxford University Press. Borod, J. C., Goodglass, H., & Kaplan, E. (1980). Normative data on the Boston diagnostic aphasia examination, parietal lobe battery, and the Boston Naming Test. Journal of Clinical Neuropsychology, 2, 209–216. Cahn-Weiner, D. A., Williams, K., Grace, J., Tremont, G., Westervelt, H., & Stern, R. A. (2003). Discrimination of dementia with lewy bodies from Alzheimer disease and Parkinson disease using the clock drawing test. Cognitive and Behavioral Neurology, 16, 85–92. Cosentino, S., Jefferson, A. J., Chute, D. L., Kaplan, E., & Libon, D. L. (2004). Clock drawing errors in dementia: Neuropsychological and Neuroanatomic considerations. Cognitive and Behavioral Neurology, 17, 74–83. Freedman, M., Leach, L., Kaplan, E., Shulman, K. I., & Delis, D. C. (1994). Clock drawing: A neuropsychological analysis. New York: Oxford University Press. Goodglass, H., & Kaplan, E. (1972). Assessment of aphasia and related disorders (1st ed.). Philadelphia, PA: Lea and Febiger. Goodglass, H., & Kaplan, E. (1983). Assessment of aphasia and related disorders (2nd ed.). Philadelphia, PA: Lea and Febiger. Goodglass, H., Kaplan, E., & Baresi, B. (2001). Assessment of aphasia and related disorders (3rd ed.). Philadelphia, PA: Lippincott, Williams. Head, H. (1926). Aphasia and kindred disorders of speech. New York: Macmillan. Kaplan, E. (1988). A process approach to neuropsychological assessment. In T. Boll & B. K. Bryant (Eds.), Clinical neuropsychology and brain function: Research, measurement, and practice. Washington, DC: American Psychological Association. Kaplan, E. (1990). The process approach to neuropsychological assessment of psychiatric patients. Journal of Neuropsychiatry and the Clinical Neurosciences, 2, 72–87. Leach, L., Kaplan, E., Rewilak, D., Richards, B., & Proulx, G.-B. (2000). The kaplan baycrest neurocognitive assessment. San Antonio, TX: The Psychological Corp. Lezak, M., Howison, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

Libon, D. J., Malamut, B. L., Swenson, R., & Cloud, B. S. (1996). Further analyses of clock drawings among demented and non-demented subjects. Archives of Clinical Neuropsychology, 11, 193–211. Libon, D. J., Swenson, R., Barnoski, E., & Sands, L. P. (1993). Clock drawing as an assessment tool for dementia. Archives of Clinical Neuropsychology, 8, 405–416. Marcopulos, B. A., McLean, A., & Giuliano, A. J. (1997). Cognitive impairment or inadequate norms: A study of healthy, rural, older adults with limited education. The Clinical Neuropsychologist, 11, 111–131. Mayer-Gross, W. (1935). Some observations on apraxia. Proceeding of the Royal Society of Medicine, 28, 1203–1212. McFie, J., & Zangwill, O. L., (1960). Visuo-constructive disabilities associated with lesions of the left cerebral hemisphere. Brain, 83, 243–260. Rouleau, I., Salmon, D. P., & Butters, N. (1996). Longitudinal analysis of clock drawing in Alzheimer’s disease patients. Brain and Cognition, 31, 17–34. Rouleau, I., Salmon, D. P., Butters, N., Kennedy, C., & McGuire, K. (1992). Quantitative and qualitative analyses of clock drawings in Alzheimer’s and Huntington’s disease. Brain and Cognition, 18, 70–87. Rubin, L. A., Barr, W. B., & Burton, L. A. (2005). Assessment practices of clinical neuropsychologists in the United States and Canada: A survey of INS, NAN, and APA Division 40 members. Archives of Clinical Neuropsychology, 20, 33–65. Tranel, D., Rudrauf, D., Vianna, E. P. M., & Damasio, H. (2008). Does the clock drawing test have focal Neuroanatomical correlates? Neuropsychology, 22, 553–562. Tuokko, H., Hadjustavropoulos, T., Miller, J. A., & Beattie, B. L. (1992). The clock test: A sensitive measure to differentiate normal elderly from those with Alzheimer disease. Journal of the American Geriatrics Society, 40, 579–584. Van der Horst, L. (1934). Constructional apraxia: Osychological views on the conception of space. Journal of Nervous and Mental Disease, 80, 645–650.

C-Log ▶ Cognitive-Log

Clomipramine J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Clomipramine

Brand Name Anafranil

Clonazepam

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Class

Additional Information

Tricyclic antidepressant


Proposed Mechanism(s) of Action Boosts neurotransmitters serotonin and norepinephrine/ noradrelaline; blocks serotonin transporter; apparently desensitizes both serotonin 1A receptors and beta adrenergic receptors

Indication

Clonazepam J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Obsessive-compulsive disorder, depression, cataplexy syndrome

Generic Name Off Label Use

Clonazepam

Anxiety, insomnia, chronic pain

Brand Name

Side Effects Serious Paralytic ileus, hyperthermia, lowered seizure threshold and rare seizures, orthostatic hypotension, sudden death, arrhythmias, tachycardia, QTc prolongation, increased intraocular pressure, hepatic failure, extrapyramidal symptoms, mania, and suicidal ideation

Klonopin

Class Anxiolytic

Proposed Mechanism(s) of Action

Common

Binds to benzodiazepine receptors at the GABA-A ligandgated channel, thus allowing for neuronal hyperpolarization. Benzodiazepines enhance the inhibitory action of GABA via boosted chloride conductance.

Blurred vision, constipation, increased appetite, urinary retention, dry mouth, nausea, diarrhea, heartburn, strange taste in mouth, weight gain, fatigue, weakness, dizziness, headache, anxiety, nervousness, restlessness, sedation, sexual dysfunction, sweating

Indication Panic disorders (with or without agoraphobia), Lennox– Gastaut syndrome, akinetic seizures, myoclonic seizure, absence seizures


Off Label Use Atonic seizures and other anxiety disorders, acute psychosis, insomnia

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Clonidine

Side Effects


Serious

Centrally acting alpha 2 agonist

Respiratory depression, hepatic dysfunction (rare), renal dysfunction and blood dyscrasias, Grand mal seizures

Indication Hypertension

Common Sedation, fatigue, depression, dizziness, memory problems, dysinhibition, confusion, ataxia, slurred speech



Clonidine J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Off Label Use Attention deficit hyperactivity disorder, Tourette’s syndrome, anxiety disorders including PTSD and social anxiety disorder, substance withdrawal including opiates and alcohol, menopausal flushing, clonadine-induced hypersalavation, severe pain in cancer patients

Side Effects Serious Sinus bradycardia, atrioventricular block during withdrawal, hypertensive, encephalopathy, cerebrovascular accidents, and death

Common Dry mouth, dizziness, constipation, sedation, major depression, weakness, fatigue, impotence, loss of libido, insomnia, headache, dermatologic reactions, hypotension, occasional syncope, nervousness, agitation, tachycardia, nausea, vomiting

References and Readings Generic Name Clonidine

Brand Name



Duraclon, Catapres, Catapres-TTS, Clorpres

Class Antihypertensive

Drug Inter action Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Closure

Clorazepate J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Clorazepate

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Common Sedation, fatigue, depression, dizziness, memory problems, dysinhibition, confusion, ataxia, and slurred speech.


Brand Name Azene, Tranxene

Class Anxiolytic

Additional Information Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.com: 8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Proposed Mechanism(s) of Action Binds to benzodiazepine receptors at the GABA-A ligandgated channel, thus allowing for neuronal hyperpolarization. Benzodiazepines enhance the inhibitory action of GABA via boosted chloride conductance. Clorazepate is also hypothesized to inhibit neuronal activity in amgydala-centered fear circuits.

Indication

Closure R ONALD A. C OHEN Brown University Providence, RI, USA

Synonyms Visual integration; Visual synthesis

Anxiety Disorder, symptoms of anxiety, and acute alcohol withdrawal.

Definition Off Label Use Partial seizures (as an adjunct).

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Visual closure refers to the ability to perceive and recognize objects, shapers, features, or symbols from incomplete or degraded visual stimuli. It reflects the capacity of humans to fill in missing information from incomplete sensory input to achieve a meaningful percept.


Historical Perspective

Respiratory depression, hepatic dysfunction (rare), renal dysfunction and blood dyscrasias, and Grand mal seizures.

The principle of visual closure had its roots in Gestalt psychology (Ellis, 1938; Harlow, 1938; Ko¨hler, 1929). Gestalt psychology theorized that operationally brain

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functions (i.e., perception and cognition) are holistic consisting of analog processes that occur in a parallel manner and are self-organizing. This led to the wellknown conclusion regarding perception, and cognition more generally, that ‘‘the whole is greater than the sum of its parts.’’ Based on this framework, all perceptual processes act to achieve optimal organization and reconciliation with the objects that are being perceived. A critical principle driving Gestalt perception is pra¨gnanz (law of conciseness), which maintains that people organize their experience in an orderly, symmetric, and simple manner when possible. Visual closure was one of the five laws of pra¨gnanz, with others including the laws of similarity, proximity, symmetry continuity, and common fate. While each of these laws has potential value in accounting for elements of visual integration, the law of visual closure seems to have had the most direct impact, particularly with respect to clinical neuropsychology. Early clinical studies of the effects of posterior cortical lesions on visual perception indicated that certain patients had difficulty in simultaneously processing all the elements of their visual sensorium to achieve a unified percept, a syndrome that was labeled simultagnosia (Poppelreuter, 1990).

Current Knowledge The idea that visual perception occurs as a function byproduct of active self-organizing processes is now widely accepted by most visual scientists, though many would reject a pure holistic view. Instead, visual perception and higher-order visual processes tend to be conceptualized as the by-product of computational processes carried out by modular neural networks responsible for specific operations. Visual closure is thought to result from such processes occurring in extra-striatal systems found primarily in the parietal cortex. Psychometric studies of closure have tended to employ tests such as the Gollin Figures and Mooney test (Foreman, 1991; Holmes, 1968; Jones & Dennis, 1972; Mooney & Ferguson, 1951). In healthy adults, the ability to recognize line drawings that have been degraded has been shown to be a function of the size of gaps in the drawing (Jones & Dennis, 1972), which in turn reflects the amount of missing information. Performance on closure tests has been shown to not be strongly associated with visual search performance, suggesting that these are distinct visual processes (Foreman, 1991). While tests of closure have existed for over 40 years, there are relatively few studies demonstrating consistent

impairments on tests such as the Gollin Figures and Mooney Closure tests. This may reflect the fact that impairments on this type of task tend to be embedded in other visual perception deficits that are more striking. However, it is also clear that closure paradigms have not been systematically implemented into standard neuropsychological batteries using modern computerized methods, so that definitive conclusions regarding impairments of closure secondary to localized and global brain disorders cannot be reached at this point.

Cross References ▶ Gollin Figures ▶ Hooper Visual Organization Test ▶ Simultanagnosia

References and Readings Ellis, W. D. (1938). A source book of Gestalt psychology. New York: Harcourt, Brace & World. Foreman, N. (1991). Correlates of performance on the Gollin and Mooney tests of visual closure. Journal of General Psychology, 118 (1), 13–20. Harlow, R. F. (1938). Philosophy’s contribution to Gestalt psychology. Journal of Psychology: Interdisciplinary and Applied, 5, 185–200. Holmes, D. S. (1968). Search for ‘‘closure’’ in a visually perceived pattern. Psychological Bulletin, 70(5), 296–312. Jones, E. C., & Dennis, M. E. (1972). Perceptual closure as a function of gap size. Perceptual and Motor Skills, 35(1), 126. Ko¨hler, W. (1929). Gestalt psychology. New York: H. Liveright. Mooney, C. M., & Ferguson, G. A. (1951). A new closure test. Canadian Journal of Psychology/Revue Canadienne de Psychologie, 5(3), 129–133. Poppelreuter, W. (1990). Disturbances of lower and higher visual capacities caused by occipital damage: with special reference to the psychopathological, pedagogical, industrial, and social implications. Oxford/New York: Clarendon Press/Oxford University Press.

Clot Buster ▶ Recombinant Tissue Plasminogen Activator

Clot Busting ▶ Thrombolysis

Clustering

Clotting ▶ Thrombosis

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Side Effects Serious Agranulocytosis, neuroleptic malignant syndrome, seizures, pulmonary embolism, myocarditis, hyperglycemia.

Clozapine J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metarie, LA, USA

Generic Name

Common Increased risk for diabetes, sweating, and increased salivation.


Clozapine

Additional Information Brand Name Clozaril, Leponex

Drug interaction effects: http://www.drugs.com/drug_interactions.html Drug molecule images: http://www.worldofmolecules.com/drugs/ Free drug online and PDA software: www.epocrates.com Gene-based estimate of drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill identification: http://www.drugs.com/pill_identification.html

Class Atypical Neuroleptic

Clumsiness ▶ Ataxia

Proposed Mechanism(s) of Action Blocks dopamine 2 receptors, inhibits serotonin 2A receptors, thus increasing presynaptic release of related catecholamines

Indication Schizophrenia (treatment-resistant), reduction of suicidal behavior.

Cluster Analysis ▶ Clustering

Clustering M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Off Label Use Synonyms Bipolar disorder (treatment-resistant), violence, and aggression associated with psychosis or brain dysfunction.

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Cluster analysis

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Definition Clustering can be thought of as the obverse of factoring. In factor analysis, observations are correlated with each other and the correlation matrix is examined to see which items covary among themselves. In cluster analysis, the correlation matrix is examined to see which individuals or observations covary among themselves. Once the groups of individuals are composed, they are compared to each other in order to see if some external correlate exists. For example, a large group of patients might be administered a series of cognitive measures. The scores on those measures are examined to see which individuals seem to be most similar to each other. Once the groups are empirically formed, their characteristics are examined to see if age or diagnosis or injury severity discriminates among the groups.

Cross References ▶ Correlation Coefficients ▶ Factor Analysis

References and Readings Corter, J. (1996). Tree models of similarity and association. Thousand Oaks, CA: Sage. Kaufman, L., & Rousseeuw, P. (2005). Finding groups in data: An introduction to cluster analysis. Hoboken, NJ: Wiley. McIntyre, R. M., & Blashfield, R. K. (1980). A nearest-centroid technique for evaluating the minimum-variance clustering procedure. Multivariate Behavioral Research 15, 2225–2238.

Cmax N ADIA W EBB Children’s Hospital of New Orleans New Orleans, LA, USA

Synonyms

elimination. The maximum concentration of a drug in blood plasma represents a drug’s peak effect. Cmax is one of the primary pharmacokinetic measures for evaluating how the body acts upon a drug. The same dose of a drug may result on different plasma levels because the body is not a passive recipient. Plasma levels of a drug can be altered by the route of administration, the ease of absorption, the distribution of the drug with the body, the bioavailability or accessible concentration of the drug, and the efficiency with which a drug is metabolized or eliminated. Common factors that affect plasma levels of a drug could include the size of the molecule and its fat solubility; the patient’s gastric pH, physical health or age, and the presence of other medications or foods that may expedite or slow absorption from the gastrointestinal tract into general circulation or alter the drug’s metabolism and excretion. These factors may require changes in dosing in order to achieve therapeutic levels of a drug or alterations in diet, such as to take a drug with or without food. It may also include recommendations to avoid particular foods that are known to induce or inhibit the specific enzyme pathways that metabolize the drug (e.g., grapefruit juice). Drug–drug interactions can alter the plasma levels of a drug, including its peak effect. There are also mediations that inhibit metabolism within a particular liver-mediated enzyme system, thus altering the availability of other agents of that drug itself. This can alter the availability of a drug, including its Cmax. Fluoxetine’s impact on codeine is a good example. Codeine is ultimately synthesized into morphine and via a transformation that requires an enzyme that makes this change possible. Fluoxetine appears to inhibit this process, thus dramatically reducing the pain control intended via the administration of codeine. For some drugs, there is no clear relationship between the concentration of a drug in blood plasma and its pharmacological effect. For other drugs, the concentration must be tightly monitored, typically through regular plasma assessments of drug levels (assays). As a rule of thumb, adverse drug reactions and side effects become more likely as the dose of a drug increases. Consequently, adverse drug effects are more likely at the time of Cmax.

Maximum concentration; Peak concentration

Cross References Definition Plasma concentrations reflect a time curve from the administration of a drug through its peak effect and eventual

▶ p450 Cytochrome System ▶ Pharmacokinetics ▶ Side Effects

Cochlear Nuclei (Dorsal and Ventral)

References and Readings Brunton, L. B., Lazo, J. S., & Parker, K. L. (Eds.). (2005). Goodman & Gilman’s the pharmacological basis of therapeutics (11th ed.). New York: McGraw Hill. Stahl, S. M. (2008). Stahl’s essential psychopharmacology: Neuroscientific basis and practical applications. New York: Cambridge University Press.

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waves in the cochlea are transduced into bioelectrical nerve impulses. The acoustic division of the eighth cranial nerve has its cell bodies in the spiral ganglion of the cochlea.

C Cross References ▶ Auditory System

CMS ▶ Children’s Memory Scale

References and Readings Ropper, A. H., & Brown, R. J. (2005). Deafness, dizziness, and disorders of equilibrium. In Adams and Victor’s principles of neurology. New York: McGraw Hill.

CNC ▶ Coma/Near Coma Scale

Cochlear Nerve ▶ Vestibulocochlear Nerve

CNS Lupus ▶ Lupus Cerebritis

Cochlear Nuclei (Dorsal and Ventral) Cochlea K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Definition The cochlea, a small conical structure, is the part of the inner ear that converts mechanical energy (vibrations) into nerve impulses sent to the brain. It is also known as the organ of hearing. The word cochlea is a Latin word derived from the Greek kokhlos, which refers to the land snail. A coiled tube, the cochlea winds around a central axis, forming the anterior part of the labyrinth. It contains the organ of Corti, which includes the hair cells that constitute the primary mechanisms by which pressure

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Definition Cochlear nuclei is the nuclei that receive first-order auditory input from the organ of Corti in the cochlea of the inner ear.

Current Knowledge The cochlear nuclei are divided into a dorsal and a ventral group. The dorsal cochlear nuclei give rise to the dorsal acoustic stria, which immediately cross the midline and contribute fibers that ascend contralaterally

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in the lateral lemniscus. The ventral cochlear nuclei are the source of two other auditory pathways, the intermediate and the ventral acoustic stria. The former also crosses the midline and, like the dorsal acoustic stria, ascends in the contralateral lateral lemniscus. Fibers making up the ventral acoustic stria, the largest of these three pathways, take three different paths after leaving the nucleus. They (1) synapse in both the ipsilateral or contralateral superior olivary nuclei, which in turn send tertiary fibers to the inferior colliculi via the ipsilateral and contralateral lateral lemniscus, and (2) send fibers directly to the contralateral inferior colliculi, bypassing the superior olivary nuclei. The crossing fibers of the ventral acoustic stria make up what is known as the trapezoid body of the pontine tegmentum. The dorsal and ventral cochlear nuclei themselves are located laterally in the rostral medulla at the pontine–medullary junction near the vestibulocochlear nerve. Since the first-order neurons from the auditory nerve terminate in the cochlear nuclei and the decussations within the auditory system only begin with the secondorder neurons originating in the cochlear nuclei, these nuclei receive input only from one ear. Hence, unilateral pontine lesions affecting the dorsal and ventral cochlear nuclei would be expected to result in ipsilateral hearing deficits (loss).

Cross References ▶ Auditory System

References and Readings Wilson-Pauwek, L., Akesson, E. J., Stewart, P. A., & Spacey, S. D. (2002). Cranial nerves in health and disease. Hamilton, ONT: B.C. Decker.

Coding ▶ Digit Symbol Substitution Test

Cog-Log ▶ Cognitive-Log

Cogniform Disorder D EAN C. D ELIS San Diego School of Medicine San Diego Veterans Affairs Healthcare System La Jolla, CA, USA

Synonyms Conversion disorder; Malingering; Somatoform disorders

Definition In neuropsychology, the assessment of test-taking effort has captured the focus of considerable research and debate. In the past 20 years, over 500 studies have been published in peer-reviewed neuropsychological journals that address the breadth and scope of this problem (see reviews by Hom & Denny, 2002; Iverson & Binder, 2000; Larrabee, 2005; Sweet, 1999). Considerable advances have been made in the development of empirically based methods for identifying individuals who are simulating cognitive problems, including the use of instruments designed specifically to assess cognitive validity (Binder, 1993; Frederick, 1997; Green et al., 1999; Tombaugh, 1996), analysis of atypical performances on standard ability tests (Larrabee, 2003; Millis et al., 1995), and analysis of test–retest profile inconsistencies (Hom & Denny, 2002; Iverson & Binder, 2000). In addition, specific guidelines and criteria have been developed for diagnosing suboptimal effort and malingering on neuropsychological tests (e.g., Slick et al., 1999). Using these methods and guidelines, neuropsychologists have found that the frequency of individuals exhibiting excessive or exaggerated cognitive symptoms in medicolegal evaluations often ranges from 20% to 40% (Miller, 2001; Millis et al., 1995; Mittenberg et al., 2002). In light of the pervasiveness of this problem, it is now generally accepted that cognitive validity testing is an important part of the neuropsychological assessment process, particularly for evaluations that occur in the context of medicolegal or disability-application settings (Bush et al., 2005). While considerable advances have been made in the methods used to detect individuals who are exhibiting inadequate effort and symptom exaggeration on cognitive testing, neuropsychologists often find themselves in a quandary in terms of the diagnostic labels to ascribe to these individuals once they have been identified. The DSM-IV

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offers several possible categories for diagnosing individuals with excessive cognitive symptoms (e.g., Malingering and Conversion Disorder); however, shortcomings of these conditions have been noted in the literature. In particular, Malingering has been the subject of considerable debate and criticism, especially with regards to the objectivity with which clinicians can assess if feigned symptoms were intentionally or unintentionally produced. Improvements have been made in establishing criteria for this condition (e.g., Slick et al., 1999), but clinicians nevertheless often remain reluctant to use any diagnosis that requires them to make judgments about intentionality of symptom exaggeration. Clinicians often face three general problems in trying to use existing DSM-IV categories to classify individuals with excessive cognitive symptoms. These problems include (a) lack of a diagnostic category that adequately targets the specific features of this relatively common condition, (b) the use of criteria that require the clinician to make judgments about internal states that are exceedingly difficult to evaluate in an objective manner (e.g., intentional versus unintentional production of excessive symptoms), and (c) difficulties in determining the relative role that external incentive and sick-role factors may play in the symptom production. Symptom Specificity. The existing DSM-IV categories addressing excessive symptomatology can be divided into two general types: symptom-specific versus symptom-nonspecific conditions. Symptom-specific conditions are those that require amplification of only certain types of symptoms. The DSM-IV offers a relatively small number of symptom-specific categories, which fall only among the somatoform disorders (e.g., Somatization Disorder and Conversion Disorder) and dissociative disorders (e.g., Dissociative Amnesia and Dissociative Fugue). In addition, the DSM-IV offers two symptom-nonspecific conditions, Malingering and Factitious Disorder, which are discussed in the next section. A major problem in trying to subsume individuals with excessive cognitive complaints or invalid test performances into one of the symptom-specific diagnoses is that the cognitive symptoms of many of these cases simply fail to fit adequately in these categories. Following are explanations of this problem for each of the symptom-specific categories provided in the DSM-IV: (a) Somatization Disorder requires at least four pain symptoms, two gastrointestinal symptoms, one sexual symptom, and one pseudoneurological symptom. However, many individuals who present with primarily excessive cognitive symptoms have few if any physical complaints (Larrabee, 2005).

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(b) Undifferentiated Somatoform Disorder requires ‘‘one or more physical complaints,’’ with no reference made to cognitive difficulties. (c) Conversion Disorder requires ‘‘one or more symptoms or deficits affecting voluntary motor or sensory function’’ [emphasis added], without mention of cognitive or memory difficulties among the specific criteria. (d) Pain Disorder requires only excessive pain symptoms. (e) Somatoform Disorder NOS could conceivably include individuals with predominantly excessive cognitive symptoms; however, ‘‘soma’’ denotes physical rather than cognitive problems, and the list of example cases provided in the DSM-IV for this catchall category makes no reference to excessive cognitive symptoms. (f) Dissociative Amnesia requires one specific type of cognitive problem, namely, ‘‘an inability to recall important personal information, usually of a traumatic or stressful nature’’ [emphasis added]. However, individuals presenting with excessive cognitive symptoms do so in a myriad of ways (Bush et al., 2005; Delis & Jacobson, 2000; Larrabee, 2003). Some people endorse problems in all cognitive domains queried, including attention, language, math, visualspatial functions, higher-level executive functions, new learning and memory, and remote recall of important personal information. In contrast, other individuals endorse difficulties in only one or a few specific cognitive skills (e.g., short-term memory and concentration), while denying problems in other cognitive domains, including recall of important personal information. In fact, cases of isolated difficulty in remembering important autobiographical information are relatively rare, illustrating the limited utility of this diagnostic category for the vast majority of cases with excessive cognitive symptoms. (g) Dissociative Fugue not only requires one specific cognitive difficulty (‘‘inability to recall some or all of one’s past’’), but carries the added stipulation that this difficulty must surface in the context of a ‘‘sudden, unexpected, travel away from home or one’s customary place of daily activities’’ [emphasis added]. These cases are extremely rare among individuals presenting with excessive cognitive symptoms, thereby precluding the use of this category for almost all cases. (h) Dissociative Identity Disorder is thought to occur in individuals with multiple personalities in which they exhibit an inability to recall important

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information about one or more personality states when they are in a different personality state. However, cases of multiple personalities are relatively rare, particularly in clinical–neuropsychological practice, and thus this diagnosis is seldom applicable to individuals with excessive cognitive symptoms. (i) Dissociative Disorder NOS is another catchall category that, conceivably, could encompass individuals with excessive cognitive complaints. However, the tenor of this category is for individuals who exhibit an inability to recall personal information that was of a traumatic or stressful nature, thereby greatly limiting the utility of this category for most cases of excessive cognitive symptoms. Taken together, the aforementioned nine symptom-specific categories either fail to include cognitive complaints, target only highly specific, relatively rare types of cognitive problems, or require that other, qualitatively different symptoms or conditions also be present (e.g., extensive physical symptoms for Somatization Disorder). For these reasons, these diagnoses generally fail to capture the vast majority of individuals presenting with excessive cognitive symptoms. Intentionality. Another difficulty in using existing DSM-IV categories has to do with required criteria related to intentional/unintentional or voluntary/involuntary control over the production of the excessive complaints or symptoms. For example, a key required criterion for the two symptom-nonspecific categories – Malingering and Factitious Disorder – is that the clinician must determine if the excessive symptoms were generated in an intentional or volitional manner. The problem here is that this criterion reflects a causative internal state that, for the majority of cases, is difficult if not impossible to assess in an objective manner. That is, the degree to which a person may be exhibiting excessive symptoms or behaviors in an intentional, voluntary, or conscious manner versus an unintentional, involuntary, or unconscious manner represents an untestable diagnostic hypothesis for many cases (see also Slick et al., 1999). A clinician may have a ‘‘hunch’’ about whether an individual’s excessive complaints or symptoms were under the voluntary or involuntary control of the person, but usually these impressions are not substantiated by objective data, such as a disclosure or confession made by the individual to a clinician or other uninvolved, reliable third party. Another difficulty in this area of diagnosis is that intentionality is likely multifactorial in nature. For example, there may be at least two key components of intentionality that can be dissociated: conscious awareness and

goal-directed motivation. An individual may be both conscious of producing feigned behavior (e.g., is capable of admitting to self and others that he or she is simulating symptoms) and motivated to do so for some type of personal gain; these features would meet criteria for a DSM-IV diagnosis of Malingering. However, someone may be largely unconscious of the feigned behavior (e.g., has convinced himself or herself that the excessive symptoms are real), yet the feigned behavior may still arise due to a specific, goal-directed purpose. For example, it was noted during World War II that some soldiers, when faced with the prospect of entering the frontlines of battle, would develop psychogenic paralysis (what would now be diagnosed as Conversion Disorder given that the symptom amplification occurred primarily in the motor domain). These individuals often appeared to truly believe they were paralyzed, thereby suggesting an unconscious (conversion) process. However, their exaggerated behavior (paralysis) was clearly goal-directed, because it was manifested in the context of an external incentive (avoidance of danger). In these cases, the conscious component of intentionality may have been absent, but the goal-directed motivational component for producing the symptom was likely present. In neuropsychological practice, the same type of dissociation may occur in which individuals may produce excessive cognitive symptoms in reaction to an external incentive (e.g., litigation), thereby suggesting goal-directed motivation for the symptom production. However, these individuals may have nevertheless convinced themselves that their symptoms are real, thereby suggesting a lack of a conscious component to the symptom production. Thus, for these individuals, only certain components of intentionality may be present, with the lack of conscious awareness calling into question whether they would adequately meet the required criteria for a diagnosis of Malingering. Another complicating factor in the assessment of intentionality is that conscious awareness likely exists on a continuum, with individuals varying from being fully conscious, to semiconscious, to largely unconscious of the production of the feigned behavior. Although an operational definition of intentionality is beyond the scope of this chapter, the important point here is that intentionality of symptom production not only refers to an elusive internal state, but it likely has component features that exist on a continuum (e.g., levels of conscious awareness), thereby making this construct exceedingly difficult for clinicians to assess in an objective manner. Consequently, many clinicians are reluctant to use diagnoses such as Malingering, Factitious Disorder, and Conversion Disorder at least in part because of difficulty in objectively assessing the presence or absence

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of intentionality in the generation of the excessive symptom. External Incentive. A third difficulty in using existing DSM-IV categories to diagnose individuals with excessive cognitive symptoms is related to another required criterion for the two symptom-nonspecific categories – Malingering and Factitious Disorder – regarding the presence or absence of external incentive in the production of the symptoms. Specifically, external incentive is a required inclusionary criterion for Malingering and required exclusionary criterion for Factitious Disorder. (If there is an absence of external incentive, then the clinician must make a further determination of whether or not an individual has adopted the sick role in order to diagnose Factitious Disorders). However, the criterion of external incentive carries its own inherent difficulties for clinicians to identify when considering these diagnoses. First, for many cases, practitioners may not have access to sufficient background information about a person’s life to be able to assess if external incentives are operative in the case. That is, a practitioner may be unaware that a patient has or is planning to apply for disability or to initiate a civil lawsuit in the future, or has committed a crime and fears that he or she may soon be apprehended. This lack of knowledge about possible covert sources of external incentives makes it difficult to utilize the diagnoses of Malingering or Factitious Disorder for a number of cases, especially given that such information is a required criterion rather than an optional one for these categories. Second, as currently written, the DSM-IV criteria do not allow for the possibility that a comorbidity may occur between the adoption of the sick role and the presence of external incentives (see also Slick et al., 1999). For example, some individuals may gradually develop into a progressively worsening sick role without the presence of external incentives. However, after a period of time, these individuals may present as so ‘‘disabled’’ that they begin to receive disability payments, without necessarily having actively sought out such compensation. The financial gain, however, likely buttresses and propagates the continuation of the sick role. According to the DSM-IV, these individuals would have started out as having Factitious Disorder, but as soon as the external incentive was initiated and became a reinforcing factor, the diagnosis of Factitious Disorder would be called into question (again, because external incentive is a required exclusionary criterion for this condition). However, for these cases, the predominant causative factor for the excessive symptomatology may still be the adoption of the sick role, with the external incentive playing a secondary or supportive role in the continuation of the symptoms.

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As another example, some individuals may begin to feign symptoms intentionally and consciously in reaction to an external incentive (e.g., a lawsuit). However, these individuals may gradually, and perhaps unconsciously, assume a progressively worsening sick role due to (a) a prolongation in obtaining the external incentive (e.g., caused by delays in the lawsuit); and (b) increased skepticism and questioning on the part of family members, coworkers, or health providers about the authenticity of the individual’s complaints. This prolonged scrutiny may be overwhelming to these individuals, compelling them to adopt the sick role and exhibit illness behavior in widespread areas of their lives, to the point where they may even convince themselves of the authenticity of their symptoms. In other words, while the DSM-IV treats external incentive and sick role as mutually exclusive diagnostic criteria for differentiating Malingering and Factitious Disorder, in reality, as is the case for most psychiatric conditions, they may co-occur in varying degrees (Slick et al., 1999). Given these limitations in the DSM-IV, the following two diagnostic categories were proposed by Delis and Wetter (2007) to encompass cases of excessive cognitive complaints or poor (invalid) test performances in the absence of sufficient evidence of intentionality of symptom production to warrant a diagnosis of Malingering.

Neuropsychology of Cogniform Disorder The essential feature of Cogniform Disorder is a pattern of cognitive complaints or low scores on psychometric cognitive tests that are considered to be excessive because they cannot be fully explained by a neurological disorder, by another mental disorder that is associated with CNS dysfunction (e.g., schizophrenia), by a general medical condition known to affect CNS function (e.g., renal disease), by the direct effects of a substance (e.g., opioid medications), or by other factors known to affect cognitive functioning (e.g., developmental learning disorder, insomnia, and normal aging process). If the cognitive complaints or poor test performances occur in the presence of a known neurological or mental disorder or any other factor known to affect CNS function (e.g., medication), the cognitive symptoms are in excess of what would be expected from the history, physical examination, laboratory tests, or psychometric validity testing. Findings from the clinical interview or psychometric testing of cognitive functions do not substantiate the degree of cognitive complaints or symptoms because of the presence of at least two of the following features:

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(a) Cognitive complaints or poor test performances that are rare for patients with documented mild to moderate generalized brain damage (e.g., loss of remote autobiographical memories and inability to perform overlearned verbal skills like reading, spelling, or simple math) (b) Inconsistencies between the individual’s excessive cognitive complaints or poor test performances and the relatively mild nature of the injury or illness as documented in the medical records (c) Inconsistencies between the individual’s excessive cognitive complaints or poor test performances and observed behavior (d) Delayed onset of excessive cognitive complaints or symptoms after an injury and/or significant worsening of symptoms over time without an adequate explanation for the decline in functioning (e.g., subsequent neurological complications) (e) Significant inconsistencies in cognitive test scores or profiles across repeat evaluations (f) Patterns of cognitive test scores within an examination that are rare for brain-damaged patients (g) Significant inconsistencies in cognitive complaints or symptoms over time (h) Evidence of insufficient test-taking effort or exaggeration on tests designed specifically to assess validity of cognitive performance (j) Evidence of insufficient test-taking effort or exaggeration on specific measures obtained from standard ability tests that have been empirically found to assess validity of cognitive performance Considerable individual differences are found in the performances of people with this condition on psychometric tests of cognitive skills (Larrabee, 2003; Slick et al., 1999). Some individuals obtain markedly low scores on most cognitive tests administered; these individuals are often less sophisticated about medical and psychological conditions and more blatant in their symptom amplification. Other people may obtain low and invalid scores on only a few tests administered (e.g., memory tasks); these individuals may be more subtle in their symptom exaggeration and, as a result, more difficult to identify. Occasionally, an individual may perform within expected ranges on most cognitive tests administered, including cognitive validity tests, and yet continue to complain of extensive cognitive problems and dysfunction in their daily lives. These individuals may have learned from other sources (e.g., Internet; attorney coaching) that neuropsychological tests are capable of detecting poor test-taking effort, and consequently exert adequate effort on psychometric tests despite

reporting significant cognitive complaints and dysfunction in their daily lives. The primary distinguishing feature between Cogniform Disorder and Cogniform Condition (see below) concerns the degree to which the individual presents as cognitively impaired in widespread areas of his or her life. Specifically, a diagnosis of Cogniform Disorder should be made if there is reasonable evidence that the individual exhibits excessive cognitive symptoms in most if not all areas of his or her life and seemingly at all times, thereby suggesting a conversionlike adoption of the sick role manifested primarily as cognitive dysfunction. In addition, in Cogniform Disorder, the degree of claimed disability in performing activities of daily living will often parallel the individual’s complaints of cognitive dysfunction and poor (invalid) cognitive test performance. For example, the individual not only obtains severely deficient (and likely invalid) scores on tests of visual-motor and visual-spatial functioning, but he or she also ceases to drive a vehicle because of the perceived cognitive problems. In many ways, Cogniform Disorder is analogous to the somatoform condition of Conversion Disorder, but with the excessive symptoms manifested primarily in terms of cognitive dysfunction rather than deficits affecting primarily motor or sensory functions (e.g., nonepileptic seizures). For this reason, Cogniform Disorder should be considered as a new subtype of the somatoform disorders.

Neuropsychology of Cogniform Condition The essential features of Cogniform Condition are the same as those of Cogniform Disorder in every respect, with the exception of the degree to which the individual exhibits cognitive dysfunction in widespread areas of his or her everyday life. That is, in Cogniform Condition, there is (a) a lack of reasonable evidence that the individual presents as cognitively dysfunctional in many areas of his or her life, and (b) evidence of significant inconsistencies between the individual’s excessive cognitive complaints or poor test performances in an evaluation and his or her higher level of everyday functioning. For example, an individual may obtain severely deficient (and likely invalid) scores on tests of visual-motor and visual-spatial functioning and yet continues to drive a vehicle without apparent difficulty. In other words, in Cogniform Condition, the individual is not given a diagnosis of ‘‘disorder,’’ because there is a lack of reasonable evidence that the individual is acting out the ‘‘sick role’’ of being cognitively dysfunctional in widespread areas of his or her life despite presenting to the clinician in a manner that suggests that

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he or she should be markedly impaired in everyday functioning.

Cogniform Disorder and Condition Versus Malingering: Similarities and Differences Cogniform Disorder, Cogniform Condition, and Malingering (when manifested in the form of cognitive dysfunction) are similar in that the individual may present with excessive cognitive complaints or exhibit evidence of inadequate effort and exaggeration on formal neuropsychological testing. However, a diagnosis of Cogniform Disorder or Cogniform Condition should not be made if there is reasonable evidence that the excessive cognitive symptoms are produced in an intentional or volitional manner, in which case a diagnosis of Malingering may be warranted. As noted above, however, this determination can be difficult to make for many cases due to inherent problems in objectively assessing the internal state of the intentionality of simulated behavior. For this reason, it is likely that many cases of excessive cognitive symptoms would receive the more neutral diagnosis of Cogniform Condition, and possibly a diagnosis of Cogniform Disorder if the individual exhibits cognitively dysfunctional behavior in widespread areas of his or her life. However, when evidence emerges that implicates at least a conscious component in the production of the excessive cognitive symptoms, then a diagnosis of Malingering (or Malingered Neuropsychological Dysfunction; Slick et al., 1999) may be warranted. Following are different examples of evidence that can be supportive of a diagnosis of Malingering: (a) On psychometric testing, an individual obtains an accuracy score on a forced-choice recognition memory test that falls significantly below a chance level. Such a score provides empirical evidence that the individual correctly remembered the right answers above a chance level and used this knowledge to frequently select the wrong answer (Larrabee, 2003; Millis, 1992). (b) A person who is involved in two separate personalinjury lawsuits for different accidents complains of one set of symptoms and injuries to doctors associated with one lawsuit and different symptoms and injuries to other doctors associated with the second lawsuit. Such selective reporting of symptoms that correspond to the different lawsuits suggests a conscious component to the symptom amplification. (c) An individual ‘‘confesses’’ to intentionally performing poorly when taking cognitive tests.

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As proposed here, a diagnosis of Cogniform Disorder or Cogniform Condition does not exclude the possibility of intentional production of the excessive symptoms; rather, these categories imply only that there is insufficient evidence at the time of the assessment to formulate a diagnosis of intentionality and therefore Malingering. Indeed, an advantage of having diagnostic categories such as Cogniform Disorder and Cogniform Condition is that they allow the clinician to label the cognitive symptoms as excessive using more neutral terms that avoid the accusatory implications of Malingering when there is a lack of clear evidence to make that diagnosis. In addition, as discussed above, intentionality is likely multifactorial in nature and is comprised of at least two key components: conscious awareness and goal-directed motivation. The individual who has convinced himself or herself that the feigned behavior is real may not be fully or even partially conscious of his or her symptom amplification, but this person may nevertheless have developed the symptoms in reaction to the presence of external or interpersonal incentives for personal gain. The categories Cogniform Disorder and Cogniform Condition allow the clinician to acknowledge the presence of incentives that may have played a significant role in the goal-directed motivation for the excessive symptomatology without having to make the difficult determination of whether the individual is conscious or unconscious of these dynamics.

References and Readings Binder, L. M. (1993). Assessment of malingering after mild head trauma with the Portland Digit Recognition Test. Journal of Clinical and Experimental Neuropsychology, 15, 170–182. Binder, L. M., Storzbach, D., Anger, W. K., Campbell, K. A., & Rohlman, D. S. (1999). Subjective cognitive complaints, affective distress, and objective cognitive performance in Persian Gulf War Veterans. Archives of Clinical Neuropsychology, 14, 531–536. Bush, S. S., Ruff, R. M., Troster, A. I., Barth, J. T., Koffler, S. P., & Pliskin, N. H. (2005). Symptom validity assessment: Practice issues and medical necessity. Archives of Clinical Neuropsychology, 20, 419–426. Delis, D. C., & Jacobson, M. (2000). Neuropsychological testing. Encyclopedia of Psychology. New York: American Psychological Association/ Oxford University Press. Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (2000). The California verbal learning test (2nd ed.). San Antonio: The Psychological Corporation. Delis, D. C., & Wetter, S. R. (2007). Cogniform disorder and cogniform condition: Proposed diagnoses for excessive cognitive symptoms. Archives of Clinical Neuropsychology, 22, 589–604. Frederick, R. I. (1997). Validity indicator profile. Minnetonka: NCS Assessments. Gervais, R. O., Russell, A. S., Green, P., Allen, L. M., Ferrari, R., & Pieschl, S. D. (2001). Effort testing in patients with fibromyalgia and disability incentives. Journal of Rheumatology, 28, 1892–1899.

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Green, P., Rohling, M. L., Lees-Haley, P. R., & Allen, L. M. (2001). Effort has a greater effect on test scores than severe brain injury in compensation claimants. Brain Injury, 15, 1045–1060. Hom, J., & Denney, R. L. (2002). Detection of response bias in forensic neuropsychology. Journal of Forensic Neuropsychology, 2, 1–166. Iverson, G. L., & Binder, L. M. (2000). Detecting exaggeration and malingering in neuropsychological assessment. The Journal of Head Trauma Rehabilitation, 15, 829–858. Iverson, G. L., & Franzen, M. D. (1996). Using multiple objective memory procedures to detect stimulated malingering. Journal of Clinical and Experimental Neuropsychology, 18, 38–51. Larrabee, G. J. (2003). Detection of malingering using atypical performance patterns on standard neuropsychological tests. The Clinical Neuropsychologist, 17, 410–425. Larrabee, G. J. (2005). Assessment of malingering. In G. L. Larrabee (Ed.), Forensic Neuropsychology. New York: Oxford University Press. Millis, S. R. (1992). The recognition memory test in the detection of malingered and exaggerated memory deficits. The Clinical Neuropsychologist, 6, 406–414. Millis, S. R., Putnam, S. H., Adams, K. M., & Ricker, J. H. (1995). The California verbal learning test in the detection of incomplete effort. Psychological Assessment, 7, 463–471. Mittenberg, W., Patton, C., Canyock, E. M., & Condit, D. C. (2002). Base rates of malingering and symptom exaggeration. Journal of Clinical and Experimental Neuropsychology, 24, 1094–1102. Slick, D. J., Sherman, E. M. S., & Iverson, G. L. (1999). Diagnostic criteria for malingered neurocognitive dysfunction: Proposed standards for clinical practice and research. The Clinical Neuropsychologist, 13, 545–561. Sweet, J. J. (1999). Malingering: Differential diagnosis. In J. J. Sweet (Ed.), Forensic Neuropsychology. Lisse: Swets and Zeitlinger. Tombaugh, T. N. (1996). Test of memory malingering. New York: Multi Health Systems.

Cognistat DANIEL N. A LLEN University of Nevada Las Vegas Las Vegas, NV, USA

Synonyms Neurobehavioral cognitive status examination (NCSE)

Description The Cognistat test battery (Kiernan, Mueller, & Langston, 1995), formerly called the Neurobehavioral Cognitive Status Examination, is a screening tool designed to assess a number of different cognitive domains including Orientation, Attention, Language Abilities (Comprehension, Repetition, Naming), Construction, Memory, Calculations, and Reasoning (Similarities, Judgment). A rating

for Level of Consciousness is also provided. The various subscales are modeled after more extensive and well-validated neuropsychological tests but in an abbreviated from. For example, Attention is assessed using digit repetition similar to that used on the Wechsler scales. Unlike other screening procedures that yield a single summary score, Cognistat is designed to yield a score for each domain and thus produce a differentiated profile of cognitive abilities. Cognistat also employs an adaptive testing approach (referred to as a screen and metric approach) to decrease the time spent in administration. In this approach, an item that is of average difficulty is first administered for each subtest. If that item is passed, no other items are administered from that subtest, but if it is failed, additional easier items are administered. The raw scores for each subscale are then plotted on a standard profile form, and performance is classified as being in the average range or as indicative of mild, moderate, or severe impairment.

Current Knowledge Because extensive validity and normative information were not available when Cognistat was originally published, a number of studies have subsequently examined its sensitivity to brain damage as well as the influence of demographic variables on test performance. Research revealed that the Cognistat may be more sensitive to brain damage than the Mini Mental State Exam, Cognitive Capacity Screening Examination, and Mattis Dementia Rating Scale (e.g., Drane et al., 2003). Cognistat has also been found to be sensitive to a variety of neurological and psychiatric disorders, and to age-related changes in cognitive abilities (for brief review see Doninger, Ehde, Bode, Knight, Bombardier, & Heinemann, 2006). Correlations between its various subtests and neuropsychological measures of similar abilities provide evidence for its construct validity (Nabors, Millis, & Rosenthal, 1997). The Cognistat battery also has limitations. For example, performance is influenced by demographic variables, including age and education. Although the original validation study of the Cognistat demonstrated no differences in performance between age groups (Kiernan, Mueller, Langston, & Van Dyke, 1987), subsequent investigations found that increased age is associated with poorer performance on the Construction, Memory, Similarities, Attention, and Calculation domains, with Construction and Memory appearing to be the most consistently impacted by age (Drane & Osato, 1997). Additionally, years of education and low levels of educational attainment are

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associated with diminished performance (Macauly et al., 2003; Ruchinskas et al., 2001). With only limited normative data available to correct for these influences (Drane et al., 2003), interpretation of performance in the elderly and those with limited education must be tentative. Finally, a recent study of community dwelling and hospitalized individuals with traumatic brain injury suggests that the Cognistat should not be used to profile neurocognitive strengths and weaknesses because measurement error accounted for the majority of variance in subtest scores (Doninger et al., 2006). Thus, the Cognistat provides a useful method to screen patients with a variety of neurological and psychiatric disorders for neurocognitive impairment, although additional research appears warranted before that it can be used to make inferences regarding impairment of discrete cognitive abilities, and more extensive normative data is needed.

Cross References ▶ Mattis Dementia Rating Scale (DRS) ▶ Mini Mental State Exam (MMSE)

References and Readings Doninger, N. A., Ehde, D. M., Bode, R. K., Knight, K., Bombardier, C. H., & Heinemann, A. W. (2006). Measurement properties of the neurobehavioral cognitive status examination (Cognistat) in traumatic brain injury rehabilitation. Rehabilitation Psychology, 51(4), 281–288. Drane, D. L., & Osato, S. S. (1997). Using the neurobehavioral cognitive status examination as a screening measure for older adults. Archive of Clinical Neuropsychology, 12(2), 139–143. Drane, D. L., Yuspeh, R. L., Huthwaite, J. S., Klingler, L. K., Foster, L. M., Mrazik, M., & Axelrod, B. N. (2003). Healthy older adult performance on modified version of the Cognistat (NCSE): Demographic issues and preliminary normative data. Journal of Clinical and Experimental Neuropsychology, 25(1), 133–144. Kiernan, R. J., Mueller, J., & Langston, J. W. (1995). Cognistat (Neurobehavioral Cognitive Status Examination). Lutz, FL: Psychological Assessment Resources. Kiernan, R. J., Mueller, J., Langston, J. W., & Van Dyke, C. (1987). The Neurobehavioral Cognitive Status Examination: A brief but quantitative approach to cognitive assessment. Annals of Internal Medicine, 107(4), 481–485. Macaulay, C., Battista, M., Lebby, P. C., & Mueller, J. (2003). Geriatric performance on the Neurobehavioral Cognitive Status Examination (Cognistat) what is normal? Archives of Clinical Neuropsychology, 18, 463–471. Nabors, N. A., Millis, S. R., & Rosenthal, M. (1997). Use of the Neurobehavioral Cognitive Status Examination (Cognistat) in traumatic brain injury. Journal of Head Trauma Rehabilitation, 12(3), 79–84. Schrimsher, G. W., Parker, J. D., & Burke, R. S. (2007). Relation between cognitive testing performance and pattern of substance use in males at treatment entry. Clinical Neuropsychologist, 21(3), 498–510.

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Cognition ▶ Cognitive Functioning

C Cognitive Affective Syndrome R OBERT R IDER Drexel University Philadelphia, PA, USA

Synonyms Cerebellar cognitive affective syndrome

Definition First described by Schmahmann and Sherman (1997), cerebellar cognitive affective syndrome (CAS) refers to a cluster of impairments involving higher-order cognitive processes and affective functioning. Symptoms tend to cluster in executive dysfunction, including problems with planning, set shifting, verbal fluency, abstract reasoning, perseveration, attentional dysregulation, hyperactivity, impulsivity and disinhibition, and deficits in working memory. However, symptoms may also include visuospatial disorders, expressive language disorders, affective abnormalities, difficulties with visuospatial organization, visual memory, logical sequencing, and blunted or inappropriate affect (Schmahmann & Sherman, 1997).

Current Knowledge Causes and Correlates of CAS The co-occurrence of these cognitive and affective symptoms arises from the disruption of neuroanatomical circuits connecting the cerebellum with frontal, parietal, temporal, and limbic cortices. Damage to these connections can occur in association with cerebellar infarct (Schmahmann and Sherman, 1997), cerebellar atrophy associated with severe alcoholism (Fitzpatrick, et al., 2008), cerebellar tumor or tumor resection (Levihson, et al., 2000; Konczak, 2005), trauma, neurodegenerative disorders, or cerebellitis. Affective symptoms have been associated with damage to the cerebellar vermis (Levihson, et al., 1997). Lesions of the anterior lobe of the cerebellum

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tend to produce only minor changes in executive and visual–spatial functions. Children with a cognitive affective syndrome can also have autistic characteristics, and diagnosis of autism can be confounded by cerebellar lesions.

References and Readings Schmahmann, J., & Sherman, J. (1998). The cerebellar cognitive affective syndrome. Brain, 121, 561–579. Schmahmann, J., Weilburg, J. D., Sherman, J. B., & Janet, C. (2007). The neuropsychiatry of the cerebellum - insights from the clinic. Cerebellum, 6(3), 254–267.

Cognitive Archives ▶ Cognitive Correctors

Cognitive Assessment System L EESA V. H UANG California State University Chico, CA, USA

Synonyms CAS

Description The Das–Naglieri Cognitive Assessment System (CAS; Naglieri & Das, 1997a, 1997b) is a cognitive assessment to assess children aged 5 years, 0 months to 17 years, 11 months. Individual administration time is approximately 1 h. The CAS is arranged in three separate, yet interrelated levels of scores: individual subtests, PASS (Planning, Attention, Simultaneous, and Successive) composite scales, and a Full Scale quotient. Twelve subtests comprise the CAS and each subtest generates a scaled score (M = 10; SD = 3). The Standard Battery utilizes 12 subtests with three subtests per PASS process, while the Basic Battery includes eight subtests, two subtests for each PASS process. Each of the four PASS composite scores (M = 100; SD = 15) is a combination of the subtests included in each respective process. Finally, the Full Scale Score (M = 100; SD = 15) is the aggregate total of the four PASS cognitive processes scales, which are equally weighed.

The Planning scale consists of: matching numbers, planned codes, and planned connections. 1. Matching numbers – The individual is asked to locate and underline a pair of matching numbers. The task begins with 1 digit and progressively moves into 7 digit numbers. 2. Planned codes – A client is requested to complete a series of boxes according to a corresponding code provided at the beginning of each item. 3. Planned connections – This subtest is similar to the original trail making task. In this subtest, both numerical and alphabetical sequences are employed. The Attention scale encompasses: number detection, expressive attention, and receptive attention. 1. Number detection – This subtest consists of rows of numbers with both target and distracter stimuli. At the top of each item page, a key is printed with the target numbers. Children are instructed to underline only the target specified. 2. Expressive attention – Children aged 5–7 are to identify the size of an assortment of animals, in spite of the size depicted on the page. For children aged 8–17, this subtest is similar to the Stroop Test. Color words are presented in a different colors of ink (e.g., the word ‘‘red’’ might be in blue ink) and the children are requested to name the color of the ink. 3. Receptive attention – First, a series of pictures or words are presented in which the client must identify and underline identical stimuli. Second, children are requested to recognize and identify two items that share a common characteristic. This subtest is omitted for the Basic Battery. The Simultaneous Processing scale includes: nonverbal matrices, verbal–spatial relations, and figure memory. 1. Nonverbal matrices – A variety of pictures with geometrical shapes or patterns are shown to the student. The student needs to select one option that is consistent with the presented relationship or pattern. 2. Verbal spatial relations – The individual receives auditory information and determines which picture best represents the verbal description given. Presented in a multiple-choice format, the series of pictures allows the student to demonstrate understanding of logical, grammatical, and spatial information. 3. Figure memory – A client is required to trace geometric design previously observed, which is embedded within a larger and more intricate geometrical design. This is omitted from the Basic Battery.

Cognitive Assessment System

The Successive Processing scale consists of: word series, sentence repetition, sentence questions, and speech rate. 1. Word series – Individuals are instructed to repeat a series of commonly used words in the same consecutive order given. The difficulty level increases as the word list starts with two words and ends with nine words. 2. Sentence repetition – This subtest demands that individuals repeat sentences that gradually become longer. The sentences in this subtest utilized color words to reduce the contextual meaning and possible interference with simultaneous processing. The score is based on the total number of correctly repeated items. 3. Speech rate – This subtest is administered only to children aged 5–7. Children are verbally presented with a series of three word combinations and are requested to repeat each combination as quickly and as many times as possible within 30 s. 4. Sentence questions – Administered to children aged 8–17 instead of speech rate. In an extension of the sentence repetition task, this subtest requires that children respond to a question about a nonsensical sentence.

Historical Background The CAS is one of few cognitive processing instruments which incorporate a neuropsychological foundation. The theoretical basis of the CAS is an extension of Alexander S. Luria’s work relating to the brain’s three functional units (Naglieri, 1999, 2005). It was modified and refined by Das, Naglieri, and Kirby into four processing components: Planning, Attention, Simultaneous, and Successive Processing, otherwise known as PASS, to explain differences in cognitive processing of children (Das & Naglieri, 2001). The Planning subtests require individuals to engage in a problem-solving sequence to complete novel tasks (Naglieri, 1999). The development, selection, application, and evaluation of strategies are crucial to the success of performance (Naglieri & Das, 1997b). Subtests in the Attention scale require a combination of three components: focused, selective, and sustained attention (Naglieri, 2005). Focused attention involves the act of attending to presented stimuli in the environment. Selective attention is the concentration of attention to chosen stimuli while disregarding nonessential or competing stimuli. Sustained attention is the differential effort (over time, especially) an individual applies toward task completion. Simultaneous Processing subtests require the ability of an individual to incorporate and comprehend unconnected entities and its relation/

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position to a collective whole (Naglieri, 2005). Successive Processing is described as the unidirectional, consecutive organization of stimuli (Naglieri & Das, 2005).

Psychometric Data The standardization sample of 2,200 children is representative of the US population on nine criteria (Naglieri & Das, 1997b). The internal consistency of the CAS subtests range from 0.75 to 0.89, with a median reliability of 0.82. The Standard Battery had average reliabilities of 0.88 (Planning), 0.88 (Attention), 0.93 (Simultaneous), and 0.93 (Successive). The reliability coefficient range for the Full Scale score was 0.95–0.97. The theoretical premise of the CAS was constructed on a four-factor model; however, factor analyses generated empirical support for both three- and fourfactor models depending upon the age category. Further research has provided support for the CAS as a measure of g. Weak correlations were found between the CAS Standard Battery Full Scale and PASS scales (ranging from 0.37 to 0.67) with the Wechsler Intelligence Scale for Children-third edition (WISC-III; Wechsler, 1991). Predictive validity was moderately established with cluster and subtest scores from the Woodcock–Johnson PsychoEducational Battery-Revised Tests of Achievement (Woodcock & Johnson, 1989).

Clinical Uses The purpose of the CAS is to provide an analysis of an individual’s cognitive abilities through the measurement of the PASS processes (Naglieri, 2005). The authors suggested that the CAS is a valuable alternative tool to the traditional Wechsler or Stanford–Binet scales, when assessing individuals who may have attentiondeficit/hyperactivity disorders (ADHD), LD, mental retardation, traumatic brain injury, serious emotional disturbance, giftedness, and planning problems (Naglieri & Das, 1997b). Furthermore, possessing an understanding of an individual’s PASS profile will provide essential information for the selection and evaluation of instructional recommendations (Das & Naglieri, 2001; Naglieri & Das, 1997b).

References and Readings Das, J. P., & Naglieri, J. A. (2001). The Das-Naglieri cognitive assessment system in theory and practice. In J. J. W. Andrews, D. H. Saklofske, &

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H. L. Janzen (Eds.), Handbook of psychoeducational assessment: Ability, achievement, and behavior in children (pp. 33–63). San Diego, CA: Academic Press. Naglieri, J. A. (1999). Essentials of CAS assessment. New York: Wiley. Naglieri, J. A. (2005). The cognitive assessment system. In D. P. Flanagan & P. L. Harrison (Eds.), Contemporary intellectual assessment: Theories, tests, and issues (2nd ed., pp. 441–460). New York: The Guilford Press. Naglieri, J. A., & Das, J. P. (1997a). Cognitive assessment system: Administration and scoring manual. Itasca, IL: Riverside Publishing. Naglieri, J. A., & Das, J. P. (1997b). Cognitive assessment system: Interpretive handbook. Itasca, IL: Riverside Publishing. Naglieri, J. A., & Das, J. P. (2005). Planning, attention, simultaneous, and successive (PASS) theory: A revision of the concept of intelligence. In D. P. Flanagan & P. L. Harrison (Eds.), Contemporary intellectual assessment: Theories, tests, and issues (2nd ed., pp. 120–135). New York: The Guilford Press. Wechsler, D. (1991). Wechsler intelligence scale for children-third edition. San Antonio, TX: Psychological Corporation. Woodcock, R. W., & Johnson, M. B. (1989). Woodcock–Johnson psychoeducational battery-revised, tests of achievement. Itasca, IL: Riverside.

Definition Cognitive Behavioral Couples Therapy (CBCT) has become one of the most well researched approaches for the treatment of marital and couple distress, with growing empirical support for it effectiveness. Theoretically grounded in both social learning and social exchange theories, the premise of CBCT is that an individual’s behavior both influences and is influenced by his/her environment. When applied to a marriage or other longterm relationship, this premise suggests that one partner’s behavior influences and is influenced by the actions of the other. CBCT typically focuses on two aspects of this process: (a) exchanges of positive and negative behaviors; (b) communication skills that influence the interaction process (Epstein, Baucom, & Daiuto, 1997).

Current Knowledge

Cognitive Assessors ▶ Cognitive Correctors

Cognitive Assistive Technology ▶ Prosthetic Memory Aids

Cognitive Awareness ▶ Metacognition

Cognitive Behavioral Couples Therapy TAMARA G OLDMAN S HER Illinois Institute of Technology Chicago, IL, USA

Synonyms Behavioral marital therapy; CBCT; Cognitive behavioral marital therapy; Couples therapy; Marital therapy

Couples and Health A patient’s ongoing, long-term relationship can influence a range of psychosocial variables related to health behaviors. The health-enhancing properties of intimate and long-term relationships have been repeatedly documented (Kiecolt-Glaser & Newton, 2001; Wilson, 2001). Various mechanisms of action for this relationship have been proposed, including selection and protection (Kiecolt-Glaser & Newton, 2001). That is, healthier people are more likely to be in and stay in intimate relationships, and they tend to have more resources and take care of themselves better than their counterparts without such relationships. Additional research has investigated other mechanisms for the protective benefits of long-term relationships, including partner attitudes or behaviors, and caretaking (Keefe et al., 1996; Wilson, 2001).

Treatment Procedures A CBCT approach to treatment with both relationship distressed and health impaired couples focuses on three factors: behavioral factors, affective/emotional factors, and cognitive factors. The behavioral component includes increasing positive behaviors such as spending more time together and decreasing negative behaviors such as criticizing or nagging. In addition, because communication problems are the most commonly reported presenting complaint of distressed couples, the behavioral aspects of the treatment also typically involve a skillsoriented approach to communication change where the

Cognitive Behavioral Therapy

value and skills of working together to solve a problem are the foci. Affect is a focus of therapy insomuch as it is an indicator of significant relationship distress and for its ability to direct the therapist in exploring links between the emotions of the partners and their behaviors. Affect can be approached with a skills approach in helping partners learn to express their own and listen to the other person’s emotions and by linking specific emotions to specific relationship issues. The third factor in CBCT is cognition. As Epstein et al. (1997) note, ‘‘the importance of cognitive factors in relationship functioning lies in the fact that objectively observable behavioral events are often subjectively experienced quite differently by the partners’’. The therapist works to uncover underling cognitive factors shaping the behavior and affect of the partners in order to increase understanding and promote behavioral change.

Efficacy Information CBCT, and its predecessor, Behavioral Marital Therapy (BMT) have been one of the most researched forms of couples therapy (Shadish & Baldwin, 2003, 2005). Results of efficacy trials repeatedly demonstrate that those who receive either CBCT or BMT report less distress than those who receive no treatment and that this finding remains not only for couples presenting with general marital distress, but also for depression, agoraphobia, and alcohol abuse (Baucom, Shoham, Mueser, Daiuto, & Stickle, 1998). Based upon recent meta-analyses of studies using CBCT, Shadish and Baldwin (2003, 2005) reported an overall mean effect size ranging from 0.59 to 0.84 for couples therapy generally, with no differential effectiveness across theoretical orientation found.

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References and Readings Baucom, D. H., Shoham, V., Mueser, K. T., Daiuto, A. D., & Stickle, T. (1998). Empirically supported couple and family interventions for marital distress and adult mental health problems. Journal of Consulting and Clinical Psychology, 66, 53–88. Epstein, N. B., & Baucom, D. H. (2002). Enhanced cognitive-behavioral therapy for couples. Washington, DC: American Psychological Association. Epstein, N. H., Baucom, D. H., & Daiuto, A. (1997). Cognitive-behavioral couples therapy. In W. K. Halford, & H. J. Markman (Eds.), Clinical handbook of marriage and couples intervention (pp. 415–449). West Sussex, England: Wiley. Halford, W. K., & Markman, H. J. (Eds.). (1997). Clinical handbook of marriage and couples intervention. New York: Wiley. Keefe, F. J., Caldwell, D. S., Baucom, D. H., Salley, A., Robinson, E., Timmons, K., et al. (1996). Spouse-assisted coping skills training in the management of osteoarthritic knee pain. Arthritis Care and Research, 9(4), 279–291. Kiecolt-Glaser, J. K., & Newton, T. L. (2001). Marriage and health: His and hers. Psychological Bulletin, 127(4), 472–503. Schmaling, K., & Sher, T. G. (2000). The psychology of couples and illness: Theory, research, and practice. Washington, D.C.: American Psychological Association. Shadish, W. R., & Baldwin, S. A. (2003). Meta-analysis of MFT interventions. Journal of marital and family therapy, 29, 547–570. Shadish, W. R., & Baldwin, S. A. (2005). Effects of Behavioral Marital Therapy: A Meta-analysis of randomized controlled trials. Journal of Consulting and Clinical Psychology, 73(1), 6–14. Wilson, S. E. (2001). Socioeconomic status and the prevalence of health problems among married couples in later midlife. American Journal of Public Health, 91(1), 131–135.

Cognitive Behavioral Marital Therapy ▶ Cognitive Behavioral Couples Therapy

Qualifications of Providers

Cognitive Behavioral Therapy CBCT can be conducted by a variety of treatment providers, specifically trained in its use with various populations. This includes neuropsychologists, clinical psychologists, marriage and family therapists, counselors, social workers, and clergy.

TARYN M. S TEJSKAL Virginia Commonwealth University Medical Center Richmond, VA, USA

Definition Cross References ▶ Behaviorism ▶ Cognitive Behavior Therapy

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Cognitive behavioral therapy (CBT) is a theoretical framework based on the premise that a person’s cognitions influence their emotions and behavior. CBT

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provides considerable utility in addressing a variety of common emotional consequences of neurological disorders including anxiety and depression, as well as behavior modification for brain injury survivors.

Historical Background CBT grew out of Albert Ellis’s (Ellis, 1975) work on Rational Emotive Behavioral Therapy (REBT) and examination of irrational beliefs in the 1950s. Ellis concluded that irrational beliefs (e.g., I am powerless to solve my problems; I am unlovable) were associated with the development of mood disorders. Beck, Rush, Shaw, and Emery (1979) developed Cognitive Therapy on the premise that cognitive errors (e.g., over-generalizing, magnification, personalization) were associated with the development of depression and anxiety. Further, they viewed depression as accompanied by of a triad of negative cognitions consisting of a negative view of self, the world, and the future. Arnold Lazarus (1971) was the first to introduce the term ‘‘behavior therapy’’ into the professional literature. Further expanding the lens of CBT, Lazarus and Folkman (1984) developed the stress, appraisal, and coping model (1984) that acknowledges the importance of how an individual views the environment (primary appraisal) and their available coping resources (secondary appraisal). Finally, attribution theory, proposed by Fritz Heider (1958) posits that people can interpret events as caused by internal (i.e., factors within the person such as a person’s own intelligence and behavior) or external factors (i.e., factors external to the person such as the weather or luck). When internal attributions are made, people are said to have an internal locus of control, or hold cognitions that support their sense of efficacy in affecting what happens to them. When external attributions are paramount, people are said to have an external locus of control, that is, they believe that they exert less control over their environment.

alleviation. To reduce the emotional experience of frustration, a neuropsychologist working with a brain-injured patient would encourage the patient to appreciate the gains made since the injury. In order to avoid unrealistic expectations for post injury functioning that could create frustration; the patient would be cautioned against comparing present capabilities to preinjury functioning.

Treatment Participants CBT is broadly applicable to a variety of clinical neurological diagnoses including anxiety, depression, obsessive compulsive disorder (OCD), and posttraumatic stress disorder (PTSD). CBT can be used with individuals, couples, and families. Further, CBT can be used with patients at developmental levels from young children to older adults. CBT therapists act more as coaches collaborating with patients alter processes of thinking, feeling, and action as opposed to an analyst making expert interpretations.

Treatment Procedures CBT encompasses a variety of clinical interventions for individuals with a neurological disorder as well as couples and families in which a member has a neurological diagnosis or injury. Within all CBT interventions, behaviors, cognitions, and emotions are integrally related; though interventions may focus on behavior or cognition, these distinctions are often made for heuristic purposes (Epstein & Baucom, 2002). Further, CBT posits that change in one domain will produce changes in others domains. In the past, behavior-oriented therapies have been ripe with skills training in areas such as problemsolving, communication, and relaxation with the idea being that maladaptive patterns arise around areas of skill deficits. Though CBT therapists also deliver a judicious amount of skills-based training, therapy moves beyond behavior modification to the meaning and interpretations made as a result of interactions and experience.

Goals and Objectives Neuropsychologists using CBT as a conceptual framework work toward modifying maladaptive cognitions such as negative attributions, unattainable expectations and standards, and faulty belief systems. Through the modification of these problematic (i.e., schemas), CBT seeks to alter people’s emotional and behavioral responses in service of symptom management, reduction, and

Behavior-Focused Interventions Patients with neurological diagnoses have a host of behavior patterns amenable to CBT intervention. One such pattern, negative reciprocity, is the idea that negative behavior increases the propensity that a person will respond to expressed negative behavior with more


negative behavior (Epstein & Baucom, 2002). For example, family members may respond angrily toward a patient who acts aggressively after a brain injury. Over time negative reciprocity pervades relationships, invading cognitions and emotions such that family members make global negative attributions about another person’s intentions and behavior (Epstein & Baucom, 2002). An important aspect of CBT is skills-based training; meant to enhance positive behavior and decrease negative behavior. In an instance where a person receives a diagnosis of schizophrenia, a CBT clinician may intervene with a family to teach communication skills. Empirical literature has discussed the detrimental impact of expressed emotion (EE) in families with a schizophrenic family member. Further, the family members may be overwhelmed and have many questions, concerns, and emotions they need to express surrounding the diagnosis. Communication skills training teaches families how to communicate more productively with one another about complex issues and reduce detrimental patterns of interaction such as EE.

Cognition-Focused Interventions The manner in which a person cognitively ascribes meaning to behavior is an important factor in CBT. Therapy often focuses on reassessing and amending these cognitions. Areas of inquiry often include attributions, expectancies, assumptions, standards, and beliefs. In order to evaluate and modify existing cognitions, clinicians intervene using guided discovery during which clients are asked to identify and evaluate their cognitions (Epstein & Baucom, 2002). When a patient is depressed about a neurological diagnosis or injury, clinicians may intervene at the cognitive level challenging catastrophic thinking (e.g., I will never get better). A therapist may focus decreasing negative self talk (e.g., I am worthless the way I am now) by encouraging the patient to keep a journal of thoughts occurred and the impact of the thoughts on the patient’s mood (Epstein & Baucom, 2002).

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encouraging healthy compartmentalization of emotion (Epstein & Baucom, 2002). In cases of neurological diagnosis or injury, emotions may either be exacerbated or minimized. To enhance the expression of emotions, therapists may ask probing questions to bring awareness to emotional experience such as: What happens to you when. . .What is it like for you when. . .How do you feel as you listen to your son expressing his experience. . . (Epstein & Baucom, 2002). In this way, individuals and family members can be encouraged to share their emotional experience in the context of a safer therapeutic environment.

Efficacy Information CBT has been empirically validated for the treatment of many disorders including anxiety (Barlow, O’Brien, & Last, 1984), sexual dysfunction (Baucom, Shoham, Mueser, Daiuto, & Stickle, 1998), depression (Beach, Sadeen, & O’Leary, 1990), bipolar disorder, schizophrenia, and bulimia nervosa (Baucom et al., 1998). CBT is often used in conjunction with medication for a variety of mental health concerns. In addition, CBT effectively enhances coping skills for adults with chronic illness (Rybarczyk, DeMarco, DeLaCruz, Lapidos, & Fortner, 2001) and caregivers (Gallagher-Thompson, Lovett, Rose, McKibbin, Coon, et al., 2000). Recently, advances in computer software have given rise to computerized versions of CBT. Though computerized cognitive behavioral therapy (CCBT) is not meant t o replace face-to-face therapy, it does provide an additional treatment option. CCBT allows clients to participate in therapy when there is a paucity of available therapists, the associated costs are prohibitive, or the prospect of speaking to someone face-to-face seems off-putting. In 2006, the United Kingdom’s National Institute of Health and Clinical Excellence (NICE) provided guidelines recommending CCBT as a result of randomized controlled trials for mild to moderate depression and anxiety (NICE, 2006).

Emotion-Focused Interventions

Outcome Measurement Interventions within the realm of emotions may range from expanding minimized emotional experience to containing heightened emotional experience. Clinicians may draw on a variety of strategies to access, heighten, or limit emotional experience including normalizing emotional responses, metaphor, acceptance of emotional expression, enhancing tolerance for distressing emotions, and

Many clinicians used standardized instruments to assess the presence and severity of a variety of neuropsychological diagnoses. With regard to depression, clinicians may use self-report inventories such as the Beck Depression Inventory (BDI II; Beck, Steer, & Brown, 1996) Finally, the Beck Anxiety Inventory (BAI: Kabacoff, Segal, Hersen, &

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Van Hasselt, 1997) has shown considerable utility in diagnosing anxiety and identifying the severity of the anxiety symptoms.

Qualifications of Treatment Providers CBT can be used by a variety of treatment providers such as neuropsychologists, clinical psychologists, marriage and family therapists (MFTs), counselors, and social workers. Treatment providers using CBT should have appropriate clinical training in the model. Further, clinical providers learning to use CBTas a conceptual framework to guide therapy should seek supervision from an experienced individual trained in the model of CBT.

Inventory and the State-Trait Anxiety Inventory with older adult psychiatric outpatients. Journal of Anxiety Disorders, 11(1), 33–47. Lazarus, A. A. (1971). Behavior therapy and beyond. New York: McGrawHill. Lazarus R. S., & Folkman, S. (1984). Stress, appraisal and coping. New York: Springer. Leahy, R. L. (Ed.) (1997). Practicing cognitive therapy: A guide to interventions. Northvale, NJ: Jason Aronson. National Institute of Health and Clinical Intervention (NICE). (2006). Depression and anxiety computerized cognitive behavioral therapy (CCBT). Retrieved on July 17, 2007 at http://guidance.nice.org.uk/ TA97 O’Farrell, T. J., Choquette, K. A., Cutter, H. S. G., Brown, E. D., & McCourt, W. F. (1993). Behavioral marital therapy with and without additional couples relapse prevention sessions for alcoholics and their wives. Journal of Studies on Alcohol, 54, 652–666. Rybarczyk, B., DeMarco, G., DeLaCruz, M., Lapidos, S., & Fortner, B. (2001). A classroom mind-body wellness intervention for older adults with chronic illness: Comparing immediate and one year benefits. Behavioral Medicine, 27, 15–27.

Cross References ▶ Behaviorism ▶ Behavior Modification ▶ Psychotherapy

Cognitive Behaviorism ▶ Behaviorism

References and Readings Barlow, D. H., O’Brien, G. T., & Last, C. G. (1984). Couples treatment of agoraphobia. Behavior Therapy, 15, 41–58. Baucom, D. H., Shoham, V., Mueser, K. T., Daiuto, A. D., & Stickle, T. R. (1998). Empirically supported couples and family therapies for adult problems. Journal of Consulting and Clinical Psychology, 66, 53–88. Beach, S. R. H., Sadeen, E. E., & O’Leary, K. D. (1990). Depression in marriage: A model for etiology and treatment. New York: Guilford Press. Beck, A. T., Rush, A. J., Shaw, B. F., & Emery, G. (1979). Cognitive therapy of depression. New York: Guilford. Beck, A. T., Steer, R. A., & Brown, G. K. (1996). Manual for the Beck Depression Inventory-II. San Antonio, TX: Psychological Corporation. Beck, J. S. (1995). Cognitive therapy: Basics and beyond. New York: Guilford. Dattilio, F. M., & Freeman, A. (Eds.). (2003). Cognitive-behavioral strategies in crisis intervention (3rd ed.). New York: Guilford. Ellis, A. (1975). A new guide to rational living. Englewood Cliffs: Prentice Hall. Epstein, N. B., & Baucom, D. H. (2002). Enhanced cognitive-behavioral therapy for couples. Washington: American Psychological Association. Gallagher-Thompson, D., Lovett, S., Rose, J., McKibbin, C., Coon, D., Futterman, A., & Thompson, L.W. (2000). Impact of psychoeducational interventions on distressed family caregivers. Journal of Clinical Geropsychology, 6, 91–110. Heider, F. (1958). The psychology of interpersonal relations. New York: Wiley. Kabacoff, R. I., Segal, D. L., Hersen, M., & Van Hasselt, V. B. (1997). Psychometric properties and diagnostic utility of the Beck Anxiety

Cognitive Control ▶ Controlled Attention

Cognitive Correctors R ICK PARENTE Towson University Towson, MD, USA

Synonyms Behavioral memory aids; Behavioral prothestics; Cognitive archives; Cognitive assessors; Cognitive monitors; Cognitive orthotics; Cognitive robots; Cognitive trainers; External aids; Prosthetic devices

Definition A device, external to the mind, that enhances or replaces a memory or cognitive function.

Cognitive Functioning

Current Knowledge Parente and Herrmann (2010) defined several different external aids that take over a deficient cognitive or memory function. Table 1 surveys the various types of cognitive correction devices. Cognitive prosthetics refer to a general class of devices that take over some memory process. Examples of particularly useful prosthetics include: color coding, which creates simplistic visual organization; checklists, which improve consistency and sequential ordering; medication organizers, – which organize medications and remind a person which ones to take on each day; notepads and postit notes, –which are useful for quickly writing a message or for placing a written message somewhere where it will be seen later; appointment calendars and diaries, – which remind the person of important appointments and provide a record of daily activities. Cognitive robots carry out a repetitive task for an individual. For example: calculators perform the same mathematical operations; cycling timers turn appliances on and off at preset times; telememo wrist watches remind the person of appointments at the same time every day, week, month, or year; motion-sensitive detectors automatically turn lights on and off when motion or the lack thereof is sensed; smart irons turn themselves off after a short period of disuse. Cognitive Orthotic Devices replace some cognitive function. For example: spelling machines find the correct spelling by keying in a close match; internet search engines search the internet using key words and Boolean logic; grammar checkers check a document for accuracy of grammar.

Cognitive Correctors. Table 1 Kinds of cognitive correctors

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Cognitive trainers and archives teach a skill and/or store large searchable databases. For example: academic remediation software teaches basic academic skills; cognitive remediation software provides drill and practice exercises that improve cognitive skills; skills training software teaches skills like typing or the use of specific software packages (e.g., Microsoft Office). Cognitive archives (e.g., electronic encyclopedias) store large amounts of information and provide a search engine for finding specific topics.

References and Readings Parente, R., & Herrmann, D. (2010). Retraining cognition: Techniques and applications. Austin, TX: ProEd Publishers.

Cognitive Exercises ▶ Homework

Cognitive Flexibility ▶ Mental Flexibility ▶ Stroop Effect

Cognitive Functioning C HRISTINA A. PALMESE Beth Israel Medical Center New York, NY, USA

Behavioral prosthetics

Changes in behavior that are specifically designed to remind a person to do something

Cognitive robot

Carries out the same task consistently and correctly

Cognitive orthotic

Performs a thinking task

Cognitive trainers

Help the person to learn new skills and to practice them

Definition

Cognitive archives

Maintain knowledge and records of past experience so that a person does not have to store and retain this information internally in his or her memory

‘‘Cognitive functioning’’ is a general term used to describe the different ways that people think. It refers to faculties such as attention, mental processing speed, executive functions, language, visual spatial skills, memory, and

Synonyms Cognition; Mentation; Thinking

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fine motor dexterity. Different cognitive functions are supported by distinct cortical and subcortical brain regions. Disruption of neural processes in these brain regions can result in a range of cognitive deficits and syndromes.

Historical Background In the 1600s, Descartes, a philosopher, was one of the first scholars to establish the idea that the brain controls behavior. In the late 1700s, Franz Gall, a forefather of phrenology (the study of behavior based on the size and shape of the skull) helped identify that different parts of the brain regulate distinct aspects of thought, personality, and behavior. Later on in the 1800s and 1900s,Wilder Penfield, Hughlings Jackson, Paul Broca, and Carl Wernicke, to name only a few researchers who contributed significantly to the field of neuropsychology, used epilepsy and lesion models to delineate distinct neuroanatomical correlates of cognitive and motor functions. More recently, functional (e.g., fMRI, PET) and structural (e.g., MRI, CT) neuroimaging techniques have provided an even more well defined understanding of brain-behavior relationships.

Current Knowledge Attention Attention refers to the ability to concentrate on information that is heard and seen in the environment in both the presence and absence of distractions. It also allows us to concentrate on two things at once, such as balancing a checkbook while talking on the phone. It is regulated by the frontal lobes, although pathways involving the pons, parietal lobe, and thalamus are involved in the mediation of attention, as well. Dysfunction along these pathways can contribute to various types of attention problems. Attention is hierarchically organized, and disruption of the most basic attention skills leads to disruption of more complex attention abilities. Focused attention is the most rudimentary level of attention that permits us to concentrate or be vigilant to something in the environment for a very brief time period. Sustained attention refers to maintenance of concentration for minutes or hours. Selective attention requires even greater attention capacity that allows us to attend to a particular task while filtering out irrelevant, background information. For example, this form of attention is used when we read the newspaper and are not distracted by noises such as the

radio or television nearby. Divided attention allows us to pay attention to two or more tasks simultaneously. At the top of the hierarchy is alternating attention, which is the most complex form of attention that involves shifting of attention from one task to another. Many neuropsychological measures have been designed to evaluate attention. One such measure is the digit span test. This task assesses basic attention capacity through repetition of series of numbers of increasing length (e.g., 1-2-3-etc). Continuous performance tests are computerized assessment tools that evaluate attention capacity over an extended time period of 10–15 min. Higher level attention skills can be examined using tests involving mental arithmetic, more complex mental arithmetic, connecting numbers and letters in alternating sequence (e.g., 1-A-2-B-3-etc), and resequencing of numbers and letters in numeric and alphabetic order (e.g., transform ‘‘8-K-2’’ to 2-8-K). Attention problems can be seen across the lifespan. In children, poor concentration, distractibility, and trouble regulating behavior can be related to an Attention Deficit Disorder, a diagnosis that is made when pervasive attention difficulties are demonstrated prior to age seven years and are observable in at least two different environments. However, attention difficulties in children also may develop secondary to anxiety, language disorders, or neurological disorders such as epilepsy. In adults, attention deficits may manifest secondary to a variety of neurological and medical conditions, including subarachnoid hemorrhage, epilepsy, dementia, head injury, diabetes, and hypothyroidism. Stress, depression, and medication side effects often contribute to attention problems in adults, as well.

Mental Processing Speed Mental processing speed, a term used synonymously with reaction time, refers to the speed at which an individual thinks and completes activities. It is largely regulated by the frontal lobes and subcortical regions, and it has global effects on cognition. That is, if mental processing speed is poor slowness also is observed in areas of attention, language, and spatial processing abilities. This mental slowing is referred to as bradyphrenia. Reaction time can be evaluated in several ways. Measures requiring manual transcription of numbers and shapes are very sensitive to bradyphrenia. Other tasks may involve rapid reading of color names and naming of colors, timed symbol search, and rapid generation of words.


Bradyphrenia is commonly observed in medical and neurological conditions across the lifespan, including dementia, brain injury, and Parkinson’s disease.

Executive Functions Executive functioning refers to some of the most highly complex aspects of thinking. It includes skills such as problem solving ability, task execution and efficiency, self-monitoring, strategizing, mental flexibility, planning, and conceptualization. Executive functions are largely mediated by the frontal lobes, and they are highly sensitive to cerebral dysfunction. Deficits in executive functioning can occur secondary to neurodevelopmental immaturity, such as seen in attention deficit disorder. For the most part, however, executive dysfunction is either acquired through brain injury (e.g., stroke, head injury) or associated with neurodegenerative diseases, such as Alzheimer’s disease, multiple sclerosis, and Huntington’s disease.

Language At the most fundamental level, language can be broken down into two categories: receptive and expressive language. Receptive language refers to the ability to understand language; it is mediated by the posterior region of the superior temporal gyrus, which is known as Wernicke’s area. Expressive language, on the other hand, pertains to oral and written expression and it is regulated by the posterior frontal/anterior temporal lobe. This region commonly is referred to as Broca’s area. Deficits in receptive and expressive language can be idiopathic (i.e., of unknown origin) or acquired. If language problems are idiopathic, they typically are identified during a child’s development and are referred to as a developmental language delay. As some children mature, their language difficulties might resolve; however, other children experience residual language deficits throughout adulthood. On the other hand, acquired language deficits such as those resultant from stroke, head injury, and tumor are characterized as aphasia. Depending on the location of brain injury, different aphasia syndromes can be observed. Listed below are three of the most commonly referenced syndromes. Traditionally, language skills in adults are assessed with an aphasia battery (e.g., Boston Diagnostic Aphasia Examination (BDAE)), letter and category fluency, and visual confrontation naming tasks. As one of the most

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Cognitive Correctors. Table 1 Aphasia syndrome

Neuroanatomy Symptom(s)

Broca’s (expressive) aphasia

Posterior, lateral frontal lobe

Wernicke’s (receptive) aphasia

Anterior, lateral Poor comprehension, temporal lobe fluent speech, nonsensical speech content

Anomic aphasia

Temporalparietal border

Poor articulation, telegraphic, non-fluent, limited speech, intact comprehension

Poor naming, intact comprehension, intact fluency

commonly observed language problems in adults is a naming deficit (which is synonymous with word finding difficulty, dysnomia, and ‘‘tip of the tongue’’ experiences), auditory naming tests have proven to be useful for identification of anomia, as well. Similar measures are used during evaluation of pediatric populations. In addition, examination of children also should include assessment of phonics, grammar, syntax, language formulation, and language organization skills.

Visual Spatial Processing Cognitive abilities such as basic spatial perception, visual construction, and nonverbal problem solving ability are characterized as spatial processing skills. These faculties involve identifying ‘‘what’’ and ‘‘where’’ items are in space. The ‘‘what,’’ or ventral, pathway is mediated by the right (nondominant) temporal lobe. Disruption of this pathway by acquired brain injury can result in agnosia, which is a loss of awareness and inability to recognize entities in the environment. Agnosias can be domain specific, affecting auditory, visual, or somatosensory functioning. Prosopagnosia is a unique agnosia in which there is an inability to recognize faces. The ‘‘where’’ or dorsal pathway is regulated by the parietal lobe. Dysfunction in the parietal lobe can contribute to spatial neglect in which a portion of space is neglected or ignored. Neglect can be detected during visual field screening and on line bisection and drawing tasks. An individual with spatial neglect will not respond to information presented in part or all of the visual field. Left hemispatial neglect, in which the entire left side of space is ignored, is the most commonly observed form of neglect that results from damage to the right parietal lobe.

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Cognitive Functioning in the Elderly

Memory

Cross References

Memory involves learning, retrieval, retention, and recognition of information. Memory functions are lateralized, with verbal memory regulated by left (dominant) temporal regions and visual-spatial memory regulated by the right temporal lobe. In order for new learning to occur, we must be able to attend to the material and store it temporarily in our short-term memory. Consolidation of this information into long-term memory stores occurs within the CA1-CA3 regions of the hippocampus, a subcortical brain region rich in acetylcholine. Once learned, information is then stored in various neural networks throughout the brain. Learning deficits can be attributed to depletion of acetylcholine or loss of brain cell within the hippocampus. This type of memory problem typically is associated with cortical dementia, such as Alzheimer’s disease. Cholinesterase inhibitors, which are medications that promote cholinergic availability, are common treatment options to delay or slow the progression of a learning deficit. Retrieval deficits, on the other hand, are characterized by poor spontaneous recall, yet adequate recognition of newly learned material. They are associated with cell loss and neurochemical depletion of dopamine and glutamate in the basal ganglia and other subcortical regions. Retrieval deficits are observed in subcortical dementias, including Parkinson’s disease and Huntington’s disease. Although cholinesterase inhibitors can be used to treat subcortical memory dysfunction, their efficacy is relatively weaker in subcortical versus cortical dementia populations.

▶ Agnosia ▶ Agraphia ▶ Alexia ▶ Amnesia ▶ Anomia ▶ Aphasia ▶ Attention ▶ Learning ▶ Memory ▶ Processing Speed ▶ Syndrome ▶ Verbal Fluency ▶ Visuoperceptual

References and Readings Hamberger, M. J., Goodman, R. R., Perrine, K., & Tamny, T. (2001). Anatomic dissociation of auditory and visual naming in the lateral temporal cortex. Neurology, 56(1), 56–61. Heilman, K. M., & Valenstein, E. (2003). Clinical neuropsychology (4th ed.). New York, NY: Oxford University Press. Kolb, B., & Wishaw, I. (2008). Fundamentals of human neuropsychology (6th ed.). New York, NY: Worth Publishers, Inc. Lezak, M., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York, NY: Oxford University Press. Squire, L. R., & Schacter, D. L. (2002). Neuropsychology of memory (3rd ed.). New York, NY: The Guilford Press.

Cognitive Functioning in the Elderly Motor Motor abilities refer to skills such as hand-eye coordination, manual dexterity, and visual-motor integration. These skills all have contralateral representation within the parietal lobes. That is, right sided motor functions are regulated by left parietal lobe regions, and vice versa. Motor dysfunction can be observed in children with developmental delay, as well as in adults and children with medical conditions including cerebral palsy, spasticity, and dementia. It often manifests as clumsiness, poor handwriting, or trouble tying shoe laces and buttoning shirts. Motor abilities can be evaluated using tasks requiring rapid placement of pegs into a pegboard, drawing, imitation of hand movements, finger tapping, and grip strength.

▶ Normal Aging

Cognitive Impairment No Dementia (CIND) ▶ Mild Cognitive Impairment

Cognitive Log ▶ Cognitive-Log

Cognitive Processing Speed

Cognitive Monitors ▶ Cognitive Correctors

Cognitive Orthotics ▶ Cognitive Correctors ▶ Prosthetic Memory Aids

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psychology approach to better understanding cognitive processing has been through the development of computational models, such as artificial intelligence. In cognitive neuroscience research, cognitive processing concepts are used to explore the relation between brain and behavior, as exemplified by George Miller’s research (1956) on the capacity of short-term memory to hold seven plus or minus two items and Baddeley’s theory (1974) of a central executive, phonological loop, and visuospatial sketchpad.

Current Knowledge

Cognitive Potential ▶ Best Performance Method

Cognitive Processing D ENISE K RCH Kessler Foundation Research Center West Orange, NJ, USA

Definition Cognitive processing is a general term to describe a series of cognitive operations carried out in the creation and manipulation of mental representations of information. Cognitive processes may include attention, perception, reasoning, emoting, learning, synthesizing, rearrangement and manipulation of stored information, memory storage, retrieval, and metacognition. These functions can be conscious (e.g., learning a concept) or unconscious (e.g., learning a skill) and can be internally generated (e.g., recalling a memory) or initiated by a novel sensory input from the environment (e.g., solving a problem). From a cognitive psychology perspective, cognitive processing is approached as a sequence of ordered stages wherein sensory input is transformed, reduced, elaborated, stored, recovered, and utilized. Early views of cognitive processing emphasized linear temporal processing, whereas contemporary models assume a less linear, more complex flow of dynamics, including bottom-up (sensory-driven) and top-down (concept-driven) processes. One cognitive

The cognitive processing model is currently used in the assessment and treatment of learning disabilities, alcohol and drug addictions, and trauma and abuse. This model emphasizes how new information is processed, internalized, and retrieved in the context of a person’s existing mental representations of information, and of his/her beliefs, desires, knowledge, preferences, and intentions.

Cross References ▶ Attention ▶ Cognitive Functioning ▶ Learning ▶ Memory ▶ Metacognition ▶ Perception ▶ Reasoning ▶ Retrieval, Retrieval Techniques ▶ Short Term Memory

References and Readings Coren, S., Ward, L., & Enns, J. (2004). Sensation and perception. New York: Harcourt Brace. Groome, D., Brace, N., Edgar, H., Esgate, A., Pike, G., Stafford, T., et al. (2006). An introduction to cognitive psychology: Processes and disorders (2nd ed.). London: Routledge.

Cognitive Processing Speed ▶ Information Processing Speed

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Cognitive Rehabilitation

Cognitive Rehabilitation M ARIANNE H RABOK , K IMBERLY A. K ERNS University of Victoria Victoria, BC, Canada

Synonyms Cognitive Remediation

Definition Cognitive rehabilitation (CR) can be defined as efforts to promote maximal adaptive cognitive functioning in people with neurologically induced cognitive deficits (Barrett & Gonzalez-Rothi, 2002).

Historical Background The field of CR has grown rapidly over the last few decades, but historically can be traced to the 1800s (Ponsford, 2004; Sohlberg & Mateer, 2001). For example, Broca administered language rehabilitation in the 1800s and until the 1980s most rehabilitation programs focused on remediation of language deficits (as reviewed by Ponsford, 2004). Many further developments in CR were a result of the confluence of societal influences and scientific and technological advances. During WWI, Goldstein established CR programs for brain-injured soldiers. During WWII, Luria advanced the field of CR through his theoretical model of brain functioning, recovery, and rehabilitation (Ponsford, 2004). Advances in medical practice and an increase in the number of survivors of traumatic brain injury (TBI) led to a greater awareness of the needs of people who sustained TBI, and an expansion of the number and focus of CR programs (the ‘‘era of proliferation’’; Coelho, 1997, as cited by Sohlberg & Mateer, 2001). However, current trends in service delivery have resulted in an ‘‘era of consolidation,’’ a term describing the significant downsizing of CR programs. It has been suggested that the reduced length of inpatient stays, outpatient health coverage, and more limited support for CR programs have made evidence and theory-based CR practices increasingly relevant to contemporary practice (Cicerone et al., 2005; Levine & Downey-Lamb, 2002; Sohlberg & Mateer, 2001).

Rationale or Underlying Theory CR is multidisciplinary and draws from a range of fields, including neuropsychology, learning theory, cognitive behavioral therapy and psychotherapy, among many others. One main category of theories underlying CR is that specific to the cognitive or behavioral domain of CR focus. For example, attention rehabilitation programs are based on theoretical models of attention, memory rehabilitation programs on theoretical models of memory, and so on. A theory-driven approach provides a rational and empirical basis for intervention, and guidance on the structure and delivery of CR (Levine & Downey-Lamb, 2002; Sohlberg & Mateer, 2001). Another major theory underlying many forms of CR is neuroplasticity (Kolb & Cioe, 2004), the concept that the brain is amenable to change in structure and function. These changes occur at multiple levels, and are manifest in various ways, including changes at the synapse, changes in neurotransmitter systems, and changes in neural networks (Barrett & Gonzalez-Rothi, 2002; Sohlberg & Mateer, 2001). Neuroplasticity has many implications to CR, including the type and timing of CR, and the effect of environmental factors on recovery of cognitive function following brain injury (Barrett & Gonzalez-Rothi, 2002).

Goals and Objectives CR aims to foster natural recovery, decrease the development of maladaptive patterns, and increase functional recovery (Sohlberg & Mateer, 2001). The primary goal of CR is to help people achieve an optimal level of functioning in the context of impairments. CR emphasizes improving function in everyday contexts, rather than on specific cognitive tasks per se.

Treatment Participants CR has been used with a variety of populations, including but not limited to: TBI, stroke, acquired brain injury (ABI) of varying etiology, developmental disorders, Alzheimers’ dementia, and schizophrenia. CR has been most commonly used among people who have sustained TBI and stroke. Research on CR aimed at neurocognitive deficits associated with schizophrenia has been growing over the last 10 years (Kurtz & Nichols, 2007). Variables contributing to the pattern and degree of recovery following brain injury include: demographic


(e.g., age, education, gender, culture), injury-related (e.g., time since injury, extent, and severity of injury), and psychological characteristics (e.g., therapeutic alliance, comorbid psychological disorders, awareness).

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rapid recovery than diffuse injury (Sohlberg & Mateer, 2001).

Psychological Factors Demographic Variables Age : Younger adults show better levels of recovery than older adults (Teuber, 1975 as cited by Sohlberg & Mateer, 2001). ABI in older adults may be complicated by a number of factors, including the superimposition of effects of ABI on declining cognitive abilities (Richards, 2000 as cited by Sohlberg & Mateer, 2001), and psychosocial difficulties more prevalent in the population, including reduced levels of social support and financial resources (Goleburn & Golden, 2001). However, it has also been suggested that older adults often have a greater degree of stability, coping skills, fewer life demands, and effective compensatory techniques, which may be helpful to promoting recovery (Sohlberg & Mateer, 2001). Education and Intelligence: Premorbid intelligence and education are significantly related to recovery and adjustment (Anson & Ponsford, 2006). Gender: Some research suggests that women have better recovery following left hemisphere lesions than men (Kimura, 1983), and circulating sex hormones have also been shown to have neuroprotective effects (e.g., Roof, Duvdevani, & Stein, 1993). Culture: Culture influences beliefs regarding the nature and cause of loss, service utilization, degree of personal responsibility for health, role of family, and many other facets of psychological and behavioral functioning relevant to recovery and participation in CR (Sohlberg & Mateer, 2001).

Injury Related Variables Time since Injury: Spontaneous recovery typically occurs at a faster rate immediately following brain injury, particularly within the first 6 months, with significant recovery also occurring up to 2 years following injury (Sohlberg & Mateer, 2001). However, it is important to note that compensatory techniques can be implemented and underlying motor and cognitive skills improved years after injury (e.g., Shaw et al., 2005). Extent and Severity of Injury: Relatively mild injuries are associated with faster recovery rate and better outcomes. Focal injuries are often associated with more

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Therapeutic Alliance: CR should be an interactive partnership between the client, their significant others, and the therapist. Cultivating a relationship characterized by attentiveness, respect, trust, commitment, and rapport is a critical component of CR. Open communication and involvement of the client and family in goal setting can also enhance engagement in rehabilitation (Sohlberg & Mateer, 2001). Comorbid Psychological Disorders: Depression and anxiety are frequently associated with brain injury (e.g., Anson & Ponsford, 2006). These can impede CR and adjustment following injury due to their propensity to decrease motivation and contribute to a feeling of hopelessness (Sohlberg & Mateer, 2001). Awareness: Lack of awareness can occur following brain injury, and has been associated with poor selfregulation, disengagement in CR programs (Allen & Ruff, 1990), and poorer outcome. Although these factors provide important information related to the pattern and degree of recovery following brain injury, it should also be noted that much more research is needed to better identify therapy factors and client characteristics that optimize clinical outcomes of CR (Cicerone et al., 2005).

Treatment Procedures Examples of domains that have been a focus of CR include: attention, memory, language, visuoperceptual difficulties, executive functions, and socioemotional and behavioral disturbances. CR encompasses a range of interventions. These can be broadly divided into two types of techniques. The first are those that aim to restore or enhance function, by targeting the underlying impairment (Glisky & Glisky, 2002; Sohlberg & Mateer, 2001). For example, Attention Process Training (APT) is a theoretically-driven program that contends that attention can be improved through repeated activation of attentional systems (Sohlberg & Mateer, 1987, 2001). APT consists of a group of hierarchically organized tasks that exercise different components of attention (e.g., sustained, selective, alternating, divided attention). In CR of memory deficits, restorative/generalized memory approaches aim to improve specific memory systems

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across tasks and contexts (e.g., prospective memory training, Raskin & Sohlberg, 1996 as cited by Sohlberg & Mateer, 2001). Various approaches to executive function rehabilitation provide practice in executive skills (e.g., planning) and guiding behavior through self-talk (e.g., self instructional training, Cicerone & Giacino, 1992). The second category of CR interventions is compensatory techniques, which aim to compensate for, or bypass deficits (Sohlberg & Mateer, 2001; Wilson & Zangwill, 2003). These include environmental supports (e.g., organization of physical space, manipulation of physiological factors such as sleep, nutrition etc.) and external aids (e.g., calendars, pagers, checklists, etc.; Manly, Ward, & Robertson, 2002; Wilson & Zangwill, 2003). Compensatory techniques can be helpful in managing diverse types of cognitive difficulties (Sohlberg & Mateer, 2001). A third approach involves the use of specialized approaches to teaching and stabilizing new behaviors and knowledge in people with memory difficulties. These include instructional techniques such as errorless learning, in which mistakes are minimized (Wilson, Baddeley, Evans, & Shiel, 1994), the method of vanishing cues (Glisky & Glisky, 2002) and traditional behavioral shaping and training techniques. Psychosocial support or psychotherapy (e.g., supported listening, brain injury education, relaxation training) can also be an integral part of a rehabilitation program, depending on the needs of the client (Sohlberg & Mateer, 2001). Computer programs can be used as an adjunct to CR, but should not be the sole form of CR (Cicerone et al., 2005). It has been recommended that computer CR programs be focused, structured, monitored, and ecologically valid. A successful rehabilitation program typically involves a combination of interventions, specifically tailored to the individual’s level of disability and personal goals (Manly et al., 2002; Solhberg & Mateer, 2001). The duration and frequency of CR varies widely (e.g., Geusgens, Winken, van Heugten, Jolles, & van den Heuvel, 2007; Kurtz & Nichols, 2007). CR has been delivered both on an individual and on a group basis. Significant others (e.g., family) are viewed as an integral part of treatment (Sohlberg & Mateer, 2001).

Efficacy Information Cicerone et al. (2005) examined differential treatment effects of CR compared to alternate treatment conditions in 46 class I studies with TBI and stroke populations. Their review suggested a clear differential benefit of CR

compared to a number of alternate treatment conditions, with no comparison demonstrating a benefit for an alternate treatment condition. Although implementation of CR programs results in positive change, a number of methodological problems have been identified in the CR literature, including but not limited to: 1. Variability in client characteristics and treatment settings 2. Insufficient description of samples 3. Small sample sizes 4. Inadequate description of interventions 5. A lack of standardized treatment protocols and treatment approaches (including type of intervention, length, and intensity) 6. Lack of appropriate control conditions (e.g., no treatment or alternate treatment) 7. Lack of uniform outcome measures Evidence-based standards of CR are frequently identified as important in advancing the field of CR, both in terms of quality of treatment and for fiscal support at an organizational level (e.g., Sohlberg & Mateer, 2001). Cicerone et al. (2005) reviewed evidence for CR interventions used with TBI and stroke populations. A number of interventions with strong empirical support were identified. Strategy training (e.g., APT or Time Pressure Management) was effective during postacute rehabilitation for TBI. A number of visuospatial rehabilitation techniques were empirically supported for improving neglect following right hemisphere stroke (e.g., visuospatial rehabilitation, scanning training). Specific gestural or strategy training was found to be effective in the treatment of apraxia following left hemisphere stroke. A number of language interventions were shown to have empirical support, including cognitive linguistic therapies, pragmatic conversational skills, interventions for specific language impairments (e.g., reading comprehension), and adjunctive computer-based interventions. Strong empirical support was shown for memory strategy training (e.g., visual imagery and external aids, such as memory notebooks). Some empirical support was found for selfinstruction and self-monitoring interventions for executive functions. Empirical support was found for comprehensive-holistic CR programs, which address multiple aspects of impairment. Interventions that meet criteria for inclusion as an empirically supported treatment do not represent an exhaustive list of CR interventions that may be effective, but reflect the empirical status of the field of CR research and practice. A successful rehabilitation program typically


involves a combination of interventions, specifically tailored to the individual’s level of disability and personal goals (Manly et al., 2002; Solhberg & Mateer, 2001). Therefore, clinical considerations may demand CR interventions with strong empirical support, as well as those that have less empirical support but may be beneficial to the client. A discussion of limitations and strengths of empirically supported and empirically validated interventions can be found elsewhere (e.g., Chambless & Ollendick, 2001). There is less empirical study of CR with populations beside TBI and stroke. A review of CR with children who have sustained ABI concluded that there is a sufficient amount of evidence to recommend attention remediation, involvement of family members in the treatment plan, and provision of psychoeducation to guardians (Laatsch et al., 2007). A review of studies of CR with people with early stage Alzheimer’s disease suggested CR could be beneficial, particularly when flexible to the needs of clients, of sufficient duration, and involving caregivers (Clare, 2003). A review of studies of CR with people with schizophrenia has suggested improvements on memory and executive functions, although it should be noted that this is an emerging field so is based on a small number of studies (Kurtz & Nichols, 2007). A review of CR for people with multiple sclerosis suggested empirically based support for verbal learning and memory intervention, and suggested additional research support is needed (O’Brien, Chiaravalloti, Govereover, & DeLuca, 2008).

Outcome Measurement Levine and Downey-Lamb (2002) recommend inclusion of both specific outcome measures that relate closely to the construct addressed by the intervention, and well as general measures of functional outcomes, such as return to work/school, interpersonal relationships, and leisure activities. When possible, measures should have known psychometric properties, and be completed by both the client and significant others. Neuropsychological assessment can be a valuable tool at various points to: predict outcome, guide appropriate rehabilitation strategies, guide vocational and educational planning, explain behavior, and help evaluate the extent of injury in conjunction with other information (Bergquist & Malec, 2002). Measurement of transfer of training to everyday life has been measured through assessment of performance on tasks similar to tasks used during training,

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standardized observations of simulated performance of daily tasks in a laboratory environment, and standardized and nonstandardized reports of transfer to daily functioning (Geusgens et al., 2007). There is a need for further development of standardized measure of transfer with good psychometric properties (Geusgens et al., 2007). It has been recommended that generalization should not be ‘‘expected,’’ but should be ‘‘programed’’ throughout the CR program (Sohlberg & Mateer, 2001).

Qualifications of Treatment Providers CR is typically provided by registered psychologists, and occupational therapists. Speech and language therapists typically provide rehabilitation for language and communication deficits.

Cross References ▶ Assistive Technology ▶ Attention Training ▶ Behavioral Memory Aids ▶ Brain Plasticity ▶ Compensatory Strategies ▶ Environmental Modifications ▶ Errorless Learning ▶ Head Injury ▶ Insight, Effects on Rehabilitation ▶ Interdisciplinary Team Rehabilitation ▶ Memory ▶ Neglect and Heminattention ▶ Neuropsychological Rehabilitation ▶ Prosthetic Memory Aids ▶ Rehabilitation Psychology ▶ Traumatic Brain Injury

References and Readings Allen, C. C., & Ruff, R. M. (1990). Self-rating vs. neuropsychological performance of moderate vs. severe head-injured patients. Brain Injury, 4, 7–17. Anson, K., & Ponsford, J. (2006). Coping and emotional adjustment following traumatic brain injury. The Journal of Head Trauma Rehabilitation, 21, 248–259. Barrett, A. M., & Gonzalez-Rothi, L. J. (2002). Theoretical bases for neuropsychological interventions. In P. J. Eslinger (Ed.), Neuropsychological interventions: Clinical research and practice (pp. 16–37). London: The Guilford Press. Bergquist, T. F., & Malec, J. F. (2002). Neuropsychological assessment for treatment planning and research. In P. J. Eslinger (Ed.),

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Neuropsychological interventions: Clinical research and practice (pp. 38–58). London: The Guilford Press. Chambless, D. L., & Ollendick, T. H. (2001). Empirically supported psychological interventions: Controversies and evidence. Annual Review of Psychology, 52, 685–716. Cicerone, K. D., Dahlberg, C., Malec, J. F., Langenbahn, D. M., Felicetti, T., Kneipp, S., et al. (2005). Evidence-based cognitive rehabilitation: Updated review of the literature from 1998 through 2002. Archives of Physical Medicine Rehabilitation, 86, 1681–1692. Cicerone, K. D., & Giacino, J. T. (1992). Remediation of executive function deficits after traumatic brain injury. Neurorehabilitation, 2, 73–83. Clare, L. (2003). Cognitive training and cognitive rehabilitation for people with early-stage dementia. Reviews in Clinical Gerontology, 13, 75–83. Geusgens, C. A. V., Winken, S. I., van Heugten, C. M., Jolles, J., & van den Heuvel, W. J. A. (2007). Occurrence and measurement of transfer in cognitive rehabilitation: A critical review. Journal of Rehabilitation Medicine, 39, 425–439. Glisky, E. L., & Glisky, M. L. (2002). Learning and memory impairments. In P. J. Eslinger (Ed.), Neuropsychological interventions: Clinical research and practice (pp. 137–162). London: The Guilford Press. Goleburn, C. R., & Golden, C. J. (2001). Traumatic brain injury outcome in older adults: A critical review of the literature. Journal of Clinical Geropsychology, 7, 161–187. Kimura, D. (1983). Sex differences in cerebral organization for speech and practice functions. Canadian Journal of Psychology, 37, 19–35. Kolb, B., & Cioe, J. (2004). Neuronal organization and change after neuronal injury. In J. Ponsford (Ed.), Cognitive and behavioral rehabilitation: From neurobiology to clinical practice (pp. 7–29). London: The Guilford Press. Kurtz, M. M., & Nichols, M. C. (2007). Cognitive rehabilitation for schizophrenia: A review of recent advances. Current Psychiatry Reviews, 3, 213–221. Laatsch, L., Harrington, D., Hotz, G., Marcantuoro, J., Mozzoni, M. P., & Walsh, V., et al. (2007). An evidence-based review of cognitive and behavioral rehabilitation treatment studies in children with acquired brain injury. Journal of Head Trauma Rehabilitation, 22, 248–256. Levine, B., & Downey-Lamb, M. M. (2002). Design and evaluation of rehabilitation experiments. In P. J. Eslinger (Ed.), Neuropsychological interventions: Clinical research and practice (pp. 80–104). London: The Guilford Press. Manly, T., Ward, S., & Robertson, I. (2002). The rehabilitation of attention. In P. J. Eslinger (Ed.), Neuropsychological interventions: Clinical research and practice (pp. 105–136). London: The Guilford Press. O’Brien, A., Chiaravalloti, N. D., Govereover, Y., & DeLuca, J. (2008). Evidenced based cognitive rehabilitation for persons with multiple sclerosis: A review of the literature. Archives of Physical Medicine and Rehabilitation, 89, 761–769. Ponsford, J. (2004). Introduction. In J. Ponsford (Ed.), Cognitive and behavioral rehabilitation: From neurobiology to clinical practice (pp. 1–6). London: The Guilford Press. Roof, R. L., Duvdevani, R., & Stein, D. G. (1993). Gender influences outcome of brain injury: Progesterone plays a protective role. Brain Research, 607, 333–336. Shaw, S. E., Morris, D. M., Uswatte, G., McKay, S., Meythaler, J. M., & Taub, E. (2005). Constraint-induced movement therapy for recovery of upper-limb function following traumatic brain injury. Journal of Rehabilitation Research and Development, 42, 769–778.

Sohlberg, M. M., & Mateer, C. A. (1987). Effectiveness of an attention training program. Journal of Clinical and Experimental Neuropsychology, 19, 117–130. Sohlberg, M. M., & Mateer, C. A. (2001). Cognitive rehabilitation: An integrated neuropsychological approach. New York: Guilford Press. Wilson, B. A., Baddeley, A. D., Evans, J. J., & Shiel, A. (1994). Errorless learning in the rehabilitation of memory impaired people. Neuropsychological Rehabilitation, 4, 307–326. Wilson, B. A., & Zangwill, O. (Eds.). (2003). Neuropsychological rehabilitation: Theory and practice. Exton, PA: Psychology Press.

Cognitive Remediation ▶ Cognitive Rehabilitation ▶ Neuropsychological Rehabilitation

Cognitive Reserve J ESSE C HASMAN Brown University Providence, RI, USA

Synonyms Brain reserve; Neural compensation; Neural reserve

Definition Cognitive reserve is a concept often used to describe how individual differences mediate the clinical expression of brain damage. In this context, some individuals may cope better than others and function within relatively normal limits, despite the presence of neuropathology.

Historical Background Historically, one of the earliest observations of cognitive reserve was described in a study that found characteristic senile plaques and neurofibrillary tangles commonly associated with Alzheimer’s pathology present in healthy, cognitively unimpaired elderly (Blessed, Tomlinson, & Roth, 1968). Similar observations between brain pathology and performance variability frequently have been described in the extant literature.

Cognitive Stimulation

Current Knowledge While the underlying mechanisms that support cognitive reserve remain unclear, current theories focus on how the brain may develop alternative or more efficient networks to compensate for pathology. One theory proposed by Satz (1993) holds that brain mass and neuronal count – or brain reserve capacity (BRC) – may raise or lower the brain’s threshold to withstand a lesion or degenerative process. In this model, lower BRC would make an individual more vulnerable to neurological insult and increase test sensitivity in detecting impairment. Similarly, greater BRC would provide a higher threshold before the effects of neuropathology are observed. For instance, one study (Katzman et al., 1988) compared a high-functioning group of nursing home residents with Alzheimer’s pathology with a group of healthy nursing home controls. When compared across several cognitive domains, the Alzheimer’s group performed at or above the levels of healthy controls. In this study, the observed cognitive deficits were much lower than predicted, given the level of brain pathology in these individuals. This was explained, in part, by the finding that those in Alzheimer’s group had larger brains with more neurons than the control group, which may have helped those individuals compensate for their neuropathology. Genetics may play a primary role in building greater BRC by increasing overall brain mass, synaptic density, or neurogenesis, all of which could provide greater resiliency against lesions or a degenerative process. By contrast, the concept of cognitive reserve suggests that individual differences in genetics or life experiences provide a buffer to the effects of brain disease, including dementia. Neural reserve or neural compensation models (Stern, 2002) are proposed as potentially active processes that facilitate the brain’s attempts to adapt to disease pathology. Various lines of animal studies have supported this model noting the benefits of enriched environments on neurogenesis and plasticity in the brain. In humans, years of education and occupational attainment are associated with the preservation of cognitive functions. For instance, individuals with lower education often demonstrate clinical manifestations of dementia earlier in the disease process compared to those with higher education (Stern, 2003). One of the important implications of the cognitive reserve construct is that lifetime experiences can influence individuals’ level of functioning. It is important to highlight that current research does not suggest that cognitive reserve prevents neuropathology from developing. Rather, it is believed that a lifetime spent engaging in stimulating

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activities, such as education, occupation, exercise, or social involvement, may provide buffers within neural networks to help delay the behavioral symptoms associated with brain pathology.

C Future Directions An important implication of this concept may be the development of interventions that aim enhances cognitive reserve. In aging research, for instance, growing interest has revolved around the effectiveness of brain ‘‘training’’ games, exercise, or lifestyle modifications that may strengthen and/or expand neural networks, and improve cognitive functioning.

Cross References ▶ Brain Plasticity ▶ Brain Reserve Capacity

References and Readings Blessed, G., Tomlinson, B. E., & Roth, M. (1968). The association between quantitative measures of dementia and of senile change in the cerebral grey matter of elderly subjects. The British Journal of Psychiatry, 114(512), 797–811. Katzman, R., Terry, R., DeTeresa, R., Brown, T., Davies, P., Fuld, P., et al. (1988). Clinical, pathological, and neurochemical changes in dementia: A subgroup with preserved mental status and numerous neocortical plaques. Annals of Neurology, 23(2), 138–144. Satz, P. (1993). Brain reserve capacity on symptom onset after brain injury: A formulation and review of evidence for threshold theory. Neuropsychology, 7(3), 273–295. Stern, Y. (2003). The concept of cognitive reserve: A catalyst for research. Journal of Clinical and Experimental Neuropsychology, 25(5), 589–593. Stern, Y. (2006). Cognitive reserve: Theory and application (studies on neuropsychology, neurology, and cognition). New York: Psychology Press.

Cognitive Robots ▶ Cognitive Correctors

Cognitive Stimulation ▶ Reality Orientation

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Cognitive Trainers

Cognitive Trainers ▶ Cognitive Correctors

Cognitive-Behavioral Modification ▶ Behavior Modification

Cognitive-Communication Disorder S ARAH S. C HRISTMAN B UCKINGHAM The University of Oklahoma Health Sciences Center Oklahoma, OK, USA

Synonyms Cognitive-communication impairment; Language of confusion; Language of generalized intellectual impairment; Right hemisphere impairment/disorder

nondegenerative and degenerative neurologic diseases (including the dementias)’’ (ASHA, 2005). CCDs were recognized in the early 1980s as research mounted, illustrating the interdependency of cognition and language, particularly with regard to the roles of attention and memory in language processing and with regard to the pervasive impact that cognitive impairments have on functional communication abilities (Bayles & Tomoeda, 2007; Myers & Blake, 2008; Ylvisaker, Szekeres, & Feeney, 2008). Speech-language pathologists (SLPs) now routinely assess and treat those aspects of cognition that either support, or are influenced by, speech, language, and communication; other clinicians such as neuropsychologists, rehabilitation psychologists, and cognitive remediation specialists may do so as well.

Categorization CCDs are differentiated from linguistic impairments (aphasias) and from motor speech disorders (dysarthrias and apraxia of speech) by the use of overly concrete, poorly organized, and socially insensitive communication despite preserved speech and language skills. CCDs may be caused and/or complicated by impairments of attention, memory, executive functions, and pragmatics. Symptoms will vary by etiology, patterns of brain damage, and individual differences in the neural organization of cognitive functions.


Epidemiology

The American Speech-Language-Hearing Association (ASHA) has defined cognitive-communication disorders (CCDs) as those that, ‘‘. . .encompass difficulty with any aspect of communication that is affected by disruption of cognition. Communication may be verbal or nonverbal and includes listening, speaking, gesturing, reading, and writing in all domains of language (phonologic, morphologic, syntactic, semantic, and pragmatic). Cognition includes cognitive processes and systems (e.g., attention, perception, memory, organization, executive function). Areas of function affected by cognitive impairments include behavioral self-regulation, social interaction, activities of daily living, learning and academic performance, and vocational performance. Cognitive-communication disorders may be congenital or acquired. Congenital etiologies include but are not limited to genetic disorders and pre, peri, and postnatal neurologic injuries and diseases. Acquired etiologies include but are not limited to stroke, brain tumor, traumatic brain injury, anoxic or toxic encephalopathy, and

Approximately 1.4 million individuals will suffer strokerelated CCDs each year (CDC, 2007; Tomkins, 1995). One to two million children will acquire post-traumatic CCDs annually (Blosser & DePompei, 2003; CDC, 2001; Ylvisaker et al., 2008), whereas adult and elderly individuals will sustain head injuries at rates approximating 100–200 per 100,000, respectively (Bruns & Hauser, 2003). The number of individuals expected to be living with dementia of the Alzheimer’s type (DAT), and thus living with CCDs, is expected to increase from 4 million (in the year 2000) to 31.2 million people by the year 2050.

Natural History, Prognostic Factors, and Outcomes The natural history and prognosis for improvement of cognitive-communication dysfunction are tied to many factors, including etiology, initial severity of disorder,

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the presence or absence of comorbid illnesses, and the nature of specific individual variables such as age, gender, and psychological state. In the absence of confounding circumstances, improvement of CCD is anticipated when caused by stroke, excisable tumor, remitting disease, or traumatic brain injury. When caused by progressive debilitating conditions such as non-excisable tumors or dementing diseases, CCD will worsen over time. CCD of greater initial severity has a poorer prognosis than CCD of mild or moderate initial severity, although in the case of recovery from TBI, the degree of functionality at hospital discharge may be more predictive than the initial severity of injury (Testa, Malec, Moessner, & Brown, 2005). The presence of co-occurring illnesses may compromise the speed and extent of recovery from CCD at any severity level; CCDs arising from traumatic injuries causing diffuse brain damage, for example, are likely to be accompanied by paralysis, motor speech disorders, and injuries to vital organs. Recovery from CCD differs by etiology. Recovery after stroke is usually most rapid in the first 3 months post onset. Recovery from thrombo-embolic stroke may continue after 6 months post onset, whereas recovery from hemorrhagic stroke may plateau at the 6-month point. Functional improvement in cognitivecommunication abilities after severe traumatic brain injury is generally slower at the outset when compared with stroke and typically proceeds in a stairstep fashion over months or years. Recovery from mild stroke or brain injury often seems rapid in comparison. Many individuals with mild brain injuries appear to recover quickly (Ylvisaker et al., 2008), but as many as 15–20% suffer from persistent fatigue and reduced information processing speed for years after injury. Frequently unrecognized and untreated, these deficits cause lifelong cognitive challenges that threaten social adjustment and successful community reentry. In contrast to stroke and TBI, recovery from dementing illness is not expected. Early stages of cognitive decline in DAT are accompanied by forgetfulness, word-finding difficulties, and changes in social pragmatics; mid stages are characterized by increased memory loss, anomia, and social withdrawal; and late stages are associated with loss of most useable cognitive and physical functions (Bayles & Tomoeda, 2007).

Neuropsychology and Psychology of Cognitive-Communication Disorder Damage to the prefrontal and frontal association regions of the right (or nonlanguage-dominant) hemisphere

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of the cerebrum may cause difficulty with pragmatics, context-sensitive semantics, and expressive affective prosody, obscuring indications of mood and compromising the ability to communicate successfully in social situations. Egocentrism may impair recognition of these problems and reduce insight into the communication needs of others. Deficits in vigilance, sustained and selective attention, and/or in attention switching can cause salient information to be missed, and reduce the sequential or simultaneous processing of information from multiple sources. Difficulties drawing inferences, extracting themes and topics in discourse, interpreting nonliteral language, and/or reading affective and prosodic cues for meaning during conversational exchanges may be present (Ylvisaker et al., 2008). A tangential communication style often emerges, where excessive, vaguely relevant details are inserted inappropriately into narratives and discourse (Myers & Blake, 2008). Damage to the right parietal association cortex and to the right parietal-temporal-occipital (PTO) cortex can lead to contralateral inattention, impeding reading, writing, and listening for stimuli in left hemispace. Lesions in PTO cortex can also cause visual-spatial perception and recognition deficits (including topographical and geographical agnosias), which can impair navigation in familiar environments if verbal mediation strategies are not used to compensate. Damage to secondary (superior temporal) association cortex may reduce the efficiency of auditory language processing if stimuli are complex or if they must be processed quickly. Damage may also impair the interpretation of affective prosody when produced by others. Lesions to prefrontal, parietal, and temporal cortex have been associated with anosognosia, the failure to recognize the existence or presence of illness and a problem that can reduce compliance with treatment activities (Myers, 2001; Myers & Blake, 2008; Tomkins, 1995). When damage to the cerebral cortex is bilateral, deficits across multiple systems may interact to impair communication to different degrees. Bilateral cortical damage, for example, can cause impairments in the selfregulation of communicative behaviors that range from failure to organize discourse efficiently to failure to inhibit inappropriate utterances and actions. It can also diminish the ability to focus attention and memory so as to make them useful during conversational exchanges (Ylvisaker et al., 2008). However, if bilateral damage involves subcortical hippocampal structures, then fundamental disruptions of declarative (semantic, episodic, and lexical) and explicit memory may occur. Hippocampal damage/deterioration is common with brain injury/ dementing disease, and it can lead to a host of

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impairments ranging from difficulty learning new information to the presence of a severe, unremitting amnesia. Bilateral damage to lower brain stem reticular activating circuits can severely compromise arousal, alertness, and awareness. This can lead to brief losses of consciousness or to intractable coma. When caused by dementing disease, coma is most likely followed by death. However, recovery from traumatic coma frequently leads to return of function through increasing levels of responsivity, communication, orientation, self-regulation, and cognitive integration. A common sequela of traumatic brain injury is a period of post-traumatic amnesia (PTA), that is, inability to form new memories of events happening after brain injury with disorientation to time, place, or person. In individuals who are verbal, confabulation may be present until disorientation and confusion diminish. As cognitive functioning improves, individuals with brain injuries will benefit from environmental structure and external direction to support increasingly purposive, flexible, and goal-oriented behavior.

Evaluation Evaluation of a suspected CCD requires examination of cognition as it affects and interacts with skills of speech planning and execution, language comprehension and production, and pragmatic/discourse aspects of communication in everyday social contexts (Turkstra, Cohelo, & Ylvisaker, 2005). Tests of cognitive-communication skills are used to evaluate the effects that deficits in attention, orientation, perception, memory, organization, and executive functions can have on communication. Examples of tasks frequently included in formal and informal assessments are provided for illustration: 1. Attention to left hemispace is frequently tested with line-bisection, cancellation, and drawing tasks as well as with more complex reading, writing, and listening tasks that require individuals to process communication stimuli from both right and left sides of body midline. 2. Inferencing abilities are tested by asking patients to interpret humor, to recognize indirect requests for actions, and to follow the themes of conversations. 3. Orientation is assessed by asking patients to respond to questions about time, place, and person. 4. Memory for facts, events, and procedures is evaluated with yes/no questions, narratives, and performance of familiar routines.

5. The ability to discriminate relevant from irrelevant detail and integrate disparate parts into a coherent whole can be evaluated with scene description tasks. 6. Cognitive flexibility and functional problem-solving abilities can be assessed with tasks that require the generation of multiple strategies for achieving a goal and that require repairs of failed communicative interchanges with others (Myers, 1999). Standardized tests used to assess cognitive-communication functions subsequent to right hemisphere impairment include the Mini-Inventory of Right Brain Injury (Pimental & Kingsbury, 1989), The Rehabilitation Institute of Chicago Clinical Management of Right Hemisphere Dysfunction (Halper, Cherney, & Burns, 1996), and The Burns Brief Inventory of Communication and Cognition (Burns, 1997). While these instruments will elicit symptoms of CCD associated with right hemisphere damage, findings must be interpreted with a view toward evaluating impairments of underlying neuropsychological processes, for it is here that treatment will be most profitably directed (Myers, 1999). Standardized tests used to assess CCD after TBI include the American Speech-Language-Hearing Association Functional Assessment of Communication Skills in Adults (Frattali, Thompson, Holland, Wohl, & Ferketic, 1995), the Behavioral Rating Inventory of Executive Function (Roth, Isquith, & Gioia, 2005), the Brief Test of Head Injury (Helm-Estabrooks & Hotz, 1991), the Ross Information Processing Assessment-2 (Ross-Swain, 1996), and the Scales of Cognitive Ability for Traumatic Brain Injury (Adamonovich & Henderson, 1992). The Glasgow Coma Scale (Jennett & Teasdale, 1981) and the Rancho Los Amigos Levels of Cognitive Function Scale (Hagen, Malkmus, Durham, & Bowman, 1979) or its revisions, including a version adapted for children under 14 years of age (Blosser & DePompei, 2003), are frequently used to assess consciousness and track recovery of cognitive functions after coma. Informal situational assessments of cognition and communication may yield valuable information that cannot be obtained from formal standardized tests (Blosser & DePompei, 2003; Turkstra et al., 2005). This naturalistic approach to assessment has been termed, ‘‘functional, collaborative, context-sensitive, hypothesis-testing assessment,’’ and it should be conducted with the purpose of identifying situational variables that can be manipulated to improve the successful participation of injured individuals as they operate within their everyday environments (Ylvisaker et al., 2008). Best-practice assessment procedures for individuals who have suffered brain injuries


should, therefore, include (1) completion of formal testing in specific skill domains and (2) completion of observational checklists (or quality of life inventories) where children (or adults) may be evaluated in natural environments (Blosser & DePompei, 2003; Turkstra et al., 2005). This type of assessment might include administration of the Functional Assessment of Verbal Reasoning and Executive Strategies test (MacDonald, 2005), a tool that was developed for evaluation of cognitive-communication skills specifically related to reading, writing, and reasoning (Turkstra et al., 2005), and the Quality of Communication Life Scale (Paul et al., 2005). Assessment of CCD in dementia employs standardized screening tests, severity staging instruments, and comprehensive assessment batteries (Hopper & Bayles, 2008). Screening tests include the Story Retelling Subtest of the Arizona Battery for Communication Disorders of Dementia (Bayles & Tomoeda, 1993) and the FAS Verbal Fluency Test (Borkowski, Benton, & Spreen, 1967). Tests for estimating the severity of cognitive decline include the Mini-Mental State Examination (Folstein, Folstein, & McHugh (1975)) and the Global Deterioration Scale (Reisberg, Ferris, deLeon, & Crook (1982)). Comprehensive assessment batteries include the Arizona Battery for Communication Disorders of Dementia (Bayles & Tomoeda, 1993) and the Functional Linguistic Communication Inventory (Bayles & Tomoeda, 1994), the latter being most useful for individuals with severe dementia (Hopper & Bayles, 2008). Assessment findings can help families work with SLPs to understand and compensate for the symptoms of cognitive decline.

Treatment Treatment for CCD has traditionally been decontextualized (implemented in rehabilitation settings or at bedside) and deficit oriented (designed to improve/ support impaired cognitive-communication processes). Conventional pencil/paper or computer tasks are used to stimulate underlying cognitive processes (e.g., attention, memory) with the expectation that as they improve, so will the related functional skills (e.g., readiness to listen to speakers taking turns, remembering a set of instructions given by one’s boss). Myers (1999) advocates the use of decontextualized treatments to facilitate cognitivecommunication skills in individuals who have acquired CCD from right hemisphere stroke. She argues that although the value of any treatment approach ‘‘rests on its

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functional merits’’ (p. 209), improving fundamental cognitive processes will have the greatest automatic generalization to the many untrained tasks where those processes are needed. Generalization may be more difficult to achieve when CCD is associated with diffuse brain injury versus focal stroke. Rehabilitation approaches that are more contextualized (implemented in natural settings) and more function oriented (designed to support activities of daily living) than traditional methods are ideal for promoting rapid skill mastery and transfer. Ylvisaker et al. (2008) suggest that treatment strategies designed to compensate for impaired executive functions and self-regulation abilities will most often lead to successful rehabilitation after brain injury. They advocate practicing essential cognitivecommunication tasks (such as conversing with family members or taking notes during lectures) within supportive real-world environments, where stimuli likely to trigger errors have been removed and where the use of external aids is encouraged. Intervention for CCD in DAT emphasizes management rather than rehabilitation. Goals are designed to help individuals maintain functional competence for as long as possible. Decontextualized errorless learning activities that drill attention and memory can bolster the encoding and retrieval of factual information in early stages of dementia. Intervention tasks that employ external memory aids that recruit long-term (remote) memory (such as reminiscing about past events) and that draw on procedural memory for familiar routines (such as describing how to get home) can help promote social communication and safety while capitalizing on those aspects of cognition that are available in midstage dementia. Tangible sensory stimuli (including dolls and stuffed animals) can help support communication and reduce agitation as dementia advances (Bourgeois, 1990, 1991, 1992; Hoerster, Hickey, & Bourgeois, 2001; Hopper & Bayles, 2008).

Cross References ▶ Attention ▶ Brain Injury ▶ Cognitive Functioning ▶ Cognitive Processing ▶ Cognitive Rehabilitation ▶ Dementia ▶ Executive Functioning ▶ Functional Neuroanatomy

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▶ Memory Impairment ▶ Mild Brain Injury ▶ Mild Cognitive Impairment ▶ Moderate Brain Injury ▶ Pragmatic Communication ▶ Severe Brain Injury

References and Readings Adamonovich, B., & Henderson, J. (1992). Scales of cognitive ability for traumatic brain injury. Chicago, IL: Riverside. American Speech-Language-Hearing Association. (2005). Roles of speechlanguage pathologists in the identification, diagnosis, and treatment of individuals with cognitive-communication disorders: Position statement [Position Statement]. www.asha.org/policy. Bayles, K. A., & Tomoeda, C. (1993). The arizona battery for communication disorders of dementia. Austin, TX: Pro-Ed. Bayles, K. A., & Tomoeda, C. (1994). The functional linguistic communication inventory. Austin, TX: Pro-Ed. Bayles, K. A., & Tomoeda, C. (2007). Cognitive-communication disorders of dementia. San Diego, CA: Plural Publishing. Blosser, J. L., & DePompei, R. (2003). Pediatric traumatic brain injury: Proactive intervention (2nd ed.). Clifton Park, NY: Delmar Learning. Borkowski, J. G., Benton, A. L., & Spreen, O. (1967). Word fluency and brain damage. Neuropsychologia, 5, 135–140. Bourgeois, M. S. (1990). Enhancing conversation skills in patients with Alzheimer’s disease using a prosthetic memory aid. Journal of Applied Behavior Analysis, 23(1), 29–42. Bourgeois, M. S. (1991). Communication treatment for adults with dementia. Journal of Speech and Hearing Research, 34(4), 831–844. Bourgeois, M. S. (1992). Evaluating memory wallets in conversations with persons with dementia. Journal of Speech and Hearing Research, 35(6), 1344–1357. Bruns, J. Jr., & Hauser, W. A. (2003). The epidemiology of traumatic brain injury: A review. Epilepsia, 44(Suppl. 10), 2–10. Burns, M. (1997). The burns brief inventory of communication and cognition. San Antonio: Psychological Corporation. Centers for Disease Control and Prevention. (2001). Traumatic brain injury in the United States: A report to Congress. www.cdc.gov. Centers for Disease Control and Prevention (CDC). (2007). Prevalence of stroke—United States, 2005. Morbidity and Mortality Weekly Report, 56(19), 469–474. Folstein, M. F., Folstein, S. E., & McHugh, P. R. (1975). ‘‘Mini-mental state’’: A practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research, 12, 189–198. Frattali, C., Thompson, C., Holland, A., Wohl, C., & Ferketic, M. (1995). American Speech-Language- Hearing Association Functional Assessment of Communication Skills for Adults. Rockville, MD: American Speech- Language-Hearing Association. Hagen, C., Malkmus, D., Durham, P., & Bowman, K. (1979). Levels ofcognitive functioning. In Rehabilitation of the head injured adult: Comprehensive physical management. Los Angeles: Professional Staff Association of Rancho Los Amigos Hospital. Halper, A., Cherney, L. A., & Burns, M. S. (1996). Clinical management of right hemisphere dysfunction (2nd. ed.). Gaithersburg, MD: Aspen. Helm-Estabrooks, N., & Hotz, G. (1991). Brief test of head injury. Chicago, IL: Riverside.

Hoerster, L., Hickey, E. M., & Bourgeois, M. S. (2001). Effects of memory aids on conversations between nursing home residents with dementia and nursing assistants. Neuropsychological Rehabilitation, 11(3/4), 399–427. Hopper, T., & Bayles, K. A. (2008). Management of neurogenic communication disorders associated with dementia. In R. Chapey (Ed.), Language intervention strategies in aphasia and related neurogenic communication disorders (5th ed., pp. 988–1008). Philadelphia: Lippincott Williams & Wilkins. Jennett, B., & Teasdale, G. (1981). Management of severe head injuries. Philadelphia: F. A. Davis. MacDonald, S. (2005). Functional assessment of verbal reasoning and executive strategies. Guelph, Ontario: CCD Publishing. Myers, P. S. (1999). Right hemisphere damage: Disorders of communication and cognition. San Diego: Singular Publishing Group. Myers, P. S. (2001). Toward a definition of RHD syndrome. Aphasiology, 15, 913–918. Myers, P. S., & Blake, M. L. (2008). Communication disorders associated with right-hemisphere damage. In R. Chapey (Ed.), Language intervention strategies in aphasia and related neurogenic communication disorders (5th ed., pp. 963–987). Philadelphia: Lippincott Williams & Wilkins. Paul, D., Frattali, C. M., Holland, A. L., Thompson, C. K., Caperton, C. J., & Slater, S. (2005). Quality of communication life scale. Rockville, MD: American Speech-Language-Hearing Association. Pimental, P. A., & Kingsbury, N. A. (1989). Mini inventory of right brain injury. Austin, TX: Pro-Ed. Reisberg, B., Ferris, S. H., deLeon, M. J., & Crook, T. (1982). The global deterioration scale (GDS): An instrument for the assessment of primary degenerative dementia (PDD). American Journal of Psychiatry, 139(1), 136–139. Ross-Swain, D. (1996). Ross information processing assessment (RIPA-2). Austin, TX: Pro-Ed. Roth, R. M., Isquith, P. K., & Gioia, G. A. (2005). Behavior rating inventory of executive function- Adult Version. Lutz, FL: Psychological Assessment Resources. Testa, J. A., Malec, J. F., Moessner, A. M., & Brown, A. W. (2005). Outcome after traumatic brain injury: Effects of aging on recovery. Archives of physical medicine and rehabilitation, 86(9), 1815–1823. Tomkins, C. A. (1995). Right hemisphere communication disorders: Theory and management. San Diego, CA: Singular Publishing Group. Turkstra, L. S., Cohelo, C., & Ylvisaker, M. (2005). The use of standardized tests for individuals with cognitive-communication disorders. Seminars in Speech and Language, 26(4), 215–222. Ylvisaker, M., Szekeres, S. F., & Feeney, T. (2008). Communication disorders associated with traumatic brain injury. In R. Chapey (Ed.), Language intervention strategies in aphasia and related neurogenic communication disorders (5th ed., pp. 879–962). Philadelphia: Lippincott Williams & Wilkins.

Cognitive-Communication Impairment ▶ Cognitive-Communication Disorder

Cognitive-Log

Cognitive-Log T HOMAS A. N OVACK University of Alabama at Birmingham Birmingham, AL, USA

Synonyms C-Log; Cog-Log; Cognitive Log

Description The Cog-Log is a ten-item scale designed for serial bedside measurement of cognitive functions in individuals completing inpatient rehabilitation. The scale includes items assessing orientation, immediate and delayed verbal recall, concentration, executive function, response inhibition, and praxis. All responses are scored according to the following criteria: 3 points = spontaneously correct response, no errors; 2 points = correct on logical cueing (e.g., ‘‘That was yesterday, so today is?’’) or in the presence of 1 error; 1 point = correct on multiple-choice cueing or in the presence of 2 errors; 0 points = incorrect despite cueing, more than 2 errors, unable to complete. Points for time estimation are calculated as follows: 3 points = correct within 5 s; 2 points = correct within 10 s; 1 point = correct within 15 s. Incorrect/absent responses are followed by cueing at the next level for the orientation and delayed memory items. Multiple-choice cueing, used only with the orientation items, involves provision of three choices, varying the location of the correct response. Item scores are summed to provide a total score ranging from 0 to 30. Daily scores can be plotted to permit quick visual analysis of recovery trends. The following specific items are included in the CogLog: three items assessing the orientation to the date, time, and hospital name; two items assessing immediate and delayed recall for a short address; counting backwards from 20; reciting the months in reverse order; and estimating when 30 s has passed. Two motor tasks involving hand gestures – a movement sequence (fist-edge-palm) and a response inhibition task (go/no-go) – are also included.

Historical Background Measurement of orientation and higher neurocognitive processes are important aspects of early neurorehabilitation.

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Reliable serial assessment is crucial to document the rapid changes in behavior and cognition during recovery from acquired brain disorders. Therefore, there is a need for a brief, bedside evaluation instrument to assess other areas of cognition that are frequently affected by acquired brain injuries. Existing scales are typically too lengthy or involved to present as part of morning rounds. Other scales fail to adequately capture the primary limitations resulting from acquired brain injury. The brief scales that have been created for serial administration in an inpatient setting have been presented without adequate psychometric properties or scaling. The Cog-Log was created to serve as a brief bedside measure to chart neurocognitive recovery and assist in planning rehabilitation interventions with a wide variety of patients.

Psychometric Data The reliability and validity of the Cog-Log have been assessed in several ways. A sample of 150 individuals with acquired brain injury was examined with the Cog-Log. Most of the sample (80%) had sustained moderate to severe TBI, with the remainder including patients with CVA and anoxia. Internal consistency analysis (Cronbach’s alpha) was conducted for the total Cog-Log score. Inter-rater reliability estimates (Spearman’s rho) were calculated using a subset of 19 patients (75 total observations). High internal consistency (Cronbach’s alpha = 0.778) was demonstrated with a standard error of measurement of 0.53. Inter-rater reliability coefficients for each of the ten Cog-Log items ranged from 0.749 (Go/No-go task) to 1.0 (Time Estimation). Standard errors of measurement were no more than 0.10 for single item scores, which range from 0 to 3. Factor analysis of the Cog-Log using principal components extraction (N = 150) revealed a unitary factor (Eigenvalue = 3.48), with all items loading on that factor. The highest loadings were for delayed recall of verbal information and recitation of months backwards, suggesting a stronger contribution of complex working memory and verbal recall to this unitary factor. The Cog-Log exhibited a significant correlation with neuropsychological assessment completed on the same day, including tests such as immediate and delayed recall of the Wechsler Memory Scale-III Logical Memory subtest, Rey Auditory Verbal Learning Test, Digit Span subtest of the Wechsler Adult Intelligence Test-III, and the Trail Making Test. The lowest Cog-Log score obtained during acute rehabilitation also significantly predicted 1-year outcome in three of six neuropsychological domains (attention, executive functioning, and visuospatial abilities) after

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controlling for demographics and injury severity. The CogLog was also significantly correlated with the results of the Mini Mental State Examination, a well-known cognitive screening test (r = 0.75, P < 0.001).

Lee, D., LoGalbo, A. P., Banõs, J. H., & Novack, T. A. (2004). Prediction of cognitive abilities one year following TBI based on cognitive screening during rehabilitation. Rehabilitation Psychology, 49, 167–171. Penna, S., & Novack, T. (2007). Further validation of the orientation and Cognitive Log relative to the mini mental status exam. Archives of Physical Medicine and Rehabilitation, 88, 1360–1361.

Clinical Uses Individuals without known neurological insult received average total Cog-Log scores of 28 ( 2), and mean individual item scores were greater than or equal to 2.4. Age, education, and gender did not predict total or individual item scores (p > 0.05). Stepwise discriminant analyses on a sample of 82 persons with brain injury and 82 normal controls matched for age, education, and gender revealed that a cut-off score of 25 correctly classified 88.4% of individuals in their respective groups. The Cog-Log is a companion instrument to the Orientation Log (O-Log). Generally, the Cog-Log is not administered until a score of at least 15 is achieved on the O-Log, indicating that the person is responding and able to respond correctly to some orientation questions. Administering the Cog-Log prior to this point has not proven fruitful. The Cog-Log can be administered every day, but typically three times a week is sufficient to monitor progress or detect deterioration. Efficiency and ease of assessment were considered when choosing items; tasks requiring additional stimuli (e.g., block construction) or extended administration times were not included. The Cog-Log was designed for flexible administration to patients with severe cognitive and behavioral disturbances. Administration time ranges from 7 to 10 min for confused patients, but can be as short as 5 min for those who perform well.

Collaborative Care ▶ Family-Centered Care

Collagen Vascular Disease E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Connective tissue disease

Definition Collagen vascular diseases are a group of conditions that are characterized by malfunction of the tendons, bones, and connective tissue, that are supported by collagen. Their pathogenesis is autoimmune in nature.

Current Knowledge Cross References ▶ Cognitive Functioning ▶ Galveston Orientation and Amnesia Test ▶ Mini Mental State Examination ▶ Traumatic Brain Injury

References and Readings Alderson, A. L., & Novack, T. A. (2003). Reliable serial measurement of cognitive processes in rehabilitation: The Cognitive-Log. Archives of Physical Medicine and Rehabilitation, 84, 668–672. Center on Outcome Measurement in Brain Injury (COMBI). www.tbims. org/combi. Accessed May 26, 2009.

The most common collagen vascular disorders include rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and dermatomyositis. Others include polymyositis, polyarteritis nodosa, ankylosing spondylitis, and a number of vasculopathies. These diseases are frequently associated with diffuse inflammatory changes, abnormal immunity. Vascular abnormalities that result from these conditions serve as frequent causes of various types of vasculitis. Common features include arthritis, skin changes, eye changes, pericarditis, pleuritis, myocarditis, nephritis, and vasculitis of the brain, peripheral nerves, or extremities. They also may have a variety of hematological changes causing clotting or bleeding, and a number of abnormal circulating blood proteins.

Color Agnosia

The cause of most of these diseases is unknown. Hereditary factors and deficiencies, autoimmunity, environmental antigens, infections, allergies, and antigenantibody complexes in various combinations are probably involved.

Cross References ▶ Cerebral Angiitis ▶ Lupus Cerebritis ▶ Vasculitis

References and Readings Klippel, J. H., Stone, J. H., Crofford, L. J., & White, P. H. (Eds.). (2008). Primer on the rheumatic diseases (13th ed.). New York: Springer.

Collapsed Lung ▶ Pneumothorax

Colliculi ▶ Inferior Colliculi

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color perception (i.e., retaining the ability to match colors or to identify the numbers on the Ishihara plates). They also have difficulty matching colors, either verbally or visually, to familiar colored objects (e.g., identifying the color normally associated with cherries, lettuce, or bananas). Relatively rare, pure color agnosia must be distinguished from other disturbances of color perception and color naming (color anomia). In color blindness, the individual is unable to perceive or distinguish either certain colors or possibly all color. In the latter case, the world is seen in shades of black and white. While color blindness is usually congenital, it can also be acquired, a condition known as central achromatopsia. The latter is a perceptual deficit thought to result from lesions in the visual cortices (e.g., lingual gyrus and the occipitotemporal (fusiform) gyrus). In this disorder, the patient may have difficulty verbally naming a visually presented color, pointing to a color named by the examiner, or simply matching or sorting colored objects to others of a similar hue, yet still be able to indicate (name) the colors normally associated with common objects (e.g., the colors of the outside, inside, and seeds of a watermelon). In a milder form of this condition (dyschromatopsia), colors are described as ‘‘dull,’’ ‘‘washed out,’’ or ‘‘faded.’’ In color anomia, the problem is not one of perceptions, but of language. The patient can perceive and match colors, but has difficulty naming specific colors or pointing to colors named by the examiner. In the few published cases, lesions associated with color agnosia tend to occur in the left or bilateral occipitotemporal area.

Color Agnosia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Cross References ▶ Achromatopsia ▶ Color Anomia

Definition Literally, a loss of previous color knowledge.

Current Knowledge In pure color agnosia, patients have difficulty naming or pointing to named colors, despite relatively preserved

References and Readings Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed., pp. 236–295). New York: Oxford University Press. Tranel, D. (2003). Disorders of color processing. In T. E. Feinberg & M. J. Farah (Eds.), Behavioral neurology and neuropsychology (pp. 243– 256). New York: McGraw-Hill.

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Color Anomia

Color Anomia J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

References and Readings Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman, & E. Valenstein (Eds.), Clinical neuropsychology (4th ed.) (pp. 236– 295). New York: Oxford University Press. Tranel, D. (2003). Disorders of color processing. In T. E. Feinberg, & M. J. Farah (Eds.), Behavioral neurology & neuropsychology (pp. 243–256). New York: McGraw-Hill.

Definition Anomia is the inability to name colors in the absence of a more global anomia associated with an aphasic disorder.

Color Blindness ▶ Achromatopsia

Current Knowledge To be classified as a color anomia, the disorder should occur in the absence of problems with color perception or recognition (i.e., the patient should be able to match or sort colors). Two subtypes of the disorder have been identified. In one, the problem is limited to an inability to name colors that are visually presented or to point to colors named by the examiner. This type of color anomia is usually associated with the syndrome of alexia without agraphia and results from lesions involving the primary visual cortex of the dominant hemisphere (resulting in a right homonymous hemianopsia) and the splenium of the corpus callosum. Visual information is thus restricted to the left visual field (right hemisphere) and the color information cannot cross the involved splenium of the corpus callosum to reach the left (verbal) hemisphere. In the second subtype, specific color anomia, the patient has difficulty with purely verbal color naming tasks, in addition to difficulty in naming visually presented colors. Thus, there would be a naming deficiency if the patient were asked to name the colors associated with the inside and outside of a watermelon. As in the first case, color matching or sorting should be intact. In specific color anomia, other aphasic (naming) deficits may be present, but color names are most affected.

Cross References ▶ Alexia Without Agraphia ▶ Color Agnosia

Color Imagery J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition The ability to visualize a color in its absence. When asked, most individuals would be able to identify the outer color of a watermelon as well as that of the inside of the rind, the fleshy part of the melon, and its seeds. Color imagery is more than an ability to simply recall a particular visual image; it also involves the capacity to mentally conjure up and manipulate colors at will. For example, one may imagine a blue horse or a person wearing an article of clothing of a specific color, never having seen either before. While disturbances of color perception and color imagery are frequently linked, in some cases the two can be distinguished clinically. Thus, while a given patient may be able to name or match colors presented visually, that same patient may not be able to name the color of an apple in its absence. Similarly, while accurately identifying the color red from an array, the patient may not be able to match it to a black-and-white picture of the fruit, although the latter may be identified by its shape. While the exact anatomical site responsible for disturbances of color imagery has not been firmly established, the left temporal-occipital cortex is believed to be involved in most cases.

Colored Progressive Matrices

Cross References ▶ Apperceptive Visual Agnosia ▶ Associative Visual Agnosia ▶ Color Agnosia ▶ Color Anomia

References and Readings Farah, M. J. (2003). Disorders of visual-spatial perception and cognition. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed., pp. 146–160). New York: Oxford University Press. Tranel, D. (2003). Disorders of color processing. In T. E. Feinberg & M. J. Farah (Eds.), Behavioral neurology and neuropsychology (2nd ed., pp. 243–256). New York: McGraw-Hill.

Colored Progressive Matrices V ICTORIA M. L EAVITT Kessler Foundation Research Center West Orange, NJ, USA

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D, and E of the standard progressive matrices (SPM) so that intellectual capacity can be more accurately assessed (the score for set Ab would be omitted to determine a percentile based on SPM scoring).

C Historical Background For information about the historical background of the original test, please refer to ▶ Raven Progressive Matrices.

Psychometric Data Norms for ages 5.5–11.5 for North America are presented by Raven, Raven and Court (1998, 2000). By age 9 years, a nearly perfect score is obtained (35/36) by the upper 5% of the sample. Education corrected norms are available for an abbreviated version (sets A and B, excluding Ab which is correlated >0.90 with the sum of A and B) for ages 55–85. In children, split-half reliability has been shown to be high (>0.80) (Raven et al. 1998), as is test-retest reliability following days or weeks (>0.80). Over longer intervals (6 months to 1 year) these values decline (0.59–0.79).

Clinical Uses Description

The colored progressive matrices (CPM) are an alternate form of the Raven’s progressive matrices (RPM) that was published in the 1940s. Shorter and simpler than the original, this version was designed for younger children (ages 5–11 years), the elderly (over 65 years), and people with moderate or severe learning difficulties. As such, it also tends to be used more frequently in research protocols, although it is important to note that the CPM and the RPM are not interchangeable, nor may derived scores from the two tests be interpreted the same. CPM contains 36 items, grouped into three sets (A, Ab, B) of 12 items each: A and B from the original version, with the addition of set Ab. Most items are presented on a colored background to make the test visually stimulating for participants; the bright background does not seem to detract from the clarity of the stimuli. As the last few items in set B are exactly the same as they appear in the standard version, an examinee who succeeds on these may go on to Sets C,

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The CPM was designed for use with children, older people, for anthropological studies, and for clinical work. Its format makes it valuable for individuals who cannot understand English; people with physical disabilities, aphasias, cerebral palsy, or deafness; and people with below normal intelligence. The colored backgrounds were introduced to attract the patient’s attention; the test can be administered in the form of illustrations in a booklet or as boards with moveable pieces. Patients with left hemisphere damage perform better on the colored matrices than on the standard form (as described in Lezak, Howieson, & Loring, 2004). This is likely attributable to the fact that while only one-fifth of RPM items test visuospatial skills almost exclusively, fully one-third of items on the CPM are predominantly visuospatial, with other items involving more problem-solving. Also likely due to the visuospatial task demands, individuals with Lewy body dementia tend to have more difficulty on the CPM than Alzheimer’s patients with similar levels of dementia.

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Coma

Cross References ▶ Advanced Progressive Matrices ▶ Raven’s Progressive Matrices ▶ Standard Progressive Matrices

rating, and include the Glasgow Coma Scale (Teasdale & Jennett, 1976) and the FOUR Score (Wijdicks, Bamlet, Maramattom, Manno, & McClelland, 2005).

Prognosis

References and Readings Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Raven, J. C. (1938, 1996). Progressive Matrices: A perceptual test of intelligence. Oxford: Oxford Psychologists Press Ltd. Raven, J., Raven, J. C., & Court, J. H. (1998). Raven manual: Section 2. Colored progressive matrices. Oxford: Oxford Psychologists Press Ltd. Raven, J., Raven, J. C., & Court, J. H. (2000). Raven manual research supplement 3: American norms, neuropsychological applications. Oxford: Oxford Psychologists Press Ltd. Raven, J., Raven, J. C., & Court, J. H. (2003). Manual for Raven’s progressive matrices and vocabulary scales. Section 1: General overview. San Antonio, TX: Harcourt Assessment. Strauss, E., Sherman, E. M. S., & Spreen, O. (Eds.). (2006). A compendium of neuropsychological tests (3rd ed.). New York: Oxford University Press.

Coma J ACOB K EAN Indiana University School of Medicine Indianapolis, Indiana, USA

Definition Coma is a state of unconsciousness in which the patient is incapable of being awake, and is unarousable, even with vigorous stimulation. Coma is usually the result of disease or injury, and rarely lasts for more than 4 weeks. While comatose, a patient may respond to painful stimuli but lack the ability to demonstrate localized response or defensive movements (Posner, Saper, Schiff, & Plum, 2007).

Current Knowledge Diagnosis The diagnosis of patients in coma necessarily involves examination of physiological functions, including arousal, pupillary responses, respiration, motor function, and reflexes. Several diagnostic scales are available for severity

Mortality rates for patients in coma vary with etiology, but commonly reach or exceed 50%. Early prognostic variables of poorer outcome include lower Glasgow Coma Scale scores (Teasdale & Jennett, 1976), increased age, absent pupillary responses, systolic blood pressure 1 year post-stroke, respectively; Page, Levine, & Leonard, 2005). Adherence to treatment and level of residual motor function prior to CIT is positively related to treatment gains (Shaw et al., 2005; Taub & Uswatte, 2005). Cognitive impairment is linked to less improvement (Morris et al., 2006). An inherent assumption in CIT is that a residual level of motor function remains in the affected extremity, which enables a degree of positive reinforcement for its use (Taub & Uswatte, 2006). A minimal level of residual motor function is often specified as an inclusion criterion in research studies of CIT, as is a lack of general cognitive impairment (e.g., Nichols-Larsen et al., 2005). Due to the higher degree of neural plasticity early in development, it has been suggested that children may benefit from CIT to an even greater extent than adults (e.g., Taub & Crago, 1995 as cited by Charles & Gordon, 2005). Specific modifications have been made to make CIT more amenable to childhood populations, including conducting CIT in the home environment and practice sessions in the context of play (DeLuca, Echols, Law, & Ramey, 2005).

Treatment Procedures CIT is generally administered as a package treatment, incorporating restraint of the unaffected limb, and structured practice (e.g., shaping and repetitive practice) with the affected limb. A ‘‘transfer package’’ involving behavioral techniques used to promote transfer of gains from the laboratory to daily life is emphasized in CIT

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(Taub & Uswatte, 2005). Structured practice with the affected arm involves everyday functional tasks (Alberts et al., 2004), such as eating lunch, throwing a ball, etc. (Glover, Mateer, Yoell, & Speed, 2002). Conventional CIT treatment aims for restraint of the unaffected limb for 90% of waking hours, and structured practice 6–8 h per day (Kaplon, Prettyma, Kushi, & Winstein, 2007). The treatment period for restraint and structured practice is 5 days per week, for 2–3 consecutive weeks with a 1:1 therapist–patient ratio. The intensive nature of CIT has prompted concerns regarding its applicability to clinical settings, due to institutional and clinical factors. For example, facilities may lack the space, therapist time, and associated financial resources to administer CIT (Levine & Page, 2004). Concerns have also been raised regarding attrition rates, compliance, and health care coverage (Levine & Page, 2004; Page & Levine, 2003). Because these factors can impede service delivery and the aims of CIT, modifications of treatment protocols have been introduced. For example, group delivered CIT (Brogardh, 2006), automatized delivery of CIT with less therapist involvement (AutoCITE; Lum, Uswatte, Taub, Hardin, & Mark, 2006; Taub, Lum, Hardin, Mark, & Uswatte, 2005), and fewer hours of restraint and shorter practice sessions over longer periods (Levine & Page, 2004; Page & Levine, 2003; Page, Sisto, Johnston, & Levine, 2002) have all resulted in significant treatment gains, often comparable to the gains of conventional CIT (e.g., Levine & Page, 2004; Lum et al., 2006). Current research suggests that particularly important features are prolonged rehearsal on specific tasks involving the affected limb and shaping in effort to improve everyday activity (Pomeroy & Tallis, 2002).

Efficacy Information CIT has been shown to significantly improve performance on laboratory tests of motor ability and functional outcome measures. The effect sizes for functional outcome measures tend to be very large in magnitude (Taub & Uswatte, 2005). Effect sizes on laboratory tests tend to be more variable (as reviewed by Lillie & Mateer, 2006).

Outcome Measurement There can be significant discrepancies between ‘‘real world’’ spontaneous functional use and motor ability as

measured in a laboratory setting, and it is therefore recommended that these dimensions be assessed separately (Uswatte & Taub, 2005). The Wolf Motor Function Test is a frequently used measure of laboratory motor performance (e.g., Uswatte & Taub, 2005). Functional outcome is most often studied through use of Motor Activity Log (MAL), a semistructured interview of extremity use in real-life settings (e.g., Winstein et al., 2003).

Qualifications of Treatment Providers Physiotherapists most frequently provide services. Occupational therapists have also been identified as providing services (e.g., Brogardh, 2006). Specialized training in CIT is recommended (DeLuca et al., 2005; Winstein et al., 2003).

Cross References ▶ Brain Plasticity ▶ Cognitive Rehabilitation ▶ Head Injury ▶ Hemiparesis ▶ Hemiplegia ▶ Physical Therapy ▶ Rehabilitation Psychology ▶ Traumatic Brain Injury

References and Readings Alberts, J. L., Butler, A. J., & Wolf, S. L. (2004). The effects of constraintinduced therapy on precision grip: A preliminary study. Neurorehabilitation and Neural Repair, 18, 250–258. Brogardh, C. (2006). Constraint-induced movement therapy in patients with stroke: A pilot study on effects of small group training and of extended mitt use. Clinical Rehabilitation, 20, 218–227. Charles, J., & Gordon, A. M. (2005). A critical review of constraintinduced movement therapy and forced use in children with hemiplegia. Neural Plasticity, 12, 245–261. DeLuca, S. C., Echols, K., Law, C. R., & Ramey, S. L. (2005). Intensive pediatric constraint-induced therapy for children with cerebral palsy: Randomized, controlled, crossover trial. Journal of Child Neurology, 21, 931–938. Dong, Y., Dobkin, B. H., Cen, S. Y., Wu, A. D., & Winstein, C. J. (2006). Motor cortex activation during treatment may predict therapeutic gains in paretic hand function after stroke. Stroke, 37, 1552–1555. Glover, J. E., Mateer, C. A., Yoell, C., & Speed, S. (2002). The effectiveness of constraint induced movement therapy in two young children with hemiplegia. Pediatric Rehabilitation, 5, 125–131.

Constructional Apraxia Kaplon, R. T., Prettyma, M. G., Kushi, C. L., & Winstein, C. J. (2007). Six hours in the laboratory: A quantification of practice time during constraint-induced therapy (CIT). Clinical Rehabilitation, 21, 950–958. Lillie, R., & Mateer, C. A. (2006). Constraint-based therapies as a proposed model for cognitive rehabilitation. Journal of Head Trauma Rehabilitation, 21, 119–130. Levine, P., & Page, S. J. (2004). Modified constraint-induced therapy: A promising restorative outpatient therapy. Topics in Stroke Rehabilitation, 11, 1–10. Lum, P. S., Uswatte, G., Taub, E., Hardin, P., & Mark, V. W. (2006). A telerehabilitiation approach to delivery of constraint-induced movement therapy. Journal of Rehabilitation Research and Development, 43, 391–400. Morris, D. M., Shaw, S. E., Mark, V. W., Uswatte, G., Barman, J., & Taub, E. (2006). The influence of neuropsychological characteristics on the use of CI therapy in persons with traumatic brain injury. Neurorehabilitation, 21, 131–137. Morris, D. M., & Taub, E. (2001). Constraint-induced therapy approach to restoring function after neurological injury. Topics in Stroke Rehabilitation, 8, 16–30. Nichols-Larsen, D. S., Clark, P. C., Zeringue, A., Greenspan, A., & Blanton, S. (2005). Factors influencing stroke survivors’ quality of life during subacute recovery. Stroke, 36, 1480–1484. Page, S., & Levine, P. (2003). Forced use after TBI: Promoting plasticity and function through practice. Brain Injury, 17, 675–684. Page, S. J., Levine, P., & Leonard, A. C. (2005). Modified constraintinduced therapy in acute stroke: A randomized controlled pilot study. Neurorehabilitation and Neural Repair, 19, 27. Page, S. J., Sisto, S., Johnston, M. V., & Levine, P. (2002). Modified constraint-induced therapy after subacute stroke: A preliminary study. Neurorehabiliation and Neural Repair, 16, 290–295. Park, S. W., Butler, A. J., Cavalheiro, V., Alberts, J. L., & Wolf, S. I. (2004). Changes in serial optical topography and TMS during task performance after constraint-induced movement therapy in stroke: A case study. American Society of Neurorehabiliation, 18, 95–105. Pomeroy, V., & Tallis, R. (2002). Neurological rehabilitation: A science struggling to come of age. Physiotherapy Research International, 7, 76–89. Shaw, S. E., Morris, D. M., Uswatte, G., McKay, S., Meythaler, J. M., & Taub, E. (2005). Constraint-induced movement therapy for recovery of upper-limb function following traumatic brain injury. Journal of Rehabilitation Research and Development, 42, 769–778. Sterr, A., Szameitat, A., Shen, S., & Freivogel, S. (2006). Application of CIT concepts in the clinical environment: Hurdles, practicalities, and clinical benefits. Cognitive Behavioral Neurology, 19, 48–53. Taub, E., Lum, P. S., Hardin, P., Mark, V. W., & Uswatte, G. (2005). AutoCITE: Automated delivery of CIT with reduced effort by therapists. Stroke, 36, 1301–1304. Taub, E., Miller, N. E., Novack, T. A., Cook, E. W. III, Fleming, W. C., Nepomuceno, C. S., et al. (1993). Technique to improve chronic motor deficit after stroke. Archives of Physical Medicine and Rehabilitation, 74, 347–354. Taub, E., & Uswatte, G. (2005). Use of CI therapy for improving motor ability after chronic CNS damage: A development prefigured by Paul Bach-Y-Rita. Journal of Integrative Neuroscience, 4, 465–477. Taub, E., & Uswatte, G. (2006). Constraint-induced movement therapy: Answers and questions after two decades of research. Neurorehabilitation, 21, 93–95. Uswatte, G., & Taub, E. (2005). Implications of the learned non-use formulation for measuring rehabilitation outcomes: Lessons from

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constraint-induced movement therapy. Rehabilitation Psychology, 50, 34–42. Winstein, C. J., Miller, J. P., Blanton, S., Taub, E., Uswatte, G., Morris, D., et al. (2003). Methods for a multisite randomized trial to investigate the effects of constraint-induced movement therapy in improving upper extremity function among adults recovering from a cerebrovascular stroke. Neurorehabilitation and Neural Repair, 17, 137–152. Wolf, S. L., Winstein, C. J., Miller, J. P., Taub, E., Uswatte, G., Morris, D., et al. (2006). Effects of constraint-induced movement therapy on upper extremity function 3 to 9 months after stroke: The EXCITE randomized clinical trial. JAMA, 296, 2095–2103.

Constructional Apraxia A MY K. B YERLEY, A NDREW S. DAVIS Ball State University Muncie, Indiana, USA

Synonyms Constructional dyspraxia

Short Description or Definition Constructional apraxia is an inability to reproduce patterns or join component parts into a whole. This condition is assessed through observation of a patient completing activities such as drawing, copying, or building threedimensional objects (Lezak, Howieson, & Loring, 2004). Impairments in processing spatial forms observed in constructional apraxia can occur in the absence of apraxia of singular motor movements (Benton, 1969).

Categorization Recent studies suggest qualitatively different types of constructional apraxia determined by the location of brain insult. In general, patients with right hemisphere impairment make more coordinate type errors (e.g., distance and angular distortions), whereas those with left hemispheric impairment tend to commit categorical errors (e.g., position exchanges and pattern reversals) (Laeng, 2006). Patients with right hemispheric impairment generally experience difficulties with the overall gestalt of the constructional task. Their approach to the task may appear to be more fragmented and disjointed, not coming

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together as a whole. Constructions created by patients with right hemisphere impairment may make drawing that are sparse or may be significantly distorted with regard to perspective or proportion. Some patients with right hemisphere injury may create overdetailed or repetitive drawings, while others may draw elaborate pictures that are missing important components. Additionally, patients with right hemispheric lesions frequently demonstrate left-sided visual inattention and include more details on construction tasks. In contrast, those with left hemisphere impairment may be able to construct the overall concept and proportions accurately, and their constructions may also be symmetrical. However, they often tend to generate drawings with fewer details (McFie & Zangwill, 1960). Patients with left hemisphere impairment may perform construction tasks better when given a model rather than working on command. They tend to focus on overall shape rather than specific details. Overall, construction deficits tend to be more common when the lesion is located in the posterior portion of the brain rather than the anterior areas. Furthermore, while patients with cortical lesions make the same types of constructional errors as patients with subcortical lesions, those with subcortical impairment appear to have more significant impairment.

Epidemiology A review of literature revealed no known reports of prevalence of constructional apraxia. In fact, constructional apraxia is generally included as a subcategory of apraxia, which is listed as a ‘‘rare disease’’ by the office of rare diseases (ORD) of the National Institutes of Health (NIH). Therefore, apraxia is known to affect less than 200,000 people in the population of the USA. Thus, as a subcategory of apraxia, constructional apraxia affects even fewer individuals. Common causes of constructional apraxia include dementia (e.g., Alzheimer’s Disease) and stroke, which are two of the most frequent neurological diseases.

Natural History, Prognostic Factors, and Outcomes Constructional apraxia is not in itself a disease but a symptom of another neurological illness. Therefore, prognosis of the condition is related to the prognosis of the underlying neurological condition. Prognosis for individuals with constructional apraxia varies and has not been well studied. Some patients may improve via continued

treatment, while others may have symptoms spontaneously diminish. For other patients, symptoms may be permanent. Further research for prognostic factors of constructional apraxia is warranted.

Neuropsychology and Psychology of Constructional Apraxia Symptoms associated with constructional apraxia frequently indicate deficits associated with right parietal dysfunction, or the non-dominant hemisphere and global cognitive processing impairment. However, recent research has shown involvement of both hemispheres, namely the parietal lobes, in constructional apraxia. Furthermore, neuroimaging research has implicated the ventral premotor area, posterior part of the inferior temporal sulcus, and occipital cortex in constructional deficits (Makuuchi, Kaminaga, & Sugishita, 2003; Nielson, Cummings, & Cotman, 1996). Constructional apraxia should not be conceptualized as a unitary syndrome caused by discrete lesions (Gue´rin, Ska, & Belleville, 1999), but as resulting from impairment in lateralized perceptual processing of spatial abilities (Laeng, 2006). Constructional apraxia manifests more frequently in patients with lesions in the non-dominant hemisphere (De Renzi, 1997). Consequently, constructional apraxia often co-occurs with deficits in visual-spatial perception (Benton, 1982), although both conditions may exist independently. Therefore, the presentation of constructional apraxia differs among patients; some struggle with copying figures, whereas others demonstrate difficulty constructing designs with blocks (Lezak et al., 2004).

Evaluation The utility of constructional apraxia as an estimate of global cognitive functioning has a well-established history in neurology, neuropsychology, and rehabilitative settings. Tasks commonly utilized to assess constructional apraxia include copying, drawing, or constructing simple figures, such as clocks or crosses. These efficient, brief, and easy-to-administer construction tasks are attractive to clinicians for use with patients with impaired test-taking skills. The tasks used to assess constructional apraxia have proven to be remarkably sensitive to global neurological impairment because of the involvement of various cognitive processing domains necessary for completing construction

Content-Referenced Testing

tasks. Construction tasks can require complex cognitive processes, including visual-spatial and visuoperceptual abilities, receptive language skills, numerical knowledge, graphomotor skills, and intact executive functioning (Freedman et al., 1994; Lezak et al., 2004; Shulman, 2000). The sensitivity of construction tasks to global cognitive impairment renders these tasks advantageous in the assessment of patients with known or suspected neurological impairment, including those with Alzheimer’s Disease, Huntington’s Disease, Parkinson’s Disease, aphasia, seizures, CVAs, and TBIs. As an example, construction tasks have been shown to be useful as screening tools and markers for disease progression with Alzheimer’s patients. Furthermore, the presence of constructional apraxia early in the course of Alzheimer’s Disease has been shown to be a predictor of accelerated cognitive decline (Smith, Esiri, Barnetson, King, & Nagy, 2001).

Treatment While physical and occupational therapies may modestly improve the functioning of an individual with constructional apraxia, no specific medical treatment has been found to be effective with significantly improving constructional deficits. Furthermore, medications that target the slowing of dementia do not appear to help with constructional apraxia. The most beneficial approach for treatment of individuals with constructional apraxia involves ensuring their environments are safe. For example, it is important to arrange furniture in the home in a way that the patient can navigate safely.

Cross References ▶ Apraxia ▶ Dementia ▶ Stroke ▶ Visual-Motor Function

References and Readings Benton, A. L. (1969). Constructional apraxia: Some unanswered questions. In A. L. Benton (Ed.), Contributions to clinical neuropsychology (pp. 128–141). Chicago: Aldine. Benton, A. L. (1982). Spatial thinking in neurological patients: Historical aspects. In M. Potegal (Ed.), Spatial abilities: Development and physiological foundations (pp. 253–271). New York: Academic Press.

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Benton, A. L. (2009). Constructional apraxia: Some unanswered questions. In A. L. Benton (Ed.), Brain & behavior: Research in clinical neuropsychology (pp. 129–141). Chicago: Aldine. De Renzi, E. (1997). Visuospatial and construction disorders. In T. E. Feinberg, & M. J. Farah (Eds.), Behavioral neurology and neuropsychology (pp. 297–304). New York: McGraw-Hill. Fischer, J. S., & Loring, D. W. (2004). Construction. In M. D. Lezak, D. B. Howieson, & D. W. Loring (Eds.), Neuropsychological assessment (4th ed., pp. 531–568). New York: Oxford University Press. Freedman, M., Leach, L., Kaplan, E., Winocur, G., Shulman, K., & Delis, D. C. (1994). Clock drawing: A neuropsychological analysis. New York: Oxford University Press. Gue´rin, F., Ska, B., & Belleville, S. (1999). Cognitive processing of drawing abilities. Brain and Cognition, 40:464–478. Laeng, B. (2006). Constructional apraxia after left or right unilateral stroke. Neuropsychologia, 44:1595–1606. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological Assessment (4th ed.). New York: Oxford University Press. Makuuchi, M., Kaminaga, T., & Sugishita, M. (2003). Both parietal lobes are involved in drawing: A functional MRI study and implications for constructional apraxia. Brain Research, 16:338–347. McFie, J., & Zangwill, O. L. (1960). Visual construction disabilities associated with lesions of the left cerebral hemisphere. Brain, 83:243–260. Nielson, K., Cummings, B., & Cotman, C. (1996). Constructional apraxia in Alzheimer’s disease correlates with neuritic neuropathology in occipital cortex. Brain Research, 741:284–293. Shulman, K. L. (2000). Clock-drawing: Is it the ideal cognitive screening test? International Journal of Geriatric Psychiatry, 15:548–561. Smith, M., Esiri, M., Barnetson, L., King, E., & Nagy, Z. (2001). Constructional apraxia in Alzheimer’s disease: Association with occipital lobe pathology and accelerated cognitive decline. Dementia and Geriatric Cognitive Disorders, 12:281–288.

Constructional Dyspraxia ▶ Constructional Apraxia

Content Validity ▶ Test Validity

Content-Referenced Testing ▶ Domain Referenced Test Interpretation

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Contextual Memory

Contingency Table. Table 2

Contextual Memory

TBI

▶ Source Memory

No TBI

Test +

35

15

50

Test

5

45

50

40

60

100

Cond. = Condition

Contingency Table C HRISTIAN S CHUTTE , B RADLEY A XELROD John D. Dingell VA Medical Center Detroit, MI, USA

Synonyms 2 2 Table

Definition A contingency table is generally a representation of categorical data in a tabular format, such as a 2 2 table, though the table can have three or more variables.

Current Knowledge There are row variables on the horizontal axis and column variables on the vertical axis. It represents mutually exclusive variables. Good examples of contingency tables are hit rate, sensitivity, and specificity, as well as positive and negative predictive power. In the case of hit rates to assess the number of correct classification decisions that result from the use of a particular test or measure, one would enter the number of true positives, true negatives, false positives, and false negatives into a contingency table like the one below.

In this example, hit rate would be calculated by adding the number of true negatives and false negatives and then dividing them by the number of total decisions ((TP + FN)/total decisions). This allows for a relatively simple way to assess the relationships between categorical variables. A concrete example would be a group of 100 patients, 40 of whom have a true diagnosis of traumatic brain injury (TBI) who are administered a brief test to diagnose the presence of the disorder. By comparing the obtained scores to a cutting score, 55 patients are tagged as having the disorder and 45 patients as not having the disorder. Using this information, a contingency table to find hit rate can quickly be defined, sensitivity, and specificity, as well as positive and negative predictive power. One can then calculate hit rate ((TP + FN)/total decisions) or (35 + 5)/100 = .4, sensitivity (TP/(TP + FN)) or 35/(35 + 5) = .875, specificity (TN/(TN + FP)) or 45/(45 + 15) = .75, positive predictive power (TP/(TP + FP)) or 35/(35 + 15) = .70, and negative predictive power (TN/(TN + FN)) or 45/(45 + 5) = .90. In verbal form, hit rate is a proportion of the number of correctly identified TBI patients and incorrectly ruled out TBI patients divided by the total number of patients (40% in this case) or sensitivity is computed by the percentage of people who actually have the disorder who were appropriately detected by the test (87.5% in this case). More complex contingency tables can be used, such as 3 2, 3 3, or more, though relationships between variables are less clear owing to difficulties with interaction effects. More complex contingency tables are often analyzed with more complex statistical procedures, such as log-linear analysis.

Contingency Table. Table 1

Cross References

Cond. +

Cond.

True + (TP)

False + (FP)

Total + Decisions

Test False (FN) True (TN)

Total Decisions

Test +

Base Rate Cond. = Condition

Cond. Absent Total of all decisions

▶ Negative Predictive Power ▶ Positive Predictive Power ▶ Sensitivity ▶ Specificity

Continuous Performance Tests

Continuous Performance Tests R ONALD A. C OHEN Brown University Providence, RI, USA

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more commonly used in standard neuropsychological evaluations. In fact, the CPT was one of the first neuropsychological paradigms widely adapted for computerized assessment. Subsequently, impairments on this paradigm were demonstrated across various disorders, most notably schizophrenia (Nuechterlein, 1983) and attention deficit disorder (Epstein et al., 2003).

Synonyms CPT

Description An attention paradigm that has evolved into a class of neuropsychological tests used to assess sustained attention. There is not a single continuous performance test (CPT) test, as a number of commercially available and research CPT tasks exist and have been published in the neuropsychological literature. The common characteristic of all CPT tests is that they involve sequential presentation of stimuli, usually letters or numbers, over an extended period of time. The task demand is to attend and respond to particular target stimuli, while ignoring other stimuli that serve as nontarget distractors.

Historical Background Early efforts by psychologists to assess attention in the context of intellectual or other cognitive testing typically relied on tests such as digit span, which provided a useful measure of attentional focus and span, but did not address other important elements of attention, such as the patients’ ability to selectively attend to information or to sustain attention (Cohen, 1993). Sustained attention was particularly difficult to assess using traditional paper-andpencil tests, as it required the measurement of signal detection performance over extended periods of time. Psychologists typically relied on behavioral observation or analysis of patterns of inconsistency in test performance over time to derive evidence of sustained attention problems. The development of the tachistoscope for rapid presentation of visual stimuli with controlled timing provided a means of circumventing this problem. Mirsky et al. (1956) described the continuous performance paradigm and provided research data supporting its sensitivity in detecting brain damage. The advent and widespread availability of computer technology in the decades that followed led to more widespread experimentation with this paradigm and it eventually being

Psychometric Data In its original form, the CPT provided measures of accuracy and response bias over the course of a fixed time period using signal detection methodology. Although the tests may vary in terms of length and type of stimulus used, the underlying paradigm is the same across versions of the test. Patients are presented with series of letters (typically) or other stimuli on a screen, and are told to push a response key only when they see the ‘‘target’’ stimulus. They are instructed not to respond when they see any other stimuli. The letter ‘‘x’’ has often been used as the target in CPT paradigms, with the task to respond to this letter while ignoring other letters that flash before them. Several common variations of the standard CPT paradigm exist, in particular, a conditional task, in which the patient must only respond to the target letter when another stimulus occurs immediately before it (e.g., A-X), which increases the difficulty of the task. In recent years, a variety of tests based on computerized CPT paradigms have been developed, with varying degree of sensitivity and specificity to clinical disorders of attention. At the present time, the most widely used commercially available CPT tests are versions by Conners, Epstein, Angold, and Klaric (2003) and the Test of Variables of Attention (TOVA), though a variety of others exist, such as VIGIL (1990) and the d2 Test of Attention (Brickenkamp, 1992). Typically, a CPT is included as part of a more comprehensive battery of tests of attention and executive functioning. Given that the CPT paradigm is based on signal detection theory and method, there are certain indices common to all versions of the test; correct responses, errors of omission (misses), errors of commission (false positives), and response time (RT). Correct responses are based on the sum of the number of times the patient correctly responds to the target and correctly avoids responding to distractors. Omission errors (misses) are errors involving a failure to respond to the target, while commission errors (false positives) are errors involving a response to nontargets. Errors of commission reflect a failure to inhibit responding. High omission rates indicate that either the patient is not

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Continuous Performance Tests

focusing adequately on the stimuli or that their processing speed is slow and they are unable to respond rapidly enough. CPT tests usually also provide a measure of mean response time (RT), which reflects the processing speed of the patient during the task. Higher rates of correct detections indicate better attention performance. From these primary measures, signal detection indices are usually derived based on a comparison of error types. Based on the total number of errors of commission and omission on the CPT, a discrimination index (d0 ) is calculated which provides a measure of accuracy based on standardized scores (z-scores) from normative samples. A measure of response bias (b) is also usually derived based on the difference in standard scores for each type of error. Response bias indices provide a better way of interpreting tendencies to make one type of error or the other because they account for the total number of errors of each type. Receiver operator characteristics (ROC) metrics can also be derived, which provide a way of interpreting the tendency to make errors of each type as a function of the signal detection parameters of the task, such as percentage of targets to nontargets. General equations for d0 and b are shown below. d0 ¼ zðmissesÞ þ zðfalse positivesÞ b ¼ zðmissesÞ zðfalse positivesÞ=z ðtotal errorsÞ In the past, many versions of the CPT provided only these basic indices, which limited their usefulness in characterizing problems with sustained attention. While d0 and b provide excellent measures of detection accuracy, response deposition, and overall attention performance, they do not directly provide measures of performance over time. Recent versions of the Conner’s and other CPT tests tend to now include a measure of temporal variability in performance. While there are a variety of ways that the temporal nature of performance can be determined, two general types of measures exist: (1) performance decrement and (2) performance inconsistency. Performance decrement provides a measure of the change in performance between the beginning and end of the test. In theory, if a person is failing to sustain attention, their performance should worsen the longer they stay on task. However, often this measure is not impaired except when there is a severe problem with sustained attention and fatigue after several minutes of effort. Performance inconsistency indices provide an alternative temporal measure based on variance across time, as opposed to linear decrement. A primary problem in interpreting the meaning and clinical significance of CPT findings stems from the lack

of consistency across versions of the tests, particularly with respect to basic parameters such as the duration of the interstimulus interval (ISI), the total duration of the task, and the task demand. For example, a task requiring detection of a single letter is much easier than a conditional paradigm requiring detection of a letter sequence (A-X). Furthermore, the duration of a typical CPT test can range from several minutes to over 20 min. Versions with a long ISI tend to be relatively easy to perform, but are tedious, with behavioral challenge arising from the monotony of the task, which over a long test period is really a test of vigilance. In contrast, versions that have a short ISI with conditional task demands or other characteristics that increase difficulty tend to be more sensitive to sustained attention in the context of greater information processing demand and requirement for focused attention. Adaptive-rate continuous performance methods provide a means of circumventing this issue. For example, the adaptive-rate continuous performance test (ARCPT; Cohen, 1993) adjusts its ISI over the course of the test based on accuracy of response to compensate for the slow speed of processing. The final ISI that is maintained by the end of the test provides a strong measure of capacity limitations related to processing speed deficits. Also the ARCPT provides separate vigilance decrement and inconsistency indices to enable assessment of the temporal dynamics of performance over 10 blocks of time over the duration of the test. The ARCPT measures attentional performance on a rapid and challenging task which is less subject to boredom. Therefore, the ARCPT provides enables the assessment of sustained attention during a cognitively demanding task compared to standard CPT paradigms. Also, because the ISI adjusts to shorter durations when a patient is performing well, the test can typically be completed in about 10 min. The ARCPT also provides several metrics that enable assessment of temporal inconsistencies in performance. Despite the fact that CPT tests vary in a variety of ways, many of the commonly used measures (e.g., Conner’s CPT) report good test–retest reliability in healthy adults. CPT performance tends to correlate well with performance on other information processing-based measures and performance on speeded tests of attention and executive function, such as the Stroop and trailmaking test. Validation studies conducted with the ARCPT indicate strong reliability (r = .95) for accuracy of performance (d0 ) across samples and strong validity as indicated by the sensitivity of CPT indices to measures of structural and functional brain and systemic physiological disturbances as discussed in greater detail below.

Contraindication

Clinical Uses CPT tests should be included in all comprehensive neuropsychological batteries designed to provide a thorough assessment attention, executive functioning, and processing speed. There is a well-established research and clinical literature demonstrating that this paradigm is highly sensitive to brain dysfunction. The fact that the CPT yields measures of information processing characteristics based on a task with controlled timing and consistency of stimulus presentation makes it a valuable addition to standard paper-and-pencil tests of cognitive function. Performance deficits on the CPT are evident in patients with various forms of brain dysfunction (Rosvold, Mirsky, Sarason, Bransome, & Beck, 1956). The most obvious use for the CPT is in the assessment of attention deficit disorder (ADD), for which the objective assessment of sustained attention is central to the diagnosis. However, there is now an extensive research literature demonstrating deficits or outright impairments of CPT performance among patients with a wide range of neurological and psychiatric conditions. For this reason, impaired CPT performance should be considered a highly sensitive measure of functional attention impairment and brain dysfunction, though not necessarily specific to one type of neurological or psychiatric condition. Impairments of CPT are evident in patients with neurodegenerative disorders like Alzheimer’s disease, as well as disorders affecting subcortical and white matter brain systems, such as multiple sclerosis, HIV, and cerebrovascular disease. In fact, patients with cardiovascular disease show a relationship between cardiac output and CPT performance (Jerskey et al., 2009). Among patients with severe affective disorders, CPT performance is often impaired and associated with problems with effort (Cohen, Lohr, Paul, & Boland, 2001). CPT has been shown to be sensitive to sustained attention and information processing deficits associated with neurotoxic exposure to lead, solvents, drugs, or other substances. An important clinical determination that needs to be made when examining performance on the CPT is whether there is evidence of an actual problem with sustained performance, or whether a broader problem with focused and selective attention exists that occurs regardless of the time spend on task.

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▶ Sustained Attention ▶ Vigilance

References and Readings Brickenkamp, R. Z. E. (1992). The d2 test of attention. Seattle, WA: Hogrefe & Huber. Cohen, R. A. (1993). Neuropsychology of attention. New York: Plenum. Cohen, R., Lohr, I., Paul, R., & Boland, R. (2001). Impairments of attention and effort among patients with major affective disorders. Journal of Neuropsychiatry and Clinical Neurosciences, 13(3), 385–395. Conners, C. K., Epstein, J. N., Angold, A., & Klaric, J. (2003). Continuous performance test performance in a normative epidemiological sample. Journal of Abnormal Child Psychology, 31(5), 555–562. Epstein, J. N., Erkanli, A., Conners, C. K., Klaric, J., Costello, J. E., & Angold, A. (2003). Relations between continuous performance test performance measures and ADHD behaviors. Journal of Abnormal Child Psychology, 31(5), 543–554. Gunstad, J., Cohen, R. A., Paul, R. H., & Gordon, E. (2006). Dissociation of the component processes of attention in healthy adults. Archives of Clinical Neuropsychology, 21(7), 645–650. Jerskey, B., Cohen, R. A., Jefferson, A. L., Hoth, K. F., Haley, A. P., Gunstad, J. J., et al. (2009). Sustained attention is associated with left ventricular ejection fraction in older adults with heart disease. Journal of the International Neuropsychological Society, 15(1), 137–141. Leark, R. A., Greenberg, L. K., Kindschi, C. L., Dupuy, T. R., & Hughes, S. J. (2007). Test of variables of attention: Clinical manual. Los Alamitos: The TOVA Company. Nuechterlein, K. H. (1983). Signal detection in vigilance tasks and behavioral attributes among offspring of schizophrenic mothers and among hyperactive children. Journal of Abnormal Psychology, 92(1), 4–28. Rosvold, H. E., Mirsky, A. F., Sarason, I., Bransome, E. D., Jr., & Beck, L. H. (1956). A continuous performance test of brain damage. Journal of Consulting Psychiatry, 20, 343–350. VIGIL: A continuous performance test. (1990). New Hampshire: Forethought.

Contraindication M ARLA S ANZONE Independent Practice Annapolis, MD, USA

Synonyms Conflict; Counterindicant

Definition Cross References ▶ Attention Deficit Disorder (ADD) ▶ Signal Detection

701

A contraindication is a circumstance, condition, symptom, or factor that increases the risk associated with a medical procedure, drug, or treatment. A contraindication refers to

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Contrecoup Injury

any intervention considered inappropriate or inadvisable based upon unique factors of the situation such as potential harmful interactions between drugs or medical conditions that renders an individual vulnerable if implemented. A contraindication may be absolute or relative. Absolute contraindications are those which are inadvisable without exception or qualification. They are either permanently recommended against, or temporarily until the disqualifying condition is remediated. The use of the atypical antipsychotic medication, clozapine carries a risk of agranulocytosis, a sever low white blood cell disorder condition. Clozopine would be an absolute contraindication in an individual with a history of bone marrow suppression Relative contraindications refer to circumstances in which procedures or treatments are considered in comparative terms. They are contingent upon a risk/benefit analysis of the relevant factors. The proportionate value of an intervention is compared with its potential for negative consequences. An example of a relative contraindication would be the use of an anti-convulsant/mood stabilizing medication in the ongoing treatment of a pregnant woman with severe bipolar mania and suicidality, but whose has been asymptomatic only while on lithium.

Cross References ▶ Extrapyramidal Side Effects ▶ Iatrogenic ▶ Signs

results from acceleration–deceleration events during which the force impacting the head causes the brain to slam into the skull on the opposite side of the blow. Motor vehicle accidents, falls, sports injuries, and physical assaults with blunt objects frequently result in contrecoup injuries. Skull characteristics make the most probable sites of injury in the frontal and temporal lobes, as tips of the skull can more easily be forced into the underlying brain tissue in the frontal and temporal lobes. Neuropsychological evaluation can help to identify cognitive impairments arising from both the primary and secondary sites of injury.

Cross References ▶ Acceleration Injury ▶ Biomechanics of Injury ▶ Cortical Contusion ▶ Traumatic Brain Injury

References and Readings Graham, D. I., Saatman, K. E., Marklund, N., Copnte, V., Morales, D., Royo, N., & McIntosh, T. K. (2006). The neuropathology of trauma. In R. W. Evans (Eds.), Neurology and trauma (2nd ed., pp. 45–94). New York: Oxford University Press.


Controlled Attention Mosby. (2006). Contra- (definition). In T. Myers (Ed.), Mosby’s dictionary of medicine, nursing & health professions (7th ed., pp. 453). Missouri: Mosby Elsevier. Venes, D., Thomas, C., Egan, E., & Houska, A. (2001). Contraindication (definition). In D. Venes, et al. (Eds.), Taber’s medical dictionary (19th ed., pp. 479). Philadelphia: FA Davis Company.

A NNA M AC K AY-B RANDT Brown University Medical School Providence, RI, USA

Synonyms

Contrecoup Injury B ETH R USH Mayo Clinic Jacksonville, FL, USA

Central executive; Cognitive control; Supervisory attentional system

Definition Definition Contrecoup injury is an injury to the brain tissue directly beneath the skull, opposite to the point of impact. It

Higher-order cognitive processes that influence the regulation of behavioral responses and the selection of information to be attended to are based on task-related goals.

Controlled Oral Word Association Test

Current Knowledge

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▶ Frontal Lobe ▶ Vigilance

Neuroanatomical From a neuroanatomical perspective, brain networks other than those related to frontal lobe functions can be involved in controlled aspects of attention. For example, parietal lobe structures appear to be important for response intention and sensory selective attention. However, control behaviors such as response planning, selection, and execution are associated with networks of cortical and subcortical regions linked to the frontal lobes. It is these functions that are most commonly refer to when discussing control functions of the brain. Although there is not a clear anatomical mapping, studies suggest that different regions of the frontal lobe (and associated networks) relate to different aspects of control, such that there is a heterogeneity of functioning that would argue against any unitary conceptualization of attentional control (Cohen, 1993).

Cognitive Similarly, cognitive researchers have made an effort to move away from a homunculus view of control functions, pointing out that terms such as central executive (Baddely & Hitch, 1974), supervisory attentional system (Shallice, 1988), and executive control (e.g., Anderson & Green, 2001) all take on the role of a poorly defined black box and give the impression of a unitary concept. These terms may have been useful as placeholders, while cognitive researchers worked out the details of other related systems, such as the slave systems of Baddely and Hitch’s (1974) model of working memory. However, with an emerging focus on attentional control and with new tools for the direct study of phenomena such as task switching, response inhibition, goal formation, and planning in the form of functional neuroimaging (e.g., Shallice, Picton, Alexander, & Gillingham, 2008), computational modeling (O’Reilly, Braver, & Cohen, 1999), and statistical modeling techniques (Miyake et al., 2000), there is a growing body of information that specifies the subprocesses involved in these selection functions with increasing clarity (see also Monsell & Driver, 2000 for a review).

Cross References ▶ Divided Attention ▶ Focused Attention

References and Readings Anderson, M. C., & Green, C. (2001). Suppressing unwanted memories by executive control. Nature, 410, 366–369. Baddeley, A. D., & Hitch, G. (1974). Working memory. In G. H. Bower (Ed.), The psychology of learning and motivation: advances in research and theory (Vol. 8, pp. 47–89.). New York: Academic Press. Cohen, R. (1993). The neuropsychology of attention. New York: Plenum. Miyake, A., Friedman, N. P., Emerson, M. J., Witzki, A. H., Howerton, A., & Wager, T. D. (2000). The unity and diversity of executive functions and their contributions to complex ‘‘frontal lobe’’ tasks: A latent variable analysis. Cognitive Psychology, 41, 49–100. Monsell, S., & Driver, J. (2000). Attention and performance XVIII: Control of cognitive processes. Cambridge, MA: MIT Press. O’Reilly, R. C., Braver, T. S., & Cohen, J. D. (1999). A biologically-based neural network model of working memory. In P. Shah & A. Miyake (Eds.), Models of working memory. New York: Cambridge University Press. Shallice, T. (1988). Form neuropsychology to mental structure. Cambridge: Cambridge University Press. Shallice, T., Stuss, D. T., Picton, T. W., Alexander, M. P., & Gillingham, S. (2008). Mapping task switching in frontal cortex through neuropsychological group studies. Frontiers in Neuroscience, 2(1), 79–85.

Controlled Oral Word Association Test J ANET PATTERSON California State University East Bay Hayward, CA, USA

Synonyms Category fluency; CFL test; COWA; COWAT; F-A-S test; Letter fluency; Phonemic fluency; Verbal fluency

Description The Controlled Oral Word Association Test (COWAT) is a measure of verbal fluency and is a subtest of the Multilingual Aphasia Examination (MAE; Benton, Hamsher, & Sivan, 1994). The COWAT uses the three letter set of C, F, and L to assess phonemic fluency. Individuals are given 1 min to name as many words as possible beginning with one of the letters. The procedure is then repeated for the remaining two letters (see Strauss, Sherman, & Spreen, 2006 and Benton, Hamsher, Rey, & Sivan, 1994 for

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specific administration instructions). Several tests of phonemic fluency exist, some of which are part of larger test batteries (e.g., the MAE or the Neurosensory Center Comprehensive Examination for Aphasia; Spreen & Benton, 1977) and others that can be administered independently (e.g., the F-A-S Test). Verbal fluency is a cognitive function that facilitates information retrieval from memory. Successful retrieval requires executive control over cognitive process such as selective attention, mental set shifting, internal response generation, and self-monitoring. Tests of verbal fluency evaluate an individual’s ability to retrieve specific information within restricted search parameters (Lezak, Howieson, Loring, Hannay, & Fischer, 2004). The two most common parameters are semantic fluency and phonemic fluency.

Historical Background Borkowski, Benton, and Spreen (1967) were early proponents of systematically examining word fluency in persons with brain damage. They recognized that so-called word fluency tasks had been used in neuropsychological investigation of patients with brain damage and undertook two studies of the task. The first established the relations between word fluency and various English letters; the second examined word fluency data for persons with brain damage and control patients. They presented normative data for the first study and comparative data for the second, clearly supporting their hypotheses of the utility of word fluency assessment. Since publication of these data, word fluency tasks, and the COWAT in particular, have been investigated in detail. Spreen and Risser (2003) suggest that this assessment tool in its various forms has been one of the most frequently and thoroughly investigated neuropsychological assessment measures for unimpaired and neurologically impaired persons. A search of electronic databases confirms this suggestion.

Psychometric Data Psychometric data for the COWAT and other phonemic fluency tests, as well as other verbal fluency tasks (e.g., semantic fluency) are readily available. Norms have been published for children and adults of varying ages, levels of education, ethnic diversity, and geographical diversity (Loonstra, Tarlow, & Sellers, 2001; Strauss et al., 2006). Some differences have been noted between test forms, most notably, between the COWAT and F-A-S Test (Barry, Bates, & Labouvie, 2008); the CFL form appeared

more difficult, while results for the F-A-S form appeared more variable. In addition, COWAT scores have been correlated with neuropsychological measures such as reading tests and IQ tests.

Clinical Uses Scoring for the COWAT and other verbal fluency tests is straightforward. The examiner writes each word as it is produced by the individual. The transcript is reviewed and inadmissible words (i.e., repetitions, proper names, or slang) are eliminated. The test score is the total number of different words produced for all three letters (see Strauss et al., 2006 and Benton et al., 1994 for specific administration instructions). Supplementary scoring measures for the COWAT and other phonemic fluency tests provide additional information in clinical diagnosis and treatment. Supplementary scorning measures are error analysis, and cluster and switching analysis (see Table 1). In error analysis, the examiner notes any observable pattern of production of errors that suggests a loosening of executive control over cognitive processes that would result in errors. For example, a pattern of multiple repetitions of previous responses suggests perseveration and inefficient self-monitoring. Error patterns provide qualitative performance data and may appear as common patterns such as repetition of a word, or idiosyncratic patterns. Clustering and switching analyses evaluate the depth of the searchable knowledge base (clusters) and the cognitive flexibility within and across categories (switching) (Troyer, Moscovitcvh, & Winocur, 1977). An example of an efficient search strategy would be identifying a cluster or subcategory within the category (e.g., words that begin with ‘‘cr’’ in the COWAT task of naming words that begin with C) and producing as many items in that category as possible and then switching clusters (e.g., to words beginning with ‘‘cl’’). Clusters are scored by counting the number of clusters and calculating the mean cluster size; switches are scored by counting the number of transitions between clusters. Rules for scoring cluster size and number of switches appear in Troyer et al. (1997) and normative data for healthy adults appear in Troyer (2000). Scores from the COWAT are useful in evaluation of persons with neurogenic communication disorders, such as aphasia following stroke, traumatic brain injury, and dementia. Studies have included COWAT in the diagnostic batteries given to several patient populations and also in treatment studies as measures of behavioral change. The utility of the COWAT in identifying the nature


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Controlled Oral Word Association Test. Table 1 An example of F-A-S Test results and cluster and switch scoring for a person with aphasia

FAS-Test: ‘‘F’’

Responses

Clusters and Switches

fast, fun, fickle, fuschsia, finger of fate, four, fifty-four, forty, fornicate

Clusters = 8 (mean size = 1.1) fast fun fickle fuchsia finger of fate four fifty-four forty, fornicate

Words = 9

Switches = 7 FAS-Test: ‘‘A’’

Clusters = 5 (mean size = 1) apple aardvark alpaca ammonia arsenic

apple, aardvark, alpaca, ammonia, arsenic Words = 5

Switches = 4 FAS-Test: ‘‘S’’

substantial, sum, subtraction, stuck, structure, symbol, sympathy, stroke, sixty Words = 9

Clusters = 5 (mean size = 1.8) substantial, sum, subtraction stuck, structure symbol, sympathy stroke sixty Switches = 4

Total

Words = 23

and severity of performance deficits in clinical populations has been supported; however, conflicting findings have been reported. Typically, the total number of acceptable responses is the reported test result; however, increasingly cluster and switching scores are reported as well. The COWAT is valuable in detecting cognitive dysfunction, but it requires further study before definitive conclusions are possible with regard to performance patterns that can be linked with specific neurogenic behavioral deficits.

Cross References ▶ Aphasia ▶ Aphasia Tests ▶ Benton, Arthur

Clusters = 18 Mean size = 1.27 Switches = 15

▶ Boston Diagnostic Aphasia Examination ▶ Circumlocution ▶ Clustering ▶ Cognitive-Communication Disorder ▶ Cognitive Functioning ▶ Cognitive Processing ▶ Cue ▶ Cued Recall ▶ Error Recognition and Correction ▶ Free Recall ▶ Mayos Older American Normative Studies (MOANS) ▶ National Adult Reading Test ▶ Phonemic Cue ▶ Semantic Cue ▶ Semantic Fluency ▶ Verbal Mediation ▶ Western Aphasia Battery

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References and Readings Barry, D., Bates, M. E., & Labouvie, E. (2008). FAS and CFL forms of verbal fluency differ in difficulty: A meta-analytic study. Applied Neuropsychology, 15, 161–166. Benton, A. L., Hamsher, K., Rey, G. L., & Sivan, A. B. (1994). Multilingual Aphasia Examination (3rd ed.). Iowa City, IA: AJA Associates. Borkowski, J. G., Benton, A. L., & Spreen, O. (1967). Word fluency and brain damage. Neuropsychologia, 5, 135–140. Goodglass, H., Kaplan, E., & Baressi, B. (2001). Boston Diagnostic Aphasia Examination (3rd ed.). San Antonio TX: Psychological Corporation. Kertesz, A. (2006). Western Aphasia Battery. New York: Grune & Stratton. Lezak, M. D., Howieson, D. B., Loring, D. W., Hannay, H. J., & Fischer, J. S. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Loonstra, A. S., Tarlow, A. R., & Sellers, A. H. (2001). COWAT metanorms across age, education and gender. Applied Neuropsychology, 8, 161–166. Spreen, O., & Benton, A. L. (1977). Neurosensory Center Comprehensive Examination for Aphasia. Victoria BC: University of Victoria Neuropsychology Laboratory. Spreen, O., & Risser, A. H. (2003). Assessment of aphasia. Oxford: Oxford University Press. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). Compendium of neuropsychological tests: Administration, norms, and commentary (3rd ed., p. 502). Oxford: Oxford University Press. Troyer, A. K. (2000). Normative data for clustering and switching on verbal fluency tasks. Journal of Clinical and Experimental Neuropsychology, 22, 370–378. Troyer, A. K., Moscovitch, M., & Winocur, G. (1997). Clustering and switching as two components of verbal fluency: Evidence from younger and healthy adults. Neuropsychology, 11, 138–146.

Convulsion ▶ Grand Mal Seizure

Convulsive Disorder ▶ Epilepsy

COPD ▶ Chronic Obstructive Pulmonary Disease

Coping J OEL W. H UGHES Kent State University Kent, OH, USA

Synonyms Stress management

Definition

Contusion (Cerebral) ▶ Cortical Contusion

Conventional Antipsychotics ▶ Antipsychotics

Conversation Analysis ▶ Discourse Assessment

Conversion Disorder ▶ Cogniform Disorder ▶ Somatization

Coping is responding to environmental stimuli, events, and circumstances for the purpose of minimizing or managing stress, solving problems, and modulating physiological and emotional responses. Coping is often paired with stress (as the latter elicits the former) in what has become the stress and coping literature associated with Richard Lazarus and Susan Folkman. Stress responses typically involve appraising the stimulus or event, which begins the process of assigning value (e.g., distressing) and determining responses (e.g., fight, flight, freeze). Coping is a process that follows stress appraisals, and coping responses seek to manage stress with cognitive, physiological, and behavioral responses. Various coping strategies have been categorized, such as appraisalfocused, problem-focused, or emotion-focused coping.

Cross References ▶ Self-Regulation ▶ Stages of Adjustment

Coronary Artery Bypass Graft

▶ Stress ▶ Stress Management

References and Readings Carver, C. S., Scheier, M. F., & Weintraub, J. K. (1989). Assessing coping strategies: A theoretically based approach. Journal of Personality and Social Psychology, 56, 267–283. Lazarus, R. S., & Folkman, S. (1984). Stress, appraisal and coping. New York: Springer.

Core Battery ▶ Flexible Battery

Coronary Angioplasty ▶ Angioplasty

Coronary Artery Bypass Graft F LORA H AMMOND 1, LORI G RAFTON 2 1 Indiana University Indianapolis, IN, USA 2 Carolinas Rehabilitation Charlotte, NC, USA

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Patients undergo general anesthesia and a midline incision (median sternotomy) allows the surgeon to visualize the heart and vessels. The artery or vein grafts are then harvested. Frequently used vessels include the internal thoracic arteries, radial arteries, and saphenous veins. The heart is then stopped using a special mixture of chemicals and the patient is placed on cardiopulmonary bypass where the blood flow returning to the heart is diverted through a heart–lung machine that provides extracorporeal circulation and oxygenation. The graft is then sewn into place and the heart is restarted. The sternum is then closed with wires, and the incision is sutured.

Current Knowledge In the early years of cardiac surgery several investigators noted transitive postoperative delirium, whereas others reported immediate postoperative neurological abnormalities, however, no well-conducted or controlled studies were available. More recent studies have shown a short-term neuropsychological decline in the 7–14 days after CABG, including deficits in psychomotor speed, attention, verbal learning, and memory. Studies have reported an 11–75% incidence of postoperative cognitive decline (POCD) after cardiac surgery. Age is considered to be the strongest predictive factor of postoperative cognitive dysfunction. It is hypothesized that ‘‘off-pump’’ surgery causes less cognitive decline as it avoids extracorporeal circulation, but studies have not shown this to be true. Other potential causes of neurocognitive decline include intraoperative cerebral oxygen desaturation or microemboli.

References and Readings Synonyms CABG

Definition Coronary artery bypass surgery (CABG) is a surgical procedure for coronary artery disease in which arteries or veins from other parts of the body are grafted from the aorta to the coronary arteries in order to bypass the blocked portions. Indications for surgery include disease of the left main coronary artery and/or disease of all three coronary arteries and abnormal ventricular function. It may be performed in patients with severe angina which is unresponsive to medical management.

Eagle, K. A., Guylon, R. A., Davidoff, R., Ewy, G. A., Fonger, J., Gardner, T. J., et al. (1999). http://circ.ahajournals.org/cgi/content/ full/100/13/1464 Jensen, B. Ø., Rasmussenb, L. S., & Steinbru¨chel, D. A. (2008). Cognitive outcomes in elderly high-risk patients 1 year after off-pump versus on-pump coronary artery bypass grafting. A randomized trial. European Journal of Cardio-Thoracic Surgery, 34(5), 1016–1021. Slater, J. P., Guarino, T., Stack, J., Vinod, K., Bustami, R. T., Brown, J. M., III, et al. (2009). Cerebral oxygen desaturation predicts cognitive decline and longer hospital stay after cardiac surgery. Annals of Thoracic Surgery, 87(1), 36–44, discussion 44–45 Tarter, R. E., Butters, M., Beers, S. R. (2001). Medical neuropsychology (2nd ed., pp. 69–71). New York: Kluwer Academic. http://www.nlm.nih.gov/medlineplus/ency/article/002946.htm. Accessed 29 August, 2007. http://www.sts.org/sections/patientinformation/adultcardiacsurgery/cabg/ index.html?CFID=3659926&CFTOKEN=93793791. Accessed 29 August, 2007.

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Coronary Artery Disease

Coronary Artery Disease ▶ Coronary Disease

Coronary Disease E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Atherosclerotic heart disease; Coronary artery disease; Coronary heart disease; Ischemic heart disease

Definition Coronary disease, or coronary artery disease (CAD), results from atherosclerosis, or narrowing of the arteries that provide blood supply to the heart muscle (‘‘myocardium’’).

Current Knowledge Atherosclerosis, which is the process by which cholesterol and fat deposits adhere to the inside walls of blood vessels, creates ‘‘plaques’’ that block the blood supply through blood vessels. When this narrowing or occlusive process blocks the coronary artery, the accompanying obstruction to blood flow reduces the supply of oxygen and nutrients to the heart muscle, creating ischemia that causes chest pain (‘‘angina pectoris’’), or death of the cells of the heart (‘‘myocardial infarction’’). At times, it can cause other problems such as arrhythmias (abnormal heart rhythm) or congestive heart failure, in which the heart is unable to pump sufficient blood to the remainder of the body. CAD is the most common type of heart disease and the leading cause of death in the USA. Risk factors for CAD have been well studied; these include smoking, diabetes, hypertension, hyperlipidemia, obesity, sedentary lifestyle, excessive alcohol intake, and family history. Although chest pain is the most common symptom, dyspnea, palpitations, diaphoresis, nausea, radiation of the pain to the neck and left arm, and no symptoms at all, also are possible. The diagnosis relies on electrocardiogram, blood levels of cardiac enzymes, echocardiography, select

cardiac stress tests, and direct visualization on coronary angiography or other imaging studies. The treatment includes the use of beta-blockers, calcium channel blockers, angiotensin converting enzyme inhibitors, nitroglycerine, aspirin, or other medications; antihypertensive and lipidlowering agents; diet, exercise, and lifestyle changes; thrombolysis; angioplasty with coronary stent placement; and coronary bypass graft surgery.

Cross References ▶ Angioplasty ▶ Anticoagulation ▶ Antiplatelet Therapy ▶ Atherosclerosis ▶ Cholesterol ▶ Congestive Heart Failure ▶ Infarction ▶ Ischemia ▶ Myocardial Infarction ▶ Stent ▶ Thrombosis ▶ Tissue Plasminogen Activator ▶ Vasospasm

References and Readings Hansson, G. K. (2005). Inflammation, atherosclerosis, and coronary artery disease. New England Journal of Medicine, 352, 1685–1695. Virmani, R., Kolodgie, F. D., Burke, A. P., Farb, A., & Schwartz, S. T. (2000). Lessons from sudden coronary death: a comprehensive morphological classification scheme for atherosclerotic lesions. Arteriosclerosis, Thrombosis, and Vascular Biology, 20, 1262–1275.

Coronary Heart Disease ▶ Coronary Disease

Corporis Callosi ▶ Corpus Callosum

Correction Factor

Corpus Callosum J EFF D UPREE Virginia Commonwealth University Richmond, VA, USA

Synonyms Commissural magna; Corporis callosi; Interhemispheric commissure

Definition Corpus callosum is the largest axonal tract of the adult brain that provides symmetrical connections between the two hemispheres.

Current Knowledge The corpus callosum is the largest commissure of the adult brain that provides a bridge for the passing of information from one cerebral hemisphere to the other by 200–300 million myelinated and unmyelinated axons. The size of the corpus callosum varies greatly but is generally larger in females than in males. In the human, the corpus callosum begins development around the 11th week of gestation and continues through adolescence. Initially, the corpus callosum is composed of astrocytic processes, which serve as conduits for growing axons extending to the contralateral hemisphere. This interhemispheric commissure lies beneath the cortex at the bottom of the cerebral longitudinal fissure. It forms much of the roof of the lateral ventricles and is composed of four parts: the rostrum, the genu, the body (also known as the trunk), and the splenium. Each portion of the corpus callosum is responsible for connecting symmetrical regions of the two hemispheres with the rostrum and genu connecting portions of the prefrontal and premotor cotices, the body interconnecting the premotor, motor, supplementary motor, and the posterior parietal cortices, while the splenium connects portions of the temporal, occipital, and parietal lobes. Although being the largest white matter tract of the brain, the corpus callosum is not essential for life. The complete or partial absence of the corpus callosum is known as agenesis of the corpus callosum. This condition is rare, and estimates of incidence

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vary widely but are generally reported to range between 0.3 and 0.7% in the general population and as high as 3% in individuals with development disabilities. Agenesis of the corpus callosum results in symptoms ranging from asymptomatic with normal intelligence to intellectual retardation, seizures, hydrocephalus, and spasticity. In individuals suffering from severe epilepsy, a surgical procedure known as corpus callostomy may be performed, which involves the partial or complete transaction of the corpus callosum. This procedure is only performed in patients who are at risk of accidental injury resulting from severe seizures. The layman’s term for this condition is split brain due to the loss of interhemispheric communication. Much of the original research detailing the consequence of corpus callostomy was conducted by Dr. Roger Wolcott Sperry.

Cross References ▶ Ganglion ▶ Gazzaniga, M. S. (1939– ) ▶ Sperry, Roger Wolcott (1913–1994) ▶ Split-brain

References and Readings Barr, M., & Kiernan, J. (1983). The human nervous system – An anatomical viewpoint (4th ed.). Philadelphia: Harper and Row. Gazzaniga, M. S. (2005). Forty-five years of split-brain research and still going strong. Nature Reviews Neuroscience, 6(8), 653–659. Haines, D. (2006). Fundamental neuroscience for basic and clinical applications (3rd ed.). Philadelphia: Churchill Livingstone Elsevier. Kandel, E., Schwartz, J. H., & Jessel, T. M. Principles of neural science (4th ed.). New York: McGraw-Hill. Paul, L. K., Brown, W. S., Adolphs, R., Tyszka, J. M., Richards, L. J., Mukherjee, P., et al. (2007). Agenesis of the corpus callosum: Genetic, developmental and functional aspects of connectivity. Nature Reviews Neuroscience, 8(4), 287–299.

Corpus Mamillare ▶ Mammillary Bodies

Correction Factor ▶ K Scale

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Correlation Coefficients

Correlation Coefficients C HRISTIAN S CHUTTE , B RAD N. A XELROD John D. Dingell VA Medical Center Detroit, MI, USA

Synonyms Pearson r, r, rho

Definition A measure of the strength of the relationship between two variables, x and y.

plot of the data. The values of a correlation can range between þ1 and 1 with these two extremes representing perfect relationships, which rarely if ever occur in research, and a correlation coefficient of 0 would represent no relationship, given certain assumptions are made. A line can be drawn through data that are related that generally approximates a regression line, which is how Galton identified the first regression line in his heritability studies with peas. Correlation is a measure of the strength of the relationship between two variables. Thus, if data in a scatter plot are randomly scattered, such as in the example below, then there is no relationship, as no single line adequately represents the data and the correlation would be roughly 0. 10 8

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The correlation, or co-relation, coefficient is typically attributed to Karl Pearson who developed the formalized idea of correlation during the mid- to late 1800s. However, the beginning of the idea of correlation may have come from Sir Francis Galton, cousin of Charles Darwin. Galton worked on genetic heritability of sweet peas. Through his work on heritability, he developed the beginnings of regression and correlation. Pearson, who worked in Galton’s lab and was later his biographer, attributed the development of the regression slope to Galton. Pearson then generalized Galton’s work into the Pearson Product Moment Correlation (PPMC), or ‘‘moment’’ meaning the average of a set of products. The PPMC is often identified as ‘‘r,’’ which Galton originally used to denote ‘‘regression’’ and Pearson later used as notation for correlation. In 1896, Pearson published an article in which he credited Bravais for developing the rudiments of the correlation formula. In this work published in the Philosophical Transactions of the Royal Society of London, Pearson showed that the optimum values for the regression line and correlation could be derived from the product moment or S xy=n, where x and y are deviations from their respective means, usually expressed as standard z scores and n is the number of pairs.

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If data are positively related, such that as one variable increases in value so does the other, then one would be able to draw a line that would approximately represent the data. The below example illustrates an ideal relationship that rarely occurs in research of a þ 1 correlation such that for every one unit that x goes up, y goes up one unit. 30

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Current Knowledge A correlation, or co-relation, of two variables can be visually scanned initially by simply looking at a scatter

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There may also be negative associations, such that for every unit that x goes up, y goes down, or a 1 relationship, such as the below idealized example.


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However, typically a scatter plot will not be as obvious and the data will look more like an ellipse such as in the example below, which still illustrates a strong correlation of over .8.

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The above examples show linear relationships between variables, which is an assumption of most correlation coefficients. An examination of scatter plots can also show nonlinear relationships, which are not well represented by standard correlation coefficients, such as the PPMC, but can be shown with other methods, such as eta. The below example has a near 0 Pearson r, but the variables are clearly related nonlinearly. 25 20

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The correlation coefficient is therefore a measure of the extent to the relation of pairs of observed data points can be expressed as a linear function. The greater the summed square of differences between the observed data and the values described by the linear function, the lower the correlation coefficient. The closer the observed data points lie to the line of best fit, the higher the correlation coefficient. There are several forms of correlation that need to be addressed including those for continuous variables, categorical variables, and correlations that are better descriptions of nonlinear and skewed relationships. The scope of this article is to cover the basic foundations of correlation coefficients. The use of correlation ranges far and wide and a thorough examination of this subject is the subject of chapters and entire texts referenced below. The object of this entry is to cover foundational issues related to correlation coefficients. There are more correlation coefficients than those presented below, though several different forms of correlation and their properties are considered. It is important to note that correlation, even a strong correlation, does not infer causation. In other words, simply because two variables are related does not mean that one causes the other. For example, you need light to read this text. Simply because the light is on while you are reading does not mean that light is causing you to read, they are certainly related, as there will always be some light source when you read, but one does not cause the other. A determination of causation requires adequate experimental design. There are certain important components that underlie most correlations. Correlations are most meaningful when certain criteria are met, and different correlation coefficients are affected differently by failure to meet different assumptions. The Pearson product moment correlation coefficient has the most stringent set of assumptions. These include: 1. The data are interval: The level of measurement must at least be interval as opposed to nominal or ordinal, though this can be managed if it is violated. 2. The data must have a linear relationship: Most correlations assume that the data are linearly related, as opposed to nonlinear. To the degree which the data are better explained by a nonlinear relationship correlation coefficients will underestimate the relationship. Prior to completing a correlation analysis this can be examined by looking at a scatter plot, as discussed above. 3. The variables have similar and normal underlying distributions (more formally in a bivariate correlation, such as the Pearson r): To the degree that the

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distributions of the different variables differ, the correlation will attenuate the relationship between them. For example, if correlating a variable that is normally distributed, like height, with one that is highly skewed like ability to walk. This is done when correlating a continuous variable with a dichotomous variable or when correlating interval data with ordinal. If this assumption is violated to a significant degree then rank order correlation may be used, such as the Spearman’s rho or other nonparametric correlations. The data must have homoscedasticity: The error, or random variance in X and Y, contained in the data, is assumed to be equal along the entire distribution. If, for example, one portion of the distribution has a much larger random variance, then the correlation may overrepresent the relationship because it would underrepresent the amount of variance that is attributable to error. If data are heteroscedastic, then the distribution will not be normal and the correlation may misrepresent the data. The data has no or limited outliers: Correlation is sensitive to outliers. Some forms of correlation are more robust to a violation of this assumption than others, but normally correlation is a representation of deviations of mean values. Thus, to the degree that the mean is affected by outliers then the correlation will be also. If there are many outliers, or few that are large, this problem can be managed through use of a Spearman rho, which ranks data. There is limited measurement error present in the sample: It is assumed that the measurement is relatively free from or has limited error. In other words, if one has an invalid test that only measures error then the correlation will be near zero, as the data will be random and show little relationship. It is rare in behavioral research to be free from error, but limiting it is an important aspect of experimental control. The data have adequate variance: In order to adequately assess relationships among variables there must be variance among the variables. In general, the more variance the higher the correlation will be.

To the extent that these criteria are violated, which they often are, the correlation coefficient is more or less impacted depending on the type of correlation. There are several different methods calculating basic correlation coefficients depending on the type and characteristics of the data being analyzed. There are several different types of correlation that depend on the level of measurement (nominal, ordinal, interval, ratio) being made. Most are special cases of the Pearson r. There are

also other methods that are more robust to violations of the above assumptions, such as Spearman’s rho or eta in the case of a nonlinear relationship. The correlation coefficient is considered a measure of effect size, which is a measure of how different two distributions are. By convention a correlation of .1 is small, .3 moderate and .5 large (Cohen, 1988), at least in the behavioral sciences that can expect weaker relationships in general due to the complexity of the subject matter. For example, the correlation between IQ and academic achievement is approximately .55 (Griffenstein & Baker, 2003), but one would expect no effect for a correlation between IQ and height. The correlation coefficient can also be squared to give the coefficient of determination, which is the amount of variance in one variable that is accounted for by another. For example, if one had a correlation of .55 between IQ and academic achievement then the coefficient of determination (r 2) would be .30, which means that approximately 30% of the variance in academic achievement is accounted for by IQ. 1. PPMC or r: The PPMS is an association of two continuous variables that shows the degree of linear relationship between them. It can be calculated using raw scores, deviation scores, or by using a covariance formula. A common method is to calculate the standard score (or z score) and sum the products of the two variables and divide them by the degrees of freedom (n 1) or S xy=n S 1. 2. Point-biserial or rpb: This is a special case of the PPMC for use when there is a continuous variable, such as height, and a dichotomous nominal variable, such as gender. The point-biserial correlation is for naturally dichotomous variables, such as gender, not artificially dichotomized variables, such as taking a naturally continuous distribution, such as intelligence, and making it into high and low intelligence. 3. Phi: This is a special case of the PPMC for use when both variables are dichotomous and nominal. Note that as with rpb this is for naturally dichotomous variables, such as gender, not artificially dichotomized variables. 4. Biserial or rb: This is for use when there is one continuous variable, such as height, and a dichotomized variable, such as high and low intelligence. So, the biserial correlation measures the relationship between X and Y as if Y were not artificially dichotomized. This is similar to the point-biserial, but the formula is designed to replace some of the variance that is lost. One of the important components listed above is that the data have adequate variance. However, when a variable is dichotomized much of the variance is lost.

Cortical Blindness

Thus, the biserial formula replaces some of that variance, which always yields a higher correlation than a point-biserial correlation. 5. Tetrachoric: This is similar to the phi, except that it is assumed that there is a continuous distribution underlying the dichotomized variables and it thusly replaces some of that variance, as opposed to phi that assumes that the two variables are naturally dichotomous, such as gender and employment status. 6. Spearman’s rho or rs or r: Spearman’s rho is a nonparametric statistic. In other words, it makes no assumptions about the underlying distribution of the variables. One of the primary components of the typical PPMC is that there is a normal or bell-shaped distribution underlying the variable. However, in many cases, distributions are highly skewed. For example, if one were to look at a distribution of people who live into adulthood in the USA, the distribution would likely be very skewed to the left, meaning that the vast majority of people live at least until they are of adult age. Relate that distribution to the number of people who vote, which would be positively skewed, meaning that few people of adult age vote proportionally and a PPMC would not yield an inaccurate representation, as this distribution significantly violates the assumptions of the PPMC. Thus, one could convert raw scores into ranks and complete the correlation. This is more robust to violations of some of the underlying components of correlation. 7. Measures of nonlinear relationships: Eta is a measure of association that can be used if the data are nonlinear, as in the final scatter plot above. It is a measure of the strength of the effect and is always positive. Eta is always higher than jrj and is therefore a biased estimate of the effect. In practical usage, many of the different correlation coefficients are calculated using the same method, such as the PPMC and the point-biserial, given the ubiquity of computer statistical packages. What is important to note with any correlation being used are the number and degree of the components that are violated and what impact that has on the relationship between the variables. For example, if one is using a PPMC to assess highly skewed data then the relationship may be very low, where actually, if a nonparametric method is used, such as the Spearman’s rho, then the relationship may be significant. Conversely, if a PPMC is used on data that have many large outliers the relationship may look strong and be significant, but in reality it is the impact of those large outliers that are falsely raising the strength of the associations.

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Cross References ▶ Statistical Significance ▶ z Score

C References and Readings Chen, P., & Popovich, P. (2002). Correlation: Parametric and nonparametric measures. Thousand Oaks, CA: Sage. Cohen, J. (1988). Statistical power analysis for the behavioral sciences (2nd ed.). New Jersey: Erlbaum.

Cortex ▶ Cerebral Cortex

Cortical Activation ▶ Arousal

Cortical Arousal ▶ Arousal

Cortical Blindness S TEVEN Z. R APCSAK , G. A LEX H ISHAW University of Arizona Tucson, Arizona, USA

Synonyms Anton’s syndrome; Blindsight

Short Description or Definition Cortical blindness refers to severe visual loss produced by bilateral damage to the geniculostriate visual

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Cortical Blindness

pathways. The underlying pathophysiological mechanism involves direct destruction and/or de-afferentation of primary visual cortex (striate cortex, Brodmann area 17, or V1). The term Anton’s syndrome is applied to patients with cortical blindness who demonstrate explicit denial or unawareness (anosognosia) of their visual impairment.

Categorization Although no universally agreed upon classification system exists, patients with cortical blindness differ in terms of the severity of the visual loss, the presence/absence of spared visual abilities (conscious or unconscious), the degree of awareness of the deficit, and the extent of functional recovery. There are also variations across cases with respect to the capacity to generate internal visual representations using mental imagery and the susceptibility to experience abnormal visual sensations/hallucinations. The precise neurobiological mechanisms underlying these individual differences in clinical presentation remain to be determined.

Epidemiology Cortical blindness is a relatively rare condition, typically caused by bilateral occipital strokes in the territory of the posterior cerebral arteries. Other less common etiologies include anoxic brain damage, carbon monoxide poisoning, head trauma, and occipital lobe tumors. Transient cortical blindness can be seen in the context of the reversible posterior leukoencephalopathy syndrome (RPLS) related to hypertensive encephalopathy, the use of chemotherapeutic and immunosuppressant drugs, or the injection of radiological contrast agents during cerebral angiography.

Natural History, Prognostic Factors, Outcomes Although some recovery of visual function is observed in most cases of cortical blindness, patients with neuroimaging evidence of extensive structural damage to occipital cortex typically do not regain full normal vision. Residual visual field defects are common, and cortical blindness may evolve into visual agnosia characterized by a persistent inability to recognize objects, faces, or words despite the return of more elementary visual functions. It is

currently unclear whether recovery of visual capacity in cortical blindness is mediated by spared neural tissue within primary visual cortex, or whether it reflects the strengthening and increasing utilization of alternative visual pathways to extrastriate cortical regions that bypass the damaged geniculostriate system, or both. Anosognosia for visual loss in patients with Anton’s syndrome also tends to diminish over time, often parallel to the resolution of the visual impairment, but partial defects of awareness are not uncommon. In cases of transient cortical blindness due to RPLS, full recovery of vision can occur within a few days along with complete resolution of the characteristic neuroradiological abnormalities.

Neuropsychology and Psychology of Cortical Blindness In severe cases of cortical blindness, all forms of conscious visual perception may be abolished. However, this type of total visual loss is relatively uncommon and many patients retain at least some rudimentary visual awareness of motion or light. Visual form discrimination is profoundly impaired and objects, faces, or written words cannot be identified. Visuospatial orientation is also severely compromised and patients may repeatedly bump into objects when attempting to navigate in their environment. Although individuals with cortical blindness do not have normal conscious awareness of visual events, they can sometimes demonstrate surprising ability to respond to stimuli presented within the blind portions of their visual fields. Specifically, patients may be able to detect, locate, and discriminate visual stimuli that they claim not to see. Residual visual capacity within the cortically blind field in the absence of conscious awareness has been referred to as ‘‘blindsight’’ (Weiskrantz, 1986; Stoerig & Cowey, 1997, 2007; Stoerig, 2006). The fact that blindsight can be observed in patients with extensive destruction or de-afferentation of primary visual cortex suggests that the preserved visual abilities of these individuals are mediated by neural pathways from retina to extrastriate visual cortex that bypass the damaged geniculostriate system. There are in fact a number of alternative non-geniculostriate pathways capable of transmitting visual information to a variety of temporo-parietal extrastriate cortical areas via subcortical relay nuclei in the midbrain and diencephalon (Weiskrantz, 1986; Stoerig & Cowey, 1997, 2007; Stoerig, 2006). The specific visual functions retained in blindsight may depend on which of these multiple parallel visual pathways are available to patients. For instance, the

Cortical Blindness

residual capacity to detect motion and to locate and manually grasp targets presented in the blind field may depend on projections from the retina to the superior colliculus, with additional connections to the pulvinar and to middle temporal (MT/V5) and dorsal parietal cortex (Danckert & Rossetti, 2005). Other alternative pathways from retina to ventral extrastriate cortex or amygdala might be involved in mediating unconscious visual form discrimination (Trevethan, Sahraie, & Weiskrantz, 2007) and implicit recognition of emotional facial expressions (Morris, DeGelder, Weiskrantz, & Dolan, 2001; Pegna, Khateb, Lazeyras, & Seghier, 2005). Functional imaging studies in patients with blindsight are consistent with the notion that unconscious processing of visual information depends on non-geniculostriate visual pathways. Specifically, these investigations have demonstrated activation in midbrain/thalamic nuclei, extrastriate cortical areas, and the amygdala in the absence of concomitant activity in the lesioned primary visual cortex during visual stimulation of the blind field (Sahraie, Weiskrantz, Barbur, Simmons, Williams, & Brammer, 1997; Stoerig, 2006; Morris et al., 2001; Pegna et al., 2005). Patients with cortical blindness may demonstrate unawareness of their profound visual impairment. This striking clinical condition is referred to as Anton’s syndrome. Anosognosia for visual loss can take several different forms. In extreme cases, patients emphatically deny being blind and produce confabulatory responses when questioned about their visual abilities. Other patients acknowledge a change in their vision but they typically offer a variety of excuses to explain the difficulty, attributing it to poor lighting conditions or to a problem with their eyeglasses. A number of theories have been advanced to explain anosognosia for blindness (Bisiach & Geminiani, 1991; Heilman, 1991; Celesia, Brigell, & Vaphiades, 1997; Adair, Schwartz, & Barrett, 2003). For instance, it has been proposed that normal visual awareness depends on a hypothetical monitor located in extrastriate visual association areas that receives afferent input from primary visual cortex (Heilman, 1991). In addition to evaluating activity within the visual areas of the brain, the monitor also sends efferent information to cortical language areas enabling subjects to verbally comment on their visual experiences. Lesions that disrupt the input and/or output connections of the visual monitor, or produce damage to the monitor itself, result in anosognosia for visual impairment that may include verbal denial of the deficit and the production of confabulatory responses by the disconnected language areas. It has also been suggested that in cases of cortical blindness, internally generated visual experiences in the form of hallucinations or

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visual imagery may provide faulty input to an otherwise intact monitor. The loss of normal visual input following damage to primary visual cortex may in fact give rise to frequent ‘‘release’’ hallucinations in patients with cortical blindness due to increased cortical excitability in de-afferented extrastriate areas. Furthermore, since visual perception and imagery are mutually inhibitory under normal circumstances, the absence of bottom-up activation by geniculostriate afferent signals may be accompanied by a relative enhancement of visual imagery mediated by unopposed top-down activation of sensory representations in extrastriate cortex. Patients may deny blindness because they continue to experience internally generated visual sensations and may mistake these for veridical perceptions resulting from retinal stimulation by external visual events. To understand anosognosia for visual loss, it is useful to briefly consider what is currently known about the neural correlates of normal visual awareness. In this context, it is important to emphasize that conscious awareness of visual events normally entails the capacity to acknowledge, describe, reflect upon, and make appropriate cognitive judgments about ongoing visual experiences (Weiskrantz, 1997; Block, 2005; Dehaene, Changeux, Naccache, Sackur, & Sergent, 2006). Thus, awareness and the ability to provide introspective report or commentary about the quality, content, and veridicality of visual perceptions are closely related functions. Although localized neural activity in cortical visual areas (striate and extrastriate cortex) is obviously necessary for visual information to reach this ‘‘commentary stage’’ of conscious awareness, it is by itself not sufficient (Weiskrantz, 1997; Rees, Kreiman, & Koch, 2002; Dehaene et al., 2006). In particular, evidence from recent neuroimaging studies suggests that conscious vision requires the additional recruitment of dorsolateral prefrontal and parietal cortical regions implicated in visual attention and working memory (Rees, Kreiman, & Koch, 2002; Dehaene et al., 2006). Prefrontal cortex is also involved in mediating strategic cognitive operations necessary for critically evaluating and interpreting the meaning and significance of visual experiences in order to determine the most appropriate behavioral response. Activation of frontal and parietal regions by input from cortical visual areas is also likely to play an essential role in awareness of abnormal visual function. Specifically, damage to primary visual cortex and extrastriate areas may give rise to visual field defects and/or domainspecific impairments in processing distinct visual stimulus attributes (e.g., form, color, motion). If the same lesions also interfere with the bottom-up activation of the frontoparietal cortical network that normally enables conscious

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awareness and introspective report about visual experiences, anosognosia for the visual impairment ensues. Defective awareness of visual function may also result from direct damage to the putative fronto-parietal network that provides the critical top-down attentional amplification required for perceptual information processed within visual cortical modules to enter consciousness, or it may be produced by lesions that disrupt feedforward/feedback connections between fronto-parietal cortex and visual processing areas. In summary, conscious awareness of both normal and abnormal vision requires dynamic reciprocal interactions between cortical visual areas and specialized regions within frontal and parietal cortex. Lesions that disrupt the spatial distribution, intensity, or timing of activation across the different functional components of this large-scale neural system may give rise to a variety of clinical conditions characterized by unawareness of preserved or impaired visual processing, including blindsight, visual neglect, and anosognosia for blindness.

Evaluation Neuro-ophthalmological examination in cortical blindness confirms the normal fundoscopic appearance of the retinas and optic nerves. The pupillary light response is characteristically preserved, as the retinal fibers that mediate this reflex leave the optic tract prior to the origin of the geniculostriate pathways. Reflexive blinking to threatening visual gestures is usually absent, and in the majority of cases optokinetic nystagmus cannot be elicited by moving a striped object in front of the patient’s eyes. The dissociation between preserved pupillary light response and absent optokinetic nystagmus can have diagnostic utility in distinguishing patients with cortical blindness from patients with severe peripheral visual loss due to bilateral eye or optic nerve pathology in whom both responses are lost, and also from individuals with psychogenic blindness in whom both responses are retained. Clinical evaluation of visual function in patients with cortical blindness should include tests of visual acuity, determination of light and motion perception in different sectors of the visual field, tests of spatial localization, as well as assessments of color perception, object and face recognition, and reading ability. Studying blindsight usually requires the use of specialized testing equipment in an experimental laboratory environment. However, in the clinical setting blindsight can be tested by requiring patients to guess the presence/absence, location,

movement, or identity of objects presented in their blind field using a forced-choice response method (e.g., was it a key or a coin?). Better than chance performance on these types of tasks is taken to be indicative of blindsight. Alternatively, patients can be asked to try to reach for and grasp objects in their blind field. Regardless of the testing method used, it is important to establish after each trial whether the patient had any conscious awareness of the visual stimulus. Visual imagery can be tested by asking the subjects to answer questions about the visual attributes of familiar objects or animals (e.g., do polar bears have long or short tails?). Patients should be questioned about abnormal visual perceptions and hallucinations. Unawareness of deficit may be revealed by spontaneous comments or behavior, but patients should also be specifically asked to describe the quality and content of their visual experiences. The severity of anosognosia can be quantified by simple rating scales (Bisiach & Geminiani, 1991; Celesia, Brigell, & Vaphiades, 1997). Structural neuroimaging (CT/MRI) studies in patients with cortical blindness due to stroke typically reveal extensive bilateral infarctions involving primary visual cortex and underlying white matter, often with evidence of lesion extension into adjacent extrastriate visual association areas (Brodmann areas 18/19, 37) (Figure 1). SPECT/PET scans frequently demonstrate blood flow/metabolic abnormalities that extend beyond the boundaries of the lesions seen on CT/MRI, providing evidence that the cortical visual areas that appear spared by structural imaging studies are in fact functionally compromised. In patients with transient cortical blindness due to RPLS, neuroimaging studies have demonstrated reversible bilateral subcortical white matter abnormalities in posterior occipito-temporo-parietal regions attributable to vasogenic edema.

Treatment A number of behavioral approaches have been tried with varying degrees of success to restore visual function in the cortically blind field and/or help patients learn alternative strategies to compensate for their visual impairment (Kerkhoff, 2000). Attempts to increase awareness of the visual deficit in patients with anosognosia constitute an important component of the treatment program. It has been shown that blindsight performance can be improved by training, and the use of these techniques may aid the recovery of vision in individuals with cortical blindness (Stoerig, 2006; Sahraie et al., 2006).

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Cortical Blindness. Figure 1 Diffusion-weighted MRI scan in a patient with cortical blindness following bilateral posterior cerebral artery strokes. Note massive destruction of primary visual cortex (Brodmann area 17), with lesion extension into ventral extrastriate visual association areas (Brodmann areas 18,19,37). This individual demonstrated complete denial of blindness (anosognosia), consistent with Anton’s syndrome

Cross References ▶ Anosognosia ▶ Visual Hallucinations

References and Readings Adair, J. C., Schwartz, R. L., & Barrett, A. M. (2003). Anosognosia. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (pp. 193–197). New York: Oxford University Press. Bisiach, E., & Geminiani, G. (1991). Anosognosia related to hemiplegia and hemianopia. In G. P. Prigatano & D. L. Schacter (Eds.), Awareness of deficit after brain injury: Clinical and theoretical issues (pp. 17–39). New York: Oxford University Press. Block, N. (2005). Two neural correlates of consciousness. Trends in Cognitive Sciences, 9, 46–52. Celesia, G. G., Brigell, M. G., & Vaphiades, M. S. (1997). Hemianopic anosognosia. Neurology, 49, 88–97. Danckert, J., & Rossetti, Y. (2005). Blindsight in action: what can the different sub-types of blindsight tell us about the control of visually guided actions? Neuroscience and Biobehavioral Reviews, 29, 1035–1046. Dehaene, S., Changeux, J. -P., Naccache, L., Sackur, J., & Sergent, C. (2006). Conscious, preconscious, and subliminal processing: A testable taxonomy. Trends in Cognitive Sciences, 10, 204–211. Heilman, K. M. (1991). Anosognosia: Possible neuropsychological mechanisms. In G. P. Prigatano & D. L. Schacter (Eds.), Awareness of deficit after brain injury: Clinical and theoretical issues (pp. 53–62). New York: Oxford University Press.

Kerkhoff, G. (2000). Neurovisual rehabilitation: Recent developments and future directions. Journal of Neurology, Neurosurgery, and Psychiatry, 68, 691–706. Morris, J. S., DeGelder, B., Weiskrantz, L., & Dolan, R. J. (2001). Differential extrageniculostriate and amygdala responses to presentation of emotional faces in a cortically blind field. Brain, 124, 1241–1252. Pegna, A. J., Khateb, A., Lazeyras, F., & Seghier, M. L. (2005). Discriminating emotional faces without primary visual cortices involves right amygdala. Nature Neuroscience, 8, 24–25. Rees, G., Kreiman, G., & Koch, C. (2002). Neural correlates of consciousness in humans. Nature Reviews Neuroscience, 3, 261–270. Sahraie, A., Weiskrantz, L., Barbur, J. L., Simmons, A., Williams, S. C. R., & Brammer, M. J. (1997). Pattern of neural activity associated with conscious and unconscious processing of visual signals. Proceedings of the National Academy of Sciences, 94, 9406–9411. Sahraie, A., Trevethan, C. T., MacLeod, M. J., Murray, A. D., Olson, J. A., & Weiskrantz, L. (2006). Increased sensitivity after repeated stimulation of residual spatial channels in blindsight. Proceedings of the National Academy of Sciences, 103, 14971–14976. Stoerig, P. (2006). Blindsight, conscious vision, and the role of primary visual cortex. Progress in Brain Research, 155, 217–234. Stoerig, P., & Cowey, A. (1997). Blindsight in man and monkey. Brain, 120, 535–559. Stoerig, P., & Cowey, A. (2007). Blindsight. Current Biology, 17, R822–R824. Trevethan, C. T., Sahraie, A., & Weiskrantz, L. (2007). Form discrimination in a case of blindsight. Neuropsychologia, 45, 2092–2103. Weiskrantz, L. (1986). Blindsight: A case study and implications. Oxford: Clarendon Press. Weiskrantz, L. (1997). Consciousness lost and found. Oxford: Oxford University Press.

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Cortical Color Blindness

Cortical Color Blindness ▶ Achromatopsia

Cortical Contusion B ETH R USH Mayo Clinic Jacksonville, FL, USA

Synonyms

Cross References ▶ Contrecoup Injury ▶ Edema ▶ Focal Lesion, Contusion ▶ Intracranial Pressure

References and Readings Bigler, E. D. (2001). The lesions in traumatic brain injury: Implications for clinical neurophysiology. Archives of Clinical Neuropsychology, 16, 95–131. Graham, D. I., Saatman, K. E., Marklund, N., Conte, V., Morales, D., Royo, N., & McIntosh, T. K. (2006). The neuropathology of trauma. In R. W. Evans (Eds.), Neurology and Trauma (2nd ed., pp. 45–94). New York: Oxford University Press.

Bruise; Contusion (cerebral)

Definition Cortical contusions are bruises on the brain tissue that form from the small blood vessel leaks (veins and arteries covering the parenchymal tissue), or a series of microhemorrhages following trauma. Trauma is usually the result of physical blows to the head such as those sustained in a motor vehicle accident, direct blow to the head from assault, or significant sports-related injuries. Veins and arteries on the surface of the brain are damaged, which results in bleeding and bruising. When the blood vessel is torn, blood escapes from the vessel at a rate that is faster than the blood that can be absorbed by the brain. Consequently, cortical contusions commonly result in edema and increased intracranial pressure.

Current Knowledge Second to diffuse axonal injury, cortical contusion is the most common type of intra-axial lesion following brain trauma. By radiologic definition, a cortical contusion must involve some portion of the superficial gray matter. Because gray matter has more vasculature than white matter, most cortical contusions are hemorrhagic, whereas diffuse axonal injuries are rarely hemorrhagic. The frontal and temporal lobes are the most common sites of cortical contusions. When present, cortical contusions are usually found bilaterally. Compared to diffuse axonal injury lesions, cortical contusions are much less likely to involve an initial presentation of coma or altered loss of consciousness.

Cortical Deafness ▶ Pure Word Deafness

Cortical Lewy Body Disease (CLBD) ▶ Dementia with Lewy Bodies

Cortical Magnification R ONALD A. C OHEN Brown University Providence, RI, USA

Definition Cortical magnification refers to the fact that the number of neurons in the visual cortex responsible for processing the visual stimulus of a given size varies as a function of the location of the stimulus in the visual field. Stimuli occurring in the center of the visual field that have been detected in the fovea of retina are processed by a very large number of neurons in the primary visual cortex of the occipital lobe, though these neurons handle only a very small region of the central visual field. Conversely,

Cortical Mapping

stimuli detected in the peripheral visual field tend to be processed by a much smaller number of neurons in the primary visual cortex.

Current Knowledge Cortical magnification reflects an important concept in the field of cognitive neuroscience; the cortical volume, and ultimately the number of neurons allocated to a particular function, typically varies as a function of the significance of the function. For example, since the sense of touch is particularly important for the hands, there are many more nerve receptors in the finger tips than in the trunk of the body. Similarly, the volume of motor cortex dedicated to controlling the hands and mouth in humans is much greater than the volume dedicated to large muscle groups with more limited action. Given the critical role that vision plays in human cognition, tremendous magnification of neurons is dedicated to visual processing, with this magnification occurring at various processing stages along the visual pathways beginning in the retina. The extent of cortical magnification is often expressed as a ratio of millimeters of cortical surface per degree of visual angle. This ratio varies across visual areas. Among primates, neurons devoted to processing foveal input from the retina are about 100 times more prevalent than neurons devoted to peripheral stimuli in the primary visual cortex (Daniel & Whitteridge, 1961). The principle of cortical magnification indicates an important relationship between the number of neurons dedicated to big or small visual angles and the receptive field of those neurons. When a large number of neurons are involved in a small visual angle, there is inherently a large processing capacity being assigned to a smaller area of visual focus. Conversely, a smaller number of neurons handling a visual angle are indicative of a larger receptive field, as each neuron must be sensitive to changes occurring across a larger area of space. This creates an inherent relationship between the spatial frequency of the visual information being processed and the size of the receptive field that is essential for considering how neurons across different visual cortical areas are tuned to respond to the featural and spatial characteristics of visual input. A consequence of this organization for the primary visual cortex is that visual acuity and the ability to detect small features of stimuli are best in the center of the visual field and poorest in the periphery. Yet, broad spatial changes with movement are easily detected at the periphery. Since visual cortical areas differ in their emphasis on

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specific informational characteristics (e.g., shape, color, texture, position, movement), neurons with greatest sensitivity to these dimensions vary as a function of cortical magnification factors and a tradeoff of size of the receptive field and the number of neurons dedicated to each part of the field.

Cross References ▶ Feature Detection ▶ Magnocellular Neurons ▶ Parvocellular Neurons ▶ Spatial Frequencies ▶ Spatial Processing

References and Readings Daniel, P. M., & Whitteridge, D. (1961). The representation of the visual field on the cerebral cortex in monkeys. Journal of Physiology, 159, 203–221.

Cortical Malformation ▶ Heterotopia

Cortical Mapping M ARLA J. H AMBERGER Columbia University New York, NY, USA

Synonyms Direct stimulation mapping; Electrical stimulation mapping (ESM); Functional mapping

Description Cortical mapping is an invasive procedure in which electrical stimulation is applied briefly to the cortical

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surface for the purpose of identifying areas critical for sensory, motor or language function. This procedure is utilized when brain surgery involves the removal or disruption of potentially functional cortical areas. Sites identified via cortical mapping are typically spared from resection, with the goal of preserving function postoperatively. Stimulation is applied using a bipolar stimulator, usually via pairs of adjacent subdural electrodes. The procedure can be conducted intra-operatively in a conscious patient before resection of brain tissue, or extra-operatively, if subdural electrodes have been implanted, most commonly in pharmacologically resistant epilepsy patients who require intracranial EEG monitoring to delineate the region of seizure onset (Fig. 1). The identification of sensory and motor sites is based on stimulation-evoked ‘‘positive’’ responses, such as a subjective sensation (e.g., tingling) or an observable movement (e.g., muscle twitch). In contrast, stimulation of language cortex elicits no experiential or observable response in an inactive patient. Rather, cortical language mapping relies on ‘‘negative’’ responses, in that the patient must be engaged in a language task and stimulation of language cortex will disrupt task performance. Thus, for language mapping, stimulation produces a discrete, reversible lesion, theoretically enabling the examiner to observe the functional consequences of damage to the site(s) stimulated. Stimulation of frontal language cortex typically produces speech arrest, whereas stimulation of posterior (temporal/parietal) language cortex typically elicits comprehension, naming or reading difficulties. Because cortical language mapping

is based on negative responses, thorough mapping of language cortex requires administration of tasks assessing a range of language functions. Depending on the location of the area identified and the nature of the response elicited by stimulation, it is generally held that removal of a positive sensory, motor, or language site identified by stimulation will result in impaired function postoperatively. Of the few clinical series published, results suggest that postoperative function is best preserved when the resection margin exceeds 1 cm from the functional site. On the other hand, there is some evidence that certain sites identified by stimulation can be removed without concern of postoperative decline. These include motor sites identified in the supplementary motor area, tongue, and lower face areas (due to their bilateral representation), and possibly, language sites identified in the basal temporal region, although this is somewhat controversial. For motor and sensory mapping, the duration of electrical stimulation is typically 2 s, whereas language mapping typically requires 4–8 s of stimulation, depending on the particular task under assessment. The level of stimulation administered ranges from 1 mA to a maximum of 17 mA, with motor and sensory cortex typically utilizing 10 mA. Under normal circumstances, cortical stimulation causes neither pain nor discomfort. One risk of stimulation, however, is the evocation of a seizure, particularly in patients with epilepsy, due to a likely lower seizure threshold in epileptogenic areas. To minimize the probability of a stimulation induced seizure, EEG is monitored on an ongoing

Cortical Mapping. Figure 1 Implanted subdural electrode grid used for EEG recording and electrical stimulation mapping

Cortical Motor Pathways

basis, and the stimulation intensity is lowered when abnormal discharges are associated with stimulation. Nevertheless, benzodiazepines are typically kept close at hand for IV administration for instances when a seizure occurs and fails to resolve rapidly on its own.

Historical Background Alteration of function via cortical stimulation in both animals and humans dates back to the mid 1800s. The procedure came into clinical use in the early twentieth century in association with surgical resection of epileptogenic cortex in patients with pharmacologically refractory epilepsy. Initially used to identify sensory and motor cortex, Wilder Penfield and colleagues pioneered the technique in the 1950s for use in the identification of language cortex. Stimulation-based language mapping was further refined by George Ojemann and colleagues who essentially established the current clinical standards. In addition to its clinical utility, investigators have used the opportunity provided by clinical stimulation to investigate structurefunction relations. These studies build upon the lesion model, contributing more precise information regarding functional localization due to both the controlled setting, and the smaller, more discrete ‘‘lesions’’ induced temporarily by stimulation than that typically found with naturally occurring lesions.

Psychometric Data Cortical mapping procedures remain unstandardized. Due to its highly invasive nature, data from cortical mapping are based on clinical rather than normal populations, and therefore, classic psychometric data are unavailable. It has also been difficult to assess reliability, as cortical mapping is rarely performed more than once in the same patient. Nevertheless, patients with indwelling subdural grids who undergo mapping over 2 or more days, and patients who require a second surgery involving adjacent brain regions provide opportunities for repeat mapping, although these circumstances are relatively infrequent. In the absence of published studies addressing this issue, anecdotal reports suggest a reasonable level of consistency in the location of simulationidentified sites within individuals. Across individuals, consistency is relatively high for the location of motor and sensory cortex. Frontal language cortex is slightly more variable, with most positive sites clustered in the frontal opercular region, anterior to the tongue area. The

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location of posterior temporal and parietal language sites appears to vary more among individuals, although this might merely reflect alterations in the distribution of language sites in patients with epileptogenic cortex in the temporal region.

Clinical Uses One of the main challenges with brain surgery involving cortical regions is to remove a sufficient amount of pathological tissue without removing areas critical for function. Cortical mapping is used to identify cortical regions critical for function, in order to spare these areas from resection or protect them from damage during surgical procedures. Cortical mapping is typically performed when there is concern that resective brain surgery (e.g., tumor resection, epilepsy surgery) might impinge upon, or possibly overlap with cortical regions necessary for function.

Cross References ▶ Epilepsy ▶ Functional Imaging ▶ Localization

References and Readings Hamberger, M. (2007). Cortical language mapping in epilepsy: A critical review. Neuropsychology Review, 4, 477–489. Ojemann, G. A. (1983). Electrical stimulation and the neurobiology of language. The Behavioral and Brain Sciences, 2, 221–230.

Cortical Motor Pathways C HRISTINA R. M ARMAROU Virginia Commonwealth University Richmond, VA, USA

Synonyms Corticospinal tract; Pyramidal tract; Voluntary motor tract

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Cortical Motor Pathways

Definition The cortical motor pathway consists of four regions of the cerebral cortex (primary motor cortex, posterior parietal cortex, premotor cortex, and supplementary motor cortex) whose neuronal cell bodies are located in layer V (five) and whose projections are involved with the execution of muscle contraction largely on the contralateral side of the body.

Current Knowledge The cortical motor pathway describes a trajectory of fibers whose cells of origin are located in layer V of the cerebral motor cortex. The cerebral motor cortex is a term that describes the four main areas of the cerebral cortex that contribute to the planning, control, and execution of voluntary motor fibers; the primary motor cortex (M1), secondary motor cortices, premotor cortex, and the supplementary motor area (SMA).

pyramidal decussation, ultimately terminating in the ventral horn of the cervical-through-lumbar spinal cord. These cortical motor pathways work in tandem with other cortical motor areas of the brain – notably the cerebellum and subcortical motor nuclei (the basal ganglia) – to execute planning and control of voluntary motor activity. Other projections from layer V of M-I include the corticostriatal fibers to the striatum (caudate and putamen), corticorubral fibers to the red nucleus, and projections that terminate in the reticular formations with the medulla and pons of the brain stem. M-I projections also modulate other motor areas within the cortex and include reciprocal connections with the supplementary motor area, the premotor and posterior parietal motor areas. These fibers are part of a vast reciprocal network that cross via the corpus callosum and are referred to as callosal connections. The SMA contributes to the corticospinal tract and has reciprocal callosal projections to contralateral areas of the motor cortex. The premotor cortex (PMC) contributes to the corticospinal tract and also has extensive reciprocal connections to both SMA and MI.

Cortical Location and Overall Function

Cross References The primary motor cortex (M1) is located in the frontal lobe of the brain and the cells of origin, in layer V, are found within the precentral gyrus. These cells generate neural impulses that control the execution of movement directed to skeletal muscles on the contralateral side of the body. Other regions of the cortex that contribute to the cortical motor pathway – termed secondary motor cortices – include the posterior parietal area (PMA), the premotor cortex (PMC) and the supplementary motor area (SMA). The posterior parietal cortex is responsible for transforming visual information into motor commands relayed via the premotor and supplementary motor area. The premotor cortex is involved in sensory guidance of movement and control of proximal and trunk muscles of the body. The supplementary motor area is involved in the planning and coordination of complex movements, such as those requiring coordination of two-handed movement. Multiple pathways arise from the efferent projections of the motor cortices. Neuronal cell bodies located in layer V of the M1, SMA, and premotor cortex send vast projections that collectively give rise to the largest single pathway, the pyramidal or corticospinal tract. The tract descends through the internal capsule and forms the pyramids of the medulla, crossing midline at the

▶ Cerebral Cortex ▶ Decerebrate Posturing ▶ Decorticate Posturing ▶ Hemiplegia ▶ Homunculus ▶ Internal Capsule ▶ Periventricular White Matter ▶ Precentral Gyrus ▶ Pyramidal System ▶ Sensorimotor Assessment ▶ Supplementary Motor Area

References and Readings Berne, R. M., & Levy, M. N. (2000). Principles of physiology. St. Louis, MO: Mosby. Fix, J. (1995). Neuroanatomy. Baltimore, MD: Williams & Wilkins. Haines, D. E. (2000). Neuroanatomy. Philadelphia, PA: Lippincott Williams & Wilkins. Haines, D. E. (2004). Neuroanatomy: An atlas of structures, sections, and systems. Philadelphia, PA: Lippincott Williams & Wilkens. Kandel, E. R., Schwartz, J. H., & Jessel, T. M. (1991). Principles of neuroscience. Norwalk, CT: Appleton and Lange.

Cortical–Subcortical Loop

Cortical–Subcortical Loop J ANNA L. H ARRIS University of Kansas Medical Center Kansas, KS, USA

Synonyms Basal ganglia-thalamocortical ganglia loop

circuit;

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inhibition related to social context or emotional subject matter. In this loop, the lateral orbitofrontal cortex (OFC) projects via the ventromedial caudate nucleus to VA and MD thalamic nuclei, which in turn send projections back to lateral OFC. Affective-motivational loop. This loop is also called the ‘‘Limbic loop.’’ This circuit plays a role in emotional and motivational behaviors. Widespread areas of ‘‘limbic cortex’’ including the anterior cingulate gyrus, medial OFC, and portions of the temporal lobe all send projections via the nucleus accumbens to MD thalamus. The circuit is completed by thalamocortical projections from MD to the anterior cingulate and medial OFC.

Definition The cortical–subcortical loop describes a class of distinct, parallel circuits that connect specific regions of cerebral cortex with the basal ganglia and specific thalamic nuclei. The thalamic nuclei complete the loop by projecting back to the same regions of cortex from which the circuits originate.

Current Knowledge Several distinct, anatomically segregated cortical–subcortical loops may be characterized based on the functional role of the cortical regions involved. Motor loop. The motor loop plays a role in the preparation and execution of movement. Primary motor cortex and associated premotor areas project via the putamen to the ventral tier nuclei of the thalamus, which then complete the loop with projections back to motor cortex. Somatotopic organization is maintained through all stages of this circuit. Oculomotor loop. The oculomotor loop is involved in the control of eye movements. The frontal eye fields (FEF) and supplementary eye fields (SEF) project via the body of the caudate nucleus to the ventral anterior (VA) and mediodorsal (MD) thalamic nuclei, which complete the loop by projecting back to FEF and SEF. Prefrontal associative loops. The prefrontal associative loops describe two distinct components, which subserve different aspects of higher cognitive processing. The ‘‘dorsolateral prefrontal loop’’ plays a role in cognitive processes including spatial memory and working memory. In this circuit, the dorsolateral prefrontal cortex (PFC) projects via the dorsolateral head of the caudate to VA and MD thalamic nuclei, which then project back to dorsolateral PFC. The ‘‘lateral orbitofrontal loop’’ plays a role in cognitive processes including the ability to select and shift behavioral sets, and response

Clinical Disorders and Treatment Approaches An understanding of the architecture of cortical–subcortical loops has given rise to a prevailing view of numerous clinical disorders as essentially circuit disorders, arising from abnormal neuronal activity at some stage of the finely tuned circuit. The best-studied examples involve the motor loop, where disturbances within the circuit can result in either hypokinetic movement disorders (e.g., Parkinson’s disease) or hyperkinetic disorders (e.g., chorea, ballismus, and dystonia). In addition, abnormal activity within the non-motor loops may be associated with disorders as diverse as obsessive–compulsive disorder, schizophrenia, and Tourette syndrome. Recently, interventions that surgically remove or modify (e.g., with deep brain stimulation) the dysfunctional component of the cortical–subcortical loop have met with considerable success. These promising treatment approaches are the subject of intensive ongoing research.

Cross References ▶ Basal Ganglia ▶ Deep Brain Stimulator (Parkinsons) ▶ Parkinson’s Disease

References and Readings Alexander, G. E., Crutcher, M. D., & DeLong, M. R. (1990). Basal gangliathalamocortical circuits: Parallel substrates for motor, oculomotor, ‘‘prefrontal’’ and ‘‘limbic’’ functions. Progress in Brain Research, 85, 119–146. DeLong, M. R., & Wichmann, T. (2007). Circuits and circuit disorders of the basal ganglia. Archives of Neurology, 64(1), 20–24.

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Corticobasal Degeneration

Corticobasal Degeneration A LEXANDER I. T RO¨ STER University of North Carolina Chapel Hill, NC, USA

Synonyms Corticobasal syndrome; Corticodentatonigral degeneration with neuronal achromasia

Short Description or Definition First described as corticodentatonigral degeneration with neuronal achromasia by Rebeiz et al. in 1968, corticobasal degeneration (CBD) was long thought to be predominantly a motor disorder. Indeed, the original description of the disorder emphasized the relative preservation of mental faculties. More recently, emphasis has been placed on the neurobehavioral features of CBD, and the overlapping clinical and neuropathological features of CBD with frontotemporal lobar degenerations continue to generate debate about the classification and nosology of the disorder. The motor presentation of CBD most often involves an asymmetric, progressive, akinetic-rigid parkinsonism of gradual onset that responds minimally if at all to levodopa and is sometimes accompanied by dystonia or myoclonus. Cortical signs that are common in CBD include asymmetric apraxia, cortical sensory signs (e.g., astereognosis, graphesthesia), and alien hand sign. The latter may involve a sense of lack of ownership of the limb in the absence of visual cues, involuntary purposeful movements, or frank interference of one limb with the other’s execution of purposeful movement. These motor and cortical signs are core features of CBD.

Categorization The neurobehavioral expression of CBD can be quite variable, and cases with confirmed CBD neuropathology have presented with features suggestive of primary progressive aphasia and frontotemporal dementia. Coupled with the recognition of CBD as a tauopathy, the occasional neurobehavioral resemblance of CBD to frontotemporal dementia and primary progressive aphasia has lead some to argue that CBD is a member of the ‘‘Pick

complex’’ of disorders (Kertesz, 2003). Given the clinically heterogeneous presentation of CBD, and the fact that the core features of CBD can be produced by other conditions, it has been recommended that the term corticobasal syndrome (CBS) be applied to the core clinical features of CBD regardless of etiology. In contrast, the term corticobasal degeneration (CBD) should be reserved for the distinctive neuropathological condition of CBD, regardless of its clinical presentation (Lang, 2003). From a neuropathologic standpoint, CBD, like frontotemporal lobar degeneration, has been categorized as a tauopathy. Tau is a microtubule-associated protein that promotes the polymerization of tubulin and thus, microtubule assembly. The human tau gene, containing 16 exons, is located on the long arm of chromosome 17 and encodes for the six isoforms of tau found in the central nervous system. The isoforms differ by the presence or absence of amino acid inserts encoded by exons 2, 3, and 10. Whether the transcript of exon 10 is spliced in or out of the final tau protein product determines whether the isoform has three or four repeated microtubule-binding domains (three isoforms have three repeats and three isoforms have four repeats). The four repeat isoforms of tau (4R tau) promote microtubule assembly at more than twice the rate of the three repeat (3R tau) isoforms. Although the expression of 3R and 4R tau is cell-type specific, the 3R tau expression in normal human brain is 1–1.5-fold higher than the 4R expression level. In spontaneous and genetic CBD, 4R tau represents the main pathological inclusion. Recent findings that mutations associated with parkinsonism (in LRRK2) and frontotemporal lobar degeneration (in progranulin) can be seen in some cases presenting with corticobasal syndrome further highlight the heterogeneity of corticobasal syndrome (CBS). Autopsy in CBD cases reveals asymmetric frontal and parietal atrophy, depigmentation of the substantia nigra without Lewy bodies, and often the presence of ballooned cells in cortex. Tau-positive astrocytic plaques, oligodendroglial coiled bodies, and threadlike lesions are seen in white and gray matter, especially the superior frontal and parietal gyri and the pre- and post-central gyri, and in the striatum.

Epidemiology Prevalence and incidence of CBD are unknown, and poor diagnostic accuracy no doubt contributes to this. Although the H1/H1 tau haplotype has been identified as heightening susceptibility to both CBD and progressive

Corticobasal Degeneration

supranuclear palsy, no clear genetic etiology has been identified. Dementia and other cognitive and behavioral abnormalities were thought to be rare in CBD until the last decade, but it is now appreciated that the frequency of neurobehavioral abnormalities observed as a presenting problem or during the course of the condition is quite high. It might be that the inconsistent incidence and prevalence estimates of cognitive impairment in CBD are a function of whether patients were drawn from movement disorder, dementia, or psychiatry clinics.

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retrieval deficits. Remote memory has been little studied in CBD, but the pattern of poor recall but intact recognition on remote memory tasks suggests a retrieval deficit. Visuospatial impairments have been observed. With respect to emotional and neuropsychiatric issues, depression is common in CBD (73% of patients), though apathy (40%), irritability (20%), and agitation (20%) also occur with frequency.

Evaluation Natural History, Prognostic Factors, Outcomes Disease onset is usually in the sixth decade of life, and mean time to death from diagnosis is about 7 years.

Neuropsychology and Psychology of Corticobasal Degeneration CBD involves an asymmetric apraxia, most often of the ideomotor type, but ideational and limb kinetic apraxias also occur. Thus, patients most often have difficulty demonstrating the use of tools. Poor drawing (constructional apraxia) is also commonly seen. Language disturbance occurs early or during progression of the syndrome, and the aphasia is most often non-fluent (about 56% of cases) or anomic (30%). The pattern of performance on language tests in patients with the traditional CBD presentation is somewhat inconsistent across studies, but phonological impairments may be an important feature. Performance on verbal fluency tests, especially lexical or phonemic fluency tests, is usually impaired either due to the executive demands of those tasks or aphasia. Performance on semantic memory tasks such as conceptual matching and visual confrontation naming and expressive vocabulary is relatively preserved and impaired in a minority of patients, although some studies have reported considerable impairment on semantic tasks early in the disease. When naming is impaired, disproportionate benefit is derived from cuing suggesting a retrieval rather than semantic memory deficit. Comprehension is typically preserved early, but comprehension of grammatically complex material declines with disease progression. Executive dysfunction, as indicated by poor performance on tasks such as the card sorting tasks and the Trailmaking test is common. Episodic memory impairments in CBD are relatively mild early in the course of the condition and appear to involve both encoding and

The selection of specific neuropsychological tests in CBD, like any other condition, should be guided by the referral questions and the patient’s ability to cooperate and meet task demands. However, tests of executive function (e.g., planning, abstraction, and cognitive flexibility), praxis, visuospatial functions, attention, learning and memory, and word retrieval should be employed. Symptom inventories relating to apathy, depression, and ‘‘frontal’’ behavior syndromes, such as the Neuropsychiatric Inventory, Hamilton depression scale, and Frontal Systems Behavior Scale can be helpful in characterizing neuropsychiatric features of CBD.

Treatment Some cases may show transient improvement in parkinsonian features with levodopa treatment; dopamine agonists are generally even less helpful than levodopa. Tremor, if present, may be alleviated by benzodiazepines. Antidepressants with anticholinergic profiles are to be avoided given possible adverse cognitive side effects, but selective serotonin reuptake inhibitors may be helpful in treating depression. Speech therapy is helpful in treating dysphagia.

Cross References ▶ Basal Ganglia ▶ Corticobasal Degeneration ▶ Frontal Lobes ▶ Frontal Temporal Dementia ▶ Frontotemporal Lobar Degeneration ▶ Gait Disorders ▶ Movement Disorders ▶ Parkinson Plus Syndromes ▶ Parkinson’s Disease ▶ Parkinsonian Movement

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Cortico-Basal Ganglia Loop

▶ Striatum ▶ Tauopathy

References and Readings Belfor, N., Amici, S., Boxer, A. L., Kramer, J. H., Gorno-Tempini, M. L., Rosen, H. J., et al. (2006). Clinical and neuropsychological features of corticobasal degeneration. Mechanisms of Ageing and Development, 127, 203–207. Boeve, B. F. (2007). Parkinson-related dementias. Neurologic Clinics, 25, 761–781. Geda, Y. E., Boeve, B. F., Negash, S., Graff-Radford, N. R., Knopman, D. S., Parisi, J. E., et al. (2007). Neuropsychiatric features in 36 pathologically confirmed cases of corticobasal degeneration. Journal of Neuropsychiatry and Clinical Neurosciences, 19, 77–80. Graham, N. L., Bak, T. H., & Hodges, J. R. (2003). Corticobasal degeneration as a cognitive disorder. Movement Disorders, 18, 1224–1232. Kertesz, A. (2003). Pick complex: An integrative approach to frontotemporal dementia: Primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy. The Neurologist, 9, 311–317. Lang, A. E. (2003). Corticobasal degeneration: Selected developments. Movement Disorders, 18(Suppl. 6), S51–56. Litvan, I., Cummings, J. L., & Mega, M. (1998). Neuropsychiatric features of corticobasal degeneration. Journal of Neurology, Neurosurgery, and Psychiatry, 65, 717–721. Murray, R., Neumann, M., Forman, M. S., Farmer, J., Massimo, L., Rice, A., et al. (2007). Cognitive and motor assessment in autopsyproven corticobasal degeneration. Neurology, 68, 1274–1283. Sha, S., Hou, C., Viskontas, I. V., & Miller, B. L. (2006). Are frontotemporal lobar degeneration, progressive supranuclear palsy and corticobasal degeneration distinct diseases? Nature Clinical Practice Neurology, 2, 658–665. Tro¨ster, A. I., & Fields, J. A. (2008). Parkinson’s disease, progressive supranuclear palsy, corticobasal degeneration, and related disorders of the frontostriatal system. In J. E. Morgan & J. H. Ricker (Eds.), Textbook of clinical neuropsychology (pp. 536–577). New York: Psychology Press.

Corticoliberin ▶ Corticotropin-Releasing Hormone

Corticospinal Tract ▶ Cortical Motor Pathways

Corticotropin-Releasing Factor (CRF) ▶ Corticotropin-Releasing Hormone

Corticotropin-Releasing Hormone B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms

Cortico-Basal Ganglia Loop Corticoliberin; Corticotropin-releasing factor (CRF) ▶ Cortical–Subcortical Loop

Corticobasal Syndrome ▶ Corticobasal Degeneration

Corticodentatonigral Degeneration with Neuronal Achromasia ▶ Corticobasal Degeneration

Definition Corticotropin-releasing hormone (CRH) is a hormone that is primarily produced by the hypothalamus and is involved in the stress response. It is released from the paraventricular nucleus of the hypothalamus with the primary action within the anterior lobe of the pituitary to initiate the release of adrenocorticotropic hormone (ACTH). CRH (41 amino acids long) is derived from a 191 amino acid preprohormone. Other areas of CRH synthesis include peripheral tissues, and it is highly expressed in the placenta.

CPT

Cross References ▶ Hormone

Cortisteroids

▶ Contraindication

C Couples Therapy ▶ Cognitive Behavioral Couples Therapy

COWA ▶ Controlled Oral Word Association Test ▶ F-A-S Test ▶ Verbal Fluency

COWAT ▶ Controlled Oral Word Association Test ▶ F-A-S Test ▶ Verbal Fluency

▶ Steroids

CPM Coumadin®

▶ Raven Matrices

▶ Warfarin (Coumadin)

Counseling ▶ Psychotherapy

Counseling Relationship ▶ Therapist–Patient Relationship

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Counterindicant

References and Readings Bennett, M. (2008). Stress and anxiety in schizophrenia and depression: Glucocorticoids, corticotropin-releasing hormone and synapse regression. Australian & New Zealand Journal of Psychiatry, 42(12), 995–1002. Fabricio, A., Tringali, G., Pozzoli, G., & Navarra, P. (2005). Mirtazapine acutely inhibits basal and Kþ-stimulated release of corticotropinreleasing hormone from the rat hypothalamus via a non-genomic mechanism. Psychopharmacology, 178(1), 78–82. Guendelman, S., Lang Kosa, J., Pearl, M., Graham, S., & Kharrazi, M. (2008). Exploring the relationship of second-trimester corticotropin releasing hormone, chronic stress and preterm delivery. Journal of Maternal-Fetal & Neonatal Medicine, 21(11), 788–795. Watts, A., Kelly, A., & Sanchez-Watts, G. (1995). Neuropeptides and thirst: The temporal response of corticotropin-releasing hormone and neurotensin/neuromedin N gene expression in rat limbic forebrain neurons to drinking hypertonic saline. Behavioral Neuroscience, 109(6), 1146–1157. Weber, M., & Richardson, R. (2001). Centrally administered corticotropin-releasing hormone and peripheral injections of strychnine hydrochloride potentiate the acoustic startle response in preweanling rats. Behavioral Neuroscience, 115(6), 1273–1282.

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CPRS ▶ Conners Rating Scales

CPT ▶ Continuous Performance Tests

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Craig Handicap Assessment and Reporting Technique

CHART; CHART-SF

The CHART was designed to be administered by interview, either in person or by telephone and takes approximately 15 min to administer. Participant-proxy agreement across disability groups on the CHART has provided evidence in support of the use of proxy data for persons with various types of disabilities (Cusick, Brooks, & Whiteneck, 2001). There is no set time period for administering the CHART; however, it is recommended that multiple measurements be taken over the course of a person’s lifetime to assess changes with adaptation to the disability and to gain insight into changes in participation, which may occur over time.

Description


The Craig Handicap Assessment and Reporting Technique (CHART) is a 32-item instrument designed to provide a simple, objective measure of the degree to which impairments and disabilities result in handicaps (societal participation limitations) for adolescents and adults (15 years and older) in the years after initial rehabilitation. The CHART includes six subscales (physical independence, cognitive independence, mobility, occupation, social integration, and economic independence), which closely reflect the disablement model developed by the World Health Organization (WHO, 1980, 2001). Each subscale contains 3–7 questions, which together quantify the extent to which individuals fulfill various social roles. CHART focuses on objective, observable criteria that are easily quantifiable and unlikely to be open to subjective interpretation. Each of the domains or subscales of the CHART has a maximum score of 100 points, which is considered to be the level of performance typical of the average nondisabled person. High subscale scores indicate less handicap or higher social and community participation. The CHART was developed in 1992 for use with persons with spinal cord injury (SCI) and originally did not address the WHO handicap dimension described as ‘‘orientation’’ (Whiteneck, Charlifue, Gerhart, Overhosler, & Richardson, 1992). The current CHART was revised in 1995 with the addition of a ‘‘Cognitive Independence’’ subscale (to assess orientation) and has proven to be an appropriate measure of societal participation that can be used with individuals having a range of physical or cognitive impairments (Mellick, Walker, Brooks, & Whiteneck, 1999). A 19-item Short Form with subscales closely approximating the subscale scores for the CHART long form is recommended for those applications or populations in which time is at a minimum (Whiteneck et al., 1998).

WHO describes a conceptual model of disablement that includes impairment at the organ level, disability describing functional status, and ‘‘handicap,’’ or more recently, ‘‘participation,’’ encompassing the roles one plays in the world and society. Despite its importance as a rehabilitation goal, handicap (absence of social participation) is the least often measured of all rehabilitation outcomes. A great deal of work has been done in developing tools to measure and document impairment and disability; however, limited attempts have focused on the measurement and assessment of long-term participation limitations (handicap), despite the fact that psychosocial adjustment is clearly regarded as the ultimate outcome of rehabilitation. The CHART was specifically developed to help fill this gap – to assess the WHO dimensions of handicap and to provide a simple, objective measure of the degree to which impairments and disabilities result in participation limitations in the years after initial rehabilitation. The model of disablement suggested by the WHO provides useful conceptual distinctions for impairment, disability, and handicap (WHO, 1980). In practical terms, impairment occurs at the organ level, representing any loss or abnormality of psychological, physiological, or anatomical structure or function. Disability occurs at the person level, demonstrated as any restriction or lack of ability (resulting from impairment) to perform any activity in the manner or within the range considered normal for a human being. Handicap occurs at the societal level. It is a disadvantage for a given individual, resulting from an impairment or a disability that limits or prevents the fulfillment of a role that is normal (depending on age, sex, and social and cultural factors) for that individual. The initial disablement model, the ‘‘International Classification of Impairment, Disability and Health (ICIDH)’’

Craig Handicap Assessment and Reporting Technique G ALE G. W HITENECK Craig Hospital Englewood, CO, USA

Synonyms

Craig Handicap Assessment and Reporting Technique

(WHO, 1980), was later revised as the ‘‘International Classification of Functioning, Disability and Health (ICF)’’ (WHO, 2001). The domain of ‘‘handicap’’ was reconceptualized and changed to ‘‘participation.’’ The migration away from the use of ‘‘handicap’’ toward the more widespread use of ‘‘participation’’ is evident in literature published since 2001. According to the WHO, handicap (participation) describes the total effects and interplay of all the consequences of disability: social, economic, cultural, and environmental. Each CHART dimension of handicap is characterized by directly observable qualities which lend themselves to easy quantification. While an infinite number of factors might have been included, to keep the instrument to a practical length the following dimensions have been operationalized based on the WHO definitions. Physical Independence is the individual’s ability to sustain a customarily effective independent existence. The major component of this subscale is the number of hours per day someone is needed to provide routine or occasional assistance (whether paid or unpaid). Individuals are viewed as somewhat less handicapped if they take primary responsibility for instructing and directing people who are providing assistance to them. Cognitive Independence is the individual’s ability to sustain a customary level of independence without the need for supervision. The factors included in this subscale reflect the amount of hours that a person needs supervision both inside and outside the home, as well as the amount of difficulty an individual has in remembering, communicating, and managing money. Mobility is the individual’s ability to move about effectively in his/her surroundings and is demonstrated by the hours per day out of bed, days per week out of the house, nights per year spent away from home, accessibility of the home, and transportation utilization. Occupation is the individual’s ability to occupy time in the manner customary to that person’s sex, age, and culture. The time spent in various activities is used to measure this dimension. The relative value society places on different activities is used to weight the time in each category. Although there was a potential for subjective bias based on value judgments in developing the scale in this dimension, priority has been give to gainful employment, schooling, and active homemaking and maintenance, and this prioritization has been supported by validity and reliability testing. Other elements documented include volunteer work, recreational pursuits, and self-improvement activities. Social Integration is the individual’s ability to participate in and maintain customary social relationships. The

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factors included in this subscale include household composition, romantic involvement; the number of relatives, business associates, and friends with whom regular written or oral contract is maintained, and the frequency of initiating conversations with strangers. Economic Self-Sufficiency is the individual’s ability to sustain customary socioeconomic activity and independence. This dimension is defined as the remaining disposable household family income after nonreimbursed medical expenses have been excluded.

Psychometric Data Initial calibration of the CHART scoring system was based on administration of the instrument to 88 able-bodied individuals and 100 persons with SCI. Once the norms had been established, two studies were conducted to assess the psychometric properties. CHART showed high test–retest reliability – 0.93 for the total score and from 0.80 to 0.95 for the subscales. The correlation of subject‐ proxy scores was 0.83 for the total CHART score. Rasch analysis established that CHART is a well-calibrated linear scale, with a good fit of both items and persons to its data (Whiteneck et al., 1992).These studies established the CHART as a reliable and valid instrument, as well as a well-calibrated linear scale (Whiteneck et al.; Dijkers, 1991). A decade later, subsequent testing on a group of 236 persons with SCI reported similar results – test–retest correlations of 0.87 and subject‐proxy correlations of 0.85 were reported (Whiteneck, Brooks, & Mellick, 1997). The Revised CHART which included the ‘‘Cognitive Independence’’ subscale was tested on 1,110 persons in six impairment categories – SCI, traumatic brain injury, stroke, multiple sclerosis, amputation, and burn (Whiteneck , Brooks et al., 1997). Results indicated that the cognitive subscale of the CHART was reliable and enhanced the appropriateness of the CHART in assessing handicap among persons having cognitive impairments (Mellick et al., 1999). Participant-proxy agreement across the six disability groups provided evidence in support of the inclusion of proxy data for persons with various types of disabilities (Cusick et al., 2001) In an effort to reduce the number of items in the CHART, a short form was developed. A multidimensional analysis was performed which showed that fewer variables were needed to obtain CHART scores. Regression analyses were performed on each subscale with the dependent measure being the scale score and the variables contributing to the subscale acting as the predictor variables. All CHART subscale scores could be reduced by fewer questions to reach

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Craig Handicap Assessment and Reporting Technique (CHART) Short Form

90% explained variance except Economic Self-Sufficiency, which using the main variables could only explain 45%. For additional information about the development, testing, and scoring procedures for the CHART and CHART-SF please consult the Guide for use of the CHART: Craig Handicap Assessment and Reporting Technique at www.craighospital.org/Research/CHART.

Clinical Uses The CHART is a useful tool to measure handicap (participation limitations) in populations with injury or chronic illness with or without rehabilitation intervention. The CHART is designed as an interview tool, which can be administered face-to-face or by telephone. Each item on the instrument has been carefully and concisely worded to minimize ambiguity of interpretation. It is possible to use the instrument as a mailed questionnaire, although some valuable data potentially would be lost in the absence of interaction with an interviewer providing consistent prompts. There is no set time period for administering the CHART; however, it is recommended that multiple measurements be taken over the course of a person’s lifetime to assess changes with adaptation to the disability and to gain insight into changes in participation which may occur over time. CHART has been demonstrated to be a reliable measure of societal participation limitations in a variety of populations including people with physical and/or cognitive functional limitations.

Cross References ▶ CHART Short Form

Mellick, D., Walker, N., Brooks, C. A., & Whiteneck, G. (1999). Incorporating the cognitive independence domain into CHART. Journal of Rehabilitation Outcome Measures, 3(3), 12–21. Segal, M. E., & Schall, R. R. (1995). Assessing handicap of stroke survivors. A validation study of the Craig handicap assessment and reporting technique. American Journal of Physical Medicine & Rehabilitation, 74, 276–286. Walker, N., Mellick, D., Brooks, C. A., Whiteneck, G. G. (2003). Measuring participation across impairment groups using the Craig handicap assessment and reporting technique. American Journal of Physical Medicine and Rehabilitation, 82(12), 936–941. Whiteneck, G., Brooks, C. A., Charlifue, S., Gerhart, K. A., Mellick, D., Overholser, D., et al. (1998). Guide for use of the CHART: Craig handicap assessment and reporting technique. www.craighospital. org/Research/CHART December 29, 2009. Whiteneck, G. G., Brooks, C. A., & Mellick, D. C. (1997). Handicap assessment – Final report. Rehabilitation research and training center on functional assessment and evaluation of rehabilitation outcomes. Buffalo, NY: State University of New York. Whiteneck, G. G., Charlifue, S. W., Gerhart, K. A., Overhosler, J. D., & Richardson, G. N. (1992). Quantifying handicap: A new measure of long-term rehabilitation outcomes. Archives of Physical Medicine and Rehabilitation, 73, 519–526. Whiteneck, G. G., Fougeyrolles, P., & Gerhart, K. A. (1997). Elaborating the model of disablement. In M. Fuhrer (Ed.), Assessing medical rehabilitation practices: The promise of outcomes research. Baltimore: Paul H. Brooks Publishing Co. World Health Organization. (1980). International classification of impairments, disabilities and handicaps: A manual of classification relating to the consequences of disease. Geneva: World Health Organization. World Health Organization. (2001). International classification of functioning, disability and health. Geneva: World Health Organization.

Craig Handicap Assessment and Reporting Technique (CHART) Short Form ▶ CHART Short Form

References and Readings Cusick, C. P., Brooks, C. A., & Whiteneck, G. G. (2001). The use of proxies in community integration research. Archives of Physical Medicine and Rehabilitation, 82(8), 1018–1024. Manuscript in development. Cusick, C. P., Gerhart, K. A., & Mellick, D. C. (2000). Participant-proxy reliability in traumatic brain injury outcome research. Journal of Head Trauma Rehabilitation, 15(1), 739–749. Dijkers, M. (1991). Scoring CHART: Survey and sensitivity analysis. Journal of the American Paraplegia Society, 14, 85–86. Hall, K. M., Dijkers, M., Whiteneck, G., Brooks, C. A., & Krause, J. S. (1998). The Craig handicap assessment and reporting technique (CHART): Metric properties and scoring. Topics in Spinal Cord Injury Rehabilitation, 4(1), 16–30.

Cramping ▶ Dystonia

Cranial Aerocele ▶ Pneumocephalus

Cranial Nerves

Cranial Nerves M ELISSA J. M C G INN Virginia Commonwealth University School of Medicine Richmond, VA, USA

Definition Cranial nerves serve as conduits for communication between the brain and the body, providing motor and sensory innervation to structures in the head and neck as well as the thoracic and abdominal viscera. There are 12 pairs of cranial nerves, each of which is designated by a Roman numeral and a name (see Table 1). Roman numerals I–XII indicate the rostrocaudal order in which cranial nerves originate from the brain, while the name designated to each pair of cranial nerves denotes either its function or distribution.

Historical Background The enumeration of the cranial nerves can be traced back to second century Greek physician–philosopher Galen; whose initial description included 7 of the 12 currently accepted pairs. English physician and neuroanatomist Thomas Willis’ reclassification of the cranial nerves in the seventeenth century consisted of nine pairs and superseded Galen’s previous description. German anatomist Samuel Thomas von Soemmerring introduced the contemporary classification system, comprising 12 pairs of cranial nerves, in the late eighteenth century.

Current Knowledge Cranial Nerve Nuclei. The majority of cranial nerves (CNs) originate from collections of neurons (nuclei) located within the brainstem (exceptions to this rule are CNs I and II, which are associated with the forebrain and diencephalon, respectively). CNs III and IV emerge from the midbrain portion of the brainstem, CNs V–VIII arise from the pons, and the remaining cranial nerves (CNs IX–XII) originate in the medulla. Taken together, the cranial nerves convey both motor and sensory innervation. However, individual cranial nerves may transmit sensory information only (CNs I, II, and VIII), motor innervation only (CNs III, IV, VI, XI, and XII), or they

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may be considered mixed nerves, meaning that they carry a combination of motor and sensory fibers (CNs V, VII, IX, and X). As a general rule, the brainstem nuclei associated with sensory cranial nerve fibers are located more laterally in the brainstem, while the motor nuclei are positioned more medially. The sulcus limitans is the landmark that demarcates the boundary between these efferent (motor) and afferent (sensory) nuclear zones in the brainstem, which are typically arranged in a longitudinal columnar manner according to functional components (see Functional Components below). Motor neurons that comprise these brainstem nuclei send axonal projections, via cranial nerves, to control glandular tissue secretions and the contraction of various types of muscle (skeletal, smooth, and cardiac muscle). Conversely, the sensory cranial nerve fibers typically originate from sensory neurons located in sensory ganglia (collections of neurons residing outside of the brain) and function to transmit sensation from various types of sensory receptors (i.e., pain and temperature receptors on the skin) to the brainstem. Functional Components. In addition to the generalized classification of cranial nerves as sensory, motor, or mixed nerves, the fibers that comprise each CN can be further categorized according to the specific nature of the afferent or efferent information being transmitted and the types of structures innervated. These fiber classifications, which are often referred to as functional components include somatic motor, visceral motor, branchial motor, somatic sensory, visceral sensory, and special sensory fibers. A cranial nerve can carry one or several of these functional components. Motor Cranial Nerves. Three of the six functional classifications of cranial nerve fibers convey motor innervation: somatic motor, branchial motor, and visceral motor fibers. Somatic motor fibers originate in motor nuclei located in the medial-most cell column of the brainstem and function to transmit motor impulses to voluntary skeletal muscle (of developmental somatic myotome origin) in the head and neck. CNs III, IV, and VI carry somatic motor fibers that innervate the extraocular muscles, CNXI provides somatic motor innervation to two muscles located within the neck/shoulder region and CN XII conveys somatic motor information to the intrinsic muscles of the tongue. Branchial motor fibers are similar to somatic motor fibers in that they provide motor innervation to voluntary striated muscles located within the head and neck region. However, branchial motor fibers and the muscles that they innervate are afforded a separate classification based on their embryologic derivation from branchial/

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Cranial Nerves

Cranial Nerves. Table 1 List of cranial nerves Number Name

Functional componenta

Function

Deficits/symptoms of dysfunction

I

Special sensory

Olfaction (smell)

Hyposmia and anosmia

Olfactory N.

II

Optic N.

Special sensory

Vision

Anopsia

III

Oculomotor N.

Somatic motor

Extraocular muscles (superior, inferior, and medial rectus muscles)

Diplopia and ptosis

Visceral motor

Ciliary and pupillary constrictor muscles

Pupil dilation

IV

Trochlear N.

Somatic motor

Extraocular muscle (superior oblique muscle) Diplopia

V

Trigeminal N.

Somatic sensory

Face, mouth/jaw, teeth, sinuses, meninges, oral and nasal mucosa

Trigeminal neuralgia

Branchial motor

Muscles of mastication

Asymmetric chewing

VI

Abducens N.

Somatic motor

Extraocular muscle (lateral rectus muscle)

Diplopia and medial deviation of eye

VII

Facial N.

Loss of taste

Special sensory

Taste (anterior 2/3 of tongue)

Somatic sensory

Skin on ear and tympanic membrane

Branchial motor

Muscles of facial expression

Visceral motor

All salivary glands (excluding the parotid gland) and lacrimal glands

VIII

Vestibulocochlear N. Special sensory

IX

Glossopharyngeal N. Special sensory

X

Vagus N.

Facial paralysis and Bell’s palsy

Audition and balance

Deafness, tinnitus, and vertigo

Taste (posterior 1/3 of tongue)

Loss of taste

Somatic sensory

Skin on ear, tympanic membrane, posterior 1/3 of tongue, and tonsillar fossa

Branchial motor

Stylopharyngeus muscle

Visceral motor

Parotid gland

Visceral sensory

Carotid body and carotid sinus

Blood pressure regulation deficits Loss of taste

Special sensory

Taste (epiglottis)

General sensory

Skin on external ear

Branchial motor

Muscles of the larynx and pharynx

Visceral motor

Glands, smooth and cardiac muscle in the neck, thorax, and abdomen

Visceral sensory

Pharynx, larynx, and thoracic and abdominal viscera

Dysphagia and dysphonia

XI

Spinal Accessory N.

Somatic motor

Trapezius and sternocleidomastoid muscles

Shoulder drop and weakened neck rotation

XII

Hypoglossal N.

Somatic motor

Muscles of the tongue

Tongue deviation

a

Alternative names for the functional components include following: General somatic efferent (GSE), somatic motor; general visceral efferent (GVE), visceral motor; special visceral efferent (SVE), branchial motor; general somatic afferent (GSA), somatic sensory; general visceral afferent (GVA), visceral sensory; special somatic afferent (SSA) and special visceral afferent (SVA), special sensory.

pharyngeal arches and the fact that branchial motor nuclei are located in distinct brainstem columns (immediately adjacent and lateral to the somatic motor nuclei). Muscles of branchial origin include the muscles of facial

expression (innervated by CN VII), the pharyngeal and laryngeal muscles (innervated by CNs IX, X and the cranial portion of CN XI), and the muscles of mastication (innervated by CN V).

Cranial Nerves

Visceral motor fibers provide autonomic (parasympathetic) innervation to the head, neck, thoracic, and abdominal viscera, where they control glandular secretions and smooth and cardiac muscle contraction. The motor neurons that regulate parasympathetic visceral motor processes are typically positioned immediately lateral to the branchial motor nuclei column in the brainstem. Visceral motor fibers in CN III transmit parasympathetic innervation to structures in the eye that regulate pupil constriction and lens accommodation. CN VII regulates the secretion of tears (via the lacrimal glands) and salivary gland secretions (along with CN IX). CN X conveys visceral motor innervation to glandular tissue, smooth and cardiac muscles of the gastrointestinal, pulmonary, and cardiovascular systems. CN X has the most extensive distribution of the cranial nerves, with its innervation spanning structures within the head and neck down to the thoracic and abdominal regions. Sensory Cranial Nerves. The remaining three functional classifications of cranial nerve fibers convey sensory information and include somatic sensory, visceral sensory, and special sensory fibers. As previously mentioned, the brainstem nuclei associated with these sensory fibers are located more laterally in the brainstem relative to motor nuclei and are arranged in longitudinal columns according to functional components (from lateral to medial: special sensory, somatic sensory, and visceral sensory nuclei). Somatic sensory fibers carry information from exteroreceptors and proprioceptors in the skin, muscles, tendons, and joints of the head and neck, mediating the perception of pain, temperature, touch, and proprioception. CN V is the major somatic sensory nerve of the head, mediating cutaneous and proprioceptive sensation from the skin, muscles, and joints in the face, mouth, and jaw as well as sensory innervation of the teeth, oral and nasal mucosa, sinuses, and meninges. CN IX also transmits somatic sensory information from a portion of the oral mucosa (the posterior third of the tongue and tonsillar fossa) and, together with CNs VII and X, mediates sensation of the skin on the outer ear. In contrast to somatic sensory fibers, visceral sensory fibers receive sensory input from receptive endings in visceral structures, such as walls of blood vessels or of the digestive tract. Congruent with its expansive distribution, CN X mediates the majority of visceral sensation in the pharynx, larynx, thoracic, and abdominal cavities. CN IX transmits visceral sensory information from the carotid sinus and carotid body, important structures in the regulation of blood pressure and respiration. The final category of sensory cranial nerve fibers is the special sensory fibers, which convey sensory information

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relating to olfaction (CN I), vision (CN II), audition (CN VIII), balance (CN VIII), and taste (CNs VII, IX, and X). This special sensory designation is an all-encompassing classification in that it does not distinguish between the somatic senses (vision, audition, and balance) and the more visceral sensations (olfaction and taste). Intracranial Courses. Cranial nerves must traverse through foramina (small holes) within the skull in order to navigate the path between the brain and the various structures to which they provide innervation. Often, cranial nerves will travel through these foramina together in groups as they exit the cranium. For example, CNs IX, X, and XI pass through the jugular foramen on their descent to structures in the neck, thoracic, and abdominal cavities; while CNs III, IV, and VI all traverse the superior orbital fissure to enter into the orbit to innervate the extraocular muscles. Knowledge of the origins of cranial nerves (i.e., brain stem nuclei), their intracranial course and the cranial foramina through which they pass is crucial to any neurological exam, as the diagnosis of dysfunction of specific nerves can help to pinpoint the site of and provide valuable information about damage or injury to the brain. For example, one of the early symptoms of a pituitary adenoma is impaired vision, which results from the close proximity between the pituitary gland and fibers of CN II, which can become compressed as a result of the tumor bulk. Similarly, CN VI has a very long intracranial course and, due to its emergence near the bottom of the brain and its course through the cavernous sinus, it is often the first cranial nerve to be affected in the case of elevated intracranial pressure, common symptoms of which include painful eye movement and blurred or double vision (diplopia). Unlike the majority of cranial nerves, which exit from the ventral surface of the brainstem, CN IV exits the midbrain dorsally and wraps around the lateral surface of the brainstem to enter the orbit. Due to this long peripheral course around the brainstem, CN IV is particularly susceptible to head trauma, where damage to this nerve is manifested by diplopia or blurred vision. Such examples demonstrate the manner in which cranial nerve dysfunction can provide insight into the various pathological situations that can occur in the brain. Cranial Nerve Dysfunction. Cranial nerve dysfunction is not uncommon and can result from a variety of underlying pathologies, ranging from brain trauma to various forms of neurological disease. Common cranial nerve dysfunctions/disorders include Trigeminal Neuralgia (CN V), Bell’s Palsy (CN VII), Ramsay Hunt Syndrome (CN VII), acoustic neuroma (CN VIII), Glossopharyngeal Neuralgia (CN IX), eye movement-related cranial nerve

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palsies (CNs III, IV, and VI), hyposmia/anosmia (CN I), and various anopsias (CN II), among others. Due to its expansive innervation, CN X damage/dysfunction can result in a variety of deficits relating to visceral processes in the heart, lungs, and abdomen. Interestingly, CN X nerve stimulation is an emerging adjunctive treatment for certain types of intractable epilepsy and refractory (treatment-resistant) depression; however, the manner in which CN X stimulation exerts its therapeutic effects is yet to be fully established.

Cranial Pneumocyst ▶ Pneumocephalus

Craniectomy J AMES F. M ALEC Mayo Clinic Indianapolis, IN, USA

Cross References Synonyms ▶ Acoustic Neuroma ▶ Anosmia ▶ Auditory Pathway ▶ Auditory System ▶ Autonomic Nervous System ▶ Bell’s Palsy ▶ Deaf/Hearing Impairment ▶ Diplopia ▶ Dysphagia ▶ Dysphonia ▶ Medulla ▶ Midbrain ▶ Neurologic Examination ▶ Olfaction ▶ Olfactory Bulb ▶ Optic Nerve ▶ Optic Neuropathy ▶ Pons ▶ Ptosis ▶ Pupillary Light Response ▶ Taste ▶ Tinnitus ▶ Trochlear Nerve ▶ Vestibulocochlear Nerve ▶ Visual Field Deficit ▶ Visual System ▶ Vertigo

References and Readings Kandel, E. R., Schwartz, J. H., & Jessell, T. M. (2000). Principles of neural science (4th ed.). New York: McGraw-Hill. Shaw, J. P. (1992). A history of the enumeration of cranial nerves by European and British Anatomists from the time of Galen to 1895, with comments on nomenclature. Clinical Anatomy, 5(6), 466–484.

Decompressive craniectomy

Definition Craniectomy or decompressive craniectomy is a surgical procedure in which a section of the skull is removed and not immediately replaced (Hutchinson, Timofeev, & Kirkpatrick, 2007; Aarabi, Hesdorffer, Ahn, Aresco, Scalea, & Eisenberg, 2006). This procedure is most frequently used when increased intracranial pressure following traumatic brain injury does not respond to other less aggressive interventions. Following brain trauma, the brain may expand within the skull. The resulting increased intracranial pressure can compromise brain function, particularly in the brain stem. Compression of the brain stem can compromise its basic life support functions, that is, cardiac and respiratory regulation, creating a life-threatening situation. By removing part of the skull, the swelling brain is provided room to expand, reducing intracranial pressure and pressure on the brainstem. Although the section of the skull that is removed in a craniectomy is not immediately replaced, the bone removed may be stored and replaced at a later date when brain swelling is reduced and stable. Artificial materials may also be used to replace the removed skull. In a craniotomy, a section of the skull is removed and replaced as part of the initial surgical procedure. Craniotomy is more frequently performed as part of surgical intervention for disorders such as brain tumor or arteriovenous malformation. However, a craniectomy may be preferred in such cases if the condition appears to be associated with brain swelling. Craniectomy may have no advantage over craniotomy in long-term outcome after severe brain injury (Woertgen, Rothoerl, Schebesch, & Albert, 2006).

Cranioplasty

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However, standard craniectomy appears to result in better outcomes than limited craniectomy (Jiang et al., 2005).

Cross References

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▶ Brain Swelling ▶ Craniotomy

References and Readings Aarabi, B., Hesdorffer, D., Ahn, E., Aresco, C., Scalea, T. M., & Eisenberg, H. M. (2006). Outcome following decompressive craniectomy for malignant swelling due to severe head injury. Journal of Neurosurgery, 104, 469–479. Hutchinson, P., Timofeev, I., & Kirkpatrick, P. (2007). Surgery for brain edema. Neurosurgical Focus, 22(5), E14. Jiang, J. Y., Xu, W., Li, W. P., Xu, W. H., Zhang, J., Bao, Y. H., et al. (2005). Efficacy of standard trauma craniectomy for refractory intracranial hypertension with severe traumatic brain injury: A multicenter, prospective, randomized controlled study. Journal of Neurotrauma, 22(6), 623–628. Woertgen, C., Rothoerl, R. D., Schebesch, K. M., & Albert, R. (2006). Comparison of craniotomy and craniectomy in patients with acute subdural haematoma. Journal of Clinical Neuroscience, 13(7), 718–721.

Craniopharyngioma. Figure 1 Courtesy Michael Fisher, MD, Peter C. Phillips, MD. The Children’s Hospital of Philadelphia

References and Readings Fahlbusch, R., Honegger, J., Paulus, W., Huk, W., & Buchfelder, M. (1999). Surgical treatment of craniopharyngiomas: experience with 168 patients. Journal of Neurosurgery, 90, 237–250.

Craniopharyngioma Cranioplasty E THAN M OITRA Drexel University Morgantown, WV, USA

J ACINTA M C E LLIGOTT National Rehabilitation Hospital Dun Laoghaire Co., Dublin, Ireland

Definition Definition Craniopharyngioma is a slow-growing, extra-axial, epithelial-squamous, calcified cystic tumor. It occupies the suprasellar/sellar region and shows benign histology but malignant behavior, as it may invade surrounding areas and recur after the treatment (Fahlbusch, Honegger, Paulus, Huk, & Buchfelder, 1999). Craniopharyngiomas may develop embryogenetically, arising from remnants of the craniopharyngeal duct and/or Rathke cleft or metaplastically because of residual squamous epithelium. The most common presenting symptoms are endocrine dysfunction, headache, and visual disturbances. Craniopharyngiomas are treated with surgery or surgery followed by radiotherapy.

Cranioplasty or replacement of bone flap or prosthesis is a surgical procedure usually performed to fill in, or replace a defect in the skull following a craniectomy or removal of a bone flap (Fig. 1).

Further Reading Cranioplasty can be performed using the patients own bone flap, which has been frozen or stored in the patient’s abdomen, or multiple materials such as titanium mesh, hydoxyapatite, and polymethylmethacrylate can be used

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Craniospinal Irradiation Gardner, W. J. (1945). Closure of defects of the skull with tantalum. Surgery, Gynecology Obstetrics, 80, 303–312. Long, D. F. (2007). Diagnosis and management of late intracranial complications of TBI. In N. D. Zasler, D. L. Katz, & R. D. Zafonte (Eds.), Brain injury medicine. Principles and practice. New York: Demos. Winder, R. J., Cooke, R. S., Gray, J., Fannin, T., & Fegan, T. (1999). Medical rapid prototyping and 3D CT in the manufacture of custom made cranial titanium plates. Journal of Medical Engineering and Technology, 23(1), 26–28.

Craniospinal Irradiation ▶ Craniospinal Radiotherapy Cranioplasty. Figure 1 Source: Winder, Cooke, Gray, Fannin, and Fegan (1999)

as alternative to create a cranioplasty flap, where necessary computerized techniques may be used to generate a custom made cranioplasty (Long, 2007). The decision to perform and the timing of the cranioplasty to replace the skull defect following craniectomy can be variable. Originally considered to be largely a cosmetic rather than a therapeutic procedure, cranioplasty may be now be performed to prevent late neurological complications associated and identified with craniectomy including the so called ‘‘syndrome of trephined’’ (Dujovny, Agner, & Aviles, 1999; Long, 2007). Characteristics of this syndrome include headache, dizziness, irritability, epilepsy, discomfort, and psychiatric symptoms, and in addition, the skin overlying the skull defect may become indented (Dujovny, Aviles, Fernandez, & Charbel, 1997). The pathophysiology of the ‘‘the syndrome of the trephined’’ is unknown, however, a number of factors have been implicated including atmospheric pressure, cerebral blood flow, and cerebrospinal fluid changes (Dujovny et al., 1997). Neurological and functional improvements have been shown to improve following cranioplasty in this syndrome, for example, in 1945 Gardner described the improvement in symptoms in some patients following cranioplasty with tantalum (Dujovny et al., 1997; Gardner, 1945).

References and Readings Dujovny, M., Agner, C., & Aviles, A. (1999). Syndrome of the trephined: Theory and facts. Critical Reviews in Neurosurgery: CR, 9(5), 271–278. Dujovny, M., Aviles, A., Fernandez, P., & Charbel, F. T. (1997). Cranioplasty: Cosmetic or therapeutic? Surgical Neurology, 47, 238–241.

Craniospinal Radiotherapy J ACQUELINE L. C UNNINGHAM Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms Craniospinal irradiation; CSI

Definition Craniospinal radiotherapy is an irradiation that is directed at the whole brain and length of the spinal axis, including the meninges, as part of the cancer treatment to control malignant cells. It serves as a radical (curative) antineoplastic therapy, as a prophylaxis against a neoplasm’s involvement with the central nervous system, or as a palliative recourse when cure is impossible. Craniospinal irradiation (CSI) is technically challenging, and is used with computed tomography (CT) simulation and multimodality MRI registration to define a large target volume, which spares healthy tissues, and assures exact reproducibility of treatment from day-to-day. MRI evidence of the craniospinal radiation injury to the brain has been seen in l’Hermitte’s sign (a side effect of radiotherapy on the spinal cord, experienced as shock sensations), telangiectasia (dilated capillaries), white matter changes, basal ganglia change, necrosis, and cerebral atrophy. Although the differential sensitivity of specific brain regions to radiotherapy has not been determined,

Craniotomy

factors relating to total dose, dose per fraction, and interval between fractions have been identified as important variables influencing the brain’s response to radiation. Present research focuses on the development of treatment protocols based on the efficacy in tumor control while using the least dose of craniospinal radiotherapy (1,800 cGy), often in conjunction with chemotherapy, as the efficacy of CSI dose reduction in ameliorating neuroendocrine and neurocognitive sequelae remains unclear. Today, in contrast to the much higher doses used in the past decades, CSI doses of 2,400 and 3,600 cGy (with daily fractions of 150 or 180 cGy) are standard. Studies continue to evaluate how low a dose will remain effective in a risk-adapted setting. Controversy also exists on the expression of radiation effects on specific neurocognitive domains. Attention and memory are known to bear a vulnerability to neurotoxicity, but issues of individual differences, including premorbid and disease-related risk factors, are expected to influence neuropsychological outcomes.

Cross References ▶ Radiation Injury ▶ Radiation Oncology ▶ Radiotherapy

References and Readings Armstrong, C. L., Gyato, K., Awadalla, A. W., Lustig, R., & Tochner, Z. A. (2004). A critical review of the clinical effects of therapeutic irradiation damage to the brain: The roots of controversy. Neuropsychology Review, 14, 65–86. Brady, L. W., Heilmann, H. P., Molls, M., & Schlegel, W. (2006). New techniques in radiation oncology. New York: Springer.

Craniotomy E DUARDO L OPEZ Johnson Rehabilitation Institute Edison, NJ, USA

Synonyms Craniectomy; Trephination

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Definition Neurosurgical procedure involving the opening of the skull as a means of decreasing intracranial pressure and/or for purposes of removal of a mass lesion.

Current Knowledge Craniotomy as a treatment for increased ICP from a mass lesion has its foundation early in the history of neurosurgery. Decompressive craniotomy (DC) initially was introduced to lower the intracranial pressure (ICP) in patients with inoperable tumors and in managing uncontrolled ICP after traumatic brain injury (Brit & Hamilton, 1978). In recent years, DC has been recommended as an alternative treatment for space occupying acute hemispheric infarction with or without massive medically uncontrolled brain edema (Schwab, 1998). During the acute period following cerebral infarction, neurologic decline is often attributed to surrounding edema. Apart from relieving the mass effect, restoration of the microcirculation around the infarcted area is the target of DC. The management of increased intracranial pressure is a common clinical scenario in neurosurgery. Strategies for the management of ICP fall into two general categories: to reduce the volume of the intracranial compartment (medical management) and to remove the mechanical constraints imposed by the cranial vault (surgical). In patients who sustain a severe non-penetrating head injury, overall 25–45% require a craniotomy for evacuation of a hemorrhagic mass lesion, including epidural, subdural, and intracerebral hematomas (Miller, 1981). There is little debate in the surgical management of a rapidly deteriorating patient with a focal neurological deficit and neuroimaging findings of an expanding intracranial hematoma associated with significant mass effect and midline shift. For less obvious situations controversy remains given the lack of class I and II data to support any treatment standard. Complete removal of a brain tumor without inflicting neurological deficits is a desirable end result in neurosurgical practice. Craniotomy was tailored to encompass tumor plus adjacent areas presumed to contain eloquent cortex. Magnetic brain stimulation or intraoperative cortical stimulation can be used to guide resection of functional cortex. DC remains a controversial procedure in spite of a number of studies published in the literature on its use in the treatment of intracranial hypertension secondary to malignant cerebral edema, traumatic brain injury, aneurysmal subarachnoid hemorrhage, central venous

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thrombosis, encephalitis, intracerebral hematoma, and metabolic encephalopathies. It has been shown to be effective in reduction of ICP refractory to medical therapy but evidence supporting an improvement in clinical outcome has not been conclusive.

conditions are characterized pathologically by neuronal loss, spongiform change, and astrocytic gliosis. Cell loss can be seen microscopically as multiple perforations to the brain tissue creating the characteristic ‘‘sponge-like’’ appearance. Prion diseases are caused by infectious agents, which are abnormal self-replicating forms of a normal brain protein, prion protein.

Cross References ▶ Temporal Lobectomy

References and Readings Brit, R., & Hamilton, R., (1978). Large decompressive craniotomy in the treatment of acute subdural hematoma. Neurosurgery, 2(3), 195–200. Bullock, R., Chestnut, R., Ghajar, J., Gordon, D., Hartl, R., Newell, D. W., et al. (2006). Surgical management of subdural hematoma. Neurosurgery, 58(3), S2–16–S2–24. Chen, C., Smith, E., Ogilvy, C., Carter, B. S., (2006). Decompressive craniectomy: Physiological rationale, clinical indications, and surgical considerations. 5, 70–80.

Cretinism ▶ Hypothyroidism

Categorization and Epidemiology Creutzfeldt–Jakob Disease (CJD) may be sporadic (that is develop spontaneously without apparent cause), familial (inherited), or acquired (transmitted by infection). CJD occurs worldwide with a mean annual incidence of approximately 1–2 cases per million population (Ladogana et al., 2005). Except for variant and iatrogenic CJDs, which are in decline, the disease has a relatively stable incidence with no convincing evidence of geographical clustering (although there are regions with an increased incidence of familial cases). The gender incidence is equal.

Natural History, Prognostic Factors, Outcomes Historical Background

Creutzfeldt-Jakob Disease K ARI H AWKINS 1,2 , R. G. W ILL 1, N ARINDER K APUR 2 1 University of Edinburgh Edinburgh, UK 2 Addenbrooke’s Hospital Cambridge, UK

Synonyms CJD; Prion disease; Transmissible spongiform encephalopathy (TSE)

Short Description or Definition Creutzfeldt–Jakob Disease (CJD) is a rare, fatal neurodegenerative disease, which is one of the transmissible spongiform encephalopathies or prion diseases. These

Sporadic and familial CJDs have been recognized as prion diseases for many years, showing wide geographical spread; however, variant CJD has been confined largely to the United Kingdom, with the first cases reported in 1996. It is thought that meat products intended for human consumption contained contaminated brain and spinal cord from animals affected by the epidemic of a prion disease in cattle (Bovine Spongiform Encephalopathy) in the 1980s (Hilton, 2006). Initial fears of a catastrophic epidemic of vCJD among humans have been partially quelled, with a peak in new cases during 2000 reducing steadily to only one new case in 2007 (http://www.cjd.ed.ac.uk). However, all vCJD cases to date have shared a common genotype (Methionine/Methionine) raising the possibility of subsequent peaks, which occur due to lengthened incubation times among other genotypic groups. In addition, evidence from Kuru, an acquired prion disease arising from cannibalistic funeral practices among the Fore people of Papua New Guinea carried out until 1950s, suggests that incubation times for acquired prion disease may stretch to several decades (Collinge et al., 2006). It is

Creutzfeldt-Jakob Disease

also possible for a person-to-person spread to occur, as has been seen in four cases of blood transfusion-associated vCJD infection (most recent incidence figures are available from http://www.cjd.ed.ac.uk).

Natural History and Prognosis Different CJD phenotypes develop and progress at different rates, although all subtypes develop profound dementia and multiple neurological features progressing to loss of awareness and death. A summary of clinical presenting features is given in Table 1. sCJD presents with cumulative multifocal neurological deficits in association with a rapidly progressive dementia. The cardinal clinical signs are dementia and myoclonus, with a significant proportion of cases exhibiting ataxia and paratonic rigidity of the limbs. The mean survival from onset to death is only 4 months, although patients in the younger age groups often survive for more than a year. sCJD affects predominantly the older age groups with the mean age being 65 years at death. The clinical presentation in familial CJD is often similar to sCJD, but the age of onset is about 10 years earlier, and in some forms, there may be early ataxia and/or slow progression. Iatrogenic CJD may present as in sCJD, but human pituitary hormone recipients typically develop progressive ataxia and cognitive impairment develops late, if at all.

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Variant CJD presents with a psychiatric syndrome, including depression and anxiety, for about 6 months before there is a progressive neurological and cognitive decline as in sCJD, although chorea and dystonia occur as well as myoclonus. The mean survival is 14 months, and vCJD affects a younger age group, with the mean age being 29 years at death.

Neuropsychology and Psychology of Creutzfeldt–Jakob Disease The Presentation of the CJD Patient The differential diagnosis is often the question at referral, in particular, the distinction between a psychiatric or neurological basis for the presenting symptoms or to distinguish CJD from other neurological conditions. A thorough history, as always, is essential in establishing both the profile of the presenting symptoms and the course of the illness, and care should be taken to obtain corroborative reporting from relatives given the difficulties of accurate history taking in individuals with cognitive decline. Sporadic CJD can often be distinguished from other disorders by the speed and degree of cognitive decline, the short duration of illness, and the associated neurological signs. Some cases of fCJD present very similarly to sCJD; however, in fCJD, there is often a younger age of onset, a longer disease duration,

Creutzfeldt-Jakob Disease. Table 1 Forms, causes, and incidence of CJD Form

Phenotype

Cause

Incidence

Sporadic Sporadic Creutzfeldt– Jakob Disease (sCJD)

Unknown.

Approximately one case per million population. Accounts for around 85% of CJD cases.

Familial

Inheritance of mutation in the PrP gene.

Approximately 10–15% of CJD cases are familial.

Case-to-case transmission via contaminated neurosurgical instruments, human dura mater grafts, or exposure to human pituitary hormones. The variant form of CJD can also be transmitted via blood transfusion.

Less than 1% of CJD cases arise through acquired infection.

Ingestion of contaminated meat products from cattle infected with Bovine Spongiform Encephalopathy.

166 cases in total in the UK, 23 cases in France, and a total of 18 cases elsewhere in the world (as of May 2008).

Familial Creutzfeldt– Jakob Disease (fCJD)

Acquired Iatrogenic

Variant CJD (vCJD)

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Creutzfeldt-Jakob Disease. Table 2 Clinical presentation of CJD

Form

Age at onset

Approximate duration of illness

Sporadic

90% of cases between 50 and 80 years, mean 65 years

4 months (65% of cases survive 12 months Heidenhain form: very focal visual difficulties for weeks/months before other cognitive symptoms.

Look for: Rapid cognitive decline Brownell– Oppenheimer over multiple test sessions. form: pure Fluctuations in responsiveness cerebellar and distractibility. syndrome for Intrusion errors. several weeks or Verbal and motor perseveration. months before cognitive decline Familial

Dependent on the specific mutation of the prion protein gene, but most often between the ages of 30 and 50 years.

Dependent on the specific mutation, but on average 2–5 years

Dependent on mutation, may present in a similar fashion to sCJD or with predominant sleep and autonomic disturbance or cerebellar ataxia (see Exceptions, right). Deterioration is usually slower than in vCJD or sCJD with a longer disease course.

fCJD cases may demonstrate less severe/rapid cognitive decline at the early stages of a longer disease course than sCJD or vCJD cases. A single case in 2000 showed specific isolated deficits in delayed verbal memory and word finding prior to global involvement. One study suggests that naming ability may be preserved in some cases in comparison to sCJD and vCJD. Look for: Family history of CJD or other (possibly misclassified) neurological disease

Some cases can have an illness duration of years. Fatal Familial Insomnia: early sleep disturbance and autonomic dysfunction is prominent Gerstmann Straussler syndrome: progressive cerebellar ataxia


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Creutzfeldt-Jakob Disease. Table 2 (Continued)

Form

Age at onset

Iatrogenic Dependent on age at exposure. Incubation period following intracerebral exposure is 19–46 months, extending to many years or decades with peripheral exposure. Variant

Approximate duration of illness Following dura mater graft similar to sCJD, With human pituitary hormones 12–18 months

Median age 28 Median 14 (range 12–74) months

Early clinical features

Neuropsychological findings

Human growth hormone infection: progressive cerebellar syndrome, delayed onset of dementia Human dura mater infection: rapidly progressive dementia, similar to sCJD

Dependent on mode of transmission; human growth hormone patients present with ataxia with relatively preserved cognitive function until later stages, while intracerebral cases present with broad-ranging dementia with rapid deterioration as seen in sCJD. Look for: Rapid cognitive decline over multiple test sessions for intracerebral exposure cases. History of relevant exposure to differentiate from sCJD.

Most commonly, initial presentation is psychiatric disturbance including depression, agitation, and behavioral changes, although in some cases cognitive changes may be the first sign of abnormality. A delay of months is possible before distinct neurological signs, although cognitive changes may be found earlier in the disease course. Sensory symptoms such as pain or odd sensation in limbs or face may be reported. Ataxia, myoclonus, and significant cognitive impairment (such as memory) develop as the disease progresses

Measurable impairments on tests of both verbal and nonverbal memory, executive function, speed of attention, and nominal skills have characterized descriptions of published cases. Language, verbal reasoning, and visuoperceptual skills may be less frequently impaired, although this may reflect an earlier disease stage at the time of testing. One study suggests possible preserved ability in some components of visuoperception in comparison to patients suffering from sCJD or fCJD. Global involvement follows with rapid disease progression. Look for: Fluctuating attention and effort during testing. Cognitive impairment more profound than expected for depression or including areas of deficit unusual for psychiatric disorders. Significant cognitive decline over follow-up assessment sessions.

Sources of information for this table are referenced under ‘‘References and Readings’’.

Exceptions

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Creutzfeldt-Jakob Disease. Table 3 Investigations used in the diagnosis of CJD Phenotype MRI

EEG

CSF 14–3–3

Tonsil biopsy

Sporadic (sCJD)

Important in excluding other conditions. High signal on FLAIR or DWI sequences in the caudate and putamen in about 70% of cases

In 60–80% of cases, generalized bi- or triphasic periodic sharp wave complexes at 1/s. May not appear until later stages of the disease

A positive 14-3-3 Negative CSF immunoassay is strongly supportive of a diagnosis of sCJD in the appropriate clinical context i.e., rapidly progressive dementia. Positive in 90% of cases of sCJD

Familial (fCJD)

Some cases similar to sCJD

Characteristic Positive less periodic pattern is frequently than less frequently sCJD seen than in sCJD.

Blood test

History

At codon 129 of the prion protein gene: 70% methionine homozygous

Negative

Analysis of the prion protein gene for mutations

Positive family history of CJD in about 30% of cases

Iatrogenic Similar to sporadic Characteristic Positive in a CJD periodic pattern is proportion of less frequently cases seen than in sCJD.

Negative

Mainly homozygous methionine or valine

Relevant exposure risk such as treatment with cadaveric derived human growth hormone or human dura mater graft

Variant (vCJD)

As vCJD, unlike the other CJD phenotypes, involves the lymphoreticular system, abnormal protein may be found in the biopsy of tonsil tissue in about 90% of cases.

All tested cases methionine homozygous

Characteristic high signal in the posterior thalamic region (the ‘‘pulvinar sign’’) in over 90% of cases on FLAIR or DWI sequences.

Normal or nonspecifically abnormal. Characteristic periodic pattern in two cases late in clinical course.

Positive in 50% of cases, but does not distinguish from sCJD

Adapted from The National Creutzfeldt–Jakob Disease Surveillance Unit website (http://www.cjd.ed.ac.uk/investigations.htm).

and a family history of either CJD or another neurological disorder. In iatrogenic cases, there should be a clear history of a relevant exposure. The history in vCJD cases includes early psychiatric symptoms (including mood, delusion, and agitation) and may also reveal cognitive decline in everyday activities, of the type that might typically be attributed to depression. In some cases, neuropsychological symptoms may precede psychiatric or neurological indicators that develop as the disease progresses. The early presenting features in all CJD subtypes are summarized in Table 2.

Neuropsychological Testing Patients with CJD often present at too late a stage for formal testing with a full neuropsychological battery. This is reflected in the literature, in which reports focus on small cohorts and case studies. Efforts should be made to obtain sufficient breadth across cognitive domains when testing to aid diagnosis and enable repeat testing if appropriate. Observations of test behavior will also be helpful. A study is currently underway to establish whether a brief bedside screening test might be sufficient to give an


indication of whether the degree or pattern of impairment seen in a patient could indicate CJD.

Evaluation Neuropsychological testing may occur prior to, in parallel with, or following medical investigations and may provide support for a diagnosis of CJD or prompt the clinician to instigate more extensive investigation than would usually be undertaken in patients presenting, for example, with primarily psychiatric complaints as in vCJD. A number of further investigations may be used in the diagnosis of CJD (Table 3).

Treatment CJD is fatal. There is currently no effective treatment for the disease itself, despite ongoing trials of Quinacrine, Pentosan Polysulfate, and Flupirtine (Stewart, Rydzewska, Keogh, & Knight, 2008; see the MRC New Therapies Scrutiny Group for Prion Disease website for up-to-date information concerning recent treatment studies). Medical management should focus on alleviating discomfort, including the use of medication to manage myoclonic jerks or pain and, as the disease progresses, the management of issues such as feeding or continence. Intervention may be needed for the management of mood or psychotic symptoms. Since a high level of care will inevitably become necessary, planning for the provision of this should begin early, in consultation with the family. In the case of a diagnosis of familial CJD, the family will face difficult decisions regarding genetic screening and should be guided through such a process by an appropriately qualified professional. It is often the case that the cognitive symptoms of CJD show extremely rapid deterioration and, in view of this, cognitive rehabilitative efforts are unlikely to produce helpful returns. However, in cases with early referral or a longer disease course, supportive aids (such as a calendar for orientation) or environmental adaptations may produce improvements in activities of daily living, self-efficacy, and mood, at least in the early days, as is the case in other dementias (Clare, 2007; Smith-Bathgate, 2005). While the patients themselves are likely to lose awareness of their predicament as their cognitive ability declines, their families observe a devastating deterioration in their loved ones. There is a role for the clinical psychologist, nurse practitioner, or other qualified healthcare

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professionals in supporting individuals and their families through a period of acceptance, adjustment, loss, and ultimately grief. Provision might also be made for the counselling of healthcare staff involved in these distressing cases.

Cross References ▶ Prion Disease ▶ Spongiform Encephalopathy

References and Readings Clare, L. (2007). Neuropsychological rehabilitation and people with dementia. Hove, England: Psychology Press. Collinge, J., Whitfield, J., McKintosh, E., Beck, J., Mead, S., Thomas, D. J., et al. (2006). Kuru in the 21st century – an acquired human prion disease with very long incubation periods. Lancet, 367(9528), 2068–2074. Cordery, R. J., Alner, K., Cipolotti, L., Ron, M., Kennedy, A., Collinge, J., et al. (2005). The neuropsychology of variant CJD: a comparative study with inherited and sporadic forms of prion disease. Journal of Neurology, Neurosurgery and Psychiatry, 76, 330–336. Creutzfeldt-Jakob Disease Foundation website. http://www.cjdfoundation. org Gass, C. S., Luis, C. A., Meyers, T. L., & Kuljis, R. O. (2000). Familial Creutzfeldt-Jakob disease: a neuropsychological case study. Archives of Clinical Neuropsychology, 15(2), 165–175. Hilton, D. A. (2006). Pathogenesis and prevalence of variant CreutzfeldtJakob disease. Journal of Pathology, 208(2), 134–141. Kapur, N., Abbott, P., Lowman, A., & Will, R. G. (2003). The neuropsychological profile associated with variant Creutzfeldt-Jakob disease. Brain, 126, 2693–2702. Ladogana, A., Puopolo, M., Croes, E. A., Budka, H., Jarius, C., Collins, S., et al. (2005). Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology, 64, 1586–1591. Medical Research Council New Therapies Scrutiny Group for Prion Disease website http://www.mrc.ac.uk/PolicyGuidance/PolicyDevelopment/NewTherapiesScrutinyGroupforPrionDisease. National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) website. http://www.cjd.ed.ac.uk National Prion Clinic website. http://www.nationalprionclinic.org Smith-Bathgate, B. (2005). Creutzfeldt-Jakob disease: diagnosis and nursing care issues. Nursing Times, 101, 52–53. Snowden, J. S., Mann, D. M. A., & Neary, D. (2002). Distinct neuropsychological characteristics in Creutzfeldt-Jakob disease. Journal of Neurology, Neurosurgery and Psychiatry, 73, 686–694. Spencer, M. D., Knight, R. S. G., & Will, R. G. (2002). First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features. British Medical Journal, 324, 1479–1482. Stewart, L. A., Rydzewska, L. H. M., Keogh, G. F., & Knight, R. S. G. (2008). Systematic review of therapeutic interventions in human prion disease. Neurology, 70, 1272–1281.

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Criminal Forensics

Criminal Forensics M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

Denney, R. L., & Sullivan, J. P. (2008). Clinical neuropsychology in the criminal forensic setting. New York: Guilford. Denney, R. L., & Wynkoop, T. F. (2000). Clinical neuropsychology in the criminal forensic setting. Journal of Head Trauma Rehabilitation, 15, 804–828. Mrad, D. (1996). Criminal responsibility evaluations. Paper presented at issues in forensic assessment symposium. Atlanta, GA: Federal Bureau of Prisons.

Definition Within the field of forensic psychology, the utilization of clinical neuropsychological expertise for criminal forensic cases can be considered a subspecialty of the field. Denney and Wynkoop (2000) modified Mrad’s (1996) multiple data source model (MDSM) to the practice of criminal forensic neuropsychology. The purpose of the model is to provide a framework for clinicians to evaluate all relevant sources of information, most notably information relevant to the defendant’s mental state at the time of the offense. The model covers three time points of analysis: present, time of offense, and prior history. Moreover, the model assesses symptoms/behaviors, explanations, etc. via the self-report of the defendant as well as via other sources of data (e.g., neuropsychological tests, mental status exam, medical/neurological exam, arrest reports, witness statements, physical evidence, hospital/psychiatric records, employment records, family/friend reports, etc.). Once all of the relevant pieces of information are gathered, spanning the three time points, it is the role of the forensic neuropsychologist to consolidate the information and formulate opinions. Evaluators involved in criminal forensics typically have very different roles compared to general practitioners. Specifically, in forensic evaluations, the client is typically not the person being examined, and the ultimate goal is to evaluate the facts, not to maintain an alliance with the examinee as is the case in a clinical context. Moreover, forensic criminal evaluations typically involve much more extensive application of corroborative information as well as validated assessments of negative response bias, symptom validity, and malingering.

Cross References ▶ Criminal Litigation

References and Readings Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press.

Criminal Litigation M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

Definition In civil litigation, a lawsuit is filed by a private party, seeking damages from another party as a result of some type of injury, negligence, or malpractice. In criminal litigation, the case is filed by the government against a defendant whom the government believes has committed a crime. Crimes are classified into one of two categories: misdemeanors or felonies. Punishment for misdemeanors involve a maximum possible sentence of less than 1 year of incarceration; felonies carry a maximum possible sentence of more than 1 year of incarceration. The burden of proof in criminal litigation is always assumed by the state. Thus, it is the state’s responsibility to prove that the defendant is guilty of having committed a crime. However, if a defendant claims insanity (e.g., cannot appreciate the wrongfulness of the act nor conform their conduct to the requirements of the law), then the burden of proof in proving one’s insanity falls on the defendant. Under criminal litigation, the state must demonstrate that the accused satisfied each element of the statutory definition of the crime and prove the defendant’s involvement beyond a reasonable doubt. In the context of criminal litigation, forensic neuropsychologists often provide determinations regarding ‘‘mens rea’’ (e.g., guilty mind) or not guilty by reason of insanity (NGRI), competent waiver of Miranda rights, and/or competence to proceed (e.g., stand trial, to be sentenced, etc.).

Cross References ▶ Actus Rea ▶ Criminal Forensics

Crisis Intervention

▶ Insanity ▶ Mens Rea

References and Readings Denney, R. L., & Sullivan, J. P. (2008). Clinical neuropsychology in the criminal forensic setting. New York: Guilford. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney-neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press.

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related issue. Nonetheless, neuropsychologists are increasingly being called upon to determine whether or not brain pathology may contribute to criminal behavior (Barr, 2008).

C Cross References ▶ Actus Rea ▶ Diminished Capacity ▶ Diminished Responsibility ▶ Insanity ▶ Intent ▶ Mens Rea

Criminal Responsibility R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition Criminal responsibility, or the conclusion of guilt for a criminal offense, centers on four elements: (1) The defendant must have committed the act (actus reus). (2) The defendant’s actions must have caused the crime. (3) The defendant must have committed the crime with a guilty state of mind (mens rea). (4) There must be no circumstance constituting a legal defense for the charged crime (e.g., self-defense). In short, there must be the criminal act and the criminal intent and both must be proven, beyond a reasonable doubt. Mental health professionals are typically involved with establishing intent and mens rea, which involves the assessment and professional opinions related to a defendant’s sanity and/or diminished capacity (which includes a decreased level of intent). Criminal responsibility evaluations are also called sanity evaluations or assessment of mental state at the time of the offense evaluations. Determining whether or not a defendant was sane at the time of the offense is one of the most controversial questions forensic examiners are asked to address given that the purpose of the exam is to identify individuals who should not be held morally responsible (‘‘not guilty by reason of insanity’’) for their acts (Yates & Denney, 2008). An insanity plea is pursued in about 9 out of 1,000 cases, and it is successful approximately 25% of the time (Wrightsman, Greene, Nietzel, & Fortune, 2002). It is very rare when a defendant is acquitted secondary to insanity caused by a brain-injury-

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References and Readings Barr, W. B. (2008). Neuropsychological approaches to criminality and violence. In R. Denney, & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford. Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Heilbronner, R. L., & Waller, D. (2008). Neuropsychological consultation in the sentencing phase of capital cases. In R. Denney, & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford. Shapiro, D. L. (1999). Criminal responsibility evaluations: A manual for practice. Sarasota, FL: Professional Resource Press. Wrightsman, L. S., Greene, E., Nietzel, M. T., & Fortune, W. H. (2002). Psychology and the legal system (5th ed.). Belmont, CA: Wadsworth, Thompson Learning. Yates, K. F., & Denney, R. L. (2008). Neuropsychology in the assessment of mental state at the time of the offense. In R. Denney, & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford.

Crisis Intervention E LISE K. H ODGES 1, J EFFREY G. K UENTZEL 2 1 University of Michigan Health System Ann Arbor, MI, USA 2 Wayne State University Detroit, MI, USA

Synonyms Emergency mental health treatment

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Definition Crisis intervention techniques target the resolution of an immediate crisis and restoration of the affected individual’s previous level of functioning. Roberts (2005) defines a crisis as ‘‘a period of psychological disequilibrium, experienced as a result of a hazardous event or situation that constitutes a significant problem that cannot be remedied by using familiar coping strategies.’’

Current Knowledge It is important to stress that most individuals who experience a crisis or traumatic event tend to recover on their own accord (i.e., crisis intervention may not be needed).

Roberts (2005) developed a seven-stage crisis intervention model: 1. 2. 3. 4. 5. 6.

Conduct a biopsychosocial/imminent danger assessment Establish a collaborative relationship Identify the major problems and crisis precipitants Encourage exploration of feelings Generate alternatives and new coping strategies Restore functioning via implementation of an action plan 7. Plan follow-up and booster sessions Hospitals, clinics, and schools typically have in place a set of policies and procedures for how staff should intervene in the crisis situations that are most likely to be encountered. Staff trainings on these protocols should be conducted routinely to ensure a high level of preparedness.

Cross References Crisis Intervention Approaches ▶ Stress Management Critical incident stress management (CISM; Mitchell & Everly, 1993) is an approach to crisis intervention that has garnered considerable attention since its initial development in the 1980s. A key component of CISM is the critical incident stress debriefing (CISD), a group session held shortly after an incident. Led by CISM-trained facilitators, the CISD session allows participants to share their experiences of the incident, and the leaders provide psychoeducation about stress reactions and recommended coping strategies. In the 1990s, a controversy developed surrounding CISM, as research evidence mounted that failed to show its effectiveness. A National Institute of Mental Health (NIMH) report (2002) suggested that stand-alone CISD does not consistently prevent post-traumatic disorders, and that some individuals, such as those with high arousal, may be put at heightened risk for adverse outcomes as a result of CISD-type interventions. Thus, mandatory participation in group debriefing sessions is considered very questionable. An alternative to CISM is psychological first aid (PFA; National Child Traumatic Stress Network and National Center for PTSD, 2006). PFA is an evidenceinformed, pragmatic approach that targets acute stress reactions and the immediate needs of persons exposed to a critical incident (NIMH, 2002). The goals of PFA include enhancement of safety (both objective and subjective), reduction of stress-related symptoms, restoration of rest and sleep, linkage with resources, and facilitation of social support.

References and Readings Mitchell, J. T., & Everly, G. S. (1993). Critical incident stress debriefing: An operations manual for the prevention of traumatic stress among emergency services and disaster workers. Ellicott City, MD: Chevron. National Child Traumatic Stress Network and National Center for PTSD (2006). Psychological first aid: Field operations guide, (2nd ed.). Retrieved March 5, 2010, from http://www.ncptsd.va.gov/ncmain/ ncdocs/manuals/PFA_V2.pdf. National Institute of Mental Health (2002). Mental health and mass violence: Evidence-based early psychological intervention for victims/ survivors of mass violence. A workshop to reach consensus on best practices. NIH Publication No. 02-5138, Washington, D.C.: U.S. Government Printing Office. Roberts, A. R. (2005). Crisis intervention handbook: Assessment, treatment, and research. (3rd. ed.). New York: Oxford University Press.

Criterion Validity ▶ Test Validity

Criterion-Referenced Testing ▶ Domain Referenced Test Interpretation

Cross-Examination

Critical Periods ▶ Sensitive Periods

Crossed Aphasia PATRICK C OPPENS SUNY Plattsburgh Plattsburgh, NY, USA

Definition Crossed aphasia is an acquired language impairment following a lesion in the right hemisphere in a right-handed individual.

Current Knowledge The term ‘‘crossed aphasia’’ (CA) was coined by Byrom Bramwell (1899) to indicate an aphasia caused by a cerebral lesion ipsilateral to the dominant hand regardless of handedness. Currently, CA only refers to right-handed individuals. The frequency of CA among stroke survivors is rare (1–3%). Although some earlier authors considered CA to be the consequence of a weaker language lateralization, it appears that individuals with CA have language as strongly lateralized as those with left-hemisphere aphasia (LHA), mainly because both populations show a similar prognosis. CA can be mirror image or anomalous (Alexander, Fischette, & Fischer, 1989). Mirror-image CA denotes the expected correspondence between symptomatology and lesion location within the language-dominant hemisphere, whereas anomalous CA implies the presence of unexpected language symptoms given the lesion location (e.g., Wernicke’s aphasia following a frontal lesion [Basso, Capitani, Laiacona, & Zanobio, 1985], a mixed transcortical aphasia with preserved naming [Fujii, Yamadori, Fukatsu, Ogawa, & Suzuki, 1997]). Close to two thirds of CA cases are mirror image. The CA language symptomatology is virtually indistinguishable from LHA, and all aphasia types have been reported in CA. The cause of CA is essentially unknown, but the influence of left-handedness in the family has long been considered an important causal factor, a hypothesis called familial

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sinistrality. However, familial sinistrality is absent in the majority (63%) of reported CA cases (Coppens, Hungerford, Yamaguchi, & Yamadori, 2002). Further, there does not seem to be any difference in CA symptomatology when comparing patients with and without familial sinistrality, and the presence of familial sinistrality does not increase the number of anomalous cases, as could be expected if language laterality were weak (Coppens et al., 2002). Individuals with CA also often display typical righthemisphere symptoms (e.g., left-side neglect, visuospatial/visuoconstruction problems, affective dysprosody, and spatial agraphia) which indicates that those skills may lateralize independent of language. The aphasic difficulties may obscure these right-hemisphere signs, but these represent a major difference in symptomatology between CA and LHA.

Cross References ▶ Aphasia ▶ Handedness

References and Readings Alexander, M. P., Fischette, M. R., & Fischer, R. S. (1989). Crossed aphasias can be mirror image or anomalous. Brain, 112, 953–973. Basso, A., Capitani, E., Laiacona, M., & Zanobio, M. E. (1985). Crossed aphasia: One or more syndromes? Cortex, 21, 25–45. Bramwell, B. (1899). On ‘‘crossed’’ aphasia. Lancet, 1473–1479. Coppens, P., Hungerford, S., Yamaguchi, S., & Yamadori, A. (2002). Crossed aphasia: An analysis of the symptoms, their frequency, and a comparison with left-hemisphere aphasia symptomatology. Brain and Language, 83, 425–463. Fujii, T., Yamadori, A., Fukatsu, R., Ogawa, T., & Suzuki, K. (1997). Crossed mixed transcortical aphasia with hypernomia. European Neurology, 37, 193–194.

Cross-Examination M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

Definition A deposition or actual trial testimony consists of two parts: the direct examination and the cross-examination.

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Direct examination precedes the cross-examination and involves testimony brought forth by the retaining attorney. Cross-examination occurs immediately after the direct examination and is carried out by the opposing attorney. The main purpose of cross-examination is to test the ‘‘reliability, accuracy and credibility’’ of witnesses’ testimony produced during the direct examination. Questions posed during cross-examination typically fall into two categories: those intended to expose weaknesses or errors in the expert witnesses’ data acquisition or interpretations, and those related to expose biases in the testimony. During cross-examination, expert witnesses are expected to give responsive answers. That is, they are to provide relevant answers, but the answers need not be those implicitly desired by the opposing attorney. The opposing attorney may use several tactics during cross examination including: challenging credibility, establishing doubt, leading questions, feigned ignorance, the cut-off (e.g., testimony of witness terminated to stop the witness from providing further information that could be detrimental to the opposing attorney’s position), intentional ambiguity, implying impropriety, rattling the witness, and many others.

Cross References

CRS-R ▶ Coma Recovery Scale

Crystallized Intelligence ▶ Intelligence

CSI ▶ Craniospinal Radiotherapy

CS-PFP ▶ Physical Functional Performance

▶ Direct Examination

CS-PFP10 References and Readings Brodsky, S. L. (2004). Coping with cross-examination and other pathways to effective testimony. Washington, DC: American Psychological Association. (1975). Federal rules of evidence for United States courts and magistrates. St. Paul, MN: West Publishing. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press.

CRS ▶ Coma Recovery Scale ▶ Conners Rating Scales

▶ Physical Functional Performance

CT ▶ Category Test ▶ Computed Tomography

CT Scan ▶ Computed Tomography

Cue

Cue J ANET PATTERSON California State University Hayward, CA, USA

Synonyms Discriminative stimulus; Prime; Prompt

Definition A cue is a verbal or nonverbal instruction to induce behavior change. Cues are self-generated or may come from the environment (including an examiner). They

Present Cue # 1

may appear spontaneously as a behavior is unfolding in order to effect immediate change. For example, when one sees the cue of a deer running into the road one veers away to avoid a collision, or when one is engaged in conversation and hears the cue of his or her sentence that does not adequately express the intended meaning one immediately changes the sentence. Cues may also appear as a learned strategy and be repeated in a consistent format. For example, drivers know that when the yellow traffic light appears they should slow the car in preparation to stop when the red traffic light appears, or in a communication interaction when another individual offers a greeting one should respond. Some examples of self-generated, internal cues are reminders for future action (e.g., remember to walk the dog), mental sequences used in the tip-of-the-tongue state (e.g., telling oneself that the

Present Cue # 4

Present a picture and say, “Say the name of this,” or “What’s this” or “Tell me the name of this”

Say “It starts with ___” and say the first sound

If correct response (a) Say “correct” or “right” (b) Ask for five repetitions (c) Move to next picture item

(a) (b)

If correct response Say “correct” or “right” Present Cue #3

(a)

If incorrect response Present Cue #5

(a)


Present Cue # 2

Present Cue # 5 Say “The word is ___” and say the name of the item

Say “It goes with ____” and give the semantic cue If correct response (1) Say “correct” or “right” (2) Present Cue #1

(a) (b)

If incorrect response (a) Present Cue #3

(a) (b)

Present Cue # 3

If correct response Say “correct” or “right” Present Cue #4 If incorrect response Repeat cue and ask for repetition If unable to repeat item name after three consecutive presentations of Cue #5 move to next picture item

Say “It sounds like ___” and give the rhyme cue

(a) (b)

If correct response Say “correct” or “right” Present Cue #2

(a)


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Cue. Figure 1 An example of a cueing hierarchy for oral naming using semantic and phonological cues

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name rhymes with sock), or teaching/treatment strategies (e.g., using an association strategy for naming, such as reciting the alphabet to cue the name). Examples of external cues are environmental signs (e.g., walk/do not walk signals), preprogrammed reminders (e.g., alarm clock), or teaching/treatment techniques (e.g., semantic-phonological cueing hierarchy). A cueing hierarchy is a set of cues progressing from weak cues providing little information about the target response, to strong cues providing much information (Wambaugh, Linebaugh, Doyle, Martinez, Kalinyak-Fliszar, & Spencer, 2001) (Fig. 1).

Cross References ▶ Cue Dominance ▶ Cued Recall ▶ Free Recall ▶ Phonemic Cue ▶ Recency Effect ▶ Semantic Cue ▶ Semantic Fluency ▶ Verbal Fluency

References and Readings Patterson, J. P., & Hinnach, S. E. (2005). Word familiarity effects during naming and discourse production in aphasia. Presentation to the American Speech-Language-Hearing Association Annual Convention. San Diego. Wambaugh, J. L., Linebaugh, C. W., Doyle, P. J., Martinez, A. L., Kalinyak-Fliszar, M., & Spencer, K. A. (2001). Effects of two cueing treatments on lexical retrieval in aphasic speakers with different levels of deficit. Aphasiology, 15(10/11), 933–950.

Cue Dominance R ONALD A. C OHEN Brown University Providence, RI, USA

Synonyms Cue salience; Orienting stimulus; Stimulus strength

Definition Cue dominance refers to the tendency to perceive or respond to a particular stimulus or class of stimuli over others in the environment. Stimuli may either have intrinsic properties that give them strength or ‘‘dominance’’ based on physical attributes (e.g., loudness, color), or may acquire dominance (i.e., propensity to elicit a response) as a function of associative learning and task demands.

Current Knowledge Cue dominance is an important principle derived from behavioral studies of animal conditioning and learning theories. It provides a theoretical foundation for the neuroscience of selective attention, linking basic behavioral and learning processes with higher order information processing. In the context of behavioral conditioning, cues refer to stimuli that have the capacity to elicit an orienting response, anticipation, subsequent attention, and response intention and preparation. Cues lack the inherent biological salience of unconditioned stimuli, and do not elicit unconditioned Pavlovian responses, but can become conditioned stimuli through associative learning. Studies of discrimination learning conducted in the middle of the twentieth century established many of the operational characteristics underlying cue dominance. For example, studies examining intrinsic cue dominance demonstrated that monkeys have a natural preference for response to color over other perceptual dimensions, such as size and shape (Draper, 1965). Experiments with human infants demonstrated cue dominance for certain shapes over others based on whether they resembled the shape of a human face (Fantz & Miranda, 1975). While certain stimulus features have intrinsic cue dominance across many animal species, the ability to form complex cues based on the association of simple cues appears greatest among humans, as is the ability to exhibit reversal learning in order to shift response from one cue to another (Kendler & Ward, 1972; Kendler, 1971). In neuropsychological studies and assessment methods, cues are often used to either facilitate attention to particular spatial locations, semantic information, or response demands. Alternatively, cues are used to create interference to test the effects of distraction or the redirection of attention away from the primary demands of a task. This is a fundamental element of the spatial selective attention paradigms that involve cueing to spatial locations (Posner, Snyder, & Davidson, 1980). Furthermore, the tendency of the semantic value associated with a word

Cued Recall

to be dominant over color and to interfere with attention to and naming of the actual color that is being presented underlies the Stroop effect. Researchers and clinicians studying or assessing attentional processes need to account for both the inherent and acquired cue dominance of stimuli being used in the context of particular tasks.

Cross References ▶ Enhancement ▶ Selective Attention ▶ Visual Discrimination

References and Readings Draper, W. A. (1965). Cue dominance in oddity discriminations by rhesus monkeys. Journal of Comparative and Physiological Psychology, 60(1), 140–141. Fantz, R. L., & Miranda, S. B. (1975). Newborn infant attention to form of contour. Child Development, 46(1), 224–228. Kendler, T. S. (1971). Continuity theory and cue-dominance. In H. H. Kendler & J. T. Spence (Eds.), Essays in neobehaviorism: A memorial volume to Kenneth W. Spence. East Norwalk: Appleton-CenturyCrofts. Kendler, H. H., & Ward, J. W. (1972). Reversal learning: The effects of conceptual and perceptual training in the absence of differential observing responses. Psychonomic Science, 28(6), 346–348. Posner, M. I., Snyder, C. R., & Davidson, B. J. (1980). Attention and the detection of signals. Journal of Experimental Psychology: General, 109(2), 160–174.

Cue Salience ▶ Cue Dominance

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from free recall in that a cue or word is presented that is related to the information being remembered. This aides in the process of memory retrieval. Some examples of cued recalls are the names of the categories in which words were originally grouped or the presentation of related words. For instance, in remembering the word feather, the word bird may be used as a cued recall.

Current Knowledge Tests of Cued Recall There are many tests of cued recall. One of the most commonly used tests of cued recall is the California Verbal Learning Test (CVLT) developed by Delis et al. This semantic test, like many other tests of memory, utilizes both free and cued recall.

Clinical Uses of Cued Recall Testing In addition to the use of free recall tests, tests of cued recall may be used in identifying memory impairments in a wide array of disorders including mild cognitive impairment and Alzheimer’s disease. In some instances, tests of cued recall may be more accurate than free recall tests in detecting cognitive changes. For example, Ivanoiu et al. (2005) found that a cued recall test is more reliable in testing memory impairment in Alzheimer’s disease than free recall testing. While both tests of memory are impaired in such individuals, cued recall may be a more accurate measure of cognitive decline. This is because deficits other than pure memory deficits, such as impaired attention or depression, may account for a decreased performance in free recall. Conversely, cued recall tests may be used to more directly measure the encoding and retrieval that takes place in memory tasks.

Cross References

Cued Recall M ARGARET M OULT Institute of Living Hartford CT, USA

Definition Cued recall is the retrieval of memory with the help of cues. Such cues are often semantic. Cued recall differs

▶ California Verbal Learning Test (California Verbal Learning Test-II) ▶ Cue ▶ Free Recall

References and Readings Carpenter, S. K., Pashler, H., & Vul, E. (2006). What types of learning are enhanced by a cued recall test? Psychonomic Bulletin & Review, 13, 826–830.

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Ivanoiu, A., Adam, S., Van der Linden, M., Salmon, E., Juillerat, A. C., Mulligan, et al. (2005). Memory evaluation with a new cued recall test in patients with mild cognitive impairment and Alzheimer’s disease. Journal of Neurology, 252, 47–55. Vogel, A., Morentsen, L., Gade, A., & Waldemar, G. (2007). The category cued recall tests in very mild Alzheimer’s disease: Discriminative validity and correlation with semantic memory functions. European Journal of Neurology, 14, 102–108.

Cultural Diversity in Neuropsychology S ARAH K. L AGEMAN Emory University Atlanta, GA, USA

Definition The term ‘‘cultural diversity’’ generally refers to the differences in defining cultural features that exist between people (or within a given population), such as language, dress, and traditions, as well as more abstract concepts involving significant variations in societal organization and interaction styles between individuals and environments. With respect to neuropsychology, the term encompasses the racial and ethnic diversity among neuropsychologists themselves and the populations they interact with, as well as issues related to the influences of race and ethnicity on neuropsychological evaluations and treatments.

Current Knowledge Multicultural competency is a developing area of focus within the field of neuropsychology. As the ethnic and racial diversity of the United States increases, so do efforts to provide neuropsychological services to ethnic minorities. Thus, clinical and research studies have begun to examine how cultural variables influence performance on neuropsychological measures. While the past decade has witnessed some publications providing test norms for specific racial and ethnic groups, the use of racial and ethnic norms for neuropsychological measures remains a contentious issue. Readers are encouraged to examine the summary of the 2008 Diversity Summit for additional details (Romero et al., 2009). Highlights of the summit and recent literature are provided below.

Recent studies emphasize that the deconstruction of race is critical for improved clinical care of ethnically diverse populations. The construct has been identified as a ‘‘proxy for more meaningful but complex variables’’ such as acculturation and indicators of quality of education, which account for significant proportions of racial and ethnic differences in neuropsychological test scores (Romero et al., 2009, p. 765). However, increasing emphasis has been placed on the importance of the clinician knowing when to apply race- and ethnic-based norms, as their use can actually reduce detection of neuropsychological impairment. The complexity of this issue was demonstrated in one of the Mayo’s Older African American Normative Studies (MOAANS) project studies. In this study, adjustment of test scores for reading level surprisingly reduced detection of cognitive impairment in African Americans. Lucas and colleagues (2005) suggest that their findings indicate that cohort differences in reading level may be more reflective of individual differences in cognitive ability rather than contextual differences such as educational backgrounds. While race may be a very efficient and parsimonious variable for clinicians to use at times, the confounding of causes and effects of these variables presents a significant challenge. The Diversity Summit participants unanimously agreed that guidelines for neuropsychological practice among ethic and racial minorities are needed to further improve clinical service for ethic and racial minorities. In an effort to initiate the development of practical guidelines regarding the use of demographic corrections, clinical criteria have been developed. As detailed in the Diversity Summit proceedings, demographic corrections are useful to identify and characterize acquired neurocognitive impairments in adults who (1) are natives of the country of assessment, (2) developed normally, (3) received mainstream education, and (4) speak English as their first language (for the U.S. norms). Demographic corrections are sometimes useful to identify and characterize acquired neurocognitive impairments in (1) teenagers or young adults who have not completed their education, (2) adults who may have had a mild developmental disorder, or (3) anyone with a linguistic, cultural, or educational background not well represented in the normative subject sample. Demographic corrections are not recommended when the clinician is asked (1) to characterize ‘‘absolute’’ levels of functioning, (2) to identify or characterize possible acquired impairment in capital punishment cases or when determining qualifications for special services, or (3) in cases of possible acquired impairment in individuals who have developmental disability, (4) to characterize acquired cognitive impairment in individuals who

Cultural Diversity in Neuropsychology

have major background differences from the normative sample, (5) to predict future performance in employment or academic settings, and (6) for employment selection decisions. In addition to these clinical guidelines, emphasis is being increasingly placed on correctly using probabilistic statistics to predict group membership (i.e., diagnosis) for individuals. Readers are encouraged to review recent publications regarding likelihood ratios, which express the risk associated with certain test scores (Smith, Ivnik, & Lucas, 2008). While additional study of the deconstruction of race is needed, development of ethnic group norms itself is a challenging and complex task. Test publishers are increasingly making efforts to include important background variables in normative samples. However, the cost of recruiting and assessing large cohorts is prohibitive for individual centers and researchers and is often even a challenge for larger test publishing companies. While inclusion of demographic variables in research articles is an important piece to further develop this area, federal and foundation-funded grants may be necessary to support multicenter partnerships, ideally suited to study large, diverse cohorts. Despite the development of racially and ethnically diverse norms, including the widely used Heaton norms for African American and Caucasian adults, the almost endless variety possible with regard to individuals’ language, culture, and education backgrounds limits the application of demographically corrected norms for every possible clinical situation (Heaton, Miller, Taylor, & Grant, 2004). Local norms may be helpful for clinicians working with population groups with less common background variables; however, integration of test scores with a person’s individual context is ultimately critical for formulation of an accurate diagnosis. In clinical practice, other issues regarding assessment of ethnic, linguistic, and cultural minorities are also present. The use of translated tests, interpreters, and bilingual psychometrists are all current methods for evaluating non-English-speaking patients. However, the shear complexity of language fluency is important to consider. Language fluency of an individual can vary widely with regard to (1) age of acquisition, (2) proficiency, (3) manner of learning, (4) amount of language exposure, (5) predominant mode of bilingual interaction, and (6) language structure variables (i.e., alphabetic versus logographic languages and phonemic versus nonphonemic status). Currently, the use of translated tests and official interpreters or bilingual psychometrists are preferred over using family members as interpreters and site translation, which refers to the practice of quickly translating a test with no standardization procedures. The

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importance of training in cross-cultural neuropsychology will become increasingly important as the Spanishspeaking Latino population in the United States grows. In keeping with the demographic changes in the United States, training programs and national organizations are brainstorming ways to promote recruitment and retention of students with diverse racial and ethnic backgrounds in the study of neuropsychology. Emphasis has been placed on student exposure to neuropsychology earlier in academic careers, mentoring with minority and nonminority faculty who are sensitive to diversity issues and financial support of minority students. The study of cultural diversity itself has gained support and many licensing bureaus require documentation of diversity training in graduate school for licensure. Numerous organizations, including the American Psychological Association (APA) Division 40, the International Neuropsychological Society (INS), and the National Academy of Neuropsychology (NAN), promote awareness of diversity issues and foster collaborations among clinicians and researchers interested in diversity issues. Readers are encouraged to review current guidelines, including the APA Ethical Principles (2002) and the APA Multicultural Guidelines (2002) for further details. Diversity in neuropsychology has become of increasing interest and importance; however, measures to improve diagnostic assessment of ethnic minorities may not fully address the issue of improving clinical care of ethnic minorities, given limited health care access of non-Englishspeaking patients. Access to care and other factors affecting ethnic minority patient outcomes have increasingly been examined in the field of neuropsychology. Readers are encouraged to review two issues of the Journal of Head Trauma Rehabilitation, dedicated to U.S. and international cultural issues in the rehabilitation of survivors of traumatic head injury (‘‘Cultural Issues,’’ 2007 and ‘‘International Programs and Perspectives,’’ 2007). Articles in these issues highlight complex relationships between functional outcomes, disability severity, return to work, and utilization of professional psychological services in ethnic minority groups. The international issue describes models of care used in the context of various health care systems as well as international perspectives on research and development in the field of traumatic brain injury rehabilitation. The study of health disparities among ethnic minority populations is a complex, but critical field of research. Multiple dimensions of disparity exist, including many of the variables discussed regarding the deconstruction of race such as ethnicity, education, income, occupation, and geography. Continuous study of these variables is critical to improve clinical care for ethnic minorities.

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Cross References ▶ AACN Practice Guidelines ▶ Clinical Practice Guidelines ▶ Cultural Sensitivity ▶ Culture Fair Tests ▶ Ethics in the Practice of Neuropsychology

ethnic minorities: Summit proceedings. The Clinical Neuropsychologist, 23, 761–779. Smith, G. E., Ivnik, R. J., & Lucas, J. (2008). Assessment techniques: Tests, test batteries, norms, and methodological approaches. In J. E. Morgan & J. H. Ricker (Eds.), Textbook of clinical neuropsychology (pp. 38–57). New York: Taylor & Francis. Uzzell, B. P., Pontoń, M., & Ardila, A. (Eds.). (2007). International handbook of cross-cultural neuropsychology. Mahwah, NJ: Lawrence Erlbaum Associates, Inc.

References and Readings American Academy of Clinical Neuropsychology (AACN). Multicultural references. Retrieved March 16, 2010, from the AACN Web site: http://www.theaacn.org/position_papers/AACN_multicultural_ref erences.pdf American Psychological Association’s Ethical Principles of Psychologists and Code of Conduct with the 2010 Amendments (http://www.apa. org/ethics/code/index.aspx) American Psychological Association’s Ethnic Minority Affairs Office (http://www.apa.org/pi/oema/index.aspx) American Psychological Association’s Minority Fellowship Program (http://www.apa.org/pi/mfp/) American Psychological Association’s Division 45, Society for the Psychological Study of Ethnic Minority Issues (http://www.apa.org/ divisions/div45/) American Psychological Association. (2002). Guidelines on multicultural education, training, research, practice, and organizational change for psychologists. Retrieved June 30, 2010, from http://www.apa.org/ practice/guidelines/multicultural.pdf Arango-Lasprilla, J. C., & Niemeier, J. (Eds.). (2007). Cultural issues in the rehabilitation of TBI survivors: Recent research and new frontiers. Journal of Head Trauma Rehabilitation, 22(2), 73–139. Ardila, A., Rosselli, M., & Puente, A. (1994). Neuropsychological evaluation of the Spanish speaker. New York: Plenum Press. Board of Directors (2007). American Academy of Clinical Neuropsychology (AACN) Practice Guidelines for Neuropsychological Assessment and Consultation. The Clinical Neuropsychologist, 21, 209–231. Retrieved March 16, 2010, from http://www.theaacn.org/position_ papers/AACNPractice_Guidelines.pdf Ferraro, F. R. (Ed.). (2002). Minority and cross-cultural aspects of neuropsychological assessment. Lisse, Netherlands: Swets & Zeitlinger. Fletcher-Janzen, E., Strickland, T. L., & Reynolds, C. R. (Eds.). (2000). Handbook of cross-cultural neuropsychology. New York: Kluwer Academic/Plenum Publishers. Heaton, R. K., Miller, S. W., Taylor, M. J., & Grant, I. (2004). Revised comprehensive norms for an expanded Halstead-Reitan battery: demographically adjusted neuropsychological norms for African American and Caucasian adults. Lutz, FL: Psychological Assessment Resources, Inc. International Programs and Perspectives on Traumatic Brain Injury Rehabilitation. (2007). Journal of Head Trauma Rehabilitation, 22(4), 209–256. Lucas, J. A., Ivnik, R., Willis, F., Ferman, T., Smith, G., Parfitt, F., et al. (2005). Mayo’s Older African American Normative Studies: Normative data for commonly used clinical neuropsychological measures. The Clinical Neuropsychologist, 19, 162–183. Nell, V. (1999). Cross-cultural neuropsychological assessment: Theory and practice. Mahwah, NJ: Lawrence Erlbaum Associates, Inc. Romero, H. R., Lageman, S. K., Kamath, V., Irani, F., Sim, A., Suarez, P., et al. (2009). Challenges in the neuropsychological assessment of

Cultural Sensitivity C HRISTIAN S CHUTTE , B RADLEY A XELROD John D. Dingell VA Medical Center Detroit, Michigan, USA

Synonyms Multicultural

Definition Awareness of how a patient’s background, including ethnicity and demographic factors, affects treatment, assessment, and research.

Current Knowledge There are a couple of pertinent definitions, or applications, of cultural sensitivity to the field of neuropsychology. The position of the American Psychological Association (APA), which is reflected in the 2002 APA ethical principles of psychologists and code of conduct, state ‘‘Psychologists are aware of and respect cultural, individual, and role differences, including those based on age, gender, gender identity, race, ethnicity, culture, national origin, religion, sexual orientation, disability, language, and socioeconomic status and consider these factors when working with members of such groups. Psychologists try to eliminate the effect on their work of biases based on those factors, and they do not knowingly participate in or condone activities of others based upon such prejudices.’’ Cultural sensitivity is also reflected in the 2002 APA Guidelines on multicultural education, training, research, practice, and organizational change for psychologists. The APA defines ‘‘multicultural’’ specifically as referring to interactions with ‘‘individuals from minority ethnic and racial groups in the United States and the dominant

Culture Fair Test

European-American culture.’’ This document states that all individuals are in a social, political, and economic context. Therefore, it is increasingly important for psychologists to be aware of the specific needs of individuals based on their ethnic/racial heritage and social group identity. Guidelines relating to research direct psychologists to ‘‘recognize the importance of conducting culture-centered and ethical psychological research among persons from ethnic, linguistic, and racial minority backgrounds.’’ A second area of cultural sensitivity that directly relates to neuropsychology is the impact of culture on testing and on normative data. It is important that an individual examinee’s test performance be related to individuals of similar backgrounds. For example, the normative group of an elderly, well-educated African American man should closely match his background. Without close matching of cultural, demographic, and educational background, unacceptable problems with misclassification of normal test results as abnormal can result in misdiagnosis of the patient. There is evidence that examiners belonging to a culture different from that of the examinee can elicit some anxiety, which may have a deleterious effect on test scores on more demanding cognitive tasks (Nagra, Skeel, & Sbrage, 2007). Additionally, cultural background, demographics and educational history, can have a sizeable impact on normative data. Smith, Ivnik and Jucan (2008) discuss the development of norms specifically for older adults on commonly used neuropsychological tests. They subsequently developed more specific norms for older African Americans, as it was found that culture had a significant impact on normative data. Heaton, Miller, Taylor and Grant (2004) have published norms that take into account age, education, and cultural background. This area of cultural sensitivity plays an important role in neuropsychology in terms of diagnosis and test evaluation.

Cross References ▶ Cultural Diversity ▶ Normative Data

References and Readings American Psychological Association. (2002). Ethical principals of psychologists and code of conduct. American Psychological Association. (2002). Guidelines on multicultural education, training, research, practice and organizational change for psychologists.

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Heaton, R. K., Miller, S. W., Taylor, M. J., & Grant, I. (2004). Revised comprehensive norms for an expanded Halstead-Reitan Battery: demographically adjusted neuropsychological norms for African American and Caucasian Adults. Lutz, FL: Psychological Assessment Resources Inc. Nagra, A., Skeel, R. L., & Sbrage, T. P. (2007). A pilot investigation on the effects of stress on neuropsychological performance in Asian-Indians in the United States. Cultural Diversity & Ethnic Minority Issues, 13, 54–63. Smith, G. E., Ivnik, R. J., & Jucan, J. (2008). Assessment techniques: tests, test batteries, norms and methodological approaches. In J. E. Morgan, & J. H. Ricker (Eds.), Textbook of clinical neuropsychology (pp. 38–57). New York: Taylor & Francis.

Culturally Reduced Test ▶ Culture Fair Test

Culture Fair Test G LEN E. G ETZ Allegheny General Hospital Pittsburgh, PA, USA

Synonyms Culture free test; Culturally reduced test

Definition Culture fair test is a test that is equally fair to all cultural groups. Fairness is related to a lack of bias in the interpretation or use of a test to classify or diagnose. In a culture fair test, the validity of the interpretation is similar across different cultural groups. It is unlikely that any test can entirely eliminate the influence of learning and cultural experience, given that the test content, language, directions, and validity criteria are culturally bound. However, avoiding culturally loaded items, items that are found to be unfair to certain groups of people, increases the likelihood of it being a culturally fair test. Culturally loaded items, such as those that utilize pictures or general information that are differentially prevalent for certain cultures, decrease the likelihood of a culturally fair test.

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Cross References ▶ Cultural Sensitivity ▶ Raven’s Progressive Matrices

References and Readings Jensen, A. R. (1974). How biased are culture-loaded tests? Genetic Psychology Monographs, 90, 185–244. Scarr, S. (1994). Culture fair and culture free test. In R. J. Sternberg (Ed.), Encyclopedia of human intelligence. New York: Macmillan.

Culture Free Test ▶ Culture Fair Test

Cuneate Nucleus ▶ Nucleus Cuneatus

Cuneus R ONALD A. C OHEN Brown University Providence, RI, USA

Synonyms Broadmann area 17

Structure The term cuneus comes from the Latin term for wedge, which reflects the shape of this occipital brain area. The cuneus is a wedge-shaped cortical area located in the medial occipital gyri, superior to the calcarine fissure and posterior to the parietal–occipital fissure. The cuneus

is part of the occipital lobe, corresponding to Broadmann area 17. Pyramidal cells in the cuneus (striate cortex) project to extrastriate cortices (Broadmann areas 18 and 19). The cuneus consists of both striatal and extrastriatal visual cortex consisting of five layers. The striatal areas are largely posterior and are idiotypic homomodal (Mesulam’s classification), while the extrastriatal areas contain heterotypic cell types that respond to more complex visual information. The cuneus receives input from the contralateral superior retina corresponding to the lower visual field. From the extrastrial areas of cuneus, information is processed through both ventral and dorsal pathways. The dorsal pathway is particularly important for higher spatial analysis and visual integration.

Function The cuneus plays as essential role in primary visual processing. The striatal regions that contain primary visual processing areas contain neurons with small receptive fields that are sensitive to very basic visual frequency information related to position, local orientation, spatial-frequency, and color (Beason-Held et al., 1998; Jeannerod, 2004; Ulbert, Karmos, Heit, & Halgren, 2001; Ungerleider & Pribram, 1977). However, more anterior to the primary visual areas are neurons that respond to more complex information contained in the visual information processed by the primary receptors (Ungerleider & Haxby, 1994). Accordingly, the cuneus plays a role in both primary and secondary visual processing. Extrastriatal areas of the cuneus are known to respond to reward, anticipatory, attention, and working memory manipulations, and there is even evidence that posterior striatal areas respond to attentional signals, providing evidence that this cortical plays some role in higher cognitive function involving basic visual information.

Illness Damage to the cuneus would typically occur among patients experiencing focal brain lesions. Most often such lesions occur secondary to cerebral infarction (stroke) typically involving the posterior cerebral circulation, though neurosurgery to remove neoplasm may result in focal damage to the cuneus as well. Some atypical neurodegenerative diseases are also known to cause tissue loss in this occipital area. The effects of focal damage to the cuneus are known primarily from experimental ablation in primate studies and to some extent from single-

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case studies of stroke. Posterior cuneus lesions tend to disrupt primary visual perception, particularly response to stimuli occurring in the lower visual fields. Damage to more anterior areas of the cuneus also affects visual functions (e.g., movement perception), though often for higher level operations involved in object perception and spatial analysis, including attention (De Weerd, Peralta, Desimone, & Ungerleider, 1999). Interestingly, there is also evidence that cuneus volume is related to other behavioral processes not obviously tied in a direct way to visual processing. For example, one recent study found greater cuneus volume to be associated with greater inhibitory control among patients with bipolar disorder affective disorder (Haldane, Cunningham, Androutsos, & Frangou, 2008). Another study found that compulsive gamblers had increased activity in dorsal visual processing pathways, including the cuneus when compared with controls (Crockford, Goodyear, Edwards, Quickfall, & el-Guebaly, 2005). Whether such findings reflect a specific role of the cuneus in these psychiatric disorders versus more incidental findings needs to be determined in future investigations.

Cross References ▶ Feature Detection ▶ Spatial Frequency Analysis

▶ ‘What System’ ▶ ‘Where System’

References and Readings Beason-Held, L. L., Purpura, K. P., Krasuski, J. S., Maisog, J. M., Daly, E. M., Mangot, D. J., et al. (1998). Cortical regions involved in visual texture perception: A fMRI study. Brain Research Cognitive Brain Research, 7(2), 111–118. Crockford, D. N., Goodyear, B., Edwards, J., Quickfall, J., & el-Guebaly, N. (2005). Cue-induced brain activity in pathological gamblers. Biological Psychiatry, 58(10), 787–795. De Weerd, P., Peralta, M. R., III, Desimone, R., & Ungerleider, L. G. (1999). Loss of attentional stimulus selection after extrastriate cortical lesions in macaques. Nature Neuroscience, 2(8), 753–758. Haldane, M., Cunningham, G., Androutsos, C., & Frangou, S. (2008). Structural brain correlates of response inhibition in Bipolar Disorder I. Journal of Psychopharmacology, 22(2), 138–143. Jeannerod, M. (2004). Visual and action cues contribute to the self-other distinction. Nature Neuroscience, 7(5), 422–423. Ulbert, I., Karmos, G., Heit, G., & Halgren, E. (2001). Early discrimination of coherent versus incoherent motion by multiunit and synaptic activity in human putative MT + . Human Brain Mapping, 13(4), 226–238. Ungerleider, L. G., & Haxby, J. V. (1994). ‘‘What’’ and ‘‘where’’ in the human brain. Current Opinion in Neurobiology, 4(2), 157–165. Ungerleider, L. G., & Pribram, K. H. (1977). Inferotemporal versus combined pulvinar-prestriate lesions in the rhesus monkey: Effects on color, object and pattern discrimination. Neuropsychologia, 15(4–5), 481–498.

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Cushing’s Syndrome

Cushing’s Syndrome I SABELLE B OURDEAU 1, H E´ LE` NE F ORGET 2 1 CHUM-Hoˆtel-Dieu Montreál, QC, Canada 2 Universite´ du Que´bec en Outaouais Gatineau, QC, Canada

Short Description or Definition Endogenous Cushing’s syndrome (CS) is a rare disorder occurring in about 0.7–2.4 per million population per year (Newell-Price, Bertagna, Grossman, & Nieman, 2006). CS includes symptoms and signs resulting from chronic supraphysiological exposure to glucocorticoids (GC). The classical clinical features of CS are centripetal obesity with abnormal fat distribution, mainly affecting the face, neck, trunk, and abdomen, and sparing the extremities. Other findings are facial plethora, easy bruisability, purple abdominal striae, hirsutism, muscle weakness, hypertension, and glucose intolerance (Stewart, 2008). In addition, mood alterations and psychiatric diseases – mainly depressive and anxiety disorders – as well as cognitive impairment are highly prevalent in CS patients (Bourdeau et al., 2005). The principal aim of this chapter is to present psychiatric and cognitive data on adult patients with CS. The recognition of psychoneurological abnormalities associated with hypercortisolism is of clinical importance in the management of patients affected by CS.

peripheral circulation and reaches cells in the cortex of the adrenal gland where cortisol biosynthesis is initiated. Cortisol itself exerts negative feedback control on ACTH and CRH secretion, maintaining a normal secretion rate in case of HPA activation, in stress for example (Fig. 1). ACTH is released physiologically in a series of secretory episodes, followed by an equal number of cortisol bursts in plasma. Thus, plasma cortisol levels are maximum in the early morning hours around awakening, gradually decline throughout the morning, and reach nadir values late in the evening. Midnight serum cortisol >200 nmol/L strongly suggests the diagnosis of CS (Stewart, 2008). Variations of ACTH and cortisol values constitute the normal circadian rhythm. CS is caused by pituitary or adrenal abnormalities or may be secondary to ectopic ACTH or CRH secretion by various nonpituitary tumors, such as lung carcinoma. CS is classified into ACTH-dependent and -independent causes which are described in Table 1. Cushing’s disease refers to CS due to excessive pituitary ACTH secretion from pituitary tumors.

Evaluation or Diagnosis The diagnosis of endogenous CS may be challenging and require the expertise of an endocrinologist. Authors will briefly discuss the evaluation and various tests used to diagnose CS. The first step is to confirm CS by biochemical investigation. Then, the cause of the disease should be

Hypothalamus

Etiology Exogenous CS refers to iatrogenic CS resulting from chronic GC therapy. GC, which have potent anti-inflammatory and immunologic actions, are widely used for the treatment of various diseases such as inflammatory bowel disease, asthma, rheumatoid arthritis, and organ transplantation. Thus, exogenous CS is the most common form of CS. Endogenous CS may be caused by dysregulation at various levels of the hypothalamic–pituitary–adrenal axis (HPA), resulting in cortisol overproduction. The regulation of cortisol synthesis and secretion is mediated by the hypothalamus, the pituitary, and the adrenal glands. Corticotropin (ACTH) is synthesized and secreted by corticotrophs of the pituitary which are mainly regulated by two hypothalamic hormones, corticotropin-releasing hormone (CRH), and arginine-vasopressin. Pituitary ACTH is secreted in the

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Cortisol Cushing’s Syndrome. Figure 1 Schematic representation of the hypothalamus–pituitary axis. Normally cortisol negatively regulates corticotropin-releasing hormone (CRH) and ACTH secretion


Cushing’s Syndrome. Table 1 Classification of the etiologies of endogenous Cushing’s syndrome (CS) ACTH-dependent CS (80% of cases) Pituitary adenoma (Cushing’s disease) (80%) Ectopic ACTH or corticotropin-releasing hormone (CRH) secretion from nonpituitary neoplasms (20%) ACTH-independent CS (20% of cases) Adrenal adenoma (60%) Adrenal carcinoma (40%) Bilateral ACTH-independent macronodular adrenal hyperplasia (rare) Bilateral micronodular adrenal hyperplasia (primary pigmented nodular adrenocortical disease and nonpigmented adrenal hyperplasia) (rare)

identified. During the investigation, it is important to evaluate patients for other illnesses, drugs, alcohol, or depression. All these conditions may lead to misinterpretation of the endocrine test results. The overnight 1-mg dexamethasone suppression test, a screening tool for the diagnosis of CS, consists of administering 1 mg of dexamethasone at bedtime (23:00 h) with measurement of plasma cortisol the following morning (08:00–09:00 h). Normal subjects should suppress plasma cortisol to less than 50 nmol/L. Measurement of free cortisol in 24h urine collection is useful to confirm the diagnosis; values fourfold greater than the upper limit of normal are highly suggestive of CS. More recently, late-night salivary cortisol has proven useful in the diagnosis of CS (Nieman et al., 2008). Once a diagnosis of CS is confirmed, the next step is to establish the etiology. Plasma ACTH will distinguish between ACTH-dependent and independent CS. ACTH values lower than 1.1 pmol/L indicate ACTH-independent CS, and CT imaging of the adrenal glands should be performed (Newell-Price et al., 2006). ACTH values above 3.3 pmol/L suggest ACTHdependent CS, and further endocrine investigations should be undertaken, including magnetic resonance imaging of the pituitary glands and other tests that are beyond the scope of this chapter.

Psychiatric Diseases and Neuropsychological Disturbances Associated with CS Understanding the importance and complexity of adrenal hormones impacting cerebral function, particularly affect

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and cognition, has increased considerably in the recent years. McEwen, Weiss, and Schwartz (1968) demonstrated the presence of glucocorticoid receptors (GR) in the rat brain. Loss of brain volume was documented in patients with endogenous and exogenous CS (Bourdeau et al., 2002). In addition, cerebral magnetic resonance spectroscopy revealed abnormalities of cerebral metabolism in patients with CS (Khiat et al., 1999). Psychiatric disturbances occur in patients with both exogenous and endogenous CS. Interestingly, patients with endogenous depression frequently have high cortisol levels, and present HPA axis dysregulation, disclosed by abnormal suppression on the dexamethasone test. However, diurnal rhythm is usually maintained in depressed patients, and they do not develop physical signs of CS. CS is also associated with an increased prevalence of cognitive impairments, particularly of attention, learning, and memory. Patients with CS represent a natural model that illustrates the interactions between hypercortisolism and brain function. The following section focuses on the psychiatric and neuropsychological anomalies in CS.

Psychiatric Disorders Associated with CS Depression is a frequent feature of CS. In fact, several reports indicate that more than 50% of CS patients present severe depressive symptoms that reach the threshold of a major depressive disorder. Haskett (1985) observed a lifetime history of psychiatric symptoms and signs in patients with proven CS. He found that the majority of them experienced psychiatric disturbances that closely resembled typical syndromes, with episodes of major affective disorder (endogenous depression, mania, or hypomania). Moreover, Haskett (1985) noted that there were no significant differences between pituitarydependent and -independent forms of CS in the occurrence of major depression. His observations were later confirmed by independent investigations (Kelly, 1996; Sonino, Fava, Belluardo, Girelli, & Boscaro, 1993). Using DSM-III criteria (American Psychiatric Association, 1980), Hudson, Hudson, Griffing, Melby, and Pope (1987) reported a high lifetime diagnosis of mood disorders in CS patients, of whom 14% were assessed to have major affective disorder. Loosen, Chambliss, DeBold, Shelton, and Orth (1992) recognized major depressive disorder in 68% of adult patients with CS. In addition, this disorder was frequently associated with anxiety disorder (generalized anxiety disorder and/or panic disorder), indicating a syndrome of anxious depression in

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patients with active CS (Loosen et al., 1992). Focusing on Cushing’s disease, Sonino, Fava, Raffi, Boscaro, and Fallo (1998) found major depression in more than half of their patients studied. Moreover, major depression was significantly correlated with older age, female gender, higher pretreatment urinary cortisol levels, and more severe clinical conditions. Kelly, Kelly, and Faragher (1996) reported that some patients with CS were diagnosed to have depression and were more depressed than patients with other pituitary tumors. Concentrating on the question of whether depression in CS more consistently resembles any of the clinical subcategories, Dorn et al. (1995) established that patients with active endogenous CS exhibited significant psychopathology, expressed primarily by ‘‘atypical’’ depression. This concept of atypical depression refers to the presence of weight gain or increased appetite, fatigue, hypersomnia, leaden paralysis, and longstanding interpersonal rejection sensitivity (American Psychiatric Association, 1994).

reported generalized impairment of cognitive functions (decreased concentration and memory, perceptual distortions). More recently, Starkman, Gebarski, Berent, and Schteingart (1992) observed moderate-to-severe deficits in a wide variety of language and nonverbal subtests among more than two-thirds of their CS patients. Difficulties with reasoning ability, comprehension, and processing of new information also were found. Deficits in these areas of cognition were confirmed in a recent controlled study of 19 CS patients (Forget, Lacroix, Somma, & Cohen, 2000). In fact, memory was the most studied function in CS because the emphasis was on the hippocampus which is rich in GR. However, the distribution of GR in many areas of the cerebral cortex suggests that extrahippocampic sites could also be the target of cortisol, and generalized impairment of cognitive functions after chronic hypercortisolism may be related to this wide dispersion of GR.

In summary: 1. More than 50% of patients with CS report severe depressive symptoms 2. There are no significant differences between ACTHdependent and -independent forms of CS in the occurrence of major depression 3. Major depressive disorders are commonly associated with anxiety disorders 4. Major depression is significantly correlated with older age, female gender, higher pretreatment urinary cortisol levels, and more severe clinical conditions 5. A high prevalence of atypical depression features is seen in CS

Treatment The goal of CS treatment is to correct the hypersecretion of adrenal hormones. In most cases, tumor-specific surgery will be performed. In Cushing’s disease, pituitary tumors should be resected, and in adrenal CS adrenalectomy performed. In cases of ectopic ACTH secretion secondary to benign tumors, the latter should be removed; but in most cases, there are metastatic malignant tumors. If surgery is unsuccessful, drugs that block steroid synthesis may be administrated to achieve eucortisolism (Biller et al., 2008; Stewart, 2008).

Neuropsychological Disorders Associated with CS


Neuropsychological disorders have been detected in about two-thirds of patients with CS. The first such investigation evaluated unselected CS patients on the Michigan Neuropsychological Test Battery (Whelan Schteingart, Starkman, & Smith, 1980). This test battery included standardized and objective measures with a broad range of discrete language, verbal and nonverbal reasoning, auditory and visual memory, and sensory and motor functions. The study revealed diffuse bilateral cerebral dysfunction in CS with impairment in nonverbal, visual– ideational, visual-memory, and spatial–constructional abilities. Starkman, Schteingart, and Schork (1981)

The reversibility of brain volume loss after the correction of hypercortisolism in patients affected by CS was previously demonstrated (Bourdeau et al., 2002). Similarly, treatment of hypercortisolism is often efficacious in decreasing the depressive components of illness. Significant improvement in depressive symptoms was observed when patients with CS were assessed by psychometric methods before and after the treatment of their endocrine condition, (Dorn et al., 1997; Forget, Lacroix, & Cohen, 2002; Kelly et al., 1996; Starkman, Giordani, Gebarski, & Schteingart, 2006; Starkman, Schteingart, & Schork, 1986). In a longitudinal study of depressive and


neuropsychological symptoms in CS, Forget et al. (2002) reported a significant improvement in Beck Depression Inventory scores 1 year after the correction of hypercortisolism. More recently, Starkman et al. (2006) showed significant changes in depression and anxiety subscales on SCL-90-R after the successful treatment of hypercortisolism in CS. However, psychological distress does not always disappear upon proper treatment. For example, Dorn et al. (1997) disclosed that the incidence of atypical depression progressively decreased after the correction of hypercortisolism – this disorder, however, continued to be the prevailing psychiatric diagnosis after the successful cure of CS. It is likely that the psychopathology symptoms still seen after the correction of CS may be related to the as yet unexplored interaction between endocrine and psychosocial factors. Indeed, despite the correction of hypercortisolism, patients with CS experience a considerable decrease in quality of life, even after long-term cure (Lindsay, Nansel, Baid, Gumowski, & Nieman, 2006; van Aken et al., 2005). The authors postulated that there might be irreversible changes in central neural function in these patients (Heald et al., 2004). Few studies have objectively examined the reversibility of cognitive disorders after successful treatment. Mauri et al. (1993) demonstrated significant amelioration in verbal memory performance and attentive and visuomotor functions 6 months after surgery. No changes in other cognitive functions could be detected. On the other hand, no differences between Cushing’s patients and their controls were found in cognitive function 12 months post-treatment (Dorn & Cerrone, 2000). Forget et al. (2002) evaluated subjects who presented with CS on a battery of tests, including attention, visuospatial processing, memory, reasoning, and verbal fluency. Except for one task of visual organization, the results showed little change in performance, suggesting that prolonged exposure to high cortisol levels can cause long-lasting deleterious effects on cognitive function. The data indicate that correction of hypercortisolism is not necessarily correlated with short-term improvement in cognitive function. More recently, Hook et al. (2007) postulated that the age of Cushing’s patients appeared to be a significant factor determining when and how cognitive function manifested signs of recovery. In conclusion, the psychiatric and neuropsychological disturbances associated with CS may compromise quality of life and produce a broad array of handicaps and disabilities. Moreover, these functional impairments may persist long after successful treatment and in clinical remission. Thus, such symptoms warrant clinical attention.

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References and Readings van Aken, M. O., Pereira, A. M., Biermasz, N. R., van Thiel, S. W., Hoftijzer, H. C., Smit, J. W. A., et al. (2005). Quality of life in patients after long-term biochemical cure of Cushing’s disease. The Journal of Clinical Endocrinology and Metabolism, 90, 3279–3286. American Psychiatric Association. (1980). Diagnostic and statistical manual of mental disorders (3rd ed.). Washington, DC: American Psychiatric Association. American Psychiatric Association. (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: American Psychiatric Association. Biller, B. M., Grossman, A. B., Stewart, P. M., et al. (2008). Treatment of adrenocorticotropin-dependent Cushing’s syndrome: A consensus statement. The Journal of Clinical Endocrinology and Metabolism, 93, 2454–2462. Bourdeau, I., Bard, C., Forget, H., Boulanger, Y., & Cohen, H., Lacroix, A. (2005). Cognitive function and cerebral assessment in patients with Cushing’s syndrome. In J. W. Findling & H. Raff (Eds.), Cushing’s Syndrome. Endocrinology and Metabolism Clinics of North America, 32(2), 441–458. Bourdeau, I., Bard, C., Noel, B., Leclerc, I., Cordeau, M. P., Be´lair, M., et al. (2002). Loss of brain volume in endogenous Cushing’s syndrome and its reversibility after correction of hypercortisolism. The Journal of Clinical Endocrinology and Metabolism, 87, 1949–1954. Dorn, L. D., Burgess, E. S., Dubbert, B., Simpson, S. E., Friedman, T., Kling, M., et al. (1995). Psychopathology in patients with endogenous Cushing’s syndrome: ‘Atypical’ or melancholic features. Clinical Endocrinology, 43, 433–442. Dorn, L. D., Burgess, E. S., Friedman, T. C., Dubbert, B., Gold, P. W., & Chrousos, G. P. (1997). The longitudinal course of psychopathology in Cushing’s syndrome after correction of hypercortisolism. The Journal of Clinical Endocrinology and Metabolism, 82, 912–919. Dorn, L. D., & Cerrone, P. (2000). Cognitive function in patients with Cushing syndrome. Clinical Nursing Research, 9, 420–440. Forget, H., Lacroix, A., & Cohen, H. (2002). Persistent cognitive impairment following surgical treatment of Cushing’s syndrome. Psychoneuroendocrinology, 27, 367–383. Forget, H., Lacroix, A., Somma, M., & Cohen, H. (2000). Cognitive decline in patients with Cushing’s syndrome. Journal of the International Neuropsychological Society, 6, 20–29. Haskett, R. F. (1985). Diagnostic categorization of psychiatric disturbance in Cushing’s syndrome. The American Journal of Psychiatry, 142, 911–916. Heald, A. H., Ghosh, S., Bray, S., Gibson, C., Anderson, S. G., Buckler, H., et al. (2004). Long-term negative impact on quality of life in patients with successfully treated Cushing’s disease. Clinical Endocrinology, 61, 458–465. Hook, J. N., Giordani, B., Schteingart, D. E., Guire, K., Giles, J., Ryan, K., et al. (2007). Patterns of cognitive change over time and relationship to age following successful treatment of Cushing’s disease. Journal of International Neuropsychological Society, 13, 21–29. Hudson, J. I., Hudson, M. S., Griffing, G. T., Melby, J. C., & Pope, H. G., Jr. (1987). Phenomenology and family history of affective disorder in Cushing’s disease. The American Journal of Psychiatry, 144, 951–953. Kelly, W. F. (1996). Psychiatric aspects of Cushing’s syndrome. QJM: Monthly Journal of the Association of Physicians, 89, 543–551.

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Kelly, W. F., Kelly, M. J., & Faragher, C. (1996). A prospective study of psychiatric and psychological aspects of Cushing’s syndrome. Clinical Endocrinology, 45, 715–720. Khiat, A., Bard, C., Lacroix, A., et al. (1999). Brain metabolic alterations in Cushing’s syndrome as monitored by proton magnetic resonance spectroscopy. NMR in Biomedicine, 12, 357–363. Lindsay, J. R., Nansel, T., Baid, S., Gumowski, J., & Nieman, L. K. (2006). Long-term impaired quality of life in Cushing’s syndrome despite initial improvement after surgical remission. The Journal of Clinical Endocrinology and Metabolism, 91, 447–453. Loosen, P. T., Chambliss, B., DeBold, C. R., Shelton, R., & Orth, D. N. (1992). Psychiatric phenomenology in Cushing’s disease. Pharmacopsychiatry, 25, 192–198. Mauri, M., Sinforiani, E., Bono, G., Vignati, F., Berselli, M. E., Attanasio, R., et al. (1993). Memory impairment in Cushing’s disease. Acta Neurologica Scandinavica, 87, 52–55. McEwen, B. S., Weiss, J. M., & Schwartz, L. S. (1968). Selective retention of corticosterone by limbic structures in rat brain. Nature, 220, 911–912. Newell-Price, J., Bertagna, X., Grossman, A. B., & Nieman, L. K. (2006). Cushing’s syndrome. Lancet, 367, 1605–1617. Nieman, L. K., Biller, B. M., Findling, J. W., et al. (2008). The diagnosis of Cushing’s syndrome: An endocrine society clinical practice guideline. The Journal of Clinical Endocrinology and Metabolism, 93, 1526–1540. Sonino, N., Fava, G. A., Belluardo, P., Girelli, M. E., & Boscaro, M. (1993). Course of depression in Cushing’s syndrome: Response to treatment and comparison with Graves’ disease. Hormone Research, 39, 202–206. Sonino, N., Fava, G. A., Raffi, A. R., Boscaro, M., & Fallo, F. (1998). Clinical correlates of major depression in Cushing’s disease. Psychopathology, 31, 302–306. Starkman, M. N., Gebarski, S. S., Berent, S., & Schteingart, D. E. (1992). Hippocampal formation volume, memory dysfunction, and cortisol levels in patients with Cushing’s syndrome. Biological Psychiatry, 32, 756–765. Starkman, M. N., Giordani, B., Gebarski, S. S., & Schteingart, D. E. (2006). Improvement in mood and ideation associated with increase in right caudate volume. Journal of Affective Disorders, 101, 139–147. Starkman, M. N., Schteingart, D. E., & Schork, M. A. (1981). Depressed mood and other psychiatric manifestations of Cushing’s syndrome: Relationship to hormone levels. Psychosomatic Medicine, 43, 3–18. Starkman, M. N., Schteingart, D. E., & Schork, M. A. (1986). Cushing’s syndrome after treatment: Changes in cortisol and ACTH levels, and amelioration of the depression syndrome. Psychiatry Research, 19, 177–188. Stewart, P. M. (2008). The adrenal cortex. In P. R. Larsen, H. M. Kronenberg, S. Melmed, & K. S. Polonsky (Eds.), Williams textbook of endocrinology, pp. 445–503. Whelan, T. B., Schteingart, D. E., Starkman, M. N., & Smith, A. (1980). Neuropsychological deficits in Cushing’s syndrome. The Journal of Nervous and Mental Disease, 168, 753–757.

Customized Job Retention Services A MY J. A RMSTRONG Virginia Commonwealth University Richmond, VA, USA

Definition Job retention is the process of facilitating the tenure of an individual in a particular employment setting. Customized job retention services begin with the completion of a thorough assessment to determine the individual’s strengths, interests and preferences, and support needs. The results of the assessment will inform the job development and selection process. Placement and retention plans are individualized; however, general guidelines insure job retention including promoting consumer choice and involvement, identifying jobs that are meaningful in terms of wages earned, benefits, inclusion, and opportunities for career growth, and the provision of long-term supports. Research indicates that the provision of ongoing supports increases the employment retention rate of individuals with significant disabilities. Retention services may also include supports addressing skill acquisition, production demands, the development of soft skills such as interpersonal communication, problem-solving skills and responding to the daily expectations and stressors associated with the respective position. Retention services must also ensure that the needs of the employer are understood and met. Job retention is directly related to quality of life, as reported by individuals with disabilities. Employment statistics (i.e., employment rate, ability to stay in a job overtime) document the need for the provision of customized job retention services. Improved job tenure rates for individuals with disabilities are a primary objective for rehabilitation services.

Cross References ▶ Customized Employment ▶ Supported Employment


Customized Employment ▶ Supported Employment

Griffin, C., Hammis, D., & Geary T. (2007). The job developer’s handbook: Practical tactics for customized employment. Baltimore, MD: Paul H. Brookes.

CVLT-C Rubin, S., & Roessler, R. (2008). Foundations of the vocational rehabilitation process. Austin, TX: Pro-Ed. Szymanski, E. M., & Parker, R. M. (2003). Work and disability: Issues and strategies in career development and job placement (2nd ed.). Austin, TX: Pro-Ed. Wagner, C. C., Armstrong, A. J., Fraser, R. T., Vandergoot, D., & Thomas, D. F. (2006). Evidence-based employment practices in vocational rehabilitation. In K. J. Hagglund & A. W. Heinemann (Eds.), Applied disability and rehabilitation research. New York: Springer. Wehman, P., Inge, K. J., Revell, G., & Brooke, V. (2007). Real work for real pay: Inclusive employment for people with disabilities. Baltimore, MD: Paul H. Brookes.

Cut Scores ▶ Cut Off Scores, Cutting Scores

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to assess the disorder must be reliably able to measure the particular abilities, taking into consideration the span of the standard error, to place an education-altering label on that child. If such standards are not followed, then clinicians run the risk of basing a decision on a ‘‘false-positive’’ (the discovery of a limitation when none exists in actuality) or a ‘‘false-negative’’ (the missing of a limitation when one truly exists). To consider an example, a cutoff score may be the score differentiating between intact performance and impairment on a particular test. To speak in terms of diagnosing cognitive impairment, in a normal distribution of scores, a cutoff score would be any score below 95% which is obtained by the intact group within a particular standardization sample. Clinicians may adjust cutoff scores because of individual or cultural variables that render the reliability of a test nonapplicable to the individual or sample being assessed. Otherwise, the clinician could run the risk of pathologizing an individual who does not truly exhibit the pathology.

Cut Off Scores, Cutting Scores S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

References and Readings Nunnally, J. C., & Bernstein, I. H. (1994). Psychometric theory (3rd ed.). New York: McGraw-Hill.

Synonyms Cut scores

CVA Definition

▶ Stroke

Cutoff scores, also referred to as cutting scores, are scores that differentiate the levels of performance.

CVLT Current Knowledge The reliability of a measure is an essential factor to consider when using cutoff scores. Nunnally and Bernstein (1994) suggest that a test must have an internal consistency coefficient of at least 0.90, and ideally above 0.95, to designate a particular score as a diagnostic cutoff point, although this might be a high standard. For instance, if a child is demonstrating a learning disorder, the tests used

▶ California Verbal Learning Test (California Verbal Learning Test-II)

CVLT-C ▶ California Verbal Learning Test – Children’s Version

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Cyst

Cyst E THAN M OITRA Drexel University Philadelphia, PA, USA

Synonyms Nodule; Polyp

Definition Cyst refers to an abnormal growth of benign cells, typically containing normal cerebrospinal fluid. Central nervous system cysts may occur as neoplasms or subsequent to leptomeningitis, hemorrhage, or surgery. Most patients presenting with cyst are asymptomatic, though headache; calvarial bulging; intracranial hypertension; craniomegaly; developmental delay; visual loss; precocious puberty; and seizures, with focal neurologic signs can occur. Additional cognitive consequences may include symptoms of dementia and inattention. Arachnoid cysts represent the most common form of benign cyst and occur in the cerebrospinal axis. Size of arachnoid cysts is not directly affected by changes in the ventricular system.

Cross References ▶ Neoplasm

Cytochrome P450 N ADIA W EBB Children’s Hospital of New Orleans New Orleans, LA, USA

Synonyms

major role in drug metabolism and is the cause for some adverse drug reactions. Metabolism and clearance of a drug are presumed to be stable, allowing for basic predictions about a drug’s dose, blood levels, and pharmacological effects. Drugs are typically metabolized by a limited number of mechanisms, some of these include the P450 enzyme pathways. Drugs, foods, or herbal preparations that alter the efficiency of those pathways may also alter the plasma concentration of some drugs by inhibiting or inducing (increasing) the efficiency of a particular enzymatic pathway. There are drugs/herbs that can simultaneously induce and inhibit different isoenzymes (St. John’s Wort), or simply induce their own metabolism (carbamazepine). Although over the counter preparations are less likely to be mentioned, some effect P450 enzyme pathways (e.g., cigarettes or the topical antifungal ketoconazole). Smokers, for example, may require increased doses of drugs because cigarette smoking has the ability to induce an enzyme that deactivates some medication. As a corollary, an abrupt cessation of smoking would then increase the plasma level of these drugs unless and until the dose is modified. P450 isoenzymes are widely distributed, but often thought of as liver enzymes because the liver metabolizes drugs through oxidation as well as reduction and hydrolysis. The p450 cytochrome system is critical to a number of oxidative reactions (the addition of an oxygen molecule or the removal of hydrogen from a molecule). The P450 isoenzymes share a heme (iron) cofactor, although they may use a number of different molecules as a basis for enzyme reactions. The term cytochrome arose because the isoenzymes were differentiated by their cellular (cyto) location and their color (chrome) or wavelength during spectrophotometric studies. P450 enzymes absorb light at 450 nm. Specific CYP enzyme pathways are noted in shorthand (e.g., CYP3A4 or CYP 2D6). Typically, authors include the prefix CYP before a three character alphanumeric 3A4, but it may be abandoned if the author is writing casually or for an audience familiar with the terms. The alphanumeric denotes the gene family, subfamily, and individual gene. The three most common isoenzymes involved in human drug metabolism are CYP3A4, CYP2D6, and CYP2C9.

P450 isoforms; P450 isoenzymes; P450 system

Definition

Cross References

The liver-mediated cytochrome P450 isoenzymes (abbreviated as CYP) constitute an enzyme family that plays a

▶ Cmax ▶ p450 Cytochrome System

Cytotoxic Edema

▶ Pharmacokinetics ▶ Side Effects

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Stahl, S. M. (2008). Stahl’s essential psychopharmacology: Neuroscientific basis and practical applications. New York: Cambridge University Press.

References and Readings Brunton, L. B., Lazo, J. S., & Parker, K. L. (Eds.). (2005). Goodman & Gilman’s the pharmacological basis of therapeutics (11th ed.). New York: McGraw Hill.

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D DAD ▶ Disability Assessment for Dementia

DAFS ▶ Direct Assessment of Functional Status

DAI ▶ Diffuse Axonal Injury

Selected Honors and Awards

Damasio, Antonio R. (1944– ) J OHN RYAN , T RICIA Z. K ING Georgia State University Atlanta, GA, USA

Education and Training University of Lisbon Medical School, Portugal (M.D.; 1969) Aphasia Research Center (Research Fellowship with Norman Geschwind; 1967) University of Lisbon, Portugal (Ph.D.; 1974) University Hospital, Lisbon, Portugal (Residency in Neurology, 1970–1972)

Language Research Laboratory (Center de Estudos Egas Moniz, Lisbon, Portugal, 1971–1975) Chief, Division of Behavioral Neurology & Cognitive Neuroscience (Department of Neurology, University of Iowa, Iowa City, IA, 1977–2005)

Sandoz Prize in Neurology (Portuguese Society of Neurology, 1971, 1973, 1974) William Beaumont Prize (American Medical Association, 1990) European Academy of Arts and Sciences (Elected 1993) Institute of Medicine of the National Academy of Sciences (Elected 1994) American Academy of Arts and Sciences (Elected 1997) Bavarian Academy of Sciences (Elected 2002) Signoret Prize in Cognitive Neuroscience (shared with Hanna Damasio, 2004) Prince of Asturias Award for Scientific and Technical Research (2005)


Major Appointments

Professor (University of Iowa, Iowa City, IA, 1980– 2005) President (Academy of Aphasia, 1983) President (Behavioral Neurology Society, 1985) Adjunct Professor (The Salk Institute for Biological Studies, La Jolla, CA, 1989-present) David Dornsife Professor of Neuroscience (University of Southern California, Los Angeles, CA, 2005–present) Director, Brain and Creativity Institute (University of Southern California, Los Angeles, CA, 2006–Present)

Antonio Damasio is a prominent neurologist, neuroscientist, and author. His research investigating the relations between brain damage and functioning has had a major influence on both Psychology and Philosophy, especially in the domains of emotion and decision-making. He has made major contributions in the areas of language, memory, and perception as well. His Somatic Marker Hypothesis posits that emotional states actually occurring in the body or simulated in the brain can influence decision-making. As a neuropsychologist, Damasio’s work has spanned numerous domains. Early in his career, his primary


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focus was on the relation between the brain and language, but his interests expanded to include memory, perception, and autism in the 1980s. By the late 1980s, his research focused on how brain injuries can teach about how concepts and categories are stored in the brain. He proposed a neural architecture for learning and recall based on convergence/divergence of signals from critical nodes called convergence–divergence zones. Throughout his career, he has focused primarily on the study of how brain damage affects psychological functioning, thereby inferring how the healthy brain operates. Damasio’s largest influence has been his theories on emotion and decision-making. Before 1995, the study of judgment and decision-making rarely or tangentially considered how emotion could influence behavior. In Descartes’ Error, Damasio advanced the hypothesis that not only could emotion aid reason in decision-making, but reason itself evolved from emotion and the two remain critically interconnected. Central to Damasio’s theory is the Somatic Marker Hypothesis, which proposes that, when making decisions, options are marked either negatively or positively, consciously or unconsciously, thus deterring an individual from making a negative choice or, on the contrary, promoting a positive choice. In the years since this revolutionary idea, numerous studies have lent support to the idea that emotion and reason are interdependent. These studies have included patients with ventromedial prefrontal damage who are unable to monitor visceral states and, as a result, make poor decisions. The Somatic Marker Hypothesis, however, has not been immune to criticism. Recently, these criticisms have focused on the extensive reliance of the hypothesis on the Iowa Gambling Task and sometimes-conflicting evidence from other laboratories regarding the mediational role of the ventromedial prefrontal cortices. Since the publication of Descartes’ Error, Damasio has extended his work on emotion to include research and philosophy on the nature of feelings, morality, and consciousness. He has proposed that, in addition to mapping representations of the external world and internal states, regions such as the posterior cingulate cortex and related cortices (the postero-medial cortices) create a ‘‘second-order representation’’ which allows an organism to develop a sense of self. These studies have begun to form a bridge between neuropsychology and philosophy, posing testable hypotheses regarding metacognition.

Short Biography Antonio Damasio was born in Lisbon, Portugal in 1944. He attended the University of Lisbon Medical School, and received his M.D. in 1969 followed by his Ph.D. in 1974 from the University of Lisbon. In 1967, he studied with Norman Geschwind at the Aphasia Research Center (Boston, MA) and later returned to America in 1975 when he became an Assistant Professor at the University of Iowa, earning the distinction of Full Professor in 1980. His association with the Salk Institute began in the 1980s. He remained at the University of Iowa until 2005 when he moved to the University of Southern California as the director of the Brain and Creativity Institute, and Professor of Psychology, Neuroscience, and Neurology.

Cross References ▶ Geschwind, Norman (1926–1984)

References and Readings Damasio, A. R. (1989a). Time-locked multiregional retroactivation: A systems level proposal for the neural substrates of recall and recognition. Cognition, 33, 25–62. Damasio, A. R. (1989b). The brain binds entities and events by multiregional activation from convergence zones. Neural Computation, 1, 123–132. Damasio, A. R. (1996). The somatic marker hypothesis and the possible functions of the prefrontal cortex. Transactions of the Royal Society (London), 351, 1413–1420. Damasio, A. R. (1999, 2000). The feeling of what happens: Body and emotion in the making of consciousness. New York: Harcourt. Damasio, A. R. (2003). Looking for Spinoza: Joy, sorrow and the feeling brain. New York: Harcourt. Damasio, A. R. (2005). Descartes’ error: 10th anniversary edition, with a new author preface. New York: Penguin Books. Damasio, A. R., Grabowski, T. J., Bechara, A., Damasio, H., Ponto, L. L. B., Parvizi, J., et al. (2000). Subcortical and cortical brain activity during the feeling of self-generated emotions. Nature Neuroscience, 3, 1049–1056. Damasio, A. R., & Meyer, K. (2008a). Behind the looking-glass. Nature, 454, 167–168. Damasio, A. R., & Meyer, K. (2008b). Consciousness: An overview of the phenomenon and of its possible neural basis. In S. Laureys & G. Tononi (Eds.), The neurology of consciousness. Oxford: Elsevier. Dunn, B. D., Dalgleish, T., & Lawrence, A. D. (2006). The somatic marker hypothesis: A critical evaluation. Neuroscience and Biobehavioral Reviews, 30, 239–271. Gluck, A. (2007). Damasio’s error and descartes’ truth: An inquiry into consciousness, metaphysics, and epistemology. Scranton, PA: University of Scranton Press.

Daubert v. Merrell Dow Pharmaceuticals (1993)

D-Amphetamine J OANN T. T SCHANZ 1, K ATHERINE T REIBER 1,2 1 Utah State University Logan, UT, USA 2 University of Massachusetts Medical School Worcester, MA, USA

Synonyms Dextroamphetamine; Dexedrine

Definition Dextroamphetamine (D-amphetamine) is one of two chemical forms of the synthetic stimulant ‘‘amphetamine,’’ and is a potent central nervous system stimulant that increases the release and blocks the reuptake of the monoaminergic neurotransmitters, dopamine, norepinephrine, and serotonin. Acute administration of the stimulant is associated with increased alertness, confidence, euphoria, and cognition. In animals, there is a dose-dependent effect of increasing activity such as locomotion and at higher doses, stereotyped motor behaviors. D-amphetamine’s reinforcing properties and potential for abuse are thought to reflect increased dopamine neurotransmission in the nucleus accumbens. The drug also increases both systolic and diastolic blood pressure and increases respiration and heart rate, among other autonomic nervous system effects (Feldman, Meyer, & Quenzer, 1997; Iversen, Iversen, Bloom, & Roth, 2009).

Current Knowledge Although historically, D-amphetamine has had clinical uses for narcolepsy, as an appetite suppressant, and cognitive enhancer, much has been written of its addictive properties. As with other drugs of abuse, dependence and tolerance can develop with chronic use, resulting in the administration of increasing doses to achieve the desired effects. Repetitive, stereotyped behaviors may emerge with sustained use. With chronic abuse, a psychotic syndrome resembling the symptoms of paranoid schizophrenia may emerge. Although some differences between amphetamine-induced psychosis and schizophrenia have been identified, this observation has provided some support for the dopamine hypothesis of schizophrenia (Iversen et al., 2009). Other negative effects of chronic

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amphetamine abuse include possible neurotoxic damage to the dopamine system with reduced numbers of transporters and receptors. Impairments in attention and memory have been reported with abuse (Iversen et al., 2009).

Cross References ▶ Amphetamine

References and Readings Feldman, R. S., Meyer, J. S., & Quenzer, L. F. (1997). Stimulants: Amphetamine and Cocaine. In Principles of neuropsychoparhmacology (pp. 549–568). Sunderland, MA: Sinauer. Iversen, L. L., Iversen, S. D., Bloom, F. E., & Roth, R. H. (2009). Psychostimulants. In Introduction to neuropsychopharmacology (pp. 447–472). New York: Oxford University Press.

Dance-like ▶ Chorea

DAS-II ▶ Differential Ability Scales (DAS and DAS-II)

Daubert v. Merrell Dow Pharmaceuticals (1993) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition This case involved two persons, Jason Daubert and Eric Schuller, who had been born with severe birth defects. Along with their parents, they sued Merrell Dow Pharmaceutical Inc., based on the claim that the drug Bendectin had caused their birth defects. Merrell Dow had the case placed in federal court and moved for summary judgment due to the fact that their expert witness provided documents indicating that no published scientific study existed to provide support for a causal relationship

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between Bendectin and birth defects. By contrast, Daubert and Schuller provided their own expert evidence which indicated that Bendectin could indeed lead to birth defects. This expert testimony was based primarily on in vitro and in vivo animal studies, pharmaceutical studies, and a reanalysis of already published studies. However, because such methodologies had not garnered acceptance within the scientific community, the court awarded summary judgment to Merrell Dow. Daubert and Schuller appealed the decision to the Ninth Circuit Court. The Court deemed that the district court had correctly granted summary judgment since the plaintiff’s evidence was not deemed as utilizing reliable methodologies as determined by the scientific community. Additionally, it appeared as if the evidence produced by the plaintiff seemed to be created only for the purpose of litigation. The case was reviewed by the U.S. Supreme Court who determined that the Federal Rules of Evidence (FRE) govern the admissibility of scientific evidence presented in federal court. Moreover, it was decided that the judge is to act as the ‘‘gatekeeper’’ prior to admitting evidence in order to ensure that the evidence is scientifically valid and relevant to the particular case. The court provided certain criteria to make determinations regarding the scientific validity of information presented. For example, the guidelines include: falsifiability (has the methodology been tested), peer review (has the methodology been subjected to peer-review process), acceptable error rate, general acceptance of methods or principles, technical manual/guide for use of instrument, etc.

Current Knowledge The decision in the Daubert case has created some controversy within the field of psychology in general and forensic neuropsychology more specifically. Most notably, Reed (1996) viewed the Daubert ruling as implying that only fixed battery, commercially acceptable, neuropsychological batteries may be admissible. Given that most neuropsychologists employ a flexible battery approach, this created unrest among the neuropsychological community. However, many have challenged Reed’s interpretation of the Daubert case and subsequent rulings (e.g., ▶ Chapple v. Ganger) have deemed neuropsychological data based on flexible batteries as admissible. Challenges to scientific information relayed by expert witnesses are referred to as Daubert or Frye and such hearings are held in limine, or away from the jury. Specifically, the judge listens to the pros and cons of the scientific evidence and the attorneys usually argue for or against admissibility of a specific test or conclusion.

Cross References ▶ Admissibility ▶ Chapple v. Ganger ▶ Joiner v. General Electric (1997) ▶ Kumho Tire v. Carmichael

References and Readings Chapple vs. Ganger, 851 F. Supp. 1481, E.D. of Washington, (1998). Daubert vs. Merrell Dow, 509, U.S. 579 (1993). Frye v. U.S. D.C. Cir., 293 F. 1013 (1923). Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney – Neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Joiner v. General Electric, 522 U.S. 136 (1997). Kumho Tire v. Carmichael 526 U.S. 137 (1999). Reed, J. E. (1996). Fixed versus flexible neuropsychological test batteries under the Daubert standard for admissibility of scientific evidence. Behavioral Sciences and the Law, 14, 315–322.

DCT ▶ Dot Counting Test

De Renzi, Ennio (1924– ) M ICHELLE P ROSJE University of Florida Gainesville, FL, USA

Major Appointments

Fellowship (School of Medicine, University of Pavia, 1953–1956) Faculty (Department of Nervous and Mental Diseases, University of Modena, 1956–1957) Faculty (University of Milan, 1958–1969) Professor of Neurology (University of Trieste, 1969– 1971) Chair of Nervous and Mental Diseases (School of Medicine, University of Trieste, 1969–1971) Professor of Neurology (University of Milan, 1971– 1974) Professor and Head (Department of Neurology, University of Modena, 1974–2002) Honorary Visiting Professor (University of Aberdeen, 1996)

De Renzi, Ennio (1924– )


President of the European Brain and Behavior Society (1973–1974) Carlo Riquier Award (University of Pavia, 1988) Honorary Degree in Psychology (University La Sapienza of Rome, 1993) Member of the Academia Europea (1994) Honorary Member of the American Neurological Association (1995) Honorary Member of the Academy of Neurology (1995) Honorary Member of the British Neuropsychological Society (1996) Professor Emeritus (University of Modena, 2002) Member by Election, Academy of Aphasia Member of Research Group on Aphasia of the World Federation of Neurology Member of International Neuropsychological Symposia


Ennio De Renzi’s fame stems from his heartfelt devotion to establishing neuropsychology as a scientific discipline in Italy. One of the most influential postwar neuropsychologists (Milner, 1997), De Renzi contributed greatly to the understanding of a broad range of neurological phenomena, including aphasia, apraxia, agnosia, topographical amnesia, and disorders of language, spatial cognition, and memory. His seminal works in these areas, particularly hemispheric specialization and neuropsychological deficits in patients with localized brain lesions, were ahead of his time. His insatiable exploration into unchartered neurological and psychological territories spanned more than 40 years and prompted further neuropsychological research. During his early years studying neurology and psychiatry, De Renzi worked closely with Professor Gildo Gastaldi from the University of Pavia at two psychiatric and two neurological wards. Gastaldi ultimately asked De Renzi to join his group in the Department of Nervous and Mental Disease at the University of Modena. A few years later when Gastaldi accepted a new position and moved to Milan to become the Chair of Nervous and Mental Disease, De Renzi followed in Gastaldi’s footsteps, subsequently deciding to put aside his interest in psychiatry and devote himself entirely to neurology.

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De Renzi’s initial studies on aphasia were conducted in collaboration with Luigi A. Vignolo and took Geschwind’s model into consideration. Vignolo had completed a thesis on aphasia and had considerable training in language and its disorders. De Renzi and Vignolo garnered the assistance of a local mime group to study the potential role of nonverbal information in the aphasics’ comprehension deficit (De Renzi, 1998). The group presented short sketches miming everyday actions (e.g., hammering a nail). In support of their original impairment hypothesis, De Renzi and Vignolo found that aphasics demonstrated significant comprehension deficits. For the Seventh International Congress of Neurology, they submitted a presentation about the aphasics’ impairment hypothesis including their recent findings about comprehension dysfunction. Arthur L. Benton reviewed their manuscript, providing encouragement and prompting them to contemplate the behavior of normal subjects. This first contact with Benton soon evolved into a lifelong mentorship and friendship for De Renzi. Benton not only shared knowledge and presented lectures, but also pushed De Renzi to develop strong methodological discipline during his time in Iowa City as a Fullbright Professor. Benton’s influence became evident during the 1960s and 1970s when De Renzi began to shift his focus from examining small groups of selected patients to studying unselected clinical cases. Vignolo and De Renzi, together with other members of an interdisciplinary group which originally included such well-known scientists as Henry Hećaen, Hans Teuber, and Clemens Faust, met to discuss various neurological phenomena, namely aphasia, constructional apraxia, alexia, cerebral dominance, effects of unilateral versus bilateral lesions, and disorders of time sense and body schema. In 1962, the efforts of De Renzi, Vignolo, Brenda Milner, Klaus Poeck, and Oliver Zangwill renewed the group that formally adopted the name of the International Neuropsychological Symposium (Stringer & Cooley, 2002). Also in 1962, De Renzi and Vignolo collaborated on a paper about the Token Test, which has been one of De Renzi’s most influential works. During the early 1960s, establishing research protocols was challenging in light of scarce grant funding. De Renzi put forth great effort to assemble a group of colleagues willing to devote countless hours in exchange for name recognition on papers. This group came to be known as the Milan Group and consisted of Vignolo, Pietro Faglioni, Hans Spinnler, and Anna Basso. Piero Faglioni, an Italian neurologist with a

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passion and expertise for statistical analysis, played a principal role in influencing De Renzi’s approach to experimental design. De Renzi’s application of clinical cases and appropriate methodology fostered inferential thinking about his patients with unilateral hemispheric damage and consequently prompted examination of the interplay between specialization/ asymmetry and anterior–posterior lesion location. In 1964, one year after Henry Hećaen established the journal Neuropsychologia, De Renzi founded Cortex. Cortex was created as an international journal devoted not only to the anatomy of the central nervous system, but more so to a focus on cognition and the relation between the central nervous system and mentation. Of particular interest was the behavior of patients, including children, with acquired brain lesions versus normal controls. In addition, the journal sought to provide insight into the use of functional neuroimaging techniques to record brain activity. De Renzi noted that the only way to subject one’s research to a broader audience and to contribute to the field was to publish in international journals rather than ‘‘burying (it) in Italian journals (2006).’’ With the birth of this journal, De Renzi embarked on a challenging mission to acquire not only the English language, but also a specific manner in which to report statistical data. The first editorial board of the journal included numerous prominent intellectual critics of the time, namely, Arthur Benton, George Ettlinger, Norman Geschwind, Harold Goodglass, and Klaus Poeck. Gastaldi, one of De Renzi’s early teachers, was the editor in chief. When Gastaldi died in 1973, De Renzi assumed the role of editor in chief and actively held this position until 2000 when Sergio Della Sala succeeded him. During the 1960s, the European Brain and Behavior Society was formed, whose aim was to provide an opportunity for international scientists to assemble to share interests and beliefs about the biological foundations of behavior. De Renzi actively participated in the preliminary discussions to create the society and was nominated as president for 1971–1973. In 1969, the Italian Society of Neurology’s invitation to De Renzi to present about hemispheric dominance represented a momentous shift in the field. At the meeting, De Renzi reviewed his findings from the previous 6 years of research on a wide range of neuropsychological topics. At this time, neuropsychology seemed to be gaining in popularity as young students became impressed with neuropsychology, and an increasing number of psychological and neurophysiological departments were emerging around the world (De Renzi, 1998). In

1969, De Renzi was called to hold the Chair of Nervous and Mental Diseases by the School of Medicine at the University of Trieste. Following his tenure in Milan and Gastaldi’s death in 1973, De Renzi accepted a position as Chair of Nervous and Mental Diseases in Modena. He acted as chief of a department with 70 neurological beds and 40 psychiatric beds. In 1982, De Renzi published his influential book Disorders of Space Exploration and Cognition, a book that remains a cornerstone in the field of neuropsychology, with particularly rich insight into perceptual and visuomotor disorders. He eloquently and perceptively combined pertinent animal research with detailed discussions of human neuropsychological research in his book. Over the span of his 50-year devotion to neuropsychology, De Renzi has published on a broad range of topics. He distinguished four levels in the processing of spatial information, including exploration, perception, memory, and conceptualization. He described the phenomena of constructional apraxia, hand and eye forced deviation, and disorders of space perception. De Renzi and his group examined hemispatial neglect and developed a purely acoustic test for auditory neglect. He also directed attention toward agnosia. Along with Faglioni and Spinnler, De Renzi devised a strategy to tackle recognition disorders by investigating each hemisphere’s contribution to visual information processing. As technology advanced, De Renzi utilized computed tomography scans to enhance his research. From prosopagnosia and Balint’s syndrome to memory disorders and frontal signs, De Renzi’s extensive research agenda contributed much to the field of neuropsychology as it is know today. In fact, he more recently examined the anatomy of disorders in depth perception (Turnbull, Driver, & McCarthy, 2004) and focused on tactile agnosia as his final area of research. De Renzi has considered himself primarily as a clinician who engages in research and teaching not only for selfedification, but also to shed light on the patient’s conditions and optimal interventions. De Renzi believed that the pure essence of neuropsychology dwells in the interface of the patient’s treatment, research protocols, and teaching. ‘‘I always felt the opportunity to shift from patient examinations to research and teaching, far from causing loss of concentration and dissipation of energy, is an asset because it keeps your mind open and inquisitive on a wide range of practical and theoretical aspects of reality and can be immensely gratifying’’ (De Renzi, 1998).

Deaf

Short Biography Ennio De Renzi was born in the agricultural hub of Cremona, Italy on December 18, 1924. The atmosphere in Italy during the first 20 years of his life was filled with fascist propaganda and devoid of any freedoms or liberties. As such, De Renzi noted it was ‘‘repressive, untruthful, and boring’’ (De Renzi, 1998). His maternal grandfather played a large role during De Renzi’s early years, prompting him to devour as many books as possible. As a voracious reader during his school years, De Renzi learned to read literature in the original languages of French and German. Fortunately, De Renzi’s book knowledge flourished at a time when he was required to relocate to 14 different cities during the first 17 years of his life due to his father’s military career. Unfortunately, he had significant difficulties establishing strong interpersonal relationships with classmates and teachers. Interestingly, De Renzi had no interest in the sciences during secondary school. So, he decided to study law at the university. Patriotic propaganda permeated the political climate at that time while underneath the country was struggling. De Renzi noted that after the Nazi occupation of Italy and his attempt to locate a niche in the free part of the country, he enlisted as a private and served alongside his father as a colonel in the Italian army (1998). Even though he had already passed seven law examinations, De Renzi was drawn to medicine, particularly during wartime turmoil. After reading a book about brain functioning, he became fascinated with the workings of the mind and decided to abandon law, take his medical entrance exams, and forge ahead into the field of medicine. In 1945, he accepted a position at the Ghislieri College in Pavia, Italy. This institution was one of the few that offered deserving students from less-affluent families the opportunity for a university education free of charge. And so began his odyssey into the field of neurology and ultimately neuropsychology. From his early childhood years, De Renzi sought to broaden his cultural horizons. As a youngster, he avidly read literature from various countries. During college, he desired to interact with friends from different regions of the world. He established the international journal, Cortex, to facilitate international communication and dissemination of knowledge. De Renzi firmly believed that visiting foreign cities and spending time with colleagues around the world were added bonuses to his profession. He gleaned intimate knowledge about other places and expanded his cultural horizons throughout every

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stage of his life. He commented that he sees his life as positive and gratifying in that his interest and curiosity about culture have enriched every day on earth, and he has led both the family and career life he has desired (De Renzi, 1998).

Cross References ▶ Agnosia ▶ Anomic Aphasia ▶ Apraxia ▶ Arthur L. Benton ▶ Balint’s Syndrome ▶ Hemispatial Neglect ▶ Hemispheric Specialization ▶ Prosopagnosia

References and Readings De Renzi, E. (1996). Balint-Holmes’ syndrome. In C. Code (Ed.), Classic cases in neuropsychology (pp. 123–144). New York, NY: Psychology. De Renzi, E. (1998). Ennio De Renzi. In L. R. Squire (Ed.), The history of neuroscience in autobiography (Vol. 5, pp. 227–269). The Society for Neuroscience. http://www.sfn.org/skins/main/pdf/history_of_neuroscience/hon_vol_5/c5.pdf. Retrieved 19 June 2009. De Renzi, E. (1999). Agnosia. In G. Denes & L. Pizzamiglio (Eds.), Handbook of clinical and experimental neuropsychology (pp. 371–408). New York, NY: Psychology. De Renzi, E. (2000). Prosopagnosia. In M. J. Farah & T. E. Feinberg (Eds.), Patient-based approaches to cognitive neuroscience (pp. 85–95). Cambridge, MA: MIT. De Renzi, E., Cavalleri, F., & Facchini, S. (1996). Imitation and utilization behavior. Journal of Neurology, Neurosurgery, and Psychiatry, 61, 396–400. De Renzi, E., Lucchelli, F., Muggia, S., & Spinnler, H. (1997). Is memory loss without anatomical damage tantamount to a psychogenic deficit? The case of pure retrograde amnesia. Neuropsychologia, 35(6), 781–794. Milner, A. D. (1997). Ennio De Renzi. In N. Sheehy, A. J. Chapman, & W. Conroy (Eds.), Biographical dictionary of psychology (pp. 137–138). New York, NY: Routledge. Stringer, A. Y., & Cooley, E. L. (2002). Neuropsychology: a twentiethcentury science. In A. Y. Stringer, E. L. Cooley, & A.-L. Christenson (Eds.), Pathways to prominence in neuropsychology: reflections of twentieth- century pioneers (pp. 3–26). New York, NY: Psychology. Tesak, J., & Code, C. (2008). Milestones in the history of aphasia: Theories and protagonists. New York, NY: Psychology. Turnbull, O. H., Driver, J., & McCarthy, R. A. (2004). 2D but not 3D: Pictorial-depth deficits in a case of visual agnosia. Cortex, 40, 723–738.

Deaf ▶ Deaf/Hearing Impairment

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Deaf/Hearing Impairment D EBORAH W ITSKEN University of Northern Colorado Greeley, CO, USA

Synonyms

intensities (measured by decibels). Audiologists classify hearing loss as normal (0–15 db loss), mild (25–40 db loss), moderate (41–55 db loss), moderately severe (56–70 db loss), severe (71–90 db), or profound (>90 db). Degree of hearing loss may also be delineated based on functional descriptions of the hearing loss impact (e.g., those with mild hearing loss may be able to communicate using the telephone whereas those with a profound hearing loss often cannot understand spoken language via telephone).

Deaf; Hard of hearing; Hearing impaired; Hearing loss

Epidemiology Short Description or Definition Medical definitions of deafness refer to impairment in the physical structures necessary for hearing and understanding language. The term ‘‘deaf ’’ refers to a degree of hearing loss that significantly impacts access to auditory language; ‘‘hard of hearing’’ typically refers to hearing loss that still allows for some access to auditory information. Medical and functional definitions of deafness do not necessarily correspond with an individual’s cultural identity. The cultural definition (commonly indicated by the capitalized term ‘‘Deaf ’’) refers to individuals who reject the medical notion that deafness represents impairment per se and identify with a community of individuals that share common experiences, a rich cultural heritage, and a shared language – sign language (Lane et al., 1996).

The World Health Organization (2005) estimates indicate 278 million people worldwide have moderate to profound hearing loss in both ears. Eighty percent of individuals with hearing loss live in low- and middle-income countries. Men are more likely to experience hearing loss than women. The majority of individuals experiencing hearing loss are over 30 years old. In the United States, although estimates vary, most indicate that one to six per 1,000 babies are born with severe to profound congenital hearing loss (ASHA, 2009). The most common cause of conductive hearing loss is chronic otitis media. Causes of sensorineural hearing loss include genetics, premature birth, anoxia, maternal rubella during pregnancy, use of ototoxic drugs, infectious diseases (e.g., meningitis, measles, mumps), head injury, foreign bodies blocking or damaging the ear canal, and prolonged exposure to excessive noise.

Categorization Hearing is commonly defined according to where the problem occurs and the degree of hearing loss. Conductive hearing impairment refers to problems occurring in the outer or middle ear, which are commonly medically or surgically treatable (i.e., otitis media). Sensorineural hearing impairment typically refers to a failure in the cochlea’s ability to translate mechanical energy produced by the middle ear into neural impulses sent to the eigth nerve. Mixed hearing loss refers to simultaneous occurrence of both difficulties. Conductive and sensorineural hearing losses are differentiated from various types of central hearing loss, often referred to as ‘‘cortical deafness,’’ which refers to impairment in the ability to interpret sounds despite adequate functional mechanisms for hearing (e.g., ▶ pure word deafness, ▶ auditory agnosia). Degree of hearing loss is typically defined by one’s ability to perceive sounds of different frequencies (measured by hertz or cycles per second) and at different

Natural History, Prognostic Factors, Outcomes The impact of hearing loss varies widely depending on the cause, level, and type of hearing impairment and the age of onset. Onset prior to the development of speech and language is associated with greater difficulties with language acquisition, academic achievement, and social and occupational functioning. Research indicates that early identification of hearing loss and intervention yields significant benefits on language, academics, and social-emotional development (Calderon & Naidu, 2000; Yoshinaga-Itano, Sedey, Coulter, & Mehl, 1998). With the adoption of newborn infant hearing screening procedures, many children are identified with hearing loss in the first months of life, compared to an average age of 18 months prior to universal screenings (Mertens, Sass-Lehrer, & Scott-Olson, 2000). Full access to language has a significant impact on predicted outcomes. Research has indicated that reading


levels of deaf and hard of hearing high school graduates have traditionally hovered around the 4th grade level while deaf undergraduate college students with deaf parents read on average at 11th grade level (Hauser, 2001; Traxler, 2000). Recognizing the importance of communication, many families struggle to determine the most appropriate method for augmenting hearing and providing access to communication – whether in English and/or through a signed language. No single solution has emerged as panacea for all individuals with hearing loss. Regardless of communication modality, positive parent– child interaction has been identified as a predictor of the child’s linguistic development (Calderon & Naidu, 2000; Pressman, Pipp-Siegel, Yoshinaga-Itano, & Deas, 1999). Research indicating that over 90% of children who are deaf have parents who are hearing highlights the common struggle experienced by many families to find a suitable communication system (Mitchell & Karchmer, 2002). Struggles with literacy secondary to limited access to spoken English often translate into career challenges. Unfortunately, compared to hearing peers, unemployment rates are higher for individuals who are deaf and hard of hearing and large numbers receive Supplemental Security Disability Insurance (SSDI) (Danek & Busby, 1999). However, the employment challenges of individuals with hearing loss are not significantly different from other individuals with disabilities. For example, there is no difference in the probability of employment between youth with learning disabilities and those with hearing loss (Wagner, Newman, Cameto, Garza, & Levine, 2005). Notably, these group means may overshadow the satisfactory quality of life attained by many individuals who are deaf and hard of hearing, many of whom attain successful careers in areas such as education, psychology, neuropsychology, medicine, and law. Advances in technology and legal precedent established through the Americans with Disability Act continue to broaden opportunities available to individuals who are deaf and hard of hearing.

Neuropsychology and Psychology of Deafness/Hearing Loss Neuropsychologists should be cognizant that many causes of hearing loss, particularly nongenetic causes, are commonly associated with other neurological factors that may have a severe impact on functioning. When isolated from comorbid neurological conditions, hearing loss has been shown to impact cognitive organization but not intellectual functioning (Braden, 1994). fMRI studies have indicated that similar regions in the left frontal and temporal

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lobes are activated in individuals using sign language and those relying on spoken language. However, in native sign language users, there is additional activation in the righthemisphere superior temporal and inferior parietal regions (Newman, Bavelier, Corina, Jezzard, & Neville, 2002). Other subtle differences in cognitive organization have been found among individuals with hearing loss who prefer to communicate using sign language, including improved peripheral visual attention, mental rotation, image generation, and face processing (for review, see Hauser, Wills, & Isquith, 2006; Marscharck & Hauser, 2009). Despite these subtle differences, research has indicated that when assessed nonverbally by an evaluator familiar with their preferred communication mode, individuals who are deaf and hard of hearing perform similarly to hearing peers on standardized, nonverbally administered intelligence tests (Braden, 1994; Maller, 2003). Considerable controversy exists regarding whether there exists a ‘‘psychology of deafness,’’ that is, whether there are certain traits that are uniquely associated with individuals who are deaf or hard of hearing (Paul & Jackson, 1993). Others suggest that this viewpoint represents a culturally biased perspective (Lane, 1988). Some researchers have suggested that there may be indirect developmental consequences of growing up with hearing loss in a world that relies heavily on auditory input (Hauser et al., 2006). Compared to same-age hearing peers, individuals who are deaf and hard of hearing may be more likely to be exposed to risk factors that can contribute to developmental and behavioral challenges. These factors may include academic difficulties, low selfesteem, inconsistent discipline, and sexual/physical abuse (Black & Glickman, 2005; Simeonsson, Wax, & White, 2001). Although children with hearing loss develop attachments to caregivers similarly to their peers, the diagnosis of a child’s hearing loss may impact parents’ attachment to their child and contribute to difficult parent–child interactions (Meadow-Orlans, 1990). Children who have not developed adequate language and communication skills regardless of modality are more likely to demonstrate difficulties with self-regulation and social skills (Rhine-Kahlback, 2004). Findings of greater prevalence of psychopathology among individuals with hearing loss are controversial and may reflect methodological flaws. Greater rates of psychopathology may not be attributable to hearing loss per se, but rather may reflect characteristics of dysfunctional families such as those commonly associated with greater rates of psychopathology among individuals who do not have hearing loss (Powers, Elliott, Patterson, & Shaw, 1995). Limited communication skills in any modality are

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associated with poor psychosocial functioning and may significantly limit access to therapeutic intervention (Black & Glickman, 2005). Clinicians who are not skilled in working with deaf clients may be more likely to provide a narrow range of diagnoses to clients who are deaf and may over diagnose psychotic disorders. In contrast, when assessed by deafness mental health specialists, lower rates of psychosis and a greater range of less severe psychiatric problems are reported (Black & Glickman, 2009).

Evaluation Hearing loss is typically identified through audiological assessment which may include behavioral, electroacoustic, and electrophysiological measures. Immittance audiometry identifies outer and middle ear function. Audiological evaluation also involves obtaining a puretone audiogram which depicts an individual’s hearing sensitivity at varying frequencies and intensities. Newborn infant hearing screening procedures, which allow for early diagnosis of hearing loss, may include otoacoustic emissions screening (OAE) and auditory brain stem response screening (ABR). Numerous factors impact the validity and reliability of neuropsychological assessment of individuals who are deaf and hard of hearing. Misdiagnoses of mental retardation, learning problems, and psychopathology are common when assessed by individuals not appropriately trained to form clinical diagnoses based on an understanding of the dynamic interplay between deafness and its associated causes, language functioning and accessibility, and family dynamics. Factors complicating the assessment process include the match between the evaluator and client’s preferred communication modality, selection of assessments demonstrated to be valid and reliable with clients who are deaf, use of interpreters, and interpretation of test results. Translation into a different communication modality or use of other accommodations may alter an assessment’s underlying construct and its psychometric properties, therefore invalidating the results. As an example, one of the most commonly used assessments of intelligence, the Wechsler Intelligence Scale for Children – III, has been demonstrated to measure the construct of intelligence among children with hearing loss differently than same-age hearing peers at the factor and item levels (Maller & Ferron, 1997). Nonverbal assessment tools which do not rely heavily on verbal content reflect best practice when assessing individuals with hearing loss (Braden, 1994; Maller, 2003). Specialized training is required to adequately conduct psychological and

neuropsychological evaluations with individuals from this unique population. Neuropsychologists are strongly encouraged to consult with and refer to clinicians trained in deafness.

Treatment Treatment varies depending on the cause and nature of the hearing loss as well as one’s cultural perspective. No one treatment approach has been demonstrated to be effective for all individuals who are deaf or hard of hearing. Conductive hearing losses are often treated with antibiotics or minor surgeries, such as insertion of pressure-equalization tubes. Individuals with sensorineural hearing loss may benefit from use of hearing aids or a cochlear implant (CI) (refer to Pisoni et al., 2008 for a review of factors impacting outcomes of CI users). Rehabilitation may include use of an amplification system in addition to language stimulation programs, use of assistive devices, auditory training, and speech therapy. Environmental accommodations (e.g., preferential seating), use of adaptive technology to improve access (e.g., flashing light systems), and communication repair strategy training are also essential. The mental health needs of individuals who are deaf and hard of hearing traditionally have been underserved. Referral to a mental health professional trained in deafness, preferably one with fluency in the client’s preferred communication mode, reflects best practice. Indirect communication through an interpreter during therapy may negatively impact the therapeutic process by interfering with the patient–therapist relationship, increasing the likelihood of miscommunication, and interfering with the therapist’s ability to reliably assess the client’s mental status (Leigh, Corbett, Gutman, & Morere, 1996; Sussman & Brauer, 1999). When interpreters must be used, it is preferable to use a consistent, certified interpreter.

Cross References ▶ Auditory Cortex ▶ Auditory System

References and Readings American Speech-Language-Hearing Association (2009). The prevalence and incidence of hearing loss in children. Retrieved June 11, 2009, from http://www.asha.org/public/hearing/disorders/children.htm

Dean–Woodcock Neuropsychological Assessment System Black, P., & Glickman, N. (2005). Language dysfluency in the deaf inpatient population. JADARA, 39(1), 303–321. Black, P., & Glickman, N. (2009). Language and learning challenges in the deaf psychiatric population. In Cognitive-behavioral therapy for deaf and hearing persons with language and learning challenges (pp. 1–46). New York: Taylor & Francis. Braden, J. P. (1994). Deafness, deprivation, and IQ. New York: Plenum. Calderon, R., & Naidu, S. (2000). Further support for the benefits of early identification and intervention for children with hearing loss. The Volta Review, 100(5), 53–84. Danek, M. M., & Busby, H. (1999). Transition planning and programming: empowerment through partnership. Washington, DC: Gallaudet University Press. Hauser, P. C. (2001). Deaf readers’ phonological encoding: An electromyogram study of covert reading behaviour. Dissertation Abstracts International, 62, 4B. (UMI No. AAI3012772). Hauser, P. C., Wills, K., & Isquith, P. K. (2006). Hard-of-hearing, deafness, and being Deaf. In J. E. Farmer, J. Donders, & S. A. Warschausky (Eds.), Treating neurodevelopmental disabilities (pp. 119–131). New York: Guilford. Lane, H. (1988). Is there a ‘‘psychology of the deaf ’’? Exceptional Children, 55, 7–19. Lane, H., Hoffmeister, R., & Bahan, B. J. (1996). A journey into the deafworld. San Diego, CA: Dawn Sign Press. Leigh, I., Corbett, C. A., Gutman, V., & Morere, D. A. (1996). Providing psychological services to deaf individuals: A response to new perceptions of diversity. Professional Psychology: Research and Practice, 27, 364–371. Maller, S. J. (2003). Intellectual assessment of deaf people: A critical review of core concepts and issues. In M. Marshark & P. E. Spencer (Eds.), Oxford handbook of deaf studies, language, and education (pp. 451–463). New York: Oxford University Press. Maller, S. J., & Ferron, J. (1997). WISC-III factor invariance across deaf and standardization samples. Educational and Psychological Measurement, 7, 987–994. Marscharck, M., & Hauser, P. C. (Eds.). (2009). Deaf cognition: Foundations and outcomes. New York: Oxford University Press. Meadow-Orlans, K. P. (1990). Research on developmental aspects of deafness. In D. E. Moores & K. P. Meadow-Orlans (Eds.), Education and developmental aspects of deafness (pp. 283–298). Washington, DC: Gallaudet University Press. Mertens, D. M., Sass-Lehrer, S., & Scott-Olson, K. (2000). Sensitivity in family professional relationships: Potential experiences of families with young deaf and hard of hearing children. In P. T. Spencer, C. J. Erting, & M. Marschark (Eds.), The deaf child in the family at school (pp. 133–150). Mahwah, NJ: Erlbaum. Mitchell, R. E., & Karchmer, M. A. (2002). Chasing the mythical ten percent: Parental hearing status of deaf and hard of hearing students in the United States. Sign Language Studies, 4, 138–163. Newman, A. J., Bavelier, D., Corina, D., Jezzard, P., & Neville, H. J. (2002). A critical period for right hemisphere recruitment in American Sign Language processing. Nature Neuroscience, 5, 76–80. Paul, P. V., & Jackson, D. W., (1993). Toward a psychology of deafness. Needham Heights, MA: Allyn & Bacon. Pisoni, D., Conway, C., Kronenberger, W., Horn, D. L., Karpicke, J., & Henning, S. C. (2008). Efficacy and effectiveness of cochlear implants in deaf children. In M. Marscharck & P. C. Hauser (Eds.), Deaf cognition: foundations and outcomes (pp. 52–101). New York: Oxford University Press. Powers, A. R., Elliott, R. N., Patterson, D., & Shaw, S. (1995). Family environment and deaf and hard of hearing students with mild

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additional disabilities. Journal of Childhood Communication Disorders, 17, 15–19. Pressman, L., Pipp-Siegel, S., Yoshinaga-Itano, C., & Deas, A. (1999). The relation of sensitivity to child expressive language gain in deaf and hard-of-hearing children whose caregivers are hearing. Journal of Deaf Studies and Deaf Education, 4, 294–304. Rhine-Kahlback, S. (2004). The assessment of developmental language differences, executive functioning, and social skills in deaf children. Unpublished doctoral dissertation, Gallaudet University, Washington, DC. Simeonsson, R. J., Wax, T. M., & White, K. (2001). Assessment of children who are deaf or hard of hearing. In R. Simeonsson & S. Rosenthal (Eds.), Psychological and developmental assessment: Children with disabilities and chronic conditions (pp. 248–266). New York: Guilford. Sussman, A. E., & Brauer, B. A. (1999). On being a psychotherapist with Deaf clients. In I. W. Leigh (Ed.), Psychotherapy with deaf clients from diverse groups (pp. 3–22). Washington, DC: Gallaudet University Press. Traxler, C. B. (2000). The Stanford achievement test, 9th edition: National norming and performance standards for deaf and hard of hearing students. Journal of Deaf Studies and Deaf Education, 5(4), 337–348. Wagner, M., Newman, L., Cameto, R., Garza, N., & Levine, P. (2005). After high school. A first look at the postschool experiences of youth with disabilities. A report from the National Longitudinal Transition Study – 2 (NLTS-2). Menlo Park, CA: SRI International. Retrieved June 1, 2009, from http://www.nlts2.org/pdfs/afterhighschool_ report.pdf WHO (2005). Deafness and Hearing Impairment. Retrieved 6/11/2009, from http://www.who.int/mediacentre/factsheets/fs300/en/print. html Yoshinaga Itano, C., Sedey, A. L., Coulter, D. K., & Mehl, A. L. (1998). The language of early and later identified children with hearing loss, Pediatrics, 102, 1161–1171.

Dean–Woodcock ▶ Dean–Woodcock Neuropsychological Assessment System

Dean–Woodcock Neuropsychological Assessment System S COTT L. D ECKER University of South Carolina Columbia, SC, USA

Synonyms Dean–Woodcock; DW; DWNB

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Dean–Woodcock Neuropsychological Assessment System

Description The Dean–Woodcock Neuropsychological Battery (DWNB) (Dean & Woodcock, 2003) consists of a Sensory-Motor Battery (SMB), Structured Neuropsychological Interview, and Emotional Status Exam. The DWNB contains many components of a traditional neuropsychological assessment. The SMB consists of measures traditionally found in other neuropsychological batteries such as the Halstead–Reitan. Although the DWNB may be used with any cognitive measure, it was co-normed with the Woodcock–Johnson Third Edition (Woodcock, McGrew, & Mather, 2001). The SMB includes one measure of laterality, six sensory measures, and seven motor measures. The sensory tests assess a variety of functions including peripheral and near point visual acuity, auditory acuity, and a variety of tactile functions. The motor measures assess gross motor (i.e., Gait and Station and Romberg), visualmotor construction and coordination, and a variety of other motor functions including finger tapping and grip strength. Once the test is administered, raw scores are converted into W-scores. W-scores are Rasch-based measures with equal-interval properties. Next, the Wscore is subtracted from a Reference W, which represents the average W-score for a particular age range. Qualitative interpretation of impairment based on scores includes the labels within normal limits, mild impairment, moderate impairment, and severe impairment. Since many tests include lateralized indicators of functioning (right and left), lateralized impairment may be easier to detect in the summary of scores by marking the impairment label with an ‘‘L’’ for left and an ‘‘R’’ for right. Standard scores are not provided because of the non-normal distribution of scores for most of the tests. Base rates for frequency of impairment are provided in the manual. Total composite of sensory and motor functioning may be obtained by adding W-scores across tests, although the validity of such indices is unknown. Spanish administration procedures are identical to English procedures. The Emotional Status Examination and the Neuropsychological Interview are important components of the DWNB. The Emotional Status Examination consists of three sections. The first section records relevant demographic information such as name and age. The second section systematically reviews 50 psychiatric symptoms sampled from a large variety of psychiatric illnesses. The purpose is to cover a large number of disorders relatively quickly. Endorsement of particular psychiatric signs or symptoms may require additional follow-up or testing.

Each psychiatric symptom is rated on a rating scale of severity and duration. The last section, Clinical Observations and Impressions, provides an assessment of behavior based on clinical judgment. The Structured Neuropsychological Interview covers all major background elements important for a clinical evaluation. Information is directly collected from the subject or parent and covers biographical information, referral concerns, medical history, psychiatric history, social history, family history, and development. Information obtained from the Structured Neuropsychological Interview, as well as the Emotional Status Examination, are useful in helping to provide meaningful interpretation to the performance measures in either the sensorimotor exam or from tests of cognition or achievement.

Historical Background Subtests of the DWNB are based on theories of neuropsychological functioning and examination procedures historically used in neurology. Many of the neurological exams grew from the historic roots of early clinical observations of brain–behavior correspondence. For example, Broca’s early observations of the left-inferior frontal lobe involvement in language production are now viewed as a type of aphasia and assessed through language measures on the DWNB. One primary difference between subtests of the DWNB and the historic counterparts from neurology is the use of psychometric measurement theory. Whereas neurologists may use similar procedures, the DWNB provides standardization procedures and normative information. Normative information is useful for developmentally sensitive measures where less than perfect performance is normal. Additionally, base rates of impairment help determine the likelihood of performance across various clinical conditions such as apraxia, agnosia, aphasia, dysarthria, somesthesia, etc.

Psychometric Data Numerous reliability studies were reported from both the manual and published resources. Reliability estimates for each measure are reported across the developmental age range and in general are satisfactory for their intended purpose. The normative information is based on a sample of 1,011 subjects from age 4 to 80þ. The sample was matched to the U.S 2000 census by gender and race. Numerous validity studies were also

Death Penalty

reported including content, developmental, and construct validity.

Clinical Uses The DWNB provides a fairly comprehensive neuropsychological battery that assesses most areas of relevance in neuropsychology. Additionally, its psychometric properties increase its value in clinical assessment. Clinicians familiar with the components can complete the battery in about an hour. Scoring software may be purchased separately and reduces the chances of calculation errors.

Cross References ▶ Halstead–Reitan Neuropsychological Test Battery ▶ Neuropsychology

References and Readings Dean, R. S., & Woodcock, R. W. (2003). Dean-Woodcock neuropsychological battery. Itasca, IL: Riverside. Dean, R. S., Woodcock, R. W., Decker, S. L., & Schrank, F. A. (2003). A cognitive neuropsychology assessment model. In F. A. Schrank & D. Flanagan (Eds.), WJ III clinical use and interpretation: Scientistpractitioner perspective (pp. 345–377). SanDiego, CA: Academic. Woodcock, R. W., McGrew, K. S., & Mather, N. (2001). WoodcockJohnson III. Itasca, IL: Riverside.

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Definition The death penalty, otherwise known as ‘‘capital punishment,’’ is one of three possible punishments rendered for defendants found guilty of murder or other capital crimes. The death penalty involves execution of the defendant. The U.S. Supreme Court has determined that the death penalty is not typically a violation of the Eight Amendment’s ban on cruel and unusual punishment. Moreover, the Sixth Amendment does not necessitate a jury trial in order to sentence someone to death. Capital punishment in the USA is a highly controversial issue as evidenced by the prohibition of capital punishment in certain states. Moreover, various religious affiliations possess disparate views related to the moral grounds of the death penalty. There are several issues for forensic neuropsychologists to consider related to capital punishment. First and foremost, it is crucial that prior to accepting a role in a capital case, that the practitioner evaluate his/her personal positions on the death penalty and in accordance with ethical standards. If one’s personal views hold the potential to impede with one’s professional role, then they should not assume the role of a consultant or expert in a capital case. Neuropsychologists involved with death penalty cases usually are involved with determinations of competency to be executed, competency to waive death penalty appeals, assessment of mental retardation, and other mitigating factors (e.g., brain damage, effects of a history of abuse, etc.)

Cross References

Death ▶ Brain Death

Death Penalty R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Capital punishment

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▶ Aggravating Factors ▶ Atkins v. Virginia ▶ Mitigating Factors

References and Readings Cunningham, M. D., & Goldstein, A. M. (2003). Sentencing determinations in death penalty cases. In A. Goldstein (Ed.), Handbook of psychology (Vol. 11). Forensic psychology, New Jersey: Wiley. Heilbronner, R. L., & Waller, D. (2008). Neuropsychological consultation in the sentencing phase of capital cases. In R. Denney, & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford. Reynolds, C., Price, J. R., & Niland, J. (2003). Applications of neuropsychology in capital felony (death penalty) defense. Journal of Forensic Neuropsychology, 3, 89–123.

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Deceleration Injury

Deceleration Injury B ETH R USH Mayo Clinic Jacksonville, FL, USA

Cross References ▶ Acceleration Injury ▶ Biomechanics of Injury ▶ Diffuse Axonal Injury

Synonyms


Acceleration–deceleration injury

Barth, J. T., Freeman, J. R., Broshek, D. K., & Varney, R. N. (2001). Acceleration-deceleration sport-related concussion: The gravity of it all. Journal of Athletic Training, 36(3), 253–256. Meythaler, J. M., et al. (2001). Archives of Physical Medicine and Rehabilitation, 82, 1461–1471. Olvey, S. E., et al. (2004). Neurosurgery, 54, 672–677.

Definition Deceleration injury is a traumatic injury to the brain, typically following an acceleration injury to the brain in a high-speed situation such as a motor vehicle accident or high-impact sports. Deceleration injuries typically result in additive damage to an acceleration injury after the speed and inertia of momentum is obstructed. In this situation, the brain that is moving forward in the direction of inertia falls backward in the opposite direction, which may result in further compression of the underlying brain tissue and white matter connections.

Decerebrate Posturing J ACOB K EAN Indiana University School of Medicine Indianapolis, Indiana, USA

Synonyms Current Knowledge Extensor posturing Similar to acceleration injuries, primary injury in deceleration injury results in bruising, hemorrhage, and shearing of the underlying tensile strength of white matter connections deep within the brain. Secondary injury may occur hours or even days after the inciting traumatic event. Secondary effects of injury can include decreased cerebral blood flow, edema, hemorrhage, increased intracranial pressure, and biochemical changes that may cause excitotoxicity and more extensive damage to the surrounding brain structures and their associated connections. Acceleration and deceleration injuries are not uncommon in high-impact sports such as football and hockey. Neuropsychologists are now often involved in baseline cognitive screens for professional sports players and in subsequent cognitive evaluations following a traumatic event in which a concussion or traumatic brain injury is suspected. Professional sports organizations strongly endorse the use of helmets in such sports activities to reduce the amount of free range of motion in the head that can potentially cause a more severe acceleration– deceleration injury.

Definition Decerebrate posturing is a pattern of movement commonly produced by extensive forebrain lesions including the subcortex. It is characterized by rigidity, extension of the arms with wrists pronated, extension of the legs, downward pointing of the toes, and sometimes arching of the spine. Decerebrate posturing may be partial or asymmetric. Decerebrate (or extensor) posturing is distinguished from decorticate (or flexor) posturing by the extension of the arms. Decerebrate posturing is generally the result of massive and bilateral forebrain lesions or other damage that includes upper brainstem and sometimes the rostral pons. Common causes include cerebral infarction (stroke), intracranial hemorrhage, primary brain tumor, secondary brain tumor, traumatic head injury, increased intracranial pressure from any cause, brainstem tumor, and metabolic or hepatic encephalopathies.

Declarative Memory

Cross References

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Declarative (Explicit) Memory

▶ Decorticate Posturing

References and Readings Episodic Memory Posner, J. B., Saper, C. B., Schiff, N. D., & Plum, F. (2007). Plum and Posner’s diagnosis of stupor and coma. New York: Oxford University Press.

Declarative Memory J ILLIAN S CHUH University of Connecticut Storrs, CT, USA

Synonyms Explicit memory

Definition Declarative memory refers to long-term memory, which stores information that can be consciously discussed or declared. Declarative memory, which is the form of memory as viewed by most lay people, involves the deliberate encoding of information for objects, events, or facts. One of several distinct memory systems (Lezak, Howieson, & Loring, 2004), declarative memory is often contrasted with non-declarative or procedural memory, which refers to the unconscious encoding of procedures, routines, or patterned information for learning skills or processes. Declarative memory is further divided into episodic, the memory for experiences localized in time and space, and semantic, the memory for learned knowledge that is not time locked to a specific event; e.g., rote memorization of a historical fact or the letters of the alphabet (Fig. 1).

Historical Background The theory of multiple memory systems largely began with the distinction between declarative and procedural memory. This distinction is observed in patients with anterograde amnesia, who have an inability to store new declarative memories, while memory for skills or

- Experiences and events localized in time and space - Awareness of the personal experience

Semantic Memory - Not localized in time and space - Not connected to experience where acquired - Factual information

Declarative Memory. Figure 1 Components of declarative memory

procedures remains largely intact. One famous patient, H.M., described by Brenda Milner and William Beecher Scoville in a classic 1957 paper, underwent surgery to reduce seizures caused by intractable epilepsy. After the bilateral removal of his hippocampus and medial temporal lobes, H.M. suffered profound anterograde amnesia. He was no longer able to store new declarative memories and had a limited ability to retrieve previously stored memories (Corkin, 1968; Milner, 1965). However, his ability to learn new skills (procedural memory) appeared largely unaffected. For example, in a task of mirror drawing, H.M.’s accuracy improved with each testing session. However, H.M. was unable to recognize the details of the testing environment, and thought each session to be novel, as though he were encountering the testing materials for the first time (Milner, 1965; Scoville & Milner, 1957). H.M.’s case provides an example of anatomic dissociation, in which tasks that differ in their declarative versus procedural memory demands are associated with distinct neuroanatomical structures. This anatomic dissociation suggests that declarative and procedural tasks may rely on distinct memory storage and retrieval systems in the brain.

Current Knowledge Since the time of H.M., many studies have examined memory in a variety of neurological presentations. In particular, the consolidation of declarative memory has been an area of interest and debate. While several memory functions have been identified, there is controversy over the stages involved in the formation of declarative memories (Parkin & Rapp, 2001). For example, there is longstanding debate as to whether short term memory is a distinct function or one element in the consolidation

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process (Fuster, 2003; McGeoch, 1932). Many studies suggest that there are three stages that are clinically useful (Loring, 1999): sensory memory, short term/immediate memory, and long term/secondary memory (Fig. 2). In the first stage, lasting several seconds at the most, information is held in a sensory store, while extraneous stimuli in the environment are filtered out (Crowder, Surprenant, & Kazdin, 2000). The information either quickly decays or is further processed as short-term memory if there is rehearsal or additional support for its salience. Short-term memory, known as immediate memory, serves as a temporary store. Lasting from roughly 30 s to several minutes (Lezak et al., 2004), this storage allows for the integration of relevant perceptual information. If the memory trace is not processed for long-term consolidation, the memory quickly dissipates. For example, upon receiving the new phone number of a friend, you may remember it for the several minutes before dialing. However, it may be difficult to recall that number several hours later. The number is retained while it is relevant, but after a short period of time this memory dissipates. Memory traces can be retained for up to several hours through rehearsal (the engagement of repetitive mental processes; Brown, Craik, & Tulving, 2000). If this number was important to remember, you might repeat it several times or use a mnemonic device (a word or phrase representing the numbers) to enhance your memory. The process of rehearsal increases the chance of that information being retained or encoded in long-term memory. However, it does not guarantee long term storage, as conscious rehearsal can only be maintained temporarily. Rehearsal techniques often imply that longterm memory formation happens through effortful attention. However, memories may also form through incidental learning, which requires little conscious effort.

Sensory memory (milliseconds to several seconds)

Repetition Short term/ Immediate Memory (thirty seconds to several minutes)

Long term/ Secondary Memory (hours to years)

Memory decay/ Forgetting

Declarative Memory. Figure 2 Stages of declarative memory

The consolidation of long-term memories is thought to occur through a process known as long-term potentiation (LTP; Tranel & Damasio, 2002). LTP, lasting anywhere from hours to months, is the simultaneous stimulation of two or more neurons that results in an increased synaptic strength, improving communication between the synapses (Cooke & Bliss, 2006). This stimulation of a neural network primes the system for learning. Long-term memory formation occurs when continued stimulation increases synaptic strength, allowing the learning to be retained. Memory storage does not occur at particular sites in the brain, but rather results from the contribution of many cortical networks (Markowitsch, Tulving, & Craik, 2000). Several areas of the brain have been identified as particularly critical in declarative memory consolidation. These include the hippocampus, midline diencephalon, medial temporal lobe, mammillary bodies, thalamus, fornix, and frontal lobes (Parkin & Rapp, 2001).

Future Directions While it is clinically useful to organize the process of declarative memory formation into distinct stages, these stages may be functionally interdependent. For example, recent research suggests that short-term memory may simply be the early part of the consolidation process in memory formation (Fuster, 2003). Additionally, the distinction between episodic and semantic memory can also be fuzzy. If you learn that Nouakchott is the capital of Mauritania, tomorrow you are likely to remember the event of learning it (e.g., reading this article, where you were when reading, what happened before and after, etc.), making it an episodic memory. However, what happens if tomorrow, a radio program has a feature on Mauritania? If you repeatedly hear this fact, the episodic memory will become a semantic memory. Conversely, if you have the semantic knowledge that Nouakchott is the capital of Mauritania, and then help your friend with a geography project on Mauritania, you now have an episodic memory involving a semantic memory. It is likely these components are not separate memory systems, but rather two processes that are closely intertwined (Willingham, 2004). Even the distinction between declarative and procedural memory can become blurred. Some researchers speculate that there is a developmental progression during learning upon which knowledge moves from more implicit representations to more declarative representations as the learner matures (Alibali & Koedinger, 2000). Research continues to address these relationships, and it is likely that a multidisciplinary approach will further the

Decorticate Posturing

knowledge of declarative memory. Brain imaging studies are improving the understanding of the anatomical networks involved in declarative memory processes. Such an understanding will guide the examination of declarative memory formation at the cellular level, so that we will not only be able to understand the anatomical areas important for declarative memory, but have better insight into the formation of declarative memory. Additionally, computational modeling can shed insight into how one memory processes may interact with another. Finally, much of the research on declarative memory in humans has focused on typical adults or patients with lesions occurring later in life (as in the cases of studies of anterograde amnesia). While this has given immense knowledge of the declarative memory system, relatively little empirical work has examined the development of this system. One imaging study by Ofen et al. (2007) comparing children to adults found increased activation in the prefrontal cortex with increasing age. Additionally, activation in the prefrontal cortex correlated with developmental gains in the ability to remember details of an experience. Further examination of developmental processes will not only provide insight on the maturational trajectory of declarative memory, but also help further the understanding of the relationship between those memory systems which appear seemingly distinct.

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Fuster, J. M. (2003). Cortex and mind: Unifying cognition. New York: Oxford University Press. Lezak, M. D., Howieson, D., & Loring, D. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Loring, D. W. (1999). INS dictionary of neuropsychology. New York: Oxford University Press. Markowitsch, H. J., Tulving, E., & Craik, F. I. M. (2000). Neuroanatomy of memory. The Oxford handbook of memory (pp. 465–484). New York: Oxford University Press. McGeoch, J. A. (1932). Forgetting and the law of disuse. Psychological Review, 39(4), 352–370. Milner, B. (1965). Memory disturbance after bilateral hippocampal lesions. In P. Milner & S. Glickman (Eds.), Cognitive processes and the brain. Princeton: Van Nostrand. Ofen, N., Kao, Y. C., Sokol-Hessner, P., Kim, H., Whitfield-Gabrieli, S., & Gabrieli, J. D. (2007). Development of the declarative memory system in the human brain. Nature Neuroscience, 10(9), 1198–1205. Parkin, A. J., & Rapp, B. (2001). The structure and mechanisms of memory. The handbook of cognitive neuropsychology: What deficits reveal about the human mind (pp. 399–422). New York: Psychology Press. Scoville, W. B., & Milner, B. (1957). Loss of recent memory after bilateral hippocampal lesions. Journal of Neurology, Neurosurgery, and Psychiatry, 20(1), 11–21. Tranel, D., & Damasio, A. R. (2002). Neurobiological foundations of human memory. In A. D. Baddeley, B. A. Wilson, & F. N. Watts (Eds.), Handbook of memory disorders. (2nd ed., pp. 27–50). Oxford: John Wiley & Sons. Willingham, D. T. (2004). Cognition: The thinking animal (2nd ed.). Upper Saddle River, NJ: Pearson/Prentice Hall.

Cross References ▶ Alzheimer’s Dementia ▶ Alzheimer’s Disease ▶ Anterograde Amnesia ▶ Dementia ▶ Episodic Memory ▶ Procedural Memory ▶ Semantic Memory

References and Readings Alibali, M. W., & Koedinger, K. R. (2000). The developmental progression from implicit to explicit knowledge: A computational approach. Behavioral and Brain Sciences, 22(05), 755–756. Brown, S. C., Craik, F. I. M., & Tulving, E. (2000). Encoding and retrieval of information. The Oxford handbook of memory (pp. 93–107). New York: Oxford University Press. Cooke, S. F., & Bliss, T. V. P. (2006). Plasticity in the human central nervous system. Brain: A Journal of Neurology, 129(7), 1659–1673. Corkin, S. (1968). Acquisition of motor skill after bilateral medial temporal-lobe excision. Neuropsychologia, 6(3), 255–265. Crowder, R. G., Surprenant, A. E. M., & Kazdin, A. E. (2000). Sensory stores. Encyclopedia of psychology (Vol. 7) (pp. 227–229). Washington, DC: American Psychological Association.

Decompressive Craniectomy ▶ Craniectomy

Decorticate Posturing J ACOB K EAN Indiana University School of Medicine Indianapolis, Indiana, USA

Synonyms Flexor posturing

Definition Decorticate posturing is a pattern of movement produced by extensive lesions in white matter, internal capsule, or

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thalamus, and characterized by rigidity, flexion of the arms, fists and fingers, and extension of the legs. Decorticate (or flexor) posturing may be partial or asymmetric, and is distinguished from decerebrate (or extensor) posturing by the flexion in the arms, which positions the hands close to the heart.

Definition Bilateral implantation of electrode leads into target areas of the brain to treat movement disorders.

Current Knowledge Cross References ▶ Decerebrate Posturing

References and Readings Posner, J. B., Saper, C. B., Schiff, N. D., & Plum, F. (2007). Plum and Posner’s diagnosis of stupor and coma. New York: Oxford University Press.

De-efferented State

DBS was first performed in 1987 to treat tremor of Parkinson’s disease and essential tremor. The exact mechanism of action for DBS is unknown, though it is likely related to the inhibition/blockade of excitatory membrane potentials. In Parkinson’s disease, leads are usually implanted in the subthalamic nucleus and internal part of the globus pallidus (GPi). The procedure is performed with stereotactic localization of the selected nuclei and can be performed in one or two surgeries depending on regional preferences and standard of care. DBS generally allows for reduction in the need for medications for the symptoms of Parkinson’s disease. Motor fluctuations and tremor are most affected by DBS. Improvements in tremor, rigidity, and bradykinesia are sustained. DBS is considered a reversible procedure.

▶ Locked-In Syndrome

Cross References

Deep Brain Stimulation (DBS)

▶ Parkinson’s Disease ▶ Tremor

▶ Deep Brain Stimulator (Parkinsons)


Deep Brain Stimulator (Parkinsons) C INDY B. I VANHOE Baylor College of Medicine Houston, TX, USA

Synonyms Activa®; Deep brain stimulation (DBS); Kinetra®

Erola, T., Heikkinen, R. R., Haapaniemi, T., Tuominen, J., Juolasmaa, A., & Myllyla, V. (2006). Efficacy of bilateral subthalamic nucleus (STN) stimulation in Parkinson’s disease. Acta Neurochirurgica, 148, 389–394. Kleiner-Fisman, G., Fisman, D., Sime, E., Saint-Cyr, J. A., Lozano, A. N., & Lang, A. (2003). Long term follow up of bilateral deep brain stimulation of the subthalamic nucleus in patients with advanced Parkinson disease. Journal of Neurosurgery, 99, 489–495. Krause, M., Fogel, W., Mayer, P., Kloss, M., & Tronnier, V. (2004). Chronic inhibition of the subthalamic nucleus in Parkinson’s disease. Journal of the Neurological Sciences, 219, 119–124. Vesper, J., Chabardes, S., Fraix, V., Sunde, N., & Ostergaard, K. (2002). Dual Channel deep brain stimulation system (kinetra) for Parkinson’s disease and essential tremor: a prospective multicenter open clinical study. Journal of Neurology, Neurosurgery, and Psychiatry, 73, 275–280.

Default Network

Deep Venous Thrombosis ▶ Venous Thrombosis

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Default Network L AWRENCE H. S WEET Brown University Providence, RI, USA

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De-escalation Synonyms DAWN E. B OUMAN Drake Center, Cincinnati, OH, USA

Default mode network; Task-negative network

Definition Definition De-escalation is the reduction in the intensity of out-ofcontrol, agitated behavior and the prevention of further behavioral crisis. Following a traumatic brain injury or other neurological disorder, including stroke or brain tumor, some people become more easily confused, overwhelmed, threatened, fearful, uncertain, impulsive, and less able to control their behavior, resulting in increasing levels of verbal and physical aggression. De-escalation techniques – including decreasing stimulation, offering calm reassurance, redirecting, and reorienting – can decrease agitation and lessen the intensity of a behavioral outburst.

The default network is a system of brain regions that is active when there are no external cognitive demands. The greatest brain activity is observed in this system during unstructured rest, and the least activity is observed during tasks that require concerted external focus. The existence and core location of the default network are widely accepted; however, its function and subsystems remain under investigation. The default network is distributed bilaterally and is comprised of at least two core midline regions, the posterior cingulate cortex and medial frontal gyrus, plus the inferior parietal lobule and areas in the medial and lateral temporal lobe.

Historical Background Cross References ▶ Agitation ▶ Behavior Management ▶ Catastrophic Reaction ▶ Crisis Intervention

References and Readings Gervasio, A. H., & Matthies, B. K. (1995). Behavioral management of agitation in the traumatically brain-injured person. Neurorehabilitation, 5, 309–316.

Default Mode Network ▶ Default Network

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Identification of the default network emerged unexpectedly from functional neuroimaging studies that administered paradigms with a variety of active cognitive challenges. Functional neuroimaging designs frequently sample brain activity during resting states in order to provide a baseline to which the task-elicited brain response to active experimental challenges are compared. During the middle of the 1990s, it became apparent that a set of brain regions consistently exhibited greater activity during the resting baseline periods compared to different active experimental challenges. This surprisingly consistent pattern of ‘‘resting activity’’ appeared as ‘‘deactivation’’ when compared to the active experimental conditions on maps of significant brain response. Moreover, this significant deactivation was often as robust as the expected task-related activation. This discovery motivated discussions about improving experimental designs to better control baseline assessments and much greater attention to deactivation patterns. Although deactivation patterns were often not even reported in early studies, within a decade, they became a major focus of

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functional neuroimaging research. An early meta-analysis of visual processing generated the first major interest in deactivation patterns in 1997. Schulman and colleagues found common deactivation patterns across studies that administered different visual tasks. These deactivations corresponded to regions now known as the default network. In 2001, Raichle and colleagues first labeled this baseline activity ‘‘a default mode’’ of brain function in a series of influential papers that substantially increased research interest in this system.

Current Knowledge Investigation of default network activity has revealed consistent inverse relationships with a wide variety of externally focused tasks that range from visual processing to more complex working memory tasks. Consistent positive relationships have been observed with several internal thought processes such as stimulus independent thoughts, daydreaming, mind wandering, imagining another person’s perspective, imagining the future, selfreferential thought, and retrieving memories. At rest and during externally focused cognitive challenges, default network activity has been found to be inversely correlated with another set of brain regions that are recruited during externally focused goal-directed processing (i.e., task-positive network). Therefore, the default network may represent one component of an opponent process system that is responsible for introspection, while the task-positive network may be related to processing with external attentional focus. It has been suggested that the general function of the default network is flexible mental simulation. Cognitive subprocesses may also be mapped onto different subsystems of the default network. For instance, areas in the medial temporal lobes appear to be recruited during memory contributions to default processing, while increased activity in the medial frontal cortices has been associated with evaluation of self-relevant situations. Altered default network activity has been reported among older adults and several clinical populations, including patients with autism, Alzheimer’s disease, depression, anxiety disorders, and schizophrenia.

Future Directions There has been rapid growth in research on the default network since it was first recognized as more than simply

uncontrolled baseline processing. The research focus very quickly transitioned from explaining observed deactivation patterns, to mapping the default network, and finally to the larger implications for cognitive neuroscience and neuropsychiatry. Although core regions have been well described and empirically supported, the full anatomical extent and specific functions of this network remain under investigation. Future work will likely be aimed at narrowing and integrating the broad cognitive functions that have been attributed to the default network and its subsystems, for example, the theory that the default network serves as an imagery simulator for creative thinking. These and other cognitive constructs associated with the default network will require reliable and valid operational definitions that are feasible to use in functional neuroimaging environments. Integration of the default network with other brain systems such as the task-positive network and attentional systems is also likely to be a major goal of future research. Investigation of the role of the default network in neuropsychiatric disorders is providing novel findings and a new framework within which to interpret them. Clinical research on the default network remains limited; however, this direction is very likely to grow rapidly.

References and Readings Buckner, R. L., Andrews-Hanna, J. R., & Schacter, D. L. (2008). The brain’s default network: Anatomy, function, and relevance to disease. Annals of the New York Academy of Sciences, 1124, 1–38. Fox, M. D., Snyder, A. Z., Vincent, J. L., Corbetta, M., Van Essen, D. C., & Raichle, M. E. (2005). The human brain is intrinsically organized into dynamic, anticorrelated functional networks. Proceedings of the National Academy of Sciences USA, 102(27), 9673–9678. Raichle, M. E., MacLeod, A. M., Snyder, A. Z., Powers, W. J., Gusnard, D. A., et al. (2001). A default mode of brain function. Proceedings of the National Academy of Sciences USA, 98, 676–682. Shulman, G. L., Fiez, J. A., Corbetta, M., Buckner, R. L., Miezin, F. M., et al. (1997). Common blood flow changes across visual tasks: II.: Decreases in cerebral cortex. Journal of Cognitive Neurosciences, 9, 648–663.

Defective Visual Localization ▶ Optic Ataxia

Defensiveness ▶ K Scale

Dejerine, Joseph Jules (1849–1917)

Deficit Measurement S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

Definition Deficit measurement is the measurement of impairment when an examinee’s performance is significantly below an actual or estimated previous level of functioning.

Current Knowledge In neuropsychology, deficit measurement can be achieved by assessing cognitive functioning as separate domains or as a composite of domains. For instance, cognitive deficits in a particular ability domain, such as list-learning ability, can be measured via the administration of a list-learning task during which the examinee must recall a list of words both immediately and after a specified time delay. If the examinee performs poorly, the score on this subtest provides information about deficits associated with the examinee’s list-learning ability. In the case of using composite scores, several subtests are combined to generate a composite score that represents performance in a general domain of functioning, such as auditory memory. This could include several separate aspects of functioning, such as list-learning, narrative memory, encoding of abstract concepts, etc. The level of deficit is determined by comparing the examinee’s subtest or composite score with that of the normative group (the group of individuals in the population who have been examined with the same test) or with the examinee’s previous level of functioning. Deficit measurement thus highlights the concept of ‘‘normality,’’ given that a particular level of performance to represent a deficit when the level of performance falls below the level most commonly observed (also called the criterion level) in the population at large is considered. Deficit measurement is often captured indirectly, by estimating the examinee’s premorbid ability level, ideally by clinical interview with the examinee and others, behavior rating scales, and premorbid test results, and comparing the examinee’s present performance with this estimate. More direct comparison can be challenging because objective measures of an examinee’s previous level of functioning may be unavailable.

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Cross References ▶ Best Performance Method ▶ Individual Comparison Standards ▶ National Adult Reading Test ▶ Premorbid Functioning ▶ Wechsler Test of Adult Reading

References and Readings Knapp, S. J., & VandeCreek, L. D. (2006). Assessment. Washington, DC: American Psychological Association. Lezak, M. D., Howieson, D. B., Loring, D. W., Hannay, H. J., & Fischer, J. S. (2004). Neuropsychological Assessment. New York: Oxford University Press. Snyder, P. J., Nussbaum, P. D., & Robins, D. L. (2006). Clinical neuropsychology: A pocket handbook for assessment. Washington, DC: American Psychological Association.

Degenerative ▶ Atrophy

Deglutition Disorder ▶ Dysphagia

Dejerine, Joseph Jules (1849– 1917) J ACKIE L. M ICKLEWRIGHT, T RICIA Z. K ING Georgia State University Atlanta, GA, USA

Education and Training

1868 and 1870: Academie de Geneve (Bachelors of Science in Biology and Comparative Anatomy) 1875: Hospital de la Pitie (Internship) 1879: Faculty of Medicine of Paris (Doctor of Medicine)

Major Appointments

Head of Clinic, Hospital Bicetre (1879–1886) Professor of Neurology and Chief Consultant, Hospital Bicetre (1887–1894)

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Dejerine, Joseph Jules (1849–1917)

Professor of Neurology, Salpetriere (1895–1911) Clinical Chair in Diseases of the Nervous System, Salpetriere (1911–1917)


Vice-President Societe de Biologie (1895) Founding member of the French Neurological Society (1899) Moxon Gold Medal of the Royal College of Physicians of London (1914)


French neurologist J. Jules Dejerine earned acclaim for his localizationist approach to the study of the nervous system by combining the anatomical approach of Charcot with the experimental approach of Vulpian. Dejerine’s early research focused on the pathophysiology of sensation and acquired disorders of spoken and written language. His body of work yielded detailed clinical and anatomical descriptions of a number of cognitive disorders, including pure alexia, aphasia, tactile agnosia, and thalamic syndrome.

Short Biography J. Jules Dejerine was born into a modest family in 1849, in Geneva, Switzerland. As a young man during the FrancoPrussian war, Dejerine cared for wounded soldiers in a Geneva hospital. After earning degrees in biology and comparative anatomy from the Academie de Geneve, he departed for Paris in 1871 to pursue medical studies. Under the mentorship of Edme Felix Alfred Vulpian at Hospital de la Pitie, Dejerine published numerous papers on pathologies of the peripheral nervous system. In 1879, after completing his dissertation on acute ascending paralysis, Dejerine received a Doctor of Medicine degree from the Faculty of Medicine of Paris. In 1888, Dejerine found both a partner and collaborator when he wed American Augusta Marie Klumpke, the first female medical intern in Paris. Dejerine’s hard-working reputation preceded him, and upon completion of his degree, he was offered the Head of Clinic position under Professor Alfred Hardy at the Hospital Bicetre in Paris. He remained in this position until 1886 when he was nominated Professor Agre´ge´. Shortly following his nomination Dejerine learned that Jean Martin

Charcot was planning to block his candidacy; Dejerine confronted Charcot and demanded an explanation. After a heated discussion, Jean Martin Charcot conceded and promised Dejerine his vote. Dejrine was appointed Professor Agre´ge´ and Chief Consultant for Hospital Bicetre in 1887. During his 8-year tenure he began his seminal work on cerebral localization, outlining the somatotopy of pyramidal tracks, ascending sensory tracts, and thalamo-cortical connections. Dejerine also published widely on acquired disorders of spoken and written language. His first publication on writing disorders in aphasia patients (1891) provided an outline of the neural architecture necessary for reading, writing, and pronunciation. He went on to provide detailed clinical and organic descriptions of progressive aphasia and pure alexia. Dejerine was the first to demonstrate that pure alexia resulted from the disconnection of the angular gyrus from both visual cortices. In 1895, Dejerine joined the faculty of the famed Salpetriere as a Professor of Neurology. In the years to follow, his collaborations yielded numerous publications and clinical descriptions of disorders of the nervous system, including peripheral and central ataxia, sensory parietal syndrome, and thalamic syndrome. His body of work culminated in the publication of Anatomie des centres nerveux in 1895 and Semiologie des affections du systeme nerveux in 1914. Akin to many of the French neurologists before him, Dejerine developed an interest in the therapeutic treatment of mental disorders. Dejerine believed that rational and theoretical aspects of psychotherapy are secondary to the emotional elements; he emphasized nonspecific contributors to therapeutic change such as support, empathy, and understanding. In 1915, he published. The psychoneuroses and their treatment by psychotherapy. In addition to his prolific research career, Dejerine served as a founding member of the French Neurological Society. He also was actively involved with the Academie de Medicine and Societe de Biologie, which elected him Vice-President in 1895. Dejerine’s final contributions came during the First World War when he worked endlessly to care for the neurologically wounded in a military hospital. After many years of service, Dejerine died of uremia in 1917 at the age of 67.

Cross References ▶ Agraphia ▶ Alexia ▶ Dejerine–Roussy Syndrome

Delayed Response Tasks

References and Readings Bassetti, C. L., & Jagella, E. C. (2006). Joseph Jules Dejerine (1849–1917). Journal of Neurology, 253, 823–824. Bub, D. N., Arguin, M., & Roch Lecours, A. (1993). Jules Dejerine and his interpretation of pure alexia. Brain and Language, 45, 531–559. Dejerine, J. J. (1891). Sur un cas de cecite verbale avec agraphie suivi d’autopsie. Memoires Societe Biologique, 3, 197–201. Dejerine, J. J. (1914). Semiologie des affections du systeme nerveux. Paris: Masson. Dejerine, J. J., & Dejerine-Klumpke, A. M. (1895). Anatomie des centres nerveux. Paris: Masson. Dejerine, J. J., & Gauckler, E. (1915). The psychoneuroses and their treatment by psychotherapy (S. E. Jelliffe, Trans.). Oxford, England: Lippincott. Dejerine, J. J., & Roussy, G. (1906). Le syndrome thalamique. Review of Neurology (Paris), 12, 521–532. Gauckler, E. (1922). Le Professeur Dejerine (1849–1917). Paris: Masson. Miller, M. (1967). Three great neurologists. Proceedings of the Royal Society of Medicine, 60, 399–405. Schurch, B., & Dollfus, P. (1998). The ‘Dejerines’: An historical review and homage to two pioneers in the field of neurology and their contribution to the understanding of spinal cord pathology. Spinal Cord, 36, 78–86.

Dejerine–Roussy Syndrome J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

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Delayed Alternation ▶ Delayed Response Tasks

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Delayed Nonmatching-to-Sample ▶ Delayed Response Tasks

Delayed Object Alternation ▶ Delayed Response Tasks

Synonyms Thalamic pain syndrome

Delayed Prompting ▶ Errorless Learning

Definition Contralateral loss of or diminished somatosensory sensation, particularly proprioception or position sense following a thalamic lesion. The syndrome most commonly results from a vascular lesion affecting the ventral posterior nucleus. Most notable in this syndrome is the concomitant presence of diffuse, lingering pain which may be produced by relatively minor and even noncutaneous stimuli, while the response to actual painful-type stimuli may be diminished.

Delayed Response Tasks PAUL M ALLOY Alpert Medical School of Brown University Providence, RI, USA

Synonyms Cross References ▶ Thalamic Pain Syndrome

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Delayed alternation; Delayed matching-to-sample; Delayed nonmatching-to-sample; Delayed object alternation; Delayed spatial alternation

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Delayed Response Tasks

Description Delayed response (DR) tasks represent the classic paradigm for studying working memory in both animals and humans. Working memory refers to the temporary storage of information in a ‘‘buffer’’ for rehearsal and organization of new information, and frontal systems are thought to subserve this process. Frontal working-memory systems are also involved in the active retrieval of previously stored information through temporal tagging and organization of retrieval strategies. The hippocampus and other posterior brain regions, in contrast, are thought to be more involved in memory consolidation and permanent storage. Studies utilizing DR tasks have been used to elucidate the neuroanatomical organization of memory systems in the brain through behavioral, lesion, and electrophysiological studies. In DR tasks, information is presented to a subject, then withdrawn, and a relatively brief delay interval ensues. The subject is then presented with a choice of two or more response alternatives and is required to choose the one presented previously to obtain reinforcement. While intact animals can perform these tasks easily, animals with frontal lesions often have trouble performing correctly with delay intervals as brief as 5–20 s. DR tasks must be made more difficult to detect deficits in human patients. This can be accomplished by increasing the delay interval, interpolating a cognitive distraction task during the delay, and/or increasing the number of choices after the delay. There are several variations of DR tasks, which can be divided into spatial- and feature-oriented tasks. In general, spatial DR tasks are thought to depend on more dorsal frontal systems, whereas feature DR tasks are more dependent on ventral frontal systems. Delayed spatial alternation (DA) is the simplest spatial DR task. In a typical animal DA experiment, the subject is presented with two food dishes, and a food reward is placed in one of the dishes in view of the animal. The dishes are covered and hidden for a delay period and then the animal is required to choose the correct dish to obtain the food reward. After a correct response on one side, the correct side is switched (alternation), another delay interval ensues and the subject must choose the other side to obtain the reward. Frontal animals have trouble making the switch to the new side, instead tending to perseverate on the previously rewarded side. This mirrors the tendency of frontally lesioned patients to perseverate on habitual or recently reinforced behavior, even when it is inappropriate to current environmental circumstances. Spatial DR tasks can be made more complex by adding multiple spatial choice locations. Animals can be restrained and trained to

fixate on a central location during the delay interval, to prevent the use of body posture (rather than working memory), and to cue them for the correct response. In feature-oriented DR tasks, the location of the reward is randomized, and instead some feature of the response choice is the critical cue to correct responding. For example, a cube-shaped cover may be placed over the food dish containing the reward, and a dome-shaped cover may be placed over the other dish. After the delay, the subject must choose the cube-shaped dish, regardless of whether it is on the left or right. That is, the features of the choice, rather than spatial location, are the cue for correct responding. The delayed object alternation (DOA) task is a two-choice task in which the monkey needs to correctly select an object that alternates between trials. For delayed matching-to-sample (DMTS) tasks, the subject is presented with a stimulus array with multiple alternatives (e.g., squares containing different visual patterns), and must choose the correct pattern after the delay. Delayed nonmatching-to-sample (DNMTS) tasks are similar to DMTS paradigms, except that the subject is required to choose the stimulus that does not match the stimulus that was presented before the recall delay.

Historical Background Most early DR studies were conducted using the Wisconsin General Test Apparatus (WGTA). In the WGTA, a monkey sat in a cage in front of a tray that contained recessed food wells. The experimenter baited one of the wells with food, covered it, and then lowered a screen to block the wells from the monkey’s view. After the delay interval, the screen was raised and the monkey made a choice of food wells. The WGTA was used for both spatialand feature-oriented DR tasks. Currently, most animal and human experiments are conducted using computerized response panels and automated reinforcement devices. These devices provide greater precision in timing of responses, and can accommodate a much wider variety of stimuli and response devices. Computerized DR tasks are also amenable to data collection in functional imaging studies, although some adaptation of the tasks is typically necessary to limit subject movement.

Clinical Uses Delayed response deficits have been demonstrated in a variety of diseases affecting frontal systems, including

Delirium

traumatic brain injury, anterior communicating artery aneurysm, Korsakoff syndrome, degenerative disorders (Oscar-Berman, McNamara, & Freedman, 1991), and schizophrenia. Many studies have investigated the effects of pharmacological challenges on DR tasks in order to study the role of various neurotransmitters in working memory. However, most versions of DR tasks remain experimental methods for cognitive neuroscience experiments. There are a few standardized DR tasks suitable for clinical use. The CANTAB (Cambridge Neuropsychological Test Automated Battery) consists of computerized tests of memory, attention, and executive function. It includes a DMTS task for the assessment of working memory.

Cross References ▶ CANTAB ▶ Examiner ▶ Working Memory

References and Readings Freedman, M., & Oscar-Berman, M. (1986). Bilateral frontal lobe disease and selective delayed response deficits in humans. Behavioral Neuroscience, 100, 433–435. Fuster, J. M. (1989). The prefrontal cortex (2nd ed., p. 255). New York: Raven. Goldman-Rakic, P. S. (1987). Circuitry of the prefrontal cortex and the regulation of behavior by representational knowledge. In F. Plum, & V. Mountcastle (Eds.), Handbook of physiology (pp. 373–417). Bethesda: American Physiological Society. Goldman-Rakic, P. S. (1995). Cellular basis of working memory. Neuron, 14, 477–485 Oscar-Berman, M., McNamara, P., & Freedman, M. (1991). Delayedresponse tasks: Parallels between experimental ablation studies and findings in patients with frontal lesions. In H. S. Levin, H. M. Eisenberg, & A. L. Benton (Eds.), Frontal lobe function and dysfunction. New York: Oxford University Press. Rodriguez, J. S., & Paule, M. G. (2009). Working memory delayed response tasks in monkeys. In J. J. Buccafusco (Ed.), Methods of behavior analysis in neuroscience. Boca Raton: CRC Press/Taylor & Francis Group. http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi? book=frbehav&part=ch12 Rosenkilde, C. E. (1979). Functional heterogeneity of the prefrontal cortex in the monkey. Behavioral and Neural Biology, 25, 301–345.

Delayed Spatial Alternation ▶ Delayed Response Tasks

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Delirium R OBERT J. B OLAND The Warren Alpert School of Medicine at Brown University Providence, RI, USA

Synonyms Acute brain failure; Acute confusional state; Acute encephalopathy; Intensive care unit (ICU) psychosis; Toxic-metabolic encephalopathy

Short Description or Definition ‘‘Delirium’’ describes a wide variety of confusional states. Generally, it includes several features that are central to the disorder: it is acute, has a fluctuating course, and has a gross effect on brain functioning. Most typically, delirium affects a person’s level of consciousness; that is, the person’s ability to focus, sustain, and shift attention. In addition, it affects a person’s level of arousal, and a delirious person’s behavior can range from lethargic to agitated; often it will fluctuate between the two states within relatively brief intervals of time (e.g., several hours). In addition, it can affect any area of cognitive functioning: delirious persons are confused and disoriented (i.e., unaware of their surroundings). Their thoughts are often disorganized, and may include loose associations, circumstantial or tangential thought, perseverations, or thought blocking. Delirious persons may have disturbances of perceptions and thought content and they may experience hallucinations or delusions. Higher intellectual functions such as the ability to make decisions are usually impaired because of disorders in these more basic cognitive functions.

Categorization Delirium is not a disorder, per se, but a syndrome describing the neuropsychiatric expression of gross dysfunctions in brain physiology, and as such, it is quite heterogeneous. Typically, delirium is categorized based on a person’s level of arousal and is usually divided into hyperactive and hypoactive types. This distinction dates back to the ancient Greeks, who called the two types as ‘‘phrenitis’’ and ‘‘lethargus.’’ The delirium associated with complicated alcohol withdrawal (‘‘delirium tremens’’) is sometimes included as

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an example of a hyperactive delirium; however, the condition, along with the withdrawal caused by other sedatives, may represent a distinct condition with unique pathophysiology. Hypoactive delirium, although also common is under recognized given its subtler phenomenology, and it is sometimes called ‘‘quiet delirium.’’ That the hyperactive and hypoactive states can co-occur in a mixed state (which may be more common than either state alone) lends support to the idea that the two types are not distinct entities, but rather two expressions of the same syndrome. It should be noted that in the neurology literature, the word ‘‘delirium’’ is sometimes used to describe only the hyperactive type, and the hypoactive category is referred to terms such as ‘‘acute encephalopathy.’’ However, as discussed above, there is little evidence to suggest that the two categories represent meaningful distinctions, and in the psychiatric literature, ‘‘delirium’’ refers to both the hyperactive and hypoactive types. Delirium is also occasionally categorized by the site in which it takes place: thus, the term ‘‘Intensive Care Unit (ICU) Psychosis’’ is sometimes used in the medical literature to describe a particularly troublesome example of a hyperactive delirium seen in the intensive care setting. Again, such categorizations are of little heuristic or diagnostic value and there is no reason to suspect that the delirium occurring in that setting differs from that seen elsewhere.

Epidemiology Delirium is likely the most common syndrome affecting patients in general hospitals. Accurate prevalence figures are difficult to obtain given the methodological limitations of the various investigations into the disorder. Epidemiological and clinical studies suggest that 10–20% of all hospitalized adults, 30–40% of elderly hospitalized patients and up to 80% of patients in intensive care units experience delirium at some time during their hospitalization. Delirium is most likely underreported in any setting; the hypoactive type in particular is often not noticed by clinical teams, or misdiagnosed as another disorder. The development of validated instruments designed to detect delirium (see below) has improved the detection of delirium.

Natural History, Prognostic Factors, Outcomes By definition, delirium is an acute syndrome, meaning that it comes on rapidly, often in the course of hours to

days. It may be preceded by a subsyndromal period of more subtle cognitive deficits. The level of confusion and degree of arousal generally fluctuates throughout the day. The following vignette illustrates a typical presentation of delirium: An 82-year-old man was transferred from a nursing home to a general medical hospital for a ‘‘change in mental status.’’ Usually quiet and pleasant, he became increasingly suspicious and annoyed at the nursing home staff. He had a history of Alzheimer’s disease, which was considered moderate in severity. On admission to the hospital, he was lethargic and slept for most of the morning. Later in the day, he was seen by the attending physician; at that time, he was awake, alert, and only mildly confused and the physician questioned the appropriateness of the patient’s admission to the hospital. However, later that evening the patient became argumentative with staff, demanding to leave the hospital. He seemed to believe he was in fact ‘‘locked up’’ in a prison, and worried that people were trying to break in through his window in order to harm him. He was given haloperidol, which helped calm him; however, he continued to be paranoid and confused. On medical evaluation, a urinary tract infection was discovered, and appropriate antibiotics were initiated. His periods of belligerence slowly decreased over the next several days, and within a week, although still more confused than normal, he was able to return to his nursing home. This vignette illustrates several typical aspects of delirium, most notably the fluctuating course, which can confuse the treating team. The patient’s dementia can be seen as a predisposing factor, while the infection was the likely precipitant. Although treating the precipitants is the mainstay of treatment, it is typical that cognitive improvement lags behind treatment of the underlying medical condition. The prognosis and outcome of delirium is understandably variable, and dependent on the actual precipitants. Although much literature – including the DSM-IV-Text Revision (DSM-IV-TR) (American Psychiatric Association, 2000) – describes delirium as a transient and reversible state, more recent work suggests that there are often permanent cognitive deficits following an episode of delirium. The syndrome is a poor prognostic indicator that is associated with high morbidity and mortality. That this is so may simply reflect the many serious illnesses than can precipitate a delirium, however some investigators have suggested that the syndrome itself can have a deleterious effect on the course of an illness, perhaps through the damaging effects of the abnormal neurochemical activity that occurs during a delirious episode.

Delirium

Neuropsychology and Psychology of Delirium Delirium is a neuropsychological syndrome that can affect any area of cognitive functioning. Its effect on the most primitive of functions (attention, concentration, and arousal) clearly has effects on more complex functions such as abstract thinking, judgment, and insight. Decision-making capacity is generally impaired, and patients (and their families) are encouraged to postpone important decisions until the delirium can be treated. Many delirious patients, once recovered, do not remember the episode. In one study, of those who were able to recall it, at least half experienced their delirious episode as highly distressing (Breitbart, Gibson, & Tremblay, 2002). This is true even for those patients who appeared calm or sedated at the time they were delirious.

Evaluation The provocative behavioral effects of delirium (such as agitated and violent behavior) usually bring patients to clinical attention. When these symptoms are lacking, delirium is often missed; thus, the most important part of the evaluation is maintaining a high level of suspicion for delirium in the clinical setting. Delirium should be suspected in individuals who have factors that predispose them to the syndrome. These include preexisting cognitive disorders, such as any dementia, substance use disorders (particularly alcoholism), neurological disorders, and the presence of any collection of chronic medical disorders and/or the use of multiple therapeutic medications. Psychiatric disorders alone, apart from the dementias and substance use disorders do not significantly increase the risk of a delirium. When delirium is suspected, the evaluation should focus on the cognitive examination. Evidence of impaired attention, disorientation to date and place, and gross confusion all make delirium a likely explanation. General cognitive testing such as that used for the Mini-Mental State Exam can show evidence of the gross cognitive impairment found in delirious patients; however, the test is not specific for delirium. It can be useful to include tasks that focus on the attentional deficits seen in delirium, and such as Clock Drawing and the Trail Making Test. These tests, although limited in scope have the advantage of being simple to use and require little training. A number of tests specifically designed to detect delirium have been developed; these require specific training in their use, and include such tests as the Delirium

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Rating Scale (Trzepacz, Mittal, Torres, Kanary, Norton, & Jimerson, 2001), the Memorial Delirium Assessment Scale (Breitbart et al., 1997), and the Confusion Assessment Method (Inouye, VanDyck, Alessi, Balkin, Siegal, & Horwitz, 1990). Many disorders can mimic the symptoms of delirium, and the differential diagnosis can be difficult in the acute setting. One of the more challenging distinctions is between delirium and the dementias. Although both are defined by their cognitive effects, patients with dementia generally have an intact level of consciousness, and the course of dementia (particularly the common ones such as Alzheimer’s disease) is more chronic and less fluctuating. However, the two disorders frequently co-occur, and comorbid delirium – including subsyndromal delirium – should be suspected in cases where patients with dementia have perceptual disturbances or sudden changes in their mental state (Meagher & Trzepacz, 2007). Both delirious and psychotic patients can have hallucinations and delusions; however, patients with primary psychotic disorders such as schizophrenia tend to experience their symptoms in the setting of a clear sensorium: they feel alert, awake, and aware of their surroundings. Schizophrenic patients generally lack the profound confusion and general cognitive deficits seen with delirious patients. In addition, the delusions and hallucinations experienced in schizophrenia tend to be more elaborate and fixed than with delirious patients. Delirium, particularly hypoactive delirium, can be mistaken for depression, and the psychosomatic medicine literature suggests that this misdiagnosis is common in the general hospital setting. Although the two share some features such as impairments in concentration and attention, depression has a more gradual onset and a less fluctuating course. Delirious patients may look sad at times, even tearful, however their mood tends to be more labile than with depressed patients. Similarly, mania, particularly severe mania can mimic the confusion and agitation associated with delirium. Again, the course can help distinguish the two, with mania usually having a history of a more progressive course leading to the current symptoms. In addition, although severely manic patients can be confused, they tend not to have the gross cognitive changes seen with delirium. In cases where the clinical exam cannot distinguish between delirium and other psychiatric disorders, an electroencephalography (EEG) may help: the EEG shows diffuse slowing, whereas the EEG is normal in most other psychiatric disorders. Once convinced of the presence of a delirium, the major evaluative task becomes one of discovering the

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underlying etiology. Essentially, any process that can cause a disruption in the normal functioning of the brain can cause a delirium. The most common causes are infections and the toxic effects of drugs and other substances. There are a number of useful mnemonics meant to help one remember the potential precipitants: one popular mnemonic is the acronym ‘‘I WATCH DEATH’’ (a potent reminder of the morbidity associated with delirium): Infections Withdrawal (Alcohol and other substances) Acute Metabolic Trauma Central nervous system pathology Hypoxia Deficiencies (vitamin, other nutritional) Endocrinopathies Acute vascular disorders Toxins/drugs Heavy metals It is apt to consider these as ‘‘precipitants’’ than ‘‘causes.’’ In reality, the cause is usually multifactorial, combining predisposing factors with acute precipitants that upset an already tenuous cognitive balance. It can be argued that neither is a direct cause, but that the combination initiates a cascade of events in the patient’s brain. A number of experts have used the term ‘‘acute brain failure’’ to describe delirium: the term is meant to imply an analogy to heart failure, in which an already impaired organ can suddenly and rapidly malfunction after the onset of a seemingly minor insult.

Treatment The most important step in the treatment of delirium is recognition of the syndrome. Once recognized, treatment primarily focuses on discovering and treating the underlying precipitants, and the particular treatment is specific to the medical or neurological disorder. A number of behavioral strategies have been suggested to minimize the confusion of the delirious patient. These strategies include indirect environmental interventions, for example introducing lighting variations (or windows) into intensive care settings to help orient patients to the time of day, and the minimization of unnecessary noise. Objects from the patient’s home may be placed in a hospital room to increase the familiarity of the surroundings or family members may be encouraged to remain with the patient whenever possible. Direct behavioral interventions include frequent reorientation of the patient, minimization of restraints, use of sensory aids (eyeglasses, hearing aids)

simple exercises, and sleep hygiene protocols. Most of these behavioral strategies have limited support in the literature, and many have never been tested in the acute setting; however, they have anecdotal support, face validity, and are unlikely to harm the patient. There is some methodologically sound data to support behavioral interventions for the prevention of delirium in predisposed individuals (Inouye et al., 1999) as well as for the efficacy of staff education to recognize delirium early (Lundstrom, Edlund, Karlsson, Bra¨nnstro¨m, Bucht, & Gustafson, 2005). At times, symptomatic pharmacological treatment of delirium becomes necessary. Such treatment is usually initiated when the behavioral manifestations of delirium, particularly aggressive or violent behaviors, make it impractical or dangerous to wait for the natural improvement that will follow the successful treatment of the underlying precipitants. Alternately, no clear underlying disorder may be found, and yet palliative care may be indicated to relieve the delirious patient’s distress. Most commonly, sedating medications are given to calm the delirious patient. Most of the evidence for such treatment is anecdotal or derived from uncontrolled studies. Historically, the most common medications used in the acute setting were narcotics, benzodiazepines, and antipsychotics. Narcotics are rarely used now except in cases where pain or dyspnea is thought to play a role in the patient’s general distress. Benzodiazepines are still commonly used, however should not be considered a ‘‘first-line’’ medication given their capacity to confuse patients further. Although this side effect is sometimes labeled as a ‘‘paradoxical’’ effect of the drug, it is in fact a common side effect of all sedative-hypnotic medications. Antipsychotics are most commonly used, both ‘‘typical’’ antipsychotics such as haloperidol, and the ‘‘atypical’’ agents such as risperidone, olanzapine, quetiapine, and newer agents. All antipsychotics seem to carry the benefit of having a calming effect without causing the level of sedation and confusion seen with benzodiazepines. It is thought that the antipsychotic effect of these agents can help organize a patient’s thoughts; this latter supposition is not as well supported, and seems counter to the understanding of the pharmacodynamic effects of a group of drugs that often take weeks to treat psychosis. Anecdotally, however, patients do seem less confused and psychotic after only a short term of antipsychotics; it may simply be that patients can organize their thoughts better once they are calmer. Many of the side effects that limit the use of antipsychotics, such as parkinsonian symptoms or tardive dyskinesia, are less relevant in the acute care setting. When using antipsychotics, once should watch for the rare but

Delirium

serious adverse effects that could occur, such as an acute dystonia, cardiac dysfunction, or the neuroleptic malignant syndrome; of these, the latter two are life threatening and require immediate discontinuation of the offending agent, close monitoring, and intense general medical support. Other side effects, such as akathisia can be difficult to distinguish from the agitation associated with delirium, but should be suspected in cases where the addition of an antipsychotic seems to make the patient more restless. Many antipsychotics are anticholinergic and thus can cause confusion, and the agents with a higher anticholinergic profile should be avoided; these included many of the lower potency antipsychotics such as chlorpromazine and thioridazine. Among the newer agents, clozapine and olanzapine are the most anticholinergic. Despite these concerns, in the majority of cases antipsychotics appear to be a safe and at least moderately effective approach. Despite the reported safety of these agents in the treatment of delirium, concern has been raised in the wake of the US Food and Drug Administration’s (FDA, 2005) warnings about the increased risk of mortality found in elderly patients with dementia who are treated with antipsychotics. The debate on the appropriate risk and benefit balance continues, and specific data on the safety in elderly patients with delirium is lacking. Until more guidance is available, delirious patients who are elderly, or who have predisposing cardiac conditions should be monitored closely if an antipsychotic is used. The particular agent to choose is largely a clinical decision based on an assessment of likely side effects and any history of prior response to a particular drug. Both the American Psychiatric Association (1999) and the Society of Critical Care Medicine (Pandharipande, Jackson, & Ely, 2005) recommend the use of haloperidol for the treatment of delirium. Newer agents are likely to be helpful, but currently lack sufficient data to support any particular agent. Available routes of administration can be important in choosing an agent as well: haloperidol is available in a short-acting injectable formulation as is olanzapine; most of the other agents are not.

Cross References ▶ Akathisia ▶ Alzheimer’s Disease ▶ Anticholinergic ▶ Antipsychotics ▶ Arousal ▶ Benzodiazepines ▶ Capacity

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▶ Chlorpromazine ▶ Clock Drawing ▶ Dementia ▶ Dystonia ▶ Electroencephalography ▶ Haloperidol ▶ Metabolic Encephalopathy ▶ Mini-Mental State Exam ▶ Olanzapine ▶ Parkinsonism ▶ Quetiapine ▶ Risperidone ▶ Schizotypal Personality Disorder ▶ Tardive Dyskinesia ▶ Thioridazine ▶ Trail Making Test

References and Readings American Psychiatric Association (1999). Practice guideline for the treatment of patients with delirium. American Journal of Psychiatry, 156 (Supplement), 1–20. American Psychiatric Association (2000). Diagnostic and statistical manual of mental disorders, (4th edn., Text Revision). Washington, DC: American Psychiatric Association. Breitbart, W., Gibson, C., & Tremblay, A. (2002). The delirium experience: Delirium recall and delirium-related distress in hospitalized patients with cancer, their spouses/caregivers, and their nurses. Psychosomatics, 43, 183–194. Breitbart, W., Rosenfeld, B., Roth, A., Smith, M. J., Cohen, K., & Passik, S. (1997). The memorial delirium assessment scale. Journal of Pain and Symptom Management, 13, 128–137. Inouye, S. K., Bogardus, S. T., Charpentier, P. A., et al. (1999). A multicomponent intervention to prevent delirium in hospitalized older patients. New England Journal of Medicine, 340, 669–676. Inouye S. K., VanDyck, C. H., Alessi, C. A., Balkin, S., Siegal, A. P., & Horwitz, R. I. (1990). Clarifying confusion: The confusion assessment method: a new method for detection of delirium. Annals of Internal Medicine, 113, 941–948. Lundstrom, M., Edlund, A., Karlsson, S., Bra¨nnstro¨m, B., Bucht, G., & Gustafson Y. (2005). A multifactorial intervention program reduces the duration of delirium, length of hospitalization, and mortality in delirious patients. Journal of the American Geriatrics Society, 53, 622–628. Meagher, D., & Trzepacz, P. T. (2007). Phenomenological distinctions needed in DSM-V: Delirium, subsyndromal delirium, and dementias. The Journal of Neuropsychiatry and Clinical Neurosciences, 19, 468–470. Pandharipande, P., Jackson, J., & Ely, E. W. (2005). Delirium: Acute cognitive dysfunction in the critically ill. Current Opinion in Critical Care, 11, 360–368. Trzepacz, P. T., Mittal, D., Torres, R., Kanary, K., Norton, J., & Jimerson, N. (2001). Validation of delirium rating scale revised-98: Comparison to the delirium rating scale and cognitive test for delirium. The Journal of Neuropsychiatry and Clinical Neurosciences, 13, 229–242.

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U.S. Food and Drug Administration. (2005). FDA public health advisory: Deaths with antipsychotics in elderly patients with behavioral disturbances. Rockville, MD: U.S. Food and Drug Administration.

Delirium Tremens ▶ Alcoholic Brain Syndrome

Delis–Kaplan Executive Functioning System E RIC M. F INE 1, D EAN C. D ELIS 2 1 San Diego, CA, USA 2 University of California, San Diego, School of Medicine La Jolla, CA, USA

Synonyms D-KEFS

Description The Delis–Kaplan Executive Function system (D-KEFS) is the first battery of tests designed exclusively for the assessment of executive functions in children and adults that has been normed on a large national sample representative of the demographic characteristics of the U.S. population (Delis, Kaplan, & Kramer, 2001a). Key objectives in the development of D-KEFS measures were to provide psychologists a comprehensive tool for assessing a wide array of executive functions, including cognitive flexibility, problem-solving, conceptual reasoning, inhibition, multitasking, and nonverbal and verbal creativity. The D-KEFS can be distinguished from most other executive-function tests by its embrace of the ‘‘cognitive process approach’’ in which multiple measures are generated to isolate the mechanism of a patient’s poor score on a particular task (Delis, Kramer, Kaplan, & Ober, 2000; Kaplan, Fein, Kramer, Delis, & Morris, 1999). With the exception of the Wisconsin Card Sorting Test (Heaton, Chelune, Talley, Kay, & Curtiss, 1993), the majority of existing clinical measures of higher-level cognitive functions yield a single achievement score for each task (e.g., the Category Test and the traditional Trail-Making Test).

The single-score method is particularly problematic for executive-function tests because these tasks typically require both more fundamental cognitive skills (e.g., ▶ language, ▶ visuoperception, and ▶ fine motor skills) and higher-level executive functions for successful performance. The D-KEFS was developed to provide measures that assess (a) both fundamental and higher-level skills in order to evaluate if a patient’s poor performance on a task is due to an executive-function deficit or to an impairment in a more fundamental cognitive skill and (b) strategies and error types in order to more precisely characterize the nature of the executive dysfunction. Another objective of the D-KEFS was the development of test-design features that enhanced the instruments’ sensitivity to mild executive dysfunction or mild frontallobe injury. For instance, switching conditions were added to several traditional executive-function tasks that previously did not require cognitive flexibility. As an example, in addition to the three standard ‘‘Stroop’’ conditions (color naming, word reading, and inhibition; ▶ Stroop Color Word Test, Adult), a new ‘‘Stroop’’ condition was developed for the D-KEFS Color-Word Interference Test that requires examinees to switch back and forth between naming the dissonant ink color and reading the dissonant word. Cognitive switching, or the ability to abandon a previous response in order to generate a novel or complimentary response (Delis et al., 2001a), is considered one of the hallmarks of executive functions and is particularly dependent on the integrity of frontal-lobe functioning. In addition, certain D-KEFS tests were designed to contain ‘‘capture stimuli’’ that pull for concrete or stimulusbound responding in patients who are vulnerable to this tendency due to frontal-lobe injury.

Descriptions of the D-KEFS Tests The D-KEFS consists of nine tests measuring a wide spectrum of verbal and nonverbal executive functions. These tests were either (a) modifications of existing clinical tests of executive functions (e.g., Stroop Color-Word Interference Test and Trail-Making Test) in order to increase their sensitivity to frontal-lobe dysfunction, (b) or modifications of tasks used in past experimental studies of executive functions but that had not been developed into standardized clinical instruments, or (c) ‘‘relatively new’’ tests that Delis and colleagues developed.

D-KEFS Trail-Making Test The D-KEFS Trail-Making Test is a modification of the traditional Trail-Making Test that was first developed by

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Partington (Partington & Leiter, 1949), modified by army psychologists, and then included in the Halstead–Reitan battery. It is one of the most commonly used neuropsychological measures for assessing executive functions. While the traditional version offers two conditions – Part A (number sequencing) and Part B (number–letter switching) – the D-KEFS version offers five conditions: visual scanning, motor speed, number sequencing, letter sequencing, and number–letter switching. The four baseline conditions enable the clinician to assess empirically if a patient’s poor performance on the switching condition is due to a deficit in cognitive flexibility or to impairment in one or more of the underlying component skills needed to perform the switching task (i.e., motor speed, visual scanning, number sequencing, or letter sequencing).

D-KEFS Verbal Fluency Test Verbal fluency tasks are among the most commonly administered neuropsychological measures (▶ Verbal Fluency). The D-KEFS Verbal Fluency Test is a timed measure that includes (a) a letter fluency condition that requires examinees to generate as many words as possible that start with a particular letter, (b) a category fluency condition that requires examinees to generate as many words as possible from designated semantic categories, and (c) a category switching condition that requires examinees to alternate between generating words from two different semantic categories. In general, patients with predominately frontal-lobe damage tend to have more difficulty on the letter fluency task relative to the category fluency task, whereas patients with early Alzheimer’s disease often show the opposite pattern due to a breakdown in semantic knowledge (Delis et al., 2001b).

D-KEFS Design Fluency Test Design fluency measures, developed as a nonverbal analog of verbal fluency measures (Jones-Gotman & Milner, 1977; ▶ Design Fluency), require examinees to produce as many different designs as possible that meet a particular criterion (e.g., the designs must always contain four lines) within a time interval. The D-KEFS Design Fluency Test is a modified version of traditional procedures. For each condition, the examinee is presented rows of boxes that contain an array of dots and is instructed to draw as many different designs as possible using only four straight lines. The three conditions of the test vary in difficulty, with Condition 1 (filled dots) assessing basic design fluency, Condition 2 (empty dots only) requiring examinees to inhibit connecting the filled dots while connecting only the empty dots, and Condition 3 (switching) requiring examinees to switch between connecting filled

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and empty dots. The switching condition, which is novel to the D-KEFS, has been found to be particularly sensitive to frontal-lobe dysfunction (e.g., Cato, Delis, Aildskov, & Bigler, 2004; Kramer et al., 2007).

D-KEFS Color-Word Interference Test The D-KEFS Color-Word Interference Test is a variant of the Stroop Test (Stroop, 1935; Stroop Color Word Test, Adult), a commonly used measure of response inhibition. The D-KEFS version includes two baseline conditions – naming of color squares (Condition 1) and reading of color words printed in black ink (Condition 2) – and the traditional interference condition (Condition 3) in which the examinee must inhibit reading the words in order to name the incongruent ink colors in which these words are printed in. The D-KEFS version differs from other Stroop tasks by the inclusion of a fourth condition that requires the examinee to switch back and forth between naming the dissonant ink colors and reading the conflicting words. This condition has been shown to be particularly sensitive to frontal-lobe dysfunction (e.g., Cato et al., 2004).

D-KEFS Sorting Test The D-KEFS Sorting Test (formerly called the California Card Sorting Test; Delis, Squire, Bihrle, & Massman, 1992) was designed to provide a standardized measure of conceptual–reasoning skills. Specifically, Condition 1, free sorting, requires examinees to sort cards according to eight possible target rules, including five primarily perceptual or nonverbal rules (e.g., straight versus curved outer edges), and three primarily verbal rules (e.g., clothing versus body parts). In Condition 2 (sort recognition), the examiner sorts the same sets of cards into two groups according to the eight target sorts and, after each sort, asks the examinee to identify and describe the correct rules used to generate the sort. Performance is evaluated both in terms of the total number of correct target concepts reflected in the examinee’s sorts, as well as the accuracy and level of abstraction of the examinee’s sort descriptions. This measure has been found to be sensitive to frontal-lobe dysfunction (e.g., Huey et al., 2009; Fine et al., 2009).

D-KEFS Twenty Questions Test The D-KEFS Twenty Questions Test is a modification of a popular, informal game played by children and adults, and assesses categorical processing, hypothesis testing, and concept formation. For this task, the examinee is presented with a stimulus page depicting pictures of 30 common objects. The examinee tries to ask the fewest number of yes/no questions in order to identify the unknown target

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object. Multiple process measures are scored, including level of abstract thinking, error types, and response strategies (e.g., verbal versus nonverbal). This test has been found to be sensitive to focal frontal lesions (Baldo, Delis, Wilkins, & Shimamura, 2004).

D-KEFS Word Context Test The D-KEFS Word Context Test is an adaptation of a test developed originally for studying how children acquire word meanings (Werner & Kaplan, 1952). The examinee is asked to discover the meaning of mystery words based on clues given in sentences. For each mystery word, the examinee is shown five sentences (clues) that assist with decoding the meaning of the words. This test is a measure of deductive reasoning and verbal abstract thinking and patients with focal frontal lesion exhibit deficits on this measure (Keil, Baldo, Kaplan, Kramer, & Delis, 2005).

D-KEFS Towers Test The D-KEFS Towers Test is a modified version of other tower tasks that have been used in experimental studies. The examinee is asked to build target towers by moving two to five disks of different sizes across three pegs in as few moves as possible while adhering to two rules: (a) move only one disk at a time and (b) never placing a larger disk over a smaller disk. The D-KEFS Tower Test assesses several aspects of executive functioning, including spatial planning, rule violations, and inhibition. This test has been found to be sensitive to focal frontal lesions (Yochim, Baldo, Kane, & Delis, 2009).

D-KEFS Proverb Test The D-KEFS Proverb Test was modeled after a proverb interpretation measure developed in the 1950s by Gorham (1956). For the D-KEFS version, an examinee is asked to interpret proverbs in two conditions: (a) free inquiry where the examinee generates his or her own interpretations of the proverbs and (b) multiple choice where the examinee selects the best interpretation from among four alternatives. This task measures metaphorical thinking and provides a means for comparing generation versus comprehension of abstract verbal information. This test has been found to be sensitive to frontal-lobe dysfunction (McDonald, Delis, Norman, Tecoma, & Iragui-Madozi, 2008)

Administration and Scoring Instructions for administration are given in the stimulus booklet or record form. The required materials, discontinued rules, time limits, and standardized prompts are

clearly displayed to the examiner in the stimulus booklet and the response form. Each D-KEFS test is a stand-alone instrument that can be administered individually or with other D-KEFS tests. For all of the ‘‘primary’’ measures and many of the ‘‘optional’’ measures, the raw scores are converted to scaled scores, with a mean of 10 and a standard deviation of 3, for each of the following 16 age groups: 8, 9, 10, 11, 12, 13, 14, 15, 16–19, 20–29, 30–39, 40–49, 50–59, 60–69, 70–79, and 80–89. Raw scores for several of the optional measures have limited ranges in normative (nonclinical) populations, and therefore they are corrected using cumulative percentile ranks for each of the 16 age groups. The D-KEFS scoring software is available, which automatically computes many of the scoring formulas and converts raw scores into standardized scores. Most traditional executive-function tests do not provide alternate forms, which can be problematic for repeat examinations. However, the D-KEFS provides alternate forms for three of the tests that are particularly prone to practice effects (i.e., Sorting Test, Twenty Questions Test, and Verbal Fluency Test).

Normative Sample The D-KEFS was standardized on a nationally representative, stratified sample of 1750 children, adolescents, and adults, aged 8–89 years. Stratification was based on age, sex, race/ethnicity, years of education, and geographic region. The 2000 U.S. census figures were used as target values for the composition of the D-KEFS normative sample. There were 75 –175 people in each of the 16 age groups. The D-KEFS sample was comprised of approximately equal proportions of men and women for most of the age groups; however, the older age groups had more women than men, which is consistent with the census data. The D-KEFS sample was divided into five major educational groups used by the U.S. Census: less than or equal to 8 years, 9–11 years, 12 years, 13–15 years, and greater than or equal to 16 years. For examinees between the ages of 8 and 19, the mean parental education was substituted.

Historical Background Most of the D-KEFS tests were developed in the late 1980s and early 1990s by Delis and colleagues, with the entire set of D-KEFS instruments assembled in 1994. The preliminary measures underwent a ‘‘try-out’’ study of approximately 300 normative subjects and 50 mixed neurological patients, and modifications were made to improve the reliability and validity of the measures.


Psychometric Data Reliability and Validity Evidence of the reliability and validity of the D-KEFS is discussed in detail in the technical manual (Delis, Kaplan, & Kramer, 2001b; also see, Delis, Kramer, Kaplan, & Holdnak, 2004; Homack, Lee, & Riccio, 2005). Internal consistency (split-half coefficients), test–retest reliability, standard errors of measurement, and confidence intervals were estimated for each D-KEFS test. The split-half reliability estimates varied across measure and across age group, and the majority of tests had moderate or better reliability estimates (Delis et al., 2001b; Homack et al., 2005). Similarly, test–retest reliability estimates varied considerably across task and age group, and most of the test–retest coefficients were adequate (Delis et al., 2001b; Homack et al., 2005). Alternate forms reliability was provided for the D-KEFS Verbal Fluency Test, Sorting Test, and Twenty Questions Test. Most of the measures possessed adequate alternate forms reliability, although the reliability coefficients for some measures from the Twenty Questions Test were relatively low (Delis et al., 2001b). The technical manual includes evidence of validity in the form of correlations between D-KEFS measures, between the D-KEFS and other tasks (i.e., California Verbal Learning Test-II (CVLT-II); Delis et al., 2000; and the Wisconsin Card Sorting Test; Heaton et al., 1993), and findings from clinical populations (see clinical uses below). Many of the validity studies of the D-KEFS have been published in refereed scientific journals rather than in the test manual, with scientific journals often requiring more rigorous methodology than the validity studies often reported in test manuals (Delis et al., 2004). In general, primary measures from the same test were more highly correlated, and the strength and association between variables varied considerably across the age groups. The correlations between optional (‘‘process’’) measures tended to be low, which was expected given that normative (nonclinical) populations often have reduced ranges of these scores (e.g., error measures are typically low in normative populations but significantly elevated in certain clinical populations). As evidence of convergent validity, the manual reports that the D-KEFS Sorting Test and the Wisconsin Card Sorting Test were moderately correlated in a small sample of participants (n = 23). In addition, a recent study by Floyd, McCormack, Ingram, Davis, Bergeron, and Hamilton (2006) demonstrated that particular clinical clusters from the Woodcock–Johnson-III Tests of Cognitive Abilities that draw upon aspects of executive functions were significantly correlated with

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performance on a number of D-KEFS measures. The test manual includes correlations between the CVLT-II (Delis et al., 2000), a measure of verbal learning and memory (▶ California Verbal Learning Test), and the D-KEFS in a sample of 292 adults. Correlations between the tests were for the most part not significant, which supports the discriminate validity of the D-KEFS (for additional discussion of validity studies of the D-KEFS, see Delis et al., 2001b; Delis et al., 2004; Homack et al., 2005).

Clinical Uses Consistent with a stated aim of the D-KEFS, numerous studies have demonstrated that the D-KEFS is sensitive to frontal-lobe dysfunction and executive-function deficits associated with a variety of neurological, developmental, and psychiatric disorders. A series of studies by Baldo and colleagues have demonstrated that patients with focal frontal-lobe injury have deficits on a variety of D-KEFS measures, including the D-KEFS Towers Test (Yochim et al., 2009), the D-KEFS Trail-Making Test (Yochim, Baldo, Nelson, & Delis, 2007), the Twenty Questions Test (Baldo et al., 2004), and the Word Context Test (Keil et al., 2005). Further evidence of the D-KEFS’ sensitivity to frontal-lobe dysfunction is found in a series of studies by McDonald and colleagues examining DKEFS performance in patients with frontal-lobe epilepsy. Results from these studies indicate that frontal-lobe epilepsy is associated with deficits on D-KEFS measures that emphasize cognitive switching (i.e., the number–letter switching from the Trail-Making Test, design fluency switching, inhibition/switching from the Color-Word Interference Test; McDonald, Delis, Norman, Tecoma, & Iragui, 2005a, b, c) and verbal abstraction (i.e., the Proverbs Test; McDonald, Delis, Kramer, Tecoma, & Iragui, 2008). In addition, recent studies utilizing quantitative magnetic resonance imaging (MRI) to elucidate the brain regions underlying performance on the D-KEFS have provided further support for the utility of particular D-KEFS measures (i.e., Design Fluency Test, Towers Test, and Sorting Test) in assessing frontal-lobe functions (see Kramer et al., 2007; Carey et al., 2008; Fine et al., 2009). In addition, the clinical utility of various D-KEFS subtests has been demonstrated in studies of clinical populations, including multiple sclerosis (Parmenter et al., 2007), cardiovascular disease (Jefferson, Poppas, Paul, & Cohen, 2007; Kramer, Reed, Mungas, Weiner, & Chui, 2002), autistic spectrum disorders (Kleinhans, Akshoomoff, & Delis, 2005), Parkinson’s disease (Beatty & Monson, 1990), schizophrenia (Lysaker, Whitney, & Davis, 2006;

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Kiang, Light, Prugh, Coulson, Braff, & Kutas, 2007), and in children with heavy prenatal alcohol exposure (Mattson, Goodman, Caine, Delis, & Riley, 1999; Schonfield, Mattson, Lang, Delis, & Riley, 2001).

Cross References ▶ California Verbal Learning Test (California Verbal Learning Test-II) ▶ Category Test ▶ Design Fluency Test ▶ Executive Functioning ▶ Frontal Lobe Syndrome ▶ Stroop Color Word Test, Adult ▶ Trail Making Test ▶ Verbal Fluency ▶ Wisconsin Card Sorting Test

References and Readings Baldo, J. V., Delis, D. C., Wilkins, D. P., & Shimamura, A. P. (2004). Is it bigger than a breadbox? Performance of patients with prefrontal lesions on a new executive function test. Archives of Clinical Neuropsychology, 19(3), 407–419. Beatty, W. W., & Monson, N. (1990). Problem solving in Parkinson’s disease: Comparison of performance on The Wisconsin and California card sorting tests. Journal of Geriatric Psychiatry & Neurology, 3, 163–171. Carey, C. L., Woods, S. P., Damon, J., Halabi, C., Dean, D., Delis, D. C. et al. (2008). Discriminant validity and neuroanatomical correlates of rule monitoring in frontotemporal dementia in Alzheimers disease. Neuropsychologia, 46(4), 1081–1087. Cato, M. A., Delis, D. C., Aildskov, T. J., & Bigler, E. (2004). Assessing the elusive cognitive deficits associated with ventromedial prefrontal damage: A case of a modern-day Phineas Gage. Journal of International Neuropsychological Society, 10(3), 453–465. Delis, D. C., Squire, L. R., Bihrle, A., & Massman, P. (1992). Componential analysis of problem-solving ability: Performance of patients with frontal lobe damage and amnesic patients on a new sorting test. Neuropsychologia, 30, 683–697. Delis, D. C., Kramer, J., Kaplan, E., & Ober, B. A. (2000). California verbal learning test - Second Edition. San Antonio, TX: The Psychological Corporation. Delis, D. C., Kaplan, E., & Kramer, J. H. (2001a). Delis-Kaplan executive function system: Examiner’s manual. San Antonio, TX: The Psychological Corporation. Delis, D. C., Kaplan, E., & Kramer, J. H. (2001b). Delis-Kaplan executive function system: Technical manual. San Antonio, TX: The Psychological Corporation. Delis, D. C., Kramer, J. H., Kaplan, E., & Holdnak, J. (2004). Reliability and validity of the Delis-Kaplan executive function system: An update. Journal of the International Neuropsychological Society, 10, 301–303. Fine, E. M., Delis, D. C., Dean, D., Beckman, V., Miller, B. L., Rosen, H. J., & Kramer, J. H. (2009). Left frontal lobe contributions to concept formation: A quantitative MRI study of performance on the Delis-

Kaplan executive function system sorting test. Journal of Clinical and Experimental Neuropsychology, 31(5), 624–631. Floyd, R. G., McCormack, A. C., Ingram, E. L., Davis, A. E., Bergeron, R., & Hamilton, G. (2006). Relations between the Woodcock-Johnson III clinical clusters and measures of executive functions from the Delis-Kaplan executive function system. Journal of Psychoeducational Assessment, 24, 303–317. Gorham, D. R. (1956). Use of the proverbs test for differentiating schizophrenics from normals. Journal of Consulting Psychology, 20(6), 435–440. Heaton, R. K., Chelune, G. J., Talley, J. L., Kay, G. G., & Curtiss, G. (1993). Wisconsin card sorting test manual - Revised and expanded. Odessa, FL: Psychological Assessment Resources. Homack, S., Lee, D., & Riccio, C. A. (2005). Delis-Kaplan executive function system (test review). Journal of Clinical and Experimental Neuropsychology, 27, 599–609. Huey, E. D., Goveia, E. N., Paviol, S., Pardini, M., Krueger, F., Zamboni, G., et al. (2009). Executive dysfunction in frontotemporal dementia and corticobasal syndrome. Neurology, 72(5), 453–459. Jefferson, A. L., Poppas, A., Paul, R. H., & Cohen, R. A. (2007). Systemic hypoperfusion is associated with executive dysfunction in geriatric cardiac patients. Neurobiology of Aging, 28, 477–483. Jones-Gotman, M., & Milner, B. (1977). Design fluency: The invention of nonsense drawings after focal cortical lesions. Neuropsychologia, 155 (4–5), 653–674. Kaplan, E., Fein, D., Kramer, J., Delis, D., & Morris, R. (1999). WISC-III as a process instrument. San Antonio, TX: The Psychological Corporation. Keil, K., Baldo, J., Kaplan, E., Kramer, J., & Delis, D. C. (2005). The role of the frontal cortex in inferential reasoning: Evidence from the word context test. Journal of the International Neuropsychological Society, 11, 426–433. Kiang, M., Light, G. A., Prugh, J., Coulson, S., Braff, D. L., & Kutas, M. (2007). Cognitive, neurophysiological, and functional correlates of proverb interpretation abnormalities in schizophrenia. Journal of the International Neuropsychological Society, 13, 653–663. Kleinhans, N., Akshoomoff, N., & Delis, D. C. (2005). Executive functions in autism and Asperger’s disorder: Flexibility, fluency, and inhibition. Developmental Neuropsychology, 27(3), 379–401. Kramer, J. H., Reed, B. R., Mungas, D., Weiner, M. W., & Chui, H. C. (2002). Executive dysfunction in subcortical ischemic vascular disease. Journal of Neurology, Neurosurgery, & Psychiatry, 72, 217–220. Kramer, J. H., Quitania, L., Dean, D., Heuhaus, J., Rosen, H. J., Halabi, C., et al. (2007). Magnetic resonance imaging correlates of set shifting. Journal of the International Neuropsychological Society, 13(3), 386–392. Lysaker, P. H., Whitney, K. A., & Davis, L. W. (2006). Awareness of illness in schizophrenia: Associations with multiple assessments of executive functions. Journal of Neuropsychiatry & Clinical Neurosciences, 18, 516–520. Mattson, S. N., Goodman, A. M., Caine, C., Delis, D. C., & Riley, E. P. (1999). Executive functioning in children with heavy prenatal alcohol exposure. Alcoholism: Clinical and Experimental Research, 23 (11), 1808–1815. McDonald, C. R., Delis, D. C., Norman, M. A., Tecoma, E. S., & IraguiMadozi, V. J. (2005a). Is impairment in set-shifting specific to frontal lobe dysfunction? Evidence from patients with frontal lobe or temporal-lobe epilepsy. Journal of the International Neuropsychological Society, 11(4), 477–481. McDonald, C. R., Delis, D. C., Norman, M. A., Tecoma, E. S., & Iragui, V. J. (2005b). Discriminating patients with frontal lobe epilepsy and

Delusion temporal lobe epilepsy: Utility of a multi-level design fluency test. Neuropsychology, 19, 806–813. McDonald, C. R., Delis, D. C., Norman, M. A., Wetter, S. R., Tecoma, E. S., & Iragui, V. J. (2005c). Response inhibition and set-shifting in patients with frontal lobe epilepsy or temporal-lobe epilepsy. Epilepsy & Behavior, 7, 438–446. McDonald, C. R., Delis, D. C., Kramer, J. H., Tecoma, E. S., & Iragui, V. J. (2008). A componential analysis of proverb interpretation in patients with frontal lobe epilepsy and temporal lobe epilepsy: relationships with disease-related factors. The Clinical Neuropsychologist, 22(3), 480–496. Parmenter, B. A., Zivadinov, R., Kerenyi, L., Gavett, R., WeinstockGuttman, B., Dwyer, M. G., et al. (2007). Validity of the Wisconsin Card sorting and Delis-Kaplan executive function system (D-KEFS) sorting tests in multiple sclerosis. Journal of Clinical and Experimental Neuropsychology, 29, 215–223. Partington, J. E., & Leiter, R. G. (1949). Partington’s pathway test. The Psychological Service Center Bulletin, 1, 9–20. Reitan, R. M., & Wolfson, D. (1993). Halstead-Reitan neuropsychological battery. Tuscon, AZ: Neuropsychology Press. Schonfield, A. M., Mattson, S. N., Lang, A. R., Delis, D. C., & Riley, E. P. (2001). Verbal and nonverbal fluency in children with heavy prenatal alcohol exposure. Journal of Studies on Alcohol, 62, 239–246. Stroop, J. R. (1935). Studies of interference in serial verbal reaction. Journal of Experimental Psychology, 18, 643–662. Werner, H., & Kaplan, E. (1952). The acquisition of word meanings: A developmental study. Monographs of the Society for Research in Child Development,15 (Serial No. 51). United Kingdom: Blackwell Publishing. Yochim, B., Baldo, J., Nelson, A., & Delis, D. C. (2007). D-KEFS Trail Making Test performance in patients with lateral prefrontal cortex lesions. Journal of the International Neuropsychological Society, 13, 704–709. Yochim, B. P., Baldo, J. V., Kane, K. D., & Delis, D. C. (2009). D-KEFS Tower Test performance in patients with lateral prefrontal cortex lesion: The importance of error monitoring. Journal of Clinical and Experimental Neuropsychology, 31(6), 658–663.

Synonyms Psychotic misconception

Description A delusion is an erroneous belief typically associated with misinterpretation of perceptions or experiences. These

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beliefs are sustained despite incontrovertible evidence to the contrary. Delusions can occur in the context of a wide variety of psychiatric and medical conditions (for a review see Fenning et al., 2005) including the following:

Delusion A NTHONY C. RUOCCO 1, FARZIN I RANI 2 1 University of Illinois at Chicago Chicago, IL, USA 2 University of Pennsylvania Philadelphia, PA, USA

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Psychiatric disorders including psychotic disorders (schizophrenia, schizoaffective disorder, and delusional disorder), mood disorders (bipolar disorder and depression), and substance-related disorders (amphetamines, cocaine, alcohol, cannabis, and hallucinogen). Neurodegenerative disorders such as Alzheimer’s disease, frontotemporal dementia, dementia with Lewy bodies, Huntington’s disease, basal ganglia calcification, and multiple sclerosis. Other central nervous system disorders including brain tumors, particularly temporal lobe and deep hemispheric tumors, epilepsy, subdural hematomas, anoxic brain injuries, and fat embolism. Metabolic conditions including hypercalcemia, hyponatremia, hypoglycemia, uremia, hepatic encephalopathy, and porphyria. Vascular conditions such as atherosclerotic vascular disease, especially when associated with diffuse, temporoparietal or subcortical lesions, hypertensive encephalopathy, subarachnoid hemorrhage, and temporal arteritis. Infectious diseases such as human immunodeficiency virus/acquired immune deficiency syndrome, Creutzfeldt–Jakob disease, syphilis, malaria, and acute viral encephalitis. Endocrinopathies such as Addison disease, Cushing syndrome, hyperthyroidism or hypothyroidism, and panhypopituitarism. Vitamin deficiencies including vitamin B-12 deficiency, folate deficiency, thiamine deficiency, and niacin deficiency. Medications including adrenocorticotropic hormones, anabolic steroids, corticosteroids, cimetidine, antibiotics (cephalosporins, penicillin), disulfiram, and anticholinergic agents. Toxins such as mercury, arsenic, manganese, and thallium.

Categorization Delusions are usually categorized according to the following themes: Other delusions may involve themes of guilt, jealousy, or religion. Delusions may also be classified as bizarre or non-bizarre. Bizarre delusions are implausible beliefs, which are incomprehensible and are not associated with

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typical life experiences (e.g., person believes tiny robots are recording his or her conversations by installing microphones in his or her teeth). Bizarre delusions are associated with schizophrenia and its spectrum disorders. Non-bizarre delusions are plausible, understandable, and consistent with a person’s daily experiences (e.g., person believes his or her spouse is unfaithful). Delusions can be systematized or organized around a common theme. Delusions may be congruent with the person’s mood (e.g., person believes he or she can walk on water during the course of a manic episode) or the content of the delusion can also be incongruent with the person’s mood (e.g., person believes he or she is dead but is not depressed).

Epidemiology A World Health Organization survey of first-contact incidence of schizophrenia in 10 countries found the incidence of referential and persecutory delusions to be the most common (Sartorius et al., 1986). Delusional thinking may be quasidimensional in nature (i.e., both continuous and dichotomous), ranging from overvalued ideas on one hand to frank psychosis on the other. The prevalence of delusional beliefs in nonclinical samples is estimated at between 1 and 3 percent, which may vary depending on the content of the delusion and the demographic characteristics of the sample (for a review, see Freeman, 2007).

Natural History, Prognostic Factors, Outcomes Karl Jaspers, a German philosopher and physician, was among the first to describe the nature of delusions. In his book General Psychopathology, originally published in 1913, he outlined three classic criteria for a delusion: conviction, incorrigibility, and impossibility. These notions remain visible in modern descriptions of delusion, such as in the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders. Current attempts at understanding the nature of delusions examine the contributions of life experiences (e.g., paranoia-inducing events), perceptual and attentional factors (e.g., selective attention to threat-related information), and inferential processes (e.g., attributions of negative events) to the development and maintenance of delusions. Over the lifespan, delusions tend to decrease in severity in patients with schizophrenia (Mancevski et al., 2007). Outcomes for delusional patients with major depressive disorder tend to be worse than those without

psychotic features, as they have a longer time to recovery and are more likely to be treated with antipsychotic medication (Maj, Pirozzi, Magliano, Fiorillo, & Bartoli, 2007).

Neuropsychology of Delusion A delusion is a symptom of psychosis, which may be present in a variety of neuropsychiatric disorders, including delirium, dementia, brain injury, and psychiatric disorders. One prominent theory of the neuropathogenesis of delusions and hallucinations in schizophrenia is a reactive synaptic regeneration in brain regions, such as the frontal cortex, which receive degenerating temporal lobe connections (Stevens, 1992). These connections might result in spontaneous, stimulus-independent retrieval of episodic memories that resemble delusions. Positive symptoms of schizophrenia, including delusions, share minimal relations with neuropsychological function, whereas cognitive deficit is more commonly associated with negative symptoms (e.g., deficiencies in emotional responsiveness, spontaneous speech, and volition) (O’Leary et al., 2002). Several brain structures have been associated with delusions in a variety of neuropsychiatric disorders. Delusions in Alzheimer’s disease are correlated with lower gray matter density in the left frontal lobe, right frontoparietal cortex, and left claustrum (Bruen, McGeown, Shanks, & Venneri, 2008). Lower prefrontal cortex volumes are related to delusions in older adults with depression (Kim et al., 1999). In patients with schizophrenia, delusional symptoms have been associated with smaller right planum temporale volumes (Yamasaki et al., 2007).

Evaluation In evaluating the presence of delusions, a distinction should be drawn between delusions and overvalued ideas, which may be held with less conviction, particularly in the face of clear contradictory evidence regarding the truthfulness of the belief. In neuropsychological evaluation, the presence of delusions may aid in differential diagnosis. For example, Alzheimer’s disease and dementia with Lewy bodies are more likely to present with delusions as a component of the neuropsychiatric disorder. Certain delusions are sometimes present in specific neuropsychiatric disturbances. For example, Capgras delusion, or the belief that impostors have replaced persons familiar to the individual, is sometimes observed in patients with Alzheimer’s disease, dementia with Lewy bodies, and schizophrenia.

Delusion

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Delusion. Table 1 Theme

Description

Persecutory

Belief of being tormented, followed, tricked, spied on, or ridiculed

Referential

Belief that certain gestures, comments, passages from books, newspapers, song lyrics, or other environmental cues are specifically directed at the patient

Grandiose

Belief about exaggerated power, knowledge, importance, or a special relationship to a deity or famous person

Somatic

Belief that there is a change or disturbance in one’s body appearance or functioning

Control

Belief that one’s feelings, impulses, thoughts, or actions are under the control of some external force

Thought broadcasting

Belief that one’s thoughts are audible to others

Thought insertion

Belief that thoughts which are not one’s own have been inserted into his or her mind

Thought withdrawal

Belief that one’s thoughts have been removed from his or her mind

Erotomanic

Belief that another person is in love with him or her

Treatment Antipsychotics are the drugs of choice for treating delusions. The strong connection between neuropsychological function and functional disability makes improving cognitive abilities a primary target of pharmacologic treatment in schizophrenia. Antipsychotic drugs, however, appear to have variable effects on neuropsychological function (Jerrell & Ramirez, 2008). Conventional antipsychotics have several adverse side effects, including neurologic (extrapyramidal reactions, tardive dyskinesia, and miscellaneous neurologic effects), cardiovascular (orthostatic hypotension, tachycardia, and electrical conduction delays), and anticholinergic (both peripheral and central manifestations). Atypical antipsychotics tend to have comparatively fewer side effects than conventional antipsychotics, but notable risks include extrapyramidal side effects and metabolic syndrome. Caution must be taken in prescribing antipsychotic drugs to older adults with psychosis. In 2005, the Food and Drug Administration issued a ‘‘black-box’’ warning because atypical antipsychotics were associated with increased risk of death in dementia patients. The conventional antipsychotics may also increase dementia patients’ risk of death. Cognitive-behavioral psychotherapy has also demonstrated efficacy for the treatment of delusions in medication-resistant schizophrenia (Rathod, Kingdon, Weiden, & Turkington, 2008).

Cross References ▶ Alzheimer’s Dementia ▶ Capgras Syndrome ▶ Delirium

▶ Dementia with Lewy Bodies ▶ Psychotherapy

References and Readings Bruen, P. D., McGeown, W. J., Shanks, M. F., & Venneri, A. (2008). Neuroanatomical correlates of neuropsychiatric symptoms in Alzheimer’s disease. Brain, 131, 2455–2463. Fennig, S., Fochtmann, L. J., & Bromet, E. J. (2005). Delusional and shared psychotic disorder. Kaplan & Sadock’s comprehensive textbook of psychiatry (8th ed., pp. 1525–1533). Philadelphia, PA: Lippincott Williams & Wilkins. Freeman, D. (2007). Suspicious minds: The psychology of persecutory delusions. Clinical Psychology Review, 27, 425–457. Jerrell, J. M., & Ramirez, P. M. (2008). Changes in neuropsychological functioning following treatment with risperidone, olanzapine, and conventional antipsychotic medications. Human Psychopharmacology, 23, 595–604. Kim, D. K., Kim, B. L., Sohn, S. E., Lim, S. W., Na, D. G., et al. (1999). Candidate neuroanatomic substrates of psychosis in old-aged depression. Progress in Neuro-psychopharmacology & Biological Psychiatry, 23, 793–807. Maj, M., Pirozzi, R., Magliano, L., Fiorillo, A., & Bartoli, L. (2007). Phenomenology and prognostic significance of delusions in major depressive disorder: A 10-year prospective follow-up study. Journal of Clinical Psychiatry, 68, 1411–1417. Mancevski, B., Keilp, J., Kurzon, M., Berman, R. M., Ortakov, V., et al. (2007). Lifelong course of positive and negative symptoms in chronically institutionalized patients with schizophrenia. Psychopathology, 40, 83–92. O’Leary, D. S., Flaum, M., Kesler, M. L., Flashman, L. A., Arndt, S., et al. (2000). Cognitive correlates of the negative, disorganized, and psychotic symptom dimensions of schizophrenia. Journal of Neuropsychiatry & Clinical Neurosciences, 12, 4–15. Rathod, S., Kingdon, D., Weiden, P., & Turkington, D. (2008). Cognitivebehavioral therapy for medication-resistant schizophrenia: A review. Journal of Psychiatric Practice, 14, 22–33. Sartorius, N., Jablensky, A., Korten, A., Ernberg, G., Anker, M., et al. (1986). Early manifestations and first-contact incidence of

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schizophrenia in different cultures. A preliminary report on the initial evaluation phase of the WHO Collaborative Study on determinants of outcome of severe mental disorders. Psychological Medicine, 16, 909–928. Stevens, J. R. (1992). Abnormal reinnervation as a basis for schizophrenia: A hypothesis. Archives of General Psychiatry, 49, 238–243. Yamasaki, S., Yamasue, H., Abe, O., Yamada, H., Iwanami, A., et al. (2007). Reduced planum temporale volume and delusional behaviour in patients with schizophrenia. European Archives of Psychiatry and Clinical Neuroscience, 257, 318–324.

Dementia K ATHRYN V. PAPP The University of Connecticut Storrs, CT, USA

Synonyms Senility

Short Description or Definition Dementia is characterized by a progressive decline in multiple domains of cognitive functioning which ultimately interferes with independent daily living and results in a shortened lifespan. It is not a disease or a disorder in itself but a descriptive symptom complex with a number of potential underlying pathologies and/or traumas which result in deficits in multiple domains. It is largely a diagnosis of the elderly, but certain forms such as Multiple sclerosis and Huntington’s disease can appear in middle age. Variations such as Creutzfeldt–Jakob disease and head trauma, which results in a nonprogressive dementia, can occur at any age. In progressive dementia, early symptoms can include memory complaints (forgetting names and appointments) and difficulty in learning new material. As dementia progresses, complaints may include difficulties with expressive and receptive language, impairments in executive function (poor judgment, difficulty planning), an inability to remember already learned information (such as names of family members), and changes in personality. Dementia patients ultimately require full nursing care in the last stages of disease; they suffer from incontinence, motor inability, echolalia, paraolia, and the return of primitive reflexes. A common cause of death is infection. The general characteristics of dementias (as outlined in detail for specific dementias in the DSM-IV-TR) are:

1. Multiple cognitive deficits are present, including the following: (a) Memory impairment (new learning or recall) (b) One or more of aphasia, apraxia, agnosia, or executive dysfunction 2. Cognitive deficits significantly impair social or occupational functioning and reflect a significant decline from a previous level of higher functioning 3. Cognitive deficits are not exclusively present during a delirium 4. Cognitive deficits cannot be better attributed to another Axis I disorder, such as depression or schizophrenia (Rabin, Wishart, Fields, & Saykin, 2006) While the etiology of every type of dementia is not fully understood, there are a wide variety of causes including vascular changes, inherited genes, vitamin deficiencies, prions, and viruses. The causes of dementia may not be mutually exclusive, with a number of pathologies contributing to a patient’s symptoms. Multiple types of dementia have been described, which roughly fall into two groups that exemplify the initial area of degeneration: Cortical versus subcortical. Dementias are more easily distinguished from one another in early stages but become increasingly difficult to differentiate as pathology becomes more global. The time period from diagnosis to death involves interindividual variation and variation based on the dementia’s etiology. Mortality can be as short as 2 months after symptom onset in AIDS dementia and Creutzfeldt– Jakob disease and as long as 20 years in Alzheimer’s disease (AD).

Categorization Dementia can be broadly characterized as Cortical (originating in cortical areas; characterized by intellectual and memory dysfunction, aphasia, agnosia, and apraxia) versus Subcortical (originating in frontosubcortical systems such as brainstem, thalamus, and basal ganglia; initial motor dysfunction and cognitive slowing, with language and memory the last faculties affected). These distinctions are not necessarily mutually exclusive and there is much overlap (Table 1). Until approximately 10–15 years ago, dementia was largely subdivided into nonspecific atrophy, Parkinson’s disease, dementia with Lewy bodies, AD, and Pick’s disease. Since then, an explosion in both research and imaging techniques as well as a more thorough study of the cognitive deficits earlier in the disease course has shown that these categorizations may oversimplify the

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Dementia. Table 1 Types of dementias Cortical dementias

Subcortical dementias

Other dementias

Alzheimer’s disease (AD)

Parkinson’s disease

Multiple sclerosis

Lewy body dementia

Huntington’s disease

AIDS dementia

Frontotemporal dementia

Wilson’s disease

Wernicke–Korsakoff’s disease

Pick’s disease

Normal pressure hydrocephalous

Creutzfeldt–Jakob disease

Semantic dementia/primary progressive aphasia

Progressive supranuclear palsy

Head trauma

Vascular dementia/multi-infarct dementia

differential etiology and neuropathology of dementia. Further research may parse out further categories and stages of dementia in the future.

Epidemiology AD is the most common form of dementia with estimates that account for 50–80% of dementia cases. Other common forms include Vascular dementia, which accounts for 15% of cases, and dementia with Lewy Bodies which, accounts for 20% of all incidence (McKeith, Galasko, Kosaka, Perry, Dickson, & Hansen, 1996). Less prevalent are Parkinson’s disease, progressive supranuclear palsy (PSP), Pick’s disease, frontotemporal dementia, normal pressure hydrocephalus, and Wernicke-Korsakoff ’s syndrome. Relatively rare dementias are prion diseases such as Creutzfeldt–Jakob disease which occurs in one in one million (Kapur, Abbott, Lowman, & Will, 2003). Vascular dementia is more common in Japan versus AD although the overall dementia rates are equal (Otsuka, 1994; Seno, Ishino, Inagaki, Iijima, Kaku, & Inata, 1999). It is unclear if this difference occurs because of different diagnostic trends or environmental and/or gene pool differences. AD is particularly prevalent in Israeli Arabs (Wertman, Brodsky, King, Bentur, & Chekhmir, 2007) and less prevalent in China (Liu, Guo, Zhou, & Xia, 2003) and India (Ferri et al., 2005). The prevalence of dementia in developed countries is 1.5% in people aged 65. The incidence of dementia increases dramatically with age, doubling every 5 years after age 65 (Kukull & Ganguli, 2000). The largest risk factors are increasing age and familial history. Those diagnosed with mild cognitive impairment (MCI) or Down’s syndrome and those with a history of traumatic brain injury, stroke, or migraine are at greater risk. A variety of lifestyle risk factors have been studied and variably implicated, including vascular symptoms associated with hypertension, obesity, and diabetes (Hofman,

Ott, Breteler, Bots, & Slooter, 1997); high stress (Pope, Shue, & Beck, 2003); exposure to aluminum, copper, and iron in the water supply (Christen, 2000); and occupational exposure to fumigants and/or defoliants (Tyas, Manfreda, Strain, & Montgomery, 2001). Several protective factors have also been explored, with varying degrees of empirical support, including high educational attainment (Ott, Van Rossum, Van Harskamp, Van de Mheen, Hofman, & Breteler, 1999), strong social networks (Fratiglioni, Paillard-Borg, & Winbald, 2004), participation in leisure activities (Scarmeas, Levy, Tang, Manly, & Stern, 2001; Verghese, Lipton, Katz, Hall, Derby, & Kuslansky, 2003), exercise (Colcombe & Kramer, 2003), the use of nonsteroidal antiinflammatory drugs, the use of antioxidants (Fotuhi et al., 2008), and diets with high levels of vitamins B6, B12, and folate (Tyas et al., 2001).

Natural History, Prognostic Factors, Outcomes Dementia was long considered a natural part of aging (Berchtold & Cotman, 1998). The term was first used in a clinical context by Pinel and Esquirol in the eighteenth century (Berchtold & Cotman; Cummings & Benson, 1992); however, the link between neuropathology and dementia was not made until 1907 by Alois Alzheimer. A date considered as the beginning of the modern study of dementia is 1968 which marks the publication by Blessed et al. of an article linking cognition with histopathology. The general prognosis for dementia is poor, although if caught early, Wernicke–Korsakoff ’s dementia, Normal Pressure Hydrocephalous, and Wilson’s dementia can be treated with relative success. The disease course from diagnosis to death is quite variable with Creutzfeldt– Jakob disease, resulting in death within a few weeks for some patients; Lewy body dementia averaging 3 years to mortality; and AD from 5 to 20 years.

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Cortical Dementias AD involves a slowly progressive, prominent memory disturbance. It extends into other cognitive domains and leads to behavioral and personality alterations. It is degenerative and ultimately leads to death within 5–20 years. AD results in the reduction in size of temporal and frontal lobes, the brain regions involved in learning and memory processes. The classic, neuronal–pathological features seen in autopsy sections are neuritic plaques and neurofibrillary tangles. Definitive diagnosis is still only available post mortem and is indicated by a sufficient number of plaques and tangles. Lewy body dementia is characterized by parkinsonian pathology with early dementia as well as visual hallucinations. Duration is relatively short with the average diagnosis to death lasting 3 years. Frontotemporal dementia is characterized by cerebral atrophy which begins in the frontal regions and progresses from anterior to posterior regions. Plaques and tangles are not present but postmortem pathology may reveal Pick’s bodies. Pick’s disease presents similarly to frontotemporal dementia. It is characterized by frontal and temporal atrophy and Pick’s bodies. In its first stages, it is characterized by disinhibition, loss of judgment/insight, and utilization behavior. The second stage involves echolalia, agnosia, and intellectual dysfunction with relatively preserved memory. The final stage resembles the end-stage AD. Mortality occurs in approximately 6–12 years with onset earlier than most other dementias (from age 40 to 60). Semantic dementia/Primary progressive aphasia usually involves 2 years of progressive aphasia before other symptoms present themselves. It is considered a subtype of frontotemporal dementia; it is defined by its symptoms as the exact pathology is not yet understood. Vascular dementia/Multi-infarct dementia is characterized by an abrupt onset. It originates in the blood vessels with a series of small strokes and can progress in a stepwise fashion if more infarcts occur. The neuropsychology of this type of dementia is dependent on the locations of the infarcts.

Subcortical Dementias Parkinson’s disease is characterized by a loss of dopamine cells in the substantia nigra. Up to 90% of patients exhibit cognitive impairments. Huntington’s disease is a genetic dominant disorder whose symptoms typically begin in people between ages

40 and 50; however, there is juvenile onset in 5–10% of cases. Neurotransmitters GABA and acetylcholine are depleted. Degeneration begins in striatum, but is also seen in globus pallidus, hypothalamus, subthalamic nucleus, substantia nigra, and thalamus. The majority of patients experience early difficulties in attention, executive function, and spatial abilities. Wilson’s disease results from a genetic metabolic disorder which results in the accumulation of copper in the basal ganglia. About 40% of patients present with a liver disorder, another 40% exhibit motor problems, and the remaining 20% suffer from psychiatric symptoms which present as mania or schizophrenia-like symptoms. Diagnosis can be confirmed by looking for a buildup of copper in the eyes. Normal pressure hydrocephalous results from a blockage of cerebral spinal fluid (CSF). It is characterized by progressive gait ataxia, incontinence, and amnesia. However, these symptoms are reversible if caught early and treated with a shunt to drain CSF. Progressive supranuclear palsy results in cell loss in the brainstem, cerebellum, and basal ganglia. It is characterized by difficulty in moving the eyes and gait disturbance.

Other Dementias Multiple sclerosis is a disorder of myelin which follows a pattern of relapse and remission. Effective treatments can slow the progression of the disease, but once cognitive deficits – which include attention, language, memory, and executive function difficulties – are present, there are fewer and shorter periods of remission. AIDS dementia results from HIV replication in the central nervous system, producing a progressive encephalopathy. It is associated with increasing age, increased viral load, and decreased CD4 cell count. Once AIDS patients become demented, time to death averages 2 months. Wernicke–Korsakoff ’s disease results from thiamine deficiency (vitamin B1) and is most often seen in chronic alcoholics. It is characterized by anterograde and retrograde amnesia, confabulation, apathy, and lack of insight. Creutzfeldt–Jakob disease is the most common human prion disease. It involves a rapidly progressive dementia, with memory loss, personality changes, hallucinations, speech impairment, myoclonus, ataxia, and changes in gait and seizures. Head trauma can produce nonprogressive dementia and also confer greater risk for developing other dementias.

Dementia

Neuropsychology and Psychology of Dementia Differential Diagnosis Memory impairment is necessary for a diagnosis of dementia; however, it must be accompanied by an impairment in at least one more cognitive domain, to be distinguished from Amnestic Disorder. It must also be distinguished from Delirium which can include deficits in multiple cognitive domains including memory; however, delirium primarily presents with decreased awareness and altered consciousness. Delirium is also characterized by abrupt onset and fluctuating course whereas dementia onset is usually insipid and progressive. Depression is comorbid in the early stages of a number of dementias, but because Depression itself can present as cognitive impairment, it must be ruled out as the primary diagnosis. Depression can also be a predictor of later dementia; following the cognitive changes of an individual will enable the practitioner to make the most accurate diagnosis (Cummings, 2003). Dementia must also be distinguished from the cognitive changes (such as lowered speed of processing and changes in attention) which are seen in normal aging. Recent research has also outlined the state between normal aging and dementia. The criteria for diagnosis of MCI include (1) cognitive complaint (usually memory), preferably corroborated by an informant; (2) cognitive impairment (usually memory), for age and education; (3) essentially normal general cognitive function; (4) largely preserved activities of daily living and (5) no dementia

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(Kelley & Petersen, 2007). Petersen et al. (2001) divided MCI into categories to reflect a subgroup of patients with episodic memory complaints who convert to AD at a greater rate: Amnestic (primary memory complaint) versus nonamnestic (complaints can include language, attention/executive function, and visuospatial skills). Those diagnosed with Amnestic MCI (MCIa) progress to AD at a rate of 10–15% per year in comparison with a rate of 1–2% per year in healthy elderly (Petersen et al). Presentations typical of cortical and subcortical dementias are outlined in Table 2. The ability to distinguish between dementias is greatest at the early stages of the disease. Late stages of dementia present similarly as deterioration becomes profuse. These mid- and late-stage dementia symptoms can include personality changes with delusions of persecution, comabtiveness, agitation (especially at night which is termed ‘‘sundowning effect’’), gait disturbances, and delirium.

Evaluation The goals of a neuropsychological evaluation for dementia are obtaining baseline measures of functioning to provide a benchmark of change over time, determining areas of strength and weakness, assisting in making a diagnosis and providing treatment recommendations, and determining the validity of a prior diagnosis or the effectiveness of a treatment/intervention. Diagnosis of dementia involves a detailed patient history, a clinical exam, and radiology to detect structural or electrical abnormalities in the brain. The clinical exam covers the following domains: (1) general

Dementia. Table 2 Presentations of typical cortical and subcortical dementias Cortical dementia

Subcortical dementia

Intellect

Aphasia, amnesia, visuospatial disorder, poor abstraction, acalculia, apraxia, agnosia

Psychomotor retardation, forgetfulness, impaired insight, poor strategy formulation

Personality

Indifference, depression

Depression or mania, apathy

Speech

Normal

Dysarthria

Language

Anomia, paraphasia

Normal

Memory

Unable to learn

Retrieval problems

Motor system

Normal

Abnormal (parkinsonian, chorea, dystonia, tremor)

Stance

Normal

Stooped

Gait

Normal or pacing

Dancing, unsteady, festinating, ataxic

Activity

Normal

Slow

Appearance Normal

Inform, disheveled, perplexed

Visuospatial Construction problems

Sloppy because of movement

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awareness and cooperativeness; (2) function of cranial nerves; (3) motor function; (4) reflexes; (5) sensory function; and (6) mental status.

Treatment Cortical Dementia Current treatments for cortical dementias are symptomatic. Cholinesterase inhibitors are used to treat AD and Lewy body dementia as they enhance cholinergic function early in disease course (often up to 4 years). However, this class of drug confers less benefit on cognition as cholinergic cell death increases throughout the disease progression. Currently used anticholinesterase inhibitors include Reminyl (galantamine), Aricept (Donepezil), and Exelon (rivastigmine). Side effects include gastrointestinal difficulties, particularly anorexia, nausea, and vomiting, and serious side effects which occur in 1% of patients are gastrointestinal ulceration, gastrointestinal hemorrhage, and mild pancreatitis. Also reported are diarrhea, vivid dreams, cramps, and dizziness. Cholinesterase inhibitors may decrease heart rate and increase shortness of breath in patients with asthma. Patients who respond with improvement in both cognitive and behavioral symptoms, while taking a cholinesterase inhibitor are more likely to have dementia with Lewy bodies rather than AD (McKeith, 2002). Disease-modifying drugs, some of which are in human trials, continue to be investigated (Cummings, Vinters, Cole, & Khachaturian, 1998). Patients with comorbid depression are also treated with antidepressants, and those with psychotic symptoms such as frequent hallucinations and delusions are treated with antipsychotic agents. In the earlier stages of dementia, patients might benefit from supportive therapy or group therapy as well as environmental adjustments; however, as insight diminishes, family education and counseling are more useful for caregivers. Compensatory strategies, behavior modifications, and relearning of rote tasks have shown moderate utility in activities of daily living.

Subcortical Dementia Treatment for Parkinson’s disease focuses on neuroprotection to decrease the rate of disease progression. Sinemet (a combination of L-Dopa, a precursor to dopamine, and Carbidopa which decreases nausea and vomiting caused by L-Dopa alone) is used to decrease parkinsonian symptoms. The therapeutic effect of L-Dopa diminishes after 2–3 years and its efficacy for improving the cognitive

status is equivocal. Side effects include mild psychotic symptoms such as visual hallucinations, paranoid delusions, vivid dreams, confusional states, and dyskinesias. Selegiline, a monoamine oxidase (MAO) inhibitor has been used to extend the efficacy of L-Dopa (Nutt, Hammerstad, & Gancher, 1992). Anticholinergic medications have been used to treat motor effects, but they tend to have adverse effects on some executive functions (Glatt & Koller, 1992). Deep brain stimulation or lesioning in the globus pallidus, subthalamic nucleus, or ventral intermediate thalamic nucleus has been used in patients with no sign of cognitive symptoms and difficulty in managing medication (Eskandar, Cosgrove, & Sinobu, 2001). When successful, these treatments result in improved motor function as well as increased psychomotor speed and working memory (Pillon, Ardouin, & Damier, 2000). Treatment options for Huntington’s are limited to palliative care. Neuroleptic drugs have been used to relieve choreic movements, however, they tend to promote Parkinson-like symptoms (Lerner & Whitehouse, 2002). PSP does not respond to dopamanergic or anticholinergic drugs despite its shared features with Parkinson’s disease (Kompoliti, Goetz, & Litvan et al., 1998). Antidepressants have been used to treat its emotional symptoms.

Cross References ▶ Alzheimer’s Dementia ▶ Alzheimer’s Disease ▶ Subcortical Dementia ▶ Vascular Dementia

References and Readings Berchtold, N. C., & Cotman, C. W. (1998). Evolution in the conceptualization of dementia and Alzheimer’s disease: Greco-roman period to the 1960s. Neurobiology of Aging, 19(3), 173–189. Christen, Y. (2000). Oxidative stress and Alzheimer disease. American Journal of Nutrition, 71, 621S–629S. Colcombe, S., & Kramer, A. F. (2003). Fitness effects on the cognitive function of older adults: A meta-analytic study. Psychological Science, 14(2), 125–130. Cummings, J. L. (2003). Neuropsychiatric symptoms. In R. C. Petersen (Ed.), Mild cognitive impairment: Aging to Alzheimer’s disease (pp. 41–61). New York: Oxford University Press. Cummings, J. L., & Benson, D. F. (1992). Dementia: A clinical approach. Boston: Butterworth-Heinemann Medical. Cummings, J. L., Vinters, H. V., Cole, G. M., & Khachaturian, Z. S. (1998). Alzheimer’s disease: Etiologies, pathophysiology, cognitive reserve, and treatment opportunities. Neurology, 51, S2–S17.

Dementia of Frontal Lobe Type Eskandar, E. N., Cosgrove, G. R., & Sinobu, L. A. (2001). Surgical treatment of Parkinson disease. Journal of the American Medical Association, 286, 3056–3059. Ferri, C. P., Prince, M., Brayne, C., Brodaty, H., Fratiglioni, L., Ganguli, M., et al. (2005). Global prevalence of dementia: A delphi consensus study. Lancet, 366(9503), 2112–2117. Fotuhi,M., Zandi, P. P., Hayden, K. M., Khachaturian, A. S., Szekely, C. A., Wengreen, H., et al. (2008). Better cognitive performance in elderly taking antioxidant vitamins E and C supplements in combination with nonsteroidal anti-inflammatory drugs: The cache county study. Alzheimer’s and Dementia, 4(3), A4. Fratiglioni, L., Paillard-Borg, S., & Winbald, B. (2004). An active and socially integrated lifestyle in late life might protect against dementia. Lancet Neurology, 3, 343–353. Glatt, S. L., & Koller, W. C. (1992). Effect of antiparkinsonian drugs on memory. In S. J. Huber & J. L. Cummings (Eds.), Parkinson’s disease: Neurobehavioral aspects. New York: Oxford University Press. Hofman, A., Ott, A., Breteler, M. M. B., Bots, M. L., Slooter, A. J. C., et al. (1997). Atherosclerosis, apolipoprotein E, and prevalence of dementia and Alzheimer’s disease in the rotterdam study. Lancet, 349, 151–154. Kapur, N., Abbott, P., Lowman, A., & Will, R. G. (2003). The neuropsychological profile associated with variant creutzfeldt-jakob disease. Brain, 126, 2693–2702. Kelley, B. J., & Petersen, R. C. (2007). Alzheimer’s disease and mild cognitive impairment. Neurologic Clinics, 25, 577–609. Kompoliti, K., Goetz, C. G., & Litvan, I., et al. (1998). Pharmacological therapy in progressive supranuclear palsy. Archives of Neurology, 55, 1099–1102. Kukull, W. A., & Ganguli, M. (2000). Epidemiology of dementia: Concepts and overview Neurologic Clinics, 18(4), 923–950. Lerner, A. J., & Whitehouse, P. J. (2002). Neuropsychiatric aspects of dementias associated with motor dysfunction. In S. C. Yudofsky & R. E. Hales (Eds.), Textbook of neuropsychiatry and clinical neurosciences (4th ed.). Washington, DC: American Psychiatric Publishing. Lezak, M. D., Howieson, D. B., & Loring, D. W. (Eds.). (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Liu, L., Guo, X. E., Zhou, Y. Q., & Xia, J. L. (2003). Prevalence of dementia in China. Dementia and Geriatric Cognitive Disorders, 15 (4), 226–230. McKeith, I. G. (2002). Dementia with lewy bodies. British Journal of Psychiatry, 180, 144–147. McKeith, I. G., Galasko, D., Kosaka, K., Perry, E. K., Dickson, D. W., & Hansen, L. A. (1996). Consensus guidelines for the clinical and pathologic diagnosis of dementia and lewy bodies: Report on the consortium on DLB international workshop. Neurology, 47, 1113–1124. Nutt, J. G., Hammerstad, J. P., & Gancher, S. T. (1992). Parkinson’s disease. 100 maxims. Mosby Year Book. Otsuka, T. (1994). Epidemiology of dementia in the aged: From recent epidemiological survey. Journal of Senile Dementia, 8, 283–290. Ott, A., Van Rossum, C. T. M., Van Harskamp, F., Van de Mheen, H., Hofman, A., & Breteler, M. M. B. (1999). Education and the incidence of dementia in a large population-based study: The rotterdam study. Neurology, 52, 663. Petersen, R. C., Doody, R., Kurz, A., Mohs, R. C., Morris, J. C., Rabins, R. V., et al. (2001). Current concepts in mild cognitive impairment. Archives of Neurology, 58, 1985. Pillon, B., Ardouin, C., & Damier, P. (2000). Neuropsychological changes between ‘off ’ and ‘on’ STN or GPi stimulation in Parkinson’s disease. Neurology, 55, 411–418.

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Pope, S. K., Shue, V. M., & Beck, C. (2003). Will a healthy lifestyle help prevent Alzheimer’s disease? Annual Review Public Health, 24, 111–132. Rabin, L. A., Wishart, H. A., Fields, R. B., & Saykin, A. J. (2006). The dementias. In P. J. Snyder, P. D. Nussbaum, & D. L. Robins (Eds.), Clinical neuropsychology: A pocket handbook for assessment (2nd ed., p. 210). Washington, DC: American Psychological Association. Scarmeas, N., Levy, G., Tang, M. X., Manly, J., & Stern, Y. (2001). Influence of leisure activity on the incidence of Alzheimer’s disease. Neurology, 57, 2236–2242. Seno, H., Ishino, H., Inagaki, T., Iijima, M., Kaku, K., & Inata, T. (1999). A neuropathological study of dementia in nursing homes over a 17year period, in shimane prefecture, Japan. Gerontology, 45, 44–48. Snyder, P. J., Nussbaum, P. D., & Robins, D. L. (Eds.). (2006). Clinical neuropsychology: Pocket handbook for assessment (2nd ed.). Washington, DC: American Psychological Association. Sunderland, T., Jeste, D. V., Baiyewu, O., Sirovatka, P. J., & Regier, D. A. (Eds.). (2007). Diagnostic issues in dementia: Advancing the research agenda for DSM-V. American Psychiatric Publishing. Tyas, S. L., Manfreda, J., Strain, L. A., & Montgomery, P. R. (2001). Risk factors for Alzheimer’s disease: A population-based, longitudinal study in manitoba, Canada. International Journal of Epidemiology, 30, 590–597. Verghese, J., Lipton, R. B., Katz, M. J., Hall, C. B., Derby, C. A., Kuslansky, G., et al. (2003). Leisure activities and the risk of dementia in the elderly. The New England Journal of Medicine, 348, 2508–2516. Wertman, E., Brodsky, J., King, Y., Bentur, N., & Chekhmir, S. (2007). An estimate of the prevalence of dementia among community-dwelling elderly in Israel. Dementia and Geriatric Cognitive Disorders, 24(4), 294–299. Wiener, M. F., & Lipton, A. M. (Eds.). (2003). The dementias: Diagnosis, treatment and research. Washington, DC: American Psychiatric Publishing.

Dementia due to Vascular Disease/lacunar State ▶ Vascular Dementia

Dementia in Stroke ▶ Multi-infarct Dementia

Dementia of Frontal Lobe Type ▶ Frontal Temporal Dementia ▶ Frontotemporal Lobar Degenerations ▶ Pick’s Disease

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Dementia Rating Scale-2

Dementia Rating Scale-2 S TEPHANIE G RIFFITHS 1, E LISABETH M. S. S HERMAN 2 E STHER S TRAUSS 3 1 Simon Fraser University Burnaby, British Columbia, Canada 2 University of Calgary Calgary, Alberta, Canada 3 University of Victoria Victoria, British Columbia, Canada

Synonyms DRS-2; Mattis dementia rating scale

Historical Background The original version of the DRS was developed by Mattis in the early to mid-1970s so that clinicians and researchers could evaluate patients who suffered from ‘‘organic mental syndromes.’’ Although the initial descriptions of the dementia rating scale included relatively small samples of cognitive impaired patients, considerable psychometric evidence supporting its utility as a dementia-screening tool has accumulated in the intervening decades (e.g., Strauss et al., 2006). The test revision (DRS-2) has not changed any of the original DRS items, but the clarity of scoring instructions has improved and extensive new normative data are available. An alternate version (DRS-2: Alternate Form) with a new item content has also been developed (Schmidt & Mattis, 2004) for use in serial assessments.

Psychometric Data Description The Dementia Rating Scale-2 (DRS-2) is the most recent edition (2001) of a battery-style assessment of mental status in older patients with suspected dementia. The DRS-2 consists of 24 brief subtests whose scores are combined into five subscales of attention, initiation/perseveration, construction, conceptualization, and memory (for details, see Strauss, Sherman & Spreen, 2006). In addition to quantifying impairment in these specific cognitive domains, the sum of all five subscale scores (out of a possible total of 144 points) yields an estimate of overall dementia severity. As subtest items are arranged and administered in order of decreasing difficulty, patients who successfully complete the initial items on any subscale can be given credit for adequate performance on the entire subscale. Historically, clinical cutoff scores were used to identify individuals with cognitive impairment. More recently, normative data for the DRS were collected through the Mayo Older Americans Normative Study (MOANS; Lucas et al., 1998). As the DRS-2 items and subscales are identical to those of the DRS, the MOANS normative data can be used to calculate age-corrected scaled scores (M = 10, SD = 3) and percentile rank equivalents for subscale and total scores (see also Strauss et al., 2006). Administration time for the entire battery ranges from 10 to 15 minutes for healthy older individuals to 30–45 minutes for those with dementia. This instrument facilitates comprehensive screening of mental status in patients whose cognitive impairments preclude the use of more demanding tests.

Estimates of internal consistency (Cronbach’s alpha) vary by subscale, with total score, construction, conceptualization, and memory falling above 0.70, attention falling above 0.65, and initiation/perseveration at approximately 0.45 (Smith et al., 1994). The estimated alternate form reliability for the DRS-2 total score falls above 0.80, and ranges from 0.66 (initiation/perseveration) to 0.80 (Memory) for subscale scores (Schmidt et al., 2005). Practice effects appear to be reduced when the alternate form is given. Numerous studies report strong correlations between DRS total scores and other mental status evaluations, such as the Mini Mental State Exam (MMSE), and the DRS has demonstrated adequate sensitivity and specificity for cognitive impairment in a variety of samples (Strauss et al., 2006). However, the validity of specific subscales is not as consistently supported (especially for construction, attention, and initiation/ perseveration) and subscale scores should be interpreted with caution.

Clinical Uses Like the original version, the DRS-2 provides screening information regarding mental status. As such, it does not provide a comprehensive evaluation of cognitive functioning and, if impairment is evident, patients should be referred for a full neuropsychological evaluation. The DRS-2 has the advantage of removing floor effects for some lower functioning patients who would be difficult to test using other cognitive batteries. A corresponding disadvantage is its potential insensitivity to cognitive deficits, or ceiling effects, in high-functioning individuals.

Dementia with Lewy Bodies

As a screening instrument, the DRS accurately differentiates individuals with probable Alzheimer’s dementia from cognitively normal elderly adults, and is capable of detecting both early signs of dementia (Knox et al., 2003) and cognitive impairment in low-functioning samples (Das et al., 1995). The DRS also allows clinicians to assess the initial severity of cognitive impairment in the elderly and, particularly with its alternate version, can be used to track the progression of dementia over time. There is evidence that patterns of performance on the subscales of the DRS permit clinicians to discriminate between common forms of dementia such as AD, Huntington’s disease (HD), Parkinson’s disease (PD), and vascular dementia (e.g., Cahn-Weiner et al., 2002). Further, DRS total scores appear to predict adaptive functioning in community-dwelling elderly (e.g., Plehn et al., 2004) and are related to objective indicators (MRI) of cortical atrophy (Fama et al., 1997). The MOANS DRS-2 normative data has been criticized for over-sampling highly educated Caucasians living in urban areas, thus, underestimating the abilities of individuals with lower education and socioeconomic status or of minority ethnicities. To address this concern, new ageand education-corrected data from a large group of African–American community volunteers (Mayo African American Normative Studies, MOAANS) have been provided by Rilling and colleagues (2005). Like the MOANS data, these DRS norms may be extended to the DRS-2.

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Jurica, P. J., Leitten, C. L., & Mattis, S. (2001). Dementia Rating Scale-2. Odessa, FL: Psychological Assessment Resources. Knox, M. R., Lacritz, L. H., Chandler, M. J., & Cullum, C. M. (2003). Association between Dementia Rating Scale performance and neurocognitive domains in Alzheimer’s disease. The Clinical Neuropsychologist, 17(2), 216–219. Lucas, J. A., Ivnik, R. J., Smith, G. E., Bohac, D. L., Tangalos, E. G., Kokmen, E., Graff-Radford, N. R., & Petersen, R. C. (1998). Normative data for the Mattis Dementia Rating Scale. Journal of Clinical and Experimental Neuropsychology, 20(4), 536–547. Plehn, K., Marcopulos, B. A., & McLain, C. A. (2004). The relationship between neuropsychological test performance, social functioning, and instrumental activities of daily living in a sample of rural older adults. The Clinical Neuropsychologist, 18(1), 101–113. Rilling, L. M., Lucas, J. A., Ivnik, R. J., Smith, G. E., Willis, F. B., Ferman, T. J., Petersen, R. C., & Graff-Radford, N. R. (2005). Mayo’s Older African American Normative Studies: Norms for the Mattis Dementia Rating Scale. The Clinical Neuropsychologist, 19(2), 229–242. Schmidt, K., & Mattis, S. (2004). Dementia Rating Scale-2: Alternate form. Lutz, FL: Psychological Assessment Resources. Schmidt, K. S., Mattis, P. J., Adams, J., & Nestor, P. (2005). Alternate-form reliability of the Dementia Rating Scale-2. Archives of Clinical Neuropsychology, 20(4), 435–441. Smith, G. E., Ivnik, R. J., Malec, J. F., Kokmen, E., Tangalos, E. G., & Petersen, R. C. (1994). Psychometric properties of the Mattis Dementia Rating Scale. Assessment, 1(2), 123–131. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests. New York: Oxford University Press.

Dementia Scale (DS) ▶ Blessed Dementia Scale

Cross References ▶ Alzheimer’s Dementia ▶ Frontal Temporal Dementia ▶ Mental Status Examination ▶ Vascular Dementia

References and Readings Cahn-Weiner, D. A., Grace, J., Ott, B. R., Fernandez, H. H., & Friedman, J. H. (2002). Cognitive and behavioural features discriminate between Alzheimer’s and Parkinson’s disease. Neuropsychiatry, Neuropsychology & Behavioural Neurology, 15(2), 79–87. Das, J. P., Mishra, R. K., Davison, M., & Naglieri, J. A. (1995). Measurement of dementia in individuals with mental retardation: Comparison based on PPVT and Dementia Rating Scale. The Clinical Neuropsychologist, 9, 32–37. Fama, R., Sullivan, E. V., Shear, P. K., Marsh, L., Yesavage, J., Tinklenberg, J. R., Lim, K. O., & Pfefferbaum, A. (1997). Selective cortical and hippocampal volume correlates of Mattis Dementia Rating Scale in Alzheimer disease. Archives of Neurology, 54(6), 719–728.

Dementia Syndrome of Depression ▶ Subcortical Dementia

Dementia with Lewy Bodies M ATTHEW K RAYBILL , YANA S UCHY University of Utah Salt Lake City, UT, USA

Synonyms Cortical lewy body disease (CLBD); Diffuse lewy body disease (DLBD); Lewy body dementia (LBD); Senile dementia of lewy type

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Definition Dementia with Lewy bodies (DLB) is a degenerative disorder characterized by progressive cognitive decline combined with three additional defining features: (1) pronounced fluctuations in alertness and attention (e.g., frequent drowsiness, lethargy, and disorganized speech), (2) visual hallucinations, and (3) parkinsonian motor symptoms (e.g., resting tremor, muscle rigidity, bradykinesia, and/or postural instability) (McKeith et al., 1996). The most recent consensus criteria have been modified to include additional features that may be suggestive of possible DLB (McKeith et al., 2005) (See Table 1). DLB is believed to account for 15–20% of all autopsyconfirmed cases of dementia and as such may be the second most common type of degenerative dementia in older adults (after Alzheimer’s disease). The symptoms of DLB are believed to be caused by Lewy body (LB) pathology, which is an abnormal aggregation of alpha-synuclein protein inside the nuclei of neurons. LBs accumulate in areas of the brain that control particular aspects of memory and

motor functions, including subcortical and brainstem nuclei (e.g., substantia nigra, basal forebrain, and locus ceruleus), limbic structures (e.g., hippocampus, amygdala, and nucleus basalis of Meynert), and cortical areas. There are at least two subtypes of DLB. The most common form consists of coexistent Lewy bodies and Alzheimer’s pathology, while the other form is believed to overlap with Parkinson’s disease dementia (PDD). Although DLB usually occurs sporadically with no family history of the disease, rare familial cases have been reported.

Historical Background In 1912, Friedrich Lewy first described the neuronal inclusions that were found in the substantia nigra of patients diagnosed with Parkinson’s disease. In 1923, Lewy published a detailed account of 43 patients with Parkinsonism (including 21 with dementia), integrating neurological, psychiatric, and neuropathological data. The DLB syndrome continued to be clarified by researchers, and by

Dementia with Lewy Bodies. Table 1 Revised criteria for the clinical diagnosis of probable or possible DLB (Adapted from McKeith et al., 2005)

Cognitive

Central features

Core features

Progressive dementia with impairments in (a) memory (b) attention (c) executive functioning (d) visuospatial ability

Fluctuating cognition with pronounced changes in attention and alertness

Suggestive features

Supportive features

Psychiatric

Recurrent, detailed visual hallucinations

Neuroleptic sensitivity

(a) Nonvisual hallucinations (b) delusions (c) depression

Behavioral/ somatic

Parkinsonism

REM sleep disturbance

(a) Autonomic dysfunction (e.g., orthostatic hypotension, urinary incontinence) (b) repeated falls and syncope (c) transient, unexplained loss of consciousness

SPECT/PET: Low dopamine transporter uptake in basal ganglia

(a) CT/MRI: Relative preservation of medial temporal lobe (b) SPECT/PET: Generalized low uptake with reduced occipital activity (c) EEG: Prominent slow wave activity with temporal lobe transient sharp waves (d) MIBG myocardial scintigraphy: Abnormally low uptake

Neuroimaging

Note: Central features are essential for DLB diagnosis. Two or more core features are needed for probable DLB diagnosis, and one for possible DLB diagnosis. One or more suggestive feature in addition to one or more core feature are needed for probable DLB diagnosis, and one or more suggestive features with no core feature is sufficient for possible DLB diagnosis. Suggestive features are commonly present but not proven to have diagnostic specificity


the early 1970s, the term ‘‘incidental Lewy body disease’’ began to be used. Clinical and pathological features of demented patients with Lewy bodies were subsequently detailed by a number of Japanese neuropathologists. By the late 1980s, it came to be recognized that DLB occurred much more commonly than previously suspected, as it was reported to be the second most common type of dementia among the elderly. With advances in histological techniques, LBs and Lewy neurites were identified outside of the substantia nigra, including limbic and cortical areas. In October of 1995, an international workshop in Newcastle, England was organized to establish clinical diagnostic criteria and pathological protocols for DLB, the results of which were published by McKeith et al. (1996). These criteria were reviewed again at a 1999 international workshop (McKeith, Perry and Perry, 1999) and then most recently revised in 2005 (see above) (McKeith et al., 2005).

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whether it lies on the same disease spectrum as PDD. The current guidelines regarding clinical diagnosis consider whether cognitive or motor symptoms are present first (McKeith et al., 2005). If cognitive symptoms appear prior to, or concurrently with, motor symptoms, then a diagnosis of DLB can be made. Although there is also considerable similarity between the cognitive symptoms and clinical course of DLB and AD, retrospective clinical assessments of pathologically confirmed DLB have revealed distinct cognitive and psychiatric features of the disorder, with the presence of visual hallucinations and visuospatial or constructional dysfunction suggesting DLB. There is increasing consensus that the DLB syndrome can be identified and can be differentiated from other dementia syndromes in routine clinical practice, prior to pathological confirmation (Lennox & Lowe, 1996). Differentiating between brainstem predominant, limbic and diffuse neocortical LB pathology may also be relevant to the clinical syndrome (McKeith et al., 2005).

Current Knowledge Clinical Symptoms

Neuropathology

A description of DLB symptoms can be found in Table 1. In addition to the more typical signs of dementia, complex visual hallucinations accompanied by a fluctuating course are often viewed as the tell tale indicators of DLB (McKeith et al., 2005). Hallucinations are often accompanied by delusions, anxiety, and behavioral disturbances. Additionally, patients with DLB often suffer from sleep disturbances and a disruption of the day/night cycle, which may be the result of vivid, frightening dreams that occur during REM sleep. It is not uncommon for patients to appear to act out their dreams by vocalizing or violently moving around in bed. Therefore, when assessing for DLB, screening questions that address sleep issues should always be utilized. With respect to the extrapyramidal motor features, patients with DLB may exhibit similar, but somewhat more severe, difficulties as compared to patients with PD. These include action tremor (resting tremor is less common in DLB), slowness of movement, postural instability, gait difficulty, facial immobility, and muscle rigidity. However, the assessment of motor features can be complicated by the presence of cognitive impairment. Fluctuations may be one of the most difficult clinical symptoms to assess and inter-rater reliability is often low. Because DLB appears to have motor symptoms, cognitive profiles, neuropathological features, and a clinical course that are comparable to those of Parkinson’ disease dementia (PDD), there is an ongoing debate in the current literature about whether DLB is a distinct entity or

The original neuropathologic criteria for DLB only required the presence of LBs somewhere in the brain of a patient who was clinically diagnosed with dementia (McKeith et al., 1996). Other coexistent pathological features such as amyloid plaques, neurofibrillary tangles, and neuronal loss were neither inclusive nor exclusive for a diagnosis of DLB. Although this liberal definition had the advantage of including many cases, the development of more sensitive methods for detecting LBs has lead to many neuropathologically positive cases being identified as DLB even without the clinical syndrome. The most recent consensus guidelines regarding neuropathological criteria for DLB recommend a semiquantitative grading scale (e.g., mild, moderate, severe, and very severe) for lesion density, as opposed to simply identifying the presence or absence of LB pathology (McKeith et al., 2005). The density of LB pathology can be highly variable, and LBs can be frequently found in brainstem nuclei, as well as various limbic, and neocortical regions.

Treatment The clinical management of patients with DLB can be complex and includes (a) efforts to detect and appropriately diagnose cognitive impairment early on in the disease process, (b) assessment and management of neuropsychiatric and/or behavioral symptoms, (c)

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Demographically-Adjusted T Score

treatment of the movement disorder, and (d) monitoring and management of autonomic dysfunction and sleep disorder. Non-pharmacologic interventions have not been systematically evaluated, but may include strategies that increase social interactions and increase levels of arousal as a way of reducing the presence and impact of visual hallucinations (McKeith et al., 2005). Pharmacologic treatments such as Levodopa can be used to help alleviate the parkinsonian motor disorder associated with DLB. However, dopaminergic medications may exacerbate psychiatric symptoms, and as such should be started at low doses and increased only gradually (McKeith et al., 2005). Other pharmacologic interventions may include cholinesterase inhibitors or atypical antipsychotic medications. Cholinesterase inhibitors may specifically be useful for the fluctuating cognitive impairments that are associated with DLB, and may improve global functioning as well as activities of daily living. Although selective serotonin reuptake inhibitors (SSRIs) or serotonin-norepinephrine reuptake inhibitors (SNRIs) have been reported as the preferred pharmacologic intervention for depression in DLB, there have been no systematic studies of the treatment of depression in patients with this disorder.

Lennox, G., & Lowe, J. (1996). The clinical diagnosis and misdignosis of Lewy body dementia, In R. Perry, I. McKeith, & E. Perry (Eds.), Dementia with Lewy bodies: Clinical, pathological, and treatment issues (pp. 9–20). Cambridge, UK: Cambridge University Press. McKeith, I. G., Dickson, D. W., Lowe, J., Emre, M., O’Brien, J. T., Feldman, H. et al. (2005). Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology, 65(12), 1863–1872. McKeith, I. G., Galasko, D., Kosaka, K., Perry, E. K., Dickson, D. W., Hansen, L. A. et al. (1996). Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology, 47(5), 1113–1124. McKeith, I. G., Perry, E. K., & Perry, R. H. (1999). Report of the second dementia with Lewy body international workshop: diagnosis and treatment. Consortium on Dementia with Lewy Bodies. Neurology, 53(5), 902–905.

Demographically-Adjusted T Score ▶ T Scores

Demyelination Future Directions Efforts to find new ways of treating DLB have begun to focus on early detection in hopes of finding ways of potentially slowing the progression of the disease. For example, practical biomarkers or disease modifying strategies that target alpha-synuclein deposition would be significant developments that hopefully will occur in the near future (Goldmann Gross, Siderowf, & Hurtig, 2008).

K ATHLEEN L. F UCHS University of Virginia Health System Charlottesville, VA, USA

Definition

▶ Alzheimer’s Disease ▶ Lewy Bodies (Alpha-Synuclein Inclusions) ▶ Metabolic Encephalopathy ▶ Parkinson’s Dementia ▶ Parkinson’s Disease

Demyelination is the process in which myelin, the protective protein and lipid sheath around a nerve fiber, is broken down. This impacts the efficiency of nerve conduction and leaves the axon vulnerable to damage and degeneration. In multiple sclerosis, demyelination in the central nervous system is presumed to result from immune system activation of inflammatory processes that ‘‘attack’’ myelin and inhibit repair (remyelination) by oligodendrocytes. Axonal damage and loss are thought to produce some of the symptoms of multiple sclerosis, especially in myelin-rich fibers such as the optic nerve.


Cross References

Cross References

Goldmann Gross, R., Siderowf, A., & Hurtig, H. I. (2008). Cognitive impairment in Parkinson’s disease and dementia with lewy bodies: A spectrum of disease. Neurosignals, 16(1), 24–34.

▶ Multiple Sclerosis ▶ Myelin ▶ Neuron

Dentate Gyrus

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Compston, A., Lassmann, H., & Smith, K. (2006). The neurobiology of multiple sclerosis. In A. Compston et al. (Eds.), McAlpine’s multiple sclerosis (4th ed., pp. 449–490). Philadelphia, PA: Elsevier.

American Psychiatric Association (1994). Diagnostic and statistical manual of mental disorders (4th edn.). Washington, DC: American Psychiatric Association. Caplan, B. (2010). Rehabilitation psychology and neuropsychology with stroke survivors. In R. G. Frank, M. Rosenthal, & B. Caplan (Eds.), Handbook of rehabilitation psychology (2nd edn., pp. 63–94). Washington DC: American Psychological Association. Prigatano, G. P., & Klonoff, P. S. (1998). A clinician’s rating scale for evaluating impaired self-awareness and denial of disability after brain injury. The Clinical Neuropsychologist, 12(1), 56–67.

Denial DAWN E. B OUMAN Drake Center Cincinnati, OH, USA

Definition A psychological coping mechanism used to protect against stressful events. Early conceptualizations by Freud, as well as recent references such as DSM-IV describe this ‘‘automatic psychological process that protects the individual against anxiety and from awareness of internal or external stressors or dangers.’’ (American Psychiatric Association, 1994). Denial can be adaptive or maladaptive, depending upon the context and extent to which it is used. In neurological impairments, denial of disability is contrasted with anosognosia and unawareness. Simplified conceptualizations view denial as a psychological process that occurs independently of cognitive impairment, while anosognosia is viewed as a problem of insight that is neurologically and cognitively mediated. More comprehensive conceptualizations consider both denial and impaired selfawareness as ‘‘continuous variables which may interact in a given individual’’ following brain damage (Prigatano & Klonoff, 1998). One scale used to tease apart and describe the constructs of denial of disability and impaired self-awareness is described by Prigatano and Klonoff (1998).

Dentate Gyrus S EVERN B. C HURN Virginia Commonwealth University Richmond, VA, USA

Synonyms Gyrus dentata

Definition One of the two stratified folds of gray matter comprising the hippocampus, the dentate gyrus is situated between the major cortical connection to the hippocampus, the entorhinal cortex, and the other hippocampal regions CA1, CA2, and CA3. Granule cells of the dentate granular layer receive one of the two major entorhinal outputs, the ‘‘perforant pathway,’’ and their excitatory axons (‘‘mossy fibers’’) relay mostly to interneurons but also to CA3 and possibly CA2. Neural progenitor cells of the subgranular layer enable neurogenesis, a perpetual process believed to support learning and memory, be enhanced by exercise and negatively influenced by stress, depression, and aging.

Cross References ▶ Hippocampus

Cross References ▶ Anosognosia ▶ Anosodiaphoria ▶ Coping ▶ Insight, Effects on Rehabilitation

References and Readings Scharfman, H. E. (Ed.). (2007). The dentate gyrus: A comprehensive guide to structure, function, and clinical implications: Vol. 163. Progress in brain research. Amsterdam: Springer.

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Deoxyribonucleic Acid (DNA)

Deoxyribonucleic Acid (DNA) R OHAN PALMER 1, M ARTIN H AHN 2 1 University of Colorado Boulder, CO, USA 2 William Paterson University Wayne, NJ, USA

Synonyms Allele; Chromosome; Gene

Definition Deoxyribonucleic acid (DNA) is a double-stranded polynucleotide chain that encodes genetic information responsible for the development and functioning of organisms.

Current Knowledge

specific protein. A gene may also include regulatory elements that determine when expression occurs. Within a cell, DNA is organized into structures called chromosomes that are located within the nucleus of the cell. Human somatic cells contain 23 pairs of homologous chromosomes and one pair of sex chromosomes. The DNA within a cell is accessed by unfolding the double helix. Once unfolded, depending on the process at hand, DNA can either be replicated by a process called DNA replication, or genes may be expressed by the cooperation of two process, transcription and translation. DNA replication is the reproduction of DNA to create a copy. For gene expression to occur, DNA is first transcribed into messenger RNA (ribonucleic acid; mRNA). From each gene, mRNA comprises exons that specify the amino acid sequence. Once complete, mRNA is transported into the cytoplasm of the cell where it is translated to create an amino acid chain. Both DNA replication and gene expression are built around the complimentary base-pair sequencing of the nucleotide. DNA replication is simplified by the phenomenon of complimentary-base pairing because only one strand of the molecule needs to be read. Gene expression is simplified since the mRNA sequence indicates the specific amino acid.

DNA Structure A DNA molecule consists of two polynucleotide chains held together in the shape of a double helix by weak hydrogen bonds (Fig. 1). The polynucleotide strands run in alternate directions. Nucleotides within DNA contain a 50 deoxyribose sugar, a phosphate molecule, and one of the four bases (adenine (A), guanine (G), cytosine (C), and thymine (T)). As a result, DNA is made up of sequences of combinations of the four possible nucleotides. The four DNA nucleotides have specific pairings: adenine pairs with thymine and guanine pairs with cytosine. The number of adenine bases in a DNA molecule is equal to the number of thymine bases; the number of cytosine bases equals the number of guanine bases. Within the helix, nucleotides are stacked upon each other.

DNA Function DNA is referred to as the blueprint of an organism because it codes for the makeup of the entire organism, such as cell components and proteins. A gene is a segment of DNA that determines the amino acid sequence for a

Application in Neuropsychology Twin and adoption studies indicate that most psychiatric disorders, such as schizophrenia and bipolar disorder (Bestelmeyer, Phillips, Crombie, Benson, & St Clair, 2009) are heritable. Neuropsychiatric genetics attempts to understand individual differences in disorder risk by examining differences in genetic risk factors. The ultimate goal of these studies is the development of new drugs to treat the genetic basis of a disorder. For instance, using high-angular resolution diffusion imaging on a sample of young adult twins, Chiang et al. (2009) demonstrated that common genes influence brain fiber architecture and IQ. A future goal of the project is to identify genes involved in the myelination of brain circuits in order to preclude diseases such as multiple sclerosis. Gene expression profiling using high throughput methods is an affordable method used to correlate the expression of genes in specific brain regions with specific disorders. For example, microarray studies of bipolar disorder have implicated several genes (e.g., CASP8, ERBB2, and neuropeptide Y) that influence the risk for bipolar disorder (Bezchlibnyk, Wang, McQueen, &

Deposition

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Base pairs Adenine

Thymine

Guanine

Cytosine

Sugar phosphate backbone

U.S. National Librany of Medicine

Deoxyribonucleic Acid (DNA). Figure 1 Structure of DNA. (This figure is the property of the US National Library of Medicine (2009))

Young, 2001; Konradi et al., 2004; Kuromitsu et al., 2001), as well as the risk for schizophrenia (Tkachev et al., 2003).

References and Readings Bestelmeyer, P. E., Phillips, L. H., Crombie, C., Benson, P., & St Clair, D. (2009). The P300 as a possible endophenotype for schizophrenia and bipolar disorder: Evidence from twin and patient studies. Psychiatry Research, 169, 212–219. Bezchlibnyk, Y. B., Wang, J. F., McQueen, G. M., & Young, L. T. (2001). Gene expression differences in bipolar disorder revealed by cDNA array analysis of postmortem frontal cortex. Journal of Neurochemistry, 79, 826–834. Chiang, M. C., Barysheva, M., Shattuck, D. W., Lee, A. D., Madsen, S. K., Avedissian, C., et al. (2009). Genetics of brain fiber architecture and intellectual performance. Journal of Neuroscience, 29, 2212–2224. Konradi, C., Eaton, M., MacDonald, M. L., Walsh, J., Benes, F. M., & Heckers, S. (2004). Molecular evidence for mitochondrial dysfunction in bipolar disorder. Archives of General Psychiatry, 61, 300–308. Kuromitsu, J., Yokoi, A., Kawai, T., Nagasu, T., Aizawa, T., Haga, S., et al. (2001). Reduced neuropeptide Y mRNA levels in the frontal cortex of people with schizophrenia and bipolar disorder. Brain Research. Gene Expression Patterns, 1, 17–21. Tkachev, D., Mimmack, M. L., Ryan, M. M., Wayland, M., Freeman, T., Jones, P. B., et al. (2003). Oligodendrocyte dysfunction in schizophrenia and bipolar disorder. Lancet, 362, 798–805.

U.S. National Library of Medicine. Structure of DNA. Accessible at: http://ghr.nlm.nih.gov/handbook/basics/dna. Accessed 23 Sep 2009.

Deposition R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition In cases where forensic neuropsychology is involved, a deposition occurs following the determination that the specific psychological or neuropsychological methods are admissible. Written opinions or oral opinions are provided by the expert witness under oath. Such opinions are scrutinized by the opposing counsel and by the trier of fact (e.g., judge or jury). The sworn testimony can be delivered in several ways. First, it can be presented in written form (e.g., affidavit) or orally via a deposition or in the courtroom. A deposition is considered a form of legal discovery and allows for litigants (e.g., their

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attorneys) to question fact or expert witnesses to make decisions regarding the testimony to be presented at the trial. During a deposition, both attorneys and a court recorder are present, but no judge or jury is in attendance. Everything asked and answered during the deposition is transcribed by a court reporter. There are essentially two types of depositions: discovery deposition and trial deposition. A discovery deposition is held by the opposing counsel and is intended to extract information about an expert’s opinions, refine the dispute by focusing on the most relevant issues, and to gain a sense of the expert witness’ demeanor. A trial deposition (de bene esse) serves to preserve an expert witness’ testimony if he/she is not able to appear at trial. A trial deposition and live trial testimony consist of two parts: direct examination and cross-examination. For further details on direct and crossexaminations please see each respective entry.

Cross References ▶ Cross-Examination ▶ Direct Examination

References and Readings Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan, & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Psychology Press. Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts (3rd ed.). New York: Guilford Press.

cerebral blood vessels. Individuals with depressed skull fracture may develop raccoon eyes, or a battle sign that tips the clinician off about the presence of a skull fracture. Depressed skull fracture is common after blunt trauma to the head (i.e., direct blow with a hammer or any other hard object). Individuals with depressed skull fracture are at the increased risk of developing brain infection, as the brain tissue may be directly exposed to the outside environment. As such, emergent management of the skull fracture, with removal of bone fragment and appropriate dressing of the skull wound, is pursued, typically with surgical intervention.

Cross References ▶ Battle Sign ▶ Raccoon Eyes ▶ Skull Fracture

References and Readings Braakman, R. (1972). Depressed skull fracture: Data, treatment, and follow-up in 225 consecutive cases. Journal of Neurology, Neurosurgery, & Psychiatry, 35, 395–402. Graham, D. I., Saatman, K. E., Marklund, N., Conte, V., Morales, D., Royo, N., & McIntosh, T. K. (2006). The neuropathology of trauma. In R. W. Evans (Eds.), Neurology and trauma (2nd ed., pp. 45–94). New York: Oxford University Press. Victor, M., & Ropper, A. H. (2001). Principles of neurology (7th ed., pp. 925–953). New York: McGraw-Hill.

Depressed Skull Fracture B ETH R USH Mayo Clinic Jacksonville, FL, USA

Depression ▶ Depressive Disorder ▶ Dysphoria

Definition Depressed skull fracture is a fracture or break of the cranial bone that results in depression of the bone fragment into the underlying brain tissue.

Current Knowledge This may result in bruising because of compression of the underlying brain tissue, or disruption to the underlying

Depression Equivalent ▶ Masked Depression

Depression Without a Depression ▶ Masked Depression

Depressive Pseudodementia

Depressive Disorder R OBERT G. F RANK Kent State University Kent, OH, USA

Synonyms Affective disorder; Depression; Emotional disorder; Mood disorder

Short Description or Definition A depressive disorder refers to the presence of sad or irritable mood that lasts for more than 2 weeks. In addition, the individual must experience lack or increase in appetite with decrease or increase in weight, insomnia or hypersomnia, feelings of worthlessness, despair, suicidal ideation or intent, guilt, and impaired social and occupational functioning. In the USA in DSM-IV, depressive disorders are divided by severity and duration into major depressive episode and dysthymic disorder.

Epidemiology The National Comorbidity Study (Kessler et al., 1994) reported a prevalence of 17.1% for depressive disorders. Males were less affected (12.7%) than females (21.3%). Dysthymic disorder is less common, affecting 4.8% of men, 8% of women, and 6.4% of the population overall. A more recent replication of the National Comorbidity Study focusing on major depressive disorder (Kesser et al., 2003) found a month prevalence of 6.6% of the population, or about 13.1–14.2 million adult Americans. The majority (59%) of individuals with major depressive episode had significant or very severe role impairment.

Evaluation The assessment and treatment of depressive disorders relies upon clinical evaluation or self-report instruments. Many self-report inventories allow patients to assess their own symptoms. Also, structured clinical interviews based on DSM-IV exist. The majority of these tools focus on major depressive episode, though some provide a blended rating that does not equate to DSM-IV diagnoses.

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Treatment Treatment of depression includes pharmacological and psychotherapeutic interventions. A wide range of antidepressant medications thought to modify the level of neurotransmitters in the postsynaptic cleft. In particular, the neurotransmitters serotonin, norepinephrine, and domanine have been identified as associated with depression. Combined treatment providing psychopharmacological antidepressants and psychotherapy has been shown to be effective (Thase et al., 1997). In the National Comorbidity Study (Kessler et al., 2003), 51.65 of those with 12 month in major depressive episode treatment was adequate in only 41.9% of the cases, resulting in only 21.7% of those with major depressive disorder being treated adequately.

References and Readings American Psychiatric Association (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: American Psychiatric Association. American Psychiatric Association (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: American Psychiatric Association. Kessler, R. C., Berglund, P., Demler, O., Jin, R., Koretz, D., Merikangas, K. R., et al. (2003). The epidemiology of major depressive disorder: Results from the National Comorbidity Survey Replication (NCSR). The Journal of the American Medical Association, 289, 3095–3105. Kessler, R. G., McGonagle, K. A., Zhao, S., Nelson, C. B., Hughes, M., Eshleman, S., et al. (1994). Lifetime and 12 month prevalence of DSM-III-R psychiatric disorders in the United States: Results from the National Comorbidity Study. Archives of General Psychiatry, 51, 8–19. Thase, M. E., Greenhouse, J. B., Frank, E., Reynolds, C. F., Pilkonis, P. A., Hurley, K., et al. (1997). Treatment of major depression with psychotherapy or psychotherapy-pharmcotherapy combinations. Archives of General Psychiatry, 54(11), 1009–1015.

Depressive Disorder Not Otherwise Specified ▶ Minor Depressive Disorder

Depressive Pseudodementia ▶ Pseudodementia

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Dermatome

Dermatome J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition Dermatome is the area of the skin supplied by a spinal sensory nerve root. Since the nerve roots come in pairs, the dermatome on one side of the body is a mirror image of the other (see Fig. 1). The numbers in figure refer to the level at which the nerve roots enter the spinal vertebrae.

Dermatome. Figure 1 Approximate distribution of the segments of the skin represented by the various dorsal roots of the spinal cord

Design Fluency Test

Desensitization ▶ Tachyphylaxis

Design Fluency Test R ONALD RUFF San Francisco Clinical Neurosciences & University of California San Francisco San Francisco, CA, USA

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sum of all perseverations and errors (e.g., drawing with the wrong number of lines or nameable by participant as representative of object). The original scoring criteria yielded interrater reliabilities that were marginal to adequate. However, with more refined criteria, the interrater reliability has improved (Jones-Gotman, 1991; Harter, Hart, & Harter, 1999). Nonetheless, the scoring of this test is challenging. The standardized samples are relatively small for the adults, with 45 adults ranging in age between 14 and 54 years and 10 elderly between 58 and 72 years; however, the pediatric sample of 256 children is larger and ranges between the ages of 5 and 14 (Strauss, Sherman, & Spreen, 2006).

Synonyms

Psychometric Data

DFT

In a sample of college students, the retest reliability following a 1-month interval was r = 0.56 for the total number of drawings in the free condition and r = 0.70 in the fixed condition. As expected, the correlation coefficients were lower for the error scores (Ross, Axelrod, Hank, Kotasek, & Whitman, 1996). Harter, Hart, and Harter (1999), in a different college sample that was tested again 1 month apart, found a slightly higher retest reliability of r = 0.69 for the free condition. As to the concurrent validity, according to Demakis and Harrison (1997), the fixed condition of the DFT is modestly correlated with Ruff Figural Fluency Test (r = 0.38), whereas the correlation with free condition is minimal (r = 0.25).

Historical Background Dr. Brenda Milner is one of the most prominent pioneers for advancing the discipline in neuropsychology. In particular, her seminal research established the neuropsychological assessment of frontal lobe functioning. In collaboration with Dr. Jones-Gotman, Dr. Milner designed the design fluency test (DFT) measure analogous to the Thurston verbal fluency test. Their seminal study (Jones-Gotman & Milner, 1977) documented prefrontal lobe involvement in both verbal and design fluency. Initially, the DFT was introduced as an experimental measure, and over the years, a number of neuropsychologists have contributed toward establishing the DFT as a psychometric tool.

Description In the first part of the DFT, the participant is asked to ‘‘invent drawings’’ that are neither scribbles nor nameable objects or forms (e.g., geometrical shapes). During 5 min, as ‘‘many different’’ designs as possible are to be drawn on a blank piece of paper. During the second part, the participant is given examples and instructions to limit the drawings to four straight or curved lines. Over a period of 4 min, the participants are asked to generate as ‘‘many different’’ four-line drawings as possible. For each condition, there is a novel output score which consists of the total number of unique drawings minus the

Clinical Uses As a research measure, the DFT has contributed to the understanding of frontal lobe functioning. Research studies have shown that patients with right frontal lobe damage are more impaired compared to patients with lesions in other brain regions. There are a variety of neurological conditions that have also demonstrated the DFT’s sensitivity and these include frontal lobe dementias and traumatic brain injuries. However, the DFT has not yet been developed into a solid psychometric test. There is no published test manual available. Reviewing the clinical uses of the DFT, Strauss, Sherman, and Spreen (2006) stated: ‘‘One of the common criticisms of this test. . .has been that the scoring criteria are difficult to interpret and overly subjective’’. Although more detailed administration and scoring criteria have been advanced, the interrater agreement remains suboptimal, especially

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for nameable errors and designs that are drawn with the wrong number of lines. Moreover, Strauss, Sherman, and Spreen, (2006) concluded: ‘‘. . .the norms for older adults are based on a very small sample of individuals and should be used with considerable caution’’. Thus, the DFT is a sensitive measure, but it is difficult to score and the available normative data for adults are suboptimal.

J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Cross References

Desipramine

▶ Delis-Kaplan Executive Function System (D-KEFS) ▶ Figural Fluency Test

Brand Name

Desipramine

Generic Name

Norpramin

References and Readings Boone, K. B., Miller, B. L., Lee, A., Berman, N., Sherman, D., & Stuss, D. T. (1999). Neuropsychological patterns in right versus left frontotemporal dementia. Journal of the International Neuropsychological Society, 5, 612–622. Carter, S. L., Shore, D., Harnadek, M. S., & Kubu, C. S. (1998). Normaltive data and interrater reliability of the Design Fluency Test. The Clinical Neuropsychologist, 12, 531–534. Demankis, G. J., & Harrison, D. W. (1997). Relationships between verbal and nonverbal fluency measures: Implications for assessment of executive functioning. Psychological Reports, 81, 443–448. Elfgren, C. I., & Risberg, J. (1998). Lateralized frontal blood flow increases during fluency tasks: Influence of cognitive strategy. Neuropsychologia, 36, 505–512. Harter, S. L., Hart, C. C., & Harter, G. W. (1999). Expanded scoring criteria for the Design Fluency Test: Reliability and validity in neuropsychological and college samples. Archives of Clinical Neuropsychology, 14, 419–432. Jones-Gotman, M. (1991). Localization of lesions by neuropsychological testing. Epilepsia, 32, S41–S52. Jones-Gotman, M., & Milner, B. (1977). Design fluency: The invention of nonsense drawings after focal cortical lesions. Neuropsychologia, 15, 653–674. Levin, H. S., Culhane, K. A., Hartmann, J., Harword, H., Ringholtz, G., Ewing-Cobbs, L., & Fletcher, J. M. (1991). Developmental changes in performance on tests of purported frontal lobe functioning. Developmental Neuropsychology, 7, 377–395. Ross, T. P., Axelrod, B. N., Hanks, R. A., Kotasek, R. S., & Whitman, R. D. (1996). The interrater and test-retest reliability of the Design Fluency and Ruff Figural Fluency Tests. Paper presented to the 24th meeting of the International Neuropsychological Society, Chicago. Strauss, E., Sherman, E., & Spreen, O. (2006). A compendium of neuropsychological tests (3rd ed.). New York: Oxford University Press. Varney, N. R., Roberts, R. J., Struchen, M. A., Hanson, T. V., Franzen, K. M., & Connell, S. K. (1996). Design fluency among normals and patients with closed head injury. Archives of Clinical Neuropsychology, 11, 345–353. Woodward, J. L., Axelrod, B. N., & Henry, R. R. (1992). Interrater reliability of scoring parameters for the Design Fluency Test. Neuropsychology, 6, 173–178.

Class Tricyclic Antidepressant

Proposed Mechanism(s) of Action Desipramine acts primarily as an inhibitor of the norepinephrine reuptake pump. This results in a boost in neurotransmitter availability in the cleft and, since dopamine is inactivated by norepinephrine reuptake in the prefrontal cortex, it is hypothesized that desipramine also increases to total ambient availability of dopamine as well. At higher doses, it appears to boost presynaptic serotonin production and release.

Indication Depression.

Off Label Use Anxiety, insomnia, neuropathic pain, and treatment resistant depression.

Side Effects Serious Paralytic ileus, hyperthermia, lowered seizure threshold, sudden death, cardiac arrhythmias, tachycardia, QT

Detroit Edison v. NLRB (1979)

prolongation, hepatic failure, mania, and potential for activation of suicidal ideation.

Common Blurred vision, constipation, urinary retention, increased appetite, dry mouth, diarrhea, heartburn, weight gain, fatigue, weakness, dizziness, anxiety, sexual dysfunction, sweating, rash, and itching.

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thought to originate in multipotential cells found in the neural plate in the embryonic stage of human development. In patients younger than 2 years, the desmoplastic ganglioglioma presents as a large, cystic, often dura-attached mass. Although it is usually of low grade, the desmoplastic ganglioglioma can be slow-growing, infiltrating, and become fatal in its progression. Surgery is the treatment of choice, with no complimentary treatment needed in cases of complete tumor resection.

Cross References References and Readings Physicians’ Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson PDR. Stahl, S. M. (2007). Essential psychopharmacology: The prescriber’s guide (2nd ed.). New York, NY: Cambridge University Press.

▶ Brain Tumor ▶ Ganglioglioma ▶ Glioma ▶ Neoplasms

References and Readings Additional Information Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver = 4&task = getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Keating, R. F., Goodrich, J. T., & Packer, R. J. (2001). Tumors of the pediatric central nervous system. New York: Thieme. Pizzo, P. A., & Poplack, D. G. (2005). Principles and practice of pediatric oncology (5th ed.). New York: Lippincott.

Desmoplastic Ganglioglioma ▶ Desmoplastic Gangliocytoma

Desmoplastic Gangliocytoma J ACQUELINE L. C UNNINGHAM The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms

Detroit Edison v. NLRB (1979) R OBERT H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Desmoplastic ganglioglioma

Synonyms

Definition

Disclosure of tests and raw data; Release of psychological test materials; Test security

A variant of gangliogliomas, the desmoplastic ganglioglioma, is a rare intracranial tumor of mixed cell type, containing properties of both glial cells and neuronal cells. It is further characterized by the fibrosis forming in the vascular stroma (framework) of the tumor. The origin and cellular makeup of a desmoplastic gangliocytoma is incompletely understood. However, its derivation is

Historical Background Petitioner employer (Detroit Edison), in response to a request made by a Union (National Labor Relations Board: NLRB) in connection with arbitration of a grievance filed on behalf of employees in a bargaining unit,

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supplied the Union with certain information pertaining to petitioner’s employee psychological aptitude testing program under which certain unit employees had been rejected for certain job openings because of their failure to receive ‘‘acceptable’’ test scores. However, petitioner refused to release the actual test questions, the actual employee answer sheets, and the scores linked with the names of the employees who received them, maintaining that complete confidentiality of these materials was necessary to insure the future integrity of the tests and to protect the examinees’ privacy interests. Petitioner offered to turn over the scores of any employee who signed a waiver releasing petitioner’s psychologist from his pledge of confidentiality, but the Union declined to seek such releases. In unfair labor practice proceedings against petitioner – based on the Union’s charge that petitioner had violated its duty to bargain collectively under 8(a)(5) of the National Labor Relations Act by refusing to provide relevant information needed by the Union for the proper performance of its duties as the employees’ bargaining representative – the NLRB contended that all the requested items were relevant to the grievance and ordered petitioner to turn over all of the materials directly to the Union, subject to certain restrictions on the Union’s use of the information. The Board rejected petitioner’s request that, in order to preserve test secrecy, the tests and answer sheets be turned over to a psychologist selected by the Union. The Board and the Court of Appeals, in its decision enforcing the Board’s order, rejected petitioner’s claim that employee privacy and the professional obligations of petitioner’s industrial psychologists should outweigh the Union’s request for the employee-linked scores. In its decision, the High Court held that the NLRB abused its discretion when it ordered the consulting I/O psychologists to release to the union standardized test questions, answers, and results from psychological aptitude tests.

NLRB decisions (NLRB v. Pfizer, 1985; NLRB v. U.S. Postal Service, 1994) uniformly recognize that discovery of psychological tests is restricted under Detroit Edison v. NLRB and essentially amounts to an ‘‘implied federal common law psychologist nondisclosure privilege.’’ However, in another case (Chiperas v. Rubin, 1998), the Court did not agree that a psychologist had no standing to quash a subpoena (for records) issued to someone who is not a party to the action, ‘‘unless that party (e.g., the psychologist) claims some personal right or privilege with regards to the documents sought.’’ In other words, if the psychologist does not assert a privilege, then there is no basis to object to the release of psychological test materials. Kaufmann (2009) provides a comprehensive review of the issues surrounding psychological and neuropsychological test security and recommended practices and legal arguments against disclosure of test materials. Some of the recommended practices include: (1) informal negotiation and agreement among the parties, (2) file a motion to quash the subpoena, (3) file a motion to intervene as a right, and (4) consider contempt citations carefully. Legal arguments include: (1) assertion of psychological nondisclosure privilege based on state and federal law and (2) assertion of intellectual property restrictions and contractual obligations. Other procedures include: (1) move for protective orders and (2) seeking an in camera review. This elaborates upon previous position papers from neuropsychological organizations (NAN, 2000; 2003; AACN, 2003; 2007) related to issues of test security and disclosure of tests and raw test data.

Cross References ▶ Confidentiality ▶ In Camera Review ▶ Privilege

Current Knowledge According to Kaufmann (2009), there are no U.S. Supreme Court holdings directly addressing whether or not psychologists must release test materials to nonpsychologists or may refuse to release those materials. However, in Detroit Edison v. NLRB, the Court spoke of the public policy of test security for standardized psychological instruments. The Court determined that the right of the psychologists to refuse release of test materials superseded the rights of a Union to discovery of such information. Additional federal appellate cases and

References and Readings American Academy of Clinical Neuropsychology (2003). Official position of the American Academy of Clinical Neuropsychology on ethical complaints made against clinical neuropsychologists during adversarial proceedings. The Clinical Neuropsychologist, 17, 443–445. American Academy of Clinical Neuropsychology (2007). AACN practice guidelines for neuropsychological assessment and consultation. The Clinical Neuropsychologist, 21, 209–231. American Psychological Association (2002). Ethical principles of psychologists and code of conduct. American Psychologist, 57, 1060–1073.

Developmental Delay Bush, S. S., & Martin, T. A. (2006). The ethical and clinical practice of disclosing raw test data: Addressing the ongoing debate. Applied Neuropsychology, 13, 125–136. Detroit Edison Co. v. NLRB, 440 U.S. 301, (U.S. 1979). Freides, D. (1993). Proposed standard of professional practice: Neuropsychological reports display all quantitative data. The Clinical Neuropsychologist, 7, 234–235. Grote, C. (2005). Ethical practice of forensic neuropsychology. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Jaffe v. Redmond, 518 U.S. 1 (1996). Kaufman, P. M. (2009). Protecting raw data and psychological tests from wrongful disclosure: A primer on the law and other persuasive strategies. The Clinical Neuropsychologist, 23, 1130–1159. National Academy of Neuropsychology (2000). Test security: Official statement of the National Academy of Neuropsychology. Archives of Clinical Neuropsychology, 15, 383–386. National Academy of Neuropsychology Policy and Planning Committee (2003). Test security: An update. Official statement of the National Academy of Neuropsychology. See http://www.nanonline.org/NAN/ Files/PAIC/PDFs/NANTestSecurityUpdate.pdf. NLRB v. Pfizer, Inc., 763 F.2d 887 (7th Cir., 1985). NLRB v. U.S. Postal Service, 17 F. 3d 1434 (4th Cir., 1994).

Developmental Coordination Disorder ▶ Soft Signs

Developmental Delay

A developmental delay in a specific domain may occur in isolation or in conjunction with delays in other domains. To recognize a delay and to select an appropriate intervention, clinicians must first have an understanding of developmental norms (Schroeder & Gordon, 2002). It is important to consider that patients may present with a developmental delay as their primary or secondary area of concern, and clinicians should be able to evaluate and recognize such a delay in both contexts. This is especially important in medical settings where a developmental delay may be secondary to a medical condition (Stone, MacLean, & Hogan, 1995). Assessing for the presence of developmental delays is critical in making accurate diagnoses of developmental disorders and formulating subsequent treatment plans. The following chart provides examples of developmental disorders, developmental delays associated with them, and the age parameters based on the DSM-IV-TR criteria (American Psychiatric Association, 2000). To better understand the role of developmental delays within the context of a developmental disorder, a closer look at a specific diagnosis is warranted. For example, as Table 1 depicts, developmental delays in the intellectual and Developmental Delay. Table 1 Developmental disorder

Developmental delays observed Age of onset

Mental Retardation (MR)

Intellectual

Prior to age 3

Communication/ Language Asperger’s Disorder

Social

Rett’s Disorder

Social Typical development Communication/ followed by onset of delays that may begin Language as early as 5 months of Physical age Motor

Childhood Disintegrative Disorder

Social

Developmental retardation

Developmental delay is the failure to achieve certain developmental milestones at the appropriate age. Such delays may occur in one or more of the following areas: intellectual, emotional, social, motor, physical, language/ communication, and adaptive skills.

Prior to age 18

Adaptive

Emotional

L AURA C RAMER-B ERNESS William Paterson University Wayne, NJ, USA

Definition

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Autistic Disorder Social

Synonyms

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No age specified

Emotional

Typical development until at least age 2 and loss of skills prior to Communication/ age 10 Language Emotional

Physical Motor

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adaptive domains are necessary for a diagnosis of mental retardation. As currently defined in the DSM-IV-TR (American Psychiatric Association, 2000), an intellectual quotient (IQ) of less than 70 indicates an intellectual delay. The criteria for delayed adaptive skills are a significant impairment in two or more of the following areas: communication, self-care/home living, social/interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health, and safety. If this combination of developmental delays is present prior to the age of 18, then a diagnosis of mental retardation may be made. It is important to note that there may be an overlap in certain diagnoses. For instance, a patient may be diagnosed with mental retardation and autistic disorder. This comorbidity of disorders would then need to be considered in how to best address the developmental delays associated with each.

Short Description or Definition Developmental Gerstmann syndrome (Gerstmann’s syndrome) is a rare disorder that has been infrequently described in the literature. The descriptions focus on four ‘‘classic’’ deficits: difficulty writing (dysgraphia or agraphia), difficulty with arithmetic (dyscalculia or acalculia), an inability to distinguish left from right, and difficulty identifying fingers (finger agnosia). Constructional dyspraxia, an inability to copy simple figures, is often included as a fifth symptom. The cause is not known, but over the last 10 years, studies using advanced imaging techniques have reported the deficits to be associated with posterior inferior parietal lesions located on the left side of the brain (Miller & Hynd, 2004).

Characterization Cross References ▶ Disability ▶ Intellectual Disability

References and Readings American Psychiatric Association (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: American Psychiatric Association. Schroeder, C. S., & Gordon, B. N. (2002). Assessment & treatment of childhood problems (2nd ed.). New York: Guilford. Stone, W. L., MacLean, W. E., & Hogan, K. L. (1995). Autism and mental retardation. In M. C. Roberts (Ed.), Handbook of pediatric psychology (pp. 655–675). New York: Guilford Press.

Developmental Dyslexia ▶ Dyslexia

Developmental Gerstmann Syndrome B ONNY J. F ORREST San Diego Center for Children San Diego, CA, USA

Synonyms Developmental right parietal syndrome; Nonverbal disorders of learning; Nonverbal learning disability; Right hemisphere Impairment/Disorder

Fewer than 25 cases have been reported in the literature and, in the last 15 years, fewer than five appear to have been described. As a result, there is not yet a consensus about how to define the syndrome. It was first described as a discrete syndrome by Kinsbourne and Warrington (Kinsbourne & Warrington, 1963) who reported on seven children. All presented with left-right confusion, finger agnosia, and constructional dyspraxia, and about half also presented with dyscalculia and dysgraphia. As that description suggests, there is some disagreement in the literature as to whether all four classic deficits must occur for a diagnosis to be reached. (A similar lack of consensus applies to adult cases as well.) At least one author has theorized that the disorder may present in a ‘‘partial’’ form if a child has simply learned to compensate to some degree in a deficit area of the syndrome (PeBenito, 1987).

Epidemiology No epidemiological studies of the syndrome have been reported and some authors have stated that there is ‘‘no clear basis for considering it to be a unique syndrome’’ (Miller & Hynd, 2004).

Natural History, Prognostic Factors, and Outcomes of Gerstmann’s Syndrome Almost all reported cases involve symptoms that could be attributed to other diagnoses, including fragile

Developmental Milestones, Stages

X syndrome, Williams syndrome, nonverbal or right hemisphere learning disabilities, or autism spectrum disorders. Most cases are identified when children reach school age, a time when they are challenged with writing and math exercises. Although it has been suggested that children’s symptoms may diminish over time, it appears likely that most children probably do not overcome their deficits, but rather learn to adjust to them.

Neuropsychology and Psychology of Developmental Gerstmann Syndrome As noted above, the cluster of neuropsychological features described in Gerstmann syndrome may represent soft neurological signs associated with a number of neurodevelopmental disorders. In addition to the four classic deficits previously described and constructional dyspraxia, other symptoms frequently co-occur with Gerstmann’s syndrome. These include average or better intelligence, elevated verbal skills, average or better reading abilities, negative family history for learning disabilities, lack of clinically significant delays in language, and no history of a head injury. A number of theories have been put forth to account for the cluster of symptoms observed in the syndrome. Kinsbourne and Warrington, referencing adult cases with lesions in the parietal lobes of the left hemisphere, postulated that in children the symptoms were likely the result of a developmental learning disorder (Kinsbourne & Warrington, 1963). Other authors have speculated that the syndrome occurs as a result of a neurological abnormality (Shaley & Gross-Tsur, 1993), or abnormal developmental processes (Semrud-Clikeman & Hynd, 1990). Given the lack of consensus about the definition, and the resulting paucity of cases, there has been no clear explanation of the syndrome’s ‘‘cause’’ although, as Kinsbourne and Warrington hypothesized, the deficits appear to be associated with left-sided posterior inferior parietal lesions (Miller & Hynd, 2004).

Treatment There is no cure for Gerstmann’s syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may help diminish the dysgraphia and apraxia. In addition, calculators and word processors may help school children cope with the symptoms of the disorder.

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References and Readings Kinsbourne, M, & Warrington, E. K. (1963). The developmental Gerstmann syndrome. Archives of Neurology, 8(5), 490–501. Miller, C. J., & Hynd, G. W. (2004). What ever happened to developmental Gerstmann’s syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes. Journal of Child Neurology, 19 (4):282–289. PeBenito, R. (1987). Developmental Gerstmann syndrome: Case report and review of the literature. Developmental Behavioral Pediatrics, 8(4), 229–232. Semrud-Clikeman, M., & Hynd, G. W. (1990). Right hemispheric dysfunction in nonverbal learning disabilities: Social, academic, and adaptive functioning in adults and children. Psychological Bulletin, 107, 198–209. Shaley, R. S., & Gross-Tsur, V. (1993). Developmental dyscalculia and medical assessment. Journal of Learning Disabilities, 26, 134–137.

Developmental Milestones, Stages L EESA V. H UANG 1, S ARAH F REEMAN 2 1 California State University Chico, CA, USA 2 San Jose Unified School District San Jose, CA, USA

Synonyms Developmental stages

Definition Developmental milestones are a set of functional skills that a majority of children have acquired during a certain age range. Infants and children develop skills in five main areas: cognition, social and emotional, speech and language, fine motor, and gross motor. All milestones develop sequentially; that is, a child will need to develop some basic skills before developing more advanced skills. Although each stage has a specific time frame, the actual age at which an individual child may successfully demonstrate a skill varies widely. Therefore, developmental milestones should be viewed as guidelines for the development of age-appropriate skills. These age-specific tasks allow educational and medical professionals to determine how a child is progressing and what areas may need additional support or intervention.

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Developmental Psychology

In relation to neuropsychology, development involves two main phases of neurological organization. The first stage commences at the time of conception and consists of neurulation, proliferation, migration, and differentiation. The second phase is characterized by the reorganization of the cortex, which begins during gestation and may continue through young adulthood (second decade of life). Dendritic and axonal growth, synapse production, neuronal and synaptic pruning, and changes in neurotransmitter sensitivity occur. Due to the rapid growth of the brain during the first several years, developmental milestones assist practitioners in the prediction of when certain skills develop in infants and children.

Developmental Test of Visual Motor Integration ▶ Beery Developmental Test of Visual-Motor Integration (VMI)

Dexamethasone DAVID J. L IBON Drexel University, College of Medicine Philadelphia, PA, USA

References and Readings Definition Berk, L. E. (2006). Child development (7th ed.) New York: Pearson/Allyn and Bacon. Webb, S. J., Monk, C. S., & Nelson, C. A. (2001). Mechanisms of postnatal neurobiological development: Implications for human development. Developmental Neuropsychology, 19, 147–171.

Developmental Psychology ▶ Normal Aging

Dexamethasone is a medication used to diagnose and treat medical as well as psychiatric conditions. Dexamethasone is often used to block inflammation. As such dexamethasone can be used as an anti-inflammatory agent to treat a number of medical conditions including a wide array of auto-immune disorders. Dexamethasone can also be used to assess for negative feedback regarding the ability of the pituitary gland to suppress adrenocortical activity.

Current Knowledge

Developmental Retardation ▶ Developmental Delay

Developmental Right Parietal Syndrome ▶ Developmental Gerstmann Syndrome ▶ Nonverbal Learning Disabilities

Developmental Stages ▶ Developmental Milestones, Stages

As such, the dexamethasone suppression test (DST) was developed to diagnosis medical conditions such as Cushing disease. However, the DST has also been used to diagnose major depression (Carroll, Martin, & Davies, 1968). The rationale for the diagnostic use of the DST to treat major depression was initially based on observations of increased adrenocortical activity in depression. The inability to suppress adrenocortical activity has been used as a means to diagnose depression and related psychiatric illnesses. Data on the efficacy of the DST has been mixed; however, recent data provided by Fountoulakis et al. (2008) suggests that under certain circumstances the DST may be helpful to better characterize patients with major depression.

Cross References ▶ Hypothalamus

DFT


Off Label Use

Carroll, B. J., Martin, F. I., & Davies, B. (1968). Resistance to suppression by dexamethasone of plasma 11-O.H.C.S. levels in severe depressive illness. British Medical Journal, 3, 285–287. Fountoulakis, K. N., Gonda, X., Rihmer, Z., Fokas, C., & Iacovides, A. (2008). Revisiting the Dexamethasone Suppression Test in unipolar major depression: an exploratory study. Annals of General Psychiatry, 7, 22–31.

Narcolepsy and depression (treatment-resistant).

Dexedrine ▶ Amphetamine ▶ D-Amphetamine

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Side Effects Serious Psychotic episodes, seizures, neuroleptic malignant syndrome, hypomania, mania, and possible suicidal ideation.

Common Insomnia, irritability, tremor, anxiety, and tics (exacerbation, dizziness, anorexia, blurred vision).

Dexmethylphenidate J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metarie, LA, USA


Generic Name Additional Information Dexmethylphenidate

Brand Name Focalin, Focalin XR

Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver = 4&task = getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Class Stimulant


Dextroamphetamine ▶ Amphetamine ▶ D-Amphetamine

Dexmethylphenidate facilitates the release and blocks the reuptake of both norepinephrine and dopamine.

Indication Attention-deficit hyperactivity disorder.

DFT ▶ Design Fluency Test

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Diabetes Mellitus

Diabetes Mellitus G EORGE D EMAKIS University of North Carolina Charlotte Charlotte, NC, USA

Synonyms Type 1 diabetes, previously termed juvenile-onset or insulin-dependent diabetes; Type 2 diabetes, previously termed non-insulin-dependent diabetes or adult-onset diabetes

Short Description or Definition Diabetes mellitus is a complex set of metabolic disorders involving chronically high levels of blood glucose (hyperglycemia) secondary to the impaired production of the hormone, insulin. Classic symptoms include polyuria (excessive urine), polydipsia (intense thirst), polyphagia (intense hunger), and fatigue.

Categorization There are two main types of diabetes: Type 1 diabetes is much less common, accounting for about 10% of all diagnosed cases, and is characterized by an inability to secrete insulin due to destruction of the beta cells of the pancreas. This type of diabetes usually is diagnosed before the age 20. The more common Type 2 typically occurs in adulthood and results from the development of insulin resistance, which can result in chronic hyperglycemia. Risk factors for Type 2 diabetes include heredity, age, and obesity.

Epidemiology Diabetes affects over 20 million individuals in the United States. Men, individuals older than 60, and ethnic minorities are more likely to be affected.

Natural History, Prognostic Factors, Outcomes: Emphasis on Neuropsychological and Psychological Issues Acute complications of diabetes may include hypoglycemia and ketoacidosis, whereas chronic complications may

include stroke, cardiovascular disease and hypertension, nephropathy, retinopathy, neuropathy, and foot difficulties. Considerable research has also evaluated the effect of diabetes on cognitive functioning. This research is characterized by considerable variety in the sample’s age, gender, duration of diabetes and comorbid conditions, methodological approaches including cross-section and longitudinal designs, and neuropsychological measures employed (Ryan, 2001). In a review of many studies with children and adolescents with Type 1 diabetes, Desrocher and Rovet (2004) concluded that a variety of cognitive deficits were associated with an early onset (prior to 5 years of age), recurrent episodes of hypoglycemia, and longer duration of illness. Though not observed in all studies, cognitive deficits tended to be observed in areas such as motor functioning, attention, memory, and set-shifting, and in general, were judged to be rather small. Awad, Gagnon, and Messier (2004) conducted a meta-analytic review of studies that compared adults with treated Type 2 diabetes with controls. Across the various studies, the diabetic groups tended to perform somewhat worse on measures of verbal memory and processing speed. There were either minimal or no differences across the studies on measures of verbal and nonverbal memory, attention, visuospatial functioning, and executive functioning. Worse performance by diabetic samples was associated with older age, poorer glycemic control, and insulin treatment as opposed to oral hypoglycemic treatment or diet. A meta-analysis of the effect of Type 1 diabetes on cognition in adults (Brands, Biessels, de Haan, Kappelle, & Kessels, 2005) found that individuals with diabetes scored significantly lower than non-diabetic controls on a variety of cognitive measures. Poorer cognitive performance was associated with microvascular complications (neuropathy or retinopathy), but not with the occurrence of severe hypoglycemic episodes or poor metabolic control. Despite these findings, it is important to note that some studies have not found cognitive differences between diabetics and non-diabetes (e. g., Jacobson et al., 2007), and that if cognitive impairment does exist, it usually tends to be relatively minor. What is the cause or mechanism of these neuropsychological deficits? Glucose is the primary energy substrate of the brain and normal brain structure and function are dependent on its metabolic control. Hypoglycemia, if left untreated, can lead to seizures, loss of consciousness, brain damage, and even death. Hyperglycemia, if left untreated, can be associated with vascular damage, leading to a variety of complications including stroke, heart attack, and hypertension. As an example of research in this area, Jongen, van der Grond, Kappelle, Biessels, Viergever, and Pluim (2007), using magnetic resonance imaging (MRI) of the brain,

Diagnosis

found that Type 2 diabetics had smaller volume gray matter, larger lateral ventricle volume, and a larger white matter lesion volume than those in the controls. This same research group found that cognitive functioning, particularly attention/executive functioning and information processing speed, was inversely related to white matter lesions, brain atrophy, and the presence of infarcts (Manschot et al., 2006). In addition to these neuropsychological issues, it is not surprising that diabetes is associated with higher levels of psychological distress than in controls (Ryan, 2001). For instance, in a meta-analysis, Anderson, Clouse, Freedland, and Lustman (2001) found that prevalence rates of depression varied widely across studies (3–60%, but with most studies around 20%), and that Type 1 and Type 2 diabetics were twice as likely to be depressed as the controls. The prevalence of comorbid depression was higher in women than in men, in clinical versus community samples, and when assessed via self-report versus standardized diagnostic interviews. When depression has been evaluated in diabetes populations, Watari et al. (2006) found that depressed Type 2 diabetics performed more poorly than non-depressed diabetes and controls in cognitive domains of attention and executive functioning. In contrast, Brands et al. (2007) found no relationship between selfreport measures of psychological distress and cognition in individuals with type 2 diabetes.

Evaluation A variety of relatively minor cognitive deficits can be associated with diabetes and, as such, any neuropsychological measure used in this population should be quite sensitive to cognitive impairment. Domains to be assessed by the neuropsychologist should include, at minimum, verbal memory and learning, processing speed, attention, setshifting, and motor functioning.

Treatment Treatment for Type 1 includes insulin injections, whereas for Type 2 exercise and diet, oral hypoglycemic agents, and, if necessary, insulin is prescribed.

Cross References ▶ Hypoglycemia

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References and Readings Anderson, R. J., Clouse, R. E., Freedland, K. E., & Lustman, P. J. (2001). The prevalence of comorbid depression in adults with diabetes: A meta-analysis. Diabetes Care, 24, 1069–1078. Awad, N., Gagnon, M., & Messier, C. (2004). The relationship between impaired glucose tolerance, Type 2 diabetes, and cognitive function. Journal of Clinical and Experimental Neuropsychology, 26, 1044–1080. Brands, A. M. A., Biessels, G. J., de Haan, E. H. F., Kappelle, L. J., & Kessels, R. P. C. (2005). The effects of Type 1 diabetes on cognitive performance: A meta-analysis. Diabetes Care, 28, 726–735. Brands, A. M. A., van den Berg, E., Manschot, S. M., Biessels, G. J., Kappelle, L. J., de Haan, E. H. F., & Kessels, R. P. C. (2007). A detailed profile of cognitive dysfunction and its relation to psychological distress in patients with type 2 diabetes mellitus. Journal of the International Neuropsychological Society, 13, 288–297. Desrocher, M., & Rovet, J. (2004). Neurocognitive correlates of Type I diabetes mellitus in childhood. Child Neuropsychology, 10, 36–52. Jacobson, A. M., Musen, G., Ryan, C. M., Silvers, N., Cleary, P, Waberski, B., Burwod, A., Weinger, K., Bayless, M., Dahms, W., Harth, J., & The Diabetes Control and Complications Trial/Epidemiology of Diabetes Interventions and Complications Study Research Group. (2007). Long-term effect of diabetes and its treatment on cognitive function. The New England Journal of Medicine, 356, 1842–1852. Jongen, C., van der Grond, J. V. D., Kappelle, L. J., Biessels, G. J., Viergever, M. A., & Pluim, J. P. W. (2007). Automated measurement of brain and white matter lesion volume in type 2 diabetes mellitus. Diabetologia, 50, 1509–1516. Manschot, S. M., Brands, A. M. A., van der Grond, J., Kessels, R. P. C., Algra, A., Kappelle, L. J., & Biessels, G. J. (2006). Brain magnetic resonance imaging correlates of impaired cognition in patients with type 2 diabetes. Diabetes, 55, 1106–1113. Ryan, C. M. (2001). Neurobehavioral disturbances associated with disorders of the pancreas. In R. E. Tarter, M. Butters, & S. R. Beers (Eds.), Medical neuropsychology (2nd ed., pp. 127–162). New York: Kluwer Academic/Plenum. Watari, K., Letamendi, A., Elderkin-Thompson, V., Haroon, E., Miller, J., Darwin, C., & Kumer, A. (2006). Cognitive function in adults with type 2 diabetes and major depression. Archives of Clinical Neuropsychology, 21, 787–796.

Diabetic Retinopathy ▶ Retinopathy

Diagnosis ▶ Behavior Assessment System for Children (BASC)

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Diagnostic Dyspraxia

Diagnostic Dyspraxia ▶ Alien Hand Syndrome

Diagnostic Interview

Originally coined by von Monakow in 1914, the term ‘‘diaschisis’’ currently is used to describe a depression of regional neuronal metabolism and cerebral blood flow caused by dysfunction in an anatomically separate but functionally related region of the brain. It is important to note that diaschisis is a dysfunction of tissue that is not damaged but for which the blood supply has been reduced because of interruption of neurons that are connected to it.

▶ Clinical Interview

Cross References

Diaschisis E LLIOT J. R OTH Northwestern University Chicago, IL, USA

▶ Cerebral Blood Flow ▶ Ischemic Penumbra ▶ Ischemic Stroke

References and Readings Definition Diaschisis is a sudden loss of function in a portion of the brain connected to, but at a remote distance away from, a damaged area.

Current Knowledge The site of the area that has been injured by an acute focal disturbance such as stroke or penetrating brain injury, and the site of the diaschisis are connected to each other by neurons. The loss of the damaged structure disrupts the function of the remaining intact systems and causes a physiological imbalance. These changes are most pronounced during the first few days following cerebral infarction or injury. Some function may be restored with a gradual readjustment of the otherwise intact but suppressed areas. Diaschisis can be classified according to the connecting neuronal fibers involved. When the connecting fibers are intra-hemispheric, the phenomenon of ipsilateral thalamic or subcortical-cortical diaschisis may be seen; when they are interhemispheric, there is transcallosal diaschisis, and if they are cerebellar, the diaschisis is of the contralateral cerebellum or crossed cerebellar diaschisis. Ipsilateral thalamic and crossed cerebellar diaschisis are frequently observed, but have no clinical significance. Reversal of the effects of the subcortical-cortical and transcallosal diaschisis might explain the neuropsychological and functional neuroimaging changes observed over the first few months after the cerebrovascular incident.

Finger, S., Koehler, P. J., & Jagell, C. (2004). The Monakow concept of diaschisis: Origins and perspectives. Archives of Neurology, 61, 283–288. Infeld, B., Davis, S. M., Lichtenstein, M., Mitchell, P. J., & Hopper, J. L. (1995). Crossed cerebellar diaschisis and brain recovery after stroke. Stroke, 26, 90–95. Komaba, Y., Mishina, M., Utsumi, K., Katayama, Y., Kobayashi, S., & Mori, O. (2004). Crossed cerebellar diaschisis in patients with cortical infarction: Logistic regression analysis to control for confounding effects. Stroke, 35, 472–476.

Diazepam J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Diazepam

Brand Name Valium, Diastat

Class Anxiolytic

Dichotic Listening

Proposed Mechanism(s) of Action Binds to benzodiazepine receptors at the GABA-A ligandgated channel, thus allowing for neuronal hyperpolarization. Benzodiazepines enhance the inhibitory action of GABA via boosted chloride conductance. Diazepam also produces inhibitory action in the spinal cord and may provide therapeutic benefits for muscle spasms.

Indication Anxiety disorder, symptoms of anxiety, acute agitation, muscle spasm, spasticity caused by upper motor neuron disorders, athetosis, stiff-man syndrome, convulsive disorders (adjunctive), preoperative anxiety, and initial treatment of status epilepticus.

Off Label Use Insomnia

Side Effects Serious Respiratory depression, hepatic dysfunction (rare), renal dysfunction and blood dyscrasias, Grand mal seizures

Common Sedation, fatigue, depression, dizziness, memory problems, disinhibition, confusion, ataxia, slurred speech

References and Readings Physicians’ Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson PDR. Stahl, S. M. (2007). Essential psychopharmacology: the prescriber’s guide (2nd ed.). New York, NY: Cambridge University Press.


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Dichotic Listening M ELISSA A MICK VA Boston Healthcare System Boston, MA, USA

D Description In the dichotic listening – words test (Hayden & Spellacy, 1978), the participant hears three pairs of one-syllable words (one presented to each ear simultaneously). Each word pair begins with the same consonant (see Strauss, Sherman, & Spreen, 2006, for examples). The audiotape is played through a cassette recorder and headphones. The stimuli presented to the left ear are recorded on the left channel of the tape, and played through the left headphone. The reverse arrangement is used for the right ear. According to Strauss et al. (2006), the earphones should be calibrated to ensure that each is producing approximately 65–70 dB for each ear. It is also recommended that the earphones be reversed halfway through testing, to avoid the possible effect of poor earphone calibration. Discrepancies in hearing between ears would create an advantage for the intact ear, and if hearing differs significantly between ears, the test should not be administered. There are other versions of the dichotic listening task. Another common procedure is to use stop consonant vowel syllable pairs (e.g., /ba/, /da/, /ga/) rather than words. The participant’s task is to identify the one syllable heard clearly (Hugdahl, Carlsson, Uvebrant, & Lundervold, 1997). Dichotic listening has also been examined using the Fused Dichotic Word Test (Hiscock, Cole, Benthall, Carlson, & Ricketts, 2000). The stimuli employed for this dichotic listening task are rhyming consonant vowel consonant pairs (e.g., hear–fear, cage– gage). A forced choice response is required from four possible options. Attention can also be manipulated in dichotic listening tasks. In forced attention conditions, the participant is instructed to report stimuli presented to a specified ear, while in the unforced condition, participants are not directed to attend to either ear (Gadea et al., 2002).

Historical Background Early studies with the dichotic listening task revealed a right ear advantage (REA, inferred left hemisphere

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Dichotic Listening

advantage) for spoken words (digits). Kimura (1961a, b, 1967) observed that patients with damage to the left temporal lobe performed more poorly than patients with right temporal lobe damage as well as adult control participants. These findings were expected as patients with damage to the left hemisphere should have more difficulty performing language-based tasks relative to the other groups. Surprisingly, all groups demonstrated better detection for information presented to the right relative to the left ear. The REA was initially unexpected because each ear projects to the contralateral and ipsilateral hemisphere. Based on these redundant connections, each ear should have access to the language center. Kimura reasoned that a REA occurred because the ipsilateral pathway is suppressed during dichotic but not monaural stimuli presentation. According to her model, in patients with speech localized to the left hemisphere, information from the left ear is sent to the right hemisphere and then through the cerebral commissures before gaining access to the left hemisphere for processing of the verbal material. By contrast, the right ear projects to the left hemisphere (without the additional step of crossing over the cerebral commissure) that results in a REA (see Kimura, 1967 for review). Further support for Kimura’s model of dichotic listening comes from work done with patients who have undergone the intracarotid sodium amytal procedure (Wada test) to determine which hemisphere mediates language. Unlike patients with language lateralized to the left hemisphere with the typical REA, patients who have language lateralized to the right hemisphere showed a left ear advantage (LEA, Kimura, 1961b, 1967). Data from patients with complete disconnection of the hemispheres via commissurotomy have also been cited as support for Kimura’s model. Split-brain patients are able to identify spoken words presented monaurally, indicating that the ipsilateral pathway is intact in the absence of competing information. However, under dichotic presentation, an exaggerated REA occurs. In fact, seven out of ten patients with a commissurotomy detected no information presented to the left ear (Milner, Taylor, & Sperry, 1968), and this extinction of information was also described in two patients with complete disconnection of the hemispheres (Springer & Gazzaniga, 1975). According to Kimura’s model, the extinction of information occurs because information presented to the left ear is projected to the disconnected right hemisphere. The right hemisphere is not specialized to process this type of verbal material, and this information cannot be projected to the left hemisphere because the corpus callosum is cut. Consequently, split-brain patients are not able to report hearing information presented to the left ear. Similarly,

patients with complete hemispherectomy are able to process auditory material presented monaurally but are impaired under dichotic presentation. Specifically, these patients are able (74–89% accuracy) to detect dichotic information in the ear contralateral to the hemispherectomy but accuracy was extremely poor (3–12%) for the ear ipsilateral to the lesion (de Bode, Sininger, Healy, Mathern, & Zaidel, 2007). An attentional model has also been proposed to explain the REA observed on dichotic word listening tasks. Kinsbourne (1975) proposed that a REA occurs because attention is shifted toward the contralateral side of space following engagement of a hemisphere. For example, a REA occurs during dichotic word listening because the left hemisphere is engaged for processing verbal material, and the activation of the left hemisphere leads to a shift of attention toward information presented to the right side of space (see Springer & Deutsch, 1985 for review). In support of the attentional model, a double dissociation was found in dichotic listening depending upon the type of material (verbal or melodic) that had to be remembered across trials. That is, a REA was found when participants had to remember a sentence across dichotic trials and a modest LEA (as measured by reaction time) occurred when subjects compared melodies presented just before and immediately following dichotic syllable pairs (Morais & Landercy, 1977). According to the attentional model, activation of the right hemisphere during melodic processing would focus attention to the left side of space creating the LEA.

Psychometric Data Strauss et al. (2006) report normative data for children (age 2 through grade 6) as well as mean scores for righthanded adults. The authors caution that the adult norms are based upon university students and therefore are only applicable for younger adults. Normative data for older adults is not available for this particular task. There are published means and standard deviations for adults ranging in age from 16 to 79, split into decades (Meyers, Roberts, Bayless, Volkert, & Evitts, 2002) using a similar task with more word pairs (Dichotic Word Listening Test, Roberts et al., 1994). Generally, normative data for dichotic listening tasks are not presented separately for men and women, as it is thought that gender does not significantly influence dichotic listening performance. A recent meta-analysis of 12 studies involving a total of 3,822 subjects concluded that there were no significant differences between sexes in terms of dichotic listening

Dichotic Listening

performance (Sommer, Aleman, Somers, Boks, & Kahn, 2008). Test–retest reliability for dichotic listening word pairs is variable. Hiscock and colleagues (2000) report a reliability of 0.45 with a 1 week interval between testing. This was conducted with a sample of 340 right- and left-handed college students. Reliability did improve when more trials were added (0.76). Test–retest reliability has been reportedly higher (0.76–0.92) when testing was performed in the same day with a smaller sample of epileptic patients (Strauss, Gaddes, & Wada, 1987). According to Kimura’s model, the ear advantage on dichotic word listening tasks should indicate which hemisphere is dominant for language. Test validity data is derived from patients who have completed both the dichotic listening task and undergone the Wada test to determine which hemisphere mediates language. Hugdahl and colleagues (1997) reported a REA for 8/10 participants who demonstrated left hemisphere language dominance, whereas all three patients with right hemisphere language dominance showed a LEA. Similarly, in a larger sample of epileptic patients, a REA was found in the patient group with left hemisphere language dominance, whereas patients with right hemisphere language dominance reported words presented to the left ear more accurately than patients with left or bilateral hemisphere language dominance (Strauss et al., 1987). These findings are also consistent with the original studies reported by Kimura (1961b, 1967) described above. High concordance has also been found for fused dichotic word listening and lateralized fMRI activation during a verb generation task performed by children with epilepsy. All eight patients with greater left than right hemisphere activation during the verb generation task showed a REA, whereas bilateral activation was associated with a REA in three of the patients and no ear advantage in the other three (Fernandes, Smith, Logan, Crawley, & McAndrews, 2006). As seen above, dichotic listening performance is not always predictive of the hemisphere that mediates language. A significant factor to consider is that patients who undergo Wada testing have epilepsy or other brain abnormalities, which can effect brain organization and the lateralization of different cognitive abilities.

Clinical Uses Dichotic listening is used frequently with patients who have epilepsy or focal brain tumors, as described above (see Historical Background and Psychometric Data). This task also has clinical utility with other neurological

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and psychiatric populations. Patients with multiple sclerosis (MS) also show abnormal dichotic listening performance. For example, relative to control participants, patients with MS have been observed to demonstrate a greater REA in unforced and right ear forced attention conditions. By contrast, in the left ear forced attention condition, the control group demonstrated the expected LEA, while the MS group persisted in demonstrating a REA (Gadea et al., 2002). Patients with other neurodegenerative diseases can also show an exaggerated REA relative to a neurologically normal comparison group. This has been observed in patients with very mild and mild Alzheimer’s disease (Duchek & Balota, 2005; Gootjes et al., 2006, Claus & Mohr, 1996). Additionally, patients with Alzheimer’s disease and Huntington’s disease have been found to demonstrate a REA even under left ear forced attention conditions, whereas demented and non-demented patients with Parkinson’s disease are able to shift attention to the left ear and show the expected LEA (Claus & Mohr, 1996). Dichotic listening has also been examined in patients with depression and schizophrenia, disorders often associated with right hemisphere dysfunction. An exaggerated REA in depressed individuals has been associated with response to antidepressant treatment (see, Bruder et al., 1999 for review), and patients with schizophrenia also show a REA even under left ear forced attention conditions (Hugdahl et al., 2003).

Cross References ▶ Auditory Cortex ▶ Auditory Pathway ▶ Auditory Processing ▶ Auditory System ▶ Intracarotid Sodium Amobarbital Test ▶ Split-Brain ▶ Wada Test

References and Readings Bruder, G. E., Tenke, C. E., Stewart, J. W., McGrath, P. J., & Quitkin, F. M. (1999). Predictors of therapeutic response to treatments for depression: A review of electrophysiologic and dichotic listening studies. CNS Spectrums, 4, 30–36. de Bode, S., Sininger, Y., Healy, E. W., Mathern, G. W., & Zaidel, E. (2007). Dichotic listening after cerebral hemispherectomy: Methodological and theoretical observations. Neuropsychologia, 18, 2461–2466. Claus, J. J., & Mohr, E. (1996). Attentional deficits in Alzheimer’s, Parkinson’s, and Huntington’s diseases. Acta Neurologica Scandinavica, 93(5), 346–351.

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Duchek, J. M., & Balota D. A. (2005). Failure to control prepotent pathways in early stage dementia of the Alzheimer’s type: Evidence from dichotic listening. Neuropsychology, 19, 687–695. Fernandes, M. A., Smith, M. L., Logan, W., Crawley, A., & McAndrews, M. P. (2006). Comparing language lateralization determined by dichotic listening and fMRI activation in frontal and temporal lobes in children with epilepsy. Brain and Language, 96, 106–114. Gadea, M., Marti-Bonmatı´, L., Arana, E., Espert, R., Casanova, V., & Pascual, A. (2002). Dichotic listening and corpus callosum magnetic resonance imaging in relapsing-remitting multiple sclerosis with emphasis on sex differences. Neuropsychology, 16, 275–281. Gootjes, L., Bouma, A., Van Strien, J. W., Van Schijndel, R., Barkhof, F., & Scheltens, P. (2006). Corpus callosum size correlates with asymmetric performance on a dichotic listening task in healthy aging but not in Alzheimer’s disease. Neuropsychologia, 44, 208–217. Hayden, S., & Spellacy, F. (1978). Dichotic word listening test. Neuropsychology Laboratory, Department of Psychology, University of Victoria. Hiscock, M., Cole, L. C., Benthall, J. G., Carlson, V. L., & Ricketts, J. M. (2000). Toward solving the inferential problem in laterality research: Effects of increased reliability on the validity of the dichotic listening right-ear advantage. Journal of the International Neuropsychological Society, 6, 539–547. Hugdahl, K., Carlsson, G., Uvebrant, P., & Lundervold, A. J. (1997). Dichotic-listening performance and intracarotid injections of amobarbital in children and adolescents. Preoperative and postoperative comparisons. Archive of Neurology, 54, 1494–1500. Hugdahl, K., Rund, B. R., Lund, A., Asbjørnsen, A., Egeland, J., Landrø, N. I., et al. (2003). Attentional and executive dysfunctions in schizophrenia and depression: evidence from dichotic listening performance. Biological Psychiatry, 53, 609–616. Kimura, D. (1961a). Some effects of temporal-lobe damage on auditory perception. Canadian Journal of Psychology, 15, 156–165. Kimura, D. (1961b). Cerebral dominance of the perception of verbal stimuli. Canadian Journal of Psychology, 15, 166–171. Kimura, D. (1967). Functional asymmetry of the brain in dichotic listening. Cortex, 3, 163–178. Kinsbourne, M. (1975). The mechanism of hemispheric control of the lateral gradient of attention. In P. M. A. Rabbitt, & S. Dornic (Eds.), Attention and performance V (pp. 81–97). London: Academic Press. Meyers, J. E., Roberts, R. J., Bayless, J. D., Volkert, K., & Evitts, P. E. (2002). Dichotic listening: Expanded norms and clinical application. Archives of Clinical Neuropsychology, 17, 79–90. Milner, B., Taylor, L., & Sperry, R. W. (1968). Lateralized suppression of dichotically presented digits after commissural section in man. Science, 161, 184–186. Morais, J., & Landercy, M. (1977). Listening to speech while retaining music: What happens to the right-ear advantage? Brain and Language, 4, 295–308. Roberts, M. A., Persinger, M. A., Grote, C., Evertowski, L. M., Springer, J. A., & Tuten, T., et al. (1994). The dichotic word listening test: Preliminary observations in American and Canadian samples. Applied Neuropsychology, 1, 45–56. Sommer, I. E., Aleman, A., Somers, M., Boks, M. P., & Kahn, R. S. (2008). Sex differences in handedness, asymmetry of the planum temporale and functional language lateralization. Brain Research, 1206, 76–88. Springer, S. P., & Deutsch, G. (1985). Left brain, right brain (Rev. ed.). New York: W.H. Freeman. Springer, S. P., & Gazzaniga, M. S. (1975). Dichotic testing of partial and complete split brain subjects. Neuropsychologia, 13, 341–346.

Strauss, E., Gaddes, W. H., & Wada, J. (1987). Performance on a freerecall verbal dichotic listening task and cerebral dominance determined by the carotid amytal test. Neuropsychologia, 25, 747–753. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests (3rd ed.). New York: Oxford University Press.

Diencephalic Seizures ▶ Hemodynamic Response

Diencephalon L INDA L. P HILLIPS Virginia Commonwealth University Richmond, VA, USA

Synonyms Epithalamus-pineal gland, habenular nuclei; SubthalamusSubthalamic nucleus, zona incerta

Structure The diencephalon can be divided into four regions: epithalamus, thalamus, subthalamus, and hypothalamus. The epithalamus constitutes a small area of the diencephalon, located dorsally above the thalamus. It contains the pineal, a lobular midline structure, just rostral to the superior colliculi. Historically thought to be the ‘‘seat of the soul,’’ neurons in this single midline structure are called pinealocytes, which secrete melatonin and are linked with the retina through a circuitous path via the sympathetic chain. At the base of the pineal, a stalk connects the structure to the remainder of the diencephalon. The bilateral habenula is the other component of the epithalamus, positioned rostral to the pineal and posterior commissure. The two habenular nuclei are joined by a commissure, and receive input from the dorsal thalamus via the stria medullaris fibers. Output from the habenula targets the midbrain reticular formation. The largest portion of the diencephalon is the dorsal thalamus. It extends rostrally to the interventricular foramen, superiorally to the transverse cerebral fissure and the floor of the lateral ventricles, inferiorly to the hypothalamic sulcus, and posteriorly it overlaps the midbrain.

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Topographically, the thalamus is divided into nuclear groups, designated medial or lateral based upon their relationship to an interposed sheet of axons called the internal medullary lamina. Largely rostral to this lamina is an anterior set of nuclei, collectively grouped into one entity and bordering the interventricular foramen. The medial division of the dorsal thalamus contains a single dorsomedial nucleus. Laterally, the nuclei are divided into dorsal and ventral layers. Dorsally, they include lateral dorsal, lateral posterior, and pulvinar groups. Ventrally, a more complex arrangement exists forming the ventral anterior and lateral nuclei, with the posterior group having a posteriolateral and posteriomedial region. More laterally arranged are the geniculate bodies, made up of a medial auditory and lateral visual nuclear group. In one region the internal medullary lamina splits to surround two centrally embedded nuclei, the centromedian and parafascicular groups. Finally, the thalamus has two more thinly distributed nuclear groups: the thalamic reticular nucleus surrounding portions of the thalamic perimeter and the midline nuclei, which are essentially a sheet of cells that extend from the periaqueductal gray zone to cover the midline surface of the thalamus. Major thalamic input is derived from ascending sensory and motor pathways, as well as limbic inputs, information that is processed within the thalamus. A subset of this input is routed as relay connections to specific areas of functional cortex or to more broadly integrative regions of association cortex. Fiber routes for input include the mammillothalamic tract, fornix, medial lemniscus, central tegmental tract, inferior colliculi, optic tract, and the white matter tracts of the prefrontal, parietal, occipital, and temporal lobes. In this way, the thalamus acts as a gateway to influence output from a wide range of cortical and subcortical circuits. A part of the midbrain gray matter is wedged rostrally between the hypothalamus and internal capsule to form the subthalamus. It lies inferior to the dorsal thalamus and contains nuclear extensions from the midbrain red nucleus and substantia nigra as they project to the thalamus. Specific cellular groups in the subthalamus include the subthalamic nucleus and the zona incerta. The subthalamic group is the largest and has extensive connections with the basal ganglia. Dorsal to the subthalamic nucleus is the thinner zona incerta, which represents an extension of midbrain reticular formation. Most ventral in the diencephalon is the hypothalamus. It extends along the descending portion of the third ventricle, rostrally to the lamina terminalis, superiorly to the hypothalamic sulcus at its border with the dorsal thalamus; inferiorly it forms the most ventral diencephalic tissue, a portion extending into the posterior lobe of

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the pituitary. The hypothalamus has three sets of nuclear groups based upon their rostrocaudal position. As with the dorsal thalamus, both medial and lateral areas are identified. The anterior group, arranged above the optic chiasm, includes medially the preoptic, paraventricular, anterior, and supraoptic nuclei. Laterally, an anterior preoptic group is also identified and a broadly dispersed lateral nucleus. In dorsoventral alignment with the tuber cinereum (pituitary stalk) is the tuberal group of nuclei. These include dorsomedial, ventromedial, and arcuate nuclei in the medial area. In the lateral tuberal zone, the rostral lateral nucleus extends more caudally, accompanied by the lateral tuberal nuclei and tuberomammillary nuclei. Finally, the posterior nuclear group of the hypothalamus is formed primarily of the mammillary bodies and the posterior nuclear groups. Laterally, this region contains the most caudal extension of the lateral nucleus. The hypothalamic nuclei are traversed by major fiber bundles, including the fornix, stria terminalis, median forebrain bundle, dorsal longitudinal fasciculus, and the two major mammillary body connections, the mammillothalamic tract and mammillotegmental tract. These pathways interconnect the hypothalamic nuclei and represent their major input (e.g., fornix stria terminalis, median forebrain bundle)/output (e.g., mammillothalamic, mammillotegmental tracts, median forebrain bundle, and dorsal longitudinal fasciculus) routes to interface with other brain regions. A unique relationship exists between the hypothalamus and the pituitary. Through two different mechanisms, the hypothalamus interacts with the pituitary gland: either via direct axonal projections to the posterior pituitary (neurohypophysis) or through the secretion of regulatory factors at a portal vein interface in the pituitary stalk (adenohypophysis).

Function The primary function associated with the epithalamus is regulation of circadian rhythm through the pineal gland. The pineal is an endocrine gland, secreting high levels of melatonin hormone during darkness. Melatonin provides a rhythm for setting the sleep–wake cycle, and can affect gonadotrophic hormone production as a function of daylight period. It is thought that hormonally stimulated reproductive behavior may be optimized by linking longer day length with sexual cycles. Although the specific function of the habenular nucleus is not fully understood, it receives input from the stria medullaris and projects to the midbrain reticular formation, potentially mediating limbic influence on brainstem output.

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The thalamus is generally thought to function as a gateway for information transfer to the cerebral cortex. The physiological firing of thalamic neurons can occur in either a tonic or bursting mode. Tonic firing permits point-to-point information transfer, where signals from the lower levels of the CNS can pass directly to the cortex. Thalamic neurons also have a burst mode of signaling, which conveys longer, rhythmic waves of depolarization, critical to functional patterns of activation such as those that occur during sleep/wake cycles. Different thalamic nuclear groups target specific brain regions to affect motor coordination (cortex, basal ganglia, and cerebellum), autonomic integration (midbrain), hormonal regulation (hypothalamus), hearing, vision, cognition, and emotion (auditory, visual, and limbic cortices). Of all the diencephalic divisions, least is known about subthalamic function. The subthalamic nucleus is interconnected with the basal ganglia, and is part of the indirect pathway between that region and the cerebral cortex. At this position, the subthalamic nucleus may act to regulate inhibitory output from the basal ganglia. Much less is known about the zona incerta, its function most likely related to its continuity with the midbrain reticular formation. Hypothalamic function is mediated by two types of interface: reciprocal connections with regions providing its major afferent pathways (thalamus, septum, and brainstem), which influence the behavioral output directed by those regions, and projections to brainstem autonomic nuclei (midbrain, medulla, and spinal cord), which directly regulate visceral functions such as circadian cycles, osmoregulation, appetite, body temperature, and fight/ flight response. These systemic responses are also influenced by hypothalamic connections to the limbic system (via the mammillothalamic tract, fornix, and stria terminalis). Two other major functions of the hypothalamus are the regulation of growth and reproductive cycles, controlled through broad feedback loops, utilizing receptormediated monitoring in both the hypothalamus and the affected peripheral organs. Growth and reproduction are particularly dependent upon interaction with the pituitary lobes, either via secretion of hypothalamic-generated hormones within the lobe (neurohypophysis), or release of hypothalamic regulatory factors into the hypothalamic– hypophyseal portal system for control of pituitary hormone production (adenohypophysis).

Illness Illness resulting from structural and metabolic pathology has been documented for all subregions of the

diencephalon. In the epithalamus, the primary target of pathology is the pineal gland. Both tumor and cyst formation can occur in the pineal. Tumors often alter the production and release of melatonin, affecting sleep–wake cycle patterns and, in some cases, are correlated with ocular dysfunction. Since melatonin also has gonadotrophic effects, shifts in its production may change reproductive behavior. Non-neoplastic parenchymal cysts can occur within the pineal. Although a rare occurrence, these may reach a size that compresses the adjacent cerebral aqueduct and tectal plate. Such compression could restrict CSF flow, with the potential for producing hydrocephaly. Within the dorsal thalamus, pathology is usually linked to neuronal loss, astrocytosis, or myelin breakdown. These can occur due to metabolic dysfunction, as in Wernicke–Korsakoff syndrome or Parkinson’s disease, in the prion-induced plaque pathology of Creutzfeldt– Jakob disease and sporadic fatal insomnia, or with the characteristic spongy white matter condition generated in Kearns–Sayre syndrome. With the loss of neurons and axonal degeneration, abnormal thalamocortical integration affects motor function and may generate spasticity, epileptic seizure activity, or dementia. Functional change in the subthalamus is thought to contribute to the muscular rigidity of Parkinson’s disease. In that case, over activity of neurons in the subthalamic nucleus and the related globus pallidus leads to increased inhibition of the thalamus, with reduced cortical motor activation. Focal vascular lesions of the subthalamus can also produce the violent involuntary limb movement seen with hemiballismus. Pathology in the hypothalamus/pituitary is most extensively described, due to the significant role of these regions in autonomic nervous system regulation and their interface with a variety of peripheral organs. For example, lesions within specific hypothalamic nuclei controlling visceral functions of appetite, thirst, sleep–wake cycle, regulation of body temperature, or emotional state often result in extreme shifts within the normal range of these functions. Many of the human responses to such hypothalamic lesions can be replicated in animal models. Functional disorders of the hypothalamus/pituitary axis can also be driven by abnormal metabolism. One of the best-known examples is thiamine deficiency, where microvascular lesions occur in the mammillary bodies. These lesions underlie Wernicke–Korsakoff syndrome encephalopathy, which presents as a variety of ophthalmic abnormalities and cognitive deficits. A wide range of tumors, including gliomas and adenomas, are also common in the hypothalamus and pituitary. They selectively affect the secretion of

Differential Ability Scales (DAS and DAS-II)

pituitary hormones, producing syndromes ranging from gigantism and infertility to the more complex cortisolrelated abnormalities of Cushing’s disease.

Cross References ▶ Associational Cortex ▶ Basal Forebrain ▶ Cerebral Cortex ▶ Cortical–Subcortical Loop ▶ Fornix ▶ Hypothalamic Glioma ▶ Korsakoff ’s Syndrome ▶ Mediodorsal Nucleus of Thalamus ▶ Parkinson’s Disease ▶ Pineal Tumors ▶ Pituitary Adenoma ▶ Pituitary Mass ▶ Thalamus

References and Readings Felten, D. L., & Jozefowicz, R. F. (2003). Netter’s atlas of human neuroscience, Teterboro, NJ: MediMedia. Haines, D. E. (2006). Fundamental neuroscience for basic and clinical applications (3rd ed.). Philadelphia, PA: Elsevier. Horvath, E., Scheithauer, B. W., Kovacs, K., & Lloyd, R. V. (2002). Hypothalamus and pituitary. In D. I. Graham & P. L. Lantos (Eds.), Greenfield’s neuropathology (Vol. I, pp. 983–1062). London: Arnold. Kandel, E. R., Schwartz, J. H., & Jessell, T. M. (2000). Principles of neural science (4th ed.). New York: McGraw-Hill. Lantos, P. L., Louis, D. N., Rosenblum, M. K., & Kleihues, P. (2002). Tumours of the nervous system. In D. I. Graham & P. L. Lantos (Eds.), Greenfield’s neuropathology, (Vol. II, pp. 767–1052). London: Arnold. Lowe, J. S., & Leigh, N. (2002). Disorders of movement and system degenerations. In D. I. Graham & P. L. Lantos (Eds.), Greenfield’s neuropathology, (Vol. II, pp. 325–430). London: Arnold. Netter, F. H. (1974). The hypothalamus. In F. H. Netter (Ed.), The Ciba collections of medical Illustrations, (Vol. I, pp. 147–168). New York: Case–Hoyt. Notle, J. (2002). The human brain: An introduction to its functional anatomy (5th ed.). St. Louis, MO: Mosby (Harcourt Health Sciences).

Diener Life Satisfaction Scale ▶ Satisfaction with Life Scale

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Differential Ability Scales (DAS and DAS-II) K ELLY C ROTTY, I DA S UE B ARON Inova Fairfax Hospital for Children Falls Church, VA, USA

Synonyms DAS-II

Description The Differential Ability Scales-II (DAS-II; Elliott, 2007) is an individually administered, comprehensive cognitive ability battery. It consists of 20 subtests for children and adolescents, aged 2 years 6 months to 17 years 11 months. Results of the DAS-II yield a General Conceptual Ability (GCA) score (consisting of Verbal Comprehension, Naming Vocabulary, Picture Similarities, Matrices, Pattern Construction, and Copying), diagnostic scores (consisting of Early Number Concepts, Recall of Digits Forward, and Recognition of Pictures), and a Special Nonverbal Composite (SNC) score (consisting of Picture Similarities, Pattern Construction, Matrices, and Copying). Core subtests are used to estimate a child’s GCA score, which is derived from verbal, nonverbal, and spatial cluster scores. GCA focuses on a child’s overall reasoning and conceptual abilities. The SNC score is derived only from the nonverbal and spatial clusters and provides a valid comprehensive score for children whose oral responses may not be valid (see Riccio, Ross, Boan, Jemison, & Houston, 1997). Diagnostic subtests do not contribute to GCA, but instead, ‘‘measure aspects of memory, processing speed, and foundational abilities for early school learning (Elliott, 1990).’’ The DAS-II battery is divided into Early Years for children 2:6–6:11, and School-Age for children 7:0– 17:11. The Early Years battery is further divided into Lower Level (2:6–3:5) and Upper Level (3:6–6:11). Upper Level yields six core subtests and 11 diagnostic subtests, while the Lower Level yields four core subtests and three diagnostic subtests. The Early Years and SchoolAge batteries are normed on an overlapping sample of children 5:0–8:11 to accommodate children who may possess strengths or weaknesses above or below their

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age-matched peers. This flexibility in test administration allows for the assessment of children based on ability rather than age. Administration time for the core battery is 25–65 min for Early Years, and 45–65 min for School-Age. Diagnostic subtests average an additional 15–30 min of testing time. Each subtest offers predetermined starting points based on the child’s age that eliminates items likely to be too easy or too difficult for a child. Discontinuation rules are based on decision points, stopping points, and basal and ceiling guidelines. Raw scores are converted to T-scores that are then converted into standard scores. Verbal, nonverbal, and spatial cluster scores can be derived from subtest standard scores. The DAS-II manual suggests that qualifications to administer the test battery include formal graduate or professional training in psychological assessment and interpretation of results. A trained technician can administer and score the battery, but should not be involved in the interpretation.

Historical Background The original DAS (1990) was designed as a revision and extension of the British Ability Scales (BAS), published in 1979. The BAS is a cognitive ability battery developed to meet the need of British psychologists and educators for an assessment similar to the Wechsler Intelligence Scale for Children (WISC), but valid for British culture and standardized on a British population. The BAS also aimed to resolve weaknesses of the available ability and IQ tests such as ethnic and social class bias within measures and minimal focus on diagnostic subtests. A further aim was to incorporate findings of emerging research of early cognitive development that were not measured by existing ability-test batteries. The development of the DAS began in 1984 by obtaining reviews, opinions, and feedback of the BAS’s perceived strengths and weaknesses from educators and clinical psychologists. Based on this feedback, six subtests were dropped and four subtests were added. Other revisions included reconstruction of existing subtests to increase efficiency and reliability. Standardization of the DAS was performed during 1987–1989 in 70 cities across the USA. The DAS-II was published in 2007, and offers new and updated artwork and materials, revised subtests, and updated standardized norms. Four new subtests were added to the second edition: recall of sequential order,

recall of digits backward, phonological processing, and rapid naming.

Psychometric Data The manual states that the internal reliability for GCA is 0.90 for lower-level preschool, 0.94 for upper-level preschool, and .95 for school-age. Internal reliabilities of subtests vary between 0.70 and 0.95. Test–retest reliability was reported to be quite strong with GCA score reliability ranging from 0.89 to 0.94. The DAS handbook includes a number of studies reporting consistently high correlations of the DAS GCA score with overall composite scores of other cognitive batteries, such as the Wechsler Preschool and Primary Scale of Intelligence, Stanford–Binet, Woodcock–Johnson, and Wechsler Intelligence Scale for Children.

Clinical Uses The DAS-II is designed to provide an understanding of a child’s definable strengths and weaknesses within a wide range of cognitive abilities. The DAS-II helps determine why a child is not learning and targets the specific nature of the problem. Interpretation of the DAS-II allows for intervention strategies to be identified and implemented. Results can assist in placement decisions for educational settings, and provide useful clinical information for neuropsychological evaluation and research purposes. The DAS-II is not considered an intelligence test. The aim of the DAS-II is to provide a summary of meaningful and distinct subtest scores, with IQ assessment as a secondary function. Interpretation of the DAS-II is intended to identify specific strengths and weaknesses. A unique aspect of the DAS-II is that the diagnostic subtests are fairly independent of composite scores. Therefore, ‘‘these subtests are capable of providing unique information beyond that provided by composite scores (Elliott, 2007).’’ Minimal research is currently available on the DAS-II because of its recent publication date; however, the original DAS has proven useful in differentiating children with learning disability from control children as well as providing distinct profiles for various subtypes of learning disability (McIntosh & Gridley, 1993).

Cross References ▶ Kaufman Assessment Battery for Children (K-ABC) ▶ NEPSY-2nd Edition

Diffuse Axonal Injury

▶ Stanford–Binet 5 ▶ Wechsler Intelligence Scale for Children ▶ Wechsler Preschool and Primary Scale of IntelligenceIII (WPPSI-III)

References and Readings Elliott, C. D. (1990). Differential ability scales: Introductory and technical handbook. San Antonio, TX: The Psychological Corporation. Elliott, C. D. (2007). Differential ability scales – second edition: Administration and scoring manual. San Antonio, TX: Harcourt Assessment, Inc. McIntosh, D. E., & Gridley, B. E. (1993). Differential ability scales: Profiles of learning-disabled subtypes. Psychology in the Schools, 30, 11024. Riccio, C., Ross, C., Boan, C., Jemison, S., & Houston, F. (1997). Use of the differential ability scales (CAS) special nonverbal composite among young children with linguistic differences. Journal of Psychoeducational Assessment, 15(3), 196–204.

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consideration the possible organic sources of the presentation (e.g., congenital brain anomaly or event, HIV status, brain tumor, neuronal demyelination, etc.), psychiatric concerns (psychosis, severe depression), medical interventions (e.g., pharmaceuticals), and substance use. In addition, age of onset of dysfunction, change over time in functioning, neurological findings, and cultural beliefs and rituals may assist in differentiating a diagnosis. For example, in differentiating dementia from memory deficits occurring secondary to a severe mood disorder, one must consider age of onset of difficulties, change in functioning across time, medical conditions associated with memory difficulties and mood symptoms, brain injury, acute and long-term substance use, developmental history, presentlife circumstances and any previous treatment. Assessment over time is also very likely to contribute to differentiating a dementing disorder from cognitive difficulties occurring secondary to psychiatric disturbance.

Diffuse Astrocytoma Differential Diagnosis S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

Definition Differential diagnosis refers to the process of differentiating one diagnosis from another, and in turn, providing the most fitting diagnosis based on an individual’s presentation. The Diagnostic and Statistical Manual of Mental Disorders, for instance, has historically defined diagnoses from a categorical perspective, with many diagnoses having features in common with one another. This requires careful consideration of all features of an individual’s presentation and the fine details of the diagnosis itself.

Current Knowledge In neuropsychology, the process of providing the most fitting diagnosis for a patient involves assessment of the possible causes of the patient’s current condition as well as a consideration of characteristics of the cognitive and neurobehavioral presentation. This process must take into

▶ Fibrillary Astrocytoma

Diffuse Axonal Injury B ETH R USH Mayo Clinic Jacksonville, FL, USA

Synonyms DAI

Definition One of the major types of traumatic brain injury that results from acceleration–deceleration effects rather than direct impact on the brain. Typically, the injury is the result of high-speed situations such as motor vehicle accidents or violent shaking of the head from side to side. The forceful motion of injury disturbs the delicate underlying white matter tracts of the brain that are responsible for connecting the functional areas controlling motor skills, cognitive skills, language skills, and behavior. Because each neural cell or vessel has a fixed length and is

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held in position along its path by other cells, there is limited elasticity in any cell or vessel. Thus, acceleration– deceleration forces can disrupt cells and vessels despite an absence of direct trauma to the cell or vessel. Damage may result from stretching, twisting, or rotation. Diffuse axonal injury (DAI) is more likely to occur with lateral movements of the brain and least likely to occur with sagittal movements of the brain.

Historical Background The first detailed pathological and histological account of diffuse white matter damage following traumatic brain injury occurred in 1956 (Strich, 1956). In 1982, the first paper using the term ‘‘diffuse axonal injury’’ was published (Gennarelli et al., 1982). Since that time, the idea of DAI has further evolved and it is now clear that axons are not affected in isolation, but rather there is injury to the functional areas of the brain that are disconnected from one another as a result of axonal damage. There is also secondary damage to brain areas resulting from biochemical changes and edema that evolve following disruption of the axons. It has been suggested that diffuse axonal injury is therefore a misnomer and that it may be more appropriate to describe this type of injury as diffuse brain injury, a term that would include DAI.

simple terms, DWI makes it possible to see the disruption of cortical and subcortical connections throughout the brain that may appear ‘‘normal’’ on a structural image of the brain such as a standard brain MRI or head CT. With DWI techniques, the understanding of DAI and the implications of such damage have exploded. For example, DWI images can now demonstrate structural abnormalities in traumatic brain injury patients even when DAI cannot be visualized using standard imaging techniques (Hou et al., 2007). DWI images have also been used as prognostic indicators to determine the length of coma following traumatic brain injury (Zheng et al., 2007). Cognitive studies show that the greater the white matter damage in diffuse axonal injury, the greater the cognitive deficits observed in traumatic brain injury survivors (Kraus et al., 2007). These improved methods for studying DAI will most likely expand the knowledge of the heterogeneity of clinical presentation and outcome in the so-called mild traumatic brain injury, suggesting that mild traumatic brain injury may not be mild at all. DAI disturbs white matter tracts that are responsible for efficient and coordinated communication between functional areas of the brain. Consequently, patients with DAI undergoing neuropsychological evaluation typically demonstrate diffuse cognitive impairment most prominently characterized by markedly slowed cognitive processing speed, poor attention, and difficulties with executive functions like frontal lobe connections to other functional brain areas are disturbed.

Current Knowledge DAI may manifest as transient biochemical change, or permanent injury to the axons of neurons. Three levels of DAI have been suggested (Gennarelli et al., 1982). Grade I accounts for widespread axonal damage with no focal abnormalities observed on the brain imaging. Grade II involves the presence of Grade I damage, with additional focal abnormalities often in the corpus callosum. Grade III injury involves the presence of Grade I and II injuries, with additional injury observed in the brainstem. Studies have shown that the earliest that DAI can be detected is within 1 h of the onset of injury. The full spectrum of pathological change may take place over the first 24–72 h following the onset of injury. Total brain volume may be affected in the acute stages of recovery and may continue to reduce in response to injury, up to 3 years following injury. The advent of new neuroimaging methods have allowed further understanding of diffuse axonal injury. Diffusion-weighted images (DWIs) allow clinicians to more precisely measure the extent of white matter and parenchymal damage following traumatic brain injury. In

Future Directions As neuroimaging techniques continue to advance, it will be possible to study recovery from DAI and to understand the time course of recovery for such an injury. These improved methods open up the horizon for further research studies. For example, using DWI, it may be possible to describe detailed effects of early rehabilitation interventions or pharmacological treatments that can enhance recovery and limit the extent of long-term brain damage that evolves following the onset of DAI.

Cross References ▶ Acceleration–Deceleration Injury ▶ Diffusion-Weighted Imaging ▶ Shearing Injury, Shear Strain ▶ Traumatic Brain Injury

Diffusion Tensor Imaging

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Definition

Bigler, E. D. (2001). Distinguished Neuropsychologist Award Lecture 1999. The lesion(s) in traumatic brain injury: Implications for clinical neuropsychology. Archives of Clinical Neuropsychology, 16, 95–131. Gennarelli, T. A., Thibault, L. E., Adams, J. H., Graham, D. I., Thompson, C. J., & Marcincin, R. P. (1982). Diffuse axonal injury and traumatic coma in the primate. Annals of Neurology, 12, 564–574. Hou, D. J., Tong, K. A., Ashwal, S., Oyoyo, U., Joo, E., Shutter, L., & Obenaus, A. (2007). Diffusion-weighted magnetic resonance imaging improves outcome prediction in adult traumatic brain injury. Journal of Neurotrauma, 24, 1558–1569. Kraus, M. F., Susmaras, T., Caughlin, B. P., Walker, C. J., Sweeney, J. A., & Little, D. M. (2007). White matter integrity and cognition in chronic traumatic brain injury: A diffusion-tensor imaging study. Brain, 130, 2508–2519. Strich, S. J. (1956). Diffuse degeneration of the cerebral white matter in severe dementia following head injury. Journal of Neurology, Neurosurgery, and Psychiatry, 19, 163–185. Zheng, W. B., Liu, G. R., Li, L. P., & Wu, R. H. (2007). Prediction of recovery from a post-traumatic coma state by diffusion-weighted imaging (DWI) in patients with diffuse axonal injury. Neuroradiology, 49, 271–279.

Diffusion tensor imaging (DTI) is a magnetic resonance imaging (MRI) technique that reveals the integrity of white matter tracts that link regions of the brain to each other. DTI exploits the characteristic that water molecules are in constant motion. In regions such as the ventricles, which offer little physical constraint, movement occurs randomly in every direction. In contrast, water molecules in white matter fibers are constrained by the physical boundaries of the axon sheath, which cause greater movement along the long axis of the fiber than across it. The diffusion properties of water molecules are studied within and between three dimensional elements called voxels. The technique is called diffusion tensor imaging because a tensor, a mathematical description of the orientation and magnitude of diffusion, is computed for each voxel. Images acquired in different planes will highlight different white matter tracts or provide different views of them. Within a uniformly oriented tract, anything that disrupts the regular structure of white matter, such as loss of the protective myelin sheath or deterioration of the axons, might allow the water molecules to move more freely, resulting in an altered image.

Diffuse Cerebral Gliomatosis ▶ Gliomatosis Cerebri

Diffuse Lewy Body Disease (DLBD) ▶ Dementia with Lewy Bodies

Diffuse Plaques ▶ Amyloid Plaques

Current Knowledge DTI has demonstrated white matter abnormalities in Alzheimer’s disease, schizophrenia, AIDS, depression, and normal aging. DTI has also been used to examine brain white matter microstructural integrity in alcoholism and in the corticospinal tracts after stroke. It can reveal microstructural abnormalities in white matter, such as myelin loss, enlargement of microtubules, and degradation of membranes, even when that region appears normal on structural MRI. In the area of traumatic brain injury, there is early evidence to suggest that DTI may be of value in assessing diffuse axonal injury, which is typically difficult to image with other available techniques.

Diffusion Tensor Imaging Cross References S USAN L ADLEY-O’B RIEN Denver Health Medical Center Denver, CO, USA

▶ Magnetic Resonance Imaging

References and Readings Synonyms DTI

Bigler, E. D. (2007). Neuroimaging correlates of functional outcome. In N. D. Zasler (Ed.), Brain injury medicine (pp. 202). New York: Demos.

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Rosenbloom, M. (2003). Using magnetic resonance imaging and diffusion tensor imaging to assess brain damage in alcoholics. Alcohol Research and Health, 27, 146. Xu, J., et al. (2007). Diffuse axonal injury in severe traumatic brain injury visualized using high-resolution diffusion tensor imaging. Journal of Neurotrauma, 24(5), 753–65.

Diffusion-Weighted Imaging E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms DWI

Definition Diffusion-weighted imaging (DWI) produces magnetic resonance images of biological tissues weighted with the local characteristics of water diffusion. Acquisition of images using regular MRI utilizes the behavior of protons in water to generate contrasting appearances between features of the different tissues being studied. This ability to produce contrast across tissues is called ‘‘weighting,’’ and is stimulated by imposing a strong magnetic field that makes the protons in water molecules move differently depending on their specific tissue environment. In certain clinical situations, this can generate contrast between an area of pathology and the surrounding healthy tissue. In DWI, the specific magnetic field used causes the protons to behave in a particular manner that varies depending on the characteristics of the tissue. Each section of the image that is produced has a unique apparent diffusion coefficient (ADC), and each section can then be mapped as an image illustrating diffusion levels at each section as the contrast marker.

Current Knowledge Although DWI is now used for a variety of clinical conditions, it was first applied to imaging of the brain following stroke. Injured areas show up ‘‘darker’’ on an ADC map compared to healthy tissue. As a consequence, diffusion-weighted MR images are useful to diagnose acute ischemic strokes. DWI more accurately detects early-onset pathophysiologic changes of infarction than does traditional MRI or CT. It also may detect the

infarction ‘‘core,’’ which usually progresses to full infarction unless there is early reperfusion. The initial DWI lesion volume and ADC values correlate with infarction volume and with neurologic assessment tests. ADC values may be useful in differentiating tissue destined to infarct from that potentially salvageable with reperfusion therapy. With improvements in MR software and hardware, diffusion MR will continue to improve the management of patients with acute stroke.

Cross References ▶ Ischemic Penumbra ▶ Perfusion-Weighted Imaging

References and Readings Schaefer, P. W., Grant, P. E., & Gonzalez, R. G. (2000). Diffusion-weighted MR imaging of the brain. Radiology, 217, 331–345. Gonzalez, R. G., Schaefer, P. W., Buonanno, F. S., et al. (1999). Diffusion-weighted MR imaging: Diagnostic accuracy in patients imaged within 6 hours of stroke symptom onset. Radiology, 210, 155–162.

Digit Memory Test ▶ Hiscock Forced-Choice Test

Digit Span D ENENE WAMBACH 1, M ELISSA L AMAR 2, R OD S WENSON 3, DANA L. P ENNEY 4, E DITH K APLAN 5, DAVID J. L IBON 1 1 Drexel University College of Medicine Philadelphia, PA, USA 2 King’s College London London, UK 3 University of North Dakota Medical School Fargo, ND, USA 4 The Lahey Clinic Burlington, MA, USA 5 Suffolk University Boston, MA, USA

Synonyms Backward digit task; Digit span test

Digit Span

Historical Background The term digit span encompasses several important constructs and names of tests designed to measure these constructs. First and foremost digit span, along with reaction time, may be viewed as one of the two original paradigms used by experimental psychologists to investigate cognition. The origins of digit span as a psychological construct date from the work Gottfried Leibniz (1646–1716). Leibniz suggested that individuals have a finite capacity to prospectively process or hold in mind the information from the environment. He termed this capacity the span of apperception. (Much of the historical information reviewed in this essay is drawn from JTE Richardson’s paper: Measurements of short-term memory: A historical review. Cortex, 43, 635–650, 2007.) In the nineteenth century, Herman Ebbinghaus (1850–1909; 1885/1964 cited in Richardson, 2007) was the first cognitive scientist to show how span could be used as an experimental paradigm to investigate memory and learning. In America, Oliver Wendell Homes pater (1809–1894; 1871 cited in Richardson, 2007) made cogent observations on the parameters of digit span as a method to assess span of apperception, i.e., ‘‘in uttering distinctly a series of unconnected numbers or letters before a succession of careful listeners, I have been surprised to find how generally they break down, in trying to repeat them, between seven and ten figures or letters’’ (Holmes, 1871, cited in Richardson, 2007). (Oliver Wendell Holmes, Sr., M.D., a resident of Cambridge, Massachusetts, was a very influential nineteenth century physician. In addition to his achievements as Dean of Harvard Medical School, Dr. Holmes popularized the use of the stethoscope as medical instrument. Equally impressive were Dr. Holmes’ literary achievements. Admired by Edgar Allan Poe, Dr. Holmes was a member of an illustrious group of Boston literati and could count as his friends such individuals as Ralph Waldo Emerson and Henry Wadsworth Longfellow. Known for his essays and poetry, Dr. Holmes was a founder of the Atlantic Monthly magazine. In addition to his other achievements Dr. Holmes is credited for naming the city of Boston The Hub of the Universe. Holmes’ son, Oliver Wendell Holmes, Jr., was a well known jurist and was appointed Associate Justice to the Supreme Court by President Theodore Roosevelt.) Several years later, Ebbinghaus made a similar observation commenting, ‘‘the question can be asked, what number or syllables can be correctly recited after only one reading. . . for me the number is usually seven’’, (1885, cited in Richardson, 2007). Thus, more than 80 years before George Miller’s seminal paper (1956) ‘‘The

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magical number seven, plus or minus two: Some limits on our capacity for processing information’’, the basic paradigm of span as a psychological method had already been examined. The second and more frequently used meaning of the term digit span is as the name of psychological tests to assess working memory and/or the ability to retain and keep in mind a circumscribed amount of information for a short period of time (Wechsler, 1997). As a psychological test, digit span and other techniques to measure span of apperception have long history. James McKeen Cattell (1860–1944; 1890), who worked in Wilhelm Wundt’s laboratory in Leipzig, included a test of span for consonants in his compendium of mental tests. (After being fired from Columbia University for his opposition to World War I, Cattell, along with Robert Woodward and Edward Lee Thorndike, founded the Psychological Corporation in 1921 in New York City. In 1939, Woodward’s former student, David Wechsler, published the first version of the intelligence test that has so very much influenced the science and practice of psychology.) In addition, span was included as part of the intelligence tests devised by Binet and Simon (1905; cited in Richardson, 2007). In this version, up to three trials were administered for each digit sequence. Terman (1916) also included a digit span test in the Stanford revision of the Binet–Simon scales, but in Terman’s version, digit sequences both forward and backward were administered.

Psychometric Data: Digits Forward/ Digits Backward From the beginning, a digit span subtest has been at least included in virtually all Wechsler intelligence and memory scales for both children and adults. Wechsler combined performance on the digits forward and digits backward tasks into a single score on the basis of psychometric considerations. However, clinical observations have long demonstrated the value of treating each procedure as a separate test as combining forward and backward test performance potentially obscures the value of each test condition to measure related, but separate psychological constructs. A lively debate has centered on the cognitive constructs that underlie successful performance for both digits forward and backward test conditions. Indeed, Kaplan and colleagues (1991) tend to view the digits forward test condition as a means for measuring span or

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how many bits of information can be held in mind, whereas the digits backwards test condition is viewed as a test of mental manipulation. In this context, the observations of Kaplan and colleagues (1991) are consistent with the constructs put forth by Baddeley and Hitch (1974) regarding working memory. Other researchers have made similar observations. For example, Ramsey and Reynolds (1995) and Reynolds (1997) studied large samples of children and adolescents using a protocol of tests requiring the repetition of sequences of digits and letters both forwards and backwards. An analysis of forward and backward test scores using principal component analysis found that forward and backward span loaded on different factors. These researchers therefore concluded that the forward and backward span should not be combined and reflect distinct underlying cognitive processes. Weinberg, Diller, Gerstman, and Schulman (1972) studied patients with visual scanning deficits associated with right hemisphere pathology and noted differential impairment on digits backwards versus digits forward. These researchers advanced the notion that repeating digits backward may rely on a capacity to form and keep in mind a visual image of the digit sequence. Successful performance on digits backward may be viewed in terms of the ability to keep this visual image in mind while peeling numbers off in reverse order. Rudel and Denckla (1974) retrospectively studied digit forward and backwards test performance in children with learning disabilities. Children with evidence of left hemisphere dysfunction produced particularly reduced scores on digits forward. Children with evidence of right hemisphere dysfunction appeared disadvantaged when asked to repeat digits backward. Rudel and Denckla (1974) believed that their data suggested the successful performance for digits forward is governed by a left hemisphere-mediated cognitive construct involving auditory storage/ rehearsal while successful performance for digit backwards is governed by a right hemisphere mediated construct involving visual imagery operations. The value of separating forward from backwards test conditions is acknowledged in the latest version of Wechsler’s IQ test (WAIS-IV) wherein separate scale scores are now provided for digits forward and backward.

Clinical Uses Wechsler’s Digit Span Task This task is most often associated with the corpus of intelligence tests authored by David Wechsler. Wechsler’s Digit Span subtest comprises of two parts – digits forward,

where the examinee is asked to repeat increasing spans of digits in the order they were presented; and digits backwards, where the examinee is asked to repeat increasing spans of digits in reverse order. In both test conditions, two trials are administered for each span length. Each condition is discontinued when the examinee fails both trials for a given span. The raw score is the sum of trials correctly repeated. This raw score is converted into an age-corrected scale score. Developed as part of the original Bellevue Intelligence Tests (Wechsler, 1939), Wechsler writes ‘‘there is perhaps no test that has been so universally used in scales of intelligence as that of Memory Span for Digits’’ (Wechsler, 1944). Wechsler acknowledges that ‘‘low scores on the Memory Span for Digits Test are frequently associated with attention deficits’’ (Wechsler, 1944). He further states that the problems with attention and concentration as measured with his Memory Span for Digits Test are often associated with differentially poor repetition for digits backward, writing ‘‘this deficiency is often referred to by psychologists as a lack of mental control ’’ (Wechsler, 1944). Wechsler’s use of the term mental control is consistent with what is now understood as working memory. Thus, although Wechsler combined forward and backward digit test performance, he was certainly cognizant that his combined scale score could be fractionated to measure a variety of cognitive functions. Kaplan and colleagues (1991) have suggested a number of additional administration and scoring techniques to complement the traditional administration of the Wechsler Digit Span Test. First, Kaplan and colleagues (1991) suggest the investigator administer test trials until the examinee fails to repeat the exact number of digits that were administered, rather than discontinuing after failure within a pair of trials within a particular span, regardless of whether the examinee’s response is correct or not. By following this procedure, particularly for the digits forward test condition, one is able to assess the patient’s span of apperception, or the number of bits of information the patient is able to keep in mind. Moreover, for some brain damaged patients where attention, concentration, and working memory may be compromised or fluctuate it is possible that despite, say, failing both 5-span test trials, a patient could be successful on one or more 6-span test trials or on even longer span lengths. Kaplan (personal communication) has also said that a better scoring procedure for the Digit Span Test would be to tally the number of digits correctly recalled, rather than the number of successful trials. (Dr. Kaplan is well known for saying: ‘‘If they want numbers, give them numbers that are meaningful.’’) The interpretation of a

Digit Span

juxtaposition of two digits in the middle of a trial could be very different compared to instances of omissions or obvious perseverations. The idea of tallying the number of digits correctly recalled was the impetus for the creation of The Backwards Digits Test (BDT; Lamar & colleagues, 2007, 2008) described below. Kaplan (1988) and Kaplan and colleagues (1991) also call the attention to the variety of errors patients make on the digit span test. Omission – Refers to responses characterized by a missing digit. On the digits forwards task, an omission error occurs if a four-digit response of 6-8-2-1 is given to the five-digit stimulus 6-8-2-5-1. Interpretation of omission errors typically considers the length of the number sequence: whether this kind of error occurs on shorter (4span) versus longer (7 or 8-span) digit sequences. Addition – Refers to responses characterized by added digits. On the digits forward task, an addition error occurs if a five-digit response for 4-7-2-3-1 is given to the four-digit stimulus 4-7-2-3. One type of addition error is an automatized addition error. An automatized addition error occurs when the added number is sequential, i.e., 7-4-5-3 repeated as 7-3-4-5-6. Automatized addition errors suggest an executive control or frontal system problem. Perseveration – A perseveration refers to a number duplication error. Perseveration errors occur within and/or between trials. When noting a perseveration, one should observe whether the perseverated responses were captured within or between test trials. Substitution – A substitution error refers to a unique number that is contained in the digit response string that replaces an original number and does not change the original digit string length. This differs from the addition errors described previously, because addition errors change the digit string length. This is coded when any digit is given instead of a digit contain on the stimulus. Sequence Error – A sequence error refers to a correctly repeated digit that is simply misordered, but does not change the number string length. Capture Error – A type of sequence error that is characterized by the grouping of contiguous numbers together.

The Backwards Digit Test (BDT) Lamar and colleagues (2007, 2008) devised The Backward Digits Test (BDT), a digit backward paradigm designed specifically to assess for working memory deficits in dementia. This test consists of seven trials of 3, 4, and

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5-digit span lengths for a total of 21 trials. All 4 and 5-span trials were constructed so that contiguous numbers were placed in strategic positions. For example, in 4-span trials contiguous numbers were placed in either the first and third or second and fourth digit positions, e.g., 5269 or 1493. In 5-span trials contiguous numbers were placed in the three middle digits positions, e.g., 16579. Three-span test trials were not constructed in this fashion because of the primacy and recency effects. This placement strategy was devised to examine the capacity of patients to resist the temptation to produce ‘capture’ errors or to erroneously group contiguous numbers together, e.g., 15679 incorrectly reported backward as ‘‘96751’’. The BDT is administered using standardized Wechsler procedures except that there is no discontinuation. All 21 trials are administered. The two major indices derived from this test are percent of digits recalled in ANY ORDER and percent of digits recalled in SERIAL ORDER. Percent Correct ANY-ORDER – This score reflects the sum total of digits correctly recalled regardless of their serial position, divided by the total possible correct, multiplied by 100, [(total number of correct digits ANYORDER)/ (total possible correct)] 100. By eliminating the importance of serial position, this measure is believed to reflect less complex aspects of working memory characterized mainly by short term or immediate storage and rehearsal mechanisms. Percent Correct SERIAL-ORDER – This score reflects the total number of digits correctly recalled in exact serial position, divided by the total possible correct, multiplied by 100, [(total number correct digits SERIAL-ORDER)/ (total possible correct)] 100. This measure is believed to assess the more executively demanding aspects of working memory that are associated with mental manipulation, such as disengagement and temporal reordering. In addition to ANY and SERIAL ORDER recall, the BDT affords an opportunity to examine for capture errors or the tendency of patients to erroneously group contiguous numbers together. Two major types of capture errors are scored (see Kaplan, Fein, Morris, & Delis, 1991). Within Trial Capture Errors – This error is coded on 4 and 5-span trials when the capture error is confined to digits within the trial at hand, i.e., 1493–‘3491’; 16579–‘95671’. Between Trial Capture Errors – This error is coded on 3, 4 and 5-span test trials when participants ‘pull down’ or incorporate a digit or digits from an immediately preceding test trial. In a larger sense, both types of capture errors could be viewed as perseverations.

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Lamar and colleagues (2007) studied two groups of dementia patients where the onset of the illness was insidious and the overall severity of the dementia was mild. The severity of periventricular and deep white disease was measured using MRI scans, and patients were categorized as presenting with either mild versus moderate/severe white matter disease. No difference was found for ANY ORDER recall, suggesting that span, storage, and rehearsal mechanisms did not differ between these dementia groups. However, significant differences were found for SERIAL ORDER recall suggesting differential working memory impairment for dementia patients who had significant subcortical white matter disease, a finding well documented in the literature (see Lamar et al., in press; Libon, Price, Garrett, & Giovannetti, 2004 for reviews). A series of step-wise regression analyses found that ANY ORDER recall was primarily related to performance on the MMSE or dementia severity. By contrast, SERIAL RECALL was related to performance on wide number of other executive tests including letter fluency, The Boston Revision of the Wechsler Memory Scale Mental Control Test (Lamar et al., 2002), and clock drawing errors (Cosentino, Jefferson, Chute, Kaplan, & Libon, 2004). In a follow-up study, Lamar and colleagues (2008) examined regional MRI white disease and performance on ANY and SERIAL order recall and found that reduced SERIAL ORDER recall was related to left inferior parietal white matter disease. These data are consistent with ideas put forth by Rudel and Denckla (1974), suggesting that a visual imagery mechanism may underlie successful backward digit span performance. The importance of number sequencing is acknowledged in the latest version of Wechsler’s IQ test (WAIS-IV) wherein a separate score is now calculated for the longest digit string correctly sequenced. Digit span is one of the original paradigms used to investigate cognitive psychology. Attention to error type, i.e., the act of focusing on the process by which the scaled score is obtained (Kaplan, 1988), particularly where errors occur and differential performance on forward versus backward recall, can yield a wealth of information about cognitive disorders in brain-injured patients. After more than a century, a seemingly simple task of digit repetition remains an important construct in the understanding and assessment of cognition.

Cross References ▶ Serial Recall

References and Readings Baddeley, A. D., & Hitch, G. J. (1974). Working memory. In (Ed.), G. H. Bower, The psychology of learning and motivation: Advances in research and theory (vol. 8). New York: Academic Press. Cattell, J. M. (1890). Mental tests and measurements. Mind, 15, 373–381. Cosentino, S., Jefferson, A. J., Chute, D. L., Kaplan, E., & Libon, D. L. (2004). Clock drawing errors in dementia: Neuropsychological and neuroanatomic considerations. Cognitive and Behavioral Neurology, 17, 74–83. Ebbinghaus, H. (1964). Memory: A contribution to experimental psychology (Ruger HA and Bussenius CE, Trans) (Original work published in 1885). New York: Dover. Holmes, O. W. (1871). Mechanism in thought and morals: An address delivered before the Phi Beta Kappa Society of Harvard University, June 29, 1870, with notes and afterthoughts. Boston: Osgood. Kaplan, E. (1988). A process approach to neuropsychological assessment. In T. Boll, & B. R. Bryant (Eds.), Clinical neuropsychology and brain function: Research, measurement, and practice: Master lectures. Washington, DC: The American Psychological Association. Kaplan, E., Fein, D., Morris, R., & Delis, D. (1991). The WAIS-R as a neuropsychological instrument. San Antonio, TX: The Psychological Corporation. Lamar, M., Catani, M., Heilman, K. M., & Libon, D. J. (2008). The impact of region-specific leukoaraiosis on working memory deficits in dementia. Neuropsychologia, 46, 2597–2601. Lamar, M., Price, C., Davis, K. L., Kaplan, E., & Libon, D. J. (2002). Capacity to maintain mental set in dementia. Neuropsychologia, 40, 435–445. Lamar, M., Price, C. C., Libon, D. J., Penney, D. L., Kaplan, E., Grossman, M., et al. (2007). Alterations in working memory as a function of leukoaraiosis in dementia. Neuropsychologia, 2007; 45, 245–254. Libon, D. J., Price, C., Garrett, K. D., & Giovannetti, T. (2004). From Binswanger’s disease to Leukoaraiosis: What we have learned about subcortical vascular dementia. The Clinical Neuropsychologist – Vascular Dementia Special Edition, 18, 83–100. Miller, G. A. (1956). The magical number seven, plus or minus two: Some limits on our capacity for processing information. Psychological Review, 63, 81–97. Ramsey, M. C., & Reynolds, C. R. (1995). Separate digits tests: A brief history, a literature review, and a reexamination of the factor structure of the Test of Memory and Learning (TOMAL). Neuropsychology Review, 5, 151–171. Reynolds, C. R. (1997). Forward and backward memory span should not be combined for clinical analysis. Archives of Clinical Neuropsychology, 12, 29–40. Richardson, J. T. E. (2007). Measurements of short-term memory: A historical review. Cortex, 43, 635–650. Rudel, R. G., & Denckla, M. B. (1974). Relation of forward and backward digit repetition to neurological impairment in children with learning disabilities. Neuropsychologia, 12, 109–118. Terman, L. M. (1916). The measurement of intelligence: An explanation of and a complete guide for the use of the Stanford revision and extension of the Binet-Simon intelligence scale. Boston: Houghton Mifflin. Wechsler, D. (1939). The measurement and appraisal of adult intelligence (1st ed.). Baltimore: Williams and Wilkins Corporation. Wechsler, D. (1944). The measurement and appraisal of adult intelligence (3rd ed.). Baltimore: Williams and Wilkins Corporation. Wechsler, D. (1997). The Wechsler adult intelligence scale-III. San Antonio, TX: Psychological Corporation.

Digit Symbol Substitution Test Weinberg, J., Diller, L., Gerstman, L., & Schulman, L. (1972). Digit span in right and left hemiplegics. Journal of Clinical Psychology, 28, 361.

Digit Span Test ▶ Digit Span

Digit Supraspan ▶ Serial Digit Learning

Digit Symbol Substitution Test B RIANNE M AGOUIRK B ETTCHER 1, DAVID J. L IBON 2 E DITH K APLAN 3, R OD S WENSON 4, DANA L. P ENNEY 5 1 Temple University Philadelphia, PA, USA 2 Drexel University College of Medicine Philadelphia, PA, USA 3 Suffolk University Boston, MA, USA 4 University of North Dakota Medical School Fargo, ND, USA 5 The Lahey Clinic Burlington MA, USA

Synonyms Coding; Digit symbol subtest

Description The Digit Symbol substitution test (DS): This is a subtest from the corpus of intelligence tests authored by David Wechsler. Since Wechsler’s publication of the Bellevue Intelligence Scale (BIS; Wechsler, 1939), the basic format and concept of this test has changed very little. The test is timed. Various versions from the Wechsler corpus allow 90 or 120 s. The test requires the examinee to transcribe a unique geometric symbol with its corresponding Arabic number. The examinee is initially shown a key containing the numbers from 1 to 9. Under each number there is a

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corresponding geometric symbol. The examinee is then shown a series of boxes containing numbers in the top boxes, and blank boxes below them. After a short practice trial, they are then asked to copy the corresponding geometric symbol under each number. The raw score is the number of correct items completed within the prescribed time limit. The Symbol Digit Modalities Test (SDMT; Smith, 1982): This test is very similar to Wechsler’s DS test, except that rather than transposing symbols to numbers, the examinee is asked to transpose numbers to symbols. An advantage of the SDMT is that there are both written and oral administrations. Smith (1984) suggests the administration of both the written and oral versions of the test, with the written version being administered first. In addition, the SDMT provides the additional advantage of allowing the examinee to write familiar numbers (1–9) rather than geometric symbols (Lezak, Howieson, & Loring, 2004). Smith (1984) reported greater sensitivity of the SDMT compared to the DS subtest in detecting brain damage. Both the DS and SDMT are well known to be highly sensitive to the presence of cognitive impairment (see Lezak et al. (2004) for a review). The general sensitivity of these tests stems from the fact that multiple cognitive skills are necessary for optimum performance. For example, at a minimum the examinee must be able to produce written output (graphomotor skills); visually track back and forth from the test key to the test form (visual scanning); and locate the test items (visuospatial ability). Performance improves to the extent the examinee ‘‘learns’’ the symbol/number pairs without referring to the test key for each test item (incidental memory). In addition to norms provided in the Wechsler test manuals, additional adult norms for DS can be found in D’Elia, Boone, and Mitrushina (1995); Heaton, Grant, and Matthews (2004); and Ivnick, Malec, and Smith, (1992). DS performance is highly sensitive to the effects of age (Lezak et al., 2004). Wechsler (1981) reported low correlations between DS and many of his other subtests. Since the addition of the Symbol Search subtest to the Wechsler corpus, factor analysis suggests that DS and Symbol Search comprise a general processing speed factor. In order to disambiguate the specific brain-behavior relationships that underlie impaired performance on the DS test, Kaplan and colleagues (Kaplan, 1988; Kaplan, Fein, Morris, & Delis, 1991) have developed additional test procedures (see WAIS-R-NI; Kaplan et al., 1991) as described below. Symbol Copy Test Condition: The symbol copy test condition of DS test involves the direct copying of

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geometric symbols without the burden of visual scanning or incidental memory. The purpose of this measure is to assess the contribution of both motor speed and graphomotor ability to DS performance. It is also suggested that the examiner keep track of the output produced in each 30 s epoch (Kaplan et al., 1991). Kaplan et al. (1991) noted that the output produced by some patients, particularly with subcortical disease, may decline on the latter portion of the test. Incidental Learning: This test condition involves two procedures. After the DS test is concluded the examinee may be asked to recall and write the symbols that were coupled with each number (pairing). This is followed by asking the examinee to recall and write as many of the symbols as possible without any visual cues (free recall). Part of the rationale for this procedure is that, although examinees are not instructed to memorize the number/ symbol pairs, some degree of incidental learning can reasonably be expected to occur.

Historical Background Wechsler does not provide a great deal of background or detailed rationale regarding the selection of the corpus of tests originally included for his intelligence scale(s). In his magnum opus The Measurement and Appraisal of Adult Intelligence, Wechsler (1939) described the Bellevue Intelligence Scale including the Substitution Test. In the 3rd edition of The Measurement and Appraisal of Adult Intelligence (the so-called ‘‘war’’ edition, 1944, p.v), Wechsler describes the ‘‘Digit Symbol or Substitution Test as one of the oldest and best established psychological tests’’. He credited the introduction of the Substitution Test to Otis’ (1920) group intelligence test for children. Wechsler states that the DS test he developed was taken from the Army Beta Test, an IQ test first employed by the army during World War I to assess intelligence in recruits who were illiterate.

indicating that the tests share 50% of variance. This further reiterated the integral role of processing speed in DS performance. Joy, Kaplan, and Fein (2004) also examined data (n = 1167) from the WAIS-III/WMS-III standardization sample for individuals who completed at least one DS supplemental procedure. Consistent with previous findings, results suggested that speed (i.e., Symbol Copy) accounted for approximately 50% of the variance in DS. Using the incidental memory learning indices, memory accounted for only 5–7% of DS variance. However, performance on the pairing and free recall tests provided incremental explanatory power, authenticating a secondary (albeit small) role of memory in DS performance. Contributory role of incidental memory: The incidental memory measures (pairing and free recall) have been previously used as supplementary indices of memory without extensive research regarding their construct validity and without normative data. Joy, Kaplan, and Fein (2003) examined the performance of WAIS-III/ WMS-III standardization samples on pairing/free recall measures and WMS-III auditory and visual memory indices. The Incidental Learning measures correlated moderately with standardized memory measures, validating its use as a clinical measure of memory. These authors also offered guidelines for clinical use of the DS incidental learning procedures. The following cutoffs reflect the score necessary to suggest memory impairment and merit additional investigation. Scores of 15 or higher for older adults indicate that memory assessment is unwarranted. Perfect scores indicate that further memory assessment is unwarranted.

Digit Symbol Substitution Test. Table 1 Pairing (clinically significant cutoffs) Score

Age

6 or below (three or fewer pairs mastered)

Younger adults (.75). Test-retest reliability has been examined less rigorously but was found to be sufficiently high (r = .71) in a sample of 30 participants. Construct validity using factor analysis indicates that the figure ground task significantly loaded on the expected motor reduced visual perception construct.

Clinical Uses Research involving clinical populations provides further support for the involvement of visuospatial abilities mediated by higher-level visual association areas in EFT performance. For instance, Corkin and colleagues (1989) observed that patients with brain lesions in the right

Embolism

parietal lobe demonstrated a significant decline, over a 30year period, on a task of Hidden Figures. In patients with Alzheimer’s disease, who have known visual association cortex involvement, Mendez and colleagues (1990) found that all 30 of their participants demonstrated disturbances in figure-ground analysis despite documented preserved visual acuity and color recognition. Parkinson’s disease (PD) is associated with a disruption of higher level visuospatial functions likely due to disruption of striatal-parietal circuits and PD patients with mild (Levin et al.,1989), moderate and severe disease stage (Flowers & Robertson, 1995) have been found to perform more poorly on a figure-ground task relative to control participants. Patients with Autism or Asperger syndrome are faster at performing the EFT relative to control participants (Jolliffe & Baron Cohen, 1997). This finding may also support a specialized role for the left hemisphere in disembedding the figure from the ground as Autism and Asperger’s syndrome have been associated with compromise of right hemisphere functions.

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Panek, P. E., Funk, L. G., Nelson, P. K. (1980). Reliability and validity of the group embedded figures test across the life span. Percept Mot Skills, 50(3 Pt 2), 1171–1174. Reynolds, C. R., Pearson, N. A., & Voress, J. K. (2002). Developmental test of visual perception adolescent and adult. Austin: Pro-Ed. Ring, H. A., Baron-Cohen, S., Wheelwright, S., Williams, S. C., Brammer, M., Andrew, C., et al. (1999). Cerebral correlates of preserved cognitive skills in autism: A functional MRI study of embedded figures task performance. Brain, 122, 1305–1315. Spreen, O., & Benton, A. L. (1969). Embedded figures test; Manual of instructions. Neuropsychology Laboratory, Department of Psychology, Victoria: University of Victoria. Witkin, H. A., Oltman, P. K., Raskin, E., & Karp, S. A. (1971). A manual for the embedded figures test. Palo Alto, CA: Consulting Psychologist Press. Witkin, H. A., Oltman, P. K., Raskin, E., & Karp, S. A. (2002). Group embedded figures test manual. Palo Alto, CA: Consulting Psychologist Press.

Embolic Stroke ▶ Cerebral Embolism

Cross References ▶ Dorsal Visual Pathway ▶ Figure-Ground Discrimination ▶ Global Versus Local Processing ▶ Ventral Visual Pathway

References and Readings Corkin, S., Rosen, T. J., Sullivan, E. V., & Clegg, R. A. (1989). Penetrating head injury in young adulthood exacerbates cognitive decline in later years. Journal of Neuroscience, 9, 3876–3883. Flowers, K. A., & Robertson, C. (1995). Perceptual abnormalities in Parkinson’s disease: Top-down or bottom-up processes? Perception, 24, 1201–1021. Ivry, R. B., & Robertson, L. C. (1998). The two side of perception. Cambridge, MA: MIT Press. Jolliffe, T., & Baron-Cohen, S. (1997). Are people with autism and Asperger syndrome faster than normal on the Embedded Figures Test? Journal of Child Psychology and Psychiatry, 38, 527–534. Levin, B. E., Llabre, M. M., & Weiner, W. J. (1989). Cognitive impairments associated with early Parkinson’s disease. Neurology, 39(4), 557–561. Likova, L. T., & Tyler, C. W. (2008). Occipital network for figure/ground organization. Experimental Brain Research, 189, 257–267. Mendez, M. F., Mendez, M. A., Martin, R., Smyth, K. A., & Whitehouse, P. J. (1990). Complex visual disturbances in Alzheimer’s disease. Neurology, 40, 439–443. Navon, D. (1977). Forrest before trees: The precedence of global features in visual perception. Cognitive Psychology, 9, 353–383.

Embolism D ONA L OCKE Mayo Clinic Scottsdale, AZ, USA

Definition An embolism occurs when an object or embolus migrates from one part of the body through the blood vessels and causes blockage in a blood vessel in another part of the body. An embolus that migrates through the vascular system to the brain will likely cause an ischemic stroke. Foreign substances that can cause an embolism include a blood clot, an air bubble, amniotic fluid, a globule of fat, a clump of bacteria, chemicals (such as talc), and drugs (mainly illicit ones). Blood clots are the most common cause of embolism. Embolism can be contrasted with a thrombus which is the formation of a clot within a blood vessel, rather than being carried from somewhere else. Prevention and treatment for embolism vary depending on specific pathology (e.g., fat, air, bacteria, and blood clot) and source (e.g., bone fracture, cardiac, surgical procedure, and atherosclerotic plaque).

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Emergency Mental Health Treatment

Cross References ▶ Stroke

Emergency Mental Health Treatment ▶ Crisis Intervention

Emotion ▶ Emotional Intelligence

emotionally disabled, psychologically and emotionally handicapped, emotionally disordered, and emotionally/ behaviorally disabled (EBD). While services have been provided from some public schools for more than a century, it was in 1975 that PL 94–142 required all schools to serve students with an ED. At this time, many new programs were begun although this population has traditionally been underserved. This term is one of the 12 disabilities specified by the Individuals with Disabilities Education Act (IDEA). It mainly covers the age group from birth to age 21. Based on the IDEA (1997), it is a condition demonstrating one or more of the following characteristics over a long period of time and to a marked degree, which adversely affects educational performance:

Emotional Disorder ▶ Affective Disorder ▶ Depressive Disorder

Emotional Disturbance L ANSHIN C HANG , R IK C ARL D’A MATO University of Macau Taipa, Macau SAR, China

An inability to learn which cannot be explained by intellectual, sensory, or health factors; An inability to build or maintain satisfactory interpersonal relationships with peers and teachers; Inappropriate types of behavior or feelings under normal circumstances; A general pervasive mood of unhappiness of depression; or A tendency to develop physical symptoms or fears associated with personal or school problems. [Code of Federal Regulations, Title 34, Section 300.7(c)(4) (i)] A student who is diagnosed with an ED is entitled to special education services. As defined by IDEA Emotional Disturbance includes Schizophrenia but does not apply to children who are socially maladjusted unless it is determined that they have an emotional disturbance. [Code of Federal Regulation, Title 34, Section 300.7(c)(4)(ii)]

Description/Definition

Evaluation

An emotional disturbance (ED) is a major uncontrollable emotional or behavioral condition, which impairs the individual to a significant degree and does not allow the child or youth to profit from conventional educational services. It is most often used to refer to children and youth in school systems. An emotional disturbance is not caused by any obvious physical abnormalities of the brain, although research in the last 2 decades has begun to show a neuropsychological base for these disorders (Hartlage & D’Amato, 2008). Children and youth with ED are called by many similar names including students who are behavioral disabled (BD),

The definition of ED is not clear and changes from state to state in the USA. Since no specific diagnostic instruments are required to qualify for services, the diagnosis of precise conditions such as what is a general and pervasive mood of unhappiness or depression requires a highly trained specialist such as a psychiatrist or school psychologist. Students who exhibit dramatic externalizing behaviors (e.g., aggression) are more easily diagnosed, but to those who display internalizing behaviors (e.g., depression) are more difficult to identify. There are more essential criteria to label a student as emotionally disturbed. One, a student’s behavior must

Emotional Intelligence

be unusual and problematic for his parents and/or teacher across settings. Moreover, at least two documented interventions must also show that the behavior is not amenable to change. Two, because of the complexity of these disorders, a multidisciplinary team approach is required made up of various members such as regular education teachers, special education teachers, principals, parents, and a psychologist.

Treatment For educators, in order to decide what kind of special education services are suitable for eligible students who manifest an ED, a functional behavioral assessment is the most popular technique currently in use. Using a variety of data and strategies, educators are able to develop a comprehensive behavior intervention plan which usually has school and home components. Recent laws as well as research have suggested that only evidence-based interventions should be used (e.g., Traughber & D’Amato, 2005). Intervention plans typically cover five distinct components – including cognitive (e.g., poor memory, short-attention span), academic (e.g., poor educational performance not matching cognitive abilities), physical (e.g., stomachache, absenteeism), behavioral (e.g., aggression, depression, compulsive behaviors, attention seeking), and communication (e.g., echolalia, illogical speech difficulties) areas. In addition, many students who manifest an ED also are treated using a psychopharmacological intervention.

Cross References ▶ Emotional and Behavioral Disorders ▶ Social Problems

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Forness, S. R., & Knitzer, J. (1992). A new proposed definition and terminology to replace ‘‘serious emotion disturbance’’ in individuals with disabilities act. School Psychology Review, 21(1), 12–20. Hartlage, L. C., & D’Amato, R. C. (2008). Understanding the etiology of neurological and psychiatric disorders. In A. MacNeil Horton Jr., & D. Wedding (Eds.), The neuropsychology handbook (3rd ed., pp. 87–108). New York: Springer. Individuals with Disabilities Education Act (2010). In Wikipedia encyclopedia online. Retrieved February 10, 2010, from http://en. wikipedia.org Kutash, K., Duchnowski, A. J., & Friedman, R. M. (2005). The system of care twenty years later. In M. H. Epstein, K. Kutash, & A. J. Duchnowski (Eds.), Outcomes for children with emotional and behavioral disorders and their families: Program and evaluation best practices (2nd ed., pp. 3–22). Austin, TX: Pro-Ed. Miller, M. A., Fellbaum, C., Tengi, R., & Langone, H. (n.d.). Emotional disturbance. Retrieved January 20, 2010, from http://wordnetweb. princeton.edu NICHCY (2004). Disability fact sheet No.5 – Emotional disturbance. National Dissemination Center for Children with Disabilities. Retrieved January 20, 2010, from http://www.nichcy.org Traughber, M. C., & D’Amato, R. C. (2005). Integrating evidence-based neuropsychological services into school settings: Issues and challenges for the future. In R. C. D’Amato, E. Fletcher-Janzen, & C. R. Reynolds (Eds.), Handbook of school neuropsychology (pp. 827–858). New York: Wiley.

Emotional Incontinence ▶ Emotional Lability

Emotional Intelligence S HERRI G ALLAGHER Flagstaff Unified School District Flagstaff, AZ, USA

Synonyms References and Readings Algozzine, R., & Ysseldyke, J. (2006). Teaching students with emotional disturbance. CA: Corwin. Council for Exceptional Children (CEC) (2010). Behavior disorders/ emotional disturbances. Retrieved January 15, 2010, from http:// www.cec.sped.org Department of Education (1999). 34 CFR Parts 300 and 303 Assistance to states for the education of children with disabilities and the early intervention program for infants and toddlers with disabilities. Federal Register, 64(48).

Emotion; Intelligence

Definition Emotional intelligence (EI) is defined as ‘‘the ability to carry out accurate reasoning about emotions and the ability to use emotions and emotional knowledge to enhance thought’’ (Mayer, Roberts, & Barsade, 2007).

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Historical Background Intelligence in regards to emotions was discussed by Wechsler in the 1940s as the ‘‘aggregate or global capacity of the individual to act purposefully, to think rationally and to deal effectively with his environment’’ (Mayer, Salovey, & Caruso, 2000). Piaget also mentioned emotional intelligence within his stage model of development. Psychologists felt that intellect incorporated more than just knowledge and expressed interest in multiple intelligences by the 1980s. Studies by John Mayer and Peter Salovey began theoretical research on emotional intelligence in the early 1990s. During this decade, Goleman (1995) published the book Emotional Intelligence which popularized the term and it received national media attention. Daniel Goleman made strong claims that emotional intelligence made major contributions to individuals and society. This claim evoked controversy among other EI researchers. Also during this time early measures of EI were developed. Emotional Intelligence is now an active research area in psychology, business, and education.

Current Knowledge Theorists believe that EI is distinct from other cognitive mental processes because it involves a primary focus on a more specific area of problem solving (Mayer et al., 2007). Emotional intelligence theorists have focused their research on emotional ability itself and attempted to quantify EI as a separate processing domain from other intelligences with tests. Most of the current research has focused on three approaches (specific-ability, integrativemodels, and mixed models) to explain emotional intelligence along with their theoretical and methodological perspectives (Mayer et al., 2007). Specific-ability approach focuses on particular skill(s) that can be considered essential to EI. These skills and abilities are accuracy in emotional perception, ways in which emotions facilitate thinking, emotional reasoning and understanding, and emotional self-management. The second approach, the integrative-model, connects several specific abilities to obtain an overall EI, including Izard’s Emotional Knowledge Approach (Izard et al., 2001) and the four-branch model (Mayer & Salovey, 1997) of EI. The final approach to explain EI is Mixed Model which uses broad definitions such as non-ability competencies, personality dispositions, and emotionally and socially intelligent behavior to describe EI (Mayer et al., 2007). Most measures under this category assess emotional intelligence attributes but interject other variables which

are not considered part of EI. Many of the mixed-model methods are self-report scales and seem to assess personality traits rather than EI. Mayer et al. (2007) believe that a clearer approach to research would be to consider EI a discrete variable and then study its related variables. A major critique of mixed model scales suggests that they lack an EI definition that is consistent with scientific theory (Mayer et al., 2007).

Measurement of Emotional Intelligence Measures of EI are based on specific-ability and integrative-model theories. The measures discussed are performance tests, rather than self-report measures, where subjects are asked to perform a specific skill (Mayer et al., 2007). Specific-ability measures include the Diagnostic Analysis of Nonverbal Accuracy 2 (DANVA2), the Japanese and Caucasian Brief Affect Recognition Test (JACBART), and the Levels of Emotional Awareness Scale (LEAS). The DANVA2 and JACBART include scales of emotional perception while the LEAS measures emotional understanding. Integrative-model measures include Izard’s Emotional Knowledge Test (EKT; Izard et al., 2001) which provides an integrative measure of EI focusing on emotional perception and understanding. The Four-branch model connects abilities from the following areas: accurately perceiving emotions; using emotions to facilitate thought; understanding emotion; and managing emotions (Mayer & Salovey, 1997; Mayer, Salovey, Caruso, & Sitarenios, 2003). These abilities are measured by the Mayer–Salovey–Caruso Emotional Intelligence Test (MSCEIT) (Mayer, Salovey, & Caruso, 2002). Content and response-process validity were adequate for the Specific-ability and integrativemodel measures (Mayer et al., 2007). Internal consistency reliabilities for the measures fall in the moderate-to-high range. The reliabilities for most full-scale measures are adequate for research and at the higher numbers, a reliable assessment of an individual (Mayer et al., 2007). Through a factor analysis study, a single, global EI factor was found to describe MSCEIT data (Mayer et al., 2003). Additionally, two factor models indicate loadings on Experiential and Strategic EI while three or four models emphasize Emotional Perception, Understanding, and Management. Factor analysis studies have suggested that EI measures outline recognizable factors making these findings consistent with a hierarchical view of intelligence (Mayer et al., 2007). However, convergent validity across EI measures is lacking and it continues to be a concern for EI researchers. Overall, research has shown that measures


of EI are relatively reliable and valid, but more research needs to be conducted. Based on studies with EI measures, EI has been related to biopsychosocial processes, other related mental abilities, emotions and empathy, and personality traits. A study conducted by Reis et al. (2007) indicated that while solving MSCEIT problems the brain areas activated were the left frontal polar and left anterior temporal regions which are linked to social cooperation. Another study (Lane, Quinlan, Schwartz, Walker, & Zeitlin, 1998) with the LEAS indicated that subject with higher scores exhibited greater responsiveness to stimuli in area 24 of the anterior cingulate cortex, which is linked to emotional processing. Researchers have also found that individuals higher in EI utilize less brain activity to solve emotional problems (Jausovec & Jausovec, 2005). In addition, factor analysis studies indicate that EI seems to be related to socio-emotional reasoning but not to creativity (Mayer et al., 2007). Individuals with higher EI scores on several measures correlated with self-judgments of empathic feeling (Mayer et al., 2000). Scores from the MSCEIT Emotional Understanding show the strongest individual relationship with verbal/crystallized intelligence measures across many studies (Mayer et al., 2007).

Emotional Intelligence and Life Effects Research on emotional intelligence demonstrates that it correlates with social relationships in individuals’ environments, their performance in school and work settings, and their well-being. In childhood and adolescence, EI consistently predicts positive social and academic outcomes in children. Skills in emotional regulation and emotional knowledge also affect their well-being (Denham et al., 2003). Studies also suggest that adults with high EI are seen more positively and chosen as friends (Mayer et al., 2007). Negative correlations were found with EI and social deviance, critical remarks, destructive responses. In family and intimate relationships, research outcomes suggest that parental warmth and parental support were slightly correlated with some scales of EI. Several studies on intimate relations show a strong correlation between EI, relationship health and perceived quality of relationships. When comparing EI and academic performance, research shows very little correlation with academic achievement, especially when cognitive intelligence and personality measures are controlled. However, Izard et al. (2001) found that emotional knowledge in preschoolers did predict third grade teachers’ ratings of academic

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aptitude. In the work settings, individuals with high EI scores correlate with successful problem solving, positive working relationships, workplace effectiveness and performance, higher wages, and effective leadership (Mayer et al., 2007). Gender differences in emotional abilities have been shown in many of the aforementioned studies. Emotional Intelligence is correlated with positive life satisfaction, higher self-esteem, and lower evaluations of depression.

Future Directions Further research should be conducted to determine if emotional and social intelligences could be included in the taxonomy of mental abilities (Mayer et al., 2007). Additional empirical studies could be conducted to determine if emotional intelligence predicts a wider range of school and work outcomes than discussed above. Further meta-analyses of effects of EI, focusing in particular on the correlates of measures based on specificability and integrative-model approaches need to be conducted. Additional research should be performed on gender differences in EI. The effects of teaching emotional knowledge and reasoning in school, work, and home settings should be evaluated. Since EI research has only been conducted over the last 2 decades, information gathered from future research will continue to direct emotional intelligence theory and measurement.

References and Readings Denham, S. A., Blair, K. A., DeMulder, E., Levitas, J., Sawyer, K., & Auerbach-Major, S. (2003). Preschool emotional competence: Pathway to social competence. Child Development, 74, 238–256. Goleman, D. (1995). Emotional intelligence. New York: Bantam. Izard, C. E., Fine, S., Schultz, D., Mostow, A. J., Ackerman, B., & Youngstrom, E. (2001). Emotion knowledge as a predictor of social behavior and academic competence in children at risk. Psychological Science, 12, 18–23. Jausovec, N., & Jausovec, K. (2005). Differences in induced gamma and upper alpha oscillations in the human brain related to verbal/ performance and emotional intelligence. International Journal of Psychophysiology, 56, 223–235. Lane, R. D., Quinlan, D. M., Schwartz, G. E., Walker, P. A., & Zeitlin, S. B. (1998). Neural correlates of levels of emotional awareness: Evidence of an interaction between emotion and attention in the anterior cingulate cortex. Journal of Cognitive Neuroscience, 10, 525–535. Mayer, J. D., Roberts, R. D., & Barsade, S. G. (2007). Human abilities: Emotional intelligence. Annual Review of Psychology, 59, 507–536. Mayer, J. D., & Salovey, P. (1997). What is emotional intelligence? In P. Salovey & D. Sluyter (Eds.), Emotional development and emotional intelligence: Educational implications (pp. 3–31). New York: Basic Books.

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Mayer, J. D., Salovey, P., & Caruso, D. (2000). Models of emotional intelligence. In R. Sternberg (Ed.), Handbook of intelligence (pp. 396–420). Cambridge, UK: Cambridge University Press. Mayer, J. D., Salovey, P., & Caruso, D. (2002). Mayer-Salovey-Caruso emotional intelligence test (MSCEIT) user’s manual. Toronto, ON: MHS Publication. Mayer, J. D., Salovey, P., Caruso, D., & Sitarenios, G. (2003). Measuring emotional intelligence with the MSCEIT V2.0. Emotion, 3, 97–105. Reis, D. L., Brackett, M. A., Shamosh, N. A., Kent, A. K., Salovey, P., & Gray, J. R. (2007). Emotional intelligence predicts individual differences in social exchange reasoning. NeuroImage, 35, 1385–1391.

Emotional Lability R OBERT F RANK Kent State University Kent, OH, USA

Synonyms Emotional incontinence

Definition Emotional lability is defined as rapid emotional change, most often associated with changes in affect. Lability is observed conditions such as CVA, traumatic brain injury, senile dementia, schizophrenia, bipolar disorder, or borderline personality disorder. Treatment with various antidepressant medications appears to be the most effective intervention.

Cross References ▶ Pseudobulbar Palsy ▶ Traumatic Brain Injury

References and Readings Caplan, B. (2009). Rehabilitation psychology and neuropsychology with stroke survivors. In R. G. Frank, M. Rosenthal, & B. Caplan, Handbook of rehabilitation psychology, 2nd Edition (pp. 63–94). Washington, D.C.: American Psychological Association. Morris, P., Robinson, R. G., & Raphael, B. (1993). Emotional lability after stroke. Australian and New Zealand Journal of Psychiatry, 27(4), 601–605. Silver, J., Arciniegas, D., & Yudovsky, S. (2005). Psychopharmacology. In J. Silver, T. McAllister, & S. Yudovsky (Eds.), Textbook of traumatic brain injury. Arlington, VA: American Psychiatric.

Emotionality S COTT L. D ECKER , C ATHERINE C ADENHEAD Georgia State University Atlanta, GA, USA

Definition Emotionality includes a variety of subjective feeling states that predictably influence observable behavior and physiological responses for functional purposes related to adaptation. Emotions typically involve multiple components including autonomic, hormonal, behavioral, and cognitive component. Physiological signs of emotions may include change in autonomic nervous system activity which includes changes in heart rate, muscle tension, perspiration, and metabolic changes. Ekman’s research on the cross-cultural invariance of emotional identification and expression is suggestive of emotions as a speciestypical response. The coordination of the emotional state is facilitated by the amygdala. The amygdala, a cluster of nuclei in the limbic system near the temporal lobes, has been found to be important for eliciting a cascade of physiological changes involved in emotional behavior. Various theories of emotions have been proposed. The James–Lange theory postulates that emotions are the byproduct of the physiological changes occurring in the body. In contrast, the Cannon–Bard theory proposed that emotional experience precedes the physiological responses corresponding to emotions. Most contemporary models of emotion provide a synthesis of these two views that include the interacting influence of both cognitive appraisal and physiological events. Assessment of emotional behavior is of critical importance in clinical neuropsychology as many emotional behaviors are symptomatic of clinical conditions. The Diagnostic and Statistics Manual of Mental Disorders Fourth Edition (DSM-IV) includes emotional behaviors or symptoms as part of many disorders including anxiety, depression, schizoaffective, and a variety of other disorders. A variety of instruments are available to screen or assess the emotional symptoms of particular disorders, such as the Beck Depression Inventory, or to widely screen multiple disorders, such as the Emotional Status Exam of the Dean–Woodcock.

Cross References ▶ Emotion ▶ Emotional Intelligence

Encephalitis (Viral)

References and Readings Dean, R. S., & Woodcock, R. W. (2003). Dean-Woodcock neuropsychological battery. Itasca, IL: Riverside Publishing. Ekman, P. (1980). The face of man: Expressions of universal emotions in a New Guinea Village. New York: Garland STPM Press.

Emphysema ▶ Chronic Obstructive Pulmonary Disease

Employment Coach ▶ Employment Specialist

Employment Facilitation ▶ Job Advocacy

Employment Specialist A LLEN N. L EWIS J R *, PAMELA H. L EWIS * Virginia Commonwealth University Richmond, VA, USA

Synonyms

career potential. Employment specialists function as a consultant and work in partnership with others to build on existing supports or develop new supports that meet individualized needs in maximizing career potential. Employment specialists are primarily responsible for providing or securing an assessment of the person with the disability, job development, job placement, job-site training, and ongoing follow along services. The goal of an employment specialist is to train the person with the disability to perform and be successful on the job, then gradually fade out of the picture over time. Employment specialists have the ultimate goal of assisting individuals with disabilities to obtain and succeed in work settings of the person’s choice.

Cross References ▶ Job Coach ▶ Supported Employment

References and Readings Blanck, P. D. (2000). Employment, disability, and the Americans with Disabilities Act: Issues in law, public policy, and research. Evanston: Northwestern University Press, Inc. Griffin, C., Hammis, D., & Geary, T. (2007). The job developer’s handbook: Practical tactics for customized employment. Baltimore: Paul H. Brookes Publishing Co., Inc. Unger, D., Kregel, J., Wehman, P., & Brooke, V. (2002). Employers’ views of workplace supports: VCU charter business roundtable’s national study of employers’ experiences with workers with disabilities. Richmond: VCU RRTC on Workplace Supports and Job Retention Monograph. Wehman, P., Inge, K. J., Revell, W. G., & Brooke, V. A. (2006). Real work for real pay: Inclusive employment for people with disabilities. Baltimore: Paul H. Brookes Publishing Co., Inc.

Employment coach; Job coach; Job development specialist; Job placement specialist; Vocational specialist; Work specialist

Encephalitis (Viral) Definition A provider or facilitator of services in the work context and community to locate and secure support options for persons with disabilities for the purpose of optimizing full

B RUCE J. D IAMOND, J OSEPH E. M OSLEY William Paterson University Wayne, NJ, USA

Synonyms *The authors are not related by blood or marriage.

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Herpes simplex encephalitis; Meningoencephalitis

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Short Description Viral encephalitis is a condition in which a virus infects the brain, causing immune mediated inflammatory changes and destruction of gray matter that is usually accompanied by inflammation of the adjacent meninges. (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Viral encephalitis sometimes causes irreversible impairments in brain function. Therefore, it is critical to reach the appropriate diagnosis and begin treatment in a timely manner.

Categorization Viral encephalitis is categorized as (1) acute, (2) subacute/ chronic, or (3) parainfectious (Boos & Esiri, 2003). Symptom profiles that deteriorate rapidly over a period of two to three days show an acute onset and require immediate medical intervention. Herpes Simplex Virus (HSV) is the most common cause of viral encephalitis and follows this temporal pattern (Anderson, 2001). By contrast, symptoms that develop over a period of weeks or months are characteristic of subacute/chronic disease processes, such as HIV/AIDS and the prion disorder – Creutzfeldt-Jakob disease. Parainfectious or postvaccination encephalitis is a complication that occurs following a systemic viral infection (e.g., measles), nonspecific febrile illness, or antigen challenge (i.e., immunizations). Individuals often report an asymptomatic period prior to the onset of neurological symptoms which may affect multiple regions of the central nervous system (CNS).

targeted other organs (Anderson, 2001). Therefore, encephalitis falls into categories and subcategories that are characterized by different viruses and underlying disease processes, including acute sporadic, acute epidemic, subacute/chronic sporadic, and subacute/chronic epidemic. Herpes Simplex encephalitis (HSE) is an example of acute sporadic encephalitis, and the AIDS dementia complex is the most frequent form of chronic epidemic encephalitis (Boos & Esiri, 2003). A variety of viral pathogens can invade the CNS and produce encephalitis (see Tables 1 and 2). Often disabling or fatal, the most common cause of acute sporadic viral encephalitis worldwide is the herpes simplex virus (Anderson, 2001; Halperin, 2007). It accounts for 10–20% of viral encephalitis cases in the US and Europe with an estimated prevalence of 0.1–0.4 per 100,000 population per year (Anderson, 2001). Epidemics of viral encephalitis in the US are usually caused by the arboviruses, or arthropod borne viruses, whose principal vector is the mosquito (see Table 2). Within the US, West Nile Virus (WNV) recently became the most common form of vector-borne epidemic encephalitis (Halperin, 2007). The Centers for Disease Control (CDC) reported 687 cases of WNV encephalitis in 2008, with 1,356 total cases of WNV infection resulting in approximately 44 fatalities (CDC, 2009). In addition to WNV, there are four seasonally active arboviruses that are geographically specific and account for most other

Encephalitis (Viral). Table 1 Non-arthropod borne viruses associated with encephalitis Type

Epidemiology Encephalitis can be sporadic or epidemic, and differences in epidemiological and etiological characteristics further help define the various categories of encephalitis (Anderson, 2001; Boos & Esiri, 2003). The clustering of similar cases, which may range from pairs into hundreds of persons, indicates a common transmission vector and points toward particular viral agents (Boos & Esiri, 2003). Transmission mechanisms in epidemics can involve human-to-human transmission, as with influenza and the enteroviruses; insect-to-human transmission, as is the case with arboviruses; and animal-to-human transmission, as seen in Nipah virus encephalitis (Boos & Esiri, 2003). Encephalitis may be a secondary CNS complication in cases where the primary epidemic infection initially

Virus

DNA viruses Herpesviruses

Herpes simplex type 1, herpes simplex type 2, varicella-zoster, cytomegalovirus, epstein-barr virus

Adenoviruses

Adenovirus types 6, 7, 11, 12

RNA viruses Retroviruses

Human immunodeficiency virus type 1

Enteroviruses

Polioviruses, coxsackieviruses group A, coxsackieviruses group- B, echoviruses, enteroviruses 70, 71

Rubivirus

Rubella

Arenavirus

Lymphocytic choriomeningitis

Paramyxoviruses

Measles, mumps

Orthomyxoviruses Influenza, parainfluenza Rhabdovirus

Rabies


Encephalitis (Viral). Table 2 Arthropod-bornea viruses associated with encephalitis Type

Virus

RNA viruses Bunyaviruses California encephalitis, jamestown canyon virus, laCrosse- virus, rift valley fever, snowshoe hare virus (Canada) Flaviviruses Mosquitoborne

West nile virus, Japanese encephalitis (Asia), St. Louis encephalitis, Murray valley encephalitis (Australia, New Guinea)

Tick-borne

Powassan virus, central European encephalitis, Russian spring-summer encephalitis, louping Ill (British islands), Kyassanur forest virus (India)

Orbivirus

Colorado tick fever

Togaviruses

Venezuelan equine encephalitis, western equine encephalitis, eastern equine encephalitis

a

Principal transmission vectors are ticks or mosquitoes

cases of encephalitis in the US: The Eastern Equine, Western Equine, St. Louis, and LaCrosse Viruses (Anderson, 2001; Halperin, 2007). The Powassan virus is present in the US and causes a small number of cases in the Northeast annually. The Japanese arbovirus is the most common cause of epidemic viral encephalitis worldwide. Clusters of infection occur in Eastern Asia from Siberia to India. In Central and South America, the Venezuelan equine virus is the major pathogen. The most common arboviruses in Europe are principally transmitted via ticks during the spring and summer months. The Russian Spring-Summer and Central European viral strains cause the greatest number of human cases of encephalitis, with incidences ranging from 0.9 to 72.5 per 100,000 depending on location.

Natural History, Prognostic Factors, Outcomes The first accounts of inflammatory changes in the brain were reported during the nineteenth century (Boos & Esiri, 2003). It was not until the epidemic of encephalitis lethargica during World War I that detailed case reports noting pathology were recorded. Knowledge of the pathological features associated with encephalitis is based

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upon examination of the brain at autopsy, neuroimaging studies, electroencephalography (EEG), and studies of brain biopsy specimen (Boos & Esiri, 2003). The onset and progression of viral encephalitis is most commonly acute, with encephalitic symptoms usually developing within a 24 h period following a non-specific febrile prodrome (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). In most cases, virus enters the body through the skin (i.e., insect bite, abrasion) or is transmitted via the mucosal, respiratory or gastrointestinal route (Anderson, 2001; Halperin, 2007). Virus replicates at the entry site and travels to the blood, causing a systemic viremia. Thereafter, a secondary viremia ensues as the virus concentrates in various locations such as the lymph tissue, liver, and spleen. The secondary viremia produces a quantity of virus sufficient to breach the vessels of the choroid plexus, the meninges and the brain (Anderson, 2001). Thus, with the exception of rabies virus and neurotropic herpes viruses, virus reaches the CNS hematogenously by passing through the endothelium of the blood-brain barrier (Nath & Berger, 2000). By contrast, the herpes viruses and rabies spread centripetally from the site of infection through the sensory and cranial nerves via axonal transport to the CNS (Anderson, 2001; Halperin, 2007). Adults and older children usually present with symptoms of an acute febrile illness, with evidence of some meningeal inflammation, abnormal mental state, an altered level of consciousness, and either focal or diffuse neurologic signs (Roos & Tyler, 2008). Evidence of systemic infection (i.e., fever, leukocytosis) is not always present, especially in older or immunocompromised individuals (Halperin, 2007). The inclusion of encephalitis in the differential diagnosis will depend upon clinical evidence of brain dysfunction (Halperin, 2007). The generalized or focal neurologic aberrations of encephalitis may be stationary, progressive, or fluctuating, with neurologic signs reflecting the location(s) of the infection and inflammation (Roos & Tyler, 2008). Both focal and generalized seizures occur in more than 50% of patients and may be difficult to control (Nath & Berger, 2000; Roos & Tyler, 2008). The most commonly observed focal symptoms are ataxia, aphasia, hemiparesis, cranial nerve deficits, and involuntary movements (Roos & Tyler, 2008). In some cases, involvement of the hypothalamic-pituitary axis may result in hypothermia or hyperthermia, diabetes insipidus, or autonomic dysfunction (Nath & Berger, 2000; Roos & Tyler, 2008). Level of consciousness may range from mild lethargy to deep coma (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Roos & Tyler, 2008).

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Neuropsychological and Psychological Outcomes Neuropsychological Viral Encephalitis can include motor deficits, aphasia, amnesia, global cognitive decline, and epilepsy (Hokkanen & Launes, 2007). Due to the effects of the virus in the temporal lobes, the most commonly reported neuropsychological impairments, which may occur together or in isolation, are anterograde and retrograde amnesia (Hokkanen & Launes, 2007; Pewter, Williams, Haslam, & Kay, 2007). The retrograde amnesia can include impairments in semantic or episodic memory and in the ability to recall names, faces, and episodes involving familiar or famous people. Less widespread damage to the temporal lobes and fronto-basal areas may result in less severe amnestic disorders (Hokkanen & Launes, 2007). While not typical in HSE, isolated amnesia or category specific anomia has been reported (Hokkanen & Launes, 2007). In addition to aphasia, anomia (i.e., encompassing visual identification, drawing, or sorting of pictures which may suggest widespread loss of semantic knowledge), executive dysfunction (e.g., the Stroop task, letter fluency, card sorting tasks and the Cognitive Estimations Test), and impairments in visual perception (Hokkanen & Launes, 2007; Pewter, Williams, Haslam, & Kay, 2007) have also been reported. Cognitive impairments may remain stable or diminish over time. However, some HSE patients may show a gradual, global cognitive deterioration (Hokkanen & Launes, 2007). Another common sequel of encephalitis is epilepsy, which when accompanied by frequent seizure activity may have a detrimental effect on cognitive functioning, with memory performance particularly vulnerable due to neuronal loss in hippocampal regions (Hokkanen & Launes, 2007). In general, the cognitive deficits in non-HSE encephalitis are less frequent as well as less severe than those in HSE (Hokkanen & Launes, 2007). However, while other forms of encephalitis have received less thorough neuropsychological investigation, cognitive impairments have been reported following herpes simplex virus type 2, St Louis encephalitis, influenza virus types A and B, and Japanese encephalitis (Pewter, Williams, Haslam, & Kay, 2007).

Psychological The patient’s mental state is frequently confused and disoriented with neuropsychiatric symptoms that may include agitation, emotional lability, behavioral disorders, personality changes, hallucinations, and frank psychosis (Roos & Tyler, 2008). In HSE, psychiatric and behavioral symptoms have been reported to precede, accompany,

and follow the acute illness (Hokkanen & Launes, 2007). In some patients, the altered behavior and psychotic episodes are first observed long before the onset of neurological symptoms, which may delay the process of arriving at the correct diagnosis (Hokkanen & Launes, 2007). Following treatment with acyclovir, 40–60% of the HSE survivors demonstrate persistent personality and behavioral abnormalities and instability, but the emotional symptoms appear to be milder (Hokkanen & Launes, 2007). Common symptoms include panic and anxiety disorders, phobic anxiety, major affective disorders (e.g., bipolar), manic behavior, aggressive outbursts, irritability, depression, and obsessive-compulsive behaviors (Hokkanen & Launes, 2007; Pewter, Williams, Haslam, & Kay, 2007). Single-case studies have reported more severe cases with schizophreniform disorder (Pewter, Williams, Haslam, & Kay, 2007). The medical literature suggesting encephalitis as a cause of secondary psychosis appears to consist primarily of case reports or anecdotal observations, with the only reliable finding of psychosis occurring during the degenerative process of chronic HIV encephalitis (Pewter, Williams, Haslam, & Kay, 2007). While psychiatric symptoms may reflect an emotional reaction to a possibly fatal illness, damage to the limbic system and amygdalo-frontal pathways may be key etiological factors (Hokkanen & Launes, 2007). The neuropsychiatric symptoms arising from limbic lobe involvement are well-known and can include emotional or mood disorders (e.g., rage, aggression, depression and mania), delusions, hallucinations, anxiety and dissociative disorders as well as sexuality changes, and hyperoral behaviors (Hokkanen & Launes, 2007).

Prognosis The prognosis for encephalitis depends upon the strain of virus, the degree to which level of consciousness is altered, and the age of the patient (Boos & Esiri, 2003; Nath & Berger, 2000; Roos & Tyler, 2008). Arbovirus-associated encephalitis has a variable mortality rate. Eastern equine encephalitis is the most virulent strain and thus has the highest mortality rates. California virus encephalitis has the lowest mortality rate. Among the population, children younger than age 4 and the elderly have the highest mortality rates. Encephalitis caused by eastern, western, and St. Louis viruses has a comparatively high rate of neurologic sequelae (Roos & Tyler, 2008). Approximately 80% of survivors of Eastern equine encephalitis have severe neurologic deficits (Roos & Tyler, 2008). However, Epstein-Barr viral encephalitis, California encephalitis virus, and Venezuelan equine encephalitis rarely result in


sequelae (Roos & Tyler, 2008). Patients who present with severe neurologic impairments (Glasgow coma score 6) either die or survive with severe sequelae. Younger patients (30), with a relatively low degree of altered consciousness at the initiation of therapy, do significantly better (100% survival, 62% with no or mild sequelae) than older (30) patients (64% survival, 57% with no or mild sequelae) (Roos & Tyler, 2008). Overall, better long-term outcomes in HSE are associated with right-lateralized effects (Laurent et al., 1990).

Evaluation Clinical evidence of both inflammation and of brain involvement is required for the diagnosis (Halperin, 2007). Diagnosis requires an explanation of the patient’s systemic inflammatory response (if present) and encephalopathy by systematically excluding infectious and noninfectious mimics of encephalitis (Boos & Esiri, 2003; Halperin, 2007; Roos & Tyler, 2008). Differential diagnosis is complicated by the fact that other illnesses may produce the symptoms of viral encephalitis, such as the vascular diseases, tumors, abscess, fungal, parasitic, rickettsial and tuberculous infections, Reye’s syndrome, toxic encephalopathy, subdural hematoma, and systemic lupus erythematosus (Roos & Tyler, 2008). The main diagnostic priority is to identify treatable causes of acute encephalitis, and determination of etiology is also essential from prognostic, therapeutic, and public health perspectives (Halperin, 2007; Roos & Tyler, 2008). The three treatable agents responsible for encephalitis are HSV-1, HSV-2, and varicella-zoster virus (Halperin, 2007). A careful epidemiological investigation is part of the case history and may help provide a focus for the clinical and laboratory assessments (Boos & Esiri, 2003; Roos & Tyler, 2008). Consideration should be given to the possibility of exposure to infectious diseases at home, work, and during travel and to season of the year, patient age, geographic region, and possible exposure to animal or insect bites. If viral encephalitis or other parenchymal brain disease is suspected, the diagnostic approach to the patient will include neuroimaging, lumbar puncture, cerebrospinal fluid (CSF) examination, serological studies, electroencephalography (EEG), and (rarely) brain biopsy (Boos & Esiri, 2003; Halperin, 2007; Roos & Tyler, 2008). Computed Tomography (CT) may be used to identify mass effect, but MRI using contrast agents is more sensitive in demonstrating changes in cerebral edema, white matter disturbances, infarction, and blood-brain barrier

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irregularities (Anderson, 2001; Halperin, 2007). ICP monitoring should be implemented if the Glasgow Coma Scale (GCS) is 8, or if there is imaging evidence of significant cerebral edema that cannot be controlled (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). If imaging demonstrates a lack of posterior fossa or supratentorial mass effect and intracranial pressure is not dangerously elevated, lumbar puncture can be performed with minimal risk of herniation (Anderson, 2001; Halperin, 2007; Roos & Tyler, 2008). The characteristic encephalitic CSF findings are a lymphocytic pleocytosis (10–1,000 cells/mm3), a slightly elevated protein level, and normal glucose level (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Early examination may show polymorphonuclear leukocytes, and around 20% of patients will have a significant number of red blood cells (>500/L) in the CSF (Anderson, 2001; Nath & Berger, 2000; Roos & Tyler, 2008). Bacterial cultures and polymerase chain reaction (PCR), along with CSF and serum antibody estimation, should be performed for herpes and other etiologic agents as deemed necessary based upon case history and epidemiologic factors (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). EEG is used infrequently as a diagnostic tool. However, it can help corroborate the Encephalitis diagnosis. Patients often exhibit an abnormal EEG, with diffuse slow wave activity that is directly related to the severity of the infection (Anderson, 2001). Herpes simplex encephalitis has a somewhat characteristic EEG profile involving abnormal activity from a predominantly temporal focus that may include spike and slow wave activity, and periodic lateralized epileptiform discharges (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). The need for brain biopsy to definitively diagnose encephalitis has greatly declined with the development and availability of CSF PCR amplification techniques for HSV and other viruses (Boos & Esiri, 2003; Roos & Tyler, 2008). However, biopsy is considered when clinical and diagnostic studies are inconclusive and/or suggest the possibility of another illness (Boos & Esiri, 2003).

Treatment Early stage severe encephalitis may require the resources of an intensive care unit (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). As with any delirious or unconscious patient, basic management and supportive therapy include monitoring and maintaining satisfactory nutrition, hydration,

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electrolyte balance, respiration, blood pressure, and body temperature (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Because of the risk of raised intracranial pressure (ICP), ICP must be repeatedly assessed to ensure proper brain perfusion and avoid secondary ischemia, infarction, and tissue shifting (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Roos & Tyler, 2008). The typical first line treatment for increased ICP is administration of Mannitol (1–2 g/kg) in a 20% solution (Halperin, 2007). Seizures, which develop in approximately 50% of severe cases (Roos & Tyler, 2008), can be controlled with anticonvulsants, and prophylactic therapy should be considered if ICP is elevated and/or if there is evidence of substantial cerebral swelling or mass effect (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Drug therapy for viral encephalitis is limited to specific pathogens, but should be introduced as soon as a presumptive diagnosis is reached (Boos & Esiri, 2003; Griffin, 2000; Roos & Tyler, 2008). Acyclovir is an effective treatment for HSV encephalitis, as well as infections due to the Epstein-Barr and Varicella-Zoster viruses (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). The recommended dosage is 10–15 mg/kg of acyclovir i.v. every 8 h for 14–21 days (Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Roos & Tyler, 2008). The relatively alkaline pH of acyclovir may result in phlebitis (Roos & Tyler, 2008). Other side effects may include elevated creatinine levels, thrombocytopenia, gastrointestinal disturbances, and neurotoxicity (Roos & Tyler, 2008). Ganciclovir and foscarnet have proven to be effective against cytomegalovirus infections of the CNS. The usual induction dose of ganciclovir is 5 mg/kg i.v. every 12 h, followed by a 5 mg/kg daily maintenance dose (Roos & Tyler, 2008). Foscarnet is administered at an induction dosage of 60 mg/kg i.v. every 8 h, switching to a maintenance dose of 60–120 mg/kg each day (Roos & Tyler, 2008). Therapy of acute parainfectious encephalitis involves immunomodulating or immunosuppressive drug regimens (Boos & Esiri, 2003). The first line treatment is usually pulse steroids, which in non-responders may be followed by i.v. immunoglobulin (Ig), plasmapheresis, or repeat steroid treatment (Boos & Esiri, 2003). The neurological complications of HIV infection, categorized as a chronic encephalitic condition, are managed with antiretroviral treatments (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007). Encephalitis patients who are comatose should receive chest physical therapy and passive range of motion

exercises (Halperin, 2007). Stretching exercises reduce the likelihood of contractures (Halperin, 2007). Neurorehabilitation can help mitigate impairments due to brain injury (Halperin, 2007). Speech therapy may be indicated in certain cases, and pharmacological intervention with a combination of zolpidem and selegiline or bromocriptine may be beneficial for abulic patients with severe catatonia and rigidity (Halperin, 2007).

Cross References ▶ Acquired Immunodeficiency Syndrome (AIDS) ▶ Brain Swelling ▶ Cerebral Edema ▶ Cerebral Perfusion Pressure ▶ Epstein-Barr Virus ▶ Intracranial Pressure ▶ Lumbar Puncture ▶ Mass Effect ▶ Meningitis ▶ Prion Disease

References and Readings Anderson, M. (2001). Encephalitis and other brain infections. In M. Donaghy (Ed.), Brain diseases of the nervous system (pp. 1117–1180). New York: Oxford University Press. Boos, J., & Esiri, M. M. (2003). Viral encephalitis in humans. Washington, DC: ASM Press. Centers for Disease Control. (Apr. 2009). 2008 West Nile virus activity in the United States (Reported to CDC as of April 10, 2009). Retrieved July 5, 2009 from http://www.cdc.gov/ncidod/dvbid/westnile/surv & controlCaseCount08_detailed.htm Griffin, D. E. (2000). Encephalitis, myelitis, and neuritis. In L. G. Mandell, J. E. Bennett, & R. Dolin (Eds.), Principles and practice of infectious diseases (pp. 1143). Philadelphia, PA: Churchill Livingstone. Halperin, J. J. (Ed.). (2007). Encephalitis: Diagnosis and treatment. New York: Informa Healthcare. Hokkanen, L., & Launes, J. (2007). Neuropsychological sequelae of acuteonset sporadic viral encephalitis. Neuropsychological Rehabilitation, 17(4/5), 450–477. Laurent, B., Allegri, R. F., Michel, D., Trillet, M., Naegele-Faure, B., Foyatier, N., et al. (1990). Primarily unilateral herpes encephalitis. Long-term neuropsychological study of 9 cases. Revue Neurologique (Paris), 146, 671–681. Nath, A., & Berger, J. R. (2000). Acute viral meningitis and encephalitis. In L. Goldman & J. C. Bennett (Eds.), Cecil textbook of medicine (pp. 2123–2126). Philadelphia, PA: W. B. Saunders. Pewter, S. M., Williams, W. H., Haslam, C., & Kay, J. M. (2007). Neuropsychological and psychiatric profiles in acute encephalitis in adults. Neuropsychological Rehabilitation, 17(4/5), 478–505. Roos, K. L., & Tyler, K. L. (2008). Meningitis, encephalitis, brain abscess and empyema. In B. Fauci, H. Kasper, J. Longo, & Loscalzo (Eds.), Harrison’s principles of internal medicine (17th ed., pp. 2621–2640). New York: McGraw Hill.

Endothelial Proliferation

Encephalopathy J ENNIFER C. G IDLEY L ARSON , YANA S UCHY University of Utah Salt Lake City, UT, USA

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Encephalopathy of Prematurity ▶ Periventricular Leukomalacia

Endolymphatic Hydrops Synonyms

▶ Meńie`re’s Syndrome

Brain disease

Endothelial Proliferation Definition Encephalopathy is a nonspecific term that refers to diffuse dysfunction within the brain that causes disturbances in function and mental status. Encephalopathy can be associated with either morphological changes within the brain or metabolic imbalances. It originates from a variety of factors including, but not limited to, genetic susceptibility/mutation, traumatic brain injury, cerebrovascular accident, psychiatric disorders, toxic agents, and systemic disease. Encephalopathy can either be acute (i.e., sudden onset such as metabolic encephalopathy or traumatic brain injury), chronic (i.e., gradual deterioration such as dementia, HIV, or schizophrenia), or static (i.e., a nonprogressive abnormality such as mental retardation).

Cross References ▶ Alzheimer’s Disease ▶ Binswanger’s Disease ▶ Brain Tumor ▶ Brainstem Glioma ▶ Dementia ▶ Huntington’s Disease ▶ Hypertensive Encephalopathy ▶ Parkinson’s Disease ▶ Toxic-Metabolic Encephalopathy

References and Readings Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sutherland, MA: Sinauer Associates. Kumar, V., Fausto, N., & Abbas, A. (2004). Robbins & Cotran pathologic basis of disease (7th ed.). Philadelphia, PA: Saunders.

C AROL L. A RMSTRONG The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms Vascular endothelial proliferation

Definition Endothelial proliferation is an increase in vascular endothelial cells needed for the growth of new or existing blood vessels. It is stimulated by solid tumors that need to generate blood vessels to continue growth. It contributes to angiogenesis in tumor formation (Louis & Cavenee, 2005), and results from overexpression of vascular endothelial growth factor (VEGF). Endothelial proliferation is one of the characteristics of malignancy in gliomas as defined by the World Health Organization’s primary central nervous system tumor grading system (Kleihues, Burger & Scheithauer, 1993). Endothelial proliferation is associated with breakdown in the blood–brain barrier (Kracht et al., 2004). It is a feature of malignancy that indicates poor prognosis, but is considered in combination with other features which is not unique.

References and Readings Kleihues, P., Burger, P. C., & Scheithauer, B. W. (1993). The new WHO classification of brain tumours. Brain Pathology, 3(3), 255–268. Kracht, L. W., Miletic, H., Busch, S., Jacobs, A. H., Voges, J., Hoevels, M., et al. (2004). Delineation of brain tumor extent with [11C]Lmethionine positron emission tomography: Local comparison with stereotactic histopathology. Clinical Cancer Research, 10, 7163–7170. Louis, D. N., & Cavenee, W. K. (2005). Molecular biology of central nervous system tumors (pp. http://brain.mgh.harvard.edu/MolecularGenetics. htm). Boston, MA: Massachusetts General Hospital, MGH Neurosurgical Service, Brain Tumor Center.

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Endotracheal Tube

Endotracheal Tube A NUJ S HARMA Virginia Commonwealth University School of Medicine Richmond, VA, USA

Synonyms Breathing tube; ETT

Definition Endotracheal tube (ETT) is a tube placed into the trachea for mechanical ventilation and management of a patient’s airway. The tube is placed in the patient’s trachea to ensure a patent airway allowing oxygen, anesthetics, or gaseous medications to reach the lungs.

Types The different types of ETT are cuffed or uncuffed, oral or nasal, reinforced tubes, double lumen tubes, and tracheostomy tubes. The internal diameter of these tubes range from 2–10.5 mm and are chosen based on the patient’s body size, with smaller sizes used in neonates and pediatric patients. While most tubes used are cuffed, uncuffed tubes are often used in pediatric patients under eight years of age. Nasal tubes are often utilized for intubation during faciomaxillary surgeries. Reinforced tubes are used when there is a concern that the ETT will be damaged during intubation. However, these tubes cannot be cut due to an internal metal ring. Also, these tubes often slide down the right main bronchus resulting in the ventilation of only one lung. Conversely, double-lumen endo-bronchial tubes are often used during thoracic surgery to preferentially ventilate one lung. The other lung is purposefully collapsed making it easier to operate on the deflated lung. A tracheostomy tube is a shortened tube inserted into the trachea through an opening in the neck.

Current Knowledge Complications Intubation, usually performed with general anesthesia, is the process of inserting an ETT. A laryngoscope, which consists of a blade of varying shapes and sizes, a handle, and a light source, is an instrument used to help visualize the larynx and surrounding structures. A stylet, a malleable piece of metal placed inside the ETT, can also be used to aid intubation by providing stiffness and curvature at the end of the tube allowing for easier insertion. It is removed after intubation and the ETT is attached to a ventilator or selfinflating bag. Placement of the ETT is confirmed by auscultating both sides of the chest with a stethoscope.

Indications for ETT Endotracheal intubation should be considered when establishment of a definitive airway is needed for a number of circumstances: respiratory arrest; respiratory failure; airway obstruction; inadequate air exchange; need for prolonged ventilatory support; severe flail chest or pulmonary contusion; multiple trauma, head injury, and abnormal mental status; status epilepticus; inhalation injury; protection from aspiration; patient unable to protect his airway; when other means of ventilation are not possible or not effective; or when undergoing general anesthesia.

During ETT intubation, trauma can occur to teeth, oropharynx, or vocal cords and may result in edema or bleeding. Also, if the ETT is misplaced deep into either the right of left main bronchus, only one lung will be adequately ventilated. This may result in a pneumothorax of the ventilated lung. Inadequate ventilation will also occur if the ETT is incorrectly placed in the esophagus. Additionally, aspiration of stomach contents can occur resulting in pneumonia or acute respiratory distress syndrome. Other rare but more severe complications include tracheal or esophageal perforation, spinal cord and vertebral column injury, cardiorespiratory arrest, brain damage, and death.

Cross References ▶ Polytrauma ▶ Traumatic Brain Injury

References and Readings Divatia, J. V., & Bhowmick, K. (2005). Complications of endotracheal intubations and other airway management procedures. Indian Journal Anaesthesia, 49(4), 308–318.

Environmental Dependency Finucane, B. T., & A. H. Santora. (2003). Principles of airway management. New York: Springer. Stewart, C. E. (2002). Advanced airway management. Upper Saddle River, NJ: Prentice Hall Publishing.

Enhancement R ONALD A. C OHEN Brown University Providence, RI, USA

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stimuli rather than stimulus-evoked responses. Attentional biases created by constantly changing ‘‘salience maps’’ in the parietal lobes influence the probability of response to particular locations or features at the moment when the stimulus occurs (Gee, Ipata, Gottlieb, Bisley & Goldberg, 2008). Ultimately, visual-selective attention seems to be governed by enhancement occurring as a result of the preexisting response biases and the effects of cueing resulting from information associated with new stimuli as they are processed. Enhancement has direct implications for explaining the covert shifts of attention that have been the subject of extensive cognitive neuroscientific inquiry (Posner, Walker, Friedrich & Rafal, 1987).

Definition Cross References Enhancement is the neural process by which the intensity of focus is increased for specific locations to facilitate spatial-selective attention.

▶ Inferior Parietal Cortex ▶ Selective Attention

Current Knowledge


A relationship is known to exist between selective attention and an increase in the response of neurons in visual areas when a laboratory animal focuses on stimuli in the receptive field. This was first discovered for neurons in the superior colliculus of monkeys using electrophysiological methods (Goldberg & Wurtz, 1972), as neurons responded more intensely to the onset of a stimulus when saccadic movement to the stimulus was required, compared with when they maintained fixation. Subsequently, similar neuronal enhancement was demonstrated across a variety of other brain regions containing higher-order visual processing areas, including the inferior parietal and prefrontal cortex, though similar responses have also been found in other extrastriatal areas (e.g., V4) and even the primary visual cortex. These studies were considered to provide an essential foundation for explaining the neural mechanisms by which increased attention to a stimulus occurs triggered by initial stimulation, which results in an ‘‘enhanced’’ response to the stimulus and its spatial context. This model of visual-selective attention assumes a bottomup approach, in which attention is driven by neuronal events caused by prior stimulation. Considerable visual neuroscience research has focused on the mechanisms that drive enhancement, and spatial, temporal, and other factors that constrain the response. In studies conducted over the past decade, there has been increasing evidence that ‘‘top-down’’ processes may have greater influence on the neuronal response to novel

Gee, A. L., Ipata, A. E., Gottlieb, J., Bisley, J. W., & Goldberg, M. E. (2008). Neural enhancement and pre-emptive perception: the genesis of attention and the attentional maintenance of the cortical salience map. Perception, 37(3), 389–400. Goldberg, M. E., & Wurtz, R. H. (1972). Activity of superior colliculus in behaving monkey. II. Effect of attention on neuronal responses. Journal of Neurophysiology, 35(4), 560–574. Posner, M. I., Walker, J. A., Friedrich, F. A., & Rafal, R. D. (1987). How do the parietal lobes direct covert attention? Neuropsychologia, 25(1A), 135–145.

Environmental Adaptation ▶ Environmental Modifications

Environmental Dependency DAVID J. L IBON , J OEL E PPIG , D ENENE M. WAMBACH , C HRISTINE N IEVES Drexel University, College of Medicine Philadelphia, PA, USA

Synonyms Imitation behavior; Pull to stimulus; Utilization behavior

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Environmental Dependency

Short Description or Definition Environmental dependency refers to instances when the visual or visuo-tactile presence of objects ‘‘compels’’ patients to either grasp and/or use the objects.

Categorization Environmental dependency is usually best understood as part of greater, superordinate frontal lobe syndrome.

Epidemiology The incidence and prevalence of environmental dependency syndrome is unknown.

Natural History, Prognostic Factors, Outcomes Environmental dependency syndrome or utilization behavior has not been extensively studied. Therefore, there is little information regarding natural history, prognostic factors, and outcomes.

Neuropsychology and Psychology of Environmental Dependency Syndrome As noted above, environmental dependency refers to instances when the mere visual or visuo-tactile presence of objects ‘‘compels’’ patients to either grasp the object and/ or use it. When patients with environmental dependency syndrome or utilization behavior are pulled to grasp and use objects, patients general show intact recognition of the object and its proper use. In this sense, environmental dependency or utilization behavior is not associated with an appreciative or associative agnosia. Lhermitte (1983) noted that elements of an environmental dependency syndrome were first described by Wilson and Walshe (1914) and Adie and Critchley (1927). These authors described patients with ‘‘forced grasping’’ of objects in association with either left or right frontal lobe lesions. Denney-Brown (1958) is generally credited with coining the term magnetic apraxia to describe a complex manual response or activity where patients not only grasp but may also use objects correctly though in an inappropriate context. For

example, presented with the examiners glasses and merely asked to name the object, a patient with magnetic apraxia may take the object from the examiner and put them on. Denney-Brown (1958) interpreted this phenomenon as representing a release behavior mediated by the parietal lobe due to the suppression of inhibitory mechanisms that would be otherwise governed by the frontal lobes. Lhermitte (1983) described a series of patients who exhibited environmental dependency syndrome/ utilization behavior. The aetiology of the underlying neurological condition was variable. However, when the examiner displayed common objects, all patients grasped the object from the examiner and would demonstrate its correct use. In this series of case studies, Lhermitte commented that other aspects of a frontal lobe syndrome were invariably present. In several patients, environmental dependency syndrome/utilization behavior was present acutely but then resolved. All patients described by Lhermitte (1983) presented with evidence of left or right frontal lobe lesions; however, he was reluctant to associate these behaviors to a precise locale within the frontal lobes solely on the basis of this handful of cases. In addition to this series of case studies, Lhermitte (1983) described a formal examination procedure for the presence of environmental dependency/utilization behavior whereby the examiner sits in front of the patient with common objects. The first test condition is designed to establish a baseline for the presence of environmental dependency/utilization behavior, in that patients are shown objects and utilization behavior is observed. In the second test condition, patients are specifically told not to either grasp or use objects that are presented. If patients once again grasp the displayed objects in this second test condition, the examiner may consider the patient as having an environmental dependency/ utilization behavior. In his 1983 report, Lhermitte describes testing a neurologically normal control group where neither test condition elicited this type of grasping behavior. Better known, perhaps, are a series of two papers authored by Lhermitte and colleagues in 1986. In part one of this series, Lhermitte, Pillon, and Serdaru (1986) prospectively tested 75 patients who were divided into three groups presenting with focal lesions involving the frontal lobes, as well as non-focal frontal lesions: group 1 demonstrated what Lhermitte et al. (1986) called imitation behavior. Using testing procedures described by Lhermitte (1983), these patients imitated the examiner’s gestures despite being told not to do so; group 2 demonstrated both imitation behavior and utilization behavior as described by Lhermitte (1983); group 3 presented with

Environmental Dependency

neither imitation nor utilization behavior. Neuropsychological and neurological assessment was obtained on all participants, as well as laboratory studies such as CT scans. Among patients with focal lesions, neuropsychological testing found greater perseveration in groups 1 and 2 compared to group 3 on tests such as Luria’s Graphical Sequences (see Luria, 1980, pp. 296–309; Goldberg, 1986; Lamar, Podell, Carew, Cloud, Kennedy, & Goldberg, 1997), Luria’s three-step hand sequence (fist-palm-side; Luria, 1980), and the Wisconsin Card Sorting Test. No differences were noted when groups 1 and 2 were compared. Lhermitte et al., (1986) also commented on differences in behavior such that groups 1 and 2 exhibited greater indifference, disinterestedness, indifference to social rules, and apathy compared to group 3. Less specificity with respect to impairment on executive or frontal lobe tests was found in patients with nonfocal lesions. An analysis of CT scan found that ‘‘the lower half of the frontal lobe was affected in all patients’’ with less imitation and utilization behavior in ‘‘upper region’’ of the frontal lobes (p. 330). Also, few patients with posterior or retro-rolandic lesions exhibited either imitation or utilization behavior. Finally, imitation and utilization behavior could be found in patients with caudate and/or thalamic lesions. Lhermitte et al., (1986) describe imitation behavior as a less severe presentation of either utilization behavior or an environmental dependency syndrome. The authors stress an ‘‘imbalance in patients between dependence on and independence from external stimuli, which leads them to become dependent on these stimuli. ‘‘The sight of an object implies the order to use it’’ (p. 331). Lhermitte et al., (1986) assert both imitation and utilization behavior can be present in patients with dementia, but that general intellectual deterioration fails to either predict the presence or severity of imitation or utilization behavior. These authors went on to describe several patients in whom imitation or utilization behavior could be elicited who were able to return to relatively normal activities. Lhermitte (1986) studied in vivo two patients with utilization behavior/environmental dependency syndrome; that is, patients were studied in natural settings including a ‘‘doctor’s office, a lecture room, a car, a garden, an apartment where various activities were possible, and a gift shop’’ (p. 335). Both patients had documented frontal lobe lesions. In this paper, Lhermitte (1986) accompanied both patients while they engaged in natural or normal daily activates. The degree each patient either imitated behavior or engaged in behavior that was inappropriate, but contextually consistent with their

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environment, is quite striking. Lhermitte’s description of the behavior of his patients is quoted below: " On the same day on which Patient 1 had walked around the

apartment as if it were part of a museum, we returned to the bedroom. The bedspread had been taken off and the top sheet had been turned back in the usual way. When the patient saw this, he immediately began to get undressed. He got into bed, pulled the sheet up to his neck, and prepared to go to sleep. Later, when I picked up an article of clothing, the patient got up and dressed in an orderly fashion. (p. 339) As soon as Patient 2 saw the bed, she tucked in the covers on both sides. She did not get undressed. I walked toward the bed carrying my stethoscope. The patient lay down immediately. Realizing that the clothes were getting in my way, she helped me unbutton her blouse and undo her brassiere, so that her chest was completely bare. After this test, she accompanied me to a table where various items used for intramuscular injections were laid out. I went through the movements of preparing an injection. She immediately lifted her dress and pulled down her pantyhose to bare her right buttock. Later, I showed her the syringe. She took it. When I took off my jacket and shirt, she picked up the needle and a cotton ball, which she soaked in antiseptic, then bent down to my buttock to give the injection. (p. 340)

In describing this very striking behavior, Lhermitte (1986) stresses the environmental context in which behavior occurs. Patients are unable to desist or reframe from action, that is, objects in the environment and/or the context of the situation appears to ‘demand’ action on the part of the patient despite the inappropriate nature of their action or behavior. This is clearly indicated by patient 2 who, upon seeing a syringe and related equipment, prepares to administer an injection to Dr. Lhermitte. Lhermitte (1983, 1986) and Lhermitte et al., (1986) clearly document an association between utilization behavior/environmental dependency syndrome and frontal lobe lesions. However, these behaviors have also been described in dementia and other medical/neurological conditions (Conchiglia, Della Rocca, & Grossi, 2007; Hoffmann, 2007; Hoffmann & Bill, 1992; Tanaka, Albert, Hara, Miyashita, & Kotani, 2000).

Evaluation No formal, normative-based assessment is available for the presence and/or severity of environmental dependency syndrome.

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Environmental Illness

Treatment No formal treatment studies for environmental dependency are available.

Cross References ▶ Perseveration

References and Readings Adie, W. J., & Critchley, M. (1927). Forced grasping and groping. Brain, 50, 142–170. Conchiglia, G., Della Rocca, G., & Grossi, D. (2007). On a peculiar environmental dependency syndrome in a case with frontal– temporal damage: Zelig-like syndrome. Neurocase, 13, 1–5. Denney-Brown, D. (1958). Nature of apraxia. Journal of Nervous and Mental Disease, 126, 9–32. Goldberg, E. (1986). Varieties of perseveration: A comparison of two taxonomies. Journal of Clinical and Experimental Neuropsychology, 8, 710–726. Hoffmann, M. (2007). Transient environmental dependency syndrome due to phendimetrazine tartrate. European Neurology, 58, 49–50. Hoffmann, M. W., & Bill, P. L. (1992). The environmental dependency syndrome, imitation behaviour and utilisation behaviour as presenting symptoms of bilateral frontal lobe infarction due to moyamoya disease. South African Medical Journal, 81, 271–273. Lamar, M., Podell, K., Carew, T. G., Cloud, B. S., Kennedy, C., & Goldberg, E. (1997). Perseverative behavior in Alzheimer’s disease and subcortical ischaemic vascular dementia. Neuropsychology, 11, 523–534. Lhermitte, F. (1983). ‘Utilization behavior’ and its relation to lesions of the frontal lobes. Brain, 106, 237–255. Lhermitte, F., Pillon, B., & Serdaru, M. (1986). Human autonomy and the frontal lobes. Part I. Imitation and utilization behavior: A neuropsychological study of 75 patients. Annals of Neurology, 19, 326–334. Lhermitte, F. (1986). Human autonomy and the frontal lobes. Part II. Patient behavior in complex and social situations: The ‘‘environmental dependency syndrome’’. Annals of Neurology, 19, 335–343. Luria, A. R. (1980). Higher cortical functions in man. New York: Basic Books. Paquier, C. R., & Assal, F. (2007). A case of oral spelling behavior: Another environmental dependency syndrome. Cognitive and Behavioral Neurology, 20, 235–237. Tanaka, Y., Albert, M. L., Hara, H., Miyashita, T., & Kotani, N. (2000). Forced hyperphasia and environmental dependency syndrome. Journal of Neurology, Neurosurgery, and Psychiatry, 68, 224–226. Wilson, S. A. K., & Walshe, F. M. R. (1914). The phenomenon of tonic innervation and its relation to motor apraxia. Brain, 37, 199–246.

Environmental Illness ▶ Multiple Chemical Sensitivity

Environmental Intervention ▶ Environmental Modifications

Environmental Modifications J AY B EHEL Rush University Medical Center Chicago, IL, USA

Synonyms Environmental adaptation; Environmental intervention; Home modification

Definition Environmental modifications (EM) entail the rearranging, rebuilding, or retrofitting of an existing space or building to make it accessible, usable, and safe either for a specific individual or for people with disabilities, in general. This type of modification can involve installing equipment, simple structural changes to a single room or significant alterations to an entire structure.

Current Knowledge Modifications may be undertaken (1) out of an individual’s desire to optimize mobility and independence; (2) family’s concern about enhancing the safety of a physically or cognitively compromised individual; (3) under direction from a rehabilitation professional; or (4) under a legal mandate such as the American Disabilities Act. Whatever the case, EM can entail simple installation of prefabricated adaptive devices (durable medical equipment, DME) such as grab bars and a shower bench, significant structural changes such as adding a ramp or widening a doorway or a combination of the two. DME can be fabricated to an individual’s needs or specifications but is much more commonly mass produced with limited capacity for adjustment. EM within one’s own home or other permanent residence typically are most extensive. However, the principles and technical changes associated with home modification may be applied to the workplace and other places of public accommodation. Although restraints and other

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devices that manage the mobility of ambulatory but cognitively compromised individuals (such as gates between rooms) typically are not considered as comparable to mobility-enhancing EM, the concept of restructuring a preexisting space for an individual’s well being certainly extends to this class of modifications. Goals for EM: Although a desire to optimize safety, mobility, and independence underpins most EM projects, there is considerable variability in the pragmatic goals of any given set of modifications. Such goals may include facilitating completion of basic activities of daily living (ADL) such as feeding, grooming, and toileting oneself. At higher levels, independent ADL’s (IADL) such as meal preparation and household chores such as laundry may be integrated into planned EM. Finally, environmental modifications may be undertaken to facilitate community reentry and return to work, a class of adaptations that typically entails changes beyond the home. Finances: Generally, a narrowly defined subset of DME is covered by Medicare, Medicaid, and many private insurance companies. Moreover, some DME companies have programs to provide this equipment to individuals with limited financial resources. Beyond DME, EM are not directly paid for by insurers. Although some State, Federal, and private assistance is available, the costs of EM typically are borne by the individual, rendering some types of EM inaccessible to significant numbers of people with disabilities. Trend and Counter-trend: Historically, DME and mass produced accessibility hardware has been medical in appearance, utilitarian in design, and uniform in size and shape. However, with an aging population and assertive disability communities, the design and appearance of EM have become increasingly consumer driven and tailored for specific individuals, spaces, and tastes. In the United States, there has been a recent trend toward newly constructed housing for older adults and to a lesser extent, people with disabilities. These new structures are designed to be accessible from the outset thus obviating any need for modification in the future.

Cross References ▶ Community Reentry ▶ Discharge Planning

References and Readings Fielo, S. B., & Warren, S.A. (2001). Home adaptation: Helping older people age in place. Geriatric Nursing. 22(5), 239–247.

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Tse, T. (2005). The environment and falls prevention: Do environmental modifications make a difference? Australian Occupational Therapy Journal. 52, 271–281.

Enzyme J OSE A. R EY Nova Southeastern University Ft. Lauderdale, FL, USA

Definition Enzymes are protein molecules that are necessary to cellular processes and are potential pharmacological targets for the treatment of various diseases. The purpose of an enzyme is to act as a catalyst and lower the activation energy needed to carry out a biochemical reaction, and they often greatly increase the rate of a reaction as well. Most enzyme names have the suffix ‘‘-ase’’ attached to the substrate of the reaction or to describe the action performed. Enzymes assist in converting a given substrate into a different product. This process may result in breaking down an existing molecule (catabolism) into more basic components, or through synthesis, the combination and creation of a larger product (anabolism). When the substrate, and other molecules, interact with the enzyme at its active site, a conformational (or allosteric) change in the enzyme’s shape may occur to influence (induce or inhibit) the activity of the enzyme. Factors potentially affecting enzyme specificity for a substrate include charge, shape, or hydrophilic qualities of both the enzyme and the complimentary substrate. Examples of enzymes that are targets of psychopharmacological agents include acetylcholinesterase and monoamine oxidase types A and B. In these examples, the goal of pharmacotherapy is to increase the synaptic concentration of the respective neurotransmitter that is naturally metabolized (broken down) by these enzymes. Enzyme inhibition may be either reversible or irreversible and either competitive or noncompetitive. The family of oxidative enzymes known as cytochrome P450 (e.g., CYP-2D6), which are a necessary part of phase I metabolism of many psychopharmacological agents, may be involved in drug– drug interactions when inhibited (e.g., fluoxetine) or induced (e.g., carbamazepine) by other pharmacological agents.

References and Readings Brunton, L. L., Lazo, J. S., & Parker, K. L. (Eds.). (2006). Goodman & Gilman’s The pharmacological basis of therapeutics (11th ed.). New York: McGraw-Hill.

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Cooper, J. R., Bloom, F. E., & Roth, R. H. (Eds.). (2003). The biochemical basis of neuropharmacology (8th ed.). New York: Oxford University Press. Davis, K. L., Charney, D., Coyle, J. T., & Nermeroff, C. (Eds.). (2002). Neuropsychopharmacology: The fifth generation of progress. Philadelphia, PA: Lippincott Williams & Wilkins. Stahl, S. M. (2008). Stahl’s essential psychopharmacology: Neuroscientific basis and practical applications (3rd ed.). New York: Cambridge University Press. Voet, D., Voet, J. G., & Pratt, C. W. (Eds.). (2008). Fundamentals of biochemistry (3rd ed.). New Jersey: Wiley.

EOWPVT ▶ Expressive One-Word Picture Vocabulary Test

Ependymoma M I -Y EOUNG J O Private Practice Los Angeles, CA, USA

Definition Ependymomas are tumors that arise from the ependymal cells that line the ventricular system in the brain or spinal cord. There are four main types, classified according to tumor grade: myxopapillary ependymomas and subependymomas (grade I), ependymomas (grade II), and anaplastic ependymomas (grade III). Ependymomas are one of the most common types of tumors seen in children, especially those aged 3 years and younger. In children, ependymomas are usually located intracranially, primarily in the posterior fossa, while in adults, they are usually located spinally. Ependymomas are typically slow growing and the main symptoms are due primarily to raised intracranial pressure. A shunt may be necessary. Depending on the location, symptoms can include nausea, headaches, vomiting, papilledema, paresthesias, ataxia, spinal pain, and changes in mood and personality. Supratentorial ependymomas are often accompanied by hemiparesis, sensory disturbances, aphasia, and other types of cognitive impairment. Seizures and focal neurological deficits can also occur. Surgical intervention is usually first-line intervention, followed by radiotherapy. In young children, chemotherapy is the treatment of choice over

Ependymoma. Figure 1 Courtesy Michael Fisher, MD, Peter C. Phillips, MD. The Children’s Hospital of Philadelphia

radiotherapy because of the possible long-term effects of radiotherapy.

Cross References ▶ Brain Tumor ▶ Edema ▶ Neoplasms ▶ Posterior Fossa ▶ Radiotherapy ▶ Tumor Grade

References and Readings Applegate, G. L., & Marymont, M. H. (1998). Intracranial Ependymomas: a review. Cancer Investigations, 16(8), 588–593. Moynihan, T. J. (2003). Ependymal tumors. Current Treatment Options in Oncology, 4(6), 517–523.

EPI ▶ Eysenck Personality Inventory

Epidural Hematoma

Epicritic Pain K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Definition A variant of nocioceptive pain (pain resulting from ongoing activation of primary afferent neurons by noxious stimuli), epicritic pain is transmitted to the spinal cord by A Delta (d) fibers. These fibers are sparsely myelinated, large-diameter, and fast-conducting, which transmit sharp, well-localized pain. A d fibers are mostly sensitive to mechanical and thermal stimuli. Epicritic pain is less responsive to opioid therapy than is protopathic pain.

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Cross References ▶ TBI

References and Readings Centers for Disease Control and Prevention. (2007). What is epidemiology?. Retrieved December 26, 2007, from http://www.cdc.gov/malaria/ distribution_epi/epidemiology.htm. Ficker-Terrill, C., Flippo, K., Antoinette, T., & McMorrow, D. B. (2007). Overview of brain injury. In The essential brain injury guide (4th ed., pp. 1–24). McLean, VA: Brain Injury Association of America.

Epidural Hematoma B ETH R USH Mayo Clinic Jacksonville, FL, USA

▶ Protopathic Pain

Synonyms References and Readings EDH McCaffery, M., & Pasero, C. (1999). Pain clinical manual (2nd ed.). St. Louis: Mosby.

Definition

Epidemiology S AMANTHA B ACKHAUS Rehabilitation Hospital of Indiana Indianapolis, IN, USA

Definition Epidemiology is a branch or subspecialty of medical studies and sciences that examines the incidence, causes, distribution, control, and transmission of a disease within a population of interest. It can also examine the genesis and developmental characteristics of a specific disease. For example, in examining the epidemiology of traumatic brain injury (TBI), one would expect to learn more about the incidence, prevalence, annual rates, risk factors (including sex, race, and age), and causes of TBI.

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Epidural hematoma is a traumatic accumulation of blood that forms following blunt force on the head (i.e., baseball bat, hammer). The force of impact is strong enough to tear the dural cover of the brain from the skull. This tearing of the dural cover disrupts the nearby arteries and veins, as well as dural branches of these blood vessels. One of the most common sites for epidural hematoma is the tempoparietal area due to skull fracture disrupting the middle meningeal artery or its dural branches. Motor vehicle accidents and physical assaults are common causes of epidural hematoma. Frequently neurosurgical intervention is required on an emergent basis to prevent an increase in intracranial pressure from rapidly accumulating blood. If intracranial pressure elevates too quickly or too high, the patient can become comatose. Most commonly, craniotomy with evacuation is used to intervene in cases of epidural hematoma. On some occasions, an epidural drain is placed to assist in the funneling off of extra fluid from the surface of the brain.

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Cross References ▶ Cerebral Hemorrhage ▶ Skull Fracture

References and Readings Ammerman, J. M., Jarrell, S. T., & Polin, R. S. (2006). Traumatic intracranial hemorrhage. In R. W. Evans (Eds.), Neurology and trauma (2nd ed., pp. 156–166). New York: Oxford University Press.

Epilepsy V ICTORIA M. L EAVITT 1, K ENNETH R. P ERRINE 2,3 1 Kessler Foundation Research Center West Orange, NJ, USA 2 Northeast Regional Epilepsy Group Hackensack, NJ, USA 3 Weill-Cornell College of Medicine New York, NJ, USA

Synonyms Cerebral seizures; Convulsive disorder; Seizure disorder

Short Description or Definition Epilepsy is a common neurological disorder characterized by recurrent seizures of cerebral origin. Neither a specific disease nor a single syndrome, epilepsy encompasses a variety of symptom complexes arising from multifarious brain dysfunctions from various pathologic processes that produce seizures from a flurry of abnormal electrical activity in the brain. The hallmark of epilepsy is two or more unprovoked seizures (excluding e.g., seizures secondary to alcohol withdrawal) occurring more than 24 h apart. Seizures may be accompanied by a wide range of behavioral disturbances, and may or may not entail a loss of consciousness. They sometimes involve an ‘‘aura’’ (see entry for aura), a subjective sensation signaling the onset of a seizure that may include sensory experiences such as smells and visual distortions. The term aura, which is actually a delimited simple partial seizure, is attributed to the Roman physician Galen (130–200 AD) who reportedly overheard a boy say just before a seizure that he felt a cool breeze, ‘‘aura’’ in Latin (Bennett, 1992). There is a great deal of heterogeneity in epilepsy’s presentation, any accompanying auras,

behavioral components, seizure control, the degree to which the affected individual’s quality of life is impacted, and the underlying etiology. Some seizures are not the result of epileptic discharges generated in the brain and are termed NES for non-epileptic seizures (the term ‘‘pseudoseizures’’ is generally avoided because of the pejorative tone and lack of precision). NES can arise from medical disorders such as syncope, migraine, stroke/TIA, movement disorders, sleep disorders, or metabolic disturbances such as diabetes. NES can also have psychological origins, including conversion disorder, somatization disorder, or Munchausen syndrome. Some patients may have episodes of both NES and epileptic seizures, which can greatly complicate accurate diagnosis. Finally, some patients feign seizures for primary gain, usually within the context of personal injury litigation or seeking financial support such as long-term disability.

Categorization Seizures are classified according to the extent of presumed underlying neural involvement. Broadly speaking, they are characterized as generalized (involving the whole brain) or partial (starting in a specific area, or seizure focus; for more on this see Seizure entry). Electroencephalographic (EEG) monitoring through the use of scalp electrodes and the clinical history are generally employed for diagnosis. However, positive EEG findings do not occur in all patients with epilepsy, and having a negative EEG does not rule out epilepsy. EEG can be helpful although not necessarily definitive as a means of determining whether seizures originate across the whole brain or from a localized seizure focus. EEG localization is especially important in treatment planning for surgical intervention. Epilepsy can also be classified as one of three etiologies describing the presumptive cause: idiopathic, symptomatic, and cryptogenic. Whereas symptomatic and cryptogenic epilepsies are caused by a lesion (not readily apparent in cryptogenic types), in idiopathic epilepsy, the underlying pathology is unknown. In addition to being characterized by different types or etiologies of seizures, some cases of epilepsy are associated with specific syndromes. These syndromes may become evident in early childhood, such as Lennox–Gastaut, syndrome, West syndrome, benign Rolandic epilepsy, and Landau–Kleffner syndrome. Others include Rasmussen’s encephalitis juvenile myoclonic epilepsy, temporal lobe epilepsy (TLE), frontal lobe epilepsy, and neurocutaneous disorders including Type 1 neurofibromitosis, Sturge–Weber syndrome, and

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tuberous sclerosis. Chronic TLE arising from mesial temporal sclerosis may also be regarded as a syndrome. Finally, some classification schemes combine various aspects of seizure type, semiology, etiology, and associated syndrome features (Engel, 2006; Loddenkemper et al., 2005).

Epidemiology Present estimates of the prevalence of epilepsy range from 0.5 to 1% of the population; these estimates are generally consistent across geographic regions and ethnicity. Seizure onset most commonly occurs during childhood with a second smaller peak in senescence. Almost any type of brain pathology can cause seizures, including cerebrovascular disease, prenatal or perinatal insults, head injury, tumor, CNS infections, meningitis, encephalitis, and developmental migrational or dysplastic defects (e.g., heterotopias). Comorbidities include psychiatric disorders, which are present in an estimated 25–50% of individuals with epilepsy, with higher prevalence among patients with poorly controlled seizures. These include depression, anxiety, psychotic disorders, as well as cognitive and personality changes occurring in the interictal or ictal/post-ictal states. Furthermore, research in epilepsy and depression suggests a possible shared pathogenic mechanism between seizures and mood disorders (Jests & Friedman, 2006). In addition, behavioral disorders may arise in response to neurobiological effects, treatment effects, and psychosocial effects; these can include general psychopathology, personality changes, aggression, sexual dysfunction, affective disorders, and psychosis.

Natural History, Prognostic Factors, Outcomes The natural history of epilepsy varies widely, based on age of onset, seizure type, frequency, etiology, and associated syndrome features. Generalized tonic–clonic seizures (GTCs) are usually well controlled with medication unless they are part of the more complex epilepsy syndromes. Complex partial seizures tend to be more uncontrolled; typically, patients suffering from these disorders undergo multiple trials of AEDs to find a combination that will prevent breakthrough seizures. Prognostic factors are complicated by the heterogeneity of the syndrome. While approximately 30% of individuals with epilepsy have seizures that are well controlled through antiepileptic medications (AEDs), other individuals have chronic unremitting epilepsy that is treatment refractory, and still others have chronic epilepsy that

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shows only partial response to AEDs. It is also important to consider the differences in AED responsiveness that are seen in generalized versus partial cases, as well as the epilepsy syndromes, which tend to be very poorly controlled by AEDs. Idiopathic GTCs and absence seizures generally tend to show the best response to medication. If medication trials fail patients may proceed to epilepsy surgery; more than 80% of patients with complex partial seizures of mesial temporal lobe origin achieve postsurgical seizure freedom. Less success is seen when surgery is in neocortical or extra-temporal regions. Presurgical neuropsychological testing combined with demographic variables and other neurodiagnostic findings have been shown to be helpful in determining which patients are at greatest risk for postoperative decline (Bennett, 1992). Across all types of epilepsies and treatments, greater seizure frequency with poorer control is associated with the most deleterious impact on cognitive abilities and quality of life. Multiple episodes of status epilepticus (a flurry of continuous seizures or many seizures with little seizurefree time between episodes) also produce significant declines in cognition, mood, and quality of life. Sudden unexplained death in epilepsy (SUDEP) occurs in a very small minority of patients (approximately 1 in 1,000 individuals with epilepsy per year). There are no known precursors, and while it is generally associated with GTCs, it is otherwise poorly understood. Although research suggests that epilepsy involves an increased mortality risk, the risk is predominantly present at a younger age and early after diagnosis, and the absolute risk is moderate.

Neuropsychology and Psychology of Epilepsy Cognitive impairments are sometimes seen in individuals with epilepsy, and are related to seizure type, seizure frequency and severity, age of seizure onset, medication variables, and the underlying pathology. Generalized epilepsies cause more cognitive impairment if they are not well controlled; frequency and other factors contribute to the severity of impairment. Generalized syndromes are associated with the most severe cognitive difficulties. Idiopathic generalized epilepsy is usually well controlled and does not tend to have as many cognitive implications. In cases of partial epilepsy, if seizures are well controlled minimal impact on cognitive functioning is generally seen. However, when seizures are not well controlled, there are more localization factors; in such cases, a degree of concordance tends to be seen between cognitive deficits and site of seizure generation. However, individuals with severe and

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frequent refractory partial epilepsy may fail to show an expected lateralization pattern. TLE accounts for 70% of chronic symptomatic partial cases; therefore, the cognitive deficits related to TLE are common. In cases involving a left-sided seizure focus, these deficits may include verbal learning and memory deficits including consolidation, retrieval, and delayed recall, as well as naming deficits (i.e., semantic memory). With right TLE, deficits may involve nonverbal memory deficits, although traditional measures for these deficits tend to be relatively insensitive to the effects of right temporal lobe dysfunction (for more on this, see Barr, 1997). Frontal deficits can also be seen in TLE, as involvement of areas distal to the seizure onset zone is not uncommon; these may include deficits of executive functions such as attention, working memory, mental flexibility, response inhibition, and planning. AEDs also contribute to cognitive deficits in epilepsy (see Meador, 2006). The cognitive deficits most commonly associated with AEDs are attention, concentration, and psychomotor speed. However, the presence and extent of cognitive effects of AEDs on cognition can vary by whether older or newer generation medications are being taken. Generally, the barbiturates have the most deleterious effects on cognitive abilities. Fewer but sometimes still significant cognitive side effects can be seen in other older generation drugs such as phenytoin, carbamazepine, and valproate. Newer generation drugs approved in the 1990s and beyond tend to have a less severe impact on cognition. Polypharmacy is often associated with greater cognitive impairment. However, effective seizure control is usually more contributory to cognitive status than the specific AED(s) being taken.

Evaluation The neuropsychological evaluation can be important for several reasons. First, it allows for an understanding of the extent of cognitive impairment in individuals with epilepsy as well as aides in characterizing psychosocial and quality of life issues that may be amenable to intervention. Secondly, it can be an important means of providing corroborating evidence for localizing the epileptogenic zone, which is particularly critical for presurgical evaluations. This information can help with presurgical treatment planning and prediction of postsurgical sequelae, including appropriateness of cognitive remediation. There are specialized procedures in which neuropsychologists participate as part of neurosurgical interventions for epilepsy. Prior to surgery, many centers conduct the

Wada test to identify the laterality of language or to identify language dominance and to identify the memory capability of each hemisphere separately. The test, named after Dr. Juhn Wada who developed the procedure at the Montreal Neurological Institute, involves anesthetizing one hemisphere at a time and then conducting brief language and memory testing of the intact hemisphere. This is accomplished by injection of sodium amobarbital into the internal carotid artery as part of an angiogram. The Wada test can also help predict possible postoperative memory declines and assist in corroborating seizure localization by examining the disparity of memory scores between the two hemispheres. Although functional MRI is being used by many centers for language lateralization, it has not yet supplanted the Wada test and is less effective in assessing memory. Neuropsychologists also frequently assist in cortical mapping with electrical stimulation of the brain to more precisely localize language, other cognitive abilities, and sensorimotor areas. This mapping is conducted during stimulation through chronically implanted (days to weeks) subdural grids and strips involving two craniotomies, or during intraoperative stimulation with the patient awake after the craniotomy opening in a one-stage procedure.

Treatment Antiepileptic drugs (AEDs), also known as anticonvulsants (a misnomer because not all seizures are convulsive), are a common treatment. The primary mechanism of action of these drugs varies, but includes sodium channels, calcium channels, GABA, glutamate, calcium inhibitors, and hormones. The side effects of AEDs vary considerably depending on the specific medication. They can include diplopia, cognitive effects, somnolence, weight gain/loss, gingival hyperplasia, osteoporosis, gastrointestinal complaints, headache, and rash. In addition to AEDs, other interventions include hormonal therapy and a ketogenic diet. Surgical interventions include resective surgery, corpus collosotomy, hemispherectomy, and implantation of stimulating devices, e.g., vagus nerve stimulator implantation. Alternative techniques such as yoga and neurofeedback are controversial and have not been subject to controlled clinical trials.

Cross References ▶ Absence Seizure ▶ Anticonvulsants ▶ Aura

Epinephrine

▶ Clonazepam ▶ Generalized Seizure ▶ Grand Mal Seizure ▶ Juvenile Myoclonic Epilepsy ▶ Myoclonic Epilepsy of Infancy ▶ Nonepileptic Seizures ▶ Partial Seizure ▶ Phenobarbital ▶ Seizure ▶ Wada Test

References and Readings Allen Hauser, W. (1990). Epilepsy: Frequency, causes, and consequences. New York: Demos Publications. Barr, W. B. (1997). Examining the right temporal lobe’s role in nonverbal memory. Brain and Cognition, 35(1), 26–41. Bennett, T. L. (Ed.). (1992). The neuropsychology of epilepsy. New York: Plenum Press. Engle, J., Jr. (2006). Report of the ILAE classification core group. Epilepsia, 47(9), 1558–1568. Engle, J., Pedley, T. A. (Eds.). (2008). Epilepsy: A comprehensive textbook (2nd ed.). Philadelphia: Lippincott Williams & Wilkins. Jests, D. V., & Friedman, J. H. (2006). Psychiatry for neurologists. Totowa, NJ: Humana Press. Loddenkemper, T., Kellinghaus, C., Wyllie, E., Najm, I. M., Gupta, A., Rosenow, F., et al. (2005). A proposal for a five-dimensional patient-oriented epilepsy classification. Epileptic Disorders, 7 (4), 308–316. Meador, K. J. (2006). Cognitive and memory effects of the new antiepileptic drugs. Epilepsy Research, 68(1), 63–67.

Epinephrine M ARLA S ANZONE Independent Practice Annapolis, MD, USA

Synonyms Adrenalin; Adrenaline

Indications Epinephrine (E), also called adrenalin, is a sympathomimetic monoamine neurotransmitter that acts as a hormone. It is a catecholamine, derived from the amino acids phenylalanine and tyrosine, and released from the adrenal medulla. In 1895, the Polish physiologist, Napoleon

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Cybulski discovered E in the adrenal gland. In 1901, a Japanese chemist, Jokichi Takamine isolated the same hormone from cow glands. And in 1904, Friedrich Stolz first artificially synthesized E. E shares common pathways with catecholamines, dopamine, and norepinephrine. During times of stress, the splanchnic nerves in the adrenal medulla stimulate the sympathetic nervous system (SNS) to release E and adrenocorticotropic hormone (ACTH). ACTH activates the adrenal cortex to produce the stress-reactive hormone, cortisol. This process interacts with the synthesis of E. Tyrosine hydroxylase converts tyrosine to L-dopa. L-dopa synthesizes dopamine via dopa decarboxylase, and dopamine b-hydroxylase converts dopamine into norepinephrine. Norepinephrine then synthesizes E. Specialized neurons secrete E and norepinephrine when modified preganglionic sympathetic fibers synapse onto neuroendocrine cells. Sympathomimetic or adrenergic drugs bind to E receptors, also called adrenoceptors.

Mechanisms of Action When E is secreted into the bloodstream, it rapidly activates the sympathetic nervous system to prepare the body for emergencies. Although E is not itself considered psychoactive, SNS arousal also releases norepinephrine which is the precursor to E, is psychoactive, and has similar actions in the body. In the liver, E binds to both a and b receptors. The effects of E are mediated as a nonselective agonist through sympathetic adrenoceptors, a1, a2, b1, and b2. When bound to a1 receptors, E activates the inositol-phospholipid signaling pathway. Glycogen synthase is then inactivated and phosphorylation activates phosphorylase kinase. This triggers the enzyme, glycogen phosphorylase to stimulate glycogenolysis. In the liver and muscle cells, E catalyzes the adenylate cyclase signaling pathway to activate glycogenolysis via b-adrenergic receptors. Glycogenolysis results in the release of glucose into the bloodstream via the breakdown of glycogen. Blood glucose levels increase as a function of glycogen catabolism in the liver. Lipolysis in fat cells is activated and glucose supplies to the brain increase. This contributes to heightened mental alertness. When E is released, it acts as a hormone, increasing SNS activity. SNS activation leads to an elevated metabolic rate, respiration, and heart rate, increased stroke volume and blood pressure, dilation of respiratory passageways and pupilary muscles, and activation of sweat glands. E also causes arterioles in skeletal muscle to dilate which increases energy, but constricts arterioles in the gastrointestinal tract and the skin

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which decreases digestion and elimination. Prolonged sympathetic activation and the associated release of stress hormones E and cortisol can lead to immune suppression (Epinephrine, 2009b).

Specific Compounds and Properties E is an organic, endogenous central nervous system sympathomimetic amine and derivative of the biologically active group, the catecholamines. It has an average molecular weight of 182.2044 and its chemical formula is C9H13NO3 (Metabolomics Toolbox, 2009).

Clinical Use (Including Side Effects) Therapeutically, E and norepinephrine are central to many actions of sympathomimetic agents such as alpha (a)- and beta (b)-adrenergic agonists, MAOI, and COMT Inhibitors. E activation in various preparations is used as a cardiac stimulant to treat cardiac arrest, to relax bronchial tissue in asthmatics and anaphylaxis, as a vasoconstrictor in cases of hemorrhage, or to reduce the rate of absorption of anaesthetic agents. It is also used to treat sepsis and prepared as a borate salt for ophthalmological use. Monoamine oxidase inhibitors (MAOIs) and catechol-O-methyl transferase (COMT) inhibitors act as indirect-acting adrenergic sympathomimetics. Monoamine oxidase (MAO) is the enzyme primarily responsible for the metabolism of E and norepinephrine. MAOIs stimulate the production and release of catecholamines in the CNS, and inhibit the normal process of monoamine oxidase degrading and removing E, norepinephrine, and dopamine from the presynaptic terminal. Preventing the metabolism of these catecholamines increases their concentration in the CNS (Hoffman, 2004; Katzung, 2004). MAOIs such as tranylcypromine and phenelzine are used to treat resistant depressions and panic disorder. By negative feedback at presynaptic a2- or b-adrenergic receptors, chronic use of MAOIs has been shown to lead to down-regulation of the synthesis of E and NE. Other side effects include sedation, insomnia, anxiety, agitation, dizziness, constipation, diarrhea, nausea, orthostatic hypotension, syncope, sexual dysfunction, appetite changes, and in rare instances hypertensive crisis, mania, seizures, and hepatotoxicity. The severe symptoms are of concern if tyramine-containing foods or drugs are ingested.

COMT Inhibitors inhibit the actions of the enzyme, catechol-O-methyl transferase (COMT). As is the function of normal MAO in the presynatic terminal, COMT degrades E and NE postsynaptically. COMT inhibitors interfere with this degradation and transformation process which enables these catecholamines to remain active. Examples of COMT inhibitors include entacapone and tolcapone used adjunctively with levodopa to treat Parkinson’s disease. Side effects include dyskinesias, diarrhea, nausea, abdominal pain, red-brown urine, and hepatotoxicity. Mixed a/b-agonists relax smooth muscle in the lungs and uterus, and in skeletal muscle vasculature. Their actions increase cardiac output and dilate bronchials and pupils. The normal action of b receptors involves E-induced stimulation. b1-adrenergic receptors are located mainly in the heart and kidneys. Stimulation increases cardiac conduction automaticity and velocity, and causes the kidneys to release renin. b2-adrenergic receptors are located primarily in the lungs, gastrointestinal tract, liver, uterus, vascular smooth, and skeletal muscle. Stimulation of b2 receptors induces glycogenolysis in the liver, and relaxes skeletal and smooth muscle in the lungs. b3 receptors are primarily in fat cells and when stimulated activate lipolysis. E, arbutamine, mabuterol, mephentermine, phenylpropanolamine, and synephrine are examples of mixed a/b-agonists prescribed for anaphylaxis, acute asthmatic reactions, cardiac arrest, and glaucoma. Side effects include dry mouth, anxiety, nervousness, insomnia, nausea, vomiting, elevated heart rate, hypertension, and hives. Nonselective and b1-selective adrenergic antagonists or blockers are used therapeutically to inhibit symptoms of physiologic hyperarousal known as ‘‘the fight/ flight response.’’ b-antagonists have intrinsic sympathomimetic actions. As anxiolytics, their effects are a function of blocking actions of E and norepinephrine, decreasing sympathetic over-activation. The sympathetic antagonism of b-blockers, such as atenolol, metoprolol, and nebivolol, appears to be the primary therapeutic mechanism enabling diminished performance and social anxiety. They are also used to treat angina, aortic aneurysm, arrhythmias, essential tremor, hyperhidrosis, hypertension, hyperthyroidism, mitral valve prolapse, and ventricular tachycardia. Nonselective or mixed a/b-antagonist such as celiprolol, inderolol, mepindolol, nadolol, sotalol, and timolol can also be used to reduce intraocular pressure in glaucoma treatment. Side effects can include abnormal vision, bradycardia, bronchospasm, cold extremities, depression, diarrhea, dizziness, edema, fatigue, hallucinations, headaches, heart block, heart failure, hypotension, insomnia, muscle cramps,

Episodic Memory

nausea, sexual dysfunction, sleep disturbances, and problematic glucose/lipid metabolism.

Cross References ▶ Autonomic Nervous System ▶ Catecholamine ▶ Dopamine ▶ Norepinephrine

References and Readings Epinephrine. (2008). In Merriam-Webster Online Dictionary. Retrieved January 13, 2009, from http://www.merriam-webster.com/dictio nary/Epinephrine. Epinephrine. (2009a). In MedicineNet. Retrieved January 13, 2009, from http://www.medterms.com. Epinephrine. (2009b). In Wikipedia. Retrieved January 13, 2009, from http://en.wikipedia.org/wiki/epinephrine. Hoffman, B. B. (2004). Adrenoceptor-activating & other sympathomimetic drugs. In B. Katzung (Ed.), Basic and clinical pharmacology (9th ed., pp. 122–141). New York: Lange Medical Books/ McGraw-Hill. Katzung, B. G. (2004). Introduction to autonomic pharmacology. In B. Katzung (Ed.), Basic and clinical pharmacology (9th ed., pp. 75–93). New York: Lange Medical Books/McGraw-Hill. Lu, D. (2009). Sites of drugs acting at the adrenergic synapses. In PHAR 402. Retrieved January 21, 2009, from http://www.uic.edu/classes/ phar/phar402. Metabolomics Toolbox. (2009). In Human Metabolome Database. Retrieved January 19, 2009, from http://hmdb.ca/scripts/show_card. cgi?METABOCARD = HMDB00216.txt. Nicol, R. A. (2004). Introduction to the pharmacology of CNS drugs. In B. Katzung (Ed.), Basic and clinical pharmacology (9th ed., pp. 336–350). New York: Lange Medical Books/ McGraw-Hill.

Episodic Memory J ONATHAN A. O LER University of Wisconsin Madison, WI, USA

Synonyms Autobiographical memory; Declarative memory; Explicit memory; Relational memory

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Definition Episodic memory is a type of associative learning where relationships between stimuli (e.g., people, places and things) form an autobiographical register of events as they occur in time. Episodic memory is the kind of memory that allows us to recollect our own past and the experiences we have lived through. Episodic memory formation is one of the primary functions of the medial temporal lobe, which is why episodic memory is often referred to as a hippocampal-dependent memory function. Damage to the hippocampus, or the cerebral cortex surrounding it, results in a loss of episodic memory known as amnesia. Episodic memory should not be confused with semantic memory, which refers to the understanding of meanings and the recollection of factual information about the world. Semantic memory is context-independent knowledge, and together with episodic memory make up the two types of declarative or explicit memory, so named because memories of this type can be brought to the forefront of consciousness and verbalized.

Current Knowledge In 1972, a Canadian psychologist Endel Tulving proposed that episodic memory is different from other kinds of memory (Tulving, 1983; 2002). According to Tulving, two closely related features that distinguish episodic from other forms of memory are autonoetic consciousness and chronesthesia. Autonoetic consciousness refers to mental ‘‘time-travel’’, or the cognitive reenactment of previous events, hypothesized to give rise to the subjective experience of remembering. Chronesthesia refers to the subjective experience of time, as in the perception of past, present, and future. Episodic memory encoding is an automatic process and occurs without effort. It is single-trial learning. A brief experience that occurred decades earlier can be involuntarily recalled if the appropriate eliciting stimuli are encountered. During memory encoding, attention plays an important role in the organization of the information being encoded by the hippocampal system, and emotion can have a profound effect on the memory encoding. The emotional modulation of memory is thought to involve interactions between the hippocampal memory system and inputs from the amygdala. For example, post-traumatic stress disorder (PTSD) is a condition of uncontrollable automatic memory recall, usually of a

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very frightening or threatening event. Although other memory mechanisms (e.g., Pavlovian conditioning) are likely to also be involved, PTSD is an example of how emotional arousal and attention can influence episodic memory function. Sensory stimuli are processed in multimodal association regions of the parietal, temporal, and occipital cortices, brain areas thought to give rise to perceptual experience. These multisensory channels are ‘‘funneled’’ into the medial temporal lobe along separate multi-synaptic pathways, and are passed into the associative networks of the hippocampal system. The hippopampal system gives rise to projections that return to the same multimodal association regions outside the medial temporal lobe. The organization of this circuitry – funneling sensory information into the hippocampus, and hippocampal projections back to the sensory regions giving rise to the initial input – is thought to allow for the consolidation of episodic memory. The term ‘‘consolidation’’ refers to the time-dependent process of long-term memory storage. Our understanding of memory consolidation comes from observation of patients with retrograde amnesia (the inability to recall things that happened in the time prior to the medial temporal lobe damage). Patients with amnesia resulting from medial temporal lobe damage are typically still able to recall facts and remote memories from the years before the injury. The ‘‘temporally graded’’ nature of retrograde amnesia suggests that memory consolidation is a protracted process that can take years, and that eventually the retrieval of that memory is no longer dependent on the medial temporal lobe. However, the long-standing hypothesis that long-term memory eventually becomes entirely independent of the medial temporal lobe has recently been challenged (Nadel & Moscovitch, 2001). Brain imaging studies of normal subjects provides evidence that the right prefrontal cortex is activated during the retrieval of episodic memories (Buckner, Raichle, Miezin, & Petersen, 1996; Nyberg, 1998). The frontal lobes are thought to be critical for associating the content of an event (semantic knowledge) with its source episodic memory (i.e., when and where the event occurred). Although episodic memory and semantic memory are both affected by medial temporal lobe damage, episodic memory and semantic memory may be dissociable in amnesic patients with severe frontal lobe damage (Squire & Zola, 1998). For example, patient K.C., who has been studied for many years by Tulving and his colleagues, has no episodic memory but does show spared semantic memory (Rosenbaum et al., 2005).

Future Directions In 1998, Clayton and Dickinson demonstrated that animals might possess episodic memory. They showed that Western Scrub-Jays (Aphelocoma californica) can remember where they cache different types of food, and depending on the perishability of the item and the amount of time elapsed since caching, could discriminate among them. Clayton and Dickinson argued that while they could not demonstrate autonoetic consciousness, the ‘‘what-where-and-when’’ components of the Scrub-Jays’ cashing behavior was evidence that the birds possessed episodic-like memory. Whether episodic memory is a uniquely human capacity remains a question of scientific debate. Hyperthymestic syndrome is a term proposed by Parker and her colleagues for a clinical case of superior autobiographical memory (Parker, Cahill, & McGaugh, 2006). The woman, known as ‘‘A.J.,’’ spends an abnormally large amount of time thinking about her personal past, and has the extraordinary capacity to recall specific events from it. Since the first report of hyperthymesia was published, several other cases have been reported, and as more of these cases of extraordinary autobiographical memory surface over time, researches may be able to utilize brain imaging techniques to reveal the mechanisms underlying both superior, as well as normal, episodic memory.

Cross References ▶ Hippocampus ▶ Medial Temporal Lobe ▶ Paired-Associate Learning

References and Readings Buckner, R. L., Raichle, M. C., Miezin, F. M., & Petersen S. E. (1996). Functional anatomic studies of memory retrieval for auditory words and visual pictures. The Journal of Neuroscience, 16(19), 6219–6235. Clayton, N. S., Griffiths, D. P., Emery, N. J., & Dickinson, A. (2001). Elements of episodic-like memory in animals. Philosophical Transactions of the Royal Society of London – Series B: Biological Sciences, 356 (1413), 1483–1491. Nadel, L., & Moscovitch, M. (2001). The hippocampal complex and longterm memory revisited. Trends in Cognitive Sciences, 5(6), 228–230. Nyberg, L. (1998). Mapping episodic memory. Behavioural Brain Research, 90(2), 107–114. Parker, E. S., Cahill, L., & McGaugh, J. L. (2006). A case of unusual autobiographical remembering. Neurocase, 12(1), 35–49. Rosenbaum, R. S., Kohler, S., Schacter, D. L., Moscovitch, M., Westmacott, R., Black, E. S., et al. (2005). The case of K.C.: Contributions

Equipotentiality of a memory-impaired person to memory theory. Neuropsychologia, 43(7), 989–1021. Squire, L. R., & Zola, S. M. (1998). Episodic memory, semantic memory, and amnesia. Hippocampus, 8(3), 205–211. Tulving, E. (1983). Elements of episodic memory. London: Oxford University Press. Tulving, E. (2002). Episodic memory: From mind to brain. Annual Review of Psychology, 53, 1–25.

Epithalamus-Pineal Gland, Habenular Nuclei ▶ Diencephalon

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glands, and the age of the patient. Usually, laboratory tests are needed for confirmation. Serologic results for persons with infectious mononucleosis include an elevated white blood cell count, an increased percentage of certain atypical white blood cells, and a positive reaction to a ‘‘mono spot’’ test. There is no specific treatment for infectious mononucleosis other than treating the symptoms. Symptoms related to infectious mononucleosis caused by EBV infection seldom last for more than 4 months. When the illness lasts for more than 6 months, it is called chronic EBV infection. However, valid laboratory evidence for continued active EBV infection is seldom found in these patients. Chronic symptoms should be investigated further to determine if they meet the criteria for chronic fatigue syndrome (CFS). This process includes ruling out other causes of chronic illness or fatigue.

EPS ▶ Extrapyramidal Symptoms

Cross References ▶ Chronic Fatigue Syndrome

Epstein–Barr Virus S USAN K. J OHNSON University of North Carolina at Charlotte Charlotte, NC, USA

References and Readings National Center for Infectious Diseases Center for Disease Control and Prevention webpage at www.cdc.gov/ncidod/diseases/ebv.htm, accessed July 17, 2008.

Definition

EQ-5D Epstein–Barr virus, frequently referred to as EBV, is a member of the herpesvirus family and one of the most common human viruses. The virus occurs worldwide, and most people become infected with EBV sometime during their lives. In the United States, as many as 95% of adults have been infected. Infection with EBV often results in mononucleosis. Symptoms of infectious mononucleosis are fever, sore throat, and swollen lymph glands. Although the symptoms of infectious mononucleosis usually resolve quickly, EBV remains dormant or latent in blood cells for lifetime.

▶ EuroQol/EQ-5D

Equipotentiality J ENNIFER S. K LEINER University of Arkansas for Medical Sciences Little Rock, AR, USA

Definition Evaluation and Treatment The clinical diagnosis of infectious mononucleosis is based on symptoms of fever, sore throat, swollen lymph

Equipotentiality – a notion developed by Karl Spencer Lashley (1890–1958) positing that all areas of the brain are equally able to perform a task. This contrasts with the theory of localization, according to which neurocognitive

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functions are specifically referable to discrete areas of the brain; hence, damage to restricted regions would be expected to produce selective cognitive deficits. Equipotentiality theory, however, hypothesized that the severity of cognitive dysfunction was directly related to the total amount of tissue damage. For example, memory functioning was thought to be diffusely distributed throughout the cortex rather than related to defined circuits or pathways. Under this theory, intact areas of the cortex could assume responsibility for discrete cognitive functions following injury. The theory did allow, however, for localization related to sensory and motor processes. The related concept of ‘‘mass action’’ posited that cognitive functions are equally and widely distributed across brain areas and that the entire cortex participates in cognitive functioning.

Error Handling ▶ Error Recognition and Correction

Error Recognition and Correction K RISTEN DAMS -O’C ONNOR Mount Sinai School of Medicine New York, NY, USA

Synonyms Error evaluation and error utilization; Error handling

Cross References ▶ Karl Lashley

References and Readings Lashley, K. S. (1929). Brain mechanisms and intelligence. Chicago: University of Chicago Press. Lashley, K. S. (1930). Basic neural mechanisms in behavior. Psychological Review, 37, 1–24.

Equivalent Forms ▶ Alternate Test Forms

Erethism ▶ Mercury Exposure

ERP’s ▶ Event-Related Paradigms

Error Evaluation and Error Utilization ▶ Error Recognition and Correction

Definition Error recognition refers to the ability to recognize or detect the presence of an error; recognition may happen as the error is being made or after it has occurred. Error correction is the ability to use knowledge about the presence of an error to remedy or correct it, allowing for an error-free outcome. Error recognition and correction are distinct cognitive processes that facilitate adaptive functional living.

Current Knowledge Human error has been a topic of interest in both clinical and applied psychology for decades, appearing in research on industrial safety, driving accidents, and computer science. More recently, investigation into the process of error recognition and correction among individuals with impaired cognitive functioning has expanded our understanding of the cognitive skills and brain regions involved. Early case studies described patients with frontal lobe damage the ability of which to identify and remedy their own errors was disturbed. Although the terms ‘‘error evaluation’’ and ‘‘error utilization’’ were used synonymously in some cases (e.g., Luria, Pribram, & Homskaya, 1964), subsequent reports of patients who were unable to correct their errors despite having intact error recognition have indicated that these abilities are distinct cognitive processes. Moreover, error recognition is considered a necessary prerequisite for error correction, which suggests that increasing error recognition may be a useful focus for intervention among individuals with cognitive impairment.

Error Recognition and Correction

Previous literature demonstrates that individuals with cognitive impairments secondary to traumatic brain injury recognize and correct significantly fewer errors during everyday tasks as compared to healthy controls (e.g., Hart, Giovannetti, Montgomery, & Schwartz, 1998). Similar findings have been documented among individuals with dementia (Bettcher, Giovannetti, Macmullen, & Libon, 2008; Giovannetti, Libon, & Hart, 2002). Errors occur in both the error recognition and correction stages of the process, depending on the cognitive functions and/or brain regions that have been impacted. Several domains of cognitive functioning are recruited during the process of recognizing and correcting errors, from simple attention, visual perception, and processing speed to higher-order executive functions including mental flexibility, problem-solving, and self-monitoring. Awareness, or the ability to be perceptive and insightful of one’s own condition, actions, or feelings, is particularly important for successful error handling (Sohlberg & Mateer, 2001). Multiple models of awareness of cognitive deficits exist, and such models provide a useful framework for conceptualizing the process of error recognition and correction. Crosson et al. (1989) suggest a pyramid model of awareness of which intellectual awareness forms the foundation for emergent awareness, each of which is necessary for anticipatory awareness. Intellectual awareness, or the ability to understand that a function is impaired, and emergent awareness, which refers to the ability to recognize a problem while it is happening, each play an important role in error recognition, and consequently error correction. Toglia and Kirk (2000) suggest a more dynamic model of awareness that distinguishes between stored metacognitive knowledge and task-specific awareness. On-line awareness, or the ability to monitor and regulate performance during a given task or situation (Toglia & Kirk), provides a more comprehensive framework for understanding the cognitive processes involved in the processes of error recognition and correction. These authors further argue that the ability to gauge the difficulty level of a task and anticipate pitfalls (emergent awareness) is related to the initiation of self-monitoring and strategy use, which allows for both error recognition and correction. Multiple brain regions are involved in the process of error recognition and correction. The frontal and prefrontal regions of the brain are strongly implicated in executive functioning, both independently and through interconnections with other brain regions. Damage to the frontal lobes can result in impairments in self-monitoring, set-shifting, and problem-solving, and has long been associated with disruption of the error handling process

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(e.g., Luria et al., 1964). Additionally, damage to the medial prefrontal cortex and anterior cingulate cortex (ACC) is associated with disturbed ability to monitor performance and recognize errors (Gehring & Fencsik, 2001; Stemmer et al., 2004), and fMRI studies report activation of the ACC when cognitive errors are made (Carter et al., 1998). Event-related potentials (ERPs) document the brain’s unique response to errors: a negative component of an event-related brain potential called an error-related negativity (ERN) is detected at the onset of an error, and peaks soon thereafter (Mathalon, Whitfield, & Ford, 2003). Finally, correlational analyses of ERP and fMRI data indicate that the rostral ACC is selectively activated during error processing (Mathalon et al.).

Cross References ▶ Anosognosia ▶ Awareness ▶ Cognitive Rehabilitation ▶ Error, Sources of

References and Readings Bettcher, B. M., Giovannetti, T., Macmullen, L., & Libon, D. J. (2008). Error detection and correction patterns in dementia: A breakdown of error monitoring processes and their neuropsychological correlates. Journal of the International Neuropsychological Society, 14, 199–208. Carter, C., Braver, T. S., Barch, D. M., Botvinick, M. M., Noll, D., & Cohen, J. D. (1998). Anterior cingulated cortex, error detection, and the online monitoring of performance. Science, 280, 747–749. Crosson, B., Barco, P. P., Velozo, C. A., Bolesta, M. M., Cooper, P. V., Werts, D., et al. (1989). Awareness and compensation in postacute head injury rehabilitation. Journal of Head Trauma Rehabilitation, 4, 46–54. Giovannetti, T., Libon, D. J., & Hart, T. (2002). Awareness of naturalistic action errors in dementia. Journal of the International Neuropsychological Society, 8, 633–644. Hart, T., Giovannetti, T., Montgomery, M. W., & Schwartz, M. F. (1998). Awareness of errors in naturalistic action after traumatic brain injury. Journal of Head Trauma Rehabilitation, 13, 16–28. Luria, A. R., Pribram, K. M., & Homskaya, E. D. (1964). An experimental analysis of the behavioral disturbance produced by a left frontal arachnoidal endothelioma (meningioma). Neuropsychologia, 2, 257–280. Mathalon, D. H., Whitfield, S. L., & Ford, J. M. (2003). Anatomy of an error: ERP and fMRI. Biological Psychology, 64, 119–141. Sohlberg, M. M., & Mateer, C. A. (2001). Management of dysexecutive symptoms. In M. M. Sohlberg & C. A. Mateer (Eds.), Cognitive rehabilitation: An integrative neuropsychological approach (pp. 230–268). New York: The Guilford Press. Toglia, J., & Kirk, U. (2000). Understanding awareness deficits following brain injury. NeuroRehabilitation, 15, 57–70.

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Error, Sources of

Error, Sources of M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Sources of error are the factors that influence a test score or item performance that are extraneous to the construct of interest. In a memory test, sources of error might include examiner imprecision or subjectivity, fatigue, time of day, or level of hunger. The effects of these sources comprise the error term in classical test theory. Test construction is conducted so as to minimize or otherwise allow an estimate and correction of the error term so as to maximize the extent to which an observed score reflects the true value of the construct of interest.

Cross References ▶ Classical Test Theory ▶ Test Reliability ▶ Test Validity

learning process. The aim of this approach is to prevent the learner from reinforcing errant behavior, which may occur with repeated mistakes. One of the earliest researched errorless learning techniques, stimulus fading, is best highlighted in animal research by Terrace (1963), the first research that demonstrated the benefits of errorless learning. Terrace demonstrated that pigeons were better able to discriminate between green and red lights using stimulus fading. First, Terrace introduced a red light, the correct response. Once the pigeons responded consistently to the red light, a green light (the incorrect response) was introduced gradually to the experiment. The green light was at first briefly presented at a dim intensity, but eventually reached the same intensity and duration as the red light exposure. Eventually, when both red and green lights were being presented simultaneously at the same intensity and for the same duration, the pigeons consistently pecked the red light, and not the green, thus demonstrating the benefit of an errorless learning technique. Other errorless learning techniques include stimulus shaping, response prevention, delayed prompting, superimposition with stimulus fading, and superimposition with stimulus shaping (see Mueller, Palkovic, & Maynard, 2007).

Current Knowledge


Errorless Learning: A Popular Theory

Kline, P. (1986). A handbook of test construction: Introduction to psychometric design. New York: Methuen.

The process of errorless learning has been theorized to be based on Hebbian plasticity. According to Hebb (1961), the synaptic connection between two neurons will be strengthened if they fire together. Learning occurs if this particular pattern of neural activity is activated on subsequent occasions. Thus, the patterned response will occur with greater frequency, whether the response is correct or incorrect. The goal of errorless learning is to increase synaptic firing between neurons while making mostly correct responses.

Errorless Learning A DAM B. WARSHOWSKY Shepherd Center Atlanta, GA, USA

Synonyms Delayed prompting; Response prevention; Stimulus fading

Definition Errorless learning refers to a type of training that reduces the learner’s opportunity to make errors during the

Applications in Current Research Research has shown that errorless learning can be useful for a number of different populations and has been demonstrated to be beneficial in the treatment of a variety of conditions. Errorless learning has been shown to be beneficial in the treatment of memory impairments due to dementia, Alzheimer’s type (Clare, Wilson, Carter, Breen, Gosses, & Hodges, 2000) and Korsakoff ’s Syndrome

Escitalopram

(Komatsu, Mimura, Kato, Wakamatsu, & Kashima, 2000), anomia (Fillingham, Sage, & Ralph, 2006) and aphasia (Fillingham, Hodgson, Sage, & Ralph, 2003) following stroke or traumatic brain injury, remediation of cognitive impairments for patients with schizophrenia (Mulholland, O’Donoghue, Meenagh, & Rushe, 2008), and has been used to enhance skill acquisition among typical school-age children and children with pervasive personality disorders. Current research continues to explore additional applications for errorless learning, such as treatment for brain injured patients with memory impairment and poor executive functioning (Pitel, Beaunieux, Lebaron, Joyeux, Desgranges, & Eustache, 2006), and the relearning of functional skills in patients after acquired brain injury (Martelli, Nicholson, & Zasler, 2008) and following acute stroke events (Mount, Pierce, Parker, DiEgidio, Woessner, & Spiegel, 2007).

Cross References ▶ Hebb, Donald (1904–1985) ▶ Learning ▶ Stimulus Control ▶ Stimulus Generalization


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Mueller, M. M., Palkovic, C. M., & Maynard, C. S. (2007). Errorless learning: Review and practical application for teaching children with Pervasive Developmental Disorders. Psychology in the Schools, 44(7), 691–700. Mulholland, C. C., O’Donoghue, D., Meenagh, C., & Rushe, T. M. (2008). Errorless learning and memory performance in schizophrenia. Psychiatry Research, 159(1–2), 180–188. Pitel, A. L., Beaunieux, H., Lebaron, N., Joyeux, F., Desgranges, B., & Eutasche, F. (2006). Two case studies in the application of errorless learning techniques in memory impaired patients with additional executive deficits. Brain Injury, 20(10), 1099–1110. Terrace, H. S. (1963). Discrimination learning with and without ‘‘errors.’’ Journal of the Experimental Analysis of Behavior, 6(1), 1–27.

Erudition ▶ Learning

Escitalopram J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Escitalopram

Clare, L., Wilson, B. A., Carter, G., Breen, K., Gosses, A., & Hodges, J. R. (2000). Intervening with everyday memory problems in Dementia of Alzheimer Type: An errorless learning approach. Journal of Clinical and Experimental Neuropsychology, 22(1), 132–146. Fillingham, J. K., Hodgson, C., Sage, K., & Lambon Ralph, M. A. (2003). The application of errorless learning to aphasic disorders: A review of theory and practice. Neuropsychological Rehabilitation, 13(3), 337–363. Fillingham, J. K., Sage, K., & Lambon Ralph, M. A. (2006). The treatment of anomia using errorless learning. Neuropsychological Rehabilitation, 16(2), 129–154. Hebb, D. O. (1961). The organization of behavior: A neuropsychological theory. Stimulus and response – and what occurs in the brain in the interval between them. New York: Science Editions, Inc. Komatsu, S., Mimura, M., Kato, M., Wakamatsu, N., & Kashima, H. (2000). Errorless and effortful processes involved in the learning of face-name associations by patients with alcoholic Korsakoff ’s syndrome. Neuropsychological Rehabilitation, 10(2), 113–132. Maritelli, M. F., Nicholson, K., & Zasler, N. D. (2008). Skill reacquisition after acquired brain injury: A holistic habit retraining model of neurorehabilitation. NeuroRehabilitation, 23, 115–126. Mount, J., Pierce, S. R., Parker, J., DiEgidio, R., Woessner, R., & Spiegel, L. (2007). Trial and error versus errorless learning of functional skills in patients with acute stroke. NeuroRehabilitation, 22, 123–132.

Brand Name Lexapro

Class Selective Serotonin Reuptake Inhibitor. Escitalopram is the s-isomer of citalopram.

Proposed Mechanism(s) of Action Escitalopram blocks the presynaptic serotonin reuptake and desensitizes serotonin receptors (especially 5HT1A).

Indication Major Depressive Disorder and Generalized Anxiety Disorder.

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Essential Tremor

Off Label Use

Definition

Panic disorder, obsessive compulsive disorder, social anxiety disorder, and premenstrual dysphoric disorder.

Essential tremor (ET) is the most common movement disorder. It typically presents with bilaterally symmetric hand tremor of 4–8 Hz which is present during postural changes. The tremor is also present during action and absent at rest. A family history is positive in one-half of patients. It is felt to have an autosomal dominant inheritance. The tremor of ET is more symmetric than the tremor of Parkinson’s disease (PD). Rigidity and bradykinesia are not seen. ET often involves the upper extremities, head, and voice. It can interfere with fine motor movements such as the use of eating utensils and handwriting. There is no significant response to dopaminergic medications. The tremor tends to worsen with time. It is worsened by emotional factors and, in some patients, temporarily improved by consumption of alcohol.

Side Effects Serious Seizures, mania, and suicidal ideation (all considered rare).



▶ Action Tremor ▶ Postural Tremor



Essential Tremor

Shaking

Estelle v. Smith (1981) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition 2

A NNA D E P OLD H OHLER , M ARCUS P ONCE DE LEON 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Synonyms

References and Readings Cersosimo, M. C., & Koller, W. C. (2004). Essential tremor. In R. L. Watts, & W. C. Koller (Eds.), Movement disorders (2nd ed., pp. 431–458). New York: McGraw-Hill.


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Cross References

On December 28, 1973, Ernest Benjamin Smith was arrested for murder as a result of an armed robbery in a grocery store in which a clerk was fatally shot by Smith’s accomplice. The state of Texas decided to seek the death penalty. Smith was interviewed by Dr. Grigson, a psychiatrist, for approximately 90 min, to determine whether or not he was competent to stand trial. Dr. Grigson found that Smith was competent to stand trial. Smith was charged and convicted of capital murder. During the

Estimation Methods

sentencing phase, Dr. Grigson underwent direct examination before the jury and testified that Smith was a ‘‘very severe sociopath’’ and that ‘‘he will continue his previous behavior and that his sociopathic behavior will ‘only get worse’.’’ Dr. Grigson also testified that Smith had no ‘‘regard for another human being’s property or for their life, regardless of who it may be,’’ that there is ‘‘no treatment. . .that in any way at all modifies or changes this behavior,’’ that he ‘‘is going to go ahead and commit other similar or same criminal acts if given the opportunity to do so,’’ and that he ‘‘has no remorse or sorrow for what he has done.’’ Smith was sentenced to death. The entirety of Dr. Grigson’s opinion and testimony was based solely on his 90-min mental status examination of Mr. Smith. However, Dr. Grigson never provided Smith with a full disclosure of the potential uses for the information gained during the competency evaluation; most notably, he did not indicate that such information could be used to generate predictions about his future behavior. Regardless, the Texas Court of Appeals upheld the conviction and death sentence. Despite this ruling, the Federal District Court removed the death penalty because it found a constitutional error with admitting Dr. Grigson’s testimony during the sentencing phase. Specifically, the court ruled that admission of Dr. Grigson’s testimony about Mr. Smith’s mental status during the penalty phase of the trial violated his 5th Amendment right against self-incrimination because he was not informed during the pretrial mental status examination that he had a right to remain silent and that any statement he made could be used against him during capital sentencing proceedings. Also, to perform a mental health evaluation without defense counsel’s knowledge is also a violation of the defendant’s 6th Amendment right to effective counsel.

Cross References ▶ Dusky v. United States (1960)

References and Readings Denney, R. L., & Sullivan, J. P. (2008). Constitutional, judicial, and practice foundations of criminal, forensic neuropsychology. In R. Denney, & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford. Dusky v. U.S. 262 U.S. 402 (1960). Estelle v. Smith, 451 U.S. 454 (1981). Weissman, H. N., & DeBow, D. M. (2003). Ethical principles and professional competencies. In A. Goldstein (Ed.), Handbook of Psychology (Vol. 11). Forensic Psychology, New Jersey: Wiley.

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Estimated Premorbid Intelligence ▶ Premorbid Intelligence

Estimation Methods J EANNIE L ENGENFELDER Kessler Foundation Research Center West Orange, NJ, USA

Synonyms Premorbid abilities

Definition An important role of neuropsychological assessment is to identify changes or impairments in cognitive functions from previous performance following an illness or injury. Since premorbid neuropsychological data are rarely available, methods of estimating premorbid abilities are utilized.

Current Knowledge Some neuropsychologists utilize a clinical estimation of premorbid abilities drawing on information from school, military, and work records as well as family or even selfreport to provide a global impression of the individuals’ premorbid functioning. While this approach allows the neuropsychologist to incorporate much information from the individual’s background and history, there is a lack of standardization to incorporate the information as well as limitations on specificity of premorbid abilities. Therefore, at best one could only conclude very general estimations of function such as above average, average, and below average. Other clinicians utilize a more formal estimation of premorbid abilities based on either (1) demographic variables, (2) current performance on standardized measures, or (3) a combination of current performance and demographics. Demographic variables commonly used to estimate premorbid ability include education, occupation, age,

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gender, and race. In particular, education and then occupation have demonstrated the highest correlations with intelligence measures. Methods for predicting premorbid intelligence quotient (IQ) scores from demographic variables have been developed for the various Wechsler measures (see Strauss, Sherman, & Spreen, 2006 for a review). A second method of estimating premorbid abilities involves using current test scores. This approach is based on the concept that an injury or illness will not impact all aspects of intelligence equally and there are certain abilities thought to be more resistant to the effects of injury or illness. These so-called ‘‘hold’’ tests typically assess crystallized knowledge, such as vocabulary. These functions are more localized in the brain and less dependent on distributed white matter pathways, and therefore remain relatively intact with many types of neurologic insult (e.g., Traumatic Brain Injury, Multiple Sclerosis). Several measures are commonly used to assess premorbid abilities and these include word reading tests such as the National Adult Reading Test (NART), the reading subtest of the Wide Range Achievement Test (WRAT), Wechsler Test of Adult Reading (WTAR), and Wechsler subtests such as Vocabulary, Information, and Picture Completion. Of course, these methods would be inappropriate in populations with known language or reading disability, such as persons with aphasia, history of dyslexia, or advanced Alzheimer’s disease. These methods would also be inappropriate for persons whose primary language is different from the language of the test. A third method of estimating premorbid abilities has been the combination of demographic variables with current test scores. This method of combining demographics and current performance has demonstrated increased ability to predict performance above what using either demographics or current performance alone. For example, using WTAR performance combined with demographics variables has demonstrated an additional 4–7% ability to predict IQ and memory performance than using the WTAR alone (Psychological Corporation, 2001).

Cross References ▶ National Adult Reading Test ▶ Wechsler Adult Intelligence Scale (All Versions) ▶ Wechsler Test of Adult Reading ▶ Wide Range Achievement Test

References and Readings Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests: Administration, norms, and commentary (3rd ed.). New York: Oxford University Press.

Ethical Principles in Forensics R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Ethics

Definition Recent surveys have demonstrated that neuropsychologists are increasingly being asked to consult in forensic cases. As a result, they need to be aware of, and vigilant about, the associated ethical issues that arise. Grote (2005) notes that neuropsychologists have to be constantly vigilant of the need to produce unbiased and appropriately informed decisions if courts can be expected to rely on their opinions and that a failure to maintain this neutrality could lead others to view the field in a negative light, with particular biases directed toward the needs of the retaining party. Secondly, because neuropsychologists may not be fully aware of all of the potential ethical and legal implications that arise when reports are used in forensic settings, they may be especially at risk of committing an ethical violation without even being aware that such a violation has occurred. Some areas that are especially at risk for ethical violations in forensic practice include: release of raw test data and other test security issues, third-party observers, informed consent for assessment, and use of interpreters. For these, and other, reasons, neuropsychologists make efforts to educate themselves about the ethical obstacles that arise in the practice of forensic neuropsychology. At the very least, psychologists should be familiar with the most recent ‘‘Ethical Principles of Psychologists and Code of Conduct’’ (American Psychological Association, 2002) as well as the ‘‘Specialty Guidelines for Forensic Psychologists’’ (American Psychological Association, 1991)

Ethics in the Practice of Neuropsychology

Cross References ▶ Specialty Guidelines for Forensic Psychologists

References and Readings American Academy of Clinical Neuropsychology (2007). AACN practice guidelines for neuropsychological assessment and consultation. The Clinical Neuropsychologist, 21, 209–231. American Psychological Association (2002). Ethical principles of psychologists and code of conduct. American Psychologist, 57, 1060–1073. Committee on Ethical Guidelines for Forensic Psychologists (1991). Specialty guidelines for forensic psychologists. Law and Human Behavior, 15, 655–665. Grote, C. (2005). Ethical practice of forensic neuropsychology. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts (3rd ed.). New York: Guilford Press. Weissman, H. N., & DeBow, D. M. (2003). Ethical principles and professional competencies. In A. Goldstein (Ed.), Handbook of psychology (Vol. 11). Forensic psychology, Wiley: New Jersey. The 2002 APA ‘‘Ethical Principles’’ can be viewed in their entirety at: http://www. apa.org/ethics/code2002.html

Ethics ▶ Ethical Principles in Forensics

Ethics in the Practice of Neuropsychology T HOMAS R. K ERKHOFF, S TEPHANIE L. H ANSON College of Public Health and Health Professions University of Florida Gainesville, FL, USA

Definition Ethics provides a framework for understanding and examining morality, which broadly defined includes socially accepted norms about appropriate and inappropriate human conduct and encompasses consensual virtues, rights, and governing principles and rules. Ethics codes reflect the morality of specific groups, such as health care professionals. The American Psychological Association’s

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Ethical Principles of Psychologists and Code of Conduct (APA, 2002) offers both guiding principles and ethical standards. Guiding principles reflect the values of the profession and are aspirational in nature. Conversely, ethical standards establish minimally acceptable professional behaviors and are enforceable codes of conduct whose ultimate purpose is protection of consumer welfare. Clinical neuropsychologists who are APA members or whose state associations or licensing boards have adopted the APA Ethics Code are bound by its standards for professional conduct. Additionally, members of the Association of Postdoctoral Programs in Clinical Neuropsychology (APPCN) are bound by the APPCN Code of Conduct, which is meant to be complementary to the APA Ethics Code and focuses on ethical conduct and the process for addressing ethics complaints.

History In 1938, the American Psychological Association (APA) created the ad hoc Committee on Scientific and Professional Ethics to evaluate the need for an ethics code. Although the 1940 committee report noted an ethics code was premature, the committee recommended that APA establish a standing committee to review complaints and formulate ethics rules as needed. By 1947, the same committee reported that the time had come for a formal code governing psychology practice (Peak, 1947). A formal process was undertaken to establish an ethics code based on Hobbs’ recommendation that the development of such a code must include broad participation by APA members. Hobbs (1948) believed that the ethics code should be useful in everyday decision-making and not simply represent a blueprint for avoiding sanction. Therefore, the Committee on Ethical Standards for Psychology requested examples of ethical issues experienced by its members. Through classification and revision of categories of the critical incidents provided (i.e., the critical incident method), the first ethics code was created, which was initially recommended for adoption at the 60th Annual Business Meeting of APA (Adkins, 1952). The APA Ethics Code has been revised 9 times, with the most recent changes having been adopted by the APA Council of Representatives in 2002 and implemented in 2003. (Previous code revisions have been extensively reviewed by Canter et al., 1994). Examples of changes incorporated in the 2002 code include clarification of multiple relationships, acceptability of release of test data to the client or others listed on the release form,

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and enhanced sensitivity to cultural factors. Professional ethics codes are by nature evolutionary, reflecting changes in the profession and the sociocultural context in which the profession functions. There have been numerous works in neuropsychology discussing the application of the Ethics Code to clinical practice issues (e.g., Binder and Thompson, 1994; Bush, 2005).

suggest that appropriate use of assessments (in addition to boundaries of competence and ethical-legal issues) is a primary ethical concern among clinical neuropsychologists (Brittain, Frances, and Barth, 1995; Bush, 2007). Clearly, proficiency in ethics is an ongoing and lifelong process. Participating in continuing education, being aware of one’s own strengths and weaknesses, and using mentors are all critical to ensure ethical competence.

Education and Training Enforcement Formal training (coursework and practical experience) in professional ethics occurs in all APA-accredited graduate training programs in psychology. Professional ethics is also included as part of the generic clinical core in education and training for clinical neuropsychologists. Postdoctoral residency requirements dictate that, upon completion, students should be eligible for board certification in clinical neuropsychology (Hannay et al., 1998). Professional ethics is currently one of three main components (the others being work sample review and fact finding) of the oral examination for certification by the American Board of Clinical Neuropsychology. Examinees are expected to be knowledgeable about the content of the APA Ethics Code and to apply the code’s concepts to their neuropsychological practice. In addition to the Ethics Code, psychologists must maintain awareness of practice guidelines recommended within their specialty, which are commonly reflected in policy statements and position papers by the relevant APA division or related organizations. The American Academy of Clinical Neuropsychology, for example, posts policy statements on its web site, and recently published guidelines regarding neuropsychological assessment and consultation (Board of Directors, 2007). Previous guidelines have been provided by AACN, APA Divisions 22 (Rehabilitation Psychology) and 40 (Clinical Neuropsychology), and the National Academy of Neuropsychology (NAN) on a broad range of issues, such as third party observers, functional magnetic resonance imaging, and working with military veterans with traumatic brain injury. Proficiency in professional ethics is required for licensure in every state. In order to address these requirements, APA, other professional organizations (e.g. International Neuropsychological Society), state psychological associations, and private companies all offer continuing education credit in applied ethics. Beyond these basic training requirements, training and supervision from experienced clinical neuropsychologists is important to ensure competence in the use of unfamiliar neuropsychological assessment tools. Both empirical and anecdotal reports

The APA Ethics Committee publishes an annual report of its activities in the American Psychologist, including ethics case data (comparative data across several years) regarding ethics complaint adjudication. Enforcement decisions can potentially affect membership in the professional association. The process of enforcement also requires ongoing cooperative relationships with state Boards of Psychology, and in some cases the legal system. Ethics committees nested within state Boards of Psychology conduct their own investigations regarding ethical requirements of licensure, and many publish an annual list of license suspensions and revocations in order to inform the public. In a very real sense, enforcement regarding the APA Ethics Code reflects different levels of processing. At the state level, decisions relate to adherence to state statute and requirements regarding licensure, while at the national level ethics committee decisions impact ethical requirements of APA membership. Professional boards are also empowered to control their membership, with ethical violations being potential grounds for rescinding membership status. For example, ABCN has such authority.

Ethical Decision-Making Ethics is as much about process as it is about content. Ethics codes and policy statements inform practice, but it is the individual psychologist who must thoughtfully weigh the factors involved in each unique clinical situation to reach an ethical decision. Several models exist that offer guidance in ethical analysis (e.g., Kitchener, 2000; Hanson, Kerkhoff, and Bush, 2005). For example, Hanson et al. (2005) illustrated a model for applied ethical decision-making in the context of a casebook relevant to psychology practice in different health settings (see Table 1). Collegial consultation during the process is strongly encouraged, particularly when faced with ambiguous, challenging, or emotionally charged issues.


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Ethics in the Practice of Neuropsychology. Table 1 Ethics decision-making model (Hanson et al., 2005) Steps Titles

Details

I.

Principles, concepts, and standards

Identify the specific ethical principles, concepts and standards in conflict

II.

Context, stakeholder issues

Understand the events leading up to the ethical conflict, including historical and social context, stakeholders’ roles and attributions

III.

Organizational/legal issues

Consider organizational rules/regulations and legal issues related to the conflict

IV.

Resolution

Synthesize information gathered to generate possible solutions based on balancing benefits and risks associated with each and desired outcome(s)

V.

Disposition

Select a course of action and implement it

VI.

Evaluation

Review the actual outcome(s) to facilitate learning and modify if necessary

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Applications in Neuropsychology The rich clinical neuropsychology literature in applied ethics reflects efforts to (1) thoughtfully deliberate on complex issues; (2) identify areas of ethical concern that enhance sensitivity to one’s own behavior and/or lead to the formulation of practice guidelines; (3) provide guidance in applying the Ethics Code; (4) define parameters of individual professional conduct; and (5) ground practice issues in empirical analysis. This literature includes both theoretical and empirical work as well as positions taken by APA Division 40 (Clinical Neuropsychology) and related organizations (e.g., other APA divisions, neuropsychology specialty boards, clinical practice organizations) regarding specific aspects of neuropsychological practice. Bush (2005) provides comprehensive collections of commentary on ethical issues in neuropsychology. Because of the range of ethical issues involved in neuropsychological practice, the APA ethical principles are used to organize the discussion below. These principles include beneficence and nonmaleficence (prevent harm/facilitate good or benefit; do no harm); fidelity and responsibility (establish and preserve trust; professionally responsible to the individual, community, and society); integrity (accuracy, honesty, and truthfulness); justice (provision of fair access to and quality of service); and respect for people’s rights and dignity (respect client self-determination and individual differences). While by no means all encompassing, the issues discussed both characterize the current state of the field and foreshadow future ethical challenges. Beneficence and Nonmaleficence: Competent neuropsychological evaluation can lead to correct diagnosis, effective advocacy, appropriate treatment and follow up,

as well as support successful litigation. Conversely, inappropriate evaluation and poorly defined role boundaries can compromise both short-term and long-term client welfare. For example, with the burgeoning opportunities for neuropsychologists in forensic evaluation, the roles available (client’s evaluator, legal consultant/expert witness, friend of the court, social advocate, etc.) must be carefully navigated to avoid potentially harmful multiple relationships. Similarly, ‘‘contingency fees, attempts by retaining parties to control examination procedures and findings,. . . test security and third-party observers’’ represent other ethical risks (Bush et al., 2008, p. 340). Thorough foundational training, continuing education, experience applying the APA Ethics Code, and setting appropriate role boundaries are all required to minimize ethical risk, especially when one faces the potentially adversarial nature of the legal system (Bush, 2005; Bush et al., 2008). Fidelity and Responsibility: Psychologists have an ethical responsibility to stay abreast of the changing field of practice as well as to carefully consider the implications of new technology and data. For example, given the literature of the past 10 years, it would be professionally irresponsible to omit symptom validity assessment from a neuropsychological evaluation without an extremely clear justification (Bush, 2005; Iverson, 2006). Similarly, psychologists must remain vigilant regarding whether specific tests are normed adequately for diverse populations of interest. As another example, the advent of the internet provides a potentially exciting methodology for developing and modifying assessment tools and reaching underserved populations. However, adherence to well articulated psychometric principles remain paramount (Naglieri et al., 2004). By extension, neuropsychologists are challenged to preserve the valuable interaction

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between observed behavior during the assessment process and test results, especially when neurological conditions are significantly played out through behavioral pathways. Achieving this critical interaction may be threatened by technological barriers inherent in distance assessment via the internet. Integrity: In addition to skills acquisition, honest, accurate therapeutic engagement is a foundation for trust. Examples of ethically questionable behaviors threatening integrity include intentionally misconstruing or not being forthright about the amount of control one has over professional records, suppressing information not favorable to one’s client, or misrepresenting credentials or findings for either the client’s or personal gain. In addition, biases, such as those held toward specific cultures, and lack of knowledge can lead to inaccurate representation of one’s skills (e.g., failing to present limitations regarding one’s ability to interpret test results). What one does not know can harm the client; therefore, achieving and maintaining professional competence (Bush et al., 2008) and self-evaluation to insure unintentional biases and inconsistent practices do not interfere with appropriate ethical behavior are necessary. Consultation with colleagues can be particularly helpful in identifying biases and determining their impact on practice. Justice: Justice is placed at risk when psychologists compromise or inequitably deliver services or avoid obligations. Neuropsychologists who choose to limit evaluations in the interest of cost control undermanaged care must ethically defend the adequacy of battery comprehensiveness to address questions of interest. Individuals choosing to avoid the contentious legal process in order to sidestep potential ethical conflicts may well serve to harm a client needing the special expertise or information held by the forensic neuropsychologist. In addition, changing one’s approach based on who is being represented (defense or prosecution) brings into question fair practice. Thorough knowledge of the intricacies of building a legal case and courtroom procedures will help the neuropsychologist avoid ethical pitfalls. To the extent that preservation of a balanced presentation of properly interpreted neuropsychological assessment data is achieved, justice can be facilitated. Respect for People’s Rights and Dignity: Acquiring informed consent and protecting confidentiality remain primary methods of respecting the autonomous rights of individuals served by clinical neuropsychologists. Both the 2002 APA Ethics Code and NAN offer guidance regarding appropriate consent acquisition (JohnsonGreene, 2005). Adequate informed consent must account

for the individual’s cognitive processing ability, prioritizing the components of the communication pertinent to the professional service to be rendered, the nature of the biological impairment that may call capacity into question, and cultural factors that may impact effective communication of information vital to allow consent. Careful inquiry into the individual’s understanding of the issues, deliberation of alternatives and consequences, and demonstrated ability to communicate a decision remain primary goals in achieving adequate informed consent (Appelbaum and Grisso, 1988). Other issues of relevance may be the referral source and limits of confidentiality. More broadly, protecting confidentiality in small communities requires extra consideration, from office location to proactively agreeing upon how to respond to chance encounters. Through ethically appropriate and proactive management of consent and confidentiality issues, the neuropsychologist facilitates the individual’s rights and welfare.

Future Challenges The proliferation of tests and rapid expansion of our understanding of brain–behavior relationships challenge even the most conscientious psychologist to stay abreast of field developments. Defining and redefining one’s area of competence will remain a challenge for all psychologists. Indeed, new neurophysiological, neurochemical, and interventional neurological technology will place even higher standards of specialized professional competence as brain–behavior concomitants of these technologies are used in research and practice (Steinbock, Arras, and London, 2009). Additionally, the explosion of computer-based technology outpaces our response to ethical concerns associated with its use/potential use for professional assessment and intervention. Similarly, medical advances will present new opportunities for which ethical guidance will not be well formulated. Intriguing ethical challenges inherent in emerging technologies in neurobiology and human enhancement relevant to neuropsychological practice and research will test current limits of ethical procedures and analyses. Balancing consumers’ rights with consumer protection in an increasingly regulated society, as illustrated by the complexities of the release of test data provision under the 2002 Ethics Code, will also pose unique challenges in aspiring to uphold all the ethical principles. Finally, the impact of increasingly culturally diverse populations on competent neuropsychological practice has yet to be fully realized.

EuroQol/EQ-5D

References and Readings Adkins, D. C. (1952). Proceedings of the sixtieth annual business meeting of the American Psychological Association, Inc., Washington, D.C. American Psychologist, 7, 645–670. American Psychological Association. (2002). Ethical Principles of Psychologists and Code of Conduct. American Psychologist, 57, 1060–1073. Appelbaum, P., & Grisso, T. (1988). Assessing patients’ capacities to consent to treatment. New England Journal of Medicine, 319, 1635–1638. Beauchamp, T., & Childress, J. (2001). Principles of biomedical ethics (5th ed.). New York: Oxford University Press. Binder, L. M., & Thompson, L. L. (1994). The ethics code and neuropsychological assessment practices. Archives of Clinical Neuropsychology, 10(1), 27–36. Board of Directors, American Academy of Clinical Neuropscychology. (2007). American Academy of Clinical Neuropsychology (AACN) practice guidelines for neuropsychological assessment and consultation. The Clinical Neuropsychologist, 21, 209–231. Bush, S. S. (Ed.). (2005). A casebook of ethical challenges in neuropsychology. New York: Taylor & Francis Publishers. Bush, S. S. (2007). Ethical decision making in clinical neuropsychology. American Academy of Clinical Neuropsychology: Oxford University Press. Bush, S. S., Grote, C. L., Johnson-Greene, D. E., & Macartney-Filgate, M. (2008). A panel interview on the ethical practice of neuropsychology. The Clinical Neuropsychologist, 22(2), 321–344. Bush, S. S., Ruff, R. M., Troster, A. I., Barth, J. T., Koffler, S. P., Pliskin, N. H., et al. (2005). Symptom validity assessment: Practice issues and medical necessity. NAN Policy and Planning Committee. Archives of Clinical Neuropsychology, 20, 419–426. Canter, M., Bennett, B., Jones, S., & Nagy, T. (1994). Ethics for psychologists: A commentary on the APA Ethics Code (1st ed.). Washington, DC: American Psychological Association. Hannay, H. J., Bieliauskas, L. A., Crosson, B. A., Hammeke, T. A., Hamsher, K. deS., & Koffler, S. P. (1998). The Houston conference on specialty education and training in clinical neuropsychology. Archives of Clinical Neuropsychology, 13, 157–250. Hanson, S., Kerkhoff, T., & Bush, S. (2005). Health care ethics for psychologists: a casebook. Washington, DC: APA Press. Hobbs, N. (1948). The development of a code of ethical standards for psychology. American Psychologist, 3, 80–84. Iverson, G. L. (2006). Ethical issues associated with the assessment of exaggeration, poor effort, and malingering. Applied Neuropsychology, 13(2), 77–90. Johnson-Greene, D. (2005). Informed consent in clinical neuropsychology practice. Official statement of the National Academy of Neuropsychology. Archives of Clinical Neuropsychology, 20, 335–340. Kitchener, K. S. (2000). Foundations of ethical practice, research, and teaching in psychology. Mahawah, NJ: Lawrence Erlbaum Associates. Naglieri, J., Drasgo, F., Schmit, M., Handler, L., Prifitera, A., Margolis, A., et al. (2004). Psychological testing on the internet. American Psychologist, 59(3), 150–162. Peak, H. (1947). Proceedings of the fifty-fifth annual business meeting of the American Psychological Association, Inc., Detroit, Michigan. American Psychologist, 2, 468–510. Steinbock, B., Arras, J., & London, A. (2009). Ethical Issues in Modern Medicine (7th ed.), Boston, Massachusetts: McGraw-Hill Publishers.

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ETT ▶ Endotracheal Tube

EuroQol Measure ▶ EuroQol/EQ-5D

EuroQol/EQ-5D J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms EuroQol measure; EQ-5D

Description Format and Subsections of the EQ-5D The EQ-5D is a global measure of health status that consists of two main sections: the EQ-5D descriptive system, and the EQ Visual Analog Scale (VAS). The former contains five items covering mobility, self-care; usual activities such as work, study, housework, family, or leisure; pain/discomfort; and anxiety/depression, with the respondent choosing one of the three statements that best describes their state of health in that domain. The three response options are ‘‘no problems’’, ‘‘some problems,’’ and ‘‘severe problems.’’ For example, in the mobility section, the response options are as follows: 1. I have no problems in walking around 2. I have some problems in walking around 3. I am confined to bed The EQ VAS is a 20-cm vertical line marked at 100 equidistant points, with every ten lines marked with a number from 0 (‘‘worst imaginable health state’’) to 100 (‘‘best imaginable health state’’). The respondent marks the line at the point that represents their health state on that 1 day.

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There is a third, optional section of the EQ-5D that consists of nine demographic questions (the socio-demographic questions, SDQ). The entire EQ-5D takes only a few minutes to complete.

Administration of the EQ-5D The EQ-5D can be either self-rated with an interviewer present, rated through an interview (face-to-face or by telephone) or completed by proxy report. An Interactive Voice Response system has also been developed, which allows participants to enter their responses over an automated telephone system, in a range of languages, to a tollfree number (see www.euroqol.org for details). As of June 2009, there were official translations of the EQ-5D available in 102 languages, with more in development. A sample version of the measure can be found online, but for other language versions and to use the EQ-5D, permission must be obtained from the EuroQol Executive Office, which may include a license fee.

then to specify the amount of time they would sacrifice to live in state 11111 instead.

Historical Background The EQ-5D was developed by the EuroQol group, an international network of researchers set up in 1987, with the sole focus of developing a standardized, non-diseasespecific measure of health-related quality of life, with the hope that this would enable cross-national comparisons to be made. It was designed to be quick and easy to administer, so that it could be used in studies that also needed more detailed disease-specific measures. The EuroQol group maintains records of researchers using the EQ-5D, and stages annual meetings regarding the relevant research. Over the years, the measure has been translated into many different languages, employed in a great number of studies across the world, and is used in a wide variety of clinical areas (see www.euroqol.org for a list of such areas).

Psychometric Data Scoring Responses to the EQ-5D Responses to each item on the EQ-5D descriptive system are given a one-digit value, with responses to all five domains giving a five-digit code, describing the respondent’s health state. The code 11111 therefore indicates no reported problems in any domain; 55555 indicates severe problems in every domain; and 11222 indicates moderate problems in usual activities, pain/discomfort, and anxiety/depression. There are 243 possible codes, along with two additional codes not obtained by means of the selfreport measure, which represent an unconscious state and death. The VAS is scored according to the number closest to the line drawn by the respondent – a value between 0 and 100. Responses from the EQ-5D can be presented in EQ-5D code format as a ‘‘health profile’’ demonstrating functioning in each of the five domains, either in terms of VAS scores as self-rated overall health or as a ‘‘weighted index’’ score. The procedure for obtaining weighted index scores differs according to the country in which the measure is being used, as the weights themselves are derived from studies, where groups representative of the general population in a given country have been asked to ‘‘value’’ particular health states. This valuation is obtained either by asking participants to assign a VAS value to a variety of theoretical health states or by the time trade-off (TTO) technique in which participants are asked to imagine they live in a certain health state for a period of 10 years, and

Brazier, Jones, and Kind (1993) compared the EQ-5D with the SF-36 (another more detailed health status measure) in a group of greater than 1,000 members of the general population in the UK to examine the validity of the EQ-5D. There was a significant association between EQ-5D and SF-36 total scores; EQ-5D scores differed significantly between groups with and without chronic health conditions. Hurst, Kind, Ruta, Hunter, and Stubbings (1997), in a study of patients with rheumatoid arthritis, found significant correlations between disease-specific measures of health status and the EQ-5D. All of these findings provide evidence for the EQ-5D’s validity, and other studies have found similar evidence in a variety of clinical conditions. However, Brazier et al. (1993) also found that the SF-36 was more sensitive to health problems than the EQ-5D, with the latter showing large ceiling effects in the general population. Dorman, Slattery, Farrell, Dennis, and Sandercock (1998) found good test–retest reliability of EQ-5D responses in a study with people who had experienced a stroke, with kappa values for individual items ranging from 0.63 to 0.8 at a 3-week interval. They also found the value-weighted index and VAS to be internally consistent, with intra-class correlation (ICC) coefficients of 0.86 for both scales. Hurst et al. (1997) also reported that the test–retest reliability of the EQ-5D at 2-week and 3-month intervals in a group of people with rheumatoid arthritis was good (ICCs > 0.70).

Event-Related Paradigms

Population norms from which to determine valueweighted scores are available for 15 different countries, based on data from between 400 and 6,000 respondents per country. These norms can be obtained from the EuroQol office.

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Hurst, N. P., Kind, P., Ruta, D., Hunter, M., & Stubbings, A. (1997). Measuring health-related quality of life in rheumatoid arthritis: Validity, responsiveness and reliability of EuroQol (EQ-5D). British Journal of Rheumatology, 36, 551–559. The EuroQol website (www.euroqol.org) contains much useful information on the EQ-5D, as well as guidance towards locating a variety of more in-depth sources of information.

Clinical Uses As the EQ-5D provides a broad overall measure of health status, its utility on an individual clinical basis may be limited; one would most likely require a more detailed condition-specific instrument as well. However, the overall indication of health provided, particularly on the EQ VAS, could be a relatively useful and informal means of measuring change in perceived health over time or in response to treatment (though it should be remembered that both parts need to be administered to constitute the official EQ-5D). The descriptive system may be a useful outcome measure in clinical trials, but again, its scope means it could well be insensitive to small changes in health status. The EQ-5D has been quite heavily criticized in terms of its valuation system, with these criticisms principally relating to the methods of obtaining such valuations (the VAS and TTO methods, each having their own limitations), and the resulting validity of those valuations. However, these criticisms relate to the weighted index score and other EQ-5D-derived valuations of health status, most often relevant in health economics. They do not relate to the EQ-5D scores most relevant in clinical neuropsychology research and practice, that is, the descriptive system, and VAS ratings of one’s current state of health.

Cross References ▶ General Well-Being Schedule ▶ SF-36/SF-12

References and Readings Brazier, J., Jones, N., & Kind, P. (1993). Testing the validity of the EuroQol and comparing it with the SF-36 health survey questionnaire. Quality of Life Research, 2, 169–180. Cheung, K., Oemar, M., Oppe, M., & Rabin, R. (2009). EQ-5D user guide. Downloaded from http://www.euroqol.org/eq-5d/whatis-eq-5d/how-to-use-eq-5d.html 13.07.2009 at 12.20 pm. Dorman, P., Slattery, J., Farrell, B., Dennis, M., & Sandercock, P. (1998). Qualitative comparison of the reliability of health status assessments with the EuroQol and SF-36 questionnaires after stroke. Stroke, 29, 63–68.

E Event-Related Paradigms J EFFREY S AMUEL North Broward Medical Center Deerfield Beach, FL, USA

Synonyms Auditory and visual evoked potentials; ERP’s; Evoked potentials

Definition Event-related paradigms (ERPs) are time locked and stereotyped brain responses to some ‘‘event.’’ The ‘‘event’’ is a stimulus that evokes an electrophysiological response. The stimulus can be a sound, a simple visual pattern, a mental event or thoughts such as recognition of a specific target stimulus or the absence of a stimulus as when an increased time elapses between stimuli. The recorded brain response is a very small electrical voltage of between 1 and 100 millionths of a volt and is recorded over the scalp by a very sensitive amplifier and computer averaging equipment that can recognize it as a consistency in the seemingly random waveforms of the overlying EEG. The response can be a singular waveform or a series of waveforms that indicate the chain of processors that respond in sequence to the stimulus as the response travels along the pathway from the primary receptor to the cortex of the brain. The location of the activity on the scalp is related to the pathway taken by the response from the primary processor to the specific area of cortex that is processing that stimulus. Responses that occur from 0 to 200 ms are called ‘‘exogenous’’ and are felt to be the direct result of the presented stimulus. Responses occurring after 200 ms are called ‘endogenous’ and are felt to be correlated with the conceptual manipulation of the stimulus, the thought.

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Current Knowledge There are both clinical and research areas where ERPs can be used. The clinical uses evaluate the integrity of the neural processors that respond to the stimulus. Abnormal timing or amplitude of the waveforms may indicate damage or dysfunction in the brain tissues that should be activated. Some of the more common clinical response patterns are the Auditory Brain Response (ABR), the Mid Latency Response (MLS), and the ‘‘slow’’ and ‘‘late’’ cortical auditory ERPs. The ABR is seen 1.5–15 ms from the stimulus. This response is the generated activity of the brain stem auditory structures in the pathway from eighth cranial nerve to the lateral lemniscus and inferior colliculus of the upper brain stem. The MLS (25–50 ms) originates in the upper brain stem and goes to the auditory cortex. The ‘‘slow’’ cortical auditory ERPs (50–200 ms) are generated in the auditory cortex. The ‘‘later’’ cortical auditory ERPs, especially the ‘‘mismatch negativity,’’ indicate a change in the characteristics of the stimulus. ERPs are valuable in research. They are very high in ‘temporal’ resolution and can reveal minute changes in sensory and cognitive brain responses to stimulus tasks and can also detect results of a thought or perception. Some of the areas where ERPs have been studied include attention and information processing (P50), emergence from the vegetative and minimally conscious states, and receptive memory. While ERPs are being recorded, fMRI can provide anatomic localization of these processes.

Handy, T. C. (2004). Event-related potentials: A methods handbook. Cambridge, MA: The MIT Press (B&T), ISBN 0262083337. http:// www.emedicine.com/neuro/topic69.html. Kemp, A. H., Hopkinson, P. J., Hermens, D. F., Rowe, D. L., Sumich, A. L., Clark, C. R., Drinkenburg, W., Abdi, N., Penrose, R., McFarlane, A., Boyce, P., Gordon, E., & Williams, L. M. (2008). Fronto-temporal alterations within the first 200 ms during an attentional task distinguish major depression, non-clinical participants with depressed mood and healthy controls: A potential biomarker? Human Brain Mapping. Luck, S. J. (2005). An introduction to the event-related potential technique. Cambridge, MA: The MIT Press, ISBN 0262621967. Vanhaudenhuyse, A., Laureys, S., & Perrin, F. (2007). Cognitive event-related potentials in comatose and post-comatose states. Neurocritical Care, Woollams, A., Taylor, J. R., Karayanidis, F., & Henson, R. N. (2008). Event-related potentials associated with masked priming of test cues reveal multiple potential contributions to recognition memory. Journal of Cognitive Neuroscience, 20, 1114–1129.

Event-Related Potentials PAUL E. K APLAN Capitol Clinical Neuroscience Folsom, CA, USA

Synonyms Cerebral evoked potentials

Definition Cross References ▶ fMRI ▶ P300

References and Readings Fabiani, M., Gratton, G., & Federmeier, K. D. (2007). Event-related brain potentials: Methods, theory, and applications. In J. T. Cacioppo, L. G. Tassinary, & G. G. Berntson (Eds.), Handbook of psychophysiology (3rd ed., pp. 85–119). Cambridge: Cambridge University Press, ISBN 0-521-84471-0. Faran, S., Vatine, J. J., Lazary, A., Ohry, A., Birbaumer, N., & Kotchoubey, B. (2006). Late recovery from permanent traumatic vegetative state heralded by event-related potentials. Journal of Neurology, Neurosurgery, and Psychiatry, 77, 998–1000, doi:10.1136/ jnnp.2005.076554.

Usually called EVOKED POTENTIALS when the stimulus is regular, programmed using signal-averaging techniques. Signal-averaging techniques will eliminate background noise and preserve the potential generated. However, evoked potentials (EP) are but a subset of event-related potentials. Stimulation of special peripheral sensory nerve systems directly or indirectly generates cerebral potentials. The stimulation – at a regular, random, or irregular rate through time – is the event. The type of stimulation could be visual, auditory, mechanical, electric, or a combination. Event-related potentials are relatively noninvasive and complications/sequella of these procedures are infrequent and mild. The cost of these procedures is but a small fraction of MRI and CT scan studies. Both the latency and the pattern, shape of the EPs are associated with the type of stimulus and are typically recorded on the scalp. Normal values have long been established for each EP. However, as these EPs are

Evidence Based Practice

placed within background EEG activity and characteristically have low voltage, computer averaging of thousands of responses is required. Fortunately, digital equipment requires but the press of a button to be activated. Lesions within the central nervous system (CNS) which disrupt these special sensory pathways will generate an abnormal EP and thus help confirm both the presence and/or location of that lesion. Each EP has components which could be generated by a different part of the CNS and therefore could be employed in mapping the lesion. Somatosensory EPs have been used to determine nerve conduction velocity values. An EP can also be monitored during an invasive or surgical procedure to see if an affected spinal nerve root entity is still functioning. Irregular event-related potentials have also been used to determine normal and clinically abnormal dermatome patterns using tactile stroking movements. For example, after closed head injury with associated inner ear damage, absence of wave I using brainstem auditory EPs will occur. In patients with coma, using somatosensory EPs to demonstrate loss of activity after P15 will usually predict death.

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Evidence Based Practice C INDY B. I VANHOE , N ATASHA K. E ADDY Houston, TX, USA

Synonyms Evidence based medicine

Definition Evidence based practice has been defined as ‘‘the conscientious, explicit, and judicious use of current best evidence in making decisions about the care of individual patients. It is the practice of integrating individual clinical expertise with the best available external clinical evidence from systematic research.’’

Current Knowledge Current Knowledge Somatosensory cerebral evoked potentials are useful to determine sensory nerve conduction velocity within the CNS.

Cross References ▶ Cerebral Cortex ▶ Somatosensory System

References and Readings American Electroencephalographic Society. (1994). Guidelines on evoked potentials. Journal of Clinical Neurophysiology, 11, 40–73. Aminoff, M. J. (1990). Evoked potential studies in neurological diagnosis and management. Annals of Neurology, 28, 706–710. Rosenfeld, J. V., & Lennarson, P. J. (2007). Coma and brain death. In Schapira, A. H. V. et al., (Eds.), Neurology and clinical science (pp. 97–116). Philadelphia: Mosby.

Evidence Based Medicine ▶ Evidence Based Practice

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Evidence based medicine requires that a practitioner asks questions, researches the relevant information, evaluates the data for its validity and usefulness, and determines whether or not the information reviewed is appropriate for implementation in his/her specific clinical practice. Assessment of validity involves four questions: (1) Was there an independent, blind comparison with a reference standard of diagnosis? (2) Was the diagnostic test evaluated in an appropriate population of patients comparable to the patient of interest? (3) Was the reference standard applied regardless of the diagnostic test result? (4) Was the test validated in a second, independent group of patients? The topic of EBM has been criticized by some because even the most well thought out controlled experimental research study cannot control for all variables. What has been found to be true for one subgroup of patients across studies may not be able to be applied to all patients because of differing ethnicity, age, cultural, social, and economic factors. Treatment recommendations also are no longer just based on levels of evidence, but also on risk benefit ratio and cost. Therefore, evidence based practice should not supplant the practitioner’s own clinical judgment and expertise, but rather should be used, along with patient preference, as an adjunct in order to provide optimal care for their patient population.

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References and Readings Fisher, C., & Wood, K. (2007). Introduction to and techniques of evidence-based medicine. Spine, 32(Suppl. 19), 66–72. Hawkins, R. (2005). The evidence based medicine approach to diagnostic testing: Practicalities and limitations. The Clinical Biochemist Reviews, 26, 7–18. Marwick, C. (1997). Proponents gather to discuss practicing evidencebased medicine. Journal of the American Medical Association, 278(7), 531–532. Sackett, D. L., Rosenberg, W. M. C., Gray, J. A. M., Hayne, R. B., & Richardson, W. S. (1996). Evidence based medicine: What it is and what it isn’t. BMJ, 312(7023), 71–72.

Evoked Potentials C HRISTINA K WASNICA Barrow Neurological Institute Phoenix, AZ, USA

Absence of responses early after anoxic brain injury accurately predicts mortality due to the injury. Abnormal responses are also predictive of failure to emerge from coma in traumatic brain injury. Normal responses can be predictive of good outcomes both in traumatic and anoxic brain injury. Electrophysiologic recovery often precedes clinical recovery, making these tests valuable. Recent research has focused on the use of event-related potentials (ERPs). These are long latency responses, occurring more than 70 ms after sensory stimulation. In contrast to evoked potentials, ERPs are reflective of changes in the electrophysiology of the brain as it performs a cognitive task. They can be used to define areas of pathology in the diseases of central nervous system, such as Alzheimer’s disease and mild cognitive impairment. Finally, ERPs may have clinical utility in diagnosis of psychiatric disease such as schizophrenia as well as assistance in prediction of response to pharmacotherapy.

Cross References Definition Evoked potentials are waveforms that can be recorded from a peripheral nerve, spinal cord, or cerebral cortex after stimulation of a peripheral nerve. They are named based on the pathways stimulated. Somatosensory evoked potentials (SSEPs) are recorded after stimulation of upper or lower extremity peripheral nerves. Visual evoked potentials (VEPs) are cortical responses measured after visual input. Brainstem auditory evoked potentials (BAEPs) are cortical responses measured after timed auditory stimuli.

Historical Background Current Knowledge Evoked potentials have many clinical and research implications. Using sensory and motor pathways, intraoperative monitoring can be used to follow functional integrity of pathways during spinal and intracranial neurosurgical procedures. SSEPs can also be used diagnostically to delineate the location of lesions, such as peripheral nerve or spinal cord. The use of cortical evoked potentials, such as VEPs and BAEPs, can also supplement neuroimaging information in diagnostic working-up for diseases such as multiple sclerosis. Short latency evoked potentials, such as SSEPs, VEPs, and BAEPs, have been studied as prognostic indicators in traumatic and anoxic brain injury.

▶ Event-Related Paradigms ▶ Somatosensory Evoked Potentials ▶ Visual Evoked Potentials

References and Readings Claussen, J., & Hansen, H. C. (2001). Early recovery after closed traumatic head injury: Somatosensory evoked potentials and clinical findings. Critical Care Medicine, 29, 675–677. Lew, H. L., Poole, J. H., Castaneda, A., Salerno, R. M., & Gray, M. (2006). Prognostic value of evoked and event-related potentials in moderate to severe brain injury. The Journal of Head Trauma Rehabilitation, 21, 350–360. Pogarell, O., Mulert, C., & Hegerl, U. (2007). Event-related potentials in psychiatry. Clinical EEG and Neuroscience, 38, 25–34. Robinson, L. R., Micklesen, P. J., Tirschwell, D. L., & Lew, H. L. (2003). Predictive value of somatosensory evoked potentials for awakening from coma. Critical Care Medicine, 31, 960–967. Sala, F., Manganotti, P., Tramontano, V., Bricolo, A., & Gerosa, M. (2007). Neurophysiologie Clinique, 37, 399–406. Taylor, J. R., & Olichney, J. M. (2007). From amnesia to dementia: ERP studies of memory and language. Clinical EEG and Neuroscience, 38, 8–17.

Ewing Sarcoma Family Tumors (ESFT) ▶ Ewing’s Sarcoma

Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research

Ewing’s Sarcoma M I -Y EOUNG J O Private Practice Los Angeles, CA, USA

Synonyms Ewing sarcoma family tumors (ESFT); Peripheral primitive neuroectodermal tumors (pPNET)

Definition Ewing sarcoma is named after Dr. James Ewing, who first described this tumor in the 1920s. It occurs primarily in bone or soft-tissue and is most often found in the pelvis, femur, tibia, and ribs. It is one of the most common types of pediatric cancers and is usually found in children and young adults between the ages of 10 and 20, and rare past the age of 30. There is a slightly higher incidence in males than females. Pain is usually the earliest symptom and can be accompanied by swelling and fever. Nerve and spinal cord compression may cause additional symptoms such as numbness, tingling, incontinence, and paralysis. This type of tumor often metastasizes to other parts of the body. It is usually malignant with unfavorable prognosis, particularly in those with metastatic disease. Treatment often involves whole-body chemotherapy, localized surgery, and/or radiotherapy. In children, possible late delayed effects of radiotherapy may include slowed bone growth and infertility.

Cross References ▶ Late-Delayed Effects of Radiation ▶ Metastasis ▶ Neoplasm ▶ Primitive Neuroectodermal Tumor ▶ Radiotherapy ▶ Sarcoma

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Exacerbating–Remitting Multiple Sclerosis ▶ Relapsing–Remitting Multiple Sclerosis

Exaggerating ▶ Malingering

EXAMINER ▶ Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research

Excessive Alcohol Use ▶ Alcohol Abuse

Excessive Sleepiness ▶ Hypersomnia

Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research J OEL H. K RAMER , C ASEY E. K RUEGER , L ENA S INHA UCSF Memory and Aging Center San Francisco, CA, USA

Synonyms EXAMINER


Description

Ewing, J. (1921). Diffuse endothelioma of bone. Proceedings of the New York Pathological Society, 21, 17–24.

The EXAMINER is an NIH-sponsored project to develop a neuropsychological test battery that reliably and validly

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assesses executive functions (often defined as the ability to engage in goal-oriented behavior) for clinical investigations and clinical trials, and that is adaptable across a wide range of ages and disorders. Executive function (EF) refers to a constellation of cognitive abilities that include the ability to plan, organize, manage multiple tasks simultaneously, mental flexibility, inhibition, and selfmonitoring. These important behaviors are compromised in healthy elders and in people with neurological, mental and age related disorders, leading to disruption of lifestyle and loss of independence. One obstacle for progressive research in this area of cognition is a paucity of valid and reliable tasks that specifically tap domains of EF. The EXAMINER project is working to create a battery of domain specific executive function tasks that will make it easier for clinical researchers to measure these constructs in standardized ways. Cross-cultural issues are also important; the resulting tool will be available in Spanish. Although there is a general consensus that executive abilities are a central component of human cognition, there is little agreement about what these executive abilities are, how they are organized, what their relationships are with frontal brain regions, or how they should be measured. Presently, three separate approaches are integrated to conceptualize and measure executive functioning. The first approach is to parse executive ability into more discrete and measurable constructs such as working memory, set shifting, fluency, and inhibition. The second approach is to utilize tasks that may be cognitively more complex, but more strongly resemble tasks that subjects encounter in daily life (e.g., insight, decision making, social cognition). The third approach is to apply informant-based rating scales. Measures reflecting these three approaches are described below.

Discrete Constructs EF has been subdivided into more discrete subunits that are only modestly correlated with one another. These subunits include working memory, set shifting, fluency, and inhibition.

Working Memory Spatial working memory: Spatial working memory is assessed using the n-back paradigm. The n-back is composed of two subtests: the 1-back and the 2-back. In each subtest, the participant views a series of squares that vary in location. In the 1-back, the participant indicates via a keyboard press whether each square is in the same

location as the immediately preceding square. This is a test of spatial attention. In the 2-back, the participant indicates whether each square is in the same location as the square 2 before. This is a test of spatial working memory and relies on flexible updating capabilities. Verbal working memory: Verbal working memory is assessed with a dot counting task. Subjects count the number of blue circles on a computer screen filled with blue and green circles and squares. These screens are presented in series that range in length from two to six screens. At the end of each series of screens, subjects state the number of blue circles on each screen in that series. The length of the series ranges from two to six.

Set Shifting General and specific shift costs: This task is computeradministered. In task-homogeneous blocks, participants perform either Task A (e.g., classifying shapes) or Task B (e.g., classifying colors). In task-heterogeneous blocks, participants alternate between the two tasks pseudorandomly. The combination of task-homogeneous and task-heterogeneous blocks allows measurement of general switch costs (latency differences between heterogeneous and homogeneous blocks) and specific switch costs (latency differences between switch and non-switch trials within heterogeneous block).

Fluency Verbal fluency: Phonetic verbal fluency (words generated in 60-s beginning with the letters F and L); semantic fluency (animals and vegetables).

Inhibition Flanker test: In this computer-administered task, the target stimulus is a centrally presented arrow facing either to the left or right. Subjects are instructed to identify the direction of this central arrow by pressing one key for the left direction and a different key for the right direction. The targets can be flanked on either side by two arrows that are either facing in the same direction (congruent condition) or in the opposite direction (incongruent condition). The capacity for inhibition is reflected in the latency differences between the congruent and incongruent conditions. Continuous performance/Go-No Go: In this computeradministered task, there are four blocks of trials consisting of 48 successively presented stimuli. The ratio of targets to non-targets varies across the four blocks of trials, ranging from 5:1 to 1:5. Anti-saccade: There are two blocks of trials in which subjects look at a fixation point in the center of a


computer screen and move their eyes upon presentation of a laterally presented stimulus. In the first block, subjects are instructed to move their eyes in the direction of the presented stimulus. In the second block (antisaccade), subjects are instructed to move their eyes in the opposite direction of the presented stimulus. Random number generation: Subjects are asked to generate a set of 100 numbers randomly, avoiding any obvious or predictable patterns.

Cognitively Complex Tasks Insight: Subjects are asked to rate themselves on their performance immediately after completing the verbal fluency tasks. Complex task of decision-making: This is an unstructured task modeled after the 6-elements test. Subjects are presented with three booklets, each containing five pages of simple puzzles (six per page). The puzzles were designed to be cognitively quite simple (e.g., connect the dots; trace the design) but require on average between 4 and 80 s to complete. Each puzzle has a designated dollar value. Subjects are given 6 min to earn as many dollars as possible. The cost-benefit ratio of the puzzles varies systematically, requiring subjects to plan ahead and to avoid items that have a large reward value but are strategically poor choices. In addition, the average cost-benefit ratio of the items increases as a subject progresses through a book, requiring further strategic decisions about when to switch booklets.

Social Cognition The Awareness of Social Inference Test (TASIT): This modified version of the TASIT contains items from the first subtest, called the Emotion Evaluation subtest. This is designed to assess subjects’ emotion comprehension with dynamic, ecologically valid stimuli. Subjects must interpret naturalistic emotional displays that include multiple matched modalities of emotional expression, including facial movement, voice prosody, and upper body posture and gestures. Subjects watch brief (20-s) vignettes of professional actors depicting one of five basic emotions (surprised, sad, fearful, angry, and disgusted) with a semantically neutral script. The facial expressions in these videos have also been FACS-coded to ensure reliability and validity of emotional expression. After watching each video, subjects are asked to select the correct emotion from a screen with the five emotions written on it. This subtest includes 20 vignettes, four for each of the five emotions. Social norms questionnaire (SNQ): The SNQ measures subjects’ crystallized knowledge of social norms in a linguistically and cognitively simple manner. This

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20-item yes-no questionnaire is designed to determine the degree to which subjects actually understand and can accurately identify implicit but widely accepted social boundaries in the dominant US culture. This social norms questionnaire includes both inappropriate behaviors (e.g., ‘‘Cut in line if you are in a hurry,’’ ‘‘Pick your nose in public,’’ and ‘‘Wear the same shirt every day’’) and generally acceptable behaviors (e.g., ‘‘Tell a coworker your age,’’ ‘‘Blow your nose in public,’’ and ‘‘Eat ribs with your fingers,’’). The subject must decide whether the behavior is socially appropriate or not. This measure also has an alternate form for test-retest purposes. Revised Self-Monitoring Scale (RSMS): In order to measure subjects’ awareness of their own social behavior, their own self-reported degree of self-concern and self-focus will be obtained using the Lennox and Wolfe version of the Revised Self-Monitoring Scale informant-based reports. The RSMS is a 13-item measure of the subjects’ sensitivity to the expressive behavior of others, and their ability to monitor their self-presentation. The subjects’ responses will be compared to those of their informants (see below) as an indirect measure of how accurate their self-assessment is.

Informant-Based Reports The Frontal Systems Behavior Scale (FrSBe): The FrSBe is a 46-item behavior rating scale that is intended to measure behavior associated with damage to frontal systems. It consists of two rating forms: a self-rating form to be completed by the patient and a family rating form to be completed by an informant who has regular contact with the patient. Each FrSBe form yields a total score and scores for subscales measuring apathy, disinhibition, and executive dysfunction. Scores are obtained on each scale for baseline behavior and current behavior. The Behavior Rating Inventory of Executive Functions (BRIEF): The BRIEF is an inventory designed to measure executive dysfunction in children aged 5–18. It produces two indexes composed of several clinical subscales. The Behavioral Regulation Index is made up of the Inhibit, Shift, and Emotional Control subscales. The Metacognition Index is made up of the Initiate, Working Memory, Plan/Organize, Organization of Materials, and Monitor subscales. There is also a Global Executive Composite score incorporating all eight clinical scales. The Interpersonal Reactivity Index (IRI): The IRI is a questionnaire measure of both the cognitive and the affective components of empathy. Its 28 items include two 7-item subscales measuring cognitive empathy, perspective taking (PT: the tendency to spontaneously imagine the cognitive perspective of another person)

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and fantasy (FS: the tendency to project oneself into the place of fictional characters in books and movies), as well as 27-item subscales measuring emotional empathy, empathic concern (EC: the other-centered emotional response resulting from the perception of another’s emotional state) and personal distress (PD: the selfcentered emotional response involving fear or distress that results from witnessing another’s stressful circumstances or negative emotional state). Theoretically, the PD subscale reflects a primitive form of empathy that actually interferes with an effective empathic response; thus it tends to drop as the other scales rise (particularly in relation to the PT scale), and is negatively related to measures of overall social functioning. Higher scores on the PT, FS, and EC scales are associated with a more highly developed capacity for empathy. Revised Self-Monitoring Scale (RSMS): In order to measure subjects’ awareness of their own social behavior, informant-based reports will be obtained on the Lennox and Wolfe version of the Revised Self-Monitoring Scale, 13-item measure of the patient’s degree of self-concern and self-focus. This questionnaire measures the subject’s sensitivity to the expressive behavior of others, and their ability to monitor their self-presentation. This questionnaire will also be filled out by the subjects about themselves (see above), and their responses will be compared to those of their informant as another, indirect measure of how accurate their self-assessment is.

Historical Background Executive function is of increasing interest and importance in cognitive neuroscience and clinical assessment. The frontal lobes represent over 30% of the cortical surface and play a major role in the organization of behavior and cognition. The functions represented in this vast cortical expanse are complex and multi-faceted, often categorized under the category of ‘‘executive control.’’ Simple theories regarding a unitary function of the frontal lobes have been supplanted by sophisticated cognitive and neuroscience research that has helped to fractionate the broad term ‘‘executive control’’ into multiple sub-components bound together to help with the organization, initiation and control of cognition and behavior. Additionally, it is now evident that executive control is not anatomically restricted to the frontal lobes but also depends upon the intactness of subcortical white matter and the basal ganglia, and neural networks that rely on input from posterior structures (Ravizza & Ciranni, 2002).

Despite these advances, clinical investigators are faced with several challenges. For example, while the importance of preserved executive abilities for daily living is widely recognized, most neuropsychological test batteries for clinical trials do not include measures of executive ability, ignore social cognitive aspects of executive control, and do not link executive function to changes in daily living. In addition, the sheer number of available tasks reputed to measure executive function is overwhelming. It has also become clear that simple paper and pencil tasks will not always capture the real-life social and executive deficits in patients with ventromedial prefrontal injury. Tasks that capture deficits in executive control in the areas of social-cognition and activities of daily living are needed. The psychometric properties of executive tasks pose yet another challenge to clinical investigators interested in measuring executive functioning. Construct validity refers to how well an instrument measures what it purports to measure. Clinical neuropsychological instruments have been criticized for being multifactorial, drawing on several non-executive component skills. In fact, several of the top 20 ‘‘executive tasks’’ listed by Rabin, Barr and Burton (2005) in their neuropsychologists’ survey were a memory task (CVLT) and visuospatial tasks such as clock drawing, Rey–Osterrieth Complex Figure, and block design. Another psychometric issue is test-retest reliability. This has particular importance for clinical trials where researchers must be able to attribute change in cognition to the intervention and not poor reliability or practice effects (Beglinger et al., 2005; Bowden, Benedikt, & Ritter, 1992). Most current measures of executive function also have limited cross-cultural application. Often the stimuli require reasonable mastery of English (e.g., Similarities; Stroop; D-KEFS Card Sorting) or are culturally based (e.g., proverb interpretation). Executive tasks also tend to be highly correlated with education, and education levels vary across ethnic groups. Even when tasks appear to be readily translatable, assessment of differential item functioning has revealed item bias (Marshall, Mungas, Weldon, Reed, & Haan, 1997). The shortage of validated clinical measures that are applicable across ethnic and language groups poses a major obstacle to clinical research. In sum, despite the wealth of available instruments, there are continued concerns about psychometric properties, validity, generalizability to settings and populations other than the ones they were developed for, suitability for all ages and non-English speaking subjects, and adaptability for clinical trials. There is also no consensus on what the primary components of executive functioning are or how terms are defined. There remains a compelling

Executive Functioning

need to have a battery of tests that can be routinely integrated into neurobehavioral research that will reliably and validly measure constructs that clinical investigators agree are important.

Psychometric Data Psychometric properties of the EXAMINER are to be determined. The EXAMINER project is currently in year three of a 5 year contract ending December 2010. Data collection is ongoing at ten academic and clinical sites. To date, there have been approximately 540 participants in the EXAMINER project. Roughly half of the participants are normal controls and half have been clinically diagnosed with conditions included in our study design. Diagnoses include Attention-Deficit Hyperactivity Disorder, Alzheimer’s disease, mild cognitive impairment, frontotemporal dementia, Huntington’s disease, Parkinson’s disease, multiple sclerosis, progressive supranuclear palsy, sickle cell anemia, traumatic brain injury, focal lesions, and very low birth weight children. Currently, 22% of participants are Hispanic, 12% are African-American, 49% are Caucasian, and 17% are of other ethnicities.

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Bowden, S. C., Benedikt, R., & Ritter, A. J. (1992). Delayed matching to sample and concurrent learning in nonamnesic humans with alcohol dependence. Neuropsychologia, 30(5), 427–435. Marshall, S. C., Mungas, D., Weldon, M., Reed, B., & Haan, M. (1997). Differential item functioning in the mini-mental state examination in English- and Spanish-speaking older adults. Psychology of Aging, 12(4), 718–725. Miller, B. L., & Cummings, J. L. (2007). The human frontal lobes, second edition: Functions and disorders. New York: The Guildford Press. Rabin, L. A., Barr, W. B., & Burton, L. A. (2005). Assessment practices of clinical neuropsychologists in the United States and Canada: A survey of INS, NAN, and APA Division 40 members. Archives of Clinical Neuropsychology, 20(1), 33–65. Ravizza, S. M., & Ciranni, M. A. (2002). Contributions of the prefrontal cortex and basal ganglia to set shifting. Journal of Cognitive Neuroscience, 14(3), 472–483.

Executive Functioning C ASEY R. S HANNON , C LAIRE T HOMAS -D UCKWITZ University of Northern Colorado Greeley, CO, USA

Synonyms Executive processes

Clinical Uses The EXAMINER will reliably and validly assess executive function for clinical investigations and clinical trials. The test battery will be adaptable across a wide range of ages and disorders.

Cross References ▶ Behavioral Assessment of Dysexecutive Syndrome (BADS) ▶ Cambridge Neuropsychological Test Automated Battery (CANTAB) ▶ Delis–Kaplan Executive Function System (D-KEFS) ▶ NEPSY-II

References and Readings Beglinger, L. J., Gaydos, B., Tangphao-Daniels, O., Duff, K., Kareken, D. A., Crawford, J., et al. (2005). Practice effects and the use of alternate forms in serial neuropsychological testing. Archives of Clinical Neuropsychology, 20(4), 517–529.

Definition Executive functioning refers to higher-level abilities in the following areas: planning, problem-solving, attention, mental flexibility, initiation, judgment, inhibition, and abstract reasoning. This set of abilities is most commonly associated with the frontal lobe region of the brain. It should be noted that executive functioning is not exclusive to the frontal lobes and is thought to extend to other regions of the brain.

Current Knowledge Most of the current knowledge surrounding executive functioning has resulted from studying individuals with frontal lobe lesions. Executive functioning process may be disrupted by traumatic brain injury, other cerebral lesions, substance abuse, and maternal substance abuse during pregnancy. These disruptions may be manifested in personality changes or adaptive functioning. For

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example, a person who experiences brain damage that impacts his or her executive processes may instantly, or over a period of time, begin to display symptomology that deviates from the ways he or she may have previously been characterized (e.g., cognitions, behaviors, emotions). The developmental trajectory spans through early adulthood.

References and Readings Dise-Lewis, J. E., Calvery, M. L., & Lews, H. C. (2002). BrainStars D’Amato, R. C., Fletcher-Janzen, E., & Reynolds, C. R. (2005). Handbook of school neuropsychology. Hoboken, New Jersey: Wiley. Jurado, M., & Rosselli, M. (2007). The elusive nature of executive functions: A review of our current understanding. Neuropsychological Review, 17, 213–233. Lezak, M. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

Executive Interview D ONALD R OYALL The University of Texas Health Center at San Antonio San Antonio, TX, USA

Synonyms EXIT; EXIT15; EXIT-25; TEXAS

Description The Executive Interview (EXIT25) (Royall, Mahurin, & Gray, 1992) provides a brief, clinical-based, bedside measure of executive control functions (ECF). EXIT25 scores have been used to identify the presence and severity of executive impairments and to predict problems in selfcare, functional status, decision-making capacity, and problem behavior. The EXIT25 has been validated mainly in elderly persons but has also been used in a wide range of other populations, including medical patients, psychiatric patients, community samples, and as an outcome measure in clinical trials. It is available in many languages including English and Spanish, but published validation studies exist only for Dutch, Brazilian Portuguese, and Cantonese Chinese translations. The EXIT25 is a compilation of 25 items that target motor, behavioral, and cognitive features associated with frontal systems dysfunction. Items include verbal and design fluency, an aural Trail Making task, repetition of

anomalous sentences (e.g., ‘‘Mary fed a little lamb’’), a stroop-like interference task, a go/no-go task, Luria hand sequences, etc. Additionally, neurological procedures are incorporated (e.g., to elicit paratonia, grasp reflex, snout reflex, and echopraxia). Thus, the EXIT25 provides an opportunity for the examiner to document a wide range of frontal-type clinical features, including perseveration, disinhibition, motor impersistence, and intrusions. Moreover, the ‘‘interview’’ is choreographed to facilitate the examiner–testee interaction, and to support test–retest and inter-rater reliability. Shortened versions (e.g., the EXIT15 and the Telephone Executive Assessment Scale [TEXAS]) have been developed for specific applications but are less well studied. The EXIT25 takes 10–15 min to administer. The items are presented ‘‘in rapid succession and with minimal instruction, which allows little time for reflection and therefore may enhance any tendency of disinhibition or inappropriate responses’’ (Stokholm, Vogel, Gade, & Waldemar, 2005, p. 1578). Each item uses a three-point response scale: 0 (intact performance), 1 (a specific partial error or equivocal response), and 2 (specific incorrect response or failure to perform). Total score ranges from 0 to 50, with higher scores indicating greater impairment. A cut-off score of 15/16 is recommended. In a brief, practical bedside format, the EXIT25 provides a reliable, sensitive, discriminating, and valid measure of ECF. The EXIT25 has particular utility for the assessment of functional status and potential as a measure of decision-making capacity. A notable feature of the EXIT25 is that it can demonstrate executive impairments across a broad spectrum of disorders and across a broad range of severity within those conditions. Traditional neuropsychological examinations, however, may be required to assess impairments in specific executive domains. The EXIT25 is not well suited for the discrimination between dementing disorders, as executive impairments are likely to be present in any dementing illness that has evolved to a clinically disabling point in its natural history. However, when used in combination with nonexecutive measures, accurate discriminations are possible on the basis of the pattern of test scores that emerges (Royall, 2002). The EXIT25’s ability to detect comparably severe executive deficits in ‘‘non-demented’’ medical patients without significant memory or global cognitive impairment poses a challenge to dementia case finding. A general description of items comprising the EXIT25 is contained in Royall et al. (1992). The test itself is available from Dr. Royall. It was recently reviewed by Tate (2010).

Executive Interview

Historical Background Development of the EXIT25 started with a set of 50 items, generated from review of the literature and the author’s clinical experience. Items were selected for acceptability to the examinee, suitability for bedside testing, and unambiguous scoring. During a process of pilot testing, item content was refined by excluding those with low clinical utility and poor intercorrelation. The final set of 25 items has a fixed order of presentation and standardized administration procedures.

Psychometric Data Royall et al. (1992) standardized the EXIT25 on a sample of 40 elderly retirees (descriptive data below), who were recruited from 537 residents of a single comprehensive care retirement community (CCRC) in San Antonio, Texas, USA. The community had four levels of care: (1) retirement apartments without services, (2) domestic services provided, (3) intermediate care nursing units, and (4) ‘‘Alzheimer’s Care Units’’. Levels 1 and 2 were designated by the authors as ‘‘noninstitutionalized’’; Levels 3 and 4 as ‘‘institutionalized.’’ Ten subjects were selected from each level and interviewed blind to their level of care. Validation instruments included the MiniMental State Examination (MMSE), TMT, Wisconsin Card Sorting Test (WCST), Serial Attention Test (SAT), and Nursing Home Behavior Problem Scale (NHBPS). Cognitive testing was performed blind to EXIT25 scores. No significant correlation coefficients were found between EXIT25 scores and age, sex, or education. Interrater reliability was examined in an independent sample (n = 30) from a range of accommodation levels who were tested independently by two physicians. Cut-off scores were explored in the standardization sample, and were provisionally set at 15/16, below which no abnormal score on the NHBPS was found. In a subsequent publication, Royall, Rauch, Romań, Cordes, and Polk (2001) cite data from a sample of 200 people, in which a receiver operating characteristic (ROC) analysis yielded an area under the curve of 0.93 (sensitivity 93%; specificity 83%) for the discrimination of institutionalized and noninstitutionalized subjects. In a study involving the Chinese translation, Chan, Chiu and Lam (2006) confirm an ROC of c = 0.986 for the discrimination between all-cause dementia and controls. The best discrimination was again obtained at the 15/16 cutoff. Stokholm et al. (2005) have confirmed significant associations between the EXIT25s and traditional executive

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measures, including the Stroop Test and verbal fluency. Chan et al. (2006) report significant correlations between the EXIT25 and ‘‘Category Number,’’ ‘‘Failure to Maintain Set,’’ ‘‘Total Errors,’’ ‘‘Perseverative errors,’’ and ‘‘NonPerseverative Errors’’ from a Chinese translation of the WCST. Discriminant validity was assessed by Royall, Cabello and Polk (1998). They tested the EXIT25’s ability to discriminate among residents at different levels of care among 107 older retirees (mean age = 83.7 7.2 years), including 17 community-dwelling, well, older controls and 90 CCRC residents. Sixty-one subjects resided at a noninstitutionalized level of care and 46 were institutionalized. The EXIT25 was significantly associated with the level of care, independently of medication usage, depression ratings, and problem behavior. Together, these three variables accounted for 69% of the total variance in the level of care (R2 = 0.69; F [df 7.99] = 32.1, p < .001). In a subsequent study (Royall, Chiodo, & Polk, 2005) in a larger sample (n = 193) from a second retirement community, EXIT25 scores contributed significantly to an algorithm for the detection of residents in need of an ‘‘assisted living’’ vs. independent living levels of care. Royall et al. (2001) have examined the association between magnetic resonance imaging (MRI) and EXIT25 scores in a convenience sample of 52 older people recruited from a dementia assessment clinic. EXIT25 scores were associated with ratings of lesion severity performed by a neuroradiologist (blinded to EXIT25 scores). EXIT25 scores were significantly associated with left frontal (p < .002), left medial (p < .03), right frontal (p < .02), and right medial (p < .02) cortical lesions by MRI, independently of age and MMSE scores. The EXIT25’s associations with right hemisphere lesions did not persist after adjusting for left frontal lesions. Left posterior lesions did not significantly affect the EXIT25. Similarly, left frontal circuit pathology worsened EXIT25 scores (p < .05). Pathology in left anterior subcortical structures showed a trend (p = .052). EXIT25 scores were neither affected by right subcortical pathology nor by pathology in either hippocampus. Kochunov et al. (2009) have explored the relationship between structural neuroimaging-based indices of cerebral integrity and EXIT25 scores across the life span. In aging adults, higher EXIT25 scores were associated with atrophic changes in cerebral white matter (WM) (sulcal and intergyral span) primarily in the superior frontal and anterior cingulate regions. Sixty-two percent of the variance in EXIT25 scores could be explained by variability in the structural indices from those two regions.

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Evidence for the ecological validity of the EXIT25 is provided by the authors and independent research groups. The EXIT25 is significantly associated with problem behaviors as measured by the Frontal Behavioral Inventory (FBI) (Stokholm et al., 2005) and strongly associated with resistiveness in demented elderly (r = 0.73) (Stewart, Gonzalez-Perez, Zhu, & Robinson, 1999) as well as with NHBPS scores (r = 0.79) in its original standardization sample. Apathy is unrelated to EXIT25 scores in depressed elderly patients (Marin, Butters, Mulsant, Pollock, & Reynolds, 2003), while insight is unrelated to EXIT25 scores in schizophrenia (Hwang, Lee, & Cho, 2009). This suggests that the EXIT25 may be specifically associated with some ‘‘frontal’’ behaviors more than others. Similarly, the EXIT25 loads most strongly on a psychometric factor that is co-labeled by verbal fluency, digit symbol substitution, and an executive clock-drawing task, but neither WCST indices nor TMT, each of which labels orthogonal ECF factors (Royall, Chiodo, & Polk, 2003). EXIT25 scores are stable over time. The annual rate of change in CCRC residents is estimated at EXIT25: 0.89 points (SE = 0.16) (Royall et al., 2004). There are no practice effects (Royall et al., 2005). O’Shaughnessy et al. (2005) have used the EXIT25 in a placebo-controlled clinical trial of erythropoetin for chemotherapy-related cognitive impairment. After 4 weeks of therapy, the mean change in EXIT25 scores in the placebo group was only 0.3 2.4.

Clinical Uses The EXIT25 was originally validated in geriatric samples. It has been widely used in elderly patients with dementia, and normative data is available well into the eighth decade. It has also been used in younger patients with a wide variety of conditions (reviewed in Royall et al., 2002), including head injury (Dreer, DeVivo, Novack, Krzywanski, & Marson, 2008; Larson, Leahy, Duff, & Wilde, 2008), HIV dementia, frontotemporal dementia, and mental disorders including schizophrenia (Hwang et al., 2009) and bipolar illness (BPI) (Gildengers et al., 2004; Altshuler et al., 2007). In schizophrenia, EXIT25 scores are significantly associated with the level of care, the medication adherence, and the risk of extrapyramidal movement disorders after neuroleptic exposure. In patients with BPI, the EXIT25 is associated with employment status (Altshuler et al., 2007) and with suicide attempts in depressed elderly patients (Dombrovski et al., 2008). The TEXAS, a five-item telephone version,

predicts relapse risk in recently discharged BPI patients (Bauer, McBride, Shea, Gavin, & Fogel, 1994). Dementia screening: Chan et al. (2006) report and ROC of c = 0.986 for the EXIT25’s discrimination between all-cause dementia and controls. More difficult discriminations are those between normal controls and ‘‘Mild Cognitive Impairment’’ (MCI), or between MCI and dementia. However, the EXIT25 discriminates MCI patients from both normal controls and demented patients (Periera, Yassuda, Oliveira, & Forlenza, 2008) and among subjects at each dementia stage, as assessed by the Clinical Dementia Rating (CDR) scale (Chan et al., 2006). Outside of Alzheimer’s disease (AD), the EXIT25 also distinguishes non-demented patients with Parkinson’s disease (PD) from both normal controls and demented PD patients (Martin et al., 2008) and between ‘‘prodromal’’ ischemic vascular disease and vascular dementia (Ramos-Este´banez et al., 2008). The EXIT25 can distinguish different levels of care among non-demented subjects, as well as predict their use of prostheses and Instrumental Activities of Daily Living (IADL) impairment (Periera et al., 2008). In contrast, Larson et al. (2008) report a ceiling effect in mild traumatic brain injury (TBI). However, this study was performed among the subset of referrals previously selected for rehabilitation, which may exert a positive bias on EXIT25 scores. The EXIT25 has also been used successfully in patients with HIV and frontotemporal dementia (FTD). Moreover, there is a high prevalence of unrecognized executive impairment among medical patients with normal MMSE scores (Schillerstrom, Deuter, Wyatt, Stern, & Royall, 2003;2005 Schillerstrom et al., ; Fuller et al., 2008). Such cases are demonstrably impaired in their functional status and decision-making abilities and are not merely ‘‘preclinical’’ MCI cases. This is particularly true in the presence of vascular risk factors (DeCoteau et al., 2006; Thabit et al., 2009). Because their memory performance is generally normal, such cases are not currently diagnosable with ‘‘dementia,’’ at least according to the Diagnostic and Statistical Manual (DSM). However, they are not adequately described as MCI either, because of the strong and specific association between executive function, as measured by the EXIT25 and functional status (Royall, 2006). Royall has suggested that this issue can be reconciled by the recognition of a second dementia syndrome, dominated by executive impairment (Royall & Polk, 1998; Royall, 2000). This proposal would effectively make executive impairment the ‘‘essential’’ feature of dementia, while impairments in memory and other specific cognitive domains would be relegated to the status of optional

Executive Interview

features (Royall, 2006). This approach is supported by the failure of several authors to distinguish between dementing illnesses on the basis of their EXIT25 scores. When disorders are matched to functional status, EXIT25 scores are effectively matched as well (Royall et al., 1993). Functional status assessment: In its review of the cognitive correlates of functional status, the Research Committee of the American Neuropsychiatric Association identified 12 published multivariate regression models, comprising 813 unique subjects, in which the EXIT25 was used as a predictor of a functional outcome measure. The mean partial R2 reported in these models was R = 0.24 (Royall et al., 2007). The EXIT25 is a significant predictor of IADL in both cross-sectional and longitudinal. It is strongly associated with performance-based functional assessments, e.g., the Cognitive Competency Test (CCT) (partial R = 0.40) (DeCoteau et al., 2006) and the Direct Assessment of Functional Status (DAFS-R) (r = 0.872, p < 0.001). The latter effect is independent of age, gender, education, and Cambridge Cognitive Test (CAMCOG) performance (Pereira et al., 2008). At its recommended cut-point is of 15/50, the EXIT25 perfectly predicts the capacity of older patients with chronic obstructive pulmonary disease (COPD) to learn to use an inhaler correctly (Allen, Jain, Ragab, & Malik, 2003). The EXIT25 is less strongly associated with Basic Activities of Daily Living (BADL). The EXIT25 is significantly associated with level of care in elderly populations, adherence to medications and safe sexual practices in human immunodeficiency virus (HIV) infected patients. EXIT25 scores are inversely correlated with self-care activities in diabetic patients (Thabit et al., 2009) and are significantly associated with employment status in younger adults with BPI, independently of lifetime psychiatric hospitalizations and number of psychotropic medications (Altshuler et al., 2007). Capacity assessment: The EXIT25’s associations with capacity assessments have been reviewed elsewhere (Royall et al., 2007). EXIT25 scores predict financial capacity. As measured by the Capacity to Consent to Treatment Instrument (CCTI) (r = 0.53–0.75). EXIT25 was the strongest predictor of the ‘‘understanding treatment’’ standard (R2 = 0.56) and the only significant predictor of a ‘‘rational reasons’’ standard (R2 = 0.45). In a followup study of patients with traumatic brain injury, the baseline EXIT25 was significantly associated with recovery of capacity after six months (Dreer et al., 2008). The EXIT25 is significantly associated with medical decision-making and the capacity to manage one’s affairs. The EXIT25 correlates significantly with each MacArthur Competency Assessment Tool-Treatment (MacCAT-T)

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decision-making capacity domain, i.e., ‘‘Understanding,’’ ‘‘Appreciation,’’ and ‘‘Reasoning’’ (Schillerstrom, Rickenbacker, Joshi, & Royall, 2007). It is significantly correlated with the Hopkins Competency Assessment Test (HCAT), independently of MMSE scores, age, education, or number of prescribed medications. The best EXIT25 score for the discrimination of subjects who have lost their HCAT rated capacity to make an advance directive was 24/50 (c = 0.95). However, the EXIT25 out-performs the HCAT as a screen for the capacity to consent to inpatient psychiatric treatment. Among Adult Protective Services (APS) referrals, the EXIT25 is strongly related to autonomy, as measured by the Kohlman Evaluation of Living Skills (KELS) (r = .705) (Burnett, Dyer, & Naik, 2009). Epidemiological applications: Royall et al. have published extensively on the EXIT25 among CCRC residents. In addition, the EXIT25 has been used to demonstrate a high prevalence of executive impairment among large community samples of patients with schizophrenia and bipolar disorder. However, the EXIT25 may be too long for more widespread acceptance in an epidemiological context. On the other hand, a 15-item version of the EXIT (EXIT15) has been employed in the Health, Aging and Body Composition (Health ABC) study, a large community survey (n = 2,349 older subjects). In that study, the EXIT15 was significantly associated with longitudinal changes in gait speed, after adjusting for baseline speed (Atkinson et al., 2007). The TEXAS may be suitable for telephone screening of community samples. It correlates r = 0.88 with the EXIT25. At a threshold of 3/15, the TEXAS has sensitivity = 1, specificity = 0.88 vs. EXIT25 scores >10/50 (Bauer et al., 1994). Clinical trials: Because of its brevity, test–retest reliability, sensitivity to change, and strong association with functional status, the EXIT25 has been used in several clinical trials (Auchus et al., 2007; Dichgans et al., 2008; O’Shaughnessy et al., 2008; Boxer et al., 2009). Cholinesterase inhibitors appear to have modest effects on EXIT25 scores in subcortical infarcts and leukoencephalopathy (CADASIL) (Dichgans et al., 2008) but not in vascular dementia (Auchus et al., 2007; Romań, Salloway, Black, Royall, & DeCarli, in press). In contrast, sertraline may improve EXIT25 scores in vascular cases (Royall et al., 2009).

Cross References ▶ Executive Functioning

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References and Readings Allen, S. C., Jain, M., Ragab, S., & Malik, N. (2003). Acquisition and short-term retention of inhaler techniques require intact executive function in elderly subjects. Age Ageing, 32, 299–302. Altshuler, L., Tekell, J., Biswas, K., Kilbourne, A. M., Evans, D., Tang, D., et al. (2007). Executive function and employment status among veterans with bipolar disorder. Psychiatric Services, 58, 1441–1447. Atkinson, H. H., Rosano, C., Simonsick, E. M., Williamson, J. D., Davis, C., Ambrosius, W. T., et al. (2007). Cognitive function, gait speed decline, and comorbidities: The health, aging and body composition study. Journals of Gerontology. Series A, Biological Sciences and Medical Sciences, 62, 844–8450. Auchus, A. P., Brashear, H. R., Salloway, S., Korczyn, A. D., De Deyn, P. P., & Gassmann-Mayer, C. (2007). GAL-INT-26 study group Galantamine treatment of vascular dementia: A randomized trial. Neurology, 69, 448–458. Bauer, M., McBride, L., Shea, N., Gavin, C., & Fogel, B. (1994). Screening for executive cognitive dysfunction in bipolar disorder. Biological Psychiatry, 35, 636. Boxer, A. L., Lipton, A. M., Womack, K., Merrilees, J., Neuhaus, J., Pavlic, D., et al. (2009). An open-label study of memantine treatment in 3 subtypes of frontotemporal lobar degeneration. Alzheimer Disease and Associated Disorders, 23, 211–217. Burnett, J., Dyer, C. B., & Naik, A. D. (2009). Convergent validation of the Kohlman evaluation of living skills as a screening tool of older adults’ ability to live safely and independently in the community. Archives of Physical Medicine and Rehabilitation, 90, 1948–1952. Chan, S. M., Chiu, F. K., & Lam, C. W. (2006). Correlational study of the Chinese version of the executive interview (C-EXIT25) to other cognitive measures in a psychogeriatric population in Hong Kong Chinese. International Journal of Geriatric Psychiatry, 21, 535–541. DeCoteau, W. E., Selk, B., Stirling, G., Fentiman, P., Savage, C., & Landeryou, J. (2006). Dementia due to hypertensive microvascular disease of the brain often goes unrecognized because the patient is not tested, or the wrong tests are done. The Journal of Clinical Hypertension, 8(Suppl. A No. 5), 36. Dichgans, M., Markus, H. S., Salloway, S., Verkkoniemi, A., Moline, M., Wang, Q., et al. (2008). Donepezil in patients with subcortical vascular cognitive impairment: A randomised double-blind trial in CADASIL. Lancet Neurology, 7, 310–318. Dombrovski, A. Y., Butters, M. A., Reynolds, C. F. 3rd, Houck, P. R., Clark, L., Mazumdar, S., et al. (2008). Cognitive performance in suicidal depressed elderly: Preliminary report. American Journal of Geriatric Psychiatry, 16, 109–115. Dreer, L. E., DeVivo, M. J., Novack, T. A., Krzywanski, S., & Marson, D. C. (2008). Cognitive predictors of medical decision-making capacity in traumatic brain injury. Rehabilitation Psychology, 53, 486–497. Fuller, C. D., Schillerstrom, J. E., Jones, W. E. 3rd, Boersma, M., Royall, D. R., & Fuss, M. (2008). Prospective evaluation of pretreatment executive cognitive impairment and depression in patients referred for radiotherapy. Journal of Radiation Oncology, Biology, Physics, 72, 529–533. Gildengers, A. G., Butters, M. A., Seligman, K., McShea, M., Miller, M. D., Mulsant, B. H., et al. (2004). Cognitive functioning in late-life bipolar disorder. American Journal of Psychiatry, 161, 736–738. Hwang, S. S., Lee, J. Y., Cho, S. J., et al. (2009). The model of the relationships among the predictors of quality of life in chronic

stage of schizophrenia. Progress in Neuropsychopharmacology & Biological Psychiatry, 33, 1113–1118. Kochunov, P., Robin, D. A., Royall, D. R., Coyle, T., Lancaster, J., Kochunov, V., et al. (2009). Can structural MRI indices of cerebral integrity track cognitive trends in executive control function during normal maturation and adulthood? Human Brain Mapping, 30, 2581–2594. Larson, E. B., Leahy, B., Duff, K. M., & Wilde, M. C. (2008). Assessing executive functions in traumatic brain injury: An exploratory study of the executive interview. Perceptual and Motor Skills, 106, 725–736. Marin, R. S., Butters, M. A., Mulsant, B. H., Pollock, B. G., & Reynolds, C. F. 3rd. (2003). Apathy and executive function in depressed elderly. Journal of Geriatric Psychiatry and Neurology, 16, 112–116. Martin, R. C., Okonkwo, O. C., Hill, J., et al. (2008). Medical decisionmaking capacity in cognitively impaired Parkinson’s disease patients without dementia. Movement Disorders, 23, 1867–1874. O’Shaughnessy, J. A., Vukelja, S. J., Holmes, F. A., Savin, M., Jones, M., Royall, D., et al. (2005). Feasibility of quantifying the effects of epoetin alfa therapy on cognitive function in women with breast cancer undergoing adjuvant or neoadjuvant chemotherapy. Clinical Breast Cancer, 5, 439–446. Osorio, R., Garcıá de Lo´zar, B., Ramos, I., & Aguëra, L. (2009). Executive function in patients with late onset depression. Actas Espanõlas De Psiquiatrıá, 37, 196–199. Pereira, F. S., Yassuda, M. S., Oliveira, A. M., & Forlenza, O. V. (2008). Executive dysfunction correlates with impaired functional status in older adults with varying degrees of cognitive impairment. International Psychogeriatrics, 1–12, 20, 1104–1115.1–12, Ramos-Este´banez, C., Moral-Arce, I., Munõz-Arrondo, R., Gonza´lezMandly, A., Matorras-Galań, P., Gonza´lez-Macias, J., et al. (2008). Vascular cognitive impairment: Prodromal stages of ischemic vascular dementia. Dementia and Geriatric Cognitive Disorders, 25, 451–460. Romań, G. C., Salloway, S., Black, S. E., Royall, D. R., & DeCarli, C. (2010). A randomized, placebo-controlled clinical trial of donepezil in vascular dementia: Differential effects by hippocampal size. Stroke, (in press). Royall, D. R. (2000). Executive cognitive impairment: A novel perspective on dementia. Neuroepidemiology, 19, 293–299. Royall, D. R. (2002). Bedside assessment of vascular dementia. In S. Gauthier & T. Erkinjuntti (Eds.), Vascular cognitive impairment (Chap. 18., pp. 307–322) London: Martin Dunitz Ltd. Royall, D. R. (2006). Mild cognitive impairment and functional status. Journal of the American Geriatrics Society, 54, 163–165. Royall, D. R., Cabello, M., & Polk, M. J. (1998). Executive dyscontrol: An important factor affecting the level of care received by older retirees. Journal of the American Geriatrics Society, 46, 1519–1524. Royall, D. R., Chiodo, L. K., & Polk, M. (2003). Executive dyscontrol in normal aging: Normative data, factor structure, and clinical correlates. In J. C. M. Brust & S. Fahn (Eds.), Current neurology and neuroscience reports (Vol. 3(6), pp. 487–493). Philadelphia: Current Science Inc. Royall, D. R., Chiodo, L. K. & Polk, M. J. (2005). An empiric approach to level of care determinations: the importance of executive measures. Journals of Gerontology, 60A, 1059–1064. Royall, D. R., Lauterbach, E. C., Cummings, J. L., Reeve, A., Rummans, T. A., Kaufer, D. I., et al. (2002). Executive control function: A review of its promise and challenges to clinical research. Journal of Neuropsychiatry and Clinical Neurosciences, 14, 377–405. Royall, D. R., Lauterbach, E. C., Kaufer, D. I., Malloy, P., Coburn, K. L., & Black, K. J. (2007). The cognitive correlates of functional status: A

Expanded Disability Status Scale review from the committee on research of the American Neuropsychiatric Association. Journal of Neuropsychiatry and Clinical Neurosciences, 19, 249–265. Royall, D. R., Mahurin, R. K., & Gray, K. F. (1992). Beside assessment of the executive cognitive impairment: The executive interview. Journal of the American Geriatrics Society, 40, 1221–1226. Royall, D. R., Mahurin, R. K., True, J., Anderson, B., Brock, P. I., Freeburger, L., et al. (1993). Executive impairment among the functionally dependent: Comparisons between schizophrenic and elderly subjects. American Journal of Psychiatry, 150, 1813–1819. Royall, D. R., & Polk, M. J. (1998). Dementias that present with and without posterior cortical features: An important clinical distinction. Journal of the American Geriatrics Society, 46, 98–105. Royall, D. R., Rauch, R., Romań, G. C., Cordes, J. A., & Polk, M. J. (2001). Frontal MRI findings associated with impairment on the executive interview (EXIT25). Experimental Aging Research, 27, 293–308. Royall, D. R., Romań, G. C., Cordes, J. A., Velez, A., Edwards, A., & Schillerstrom, J. S. (2009). Sertraline improves executive function in patients with vascular cognitive impairment. Journal of Neuropsychiatry and Clinical Neurosciences, 21, 445–454. Schillerstrom, J. E., Deuter, M. S., Wyatt, R., Stern, S. L., & Royall, D. R. (2003). Prevalence of executive impairment in patients seen by a psychiatry consultation service. Psychosomatics, 44, 290–297. Schillerstrom, J. E., Horton, M. S., Schillerstrom, T. L., Joshi. K. G., Earthman, B. S., Velez, A. M., et al. (2005). Prevalence, course and risk factors for executive impairment in patients hospitalised on general medicine service. Psychosomatics, 46, 411–417. Schillerstrom, J. E., Rickenbacker, D., Joshi, K. G., & Royall. D. R. (2007). Executive function and capacity to consent to a noninvasive research protocol. American Journal of Geriatric Psychiatry, 15, 159–162. Stewart, J. T., Gonzalez-Perez, E., Zhu. Y., & Robinson, B. E. (1999). Cognitive predictors of resistiveness in dementia patients. American Journal of Geriatric Psychiatry, 7, 259–263. Stokholm, J., Vogel, A., Gade, A., & Waldemar, G. (2005). The executive interview as a screening test for executive dysfunction in patients with mild dementia. American Journal of Geriatric Psychiatry, 53, 1577–1581. Tate, R. L. (2010). A compendium of tests, scales, and questionnaires: The practitioner’s guide to measuring outcomes after acquired brain impairment. Hove, UK: Psychology Press. Thabit, H., Kennelly, S. M., Bhagarva, A., Ogunlewe, M., McCormack, P. M., McDermott, J. H., et al. (2009). Utilization of frontal assessment battery and executive interview 25 in assessing for dysexecutive syndrome and its association with diabetes self-care in elderly patients with type 2 diabetes mellitus. Diabetes Research and Clinical Practice, 86, 208–212.

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Exhaustion ▶ Fatigue

EXIT ▶ Executive Interview

EXIT15 ▶ Executive Interview

EXIT-25 ▶ Executive Interview

Expanded Disability Status Scale TAMARA B USHNIK Rusk Institute for Rehabilitation Medicine – NYU Langone Medical Center New York, NY, USA

Synonyms EDSS; Kurtzke expanded disability status scale

Description

Executive Processes ▶ Executive Functioning

Exencephaly ▶ Anencephaly

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The Expanded Disability Status Scale (EDSS) is based upon the original Kurtzke Disability Status Scale (DSS) and Functional Systems (FS) (Kurtzke, 1961). There are eight FSs: pyramidal, cerebellar, brainstem, sensory, visual, bowel and bladder, cerebral, and other. Following neurological examination, each FS is rated on a scale of 0–5 (cerebellar and brainstem), 0–1 (other), and 0–6 (all others). These ratings assist in assigning an EDSS score

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which ranges from 0 to 10 in 0.5 increments, where 0 indicates a normal neurological examination (but cerebral grade 1 allowed) and 10 corresponds to death due to multiple sclerosis (MS) (see Kurtzke, 1983 for a full description of the rating scale). In general, EDSS scores ranging from 1.0 to 4.5 quantify the disability/impairment experienced by individuals with MS who are ambulatory, while EDSS scores from 5.0 to 9.5 correspond to disability/impairment of individuals who have some impairment of ambulation. For example, an EDSS of 3.5 describes an individual who is fully ambulatory but has moderate disability in one FS and more than minimal disability in several other FSs, while an EDSS of 8.5 corresponds to an individual who is restricted to bed for most of the day but has some use of the arms and is able to perform some self-care functions.

Psychometric Data Reliability: Inter- and intra-rater reliabilities are dependent upon the definition of ‘‘agreement’’ that is used. When agreement between raters or after an interval is defined as a difference no greater than 1.0 point, inter-rater agreement was 96% (Sharrack, Hughes, Soudain, & Dunn, 1999); however it fell to 89% if the difference was defined as no greater than 0.5 point. Validity: The EDSS has high correlations with the FIM, Scripps Neurological Rating Scale, and patient’s self-assessment of disability (Sharrack et al., 1999). It has also shown high correlations with the Barthel Index but not the physical and mental component scores of the SF36, the London Handicap Scale, and the General Health Questionnaire (Hobart, Freeman, & Thompson, 2000); this indicates that it can discriminate overall disability from related health constructs.

Historical Background The development of the DSS and FS, upon which the EDSS is based, occurred in the early 1960s, when the neurological examinations of 762 men who had been diagnosed with MS while in the army were systematically reviewed (Kurtske, 1961). The examinations spanned approximately the first 20 years of the course of the disease. A team of neurologists agreed as to the primary diagnosis of MS for 527 of the sample, while 146 were classified as ‘‘definitely not’’ MS (Nagler et al., 1966). These two groups were examined over time for the development of MS-specific signs and symptoms which served to validate the DSS and FS categories (Beebe, Kurtzke, Kurland, Auth, & Nagler, 1967). The EDSS is one of the most widely used impairment measures for clinical trials in multiple sclerosis (MS). The EDSS has been criticized in that it is not a linear, ordinal assessment tool nor is it an equal-interval or ratio scale; thus parametric statistics and stepwise comparisons are not appropriate. In addition, the average time that an individual spends at each level of the scale is highly variable and tends to be longer at the two extremes of the scale (Weinshecker et al., 1989). Some recent trials have used a measure of treatment failure (TF) and appropriate nonparametric statistics, examining the proportion of patients presented TF and/ or time to TF. Since there is nonequivalence of intervals, it has been suggested that the TF should be taken as a worsening of 1.0 point for individuals with a baseline EDSS of 5.5 or less, while at a baseline EDSS of 5.5 or greater, TF should be considered a worsening of 0.5 point (Amato and Ponziani, 1999).

Clinical Uses The EDSS was not sensitive to clinical change over a 9-month follow-up period in 25 individuals with MS who reported changes in symptom and function (Sharrack et al., 1999). It was also unresponsive in a cohort of 64 individuals with moderate to severe disability (Hobart, Lamping, Freeman, & Thompson, 1996) and a group of 137 individuals with clinically definite MS (Hobart et al., 2000). In addition, the EDSS had limited variability in scores in a sample of 137 individuals when compared to the FIM and Barthel Index (Hobart et al., 2000). It is the contention of multiple authors that the EDSS is based on sound clinical intuition, but does not measure up to psychometric evaluation (Hobart et al., 2000; Sharrack et al., 1999; Thompson and Hobart, 1996).

Cross References ▶ Barthel Index ▶ Functional Assessment Measure ▶ SF-36/SF-12

References and Readings Amato, M. P., & Ponziani, G. (1999). Quantification of impairment in MS: discussion of the scales in use. Multiple Sclerosis, 5(4), 216–219.

Expert Witness Beebe, G. W., Kurtzke, J. F., Kurland, L. T., Auth, T. L., & Nagler, B. (1967). Studies on the natural history of multiple sclerosis. 3. Epidemiologic analysis of the Army experience in World War II. Neurology, 17, 1–7. Hobart, J. C., Lamping, D. L., Freeman, J. A., & Thompson, A. J. (1996). Reliability, validity and responsiveness of the Kurtzke expanded disability status scale (EDSS) in multiple sclerosis (MS) patients. European Journal of Neurology, 3(Suppl 4), 13. Hobart, J., Freeman, J., & Thompson, A. (2000). Kurske scales revisited: the application of psychometric methods to clinical intuition. Brain, 123, 1027–1040. Kurtske, J. F. (1961). On the evaluation of disability in multiple sclerosis. Neurology, 11, 686–694. Kurtzke, J. F. (1983). Rating neurological impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology, 33, 1444–1452. Nagler, B., Beebe, G. W., Kurtzke, J. F., Kurland, L. T., Auth, T. L., & Nefzger, M. D. (1966). Studies on the natural history of multiple sclerosis. 1. Design and diagnosis. Acta Neurol Scand 42(Suppl 19), 141–156. Sharrack, B., Hughes, R. A. C., Soudain, S., & Dunn, G. (1999). The psychometric properties of clinical rating scales used in multiple sclerosis. Brain, 122, 141–159. Weinshecker, B. G., Bass, B., Rice, G. P., Noseworthy, J., Carriere, W., Baskerville, J., & Ebers, G. C. (1989). The natural history of multiple sclerosis: a geographically based study. I. Clinical course and disability. Brain, 112, 133–146.

Expert v. Treater Role R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition Often, psychologists may begin a case as a treater in a clinical setting and then they are asked to serve in a forensic role. A particular conflict arises when this occurs and the psychologist must be careful not to occupy dual roles. Indeed, there is a clear distinction between the role of a treater and an expert. In clinical settings, a psychologist embodies the treater role and is an advocate for the patient. Conversely, the expert role requires full objectivity at all times and the expert is an advocate of the facts which could be potentially damaging to the plaintiff ’s (e.g., their patient) case. The APA Ethical Principles (2002) strongly advise against performing ‘‘multiple and potentially conflicting roles in forensic matters.’’ In order to protect against serving multiple roles, forensic psychologists should demand to only fulfill one of three possible roles: treater, trial consultant, or

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testifying expert. Greenberg and Shuman (1997) provide an extensive overview of the problems associated with treaters who are asked to also serve as an expert witness. Most notably, if a psychologist accepts a role as an expert witness, then his/her patient is no longer the client; instead, it is the attorney who becomes the client. Shuman, Greenberg, Heilbrun, and Foote (1998) argue that treaters should be prohibited from testifying as expert witnesses and that attorneys for both the plaintiff and the defense should be required to obtain their own independent examiners.

Cross References ▶ Expert Witness ▶ Independent Neuropsychological Examination

References and Readings American Psychological Association (2002). Ethical principles of psychologists and code of conduct. American Psychologist, 57, 1060–1073. Committee on Ethical Guidelines for Forensic Psychologists (1991). Specialty guidelines for forensic psychologists. Law and Human Behavior, 15, 655–665. Greenberg, S. A., & Shuman, D. W. (1997). Irreconcilable conflict between therapeutic and forensic roles. Professional Psychology: Research and Practice, 28, 50–57. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney Neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Shuman, D. W., Greenberg, S., Heilbrun, K., & Foote, W. E. (1998). An immodest proposal: Should treating mental health professionals be barred from testifying about their patients? Behavioral Sciences and the Law, 16, 509–523.

Expert Witness M OIRA C. D UX University of Maryland Medical Center/Baltimore VA Baltimore, MD, USA

Definition First and foremost, one must distinguish between a ‘‘fact witness’’ and an ‘‘expert witness.’’ A fact witness only provides testimony regarding direct knowledge of the

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facts related to the issue at hand (e.g., an ‘‘eye witness’’ account of things). In contrast, an expert witness can provide facts, but can also offer opinions and report hearsay. Since the decision rendered in Jenkins v. United States (1962), psychologists have been allowed to offer expert witness testimony in civil and criminal proceedings. Still, the admissibility of expert witness testimony is a critical issue. This determination is guided by a series of rules from the Federal Rules of Evidence Article (FRE) VII (‘‘Opinions & Expert Testimony’’). Specifically, FRE rule 702 states, ‘‘If scientific, technical or other specialized knowledge will assist the trier of fact to understand the evidence or to determine a fact in issue, a witness qualified as an expert by knowledge, skill, experience, training, or education, may testify thereto in the form of an opinion or otherwise.’’ In the case of Frye v. United States (1923), the Frye standard was established, which stated that: only scientific methods and concepts with ‘‘general acceptance’’ within a particular field are admissible. In the more recent case of Daubert v. Merrell Dow (1993), it was determined that scientific testimony has to abide by two criteria: the testimony must be (a) scientifically valid and (b) relevant to the case at hand. Once an expert witness has been identified, he/she is subject to discovery as well as direct and cross-examinations. During the first phase of the direct examination, the opposing attorney challenges the expert witness’ credentials and requests that the court voir dire him/her. This involves asking additional questions to assess the expert’s competency. After this phase, a trial judge decides whether the testimony of the expert witness is to be permitted. If allowed, direct examination by the retaining attorney reconvenes and cross-examination by the opposing attorney follows.

Frye v. U.S. (D.C. Cir. 1923). 293 F. 1013. Goldstein, A. M. (2003). Overview of forensic psychology. In A. Goldstein (Ed.), Handbook of Psychology (Vol 11). Forensic Psychology. New Jersey: John Wiley & Sons. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Jenkins v. U.S., 307 F. 2d 637 (1962).

Explicit Memory ▶ Declarative Memory ▶ Episodic Memory

Expressive Aphasia ▶ Broca’s Aphasia ▶ Nonfluent Aphasia

Expressive One-Word Picture Vocabulary Test DAVID M ICHALEC 1, N ATHAN H ENNINGER 2 1 Ohio State University, Nationwide Children’s Hospital Columbus, OH, USA 2 Ohio State University Columbus, OH, USA

Synonyms Cross References EOWPVT ▶ Cross-Examination ▶ Daubert v. Merrell Dow ▶ Direct Examination ▶ Discovery ▶ Expert v. Treater Role ▶ Federal Rules of Evidence ▶ Testifying Expert Versus Fact Witness

References and Readings Daubert v. Merrell Dow, 509 U.S. 579 (1993). Federal Rules of Evidence (1975).

Description The Expressive One-Word Picture Vocabulary Test (EOWPVT) is an individually administered instrument designed to evaluate a person’s knowledge of Englishspeaking vocabulary. The test is norm-referenced and designed to be used with individuals between the ages of 2 years, 0 months to 18 years, 11 months. During administration, the examiner shows the examinee a series of pictures depicting actions, objects, and concepts. The examinee is asked to name the illustration presented

Expressive One-Word Picture Vocabulary Test

and then the answer is recorded in the EOWPVT protocol. The test kit consists of 170 test plates in a spiral-bound easel. According to the manual, administration and scoring time takes between 15 to 20 min, depending on the individual. Raw scores are obtained which are then translated to standard scores, percentiles, and age equivalents.

Historical Background Measures of vocabulary have long held an important place in psychoeducational assessment. Research consistently demonstrates the effect that vocabulary has on academic achievement (Baker, Simmons, & Kameenui, 1998). The historical significance of vocabulary assessment has been noted as early as 1916 by Terman, who demonstrated that vocabulary is an excellent predictor of cognitive ability. Furthermore, identifying young children with language difficulties and monitoring speech rehabilitation are just some of the worthy roles that vocabulary tests, like the EOWPVT, have taken in clinical settings. The current edition of the EOWPVT (2000) is an update from the original publication in 1979 and its subsequent revision in 1990. In the newest edition, norms are improved, outdated items removed, clearer illustrations are in full color, and slight administration changes were made to reduce examinee confusion. It is often used in conjunction with its ‘‘sister’’ test, the Receptive One-Word Picture Vocabulary Test (ROWPVT), a measure of receptive vocabulary. Use of both measures results in a comprehensive assessment of receptive and expressive vocabulary.

Psychometric Data Internal consistency, test–retest reliability, and interrater reliability have all been evaluated for the EOWPVT. The manual reports acceptable coefficient alphas ranging from 0.93 to 0.98 with a median of 0.96. Split-half coefficients (corrected for the full length) are reported to have a median of 0.98. Two hundred and twenty-six examinees were retested to investigate test–retest reliability. A coefficient of 0.90 was found for the entire sample, providing evidence that the tool is suitably stable over time. Interrater reliability was investigated in three different ways and all of them demonstrated strong consistency among multiple examiners. The authors established content validity by using only those words that could obviously be illustrated. Early items for the test were collected through parent questionnaires that asked parents to indicate the first words spoken by their children while some obscure words

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were used to tap into superior vocabulary ability at the higher levels. The rest of the words were selected with reference to their frequency in written material and the grade level at which the words appear in curriculum materials. Criterion-related validity for the EOWPVT has been amply established between it and a host of other vocabulary tests. Finally, the authors have verified construct validity between their instrument and a variety of sources including cognitive ability, academic ability, previous editions of the test, and expressive and receptive vocabulary. Correlations range from .64 to .89 with these measures. The measure has been newly standardized in 1999 when it was administered to 3,661 people. From this pool, 2,327 were selected, at random, to meet demographic criteria for establishing norms. Overall, the sample closely mirrored the U.S. population in terms of region of residence, race/ethnicity, gender, disability status, residence (urban vs. rural), and parent educational attainment.

Clinical Uses Although the EOWPVT can be used for research purposes, its primary use is clinical in nature. Results from EOWPVT testing can help identify language impairment in preschool children, monitor growth over a period of time and/or treatment, or as a way to evaluate a person learning English as a second language. Other uses are for diagnosing aphasia, diagnosing reading difficulty, or as a secondary measure of cognitive ability. Versatile in its clinical use, the test is used in educational, hospital, clinical, early childhood, and rehabilitation settings. Some limitations must, of course, be noted. For one, the tool only assesses a fraction of one’s English vocabulary. As with all tests, results from the EOWPVT should not be used in isolation. A single test cannot confirm or deny the presence of a clinical diagnosis. Rather, it must be used in conjunction with historical information, other test results, and observations that coalesce to a diagnostic impression. Examiners should always be aware of individual factors like hearing, visual, or attentional problems that may lead to an invalid administration. Finally, the EOWPVT can only be used with native English speakers or those learning the English language.

Cross References ▶ Language ▶ Speech ▶ Speech/Communication Disabilities

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▶ Speech-Language Pathology ▶ Speech-Language Therapy ▶ Vocabulary

Short Description or Definition Extinction refers to a failure to detect one of two simultaneously presented stimuli, even though when presented individually each stimulus can be detected.

References and Readings Baker, S., Simmons, D., & Kameenui, E. (1998). Vocabulary acquisition: Research bases. In D. C. Simmones & E. J. Kameenui (Eds.), What reading tells us about children with diverse learning needs. Mahwah, NJ: Lawrence Erlbaum Associates. Brownell, R. (2000). Expressive one-word picture vocabulary test manual. Novato, CA: Academic Therapy Publications.

Categorization Extinction during DSS can occur in the somatasensory, auditory, and visual modalities.

Epidemiology

Extended Glasgow Outcome Scale ▶ Glasgow Outcome Scale – Extended

Extensor Posturing ▶ Decerebrate Posturing

External Aids ▶ Cognitive Correctors

External Memory Aids ▶ Prosthetic Memory Aids

Extinction R ICHARD F. K APLAN UConn Health Center Farmington, CT, USA

Synonyms Sensory extinction; Extinction to double simultaneous stimulation (DSS)

Extinction most often occurs with damage to the right hemisphere usually seen after stroke or neoplasm. However, under rare circumstances, extinction has been shown to occur in normal individuals and in patients with left hemisphere lesions. Extinction is usually considered as a manifestation of the neglect syndrome but often persists after other symptoms of neglect resolve. Neglect in humans can occur with lesions in the inferior parietal lobe, dorsal frontal lobe, cingulated gyrus, basal ganglia, and thalamus, but is most frequently observed after temporoparietal lesions.

Natural History, Prognostic Factors, Outcomes In a study of behavioral recovery from abnormalities after right hemisphere stroke (Hier et al., 1993), 26 of 41 patients initially presented with extinction. Whereas all patients recovered from neglect, 20% continued to show extinction after 1 year. Patients with hemorrhage recovered more quickly than those with infarcts. Recovery is less complete in patients with prestroke cortical atrophy, suggesting that the integrity of the left hemisphere is essential.

Neuropsychology of Extinction From a neuropsychological perspective, extinction provides a glance into how the brain mediates spatial attention. Walter Poppelreuter and Gordon Holmes provided some of the earliest descriptions of visual extinction early in the twentieth century. Because visual acuity could be completely normal in the affected hemi-field when one stimulus was presented, Poppelreuter (1990) called the phenomenon a ‘‘hemianoptic weakness of attention.’’ Their recognition that this was an impairment of attention provided much of the groundwork for later theories about how the brain

Extrapyramidal Adverse Reactions

mediates spatial attention (see Halligan & Marshall, 1993). Additional evidence that extinction is an attentional disorder comes from studies showing that extinction can be modified by experimental manipulations. When a leftsided stimulus is presented less than a second before the right-sided stimulus, extinction decreases dramatically. The probability that extinction will occur in the right hemisphere damaged patients can also be modified by expectation (Kaplan, Verfaellie, DeWitt, & Caplan, 1990). By manipulating the unilateral stimuli prior to DSS, these investigators showed that they could increase or reduce the probability of extinction. To what extent learning to anticipate left-sided stimulation is part of the recovery process is unclear, but it may be of compensatory strategy. Theories of neglect are based on the premise that there is asymmetry between the cerebral hemispheres in mediating attention in space. The normal right hemisphere directs attention to both sides of space, whereas the left hemisphere mediates attention to contralateral space. Consequently, after right hemisphere damage, the patient will show a strong attentional bias to the left side of space, and neglect, including extinction, for the right side of space. Moreover, because the normal right hemisphere can direct attention to both sides of space, neglect after left hemisphere damage is relatively rare.

Evaluation Testing for extinction to DSS is typically done at the bedside or office. To elicit visual extinction, the patient faces the examiner, while the examiner presents a finger to the left, right, or both visual fields. After each trial, the patient indicates whether he or she saw a finger on the left, right, or both sides. During testing for auditory extinction, the examiner stands behind the patient, and rubs his or her fingers together next to one of both ears using a method similar to that described above. For somatasensory extinction testing, the patient is blindfolded or asked to close his/her eyes and the examiner touches either the back of the patient’s hand or face. A standardized procedure for testing sensory extinction is part of the Rivermead Assessment of Somatasensory Performance battery (Strauss, Sherman, & Preen, 2006).

Treatment There is no treatment for extinction separate from treatments for neglect, as extinction is considered part

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of the neglect syndrome. Most treatments involve manipulations designed to make the patient scan the neglected hemi-field. Unfortunately, most techniques have proved to be short-lived and do not translate to activities of daily living. That being the case, patients should be instructed to avoid activities, i.e., driving, that could cause injuries to themselves or others.

Cross References ▶ Hemiinattention ▶ Neglect Syndrome

References and Readings Halligan, P. W., & Marshall, J. C. (1993). The history and clinical presentation of neglect. In I. H. Robertson & J. C. Marshall (Eds.), Unilateral neglect: Clinical and experimental studies (pp. 3–19). Hillsdale, NJ: Lawrence Erlbaum. Hier, D, B., Mondlock, J., & Caplan. L. R. (1983). Recovery of behavioral abnormalities after right hemisphere stroke. Neurology, 33, 345–350. Kaplan, R. F., Verfaellie, M., DeWitt, L. D., & Caplan, L. R. (1990). Effects of changes in stimulus contingency on visual extinction. Neurology, 40, 1299–1301. Poppelreuter, W. (1990). Disturbances of lower and higher visual capacities by occipital damage. (J. Zihl, Trans.). Oxford: Clarendon Press. Strauss, E., Sherman, E., & Preen, O. (2006). A compendium of neuropsychological tests (3rd ed.). New York: Oxford.

Extinction to Double Simultaneous Stimulation (DSS) ▶ Extinction

Extraneous Variable ▶ Confounding Variables

Extrapyramidal Adverse Reactions ▶ Extrapyramidal Symptoms

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Extrapyramidal Disease

Extrapyramidal Disease ▶ Extrapyramidal Symptoms

Extrapyramidal Side Effects ▶ Extrapyramidal Symptoms

Extrapyramidal Symptoms M ARLA S ANZONE Independent Practice Annapolis, MD, USA

Mechanisms of Action The extrapyramidal system comprises interconnections among brain stem nuclei and basal ganglia, relaying signals between the efferent fibers of the thalamus, cerebral cortex, the brainstem and cerebellum, and the five nuclei of the basal nuclei, including the corpus striatum, red nucleus, subthalamic nucleus, substantia nigra, and the reticular formation. The extrapyramidal pathways are the undecussated or uncrossed functional motor nerve nuclei from the brain to the spinal cord’s anterior horns. Essential to coordinating locomotion and supporting static and postural reflexes through its efferent fibers, the extrapyramidal tracts are the relay mechanism responsible for sequential and simultaneous movements. The efferent fibers of the extrapyramidal system do not act directly on the lower motor neurons of the spinal cord, but are confined to the brain and modulate the pyramidal or corticospinal tracts (Afifi and Bergman, 1998; Stahl, 2000).

Specific Compounds and Properties Synonyms EPS; Extrapyramidal adverse reactions; Extrapyramidal disease; Extrapyramidal side effects; Parkinsonian movements

Indications ‘‘Extrapyramidal’’ refers to the nerve fibers outside the pyramidal tracts of the central nervous system. The term extrapyramidal symptoms refers to the neurological adverse effects from antipsychotic medications that mimic the characteristics of extrapyramidal disease. Pharmacological agents that decrease dopamine neurotransmission other than neuroleptics, can produce extrapyramidal symptoms as well, though neuroleptics, particularly those that block D2 receptors, are most often responsible for causing extrapyramidal adverse reactions. Standard practice when administering conventional antipsychotic medication is augmentation with anti-cholinergic agents such as benztropine (Cogentin) that block striatal cholinergic receptors can help regulate cholinergic and dopaminergic neurotransmission reducing the severity of extrapyramidal side effects (Extrapyramidal symptoms, 2009a, b; Kaufman, 2007a; Myers, 2006; Venes, Thomas, Egan, & Houska, 2001).

Extrapyramidal diseases encompass the unrelated group of conditions that compromise neuronal functioning within the extrapyramidal tracts such as tardive dyskinesia, chorea, athetosis, extrapyramidal cerebral palsy, Shy– Drager syndrome, Parkinson’s or Alzheimer’s diseases, and other degenerative neurological disorders. Physical extrapyramidal symptoms include abnormal posture, involuntary movement such as tremor and shuffling gate, muscle dystonia including muscle rigidity and contractility. Other non-movement symptoms of extrapyramidal disease can include anxiety, akathisia, slurred speech, and bradykinesia (Extrapyramidal symptoms, 2009a; Kaufman, 2007b; Myers, 2006; Venes et al., 2001).

Clinical Use Conventional neuroleptic drugs are used to treat psychotic disorders such as schizophrenia. Clinical observation of early treatments for schizophrenia provided invaluable data about the mechanisms underlying the neurobiological basis of psychosis and ultimately of the antipsychotic medications that would be developed to remediate the positive symptoms of formal thought disorders such as hallucinations. In fact, the term ‘‘neuroleptic’’ derives from ‘‘neurolepsis,’’ meaning slow or absent motor movement. The psychomotor retardation

Extrastriate

and diminished affect characteristic of the effects of firstgeneration antipsychotic drugs reflect dopamine antagonism, the mechanism by which these agents decrease psychotic symptoms. It is believed that blocking the D2 receptors specifically, has a significant effect on the reduction of positive symptoms. Unfortunately, selective D2 antagonism has not yet been achieved, and this type of dopaminergic antagonism, particularly in the postsynaptic mesolimbic area is also largely responsible for neurolepsis or extrapyramidal symptoms, the slow, dysrhythmic, uncontrolled and involuntary behavior seen among individuals on high doses of conventional/typical, and to a lesser extent atypical or second-generation antipsychotic medications. Blockade of the D2 nigrostriatal region produces marked vulnerability to tardive dyskinesia, perhaps the most severe of the extrapyramidal symptoms due to its propensity for permanence. Tardive dyskinesia afflicts approximately 20% annually of those taking conventional antipsychotic medications within 4 years (Kaufman, 2007b; Stahl, 2000). Medications within this class with considerable D2 antagonism and risk of extrapyramidal symptoms include acetophenazine (Tindal), chlorpromazine (Thorazine), chlorprothixene (Taractan), clozpine (Clozaril), fluphenazine (Prolixin), haloperidol (Haldol), loxapine (Loxitane), mesoridazine (Serentil), molindone (Moban), perphenazine (Trilafon), pimozide (Orap), prochlorperazine (Compazine), thioridazine (Mellaril), thiothixene (Navane), trifluoperazine (Stelazine), and triflupromazine (Vesprin) (Afifi and Bergman, 1998; Kaufman, 2007b; Stahl, 2000).

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Extrapyramidal symptoms. (2009a). In The free dictionary by Farlex, Retrieved January 13, 2009, from http://medical-dictionary.thefreedictionary.com Extrapyramidal symptoms. (2009b). In MedicineNet. Retrieved January 13, 2009, from http://www.medterms.com Kaufman, D. M. (2007a). Central nervous system disorders. In D. M. Kaufman (Ed.), Clinical neuropsychiatry for psychiatrists (6th ed., pp. 5–14). Philadelphia: Saunders Elsevier. Kaufman, D. M. (2007b). Congenital cerebral impairments. In D. M. Kaufman (Ed.), Clinical neuropsychiatry for psychiatrists (6th ed., pp. 295–316). Philadelphia: Saunders Elsevier. Myers, T. (2006). Extrapyramidal symptoms (definition). In T. Myers (Ed.), Mosby’s dictionary of medicine, nursing & health professions (7th ed., pp. 697). Missouri: Mosby Elsevier. Shy–Drager syndrome. (2003). In MedicineNet. Retrieved February 3, 2009, from http://www.medterms.com. Stahl, S. M. (2000). Antipsychotic agents. In S. M. Stahl (Ed.), Essential psychopharmacology: Neuroscientific basis and practical applications (2nd ed., pp. 401–458). New York: Cambridge University Press. Venes, D., Thomas, C., Egan, E., & Houska, A. (2001). Extrapyramidal symptoms (definition). In Venes, D., et al. (Eds.), Taber’s medical dictionary (19th ed., pp. 760). Philadelphia: FA Davis Company.

Extrastriate R ONALD A. C OHEN Brown University Providence, RI, USA

Synonyms Cross References ▶ Alzheimer’s Disease ▶ Antipsychotic Medications ▶ Chorea ▶ Dopamine Antagonists ▶ Extrapyramidal Cerebral Palsy ▶ Extrapyramidal Syndrome ▶ Extrapyramidal System ▶ Parkinson’s Disease ▶ Psychotropic Medications ▶ Pyramidal Tract ▶ Shy–Drager Syndrome

References and Readings Afifi, A. K., & Bergman, R. A. (1998). Basal ganglia. In A. K. Afifi & R. A. Bergman (Eds.), Functional neuroanatomy: Text & atlas (pp. 276–295). New York: McGraw-Hill.

Broadmann areas 18, 19; Heterotypic visual cortex; Secondary visual cortex; V2, V3, V4, V5

Definition The extrastriate cortex refers to occipital cortical areas found anterior to the striate cortex (i.e., primary visual cortex), corresponding to visual areas 2, 3, 4, and 5 in primates. The extrastriate consists of heterotypic cortical neurons that may respond to different types of inputs relative to the primary visual information arising from the primary visual cortex. Accordingly, it plays an essential role in mid-level visual processing or what neuropsychologists often refer to as visual integration. Extrastriatal neurons tend to respond to visual stimuli within receptive fields, with these responses modulated by other cognitive processes, such as motivational signals, attention, working memory, and response demands.

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Eye Fields

Cross References ▶ Cortical Magnification ▶ Enhancement ▶ Feature Detection ▶ Lateral Inhibition ▶ Visual Integration ▶ ‘What System’ ▶ ‘Where System’

Eye Fields M ICHAEL A. F OX Virginia Commonwealth University Medical Center Richmond, VA, USA

Definition Visual fields refer to the entire region of space observed by both eyes. Visual fields can be subdivided into right and left hemifields. Cardinal positions are the specific eye movements that assess the function of extraocular muscles and the cranial nerves that innervate them.

Current Knowledge Visual Fields Visual fields, the entire region of space observed by both eyes, can be divided either into central and peripheral regions or into right and left hemifields. Laterally located retinal cells respond to visual stimuli in central regions, whereas medially located retinal cells respond to stimuli in peripheral portions of the ipsilateral visual field. A lesion to an entire eye or its optic nerve therefore results in the loss of ipsilateral peripheral vision. Central ipsilateral and all of the contralateral visual field regions are still seen by the unaffected, contralateral eye. As ganglion cells project their fibers into the CNS, visual pathways are reorganized. Medially located ganglion cell fibers cross at the optic chiasm while those from laterally located ganglion cells remain uncrossed. As a result of partial crossing, optic tracts and visual processing centers in the brain (i.e., the lateral geniculate nucleus of the thalamus [LGN] and the primary visual cortex) receive input only from contralateral visual hemifields. Transection of the optic tract or a thalamic lesion

therefore results in the loss of an entire contralateral hemifield. A second reorganization of visual information occurs as LGN neurons relay information to visual cortex. Visual information from the superior quadrant of the contralateral hemifield is sent to inferior regions of visual cortex by thalamic fibers that follow a pathway called Meyer’s loop. Conversely, fibers carrying information from the inferior quadrant follow the optic radiations to terminate in superior regions of the visual cortex. Therefore, a lesion to the optic radiations or the superior primary visual cortex results in the loss of vision from the inferior quadrant of the contralateral hemifield. Likewise, a lesion in Meyer’s loop or in the inferior primary visual cortex results in the loss of vision from the superior quadrant of the contralateral hemifield.

Eye Movements Eye movements are controlled by six extraocular muscles: superior, inferior, medial and lateral rectus muscles, and superior and inferior oblique muscles. Three cranial nerves (CNs) originating from the brainstem innervate these muscles: trochlear nerves (CN IV) innervate superior oblique muscles, abducens nerves (CN VI) innervate lateral rectus muscles, and occulomotor nerves (CN III) innervate the rest. Clinicians test extraocular muscle function, and importantly the nerves innervating them, with cardinal positions – sequential movements that determine individual extraocular muscle function. Eyes are first directed to one side, testing the medial rectus of one eye and the lateral rectus of the other. While looking to one side, eyes are shifted upward, testing the superior rectus of the laterally gazing eye and the inferior oblique of the medially gazing eye. While still looking to one side, eyes are then shifted downward, testing the inferior rectus of the laterally gazing eye and the superior oblique of the medially gazing eye. With these six cardinal positions and the knowledge of nerves innervating each extraocular muscle, clinicians can determine potential lesions sites in the brainstem that lead to defects in eye movements.

Cross References ▶ Cranial Nerves ▶ Frontal Eye Fields ▶ Lateral Geniculate Nucleus of Thalamus ▶ Visual Cortex

Eysenck Personality Inventory

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Current Knowledge

Bear, M. F., Connors, B. W., & Paradiso, M. A. (2001). Neuroscience: Exploring the brain (2nd ed.). Baltimore, MD: Lippincott Williams & Wilkins. Snell, R. S. (1995). Clinical anatomy for medical students (5th ed.). Boston, MA: Little, Brown and Company.

Test Theory, Development, and Properties

Eye Movements ▶ Conjugate Gaze

Eye Preference ▶ Lateral Dominance

Eysenck Personality Inventory A NGELA M. B ODLING , T HOMAS M ARTIN University of Missouri—Columbia Columbia, MO, USA

Synonyms EPI

Definition The Eysenck Personality Inventory (EPI) is a self-report instrument designed to measure two central dimensions of personality, extraversion and neuroticism. This instrument is comprised of 57 yes/no items and yields total scores for extraversion and neuroticism as well as a validity score (e.g., Lie Scale). Individuals are generally classified as ‘‘high’’ or ‘‘low’’ on the two dimensions. Persons high in extraversion are seen as social, carefree, and optimistic, while low scorers are generally quiet, introspective, and reserved. Individuals classified as high in neuroticism are prone to emotional distress/instability, while those low in this dimension are generally calm and emotionally stable.

The EPI was developed in 1964 based on a conceptualization of personality that identifies extraversion and neuroticism as the two primary and independent factors comprising the global construct of personality. The specific inventory was constructed from an existing assessment (the Maudsley Personality Instrument; Eysenck & Eysenck, 1964) similarly based on this twofactor model; individual test items for the EPI (and its predecessor) were selected via factor analysis. Subsequent developments included creation of alternate forms (A & B), reported lowering of the reading level (although prior and current reading level are not specified in the manual or associated references), refined item selection to further distinguish the extraversion and neuroticism scales, addition of a 9-item ‘‘Lie Scale’’ to provide an index of validity, and improved test–retest reliability. The EPI Manual notes reliability estimates for the EPI ranging from 0.81 to 0.97 for test–retest reliability and from 0.74 to 0.91 for split-half reliability.

Revisions and Alternate Versions Since its initial development, the EPI has undergone multiple transformations, including the 1975 expansion to the 90-item Eysenck Personality Questionnaire (EPQ), which added items to assess a third personality dimension (Psychoticism), and revision of the EPQ Psychoticism Scale in 1985 to create the 100-item Eysenck Personality Questionnaire – Revised (EPQR). Each revision was designed to expand the scope of personality characteristics evaluated by the instrument. These revisions also increased the administration time of the instrument, which posed concerns for some and ultimately prompted development of several abbreviated forms (e.g., EPQR-A, EPQR-S, etc.; Francis, Brown, & Philipchalk, 1992).

Suggested Uses and Relevant Research The EPI Manual recommends use of this measure across research settings as well as in the diagnosis and treatment of individual personality dysfunction. Current use of the EPI for clinical assessment and

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diagnosis is arguably surpassed by other personality assessments (e.g., the Minnesota Multiphasic Personality Inventory – 2, Personality Assessment Inventory). However, empirically, the EPI continues to be used to assess the relations between specific personality characteristics and a variety of psychological phenomena. Relevant findings include those suggesting that individuals involved in traumatic accidents leading to disability in motor functioning tend to be higher in extroversion than those with nontraumatic injuries (Malec, 1985); individuals high in extroversion and low in neuroticism have a lower risk for developing dementia than those with other personality profiles (Wang et al., 2009); and individuals higher in neuroticism tend to be at greater risk for acute stress disorder, following mild traumatic brain injury (Bryant & Harvey, 1998). It is noteworthy that while these relations (and others) have been documented in individual studies, replication of findings is generally limited. Furthermore, while discussions of the clinical implications of these correlations exist, empirical investigation of clinical utility is lacking.

Cross References ▶ Minnesota Multiphasic Personality Inventory ▶ NEO Personality Inventory ▶ Neuroticism ▶ Personality Assessment Inventory

References and Readings Bryant, R. A., & Harvey, A. G. (1998). Predictors of acute stress following mild traumatic brain injury. Brain Injury, 12(2), 147–154. Eysenck, H. J., & Eysenck, S. B. G. (1964). Manual of the eysenck personality inventory. London: University of London Press. Francis, L. J., Brown, L. B., & Philipchalk, R. (1992). The development of an abbreviated form of the revised Eysenck Personality Questionnaire (EPQR-A): Its use among students in England, Canada, the U.S.A. and Australia. Personality and Individual Differences, 13(4), 443–449. Malec, J. (1985). Personality factors associated with severe traumatic disability. Rehabilitation Psychology, 30(3), 165–172. Wang, H. X., Karp, A., Herlitz, A., Crowe, M., Kareholt, I., Winblad, B., et al. (2009). Personality and lifestyle in relation to dementia incidence. Neurology, 72, 253–259.

F F Minus K Index R ICHARD T EMPLE CORE Health Care Dripping Springs, TX, USA

▶ Faking Good, Bad ▶ K Scale ▶ L Scale ▶ Minnesota Multiphasic Personality Inventory ▶ True Response Inconsistency Scale (TRIN, MMPI) ▶ Validity Scales (MMPI) ▶ Variable Response Inconsistency Scale (VRIN, MMPI)

Synonyms Gough dissimulation index

Definition Validity indicator on the Minnesota Multiphasic Personality Inventory (MMPI) and its revisions, designed to detect overreporting of symptoms. This index is calculated by subtracting the raw score of the K scale from the raw score of the F scale. The original cutoff set by Gough (1947) for overreporting was nine. However, the literature reports disparate results for the sensitivity and specificity of this cutoff. Given that the F scale is indicative of actual psychopathology, the F minus K scale is likely to produce a high false positive rate. As a result, this scale may be one of the less-desirable measures of overreporting of symptoms. Despite this limitation, the F and K scales, either alone or in combination, may still be utilized to aid the clinical neuropsychologist in determining the patient’s response style, particularly on the clinical interview and subjective self-report measures. With these validity scores, the clinician can interpret self-report information in the context of a continuum between magnification of symptoms to minimization/defensiveness. Readers are referred to the MMPI entry for a discussion of limitations of this self-report measure when used with neuropsychological populations (see also Gass, 2006 and Lezak, Howieson, & Loring, 2004, pp. 748–750).

Cross References ▶ F Scale ▶ Fake Bad Scale

References and Readings Gass, C. (2006). Use of the MMPI-2 in neuropsychological evaluations. In J. Butcher (Ed.), MMPI-2: A practitioner’s guide (pp. 301–326). Washington, DC: American Psychological Association. Gough, H. G. (1947). Simulated patterns on the MMPI. Journal of Abnormal and Social Psychology, 42, 215–225. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

F Scale R ICHARD T EMPLE CORE Health Care Dripping Springs, TX, USA

Synonyms Frequency scale; Infrequency scale

Definition Validity scale on the Minnesota Multiphasic Personality Inventory (MMPI) and its revisions designed to detect unusual approaches to answering test items. The scale is comprised of items endorsed by no more than 10% of an early sample of respondents. Items represent unlikely or contradictory beliefs, expectations, or self-descriptions (Dahlstrom, Welsh, & Dahlstrom, 1972). The majority


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of items are unique to this scale, although some overlap with scales 6 (paranoia) and 8 (schizophrenia). Profiles with extreme scores are generally deemed invalid, as the individual may be significantly compromised or psychotic, although the research on cutoff scores is somewhat inconsistent. Within the realm of neuropsychological evaluation, moderately elevated scores may indicate awareness of neurological compromise and a ‘‘cry for help’’ regarding these difficulties. Readers are referred to the MMPI entry for a discussion of limitations of this selfreport measure when used with neuropsychological populations (Gass, 2006 and Lezak, Howieson & Loring, 2004).

Facial Agnosia ▶ Prosopagnosia

Facial Bradykinesia ▶ Masked Facies

Facial Recognition Test Cross References ▶ F Minus K Index ▶ Fake Bad Scale ▶ Faking Good, Bad ▶ K Scale ▶ L Scale ▶ Minnesota Multiphasic Personality Inventory ▶ True Response Inconsistency Scale (TRIN, MMPI) ▶ Validity Scales (MMPI) ▶ Variable Response Inconsistency Scale (VRIN, MMPI)

References and Readings Dahlstrom, W. G., Welsh, G. S., & Dahlstrom, L. E. (1972). An MMPI handbook: Vol. 1. Clinical interpretation. Minneapolis: University of Minnesota Press. Gass, C. (2006). Use of the MMPI-2 in neuropsychological evaluations. In J. Butcher (Ed.), MMPI-2: A practitioner’s guide (pp. 301–326). Washington, DC: American Psychological Association. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

FAC ▶ Functional Ambulation Classification

Face Blindness ▶ Prosopagnosia

U RAINA C LARK The Miriam Hospital, Neuropsychology Providence, RI, USA

Synonyms Benton face recognition test; Benton faces; Test of facial recognition

Description The Facial Recognition Test assesses the ability to identify and discriminate photographs of unfamiliar faces. The test presents the patient with a series of stimulus cards showing black-and-white photographs of facial images. Each stimulus card displays a single front-view image of a target face and requires the patient to identify the person presented in the target image in an array of six photographs presented below the target image. Each card presents the patient with a different target and test image array. Both male and female images are presented; each stimulus card displays either a male or female array. The test consists of three parts: A, B, and C. In part A, six stimulus cards are presented in which the target and test image arrays are presented as front-view images. Only one image of the target face is presented in the test array, calling for a total of six responses for part A. In parts B and C the patient is asked to locate three images of the target within the array of six photographs. The stimuli in parts B and C are presented in two variations, which increase the difficulty of the test items. Cards in part B include an array of six facial images presented at three-quarter views. Cards in part C present the patient with an array of six facial photographs taken under different lighting conditions. The Long Form contains 22 stimulus cards: 6 from part

Facial Recognition Test

A, 8 from part B, and 8 from part C, thus calling for 54 responses. The Short Form of the Facial Recognition Test includes all 6 cards in part A, as well 4 from part B, and 3 from part C, giving a total of 27 responses. Patients are allowed to manipulate and hold the stimuli to improve their perception of the stimuli and reduce glare from the page. Patients are not given a time limit on their responses. It is estimated that the Long Form can take between 10 and 20 min to administer; however, the administration time can vary depending on the patient’s speed and cautiousness in responding. The total administration time for the Short Form ranges between 5 and 15 min, with a mean of 7 min. For both the Long and the Short Forms, a total score is calculated based on the number of correct responses. On the Long Form, a minimum of 25 points is expected based on chance alone. Similarly, a score of 11 may be expected on the Short Form simply on the basis of chance. Scores from the Short Form are converted to projected Long Form scores. Studies suggest that age and education level can affect performance. Age and education corrections should be added to Long Form scores according to the manual. No sex differences have been reported. The test is thought to be relatively independent of ethic or cultural factors, as studies have reported test score similar to those obtained in the standardization sample in Hispanic, Italian, and inner-city African American adults.

Historical Background The Long Form of this test was originally described by Benton and Van Allen (1968); it was employed in a study that assessed the relation of facial identification to visual field defects, lesion location, and aphasia. The 27 item Short Form was developed 7 years later, as described by Levin, Hamsher, and Benton (1975). A modified short form was put forth by Christensen, Riley, Hefferman, Love, and McLaughlin (2002); this modified short form includes items 7, 8, 10–12, 14–19, and 22.

Psychometric Data Much of the normative data was collected by Benton and colleagues more than 30 years ago. Benton, Hamsher, Varney, and Spreen (1983) reported normative data for children aged 6 through 14 years, and for adults aged 16–74 years. Christensen et al. (2002) reported normative data for older adults between 60 and 90+ years, which

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are preferred when assessing the performance of older adults. Coefficient alpha for the Long Form in a group of 206 young adults was 0.57; however, when the first six items of the test were removed, internal reliability was higher (0.66). Data from the sample collected by Christensen et al. (2002) indicated that internal consistency for the original Long and Short Forms was 0.72 and 0.53 respectively; the group’s modified short form yielded a coefficient alpha of 0.69. One-year test-retest reliability in a sample of healthy older adults was reported to be 0.60 for the Short Form, and 0.71 for the Long From. Scores for Christensen’s modified short form (Christensen et al., 2002) showed no significant change over 1 year and had a stability coefficient of 0.71. Correlations between the Long and Short Forms in samples of control and braindiseased patients were reported to be 0.88 and 0.92, respectively. Correlations between the Long Form and Christensen’s modified short form were high (>0.90) in healthy older adults. Practice effects on this test are reported to be minimal.

Clinical Uses The Facial Recognition Test can be used to assess the ability to discriminate unfamiliar faces in children and adults. Patients with right posterior lesions and those with right parietal lesions are reported to have a high failure rate, thus this test is thought to be sensitive to visuospatial dysfunction. Notably, patients with left hemisphere lesions have also been reported to perform poorly on this test. For example, one study found that a subgroup of aphasic patients with comprehension deficits performed poorly on this measure (Hamsher, Levin, and Benton, 1979); this finding has been interpreted to suggest that the test may involve a linguistic, as well as a visuospatial, component. Poor performance has been reported in patients with Parkinson’s disease, multiple sclerosis, moderate-to-severe closed head injury, and alcoholism. Impairment has been also been reported in children with perinatal brain damage. Accordingly, this test may be useful in the assessment of children with a suspected learning or developmental disability, and in the evaluation of adults with acquired brain damage, neurological disorder, or possible dementia. It should be noted that normal performance levels can be obtained by employing a feature matching strategy rather than by using a more holistic or configural approach. Further, assessments of patients with prosopagnosia have resulted in mixed findings, with some patients

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performing in the normal range on this test. Consequently, one may wish to be cautious when interpreting normal performance on this measure, as studies suggest that obtaining a normal score is not an absolute indication that a patient’s facial processing abilities are fully intact.

Cross References ▶ Prosopagnosia

References and Readings Benton, A. L., Hamsher, K. de S., Varney, N. R., & Spreen, O. (1983). Contributions to neuropsychological assessment: A clinical manual. New York: Oxford University Press. Benton, A. L., & Van Allen, M. W. (1968). Impairments in facial recognition in patients with cerebral disease. Cortex, 4, 344–358. Christensen, K. J., Riley, B. E., Hefferman, K. A., Love, S. B., & McLaughlin, M. E. (2002). Facial recognition test in the elderly: Norms, reliability and premorbid estimate. Clinical Neuropsychologist, 16, 51–56. Hamsher, K. de S., Levin, H. S., & Benton, A. L. (1979). Facial recognition in patients with focal brain lesions. Archives of Neurology, 36, 837–839. Levin, H. S., Hamsher, K. de S., & Benton, A. L. (1975). A short form of the test of facial recognition for clinical use. Journal of Psychology, 91, 223–228. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests: Administration, norms, and commentary (3rd ed.). New York: Oxford University Press.

Facilitation R ONALD A. C OHEN Brown University Providence, RI, USA

Definition Facilitation refers to processes by which the probability and/or intensity of a response is increased. At an elementary level, neural facilitation occurs when there is an increase in post-synaptic potential resulting from the occurrence of a second stimulus shortly after the first. This process contributes to sensitization and is ultimately linked to the formation of a conditioned response. In the context of attention, facilitation occurs when focus is enhanced by the occurrence of some preexisting conditions or stimuli. For example, when a target

stimulus is preceded by another stimulus, the first stimulus tends to draw attentional focus, thereby increasing the probability of detecting or intensity of focus and responding to the second stimulus. Attentional facilitation depends on complex interactions of the brain systems that underlie selective and focused attention, though neural facilitation is an important substrate of higherlevel attentional facilitation.

Current Knowledge Given that an essential aspect of attention is its selectivity enabling increased response to relevant information and decreased response to irrelevant stimuli, cognitive scientists hypothesized the existence of processes that both facilitate and inhibit subsequent processing. Facilitation refers to processes that increase the attentional response and enable greater efficiency of cognitive processing to information that is selected. Neuronal mechanisms governing inhibition and facilitation have been demonstrated by electrophysiological studies of laboratory animals and also functional imaging in humans. Inhibition has been somewhat easier to demonstrate, as studies that show reduced neuronal response in brain areas processing unattended information provide evidence of this effect. However, evidence of neuronal facilitation has also been demonstrated in studies in which subjects engage in a demanding cognitive operation in the context of interference. The co-existence of inhibition to neurons processing the distraction, along with increased response of neural areas involved in the required cognitive operation has been demonstrated (Ghatan, Hsieh, Petersson, Stone-Elander, & Ingvar, 1998). It is tempting to equate facilitation with neural excitation, though this is often not the case. While facilitation often involves increased excitation in brain areas required for focused attention to the task, facilitation may also occur as a by-product of the inhibition of unrelated areas. Accordingly, facilitation is an essential process underlying attention that may occur as the by-product of the interaction of neural inhibition and activation that typically occur simultaneously and involve complex interactions across brain systems.

Cross References ▶ Enhancement ▶ Inhibition ▶ Selective Attention

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Ghatan, P. H., Hsieh, J. C., Petersson, K. M., Stone-Elander, S., & Ingvar, M. (1998). Coexistence of attention-based facilitation and inhibition in the human cortex. Neuroimage, 7(1), 23–29.

Gorsuch, R. L. (2003). Factor analysis. In J. A. Schinka & W. F. Velicer (Eds.), Handbook of psychology: Research methods in psychology (Vol. 2, pp. 143–164). Hoboken, NJ: Wiley. Immekus, J. C., & Maller, S. J. (2010). Factor invariance of the Kaufman Adolescent and adult intelligence test across male and female samples. Educational and Psychological Measurement, 70, 91–104. Thompson, B. (2004). Exploratory and confirmatory factor analysis: Understanding concepts and applications. Washington, DC: American Psychological Association.

Factor Analysis M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Factor analysis is a statistical method of reducing a larger number of variables into a smaller number of factors by examining patterns of intercorrelation among the variables. It is an attempt to find the simple structure of the data. For example, factor analysis might be applied to a set of test items in order to determine if different items might be organized into subtests. In conducting a factor analysis, the researcher must make decisions about the type of rotation used (orthogonal or oblique), the number of factors to extracted, the criteria for considering a factor loading as being meaningful, and finally the nature of the obtained factors.

F FAD ▶ McMaster Family Assessment Device

FAI ▶ Frenchay Activity Index

FAI-18 ▶ Frenchay Activity Index

Fainting Current Knowledge There are many different methods and models of factor analysis but the two basic general models are exploratory factor analysis and confirmatory factor analysis. In exploratory factor analysis, decisions are made regarding the above-mentioned categories, and the resulting factor structure is interpreted. In confirmatory factor analysis, a specific factor structure, either on the basis of prior research with a separate sample or on the basis of theory, is evaluated as to its fit to the data. Confirmatory factor analysis is therefore an evaluation of a model and allows a statistical test of the similarity of different factor structure models to each other.

▶ Syncope

Fake Bad Scale R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms FBS; Symptom exaggeration

Description Cross References ▶ Structural Equation Modeling

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As a result of observing a distinct pattern of symptom reporting in personal injury litigants and secondary to

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the F-family’s limitations, Lees-Haley and colleagues (Lees-Haley, English, & Glenn, 1991; Lees-Haley, 1992) developed the ‘‘Fake Bad Scale’’ (FBS) of the MMPI-2. It was intended to be sensitive to personal injury exaggeration and was constructed on a ‘‘rational content basis.’’ Specifically, the authors utilized unpublished frequency counts of litigant’s MMPI-2 test data and responses that coincided with a model of goal-directed behavior characterized by: (1) appearing honest; (2) appearing psychologically normal, except for the influence of the alleged cause of injury; (3) avoiding admitting preexisting psychopathology; (4) attempting to minimize the impact of previously disclosed preexisting complaints; (5) minimizing or hiding pre-injury antisocial or illegal behavior; and (6) presenting a degree of injury or disability within perceived limits of plausibility. Since its inception, a number of studies have investigated the FBS with various patient populations including traumatic brain injury, post-traumatic stress disorder (PTSD), chronic pain, depression, job applicants, prison inmates, and in a variety of other neurologic and psychiatric disorders. It has shown adequate specificity and sensitivity (cf. Greiffenstein, Fox, & Lees-Haley, 2007). Studies differ as to the appropriate cutoff score (>22) for labeling someone a ‘‘somatic malingerer.’’ However, scores of 30+ never or rarely produce false-positive errors (i.e., calling someone a malingerer when they are not). Put in another way, the positive predictive power (PPP) of the FBS 0.27) impairment. Given the widespread use of the HRNB and FTT over 6+ decades, the measure has been (and can be) used to screen for motor impairment with nearly all clinical populations.

Cross References ▶ Halstead–Reitan Neuropsychological Test battery

References and Readings Dikmen, S. S., Heaton, R. K., Grant, I., & Temkin, N. R. (1999). Testretest reliability and practice effects of expanded Halstead-Reitan Neuropsychological Test Battery. Journal of the International Neuropsychology Society, 5(4), 346–356. Gill, D. M., Reddon, J. R., Stefanyk, W. O., & Hans, H. S. (1986). Finger tapping: Effects of trials and sessions. Perceptual & Motor Skills, 62, 675–678. Goldstein, G., & Watson, J. R. (1989). Test-retest-reliability of the Halstead-Reitan battery and the WAIS in a neuropsychiatric population. The Clinical Neuropsychologist, 3, 265–272. Groth-Marnat, G. (2003). Handbook of psychological assessment (4th ed.). Hoboken, NJ: Wiley. Jarvis, P. E., & Barth, J. T. (1984). Halstead-Reitan test battery: An interpretive guide. Odessa, FL: Psychological Assessment Resources, Inc. Reitan, R. M. (1969). Manual for the administration of neuropsychological test batteries on adults and children. Bloomington IN: Indiana University Press. Reitan, R. M., & Wolfson, D. (1993). The Halstead-Reitan neuropsychological test battery: Theory and clinical interpretation (2nd ed.). Tucson, AZ: Neuropsychology Press. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests: administration, norms, and commentary. New York: Oxford University Press. Ciety, 40, 922–935.

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Finger to Nose Test G RANT L. I VERSON University of British Columbia & British Columbia Mental Health & Addictions Vancouver, British Columbia, Canada

Description The Finger-to-Nose-Test measures smooth, coordinated upper-extremity movement by having the examinee touch the tip of his or her nose with his or her index finger. On one variation of the test, the examiner holds out his or her finger, about an arm’s length from the patient. The patient is instructed to touch the examiner’s finger, then his or her own nose. After several successful trials, the patient is then asked to repeat the action more quickly. Moving the target finger can increase the difficulty of the task.

Current Knowledge This test is part of a comprehensive neurological examination. It is typically employed as part of coordination testing. The examiner looks for evidence of intention tremor or dysmetria. Dysmetria is evidenced by difficulty in controlling the range of movement. Dysmetria can result in undershooting or overshooting the target stimuli (i.e., examiner’s finger and/or examinee’s nose). Damage to the cerebellum can adversely affect a person’s ability to perform this test adequately.

Cross References ▶ Cerebellum ▶ Tremor

References and Readings Dietrichs, E. (2008). Clinical manifestation of focal cerebellar disease as related to the organization of neural pathways. Acta Neurologica Scandinavica, 188 (Supplement), 6–11. Notermans, N. C., van Dijk, G. W., van der Graaf, Y., van Gijn, J., & Wokke, J. H. (1994). Measuring ataxia: Quantification based on the standard neurological examination. Journal of Neurology, Neurosurgery, & Psychiatry, 57, 22–26. Swaine, B. R., Desrosiers, J., Bourbonnais, D., & Larochelle, J. L. (2005). Norms for 15- to 34-year-olds for different versions of the finger-to-nose test. Archives of Physical Medicine and Rehabilitation, 86, 1665–1669.

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Fingertip Number-Writing Perception

Fingertip Number-Writing Perception J OHN E. M EYERS Private Practice, Neuropsychology Mililani, Hawaii, USA

Synonyms Fingertip writing

each group performed best with the hand ipsilateral to the lesion (Brown et al., 1989). Fingertip Number Writing Perception is more closely correlated with IQ than is Tactile Finger Recognition (Fitzhugh, Fitzhugh, Reitan, 1962). A number of mistakes are usually expected in persons of lower general intelligence. This test has also been found to be useful with children (Reitan & Wolfson, 2003).

Cross References ▶ Halstead-Reitan Neuropsychological Battery

Description References and Readings The examiner ‘‘writes’’ with a pencil each of the numbers 3, 4, 5, and 6 in a specific order on the fingertips of each of the examinee’s hand. There are a total of 20 trials for each hand.

Historical Background This test is a modified version of a similar version that was part of Ward Halstead’s original test battery. Ralph Reitan formalized the present version of these neurological procedures for the Halstead-Reitan Neuropsychological Battery (Reitan & Wolfson, 1985).

Brown, G. G., Spicer, K. B., Robertson, W. M., et al. (1989). Neuropsychological signs of lateralized arteriovenous malformations: Comparisons with ischemic stroke. The Clinical Neuropsychologist, 3, 340–252. Fitzhugh, K. B., Fitzhugh, L. C., & Reitan, R. M. (1962). The relationship of acuteness of organic brain dysfunction to Trail Making Test performances. Perceptual and Motor Skills, 15, 399–403. Harley, J. P., & Grafman, J. (1983). Fingertip number writing errors in hospitalized non-neurologic patients. Perceptual and Motor Skills, 56, 551–554. Reitan, R. M., & Wolfson, D. (1985). The Halstead-Reitan neuropsychological test battery. Tucson, AZ: Neuropsychology Press. Reitan, R. M., & Wolfson, D. (2003). The significance of sensory-motor functions as indicators of brain dysfunction in children. Archives of Clinical Neuropsychology, 18, 11–18.

Psychometric Data Aside from scoring the errors for each task, Fingertip Number Writing Perception is not scored. The purpose is to identify abnormalities of sensory perception rather than derive a score along a scaled continuum.

Fingertip Writing ▶ Fingertip Number-Writing Perception

Clinical Uses The basic information gained from administering this test is the differences in performances on the two sides of the body. When one hand perception is significantly impaired compared to the other, inferences regarding contralateral parietal lobe damage or dysfunction can be inferred. Normal subjects are more accurate with their left handed fingers versus their right, and the three middle fingers are more sensitive than the other two (Harley & Grafman, 1983). Patients with right hemisphere strokes make fewer errors than those whose strokes were on the left; however,

FIQ ▶ Fibromyalgia Impact Questionnaire

FIT ▶ Rey 15 Item Test

Fixed Battery

Fixed Battery J OHN E. M EYERS Private Practice, Neuropsychology Mililani, Hawaii, USA

Synonyms Standardized battery

Definition A fixed battery is a set of neuropsychological tests defined within a structure or conceptual framework. The battery of tests is given as a whole unit. The battery of tests usually has an understood and defined relationship of test scores, and a standardized interpretation approach. In this approach the same battery of standardized tests is used with each patient assessed; although different batteries of standardized tests may be used with different groups based on age or some other characteristic.

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The fixed battery approach to neuropsychological assessment has a number of strengths. First, the fixed approach allows for a common metric and comparison of performance across tests that are included in the fixed battery of tests. Second, a fixed battery allows for a known sensitivity, hit rates, and the calculation of Positive Predictive Power or Negative Predictive Power. One example of this is the Global Neuropsychological Deficits Scale (GNDS) (Reitan & Wolfson, 1993) from the HRB. Third, a fixed battery has the advantage of being able to compare test performance over time. With a fixed battery, one can track changes overtime using direct test re-test comparisons. Fourth, with consistent tests and administration, different evaluators can use common interpretive rules to get similar interpretive results. Thus, a fixed battery usually will have better reliability and consistency across different neuropsychologists. The fixed battery approach also has a number of weaknesses. First, this approach assumes that one combination of tests is able to assess all or the majority of patients (one size fits all). Second, a fixed battery does not adapt to new tests or testing approaches as neuropsychological science progresses. Third, a fixed battery often needs to be supplemented with other tests to provide answers to specific referral questions.

Historical Background Two of the most widely known fixed batteries of neuropsychological tests are the Halstead-Reitan Neuropsychological Battery (HRNB; Reitan & Wolfson, 1985) and the Luria Nebraska Neuropsychological Battery (Golden, Purisch, &Hammeke, 1985). More recently published are the Neuropsychological Assessment Battery (NAB) (Stern & White, 2003) and the NEPSY (Korkman et al., 1998).

Cross References ▶ Flexible Battery ▶ Halstead-Reitan Neuropsychological Test Battery ▶ Luria Nebraska Neuropsychological Battery ▶ Neuropsychological Assessment Battery ▶ NEPSY-II

References and Readings Current Knowledge The term ‘‘Fixed Battery’’ refers to a clearly defined standalone battery of tests which does not provide for ‘‘flexible’’ ad hoc addition of tests to its conceptual/analytical method, (e.g., the HRNB; Reitan & Wolfson, 1985). According to Sweet et al. (2002 and 2003), only a small percentage (i.e., 15%) of neuropsychological practitioners reported using a fixed battery approach. In contrast, the vast majority of clinicians reported using a flexible battery approach by which each clinician establishes a core set of tests that can be used to address most referral questions and then supplements the core set or reduces the number of tests depending on the specific examinee and referral question.

Golden, C. J., Purisch, A. D., & Hammeke, T. A. (1991). Luria-Nebraska neuropsychological battery: Forms I and II. Los Angeles: Western Psychological Services. Korkman, M., Kirk, U., & Kemp, S. (1998). NEPSY: A developmental neuropsychological assessment manual. San Antonio, TX: The Psychological Corporation. Reitan, R. M., & Wolfson, D. (1985). The Halstead-reitan neuropsychological test battery. Theory and clinical interpretation. Tucson, AZ: Neuropsychology Press. Reitan, R. M., & Wolfson, D. (1993). The Halstead–Reitan neuropsychological test battery. Theory and clinical interpretation (2nd ed.), Tucson, AZ: Neuropsychology Press. Stern, R. A., & White, T. (2003). Neuropsychological assessment battery: Administration, scoring, and interpretation manual. Lutz, FL: Psychological Assessment Resources. Sweet, J., Peck III, E. A., Abramowitz, C., & Etzweiler, S. (2002). National Academy of Neuropsychology/Division 40 of the American

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Psychological Association practice survey of clinical neuropsychology in the United States: Part I: Practitioner and practice characteristics, professional activities, and time requirements. The Clinical Neuropsychologist, 16(2), 109–127. Sweet, J., Peck III, E. A., Abramowitz, C., & Etzweiler, S. (2003). National Academy of Neuropsychology/Division 40 of the American Psychological Association practice survey of clinical neuropsychology in the United States: Part II: Reimbursement experiences, practice economics, billing practices, and incomes. Archives of Clinical Neuropsychology, 18(6), 557–582.

Fixed Pupils J EFFERY S AMUELS North Broward Medical Center Deerfield Beach, FL, USA

Definition One of the most commonly demonstrated findings in severe brain dysfunction is the appearance of ‘‘fixed and dilated’’ pupils. The pupillary opening becomes very large and no longer constricts when stimulated with a flashlight.

Current Knowledge It is important to know that ‘‘fixed and dilated pupils’’ can also be seen in other conditions that do not necessarily imply permanent damage and may even be reversible. Trauma to the eye can lead to atrophy of the iris, rupture of the sphincter, or scarring in the eye and pupillary dilatation.

Fixed Pupils. Figure 1 Fixed and dilated pupils

A condition called ‘‘Adies Tonic Pupil’’ is a pupillary abnormality characterized by a poor pupillary light reaction, reduced accommodation, and an enhanced pupillary response to near effort that results in a prolonged, ‘‘tonic’’ constriction, and slow pupillary redilation. This condition is seen in females more than males and is usually idiopathic. It can also be seen in viral infections and some neuropathies and is usually associated with reduced or absent deep tendon reflexes. Acute Closed Angle Glaucoma can lead to dilated pupils and is associated with edema of the corneas and increased intraocular pressure. The affected person develops eye pain, blurred vision, sees haloes around objects and may become nauseated. There are many chemicals and medicines that can lead to temporary pupillary dilatation. Atropine and scopolamine are common medicines that can do this. Quinine, tricyclics, amphetamines, cocaine and some poisons can cause this dilatation especially when in excess. There are also some medical conditions that can mimic this syndrome. Syphilis, diabetes, brainstem encephalitis, multiple sclerosis, hypothermia and guillain– barre polyneuropathy can lead to these changes and they can be reversible with recovery. Finally, a situation of potential urgency is damage and dysfunction of the third cranial nerve. Pressure on the nerve from swollen brain tissue damages the fibers of the nerve that constrict the pupil and leave the dilating fibers unopposed. Early recognition of this can lead to effective treatment. Though ‘‘fixed and dilated’’ pupils are often considered indicative of brain death, this is not always the situation and to search for treatable and reversible conditions is imperative (Fig. 1).

Flexible Battery

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Cross References


▶ Brain Death ▶ Pupillary Light Response

The concept of a ‘‘flexible’’ assessment approach to neuropsychological testing is not new. Early writers and teachers presented and described a flexible approach as early as 1951 (Shapiro, 1951). Early pioneers in the field of neuropsychology (e.g., Benton, 1977; Lezak, 1976) also advocated a flexible approach to assessment. In a survey of APA Division 40 members, Sweet, Nelson, and Moberg (2006) found that only 7% of the responding neuropsychologists used a fixed battery of tests such as the Halstead–Reitan Neuropsychological Battery (HRNB). Seventy-six percent of the responding neuropsychologists reported using a flexible battery approach and 18% used a ‘‘totally flexible’’ approach.

References and Readings http://wrongdiagnosis.com/sym/dilated_pupils.htm Moeller, J. J., & Maxner, C. E. (2007). The dilated pupil: an update. Current Neurology and Neuroscience Reports, 7(5):417–422. Thomas, P. D. (2000). The differential diagnosis of fixed dilated pupils: A case report and review. Critical Care and Resuscitation, 2, 34–37. Tien, H. C., Cunha, J.R., Wu, S. N., Chughtai, T., Tremblay, L. N., Brenneman, F. D., et al. (2006). Do trauma patients with a Glasgow Coma Scale score of 3 and bilateral fixed and dilated pupils have any chance of survival? The Journal of Trauma, 60(2), 274–278.

Current Knowledge

Fixed-Flexible or Semi-flexible ▶ Flexible Battery

Flexible Battery J OHN E. M EYERS Private Practice, Neuropsychology Mililani, Hawaii, USA

Synonyms Ability focused; Core battery; Fixed-flexible or Semiflexible

Definition The flexible battery approach to neuropsychological evaluation allows the clinician the freedom to select tests that are preferred to answer referral questions for a given examinee in a given evaluation context. Unlike the ‘‘defined’’ structure of a Fixed Battery approach, there is no empirically defined over-riding structure that organizes or unifies the flexible battery. Some flexible batteries use a core battery of tests around which other flexible tests are added (i.e., Meyers Neuropsychological Battery).

In common parlance, a ‘‘flexible battery’’ is that which is not a ‘‘fixed battery.’’ The selection of tests is idiosyncratic to the clinician. With a flexible battery, neuropsychologists have a fairly stable ‘‘core’’ selection of tests and then on an ‘‘ad hoc’’ basis add additional tests deemed beneficial for elaboration of the particular case and selected during administration. Examples of this approach are the Expanded Halstead–Reitan Battery (HRNB) (Heaton, Miller, Taylor, & Grant, 2004) and the Meyers Neuropsychological Battery (MNB) (Meyers & Rohling, 2004), and the Halstead–Russell Neuropsychological Evaluation System (Russell & Starkey, 2001). Although many neurophysiologists use a core battery of tests that they feel comfortable with, the core batteries tend not to have been standardized as a unit, but rather are assembled based on the preferences of the neuropsychologist and the clinical question being investigated. Flexibility exists in the tests selected, though the administration is based on a standardized approach to the test administration. In addition to the flexible battery approach, some clinicians use what has variously been termed a flexible approach, process approach, or totally flexible approach. In this flexible approach, a clinician may choose to administer all or part of a particular fixed battery of tests, or may choose to ‘‘test the limits’’ of performance by forgoing standardization guidelines and may as an example allow additional time to perform a particular test. An example of the flexible approach is the Boston Process Approach to Neuropsychological Assessment (Milberg, Hebben, & Kaplan;

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1996). In this approach, the standard administration process may be adhered to or discarded. The strengths of this approach are as follows: (a) the approach is highly flexible, so that nearly any procedure or approach the clinician wishes to use can be incorporated into the assessment; (b) the approach capitalizes on the individual clinician’s ability to hypothesize different types of impairment and allows for hypothesis testing. Weaknesses of this approach include: (a) a strong dependence on the individual clinician’s clinical observation skills; (b) the relationship of the tests (i.e., correlations, priming effects) of the individual tests with the other tests in the flexible battery are usually unknown; (c) test selection is based on clinical intuition and experience and may be difficult to replicate or repeat for test–retest situations; (d) the assessment process is a ‘‘moving target’’ and may not follow standardization protocols.

Larabee, G. J., Millis, S. R., & Meyers, J. E. (2008). Sensitivity to Brain Dysfunction of the Halstead Reitan vs. an Ability-Focused Neuropsychological Battery. The Clinical Neuropsychologist, 22(5), 813–825. Lezak, M. (1976). Neuropsychological assessment. New York: Oxford University Press. Meyers, J. E., & Rohling, M. L. (2004). Validation of the Meyers short battery on mild TBI patients. Archives of Clinical Neuropsychology, 19, 637–651. Russell, E. W., & Starkey, R. I. (2001). Halstead, Russell neuropsychological evaluation system-revised [Manual and Computer Program]. Los Angeles: Western Psychological Services. Shapiro, M. B. (1951). An experimental approach to diagnostic psychological testing. Journal of Mental Science, 97, 748–764. Sweet, J. J., Nelson, N. W., & Moberg, P. J. (2006). The TCN/AACN 2005 ‘‘Salary Survey’’: Professional practices, beliefs, and incomes of U.S. neuropsychologists. The Clinical Neuropsychologist, 20, 325–364. Volbrecht, M. E., Meyers, M. E., & Kaster-Bundgaard, J. (2000). Neuropsychological outcome of head injury using a short battery. Archives of Clinical Neuropsychology, 15, 251–265.

Future Directions

Flexor Posturing Clearly, the current trend in neuropsychology is the use of the flexible battery approach. The benefits of the flexible battery approach are that adaptations can be made for regional and specific clinical questions. This makes the flexible battery adaptable to future needs. The shortcomings of the flexible battery approach are the inconsistency and the difficulty with replication. Future directions for the use of a flexible battery will probably entail a core of tests around which more flexible tests will be administered and integrated into the interpretation process; one such newer battery of tests is the MNB.

▶ Decorticate Posturing

Flight of Ideas N ATALIE C. B LEVINS Indiana University Hospital Indianapolis, IN, USA

Cross References

Definition

▶ Fixed Battery

Rapid succession of fragmentary thoughts or speech in which content changes abruptly; speech may be incoherent; switching conversation topic mid-sentence or inappropriately; most commonly seen in mania.

References and Readings Benton, A. L. (1977). Psychological testing. In A. B. Baker & L. H. Baker (Eds.), Clinical neurology. New York: Harper & Row. Heaton, R. K., Miller, S. M., Taylor, M. J., & Grant, I. (2004). Revised comprehensive norms for an expanded halstead-reitan battery: Demographically adjusted neuropsychological norms for African American and Caucasian adults. Lutz, FL: Psychological Assessment Resources, Inc.

References and Readings Sadock, B. J., & Sadock, V. A. (2007). Kaplan & Sadock’s Synopsis of Psychiatry: Behavioral Sciences/Clinical Psychiatry, 10th Ed. Lippincott Philadelphia, PA: Williams & Wilkins.

Fluent Aphasia

Floor Effect

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Fluent Aphasia

S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

LYN T URKSTRA University of Wisconsin-Madison Madison, WI, USA

Synonyms

Synonyms

Floor level

Receptive aphasia; Sensory aphasia

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Short Description Definition A floor effect occurs when test items are so difficult that examinees are unable to perform well on the least challenging items on a test.

Current Knowledge When a floor effect occurs, it is difficult to compare a single individual’s performance relative to the performance in the standardization sample, given that the lowest level of actual performance possible in the standardization sample is difficult to detect. This can occur if the construct has not been defined or operationalized in such a way that lower levels of intellect or ability are able to be assessed. A significant risk of floor effects is the possibility of committing a false-positive error. An examinee who performs relatively poorly on a test is very likely to be considered as having impairment to some degree in the domain being assessed, even if the impairment is not there in actuality.

A group of aphasia subtypes characterized by fluent speech, typically with impaired language comprehension and repetition. Utterance length is normal or extended. The patient’s spontaneous speech has normal prosody and grammar and is produced with normal effort. There is a reduction in content words, and paraphasic errors are common. Reading and writing typically mirror auditory comprehension and oral production.

Categorization The fluent aphasias include Wernicke’s aphasia, transcortical sensory aphasia, conduction aphasia, and anomic aphasia. In all cases, spoken language is fluent with normal prosody and grammar, and is produced with normal effort. The subtypes are distinguished by the patient’s ability to understand and repeat language, as shown in the Table 1.

Natural History, Prognostic Factors, and Outcomes The prognosis for recovery of functional communication in individuals with fluent aphasia depends on the

Cross References ▶ Ceiling Effect

Fluent Aphasia. Table 1 Comprehension Repetition Wernicke’s aphasia

Impaired

Transcortical sensory Impaired aphasia

Floor Level ▶ Floor Effect

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Severely impaired Normal

Conduction aphasia

Relatively intact

Severely impaired

Anomic aphasia

Relatively intact

Moderately impaired

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underlying cause of the aphasia, as well as factors such as the size of lesion and the patient’s age, premorbid language skills, and comorbid health conditions. Individuals who initially present with Wernicke’s aphasia often evolve to a clinical profile of conduction or anomic aphasia, with relatively good auditory and reading comprehension and deficits primarily in word finding and the comprehension and production of complex syntax. Language comprehension typically recovers before production, most likely because comprehension is represented bilaterally in the brain. Transcortical sensory aphasia typically has a rapid recovery when caused by acute vascular etiologies, and is rarely seen in patients after the first few days post-stroke.

with an acquired communication disorder (and his or her caregivers). Fluent aphasia may be misdiagnosed as psychosis if the patient’s fluent, incomprehensible verbal output is confused with the ‘‘word salad’’ of schizophrenia. As the typical site of lesion is posterior to most cortical and subcortical motor structures, individuals with fluent aphasia often have normal motor function, supporting the diagnosis of a psychiatric etiology. Schizophrenic patients, however, do not produce errors at the level of phonology (e.g., phonemic paraphasic errors), although word substitutions (semantic paraphasias) and atypical grammar may be observed (Marini et al., 2008). The most salient language impairments in schizophrenia are at the macrolinguistic level, that is, in the coherent and organized expression of thoughts (Marini et al., 2008).

Neuropsychology and Psychology of Fluent Aphasias Evaluation According to the classical model of aphasia (see the entry for Wernicke–Lichtheim model of aphasia), fluent aphasias are associated with lesions in the posterior portion of the language-dominant hemisphere (Brodmann’s areas 22, 42, 39, and 40). Transcortical sensory aphasia is associated with lesions that spare the core perisylvian regions, but effectively disconnect them from other temporal and occipital lobe regions that are critical for language comprehension. A common psychological problem associated with stroke is depression (Carson et al., 2000). While there is a general belief that individuals with nonfluent aphasia are more likely to experience depression than those with fluent aphasia, a systematic review of the literature did not support this pattern (Carson et al., 2000). However, the true incidence and prevalence of depression are difficult to determine, because of significant limitations in the instruments used in the diagnosis (Turner-Stokes & Hassan, 2002), notably the demands on language. This is particularly likely for patients with fluent aphasia, who might not understand the examiner’s questions, particularly when they include abstract words such as ‘‘feelings’’ or ‘‘coping.’’ Fluent aphasia may be accompanied by anosognosia (unawareness of deficits), so depression might be related to frustration with others’ behaviors and imposed restrictions, rather than perceived deficits associated with aphasia. Over the long term, aphasia carries the risk of social isolation (Holland, 2007), and stroke survivors with aphasia are likely to have other negative life changes such as unemployment (Naess, Hammersvik, & Skeie, 2009). Thus, depression should be considered in any individual

As with other aphasia types, individuals with fluent aphasias typically are evaluated using a combination of standardized language tests and careful observation of extemporaneous communication. The tests and measures used depend on the goals of the assessment (e.g., diagnosis vs. prediction of functional performance vs. treatment planning), the time post-onset (e.g., comprehensive test batteries are not appropriate in the context of acute stroke), and the patient’s clinical presentation.

Treatment While there are many treatments for nonfluent aphasia, there are relatively a few designed specifically for fluent aphasia, other than treatments for anomia. The reasons vary depending on the aphasia type. Transcortical sensory aphasia typically recovers quickly, and thus often does not require specific treatment beyond conventional therapies that are appropriate for aphasia in general. Conduction aphasia, likewise, is treated with conventional aphasia therapies aimed at improving word finding in functional communication contexts (repetition is not the target of treatment unless it is a specific need for the patient). Treatment of Wernicke’s aphasia also aims to improve communication in context, with an emphasis on the treatment of auditory comprehension. There are many evidence-based treatments for word-finding problems in anomic aphasia and also in

Fluorodeoxyglucose Positron Emission Tomography

nonfluent aphasias. These are discussed under individual aphasia types.

Cross References ▶ Anomic Aphasia ▶ Aphasia Tests ▶ Conduction Aphasia ▶ Paraphasia ▶ Transcortical Sensory Aphasia ▶ Wernicke–Lichtheim Model of Aphasia

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Definition Fluorodeoxyglucose is an analog of glucose. The full chemical name is 2-flouro-2-deoxy-D-glucose(FDG). FDG is commonly used in the medical imaging modality positron emission tomography(PET). The fluorine in the FDG molecule is made into radioactive 18 F in a cyclotron. After FDG is injected into a patient, a PET scanner can form images showing the distribution of FDG around the body. The images are then interpreted by a nuclear medicine physician or radiologist to provide diagnoses of various medical conditions.

References and Readings Mechanism of Action Carson, A. J., MacHale, S., Allen, K., Lawrie, S. M., Dennis, M., House, A., et al. (2000). Depression after stroke and lesion location: A systematic review. Lancet, 356(9224), 122–126. Chapey, R. (Ed.). (2001). Language intervention strategies in aphasia and related neurogenic communication disorders (4th ed.). Philadelphia, PA: Lippincott, Williams & Wilkins. Goodglass, H. (1993). Understanding aphasia. San Diego: Academic Press. Holland, A. L. (2007). Counseling in communication disorders: A wellness perspective. San Diego: Plural Publishing. Marini, A., Spoletini, I., Rubino, I. A., Ciuffa, M., Bria, P., Martinotti, G., et al. (2008). The language of schizophrenia: An analysis of micro and macrolinguistic abilities and their neuropsychological correlates. Schizophrenia Research, 105(1–3), 144–155. Naess, H., Hammersvik, L., & Skeie, G. O. (2009). Aphasia among young patients with ischemic stroke on long-term follow-up. Journal of Stroke and Cerebrovascular Diseases, 18(4), 247–250. Turner-Stokes, L., & Hassan, N. (2002). Depression after stroke: A review of the evidence base to inform the development of an integrated care pathway. Part 1: Diagnosis, frequency and impact. Clinical Rehabilitation, 16(3), 231–247.

FDG competes with glucose in the hexokinase reaction where glucose becomes glucose-6-phosphate. FDG-6phosphate is trapped in the cell because, unlike glucose, it does not undergo glycolysis, and does not enter the fructosepentose shunt or glycogen synthesis pathway. Therefore the transmembranous exchange of glucose can be shown through cellular FDG uptake. Scans with FDG produce a measure of glucose metabolism rather than blood flow. The fluorine at the 20 position inhibits FDG from metabolic degradation or use. Once FDG decays radioactively, it is metabolized normally as glucose because its fluorine is converted to 18O, and after picking up a H+ from the environment, it becomes glucose-6-phosphate labeled with harmless nonradioactive ‘‘heavy oxygen’’ (oxygen-18) at the 20 position.

Procedure

Fluent Segmental Paraphasia ▶ Literal Paraphasia

Fluorodeoxyglucose Positron Emission Tomography F LORA H AMMOND, LORI G RAFTON Carolinas Rehabilitation Charlotte, NC, USA

A dose of FDG in solution(typically 5–10 millicuries or 200–400 MBq) is injected rapidly into saline drip running into a vein. The patient must have fasted for at least 6 h in order to have a low enough blood sugar to allow FDG to be taken up. The patient must then wait approximately one hour for the sugar to distribute and be taken up into organs which use glucose. Physical activity should be minimized to prevent uptake of the radioactive sugar into muscles as this will cause artifact. The patient is then placed in the PET scanner for a series of one or more scans, which may take from 30 to 40 min each.

Current Knowledge Synonyms FDG PET

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In PET imaging, 18F-FDG can be used for the assessment of glucose metabolism throughout the body. FDG-PET is

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Fluoxetine

useful in imaging bronchial carcinoma, colorectal cancer, esophageal and gastric cancers, testicular, breast and other gynecological cancers, ENT cancers, lymphomas, melanomas, and sarcomas. It is less useful in most prostate cancers and multiple myeloma due to decreased uptake of FDG.

5HT2C receptors, which results in the increase of presynaptic catecholamine production and mildly inhibits Norepinephrine transport.

Indication Cross References ▶ PET

Major depressive disorder, obsessive–compulsive disorder, premenstrual dysphoric disorder, Bulimia Nervosa, panic disorder, bipolar disorder, bipolar depression (in combination with Zyprexa in the form of Symbyax).

References and Readings Off Label Use http://en.wikipedia.org/wiki/Fluorodeoxyglucose GE Health page on FDG (http://www.medcyclopaedia.com/library/ topics/volume_i/f/fluorodeoxyglucose_fdg_.aspx?s=Fluorodeoxyglu cose&scope=&mode=1.) Silver, J. M., McAllister, T. W., & Yudofsky, S. C. (2005). Textbook of traumatic brain injury. Washington, DC: American Psychiatric Publishing, Inc.

Social anxiety disorder, Post-traumatic stress disorder.


Fluoxetine J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Fluoxetine

Seizures, mania, and suicidal ideation (all rare)


Brand Name


Prozac, Prozac Weekly, Serafem

Physicians’ Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson PDR. Stahl, S. M. (2007). Essential psychopharmacology: the prescriber’s guide (2nd ed.). New York, NY: Cambridge University Press.

Class Serotonin-specific reuptake inhibitor

Proposed Mechanism(s) of Action Blocks the presynaptic serotonin reuptake pump and desensitizes serotonin receptors. Theoretically increases serotonin neurotransmission. Fluoxetine antagonizes


Flynn Effect

Fluphenazine J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metarie, LA, USA

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Additional Information Generic Name Fluphenazine

Brand Name


Prolixin

Class Conventional Antipsychotic

Flynn Effect J AMES R. F LYNN The University of Otago Dunedin, New Zealand

Proposed Mechanism(s) of Action Blocks dopamine-2 receptors.

Synonyms

Indication

Generational IQ gains; IQ gains over time; Secular IQ gains

Psychotic disorders.

Definition Off Label Use Bipolar disorder.

Side Effects Serious Agranulocytosis, jaundice, seizures, and neuroleptic malignant syndrome.

Common Akathisia, extrapyramidal symptoms, neuroleptic-induced deficit syndrome, amenorrhea, galactorrhea, priapism, dizziness, hypotension, tachycardia, syncope, and weight gain.

The Flynn effect refers to the fact that for every developed nation for which data exist (almost 30), there have been massive IQ gains from one generation to the next during the twentieth century. These gains have averaged, with variation by nation and kind of test, about 9 points per generation culminating in a huge gain over 100 years.

Historical Background There is a correspondence between IQ gains and the beginning of modernity. Data by birth date show that British gains in whatever IQ tests measure began no later than the last decades of the nineteenth century at a time when, paradoxically, IQ tests did not exist. There are now data for most of continental Europe, virtually all English-speaking nations, three nations of predominately

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European culture (Israel, Brazil, and Argentina), and three Asian nations that have adopted European technology (Japan, China, and Korea). In 1985, Sweden may have become the first nation to register losses on a test of spatial visualization. Scandinavia looks as if its gains have been lower than most, perhaps because its countries offer no data from the first half of the twentieth century and perhaps because highly advanced nations are entering a time in which gains will cease. However, the UK, the USA, and Argentina show no sign of this. Recent data show three developing nations beginning to enjoy gains, namely, rural Kenya, Dominica, and the Sudan. Data covering the last 60 years reveal a pattern. Gains are largest on tests that purport to be the purest measures of intelligence, that is, tests of on-the spot problem solving that assume no previous knowledge. The best example is Raven’s Progressive Matrices, whose items require identifying the missing parts of designs presumed to be easily assimilated by people across a wide variety of cultures (Fig. 1). The Wechsler Intelligence Scale for Children (the WISC) and the Stanford-Binet differ from the Raven’s test in that they also ask questions about the kind of knowledge an acute mind normally acquires in an industrialized society. For example, the WISC includes Vocabulary, General Information, and Arithmetic among its ten subtests. As Fig. 2 shows, although gains

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1952

have occurred on all subtests, they are lowest for these three.

Current Knowledge Until recently, failure to make a key distinction clouded our understanding of IQ gains. The primary purpose of IQ tests is to measure differences between individuals for the kind of intelligence valuable in an industrializing society. However, IQ trends over time do not measure intelligence differences between people today and people in 1900. Rather they are historical artifacts that provide the raw material for a history of cognition in societies like the USA and UK in the twentieth century. Imagine that an archeologist from the distant future excavates our civilization and finds a record of performances over time on measures of marksmanship. These tests show many bullets soldiers could put in a target 100 m away in 1 min. Records from 1865 (the US Civil War) show best scores of 5, records from 1898 (Spanish-American War) show 10, and records from 1918 (World War I) show 50. The gains seem far too huge to constitute gains in marksmanship skills over 53 years. Then the archeologist discovers battlefields specific to each time: the 1865 battlefields show the presence of non-repeating rifles, the 1898

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IQ SCORES

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Flynn Effect. Figure 1 Five nations and matrices tests: rates of IQ gain compared. Every nation is normed on its own samples. Therefore, the fact that the mean IQ of one nation appears higher than another at a given time is purely an artifact

Flynn Effect

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Flynn Effect. Figure 2 America 1947–1948 to 2002: IQ gains on WISC subtests. An estimated gain for Ravens’ has been added based on Fig. 1

ones of repeating rifles, and the 1918 ones of machine guns. This explains why it was easier to get more bullets into the target over time and confirms that the score gains were not a measure of enhanced ‘‘marksmanship.’’ But they were of enormous historical and social significance: battle casualties, the industries needed to arm the troops, and so forth altered dramatically.

Minds Now and Then The important thing about IQ gains over time is not whether they constitute enhanced ‘‘intelligence’’ but to recognize that since 1900, people have acquired new habits of mind as powerful as machine guns in allowing them to solve certain cognitive problems (Flynn, 2009). The current generation is so much better on Raven’s that if the people of 1900 were measured against them, they would have a mean IQ of 50. To equate such a score, difference between the generations as an intelligence difference is absurd. The difference on the Similarities subtest of the WISC is equally large. Similarities ask the subject to classify things, for example, what do dogs and rabbits have in common? As a key to what really changed over the last century, in the 1920s, Luria (1976) interviewed rural Russians largely untouched by modernity. He asked them to classify and to do logical inference. Fish and crows. Question: What do a fish and a crow have in common? Answer: Nothing. A fish – it lives in water. A crow flies. If the fish just lies on top of the water, the crow could peck at it. A crow can eat a fish but a fish can’t eat a crow. Camels and Germany. Question: There are no camels in Germany; the city of B is in Germany; are there camels there or not? Answer: I don’t know, I have never seen German cities. If B is a large city, there should be camels

there. If B is a small village, there is probably no room for camels. These examples show people three generations ago struggling with both classification (as on the Similarities subtest) and using logic in a hypothetical context, one removed from a real-world problem (as on Raven’s). Their minds were ‘‘handicapped’’ by the fact that they have on ‘‘utilitarian spectacles.’’ The important thing for them was to manipulate the world to their advantage. This meant focusing on the differences between objects and demanding that descriptions of concrete reality be based on evidence. Over the past century people have gradually put on scientific spectacles. Today, people in developed nations still want to manipulate the concrete world, of course. But they are also open to ignoring the specificity of objects in favor of classifying them using abstract categories. Take the Similarities-type item: What do dogs and rabbits have in common. A schoolchild in 1900 would say, ‘‘You use dogs to hunt rabbits.’’ He or she might know the ‘‘right’’ answer, ‘‘they are both mammals,’’ but would not imagine that anyone could want something so trivial as a response. The important thing is what they are used for. In 2000, schoolchildren find the correct response perfectly natural. They have a new habit of mind: that it is important to classify the world in terms of abstract categories as a prerequisite to understanding it. Take Raven’s-type items all of which involve using logic to perceive sequences in a series of abstract shapes. In 1900, schoolchildren found such an application of logic alien. Today, children are habituated to it.

Significance of IQ Gains These new habits of mind are far from trivial. The modern child has a mind ready to accept the scientific mode of

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acquiring knowledge and is more apt to look behind reality for explanations. This change is reflected in the very schools our children attend. The exams given 14-year olds early in the twentieth century ask for socially valued knowledge: What are the capitals of the 48 states? Exams late in the century ask for general explanations: Why is the largest city of a state rarely the capital? (Because rural dominated state legislature hated the big city and located the capital in some rural center.) Unlike Luria’s pre-modern people, people today are willing to transcend the concrete world of experience and use logic to analyze hypothetical situations. In 1900, despite an IQ of 50 against current norms, people were not mentally retarded in the sense that someone who gets an IQ of 50 today would be. Raven’s and Similarities items were simply foreign to their habits of mind. Acquiring new habits of mind is not something that can be done on the spot, in a sort of eureka moment. It is matter of gradual assimilation like any other habit, as anyone who has taken up cross word puzzles knows. It takes time to break the habit of using words in their common meaning as simply as possible, and to develop habits like looking for other meanings, being alert to a word commonly used as a noun being used as a verb, and so forth. Taking logic and the hypothetical seriously is important. All moral debate begins with the hypothetical: ‘‘What if your skin turned black?’’ A literal response ends the argument: ‘‘That is crazy – who do you know whose skin has ever turned black?’’ Better political debate means rejection of the anecdotal. The Congressional Record shows that Congressmen in 1918 were quite capable of saying ‘‘my wife says she does not want to vote and that is good enough for me.’’

IQ Gains and the Death Penalty Understanding the Flynn effect can be a matter of life or death. The US Supreme Court has held that those with an IQ of 70 or below cannot be executed for murder, subject to examination for mental retardation. They prefer IQs tested at school. In 2000, a man of 34 is convicted of murder. In 1978, he was tested at age 12 with the original WISC normed in 1948. That means that its standard for various IQs was set by the scores of a sample of children who were representative of a time 30 years before he was tested. Because of IQ gains over time, that standard was 9 points more lenient than the standard of his own time. Rather than getting an IQ of 68 (and living), he gets an IQ of 77 (and dies). Unless judges take the significance of IQ gains into account, death

becomes a lottery of whether someone happens to take a current or obsolete test.

Future Directions Linus Pauling reduced the properties of chemical molecules to the properties of the atoms of which they are composed. Imagine that brain physiology did something similar for both measured IQ differences between individuals and cognitive trends over time. Brain ‘‘pictures’’ now reveal exactly what differs from one brain to another both when people of various IQs are compared at a give time, and when a typical person in 1900 is compared to a typical person today. They actually predict which child in a classroom will do better than another on the WISC by using images of neurons, connections between neurons, blood supply feeding the neurons, the ‘‘spray’’ from dopaminergic neurons thickening neural connections with use, and various areas of the brain interacting. And they also ‘‘predict’’ the performance difference on Raven’s and Similarities in 1900 and 2000 by ‘‘seeing’’ thicker connections in the parts of the brain where the relevant habits of mind developed over time. Physiology would have completed its reductionist task and given everything one could hope to know: the brain physiology that underlies the psychology of intelligent problem solving. But note that it would not render other levels of knowledge redundant. Physiology might also catch the differences between the biological make-up of riflemen and machine gunners, including their musculature, sensations, and so forth. But it will never give us a working model of a machine gun or explain its social consequences. Brain physiology will clarify the problems set by IQ gains over time: how the brain adjusts when new habits of mind evolve, how some skills can escalate over time (Raven’s and Similarities) while others remain unaltered, such as arithmetical reasoning, size of vocabulary, fund of general information. But it can never replace cognitive history’s insights into the structure of the habits of mind acquired in the twentieth century. Or how they transformed our lives at school (ideas rather than facts), at work (more lateral thinking), at leisure (video games), or in moral and political debate.

Cross References ▶ Atkins v. Virginia ▶ Furman v. Georgia ▶ Intelligence

Focal Seizures

References and Readings Blair, C. (2006). How similar are fluid cognition and general intelligence? A developmental neuroscience perspective on fluid cognition as an aspect of human cognitive ability. Behavioral and Brain Sciences, 29, 109–160. Flynn, J. R. (2009). What is intelligence? Beyond the Flynn Effect (expanded paperback edition). New York: Cambridge University Press. Jensen, A. R. (1998). The g factor: The science of mental ability. Westport, CT: Praeger. Luria, A. R. (1976). Cognitive development: Its cultural and social foundations. Cambridge, MA: Harvard University Press.

FM ▶ Fugl-Meyer Assessment of Sensorimotor Impairment

FMA ▶ Fugl-Meyer Assessment of Sensorimotor Impairment

fMRI ▶ Functional Magnetic Resonance Imaging

FNQ ▶ Family Needs Questionnaire

Focal Lesion, Contusion B ETH R USH Mayo Clinic Jacksonville, FL, USA

Definition Focal lesions are circumscribed areas of injury to brain tissue following brain injury. Such lesions may be created when an object penetrates the skull and directly injures an area of the brain. In closed head injury, such lesions are

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usually associated with vascular damage, such as contusions or hemorrhages.

Current Knowledge In contrast to diffuse brain injuries, such as diffuse axonal injury, focal lesions and focal contusions can result in isolated deficits of cognition, speech, sensory, and language functions depending on location. Contusions, or bruises, typically form in gray matter where there is a higher degree of vasculature than in white matter. Hemorrhages and hematomas may be subarachoid (SAH; between the dura and the skull), subdural (SDH; between dura and brain), epidural (EDH; associated with dural tears), or intracerebral (ICH; within brain tissue). Multiple focal lesions or contusions may result from diffuse vasculopathic processes that can cause a series of microhemorrhages or small leaks of blood from individual brain blood vessels. The presence of focal lesion or focal contusion is manifested in clinical presentation with a relatively specific symptom of speech, cognitive, motor, or sensory dysfunction. For example, a focal frontal lobe lesion or contusion may result in impaired executive function, or impaired speech fluency, depending on location and size. The neuropsychological profile of a patient with focal lesion or contusion is more likely to reveal isolated cognitive deficits rather than impairment in multiple domains of cognition. Focal contusion or lesion may also be indicated by a neuropsychological profile that reveals strong lateralization of left to right hemisphere dysfunction.

Cross References ▶ Cortical Contusion ▶ Epidural Hematoma ▶ Intracerebral Hemorrhage ▶ Subarachnoid Hemorrhage ▶ Subdural Hematoma

References and Readings Bigler, E. D. (2001). Archives of Clinical Neuropsychology, 16, 95–131. Ammerman, J. M., et al. (2006). Trauamtic intracranial hemorrhage. In R. W. Evans (Eds.), Neurology and trauma (2nd ed., pp. 156–66). Oxford.

Focal Seizures ▶ Partial Seizure (Simple)

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Focus

Focus ▶ Attention

Focused Attention A NNA M AC K AY-B RANDT Brown University Medical School Providence, RI, USA

Definition An aspect of attention that brings a select amount of information into conscious awareness.

Historical Background Fundamental research in attention emerged in the 1950s1956 with the rise of cognitive psychology and theories that involved process such as attention, memory, and perception that do not involve directly observable behavior (e.g., Miller, 1956; Broadbent, 1957).

Current Knowledge Focused attention has been described within the context of theories of attention, working memory, executive function, and consciousness. Baddeley (1993) proposed that the central executive in his model of working memory may function as Norman and Shallice’s (1986) Supervisory Attentional System, such that it generates higher level schemas that override lower level, automatic, or environmentally generated schemas to achieve internally produced goals. These temporary active schemas are held online within the episodic buffer and form the focus of attention. Some researchers further specify that only elements in working memory that are under the ‘‘focus of attention’’ can be consciously experienced, while there are some elements in working memory that are not contained within this focus and thus may capture some degree of attention but not conscious awareness (Cowan, 1988). In addition to higher-order cognitive control functions, focused attention plays a role in the early stages of

information processing. Focusing, along with filtering, and automatic shifting are thought to be important components involved in early sensory selection (Cohen, 1993). Attentional focusing also arises in higher-order sensory systems through motivational and responsemediating influences. Different forms of attention were defined in a clinical model proposed by Sohlberg and Mateer (1989). The model created a hierarchy of difficulty and suggested that recovery of function would progress from the simplest to the most complex forms. Focused attention, the ability to respond discretely to specific visual, auditory, or tactile stimuli, is considered the simplest form of attention. Sustained attention is the ability to maintain a consistent behavioral response over the course of an ongoing activity. Sustained attention is the capacity to maintain a behavioral or cognitive set in the context of distracting or competing stimuli. Alternating attention is the mental flexibility to shift the focus of attention between tasks with different cognitive requirements. Finally, divided attention was considered to be the most complex form of attention and was thought to be the ability to respond to multiple tasks or task demands.

Future Directions Neuroimaging and behavioral studies continue to refine an understanding of focused attention along with other aspects of attention by either confirming or questioning the theoretical fractionation of these cognitive processes. These findings may be used to inform applied research seeking to refine diagnoses11and to determine points of intervention for patient populations identified with deficits in forms of attention, such as those with schizophrenia, attention-deficit disorder, stroke, traumatic brain injury, and posttraumatic stress disorder.

Cross References ▶ Consciousness ▶ Executive Function ▶ Working Memory

References and Readings Baddeley, A. D. (1993). Working memory or working attention? In A. D. Baddeley & L. Weiskrantz (Eds.), Attention: Selection,

Ford v. Wainwright awareness, and control: A tribute to Donald Broadbent. Oxford, UK: Oxford University Press. Broadbent, D. E. (1957) A mechanical model for human attention and immediate memory. Psychological Review, 64, 205–215. Cohen, R. (1993). The neuropsychology of attention. New York: Plenum. Cowan, N. (1988). Evolving conceptions of memory storage, selective attention, and their mutual constraints within the human information processing system. Psychological Bulletin, 104, 163–191. Miller, G. A. (1956). The magical number seven, plus or minus two. Psychological Review, 63, 81–97. Norman, D., & Shallice, T. (1986). Attention to action: Willed and automatic control of behaviour. In R. J. Davidson, G. E. Schwartz, & D. E. Shapiro (Eds.), Consciousness and self-regulation: Advances in research and theory (Vol. 4, pp. 1–18). New York: Plenum. Sohlberg, M., & Mateer, C. A. (1989). Introduction to cognitive rehabilitation: Theory and practice. New York: Guilford Press.

Folling’s Disease ▶ Phenylketonuria

Foot Preference ▶ Lateral Dominance

Forced-Choice Test P HILIP S CHATZ Saint Joseph’s University Philadelphia, PA, USA

Definition A forced-choice test is one that requires the test-taker to identify or recognize a previously-presented stimulus by choosing between a finite number of alternatives, usually two. The forced choice format is useful when suboptimal effort is of concern to the examiner, and is based on the premise that even impaired individuals will not perform below ‘‘chance level’’ (e.g., 50% with two alternatives). Performance at, or below, chance raises suspicion of symptom exaggeration, suboptimal effort, or malingering. Researchers have show that forced choice recognition tests achieve a high ‘‘hit rate’’ in the detection of noncompliance.

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Cross References ▶ Malingering ▶ Portland Digit Recognition Test ▶ Recognition Memory ▶ Test of Memory Malingering ▶ Word Memory Test

References and Readings Frederick, R. I., & Speed, F. M. (2007). On the interpretation of belowchance responding in forced-choice tests. Assessment, 14(1), 3–11.

Ford v. Wainwright R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition In 1986 in Florida, Alvin Ford, the defendant, was convicted of a capital offense and was sentenced to death. During his tenure on death row, the issue of his mental health and competency to proceed surfaced. At the time of the conviction, Florida’s state procedure did not allow an adversarial or judicial determination of a defendant’s competency to be executed because the determination was made by the governor with the aid of a panel of mental health professionals. However, the US Supreme Court ruled that Florida’s competency procedure was unconstitutional and asserted that executing a person not competent as a result of mental health issues represented violation of the Eighth Amendment right to be free from cruel and unusual punishment. Moreover, it noted that of the states having death penalty legislation at the time, 26 statutes explicitly barred the execution of the ‘‘insane.’’ The Supreme Court ruled that executing someone who is not competent due to mental health issues: (1) has questionable retributive value, (2) has no deterrence value, and (3) simply affronts humanity. In this ruling, the US Supreme Court did not expand upon specific facets of competency to be executed beyond those laid out by the Dusky requirements. The court ruled that the person must possess the mental capacity to understand the nature of the death penalty and why it is utilized. Moreover, the

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defendant must understand the link between the crime and the punishment rendered.

Cross References ▶ Dusky v. United States, 1960 ▶ Estelle v. Smith (1981)

References and Readings Cunningham, M. D., & Goldstein, A. M. (2003). Sentencing determinations in death penalty cases. In A. Goldstein (Ed.), Handbook of psychology (Vol. 11). Forensic psychology. New Jersey: Wiley. Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Dusky v. U.S., 362 U.S. 402 (1960). Estelle v. Smith, 451 U.S. 454 (1981). Heilbronner, R. L., & Waller, D. (2008). Neuropsychological consultation in the sentencing phase of capital cases. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press.

Forebrain R ANDALL E. M ERCHANT Virginia Commonwealth University Medical Center Richmond, VA, USA

Definition The forebrain is the largest and most anterior of the three primary portions of the brain.

Current Knowledge The forebrain, composed of the diencephalon and telencephalon, is the largest and most anterior of the three primary portions of the brain. The diencephalon includes the thalamus, hypothalamus, limbic system, and associated structures, while the telencephalon forms the cerebrum. The cerebrum, which is divided into two cerebral hemispheres connected by the corpus callosum, includes the cerebral cortex, subcortical white matter, and basal ganglia.

Cross References ▶ Cerebrum ▶ Diencephalon ▶ Hypothalamus ▶ Limbic System ▶ Thalamus

Forensic Neuropsychologist R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition This refers to any neuropsychologist who offers opinions, with definable foreknowledge, about the psycholegal aspects of a legal case. A forensic neuropsychologist practices the application of neuropsychological assessment methods to the evaluation of criminal or civil litigants. The practice of forensic neuropsychology focuses the awareness of neuropsychologists on the critical areas of forensic practice that should be considered during each phase of a scientific neuropsychological examination/investigation. Forensic neuropsychologists provide important information to legal authorities (e.g., judges, attorneys) in cases where brain dysfunction is involved or alleged. They routinely rely upon standardized neuropsychological tests that measure various cognitive abilities and psychological states. Forensic neuropsychologists fulfill three potential roles: (1) fact witness, (2) expert witness, and (3) litigation consultant. A fact witness is a neuropsychologist who is limited to providing testimony centered on the facts about the patient: they are usually considered an advocate of their patient/client. In contrast, an expert witness is a neuropsychologist who provides facts but also is afforded the opportunity to give opinions and report hearsay; they are an ‘‘advocate of the facts.’’ A litigation consultant refers to a neuropsychologist who works ‘‘behind the scenes’’ to provide the attorney with education regarding basic neuropsychological terms and principles. A litigation consultation oftentimes reviews test data provided by another neuropsychologist and generates alternate theories for the neuropsychological facts and aids in the construction of questions to be utilized

Forensic Psychology

during the cross-examination neuropsychologist.

of

the

opposing

Cross References ▶ Expert Witness ▶ Forensic Neuropsychology ▶ Forensic Psychology

References and Readings Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis. Larrabee, G. J. (2005). A scientific approach to forensic neuropsychology. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Sweet, J. J. (1999). Forensic neuropsychology: Fundamentals and practice. Lisse: Swets & Zeitlinger.

Forensic Neuropsychology R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition In its most basic sense, forensic neuropsychology is characterized as the presentation of neuropsychological evidence to address legal questions. The neuropsychologist acts, with definable foreknowledge, with the understanding that the case is a forensic one, and the roles and requirements are clearly understood at the outset, and they are quite different from the neuropsychologist as treater role. Forensic neuropsychology typically includes cases involving: worker’s compensation, disability determinations, educational due process within public school systems, personal injury, criminal, child custody, impaired professional fitness for duty, competency, and other cases involving adversarial administrative and judicial determinations. According to Sweet (1999), the demand for neuropsychologists’ involvement in adversarial activities is a ‘‘natural outcome of the success of a strong scientist-practitioner orientation within the subspecialty of clinical neuropsychology.’’ This approach is both a

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cause and format for clinicians engaging in forensic neuropsychology. The approach is a cause in the sense that scientific gathering and objective behavioral methodologies create ongoing growth in an important knowledge area. Among the relevant by-products of a scientist–practitioner approach are: familiarity with disciplined scrutiny (i.e., peer review), clinical procedures emphasizing data-based decision-making (i.e., accountability), and comfort with hypothesis-testing (i.e., objective differential diagnosis). It is a format in the sense that related methodologies and data-based knowledge are paradigmatic in ongoing forensic activities.

Cross References ▶ Forensic Neuropsychologist ▶ Forensic Psychology

References and Readings Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney-neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Larrabee, G. J. (2005). A scientific approach to forensic neuropsychology. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: University Press. Sweet, J. J. (1999). Forensic neuropsychology: Fundamentals and practice. Lisse, the Netherlands: Swets & Zeitlinger.

Forensic Psychology N ATHALIE D E FABRIQUE Cook County Department of Corrections Chicago, IL, USA

Definition Forensic psychology is the application of psychology to the legal system. It focuses on the practice of clinical psychology and concerns the material provided in the judicial process evidenced by the compilation, evaluation, and presentation of psychological material.

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The practice of forensic psychology includes the appreciation for the laws that apply to the jurisdiction in which the case is concerned. In order for the forensic psychologist to integrate psychological findings into legal language, to testify, and to interact appropriately with judges, attorney, and legal professionals, a thorough understanding of the laws in each jurisdiction must be met. Additionally, in order to be considered a credible witness, one who engages in forensic psychology must demonstrate expertise in clinical psychology, as well as understand the rules and philosophy of the legal system. Knowledge and appreciation for the adversarial model is critical as that is the system in which the forensic psychologist functions. A lack of appreciation for the system will ultimately result in a loss of credibility in the courtroom. Typically, a forensic psychologist is not questioned about the practice of psychology. Rather they are asked legal questions and the court must understand the manner in which the response is given. For example, issues related to competency to stand trial, mental state at the time of the incident, treatment recommendations, and assessment of future risk are frequent topics evaluated by a forensic psychologist. Forensic psychologists also assist law enforcement in employee selection, training, and criminal profiling. Issues such as these are legal questions, not psychological ones but the forensic psychologist must structure the information into a legal context.

Cross References ▶ Forensic Neuropsychology ▶ Forensic Neuropsychologist ▶ Federal Rules of Evidence

References and Readings Blau, T. H. (1984). The psychologist as expert witness (pp. 19–25). New York: Wiley. ISBN 0-471-87129-X. Grisso, T. (1988). Competency to stand trial evaluations: A manual for practice. Sarasota FL: Professional Resource Exchange. ISBN 0-943158-51-6. Nietzel, M. (1986). Psychological consultation in the courtroom. New York: Pergamon Press. ISBN 0-08-030955-0. Shapiro, D. L. (1984). Psychological evaluation and expert testimony. New York: Van Nostrand Reinhold. ISBN 0-442-28183-8. Smith, S. R. (1988). Law, behavior, and mental health: Policy and practice. New York: University Press. ISBN 0-8147-7857-7.

Speciality guidelines for forensic psychologists. http://www.ap-ls.org/links/ currentforensicguidelines.pdf. Retrieved on 14 September 2007.

Forgetting E LIZABETH LOUISE G LISKY University of Arizona Tucson, AZ, USA

Synonyms Memory loss

Definition Forgetting refers to a loss of information that was previously acquired or learned.

Current Knowledge Although, strictly speaking, forgetting refers to a loss of information that was previously retained in memory, the term is often used to refer to a failure of retrieval. It can be easily shown, however, that information that is not retrievable on one occasion may be retrieved on another, indicating that the information was not forgotten but was inaccessible given the existing retrieval environment. Apparent forgetting may also occur because information was not adequately encoded or stored in the first place. Given adequate encoding and storage, however, normal forgetting is thought to be attributable primarily to interference from similar information that is acquired later and interferes with retrieval. Most evidence suggests that forgetting rates per se do not vary as a function of disease or injury. Rather, increased forgetting is a result of increased susceptibility to interference or a failure to construct or initiate appropriate retrieval strategies – problems usually associated with frontal lobe dysfunction.

Cross References ▶ Consolidation ▶ Memory ▶ Retrieval, Retrieval Techniques

Fourier Transforms

Formboard Test ▶ Tactual Performance Test

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Fourier Transforms J OAN S WEARER University of Massachusetts Medical School Worcester, MA, USA

Fornix Synonyms J OHN B IGBEE Virginia Commonwealth University Richmond, VA, USA

Fourier analysis; Linear systems analysis

Definition Definition A major fiber tract of the limbic system formed by an arching bundle of axons extending from the hippocampus. The fornix forms a portion of the Papez circuit of the limbic system which consists of a closed loop sequentially connecting the subiculum of the hippocampus, mammillary nuclei, anterior thalamus, cingulate gyrus, and entorhinal cortex. Together with other limbic-associated structures, Papez circuit functions primarily in the cortical control of emotion and memory storage.

Fourier analysis provides a quantitative way in which patterns or complex waveforms can be described by breaking the waveform down into elemental units. It has many applications in physics and engineering, and is used as a basis for studying hearing and understanding visual processing. Its advantage in the application to vision is the common basis by which one can examine optical, physiological, and psychophysical data. There are limitations to its direct application to vision (e.g., the underlying assumption of linearity). Nevertheless, it is a useful tool for conceptualizing how the visual system processes complex spatial information (De Valois & De Valois, 1988).

Current Knowledge From each hippocampal formation, axons from cells of the subiculum and to a lesser extent from pyramidal cells converge in the midline forming the body of the fornix which arches over the thalamus, extends anteriorly, then inferiorly and divides at the level of the anterior commissure. The postcommissural fornix contains axons originating in the subiculum of the hippocampus and extends to the mammillary body. The precommissural fornix contains axons of pyramidal neurons which terminate diffusely in the septal nuclei, frontal cortex, nucleus accumbens, and anterior hypothalamus.

Cross References ▶ Limbic System ▶ Papez Circuit

Historical Background Jean Baptise Fourier, an eighteenth century French physicist and mathematician, is credited with the concept that waveforms of any complexity can be broken down (analyzed) into the sum of sine and cosine waves of specified frequencies, amplitudes, and phases. Conversely, by the inverse Fourier transform, any waveform can be built up (synthesized) by summing together sine waves of appropriate frequency, amplitude, and phase. In 1969 Blakemore and Campbell (cited in Schwarz, 2004) suggested that the visual system deconstructs the retinal image into its spatial frequency components, or performs a linear systems analysis akin to a Fourier analysis.

Current Knowledge Sine Waves

Fourier Analysis ▶ Fourier Transforms

Sine waves are elemental components that can serve as building blocks to construct any complex achromatic visual scene. In spatial vision they are sinusoidal oscillations of

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alternating light and dark (luminance) across space. (Chromatic scenes can also be described by Fourier analysis but this will not be discussed here.) A sine wave grating thus consists of alternating light and dark bars, and the transition from light to dark is gradual (sinusoidal). It is necessary to specify the frequency, amplitude (or related contrast), phase, and orientation (for twodimensional stimuli) to fully describe a sine wave grating. The spatial frequency (an oscillation in luminance or one cycle of light to dark) is usually specified in cycles per degree of visual angle (cycles/degree) since the dimensions of visual stimuli are more usefully defined by the angle subtended at the eye. The luminance profile, or contrast, is related to amplitude in that both are measures of the height of the waveform. The peak or height of a waveform corresponds to its maximum luminance (lmax) and the trough is the minimum luminance (lmin), and contrast = lmax lmin/ lmax + lmin. Phase refers to the position of a sine wave with respect to a fixed reference point (‘‘absolute phase’’) or in reference to another sinusoidal waveform (‘‘relative phase’’). For example, if two sine waves are 180 out of phase, the peak of one luminance profile will be aligned with the trough of the other; and if the two are completely in phase their peaks and troughs will be in alignment. Orientation refers to the angle made by the sine wave with respect to a reference such as vertical and needs to be specified for two-dimensional waveforms (De Valois & De Valois, 1988; Schwartz, 2004).

Fourier Transforms. Figure 1 Analysis of a square wave into its components. On the left is a section of a square wave and its first four components in appropriate relative phases and amplitudes. On the right is the superposition of the components on the original square wave. From ‘‘Spatial Vision’’ by De Valois and De Valois (1988) and by permission of Oxford University Press, Inc.

Cross References ▶ Spatial Frequency Analysis

A Simple Example of Fourier Analysis

References and Readings Square wave gratings have abrupt changes in luminance, or sharp edges between light and dark bars, and are commonly used in psychophysical and physiological experiments. A square wave is a complex waveform that can be analyzed into the linear sum of its harmonically related component sine waves. The fundamental sine wave component, f, is the same frequency and phase of the square wave. Harmonic waves have frequencies that are integer multiples of the lowest, fundamental frequency. For example, the third harmonic has three times the frequency of the fundamental and one third of its contrast. As can be seen in the figure, summing the fundamental and a few odd numbered harmonics (3rd, 5th, and 7th) produces a waveform that closely resembles a square wave. The number of odd number harmonics added to the fundamental determines the degree of precision between the Fourier analysis and the square wave (De Valois & De Valois, 1988; Schwartz, 2004) (Fig. 1).

De Valois, R. L., & De Valois, K. K. (1988). Spatial vision. New York: Oxford University Press. Schwartz, S. H. (2004). Visual perception: A clinical orientation. New York: McGraw-Hill.

Fovea U RAINA C LARK The Miriam Hospital, Neuropsychology Providence, RI, USA

Synonyms Fovea centralis

Fovea Centralis

Definition The fovea is an area of the retina that supports high acuity vision – the ability to discriminate very fine details. When an object is fixated, visual information from the object projects directly onto the fovea. Because the fovea is the only area of the retina that yields high acuity vision, saccadic eye movements are required to obtain detailed visual information from the larger field of view.

Current Knowledge The fovea (derived from the Latin word for pit) is a depressed area of the retina that, in humans, is about 1.5 mm in diameter. It is located in the center of the macula or macula lutea (‘‘yellow spot’’ in Latin), which is a 5 mm circular region of the retina that is responsible for central vision. The fovea appears as a depression in the retina because blood vessels and several cell types present in other regions of the retina (e.g., bipolar cells, horizontal cells, amacrine cells, and ganglion cells) are displaced toward the periphery of the fovea and are not present in this region. This displacement improves the optical quality of visual images on the photoreceptors (i.e., rods and cones) in this area. The fovea is mostly comprised of cones and is virtually devoid of rods. This enhances spatial resolution under well lit conditions, but the lack of rods in the fovea causes this area to be less sensitive to dim light. The center of the fovea is referred to as the foveal pit or foveola, which is a region that is about 350 mm in diameter. Cones in this region are more elongated in shape than those outside of the pit. Cone density in the foveal pit increases significantly, thereby enhancing spatial sampling. Axons from individual foveal cones extend laterally and synapse with bipolar cells (one ON-center, one OFFcenter), which in turn synapse with a single ganglion cell. This organization provides the basis for highly detailed foveal vision. Ganglion cells transmit information from the fovea toward the visual cortex via the lateral geniculate nucleus of the thalamus. The nearly one to one transmission of information from foveal cones to ganglion cells results in a high representation of foveal projections onto the geniculate and primary visual cortex. In the visual cortex, foveal (and macular) information is processed in the region of the calcarine fissure that is closest to the occipital pole, while more anterior regions are devoted to peripheral vision. Because two thirds of the primary visual cortex is devoted to central (i.e., foveal

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and macular) vision, a large cortical lesion is required to disrupt central vision completely. It is more common to observe smaller lesions in visual cortex that result in a hemianopia affecting only the peripheral field, leaving the foveal field (i.e., central vision) relatively unaffected; this condition is called macular sparing. Disruption of central vision can also result from direct damage to the fovea, which is commonly seen in patients with macular degeneration and in individuals with histoplasmosis that has affected the eyes in a condition called ocular histoplasmosis syndrome (OHS). Macular degeneration is a disease associated with aging in which macular and foveal cells are damaged, leading to a gradual loss of sharp, central vision. Histoplasmosis, a flu-like condition that primarily affects the lungs, is caused by inhalation of fungus spores (histoplasma capsulatum) found in bird and bat droppings. Histoplasmosis symptoms can be subtle and are often unobserved by patients. Notably, even mild cases of histoplasmosis can cause OHS, which is a leading cause of vision loss in adults aged 20–40 years in the USA. In OHS, abnormal blood vessels develop beneath the retina, causing lesions and tissue scaring in the macula and fovea. Untreated OHS can severely impair central vision, but because OHS rarely affects peripheral vision, it does not usually lead to total blindness.

Cross References ▶ Cortical Magnification ▶ Hemianopia ▶ Lateral Geniculate Nucleus of Thalamus ▶ Visual Cortex ▶ Visual Field Deficit ▶ Visual System

References and Readings Daniel, P. M., & Whitteridge, D. (1961). The representation of the visual field on the cerebral cortex in monkeys. The Journal of Physiology, 159, 203–221.

Fovea Centralis ▶ Fovea

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Fractional Anisotropy

Fractional Anisotropy PAUL E. K APLAN Capitol Clinical Neuroscience Folsom, CA, USA

Cross References ▶ Progressive Aphasia ▶ Vascular Cognitive Impairment

References and Readings Synonyms T2 – weighted MRI images

O’Donnell, L. J., Kubicki, M., Shenton, M. E., Dreusicke, M. H., Grimson, W. E., & Westin, C. F. (2006). A method for clustering white matter fiber tracts. American Journal of Neuroradiology, 27, 1032–1036. Parker, G. J. M. (2004). Analysis of MR diffusion weighted images. The British Journal of Radiology, 77, S176–S185.

Definition Fractional anisotropy is a method that is used to emphasize and evaluate white matter fiber tracts. This method has been applied to MRI scans in diffusion weighted images. If full tensor data acquisition is applied, the fractional anisotropy (FA) is calculated from the eigenvalues l1, l2, and l3 of the diffusion tensor: pffiffiffi 3 FA ¼ pffiffiffi 2

qffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffi ðl1 lÞ2 þðl2 lÞ2 þðl3 lÞ2 qffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffiffi ; l21 þl22 þl23

where l is the mean diffusivity (trace/3). FA takes on values between 0 (perfectly isotropic diffusion) and 1 (the hypothetical case of an infinite cylinder) and is thus directly comparable between subjects. Here FA is multiplied by 1,000. A fractional process is a procedure that separates mixtures into their components. For example, the total dose of radiation required to treat a tumor will actually be delivered by several different ports or directional beams. The total dose absorbed by that tumor would be maximal and that to the surrounding tissues would thus be minimal. This type of nomenclature has also been applied to ‘‘fractional’’ procedures in molecular biology. Molecules can actually spread throughout the medium related to their thermal energy. The directional part of this spread is called anisotropy. This type of applied molecular physics is directly related to the T2-weighted images noted in MRI studies and used as an early warning exam of brain ischemia.

Current Knowledge T2-weighted MRI studies are an objective methodology of evaluating the effects of blood flow.

Fractionated Radiotherapy ▶ Radiotherapy ▶ Radiosurgery, Stereotactic Radiosurgery

Fragile X Syndrome F RANK J. G ALLO, B ONITA P. K LEIN -TASMAN University of Wisconsin-Milwaukee Milwaukee, WI, USA

Synonyms Frax-A syndrome; Marker X syndrome; Martin–Bell syndrome; X-linked mental retardation and macroorchidism

Short Description or Definition Fragile X (FXS) is a neurodevelopmental disorder that is caused by an unstable expansion (CGG repeats) in the FMR1 gene on the X chromosome. FXS is the most common form of inherited intellectual disability (ID). FXS can result in physical features including prominent ears, elongated face, flat feet, macroorchidism (enlarged testicles in males), connective tissue abnormalities, and epilepsy, among others. Males are generally more severely affected than are females. In addition to ID and various neurocognitive weaknesses, high rates of autism and attention/deficit hyperactivity disorder (ADHD) have been identified.

Fragile X Syndrome

Categorization

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The prevalence of premutation carriers in the general population is higher in females, estimated to be approximately one in 350, compared to one in 1,000 males (Sherman, 2002). The converse is true for full mutation prevalence rates, estimated to be one in 4,000 males, compared to one in 8,000 females. ID (IQ 200 CGG repeats). Full mutation expansions generally lead to transcriptional silencing of the FMR1 gene and subsequent reductions in fragile X mental retardation protein (FMRP), production of which is critical for brain maturation and function (Zalfa, Achsel, & Bagni, 2006).

Epidemiology

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visual–spatial orientation and construction, processing of abstract nonsequential verbal and nonverbal information, and dysfluent speech patterns (i.e., rapid and highly repetitive speech). There is also evidence for higher receptive versus expressive language abilities among individuals with FXS who do not have autism (Philofsky, Hepburn, Hayes, Hagerman, & Rogers, 2004). Behavioral disturbances commonly seen in those with FXS are often reported as the largest area of concern for caregivers and health care providers (Hatton et al., 2000). High rates of ADHD and autism are commonly associated with FXS. Studies show increased rates when compared to contrast groups with ID of mixed etiologies, which suggests higher probabilities than expected based on ID alone. Other reported behavioral difficulties can include aggression and self-injurious behavior, chronic tics, gaze aversion, tactile defensiveness, and hyperarousal to auditory stimuli (Hagerman, 2002). Mood disturbances can include social phobia, panic disorder, OCD, and depression. Preliminary research suggests that premutation males and females can present with many of these behavioral and emotional difficulties, albeit at a lower prevalence rate and level of severity (Cornish et al., 2008). In terms of neuroanatomy, structural MRI studies have shown increased hippocampus, thalamus, and caudate volumes in both males and females (Hessl, Rivera, & Reiss, 2004). Conversely, decreased orbital frontal, amygdala, and superior temporal gyrus volumes have been identified (Gothelf et al., 2007). Well-documented abnormalities of the cerebellum include smaller posterior vermis and enlarged lateral and fourth ventricular

volumes, relative to typical contrasts. Functional imaging studies in FXS indicate abnormal frontostriatal connectivity and function (Hoeft et al., 2007; Menon, Leroux, White, & Reiss, 2004). Attempts have been made to link structural and functional brain abnormalities with FMRP levels and FXS cognitive and behavioral features with mixed success.

Evaluation Since the discovery of the specific molecular characterization of the CGG repeat in FXS (Verkerk et al., 1991), DNA-based molecular analysis has been the preferred method for identifying both full mutation and premutation carriers. The use of prenatal ultrasound or clinician observation as methods for diagnosing FXS is not recommended as individuals display a varied range of phenotypic symptoms, none of which are pathognomonic. In the 1980s cytogenic analysis was utilized to diagnose FXS, but has since been shown to yield inaccurate results.

Treatment There is currently no cure for FXS. Optimal treatment consists of multidisciplinary involvement to address the medical, psychiatric, and neuropsychological sequelae of the disorder. Pediatricians, neurologists, ophthalmologists, orthopedists, obstetricians, and cardiologists are often sought for medical complications associated with

Fragile X Syndrome. Table 1 Psychopharmacological interventions demonstrating effectiveness in fragile X syndrome (FXS) Medication

Problem areas/symptoms

Potential side effects

Stimulants (i.e., dextroamphetamine, methylphenidate)

Hyperactivity, inattention, distractibility, Weight loss, anxiety, mood lability, aggressive behavior, impulsivity irritability, insomnia, tics. Increased side effects can occur in those with lower IQ or under 5 years of age

Adrenergic agonists (i.e., Hyperarousal, hypersensitivity, clonidine, quanfacine) hyperactivity, impulsivity, aggression, sleep disturbances

Sedation, cognitive slowing. More commonly used in children under 5 years of age

SSRI’s (i.e., fluoxetine, setraline)

Diarrhea, agitation, hyperactivity, impulsivity, sleep problems, nausea, manic symptoms (in rare cases)

Anxiety, depression, mood lability, obsessive-compulsive symptoms, aggression

Antipsychotics (i.e., Aggression, mood instability, severe risperidone, aripiprazole) hyperactivity, delusional thinking

Weight gain, sedation, nausea, constipation, tardive dyskinesias (following extended use)

Anticonvulsants (i.e., valproic acid, topiramate)

Sedation, cognitive slowing, irritability, weight changes, hypotonia, impulsivity

Seizures, mood swings

Source: Information gathered from Hagerman (2008) and Kravis and Potanos (2004)

Frankel Scale

FXS (Hagerman, 2008). It is important to note that the need for health care services may vary considerably across individuals. In terms of psychiatric symptoms, a range of psychopharmacological interventions have demonstrated effectiveness among individuals with FXS (Table 1). Psychotherapeutic techniques such as CBT for anxiety, ‘‘Stop and Think’’ approaches for impulsivity, and empiricallysupported treatments for socio-communicative difficulties associated with autism spectrum disorders may also prove beneficial. Psychoeducational and/or neuropsychological assessment can play a critical role in the development of academic intervention strategies for children and adolescents with FXS. At the very least, administration of intellectual, adaptive behavior, and academic achievement measures designed to assess for the presence of ID and learning disability is recommended (Schwarte, 2008). Examination of receptive and expressive language, speech articulation, visual–spatial skill, and attentional resources may also prove helpful in the development of an Individualized Education Plan. Educating teachers and other service providers about the difficulties commonly experienced in FXS may help them to anticipate needs that may arise as the child progresses through school. Speech and language therapy is often required in children with FXS. Alternative methods of communication (i.e., sign language, picture exchange communication system) may be particularly useful for lower functioning individuals or those who present with autism spectrum disorders. Finally, occupational therapy that incorporates supported techniques designed to ameliorate sensory integration issues may also be needed.

Cross References ▶ Attention Deficit, Hyperactivity Disorder ▶ Autistic Disorder ▶ Developmental Delay ▶ Epilepsy ▶ Intellectual Disability ▶ Syndrome

References and Reading For more information on FXS, visit the National Fragile X Foundation website at http://www.fragilex.org Berry-Kravis, E. (2002). Epilepsy in fragile X syndrome. Developmental Medicine and Child Neurology, 44, 724–728. Cornish, K. M., Lexin, L., Kogan, C. S., Jacquemont, S., Turk, J., Dalton, A., et al. (2008). Age-dependent cognitive changes in carriers of the fragile X syndrome. Cortex, 44, 628–636.

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Cornish, K. M., Turk, J., Wilding, J., Sudhalter, V., Munir, F., Kooy, F., et al. (2004). Annotation: Deconstructing, the attention deficit in fragile X syndrome: A developmental neuropsychological approach. Journal of Child Psychology and Psychiatry, 46, 1042–1053. Gothelf, D., Furfaro, J. A., Hoeft, F., Eckert, M. A., Hall, S. S., O’Hara, R., et al. (2007). Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Annals of Neurology, 63, 40–51. Hagerman, R. J. (2002). Physical and behavioral phenotype. In R. J. Hagerman & P. J. Hagerman (Eds.), Fragile X syndrome: Diagnosis, treatment and research (3rd ed., pp. 136–168). Baltimore: The Johns Hopkins University Press. Hagerman, R. J. (2008). Etiology, diagnosis, and development in fragile X syndrome. In J. E. Roberts, R. S. Chapman, & S. F. Warren (Eds.), Speech and language development and intervention in down syndrome and fragile X syndrome (pp. 27–49). Baltimore: Paul H. Brookes Publishing. Hagerman, R. J., Leehey, M., Heinrichs, W., Tassone, F., Wilson, R., Hills, J., et al. (2001). Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X. Neurology, 57, 127–130. Hall, S. S., Burns, D. D., Lightbody, A. A., & Reiss, A. L. (2008). Longitudinal changes in intellectual development in children with fragile X syndrome. Journal of Abnormal Psychology, 36, 927–939. Hatton, D. D., Bailey, D. B., Roberts, J. P., Skinner, M., Mayhew, L., Clark, R. D., et al. (2000). Early intervention services for young boys with fragile X syndrome. Journal of Early Intervention, 23, 235–251. Hessl, D., Rivera, S. M., & Reiss, A. L. (2004). The neuroanatomy and neuroendocrinology of fragile X syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 10, 17–24. Hoeft, F., Hernandez, A., Parthasarathy, S, Watson, C. L., Hall, S. S., & Reiss, A. L. (2007). Fronto-striatal dysfunction and potential compensatory mechanisms in male adolescents with fragile X syndrome. Human Brain Mapping, 28, 543–554. Incorpora, G., Sorge, G., Sorge, A., & Lorenzo, P. (2002). Epilepsy in fragile X syndrome. Brain and Development, 24, 766–769. Menon, V., Leroux, J., White, C. D., & Reiss, A. L. (2004). Frontostriatal deficits in fragile X syndrome: Relation to FMR1 gene expression. Proceedings of the National Academy of Sciences of the United States of America, 101, 3615–3620. Philofsky, A., Hepburn, S. L., Hayes, A., Hagerman, R., & Rogers, S. J. (2004). Linguistic and cognitive functioning and autism symptoms in young children with fragile X syndrome. American Journal on Mental Retardation, 109, 208–218. Schwarte, A. R. (2008). Fragile X syndrome. School Psychology Quarterly, 23, 290–300. Sherman, S. (2002). Epidemiology. In R. J. Hagerman & P. J. Hagerman (Eds.), Fragile X syndrome: Diagnosis, treatment and research (3rd ed., pp. 136–168). Baltimore: The Johns Hopkins University Press. Verkerk, A. J., Pieretti, M., Sutcliff, J. S., Fu, Y. H., Kuhl, D. P., Pizzuti, A., et al. (1991). Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome. Cell, 65, 905–914. Zalfa, F., Achsel, T., & Bagni, C. (2006). mRNPs, polysomes or granules: FMRP in neuronal protein synthesis. Current Opinion in Neurobiology, 16, 265–269.

Frankel Scale ▶ ASIA Impairment Scale

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Frax-A Syndrome ▶ Fragile X Syndrome

FRE 104, 403, 702, 704 R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Federal rules of evidence

Definition The Federal Rules of Evidence (FRE) govern the introduction of evidence in proceedings, both civil and criminal, in federal courts. While they do not apply to suits in state courts, the rules of many states have been closely modeled on these provisions. The following rules are regarded as particularly relevant to the practice of forensic neuropsychology.

Rule 104. Preliminary Questions (a) Questions of admissibility generally Preliminary questions concerning the qualification of a person to be a witness, the existence of a privilege, or the admissibility of evidence shall be determined by the court, subject to the provisions of subdivision (b). In making its determination, it is not bound by the rules of evidence except those with respect to privileges.

(b) Relevancy conditioned on fact When the relevancy of evidence depends upon the fulfillment of a condition of fact, the court shall admit it upon, or subject to, the introduction of evidence sufficient to support a finding of the fulfillment of the condition.

(c) Hearing of jury Hearings on the admissibility of confessions shall in all cases be conducted out of the hearing of the jury. Hearings on other preliminary matters shall be so conducted

when the interests of justice require, or when an accused is a witness and so requests.

(d) Testimony by accused The accused does not, by testifying upon a preliminary matter, become subject to cross-examination as to other issues in the case.

(e) Weight and credibility This rule does not limit the right of a party to introduce before the jury evidence relevant to weight or credibility.

Rule 403. Exclusion of Relevant Evidence on Grounds of Prejudice, Confusion, or Waste of Time Although relevant, evidence may be excluded if its probative value is substantially outweighed by the danger of unfair prejudice, confusion of the issues, or misleading the jury, or by considerations of undue delay, waste of time, or needless presentation of cumulative evidence.

Rule 702. Testimony by Experts If scientific, technical, or other specialized knowledge will assist the trier of fact to understand the evidence or to determine a fact in issue, a witness qualified as an expert by knowledge, skill, experience, training, or education, may testify thereto in the form of an opinion or otherwise, if (1) the testimony is based upon sufficient facts or data, (2) the testimony is the product of reliable principles and methods, and (3) the witness has applied the principles and methods reliably to the facts of the case.

Rule 704. Opinion on Ultimate Issue (a) Except as provided in subdivision (b), testimony in the form of an opinion or inference otherwise admissible is not objectionable because it embraces an ultimate issue to be decided by the trier of fact. (b) No expert witness testifying with respect to the mental state or condition of a defendant in a criminal case may state an opinion or inference as to whether the defendant did or did not have the mental state or condition constituting an element of the crime charged or of a defense thereto. Such ultimate issues are matters for the trier of fact alone.

Free Recall

Cross References ▶ Federal Rules of Civil Procedure ▶ Federal Rules of Criminal Procedures

References and Readings Federal rules of evidence for United States courts and magistrates. (1975). St. Paul, MN: West Publishing. Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis.

Free Order Recall ▶ Free Recall

Free Recall K ARL H ABERLANDT Trinity College Hartford, CT, USA

Synonyms Free order recall; Recall testing

Definition In the free recall task, a participant recalls a list of items in any order. This task resembles memorizing items on a shopping list. Assume your list includes the items milk, bananas, hamburgers, cheese, apples, and peanuts. Trying to recall the list without constraint of order represents free recall.

Current Knowledge Because the requirement of ordering the items is absent, free recall is usually easier for participants than serial recall. Dropping the order requirement gives participants a wider pool of strategies of retrieving the items. One strategy is grouping or clustering the items according to similar attributes, e.g., semantic relatedness. Accordingly

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items would be grouped in your recall protocol, e.g., bananas, apples, milk, and cheese. Another strategy is the distinctiveness strategy. Because the first and last items have no boundary items, they tend to be more distinctive, thus giving them a retrieval advantage. The U-shaped serial position curve, the accuracy profile as a function of list position, reflects the advantage for beginning (primacy) and final (recency) items. Typically, the serial position curve is not symmetrical with a stronger recency effect than primacy effect. This has been attributed to the recency strategy, perhaps working in tandem with the distinctiveness strategy. According to the recency strategy, participants first report those items which are freshest in their mind. Different memory models assume that recent items are stronger, either because there was less decay or less interference from other items. An effective strategy to boost accuracy in free recall is the story strategy where the learner uses list forms to make a story. In general, the more organization the learner imposes, the better the recall (Thompson & Madigan, 2005). Recall accuracy also depends on the stimulus attributes with better recall for words than consonants or syllables. Among words, those rich in imagery are recalled best. Finally, accuracy depends on presentation and test parameters. Usually a very rapid presentation of items diminishes recall of early and central list items, but not necessarily of the most recent items. A slower pace of presentation is thought to allow learners to process the items more deeply, thus strengthening the item’s memory trace. A lengthening of the retention interval depreciates recall accuracy. Brain regions subserving free recall include hippocampal, fusiform, and inferior prefrontal cortical regions. These regions tend to be more active when people memorize items. Indeed, subsequent recall of an item is predicted by greater activity in these areas during study (Emilien, Durlach, Minaker, Winblad, Gauthier, & Maloteaux, 2003). When any of these regions is impaired as in traumatic brain injury or in degenerative diseases like Alzheimer’s disease (AD), free recall is adversely affected. The free recall test is widely used in neuropsychological assessment. For example, Alzheimer’s patients recall less well than controls across all serial positions. As the severity of the disease increases, the primacy region is disproportionally affected. The recency effect on the other hand is relatively resilient. Free recall following a delay is sensitive to memory deficits in individuals who later develop AD’s (Emilien et al., 2003). This is perhaps because these individuals do not take sufficiently advantage of semantic clustering.

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Freedom From Distractibility (WISC-III)

Cross References

Definition

▶ Alzheimer’s Disease ▶ Memory ▶ Memory Impairment ▶ Serial Recall ▶ Short-Term Memory ▶ Working Memory

The FAI is a 15-item self-report scale that provides an index of a person’s capacity to perform instrumental activities of daily living (IADLs) necessary for living independently in the community. The FAI consists of a 4-point interval scale that requires recollection of activities over the past 3 months and over the past 6 months. The 3-month recall items relate to household and social activities such as doing household chores, hobbies, and driving a car. The 6-month recall items consist of travel outings, gardening, and gainful work. Therefore, the FAI provides a measure of activities the individual has undertaken from the relatively recent past. The scores can range from 15 to 60, higher scores indicating greater success with activities. The FAI can be separated into three subscales of domestic/chores (items 1–5), leisure/work (remaining odd numbered items), and outdoor activities (remaining even numbered items). A modified version was developed by Wade, Langton Hewer, and Skillbeck, Ismail (1983) using a 3-point interval scale with the highest score of 45.

References and Readings Emilien, G., Durlach, C., Minaker, K., Winblad, B., Gauthier, S., & Maloteaux, J. -M. (2003). Alzheimer disease: Neuropsychology and pharmacology. New York: Springer. Thompson, R. F., & Madigan, S. A. (2005). Memory: The key to consciousness. Washington, DC: Henry Press.

Freedom From Distractibility (WISC-III)

Current Knowledge

▶ Working Memory Index

Free-Response Measures ▶ Projective Technique

Frenchay Activity Index S UE A NN S ISTO Stony Brook University, School of Health Technology and Management Stony Brook, NY, USA

Synonyms FAI; SR-FAI (self-rating Frenchay Activity Index) The Adelaide Activities Index is a minor variation of this test. Other variations include the FAI-18 and the Modified FAI

The FAI was first published by Margaret Holbrook and Clive E. Skilbeck in 1983 and later developed by Shuling, de Haan, Limburg, and Groenier (1993) to measure disability and handicap after stroke and to develop normal values for a non-disease elder group. The FAI focuses on extended activities of daily living that are not age-specific. While it was initially devised for older people who had sustained a stroke, it seems to be useful with almost any disease. The FAI has been combined with other scales such as the Functional Independence Measure (FIM) that has a low ceiling effect on functional assessment to encompass the range from lower to instrumental daily activities in and outside the home (Epstein-Lubow, Beevers, Bishop, & Miller, 2009).

Administration The FAI takes approximately 5 min to complete when administered by interview. Segal and Schall (1994) reported proxy agreement for the three subscales ranging from adequate (ICC = 0.59) for leisure/work to excellent (ICC = 0.77) for domestic and outdoors. This is most likely due to the focus of the FAI on the frequency, rather than the quality of activity, thereby reducing the subjectivity of the proxy reports. On the other hand, Chen,

Frontal Behavioral Inventory

Hsieh, Mao, and Huang (2007) found limited agreement between patient and proxy reports using the FAI when self-administered, concluding that the two methods should not be used interchangeably for stroke patients with mild impairments. Scores from women and men should be considered separately because of the known gender bias, with men scoring higher in outdoor activities and women scoring higher in domestic activities.

Reliability and Validity Studies examining reliability reported excellent internal consistency, test–retest, inter-rater reliability. Studies examining validity reported excellent correlation between mailed and interview FAI scores but worse when separated by 10 days (criterion validity) and excellent correlation with several functional scales such as the Rankin Scale, SF-36, and the Barthel (construct validity).

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Epstein-Lubow, G. P., Beevers, C. G., Bishop, D. S., & Miller, I. W. (2009). Family functioning is associated with depressive symptoms in caregivers of acute stroke survivors. Archives of Physical Medical Rehabilitation, 90, 947–955. Holbrook, M., & Skilbeck, C. E. (1983). An activities index for use with stroke patients. Age and Ageing, 12(2), 166–170. McPhail, S., Lane, P., Russell, T., Brauer, S. G., Urry, S., Jasiewicz, J., et al. (2009). Telephone reliability of the Frenchay Activity Index and EQ-5D amongst older adults. Health and Quality of Life Outcomes, 29, 7–48. Schuling, J., de Haan, R., Limburg, M., & Groenier, K. H. (1993). The Frenchay Activities Index. Assessment of functional status in stroke patients. Stroke, 24, 1173–1177. Segal, M. E., & Schall, R. R. (1994). Determining functional/health status and its relation to disability in stroke survivors. Stroke, 25, 2391–2397. Wade, D. T., Langton Hewer, R., Skillbeck, C. E., & Ismail, I. M. (1983). The hemiplegic arm after stroke: Measurement of recovery. Journal of Neurology, Neurosurgery and Psychiatry, 46, 521–524. Wade, D. T., Legh-Smith, J., & Langton Hewer, R. (1985). Social activities after stroke: Measurement and natural history using the Frenchay Activities Index, International Rehabilitation Medicine, 7(4), 176–181.

Clinical Utility The clinical utility of the FAI lies in its ability to measure indoor domestic activities, outdoor domestic activities, and outdoor social activities. It can be completed via mail. McPhail et al. (2009) determined that telephone versus face-to-face administration of the FAI instrument gave comparable results when used with older adults with cognitive deficits, indicating that telephone administration is a suitable alternate approach for collection of this functional activity information. It is most often used to compare premorbid versus poststroke IADLs or 1-year poststroke retrospectively at specific intervals.

Cross References ▶ Activities of Daily Living (ADL) ▶ Activities of Daily Living Questionnaire ▶ Alzheimer’s Disease Cooperative Study ADL Scale ▶ Bristol Activities of Daily Living Scale ▶ Instrumental Activities of Daily Living (IADL) ▶ Katz Index of ADLs ▶ Lawton-Brody IADL Scale

References and Readings Chen, M. H., Hsieh, C. L., Mao, H. F., & Huang, S. L. (2007). Differences between patient and proxy reports in the assessment of disability after stroke. Clinical Rehabilitation, 21(4), 351–356.

Frequency Scale ▶ F Scale

Frontal Behavioral Inventory B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms FBI

Definition The Frontal Behavioral Inventory (FBI) is a 24-item questionnaire that measures behaviors such as apathy, indifference, disorganization, inattention, personal neglect, aspontaneity, inflexibility, concreteness, loss of insight, logopenia, verbal apraxia, and alien hand. The test

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is often used to assess frontal lobe dementia. A 4-point scale (none, mild, moderate, and severe) for each item is used and is dependent on the caregivers response. The FBI is intended to serve as a quantitative measure to determine the severity of impairment and to possibly assess a change due to therapeutic intervention.

Cross References ▶ Frontal Lobe ▶ Frontal Lobe Syndrome

References and Readings Gorenstein, E. (1982). Frontal lobe functions in psychopaths. Journal of Abnormal Psychology, 91(5), 368–379. Gordon, P., Yuanjia, W., Doorish, C., Lewis, M., Battista, V., Mitsumoto, H., et al. (2007). A screening assessment of cognitive impairment in patients with ALS. Amyotrophic Lateral Sclerosis, 8(6), 362–365. Milan, G., Lamenza, F., Iavarone, A., Galeone, F., Lore`, E., De Falco, C., et al. (2008). Frontal Behavioural Inventory in the differential diagnosis of dementia. Acta Neurologica Scandinavica, 117(4), 260–265.

the FEF area receives information from the auditory, tactual, and visual environment and is considered to be a multimodal response area.

Cross References ▶ Eye Fields ▶ Saccadic Eye Movements ▶ Visual-Motor Function ▶ Visual Tracking

References and Readings Crowne, D. (1983). The frontal eye field and attention. Psychological Bulletin, 93(2), 232–260. Machado, L., & Rafal, R. (2004). Control of fixation and saccades in humans with chronic lesions of oculomotor cortex. Neuropsychology, 18(1), 115–123. Wong, B., Cronin-Golomb, A., & Neargarder, S. (2005). Patterns of visual scanning as predictors of emotion identification in normal aging. Neuropsychology, 19(6), 739–749.

Frontal Lobe Frontal Eye Fields B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

W ILLIAM G UIDO Virginia Commonwealth University Medical Center Richmond, VA, USA

Synonyms Cerebral hemisphere

Synonyms

Definition

Eye fields

The lateral surface of each cerebral hemisphere is divided into four lobes (frontal, parietal, temporal, and occipital), named for the bones that lie above them. The frontal lobe comprises the anterior one third of the cerebral hemispheres. It is positioned in front of the parietal lobe and above the temporal lobe. It is anterior to the central sulcus and lies above the lateral fissure. The frontal lobe is essential for planning and executing learned and purposeful behaviors. There are four major gyri (precentral, superior frontal, middle frontal, and inferior frontal) and two primary sulci (precentral and central).

Definition The frontal eye fields (FEF) are part of the prefrontal cortex and considered to be Brodmann area 8 and portions of area 9. The FEF receive input from numerous brain regions and seem to coordinate and maintain eye and head movements, gaze shifts, and are involved in the orientation and attention responses to stimuli. Overall,

Frontal Lobe Defense

Current Knowledge There are four functionally distinct areas: the primary motor cortex, the premotor and supplementary motor areas, Broca’s area, and the prefrontal cortex. The primary motor cortex is located in the precentral gyrus that runs parallel and anterior to the central sulcus. This area controls voluntary movement and contains neurons whose axons comprise the corticospinal tract, and extend to the brain stem and spinal cord, which in turn innervate skeletal muscles. Damage to the primary motor cortex of one hemisphere causes muscle weakness or paralysis on the opposite side of the body. Just anterior to the primary motor cortex are the premotor and supplementary motor areas. These regions occupy part of the precentral gyrus, along with adjacent regions of the superior and middle frontal gyri. These areas take part in the planning and selection of a specific movement or sequence of voluntary movements. Broca’s area located in the opercular and triangular parts of the inferior temporal gyrus of one hemisphere (usually the left hemisphere, referred to as the dominant hemisphere) is responsible for the expressive component of language. Broca’s area is connected to a receptive language area, known as Wernicke’s area, located in the posterior part of the superior temporal gyrus. Together they coordinate language expression (Broca’s area) and comprehension (Wernicke’s area). Damage to these language areas lead to aphasia, a defect in expressing or understanding spoken or written language. Patients with Broca’s or expressive aphasia have trouble speaking or writing. Speech is telegraphic, lacks grammatical structure, and is confined largely to single word utterances of a noun or verb. Broca’s aphasia can also include an impaired ability to articulate words (dysarthria), to make certain movements (apraxia), or to read (alexia) or write (agraphia) properly. The most anterior region of the frontal lobe, the prefrontal cortex, includes the remaining parts of the superior frontal, middle, and inferior frontal gyri. This large association area of cortex is important for the development of working memory, planning, moral reasoning, impulse control, and the regulation of appropriate social behavior. The role of prefrontal cortex in behavior is complex and difficult to define. Its influence on personality and social function was first and perhaps best appreciated by the case of Phineas Gage, a nineteenth-century railroad construction worker. Following an accidental explosion, a tamping iron impaled and destroyed his prefrontal cortex. Remarkably, he survived but underwent a dramatic personality change. Prior to the accident, Phineas Gage was described as a law abiding, family man with a strong work ethic. Following

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the accident, his behavior and personality became erratic. He was impulsive, argumentative, and lost all sense of social inhibition. In general, frontal lobe defects include a lack of motivation, an inability to plan ahead, and a general lack of concern for oneself or others. Affect is also disturbed and can range from extreme apathy and emotional blunting, to highly restless states of euphoria, irritability, or aggression.

Cross References ▶ Agraphia ▶ Alexia ▶ Apathy ▶ Apraxia ▶ Broca’s Aphasia ▶ Precentral Gyrus ▶ Wernicke’s Aphasia

Frontal Lobe Defense R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition The ‘‘frontal lobe defense’’ is a strategy often employed by defense attorneys representing their clients in criminal cases where violence, aggression, impulsivity, etc., are regarded as being relevant factors in the commission of the crime(s). The neurosciences have clearly demonstrated that damage to the prefrontal cortex and other areas of the frontal lobe as well as frontal-subcortical connections can cause a behavioral disinhibition syndrome, which some have labeled as ‘‘pseudopsychopathic.’’ Perhaps, the most famous example is the nineteenth century case of Phineas Gage, a railroad worker who survived an explosion in which an iron tamping bar was blasted up under his cheekbone and out through the tope of his head. He subsequently experienced a dramatic change in personality and behavior, characterized by profanity, boisterousness, impulsivity to the point where people said ‘‘Gage was no longer Gage.’’ Damage to the prefrontal cortex was hypothesized to be the cause of that change.

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Attorneys often attempt to use the ‘‘frontal lobe defense’’ in cases of ‘‘diminished capacity’’ where the defense does not have evidence to support insanity but where the defendant’s mental state at the time might play a role in their level of intent. It is assumed that the defendant’s behavior is a product of brain-based disturbance in functioning and that damage to the frontal lobes was either a cause of the violent acting-out, or significantly lowered the threshold whereby someone could act violently. Also, it is used to explain the deficits in planning, poor organization, and impulsivity in crimes that are not considered premeditated. The most common conditions in which the frontal lobe defense is used include the following: severe traumatic brain injury (TBI), brain tumors, cases of toxic exposure, and acute and chronic alcohol and other drug abuse. Tests designed to assess executive functions (e.g., organization and planning and capacity to consider the consequences of one’s actions) are relied on most often in frontal lobe defense cases. However, the behavior of the defendant (at the time of the crime, in their life preceding the event, and during incarceration), not just the objective test results, are other important components to be examined. More recently, neuroimaging studies (e.g., functional MRIs, PET scans) are being used in criminal cases where frontal lobe damage is alleged to be a cause of violence and aggression. But, thus far, studies are methodologically flawed (e.g., include a small number of subjects and lack adequate control groups) to be admitted under Daubert in most jurisdictions.

References and Readings Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Denney, R. L., & Wynkoop, T. F. (2000). Clinical neuropsychology in the criminal forensic setting. Journal of Head Trauma Rehabilitation, 15, 804–828. Miller, B. L., & Cummings, J. L. (2007). The human frontal lobes: Functions and disorders. New York: Guilford Press. Osmon, D. C. (1999). Complexities in the evaluation of executive functions. In J. Sweet (Ed.), Forensic neuropsychology: Fundamentals and practice. Lisse: Swets & Zeitlinger.

Frontal Lobe Dementia ▶ Frontal Temporal Dementia ▶ Frontotemporal Lobar Degenerations ▶ Pick’s Disease

Frontal Lobe Disorder ▶ Dysexecutive Syndrome

Frontal Lobe Personality Scale, FLOPS ▶ Frontal Systems Behavior Scale

Frontal Lobe Syndrome D ENISE K RCH Kessler Foundation Research Center West Orange, NJ, USA

Synonyms Frontal syndrome; Prefrontal lobe syndrome; Typical frontal lobe syndrome

Definition Frontal lobe syndrome (FLS) is a cluster of behavioral, affective, and cognitive symptoms resulting from pathological processes that destroy or interfere with the function of the gray matter of the prefrontal areas of the frontal lobes. Although the quantity, severity, and variety of observable features can vary considerably across cases, it is the ensemble of deficits that is known as FLS. Behavioral and affective characteristics of FLS may include abulia, apathy, lack of concern, confabulation, perseverative responding, loss of spontaneity, inability to maintain goal-directed behavior, motor impersistence, utilization behavior, environmental dependency, stimulus-bound behavior, disorganization, inability to modify behavior to accommodate new information, risk-taking behavior, disinhibition, restlessness, distractibility, hypomania, social inappropriateness, witzelsucht, tactlessness, lack of behavioral restraint, inappropriate sexual behavior/conversation, poor self-awareness, diminished empathy, boastfulness, capriciousness, delusions, puerility, mood incongruent affect, emotional incontinence, constricted range or poor modulation of emotional expression, euphoria, aggression, and irritability. Cognitive deficits

Frontal Lobe Syndrome

may include impairments in attention, working memory, short-term memory, set-shifting, abstract reasoning, judgment, ability to suppress inappropriate responses, verbal and design fluency, strategizing and planning capacity, and capacity for temporal arrangement. FLS is not generally associated with loss of basic sensory or motor capacities or with obvious impairment of speech. FLS may be present with lesions of varying size and location within the prefrontal cortices due to brain injury, tumors, infarcts, seizures, and degenerative or developmental conditions. Furthermore, although FLS can occur from both unilateral and bilateral damage, bilateral lesions often produce more clinically obvious and behaviorally complex deficits.

Historical Background The term FLS is often associated with Alexander Luria, whose widely read works Higher Cortical Functions in Man (1966) and The Working Brain (1973) provided detailed accounts of patients who had sustained frontal lobe lesions. However, the construct of FLS originated in the mid-nineteenth century at a time when research was not theory-driven, but rather observation-based, that is, neuroanatomical features were described, and patterns of deficits were interpreted as syndromes rather than being driven by theory. Furthermore, the term ‘‘frontal lobe syndrome’’ was adopted in lieu of the more apt ‘‘prefrontal lobe syndrome,’’ as the former reflected the field of study of early investigators at the time. By the late 1800s, FLS was a well-established construct, and by the 1950s, researchers had discerned that lesions to different parts of the frontal lobe give rise to distinct patterns of disturbances. Although Luria referenced the conception of FLS in his seminal works, he also discussed the subsets of deficits that were observed in association with more discrete neuroanatomical areas of the prefrontal lobe. The term FLS continues in usage by some to this day; however, it is more accurately defined as a broad rubric comprised of several distinct syndromes, namely the orbitofrontal syndrome, the dorsolateral frontal syndrome, and the mesial frontal (or anterior cingulate) syndrome. The earliest described case of FLS dates back to 1835. However, the best-known account of the impact of frontal lobe damage was of Phineas Gage, a railroad worker who sustained a severe frontal lobe injury after a tamping bar penetrated upward into his left maxilla through his midfrontal cortex. Although Gage recovered physically and cognitively, his emotional and personality functioning

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were drastically changed. The knowledge gained from Gage’s case illustrated the link between the frontal lobes and behavior, and influenced research in various areas that included lesion studies and the development of psychosurgery in humans. Additionally, case studies of patients who had sustained prefrontal brain damage further contributed to an understanding of the neuroanatomical substrates of the prefrontal area.

Lesion Studies Animal lesion studies provided evidence for the prefrontal cortex as a neuroanatomical substrate of behavior. David Ferrier, a pioneer localizationist (1878), showed that ablation of primate prefrontal regions sometimes resulted in apathy, dullness, and loss of attentive and intelligent observation. Leonardo Bianchi, who dedicated three decades (1895–1922) to delineating the functional significance of the frontal lobes, found no observable change in function after unilateral ablations in monkeys and dogs. He discovered, however, that bilateral ablations produced decreased affection, fearfulness, diminished sociability, tendency toward violence, impulsivity, and low frustration tolerance. Bianchi believed that bilateral ablation caused disintegration of personality, which led to impaired integration of experiences and consequently to behavioral disturbances. Carlyle Jacobsen and John Fulton (1935) observed that bilateral prefrontal cortical resections cured temperamental chimpanzees, but left them without ‘‘worries over mistakes.’’ Through lesion work, researchers also began to demonstrate that damage to the prefrontal areas could result in cognitive impairments. For example, Jacobsen and Fulton observed that chimpanzees with bilateral prefrontal cortical resections had difficulty learning tasks that had an enforced delay between the stimulus and response, and Shepherd Ivory Franz (1912) showed that, whereas unilateral prefrontal ablation had no effect on a monkey’s ability to retain learning, bilateral ablation produced loss of learning. Franz determined, however, that rapid relearning took place after bilateral ablation, leading to skepticism surrounding the designation of higher-level functions to the frontal lobe. An early pattern in the research suggested that prefrontal ablation dulled affect, whereas it did not necessarily result in permanent intellectual diminution. These findings may have been the impetus for Antonio Egaz Moniz’s pioneering the human leucotomy procedure for the treatment of psychotic behaviors (1935). Moniz described his postsurgical patients as less agitated and

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paranoid but more disoriented and apathetic. The leucotomy was introduced in the USA by Walter J. Freeman and James Watts in 1936, and shortly thereafter this and similar procedures (e.g., frontal lobotomy) were carried out on more than 40,000 patients. Postsurgically, patients’ intellect appeared unchanged, and anxiety and social withdrawal were decreased; however, drawbacks frequently reported were complacency, disinhibition, placidity, apathy, abulia, lack of empathy, inattentiveness, reduced creativity and artistic expression, and cessation of the ability to dream.

Case Studies Case studies provided an additional approach to appreciating the consequences of frontal lobe damage, and thus the characteristics of the FLS. Clinical investigations generated findings that were not always consistent, but served to document diverse behavioral disorders resulting from prefrontal injury. Moses Allen Starr (1884) published two papers wherein he reviewed American literature on behavioral effects of tumors, abscesses, diseases, and acute injuries to the brain. He noted that frontal lobe damage frequently impaired attention, intellectual processes, temperament, and personality. Moritz Jastrowitz (1888) emphasized that his frontal patients often exhibited an odd, cheerful demeanor, which he termed ‘‘social moria.’’ Leonore Welt (1888) combined her own clinical findings with results from the literature, ascribing aggression, bad temper, and viciousness to frontal lobe lesions; she additionally cited numerous cases of frontal lobe disease associated with apparent diminution of intelligence in the absence of personality change. Welt was the first to emphasize the importance of lesions to the mesial orbital area in changes of character, although she noted that orbital injury did not always produce personality changes. Hermann Oppenhiem’s work (1890) highlighted the sarcastic, latently hostile nature of frontal lobe patients’ compulsive joking (i.e., ‘‘Witzelsucht’’), and observed that these deficits could arise from tumors as well as other pathology such as general paresis and trauma. The importance of the early case studies was the establishment of the relationship between various pathologies of unilateral and bilateral prefrontal damage and distinctive changes in personality and behavior. In the early 1900s, case study research added to the behavioral symptoms already known to result from prefrontal lobe lesions, and revealed that some cases of FLS occurred in individuals with no obvious damage to the frontal lobes. Based on a review of 785 cerebral tumor

cases, Paul Schuster’s monograph (1902) summarized what was known about the frontal lobes at that time. He divided changes seen in patients into categories of ‘‘mental paralyzation,’’ irritability, hypomania, and witzelsucht. His observations led to suggest that left-sided prefrontal damage was more frequently associated with impaired intellect, whereas right-sided damage was more frequently associated with defects in emotion and temperament, and with greater frequency of FLS impairments. World War I provided an opportunity to study a large cohort of individuals who sustained penetrating brain injuries of the frontal lobe, aiding in better characterization of FLS and its classification into further subcategories. Based on his diagnostic and rehabilitative experience with brain-injured solders, Kurt Goldstein (1920) purported that loss of the ability to abstract was the key feature of prefrontal lobe damage. This, in turn, manifested itself in impairment in initiative, foresight, resistance to suggestion, self-awareness, flexibility in behavior, and analyzing a complex situation into its components. In contrast, Ernst Feuchtwanger’s (1923) monograph on the behavioral changes in war victims with prefrontal injuries underlined that a variety of changes could result from prefrontal damage and that FLS was not a simplistic concept. Karl Kleist’s monograph on penetrating brain wounds placed stress on the association between orbital lesions and lack of initiative, and related the importance of the orbital areas to its connections with limbic system structures. Subsequent case studies (Brickner, 1934; Ackerly, 1937; Vincent, 1936) provided reaffirmation of the symptoms known to be included in the profile of FLS. Although there were some controversial studies that argued against the functional significance of the frontal lobe (e.g., Hebb 1939), most reviewers of the era believed that the frontal lobe was responsible for higher-order cognitive and behavioral functions. By 1947, researchers acknowledged cardinal symptoms of frontal lobe damage, and the term FLS was adopted as a convenient label; however, it was also recognized that the pattern of deficits described in association with disease of the prefrontal region was too broad. By the time of Higher Cortical Functions in Man (1966), Luria and others set to the task of refining the concept of FLS through establishing methods to assess the functions of the frontal lobe.

Current Knowledge The term ‘‘frontal lobe syndrome’’ is no longer considered appropriate, as it suggests damage to a rather large, but

Frontal Lobotomy

circumscribed, neuroanatomical area. Nomenclature that either reflects a more discrete anatomical area or functional deficits (i.e., dysexecutive syndrome) is favored. In terms of prefrontal anatomy, specific dorsolateral frontal, orbitofrontal, and mesial frontal syndromes have been described. With regard to function, the term dysexecutive syndrome was introduced by Alan Baddeley (1986) to emphasize the common cognitive or functional link between the set of problems that can occur.

Future Directions While much has been learned, there is a considerable amount of information not known yet about the frontal lobes, particularly because of their involvement in a higher-order network with intricate interconnections with subcortical and other cortical areas. Future neuroimaging studies will undoubtedly contribute to advancing our understanding of this complex and fascinating region of the brain.

Cross References ▶ Ablation ▶ Anterior Cingulate ▶ Dorsolateral Frontal System ▶ Dysexecutive Syndrome ▶ Frontal Lobe ▶ Frontal Lobotomy ▶ Gage, Phineas (1823–1860) ▶ Limbic System ▶ Luria, Alexander Romanovich (1902–1977) ▶ Mesial Frontal System ▶ Orbitofrontal System ▶ Penetrating TBI ▶ Prefrontal Cortex ▶ Traumatic Brain Injury ▶ Witzelsucht

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Orton, J. F. Fulton, T. K. Davis (Eds.), Localization of function in the cerebral cortex (pp. 259–351). Baltimore: Williams & Wilkins. Darling, S., Della Sala, S., Gray, C., Trivelli, C. (1998). Putative functions of the prefrontal cortex: Historical perspectives and new horizons. In G. Mazzoni & T. O. Nelson (Eds.), Metacognition and cognitive neuropsychology: Monitoring and control processes. Mahwah, NJ: Lawrence Erlbaum Associates. Feuchtwanger, E. (1923). Die Funktion des Stirnhirns: Ihre Pathologie und Psychologie. Berlin: Springer. Franz, S. I. (1912). New Phrenology. Science, 35, 321–328. Fulton, J. F., & Jacobsen, C. F. (1935). Fonctions des lobes frontaux; etude comparee chez l’homme et les singes chimpanzes. Paper presented at the International Neurological Congress. London. Hebb, D. O. (1939). Intelligence in man after large removals of cerebral tissue: Report on four left frontal lobe cases. Journal of General Psychology. 21, 73–87. Jastrowitz, M. (1888). Beitra¨ge zur Localisation im Grosshirn und u¨ber deren praktische Verwerthung. Deutsche Medizinische Wochenschrift. 14, 81–83, 108–112, 125–128, 151–153, 172–175, 188–192, 209–211. Lezak, M. D. Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Luria, A. R. (1973). The working brain: An introduction to neuropsychology. New York: Basic Books. Morgan, J. E., & Ricker, J. H. (Eds.). (2007). Textbook of clinical neuropsychology. New York: Taylor & Francis. Starr, M. A. (1884). Cortical lesions of the brain. A collection and analysis of the American cases of localized cerebral disease. The American Journal of the Medical Sciences, 87, 366–391. Starr, M. A. (1884). Cortical lesions of the brain. A collection and analysis of the American cases of localized cerebral disease. The American Journal of the Medical Sciences, 88, 65–83. Vincent, C. (1936). Neurochirurgische Betrachtungen u¨ber die Funktionen des Frontal-lappens. Dtsche. Med. Wochenschr, 62, 41–45. Welt, L. (1888). Ueber Charakterveranderungen des Menschen infolge von La¨sionen des Stirnhirns. Deutsche Archiv fu¨r Klinische Medizine. 42, 339–390.

Frontal Lobotomy J ACINTA M C E LLIGOTT National Rehabilitation Hospital Dun Laoghaire Co., Dublin, Ireland

Definition References and Readings Ackerly, S. (1937). Instinctive emotional and mental changes following prefrontal lobe extirpation. American Journal of Psychiatry. 92, 717–729. Baddeley, A. (1986). Working memory. Oxford: Clarendon Press. Benton, A. L. (1991). The prefrontal region: Its early history. In H. S. Levin, A. L. Benton, & H. M. Eisenberg (Eds.), Frontal lobe function and dysfunction (pp. 3–32). New York: Oxford University Press. Brickner, R. M. (1934). An interpretation of frontal lobe function based upon the study of a case of partial bilateral frontal lobectomy. In S. T.

Frontal lobotomy is a neurosurgical procedure which involves the resection of all or portions of the frontal lobes of the brain.

Historical Background The most famous case of frontal lobe injury occurred in 1848 when a railroad worker named Phineas Gage

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suffered a severe frontal injury following an explosion which resulted in a 109 cm-long spike passing through the frontal lobe of his brain, this spike entered through his orbit and exited through his skull (Mashour, Walker, & Martuza, 2005). Phineas Gage survived but suffered profound personality and behavioral changes as a result of his frontal lobe injury (Fig. 1). Psychosurgery now called ‘‘the neurosurgical treatment of psychiatric disease’’ dates back to antiquity and has a complex and controversial history. A review by George Mashour et al. in 2004 notes that the birth of modern psychosurgery is attributed to a Swiss psychiatrist Gottlieb Burkhardt, who performed the first psychosurgical procedures of the modern era in 1888 (Mashour et al., 2005). The second World Congress of Neurosurgery in 1935 seems to have been a pivotal point in the development and expansion of frontal lobe ablative techniques for the neurosurgical management of psychiatric disorders. At this meeting Fulton and Jacobsen presented data on calming and behavioral changes associated with the resection of the anterior frontal association cortex (Mashour et al., 2005). It was also at this meeting that the famous Portuguese neurologist Egas Moniz suggested the ablation of the frontal cortex in humans with psychiatric disease (Mashour et al., 2005). Moniz’s neurosurgical colleague Almeida Lima performed the first series of experimental surgeries with postoperative evaluations consisting of the subjective evaluations and psychiatric examination. The operations were reportedly deemed successful but

subsequently it was identified that the records were sparse and follow up was poor with some patients returning to asylums (Mashour et al., 2005). In 1949, Moniz received the Noble prize in Medicine or Physiology for his work in this area. Walter Freeman and his neurosurgical colleague James Watts from the George Washington Medical School in Washington DC were also at this Neurosurgery meeting of 1935, where they introduced the use of the ‘‘transorbital frontal lobotomy’’ – a procedure which was considered crucial in the evolution of psychosurgery. This procedure was relatively easy to perform which unfortunately led to its widespread use by physicians often without surgical training (Fig. 2). In his review, George Mashour points out that in 1937 over 400,000 individuals lived in 477 American psychiatric institutions and over half of hospital beds were used by psychiatric patients. Prior to 1950, there were few if any psychopharmacological treatments for mental disorders and frontal lobotomy often allowed patients to leave the crowded asylums and re-enter society. The frontal lobotomy fell out of favor as the medical and scientific literature suggested that the efficacy of the lobotomy was dubious, clinical indications poorly defined and associated side effects severe (Mashour et al., 2005). In addition, many unqualified practitioners were performing lobotomies, increasing the risk of infection and fatal consequences. Despite the serious ethical dilemmas, dubious results, and severe complications, the decline of the frontal lobotomy, according to

Frontal Lobotomy. Figure 1 Courtesy of the National Library of Medicine (arbitrarymarks.com/. . ./uploads/2007/06/gage.jpg)

Frontal Syndrome

Frontal Lobotomy. Figure 2 Walter Freeman performing frontal lobotomy. Reproduced with permission from Andrea Jaszczuk. The Lobotomy an Inside Look (http://oak.cats.ohiou. edu/aj226602/info-pub_jaszcz.htmuk)

Mashour, did not occur until the introduction of the first effective pharmacological agent for psychosis, chlorpromazine, in 1950s. In addition, the rejection of Freeman’s frontal lobotomy procedures by neurosurgeons and the increasing appeal of psychoanalysis were also considered to have attributed to the decline of psychosurgical procedures (Mashour et al., 2005).

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specific assessment of the neurocognitive and neurobehavioral deficits associated with frontal lobe dysfunction. Due to the complexity of neurobehavioral deficits multidisciplinary teams including, neurologists, psychiatrists, physiatrists, neuropsychologists, nursing, and allied health professionals may be necessary in the evaluation, treatment, and rehabilitation of patients with frontal lobe dysfunction. Neurosurgical procedures for psychiatric disorders are now very tightly controlled and largely limited to patients with affective and anxiety disorders who are refractory to pharmacological, psychotherapeutic, or electroconvulsive therapy (Mashour et al., 2005). Because of the considerable controversy and ethical dilemmas associated with psychosurgery, the patient’s psychiatrist is the only individual who may recommend a surgical procedure and must provide detailed documentation regarding the course of therapy, and the reason for discontinuing therapy and the psychiatrist is also responsible for following the patient post-procedure (Mashour et al., 2005). Psychosurgery remains destructive rather than constructive, however, advances in modern stereotactic surgical technology allows for minimal and more precise and defined surgical intervention (Mashour et al., 2005). In addition, improved understanding of the functional neuroanatomy of the frontal lobes and connections to subcortical systems and circuitry allows for improved understanding of the clinicopathological correlates of neuropsychiatric disorders and more precise discrete surgical intervention.

Current Knowledge In the clinical setting, frontal lobotomy may be necessary to remove areas of damaged or necrotic tissue in severe traumatic brain injury or in the removal of hemorrhages, cerebral abscesses, or tumors located in the frontal lobes. The neurological, neurocognitive, and neurobehavioral sequelae of frontal lobotomy can be profound and depends on the location, degree, and extent of the insult to the frontal lobes. Inertia, unresponsiveness, decreased attention span, blunted or inappropriate affect, and disinhibition are common neurobehavioral sequelae of frontal lobe injury and these characteristics are often described as frontal lobe syndrome (Mashour et al., 2005). Advances in understanding of functional neuroanatomical networks and links to subcortical neuronal structures has led to better understanding and clinicopathologic correlation of the cognitive and neurobehavioral deficits associated with frontal lobe dysfunction. Comprehensive neuropsychological assessments have been developed and standardized to allow for more

Cross References ▶ Executive Functioning ▶ Frontal Lobesfrontal Lobe Syndrome ▶ Leukotomy ▶ Lobectomy ▶ Neuropsychological Assessment

References and Readings Mashour, G. A., Walker, E. E., & Martuza, R. L. (2005). Psychosurgery past, present and future. Brain Research Reviews, 48, 409–419.

Frontal Syndrome ▶ Frontal Lobe Syndrome

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Frontal Systems Behavior Scale J ANET G RACE Memorial Hospital of RI Pawtucket, RI, USA

comparing T scores based on normative data of the ratings of prior and current patient behavior. Administration requires 10 min. Forms are hand-scorable and profile forms are available to graph before and after T scores.

Historical Background Synonyms FrSBe

Description The FrSBe16 is a 46-item behavior rating scale that was developed as a measure of behavior associated with damage to the frontal systems of the brain. The measure targets behavioral syndromes associated with three subcortical-frontal circuits described by Alexander, Strick, and Delong (1986). Specifically, apathy/abulia has been associated with the anterior cingulate circuit; executive functions such as abstraction, problem-solving hypothesis generation, and testing have been associated with the dorsolateral prefrontal circuit; and disinhibition has been associated with the orbitofrontal circuit. According to the FrSBe Manual (Grace & Malloy, 2001), the FrSBe was designed (1) to be brief, reliable, and a valid measure of adult behavior; (2) to assess frontal systems related to behavior and allow for comparison of behavior before and after frontal systems damage occurred; and (3) to permit behavior ratings by multiple observers. The test consists of two rating forms: the self-rating form and the family rating form. The self-rating form is completed by the patient, who is asked to provide ratings on a Likert scale about himself or herself prior to illness or injury (before) and at the present time (after). The family rating form is completed by a family member or close informant who has regular contact with the patient. The family rating form can be used by health-care professionals such as rehabilitation team members who are tracking patient behavior over time. FrSBe forms permit calculation of a total score and subscale scores (apathy, disinhibition, executive dysfunction) for before (baseline behavior) and after (post brain compromise) ratings. The FrSBe Professional Manual provides normative data from a sample of 436 men and women ranging in age from 18 to 95 years and in education from 10 years to doctoral level. Normative tables are stratified for gender, age, and education. T scores are provided for the self-rating form and the family rating form. Behavior change can be assessed by

The FrSBe was developed at Brown University as the Frontal Lobe Personality Scale (Grace, Stout, & Malloy, 1999) for clinical use in assessment of dementia and stroke. In 2001, Psychological Assessment Resources published the test in a revised format with the current name, Frontal Systems Behavior Scale.

Psychometric Data Reliability. Several studies of the FrSBe have reported high internal consistency (Grace et al., 1999; Grace & Malloy, 2001; Velligan, Ritch, Sui, DiCocco, & Huntzinger, 2002). Cronbach’s alpha coefficients of 0.92, 0.78, 0.80, and 0.87, for the total, apathy, disinhibition, and executive scales of the family form and 0.88, 0.72, 0.75, and 0.79, for the self-form are reported based on the FrSBe manual normative sample. Based on a large neurological sample (N = 324) described by Stout, Ready, Grace, Malloy, & Paulsen (2003a), alpha coefficient values of 0.94, 0.87, 0.84, and 0.91 for the total, apathy, disinhibition, and executive scales were found for the family form. Alpha values of 0.94, 0.88, 0.86, and 0.91, for total scale, apathy, disinhibition, and executive subscales, respectively, were found in schizophrenia patients (N = 131) by Velligan et al. They also reported that the test–retest reliability after 3 months was 0.78 for the total score and 0.68, 0.65, and 0.65 for apathy, disinhibition, and executive dysfunction subscales, respectively. Factor Structure. Stout et al. (2003a) conducted an exploratory principal factor analysis in a sample of 324 neurological outpatients and research participants from eight diagnostic categories. The diagnostic categories included focal stroke (frontal and nonfrontal), Alzheimer’s disease, Huntington’s disease, Parkinson’s disease (PD), vascular dementia, dementia due to Lewy bodies, and healthy controls. A factor structure consistent with the three subscales, which were originally proposed based upon three subcortical-frontal circuits, was confirmed in a three-factor solution. Eighty three percent of the items from the FrSBe subscales of apathy, disinhibition, and executive dysfunction, loaded on the three corresponding factors. Results from the exploratory principal factor


analysis suggest that some revision or elimination of specific items may be warranted to refine the scale and enhance the validity of the subscales. Validity. Construct validity of the FrSBe was provided by Grace et al. (1999) in a study of 24 patients with frontal brain damage, 15 patients with posterior brain damage, and 48 healthy control subjects. Significant behavior change on the FrSBe total scores of patients with frontal systems lesions were found in comparison with (a) the same patient’s behavior prior to the lesions, (b) behavior of normal control participants, and (c) behavior of patients with nonfrontal lesions. Malloy, Tremont, Grace, and Frakey (2007) and Mendez, Licht, and Saul (2008) found that the FrSBe reliably discriminated between patients with frontotemporal dementia and Alzheimer’s disease (AD). FrSBe behavior profiles differed for cortical (AD) and subcortical (Huntington’s disease and PD) dementias, with the subcortical dementia patients displaying greater levels of apathy (Paulsen et al., 1996). Further, FrSBe subscales have been found to relate to AD progression when frontal systems involvement increases (Ready, Ott, Grace, & Cahn-Weiner, 2003; Stout, Wyman, Johnson, Peavy, & Salmon, 2003b). Psychotic AD patients have been found to score higher on the disinhibition scale of the FrSBe than nonpsychotic AD patients (Paulsen et al., 2000). Convergent validity was demonstrated by relating the FrSBe to other behavioral measures. Norton Malloy, and Salloway (2001) studied 30 dementia patients and their caregivers using the FrSBe and neuropsychiatic inventory (NPI, Cummings et al., 1994), and found that the NPI total and FrSBe total scores were significantly correlated. Velligan et al. (2002) also found significant relationships between the apathy and disinhibition subscales, and specific symptoms rated from the brief psychiatric rating scale. Discriminant validity of the apathy subscale was provided by Cahn-Weiner and colleagues (2002) who found that the FrSBe apathy subscale did not correlate significantly with the geriatric depression scale in Alzheimer and Parkinson patients. Further evidence that the apathy subscale was measuring a different construct and a syndrome that is distinguishable from depression was provided by Ready et al. (2003). They found that negative mood (e.g., sadness and pessimism) was not significantly correlated with FrSBe apathy, whereas items that measured loss of interest and motivation (e.g., loss of interest and loss of energy) were significantly correlated with FrSBe apathy. Ecological validity of the FrSBe has been established in studies of activities of daily living, community reentry, and caregiver burden. Executive dysfunction and apathy

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measured by the FrSBe are associated with decline in instrumental activities of daily living (ADLs) in dementia patients (Norton et al., 2001; Boyle et al., 2003) and multiple sclerosis patients (Basso et al., 2008). Executive dysfunction and disinhibition in AD are related to caregiver burden and depression (Rymer, Salloway, & Norton, 2002; Davis & Tremont, 2007). The FrSBe was found to be a predictor of community reintegration outcomes following traumatic brain injury, whereas neuropsychological tests of executive functioning were not outcome predictors (Reid-Arndt, Nehl, & Hinkebein, 2007). While some relationship between the FrSBe and frontally mediated cognition would support the construct validity of the measure, it is also important that relationships are moderate to demonstrate a unique contribution of the measured behavior. Moderate correlations between the FrSBe and neuropsychological tests that measure working memory and executive control have been reported in multiple studies (Velligan et al., 2002; Paulsen et al., 1996; Chiaravalloti & DeLuca, 2003).

Clinical Uses Clinical use of the FrSBe was first established in stroke patients to quantify frontal behavioral syndromes seen in frontal strokes. It has also been used in differential diagnosis of dementia. Different behavioral profiles are found with the FrSBe when comparing cortical and subcortical dementia (Paulsen et al., 1996), frontotemporal dementia (FTD), and Alzheimer’s disease (Malloy et al., 2007; Mendez et al., 2008), and when comparing mild cognitive impairment and early and later stage Alzheimer’s disease (Ready et al., 2003; Stout et al., 2003b). Zamboni, Huey, Krueger, Nichelli, and Grafman (2008), used the FrSBe and voxel-based morphometry of MRI data to examine how gray matter loss in FTD relates to the behavioral syndromes of apathy and disinhibition. They found differential associations; the right dorsolateral prefrontal region related to the severity of apathy, whereas the right medio-temporal regions related to the severity of disinhibition. Clinical applications of the FrSBe in PD have included pre and postsurgical assessment with the FrSBe for both posteroventral pallidotomy and deep brain stimulation of the internal globus pallidus or subthalamic nucleus (Saint-Cyr & Trepanier, 2000). A quarter of the patients had behavioral dyscontrol postoperatively which was still evident in some patients at the 9–12 month follow-up evaluation. Apathy is a common syndrome in PD. The FrSBe has been utilized to measure apathy in PD and its relationship

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with testosterone deficiency (Ready, Friedman, Grace, & Fernandez, 2004), and performance on executive measures (verbal fluency, conceptualization; Zgaljardic et al., 2007). The FrSBe was also used to contrast preillness and postillness behavior in amyotrophic lateral sclerosis (Grossman, Woolley-Levine, Bradley, & Miller, 2007) and multiple sclerosis (Chiaravalloti & DeLuca, 2003). Preillness FrSBe scores of the MS group were in the normal range on both self and family forms. After illness, clinically and statistically significant increases in abnormal behavior were noted, especially on the FrSBe apathy and executive scales. These clinical abnormalities correlated with neuropsychological tests assessing information processing, working memory, and executive control. MS patient self-reported executive dysfunction on the FrSBe predicted neuropsychological test deficits and poor adaptive functioning (Basso et al., 2008). The FrSBe has been used in studies of impulse control disorders. These include studies of polysubstance abuse, credit card debt (Spinella, Yang, & Lester, 2004), and impulsive behavior related to PD medications. Substancedependent individuals showed greater apathy, disinhibition, and executive dysfunction on the FrSBe relative to healthy controls. These behavioral problems were associated with real-life functioning and neuropsychological tests of working memory, response inhibition, mental flexibility, and affective processing (Verdejo-Garcıá, Bechara, Recknor, & Pe´rez-Garcıá, 2006a). The severity and the type of drug use were associated with different behavioral profiles on the FrSBe which could be a useful guide for treatment approach (Verdejo-Garcıá, RivasPe´rez, Lo´pez-Torrecillas, & Pe´rez-Garcıá 2006b). The comparison between patient and caregiver ratings on the FrSBe has been used to study the concordance of patient and caregiver reports as well as patient self-awareness. A low level of agreement between PD patient and caregiver reports was found (McKinlay, Grace, Dalrymple-Alford et al., 2008). The severity of cognitive impairment, depression, and anxiety, related to the level of self-awareness and accuracy of self-report in MS patients (Goverover, Chiaravallti, & DeLuca, 2005). Interestingly, substance abusers’ self-awareness varied depending upon the severity of abuse and whether or not they were abstinent. While abusing, the patients showed less awareness of their deficits, but when abstinent, their awareness was similar to informants (Verdejo-Garcıá & Perez-Garcıá, 2008). Finally, Hoerold and colleagues (2008) explored the role of frontal systems in self-awareness. They used the level of agreement in self versus other ratings on the FrSBe as a measure of metacognitive awareness. They found that poor metacognitive

awareness correlated positively with prospective memory performance, more frequent lapses in attention, and greater absentmindedness and negatively with anxiety scores. In regard to psychiatric applications, Velligan et al. (2002) found that schizophrenic patients had significantly greater impairment on all FrSBe scales than control subjects. They also found that the FrSBe was correlated with two measures of adaptive functioning and that cognitive tests had differential and meaningful relationships with FrSBe behavioral profiles. Psychopathy in prisoners was found to be associated with executive dysfunction on the FrSBe (Ross, Benning, & Adams, 2007). There are several foreign language translations of the FrSBe available through the publisher. The Spanish translation has been validated in comparison to the American normative data (Caracuel et al., 2008).

Cross References ▶ Behavioral Assessment of the Dysexecutive Syndrome ▶ Behavior Rating Inventory for Executive Functions ▶ Disinhibition ▶ Dorsolateral Frontal System ▶ Executive Interview ▶ Frontal Behavior Inventory (FBI) ▶ Frontal Lobe Syndrome ▶ Mesial Frontal System ▶ Orbitofrontal System

References and Readings Alexander, G. D., Delong, M. R., & Strick, P. L. (1986). Parallel organization of functionally segregated circuits linking basal ganglia and cortex. Annual Review of Neuroscience, 9, 357–381. Basso, M. R., Shields, I. S., Lowery, N., Ghormley, C., Combs, D., Arnett, P. A., & Johnson, J. (2008). Self-reported executive dysfunction, neuropsychological impairment, and functional outcomes in multiple sclerosis. Journal of Clinical and Experimental Neuropsychology, 3, 1–11. Boyle, P. A., Malloy, P. F., Salloway, S., et al. (2003). Executive dysfunction and apathy predict functional impairment in Alzheimer disease. American Journal of Geriatric Psychiatry, 11, 214–221. Cahn-Weiner, D. A., Grace, J., Ott, B. R., Fernandez, H. H., & Friedman, J. H. (2002). Cognitive and behavioral features discriminate between Alzheimer’s and Parkinson’s disease. Neuropsychiatry, Neuropsychology, and Behavioral Neurology, 15(2), 79–87. Caracuel, A., Verdejo-Garcıá, A., Vilar-Lopez, R., Perez-Garcia, M., Salinas, I., Cuberos, G., Coin, M. A., Santiago-Ramajo, S., & Puente, A. E. (2008). Frontal behavioral and emotional symptoms in Spanish individuals with acquired brain injury and substance use disorders. Archives of Clinical Neuropsychology, 23(4), 447–454.

Frontal Temporal Dementia Chiaravalloti, N. D., & DeLuca, J. (2003). Assessing the behavioral consequences of multiple sclerosis: An application of the Frontal Systems Behavior Scale (FrSBe). Cognitive and Behavioral Neurology, 16(1), 54–67. Cummings, J. L., Mega, M., Gray, K., Rosenberg-Thompson, S., Mega, M., Carusi, A., & Gornbein, J. (1994). The Neuropsychiatric Inventory: Comprehensive assessment of psychopathology in dementia. Neurology, 44, 2308. Davis, J. D., & Tremont, G. (2007). Impact of frontal systems behavioral functioning in dementia on caregiver burden. Journal of Neuropsychiatry and Clinical Neuroscience, 19(1), 43–49. Goverover, Y., Chiaravalloti, N., & DeLuca, J. (2005). The relationship between self-awareness of neurobehavioral symptoms, cognitive functioning, and emotional symptoms in multiple sclerosis. Multiple Sclerosis, 11(2), 203–212. Grace, J., & Malloy, P. F. (2001). Frontal systems behavior scale. Professional manual. Lutz, FL: Psychological Assessment Resources. Grace, J., Stout, J. C., & Malloy, P. F., (1999). Assessing frontal lobe behavioral syndromes with the Frontal Lobe Personality Scale. Assessment, 6(3), 269–284. Grossman, A. B., Woolley-Levine, S., Bradley, W. G., & Miller, R. G. (2007). Detecting neurobehavioral changes in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 8(1), 56–61. Hoerold, D., Dockree, P. M., O’Keeffe, F. M., Bates, H., Pertl, M., & Robertson, I. H. (2008). Neuropsychology of self-awareness in young adults. Experimental Brain Research, 186(3), 509–515. Malloy, P., & Grace, J. (2005). A review of rating scales for measuring behavior change due to frontal systems damage. Cognitive and Behavioral Neurology, 18(1), 18–27. Malloy, P. F., Tremont, G., Grace, J., & Frakey, L. (2007). The Frontal Systems Behavior Scale discriminates frontotemporal dementia from Alzheimer’s disease. Alzheimer’s & Dementia, 3, 200–203. McKinlay, A., Grace, R. C., Dalrymple-Alford, J. C., Anderson, T. J., Fink, J., & Roger, D. (2008). ‘Neuropsychiatric problems in Parkinson’s disease: Comparisons between self and caregiver report’. Aging & Mental Health, 12(5), 647–653. Mendez, M. F., Licht, E. A., & Saul, R. E. (2008). The Frontal Systems Behavior Scale in the evaluation of dementia. International Journal of Geriatric Psychiatry. Epub April 8. Norton, L. E., Malloy, P. F., & Salloway, S. (2001). The impact of behavioral symptoms on activities of daily living in patients with dementia. American Journal of Geriatric Psychiatry, 9(1), 41–48. Paulsen, J. S., Stout, J. C., DeLaPena, J. H.., Romero, R., Tawfik-Reedy, Z., Hamilton, J., Swenson, M. R., Grace, J., & Malloy, P. F. (1996). Frontal behavioral syndromes in corticaland subcortical dementia. Assessment, 3(3), 327–337. Paulsen, J. S., Ready, R. E., Stout, J. C., et al. (2000). Neurobehaviors and psychotic symptoms in Alzheimer’s disease. Journal of the International Neuropsychological Society, 6, 815–820. Ready, R. E., Friedman, J., Grace, J., & Fernandez, H. (2004). Testosterone deficiency and apathy in Parkinson’s disease: A pilot study. Journal of Neurology, Neurosurgery, and Psychiatry, 75(9), 1323–1326. Ready, R. E., Ott, B. R., Grace, J., & Cahn-Weiner, D. A. (2003). Apathy and executive dysfunction in mild cognitive impairment and Alzheimer disease. American Journal of Geriatric Psychiatry, 11(2), 222–228. Reid-Arndt, S. A., Nehl, C., & Hinkebein, J. (2007). The Frontal Systems Behaviour Scale (FrSBe) as a predictor of community integration following a traumatic brain injury. Brain Injury, 21(13–14), 1361–1369.

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Ross, S. R., Benning, S. D., & Adams, Z. (2007). Symptoms of executive dysfunction are endemic to secondary psychopathy: An examination in criminal offenders and noninstitutionalized young adults. Journal of Personality Disorders, 21(4), 384–399. Rymer, S., Salloway, S., Norton, L., et al. (2002). Impaired awareness, behavior disturbance, and caregiver burden in Alzheimer disease. Alzheimer Disease, 16, 248–253. Saint-Cyr, J. A., & Trepanier, L. L. (2000). Neuropsychologic assessment of patients for movement disorder surgery. Movement Disorders, 15, 771–783. Spinella, M., Yang, B., & Lester, D. (2004). Prefrontal system dysfunction and credit card debt. International Journal of Neuroscience, 114(10), 1323–1332. Stout, J. C., Ready, R. E., Grace, J., Malloy, P. F., & Paulsen, J. S. (2003a). Factor analysis of the frontal systems behavior scale (FrSBe). Assessment, 10(1), 79–85. Stout, J. C., Wyman, M. F., Johnson, S. A., Peavy, G. M., & Salmon, D. P. (2003b). Frontal behavioral syndromes and functional status in probable Alzheimer disease. American Journal of Geriatric Psychiatry, 11(6), 683–686. Velligan, D. I., Ritch, J. L., Sui, D., DiCocco, M., & Huntzinger, C. D. (2002). Frontal Systems Behavior Scale in schizophrenia: Relationships with psychiatric symptomatology, cognition and adaptive function. Psychiatry Research, 113(3), 227–236. Verdejo-Garcıá, A., Bechara, A., Recknor, E. C., & Pe´rez-Garcıá, M. (2006a). Executive dysfunction in substance dependent individuals during drug use and abstinence: An examination of the behavioral, cognitive and emotional correlates of addiction. Journal of the International Neuropsychological Society, 12(3), 405–415. Verdejo-Garcıá, A., & Pe´rez-Garcıá, M. (2008). Substance abusers’ selfawareness of the neurobehavioral consequences of addiction. Psychiatry Research, 158(2), 172–180. Verdejo-Garcıá, A., Rivas-Pe´rez, C., Lo´pez-Torrecillas, F., & Pe´rez-Garcıá, M. (2006b). Differential impact of severity of drug use on frontal behavioral symptoms. Addictive Behaviors, 31(8), 1373–1382. Zamboni, G., Huey, E. D., Krueger, F., Nichelli, P. F., & Grafman, J. (2008). Apathy and disinhibition in frontotemporal dementia: Insights into their neural correlates. Neurology, 71(10), 736–742. Zgaljardic, D. J., Borod, J. C., Foldi, N. S., Rocco, M., Mattis, P. J., Gordon, M. F., Feigin, A. S., & Eidelberg, D. (2007). Relationship between self-reported apathy and executive dysfunction in nondemented patients with Parkinson disease. Cognitive and Behavioral Neurology, 20(3), 184–192.

Frontal Temporal Dementia N ANCY J OHNSON Northwestern Feinberg School of Medicine Chicago, IL, USA

Synonyms Behavioral variant frontotemporal dementia; Dementia of frontal lobe type; Frontal lobe dementia

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Frontal Temporal Dementia

Short Description or Definition Frontal temporal dementia (FTD) is one of three clinical syndromes collectively termed frontotemporal lobar degeneration (FTLD) (Neary et al., 1998). FTD is a clinical description of a form of dementia that typically begins in the presenium, and in which personality and social conduct are the first areas to be affected, while other cognitive domains remain relatively preserved early in the disease course.

Categorization Core Diagnostic Features 1. Qualitative change in personality: decline in social manners, antisocial and disinhibited behavior 2. Quantitative change in behavior: either increased (hyperactivity) or decreased (abulia) 3. Emotional blunting: shallowness, lack of empathy or sympathy 4. Loss of insight: lack of concern about financial, social, or occupational consequences of behavior

Supporting Diagnostic Features 1. Decline in personal hygiene: failure to wash, inappropriate clothing combinations 2. Mental rigidity and inflexibility: inability to adapt to novel circumstances, adherence to routine 3. Distractibility and impersistence: failure to complete tasks 4. Hyperorality and dietary change: overeating (esp. sugary food or carbohydrates), excessive consumption of liquids, alcohol, cigarettes 5. Perseveration and stereotyped behavior: humming, pacing a fixed route, hoarding objects 6. Utilization behavior – inability to resist using objects in the environment that is within reach These criteria were found to be highly specific (100%), but marginally sensitive (36.5%) for the diagnosis of FTD early in its course (Mendez et al., 2007).

Neuropathology and Genetics Genetic studies of FTD show a strong familial pattern, with 45% of patients having a positive family history of

dementia, and approximately 18% of cases showing an autosomal dominant pattern of inheritance. Amyotrophic lateral sclerosis (ALS) and FTD frequently occur together, and in these cases, there is a high frequency (59.2%) of a family history of either ALS or FTD (Goldman et al., 2005). Unlike Alzheimer’s disease (AD), where there is a very close correlation between the clinical presentation of memory loss and AD neuropathology, there is no single pathological entity that has been found to be associated with FTD. With advances in staining techniques, two major subtypes of neuropathology have been identified based on the biochemical composition of cellular inclusions found in the brain (Mackenzie & Rademakers, 2007). One type is based on finding tau positive inclusions and the other is associated with inclusions positive for ubiquitin. The most common tauopathies include Pick disease, corticobasal degeneration, progressive supranuclear palsy, and frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17). Ubiquitinated inclusions were initially found only in cases with motor neuron disease, but later studies showed they are also present in pure FTD without motor involvement. Although there is currently no way to predict neuropathology in a single patient with FTD, about half of cases are found to have ubiquitin positive inclusions while the other half are associated with one of the tauopathies (Boeve, 2007). In rare cases, the clinical profile of FTD can be found to have Alzheimer’s disease pathology.

Epidemiology Once thought to be relatively rare, the overall syndrome of FTLD, which includes all three subtypes, is now believed to be the second most common cause of dementia in individuals age 65 or younger, accounting for 5–15% of all forms of dementia (Bird et al., 2003). In the first study of the prevalence of FTLD (Ratnavalli et al., 2002), the majority of subjects (85%) met criteria for the FTD subtype. This study found an age-specific (45–64) prevalence of 15 per 100,000, with the mean age of onset of 52.8 years. There was a 14:3 ratio of male-to-female patients. Another study of the clinical prevalence rate of FTD found 3.6 cases per 100,00 in the 50–59 age group, 9.4 per 100,000 in the 6–069 age group, and 3.8 per 100,000 in the 70–79 age group. The average age of onset was 57.6 and there were no gender differences (Rosso et al., 2003), although many studies do find a male predominance (Goldman et al., 2005).


Natural History, Prognostic Factors, Outcomes Although the initial description of the clinical syndrome that would later be termed frontotemporal dementia (FTD) was made by Arnold Pick in 1892, the first consensus statement for clinical and neuropathological criteria for FTD was published in 1994 by the Lund– Manchester Groups (The Lund and Manchester groups, 1994). FTD was described primarily as a behavioral disorder with bilateral involvement of the temporal and frontal lobes, although it was noted that asymmetric degeneration in the left hemisphere may give rise to a more ‘‘linguistic’’ than behavioral presentation. The nosology was later changed to frontotemporal lobar degeneration (FTLD) by (Neary et al. 1998) in a 1998 consensus conference where the three major clinical subtypes were defined. Of all three FTLD subtypes, FTD patients have the shortest mean survival duration (3.1 years from diagnosis) (Roberson et al., 2005).

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details from a story than AD patients, but no differences in encoding were found on a word list-learning test. However, FTD patients recalled more words after a delay than AD patients, and percent retention on both tasks was greater for the FTD group. Rascovsky et al. (Rascovsky et al., 2002) retrospectively examined cognitive test scores in autopsy-confirmed FTD and AD. Their results showed FTD subjects performed worse than AD on word generation tasks and better than AD on tests of visuospatial abilities and memory. Alterations in behavior and personality are generally the most salient features in FTD, are highly specific for differentiating FTD from other forms of dementia (Miller et al., 1997), and may often precede the decline in cognition (Gregory et al., 1999). Information about behavioral changes relies heavily on the availability of a reliable informant and can be difficult to quantify objectively. The use of structured questionnaires, such as the Neuropsychiatric Inventory (NPI) (Cummings, 1997) and Frontal Behavioral Inventory (FBI) (Kertesz et al., 1997), is helpful in ensuring a comprehensive evaluation of behavioral changes.

Neuropsychology and Psychology of FTD Evaluation Based on the areas of early degeneration in FTD, predominant deficits initially occur in the domains of attention/ working memory processes and executive functioning (i.e., abstract reasoning, planning, organization, and problem solving). Several studies have demonstrated the usefulness of tasks of attention and executive function in assisting with the early differential diagnosis of FTD and AD (Kramer et al., 2003; Lindau et al., 2000; Perry & Hodges, 2000; Walker et al., 2005). However, there have also been studies that did not find consistent differences on attention/executive measures when patients with FTD and AD were directly compared (Grossman, 2002; Kertesz et al., 2003). Thompson et al. (Thompson et al., 2005) found FTD and AD group differences on a range of neuropsychological test scores across multiple domains, but these differences did not occur consistently across tests within any cognitive domains, with the exception of executive functioning. Although the majority of studies find FTD subjects less impaired on memory testing relative to AD patients (Kramer et al., 2003; Walker et al., 2005; Wicklund & Weintraub, 2005), others have not shown this difference (Diehl et al., 2005), possibly due to the type of memory testing used. Wicklund et al. (Wicklund et al., 2006) compared memory performance in FTD and AD and found that FTD patients encoded and recalled more

Many of the initial symptoms of FTD are also compatible with primary psychiatric conditions, such as depression, and FTD patients are often misdiagnosed (Passant et al., 2005) until eventual referral to a dementia clinic. As described above, in the early stages, the symptoms are most often behavioral in nature, and may present as professional performance issues, marital relationship problems, or be attributed to a ‘‘mid-life crisis.’’ A comprehensive evaluation should include not only the neuropsychological and neurological examinations, but in some cases, a psychiatric consultation is also warranted to assist in the differential diagnosis. FTD patients may score normally on neuropsychological testing, including executive measures, in the beginning stage, but a thorough symptom history from a family member or close friend will frequently reveal a history of significant behavioral and emotional changes. Although the neurological examination may also be completely normal in the early stages, the presence of increased muscle tone, apraxia, or subtle signs of Parkinsonism should suggest the possibility of FTD. Structural imaging studies with FTD subjects find atrophy of the frontoinsular and anterior frontal regions initially, a pattern distinct from the posterior parietal atrophy associated with AD. However, on an individual

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case basis, atrophy is not always found until later in the disease course. Research in functional imaging has also shown a distinct pattern in FTD subjects with hypoperfusion in the right superior frontal cortex and bilateral middle frontal cortex (Du et al., 2006); however, functional imaging has not yet become a useful diagnostic tool.

Treatment There are currently no medications that are specifically approved for treating FTD. Selective serotonin reuptake inhibitors (SSRI) are typically used in the early stages for the management of disinhibition, repetitive actions, and sexually inappropriate behaviors (Huey et al., 2006). The majority of clinical trials in the past have focused on open-label symptomatic trials for management of behavioral issues, and there have been very few double-blinded randomized studies. A 6-week placebo-controlled trial of the SSRI paroxetine found no improvement in behavioral symptoms and was associated with a decline in cognition (Deakin et al., 2004). A 6-week trial of trazodone was found to improve eating disorders, agitation, irritability, and depression as measured by the NPI, in a randomized, double-blind, placebo-controlled cross-over trial (Lebert et al., 2004). Behavioral symptoms refractory to treatment with SSRIs may respond to atypical antipsychotic medications, although FTD patients are particularly susceptible to extrapyramidal side effects and these, along with increased somnolence and weight gain, may limit efficacy (Boxer & Boeve, 2007).

Cross References ▶ Amyotrophic Lateral Sclerosis ▶ Corticobasal Ganglionic Degeneration ▶ Frontotemporal Lobar Degenerations ▶ Pick’s Disease ▶ Progressive Supranuclear Palsy ▶ Tauopathy

References and Readings Bird, T., Knopman, D., VanSwieten, J., Rosso, S., Feldman, H., Tanabe, H., et al. (2003). Epidemiology and genetics of frontotemporal dementia/pick’s disease. Annals of Neurology, 54(Suppl 5), S29–S31. Boeve, B. F. (2007). Links between frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis. Alzheimer Disease & Associated Disorders, 21(4), S31–S38.

Boxer, A. L., & Boeve, B. F. (2007). Frontotemporal dementia treatment: Current symptomatic therapies and implications of recent genetic, biochemical, and neuroimaging studies. Alzheimer Disease & Associated Disorders, 21(4), S79–S87. Cummings, J. L. (1997). The neuropsychiatric inventory: Assessing psychopathology in dementia patients. Neurology, 48(5 Suppl 6), S10–S16. Deakin, J. B., Rahman, S., Nestor, P. J., Hodges, J. R., & Sahakian, B. J. (2004). Paroxetine does not improve symptoms and impairs cognition in frontotemporal dementia: A double-blind randomized controlled trial. Psychopharmacology (Berl), 172(4), 400–408. Diehl, J., Monsch, A. U., Aebi, C., Wagenpfeil, S., Krapp, S., Grimmer, T., et al. (2005). Frontotemporal dementia, semantic dementia, and alzheimer’s disease: The contribution of standard neuropsychological tests to differential diagnosis. Journal of Geriatric Psychiatry and Neurology, 18(1), 39–44. Du, A. T., Jahng, G. H., Hayasaka, S., Kramer, J. H., Rosen, H. J., GornoTempini, M. L., et al. (2006). Hypoperfusion in frontotemporal dementia and alzheimer disease by arterial spin labeling MRI. Neurology, 67(7), 1215–1220. Goldman, J. S., Farmer, J. M., Wood, E. M., Johnson, J. K., Boxer, A., Neuhaus, J., et al. (2005). Comparison of family histories in ftld subtypes and related tauopathies. Neurology, 65(11), 1817–1819. Gregory, C. A., Serra-Mestres, J., & Hodges, J. R. (1999). Early diagnosis of the frontal variant of frontotemporal dementia: How sensitive are standard neuroimaging and neuropsychologic tests? Neuropsychiatry, Neuropsychology, and Behavioral Neurology, 12(2), 128–135. Grossman, M. (2002). Frontotemporal dementia: A review. Journal of International Neuropsychological Society, 8(4), 566–583. Huey, E. D., Putnam, K. T., & Grafman, J. (2006). A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia. Neurology, 66(1), 17–22. Kertesz, A., Davidson, W., & Fox, H. (1997). Frontal behavioral inventory: Diagnostic criteria for frontal lobe dementia. Canadian Journal of Neurological Sciences, 24(1), 29–36. Kertesz, A., Davidson, W., McCabe, P., & Munoz, D. (2003). Behavioral quantitation is more sensitive than cognitive testing in frontotemporal dementia. Alzheimer Disease & Associated Disorders, 17, 223–229. Kramer, J. H., Jurik, J., Sha, S. J., Rankin, K. P., Rosen, H. J., Johnson, J. K., et al. (2003). Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and alzheimer disease. Cognitive and Behavioral Neurology, 16(4), 211–218. Lebert, F., Stekke, W., Hasenbroekx, C., & Pasquier, F. (2004). Frontotemporal dementia: A randomised, controlled trial with trazodone. Dementia and Geriatric Cognitive Disorders, 17(4), 355–359. Lindau, M., Almkvist, O., Kushi, J., Boone, K., Johansson, S. E., Wahlund, L. O., et al. (2000). First symptoms–frontotemporal dementia versus alzheimer’s disease. Dementia and Geriatric Cognitive Disorders, 11(5), 286–293. Mackenzie, I. R., & Rademakers, R. (2007). The molecular genetics and neuropathology of frontotemporal lobar degeneration: Recent developments. Neurogenetics, 8(4), 237–248. Mendez, M. F., Shapira, J. S., McMurtray, A., Licht, E., & Miller, B. L. (2007). Accuracy of the clinical evaluation for frontotemporal dementia. Archives of Neurology, 64(6), 830–835. Miller, B. L., Ikonte, C., Ponton, M., Levy, M., Boone, K., Darby, A., et al. (1997). A study of the lund-manchester research criteria for frontotemporal dementia: Clinical and single-photon emission ct correlations. Neurology, 48(4), 937–942.

Frontotemporal Lobar Degenerations Neary, D., Snowden, J. S., Gustafson, L., Passant, U., Stuss, D., Black, S., et al. (1998). Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria [see comments]. Neurology, 51(6), 1546–1554. Passant, U., Elfgren, C., Englund, E., & Gustafson, L. (2005). Psychiatric symptoms and their psychosocial consequences in frontotemporal dementia. Alzheimer Disease & Associated Disorders, 19 (Suppl 1), S15–S18. Perry, R. J., & Hodges, J. R. (2000). Differentiating frontal and temporal variant frontotemporal dementia from alzheimer’s disease. Neurology, 54(12), 2277–2284. Rascovsky, K., Salmon, D. P., Ho, G. J., Galasko, D., Peavy, G. M., Hansen, L. A., et al. (2002). Cognitive profiles differ in autopsy-confirmed frontotemporal dementia and ad. Neurology, 58(12), 1801–1808. Ratnavalli, E., Brayne, C., Dawson, K., & Hodges, J. R. (2002). The prevalence of frontotemporal dementia. Neurology, 58(11), 1615–1621. Roberson, E. D., Hesse, J. H., Rose, K. D., Slama, H., Johnson, J. K., Yaffe, K., et al. (2005). Frontotemporal dementia progresses to death faster than alzheimer disease. Neurology, 65(5), 719–725. Rosso, S. M., Donker Kaat, L., Baks, T., Joosse, M., de Koning, I., Pijnenburg, Y., et al. (2003). Frontotemporal dementia in the netherlands: Patient characteristics and prevalence estimates from a population-based study. Brain, 126(Pt 9), 2016–2022. The Lund and Manchester Groups. (1994). Clinical and neuropathological criteria for frontotemporal dementia. Journal of Neurology, Neurosurgery & Psychiatry, 57(4), 416–418. Thompson, J., Stopford, C., Snowden, J., & Neary, D. (2005). Qualitative neuropsychological performance characteristics in frontotemporal dementia and alzheimer’s disease. Journal of Neurology, Neurosurgery & Psychiatry, 76(7), 920–927. Walker, A. J., Meares, S., Sachdev, P. S., & Brodaty, H. (2005). The differentiation of mild frontotemporal dementia from alzheimer’s disease and healthy aging by neuropsychological tests. International Psychogeriatric Association, 17(1), 57–68. Wicklund, A., & Weintraub, S. (2005). Neuropsychological features of common dementia syndromes. Turk Noroloji Dergisi, 11(6), 566–588. Wicklund, A. H., Johnson, N., Rademaker, A., Weitner, B. B., & Weintraub, S. (2006). Word list versus story memory in alzheimer disease and frontotemporal dementia. Alzheimer Disease & Associated Disorders, 20(2), 86–92.

Frontotemporal Lobar Degenerations N ANCY J OHNSON Northwestern Feinberg School of Medicine Chicago, IL, USA

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Short Description or Definition Frontotemporal lobar degeneration (FTLD) is used to describe a set of three clinical syndromes associated with neurodegenerative pathology primarily targeting the frontal and temporal lobes. All of these syndromes are notable for the relative preservation of memory early in the disease course. The clinical syndromes are (1) frontotemporal dementia (FTD) – a form of dementia in which personality and social conduct are the first areas to be affected, while other cognitive domains remain relatively preserved; (2) progressive non-fluent aphasia (PFNA) – a form of dementia characterized by a progressive loss of expressive language initially involving effortful speech production, phonologic and grammatical errors, and word retrieval difficulties (▶ Primary Progressive Aphasia); and (3) semantic dementia (SD) – a form of dementia in which the initial presentation is characterized by severe naming and word comprehension deficits, as well as an inability to recognize the meaning of visual percepts (i.e., associative agnosia).

Categorization Unlike Alzheimer’s disease (AD) where there is a very close correlation with the clinical presentation of memory loss and AD neuropathology, multiple neuropathological entities have been found to be associated with FTLD. More recent advances in staining techniques have allowed the classification of pathology based on the biochemical composition of cellular inclusions found in the brain (Mackenzie & Rademakers, 2007). Several of the pathologic findings in FTLD, including Pick’s disease, progressive supranuclear palsy, and corticobasal degeneration are all tauopathies and are characterized by the accumulation of hyperphosphorylated tau. The other main type of neuropathology associated with FTLD is characterized by cells that are immunoreactive for ubiquitin. This pathology, referred to as FTLD-U was initially identified in cases of motor neuron disease, but now has been found to also occur in cases of clinically pure FTLD without motor symptoms.

Epidemiology Synonyms Dementia of frontal lobe type; Frontal lobe dementia; Frontal temporal dementia

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Once thought to be relatively rare, FTLD is now believed to be the second most common cause of dementia in individuals aged 65 or younger, accounting for 5–15% of all forms of dementia (Bird et al., 2003). The first

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Frontotemporal Lobar Degenerations

study of the prevalence of the FTLD (Ratnavalli et al., 2002) found an age-specific (45–64) prevalence of 15 per 100,000. The mean age of onset was 52.8, and there was a 14:3 ratio of male-to-female patients. A total of 13 patients were identified as having FTLD and onset prior to age 65. Of these, 85% met criteria for FTD, and 7.5% had PNFA, and 7.5% had SD. Another study of the clinical prevalence rate of FTD found 3.6 cases per 100,00 in the 50–59 age group, 9.4 per 100,000 in the 60–69 age group, and 3.8 per 100,000 in the 70–79 age group. The average age of onset was 57.6, and there were no gender differences (Rosso et al., 2003). Up to 50% of FTLD patients have a positive family history of dementia, suggesting a strong genetic component. Mutations in the microtubule-associated protein tau (MAPT) gene have been found to cause frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). More recently, mutations in the gene encoding progranulin (PGRN) have also been identified with this clinical syndrome (Boeve & Hutton, 2008). The co-occurrence of amyotrophic lateral sclerosis (ALS) and FTLD is much higher than with other forms of dementia. Motor symptoms may precede, follow, or coincide with the onset of the behavioral and cognitive changes.

Natural History, Prognostic Factors, Outcomes Although the initial description of the clinical syndrome that would later be termed frontotemporal dementia (FTD) was made by Arnold Pick in 1892, the first consensus statement for clinical and neuropathological criteria for FTD was published in 1994 by the Lund-Manchester Groups (The Lund and Manchester Groups, 1994). FTD was described primarily as a behavioral disorder with bilateral involvement of the temporal and frontal lobes, although it was noted that asymmetric degeneration in the left hemisphere may give rise to a more ‘‘linguistic’’ than behavioral presentation. Two types of histological changes were described, one involving primarily microvacuolation and nerve cell loss, and the other consisting of Pick-like histology. The nosology was changed to frontotemporal lobar degeneration (FTLD) by Neary et al. (1998) in a 1998 consensus conference where the three major clinical subtypes were defined (Table 1). These two diagnostic criteria are still in use today, although recent genetic and histological findings now recognize corticobasal ganglionic degeneration (CBGD), progressive supranuclear palsy (PSP), and amyotrophic lateral sclerosis (ALS) as part of the FTLD-spectrum disorders.

As with any neurodegenerative disease, the prognosis for FTLD is poor. Estimates of progression range between 2 and 10 years, but there is a great deal of variation between subtypes and even individual cases. The presence of motor neuron disease typically results in a much shorter life expectancy, and rare cases have been described with history of up to 20 years from onset to death (Mesulam et al., 2008).

Neuropsychology and Psychology of FTLD Unlike studies of early AD subjects where episodic memory is almost universally found to be the primary impairment on neuropsychological testing, studies of neuropsychological functioning in early FTLD subjects are variable. This may be due to the fact that FTLD subjects may show secondary impairments in other cognitive domains as the result of executive and/or language impairments. Another potential explanation for the lack of consistent findings is that not all studies differentiate between the behavioral and language subtypes of FTLD, which may lead to increased variability within the FTLD group and obscure differences between FTLD and other groups. The following is a summary of major neuropsychological findings in each of the three subtypes.

Neuropsychological Findings in FTD Based on the areas of early degeneration in FTD, predominant deficits initially occur in the domains of attention/ working memory processes and executive functioning (i.e., abstract reasoning, planning, organization, and problem solving). Several studies have demonstrated the usefulness of tasks of attention and executive function in assisting the early differential diagnosis of FTD and AD (Kramer et al., 2003; Lindau et al., 2000; Perry and Hodges, 2000; Walker et al., 2005). However, there have been studies that did not find consistent differences on attention/executive measures when patients with FTD and AD were directly compared (Grossman, 2002; Kertesz et al., 2003). Thompson et al. (2005) found FTD and AD group differences on a range of neuropsychological test scores across multiple domains, but these differences did not occur consistently across tests within any cognitive domains, with the exception of executive functioning. Although the majority of studies find FTD subjects less impaired on memory testing relative to AD patients (Kramer et al., 2003; Walker et al., 2005; Wicklund &


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Frontotemporal Lobar Degenerations. Table 1 Summary of clinical symptoms using the Neary et al. (1998) criteria Common to all subtypes: Insidious onset and gradual decline over at least 6 months; onset usually before age 65 Subtype Primary (early) clinical presentation

Supportive features

FTD

1. Qualitative change in personality. Decline in social manners, antisocial and disinhibited behavior

1. Decline in personal hygiene. Failure to wash, inappropriate clothing combinations

2. Quantitative change in behavior. Either increased (hyperactivity) or decreased (abulia)

2. Mental rigidity and inflexibility. Inability to adapt to novel circumstances, strong adherence to routines

3. Emotional blunting. Shallowness, lack of empathy, sympathy

3. Distractibility and impersistence. Failure to complete tasks

4. Loss of insight. Unconcerned about financial, social, occupational consequences of behavior

4. Hyperorality and dietary change. Overeating (esp. sugary food or carbohydrates), excessive consumption of liquids, alcohol, cigarettes 5. Perseveration and stereotyped behavior. Humming, pacing a fixed route, hoarding objects 6. Utilization behavior

PNFA

1. Nonfluent speech. Hesitant effortful spontaneous 1. Preservation of social skills. Intact interpersonal and speech with reduced rate of output. At least one of the social conduct following: agrammatism, phonemic paraphasias, anomia 2. Impaired repetition. Less than five digits forward, less than four monosyllabic words 3. Alexia and agraphia. Effortful reading, spelling and grammatical errors in writing 4. Preservation of word meaning

SD

1. Fluent, empty speech. Little information conveyed, use 1. Press of speech. Speaking without interruption of broad generic terms 2. Loss of word meaning including both single-word comprehension and naming (i.e., two-way naming deficit)

2. Idiosyncratic word usage. Example, calling any small object a ‘‘container’’

3. Semantic paraphasias. Often consist of superordinate 3. Absence of phonemic paraphasias in speech category substitutions (e.g., animal for camel), or category errors (e.g., dog for elephant) 4. Prosopagnosia. Impaired recognition of familiar faces 4. Surface dyslexia/dysgraphia (e.g., ‘‘pint’’ pronounced to rhyme with ‘‘mint’’; ‘‘caught’’ written as ‘‘cort’’) 5. Associative agnosia. Impairment of object recognition 5. Preserved calculation present both on visual and tactile presentation 6. Preserved single-word repetition, reading, perceptual 6. Loss of sympathy and empathy matching, and drawing reproduction 7. Narrowed preoccupations 8. Parsimony. Abnormal preoccupation with money or financial economy

Weintraub, 2005), others have not shown this difference (Diehl et al., 2005), possibly due to the type of memory testing used. Wicklund et al. (2006) compared memory performance in FTD and AD and found that FTD patients encoded and recalled more details from a story than AD patients, but no differences in encoding were found on a word list-learning test. However, FTD patients recalled

more words after a delay than AD patients, and percent retention on both tasks was greater for the FTD group. Rascovsky et al. (2002) retrospectively examined cognitive test scores in autopsy-confirmed FTD and AD. Their results showed FTD subjects performed worse than AD on word generation tasks and better than AD on tests of visuospatial abilities and memory.

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Alterations in behavior and personality are generally the most salient features in FTD, are highly specific for differentiating FTD from other forms of dementia (Miller et al., 1997), and may often precede the decline in cognition (Gregory et al., 1999). Information about behavioral changes relies heavily on the availability of a reliable informant and can be difficult to objectively quantify. The use of structured questionnaires, such as the Neuropsychiatric Inventory (NPI) (Cummings, 1997) and Frontal Behavioral Inventory (FBI) (Kertesz et al., 1997) is helpful in ensuring a comprehensive evaluation of behavioral changes.

Neuropsychological Findings in PFNA and SD By definition, language deficits are the most salient feature in early PFNA and SD. Standardized neuropsychological aphasia batteries such as the Boston Diagnostic Aphasia Examination (BDAE) or the Western Aphasia Battery (WAB) are helpful in characterizing these early language impairments. PFNA should be distinguished from states of pure progressive dysarthria or phonological disintegration where the articulation rather than usage of words becomes disrupted. Although the language disorder may interfere with the ability to memorize word lists or solve reasoning tasks, these patients typically have no difficulty recalling daily events or behaving with good judgment, indicating that explicit memory, executive functions, and social skills remain intact. In SD, the most salient feature and common presenting complaint is the decline of expressive vocabulary. In addition, reductions in receptive vocabulary, knowledge of object use, and impaired person knowledge are also eventual clinical features. Debate about the ability to confirm such deficits in patients with language impairment has generated experimental paradigms that rely more on pictures, such as the ‘‘Pyramids & Palm Trees’’ test (Howard & Patterson, 1992), which assesses semantic associations nonverbally, tasks involving coloring of black and white line drawing of well-known animals, and on tests matching environmental sounds to target pictures (Bozeat et al., 2000).

Evaluation In addition to neuropsychological findings as described above, neurological evaluation and neuroimaging are also useful diagnostic tools. Although the neurological examination may be completely normal in the early stages, the

presence of increased muscle tone, apraxia, or subtle signs of parkinsonism should suggest the possibility of FTLD. As FTLD progresses, it is not uncommon to find significant dysarthria, primitive reflexes, hemiparesis, tremor, rigidity, bradykinesia or akinesia, alien hand phenomenon, hemisensory deficits, and fasciculations. Consistent with the clinical presentation, FTD subjects show atrophy of the frontoinsular and anterior regions initially, a pattern distinct from the posterior parietal atrophy associated with AD. In PNFA, atrophy has been shown to involve the left inferior frontal and insular cortex (Gorno-Tempini et al., 2004). In contrast, SD eventually involves atrophy in both the left and right anterior temporal insular regions, although it may begin unilaterally (Seeley et al., 2005).

Treatment There are currently no medications that are specifically approved for treating FTLD. The majority of clinical trials in the past have focused on symptomatic treatment of behavioral issues. Selective serotonin reuptake inhibitors are typically used in the early stages for the management of behavioral symptoms (Boxer & Boeve, 2007). Refractory behavioral symptoms may require the use of atypical antipsychotic agents, although potential negative side effects may limit the efficacy of these types of medications. Both PFNA and SD may benefit from speech-language treatment early in the course of the disease. The types of interventions used are based on the specific areas of language impairment, and are primarily aimed at reducing rate of decline in functional communication skills (Thompson & Johnson, 2005).

Cross References ▶ Amyotrophic Lateral Sclerosis ▶ Corticobasal Ganglionic Degeneration ▶ Frontal Temporal Dementia ▶ Pick’s Disease ▶ Progressive Aphasia ▶ Progressive Supranuclear Palsy ▶ Semantic Dementia ▶ Tauopathy

References and Readings Bird, T., Knopman, D., VanSwieten, J., Rosso, S., Feldman, H., Tanabe, H., et al. (2003). Epidemiology and genetics of frontotemporal dementia/pick’s disease. Annals of Neurology, 54(Suppl 5), S29–S31.

Frustration Tolerance Boeve, B. F., & Hutton, M. (2008). Refining frontotemporal dementia with parkinsonism linked to chromosome 17: Introducing ftdp-17 (mapt) and ftdp-17 (pgrn). Archives of Neurology, 65(4), 460–464. Boxer, A. L., & Boeve, B. F. (2007). Frontotemporal dementia treatment: Current symptomatic therapies and implications of recent genetic, biochemical, and neuroimaging studies. Alzheimer Disease & Associated Disorders, 21(4), S79–S87. Bozeat, S., Lambon Ralph, M. A., Patterson, K., Garrard, P., & Hodges, J. R. (2000). Non-verbal semantic impairment in semantic dementia. Neuropsychologia, 38(9), 1207–1215. Cummings, J. L. (1997). The neuropsychiatric inventory: Assessing psychopathology in dementia patients. Neurology, 48(5 Suppl 6), S10–S16. Diehl, J., Monsch, A. U., Aebi, C., Wagenpfeil, S., Krapp, S., Grimmer, T., et al. (2005). Frontotemporal dementia, semantic dementia, and Alzheimer’s disease: The contribution of standard neuropsychological tests to differential diagnosis. Journal of Geriatric Psychiatry and Neurology, 18(1), 39–44. Gorno-Tempini, M., Dronkers, N., Rankin, K., Ogar, J., La Phengrasamy, B., Rosen, H., et al. (2004). Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology, 55, 335–346. Gregory, C. A., Serra-Mestres, J., & Hodges, J. R. (1999). Early diagnosis of the frontal variant of frontotemporal dementia: How sensitive are standard neuroimaging and neuropsychologic tests? Neuropsychiatry, Neuropsychology, and Behavioral Neurology, 12(2), 128–135. Grossman, M. (2002). Frontotemporal dementia: A review. Journal of International Neuropsychological Society, 8(4), 566–583. Howard, D., & Patterson, K. (1992). Pyramids and palm trees: A test of semantic access from pictures and words. Bury St. Edmonds, Suffolk: Thames Valley Test Company. Kertesz, A., Davidson, W., & Fox, H. (1997). Frontal behavioral inventory: Diagnostic criteria for frontal lobe dementia. Canadian Journal of Neurological Sciences, 24(1), 29–36. Kertesz, A., Davidson, W., McCabe, P., & Munoz, D. (2003). Behavioral quantitation is more sensitive than cognitive testing in frontotemporal dementia. Alzheimer Disease & Associated Disorders, 17, 223–229. Kramer, J. H., Jurik, J., Sha, S. J., Rankin, K. P., Rosen, H. J., Johnson, J. K., et al. (2003). Distinctive neuropsychological patterns in frontotemporal dementia, semantic dementia, and alzheimer disease. Cognitive and Behavioral Neurology, 16(4), 211–218. Lindau, M., Almkvist, O., Kushi, J., Boone, K., Johansson, S. E., Wahlund, L. O., et al. (2000). First symptoms – frontotemporal dementia versus Alzheimer’s disease. Dementia and Geriatric Cognitive Disorders, 11(5), 286–293. Mackenzie, I. R., & Rademakers, R. (2007). The molecular genetics and neuropathology of frontotemporal lobar degeneration: Recent developments. Neurogenetics, 8(4), 237–248. Mesulam, M., Wicklund, A., Johnson, N., Rogalski, E., Leger, G. C., Rademaker, A., et al. (2008). Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Annals of Neurology, 63(6), 709–719. Miller, B. L., Ikonte, C., Ponton, M., Levy, M., Boone, K., Darby, A., et al. (1997). A study of the lund-manchester research criteria for frontotemporal dementia: Clinical and single-photon emission ct correlations. Neurology, 48(4), 937–942. Neary, D., Snowden, J. S., Gustafson, L., Passant, U., Stuss, D., Black, S., et al. (1998). Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria [see comments]. Neurology, 51(6), 1546–1554.

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Perry, R. J., & Hodges, J. R. (2000). Differentiating frontal and temporal variant frontotemporal dementia from Alzheimer’s disease. Neurology, 54(12), 2277–2284. Rascovsky, K., Salmon, D. P., Ho, G. J., Galasko, D., Peavy, G. M., Hansen, L. A., et al. (2002). Cognitive profiles differ in autopsy-confirmed frontotemporal dementia and ad. Neurology, 58(12), 1801–1808. Ratnavalli, E., Brayne, C., Dawson, K., & Hodges, J. R. (2002). The prevalence of frontotemporal dementia. Neurology, 58(11), 1615–1621. Rosso, S. M., Donker Kaat, L., Baks, T., Joosse, M., de Koning, I., Pijnenburg, Y., et al. (2003). Frontotemporal dementia in the netherlands: Patient characteristics and prevalence estimates from a population-based study. Brain, 126(Pt 9), 2016–2022. Seeley, W. W., Bauer, A. M., Miller, B. L., Gorno-Tempini, M. L., Kramer, J. H., Weiner, M., et al. (2005). The natural history of temporal variant frontotemporal dementia. Neurology, 64(8), 1384–1390. The Lund and Manchester Groups. (1994). Clinical and neuropathological criteria for frontotemporal dementia. Journal of Neurology, Neurosurgery & Psychiatry, 57(4), 416–418. Thompson, C., & Johnson, N. (2005). Rehabilitation of language deficits. In D. Koltai & K. Welsh-Bohmer (Eds.), Geriatric neuropsychology: Assessment and intervention. New York: Guilford. Thompson, J. C., Stopford, C. L., Snowden, J. S., & Neary, D. (2005). Qualitative neuropsychological performance characteristics in frontotemporal dementia and Alzheimer’s disease. Journal of Neurology, Neurosurgery & Psychiatry, 76(7), 920–927. Walker, A. J., Meares, S., Sachdev, P. S., & Brodaty, H. (2005). The differentiation of mild frontotemporal dementia from Alzheimer’s disease and healthy aging by neuropsychological tests. International Psychogeriatric Association, 17(1), 57–68. Wicklund, A., & Weintraub, S. (2005). Neuropsychological features of common dementia syndromes. Turk Noroloji Dergisi, 11(6), 566–588. Wicklund, A. H., Johnson, N., Rademaker, A., Weitner, B. B., & Weintraub, S. (2006). Word list versus story memory in Alzheimer disease and frontotemporal dementia. Alzheimer Disease & Associated Disorders, 20(2), 86–92.

FrSBe ▶ Frontal Systems Behavior Scale

Frustration Tolerance DAWN E. B OUMAN Drake Center Cincinnati, OH, USA

Synonyms Short-fuse syndrome

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1102

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FSIQ

Definition Frustration tolerance is the ability to withstand obstacles and stressful situations. Decreased frustration tolerance is a common behavior problem of people who have brain injuries. Low frustration tolerance can be a direct result of brain damage as well as a secondary reaction to the lifestyle changes and losses which might accompany a brain injury. Typically conceptualized as an executive functioning impairment, low frustration tolerance is viewed as a problem with self-regulation. Behavioral manifestations include irritability, aggression, lability, and refusal to participate. As with other behavior problems, difficulties with frustration tolerance are often exacerbated by fatigue.

Cross References ▶ Agitation ▶ Catastrophic Reaction ▶ De-escalation ▶ Emotional Lability ▶ Executive Functioning ▶ Irritability ▶ Self-Regulation

References and Readings Callahan, C. D. (2009). The assessment and rehabilitation of executive function disorders. In B. Johnstone & H. H. Stonnington (Eds.), Rehabilitation of neuropsychological disorders (2nd edn., pp. 75–106). Philadelphia: Psychology Press, Taylor and Francis Group. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th edn., pp. 37, 177). New York: Oxford University Press.

FSIQ ▶ Full Scale IQ ▶ Intelligence ▶ Intelligence Quotient

FSQ ▶ Functional Status Questionnaire

FTT ▶ Finger Tapping Test

Fugl-Meyer Assessment ▶ Fugl-Meyer Assessment of Sensorimotor Impairment

Fugl-Meyer Assessment of Sensorimotor Impairment K ARI D UNNING University of Cincinnati Cincinnati, OH, USA

Synonyms FM; FMA; Fugl-Meyer Assessment

Description The Fugl-Meyer Assessment of Sensorimotor Impairment (FM) is one of the first scales developed to quantitatively measure the recovery from hemiplegic stroke. It assesses recovery in five domains, including motor functioning of the upper and lower extremities, balance, sensation, joint range of motion, and joint pain in post-stroke patients. Each task is scored on a scale of 0–2, with 0 indicating the patient cannot perform the task and 2 indicating the patient can fully perform the task. Each domain is usually assessed individually; approximately 20 min is required to administer and score each section.

Historical Background In 1975, The Fugl-Meyer Assessment Scale was developed by A. R. Fugl-Meyer (Fugl-Meyer et al., 1975).

Psychometric Data Sanford found the FM to be a reliable outcome measure, with an overall intraclass correlation coefficient of 0.96.

Full Scale IQ

ICCs ranged from 0.61 for the pain subsection to 0.97 for the upper extremity motor component. The upper extremity subsection has high concurrent and predictive validity (Spearman rho > 0.81 and 0.51, respectively).

Clinical Uses The FM can be used in the clinical setting as a means of quantitatively tracking improvements in patients with hemiplegic stroke who are undergoing therapy. Because the FM evaluates specific areas of function, any or all sections may be used to assess recovery. (For example, the upper extremity section of the motor component may be used alone, or all domains may be assessed.) The FM should be administered by a trained physical therapist, occupational therapist, or other rehabilitation professional. The minimal detectable change for the upper extremity subsection of the motor domain is 8% of the highest possible score.

References and Readings Beckerman, H., Vogelaar, T. W., Lankhorst, G. J., & Verbeek, A. L. (1996). A criterion for stability of the motor function of the lower extremity in stroke patients using the Fugl-Meyer Assessment Scale Scandinavian. Journal of Rehabilitation Medicine, 28(1), 3–7. Berglund, K., & Fugl-Meyer, A. R. (1986). Upper extremity function in hemiplegia: A cross validation study of two assessment methods. Scandinavian Journal of Rehabilitation Medicine, 18(4), 155–157. Duncan, P. W., et al. (1983). Reliability of the Fugl-Meyer assessment of sensorimotor recovery following cerebrovascular accident. Physical Therapy, 63(10), 1606–1610. Duncan, P. W., et al. (1994). Similar motor recovery of upper and lower extremity after stroke. Stroke, 25(6), 1181–1188. Fugl-Meyer, A. R., Jaasko, L., Leyman I., Olsson S., & Steglind S. (1975). The post stroke hemiplegic patient I. A method for evaluation of physical performance. Scandinavian Journal of Rehabilitation Medicine, 7(1), 13–31. Gladstone, D. J., Dannells, C. J., & Black, S. E. (2002). The Fugl-Meyer Assessment of motor recovery after stroke: A critical review of its measurement properties. Neurorehabilitation and Neural Repair, 16 (3), 232–240. Lin, J. H., et al. (2009). Psychometric comparisons of 4 measure for assessing upper-extremity function in people with stroke. Physical Therapy, 89(8), 840–850. Malouin, F., et al. (1994). Evaluating motor recovery early after stroke: Comparison of the Fugl-Meyer assessment and the Motor Assessment Scale. Archives of Physical Medicine and Rehabilitation, 75, Nov, 1206–1212. Sanford, J., Moreland, J., Swanson, L. R., et al. (1993). Reliability of the Fugl-Meyer assessment for testing motor performance in patients following stroke. Physical Therapy, 73(7), 447–454.

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Full Scale Intelligence Quotient ▶ Full Scale IQ

Full Scale IQ R AEL T. L ANGE British Columbia Mental Health and Addiction Services & University of British Columbia Vancouver, BC, Canada

Synonyms FSIQ; Full scale intelligence quotient

Definition The Full Scale IQ is a score derived from administration of selected subtests from the Wechsler Intelligence Scales designed to provide a measure of an individual’s overall level of general cognitive and intellectual functioning. It is a summary score derived from an individual’s performance on a variety of tasks that measure acquired knowledge, verbal reasoning, attention to verbal materials, fluid reasoning, spatial processing, attentiveness to details, and visual-motor integration.

Current Knowledge Wechsler Intelligence Scales (WIS): The WIS family of tests are some of the most widely used test batteries to assess general intellectual ability in adults aged 16 years or higher (Wechsler Adult Intelligence Scale; WAIS), children aged 6–16 years (Wechsler Intelligence Scale for Children; WISC), and children aged 2–7 years (Wechsler Preschool and Primary Scale of Intelligence; WPPSI). Since the original development of these tests (WAIS, 1955; WISC, 1949; WPPSI, 1967), all three batteries have been revised on several occasions. The most recent revisions were published in 2002 (WPPSI-III), 2003 (WISCIV), and 2008 (WAIS-IV). The predecessor to the original WAIS, WISC, and WPPSI batteries was the Wechsler Bellevue scale (1939 [Form 1], 1946 [Form 2]), designed to measure intellectual abilities in individuals aged 7–69 years (Form 1) and 10–69 years (Form 2).

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Full Scale IQ

History Prior to the development of the Wechsler Bellevue scale, David Wechsler received training and mentorship by a number of influential intelligence theorists (e.g., JM. Cattell, EL. Thorndike, and C. Spearman). Two of Wechsler’s mentors had strong opposing opinions regarding the measurement of intelligence. Spearman emphasized the notion of a general factor of intelligence (g) that was believed to be responsible for how an individual would perform on a variety of tasks. In contrast, Thorndike emphasized the notion that ‘‘intelligence’’ consisted of a variety of different skills and abilities. Wechsler agreed with both perspectives and integrated these two ideas into the Wechsler Bellevue scale. The Wechsler Bellevue scale emphasized the measurement of g by inclusion of the Full Scale IQ, a score obtained by summating a person’s performance on 11 individual subtests. However, Wechsler also introduced the concept of the Verbal IQ and Performance IQ scores in an effort to differentiate between the contributions of verbal and nonverbal intellectual abilities toward the measurement of g. Wechsler later emphasized the importance of interpreting Verbal IQ and

Performance IQ scores, as well as profile analysis of the individual subtests (see Tulsky, Saklofske, & Ricker, 2003 for an excellent discussion).

Evolution Since the development of the Wechsler Bellevue scale in 1939, the WIS family of tests have undergone many changes. Although very little change actually occurred in the first 5 decades, the publication of the WISC-III in 1991 sparked a series of significant modifications to all subsequent third and fourth edition WIS batteries. Factor-based interpretation was first included in the WISCIII in 1991 (i.e., Verbal Comprehension Index, Perceptual Organization Index, Freedom From Distractibility Index, and Processing Speed Index) and then later in the WAISIII (and to some degree the WPPSI-III). The index scores were initially introduced as an ‘‘alternative’’ system for scoring and interpretation that coexisted with the three traditional IQ scores (i.e., Full Scale IQ, Verbal IQ, Performance IQ). However, in the publication of the WISCIV and WAIS-IV, Verbal IQ and Performance IQ scores were excluded for the first time, and only the Full Scale IQ

Full Scale IQ. Table 1 Core subtest composition for Full Scale IQ IN DSP VO AR CO SI WR RV LNS PC

PA BD OA DSY MR MZ AH GD PCN VP SS

WB-F1

·

·

WB-F2

·

·

WAIS

·

·

WAIS-R

·

WAIS-III WAIS-IV WISC WISC-R WISC-III

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·

·

·

·

·

·

·

·

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WISC-IV

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WPPSI WPPSI-R a

WPPSI-III

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

·

WPPSI-IIIb ·

· ·

·

·

·

·

·

·

· · ·

·

·

·

·

·

· · ·

WB-F1 ¼ Wechsler Bellevue Scale-Form 1; WB-F2 ¼ Wechsler Bellev’ue Scale-Form 2; WAIS ¼ Wechsler Adult Intelligence Scale; WISC ¼ Wechsler Intelligence Scale of Children; WPPSI ¼ Wechsler Preschool and Primary Scale of Intelligence; IN ¼ Information; DSP ¼ Digit Span; VO ¼ Vocabulary; AR ¼ Arithmetic; CO ¼ Comprehension; SI ¼ Similarities; WR ¼ Word Reasoning; RV ¼ Receptive Vocabulary; LNS ¼ Letter Number Sequencing; PC ¼ Picture Completion; PA ¼ Picture Arrangement; BD ¼ Block Design; OA ¼ Object Assembly; DSY ¼ Digit Symbol/ Coding; MR ¼ Matrix Reasoning; MZ ¼ Mazes; AH ¼ Animal House; GD ¼ Geometric Designs; PCN ¼ Picture Concepts; VP ¼ Visual Puzzles; SS ¼ Symbol Search a Ages 2:6–3:11 b Ages 4:0–7:3

Functional Ambulation Classification

score was retained. The exclusion of the Verbal IQ and Performance IQ scores represents a significant deferment from the Wechsler scale tradition, with test interpretation largely focused on the Index scores. It is interesting to note that the Full Scale IQ is the only summary score that has been retained by all versions and editions of the WIS batteries since the original development of the Wechsler Bellevue scale.

Subtest composition The core subtests used to derive Full Scale IQ vary across the WIS batteries and revisions (Table 1). Although there has been some consistency in core subtests that have contributed towards calculation of the Full Scale IQ score on the various revisions of the WAIS and WISC batteries (i.e., information, vocabulary, arithmetic, similarities, picture completion, picture arrangement, block design, and digit symbol/coding), the publication of the WAIS-IV and WISC-IV marks a substantial deferment from this trend, with (a) the inclusion of new core subtests (e.g., WAIS-IV: symbol search, visual puzzles; WISC-IV: symbol search, picture concepts, and letter-number sequencing) and (b) removal of long standing core subtests (WAIS-IV: comprehension, picture completion, and picture arrangement; WISC-IV: information, arithmetic, picture completion, picture arrangement, and object assembly) (Table 1). For the WPPSI, there has been little consistency in the core subtests used to derive the Full Scale IQ score. The core subtests used to derive Full Scale IQ across the WIS batteries and revisions are presented below. Note that only the core (not supplementary) subtests are presented.

Cross References ▶ Intelligence ▶ Perceptual Organization Index ▶ Performance IQ ▶ Stanford–Binet Intelligence Scale and Revised Versions ▶ Verbal Comprehension Index ▶ Verbal IQ ▶ Wechsler Adult Intelligence Scale (All Versions) ▶ Wechsler Intelligence Scale for Children ▶ Wechsler Preschool and Primary Scale of Intelligence

References and Readings Atkinson, L. (1991). Some tables for statistically based interpretation of WAIS-R factor scores. Psychological Assessment, 3(2), 288–291.

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Kaufman, A. S., & Lichtenberger, E. O. (2006). Assessing adolescent and adult intelligence (3rd ed.). Hoboken, NJ: Wiley. Sattler, J. M. (2008). Assessment of children: Cognitive foundations (5th ed.). San Diego, CA: Author. Tulsky, D. S., Saklofske, D. H., & Ricker, J. H. (2003). Historical overview of intelligence and memory: Factors influencing the Wechsler Scales. In D. S. Tulsky, (Eds.), Clinical interpretation of the WAIS-III and WMS-III (pp. 7–41). San Diego, CA: Academic Press. Tulsky, D. S., Saklofske, D. H., & Zhu, J. (2003). Revising a standard: An evaluation of the origin and development of the WAIS-III. In D. S. Tulsky, et al. (Eds.), Clinical interpretation of the WAIS-III and WMS-III (pp. 43–92). San Diego, CA: Academic Press.

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Functional Abilities ▶ Activities of Daily Living (ADL)

Functional Adjustment ▶ Functional Compensation

Functional Ambulation Classification G AVIN W ILLIAMS Epworth Hospital Melbourne, Vic, Australia

Synonyms FAC

Definition The Functional Ambulation Classification (FAC) was an early method for classifying mobility. The primary aim in the development of the FAC was to establish a clinically meaningful outcome measure of mobility. Secondary aims were to devise an inexpensive measure that required little time for therapist training and administration, yet was reliable and valid.

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Current Knowledge The FAC was developed in a cohort of 61 persons with stroke or multiple sclerosis. The FAC has six categories ranging from 0 (non-functional ambulation) to 5 (independent). The intermediary categories quantify levels of assistance, supervision, and independent but limited mobility. Assessors are required to observe performance over various slopes and surfaces. Spatio-temporal parameters (i.e., velocity, cadence, stride length) were correlated with the classification categories to investigate validity. Moderate to strong correlations were found between spatio-temporal parameters and the functional ambulation categories, supporting the validity of the scales. The FAC has high inter-rater and retest reliability. It is relatively quick and easy to score, requiring approximately 5–10 min to administer.

Cross References ▶ Rivermead Mobility Index (RMI)

References and Readings Holden, M., Gill, K., Magliozzi, M., Nathan, J., & Piehl-Baker, L. (1984). Clinical gait assessment in the neurologically impaired: reliability and meaningfulness. Physical Therapy, 64, 35–40.

Functional assessment is a decision process that results from the interaction between classifications such as diagnosis and measures. It aims to recognize, anticipate, or modify the interaction between the disabled person and his or her environment using measures of such factors as independence, pain, cognitive capacity, or fatigability. Functional assessments have become a cornerstone of rehabilitation medicine, assisting in well-defined aims such as quality assurance, continuous quality improvement, accountability, cost–benefit analysis, education, and research.

References and Readings Ring, H. (2007). Functional assessment in rehabilitation medicine: Clinical applications. Europa Medicophysica, 43(4), 515–523. Tesio, L. (2007). Functional assessment in rehabilitative medicine: Principles and methods. Europa Medicophysica, 43(4), 551–556.

Functional Assessment Measure J ERRY W RIGHT Santa Clara Valley Medical Center San Jose, CA, USA

Synonyms FAM; FIM þ FAM; UK FAM

Functional Amnesia ▶ Dissociative Amnesia

Functional Assessment S UE A NN S ISTO Stony Brook University, School of Health Technology and Management Stony Brook, NY, USA

Definition Functional assessment encompasses the measurement of medical, physical, and mental health. Through functional assessments, clinicians develop an understanding of an individual’s condition and activities and how they use critical skills to be successful and satisfied in their environment.

Description The Functional Assessment Measure (FAM) was developed as an adjunct to FIM™ (aka the Functional Independence Measure) to address functional areas of importance in brain injury rehabilitation that are less emphasized in FIM™, including communication, psychosocial adjustment, and cognition. The FAM consists of 12 items that are intended to be added to the 18 items of FIM™, creating a 30-item scale referred to as the FIM þ FAM. The time required to administer the FIM þ FAM is approximately 35 min. The 7-point rating scale is modeled after FIM™ and assesses the individual’s level of independence, amount of assistance required, use of adaptive or assistive devices, and the percentage of a given task completed successfully. The FAM is a nonproprietary measure; however FIM™ is proprietary. The FAM can be completed in person, by phone, or by retrospective chart review. A decision tree is available for each item to aid in rating.

Functional Assessment Measure

Administration time for the FAM is usually under 10 min. Administration for the FIM þ FAM is usually around 30 min. Training and certification for the FAM is available at the Center for Outcome Measurement in Brain Injury (www.tbims.org/combi/FAM).

Historical Background The FAM was originally developed as a clinical tool at Santa Clara Valley Medical Center in the 1980s. The FAM items were proposed by clinicians representing each discipline in an inpatient rehabilitation program. The FAM items were added to FIM™ to provide more detailed data appropriate for a traumatic brain injury (TBI) and stroke (CVA) population. The FAM was adopted for use in the National Institute of Disability and Rehabilitation Research (NIDRR) Traumatic Brain Injury Model Systems (TBIMS) National Database in 1988 and discontinued in 1999 when researchers decided that the additional time necessary to complete the FAM was not worth the additional information provided. In 1995 a FIM þ FAM users group set out to develop a UK version of the FAM, keeping the seven-level structure, but attempting to improve the objectivity of scoring. They found that the overall scoring accuracy of vignettes was improved for the UK version over the original FAM on the ten ‘‘troublesome’’ items. Information on the FAM and the UK FAM is available at the Center for Outcome Measurement in Brain Injury web site.

Psychometric Data Validity: Rasch analyses were completed on the FAM items at rehabilitation admission and discharge for data collected in the TBI National Database. FAM items rated at rehabilitation admission correlated significantly with indices of injury severity in a very similar pattern as FIM™ items. Use of untransformed ratings should be adequate for most research and clinical purposes. The FAM does not appear to contribute beyond FIM™ in predicting length of inpatient stay or costs. The FAM does appear to have increased sensitivity beyond FIM™ alone for inpatient rehabilitation discharge and post-acute rehabilitation functional assessment. There is evidence that the FAM at rehabilitation discharge is less susceptible to a ‘‘ceiling effect’’ than FIM™ and is also more strongly related to rehabilitation charges.

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In a prospective study of individuals with TBI at 6 and 24 months post-injury, FAM scores predicted return to work and community integration. Overall, the FAM did not provide any additional information over FIM™ except at 24 months, where the FAM cognitive scores slightly improved the prediction of return to work. Reliability: Preliminary data suggest that FAM items involving abstract concepts such as ‘‘attention’’ tend to be less reliable than directly observable behaviors. As a test of the FAM items’ interrater reliability, FAM training and testing vignettes (which also included FIM™ items) were completed by trained raters, including a PT, OT, COTA, data analyst, and researcher. Each item’s three vignettes were written to cover low, intermediate, and high functioning levels within the 1–7 scale. The percentage of rating agreement was 89%. The Kappa score for FIM™ was 0.87, and for the FAM, 0.85, both within the ‘‘very good’’ Kappa range. An examination of interclass correlation coefficients found 9 of the 12 FAM measures in the ‘‘excellent’’ range (0.75) and the remaining within the ‘‘good’’ range (0.60–0.74). Rasch analysis demonstrated high internal consistency and reliability for motor and cognitive subscale scores.

Clinical Uses The FAM is primarily used to document admission and discharge functional status for acute and post-acute rehabilitation for individuals with brain injury/stroke. It has also been used to examine day program outcomes, gait retraining, ability to return to driving, and the impact of bilateral hand loss.

Cross References ▶ Center for Outcome Measurement in Brain Injury ▶ Traumatic Brain Injury ▶ Traumatic Brain Injury Model System

References and Readings Center for Outcome Measurement in Brain Injury, http://www.tbims. org/combi/FAM Hall, K. M. (1997). The functional assessment measure (FAM). Journal of Rehabilitation Outcomes Measurement, 1, 63–65.

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Functional Assessment Multiple Sclerosis

Hall, K. M., Bushnik, T., Lakisic-Kazazic, B., Wright, J., & Cantagallo, A. (2001). Assessing traumatic brain injury outcome measures for longterm follow-up of community-based individuals. Archives of Physical Medicine and Rehabilitation, 82, 367–374. Hall, K. M., Hamilton, B. B., Gordon, W. A., & Zasler, N. D. (1993). Characteristics and comparisons of functional assessment indices: Disability rating scale. Functional independence measure, and functional assessment measure. Journal of Head Trauma Rehabilitation, 8, 60–74. Hall, K. M., Mann, N., High, W. M. Jr., Wright, J., Kreutzer, J. S., & Wood, D. (1996). Functional measures after traumatic brain injury: Ceiling effects of FIM, FIMþFAM, DRS, and CIQ. Journal of Head Trauma Rehabilitation, 11, 27–39. Seel, R. T., Wright, G., Wallace, T., Newman, S., & Dennis, L. (2007). The utility of the FIMþFAM for assessing traumatic brain injury day program outcomes. Journal of Head Trauma Rehabilitation, 22, 267–277. Turner-Stokes, L., Nyein, K., Turner-Stokes, T., & Gatehouse, C. (1999). The UK FIMþFAM:development and evaluation. Clinical Rehabilitation, 13, 277–287.

Functional Assessment Multiple Sclerosis V ICTOR R. P REEDY King’s College London London, UK

Synonyms FAMS

Definition A disease-specific self-report questionnaire that investigates six primary aspects of quality of life in patients with multiple sclerosis: mobility, symptoms, emotional well-being, general contentment, thinking/fatigue, and family/social well-being. Five of the six subcategories (mobility, symptoms, emotional well-being, general contentment, and family/social well-being) comprise seven items (score range: 0–28); the subcategory thinking/fatigue comprises nine items (score range: 0–36).

Cross References ▶ Multiple Sclerosis ▶ Multiple Sclerosis Quality of Life-54 Questionnaire

Acknowledgment This contribution originally published in Preedy and Watson (2010).

References and Readings Preedy, V. R., & Watson, R. R., (Eds.). (2010). Handbook of disease burdens and quality of life measures (Vol. 6., p. 4211). New York: Springer.

Functional Autonomy Measurement System J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms SMAF (from the original title ‘‘Syste`me de Mesure de l’Autonomie Fonctionnelle’’)

Description The Functional Autonomy Measurement System (SMAF) contains 29 items designed to fit the World Health Organization International Classification of Functioning, disability and health (WHO ICF) classification of impairments, disabilities, and handicap to assess functional problems in geriatric populations. Items cover the following domains: activities of daily living (ADLs), mobility, communication, mental functions, and instrumental ADLs. Ability was traditionally scored at one of four levels (autonomous, needs supervision/stimulation, needs help, and dependent), but a revision of the scale added an intermediate level indicating that a function was completed independently, but with difficulty (Desrosiers et al., 1995). Ratings should be made based on a person’s performance rather than his/her potential, and should also be made in light of any environmental modifications that may reduce handicap. Raters should use as much information as possible in making their judgments, such as interviewing and observing the patient and a family member or caregiver, and asking patients to perform relevant tasks. Scores can be sorted into 1 of 14

Functional Autonomy Measurement System

classifications of disability, indicating the level of support a patient requires (Dubuc, Hebert, Desrosiers, Buteau, & Trottier, 2006).

Historical Background Herbert, Guilbault, Desrosiers, and Dubuc (2001) state that the original SMAF was developed in 1984 and revised in 1993. It is extensively used in Quebec, Canada. A computerized version is available (Boissy, Briere, Tousignant, & Rousseau, 2007), and an additional social subscale has been developed (Pinsonnault et al., 2003).

Psychometric Data Herbert et al. (1988) in an inter-rater reliability study for the original SMAF, involving 150 community-based patients, found reasonably high agreement between two trained raters for the whole scale, with a weighted kappa of 0.75, and values between 0.47 and 0.81 for 27 of the individual items, with only 2 items achieving lower values. The revised SMAF (with the 0.5 ‘‘with difficulty’’ scoring level) was found to have similar inter-rater reliability, along with excellent internal consistency (intra-class correlations r > 0.95). There is considerable evidence regarding the validity of the SMAF, for example, through demonstrated relations between SMAF scores and the amount of health care resources required by an individual in a group of institutionalized elderly people (for a review see Herbert et al., 2001). The SMAF’s responsiveness to change has been examined by comparing change scores on the SMAF and other functional outcome measures (Barthel Index and Functional Independence Measure [FIM]) from a group of patients undergoing active rehabilitation with those from a group of people not participating in rehabilitation, whose autonomy would be expected to be stable. The SMAF seemed to be slightly more responsive than the other measures, but the differences were not significant (Langlais reported in Herbert et al., 2001). Similar findings were reported by Desrosiers et al. (2003) for people recovering from stroke, that is, the subscales of the FIM and SMAF differ somewhat in their responsiveness, but overall, both measures seem to respond well. The ISO-SMAF disability classification system has been demonstrated to be both reliable and valid, even accounting for around 80% of the variance in nursing time and nursing costs between patients. This attests to its

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validity and the clinical utility. The social subscale of the SMAF has been shown to possess adequate to good interrater reliability. Specifically, Pinsonnault et al. (2003) reported that in two separate studies, the mean percentage of scores that agreed was 60% and 74%, and mean intraclass correlations of r = 0.70 and r = 0.83. There is also some evidence of the social SMAF’s validity in terms of moderate correlations with other measures including a social functioning component (Pinsonnault, Dubuc, Desrosiers, Delli-Colli, & Hebert, 2009).

F Clinical Uses The SMAF is widely used in Quebec, Canada, to determine an individual’s needs and to assist in decision-making regarding home support or admission to residential institutions. The SMAF includes a care chart that can be used in institutional settings so that care staff can quickly find out a patient’s care needs without needing to ask them directly. The addition of a subscale assessing social functioning and the availability of software to aid scoring the SMAF are likely to further increase the clinical utility of the SMAF.

Cross References ▶ Barthel Index ▶ Functional Independence Measure

References and Readings Boissy, P., Briere, S., Tousignant, M., & Rousseau, E. (2007). The eSMAF: A software for the assessment and follow-up of functional autonomy in geriatrics. BMC Geriatrics, 7, 2 [epub]. Desrosiers, J., Bravo, G., & He´bert, R., Dubuc, N. (1995). Reliability of the revised functional autonomy measurement system (SMAF) for epidemiological research. Age Ageing, 24, 402–406. Desrosiers, J., Rochette, A., Noreau, L., Bravo, G., Hebert, R., & Boutin, C. (2003). Comparison of two functional independence scales with a participation measure in post-stroke rehabilitation. Archives of Gerontology and Geriatrics, 37, 157–172. Dubuc, N., Hebert, R., Desrosiers, J., Buteau, M., & Trottier, L. (2006). Disability-based classification system for older people in integrated long-term care services: The Iso-SMAF profiles. Archives of Gerontology and Geriatrics, 42, 191–206. He´bert, R., Carrier, R., & Bilodeau, A. (1988). The functional autonomy measurement system (SMAF): Description and validation of an instrument for the measurement of handicaps. Age Ageing, 17, 293–302. Herbert, R., Guilbault, J., Desrosiers, J., & Dubuc, N. (2001). The functional autonomy measurement system (SMAF): A clinical-based

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instrument for measuring disabilities and handicaps in older people. Geriatrics Today, September. Pinsonnault, E., Desrosiers, J., Dubuc, N., Kalfat, H., Colvez, A., & Delli-Colli, N. (2003). Functional autonomy measurement system: Development of a social subscale. Archives of Gerontology and Geriatrics, 37, 223–233. Pinsonnault, E., Dubuc, N., Desrosiers, J., Delli-Colli, N., & Hebert, R. (2009). Validation study of a social functioning scale: The socialSMAF (social-Functional Autonomy Measurement System). Archives of Gerontology and Geriatrics, 48, 40–44.

Gross, D. P., Battie, M. C. (2006). Does functional capacity evaluation predict recovery in workers’ compensation claimants with upper extremity disorders? Occupational and Environmental Medicine, 63, 404–410. Pransky, G. S., Dempsey, P. G. (2004). Practical aspects of functional capacity evaluations. Journal of Occupational Rehabilitation, 14, 217–229.

Functional Compensation Functional Capacity Evaluations TAMARA B USHNIK NYU Langone Medical Center New York, NY, USA

L INDSEY D UCA , J ON R OSE VA Palo Alto Health Care System Palo Alto, CA, USA

Synonyms Functional adjustment

Synonyms FCEs

Definition The measurement of capacity as it pertains to demands that are specific to a job and/or task. FCEs traditionally evaluate the match between the individual’s abilities and/or limitations and the functional ability required to adequately address the needs of the job/workplace. An individual’s abilities and/or limitations includes, not only consideration of anthropometrics, biomechanics, and cardiopulmonary factors, but also perceptual and cognitive abilities that are required to accomplish the job and/or task. The most common uses of FCEs are following an injury (not necessarily at the workplace) when an individual needs to be assessed for return to work possibilities, work accommodations, and/or the need for rehabilitation services. Factors influencing the validity of an FCE include involvement in legal action, lack of standardized tools, and the difficulty in assessing motivation and effort expended by the individual being assessed.

References and Readings Gross, D. P., Battie, M. C. (2005). Functional capacity evaluation performance does not predict sustained return to work in claimants with chronic back pain. Journal of Occupational Rehabilitation, 15, 285–294.

Definition Functional compensation refers to the process by which individuals who have suffered damage to the central nervous system (CNS) resulting in permanent injury compensate for deficits in various domains of functioning through the adaptive implementation of behavioural, cognitive, or physical strategies designed to enhance residual skills or to introduce alternative skills. Functional compensation uses residual structures to achieve recovery, emphasizing a behavioural rather than a neural model (Heilman & Valenstein, 2003). Instead of, or in complement to, rerouting neuronal connections, an individual with CNS damage independently develops, or is assisted in learning, new solutions to problems using existing structures.

Current Knowledge In working with an individual with CNS damage, rigorous assessment procedures maximize a clinician’s ability to identify areas of residual function, either motor, sensory, cognitive, or praxic, as a means of developing targets for rehabilitation and subsequent implementation of compensatory techniques (Robertson & Murre, 1999). The fundamental goal of assisting an individual in maximizing strategies for functional compensation is to return as closely as possible to a level of pre-morbid functioning, using different means to accomplish similar goals. Due to the range of possible impairment in

Functional Imaging

individuals with neurological injuries, acquisition of new skills can be supported by a variety of rehabilitation specialists, including speech pathologists, social workers, psychologists, and physical and occupational therapists. Specific to psychological interventions, clinicians might work with CNS-damaged individuals to employ strategies such as cognitive remediation, cognitive or behavioural self-regulation, and environmental modification to address changes in functioning in areas such as orientation, attention/concentration, visual perceptual processes, organization, learning and memory, problemsolving, executive control, communication, and selfmonitoring. As an example, an individual who has experienced an injury impacting memory might be taught to use strategies such as breaking new material into small portions or taking succinct notes while reading as a means of compensating for cognitive deficits. An individual with an injury impacting inhibition might be taught strategies for slowing down reaction times by rehearsing responses internally before verbalizing.

Cross References ▶ Behaviorism ▶ Cognitive Rehabilitation ▶ Compensatory Strategies ▶ Holistic Brain Injury Rehabilitation ▶ Neuropsychological Rehabilitation ▶ Postacute Brain Injury Rehabilitation ▶ Rehabilitation Psychology

References and Readings Department of Veterans Affairs. (2004). Traumatic brain injury. Washington, D.C.: Department of Veteran’s Affairs Employee Education System. Heilman, K. M., & Valenstein, E. (Eds.) (2003). Clinical Neuropsychology (4th ed.). New York: Oxford University Press. Robertson, I. H., & Murre, J. M. J. (1999). Rehabilitation of brain damage: brain plasticity and principles of guided recovery. Psychological Bulletin, 125(5), 544–575.

Functional Disorder ▶ Psychogenic Disorder

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Functional Imaging R OSS Z AFONTE 1, B RAD K UROWSKI 2 1 Harvard Medical School Boston, MA, USA 2 Cincinnati Children’s Hospital Medical Center Cincinnati, OH, USA

Synonyms Functional neuroimaging

Definition Imaging techniques used to noninvasively evaluate the brain-behavior relationship; specifically, they attempt to correlate cognition and behavior with neural activity.

Current Knowledge Functional imaging is typically divided into two broad categories of imaging: ‘‘resting’’ and ‘‘activated.’’ Resting studies are obtained during non-dynamic or static states; however, they are taken in close temporal relationship to a specific activity (i.e., cognitive or motor), but not during the activity. Conversely, activation studies evaluate neural activity during a specific cognitive or motor task or in response to sensory input. Because cerebral blood flow, metabolic activity, and electrical activity are known to be correlated with neural activity, most functional imaging techniques evaluate one of these physiologic processes. Functional imaging techniques have most commonly been used in the study primary brain dysfunction or injury, including strokes and traumatic brain injuries, but they have also been used in the study of spinal cord injury (SCI). They have been used to correlate brain patterns and activity in cognitive, motor, speech-language, and visuospatial recovery. They have also been used to evaluate mood. Examples of commonly used functional imaging techniques include, single-photon emission computed tomography (SPECT), positron emission tomography (PET), functional magnetic resonance imaging (fMRI), magnetic resonance spectroscopy (MRS), electroencephalography (EEG), magnetoencephalography (MEG), and transcranial Doppler (TCD).

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Cross References ▶ Electroencephalography ▶ Functional Magnetic Resonance Imaging ▶ Magnetoencephalography ▶ Magnetic Resonance Spectroscopy ▶ PET ▶ Single-Photon Emission Computed Tomography ▶ Transcranial Doppler

References and Readings Berns, G. S. (1999). Minireview: Functional neuroimaging. Life Sciences, 65(24), 2531–2540. Buxton, R. (2002). Introduction to functional magnetic resonance imaging: Principles & techniques. Cambridge, UK: Cambridge University Press. Ernst, M., & Rumsey, J. (2000). Functional neuroimaging in child psychiatry. Cambridge, UK: Cambridge University Press. Metting, Z., Rodiger, L., DeKeyser, J., & Naalt, J. (2007). Structural and functional neuroimaging in mild-to-moderate head injury. Lancet Neurology, 6, 699–710. Ricker, J., & Arenth, P. (2007). Chapter 12: Functional neuroimaging of TBI. In N. Zasler, D. Katz, & R. Zafonte (Eds.), Brain Injury Medicine (p. 149). New York, NY: Demos Medical Publishing, LLC. Turner, R., & Jones, T. (2003). Techniques for imaging neuroscience. British Medical Bulletin, 65, 3–20.

The FIM is an 18-item ordinal scale that can be used with all diagnoses within rehabilitation. Individual FIM item scores range from 1 (‘‘total assist’’; performs less than 25% of task) to 7 (‘‘complete independence’’). Scores falling below 6 require another person for supervision or assistance. The FIM measures independent performance in self-care, sphincter control, transfers, locomotion, communication, and social cognition. By adding the points for each item, the possible total score ranges from 18 (lowest) to 126 (highest) level of independence. During rehabilitation, admission and discharge scores are rated by clinicians observing patient function. Functioning post discharge can be accurately assessed using a telephone version of FIM(TM) when administered by qualified, trained interviewers. It is most useful for assessment of progress during inpatient rehabilitation. The scale is prone to ceiling effects when applied to samples living in the community. The FIM is a proprietary measure, requiring trained and certified raters. A structured interview or decision tree has been developed, which can be administered in person or over the phone. Additional information on the FIM is available at the Center for Outcome Measurement in Brain Injury (www. tbims.org/combi/FIM).


Functional Independence Measure J ERRY W RIGHT Santa Clara Valley Medical Center San Jose, CA, USA

Description The Functional Independence Measure (FIM)(TM) (Guide for the Uniform Data Set for Medical Rehabilitation, 1996) is the most widely accepted functional assessment measure in use in the rehabilitation community. For over 15 years, the FIM was an acronym for ‘‘Functional Independence Measure.’’ It is still often cited as this in the literature. Uniform Data System for Medical Rehabilitation (UDSMR), the current owners of the FIM™ instrument, do not want the instrument to be referenced as the Functional Independent Measure, but rather only as the FIM™ instrument.

The FIM was developed to resolve the long-standing problem of lack of uniform measurement and data on disability and rehabilitation outcomes (Granger, 1998). The FIM emerged from a thorough developmental process sponsored by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation (Granger et al., 1986). A national task force reviewed 36 published and unpublished functional assessment scales before agreeing on an instrument (Hamilton et al., 1987).

Psychometric Data The metric properties of the FIM have been reported extensively (Granger et al., 1993; Heinemann et al., 1993). The FIM(TM) has clinically appropriate validity and interrater agreement (Hamilton et al., 1991). Ceiling effects of the FIM(TM) at rehabilitation discharge, and particularly at 1 year post injury, were observed in the moderately and severely injured TBI population (Hall et al., 1996). Forty-nine percent and 84% of

Functional Independence Measure for Children

the sample had attained independence (average score of 7 or 6) by discharge and 1 year post injury, respectively. In other words, the FIM(TM) is not sensitive to more subtle changes expected after acute inpatient rehabilitation discharge.

Clinical Uses The FIM is a useful summary measure or global assessment following a brain injury. Many clinicians are familiar with the instrument and what the individual scores mean. FIM scores may be tied to reimbursement for some patients and some facilities.

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measure (FIM). Archives of Physical Medicine and Rehabilitation, 72, 790 (abstract). Heinemann, A. W., Linacre, J. M., & Hamilton, B. B. (1984). Prediction of rehabilitation outcomes with disability measures. Archives of Physical Medicine and Rehabilitation, 75, 133–143. www.udsmr.org

Functional Independence Measure for Children B ETH S LOMINE Kennedy Krieger Institute Baltimore, MD, USA

Synonyms Cross References ▶ Center for Outcome Measurement in Brain Injury ▶ Traumatic Brain Injury ▶ Traumatic Brain Injury Model System

References and Readings Center for Outcome Measurement in Brain Injury (www.tbims.org/ combi/FIM). Granger, C. V. (1998). The emerging science of functional assessment: Our tool for outcomes analysis. Archives of Physical Medicine and Rehabilitation, 79(3), 235–240. Granger, C. V., Hamilton, B. B., Keith, R. A., Zielesny, M., & Sherwin, F. S. (1986). Advances in functional assessment for medical rehabilitation. Topics in Geriatric Rehabilitation, 1, 59–74. Granger, C. V., Hamilton, B. B., Linacre, J. M., Heinemann, A. W., & Wright, B. D. (1993). Performance profiles of the functional independence measure. American Journal of Physical Medicine & Rehabilitation, 72, 84–89. Guide for the Uniform Data Set for Medical Rehabilitation (including the FIM(TM) instrument), (1997). Version 5.1. Buffalo, NY 14214–3007: State University of New York at Buffalo. Hall, K. M., Bushnik, T., Lakisic-Kazazic, B. et al. (2001). Assessing traumatic brain injury outcome measures for long-term follow-up of community-based individuals. Archives of Physical Medicine and Rehabilitation, 82, 367–374. Hall, K. M., Mann, N., High, W. M., et al. (1996). Functional measures after traumatic brain injury: ceiling effects of FIM, FIMþFAM, DRS, and CIQ. Journal of Head Trauma Rehabilitation, 11, 27–39. Hamilton, B. B., Granger, C. V., Sherwin, F. S., Zielezny, M., & Tashman, J. S. (1987). A uniform national data system for medical rehabilitation. In M. Fuhrer (Ed.), Rehabilitation outcomes: Analysis and measurement (pp. 137–147). Baltimore: Brookes. Hamilton, B. B., Laughlin, J. A., Granger, C. V., & Kayton, R. M. (1991). Interrater agreement of the seven-level functional independence

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WeeFIM®; WeeFIM II®

Description The WeeFIM® is a measure of functional ability that can be used for typically developing children, aged 6 months through 7 years, as well as children over 7 years with disabilities and delays in functional development. It is an 18-item performance measurement system that documents self-care, functional mobility, and cognitive abilities. The self-care domain includes 8 items (eating, grooming, bathing, lower and upper body dressing, toileting, as well as bowel and bladder control). The mobility domain includes 5 items (chair, toilet, and tub transfers, walking or wheelchair management, and stairs). The cognitive domain includes 5 items (language comprehension and expression, social interaction, problem solving, and memory). The WeeFIM® was designed to measure outcome and change in functional status over time. Based on direct observation or caregiver report obtained by a trained examiner, WeeFIM® items are rated on a 7-level ordinal scale, where a 1 represents the need for total assistance and a 7 represents complete independence. Scores are derived for the three domains as well as a total score; the latter ranges from 18 to 126. Given that independence increases with age, a total functional quotient for each domain as well as the total score can be calculated based on normative data for children of different ages. Specific criteria for rating each item are provided in the clinical guide (Uniform Data Systems for Medical Rehabilitation, 2005). A description of the general criteria for rating items is provided in Table 1:

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Functional Independence Measure for Children. Table 1 General Level Descriptions for the WeeFIM® Rating Label

Description

7

Complete The child performs all tasks independence without assistance from a helper or device in a safe manner and reasonable amount of time.

6

Modified The child performs all tasks independence without assistance from a helper, and one or more of the following are true: The child requires an assistive device to perform tasks. The child requires a prosthesis or orthosis that is necessary for performing tasks. The child takes extra time to perform tasks. There is a concern for the child’s safety when performing tasks.

5

Supervision or The child performs all tasks but setup requires supervision (typically standing by, cuing, and coaxing) or setup (e.g., setting out necessary items and helping to apply a prosthesis or orthosis.

4

Minimal assistance

The child performs 75% or more of tasks, requiring no more help than touching.

3

Moderate assistance

The child performs 50–74% of tasks, requiring physical assistance beyond touching.

2

Maximal assistance

The child performs 25–49% of tasks.

1

Total assistance

One or both of the following are true: The child performs less than 25% of tasks. The child requires assistance from two helpers to perform tasks.

Modified from Uniform Data System for Medical Rehabilitation (2006)

Historical Background The WeeFIM® was developed by the Uniform Data System for Medical Rehabilitation (UDSMR) in 1987 by a multidisciplinary team consisting of physicians, nurses, and therapists. It was first described in the literature by Msall and colleagues who examined the WeeFIM® in

typically developing children (Msall, DiGaudio, Duffy, LaForest, Braun, & Granger, 1994) and children with developmental disabilities (Msall et al., 1994). Given the solid reliability and validity in both of these early studies, the WeeFIM® was thought to be a useful instrument for measuring functional disability in children. The WeeFIM® was based on the format of the Functional Independence Measure (FIMTM), a measure of functioning in adults medical rehabilitation inpatients with acquired disabilities (Granger, Hamilton, Keith, Zielenzy, & Sherwin, 1986). While having items similar to those on the FIMTM, the WeeFIM® contains a limited set of essential items to measure consistent and actual performance through discipline-free observations in order to track outcome across settings. In 1994, the UDSMR developed a pediatric subscriber service so that pediatric inpatient and outpatient facilities could obtain a license to use the WeeFIM® to collect data for submission to UDSMR where data are aggregated. Subscribing facilities are provided reports that allowed them to compare their facilities to other similar facilities. Based on subscriber feedback, several modifications were made to the WeeFIM® system. In 2004, the WeeFIM II system was launched. While the 18 items remained, the definitions and descriptors of many items were clarified in the revised tool. The current WeeFIM II is Version 6.0.

Psychometric Data In an early normative study (Msall et al., 1994), 417 typically developing children (ages 6 months to 8 years) were administered the WeeFIM® through face-to-face interviews with caregivers conducted by trained pediatric nurse practitioners. Normative data from the original publication by Msall, DiGaudio, Duffy, and et al. (1994) are available for the three individual domains (self-care, mobility, and cognition) for children aged 5 months to greater than 83 months, although the sample size for each age group is small (6 to 29 children). Based on the original WeeFIM®, the test–retest and inter-rater reliability of the WeeFIM® are high in both children who are typically developing (Msall et al., 1994) and those with developmental disabilities (Msall et al., 1994). There was a significant correlation between age of the child and total WeeFIM® score, revealing increasing independence with increasing chronological age (Msall et al., 1994). Principal components analysis suggests distinct motor and cognitive scales (Chen, Bode, Granger, & Heinenmann, 2005). Studies have revealed moderate to strong

Functional Magnetic Resonance Imaging

correlations with other standardized measures of functional ability such as the Vineland Adaptive Behavior Scale and the Battelle Developmental Inventory Screening Test (Ottenbacher, Msall, Lyon, Duffly, Granger, & Braun, 1999). Many additional studies have confirmed the excellent psychometric properties of the WeeFIM® in children with developmental disabilities (see, Uniform Data Systems for Medical Rehabilitation, 2005 for review); however, there is limited data describing the psychometric properties of the instrument in children with acquired injuries (Wills, Gabbe, Butt, & Cameron, 2006).

Clinical Uses The Functional Independence Measure for Children (WeeFIM®) is frequently used as a quantitative tool in pediatric rehabilitation facilities to measure level of independence in personal care, mobility, and psychosocial competence in many groups of children, including those with developmental disabilities or acquired neurological injury. Facilities subscribing to the WeeFIM® system choose indicators from this instrument along with other relevant markers of performance (i.e., length of stay) to be used for performance evaluation and hospital accreditation purposes. Over 1200 adult and pediatric facilities subscribe to UDSMR across the USA and around the world (Uniform Data System for Medical Rehabilitation, 2004–2006). Each quarter, facilities subscribing to UDSMR receive a report with the number of cases, mean, median, standard deviation, minimum, and maximum values for selected indicators of interest of their facility. Several clinical research studies have also used the WeeFIM® to measure outcome in children with a variety of disabilities (see Uniform Data System for Medical Rehabilitation, 2005 for review). In clinical settings, because the WeeFIM® has a minimal data set, it is often used in conjunction with other measures to obtain more precise measurement of motor, self-care, and cognitive functioning.

Cross References ▶ Functional Assessment ▶ Functional Independence Measure ▶ Interdisciplinary Team Rehabilitation ▶ Outcome, Outcome Measurement ▶ Vineland Adaptive Behavior Scales

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References and Readings Chen, C. C., Bode, R. K., Granger, C. V., & Heinemann, A. W. (2005). Psychometric properties and developmental differences in children’s ADL item hierarchy: A study of the WeeFIM® instrument. American Journal of Physical Medicine and Rehabilitation, 84, 671–680. Granger, C. V., Hamilton, B. B., Keith, R. A., Zielezny, M., & Sherwin, F. S. (1986). Advances in functional assessment for medical rehabilitation. Topics in Geriatric Rehabilitation, 1, 59–74. Msall, M. E., DiGaudio, D., Rogers, B. T., LaForest, S., Catanzaro, N. L., Campbell, J., et al. (1994). The functional independence measure for children (WeeFIM®): Conceptual basis and pilot use in children with developmental disabilities. Clinical Pediatrics, 33, 421–430. Msall, M. E., DiGaudio, K., Duffy, L. C., LaForest, S., Braun, S., & Granger, C. V. (1994). WeeFIM®: Normative sample of an instrument for tracking functional independence in children. Clinical Pediatrics, 33, 431–438. Ottenbacher, K. J., Msall, M. E., Lyon, N., Duffy, L. C., Granger, C. V., & Braun, S. (1999). Measuring developmental and functional status in children with disabilities. Developmental Medicine and Child Neurology, 41, 186–194. Uniform Data System for Medical Rehabilitation (2006). The WeeFIM IIsm system clinical guide: Version 6.0. Buffalo: UDSMR. Uniform Data System for Medical Rehabilitation (2004–2006). WeeFIM II® System. Retrieved December 6, 2008, from http://www.udsmr. org/Documents/WeeFIM/WeeFIM_II_System.pdf Willis, C. D., Gabbe, B. J., Butt, W., & Cameron, P. A. (2006). Assessing outcomes in paediatric trauma populations. Injury: International Journal of the Care of the Injured, 37, 1185–1196.

Functional Magnetic Resonance Imaging G LENN W YLIE Kessler Foundation West Orange, NJ, USA

Synonyms fMRI

Description The goal of functional magnetic resonance imaging is to establish where in the brain neural activity occurs (e.g., Fig. 1). Unlike electroencephalography (EEG) or magnetoencephalography (MEG), which directly record changes associated with neuronal firing, fMRI relies on the metabolic processes that accompany neural activity, and the associated changes in blood flow, to generate spatial maps that show where the neural activity is likely to occur in the

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brain (Fig. 1). Neurons consume oxygen and glucose when they are active, and because neither is stored in the cell, active neurons absorb oxygen and glucose from the surrounding capillary bed. This creates a local decrease in oxygenated blood and a corresponding increase in deoxygenated blood. To compensate this, there is an increase in regional cerebral blood flow (rCBF), which results in a large influx of oxygenated blood – far more than is needed to compensate for the decrease in oxygenated blood caused by neuronal activity. These changes in the relative concentration of oxygenated and deoxygenated blood are important in fMRI because oxygenated and deoxygenated blood have different magnetic properties. Using the blood oxygen leveldependent (BOLD) contrast, an increase in deoxygenated blood results in a decrease in the MR signal, while an increase in oxygenated blood results in an increase in the MR signal. Because neuronal activity results in an

initial increase in deoxygenated blood followed by a very large increase in oxygenated blood, researchers can use the BOLD contrast to infer where neuronal activity is likely to have occurred by looking for changes in the BOLD signal. The majority of the fMRI literature has focused on the increase in the BOLD signal associated with the increase in oxygenated blood because this signal is larger (and therefore has a better signal to noise ratio); however, with higher field magnets (greater than 1.5 T), the initial decrease in the BOLD signal associated with increased deoxygenated blood is beginning to be studied and may be a more precise marker of neural activity. While the spatial precision of fMRI is very good – generally 3–4 mm – the temporal precision of fMRI is relatively coarse. This is because while neural events occur on a timescale of milliseconds, changes in blood flow occur on a timescale of seconds. The initial increase in deoxygenated blood following neuronal activity takes approximately

Functional Magnetic Resonance Imaging. Figure 1 The four panels show the coronal (a), sagittal (b), and axial (c), as well as a three-dimensional rendering (d) of the brain of a single subject, engaged in a cognitively demanding task (working memory). Activity is evident in a broad network of areas, including frontal, parietal, and temporal cortices, as well as deeper structures such as the thalamus


1 s, and the increase in oxygenated blood that follows takes approximately 2–5 s to peak, and far longer (approximately 10 s) to return to baseline. Thus, the hemodynamic response function associated with a single neural event has a timecourse of approximately 15 s, even though the neural event itself might have taken only a fraction of a second.

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visual cortex, motor cortex). Once it was determined that the location of the BOLD signal matched known brain anatomy, researchers immediately began to map the anatomical substrate of a wide spectrum of brain functions and processes. This endeavor has since expanded to include some factors of nearly every aspect of brain function.

History Experimental Designs The fact that neural activity is associated with changes in blood flow has been known since the 1890s (Roy & Sherrington, 1890). One hundred years later, Ogawa et al. (1990) found that the difference in magnetic susceptibility between oxygenated and deoxygenated blood could be used as an endogenous contrast agent: the BOLD signal. The BOLD signal was then validated by mapping activity in anatomically distinct areas of known function (e.g.,

The change in the BOLD signal associated with neural events is very small (generally less than 5%). This means that it is necessary to aggregate together many instances of a given neural event to reliably detect a change in the BOLD signal. There are two broad classes of experimental design that have been developed to achieve this: block design and event-related design (Fig. 2). In a block design experiment

Functional Magnetic Resonance Imaging. Figure 2 Two types of experimental designs. The top panel shows a block, or ‘‘boxcar,’’ design in which an experimental condition is alternately on and off: shown in the red trace. Actual experimental data are shown in black, and it is readily apparent that the data follow the experimental manipulation. The lower panel shows an event-related design, in which each event (e.g., trial) is separately modeled. The red trace again shows the expected response, with each peak representing an event. (The events occur randomly across time, but some occur before the response to the previous event has returned to baseline; thus in some cases, the expected response remains elevated for an extended time.) As with the block design, it is evident that the BOLD response in this voxel largely follows the experimental manipulation

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Functional Mapping

(also known as a boxcar design), a particular experimental condition is presented for an entire block of time (e.g., 30 s). During this time, the subject is continuously engaged in a given experimental condition. Several such blocks are typically performed for each condition (Fig. 2). In an event-related design, the instances of each experimental condition are intermingled (Fig. 2). In this type of design, it is necessary to include numerous (usually more than 20) instances of each condition and to ensure that the individual occurrences of these ‘‘events’’ are randomly distributed across time.

Clinical Applications While fMRI is predominantly used as a research tool, clinical applications for fMRI are being developed. For example, there is some evidence that the BOLD response recorded when the brain is at rest may serve as a biomarker for incipient Alzheimer’s disease (e.g., Greicius et al., 2004). Also, there is growing interest in using fMRI as an alternative to the intracarotid amobarbital procedure or Wada test for determining language lateralization prior to brain surgery (e.g., in epilepsy: Abou-Khalil, 2007). While such applications are currently in their early stages, they are being actively developed and will almost certainly be applied in the future. In an experimental setting, fMRI has been used extensively to investigate basic questions regarding brain function. It is also being used to better understand the deficits in clinical conditions such as multiple sclerosis, traumatic brain injury, chronic fatigue syndrome, stroke, and many others. One approach is to perform a given experiment (e.g., one investigating working memory) in both a healthy cohort and a clinical cohort with the goal of comparing the pattern of BOLD signal between the two groups. If a given area responds differently in the clinical population, then one has some grounds for assuming that the disease affects that brain area and the cognitive functions that rely on that area. However, such a conclusion assumes that the metabolism of the neurons is comparable between the clinical population and the healthy population and that the ability for the brain to increase rCBF in active areas is also comparable. These assumptions have not yet been validated, and therefore the results of these experiments must be interpreted with care. Experimental approaches that use within-subject designs are less problematic. In these paradigms, the BOLD response during an experimental condition is compared to a baseline condition and then this difference

in BOLD signal is compared across groups. Such designs are less problematic because global differences in metabolism and rCBF should be represented in the baseline condition, and will thus be removed in the first comparison (when the experimental condition is compared to the baseline condition). There are numerous variants of within-subject designs (e.g., pre- and post-intervention designs, regression analyses).

References and Readings Abou-Khalil, B. (2007). An update on determination of language dominance in screening for epilepsy surgery: The Wada test and newer noninvasive alternatives. Epilepsia, 48(3), 442–455. Frackowiak, R. S. J., Friston, K. J., Frith, C., Dolan, R., & Mazziotta, J. C. (1997). Human brain function. USA: Academic. Gazzaniga, M. S., Ivry, R. B., & Mangun, G. R. (2009). Cognitive neuroscience: The biology of the mind (3rd ed.) New York: WW Norton. Greicius, M. D., Srivastava, G., et al. (2004). Default-mode network activity distinguishes Alzheimer’s disease from healthy aging: Evidence from functional MRI. Proceedings of the National Academy of Sciences of the United States of America, 101(13), 4637–4642. Huettel, S. A., Song, A. W., & McCarthy, G. (2004). Functional magnetic resonance imaging. New York: Sinnauer. Jezzard, P., Matthews, P. M., & Smith, S. M. (2001). Functional MRI: An introduction to methods. Oxford: Oxford University Press. Ogawa, S., Lee, T. M., et al. (1990). Brain magnetic resonance imaging with contrast dependent on blood oxygenation. Proceedings of the National Academy of Sciences of the United States of America, 87(24), 9868–9872. Roy, C. S., & Sherrington, C. S. (1890). On the regulation of the bloodsupply of the brain. The Journal of Physiology, 11(1–2), 85–158.

Functional Mapping ▶ Cortical Mapping

Functional Neuroanatomy J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition A description of the central and peripheral nervous system which focuses on behavioral correlates

Functional Status

associated with specific neuronal structures and/or their interconnections.

Current Knowledge The study of functional neuroanatomy typically involves several different but related approaches. One is to simply describe a neuroanatomical structure, either on a macro or more micro basis, and define what are thought to be the functions normally mediated by that structure. An example of the former might be to list the behaviors believed to be carried out by the right versus the left cerebral hemisphere, whereas the latter might focus on a particular gyrus within the hemisphere, or even on a select group of neurons contained within that gyrus. A second approach, which might be referred to as a ‘‘systems’’ approach, is to identify a particular behavior and then attempt to define the nerve centers and pathways thought to be instrumental in its expression. For example, identifying the essential neural substrates responsible for vision or comprehension of spoken language. A third approach is to study the effects of focal lesions on behavior. Thus, if a circumscribed lesion is known to reliably result in a given behavioral disturbance, it might be reasonable to presume that this portion of the nervous system is crucial for that behavior. Another technique involves the recording of activity in different parts of the nervous system during the course of ongoing behavior. This might be done through macro (e.g., electroencephalograms) or micro (e.g., wire implants) recordings of electrical impulses or investigating changes in glucose metabolism or other activity using imaging studies such as photon emission tomography or functional magnetic resonance imagery. Studies of the biochemical and hormonal patterns and changes associated with particular regions of the nervous system and their behavioral influences represent yet another way of defining the neuroanatomical correlates of behavior. In attempting to describe functional neuroanatomy, data are usually combined from several of these investigative methods.

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References and Readings Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sunderland, MA: Sinauer. Haines, D. E. (Ed.). (1997). Fundamental neuroscience. New York: Churchill Livingstone. Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy – A neurobehavioral approach. New York: Springer.

Functional Neuroimaging ▶ Functional Imaging

Functional Organization ▶ Hemispheric Specialization

Functional Reorganization ▶ Brain Plasticity

Functional Somatic Syndromes ▶ Unexplained Illness

Functional Status TAMARA B USHNIK NYU Langone Medical Center New York, NY, USA

Definition Cross References ▶ Electroencephalography ▶ Functional Magnetic Resonance Imaging ▶ Hemispheric Specialization ▶ Neuroimaging ▶ PET ▶ Single Photon Emitted Computed Tomography

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The ability to perform activities of daily living (ADLs) that are necessary to meet basic needs, fulfil social and community roles, and maintain health and well-being. These activities include, but are not limited to, walking, getting out of bed, cooking, bathing, and dressing. Functional status may be influenced by physical impairment, symptoms, cognitive abilities (or deficits), moods, and health perceptions.

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Functional Status Questionnaire

Cross References

Current Knowledge

▶ Activities of Daily Living (ADLs)

The FSQ can be used both as an initial screen and to monitor the patient over time. It has been used in a variety of other settings, including assessment of the impact of variations in hospital practice patterns, the study of relations between hospital processes of care and outcomes, and other applications, including randomized controlled trials of pharmaceuticals (Cleary & Jette, 2000). The mental health subscale of the FSQ has been adopted within the Short-Form 36 (SF-36).

References and Readings American Thoracic Society Quality of Life Resource. Functional Status. (2007). Retrieved November 10, 2009, from http://www.atsqol.org/ sections/key-concepts/functional-status.html

Administration

Functional Status Questionnaire S UE A NN S ISTO Stony Brook University, School of Health Technology and Management Stony Brook, NY, USA

Synonyms FSQ

Description The Functional Status Questionnaire (FSQ) (Jette, 1980) can be used as a self-administered functional assessment for primary care patients that provides information on physical, psychological, and social and role functions. There are five sections of the FSQ: (1) physical function in the activities of daily living (ADLs), (2) mental health function, (3) social role function, (4) social activity, and (5) other performance measures. The physical function section consists of measures of basic (3 items) and intermediate ADLs (6 items); mental health function includes measures of feelings of anxiousness and downheartedness (5 items); social and role functions include three groups of items including a six-item measure of work performance (for those employed during the previous month), a three-item measure of social function, and a five-item measure of social interaction. Single questions assess work status, bed disability days, days on which the respondent reduced usual activities, satisfaction with sexual relationships, frequency of social interactions, and an overall rating of health status. Low scores of 0–69 to 87, depending on the subscale, indicate areas that need to be addressed for improvement.

The FSQ takes approximately 10 min to administer and a computer expedites the scoring routines. The scoring of the FSQ is described by McDowell and Newell (1996). A transformed scale score is produced by summing the response scores for each grouping, then dividing the number of questions with valid information 1 then multiplying by 100. That score is then divided by the maximum–the minimum response scores.

Reliability and Validity Jette and Deniston (1978) first established the interobserver reliability of the FSQ. Subsequently, the FSQ reliability has been determined to be high when administered in a wide range of settings and patient populations if the instrument covers the substantive domains of interest. In particular, data from heart attack studies confirm the construct validity of selected FSQ scales in older women. The FSQ is a reliable and valid instrument for the assessment of a broad range of dimensions of health-related quality of life (HRQL) in patients who have received either outpatient or inpatient care and patients in community settings (Cleary & Jette, 2000).

Clinical Utility The FSQ has clinical utility for any person with a chronic disabling condition such as those with musculoskeletal, orthopedic, and rheumatological conditions.

Cross References ▶ Direct Assessment of Functional Status ▶ Functional Assessment ▶ Functional Assessment Measure

Furman v. Georgia (1972)

▶ Functional Independence Measure ▶ Functional Status ▶ Older Americans Resources and Services Multidimensional Functional Assessment Questionnaire ▶ Physical Functional Performance

References and Readings Cleary, P. D., & Jette, A. M. (2000). Reliability and validity of the functional status questionnaire. Quality of Life Research, 9, 747–753. Jette, A., & Deniston, O. L. (1978). Inter-observer reliability of the Functional Status Assessment Instrument. Journal of Chronic Diseases, 31, 573–589. Jette, A. (1980). Functional capacity evaluation: An empirical approach. Archives of Physical /Medicine and Rehabilitation, 61, 85–89. Jette, A. M., & Davies, A. R. (1986). The functional status questionnaire: Reliability and validity when used in primary care. Journal of General Internal Medicine, 1, 143–149; Journal of General Internal Medicine [Erratum], 1, 427. McDowell, I., & Newell, C. (1996). Measuring health: A guide to rating scales and questionnaires (2nd ed.). New York: Oxford University Press, ISBN 0-19-510371-8.

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William Henry Furman was robbing a person’s house and was interrupted when the homeowner arrived. Furman reportedly tried to escape, and while doing so, tripped, which caused the weapon he was carrying to fire killing the homeowner. Furman was tried for murder, found guilty (primarily as a result of his own testimony), and sentenced to death. The US Supreme Court was split five to four in rejecting the imposition of the death penalty. A result could not be agreed upon, but instead the US Supreme Court ruled that utilization of the death penalty in the USA represents ‘‘cruel and unusual’’ punishment and it violates the Eighth Amendment because it is morally unacceptable to the people of the USA ‘‘. . .at this time in their history.’’ Seven of the justices did not object to the death penalty itself as unconstitutional, but rather, indicated their disapproval of the lack of specific guidelines for indicating when a judge or jury should impose the death penalty and because of the arbitrary and inconsistent manner in which capital punishment was imposed. As a result of this decision, death penalty statutes were rewritten by 35 states to address the problems pointed out in Furman v. Georgia.

Functional Stress Syndromes ▶ Unexplained Illness

Furman v. Georgia (1972) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition This is the first US Supreme Court case to hold that capital punishment was a violation of the cruel and unusual punishments clause and therefore unconstitutional.

Cross References ▶ Capital Punishment ▶ Death Penalty

References and Readings Cunningham, M. D., & Goldstein, A. M. (2003). Sentencing determinations in death penalty cases. In A. Goldstein (Ed.), Handbook of psychology (Vol. 11). Forensic psychology. New Jersey: Wiley. Furman v. Georgia, 408 U.S. 238 (1972). Heilbronner, R. L., & Waller, D. (2008). Neuropsychological consultation in the sentencing phase of capital cases. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press.

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G GABA J OSE A. R EY Nova Southeastern University Ft. Lauderdale, FL, USA

Indications Gamma(g)-aminobutyric acid (GABA) is considered to be the major inhibitory neurotransmitter in the central nervous system (CNS). It is the product of decarboxylation, via L-glutamic acid decarboxylase (GAD), of glutamate and is thus an amino acid-derived neurotransmitter. This neurotransmitter and its respective receptors, the ionotropic GABAA receptor and the metabotropic GABAB receptor, are found throughout the CNS and are targets of drug action in an attempt to affect GABA or its receptors to cause anxiolytic, hypnotic, anesthetic, anticonvulsant, or muscle relaxant effects. Interestingly, GABA is derived from the same source as the brain’s most common excitatory neurotransmitter, glutamate, and often acts in opposition to glutamate to achieve a balance between excitation and inhibition at the synaptic level. The role of GABA is to bind to, and activate, a ligand-gated chloride ionophore channel complex to increase the entry of chloride ions into a neuron and cause hyperpolarization leading to inhibitory postsynaptic potentials (IPSPs). This ligand-gated binding occurs most often at the pentameric GABAA receptor subtype and its variants and also at lower levels of the GABAB receptor subtype, which is a G-protein-coupled metabotropic receptor affecting potassium conductance and is a target for the drug baclofen used as a muscle relaxant. Inactivation of GABA at the synaptic level after vesicle release is primarily via presynaptic uptake by transporter proteins for GABA, which is inhibited by the anticonvulsant tiagabine.

Mechanisms of Action GABA transaminase (GABA-T) is the enzyme for metabolism and breakdown of GABA, which is inhibited

by the anticonvulsant vigabatrin. A target for the ubiquitous benzodiazepines (BZD) (e.g., ▶ diazepam) and newer non-benzodiazepine hypnotics (e.g., ▶ zolpidem) is the GABA-chloride ionophore complex. This receptor complex also contains separate binding sites for alcohol, barbiturates, and other exogenous compounds. Some agents can open this chloride channel independently of GABA’s presence in the synaptic cleft and making them dangerous in overdose scenarios. This is different than the mechanism of action that BZDs have as positive allosteric modulators of GABA to increase the binding affinity and ability for GABA to activate the receptor resulting in increased rate of chloride channel opening and subsequent hyperpolarization of the postsynaptic neuron. Being allosteric modulators, benzodiazepines, or agents such as zolpidem, do not have the ability to activate the receptor complex as agonists independent of GABA. This mechanism of action contributes to the BZDs being considered as safer in overdose scenarios compared to agents such as the barbiturates or alcohol.

Specific Compounds and Properties The GABAB receptors belong to the superfamily of G-protein-coupled receptors and are considered metabotropic receptors.

Clinical Use (including Side Effects) The principle roles of the GABAB receptor are to mediate slow inhibitory postsynaptic potentials and attenuate the release of multiple CNS active neurotransmitters possibly via interactions with autoreceptors. The GABAB receptor is coupled to calcium and potassium via second-messenger systems to affect cellular membrane conductance. Given the major role that GABA serves in the CNS, this neurotransmitter will continue to be a popular target of drug development.


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Gabapentin


Off Label Use

Brunton, L. L., Lazo, J. S., & Parker, K. L. (Eds.). (2006). Goodman & Gilman’s the pharmacological basis of therapeutics (11th ed.). New York: McGraw-Hill. Cooper, J. R., Bloom, F. E., & Roth, R. H. (Eds.). (2003). The biochemical basis of neuropharmacology (8th ed.). New York: Oxford University Press. Davis, K. L., Charney, D., Coyle, J. T., & Nermeroff, C. (Eds.). (2002). Neuropsychopharmacology: The fifth generation of progress. Philadelphia, PA: Lippincott Williams & Wilkins. Stahl, S. M. (2008). Stahl’s essential psychopharmacology: Neuroscientific basis and practical applications (3rd ed.). New York: Cambridge University Press. Voet, D., Voet, J. G., & Pratt, C. W. (Eds.). (2008). Fundamentals of biochemistry (3rd ed.). New Jersey: Wiley.

Neuropathic pain, chronic pain, anxiety, and bipolar disorder.

Side Effects Serious Sudden unexplained deaths associated with epilepsy.

Common Fatigue, nystagmus, tremor, ataxia, dizziness, sedation, blurred vision, vomiting, diarrhea, constipation, and weight gain.

Gabapentin References and Readings J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metarie, LA, USA


Additional Information Generic Name Gabapentin

Brand Name


Neurontin

Gage, Phineas (1823–1860) Class Anticonvulsant, antineuralgic

Proposed Mechanism(s) of Action Binds to voltage-sensitive calcium channels via alpha-2 delta subunit, transported across blood-brain barrier and from the gut to the blood via L transport system.

Indication Partial seizures, postherpetic neuralgia.

M ICHELLE A NN P ROSJE University of Florida Gainesville, FL, USA


Phineas P. Gage is undoubtedly one of the most renowned patients to have survived severe brain damage (Macmillan, 2000). Gage holds a prominent place at the cornerstone of neurological history and is ‘‘a fixture in neurological textbooks’’ (Larner & Leach, 2002). Macmillan (2000, 2002) further described

Gage, Phineas (1823–1860)

Gage as the first reported case to elucidate the relationship between frontal lobe function and personality. Debate regarding the extent of damage caused by Gage’s penetrating injury has ensued due in part to the dearth and unreliability of evidence available from the time of injury and gleaned from postmortem studies. Although there is a lack of consensus in the field, various researchers have theorized about the location and extent of Gage’s brain damage. To gain an appreciation for Gage’s prominence in the early days of neurology, a review of the details surrounding the horrific accident and subsequent sequelae is warranted. However, caution must be exercised in such review due to the inconsistencies of reports and the apparent deviation from the original accounts of Dr. John Martyn Harlow, the physician who treated Gage immediately after and in the years following his injury (Macmillan, 2000, 2002). Reports indicate that the accident occurred at 4:30 p.m. on September 13, 1848 (Macmillan, 2000). At that time, Gage was 25 years-old, approximately 150 lbs, and 5 ft. 6 in. tall. Gage was employed as a foreman in charge of a crew building the Rutland and Burlington Railroad in Cavendish, Vermont. As part of his daily responsibilities, Gage performed manual labor using a tamping iron, which closely resembled a crowbar. Gage’s tamping iron was extraordinary in that it was longer, heavier, and more tapered than those used by his crew. Macmillan reported that Gage’s tamping iron was 3 ft. 7 in. long, weighed 13.25 lbs, and tapered from a wide end of 1.25 in. to a narrow end of 0.25 in. In the mid-nineteenth century, tamping was a conventional technique used to obtain the greatest efficiency from a charge while blasting rocks for laying a railroad foundation. The process of tamping involved creating a hole, pouring sand into the hole, and subsequently pounding the tamping iron into the hole. Once the tamping iron was pounded into the hole, it packed a charge, generated a spark, and consequently ignited an explosive charge. Historical accounts indicate that Gage mistakenly dropped the tamping iron into the hole before one of his crewmen had poured the sand. Thus, as soon as the tamping iron hit the rock, a charge was ignited, and the tamping iron instantaneously reversed direction and shot upward. The tamping iron reportedly struck Gage under his left zygomatic arch, passed completely through his brain, emerged out of the top of his head left of the midline, and landed 25–30 yards behind him (Macmillan, 2000). Gage immediately was catapulted backward from the blast. His limbs started convulsing upon

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landing. It is unclear whether or not Gage lost consciousness postaccident. Some reports indicate that he spoke to his crewmen within a few minutes of the explosion and ambulated independently to the oxcart which transported him to town. Other sources relay that Gage required assistance from his crewmen to stand and maneuver himself to the oxcart. The details surrounding the accident are likely unreliable because they consisted predominately of Gage’s self-report which was documented in Dr. Harlow’s notes. Approximately 30 min after the accident, Edward Higginson Williams, M.D. visited Gage at his residence. John Martyn Harlow, M.D. arrived approximately 1½ hours post-explosion and assisted Williams in cleaning and dressing Gage’s wounds. Upon physical examination of the tamping iron’s exit site, fragments of bone had been uplifted and brain was protruding. Harlow (1848) noted that immediately post-explosion Gage was ‘‘perfectly conscious,’’ and his mind was ‘‘clear’’ with no impairment of ‘‘sensorial powers.’’ Surprisingly, Gage was able to describe precisely every detail of the incident from the time of the explosion to the first physician’s arrival. Gage asserted that he would return to work within the few days post-injury. Sources also state that Gage recognized his mother and uncle during their visit the morning after the accident. Harlow noted that Gage vomited in the immediate aftermath of the accident. Over the following days and weeks, Harlow documented the physical changes in Gage: approximately 2 days post-accident Gage’s hemorrhage ceased, but an inflammatory reaction/infection emerged and persisted for about 3 weeks. During his examination 3 days after the explosion, Harlow noted that Gage appeared restless and delirious. His speech was described as hyperverbal but incoherent and grossly disconnected. Gage’s level of alertness fluctuated from lucidity to complete delirium, likely a result of the infection. Harlow documented that within 7 days of the accident Gage’s temperament had changed: Gage was very restless during his sleep, his hands and feet flailed constantly, and he attempted to get out of bed even though he was physically unable to raise his head off the pillow. A substantial discrepancy between Gage’s physical abilities and his mental desires emerged. He also remarked that it was impossible for Gage to recover and that Gage ‘‘shall not live long. . .’’ Harlow noted that from September 23rd until October 3rd Gage remained in a ‘‘semi-comatose state,’’ seldom spoke unless spoken to, and communicated in monosyllabic utterances. Approximately 1 month after

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the explosion, Gage’s cognitive abilities were grossly intact insofar as he had full memory of the event and immediate sequelae, remained oriented to day and time, and knew at least 50% of the people who visited him. Two months post-explosion, Harlow noted that Gage had become more impulsive and disinhibited, and his decision-making and problem-solving skills became impaired. His friends reportedly described him as ‘‘uncontrollable.’’ During one of his visits, Harlow observed Gage independently maneuvering through the house and showing signs of physical and mental improvement. Gage died 11½ years post-injury. A few years after his death, Harlow requested access to Gage’s skull, gained further information from Gage’s social connections, and elaborated on his description of Gage’s personality functioning both pre- and postaccident. He described Gage premorbidly as a hard-working, popular, and responsible young man; however, postaccident Gage was

" fitful, irreverent, indulging at times in the grossest profan-

ity (which was not previously his custom), manifesting but little deference for his fellows, impatient of restraint or advice when it conflicts with his desires, at times pertinaciously obstinate, yet capricious and vacillating, devising many plans of future operation, which are no sooner arranged than they are abandoned in turn for others appearing more feasible (Harlow, 1868).

Harlow elaborated that Gage’s friends and acquaintances remarked that he was ‘‘no longer Gage’’ (1868). Documentation describing Gage in terms of personality and behavior both pre- and postaccident is limited and, as such, open to discussion. Damasio (1994) conducted further investigation into the nature of Gage’s injury and asserted that the dramatic changes in Gage’s personality were in direct contrast to his intact cognition (e.g., ▶ attention, ▶ perception, ▶ language, ▶ intelligence, and ▶ memory). Damasio acknowledged that the most striking result of the accident was Gage’s survival. Historically, Phineas Gage has become a classical example for prefrontal lobe pathology and disturbances in executive functions (Harlow, 1868). Since the mid-nineteenth century, the case of Phineas Gage has garnered much attention in the literature, prompted considerable speculation about frontal lobe function, and provided some confirmation for the localization theories. Yet, to this day the exact amount of brain damage caused by the entrance and exit of Gage’s tamping iron is still speculative. No

autopsy was ever performed; only the skull has been examined. Various researchers continue to cogitate on the impact such penetrating injury had on various vulnerable brain regions. In 1848, Harlow believed that the brain damage was confined primarily to the anterior left lobe with accompanying diffuse damage. However, in 1868 after examination of Gage’s skull, Harlow noted that the tamping iron likely entered the left hemisphere at the Sylvian fissure, possibly punctured the cornu of the left lateral ventricle, and produced serious brain lesions as it passed through the brain and exited the skull. Macmillan (2000) provided a review of estimated damage to Gage’s brain: left frontal (Harlow, 1848); left and right prefrontal (Cobb, 1940, 1943); left anterior frontal, tip of left temporal, anterior horn of left lateral ventricle, head of caudate nucleus and putamen, right hemisphere, including right superior and cingulated gyri (Tyler & Tyler, 1982); and, anterior half of left orbital frontal cortex, polar and anterior mesial frontal cortices, anterior-most part of anterior cingulate gyrus with similar areas of damage in the right hemisphere but less marked damage in the orbital frontal region (Damasio, Grabowski, Frank, Galaburda, & Damasio, 1994). Wagar and Thagard (2004) designated Gage’s damage as the first recorded incident of damage to the ventromedial prefrontal cortex (VMPFC). Overall, it is difficult to clearly ascertain the amount of damage Gage’s brain sustained in the accident. A myriad of factors contribute to this challenge: profuse hemorrhage, overflow of brain tissue pressing out of the skull, bone fragments infiltrating brain tissue, possible harm from a concussion, and further loss of brain tissue as a result of the infection. Ferrier (1878), an early proponent of cerebral localization theory, cited Gage as a primary example of how frontal lobe injury can result in personality changes undetectable on the sensory and motor exam. Larner and Leach (2002) also asserted that Gage’s case demonstrated how particular personality and behavioral changes can be correlated with injury to focal brain regions (e.g., association between function and location).

Short Biography Born on July 9, 1823, Phineas P. Gage was the son of Jesse Eaton Gage and Hannah Truselle Swetland. Although Phineas Gage was born the first of five children, limited information is available regarding his birth and early childhood. Macmillan (2002) purported that Gage likely


grew up in East Lebanon, Vermont on a family member’s farm. In terms of education, Harlow (1868) described Gage as ‘‘untrained in schools’’ because of a lack of school documentation. However, Macmillan (2000) reviewed U.S. Census data from 1840 and 1850 and found high levels of school attendance and high rates of literacy among Caucasian adults (e.g., 99.1 and 99% for 1840 and 1850, respectively). Thus, he deduced that Gage likely attended school and was literate. Gage was employed by a firm of contractors who won the tender for preparing the foundation for the railway near Cavendish. Macmillan (2000) noted that Gage’s contractors and crewmen viewed him as ‘‘well-equipped physically and psychologically for railroad construction work.’’ Harlow (1848) noted that Gage was not only a favorite among his men but also the epitome of capability and efficiency. Harlow (1848) elaborated that Gage’s physical condition was flawless in that he was healthy, strong, and active both before and after the accident. Psychologically, Gage was described as having boundless energy and temperate habits pre-accident (Harlow, 1848). According to Macmillan (2008), Gage desired to return to work as a foreman after his physical recovery from the accident. Unfortunately he never returned to work on the railroad. In 1850, Gage caught the attention of P.T. Barnum. Barnum hired Gage as an attraction at Barnum’s American Museum in New York, was on display, and visited New England cities to lecture and exhibit himself (Macmillan, 2008). Neylan (1999) believed Barnum hired Gage because he was an anomaly in that he walked immediately following the accident, communicated sensibly, and remained lucid for the majority of the aftermath. Gage was also employed for some 18 months for Jonathan Currier who ran a livery stable and coach-line service from his Dartmouth Inn, in Hanover, NH. Gage also went to Chile with a man who planned to set up a stagecoach line (Harlow, 1868). After years driving stagecoaches, he returned to his family in San Francisco in 1859. Accounts indicate that he arrived in California in a weakened condition due to some illness he had contracted. Following his recovery from the illness, he obtained work of ploughing farms. However, one day he started having seizures which lead to status epilepticus and eventual death. The date of his death is uncertain. His mother stated he died in 1861, but Macmillan’s (2000) review of the funeral director and cemetery records indicate that Gage died on May 21, 1860. He had survived for 11½ years after his horrendous accident (Harlow, 1868; Macmillan, 2000). Gage was subsequently buried with his tamping iron which had become a constant companion and reminder for him. In 1866, Dr. Harlow obtained consent from Gage’s family to

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exhume his body and retrieve the skull and tamping iron. The skull and tamping iron are now on display at the Warren Anatomic Museum at Harvard University.

Cross References ▶ Damasio, Antonio R. (1944– ) ▶ Disinhibition ▶ Frontal Lobe ▶ Localization ▶ Severe Brain Injury ▶ Traumatic Brain Injury

References and Reading Barker, F. G. (1995). Phineas among the phrenologists: The American crowbar case and nineteenth century theories of cerebral localization. Journal of Neurosurgery, 82, 672–682. Bigelow, H. J. (1850). Dr. Harlow’s case of recovery from the passage of an iron bar through the head. American Journal of the Medical Sciences, 19, 13–22. Cobb, S. (1940). Review of neuropsychiatry for 1940. Archives of Internal Medicine, 66, 1341-1354. Cobb, S. (1943). Borderlands of psychiatry. Cambridge, MA: Harvard University Press. Damasio, A. R. (1994). Descartes error: Emotion, reason and the human brain. New York: G. P. Putnam’s Sons. Damasio, H., Grabowski, T., Frank, R., Galaburda, A. M., & Damasio, A. R. (1994). The return of Phineas Gage: Clues about the brain from the skull of a famous patient. Science, 264, 1102–1105. Ferrier, D. (1878). The Goulstonian lectures on the localization of cerebral diseases. British Medical Journal, 1, 443–447. Harlow, J. M. (1848). Passage of an iron rod through the head. Boston Medical and Surgical Journal, 39, 389–393. Harlow, J. M. (1868). Recovery from the passage of an iron bar through the head. Publications of the Massachusetts Medical Society, 2, 327–347. Larner, A. J., & Leach, J. P. (2002). Phineas Gage and the beginnings of neuropsychology. Advances in Clinical Neuroscience and Rehabilitation, 2(3), 26. Macmillan, M. (1986). A wonderful journey through skull and brains: The travels of Mr. Gage’s tamping iron. Brain and Cognition, 5, 67–107. Macmillan, M. (2000). Restoring Phineas Gage: A 150th retrospective. Journal of the History of the Neurosciences, 9(1), 42–62. Macmillan, M. (2002). An odd kind of fame: Stories of Phineas Gage. Cambridge, MA: MIT Press. Macmillan, M. (2008). Phineas Gage-unravelling the myth. Psychologist, 21(9), 828–831. Neylan, T. C. (1999). Frontal lobe function: Mr. Phineas Gage’s famous injury. Journal of Neuropsychiatry and Clinical Neurosciences, 11(2), 280–283. Tyler, K. L. and Tyler, H. R. (1982). A ‘‘Yankee Invasion’’: The celebrated American crowbar case. Neurology, 32, A191. Wagar, B. M., & Thagard, P. (2004). Spiking Phineas Gage: A neurocomputational theory of cognitive-affective integration in decision making. Psychological Review, 111(1), 67–79.

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GAI ▶ Intelligence

Categorization Movement disorder

Epidemiology

Gait Apraxia

Gait disorders are very common and increase in frequency with age.

▶ Apraxia

Natural History

Gait Disorders A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Gait disorders due to aging and neurodegenerative processes tend to worsen with time. Disorders related to static events may show significant improvement with physical therapy and balance training.

Neuropsychology and Psychology

Synonyms

Gait disorders associated with neurodegeneration and medication toxicity may be associated with neuropsychological changes.

Abnormal walking; Walking problems

Evaluation

Definition Gait disorders may be divided into structural and neurological problems. Some patients have both. Structural problems include arthritis and other degenerative joint diseases, muscle deconditioning, acquired impairments following knee or hip surgeries, back or spine problems, and other musculoskeletal disorders. These problems may lead to pain, stiffness, weakness, and numbness, which can all contribute to an abnormal walk. Obesity often makes structural problems worse. Neurological problems include Parkinson’s or other neurological diseases, impairments following stroke or other cerebrovascular incidents, and vision or inner ear problems that affect balance. Diabetes, which can lead to nerve damage, can cause sensory problems that contribute to gait disorders, while dementia and fear of falling can also be factors. Certain medications have been linked to disordered gait and falls. The chief culprits are psychotropic medications, such as neuroleptics, benzodiazepines, and antidepressants. Diuretics, which can affect blood pressure, and steroids, which can weaken muscles if taken over a long period of time, can also cause unsteadiness or dizziness. Drug interactions and high dosages can also affect gait.

Evaluation should include a detailed neurological examination to look for causes of the gait dysfunction. Imaging studies of the brain or spine, nerve conduction studies, and blood work may be necessary to define the etiology of the gait disorder. Treatment depends on the underlying cause or causes.

Cross References ▶ Huntington’s Disease ▶ Parkinson’s Disease

References and Readings Sudarsky, L. (2004). Gait disorders. In R. L. Watts & W. C. Koller (Eds.), Movement disorders (2nd ed., pp. 813–824). New York: McGraw-Hill.

Gait Imbalance ▶ Retropulsion

Galveston Orientation and Amnesia Test

Galantamine J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Galantamine

Brand Name Nivalin, Razadyne, Razadyne ER, and Reminyl

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Additional Information Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.com: 8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Class Cholinesterase inhibitor and an allosteric nicotinic/ cholinergic modulator

Proposed Mechanism(s) of Action Reversibly and competitively inhibits acetylcholinesterase, thus increasing the bioavailability of acetylcholine.

Indication

Galveston Orientation and Amnesia Test DANIEL N. A LLEN University of Nevada Las Vegas Las Vegas, NV, USA

Synonyms GOAT

Alzheimer’s disease.

Off Label Use

Description

Memory disturbances and mild cognitive impairment.

The Galveston Orientation and Amnesia Test (GOAT) is a 10-item scale that was designed to assess orientation and posttraumatic amnesia following traumatic brain injury (Levin, O’Donnell, & Grossman, 1975; Levin, O’Donnell, & Grossman, 1979; Levin, Benton, & Grossman, 1982). Posttraumatic amnesia, which is the state of disorientation and confusion following head injury, includes both anterograde and retrograde amnesia. The GOAT provides an assessment of anterograde amnesia, or the memory of events that occurred immediately before brain injury, by asking patients to recall the last event they can remember immediately before the injury, as well as details regarding the event. Similarly, asking patients to recall and describe in detail the first event they can remember following injury assesses retrograde amnesia, or

Side Effects Serious Seizure and syncope (both rare).

Common Nausea, diarrhea, vomiting, anorexia, increased gastric secretion, weight loss, headache, dizziness, fatigue, and depression.

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Gamma Knife

memory of events immediately following traumatic brain injury. Orientation items include assessment of biographic information (name, city of birth, city of current residence), place (present location, date of admission, mode of transportation to treatment facility), and time (current time and date). A total error score is derived for the GOAT, which is dependent on the extent to which the patient accurately responds to the particular item and the weighting of the items. For example, 30 possible points are awarded for the orientation item ‘‘What is the current year?’’ with 10 error points assigned for each year error to a maximum of 30 points. Consequently, the total error score can range from 0 to 108, which is then subtracted from 100 to yield total GOAT scores ranging from 8 to 100.

Current Knowledge The GOAT is widely used in the assessment of posttraumatic amnesia because of a number of positive characteristics. First, the GOAT takes little time to administer and can be administered and scored in a reliable manner with only minimal training. Administration and scoring are further facilitated by test materials and administration protocols provided by the author (Levin et al., 1979, 1982). Additionally, the GOAT can be administered as a bedside evaluation in both acute and longterm settings to characterize the extent and severity of posttraumatic amnesia as well as to document the return to normal levels of consciousness and improvement in posttraumatic amnesia as the patient recovers. Finally, numerous studies have demonstrated its reliability and validity. Interrater reliability is excellent (r = 0.99). With regard to validity, GOAT scores are associated with the severity of traumatic brain injury and also predict long-term recovery. GOAT scores are also significantly correlated with other measures of severity of traumatic brain injury, including the Glasgow Coma Scale and Orientation-log, neuropsychological measures of memory, and neuropathology. Thus, the GOAT is a highly practical, reliable, and valid method to characterize and monitor the recovery from amnesia following traumatic brain injury.

Cross References ▶ Glasgow Coma Scale ▶ Orientation Log

▶ Post-Traumatic Confusional State ▶ Traumatic Brain Injury

References and Readings Bode, R. K., Heinemann, A. W., & Semik, P. (2000). Measurement properties of the Galveston Orientation Amnesia Test (GOAT) and improvement patterns during rehabilitation. Journal of Head Trauma Rehabilitation, 15, 637–655. Goldstein, F. C., & Levin, H. S. (1995). Post-traumatic and anterograde amnesia following closed head injury. In A. D. Baddeley, B. A. Wilson, & F. N. Watts (Eds.), Handbook of memory disorders (pp. 187–209). Oxford/England: Wiley. Levin, H. S., O’Donnell, V. M., & Grossman, R. G. (1975). The Galveston Orientation and Amnesia Test: A practical scale to assess cognition after head injury. Journal of Nervous and Mental Diseases, 167, 675–684. Levin, H. S., O’Donnell, V. M., & Grossman, R. G. (1979). The Galveston Orientation and Amnesia Test: A practical scale to assess cognition after head injury. Journal of Nervous Mental Disease, 167, 675–684. Levin, H. S., Benton, A. L., & Grossman, R. G. (1982). Neurobehavioral Consequences of Traumatic Brain Injury. New York: Oxford University Press.

Gamma Knife S USAN L ADLEY-O’B RIEN Denver Health Medical Center Denver, CO, USA

Definition Gamma knife is one of the most widely used stereotactic radiosurgery devices. A concentrated dose of photon radiation is used to deliver the effect. Indications for gamma knife stereotactic radiosurgery include metastatic tumors, malignant gliomas, benign brain tumors, acoustic neuromas, arteriovenous malformations, and trigeminal neuralgia.

Current Knowledge This procedure has the greatest impact on survival in patients with single brain metastases, but it is also suitable for patients with multiple lesions and with recurrence in the brain at distant sites. Approximately, 95% of benign tumors are controlled with this procedure.

Ganglioglioma

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Patients with trigeminal neuralgia are often elderly and at high risk for conventional surgery. There is ample clinical evidence to support the use of gamma knife in this condition. More than 80% of patients have significant improvement in their pain following the procedure, but as many as 40% recur within 5 years. The primary risk of gamma knife radiosurgery is radiation necrosis, which occurs 6–24 months after treatment and is related to the dose delivered and the volume treated. Procedure related mortality itself is quite low.

G References and Readings Barker, F. G. (2005). Surgical and radiosurgical management of brain metastases. The Surgical Clinics of North America, 85, 329. Chin, L. S., et al. (2004). Neurosurgery. In C. M. Townsend, (Ed.), Sabiston textbook of surgery (17th ed., p. 239). Philadelphia, PA: Saunders. El Hamri, A. K., Monk, J., & Plowman, P. N. (2005). Stereotactic radiosurgery at St. Bartholomew’s hospital: Third quinquennial review. British Journal of Radiology, 78, 384–393.

Gamma-Aminobutyric Acid ▶ GABA ▶ Neurotransmitters

Ganglioglioma J ENNIFER T INKER Drexel University Philadelphia, PA, USA

Definition Gangliogliomas are rare, mixed neuronal-glial tumors consisting of mature neoplastic ganglion cells clustered with neoplastic glial cells. The majority of gangliogliomas are considered WHO Grade I or II; however, those demonstrating anaplastic glial components (WHO

Ganglioglioma. Figure 1 Courtesy Hui-Kuo Shu MD, and Carol Armstrong

Grade III) or Grade IV glial changes (glioblastoma) are also well documented (Nelson, Bruner, Wiestler & VandenBerg, 2000). Gangliogliomas are well-differentiated, slow-growing, and relatively benign tumors of the CNS that primarily affect children and young adults. These tumors can occur throughout the neuraxis but arise most commonly in the temporal lobe and the cerebellar hemispheres. Seizures are a common presenting symptom in the context of a temporal lobe locus, specifically complex partial seizures. Gangliogliomas (WHO Grades I–III) account for approximately 40% of chronic tumor-related refractory temporal-lobe epilepsy cases (Nelson, Bruner, Wiestler & VandenBerg, 2000). Surgical gross total resection is the dominant treatment, which is typically curative; metastasis is rare.

References and Readings Nelson, J. S., Bruner, J. M., Wiestler, O. D., & VandenBerg, S. R. (2000). Ganglioglioma and gangliocytoma. In P. Kleihues & W. K. Cavenee (Eds.), World Health Organization classification of tumours. Pathology & genetics. Tumours of the nervous system (pp. 96–98). Lyons, France: IARC Press.

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Ganglion

Ganglion

Gazzaniga, M. S. (1939– )

J OHN B IGBEE Virginia Commonwealth University Richmond, VA, USA

A IMILIA PAPAZOGLOU, T RICIA Z. K ING Georgia State University Atlanta, GA, USA

Definition


A ganglion is a collection of neuronal cell bodies in the peripheral nervous system. An analogous functional collection of neurons in the central nervous system is called a nucleus. Sensory ganglia are present as spinal ganglia on the dorsal roots of spinal nerves and the sensory roots of cranial nerves. Autonomic ganglia contain motor neurons that comprise the parasympathetic and sympathetic subdivisions of the autonomic nervous system.

Major Appointments

Cross References ▶ Autonomic Nervous System ▶ Dorsal Root Ganglia

Garden-Path Sentences ▶ Sentence Completion

Gating

▶ Thalamic Gating

Gaussian Distribution ▶ Bell Curve ▶ Normal Curve

National Institute of Health Fellowship, Institute of Physiology, Pisa, Italy, August–December, 1966 California Institute of Technology, Postgraduate Fellow, 1964–1966 California Institute of Technology, Ph.D., Psychobiology, 1964 Dartmouth College, A.B., 1961

Director, SAGE Center for the Study of the Mind (University of California, Santa Barbara, CA, 2006–Present) President, American Psychological Society (2005–2006) Dean of the Faculty (Dartmouth College, Hanover, NH, 2002–2004) David T. McLaughlin Distinguished Professor; Director, Center for Cognitive Neuroscience (Dartmouth College, Hanover, NH, 1996) Founder, Cognitive Neuroscience Society (1993) Director, Center for Neuroscience; Professor of Neurology and of Psychology (University of California, Davis, CA, 1992–1996) Andrew W. Thomson, Jr. Professor of Psychiatry; Director, Program in Cognitive Neuroscience (Dartmouth Medical School, Hanover, NH, 1988– 1992) President, Cognitive Neuroscience Institute (1982) Director, Division of Cognitive Neuroscience, Department of Neurology; Professor of Neurology and Psychology (Cornell University Medical College, Ithaca, NY, 1977–1988) Professor, Social Sciences in Medicine (State University of New York, Stony Brook, NY, 1975–1978) Elected, State University of New York University-Wide Exchange Scholar (Stony Brook, NY, 1974–1977) Professor, Psychology (State University of New York, Stony Brook, NY, 1973–1978) Professor (New York University Graduate School, NY, 1972–1973)

Gazzaniga, M. S. (1939– )

Associate Professor (New York University Graduate School, NY, 1969–1972) Associate Professor, Psychology; Chairman, Department of Psychology (University of California, Santa Barbara, CA, 1968–1969) Assistant Professor, Psychology; Department of Psychology (University of California, Santa Barbara, CA, 1967–1968)


Elected to the Institute of Medicine, 2005 Appointed to President’s Council on Bioethics, 2002 Honorary Master of Arts Degree, Dartmouth College, May 2000 C.U. Ariens Kappers Medal for Neuroscience, The Royal Netherlands Academy of Arts and Science, 2000 Elected to American Academy of Arts and Science, 1997 Elected Counselor, Society for Neuroscience, 1992–1996 Javits Neuroscience Investigator Award, 1985–1992 John Simon Guggenheim Memorial Fellowship, 1982–1983 The Camille and Henry Dreyfus Distinguished Visiting Scholar, Occidental College, 1982 Elected Fellow of the American Psychological Association, 1982 Elected Fellow of the Society of Experimental Psychologists, 1982 Elected Fellow of the American Neurological Association, 1981 Elected Fellow of the American Association for the Advancement of Science, 1980


Gazzaniga has made fundamental contributions to our understanding of the brain. In particular, he has furthered our knowledge of functional lateralization and how the left and right hemispheres of the brain communicate with each other. He is best known for his groundbreaking work with split-brain patients (individuals who underwent corpus callosotomy to reduce severe generalized seizures). Gazzaniga also is credited with increasing the accessibility to the public of research on the brain through his many books, interviews, and television appearances.

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Short Biography Gazzaniga completed his Ph.D. in Psychobiology under the guidance of Roger Sperry, who pioneered hemispheric lateralization research. During his time as a graduate student at the California Institute of Technology, Gazzaniga developed innovative methodologies to study the behavior of ‘‘split-brain patients,’’ in whom the corpus callosum has been severed (thereby blocking the interhemispheric transfer of information) in an effort to control intractable epilepsy. This neurosurgical procedure experienced a revival around the time Gazzaniga began his graduate studies. Philip Vogel and Joseph Bogen postulated that earlier surgeries had failed to provide seizure control due to an incomplete severing of the corpus callosum. As they predicted, complete severing of all cortical commissures produced much better seizure control, and Bogen approached the Sperry lab about conducting research with these patients. Gazzaniga, who had designed experimental paradigms to study split-brain patients during his time in college, but had not yet had the opportunity to test them, was given that opportunity when he began working with Sperry. Gazzaniga’s groundbreaking experimental paradigm allowed visual information to be presented to only one hemisphere. Patients fixated on a point in space, and information was quickly flashed (to prevent unwanted eye movements) either to the left or right of the fixation point using a slide projector fitted with electronic shutters (tachistoscope). Thus, information flashed to the left visual field was presented only to the right hemisphere. Using this methodology, Gazzaniga discovered that split-brain patients were unable to verbally name (requiring the left hemisphere) a visual stimulus (picture, number, letter, or short word) when presented to the left visual field/right hemisphere (LVF/RH). Although patients frequently verbally reported not having seen anything, they were able to use their left hand to pick out the stimulus from under a screen, or draw the item, or point to the item in a multiple-choice format. Gazzaniga noted that a small minority of patients, within a year of surgery, were able to make single-word vocal responses to LVF/RH stimuli, suggesting that the right hemisphere may have some capacity for language. This research led to many insights regarding hemispheric specialization and interhemispheric interaction. In most people, the left hemisphere is dominant for language, and speech is typically generated from this hemisphere. Although right hemisphere language dominance is rare in individuals without neurological insult, some right

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hemisphere language may be apparent. The ability to read is believed to be a left hemisphere function for most, but the right hemisphere may have a limited capacity for reading. The right hemisphere, in turn, is specialized for visuospatial processing. In addition, Gazzaniga’s work has demonstrated that the left hemisphere appears to be superior to the right with respect to simple calculation. The right hemisphere, however, does appear to have some capability for approximating solutions to simple calculations, even when it is unable to arrive at the exact solution. Although the corpus callosum is severed in split-brain patients, there remain interhemispheric subcortical pathways that may allow for some integration of basic information between the hemispheres. For example, there is evidence for interhemispheric transmission of crude spatial location information. The potential for interhemispheric transmission of higher-order information appears limited at best. Split-brain patients would sometimes draw an integrated picture of two words (one presented to each hemisphere) such as drawing a clock set to 10:00 for the words ‘‘ten’’ and ‘‘clock.’’ When conceptually ambiguous word pairs such as ‘‘hot’’ and ‘‘dog’’ were presented, however, patients always drew them literally (a dog that is panting from the heat rather than a sausage in a bun). In addition to interhemispheric transmission, methods of external communication between the hemispheres also were used by some patients. These techniques, known as cross-cueing, primarily involved ipsilateral manual cueing techniques, such as moving a single finger on the left hand, thereby allowing the mute right hemisphere to communicate the number one to the verbal left hemisphere. Gazzaniga also has examined the functional specificity of the corpus callosum in patients with partial lesions of the callosum. His research has shown that there are areas of the corpus callosum that are specialized for the transfer of specific types of information such as visual or motor information. Gazzaniga has played an important role in increasing the accessibility to the general public of brain research findings. He has published widely on topical issues including ethical issues in neuroscience research. His books include Human: The Science Behind What Makes Us Unique (2008), The Ethical Brain (2005), Mind’s Past (2000), The Cognitive Neurosciences (1995), and Nature’s Mind: The Biological Roots of Thinking, Emotions, Sexuality, Language, and Intelligence (1994) among many others. The ease with which he presents complex material about brain behavior relationship is seen in his participation in public television specials including The Brain and the Mind (1988) and Pieces of Mind, Scientific American Frontiers (1997). Gazzaniga has been particularly

outspoken with respect to his support of biomedical cloning (which encompasses stem cell research). His discussions on these topics are not only insightful, but are presented in a way that is easy to understand and meaningful to the general public. Gazzaniga is widely credited with founding the discipline of cognitive neuroscience. He started the Journal of Cognitive Neuroscience, where he served as Editor-in-Chief for 15 years and was Founding Editor of the Cognitive Neuroscience Society in 1993. Gazzaniga also developed the Centers for Neuroscience during his time at the University of California, Davis and Dartmouth College. Gazzaniga is committed to training new scientists, and has served as a mentor to numerous graduate students and postdoctoral fellows including Joseph LeDoux and Paul Corballis. Gazzaniga is currently Director of the SAGE Center for the Study of the Mind and Professor of Psychology at University of California, Santa Barbara. He is married, and has five daughters and one son.

Cross References ▶ Corpus Callosum ▶ Hemispheric Specialization ▶ Sperry, Roger Wolcott ▶ Split Brain

References and Readings Baynes, K., & Gazzaniga, M. S. (2005). Lateralization of language: Toward a biologically based model of language. The Linguistic Review, 22, 303–326. Funnell, M. G., Colvin, M. K., & Gazzaniga, M. S. (2007). The calculating hemispheres: Study of a split brain patient. Neuropsychologia, 45, 2378–2386. Gazzaniga, M. S. (2005a). Forty-five years of split brain research and still going strong. Nature Reviews Neuroscience, 6, 653–659. Gazzaniga, M. S. (2005b). The ethical brain.Washington, DC: Dana Press. Gazzaniga, M. S., Smylie, C. S., Baynes, K., McCleary, C., & Hirst, W. (1984). Profiles of right hemisphere language and speech following brain bisection. Brain and Language, 22, 206–220. Gazzaniga, M. S., & Sperry, R. W. (1967). Language after section of the cerebral commissures. Brain, 90, 206–220.

GBM ▶ Glioblastoma Multiforme

Gene

GCS ▶ Glasgow Coma Scale

GDS ▶ Geriatric Depression Scale

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Gene R OHAN PALMER 1, M ARTIN H AHN 2 1 University of Colorado Boulder CO., USA 2 William Paterson University Wayne, NJ, USA

Synonyms Allele; DNA

GED ▶ Tests of General Educational Development

Gegenhalten S TEPHEN P. S ALLOWAY Alpert Medical School of Brown University Providence, RI, USA

Synonyms

Definition Fundamentally, a gene is the unit of inheritance that passes information from one generation to the next. This is the gene of Mendel’s experiments in which the characteristics of pea plants (Pisum sativum) were reliably and systematically transported from the parent generation to filial generations and so on. Mendel’s findings on pea plants that genes carry information from generation to generation in all organisms have been generalized to other organisms. In humans, genes carry information about, for example, blood types, hair lines, personality, intelligence, and psychopathology.

Paratonia

Current Knowledge Definition Gegenhalten, from German to resist or hold against, is a form of hypertonia characterized by involuntary variable resistance during passive movement (i.e., a movement without effort). It is usually elicited by moving the patient’s forearm rapidly from a contracted to a stretched position, after instructing the patient not to resist. It is a primitive reflex, absent in adults, but can occur in association with frontal lobe disorders, generalized degenerative diseases, and catatonia. It should be distinguished from cogwheel rigidity, a sign of extrapyramidal syndromes.

References and Readings Goetz, C. G. (2007). Textbook of clinical neurology (3rd ed.). Philadelphia: Saunders.

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As research and thinking about genes continued after Mendel, locating genes on specific chromosomes became possible. In some pioneering work in the early 1900s, T.H. Morgan found that the white eye mutation in Drosophila melanogaster (Drosophila melanogaster normally have red eyes) was located on the X chromosome. In making that discovery, Morgan used mating crosses similar to those that Mendel had employed. With the discovery of the structure of DNA in 1953 by James Watson and Francis Crick, a way was established to locate genes precisely. Today, because of the mouse and human genome projects, genes as sections of a long DNA molecule can be located, and associations between those genes and traits can be established. Such associations, when established, allow for the study of the pathway between a gene and a trait. This allows the researcher to establish how the gene for round or wrinkled ripe seeds in the pea plant actually produces that shape. Or in the human, that may allow the researcher to establish the

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gene to trait pathway that produces amyotrophic lateral sclerosis. Knowledge of the pathway would provide opportunities for intervention. In addition to being a unit of inheritance, i.e., carrying information from generation to generation, genes contain codes that allow for the construction and operation of organism – the gene is a unit of function. Through a series of steps, genes produce proteins and enzymes that build and run organisms. In this process, the code of DNA is transcribed or written across to the similar molecule, RNA. Then, in the cytoplasm of a cell, the RNA code is translated into a sequence of amino acids or a protein. This protein might be a structural protein such as collagen or elastin, which are responsible for the properties of connective tissues in animals. Or the protein might be an enzyme such as alcohol dehydrogenase, which is one enzyme involved in the metabolism of alcohol. In summary, genes are units of function and heredity. They code for proteins that participate in complex gene to trait pathways – producing the structures and functions of organisms. Genes are passed from generation to generation and, as Mendel showed, are responsible for resemblances between one generation and the next.

Current Knowledge Genotype environment interaction refers to changes in the role of genetic factors under different environments. In genetics, this can be visualized as changes in the genetic effect (heritability) on a trait under different environmental conditions (Figs. 1 and 2). In the figures, the evidence of a G–E interaction is that the genetic effect is greater among individuals at the higher end of the environmental measure. Many behaviors and disorders result from the combination of genetic factors and environmental factors, and possibly, their interactions. This has contradicted the early deterministic view of genetics and has opened the door to promising interventions that reduce the risks from genetic predispositions. Examples of G–E interaction come in various forms. For instance, Phenylketonuria (PKU) is an autosomal recessive disorder characterized by a deficiency in phenylalanine hydroxylase, an enzyme needed to metabolize phenylalanine into tyrosine. The major side effect of this disorder is an accumulation of phenyl pyruvic acid ultimately resulting in mental retardation. One form of treatment has been a diet low on phenylalanine. This environmental intervention prevents the negative side effect of the genetic abnormality.

Cross References ▶ Allele ▶ Deoxyribonucleic Acid (DNA) ▶ Genotype

Gene–Environment Interaction R OHAN PALMER 1, M ARTIN H AHN 2 1 University of Colorado Boulder, CO, USA 2 William Paterson University Wayne, NJ, USA

Definition A gene–environment (G–E) interaction refers to the susceptibility of genotypes to different environmental circumstances. In biometrical genetics, it is akin to a statistical interaction such that genetic effects on a phenotype vary as a function of the levels of an environmental variable.

Environment High 5

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3 1 0 Wild Type Variant Genetic Risk

Gene–Environment Interaction. Figure 1 Dichotomous model of gene–environment (G–E) interaction. A simplified example of a dichotomous genotype (i.e., individuals without the genetic risk variant vs. those with the wildtype genotype) and dichotomous environment (low vs. high scores on a risk environment) interaction. The graph shows that genotype increases the odds of contracting a disorder. Additionally, possession of the genetic risk variant increases the odds of the disorder. Furthermore, the genetic risk is increased for individuals who exhibit high scores on the environment measure

General Language Composite

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Gene–Environment Interaction. Figure 2 Continuous model of G–E interaction. A complex example of a continuous genotype (i.e., individuals posses either 0, 1, or 2 copies of the risk allele) and ordinal environment (individuals presents with low, medium, high scores on a risk environment) interaction. The graph shows that as the number of risk alleles possessed increases, the odds-ratio of exhibiting the disorder increases. Furthermore, the genetic risk varies as a function of different levels of the environment

Unlike monogenic disorders such as PKU, complex disorders such as depression are more difficult when it comes to identifying environments that alter genetic susceptibility. Numerous studies have explored the relationship between the serotonin system, stressful life events, and depression; however, only a handful have identified an interaction. The most notable of these studies was conducted by Caspi et al. (2003) who reported that genetic variants in the promoters of the serotonin transporter gene interacted with stressful life events to influence the risk for major depression. In a meta-analysis of 26 studies, Risch et al. (2009) found no evidence of an interaction across these investigations. While the findings for depression are mixed, these studies are complicated by limitations in association studies (Donnelly, 2008), statistical approaches (Eaves, 2006), and identifications of ‘‘true’’ environmental risk factors. Over time, more studies of G–E interaction are expected to emerge as the inclusion of environmental measures in genetic designs becomes more commonplace.

References and Readings Caspi, A., Sugden, K., Moffitt, T. E., Taylor, A., Craig, I. W., Harrington, H., et al. (2003). Influence of life stress on depression: Moderation by a polymorphism in the 5-HTT gene. Science, 301, 386–389.

Donnelly, P. (2008). Progress and challenges in genome-wide association studies in humans. Nature Genetics, 456, 728–731. Eaves, L. J. (2006). Genotype x environment interaction in psychopathology: Fact or artefact? Twin Research and Human Genetics, 8, 1–8.

General Cognitive Ability ▶ Intelligence

General Cognitive Functioning ▶ Intelligence

General Language Composite S ETH WARSCHAUSKY University of Michigan Ann Arbor, MI, USA

Synonyms WPPSI-III GLC

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Description

Psychometric Data

The Wechsler Preschool and Primary Scale of Intelligence – Third Edition (WPPSI-III) is an individually administered intelligence test for children, ages 2 years 6 months (2;6) through 7 years 3 months (7;3). The WPPSI-III is a substantial revision of the WPPSI-R (Wechsler, 1989). The WPPSI-III provides two age-band subtest batteries for ages 2;6–3;11 and 4;0–7;3. Both batteries include a set of core subtests that yield Verbal, Performance and Full Scale composite scores. Supplemental subtests can be added to yield an additional General Language Composite (GLC). In the younger age band, the supplemental subtest Picture Naming is added to the core subtest Receptive Vocabulary to generate the GLC. In the older age band, the supplemental subtests Receptive Vocabulary and Picture Naming are combined to generate the GLC. The WPPSI-III was normed on a stratified sample of 1,700 children, divided into nine age groups, including 200 children in each 6 month interval from age 2;6 to 5;11, 200 six year olds, and 100 children, ages 7;0–7;3. Sample demographics and geographic locations were based on the U.S. Bureau of the Census for 2000.

Internal consistency of the WPPSI-III was examined using split-half methods, with the exception of the PSQ subtests. Composite score reliability coefficients range from .89–96, including an overall reliability coefficient of .94 for the GLC. The GLC standard errors of measurement are acceptable but relatively high when compared with the Verbal, Performance and Full Scale SEs, in part, reflecting the smaller number of subtests that comprise the GLC. Test-retest stability was demonstrated over a mean interval of 26 days. Average corrected stability coefficients of the subtests are adequate (.70s) to excellent (.90s). Average corrected stability coefficients of the Composite scores are excellent, including an overall GLC coefficient of .91. Regarding score differences, the WPPSI-III psychometrics include the statistical significance of Composite and subtest score differences within a profile, as well as base rate frequency of differences in the normative sample. Lines of validity evidence examine internal structure and relationships with other variables. In the younger age band, 2:6–3:11, the GLC is highly correlated with the Verbal IQ, as expected given the overlap in use of the Receptive Vocabulary subtest. In the older age band, the GLC is more highly correlated with the Verbal IQ than with the Performance IQ, though even the latter correlation is strong. Evidence for validity based on relationships with other measures include evidence that the GLC is more highly correlated with the Bayley Scales of Infant Development – II Mental than Motor scale and more highly correlated with the Differential Ability Scale (DAS) Verbal Composite than with the DAS Nonverbal Reasoning and Spatial Composite scores. The GLC also is highly correlated with Wechsler Individual Achievement Test – II Composite scores and has the highest correlation with the WIAT-II Oral Language Composite. The initial psychometric data include validity evidence from special groups but these were small samples of convenience and caution in warranted in generalizing findings to clinical practice. In samples of children previously identified with mild to moderate mental retardation (cognitive impairment), WPPSI-III GLC scores generally fell within the expected range, approximately two standard deviations below the mean for those with mild impairment and almost three standard deviations below the mean for those with moderate impairment. In a small sample (n = 21) with Autistic Disorder and FSIQ 60, consistent with previous research, the mean PIQ of 88.2 was significantly higher than the mean VIQ of 70.6.

Historical Background The Wechsler Intelligence Scale for Children (WISC; Wechsler, 1949) was developed as a children’s version of a revised Wechsler-Bellevue (Wechsler, 1939) scale. In 1967, the WPPSI was published as a preschool version of the WISC, for children ages 4:0–6:6. The initial revision of the WPPSI (WPPSI-R) was published in 1989, with an expanded age range, from 3:0–7:3. The WPPSI-R retained previous subtests but added Object Assembly. Age-determined start points for subtests were introduced. The WPPSI and WPPSI-R generated Verbal, Performance and Full Scale IQ scores. The WPPSI-III (1991) further expanded the age range to 2;6–7;3 and addressed long-standing criticisms of the WPPSI-R by reducing testing time, expressive language demands, and confounding effects of speed. In addition, revisions included more age appropriate test instructions, increased prompts, and simplified administration procedures. Supplemental subtests were added to generate optional composite scores, including the GLC and the Processing Speed Quotient.

General Well-Being Schedule

However, the mean GLC of 84.7 was significantly higher than the VIQ with no accompanying explanation. In children with expressive language disorder compared with matched controls, moderate effect sizes were noted in lower VIQ and FSIQ but GLC scores were not significantly lower. In children with mixed receptive-expressive language disorders, there were large effect sizes in significantly lower VIQ, FSIQ, and GLC Composite scores. In a sample with limited English proficiency compared to matched controls, FSIQ, VIQ, and GLC scores were significantly lower with large effect sizes.

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General Well-Being Schedule J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms GWB; GWBS

Clinical Uses The GLC is designed to be a measure of children’s general language development in receptive and expressive modalities. The GLC primarily was intended for use in the younger age band but may have some utility as a less verbally demanding language test for children in the older age band, as well. Clearly, the GLC is intended as a screening instrument with lower scores suggesting the need for follow-up evaluation with more comprehensive speech and language instruments.

Cross References ▶ Wechsler Intelligence Scale for Children ▶ Wechsler Preschool and Primary Scale of Intelligence

References and Readings Boake, C. (2002). From the Binet-Simon to the Wechsler-Bellevue: Tracing the history of intelligence testing. Journal of Clinical and Experimental Neuropsychology, 24, 383–405. Gordon, B. (2004). Test review: The Wechsler preschool and primary scale of intelligence, third edition (WPPSI-III). Canadian Journal of School Psychology, 19, 205–220. Lichtenberger, E. O. (2005). General measures of cognition for the preschool child. Mental Retardation and Developmental Disabilities Research Reviews, 11, 197–208. Lichtenberger, E. O., & Kaufman, A. S. (2004). Essentials of WPPSI-III assessment. New York: Wiley. Wechsler, D. (1939). The measurement of adult intelligence. Baltimore: Williams & Wilkins. Wechsler, D. (1949). Wechsler intelligence scale for children. San Antonio, TX: The Psychological Corporation. Wechsler, D. (1989). Wechsler preschool and primary scale of intelligence – Revised. San Antonio, TX: The Psychological Corporation. Wechsler, D. (2002). WPPSI-III: Technical and interpretative manual. San Antonio, TX: The Psychological Corporation.

G Definition The GWBS is a brief questionnaire measuring an individual’s subjective sense of well-being and distress over the preceding month. There are 18-, 22-, and 33-item versions (the 18 being most widely used), with items categorized into six domains: anxiety, depression, positive well-being, self-control, vitality, and general health. The 18-item version contains 14 items that are rated on a 6-point scale, and four items that are rated on a 10-point scale. Total scale scores and subscale scores can be derived, with cut-offs defining scores as indicating severe distress, moderate distress, or positive well-being.

Current Knowledge The GWBS was developed in the USA in the 1970s as a brief, simple assessment of health (Dupuy, 1978). It has been used in several large-scale population-based studies, and has recently been validated in ethnic minority groups (e.g., Taylor et al., 2003; Poston et al., 1998), in addition to earlier validation studies with undergraduate participants. A 17-item Japanese translation has also been developed (Nakayama, Toyoda, Ohno, Yoshiike, & Futagami, 2000). GWBS scores are correlated with other measures of depression, and the measure has good reported test-retest reliability and internal consistency (Fazio, 1977). Taylor et al. (2003) reported that several factor analytic studies have failed to support the original six-subscale structure of the GWBS, with the first factor in various models generally accounting for more than half of the observed variance, which is suggestive of a unidimensional structure. The total score is, therefore, reported in most published studies and is of greatest utility.

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Cross References ▶ EuroQol/EQ-5D ▶ SF-36/SF-12

References and Readings Dupuy, H. J. (1978). Self-representations of general psychological well-being of American adults. Paper presented at the American Public Health Association Meeting, Los Angeles, CA. Fazio, A. F. (1977). A concurrent validation study of the NCHS General Well-Being Schedule. Hyattsville, MD: National Center for Health Statistics (Vital and Health Statistics Series 2, No. 73. DHEW Publication No. 78–1347 (HRA)). Nakayama, T., Toyoda, H., Ohno, K., Yoshiike, N., & Futagami, T. (2000). Validity, reliability and acceptability of the Japanese version of the General Well-Being Schedule (GWBS). Quality of Life Research, 9, 529–539. Poston, W. S. C., Olvera, N. E., Yanez, C., Haddock, C. K., Dunn, J. K., Hanis, C. L., & Foreyt, J. P. (1998). Evaluation of the factor structure and psychometric properties of the General Well-Being Schedule (GWB) with Mexican American women. Women and Health, 27, 49–62. Taylor, J. E., Poston W. S., II, Haddock, C. K., Blackburn, G. L., Heber, D., Heymsfield, S. B., et al. (2003). Psychometric characteristics of the General Well-Being Schedule (GWB) with African–American women. Quality of Life Research, 12, 31–39.

determine the conditions or facets which are deemed to be relevant to that assessment instrument. For example, if the relevant facets are temporal stability and examiner influence, the experimental design would involve obtaining test scores on more than one occasion and using more than one examiner. A statistically significant effect for either time or examiner would indicate that there would be inaccuracies in measures across different levels of that facet.

Cross References ▶ Classical Test Theory

References and Readings Brennan, R. L. (2001). Generalizability theory. New York: Springer-Verlag. Brennan, R. L. (2002). Performance assessments from the perspective of generalizability theory. Applied Psychological Measurement, 24, 339–353. Shavelson, R. J., & Webb, N. M. (1991). Generalizability theory: A primer. Newbury Park, CA: Sage.

Generalization Generalizability Theory M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Generalizability theory was developed as an overarching structure with which to evaluate the dependability of psychological measurements. Both reliability and validity are considered to be part of the same conceptual framework. The value of the generalizability score is the result of manipulating different sources of error. The statistical analysis model used is ANOVA.

Current Knowledge In developing a generalizability model for evaluating a clinical assessment instrument, the researcher would first

▶ Stimulus Generalization

Generalized Tonic–Clonic Seizure ▶ Grand Mal Seizure

Generational IQ Gains ▶ Flynn Effect

Geniculocalcarine Fibers (Pathway) ▶ Optic Radiations

Geriatric Depression Scale

Genotype M ARTIN H AHN 1, R OHAN PALMER 2 1 William Paterson University Wayne, NJ, USA 2 University of Colorado at Boulder Boulder, CO, USA

Synonyms DNA

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Description The Geriatric Depression Scale (GDS) is a 30-item selfreport measure of depressive symptoms experienced by the respondent during the past week using a yes/no response format. For 20 items a ‘‘yes’’ response indicates depressive symptomatology and for 10 items a ‘‘no’’ response does so. The items are summed (one point per item) to provide a total score ranging from 0 to 30. Administration time typically ranges from 5 to 10 min. Items may be read to visually challenged or reading-impaired respondents, and telephone administration appears to yield valid and reliable results (Burke, Roccaforte, Wengel, Conley, & Potter, 1995).

Definition The genotype is the complete set of genes carried by an organism. In diploid species such as humans, the mechanisms of sexual reproduction, specifically meiosis and the union of egg and sperm at conception, produce new organisms with sets of genes organized into pairs. In each of those pairs of genes, one gene came from the mother, and the other came from the father. The word genotype is also used to refer to the pair of genes present at one locus of the overall set of pairs.

Cross References ▶ Deoxyribonucleic Acid (DNA) ▶ Gene ▶ Phenotype

Geographical Disorientation ▶ Topographical Disorientation

Historical Background The GDS was developed in two studies (Brink, Yesavage, Lum, Heersema, Adey, & Rose, 1982; Yesavage et al. 1983) to minimize misdiagnosis of depression in the elderly by omitting somatic symptoms that may be common among nondepressed elderly (e.g., sleep disturbance, appetite changes). An additional goal was to develop a measure of depressive symptoms that was brief, simple, and made only modest cognitive demands on the respondent. The 30 items comprising the GDS were empirically selected from a pool of 100 yes/no questions administered to a group of 47 elderly community residents and elderly individuals hospitalized for depression; all respondents in these development studies were aged 55 years or older. Although initially developed and validated with older individuals, other reports suggest the validity of this measure with younger samples, including college students and other younger individuals living in the community (Rule, Harvey, & Dobbs, 1989). A 15-item short form of the GDS was proposed as a screening alternative (Sheikh & Yesavage, 1986), and other, even briefer, forms have been proposed (Almeida & Almeida, 1999).

Geriatric Depression Scale C HAD D. V ICKERY Methodist Rehabilitation Center Jackson, MS, USA

Synonyms GDS; Mood assessment scale

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Psychometric Data The GDS has been the subject of a significant amount of research, and its reliability and validity have been explored in various populations including samples of communityresiding elderly and younger individuals, medical inpatients, psychiatric inpatients and outpatients, and institutionalized (i.e., nursing home or assisted living facility)

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elderly individuals. The properties of both the 30-item form and the 15-item short form have also been explored in individuals with neurological conditions, including mild cognitive impairment, dementia, stroke, and Parkinson’s disease.

Reliability Several forms of reliability have been estimated for the GDS. Internal consistency (Cronbach’s a) estimates range from 0.69 to 0.94 across different samples of varying size and composition (Strauss, Sherman, & Spreen, 2006), with the typical internal consistency in the low to mid 0.80s. There does not appear to be a particular pattern of internal consistency differences among most samples, with the exception of declining reliability in individuals in the advanced stages of dementia (Lezak, Howieson, & Loring, 2004; Strauss et al., 2006). Internal consistency estimates among those with neurological conditions other than dementia are quite similar to estimates in neurologically intact samples. Test–retest reliabilities range from 0.75 to 0.92 in different samples, with an average in the mid 0.80s. Test–retest times have been reported for 1 h to 1 month (McDowell, 2006). Split-half reliabilities range from 0.79 to 0.94, typically averaging in the mid 0.80s. Internal consistency for the 15-item short form has been reported to be similar to that of the 30-item form, with estimates of 0.77–0.81. Test–retest reliability has been estimated to be 0.84–0.85 after 1–2-week intervals (Strauss et al., 2006).

Validity Factor structure. The results of principal components analyses in several reports have been mixed. Five- and sixfactor solutions have been reported, but these findings have not replicated well. Other reports suggest that the GDS measures a unidimensional construct, and recommend its use as a single-factor instrument (Strauss et al., 2006). The 15-item short form generally shows high correlations with the 30-item form, ranging from 0.66 to 0.92. Concurrent validity. Concurrent validity has been explored in numerous studies through correlations with other measures of depression, including other self-report measures (e.g., ▶ Beck Depression Inventory, ▶ Center for Epidemiological Studies-Depression, ▶ Zung Self-Rating Depression Scale) and observer or interviewer-rated forms (e.g., ▶ Hamilton Depression Rating Scale). With few exceptions, these correlations tend to be greater than

0.80. However, weaker correlations between the GDS and other self-report measures are found in samples of individuals with dementia. The 15-item short form also shows significant correlations with other self-report measures, but tend to be more variable than seen in the 30-item form (ranging from 0.30 to 0.84; Almeida & Almeida, 1999; Roger & Johnson-Greene, 2009). Criterion-related validity. Using ‘‘gold standard’’ classifications of depression via criteria found in the Diagnostic and Statistical Manual of Mental Disorders (DSM), the International Classification of Diseases (ICD), and the Research Diagnostic Criteria consistently shows that those individuals judged to be depressed according to these standards obtain significantly higher GDS total scores than nondepressed individuals. In the original validation papers of the GDS, a cutoff score >10 was suggested as providing optimal classification of individuals according to external criteria (sensitivity of 0.84 and specificity of 0.95). As detailed in Strauss et al. (2006), the validity of this and other cutoff scores has been explored in numerous studies with a wide range of results and little agreement. The observed variability of results is likely in part due to the different criteria used to classify individuals as depressed (e.g., DSM, ICD, Research Diagnostic Criteria, clinical consensus). While sensitivity and specificity typically range from the 0.70s to the 0.90s (and generally >0.80) across studies, these values depend on the sample (e.g., community sample vs. medical inpatient) and the cutoff score selected. While a cutoff score >9 may be appropriate for a healthy community sample, a higher cutoff >12 may more accurately classify medical inpatients (Strauss et al., 2006). In general, a cutoff score >11 or >12 is recommended for most samples. In the original development papers (Brink et al., 1982; Yesavage et al., 1983), the authors call scores of 10 or less ‘‘within the normal range,’’ scores of 11–20 reflect ‘‘mild depression,’’ and scores of 21–30 suggest moderate to severe depression. Sheikh and Yesavage (1986) recommended a cutoff score >5 on the 15-item short form as an indicator of elevated depressive symptomatology. This and other recommended cutoff scores (e.g., >4) generally show sensitivity and specificity values similar to those seen for the 30-item form, again dependent on the sample and the criteria used to classify individuals as depressed.

Clinical Uses The GDS has high clinical utility and adequate psychometric properties across a number of different samples

Germ Cell Tumors

and medical conditions. It has the advantages of simplicity and brevity. The GDS has been translated into a number of languages, including Chinese, French, German, Greek, Hebrew, and Spanish. It has shown utility in screening for depression in older as well as younger individuals in the community, in inpatient and outpatient settings, and in different medical/neurological conditions. Normative data for the 30-item form from a large sample, broken down by age decade starting at age 17, can be found in Rule et al. (1989). Also, the 15-item short form shows comparable psychometric qualities to those of the 30-item form, although the research on this short form is not as voluminous as the full form. This should be considered in use with different samples. Despite the usefulness of the GDS in different settings, there are caveats of which the clinician must be mindful. The clinician must consider the setting in which this measure is used and the purpose of the assessment in selecting which cutoff score to use. If more accurate detection of a depressive disorder is the intended purpose, the clinician may wish to use a lower cutoff score to increase sensitivity, while being aware of the cost in specificity. Moreover, the GDS is meant to be a screening measure only; a formal diagnosis of depression should not be made based on GDS scores alone (McDowell, 2006; Strauss et al., 2006). An elevated score on the GDS should be followed by interview to confirm or refute the diagnosis. Finally, the use of the GDS in individuals with a severe dementia is contraindicated. As with other depression measures, the diagnostic accuracy of the GDS is significantly compromised in these individuals due to reports that suggest that the GDS should not be interpreted once Mini-Mental State Examination scores fall below 20 (Strauss et al., 2006).

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References and Readings Almeida, O. P., & Almeida, S. A. (1999). Short versions of the geriatric depression scale: A study of their validity for the diagnosis of a major depressive episode according to ICD-10 and DSM-IV. International Journal of Geriatric Psychiatry, 14, 858–865. Brink, T. L., Yesavage, J. A., Lum, O., Heersema, P. H., Adey, M., & Rose, T. S. (1982). Screening tests for geriatric depression. Clinical Gerontologist, 1, 37–43. Burke, W. J., Roccaforte, W. H., Wengel, S. P., Conley, D. M., & Potter, J. F. (1995). The reliability and validity of the geriatric depression rating scale administered by telephone. Journal of the American Geriatrics Society, 43, 674–679. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. McDowell, I. (2006). Measuring health: A guide to rating scales and questionnaires (3rd ed.). New York: Oxford University Press. Roger, P. R., & Johnson-Greene, D. (2009). Comparison of assessment measures for post-stroke depression. The Clinical Neuropsychologist, 23, 780–793. Rule, B. G., Harvey, H. Z., & Dobbs, A. R. (1989). Reliability of the geriatric depression scale for younger adults. Clinical Gerontologist, 9, 37–43. Sheikh, J. I., & Yesavage, J. A. (1986). Geriatric depression scale (GDS): Recent evidence and development of a shorter version. Clinical Gerontologist, 5, 165–173. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests (3rd ed.). New York: Oxford University Press. Yesavage, J. A., Brink, T. L., Rose, T. S., Lum, O., Huang, V., Adey, M. B., et al. (1983). Development and validation of a geriatric depression rating scale: A preliminary report. Journal of Psychiatric Research, 17, 37–49.

Germ Cell Tumors M I -Y EOUNG J O Private Practice Los Angeles, CA, USA

Cross References

Definition

▶ Beck Depression Inventory ▶ Center for Epidemiological Studies–Depression ▶ Hamilton Depression Rating Scale ▶ Sensitivity ▶ Specificity ▶ Structured Clinical Interview for DSM-IV (SCID-I/SCID-II) ▶ Test Reliability ▶ Test Validity ▶ Zung Self-Rating Depression Scale

Germ cell tumors are tumors comprised primarily of germ cells, which are embryonic in origin. The gonads (testes, ovaries) are the most common sites of germ cell tumors, but they also occur in other regions including the central nervous system. Histologically, germ cell tumors are divided into two types: germinomas and differentiated or non-germinoma germ cells. Intracranially, non-germinoma germ cell tumors are commonly found in midline locations such as the suprasellar region and/or the pineal region. They are relatively rare in both children and

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Gerstmann, Josef (1887–1969) M ICHAEL R. G REHER University of Colorado Denver Denver, CO, USA

Major Appointments Germ Cell Tumors. Figure 1 Courtesy Michael Fisher, MD, Peter C. Phillips, MD. The Children’s Hospital of Philadelphia

adults. Types of non-germinoma germ cell tumors include benign or malignant teratomas, embryonal carcinoma, yolk sac tumor, and choriocarcinoma. Malignant nongerminoma germ cell tumors are often resistant to treatment and have a poorer prognosis. Clinical presentation of non-germinoma germ cell tumors depends on the age of onset and tumor location. Symptoms due to raised intracranial pressure such as headache, nausea, and papilledema are common. Tumors in the suprasellar region may result in endocrine dysfunction and visual deficits. In children, this may cause growth retardation or delayed or precocious puberty. In adults, sexual dysfunction may result. Suprasellar germ cell tumors may also cause posterior pituitary dysfunction with many patients presenting with diabetes insipidus. Large suprasellar tumors can invade nearby regions such as the thalamus or the basal ganglia and produce motor or movement symptoms as a result.

Cross References ▶ Brain Tumor ▶ Intracranial Pressure ▶ Neoplasms ▶ Pineal Tumors

Reservespital, Austro-Hungarian Army, Innsbruk, Austria, World War I University of Vienna, Neurology and Psychiatry, Vienna, Austria, 1918–1930 Institute for Mental and Nervous Disorders, Maria Theresien Schlo¨ssel, Vienna, Austria, 1930–c.1938 St. Elizabeth’s Hospital, Washington, DC, USA, 1941–1942 Neurological Institute of New York, New York, USA, 1941–1945 Goldwater Memorial Hospital, New York, New York, USA, 1941–1946 Postgraduate Hospital, New York, New York, USA, 1941–1946 Neurology practice, New York, New York, USA


Decorated as a medical officer in the Austro-Hungarian Army, World War I Fellow, American Psychiatric Association Fellow, American Academy of Neurology


In 1924, Dr. Gerstmann documented the case of a 52-year-old female with a suspected left hemisphere cerebrovascular accident and symptoms that included finger agnosia, agraphia, right–left disorientation, and acalculia, with intact speech and reading. By 1930, Gerstmann had published two additional papers on the topic which described two more cases with similar presentations. He submitted that an association existed between a lesion in the left angular gyrus involving the transition area between the parietal lobe and the second occipital convolution, and that the aforementioned tetrad of symptoms represented a ‘‘new syndrome,’’ since known as ‘‘Gerstmann

Gerstmann, Josef (1887–1969)

Syndrome.’’ In his initial paper on the subject, Gerstmann also introduced the term ‘‘finger agnosia’’ (Gerstmann, 1940), although Jules Badal noted this same symptom years earlier in 1888 (Critchley, 1966). Gerstmann considered finger agnosia and agraphia the cardinal symptoms of the syndrome and by the time of his 1940 paper argued, based on case studies from a variety of sources, that it ‘‘may now be considered as established’’ (p. 399). As a whole, Gerstmann viewed the syndrome as a neurological deficit of distorted body schema or body image. In addition to its acquired form secondary to some type of neurological injury, there has also been discussion of a developmental Gerstmann Syndrome (Critchley, 1966; Lebrun, 2005). Although initially accepted by many within the field of neurology, in the late 1960s and 1970s a series of papers challenged the existence of Gerstmann Syndrome, resulting in significant scientific debate. Several studies involving systematic research, most notably by Benton (1961), Heimburger, DeMyer, and Reitan (1964), and Poek and Orgass (1966) suggested that the proposed symptoms of Gerstmann Syndrome were no more correlated with one another than they were to other symptoms such as aphasia, constructional dyspraxia, and dyslexia. Consequently, questions were raised as to whether this combination of symptoms represented an autonomous ‘‘syndrome.’’ There was criticism given the minimal number of cases with a pure and complete Gerstmann Syndrome (cases oftentimes presented without the full tetrad of symptoms or with additional associated symptoms) and findings to suggest that it had minimal utility as a method for lesion localization within the angular gyrus of the dominant hemisphere. Skepticism was also raised about the validity of its developmental form (Benton, 1977; Critchley, 1966; Lebrun, 2005). Nevertheless, several have expressed confidence in the existence of Gerstmann Syndrome based on case presentations and/or clinical opinion (Kinsbourne & Warrington, 1963; Ropper & Brown, 2005; Strub & Geschwind, 1974). Gerstmann collaborated with Ernst Straüssler and Ilya M. Scheinker on a paper in 1935 which described a degenerative process in a single family involving symptoms such as dysarthria, cerebellar ataxia, nystagmus, and progressive dementia. Today, the condition is known as Gerstmann–Straüssler–Scheinker Syndrome. It is a rare, autosomal dominant subacute spongiform encephalopathy (SSE). Gerstmann also developed a modification of the Romberg test involving stretching and flexing of the trunk with eyes closed as a method for identifying cerebellar ataxia (Triarhou, 2008).

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Short Biography Dr. Gerstmann was born July 17, 1887 to Joachim and Bertha Gerstmann in Lemberg in the province of Galacia within the Austro-Hungarian empire (today known as Liviv, Ukraine). He married Martha M. Stein on August 24, 1920. Gerstmann received his medical degree at the University of Vienna in 1912, and trained in neurology and psychiatry with Wagner von Jauregg, Nobel laureate in Physiology or Medicine in 1927. He served as a medical officer in the Austro-Hungarian army in World War I, later joined the faculty in Neurology and Psychiatry at the University of Vienna, and was subsequently appointed as the director of Vienna’s Institute for Mental and Nervous Disorders at the Maria Theresien Schlo¨ssel. Like other Jews in Europe at that time, Gerstmann fled from Vienna following the Nazi occupation of Austria and immigrated to the United States in 1938. He settled in New York City, practiced neurology, and affiliated for some years with hospitals such as St. Elizabeth’s Hospital in Washington, DC, the Neurological Institute of New York, Goldwater Memorial Hospital and Postgraduate Hospital (IRB, 1961; Lebrun, 2005; Triarhou, 2008). Gerstmann published over 100 papers, authored a monograph on the treatment of progressive paralysis in malaria (Die Malaria-behandlung der progressiven Paralyse), and gave numerous presentations at scientific meetings in both Europe (before fleeing in 1938) and in the USA. His interests and publications were varied and included topics such as epilepsy, mental retardation, astereognosis, traumatic brain injury secondary to gunshot wounds, micrographia, encephalomyelitis, and multiple sclerosis (Triarhou, 2008). However, he is most well known for the proposed syndrome which today still bears his name and the controversies and disagreements surrounding it. Gerstmann continued to work into his late years and died at the age of 82 on March 23, 1969. His wife Martha, with the assistance of Viennese neurologist Karl Gloning, had his remaining notes on Gerstmann Syndrome published posthumously (Lebrun, 2005; Triarhou, 2008). Although still controversial in terms of its validity as an actual syndrome and not a frequent consideration in differential diagnosis by most current day neuropsychologists, many in the field recognize Gerstmann Syndrome as central to the evolution of our understanding of brain–behavior relationships, particularly with regard to the tetrad of symptoms and the functions and dysfunctions of the parietal lobe. Given this and Gerstmann’s involvement in identifying what is known today as Gerstmann–Straüssler–Scheinker Syndrome, he is

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commonly regarded as an important historical figure in the fields of neurology and neuropsychology.

Cross References ▶ Acalculia ▶ Agraphia ▶ Ataxia ▶ Benton, Arthur (1909–2006) ▶ Developmental Gerstmann Syndrome ▶ Finger Agnosia ▶ Gerstmann’s Syndrome ▶ Geschwind, Norman (1926–1984) ▶ Kinsbourne, Marcel (1931– ) ▶ Parietal Lobe ▶ Prion Disease ▶ Right Left Disorientation ▶ Romberg Test ▶ Syndrome ▶ Warrington, Elizabeth

References and Readings Benke, T. (2001). Early concepts of tactile object recognition: An historical synopsis and appraisal of Josef Gerstmann’s Reine taktile Agnosie (1918). Cognitive Neuropsychology, 18, 263–266. Benton, A. (1961). The fiction of the ‘‘Gerstmann syndrome.’’ Journal of Neurology, Neurosurgery, and Psychiatry, 24, 176–181. Benton, A. (1977). Reflections on the Gerstmann syndrome. Brain and Language, 4, 45–62. Critchley, M. (1966). The enigma of Gerstmann’s syndrome. Brain, 89, 183–197. Gerstmann, J. (1940). Syndrome of finger agnosia, disorientation for right and left, agraphia and acalculia. Archives of Neurology and Psychiatry, 44, 398–407. Heimburger, R., DeMeyer, W., & Reitan, R. (1964). Implications of Gerstmann’s syndrome. Journal of Neurology, Neurosurgery, and Psychiatry, 27, 52–57. Institute for Research in Biography (1961). American men of medicine (3rd ed.). New York: Institute for Research in Biography. Kinsbourne, M., & Warrington, E. (1963). A study of finger agnosia. Brain, 85, 47–66. Lebrun, Y. (2005). Gerstmann’s syndrome. Journal of Neurolinguistics, 18, 371–326. Poeck, K., & Orgass, B. (1966). Gerstmann’s syndrome and aphasia. Cortex, 2, 421–437. Ropper, A. H., & Brown, R. H. (2005). Adams and Victor’s principles of neurology (8th ed.). New York: McGraw-Hill. Strub, R., & Geschwind, N. (1974). Gerstmann syndrome without aphasia, Cortex, 10, 378–387. Triarhou, L. C. (2008). Josef Gerstmann (1887–1969). Journal of Neurology, 255, 614–615.

Gerstmann’s Syndrome J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Synonyms Angular gyrus syndrome

Definition A collection of symptoms which includes finger agnosia, right-left disorientation, dyscalculia, and agraphia. There is controversy as to whether this constellation of deficits should be considered a ‘‘syndrome,’’ as the four elements can occur in various partial combinations as well as with a number of other neurobehavioral symptoms. However, there is greater consensus that if all four symptoms are present, there is a high level of probability that constructional deficits will also be present and the lesion will include the angular gyrus of the left hemisphere. While most frequently seen in adults following left parietal strokes, occasionally this same tetrad of symptoms, usually along with other deficits such as constructional and reading difficulties, can be found in children. The term ‘‘developmental Gerstmann’s syndrome’’ has been applied in these latter instances. However, a controversy similar to that seen in adult populations exits with regard to children, namely does this represent a specific syndrome or merely reflect a broader neurodevelopmental disorder.

Cross References ▶ Acalculia ▶ Agraphia ▶ Developmental Gerstmann Syndrome ▶ Finger Agnosia ▶ Right Left Disorientation

References and Readings Benton, A. (1961). The fiction of the Gerstmann syndrome. Journal of Neurology, Neurosurgery and Psychiatry, 24, 176–181. Benton, A. (1977). Reflections on the Gerstmann syndrome. Brain and Language, 4(1), 45–62.

Geschwind, Norman (1926–1984) Denberg, N. L., & Tranel, D. (2003). Acalculia and disturbances of the body schema. In K. M. Heilman, & E. Valenstein (Eds.), Clinical neuropsychology (pp. 161–184). New York: Oxford University Press. Hecaen, H., & Albert, M. L. (1978). Human neuropsychology. New York: John Wiley & Sons. Miller, C. J., & Hynd, G. W. (2004). What ever happened to developmental Gerstmann’s syndrome? Journal of Child Neurology, 19(4), 282–289.

Geschwind Syndrome (or Waxman–Geschwind Syndrome) ▶ Interictal Behavior Syndrome

Geschwind, Norman (1926–1984) P ETER W. S EELY 1, A NNA B ACON M OORE 1,2 1 Rehabilitation Research & Development Center at the Atlanta VAMC Decatur, GA, 2 Emory University School of Medicine Atlanta, GA, USA

Major Appointments

Mosley Traveling Fellowship, National Hospital in London, 1952–1953 United States Public Health Service Fellowship, National Hospital in London, 1953–1955 Chief Resident (Neurology), Boston City Hospital, 1955 Research Fellowship (Biology), Massachusetts Institute of Technology, 1956–1958 Neurologist, Boston Veterans Administration Hospital, 1958–1962 Chief of Neurology, Boston Veterans Administration Hospital, 1963–1966 Director, Department of Neurology, Boston University Aphasia Center, 1966–1968 James Jackson Putnam Professor of Neurology, Harvard Medical School, 1969–1984


Honorary Doctorate, Northwestern University, 1980 Honorary Doctorate, Universite´ Claude Bernard, 1981 Honorary Member, French Society of Neurology

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Honorary Member, Belgain Royal Academy of Medicine Pattison Prize in Neuroscience, 1982 Leonard Cammer Memorial Award, 1982 Fellow, American Academy of Neurology Member, Academy of Aphasia Member, American Neurological Association of Research


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Norman Geschwind’s scientific contributions span many distinct fields of research within neuropsychology and neurology. Generally, Geschwind is regarded as the father of behavioral neurology. Through emphasizing the synergy between his clinical and research experiences, he also provided the foundation of modern knowledge concerning aphasias, apraxias, and agnosias. His research in these areas, which was initiated by his interest in classic lesion studies, is the basis for behavioral neurology. One of his most provocative and enduring findings concerned the novel concept of a ‘‘disconnection syndrome.’’ Based upon the early work of Karl Wernicke, Geschwind found that disconnected language zones in the right and left hemispheres along the corpus callosum can lead to aphasia. This work resulted in the 1965 publication of ‘‘Disconnection Syndromes in Animals and Man,’’ which discussed links between areas in the brain and their subsequent effects on behavior. Geschwind’s knowledge of classic lesion studies and integration across multiple studies breathed new life into the connectionist models born in the era of Wernicke and colleagues. Although perhaps best regarded for his work in connectionist models of language, Geschwind made major contributions to the study of brain asymmetries. At the time, the prevailing view was that language was predominantly located in the left hemisphere of righthanded people; however, there was no significant evidence in support of structural asymmetries in the brain. After thoroughly analyzing classic literature once again, Geschwind found numerous studies that cited anatomic asymmetries in men and animals. After conducting his own study in 1968, he was able to definitively conclude that the temporal planum is laterally asymmetrical. Such work is still prevalent in neuropsychological research today in efforts to understand the relationship between brain symmetry, dyslexia, and other language processing disorders.

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Geschwind, Norman (1926–1984)

Geschwind’s research on temporal lobe epilepsy proved to be instrumental in understanding its subsequent effects on behavior and affect. He proposed that these effects were the result of the activation of the limbic system. His theory helped to demonstrate the connection between temporal lobe epilepsy and psychiatric symptoms. This research implied that mental patients with temporal lobe epilepsy suffered from a neurologic, rather than emotional, problem. This distinction guided future treatment and assessment. Many consider Geschwind’s most prominent scientific contribution to be the Geschwind–Behan–Galaburda (GBG) theory of handedness, which was presented in 1987. This study was centered upon the relationship between hemispheric dominance and the immunologic and endocrine systems. More specifically, the theory suggested a significant correlation between left-handedness and language, developmental disorders, and immune disorders. This contribution has inspired a substantial amount of further research and debate in the neurological community.

Short Biography Norman Geschwind was born on January 8, 1926, in New York City. His mother and father had arrived in the USA 20 years earlier from Polish Galicia. When Norman was 4 years, his father died, prompting his family to move to Brooklyn. This move proved to play an integral role in Norman’s intellectual development, as he attended the Hebrew Institute of Borough Park and, subsequently, Boy’s Day School. It was his time at the prestigious Boy’s Day School that sparked his interest in language, where he studied Greek, Latin, and French, and graduated among the top five in his class. Geschwind’s success in school led to his matriculation to Harvard College on a Pulitzer Scholarship in 1942. However, his studies were interrupted in 1944 by his military service in World War II, during which he was stationed in Czechoslovakia, Germany, and Japan. Fortunately, he returned to complete his undergraduate degree at Harvard College and graduated with honors in 1947. Geschwind continued his studies at Harvard Medical School, where he also graduated with honors in 1951. He then completed his medical internship at Beth Israel Hospital in Boston. His career in neurology truly began in 1952, when he spent 3 years in London on a Moseley Fellowship and a US Public Service Fellowship. During this time, he worked

under Sir Charles Symonds at the National Hospital in London. Geschwind returned to the USA in 1955 and became the neurology Chief Resident at Boston City Hospital under Dr. Derek Denny-Brown. Following this tenure, he engaged in a 2-year research fellowship at the Massachusetts Institute of Technology with Dr. Francis Schmitt, where he studied axonal physiology. In 1958, he joined Dr. Fred Quadfasel’s Neurology Department at the Boston Veterans Administration Hospital. This position allowed Geschwind to shift his research focus from neurophysiology to psychology, which sparked his interest in higher mental processes. It was also during this time that Geschwind began to review classic neurological texts. Following Quadfasel’s retirement in 1963, Geschwind was appointed to Chief of Neurology. Three years later, he became Professor, Director, and Chairman of the newly formed Boston University Aphasia Center. To this day, the center remains one of the most renowned aphasia research centers both nationally and internationally. He returned to Harvard Medical School in 1969, where he succeeded Dr. Derek Denny-Brown as the James Jackson Putman Professor of Neurology and Director of the Neurological Unit at Boston City Hospital. The unit was moved to Beth Israel Hospital in 1975. He received the opportunity to return to the National Hospital in London while on sabbatical in 1976, where he researched and taught for 2 years. Geschwind remained chief of the Neurological Unit while serving as a professor of neurology at Harvard until his untimely death due to a heart attack in 1984. Geschwind’s interests transcended neurological research. He was also known as an extremely engaging and popular professor. He considered studentship to be of the utmost importance, and lectured using vivid examples from his clinical experiences. He was also renowned for his ability to call on knowledge from a wide variety of areas seemingly astray from his area of expertise. Geschwind’s love for teaching resulted in numerous visits to universities throughout the world, during which he delivered keynote addresses and lectures on a variety of topics. Much to the listener’s delight, he frequently lectured in the language native of that country. In addition, many of Geschwind’s students maintain prominent roles in neuropsychology and neurology today, forming an ongoing testament to the quality of his teaching. Some of his most notable students or mentees include Dr. Kenneth Heilman, Dr. Elliot Ross, Dr. Antonio Damasio, Dr. D. Frank Benson, and Dr. David Caplan. Through his students, and now their own students, interns, and fellows, the legacy and contribution of Dr. Geschwind lives on in a very tangible and profound way.

Glasgow Coma Scale

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Cross References

Definition

▶ Aphasia ▶ Asymmetry ▶ Corpus Callosum ▶ Handedness ▶ Heilman, Kenneth M. (1938– ) ▶ Neuropsychology ▶ Temporal Lobe Epilepsy ▶ Wernicke, Karl

The glabellar reflex is elicited by repeatedly tapping the patient between the eyebrows (the glabella area), causing them to blink. Normally, the adult patient habituates to the stimulus, and ceases blinking after a few taps. If blinking persists, it is abnormal in adults. It is best to stand on the side of the patient and softly tap the glabellar area with a reflex hammer from above to avoid eliciting the blink reflex to threat. It is one of the frontal release signs, primitive reflexes that are normal in infants, disappear with brain maturation allowing inhibition, and reappear (are ‘‘released’’) in disorders that affect the frontal lobes. Like most primitive reflexes, the glabellar reflex probably has evolutionary/adaptive advantage in infant apes, protecting the eyes from threat.

References and Readings Damasio, A., & Galaburda, A. (1985). Norman Geschwind. Archives of Neurology, 42, 500–504. Devinsky, O., & Schachter, S. (1997). Behavioral Neurology and the legacy of Norman Geschwind. Philadelphia: Lippincott-Raven. Galaburda, A. (1985). Norman Geschwind 1926–1984. Neuropsychologia, 23, 297–304. Galaburda, A. M., LeMay, M., Kemper, T. L., & Geshwind, N. (1978). Right-left asymmetrics in the brain. Science, 199(4331), 852–856. Geshwind, N. (1965). Disconnexion syndromes in animals and man. Brain, 88, 237. Morrell, F. (1985). Norman Geschwind 1926–1984: An appreciation. Neurology, 35, 660–661.

Gestalt ▶ Simultaneous Processing

Gesture to Command ▶ Praxis

Gilles De La Tourette Syndrome


Glasgow Coma Scale J ERRY W RIGHT Rehabilitation Research Center, Santa Clara Valley Medical Center San Jose, CA, USA

Synonyms GCS

▶ Tourette Syndrome

Description

Glabellar Reflex S TEPHEN P. S ALLOWAY Alpert Medical School of Brown University Providence, RI, USA

Synonyms Myerson’s sign

The Glasgow Coma Scale (GCS) is the most commonly used method for measuring the level of responsiveness in patients with acute brain damage. Three factors, each rated on an ordinal scale, make up the GCS total score (range = 3–15): eye opening (1 = no eye opening to 4 = spontaneous), best motor response (1 = no motor response to 6 = obeys commands), best verbal response (1 = no verbal response to 5 = oriented). Duration of coma has been defined as the length of time during which the patient earns GCS scores less than

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or equal to 8 (Hall, 1997), as well as duration with GCS motor score less than 6 (TBIMS, 2010). Individuals with GCS scores between 3 and 8 are classified as having a severe brain injury; scores between 9 and 12 reflect moderate brain injury, and scores between 13 and 15 are consistent with mild brain injury (more recent literature has proposed that mild injuries be restricted to GCS scores of 14–15). Those with GCS of 13–15 and positive neuroradiological findings are categorized as ‘‘complicated mild’’ injuries. The GCS is a nonproprietary measure. The GCS can be completed in a brief amount of time (usually less than 5 min). For reliability purposes, it is important that the administrator be well trained. The GCS is typically collected in the field by emergency medical services, in the emergency department, and then serially administered by acute care staff (often nursing). There has been variability reported in practice on how to deal with missing GCS data (in cases of intubation and pharmacologically paralyzed patients). There currently is no consensus.

Historical Background The GCS was developed by Teasdale and Jennett (1974) with well-defined scores for motor response, verbal response, and eye opening. Prior to this approach, assessment of level of consciousness was unreliable because of subjective judgments by raters and poorly defined concepts relating to consciousness. In 1977, Jennett and Teasdale reported the results of summing the motor, verbal, and eye opening scores, leading to the GCS total or score reflecting overall responsiveness.

Clinical Uses The GCS is used to describe the severity of an acute brain injury. Serial GCS scores are also used to determine the duration or emergence from coma.

Cross References ▶ Coma ▶ Mild Traumatic Brain Injury ▶ Moderate Brain Injury ▶ Severe Brain Injury

References and Readings Hall, K. M. (1997). Establishing a national traumatic brain injury information system based on a unified data set. Archives of Physical Medicine and Rehabilitation, 78, S5–S11. Jennett, B., & Teasdale, G. (1977). Aspects of coma after severe head injury. The Lancet, 1, 878–881. Moore, L., Lavoie, A., Camden, S., Le Sage, N., et al. (2006). Statistical validation of the Glasgow Coma Score. The Journal of Trauma, 60, 1238–1244. Prasad, K. (1996). The Glasgow Coma Scale: A critical appraisal of its clinimetric properties. Journal of Clinical Epidemiology, 49, 755–763. Teasdale, G., & Jennett, B. (1974). Assessment of coma and impaired consciousness: A practical scale. The Lancet, 2, 81–84. Traumatic Brain Injury Model Systems online syllabus (2010). www.tbindsc.org. Zasler, N. D. (1997). Prognostic indicators in medical rehabilitation of traumatic brain injury: A commentary and review. Archives of Physical Medicine and Rehabilitation, 78, S12–S16.

Glasgow Outcome Scale Psychometric Data Validity: The GCS score has been shown to be highly associated with the acute issues of morbidity and mortality, but is less strongly associated with long-term functional outcomes (Zasler, 1997). Some studies report that the motor score is most important for predicting outcome, while others report that the summed total score is more informative (Moore et al., 2006). There has also been some disagreement as to the time period for the best predictive GCS score (how soon after injury), or whether the score should reflect the best or worst GCS ratings within a given time period. Reliability: The scale has good reliability, especially with trained users (Prasad, 1996).

J ERRY W RIGHT Santa Clara Valley Medical Center San Jose, CA, USA

Synonyms GOS

Description The Glasgow Outcome Scale (GOS) is perhaps the most widely used measure for assessing global outcome

Glasgow Outcome Scale

following a brain injury. The scale consists of five ordinal outcome categories: good recovery (able to live independently, able to return to work or school), moderate disability (able to live independently, unable to return to work or school), severe disability (able to follow commands, unable to live independently), persistent vegetative state (unable to interact with the environment, unresponsive), and death. A criticism of the GOS is that the categories are too broad to detect small, but potentially meaningful, changes. A nonproprietary measure, the GOS can be completed quickly (usually less than 5 min). The GOS does not require a complicated examination and can be used by professionals from many different backgrounds. A structured interview has been developed, which can be administered in person or over the phone. The interview contains multiple choice questions related to the ability to do a range of activities, from the very basic ones such as obeying simple commands or completing activities of daily living to more complex and higher-level functions like return to employment, social and leisure activities, and possible disruptions in relationships with family and friends. For each item, the pre-injury ability for the activity also figures into the score calculation. The completed structured interview yields a score for the Extended Glasgow Outcome Score (GOS-E, which has eight levels), which is then collapsed into the GOS. Additional information on the GOS is available at the Center for Outcome Measurement in Brain Injury (www. tbims.org/combi/gos).

Historical Background The GOS was developed by Jennett and Bond in 1975. On April 1, 1998, the GOS was adopted for follow-up data collection as part of the National Institute on Disability and Rehabilitation Research (NIDRR) Traumatic Brain Injury Model Systems (TBIMS) national database. Traditionally, the scoring of the GOS was assigned following an unstructured interview with the patient or a knowledgeable family member. In 1998 a structured interview was developed (Wilson, Pettigrew, & Teasdale, 1998).

Psychometric Data Validity The GOS has been found to have strong associations with injury severity (posttraumatic amnesia), other disability

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scales (Disability Rating Scale), measures of health outcome (Beck depression inventory, Short Form-36), and frequency of reported symptoms listed in the neurobehavioral functioning inventory (Wilson, Pettigrew, & Teasdale, 2000).

Reliability Use of the structured interview increases reliability (kappa value of 0.89; Wilson et al., 1998). The structured interview form can be reliably completed by phone interview (Pettigrew, Wilson, & Teasdale, 2003).

Ceiling Effects A score of ‘‘Good recovery’’ also includes individuals with mild disabilities. In a community sample, 2–9 years following moderate to severe brain injury, it was found that 69% of the individuals were at the ceiling for this instrument.

Clinical Uses The GOS is a useful summary or global assessment following a brain injury. Many clinicians are familiar with the instrument and what the individual scores mean.

Cross References ▶ Center for Outcome Measurement in Brain Injury ▶ Glasgow Outcome Scale – Extended ▶ Outcome, Outcome Measurement ▶ Traumatic Brain Injury ▶ Traumatic Brain Injury Model System

References and Readings Center for Outcome Measurement in Brain Injury (www.tbims.org/ combi/gos). Hall, K. M., Bushnik, T., Lakisic-Kazazic, B. et al. (2001). Assessing traumatic brain injury outcome measures for long-term follow-up of community-based individuals. Archives of Physical Medicine and Rehabilitation, 82, 367–374. Jennett, B., & Bond, M. (1975). Assessment of outcome after severe brain damage. The Lancet, 1, 480–484. Pettigrew, L. E. L., Wilson, J. T. L., & Teasdale, G. M. (2003). Reliability of ratings on the Glasgow Outcome Scales from in-person and

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Glasgow Outcome Scale – Extended

telephone structured interviews. Journal of Head Trauma Rehabilitation, 18, 252–258. Wilson, J. T. L., Pettigrew, L. E. L., & Teasdale, G. M. (1998). Structured interview for the Glasgow Outcome Scale and the Extended Glasgow Outcome Scale: Guidelines for their use. Journal of Neurotrauma, 15, 573–585. Wilson, J. T. L., Pettigrew, L. E. L., & Teasdale, G. M. (2000). Emotional and cognitive consequences of head injury in relation to the Glasgow Outcome Scale. Journal of Neurology, Neurosurgery & Psychiatry, 69, 204–209.

Glasgow Outcome Scale – Extended J ERRY W RIGHT Santa Clara Valley Medical Center San Jose, CA, USA

Synonyms Extended glasgow outcome scale; GOSE

The GOSE does not require a complicated examination, and can be used by professionals from many different backgrounds. A structured interview has been developed, which can be administered in person or over the phone. The interview contains multiple-choice questions related to ability to do a range of activities, from very basic ones such as obeying simple commands or completing activities of daily living to more complex and higher level functions like return to employment, social and leisure activities, and possible disruptions in relationships with family and friends. For each item, the pre-injury ability for the activity also figures into the score calculation. Additional information on the GOSE is available at the Center for Outcome Measurement in Brain Injury (www.tbims.org/combi/gose).

Historical Background The GOSE was developed by Wilson, Pettigrew, and Teasdale in 1998. On July 1, 2000, the GOSE was adopted for follow-up data collection as part of the National Institute on Disability and Rehabilitation Research (NIDRR) Traumatic Brain Injury Model Systems (TBIMS) national database.

Description Psychometric Data The Extended Glasgow Outcome Scale (GOSE) was developed to address the limitations of the original Glasgow Outcome Scale (GOS), including the use of broad categories that can be insensitive to small, but meaningful, changes in function. The GOSE extends the original 5 GOS scores to 8 by dividing each of the three middle to upper GOS scores (severe disability, moderate disability, good recovery) into two new scores. The 8 scores are: Dead, Vegetative State, Lower Severe Disability, Upper Severe Disability, Lower Moderate Disability, Upper Moderate Disability, Lower Good Recovery, and Upper Good Recovery. Broad item categories are similar to the GOS: good recovery (able to live independently and able to return to work or school), moderate disability (able to live independently and unable to return to work or school), severe disability (able to follow commands and unable to live independently), persistent vegetative state (unable to interact with the environment and unresponsive), and death. Discrimination between upper and lower scores relates to frequency of assistance, working restrictions, and social restrictions. The GOSE is a nonproprietary measure. The GOSE can be completed quickly, usually requiring less than 5 min.

Validity The GOSE has strong associations with injury severity (post-traumatic amnesia), other disability scales (Disability Rating Scale), measures of health outcome (Beck depression inventory, Short Form-36), and with frequency of reported symptoms listed in the neurobehavioral functioning inventory (Wilson et al., 2000).

Reliability Use of the structured interview increases reliability (kappa value of 0.85, Wilson, Pettigrew, & Teasdale, 1998). The structured interview form can be reliably completed by phone interview (Pettigrew, Wilson, & Teasdale, 2003).

Clinical Uses The GOSE is a useful summary or global assessment of functioning following brain injury.

Glioblastoma Multiforme

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Cross References

Definition

▶ Center for Outcome Measurement in Brain Injury ▶ Glasgow Outcome Scale ▶ Outcome, Outcome Measurement ▶ Traumatic Brain Injury ▶ Traumatic Brain Injury Model System

The glioblastoma multiforme (GBM) is a tumor of the central nervous system. It is a rapidly growing glioma, which is invasive, structurally undifferentiated (anaplastic), and undergoing mitotic division. It is graded at levels III or IV to indicate high malignancy. GBM may occur anywhere in the central nervous system. It accounts for about 15–20% of all intracranial tumors, about 55% of all gliomas, and 90% of all cerebral gliomas.

References and Readings Center for Outcome Measurement in Brain Injury (www.tbims.org/ combi/gose). Pettigrew, L. E. L., Wilson, J. T. L., & Teasdale, G. M. (2003). Reliability of ratings on the Glasgow Outcome Scales from in-person and telephone structured interviews. Journal of Head Trauma Rehabilitation, 18, 252–258. Wilson, J. T. L., Pettigrew, L. E. L., & Teasdale, G. M. (1998). Structured interview for the Glasgow Outcome Scale and the Extended Glasgow Outcome Scale: Guidelines for their use. Journal of Neurotrauma, 15, 573–585. Wilson, J. T. L., Pettigrew, L. E. L., & Teasdale, G. M. (2000). Emotional and cognitive consequences of head injury in relation to the Glasgow Outcome Scale. Journal of Neurology, Neurosurgery & Psychiatry, 69, 204–209.

Current Knowledge Symptoms The glioblastoma multiforme may grow to enormous size before clinical signs develop. Typically, focal, neurological, and neuropsychological signs that represent the locus of the tumor are present, and generalized signs that represent the mechanical distortion of the cerebral structures displaced by its size are also present.

Pathophysiology

Glia ▶ Neuroglia

Glial Cells ▶ Neuroglia

Glioblastoma Multiforme J ACQUELINE L. C UNNINGHAM The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms GBM

GBM results from neoplastic proliferations of astrocytes, the type of glial cell most commonly associated with gliomas. The cells are nonneuronal, and in their normal state, serve as supportive cells of the nervous system. In addition to abnormal glial cell proliferation, the glioblastoma multiforme develops its own vascular supply, and thus its entire structure underlies the production of a lesion of mass effect. It is unknown as to whether oligodendrogliomas can transform to GBM.

Treatment The treatment of patients with glioblastoma multiforme is usually palliative, without expectation of a cure. Patients are treated with maximal surgical resection with the hope of producing a longer survival and better quality of life. The addition of radiotherapy increases the duration of survival, but is not curative. There appears to be a small increase in median survival associated with the inclusion of chemotherapy in the overall treatment regime, with better results expected from the use of multiple chemotherapeutic agents, with nonoverlapping toxicity and independent mechanisms of action, than from the use of a single agent. Although GBMs are less common in children than in

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Glioma

Cross References ▶ Astrocytoma ▶ Brain Tumor ▶ Glioma ▶ Neoplasms ▶ Tumor Grade


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Schiff, D., & Wen, P. Y. (2003). Cancer neurology in clinical practice. New York: Humana Press. Stupp, R., Mason, W. P., & van den Bent, M. J. (2005). Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. New England Journal of Medicine, 352, 987–996.

Glioma J ENNIFER T INKER Drexel University Philadelphia, PA, USA

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c Glioblastoma Multiforme. Figure 1 Courtesy Hui-Kuo Shu MD, and Carol Armstrong

adults, the pediatric outcome is more variable than it is for adults. The immediate prognosis for children may be 2 years rather than the 6–12 months given to adults, but there are cases of children living for several years with this type of tumor, and treatment may be quite aggressive.

Definition Gliomas are the most common primary tumor of the CNS, accounting for approximately 75% of malignant adult primary CNS tumors (Kleihues & Cavenee, 2000). They arise from glial cells and their precursors within the central nervous system. Gliomas are most commonly found in the white matter of the cerebral hemispheres, but can also invade or infringe on gray matter. Known for their potential for proliferation, gliomas can also invade the spinal cord. In addition to abnormal signal seen on CT, MRI, or PET, gliomas also invade normal-appearing parenchyma that is peripheral to the abnormal signal, and may grow extensively before symptoms become apparent. However, radiotherapy targets only the 2 cm bed around the tumor, and thus tumor recurrence is common. The relative subtlety of early gliomas is presumably because the invaded normal-appearing brain tissue can still function, and a significant increase in symptoms is often found after surgical resection due to the removal of this tissue (Harpold, Alvord, & Swanson, 2007). Types of gliomas include astrocytoma, which is the most commonly diagnosed, as well as glioblastoma multiforme, oligodendrogliomas, ependymomas, and mixed gliomas (which include a combination of oligodendroglial

Gliosis

and astrocytic components). Originating from neuroglial cells, gliomas are a highly heterogeneous group of tumors that demonstrate variable response to therapy. Gliomas are classified as either low-grade tumors (e.g., ▶ pilocytic astrocytoma, ▶ Juvenile pilocytic astrocytoma), which are typically slow-growing and benign, or high-grade tumors (e.g., ▶ glioblastoma multiforme), which are rapidly proliferating, malignant tumors. In adults, gliomas are most commonly found in the cerebral hemispheres. In children, the majority of gliomas arise infratentorially. Anderson, Damasio, and Tranel (1990) compared the cognitive effects of tumor versus stroke after controlling the size and location of the lesion. The cognitive effects of tumor were found to be subtler and less severe than those caused by stroke. Tumors affect cognitive process relying on distributed systems and multiple processors, and stroke may cause disconnection syndromes that are rare in tumors unless there are multiple lesions in critical locations. Thus, the focal effects of stroke are often more severe than those seen in tumor patients.

Cross References ▶ Astrocytoma ▶ Ependymoma ▶ Glioblastoma Multiforme ▶ Oligodendroglioma

References and Readings Anderson, S. W., Damasion, H., & Tranel, D. (1990). Neuropsychological impairments associated with lesions caused by tumor or stroke. Archives of Neurology, 47, 397–405. Harpold, H. L. P., Alvord, E. C., & Swanson, K. R. (2007). The evolution of mathematical modeling of glioma proliferation and invasion. Journal of Neuropathology and Experimental Neurology, 66(1), 1–9. Kleihues, P., & Cavenee, W. K. (Eds.). (2000). World health organization classification of tumours. Pathology & genetics. Tumours of the nervous system. Lyons, France: IARC Press.

Gliomatosis Cerebri

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Definition Gliomatosis cerebri is a rare type of aggressive, malignant tumor of astrocytic origin that is characterized by individual cells that diffusely infiltrate the brain with poorly circumscribed boundaries. It affects both white and gray matter in the cerebrum and can also occur in the cerebellum, brain stem, and spinal cord. It often arises in adults in their 30s or 40s. Gliomatosis cerebri can be difficult to distinguish from other highly aggressive tumors such as glioblastoma multiforme. Personality and mental status changes are commonly seen, particularly early on in the disease. Symptoms of raised intracranial pressure such as headaches and vomiting may be present. Other symptoms can include lethargy, seizures, visual disturbance, dementia, motor symptoms, and endocrine abnormalities. Prognosis is generally unfavorable with a relatively short survival time. Surgical intervention is usually not an option with this type of tumor and aggressive chemotherapy or radiotherapy has not been shown to dramatically affect survival rates.

Cross References ▶ Astrocytoma ▶ Brain Tumor ▶ Glioblastoma Multiforme ▶ Neoplasms ▶ Tumor Grade

Glioses ▶ Gliosis

Gliosis

M I -Y EOUNG J O Private Practice Los Angeles, CA, USA

J ENNIFER C. G IDLEY L ARSON , YANA S UCHY University of Utah Salt Lake City, UT, USA

Synonyms

Synonyms

Diffuse cerebral gliomatosis; Infiltrative diffuse astrocytosis

Astrocytosis; Glioses

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Definition

Categorization

Gliosis refers to proliferation of glial cells, either by hypertrophy (increased cell size) or hyperplasia (increased cellular division), in response to a CNS insult. For example, gliosis may occur in order to encapsulate a brain tumor, or to provide a scaffolding to support healthy tissue surrounding areas of insult or lesion. In many cases, gliosis leads to scarring within the CNS. Depending on the type of insult, gliosis can affect different glial cell subpopulations, primarily astrocytes and microglia, and to a lesser extent ogligodendrocytes. Gliosis is one of the principal histopathological indicators of brain damage.

Global aphasia is a subtype of nonfluent aphasia.

Cross References ▶ Apoptosis ▶ Neuroglia

References and Readings Croisier, E., & Graeber, M. (2006). Glial degeneration and reactive gliosis in alpha-synucleinopathies: The emerging concept of primary gliodegenration. Acta Neruopathologica, 112, 517–530. Kumar, V., Fausto, N., & Abbas, A. (2004). Robbins & Cotran pathologic basis of disease (7th ed.). Philadelphia, PA: Saunders.

Global Amnesia ▶ Amnestic Disorder

Natural History, Prognostic Factors, and Outcomes Incidence studies suggest that global aphasia may be one of the most common aphasia types (Peach, 2001). Most individuals with global aphasia present with a combination of aphasia, apraxia of speech, and hemiparesis contralateral to the side of lesion, consistent with large lesions of the language-dominant hemisphere. There are cases, however, in which there is no motor involvement and primary motor areas are spared (Bang et al., 2004; Hanlon, Lux, & Dromerick, 1999). In general, the prognosis for recovery of pre-morbid language skills is poor in individuals with global aphasia. This is not to say, however, that individuals with global aphasia do not show meaningful improvement over time. In consecutive studies of patients with stroke, individuals with global aphasia showed significant recovery within the first 2–3 months post-stroke (e.g., Bakheit, Shaw, Carrington, & Griffiths, 2007; Laska, Hellblom, Murray, Kahan, & Von Arbin, 2001), at times exceeding gains by patients with Broca’s or Wernicke’s aphasia. Many individuals with global aphasia make significant gains with therapy aimed at communication via gesture, writing, or drawing. As with all aphasia types, the prognosis for recovery depends on the factors such as the site(s) and size of lesion, comorbid health conditions, and age of the patient. Individuals with global aphasia may evolve to either Broca’s or Wernicke’s aphasia, depending on the site and extent of lesion. The prognosis for recovery of functional communication for these individuals is relatively poor, however, when global aphasia persists beyond the first few months post-stroke.

Global Aphasia LYN T URKSTRA University of Wisconsin-Madison Madison, WI, USA

Short Description Global aphasia is an aphasia type in which there is no functional verbal comprehension or expression, and no use of gestures to represent language.

Neuropsychology and Psychology of Global Aphasia Global aphasia typically is associated with large cortical lesions in the language-dominant hemisphere, often affecting all perisylvian language structures with extension to the insula, basal ganglia, and internal capsule. In patients with vascular lesions, this typically is associated with lesions affecting the entire distribution of the middle cerebral artery. There is, however, great variety in the site and extent of lesions associated with this syndrome and global

Global Versus Local Processing

aphasia has been documented in individuals with lesions restricted to anterior or posterior perisylvian regions (Bang et al., 2004; Hanlon, Lux, & Dromerick, 1999). It is unclear whether individuals with global aphasia lack linguistic representations or are impaired primarily in production (i.e., in access to stored linguistic representations). In some patients, specific aspects of language function appear to be preserved, such as comprehension of specific word categories or sounds. Cognitive function is difficult to assess in individuals with global aphasia, and the results of nonverbal tests have been mixed (Peach, 2001). This likely reflects heterogeneity in lesion characteristics across patients, with greater involvement of non-perisylvian regions in some patients. Frustration and depression are major concerns for individuals with global aphasia, as their comprehension problems often preclude counseling. Patients should be closely monitored for affective and vegetative signs of depression, and pharmacotherapy should be considered as it has the potential to improve engagement with the environment and thus potential to benefit from treatment.

Evaluation As with other aphasia types, individuals with global aphasia typically are evaluated using a combination of standardized language tests and careful observation of extemporaneous communication. Tests such as the Boston Assessment of Severe Aphasia (Helm-Estabrooks, Ramsberger, & Morgan, 1989) were developed specifically for this population and include items designed to elicit automatic responses and minimize reliance on accurate yes/no and pointing responses.

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▶ Speech-Language Therapy ▶ Wernicke–Lichtheim Model of Aphasia

References and Readings Bakheit, A. M. O., Shaw, S., Carrington, S., & Griffiths, S. (2007). The rate and extent of improvement with therapy from the different types of aphasia in the first year after stroke. Clinical Rehabilitation, 21, 941–949. Bang, O. Y., Heo, K. G., Kwak, Y., Lee, P. H., Joo, I. S., & Huh, K. J. (2004). Global aphasia without hemiparesis: Lesion analysis and its mechanism in 11 Korean patients. Neurological Sciences, 217, 101–106. Chapey, R. (Ed.). (2001). Language intervention strategies in aphasia and related neurogenic communication disorders (4th ed.). Philadelphia, PA: Lippincott, Williams & Wilkins. Goodglass, H. (1993). Understanding aphasia. San Diego: Academic Press. Hanlon, R. E., Lux, W. E., & Dromerick, A. W. (1999). Global aphasia without hemiparesis: language profiles and lesion distribution. Journal of Neurology, Neurosurgery and Psychiatry, 66(3), 365–369. Helm-Estabrooks, N., Ramsberger, G., & Morgan, A. (1989). Boston assessment of severe aphasia. Chicago, Illinois: The Riverside Publishing Company. Laska, A. C., Hellblom, A., Murray, V., Kahan, T., & Von Arbin, M. (2001). Aphasia in acute stroke and relation to outcome. Journal of Internal Medicine, 249, 413–422. Peach, R. K. (2001). Clinical intervention for global aphasia. In R. Chapey (Ed.), Language intervention strategies in aphasia and related neurogenic communication disorders (4th ed.). Philadelphia: Lippincott, Williams & Wilkins.

Global Reserve ▶ Brain Reserve Capacity ▶ Cognitive Reserve

Treatment Treatment for individuals with global aphasia typically combines traditional language stimulation with compensatory approaches. The latter include training in the use of iconic sign systems such as Amer-Ind code, meaningful gestures, assistive devices such as picture-based communication boards, and drawing (Peach, 2001).

Global Versus Local Processing M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Definition Cross References ▶ Aphasia Tests ▶ Communication Ability

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Distinction between levels of information processing focusing preferentially on either details of specific information (local) versus the whole (global).

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Current Knowledge The ‘‘global versus local’’ processing dichotomy centers around the view that the brain’s handling of perceptual information differs depending on which hemisphere is predominantly engaged. The left hemisphere is thought to be more adept at processing details (‘‘local’’), while the right is superior in grasping the whole (‘‘global’’). The accompanying figure is representative of one type of stimulus used to study this phenomenon (Fig. 1). Research with brain-injured populations has tended to show differences in performance depending on the localization of the lesion, patients with left-sided lesions being faster in identifying the ‘‘H’’ (global processing of the intact right hemisphere), and those with right hemisphere lesions demonstrating a greater facility in identifying the ‘‘S’’ (local processing by the intact left hemisphere). Variables such as the size and number of the elements in the visual display and exposure time have been shown to influence this effect. Even the mood of the patient may make a difference. Depressed individuals are more likely to process information at the local level, as compared to their nondepressed, globally processing peers. Comparable findings have been reported for visualspatial constructional tasks. Edith Kaplan was one of the first to report qualitative differences in the performance of right versus left hemisphere-damaged patients on block S S S S S S S S S S S S S S S S S S S S S S SSSSSSSSSSSSSSSSSSSSSSS S S S S S S S S S S S S S S S S S S S S

Global Versus Local Processing. Figure 1 Global Versus Local Processing Stimulus: A global processor would see an ‘‘H’’ while a local processor would detect ‘‘multiple Ss’’

construction tasks. When asked to reproduce pattern using nine individual blocks, patients with left-sided lesions were more likely to maintain the 3 3 matrix of the original design, but have difficulty matching and positioning the individual elements within that overall pattern. Conversely, right-lesioned individuals might approximate the internal pattern, while failing to adhere to the 3 3 matrix of the model. Similar problems can often be seen when patients are asked to draw a complex geometric design such as the Rey–Osterrieth figure. Both right and left hemisphere-damaged patients may have considerable difficulty with this task, but for different reasons. In general, patients with left hemisphere lesions are somewhat more likely to capture the general outline (Gestalt) of the design, but struggle with the multiple internal details. Right hemisphere-damaged patient, more often draw the figure in a fragmented, ‘‘piecemeal’’ fashion, resulting in greater distortion of the overall design. While not sufficiently robust for predicting laterality of lesions, when observed, such findings are seen as illustrative of the global versus local processing abilities of the two hemispheres.

Cross References ▶ Asymmetry ▶ Hemispheric Specialization

References and Readings Blanca, M. J., Zalabardo, C., Garcia-Criado, F., & Siles, R. (1994). Hemispheric differences in global and local processing dependent of exposure duration. Neuropsychologia, 32, 1343–1351. Boles, D. B., & Karner, T. A. (1996). Hemispheric differences in global versus local processing: Still unclear. Brain and Cognition, 30(2), 232–243. Delis, D. C., Robertson, L. C., & Efron, R. (1986). Hemispheric specialization of memory for hierarchical visual stimuli. Neuropsychologia, 24(2), 205–214. Gasper, K., & Clore, G. L. (2002). Attending to the big picture: Mood and global versus local processing of visual information. Psychological Science, 13(1), 34–40. Heinze, H. J., Hinrichs, H., Scholz, M., Burchert, W., & Mangun, G. R. (1998). Neural mechanisms of global and local processing: A combined PET and ERP study. Journal of Cognitive Neuroscience, 10(4), 485–498. Van Kleeck, M. H. (1989). Hemispheric differences in global versus local processing of hierarchical visual stimuli by normal subjects: New data and a meta-analysis of previous studies. Neuropsychologia, 27, 1165–1178.

Globus Pallidus

Globus Pallidus S EEMA S HROFF Virginia Commonwealth University Richmond, VA, USA

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Enkephalinergic neurons populate the in the external segment, whereas substance P is predominant in the internal segment. Gamma-amino butyric acid (GABA) is found throughout both segments.

Circuitry and Functions

Definition The Globus Pallidus (pale globe) forms the medial part of the lentiform nucleus.

Nomenclature Striatum/Neostriatum = Caudate Nucleus (nucleus caudatus) þ Putamen Lentiform Nucleus = Putamen þ Globus Pallidus Corpus striatum (Dorsal division) = Caudate Nucleus þ Putamen þ Globus Pallidus

Cortical inputs from areas involved in planning and execution of motor movements project to the striatum (caudate nucleus and Putamen). Striatal neurons relay to the Globus Pallidus, which sends information via the ansa lenticularis and lenticular fasciculus to the thalamus (VA/VL), which in turn feeds back to the motor areas of the cortex. This circuit forms the ‘‘Direct Pathway,’’ which increases motor activity. An indirect pathway, involving the sub thalamic nucleus, that decreases motor activity also exists. The activity of these pathways is further modulated by dopamine from the nigrostrial tract and ACh from the interneurons. The basal ganglia send no direct descending pathways to the spinal cord.

Current Knowledge Anatomy The medial medullary lamina divides the Globus Pallidus into a lateral external segment (GPe) and a medial internal segment (GPi).

Relations The Globus Pallidus is bounded by the Putamen laterally. It is separated from the caudate nucleus by the anterior limb of the internal capsule. The ansa lenticularis runs inferiorly.

Pathophysiology The Globus Pallidus, as a part of the basal ganglia, is involved in motor planning and smooth execution of movements. Athetoid movements (slow, sinous, aimless movements mostly involving distal musculature) can be manifestations of pallidal pathology.

Cross References ▶ Caudate Nucleus ▶ Pallidum ▶ Putamen

Histology The cell density of the Globus Pallidus is 1/20 that of the neostriatum. Although the external segment is larger and has a higher cell density than the internal segment, the morphology of the neurons in both segments is similar. The majority of neurons are large and multipolar, with discoid dendritic arbors.

References and Readings Barr, M., & Kiernan, J. (1983). The human nervous system – An anatomical viewpoint (4th ed.). Harper and Row. Gray, H. (1995). Gray’s anatomy (38th ed.). Pearson Professional Limited.

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Glucocorticoids

Glucocorticoids B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms Adrenal hormones

References and Readings Meaney, M., Aitken, D., Bodnoff, S., Iny, L., Tatarewicz, J., & Sapolsky, R. (1985). Early postnatal handling alters glucocorticoid receptor concentrations in selected brain regions. Behavioral Neuroscience, 99(4), 765–770. Novak, T. A., & Alderson, A. A. (2002). Neurophysiological and clinical aspects of glucocorticoids and memory: A review. Journal of Clinical and Experimental Neuropsychology, 24(3), 335–355. Roozendaal, B., Portillo-Marquez, G., & McGaugh, J. (1996). Basolateral amygdala lesions block glucocorticoid-induced modulation of memory for spatial learning. Behavioral Neuroscience, 110(5), 1074–1083. Smeets, T., Jelicic, M., Merckelbach, H., Peters, M., Fett, A., Taverniers, J., et al. (2006). Enhanced memory performance on an internalinternal source monitoring test following acute psychosocial stress. Behavioral Neuroscience, 120(6), 1204–1210.

Definition Glucocorticoids are hormones produced in the cortex of the adrenal gland. They bind to the glucocorticoid receptor, which is found on almost every vertebrate animal cell. They are classified as steroids and one example is cortisol. This hormone is involved in carbohydrate metabolism and immune function. Metabolically, glucocorticoids are mainly involved in gluconeogenesis (glucose synthesis). For immune function, glucocorticoids are often used to dampen or halt inflammation via their negative feedback in the stress response pathway. Examples of synthetic glucocorticoids are hydrocortisone and prednisone. Glucocorticoids treatment is utilized for a wide variety of issues including asthma, allergies, orthopedics, and ongoing pain management, but has the potential for serious side effects including depression and suicidality. Such treatment could impact results of neuropsychological testing by artificially escalating depression scores. Additionally, it has been shown that treatment with glucocorticoids impacts the hippocampus, amygdala, and frontal lobes increasing episodic emotionallybased memories. Further, increased glucocorticoid levels improve the formation of long-term memory of emotionally-triggered actions or events, while actually inhibiting memory of non-emotionally related actions or events.

Cross References ▶ Hormones ▶ Steroids

Glutamate M ARLA S ANZONE Independent Practice Annapolis, MD, USA

Synonyms Glutamic acid; L-Glutamic acid; Monosodium glutamate (MSG)

Indications Glutamate is the carboxylate anion and salt of glutamic acid (abbreviated as Glu or E), a nonessential amino acid with GAA and GAG. Peter Usherwood identified the chemical as a neurotransmitter in 1994, but Kikunae Ikeda of Tokay Imperial University discovered glutamate in 1907 while looking for the flavor common to particular foods. He later invented monosodium glutamate (Boeree, 2003). It is one of the 20 proteinogenic amino acids, synthesized by protein hydrolysis, and is stored in synaptic vesicles. It is the only amino acid metabolized by the brain and the most abundant excitatory neurotransmitter in the human nervous system, particularly in the cerebellum and spinal cord (Glutamate, 2008, 2009c; Metabolomics Toolbox, 2009). Presynaptic chemical impulses trigger glutamate release. It binds to and activates postsynaptic effector neurons such as NMDA receptors. When in the synapse, glutamate is either reabsorbed by an ion-exchange

Glutamate

transport system or binds to astrocyte glial cells that convert it to the non-excitotoxic amino acid, glutamine. Via this glutamate–glutamine cycle, glutamine is then transported back into neurons and stored in vesicular glutamate transporters (VgluTs) of which there are three types, for reconversion into glutamate. Glutamate transporters, also known as excitatory amino acid transporters (EAATs), are neuronal and glial membrane-bound pumps similar to ion channels also found in bone and other tissues. They trigger biochemical cascades that remove glutamate from the synapse, effectively terminating the excitatory signal transduction. The accumulation of excessive amounts of glutamate in the extracellular space leads to an influx of calcium ions causing excitotoxicity, neuronal damage, and cell death. There are two classes of glutamate transporters, the EAATs, whose function depends on an electrochemical sodium and potassium gradient with a high affinity for glutamate, and those dependent on a adenosine triphosphate (ATP) hydrolyzed proton gradient, the vesicular glutamate transporters (VgluTs) with minimal affinity for glutamate compared to the EAATs (Glutamate transporter, 2009). Three subtypes of VGLUTs and five subtypes of EAAT have been identified in humans. Only EAAT subtype 4 is found in neurons. Subtypes 1, 2, and 3 are found in astrocytes, endothelial cells, microglia, and oligodendrocytes. Subtype 5 is found only in the photoreceptors and bipolar neurons of the retina (Augustin et al., 2007; Glutamate transporter, 2009; Shigeri et al., 2004). Dysregulation of EAATs has been implicated in lateral sclerosis (ALS), Alzheimer’s disease, cerebral stroke, epilepsy, HIV-associated dementia, Huntington’s disease, amyotrophic, and malignant glioma. The manifestation of schizophrenia is thought to be relevant to disruption in VGLUTs regulation (Shigeri et al., 2004).

Mechanism of Action Understanding of the role of glutamate metabolism in normal and disease physiology is limited. It is known to play key roles in cellular metabolism and excess nitrogen disposal. Glutamate is also thought to be the primary neurotransmitter responsible for memory storage at excitatory synapses and most modifiable synapses, those critical to synaptic plasticity (Zheng et al., 2002). For this reason, glutamate is believed to have a central role in cognitive functions, particularly learning and memory (Venes, Thomas, Egan, & Houska, 2001; Glutamate, 2008,

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2009c). Excess levels of glutamate can be toxic causing elevated levels of free radicals, nitric oxide (NO), intracellular calcium (Ca), and proteinase activity. Dysregulated levels and functioning of these molecules have been implicated in numerous psychiatric and neurological disorders (Beers et al., 2006).

Specific Compounds and Properties Glutamate influences numerous brain functions, including consciousness, learning, memory, mood, motor control, and sensory perception such as mediating hyperalgesia. The cellular mechanisms associated with learning and memory are thought to be related to the strength of connectivity at glutamatergic synapses. These connections enable the critical levels glutamatergic feedback that transfer information into short- and long-term potentiated circuits. The dysregulation of glutamate resulting in sickle-cell anemia originate at the level of mutation in the DNA structure of hemoglobin molecule. Glutamic acid is replaced by valine in the β-globin chain at position 6 of hemoglobin. This causes the variant whereby the shape of the red blood cell becomes prone to distortion when it is deoxygenated. When the misshapen erythrocyte is removed by the spleen, its sickle shape causes microvascular obstruction.

Clinical Use Glutamate’s role in neurologic damage, such as stroke and the neurodegenerative disorders, AIDS-dementia complex, motor neuron disease, and Alzheimer’s and Parkinson’s diseases, is thought to be a function of receptor-mediated toxicity. During traumatic brain injury, seizures, or cerebral stroke, a neurotoxic accumulation of glutamate results from dysfunction of the glutamate transporters. Insufficient ATP needed to activate the electrochemical ion gradients or overactivation of the glutamate receptors are two methods by which glutamate metabolism is reverse or disrupted leading to excitotoxicity (Beers et al., 2006; Glutamate, 2009c; Kaufnman, 2007). Insufficient removal of glutamate resulting from ischemic cascades is thought to contribute to the development of ALS (amyotrophic lateral sclerosis), autism, Alzheimer’s disease, and stroke. Changes in the resting membrane potential causing spontaneous opening of voltage-gated calcium channels, atypical electrical firing patterns,

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increased glutamic acid release, and depolarization are suspected in seizure activity. The onset and progression of neurodegenerative conditions such as Huntington’s disease, Parkinsonian dementia complex, ALS, and Alzheimer’s disease have been linked to the loss of a sodium-dependent glutamate transporter and the degeneration of motor neurons in the cerebellum and spinal cords. Overly active glutamate transporters, on the other hand, are thought to be implicated in inadequate synaptic glutamate transmission, possibly resulting in psychiatric disorders such as neurocognitive disorders, depressive symptomology, anxiety disorders and schizophrenia (Augustin et al., 2007; Glutamate, 2009c; Glutamate transporter, 2009; Kaufnman, 2007). Glutamate receptors are classified as either NMDA- or non-NMDA receptors. The development of effective pharmaceuticals aimed at effectively mitigating glutamate excitotoxicity has been met with minimal success. NMDA-receptor binding drugs include PCP (phencyclidine), and memantine used to treat Alzheimer’s disease.

Cross References ▶ Alzheimer’s Disease ▶ GABA ▶ Hormones ▶ Huntington’s Disease ▶ Neuropeptides ▶ Neurotoxins ▶ Neurotransmitters ▶ Parkinson’s Dementia ▶ Parkinson’s Disease ▶ Taste

References and Readings Augustin, H., Grosjean, Y., Chen, K., Sheng, Q., & Featherstone, D. E. (2007). Nonvesicular release of glutamate by glial xCT transporters suppresses glutamate receptor clustering in vivo. Journal of Neuroscience, 27(1), 111–123. Beers, M. H., Porter, R. S., Jones, T. V., Kaplan, J. L., & Berkwits, M. (2006). Glutamate. In M. H. Beers et al. (Eds.), The Merck manual 18th edition (pp. 1760–1761). Whitehouse Station: Merck Research Laboratories. Boeree, C. G. (2003). In General Psychology: Neurotransmitters, Retrieved January 19, 2009, from http://webspace.ship.edu/cgboer/ genpsyneurotransmitters.html. Deamination. (2009). In Wikipedia. Retrieved January 20, 2009, from http://en.wikipedia.org/wiki/Deamination. Glutamate. (2008). In Merriam-Webster Online Dictionary. Retrieved January 13, 2009, from http://www.merriam-webster.com/ dictionary.

Glutamate. (2009a). In Free Online Thesaurus, Retrieved January 13, 2009, from http://freethesaurus.net. Glutamate. (2009b). In MedicineNet. Retrieved January 13, 2009, from http://www.medterms.com. Glutamate. (2009c). In RightHealth.net. Retrieved January 17, 2009, from http://www.righthealth.com/Health/Glutamate. Glutamate transporter. (2009). In Wikipedia. Retrieved January 20, 2009, from http://en.wikipedia.org/wiki/Glutamate_transporter. Kaufnman, D. M. (2007). Clinical neurology for psychiatrists. (6th ed., pp. 72, 123–124, 519–520). Philadelphia, PA: Saunders Elsevier. Metabolomics Toolbox. (2009). In Human Metabolome Database. Retrieved January 19, 2009, from http://hmdb.ca/scripts/show_card. cgi?METABOCARD = HMDB00216.txt. Myers, T. (2006). Glutamate (definition). In T. Myers (Ed.), Mosby’s dictionary of medicine, nursing & health professions (7th ed., p. 861). Missouri: Mosby Elsevier. Nicoll, R. A. (2004). Introduction to pharmacology of CNS drugs. In B. Katzung (Ed.), Basic and clinical pharmacology (9th ed., pp. 336–350). New York: Lange Medical Books/McGraw-Hill. Shigeri, Y., Seal, R. P., & Shimamoto, K. (2004). Molecular pharmacology of glutamate transporters, EAATs and VGLUTs. Brain Research Reviews, 45(3), 250–265. Venes, D., Thomas, C., Egan, E., & Houska, A. (2001). Glutamate (definition). In D. Venes et al. (Eds.), Taber’s medical dictionary (19th ed., pp. 888). Philadelphia, PA: FA Davis Company. Zheng, X., Baker, D. A., Shen, H., Carson, D. S., & Kalivas, P. W. (2002). Group II metabotropic glutamate receptors modulate extracellular glutamate in the nucleus accumbens. Journal of Pharmacology and Experimental Therapeutics, 300(1), 162–171.

Glutamic Acid ▶ Glutamate

Go/No-Go Testing G RANT L. I VERSON University of British Columbia & British Columbia Mental Health & Addictions Vancouver, BC, Canada

Definition Go/No-Go testing is often used as a component of a behavioral neurological examination to assess inhibitory control. A classic example is to hold out two fingers, the index and middle fingers (palm down), and say to the examinee: ‘‘When I do this (showing two fingers in the form of a ‘‘V’’), you do this (showing only the index finger), and when I do this (showing the index finger

Goldman-Rakic, Patricia (1937–2003)

only), you do this (showing two fingers in the form of a ‘‘V’’).’’ The examinee first learns the pattern of alternating between sticking out one finger (index) or two fingers (‘‘V’’) against the responses of the examiner. This primes the examinee into a set. After the examinee demonstrates several consecutive correct responses, the rules are then changed: ‘‘Now, I am going to change the rules. When I do this (showing two fingers in the form of a ‘‘V’’), you do this (showing one finger), and when I do this (showing one finger), you do nothing.’’ This second task is the actual Go/No-Go Testing.

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GOAT ▶ Galveston Orientation and Amnesia Test

Goitrous Hypothyroidism ▶ Hypothyroidism

G Current Knowledge The inhibitory control of behavior is believed to be an integrative function of the prefrontal cortex. Inhibitory control protects behavior, speech, and thought from internal or external factors that might interfere with or conflict with the behavior, or lead the behavior astray (Fuster, 1997; page 236). Inhibitory control is believed to be mediated by the orbital–medial aspects of the frontal lobe, and can be adversely affected by brain injury or disease. The Go/No-Go test is a simple, bedside neurological test for assessing inhibitory control. The paradigm, however, has been adapted for research with animals and humans in a variety of contexts, ranging from developmental neurobiology to functional neuroimaging.

Goldman-Rakic, Patricia (1937–2003) RUSSELL M. B AUER University of Florida Gainesville, FL, USA


Cross References ▶ Perseveration ▶ Stimulus-Bound Behavior

References and Readings Dubois, B., Slachevsky, A., Litvan, I., & Pillon, B. (2000). The FAB: A frontal assessment battery at bedside. Neurology, 55, 1621–1626. Fuster, J. M. (1997). The prefrontal cortex: Anatomy, physiology, and neuropsychology of the frontal lobe (3rd ed.). Philadelphia: Lippincott-Raven. Jurado, M. B., & Rosselli, M. (2007). The elusive nature of executive functions: A review of our current understanding. Neuropsychological Review, 17, 213–233. Luria, A. R. (1973). The working brain: An introduction to neuropsychology (Haig, Trans.). New York: Basic Books. Stuss, D. T., & Levine, B. (2002). Adult clinical neuropsychology: Lessons from studies of the frontal lobes. Annual Review of Psychology, 53, 401–433.

Originally trained as a psychologist, Patricia Goldman-Rakic is universally recognized as one of the most prolific and creative neuroscientists of her generation, having authored or coauthored over 300 publications and coauthored three books on topics ranging from the ability of the brain to compensate for early injury to the development of a seminal model of working memory function that continues to stimulate leading-edge research in cognitive neuroscience and neuropsychology. She was a pioneer in crossplatform methodology – the use of multilevel techniques to fully elucidate neural processes and mechanisms. Her research program utilized both structural and neurochemical lesion methods, electrophysiology, immunocytochemistry, receptor autoradiography, and sophisticated behavioral methods. Early in her career, Goldman-Rakic’s work on the cognitive effects of pre- and postnatal cortical lesions in infant rhesus monkeys (Macaca mullata) showed that the effects of early brain injury were not only dependent on which cortical regions were damaged, but were also influenced by developmental stage and gender. This research also revealed that callosal connections, as well as connectivity within the visual, motor, and higher-level association cortices of the brain develop by midgestation and are present at

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birth in nonhuman primates. These anatomical and behavioral studies represented foundational contributions for our understanding of normal and abnormal cognitive development and have yielded insights into developmental neurological and psychiatric disorders, including schizophrenia. In the 1990s, Goldman-Rakic embarked on a program of research using single-cell recordings in the prefrontal cortex of the awake monkey during as it performed delayed-response tasks. In this research, she and her colleagues discovered distinct populations of neurons that showed sustained activity during the delay period, and were able to show, in a series of elegant and methodologically sophisticated studies, that this neuronal activity was the neural code for working memory. Through this work, we learned that individual neurons within this population can exhibit remarkable specificity. For example, those above the principal sulcus (PS) were preferentially involved in coding the impending spatial location of a required response, while those below the PS showed object-dependency. In this way, she was able to show that the prefrontal cortex contained memory fields that appeared organized as modules and columns that seemed to obey the distinction between object and spatial processing that had been demonstrated in posterior cortex. This modular view of working memory processes was truly revolutionary in that it drew attention to memory networks of the brain outside the classical memory areas of the medial temporal lobe memory system. Goldman-Rakic’s behavioral, anatomical, and physiological mapping of the human working memory system was critically important in its own right, but she was able to see more broadly the implications of her findings for understanding complex brain disorders. Her contribution to contemporary understanding of schizophrenia is a case in point. She believed that a fundamental deficit in working memory, and an inability to retrieve and hold ‘‘in mind’’ appropriately stored symbolic representations, was critical to the production of disordered thought processes in schizophrenia. She was able to show that schizophrenic patients had fundamental deficits in working memory processes, and was able to link these deficits to the dynamics of dopamine-containing cells in prefrontal cortex using both pharmacological and neurochemical lesion methods. Using these basic facts as a starting point, Goldman-Rakic proposed a broad research agenda for researchers in the neurobiology of schizophrenia, calling for fundamental research that could

lead to a more comprehensive understanding of neurodevelopmental, anatomical, and neurochemical factors that modulate neuronal signaling and activity within the prefrontal cortex and other cortical and subcortical systems to which it is connected. Goldman-Rakic founded the journal Cerebral Cortex, and served on editorial boards for the journals Science, Advances in Neuroscience, Journal of Neuroscience, Behavioral Brain Research, Concepts in Neuroscience, Biological Psychiatry, and many others. She also held numerous advisory positions at the National Institutes of Health, the National Institute of Mental Health, the Society for Neuroscience, and many other institutions and organizations. She was aggressively pursued as an invited lecturer at universities, research centers, conferences, and symposia around the world and was known for her gracious acceptance of many such invitations, often spending extra time and effort to interact with students. Some eminent scientists achieve their ends by adopting a competitive approach in dealing with rival views or personalities. This was not Patricia GoldmanRakic’s way, though to be sure she was passionate about her subject matter and her approach to understanding it. The impact of her discoveries was forceful, but she herself was gentle and kind. While her literature contributions are formidable and numerous, her ultimate role in the advancement of contemporary neuroscience is as a visionary, as a rare scientist whose beautiful mind was capable of synthesizing information from diverse literatures and levels of analysis so as to reveal underlying patterns of function, structure, and organization. She was universally regarded as a superb mentor, a consummate colleague, and a passionate scientist whose contributions will continue to be influential not only because of their methodological and conceptual rigor, but also for the manner in which they addressed fundamental questions and critically important problems in contemporary neuroscience.


B.A. summa cum laude in Neurobiology, Vassar College, 1959 Ph.D., Experimental /Developmental Psychology, University of California, Los Angeles, 1963 Honorary Doctorates from Utrecht University, Netherlands (2000), University of St. Andrews, Scotland (2003)


Major Appointments

Head of the Section of Developmental Neurobiology, National Institutes of Mental Health (1975–1979) Yale University, Jointly appointed (Departments of Psychiatry, Neurology, and Psychology; 1979–2003) Yale University School of Medicine, Director of Graduate Studies, Section of Neuroanatomy (1981–1986) Acting Chair, Section of Neurobiology (1986–1987) Chair, Biological Sciences Advisory Committee (1993–1996)


National Institute of Mental Health grantee (1980– 2000) Alden Spencer Award, Columbia University (1982) Krieg Cortical Discoverer Award, Cajal Club (1989) President, Society for Neuroscience (1989–1990) Inductee, National Academy of Sciences (1990) Fyssen Foundation Prize in Neuroscience (1990) American Psychological Association, Distinguished Scientific Contribution Award (1991) Lieber Prize, National Alliance for Research in Schizophrenia and Depression (NARSAD; 1991) Robert J. and Clair Pasarow Foundation Award (1993) Karl Lashley Award, American Philosophical Society (1996) Honorary Doctorate, Utrecht University (2000) Gerard Prize, Society for Neuroscience (2002) Honorary Doctorate, University of St. Andrews, Scotland (2003) Inductee, Connecticut Women’s Hall of Fame (2008)

Short Biography Patricia Schoer was born in Salem, Massachusetts on April 22, 1937, along with her twin, Ruth. Patricia, Ruth, and sister Linda (5 years younger than Patricia and Ruth), were daughters of Irvin Schoer and Jenine Pearl. Irvin Schoer was a son of Latvian immigrants and Jenine Pearl was of Russian descent. All three sisters graduated from Peabody High School, each winning the coveted George Peabody Medal, and all three eventually obtained doctorates in science. Goldman-Rakic attended Vassar College, where she obtained a B.A. summa cum laude in Neurobiology in 1959. She then went to UCLA, where she obtained a Ph.D. in Experimental/Developmental Psychology in 1963. Her

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dissertation evaluated the effects of stress and handling on cognitive development in the rat, one of her first forays into developmental neurobiology. After postdoctoral training experiences at New York University and the Massachusetts Institute of Technology, she became a postdoctoral fellow at the National Institutes of Health in Bethesda, MD in the late 1960s, where she initially worked with Enger Rosvold. This work first introduced her to the complexities of the frontal lobe. All in all, she spent 14 years at NIH, eventually ascending to Head of the Section of Neurobiology. In 1974, she began a brief tenure as a Visiting Scientist at MIT, where she worked with Walle Nauta, a renowned anatomist. While in Boston, she met her eventual husband, Pasko Rakic, who was on the faculty at Harvard, and married him in 1979 after maintaining a 3-year long-distance relationship. At that point, she and Pasko moved to Yale, where she took an appointment in the Department of Neurobiology where she remained until her death. She enjoyed an illustrious and productive career at Yale, receiving numerous awards and two honorary doctorate degrees from European Universities. During her tenure at Yale, she served not only her university community, but also the field at large, authoring critical and influential papers, serving on administrative and editorial boards, and providing invited lectures throughout the world. On July 29, 2003, she was struck by a car as she crossed a busy street in Hamden, Connecticut. She died 2 days later at Yale–New Haven Hospital at the age of 66. She is buried in Grove Street Cemetery.

Cross References ▶ Frontal Lobe ▶ Schizotypal Personality Disorder ▶ Working Memory

References and Readings Goldman-Rakic, P. S. (1980). Morphological consequences of prenatal injury to the primate brain. Progress in Brain Research, 53, 1–19. Goldman-Rakic, P. S. (1988). Topography of cognition: Parallel distributed networks in primate association cortex. Annual Review of Neuroscience, 11, 137–156. Goldman-Rakic, P. S. (1990). Cellular and circuit basis of working memory in prefrontal cortex of nonhuman primates. Progress in Brain Research, 85, 325–335. Goldman-Rakic, P. S. (1994). Working memory dysfunction in schizophrenia. Journal of Neuropsychiatry and Clinical Neuroscience, 6, 348–357. Goldman-Rakic, P. S. (1995). Cellular basis of working memory. Neuron, 14, 477–485.

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Goldman-Rakic, P. S. (1996a). Memory: Recording experience in cells and circuits: Diversity in memory research. Proceedings of the National Academy of Sciences USA, 93, 13435–13437. Goldman-Rakic, P. S. (1996b). Regional and cellular fractionation of working memory. Proceedings of the National Academy of Sciences USA, 93, 13473–13480. Goldman-Rakic, P. S. (1998). The cortical dopamine system: Role in memory and cognition. Advances in Pharmacology, 42, 707–711. Goldman-Rakic, P. S. (1999). The physiological approach: Functional architecture of working memory and disordered cognition in schizophrenia. Biological Psychiatry, 46, 650–651. Goldman-Rakic, P. S., & Selemon, L. D. (1990). New frontiers in basal ganglia research. Introduction. Trends in Neuroscience, 13, 241–244. Goldman-Rakic, P. S., & Selemon, L. D. (1997). Functional and anatomical aspects of prefrontal pathology in schizophrenia. Schizophrenia Bulletin, 23, 437–458. (50th most cited paper in history of Schizophrenia Bulletin) Goldman-Rakic, P. S., Lidow, M. S., Smiley, J. F., & Williams, M. S. (1992). The anatomy of dopamine in monkey and human prefrontal cortex. Journal of Neural Transmission (Supplement), 36, 163–177. Levy, R., & Goldman-Rakic, P. S. (1999). Association of storage and processing functions in the dorsolateral prefrontal cortex of the nonhuman primate. Journal of Neuroscience, 19, 5149–5158. Levy, R., & Goldman-Rakic, P. S. (2000). Segregation of working memory functions within the dorsolateral prefrontal cortex. Experimental Brain Research, 133, 23–32.


William James Lectures at Harvard University (1938/1939) Doctor Honoris Causa (University of Frankfurt, 1958)


Kurt Goldstein was a prominent neurologist who challenged prevailing theories of localization of function and advocated a holistic, associationist view of the brain. He developed the theory of the ‘‘catastrophic reaction,’’ which emphasized an individual’s awareness of, and reaction to, his or her deficits. His research spanned numerous domains of neuropsychology, but he is perhaps best known for his work on aphasia and for being one of the first neuropsychologists to practice rehabilitation.

Short Biography

Goldstein, Kurt (1878–1965) J OHN RYAN 1, T RICIA Z. K ING 2 1 University of Pittsburgh Pittsburgh, PA, USA 2 Georgia State University Atlanta, GA, USA

Education and Training University of Breslau (M.D., 1903)

Major Appointments

University of Koningsberg (1906–1914) Institute for Research into the Consequences of Brain Injuries (University of Frankfurt, 1916–1930) Professor of Neurology (University of Frankfurt, 1919–1930) Professor of Neurology (University of Berlin, 1930–1933) Professor of Neurology (Columbia University, New York, 1935–1940) Professor of Neurology (Tufts University, Medford, MA, 1940–1945)

Kurt Goldstein was born in 1878 in Katowice, Upper Silesia, into a large Jewish family. He first studied philosophy at the University of Heidelberg, before going to the University of Breslau and choosing medicine as his profession. Goldstein was most intrigued by anatomy and physiology, but while working with his advisor, Carl Wernicke, he became increasingly interested in how biology and psychology are interrelated. Goldstein completed his M.D. in 1903 with a dissertation that focused on the organization of the posterior columns of the spinal cord. Goldstein served as the first assistant to Dr. Ludwig Edinger in a comparative anatomy lab and was able to start his own small clinic, The Institute for Research into the Consequences of Brain Injuries. After Edinger’s death in 1919, Goldstein became a professor of neurology at the University of Frankfurt where he remained until 1930. At the Institute, following World War I, Goldstein was presented with thousands of cases of traumatic brain injury upon which he built his theories of brain–behavior relations. During his time at the Institute, his work spanned a variety of topics in neurology and psychiatry as he attempted to link clinical symptoms to postmortem findings. From early in his career, he believed that the modular approach of viewing clinical syndromes as isolated deficits fell short of the true gestalt of an individual’s psyche. This focus on a holistic assessment of functioning was very much present in the zeitgeist of early twentieth-century


Germany. Goldstein was strongly influenced by Johann Wolfgang von Goethe who advocated that science should not only attempt reductionist analyses of the world, but must also synthesize findings to achieve knowledge. Goldstein applied this view to the brain, similar to the earlier scientist Constanin von Monakow, arguing that science cannot localize function to particular regions in the brain by only studying localized deficits. Rather, by examining performance of a wide array of behaviors (including tasks on which performance is normal), the scientist can localize the symptoms. This idea was presented in a publication in 1925, Das Symptom, seine Entstehung und Bedeutung, which Luria later described as ‘‘the start of neuropsychology.’’ A portrait of Goethe hung over Goldstein’s desk for the duration of his career. At a time when many researchers were focusing on localization of brain function, Goldstein emphasized a global association model. In this model, functions were the result of activity in a large network. Goldstein believed this accounted for the ability of individuals to recover some degree of function following brain injury. Key to Goldstein’s philosophy was the idea that the patient’s symptoms are ‘‘solutions’’ that the patient’s nervous system has devised to interact with his or her environment despite their decreased functioning. Goldstein saw evidence for this viewpoint in his numerous studies of aphasia. Often, patients would present with an inability to find words but could still express themselves, sometimes through circumlocutions. Furthermore, Goldstein was one of the first to devise therapeutic interventions for patients who had acquired brain injury. Neurology in the early twentieth century was primarily focused on attempting to achieve an accurate diagnosis, but the prevailing thought was that the consequences of brain injury were permanent and irreversible. Goldstein did not accept this state of affairs, and began developing methods to help patients recover some degree of function. During his time at the Institute, Goldstein came to know and collaborate with the Gestalt psychologist Adhe´mar Gelb, a student of Carl Stumpf. The collaboration between Goldstein and Gelb was particularly close and productive. Gelb, being an experimental psychologist, helped Goldstein refine his ideas about perception and language while Goldstein offered a neurological perspective to Gelb’s Gestalt views. Although Goldstein himself never formally identified with the Gestalt movement, he was closely affiliated with them, even signing on as an editor of the preeminent Gestalt journal, Psychologische Forschung, when it was founded in 1922. The two men remained intensely close until Gelb’s unexpected death in

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1935, after which Goldstein kept a picture of Gelb over his desk. In 1930, a new department of neurology was opened in Berlin and Goldstein left Frankfurt to become the director. Following the Nazi’s ascent to power, in 1933 Goldstein was anonymously reported by a colleague for having leftist sympathies and was one of the first professors arrested. He was locked in a basement for a week and repeatedly beaten while rumors of his death circulated in academic circles. Ultimately, Eva Rothmann (who later became his wife) appealed to Hermann Goering, a Nazi cabinet member and later commander of the Luftwaffe, who secured the release of Goldstein on the condition that he would immediately leave the country and never return. During a year in exile in Amsterdam, receiving financial support from the Rockefeller Foundation, he integrated his theories of biology and holistic psychiatry culminating in his masterwork, The Organism. The entire work was dictated in 5 weeks. Upon publication, the work was widely distributed and was soon translated into numerous languages. In The Organism, Goldstein presented his holistic approach to neuropsychological assessment. Using an analogy of a physician attempting to help a patient survive despite a significant illness, Goldstein argued that in order to help a neuropsychological patient, the entirety of the individual and their experience must be taken into account – the organism as a whole. Arguing against the atomistic view of the localizationists, Goldstein noted that the central nervous system is a network of nervous tissue, and no subregion of the brain acts in seclusion; rather, every region must be considered as part of a network. Any change in activity in one region of the network will necessarily alter the state of the overall network and result in particular symptoms that may or may not be localized to the region of damage. The method of considering not only the symptoms, but also the social and physical environment in which the patient lives (what Goldstein termed a ‘‘sphere of immediacy’’) was a significant advance in neuropsychology. Healthy functioning, Goldstein argued, was an ability to achieve a state of ‘‘adequacy’’ – a necessary condition for self-realization. When interacting with the environment, ideally the organism can perform sets of ‘‘preferred behaviors’’ which achieve goals and move us toward adequacy. However, inured patients become unable to interact with the environment as before and therefore must devise new solutions to achieve the selfrealized state. Goldstein saw this discrepancy between ability and adequacy as an opportunity for therapeutic intervention.

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Expanding this remarkable view of how to approach neuropsychological injury, Goldstein warned against the influence of subjective theories of the scientist clouding judgment when attempting to empirically examine a case. Goldstein proposed several methodological postulates, which he felt were critical when attempting to interpret any constellation of symptoms. The common factor to each postulate is the scientist’s attempt to remain wholly objective. For example, the clinician ought not to have a theory of the cause of the symptoms before the examination lest this bias observation and cause the observer to overlook potentially important clues to the dysfunction. Goldstein also advocated for a wide-ranging examination of skills of the patient in an attempt to achieve a holistic picture of the nature of the disorder. In his autobiography, Goldstein recounts a case of a woman who could not name an umbrella by sight, but later in discussion casually mentioned that she owned three umbrellas at home. Goldstein noted that had the examination ended prior to this important observation, valuable information would have been missed. Following his exile to Amsterdam, Goldstein settled in New York in 1935 where he worked at the New York State Psychiatric Institute and was appointed professor of Neurology at Columbia University. During a lecture there, Goldstein presented his view that sorting and categorizing are functions unique to humans. Attending one of the lectures was a postdoctoral researcher, Harry Harlow, who became determined to show that nonhuman primates also could perform sorting tasks. This research on nonhuman primates came full circle in Wisconsin where the primate research was applied to developing a task for humans – the Wisconsin Card Sort Task. In 1938–1939 Goldstein was invited to Harvard University to give the William James lectures, which were later published under the title Human Nature in the Light of Psychopathology. At the time, the lectures were reportedly not well received, but offered an overview of his theories on the function of the mind and how it relates to the brain. In 1940, Goldstein received support from the Rockefeller Foundation and worked as a clinical professor of neurology at Tufts University. In 1941, Goldstein coauthored a paper with Martin Scheerer outlining his views on what he termed ‘‘concrete behaviors’’ and ‘‘abstract attitudes.’’ One of Goldstein’s overarching goals was to find commonalities across all neuropsychological disorders. He believed concrete behaviors and abstract attitudes could account for disorders of functioning across a wide spectrum of diseases. Abstract attitudes are the hallmark of healthy psychological functioning. These include functions such as self-reflection, the ability to

form mental sets and categories (a critical skill needed to perform well on sorting tasks), and the ability to make inferences and abstractions. Concrete behaviors are more automatic, less reflective, and rely very little on conscious awareness. Goldstein proposed that healthy psychological functioning relies on both abstract attitudes and concrete behaviors, but if structural brain injury occurs, the patient regresses and relies more on concrete behaviors. If the patient becomes aware of this decrease in functioning, Goldstein argued, they will develop a feeling of failure, a condition he termed the ‘‘catastrophic reaction.’’ The therapeutic interventions Goldstein developed were designed to move away from the catastrophic reaction and the anxiety that could provoke it by encouraging patients to find ways to function in their environment and move toward self-actualization. When funding expired in 1945, Goldstein was 67 years old but continued a private practice, taught at City College and in his late seventies commuted once a week to Brandeis University. In 1948 Goldstein published Language and Language Disturbances, a text that summarized his views on aphasia from over 30 years of clinical work. Aphasia, to Goldstein, was not merely a disorder of language. He felt that language, being a uniquely human affair, represented man’s ability to hold abstract attitudes. Goldstein was against the splitting of different types of aphasia (and for that matter, many of the other neuropsychological acquired deficits) when trying to determine the true nature of the mind. These basic disorders represented only concrete behaviors, the regression of the individual away from a prior state of adequacy. The end of his life was marked by significant recognition by his peers, but also great sadness. For his 80th birthday, numerous publications were dedicated to him and he was awarded an honorary doctorate from the University of Frankfurt. Unfortunately, his wife, Eva Rothmann-Goldstein, suffered from severe depression that ended with her suicide in 1960. Although Goldstein had lived in the US since the 1930s and become a US citizen in 1940, he never felt fully integrated into American culture and often missed his life in Europe. In 1965, Kurt Goldstein had a stroke that resulted in severe aphasia and right-sided paralysis. He died 3 weeks later at 86 years of age. Although it is difficult to assign Goldstein’s name to one particular finding, his legacy is most marked by his insistence on a comprehensive examination of a patient’s life when attempting to diagnose symptoms and devise a clinical plan. Professionally, Goldstein was active in both neurological and psychological spheres. Goldstein served as a founding editor of Psychologische Forschung, and published more than 200 articles and books. He also

Gollin Figures

helped found the International Society for Psychotherapy, and his views on holistic approaches to psychology had a significant impact on countless psychologists, including A.R. Luria, Abraham Maslow, Erich Fromm, Rollo May, and the development of psychosomatic medicine in the US.

Cross References ▶ Luria, Alexander Romanovich (1902–1977) ▶ Karl Spencer Lashley (1890–1958) ▶ Wernicke, Karl (1848–1905)

Reference and Readings Denny-Brown, D. (1966). The organismic (holistic) approach: The neurological impact of Kurt Goldstein. Neuropsychologia, 4, 293–297. Goldstein, K. (1934/1955). The organism: A holistic approach to biology derived from pathological data in man. New York: Zone Books. Goldstein, K. (1940). Human nature in the light of psychopathology. Cambridge, MA: Harvard Press. Goldstein, K. (1948). Language and language disturbance. New York: Grune & Stratton. Goldstein, K. (1954). The concept of health, disease, and therapy: Basic ideas for an organismic psychotherapy. American Journal of Psychotherapy, 8, 745–764. Goldstein, K. (1967). Autobiography. In E. G. Boring & G. Lindzey (Eds.), A history of psychology in autobiography (Vol. 5, pp. 145–166). New York: Appleton-Century-Crofts. Goldstein, K., & Scheerer, M. (1941). Abstract and Concrete Behavior: An Experimental Study With Special Tests. In J. F. Dashell (Ed.), Psychological Monographs, (Vol. 53/1941, No. 2 whole No. 239), S. 1–151. Luria, A. R. (1966). Kurt Goldstein and neuropsychology. Neuropsychologia, 4, 311–313. Pickren, W. E. (2003). Kurt Goldstein: Clinician and philosopher of human nature. In G. A. Kimble & M. Wertheimer (Eds.), Portraits of pioneers in psychology (Vol. 5). Mahwah, NJ: Lawrence Erlbaum Associates. Simmel, M. L. (1968). The reach of mind: Essays in memory of Kurt Goldstein. New York: Springer. Teuber, H. L. (1966). Kurt Goldstein’s role in the development of neuropsychology. Neuropsychologia, 4, 299–310.

Gollin Figures M ELISSA B UTTARO Brown University Providence, RI, USA

Synonyms Gollin incomplete figures test

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Description The Gollin Figures (Gollin, 1960) is a test of incomplete drawings that assesses visual perceptual functions. The original Gollin Incomplete Figures Test consists of 20 sets of pictures of familiar objects or animals (e.g., an umbrella and a bear). Each picture set contains five line drawings that are progressively less degraded, ranging from a bare sketch (Set I) to a complete drawing (Set V) (Lezak, 1995). The examinee must extract distinctive features and mentally ‘‘fill in’’ the missing visual information to guess what the object might be. A later, computerized version of the test allows for small line segments to be presented gradually and individually in a cumulative fashion, which allows for the exact determination of the point at which recognition occurs (Foreman & Hemmings, 1987).

Historical Background Systematic study of the ability to perceive incomplete figures was initiated by the pioneers of Gestalt psychology, who proposed that individuals perceive objects as wholes within their overall context (Chikhman, Shelepin, Foreman, Merkuljev, & Pronin, 2006). According to the Gestalt principle of closure, individuals tend to fill in missing contours to form a complete object. The Gollin Incomplete Figures Test is a classic test of visual closure that has been used as a measure of Gestalt functioning. Performance on the Gollin Incomplete Figures Test appears to be dependent on the integrity of several areas of the brain, with the posterior cortex in the right hemisphere playing a particularly important role.

Test Properties The Gollin Incomplete Figures Test is easy to administer and score. The examinee’s performance is rated according to the percentage of total figure contour on the card at the time of recognition (Foreman, 1991), which is the sum of all the set numbers at which each picture is correctly identified (Lezak, 1995). A maximum score of 100 is possible, with higher scores representing worse performance. Some research has explored the convergent validity of the Gollin Figures with respect to other visual closure tasks. For example, Foreman (1991) showed that an individual’s ability to identify the fragmented Gollin figures correlates significantly with performance on the Poppelreuter Overlapping Figures Test, but not with the Mooney Test of

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Incomplete Face Perception. This finding has been interpreted to mean that performance on the Gollin Incomplete Figures Test requires the visual system to enable mechanisms of signal-from-noise extraction (Chikhman et al., 2006).

Clinical Uses Within clinical neuropsychology, the ability to perceive and recognize images presented in obscured or fragmented forms has been used for decades to study not only visual closure, but also implicit memory, residual memory in amnestic patients, and visuoperceptual changes caused by brain dysfunction. Repetition of the Gollin Incomplete Figures Test results in rapidly diminishing thresholds, which is thought to be related to implicit perceptual learning and memory (Chikhman et al., 2006). As a result, this test may be a particularly useful instrument for measuring perceptual learning, priming and memory (Chikhman et al., 2006), and it has been studied in populations of individuals with dementia (Beatty, English, & Winn, 1998) and alcoholinduced Korsakoff syndrome (Fama, Pfefferbaum, & Sullivan, 2006). The Gollin Incomplete Figures Test may also successfully discriminate between patients with right and left hemisphere brain injuries, since right posterior parietal lobe damage may interfere with the perception of partial or fragmented contours (Lezak, 1995). In addition, inability to obtain visual closure on fragmented pictures of common objects is a classic test for object agnosia (Farah, 1995). Despite these different clinical uses, it should be noted that the Gollin Incomplete Figures Test is not a purely visual perceptual task, since examinees are required to name the test stimuli. Given this element of confrontation naming, differences in the integrity of semantic memory may affect performance on this measure of visual closure.

Cross References ▶ Apperceptive Visual Agnosia ▶ Closure ▶ Hooper Visual Organization Test ▶ Visual Object Agnosia ▶ Visuoperceptual

References and Readings Beatty, W. W., English, S., & Winn, P. (1998). Long-lived picture priming in normal elderly persons and demented patients. Journal of the International Neuropsychological Society, 4(4), 336–341.

Chikhman, V., Shelepin, Y., Foreman, N., Merkuljev, A., & Pronin, S. (2006). Incomplete figure perception and invisible masking. Perception, 35(11), 1441–1457. Fama, R., Pfefferbaum, A., & Sullivan, E. V. (2006). Visuoperceptual learning in alcoholic Korsakoff syndrome. Alcoholism: Clinical and Experimental Research, 30(4), 680–687. Farah, M. (1995). Visual agnosia. Cambridge, MA: MIT Press. Foreman, N. (1991). Correlates of performance on the Gollin and Mooney tests of visual closure. The Journal of General Psychology, 118(1), 13–20. Foreman, N., & Hemmings, R. (1987). The Gollin Incomplete Figures Test: A flexible, computerised version. Perception, 16(4), 543–548. Gollin, E. S. (1960). Developmental studies of visual recognition of incomplete objects. Perceptual and Motor Skills, 11, 289–298. Lezak, M. D. (1995). Neuropsychological assessment (3rd ed.). New York: Oxford University Press.

Gollin Incomplete Figures Test ▶ Gollin Figures

Gonadal Dysgenesis ▶ Turner Syndrome

Goodglass, Harold (1920–2002) J OHN RYAN 1, T RICIA Z. K ING 2 1 University of Pittsburgh Pittsburgh, PA, USA 2 Georgia State University Atlanta, GA, USA

Major Appointments

National Veterans Aphasic Center, Framingham, MA Director of the National Institutes of Health Aphasia Research Center (1969–1996) Professor of Neurology (Neuropsychology), Boston University School of Medicine President, American Psychological Association Division 40 (Clinical Neuropsychology, 1979–1980) Editorial Board, Cortex, Brain and Language

Goodglass, Harold (1920–2002)


Editor’s Award (Journal of Speech and Hearing Research, 1970) Career Contribution Award (Massachusetts Psychological Association, 1980) Distinguished Career Award (American Speech/ Language and Hearing Association, 1982) Career Contribution Award (American Board of Professional Psychology, 1993) Gold Medal Award for Life Achievement in the Application of Psychology (American Psychological Association, 1996)


Upon earning his Ph.D. in Clinical Psychology, Goodglass was the first psychologist at the National Veterans Aphasic Center in Framingham, MA. His work on cerebral dominance demonstrated that language is mediated by the left hemisphere of the brain in most people, regardless of handedness. His research also contributed to our understanding of various production and comprehension disorders of aphasia. In 1960, in collaboration with Edith Kaplan, he developed the Boston Diagnostic Aphasia Examination (BDAE). He served as the Director of the National Institutes of Health (NIH)-funded Aphasia Research Center based at Boston University from 1969 to 1996, and was the first President of Division 40 of the American Psychological Association.

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working with the Chief of Neurology, Fred Quadfasel, where he returned after graduation in 1951. In the 1950s, the dominant theory postulated that the hemisphere contralateral to the dominant hand controlled language. This included the false assumption of right hemisphere language dominance in left-handed individuals. Goodglass recognized that the theory of cerebral lateralization was not consistent with his experience treating patients. In 1954, Goodglass and Quadfasel published a paper reporting that language was mediated by the left hemisphere in most left-handed individuals, similar to the dominance seen with right-handed individuals. He also published on the neural bases of language processing, comprehension, and production. At the same time, Goodglass focused on developing standardized measures for lingustic input and output, for which there were no measures at the time. Goodglass developed techniques for measuring agrammatism, syntax comprehension, and he recognized a variety of category-specific naming disorders. Many of his instruments, including the BDAE, are widely used today. In 1965, along with Norman Geschwind and others, he organized the NIH-funded Aphasia Research Center, of which he was the director from 1969 until 1996. He was a founding member of the Academy of Aphasia and the International Neuropsychological Society. Dr. Goodglass is credited with establishing the American Psychological Association’s Division 40 (Clinical Neuropsychology) and serving as its first President (1979–1980). He published more than 130 papers with 60 different coauthors, and served on the editorial boards of Cortex and Brain and Language. Harold Goodglass died in 2002.

Cross References Short Biography Harold Goodglass was born on August 18, 1920, in New York City. After attending the City College of New York where he earned his Ph.D. in French, he worked as a New York City welfare investigator before enlisting in the US military in 1942, serving as Captain in the Army Air Force. He enrolled at New York University where he received his Master of Arts degree in Psychology. He then earned his Ph.D. in Clinical Psychology at the University of Cincinnati. During his doctoral training, he treated cases at the Fort Thomas Veterans Administration (VA) Hospital where he first began working with patients with aphasia. His dissertation focused on abstract behavior and name retrieval in individuals with aphasia. He spent the summer of 1949 at the VA hospital in Framingham, MA,

▶ Boston Diagnostic Aphasia Examination ▶ Geschwind, Norman (1926–1984) ▶ Kaplan, Edith (1924–2009)

References and Readings Goodglass, H. (1993). Understanding aphasia.San Diego, CA: Academic Press. Goodglass, H., & Blumenstein, S. (1973). Psycholinguistics and aphasia. Baltimore: Johns Hopkins University Press. Goodglass, H., Fodor, I. G., & Schulhoff, C. (1970). Prosodic factors in grammar: Evidence from aphasia. Journal of Speech and Hearing Research, 10, 5–20. Goodglass, H., & Kaplan, E. (1983). Assessment of aphasia and related disorders (2nd ed.). Philadelphia: Lea & Febiger.

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Goodglass, H., Klein, B., Carey, P., & Jones, K. (1966). Specific semantic word categories in aphasia. Cortex, 2, 74–89. Goodglass, H., & Menn, L. (1985). Is agrammatism a unitary phenomenon? In M. L. Kean (Ed.), Agrammatism. San Diego, CA: Academic Press. Goodglass, H., & Quadfasel, F. A. (1954). Language laterality in lefthanded aphasics. Brain, 77, 521–548. Goodglass, H., Quadfasel, F. A., & Timberlake, W. H. (1964). Phrase length and the type and severity of aphasia. Cortex, 1, 133–153.

Grammar LYN T URKSTRA University of Wisconsin – Madison Madison, WI, USA

Definition

GOS ▶ Glasgow Outcome Scale

GOSE ▶ Glasgow Outcome Scale – Extended

Grammar is the set of rules for a given language, including rules for word meaning (semantics) and word order (syntax).

Cross References ▶ Agrammatism ▶ Morpheme ▶ Paragrammatism ▶ Phonology ▶ Syntax

Grand Mal Seizure Gough Dissimulation Index ▶ F Minus K Index

GPT

K ENNETH P ERRINE Northeast Regional Epilepsy Group Hackensack, NJ, USA Weill-Cornell College of Medicine New York, NY, USA

Synonyms Convulsion; Generalized tonic–clonic seizure

▶ Grooved Pegboard Test

Definition

Gracile Nucleus ▶ Nucleus Gracilis

Grade II Astrocytoma ▶ Fibrillary Astrocytoma

A grand mal seizure is a lay term for a generalized tonic–clonic seizure (GTC) that includes convulsions and loss of consciousness. The term ‘‘grand mal’’ is French for ‘‘big illness,’’ and is used by the lay public to describe the most common seizure type depicted in the media and associated with epilepsy. For this entry, the proper term ‘‘generalized tonic–clonic seizure’’ (GTC) is used.

Historical Background GTCs have been described from ancient times. The Greeks attributed GTCs to disruption of the bodily humors.

Grand Mal Seizure

Julius Caesar was reported to have had ‘‘the falling sickness’’ which presumably represented GTCs. In medieval times, they were thought to be caused by witchcraft or possession by evil spirits. During the eighteenth and nineteenth centuries, physicians began more systematically describing and categorizing seizures, including GTCs. The invention of electroencephalogram (EEG) in the late nineteenth century and refined by Gibbs, Davis, and Lennox in 1935, marked a great leap in our understanding of GTCs, as the EEG could show the generalized epileptic discharges not only during the ictal period of the GTC but also during the resting interictal states. The advent of intraoperative electrocorticography (ECoG) directly from the brain at the Montreal Neurological Institute allowed for more precise identification of interictal discharges, and prolonged video-EEG monitoring with intracranial recordings (subdural grids and strips, depth electrodes) showed that what was thought to be primary generalized epilepsy with GTCs was, in some patients, actually ictal spread from a simple partial seizure focus that was amenable to neurosurgical resection.

Current Knowledge A GTC is a seizure caused by a large burst of electrical discharges that begins in or spreads to broad, bilateral brain regions simultaneously (as opposed to a partial seizure). GTCs can begin all at once across the brain, in which case they are called primary GTCs; or they may result from spread from a focal or partial onset (from either simple partial seizures or complex partial seizures); in which case, they are termed secondarily GTCs. A GTC usually follows a stereotypical pattern. During the initial tonic phase, there is often a forced respiratory expiration (‘‘epileptic cry’’) caused by contraction of the diaphragm. The head may turn to one side in a versive movement. Simultaneously or very shortly thereafter, tonic activation of other large muscle groups occurs, usually represented by extensor muscle activation. Consequently, the patient holds his/her arms and legs straight out in a stiff and sometimes, trembling posture. The tonic phase lasts 1000 –2000 on the average. It is followed by the clonic phase, in which large muscle groups convulse, resulting in large-amplitude violent shaking or jerking of the limbs, trunk, and neck. These clonic movements are usually bilateral and symmetrical (i.e., both arms or legs jerk at the same time and in similar amplitude/frequency). There is a complete loss of consciousness and motor functioning during the ictus. The patient will fall to the ground if standing. Normal respirations are disrupted by the seizing

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of the muscles controlling breathing, and the patient may become cyanotic near the end of the seizure visible in the lips or nail beds. Although biting the tongue or inside of the cheek may occur, the patient does not ‘‘swallow’’ the tongue, which instead is undergoing the same tonic– clonic movements as other muscles. Frothy saliva mixed with blood may exude from the mouth, and there may be urinary or fecal incontinence. The seizure typically lasts 1–2 min. The patient will be amnestic for anything occurring during the episode, but may recall an aura if one precedes the GTC. There are frequently postictal deficits after the end of the seizure, including marked fatigue with sleeping, muscle soreness and/or weakness, confusion, disorientation, or agitation. The seizure should be timed from start to finish. The only first aid that is required is loosening any restrictive clothing around the neck (e.g., tie, scarf), rolling the patient on his/her side so that any secretions from the mouth are exuded out rather than aspirated, and protecting the patient from injury due to the strong muscle contractions (especially placing something soft under the head to protect from head injury, and ensuring that the limbs are not trapped or striking anything to produce fractures). NO attempt should be made to put anything in the patient’s mouth; in addition to being nearly impossible due to tonic contraction of the jaw muscles, such attempts may result in injury to the patient or the helper. Bystanders should be limited to those persons actively helping to avoid embarrassment. Calling 911 or seeking medical attention is a complex issue, but generally medical help should be sought if it is the patient’s first seizure (per witnesses, or absence of a MediAlert bracelet, necklace, or pendant), if the seizure lasts more than 2 min and/or is accompanied by significant cyanosis, if the patient is pregnant; or if injuries are observed or suspected. Patients with GTCs often have no positive neuroimaging findings, but typically have bursts of 3-per-s bilaterally synchronous spike/wave epileptiform activity on routine EEG (even when not having a seizure). GTCs can be idiopathic (no known cause) or may result from a known (symptomatic) or suspected (cryptogenic) underlying cause such as a brain tumor, head trauma, cortical dysplasia, etc. GTCs can begin in childhood or adulthood, and usually respond well to medication. They may or may not remit after a period of treatment, but it is common in patients to remain on antiepileptic drugs for long durations even if they are not having seizures. GTCs may have cyclical components or triggers, such as menses, sleep deprivation, alcohol (especially withdrawal after alcohol consumption), or stress. Seizures may follow

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nocturnal or diurnal patterns, with GTCs during sleep being especially common. If a patient has only nocturnal seizures, they are sometimes not prohibited from driving. Usually, only mild (at worst) neuropsychological deficits are associated with GTCs if they occur without other seizure types and if they are well controlled with medication. However, significant cognitive deficits can occur from episodes of status epilepticus (GTCs lasting >20 min or serial GTCs occurring with only brief intervals between seizures), very frequent episodes, or in association with other seizure types in epilepsy such as Lennox– Gastaut syndrome, West syndrome, etc.

Granular or Koniocortex (Sensory Cortex) ▶ Primary Cortex

Grapheme A DELE S. R AADE Language, & Hearing Sciences Boston, MA, USA

Cross References ▶ Aura ▶ Epilepsy ▶ Seizure

References and Readings Engel, J., Pedley, T. A. (Eds.). (2008). Epilepsy: A comprehensive textbook (2nd ed.). New York: Lippincott Williams & Wilkins. www.epilepsyfoundation.org

Grandiosity N ATALIE C. B LEVINS Indiana University Hospital Indianapolis, IN, USA

Definition Grapheme refers to the visual or written representation of a particular letter (e.g., b, g, or m). When an individual engages in oral reading, he performs grapheme– phoneme conversion. That is, he pairs the particular visual image of a letter with the sound associated with that letter.

Cross References ▶ Grapheme ▶ Graphesthesia ▶ Phoneme

References and Readings Goodglass, H. (1993). Understanding aphasia. San Diego, CA: Academic Press.

Definition The term ‘‘grandiosity’’ is used to describe the largerthan-life feelings of superiority often experienced by those in a manic, hypomanic, or mixed episode. It has been described as an exaggerated sense of one’s importance, power, knowledge, or identity. It may contain religious overtones. It also occurs in delusional disorder.

References and Readings Sadock, B. J., & Sadock, V. A. (2007). Kaplan & Sadock’s synopsis of psychiatry: Behavioral sciences/clinical psychiatry (10th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.

Graphesthesia K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Definition Graphesthesia is the ability to recognize writing on the skin. Its name derives from Greek grapha (writing) and

Gray Matter

aisthesis (perception). Graphesthesia was first described in 1920 by Sir Henry Head. In the sensorimotor exam, with the patient’s eyes closed, the examiner writes single numbers or letters on the palm of the hand or fingertips with something relatively blunt, like the dull point of a pencil. Considered a measure of fine tactual discrimination, the inability to decipher the numbers or letters being written could be the result of lesions anywhere in the medial lemniscal system, from the dorsal columns to the cortex. If more of the elementary functions (such as vibration and proprioception) are intact, then the inability to perform on this task might suggest a functional disturbance in the hand region of the postcentral gyrus. Typically, the performance on the right is compared with that on the left to help identify the lateralized deficits. Body parts other than the hands could be tested, but the size of the figures should be commensurate with the sensitivity of the region. Studies have also suggested that loss of graphesthesia may be related to some psychiatric disorders, such as schizophrenia and obsessive–compulsive disorder, but in these cases, the lateralized deficiencies would not be expected.

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Definition The grasp reflex is elicited by stroking the palmar surface of the patient’s hand, causing the fingers to close and grasp any object left in the hand. It is one of the frontal release signs, primitive reflexes that are normal in infants, disappear with brain maturation allowing inhibition, and reappear (are ‘‘released’’) in disorders that affect the frontal lobes. Like most primitive reflexes, the grasp reflex has evolutionary/adaptive advantage in infant apes, allowing to cling automatically to their mother’s hair. This reflex appears at birth and persists until 5 or 6 months of age, after which it is thought to indicate damage to the contralateral frontal lobe.


Gray Matter References and Readings Segalowitz, S. J., & Rapin, I. (2003). Child neuropsychology. In F. Boller & J. Grafman (Eds.), Handbook of neuropsychology (2nd ed., pp. 393–394). Amsterdam: Elsevier. Varney, N. J. (1986). Some thesis. In H. J. Hannay (Ed.), Experimental techniques in human neuropsychology (p. 232). New York: Oxford.

J EFF D UPREE Virginia Commonwealth University Richmond, VA, USA

Synonyms Gray substance; Substantia grisea

Graphomotor Function ▶ Visual-Motor Function

Grasp Reflex S TEPHEN P. S ALLOWAY Alpert Medical School of Brown University Providence, RI, USA

Synonyms Palmar grasp reflex

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Definition Brownish gray regions of the brain and spinal cord that contain the neuronal cell bodies.

Current Knowledge Based on distinction by the naked eye, the central nervous system (CNS) can be divided into two parts known as white matter and gray matter. These regions were first distinguished by Thomas Willis in his Anatomy of the Brain published in 1664. The white matter contains high levels of lipid and thus appears white contrasting with a brownish gray hue commonly associated with the gray matter. The gray matter is formed by the cortex and nuclei of the brain, the horns of the spinal cord, and the ganglia.

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These regions are heavily populated by dense neuronal cell bodies and numerous capillaries and associated blood cells. Together, the cell bodies and the capillaries are responsible for the darker, grayish color. In addition to the neuronal cell bodies and the endothelial cells of the capillaries, the gray matter also contains neuronal processes known as dendrites and axons, astrocytes, oligodendrocytes, and microglia. In the cerebral cortices or telencephalon of the brain, the gray matter consists of a relatively thin outer layer, which covers the more centrally positioned white matter. In the spinal cord, this organization is reversed with the gray matter constituting a central core that is surrounded by white matter. Dependent on the region of the CNS, the primary function of gray matter is to process or initiate neuronal impulses frequently in response to an external stimulus. The response is conveyed from the gray matter into the white matter by extensions of the neuronal cell body known as axons. Recent studies demonstrate a direct correlation between gray matter volume and intelligence particularly with increased gray matter of the prefrontal cortex, the region of the brain associated with higher-order cognitive functions. Individuals with schizophrenia frequently display up to 25% loss of gray matter volume in some regions and patients with the most severe loss of gray matter can exhibit symptoms including hallucinations, delusions, bizarre and psychotic thoughts, hearing voices, and depression. In contrast, recent studies have shown that children with autism and Asperger syndrome, an autism-related disorder, have more gray matter particularly in the parietal lobe.

References and Readings Barr, M., & Kiernan, J. (1983). The human nervous system – an anatomical viewpoint (4th ed.). Philadelphia: Harper and Row. Casanova, M. F. (2007). The neuropathology of autism. Brain Pathology, 17(4), 422–433. Csernansky, J. G. (2007). Neurodegeneration in schizophrenia: Evidence from in vivo neuroimaging studies. ScientificWorldJournal, 7, 135–143. Haines, D. E. (2006). Fundamental neuroscience for basic and clinical applications (3rd ed.). Philadelphia: Churchill Livingstone Elsevier. Narr, K. L., Woods, R. P., Thompson, P. M., Szeszko, P., Robinson, D., Dimtcheva, T., et al. (2007). Relationships between IQ and regional cortical gray matter thickness in healthy adults. Cerebral Cortex, 17(9), 2163–2171. Whitford, T. J., Grieve, S. M., Farrow, T. F., Gomes, L., Brennan, J., Harris, A. W., et al. (2006). Progressive grey matter atrophy over the first 2–3 years of illness in first-episode schizophrenia: A tensor-based morphometry study. Neuroimage, 32(2), 511–519.

Gray Substance ▶ Gray Matter

Grip Strength ▶ Hand Dynamometer ▶ Manual Strength

Grooved Pegboard Test B RAD M ERKER , K ENNETH P ODELL Henry Ford Health Systems Detroit, MI, USA

Synonyms GPT

Description The GPT assesses eye–hand coordination and motor speed and thus requires sensory motor integration and a high level of motor processing (Roy & Square-Storer, 1994). It is considered a more complex motor task than others such as Grip Strength or Finger Tapping. As such, it requires more effort and is more sensitive to psychomotor speed (Mitrushina, Boone, Razani, D’Elia, 2005). The test has been used extensively for identifying lateralized impairment such as that in Parkinson’s disease (Demakis et al., 2002). In addition, it has been found to be sensitive in detecting general slowing due to medication or disease progression (Tiffin, 1968), and used to evaluate cognitive and motor slowing in Bipolar disorder (Wilder-Willis, Sax, Rosenberg, Fleck, Shear, Strakowski, 2001), HIV infection (Honn, Para, Whitacre, & Bornstein, 1999), and other conditions of interest to neuropsychologists. The test apparatus consists of a square metal surface (10.1 cm2) with a 5 5 matrix of keyhole shaped holes in various orientations. The task requires the examinee to pick up the keyhole shaped peg (3 mm in diameter)

Grooved Pegboard Test

individually from the well just above the 5 5 matrix of holes from left to right and top to bottom as quickly as possible using the dominant, nondominant, or sometimes both hands simultaneously. Because the holes are in various orientations, examinees must manipulate each peg in their hand (usually between the index finger and thumb) so that the peg aligns with the orientation of the hole. If desired, only the dominant hand may be tested. More commonly, the examinee is instructed to start with the dominant hand and then proceed with the nondominant hand, followed by a trial using both hands. In 2005, Bryden and Roy developed a remove task that requires examinees to remove pegs from the holes as quickly as possible and place them back into the receptacle. They proposed that the remove task may not rely as heavily on visual information and thus may provide a more pure measure of motor speed than the standard measure. The time taken for completion is recorded for the placement and removal trials for the dominant, nondominant, and both hands (Heaton, Grant, & Matthews, 1986, 1991; Bryden and Roy, 2005). The dominant hand is significantly faster than the nondominant hand across both tasks, although performance differences between the hands are larger for the place task because of the degree of manual skill required to perform that task (Bryden and Roy, 2005). To detect a lateralized effect of impairment, Bornstein (1986) proposed that a right/left score ratio of greater than 1.0 is suggestive of right hemisphere disease and a ratio less than 1.0 may be suggestive of damage to the left hemisphere, but he cautioned that data from other tests should also be used. Bornstein, Paniak and O’Brien (1987) also recommended using a cut score greater than or equal to 92 for the dominant hand and greater than or equal to 99 for the nondominant hand to classify individuals as impaired. Using these cut scores, only 11% and 9% of normal subjects were misclassified with the dominant and nondominant hands, respectively, while a higher percentage of patients was misclassified.

Historical Background The Grooved Pegboard Test (GPT) was created in 1963 and is available from the Lafayette Instrument Company (Klove, 1963; Mathews and Klove, 1964). The test is part of the Wisconsin Neuropsychological Test Battery

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(Harley et al., 1980; Mathews and Klove, 1964) and the Repeatable Cognitive Perceptual-Motor Battery (Kelland and Lewis, 1994; Lewis and Kupke, 1992).

Psychometric Data Test–retest: Test–retest and practice effects were found on GPT. Test–retest reliability is considered adequate with coefficients ranging between 0.69 and 0.76 for the dominant and nondominant hands, respectively (Ruff and Parker, 1993). Practice effects were investigated by Schmidt, Olveira, Rocha and Abreu-Villaca (2000). They found that after the first trial, there is a remarkable improvement in performance in both hands, regardless of gender. Bryden and Roy (2005) also found that the removal task was the most significantly affected by practice. They also investigated the transfer of training across hands. Results indicated that only left-handed males benefit from previous opposite-hand performance. They proposed that the transfer of training was due to a larger corpus callosum. Demographic Variables: Numerous studies have investigated the impact of hand preference and demographic variables on the Grooved Pegboard Test. Handedness was not a particularly important variable affecting test performance (Ruff and Parker, 1993). However, the expected dominant hand superiority has been demonstrated in meta-analytic studies (Mitrushina et al., 2005) with about a 10% inter-manual difference found. The effects of age have been well documented, with slowing increasing with advancing age (Ruff & Parker, 1993). In a meta-analytic review, Mitrushina and coworkers (2005) found a large age effect for both the dominant (R2 = 0.936) and nondominant (R2 = 0.907) hands. The effects of gender are more equivocal. The metaanalysis by Mitrushina et al. (2005) did not find a consistent gender effect. Numerous other studies have shown that men and women differ significantly in performance for both the dominant and nondominant hands, with women performing on average 4–6 s faster than men (Bornstein, 1985; Dodrill, 1979; Ruff & Parker, 1993; Schmidt et al., 2000). Larger sex differences were found with the preferred hand on the remove task with males being slower than females with a larger performance difference between the preferred and non-preferred hand (Bryden & Roy, 2005; Thompson, Heaton, Matthews, & Grant, 1987). However, Peters and Campagnaro (1996) suggested that this difference may be due to finger size rather than gender. Women, who generally have smaller

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fingers, perform significantly faster than men given the proximity of the pegs in the holes. The effects of education on Grooved Pegboard Test performance are less clear (Mitrushina et al., 2005). Bornstein (1985) found education differences for the dominant, but not the nondominant hand. In contrast, Heaton, Grant and Matthews (1993) failed to find education differences using a more educated sample. In a third study, education predicted performance, with more highly educated groups performing faster than the lowest educated groups for both the nondominant and dominant hands (Ruff & Parker, 1993). See Appendix 22 from Mitrushina et al. (2005) for comprehensive listing of various normative tables and their meta-analysis of these studies.

Clinical Uses The Grooved Pegboard is a test of manual dexterity used to detect cognitive slowing due to disease progression and/ or the effects of medication. It is also used in combination with other tests of motor functioning to detect lateralized impairment due to neurological injury (e.g., ▶ stroke, ▶ traumatic brain injury, ▶ tumor, ▶ Parkinson’s disease). The test has also recently been used to predict hand dominance. Brown, Roy, Rohr, Snider and Bryden (2004) looked at various motor tests ability to predict hand preference. They found that the lateralized place phase of the Grooved Pegboard Test was the most accurate predictor of hand preference.

Cross References ▶ Finger Tapping Test (FTT) ▶ Hand Dynamometer ▶ Purdue Pegboard

References and Reading Bornstein, R. A. (1985). Normative data on selected neuropsychological measures from a nonclinical sample. Journal of Clinical Psychology, 41, 651–659. Bornstein, R. A. (1986). Normative data on intermanual differences on three tests of motor performance. Journal of Clinical and Experimental Neuropsychology, 8, 12–20. Bornstein, R. A., Paniak, C., & O’Brien, W. (1987). Preliminary data on classification of normal and brain-damaged elderly subjects. Clinical Neuropsychologist, 1(4), 315–323.

Brown, S. G., Roy, E. A., Rohr, L. E., Snider, B. R., & Bryden, P. J. (2004). Preference and performance measures of handedness. Brain and Cognition, 55(2), 283–285. Bryden, P. J., & Roy, E. A. (2005). A new method of administering the grooved pegboard test: performance as a function of handedness and sex. Brain and Cognition, 58, 258–268. Dodrill, C. B. (1979). Sex differences on the Halstead-Reitan neuropsychological battery and on other neuropsychological measures. Journal of Clinical Psychology, 35(2), 236–241. Harley, J. P., Leuthold, C. A., Matthews, C. G., & Bergs, L. E. (1980). Wisconsin neuropsychological test battery T-score norms for older veterans administration medical center patients. Madison, WI: Department of Neurology, University of Wisconsin Medical School. Heaton, R. K., Grant, I., & Matthews, C. G. (1986). Differences in neuropsychological test performance associated with age, education, and sex. In I. Grant & K. Adams (Eds.), Neuropsychological assessment of neuropsychiatric disorders. New York: Oxford University Press. Heaton, R. K., Grant, I., & Matthews, C. (1991). Comprehensive norms for an expanded halstead-reitan neuropsychological battery: Demographic corrections, research findings, and clinical applications. Odessa, FL: Psychological Assessment Resources. Honn, V. J., Para, M. F., Whitacre, C. C., & Bornstein, R. A. (1999). Effect of exercise on neuropsychological performance in asymptomatic HIV infection. AIDS and Behavior, 3(1), 67–74. Kelland, D. Z., & Lewis, R. F. (1994). Evaluation of the reliability and validity of the repeatable cognitive-perceptual-motor battery. The Clinical Neuropsychologist, 8, 295–308. Klove, H. (1963). Clinical neuropsychology. In F. M. Forster (Ed.), The Medical Clinics of North America (pp. 1647–1658). New York: WB Saunders. Lewis, R., & Kupke, T. (1992). Intermanual differences on skilled and unskilled motor tasks in nonlateralized brain dysfunction. The Clinical Neuropsychologist, 6, 374–382. Matthews, C. G., & Klove, H. (1964). Instruction manual for the adult neuropsychology test battery. Madison, WI: University of Wisconsin Medical School. Mitrushina, M., Boone, K. B., Razani, J., D’Elia, L. F. (2005). Handbook of normative data for neuropsychological assessment (2nd ed.). New York: Oxford University Press. Peters, M., & Campagnaro, P. (1996). Do women really excel over men in manual dexterity. Journal of Experimental Psychology: Human Perception and Performance, 22, 1107–1112. Roy, E. A., & Square-Storer, P. A. (1994). Neuropsychology of movement sequencing disorders and apraxia. In D. W. Zaidel (Ed.), Neuropsychology. St. Louis, MO: Academic Press. Ruff, R. M., & Parker, S. B. (1993). Gender and age specific changes in motor speed and eye-hand coordination in adults: Normative values for the finger tapping and grooved pegboard tests. Perceptual and Motor Skills, 76, 1219–1230. Schmidt, S. L., Olivereira, R. M., Rocha, F. B., & Abreu-Villaca, Y. (2000). Influences of handedness and gender on the grooved pegboard test. Brain and Cognition, 44, 445–454. Thompson, L. L., Heaton, R. K., Matthews, C. G., & Grant, I. (1987). Comparison of preferred and nonpreferered hand performance on four neuropsychological motor tasks. Clinical Neuropsychologist, 1(4), 324–334. Tiffin, J. (1968). Purdue pegboard examiner’s manual. Rosemont, IL: London House. Wilder-Willis, K. E., Sax, K. W., Rosenberg, H. L., Fleck, D. E., Shear, P. K., Strakowski, S. M. (2001). Persistent attentional dysfunction in remitted bipolar disorder. Bipolar Disorders, 2, 58–62.

Group Therapy

Group Embedded Figures Test ▶ Embedded Figures Test

Group Therapy J EFFREY G. K UENTZEL 1, E LISE K. H ODGES 2, S USANNAH M ORE 2 1 Wayne State University Detroit, MI, USA 2 University of Michigan Health System Ann Arbor, MI, USA

Definition Group therapy is a form of psychotherapeutic intervention characterized by a group format (i.e., several clients meeting with a therapist together).

Historical Background Nonclinical forerunners of group psychological interventions, such as mass religious or political movements, have taken place for millennia, but it is believed that the first therapeutic groups conducted by physicians occurred in the early twentieth century (Rosenbaum & Patterson, 1995). Joseph Pratt is recognized for having initiated therapy groups as early as 1905. Pratt led ‘‘thought control classes,’’ inspirational lectures and group discussions with hospitalized tuberculosis patients, in Boston, Massachusetts (Barlow, Burlingame, & Fuhriman, 2005). Pratt sought to increase adherence with medical regimens in his patients, but also found that his group meetings resulted in strengthened hope for recovery and lifting of morale. Around the same time, J. L. Moreno was beginning development of group methods in Vienna. Moreno introduced psychodrama to the United States in 1925, and in 1932 published the first book on group psychotherapy (Fuhriman & Burlingame, 1994). The first use of the term group therapy is also attributed to Moreno. Other pioneers who helped establish the practice of group therapy include L. C. Marsh, E. W. Lazell, Trigant Burrow, and Samuel Slavson in the United States, and Wilfred Bion, S. H. Foulkes, and Rudolf Dreikurs in Europe (Rosenbaum & Patterson, 1995).

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Rationale or Underlying Theory Early theoretical influences on group therapy practice included Kurt Lewin’s field theory, which addressed group dynamics, a term he coined (Barlow, Burlingame, & Fuhriman, 2005). Psychodynamic theory, especially the work of Adler, helped guide the developers of group therapy practice in its first few decades, as did Sullivan’s strong emphasis on the interpersonal nature of personality. Irvin Yalom has made seminal contributions to the practice of modern group therapy. His volume The Theory and Practice of Group Psychotherapy, now in its fifth edition (Yalom & Leszcz, 2005) synthesizes a wide range of important research and theory. Yalom is known for his experiential, process-oriented approach to group therapy, which emphasizes the importance of a here-and-now focus on interactions among the group members. Yalom has identified what he terms therapeutic factors in group therapy. According to Yalom, groups are effective because they impart information and instill hope. Group cohesiveness enhances clients’ self-esteem through acceptance by the group. Universality is experienced when clients discover that they are not alone in their struggles. Altruism occurs when clients help other clients. Catharsis benefits clients who learn to express strong emotions appropriately. There is development of socializing techniques, and imitative behavior or modeling of more successful ways of interacting. Yalom also discusses corrective recapitulation of the primary family, in which maladaptive coping methods that were learned from family members are replaced. Finally, there is the potential for insight, and existential factors can be explored (i.e., accepting responsibility for life decisions).

Goals and Objectives Group therapy may be effective for a wide variety of psychological disorders, including depression, posttraumatic stress disorder, panic disorder, social phobia, substance abuse, eating disorders, and some personality disorders (Burlingame, Kapetanovic, & Ross, 2005). There also are self-help groups for a wide range of disorders and problems, although more evidence demonstrating their effectiveness is needed. In comparison to the vast adult group therapy literature, group therapy approaches for children have received considerably less attention. However, some childhood problems (e.g., divorce of parents, shyness, and aggressiveness) may be treated in groups (Schechtman, 2007).

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Group Therapy

Rehabilitation psychologists are developing group approaches for assisting brain-injured patients who frequently suffer from deficits in self-awareness, diminished psychosocial functioning, altered self-esteem, and mood disorders. Such group treatments are usually a part of a comprehensive neuropsychological rehabilitation program that targets focal deficits and other sequelae of traumatic brain injury. These programs often include speech therapy for aphasic patients (which may take place in groups). Further, psychoeducational groups for patients and their families can provide essential information about brain disorders and neuro-rehabilitation methods as well as provide support during the rehabilitation process (e.g., Champion, 2006).

Treatment Participants It is probably not that far-fetched to suggest that, for anyone who might be in need of group therapy, a group method has been developed that could be a reasonable fit. However, Yalom suggests that inappropriately selected patients are at high risk for dropping out early. For an experiential, process-oriented group, Yalom has recommended that brain-injured, paranoid, psychotic, sociopathic, addicted, and hypochondriacal patients not be included (Ballinger & Yalom, 1995). Nevertheless, he notes that most such patients could do well in other kinds of groups that are not process-focused (such as didactic, cognitive-behavioral, or supportive groups).

Treatment Procedures Therapy groups are to be distinguished from mutual support groups, or self-help groups. According to Barlow (2008), therapy groups can be categorized into homogeneous/theme groups (problem/diagnosis focused) or heterogeneous/process-oriented groups (a la Yalom). Problem- or diagnosis-focused groups, such as an anger-management group, tend to be shorter in duration and sometimes are set at 10 or 12 sessions. An exception is the Dialectical Behavior Therapy skills group, designed for individuals with Borderline Personality Disorder, which is heavily didactic and not process-oriented, but requires 6–12 months of weekly sessions (Linehan, 1993). Process-oriented groups usually are long-term and may be open-ended. Group sessions of most kinds are typically around 90–120 min long.

Group therapy has the potential to be especially powerful as compared to individual therapy because it can capitalize on both intrapersonal and interpersonal factors (Barlow, 2008). It is not individual therapy with an audience. Rather, each patient can benefit from being both a receiver of help, and a ‘‘therapist’’ (a giver of help to others).

Efficacy Information Several meta-analytic studies have demonstrated group therapy to be as effective as individual therapy, and in some cases, it may be more effective (Barlow, 2008). Burlingame, Fuhriman, and Mosier (2003) subjected 111 group therapy effectiveness studies to meta-analytic procedures. The overall obtained effect size for group therapy as compared to waitlist control conditions was 0.58, indicating that the average group member fared better than 72% of untreated controls. The strongest evidence of effectiveness was found for anxiety, depression, and eating disorder group therapies. Nearly two dozen studies of therapy groups for medical conditions were identified, and the effect size for these groups was significant relative to waitlist. Regrettably, little improvement was documented in this study for patients with substance abuse, thought disorder, or criminal behavior. However, another review (Burlingame, Kapetanovic, & Ross, 2005) reported that substance abuse patients who participate in group treatment show greater improvement than those who receive standard non-group care and those who refuse treatment or drop out. The American Group Psychotherapy Association (AGPA, 2007) recently released an 85-page practice guidelines document which is the culmination of task force efforts to meet the demands of evidence-based practice and greater accountability.

Outcome Measurement Literature reviews focusing on the methodological issues in group therapy research have called for measurement of outcomes in a quantifiable, reliable, and valid manner. Outcome measurement instruments should also be matched to the unique targets of the group treatment, to the patient population, and to the key research questions (Burlingame, Kircher, & Taylor, 1994). Thus, for a depression group, outcome would be determined by administering psychometrically sound depression inventories to

Guillain–Barre´ Syndrome

the group members, whereas for an eating disorders group, measures of binge eating may be needed, and for a substance abuse group, urine drug screens might be appropriate. In addition to measuring outcome, in group research it is often of interest to measure aspects of the group process. Sometimes these measures are completed by trained raters who view video-recorded group sessions. For example, group cohesion can be assessed with the Group Cohesiveness Scale (Budman et al., 1987). Another such process instrument is the Individual Group Member Interpersonal Process Scale (Davis, Budman, & Soldz (2000). The therapeutic alliance is another important process variable, both in group therapy as well as in individual therapy. The AGPA practice guidelines describe a battery of measures for assessing therapeutic alliance, cohesion, client empathy, and other process variables (‘‘Core Battery’’, AGPA, 2007).

Qualifications of Treatment Providers True expertise in group therapy may require focused training from the undergraduate to the postdoctoral level (Barlow, 2008). The AGPA provides group psychotherapist certification, and fellow status in Division 49 of the American Psychological Association (APA) is an indication of expertise in group therapy. Board certification is possible through the American Board of Group Psychotherapy (ABGP), a specialty area of the American Board of Professional Psychology (ABPP).

Cross References ▶ Patient-Family Education ▶ Psychotherapy ▶ Rehabilitation Psychology

References and Readings American Group Psychotherapy Association. (2007). Practice guidelines for group psychotherapy. Ballinger, B., & Yalom, I. D. (1995). Group therapy in practice. In B. Bongar and L. E. Beutler (Eds.), Comprehensive textbook of psychotherapy: Theory and practice (pp. 189–204). New York: Oxford. Barlow, S. H. (2008). Group psychotherapy specialty practice. Professional Psychology: Research and Practice, 39(2), 240–244. Barlow, S. H., Burlingame, G. M., & Fuhriman, A. J. (2005). The history of group practice: A century of knowledge. In S. A. Wheelan (Ed.),

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The handbook of group research and practice (pp. 39–64).Thousand Oaks, CA: Sage Publications. Budman, S. H., Demby, A., Feldstein, M., Redondo, J., Scherz, B., Bennett, M. J., Koppenaal, G., Sabin Daley, B., Hunter, M., & Ellis, J. (1987). Preliminary findings on a new instrument to measure cohesion in group psychotherapy. International Journal of Group Psychotherapy, 37, 75–94. Burlingame, G. M., Fuhriman, A. J., & Mosier, J. (2003). The differential effectiveness of group psychotherapy: A meta-analytic perspective. Group Dynamics: Theory, Research, and Practice, 7(1), 3–12. Burlingame, G. M., Kapetanovic, S., & Ross, S. (2005). Group psychotherapy. In S. A. Wheelan (Ed.), The handbook of group research and practice (pp. 387–406). Thousand Oaks, CA: Sage Publications. Burlingame, G. M., Kircher, J. C., & Taylor, S. (1994). Methodological considerations in group psychotherapy research: Past, present, and future practices. In A. J. Fuhriman & G. M. Burlingame (Eds.), Handbook of group psychotherapy (pp. 41–80). New York: Wiley. Champion, A. J. (2006). Neuropsychological rehabilitation: A resource for group-based education and intervention.Chichester, West Sussex: Wiley. Davis, M. S., Budman S. H., & Soldz, S. (2000). The individual group member interpersonal process scale. In A. P. Beck & C. M. Lewis (Eds.), The process of group psychotherapy: Systems for analyzing change (pp. 283–308). Washington, DC: American Psychological Association. Fuhriman, A. J. & Burlingame, G. M. (1994). Group psychotherapy: Research and practice. In A. J. Fuhriman & G. M. Burlingame (Eds.), Handbook of group psychotherapy (pp. 3–40). New York: Wiley. Linehan, M. M. (1993). Cognitive-behavioral treatment of borderline personality disorder. New York: Guilford Press. Rosenbaum, M., & Patterson, K. M. (1995). Group psychotherapy in historical perspective. In B. Bongar and L. E. Beutler (Eds.), Comprehensive textbook of psychotherapy: Theory and practice (pp. 173–188). New York: Oxford University Press. Schechtman, Z. (2007). Group counseling and psychotherapy with children and adolescents: Theory, research, and practice. Mahwah, NJ: L. Erlbaum Associates. Yalom, I. D. & Leszcz, M. (2005). The theory and practice of group psychotherapy. New York: Basic Books.

Guillain–Barre´ Syndrome K HAN FARY 1, N. G. L OUISA 2 1 University of Melbourne and the Royal Melbourne Hospital Parkville, Victoria, Australia 2 Royal Melbourne Hospital Parkville, Victoria, Australia

Synonyms Acute febrile polyneuritis; Acute infective polyneuritis; Acute inflammatory demyelinating polyradiculoneuropathy (AIDP); Idiopathic polyneuritis; Landry–Guillain– Barre´ syndrome; Landry–Guillain–Barre´–Strohl syndrome

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Short Description or Definition Guillain–Barre´ Syndrome (GBS) is a monophasic illness of immune etiology that presents as an evolving sensorimotor polyneuropathy of varying severity as a result of an inflammation of the peripheral nerves and nerve roots. GBS is a major cause of acute ascending neuromuscular paralysis and is often associated with autonomic dysfunction.

Guillain–Barre´ Syndrome. Table 1 Frequency of features and clinical variants of acute GBS (Reprinted with permission from Ropper (1992)) % Frequency Condition

Fully developed Initially illness

Features of syndrome Paresthesia

70

85

Arms

20

90

Legs

60

95

Face

35

60

25

50

5

15

Weakness

Categorization GBS is recognized as a heterogeneous syndrome with several variant forms (see Table 1). The commonest type of GBS is AIDP. Axonal subtypes include acute motor axonal neuropathy and acute motor and sensory axonal neuropathy. Variants include Miller Fisher syndrome (cranial nerve involvement, ataxia) and acute pan-dysautonomia.

Oropharynx Opthalmoparesis

Epidemiology GBS has an annual incidence of 1–2 per 100,000 worldwide with no geographical clustering. It affects both sexes equally and occurs at all ages, although most commonly between 30 and 50.

Sphincter dysfunction

15

5

Ataxia

10

15

Areflexia

75

90

Pain

25

30

Sensory loss

40

75

Respiratory failure

10

30

CSF protein > 0.55 g/l

50

90

Abnormal electrophysiologic findings

95

99

Clinical variantsa

Natural History, Prognostic Factors, Outcomes The first clinical description of ascending paralytic illness was by Osler (1892), while Guillain, Barre´, and Strohl (1916) reported the syndrome of radiculoneuritis associated with elevated protein in the cerebrospinal fluid. The etiology of GBS is not fully understood. It is thought that an antecedent infection (most commonly Campylobacter jejuni) evokes an immune response (both cellular and humoral), resulting in demyelination. Within 2–3 weeks, the inflammation resolves and remyelination commences. GBS generally has a favorable outcome. Eighty percent are ambulatory (ambulation without assistive devices) within 6 months of symptom onset. Fifty percent have minor residual neurological deficits while 15% have residual functional deficits, and 5% may die of secondary systemic organ failure (Ropper, 1992). In the acute phase of GBS, 5–10% may die and 25% require artificial ventilation owing to involvement of respiratory and bulbar muscles (Hughes, Raphae¨l, Swan, & van Doorn, 2006). The onset, rate, and variability of recovery is unclear (Meythaler, 1997), but

Fisher’s syndrome

5

Weakness without paresthesia or loss

3

Pharyngeal–cervical– brachial weakness

3

Paraparesis Facial paresis with paresthesia Pure ataxia

2 1 1

a

Variants are associated with diminished reflexes, demyelinating features as detected on electrophysiologic studies, and elevated cerebrospinal concentrations of fluid protein. Frequencies shown are those found in fully developed illness.

80% reach the nadir of the disease at 3 weeks after symptom onset (Pascuzzi & Fleck, 1997) and subsequently gradually improve. GBS survivors can continue to improve for 10 years after onset, although slower recovery may occur in those who experienced ICU complications, prolonged mechanical ventilation, and early axonal abnormalities (Dhar, Stitt, & Hahn, 2008). Poorer prognostic factors are older age, need for respiratory support, rapid onset, progression to quadriplegia,


severe disease at presentation, C. jejuni infection, and preceding diarrheal illness (Rees, Soudain, Gregson, & Hughes, 1995). Although most patients make good physical recovery, the ongoing impact of GBS is significant. The effects of GBS on activities of daily living, work, social activities, and health-related quality of life (QoL) is considerable at 2 years after onset and persists beyond this time (Forsberg, Press, Einarsson, de Pedro-Cuesta, & Holmqvist, 2005).

Neuropsychology and Psychology of Guillain–Barre´ Syndrome The psychological, social, and avocational outcomes of GBS are poorly studied. High levels of anxiety (82%), acute stress disorder, depressive episodes (67%), and brief reactive psychosis (25%) in an ICU setting have been reported (Weiss, Rastan, Mullges, Wagner, & Toyka, 2002). Motor deprivation and loss of communication were closely connected with the occurrence of psychotic symptoms. Therapeutically, continuous psychosocial support and psychopharmacological measures may be valuable tools to ameliorate distress (Weiss et al., 2002). Hydrocephalus and intracranial hypertension in acute stages of GBS are infrequent but well-recognized complications and often resolve spontaneously or after ventriculo-peritoneal shunting. Brain imaging is necessary in GBS patients who deteriorate in cognitive function after disease onset and neuropsychological evaluation should be considered (Liu et al., 2006). GBS has a significant impact on patients’ lives, which goes beyond their residual disability or impairment. Five years on, 27% continue to make substantial changes in their job, hobbies, or social activities despite near complete functional recovery (Bersano et al., 2006). In the transition period following discharge from hospital to the community, various adjustment issues may surface, such as perceptions of self-worth and self-image and role reversals within the family. Families may struggle to cope with new demands resulting from increased care needs, difficulties in returning to driving and work, financial worries, marital stress, and other limitations in societal participation. Ongoing monitoring, education, and counselling of the patient (and family) are important. Carer support and respite care should be discussed. Referral to local GBS Society and support groups (GBS survivors) is also recommended, as these provide patients and families with ongoing support, resources, and equipment needs.

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Evaluation The features of GBS are shown in Table 1. Most patients present with symmetric limb weakness, absent (or diminished) reflexes, paresthesias and aching pain in large muscles of back and legs, or sciatica (Ropper, 1992). Thirty percent of patients run a fulminating course with rapid progression requiring ventilatory support within a couple of days (Ropper). Disease progression may be stuttering or insidious and generally continues over the next 2 weeks until it plateaus, and is subsequently followed by a gradual recovery. The duration of illness in most patients is < 12 weeks. Weakness often commences distally and continues in an ascending fashion; resulting in variable weakness in limbs, face, and oropharynx (Rees et al., 1995). Facial weakness and cranial nerve involvement occurs in > 50% (Pascuzzi & Fleck, 1997) and 70% have autonomic dysfunction (sinus tachycardia or bradycardia, fluctuating hypertension or hypotension, flushing of the face, loss of or excessive sweating) (Zochodne, 1994). Severe dysautonomia should be recognized as this is associated with sudden death (Zochodne). Unusual features of GBS include papilloedema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes. The initial diagnosis of GBS is based on clinical presentation (see Table 2). Findings on cerebrospinal fluid examination and neurophysiology studies may then be used to confirm the diagnosis (see Tables 2 and 3). The differential diagnosis of GBS is shown in Table 4 CIDP and relapsing inflammatory polyneuropathy are distinct from GBS. These are important from the rehabilitation standpoint, as their diagnosis and prognosis is different (Meythaler, 1997).

Treatment Specialist units have improved survival rates in patients with GBS and reduced mortality rate to less than 5% (Ropper, 1992). Acute medical complications in GBS include development of sepsis, respiratory failure, aspiration pneumonia, deep vein thrombus, pulmonary embolism, and cardiac arrest (Dhar et al., 2008).

Plasmapheresis, Intravenous Immunoglobulin, and Steroids Acute management of GBS includes intensive care and ventilatory support, and immune modulating treatments

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Guillain–Barre´ Syndrome. Table 2 Diagnostic criteria for typical GBS (Adapted and reprinted with permission from Asbury & Cornblath (1990)) Features required for diagnosis Progressive weakness in both arms and both legs Areflexia Features strongly supporting the diagnosis Progression of symptoms over days to 4 weeks Relative symmetry of symptoms Mild sensory symptoms or signs Cranial-nerve involvement, especially bilateral weakness of facial muscles Recovery beginning 2–4 weeks after progression ceases Autonomic dysfunction Absence of fever at the onset Elevated concentration of protein in cerebrospinal fluid, with fewer than 10 cells/ mm3 Typical electrodiagnostic features Features making the diagnosis doubtful Diagnosis of botulism, myasthenia, poliomyelitis, or toxic neuropathy Abnormal porphyrin metabolism Recent diphtheria Purely sensory syndrome, without weakness

(plasma exchange and intravenous immunoglobulin (IVIg)) to reduce nerve damage. Early treatment with either IVIg or plasma exchange (both are equally effective) within 2–4 weeks after symptom onset may decrease recovery time (Hughes et al., 2003), although there is a higher risk of relapse following treatment with IVIg.

Rehabilitation Currently, no studies address long-term rehabilitation outcome or comparisons of different methods of treatment (Meythaler, 1997). Once medically stable, most patients require medical rehabilitation. Approximately 40% of patients with GBS need inpatient rehabilitation, particularly patients requiring ventilatory support, more severe disability, and medical complications (Meythaler). Medical complications from GBS such as dysautonomia, cranial nerve involvement, early de-afferent pain syndromes (Meythaler, 1997), and complications resulting from immobility, pneumonia, deep vein thrombus, and musculoskeletal deconditioning (Dhar et al., 2008) can persist for some time and interfere with rehabilitation.

Guillain–Barre´ Syndrome. Table 3 Proposed electrodiagnostic criteria for demyelination of peripheral nerve (Reprinted with permission from Asbury & Cornblath (1990)) These criteria concern nerve conduction studies (including proximal nerve segments) in which the predominant process is demyelination. Must have three of the following four features: 1. Reduction in conduction velocity in two or more motor nerves (a) 80% of LLN (b) 15% change in duration between proximal and distal sites and >20% drop in negative-peak area or peak-to-peak amplitude between proximal and distal sites 3. Prolonged distal latencies in two or more nerves (a) >125% of upper limit or normal (ULN) if amplitude > 80% of LLN (b) >150% of ULN if amplitude < 80% of LLN 4. Absent F-waves or prolonged minimum F-wave latencies (10–15 trials) in two or more motor nerves (a) >120% of ULN if amplitude > 80% of LLN

Supportive Ventilatory Care Up to 30% of patients develop respiratory failure and pneumonia in the first 12 weeks of illness and many will recover adequately (Ropper, 1992). Those requiring ventilatory support have longer hospital lengths of stay and increased rehabilitation costs (Meythaler, 1997). Patients are monitored closely in rehabilitation settings for signs of respiratory distress. Intubation is required if the vital capacity decreases to < 18 ml/kg, maximum inspiratory and expiratory pressures are < than 30 cmH2O and < than 40 cmH2O, respectively


Guillain–Barre´ Syndrome. Table 4 Differential diagnoses of GBS Acute polyneuropathy Vasculitis Lyme disease Porphyria Sarcoidosis Paraneoplastic disease Acute arsenic poisoning Critical illness Diseases of the spinal cord Spinal cord compression Transverse myelitis Diseases of the neuromuscular junction Botulism Myasthenia gravis Lambert–Eaton syndrome Diseases of muscle Acute polymyositis

(Lawn, Fletcher, Henderson, Wolter & Wijdicks, 2001). Weaning from ventilation depends on improvements in strength and serial lung function testing; tracheostomy may be required if lung function tests show no improvements from baseline at the 2–3 week mark (Hughes et al., 2003). Pulse oximetry and BiPAP may be indicated at night-time for patients with hypoxia or hypercapnia. Physical therapy addresses clearance of respiratory secretions, reduction in the work of breathing, breathing exercises, and resistive inspiratory training. Special weaning protocols are required to prevent over fatigue of respiratory muscles in tracheostomy patients (Meythaler, 1997). Those with cranial nerve involvement are at risk of aspiration and respiratory complications.

Deep Venous Thrombus (DVT) Pulmonary embolism can occur in up to a third of patients with GBS (Raman, Blake, & Harris, 1971). The exact incidence or risk factors for DVT in GBS are unknown. Although prophylaxis (such as subcutaneous heparin and support stockings) is recommended, the type and length of prophylaxis is unclear. In rehabilitation settings, early mobilization using progressive mobilization strategies for improving bed mobility, practising safe transfer techniques, and gait training is encouraged.

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Dysautonomia Dysautonomia can occur in up to 70% of patients with GBS and is associated with significant morbidity and mortality, need for mechanical ventilation (Dhar et al., 2008), and cardiac arrthymias (Zochodne, 1994). It extends duration of acute care (Zochodne) and rehabilitation (Meythaler, 1997). Close monitoring of fluid balance, blood pressure, cardiac rhythm, and sensitivity to vasoactive medications is essential in managing these patients and should continue until adequate recovery is under way (Meythaler). Rehabilitation involves education of staff, patients, and their families, use of compression socks, adequate hydration, postural training, and use of tilt tables where appropriate, to allow for cardiovascular and autonomic adaptation. Bladder (disturbed sensation and areflexia) (Sakakibara, Hanori, Kuwabara, Vamanishi, & Yasuda, 1997) and bowel dysfunction (ileus) in GBS can occur early and usually resolves. Ileus can be treated with erythromycin or neostigmine, but promotility agents should be avoided (Hughes et al., 2003). Patients with GBS are initially catheterized to avoid bladder distension and to maintain urinary hygiene. Rehabilitation measures ensure social continence and avoidance of complications such as urinary infections, which occur in 30% of patients (Ropper, 1992).

Complications from Immobilization Tilt table training for immobilized patients can be effectively used in rehabilitation units, as prolonged immobilization leads to reduction of blood volume. Early mobilization of patients counters immobilization hypercalcemia (Meythaler, 1997). Graduated mobility programs include maintenance of posture and alignment, joint range of motion, provision of orthotics, endurance and muscle strengthening, flexibility, and progressive ambulation using adaptive gait aids. Strengthening programs should initially be nonfatiguing to avoid paradoxical weakness. Physical measures using neurodevelopmental sequencing, partial body weight support systems, and podiatrons have been reported. Other complications from immobility such as compression nerve palsies and pressure sores can be avoided with appropriate positioning and skin and pressure care protocols. Heterotopic ossification is rare but more likely to occur in ventilated patients. Early recognition and modified therapy with joint ranging within the pain-free arc can be helpful.

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Guilty But Mentally III

Pain Up to 70% of patients can develop musculoskeletal and neuropathic pain (Hughes et al., 2003). Effective medications include: tricyclic antidepressants, tramadol, gabamimetic agents (e.g. ▶ gabapentin), membrane stabilizing agents (e.g. ▶ carbamazepine), and opiates. Treatment includes patient education and desensitization therapy using functional tasks of everyday life (grooming, dressing).

Fatigue Severe fatigue can persist in 80% of patients and is unrelated to age, duration, or severity of initial illness, but probably partly related to muscle deconditioning and forced inactivity. Fatigue is associated with a reduced QoL despite good physical recovery, as many patients remain restricted in daily and social activities. Medications (such as Amantadine) are not effective. Aerobic exercise improves functional outcome, QoL, and fatigue. Energy conservation and work simplification strategies also help manage fatigue and facilitate patient functional independence. Provision of adaptive equipment and aids facilitate personal and domestic activity, by decreasing task-associated energy expenditure.

Cross References ▶ Activities of Daily Living (ADL) ▶ Activity Restrictions, Limitations ▶ Autonomic Nervous System ▶ Cognitive Functioning ▶ Demyelination ▶ Disability ▶ Insight, Effects on Rehabilitation ▶ Interdisciplinary Team Rehabilitation ▶ Neuropsychology ▶ Quality of Life

Forsberg, A., Press, R., Einarsson, U., de Pedro-Cuesta, J., & Holmqvist, L. W. (2005). Disability and health related quality of life in GuillainBarre´ syndrome during the first two years after onset: A prospective study. Clinical Rehabilitation, 19, 900. Hughes, R. A., Wijdicks, E. F., Barohn, R., Benson, E., Cornblath, D. R., Hahn A. F., Meythaler, J. M., Miller, R. G., Sladky, J. T., & Stevens, J. C. (2003). Practice parameter: immunotherapy for Guillain-Barre´ syndrome: Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology, 61, 736–740. Hughes, R. A. C., Raphae¨l, J-C., Swan, A. V., & van Doorn, P. A. (2006). Intravenous immunoglobulin for Guillain-Barre´ syndrome. Cochrane Database of Systematic Reviews. Lawn, N. D., Fletcher, D. D., Henderson, R. D., Wolter, T. D., & Wijdicks, E. F. M. (2001). Anticipating mechanical ventilation in GuillainBarre´ syndrome. Archives of Neurology, 58, 893. Liu, C. Y., Kao, C. D., Chen, J. T., Yeh, Y. S., Wu, Z. A., Liao, K. K. (2006). Hydrocephalus associated with Guillain-Barre syndrome. Journal of Clinical Neuroscience, 13, 866–869. Meythaler, J. M. (1997). Rehabilitation of Guillain-Barre´ Syndrome. Archives of Physical Medicine and Rehabilitation, 78, 872–879. Pascuzzi, R. M., & Fleck, J. D. (1997). Acute peripheral neuropathy in adults. Neurology Clinics, 15, 529–547. Raman, T. K., Blake, J. A., & Harris, T. M. (1971). Pulmonary embolism in Landry-Guillain-Strohl syndrome. Chest, 60, 555–557. Rees, J. H., Soudain, S. E., Gregson, N. A., & Hughes, R. A. (1995). Campylobacter jejuni infection and Guillain-Barre´ syndrome. New England Journal of Medicine, 333, 1374–1379. Ropper, A. H. (1992). The Guillain-Barre´ syndrome. New England Journal of Medicine, 326, 1130–1136. Sakakibara, R., Hanori, T., Kuwabara, S., Vamanishi, T., & Yasuda, K. (1997). Micturitional disturbance in patients with Guillain-Barre´ syndrome. Journal of Neurology. Neurosurgery and Psychiatry, 63, 649–653. Springhouse. (2005). Professional guide to diseases (8th ed.). Philadelphia, PA: Lippincott Williams & Wilkins. Weiss, H., Rastan, V., Mullges, W., Wagner, R. F., Toyka, K. V. (2002). Psychotic symptoms and emotional distress in patients with GuillainBarre´ syndrome. European Neurology, 47, 74–78. Zochodne, D. W. (1994). Autonomic involvement in Guillain-Barre´ syndrome: A review. Muscle Nerve, 17, 1145–1155.

Guilty But Mentally III N ATHALIE D EFABRIQUE Cook County Department of Corrections Chicago, IL, USA

References and Readings Synonyms Asbury, A. K., & Cornblath, D. R., Assessment of current diagnostic criteria for Guillain-Barre syndrome. Annals of Neurology, 27 (Suppl):S21–24. Bersano, A., Carpo, M., Allaria, S., Franciotta, D., Citterio, A., & NobileOrazio, E. (2006). Long term disability and social status change after Guillain-Barre´ syndrome. Journal of Neurology, 253, 214–218. Dhar, R., Stitt, L., & Hahn, A. F. (2008). The morbidity and outcome of patients with Guillain-Barre´ syndrome admitted to the intensive care unit. Journal of Neurological Sciences, 264, 121–128.

Legal insanity

Definition Guilty but mentally ill is a verdict available in some jurisdictions in cases involving an insanity defense in

Gulf War Syndrome

which the defendant is considered guilty but is committed to a mental hospital rather than imprisoned. This plea is utilized if an examination shows a need for psychiatric treatment, and the metal illness interfered with the person’s ability to determine right from wrong. In general, a person who pleads guilty but mentally ill may do so if the trier of fact finds that during the time of the incident, the person was mentally ill at the time of the offense beyond a reasonable doubt. In order to proceed with the plea, the person who chooses guilty but mentally ill waives the right to trial if it is accepted by the judge. The judge will consider the rules of criminal procedure and hold a hearing for the sole purpose of determining whether sufficient evidence can be presented indicating that the defendant was mentally ill at the time of the offense. If the judge refuses to accept the plea of guilty but mentally ill, the defendant may withdraw the plea and is then entitled to a jury trial. If the defendant waives the right to a jury trial, the judge who presided at the hearing on mental illness may not preside at the subsequent trial. To be considered mentally ill by the courts, the individual lacks significant capacity to either appreciate the wrongfulness of his actions or to obey the rules of conduct to the requirements of the law as a result of the mental disease or defect.

Cross References ▶ Insanity

References and Readings Brown, M. (2007). The John Hinckley trial & its effect on the insanity defense. Dalby, J. T. (2006). The case of Daniel McNaughton: Let’s get the story straight. American Journal of Forensic Psychiatry, 27, 17–32. Ellis, J. W. (1986). The consequences of the insanity defense: Proposals to reform post-acquittal commitment laws. Catholic University Law Review, 35, 961. Schmalleger, F. (2001). Criminal justice: A brief introduction. Saddle River, NJ: Prentice Hall. ISBN 013396731X. Walker, N. (1985). The insanity defense before 1800. The Annals of the American Academy of Political and Social Science, 477, 25. doi:10.1177/0002716285477001003. at p.30.

Guilty Mind ▶ Mens Rea

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Gulf War Illness (GWI) ▶ Gulf War Syndrome

Gulf War Syndrome G UDRUN L ANGE UMDNJ-New Jersey Medical School Newark, NJ, USA

G Synonyms Gulf war illness (GWI); Medically unexplained symptoms (MUS)

Short Description or Definition Almost 700,000 US service personnel served in Operations Desert Shield and Desert Storm in 1990 and 1991 respectively. Following their service, many veterans who returned reported persistent medical and psychiatric symptoms including chronic fatigue, rash, headache, arthralgias/ myalgias, gastrointestinal complaints, impotence, sleep problems, difficulties in concentrating, memory loss, irritability, nervousness, tenseness, depressed mood, and other emotional changes. This constellation of symptoms has been called Gulf War Syndrome (GWS) or Gulf War Illness (GWI). Symptoms reported by some Gulf War veterans generally affect multiple organ systems and are not consistently associated with any objective physical signs or similar laboratory abnormalities. Symptom profiles are similar to those of other symptom-based conditions such as CFS and fibromyalgia (FM) and irritable bowel syndrome (IBS). A specific case definition for GWS does not exist. Since the symptom profile of GWS closely resembles that of Chronic Fatigue Syndrome (CFS), the disorder is often clinically diagnosed using the 1994 case definition for CFS (Fukuda, Straus, Hickie, Sharpe, Dobbins, & Komaroff, 1994) or alternatively the Chronic Multisymptom Illness case definition created by a group of epidemiologists and defined as having one or more chronic symptoms (present for 6 months) from at least two of the following categories: fatigue; mood and cognition (symptoms of feeling depressed, difficulty in remembering or concentrating, feeling moody, feeling anxious, trouble finding words, or difficulty sleeping); and musculoskeletal (symptoms

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of joint pain, joint stiffness, or muscle pain). This case definition is published in the VA/DoD Clinical Practice Guidelines (http://www.oqp.med.va.gov/cpg/cpgn/mus/ mus_base.htm).

Categorization While significant controversy initially surrounded the presence and definition of GWS, the condition is now accepted as a consequence of service in the Persian Gulf War. The Research Advisory Committee on Gulf War Veterans’ Illnesses released a report in 2004 stating that between 25% and 30% of veterans who served in the 1991 Persian Gulf War suffer from symptoms consistent with GWS. Gulf War Registries were established in the USA, Great Britain, and Canada to provide health evaluations for concerned Persian Gulf Veterans.

Epidemiology Baseline data do not exist; therefore, the Department of Veterans Affairs funded a cross-sectional populationbased epidemiological study called the National Health Survey of Gulf War Era Veterans and Their Families (Kang, Mahan, Lee, Magee, & Murphy, 2000). The study sample consisted of 15,000 US service members and 15,000 era veterans (i.e., veterans of the same time period who were not deployed to the Gulf War). Results showed that Gulf War veterans report higher prevalence of functional impairment, healthcare utilization, symptoms and medical conditions, and a higher rate of low general health. In 2004, the Research Advisory Committee on Gulf War Veterans’ Illnesses concluded that Persian Gulf War Veterans suffered from a higher rate of neurological disorders and that neither psychiatric conditions nor stress related to exposure to war could fully explain the multiple health symptoms experienced by veterans of that conflict.

Natural History, Prognostic Factors, Outcomes Like symptom-based illnesses that significantly overlap with GWS including FM, CFS, depression, IBS, headache, Posttraumatic Stress Disorder (PTSD), and panic disorder, the pathophysiology of the condition is still unknown. Much research has been devoted to solve causal

controversies and to better understand the condition experienced by troops deployed during the 1991 Persian Gulf War. Ideas range from the condition being a somatic manifestation of wartime stress-related psychiatric illnesses, such as PTSD (Sutker, Uddo, Brailey, & Allain, 1993), to its being due to exposure to low doses of organophosphate nerve gases (Haley, Kurt, & Hom, 1997). Data supporting a psychiatric etiology for GWS in deployed symptomatic Persian Gulf Veterans include high prevalence rates of psychiatric disorders, particularly PTSD. Another possibility could be exposure to environmental toxins, specifically organophosphates. Reports of neurological abnormalities, such as raised thermal and vibratory thresholds, generally impaired audio vestibular function, and increased mean somatosensory and brain stem auditory evoked potential latencies in veterans who have served during the Gulf War conflict support an organic etiology.

Neuropsychology and Psychology of Gulf War Syndrome Gulf War Syndrome is a heterogeneous disorder, similar to CFS, with a similar neuropsychological profile. Cognitive deficits have been found on tasks requiring attention, concentration, information processing, and the use of abstract concepts. Data show that the presence of psychiatric conditions common after deployment (i.e., depression, posttraumatic stress disorder) cannot completely account for the relatively poor cognitive performance observed in some Persian Gulf Veterans.

Evaluation The Department of Veterans Affairs and the Department of Defense have published clinical practice guidelines (http://www.oqp.med.va.gov/cpg/cpgn/mus/mus_base.htm) to assist clinicians evaluating Persian Gulf Veterans who suffer from ill-defined symptoms consistent with GWS, also called MUS. A neuropsychological testing battery for individuals with GWS is similar to that used in CFS. No deficits are expected in overall intellectual functioning, language, and visual perceptual function, relatively weaker performances might be seen or on tasks assessing attention, information processing, working memory. Cognitive assessment should include measures of overall current and premorbid cognitive function (i.e., Wechsler Test of Adult

Gyrus Dentata

Reading, Wechsler Adult Intelligence Scale III/IV), simple and complex attention as well as information processing and working memory (i.e., Continuous Performance Test, Gordon, Trails, Paced Auditory Serial Addition Test), executive function (i.e., subtests of Delis–Kaplan Executive Function System including verbal fluency, towers test; Wisconsin Card Sort Test), memory (i.e., Wechsler Memory Scale III/IV, California Verbal Learning Test II, Rey Osterrieth Complex Figure Test), language (i.e., Boston Naming Test), visual-perceptual function (i.e., Judgment of Line Orientation, Hooper), and motor function (i.e., grip strength, finger tapping, grooved pegboard). It is also recommended to test the level of motivation and effort expanded during neuropsychological testing as well as emotional functioning to improve interpretability of test results.

Fukuda, K., Straus, S. E., Hickie, I., Sharpe, M. C., Dobbins, J. G., & Komaroff, A. (1994). The chronic fatigue syndrome: a comprehensive approach to its definition and study. International Chronic Fatigue Syndrome Study Group. Annals of Internal Medicine, 121, 953–959. Haley, R. W., Kurt, T. L., & Hom, J. (1997). Is there a Gulf War Syndrome? Searching for syndromes by factor analysis of symptoms. JAMA, 277, 215–222. Kang, H. K., Mahan, C. M., Lee, K. Y., Magee, C. A., & Murphy, F. M. (2000). Illnesses among United States veterans of the Gulf War: a population-based survey of 30,000 veterans. Journal of Occupational and Environmental Medicine, 42, 491–501. Sutker, P., Uddo, M., Brailey, K., & Allain, A. (1993). War-zone trauma and stress-related symptoms in OperationDesert Shield/Storm (ODS) returnees. Journal of Social Issues, 49, 33–49.

Gustation ▶ Taste

Cross References ▶ Chronic Fatigue Syndrome

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Treatment Similar to the treatment for CFS, fibromyalgia, and other multi-symptom illnesses, treatment needs to be individualized and should include behavioral, pharmacological, and physical interventions. Cognitive behavioral therapy (CBT) using either a behavior modification or cognitive restructuring approach has found to be helpful. Medication management is similar to that used to treat Chronic Fatigue Syndrome, Fibromyalgia, Irritable Bowel Syndrome, Depression, and Anxiety. Graded aerobic exercise training is a safe and effective treatment for GWS resulting in increased physical conditioning, increase in physical functioning, and quality of life.

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GWB ▶ General Well-Being Schedule

GWBS ▶ General Well-Being Schedule

Gyrus Dentata ▶ Dentate Gyrus

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H H.M.; Also the Case of H.M., Molaison, Henry (1926–2008) A MY A LDERSON Emory University/Rehabilitation Medicine Atlanta, GA, USA


was separated from declarative/explicit learning, and therefore linked to other neural substrates. Not only were ideas about learning and memory challenged, but the relationship between memory and intelligence as a whole was also disputed. HM’s intact intelligence, in the face of his severely impaired learning, firmly argued for the separation of neuropsychological processes, as well as the neural substrates underlying these abilities. In this manner, HM heralded a new era of neuroscientific thinking and investigation.

Short Biography Investigations of Henry Molaison’s (HM) neurocognitive functioning following his surgical intervention have revolutionized our understanding of learning and memory processes. HM’s specific surgical intervention and associated cognitive impairments provided information not only about differential memory activities but also about the neural substrates involved in the mediation of these processes. Investigations of the alterations in HM’s memory led to a landmark paper by Brenda Milner, a psychologist working with HM, and William Scoville, his neurosurgeon. The paper, entitled Loss of recent memory after bilateral hippocampal lesions, was published in the Journal of Neurology, Neurosurgery, and Psychiatry in 1957; it brought about a sea change in the understanding of the neural substrates of memory. This paper has been cited more than 1,800 times since its initial publication. In this landmark paper, as well as in many others featuring HM’s surgical resection and associated cognitive impairments, the relationship between the mesial temporal lobe and the formation of new episodic and semantic memories was established. HM set the stage for a new generation of research investigating the mesial temporal lobe as the neural substrate for specific types of memory. The mesial temporal lobe was now associated with the commitment of new information to long-term memory, spatial learning, and factual learning. For the first time, procedural learning – including skill learning and conditioning –

Henry Molaison, also known as H.M. or Henry M., was born on February 26, 1926 to middle-class parents in Manchester, CT. When he was 9-years old, he was involved in a bicycle accident, sustaining a laceration of the left supra-orbital region with an approximate 5-min loss of consciousness. Shortly thereafter, around the age of 10, he began experiencing complex partial epileptic seizures, with a semiology that consisted of crossing both arms and legs, closing his eyes, and ‘‘half hearing what was going on.’’ Around his 16th birthday, he experienced his first generalized tonic–clonic seizure. His generalized seizures were accompanied by urinary incontinence, tongue biting, loss of consciousness, and prolonged somnolence. HM dropped out of high school shortly after he turned 16, in large part due to the effects of his seizures. Then, after a 2-year break and additional anti-epileptic medications, he returned to high school and was able to graduate on a vocational track in 1947. Following high school, he first worked on an assembly line where he inserted pieces into typewriters. He later worked as a motor winder in a small electric motor shop, literally wrapping copper wire on the armature of electric motors. By the time he was in his mid-twenties, HM was experiencing up to ten seizures and blackouts each week. His seizures continued, despite being prescribed the maximum dosage of numerous medications. He was unable to sustain his employment as a motor winder because of his seizure frequency. Although HM’s seizures may have been


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related to his bicycle accident and possible traumatic brain injury, he also had a family history of epilepsy, with three of his first cousins also having histories of epilepsy. In 1953, HM was referred to William Scoville, the Director of the Department of Neurosurgery at Hartford Hospital. After localization of seizure activity to the medial temporal lobe, HM underwent bilateral mesial temporal lobe resection on September 1, 1953. His resection extended bilaterally from the temporal pole of the mesial temporal lobe 8 cm backward, completing removing the amygdala, parahippocampal gyrus, entorhinal cortex, perirhinal cortex, and 2/3 of the hippocampus. HM’s surgery was successful in reducing the frequency of his seizures – he had about two seizures a year following his surgery. Sadly, he suffered devastating cognitive ramifications of his extensive mesial temporal lobe resection. HM was unable to remember anything that happened after his surgery (anterograde amnesia). He could not commit new information to long-term memory, although he could remember information for very brief periods of time, as long as he was not interrupted. He could be found rereading the same magazines over and over, with no recollection of having read them before. Although he worked with Brenda Milner, his psychologist for over 30 years, he never recalled having met her. He was unable to remember that his father had died or that his family had moved homes. Scientists disagree about the extent of his non-episodic impairment, but there is evidence that he also had limited ability to form new semantic memories or factual information about the world around him. In addition to difficulty learning new information after his surgery, HM was also unable to remember events that occurred right before his surgery. His memory of events occurring in the years immediately preceding his surgery was sometimes poor, but his remote memory for events occurring many years before his surgery was intact (temporally graded retrograde amnesia). Despite his limitations in learning new facts and information, HM could still learn new motor skills and other types of procedural memories without difficulty, but he had no recollection of the learning experience. He was able to learn several skills, such as mirror tracing, but he had to be reminded that he knew them. He was able to hold a simple job at a residential home with these acquired skills. Although HM had severe amnestic dysfunction, investigations of his other cognitive skills revealed an above average IQ of 118. His language skills, including comprehension, grammar, and word knowledge were also intact. Following his surgery HM continued to live with his family. After his father’s death in 1967, he attended a day rehabilitation workshop for 10 years. He continued to live

with his mother until 1977; she passed away in 1981 at the age of 94 in a nursing home. HM then moved to a nursing in Hartford, Connecticut in 1980, where he enjoyed crossword puzzles and detective shows. He continued to travel to MIT to participate in studies of his memory. After years of contribution to the field of neuroscience, HM died from respiratory failure on December 2, 2008 at the age of 82. His death was confirmed by Dr. Suzanne Corkin, a neuroscientist that had worked closely with him for decades.

Cross References ▶ Amnesia ▶ Milner, Brenda Atkinson (1918– ) ▶ Memory

References and Readings Corkin, S. (2002). Whats new with amnesic patient H.M.? Nature Reviews Neuroscience, 3, 153–160. Ogden, J. A. (2005). Fractured minds. New York: Oxford University Press. Scoville, W. B. (1968). Amnesia after bilateral mesial temporal-lobe excision: Introduction to case H.M. Neuropsychologica, 6, 211–213. Scoville, W. B., & Milner, B. (1957). Loss of recent memory after bilateral hippocampal lesions. Journal of Neurology, Neurosurgery, and Psychiatry, 20, 11–21. Scoville, W. B., & Milner, B. (2000). Loss of recent memory after bilateral hippocampal lesions. Journal of Neuropsychiatry and Clinical Neuroscience, 12(1), 103–113.

H2 ▶ Heritability

Habituation R ONALD A. C OHEN Brown University Providence, RI, USA

Definition The process by which there is a decrease in behavioral response (orienting response) to a stimulus that is repeatedly presented over time. Habituation occurs in most

Habituation

animal species from simple invertebrates to humans. Habituation typically involves an attenuation of autonomic and motor response, as well as underlying neural processes that support these responses. As habituation occurs to a novel stimulus, there is typically a shift of attention away from that stimulus to other potentially more relevant stimuli.

Historical Background Habituation was first conceptualized in the context of biological theories of adaptation and evolution of the nineteenth century. Behaviors that are rewarding to an animal and increase its survivability will be repeated, and eventually formed habits of varying strengths. This reflects acclimation, which is adaptive as it enables animals to respond to a changing environment. The concept of habituation was formalized in the classical conditioning research of Pavlov and Anrep (2003), who observed that animals typically show an initial behavioral and physiological response to novel stimuli, such as a light or noise, even though they do not have any significance in the environmental context. He labelled this as an orienting response (OR), and distinguished it from unconditioned responses (UCR) that occur to stimuli that have intrinsic biological value, such as food, water, shock, or threat. This type of stimuli was referred to as an unconditioned stimulus (UCS). An UCR will typically continue to elicit a physiological and behavioral response, unless there is a decrease in the animal’s motivational state. For example, a hungry animal will continue to salivate to food exposure when it is hungry regardless of the number of exposures. Decrease in the strength of the UCR is a function of appetitive state, not the representation of the stimulus. In contrast, stimuli that do not have such intrinsic value, but that are still salient enough to be detected in the environment tend to elicit an OR that may have some of the same characteristics of an UCR, though usually at reduced magnitude. However, unlike an UCR, the OR will tend to decrement with repeated exposure, reflecting the fact that increasingly, the animal does not experience the stimulus as meaningful. This decrementing was defined as habituation. Pavlov observed that when a stimulus that elicits an OR is associated in time with a UCS, it eventually begins to take on some of the salience of the UCS, so that over time the orienting stimulus gains associative strength through the S-S pairing, such that repeated exposure to this stimulus no longer habituates, as long as the animal is rewarded with the UCS. Eventually, the orienting

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stimulus becomes a conditioned stimulus (CS) and elicits conditioned responses, the simplest form of associative learning. The neural processes underlying habituation are fundamental to almost all animal systems. Habituation occurs automatically, often without conscious awareness. Habituation enables the animals to passively shift behavioral response relative to environmental changes and new available information, without the need for higher-order cognitive processing, making it an excellent model for simple forms of attention. Furthermore, the relationship between the habituation of the OR and classical conditioning provided an obvious area bridge between attention, learning and memory formation. The pioneering work of Pavlov spurred a tremendous amount of research aimed at understanding the stimulus and behavioral parameters that influenced the process of habituation and its relationship to conditioning, as well as investigations into the neural substrates of habituation. Behavioral researchers used these concepts to develop models for how stimuli achieve cue dominance and relative information value. Habituation probably had its greatest historical influence in the field of psychophysiology, where this process became a foundation of efforts to link cognitive and emotional behavior with physiological response. Sokolov proposed one of the first neuropsychological theories of habituation, in which he proposed that habituation results from comparison of new stimuli with existing ‘‘neuronal’’ models of stimuli (2002). He posited that the hippocampus played a critical role in this process. Groves and Thompson (1970) proposed a dual process model in which habituation and sensitization were conceptualized as two elementary and competing physiological responses that always occur following sensation. The dual process theory differed from the neuronal theory proposed by Sokolov in that it accounted for simple forms of habituation occurring across animal species without the involvement of higher cortical processes. Whereas habituation is the byproduct of passive decrementing of sensory response, according to the dual process theory, it involves a more active comparator process in Sokolov’s theory. Subsequently, Waters and his colleagues (1979) integrated these two theories, arguing that in humans there are both passive processes of sensory decrementing and also active inhibitory processes that together provide the basis for selective attention.

Current Knowledge For neuroscientists, habituation and sensitization provide ideal simple model systems for studying the neural

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bases of simple learning. These efforts are reflected in the Nobel prize winning research of Eric Kandel that demonstrated neurobiological distinctions between sensitization, habituation, and long-term potentiation, which provides the foundation for conditioning at a cellular level in an a simple organism (Kandel, 1978; Kandel & Schwartz, 1982). This work essentially extended Grove and Thompson’s dual process model by showing that habituation, sensitization and conditioning could be viewed on a continuum, with conditioning requiring the addition of processes that enabled longer term storage. In sum, habituation plays a central role in modern neuroscientific approaches to learning at a cellular and neuronal level, and also provides a conceptual link between sensation, attention, and learning. The cellular bases for sensitization, habituation, and conditioning are now relatively well understood. Yet, this level of analysis does not fully explain the behavioral phenomena of human orienting and habituation. One of the essential features of orienting and habituation in humans is that it typically occurs as a response complex that involves the muscular-skeletal, autonomic, and central nervous systems. The object of habituation is always diminished response, which typically involves decreased cardiovascular, electrodermal, ocular, and motoric response with repeated exposure. The responses of these different systems seem to be integrated during habituation, suggesting that in humans cerebral processes are playing some role. This fact also provides for a clear link between these basic behavioral processes and higher cognitive functions, most notably attention. Habituation contrasts with the process of sensitization, another form of non-associative learning during which repeated stimulation leads to an amplification of the sensory experience. There are a variety of other related phenomena associated with habituation that have bearing on attention. After habituation has occurred, stimulus re-presentation often elicits a spontaneous recovery of the OR. Re-habituation usually occurs rapidly following spontaneous recovery. The presentation of a novel stimulus often results in a recovery of the OR (i.e., dishabituation), though usually at a reduced level compared to the initial OR. Dishabituation illustrates the sensitive to nature of the habituation process to varying stimulus conditions and changes in response set. A vast psychophysiological research literature has evolved over the past 4 decades, such that the stimulus parameters and experimental factors that affect elicitation of the OR and its habituation are very well understood in humans. Factors that influence habituation of the OR include: (1) Stimulus intensity; (2) Stimulus duration;

(3) Stimulus information; (4) Signal value; (5) Interstimulus interval; and (6) the total number and duration of stimulus presentation (Siddle, 1983). In general, greater intensity, duration, and signal value of a stimulus will result in a larger initial OR and greater resistance to complete habituation. While the original concept of habituation proposed by Pavlov related to signals without intrinsic value as conditioned stimuli, in the current experimental literature, the construct has been broadened to include a variety of stimulus types that are not tied to primary learning paradigm. For example, orientation to words presented prior to an experimental task will habituation, if the words have no relevance to the task. Yet, words have semantic value, which will affect the rate of habituation, whether they are task relevant or not. Changes in stimulus characteristics will also affect habituation rate. While presenting loud sounds would normally increase resistance to habituation, when a soft tone is introduced into a string of louder tones, the softer tone can actually cause dishabituation. Similarly changing the sensory modality of stimulation will typically slow habituation, even if the semantic value of the stimulus is essentially same. The more novel a stimulus is the slower a person will be to habituate to it. While the functional significance of the habituation was originally thought to relate primarily basic conditioning, it is now obvious that the orienting response and habituation play important roles in higher cognitive functions as well.

Neuropsychological and Clinical Considerations Habituation rate is sensitive to overall severity of brain dysfunction. Patients with advanced Alzheimer’s disease typically exhibit marked abnormalities of both the OR and habituation. The degree of impairment is a function of the severity of dementia and extent of structural brain damage. Patients with Parkinson’s disease also show abnormalities of both the OR and habituation, reflecting disturbance of dopamine systems of the basal ganglia, and which have been tied to related problems with attention and executive control. Focal brain lesions have more selective effects on habituation. For example, the hippocampus was postulated by Sokolov (1975) to play a critical role in elicitation and habituation of the OR. Yet, the OR is preserved and habituates when the hippocampal function is suppressed in patients with temporal lobe epilepsy undergoing posterior cerebral artery amobarbital testing (Cohen, 1993). In contrast, suppression of the amygdale, medial temporal and frontal areas of the brain through

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Habituation. Figure 1 Abnormal habituation of the skin conductance responce (SCR) is shown for patients with brain disturbances involving the frontal cortex. Compared to age-matched healty controls, individuals who had experienced stroke affecting the frontal cortex and also one case of frontal-temporal dementia fail to habituation over the course of twelve successive presentations of an auditory tone, illustrating the important role that the frontal cortex plays in the process of habituation

the standard middle cerebral WADA procedure result in a loss of the skin conductance OR. Therefore, the amygdale seems to actually be more important to these processes in humans. The frontal cortex plays a particularly important role in maintenance of habituation, and damage to prefrontal and also paralimbic areas (e.g., ▶ anterior cingulate cortex) results disturbances of habituation (see Fig. 1). Damage to brain areas responsible for arousal and activation (e.g., reticular system), also affect the OR. In fact, there is a strong correspondence between brain regions that have been implicated in control of attention and those that affect habituation. The phenomenon of sensory extinction that is commonly observed in patients with neglect syndrome has been linked to the processes of orienting and habituation, forming one of the cornerstones of the attention-arousal theory of neglect syndrome (Heilman & Valenstein, 1979).

Future Directions Habituation is an important concept in the field of neuroscience, and there are a large number of experimental neuropsychological studies that have employed habituation paradigms to study the role of particular brain systems on these basic behavioral processes, attention and

memory. Yet, psychophysiological methods are rarely employed in clinical settings to assess whether impaired attention is associated with alterations of habituation. The OR and its habituation have the potential to be useful behavioral biomarkers in clinical assessment. The relationship of the habituation to dynamic changes in functional brain response measured by through neuroimaging methods, such as FMRI, also promises to be a fertile area of future investigation.

Cross References ▶ Arousal ▶ Attention ▶ Automaticity ▶ Extinction ▶ Orienting Response

References and Readings Cohen, R. A. (1993). Neuropsychology of attention. New York: Plenum Publishing. Cohen, R. A., Kaplan, R. F., Meadows, M. E., & Wilkinson, H. (1994). Habituation and sensitization of the orienting response following bilateral anterior cingulotomy. Neuropsychologia, 32, 609–617.

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Groves, P. M., & Thompson, R. F. (1970). Habituation: A dual-process theory. Psychological Review, 77, 419–450. Kandel, E. R. (1978). A cell-biological approach to learning. Bethesda, MD: Society for Neuroscience. Kandel, E. R., & Schwartz, J. H. (1982). Molecular biology of learning: Modulation of transmitter release. Science, 218, 433–443. Pavlov, I. P., & Anrep, G. V. (2003). Conditioned reflexes. Mineola, NY: Dover Publications. Pribram, K. H., Reitz, S., McNeil, M., & Spevack, A. A. (1979). The effect of amygdalectomy on orienting and classical conditioning in monkeys. Pavlovian Journal of Biological Science, 14, 203–217. Siddle, D. (1983). Orienting and habituation: Perspectives in human research. New York: Wiley. Sokolov, E. N. (1975). Neuronal mechanisms of the orienting reflex. In E. N. Sokolov & O. S. Vinogradova (Eds.), Neuronal mechanisms of the orienting reflex (pp. 217–235). Hillsdale, NJ: Erlbaum and Associates. Waters, W. F., McDonald, D. G., & Koresko, R. L. (1977). Habituation of the orienting response: A gating mechanism subserving selective attention. Psychophysiology, 14, 228–236.

References and Readings Brunton, L. L., Lazo, J. S., & Parker, K. L. (Eds.). (2006). Goodman & Gilman’s The pharmacological basis of therapeutics (11th ed.). New York: McGraw-Hill. Cooper, J. R., Bloom, F. E., & Roth, R. H. (Eds.). (2003). The biochemical basis of neuropharmacology (8th ed.). New York: Oxford University Press. Davis, K. L., Charney, D., Coyle, J. T., & Nermeroff, C. (Eds.). (2002). Neuropsychopharmacology: The fifth generation of progress. Philadelphia, PA: Lippincott Williams & Wilkins. Stahl, S. M. (2008). Stahl’s essential psychopharmacology: Neuroscientific basis and practical applications (3rd ed.). New York: Cambridge University Press. Voet, D., Voet, J. G., & Pratt, C. W. (Eds.). (2008). Fundamentals of biochemistry (3rd ed.). New Jersey: Wiley.

Hallucination

Half-Life J OSE A. R EY Nova Southeastern University Ft. Lauderdale, FL, USA

Definition The half-life of a drug is the time it takes for the blood or plasma concentration of a given drug to be reduced by 50%. The formula for calculating this value is: t1/2 = 0.693·Vss/CL This pharmacokinetic value is also important for determining the time to steady state concentrations in the body. It takes approximately five half-lives for a drug to achieve steady state plasma concentrations in the body, which signifies that the amount of the drug being eliminated from the body is equal to the amount of the drug being given. Subsequently, a medication usually requires approximately five half-lives for a significant majority (~97%) of the drug to be eliminated from the body after discontinuation. The half-life of a drug may influence or dictate the daily dosage regimen of a particular agent and also provide an indication of the expected time to the onset of withdrawal/discontinuation symptoms to occur if the drug is associated with withdrawal/discontinuation symptoms.

PAUL N EWMAN Drake Center Cincinnati, OH, USA

Definition A sensory perception in the absence of an external stimulus. Hallucinations are often differentiated from sensory illusions which are distortions or misinterpretations of actual sensory experiences. Hallucinations can involve any sensory modality (visual, auditory, tactile, olfactory, or gustatory). Simple (unformed) hallucinations are sensory perceptions that are typically vague and without meaning (e.g., whistling sounds, flashing lights, geometric patterns). In complex (formed) hallucinations, the perceptual experience generally concerns objects, people, or animals (e.g., hearing voices, seeing animals, or tasting chocolate). Common causes of hallucinations include:

Psychosis (resulting from delirium or psychotic disorder) Cerebral lesions (especially related to ictal discharge) Amputation (phantom pain) Certain dementias (e.g., ▶ Lewy Body)

Cross References ▶ Delirium ▶ Dementia with Lewy Bodies

Halstead Impairment Index

▶ Epilepsy ▶ Psychosis

References and Readings Tenkin, S., & Cummings, J. L. (2003). Hallucinations and related conditions. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed.). New York, NY: Oxford University Press. Capruso, D. X., Hamsher, K. D., & Benton, A. L. (1998). Clinical evaluation of visual perception and constructional ability. In P. J. Snyder & P. D. Nussbaum (Eds.), Clinical neuropsychology: A pocket handbook for assessment. (pp. 521–540). Washington, DC: American Psychological Association. American Psychiatric Association (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: American Psychiatric Association.

Haloperidol J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metairie, LA, USA

Generic Name Haloperidol

Brand Name Haldol

Class

Blocks dopamine 2 receptors

Bipolar disorder, behavioral disturbances related to dementia

Side Effects Serious Agranulocytosis, seizures, leucopenia, neuroleptic malignant syndrome

Common Akathisia, neuroleptic-induced deficit syndrome, extrapyramidal symptoms, galactorrhea, amenorrhea, dizziness, dry mouth, weight gain, hypotension



Halstead Impairment Index DANIEL N. A LLEN University of Nevada Las Vegas Las Vegas, NV, USA

Indication Psychotic disorders, Tourette’s syndrome (tics and vocal utterances), severe behavior problems, hyperactivity, schizophrenia

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Halstead Impairment Index

Description The Halstead Impairment Index is a summary index of brain damage that is derived from seven of the core test scores from the Halstead-Reitan Neuropsychological Battery (HRNB). Test scores that contribute to the index include the Category Test errors, Tactual Performance Test total time, Tactual Performance Test Localization score, Tactual Performance Test Memory score, Rhythm Test ranked score, Speech Sounds Perception Test errors, and the Finger Tapping test dominant hand performance. The Impairment Index ranges from 0.0 to 1.0 and is calculated in a straightforward manner. A determination is first made regarding how many of the seven test scores fall beyond the cutoff score for impairment. Then, the total number of test scores that fall in the impaired range is divided by the total number of test scores (i.e., 7). For example, if a patient were to score in the brain-damaged range on all seven of the tests, the Impairment Index would be 1.0 (7 impaired scores divided by 7 scores available), or, alternatively, if all of the test scores were in the normal range, the Impairment Index would be 0.0 (0 impaired scores/7 scores available). If some of the tests in the HRNB cannot be administered, a pro-rated Impairment Index can be calculated in a similar manner using the test scores that are available. For example, if six test scores are available and three of those scores fall in the impaired range, the prorated Impairment Index would be 0.5 (3 impaired scores/6 scores available).

limitation of the Impairment Index is that it relies on only seven of the many possible test scores that can be obtained from the HRNB. Because of these limitations, Reitan suggested an alternative approach, the General Neuropsychological Deficit Scale (GNDS). Unlike the Impairment Index, the GNDS is calculated based on 42 variables that are derived from all of the cores tests from the HRNB, including the Wechsler Adult Intelligence Scale. Additionally, although the Impairment Index relies solely on level of performance as an indicator of cerebral damage, the GNDS also incorporates pattern of performance, lateralization effects, and pathognomonic signs that may be indicative of brain damage. Thus, although the Impairment Index has been well validated and is still used in clinical practice, the GNDS provides a more comprehensive and possibly more sensitive indicator of the status of the brain.

Cross References ▶ Category Test ▶ Finger Tapping Test ▶ Glasgow Coma Scale ▶ GOAT ▶ Halstead-Reitan Neuropsychological (HRNB) ▶ Rancho Los Amigos Scale ▶ Seashore Rhythm Test ▶ Tactual Performance Test

Test

Battery

Current Knowledge The Impairment Index repeatedly demonstrated sensitivity to various forms of cerebral damage including cerebrovascular accidents, traumatic brain injury, and dementia, among others. Higher scores on the Impairment Index have been interpreted to indicate more severe cerebral damage, with scores from 0.0 to 0.3 considered within normal limits, a score of 0.4 considered borderline, and scores of 0.5 or greater strongly indicative of brain damage. However, the practice of interpreting high scores as indicative of greater impairment has received some criticism because the Impairment Index actually reflects consistency of impairment (i.e., number of tests beyond a cut-off for brain damage) rather than the severity of impairment on each of those tests. An additional

References and Readings Dikmen, S. S., Heaton, R. K., Grant, I., & Temkin, N. R. (1999). Test– retest reliability and practice effects of Expanded Halstead–Reitan Neuropsychological Test Battery. Journal of the International Neuropsychological Society, 5, 346–356. Loring, D. W., & Larrabee, G. J. (2006). Sensitivity of the Halstead and Wechsler Test Batteries to brain damage: Evidence from Reitan’s original validation sample. Clinical Neuropsychologist, 20, 221–229. Matarazzo, J. D., Matarazzo, R. G., Wiens, A. N., Gallo, A. E., & Klonoff, H. (1976). Retest reliability of the Halstead Impairment Index in a normal, a schizophrenic, and two samples of organic patients. Journal of Clinical Psychology, 32, 338–349. Reitan, R. M. (2006). The comparative effects of brain damage on the Halstead Impairment Index and the Wechsler-Bellevue scale. Journal of Clinical Psychology, 15, 281–285.

Halstead, Ward (1908–1968) Reitan, R. M., & Wolfson, D. (1993). The Halstead-Reitan neuropsychological test battery: Theory and clinical interpretation (2nd ed.). Tucson, AZ: Neuropsychology Press. Russell, E. W. (1992). Reliability of the Halstead Impairment Index: A simulation and reanalysis of Matarazzo et al. Neuropsychology, 6, 251–259.

" Halstead was a serious, deliberate scientist who very care-

fully weighted all aspects of the case before making any judgments. Because of this meticulous and critical appraisal of the methodology of studying the defects following head injuries, he came to the conclusion that none of the then used psychological tests were satisfactory. Accordingly, he adopted his own method of examining which, with some other tests then in use, he incorporated into a battery that he thought gave a better overall picture of the deficits than any of the pencil and paper examinations on the market


Ward Halstead opened a laboratory at the University of Chicago in 1935 for the psychological study of neurological and neurosurgical patients. As a physiological psychologist trained in brain ablation techniques, Halstead brought the meticulous methodology of the animal laboratory to his clinical lab. During the 1930s and 1940s, Halstead worked in conjunction with neurologists and neurosurgeons at the University of Chicago to evaluate patients from a neuropsychological perspective. He devised and tested great many tasks with patients, discarding more than he kept until settling upon a set of tests that eventually formed the nucleus of the Halstead–Reitan Neuropsychological Test Battery (HRNTB). Many of the tests he used with clinical subjects were drawn directly from the animal laboratory. For example, the Category Test, a mainstay of the HRNTB, was inspired by Heinrick Klu¨ver’s work on stimulus generalization in monkeys following brain ablation (Reed, 1985). Halstead reasoned that humans with brain damage might lose their ability to generalize responses across changing sets of stimuli just as frontal lesions seemed to produce this deficit in monkeys. Halstead’s early work was centered on developing a concept that he termed ‘‘biological intelligence.’’ By this, Halstead was referring to the overall integrity and health of the central nervous system (Reed, 1985). Biological intelligence was measured using a battery of 13 tests. An attempt to factor analyze this battery in order to better define biological intelligence failed because of the small numbers of subjects available relative to the number of variables Halstead attempted

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to measure. Nonetheless, Halstead attempted to infer four factors making up biological intelligence. He published his four-factor theory of biological intelligence in a 1947 book entitled Brain and Intelligence. Neither his contemporaries nor subsequent scholars have found much scientific merit in Halstead’s theory (Hartman, 1991). Nonetheless, Halstead’s meticulous methodology has long been recognized. Halstead’s contemporary, the neurosurgeon Earl Walker (quoted in Reed, 1985), observed:

Halstead, Ward (1908–1968) A NTHONY Y. S TRINGER Medicine Emory University Atlanta, GA, USA

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Ralph Reitan came to study with Halstead in 1945. Working in Halstead’s laboratory, he learned and expanded upon the initial battery. After graduating, he established his own laboratory at Indiana University Medical School where over the next 20 years he embarked upon the most thorough attempt to validate a neuropsychological test battery up to that point in time. Further expanding upon Halstead’s work were Reitan’s students, including Hallgrim Kløve at the University of Wisconsin and later at the University of Bergen, Norway; Homer Reed at the New England Medical Center in Boston; and Phillip Rennick at (the now defunct) Lafayette Clinic in Detroit, Michigan. Halstead’s legacy is clearly the battery to which he lent his name. As the first standardized, norm-referenced battery of neuropsychological tests, for decades it was the main instrument in the neuropsychologist’s toolkit and largely dominated the field of clinical assessment. While contemporary neuropsychologists make use of a broader range of tests and procedures, and fewer use the HRNTB as their sole instrument, parts of the battery continue to be employed in clinical and forensic settings.

Education and Training Ward Halstead attended Ohio State University where he earned a masters degree in 1931 and was elected to

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Phi Beta Kappa. He subsequently earned his Ph.D. from Northwestern University for research on the effect of cerebellar lesions on rotational nystagmus. This research did not necessary presage the work to come, as his subjects were pigeons. Nonetheless, the careful methodology of the animal research lab formed the basis of his subsequent work with human patient populations.

Major Appointments

Mid-1930s: Instructor, Department of Medicine, University of Chicago. Published sources differ on the date of Halstead’s initial appointment, but he is known to have established a laboratory in the Chicago School of Medicine by 1935. 1939: Joint appointment in Psychology, University of Chicago. Halstead held joint appointments in Medicine and Chicago until his death in 1968.


Though much admired by his collaborators, Halstead appears to have garnered few official accolades. Some regard the decade in which Halstead founded his laboratory as a period of quiescence in which interest in brain-behavior relations largely disappeared from the scientific agenda (Hebb, 1983). Halstead is rarely mentioned in neuropsychological textbooks (Reed, 1985). Perhaps his highest achievement is the pervasive influence of his tests. As Reed noted in 1985, some or all of the HRNTB was in use in more hospitals, clinics, and practices in North America than any other neuropsychological battery. This continues to be true, for at least parts of the battery, 25 years later.

Short Biography Ward Halstead was born December 31, 1908 in Sciotoville, Ohio. While a college student, he married Elizabeth Lee of New Haven, Connecticut. Though trained essentially as a physiological psychologist, Halstead’s first academic appointment as an instructor in the University of Chicago Department of Medicine thrust him immediately into clinical situations in which he had to evaluate patients with brain lesions. From 1939 until his death in 1969, Halstead held joint appointments in psychology and medicine at the University of Chicago. He had a long and productive career in Chicago. He established what was

arguably the first neuropsychological laboratory in the USA in 1935 (Stringer & Cooley, 2002). His early work led to the publication of Brain and Intelligence in 1947, in which he espoused a four-factor theory of biological intelligence. His book was not well received by his contemporaries (Wechsler, 1958). Halstead’s most enduring legacy has been the Halstead–Reitan Neuropsychological Test Battery. Tragically, Halstead died at age 60, still in the prime of his scholarly career, of amyotrophic lateral sclerosis. Near the end of his life, he was barely able to move and his speech was nearly unintelligible. Visiting him only weeks before his death, his student and colleague Ralph Reitan found Halstead’s bed covered with data sheets and statistical analyses. Reitan (2002) writes this of his last visit with his mentor: " As I commented on his findings, and pointed out their

significance, his attention was rapt and his eyes danced with pleasure. We had a most enjoyable and satisfying visit, even if I did most of the talking! It was the last time I saw Halstead, and I was once again reminded of what a true scientist he was and how devoted he was to our field, all the way to the end

Cross References ▶ Halstead Impairment Index ▶ Halstead-Reitan Neuropsychological Test Battery

References and Readings Halstead, W. C. (1947). Brain and intelligence: A quantitative study of the frontal lobes. Chicago: University of Chicago Press. Hartman, D. E. (1991). Reply to Reitan: Unexamined premises and the evolution of clinical neuropsychology. Archives of Clinical Neuropsychology, 6, 147–165. Hebb, D. O. (1983). Neuropsychology: Retrospect and prospect. Canadian Journal of Psychology, 37, 4–7. Reed, J. (1985). The contributions of Ward Halstead, Ralph Reitan and their associates. International Journal of Neuroscience, 25, 289–291. Reitan, R. M. (2002). The best-laid plans – and the vagaries of circumstantial events. In A. Y. Stringer, E. L. Cooley, & A.-L. Christensen (Eds.), Pathways to prominence in neuropsychology: Reflections of twentiethcentury pioneers. New York: Psychology Press. Stringer, A. Y., & Cooley, E. L. (2002). Neuropsychology: A twentiethcentury science. In A. Y. Stringer, E. L. Cooley, & A.-L. Christensen (Eds.), Pathways to prominence in neuropsychology: Reflections of twentieth-century pioneers. New York: Psychology Press. Wechsler, D. A. (1958). The measurement and appraisal of adult intelligence. Baltimore: Williams & Wilkins.

Halstead–Reitan Neuropsychological Test Battery

Halstead–Reitan Neuropsychological Test Battery DANIEL N. A LLEN University of Nevada Las Vegas, Nevada, USA

Synonyms HRB; HRNB

Description The Halstead–Reitan Neuropsychological Test Battery (HRNB; Reitan & Wolfson, 1993) is the most commonly used fixed battery approach to clinical neuropsychological assessment. The battery consists of the following core tests: Category Test, Tactual Performance Test, Seashore Rhythm Test, Speech-sounds Perception Test, Trail Making Test Parts A and B, Finger Tapping Test, Strength of Grip, Reitan-Indiana Aphasia Screening Test, Reitan–Klove Sensory Perceptual Examination, and the Lateral Dominance Examination. These core tests are typically supplemented with the Wechsler intelligence scales and often times with measures of personality. Other tests are also sometimes administered to supplement the assessment of academic achievement and memory ability. Brief descriptions of the HRNB core tests are provided here, with more comprehensive information provided by Reitan and Wolfson (1993). The following descriptions focus on the adult versions of the tests, although many have been adapted for use with children. The Category Test is a measure of abstraction and concept formation that consists of 208 items organized into seven subtests. In the original version, items were presented via a slide projector onto a 10 10 in. opaque screen. One organizing principle runs through each subtest and the examinee’s task is to deduce the principle using a trial-and-error process. For this process, each test item is presented individually and the examinee is instructed to make a response of one, two, three, or four, using one of the four keys located beneath the screen. After each response, subjects are provided feedback (correct or incorrect) that they can then use to determine the principle. The total number of errors is the score used to interpret the test results. The Tactual Performance Test (TPT) is a complex task involving psychomotor, tactile, and kinesthetic abilities,

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as well as spatial memory. Examinees are blindfolded and seated at a table on which sits a form board elevated at a 45 angle. The form board contains spaces for ten blocks representing various geometric figures. The ten blocks corresponding to each space on the form board are distributed on the table between the subject and the form board. Examinees are instructed to feel the form board and each block and then place the blocks into their correct spaces on the board. Separate trials with the dominant, nondominant, and both hands are completed, after which the testing apparatus is placed out of sight and the blindfold is removed. Examinees are then provided a piece of paper and asked to draw the geometric shapes in their correct locations. The test yields scores for Time (sum of the times for the dominant, nondominant, and both hands), Memory (number of figures correctly drawn), and Location (number of correct figures drawn in their correct locations). Because examinees are never allowed to see the form board or the blocks, the TPT proves to be a very challenging test. The Seashore Rhythm Test assesses sustained attention and auditory discrimination. The test is presented using an audiotape and tape recorder and consists of 30 pairs of rhythmic beats. After each beat pair, examinees indicate whether the pair was the same or different. Three practice trials are provided to ensure understanding of the nature of the test, but once the formal test has begun, no interruptions are permitted. The examiner is not allowed to provide any assistance if examinees lose their place or become confused. Because the beat pairs are presented at a fairly rapid rate, lapses in attention negatively impact the performance on the test. The total number of correct responses is converted to a ranked score, which is used to determine performance. The Speech-sounds Perception Test assesses sustained attention and discrimination of speech sounds, and it involves reading ability. Like the Rhythm Test, stimuli for this test are presented via audiotape with a tape recorder. Test stimuli consist of 60 nonsense words that contain ‘‘ee’’ in the middle part of the word with various consonants placed before and after. The stimuli are organized into six series (A through E) of ten words each. As each nonsense word is presented, examinees select the correct word from four alternatives that are printed on the answer sheet. Examples are provided at the beginning of the test to ensure adequate understanding, but after the test begins breaks are allowed only after a series has been completed. The total number of errors is the score for the test. The Trail Making Test (TMT) requires sustained attention, visual scanning, set shifting, sequencing,

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psychomotor speed, and working memory. It is composed of two parts, A and B. Part A consists of 25 numbered circles that are distributed across an 8.5 11 in. sheet of paper. Examinees are instructed to draw lines from one number to the next so that they are connected in sequence. Part B is similar to Part A, with the exception that both number and letter series appear on the page. Examinees are instructed to start with 1, then draw a line to A, then 2, then B, then 3, and so forth until the end. Each section is timed, and examinees are told to work as quickly as possible. If an error occurs, performance is stopped and the pencil is returned to the prior circle, with instruction to start again from that point. Timing is not stopped when the examiner corrects errors. The score is the time in seconds required to finish each part of the test. Errors increase the time it takes to complete the test. The number and types of errors are recorded, but not used in the formal scoring. The Finger Tapping test measures motor speed by requiring examinees to use their index finger to make as many taps as possible on a key counter that is affixed to a board. A minimum of five 10-s trials are consecutively administered first to the dominant and then to the nondominant hand. The mean number of taps for each hand across the trials serves as the score for this test. Strength of Grip assesses motor strength and, together with the Finger Tapping test, provides information regarding motor abilities in the right and left upper extremities. Grip strength is measured in kilograms using a hand dynamometer. Two trials are completed for each hand, alternating between the dominant and nondominant hands. The score for each hand is the mean of the two trials administered to each hand. The Reitan-Indiana Aphasia Screening Test was designed to primarily assess disturbances in language, but it also includes items that assess visuoconstruction and right–left orientation. It is intended to be a screening instrument rather than a comprehensive assessment, and its items are designed in such a manner that they will be completed accurately by most individuals, who do not have brain injuries. The test stimuli are black and white words, numbers, shapes, and line drawings that are printed on a set of small stimulus cards. The stimulus cards are presented one at a time by the examiner who asks examinees to make a specific response such as reading a word, naming a shape or picture, or drawing a shape or object. Examinees make a total of 32 responses that are scored as correct or incorrect. Some HRNB authorities have suggested that the total number of errors be used as the summary score for the test. However, Reitan’s approach is to interpret each failure individually to determine the specific symptom reflected in

the poor performance (Reitan & Wolfson, 1993). Conclusions are then drawn regarding whether the item failure results from an expressive or receptive processing problem, and whether it is suggestive of a right or left hemisphere lesion. The Sensory Perceptual Examination assesses basic sensory and perceptual functions much like what is evaluated in a behavioral neurology examination. It is made up of four different tests: Bilateral Sensory Stimulation of the eyes, ears, hand, and face; Tactile Finger Recognition; Finger-tip Number Writing; and Tactile Form Recognition Test. Each test is administered to both sides of the body. Errors occur when examinees fail to correctly perceive the test stimuli. Lateral Dominance Examination is used to determine the degree to which the subject prefers one hand (and foot and eye) to another when performing a number of simple tasks, such as throwing or kicking a ball. It is usually the first test administered in the battery to establish the dominant hand, because tests that are administered to both sides of the body (Strength of Grip, Finger Tapping, and Tactual Performance Tests) are administered first to the dominant hand. The Lateral Dominance Examination contains 14 different items, but the hand that is preferred for writing is considered the dominant hand. In addition to individual test scores, a number of summary indexes of brain damage severity are calculated. The oldest and most well-validated of the summary scores is the Impairment Index, which is derived from seven of the core test scores from the HRNB. However, Reitan has pointed out a number of limitations of the Impairment Index and in response to these limitations developed an alternative index, the General Neuropsychological Deficit Scale (GNDS). The GNDS incorporates 42 different variables derived from all of the core HRNB tests, including the Wechsler intelligence scales. Additionally, the GNDS incorporates level of performance, pattern of performance, lateralization effects, and pathognomonic signs that all may be indicative of brain damage. Thus, the GNDS is preferred because it provides a more comprehensive and potentially more sensitive indicator of the biological status of the brain.

Historical Background The HRNB was developed over the course of many years from the work of Ward C. Halstead at the University of Chicago and his student Ralph M. Reitan. Halstead was an experimental psychologist whose work included studies of


both animals and humans. His interest in the behavioral correlates of human brain function led to the establishment of laboratory dedicated solely to investigating brain– behavior relationships using tests that were sensitive to brain damage (Reitan, 1994). Halstead’s first step in selecting these tests was to interact with and observe patients to gain an understanding of the manner in which their brain damage influenced their ability to adapt in novel and routine situations of daily life, with an eye toward identifying specific behavioral abnormalities that occurred in these situations and interfered with adaptive functioning (Reitan, 1994). His emphasis on adaptation was central to his concept of ‘‘biological intelligence,’’ which was uniquely associated with the biological integrity of the frontal lobes and was essential for adaptive behavior (Halstead, 1947). Halstead then set about identifying tests that were sensitive to the behavioral abnormalities that he had observed (Reitan, 1994). His approach in selecting tests was exacting, systematic, and rigorous, and proceeded in a hypothesis testing fashion of identifying tests that were thought to be sensitive to brain damage, administering the tests in a standardized manner to persons with brain injuries, and then using quantitative scores to determine if the tests were in fact sensitive to brain damage. Thus, although Halstead and Reitan viewed as important the qualitative information derived from the processes patients used to complete the various tests, the quantitative aspects of test performance were emphasized to clearly establish the sensitivity of the tests to brain damage, to clarify the functions that the tests measured, and to establish the link between these functions and localized cerebral lesions (Reitan, 1994). As now, there was then considerable debate regarding the value of qualitative versus quantitative approaches to test interpretation, with such notable figures as Kurt Goldstein and A.R. Luria strongly promoting the former approach (Reitan, 1994). What is often lost in that debate (both then and now) is the fact that both qualitative and quantitative interpretation of test performance provides valuable information regarding the status of the brain (Reitan, 1994). However, the emphasis of Halstead and Reitan on developing standardized procedures that produce reliable findings across individual patients provided a critical link between approaches used in experimental psychology and experimental neuropsychology to what has since become the discipline of clinical neuropsychology. It was from those early influences and experimental approach to test selection that Halstead’s battery of 10 tests emerged. A major step forward was the seminal validity study conducted on Halstead’s original 10 tests

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by Reitan (1955) at the Indiana University Medical Center. Reitan’s study of 50 patients with various forms of brain damage and a matched comparison sample clearly established the sensitivity of seven of the ten Halstead measures (Reitan, 1955). Large differences were present between the brain damaged and comparison groups on those measures (p < .00000001). The seven measures were supplemented with additional tests based on continued research with persons with brain injuries, with the final result being the HRNB. Since Reitan’s 1955 validation study, the HRNB has become the most widely used and extensively researched neuropsychological test battery and has had broad application with neurological, psychiatric, and medical populations. As an example of the extensive research base, Reitan and his collaborators have published more than 100 scientific articles examining various aspects of the HRNB’s psychometric properties, as well as numerous books that have also included original data. Searches conducted in PsycInfo using the terms ‘‘Trail Making Test’’ and ‘‘Category Test’’ return 923 and 395 results, respectively. Many clinicians who do not administer the entire HRNB still rely on some of it core tests (Rabin, Barr, & Burton, 2005). Additionally, major components of the battery continued to be used in research settings and have provided insights into a number of disorders.

Psychometric Data The Halstead and Reitan approach was to compare each test against an objective criterion (i.e., brain damage) to determine if the test was able to predict the criterion. Their validation studies not only focused on differences between intact and brain-damaged groups, but also, and maybe more importantly, the classification accuracy of each test for individual subjects in each group. For example, while mean differences between groups may be apparent on a variety of tests, normal variability among individuals may cause a substantial overlap in the number of brain-damaged patients who perform as normals, and the number of normals who perform as brain-damaged. Thus, validity studies of the tests of the HRNB have focused on the classification accuracy of the individual tests with various neurological populations, providing information regarding the number of cases with brain damage that were classified as brain-damaged versus normal, as well as the number of normals classified as normal versus brain-damaged. As previously mentioned, the HRNB has been the focus of much research that demonstrates the reliability and validity of its various tests.

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A summary of those studies can be found in a variety of sources, including Reitan and Wolfson (1993).

Clinical Uses With regard to test interpretation, the HRNB has a number of appealing features. The HRNB employs an innovative approach that includes four methods of inference: (1) Level of Performance, (2) Pattern of Performance, (3) Right–Left Differences, and (4) Pathognomonic Signs (Reitan & Wolfson, 1993). The Level of Performance method is commonly used in the interpretation of neuropsychological test results and compares in an absolute fashion the performance of brain damaged patients to a reference or normative sample. Scores that are lower than expected provide evidence for the presence of brain damage. HRNB results are also examined with regard to Pattern of Performance in which the differences in performance across individual tests provide support for the presence of brain damage. For Right–Left Differences, comparisons are made between tasks that are administered bilaterally, such as the Finger Tapping test, which is administered to the dominant and nondominant hands. Deviations that are larger than expected from normal asymmetries (e.g., the dominant hand being much slower than the nondominant hand) suggest that brain damage is present. Finally, test performance is examined for the presence of Pathognomonic Signs, which are abnormal responses that rarely occur in non-brain damaged individuals and, when present, almost always indicate brain damage. Being unable to copy a simple circle is an example of a Pathognomonic Sign because persons without brain damage easily perform the task. Using these four methods of inference, interpretation of test results proceeds in a stepwise manner. The Wechsler intelligence scales are reviewed first to estimate the level of intellectual functioning prior to injury. Second, the HRNB tests with the greatest sensitivity to brain damage are compared with the performance on the Wechsler scales, with unexpectedly poor performance on the HRNB tests suggestive of brain damage. Third, the tests are examined using the four methods of inference to establish the lateralization and localization of the cerebral lesion. Fourth, the course of the lesion is considered (static or progressive) by comparing tests that are differentially sensitive to acute versus chronic conditions. Finally, information gained from all of the aforementioned steps is used to attempt to determine the neuropathological process responsible for the brain damage. Once brain damage is identified, the clinician can recommend treatment, implement cognitive

rehabilitation or retraining, make predictions regarding long-term outcome, and develop strategies to help the patient and their family cope with the impact that the brain damage will have on interpersonal, psychosocial, and occupation adjustment. A number of relatively recent innovations have further increased the clinical utility of the HRNB. With regard to cognitive retraining, the HRNB is complemented by a rehabilitation program integrated to provide specific retraining exercises based on the results of the HRNB (REHABIT; Reitan & Wolfson, 1993). Additionally, a two-stage screening procedure has been developed, which identifies adults and children who require evaluation with the entire battery (e.g., Reitan & Wolfson, 2008). The screen uses tests included in the HRNB (e.g., ▶ Trail Making Test) and takes approximately 45 min or less to complete with adults. Norms that provide corrections for age, education, gender, and ethnicity have also been introduced, which allows for examination of the impact these variables have on test performance (Heaton, Miller, Taylor, & Grant, 2004). Their application in clinical practice remains controversial (Reitan & Wolfson, 2005), largely because of concerns that the effects of brain damage may fundamentally alter the normal relationships present between demographic variables such as age and neuropsychological test performance. However, the demographically corrected norms have gained widespread acceptance and are commonly used in clinical practice.

Cross References ▶ Finger Tapping Test ▶ Fingertip Number-Writing Perception ▶ Fixed Battery ▶ Hand Dynamometer ▶ Halstead Impairment Index ▶ Reitan-Indiana Aphasia Screening Test ▶ Seashore Rhythm Test ▶ Tactile Form Recognition ▶ Tactual Performance Test ▶ Trail Making Test

References and Reading Halstead, W. C. (1947). Brain and intelligence. Chicago, IL: The University of Chicago Press. Heaton, R. W., Miller, S. W., Taylor, M. J., & Grant, I. (2004). Revised comprehensive norms for an expanded Halstead-Reitan battery. Odessa, FL: Psychological Assessment Resources.

Hamilton Depression Rating Scale Rabin, L. A., Barr, W. B., & Burton, L. A. (2005). Assessment practices of clinical neuropsychologists in the United States and Canada: A survey of INS, NAN, and APA Division 40 members. Archives of Clinical Neuropsychology, 20, 33–65. Reitan, R. M. (1994). Ward Halstead’s contributions to neuropsychology and the Halstead-Reitan neuropsychological test battery. Journal of Clinical Psychology, 50, 47–70. Reitan, R. M. (1955). An investigation of the validity of Halstead’s measures of biological intelligence. Archives of Neurology and Psychiatry, 73, 28–35. Reitan, R. M., & Wolfson, D. (1993). The Halstead-Reitan neuropsychological test battery: Theory and clinical interpretation (2nd ed.). Tucson, AZ: Neuropsychology Press. Reitan, R. M., & Wolfson, D. (2005). The effect of age and education transformations on neuropsychological test scores of persons with diffuse or bilateral brain damage. Applied Neuropsychology, 12, 181–189. Reitan, R. M., & Wolfson, D. (2008). The use of serial testing in evaluating he need for comprehensive neuropsychological testing of adults. Applied Neuropsychology, 15, 21–32.

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HAM-D ▶ Hamilton Depression Rating Scale

Hamilton Depression Rating Scale DAWN M. E HDE University of Washington Seattle, WA, USA

Synonyms HAM-D; Hamilton rating scale for depression; HDRS; HRSD

Hamartoma E THAN M OITRA Drexel University Morgantown, WV, USA

Definition A rare focal malformation that resembles a neoplasm. The most common Central Nervous System hamartoma occurs inferior to the hypothalamus, either parahypothalamically or intrahypothalamically. Initial symptoms include precocious puberty, gelastic (laughing) seizures, and vision problems. Although hamartomas rarely invade or compress the surrounding structures significantly, secondary behavioral and cognitive problems may develop in childhood, leading to significant disability. Consequently, resection and radiosurgery are often the utilized treatment options.

Cross References ▶ Neoplasm ▶ Radiosurgery

References and Readings Feiz-Erfan, I., Rekate, H. L., Shetter, A. G., Siwanuwatn, R., Horn, E. M., & Spetzler, R. F. (2004). Surgical management of hypothalamic hamartomas. Barrow Neurological Institute Quarterly, 20, 4–12.

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Description The Hamilton Depression Rating Scale (HAM-D) is a widely used, semi-structured interview that assists in assessing the presence and severity of depressive symptoms (Hamilton, 1960). More than 20 published versions of the HAM-D exist (Bagby et al., 2004), with the most commonly used versions containing 17–21 items. Administration and scoring take approximately 20–30 min. The original version contained 21 items, assessing depressive symptoms experienced in the past week such as depressed mood, guilt, psychomotor retardation, insomnia, somatic symptoms, weight loss, and suicide. As the scale was based on earlier conceptualizations of depressive disorders, it also includes items outside of current conceptualizations (per DSM-IV; American Psychiatric Association, 1994) of depressive disorders, including somatic anxiety and hypochondriasis. For the same reason, atypical depressive symptoms included in the DSM-IV, such as hypersomnia and hyperphagia, are not assessed. The first 17 items have three or five possible descriptors, which increase in severity and are scored on either a 0–4 (0 = absent to 4 = severe) or 0–2 (0 = absent to 2 = severe) scale. The remaining four items were included in the original HAM-D to subtype the depressive disorder. Although these may be omitted, as they are not used to compute depressive symptom severity, many studies compute total scores using all 21 items (Williams 2001). There is also a 24-item version that includes items assessing helplessness, hopelessness, and worthlessness

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(Williams, 2001). In addition, self-report versions have been developed (see Williams, 2001, for a summary of these). Scores on the 17-item version range from 0 to 52, with the following five categories: minimal depressive symptoms (score of 0–7), mild depressive symptoms/episode (8–13), moderate depressive symptoms/episode (14–18), severe depressive symptoms/episode (19–23), and greater than 23 indicating very severe depressive symptoms/ episode. Other cutoff scores and descriptors have been proposed across a range of patient populations. The HAM-D has been translated into many languages and is used throughout the world. Given its format as a semi-structured interview, it is commonly recommended that the HAM-D be administered by a clinician with expertise in depression assessment (such as a psychologist or psychiatrist) or a trained rater. A patient interview and clinical observations form the basis for the ratings of the depressive symptoms.

Historical Background The HAM-D was one of the earliest measures for assessing the severity of depressive symptoms. Originally published by Max Hamilton in 1960 at a time when rating scales were needed to evaluate the efficacy of the first generation of antidepressants, it was designed to assess the severity of depressive symptoms in depressed inpatients (Hamilton, 1960). As such, the HAM-D reflects symptoms and experiences consistent with the times during which the scale was developed. For example, antidepressant drugs were commonly tested in severely depressed inpatients, and such studies often included not only individuals with unipolar depressive symptoms but also those with bipolar and/or psychotic symptoms (Nelson et al., 2006). Over time, psychopharmacotherapy trials for depressive disorders have been conducted in outpatients and excluded individuals with bipolar or psychotic symptoms (Nelson et al., 2006). Considerable developments in psychometric theory and methodologies have occurred since the HAM-D was developed. Numerous authors have also argued that the HAM-D is no longer consistent with the current conceptualization of depressive disorders (e.g., Bagby et al., 2004; Nelson et al., 2006). For example, several items (e.g., anxiety psychic item, insight item) that contribute to the HAM-D score are not DSM-IV criteria of a depressive episode or disorder. Nonetheless, since its original publication, the HAM-D became the criterion standard for measuring depressive symptom severity in clinical trials

of antidepressants (Williams, 2001). It has also long been considered the standard measure to which other depression ratings scales were compared. It remains the gold standard today, but not without controversy, as described below.

Psychometric Data Early psychometric data on the HAM-D was collected from samples of inpatients (Hamilton, 1960; Hamilton, 1967). While the HAM-D has been commonly described as a standardized, highly reliable, and valid measure of depressive symptom severity, recently the standardization and psychometric data behind the HAM-D have been called into question. Williams (2001) described problems with researchers frequently failing to indicate in their manuscripts the specific version of the HAM-D they used, citing the incorrect version, and using cutoffs for the 17-item version with the 24-item version. A lack of standardization in administration and scoring has also been noted (Williams, 2001). A recent systematic review (Bagby et al., 2004) of the psychometric properties of the 17-item HAM-D as a measure of depression treatment outcome raised considerable concerns about the psychometric and conceptual underpinnings of the measure, such that the authors concluded ‘‘it is time to retire the Hamilton Depression Scale’’ (p. 2175). This review of 70 studies found that while a number of psychometric properties (internal, interrater, and retest reliability estimates for the overall score; convergent and discriminant validity) of the HAMD are adequate, many weaknesses exist, particularly at the item level. Interrater reliability was reported to be poor for many individual items. Retest reliability for the overall HAM-D score ranged from 0.81 to 0.98, whereas at the item level it ranged from 0.00 to 0.85. Use of structured interview guidelines appeared to improve item reliability (mean retest reliability across individual items = 0.54), although only four items had adequate reliability. Non-correspondence is noted between the HAM-D and other DSM-IV-based measures of depressive disorders, such as the depression section of the Structured Clinical Interview for DSM-IV (SCID-CV; First, Spitzer, Gibbons, & Williams, 2002). Application of more sophisticated psychometric techniques including item response analyses based on item response theory (IRT) and Rasch analysis identified a number of problems with the HAMD. Item response analyses found many items to be problematic, suggesting that some items are insensitive to change and thus interfere with the measure’s ability to

Hamilton Depression Rating Scale

detect clinically meaningful change in depressive symptoms. Multidimensionality was also a problem. A 6-item scale derived from the HAM-D using Rasch analysis was found to have unidimensionality, improving on the original HAM-D (see Bagby et al., 2004). Bagby et al. (2004) also discuss the challenges inherent in the use of a scale that is based upon an older construct of depression inconsistent with the DSM-IV’s conceptualization. They recommend that a new gold standard for depression outcome assessment be utilized and make recommendations in this regard based on modern psychometric theories and methodologies.

Clinical Uses The HAM-D has frequently been used in clinical trials and practice to assess clinically significant responses to treatment, with treatment response typically defined as a 50% decrease (or greater) in depressive symptom severity (Tedlow et al., 1998). It is also common in depression trials to use the HAM-D to examine remission rates, with a score of 7 or less on the HAM-D thought to indicate depressive episode remission. Although it is not explicitly discussed as an assessment measure in neuropsychology textbooks, it has been used as a depression outcome measure in a number of recent clinical trials in neurological populations, including stroke (Robinson et al., 2008) and traumatic brain injury (Ashman et al., 2009). The HAM-D is in the public domain and thus readily available. Given the wide range of versions in existence, clinicians are encouraged to ensure that the specific version administered corresponds to the scoring guidelines they use (a list of published versions of the HAM-D is available in Williams, 2001). A number of structured interview guidelines for the HAM-D have been developed with the goal of improving reliability (Williams, 1988; Williams, 2001). Other considerations include suitability for medical populations, adequacy of training in administration (including psychometrists), and noncorrespondence of the HAM-D with the DSM-IV diagnostic criteria for depressive disorders. However, concerns about the HAM-D include its insensitivity to different depressive symptom profiles and mechanisms of antidepressant actions (Nelson et al., 2006). The measure has also been criticized for being too subjective, given its dependence on the rater’s skills and impressions, and for being too sensitive to somatic symptoms. In light of these observations as well as the psychometric concerns raised by Bagby et al. (2004) and others, considerable caution is warranted in its use, both

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in clinical and research settings. Given the availability and psychometric superiority of other measures of depressive episodes and severity, including brief self-report measures such as the PHQ-9 (Kroenke et al., 2001), clinicians are encouraged to think critically about their rationale for selecting the HAM-D over other measures.

Cross References ▶ Beck Depression Inventory ▶ Center for Epidemiologic Studies-Depression ▶ Depressive Disorder ▶ Geriatric Depression Scale ▶ Item Response Theory ▶ Patient Health Questionnaire ▶ Structured Clinical Interview for DSM-IV ▶ Zung Self-Rating Depression Scale

References and Readings American Psychiatric Association (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: Author. Ashman, T. A., Cantor, J. B., Gordon, W. A., Spielman, L., Flanagan, S., Ginsberg, A., et al. (2009). A randomized controlled trial of sertraline for the treatment of depression in persons with traumatic brain injury. Archives of Physical Medicine and Rehabilitation, 90, 733–740. Bagby, R. M., Ryder, A. G., Schuller, D. R., & Marshall, M. B. (2004). The Hamilton depression rating scale: has the gold standard become a lead weight? American Journal of Psychiatry, 161, 2163–2177. First, M. B., Spitzer, R. L., Gibbon, M., & Williams, J. B. W. (2002). Structured clinical interview for DSM-IV Axis I disorders, clinician version (SCID-CV). New York: Biometrics Research, New York State Psychiatric Institute. Hamilton, M. A. (1960). A rating scale for depression. Journal of Neurology, Neurosurgery, and Psychiatry, 23: 56–62. Hamilton, M. A. (1967). Development of a rating scale for primary depressive illness. British Journal of Social and Clinical Psychology, 6, 278–296. Kroenke, K., Spitzer, R. L., & Williams, J. B. W. (2001). The PHQ-9: validity of a brief depression severity measure. Journal of General Internal Medicine, 16, 606–613. Nelson, J. C., Portera, L., & Leon, A. C. (2006). Assessment of outcome in depression. Journal of Psychopharmacology, 20(4) Supplement, 47–53. Robinson, R. G., Jorge, R. E., & Clarence-Smith, K. (2008). Double-blind randomized treatment of poststroke depression using nefiracetam. Journal of Neuropsychiatry and Clinical Neuroscience, 20, 178–184. Tedlow, J., Fava, M., Uebelacker, L., Nierenberg, A. A., Alpert, J. E., & Rosenbaum, J. (1998). Outcome definitions and predictors in depression. Psychotherapy Psychosomatics, 67, 266–270. Williams, J. B. (1988). A structured interview guide for the Hamilton Depression Rating Scale. Archives of General Psychiatry, 45, 742–747. Williams, J. B. (2001). Standardizing the Hamilton Depression Rating Scale: past, present, and future. European Archives of Psychiatry and Clinical Neuroscience, 251(suppl 2), 11-6–11-12.

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Hamilton Rating Scale for Depression

Hamilton Rating Scale for Depression ▶ Hamilton Depression Rating Scale

Hand Dynamometer K ENNETH P ODELL Henry Ford Health System Detroit, MI, USA

Synonyms Grip strength; Hand strength; Strength of grip

Description The hand dynamometer measures unimanual motor strength (Russell & Starkey, 1993) and is manufactured by The Lafayette Instrument Company. The dynamometer apparatus measures grip strength in kilograms of resistance by squeezing an adjustable (for hand size) stirrup. Neuropsychologically, the hand dynamometer is considered as a measure of motor cortex integrity (Swiercinsky, 1978). However, it has long been used as a general measure of contralateral cerebral integrity and considered to be a sensitive measure to brain dysfunction (Reitan & Wolfson, 1985; Lezak, Howieson, & Loring, 2004; D’Elia, Satz, Uchiyama, & White, 1996). The original procedures (Reitan & Wolfson, 1985) require examinees to stand holding the adjusted apparatus in their hand gripping the stirrup and extending their arm straight down at their side. Examinees are asked to grip (pull) on the stirrup as hard as possible. Originally, one practice trial was allowed and the average force of two consecutive trials was recorded. However, others have modified the procedures in terms of number of trials given, administration order, and criteria for interpretation (Lezak et al., 2004; D’Elia, Satz, Uchiyama, & White, 1996). A common administration uses the average in kilograms across five trials alternating between hands with a 10-s rest between hands.

Historical Background The hand dynamometer test was first introduced as a measure of brain dysfunction by Reitan when he added

it to Halstead’s original battery and made it part of the original Halstead–Reitan Neuropsychological Battery (Reitan & Wolfson, 1985).

Psychometric Data Healthy controls can show fatigue, but not until the later trials. In fact, studies have shown little change or even slight improvement across the first two to three trials (Dunwoody, Tittmar, & McClean, 1996; Reddon, Stefanyk, Gill, & Renney, 1985;). Test/retest reliability is very good, ranging from 0.79 to 0.98 (Lezak et al., 2004). Reitan and Wolfson (1985) originally reported a 10% differential in grip strength for the dominant over the nondominant hand. A subsequent meta-analysis (Mitrushina, Boone, Razani, & De’Elia, 2005) found only a few point intermanual difference in males (2.39 kg; approximately 5%), but the expected difference in females (3.25 kg; 10%). Moreover, studies have consistently shown high intraindividual inter-manual variability leading to unacceptably high false-positive classification when the original criterion of a 20% intermanual difference (Golden, 1978) was used, particularly in left-handed subjects (Bornstein, 1986; Koffler & Zehler, 1985; Thompson, Heaton, Matthews, & Grant, 1987). Several demographic variables are known to modulate grip strength. Gender has the strongest effect on motor performance, with the largest discrepancy for the dynamometer (Mitrushina et al., 2005). Relatively recent data do not support age or education as modifying demographic factors, at least until the age of 69 (8–23% of the variance; Mitrushina et al., 2005). However, few studies have adequately studied grip strength in the elder population, where it will most likely have a larger effect. Also, several other modifying variables such as physical health, arthritis, and employment status have not been studied. Numerous studies have demonstrated that the hand dynamometer is sensitive to unilateral brain dysfunction (Lezak et al., 2004; Mitrushina et al., 2005). See Appendix 22 from Mitrushina et al. (2005) for a comprehensive listing of various normative tables and their meta-analysis of these studies.

Clinical Uses Grip strength is used neuropsychologically as a direct measure of unilateral motor dysfunction (motor cortex) and indirectly as a generally sensitive measure of contralateral cerebral dysfunction. It should not be used in

Handedness

isolation as a measure of cerebral laterality/dysfunction given the multitude of factors that influence performance. Rather, its diagnostic accuracy improves with its consistency of findings with other motor measures and across other tasks (Bornstein, 1986).

Cross References ▶ Finger Tapping Test ▶ Grooved Pegboard ▶ Halstead–Reitan Neuropsychological Battery ▶ Motor Testing ▶ Purdue Pegboard


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Hand Strength ▶ Hand Dynamometer ▶ Manual Strength

Handedness M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Synonyms Laterality

Bornstein, R. A. (1986). Normative data on intermanual differences on three tests of motor performance. Journal of Clinical and Experimental Neuropsychology, 8, 12–20. D’Elia, L. F., Satz, P., & Schretlen, D. (1989). Wechsler Memory Scale: A critical appraisal of the normative studies. Journal of Clinical and Experimental Neuropsychology, 11(4), 551–568. D’Elia, L., Satz, P., Uchiyama, C., & White, T. (1996). Color trails test, Professional manual. Odessa, FL: Psychological Assessment Resources. Dunwoody, L., Tittmar, H. G., & McClean, W. S. (1996). Grip strength and intertrial rest. Perceptual and Motor Skills, 83, 275–278. Golden, C. (1978). Stroop experimental uses. Chicago: Stoelting. Koffler, S. P., & Zehler, D. (1985). Normative data for the hand dynamometer. Perceptual and Motor Skills, 61, 589–590. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Mitrushina, M., Boone, K. B., Razani, J., & De’Elia, L. F. (2005). Handbook of normative data for neuropsychological assessment (2nd ed.). New York: Oxford University Press. Reddon, J. R., Stefanyk, W. O., Gill, D. M., & Renney, C. (1985). Hand dynamometer: Effects of trials and sessions. Perceptual and Motor Skills, 61, 1195–1198. Reitan, R. M., & Wolfson, D. (1985). Traumatic brain injury. Volume II: Recovery and rehabilitation. Tucson, AZ: Neuropsychology Press. Russell, E., & Starkey, R. (1993). Halstead-Russell neuropsychological evaluation system (HRNES). Los Angeles: Western Psychological Services. Swiercinsky, D. P. (1978). Manual for the adult neuropsychological evaluation. Springfield, IL: Thomas. Thompson, L. L., Heaton, R. K., Matthews, C. G., & Grant, I. (1987). Comparison of preferred and nonpreferred hand performance on four neuropsychological motor tasks. Clinical Neuropsychologist, 1(4), 324–334.

Hand Preference ▶ Handedness

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Definition Handedness is the preferential use of, or superior performance with, one hand for the completion of manual tasks.

Current Knowledge The manifestation of motor asymmetry is easily observed in one’s preference for and/or the superior skill with which one carries out unilateral manual tasks such as writing or throwing a ball. (NB: Preference for one hand does not necessarily lead to better performance with that hand.) In virtually all right-handers, this manual superiority is mediated by the same hemisphere that is dominant for language, namely the left. In left-handers, this is less frequently the case as the left hemisphere is still more commonly the dominant hemisphere for language. It should be noted that hand preference is imperfectly associated with other peripheral lateralized functions such as footedness and eyedness. Until recently, the association between the functional asymmetry of hand preference and any neuroanatomic correlates had not been widely studied. Examination of differences in the morphologic makeup of the central sulcus in left- versus right-handed individuals has revealed structural differences in sulcal depth that discriminate among groups, with handedness related to deeper sulci on the contralateral side. In addition, increased cellular volume in Brodmann’s area 4 was found in the same relationship as that established for sulcal depth. These findings suggest greater connectivity, and therefore

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Haptic Agnosia

better fine motor control, that may underlie hand preference in humans. However, handedness is not an all-or-none phenomenon. Some individuals are either very strongly right- or left-handed, a small percentage of individuals appear to be equally proficient with either hand for most tasks, while others fall along a continuum of handedness. The Edinburgh Handedness Questionnaire is one of the more commonly used instruments to identify degree of handedness in research studies. Left-handedness has been associated with a number of factors including male sex, culture, family history of sinistrality, and (perhaps surprisingly) both superior and limited cognitive ability.

Cross References

Hard of Hearing ▶ Deaf/Hearing Impairment

Hardening of the Arteries ▶ Atherosclerosis

Hare Psychopathy Checklist N ATHALIE D E FABRIQUE Cook County Department of Corrections Chicago, IL, USA

▶ Anomalous Dominance ▶ Asymmetry ▶ Dominance, Cerebral ▶ Hemispheric Specialization

Synonyms


Description

Amunts, K., Schlaug, G., Schleicher, A., Steinmetz, H., Dabringhaus, A., Roland, P. E., et al. (1996). Asymmetry in the human motor cortex and handedness. Neuroimage, 4(3), 216–222. Fennell, E. (1986). Handedness in neuropsychological research. In H. J. Hannay (Ed.), Experimental techniques in human neuropsychology. New York: Oxford Univeristy Press. Howieson, D., Loring, D., & Hannay, H. J. (2004). Neurobehavioral variables and diagnostic issues. In M. Lezak, D. Howieson, & D. Loring (Eds.), Neuropsycholgical assessment (4th ed., pp. 304– 307). New York: Oxford University Press. Schenker, N. M., Sherwood, C. C., Hof, P. R., & Semendeferi, K. (2007). Microstructural asymmetries of the cerebral cortex in humans and other mammals. Special Topics in Primatology, 5, 92–118.

The Hare Psychopathy Checklist is an assessment tool used to review an individual’s psychopathic or antisocial traits.

Haptic Agnosia

PCL-R

Historical Background The Hare Psychopathy Checklist – Revised (PCL-R) was developed by clinician, Robert D. Hare. It is considered the most commonly used assessment to assess psychopathy. A clinician utilizing the PCL-R to determine future risk should be adequately trained. Incorrect administration or interpretation can have serious consequences for the subject. Thus, experienced and qualified clinicians must adhere to the principles outlined for employing the PCL-R.

▶ Tactile Agnosia

Psychometric Data

HAQ ▶ Health Assessment Questionnaire

The PCL-R is a 20 item semi-structured interview with a clinical three-point rating scale ranging from zero to two (0, 1, 2) per item. A score of zero is given if the item does not apply, one if it somewhat applies, and two if it fully

Head Injury

applies. The PCL-R assesses superficial charm, manipulation, grandiosity, lack of remorse, irresponsibility, and failure to accept the consequences of actions. The overall scores are designed to assist in predicting risk for recidivism and likelihood of rehabilitation.

Clinical Uses Currently, the PCL-R lists four factors that are assessed by factor analysis and a summary of factors is generated. The first two factors are labeled with terms such as selfish and callous. The next factor considers a ‘‘chronically unstable, antisocial and socially deviant lifestyle.’’ The first two factors are often correlated with a diagnosis of narcissistic and histrionic personality disorder. Common factors associated with these two are extroversion and optimistic affect. The second two factors are associated with antisocial personality disorder, reactive anger, and violence. The quality of the assessment is dependent upon the honesty of the subject, as well as the amount of historical information that is provided by the subject.

References and Readings Hare, R. D. (1991). Manual for the revised psychopathy-checklist. Toronto: Multi-Health Systems. Hare, R. D. (1993). Without conscience: the disturbing world of the psychopaths among us. New York: Guilford Press.

Hashimoto Disease

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HCT ▶ Category Test

HDRS ▶ Hamilton Depression Rating Scale

Head Injury S AMANTHA B ACKHAUS Rehabilitation Hospital of Indiana Indianapolis, Indiana, USA

Synonyms Brain injury; Head trauma

Definition A head injury is any trauma that can result in damage to the skull, scalp, or brain tissue. Head injuries can be open or closed. An open or penetrating injury means that an object pierces through the skull and enters brain tissue. A closed injury involves a blow to the head from a striking or being struck by an object.

▶ Hypothyroidism

Current Knowledge Causes

Hashimoto Thyroiditis ▶ Hypothyroidism

Hayling Sentence Completion Task ▶ Sentence Completion

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Causes of head injury often include motor vehicle accidents, falls, violent incidents such as gunshot wounds or assaults, sports-related injuries, and other accidents at home or work. Epidemiological information suggests that falls is the leading cause of head injuries (29%), followed by motor vehicle accidents (20%), and being struck by or against (19%). For individuals with 65 years of age and older, falls are the leading cause (52%). For children between 0 and 14 years, falls account for 39% of head injuries.

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Head Trauma

Types


Various characteristics of head injuries correlate with the severity of intracranial injury. Extracranial injury without brain injury: An injury to the outside of the cerebral cortex not causing internal or intracranial damage. Concussion: A concussion occurs when trauma occurs to the head causing a ‘‘jarring’’ injury to the brain. A person typically experiences an alteration in mental status that can result in a feeling of daze, brief loss of consciousness, amnesia, or other disruptions in neurological functioning. Skull fractures: A skull fracture is a break in the bone surrounding the brain and other structures within the skull. These can include linear, depressed, or basilar skull fractures. Intracranial hemorrhages: An intracranial hemorrhage is a bleeding that occurs inside the skull. This can include a subdural hematoma, epidural hematoma, and intraparenchymal hemorrhage or cerebral contusion.

Fusco, E. (2005). Head injury. Retrieved December 26, 2007, from http:// www.emedicinehealth.com/head_injury/article_em.htm Gennarelli, T. A., & Graham, D. I. (2005). Neuropathology. In J. M. Silver, T. W. McAllister, & S. C. Yudofsky (Eds.), Textbook of traumatic brain injury (pp. 27–50). Arlington, VA: American Psychiatric Publishing. Lucas, J. A. (1998). Traumatic brain injury and postconcussive syndrome. In P. J. Snyder, & P. D. Nussbaum (Eds.), Clinical neuropsychology: A pocket handbook for assessment (pp. 243–303). Washington, DC: American Psychological Association. Perez, E. (2007). Head injury. Retrieved December 26, 2007, from http:// www.nlm.nih.gov/medlineplus/ency/article/000028.htm Putukian, M. (2001). Head injuries. In W. E. Garrett, Jr., D. T. Kirkendall, & D. L. Squire (Eds.), Principles and practice of primary care sports medicine (pp. 331–352). Philadelphia, PA: Lippincott Williams & Wilkins.

Symptoms Symptoms can range in severity, depending on the severity of associated brain injury. The symptoms can have an abrupt onset or develop slowly. Initially, the person may present with a vacant stare, delayed responding, poorly focused attention, disorientation, confusion, slurred or incoherent speech, motor incoordination, excessive emotional lability, and forgetfulness. Other symptoms may include headaches, dizziness, nausea, vomiting, drowsiness, and blurred vision. If the person has experienced bleeding into the brain as a result of the head injury, then more serious complications can occur such as loss of consciousness, confusion, changes in blood pressure, convulsions, skull fractures, hemorrhages, pupillary changes, motor or sensory changes, and cognitive changes.

Head Trauma ▶ Head Injury ▶ Penetrating TBI ▶ Traumatic Brain Injury (TBI)

Headache N ATHAN D. Z ASLER Concussion Care Centre of Virginia, Ltd. Glen Allen, Virginia, USA Tree of Life Services, Inc. Richmond, Virginia, USA VCU Department of Physical Medicine and Rehabilitation Richmond, Virginia, USA University of Virginia Charlottesville, Virginia, USA

Synonyms Cross References ▶ Acceleration/Deceleration Injury ▶ Coma ▶ Concussion ▶ Loss of Consciousness ▶ Mild Brain Injury ▶ Post-Concussion Disorder (Syndrome) ▶ Traumatic Brain Injury (TBI)

Cephalalgia

Short Description A headache is a pain or discomfort that is perceived to be in the head, although sometimes the pain may actually be referred from other structures such as the neck. Lifethreatening causes of headache, although rare, must be

Headache

considered as more expedient treatment can be life saving. Experienced clinicians should be able to determine the underlying cause for headache with appropriate time taken to acquire an adequate history, as well as conduct a careful physical evaluation and, as necessary, order appropriate diagnostic testing. Once the appropriate diagnosis is made, treatment should be instituted in a holistic fashion with a sensitivity to maximizing the benefit/risk ratio of any particular intervention, prescribing treatment that can be optimally complied with and educating the patient and family regarding the condition, its treatment, and prognosis.

Categorization Headaches have been categorized using various systems. The two most popular and well-accepted classification systems are the ICHD-2 advocated for by the International Headache Society and the ICD-10 which the World Health Organization (WHO) advocates using. There are controversies and debates with regards to both classification systems. Headaches can also be classified into primary and secondary headache disorders. Secondary headaches may be harbingers of a more serious underlying condition such as a bleed or tumor.

Etiology There are multiple sources of head pain, both inside and outside of the head. The brain itself is not a source of pain. Headache typically results from six major abnormal physiological phenomena:

Displacement of intracranial (within the skull) structures Inflammation Ischemia (decreased blood flow) and/or metabolic changes Myodystonia (increased muscle tone) Meningeal irritation (inflammation/irritation of the thin layers of tissue ‘‘coating’’ the brain) Increased or decreased intracranial pressure

Natural History, Prognostic Factors, Outcomes The natural history, prognosis, and outcome of both primary and secondary headache conditions are variable and dependent upon the specific headache disorder.

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Neuropsychology and Psychology of Headache It is quite common that persons with chronic pain develop emotional difficulties such as depression and/or anxiety which may further increase their perception of their pain and their subjective level of distress. Many times referral to a psychologist or pain specialist may be indicated to help the person with the headache condition learn to deal better with their pain. Biofeedback, stress management, and cognitive-behavioral therapies do help many patients with headache including those without evidence of psychological problems. Education of the patient with headache is crucial to optimizing treatment success and decreasing distress and poor adaptation to pain, particularly when chronic. One of the most important pieces of education is making sure the patient understands their disease process and the expectations of treatment. Another very important educational component is making sure the patient understands how to take their medication and the potential adverse effects of non-compliance (e.g., suboptimal headache control) and/or overuse (e.g., rebound headache). Pain associated with headache can interfere with thinking in terms of decreased attention and concentration with perceived memory problems. Primary headache pain can also disrupt sleep, as well as, behavior (i.e., predominantly manifesting as irritability, depression, and/or anxiety). Headache has also been noted to be a primary somatic symptom of PTSD.

Evaluation All too often, patients are simply given a diagnosis of traumatic headache and no further elaboration is made relative to the problem causing the pain. The major questions relative to the headache profile that need to be asked are expressed in the mnemonic ‘‘COLDER’’: Character, Onset, Location, Duration, Exacerbation, and Relief. Other descriptors including the frequency, severity, associated symptoms, and presence/absence of aura, degree of functional disability associated with headache episodes, as well as, the time of day that headaches come on are all important parameters to inquire about. A good history regarding the patient’s historical headache status, as well as any genetic loading risk factors for headache should also be sought. Adequate physical examination is paramount to an appropriate diagnosis and should include inspection, palpation, auscultation, and percussion as appropriate. The neurological exam should be a centerpiece of this

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assessment; however, adequate examination of cranial and cervical (i.e., neck) structures including inspection, percussion, and palpation of the head, neck, and shoulders, as appropriate, is a crucial yet often overlooked aspect of a complete headache exam.

Treatment Headache treatment should occur in a holistic biopsychosocial approach as most headache conditions reflect an interaction of organic and emotional factors. Treatments that target not only the pain generator(s), but also the patient’s reaction to pain within his/her daily life typically fare better than treatments with a more narrow focus (e.g., medication management or nondrug therapies alone). Understanding vulnerability issues as predictors of poor chronic pain adaptation is also critical in this context.

Cross References ▶ Pain ▶ Posttraumatic Headache

References and Readings International Headache Society. The International Headache Classification (ICHD-2). http://www.ihs-classification.org/en. Accessed Jan 1, 2009. Lance, J. W., & Goadsby, P. J., (Eds.). (2005). Mechanism and management of headache (7th ed.). Philadelphia, PA: Elsevier. National Headache Foundation. http://www.headaches.org. Accessed Jan 1, 2009. Silberstein, S. D., Lipton, R. B., & Dodick D. W., (Eds.). (2007) Wolff ’s headache and other head pain. New York: Oxford University Press.

Health Assessment Questionnaire TAMARA B USHNIK NYU Langone Medical Center New York, NY, USA

Synonyms HAQ; Stanford HAQ

Description The Health assessment questionnaire (HAQ) (1978) provides a comprehensive assessment of patient health outcome, as well as a means of longitudinal tracking of health care outcomes (Bruce & Fries, 2003a). It consists of five generic health dimensions: avoidance of disability, freedom from pain and discomfort, avoidance of adverse treatment effects, maintenance of low treatment costs, and postponement of death. Two formats are available. The two-page HAQ captures information on three scales: the HAQ Disability Index (HAQ-DI), the HAQ visual analog (VAS) pain scale, and the VAS patient global health scale. The full HAQ also collects information on drug toxicity data, direct cost data, and mortality-related data. The HAQ is typically self administered, but can be given over the telephone or by interview in a clinical setting using trained interviewers. The two-page HAQ can be completed in about 5 min; the full HAQ takes between 20 and 30 min. The HAQ-DI is scored on a four-level difficulty scale ranging from zero (normal, no difficulty) to three (unable to do, extreme difficulty). There are 20 questions covering eight domains: dressing, rising, eating, talking, hygiene, reach, grip, and usual activities. The highest score for an item within each domain is taken as the score for that domain; the HAQ-DI total score is the average of the eight domain scores, and therefore ranges between 0 and 3. Scores of 0–1 represent mild to moderate difficulty; 1–2 represent moderate to severe disability; 2–3 represent severe to very severe disability. In a population-based study, average scores were 0.49; in osteoarthritis and rheumatoid arthritis, scores were 0.8 and 1.2 respectively (Bruce & Fries, 2003a). The HAQ pain scale is a visual analog scale which asks the individuals to rate their arthritis-related pain and its severity over the past week. The HAQ is in the public domain, but is copyrighted by Stanford University to ensure that it is not modified. There is no charge for using the English version of the HAQ, but other groups that have translated the HAQ into other languages may charge a fee for access to that version.

Historical Background The HAQ was developed to reflect the recognition that patient-centered health outcomes were important and that measures were needed to track self-assessed health. The HAQ is one of the first instruments designed to capture long-term health outcomes in a reliable and valid way, using a standard protocol, and to allow

Hearsay Evidence

supplemental information to be collected with additional measures in a study-specific manner (Bruce & Fries, 2003b). It was initially used in the rheumatology field, but it is not disease-specific and has been administered across diverse disciplines and in different cultures.

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Ramsey, D., Fries, J., Singh, G., & Lane, N. (1998). Outcome assessment in osteoarthritis (OA): WOMAC or HAQ? Arthritis & Rheumatism, 41, Suppl:S223. http://aramis.stanford.edu

Hearing Impaired Psychometric Data Validity: The HAQ is sensitive to change; the minimal important clinical difference ranges between 0.1 and 0.22 (Ramsey, Fries, & Singh, 1996). Correlations between HAQ scores and task performance range between 0.71 and 0.95 (criterion validity). HAQ scores correlated well with the Western Ontario-McMaster Universities OA Index (WOMAC) (Ramsey, Fries, Singh, & Lane, 1998). Reliability: Test–retest correlations range from 0.87 to 0.99 (Bruce & Fries, 2003a). Correlations between HAQ scores obtained through interview versus self-report questionnaire range between 0.85 and 0.95 (Bruce & Fries, 2003a).

▶ Deaf/Hearing Impairment

Hearing Loss ▶ Deaf/Hearing Impairment

Hearsay Evidence N ATHALIE D E FABRIQUE Cook County Department of Corrections Chicago, IL, USA

Clinical Uses Synonyms The HAQ has been administered by the Stanford Arthritis, Rheumatism, and Aging Medical Information System (ARAMIS) more than 200,000 times for a number of different purposes: assess clinical status, evaluate clinical and observational trial effectiveness, and define health outcomes. It is available in more than 60 languages (http://aramis.stanford.edu).

Cross References ▶ Visual Analog Scale

References and Readings Bruce, B., & Fries, J. F. (2003a). The Stanford Health Assessment Questionnaire: A review of its history, issues, progress, and documentation. Journal of Rheumatology, 30, 167–178. Bruce, B., & Fries, J. F. (2003b). The Stanford Health Assessment Questionnaire: Dimensions and practical applications. Health and Quality of Life Outcomes, 1, 20–26. Ramsey, D., Fries, J., & Singh, G. (1996). The Health Assessment Questionnaire 1995 – Status and review. In B. Spilker (Ed.), Quality of life and pharmacoeconomics in clinical trials (2nd ed., pp. 227–237). Philadelphia: Lippincott-Raven.

Federal rules of evidence

Definition Hearsay is generally considered unverified information heard or received from another. As it relates to the law, hearsay evidence is a legal principle involving the reiteration of statements made out of court and the possible admission of those statements in court. In general, hearsay is based on the reports of others rather than the personal knowledge of a witness and therefore generally not admissible as testimony. A common misconception is that hearsay evidence is never permitted in judicial proceedings. While it is accurate that it is generally not accepted, there are some exemptions. For example, not all out of court statements are considered hearsay. If a statement is used for purposes other than to prove the truth of assertion, it can be admissible in judicial proceedings. The statement is considered hearsay if it is offered to prove the truth of what the statement claims. Another fallacy regarding hearsay is that it only applies to oral statements. However, it also includes written statements or nonverbal conduct of a person.

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Cross References


▶ Admissibility

References and Readings FRE 804(b)(3). Rule 801, 28 U.S.C. App. See Rule for Courts-Martial 801, Manual for Court Martial, United States (2005 edn.). Wigmore on Evidence }1360.

Heart Attack ▶ Myocardial Infarction


Heart Failure ▶ Congestive Heart Failure

‘‘Heat of the Moment’’ ▶ Irresistible Impulse

Hebb, Donald (1904–1985) A MY A LDERSON Emory University/Rehabilitation Medicine Atlanta, GA, USA

Major Appointments

Professor, Queen’s University, Kingston, Ontario (1932–1942) Professor, McGill University, Montreal, Quebec (1947–1972) Professor Emeritus, McGill University, Montreal, Ontario (1972–1980) Professor Emeritus, Dalhousie University, Halifax, Nova Scotia (1980–1985)

Hebb was a longstanding member of both the American Psychology Association (APA) and Canadian Psychological Association (CPA). In 1960, he was the first non-American to be elected President of the APA. The Donald O. Hebb Award, so named in his honor, is awarded to Canadians who have made distinguished contributions to the sciences. Hebb was the first recipient of this award in 1980.

A relative late-comer to psychology, Hebb graduated cum laude from McGill University in 1932 with a Master’s degree in psychology. His thesis was entitled Conditioned and Unconditioned Reflexes and Inhibition. Four years later in 1936, under the tutelage of Karl Lashley, he received his Ph.D. from Harvard University. Throughout his career, Hebb was devoted to illuminating the neurophysiological underpinnings of mental processes such as memory, emotion, and volition. During his early work as a post-doctoral fellow at the Montreal Neurological Institute, which he accepted in 1937, Hebb worked closely with Wilder Penfield to investigate the ways that damage to various regions of the brain altered intelligence and behavior. His investigations in this area led him to conclude that many cognitive processes, especially learning and memory, are not locally stored in the brain but more widely distributed. He also found that while children with brain damage are often able to regain normal functioning related to the area of the brain that was damaged, adults experienced significantly greater difficulty with recovery of neurological functioning. During his tenure at McGill University, Hebb’s notable students – Mortimer Mishkin, Haldor Enger Rosvold, and Brenda Milner – continued his groundbreaking research with neurosurgeon Wilder Penfield and continued to contribute to the wealth of knowledge about the neurophysiological underpinnings of cognitive, behavioral, and emotional processes. Recognized as one of the most important contributions to biology, Hebb’s landmark The Organization of Behavior: A Neuropsychological Theory was published in 1949. In this monograph, he presented his concepts of Hebbian learning and cell assemblies.

Hebb, Donald (1904–1985)

Hebbian learning presented a means for increasing synaptic efficiency based on concomitant cell firing such that, ‘‘When an axon of cell A is near enough to excite a cell B and repeatedly or persistently takes part in firing it, some growth process or metabolic change takes place in one or both cells such that A’s efficiency, as one of the cells firing B, is increased.’’ The principles underlying Hebb’s learning postulate laid the foundation for timedependent, interactive, and local mechanisms by which synaptic processes could be altered as a means of preand post-synaptic activity. In simpler terms, ‘‘cells that fire together, wire together.’’ A corollary of his learning theory, Hebb’s cell assembly theory postulated that once activated together, groups of neurons will continue to fire together, forming a sort of cellular memory. Although neurophysiology was inadequate to either prove or disprove Hebb’s learning and cell assembly theories at the time that The Organization of Behavior was originally published, these postulates have become the foundations of modern neuropsychological understanding of synaptic modification and neural learning. For example, processes such as long-term potentiation are now accepted to rely upon this mechanism. Cognitive neuroscience and computational modeling networks also use Hebb’s learning rule as a basic algorithm to adjust connection weights in neural network models. In addition to his landmark contributions to synaptic learning theory, Hebb also developed human and animal intelligence and cognition tests. These tests included the Hebb–Williams maze, which has been used to study comparative intelligence across various animal species, as well as the Adult Comprehension Test and the Picture Anomaly Test, used to explore changes in human cognitive functioning after neurological insult. As a tribute to the depth and breadth of scientific contributions made by Hebb, he was the first nonAmerican to be elected President of the APA in 1960. Further, the Donald O. Hebb Award, so named in his honor, is awarded to Canadians who have made distinguished contributions to the sciences. Hebb was the first recipient of this award in 1980.

Short Biography Donald Olding Hebb was born in Chester, Nova Scotia, the oldest of four children born to two physicians. Hebb

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was homeschooled until the age of 8 and later completed high school at Halifax County Academy in Halifax. While some of Hebb’s siblings went on to careers in medicine and physiology, his older brother pursued a career in journalism and insurance after obtaining a degree in law. Hebb, himself, had limited interest in psychology or medicine; instead, he earned a Bachelor of Arts degree from Dalhousie University with hopes of becoming a novelist. Hebb then returned to his home town to teach. He later became a farmer and laborer before enrolling in graduate school. While studying part-time at McGill University, he taught part-time in the suburbs of Montreal, using an innovative educational approach that created a motivational learning environment. Hebb obtained his Master’s degree from McGill University in 1932. Then, in 1934, following the death of his first wife, he enrolled in a doctoral training program with Karl Lashley at the University of Chicago. His initial thesis topic involved spatial orientation and place learning. He later followed Lashley to Harvard in 1935, earning his Ph.D. in 1936. His research addressed the effects of early visual deprivation on size and brightness perception in rats. In 1937, Hebb married his second wife, Elizabeth Nichols Donovan, and also obtained a fellowship at the Montreal Neurological Institute with Wilder Penfield. Working with Penfield, he explored the effects of brain insult on adult and child behavior. During this time, he also created a number of measures to explore changes in neurocognitive functioning occurring after neurological insults, among them the Adult Comprehension Test and the Picture Anomaly Test. In 1939, Hebb was appointed to a teaching position at Queen’s University, where he developed the Hebb– Williams maze as a means of assessing animal intelligence and frontal lobe functioning. He later moved to Orange Park, Florida, to once again work with Karl Lashley at the Yerkes National Primate Research Center. During the course of his research there, he wrote his landmark work The Organization of Behavior: A Neuropsychological Theory. Hebb returned to McGill in 1947 as a professor and stayed until his retirement in 1972, continuing to teach a graduate seminar as Professor Emeritus until 1980. In 1980, he returned to Dalhousie University as Professor Emeritus. Hebb’s second wife died in 1962, and he remarried in 1966 to Margaret Doreen Wright (nee Williamson). Donald Hebb died in 1985, 2 years after Margaret. He is survived by two daughters from his second marriage, Mary Ellen Hebb and Jane Hebb Paul.

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Cross References ▶ Milner, Brenda Atkinson (1918– ) ▶ Mishkin, Mortimer (1926– ) ▶ Penfield, Wilder (1891–1976)

References and Readings Brown, R. E., & Milner, P. M. (2003). The legacy of Donald O. Hebb: More than the Hebb synapase. Nature Reviews Neuroscience, 4, 1013–1019. Hebb, D. O. (1949). The organisation of behaviour. New York: John Wiley. Hebb, D. O. (1955). Drives and the conceptual nervous system. Psychological Review, 62, 243–254. Hebb, D. O. (1958). Alice in Wonderland or psychology among the biological sciences. In H. F. Harlow & C. N. Woolsey (Eds.), Biological and biochemical bases of behaviour. Madison: University of Wisconsin Press. Hebb, D. O. (1976). Physiological learning theory. Journal of Abnormal Child Psychology, 4, 309–314. Hebb, D. O. (1981). Consider mind as a biological problem. Neuroscience, 6, 2419–2422. Hebb, D. O. (1983). Neuropsychology: Retrospect and prospect. Canadian Journal of Psychology, 37, 4–7. Hebb, D. O. (2002). The organization of behavior: A neuropsychological theory. New Jersey: Lawrence Erlbaum. Seung, H. S. (2000). Half a century of Hebb. Nature Neuroscience, 3(Supplement), 1166.

Hećaen, Henry (1912–1983) A DAM H UDEPOHL 1, A NTHONY Y. S TRINGER 2 1 Georgia State University Atlanta, GA, USA 2 Medicine Emory University Atlanta, GA, USA


The contemporary identity of neuropsychology was in many ways shaped by the work of Henry Hećaen. While his career was still in its nascent stages, Hećaen moved away from his initial interest in psychiatry, and became increasingly involved in the study of neurology, under the mentorship of Jean Lhermitte. Although the term was not then widely used, Hećaen had been described as a neuropsychologist ‘‘from that moment until the end’’ (Lhermitte, Lecours, Poncet, Marcie, & Whitaker, 1985). Although the more prestigious routes of the Salpeˆtrie`re and Biceˆtre were closed to him, due to the rigidity of the French medical

establishment (Boller, 2006), Hećaen managed to harness the opportunities available to him to become one of the most influential and essential individuals in the establishment of neuropsychology as a distinct discipline. The end of the Second World War and the liberation of France allowed Hećaen to concentrate on the practice of neurology and neuropsychological research. In collaboration with Julian de Ajuriaguerra, he published the first of his most influential works, Le cortex cerebral (Ajuriaguerra & Hećaen, 1949). This book, considered the first textbook of neuropsychology ever written (Lhermitte et al., 1985), provided a detailed summary of what was then understood about the relations between the brain and the behavior (Benton, 1994). Soon after this book was published, his research group moved to Sainte-Anne Hospital in Paris where it grew ‘‘steadily in size and influence over the years’’ (Benton, 1983). In 1952, he spent a year as a visiting professor at McGill University, which by all accounts, profoundly influenced his subsequent research. In Canada, Hećaen was exposed to the positivism and strict scientific methodological rigor practiced in North America during the 1950s (Tzavaras, 1986). This allowed Hećaen to incorporate the experimental and statistical designs into his already rich clinical experience (Tzavaras & Albert, 2002) and harkened in the early modern period of neuropsychological research. Upon his return to Paris, Hećaen published two of his most influential works. In 1954, he published a paper with de Ajuriaguerra on Balint’s syndrome, which became a seminal paper in contemporary neuropsychology (Boller, 2006). His next paper, ‘‘The syndrome of apractognosia due to lesions of the minor cerebral hemisphere,’’ successfully dispelled the longstanding notion of the dominance of the left hemisphere in cognition and illuminated the specific contributions of the right hemisphere in the mediation of visuoperceptual and visuoconstructional processes (Hećaen, Penfield, Bertrand, & Malmo, 1956). Hećaen and Oliver Zangwill in Britain exerted considerable influence on contemporary formulations of functional cerebral organization (Benton, 1991). Hećaen and his collaborators published on nearly every neuropsychological phenomenon known, producing groundbreaking research on conduction aphasia, agraphia, dressing apraxia, acalculia, body schema disturbances, constructional apraxia, and topographical disorientation. In collaboration with linguists, he published extensively on aphasic disorders. He was

Hećaen, Henry (1912–1983)

instrumental in convincing the American clinicians of the validity of prosopagnosia as a neurological illness (Benton, 1994) and also published on the implications of left-handedness. Hećaen will also be remembered as a tireless promoter of the field and as an enthusiastic disseminator of neuropsychological research. He is perhaps best known for founding the International Neuropsychological Symposium along with Hans Hoff, Klaus Conrad, and Oliver Zangwill (Zangwill, 1984), although the latter gives him full credit for the generation of the idea. He was also the creator as well as the first and longtime editor in chief of the first publication dedicated solely to the dissemination of neuropsychological research, Neuropsychologia.

Education and Training Hećaen received his medical degree from the Naval Medical School in Bordeaux in 1934 at the age of 22. His doctoral dissertation entitled ‘‘Artistic Inspiration and Its Relation to Madness,’’ examined the case of German composer Hugo Wolf and was a characteristic of Hećaen’s dynamic interests as it drew on influences from ancient Greek literature to Freudian psychoanalysis. After his discharge from the navy (although he returned briefly at the outbreak of the Second World War), Hećaen traveled to Paris with the intention of studying psychiatry. He secured an appointment as a Medecin des Hoˆpitaux Psychiatriques at Sainte-Anne Hospital, where he met his longtime friend and collaborator Julian de Ajuriaguerra. There, both men were trained in psychiatry by Henri Ey and also mentored by the neurologist Jean Lhermitte, whom both men referred to as Maıˆtre (Master). In 1952, he spent a year on sabbatical at McGill University and the Montreal Neurological Institute in the company of the neurosurgeon Wilder Penfield.

Major Appointments

Sainte-Anne Hospital, Paris, France, 1942 Ećole des hautes etudes, Paris, France, 1965 Paul Broca Center, Paris, France, 1970


Croix de Guerre, France Commadeur de la Le´gion d’Honneur, France

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Commandeur des Palmes Acade´miques, Peru Prix de la Fondation pour la Recherche Medical Française Doctor Honoris Causa of McGill University

Short Biography Henry Hećaen was born on May 5, 1912 in the city of Brest in Brittany. His father was killed early in the course of the Second World War while Hećaen was an infant. He identified throughout his life as a ‘‘Breton’’ and not a French, and never missed an opportunity to extol the virtues of British as opposed to French footballers (Lhermitte et al., 1985). He was raised by a single mother in a middle class home, in contrast to many of his contemporaries who were raised in well-connected and wealthy Parisian families (Tzavaras & Albert, 2002). The influence of his paternal grandfather and an uncle (who served as a physician in the French navy) instilled in Hećaen a desire to seek a professional life. Due to economic concerns, he entered the Naval Medical School in Bordeaux where he received his degree in 1934. He married a talented violinist and daughter of an admiral in the late 1930s (Tzavaras & Albert, 2002). He soon realized that a life at sea was not for him and discharged. Hećaen traveled to Paris to begin his study of psychiatry, but soon returned to the French Navy at the outbreak of the Second World War. The navy oil tanker that Hećaen was serving on was captured by German troops at Dunkerque. Hećaen was struck in the leg by a stray bullet during the skirmish and taken prisoner. After the capitulation of France in 1940, Hećaen returned to Paris and began his work at Sainte-Anne Hospital in Paris. Hećaen was active in the French resistance throughout the Nazi occupation and was later honored with the croix de guerre (cross of war) for this work by the French government. To truly understand the life of Henry Hećaen and appreciate his contributions to the field of neuropsychology, it is important to consider the position that he was in throughout his career. His identity as a Breton and his middle-class upbringing made him an outsider in the French medical establishment. He received a half-time appointment as a neurologist, and as such, had to support himself financially by running a private practice in the afternoon. This, of course, meant that he often had to meet with his research team and work on his manuscripts well into the early hours of the morning. Recognition for the value of his work was slow to come, especially in his own country. As late as 1965, Hećaen was still sharing a tiny office with two colleagues in the deteriorating

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basement of Sainte-Anne Hospital. Indeed, he was not given an academic appointment until this same year and not provided with adequate research space until 1970, when his research team moved to the newly created Paul Broca Center, which he ran until his retirement in 1982. Hećaen overcame these obstacles by synthesizing his brilliant scientific imagination with rigorous methodological techniques, a tireless work ethic, and interpersonal acumen and warmth, to become one of the key influential figures in the creation of neuropsychology as an independent field. Hećaen passed away from the complications of myocardial infarction on June 8, 1983. As he lay dying, he reflected on his life with a friend, stating that he was proud to have helped found the International Neuropsychological Symposium and for his contributions to the world of neurology and neuropsychology.

Tzavaras, A. (1986). The evolution of Henry Hećaen’s thoughts: Neuropsychiatry and the theoretical substratum of a neuropsychologist. Journal of Neurolinguistics, 2(1), 201–207. Tzavaras, A., & Albert, M. (2002). Henry Hećaen: Evolution of his thought. In A. Stringer, E. Cooley, & A. Christensen (Eds.), Pathways to prominence in neuropsychology: Reflections of twentieth-century pioneers (pp. 41–47). New York: Psychology Press. Zangwill, O. (1984). Henry Hećaen and the origins of the International Neuropsychological Symposium. Neuropsychologia, 22(6), 813–815.

Heilman, Kenneth M. (1938– ) A LYSSA B RAATEN Emory University/Rehabilitation Medicine Atlanta, GA, USA

Name and Degrees Cross References ▶ Acalculia ▶ Aphasia ▶ Balint’s Syndrome ▶ Body Schema ▶ Cerebral Dominance ▶ Conduction Aphasia ▶ Constructional Apraxia ▶ International Neuropsychological Society ▶ International Neuropsychological Symposium ▶ Prosopagnosia ▶ Visual Agnosia

Dr. Heilman attended college at the University of Virginia College of Arts and Sciences from 1956 to 1959. He subsequently received his M.D. from the University of Virginia School of Medicine in Charlottesville, Virginia in 1963. He spent 2 years for training in Internal Medicine at Cornell University Medical Center’s Bellevue Hospital. In 1965, he enrolled in the U.S. Air Force and became Chief of Medicine at the NATO hospital in Izmir, Turkey. Upon discharge from military service, he obtained a neurology residency and fellowship at the Harvard Neurological Unit of Boston City College with Dr. Derek DennyBrown and continued his studies with Dr. Norman Geschwind thereafter.

References and Readings Major Appointments Ajuriaguerra, J. de., & Hećaen, H. (1949). Le cortex cerebral. Paris: Masson. Benton, A. (1983). Henry Hećaen (1912–1983). Cortex, 19(4), 425–426. Benton, A. (1991). The Hećaen-Zangwill legacy: Hemispheric dominance examined. Neuropsychology Review, 2(4), 267–280. Benton, A. (1994). Four neuropsychologists. Neuropsychology Review, 4(1), 31–44. Boller, F. (2006). Modern neuropsychology in France: Henry Hećaen (1912–1983) and the Sainte-Anne hospital. Cortex, 42(8), 1061–1063. Galtier, A. (1984). Publications of Henry Hećaen. Neuropsychologia, 22(6), 647–659. Hećaen, H., Penfield, W., Bertrand, C., & Malmo, R. (1956). The syndrome of apractognosia due to lesions of the minor cerebral hemisphere. AMA Archives of Neurology and Psychiatry, 75(4), 400–434. Lhermitte, F., Lecours, A., Poncet, M., Marcie, P., & Whitaker, H. (1985). In memoriam: Henry Hećaen (1912–1983). Brain and Cognition, 4(2), 133–139.

University of Florida, Gainesville, Florida, Distinguished Professor, 1998–present. North Florida/South Georgia Veterans Affairs Medical Center, Gainesville, Florida, Chief of Neurology, 1996–present. University of Florida, Gainesville, Florida, James E. Rooks, Jr., Professor of Neurology, 1990–2007. University of Florida, Gainesville, Florida, Director, Cognitive and Memory Disorder Clinic, 1998–2007. University of Florida College of Medicine, Gainesville, Florida, Director, Center for Neuropsychological Studies, 1984–2007. University of Florida, Gainesville, Florida, Professor, Department of Clinical Psychology, 1977–present.

Heilman, Kenneth M. (1938– )

North Florida/South Georgia Veterans Affairs Medical Center, Gainesville, Florida, Staff Physician, 1977– 1996. University of Florida College of Medicine, Gainesville, Florida, Associate Professor of Clinical Psychology, 1973–1977. University of Florida, Gainesville, Florida, Professor, Department of Neurology, 1975–1998. University of Florida College of Medicine, Gainesville, Florida, Associate Professor of Neurology, 1973–1975. University of Florida College of Medicine, Gainesville, Florida, Assistant Professor of Medicine, Division of Neurology, 1970–1973.


Dr. Heilman is a member of many organizations and has received many awards over the years including the Outstanding Achievement Award from the Society for Behavioral and Cognitive Neurology in 1996. He received the University of Florida Research Foundation Professorship from 1997 to 1999, and again from 2005 to 2007. He also received the Faculty Research Award in Clinical Science from the University of Florida College of Medicine in 1993, the Distinguished Service Award for Scientific and Educational Contributions from the American Speech and Hearing Association in 2003, and the University of Florida College of Medicine Lifetime Achievement Award in 2008. He is a member of Alpha Omega Alpha, Phi Kappa Phi, Sigma Xi, and the Dana Foundation. Dr. Heilman is a member of the American Academy of Aphasia, American Society of Neurorehabilitation, the National Aphasia Association, the International Society for Research of Emotion, the Society for Behavioral and Cognitive Neurology, the Aphasia Research Group of the World Federation of Neurology, the International Neuropsychological Society, the Florida Society of Neurology, the Florida Medical Society, the Association of University Professors, the American Neurological Association, the American Academy of Neurology, and the American Speech and Hearing Association. He served on the Governing Board of the American Academy of Aphasia from 1987 to 1990, on the Advisory Board of the National Aphasia Association in 1989, and on the Board of Directors of the International Society for Research of Emotion in 1984. He was also the President of the Society for Behavioral and Cognitive Neurology from 1982 to 1983. He holds certifications from the American

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Society of Neurorehabilitation, American Board of Psychiatry and Neurology, and the United Council for Neurologic Subspecialties-Behavioral Neurology.


Dr. Heilman is the author, coauthor, or editor of 11 books and more than 500 publications. He and his coworkers are responsible for many landmark scientific contributions including demonstrating the role of a cortical (frontal-parietal)-limbic (cingulate)-reticular (thalamic and mesencephalic) network in mediating attention; that the right hemisphere is dominant for mediating attention and arousal; that the unilateral neglect can be caused by attentional as well as action-intentional deficits; that the right hemisphere, especially the frontal lobe, is dominant for ‘‘when’’ action-intentional computations including, when to initiate an act, when not to act, and when to stop actions; that certain patients with neglect improve when treated with dopamine agonists; and that the right hemisphere is important for emotional communication, including the understanding and expression of emotional prosody and facial expression. He demonstrated that skilled movement, such as using a pair of scissors, is mediated by a left hemisphere modular network where the parietal lobe contains the memories of the spatial trajectories needed to perform skilled movement and the frontal lobe (premotor cortex) performs the computations that transfer this knowledge to a motor code. He demonstrated that the left hemisphere is also dominant for storing mechanical knowledge and that a loss of this knowledge leads to a deficit called conceptual apraxia. He also demonstrated that the left hemisphere’s motor systems help control deftness (the making of precise movements) in both hands.

Short Biography Dr. Kenneth Heilman was born on June 2, 1938 in Brooklyn, New York. In 1970, after the completion of both residency and fellowship, he joined the faculty at the University of Florida as an assistant professor. He was promoted as associate professor in 1973 and as professor in 1975. In 1990, he received an endowed chair, becoming the first James E. Rooks, Jr. Professor of Neurology. In addition to his work at the University of Florida, he served

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Helping Alliance

Heilman, Kenneth M. (1938– ). Figure 1

on the faculty at the Veterans Affairs Medical Center in 1977, initially with part-time status as a staff neurologist. In 1996, he became Chief of the Veterans Affairs Medical Center’s Neurology Service. He has taught both medical and psychology students, and directed a Behavioral Neurology-Dementia postdoctoral program.

Cross References ▶ Anosognosia ▶ Intention ▶ Neglect

References and Readings Heilman, K. M. (1973). Ideational apraxia – A re-definition. Brain, 96, 861–864. Heilman, K. M. (1974). Neuropsychologic changes in the stroke patient. Geriatrics, 29, 153–160. Heilman, K. M. (1975a). A tapping test in apraxia. Cortex, 11, 259–263. Heilman, K. M. (1975b). Reading and writing disorders caused by central nervous system defects. Geriatrics, 30, 115–118. Heilman, K. M. (1984). Orthostatic tremor. Archives of Neurology, 41, 880–881. Heilman, K. M. (2004). Intentional neglect. Frontiers in Bioscience, 9, 694–705. Heilman, K. M., Bowers, D., Speedie, L., & Coslett, H. B. (1984). Comprehension of affective and nonaffective prosody. Neurology, 34, 917–921. Heilman, K. M., & Gilmore, R. L. (1998). Cortical influences in emotion. Journal of Clinical Neurophysiology, 15(5), 409–423. Heilman, K. M., Gonzalez-Rothi, L. J., & Valenstein, E. (1982). Two forms of ideomotor apraxia. Neurology, 32, 342–346. Heilman, K. M., Pandya, D. N., Karol, E. A., & Geschwind, N. (1971). Auditory inattention. Archives of Neurology, 24, 323–325.

Heilman, K. M., & Satz, P. (Eds.). (1983). Neuropsychology of human emotion. New York: Guilford. Heilman, K. M., Safran, A., & Geschwind, N. (1971). Closed head trauma and aphasia. Journal of Neurology, Neurosurgery, and Psychiatry, 34, 265–269. Heilman, K. M., Schwartz, H. D., & Geschwind, N. (1975). Defective motor learning in ideomotor apraxia. Neurology, 25, 1018–1020. Heilman, K. M., & Valenstein, E. (1972a). Frontal lobe neglect in man. Neurology, 22, 660–664. Heilman, K. M., & Valenstein, E. (1972b). Auditory neglect in man. Archives of Neurology, 26, 32–35. Heilman, K. M., & Valenstein, E. (1979a). Mechanisms underlying hemispatial neglect. Annals of Neurology, 5, 166–170. Heilman, K. M., & Valenstein, E., (Eds.). (1979b). Clinical neuropsychology. New York: Oxford University Press. Heilman, K. M., Valenstein, E., & Watson, R. T. (1984). Neglect and related disorders. Seminars in Neurology, 4, 209–219. Heilman, K. M., Valenstein, E., & Watson, R. T. (1994). The what and how of neglect. Neuropsychological Rehabilitation, 4(2), 133–139. Heilman, K. M., & Valenstein, E. (Eds.). (2003). Clinical neuropsychology, (4th ed.). New York: Oxford University Press. Heilman, K. M., & Watson, R. T. (1977). The neglect syndrome - A unilateral defect of the orienting response. In S. Harnad, R. W. Doty, J. Jaynes, L. Goldstein, & G. Krauthamer (Eds.), Lateralization in the nervous system (pp. 285–302). New York: Academic. Heilman, K. M., Watson, R. T., & Greer, M. (1977). The differential diagnosis of neurological diseases. New York: Appleton-CenturyCroft. Heilman, K. M., & Wilder, B. J. (1971). Evaluation and treatment of chronic simple dementias. Modern Treatment, 8, 219–230. Valenstein, E., & Heilman, K. M. (1981). Unilateral hypokinesia and motor extinction. Neurology, 31, 445–448.

Helping Alliance ▶ Therapist–Patient Relationship

Hemangioblastoma E THAN M OITRA Drexel University Philadelphia, PA, USA

Definition Hemangioblastoma is a rare slow-growing neoplasm often found in the cerebellum, including in the posterior fossa region, or found in the spinal column. Symptoms may include ataxia, discoordination, headaches, nystagmus, and vomiting. Etiology is unknown as most arise sporadically, but it may be linked to genetic abnormalities.

Hemangiopericytoma

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In approximately one quarter of all cases, they are associated with von Hippel-Lindau disease (VHL), an autosomal dominant hereditary syndrome. Standard treatment is surgical excision, sometimes preceded by preoperative embolization to reduce vascularity. Long-term prognosis is generally good and recurrence risk is relatively low, even when associated with VHL.

Cross References

Cross References


▶ Hemangioma ▶ Neoplasm

Lam, S. M., & Williams, E. F. (2002). Vascular anomalies: review and current therapy. Current Opinion in Otolaryngology & Head & Neck Surgery, 10, 309–315. Peru, A., Pavesi, G., & Campello, M. (2004). Impairment of executive functions in a patient with a focal lesion in the anterior cingulated cortex. Evidence from neuropsychological assessment. Functional Neurology, 19, 107–111.


▶ Cavernoma ▶ Cavernous Angioma ▶ Cavernous Hemangioma ▶ Neoplasm

Lonser, R., Glenn, G., Walther, M., Chew, E., Libutti, S., Linehan, W., et al. (2003). von Hippel-Lindau disease. Lancet, 361, 2059–2067.

Hemangioma E THAN M OITRA Drexel University Morgantown, WV, USA

Hemangiopericytoma J ENNIFER T INKER Drexel University Philadelphia, PA, USA

Definition Synonyms Angioma

Definition Hemangioma is the most common benign congenital neoplasm. It is classified as superficial (formerly known as capillary) or deep (formerly known as cavernous). Deep hemangiomas are located in the subcutaneous tissue and can occur in the brain. Brain hemangioma may lead to cognitive impairment including poor planning and cognitive inflexibility (e.g., Peru, Pavesi, & Campello, 2004). The formation of platelet clots is due to blood vessel tortuosity, and hemorrhage. Magnetic resonance signal of T1 hyperintense perilesional signal abnormality has been shown to help differentiate deep hemangiomas from hemorrhagic tumors and intracerebral hemorrhages. Etiology is unknown. Treatment options vary and the decision to intervene or not remains controversial (Lam & Williams, 2002).

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A hemangiopericytoma (HPC) is a highly vascularized soft tissue neoplasm arising from pericytes (connective tissue cells) encapsulating capillaries and postcapillary venules. CNS hemangiopericytomas are almost exclusively found to occur within the epidural space of the brain and spinal cord, without infiltrating the surrounding CNS parenchyma. Previously considered a variant of ‘‘angioblastic meningioma,’’ CNS hemangioperictyoma is now classified as a distinct entity by the World Health Organization (Jaaskelainen, Louis, Paulus, & Haltia, 2000). Hemangiopericytomas are particularly aggressive tumors that have high rates of metastasis and a tendency to recur, even following gross-total resection, which is the principal treatment strategy (32–67% of published cases). Although these tumors are extra-axial, they can cause limited cognitive impairment, but the mechanism is not clear. These tumors occur primarily in adults (median age 45 years), with fewer than 10% of reported occurrences in children (Ecker et al., 2003). Radiotherapy is also an adjuvant therapy, and cognitive impairments can be associated with these tumor treatments.

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Cross References ▶ Meningioma

and location, and whether it causes swelling and inflammation. Some hematomas are managed with observation, while others require surgical treatment.


Cross References

Ecker, R. D., Marsh, W. R., Pollock, B. E., Kurtkaya-Yapicier, O., McClelland, R., Scheithauer, B. W. et al. (2003). Hemangiopericytoma in the central nervous system: Treatment, pathological features, and long-term follow up in 38 patients. Journal of Neurosurgery, 98, 1182–1187. Jaaskelainen, J., Louis, D. N., Paulus, W., & Haltia, M. J. (2000). Haemangiopericytoma. In P. Kleihues & W. K. Cavenee (Eds.), World health organization classification of tumours. Pathology & genetics. Tumours of the nervous system (pp. 190–192). Lyons, France:0020IARC Press.

▶ Hemorrhage ▶ Hemorrhagic Stroke ▶ Subdural Hematoma

References and Readings Zimmerman, L. H. (2007). Causes and consequences of critical bleeding and mechanisms of blood coagulation. Pharmacotherapy, 27(9, pt 2), 45S–56S.

Hematoma E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms

Hemiagnosia ▶ Hemispatial Neglect ▶ Hemiinattention ▶ Neglect Syndrome ▶ Visual Neglect

Blood clot; Bruise

Definition A hematoma is a collection of blood outside of the blood vessels, usually resulting from internal hemorrhage. It could derive from an artery, vein, or capillary, when any of them are damaged and blood leaks out of the vessel. Hematomas that are bruises of the skin and soft tissue are called ecchymoses; Blood collections inside muscles are called intramuscular hematomas, while blood collections between muscle layers are called intermuscular hematomas.

Hemianopia M ARY-E LLEN M EADOWS Brigham and Women’s Hospital Boston, MA, USA

Short Description or Definition A hemianopia refers to a loss of vision in one-half of the visual field.

Current Knowledge Bleeding into the skull and brain may cause intracranial, intracerebral, subdural, or epidural hematomas. While a hematoma is a stable collection of blood, hemorrhage is the term applied to the active process of bleeding. Blood that escapes from the blood vessel is irritating to the tissues and may cause inflammation and pain, swelling, and redness. Symptoms of a hematoma depend on its size

Categorization There are various subtypes of hemianopia that are caused by different lesions along the visual system. A bitemporal heimanopia can occur by damage to the optic chiasm. This is usually caused by intercerebral masses that press against the chiasm, such as a pituitary adenoma,

Hemi-attention Syndrome

meningioma, craniopharyngioma, or hypothalamic glioma. Lesions of the lateral geniculate nucleus in the thalamus may result in a contralateral homonymous hemianopia. Other visual defects can occur that result in a quandrantanopia, which is a loss of vision in selected visual field quadrants. These occur in lesions of the temporal lobe in the optic radiations. However, damage to the entire optic radiation on one side can cause a contralateral homonymous hemianopia.

Natural History, Prognostic Factors, Outcomes Hemianopia can be caused by a brain tumor, or stroke, or trauma to the brain. An embolic stroke that affects the left posterior cerebral artery can also cause a right visual field loss. Individuals with posterior cortical atrophy or progressive visuospatial dysfunction can also develop a hemianopia. Not all hemianopias are caused by disease or trauma. Some individuals can have an aura that resembles a homonymous hemianopia as a prelude to migraine or seizure.

Evaluation Visual field testing done at the bedside can assess for visual field loss. The patient is instructed to fixate on the examiner’s eye or nose while the examiner uses his/her fingers or other stimulus to test the patient’s visual fields. All quadrants are tested, and are based on the cooperation of the patient. Blink to threat may be useful in assessing visual fields in uncooperative or lethargic patients. Formal visual field testing is done using a manual Goldman perimetry. Lights of different sizes and intensities are displayed on a screen in front of a patient. Automated computer versions are now available, but are limited with respect to the central 30 of the visual field.

Treatment If a hemianopia is the initial presenting symptom of a stroke and the individual is a candidate for medication that dissolves blood clots, such as tissue plasminogen activator (tPA), then the residual deficits may be ameliorated or lessened. However, treatment needs to be initiated within the first 2 or 3 h of the first symptoms. In general, the effects of hemianopia are usually permanent with respect to visual field loss, though patients

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may be able to learn compensatory strategies to help them cope with their deficits. This is typically done by occupational therapists or visual rehabilitation therapists. Behavioral optometrists can also help individuals learn strategies.

Cross References ▶ Homonymous Quandrantanopia ▶ Visual System

References and Readings Bradley, W. G., Daroff, R. B., Fenichel, G. M., & Marsden, C. D. (2000). Neurology in clinical practice: Principles of diagnosis and management. Boston, MA: Butterworth-Heinemann.

Hemi-attention Syndrome J ANNA L. H ARRIS University of Kansas Medical Center Kansas City, KS, USA

Synonyms Hemi-neglect; Unilateral neglect

Definition Hemi-attention is the failure to respond to sensory stimuli within one hemispace after an acquired lesion to the contralateral hemisphere of the brain. Hemi-attention is observed more frequently after damage to the right hemisphere than the left, and may result from lesions within several brain regions including parietal cortex, frontal cortex, cingulate gyrus, basal ganglia, or cerebellum. A patient with hemi-attention syndrome faces significant difficulties in day-to-day life, as he/she may fail to identify objects on one side of the visual field, fail to localize sounds originating from the contralesional hemispace, fail to dress one side of his/her body, or shave one side of his face. Hemi-attention patients are often unaware that they have any deficit. For many patients the symptoms of hemi-attention syndrome resolve over the course of several months.

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Cross References ▶ Anosognosia ▶ Hemispatial Neglect ▶ Neglect and Hemi-inattention ▶ Neglect Syndrome

Hemikinesis H. B RANCH C OSLETT University of Pennsylvania Philadelphia, PA, USA

Synonyms

Hemiinattention Hemispatial motor planning deficit; Hypokinesia M ARK M ENNEMEIER University of Arkansas for Medical Sciences Little Rock, AR, USA

Synonyms Amorphosynthesis; Hemiagnosia; left (or right) neglect; Spatial neglect; Unilateral neglect; Visual neglect; Visuospatial angnosia; Visuospatial neglect

Definition Hemiinattention is defined as the failure to report or respond to stimulation located contralateral to a brain lesion when the failure cannot be attributed to a primary sensory loss or motor impairment (Heilman, Watson, & Valenstein, 1985). Patients with hemiinattention may be able to detect stimulation when their attention is drawn to stimuli in contralateral space, but they may become inattentive to stimuli in ipsilateral space. Hemiinattention implies that neglect is most pronounced in hemispace located opposite brain injury.

Cross References ▶ Hemi-attention Syndrome ▶ Hemispatial Neglect ▶ Inferior Parietal Area ▶ Neglect Syndrome ▶ Neglect ▶ Visual Neglect

References and Readings Heilman, K. M., Watson, R. T., & Valenstein, E. (1985). Neglect and related disorders. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (2 ed., pp. 243–293). New York: Oxford University Press.

Definition Hemiakinesia is a disorder of action in which subjects with a brain lesion exhibit difficulty in moving the contralesional body that cannot be attributed to hemiparesis or peripheral nervous system dysfunction. Subjects with this disorder may fail to gaze to the side opposite a lesion or, when instructed to lift both arms, may fail to elevate the contralesional arm. Crucially, these subjects may be shown in other tasks to have the capacity to perform the task; for example, the subject who fails to lift the contralesional arm when instructed to elevate both arms may succeed in lifting the arm when instructed to lift only that arm.

Current Knowledge Evidence for the clinical disorder of hemiakinesia comes from the often-striking effects of hemispace on motor performance. A number of investigators have demonstrated that subjects may perform better when using the impaired limb in the contralesional as opposed to ipsilesional hemispace (e.g., Coslett, Schwartz, Goldberg, Haas, & Perkins, 1993). One subject was both stronger (as judged by grip strength) as well as more dextrous (as judged by finger tapping) when using the contralesional hand in the ipsilesional when compared with the contralesional hemispace. The hemispace effect was observed not only with the impaired hand but also with the ‘‘good’’ hand; he performed significantly less well on motor tasks with the ipsilesional hand in the contralesional hemispace. Similar effects were observed in a group study. Coslett (1999) found that 8 of 30 subjects with unilateral hemispheric strokes performed significantly less well on a finger tapping or grip strength task (or both) when acting in the contralesional when compared with ipsilesional hemispace; all subjects who exhibited this effect had lesions involving the parietal lobe whereas no subject without the involvement of the

Hemiparesis

parietal lobe exhibited this effect. In this study, language performance was also influenced by the location to which subjects attended, demonstrating that at least for some subjects, hemiakinesia may be a manifestation of a supramodal processing deficit. Additional evidence for the role of hemiakinesia in impaired motor performance comes from the performance of subjects with medial, frontal, and/or callosal lesions. Not uncommonly, these subjects may be unable to move one hand when instructed to do so and may fail to use the hand in activities of daily living. When asked to perform bimanual tasks (tie a shoe), however, the same subjects may use the affected hand dexterously and effectively. Subjects with an ‘‘alien hand’’ may show improvement on bimanual tasks that are hemispace specific; for example, one subject with a right medial frontal lesion was able to use his ‘‘alien’’ hand to tie a shoe in the right but not left hemispace (Coslett, unpublished observations). Hemiakinesia is assumed to be a ‘‘pre-motor’’ impairment that reflects a disruption of the mechanism(s) by which descending motor systems are controlled or activated. In other words, hemiakinesia is a disorder in which spatial or ‘‘high-level’’ motor skills are impaired. The fact that the motor skills may be eliminated by directing attention to the effector or simply changing the location in which the action is performed demonstrate that it is not simply attributable to a corticospinal tract deficit. Thus, hemiakinesia is, in principle, differentiated from hemiparesis in that the latter is assumed to reflect a ‘‘lower-level’’ deficit that disrupts the implementation of the motor plan or the transmission of this information to the motor effectors. Hemiakinesia and hemiparesis are not mutually exclusive and frequently co-exist. Hemiakinesia is most commonly observed in the context of the neglect syndrome. It can also be observed, however, after left hemisphere lesions (Coslett, 1999; Coslett et al., 1993). Whereas some observations suggest that hemiakinesia is most frequently observed with parietal lesions (Coslett, 1999), a recent transcranial magnetic stimulation study in normal subjects demonstrated that motor-intentional neglect, a disorder closely related to hemiakinesia, is induced by frontal rather than parietal stimulation.

Cross References ▶ Akinesis ▶ Hemiinattention ▶ Neglect Syndrome

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References and Readings Coslett, H. B. (1999). Spatial influences on motor and language function. Neuropsychologia, 37, 695–706. Coslett, H. B., Schwartz, M. F., Goldberg, G., Haas, D., & Perkins, J. (1993). Multi-modal hemispatial deficits after left hemisphere stroke: A deficit in attention? Brain, 116, 527–554. Ghacibeh, G. A., Shenker, J. I., Winter, K. H., Triggs, W. J., & Heilman, K. M. (2007). Dissociation of neglect subtypes with transcranial magnetic stimulation. Neurology, 69, 1122–1127. Heilman, K. M., Valenstein, E., & Watson, R. T. (2000). Neglect and related disorders. Seminars in Neurology, 20, 463–470.

Hemi-Neglect ▶ Hemi-attention Syndrome

Hemiparesis T HESLEE J OY D E P IERO Boston University School of Medicine Boston, MA, USA

Definition Hemiparesis is weakness of the arm and leg on the same side of the body. The face may or may not be involved. Hemiparesis may be mild to severe. It is almost always caused by lesions involving the corticospinal tract.

Pattern of Weakness Weakness caused by lesions of the corticospinal tract causes different muscle groups to be weakened in a particular pattern, often called predilection pattern weakness. In the upper extremity, the extensors are weaker than the flexors: the arm is held close to the body (adducted), and flexed at the elbow, pronated at the forearm, and flexed at the wrist and fingers. In the lower extremity, the leg is extended at the hip, knee and plantar flexed at the ankle. Drift or pronator drift refers to an examination technique that can detect mild weakness in the upper extremity due to corticospinal tract lesions: with the eyes closed, the patient’s arms are extended forward from the body, parallel to the floor, palms up, activating all the muscles that are most affected in corticospinal (or upper

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motor neuron) lesions. If there is no injury to the corticospinal tract, the arms remain in that position. If there is a mild injury to the corticospinal tract, the arm on the affected side drops slightly at the shoulder, flexes at elbow, wrist, and fingers, and the forearm pronates.

or total paralysis, as opposed to hemiparesis, which implies some retained movement.

Causes

Hemiparesis may be spastic or flaccid, depending on the cause and acuity. In the acute phase, flaccid paralysis, with muscle tone less than normal, is most common. With time, spasticity develops, manifest by increasing muscle tone in all muscles, especially the arm flexors and leg extensors in most cases. Slowly developing lesions, such as tumors often have no flaccid phase.

Hemiplegia is most commonly caused by lesions of the corticospinal tract in the brain, brain stem, or cervical spinal cord. Depending on the location of the lesion, there may be accompanying abnormalities, such as cognitive or language disorders, visual impairments, or sensory disorders. In lacunar strokes, the hemiplegia involves the face, as well as arm and leg. Dysarthria may be present, but there are no sensory, cognitive, or language deficits. In the spinal cord, there are almost always other abnormalities associated with hemiplegia, typically sensory deficits.

Causes

Types

In the brain: stroke, hemorrhage (intracerebral, subdural, subarachnoid), trauma, infection, and tumors; in the cervical spine: trauma, infection, disk disease, spondylosis, tumors, and epidural hemorrhage. Children with cerebral palsy often have a dystonic component to their hemiparesis.

Hemiplegia may be spastic or flaccid. Often there is initial flaccidity followed by spasticity.

Types

Cross References ▶ Corticospinal Tract ▶ Pure Motor Hemiparesis

Etiology Stroke, tumors, trauma, hemorrhages, and congenital.

Cross References ▶ Corticospinal Tract ▶ Hemiparesis ▶ Lacunar Stroke

References and Readings References and Readings Victor, M., & Ropper, A. H. (2001). Adams and Victor’s principals of neurology (7th ed.). New York: McGraw-Hill.

Hemiplegia T HESLEE J OY D E P IERO Boston University School of Medicine Boston, MA, USA

Definition Paralysis of one side of the body: arm and leg, with or without weakness of the face. Hemiplegia implies a severe

Victor, M., & Ropper, A. H. (2001). Adams and Victor’s principals of neurology (7th ed.). New York: McGraw-Hill.

Hemisection of Spinal Cord ▶ Brown–Se´quard Syndrome

Hemispatial Motor Planning Deficit ▶ Hemikinesis

Hemispatial Neglect

Hemispatial Neglect M ARK M ENNEMEIER University of Arkansas for Medical Sciences Little Rock, AR, USA

Synonyms Hemiagnosia; Left (or right) neglect; Spatial neglect; unilateral neglect; Visual neglect; Visuospatial angnosia; Visuospatial neglect

Definition Hemispatial neglect is one element of the neglect syndrome. Neglect is operationally defined as the failure to report, respond or orient either to external sensory stimulation or mental representations of sensory events when the failure is not attributable to a primary sensory or motor deficit (Heilman, Watson, & Valenstein, 1985). Hemispatial neglect denotes the consistency with which neglect occurs on one side of space located contralateral to brain injury. Hemispace is not synonymous with the sensory hemifields; rather, each cerebral hemisphere is presumed to direct attention and movement in contralateral hemispace independent of the sensory hemifields or of the extremity used to operate in hemispace. In other words, the cerebral hemispheres are organized hemispatially with regard to attention and intention. While neglect may not be strictly confined to contralateral space because it can also be observed ipsilateral to brain injury, hemispatial neglect on tasks with functional significance is certainly most pronounced in space located contralateral to brain injury (i.e., contralesional space) (Mark, 2003). In fact, one would not diagnose hemispatial neglect without this condition being satisfied.

Current Knowledge This section provides a brief overview of current knowledge about hemispatial neglect. The reader is referred to entries in this volume entitled ‘‘the neglect syndrome’’ and ‘‘visual neglect’’ for a more comprehensive discussion. In daily activities, patients with hemispatial neglect frequently pocket food on the contralateral side of the mouth and throat, fail to dress or groom the contralateral side of the body, and fail to recognize salient stimulation located in contralesional space. Bedside tests for

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hemispatial neglect include drawing and copying tasks, line bisection, target cancellation, and reading tasks. Hemispatial neglect is indicated by failing to either reproduce or to correctly position the contralesional features of drawings; by bisecting standard length lines on the ipsilesional side of true center; by failing to cancel targets on the contralesional side of the page; and by failing to read portions of words, sentences, or paragraphs located contralesional to brain injury (Chatterjee & Mennemeier, 1998). Whereas many of the bed side tests of neglect are visual, it is important to recognize that hemispatial neglect can be observed in multiple sensory modalities. Patients with neglect may fail to acknowledge sounds or voices originating in contralesional space, and they may extinguish auditory and tactile stimulation on the contralesional side of the body when both sides of the body are stimulated at the same time (extinction to double simultaneous stimulation). They may report stimulation of the ipsilesional limb when the contralesional limb has been touched (allesthesia). Hemispatial neglect is associated with damage to supramodal processing areas including the inferior parietal, superior temporal, anterior cingulate, and dorsolateral prefrontal cortices (Chatterjee & Mennemeier, 1998; Karnath, Ferber, & Himmelbach, 2001; McIntosh & Milner, 2005). Subcortical lesions that produce neglect involve the basal ganglia and thalamus. Neglect is more frequent, severe and lasting after right hemisphere damage, but it also occurs following damage to similar brain areas in the left hemisphere (Ringman, Saver, Woolson, Clarke, & Adams, 2004). Anatomical, behavioral, and experimental observations suggest that neglect is primarily a disorder of spatial attention, arousal and of the intention to move in space (Chatterjee & Mennemeier, 1998). Associated deficits in spatial working memory, sustained attention, and estimating stimulus intensity contribute to and possibly explain different features of hemispatial neglect (Chatterjee & Mennemeier, 1998; Husain & Rorden, 2003). Hemispatial neglect is a severely debilitating disorder that predicts poor outcome following rehabilitation (Chatterjee & Mennemeier, 1998; Mark, 2003). Hemispatial neglect is responsive to behavioral and pharmacological interventions, but no widely efficacious form of treatment exists for hemispatial neglect at the present time (Pierce & Buxbaum, 2002).

Cross References ▶ Inferior Parietal Area ▶ Neglect Syndrome ▶ Visual Neglect

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References and Readings Chatterjee, A., & Mennemeier, M. (1998). Diagnosis and treatment of spatial neglect. In R. B. Lazar (Ed.), Principles of neurologic rehabilitation (pp. 597–612). New York: McGraw-Hill. Heilman, K. M., Watson, R. T., & Valenstein, E. (1985). Neglect and related disorders. In K. M. Heilman & E. Valenstein (Eds.), Clinical Neuropsychology (2 ed., pp. 243–293). New York: Oxford University Press. Husain, M., & Rorden, C. (2003). Non-spatially lateralized mechanisms in hemispatial neglect. Nature Reviews Neuroscience, 4, 26–36. Karnath, H. O., Ferber, S., & Himmelbach, M. (2001). Spatial awareness is a function of the temporal not the posterior parietal lobe. Nature, 411, 950–953. Mark, V. W. (2003). Acute versus chronic functional aspects of unilateral spatial neglect. Frontiers in Bioscience, 8, 72–89. McIntosh, R. D., & Milner, A. D. (2005). The neurological basis of visual neglect. Current Opinion in Neurology, 18, 748–753. Pierce, S. R., & Buxbaum, L. J. (2002). Treatments of unilater neglct: A review. Archives of Physical Medicine and Rehabilitation, 83, 256–268. Ringman, J. M., Saver, J. L., Woolson, R. F., Clarke, W. R., & Adams, H. P. (2004). Frequency, risk factors, anatomy, and course of unilateral neglect in an acute stroke cohort. Neurology, 63, 468–474.

Hemispherectomy J OHN W HYTE Moss Rehabilitation Research Institute, Albert Einstein Healthcare Network Elkins Park, PA, USA

Definition Hemispherectomy refers to a radical neurosurgical procedure in which a complete cerebral hemisphere is removed. The most common indication for the procedure is Rasmussen syndrome, a form of epilepsy associated with progressive destruction of one cerebral hemisphere. The outcome of hemispherectomy with respect to epilepsy and functional abilities depends on the underlying disease being treated. Across a mixed sample of hemispherectomy patients, however, the majority are seizure-free postoperatively, and many are free of anticonvulsants as well.

Current Knowledge Hemispherectomy, though a disabling procedure, may result in an improved quality of life when compared

with the constant seizures and toxic effects of anticonvulsant drugs used in an attempt to control them. In addition, it has provided a fascinating window into the limits of neural plasticity. Although cognitive function and IQ are below normal in many hemispherectomy patients, a surprising amount of function normally attributed to the removed hemisphere typically remains. For example, those with left hemisphere removals show preservation of language, although with greater impairment than those with right hemisphere removals. Similarly, bilateral motor activity is possible for many patients, and imaging studies suggest the development of ipsilateral movement control.

Cross References ▶ Epilepsy

References and Readings Bernasconi, A., Bernasconi, N., Lassonde, M., Toussaint, P. J., Meyer, E., Reutens, D. C., et al. (2000). Sensorimotor organization in patients who have undergone hemispherectomy: A study with (15)O-water PET and somatosensory evoked potentials. Neuroreport, 11(14), 3085–3090. Bode, S., Firestine, A., Mathern, G. W., & Dobkin, B. (2005). Residual motor control and cortical representations of function following hemispherectomy: Effects of etiology. Journal of Child Neurology, 20(1), 64–75. Pardo, C. A., Vining, E. P. G., Guo, L., Skolasky, R. L., Carson, B. S., & Freeman, J. M. (2004). The pathology of Rasmussen syndrome: Stages of cortical involvement and neuropathological studies in 45 hemispherectomies. Epilepsia, 45(5), 516–526. Pulsifer, M. B., Brandt, J., Salorio, C. F., Vining, E. P. G., Carson, B. S., & Freeman, J. M. (2004). The cognitive outcome of hemispherectomy in 71 children. Epilepsia, 45(3), 243–254. Terra-Bustamante, V. C., Fernandes, R. M. F., Inuzuka, L. M., Velasco, T. R., Alexandre, V., Jr., Wichert-Ana, L., et al. (2005). Surgically amenable epilepsies in children and adolescents: Clinical, imaging, electrophysiological, and post-surgical outcome data. Childs Nervous System, 21(7), 546–551. Vanlancker-Sidtis, D. (2004). When only the right hemisphere is left: Studies in language and communication. Brain & Language, 91(2), 199–211.

Hemispheric Asymmetry (Anatomical) ▶ Hemispheric Specialization

Hemispheric Specialization

Hemispheric Disconnection ▶ Split-Brain

Hemispheric Specialization J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Synonyms Hemispheric asymmetry (anatomical)

Definition The concept refers to the fact that each cerebral hemisphere has unique functional characteristics and mediates different aspects of behavior. While hemispheric specialization (HS) certainly includes the phenomena of contralateral motor and sensory representation, the concept most often arises in the context of higher cognitive or perceptual rather than more elementary sensorimotor functions.

Current Knowledge Awareness of HS was not firmly established in the scientific community until the middle of the nineteenth century when clinical case studies revealed that damage limited to one cerebral hemisphere resulted in behavioral deficits that were qualitatively different from those following lesions of the other hemisphere. In the middle of the twentieth century, hemispheric asymmetries began to be identified which suggested links between these anatomical and behavioral differences. Among the more prominent differences are the increased size of the planum temporale in the left hemisphere (thought to subserve the processing of verbal input) and the asymmetries in the posterior ascending ramus of the lateral fissure. Wada studies in which a barbiturate was injected into one carotid artery suppressing the activity of the ipsilateral cerebral hemisphere, dichotic listening tasks, and the tachistoscopic presentation of stimuli to one visual field offered

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additional methods of assessing HS in the intact brain. Recent advances in imaging techniques such as functional magnetic resonance imaging (fMRI) have provided less intrusive, more reliable means of studying similar behavioral phenomena in normal subjects. In terms of functional asymmetries, the most reliable clinical differences are found in the motor, somatosensory, and visual systems where contralateral representation is clearly evidenced. The right hemisphere controls the limbs on the left side of the body (including voluntary conjugate eye movements to the left), while the left hemisphere controls the right extremities. Similarly, visual and somatosensory inputs from the left side of space or left side of the body are mediated by the right hemisphere, and vice versa for right-sided stimulation. Hearing is different in that there is a greater degree of bilateral input from the two ears, although even here, there is a preponderance of contralateral input. As noted, within the field of neuropsychology, the concept of HS is most commonly associated with complex cognitive and perceptual processes. Based on postmortem studies of a series of patients who had suffered from aphasia, by the middle of the nineteenth century, language was the first such behavior asymmetry to be clearly documented and associated with damage to the left cerebral hemisphere. By the first part of the twentieth century, the left hemisphere was also established as being critical in controlling certain skilled activities (associated with apraxia), regardless of which hand was being used. By contrast, initially, the role of the right hemisphere in higher order functions was less obvious. However, by the early twentieth century, it was viewed as superior in attending to and grasping situations as a whole and in managing certain types of visual-spatial or visualperceptual problems. Later it came to be recognized as being critical in understanding and expressing emotions. Based on these findings, it has been hypothesized that not only are the hemispheres distinct with regard to the types of information they process, but that they also likely handle information in somewhat different fashions. Thus, the left hemisphere is believed to excel at processing input in a logical, analytical, and sequential manner, while the right hemisphere is superior at taking a global, wholistic, and ‘‘intuitive’’ approach. Given the foregoing discussion, it is tempting to think of the brain in dichotomous terms – the left hemisphere handles this type of task, while the right hemisphere processes that type of information. But this does not appear to be the case. Stimuli and the behavioral responses to them are highly complex and multifactorial. For any given language-based task, such as listening to

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someone speak or responding verbally, one might think of the left hemisphere as the critical or leading hemisphere. But it is not acting alone even with regard to these very specific tasks. While the left hemisphere is likely essential for decoding speech sounds into meaningful auditory percepts, attaching semantic relevance to these percepts, and appreciating the syntax or grammatical structure of the sentences, the right hemisphere contributes by attending to the inflections and tone of the voice, as well as the context in which this communication takes place. In some situations, the message conveyed by the latter components is more important than, and perhaps even diametrically opposite, that expressed by the words themselves. Whether considered on a micro (e.g., a very narrow column of cortical cells) or macro (e.g., an entire gyrus), the various units of the brain work together to different degrees and in different ways or combinations in executing a behavioral response in what has been described as a distributed systems approach to understanding brain organization and function. This also applies to interhemispheric cooperation. Finally, the foregoing description of hemispheric specialization is generally predicated on the assumption that the left hemisphere is the ‘‘dominant’’ hemisphere that is responsible for propositional speech and language comprehension and controls the ‘‘favored’’ hand. If an individual is left-handed, the above pattern may not always apply. In some cases, for example, both hemispheres may be more or less adept at processing syntactic and semantic aspects of language. In other cases, the hemisphere that is ‘‘dominant’’ for language may not be the same hemisphere that controls the ‘‘dominant’’ hand. ‘‘Anomalous dominance’’ is the term used to describe such instances.

Cross References ▶ Anomalous Dominance ▶ Asymmetry ▶ Distributed Systems ▶ Dominance (Cerebral) ▶ Global Versus Local Processing

References and Readings Hannay, H. (Ed.). (1986). Experimental techniques in human neuropsychology. New York: Oxford University Press.

Kinsbourne, M. (Ed.). (1978). Asymmetrical function of the brain. London: Cambridge University Press. Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach (pp. 334–368). New York: Springer. Mesulam, M.-M. (1990). Large-scale neurocognitive networks and distributed processing for attention, language and memory. Annals of Neurology, 28, 597–613.

Hemisphericity J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition Hemisphericity refers to a theoretical and controversial construct suggesting that some individuals have an innate superiority or habitual propensity for processing information with one or the other cerebral hemisphere, more or less independent of situational demands. This notion is predicated on the assumptions that (1) the two hemispheres differ in their ability to process certain types of information (e.g., verbal for the left, and emotional and visual–spatial for the right), and (2) the two hemispheres process information in fundamentally different ways. Thus, the left hemisphere is thought to operate in a more analytical, logical, sequential manner, while the right hemisphere is seen as functioning in a more wholistic, intuitive, simultaneous manner. At the core of the controversy is whether individuals can be classified on an a priori basis as being either predominantly ‘‘right-brained’’ or ‘‘left-brained’’ and whether this has broader implications for their personality, mood, and general behavioral approach to their environment, as well as particular cognitive-perceptual abilities. According to this theory, left-brained individuals are expected to be more logical, organized, and less emotional than the right-brained, whereas the latter are more spontaneous, creative, and better able to ‘‘see the big picture.’’ Virtually no empirical support exists for this dichotomy.

Cross References ▶ Hemispheric Specialization

Hemodynamic Response

Hemodynamic Response E DUARDO L OPEZ JFK Medical Center, Johnson Rehabilitation Institute Edison, NJ, USA

Synonyms Autonomic dysregulation; Brain storming; Diencephalic seizures; Tonic fit

Current Knowledge The autonomic nervous system is of prime importance in regulation of the heart rate and contractility. After acute trauma, an immediate sympathetic surge with massive catecholamine response occurs to compensate for the effects of the injury. Cardiac abnormalities have been previously correlated with significant increases (threefold or greater) in plasma catecholamine levels after subarachnoid hemorrhage with the degree of catecholamine release directly related to the severity of the brain injury (Clifton & Ziegler, 1981). Cardiac dysfunction as a result of hyperadrenergic state will be observed in 20% or more of the patients and characterized by global myocardial dysfunction and electrocardiographic (EKG) changes. Development of ST–T wave changes as well as fatal ventricular arrhythmias are observed in these patients. Traumatic brain injury (TBI) associated sympathetic hyperactivity has also been demonstrated to cause ventricular wall motion abnormalities (hypokinesia) in over 50% of patients (Cotton et al., 2007). In multisystem injury, there may also be brain lesions causing myocardial contusion, which may cause tachycardia and EKG changes similar to those. With isolated TBI, neurogenic hypotension has been found to occur in one study and associated with a higher mortality than that from hemorrhagic hypotension. In addition to reflecting the severity of injury neurogenic myocardial dysfunction may be deleterious. The clinical syndrome may be a result of a ‘‘release phenomenon,’’ within the brainstem and/or diencephalons, loss of overriding cortical or subcortical inhibition (Bullard, 1987), while others suggest localization to the central sympathoexcitatory regions. Evidence of alteration in hemodynamic response as a consequence of

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sympathetic overactivity has been demonstrated in both acute and chronic acquired brain injury (Baguley, Heriseanu, Nott, Chapman, & Sandanam, 2009).

Treatment The standard treatment for sympathetic hyperactivity during the acute phase includes short acting opiates or benzodiazepines and adrenergic blockers. Unfortunately sedating side effects can further decrease the level of consciousness in these low level patients. Other medications that have also shown effectiveness in symptomatic treatment include dantrolene and bromocriptine (Rodney, 2001). Frequently, it is through trial and error that the appropriate effective medications are found. Stabilization of symptoms may signify improved regulatory control, therefore, slow withdrawal of medication is considered. Treatment is aimed at controlling the duration and severity of the symptoms to improve prognosis and prevent additional brain injury.

Cross References ▶ Antihypertensive ▶ Autonomic Nervous System ▶ Benzodiazepine ▶ Bromocriptine ▶ Cholinergic System

References and Readings Baguley, I. J., Heriseanu, R. E., Nott, M. T., Chapman, J., & Sandanam, J. (2009). Dysautonomia after severe traumatic brain injury. American Journal of Physical Medicine & Rehabilitation, 88, 615–622. Bullard, D. E. (1987). Diencephalic seizures: Responsiveness to bromocriptine and morphine. Annals of Neurology, 21, 609–611. Clifton, G. L., & Ziegler, M. G. (1981). Circulating catecholamine and sympathetic activity after brain injury. Neurosurgery, 8, 10–14. Cotton, B. A., Snodgrass, K. B., Fleming, S. B., Carpenter, R. O., Kemp, C. D., Arbogast, P. G., et al. (2007). Beta-blocker exposure is associated with improved survival after severe traumatic brain injury. The Journal of Trauma, 62, 26–35. RossZygun, D. A., Kortbeek, J. B., Fick, G. H., Laupland, K. B., & Doig, C. J. (2005). Non-neurologic organ dysfunction in severe traumatic brain injury Critical Care Medicine, 33, 654–660.

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Hemoglobin

Hemoglobin N ATHAN D. Z ASLER Concussion Care Centre of Virginia, Ltd. Glen Allen, Virginia, USA Tree of Life Services, Inc. Richmond, Virginia, USA VCU Department of Physical Medicine and Rehabilitation Richmond, Virginia, USA University of Virginia Charlottesville, Virginia, USA

Definition Hemoglobin is the protein molecule in red blood cells that carries oxygen from the lungs to the body’s tissues and returns carbon dioxide from the tissues to the lungs. Hemoglobin is made up of four protein molecules (globulin chains) that are connected together. The normal adult hemoglobin (Hgb) molecule contains two alpha-globulin chains and two beta-globulin chains. In fetuses and infants, there are only a few beta chains and the hemoglobin molecule is made up of two alpha chains and two gamma chains. As the infant grows, the gamma chains are gradually replaced by beta chains. Each globulin chain contains an important central structure called the heme molecule. Embedded within the heme molecule is iron that transports the oxygen and carbon dioxide in blood. The iron contained in hemoglobin is also responsible for the red color of blood. Hemoglobin also plays an important role in maintaining the shape of the red blood cells. Abnormal hemoglobin structure can, therefore, disrupt the shape of red blood cells and impair function and flow through blood vessels. The hemoglobin level in the body is expressed as the amount of hemoglobin in grams (gm) per deciliter (dl) of whole blood, a deciliter being 100 ml. The normal ranges for hemoglobin depend on the age and, beginning in adolescence, the sex of the person. The normal ranges for adults are between 12–18 gm/dl and for children, depending on age, 11–22 gm/dl. Anemia occurs when the blood hemoglobin is low. Some of the more common reasons are loss of blood (traumatic injury, surgery or pathologic blood loss), nutritional deficiency (vitamin B12, folate, and/or iron), bone marrow problems (replacement of bone marrow by cancer, suppression by drugs, kidney failure),

and/or physiologically abnormal hemoglobin (sickle cell anemia). Higher than normal hemoglobin levels can be seen due to residing at high altitudes or secondary to chronic smoking. Dehydration may produce falsely high hemoglobin, which resolves when proper fluid balance is restored. Some other less frequent causes of high hemoglobin are lung disease, certain tumors, polycythemia vera, and abuse of the drug erythropoietin (Epogen). Polycythemia vera may be due to a myeloproliferative condition or it may be a reaction to chronically low oxygen levels or, rarely, a malignancy. Physical findings that may provide clues as to the etiology of anemia include, but are not limited to liver or spleen enlargement, lymphadenopathy, jaundice including scleral icterus, bone tenderness, new onset neurological signs or symptoms, and occult or frank blood loss. Anemia, particularly in older individuals, may negatively impact physical, as well as cognitive function.

References and Readings Blinder, M. A. (1998). Anemia and transfusion therapy. In C. F. Carey, H. H. Lee, K. F. Woeltje, & R. A. Schaiff (Eds.), The Washington manual of medical therapeutics (pp. 360–374). Philadelphia, PA: Lippincott Williams & Wilkins. Chaves, P. H. (2008). Functional outcomes of anemia in older adults. Seminars in Hematology, 45(4), 255–260. Rodak, B. F., Fritsma, F. A., & Doig, K. (2007). Hematology: Clinical principles and applications. St. Louis, MO: Elsevier Science.

Hemoglobin SS Disease ▶ Sickle-Cell Disease

Hemorrhage E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Bleeding

Hemorrhagic Stroke

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Definition

Definition

Hemorrhage is active bleeding, in which blood escapes from the blood vessels, either into the internal organs and tissues or outside of the body. It may be caused by trauma, vascular disease, clotting disorders, or other diseases. Virtually any organ may hemorrhage, but common locations for internal hemorrhaging include the brain, stomach, oral cavity, small and large intestines, and abdominal cavities. When it is uncontrollable, a hemorrhage can become a medical emergency. Symptoms depend on the size, rate, and location of the bleeding, but at times, certain hemorrhages may be asymptomatic and undetected. It is usually treated by compression of the vessel, closure of the site of leakage, enhancement of clotting mechanisms, or removal of the inciting factor. These actions may require local compression, use of selected medications, or surgical intervention.

A hemorrhagic stroke results from extravasation of blood from blood vessels into the brain. This occurs when the vessel walls are weak, as occurs with sudden acute episodes of extreme high blood pressure or in the presence of cerebral arteriovenous malformations or aneurysms. There are two main types of hemorrhagic strokes: intracerebral hemorrhage causing bleeding into to the brain tissue itself and accounting for 10–15% of all strokes, and subarachnoid hemorrhage causing bleeding between the protective layers that surround the brain and accounting for about 5% of all strokes. Intracerebral hemorrhages are most commonly caused by high blood pressure, but other causes exist as well, such as cocaine and amphetamine use, cerebral amyloid angiopathy, cerebral vasculitis, and bleeding disorders. Symptoms typically have an abrupt onset, consisting of severe headache, usually while awake, nausea and emesis, weakness or paralysis, sensory loss, aphasia or dysarthria, visual changes, confusion or altered consciousness. Neuroimaging using CTor MRI scanning confirms the diagnosis, location, and size of the hemorrhage. Hemorrhagic strokes tend to be fatal more frequently than ischemic strokes. Although recovery occurs in many patients, some are left with residual neurological deficits requiring rehabilitation and ongoing care. Treatment includes supportive management and pharmacological measures to reduce blood pressure, reverse bleeding tendencies, or treat elevated intracranial pressure, if these exist. The hemorrhage usually stops on its own and the blood is resorbed spontaneously. Surgical management is rare because of the dangers involved and its failure to reverse the progression of the disease, except for cerebellar hemorrhages, where surgical evacuation of the hematoma is lifesaving. Subarachnoid hemorrhage is commonly caused by rupture of vascular abnormalities such as cerebral aneurysms or arteriovenous malformations. There is usually a sudden severe headache, followed soon afterward by loss of consciousness. It can be life-threatening, with onethird to one-half of patients dying prior to reaching the hospital. In addition to the sudden headache, symptoms include eye pain, nausea, drowsiness, stiff neck, seizures, loss of consciousness, weakness, sensory loss, and aphasia. Complications can include hydrocephalus, vasospasm, or subsequent recurrent hemorrhage, all of which can cause additional neurological deficits. Diagnosis is made on neuroimaging, and pharmacological management is used to reduce intracranial pressure and pain. Surgical management is used to control or remove the vascular abnormality. Although complete recovery occurs in some

Cross References ▶ Cerebellar Hemorrhage ▶ Hematoma ▶ Hemorrhagic Stroke ▶ Intracranial Hemorrhage ▶ Intracerebral Hemorrhage ▶ Intraventricular Hemorrhage ▶ Lobar Hemorrhage ▶ Subarachnoid Hemorrhage

References and Readings Cornwell, E. E. (2004). Initial approach to trauma. In J. E. Tintinalli, G. D. Kelen, J. S. Stapczynski, O. J. Ma, & D. M. Cline (Eds.), Emergency medicine: a comprehensive study guide (6th ed., Chap. 251). New York: McGraw-Hill.

Hemorrhagic Stroke E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Brain hemorrhage; Cerebral hemorrhage

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patients, many experience residual cognitive and physical effects, requiring rehabilitation and ongoing care. Mortality tends to be higher in hemorrhagic stroke than in ischemic stroke. The 30-day mortality for hemorrhagic stroke is between 40% and 80%, with about onehalf of these deaths occurring in the first 48 h.

Cross References ▶ Cerebellar Hemorrhage ▶ Cerebral Amyloid Angiopathy ▶ Cerebral Angiitis ▶ Inraventricular Hemorrhage ▶ Intracranial Hemorrhage ▶ Intracerebral Hemorrhage ▶ Lobar Hemorrhage ▶ Subarachnoid Hemorrhage ▶ Thalamic Hemorrhage ▶ Vascular Malformation ▶ Vasculitis

References and Readings Broderick, J., Connolly, S., Feldmann, E., et al. (2007). Guidelines for the management of spontaneous intracerebral hemorrhage in adults: 2007 update: A guideline from the American Heart Association/ American Stroke Association Stroke Council, High Blood Pressure Research Council, and the Quality of Care and Outcomes in Research Interdisciplinary Working Group. Circulation, 116, e391–e413. Zia, E., Engstro¨m, G., Svensson, P. J., Norrving, B., & Pessah-Rasmussen, H. (2009). Three-year survival and stroke recurrence rates in patients with primary intracerebral hemorrhage. Stroke, 40, 3567–3573.

Heparin E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition Heparin is a non-oral anticoagulant chemical that is often used to prevent or treat thromboembolic disorders such as stroke, myocardial infarction, peripheral artery disease, venous thrombosis, pulmonary embolism, and others. Unlike the oral anticoagulant warfarin, heparin is administered intravenously or subcutaneously. It also is

distinguished from warfarin by its rapid onset of action, usually within minutes.

Current Knowledge The anticoagulant properties of heparin derive from its ability to bind to a naturally occurring anticoagulant chemical in the body known as antithrombin (AT), enhancing its activity. AT activity, in turn, inactivates thrombin, an important molecule that causes clotting, and another naturally occurring clotting factor. By inactivating thrombin, heparin prevents formation of fibrin and inhibits activation of platelets and other clotting factors that are normally induced by thrombin. Most adverse consequences of heparin derive from its anticoagulant effect, including bleeding in the brain, gastrointestinal tract, muscle, kidney, and elsewhere in the body. Easy bruising is common. It also can cause allergic reactions, osteoporosis with prolonged use, and heparininduced thrombocytopenia (‘‘HIT’’), which causes a severe reduction in the number of platelets, the blood clotting cells. The anticoagulant effects and the adverse effects of heparin increase at higher doses. Establishing and maintaining optimal dosing can be accomplished by monitoring the results of a simple blood test, called ‘‘partial thromboplastin time’’ or PTT. The ideal PTT range to provide an anticoagulant effect while minimizing bleeding risk has been established for many conditions. Because of the need for administration by injection, heparin often is not continued long term; oral warfarin administration is commonly substituted for heparin to provide anticoagulant effect in the long term. At low doses, repeated subcutaneous administration of heparin has been found to be effective in preventing venous thromboembolism and other thrombotic disorders, especially in conditions such as stroke, paralysis, postoperative states, prolonged hospitalization, and immobility. Naturally occurring heparin is comprised of several different types of molecules. When these are purified, fractionated, and isolated, the individual molecules are known as ‘‘low-molecular weight heparins’’ (LMW) of which several have been identified, marketed, and used to date; these include enoxaparin, tinzaparin, dalteparin, and others. Similar to the role of ‘‘unfractionated heparin,’’ LMW heparins can be used to prevent and treat clots. Although studies comparing standard unfractionated heparin and the newer LMW heparins are ongoing, most of the LMW heparins have been found to have the advantages of having generally fewer hemorrhagic complications, and in some cases, more effectiveness in

Herniation Syndromes

preventing the formation of new clots. LMW heparins also have easier once-daily administration by subcutaneous injection rather than by continuous intravenous infusion, reduced need for blood test monitoring, reduced risk of osteoporosis, and lower incidence of heparin-induced thrombocytopenia. These medications are more expensive than the common unfractionated heparin.

Cross References ▶ Anticoagulation ▶ Thrombosis ▶ Venous Thrombosis ▶ Warfarin

References and Readings Brieger, D. B., Mak, K. H., & Kottke-Marchant, E. J. (1998). Heparininduced thrombocytopenia. Journal of the American College of Cardiology, 31, 1449–1459. Hirsh, J., Anand, S. S., Halperin, J. L., & Fuster, V. (2001a). Guide to anticoagulant therapy: Heparin. A statement for healthcare professionals from the American Heart Association. Circulation, 103, 2994–3018. Hirsh, J., Anand, S. S., Halperin, J. L., & Fuster, V. (2001b). Mechanism of action and pharmacology of unfractionated heparin. Arteriosclerosis, Thrombosis, Vascular Biology, 21, 1094.

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Definition Heritability is the proportion of phenotypic variance among individuals that can be attributed to genetic variance in a population. For a given trait (or phenotype) such as eye color, a researcher might wish to know how much of the total variance in the population is due to genetic causes. This variance, heritability (or h2), is calculated by dividing genetic variance by phenotypic variance: h2 ¼ Vg=Vp Scientists now know that numerous behavioral and cognitive phenotypes have a considerable genetic component. These include intelligence, information processing speed, memory, attention, and problem-solving; language and reading impairments, as well as clinical disorders, such as Huntington’s disease, schizophrenia, dementia, affective disorders, attention deficit hyperactivity disorder, and autism.

Cross References ▶ Attention ▶ Intelligence ▶ Phenotype ▶ Processing Speed ▶ Reading

Hepatolenticular Degeneration ▶ Wilson’s Disease

Hereditary Multi-Infarct Dementia

Herniation Syndromes O LGA N OSKIN Columbia University New York, NY, USA

▶ Cadasil

Frequently Used Terms

Heritability J OHN D E LUCA Kessler Foundation Research Center West Orange, NJ, USA

Synonyms H2

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Subfalcian or cingulate herniation; Uncal herniation; Rostrocaudal or Central herniation

Short Description or Definition Because the intracranial compartments are generally noncompressible and the intracranial volume is essentially constant, any additional pressure-producing solid or liquid mass within the intracranial cavity will result in

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Herniation Syndromes

displacement of healthy tissues. Supratentorial expanding mass lesions, such as tumors, abscesses, ischemic or hemorrhagic strokes, or traumatic hematomas, can produce herniation by displacing adjacent and remote brain tissue, especially diencephalon. Such displacement may occur across the midline (e.g., subfalcian herniation) or via the rostro-caudal direction by compressing the deep diencephalic and midbrain structures (e.g., uncal and central herniation).

Etiology/Categorization Rostral-caudal/Central herniation: a nonlateralizing downward pressure, associated with hydrocephalus, diffuse cerebral edema and increased intracranial pressure (ICP), resulting in the diencephalon and parts of the temporal lobes being pushed through the tentorium cerebelli. Cingulate/Subfalcian herniation: occurs in cases of a lateral shift of the expanding cerebral hemisphere and displacement of the cingulated gyrus under the falx cerebri. This syndrome most commonly results in ipsilateral anterior cerebral artery (ACA) compression against the falx with resulting ACA territory infarction along with damage to the healthy hemisphere from compression. Uncal herniation: occurs from expanding lesions in the temporal lobe resulting in the uncus and the hippocampal gyrus being pushed against the edge of the tentorium cerebelli resulting in a direct compression of the midbrain. Upward transtentorial herniation: occurs when tissues in the posterior fossa expand, forcing the cerebellum and mesencephalon to be herniated through the tentorial notch. This results in hydrocephalus due to aquaductal or lateral ventricular obstruction or venous congestion due to compression of the veins of Galen and Rosenthal.

Clinical Course, Predictors/Risk Factors, and Outcomes Downward herniations can produce brainstem ischemia and hemorrhages, which are primarily arterial in origin. The stretching of the midbrain and pons in the downward direction results in stretch injury in the perforating branches of the basilar artery, resulting in ischemia and hemorrhages (also known as Duret hemorrhages) in the brainstem. Thus, clinical signs associated with herniation typically follow this downward progression of compression and ischemia in the brainstem. A progressive and irreversible sequence of respiratory, ocular, and motor

signs follow the order of diencephalon, midbrain, pons, and medulla. Compression of the aqueduct of Sylvius can result in functional blockade of the cerebrospinal fluid circulation, which may result in rapidly developing obstructive hydrocephalus and additional pressure necrosis in the parahippocampal gyrus. Kocher–Cushing sign of a rising blood pressure and slow pulse rate can occur with posterior fossa lesions. The first clinical signs of downward herniation present in the form of a ptotic, mydriatic pupil, and ipsilateral corticospinal involvement due to the pressure exerted on the contralateral midbrain peduncle (aka Kernihan’s notch) and on the third cranial nerve. Diminished consciousness may rapidly follow owing to the structural involvement of the nearby midline thalamic nuclei and the reticular activating system. The pupillary size, position, and reactivity are helpful in determining the location, extent, and clinical outcome of herniation syndromes. For instance, pupils that are nonreactive to light imply upper brainstem damage. Disconjugate ocular movements are seen when damage occurs to the medial longitudinal fasciculus and/or the cranial nerve six tracts in the pons. Both direct and indirect (also known as ‘‘false localizing signs’’) effects of ICP may result in other cranial nerve and parenchymal dysfunctions, such as ataxias, facial nerve palsies, skew deviation, lateral gaze palsy, ocular bobbing, and respiratory loss.

Evaluation A thorough and frequent neurological examination is in order for patients with suspected increased intracranial pressure, progressive worsening of their mental status, and sequential loss of function. If exam worsens, brain death may ensue and can be established by the rigid criteria to be individually evaluated by a trained physician over the course of 12 h. These include: (1) no evidence of hypothermia, (2) irreversible organ failure, (3) no behavioral correlate to noxious stimuli in the body, (4) no brainstem reflexes, such as pupillary light reflex, oculocephalic maneuver, cold calorics, gag, corneal reflex, or respiratory drive at hypercarbic levels. Additional criteria may include the absence of brain wave activity and lack of blood flow evidenced by invasive and noninvasive cerebrovascular imaging methods such as transcranial doppler evaluation and nuclear cerebral flow study.

Treatment Treatment of the underlying cause and prevention of irreversible damage to healthy tissues is imperative.

Herpes Simplex Encephalitis

Removal of expanding mass lesions may call for neurosurgical intervention with complete evacuation or a craniectomy where removing part of the skull will serve to de-pressurize contents within the skull, thus preserving healthy brain. In cases of eloquent brain tissue involvement or proximity, treatment of the associated edema is often preferred to radical surgical intervention. Thus, in select cases, steroids and osmotic treatments including mannitol as well as hypertonic saline infusion have been utilized. Deep sedation as well as external or intravenous cooling resulting in decreased metabolic demand and blood flow can help maintain low ICP. Direct ICP monitoring is now being employed in many neurological intensive care units. Hydrocephalus can be treated by placing an external or internal ventricular drain. While prognosis is typically poor once brainstem reflexes are lost, current treatments can now prevent impending herniation before its onset, thus reversing the clinical course.

References and Readings Plum, F., & Posner, J. B. (1982). The diagnosis of stupor and coma (3rd ed). Oxford: Oxford University Press. Suarez, J. I. (Ed.). (2004). Critical care neurology and neurosurgery. Totowa, NJ: Humana.

Herpes Encephalitis ▶ Herpes Simplex Encephalitis

Herpes Simplex Encephalitis B RUCE J. D IAMOND, J OSEPH E. M OSLEY William Paterson University Wayne, NJ, USA

Synonyms Herpes encephalitis; encephalitis

Meningoencephalitis;

Viral

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Short Description Herpes simplex encephalitis (HSE) is a direct infection of parenchymal brain tissue by either the herpes simplex 1 virus (HSV-1) or, less frequently, the herpes simplex 2 virus (HSV-2). Central nervous system (CNS) infection with HSV-1 or HSV-2 is most often characterized by inflammatory changes and tissue destruction in the temporal lobe. If HSV encephalitis is suspected, it is essential to begin antiviral therapy as soon as possible. When left untreated, HSV infections of the CNS are fatal.

Categorization

H The herpesviruses are DNA viruses that belong to the larger herpes viridae family (Halperin, 2007). The HSV1 and HSV-2 viruses are further subcategorized as Alphaherpesvirinae based on various characteristics that establish how they will manifest as disease in humans (Halperin, 2007). The Alphaherpesvirinae is known to cause vesicular orofacial or genital lesions and keratitis during primary and recurrent infection (Halperin, 2007). It is relatively rare that HSV-1 or HSV-2 will develop into a fulminant infection of the CNS (Boos & Esiri, 2003; Halperin, 2007). Although the Alphaherpesvirinae can cause encephalitis upon primary infection, most cases occur during reactivation (Boos & Esiri, 2003; Halperin, 2007). Herpesviruses tend to remain latent in host cells for extended periods without causing damage, only to cause symptoms that may differ from the primary infection during reactivation (Boos & Esiri, 2003; Halperin, 2007). The HSV-1 and HSV-2 viruses are closely related, sharing about 50% identical DNA (Boos & Esiri, 2003; Halperin, 2007). The major distinction between HSV-1 and HSV-2 is the route of entry into the host. HSV-1 enters the body through oral or ocular mucosa, while HSV-2 commonly enters through the genital mucosa (Halperin, 2007). Both viruses may also enter through skin and mucosal abrasions (Halperin, 2007). The primary infection at the site of exposed mucoepithelium may cause a vesicular rash but is most often asymptomatic (Halperin, 2007). Following primary infection, the virus gains entry to sensory neurons and travels via retrograde or anterograde transport to the trigeminal ganglion or dorsal root ganglia, where it may remain latent (Boos & Esiri, 2003; Halperin, 2007). The virus then spreads through axonal transport to the brainstem and eventually to the brain (Boos & Esiri, 2003; Halperin, 2007). The risk of latent HSV reactivating in the cranial or dorsal ganglia to cause

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encephalitis increases with age and degree of immunosuppression (Halperin, 2007).

Epidemiology The Alphaherpesvirinae is present in 40–80% of the population worldwide (Halperin, 2007). However, the virus is latent in most infected individuals, and therefore not all individuals will actively manifest symptoms. Socioeconomic status and age are modifiers of HSV prevalence (Halperin, 2007). HSV antibodies are present in about 20% of middle class children, while approximately 30% of lower socioeconomic status children have HSV infection (Halperin, 2007). By adolescence, 70–80% of lower socioeconomic individuals have been infected, compared to 40–60% in middle class populations (Halperin, 2007). HSV is the most common cause of acute sporadic encephalitis worldwide (Anderson, 2001; Halperin, 2007). The USA alone reports approximately 20,000 cases of encephalitis annually (Halperin, 2007). In cases of encephalitis where the etiologic pathogen has been identified, approximately one fifth or 1,200 cases are caused by HSV-1 or HSV-2 each year (Halperin, 2007). The estimated incidence is five cases per 1,000,000 individuals (Halperin, 2007). Among cases of HSV encephalitis, HSV-1 is the most common cause, accounting for 85–95% of cases with HSV-2 responsible for the remaining cases (Halperin, 2007).

Natural History, Prognostic Factors, Outcomes The first reports of HSV date back to ancient Greece, when Hippocrates termed the characteristic HSV skin lesions herpein, meaning to creep or crawl (Halperin, 2007). In modern times, there was considerable doubt and confusion within the medical community about the role of HSV in causing encephalitis (Boos & Esiri, 2003). It was not until around the middle of the twentieth century that doctors acknowledged HSV as a potential cause of severe central nervous system infection (Boos & Esiri, 2003). It is now established that HSV-1 is the most common cause of necrotizing encephalitis (Boos & Esiri, 2003). HSV encephalitis produces damage that is mainly confined to the limbic system at the level of the temporal lobes (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). The virus travels along known ipsilateral and contralateral pathways within the limbic system and its associated structures, causing bilateral temporal lobe lesions

(Boos & Esiri, 2003). Uncertainty exists concerning the pathogenesis of HSV encephalitis and the manner in which the virus reaches the limbic system (Anderson, 2001; Boos & Esiri, 2003). Although it has been hypothesized that the virus may reach the inferior frontal and temporal lobes by spreading centripetally from the trigeminal ganglion following reactivation, there is as yet no evidence to support this notion (Anderson, 2001; Boos & Esiri, 2003). Other explanations have focused on the olfactory tract as a route through which HSV may reach the infero-medial aspect of the temporal lobe (Anderson, 2001; Boos & Esiri, 2003). There is some evidence from electron microscopy and viral antigen studies to support the involvement of the olfactory pathway in HSV encephalitis (Anderson, 2001; Boos & Esiri, 2003). In fatal cases, the gross pathological changes that occur when brain has been infected with HSV represent a necrotizing encephalitis (Anderson, 2001; Boos & Esiri, 2003). The brain becomes swollen, and the frontal and temporal lobes show necrosis and liquefaction due to hemorrhaging (Anderson, 2001). At the cellular level, spreading virus causes destruction of neurons, oligodendrocytes, and astroglia (Anderson, 2001). Microscopic examination of tissue at necropsy shows capillary congestion and petechial hemorrhages on the brain surface, as well as cortical and subcortical white-matter inflammatory changes (Anderson, 2001). Abnormalities within neurons and glia such as eosinophilic intranuclear occlusion bodies or Lipschutz inclusion bodies are often apparent during the first week of the illness (Anderson, 2001). As with other causes of encephalitis, age and degree of altered consciousness emerge as the most important prognostic indicators (Anderson, 2001; Halperin, 2007). Patients who present with a Glasgow coma scale (GCS) of seven or higher and those who are less than 30 years old have the lowest morbidity and mortality rates (Halperin, 2007). Before the introduction of antiviral drug therapy, mortality rates for HSV encephalitis approached 70%. Taken together, these considerations demonstrate the importance of early detection and prompt treatment with appropriate medication before deterioration of consciousness. The course of the first two weeks is crucial, during which time stabilization and improvement often becomes evident, although recurrence is possible (Anderson, 2001). Survivors have variable periods of recovery that can continue beyond 6 months (Boos & Esiri, 2003). Although significant advances have been realized in diagnosing and treating herpes encephalitis with the introduction of polymerase chain reaction (PCR) testing


and antiviral drugs, patients often develop severe sequelae (Boos & Esiri, 2003). The most common sequelae among those who have had serious disease of the temporal lobes are epilepsy, personality and behavioral abnormalities, and memory impairments (Anderson, 2001; Boos & Esiri, 2003). However, even in patients who make relatively good functional recoveries, enduring neurologic symptoms, and subtle cognitive deficits are evident on neuropsychological testing (Anderson, 2001; Halperin, 2007).

Neuropsychological and Psychological Outcomes Neuropsychological Survivors of HSE can exhibit severe disability and dependency (due to problems in performing activities of daily living) along with personality change, motor deficits, aphasia, amnesia, global cognitive decline, and epilepsy (Hokkanen & Launes, 2007). Reports of mild forms of untreated HSE have been few, but in one report three patients with HSE achieved good recovery without antiviral therapy. Minor cognitive sequelae in these cases included partial loss of memory of events prior to illness (i.e., retrograde memory impairments) and impaired abstract reasoning as well as behavioral disinhibition. Explicit memory seems to be particularly vulnerable in HSE, while implicit memory (e.g., acquisition of habits, priming effects and conditioning) may remain intact (Hokkaunen & Launes, 2007). Anterograde memory impairments due to the effects of the virus in the temporal lobes have been the most consistent finding in HSE (Hokkaunen & Launes, 2007). More severe manifestations of HSE can result in profound memory impairments (i.e., amnesia) and increased rates of forgetting. Retrograde amnesia can affect any of the subsystems in remote memory (semantic or episodic) and impairments in the ability to recall names, faces, and episodes involving familiar or famous people have also been observed. Some HSE patients have relatively preserved or intact anterograde memory but have disproportionately impaired retrograde memory. While common in psychiatric literature, this pattern of impairment is rare in neurological patients and by 1999 there had been only around 30 such patients reported, with seven of them postencephalitic (Hokkaunen & Launes, 2007). Neither the memory mechanisms behind the phenomenon (storage vs. retrieval deficit), nor the pattern of structural lesions causing it are fully understood (Hokkaunen & Launes, 2007).

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Aphasia, which is often encountered in HSE, is commonly accompanied by an anomia that can involve impairments in visual identification and drawing or sorting pictures of tested items (Hokkaunen & Launes, 2007). Executive dysfunction can result in a variety of behavioral problems (e.g., impulsivity, apathy, lack of planning, perseveration, difficulty in making shifts in attention, rigidity, emotional flattening or lability) that are related to the location of tissue damage. Epilepsy is also a common sequel of encephalitis and seizures that are not always controlled by medication. A high seizure frequency may have a detrimental effect on cognitive functioning, with memory performance particularly vulnerable due to deterioration because of epilepsyrelated neuronal loss in the hippocampus. If this is the sole cause of deterioration, the problem may be alleviated with newer anti-seizure medications. Although most of the cognitive impairments either diminish or remain stable, some HSE patients deteriorate over the years, as seen on repeated testing (Hokkanen & Launes, 2007). Relatedly, there are case reports suggesting a relapsing HSE course due to viral reactivation. Although rare, this may present another explanation for continuing cognitive deterioration (Hokkaunen & Launes, 2007). In a longitudinal study of eight HSE patients, two had difficulty in activities of daily living and showed significant cognitive impairments at neuropsychological reevaluation (3.7 years post-onset) (Hokkanen & Launes, 2007). Overall, less widespread damage to the temporal lobes and fronto-basal areas produces less severe cognitive impairment (particularly memory disorders). Less severe cognitive impairment may also result in the disease going unrecognized and, therefore, undiagnosed. Variability in the extent and location of brain lesions also contributes to variable cognitive impairment profiles with some patients showing rapid forgetting, while others demonstrate ‘‘frontal type’’ retrieval difficulty and dysexecutive syndrome (Hokkanen & Launes, 2007).

Psychological After the introduction of acyclovir, personality and behavioral abnormalities have still been found in 40–60% of the HSE survivors, but the emotional symptoms appear milder. Common symptoms include panic, anxiety and affective disorders, manic behavior, aggressive outbursts, irritability, and depression. Psychiatric symptoms can be explained, in some cases, by an emotional reaction to the illness, but it is likely that damage to the limbic system and

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amygdalo-frontal pathways caused by encephalitis also plays an etiological role. Neuropsychiatric symptoms arising from limbic lobe dysfunction are well known and include emotional or mood disorders (e.g., rage, aggression, depression and mania), delusions, hallucinations, anxiety and dissociative disorders as well as sexuality changes and hyperoral behaviors (Hokkaunen & Launes, 2007).

Evaluation Nonspecific prodromal symptoms may last between 3 and 10 days, usually consisting of fever, lethargy, headache, irritability, and gastrointestinal symptoms (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). The prodromal phase gives way to a neurological stage in which there is progressive and severe deterioration that includes meningeal irritation, impaired consciousness, and epileptic seizures that may be focal or diffuse (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Signs and symptoms commonly localize to the temporal lobe and contiguous areas of the frontal or parietal lobes, with memory loss, personality change, hallucinations, dysphasia, hemiparesis, and parietal syndromes appearing as the disease progresses (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). As HSV spreads, intracranial pressure rises (Anderson, 2001) and if not reversed, the natural progression of HSV encephalitis leads to decerebration and death (Anderson, 2001; Halperin, 2007). There are no clinical features of HSV encephalitis that are pathognomonic (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). However, the appearance of an acute febrile illness with altered consciousness, seizures, and focal signs must immediately raise the suspicion of HSV encephalitis and be thoroughly investigated (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). Clinicians must be particularly attuned to the possibility of HSV encephalitis in elderly febrile patients, who often present with confusion, disorientation, and forgetfulness (Boos & Esiri, 2003). Blood studies are of no immediate value to the differential diagnosis, and serum antibody tests alone are insufficient to definitely diagnose HSV encephalitis (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). Brain imaging should be performed as soon as possible, preferably with MRI, to screen for mass effect and locate abnormalities (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos

& Tyler, 2008). The typical lesion seen on MRI appears as a T1 hypointensity or T2 hyperintensity in the inferomedial area(s) of the temporal lobe(s) that may extend into the external capsule and insula (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). If deemed safe, lumbar puncture should be undertaken to examine CSF (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Other infections can then be ruled out and HSE can be positively diagnosed by detecting HSV DNA using PCR (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Other CSF abnormalities include increased opening pressure, a lymphocytic pleocytosis, elevation of the protein concentration, and normal to mildly reduced glucose concentration (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). Viral cultures rarely isolate HSV in the CSF (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). While EEG has low specificity for diagnosing HSV encephalitis, it may be clinically useful in supporting the diagnosis and managing the patient through the course of the illness (Boos & Esiri, 2003). EEG changes include generalized or focal slowing, possibly with periodic lateralized epileptiform discharges (PLEDs). These findings are characteristic of many cerebral disease processes, however, and are therefore non-specific (Anderson, 2001; Boos & Esiri, 2003; Halperin, 2007). Neuropsychological assessment is likely to reveal impairments in even mild HSE, and should always be carried out when encephalitis is suspected (Hokkaunen & Launes, 2007).

Treatment Halperin (2007) recommends beginning therapy with acyclovir based on a presumptive diagnosis reached when a patient presents with fever, altered consciousness, and lymphocytic pleocytosis. Boos & Esiri (2003) recommend beginning drug therapy, as soon as emergency imaging, excludes other types of focal lesions, such as tumor, stroke or hemorrhage. As previously discussed, degree of altered consciousness is an important prognostic indicator, and thus, beginning antiviral therapy rapidly is crucial and should be undertaken before deterioration occurs (Anderson, 2001; Boos & Esiri, 2003; Griffin, 2000; Halperin, 2007; Nath & Berger, 2000; Roos & Tyler, 2008). The currently recommended therapy with acyclovir is 10–15 mg/kg i.v. for 14–21 days, along with standard patient management procedures for cases of viral encephalitis (Boos & Esiri, 2003; Halperin, 2007). Although

Heteromodal Cortex

steroids have been used successfully in the treatment of bacterial meningitis, there is insufficient evidence to conclude that they provide any significant benefits in cases of HSV encephalitis (Halperin, 2007). It is important to note that acyclovir-resistant strains of HSV have been isolated in a percentage of immunocompromised patients (Halperin, 2007). Generally, foscarnet dosed at 60 mg/kg every 8 h for 21 days has been effective (Halperin, 2007). For HSV infections resistant to acyclovir and foscarnet, cidofovir may be useful (Halperin, 2007). However, recent research has shown certain strains of HSV to be cidofovirresistant, and there is currently no recommended dose for treating HSV encephalitis (Halperin, 2007).

Cross References ▶ Brain Swelling ▶ Cerebral Edema ▶ Cerebral Perfusion Pressure ▶ Encephalitis (Viral) ▶ Epstein-Barr Virus ▶ Intracranial Pressure ▶ Lumbar Puncture ▶ Mass Effect ▶ Meningitis ▶ Prion Disease

References and Readings Anderson, M. (2001). Encephalitis and other brain infections. In M. Donaghy (Ed.), Brain diseases of the nervous system (pp. 1117– 1180). New York: Oxford University Press. Boos, J., & Esiri, M. M. (2003). Viral encephalitis in humans. Washington, DC: ASM Press. Centers for Disease Control. (Apr. 2008). 2007 West Nile virus activity in the United States (Reported to CDC as of April 1, 2008). Retrieved April 26, 2008 from http://www.cdc.gov/ncidod/dvbid/westnile/ surv&controlCaseCount07_detailed.htm Griffin, D. E. (2000). Encephalitis, myelitis, and neuritis. In G. L. Mandell, J. E. Bennett, & R. Dolin (Eds.), Principles and practice of infectious diseases (pp. 1143). Philadelphia, PA: Churchill Livingstone. Halperin, J. J. (Ed.). (2007). Encephalitis: Diagnosis and treatment. New York: Informa Healthcare. Hokkanen, L., & Launes, J. (2007). Neuropsychological sequelae of acuteonset sporadic viral encephalitis. Neuropsychological Rehabilitation, 17(4/5), 450–477. Nath, A., & Berger, J. R. (2000). Acute viral meningitis and encephalitis. In L. Goldman & J. C. Bennett (Eds.), Cecil textbook of medicine (pp. 2123–2126). Philadelphia, PA: W. B. Saunders. Roos, K. L., & Tyler, K. L. (2008). Meningitis, encephalitis, brain abscess and empyema. In B. Fauci, H. Kasper, J. Longo, & Loscalzo (Eds.), Harrison’s principles of internal medicine (17th ed., pp. 2621–2640). New York: McGraw Hill.

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Heschl’s Gyrus J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Synonyms Primary auditory cortex; Transverse gyri of Heschl

Definition That portion of the superior temporal gyrus that lies inside the banks of the lateral fissure (the temporal operculum). This area is also referred to as the transverse gyri of Heschl as the individual gyri are oriented in a lateral– medial plane within the operculum (as opposed to a more anterior–posterior or inferior–superior plane of most of the cortical gyri). This region is the primary cortical projection area for auditory information coming from the medial geniculates, the specific auditory relay nuclei of the thalamus. Because of the extensive bilateral projections that characterize the auditory system, unilateral lesions restricted to Heschl’s gyrus would be expected to produce only very subtle deficits and may be limited to increased difficulty with sound localization. While quite rare, bilateral lesions to this structure could result in deafness.


Heteromodal Association Cortex ▶ Heteromodal Cortex

Heteromodal Cortex K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Synonyms Heteromodal association cortex; Tertiary cortex

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Definition The heteromodal cortex refers to a region that receives input from multiple sensory or multimodal areas. Two major areas of heteromodal cortex are generally identified. Posteriorly, there is the PTO cortex (the confluence of the parietal, temporal, and occipital lobes), as well as certain occipitotemporal and occipitoparietal areas. Essentially, these are areas that receive input from multiple unimodal sensory association areas and/or other heteromodal cortices. Anteriorly, the prefrontal cortices are generally considered multimodal or heteromodal in that they also receive input from multiple sensory or other polymodal cortices. Because of their multimodal inputs, these cortical areas are considered to be responsible for more complex or integrated cognitive activities. For example, when we think of an apple, we imagine not only how it looks and tastes, but also its smell, its texture, its weight, and even how it sounds when we to bite into it. This ‘‘multimodal sensory image’’ is thought to be the result of the integration that takes place in the posterior heteromodal cortex. Similarly, the prefrontal association cortices utilize multiple sensory, limbic, and motor areas in the course of its supraordinate planning, organization, and ongoing monitoring of behavioral responses to the environment. Lesions in the PTO may result in impaired recognition of faces, objects, or voices. Anterior heteromodal lesions are more likely to result in a ‘‘frontal lobe syndrome.’’ This syndrome is typified by deficient attention and executive functions, along with behavioral changes. The behavioral abnormalities often follow one of two paths: apathy, with attendant loss of initiative and emotional blunting, or disinhibition, marked by impulsivity and poor judgment and insight.

References and Readings Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach (pp. 313–314, 387–390, 393–395, 403–405, 409–455). New York: Springer. Mesulam, M. (2000). Large-scale networks, association cortex, frontal syndromes, the limbic system, and hemispheric specialization. In M. Mesulam (Ed.), Principles of behavioral neurology (Chap. 1, pp. 1–120). Philadelphia: F. A. Davis. Mesulam, M. M. (2000). Principles of behavioral and cognitive neurology (2nd ed., pp. 34–36, 41–48). New York: Oxford.

Heteroreceptor ▶ Autoreceptor ▶ Receptor Spectrum

Heterotopia A MY M UGG , A MIT M ALHOTRA Kaiser Permanente Medical Center Oakland, CA, USA

Synonyms Cortical malformation

Definition Heterotopia is a collection of disorganized grey matter resulting from abnormal neuronal migration from the deep proliferative areas to the developing cortical structures at the brain surface. They may be found anywhere from the subependyma to the cortical mantle and have been associated with abnormalities in the overlying cortex. They are stable in size in proportion to brain volume; as brain volume increases, often there is a relatively proportional increase in size, though this is not universal. If they grow markedly out of proportion, concern arises that they signify oligodendrogliomas, DNETs or other slow growing tumors. The genetics of the syndromes that have heterotopias as a component range in inheritance from sporadic, X-linked, autosomal dominant, and autosomal recessive. Patients show a wide spectrum of manifestations from clinically asymptomatic to intractable epilepsy and mental retardation. Interestingly, heterotopia may have varying degrees of functionality with studies suggesting normal glucose utilization, functional hyperemia, and activation of the overriding cortex.

References and Readings Barkovick, A. J., & Kuzniecky, R. I. (2000). Grey matter heterotopia. Neurology, 55, 1603–1608.

High-Level Mobility Assessment Test Chang, B. S., & Walsh, C. A. (2003). Mapping form and function in the human brain: The emerging field of functional neuroimaging in cortical malformations. Epilepsy & Behavior, 36, 618–625. Golden, J. (2001). Cell migration and cerebral cortical development. Neuropathology and Applied Neurobiology, 27, 22–28. Kobayashi, E., Bagshaw, A. P., Grova, C., Gotman, J., Dubeau, F. (2006). Grey matter heterotopia: What EEG-fMRI can tell us about epileptogenicity of neuronal migration disorders. Brain, 129, 366–374.

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Heterotypic Cortex ▶ Idiotypic Cortex

Heterotypic Visual Cortex ▶ Extrastriate

Heterotopic Ossification B ETH R USH Mayo Clinic Jacksonville, FL, USA

Synonyms HO

HFD ▶ Human Figure Drawing Tests

Hidden Depression ▶ Masked Depression

Definition Heterotopic ossification (OH) is a formation of lamellar bone in soft tissues following brain or spinal injury. HO usually involves the large joints of the body such as the hip, elbow, shoulders, and knees. In cases of traumatic brain injury, one of the most common sites is the hip. In cases of spinal injury, HO always occurs below the level of injury. Research has not clearly identified the causes of HO. Some have suggested that connective tissue cells change their characteristics into bone-forming cells as some type of inflammatory reaction to injury. Individuals with other bone-forming disorders such as ankylosing spondylitis, Paget disease, and idiopathic skeletal hyperostosis are at the risk for HO following traumatic brain or spinal cord injury. HO resultant from brain injury may not remit and can create considerable pain and disability for a brain injury patient. Initially, pain and swelling are present, which typically subside within the first few weeks following the onset. In some cases, a hard, ossified lesion arises 6–12 weeks following injury onset. In these cases, pain symptoms and swelling persist. In any case, the pain and swelling resulting from HO limits the patients functionally and limits potential gains from rehabilitation.

Hidden Figures Test ▶ Embedded Figures Test

High Blood Pressure ▶ Hypertension

High-Level Mobility Assessment Test G AVIN W ILLIAMS Epworth Hospital Melbourne, Vic, Australia

Synonyms HiMAT

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High-Level Mobility Assessment Test

Description The High-level Mobility Assessment Tool (HiMAT) is a unidimensional measure of mobility. It comprises 10 items that are recorded by stopwatch or measure-tape. Patients are allowed a practice trial for each item. They are instructed to perform each item at their maximum safe speed, except for the stair items, where they are instructed to complete the task at ‘‘their normal speed.’’ Performances are then classified by performance quartiles outlined on a scorecard. The test items are: 1. 2. 3. 4. 5. 6. 7. 8a. 8b. 9a. 9b. 10a. 10b.

Walking Walking backward Walking on toes Walking over an obstacle (house-brick) Running Skipping Hopping forward on the more affected leg Bound (onto the more affected leg) Bound (onto the less affected leg) Up stairs – dependent Up stairs – independent Down stairs – dependent Down stairs – independent

Most items are scored from 0 to 4. A score of 0 indicates the inability to perform the item, while scores of 1–4 represent improving ability on each of the items. Performance on the stair items is only required once; ascending and descending stairs are scored separately. Items 9a and 10a are scored ‘‘5,’’ if the patient is able to complete the task independently (reciprocal gait pattern without the use of a rail). They are then rated on the corresponding independent items (9b and 10b). Subjects who require the use of a rail or are unable to perform reciprocally are categorized as ‘‘dependent.’’ The bounding item is scored separately for each leg. The HiMAT has been designed so that it can be used in most clinical settings, it is minimally dependent on equipment, and it is quick to administer. Most tests are performed on a 20-m walkway (performance over the middle 10 m is recorded) or a flight of stairs (14). Equipment required includes a stopwatch, house-brick, and measure-tape. The HiMAT takes approximately 10 min to administer. Normative values for 18 to 25-year-old males and females have been developed.

populations (Williams et al., 2004). These scales typically measure mobility at the ‘‘inpatient’’ phase of rehabilitation and include items such as bed mobility, transfers, and walking. Some scales include a step or stair item. These scales are susceptible to a ceiling effect, when used to measure mobility in a young traumatic brain injury (TBI) population, where goals may require high-level mobility skills, such as running and jumping. The HiMAT was specifically designed to measure thehigh-level mobility requirements of young people with traumatic brain injury (TBI) returning to social, leisure, sporting, and employment roles. It was initially developed in the TBI population, but has more recently been validated in a diverse adult neurological rehabilitation population. Investigation is also underway into the validity of the HiMAT in the pediatric TBI population. The initial development of the HiMAT sought to achieve several goals. Primarily, it was designed to quantify high-level mobility to a greater extent than the existing scales. Also important was the clinical utility in that the HiMAT had to be quickly and easily to use in almost any clinical environment with minimal dependence on equipment. Items developed for inclusion in the HiMAT had to be simple and easily understood, so there would be a minimal impact on performance for people with significant cognitive impairment following TBI (Williams et al., 2005a).

Psychometric Data The HiMAT was developed in a population of 103 people with TBI. The validity and unidimensionality of the HiMAT was established using Rasch analysis (Williams et al., 2005b). Concurrent validity was established with the motor FIM (r = 0.53, p < 0.01) and gross function Rivermead Mobility Assessment (r = 0.87, p < 0.01) (Williams et al., 2006b). It had high internal consistency (0.97), high inter-rater (0.99), and retest (0.99) reliability (Williams et al., 2006a). It was also responsive; with minimal detectable change (MDC) scores 3 points, indicating improvement >3 points over a 3 month period is clinically significant (Williams et al., 2006b). It was found to be more responsive to change and less susceptible to a ceiling effect when compared to the gross function Rivermead Mobility Assessment.


Clinical Uses

The majority of mobility scales used in adult neurological rehabilitation have been developed in elderly or stroke

The HiMAT was developed for people who are independently ambulant yet still experiencing mobility

Hindbrain

limitations. It is therefore most useful in the outpatient phase or later stages of rehabilitation when clients may have aspirations to return to physically demanding employment roles, or social, leisure, and sporting activities. It is less susceptible to a ceiling effect, and more responsive to clinically meaningful change in mobility than other existing scales. The HiMAT was originally developed for the young survivors of traumatic brain injury who may have some high-level goals. It has since been validated in the wider neurological rehabilitation population, so is applicable for most adults with neurological conditions. Normative values have also been established for males and females to give patients an idea of normal age and sex mobility performances. Although the HiMAT is simple and quick to administer, it does require access to a 20 m walkway and a flight of stairs. Stair time conversion is valid (if no access is available to a flight of 14 steps) providing there are more than six steps. The unidimensional nature of the HiMAT means there are no dual-task items, so it can be used for people with very severe cognitive impairment.

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High-Potency/Low-Potency Groups of Antipsychotics ▶ Antipsychotics

HII ▶ Halstead Impairment Index

H HiMAT ▶ High-Level Mobility Assessment Test

Hindbrain Cross References ▶ Rivermead Mobility Index

R ANDALL E. M ERCHANT Virginia Commonwealth University Medical Center Richmond, VA, USA


Definition

Williams, G., Greenwood, K., Robertson, V., Goldie, P., & Morris, M. E. (2006a). High-level Mobility Assessment Tool (HiMAT): Inter-rater Reliability, Retest Reliability and Internal Consistency. Physical Therapy, 86, 395–400. Williams, G., Greenwood, K., Robertson, V., Goldie, P., & Morris, M. E. (2006b). The concurrent validity and responsiveness of the Highlevel Mobility Assessment Tool (HiMAT) for measuring the mobility limitations of people with traumatic brain injury. Archives of Physical Medicine and Rehabilitation, 87, 437–442. Williams, G., Robertson, V., & Greenwood, K. (2004). Measuring highlevel mobility after traumatic brain injury. American Journal of Physical Medicine and Rehabilitation, 83, 910–920. Williams, G., Robertson, V., Greenwood, K., Goldie, P., & Morris, M. E. (2005a). The High-level Mobility Assessment Tool (HiMAT) for traumatic brain injury. Part 1: Item Generation. Brain Injury, 19(11), 925–932. Williams, G., Robertson, V., Greenwood, K., Goldie, P., & Morris, M. E. (2005b). The High-level Mobility Assessment Tool (HiMAT) for traumatic brain injury. Part 2: Content Validity and Discriminability. Brain Injury, 19(10), 833–843. http://www.tbims. org/combi/

The hindbrain is the portion of the brain that connects the brain with spinal cord.

Current Knowledge The hindbrain is the caudal most of the three primary portions of the brain, connecting the brain with spinal cord. The hindbrain consists of the cerebellum, medulla oblongata, and the pons. These areas of the brain coordinate muscle movements and equilibrium as well as control some of the most basic essential physiological processes such as breathing and blood circulation. Since cranial nerves V through VIII arise from the pons and cranial nerves IX through XII arise from the medulla, injuries or tumors of the hindbrain can adversely affect the functions of these nerves.

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Cross References ▶ Cerebellum ▶ Medulla ▶ Pons

Hippocampus

Hindsight Bias ▶ Bias

Hippocampal Formation ▶ Hippocampus

Hippocampal System ▶ Medial Temporal Lobe

Hippocampus A MANDA WAXMAN City University of New York New York, NY, USA

Synonyms Hippocampal formation

Definition The hippocampus is a C-shaped, three-layered, subcortical structure, located within the medial temporal lobes, adjacent to the amygdala (Fig. 1). This phylogenetically ancient structure is divided into three continuous sections, referred to as cornu Ammonis [CA], one through three (Fig. 2). The hippocampus proper is an anatomical subdivision of the hippocampal formation, which also encompasses the dentate gyrus and the subiculum (Fig. 3). Here, the term ‘‘hippocampus’’ will be utilized to refer to all three components of the hippocampal

Hippocampus. Figure 1 The hippocampal formation in a sagittal magnetic resonance imaging. (Reproduced from Mendoza & Foundas, 2008)

formation. Possessing a unique neuroanatomical, neurochemical, and electrophysiological organization, the hippocampus serves as a component of the limbic system as well as primarily functions in the formation of new memories and in spatial navigation.

Historical Background Around 1564, an anatomist named Giulio Cesare Aranzi first utilized the term hippocampus to describe the limbic system component because of its visual resemblance to a sea horse. Toward the end of the nineteenth century, Theodor Meynert, Camillo Golgi, and Santiago Ramon y Cajal significantly contributed to identifying the structural anatomy of the hippocampus. Then, around 1900, Vladimir Bekhterev first reported the role of the hippocampus in memory acquisition. However, most scientists viewed the hippocampus as involved in emotion until the emergence of the patient H. M. (Scoville & Milner, 1957). Following the bilateral resection of the medial temporal lobes (including the hippocampus and parahippocampal gyri) in order to relieve epileptic seizures, H. M. suffered from numerous anterograde and temporally graded retrograde memory impairments. Experimental inquiry determined that he was unable to learn new facts or recall new experiences but retained his ability to learn procedural tasks. In other words, he suffered from a loss of declarative memory while his nondeclarative memory remained intact. Furthermore, his temporally graded retrograde amnesia destroyed his memory of events within several years of the surgery; however, his memory of remote events from childhood and up to several years before the surgery was undisturbed. The memory deficits of H. M. prompted medical

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Hippocampus. Figure 2 Cross section of the hippocampal formation and parahippocampal gyrus. (Reproduced from Mendoza & Foundas, 2008)

Amygdala

Inferior horn of lateral ventricle

Hippocampal formation Parahippocampal gyrus

Hippocampus. Figure 3 The hippocampal formation on an axial cut at the level of the midbrain. (Reproduced from Mendoza & Foundas, 2008)

professionals to further investigate the role of the hippocampus in memory. Numerous cases of bilateral medial temporal lobe resections, such as M. B., D. C., A. Z., and M. R., demonstrated a positive correlation between the extent of destruction to the hippocampal complex and the degree of memory impairment (Scoville & Milner, 1957). Additionally, Wilder Penfield, a Canadian

neurosurgeon, started to perform unilateral partial temporal resections for patients with localized injury-causing seizures. Penfield and his colleague Brenda Milner (1958) determined that these operations only produced moderate memory impairments. The cases that emerged in the late 1950s and 1960s provoked the formulation of disparate views regarding the role of the hippocampus in 1978. John Horel (1978) implicated other medial temporal structures, such as the temporal stem, amygdala, and temporal neocortex, as essential to memory. Contrarily, John O’Keefe and Lynn Nadel (1978) proposed that the hippocampus provides a spatial map. Hippocampal pyramidal cells, previously identified as place cells, have spatial firing fields and can be sensitive to location, direction of travel, and/or head orientation. They believed that the hippocampus forms a mental representation of the spatial layout of the environment and therefore, plays a vital role in spatial navigation. However, these findings in experimental animals did not entirely correspond with the findings from human memory and amnesia. Lastly, Mortimer Mishkin (1978) suggested that the experimental paradigms for testing memory in animals had not been adequate to demonstrate the amnesia that resulted from medial temporal resections in humans. Therefore, he demonstrated that monkeys with bilateral medial temporal lobe lesions showed deficits in explicit memory for places and objects similar to those observed in H. M.

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In 1982, Zola-Morgan, Squire, and Mishkin (1982) developed an animal model of human amnesia in the nonhuman primate. With this model and about 10 years of experimental work, Squire and Zola-Morgan (1991) identified the anatomical components of the medial temporal-lobe memory system. The important structures include the hippocampus proper, dentate gyrus, subiculum, entorhinal cortex, perirhinal cortex, and parahippocampal cortex. Lesions restricted to any major component of this system produce these significant deficits in declarative memory, as demonstrated by the case of R. B. (ZolaMorgan, Squire, & Amaral, 1986).

Current Knowledge Declarative Memory The hippocampus is selectively involved in the process of encoding new short-term memories about facts and experiences into long-term memories. As epitomized by H. M., damage to the hippocampus produces a limited form of global anterograde amnesia, a memory deficit characterized by an inability to make lasting memories about new facts, events, and experiences. This form of memory, the factual knowledge of people, places, and things and the meaning of these facts, is defined as declarative, or explicit, memory. Declarative memory is contrasted with nondeclarative memory, which encompasses new skills or procedures, priming, and simple forms of conditioning. These amnesics show relatively unimpaired nondeclarative memory. Endel Tulving (1972) further classified declarative memory into episodic memory, a memory for personal events and experiences, and semantic memory, knowledge for objects, facts, and concepts. The hippocampus appears to play an essential role in encoding new episodic and semantic memories into long-term memories. Additionally, H. M. as well as patients described by Penfield and Milner (1958) possessed intact memory for remote events that occurred years before the surgery. This temporally graded retrograde amnesia provides one of the main sources of evidence in support of the idea of memory consolidation. According to the medial temporal-lobe memory system, the hippocampus is viewed as a temporary memory system, utilized until long-term consolidation is complete and a permanent memory is established in the neocortex (Alvarez & Squire, 1994; Squire & Zola-Morgan, 1991). Therefore, since the medial temporal-lobe memory system processes declarative memory, both episodic and semantic memory

should be equally affected by temporally graded retrograde amnesia following medial temporal lobe lesions. However, recent studies provide evidence that episodic and semantic memory can be separately disturbed (Nadel & Moscovitch, 1997). Individuals with retrograde amnesia demonstrate less severe deficits with personal semantic memory than in autobiographical episodic memory. Additionally, general semantic memory, which includes conceptual knowledge of words, grammar, and objects, is mostly preserved in retrograde amnesia. Certain theories, such as the multiple trace theory, have been generated to explain these dissociations in retrograde amnesia between different types of declarative memory (Nadel & Moscovitch, 1997), but further investigations are necessary. Lastly, human and animal lesion studies continue to elucidate the exact functional roles of each hippocampal structure and to know how the local circuitry of the hippocampus supports declarative memory.

Spatial Memory The role of the hippocampus in spatial memory was pioneered independently by two studies. First, in 1971, John O’Keefe and John Dostrovsky discovered that hippocampal pyramidal cells can encode information in space (O’Keefe & Dostrovsky, 1971). Individual place cells fire at particular locations within an environment, creating a cognitive map of the spatial environment. In 1973, Tim Bliss and Terje Lømo discovered that a brief high-frequency period of electrical stimulation (called a tetanus) applied artificially to a hippocampal pathway produced a stable increase in synaptic efficacy lasting from hours to weeks. This increase in synaptic strength is called long-term potentiation (LTP). LTP may serve as the hippocampal-dependent memory storage mechanism. LTP has been found to occur within each of the three principal pathways of the hippocampus: the perforant pathway, which projects from the entorhinal cortex to the granule cells of the dentate gyrus; the mossy fiber pathway, which consists of the axons of the granule cells and travels to the pyramidal cells in the CA3 region; and the Schaffer collateral pathway, which consists of the pyramidal cells in the CA3 region and ends on the pyramidal cells of the CA1 region (Kandel, 2000). This enhanced stimulation can be homosynaptic (caused by repetitive firing of one input), heterosynaptic (occurring when two particular inputs to a single neuron fire coincidentally), or hebbian (occurring when stimulation of neuronal afferent occurs when a neuronal cell body is

Hippocampus

coincidentally depolarized). Therefore, in the hippocampus, LTP serves as a biological substrate for maintaining a coherent spatial map over time. More broadly and requiring further investigation, LTP could plausibly serve as the biological basis for all memory.

Place Cells and Declarative Memory Extensive work has been devoted to determining a connection between the two primary functions of the hippocampus: generating cognitive maps and mediating declarative memory processes. By synthesizing the human lesion literature, the experimental lesion literature, and hippocampal electrophysiology, Cohen and Eichenbaum (1993) theorize that declarative memory and cognitive mapping share a fundamental basis in relational representation. This view hypothesizes that the hippocampus mediates the processing and storage of memories in a multidimensional network, in which items are organized according to the relevant relationships among them. These relational representations not only support the comparison and contrast of items but also enable the expression of memories in new situations. With declarative memory extremely dependent on these relational representations, various studies, utilizing different behavioral paradigms, were conducted to assess the importance of the demand for relational representations in cognitive mapping in animals (Eichenbaum, Fagan, Mathews, & Cohen, 1988; Eichenbaum, Mathews, & Cohen, 1989; Eichenbaum, Stewart, & Morris, 1990). Through application of this extensive work, Eichenbaum and Cohen demonstrate how relational representations and representational flexibility form a connection between declarative memory impairments in human amnesia and cognitive mapping deficits in animals. Furthermore, with animal models, numerous studies have demonstrated that hippocampal place cells are involved in processing the contextual information, beyond the spatial geometry of the environment, present in any learning situation (Smith & Mizumori, 2006). For example, Wood and colleagues (2000) determined that the firing patterns of rat hippocampal place cells encode information related to each episode, when they performed a multi-trial spatial alternation task on a T-maze. Since contextual information is an essential component of any episodic memory, place cells seem to contribute to the formation of episodic memories. Thus, the link between hippocampal place cells and declarative memory, through the identification of cognitive and neural mechanisms, continues to be explored.

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Aging and Alzheimer’s Disease The relationship between prolonged stress and memory impairments in the aging population has received significant attention. Recent studies have demonstrated that aged humans with significant prolonged cortisol elevations show reduced hippocampal volume and deficits in hippocampus-dependent memory tasks compared to normal-cortisol controls (Lupien et al., 1998). The degree of hippocampal atrophy correlates significantly with both the degree of cortisol elevation over time as well as the current basal cortisol levels. Therefore, this research reveals that prolonged stress produces sustained high levels of cortisol, which in turn, cause reductions in hippocampal volume. As a result, this hippocampal atrophy leads to memory impairments. Additionally, Alzheimer’s disease (AD), the most common dementia, includes striking deficits in new learning and memory. Magnetic resonance scanning studies demonstrate atrophy in the hippocampal regions at the time of Alzheimer’s disease (AD) diagnosis (Jack et al., 1997). A strong correlation between the amount of hippocampal atrophy and the degree of memory impairment has also been shown (Peterson et al., 2000).

Mesial Temporal-Lobe Epilepsy Mesial temporal-lobe epilepsy (MTLE), the most prevalent of the partial epileptic syndromes, arises in the hippocampus, amygdala, and adjacent parahippocampal cortex, and is characterized by recurrent complex partial seizures (Chang & Lowenstein, 2003). The most common pathological feature in patients with mesial temporal-lobe epilepsy (MTLE) is hippocampal sclerosis. This lesion is characterized by a ragged granule cell layer, a thin pyramidal cell layer from profound loss of CA1 and CA3 pyramidal cells, and changes in thickness due to profound loss of neurons in dentate hilus. A dense gliosis also accompanies this loss of neurons and causes shrinkage and hardening of tissue. Clinical features of MTLE include auras associated with gustatory or auditory sensations, loss of consciousness, postictal memory deficits, postictal aphasia, and automatisms (Vinken, Meinardi, & Bruyn, 2000). Resection of medial temporal lobe structures in refractory MTLE patients is extremely effective and renders approximately 80% of patients seizure-free.

Cross References ▶ Amnestic Disorder/Syndrome ▶ Declarative Memory

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▶ Episodic Memory ▶ Global Amnesia ▶ H.M.; Also the Case of H.M., Molaison, Henry (1926–2008) ▶ Memory ▶ Milner, Brenda Atkinson (1918– ) ▶ Mishkin, Mortimer (1926– ) ▶ Penfield, Wilder (1891–1976)

References and Readings Alvarez, P., & Squire, L. R. (1994). Memory consolidation and the medial temporal lobe: A simple network model. Proceedings of the National Academy of Science USA, 91, 7041–7045. Bliss, T. V., & Lomo, T. (1973). Long-lasting potentiation of synaptic transmission in the dentate area of the anaesthetized rabbit following stimulation of the perforant path. Journal of Physiology, 232, 331–356. Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sunderland, MA: Sinauer Associates. Chang, B. S., & Lowenstein, D. H. (2003). Mechanisms of disease: Epilepsy. The New England Journal of Medicine, 349, 1257–1266. Cohen, N. J., & Squire, L. R. (1980). Preserved learning and retention of pattern analyzing skill in amnesia: Dissociation of knowing how and knowing that. Science, 210, 207–209. Cohen, N. J., & Eichenbaum, H. (1993). Memory, amnesia, and the hippocampal system. Boston: MIT Press. Eichenbaum, H., Fagan, A., Mathews, P., & Cohen, N. J. (1988). Hippocampal system dysfunction and odor discrimination learning in rats: Impairment or facilitation depending on representational demands. Behavioral Neuroscience, 102, 331–339. Eichenbaum, H., Mathews, P., & Cohen, N. J. (1989). Further studies of hippocampal representation during odor discrimination learning. Behavioral Neuroscience, 103, 1207–1216. Eichenbaum, H., Stewart, C., & Morris, R. G. (1990). Hippocampal representation in place learning. Journal of Neuroscience, 10, 3531–3542. Gold, J. J., & Squire, L. R. (2006). The anatomy of amnesia: Neurohistological analysis of three new cases. Learning and Memory, 13, 699–710. Horel, J. A. (1978). The neuroanatomy of amnesia. A critique of the hippocampal memory hypothesis. Brain, 101, 403–445. Jack, C. R., Jr., Peterson, R. C., Xu, Y. C., Waring, S. C., O’Brien, P. C., Tangalos, E. G., et al. (1997). Medial temporal atrophy on MRI in normal aging and very mild Alzheimer’s disease. Neurology, 49, 786–794. Kandel, E. R. (2000). Cellular mechanisms of learning and the biological basis of individuality. In E. R. Kandel, J. H. Schwartz, & T. M. Jessell (Eds.), Principles of Neural Science (pp. 1247–1277). New York: McGraw-Hill. Lupien, S. J., de Leon, M., de Santi, S., Convit, A., Tarshish, C., Nair, N. P., et al. (1998). Cortisol levels during human aging predict hippocampal atrophy and memory deficits. Nature Neuroscience, 1, 69–73. Mendoza, J., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach. New York: Springer. Mishkin, M. (1978). Memory in monkeys severely impaired by combined but not by separate removal of amygdala and hippocampus. Nature, 273, 297–298.

Nadel, L., & Moscovitch, M. (1997). Memory consolidation, retrograde amnesia and the hippocampal cortex. Current Opinion in Neurology, 7, 217–227. O’Keefe, J., & Dostrovsky, J. (1971). The hippocampus as a spatial map. Preliminary evidence from unit activity in the freely moving rat. Brain Research, 34, 171–175. O’Keefe, J., & Nadel, L. (1978). The hippocampus as a cognitive map. New York: Oxford University Press. Penfield, W., & Milner, B. (1958). Memory deficits produced by bilateral lesions in the hippocampal zone. Archives of Neurological Psychiatry, 79, 145–154. Peterson, R. C., Jack, C. R., Jr., Xu, Y. C., Waring, S. C., O’Brien, P. C., Smith, G. E., et al. (2000). Memory and MRI-based hippocampal volumes in aging and AD. Neurology, 54, 581–587. Scoville, W., & Milner, B. (1957). Loss of recent memory after bilateral hippocampal lesions. Journal of Neurology, Neurosurgery, and Psychiatry, 20, 11–21. Smith, D. M., & Mizumori, S. J. Y. (2006). Hippocampal place cells, context, and episodic memory. Hippocampus, 16, 716–729. Squire, L. R., & Zola-Morgan, S. (1991). The medial temporal lobe memory system. Science, 253, 1380–1386. Tulving, E. (1972). Episodic and semantic memory. In E. Tulving & W. Donaldson (Eds.), Organization of memory (pp. 381–403). New York: Academic Press. Vinken, P. J., Meinardi, H., & Bruyn, G. W. (2000). Handbook of clinical neurology: The epilepsies. Amsterdam: Elsevier Science B. V. Wood, E. R., Dudchenko, P. A., Robitsek, R. J., & Eichenbaum, H. (2000). Hippocampal neurons encode information about different types of memory episodes occurring in the same location. Neuron, 27, 623–633. Zola-Morgan, S., Squire, L. R., & Amaral, D. G. (1986). Human amnesia and the medial temporal region: Enduring memory impairment following a bilateral lesion limited to field CA1 of the hippocampus. Journal of Neuroscience, 6, 2950–2967. Zola-Morgan, S., Squire, L. R., & Mishkin, M. (1982). Neuroanatomy of amnesia: Amygdala-hippocampus versus temporal stem. Science, 218, 1337–1339.

Hiscock Forced-Choice Test M ERRILL H ISCOCK University of Houston Houston, TX, USA

Synonyms Digit Memory Test; Hiscock Malingering Test

Description The Hiscock Forced-Choice Test (hereafter referred to as the Hiscock test) was described in a case report (Hiscock & Hiscock, 1989). Although the authors subsequently


referred to the test as the Digit Memory Test (DMT), it is commonly known as the Hiscock malingering test or simply the Hiscock test. The test has never been distributed as a commercial product, but the senior author has responded to requests for information about the test by sending instructions for constructing the stimuli, administering the test, and interpreting the results. The Hiscock test comprises 72 stimulus cards, each of which contains a five-digit target. The target, which varies randomly from one card to the next, is presented for 5 s. After a variable delay, the patient is shown a response card containing two five-digit numbers, one of which is identical to the previous target. The other five-digit number on the response card (the foil) always differs from the target with respect to both first and last digits. Representative cards are shown in Fig. 1. The test is divided into three blocks of 24 trials. The blocks differ in the length of the delay between presentation of the target and presentation of the response card: the intervals are 5, 10, and 15 s, respectively. Half of the correct responses are located on the left side of the response card and half are located on the right side. Patients respond by pointing to the correct number on the response card. Correct responses are summed to obtain a score for each block of 24 trials, and a total score for the 72 trials. Prior to the first trial, the patient is told only that the test is a memory test. The original procedures require

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telling the patient whether he or she was correct or not following each response. However, Prigatano, Smason, Lamb, and Bortz (1997) subsequently suggested that the sensitivity of the test for detecting malingering might be enhanced if no feedback were given. After completing the first and second blocks of trials, patients are told that they performed well and that, accordingly, the retention interval will be increased to make the next block of trials more difficult. A simple nonparametric test called the binomial test can be used to determine whether a patient’s total score, as well as the score for each block of 24 trials, falls below the chance range. In addition, it may be informative to determine whether performance decreases across the three blocks of trials (e.g., when chance-level performance on the first block drops to below-chance levels on subsequent trial blocks).

Historical Background The Hiscock test rests on the two-alternative forced-choice principle. Although the origin of this principle is uncertain, similar detection strategies had been applied to a case of hysterical blindness more than 2 decades prior to the publication of the Hiscock and Hiscock paper (Brady & Lind, 1961; Grosz & Zimmerman, 1965). Pankratz and his associates (Binder & Pankratz, 1987; Pankratz, 1979, 1983; Pankratz, Fausti, & Peed, 1975) applied twoalternative forced-choice procedures to patients with

Hiscock Forced-Choice Test. Figure 1 Typical stimulus cards and response cards from the Hiscock test. Each trial begins with the presentation of a stimulus card for 5 s. After a 5-, 10-, or 15-s interval, the corresponding response card is presented. The patient responds by pointing to the correct number on the response card

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bogus memory deficits as well as patients with hysterical or malingered sensory deficits, and Lezak (1983) summarized the principle in the second edition of her book on neuropsychological assessment. Thus, the Hiscock test was not an entirely novel means of detecting malingering, but it was a significant refinement of the forced-choice methods that existed prior to the 1990s. The forced-choice principle is straightforward. When a person who has no knowledge of the correct answer must choose between alternative A and alternative B, performance will converge on 50% correct over a series of trials. If the percent correct falls below 50% over the series of trials, and particularly if it falls so far below 50% as to lie outside the range expected on the basis of chance, then there can be only one explanation. The person must have sufficient relevant knowledge to be able to score above chance but, for some reason, he or she is using that knowledge to make predominantly incorrect choices. The person either has misunderstood the instructions or is deliberately using his or her knowledge to perform poorly. This principle underlies not only the Hiscock test but also the various other forced-choice tests that are known collectively as symptom validity tests (Strauss, Spreen, & Sherman, 2006).

Psychometric Data Results from the Hiscock test and other symptom validity tests can be interpreted in either of two ways. If decisions are made on the basis of performance that falls significantly below chance, positive outcomes are almost unequivocal indicators of dishonest performance, but sensitivity is likely to be unacceptably low as only a minority of malingering patients will be identified. If classification is based on empirically determined cutoff scores, the sensitivity of the test will increase dramatically but with possibly decreased specificity (i.e., more false positives). Clinicians typically regard either 90% or 95% correct as an appropriate cutoff score for the Hiscock test (Guilmette, Hart, & Giuliano, 1993; Prigatano & Amin, 1993). In most clinical settings, honestly performing patients typically make no errors; thus, a score below 95% would indicate probable malingering or lack of cooperation with the assessment procedures. Available evidence indicates that the specificity of the Hiscock test seldom falls below 90% even when a cutoff score of 95% is used (e.g., D’Arcy & McGlone, 2000; Guilmette et al., 1993; Inman & Berry, 2002; Prigatano & Amin, 1993; Vickery, Berry, Inman, Harris, & Orey, 2001). Specificity does become a concern if the objective is to differentiate

malingering from severe dementia (Hiscock & Hiscock, 1989; Prigatano et al., 1997).

Clinical Uses The Hiscock test has been evaluated not only in clinical studies but also in analog studies, in which healthy subjects or patients with known neurological disorders are instructed to feign memory impairment (e.g., Inman & Berry, 2002). Results of these different evaluations converge on three conclusions. First, as a group, symptom validity tests such as the Hiscock test are more accurate than other kinds of tests in differentiating malingering (or suboptimal performance) from honest performance. Researchers nevertheless caution against relying entirely on any test to formulate a conclusion about a patient’s effort or honesty. Rather, results from a symptom validity test should be corroborated by other information such as the presence or absence of incentives to feign impairment, performance on neuropsychological tests (with particular consideration of embedded measures of effort), the patient’s history, and self-reported symptoms (Slick, Sherman, & Iverson, 1999). Even though below-chance performance on a test such as the Hiscock test constitutes definitive evidence of dishonest performance on that particular test, it does not automatically nullify the validity of scores on other tests nor does it automatically invalidate the patient’s self-reported symptoms and disabilities. The second conclusion from evaluation studies is that, among symptom validity tests, the Hiscock test is especially useful for identifying dishonest performance in a population of moderately to severely impaired patients (e.g., D’Arcy & McGlone, 2000; Guilmette et al., 1993, Guilmette, Hart, Giuliano, & Leininger, 1994; Prigatano & Amin, 1993; Prigatano et al., 1997). This is probably due to its minimal cognitive demands. Although the requirement to recognize five-digit numbers initially may strike the patient as a challenging task, the difficulty is illusory. A correct response could be made on the basis of only the first or last digit in each series. While the greater complexity and higher difficulty level of some symptom validity tests might make those tests better suited for relatively high-functioning populations, simplicity and low difficulty are advantageous when the objective is to detect dishonest test performance in individuals with unequivocal neurological impairments associated with dementia, stroke, documented severe or moderate traumatic brain injury, etc. (e.g., D’Arcy & McGlone, 2000; Prigatano et al., 1997).


The third conclusion from evaluation studies is that symptom validity tests seldom elicit below-chance performance in suspected malingerers. Although the actual proportion of malingering patients who score below chance is difficult to estimate and probably varies greatly across clinical settings, experimental studies indicate that no more than one-third of normal subjects perform significantly below chance when instructed to feign impairment (Hiscock, Branham, & Hiscock, 1994). Fortunately, as noted above, the clinical utility of symptom validity tests can be enhanced considerably by employing normative standards (i.e., 90% or 95% correct) in addition to the definitive standard of belowchance performance. Identification of feigned or exaggerated cognitive impairment is recognized as critical to interpreting patients’ test performances in clinical neuropsychology settings. The Hiscock test probably has had less visibility as a clinical test than as a stimulus for the development of other related instruments such as the Portland Digit Recognition Test (Binder & Willis, 1991), the Victoria Symptom Validity Test (Slick, Hopp, Strauss, & Thompson, 1997), the Test of Memory Malingering (Tombaugh, 1996), and the Letter Memory Test (Inman, Vickery, Berry, Lamb, Edwards & Smith, 1998). Symptom validity tests, including the Hiscock test, are highly effective means of detecting dishonest performance by adult patients on tests of learning and recall. The forced-choice principle can be extended to realms of functioning other than memory (e.g., Hiscock, Rustemier, & Hiscock, 1993) and to the detection of suboptimal test performance by children (Strauss et al., 2006).

Cross References ▶ Computerized Assessment of Response Bias ▶ Malingering ▶ Portland Digit Recognition Test ▶ Test of Memory Malingering ▶ Victoria Symptom Validity Test

References and Readings Binder, L. M., & Pankratz, L. (1987). Neuropsychological evidence of a factitious memory complaint. Journal of Clinical and Experimental Neuropsychology, 9, 167–171. Binder, L. M., & Willis, S. C. (1991). Assessment of motivation after financially compensable minor head trauma. Psychological Assessment: A Journal of Consulting and Clinical Psychology, 3, 175–181. Brady, J. P., & Lind, D. L. (1961). Experimental analysis of hysterical blindness. Archives of General Psychiatry, 4, 331–339.

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D’Arcy, R. C. N., & McGlone, J. (2000). Profound amnesia does not impair performance on 36-item digit memory test: A test of malingered memory. Brain and Cognition, 44, 54–58. Grosz, H. J., & Zimmerman, J. (1965). Experimental analysis of hysterical blindness: A follow-up report and new experimental data. Archives of General Psychiatry, 13, 255–260. Guilmette, T. J., Hart, K. J., & Giuliano, A. J. (1993). Malingering detection: The use of a forced-choice method in identifying organic versus simulated memory impairment. The Clinical Neuropsychologist, 7, 59–69. Guilmette, T. J., Hart, K. J., Giuliano, A. J., & Leininger, B. E. (1994). Detecting simulated memory impairment: Comparison of the Rey Fifteen-Item Test and the Hiscock Forced-Choice Procedure. The Clinical Neuropsychologist, 8, 283–294. Hiscock, C. K., Branham, J. D., & Hiscock, M. (1994). Detection of feigned cognitive impairment: The two-alternative forced-choice method compared with selected conventional tests. Journal of Psychopathology and Behavioral Assessment, 16, 95–110. Hiscock, C. K., Rustemier, P. J., & Hiscock, M. (1993). Determination of criminal responsibility: Application of the two-alternative forcedchoice stratagem. Criminal Justice and Behavior, 20, 391–405. Hiscock, M., & Hiscock, C. K. (1989). Refining the forced-choice method for the detection of malingering. Journal of Clinical and Experimental Neuropsychology, 11, 967–974. Inman, T. H., Vickery, C. D., Berry, D. T. R., Lamb, D. G., Edwards, C. L., & Smith, G. T. (1998). Development and initial validation of a new procedure fo evaluating adequacy of effort given during neuropsychological testing: The letter memory test. Psychological Assessment, 10, 128–139. Inman, T. H., & Berry, D. T. R. (2002). Cross-validation of indicators of malingering: A comparison of nine neuropsychological tests, four tests of malingering, and behavioral observations. Archives of Clinical Neuropsychology, 17, 1–23. Lezak, M. D. (1983). Neuropsychological assessment (2nd ed.). New York: Oxford University Press. Pankratz, L. (1979). Symptom validity testing and symptom retraining: Procedures for the assessment and treatment of functional sensory deficits. Journal of Consulting and Clinical Psychology, 47, 409–410. Pankratz, L. (1983). A new technique for the assessment and modification of feigned memory deficit. Perceptual and Motor Skills, 57, 367–372. Pankratz, L., Fausti, S. A., & Peed, S. (1975). A forced-choice technique to evaluate deafness in the hysterical or malingering patients. Journal of Consulting and Clinical Psychology, 43, 421–422. Prigatano, G. P., & Amin, K. (1993). Digit Memory Test: Unequivocal cerebral dysfunction and suspected malingering. Journal of Clinical and Experimental Neuropsychology, 15, 537–546. Prigatano, G. P., Smason, I., Lamb, D. G., & Bortz, J. J. (1997). Suspected malingering and the Digit Memory Test: A replication and extension. Archives of Clinical Neuropsychology, 12, 609–619. Slick, D. I., Hopp, G., Strauss, E., & Thompson, G. B. (1997). Victoria Symptom Validity Test. Odessa, FL: Psychological Assessment Resources. Slick, D. I., Sherman, E. M. S., & Iverson, G. L. (1999). Diagnostic criteria for malingered neurocognitive dysfunction: Proposed standards for clinical practice and research. The Clinical Neuropsychologist, 13, 545–561. Strauss, E., Spreen, O., & Sherman, E. M. (2006). A compendium of neuropsychological tests: Administration, norms, and commentary (3rd ed.). New York: Oxford University Press.

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Tombaugh, T. (1996). Test of Memory Malingering (TOMM). North Tonawanda, NY: Multi Health Systems. Vickery, C. D., Berry, D. T. R., Inman, T. H., Harris, M. J., & Orey, S. A. (2001). Detection of inadequate effort on neuropsychological testing: A meta-analytic review of selected procedures. Archives of Clinical Neuropsychology, 16, 45–73.

Hiscock Malingering Test ▶ Hiscock Forced-Choice Test

Histamine Antagonist ▶ Antihistamines

History (Medical, Social, Psychological) B RADLEY A XELROD, C HRISTIAN S CHUTTE John D. Dingell VA Medical Center Detroit, MI, USA

Synonyms Background information

Definition Historical material regarding the examinee’s injury or medical event, acute treatment, subsequent treatment, developmental milestones, medical status, vocational history, educational background, and other foundational material against which test results are evaluated.

Current Knowledge When obtaining background information from an individual being examined, clinicians will often choose to review available materials documenting the patient’s accident, medical treatment, educational records, employment history, mental health treatment, and other historical information. The information is obtained through a face-to-face clinical interview as well as from

a review of existing documented records. The written materials may provide an objective reporting of the information. The interview may provide a subjective reporting. However, subjectively recalled information from the patient alone might fail to provide a complete or accurate history. One use of the history is to estimate an individual’s maximal capabilities based on their academic achievement and employment history. The highest level of education achieved and the evaluation of performance in school can provide an estimate of overall cognitive functioning. Similarly, the type of employment and occupational activities can provide estimates of cognitive functioning. Likewise, a thorough review of an individual’s medical and mental health history offers a context against which a current evaluation can be placed. If prior brain injuries are documented, then the additional effects of a current event might be viewed differently. Concurrent medical conditions, such as chronic obstructive pulmonary disease, sleep apnea, diabetes mellitus, and vascular disease, can have cognitive effects unrelated to an acute event. Similarly, mental health diagnoses relating to alcohol dependence, substance abuse, and severe mental illness can also have effects on cognition. Hence, the need to gain access, through interview and documentation, details pertaining to past and current medical, mental health, and academic and vocational information. In the context of an evaluation, a review of objectively authored paper records independent of patient report can provide a more thorough assessment. Patients may not recall aspects of an accident, specific injuries they sustained, cognitive rehabilitation, dates of prior assessments, lengths of hospitalization, or details regarding ongoing care. In addition to obtaining information external to the individual that they are unable to recall, objective records offer comprehensive historical data relating to prior medical care, including past inpatient hospitalizations. Furthermore, treatment records and discharge data from prior mental health and substance abuse treatment programs offer important information often authored by like-minded professionals. Of course, the ability to review raw neuropsychological test data, or at least the narrative report, from a previous evaluation is invaluable in determining how a current evaluation compares to a prior one.

Cross References ▶ Premorbid Estimate ▶ Premorbid Functioning ▶ Neuropsychological Report

Holistic

References and Readings Hebben, N., & Milberg, W. (2009). Essentials of neuropsychological assessment (2nd ed.). Hoboken, NJ: Wiley.

HO ▶ Heterotopic Ossification

Hold Measures ▶ Hold–Don’t Hold Tests

Hold–Don’t Hold Tests G LEN E. G ETZ Allegheny General Hospital Pittsburgh, PA, USA

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be resistant to change. These tests are known as ‘‘hold’’ subtests. The ‘‘hold’’ subtests from traditional intelligence measures, such as the Wechsler Intelligence Scale-III, include vocabulary, information, picture completion, and object assembly. The results from these measures are thought to best represent a relatively accurate presentation of pre-morbid functioning. It is thought that performance on the vocabulary subtest is the least likely to change and is therefore the best indicator of pre-morbid functioning. The ‘‘don’t hold’’ tests are more susceptible to change in performance following a brain insult or cognitive-based disturbance. The ‘‘don’t hold’’ subtests are often timed or involve a higher degree of attention, aspects of cognitive functioning that are more likely to be compromised by brain insult. Research suggests that ‘‘hold’’ tests are often inaccurate, simplistic, and not reliable of premorbid functioning. Specifically, left-hemisphere brain damage has been found to negatively effect ‘‘hold’’ measures. Furthermore, these approaches assume that an individual has relatively similar level of functioning across various cognitive domains, a condition that is frequently not met. Regardless, utilization of this technique along with other techniques, such as consideration of past levels of academic and occupational achievement, can assist in estimating pre-morbid functioning even if it is not sufficiently accurate when used in isolation.

Synonyms Hold measures

Definition Tests that in comparison to each other are thought to be either relatively resistant to aging or cognitive impairment associated with brain dysfunction or relatively sensitive to the same processes.

Cross References ▶ National Adult Reading Test ▶ Premorbid Estimate ▶ Premorbid Functioning

References and Readings Vanderploog, R. D. (1994). Estimating premorbid level of functioning. In Clinician’s guide to neuropsychological assessment (pp. 43–69). Hillsdale, NJ: Erlbaum.

Current Knowledge When interpreting performance on cognitive tests in individuals who sustain brain insult or cognitive disturbance, it is often important to consider pre-morbid level functioning or innate cognitive ability. One approach toward estimating pre-morbid intelligence is by relying on tests that measure present abilities that are thought to

Holistic ▶ Simultaneous Processing

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Holistic Brain Injury Rehabilitation

Holistic Brain Injury Rehabilitation J AMES F. M ALEC Rehabilitation Hospital of Indiana Indianapolis, IN, USA

Definition Holistic brain injury (BI) rehabilitation is an approach to postacute (posthospital) rehabilitation following brain injury that is directed at the ‘‘whole person.’’ As such, holistic programs include interventions that target cognitive, affective, behavioral, physical, social, and vocational difficulties and barriers confronting the person with BI.

Historical Background Holistic brain injury rehabilitation was pioneered by Yehuda Ben-Yishay, Ph.D., in the 1980s, and elaborated by George P. Prigatano, Ph.D. In 1994, Lance Trexler, Ph.D., convened a consensus conference in Zionsville, IN, that included Dr. Ben-Yishay and other seminal practitioners in holistic rehabilitation. This group identified the following key features of holistic rehabilitation:

Holistic rehabilitation planning Neuropsychological orientation Individualized goal setting Therapeutic milieu Outcome-oriented rehabilitation planning High intensity of rehabilitation program Brain injury rehabilitation expertise

Treatment Procedures Rehabilitation typically occurs in a group setting and capitalizes on the therapeutic milieu. The treatment team works to develop a milieu in which participants with brain injury confront their limitations and identify their strengths with support and feedback from both staff and other patients. Family and others involved play an integral role in the rehabilitation process. Rehabilitation in such programs is intensive, typically occurring 5 days each week for most of the day and extending for 6 months

or more. An integral component of the therapeutic milieu is the transdisciplinary team. In contrast to an interdisciplinary team (that is, a multidisciplinary team that coordinates their interventions), a transdisciplinary team not only coordinates their interventions but is able to assume each other’s roles temporarily to respond therapeutically to patients. The theory behind the transdisciplinary approach is that, because of cognitive impairments, patients with brain injury require a response to targeted behaviors in close temporal proximity to the occurrence of these behaviors. For instance, an angry response from a patient during a session facilitated by an occupational therapist requires the occupational therapist momentarily to assume the role of the psychologist and respond according to the behavioral plan to address the patient’s anger. Conversely, memory concerns that may be primarily addressed in the program by the occupational therapist and present during a session led by the psychologist will require the psychologist to assume the occupational therapist’s role and respond according to the plan outlined for memory rehabilitation. Because of deficits in memory and generalization of learning, patients with brain injury often require a therapeutic response in the ‘‘here and now’’ and cannot wait to process therapeutic opportunities at designated times in the future.

Efficacy Information Research on the effectiveness of holistic rehabilitation has generally reported positive results but has been criticized for the absence of controls and subject selection bias. Because of the intensity and length of holistic rehabilitation programs, identification of viable control conditions is challenging. Over the years, holistic day rehabilitation programs have evolved toward group and individual interventions that focus on specific patient goals within a more limited time frame while retaining a transdisciplinary team and a therapeutic milieu. Studies of such programs with nonrandomized controls have reported superior results for the holistic programs in contrast to standard of care or historic controls. Because of the intensity and cost of holistic day treatment, such programs merit further scrutiny and further investigation to determine what types of patients may require this level of postacute intervention. Current knowledge and clinical experience suggest that holistic day treatment may be required to improve substantially the social and vocational integration of patients with severe and pervasive deficits in multiple domains including impaired self-awareness.

Homework

Cross References ▶ Cognitive Rehabilitation ▶ Postacute Brain Injury Rehabilitation

References and Readings Christensen, A. -L., & Uzzell, B. P. (Eds.). (2000). International handbook of neuropsychological rehabilitation. New York: Kluwer/Plenum. Cicerone, K. D., Dahlberg, C., Kalmar, K., Langenbahn, D. M., Malec, J. F., Bergquist, T. F., et al. (2000). Evidence-based cognitive rehabilitation: Recommendations for clinical practice. Archives of Physical Medicine and Rehabilitation, 81, 1596–1615. Cicerone, K. D., Dahlberg, C., Kalmar, K., Malec, J. K., Langenbahn, D. M., Felicetti, T., et al. (2005). Evidence-based cognitive rehabilitation: Updated review of the literature from 1998 through 2002. Archives of Physical Medicine and Rehabilitation, 86, 1681–92.

Home Modification ▶ Environmental Modifications

Homework G ERALD S HOWALTER University of Virginia School of Medicine Charlottesville, VA, USA

Synonyms Between-session assignments; Cognitive exercises

Definition Homework, as used in cognitive rehabilitation after acquired brain injury, is any task or series of tasks assigned by a therapist to be completed between treatment sessions by a participant in cognitive rehabilitation, for the purpose of helping the participant consolidate information and strategies learned in treatment sessions and apply this information within real-world settings. The involvement of a participant’s family member or significant other is often essential for support and observation of the participant’s progress in completing and benefiting from these between-session assignments. Homework encompasses a broad array of strategies and formats depending upon the specific cognitive

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functions or behaviors to be addressed, and the unique needs of participants. Assignments to address executive functioning, for instance, might involve identifying an overall task of interest to the participant, identifying component subtasks, and asking the participant to complete each subtask in sequence until the goal is reached. If memory difficulties are also present, each task component might be entered into a personal digital assistant or smart phone with capability to alert and remind the participant of tasks to be accomplished. Homework addressing attention problems might call for the participant to practice staying focused on a chosen activity of interest for progressively lengthier periods of time, with use of a timer and log book to record these activities for subsequent review and monitoring. Homework is also used by family therapists and providers of individual counseling or psychotherapy for purposes similar to, but slightly different from those above. Within these treatment domains, the emphasis of homework is less cognitive in nature, and is more focused on helping participants improve skills for coping with emotional distress, as well as strengthening patterns of communication and problem solving within family systems and in the context of important social relationships. For example, an individual with a social anxiety disorder might be given homework assignments to utilize stress management skills learned in treatment sessions within actual social settings between treatment visits. Effective homework exercises tend to be highly relevant to the real-life activities and the needs of participants. Ideally, such exercises are tailored to maximize and maintain the interest of participants. They involve a series of brief, concrete, and readily understood steps to complete, with instructions presented in modalities appropriately suited to the client’s functional capabilities. Tasks comprising these exercises can be repeatedly practiced and incorporated into other daily activity routines.

Cross References ▶ Cognitive Rehabilitation ▶ Ecological Validity ▶ Generalization of Skills

References and Readings Holland, A. L., Ratner, N. B., & Turkstra, L. S. (Eds.). (2005). Evidencebased practice for cognitive-communication disorders after traumatic brain injury.

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Homonymous Hemianopia

Definition

Homonymous Hemianopia

A visual defect characterized by blindness in one-half of the visual field that is comparable in both eyes. For example, with a left homonymous hemianopsia, when looking straight ahead the patient would not be able to see anything to the left of midline regardless of which eye was being tested. Such a visual field defect is the result of an interruption of the visual pathways anywhere after the partial crossing of the visual fibers in the optic chiasm to the occipital cortex. This then could involve the optic tract, the lateral geniculate nucleus, the optic radiations, or the primary visual cortex. Two potential exceptions to this rule might be noted. One is that if the lesion producing the hemianopsia is in the visual cortex, the patient might retain a small amount of foveal vision in the very central part of the left visual field, a phenomenon known

▶ Homonymous Hemianopsia

Homonymous Hemianopsia K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Synonyms Homonymous hemianopia

Right eye

Left eye Temporal Hemifield

Nasal Hemifield

Nasal Hemifield

Visual fields

Temporal Hemifield

Lesion

Lt. eye Rt. eye

1

monocular blindness

2

Bitemporal (heteronymous) heminanopia1

3

Ipsilateral Right nasal hemianopia

4

Left homonymous hemianopia

5

Left inferior quadrantanopia2

6

Left superior quadrantanopia3

7

Left homonymous hemianopia4

8

Left homonymous hemianopia with macular sparing

Lesion 1

Lesion 3 Lesion 4

Lesion 2 Lat. Geniculate body

Inferior (temporal) optic radiations (Meyer’s Loop) Superior (parietal) optic radiations

Geniculo– calcarine fibers

Lesion 6 Lesion 5 Lesion 7

Calcarine cortex

C (LG) Lesion 8

1 May rusult from tumor pressing on Opitc Chiasm. Visual field deficit usually not this cleanly divided or symmetrical. 2 May also result from lesions restricted to Cuneus 3 May also result from lesions restricted to Lingual Gyrus. 4 Total hemianopia less likely as compared to lesions of Optic Tract.

5–7 Homonymous Hemianopsia. Figure 1 Courtesy John E. mendoza (Mendoza & Foundas, 2008)

Homonymous Quadrantanopsia

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as macular sparing (probably due to overlapping vascular distributions for this critical visual area). The second is that because of their relatively broad distribution after leaving the lateral geniculate nuclei, it would be relatively rare for lesions of the optic radiations to produce a complete homonymous hemianopsia. In the latter instance one would more commonly expect a quadrantanopsia or partial (left or right) visual field cut. The most common cause of such defects is stroke, but they can be caused by any lesion that interrupts these pathways. A transient disruption of the visual field can be associated with migraine headaches (Fig. 1).


Cross References

Definition

▶ Homonymous Quadrantanopsia ▶ Visual System

Homonymous quadrantanopsia is defined as the visual loss that is restricted to one quadrant of the visual field and is

Left eye Temporal hemifield

Wilson-Pauwek, L., Akesson, E. J., Stewart, P. A., & Spacey, S. D. (2002). Cranial nerves in health and disease. Hamilton, ONT: B.C. Decker, Inc.

Homonymous Quadrantanopsia K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

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Right eye

Nasal hemifield

Nasal hemifield

Visual fields

Temporal hemifield

Lesion Left eye Right eye

Lesion 1

Lesion 3

Lat. Geniculate body

Inferior (temporal) optic radiations (Meyer’s Loop) Superior (parietal) optic radiations

1

Monocular blindness

2

Bitemporal (heteronymous) hemianopia1

3

Ipsilateral right nasal hemianopia

4

Left homonymous hemianopia

5

Left inferior quadrantanopia2

6

Left superior quadrantanopia3

7

Left homonymous hemianopia4

8

Left homonymous hemianopia with macular sparing

Lesion 4

Lesion 2

Geniculocalcarine fibers

Lesion 6 Lesion 5 Lesion 7

Calcarine cortex

C (LG) Lesion 8

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1 May result from tumor pressing on opitc Chiasm. Visual field deficit sually not this cleanly divided or symmetrical. 2 May also result from lesions restricted to Cuneus 3 May also result from lesions restricted to Lingual Gyrus. 4 Total hemianopia less likely as compared to lesions of Optic tract.

Homonymous Quadrantanopsia. Figure 1 Courtesy Mendoza (Mendoza & Foundas 2008)

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Homotypic Cortex

comparable in both eyes. Thus, when looking straight ahead, patients may have difficulty seeing objects or movement in the upper or lower quadrant of the visual field, but it will be in the same quadrant (i.e., either to the right or to the left) regardless of which eye is being tested. Depending on the extent of the lesion, slightly more or slightly less than one quarter of the visual field may actually be affected. Homonymous quadrantanopsia usually results from lesions posterior to the lateral geniculate nuclei, which encroach either on the optic radiations or the primary visual cortex. The visual fibers from the upper portion of the retina retain their relative superior position as they travel back to the visual cortex. The opposite is true of the fibers derived from the lower portion of the retina. Consequently, damage restricted to the more dorsal portions of the optic radiations (underlying the parietal lobes) or to the cortex on the upper bank of the calcarine fissure in the occipital lobe (the cuneus) will result in an inferior homonymous quadrantanopsia. Conversely, lesions affecting the ventral portions of the optic radiations (underlying the temporal lobes or Meyer’s loop) or the lower bank of the occipital cortex (the lingual gyrus) produce superior quadrantic deficits (Fig. 1).

Cross References

Definition Those areas of the neocortex in which the individual cellular layers are easily distinguishable (as opposed to idiotypic (primary) cortices in which layers 2 through 5 are more uniform). Homotypic cortex is generally further subdivided into unimodal and heteromodal association cortices, depending on whether it receives input from a single or multiple sensory modalities.

Cross References ▶ Cerebral Cortex ▶ Heteromodal Cortex ▶ Idiotypic Cortex ▶ Unimodal Cortex

References and Readings Mesulam, M. (2000). Behavioral neuroanatomy: Large-scale networks, association cortex, frontal syndromes, the limbic system, and hemispheric specialization. In M. Mesulam (Ed.), Principles of behavioral and cognitive neurology (2nd ed., pp. 1–120). New York: Oxford University Press. Stewart, O. (2000). Functional neuroscience. New York: Springer.

▶ Homonymous Hemianopsia ▶ Visual System

Homunculus References and Readings Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach. New York: Springer.

T HESLEE J OY D E P IERO Boston University School of Medicine Boston, MA, USA

Definition

Homotypic Cortex M ARYELLEN R OMERO Tulane University Health Sciences Center New Orleans, LA, USA

Synonyms Association cortex

A homunculus is a pictorial representation of the motor areas of the precentral gyrus controlling voluntary movement in the human brain. Each body part is represented by a drawing of that part, scaled to reflect the proportion of the motor strip dedicated to controlling it. Large parts of the motor strip control the larynx, lips, face, thumb, and individual fingers. Much smaller parts control the proximal arm, trunk, leg, and foot, giving the homunculus (‘‘little man’’) a distorted appearance. The larynx is closest to the Sylvian fissure, the shoulder and hips at the superior surface of the hemisphere, and

Hooper Visual Organization Test

the legs, feet, and urogenital control on the medial surface of the hemisphere.

Cross References ▶ Motor Strip ▶ Precentral Gyrus ▶ Sylvian Fissure

Hooper Visual Organization Test M ELISSA B UTTARO Brown University Providence, RI, USA

Synonyms HVOT

Description The Hooper Visual Organization Test (HVOT; Hooper, 1958) is a 30-item screening instrument that measures visual organizational skills. It consists of line drawings of simple objects that have been cut into pieces and rearranged, such as in a puzzle. The examinee’s task is to name what each object would be if the pieces were put back together. Successful performance on this task depends on primary visual analytic skills, the capacity to integrate or synthesize fragmented pieces of an object into a gestalt, and the ability to label objects either verbally or in writing (Hooper, 1983).

Background and Psychometric Data 1. Administration: The HVOT was originally designed to identify patients in mental hospitals with organic brain conditions (Hooper, 1983). It is intended for use in adolescents and adults, aged 13 and older. Administration can take place individually, in which case the examinee names each object aloud, or in a group format, in which case examinees write their responses in the spaces provided in the test booklet. The test items start at an easy level, but some of the later items are less easily recognized. Administration may be discontinued

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after five consecutive errors without significantly affecting the rating ability of this test (Wetzel & Murphy, 1991). The entire test can generally be administered and scored in less than 15 min. The absence of time limits in the HVOT makes it a more specific measure of visual integration ability; however, it is not a ‘‘pure’’ measure of visual organization, since it does require basic vocabulary skills and the capacity to name common objects. A multiple-choice response format for the HVOT was proposed by one group of researchers (Schultheis, Caplan, Ricker, & Woessner, 2000) to reduce the object naming demand. Because some objects are more culturally specific (e.g., ▶ lighthouse, alphabet block), the use of this test with individuals from varied cultural backgrounds may not be appropriate. However, some authors have suggested that cross-cultural differences in performance are minimal; for example, Giannakou and Kosmidis (2006) recently reported that the HVOT is a reliable and valid neuropsychological tool for the Greek population. 2. Scoring: To score the HVOT, one point is given for each correct item, and partial credit (1/2 point) is assigned for certain responses that occur with moderate frequency in a non-impaired population. The Total Raw Score for the HVOT is obtained by summing the number of correct and partially correct responses, and it ranges from 0 to 30. Instructions for obtaining a Corrected Raw Score, which adjusts for differences in the examinee’s age and educational level, are available in the test manual (Hooper, 1983). However, it should be noted that these corrections are based on an allmale, Veterans Administration hospital population. The Corrected Raw Score may be transformed to a T score by using a table in the test manual appendix. More recent normative data are available in Mitrushina, Boone, and D’Elia (1999) and Spreen and Strauss (1998). 3. Quantitative Interpretation: Interpretation of the HVOT has historically depended on the Total Raw Scores. Higher raw scores reflect greater ability to organize visual stimuli, whereas lower raw scores suggest difficulty in this area and possibly organic impairment. The major advantage in using Total Raw scores for interpretation is that they are familiar to established users of the test and are often used in the research literature; however, a major drawback to this approach is that raw scores do not make any adjustments for the patient’s age or educational level. Recommended raw cutoff scores have ranged from 20 to 25, depending on the population and purpose for the screening (Hooper, 1983).

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Hopkins Verbal Learning Test

As a general guideline for interpretation, Hooper (1958) suggested the following categories according to the degree of impairment: Severe (0–9 items correct), Moderate (10–19 correct), Mild (20–24 correct), and None (25–30 correct). Lezak (1995) suggests that cognitively intact individuals usually fail no more than six items; those who fail 7–11 items make up a borderline group who have a low to moderate likelihood of organic impairment (e.g., the mentally ill and individuals with mild to moderate brain disorders); and greater than 11 failures typically suggest organic brain pathology. In general, low scores on the HVOT usually indicate the presence of brain damage, since false-positive performances are rare (Lezak, 1995). 4. Qualitative Interpretation: In addition to the traditional quantitative scoring, qualitative examinations of responses can also be useful. The HVOT Test Manual (1983) describes four qualitative types of errors: 1. Isolate (‘‘concrete’’ or ‘‘part’’) responses refer to the use of just one piece of the picture to generate a response (e.g., a response of ‘‘flying duck’’ for item number one). These single elements may be stronger stimulus pulls than the instructions to combine the pieces (Lezak, 1995). 2. Perseverative responses occur when answers from a previous item are repeated in subsequent items. Although perseverative responses are less common, they can be useful indicators of stereotyped thinking, inability to switch response sets, or a preoccupation with particular themes. 3. A bizarre response lacks direct relationship to the test item. These responses may occur in individuals with schizophrenia, poor attention, or organic psychoses. 4. Neologistic responses are words that hold no meaning to the examiner. These types of errors occur almost exclusively in severely disturbed patients and can help differentiate between a functional and an organic etiology.

attending to any of the other pieces (e.g., item 22 might be described as a ‘‘pipe’’). In general, the HVOT can be a useful tool to help tease apart the nature of an individual’s visual impairment, by separating visual perceptual skills from three-dimensional construction abilities and graphic competency.

Cross References ▶ Embedded Figures Test ▶ Gollin Figures ▶ Judgment of Line Orientation

References and Readings Giannakou, M., & Kosmidis, M. H. (2006). Cultural appropriateness of the Hooper Visual Organization Test? Greek normative data. Journal of Clinical and Experimental Neuropsychology, 28(6), 1023–1029. Hooper, H. E. (1958). The Hooper Visual Organization Test manual. Los Angeles, CA: Western Psychological Services. Hooper, H. E. (1983). Hooper Visual Organization Test (VOT) manual. Los Angeles, CA: Western Psychological Services. Lezak, M. D. (1995). Neuropsychological assessment (3rd ed.). New York: Oxford University Press. Mitrushina, M. N., Boone, K. B., & D’Elia, L. F. (1999). Handbook of normative data for neuropsychological assessment. New York: Oxford University Press. Nadler, J. D., Grace, J., White, D. A., Butters, M. A., & Malloy, P. F. (1996). Laterality differences in quantitative and qualitative Hooper performance. Archives of Clinical Neuropsychology, 11(3), 223–229. Schultheis, M. T., Caplan, B., Ricker, J. H., & Woessner, R. (2000). Fractioning the Hooper: A multiple-choice response format. The Clinical Neuropsychologist, 14(2), 196–201. Spreen, O., & Strauss, E. (1998). A compendium of neuropsychological tests: Administration, norms, and commentary. New York: Oxford University Press. Wetzel, L., & Murphy, S. G. (1991). Validity of the use of a discontinue rule and evaluation of discriminability of the Hooper Visual Organization Test. Neuropsychology, 5(2), 119–122.

Hopkins Verbal Learning Test Clinical Uses The HVOT is thought to be sensitive to general neurological impairment and a useful indicator of both right and left hemisphere dysfunction (Lezak, 1995; Nadler, Grace, White, Butters, & Malloy, 1996). Patients with frontal lobe damage (usually right frontal lobe) or those who tend to view the world in a fragmented way sometimes respond to a single piece of the object, without

S TACY B ELKONEN One Gustave L. Levy Place New York, NY, USA

Synonyms HVLT-R

Hopkins Verbal Learning Test

Description The Hopkins Verbal Learning Test – Revised (HVLT-R) is the most recent (2001) version of the verbal learning and memory test. The current HVLT-R offers six alternate forms. Each form contains 12 nouns, four words each from one of three semantic categories (e.g., precious gems, articles of clothing, vegetables, etc.), to be learned over the course of three learning trials. Approximately 20–25 min later, a delayed recall trial and a recognition trial are completed. The delayed recall requires free recall of any words remembered. The recognition trial is composed of 24 words, including the 12 target words and 12 false-positives, 6 semantically related, and 6 semantically unrelated. When scoring the HVLT-R, the three learning trials are combined to calculate a total recall score; the delayed recall trial creates the delayed recall score; the retention (%) score is calculated by dividing the delayed recall trial by the higher of learning trial 2 or 3; and the recognition discrimination index is comprised by subtracting the total number of false positives from the total number of true positives. These scores are then converted to age-based T scores based on a norm population of 1,179 English-speaking individuals aged 16–92 years old.

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Test-retest reliability coefficients range from 0.39 to 0.74 depending on the score (Benedict et al., 1998; Rasmusson, Bylsma, & Brandt, 1995). Research, outlined in the manual, has shown that the HVLT-R has construct, concurrent and discriminant validity.

Clinical Uses The HVLT-R was created as a brief verbal learning and memory test and therefore is ‘‘intended primarily for the use with the brain-disordered populations’’ (Brandt & Benedict, 2001). Since the HVLT-R has six alternate forms, it is useful in populations that require follow-up through neuropsychological assessment (e.g., degenerative diseases) and to examine whether treatment has been effective. The HVLT-R has been used extensively in the research with diverse populations including the elderly, individuals with mild cognitive impairment, dementia, HIV, traumatic brain injury, Huntington’s disease, Parkinson’s disease, schizophrenia, multiple sclerosis (Chiaravalloti, DeLuca, Moore, & Ricker, 2005), and individuals who have undergone chemotherapy.

Cross References Historical Background The HVLT was initially published in 1991 (Brandt, 1991) as an alternative to longer list learning tasks and included the three learning trials and a recognition trial given immediately after the three learning trials. The addition of the delayed recall trial came in 1998, (Benedict, Schretlen, Groninger, & Brandt, 1998) creating the HVLT-R.

Psychometric Data Reliability of the alternate forms of the HVLT-R found that the learning trial score, delayed recall score, retention (%) score, and number of delayed recognition truepositives were not significantly different based on the form (Benedict et al., 1998). However, the number of false-positive errors and the recognition discrimination index were significantly different based on the form. Therefore, the normed recognition discrimination score for Forms 1, 2, and 4 are equivalent and different than the recognition discrimination index for Forms 3, 5, and 6.

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▶ Bushcke Selective Reminding Test ▶ California Verbal Learning Test (California Verbal Learning Test-II) ▶ Rey Auditory Verbal Learning Test

References and Readings Benedict, R. H. B., Schretlen, D., Groninger, L., & Brandt, J. (1998). The Hopkins verbal learning test-revised: Normative data and analysis of interform and test–retest reliability. Clinical Neuropsychologist, 12, 43–55. Brandt, J. (1991). The Hopkins verbal learning test: Development of a new memory test with six equivalent forms. Clinical Neuropsychologist, 5, 125–142. Brandt, J., & Benedict, R. H. B. (2001). Hopkins verbal learning test – Revised. Administration manual. Lutz, FL: Psychological Assessment Resources. Chiaravalloti, N. D., DeLuca, J., Moore, N. B., & Ricker, J. H. (2005). Treating learning impairments improves memory performance in multiple sclerosis: A randomized clinical trial. Multiple Sclerosis, 11, 58–68. Rasmusson, D. X., Bylsma, F. W., & Brandt, J. (1995). Stability of performance on the Hopkins verbal learning test. Archives of Clinical Neuropsychology, 10, 21–26.

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Horizontal Gaze Palsy

Horizontal Gaze Palsy ▶ Lateral Gaze Palsy

Hormones B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms Internal secretion

Definition A hormone is a chemical messenger and is mainly synthesized within the endocrine glands, testis, and/or ovaries, but can also be produced by cells having neuroendocrine function (such as the hypothalamic–adrenal pathway). Once hormones are released, they mainly travel through the bloodstream (although they can also have cell–cell communications) and attach to a specific receptor of a target cell. Depending on the receptor, the hormone will activate a certain process (gene expression, metabolic pathway, etc.). Hormones are very powerful and affect almost every function of the body (growth and development, reproduction, mood, metabolism, and so on).

Cross References ▶ Autoreceptor ▶ Hypothalamus ▶ Neuropeptides ▶ Neurotransmitters

References and Readings Erlanger, D. M., Kutner, K. C., & Jacobs, A. R. (1999). Hormones and cognition: Current concepts and issues in neuropsychology. Neuropsychology Review, 9(4), 175–207.

Forgie, M., & Kolb, B. (2003). Manipulation of gonadal hormones in neonatal rats alters the morphological response of cortical neurons to brain injury in adulthood. Behavioral Neuroscience, 117(2), 257–262. Galea, L., Uban, K., Epp, J., Brummelte, S., Barha, C., Wilson, W., et al. (2008). Endocrine regulation of cognition and neuroplasticity: Our pursuit to unveil the complex interaction between hormones, the brain, and behaviour. Canadian Journal of Experimental Psychology/ Revue canadienne de psychologie expe´rimentale, 62(4), 247–260. Kiecolt-Glaser, J., Bane, C., Glaser, R., & Malarkey, W. (2003). Love, marriage, and divorce: Newlyweds’ stress hormones foreshadow relationship changes. Journal of Consulting and Clinical Psychology, 71(1), 176–188. Mead, L., & Hampson, E. (1996). Asymmetric effects of ovarian hormones on hemispheric activity: Evidence from dichotic and tachistoscopic tests. Neuropsychology, 10(4), 578–587.

House-Tree-Person Test R OBERT M. G ORDON , A LEXANDRA RUDD -B ARNARD New York University Langone Medical Center New York, NY, USA

Synonyms H-T-P Test

Description The House-Tree-Person (H-T-P) technique, developed by John Buck (1948) and Emmanuel Hammer (1958), is one of the most widely used projective tests for children and adults. It can be used with individuals aged 3 years and older and is almost entirely unstructured; the respondent is simply instructed to make a freehand drawing of a house, a tree, and a person. Analysis of the H-T-P is a two-phased, four-step process. In phase one, the first step in testing is nonverbal and almost entirely unstructured; the medium of expression is the freehand, pencil drawings of a house, tree, and person (Buck, 1966). The second step is verbal, apperceptive, and more formally structured. In it, the subject is given the opportunity to describe, define, and interpret his or her drawn objects and their respective environment, and to respond to various open-ended questions. In phase two, the first step again involves the freehand drawing of a house, tree, and person, but with crayons (Buck, 1966). The second step provides the subject with the opportunity to describe, define, and interpret his or her chromatic drawings and to respond to specific

House-Tree-Person Test

queries. Handler (1996) adapted a number of open-ended questions developed by Buck (1966) to explore the respondent’s underlying personality dynamics including: What do the house, tree, and person need the most and why? What kinds of things make the person sad, happy, or angry? What are the strongest and weakest parts of the house? Is the tree alone or in a group of trees? H-T-P drawings can be a valuable clinical tool for generating hypotheses about developmental and emotional functioning. The objects of the house, tree, and person were chosen because they were familiar items or concepts even to very young children, were often welcomed by inhibited and guarded individuals, and appeared to stimulate more revealing verbalizations than did other items (Handler, 1996). In the past decade or more, there has been a renewed interest regarding drawings and drawing directives used as adjuncts in the assessment process (Hammer, 1997; McNeilly & Gilroy, 2000; Safran, 2002; Silver, 1996). Within the psychological test battery, drawings serve a special function by offering a minimally threatening, yet maximally absorbing introduction that can reveal deeper psychological processes and information out of the subject’s awareness (West, 1998).

Historical Background The interpretation of the H-T-P has been largely dominated by psychoanalytic and developmental theory. Goodenough (1926), whose work was extended by Harris (1963), investigated normative development of human figure drawings from childhood through adolescence and related drawing maturation to intellectual development. Buck (1948) and Buck and Hammer (1969) introduced and evaluated H-T-P drawings as a reflection of developmental maturity and projectively as a measure of personality characteristics. Machover (1949) provided detailed clinical applications of projective drawings and suggested that individuals with psychopathology, including schizophrenia, obsessive–compulsive disorder, and schizoid tendencies, could be differentiated from each other and from ‘‘normal’’ individuals by looking at their drawings. Detailed scoring manuals such as that by Jolles (1964) describe the characteristics of the separate components of the house, tree, and person and give illustrations as to how various items should be scored. Since scoring and interpreting the H-T-P is difficult, clinicians administering the test must be properly trained; the test publishers provide a detailed 350-page administration and scoring manual. More recently, Hammer (1971) has expanded the clinical applications of projective drawings,

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illustrating their value for the psychotherapeutic process. For example, encouraging patients to draw in order to express themselves symbolically and the process going on within themselves.

Psychometric Data Quantitative Scoring A quantitative scoring scheme analyzing the details of the drawings was devised by Buck (1966) after it was speculated that H-T-P drawings may be used to assess intellectual maturity, including the ability to form abstract concepts (Sattler, 1988). According to this procedure, items are scored based on detail, proportion, and the perspective the subject used in producing his or her house, tree, and person, in accordance with the factor symbol assigned each item in the quantitative scoring tables (Buck, 1966). However, the H-T-P was never intended to be an intelligence test in the usually accepted definition of the term (Buck, 1966). Rather, it has been suggested that by comparing scores on the H-T-P and an intelligence test, H-T-P scores may provide insight into the effects of emotional factors on cognitive functioning. The primary use of the H-T-P is related to the qualitative scoring scheme in which the examiner subjectively analyzes the drawings and the responses to questions in a way that assesses subjects’ personality. Koppitz (1968) validated a number of emotional signs along with the ages that they become clinically significant for boys and girls. These indicators included impulsivity, transparencies, shading of the face, anxiety, shyness, and arms clinging to the side of the body (Handler, 1996). The presence of one or two emotional factors did not reflect significant psychopathology, whereas the presence of several emotional factors suggests a greater likelihood of clinical relevant psychopathology (Handler, 1996). Despite being a frequently used assessment tool, research involving H-T-P drawings has provided little support for their ability to provide diagnostic information such as child sexual abuse (Palmer et al., 2000) and selfesteem (Groth-Marnat & Roberts, 1998). Using a multivariate method utilizing hierarchical cluster analysis in order to explore the interrelated patterns of traits in the projective drawings, Vass (1998) found that the H-T-P drawings have definite, consistent inner structures. Vass (1998) determined that these structures must be different in the normal and various clinical groups. According to these findings, relevant deviation from this structure can be interpretable information about the pathology of patients.

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House-Tree-Person Test

Clinical Uses Qualitative Appraisal The major approach to the appraisal of a subject’s drawings and his spontaneous and induced comments concerning them is qualitative analysis and interpretation. The H-T-P can be interpreted utilized using both structural and content variables, as well as in a holistic, impressionistic manner. The structural variables concern stylistic elements of the drawings, including size, pressure, line quality, amount of details, proportion, perspective, shading, and erasure (Handler, 1996). For example, numerous erasures can indicate indecisiveness, anxiety, or conflict; size can be a reflection of self-esteem; and excessive shading may be an indication of anxiety/conflict. Placement on the right side of the page has been understood as a reflection of emotional stability, rigidly controlled behavior, an orientation toward the future, and a tendency to intellectualize, while placement on the left side of the page indicates extroversion, and orientation toward the past, and impulsivity (Handler, 1996). Of course, drawings skewed to the right side of the page are characteristic of patients with left-sided neglect resulting from right hemisphere lesions. Placement on the top of the page has been seen as a sign of striving for achievement and drawings on the bottom of the page suggest feelings of insecurity and a lack of selfconfidence (Handler, 1996). In general, the type and the amount of details used, the method of placing them, the order of producing them, and the emphasis placed upon them may be regarded as an index of the subject’s recognition of, concern with, and reactions to everyday living. For example, the more the essential details that are missing from the drawing, the greater the degree of psychopathology (Buck, 1966). The proportional values that the subject employs in his or her drawings may be regarded as a crude indicator of his or her ability to assign objective values to the elements of reality, of his ability to make judgments in a flexible manner, and/or his ability to solve the more immediate and concrete problems of daily living. If the roof is overly large in relation to the rest of the house, it is assumed that the subject devotes a considerable amount of time to fantasy (Buck, 1966). Evaluation of spontaneous verbal or written comments made by the subject while drawing can be informative, as these are usually triggered by the part of the drawing the subject has just completed, is working on, and about to draw (Buck, 1966). The specific details of the H-T-P can provide clinically relevant information. Particularly with children, the house is viewed as conveying one’s relationships with

parents and siblings (Handler, 1996). For example, a very small house may indicate a rejection of one’s family’s values. The tree is considered as representing deeper levels of one’s emotional history including trauma. A tree that has a slender trunk, but has large expansive branches might indicate a need for nurturance and dependency (Handler, 1996). The drawing of a person represents one’s self-concept, conflict areas, and attitude toward life (Handler, 1996). A drawing of person that has a great deal of facial details could indicate a need to present oneself in a favorable light, while lack of details could indicate withdrawal tendencies (Handler, 1996). Qualitative studies by Yama (1990) measuring the overall adjustment of Vietnamese children and Tharinger and Starks’ (1990) investigation comparing children with anxiety and mood disorders have indicated that qualitative and impressionistic measures of the human figure drawing tend to yield more useful clinical data than quantitative studies. Information from the H-T-P can also be utilized as an aid for exploration during psychotherapy and as a global measure of change in psychotherapy. Blatt and Ford (1994) conducted a study that measured changes related to intensive psychotherapy with psychiatrically hospitalized adults and found that the second drawings of the human figure tended to be more differentiated, centered, and organized. Clinician using the H-T-P should bear in mind that no single sign is an infallible indication of any strength or weakness, that each feature of the three drawings has multiple meanings, and that the amount of diagnostic and prognostic data from each of the points of analysis may vary greatly from subject to subject. In addition, although numerous clinical hypotheses can be formulated utilizing qualitative and impressionistic methods to the H-T-P, it is critical to corroborate these ideas with other data in the neuropsychological test battery and clinical interview (Handler, 1996). There should be a convergence of multiple sources of data to utilize the qualitative information from the H-T-P (Handler, 1996). In addition, the clinician should be highly cautious in using the information for forensic evaluations and when evaluating for sexual abuse (Garb, Wood, Lilienfeld & Nezworski, 2002; Palmer et al., 2000) and when interpreting drawings with individuals with perceptual, executive functioning, & graphomotor deficits.

Cross References ▶ Human Figure Drawing Test ▶ Projective Tests

Human Figure Drawing Tests

▶ Rorschach ▶ Sentence Completion Test ▶ Thematic Apperception Test

References and Readings Blatt, S., & Ford, R. (1994). Therapeutic change. New York: Plenum. Buck, J. N. (1948). The H-T-P. Journal of Clinical Psychology, 4, 151–159. Buck, J. N. (1966). The House-Tree-Person technique: Revised manual. Beverly Hills: Western Psychological Services. Buck, J. N. (1978). A qualitative and quantitative scoring manual. Journal of Clinical Psychology, 4, 397–405. Buck, J. N., & Hammer, E. F. (Eds.). (1969). Advances in House-TreePerson techniques: Variations and applications. Los Angeles: Western Psychological Services. Garb, H. N., Wood, J. M., Lilienfeld, S. O., & Nezworski, M. T. (2002). Effective use of projective techniques in clinical practice: Let the data help with selection and interpretation. Professional Psychology: Research and Practice, 5, 454–463. Goodenough, F. L. (1926). Measurement of intelligence by drawings. New York: Harcourt, Brace and World. Groth-Marnat, G., & Roberts, L. (1998). Human Figure Drawings and House Tree Person drawings as indicators of self-esteem: A quantitative approach. Journal of Clinical Psychology, 54, 219–222. Hammer, E. F. (1958). The clinical application of projective drawings. Springfield: Charles Thomas. Hammer, E. F. (1971). The clinical application of projective drawings. Springfield, IL: Charles C. Thomas. Hammer, E. F. (1997). Advances in projective drawing interpretation. Springfield, IL: Charles C. Thomas. Handler, L. (1996). The clinical issues of figure drawings. In C. S. Newmark (Ed.), Major psychological assessment instruments (2nd ed., pp. 206–293). Needham Heights, Mass.: Allyn & Bacon. Harris, D. B. (1963). Children’s drawings as measures of intellectual maturity. New York: Harcourt, Brace and World. Jolles, I. A. (1964). Catalogue for the qualitative interpretation of the HouseTree-Person (H-T-P). Los Angeles: Western Psychological Services. Koppitz, E. M. (1968). Psychological evaluation of children’s Human Figure Drawings. New York: Grune & Stratton. Machover, K. (1949). Personality projection in the drawing of the human figure. Springfield, IL: Charles C. Thomas. McNeilly, G., & Gilroy, A. (Eds.). (2000). The changing shape of art therapy: New developments in theory and practice. London: Jessica Kingsley. Palmer, L., Farrar, A. R., Valle, M., Ghahary, N., et al. (2000). An investigation of the clinical use of the House-Tree-Person projective drawings in the psychological evaluation of child sexual abuse. Child Maltreatment, 5, 169–175. Safran, D. S. (2002). Art therapy and AD/HD: Diagnostic and therapeutic approaches. London: Jessica Kingsley. Sattler, J. M. (1988). Assessment of children (3rd ed.). San Diego, CA: Jerome M. Sattler. Silver, R. (1996). Silver drawing test of cognition and emotion. Sarasota, FL: Ablin. Tharinger, D. & Stark, K. (1990). A qualitative versus quantitative approach to evaluating a Draw–A-Person and Kinetic Family Drawing: Study of mood- and anxiety-disordered children. Psychological Assessment: Journal of Consulting and Clinical Psychology, 2, 365–375.

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Vass, Z. (1998). The inner formal structure of the H-T-P drawings: An exploratory study. Journal of Clinical Psychology, 54, 611–619. West, M. M. (1998). Meta-analysis of studies assessing the efficacy of projective techniques in discriminating child sexual abuse. Child Abuse and Neglect, 22, 1151–1166. Yama, M. (1990). The usefulness of human figure drawings as an index of overall adjustment. Journal of Projective Techniques, 12, 202–215.

HRB ▶ Halstead–Reitan Neuropsychological Test Battery

HRNB ▶ Halstead–Reitan Neuropsychological Test Battery

HRSD ▶ Hamilton Depression Rating Scale

H-T-P Test ▶ House-Tree-Person Test

Human Figure Drawing Tests E LEANOR H OLTZ -E AKIN 1, I DA S UE B ARON 2 1 American University Washington, DC, USA 2 Inova Fairfax Hospital for Children Falls Church, VA, USA

Synonyms Draw-A-Person Test (DAP); HFD

Description The Human Figure Drawing Test has evolved over many years of clinical use. The original Draw-A-Person test

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(DAP) was created by Florence Goodenough and later modified by Dale B. Harris. It remains in use as the prominent human figure drawing measure applicable across the life span. Administration time is about 10–15 min. The subject is provided paper and pencil, and one of a number of test instructions, for example, ‘‘draw a whole figure, not a stick figure,’’ or provided three sheets of paper and asked to ‘‘draw a man, a woman, and yourself.’’ For the latter version, drawings are scored on 73 dimensions that evaluate different figural aspects, including body parts, motor coordination, and face and limb proportion (Harris, 1963). A total composite score is computed for all three drawings (Cosden and Willis, 1992). Other scoring systems are available. For the Draw-A-Person test, the Quantitative Scoring System (DAP: QSS) estimates nonverbal intelligence (Gale Reference Team, 2001). Drawings are scored on 64 items, measuring 14 different aspects, including body parts, clothing, presence or absence of particular detail, and proportion (Gale Reference Team, 2001). There is also a Screening Procedure for Emotional Disturbance (SPED) (Cosden and Morrison, 1995). Eight dimensions of each of the three drawings are examined and compared to normative data appropriate for the subject’s chronological age. In addition, 47 items of each drawing are scored, as for the QSS (Gale Reference Team, 2001). The most recent version is the Draw-A-Person Intellectual Ability Test for children, adolescents, and adults, in which the subject draws himself/herself in the frontal view. There is no time limit, and usually takes 8–15 min to complete. There are 23 scoring elements; 1–4 points are assigned according to feature, for a maximum of 49 points (Hiltonsmith and Sandoval, 2007).

Historical Background The Human Figure Drawing Test has a long history. Florence L. Goodenough developed the Draw-a-Man test for preschoolers and older children in 1926. The production was scored according to strict criteria that dictated a score of 1 or 0 for body parts Goodenough used it to study the intellectual status of young children (Goodenough, 1926). Dale B. Harris later modified the test in 1963 by extending it to older groups and creating an alternate form known as the Draw-A-Person test. The DAP required the individual to draw a man, draw a woman, and draw himself/herself. Harris re-standardized the point system created by Goodenough on representative samples and found that the DAP assessed intellectual maturity for children aged 4–14 years, which Harris

defined as ‘‘the ability to form concepts of increasingly abstract character’’ (Harris, 1963). Karen Machover described the use of the human figure drawing to elicit possible personality issues in Personality Projection in the Drawing of the Human Figure (Machover, 1949). Patients had to draw a man and a woman, and the drawings were analyzed for size, content, and comparison between the two figures, as well as any inconsistencies in style of drawing or omission of specific parts. Machover used these inconsistencies to determine areas of the body about which the patient might have conflicted feelings (Weiss, 1950). The Goodenough–Harris DAP test remains in use. The most recent addition to the DAP test history is the creation of the Draw-A-Person Intellectual Ability Test for Children, Adolescents, and Adults (DAP:IQ). Published in 2004 by Cecil Reynolds and Julia Hickman, this test is normed for subjects 4–89 years of age. The DAP:IQ is the first to extend use beyond children and adolescents to adults, and it uses the human figure drawing to derive measures of intellectual ability through an objective scoring system. It requires only that the subject draw himself/ herself from the frontal view (Hiltonsmith and Sandoval, 2007).

Psychometric Data For the QSS, internal consistency coefficients for the three individual drawings range from 0.56 to 0.78, and for the composite score from 0.83 to 0.89. The interrater reliabilities for both the individual tests and the composite score ranged from 0.86 to 0.95. Mean test–retest reliabilities obtained for 112 students over 4 weeks were 0.74 for composite score, 0.70 for the man, 0.65 for the woman, and 0.58 for the self-drawing. The validity of the DAP:QSS as a measure of nonverbal intelligence is low. A statistically significant but weak correlation between the DAP and the Matrix Analogies Test-Short Form (a test measuring similar intellectual functioning) ranged from 0.17 to 0.31 (Cosden and Willis, 1992). For the SPED, interrater reliability ranged from 0.8 to 0.9. Test–retest reliability over 1 week was 0.67, suggesting DAP:SPED scores were relatively stable over time. Validity of the DAP:SPED was examined by comparing children with documented emotional problems to children without such problems. The former scored higher on the DAP: SPED than those without. Notably, this test is less valid than a measure of intellectual ability (Cosden and Morrison, 1995).

Huntington’s Disease

For the DAP:IQ, median internal consistency using coefficient alpha was 0.82, and ranged from 0.74 to 0.87, test–retest reliability over a 1-week period was 0.84, and interrater reliability from two separate studies was 0.91 and 0.95. Construct validity between the DAP:IQ and the Detroit Tests of Learning Aptitude-Primary: Second Edition (DTLA-P:2) was examined in a sample of 1,233 children, and between the DAP:IQ and the Wechsler Intelligence Scale for Children-III (WISC-III) for 211 children. Higher correlations were found between the DAP: IQ and nonverbal sections of each intelligence test, but a range of 0.40–0.60 correlation was found overall. Validity of the DAP:IQ as a test of academic achievement was also assessed. Correlations between the DAP:IQ and the Woodcock–Johnson-Revised Tests of Achievement and the Wechsler Individual Achievement Test were only moderate (Hiltonsmith and Sandoval, 2007).

Clinical Uses Human figure drawing use in clinical settings varies widely. It is a simple to administer, brief screening test that is often appealing as a first test in clinical evaluations of young children. As an indirect measure, it is also useful when a child cannot easily express concerns verbally. It has not been validated with respect to brain function and therefore remains merely an interesting adjunct measure for neuropsychologists. However, it can reveal areas of emotional significance and there are features that tend to appear more frequently in the drawings of children with long-term neurological disease, such as asymmetries, figural imbalance, dehumanized features, and paucity of detail. These do not confirm neurological impairment but, when present, do emphasize the need for further examination.

Cross References

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eleventh mental measurements yearbook (pp. 287–290). Lincoln, NE: Buros Institute of Mental Measurements. Gale Reference Team (2001). Draw-A-Person Test. In Gale encyclopedia of psychology. Retrieved September 22, 2008, from Gale Group database Goodenough, F. L. (1926). Measurement of intelligence by drawings. Chicago, IL: World Book Company. Harris, D. B. (1963). Children’s drawings as measures of intellectual maturity. New York, NY: Harcourt, Brace & World, Inc. Hiltonsmith, R. W., Sandoval, J. (2007). Review of the Draw-A-Person intellectual ability test for children, adolescents, and adults. In K. F. Geisinger, R. A. Spies, J. F. Carlson, & B. S. Plake (Eds.), The seventeenth mental measurements yearbook (pp. 282–286). Lincoln, NE: Buros Institute of Mental Measurements. Machover, K. (1949). Personality projection in the drawing of the human figure: a method of personality investigation. Springfield, IL: Charles C. Thomas. Weiss, J. (1950). Personality projection in the drawing of the human figure: by Karen Machover, Ph.D. Springfield, Illinois: Charles C. Thomas, 1949. 181 pp. Psychoanalytic Quarterly, 19, 122–123.

Human Prion Disease ▶ Kuru

Huntington’s Chorea ▶ Huntington’s Disease

Huntington’s Disease J ENNIFER C. G IDLEY L ARSON , YANA S UCHY University of Utah Salt Lake City, UT, USA

Synonyms

▶ Projective Tests ▶ Projective Technique

Huntington’s chorea


Definition

Cosden, M., Morrison, G. M. (1995). Review of the Draw A Person: screening procedure for emotional disturbance. In J. C. Conoley & J. C. Impara (Eds.), The twelfth mental measurements yearbook (pp. 320–323). Lincoln, NE: Buros Institute of Mental Measurements. Cosden, M., Willis, W. G. (1992). Review of the Draw A Person quantitative scoring system. In J. J. Kramer & J. C. Conoley (Eds.), The

Huntington’s disease (HD) is a fatal, autosomal dominant, neurodegenerative disease characterized by movement disorder (generally a choreiform movement disorder, marked by involuntary continuous fluid or jerky ‘‘dance-like’’ movements), psychiatric disturbance, and cognitive decline.

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Historical Background Observations of dance-like movements (i.e., chorea) have been documented since the mid-fourteenth century. In 1500, it was suggested that chorea evolves from the central nervous system; in 1686, post-infectious chorea was described; and in 1832, an inherited form of chorea was identified (Walker, 2007). In 1872, George Huntington (1850–1916) published his seminal paper titled On Chorea, in which he provided a detailed description of the inherited form of chorea that manifests most often in adults and is marked by both psychiatric and cognitive impairment. Using the reports of his father and grandfather, both family practitioners, Huntington was able to describe not only the manifestations of the disease at one point in time, but also its course over time. The disorder that he so vividly described now bears his eponym – Huntington’s disease. In 1983, a genetic analysis of two families with HD led to the localization of the HD gene to chromosome 4p16.3. Subsequently, in 1993, the Huntington’s Disease Collaborative Research Group isolated the human huntingtin protein, which is the defective or mutated gene underlying HD.

35 or fewer CAG repeats will not develop the disorder. However, there are some inconsistencies as to the number of repeats needed to be considered fully penetrated by HD, ranging from 36 to 41 repeats.

Age of Onset An individual who inherits the HD gene and lives a normal lifespan will inevitably develop HD symptoms. While somewhat variable, the typical age of onset for HD is between 30 and 50 years of age. However, cases with onset as young as 2 years and as old as 80 years have also been reported. Age of onset has been highly correlated with the number of CAG repeats, with higher numbers of repeats associated with earlier onset. Individuals who develop symptoms prior to 20 years of age are diagnosed with juvenile HD. Juvenile HD is often associated with paternal gene transmission and the number of CAG repeats usually exceeds 55. Individuals developing symptoms after 50 years of age have late-onset HD. Those with juvenile or late-onset HD tend to have greater disease severity than those with midlife onset; however, it remains unclear as to whether individuals with juvenile or lateonset HD have more rapid deterioration and disease progression than those with midlife onset.

Current Knowledge Prevalence In most Caucasian populations, HD has been found to affect 5–10 individuals per 100,000. HD is much less common in Japan, occurring in approximately 0.5 individuals per 100,000, and even less prevalent in other Asian and African countries. The literature suggests that the increase in HD incidence in Caucasian populations may be due to a higher frequency of mutation on huntingtin alleles (described below). HD affects both men and women equally (Walker, 2007).

Pathogenesis HD is an autosomal dominant disease. The defective or mutated gene underlying HD is the human huntingtin protein, also known as the IT15 gene and is located on the short-arm of chromosome 4p16.3. The huntingtin protein is associated with excessive CAG trinucleotide (polyglutamine) repeat. Specifically, individuals whose alleles at this location contain 36 or more CAG repeats will inevitably develop HD, while those whose alleles contain

Neuropathology HD is currently classified as a subcortical dementia syndrome due to the extensive degeneration of subcortical tissue within the extrapyramidal motor system, predominantly the striatum. The earliest neurodegenerative signs of HD begin taking place as early as 10–20 years prior to HD diagnosis, specifically bilateral atrophy of the medium spiny GABAergic neurons within the caudate nucleus and putamen. As the disease progresses, neuronal atrophy can further be seen in the globus pallidus, substantia nigra, thalamus, hippocampus, cerebellum (particularly loss of Purkinje cells), cerebral cortex (frontal lobes and in layers III, V, and VI), brainstem, and spinal cord (Montoya, Price, Menear, & Lepage, 2006; Walker, 2007).

Clinical Features and Disease Progression With the localization of the HD gene and the advancement of genetic testing, individuals with an affected parent can be tested for the HD gene prior to the onset of symptoms. Individuals who have not yet been diagnosed


with HD but who are found to be carriers of the gene are referred to as presymptomatic or preclinical gene carriers (PSGC). Despite their ‘‘presymptomatic’’ status, PSGC individuals tend to exhibit evidence of neuroanatomical degeneration as well as subtle motor, cognitive, and psychiatric signs, as early as 10–20 years prior to diagnosis. Generally, such preclinical symptoms are not noticed or are denied by the patient. As the disease progresses, presymptomatic features begin to worsen in both severity and frequency. HD is most often, but not always, diagnosed by unequivocal movement disorder and the presence or report of HD in a family member. Following diagnosis of HD, the disease tends to progress steadily, with death occurring approximately 10–15 years after diagnosis. The clinical features of HD have been divided into behavioral, neuropsychiatric, and neuropsychological.

Behavioral Features It is posited that early atrophy of the caudate and putamen nuclei, particularly the neurons in the indirect pathway (i.e., the cortical input through the basal ganglia via subthalamic nucleus), affects cognitive and motor functions by interfering with the fronto-striatal-thalamic circuitry. In many cases, PSGCs tend to demonstrate subtle abnormalities in body movements, such as restlessness, choreiform movements, and impaired motor planning, as well as in eye movements, such as slowed saccades and impaired smooth pursuit (Blumenfeld, 2002). The initial motor symptoms eventually give way to involuntary jerking movements (i.e., chorea), poor motor coordination, motor impersistence, and slowed occulomotor responses. As the disease progresses, other abnormal movements appear, including tics, dystonic posturing, and athetosis. During the late stages of the disease, there is drastic decline in the ability to make purposeful movements. Additionally, dysarthria and dysphagia are present, as well as incontinence and weight loss. During the final stages of the disease, there is a decrease in choreiform movements as individuals become akinetic, mute, and fully dependent on others. If the disease runs its course, respiratory infections are the primary cause of death in HD patients.

Neuropsychiatric Features The neuropsychiatric symptoms and disorders associated with HD are consistent with the neuropathology of the

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disease, particularly the frontal-striatal atrophy. Typical symptoms include, but are not limited to, elevations in anxiety, depression, dysphoria, obsessive-compulsiveness, hostility, agitation, irritability, apathy, disinhibited aggression, and interpersonal sensitivity. Although psychiatric symptoms may exist as early as 10 years prior to diagnosis, they tend to increase considerably as PSGCs near the diagnosis of HD. Rarely, psychosis, hallucinations, and delusions may present within the early stages of the disorder. Finally, patients prior to and just following the diagnosis of HD tend to have approximately four times greater suicide rate than the general population (Walker, 2007).

Neuropsychological Features The pattern of cognitive decline in individuals diagnosed with HD is reflective of the progression of neurodegeneration, with striatal atrophy occurring first, followed by slow atrophy of the projections from the striatum to the cortex, particularly frontal-striatal circuitry. Cognitive decline begins early in the disease, possibly preceding motor symptoms, and develops into a subcortical dementia characterized by slowed processing, apathy, lack of motivation, and eventually global impairment in cognitive functioning. Decline in psychomotor speed is evident in PSGC and worsens over the course of the disease. Psychomotor slowing likely contributes to the decline in performance on measures of fluid abilities, particularly those dependent upon psychomotor speed and reasoning (i.e., block design, matrices, digit-symbol coding, and symbol search) as well as executive abilities (e.g., fluency tasks). However, it should be noted that after controlling for motor impairment and psychomotor speed, individuals with HD still show a steady decline in executive functions, most notably in tasks that assess planning, switching, mental flexibility, and adherence to rules (i.e., Trail Making Test Part B, Tower of Hanoi/London, Wisconsin Card Sorting Test). Further, psychomotor slowness, particularly slowed eye movements, may underlie impairments in visual scanning and in visuospatial abilities (for review see Lezak, Howieson, & Loring, 2004; Montoya et al., 2006). Attention and memory problems are also evident in PSGCs and worsen over the course of the disease. As the disease progresses, attentional span (spatial span and digit span) becomes shorter and memory problems become more pervasive. Memory decline is most prominent with respect to verbal and visual memory and learning, as well as implicit or procedural memory.

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Finally, evidence suggests that individuals with HD, as well as PSGCs, exhibit an impaired ability to identify negative emotions such as anger, fear, sadness, and disgust.

Differential Diagnosis There are several disorders that present somewhat similarly to HD, including Huntington’s disease-like 2, Neuroacanthocytosis, tardive dyskinesia, chorea gravidarum, unilateral post-infectious (Sydenham’s) chorea, hyperthyroid chorea, vascular hemichorea, and chorea. The differential diagnosis of these disorders would not be made by a neuropsychologist.

Treatment The treatment options are currently very limited and vary during the different stages of the disease. Antichoreic or neuroleptic medications are often prescribed to relieve choreic movement, although parkinsonian-like side effects can occur (i.e., rigidity, bradykinesia, and depression). Tetrabenazine (Xenazine®) is the first FDA approved medication specifically for treating HD motor symptoms. The neuropsychiatric symptoms are generally treated in the same manner that they are treated in nonHD populations. Unfortunately, there are currently no treatments to slow the cognitive decline or dementia exhibited in HD. Support groups and psychotherapy for the PSGCs, the affected individual, as well as their family, can help patients and families cope with the difficulties associated with HD.

Cross References ▶ Chorea ▶ Movement Disorders ▶ Parkinson’s Disease ▶ Subcortical Dementia

References and Readings Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sutherland, MA: Sinauer Associates. Lezak, M., Howieson, D., & Loring, D. (2004). Neuropsychological assessment. New York: Oxford. Montoya, A., Price, B., Menear, M., & Lepage, M. (2006). Brain imaging and cognitive dysfunctions in Huntington’s disease. Journal of Psychiatry & Neuroscience, 31(1), 21–29. NINDS. (2008). Huntington’s disease: Hope through research. Retrieved April 15, 2008, from http://www.ninds.nih.gov/disorders/huntington/ detail_huntington.htm Walker, F. (2007). Huntington’s disease. Lancet, 369, 218–228.

HVLT-R ▶ Hopkins Verbal Learning Test

HVOT ▶ Hooper Visual Organization Test

Future Directions Current research in HD is primarily focusing on treatment, early identification, and examination of neuroprotective agents. Several clinical trials for medications shown to slow the progression of HD in mice are currently underway and appear to be promising. Additionally, while controversial, clinical trials are underway examining the effect of transplanting fetal neurons into the striatum of HD patients. Further, pathological biomarkers are currently in the process of being identified in PSGCs. The identification of biomarkers could facilitate early diagnosis leading to early intervention and treatment. Lastly, research is being conducted on PSGC in order to find neuroprotective agents and therapies that may prevent or delay the onset of full HD symptoms.

Hydrocephalus G ARY T YE 1, J OHN B ROWN 2 1 Virginia Commonwealth University Richmond, VA, USA 2 Medical College of Georgia Augusta, GA, USA

Definition Hydrocephalus is a condition resulting from inadequate drainage or absorption of cerebrospinal fluid (CSF) from the brain.

Hydrocephalus

Current Knowledge Introduction Hydrocephalus is a common pediatric disorder, resulting from inadequate drainage or absorption of CSF. This is most commonly due to obstruction to flow, although, overproduction of CSF secondary to tumor formation may be a rarely occurring cause (Rekate, 2008). The excess of fluid may lead to dilation of the ventricles (Fig. 1) and subsequent elevation of ICP, which can further cause damage to surrounding neural tissue resulting in neurologic deficits such as ataxia, impaired cognitive function, and endocrine disorders (Del Bigio, 2001; Kaiser, Ruedeberg, & Arnold, 1989; Sorensen, Jansen, & Gjerris, 1986).

Etiology and Epidemiology Obstructive hydrocephalus can be secondary to intraventricular hemorrhage, brain tumor, meningitis, or congenital malformations such as myelomeningocele or aqueductal stenosis (Anderson, Garton, & Kestle, 2008).

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nausea and vomiting, headache, irritability, lethargy, diplopia, and seizures (Anderson et al., 2008). Older children may also complain of urinary incontinence. Physical exam may demonstrate increased head circumference with or without a bulging fontanelle. Additional findings may include papilledema, cranial nerve palsies, hemiparesis, and developmental delay. Radiographic evidence of ventricular enlargement is typically present.

Treatment The mainstay of treatment for hydrocephalus is by surgical correction of CSF outflow either by placement of a ventriculoperitoneal or ventriculoatrial shunt or by performing a third ventriculostomy (Del Bigio, 2001). Shunting can be successful at relieving ventricular dilation (Fig. 2) and elevated ICP, but may be associated with a high incidence of malfunctioning causing re-obstruction and infection (Epstein, 1985). Rates of infection range from 7 to 9% (Enger, Svendsen, & Wester, 2003). Approximately 81% of children receiving shunts will have one or more complications requiring inpatient therapy (Sainte-Rose et al., 1991).

Cognitive Aspects Presentation Children with hydrocephalus can present with a wide range of signs and symptoms. Common complaints include

There are multiple studies examining the effects on the cognitive aspects of hydrocephalus. Dalen et al., (2006) looked at the incidence of nonverbal learning on children

Hydrocephalus. Figure 1 Axial head CT showing severe hydrocephalus

Hydrocephalus. Figure 2 Axial head CT with shunt in place showing decompression of the venricles

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Hydrocephalus. Figure 3 Saggital T2 weighted MRI Showing flow void through patent third ventriculostomy

Hydrocephalus. Figure 5 Head CT showing decompressed ventricles with two shunt catheters in place

normal schools was possible for almost 60% of children with hydrocephalus, including one child who entered medical school. Kojima (1988) found a significant difference in intelligence in the presence of shunt infections, especially if suffered in the first 2 months of life.

Cross References ▶ Subcortical Dementia ▶ Ventricles ▶ Ventricular Enlargement ▶ Ventriculostomy

References and Readings Hydrocephalus. Figure 4 Head CT showing untreated hydrocephalus

with hydrocephalus and found that the incidence of nonverbal learning problems was significantly higher in children with infantile hydrocephalus. Lindquist et al. (2005), in a study of 103 children born with hydrocephalus, found that one-third of the children had an IQ > 85, another one-third had a low to average IQ of 70–84, and yet another one-third had learning disabilities with an IQ < 70. The median IQ was found to be 75. Topczewska-Lach et al. (2005) found that integration into

Anderson, C. E., Garton, J. L., & Kestle, J. R. W. (2008). Treatment of hydrocephalus with shunts. In: A. L. Albright, I. F. Pollack, & P. D. Adelson (Eds.), Principles and practice of pediatric neurosurgery (2nd ed., pp. 109–144). New York: Thieme. Dalen, K., et al. (2006). Non-verbal learning disabilities in children with infantile hydrocephalus, aged 4–7 years: A population-based, controlled study. Neuropediatrics, 37, 1–5. Del Bigio, M. R. (2001). Future directions for therapy of childhood hydrocephalus: A view from the laboratory. Pediatric Neurosurgery, 34, 172–181. Enger, P. O., Svendsen, F., & Wester, K. (2003). CSF shunt infections in children: Experiences from a population-based study. Acta Neurochirurgica (Wien), 145, 243–248; discussion 248. Epstein, F. (1985). How to keep shunts functioning, or ‘‘the impossible dream.’’ Clinical Neurosurgery, 32, 608–631.

Hygroma Kaiser, G., Ruedeberg, A., & Arnold, M. (1989). Endocrinological disorders in shunted hydrocephalus. Zeitschrift Fur Kinderchirurgie Und Grenzgebiete, 44, (Suppl. 1), 16–17. Kojima, N., et al. (1988). Evaluation of shunt treatment in hydrocephalus with myelomeningocele: Some factors relating to mental prognosis. No To Shinkei, 40, 1181–1187. Lindquist, B., et al. (2005). Learning disabilities in a population-based group of children with hydrocephalus. Acta Paediatrica, 94, 878–883. Rekate, H. L. (2008). Treatment of Hydrocephalus. In A. L. Albright, I. F. Pollack, & P. D. Adelson (Eds.), Principles and practice of pediatric neurosurgery (2nd ed., pp. 94–108). New York: Thieme. Sainte-Rose, C., Piatt, J. H., & Renier, D., et al. (1991). Mechanical complications in shunts. Pediatric Neurosurgery, 17, 2–9. Sorensen, P. S., Jansen, E. C., & Gjerris, F. (1986). Motor disturbances in normal-pressure hydrocephalus. Special reference to stance and gait. Archives of Neurology, 43, 34 –38. Topczewska-Lach, E., et al. (2005). Quality of life and psychomotor development after surgical treatment of hydrocephalus. European Journal of Pediatric Surgery, 15, 2–5.

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surgery, anxious withdrawal from medication, delirium tremens, and nausea/vomiting.

Off Label Use Insomnia.

Side Effects Serious Convulsions and cardiac arrest (both rare), bronchodilation, and respirator depression.

Common

Hydroxyzine J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name

Dry mouth, sedation, and tremor.


Hydroxyzine

Additional Information Brand Name Atarax, Vistaril

Class


Antihistamine

Hygroma Proposed Mechanism(s) of Action Blocks histamine type I receptors.

J ENNIFER T INKER Drexel University Philadelphia, PA, USA

Indication Synonyms Anxiety, anxiety produced by organic states, pruritus (itching), premedication sedation, sedation following

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Hyperbaric Oxygen Therapy (HBOT)

Hyperbaric Therapy B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms Hyperbaric oxygen therapy (HBOT)

Definition

Hygroma. Figure 1 Subdural hygroma

Definition A subdural hygroma is characterized by an accumulation of cerebrospinal fluid (lacking blood) within a separation of the dura-arachnoid space. A hygroma can develop following traumatic head injury, meningitis, ventriculoperitoneal shunting (secondary to the sudden decrease in intracranial pressure), or infrequently, as the result of a tearing of an arachnoid cyst. Generally, hygroma can be differentiated from hematoma using CT scan although chronic subdural hematomas may more closely resemble hygroma via CT, in which case MRI scans are utilized. Clinically, hygromas produce similar symptoms as hematomas. These include drowsiness, confusion, irritability, and low-grade fever, which dissipate when the CSF is drained (Victor & Ropper, 2001).

References and Readings Victor, M., & Ropper, A. H. (2001). Adam’s and Victor’s principles of neurology: Seventh edition. New York: McGraw-Hill.

Hyperbaric Oxygen Therapy (HBOT) ▶ Hyperbaric Therapy

Hyperbaric therapy is a treatment using 100% oxygen at greater than normal atmospheric pressure. Once the hemoglobin is saturated with oxygen, the blood becomes hyperoxygenated by dissolving oxygen within the plasma, body tissues, and fluids up to 10 times the normal concentration. Since the oxygen is in solution, it can reach areas where red blood cells may not be able to pass and oxygenate areas of impaired hemoglobin concentration or function. Although still controversial, hyperbaric therapy is used to treat atherosclerosis, stroke, peripheral vascular disease, diabetic ulcers, wound healing, cerebral palsy, brain injury, multiple sclerosis, macular degeneration, and so on.

Cross References ▶ Anoxia

References and Readings Allen, K., Danforth, J., & Drabman, R. (1989). Videotaped modeling and film distraction for fear reduction in adults undergoing hyperbaric oxygen therapy. Journal of Consulting and Clinical Psychology, 57(4), 554–558. Golden, Z., Golden, C., & Neubauer, R. (2006). Improving neuropsychological function after chronic brain injury with hyperbaric oxygen. Disability & Rehabilitation, 28(22), 1379–1386. Messier, L., & Myers, R. (1991). A neuropsychological screening battery for emergency assessment of carbon-monoxide-poisoned patients. Journal of Clinical Psychology, 47(5), 675–684. Stoller, K., Shaughnessy, M., & Greathouse, D. (2005). An interview with Kenneth Stoller about hyperbaric medicine and brain injury trauma. North American Journal of Psychology, 7(2), 217–227.

Hyperlexia

Hyperesthesia K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Definition

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Robinson, M. D., & Shannon, S. (2002). Rehabilitation of peripheral nerve injuries. Physical Medicine and Rehabilitation Clinics of North America, 13(1), 109–135. Review. PubMed PMID: 11878078.

Hyperkinetic Disorder ▶ Attention Deficit, Hyperactivity Disorder

Hyperesthesia refers to increased sensitivity to sensory stimulation, such as touch or temperature. The term derives from the Greek hyper (over) and aisthesis (feeling) and is usually used in relation to cutaneous sensation. It is most commonly expressed in terms of increased pain sensitivity.

S TEPHEN M. K ANNE , M ICAH O. M AZUREK University of Missouri Columbia, MO, USA

Current Knowledge

Description and Definition

The most common cause of hyperesthesia is peripheral neuropathy, such as might occur with diabetes or chronic alcohol abuse. Frequently described as a chronic burning type pain, it can also manifest as increased sensitivity to touch. In extreme cases, patients may not even be able to tolerate any type of covering over their feet while in bed. Thalamic lesions, particularly those involving the posterior thalamus, can also be associated with increased pain syndromes. Such lesions may initially produce a loss or decrease in sensation on the contralateral side of the body, but after a period of time the patient may begin experiencing distressing pain on the side contralateral to the lesion. Such pain generally takes on more of the characteristics of the kind of pain mediated by the paleospinothalamic system (i. e., poorly localized, aching or burning type of pain). It can often be set off by minimal stimuli and will tend to persist and/or expand well beyond the time and space parameters of the eliciting stimulus. It should also be recalled that pain is a subjective symptom and can be influenced by one’s mental or emotional state, such as depression.

Hyperlexia is generally characterized by the spontaneous and precocious development of single-word reading that is more advanced than both reading comprehension skills and general cognitive ability. In addition to these defining characteristics, children with hyperlexia often have accompanying difficulties with social development, obsessive preoccupations with reading, and delays in language and cognitive development. Furthermore, advanced singleword reading in hyperlexia occurs very early, often by the age of 3, and without formal reading instruction. Research into hyperlexia has been hampered by both relatively small sample sizes and considerable disagreement with regard to characteristics that define the hyperlexic reading profile. Some propose that the condition should be defined by a significant discrepancy between single-word reading and general cognitive level. Others argue that the defining discrepancy should be between single-word reading and reading comprehension. A third group suggests that there should be both higher than expected single-word reading (based on developmental expectations) and lower than expected reading comprehension (based on word recognition abilities) (see Grigorenko, Klin, & Volkmar, 2003 for review). Clearly, the lack of a consistent operational definition has resulted in inconclusive evidence regarding neuropsychological processes in hyperlexia.

Cross References ▶ Paresthesia

References and Readings Peyron, R., Laurent, B., & Garcıá-Larrea, L. (2000). Functional imaging of brain responses to pain. A review and meta-analysis. Neurophysiologie Clinique, 30(5), 263–288. Review. PubMed PMID: 11126640.

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Categorization There has also been disagreement with regard to classification and related characteristics of hyperlexia. The first

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such question that has been the topic of some debate relates to whether hyperlexia should be considered a distinct syndrome (as argued by Healy, 1982), or whether it should be considered to be a behavioral characteristic (as argued by Nation, 1999). Some researchers view hyperlexia as a variant of typical reading development in which children exhibit precocious reading of single words, while lacking accompanying comprehension. From within this view, hyperlexic reading can occur among children with a wide range of cognitive and verbal abilities (e.g., Pennington et al., 1987). Others have viewed hyperlexia as a subtype of different developmental and learning difficulties, including dyslexia, specific language impairment, and reading comprehension disorder. Research has not supported the classification of hyperlexia as a subtype of dyslexia. However, the relationship between hyperlexia and language impairment, particularly semantic/pragmatic difficulties, has received some support. Furthermore, the essential features of reading comprehension disorder (e.g., a discrepancy between word recognition and reading comprehension) are identical to the pattern of reading skills found in hyperlexia (see Nation, 1999). Despite these areas of overlap, these categorizations alone fail to capture the accompanying social and cognitive deficits that have long been associated with hyperlexia. Thus, the third view (e.g., Grigorenko et al., 2003) posits that hyperlexia represents a subset of children with autism spectrum disorder (ASD) or pervasive developmental disorder (PDD) diagnoses. Indeed, there is considerable evidence to suggest that children with hyperlexia in early studies also exhibited many characteristics of autism, even when not formally diagnosed (e.g., social impairment, repetitive behaviors, intense preoccupations). In fact, the intense preoccupation with reading that is seen in hyperlexia mirrors circumscribed and repetitive interests that are characteristics of autism. Furthermore, the verbal (and reading) comprehension difficulties in hyperlexia also mirror those seen in autism.

Epidemiology There have been relatively few studies of children with hyperlexia, the majority of which have included either single cases or small clinical samples. As a result, incidence and prevalence rates among the general population are not available. The prevalence rate of hyperlexia among individuals with PDD has been estimated to be between 5% and 10% (Burd & Kerbeshian, 1985).

Natural History, Prognostic Factors, and Outcomes The developmental course of hyperlexia is characterized by the early emergence of reading skills well before formal education. Studies have documented the emergence of advanced single-word reading by age 3 in many cases, and prior to age 5 in most cases. Single-word reading remains advanced throughout early childhood with respect to developmental expectations; however reading comprehension remains lower than expected (based on either developmental level or word recognition abilities, depending on the operational definition used). Over time, word recognition abilities appear to get stabilized such that by age 10, most children with hyperlexia exhibit single-word reading at age-expected levels. In contrast, reading comprehension remains impaired (see Newman, Macomber, Naples, Babitz, Volkmar, & Griogorenko, 2007). In terms of prognosis, some early studies suggested that hyperlexia was associated with better outcomes for children with PDD. However, other studies have found no outcome differences between groups.

Neuropsychology and Psychology of Hyperlexia Given the unusual pattern of reading without comprehension in hyperlexia, researchers have been interested in the neuropsychological processes that underlie it. There is consistent evidence that children with hyperlexia typically show severe language deficits, including delayed speech acquisition, echolalia, pragmatic problems, and semantic difficulties. In terms of cognitive ability, a wide range of IQ scores have been found. Most studies cite relative weaknesses in verbal comprehension and abstract reasoning, with relative strengths in visual discrimination and in rote memorization. Also of interest is the process by which children with hyperlexia learn to read. Despite the unusual quality of their reading development, researchers have found that children with hyperlexia appear to employ the same strategies as typical readers. Specifically, they appear to use both phonological and orthographic strategies in reading single words, with no apparent overreliance on either strategy. Some have argued (e.g., Nation, 1999; Newman et al., 2007) that the precocious development of singleword reading in hyperlexia may be the result of obsessive preoccupation with reading and the alphabet. Given that children with hyperlexia typically spend the majority of their time in reading or related activities (to the exclusion

Hypersexuality/Hyposexuality

of play or social activities), they accrue many hours of intense practice with the printed word. This may allow them to develop single-word recognition much earlier than their peers, while their weaknesses in verbal comprehension may prevent an understanding of the words they are reading. Thus, the preoccupation with letters and words combined with semantic weaknesses foster the development of advanced word calling skills (the mechanics of reading), without the accompanying comprehension of their meaning.

Evaluation The evaluation of hyperlexia depends largely on the operational definition used (see above). Regardless of the definition used, however, word recognition skills, reading comprehension skills, and general cognitive ability will need to be assessed. Common instruments assessing single-word reading and reading comprehension include the Woodcock-Johnson Tests of Achievement (Letter-Word Identification and Passage Comprehension subtests) and the Wechsler Individual Achievement Test (Word Reading and Reading Comprehension subtests). Cognitive ability may be assessed by any number of IQ tests designed for children (e.g., Wechsler Intelligence Scale for Children/▶ Wechsler Preschool and Primary Scale of Intelligence, ▶ Stanford–Binet Intelligence Scales, ▶ Kaufman Assessment Battery for Children, ▶ Differential Ability Scales).

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Burd, L., Kerbeshian, J., & Fisher, W. (1985). Inquiry into the incidence of hyperlexia in a statewide population of children with pervasive developmental disorder. Psychological Reports, 57(1), 236–238. Grigorenko, E. L., Klin, A., & Volkmar, F. (2003). Annotation: Hyperlexia: Disability or superability? Journal of Child Psychology and Psychiatry, 44, 1079–1091. Healy, J. M. (1982). The enigma of hypertexia. Reading Research Quarterly, 17(3), 319–338. Nation, K. (1999). Reading skills in hyperlexia: A developmental perspective. Psychological Bulletin, 125, 338–355. Newman, T. M., Macomber, D., Naples, A. J., Babitz, T., Volkmar, F., & Griogorenko, E. L. (2007). Hyperlexia in children with autism spectrum disorders. Journal of Autism and Developmental Disabilities, 37, 760–774. Pennington, B. F., Johnson, C., & Welsh, M. C. (1987). Unexpected reading precocity in a normal preschooler: Implications for hyperlexia. Brain and Language, 30(1), 165–180.

Hyperphenylalaninemia ▶ Phenylketonuria

Hyperphosphorylated Tau ▶ Neurofibrillary Tangles

Hyperserotonemia ▶ Serotonin Syndrome

Treatment Not applicable.

Cross References ▶ Asperger’s Disorder ▶ Autistic Disorder ▶ Pervasive Developmental Disorder NOS ▶ Reading ▶ Reading Comprehension ▶ Semantic Pragmatic Disorder

References and Readings Aram, D. M., & Healy, J. M. (1988). Hyperlexia: A review of extraordinary word recognition. In L. Ober & D. Fein (Eds.), The exceptional brain: Neuropsychology of talent and special abilities (pp. 10–102). New York, NY: Guilford Press.

Hypersexuality/Hyposexuality R OBERT F RANK Kent State University Kent, OH, USA

Definition Hypersexuality and hyposexuality refer to levels of sexual interest and/or activity that are unusually high or low, respectively. Marked changes in the sexual behavior may reflect biological or psychological conditions. Hypersexuality or hyposexuality may reflect changes in acute or long-term endocrine and neurological or psychological functioning. The behavioral disinhibition often seen after traumatic brain injury may take the form of sexual ‘‘acting out,’’ but diminished libido occurs frequently as well.

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Cross References


▶ Kluver Bucy Syndrome ▶ Sexual Disinhibition

American Academy of Sleep Medicine. (2001). International classification of sleep disorders, revised: Diagnostic and coding manual. Chicago, IL: American Academy of Sleep Medicine.

References and Readings Gorman, D. G., & Cummings, J. L. (1992). Hypersexuality following septal injury. Archieves of Neurology, 49(3), 308–310. Miller, B. L., Cummings, J. L., McIntyre, H., Ebber, G., & Grode, M. (1986). Hypersexuality or altered sexual preference following brain injury. Journal of Neurology, Neurosurgery, and Psychiatry, 49(8), 867–873. Zasler, N., & Martelli, M. (2005). Sexual dysfunction. In J. Silver, T. McAllister, & S. Yudovsky (Eds.), Textbook of traumatic brain injury. Arlington, VA: American Psychiatric.

Hypertension E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms High blood pressure

Hypersomnia

Definition

D ONA EC LOCKE Mayo Clinic Scottsdale, AZ, USA

Hypertension (or ‘‘high blood pressure’’) is a chronic medical condition in which the systemic arterial blood pressure is elevated.

Synonyms

Current Knowledge

Excessive sleepiness

Hypersomnia or excessive sleepiness is a condition in which a person has trouble staying awake during the day. There are many potential causes of hypersomnia including sleep disorders, sleep deprivation, obesity, drug or alcohol abuse, head injury or neurological disease, prescription medications, and genetics. According to the International Classification of Sleep Disorders, Revised Diagnostic and Coding Manual (ICSD-R), hypersomnia is a symptom of multiple sleep disorders. In addition to being a symptom of many sleep disorders, there are three specific hypersomnia diagnoses included in the ICSD-R: recurrent hypersomnia, idiopathic hypersomnia, and posttraumatic hypersomnia.

More than 90% of hypertension is ‘‘essential’’ or ‘‘primary,’’ with no associated medical cause. ‘‘Secondary hypertension’’ is the result of the presence of other conditions such as kidney disease or certain rare tumors. Persistent hypertension is an important risk factor for ischemic stroke, myocardial infarction, congestive heart failure, chronic renal failure, and premature death. Acute elevations of blood pressure may cause hemorrhagic strokes or other neurologic syndromes. Dietary salt reduction, weight loss, and increased exercise can be effective in reducing elevated blood pressure and maintaining it at normal levels. Treatment often consists of the systematic and consistent use of select medications, of which there are many. Diuretics, vasodilators, beta-blockers, alpha-adrenergic antagonists, calcium channel blockers, angiotensin converting enzyme inhibitors are among these. Regular monitoring of blood pressure is advised in order to insure that it is being maintained at normal levels.

Cross References

Cross References

▶ Insomnia ▶ Sleep Disturbance

▶ Atherosclerosis ▶ Cerebrovascular Disease

Definition

Hypertensive Encephalopathy

▶ Coronary Disease ▶ Hemorrhagic Stroke ▶ Ischemic Stroke

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cerebral autoregulation, causing leakage of fluid into perivascular tissue and resulting in cerebral edema and increased intracranial pressure.

References and Readings Current Knowledge Chobanian, A. V., Bakris, G. L., Black, H. R., Cushman, W. C., Green, L. A., Izzo, J. L., et al. (2003). National High Blood Pressure Education Program Coordinating Committee: Seventh report of the joint national committee on prevention, detection, evaluation, and treatment of high blood pressure. Hypertension, 42, 1206–1252. Rosendorff, C., Black, H. R., Cannon, C. P., Gersh, B. J., Gore, J., Izzo, J. L., et al. (2007). Treatment of hypertension in the prevention and management of ischemic heart disease: A scientific statement from the American Heart Association Council for High Blood Pressure Research and the Councils on Clinical Cardiology and Epidemiology and Prevention. Circulation, 115, 2761–2788.

Hypertensive Crisis ▶ Hypertensive Encephalopathy

Hypertensive Emergency ▶ Hypertensive Encephalopathy

Pathophysiology In normotensive patients, a mean arterial pressure (MAP) above approximately 60 mm Hg triggers autoregulation. Autoregulation refers to the capacity to maintain cerebral blood flow by fluctuations in the vascular tone of the cerebral resistance arteries. Once MAP approaches 160 mm Hg, autoregulatory mechanisms are less able to sustain control over cerebral blood flow. Patients with chronic hypertension may develop encephalopathy at much higher MAP levels as cerebral autoregulatory range adapts to higher pressures over time. Once the autoregulatory capacity is exceeded, the brain becomes hyperperfused, causing leakage into perivascular tissue and resulting in a wide range of cerebral consequences, including cerebral edema, increased intracranial pressure, vasospasm, ischemia, increased vascular permeability, punctuate hemorrhages, petechial hemorrhages, necrotizing arteriolitis, microinfarcts, multiple small thrombi, and damage to small blood vessels.

Prevalence/Demographics

Hypertensive Encephalopathy H OLLY R AU, YANA S UCHY University of Utah Salt Lake City, UT, USA

Synonyms Accelerated hypertension; Hypertensive crisis; Hypertensive emergency; Hypertensive urgency; Malignant hypertension

Prevalence corresponds to the occurrence of hypertension in the general population. Hypertension is seen in men more often than women, and is more prevalent among African-Americans, with young black men being particularly prone to hypertensive crisis. Incidence is lowest in Caucasians. Although hypertensive encephalopathy can occur at any age, most patients are middle-aged with a longstanding history of hypertension. Less than 1% of the 60 million Americans with hypertension will develop a hypertensive emergency, which is the precursor to hypertensive encephalopathy.

Symptoms/Presentation

Definition Hypertensive encephalopathy refers to an abrupt, sustained rise of blood pressure that exceeds the limits of

Onset is typically acute over 24–72 h. Patients may present with headaches, nausea, vomiting, irritability, drowsiness, visual disturbances (e.g., blurriness,

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blindness), seizures, and alterations in consciousness, including confusion, stupor, or coma. On occasion, neurological symptoms such as aphasia or hemiplegia may also be present.

▶ Meningitis ▶ Metabolic Encephalopathy

References and Readings Diagnosis The greatest indicators are encephalopathy accompanied by high blood pressure and damage to optic structures, including papilledema, retinal linear hemorrhages, and hypertensive retinopathy. Cerebral white matter changes evidenced by neuroimaging are required for a diagnosis.

Blumenfeld, J. D., & Laragh, J. H. (2001). Management of hypertensive crisis: The scientific basis for treatment decisions: Review. American Journal of Hypertension, 14(11–1), 1154–1167. Chang, R. C. (2006). Hypertensive Encephalopathy: http://www.emedicine.com/med/byname/encephalopathy-hypertensive.htm Goldman, L., & Ausiello, D. (Eds.). (2007). Cecil medicine (23rd ed.). Philadelphia, PA: Saunders. Libby, P., Bonow, R. O., Mann, D. L., & Zipes, D. P. (Eds.). (2007). Braunwald’s heart disease: A textbook of cardiovascular medicine (8th ed.). Philadelphia, PA: Saunders.

Treatment Efforts at lowering blood pressure are typically managed with oral medications. To prevent cerebral ischemia and watershed infarctions, excessive or overly rapid reduction of blood pressure must be avoided, with the goal being to reduce blood pressure by no more than 25%. The patient’s blood pressure should be monitored in an acute inpatient intensive care unit, with frequent assessments aimed at detecting signs of neurologic deterioration. Behavioral modifications, including weight reduction, increased physical activity, restrained alcohol and sodium intake, and cessation of tobacco products are recommended to prevent future hypertensive emergencies.

Hypertensive Urgency ▶ Hypertensive Encephalopathy

Hypertrophy T HESLEE J OY D E P IERO Boston University School of Medicine Boston, MA, USA

Definition Prognosis While acute and reversible with prompt initiation of therapy, hypertensive encephalopathy can proceed to coma and death within a few hours if untreated.

Cross References ▶ Brain Swelling ▶ Cerebral Edema ▶ Cerebral Perfusion Pressure ▶ Coma ▶ Encephalopathy ▶ Intracranial Pressure

Overdeveloped, larger than normal. Hypertrophy is due to the enlargement of the normal parts of a structure, not due to additional parts. There are no more cells than normal, but all the cells are larger in size. Cardiac hypertrophy is often due to untreated hypertension. The most common cause of muscular hypertrophy is exercise. If a structure such as a muscle is larger than normal due to additional tissue/cells, it is referred to as pseudohypertrophy, for example, in Duchenne’s muscular dystrophy.

Cross References ▶ Hypertension

Hypokinesis

Hypoarousal J ULIE T ESTA F LAADA Rochester, MN, USA

Definition Central nervous system arousal is fundamental to all cognitive and emotional functions. In an aroused state, an organism is more alert to sensory stimuli, is more motorically active, and is more emotionally reactive (Pfaff, 2006). Following a central nervous system insult, levels of arousal can range over a continuum from coma, stupor, lethargy to alertness. In states of hypoarousal, individuals are less alert, have reduced emotional and cognitive capacity, and have less motor control. Since arousal levels exist on a continuum, repeatedly administered standardized tests are the most common means of quantitatively measuring changes in arousal levels. Examples of scales that assess level of arousal, as well as other common symptoms following brain injury, include the Galveston Orientation and Amnesia Test (GOAT; Levin, O’Donnell & Grossman, 1979), the Agitated Behavior Scale (ABS; Corrigan, 1989), the Delirium Rating Scale – Revised (DRS-R; Trzepacz et al., 2001), the Cognitive Test for Delirium (CTD; Hart et al., 1996), and the Confusion Assessment Protocol (Sherer et al., 2005; http://www.tbims.org/combi/cap). Pathophysiological measurements can provide useful information to the clinician who is faced with the challenging task of determining if a patient is in a state of hypoarousal or not. Electroencephalography (EEG) and evoked potentials (EPs) evaluate the functional status of the brain and augment the clinical examination.

▶ Somatization

Hypoglycemia G EORGE J. D EMAKIS University of North Carolina Charlotte Charlotte, NC, USA

Definition Hypoglycemia is characterized by extremely low blood glucose levels. Although 60–70 mg/dL (or milligrams per deciliter) is typically cited as the lower level for normal glucose, different values have also been proposed. Hypoglycemia is commonly associated with, among other symptoms, generalized discomfort, sweating, weakness, irritability, tremor, and poor motor coordination. If left untreated, confusion, seizures, loss of consciousness, brain damage, and death are possible. Hypoglycemia is common in diabetes, particularly Type 1 diabetes and is usually due to an excessive dose of insulin, a hormone necessary to convert sugar and other foods to energy, or oral hypoglycemic agents, reduced food ingestion, or increased exercise. It can be treated by the ingestion of dextrose or foods that are digestible to glucose (e.g., orange juice).

Cross References

Cross References

Hypokinesia ▶ Hemikinesis

References and Readings Pfaff, D. (2006). Brain arousal and information theory: Neural and genetic mechanisms. Cambridge: Harvard University Press. Sherer, M., Nakase-Thompson, R., Yablon, S. A., & Gontkovsky, S. T. (2005). Multidimensional assessment for acute confusion after TBI. Archives of Physical Medicine and Rehabilitation, 86, 896–904.

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▶ Diabetes Mellitus

▶ Coma ▶ Minimally Responsive State

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Hypothalamic Glioma

Hypothalamic Glioma R OBERT R IDER Drexel University Philadelphia, PA, USA

Definition Hypothalamic glioma are tumors originating in the hypothalamus gland of the brain. These types of tumors can produce a range of symptoms, which include changes in growth rate, hyperactivity, euphoria, loss of appetite, disruptions of vision, and headache. Seizures may occur as a result of a hypothalamic glioma. The course of tumor growth may be slow or rapid and tumors may remain static in size for years. Visual field testing can aid in tracking the rate of tumor growth.

References and Readings Armstrong, C. L., Schmus, C. J., & Belasco, J. B. (In press). Neuropsychological problems in neuro-oncology. In C. L. Armstrong (Ed.), Handbook of medical neuropsychology: Applications of cognitive neuroscience. New York: Springer.

Structure The hypothalamus is bounded by the lamina terminalis rostrally, the hypothalamic sulcus dorsally, and the internal capsule laterally. Forming the walls and floor of the inferior portion of the third ventricle, this diencephalic structure is composed of three functional mediolateral zones and three anatomical anterior–posterior levels. The mediolateral organization – the periventicular, middle, and lateral zones – corresponds to the functions of the particular nuclei in each area. The periventricular zone contains nuclei that regulate the release of endocrine hormones from the pituitary; the middle zone is a major site for nuclei that regulate the autonomic nervous system and the release of hormones from the posterior pituitary gland; the lateral zone contains nuclei that are responsible for the integration and communication of information from limbic system structures (Martin, 1996). Lastly, in terms of connections, the hypothalamus receives afferent fibers from the viscera, the olfactory mucous membrane, the cerebral cortex, and the limbic system. The main descending pathways of the hypothalamus include: descending fibers to the brain and spinal cord, the mammillothalamic tract, the mammillotegmental tract, and multiple pathways to the limbic system.


Hypothalamus A MANDA WAXMAN City University of New York New York, NY, USA

Definition Hypothalamus, derived from Greek U‘pοyalamοs, meaning under the thalamus, occupies the major portion of the ventral diencephalon and forms the wall and floor of the inferior portion of the third ventricle. Comprised of specialized groups of neurons clustered in bilateral nuclei, the hypothalamus serves as the central regulator of homeostasis by interacting with and exerting regulatory influence over the following systems: the endocrine system via the pituitary gland, the autonomic nervous system, and the limbic system.

Initial studies related to the hypothalamus are focused on identifying its boundaries, structural components, nuclei, tracts, and many interconnections as well as its control of somatic activities and autonomic nervous system functions. At the beginning of the twentieth century, Stephen Ranson, Philip Cannon, and Walter Hess demonstrated how hypothalamic stimulation evoked autonomic and somatic responses characteristic of emotional states. Then, by the mid-1950s, interest developed in the hypothalamic control of thirst (Greer, 1955; Mogensen and Stevenson, 1967), sexual behavior (Baird, Wilson, Bladin, Saling, & Reutens, 2007), metabolism, and body weight (Anand & Brobeck, 1951). Additionally, at this time, Harris (1948) identified hypothalamic control of the anterior pituitary gland through the hypophysial portal system. Today, as obesity rates of Westernized nations have grown to epidemic proportions, investigations continue to focus on hypothalamic involvement in feeding behavior, glucose homeostasis, and body weight. Researchers also work to identify specific mechanisms by which the hypothalamus integrates autonomic and

Hypothalamus

endocrine functions in order to control bodily homeostasis (Benarroch, 2007; Swanson & Mogenson, 1981).

Current Knowledge Endocrine Control The hypothalamus controls the endocrine system mainly through its regulatory effect on the pituitary gland. Neurosecretory neurons in the periventricular zone of the hypothalamus control the release of anterior pituitary hormones by secreting releasing or release-inhibiting hormones. Forming the hypophysial portal system, these neurons synapse onto capillaries of the pituitary portal circulation and release their hormones. The portal veins then carry these hormones to the anterior lobe of the pituitary. Finally, the releasing or release-inhibiting hormones regulate the hormone release from the anterior pituitary. The anterior pituitary produces six hormones: adrenocorticotropic hormone (ACTH), growth hormone (GH), thyroid-stimulating hormone (TSH), prolactin, luteinizing hormone (LH), and follicle-stimulating hormone (FSH). A brief description of the function of each of these hormones will further illustrate the role of the hypothalamus in numerous metabolic processes. In order to mediate the classical response to stress, corticotropinreleasing hormone (CRH) neurons in the hypothalamic paraventricular nucleus (PVN) induce ACTH release from the pituitary. ACTH stimulates the adrenal cortex to produce corticosteroid (stress) hormones, which are vital for maintaining blood pressure and ensuring electrolyte balance. GH promotes increased growth of long bones and other tissues, whereas TSH stimulates the thyroid gland to produce thyroxine and triiodothyronine which promote cellular metabolism. Prolactin causes the mamillary glands to produce milk. Finally, LH and FSH regulate ovarian hormones responsible for the menstrual cycle and oogenesis in females, and testicular hormones and spermatogenesis in males. In contrast to the hypophysial portal system, neurons from the supraoptic and paraventricular nuclei of the hypothalamus project directly to the posterior pituitary. These neurons produce and transport oxytocin and vasopressin to the posterior pituitary gland, where they are released into the blood. Vasopressin, also known as the antidiuretic hormone (ADH), produces water reabsorption from the distal tubules of the kidney to reduce urine volume. Disruption of the transportation of vasopressin from the hypothalamus, due to a lesion in the anterior hypothalamus, can lead to diabetes insipidus (Mendoza &

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Foundas, 2008). Oxytocin stimulates uterine contractions and promotes ejection of milk from the mamillary glands.

Autonomic Control The PVN of the hypothalamus represents one of the most vital autonomic control centers in the brain. The autonomic nervous system, comprising the sympathetic and parasympathetic divisions, regulates many functions of the various organ systems of the body: cardiovascular, respiratory, gastrointestinal, exocrine, and urogenital. Originating from the PVN, descending projections initially travel through the medial forebrain bundle and then in the dorsolateral brainstem and periaqueductal gray matter. These fibers eventually synapse onto preganglionic parasympathetic nuclei in the brainstem and intermediate zone of the sacral spinal cord and onto preganglionic sympathetic neurons in the intermediolateral cell column of the thoracolumbar spinal cord (Blumenfeld, 2002). Finally, sympathetic postganglionic neurons release predominantly norepinephrine onto end organs whereas parasympathetic postganglionic neurons mainly release acetylcholine. Additionally, current research suggests that the PVN plays significant and essential roles in integrating multiple sources of afferent input and producing a unified autonomic output by modifying the excitability of multiple output pathways (Ferguson, Latchford, & Samson, 2008). Researchers suggest that this integrative circuitry may contribute to the pathology of conditions such as hypertension and congestive heart failure.

Regulation of Homeostatic Behaviors The hypothalamus serves to integrate autonomic response and endocrine function with behavior, in order to maintain homeostasis. Several of these essential homeostatic behaviors include food and water intake, circadian rhythms, sleep–wake cycles, sexual desire, and thermoregulation.

Food and Water Intake In 1942, Albert W. Hetherington and Stephen Walter Ranson demonstrated that bilateral lesions in the ventromedial hypothalamus (VMH) led to hyperphagia and obesity. Concurrently, Anand and Brobeck (1951) discovered that bilateral lesions of the lateral hypothalamus produced reduction in feeding and body weight. Therefore, these findings originally identified the VMH as the feeding

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center and the lateral hypothalamus as the satiety center. Current research has indicated that the VMH and lateral hypothalamus do not operate as isolated centers but are essential components of neural circuits that mediate feeding behavior. Furthermore, leptin has recently been identified as a circulating hormone produced by white adipose tissues that may provide the link among these neural circuits (Friedman & Halass, 1998). Specifically, leptin has been shown to regulate feeding behavior in part by modulating the activity of neuropeptide Y (NPY) and proopiomelanocortin (POMC) neurons in the hypothalamic arcuate nucleus (Elmquist, Maratos-Flier, Saper, & Flier, 1998). Additionally, disrupted hypothalamic regulation of feeding behavior and endocrine function, as well as the actions of leptin and other hormones, may contribute to eating disorders, such as anorexia nervosa and bulimia nervosa (Zigman & Elmquist, 2003). Another homeostatic mechanism, water intake results from activation of osmoreceptors in the anterior regions of the hypothalamus. Studies have demonstrated that electrical stimulation of the lateral hypothalamus induces drinking behavior in rats, goats, and other animals (Greer, 1955; Mogensen and Stevenson, 1967). However, this area is not responsible for the thirst drive. The dorsomedial hypothalamus (DMH) represents a potential candidate for the thirst drive since electrolytic lesions of the DMH cause permanent hypodipsia (Bellinger & Bernardis, 1982).

Circadian Rhythms and Sleep–Wake Cycles The suprachiasmatic nucleus (SCN) of the hypothalamus functions as the ‘‘master clock’’ for circadian rhythms. This nucleus receives direct projections from the retina, allowing visual stimuli to synchronize the body’s internal clock. In addition, the SCN forms local connections with other hypothalamic nuclei and projects to the pineal gland and other extrahypothalamic structures. The axis between the SCN and the PVN of the hypothalamus is crucial for the synchronization of the neuroendocrine and autonomic nervous system with the time of day. Specifically, this SCN-neuroendocrine PVN axis controls the time of hormonal secretions whereas the SCN-autonomic PVN axis prepares the organs for the reception of these hormones by means of the autonomic nervous system (Buijs, van Eden, Goncharuk, & Kalsbeek, 2003). In terms of the sleep–wake cycles, electrophysiological, brain activation, and lesion studies have produced a significant amount of evidence for the existence of a sleep-regulating mechanism within the preoptic area (Sewards & Sewards, 2003; Szymusiak, Alam, Steininger, & McGinty, 1998). Pathological changes in the SCN have been found in individuals suffering from sleep disorders, depression, and hypertension.

Sexual Behavior The hypothalamus appears to mediate neuroendocrine and autonomic aspects of sexual drive. As indicated by lesion and brain stimulation studies in animals, the VMH plays a crucial role in regulating sexual drive in females whereas the medial preoptic nucleus influences male sexual drive (Sewards & Sewards, 2003). The human literature related to the functional and anatomical role of the hypothalamus in sexual behavior has been more inconclusive. Roeder, Orthner, and Mu¨ller (1972) conducted stereotaxic lesions of the VMH in order to treat pedophilic homosexuality and other sexual deviations. Sexual drive was diminished or abolished in all cases. Additionally, a 50-year-old patient with hypothalamic damage resulting from an infiltrating glioma of the left midbrain hypothalamic region developed erectile and ejaculatory difficulties and a pedophilic sexual preference (Miller, Cummings, McIntyre, Ebers, & Grode, 1986). Therefore, the involvement of the hypothalamus in more complex aspects of sexuality, such as sexual preference, state of arousal, and gender identity, has also been suggested and deserves further investigation (Baird et al., 2007).

Thermoregulation Thermoregulation exemplifies the integrative role of the hypothalamus in generating patterns of autonomic, endocrine, motor, and behavioral responses to adapt to environmental challenges. This thermoregulatory control influences bodily functions, such as the cardiovascular system, respiration, muscle tone, endocrines, sweating, salivation, and piloerection (Benarroch, 2007). The medial preoptic/anterior hypothalamic area (POA) plays an essential role in thermoregulation. The primary thermosensitive area of the CNS, the POA contains warm-sensitive (WS), cold-sensitive, and temperatureinsensitive neurons as well as receives and integrates input from ascending neural pathways carrying information derived from sensory receptors in the periphery. Recent evidence has also suggested that the dorsomedial nucleus of the hypothalamus (DMH) may serve as the key hypothalamic site responsible for the integration of autonomic, endocrine, and behavioral systems that mediate thermoregulation in mammals (DiMicco & Zaretsky, 2007).

Peripheral Expression of Emotional States The hypothalamus stimulates the symptomatic manifestations of emotional states. When intense emotions such

Hypotheses

as fear, rage, pleasure, and excitement are generated, the cerebral cortex transmits signals to the hypothalamus. The hypothalamus then triggers physiological changes through the autonomic nervous system and through the release of hormones from the pituitary gland. In 1928, Phillip Bard first emphasized the role of the hypothalamus as an essential center for coordination of both the autonomic and somatic components of emotional behaviors. Bard removed both cerebral hemispheres (including cortex, underlying white matter, and basal ganglia) in cats and found that after the surgeries, these animals exhibited sham rage. This enraged behavior with no obvious target included the typical autonomic correlates of this emotion: increased blood pressure and heart rate, retraction of the eyelids, dilation of the pupils, and erection of the hairs on the back and tail (Purves et al., 2004). Additionally, the cats exhibited somatic motor components of anger, such as arching the back, extending the claws, lashing the tail, and snarling. Bard specifically identified the caudal hypothalamus as the main contributor to the sham rage. Then, in the 1940s, Walter Hess showed that electrical stimulation of discrete sites in the hypothalamus of cats also leads to a rage response and even to subsequent attack behavior. Stimulation of the lateral hypothalamus caused a defensive posture that resembled fear (Purves et al., 2004). Therefore, Hess suggested that the hypothalamus coordinates the peripheral expression of emotional states.

Cross References ▶ Autonomic Nervous System ▶ Circadian Rhythms

References and Readings Anand, B. K., & Brobeck, J. R. (1951). Localization of a feeding center in the hypothalamus of the rat. Proceedings of the Society for Experimental Biology and Medicine, 77, 323–324. Baird, A. D., Wilson, S. J., Bladin, P. F., Saling, M. M., & Reutens, D. C. (2007). Neurological control of human sexual behavior: Insights from lesion studies. Journal of Neurology, Neurosurgery, and Psychiatry, 78, 1042–1049. Bellinger, L. L., & Bernardis, L. L. (1982). Water regulation in weanling hypodipsic dorsomedial hypothalamic-lesioned rats. American Journal of Physiology, 242, R285–R295. Benarroch, E. E. (2007). Thermoregulation: Recent concepts and remaining questions. Neurology, 69, 1293–1297. Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sunderland, MA: Sinauer Associates.

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Buijs, R. M., van Eden, C. G., Goncharuk, V. D., & Kalsbeek, A. (2003). The biological clock tunes the organs of the body: Timing by hormones and the autonomic nervous system. Journal of Endocrinology, 177, 17–26. Dimicco, J. A., & Zaretsky, D. V. (2007). The dorsomedial hypothalamus: A new player in thermoregulation. American Journal of Physiology: Regulatory, Integrative, and Comparative Physiology, 292, R47–R63. Elmquist, J. K., Maratos-Flier, E., Saper, C. B., & Flier, J. S. (1998). Unraveling the central nervous system pathways underlying responses to leptin. Nature Neuroscience, 1, 445–450. Ferguson, A. V., Latchford, K. J., & Samson, W. K. (2008). The paraventricular nucleus of the hypothalamus – a potential target for integrative treatment of autonomic dysfunction. Expert Opinions in Therapeutic Targets, 12, 717–727. Friedman, J. M., & Halass, J. L. (1998). Leptin and the regulation of body weight in mammals. Nature, 395, 763–770. Greer, M. A. (1955). Suggestive evidence of a primary ‘‘drinking center’’. Proceedings of the Society for Experimental Biology and Medicine, 89, 59–62. Harris, G. W. (1948). Electrical stimulation of the hypothalamus and the mechanism of neural control of the adenohypophysis. Journal of Physiology, 107, 418–429. Martin, J. H. (1996). Neuroanatomy: Text and atlas (2nd ed.). Stamford, CT: Appleton & Lange. Mendoza, J., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach. New York: Springer. Miller, B. L., Cummings, J. L., McIntyre, H., Ebers, G., & Grode, M. (1986). Hypersexuality or altered sexual preference following brain injury. Journal of Neurology, Neurosurgery, and Psychiatry, 49, 867–873. Mogenson, G. J., & Stevenson, J. A. (1967). Drinking induced by electrical stimulation of the lateral hypothalamus. Experimental Neurology, 17, 119–127. Purves, D., Augustine, G. J., Fitzpatrick, D., Hall, W. C., Lamantia, A.-S., McNamara, J. O., et al. (2004). Neuroscience (3rd ed.). Sunderland, MA: Sinauer Associates, Inc. Roeder, F., Orthner, H., & Mu¨ller, D. (1972). The stereotaxic treatment of pedophilic homosexuality and other sexual deviations. In E. Hitchcock, L. Laitinen, & K. Vaernet (Eds.), Psychosurgery (pp. 87–111). Springfield, MA: Charles C Thomas. Sewards, T. V., & Sewards, M. A. (2003). Representations of motivational drives in mesial cortex, medial thalamus, hypothalamus and midbrain. Brain Research Bulletin, 61, 25–49. Swanson, L. W., & Mogenson, G. J. (1981). Neural mechanisms for the functional coupling of autonomic, endocrine and somatomotor responses and adaptive behavior. Brain Research Review, 3, 1–34. Szymusiak, R., Alam, N., Steininger, T. L., & McGinty, D. (1998). Sleep-waking discharge patterns of ventrolateral preoptic/anterior hypothalamic neurons in rats. Brain Research, 803, 178–188. Zigman, J. M., & Elmquist, J. K. (2003). From anorexia to obesity – the yin and yang of body weight control. Endocrinology, 144, 3749–3756.

Hypotheses ▶ Concept Learning

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Hypothesis Testing Approach (to Evaluation)

Hypothesis Testing Approach (to Evaluation) S HAHAL R OZENBLATT Advanced Psychological Assessment P.C. Smithtown, NY, USA

based on the referral question, history, and previous interventions and evaluations. In the second component, measures of intelligence and academic achievement are administered and theories of function and dysfunction are refined. Additional measures are administered to confirm or refute the theories generated, dysfunctional processes are broken down into their finer components, and interventions are developed to remediate the deficits (Hale & Fiorello).

Definition The hypothesis testing approach to neuropsychological assessment (HTA) is a flexible approach to testing. It enables the clinician to generate tentative explanations for a patient’s impaired capacities or functions in a manner that enables the explanations to be tested and, thereby, supported or refuted and refined (Lezak, Howieson, & Loring, 2004).

Current Knowledge The primary advantage of the HTA is that it ‘‘enables the examiner to generate multistage, serial hypotheses for identifying subtle or discrete dysfunctions or to make fine diagnostic or etiologic discriminations’’ (Lezak et al., 2004). The HTA requires the clinician to examine the component functions that might be involved in, or that contribute to, the phenomenon in question. Neuropsychological measures and procedures are chosen according to the clinician’s initial hypotheses. Based on the patient’s performance, a component function may be determined to contribute, or not, to the clinical phenomenon, thus enabling refinement of the original hypothesis and additional assessment. Changes in test selection or observation methods, modification of test administration (e.g., testing the limits), or pursuit of additional history or medical information may occur at any point in the evaluation process. Compared with fixed approaches to neuropsychological assessment, the ability to refine one’s theory of function and dysfunction in this way may facilitate a more accurate diagnosis and a treatment plan that is better tailored for the patient’s needs (Goldberg, 2001). The HTA has been adapted for use in a variety of settings. One example of how this approach has been used and elaborated is the Cognitive Hypothesis Testing Model (CHT; Hale & Fiorello, 2004). This model was developed for use in the school system to provide assessment and intervention for children who struggle academically and behaviorally. The first step or component of CHT involves development of a theory of the problem

Cross References ▶ Boston Process Approach ▶ Fixed Battery ▶ Flexible Battery

References and Readings Goldberg, E. (2001). The executive brain: Frontal lobes and the civilized mind. New York: Oxford University Press. Hale, J. B., & Fiorello, C. A. (2004). School neuropsychology: A practitioner’s handbook. New York: Guilford. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment, (4th ed.). New York: Oxford University Press.

Hypothyrodisis ▶ Hypothyroidism

Hypothyroidea ▶ Hypothyroidism

Hypothyroidism J ANE AUSTIN William Paterson University Wayne, NJ, USA

Synonyms Autoimmune thyroiditis; Congenital hypothryroidism; Cretinism; Goitrous hypothyroidism; Hashimoto disease;

Hypothyroidism

Hashimoto thyroiditis; Hypothyrodisis; Hypothyroidea; Idiopathic hypothyroidism; Primary hypothyroidism; Secondary hypothyroidism; Subclinical hypothyroidism; Thyroiditis

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conditions. Secondary-based causes of hypothyroidism are less common and may result from tumors of the pituitary gland or brain tumors affecting the hypothalamus and/ or treatments of these types of tumors. Also uncommon are congenital birth defects affecting the thyroid gland (cretinism) (Roberts & Ladenson, 2004).

Definition Hypothyroidism is a disorder of the endocrine system, which is a result of insufficient thyroid hormone.

Current Knowledge Hypothyroid Disorder Hypothyroidism refers to the insufficient production of thyroid hormones. Primary hypothyroidism occurs when the thyroid gland does not produce adequate amounts of thyroid hormone. Secondary causes of hypothyroidism occur when the pituitary gland fails to release adequate levels of thyrotropin (thyroid-stimulating hormone) or when the hypothalamus does not release sufficient thyrotropinreleasing hormone (Devdhar, Ousman & Burman, 2007). Hypothyroidism can be acquired or congenital and varies in severity ranging from subclinical to clinical manifestations (Roberts & Ladenson, 2004). Approximately 4.6% of the United States population has some level of hypothyroidism with clinical levels diagnosed in 0.3% and subclinical levels in 4.3%. It is more common in women and the incidence increases with age (Hollowell et al., 2002).

Symptoms While some individuals experience no or minimal symptoms related to hypothyroidism, the common symptoms include: cold intolerance, fatigue, weight gain, constipation, decreased appetite, sleepiness, coarse hair/hair loss, dry skin, depression, menstrual irregularities, impaired memory, difficulty in concentrating, bradycardia, and hoarseness (American Association of Clinical Endocrinologists, 2002).

Diagnosis and Treatment Diagnosis is generally based on symptomology and blood tests measuring hormone levels. Treatment involves daily doses of synthetic thyroid hormone levothyroxine (Synthroid, Levothroid, Levoxyl, Unithroid), which restores hormonal balance and relieves symptoms. Treatment of subclinical hypothyroidism is controversial and, at this time, the benefits of pharmacological treatment are inconclusive (Surks et al., 2004).

Prognosis Etiology Worldwide, the most prevalent cause of hypothyroidism is insufficient dietary iodine intake. In countries where iodine intake is adequate, hypothyroidism is most commonly caused by the autoimmune disease Hashimoto’s thyroiditis whereby antibodies inhibit the thyroid gland’s production of hormones. Hypothyroidism can also result from the gland being exposed to radiation, surgical removal of all or part of the gland, or certain medications such as lithium and thalidomide (Roberts & Ladenson, 2004; Devdhar, Ousman, & Burman, 2007). In addition, postpartum thyroiditis occurs in approximately 3–16% of women giving birth (Gerstein, 1990) and generally is of short duration. Other transient hypothyroid diagnoses can be linked to inflammatory or viral

Mortality from hypothyroidism is extremely rare. With adequate treatment and follow-up, hypothyroidism and its symptoms can be successfully managed.

Neurocognitive Functions Preliminary studies have pointed to neurocognitive deficits in hypothyroid adults that include: memory, complex attention and concentration, and perceptual and visuospatial function. Other deficits may also be observed; however, further research is needed to more fully understand the impact of hypothyroidism on various cognitive domains and whether the effects are reversible (Dugbartey, 1998; Wekking et al., 2004).

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Cross References ▶ Fatigue ▶ Hypothalamic Glioma ▶ Hypothalamus ▶ Pituitary Mass

References and Readings American Association of Clinical Endocrinologists. (2002). American Association of Clinical Endocrinologists medical guidelines for clinical practice for the evaluation and treatment of hyperthyroidism and hypothyroidism. Endocrine Practice, 8, 457–469. Col, N. F., Surks, M. I., & Daniels, G. H. (2004). Subclinical thyroid disease: Clinical applications. JAMA, 291, 239–243. Devdhar, M., Ousman, Y. H., & Burman, K. D. (2007). Hypothyroidism. Endocrinology and Metabolism Clinics of North America, 6, 595–615. Dugbartey, A. T. (1998). Neurocognitive aspects of hypothyroidism. Archives of Internal Medicine, 158, 1413–1418. Gerstein, H. C. (1990). How common is postpartum thyroiditis? A methodologic overview of the literature. Archives of Internal Medicine, 150, 1397–1400. Helfand, M. (2004). Screening for subclinical thyroid dysfunction in nonpregnant adults: a summary of the evidence for the U.S. Preventive Services Task Force. Annals of Internal Medicine, 140, 128–141. Hollowell, J. G., Staehling, N. W., Flanders, W. D., Hannon, W. H., Gunter, E. W., Spencer, C. A., et al. (2002). Serum TSH, T(4), and thyroid antibodies in the United States population (1988 to 1994): National Health and Nutrition Examination Survey (NHANES III). Journal of Clinical Endocrinology and Metabolism, 87, 489–499. Ladenson, P. W., Singer, P. A., Ain, K. B., Bagchi, N., Bigos, S. T., Levy, E. G., et al. (2000). American Thyroid Association Guidelines For

Detection of Thyroid Dysfunction. Archives of Internal Medicine, 160, 1573–1575. Roberts, C. G. P., & Ladenson, P. W. (2004). Hypothyroidism. Lancet, 363, 793–803. Surks, M. I., Ortiz, E., Daniels, G. H., Sawin, C. T., Col, N. F., Cobin, R. H., et al. (2004). Subclinical thyroid disease: scientific review and guidelines for diagnosis and management. JAMA, 14, 228–238. Wekking, E. M., Appelhof, B. C., Fliers, E., Schene, A. H., Huyser, J., Tijssen, J. G. P., et al. (2005). Cognitive functioning and well-being in eurthyroid patients on thyroxine replacement therapy for primary hypothyroidism. European Journal of Endocrinology, 15, 747–753.

Hypoxia ▶ Anoxia

Hysteria ▶ Somatization

Hysterical Pseudoseizures ▶ Psychogenic Non-Epileptic Seizures

I IADLs ▶ Instrumental Activities of Daily Living

Iatrogenic M ICHAEL R. V ILLANUEVA , S USAN K. J OHNSON University of North Carolina-Charlotte Charlotte, NC, USA

Definition Iatrogenic refers to a complication or adverse result of a medical therapy or diagnostic procedure that is inadvertently induced by a physician, psychologist, therapist, or other healthcare professionals.

Iatrogenic Sources In-hospital medical errors contribute to nearly 200,000 potentially preventable deaths a year in the United States, while nosocomial (i.e., an iatrogenic illness acquired during hospital care) infections kill approximately 100,000 hospitalized patients a year. Although this number may appear high, medical errors and nosocomial infections represent just a couple of iatrogenic sources. Other sources include but are not limited to misdiagnoses, polypharmacological interactions, adverse effects of prescription drugs, and blood transfusions.

ICH ▶ Intracerebral Hemorrhage

Ictal Phenomena Current Knowledge Overview Iatrogenic conditions almost exclusively refer to complications or adverse effects stemming from medical treatment, while the healthcare professional is often not blamed. In fact, sometimes an individual (patient) may react atypically to a medication or diagnostic procedure when compared with the average person under similar conditions. More specifically, an individual’s genetic makeup (e.g., genetic mutations) may variably predispose them to adversely react to certain medications. Yet, these types of outcomes are labeled idiosyncratic events by some and iatrogenic conditions by others. The term iatrogenic may appear vague to some individuals in part because its interpretation can be subjective and contextually influenced.

K ENNETH P ERRINE Northeast Regional Epilepsy Group Hackensack, NJ, USA Weill-Cornell College of Medicine New York, NJ, USA

Definition Ictal phenomena are the expressions of a paroxysmal disorder during the height of an attack. An ictus (Latin for stroke) can refer to the sudden attack seen in epilepsy, cerebrovascular accidents (CNS stroke), sunstroke, migraine, or apoplexy (a sudden rush of blood – formerly used in reference to CNS stroke). It is a word denoting the peak in a temporal continuum of one of these disorders. It is best used in neurology and neuropsychology when referring to migraine or epilepsy. The temporal course of a sudden attack of migraine or epilepsy can include a


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prodrome, aura, ictus, postictal state, and inter-ictal state. A prodrome can be a premonitory symptom preceding the attack by hours or even days. An aura (▶ aura) immediately precedes an ictal event by seconds or minutes. The ictus is the attack itself, and the postictal state refers to signs and symptoms immediately following the ictus. The inter-ictal state is the asymptomatic baseline of the individual, although in epilepsy there can be inter-ictal signs and symptoms referable to or presumably caused by the underlying repetitive seizures.

References and Readings Engel, J., Moshe, S., Aicardi, J., Dichter, M. A., & Pedley, T. A. (Eds.). (2007). Epilepsy: A comprehensive textbook, (2nd Ed.). New York: Lippincott Williams & Wilkins. Ropper, A. H., & Samuels, M. (2009). Adams and Victor’s principles of neurology, (9th Ed.). New York: McGraw-Hill. www.epilepsyfoundation.org

IDEA Current Knowledge In epilepsy, the ictus depends upon the type of seizure being manifested and, if a partial seizure, the localization of the initial epileptic discharge. In generalized seizures, the ictus is usually quite obvious and is simply the behavioral manifestations of the particular type of generalized seizure (e.g., brief staring perhaps with eye blinking for a few seconds in an absence seizure). In partial seizures, the ictus is the perceptual phenomena experienced by the patient or the behavioral manifestation, depending upon the localization of the onset. For instance, the ictus of a simple partial seizure arising from the primary motor cortex consists of clonic movements of the muscle group controlled by that part of the homunculus. The ictal segment of a complex partial seizure arising from mesial temporal structures may consist of motionless staring, motor automatisms, and other phenomena accompanied by disrupted consciousness. The ictus may have localizing value, such as the clonic movements of a focal motor seizure or the ‘‘fencer’s posture’’ seen in seizures arising from the supplementary motor cortex. The duration of the ictus in epilepsy usually ranges from seconds up to several minutes. It is sometimes difficult differentiating the aura, ictus, and postictal state accompanying epileptic seizures, and prolonged video-EEG monitoring can help identify these temporal phases with their associated localization.

Cross References ▶ Absence Seizure ▶ Aura ▶ Epilepsy ▶ Grand Mal Seizure ▶ Paroxysmal Disorder ▶ Partial Seizure ▶ Seizure

▶ Individuals with Disabilities Education Act

IDEA 2004 ▶ Individuals with Disabilities Education Act

Ideas of Reference PAUL N EWMAN Drake Center Cincinnati, OH, USA

Synonyms Referential delusion

Definition A type of delusion in which a person incorrectly believes that circumstances or external events (i.e., comments, book passages, song lyrics, etc.) concern or are directed at him or her (have ‘‘reference’’ to one’s self). Ideas of reference, like other delusions, is a symptom of psychosis.

Cross References ▶ Delusion ▶ Psychosis

Idiotypic Cortex

References and Readings American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: American Psychiatric Association.

Ideational Apraxia ▶ Apraxia

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Idiotypic Cortex K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Synonyms Heterotypic cortex; Primary cortex

Definition

IDEIA ▶ Individuals with Disabilities Education Act

Ideomotor Apraxia ▶ Apraxia

Idiopathic Environmental Intolerance ▶ Multiple Chemical Sensitivity

Idiopathic Facial Paralysis ▶ Bell’s Palsy

Idiopathic Hypothyroidism ▶ Hypothyroidism

Idiopathic Polyneuritis ▶ Guillain–Barre´ Syndrome

Idiotypic cortex are those areas of neocortex, which either receive direct projections from the specific sensory relay nuclei of the thalamus (primary sensory cortex) or represent the final common pathway for motor fibers (primary motor cortex) prior to their entering the corticobulbar and corticospinal tracts of the internal capsule.

Current Knowledge Cytoarchitecturally, idiotypic cortex is characterized by a relative lack of differentiation among the cellular layers, especially between 2 and 5. As suggested above, based on their anatomical and functional connections, idiotypic cortices can be divided into two broad types, primary sensory and primary motor areas. The primary sensory areas include the following: 1. Primary visual cortex: Brodmann’s area (BA) 17 located on the mesial surface of the occipital lobe and surrounding the calcarine fissure on both its superior (cuneus) and inferior (lingual gyrus) banks 2. Primary auditory cortex: BA 41 and probably part of 42 located primarily within the temporal operculum (Heschl’s gyrus) on the superior surface of the superior temporal gyrus 3. Primary somatosensory cortex: BA 3, 1, and 2 located on the postcentral gyrus between the central and postcentral sulci These are designated primary sensory areas in that they represent the cortical projections of the specific relay nuclei of the thalamus, meaning they represent the initial cortical input of these respective sensory systems and likely the more elementary processing of this sensory information. These areas project to unimodal association cortices, rather than to heteromodal cortex. Cytoarchitecturally, these areas

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are referred to as granular or koniocortex cortex as they are characterized by an abundance of stellate or granule cells and small pyramidal cells. Note that there is a less welldefined olfactory cortex which is thought to include the basal forebrain in the region of the anterior perforated substance or basal nuclei, portions of the amygdala (cortical amygdaloid nucleus), and adjacent pyriform and entorhinal cortices. These olfactory cortices differ from the other primary sensory cortical regions in a couple of important ways. First, these areas involve limbic and more primitive allocortex. Second, they receive direct input from the olfactory tract rather than being relayed through the thalamus. The primary motor cortex (BA 4) is located in the precentral gyrus. It represents the cortical origin of the final common pathway for executing voluntary motor control of the limbs and facial muscles. Because this area is characterized by the relative proliferation of pyramidal cells, large Betz cells, and the relative lack of stellate or granule cells, the primary motor cortex is often referred to as agranular cortex. Although all of the above are considered ‘‘primary’’ cortices, only unilateral lesions of the primary visual cortex will result in a complete loss of function (homonymous hemianopia). Unilateral lesions to the primary somatosensory area will result in reduced tactile discrimination, but not total anesthesia. Due to the extensive crossing of the auditory system, unilateral lesions to the primary auditory cortex generally have very limited effects on hearing (in either ear). Finally, lesions that are strictly limited to the precentral gyrus will typically result in significant weakness and loss of skilled movement for the affected body part, but not total paralysis.

ILS® ▶ Independent Living Scales®

Imbecillitas Phenylpyrouvica ▶ Phenylketonuria

Imipramine J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metairie, LA, USA

Generic Name Imipramine

Brand Name Tofranil

Class Tricyclic antidepressant

Cross References


▶ Auditory Cortex ▶ Sensory Cortex ▶ Visual Cortex

Increases serotonin and norepinepherine, blocks serotonin and norepinepherine reuptake

Indication References and Readings Depression Duvernoy, H. (1991). The human brain. Wien: Springer. Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach. New York: Springer. Mesulam, M. (2000). Large-scale networks, association cortex, frontal syndromes, the limbic system, and hemispheric specialization. In M. Mesulam (Ed.), Principles of behavioral neurology (Ch. 1., pp. 1–120). Philadelphia: F.A. Davis.

Off Label Use Enuresis, anxiety, neuropathic pain, chronic pain, cataplexy, insomnia

Immediate Post-Concussion Assessment and Cognitive Testing

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Description

Serious

Immediate Post-Concussion Assessment and Cognitive Testing (ImPACT) is a computerized battery of tests designed to evaluate sports-related concussions (Iverson, Lovell, & Collins, 2003). In addition to collecting basic demographic and descriptive data, the ImPACT measures various aspects of neurocognitive functioning via administration of six test modules (Fig. 1; ImPACTtest.com; Iverson et al., 2003). The Post-Concussion Symptom Scale (PCSS; Lovell & Collins, 1998), a 22-item symptom checklist used to track concussion symptoms, is also included in the ImPACT battery (Schatz, Pardini, Lovell, Collins, & Podell, 2006).

Lowered seizure threshold, seizures, QT prolongation, hepatic failure, extrapyramidal symptoms, mania, activation of suicidal ideation, hyperthermia

Common Blurred vision, constipation, fatigue, sedation, headache, diarrhea, heartburn, appetite disturbance, weight gain, anxiety, sexual dysfunction, sweating, rash

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Imitation Behavior ▶ Environmental Dependency ▶ Utilization Behavior

Immediate Post-Concussion Assessment and Cognitive Testing S UMMER I BARRA Rehabilitation Hospital of Indiana Indianapolis, IN, USA

Historical Background ImPACT version 2.0 is similar to the original version 1.0, but with some modifications (Iverson, Lovell, & Collins, 2005). Some of the modifications include the addition of the Design Memory test module and the capacity to generate two different memory composite scores (Verbal Memory and Visual Memory). Expansion of the X’s and O’s test module was also completed, thereby increasing its level of difficulty (Iverson et al., 2005). Research has documented the ImPACT version 2.0’s ability to detect the acute concussive symptoms (Iverson, Lovell, & Collins, 2003). ImPACT versions 3.0 and 4.0 are now available for updates to the previous versions (ImPACTtest. com, 2009). Normative reference data is available for versions 1.0 and 2.0 at www.impacttest.com, while age and gender referenced percentile ranks are produced immediately in the 3.0 and 4.0 score reports (Lovell, 2005). With the development of the ImPACT 2005, an additional set of composite scores can be generated measuring the following dimensions: Immediate Memory, Delayed Memory, Working Memory, X and O’s Memory-Speed Index, and Symbol Match Memory-Speed Index (Lovell, 2005). However, it should be noted that these additional composite scores have yet to be validated through peer-reviewed research and are not recommended for clinical decision-making at the present time (Lovell, 2005).

Psychometric Data Synonyms ImPACT

Research suggests the ImPACT test battery is both sensitive (81.9%) and specific (89.4%) in assessing concussion and related neurocognitive and neurobehavioral sequelae

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ImPACT Module Word memory

Neurocognitive Functioning Immediate and delayed verbal memory Immediate and delayed visual memory Reaction time, working memory, sustained attention

Composite Scores Verbal memory

Symbol match

Visual processing speed, learning, and memory

Color match

Reaction time, response inhibition, and focused attention Sustained attention, working memory, and visual-motor speed

Verbal memory, reaction time, and visual motor processing speed Reaction time and impulse control

Design memory X’s and O’s

Three letters

Visual memory Visual memory, reaction time, visual motor processing speed, and impulse control

Verbal memory and visual motor processing speed

Immediate Post-Concussion Assessment and Cognitive Testing. Figure 1 ImPACT six test modules

in a sample of high school athletes (N = 138) (Schatz et al., 2006). Convergent validity has been established between the Processing Speed and Reaction Time composite scores of the ImPACT and a traditional neuropsychological measure, the Symbol Digit Modalities Test (SDMT) (Iverson et al., 2005). Further, construct validity between the ImPACT memory composite scores and Processing Speed composite scores and other traditional neuropsychological measures including Trailmaking Tests A & B, Brief Visuospatial Memory Test (BVMT), and SDMT, has also been supported (Iverson, Franzen, Lovell, & Collins, 2003). A study of test–retest reliability with 118 student volunteers revealed low to moderate coefficients ranging from 0.15 to 0.39 from baseline to day 45 assessment and 0.39 to 0.61 for day 45 to day 50 assessments (Broglio, Ferrara, Macciocchi, Baumgartner, & Elliot, 2007). Psychometric data for the Post-Concussion Symptom Scale of the ImPACT version 2.0 revealed internal consistency reliability ranging from 0.88 to 0.94 in a large sample of high school and college students (N = 2, 304) (Iverson et al., 2003). Test–retest reliability measured in 82 concussed athletes at the high school and college levels was 0.80 (Iverson et al., 2003).

Clinical Uses The primary purpose of the ImPACT test is for assessment and management of concussive injuries in an athletic environment (Schatz et al., 2006). ImPACT ‘‘evaluation

can help to objectively evaluate the concussed athlete’s post-injury condition and track recovery for safe return to play, thus preventing the cumulative effects of concussion’’ (ImPACTtest.com, 2009). Completion of six test modules allows for generation of five composite scores including Verbal Memory, Visual Memory, Visual Motor Processing Speed, Reaction Time, and Impulse Control (Fig. 1, Schatz et al., 2006). The Impulse Control score represents the total errors across each module and is helpful in establishing validity of the battery (ImPACTtest.com, 2009). ImPACT requires minimal training and can be administered by athletic trainers, team coaches, neuropsychologist, and/or a physician (ImPACTtest.com, 2009). The test participant can complete the entire battery in approximately 20 min in either an individual or group setting (ImPACTtest. com, 2009). Upon completion, the data is automatically stored and a comprehensive report of the testing is immediately generated (ImPACTtest.com, 2009). The battery can be administered to establish a baseline of neurocognitive functioning and/or can be used to assess post-concussion status (ImPACTtest.com, 2009). When used following a concussion, the administrator is asked to also enter data regarding the characteristics of the injury and any treatment administered, including on-site symptoms, presence of retrograde amnesia, and any loss of consciousness (ImPACTtest.com, 2009). A Reliable Change Index (RCI) score can be generated in versions 3.0 and higher as a means of assessing change between baseline and post-concussive functioning on each composite score (Lovell, 2005). However, if baseline testing has not been completed, comparison to normative data for age and gender matching is recommended, thereby

Impact on Participation and Autonomy Questionnaire

allowing for comparison of the data to the participant’s peer group (Lovell, 2005).

Advantages The ImPACT has been noted as an effective instrument for concussion assessment and management, largely due to its utility for repeated administrations without the risk of practice effects (Schatz et al., 2006). Each administration is derived from a randomized sample of the stimulus (ImPACTtest.com, 2009). Further, it is able to reliably measure reaction time up to 1/100th of a second for each test module (ImPACTtest.com, 2009).

Limitations Designed as a brief screening measure, the ImPACT does not offer an in-depth comprehensive neuropsychological evaluation that may be required for complete assessment in certain cases (Lovell, 2005).

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ImPACT ▶ Immediate Post-Concussion Assessment and Cognitive Testing

Impact on Participation and Autonomy Questionnaire J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

I Synonyms IPA

Cross References

Definition

▶ Concussion ▶ Postconcussion Disorder (Syndrome) ▶ Second Impact Syndrome ▶ Sports Related Concussion

The IPA was developed by Cardol, Beelen, van den Bos, de Jong, de Groot, and de Haan (2001). It is a 39-item (or 41-item in the revised version) self-report measure encompassing the areas of mobility, self-care, household tasks and family roles, spending money, leisure, social relations, paid and voluntary work, education, and learning. Each has a set of questions associated with participation in that area, rated on a five-point scale, and a final question regarding the effect of disability on participation in that domain, rated on a three-point scale. Scores are derived for five domains, autonomy indoors, autonomy outdoors, family role, social relationships, and work/education.

References and Readings Broglio, S. P., Ferrara, M. S., Macciocchi, S. N., Baumgartner, T. A., & Elliot, R. (2007). Test-retest reliability of computerized concussion assessment programs. Journal of Athletic Training, 42, 509–514. ImPACTtest.com (2009). ImPACT background. www.impacttest.com/ impactbackground.php. Retrieved 10 March 2009. Iverson, G. L., Franzen, M. D., Lovell, M. R., & Collins, M. W. (2003). Construct validity of ImPACT in athletes with concussions. Abstract presented at National Academy of Neuropsychologist’s Annual Meeting. Iverson, G. L., Lovell, M. R., & Collins, M. W. (2003). Immediate postconcussion assessment and cognitive testing (ImPACT): Normative Data version 2 only. www.impacttest.com. Retrieved 10 March 2009. Iverson, G. L., Lovell, M. R., & Collins, M. W. (2005). Validity of ImPACTfor measuring processing speed following sports-related concussion. Journal of Clinical and Experimental Neuropsychology, 27, 683–689. Lovell, M. R., Collins, M. W. (1998). Neuropsychological assessment of the college football player. Journal of Head Trauma Rehabilitation, 13, 9–26. Lovell, M. R. (2005). ImPACT 2005 (4.0): Clinical interpretation manual. www.impacttest.com. Retrieved 14 May 2009. Schatz, P., Pardini, J. E., Lovell, M. R., Collins, M. W., & Podell, K. (2006). Sensitivity and specificity of the ImPACT test battery for concussion in athletes. Archives of Clinical Neuropsychology, 21, 91–99.

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Current Knowledge The original version of the IPA was developed in the Netherlands, but it has since been translated into English, German, and Swedish. It has been used in several patient groups, including spinal cord injury (SCI, Cardol et al., 2001), Parkinson’s Disease (Franchignoni, Ferriero, Giordano, Guglielmi, & Picco, 2007), multiple sclerosis, and rheumatoid arthritis (Sibley, Kersten, Ward, White, Mehta, & George, 2006). Initial investigations in a group of 126 people of mixed diagnoses receiving rehabilitation therapy suggested that the scale showed good internal

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consistency (Cronbach’s alpha .81–.91 for the five scores), and test-retest reliability at a 2-week interval (intra-class correlations between .83 and .91). Convergent validity was acceptable, as judged by other questionnaires with items relevant to participation (correlations around .4–.6), as was divergent validity with ‘‘economic selfsufficiency’’ and ‘‘orientation’’ subscales from another questionnaire at .01 and .29, respectively. Cardol et al. (2002) found encouraging preliminary evidence of the scale’s responsiveness to change, but this requires further investigation. Rasch analysis on data from a group of patients with SCI reported in Lund, Fisher, Lexell, & Bernspa˚ng (2007) identified separate ‘‘participation’’ and ‘‘problem’’ scales after removal of some items, with separation reliability (similar to Cronbach’s Alpha) for participation items 0.94, and for problem questions, 0.82.

Impaired Self-Awareness J ULIE T ESTA F LAADA Rochester, MN, USA

Definition Impaired self-awareness (ISA) is a disturbance of personal experience, which disrupts the integration of thinking and feeling associated with human insight, or awareness (Prigatano, 1999). As such, a person may lack awareness of cognitive, emotional, and to lesser extent, physical deficits, and have difficulty monitoring his/her own behavior and interpersonal relationships.

Cross References Current Knowledge ▶ Lawton-Brody iADL Scale ▶ LIFE-H

References and Readings Cardol, M., Beelen, A., van den Bos, G. A., de Jong, B. A., de Groot, I. J., & de Haan, R. J. (2002). Responsiveness of the impact on participation and autonomy questionnaire. Archives of Physical Medicine and Rehabilitation, 83, 1524–1529. Cardol, M., de Haan, R. J., de Jong, B. A., van den Bos, G. A., & de Groot, I. J. (2001). Psychometric properties of the impact on participation and autonomy questionnaire. Archives of Physical Medicine and Rehabilitation, 82, 210–216. Franchignoni, F., Ferriero, G., Giordano, A., Guglielmi, V., & Picco, D. (2007). Rasch psychometric validation of the impact on participation and autonomy questionnaire in people with Parkinson’s disease. Europa Medicophysica, 43, 451–461. Lund, M. L., Fisher, A. G., Lexell, J., & Bernspa˚ng, B. (2007). Impact on participation and autonomy questionnaire: Scale validity of the Swedish version for use in people with spinal cord injury. Journal of Rehabilitation Medicine, 39, 156–162. Sibley, A., Kersten, P., Ward, C. D., White, B., Mehta, R., & George, S. (2006). Measuring autonomy in disabled people: Validation of a new scale in a UK population. Clinical Rehabilitation, 20, 793–803. Van de Port, I. G. L., van den Bos, G. A. M., Voorendt, M., Kwakkel, G., & Lindeman, E. (2007). Identification of risk factors related to perceived unmet demands in patients with chronic stroke. Disability and Rehabilitation, 29, 1841–1846.

Impaired Reabsorption ▶ Reuptake Inhibition

Disturbances in self-awareness have been reported to occur following many types of central nervous system dysfunction, such as cerebrovascular events, dementia, and traumatic brain injury (TBI). The degree of ISA has been reported to be significantly associated with the number but not with location or volume of focal lesions early after TBI (Sherer et al., 2005). Persistent problems of ISA may occur following bilateral and diffuse cerebral damage (Prigatano, 1999). ISA is associated with TBI severity and contributes to poorer functional outcome. ISA is associated with poor treatment compliance, longer rehabilitation stays, and greater caregiver distress and is predictive of functional status at rehabilitation discharge, ability to live independently, and employability. ISA, however, also appears to serve as a barrier to depression. Patients with significant ISA tend to minimize impairments that are apparent to their significant others and, presumably because of their lack of awareness of impairment, tend not to be depressed (Malec et al., 2007). Comprehensive rehabilitation programs help patients with ISA gain better awareness of their cognitive, emotional, behavioral, social and physical disabilities by giving patients the opportunity to observe their behavior over time and by providing objective feedback about their strengths and weaknesses while also teaching compensatory strategies (Prigatano, 1999). Anosognosia refers to a state in which patients with brain injury lack awareness of a specific neurologic deficit, as described by Babinski in 1914. Anosognosia for hemiparesis, blindness, aphasia, and memory loss have been reported.

Implicit Memory

Anosognosia is typically associated with a nondominant hemisphere injury, particularly parietal lobe damage.

Cross References ▶ Anosognosia ▶ Holistic Brain Injury Rehabilitation ▶ Post-acute Brain Injury Rehabilitation ▶ Traumatic Brain Injury

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characterized by the inability to sustain a movement. The term is also used in memory when one is unable to correctly recall a recorded memory or thought. This term can be used for numerous areas such as task impersistence, as being the inability to consistently complete a task. Motor impersistence is commonly assessed in neurodegenerative and some psychological disorders as well as stroke and other cerebrovascular conditions.

Cross References References and Readings Malec, J. F., Testa, J. A., Rush, B. K., Brown, A. W., & Moessner, A. M. (2007). Self-assessment of impairment, impaired self-awareness, and depression after traumatic brain injury. Journal of Head Trauma Rehabilitation, 22, 156–166. Prigatano, G. P. (1999). Principles of neuropsychological rehabilitation. New York: Oxford University Press. Prigatano, G. P. (2005). Disturbances of self-awareness and rehabilitation of patients with traumatic brain injury: A 20-year perspective. Journal of Head Trauma Rehabilitation, 20, 19–29. Sherer, M., Hart, T., Nick, T. G., Whyte, J., Thompson, R. N., & Yablon, S. A. (2003). Early impaired self-awareness after traumatic brain injury. Archives of Physical Medicine and Rehabilitation, 84, 168–176. Sherer, M., Hart, T., Whyte, J., Nick, T. G., & Yablon, S. A. (2005). Neuroanatomic basis of impaired self-awareness after traumatic brain injury: findings from early computed tomography. Journal of Head Trauma Rehabilitation, 20, 287–300.

Impairment-Based Therapy

▶ False Memory ▶ Interference ▶ Motor Impersistence ▶ Psychomotor Retardation

References and Readings Lampinen, J., & Schwartz, R. (2000). The impersistence of false memory persistence. Memory, 8(6), 393–400. Levin, H. (1973). Motor impersistence in patients with unilateral cerebral disease: A cross-validational study. Journal of Consulting and Clinical Psychology, 41(2), 287–290. Marchetti, C., Carey, D., & Della Sala, S. (2005). Crossed right hemisphere syndrome following left thalamic stroke. Journal of Neurology, 252(4), 403–411. Sitnikova, T., Goff, D., & Kuperberg, G. (2009). Neurocognitive abnormalities during comprehension of real-world goal-directed behaviors in schizophrenia. Journal of Abnormal Psychology, 118(2), 256–277.

▶ Process Training

Implicit Memory Impersistence B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

E LIZABETH LOUISE G LISKY University of Arizona Tucson, AZ, USA

Synonyms Non-declarative memory

Definition

Definition

Impersistence is defined as a transitory existence or occurrence lasting only a short time. Impersistence is used with motor skill where motor impersistence is

Implicit memory refers to a change in behavior or performance that occurs as a result of prior experience without conscious recollection of that prior experience. It is

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usually contrasted with explicit memory, which refers to conscious recollection of a specific experience from the past.

Current Knowledge Although ideas about implicit memory date back to the seventeenth century philosophers, modern usage of the term dates to the 1980s, when it was first used to describe a phenomenon based on observations of amnesic patients. Warrington and Weiskrantz, two British neuropsychologists, had reported that although patients with severe amnesia were unable to recall recently-presented words or pictures, they were able to generate that information when shown degraded or fragmented forms of the pictures or words, although such information could not be produced without prior exposure. Similarly, Milner and her colleagues reported that the densely amnesic patient H.M., although unable to acquire any new information about specific recent events or experiences, was nevertheless able to learn motor skills at a normal rate. Since that time, numerous studies have demonstrated that patients with amnesia, which results in a loss of explicit memory (i.e., the ability to retrieve prior events or experiences explicitly), nevertheless have a relatively or completely intact implicit memory that allows them to acquire information that may subsequently influence behavior, although they have no explicit recollection of the prior learning experience. These findings suggest that the brain regions underlying implicit memory must be at least partly non-overlapping with those supporting explicit memory, such that damage to the hippocampus and surrounding medial temporal regions impairs explicit memory, but not implicit. Subsequent studies have suggested that there may be several kinds of implicit or nondeclarative memory, which include repetition priming, skill learning or procedural memory, and conditioning, each of which involves brain regions that are preserved in amnesia, including areas of neocortex, the basal ganglia, and the cerebellum. Finding ways to tap into preserved implicit memory to compensate for impaired explicit memory has been a focus of rehabilitation research.

References and Readings Graf, P., & Masson, M. E. J. (1993). Implicit memory: New directions in cognition, development, and neuropsychology. Hillsdale, NJ: Erlbaum. Schacter, D. L. (1987). Implicit memory: History and current status. Journal of Experimental Psychology: Learning, Memory and Cognition, 13, 501–518. Schacter, D. L. (1996). Searching for memory: The brain, the mind, and the past. New York: Basic Books.

Impulsive Petit Mal ▶ Juvenile Myoclonic Epilepsy

Impulsivity B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Definition Impulsivity is defined as a personality trait characterized by the inclination of an individual to initiate behavior without adequate forethought as to the consequences of his or her actions (impulse action), thus acting on the spur of the moment. Some related behaviors are risktaking, lack of planning, and rush to judgment or decision-making. Impulsivity is linked to several psychological disorders such as ADHD, bipolar disorder, and other substance-abuse disorders. In addition to a potential genetic link, impulsivity may also be acquired via neurodegeneration, traumatic brain injury, hypoxia, infection, or neurotoxicity.

Cross References

Cross References

▶ Amnesia ▶ H.M.; Aslo the Case of H.M., Molaison, Henry (1926– 2008) ▶ Procedural Memory

▶ Disinhibition ▶ Distractibility ▶ Habituation ▶ Irresistible Impulse

Incidental Memory


Cross References

Borkowski, J., Peck, V., Reid, M., & Kurtz, B. (1983). Impulsivity and strategy transfer: Metamemory as mediator. Child Development, 54(2), 459. Brown, S., Manuck, S., Flory, J., & Hariri, A. (2006). Neural basis of individual differences in impulsivity: Contributions of corticolimbic circuits for behavioral arousal and control. Emotion, 6(2), 239–245. Dolan, M., & Anderson, I. (2002). Executive and memory function and its relationship to trait impulsivity and aggression in personality disordered offenders. Journal of Forensic Psychiatry, 13(3), 503–526. Walderhaug, E., Landrø, N., & Magnusson, A. (2008). A synergic effect between lowered serotonin and novel situations on impulsivity measured by CPT. Journal of Clinical & Experimental Neuropsychology, 30(2), 204–211. Wassenberg, R., Hendriksen, J., Hurks, P., Feron, F., Keulers, E., Vles, J., et al. (2008). Development of inattention, impulsivity, and processing speed as measured by the d2 test: Results of a large crosssectional study in children aged 7–13. Child Neuropsychology, 14(3), 195–210.

▶ Confidentiality ▶ Independent Examination

American Psychological Association (2002). Ethical principles of psychologists and code of conduct. American Psychologist, 57, 1048–1051. Bush, S. S., & the NAN Policy & Planning Committee (2005). Independent and court-ordered forensic neuropsychological examinations: Official statement of the National Academy of Neuropsychology. Archives of Clinical Neuropsychology, 20, 997–1007. Committee on Legal Issues, American Psychological Association (2006). Strategies for private practitioners coping with subpoenas or compelled testimony for client records or test data. Professional Psychology: Research and Practice, 37, 215–222.

▶ Community-Based Rehabilitation


Inattention Definition In camera is a Latin term meaning ‘‘in chambers.’’ It refers to a hearing or discussions with the judge in the privacy of his/her chambers (office rooms) or when spectators and jurors have been excluded from the courtroom. An in camera examination may be made of confidential or sensitive information (e.g., raw test data) to determine whether to introduce it to the jury and make it part of public record. An in camera hearing can be either on or off the record, though they are usually recorded. In camera hearings are often held to shield a jury from any potential bias caused by controversial matters, or to protect the privacy of the people involved, and are common in cases of guardianships, personal injury, medical malpractice, adoptions, and custody disputes alleging child abuse. In forensic neuropsychology, in camera reviews most often take place in response to a neuropsychologist’s duty/request to protect the security of the raw test data. He/she makes a request that the judge perform an in camera review of the data so that the materials do not get distributed to non-psychologists (e.g., attorneys, jury members).

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▶ Distractibility ▶ Neglect Syndrome

Incidental Learning ▶ Incidental Memory

Incidental Memory E LIZABETH LOUISE G LISKY University of Arizona Tucson, AZ, USA

Synonyms Incidental learning

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Inclusion

Definition Incidental memory refers to memories that are acquired without intention.

Current Knowledge Although intentional learning may often lead to good memory, Craik and Tulving demonstrated that it was not the intention to learn that was critical for later memory, but rather the type of processing engaged at the time of encoding. Information that was processed meaningfully was well remembered whether or not there was an intention to remember. People also acquire information incidentally in the course of other activities, even though they have no intention of doing so and may not have processed the information meaningfully. Memories that are acquired in this way may be referred to as incidental memories.

References and Readings Craik, F. I. M., & Tulving, E. (1975). Depth of processing and the retention of words in episodic memory. Journal of Experimental Psychology: General, 104, 268–294.

Inclusion ▶ Mainstreaming

Incompetence ▶ Competence

Incomplete Effort ▶ Response Bias

Incomplete Sentences Blank ▶ Sentence Completion

Increased Tone ▶ Rigidity

Independent Living Centers A MY J. A RMSTRONG Virginia Commonwealth University Richmond, VA, USA

Definition The first Center for Independent Living was founded in 1972 by disability advocates in Berkeley, California. An Independent Living Center (ILC) is defined in Section 702 of the Rehabilitation Act of 1973 (as amended) as ‘‘. . .a consumer-controlled, community-based, crossdisability, nonresidential private nonprofit agency that is designed and operated within a local community by individuals with disabilities and provides an array of independent living services.’’ 51% of the staff of an ILC and 51% of the Board of Directors must be persons with disabilities. As such CILs are a grassroots, civil rights advocacy program. Centers are funded in part by the Department of Education, Rehabilitation Services Administration, Independent Living Branch. The four core services of ILCs include, but are not limited to: information and referral, independent living skills training, individual and systems advocacy, and peer counseling. The philosophical basis of independent living is that people with disabilities have the right to control their own lives, choices, and decisions, and the right to have access to the same options as people without disabilities. CILs work to remove architectural, structural, programmatic, economic, social, political, and medical barriers that serve to impede the full inclusion and independence of people with disabilities.

Cross References ▶ Independent Living ▶ Medical Model

Independent Medical Evaluation

References and Readings DeJong, G. (1979). Independent living: From social movement to analytic paradigm. Archives of Physical Medicine and Rehabilitation, 60, 435–446. Wolfensberger, W. (1972). The principle of normalization in human services. Toronto: National Institute on Mental Retardation.

Independent Living Scales® J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms ILS®

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schizophrenia diagnoses needing maximum, moderate, and minimal levels of supervision, suggesting it is sensitive to functional competence in everyday life.

Cross References ▶ Alzheimer’s Disease Cooperative Study ADL scale ▶ Bristol Activities of Daily Living scale ▶ Disability Assessment for Dementia ▶ Katz Index of ADLs ▶ Lawton-Brody ADL Scale

References and Readings Baird, A. (2006). Fine tuning recommendations for older adults with memory complaints: Using the independent living scales with the dementia rating scale. Clinical Neuropsychologist, 4, 649–661. Loeb, P. A. (2003). The independent living scales (ILS). San Antonio, TX: Pearson Assessment. Revheim, N., & Medalia, A. (2004). The independent living scales as a measure of functional outcome for schizophrenia. Psychiatric Services, 55, 1052–1054.

Definition The ILS is a standardized, performance-based tool for assessing competence in activities necessary for independent living. It consists of a set of screening items (vision, reading, hearing, speech, signature, writing, and walking), and a further 68 items arranged according to five subscales: memory/orientation, money management, managing home and transportation, health and safety, and social adjustment. Performance on screening items is rated as ‘‘adequate’’ or ‘‘inadequate’’ but is not included in the total score. The 68 main items are scored according to competence on a 0–2 scale, with scores being summed to give five subscale scores and a total score. Two additional subscale scores (performance/information subscale and problemsolving subscale) can be derived from the same 68 items.

Current Knowledge The ILS manual provides norms for people aged over 65 years, along with performance data from younger adults (17 years and above) with psychiatric disorders, dementia, mental retardation, and traumatic brain injury. Scores on the ILS have been found to correlate strongly with global cognitive function in older adults with varying degrees of cognitive impairment (Baird, 2006). Revheim and Medalia (2004) have found that scores on the problemsolving subscale differentiate between patients with

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Independent Medical Evaluation R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Independent neuropsychological examination

Definition An independent medical examination (IME) is an evaluation performed by a doctor who is not involved in the patient’s care for the purpose of establishing medical and job-related issues. The self-insured employer or worker’s compensation insurance carrier has a legal right to request an independent medical examination. Should an IME determine that the patient’s medical condition is not work-related, the insurer can deny the claim and refuse payment. But, the insurer must have a physician’s medical opinion prior to denying a claim. IMEs are often conducted for injured workers to determine the cause, extent, and medical treatment of an

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injury. Most requests for IMEs are for discovering whether a worker has reached maximum benefit from treatment or whether any permanent impairment remains from the injury. While an independent medical examination involves a physician and a patient, an independent medical examination does not constitute the normal physician–patient treating relationship. Although doctors conduct IMEs, they are often criticized for being neither independent nor for having conducted a real medical examination. The former complaint is because the doctors are hired and paid by the insurance carrier or a self-insured company; both the company and the insurer have a financial interest in the outcome of the review. The second complaint is because an IME is different from what is traditionally thought of as a medical exam, which is usually regarded as a much more thorough examination, designed to provide a sound basis for important decisions about wage-replacement benefits and medical treatment for injured and sick workers. Critics say that the independent medical examination is generally limited to the completion of a medical history by the claimant; a review of available documents provided by the treating doctor; a brief medical exam; the reviewing doctor asking questions about the claimant’s symptoms, and recording his or her impressions regarding the case and describing the treatment required. IMEs are also used for determinations used by insurance carriers in connection with paying medical bills and settling a case, arbitrating, and litigating claims over wage-replacement benefits. They address questions regarding the final degree of disability. Insurance carriers also use IMEs as a safeguard against fraudulent suits and claims without merit.

Cross References ▶ Confidentiality

References and Readings Bush, S. S., & the NAN Policy & Planning Committee (2005). Independent and court-ordered forensic neuropsychological examinations: Official statement of the National Academy of Neuropsychology. Archives of Clinical Neuropsychology, 20, 997–1007. Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis. Oakes, H. (2008). Cents and scentability: A disability claim due to multiple chemical sensitivity. In R. Heilbronner (Ed.), Neuropsychology in the Courtroom: Expert analysis of reports and testimony. New York: Guilford Press.

Independent Neuropsychological Examination R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition This is the neuropsychological version of an independent medical exam (IME). Independent neuropsychological examinations (INEs) are performed by neuropsychologists who are hired as independent contractors by third parties (e.g., insurance company, disability carrier) to make determinations regarding a claimant’s neuropsychological functioning. They typically occur in civil cases (e.g., personal injury, Workers’ Compensation, and disability) cases, but might also arise in criminal litigation (e.g., to assess competency to stand trial and issues of responsibility for the crime). The responsibilities of the neuropsychologist in the context of performing an INE differ from those of the clinical examination and there are several areas of distinction between an independent exam versus a clinical exam. One primary difference is the nature of the doctor– patient relationship. In the INE, the neuropsychologist is hired as an independent contractor by a third party or the court to make a determination regarding neuropsychological functioning. That third party is the client; the person being examined (e.g., examinee) is not. Nonetheless, a relationship exists between the examining neuropsychologist and the person being examined, but it differs in many ways from that of a clinical examination and the neuropsychologist remains obligated to perform his/her evaluation in a manner consistent with recognized ethical codes and the responsibilities inherent in any professional clinical evaluation (APA Ethics Code, 2002). Other issues such as the limited confidentiality of the exam, informed consent, the presence of third party observers, release of raw data, and who the report is directed to are somewhat different in the context of an INE. The neuropsychologist should be familiar with existing guidelines (Bush & NAN Policy & Planning Committee, 2005) about the roles and responsibilities of neuropsychologists when conducting INEs in order to guarantee the objectivity of the examination, to maximize the true independent nature of the process, and to facilitate the public’s perception of neuropsychology and psychology as disciplines dedicated to the highest moral and ethical standards.

Individual Education Program

Cross References ▶ Independent Medical Evaluation

References and Readings American Psychological Association (2002). Ethical principles of psychologists and code of conduct. American Psychologist, 57, 1048–1051. Bush, S. S. & the NAN Policy & Planning Committee (2005). Independent and court-ordered forensic neuropsychological examinations: Official statement of the National Academy of Neuropsychology. Archives of Clinical Neuropsychology, 20, 997–1007. Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis.

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comparison standards designate different developmental achievements, such as successive approximations of successful milestone attainment. In terms of decline in abilities, these standards may demonstrate increasing deficits over time, such as memory loss as seen in Alzheimer’s Dementia. Clinicians can benefit from using an individual’s rate of change as a prediction tool, such as by establishing a rate of improvement following a traumatic brain injury to predict a time period after which the patient may be able to resume work.

References and Readings Lezak, M. D. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

I Indicator ▶ Symptom

Individual Education Plan ▶ Individual Education Program

Individual Comparison Standards S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

Individual Education Program S HELLEY P ELLETIER Shoreline Pediatric Neuropsychological Services, LLC Old Saybrook, CT, USA

Synonyms Intra-individual comparisons

Definition The concept of individual comparison standards refers to the assessment of change over time of a function, or trait that is normally distributed in the population.

Current Knowledge Individual comparison standards are otherwise described as intraindividual comparisons that are assessed at different points in time. Developmentally, individual

Definition An Individual Education Program (IEP) is a formal written document, developed by an educational team, to address the unique learning needs of a child who has been found eligible to receive special education services. The development team consists of parents, teachers, related service professionals, school administrators, and students (when appropriate). Other individuals, such as advocates, attorneys, outside therapists, or other individuals providing support for the parent or child, may also attend the meeting. The law specifically requires that the following individuals are in attendance: the child’s parent (s) or guardian, a special education teacher, at least one regular education teacher, a person who can interpret the educational implications of evaluation results, and a representative of the school district who has the ability

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to allocate school resources (20 U.S.C. 1414 (d) (1) (b)). Specific states may have additional requirements. Although nothing in the law specifically requires that children be classified or labeled in order to receive services (20 U.S.C. 1412 (a) (3) (B)), the law (20 U.S.C. 1401 (3) (A)) does indicate that the child must have a disability and further defines that a disability means a child who has one of the following disorders and ‘‘who, by reason thereof, needs special education and related services.’’ Students may be found eligible based on the identification of one of the following disorders: Specific learning disability Emotional disturbance Mental retardation Autism Hearing impairment Visual impairment Speech or language impairment Other health impairment Traumatic brain injury Developmental delay (for children aged 5–9) Not only must the child be identified as having a disability but the disability must also have an adverse effect on child’s educational performance, and the child must need special education services in order to progress. A child with a disability who is making adequate educational progress without special services may not be found eligible. On the other hand, simply advancing from grade to grade without failing grades is not a clear evidence of educational progress, and a child may still be identified as a child with a disability even if he or she is not receiving failing grades. In order to develop an appropriate IEP, the team must review and consider multiple sources of information regarding the child’s functioning. Specific information may include results of the most recent school-based evaluations, as well as results of any outside evaluations completed at parent request and expense. These documents should provide specific information regarding the child’s unique strengths and weaknesses. In the event that the child’s behavior interferes with his or her learning, the use of positive behavioral interventions must be considered as well. The law also specifically requires that the child’s individual needs be identified, and goals developed to address these needs prior to the determination of the location of services or placement. Placement cannot be established simply based on the child’s diagnosis or category of eligibility. The law specifies (U.S.C. 1412 (a) (3) (B)) that children with disabilities be educated with

their nondisabled peers to the maximum extent possible. This is most typically a regular education classroom. The team may consider more restrictive placements if the child’s needs cannot be met in that setting, even with the provision of supplementary aids and services. Parents have specific rights regarding the IEP process, which are explained in a document often referred to as ‘‘procedural safeguards.’’ These guidelines spell out the specific time lines that must be met for various aspects of the process. Also described is the process for parents to follow when they disagree with the team’s decisions, steps that may include mediation and due process hearings. This document must be provided to parents at least annually, or upon request (34 C.F.R. 300.504 (a)).

Historical Background Advocacy for individuals with disabilities increased in the 1950s and 1960s. This led to the Congressional approval of the ‘‘Education for All Handicapped Children Act’’ of 1975 (PL 94-142). The purpose of this law was to assist states and local districts with ‘‘protecting the rights of, meeting the individual needs of, and improving the results for infants, toddlers, children and youths with disabilities and their families.’’ As part of this law, an IEP was mandated for all children with disabilities. PL 94-142 required schools to provide a ‘‘free and appropriate public education’’ in the ‘‘least restrictive environment’’ to children with a variety of disabilities. Since that time, the law has been expanded to include early intervention services for infants, special education programs for preschoolers, and parent training and information centers. The law has been most recently reauthorized in 2004 as the ‘‘Individuals with Disability Education Improvement Act of 2004 (IDEIA)’’.

Rationale or Underlying Theory Prior to the implementation of PL 94-142 the practice of excluding children with disabilities from the public school setting was quite common. It was suggested that this exclusion would lead to greater public expense in the long run, and that greater levels of inclusion would be more beneficial to all.

Goals and Objectives The primary goal of an IEP is to provide written documentation of the plan to assist the educational


team in working with a given child. As such, the document provides a ‘‘road map’’ for the provision of services. The IEP delineates the goals to be attained and the specific services and supports that are required to meet those goals. On a broader level, the purpose of the IEP is to ensure that the purposes of the law mandating its’ use are accomplished. Specifically, the law requires that ‘‘all children with disabilities have available to them a free and appropriate public education. . .designed to meet their unique needs and prepare them for further education, employment, and independent living’’ and to ensure that ‘‘the rights of children with disabilities and parents of such children are protected’’ (20 U.S.C. 1400 (d)).

Treatment Participants It is noteworthy that this information applies primarily to children attending public schools, as children attending private schools (by parent choice) may not receive the same special education services that they would receive if they were attending a public school (20 U.S.C. 1412 (a) (10)(A)(i)).

Treatment Procedures Components of IEP The IEP is mandated, under 20 U.S.C. 1414 (d) (1)(A)(i), to include the following information: 1. A statement of the child’s present levels of academic achievement and functional performance, including how the child’s disability affects his/her involvement in the general education curriculum. 2. A statement of measurable annual goals including academic and functional goals designed to meet the unique needs of the child as related to his/her disability, to enable the child to be involved in and make progress in the general education curriculum and to meet each of the child’s other needs that result from the child’s disability. 3. A description of how and when the child’s progress toward meeting these goals will be measured and reported. 4. A statement of the special education services, related services, and supplementary aids and services, based upon peer reviewed research to the extent practicable, to be provided to the child. 5. An explanation of the extent that the child will not participate with nondisabled peers in the regular lass.

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6. A statement of necessary accommodations for state and district assessments, and if the child will not participate in state and district assessments, a statement of why not, and why the selected alternate assessments are appropriate. 7. A date for the commencement of services as well as the frequency, duration, and location of services. 8. For children aged 16 and older, goals related to transition and the services required to reach these goals. Additional guidance for working with an individual child that could be incorporated within an IEP may include plans to address behavior issues, medical issues, or environmental management issues. The law requires that IEPs be reviewed annually. At each annual review, the specific goals are examined and updated. The team reviews the levels of service to ensure that the plan addresses the child’s current needs. The plan may also be reviewed at any time, on an as-needed basis, per the request of a team member.

Efficacy Information Since the inception of special education, there has been debate regarding the effectiveness of the services provided (e.g., Allinder, 1994; Carlberg & Kavale, 1980). The implementation of No Child Left Behind Act of 2001 (NCLB) led to changes in the provision of special education services. While the benefits of NCLB have also been debated at local and national levels, the law did provide greater definition of special education services, particularly those designed to address reading difficulties. NCLB provided a legal definition of reading, a description of essential components of reading instruction and reading assessment, and criteria for scientifically based reading research. Other aspects of academic instruction are not so clearly defined.

Outcome Measurement Provisions of NCLB mandate annual proficiency testing in math, reading, and science from grades 3 through 8. The goal of annual testing is to assist with the development of lessons, identify strengths and weaknesses, and to ensure that the needs of all children are met.

Qualifications of the Treatment Providers The requirements for the qualifications of all teachers, including special education teachers have been revised

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Individuals with Disabilities Education Act

and expended in accordance with the NCLB Act of 2001. While the criteria may vary for teachers of children at various age levels, overall, highly qualified teachers must meet all state requirements, hold full state certification, and a teacher’s license. Similarly, paraprofessionals must meet minimum standards as well (20 U.S.C. 6311). Moreover, parents are entitled to know the specific qualifications of their child’s teachers (http://old.nichcy. org/reauth/tb-qual-teachers.pdf).

Cross References ▶ Advocacy ▶ Individualized Education Plan ▶ Primary Handicapping Condition ▶ Secondary Handicapping Condition

References and Readings Allinder, R. M. (1994). The relationship between efficacy and the instructional practices of special education teachers and consultants. Teacher Education and Special Education, 17(2), 86–95. Bateman, B. D., & Herr, C. M. (2006). Writing measurable IEP goals and objectives. Verona, WI: Attainment Company. Carlberg, C., & Kavale, K. (1980). The efficacy of special versus regular class placement for exceptional children: A meta-analysis. The Journal of Special Education, 14(3), 295–309. Pierangelo, R., & Giuliani, G. (2007). Understanding, developing, and writing effective IEPs: A step by step guide for evaluators. Thousand Oaks, CA: Corwin Press. Wright, P. W. D., & Wright, P. D. (2007). Special education law (2nd ed.). Hartfield, VA: Harbor House Law Press. www.ed.gov/policy/speced/gvid/idea/tb-iep.pdf www.ldonline.org/indepth/iep www.nichcy.org/educated children/iep/pages/default.aspx www.wrightslaw.com/info/iep.index.htm

Individuals with Disabilities Education Act J ACOB T. LUTZ , DAVID E. M C I NTOSH Ball State University Muncie, IN, USA

Definition The Individuals with Disabilities Education Improvement Act of 2004 (IDEA, 2004) is the latest iteration of federal law, governing the provision of early intervention, special education, and related services. Title I of IDEA 2004 mandates that children (ages 3–21 years) identified as having a disability in one or more of 13 specific categories shall be granted access to free, appropriate public education in the least restrictive environment possible. Title I also stipulates the provision of early intervention services to infants and toddlers (birth until 2 years) who have, or are at risk of developmental disabilities.

Cross References ▶ 504 Plan ▶ Accommodations

References and Readings Individuals with Disabilities Education Improvement Act of 2004, 20 U.S.C. } 1400 et seq. U.S. Department of Education, IDEA website: http://idea.ed.gov/

Infantile Autism ▶ Autistic Disorder

Infantile Spasms ▶ West Syndrome

Infarction E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition Synonyms IDEIA; IDEA 2004; Public Law 108–446

An infarction is an area of tissue that has died as a result of insufficient oxygen to maintain its viability, usually

Inferior Parietal Area

caused by atherosclerosis giving rise to an obstruction of blood supply to the tissue. Coronary artery occlusion can cause myocardial infarction. Carotid or intracerebral artery occlusion can cause a stroke by cerebral infarction. Infarctions can also occur in the intestines, kidneys, spleen, or other organs by the same mechanism.

Cross References ▶ Atherosclerosis ▶ Cerebrovascular Disease ▶ Coronary Disease ▶ Embolism ▶ Ischemia ▶ Ischemic Stroke ▶ Myocardial Infarction ▶ Peripheral Vascular Disease ▶ Thrombosis

References and Readings Antman, E. M., et al. (2004). ACC/AHA Guidelines for the Management of Patients With ST-Elevation Myocardial Infarction A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Revise the 1999 Guidelines for the Management of Patients With Acute Myocardial Infarction). Circulation, 110, e82–e293.

Inferior Colliculi

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quadrigemina. The nuclei of the inferior colliculi are primarily concerned with auditory information. They receive extensive input from the lateral lemniscus, which carries auditory fibers from the superior olivary nuclei and the cochlear nuclei. Both of these latter nuclei receive direct input from the auditory system. In addition to sending fibers to the medial geniculates (the auditory integration and relay nuclei of the thalamus) via the inferior brachium of the inferior colliculus, each nucleus of the inferior colliculus has connections with the nucleus on the opposite side and to the superior colliculi. The roles of the inferior colliculi are not fully understood, but they are thought to be important in the localization of sound and in orienting responses, such as reflexively turning the eyes, head, and neck toward an unexpected or loud sound. The latter is likely accomplished by means of connections with the superior colliculi, which are, in turn, connected to the cervical cord via the tectospinal tract, innervating the muscles of the head and neck. Unilateral lesions of the nucleus of the inferior colliculus are not typically associated with observable deficits, with the possible exception of difficulties with sound localization.




M ARK M ENNEMEIER University of Arkansas for Medical Sciences Little Rock, AR, USA

Synonyms

Synonyms

Nuclei of the inferior colliculi

IPA; Inferior parietal cortex; Posterior parietal cortex

Inferior parietal lobule;

Definition Surface feature of the caudal-most pair of nuclear masses on the posterior surface (tectum) of the midbrain (mesencephalon).

Current Knowledge Together with the superior colliculi, which lie directly above, the inferior colliculi make up the corpora

Structure Parietal cortex occupies 20–25% of the surface area in human cerebral cortex (Mountcastle, 2005). Posterior parietal cortex (posterior to the posterior central gyrus) is divided anatomically into superior and inferior lobules/ areas (SPA and IPA, respectively) by the intraparietal sulcus. Several subdivisions can be distinguished within each area by their anatomical and functional properties

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(Husain & Nachev, 2007; Zigmond, Bloom, Landis, Roberts, & Squire, 1999). In general, the SPA serves functions related to somesthesis, tactile cognition, visually guided action, and intentions to move; whereas, the IPA serves functions related to both spatial and nonspatial cognition, attention, and awareness of the body and space (Husain & Nachev; Zigmond et al.). This entry focuses on structure, function, and illness of the IPA with contrasts made to the SPA. The IPA corresponds approximately to Brodmann’s areas (BA) 39 and 40 (Caspers et al., 2008). Upon gross inspection, the IPA is located in the lateral surface of the brain. The important anatomical structures surrounding the IPA include the intraparietal sulcus, the Sylvian fissure, and the postcentral sulcus. Gyral landmarks are the supramarginal gyrus (BA 40) and the angular gyrus (BA 39). The IPA extends from behind the lower part of the postcentral sulcus to the intraparietal sulcus. The anterior part is the supramarginal gyrus, which surrounds the parietal termination of the lateral fissure. Supramarginal gyrus borders the postcentral gyrus anteriorly and the superior temporal gyrus posteroinferiorly. The middle part is the angular gyrus, which caps the end of the superior temporal fissure. It arches over the superior temporal sulcus and continues posteroinferiorly with the middle temporal gyrus. The posterior part of the IPA arches over the end of the inferior temporal sulcus and borders the occipital lobe (Crosby, Humphrey, & Lauer, 1962). The supramarginal gyrus has rich associations from auditory, visual, and somesthetic association areas. Angular gyrus receives association fibers from and sends fibers to the adjacent parietal and occipital association areas. Long association pathways (the inferior occipitofrontal and the inferior longitudinal fasiculi) pass beneath and in close proximity to the supramarginal gyrus. These fiber pathways interconnect the occipital and parietal areas with regions of the frontal (the triangular and the opercular areas) and temporal corticies. Fascicles connecting auditory areas through the corpus callosum and visual radiations also underlie the supramarginal gyrus (Crosby, Humphrey, & Lauer). Cytoarchitectural descriptions of parietal cortex in humans and monkeys have been characterized by uncertainty and variety (Mountcastle, 2005). An observerindependent cytoarchitectonic analysis of the IPA of ten human brains (five male and five female) revealed seven cytoarchitectonic areas (Caspers et al., 2008). Five areas labeled PFop, PFt, PF, PFm, and PFcm cover the region of BA 40 on the supramarginal gyrus with extension into the depth of the Sylvian fissure. Two areas labeled PGa and PGp are located in BA 39 on the angular gyrus. In general,

the volumes of these areas do not differ between the two cerebral hemispheres; however, area PFcm may be larger in males than in females. Cytoarchitectionic areas were arranged in a topologically comparable pattern in all ten brains; however, variations in the position and extent of the areas could be observed both between individuals and between hemispheres of the same brain. Variation can be rather striking in some cases. For example, area PFcm is found in most cases in the upper bank of the Sylvian fissure. In some brains, however, PFcm extends onto the surface of the parietal operculum, and in others, it is buried entirely in the depths of the Sylvian fissure and not visible on the parietal surface. Importantly, none of the macroanatomical landmarks reliably marked a cytoarchitectonic border between any of the IPA areas. Both the variability in cytoarchitecture and its lack of correspondence with macroanatomy complicate attempts to infer functional deficits based on lesion location. It also compromises attempts to localize function from imaging studies.

Function Historically, the posterior parietal cortex was believed to be a sensory structure. Presently, functions of the IPA and temporoparietal junction are thought to involve multimodal sensory integration related to language, skilled movement, mathematical thought, and to spatial and nonspatial cognition, including attention and memory (Husain & Nachev, 2007; Mountcastle, 2005; Zigmond et al., 1999). The IPA is essential for a complete selfimage of the body (Zigmond et al.). In contrast, functions of the SPA and regions of the intraparietal sulcus involve sensory integration as it relates to motor control such as reaching, grasping, tactile exploration in space, oculomotor function, visually guided action, and intentions to perform movements (Anderson & Buneo, 2002; Mountcastle). A gradient of spatial to nonspatial functions has been proposed to extend from the SPA, through the intraparietal sulcus, and to the IPA, respectively (Husain & Nachev). Intermediate regions in the intraparietal sulcus may have both spatial and nonspatial functions. Functional imaging studies suggest that the IPA and regions of the ventral frontal cortex are normally activated when individuals perform nonspatial tasks, such as when subjects maintain vigilant attention and when they are required to select the target stimuli from among nontarget stimuli (Husain & Nachev, 2007). Further, these effects are observed across multiple sensory modalities and the functions appear to be independent of whether a spatial shift in attention or movement is required. In contrast, studies using neuroimaging demonstrate the SPA, regions of the


intraparietal sulcus, and the dorsolateral frontal cortex are activated during spatial shifts of attention and spatial working memory tasks (Corbetta & Shulman, 2002) and when performing saccadic eye movements or reaching for a visual target. One caveat related to functional activation of the parietal cortex, however, is that it may not be as task-specific as one might like to assume. Functional imaging has been useful in mapping cortical visual areas in the human occipital and temporal lobes (Culham & Kanwisher, 2001), but human parietal cortex (excluding, perhaps, somatosensory regions) provides challenges for neuroimaging because it represents a complex association cortex. The problem is that parietal activation can be very general. A review of neuroimaging of cognitive functions in human parietal cortex (Culham & Kanwisher) reports parietal activation in association with the following functions: (1) The intraparietal sulcal cortex is activated during visually guided grasping, reaching movements, and object matching. (2) The intraparietal sulcal cortex, the postcentral sulcal cortex, the SPA, and the IPA (including supramarginal gyrus and the temporoparietal junction) are activated in relation to ‘‘attention.’’ (3) Neural activity in the SPA, the intraparietal sulcal cortex, and in some cases the IPA increases as a function of attentional preparation. (4) Parietal cortex is activated by attentional processes independent of spatial components of the task or of eye movement. (5) Parietal activation has been reported for a wide range of tasks including motion processing, stereo vision, spatial and nonspatial working memory, mental imagery, mental rotation, response inhibition, task switching, alertness, calculation, and functions such as pain processing, swallowing, and meditation. Parietal activation in functional imaging studies may be generalized because it includes brain regions where many interrelated processes like attention, memory, and space representation converge (Culham & Kanwisher, 2001). Similar patterns of overlapping activation can be found in regions of the frontal lobes as well. Alternatively, the processes performed by parietal cortex may be so general that they are recruited by a wide range of tasks. Finally, imaging techniques cannot tell the difference between areas that are activated versus inhibited, and they may not possess the resolution necessary to capture small, localized activations of individual components within the parietal cortex.

Illness Damage to the IPA results in complex behavioral disorders involving disturbances in perception, attention, and

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movement that vary according to which hemisphere is damaged. Much of the understanding of IPA function comes from studies of patients with lesions involving this area. While it is unlikely that the lesions are completely restricted to the IPA in most cases, damage to this region is consistently associated with certain syndromes, disorders, and deficits. For example, damage to the IPA of the right hemisphere is associated with the neglect syndrome (▶ Neglect Syndrome). Neglect is a multifaceted neurological disorder involving deficits of spatial attention and movement that are often concentrated in space contralateral to the brain injury (Heilman, Watson, & Valenstein, 1985). Additionally, neglect also involves deficits in sustained attention, memory, and perception that are not necessarily worse in one spatial location (nonspatial deficits). Patients with neglect may also lack awareness of a disability (anosognosia), and sometimes, even deny ownership of a paretic limb (somatoparaphrenia). They may not show appropriate concern about their deficit or disability (anosodiaphoria). Lesions involving the IPA of the right hemisphere can also impair performance on neuropsychological testing of two- and threedimensional object construction and drawing, tactile recognition, spatial exploration and memory, and perception of line orientation (Costa & Spreen, 1985; Lezak, 1983). Damage to the superior marginal and angular gyrus of the left hemisphere is associated with Gerstmann’s syndrome (Costa & Spreen, 1985; Heilman & Valenstein, 1985). Gerstmann’s syndrome includes agnosia, agraphia, right–left confusion, and acalculia. While acalculia may also follow damage to the right hemisphere, it is the cooccurrence of symptoms in Gerstman’s syndrome that indicates damage to the left hemisphere. Certain forms of ideomotor apraxia and the inability to read single words (word blindness) are associated with damage to the posterior angular gyrus of the left hemisphere (Heilman & Valenstein; Mountcastle, 2005). Wernicke’s speech area includes parts of the IPA of the left hemisphere. Damage to these areas can give rise to disturbances of language. Unilateral damage to the IPA of the left and right hemispheres can also produce similar deficits. For example, neglect can follow damage to the IPA of the left hemisphere, though it is typically less severe and resolves more quickly than neglect after right hemisphere injury (Heilman, Watson, & Valenstein, 1985; Robertson & Halligan, 1999). Perception of stimulus intensity (magnitude estimation) may critically depend upon regions of the IPA in the right hemisphere (Walsh, 2003); however, homologous regions in the left hemisphere also process magnitude estimates, and lesions of the left hemisphere may also

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impair magnitude estimation (Pinel, Piazza, LeBihan, & Dehaene, 2004; Woods et al., 2006). Impairments of proprioceptive and tactile sensation on the contralateral side of the body have been associated with lesions to the posterior parietal cortex, which might involve the SPA as well as the IPA (Costa & Spreen, 1985). Sensation for temperature, pain, and pressure can be similarly impaired. Posterior parietal lobe lesions are associated with slowed perception of passive movement, raised thresholds for passive movement, and defective-point localization and two-point discrimination (Costa & Spreen). Bilateral damage to the IPA (and surrounding regions) is associated with Balint’s syndrome (Zigmond et al., 1999). Balint’s syndrome involves difficulty in executing eye movements to engage visual targets (psychic paralysis of gaze), inaccuracy in reaching for visual targets (optic ataxia), and a tendency not to see things in the peripheral visual field. Simultanagnosia is also described in association with Balint’s syndrome. Patients with simultanagnosia are unable to perceive multiple objects at the same time (piecemeal perception). For example, one patient with Balint’s syndrome and simultanagnosia reported seeing cows that were ‘‘floating’’ in the fields. She could recognize the animals from their heads and shoulders but could not perceive the lower bodies or legs. Balint’s syndrome reflects impairment of visually guided movement and spatial cognition involving the IPA, but may not be restricted to the IPA. In contrast, damage to the SPA and intraparietal sulcal cortex results in disorders of motor control leading to impaired grasping, optic ataxia, and visuomotor ataxia (Zigmond et al., 1999). Exploratory and manipulatory movements of the fingers may be impaired even with preserved elementary capacities for sensation and finger movement (Mountcastle, 2005). Impairments of oculomotor function may include slowness in foveating targets, difficulty in disengaging fixation, and altered smooth pursuit. The SPL and intraparietal sulcus contain maps of intentions related to eye, reaching, and grasping movements (Anderson & Buneo, 2002). Intentions are sophisticated and abstract preplans that specify the goal and type rather than the execution of movements. Patients with lesions involving the SPA exhibit poor visual guidance in reaching (optic ataxia) and difficulty in estimating the location of stimuli in 3D space rather than primary sensory or motor deficits. They exhibit a class of deficits characterized by the inability to plan movements (apraxias), ranging from an inability to follow simple commands to difficulty in executing sequences of movements. Deficits in grasping are evident, including problems shaping the hands to grasp objects.

Cross References ▶ Inferior Parietal Lobule ▶ The Neglect Syndrome

References and Readings Anderson, R. A., & Buneo, C. A. (2002). Intentional maps in posterior parietal cortex. Annual Review of Neuroscience, 25, 189–220. Caspers, S., Eickhoff, S., Geyer, S., Scheperjans, F., Mohlberg, H., Zilles, K., et al. (2008). The human inferior parietal lobule in stereotaxic space. Brain Structure and Function, 212, 481–495. Corbetta, M., & Shulman, G. L. (2002). Control of goal-directed and stimulus-driven attention in the brain. Nature Reviews Neuroscience, 3, 215–229. Costa, L., & Spreen, O. (1985). Studies in neuropsychology. Selected papers of Arthur Benton. New York: Oxford University Press. Crosby, E. C., Humphrey, T., & Lauer, E. W. (1962). Correlative anatomy of the nervous system. New York: The Macmillan Company. Culham, J. C., & Kanwisher, N. G. (2001). Neuroimaging of cognitive functions in human parietal cortex. Current Opinion in Neurobiology, 11, 157–163. Heilman, K. M., & Valenstein, E. (1985). Clinical neuropsychology. New York: Oxford University Press. Heilman, K. M., Watson, R. T., & Valenstein, E. (1985). Neglect and related disorders. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (2nd ed., pp. 243–293). New York: Oxford University Press. Husain, M., & Nachev, P. (2007). Space and the parietal cortex. Trends in Cognitive Sciences, 11, 30–36. Lezak, M. D. (1983). Neuropsychological assessment. New York, NY: Oxford University Press. McIntosh, R. D., & Milner, A. D. (2005). The neurological basis of visual neglect. Current Opinion in Neurology, 18, 748–753. Mountcastle, V. (2005). The sensory hand: Neural mechanisms of somatic sensation. Cambridge, MA: Harvard University Press. Pinel, P., Piazza, M., LeBihan, D. L., & Dehaene, S. (2004). Distributed and overlapping cerebral representations of number, size and luminance during comparative judgments. Neuron, 41, 983–993. Robertson, I. H., & Halligan, P. W. (1999). Spatial neglect: A clinical handbook for diagnosis and treatment. Hove, East Sussex: Lawrence Erlbaum Associates. Walsh, V. (2003). A theory of magnitude; common cortical metrics of time, space and quantity. Trends in Cognitive Neuroscience, 7, 483–488. Woods, A. J., Mennemeier, M., Garcia-Rill, E., Meythaler, J., Mark, V. W., Jewel, G. R., et al. (2006). Bias in magnitude estimation following left hemisphere injury. Neuropsychologia, 44, 1406–1412. Zigmond, M., Bloom, F. E., Landis, S. C., Roberts, J. L., & Squire, L. R. (1999). Fundamental neuroscience. San Diego, CA: Academic Press.

Inferior Parietal Cortex ▶ Inferior Parietal Area ▶ Inferior Parietal Lobule

Informant Questionnaire on Cognitive Decline in the Elderly

Inferior Parietal Lobule

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Inflammation

M ARK M ENNEMEIER University of Arkansas for Medical Sciences Little Rock, AR, USA

S USAN K. J OHNSON University of North Carolina-Charlotte Charlotte, NC, USA

Synonyms

Definition

Inferior parietal area; Inferior parietal cortex; Posterior parietal cortex

Inflammation is a process through which the immune system protects the body from infections and foreign substances such as bacteria and viruses. When inflammation occurs, chemicals from the white blood cells are released into the blood or affected tissues in an attempt to rid the body of pathogens. This release of chemicals increases the blood flow to the area and may result in redness and warmth. Some of the chemicals cause leakage of fluid into the tissues, resulting in swelling and pain. Inflammation is a nonspecific immune response which aids healing and is usually resolved acutely. However, inflammation is implicated in a number of conditions where the inflammatory response is chronic. Diseases generally associated with inflammation are rheumatoid arthritis, atherosclerosis, asthma, tendonitis, bursitis, polymyalgia rheumatica, and autoimmune disorders. There are a number of treatment options for inflammatory diseases including medications, rest and exercise, and surgery to correct joint damage.

Definition The IPL is comprised of heteromodal association cortex located on the lateral surface of the brain. It corresponds to Brodmann’s areas 39 and 40 and extends from behind the posterior, post central sulcus to the intraparietal sulcus (Caspers et al., 2008). The supramarginal gyrus (BA 40) makes up the anterior part of the IPL, and the angular gyrus (BA 39) makes up the middle and posterior parts. Damage to the IPL is associated with neuropsychological syndromes, disorders, and deficits including the neglect syndrome following right hemisphere injury, the Gerstmann’s syndrome following left hemisphere injury, and Balint’s syndrome following bilateral injury (Zigmond, Bloom, Landis, Roberts, & Squire, 1999).

Cross References ▶ The Neglect Syndrome

Inflicted Childhood Neurotrauma ▶ Shaken Baby Syndrome (SBS)

References and Readings Caspers, S., Eickhoff, S., Geyer, S., Scheperjans, F., Mohlberg, H., Zilles, K., et al. (2008). The human inferior parietal lobule in stereotaxic space. Brain Structure and Function, 212, 481–495. Zigmond, M., Bloom, F. E., Landis, S. C., Roberts, J. L., & Squire, L. R. (1999). Fundamental neuroscience. San Diego: Academic Press.

Infiltrative Diffuse Astrocytosis ▶ Gliomatosis Cerebri

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Informant Questionnaire on Cognitive Decline in the Elderly J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms IQCODE; Retrospective IQCODE; Short IQCODE

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Informant Questionnaire on Cognitive Decline in the Elderly

Description

Psychometric Data

The Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE) aims to assess cognitive decline in elderly participants. It is an informant-rated questionnaire, with respondents rating items such as ‘‘remembering things that have happened recently’’ and ‘‘making decisions on everyday matters,’’ as to whether the subject has improved or declined over the last 10 years, using a 5-point scale (‘‘much improved,’’ ‘‘a bit improved,’’ ‘‘not much change,’’ ‘‘a bit worse,’’ or ‘‘much worse’’). For this reason, the informant must have known the subject throughout the designated time period. The original version contained 26 items, but subsequently a 16-item short form was developed. The measure has been translated into many languages, including Chinese, Danish, Finnish, Persian, and Thai (short form only), French, Italian, Japanese, Korean, Norwegian, Polish, Portuguese, and Spanish (long form only), and Dutch, German, and Persian (both long and short forms). Cherbuin, Anstey, and Lipnicki (2008) stated that the IQCODE takes between 13 and 24 min to complete. The questionnaire is scored either in terms of total score, or total score divided by number of completed items in the event of missing items, leading to a score out of 5, corresponding with the response scale. Norms from an Australian sample of people aged 70–85 are available, but Jorm (2004) states that these are seldom used, with most studies using cutoff scores as a screening tool for dementia (see Section Clinical Uses).

In a review of the published literature on the IQCODE, Jorm (2004) provides a useful summary of the psychometric properties of the IQCODE. He reports that internal consistency of the measure is very high (seven studies reporting alpha values between 0.93 and 0.97), and test–retest reliability of 0.96 at 3 days and 0.75 at 1 year. Factor analyses have tended to find a first general factor that accounts for around 50% of the variance in scores, with subsequent factors accounting for 90%) is possible. This duration varies across individuals and the required perceptual tasks. One of the most common methods for studying inspection time is through use of visual masking during perceptual judgment. For example, a test may consist of presenting two visual patterns with the task requirement to determine if they are the same or different. The stimuli are presented for varying durations followed by a visual mask to interfere with subsequent short-term storage. In such a paradigm, inspection time is minimum exposure time that will consistently achieve the accuracy criteria. While a valuable way of assessing information processing speed capacity, this method tends not to be a standard part of clinical neuropsychological assessment at this point in time.

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IADLs

Definition Instrumental activities of daily living are a subset of activities of daily living (ADLs). They include activities that are required to live independently, such as cooking and shopping. They are contrasted with basic activities of daily living (bADLs), which include basic self-care functions such as eating and bathing.

Cross References ▶ Activities of Daily Living (ADLs) ▶ Basic Activities of Daily Living (BADLs)

References and Readings Katz, S. (1983). Assessing self-maintenance: Activities of daily living, mobility, and instrumental activities of daily living. Journal of the American Geriatric Society, 31, 331–341. Lawton, M. P., & Brody, E. M. (1969). Assessment of older people: Selfmaintaining and instrumental activities of daily living. Gerontologist, 9, 179–186.

Insula ▶ Insular Lobe

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Insular Cortex

Insular Cortex ▶ Insular Lobe

Insular Lobe M ELISSA J. M C G INN Virginia Commonwealth University School of Medicine Richmond, VA, USA

Synonyms Insula; Insular cortex; Island of reil

Intake ▶ Clinical Interview

Intake Interview ▶ Clinical Interview

Intellectual and Developmental Disabilities (IDD) ▶ Intellectual Disability

Definition Often considered to be the fifth lobe of the brain, the insular lobe lies deep within the Silvian fissure where it is concealed by the frontal, parietal, and temporal lobes. While the function of the insular lobe is not fully understood, it receives nociceptive and visceral sensory input and has been implicated in a variety of processes, ranging from autonomic control, taste perception, and pain processing to subjective emotional experience. Functional neuroimaging studies have also linked the insular lobe to conscious desires, cravings, and addiction as well as a variety of neuropsychiatric disorders, including schizophrenia, mood, panic, posttraumatic stress, and obsessive compulsive disorders.

Cross References ▶ Autonomic Nervous System ▶ Functional Imaging ▶ Sylvian Fissure

References and Readings Augustine, J. R. (1996). Circuitry and functional aspects of the insular lobe in primates including humans. Brain Research Reviews, 22, 229–244. Nagai, M., Kishi, K., & Kato, S. (2007). Insular cortex and neuropsychiatric disorders: A review of recent literature. European Psychiatry, 22, 387–394.

Intellectual Disability J AELYN R. FARRIS , J ODY S. N ICHOLSON J OHN G. B ORKOWSKI University of Notre Dame Notre Dame, IN, USA

Synonyms Developmental delay; Intellectual and developmental disabilities (IDD); Mental retardation

Definition Intellectual Disability is the currently preferred term for the condition historically referred to as Mental Retardation. Three decades ago, the American Association on Mental Retardation (AAMR; now known as the American Association on Intellectual and Developmental Disabilities or AAIDD) proposed a definition of Mental Retardation that emphasized intelligence but also considered two other important factors – adaptive behavior and the time of occurrence of the disabling condition: ‘‘Mental Retardation refers to significantly subaverage general intellectual functioning existing concurrently with deficits in adaptive behavior and manifested during the developmental period’’ (Grossman, 1983, p. 1).

Intellectual Disability

A change has occurred since the time of that definition, including a shift in terminology from Mental Retardation to Intellectual Disability (ID) as well as the designation of who should, and who should not, be classified as ID. The most recent definition defines the condition as a disability that originates before the age of 18 and ‘‘is characterized by significant limitations both in intellectual functioning and in adaptive behavior as expressed in conceptual, social, and practical adaptive skills’’ (Luckasson et al., 2002, p. 1). In essence, the refined lexicon represents a shift in terminology and yet encompasses the same population of individuals formerly referred to as individuals with mental retardation (Schalock et al., 2007). The major change, however, has to do with what is assumed to be the underlying cause of the disability. Whereas the 1992 AAMR definition of Mental Retardation viewed the disability as a defect within the person (i.e., an inherent deficit of the mind with genetic or biological underpinnings), the new definition of ID conceives the disorder as a lack of fit between the individual’s capacities and the context in which he or she functions (Wehmeyer et al., 2008). In other words, ID refers to a state of functioning rather than to an inherent disordered condition.

Categorization The concept of intelligence refers to a general mental capacity that involves a variety of cognitive abilities such as reasoning, problem solving, and abstract thinking. The quantification of intellectual functioning – as indexed through traditional assessment instruments such as the Bayley Scales of Infant and Toddler Development (Bayley, 2005), the Stanford-Binet Intelligence Scales (Roid, 2003), the Wechsler Intelligence Scale for Children (Wechsler, 2003), or the Kaufman Assessment Battery for Children (Kaufman and Kaufman, 2004) – has remained the first, and most salient, criterion of ID. Most often, intelligence is represented by a standardized intelligence quotient score (IQ), which is generally normed with a population mean of 100 and a standard deviation of 15. Limitations in intellectual functioning are assumed to be present if an individual has an IQ score of approximately two standard deviations below the population mean (i.e., 70 or below). The DSM-IV specifies four degrees of severity which reflect the level of intellectual impairment: Mild (IQ level 50–55 to approximately 70), Moderate (IQ level 35–40 to 50–55), Severe (IQ level 20–25 to 35–40), and Profound (IQ level below 20 or 25) (APA, 1994). Moreover, two additional criteria – significant limitations in adaptive behavior skills and evidence that the disability

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was present before age 18 – are required to make a diagnosis of ID (APA, 1994; Schalok et al., 2007; Wehmeyer et al., 2008). Adaptive behavior represents the conceptual, social, and practical skills that people use to function in their everyday lives. Adaptive behavior is typically assessed through standardized tests such as the Vineland Adaptive Behavior Scales (VABS; Sparrow, Balla, and Cicchetti, 1984; VABS-II; Sparrow, Cicchetti, and Balla, 2005), the Adaptive Behavior Assessment System (AABS-II; Harrison and Oakland, 2003), or the Scales of Independent Behavior (SIB-R; Bruininks, Woodcock, Weatherman, and Hill, 1996). Significant deficits are generally defined as performance that is at least two standard deviations below the mean of conceptual, social, practical, or overall adaptive skills. An individual who has pronounced deficits (i.e., two standard deviations below the population mean) in both IQ and adaptive behavior prior to age 18 meets criteria for ID. The most substantial changes resulting from recent modifications in the conceptual definition of ID emphasize the ideological and theoretical context in which intelligence is embedded, as well as a shift in emphasis from labeling to intervening. Rather than relying on IQ-based subgrouping (i.e., forming mild, moderate, severe, and profound categories), professionals are urged ‘‘to accompany diagnosis with descriptions of need supports’’ (Luckasson et al., 2002, p. 27) and, along with identifying deficits, to search for coexisting strengths in other psychosocial domains. Although the field of mental retardation is essentially left with traditional IQ tests that yield ‘‘objective’’ scores necessary to meet a specific diagnostic criterion, there is a sense, in both the 1992 AAMR and 2002 AAIDD revisions, that ‘‘something more’’ is needed in order to both identify and educate children and adolescents with intellectual disabilities. More contemporary perspectives emphasize the notion that a below average IQ score is only one criterion in determining whether an individual has ID.

Epidemiology According to the Fourth Edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV; APA, 1994), approximately 1% of the population in the USA meets criteria for Intellectual Disability. This represents a dramatic decrease in the overall prevalence from a range of 3–3.5% that was reported three decades ago. This change, however, is not a true indication of an overall reduction of ID (Zigler and Hodapp, 1986). Rather, recent

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definitional and diagnostic modifications have resulted in a shift in classification schemes in which many of the cases that were formerly placed into the Mild Mental Retardation category are now more often given alternative diagnoses, such as Learning Disorders. Although the overall prevalence rate has decreased, the remaining 1% of the population who meet criteria for ID are more likely to represent severe cases.

Natural History, Prognostic Factors, Outcomes The origins of ID can be been attributed to a variety of causal factors. The variables that predispose an individual to ID may be biological, psychosocial, or a combination of both (APA, 1994). Among individuals who present with ID in clinical settings, no clear etiology can be determined in approximately 30–40% of cases. In cases where contributing factors can be identified, major categories include heredity, early alterations of embryonic development, pregnancy and perinatal complications, general medical conditions acquired in infancy or childhood, environmental influences such as lead poisoning, and the occurrence of other mental disorders such as Autistic Disorder. The age of onset and progression of ID depend on the etiology and severity of the disability, although onset must occur by age 18 in order to meet diagnostic criteria. More severe deficits tend to be recognized early in life, especially when associated with a syndrome with a characteristic phenotype (e.g., ▶ Down Syndrome). In cases resulting from an acquired cause, such as neural damage resulting from encephalitis, the intellectual impairment might develop later and have a more abrupt onset.

Factors that Contribute to Intellectual Disability Heredity. Approximately 5% of individuals with ID develop this disorder due to heredity (APA, 1994). Examples include cases of ID associated with inborn errors of metabolism, which are often inherited through autosomal recessive mechanisms (e.g., Tay-Sachs Disease), other singlegene abnormalities with variable expression and Mendelian inheritance (e.g., tuberous sclerosis), and chromosomal aberrations (e.g., translocation Down Syndrome, Fragile X Syndrome). Early alterations of embryonic development. Approximately 30% of individuals with ID experience deficits that result from errors that occur during the embryonic period

(APA, 1994). For example, chromosomal changes (e.g., Down Syndrome due to Trisomy 21) or prenatal exposure to neurotoxins (e.g., alcohol, lead) can lead to cognitive and behavioral deficits. Pregnancy and perinatal problems. Approximately 10% of individuals with ID experienced challenges during the prenatal or perinatal period (APA, 1994). Examples include fetal malnutrition, premature birth, hypoxia, viral or other infections, and trauma. General medical conditions acquired in infancy or childhood. Approximately 5% of individuals with ID are born with typical cognitive and behavioral capacities but experience a medical condition in infancy or childhood that impairs their functioning (APA, 1994). Factors include infections, traumas, and poisoning. Environmental influences and other mental disorders. Approximately 15–20% of individuals with ID acquire their deficits through environmental influences or as a function of impairments associated with other mental disorders (APA, 1994). Examples of environmental influences that can contribute to ID include extreme deprivation of nurturance and severe neglect of social, linguistic, and/or other stimulation. Severe mental disorders may also contribute to ID due to the functional impairments resulting from the other disorder, such as the communicative and self-regulatory impairments that are common among individuals with Autistic Disorder. Multiple risk factors. Rather than considering only one contributor to ID, it is important to consider the interaction of multiple environmental, biological, and genetic conditions. For example, Phenylketonuria (PKU) is a genetic condition that results when the body is unable to metabolize phenylalanine, an amino acid present in many foods (Mayo Clinic, 2007). Individuals who are genetically predisposed to PKU are likely to develop severe deficits, including ID, if they consume foods containing phenylalanine, which are typical in most diets. However, if the genetic predisposition to PKU is detected at birth and care is taken to provide a lifelong diet free of phenylalanine, then the individual is likely to develop normally. Thus, PKU represents a case where genes and environment interact to contribute to individual variations in cognitive and behavioral development.

Developmental Course of Intellectual Disability The severity and course of ID are influenced by the course of underlying conditions as well as by environmental factors such as educational opportunities, environmental


stimulation, and appropriateness of care (APA, 1994). ID is not necessarily a lifelong disorder if appropriate identification and treatment are provided. For example, individuals who had intellectual deficits early in their lives may, with appropriate training and opportunities, develop compensatory skills in other domains that increase their level of adaptive functioning and, as a result, these individuals may no longer meet criteria for a diagnosis of ID.

Neuropsychology and Psychology of Intellectual Disability Intellectual Disability is a broad classification that can result from a variety of pathological processes (APA, 1994). As such, no specific personality and behavioral features are uniquely associated with ID. Individuals may, however, experience specific neuropsychological or psychological deficits associated with the underlying cause of their ID. In general, individuals with ID have an increased prevalence rate of comorbid mental disorders that is estimated to be three to four times greater than in the general population (APA, 1994). In some cases, this may result from shared etiology. For example, head trauma may result in ID and the comorbid diagnosis of Personality Change due to Head Trauma. Individuals with ID are at heightened risk for all types of mental disorders, and there is no evidence that the nature of a specific mental disorder is different in individuals who have ID. The most common mental disorders associated with ID include AttentionDeficit/Hyperactivity Disorder (ADHD), Mood Disorders such as Major Depressive Disorder, Pervasive Developmental Disorders such as Autistic Disorder, Stereotypic Movement Disorder, and mental disorders due to a general medical condition (e.g., Dementia due to Head Trauma). It is important to note that the diagnosis of comorbid mental disorders is often complicated because the clinical presentation of the comorbid disorder may be modified by the severity of the ID and associated deficits. For example, an individual with ID who has limited communicative abilities may not be able to express feelings such as hopelessness and helplessness that are associated with Major Depressive Disorder.

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to seek out clinical treatment (APA, 1994). Aside from individualized tests designed to assess IQ and adaptive behavior, there are no specific laboratory tests used in the assessment of ID, although diagnostic laboratory findings may be associated with accompanying medical conditions (e.g., chromosomal aberrations associated with various genetic conditions, high blood phenylalanine in PKU). Along these same lines, there are no specific physical features associated with ID; however, when ID is part of a specific syndrome, then the clinical features of that syndrome may be present (e.g., the physical features associated with Down Syndrome, such as low-set eyes that slope upward, a flattened nose bridge, and small low-set ears). Regardless of etiology, more severe cases of ID are associated with a greater likelihood of neurological, neuromuscular, visual, auditory, cardiovascular, and other conditions. Traditional, static approaches to intelligence testing are insufficient to fully evaluate the range of deficits associated with ID. Moreover, traditional assessments do not provide information that can be used to design potentially effective treatments (Haywood and Brown, 1990; Utley, Haywood, and Masters, 1992). A more contemporary and thorough approach to assessing ID focuses on ‘‘dynamic assessment.’’ Dynamic assessment yields three kinds of information: (1) ‘‘baseline’’ performance, (2) amount and type of help required to reach a higher level of performance, and (3) the individual’s response to that help (Haywood and Brown, 1990; Lidz, 1997). This approach sheds light upon children-as-learners, offers insight into the modifiability of cognitive skills (especially the extent to which children are capable of change in response to intervention), and assists clinicians in generating hypotheses about children’s learning potential and developing intervention strategies to improve functioning (Lidz and Pena, 1996). The addition of dynamic assessment compensates for the shortcomings of traditional assessments by explicitly addressing learning problems that otherwise might not be considered, thereby improving the clinician’s and teacher’s understanding of the child’s functional capabilities and limitations.

Treatment Evaluation Of the two defining characteristics of ID – intellectual deficits and lack of adaptive behavior capabilities – impairments in adaptive functioning are typically the symptoms that lead individuals with ID or their families

Problems in adaptive behavior are more likely to improve with treatment, whereas IQ tends to remain a stable characteristic (APA, 1994). However, specific skills associated with IQ, such as attention and executive functioning, can be improved through training. Adaptive

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functioning is more malleable given that these skills are influenced by the home environment, quality of education, motivation, personality characteristics, social and vocational opportunities, and comorbid mental and medical disorders. Creating goals and a cognitive behavioral treatment plan based on the principles of dynamic assessment are efficient ways to ensure optimal treatment that can help remediate the deficits associated with ID. If the disability is due to a specific condition or circumstance (e.g., Down Syndrome, lead poisoning), then additional treatment should be used in accord with medical guidelines for that condition.

Future Directions for Evaluation and Treatment Although new theories of intelligence, new approaches to assessments, and new definitional concerns with existing measures of IQ have all gained momentum during the past decade, the identification and treatment of ID has not followed suit, as evidenced by the field’s continued reliance on traditional, static, and non-contextualized assessments of intelligence. This lack of change in direction may be attributable to society’s ‘‘need’’ for a cut-off point that distinguishes individuals who meet, from those who do not meet, the major criterion associated with Intellectual Disability (i.e., IQ less than 70–75). This ‘‘need’’ is often related to practical issues, such as securing additional school funding, establishing eligibility for other entitlements, or influencing judicial consequences for individuals with diminished competence who are convicted of serious crimes (Reschly, Myers, and Hartel, 2002). It is unreasonable to expect the traditional approach to diagnosis to be completely supplanted by a new approach, at least overnight. Rather, what appears to be occurring is a phased-in process wherein traditional assessments are augmented with contextually based tests that are more useful for remedial education and the dayto-day instruction of children and adolescents with ID.

Acknowledgment The writing of this paper was supported, in part, by NIH training grant HD-07184.

Cross References ▶ Adaptive Behavior ▶ Disability

▶ Intelligence ▶ Intelligence Quotient

References and Readings American Association on Intellectual and Developmental Disabilities (AAIDD). (2007). Retrieved December 10, 2008 from http://www. aaidd.org American Psychiatric Association [APA]. (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: Author. Bayley, N. (2005). Bayley scales of infant and toddler development (BayleyIII) (3rd ed.). San Antonio, TX: Pearson. Bruininks, R. H., Woodcock, R. W., Weatherman, R. F., & Hill, B. K. (1996). Scales of independent behavior – Revised. Chicago, IL: Riverside Publishing. Grossman, H. (Ed.). (1983). Classification in mental retardation. Washington, DC: American Association on Mental Deficiency. Harrison, P. L., & Oakland, T. (2003). Adaptive behavior assessment system (2nd ed.). San Antonio: TX: The Psychological Corporation. Haywood, H. C., & Brown, A. L. (1990). Dynamic approaches to psychoeducational assessment. School Psychology Review, 19, 411–422. Kaufman, A. S., & Kaufman, N. L. (2004). Kaufman assessment battery for children (2nd ed.). San Antonio, TX: Pearson. Lidz, C. S. (1997). Dynamic assessment approaches. In D. P. Flanagan, J. L. Genshaft, & P. L. Harrison (Eds.), Contemporary intellectual assessment: Theories, tests, and issues (pp. 281–296). New York: Guildford Press. Lidz, C. S., & Pena, E. D. (1996). Dynamic assessment: The model, its relevance as a nonbiased approach, and its application to Latino American preschool children. Language, Speech, and Hearing Services in Schools, 27, 367–372. Luckasson, R., Borthwick-Duffy, S., Buntinx, W. H. E., Coulter, D. L., Craig, E. M., Reeve, A. et al. (2002). Mental retardation: Definition, classification, and systems of support (10th ed.). Washington, DC: American Association on Mental Retardation. Mayo Clinic. (2007). ‘‘Phenylketonuria (PKU).’’ Retrieved November 21, 2008 from http://www.mayoclinic.com/health/phenylketonuria/ DS00514 Reschly, D. J., Myers, T. G., & Hartel, C. R. (Eds.). (2002). Mental retardation: Determining eligibility for social security benefits. Washington DC: National Academy Press. Roid, G. (2003). Stanford-Binet intelligence scales (5th ed.). Chicago, IL: Riverside Publishing. Schalock, R. L., Luckasson, R. A., Shogren, K. A., Borthwick-Duffy, S., Bradley, V., Buntinx, W. H. E., et al. (2007). The renaming of mental retardation: Understanding the change to the term Intellectual Disability. Intellectual and Developmental Disabilities, 45, 116–124. Sparrow, S. S., Balla, D. A., & Cicchetti, D. V. (1984). Vineland adaptive behavior scales. Circle Pines, MN: American Guidance Service. Sparrow, S. S., Cicchetti, D. V., & Balla, D. A. (2005). Vineland adaptive behavior scales: (Vineland II), Survey interview form/caregiver rating form (2nd ed.). Livonia, MN: Pearson Assessments. Utley, C. A., Haywood, H. C., & Masters, J. C. (1992). Policy implications of psychological assessment of minority children. In H. C. Haywood & D. Tzuriel (Eds.), Interactive assessment (pp. 445–469). New York: Springer. Wechsler, D. (2003). WISC-IV administrative and scoring manual. San Antonio, TX: The Psychological Corporation.

Intelligence Wehmeyer, M. L., Buntinx, W. H. E., Lachapelle, Y., Luckasson, R. A., Schalock, R. L., Verdugo, M. A., et al. (2008). The intellectual disability construct and its relation to human functioning. Intellectual and Developmental Disabilities, 46, 311–318. Zigler, E., & Hodapp, R. M. (1986). Understanding mental retardation. New York: Cambridge University Press.

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as ‘‘the degree to which, and the rate at which, people are able to learn, and retain in long-term memory, the knowledge and skills that can be learned from the environment’’ (p. 44).


Intelligence Y VONNE H INDES 1, M IKE R. S CHOENBERG 2, D ONALD H. S AKLOFSKE 1 1 University of Calgary Calgary, Alberta, Canada 2 University of South Florida College of Medicine Tampa, FL, USA

Synonyms Emotional intelligence (EI); FSIQ; GAI; General cognitive ability; General cognitive functioning; IQ; PIQ; VIQ

Definition The term ‘‘intelligence’’ has been generally operationalized as a construct reflecting individual differences in cognitive abilities underlying various skills and behaviors such as educational and occupational success. However, the definition of ‘‘intelligence’’ and the abilities, aptitudes, and behaviors this construct includes has been a source of debate over the course of human history. Many definitions of intelligence have emerged over the years. For example, Binet (Binet & Simon, 1905) defined intelligence in terms of judgment, practical sense, initiative, and adaptability; whereas Wechsler (1958) later defined it as ‘‘the aggregate or global capacity of the individual to act purposefully, to think rationally, and to deal effectively with his/her environment’’ (p. 7). Moreover, intelligence was viewed by Wechsler as a composite of different abilities that is influenced by motivation and the manner in which the abilities combine together. Sternberg (1986) views intelligence as ‘‘the mental activity involved in purposive adaptation to, shaping of, and selection of real-world environments relevant to one’s life’’ (p. 33). Modern definitions have come to include metacognitive and executive processes and the interaction between knowledge and cognitive processes as well as the influence of culture. For example, Carroll (1997) describes IQ

A more recent description of intelligence has entered the psychological literature. While not new in some ways (e.g., Thorndike’s proposal of social intelligence and Gardner’s interpersonal and intrapersonal intelligence), the term emotional intelligence (EI) was more fully operationalized by Peter Salovey and John D. Mayer in 1990 as ‘‘the ability to monitor ones’ own and others’ feelings and emotions, to discriminate among them and to use this information to guide one’s thinking and actions.’’ The Salovey-MayerCaruso model reflects an ability based model with four basic abilities: (1) perceive emotions, (2) use emotions, (3) understand emotions, and (4) manage emotions. Another EI model, based on a so-called mixed model, is Daniel Goleman’s emotional competency model that consists of four main domains or constructs: (a) selfawareness, (b) self-management, (c) social awareness, and (d) relationship management. A second major mixed model of EI has been proposed by Reuven Bar-On, termed the Emotional-Social Intelligence model, which posits emotional intelligence (coined EQ) including emotionally understanding oneself and others, relating to other people, and adapting to and coping with immediate environment. These skills are thought to be malleable. These mixed models have more recently been classified as trait emotional intelligence reflecting a type of personality construct in contrast to a purely cognitive construct. While several measures have been developed to purportedly measure EI, the construct and its applicability to cognitive functioning are controversial, and readers are referred to the published works of Matthews, Roberts, and Zeidner (e.g., The Science of Emotional Intelligence, 2006).

Historical Background The suggestion of an underlying intelligence as a basis for describing individual differences has a long history. This section will focus on those developments that occurred just prior to and following the founding of psychological science. The discussion will review historical figures, associated theories of intelligence, and finally measures of intelligence.

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Intelligence

Francis Galton Much of the foundation for describing and assessing intelligence began in the late 1800s with the work of Francis Galton. His 1869 book Hereditary Genius focused on heritability of mental abilities. He believed intelligent individuals were those who had advanced sensory discrimination skills and, thus, developed tests that measured visual and auditory acuity, tactile sensitivity, and reaction time. However, these tests proved unsuccessful in measuring intelligence, reflected in observed differences, for example, in job success or academic achievement. Galton’s work spurred the development of the first psychological laboratory, which was founded by Wilhelm Wundt in Leipzig, Germany. Wundt’s colleague, James McKeen Cattell, was influenced by Galton. While the tests he developed did not provide valid or accurate measures of intelligence, Cattell contributed greatly to advancing the experimental and practical assessment of mental ability.

Alfred Binet Alfred Binet is often referred to as the ‘‘father’’ of contemporary intelligence testing. In contrast to Galton, he contended that higher mental processes were critical to describing intelligence. In an effort to develop techniques for the early identification of children who experienced significant academic and cognitive struggles, together with Theodore Simon, they developed the first practical intelligence test, the Binet–Simon Scale. This test consisted of tasks for measuring skills associated with reasoning and everyday functioning and problems (i.e., word definition, comparing lengths) at various developmental and difficulty levels. The test was revised in 1908 and again in 1911, and has since served as a framework for future intelligence tests.

Lewis Terman In 1916, the American psychologist, Lewis Terman, revised the Binet–Simon Scale for use in the USA. The new scale, which became known as the Stanford Revision and Extension of the Binet–Simon Scale, retained Binet and Simon’s practical and theoretical orientations, but contained supplemental tasks to provide a more thorough measurement of intelligence. The Revised Scale was later renamed the Stanford–Binet. Terman was also the first to quantify intelligence as a quotient (IQ), which allowed for comparison among individuals. Terman used Wilhelm

Stern’s formula for mental quotient (mental age divided by chronological age) in his calculation of IQ. The Stanford– Binet has undergone revisions and is currently in its fifth edition. During World War I, the US army used the Stanford–Binet test to screen and classify recruits. However, due to the cost and time demands associated with the administration of the test, a new approach to administration was required. In 1917, Robert Yerkes, Lewis Terman and 39 other psychologists developed two groupadministered intelligence tests, the Army Alpha and Army Beta Tests. The Beta Tests were developed in accordance with the Alpha Tests, but were used primarily with non-English speaking or illiterate individuals. These tests differed from the Stanford–Binet, not in theory or content, but in scale format. The Stanford–Binet utilized an age scale, such that test items were standardized on a group of individuals at different age levels. Yerkes did not agree with the age scale, and subsequently, developed a point scale in which points were assigned to responses based upon the correctness and quality of the answers. The net effect was the use of psychological testing during World War I, which spawned an increasing interest in psychological and cognitive testing for various selection purposes.

David Wechsler In 1939, David Wechsler developed the Wechsler–Bellevue test. The Wechsler–Bellevue test (▶ IQ and ▶ Wechsler Adult Intelligence Scales) incorporated aspects from many of the previous intelligence tests, but encompassed both verbal and nonverbal abilities. In 1949, the Wechsler Intelligence Scale for Children (WISC) was published followed in 1955, by the Wechsler Adult Intelligence Scale (WAIS). Later Wechsler also constructed a test for preschool-aged children, termed the Wechsler Preschool and Primary Scale of Intelligence (WPPSI). These Wechsler scales have undergone several revisions, but still contain some features and qualities of the original Wechsler scale.

Theory Intelligence and its measurement are grounded in two basic traditions. While some intelligence tests and other psychological measures are derived from factor analytic studies, other tests have been derived from theories of intelligence. A theory is neither right nor wrong but rather is evaluated by its usefulness. Thus, there are a number of

Intelligence

theories at present purporting to describe intelligence, most of which have resulted in the creation of tests reflecting the basic tenets of these various theories.

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upon his multifactor theory. Although these tests were not widely used then or today, his notion of measuring intelligence based on specific skills, rather than on a single general factor, has strongly influenced subsequent theories and intelligence tests.

Edward Thorndike’s Multifactor Theory Prior to the late 1920s, there was a somewhat pragmatic and philosophical approach to describing and assessing intelligence. The emphasis in test development was based on a practical rather than a theoretical orientation. However, in the 1920s Edward L. Thorndike developed a multifactor model of intelligence in which he postulated that intelligence consisted of three connected yet distinct abilities (social, concrete, and abstract intelligences).

Charles Spearman’s Two-Factor Theory In 1927, Charles Spearman was one of the first to begin the movement towards a more analytical approach to understanding intelligence. He employed the statistical techniques of correlation and factor analysis to determine the relationship between different intellectual abilities (e.g., vocabulary and spatial abilities). From this research, he concluded that intelligence consisted of two major factors or components: (1) a general ability important for performance on all types of mental tasks, and (2) special abilities required for only specific mental tasks. He suggested that both general ability and one or more specific abilities influence performance on intelligence tests. Spearman’s description of a general intellectual ability, which became known as g, has influenced many subsequent theories and tests of intelligence ranging from the Wechsler scales to the Stanford–Binet Tests.

Louis Thurstone’s Multidimensional Theory In 1938, the American psychologist, Louis L. Thurstone expanded Spearman’s concept of a g factor in a unique way. He saw intelligence not as a unitary trait, but as multifactorial, and g was considered a second-order factor. The mental abilities Thurstone believed were important to intelligence included: (1) verbal comprehension and word meaning, (2) word fluency and categorization, (3) speed and accuracy of arithmetic/ number computations, (4) spatial skills, (5) associative and rote memory, (6) perceptual speed, and (7) reasoning ability. Thurstone developed the Psychological Examinations and the Primary Mental Abilities batteries based

Raymond Cattell and John Horn’s Fluid and Crystallized Theory Raymond Cattell and John Horn hypothesized intelligence was composed of distinct but related factors and can be further divided into two major types: Fluid and Crystalized intelligence. Fluid intelligence, Gf, was described as primary reasoning or new learning abilities (e.g., nonverbal, culture-free mental operations). Crystalized intelligence, Gc, consists of acquired/factual knowledge and skills thought to be dependent on developmental level, educational experience, and culture (e.g., vocabulary and general information). Research has demonstrated that fluid intelligence decreases with age, whereas crystallized intelligence is more likely to hold with age, barring any assault to the brain, such as dementia or traumatic brain injury. Horn (1998) later expanded his theory to include seven additional abilities (visual processing, auditory processing, short-term memory, long-term memory, processing speed, decision speed, and quantitative knowledge).

J. P. Guilford’s Structure of Intellect Model J. P. Guilford argued that intelligence was comprised of multiple discrete abilities. He identified 120 abilities (and later proposed even more), and suggested these abilities combine in several ways to produce numerous unique types of intelligence. Guilford represented these abilities in a three-dimensional cube structure with operations involved in processing information, content, and products as the major dimensions. His approach to assessing intelligence stipulated that the assessment of each ability required a separate test. His theory has not received much empirical support.

Current Knowledge The development of theories and measures of intelligence to the present has not reflected revolutionary changes, but rather evolutionary advances in the study of intelligence and measurement leading to the current knowledge of intelligence. Included here are several hierarchical theories of intelligence that continue to impact both theory and

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measurement as well as other competing models. Some frequently used measures of intelligence will also be briefly described. Readers are also directed to the historical background to review theories of intelligence, which continue to influence intelligence testing today and are reflected in several current measures of intelligence.

Hierarchical Theories Philip E. Vernon and John B. Carroll believed in a multifactor approach to understanding intelligence; however, they also argued for a hierarchical approach. Vernon viewed intelligence as consisting of several abilities that exist at varying levels of generality. At the top of the hierarchy is g and at the bottom are specific abilities, similar to those identified by Spearman. In between g and the specific abilities are major factors (verbaleducation and spatial–mechanical), which are then further divided into minor factors such as verbal fluency and spatial skills. Vernon further elaborated on Cattell’s model by proposing that intelligence could be described at three levels. Intelligence A and B were derived of two categories, a biologically driven intelligence A and the interaction between the biological substrate and environment or intelligence, B. Intelligence A and B were not measureable as the first was not static, and the other was confounded by too many variables. Intelligence C was that component of intelligence measured by intelligence tests and was thus a marker for Intelligence A and/ or B. John Carroll (1997) proposed a three-stratum model based on an extensive examination of the research literature. The first level of Carroll’s model consists of 65 narrow cognitive abilities (e.g., reading comprehension and numerical facility). The second stratum includes eight broad factors: (1) fluid intelligence, (2) crystallized intelligence, (3) general memory and learning, (4) broad visual perception, (5) broad auditory perception, (6) broad retrieval capacity, (7) broad cognitive speediness, and (8) processing/decision speed. The third stratum is the general factor or g.

creative. This theory combines personality with intelligence, which is seen by some as a limitation.

Howard Gardner’s Multiple Intelligence Theory Howard Earl Gardner also supports the view that there are multiple types of intelligence. He originally described seven types of intelligence independent of one another, but these seven can be combined in multiple ways to reflect an individual’s personal strengths and weaknesses. These intelligences include: (1) verbal/linguistic, (2) logical–mathematical, (3) musical, (4) spatial, (5) bodily-kinesthetic, (6) interpersonal, and (7) intrapersonal. An eighth intelligence, naturalistic, has been subsequently described. More recently three other intelligences, spiritual, existential, and moral have been proposed, but their descriptions are fraught with problems, and have not been considered intelligences in, and of themselves.

Jean Piaget’s Developmental Theory Jean Piaget described intelligence as biological adaptation to one’s environment. His theory divides cognitive development into four developmental stages. The first stage, which is typically from birth to 2 years of age, called the Sensorimotor stage, is characterized by the differentiation of self from objects, recognition of self as an agent of action, intentional actions, and object permanence. The second stage, from 2 to 7 years old, is the Preoperational stage. During this stage, children think egocentrically, classify objects by a single factor and develop language and memory. The third stage, from 7 to 11 years old, is the Concrete Operations stage where children’s thinking is concrete, logical, and systematic. The last stage, which occurs at the age of 11 years and above, is the Formal Operations stage. This stage is characterized by the child’s capacity to think abstractly, construct theories, and make logical deductions. Currently, there are no intelligence tests based on Piaget’s developmental theory.

Robert Sternberg’s Triarchic Theory Cattell-Horn-Carroll Theory (CHC) Robert Sternberg argues that there are different types of intelligence. The emphasis of his triarchic theory is the relationship between intelligence and experience and the external and internal worlds. He has described three types of intelligences: (1) analytic, (2) practical, and (3)

Raymond Cattell and John Horn, argued against a general g construct accounting for all intelligence, and developed the Cattell–Horn theory of intelligence, describing two broad constructs termed fluid and crystallized

Intelligence

intelligence (Gf–Gc), which have different trajectories over the course of a person’s life. Fluid and crystallized intelligences were compromised of multiple (up to 100) different abilities. Fluid (Gf) intelligences reflect an individual’s ability to think and act quickly and is physiologically based. Alternatively, crystallized intelligences (Gc) includes abilities developed from learning and culturalization. The Gf–GC theory incorporates theoretical and empirical factors of intelligence, and thus provides a more comprehensive and empirical understanding of intelligence. Many experts combine the work from John Carrol’s three stratum theory with the Gf–Gc theory of Cattell and Horn, into the Cattell-Horn-Carroll (CHC) theory of intelligence. The theories of Cattell and Horn and John Carroll are similar, allowing integration, except that Carroll also argues for a central level of intelligence, Stratum III, which is more similar to Spearman’s g factor.

Modern Theories Modern theories generally support the structure of intelligence as being more than an undifferentiated single factor but further attempt to integrate biological, environmental cognitive, personality, and motivational factors. These theories have increased the understanding of the complex nature of intelligence and consequently, no single test exists that measures all the abilities associated with intelligence nor does an intelligence test portray the variability in human behavior. The recent work of such eminent psychologists as Ian Deary, Phil Ackerman and Linda Gottfredson are examples of contemporary perspectives on intelligence.

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working memory, and processing speed. The WISC-IV is complemented by the WISC-IV Integrated that can be used to add to the breadth of assessment by taping into more specific cognitive processes to better understand test performance. The current Wechsler family of tests also includes the Wechsler Preschool and Primary Scale of Intelligence- Third Edition (WPPSI-III), used in the assessment of cognitive abilities of young children, ages 2.6–7.3 years. The Wechsler Abbreviated Scale of Intelligence (WASI), was developed to provide a tool which provides a more rapid assessment of intelligence for individuals between 6 to 89 years. The Wechsler Nonverbal Scale of Ability (WNV) can be used with individuals (4– 21 years) that are non-English speaking or have other language barriers. The WNV provides a measure of intelligence designed to be independent of language/verbal skills. The Wechsler intelligence scales are also linked with a number of other measures that enhance the clinical assessment process, including the Wechsler Memory Scale (WMS-IV) and the Wechsler Individual Achievement Test (WIAT-II).

Stanford–Binet Intelligence Scales- Fifth Edition (SB5) The SB5 is the most current edition of the Stanford–Binet intelligence test that was introduced in Paris, France, by Binet and Simon in 1905 and later in the USA by Terman. The current, fifth edition evaluates fluid reasoning, knowledge, quantitative reasoning, visual–spatial processing, and working memory for individuals ranging in age from 2 to 85+ years old. It includes an overall IQ as well as domain quotient scores.

Current Tests of Intelligence A large number of intelligence tests have been developed since the original Binet tests developed at the start of the twentieth century. To follow is a description of some of the most often used current intelligence tests.

Wechsler Scales The first intelligence test developed by David Wechsler appeared in 1939. The current versions are among the most frequently used tests for assessing intelligence across the age range. The child (WISC-IV; 6–16 years) and adult (WAIS-IV; 16–90 years) tests yield a full scale intelligence quotient (FSIQ) as well as individual index scores assessing verbal comprehension, perceptual reasoning,

Differential Ability Scales- Second Edition (DAS-II) The DAS-II authored by Colin Elliott, did not evolve from one particular theory of intelligence, but instead integrated aspects from numerous theories of cognitive development. It has a hierarchical organization of cognitive abilities essential to learning that combines into an overall score. It measures the same cognitive abilities as the Wechsler Scales, but also examines visual working memory, recall ability, phonological processing, and understanding basic number concepts. The DAS-II can be administered to children aged 2 years 6 months to 17 years 11 months.

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Intelligence. Table 1 Pros and cons of intelligence and intelligence testing Pro

Con

Predictive of academic success

Can be culturally biased

Useful in identifying cognitive strengths and weaknesses, which can be used to guide educational planning, placements, and interventions

Often they do not measure underlying processes or everyday functioning/real-life situations

Standardization allows for performance comparison

Do not measure the entire domain; instead they assess only a select few abilities

Evaluate individual differences

Results can result in stereotypical categorization

Used in conjunction with other measures to provide a comprehensive and diagnostic assessment

Scores over-interpreted to diagnose presence of central nervous system (brain) dysfunction and such diagnostic classifications such as Learning Disabilities and Attention Deficit/Hyperactivity Disorder

Woodcock Johnson Tests of Cognitive Abilities – Third Edition (WJ-III COG) The WJ-III COG is another commonly used test of intelligence based upon the Cattell-Horn-Carroll (CHC) theory of cognitive abilities. The WJ-III measures various cognitive abilities of 2- to 90-years-olds. It utilizes a multifactor approach and identifies four major factors: Comprehension-Knowledge, Fluid Reasoning, Short-Term Memory, and Processing Speed. These factors are further broken down into more specific abilities.

The Good and Bad of Intelligence Testing The very idea of intelligence, the tests derived to measure intelligence, and the use of intelligence tests in assessing individual differences in schools and other settings (e.g., military) has been widely debated, and the controversy remains even today. Table 1 highlights some of the many pros and cons of intelligence testing.

areas of the brain are involved in various abilities and solving different types of problems, not only for more traditional verbal and visuospatial, but also for moral and social problems (e.g., EI). Studies from fields such as behavior genetics and cross-cultural psychology have given new insights into the ‘‘causes’’ of intelligence. The Flynn Effect that has described increases in the measured intelligence of people in developed countries has also reopened research into the various conditions that can positively or negatively impact cognitive abilities. Furthermore, there is a greater integration of intelligence, personality, conative (e.g., motivation, self-efficacy), and external factors such as home environments that have provided a much greater understanding of variability in human behavior and contributed to improved psychological assessment. Finally, the area of emotional intelligence continues to receive the attention of psychologists.

Cross References Future Directions The construct of intelligence continues to be an area of active research in neuropsychology, psychology, and the neurosciences in general. Magnetic Resonance Imaging (MRI) studies are beginning to provide valuable information about the neuroanatomical correlates of intelligence. For example, research is using MRIs to investigate the relationship between brain activity and intelligence. Moreover, functional MRI techniques and/or magnetoencephalography may provide insight into what specific

▶ Crystallized Intelligence ▶ Differential Ability Scales (DAS and DAS-II) Flynn Effect ▶ Intelligence Quotient ▶ Stanford Binet Intelligence Scales and Revised Versions ▶ Wechsler Adult Intelligence Scale (All Versions) ▶ Wechsler Adult Intelligence Tests ▶ Wechsler Intelligence Scale for Children ▶ Wechsler Preschool and Primary Scale of Intelligence ▶ Woodcock-Johnson Cognitive-Achievement Battery

Intelligence Quotient

References and Readings Binet, A., & Simon, T. (1905). Me´thode nouvelle pour le diagnostic du niveau intellectuel des anormaux. L’Anneé Psychologique, 11, 191–244. Carroll, J. B. (1997). Psychometrics, intelligence, and public perception. Intelligence, 24, 25–52. Deary, I. J. (2001). Intelligence: A very short introduction. Oxford: Oxford University Press. Flynn, J. R. (2007). What is intelligence? Beyond the Flynn effect. Cambridge: Cambridge University Press. Gottfredson, L. S. (2008). Of what value is intelligence? In A. Prifitera, D. H. Saklofske, & L. G. Weiss (Eds.), WISC-IV applications for clinical assessment and intervention Amsterdam: Elsevier. Horn, J. L. (1998). A basis for research on age differences in cognitive capabilities. In J. J. McArdle & R. W. Woodcock (Eds.), Human cognitive abilities in theory and practice (pp. 57–87). Mahwah, NJ: Erlbaum. Sattler, J. (2008). Assessment of children: Cognitive foundations La Mesa, CA: Author. Sternberg, R. J. (1986). Intelligence applied: Understanding and increasing your intellectual skills. New York: Harcourt Brace Jovanovich. Sternberg, R. J. (2007). Wisdom, intelligence, and creativity synthesized. New York: Cambridge University Press. Sternberg, R. J., & Grigorenko, E. L. (2000). Teaching for successful intelligence. Arlington Heights, IL: Skylight. Wechsler, D. (1958). The measurement and appraisal of adult intelligence (3rd Ed.). Baltimore, MD: Williams and Wilkins.

Intelligence Quotient DAVID N ORSTOKKE 1, D ONALD H. S AKLOFSKE 2 M IKE R. S CHOENBERG 2 1 University of Calgary Calgary, Canada 2 University of South Florida College of Medicine Tampa, FL, USA

Synonyms

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lower) than the average or typical scores of their peers. There are numerous intelligence tests and various definitions of intelligence, so while the IQ gleaned from a test is akin to a ‘‘score’’ on that test, the interpretation and meaning may vary from test to test (▶ intelligence).

Historical Background The Foundations for the IQ Score In 1884, Galton measured large numbers of people in an attempt to develop a test of intelligence. He measured many qualities in people, such as head size, reaction time, and strength of grip to try to uncover the key components of intelligence. Through his research, he introduced methods for the numerical classification of physical, physiological, and mental attributes. Galton proposed that a large set of measurements of human traits could be meaningfully described and summarized using two numbers: the average value of the distribution (the mean), and the dispersion of scores around that average value (the standard deviation). In addition, Galton was a pioneer in the area of correlational methods, which are central to many modern techniques for investigating the reliability and validity of tests, as well as factor analytic techniques (Schultz & Schultz, 1992). Another contributor to the methods of intellectual testing was Charles Spearman. One of Spearman’s most notable contributions to intelligence testing is the idea that all aspects of intelligence, to a certain extent, are correlated with each other. This is a very important viewpoint, as it sets the stage for finding a method of expressing the IQ composite. Spearman also contributed greatly to the development of factor analytic techniques, which are central to intelligence test development and the representation of intelligence measures.

FSIQ; IQ

The First Intelligence Tests and Introduction of the IQ Score

Definition The IQ (intelligence quotient) is a quantitative or statistical representation of an individual’s score on a standardized intelligence test. The IQ score has been widely utilized to compare an individual’s intellectual ability with the average score obtained by a sample of ‘‘similar’’ people, usually of the same age group. Thus for example, it is possible to state that a person’s intelligence, as reflected in an IQ test score, is higher (or

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The modern era of intelligence testing began just after the turn of the twentieth century. Alfred Binet and Theodore Simon were commissioned by the Paris schools to develop a method for differentiating between children who were considered intellectually capable of benefiting from schooling and those who were intellectually challenged and who should receive special education programming. The methodology was to express a child’s performance as a ratio of the child’s score against the age at which the

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average child would be able to attain the same score. It was the publication of the expanded 1908 Binet scale and the work of Stern, the German psychologist, that led to the concept of mental age (MA). An MA of 8 meant that the child, regardless of actual age, performed like a typical (average) 8-year-old on a particular task. This eventually led to the creation of the IQ score to represent a ratio of MA divided by their chronological age (CA). This ratio was put into a metric, such that one’s mental age was divided by their chronological age, and multiplied by 100, yielding an intelligence quotient or IQ score. Around 1910, Henry Goddard, director for a New Jersey school for the mentally retarded, pioneered the concept of IQ testing in the United States. However, the first time the IQ scores were made part of an intelligence test in the United States was in 1916, when Lewis Terman translated the Binet–Simon test into the Stanford–Binet. The original scoring methodology was modified so that the ‘‘average’’ child would get a score of a 100 at each age (mental age equal to chronological age) with the spread of scores or standard deviation set at 15 IQ points (Gould, 1981). For example, a child with a mental age of 6 and a chronological age of 5 would have an IQ of MA/ CA 100 or 6/5 100 = 120. During World War I, the U.S. army developed the Army Alpha and Beta tests for assigning soldiers’ positions based on their intellectual abilities, and also to identify those who were deemed intellectually unfit for military service. During that time, David Wechsler was assigned as a Military Psychologist conducting psychological examinations on individuals who had failed the army performance scale (Boake, 2002).

sample of healthy individuals at specified age groups. As a result, the meaning of the IQ score was changed from a mental age–chronological age ratio score into a standard score referenced to the average score obtained by a sample of healthy age-matched peers (Boake, 2002). The new calculation for IQ in which the total test score for an individual was divided by the average score obtained by people of the same age yielded a quotient that is more constant throughout life, and therefore can be used across the entire life-span (Bartholomew, 2004). The Wechsler–Bellevue Form 1 was published in 1939 as a measure of intelligence based on summarizing the scores from a number of subtests. In addition to a composite summary, termed Full Scale IQ, Wechsler argued that intelligence could be even more precisely measured by dividing the subtests into those primarily reflecting Verbal abilities versus those skills reflecting nonverbal, or ‘‘performance’’ intelligence abilities, giving rise to the Verbal IQ (VIQ) score and the Performance IQ (PIQ) score. Wechsler’s tests and recommendation for describing intelligence were successful, and he then developed an adult test, the WAIS (Wechsler, 1955) that was a direct off-shoot of the Wechsler–Bellevue test and also, the children’s version (WISC) published in 1949. These tests are now in their fourth revisions and widely used in modern intelligence testing. While they both currently yield an FSIQ, the VIQ–PIQ have been replaced by more factor analytically derived index scores associated with verbal, visuoperceptual/spatial reasoning, processing speed, and working memory skills (still represented by an average score of 100 and standard deviation of 15).

Current Knowledge Wechsler’s Tests and the IQ Score In 1932, Wechsler was appointed as the chief psychologist of the Bellevue Psychiatric Hospital in New York. His view of intelligence testing was based on two assumptions. The first assumption was that what we call human capacities may be treated as physical or psychological qualities, and the second was that these qualities were capable of measurement (Wechsler, 1935). He concluded that there was need for an alternative test to the Binet test, and in particular, a test of intelligence most suitable for use with adults. Wechsler also believed that the ratio of mental age/ chronological age was inappropriate for adults because mental age did not increase at the same rate as chronological age in adulthood. Thus, Wechsler proposed to use the sum of subtest scores to create a standard score based on the mean (average score) and standard deviation for a

Does an IQ score truly and accurately represent someone’s intelligence? IQ tests provide an estimate of the construct defined as ‘‘intelligence’’ noted above (▶ intelligence), but neither are IQ scores ‘‘perfect’’ measures, nor do IQ scores capture the full range of abilities that refer to a person’s intellectual abilities. The use of IQ as a measure and representation of one’s intellectual capacity has been challenged by some researchers and some have gone as far as to call for the abandonment of the IQ score because of the potential harm that can be caused by the labels associated with test results. However, as pointed out by Wechsler (1974) when discussing the IQ score, " All comparisons, of course, are odious, and comparing

people’s intelligence with one another, particularly so. Too much is at stake. . . education, a job, in certain situations one’s legal rights may be at stake. One must


obviously be careful as to how one interprets as well as one arrives at an IQ. It is not, however, an inherent fault of the IQ that incompetent or mischievous people misuse it (Wechsler, 1974; p. 53).

It has been argued that biases in IQ tests may disadvantage certain sub-groups in the United States (e.g. non-native language speakers, cultural and ethnic minorities) who may attain lower scores when compared with more advantaged groups (e.g., greater educational opportunities). The performance on at least some subtests used to derive IQ scores can be impacted by cultural and educational factors indicating IQ tests are not universal, but relate to, and reflect, the context in which they are employed. At the same time, intelligence and its structure appears to be universal construct, the issue lies more with how it is measured (IQ test and the IQ score) and the potential for bias in the tests. As a strict criterion measure, the IQ score can be an indicator of intellectual difficulties that is useful for identifying those individuals who deviate from ‘‘normal’’ intellectual capacities on either end of the IQ spectrum. The IQ is, in the final analysis, a quantitative summary of a person’s performance on an intelligence test and placed into a statistical framework that allows for the comparison of scores between individuals. It in no way tells about the ‘‘causes’’ of intelligence, but does correlate highly with a person’s performance in other areas such as school achievement and job success. Thus, the IQ score, obtained from an intelligence test with excellent psychometric properties, can provide a reliable measure of cognitive and behavioral attributes operationalized as intelligence.

IQ Scores and What They Mean The general tradition for describing IQ scores is to assign a mean of 100 and SD of 15 and then follow the distribution of the normal curve. Approximately 68% of the population will have IQ scores ranging between 85 and 115; another 14% will fall between 70 and 85 as well as 115–130. An IQ of below 70, representing slightly more than 2% of the population, indicates limitations in intellectual ability and the general category of ‘‘mental retardation’’ ranging from more severe mental retardation to mild intellectual impairment. Scores between 70 and 80 indicate ‘‘borderline’’ ability, and scores between 80 and 89 represent someone who is ‘‘low average.’’ An IQ score of 100 represents ‘‘normal’’ intelligence for the specific age group; IQ scores between 90 and 110 are considered within the acceptable range for ‘‘normal.’’ Scores between 110 and 120 are associated with ‘‘high average’’

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intellectual functioning, and scores between 120 and 130 designate ‘‘superior’’ intellectual functioning. An IQ score above 130, again found in about 2% of the population, indicates ‘‘very superior’’ intelligence. However, it is not necessary for the IQ score to have a mean of 100 and SD of 15, and, as an example, the Wechsler Adult Intelligence Scale – 3rd Edition (WAISIII; Wechsler, 1997) utilizes T-scores (mean of 50 and SD of 10) to display IQ scores that are adjusted for age and demographics. The IQ scores for the Reynolds Intellectual Assessment Scales (RIAS, Reynolds & Kamphaus, 2003) include Verbal Intelligence Index (VIX), Nonverbal Intelligence Index (NIX), and a Composite Intelligence Index (CIX) also using a mean of 50 and a SD of 10.

Future Directions The more recently published tests such as the Stanford Binet, Woodcock–Johnson Cognitive Battery, Differential Ability Scales, and the Wechsler tests all showcase changes in how intelligence tests are scored and the results presented. For example, the Wechsler Adult Intelligence Test – 4th Edition (WAIS-IV, Wechsler, 2008) and Wechsler Intelligence Scale for Children – 4th Edition (WISC-IV, Wechsler, 2003) are illustrative that IQ scores are changing in terms of what behaviors and skills are being measured by the index score. The previous measures of performance IQ (PIQ) and verbal IQ (VIQ) have given way to index scores of ‘‘IQ’’ that are defined as ‘‘Perceptual Reasoning Index’’ and ‘‘Verbal Comprehension Index.’’ In addition, advancements in IQ reflect the development of the General Ability Index (GAI). The GAI yields a measure of general cognitive ability, which differs from a traditional IQ score, and is a composite of cognitive functions thought to measure ‘‘crystallized and fluid intelligences’’ while minimizing demands on processing speed and working memory. The assessment of intelligence and cognitive processes, like all other latent traits that describe individual differences in human behavior, will continue to evolve. Intelligence and its measurement is one of the most important and well-studied areas in psychology and related disciplines. While the theories, definitions, and measurement techniques will certainly drive this growth, the need to quantify the ‘‘results’’ will remain, whether using the traditional IQ score or some other variant or metric.

Cross References ▶ Differential Ability Scales (DAS, DAS-II) ▶ Intelligence

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▶ Stanford Binet Intelligence Scales and Revised Versions ▶ Wechsler Adult Intelligence Scales (All Versions) ▶ Wechsler Intelligence Scale for Children ▶ Woodcock–Johnson Cognitive-Achievement Battery

References and Readings Bartholomew, D. J. (2004). Measuring intelligence: Facts and fallacies. New York: Cambridge University Press. Boake, C. (2002). From the Binet-Simon to the Wechsler-Bellevue: Tracing the history of intelligence testing. Journal of Clinical and Experimental Neuropsychology, 24(3), 383–405. Georgas, J., Weiss, L. G., van de Vijver, F. J. R., & Saklofske, D. H. (2003). Culture and children’s intelligence: Cross-cultural analysis of the WISC-III. San Diego, CA: Academic. Gould, S. J. (1981). The Mismeasure of Man. New York: Norton. Reynolds, C. R., & Kamphaus, R. W. (2003). RIAS: Reynolds Intellectual Assessment Scales. Lutz, FL: Psychological Assessment Resources, Inc. Sattler, J. (2008). Assessment of children: Cognitive Foundations (5th ed.). San Diego, CA: Sattler Press. Schultz, D. P., & Schultz, S. E. (1992). A History of Modern Psychology (5th Ed.). San Diego: Harcourt Brace Javanovich. Tulsky, D. S., Saklofske, D. H., Chelune, G. J., et al. (2003). Clinical interpretation of the WAIS-III and WMS-III. San Diego, CA: Academic. Tulsky, D. S., Saklofske, D. H., Wilkins, C., & Weiss, L. G. (2001). Development of a general ability index for the Wechsler Adult Intelligence Scale-Third Edition. Psychological Assessment, 13, 566–571. Wechsler, D. (1935). The concept of mental deficiency in theory and practice. Psychiatric Quarterly, 9, 232–236. Wechsler, D. (1955). The Wechsler Adult Intelligence Scale. New York: Psychological Corporation. Wechsler, D. (1974). Intelligence: Definition, theory, and the IQ. In A. J. Edwards (Ed.), Selected papers of David Wechsler. New York: Academic. Wechsler, D. (1997). Wechsler Adult Intelligence Scale-third edition. San Antonio, TX: The Psychological Corporation. Wechsler, D. (2003). Wechsler Intelligence Scale for children-fourth edition. San Antonio, TX: The Psychological Corporation. Wechsler, D. (2008). Wechsler Adult Intelligence Scale-fourth edition. San Antonio, TX: The Psychological Corporation.

Intensity-Modulated Radiation Therapy

the tumor using computer-controlled delivery of x-ray radiation. Damage to surrounding brain tissue is thus minimized and side effects tend to be fewer and less severe. Due to its high degree of specificity, it is often used for treatment of childhood tumors to minimize the impact of radiation on development. The course of treatment with IMRT typically requires multiple treatment sessions over a time course ranging from 6 to 10 weeks.

Cross References ▶ Early-Delayed Effects of Radiation ▶ Late-Delayed Effects of Radiation ▶ Late Effects of Radiation ▶ Radiation Oncology ▶ Radiotherapy ▶ Stereotactic Radiation Therapy

References and Readings Boyer, A. L., Butler, E. B., DiPetrillo, T. A., et al. (2001). Intensitymodulated radiotherapy: Current status and issues of interest. International Journal of Radiation Oncology Biology Physics, 51(2), 880–914. Ezzell, G. A., Galvin, J. M., Low, D., et al. (2003). Guidance document on delivery, treatment planning, and clinical implementation of IMRT: Report of the IMRT subcommittee of the AAPM radiation therapy committee. Medical Physics, 30(8), 2089–2115. Hall, E. J. (2006). Intensity-modulated radiation therapy, protons, and the risk of second cancers. International Journal of Radiation Oncology Biology Physics, 65(1), 1–7. Nutting, C., Dearnaley, D. P., & Webb, S. (2000). Intensity modulated radiation therapy: A clinical review. British Journal of Radiology, 73 (869), 459–469.

R OBERT R IDER Drextel University Philadelphia, PA, USA

Definition IMRT is a form of radiotherapy which achieves high precision by focusing radiation dose to the location of

Intensive Care Unit (ICU) Psychosis ▶ Delirium

Intent, General v. Specific

Intent, General v. Specific R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition Intent and motive are terms that are commonly confused, but they are distinct principles and differentiated in the law. Motive is the cause or reason that prompts a person to act or fail to act. In contrast, intent refers only to the state of mind (mens rea) with which the act is done or omitted. Because intent is a state of mind, it can rarely be proved with direct evidence and ordinarily must be inferred from the facts of the case. Evidence of intent is always admissible to prove a specific-intent crime, but evidence of motive is only admissible if it tends to help prove or negate the element of intent. Courts generally allow a wide range of direct and circumstantial evidence to be introduced at trial in order to prove the difficult element of criminal or torturous intent. In addition, the doctrine of presumed intent may be helpful in proving specific intent because it holds individuals accountable for all the natural and probable consequences of their acts. Criminal offenses requiring general intent specify a mens rea element that is no more than the negligent or reckless commission of the actus reus. The actor either knew (recklessness) or should have known (negligence) that his action (actus reus) would result in the harm suffered by the victim. Unlike offenses that require a specific intent, it is not necessary that the accused intended the precise harm or result. It is sufficient if the person meant to do the act that caused the harm or result. For example, battery is a general-intent offense. If a defendant commits a battery that results in harm to the victim, it does not matter if the defendant did not intend the harm. A range of words is used to represent shades of intention in the various criminal laws around the world. The most serious crime of murder, for example, traditionally expressed the mens rea element as malice aforethought. A person intends a consequence when he or she foresees that it will happen if the given series of acts or omissions continue and desires it to happen. The most serious level of culpability, justifying the most serious levels of punishment, is achieved when both these components are actually present in the accused’s mind. A person who plans and executes a crime is considered, rightly or wrongly, a more serious danger to the public than one who acts

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spontaneously, whether out of the sudden opportunity to steal, or out of anger to injure another. In some states, a distinction is made between an offense of basic (sometimes termed ‘‘general’’) intent and an offense of specific intent. A limited number of offenses are defined to require a further element in addition to basic intent, and this additional element is termed specific intent. There are two classes of such offenses: (a) some legislatures decide that particular criminal offenses are sufficiently serious that the mens rea requirement must be drafted to demonstrate more precisely where the fault lies. Thus, in addition to the conventional mens rea of intention or recklessness, a further or additional element is required. The rule in cases involving such offenses is that the basic element can be proved in the usual way, but the element of specific intent must be shown using a more subjective than objective test so that the legislature’s express requirement can be seen to be satisfied. (b) The inchoate offenses, such as attempt and conspiracy, require specific intent in a slightly different sense. If an accused has actually committed the full offense, the reality of the danger has been demonstrated. But, where the commission of the actus reus is in the future and the accused is merely acting in anticipation of committing the full offense at some time in the future, a clear subjective intention to cause the actus reus of the full offense must be demonstrated. Without this specific intent, there is insufficient evidence that the accused is the clear danger as feared because, at any time before the commission of the full offense, the accused may change his or her mind and not continue. Hence, this specific intent must also be demonstrated on a subjective basis.

Cross References ▶ Actus Reus ▶ Mens Rea

References and Readings Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts (3rd ed.). New York: Guilford Press. Yates, K. F., & Denney, R. L. (2008). Neuropsychology in the assessment of mental state at the time of the offense. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press.

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Intention Tremors

Intention Tremors A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Synonyms Action Tremor; Volitional tremor

Definition Intention tremor manifests as a marked increase in tremor amplitude during a terminal portion of targeted movement. Examples of intention tremor include cerebellar tremor and multiple sclerosis type tremor.

Cross References ▶ Tremor

References and Readings Fahn, S., & Jankovic, J. (Eds.). (2007). Tremors: Diagnosis and treatment. In Movement disorders (pp. 451–479). Philadelphia: Churchill Livingstone Elsevier.

Interdisciplinary Team Rehabilitation

improve the outcome of patient-focused care beyond that of team members working separately. This is in contrast to multidisciplinary care, in which individual disciplines each work with the same patient or set of patients but do not operate as a cohesive, integrated whole.

Current Knowledge Characteristics of successful interdisciplinary teams reflected in the literature include: patient-focused care with highly integrated, collective goals across disciplines, and a highly collaborative working environment with shared decision-making (see Suddick & De Souza, 2006 for a review). Activities associated with interdisciplinary teams include defining team-based goals, examining progress toward goals and revising as necessary, identifying patient and family response to treatment along with barriers to treatment and discharge, defining the discharge plan, and collaborating to implement the plan. There is little research examining the effectiveness of interdisciplinary teamwork. However, increased interdisciplinary collaboration has been associated with lower levels of cardiovascular, pulmonary, and neurologic systems failure, along with improved communication, increased preventative care, earlier identification of issues requiring clinical attention, more timely consultation with specialty services, improved patient and family satisfaction, decreased length of stay, and increased job satisfaction (Baggs, Ryan, Phelps, Richeson, & Johnson, 1992). Fewer decubitus ulcers, shorter length of stay, fewer variances from clinical pathways, and cost savings for the hospital have also resulted from interdisciplinary team rounds (Halm, Goering, Smith, 2003).

Cross References

E RIN E. E MERY Rush University Medical Center Chicago, IL, USA

▶ Family Team Conference ▶ Rehabilitation Counseling ▶ Rehabilitation Psychology

Synonyms


Rehabilitation team; Team

Definition Individuals from diverse disciplines making unique, complementary contributions with a unified purpose to

Baggs, J., Ryan, S., Phelps, C. E., Richeson, J. F., & Johnson, J. E. (1992). The association between interdisciplinary collaboration and patient outcomes in a medical intensive care unit. Heart & Lung, 21, 18–24. Halm, M. A., Goering, M., & Smith, M. (2003). Interdisciplinary rounds: Impact on patients, families, and staff. Clinical Nurse Specialist, 17(3), 133–1142.

Interference Heinemann, G. D., & Zeiss, A. M. (Eds.) (2002). Team performance in health care: Assessment and development (issues in the practice of psychology). New York: Plenum. Suddick, K. M., & De Souza, L. (2006). Therapists’ experiences and perceptions of teamwork in neurological rehabilitation: Reasoning behind the team approach, structure and composition of the team and teamworking processes. Physiotherapy Research International, 11(2), 72–83.

Interference A NNA M AC K AY-B RANDT Brown University Medical School Providence, RI, USA

Definition Irrelevant information that enters the focus of attention and impairs performance.

Current Knowledge Related to Automatic Processing of Task-Irrelevant Information (e.g., Stroop Effects) The basic principle of the Stroop effect (Stroop, 1935) is that an irrelevant information from one dimension of the stimulus to be processed interferes with the task performance related to a different dimension. This can be observed by contrasting two conditions in which the irrelevant dimension is either congruent or incongruent with the desired response in the primary dimension. For example, the classic effect involves color naming, and it is observed that when a color word is presented the speed to name the color of the ink is faster when the word and the color (‘‘red’’ in presented in red ink) are compatible compared with when the word and the color are incompatible (‘‘red’’ presented in blue ink). The Stroop effect is not limited to color naming alone. Researchers have investigated the effect of counting items when the individual items were numbers that were either compatible or incompatible with the count (e.g., ‘‘3 3 3’’ vs. ‘‘4 4 4’’) (Francolini & Egeth, 1980), or deciding if the word ‘‘above’’ or ‘‘below’’ is above or below an asterisk when the position is either compatible or incompatible with the presented word (e.g., ‘‘above’’ is above asterisk vs. ‘‘above’’ is below asterisk) (Seymour, 1973). This effect

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has been studied in many task conditions, and the differences in the strength of interference effects under different constraints have led researchers to propose theories about how attention is organized.

Related to Memory Interference is one proposed mechanism for forgetting. It can be viewed as affecting memory in two distinct ways, proactively and retroactively. Proactive interference occurs when new memories are impaired by existing older memories. For example, if you go to the store on Tuesday and have trouble remembering the items you need to buy because the items you bought on Monday come to mind. Over a shorter time course, researchers have investigated the effect of recalling digit series of increasing length and have found that there appears to be an interference effect of worse performance on a series of digits if it was preceded by earlier recall trials (May, Hasher, & Kane, 1999). Retroactive interference is the loss of older memories in the context of new learning. Consider, for example, learning Spanish vocabulary for one week and then switching to French. Although it is possible to experience both proactive and retroactive interference in this context, the phenomena of retroactive interference would be the relative difficulty you may experience in retrieving the Spanish word for cat (‘‘gato’’) after recently learning the French word ‘‘chat.’’ Similar to the theoretical work produced by studies of the boundary effects of Stroop interference, memory researchers have investigated the constraints of these effects to better delineate how memory functions.

Cross References ▶ Automaticity ▶ Stroop Effect ▶ Working Memory

References and Readings Francolini, C. M., & Egeth, H. E. (1980). On the nonautomaticity of automatic activation: Evidence of selective seeing. Perception and Psychophysics, 27, 331–342. MacLeod, C. M. (1992). The Stroop task: The ‘‘gold standard’’ of attentional measures. Journal of Experimental Psychology: General, 121, 12–14. May, C. P., Hasher, L., & Kane, M. J. (1999). The role of interference in memory span. Memory and Cognition, 27(5), 759–767.

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Interhemispheric Commissure

Seymour, P. H. K. (1973). Stroop interference in naming and verifying spatial locations. Perception and Psychophysics, 14, 95–100. Stroop, J. R. (1935). Studies of interference in serial verbal reactions. Journal of Experimental Psychology, 18, 643–662.

Interhemispheric Commissure ▶ Anterior Commissure ▶ Corpus Callosum

Interictal Behavior Syndrome C HRISTINE J. W EBER-M IHAILA Northeast Regional Epilepsy Group New York, NY, USA

Synonyms Geschwind syndrome (or Waxman–Geschwind Syndrome); Interictal personality syndrome; Temporal lobe syndrome

Definition A distinct syndrome of behavior described initially by Stephen Waxman and Norman Geschwind that occurs interictally (i.e., between seizure events) in some patients with complex partial seizures with involvement of the temporal lobe (usually associated the most with individuals with left temporal lobe epilepsy). These changes have been described to include alterations in sexual behavior (usually hyposexuality or decreased interest in sexual behavior), increased religiosity (hyper-religiosity and/or hyper-morality), increased verbal output, hypergraphia, circumstantial thinking, stickiness or viscosity in thinking and social interactions, and deepened emotionality.

Current Knowledge Although it is widely known that psychiatric comorbidity among individuals with epilepsy is high compared with the general population, the existence of a specific behavior syndrome associated with temporal lobe epilepsy is considered to be controversial. Some scholars either doubt its existence or believe it to be rare, while others

believe it to be a common entity within temporal lobe epilepsy. Early research appeared to show positive support for Waxman and Geschwind’s clinical observations. In 1977, a personality profile created by Baer and Fedio was used to study the differences between patients with origination of seizures from the left and right temporal lobes in comparison with a healthy control group and a control group of patients with neuromuscular disorders. They found significant differences between patients with epilepsy and their control counterparts, and concluded that the differences were related to epileptic abnormalities. However, following such initial research on this syndrome, there has since been significantly more research published that failed to show the existence of such a specific syndrome. Most recent studies have found similar behavioral changes in patients with different types of epilepsy, which has led most researchers to believe that a combination of biological factors, antiepileptic drugs, and psychosocial/environmental factors is associated with these behavioral changes. Further, there has been no therapeutic intervention suggested for this behavior syndrome. Thus, interictal behavior syndrome is not generally considered to be a specific or functional way to classify behavior or personality patterns in patients with epilepsy.

Cross References ▶ Epilepsy ▶ Geschwind, Norman (1926–1984) ▶ Seizure ▶ Temporal Lobe Epilepsy

References and Readings Bear, D. M., & Fedio, P. (1977). Quantitative analysis of interictal behavior in temporal lobe epilepsy. Archives of Neurology, 34, 454–467. Blumer, D. (1999). Evidence supporting the temporal lobe epilepsy personality syndrome. Neurology, 53(Suppl. 2), S9–S12. Devinsky, O., & Najjar, S. (1999). Evidence against the existence of a temporal lobe epilepsy personality syndrome. Neurology, 53 (Suppl. 2), S13–S25. Waxman, S. G., & Geschwind, N. (1975). The interictal behavior syndrome of temporal lobe epilepsy. Archives of General Psychiatry, 32 (12), 1580–1586.

Interictal Personality Syndrome ▶ Interictal Behavior Syndrome

Internal Carotid Artery

Intermanual Conflict ▶ Alien Hand Syndrome

Internal Capsule T HESLEE J OY D E P IERO Boston University School of Medicine Boston, MA, USA

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Less commonly small aneurysms (Charcot–Bouchard aneurysms) may develop, and cause intracerebral hemorrhage (ICH), sometimes referred to as intraparenchymal (IPH) hemorrhage. Distinction: ICH may stand for intracerebral hemorrhage (hemorrhage into the substance of the brain), or intracranial hemorrhage, hemorrhage inside the skull. IPH denotes only hemorrhage into the tissue of the brain.

Cross References ▶ Lacunar Strokes

Definition References and Readings The internal capsule is an anatomical structure in the brain, containing ascending and descending tracts.

Current Knowledge Location: The internal capsule is best viewed in a horizontal section of the brain, through the basal ganglia. It is composed of three sections: the anterior limb, genu, and the posterior limb. The anterior limb is lateral to the head of the caudate nucleus, and medial to the lenticular nucleus. The genu abuts the third ventricle medially, and the globus pallidus forms its lateral border. The posterior limb is lateral to the thalamus, and medial to the lenticular nucleus. Vascular supply: The anterior limb of the internal capsule is supplied by the anterior cerebral artery, and anterior choroidal artery. The posterior limb is supplied by penetrating branches of the middle cerebral artery. Function: Descending fibers of the corticobulbar and corticospinal tracts travel through the internal capsule. Corticobulbar fibers, controlling facial musculature, larynx, and tongue, are located in the genu. Posterior to the genu are corticospinal tracts controlling arm, followed by leg. The most posterior part of the internal capsule contains ascending axons of the spinothalamic tract, carrying sensory input from the body to the thalamus. The anterior limb of the internal capsule contains axons to and from frontal association cortex. Pathology: The posterior limb of the internal capsule is a frequent site for lacunar strokes. The most common syndrome is that of pure motor hemiplegia. The lenticulostriate arteries, penetrating branches of the middle cerebral artery, are frequently damaged by hypertension, diabetes, and aging, and can be occluded, causing strokes.

Fix, J. D. (2008). Neuroanatomy (3rd ed.). Baltimore, MD: Lippincott, Williams & Wilkins. Martin, J. (2003). Neuroanatomy: Text and atlas (3rd ed.). New York: McGraw-Hill.

Internal Carotid Artery E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Carotid artery; Cerebral Artery

Definition The internal carotid artery, an artery of the head and neck, is the major source of blood supply for the anterior portion of the brain. The right and left internal carotid arteries derive from the common carotid artery on each respective side of the neck. The right common carotid artery is a branch of the innominate artery, which derives from the aorta and is located behind the right clavicle in the upper chest. The left common carotid artery branches directly from the arch of the aorta shortly after the aorta leaves the heart. The internal carotid artery has several components: the cervical segment or C1, petrous segment or C2, lacerum segment or C3, cavernous segment or C4, clinoid segment or C5, ophthalmic or supraclinoid

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Internal Radiation Therapy

segment or C6, and communicating or terminal segment or C7. In the neck, the cervical segment of the internal carotid artery does not give rise to any branches. However, through its course inside the brain, it gives off several branches going deeper into the cerebral tissue. The most important branches derive from the communicating or terminal segment; these include the posterior communicating artery, the anterior choroidal artery, and the two terminal branches of the internal carotid artery, the anterior cerebral artery and the middle cerebral artery.

Current Knowledge The internal carotid artery can be completely occluded or partially blocked by atherosclerosis, a buildup of plaque composed of fat deposits, platelets, and other materials on the inside of the artery wall. This can cause stroke or transient ischemic attack. Some patients will benefit from carotid endarterectomy, a surgical procedure that removes plaque from inside the blood vessel, thereby restoring blood flow to the brain. Another, less invasive, form of treatment of carotid artery atherosclerosis is angioplasty, in which a catheter is threaded into the carotid artery using fluoroscopy, a balloon at the end of the catheter is inflated to dilate the vessel, and a stent (a small mesh tube) is placed in the vessel to keep it open. The internal carotid artery can also be the site of a cerebral aneurysm or dissection (spontaneous hemorrhage).

Cross References ▶ Anterior Cerebral Artery ▶ Atherosclerosis ▶ Dissection ▶ Middle Cerebral Artery ▶ Posterior Communicating Artery

Internal Radiation Therapy ▶ Brachytherapy

Internal Regulation System ▶ Autonomic Nervous System

Internal Secretion ▶ Hormones

International Classification of Diseases A NUJ S HARMA Virginia Commonwealth University School of Medicine Richmond, VA, USA

Synonyms International statistical classification of diseases and related health problems

Definition The ICD is an international standard diagnostic classification used for clinical decision making, health management, and general epidemiology. It is used to classify diseases and other health problems. Each disease or health problem is categorized into a group with similar diseases and then assigned a unique code up to six characters in length. This system can be used to monitor the incidence and prevalence of a disease relative to specific variables or the general health of a specific population. In addition to allowing the retrieval of diagnostic information, these records are used to compile the national mortality and morbidity statistics.

Current Knowledge In 1893, the first International List of Causes of Death (called the Bertillon Classification) was introduced at the International Statistical Institute in Chicago. The American Public Health Association recommended that Canada, Mexico, and the USA adopt the classification system in 1898. Additionally, they recommended that the system be revised every ten years to remain current and accurate. In 1900, the first international conference was held in France to revise the system with subsequent revisions every decade afterwards. The World Health Organization (WHO), in 1948, assumed

International Neuropsychological Society

responsibility for publication and revision of the system starting with the sixth edition. This edition was the first to include causes of morbidity and mortality. In 1962, the seventh revision of the ICD, also known as the International Classification of Diseases, Adapted for Indexing of Hospital Records and Operation Classification (ICDA) was published by the US Public Health Service. The ICD is currently in its tenth edition; ICD-11 will begin revision in 2015.

References and Readings Gersenovic, M. (1995). The ICD family of classifications. Methods of Information in Medicine, 34(1–2), 172–175. Israel, R. A. (1991). The history of the international classification of diseases. Health Bulletin, 49(1), 62–66. World Health Organization. (2009). International Classification of Diseases (ICD). Retrieved from http:// www.who.int/classifications/ icd/en/

International Neuropsychological Society R EBECCA M C C ARTNEY Emory University/Rehabilitation Medicine Atlanta, GA, USA

Membership International Neuropsychological Society (INS) consists of members from primarily scientific and educational backgrounds, such as researchers and scientist-practitioners. The society originated in 1967 with close to 20 members and current membership totals over 4,500 active members, with representation throughout the world. Membership includes a subscription to the society’s journal, on-line access to the journal, discounts on books and journals from selected publishers, and the INS-NET, which is the INS newsletter. There are two levels of membership: regular and associate. Regular members must have met qualifications (degrees, certifications, or other requirements) consistent with the profession in their country of origin and devote a significant proportion of their activities to neuropsychology or closely related fields. Regular members have the power to vote in the Society elections and to hold office.

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Associate members must currently be enrolled in an academic program (undergraduate or graduate), an internship program, postdoctoral training program, or residency program; they cannot vote in Society elections and may not hold office in the Society.

Mission Statement INS’s stated mission is to promote the international and interdisciplinary study of brain–behavior relationships throughout the life span. The organization’s emphasis is on science, education, and the application of scientific knowledge.

I Landmark Contributions INS was founded in 1967 and was the first openmembership organization in the field. The first president was Karl Pribram, Ph.D. The first Annual Meeting was held in New Orleans in February 1973, during the presidency of Allan Mirsky, and was chaired by Paul Satz. The theme for the meeting, attended by close to 100 people, was childhood learning problems. The INS task force on Education, Accreditation and Credentialing in Clinical Neuropsychology, formed in 1976 and chaired by Manfred Meier, was charged with addressing some difficult professional issues that were arising due to the rapid growth of the field, the paucity of formal training programs, and the absence of professional guidelines. The primary purposes of the task force were to develop guidelines for predoctoral, internship, and postdoctoral training, to make recommendations for the credentialing of individual competencies, and to formulate a strategy for accreditation of educational programs. INS later transferred the responsibilities of the task force to the American Psychological Association Division 40.

Major Activities INS hosts two meetings each year. The North American meeting is held in February and the non-North American meeting is held in June or July. The official journal, published six times a year, is the Journal of the International Neuropsychological Society (JINS). Publication of JINS began in January 1995 by Cambridge University

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Press. JINS covers a broad scope of interests in all areas of neuropsychology, including adult neuropsychology, child neuropsychology, developmental neuropsychology, disorders of speech and language, and the interface between neuropsychology and related disciplines, such as behavioral neurology, neuropsychiatry, neuroimaging, and electrophysiology. Book reviews, Grand Rounds, minutes of Society business meetings and other features are also published in JINS. INS has several committees in which members can participate. These include the continuing education committee, the international liaison committee, and the publications committee. The International Liaison Committee was established in late 1994 with a mission to promote increased communication and collaboration among neuropsychologists throughout the world. The committee serves as a broker of information and addresses obstacles faced by colleagues who manage both scientific and clinical responsibilities. This committee provides information on INS and outside programs, publishes the society’s newsletter, and maintains a website with hyperlinked databases at http:// www.ilc-ins.org. The Vivian Smith Advanced Studies Institute of the INS was started in 2002 to enhance neuropsychology as a field. Each year up to 75 graduate student fellows and up to 20 outstanding scientists with international recognition from institutions of higher learning in America, Europe, Asia, Africa, and Australia are selected to participate in the summer program in Xylocastro, Greece. The primary goals of this institute are to promote dissemination of knowledge in the fields of cognitive neuroscience and clinical neuropsychology, to foster formation of professional bonds among future and current leaders in these fields from across the world, to promote the indepth study of fundamental questions in these fields and seek solutions under conditions that optimize such academic pursuits. The practical goals of this institute are to advance the understanding of the neurophysiological mechanisms of sensory, motor, and higher psychological functions, the pathophysiology of deficits in these functions, and mechanisms of their resolution, treatment, and approaches to rehabilitation.

References and Readings Boake, C. (2008). Clinical neuropsychology. Professional Psychology: Research and Practice, 39(2), 234–239. Boake, C., & Bieliauskas, L. A. (2007). Development of clinical neuropsychology as a psychological specialty: A timeline of major events. The ABPP Specialist, 26, 42–43. Meier, M. J. (1992). Modern clinical neuropsychology in historical perspective. American Psychologist, 47(4), 550–558. Rourke, B. P., & Murji, S. (2000). A history of the International Neuropsychological Society: The early years (1955–1985). Journal of the International Neuropsychological Society, 6, 491–509. INS website: https://www.the-ins.org

International Neuropsychology Symposium A DAM H UDEPOHL 1, A NTHONY Y. S TRINGER 2 1 Georgia State University Atlanta, GA, USA 2 Emory University Atlanta, GA, USA

Membership The International Neuropsychological Symposium consists primarily of neurologists, psychiatrists, neuropsychologists, and individuals in related areas. Membership is by invitation only and the majority of its members are Europeans. At its outset, the group was very small, consisting of a handful of influential clinicians and scientists, and for many years, its membership was fewer than 40 individuals. As of 1998 (date of the last available figure), the organization had 111 members.

Mission Statement The purpose of the International Neuropsychological Symposium is to promote knowledge and understanding of brain functions and related issues at the intersection of neurology, psychology, and psychiatry.

Cross References Landmark Contributions ▶ American Academy of Clinical Neuropsychology (AACN) ▶ American Psychological Association, Division 40 ▶ National Academy of Neuropsychology

Oliver Zangwill (1984) credits Henry Hećaen with the idea of forming this international group in the summer

International Statistical Classification of Diseases and Related Health Problems

of 1949 while the two scholars were at a dinner party at Hećaen’s Paris home. Hećaen had previously discussed the idea informally with his friend and colleague, Hans Hoff, who was supportive of the idea, as were all who were in attendance at Hećaen’s dinner party. He desired that the group be informal in nature, by invitation only, and free of the obligation to publish papers delivered at its meetings. The group was called simply ‘‘The Symposium’’ in its early years and there is no record of when the name ‘‘International Neuropsychological Symposium’’ was first used. The first meeting was held in the summer of 1951 at the resort town of Mondsee near Salzburg, Austria. It was organized by Henry Hećaen, Hans Hoff, Klaus Conrad, and Oliver Zangwill. Those believed to be in attendance included Otto Po¨tzl, Eberhard Bay, Richard Jung, Franz Gu¨nther von Stockert, Klaus Gloning, Richard Oldfield, Moira Williams, Malcolm Piercy, and John MacFie. Two main themes were discussed. Oliver Zangwill presented on spatial perception and Hans Hoff introduced psychic symptoms associated with lesions of the third ventricle. In 1956, at the meeting in Brittany, Henry Hećaen raised the possibility of creating a journal that was exclusively dedicated to the dissemination of neuropsychological research. The resulting publication, Neuropsychologia, was the first of its kind and Hećaen served as its editor-in-chief until 1981. Before 1970, the conference skipped every fourth year due to the meeting of the World Federation of Neurology. German was the primary language used in the first years of the conference and presentations made in other languages were translated. The conference has been conducted exclusively in English since 1977.

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Porto Heli, Greece (2004) Topic 1: Mastery of tools and technology by human and non-human primates Topic II: New views of frontal cortex: executive control, expectation, and reward Topic III: Development of normal language and developmental dyslexia Alghero Sardinia, Italy (2005) Topic 1: Interactive processes in vision Topic 2: Productive symptoms in neuropsychology: Behavioral and physiological accounts Topic 3: Neural reorganization Estoril, Portugal (2006) Topic 1: Auditory cognition Topic 2: Adolescent brain in health and after prenatal insult Topic 3: Cognitive foundations of action

Cross References ▶ Hećaen, Henry (1912–1983) ▶ Zangwill, Oliver (1913–1987)

References and Readings Boller, F. (1999). History of the International Neuropsychological Symposium: A reflection of the evolution of a discipline. Neuropsychologia, 37(1), 17–26. Zangwill, O. (1984). Henry Hećaen and the origins of the International Neuropsychological Symposium. Neuropsychologia, 22(6), 813–815.

Major Activities The International Neuropsychological Symposium meets once a year during the last full week of June. As of 2006, there have been 51 meetings. The group meets in Western Europe or adjacent countries. The journal Neuropsychologia, first published in 1963, was a collaboration of the International Neuropsychological Symposium. Generally, a list of three topics is selected in advance for in-depth discussion by the attendees. The program is split equally between presentations on the topics of interest and general discussion in which the entire group is encouraged to share their insights and opinions. A list of recent meetings demonstrates the wide variety of topics that are discussed by the symposium:

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International Standards for the Neurological Classification of Spinal Cord Injury ▶ ASIA Impairment Scale

International Statistical Classification of Diseases and Related Health Problems ▶ International Classification of Diseases

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Internuclear Ophthalmoplegia

Internuclear Ophthalmoplegia D OUGLAS I. K ATZ Braintree Rehabilitation Hospital Braintree, MA, USA

Inter-rater Reliability R AEL T. L ANGE British Columbia Mental Health and Addiction Services and University of British Columbia Vancouver, BC, Canada

Definition Synonyms Internuclear ophthalmoplegia (INO) is an inability to produce conjugate adduction of one eye while the other eye is abducting during lateral gaze. INOs are caused by lesions in the medial longitudinal fasciculus (MLF) that interrupt connections from the cranial nerve VI (abducens) nucleus to the opposite cranial nerve III (ocularmotor) nucleus, necessary for yoked, lateral movements of the eyes. The medial rectus ipsilateral to the MLF lesion does not adequately adduct the eye while lateral rectus of the opposite, abducting eye is stimulated to contract. Often there is a lateral beating nystagmus of the abducting eye associated with the abnormal movement of the adducting eye.

Cross References ▶ Diplopia ▶ Lateral Gaze Palsy ▶ Oculomotor Nerve

References and Readings Leigh, R. J., & Zee, D. S. (2006). The neurology of eye movements (4th ed.). New York: Oxford University Press.

Concordance; Inter-observer reliability; Inter-rater agreement; Scorer reliability

Definition Inter-rater reliability is the extent to which two or more raters (or observers, coders, examiners) agree. It addresses the issue of consistency of the implementation of a rating system. Inter-rater reliability can be evaluated by using a number of different statistics. Some of the more common statistics include: percentage agreement, kappa, product–moment correlation, and intraclass correlation coefficient. High inter-rater reliability values refer to a high degree of agreement between two examiners. Low inter-rater reliability values refer to a low degree of agreement between two examiners. Examples of the use of inter-rater reliability in neuropsychology include (a) the evaluation of the consistency of clinician’s neuropsychological diagnoses, (b) the evaluation of scoring parameters on drawing tasks such as the Rey Complex Figure Test or Visual Reproduction subtest, and (c) the evaluation of qualitative variables derived from behavioural observations.

Cross References ▶ Test Reliability ▶ Test Validity

Inter-Observer Reliability


▶ Inter-rater Reliability Anastasi, A., & Urbina, S. (1997). Psychological testing (7th ed.) Upper Saddle River, NJ: Prentice Hall.

Inter-Rater Agreement ▶ Inter-rater Reliability

Intersubtest Scatter ▶ Subtest Scatter

Intracarotid Sodium Amobarbital Test

Intertest Scatter S ANDRA B ANKS Allegheny General Hospital Pittsburgh, PA, USA

Synonyms Intertest variability

Definition Intertest scatter is a measure of the variability of standardized scores on subtests within a larger test or on separate tests within a larger battery of tests. High intertest variability is depicted by a wide array of scores when the scores of more than one test are entered into a scatterplot. Low intertest variability is depicted by a minimal scattering of scores when the scores of more than one test are entered into a scatterplot. The degree of scatter needed to be considered abnormal is a function of the correlation among the tests and the reliability of the individual scores. Scatter in tests that do not correlate highly may not reflect pathology. Scatter among scores with low reliability may not reflect pathology. Specific patterns of scores on tests may assist with diagnosis. For example, when an individual’s scores are consistently intact across the indices of the Wechsler Intelligence Scale for Children – Fourth Edition (low scatter) with the exception of variable performance on the Working Memory Index (high scatter when compared with other subtests), this may indicate attention difficulties. Within a battery of tests, if an individual demonstrates relatively intact performance in the majority of cognitive domains but has weaknesses on memory and language tasks, one might consider brain injury or dementia, depending on the patient’s presenting concerns and history.

Cross References ▶ Test Interpretations

Intertest Variability ▶ Intertest Scatter

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Interview Schedules ▶ Structured Clinical Interview For DSM-IV (SCID-I/ SCID-II)

Intracarotid Sodium Amobarbital Test L ORI G RAFTON , F LORA H AMMOND Carolinas Rehabilitation Charlotte, NC, USA

Synonyms Wada test

Definition Intracarotid sodium amobarbital test also known wada test (named after Dr. Juhn Wada, who developed it,) combines neuroimaging and neuropsychological testing methods to establish which cerebral functions are localized to which hemisphere, specifically language. During the test, one side of the brain is put to sleep (anesthetized) by injecting a barbiturate into the internal carotid artery via a cannula or intra-arterial catheter from the femoral artery. The drug is injected into one hemisphere at a time. For example, when the drug is injected into the left carotid artery, the left side of the brain is anesthetized for several minutes. Because the left side of the brain controls movement on the right side of the body, the right side of the body will not be able to move for 5–15 min. Therefore, if the anesthetized side is the side that controls speech, the patient will not be able to speak until the effect of the drug clears. To test the patient’s speech, the patient will be asked to read words, identify objects, pictures, shapes and numbers, and answer questions about what they are shown.

Current Knowledge The test is performed prior to ablative surgery for epilepsy and/or tumor resection in order to verify which side of the brain is responsible for certain cognitive functions so as to minimize surgical risk to these structures.

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Intracerebral Hemorrhage

Cross References ▶ Angiography (Cerebral)

References and Readings http://www.neuro/mcg.edu/np/INS%20Wada%20Syllabus.htm http://www.neuro.mcg.edu/np/wada.html http://www.emedicine.com/med/topic3177.htm Image (http://www3.hku.hk/philodep/joelau/media/wada-test.jpg) at University of Hong Kong The Johns Hopkins Epilepsy Center (http://www.neuro.jhmi.edu/Epilepsy/wada.html)

Intracerebral Hemorrhage E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Hemorrhagic stroke; ICH; Intracranial hemorrhage; Intraparenchymal hemorrhage

malformations and aneurysms that burst. It is a medical emergency because of the increased intracranial pressure. Symptoms depend on the size and specific location of the hemorrhage, and include severe headache (‘‘the worst headache of my life’’), nausea, stiff neck, vision changes, altered consciousness with stupor or coma, cognitive and language dysfunction, motor or sensory deficits, among others. Diagnosis is made by brain imaging using CT or MRI scanning, which usually clearly show the presence of blood in the brain tissue. Treatment depends on the type, cause, severity, and location of the bleeding. This can include medications to control bleeding, blood pressure, elevated intracranial pressure, and pain; surgical procedures to evacuate a hematoma or to control or remove a vascular anomaly; and others. ICHs have relatively high mortality rates, up to 50% within the first month following stroke. Complete or nearly total recovery occurs in many, resulting from surgical removal or spontaneous resorption of the blood, but many patients have residual disability requiring rehabilitation, ongoing supportive care, and long-term monitoring.

Cross References ▶ Cerebellar Hemorrhage ▶ Lobar Hemorrhage ▶ Thalamic Hemorrhage ▶ Vascular Malformation

Definition An intracerebral hemorrhage (ICH) – bleeding into the brain – can result from trauma, but more commonly results spontaneously when the wall of a blood vessel in the brain bursts, allowing blood to leak into the tissue of the brain, producing a type of stroke.

Current Knowledge The sudden appearance of blood in the brain can be irritating or toxic to brain cells, and the increase in intracranial pressure causes additional brain damage. Although ICH usually occurs in the basal ganglia, brain stem, and cerebellum, it also can involve the cerebral hemispheres and cortex. ICH accounts for an estimated 10–15% of all strokes. It occurs at all ages, but it tends to affect younger people more commonly than do ischemic strokes, and its incidence among African-Americans is double that of other racial groups. It is most frequently caused by sudden episodes of extreme hypertension, but also can be caused by cerebral arteriovenous

References and Readings Broderick, J., Connolly, S., Feldmann, E., Hanley, D., Kase, C., Krieger, D., et al. (2007). Guidelines for the management of spontaneous intracerebral hemorrhage in adults – 2007 update: a guideline from the American Heart Association/American Stroke Association Stroke Council, High Blood Pressure Research Council, and the Quality of Care and Outcomes in Research Interdisciplinary Working Group. Stroke, 38, 2001–2023.

Intracranial Hemorrhage E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition An intracranial hemorrhage occurs when there is bleeding inside the cranium, which results from a burst of a wall of

Intracranial Pressure

a blood vessel in or around the brain, causing extravasation of blood outside of the vessel.

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▶ Subarachnoid Hemorrhage ▶ Subdural Hematoma ▶ Thalamic Hemorrhage ▶ Vascular Malformation

Current Knowledge Intracranial hemorrhage can result from trauma or spontaneously from stroke caused either by extreme hypertension or by a rupture of a cerebral aneurysm or arteriovenous malformation. It is a medical emergency because of the increased intracranial pressure, which can displace or damage brain tissue. The diagnosis is based on imaging studies such as CT and MRI scanning. There are two main types of intracranial hemorrhages: Intraaxial hemorrhage is bleeding within the brain itself, and includes intracerebral hemorrhage and intraventricular hemorrhage. These tend to be more difficult to manage. Extraaxial hemorrhage is bleeding that occurs inside the skull but outside of the brain, and includes epidural hematoma, subdural hematoma, and subarachnoid hemorrhage. Epidural hematomas occur between the dura matter and the brain, and result from trauma. These can cause rapid and deadly elevations in intracranial pressure, and so represent a true medical emergency. Patients often have a loss of consciousness and then a lucid interval, and then a sudden deterioration in consciousness. Epidural hematomas are treated surgically. Subdural hematomas result from the tearing of the small bridging veins between the dura and the arachnoid, and tend to be slower and more indolent in their development. This most commonly results from trauma, but occasionally, the history of trauma is not clearly elicited. Subarachnoid hemorrhage occurs between the arachnoid and the pia layer immediately surrounding the brain. This can result from trauma, or from rupture of an aneurysm or arteriovenous malformation. This is managed by close monitoring and, at times, urgent surgical intervention. Onset of symptoms of intracranial hemorrhage is usually during daytime activity, and presents with altered consciousness, headache, nausea, seizures, and focal neurological deficits.

References and Readings Broderick, J., Connolly, S., Feldmann, E., Hanley, D., Kase, C., Krieger, D., et al. (2007). Guidelines for the management of spontaneous intracerebral hemorrhage in adults. 2007 update. A guideline from the American Heart Association/American Stroke Association Stroke Council, High Blood Pressure Research Council, and the Quality of Care and Outcomes in Research Interdisciplinary Working Group. Stroke, 38, 2001–2023. Schellinger, P. D., & Fiebach, J. B. (2004). Intracranial hemorrhage: The role of magnetic resonance imaging. Neurocritical Care, 1, 31–45.

Intracranial Pressure G ARY T YE 1, J OHN B ROWN 2 1 Virginia Commonwealth University Richmond, VA, USA 2 Medical College of Georgia Augusta, GA, USA

Definition Intracranial pressure (ICP) is the pressure that is exerted on the brain, cerebrospinal fluid, and blood within the skull. In an adult at rest, it is usually less than 10–15 mm of Mercury. If ICP rises above normal due to trauma, hydrocephalus, hemorrhage, or tumor, patients can exhibit behavioral changes, headache, decreased consciousness, somnolence, lethargy, seizures, and/or vomiting.

Cross References Cross References ▶ Cerebellar Hemorrhage ▶ Cerebral Amyloid Angiopathy ▶ Hemorrhagic Stroke ▶ Intracerebral Hemorrhage ▶ Intraventricular Hemorrhage ▶ Lobar Hemorrhage

▶ Cerebral Perfusion Pressure ▶ Hydrocephalus ▶ Traumatic Brain Injury

References and Readings Steiner, L. A., & Andrews, P. J. (2006). Monitoring the injured brain: ICP and CBF. British Journal of Anaesthesia, 97(1), 26–38.

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Intracranial Venous Thrombosis

Intracranial Venous Thrombosis ▶ Central Venous Thrombosis

Intra-individual ▶ Ipsative

Specific patterns of scores within tests may assist with diagnosis. For example, if an individual’s performance on the Continuous Performance Test is inconsistent in terms of reaction times in response to test stimuli across the test, this will result in an intratest scatter suggestive of poor sustained attention and vigilance.

Cross References ▶ Test Interpretations

Intra-individual Comparisons ▶ Individual Comparison Standards

Intratest Variability ▶ Intratest Scatter

Intraparenchymal Hemorrhage ▶ Intracerebral Hemorrhage

Intratest Scatter S ANDRA B ANKS Allegheny General Hospital Oakmont, PA, USA

Synonyms Intratest variability

Definition Intratest scatter is a measure of the variability of item scores within a test. High variability in responses will lead to a wide scattering of scores around the mean score on the test. Low variability will lead to a minimal scattering of scores around the mean score. The degree of scatter needed to be considered abnormal is a function of the correlation within the test and the reliability of the individual scores. Scatter in tests that do not demonstrate a high correlation may not reflect pathology. Scatter among scores within a test that demonstrate low reliability may not reflect pathology.

Intraventricular Hemorrhage E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition An intraventricular hemorrhage is bleeding into the ventricles of the brain, where the cerebrospinal fluid normally circulates. It can be caused by trauma or spontaneously by hemorrhaging in stroke. It also is fairly common in premature infants of very low birth weight. It is found in up to one-third of all patients with moderate to severe traumatic brain injury. In trauma patients, it is usually associated with brain contusion or intracerebral hemorrhage, and therefore often carries a poor prognosis. In both trauma and stroke, it can cause hydrocephalus and elevated intracranial pressure, which are often manifest by changes in cognitive functioning, drowsiness, and headache. These require immediate treatment.

Cross References ▶ Intracerebral Hemorrhage ▶ Intracranial Hemorrhage ▶ Subarachnoid Hemorrhage

Intrusion Errors

References and Readings Findlay, J. M. (2000). Intraventricular hemorrhage. Neurosurgery Quarterly, 10, 182–195. Naff, N. J. (1999). Intraventricular hemorrhage in Adults. Current Treatment Options in Neurology, 1, 173–178.

Intrusion Errors K ARL H ABERLANDT Trinity College Hartford, CT, USA

Synonyms Confabulation; False alarm; False memory

Definition An intrusion error occurs when a person reports information that was not among a set of original materials, whether it is a list of words, a set of visual patterns, a text passage, or a sequence of autobiographical events. For example, if a person presented with the list car, apple, page reports car, apple, and lamp, the last word represents an intrusion.

Current Knowledge The example illustrates the fact that intrusion errors frequently occur in tandem with other error types, e.g., omission errors. In the recognition paradigm, intrusion errors are known as false positives or false alarms. In serial and free recall, intrusion errors have been attributed to interference from other lists presented within the same experimental session or from extra-experimental sources. The false memories observed in the false memory paradigm represent a type of intrusion error. In this paradigm, participants are presented with a list of thematically related items, except for the item that expresses the theme. Assume that volunteers memorize a list of spoken words including candy, cake, sugar, and chocolate, but not sweet. Reporting sweet in the test phase represents a recall intrusion. Neuroimaging (fMRI) research revealed that such intrusions do not leave an ‘‘echo’’ in the left temporal region,

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whereas correctly recalled words do (Schacter, 1996). False memory intrusions have been induced experimentally by suggesting fictitious but plausible events to participants, e.g., spilling punch at a party (Schacter, 1995). In a subsequent recall test, participants reported the events as if they were real. In general, intrusion errors tend to occur with greater frequency in individuals whose cognitive capacity is diminished relative to that of young adults. Poor readers, older participants, and neuropsychological patients exhibit more intrusion errors than controls. The greater incidence of intrusion errors in Alzheimer’s patients than in patients with head injuries or other neuropsychological conditions, including Huntington’s disease, has been considered a diagnostic of Alzheimer’s dementia (Emilien, Durlach, Minaker, Winblad, Gauthier, & Maloteaux, 2003; Lezak, 1995). Two partially overlapping factors are thought to contribute to the occurrence of intrusion errors in patients. First, deterioration in semantic memory with a resulting lack of discrimination between related concepts may result in semantic intrusion errors (e.g., when a person reports tulip when rose was correct). Second, an individual’s capacity of monitoring the recall output in the testing phase may be reduced. For example, he or she might generate several candidates during retrieval and fail to discriminate among possible choices or to inhibit erroneous responses. In the context of autobiographical recall, intrusion errors are called confabulations. Confabulations occur along with other memory distortions in patients suffering from PTSD, trauma and injury to the frontal lobes. Patients who sustained frontal injuries may experience intrusive memories that include implausible attributes, for example, denying a loss of cognitive or physical capacity. Confabulations in frontal patients are thought to result from problems in source monitoring. The patient may remember events as having occurred in one context when in fact they occurred in another situation.

Cross References ▶ Alzheimer’s Disease ▶ Autobiographical Memory ▶ Memory ▶ Memory Impairment ▶ Short-Term Memory ▶ Signal Detection Theory ▶ Working Memory

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Inverse Agonist


Definition

Emilien, G., Durlach, C., Minaker, K., Winblad, B., Gauthier, S., & Maloteaux, J.-M. (2003). Alzheimer disease: Neuropsychology and pharmacology. New York: Springer. Lezak, M. (1995). Neuropsychological assessment. New York: Oxford University Press. Schacter, D. (1995). Memory distortion: How minds, brains, and societies reconstruct the past. Cambridge, MA: Harvard University Press. Schacter, D. (1996). Searching for memory: The brain, the mind, and the past. New York: Basic Books.

Involved field radiotherapy delivers the full therapeutic dose of radiation from three dimensions to only the areas involved by the cancer. It is useful when the neoplasm is close to critical body tissues, organs, or structures because less normal tissue is are in the field of radiation. It is used for several types of cancers, including lymphoma, esophageal, liver, pancreatic, lung, breast, prostate, and brain neoplasms. Currently, delivery of radiation to the involved field can be done by several techniques, including conformal radiotherapy and intensity modulated radiotherapy (IMRT). Total doses and dose fractions are typically higher than those given in whole brain treatments, but lower than those used in radiosurgery. The clinical target volumes include the tumor volume and the presumed region of microscopic extension (2 cm margin) (Kahn, 1994). Doses are divided into fractions to decrease damage to healthy tissues, and fractions are divided into daily doses to allow DNA repair kinetics.

Inverse Agonist ▶ Receptor Spectrum

Inverse Histamine Agonists ▶ Antihistamines

Cross References

Inverted-U Function of Arousal

▶ Late Effects of Radiation Therapy

▶ Yerkes–Dodson Law


Involuntary Movements

Kahn, F. (1994). The physics of radiation therapy (2nd ed.). Baltimore, MD: Williams & Wilkins.

▶ Movement Disorders

IPA Involuntary Nervous System ▶ Autonomic Nervous System

Involved Field Radiotherapy C AROL L. A RMSTRONG Neuro-Oncology/Neuropsychology Philadelphia, Pennsylvania, USA

▶ Impact on Participation and Autonomy Questionnaire ▶ Inferior Parietal Area

Ipsative M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Synonyms Synonyms Conformal techniques; Clinical target volume; Partial field radiotherapy

Intra-individual

Irritability

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Definition

Definition

An ipsative comparison is a comparison of different scores from the same individual. Ipsative is in contrast to normative in which the score of an individual is compared to a sample of other individuals in order to determine where that individual’s score lies in that distribution. For example, an individual’s visual memory score might be compared to that individual’s verbal memory score in order to determine any relative strengths or weaknesses.

Irresistible impulse is considered a defense similar to that of the insanity defense. It is typically utilized when a defendant argues that they should not be held criminally liable for their actions. The claim is that the defendant broke the law because they could not control their actions. Irresistible impulse can be pleaded only under the defense of diminished responsibility, not under the defense of insanity. The defense can bring a murder charge and reducing the charge to manslaughter. In addition, it provides the judge discretion as to length of sentence and whether committal would be more appropriate than incarceration. In criminal law, diminished responsibility can be used as a defense to claim that although a defendant broke the law their mental functions were impaired and so they should not be held liable.

Cross References ▶ Standardized Scores

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References and Readings Cross References Broverman, D. (1962). Normative and ipsative measurement in psychology. Psychological Review, 69, 29S–30S.

IQ ▶ Intelligence ▶ Intelligence Quotient

IQ Gains Over Time ▶ Flynn Effect

▶ Diminished Capacity ▶ Intent, General v. Specific

References and Readings Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: a scientific approach. New York: Oxford University Press. Denney, R. L., & Wynkoop, T. F. (2000). Clinical neuropsychology in the criminal forensic setting. Journal of Head Trauma Rehabilitation, 15, 804–828. Goldstein, A. M., Morese, S. J., & Shapiro, D. L. (2003). Evaluation of criminal responsibility. In A. Goldstein (Ed.), Handbook of psychology: forensic psychology (Vol. 11). New Jersey: Wiley.

IQCODE ▶ Informant Questionnaire on Cognitive Decline in the Elderly

Irresistible Impulse N ATHALIE D E FABRIQUE Cook County Department of Corrections Chicago, IL, USA

Irritability B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2 1 University of California Davis, CA, USA 2 Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Definition Synonyms Diminished responsibility; ‘‘Heat of the moment’’

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Irritability is a term generally used to explain an emotional state and is characterized by an excessive response to stimuli. The term is used in many circumstances ranging

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Irritable Bowel Syndrome

from a child or parent dealing with an uncomfortable environment to very serious issues such as autism and other psychological disorders, acute or chronic infection, chronic disease, brain injury, and so on. Irritability may be demonstrated in behavioral responses to physiological and behavioral stimuli such as environmental or emotional stimuli. Irritability is not a symptom of any specific illness; however, it may be used as an early sign of serious problems.

Cross References ▶ Frustration Tolerance ▶ Stress

References and Readings Ding, C. (2005). Applications of multidimensional scaling profile analysis in developmental research: An example using adolescent irritability patterns. International Journal of Behavioral Development, 29(3), 185–196. Faraday, M., Scheufele, P., Ley, K., & Grunberg, N. (2005). A shortened psychophysical task to quantify irritability: The reactive irritability scale II (RIS-II). Journal of Behavioral Medicine, 28(1), 3–20. Farchione, T., Birmaher, B., Axelson, D., Kalas, C., Monk, K., Ehmann, M., et al. (2007). Aggression, hostility, and irritability in children at risk for bipolar disorder. Bipolar Disorders, 9(5), 496–503. Godlaski, A., & Giancola, P. (2009). Executive functioning, irritability, and alcohol-related aggression. Psychology of Addictive Behaviors, 23(3), 391–403. Paxinos, G. (1975). The septum: Neural systems involved in eating, drinking, irritability, muricide, copulation, and activity in rats. Journal of Comparative and Physiological Psychology, 89(10), 1154–1168.

Irritable Bowel Syndrome N ATALIE C. B LEVINS Indiana University Hospital Indianapolis, IN, USA

Short Description or Definition Irritable Bowel Syndrome (IBS) is characterized by chronic abdominal pain or discomfort and altered bowel habits. In the absence of detectable organic causes, IBS is referred to as a functional disorder, which is defined by symptom-based criteria known as the ‘‘Rome criteria’’ (Longstreth et al., 2006).

Categorization Symptom onset must occur at least 6 months before diagnosis. On the basis of predominant bowel habit, IBS is categorized into three subgroups: 1. IBS with diarrhea 2. IBS with constipation 3. IBS with mixed bowel habits

Epidemiology IBS is one of the most common syndromes seen by gastroenterologists and primary care providers, with a prevalence rate of 10–15% in industrialized countries (Drossman, Camilleri, Mayer, & Whitehead, 2002). Despite its relatively high prevalence rate, only about 10–30% of afflicted individuals seek medical care (Pae, Masand, Ajwani, Lee, & Patkar, 2007). Women are affected about twice as often as men in most populationbased samples (Whitehead, Palsson, & Jones, 2002) and Caucasians appear to show a higher prevalence rate than African-Americans (Pae et al., 2007). IBS is the most common functional disorder of the gastrointestinal tract (Mertz, 2003). Of the three types, IBS with diarrhea is more commonly seen in men and IBS with constipation is more commonly seen in women. However, each group accounts for about 1/3 of all patients (Longstreth et al., 2006). A significant portion of IBS patients also suffer from other chronic syndromes including fibromyalgia, headaches, dyspepsia, and chest pain (Pae et al., 2007). Comorbid psychological conditions, primarily anxiety and somatization, are also common in IBS sufferers, which can contribute to impairments in the quality of life (Spiegel et al., 2004). IBS has also been found to contribute significantly to a large segment of healthcare resource consumption. In a recent review (Maxion-Bergemann, Thielecke, Abel, & Bergemann, 2006), total direct cost estimates per patient per year ranged from $US348 to $US8750. Persons with IBS average between 8.5 and 21.6 days absent from work annually, with estimated indirect costs ranging from $US355 to $US3344 per year.

Natural History, Prognostic Factors, Outcomes The pathophysiology of IBS remains unclear. IBS has been viewed primarily as a disorder in which changes in


gastrointestinal motility, sensitivity, and secretion, as well as psychosocial factors, interact to produce the syndrome (Pae et al., 2007). Both initial presentation and symptom exacerbation in IBS are often preceded by major psychological stressors (Drossman et al., 2002) or by physical stressors (e.g., gastrointestinal infection). Given the association between IBS symptom presentation and stress, and the responsiveness in many individuals to therapies directed at the central nervous system, IBS is often described as a ‘‘brain–gut’’ disorder (Mayer, 2008).

Neuropsychology and Psychology of Irritable Bowel Syndrome Coexisting psychological problems are common in IBS. The primary co-occurring conditions include anxiety, somatization, and symptom-related fears (e.g., ‘‘I am worried that I will have severe discomfort during the day if I don’t completely empty my bowels in the morning.’’) Using structured interviews in one of the largest prospective controlled investigations, Guthrie et al. (2003) found the most common comorbid conditions were depressive disorders (26%) and anxiety disorders (30%). These comorbid symptoms contribute to further impairments in the quality of life and it is often these additional impairments in functioning that result in excessive use of healthcare resources (Levy et al., 2001).

Neurological Implications A study in the Journal of Neuroscience (Berman et al., 2008) provided evidence for the relationship between altered brain and perceptual responses to visceral stimuli reported in IBS and alterations in brain response to expectation of such stimuli. Investigators used magnetic resonance imaging (MRI) to detect differences in brain activity in IBS patients compared to healthy ones. They found that during anticipation of visceral pain, healthy subjects, but not IBS patients, downregulate homeostatic afferent processing network activity. Downregulation is associated with higher rectal distention thresholds in healthy subjects. Anticipatory downregulation is inhibited by negative emotions (i.e., stress, anxiety, anger), and these were higher in IBS patients. When mild abdominal pain was stimulated in healthy women, MRI showed decreased activity in the insula, amygdala and brainstem, areas associated with emotion and pain. IBS patients did not show decreased activity in these areas, suggesting they

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may be ‘‘hard-wired’’ to respond more strongly to pain than healthy controls.

Role of Serotonin The neurotransmitter serotonin, or 5-hydroxytryotamine (5-HT), is known to play a pivotal role in regulating colonic motor and secretory function (Kim & Camilleri, 2000). About 90% of the 5-HT in the human body is found in the GI tract (Read & Gwee, 1994). In large, randomized, double-blind, placebo-controlled trials with patients who present with diarrhea-predominant IBS, one 5-HT receptor antagonist (alosetron) was found to decrease stool frequency and bowel urgency, relieve abdominal pain and discomfort, and improve health-related quality of life (Bradesi, Tillisch, & Mayer, 2006). The use of selective serotonin reuptake inhibitors (SSRIs) has also been linked to a reduction in symptoms and improvement in overall well-being in patients with IBS. The precise mechanisms of action of SSRIs in IBS, however, are not fully understood (Pae et al., 2007).

Brain–Gut Axis Misperception of visceral stimuli has been demonstrated in experiments with IBS patients (Mertz, 2003). The current evidence suggests that a majority of IBS sufferers have inappropriate perception of physiological events. Findings indicate that IBS could reflect a hyperreactivity in the brain–gut axis that includes receptors in the gastric system, the enteric nerve systems, and the central nervous system (Mertz et al., 2000; Orr, Crowell, Lin, Harnish, & Chen, 1997). It is likely that this hyperreactivity may be triggered by both psychological and biological factors.

Evaluation According to current clinical guidelines (Drossman et al., 2002; Longstreth et al., 2006; Spiller et al., 2007; Brandt et al., 2002), IBS can generally be diagnosed without additional testing beyond a reported clinical history, general physical exam, and routine laboratory studies in patients who have met the Rome criteria (See Table 1.)

Treatment A growing body of evidence supports the use of antidepressants for IBS (Hayee & Forgacs, 2007). However,

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treatment of IBS with currently available and FDAapproved medications primarily targets the management of symptoms (Table 2). Cognitive-behavioral therapy is the best studied psychological treatment for IBS (Brandt et al., 2002; Lackner, Mesmer, Morley, Dowzer, & Hamilton, 2004). Cognitive techniques are aimed at changing catastrophic or maladaptive thinking patterns underlying the perception of somatic symptoms (Drossman et al., 2002).

Irritable Bowel Syndrome. Table 1 Rome diagnostic criteria for Irritable Bowel Syndrome (IBS) Recurrent abdominal pain or discomfort at least 3 days per month for the past 3 months, associated with two or more of the following: 1. Improvement with defecation 2. Onset associated with a change in frequency of stool 3. Onset associated with a change in form (appearance) of stool

Behavioral techniques include relaxation training, contingency management, and assertiveness training. Some trials have also shown reductions in IBS symptoms with the use of ‘‘gut-directed’’ hypnosis aimed at improving gut function. This involves the use of relaxation, change in beliefs, and self-management (Lackner et al., 2004). The optimal means of treating patients with moderate to severe symptoms remains uncertain. Guidelines for the management of IBS have been issued by the American Gastroenterology Association, the American College of Gastroenterologists, the Rome Foundation, and the British Society of Gastroenterology. Until additional data from randomized trials involving patients with IBS can be collected, these guidelines will be based largely on consensus opinion.

Cross References ▶ Serotonin ▶ Somatization

Irritable Bowel Syndrome. Table 2 Medications used in the symptomatic management of IBSa FDA-approved Symptom

Medication

Diarrhea

Common side effects

For Symptom For IBS

Loperamide (Imodium) 2 mg/day

Constipation

Yes

No

Alosetron (Lotronex)

0.5 mg/twice a day

Constipation, ischemic colitis (rare)

No

Yes

10–20 g/day

Diarrhea, bloating, cramping

Polyetheline glycol (Miralax)

17 g/twice a day

Diarrhea, bloating, cramping

Lubiprostone (Armitiza)

23 mg/twice a day Nausea, diarrhea, headache, abdominal pain

Yes

No

Tegaserod (Zelnorm)

6 mg/twice a day

Yes

Yes

Amitriptyline; desipramine

10 mg/at bedtime Dry mouth, dizziness, weight gain

No

No

Paroxetine (Paxil)

10–60 mg/day

Sexual dysfunction, headache, nausea, No sedation, insomnia, sweating, withdrawal symptoms

No

Citalopram (Lexapro)

5–20 mg/day

Sexual dysfunction, headache, nausea, No sedation, insomnia, sweating, withdrawal symptoms

No

Fluoxetine (Prozac)

20–40 mg/day

Somnolence, dizziness, headaches, insomnia

No

Constipation Lactulose

Abdominal pain

Comorbid depression or anxiety a

Initial dose

Antidepressants (TCAs, Dose as SSRIs) or anxiolytics as appropriate indicated

Adapted from Mayer (2008) and Pae et al. (2007)

Initial diarrhea, abdominal pain, cardiovascular ischemia (rare)

No

Ischemia

References and Readings Berman, S. M., Naliboff, B. D., Suyenobu, B., Labus, J. S., Stains, J., Ohning, G., et al. (2008). Reduced brainstem inhibition during anticipated pelvic visceral pain correlates with enhanced brain response to the visceral stimulus in women with irritable bowel syndrome. The Journal of Neuroscience, 28, 349–359. Bradesi, S., Tillisch, K., & Mayer, E. (2006). Emerging drugs for irritable bowel syndrome. Expert Opinion on Emerging Drugs, 11, 293–313. Brandt, L. J., Bjorkman, D., Fennerty, M. B., Lock, G. R., Olden, K., Peterson, W., et al. (2002). Systematic review on the management of irritable bowel syndrome. American Journal of Gastroenterology, 97, S7–S26. Drossman, D. A., Camilleri, M., Mayer, E. A., & Whitehead, W. E. (2002). AGA technical review on irritable bowel syndrome. Gastroenterology, 123, 2108–2131. Guthrie, E., Creed, F., Fernandes, L., Ratcliffe, J., Van der Jagt, J., Martin, J., et al. (2003). Cluster analysis of symptoms and health seeking behaviour differentiates subgroups of patients with severe irritable bowel syndrome. Gut, 52, 1616–1622. Hayee, B., & Forgacs, I. (2007). Psychological approach to managing irritable bowel syndrome. British Medical Journal, 334, 1105–1109. Kim, D. Y., & Camilleri, M. (2000). Serotonin: A mediator of the brain–gut connection. American Journal of Gastroenterology, 95, 2698–2709. Lackner, J. M., Mesmer, C., Morley, S., Dowzer, C., & Hamilton, S. (2004). Psychological treatments for irritable bowel syndrome: A systematic review and meta-analysis. Journal of Consulting and Clinical Psychology, 72, 1100–1113. Levy, R. L., Von Korff, M., Whitehead, W. E., Stang, P., Saunders, K., Jhingran, P., et al. (2001). Costs of care for irritable bowel syndrome patients in a health maintenance organization. American Journal of Gastroenterology, 96, 3122–3129. Longstreth, G. F., Thompson, W. G., Chey, W. D., Houghton, L. A., Mearin, F., & Spiller, R. C. (2006). Functional bowel disorders. In D. A. Drossman, E. Corazziari, M. Delvauz, et al. (Eds.) Rome III: The functional gastrointestinal disorders, 3rd Ed. McLean, VA: Degnon. Maxion-Bergemann, S., Thielecke, F., Abel, F., & Bergemann, R. (2006). Costs of irritable bowel syndrome in the UK and US. Pharmacoeconomics, 24, 21–37. Mayer, E. A. (2008). Irritable bowel syndrome. The New England Journal of Medicine, 358, 1692–1699. Mertz, H. R. (2003). Irritable bowel syndrome. New England Journal of Medicine, 349, 2136–2146. Mertz, H. R., Morgan, V., Tanner, G., Pickens, D., Price, R., Shyr, Y., et al. (2000). Regional cerebral activation in irritable bowel syndrome and control subjects with painful and nonpainful rectal distention. Gastroenterology, 118, 842–848. Orr, W. C., Crowell, M. D., Lin, B., Harnish, M. J., Chen, J. D. (1997). Sleep and gastric function in irritable bowel syndrome: Derailing the brain–gut axis. Gut, 41, 390–393. Pae, C. U., Masand, P. S., Ajwani, N., Lee, C., & Patkar, A. A. (2007). Irritable bowel syndrome in psychiatric perspective: A comprehensive review. International Journal of Clinical Practice, 61, 1708–1718. Read, N. W., & Gwee, K. A. (1994). The importance of 5-hydroxytryptamine receptors in the gut. Pharmacology and Therapeutics, 62, 159–173. Spiegel, B. M., Gralnek, I. M., Bolus, R., Chang, L., Dulai, G. S., & Mayer, E. A., et al. (2004). Clinical determinants of health-related quality of

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life in patients with irritable bowel syndrome. Archives of Internal Medicine, 164, 1773–1780. Whitehead, W. E., Palsson, O., & Jones, K. R. (2002). Systematic review of the comorbidity of irritable bowel syndrome with others disorders: What are the causes and implications? Gastroenterology, 122, 1140–1156.

Ischemia E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Definition Tissue ischemia occurs when there is insufficient oxygen supply to an organ, usually resulting from an occlusion of an artery that normally provides blood supply to that organ, frequently caused by atherosclerosis. The specific symptoms depend on the location and severity of the occlusion, the specific organ involved, and whether and the speed with which the blood supply is restored. Myocardial ischemia occurs when coronary artery disease causes the heart muscle to be deprived of sufficient oxygen to do its pumping, causing certain cardiac symptoms such as angina or fatigue. Cerebral ischemia occurs when carotid artery stenosis limits blood supply to selected regions of the brain, causing various neurological and neuropsychological findings that depending primarily on the affected region. Approximately 80% of all strokes are ischemic. Ischemic episodes occur suddenly, last a few minutes to a few hours, and are strong warning signs of an impending myocardial infarction or stroke. Ischemia can also affect the intestines, legs, feet and kidneys, and cause pain, organ malfunctions, and tissue damage.

Cross References ▶ Atherosclerosis ▶ Cerebrovascular Disease ▶ Coronary Disease ▶ Embolism ▶ Infarction ▶ Myocardial Infarction ▶ Peripheral Vascular Disease ▶ Thrombosis ▶ Transient Ischemic Attack

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Detection

Lipton, P. (1999). Ischemic cell death in brain neurons. Physiological Reviews, 79, 1431–1568.

Ischemic penumbra is most often detected using positron emission tomography (PET). Using PET, investigators have characterized the penumbra as a region with an increased oxygen extraction fraction and preserved oxygen consumption, but reduced regional cerebral blood flow. Unfortunately, PET is not widely available, and therefore, new imaging techniques are being developed to visualize and quantify the penumbral region, such as diffusion/perfusion magnetic resonance imaging (MRI) and perfusion computerized tomography (CT).

Ischemic Heart Disease ▶ Coronary Disease

Ischemic Leukoaraiosis ▶ Small Vessel Ischemic Disease

Ischemic Penumbra H ELEN M. G ENOVA Kessler Foundation Research Center West Orange, NJ, USA

Definition Ischemic penumbra refers to a rim of tissue lying just outside the core ischemic region (area most severely damaged by stroke or ischemic event). Within the core ischemic region, blood and oxygen flow is severely diminished, resulting in neuronal death. However, in the ischemic penumbra, cells are viable for a short amount of time. The penumbra receives its limited blood flow from the collateral arteries of the occluded vascular tree. Studies examining how long penumbral tissue remains viable have reported anywhere from 6 h to 3 days. However, it has been shown that once the subacute phase of the stroke sets in (6–11 days), the untreated penumbral area will succumb to necrosis (cell death). This is due to the fact that the demand for oxygen is too great for the occluded vascular tree to supply.

Importance The viability of the ischemic penumbra and its potential to recover make it a target for acute pharmacologic intervention following stroke.

Treatment Because of the short window of time that the penumbra remains viable, immediate and effective treatment is critical following a stroke. Acute treatment options given immediately following stroke onset generally focus on restoring blood flow to ischemic tissue. Recombinant tissue-type plasminogen activator (rt-PA), which is used to dissolve blood clots, is a thrombolytic treatment given within 3 h of the stroke onset. Studies have shown thattreatment using this drug can result in a significant improvement of long-term outcome following stroke. Unfortunately, this intervention cannot be given past the 3 h window of time, and in many cases, the stroke is diagnosed hours after its onset. The effectiveness of rtPA steadily decreased after 3 h and the drug is actually thought to increase the risk of hemorrhagic transformation. For those patients who are treated after this 3 h time period, other interventions are available that focus on reducing complications following the stroke and prevention of future stroke, such as regulating blood pressure both immediately after the stroke and during follow-up care.

References and Readings Astrup, J., Siesjo, B. K., & Symon, L. (1981). Thresholds in cerebral ischemia – the ischemic penumbra. Stroke, 12, 723–725. Fisher, M. (2004). The ischemic penumbra: Identification, evolution and treatment concepts. Cerebrovascular Diseases (Review), 17(Suppl. 1), 1–6. Kwiatkowski, T. G., Libman, R. B., Frankel, M., Tilley, B. C., Morgenstern, L. B., Lu, M., et al. (1999). Effects of tissue plasminogen activator for acute ischemic stroke at one year. National Institute of Neurological Disorders and Stroke Recombinant Tissue Plasminogen Activator Stroke Study Group. New England Journal of Medicine, 340(23), 1781–1787.

Item Analysis

Ischemic Stroke E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Atherothrombotic brain infarction; Cerebral infarction

Definition Ischemic stroke is the most common type of stroke, accounting for about 80% of all events. It is caused by a blockage, usually as a consequence of atherosclerosis, of a blood vessel that normally provides blood supply to the brain.

Current Knowledge The lack of oxygen in the brain tissue that results from the blockage causes either reversible injury (ischemia), if it is not prolonged or severe, or death or irreversible damage of the tissue (infarction) if the interruption of blood supply is prolonged and severe. The arterial blockage may derive primarily at the site of occlusion, in which case it is called a ‘‘thrombus.’’ Alternatively, it may arise elsewhere in the vascular system, usually in the heart, and leave that primary site, flow through the vessels until it encounters a narrow lumen, thereby closing off that area; in this case, it is called an ‘‘embolism.’’ About 60% of all strokes are thrombotic and 15–20% are embolic in origin. Both cause neurological deficits, which tend to be localizing. However, there are some differences; symptoms of thrombotic strokes tend to be slower to develop, while the presentation of embolic strokes tends to be more sudden and dramatic, including seizure, headache, syncope, and presentations with multiple simultaneous disparate neurological findings. Embolic strokes tend to occur in the context of known cardiac disease. Treatment depends on the specific cause, but generally relies on reducing the vascular obstruction and preventing future events. Rehabilitation and long-term care of the deficits are identical for each type of stroke.

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▶ Cerebral Embolism ▶ Infarction ▶ Ischemia ▶ Thrombosis

References and Readings Ay, H., Furie, K. L., Singhal, A., Smith, W. S., Sorensen, A. G., et al. (2005). An evidence-based causative classification system for acute ischemic stroke. Annals of Neurology, 58, 688–697. Jackson, C. A., Hutchison, A., Dennis, M. S., Wardlaw, J. M., Lewis, S. C., & Sudlow, C. L. M. (2009). Differences between ischemic stroke subtypes in vascular outcomes support a distinct lacunar ischemic stroke arteriopathy: A prospective, hospital-based study. Stroke, 40, 3679–3684.

I Island of Reil ▶ Insular Lobe

Item Analysis M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Item analysis is, as the name suggests, the evaluation of an individual item in the context of a test. Item analysis includes item difficulty, reliability, item discrimination, and relation of the item to the scale to which it has been assigned. Typically, in classical test theory, the focus has been on the scale score, whereas in item response theory, the emphasis is on the item itself. However, item analysis is an important part of test construction in classical test theory because it guides decisions regarding whether to discard, rewrite, or reassign a particular item to another scale.

Cross References Cross References ▶ Atherosclerosis ▶ Cerebrovascular Disease

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▶ Classical Test Theory ▶ Item Difficulty ▶ Reliability

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References and Readings Facon, B., & Nuchadee, M.-L. (2010). An item analysis of Raven’s Colored Progressive Matrices among participants with Down syndrome. Research in Developmental Disabilities, 31, 243–249. Green, B. F. (1981). A primer of testing. In J. M. Notterman (Ed.), The evolution of psychology: Fifty years of the American Psychologist (pp. 193–212). Washington, DC: American Psychological Association.

Item Response Theory M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Synonyms

Item Difficulty M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Item difficulty is an estimate of the skill level needed to pass an item. It is frequently measured by calculating the proportion of individuals passing an item. In order to increase efficient use of both the examiner’s and the examinee’s time, the item difficulty index values can be used to order the administration items so that a discontinue rule can be invoked to reduce the administration of more difficult items to individuals who would be unlikely to pass them.

Current Knowledge Another method of measuring item difficulty for developmental tests is to calculate the age at which some proportion of individuals would pass the item. If 50% of individuals aged 3 years pass an item, that item might be said to have an age equivalency of 3 years. The items can then be arranged in increasing order of age equivalency so that the difficulty level increases throughout the test.

Cross References

Rasch modeling

Definition Item response theory (IRT) was developed in response to observations that error terms in psychological measurement are frequently correlated with true scores. The mathematical model is known as Rasch modeling, and typically the three-parameter Rasch model is invoked. The three parameters are the guessing parameter, the likelihood that an individual will get an item correct simply by guessing; the discrimination parameter, or the probability of a correct response at a given level of difficulty; and the difficulty parameter, or the level of skill in the construct where an item has 0.5 discrimination.

Current Knowledge IRT allows an estimate of the precision of measurement for different levels of skill. IRT has facilitated the development of adaptive testing. In computerized adaptive testing, the order of presentation of items is based on the accuracy of the individual’s responses to previous items. In that way, not all possible items have to be administered to all individuals in order to obtain a precise measurement. This allows for economy of time and effort for both subjects and assessors.

Cross References

▶ Item Analysis

▶ Classical Test Theory ▶ Reliability



de la Plata, C. M., Arango-Lasprilla, J. C., Alegret, M., Moreno, A., Ta´rraga, L., Lara, M., Hewlitt, M., Hynan, L., & Cullum, C. M. (2009). Item analysis of three Spanish naming tests: A cross-cultural investigation. NeuroRehabilitation, 24, 75–85. Lord, F. M., & Novick, M. R. (1968). Statistical theories of mental test scores. Reading, MA: Addison-Wesley.

Forero, C. G., & Maydeu-Olivares, A. (2009). Estimation of IRT graded response models: Limited versus full information methods. Psychological Methods, 14, 275–299. Reise, S. P., Ainsworth, A. T., Haviland, M. G. (2005). Item response theory: Fundamentals, applications, and promise in psychological research. Current Directions in Psychological Science, 14, 95–101.

J Jamais Vu F ERRINNE S PECTOR McMaster University Hamilton, Ontario, Canada

Definition Jamais vu, from the French, meaning ‘‘never seen,’’ refers to any familiar situation that is not recognized by the observer. Largely considered the opposite of de´ja` vu, it often involves a sense of eeriness and the observer’s impression of seeing the situation for the first time, despite rationally knowing that he or she has been in the situation before.

Current Knowledge Jamais vu occurs fairly often in the typical population (between 40 and 60%). It often manifests itself as a momentary lack of recognition of a familiar word, person, or place. For example, when you look at your own face in the mirror and it begins to look strange, or if you temporarily forget what a pedal does when you are driving. It is possible to induce jamais vu in a laboratory setting. For example, if people write down familiar words such as ‘‘wood’’ 30 times in 1 min, the majority will report experiencing jamais vu. After writing the word over and over again, people may feel as if they were writing a made-up word or an incorrect word. This could occur because the mind gets fatigued after looking at one stimulus for so long, and the stimulus loses its meaning. This suggests that jamais vu is a normal memory process that can go wrong – for example, in the case of the Capgras delusion, where people believe someone they know very well has been replaced by an impostor. Studying jamais vu in the nonclinical population can help to better understand this kind of chronic jamais vu in clinical populations. Jamais vu is one kind of neuropsychological symptom associated with classic migraines, often showing up as a

manifestation of the migraine aura, or a warning that the headache is coming on. This overlaps with the symptoms of temporal lobe epilepsy, which can also be preceded by an aura, often referred to as the dream state. Individuals with temporal lobe epilepsy often experience jamais vu accompanied by a negative affective experience, mostly fear. This is in contrast to the experience of de´ja` vu in temporal lobe epilepsy auras, which is accompanied by a positive affective experience of pleasure or familiarity. Patients who experience jamais vu accompanied by negative affect of fear may be more predisposed to psychotic symptoms than those who experience de´ja` vu. Thus it is possible that repeated negative feelings associated with jamais vu are related to the development of epileptic psychoses.

Cross References ▶ Amygdala ▶ Capgras Syndrome ▶ Ictal Phenomena ▶ Temporal Lobe Epilepsy

References and Readings Bigal, M. E., Lipton, R. B., Cohen, J., & Silberstein, S. D. (2003). Epilepy and migraine. Epilepsy and Behavior, 4, S13–S24. Sengoku, A., Toichi, M., & Murai, T. (1997). Dreamy states and psychoses in temporal lobe epilepsy: The mediating role of affect. Psychiatry and Clinical Neurosciences, 51, 23–26. Sno, H. N. (2000). De´ja` vu and jamais vu. In G. E. Berrios, & J. R. Hodges (Eds.), Memory disorders in psychiatric practice. Cambridge: Cambridge University Press.

Janz Syndrome ▶ Juvenile Myoclonic Epilepsy


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Jargon S ARAH S. C HRISTMAN B UCKINGHAM University of Oklahoma Health Sciences Center Oklahoma, OK, USA

Definition Jargon refers to the construction or use of language in a phonologically and/or semantically incomprehensible manner.

Current Knowledge Jargon in Aphasia Jargon or jargon aphasia, as incomprehensible language, is an easily recognizable symptom of fluent Wernicke’s or conduction aphasia. Jargon may take phonemic, neologistic, or semantic forms, depending on the nature of the linguistic impairments caused by aphasia. Phonemic jargon exists when numerous phoneme selection and sequencing errors create meaningless spoken sound strings in utterances that also appear devoid of syntactic organization. In neologistic jargon, sentence structure is preserved but nonsense words or neologisms replace content words rendered either inaccessible by anomia or distorted by phonemic paraphasia (Buckingham, 1990). Alternatively, semantic jargon is a syndrome variant that exists when otherwise well-formed sentences are predominantly comprised of legitimate words that are chosen or arranged in such a way as to convey no coherent meaning (Marshall, 2006). Associated characteristics of jargon aphasia include poor comprehension of verbal and written language, poor verbal self-monitoring skills, severe anomia and/or phonological processing impairments, paragrammatic syntax, and poor verbal repetition abilities, despite easily produced speech that is generally free from dysarthria or verbal apraxia (Christman & Buckingham, 1989). Aside from a tendency to talk at great length, individuals with jargon aphasia typically exhibit intact social pragmatics; that is, despite attempts to use language for communicative purposes, the linguistic impairments of aphasia yield production of words and sentences with no recognizable meaning. In jargon associated with severe or acute Wernicke’s or conduction aphasias, the

production of nonsensical utterances is likely to be involuntary and more constant, rather than more intermittent, in occurrence.

Jargon in Psychiatric Conditions Jargon has been described as a secondary characteristic of some psychiatric thought disorders and, in these cases, must be distinguished from the primary linguistic jargon of Wernicke’s or conduction aphasia (Butler & Zeman, 2005). From a linguistic perspective, thought disorder in schizophrenia is primarily characterized by impaired discourse planning and structure. However, there also may exist some aphasia-like linguistic irregularities that yield the neologisms and jargon of ‘‘schizophasia’’ (Covington et al., 2005). In schizophasia, novel words may or may not be intentionally created, semantically incoherent language may be generated for purposes other than meaningful communication, and the use of jargon may be more intermittent, rather than more constant, in occurrence.

Cross References ▶ Conduction Aphasia ▶ Fluent Aphasia ▶ Literal Paraphasia ▶ Neologism ▶ Phonemic Paraphasia ▶ Semantic Paraphasia ▶ Verbal Paraphasia ▶ Wernicke’s Aphasia

References and Readings Buckingham, H. W. (1990). Abstruse neologisms, retrieval deficits, and the random generator. The Journal of Neurolinguistics, 5(213), 215–235. Butler, C., & Zeman, A. Z. J. (2005). Neurological syndromes which can be mistaken for psychiatric conditions. Journal of Neurology, Neurosurgery, and Psychiatry, 76, 31–38. Christman, S. S., & Buckingham, H. W. (1989). Jargonaphasia. In C. Code (Ed.), The characteristics of aphasia (pp. 111–130). London: Taylor and Frances Ltd. Covington, M. A., He, C., Brown, C., Naçi, L., McClain, J. T., Fjordbak, B. S., et al. (2005). Schizophrenia and the structure of language: The linguist’s view. Schizophrenia Research, 77(1), 85–98. Marshall, J. (2006). Jargon aphasia: What have we learned? Aphasiology, 20(5), 387–410.

Jenkins v. U.S. (1962)

Jebsen–Taylor Hand Function Test M ICHELLE T IPTON -B URTON Santa Clara Valley Medical Center San Jose, CA, USA

Definition The Jebsen–Taylor function test was designed to provide a short, objective test of hand functions commonly used in activities of daily living (ADLs). The target patient population includes adults with neurological or musculoskeletal conditions involving hand disabilities, although there may be other patient populations with other hand dysfunctions which may be appropriate. The test was developed to be used by health professionals working in restoration of hand function. It consists of seven items that include a range of fine motor, weighted and non-weighted hand function activities which are timed: writing (copying) a 24-letter sentence turning over a 3 500 cards, picking up small common objects such as a coin and bottle cap, simulated feeding using a teaspoon and five kidney beans, stacking checkers picking up large light objects such as an empty tin can, and picking up and moving large weighted cans. The results are measured objectively using a stop watch. This technique allows for a continuum of scores. The mean time taken for completion of each subtest and the standard deviations were obtained for each age group. The individual’s timed scores are compared to these norms.

Current Knowledge The Jebsen was developed by Jebsen, R.H., Taylor, N., Trieschman, R.B., Trotter, M.J., and Howard, L.A. in 1969. A modified version was evaluated by Bovend’Eerdt et al. in 2004 and included three test items (turning over cards, stacking four cones, and spooning five kidney beans into a bowl). An Australian version has also been developed and includes the original test items plus a grip strength measurement using a dynameter (Agnews & Maas, 1982).

Cross References ▶ Manual Dexterity

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References and Readings Bovend’Eerdt, T. J. H., Dawes, H., Johansen-Berg, H., & Wade, D. T. (2004). Evaluation of the modified Jebsen test of hand function at the University of Maryland arm questionnaire for stroke. Clinical Rehabilitation, 18, 195–202. Hill, K., Denisenko, S., Miller, K., Clements, T., & Batchelor, F. (2005). Clinical outcome measurement in adult neurological physiotherapy (3rd ed.). Victoria: Australian Physiotherapy Association National Neurology Group. Jebsen, R. H., Taylor, N., Trieschmann, R. B., Trotter, M. J., & Howard, L. A. (1969). An objective and standardized test of hand function. Archives of Physical Medicine and Rehabilitation, 50(6), 311–319. Rider, B., & Linden, C. (1988). Comparisons of standardized and nonstandardized administration of the Jebsen Hand Function Test. Journal of Hand Therapy, October-December, 2, 266–277. Stern, E. B. (1992). Stability of the Jebsen-Taylor hand function test across three test sessions. American Journal of Occupational Therapy, 46(7), 647–649.

J Jenkins v. U.S. (1962) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Admissibility of psychological evidence; Competence to testify

Historical Background Vincent E. Jenkins was a defendant who mounted an insanity defense, introducing the testimony of psychiatrists and psychologists that he was suffering a mental disease (schizophrenia) at the time of a sexual assault. The trial court instructed the jury to disregard psychometric evidence on the grounds that psychologists were disqualified from testifying about mental disease because of a lack of medical training. The appeals court for the Second District reversed the trial court ruling, ordering the new trial to include psychological testimony and psychometric findings. The appeals court noted that a diverse array of nonphysicians (e.g., doctoral level toxicologists) regularly offered opinions.

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The majority opinion offered a two-pronged test of admissibility for psychologists’ testimony: (1). the actual experience of the witness and (2). the probative value of his opinion. This opinion implies that a medical degree is neither sufficient nor necessary to testify as to a defendant’s mental state. The trial judge must determine (e.g., be the gatekeeper) a particular psychologist’s qualifications through a context-specific, fact-intensive exploration of background, training, education, and knowledge.

Kaufmann, P. M. (2008). Admissibility of neuropsychological evidence in criminal cases: Competency, insanity, culpability, and mitigation. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press.

Jennett, Bryan (1926–2008) A ARON N. J UNI The Johns Hopkins School of Medicine Baltimore, MD, USA

Current Knowledge Overall, the weight of legal authority strongly recognizes psychologists as generally competent to testify (and neuropsychologists as competent to testify specifically as to causation of neuropsychological impairment), contingent on adequate demonstration of academic coursework, peer-reviewed research, and supervised training experiences (Greiffenstein & Cohen, 2005; Shapiro, 1991). Since Jenkins v. United States (1962), there have been a number of causation testimony cases challenged by civil defendants, but for the most part, psychologists and neuropsychologists have been allowed broad scope of testimony in cases where brain injured has been alleged. The reader is referred to other related entries in this text including Frye (1923), Daubert v. Merrell Dow (1993), Kumho Tire v. Carmichael, (1999), and General Electric v. Joiner (1997).

Major Appointments

Lecturer in Neurosurgery University of Manchester, Manchester, England (1957–1962) Rockefeller Fellow University of California, Los Angeles, Los Angeles, California (1958–1959) Consultant Neurosurgeon University of Glasgow, Glasgow, Scotland (1963–1968) Professor and Chair University of Glasgow Department of Neurosurgery, Glasgow, Scotland (1968–1991) Dean of the Faculty of Medicine University of Glasgow, Glasgow, Scotland (1981–1986)

Major Honors and Awards Cross References

▶ Admissibility ▶ Federal Rules of Evidence ▶ Insanity Defense ▶ Mental State at Offense

References and Readings Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney-neuropsychologists relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Jenkins v. United States, 307 F. 2d 637 (1962).

Jennett was active in many international professional societies including honorary membership in the Society of Neurological Surgeons, American Association of Neurological Surgeons, and American Neurological Association. In addition, he served as President of the Neurology Section of the Royal Society of Medicine and President of the International Society of Technology Assessment in Health Care. Jennett received numerous awards and honors over the course of his career, including: Hunterian Professorship of the Royal College of Surgeons (1962) William Caveness Award of National Head Injury Foundation (1981) Commander of the British Empire (1992) Honorary DSc of the University of St. Andrews (1993) First recipient of the Medal of the Society of British Neurological Surgeons (2007)

Jennett, Bryan (1926–2008)


Jennett’s primary clinical and research interests involved the detection, diagnosis, and prognosis of traumatic brain injury including understanding the underlying physiological mechanisms and the long-term sequelae. In the 1960’s, Jennett codirected the ‘‘Medical Research Council (M.R.C) Circulation Research Group’’ with Murray Harper to study the effects of inhalation anesthetics on intracranial pressure and cerebral blood flow. He devised a method for measuring cerebral blood flow in the operating room which was subsequently used to identify aneurysm patients who were more likely to tolerate arterial clamping treatment. These studies led to the development of the International ICP (intracranial pressure) symposia, a series of successful meetings that highlighted the importance of monitoring intracranial pressure in patients. Jennett was ahead of his time in understanding the potential of large clinical databases, and together with Reinder Braakman and Jan Marinus Minderhoud of the Netherlands, and Theodore Kurze of UCLA, he established the International Head-Injury Data Bank, a meticulous prospective collection of the early features and outcomes of patients with severe head injuries. This collection began in the 1970s and is still in use today. In 1972, Jennett and American neurologist Dr. Fred Plum first described and coined the term ‘‘vegetative state’’ (VS) for patients with ‘‘wakefulness without awareness’’ (Lancet 1:734–737, 1972). In collaboration with professors J. Hume Adams and David I. Graham at the University of Glasgow, Jennett attempted to define the structural basis of VS through neuropathological studies of the brains of patients who had remained in VS until their death. In most of these patients, he noted that although their brainstem remained intact, there was significant cortical atrophy. This collaboration led to further advancements in the identification of avoidable factors that can lead to secondary brain deterioration in the months following brain injury acquisition. In 1974, Jennett and Dr. Graham Teasdale proposed the Glasgow Coma Scale (GCS), a structured clinical scale for assessing the level of arousal and consciousness by rating eye, motor and verbal responses. It was a major advancement over previous methods in that it utilized objective criteria and yielded a range of scores, making it ideal for monitoring progress over time. It continues to be the most widely used measure for assessing level of consciousness, and Jennett and Teasdale’s seminal

J

GCS paper (Teasdale & Jennett, 1974) has been cited over 5,000 times. In addition to its descriptive value for individual patients, the GCS has also been shown to be a strong predictor of functional outcomes (Murray et al., 2007), and has become the standard instrument for classification of TBI severity. In addition to medical advances, Jennett was also passionate about the treatment of patients following TBI. He argued for a rational approach to diagnosis and treatment and advocated against the routine use of invasive treatments such as artificial ventilation for all patients which could result in unnecessary, longlasting, debilitating consequences. As a result, in 1975 (Jennett & Bond, 1975), Jennett and Dr. Michael Bond developed the Glasgow Outcomes Scale (GOS) to better identify a patient’s current level of functioning. Originally designed as a simple 5-point scale, the GOS has been expanded into an 8-point scale with a structured interview format (Teasdale, Pettigrew, Wilson, Murray, Jennett, 1998). Jennet’s goal was to have the GCS and GOS used together as a comprehensive and systematic identification and prediction system. Indeed, over the past 30 years, his vision has been embraced by the medical community, as the GCS and GOS have received universal acceptance as the standard instruments for classifying severity of injury and outcome following TBI, and typically form the bedrock of randomized controlled trials of TBI. In the 1980’s, Jennett focused his attention on the interface of society and medicine. He explored the practical difficulties, ethical tensions, and economic concerns regarding the use of advanced technology in treating patients with severe TBI, especially those in vegetative states. Jennett was also passionate about promoting challenging and controversial issues such as the validity of using brain stem death as the criterion for solicitation of organ donation. One particularly famous exchange was his rebuttal to the now-famous 1980 BBC Panorama program entitled ‘‘Are transplant donors really dead?’’ whereby he outlined the rigorous criteria used to determine brain death and helped buttress the public’s confidence in transplant medicine. A prolific writer and researcher, Jennet published over 200 articles in peer-reviewed journals as well as numerous textbooks and book chapters. Some of his most well-known works include: Epilepsy after Blunt Head Injury (1962), An Introduction to Neurosurgery (1964) (which has appeared in five editions), Management of Head Injuries (1981), High Technology Medicine: Benefits and Burdens (1984), and The Vegetative State: medical facts, ethical and legal dilemmas (2002).

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Short Biography Professor William Bryan Jennett (1926–2008) was a neurosurgeon who revolutionized the field of neurosurgery and traumatic brain injury in the twentieth century. In the early 1960’s, when Jennett began his career, the medical community’s understanding of brain swelling, late deterioration, impaired consciousness, and brain death were rudimentary. His work only spurred fundamental improvements in the treatment of head-injured patients but also played a pivotal role in shaping the methodology, philosophy, and ethics of brain injury intervention, as well as broader issues affecting the wider medical field. Jennett graduated at the top of his class from the University of Liverpool Medical School in 1949. After graduation, he continued his surgical training with Sir Hugh Cairns and Joseph Pennybacker at Oxford, a department renowned for its clinical work on brain injury, followed by service at the Royal Army Medical Corps Military Hospital. After further training in Cardiff, he served as a Lecturer at the University of Manchester (1957–1962). During that time, he became a Rockefeller Fellow at University of California, Los Angeles with Horace Magoun, where he studied experimental brain compression, and developed his commitment to patient care guided by clinical and laboratory research. Jennett returned to Scotland in 1963 and was appointed Consultant Neurosurgeon at the University of Glasgow enabling him to combine clinical work with laboratory research. In 1968, he became Professor and chair of the newly formed Department of Neurosurgery at the University of Glasgow, where he served as chair for 23 years (1968–1991), including 4 years as Dean of the Faculty of Medicine (1981–1986). During that time, Jennett made Glasgow into a world renowned center for excellence in neurosurgery, and attracted and trained many neurosurgeons who continue to make important contributions today.

Laureys, S. (2008). In Memoriam – Bryan Jennett, MD (1926–2008). Neurology, 71(1), 13. Murray, G. D., Butcher, I., McHugh, G. S., Lu, J., Mushkudiani, N. A., & Maas, A. I. (2007). Multivariable prognostic analysis in traumatic brain injury: results from the IMPACT study. Journal of Neurotrauma, 24, 329–337. Stocchetti, N., Citerio, G., Maas, A., Andrews, P., & Teasdale, G. (2008). Bryan Jennett and the field of traumatic brain injury; His intellectual and ethical heritage in neuro-intensive care. Intensive Care Medicine, 34(10), 1774–1778. Teasdale, G., & Jennett, B. (1974). Assessment of coma and impaired consciousness. Lancet, 2(7872), 81–84. Teasdale, G. M., Pettigrew, L. E., Wilson, J. T., Murray, G., & Jennett, B. (1998). Analyzing outcome of treatment of severe head injury: a review and update on advancing the use of the Glasgow Outcome Scale. Journal of Neurotrauma, 15(8), 587–597.

Jerk ▶ Myoclonus

Jerking ▶ Tremor

JFK Coma Recovery Scale ▶ Coma Recovery Scale

JHD ▶ Juvenile Huntington Disease

JLO ▶ Judgment of Line Orientation

Cross References ▶ Brain Death ▶ Glasgow Coma Scale ▶ Glasgow Outcome Scale ▶ Traumatic Brain Injury ▶ Vegetative State

References and Readings Jennett, B., & Bond, M. (1975). Assessment of outcome after severe brain damage. Lancet, 1(7905):480–484.

Job Advocacy A LLEN N. L EWIS JR., PAMELA H. L EWIS Virginia Commonwealth University Richmond, VA, USA

Synonyms Employment facilitation; Job coach; Job support

Job Analysis

Definition Job advocacy is a process of promoting vocational adjustment for individuals with disabilities by providing systematic and intentional support toward to the acquisition and maintenance of a job, and ultimately to normalize the employment experience. Job advocacy is a wraparound concept and ranges from providing upfront effort to secure employment, to the provision of an array of supports in varying degrees of intensity that may be needed to promote adjustment to a job by an individual with a disability (e.g., improving employer awareness of the nature of disability, developing accommodations, teaching the employee to be a self advocate, and problem solving), to include non-intensive ongoing support throughout one’s job tenure to ensure long-term employment success. Job advocacy encapsulates a range of deliberate interventions and strategies that vocational rehabilitation professionals deploy to support and enhance job performance, and thus, elevate employer recognition and valuing of the work performance of persons with disabilities. Before the advent of the Americans with Disabilities Act (ADA) in 1990, much job advocacy was targeted to securing employment sites that were willing to hire persons with disabilities and then providing the necessary supports to ensure success of the worker with a disability while on the job. Since the passage of the ADA, a significant portion of job advocacy continues to occur under the model of supported employment as well as some efforts now taking the form of legal advocacy when an employer is alleged to have discriminated against an employee on the basis of disability.

Cross References ▶ Reasonable Accommodations

References and Readings Blanck, P. D. (2000). Employment, disability, and the Americans with Disabilities Act: Issues in law, public policy, and research. Evanston: Northwestern University Press, Inc. Blitz, C., & Mechanic, D. (2006). Facilitators and barriers to employment among individuals with psychiatric disabilities: A job coach perspective. Work, 26, 407–419. Griffin, C., Hammis, D., & Geary, T. (2007). The job developer’s handbook: Practical tactics for customized employment. Baltimore: Paul H. Brookes Publishing Co., Inc. Moon, M., Inge, K., Wehman, P., Barcus, J., & Brooke, V. (1990). Helping persons with severe mental retardation get and keep employment. Baltimore: Paul H. Brookes.

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Walsh, P., Rafferty, M., & Lynch, C. (1991). The OPEN ROAD project: Real jobs for people with mental handicap. International Journal Rehabilitation Research, 14, 155–161. Wehman, P., Revell, W., & Kregel, J. (1998). Supported employment: A decade of growth and impact. American Rehabilitation, 24(1), 31–43.

Job Analysis A MY J. A RMSTRONG Virginia Commonwealth University Richmond, VA, USA

Definition Job analysis is the process of gathering and documenting accurate and objective data relevant to the requirements and outcomes of a job, including what a worker does, how the work is done, why the work is done, the materials used to complete the job, the context of the job, and the characteristics and skills required to complete the job. An evaluation of the context of the job addresses the work and organizational culture, the integration of the worker, as well as environmental conditions. The job analysis is conducted by writing down in sequence all major job duties and the time required to perform each, identifying and describing each job skill that the employee will be required to perform, identifying work-related interaction between employees, and summarizing the analysis of each job. The job analysis is a tool that assists in the job selection process. The steps to conduct a job analysis include interviewing the employer/supervisor, observing a coworker completing the job duty, identifying the skills that must be completed successfully to perform a job duty, identifying all tools and machinery required, considering any modifications/ accommodations that may be needed, determining the most efficient procedure to complete each skill, and eliminating or reducing unnecessary movement. The benefits of a job analysis include determining the fundamental tasks of a job, serving as a training plan for the employee, and assuring high-quality work on the part of the employment specialist working with the employee. Job analyses are often used as a resource to the employer in employee training and the development of job descriptions, as well as developing safety procedures on a jobsite.

Cross References ▶ Task Analysis

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References and Readings Griffin, C., Hammis, D., & Geary, T. (2007). The job developer’s handbook: Practical tactics for customized employment. Baltimore, MD: Paul H. Brookes. Rubin, S., & Roessler, R. (2008). Foundations of the vocational rehabilitation process. Austin, TX: Pro-Ed. Szymanski, E. M., & Parker, R. M. (2003). Work and disability: Issues and strategies in career development and job placement (2nd ed.). Austin, TX: Pro-Ed. Wehman, P., Inge, K. J., Revell, G., & Brooke, V. (2007). Real work for real pay: Inclusive employment for people with disabilities. Baltimore, MD: Paul H. Brookes.

Job Coach ▶ Employment Specialist ▶ Job Advocacy

Job Placement Specialist ▶ Employment Specialist

Job Restructuring S TEPHANIE A. KOLAKOWSKY-H AYNER Santa Clara Valley Medical Center, Rehabilitation Research Center San Jose, CA, USA

Synonyms Reasonable accommodations

Job Development Specialist ▶ Employment Specialist

Job Placement C HRISTOPHER WAGNER Virginia Commonwealth University Richmond, VA, USA

Definition Job placement involves connecting individuals with work that suits their current needs, interests, and abilities. An essential part of the rehabilitation process, recent research suggests that the appropriateness of job placement efforts may be the greatest single contributor to long-term vocational rehabilitation (VR) effort when compared to functional limitations, demographic features, or other VR services. There are several common approaches to job placement. Traditional placement involves placing individuals in jobs available to persons with disabilities, often lower-paid jobs that individuals can readily fill or can fill with minimal training. Networking involves consumers to also build their own community contacts, including contacts of work-related friends. Active involvement of consumers increases the likelihood of successful outcomes and maximally involves consumers in their career-building efforts.

Definition A reasonable accommodation under the Americans with Disabilities Act (ADA), job restructuring entails modifying the focus and responsibilities of a job so that the employee is better able to complete tasks, improve job performance, and may be more satisfied with the job. Job restructuring could include combining job duties, eliminating tasks, adding similar tasks (horizontal restructuring), revising methods of task completion, or reallocating or redistributing subsidiary portions of the job function that the employee cannot assume secondary to disability.

Cross References ▶ Job Advocacy ▶ Vocational Counseling

References and Readings (n.d). Americans with Disabilities Act (ADA) of 1990. Hodge, J. W., Crampton, S. M. (n.d). ADA: Easier said than done. Supervisory Management, 38(4), 9. Magill, B. (1997). ADA accommodations don’t have to break the bank. HRMagazine, 42(7), 84. Maslen, D. (n.d). Accommodation: What is reasonable? HR Focus, 69(1), 3. Perkins Nancy, L. (n.d). Defining employers’ obligations under the new disability act. Management Review, 81(1), 33.

Jobsite Training

Job Retention C HRISTOPHER WAGNER Virginia Commonwealth University Richmond, VA, USA

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Job Stability ▶ Job Retention

Job Support Synonyms

▶ Job Advocacy

Career maintenance; Job stability

Definition The extent to which an individual remains employed continuously over an extended period of time.

Job Training C HRISTOPHER WAGNER Virginia Commonwealth University Richmond, VA, USA

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Current Knowledge Definition Recent reenvisioning of vocational services focuses less on initial preparation for and placement of individuals into employment situation, and more on continuing success and advancement in the workforce. With employment viewed more as an ongoing process and less as an event, focusing on career success is intended to promote career growth and stability, which is in the best interest of both consumers and employers. On-the-job consultation and support services, usually provided by employment specialists, include training and coaching, consultation regarding productivity barriers and reasonable accommodations, and career enhancement efforts. Vocational rehabilitation providers recently increased their staffing of career maintenance specialists to accommodate this growing trend.

Cross References ▶ Employment Specialist ▶ Jobsite Training ▶ Supported Employment ▶ Vocational Rehabilitation

References and Readings Fraser, R., & Clemmons, D. (1999). Traumatic brain injury rehabilitation: Practical vocational, neuropsychological, and psychotherapy interventions. Boca Raton, FL: CRC Press LLC. Leach, S. (2002). A supported employment workbook. London: Jessica Kingsley Publishers.

Job training helps consumers prepare for a transition to working in a new field or role. Traditional job training occurs on a prevocational basis, with generalized training in a controlled training setting. Jobsite training limits prevocational training and focuses on providing jobspecific training in a workplace after initial placement. Evidence currently supports jobsite training as leading to better employment outcomes.

Jobsite Training C HRISTOPHER WAGNER Virginia Commonwealth University Richmond, VA, USA

Definition Jobsite training is a recent adaptation of job training. Whereas job training is typically provided in a preparatory setting, jobsite training limits prevocational training and focuses on providing job-specific training in a workplace after initial placement. Evidence currently supports jobsite training as leading to better employment outcomes. Jobsite training services may be expanded to include job coaching, consultation regarding productivity barriers and reasonable accommodations, and career enhancement efforts.

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Joiner v. General Electric (1997)

Joiner v. General Electric (1997) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition The Daubert criteria (▶ Daubert v. Merrell Dow for more detail) was further revised in the cases of Joiner v. General Electric (1997) and Kumho Tire v. Carmichael (1999). While the Daubert and Kumho decisions dealt exclusively with methodological bases underlying an opinion, the Supreme Court used the Joiner v. General Electric decision to further expand the gatekeeper role of the judge to include admissibility of expert’s conclusions. Specifically, in Joiner, a trial judge prohibited an expert witnesses’ testimony claiming that lung cancer was caused by PCB exposure because the expert’s process of deduction relied on irrelevant epidemiological and animal studies. The studies cited very different circumstances from the case at hand and in the animal studies involved much higher doses of PCB exposure. The Joiner court ruled that it was within a trial court’s discretion to disqualify opinion evidence based on no more than the fact the expert said it (ipse dixit), irrespective if the extrapolated-from-studies were themselves scientifically sound and reliable. Any extrapolation from literature to individual legal cases has to be bridged by links other than the expert’s belief that there is a link. In neuropsychology, this could mean that introduction of valid and replicated neuropsychological principles could still be barred if not generalizable. As an example, Grieffenstein (2008) points out that the General Neuropsychological Deficit Scale may be sensitive to metastasized brain cancer, but that does not automatically mean that it is generalizable to remote mild head trauma cases.

Cross References ▶ Admissibility ▶ Daubert v. Merrell Dow ▶ Kumho Tire v. Carmichael

References and Readings Daubert v. Merrell Dow, 509 U.S. 579 (1993). Frye v. United States, 1923.

Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Psychology Press. Joiner v. General Electric, 522 U.S., 136 (1997). Kumho v. Carmichael, 526 U.S., 137 (1999).

Joint Position Sense ▶ Proprioception

JOLO ▶ Judgment of Line Orientation

JPA ▶ Pilocytic Astrocytoma, Juvenile Pilocytic Astrocytoma

Judgment ▶ Reasoning

Judgment of Line Orientation FARZIN I RANI University of Pennsylvania Philadelphia, PA, USA

Synonyms BJLOT; JLO; JOLO

Description The Judgment of Line Orientation (JLO) test is a widely used measure of visuospatial judgment that was originally conceptualized by Dr. Arthur L. Benton et al. in 1978 (Benton, Varney, & Hamsher, 1978). This test measures

Judgment of Line Orientation

accuracy of angular orientation based on judgments about a pair of angled lines that visually match an identical pair immersed within a semicircular array of 11 lines. Patients are asked to indicate which two lines from the array on the bottom page of the spiral-bound stimulus book are in exactly the same position and point in the same direction as the two lines on the top page. Feedback is provided during five practice trials prior to initiating test items. Compared to the practice trials, lines in the test items have part of the line erased to increase task difficulty. A response is scored as being correct only when both lines are identified correctly. There is no time limit for responding; however, after 30 s the patient is typically encouraged to respond. The original format has 30 test items, although more recently, 15-item shorter versions have also been developed to provide quick screens for the presence or absence of visuospatial impairment (Qualls, Bliwise, & Stringer, 2000; Woodard et al., 1996). The JLO is currently published by Psychological Assessment Resources, Inc. The stimulus booklet is available in two alternate forms, Form H and Form V, which present the same items in a different order. A scoring form is also sold separately. The scoring form includes an evaluation for different types of line segments – distal (high), middle or proximal (low). HH, MM and LL consist of two distal, middle, and low line segments, respectively. There are also mixed items that consist of two different line segments, i.e., LH, HM, MH, LM. Score corrections are provided for age and sex. Administration and scoring instructions for the original format are available in Contributions to Neuropsychological Assessment, A Clinical Manual (Benton, Hamsher, Varney, & Spreen, 1983).

Historical Background According to the manual, the impetus for developing the JLO was based on an early study with university students that showed left visual field superiority in perceptual accuracy for direction of lines presented through a tachistoscope. Patients with unilateral disease had previously shown differences in error rates on a more simple task involving making decisions about differences in slopes between pairs of lines. Results indicated that patients with right hemisphere lesions, particularly right parietal lesions, performed worse than those with left hemisphere lesions and a control group. This led Benton, Hannay, and Varney (1975) to extend their prior normative work to a clinical population and develop a more complex task that required the identification of two simultaneously presented lines of different slopes. Stimuli were again

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presented tachistoscopically to patients with unilateral disease for 300 ms in central vision followed by the identification of lines on a multiple choice response card. Results indicated that patients with right hemisphere lesions scored below the poorest score in the control group, while none of the patients with left hemisphere lesions made ‘‘defective scores’’. Subsequently, a procedure that required matching of ‘‘partial’’ lines (1.9 cm in length) to ‘‘full length’’ lines (3.8 cm in length) was developed in order to increase task difficulty and make the test more convenient for clinical use. This work reported remarkably high frequency of ‘‘defective’’ performance in those with right hemisphere lesions and laid the foundation for clinical applications of the test to patients with brain disease. Since then, the JLO has been widely used in clinical and research settings. Fifteen-item short versions of the test have also been developed as alternatives to the full format along with corresponding normative data (Qualls et al., 2000; Woodard et al., 1998). However, authors of the short forms caution that the original JLO is the most appropriate test to categorize the level of impairment and the short form is only preferred when patient fatigue is a concern, when patients cannot tolerate lengthy assessment, or when lengthy assessment might damage the examiner’s rapport with the patient. Finally, computerized versions of the test have also been developed for use in research protocols (Gur et al., 2001).

Psychometric Data The manual for the original 30-item form reports splithalf reliability for Forms H and V to be 0.94 and 0.89. In combined samples, corrected split-half reliability was 0.91 with a standard error of measurement of 1.7. Test– retest reliability coefficient was reported to be 0.90 with a standard error of measurement of 1.8 points. Construct validity has also been demonstrated. High internal consistency has been reported for the long (0.90) and 15-item short (0.82) form (Qualls et al., 2000). Similarly, a short version using the odd/even item split for Form V has also been purported to show adequate internal consistency, validity, and utility for serial assessment in research studies (Woodard et al., 1996).

Clinical Uses Assessment of visuospatial functioning is an important aspect of the neuropsychological evaluation. Some measures of visuospatial functioning have an associated

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motor component, which makes the JLO an attractive option as a relatively pure measure of visuospatial function. While attentional and fatigue factors may impact performance on the full 30-item version, proponents of shorter versions have argued that it can be a valid and reliable screening measure of visuospatial abilities. Normative neuroimaging studies have shown that the cerebral region, or neurocognitive network node, specifically associated with JLO is located in the right ventral extrastriate cortex (Ng, Bullmore, de Zubicaray, Cooper, Suckling, & Williams, 2001), although men show some left lateralized activation not seen in women (Gur et al., 2000). Patients with right hemisphere damage, particularly right parietal lesions, are typically impaired as compared to patients with left hemisphere damage and controls. This has also been demonstrated in pediatric populations. Of note however, neither the original form nor the 15-item short form was able to distinguish right and left hemisphere stroke patients (Qualls et al., 2000) or determine lesion lateralization in a pediatric population (Paquier, van Mourik, Van Dongen, Catsman-Berrevoets, Creten, & Stronks, 1999). This has led to some questions about the discriminative validity of this task in the assessment of etiologically unselected populations with brain damage. Nevertheless, the JLO remains widely used and has demonstrated value in assessing visuospatial impairment in a number of patient populations. For instance, it has been used to demonstrate impairment in populations of pediatric neurofibromatosis, dementia, Parkinson’s disease, and traumatic brain injury.

Cross References ▶ Benton, Arthur (1909–2006) ▶ Perception ▶ Visual-Spatial Ability

References and Readings Benton, A. L., Hamsher, K., Varney, N. R., & Spreen, O. (1983). Contributions to neuropsychological assessment. New York: Oxford University Press. Benton, A. L., Hannay, H. J., & Varney, N. R. (1975). Visual perception of line direction in patients with unilateral brain disease. Neurology, 25(10), 907–910. Benton, A., Varney, N., & Hamsher, K. (1978). Visuospatial judgment. A clinical test. Archives of Neurology, 35(6), 364–367. Gur, R. C., Alsop, D., Glahn, D., Petty, R., Swanson, C. L., Maldjian, J. A., et al. (2000). An fMRI study of sex differences in regional activation to a verbal and a spatial task. Brain and Language, 74(2), 157–170.

Gur, R. C., Ragland, J. D., Moberg, P. J., Turner, T. H., Bilker, W. B., Kohler, C., et al. (2001). Computerized neurocognitive scanning: I. Methodology and validation in healthy people. Neuropsychopharmacology, 25(5), 766–776. Ng, V. W., Bullmore, E. T., de Zubicaray, G. I., Cooper, A., Suckling, J., & Williams, S. C. (2001). Identifying rate-limiting nodes in large-scale cortical networks for visuospatial processing: An illustration using fMRI. Journal of Cognitive Neuroscience, 13(4), 537–545. Paquier, P. F., van Mourik, M., Van Dongen, H. R., Catsman-Berrevoets, C. E., Creten, W. L., & Stronks, D. L. (1999). Clinical utility of the judgment of line orientation test and facial recognition test in children with acquired unilateral cerebral lesions. Journal of Child Neurology, 14(4), 243–248. Qualls, C. E., Bliwise, N. G., & Stringer, A. Y. (2000). Short forms of the Benton judgment of line orientation test: Development and psychometric properties. Archives of Clinical Neuropsychology, 15(2), 159–163. Woodard, J. L., Benedict, R. H., Roberts, V. J., Goldstein, F. C., Kinner, K. M., Capruso, D. X., et al. (1996). Short-form alternatives to the judgment of line orientation test. Journal of Clinical and Experimental Neuropsychology, 18(6), 898–904. Woodard, J. L., Benedict, R. H. B., Salthouse, T. A., Toth, J. P., Zgaljardic, D. J., & Hancock, H. E. (1998). Normative data for equivalent, parallel forms of the judgment of line orientation test. Journal of Clinical and Experimental Neuropsychology, 20(4), 457.

Juvenile HD ▶ Juvenile Huntington Disease

Juvenile Huntington Disease S COTT J. H UNTER , C HRISTINA C ASNAR University of Chicago Chicago, IL, USA

Synonyms Early-onset HD; Early-onset Huntington disease; JHD; Juvenile HD; Juvenile onset Huntington disease

Definition Juvenile Huntington disease (JHD) is a rare early presentation of Huntington disease (HD). It presents prior to 21 years of age. Like the adult form of the disease, JHD is a

Juvenile Huntington Disease

dominantly inherited neurodegenerative disorder. While the typical age of onset in patients with HD is usually in the fourth or fifth decade, individuals with JHD show an onset of symptoms within the first 2 decades of life. The wide variation in onset of HD symptoms is linked to the number of trinucleotide repeats in the Huntingtin (IT-15) gene on the short arm of chromosome 4, with longer CAG repeats suggesting earlier onset (i.e., 60 repeats typically seen with early onset; Gambardella et al., 2001). In adults, HD is usually characterized by involuntary choreic movements, characteristic psychiatric changes, and variable cognitive impairments, including changes in memory, attention, coordination of movement and thought, and executive functioning, with a gradual development of dementia. In contrast, JHD presents more commonly with motor rigidity, dystonia, oral motor dysfunction, epileptic seizures, rapid changes in cognition, and bradykinesia. At this time, JHD is untreatable, although medications are utilized to help manage the symptoms associated with the disease; these range from the use of pharmacological agents to slow cognitive decline to the use of psychopharmacological agents to treat psychiatric and behavioral changes (Hunter, 2007).

Epidemiology Individuals with JHD account for approximately 10% of all HD patients (Geevansinga, Richards, Jones, & Ryan, 2006; Hunter, 2007). Onset in the first 10 years of life is estimated to be about 0.5% of all HD patients (Ruocco, Lopes-Cendes, Laurito, Li, & Cendes, 2006), with the remainder showing onset during the second decade. As reviewed in the adult HD section, HD is seen across most cultures and ethnicities, with prevalence highest in Caucasian populations. Adult onset in males and females presents with a 50/50 chance of inheriting the defected gene from either parent. In contrast, of patients with JHD, 70% have an affected father (Telenius et al., 1993).

Natural History, Prognostic Factors, and Outcome The course of JHD is typically one of significant cognitive and behavioral changes and rapid declines. Current studies suggest that the disease progresses much more rapidly in early-onset versus adult-onset HD, with progression rate positively correlated with the number of trinucleotide repeats (Claes et al., 1995). There appear to be three

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defined phases to JHD: (1) an initial phase marked by gait disturbance, behavior changes, and learning difficulties, including slowed progression of intellectual development and academic skill acquisition; (2) development of seizures, rigidity, speech disturbances, and a more prominent decline in mental abilities; and (3) a final phase that includes bed confinement, hypotonia, and increased seizures, accompanied by substantial changes in engagement, communication, and cognition (Gonzalez-Alegre & Afifi, 2006). As with adult-onset HD, the duration and survival outcome is quite uncertain, due to wide variations in disease progression. Mortality of JHD is well documented, with progression to death usually within 20 years of diagnosis. Like adult-onset HD, death often results from physical incapacity and secondary illnesses, such as pneumonia.

Neuropsychology and Psychology of Juvenile Huntington Disease Clinical symptoms and signs first observed in JHD are a mix of cognitive, psychiatric, and neurological dysfunction (Hunter, 2007). Since JHD is a degenerative disorder, affected children often lose developmental skills that they have already acquired. A decline in cognitive skill is often first noticed in school performance and is a common complaint in referrals. Psychiatric and behavioral difficulties are also common in children with JHD. Psychiatric symptoms often differ depending on the age of onset, with younger children presenting with aggression and disruptive behavior. Adolescent-onset psychiatric symptoms can range from severe, including significant regulation difficulties leading to school suspension, the development of drug and alcohol addiction, and depression with suicide attempts to mild difficulties with mood, attention, and regulation (Ribot et al., 2007). Neurologic symptoms can be quite debilitating; in children with JHD, dystonia, rigidity, and seizures serve as frequent chief complaints in many cases (Gomez-Tortosa et al., 1998), and underscore difficulties with movement, engagement, and learning. Gomez-Tortosa and colleagues (1998) compared cognitive functioning between patients with juvenile onset, adult- and late-life onset, and healthy, nonaffected controls. Findings indicated that overall cognitive performance was globally impacted in patients with HD, regardless of age of onset. Notedly, patients with early-onset HD performed better than adult-onset patients on a set of neuropsychological tasks, including the Rey Complex Figure Test, Hooper Visual Organization Test, Stroop, Symbol Digit

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Modalities Test, Trail-Making Tests, and the California Verbal Learning Test. However, performances appeared worse for individuals with JHD on executive and prefrontal function measures other than the Stroop, when compared with adult- and late-onset HD. When compared to healthy controls, patients with JHD in this study showed a modest decline in global cognitive status, with the exception again being prefrontal functions, which were significantly lower. Ribot and colleagues (2007) found similar executive functioning deficits in children with HD. On neuropsychological examination, patients with JHD presented with primary attention deficits, general slowness in information processing, difficulties with working memory, and mental shift difficulties. Both studies highlight that the pattern of decline in JHD can be variable, but is progressive. Few systematic studies exist that focus on behavioral and psychological changes in patients with JHD. This is likely due to the rapid mental deterioration that manifests in early-onset HD (Hunter, 2007). Most case studies suggest a change in frontal regulation and increased aggression, which contribute to significant emotional lability and behavioral regulation changes. Case studies to date also suggest that depression is a common emotional response in JHD, similar to what is seen with HD. Psychological changes should be monitored closely.

Huntington gene and characterize the pattern of trinucleotide repeats present. While genetic testing is the most effective means to confirm JHD, its use is typically one of confirmation. It has been suggested that premature genetic testing may prove misleading or even damaging to a child’s future, as it could delay or prevent true diagnosis of a mimicking disorder (Seneca et al., 2004). Counseling should always be provided when genetic testing is ordered, particularly with regard to JHD, given its identification of risk for the disorder within the family. Once a diagnosis of JHD is confirmed, many neurologists take several steps to learn more about the prognosis of the disease, in order to more effectively guide and support the family (Geevansinga et al., 2006). Often, the neurologist will assess for motor dysfunction using the Unified Huntington’s Disease Rating Scale and rate functioning levels using the Total Functional Capacity (TFC) score. The TFC is modified for use in children, since it assesses domains such as employability and finances, which may not be relevant in JHD patients (Gomez-Tortosa et al., 1998). Neurologists will often use the Shoulson and Fahn criteria to estimate the stage of illness (Gomez-Tortosa et al., 1998); this is frequently supplemented with cognitive and behavioral data from neuropsychological assessment, in order to guide accommodation and disease management, both at home and in the educational setting (Hunter, 2007).

Evaluation of Juvenile Huntington Disease

Treatment

The diagnosis of JHD is often a detailed process (Geevansinga et al., 2006; Wojaczynska-Stanek, Adamek, Marszal, & Hoffman-Aacharska, 2006). Patients typically present first to their primary pediatrician, who will often then make a referral to a pediatric neurologist. Following the neurological exam, which often requires an exhaustive family history, in order to rule out possible genetic and environmental factors, EEG, MRI, and CT are typically conducted. Neuropsychological testing is often considered appropriate and necessary to assist in differential diagnosis and to characterize changes in cognition and behavior, which can be monitored and supported (Hunter, 2007; Ribot et al., 2007). Typical differential diagnoses include Tourette syndrome, Juvenile Parkinsonism, Wilson disease, Lesch–Nyhan syndrome, as well as neurologic sequelae of infection, drug-induced disorders, and metabolic disorders. The final stage in diagnosis is comprehensive genetic testing, to identify changes in the

At the present time, there is no known cure or specific medication that stops, reverses, or slows the progression of JHD. Medical professionals (pediatric neurologists and pediatricians) often prescribe medications and/or therapies to help alleviate symptoms and progressive changes associated with the disease. Motor control and movement difficulties are a common area of intervention. Specific medications are generally prescribed to reduce stiffness or rigidity. Common medications used to treat rigidity include anticholinergic agents, carbidopa–levodopa, dopamine agonists, and Botox injections (Geevansinga et al., 2006; Hunter, 2007). Notably, in general, these medications can worsen chorea symptoms. Occasionally, chorea is present in JHD patients. If this is true, many doctors will prescribe dopamine-blocking agents, dopamine-depleting agents, and benzodiazepines. Medical professionals must be cautious when prescribing these medications as severe side effects, such as depression, or


worsening of chorea and rigidity can occur in JHD patients. Patients who present with seizures are typically prescribed anticonvulsant medications. It is of note that none of the above medications have been FDA approved for the treatment of HD in children (Tupper, 2007). Physical therapy and occupational therapy are strongly recommended for patients with JHD (Hunter, 2007). The physical therapist should work with patients through the entire course of the disease, due to changes in functioning level in each phase of the disease process. Many different techniques can be explored in children with JHD, including gross motor supportive assistance, stretching, and massage. Occupational therapy can assist children with fine motor difficulties in adaptive domains such as eating, dressing, and toileting. The occupational therapist can provide equipment that can help improve patient independence. As with physical therapy, occupational therapists should work with patients with JHD through the entire phase of the disease. Occupational therapy may be particularly important in later phase of JHD, when oral motor skills diminish. There are no medications that fully help improve cognitive function, stop cognitive decline, or alter dementia. Medical professionals should be sure to refer JHD patients for neuropsychological testing to assess for symptoms that may affect daily adaptive functioning and learning, including symptoms consistent with ADHD, seizures, depression, and/or anxiety. Psychologists and physicians should work with parents and school personnel to ensure understanding of features associated with cognitive and behavioral decline, in order to support the development of an appropriate education plan (Hunter, 2007; Tupper, 2007). Children with JHD often experience severe psychiatric and behavioral difficulties. The most common psychiatric symptom reported in JHD is depression. Patients are often referred to work with a well-informed child psychiatrist to access the need for pharmacological management of their mood and behavioral symptoms (Hunter, 2007). Common antidepressant medications prescribed for children with JHD include tricyclic antidepressants, selective serotonin reuptake inhibitors (SSRIs), and serotonin-norepinephrine reuptake inhibitors (SNRIs). Children with JHD should be monitored closely due to side effects and the possibility of suicide attempts in early treatment. Behavioral treatments should be explored with a patient’s psychologist, social worker, and/or psychiatrist. Therapies to help manage aggression, explosive behavior, impulsiveness, violent tendencies, obsessions, and risky

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behaviors in children with JHD have been shown to improve behavior (Hunter, 2007).

Cross References ▶ Bradykinesia ▶ Dystonia ▶ Epilepsy ▶ Huntington Disease ▶ Rigidity ▶ Seizures

References and Readings Claes, S., Van Zand, K., Legius, E., Dom, R., Malfroid, M., Godderis, J., et al. (1995). Correlations between triplet repeat expansion and clinical features in Huntington’s disease. Archives of Neurology, 52(8), 749–753. Gambardella, A., Miglia, M., Latate, A., et al. (2001). Juvenile Huntington’s disease presenting as progressive myoclonic epilepsy. Neurology, 57, 708–711. Geevansinga, N., Richards, F. H., Jones, K. J., & Ryan, M. M. (2006). Juvenile hunting disease. Journal of Paediatrics and Child Health, 42(9), 552–554. Gomez-Tortosa, E., del Barrio, A., Ruiz, P. J., Pernaute, R. S., Benitez, J., Barroso, A., et al. (1998). Severity of cognitive impairment in juvenile and late-onset Huntington disease. Archives of Neurology, 55(6), 835–843. Gonzalez-Alegre, P., & Afifi, A. (2006). Clinical characteristics of childhood-onset (juvenile) Huntington disease: Report of 12 patients and review of the literature. Journal of Child Neurology, 12(3), 223–229. Hunter, S. J. (2007). Pediatric movement disorders. In S. J. Hunter & J. Donders (Eds.), Pediatric neuropsychological intervention (pp. 314–337). Cambridge, UK: Cambridge University Press. Ribot, P., Nguyen, K., Hahn-Barma, V., Gourfinkle-An, I., Vidailhet, M., Legout, A., et al. (2007). Psychiatric and cognitive difficulties as indicators of juvenile Huntington disease onset in 29 patients. Archives of Neurology, 64(6), 913–819. Ruocco, H. H., Lopes-Cendes, I., Laurito, T. L., Li, L. M., & Cendes, F. (2006). Clinical presentation of juvenile Huntington disease. Arquivos de Neuro-Psiquiatria, 64(1), 5–9. Seneca, S., Fagnart, D., Keymolen, K., Lissens, W., Hasaerts, D., Debulpaep, S., et al. (2004). Early onset Huntington disease: A neuronal degeneration syndrome. European Journal of Pediatrics, 163(12), 717–721. Telenius, H., Kremer, H., Theilmann, J., et al. (1993). Molecular analysis of juvenile Huntington’s disease: The major influence of CAGn repeat length is the sex of the affected parent. Human Molecular Genetics, 2, 1535–1540. Tupper, D. E. (2007). Management of children with disorders of motor control and coordination. In S. J. Hunter & J. Donders (Eds.), Pediatric neuropsychological intervention (pp. 338–365). Cambridge, UK: Cambridge University Press. Wojaczynska-Stanek, K., Adamek, D., Marszal, E., & HoffmanAacharska, D. (2006). Huntington disease in a 9-year old boy: Clinical course and neuropathologic examination. Journal of Child Neurology, 21(12), 1068–1073.

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Juvenile Myoclonic Epilepsy

Juvenile Myoclonic Epilepsy J EFFREY B. T ITUS 1,2 , R EBECCA K ANIVE 1, M ICHAEL M ORRISSEY 1 1 St. Louis Children’s Hospital St. Louis, MO, USA 2 Washington University, School of Medicine St. Louis, MO, USA

Synonyms Impulsive petit mal; Janz syndrome

Natural History, Prognostic Factors, and Outcomes Age of onset is a defining feature of JME, with about 80% of patients experiencing seizure onset between the ages of 12 and 18 years. Mean age of onset has been estimated to be about 14.6 years. Among the individual seizure types exhibited in JME, generalized tonic–clonic seizures have a mean age of onset of 15.5 years, while myoclonic and absence seizures are estimated to arise on average at about 15.4 and 11.4 years of age, respectively. JME is considered to be a lifelong condition requiring continuing treatment. The rate of recurrence after discontinuing antiepileptic medications is very high and occurs in almost every case.

Definition

Neuropsychology and Psychology of JME

Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epilepsy associated with the age of onset. Most cases are identified between 15 and 24 years, though JME can sometimes be diagnosed at younger or older ages. It is one of the most common forms of epilepsy in adolescents and is characterized by myoclonic jerks that occur shortly after waking. Generalized tonic–clonic seizures also typically emerge in combination with the myoclonic jerks. Absence seizures are common in JME, but they are less frequent and are estimated to occur in about one-third of cases.

Neuropsychological features

Epidemiology Incidence rates for JME vary between studies, likely partly due to differences in the age ranges that are included. Most incidence studies have been conducted in Scandinavian countries and suggest an incidence of six cases per 100,000 children under the age of 15 years. Studies completed in Minnesota and Iceland suggest similar rates for individuals below the age of 25 years. About 5–10% of newly diagnosed epilepsy cases are believed to meet criteria for JME. There is some evidence to suggest that JME is inheritable, with some research implicating genetic abnormality on chromosome 6. Maternal inheritance has also been suggested. Most studies examining gender representation in JME find equal distribution, although some research has suggested a greater incidence in females.

IQ among individuals with JME tends to be in the average range as a group, but they fall below control group levels in most studies. A growing body of literature supports the presence of neurocognitive deficits in JME that specifically affect aspects of executive functioning, attention, working memory, mental flexibility, inhibitory control, concept formation, planning, phonemic verbal fluency, and processing speed. Further research has suggested that such deficits are specifically caused by structural and functional abnormalities in the frontal lobes. This includes lowered N-acetylaspartate concentrations in the prefrontal cortex and reductions in gray matter volumes in the mesial frontal regions. Thalamofrontal dysfunction has also been suggested, specifically in relation to the anterior and medial nuclei of the thalami. Cognitive functions associated with regions outside of the frontal lobes have also been implicated, though less consistently. These include picture-naming and semantic verbal fluency. In addition, disruptions in visual-spatial processing and verbal and visual memory have been suggested. A 2007 study in Brazil found that, on average, patients with JME score more than 2.0 standard deviations below the mean on almost 15% of neurocognitive measures. This increases to about 22% when using a more conservative criterion of 1.5 standard deviations below the mean. The number of scores below 1.5 standard deviations increases in direct correlation to the duration of a patient’s epilepsy (i.e., time since first seizure). However, this appears to be mediated by education. That is, individuals with higher

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levels of education ( 11 years) are less affected by the duration of the epilepsy, suggesting the protective influence of cognitive reserve (Pascalicchio et al., 2007).

relying solely upon history and ictal or interictal EEG could lead to the misdiagnosis of partial seizures. This can have implications on the treatment plan that is developed.

Psychological features

Treatment

In addition to its cognitive morbidity, JME is also associated with an increased risk of psychiatric impairment. Rates as high as 35% have been reported, with up to 47% of JME patients experiencing a psychiatric condition at some point in their lifetime. This includes anxiety and mood disorder rates ranging as high as 47% and 39%, respectively, increasing in relation to higher seizure frequency. In addition to Axis I disorders, JME has been associated with a high rate of personality disorders with presumed association with frontal lobe dysfunction. The impact of psychosocial stressors on the high rate of personality disorders in JME is believed to be minimal because of the relatively late onset of seizures and the fact that personality disorders are more likely to be caused by psychosocial stressors earlier in childhood. The personality profile of patients with JME has been well-documented. They are often described as emotionally labile, with unstable self-concept and immature social adjustment. They can be unreliable and suggestible, and they can display limitations in stamina, drive, and discipline. Moreover, they often project denial and/or indifference about how their condition affects their lives.

Because of its efficacy, valproic acid is typically the drug of first choice in JME, especially among males. Weight gain is a common side effect of valproic acid, and the teratogenic effects associated with valproic acid make it less than ideal for females of childbearing age. Although not as effective as valproic acid, lamotrigine has been suggested as a reasonable alternative drug of first choice for females. Among newer antiepileptic drugs, levetiracetam has been suggested as a potential replacement drug for valproic acid due primarily to its impressive safety profile and high efficacy. Phenytoin, carbamazepine, and oxcarbazepine have been associated with an exacerbation of myoclonic and absence seizures in JME, suggesting that they should be avoided. Resistance to antiepileptic drug treatment in JME has been associated with the following: comorbid psychiatric problems, focal clinical or electrographic features, and the presence of all three associated seizure types (i.e., myoclonic jerks, tonic–clonic seizures, and absence seizures). Many clinicians also emphasize the combined efficacy of nonpharmacologic techniques in the management of JME. These include careful regulation of sleep cycles, avoidance of alcohol, and reduction of photo stimulation (e.g., using backlighting, sitting farther away from video screens, etc.).

Evaluation The clinical history is the most important element in diagnosing JME. Late diagnosis is common, often after the emergence of generalized tonic–clonic seizures. Retrospective discovery of myoclonic jerks at an earlier age is frequently established after a careful review of the patient’s history. The neurological examination in JME is typically normal, with tremor being noted in about one-third of cases. In JME, ictal and interictal electrographic features are similar. They are characterized by paroxysmal bursts of frontally predominant high amplitude 3–6 Hz spikes and polyspikes and slow waves that occur superimposed upon a normal EEG background. Bursts of this activity are more frequent during periods of state transition (drowsiness), may be photosensitive (about 30% of patients), and increase during the hyperventilation activation procedure. Myoclonic jerks may or may not be time-locked with the bursts. Video-EEG monitoring can be an extremely helpful tool in the diagnosis of JME. Because JME can sometimes be associated with asymmetric clinical or EEG features,

Cross References ▶ Absence Seizure ▶ Electroencephalography ▶ Epilepsy ▶ Lamotrigine ▶ Seizure ▶ Valproate

References and Readings Auvin, S. (2008). Treatment of juvenile myoclonic epilepsy. CNS Neuroscience & Therapeutics, 14, 227–233. Behrouz, R., & Benbadis, S. R. (2008). Idiopathic generalized epilepsy of adolescence. In J. M. Pellock, B. F. D. Bourgeois, & W. E. Dodson (Eds.), Pediatric epilepsy: Diagnosis and therapy (3rd ed., pp. 359– 366). New York, NY: Demos.

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Pascalicchio, T. A., De Araujo Filho, G. M., Da Silva Noffs, M. H., Lin, K., Caboclo, L. O. S. F., Vidal-Dourado, M., et al. (2007). Neuropsychological profile of patients with juvenile myoclonic epilepsy: A controlled study of 50 patients. Epilepsy & Behavior, 10, 263–267. Pulsipher, D. T., Seidenberg, M., Guidotti, L., Tuchscherer, V. N., Morton, J., Sheth, R. D., et al. (2009). Thalamofrontal circuitry and executive dysfunction in recent-onset juvenile myoclonic epilepsy. Epilepsia, 50, 1210–1219.

Juvenile Onset Huntington Disease ▶ Juvenile Huntington Disease

Juvenile Parkinson’s Disease C ONSTANCE D ROSSOS , S COTT J. H UNTER University of Chicago Chicago, IL, USA

Synonyms Early-onset Parkinson’s disease; Juvenile Parkinsonism; Young-onset Parkinson’s disease;

Short Description or Definition Juvenile Parkinson’s disease (JPD) is a rare, very early presentation of Parkinson’s disease (PD), an idiopathic, chronic neurodegenerative disorder that affects mainly subcortical brain structures. It is characterized by multiple neuropsychiatric problems. The main symptoms are rigidity, resting tremor, bradykinesia, and postural instability. Cognitive and psychiatric symptoms are also commonly observed (Garcia et al., 2007; Janvin, Aarsland, Larsen, & Hugdahl, 2003). JPD appears to show very similar signs to the clinical features of idiopathic PD, but with an onset typically during adolescence.

Categorization PD usually presents in individuals over the age of 50; earlyonset symptom presentation is very rare prior to the fourth decade of life, but does occur. As a result, there has been some controversy in the literature considering whether early-onset Parkinson’s disease (EOPD) is in fact a

‘‘true presentation’’ of PD (Muthane, Swamy, Satishchandra, Subhash, Rao, & Subbakrishna, 1994; Yoshimura et al., 1988). There appear to be two groups within the EOPD classification, specifically young-onset Parkinson’s disease (YOPD) patients, who show onset between 21 and 40 years of age, and JPD patients, who present with onset before 20 years of age. Of note, YOPD and JPD have been used interchangeably in the literature, contributing to some confusion in classification.

Epidemiology The prevalence of JPD is unknown; however, it is seen to be very rare. Most examples in the literature have been case studies, which has made it difficult to gather any true population estimates of prevalence or occurrence. Prevalence estimates for PD are considered at 1/1,000, with an increase in presentation with advanced age (Janvin et al., 2003). In contrast, the prevalence of PD before the age of 40 is reported as approaching 0.8 cases per 100,000 (Dowding, Shenton, & Salek, 2006).

Natural History, Prognostic Factors, and Outcomes Historically, the youngest case of JPD reported in the literature describes a 10-year-old female from Oklahoma, who reportedly showed symptoms at age 2. However, it was not until age 7 that doctors conclusively diagnosed JPD. The first reported case of JPD was in 1875. This report described a 3-year-old youngster presenting with classic symptoms of PD. Following this case report, additional isolated cases of children showing symptoms of PD were published, and the pattern observed with these cases suggested a strong familial distribution. These cases were identified as presenting with ‘‘Paralysis Agitans Juvenilis Familialis.’’ With the invention of drug treatment for PD, specifically levodopa (L-dopa) therapy, reports of juvenile PD continued to occur in the literature, with a focus on the effects of drug treatment. Case reports indicated that L-dopa therapy was initially successful for JPD, but patients soon developed response fluctuations and abnormal involuntary movements that were suggestive of disease progression and diminished pharmacological response. As reports of JPD have continued to arise in the literature, researchers have taken the opportunity to more closely examine the clinical features of JPD, as compared to YOPD and idiopathic PD, and to offer some comparisons. Patients with JPD, in contrast to

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patients with YOPD, present more frequently with dystonia as their primary concern. They also have higher rates of familial parkinsonism; one study indicated that 50% of patients with JPD were shown to have a genetically based disorder (Dowding et al., 2006). Cognitive dysfunction appears to occur at similar rates between YOPD and JPD (Muthane et al., 1994). Unfortunately, due to the rare occurrence of JPD, it is still difficult to make any true predictions regarding prognostic factors and outcomes. What is known from the literature on PD is that the disease tends to have a greater impact on patient’s lives when it starts at an earlier age; therefore, the assumption is that individuals diagnosed with JPD, given its chronic neurodegenerative course, will have a poorer prognosis than individuals who develop the disease later in life. Additionally, research has shown that when PD is untreated, it progresses to a state of total disability, leading to deterioration across multiple brain functions, and can lead to death. JPD is observed to show a similar pattern, with earlier morbidity and mortality. Treated PD does not prevent impairment in affected individuals; variability in response to treatment is related to individual variability in morbidity. A similar pattern is seen with JPD.

(1992) reported, in a study of 29 patients with JPD, that peripheral nervous system involvement was evident in 50% of their patients; this highlights primary concerns with motor control and coordination. There have also been a series of case reports of JPD highlighting cognitive and behavioral changes similar to individuals with classic onset PD. For example, Rye, Johnston, Watts, and Bliwise (1999) reported a case of an adolescent female, who first presented at age 16 with Parkinson-like symptoms, including excessive daytime sleepiness as a prevalent complaint. It is also important to consider health-related quality of life when thinking about patients with JPD. Dowding et al. (2006) examined quality of life in young-onset versus older-onset patients. Results indicated that health-related quality of life is significantly worse in young-onset versus older-onset patients. Young-onset patients were more likely to be unemployed, were in a lower socioeconomic class, and had more marital and familial discord. Their disease status placed greater strain on family members. This suggests that the impact of the disease on mental state and adaptation can contribute to the onset and severity of psychiatric symptoms in patients with JPD.

Neuropsychology and Psychology of JPD

Evaluation

As mentioned above, PD is associated with multiple neuropsychiatric disturbances. According to Garcia et al. (2007), the most common psychiatric symptoms are anxiety, depression, apathy, and hallucinations with preserved insight. Neuropsychological profiles of patients with PD have indicated that weaknesses in visuospatial abilities, motor planning and sequencing, memory (specifically, delayed recall, temporal ordering, and conditional associate learning), motor aspects of speech (probably attributed to vocal-motor deficits such as dysarthria), and overall executive functioning abilities are frequently observed. There have been mixed findings regarding deficits in attention. Depression has been associated with cognitive impairment in PD patients (Janvin et al., 2003; Muslimovic, Post, Speelman, & Schmand, 2005; Zgaljardic, Borod, Foldi, & Mattis, 2003). Garcia et al. (2007) reported that the severity of psychiatric symptoms in patients with PD correlates positively with the stage of disease and the degree of cognitive impairment observed, but not with the patient’s age, medication status, presence of dyskinesia, or duration of disease. To the best of our knowledge, there have not been any published studies looking at comprehensive neuropsychological profiles of patients with JPD. Taly and Muthane

There is no specific diagnostic test for JPD. Patients presenting to their physician with symptoms consistent with JPD will be given a thorough physical exam and the physician will take a complete medical history. Specific attention will be given to physical symptoms of PD, such as tremor, rigid muscles, and slow movements. Patients typically receive a series of medical and neurologic tests, such as brain scans, blood tests, lumbar puncture, and x-rays, to rule out any other contributing conditions. Once diagnosed with JPD, patients, family members, and health-care providers may complete such PD-related rating scales as Hoehn and Yahr’s PD questionnaire (Hoehn & Yahr, 1967), or the Columbia University PD rating scales (Duvoisin, 1971), that provide an indication of disease severity. Additionally, the Northwestern University Disability Scale (Canter, de La Torre, & Mier, 1961) and the Extensive Disability Scale (Martinez-Martin, Carrasco de la Pena, Ramo, Antiguedad, & Bermejo, 1987) both provide accurate and well-normed descriptions of physical incapacity and handicap in patients with PD. This allows for planning regarding daily living needs and accommodations for supporting adaptation (Ginanneschi, Degl’Innocent, Maurello, Magnolfi, Marini, & Amaducci, 1991).

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Juvenile Parkinson’s Disease

Treatment There is no specific treatment protocol for JPD. These patients are typically managed following idiopathic PD protocols. Because JPD is progressive and not curable, the goal of treatment is to control symptoms while managing side effects. Treatment plans are developed on an individual basis, given the complexity of the disease, along with consideration of symptom presentation and possible side effects of medications. Medications are primarily used in JPD to control and manage symptoms as they increase, given changes in the level of dopamine in the brain. L-dopa, which increases dopamine levels in the brain, is considered the ‘‘gold standard’’ first-line intervention for all forms of PD. Side effects of L-dopa include an increased risk of dyskinesia. There have also been case reports of patients with JPD developing pathologic gambling when prescribed L-dopa (Garcia et al., 2007). Other dopamine agonists such as pramipexole (Mirapex), ropinirole (Requip), and bromocriptine (Parlodel) are used; however, they are more commonly prescribed for women with PD who are over age 65. Two monoamine oxidase B inhibitors, Eldepryl and Azilect, have been used in patients with JPD; they show mild benefits in regard to functioning, but do not slow down the progression of symptoms. Medications such as Comtan and Azilect have also been used to address ‘‘off-time’’ symptoms, which are present when typical medications such as L-dopa are not working and the motor symptoms of parkinsonism are present (Stacy & Galbreath, 2008). There have been experimental surgical procedures such as implantation of deep brain stimulators or destruction of tremor-causing tissue, to reduce symptoms in individuals with PD, including JPD (Kovacs et al., 2008). There have also been case reports of transplantation of adrenal gland tissue, which has had variable results (Dunnett, Kendall, Watts, & Torres, 1997). Because JPD is a complicated disease, all aspects of the disorder need to be addressed and treated as possible. Depression and other psychiatric symptoms that are typically seen with JPD require specialized care. Individual psychotherapy, family therapy, and supportive counseling by a mental health professional; participation in a psychoeducational group program; coaching in how to develop strategies for coping with the diagnosis and its impact; and medication management of mood and behavioral concerns are frequent approaches recommended for treating JPD patients and their loved ones. For individuals still participating in educational programs, the use of neuropsychological testing is

advised, to assist in identifying patterns of strength and weakness neurocognitively, and to support the development of an appropriate accommodation plan. Given some of the physical limitations and associated motor complications, physical therapy, speech therapy, and occupational therapy may help promote function and independence.

Cross References ▶ Movement Disorders ▶ Parkinson’s Disease

References and Readings Canter, G. J., de La Torre, R., & Mier, M. (1961). A method for evaluating disability in patients with Parkinson’s disease. Journal of Nervous and Mental Disease, 133, 143–147. Dowding, C. H., Shenton, C. L., & Salek, S. S. (2006). A review of the health-related quality of life and economic impact of Parkinson’s disease. Drugs Aging, 23(9), 693–721. Dunnett, S. B., Kendall, A. L., Watts, C., & Torres, E. M. (1997). Neuronal cell transplantation for Parkinson’s and Huntington’s diseases. British Medical Journal, 53(4), 757–776. Duvoisin, R. C. (1971). The evaluation of extrapyramidal disease. In J. de Ajuriaguerra & G. Gauthier (Eds.), Monoamines noyaux gris centraux et syndrome de Parkinson (pp. 313–325). Geneve: George & Cie. Garcia, R. F., Ordacgi, L. B. S., Mendlowicz, M. V., de Freitas, G. B., Rosso, A. L., Nazar, B. P., et al. (2007). Treatment of juvenile Parkinson disease and the recurrent emergence of pathologic gambling. Cognitive and Behavioral Neurology, 20(1), 11–14. Ginanneschi, A., Degl’Innocent, F., Maurello, M. T., Magnolfi, S., Marini, P., & Amaducci, L. (1991). Evaluation of Parkinson’s disease: A new approach to disability. Neuroepidemiology, 10, 282–287. Hoehn, M., & Yahr, M. (1967). Parkinsonism: Onset, progression and mortality. Neurology, 17(5), 427–442. Janvin, C., Aarsland, D., Larsen, J. P., & Hugdahl, K. (2003). Neuropsychological profile of patients with Parkinson’s disease without dementia. Dementia and Geriatric Cognitive Disorders, 15, 126–131. Kovacs, N., Balas, I., Kellenyi, L., Janszky, J., Feldman, A., Llumiguano, C., et al. (2008). The impact of bilateral subthalamic deep brain stimulation on long-latency event related potentials. Parkinsonism and related disorders, 14, 476–480. Martinez-Martin, P., Carrasco de la Pena, J. L., Ramo, C., Antiguedad, A. R., & Bermejo, F. (1987). Inter-observer reproducibility of qualitative scales in Parkinson’s disease. Archivos de Neurobiologi (Madr), 50, 309–314. Muslimovic, D., Post, B., Speelman, J., & Schmand, B. (2005). Cognitive profile of patients with newly diagnosed Parkinson disease. Neurology, 65, 1239–1245. Muthane, U. B., Swamy, H. S., Satishchandra, P., Subhash, M., Rao, N., & Subbakrishna, D. (1994). Early onset Parkinson’s disease: Are juvenile and young onset different? Movement Disorders, 9(5), 539–544. Rye, D. B., Johnston, L. H., Watts, R. L., & Bliwise, D. L. (1999). Juvenile Parkinson’s disease with REM sleep behaviour disorder, sleepiness, and daytime REM onset. Neurology, 53(8), 1868–1870.

Juvenile Pilocytic Astrocytoma Stacy, M., & Galbreath, A. (2008). Optimizing long-term therapy for Parkinson disease: Levodopa, dopamine agonists, and treatmentassociated dyskinesia. Clinical Neuropharmacology, 31(1), 51–56. Taly, A. B., & Muthane, U. B. (1992). Involvement of peripheral nervous system in juvenile Parkinson’s disease. Acta Neurologica Scandinavica, 85(4), 272–275. Yoshimura, N., Yoshimura, I., Asada, M., Hayashi, S., Fukushima, Y., Sato, T., et al. (1988). Juvenile Parkinson’s disease with widespread Lewy bodies in the brain. Acta Neuropathologic, 77, 213–218. Zgaljardic, D. J., Borod, J. C., Foldi, N. S., & Mattis, P. (2003). A review of the cognitive and behavioural sequelae of Parkinson’s disease: Relationship to frontostriatal circuitry. Cognitive and Behavioral Neurology, 16(4), 193–210.

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Juvenile Parkinsonism ▶ Juvenile Parkinson’s Disease

Juvenile Pilocytic Astrocytoma ▶ Pilocytic Astrocytoma, Juvenile Pilocytic Astrocytoma

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K K Scale R ICHARD T EMPLE CORE Health Care Dripping Springs, TX, USA

▶ Faking Good, Bad ▶ L Scale ▶ Minnesota Multiphasic Personality Inventory ▶ True Response Inconsistency Scale (TRIN, MMPI) ▶ Validity Scales (MMPI) ▶ Variable Response Inconsistency Scale (VRIN, MMPI)

Synonyms Correction factor; Defensiveness

Definition Validity scale on the Minnesota Multiphasic Personality Inventory (MMPI) and its revisions designed to identify individuals with significant psychopathology, whose profiles appear normal due to under-responding of psychopathology. The scale is also used to provide a correction factor to certain clinical scales to account for defensive responding, although research has been critical of this correction factor. Adolescent profiles are never K-corrected. Extremely low scores sometimes indicate exaggeration or fabrication of symptoms, whereas extremely high scores suggest unwillingness to self-disclose symptoms. In neuropsychological evaluation, extreme defensiveness may bring in to question the validity of other sources of subjective information (e.g., from the clinical interview and other self-report measures). Readers are referred to the MMPI entry for a discussion of limitations of this self-report measure when used with neuropsychological populations (see also Gass, 2006 and Lezak, Howieson, & Loring, 2004).

References and Readings Gass, C. (2006). Use of the MMPI-2 in neuropsychological evaluations. In J. Butcher (Ed.), MMPI-2: A practitioner’s guide (pp. 301–326). Washington, DC: American Psychological Association. Graham, J. R. (2005). MMPI-2: Assessing personality and psychopathology (pp. 28–32). Oxford University Press. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

KABC-II ▶ Kaufman Assessment Battery for Children

KAIT ▶ Kaufman Adult Intelligence Test

Cross References ▶ F Minus K Index ▶ F Scale ▶ Fake Bad Scale

Kanner’s Syndrome ▶ Autistic Disorder


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Kaplan, Edith (1924–2009)

Kaplan, Edith (1924–2009) A MY A LDERSON Emory University/Rehabilitation Medicine Atlanta, GA, USA

Major Appointments

Boston Veterans Administration Hospital, Boston University School of Medicine, Dept. of Neurology and Psychiatry, Clark University, (Affiliate Professor) (1956–1985) Suffolk University, Boston, MA (1985–2009)

Distinguished Contributions Award, New England Psychological Association (1996) Distinguished Career Contributions Award, Massachusetts Psychological Association (1997) Inducted into the Psi Chi Honor Society, St. Anselm College, Manchester, New Hampshire (1997) Edith Kaplan Award established and announced at 75th birthday (1999) Arthur Benton Award, American Psychological Association, Division 40 (2004) Lifetime Distinguished Career Award, International Neuropsychological Society (2008) Distinguished Service and Contributions to the Profession of Psychology Award, American Board of Professional Psychology (awarded posthumously) (2010)


Distinguished Service Award, Massachusetts Speech and Hearing Association (1977) Recognized by National Head Injury Foundation (1982) Ezra Paul Psychological Service Award, Massachusetts Psychological Association (1984) Distinguished Clinical Neuropsychologist Award, National Academy of Neuropsychology (1993) Edith Kaplan Neuroscience Scholarship Fund established by MeritCare Medical Center, Fargo, North Dakota (1994)


Kaplan, Edith (1924–2009). Figure 1 Edith with a clock purse

In 1956, Dr. Kaplan joined the Boston VA Hospital and began her collaboration with Harold Goodglass. With a grant from the Social Science Research Council Study Group, Kaplan and Goodglass investigated the relation between aphasia and gestural impairments, finding that gestural impairments in individuals with aphasia resulted from specific limitations in praxis, as opposed to impairments in language (Goodglass & Kaplan, 1963). Interestingly, the praxic impairments shown by aphasic patients in this study appeared almost childlike in nature; for example, patients used body parts in place of imagined tools. Springing from the results of this study, Kaplan went on to investigate the development of gestural representation, and found that children developed praxic skills in an orderly progression (Kaplan, 1968). Building upon her early work with Heinz Werner and his emphasis on observation of the qualitative features of problem-solving, Kaplan developed her process approach to neuropsychological evaluation, creating numerous assessment tools that are widely used in the USA and worldwide. In conjunction with Goodglass in 1972, Kaplan published the first edition of the Boston Diagnostic Aphasia Examination. The second edition of the Boston Diagnostic Examination and the Boston Naming Test appeared in 1983, with a third edition in 2000. The Boston Naming Test is widely used by neuropsychologists and speech therapists and has been translated into numerous languages, including Spanish, French, German, Italian, Japanese, and


Chinese. Her emphasis on attention to process led to modifications of aspects of both the WAIS-R (WAIS-R NI) and WISC-III (WISC-III PI), with alterations made in administration and scoring to permit better characterization of qualitative aspects of individuals’ strategic approaches. She developed the California Verbal Learning Test with Dean Delis in 1987, with a revised edition emerging in 1994. With Delis, she developed the Delis–Kaplan Executive Function System to investigate components of those skills that comprise the domain of executive functioning. She was also instrumental in the creation of the Kaplan–Baycrest Neurocognitive Assessment and the Boston Qualitative Scoring System for the Rey–Osterrieth Complex Figure Test, as well as the development of a comprehensive scoring system for Clock Drawing. In 1976, Kaplan became the director for pre- and postdoctoral training at the Boston VA Hospital. Here, she was able to impart her unique Boston Process Approach to neuropsychological assessment to numerous trainees. The exemplary training environment offered by the Boston VA Hospital sprang not only from her vivacious teaching style, but also from the wealth of distinguished interdisciplinary faculty, including Norman Geschwind, Neslon Butters, and Laird Cermak. In 1983, Kaplan and Dean Delis founded the Boston Neuropsychological Foundation as a nonprofit organization to fund scholarships for deserving students.

Short Biography Edith Kaplan was born on February 16, 1924, in New York City to German immigrant parents. She was effectively an only child, as her brother succumbed to diphtheria at the age of 4. Her father was a baker, and the family frequently moved. The only reading material in the home consisted of Hebrew prayer books and Yiddish newspapers; she spoke only German until entering kindergarten. Prior to entering school, her time was spent with her mother learning crafts, and it was there that she developed her skill and interest in knitting. Upon entering kindergarten, Kaplan began learning English and became an avid reader. She attended elementary and secondary public schools in Brooklyn, New York. By middle school, she had become ardently interested in medicine and science, organizing a medical club of which she was the self-appointed president. Members visited a local psychiatric hospital and observed intake interviews with patient with serious mental illness. Exhibiting her

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diverse skills, Kaplan also joined the school newspaper, contributing a weekly column under her byline. Her creative writing instructor once pointed out what he considered to be a prime example of a dangling participle, picked from the words of her high school column: ‘‘and the boys come to school with their shirttails, among other things, hanging out.’’ Following high school, Kaplan enrolled at Brooklyn College, where she met Heinz Werner and became interested in developmental psychology. In his seminal paper, Process and Achievement: A Basic Problem of Education and Developmental Psychology, Werner expressed concern over the growing use of standardized test scores to assess cognitive function without recognition of the multifactorial nature of these assessment tools. He argued that the assessment of cognition should be based on careful monitoring of the behaviors en route to the solution, as opposed to a binary right-wrong scoring system. His process-oriented approach to assessment served as the basis for her Boston Process Approach to neuropsychological assessment. During her junior year of college, Kaplan worked with Werner to develop the Word-Context Test for children, composed of 12 nonwords embedded in six sentences that provided clues to word meaning. In collaboration with Werner, she proceeded to investigate the linguistic performances of 125 school-aged children of ages 8.5–13.5. Kaplan received her Bachelor of Arts degree from Brooklyn College in 1949 and immediately enrolled at Clark University, following her mentor, Heinz Werner. Influenced by the work of Piaget and Vygotsky, her Master’s thesis work investigated the difference between speech-for-the-self and speech-for-others. She received her Master of Arts degree in 1952, the same year that the results from her investigations of the Word-Context Test were published. In this same year, her son Michael was also born. In 1956, Kaplan had fulfilled all the requirements for her Ph.D., except the qualifying examinations and the German exam. She separated from her husband, Bernard Kaplan, and left Worcester for Boston to study with Harold Goodglass at the Boston VA Hospital. Serendipitously, Goodglass had just received 6 month’s worth of salary from the Social Science Research Council Study Group for the study of gesture in aphasia, which provided for Kaplan’s initial collaborative work with him. Fortunately, her initial collaboration with Goodglass extended to a 29-year working relationship with the Boston VA Hospital. Working with Goodglass, she developed a gesture and pantomime test to assess the extent to which gestural

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impairments in aphasics were related to praxic difficulties as opposed to language impairments. The results of their studies supported apraxia as the explanation for the gestural impairments found in aphasics. Spurred by these findings, Kaplan examined the development of gestural representation in her doctoral dissertation, discovering a developmental progression from: (1) diffuse movements, (2) manipulation of the object of the action, (3) indexical behavior or pointing to where the action should take place, (4) body-part-as-object, (5) holding without extent, and finally (6) holding with the extent of the object represented. After completing her dissertation, Kaplan continued to study gestural impairments. Her collaboration with Norman Geschwind on patient PJK, published in 1962, was later nominated as a landmark paper and republished in 1998 in Neurology. PJK, who underwent surgical removal of a large left frontal glioblastoma multiforme, had a right-handed grasp reflex that impaired his writing. Interestingly, his left-handed writing was also impaired, despite the absence of a grasp reflex in that hand. He was aphasic and apractic, but only with his left hand, a finding that was explained by the thinning of his corpus callosum. In 1972, Kaplan and Goodglass published the Boston Diagnostic Aphasia Examination (BDAE), which was revised in 1983 and again in 2000. In 1983, Kaplan and Goodglass also published the Boston Naming Test, which is also now in its second edition, to clarify the nature of naming problems. The Boston Diagnostic Aphasia Examination is not only widely used in English-speaking countries, but it has also been translated into Spanish, French, German, and other languages. Kaplan took over the pre- and postdoctoral training programs at the Boston VA Hospital in 1976. The thriving scientific community comprised of individuals, such as Norman Geschwind, Nelson Butters, Laird Cermak, Marlene Oscar Berman, Margaret Naeser, Frank Benson, Michael Alexander, and Harold Goodglass created one of the finest training programs in the country. As the director of clinical neuropsychological services and clinical training, she became further convinced that solitary reliance on standardized scores, in the absence of observations about qualitative aspects of performance, sharply limits the information obtained by neuropsychological examination. Kaplan’s emphasis on the process approach to neuropsychological assessment led to modifications in the WAIS-R and WISC-III to provide more process-oriented insights into brain-behavior relations. These modifications (WAIS-R NI, WISC-III PI) encompassed expansions

and modifications of the administration and scoring procedures to provide detailed analyses of the component neuropsychological processes of individuals’ performance across tasks. For similar reasons, Kaplan worked with Dean Delis to develop the California Verbal Learning Test (1987, 1994) as an alternative to the Rey Auditory Learning Test to provide more process-oriented list-learning information. The scores derived from the California Verbal Learning Test provide information about individuals’ approach to the task across a variety of variables, including semantic clustering, serial order learning, primacy versus recency, learning curve, proactive and retroactive interference, perseveration, and quality of intrusions. In collaboration with Delis, Kaplan founded also developed the Delis–Kaplan Executive Function System to more carefully evaluate executive functioning from a processoriented approach. Each task included in the Delis–Kaplan Executive Function System was chosen to identify key components underlying the executive processes of children and adults. Amidst her numerous other scholarly and training activities, Kaplan has been the president of the International Neuropsychological Society and Division 40 of the American Psychological Association. She has also served as the president of the Boston Neuropsychological Foundation, which she founded in conjunction with Dean Delis in 1983. In her later years, Kaplan served as a professor of psychology at Suffolk University in Boston. At her 75th birthday, she was honored by some of the most prominent names in the field of neuropsychology at a formal banquet that hosted some 250 friends, family, and colleagues. Her enthusiasm for the field of neuropsychology and her quest for knowledge remained unabated to the end, and she continued to attend scientific meetings, sharing thoughts, and imparting knowledge. Dr. Kaplan died on September 3, 2009. She was survived by her son and granddaughter, as well as hundreds of loving friends, colleagues, and students.

Cross References ▶ Boston Diagnostic Aphasia Examination ▶ Boston Process Approach ▶ Butters, Nelson (1937–1995) ▶ Delis–Kaplan Executive Functioning System ▶ Geschwind, Norman (1926–1984) ▶ Goodglass, Harold (1920–2002)

Katz Adjustment Scale


Cross References

Delis, D. C., Kaplan, E., & Kramer, J. H. (2001). Delis-Kaplan executive function system. San Antonio, TX: The Psychological Corporation. Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (2000). The California verbal learning test (2nd ed.). San Antonia, TX: The Psychological Corporation. Freedman, M., Leach, L., Kaplan, E., Winocur, G., Shulman, K., & Delis, D. C. (1994). Clock drawing: A neuropsychological analysis. New York: Oxford University Press. Goodglass, H., & Kaplan, E. (1963). Disturbance of gesture and pantomime in aphasia. Brain, 86, 708–720. Goodglass, H., Kaplan, E., & Barressi, B. (2000). Boston diagnostic aphasia examination (3rd ed.). Baltimore, MD: Lippincott, Williams and Wilkins. Kaplan, E. (1983). Process and achievement revisited. In S. Wapner & B. Kaplan (Eds.), Toward a holistic developmental psychology. Hillside, NJ: Lawrence Erlbaum Associates. Kaplan, E. (1988). A process approach to neuropsychological assessment. In T. Boll & B. K. Bryant (Eds.), Clinical neuropsychology and brain function: Research measurement, and practice. Washington, DC: American Psychological Association. Kaplan, E. (2002). Serendipity in science. A personal account. In A. Y. Stringer, F. L. Cooley, & A. Christensen (Eds.), Pathways to prominence in neuropsychology: Reflections of twentieth-century pioneers. New York: Psychology Press. Kaplan, E., Fein, D., Morris, R., Kramer, J. H., & Delis, D. C. (1991a). The WAIS-R NI. San Antonio, TX: The Psychological Corporation. Kaplan, E., Fein, D., Morris, R., Kramer, J. H., & Delis, D. C. (1991b). The WISC-III as a process instrument. San Antonio, TX: The Psychological Corporation. Kaplan, E., Goodglass, H., & Weintraub, S. (1983). The Boston naming test. Philadelphia: Lea & Febiger. Stern, R. A., Javorsky, D. J., Singer, E. A., Singer Harris, N. G. Sommerville, J. A., Duke, L. M., et al. (1999). The Boston qualitative scoring system (BQSS) for the Rey-Osterrieth Complex Figure. Odessa, FL: Psychological Assessment Resources.

▶ Correlation Coefficient

Kappa Coefficient M ICHAEL F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition The kappa coefficient, sometimes known as Cohen’s kappa after its originator, is a nonparametric statistic that describes the association between categorical variables. It was originally developed as a way to assess agreement among raters when subjective qualitative judgments were made. It can be thought of as a correlation coefficient describing the relation or shared variance between two categorical-level variables.

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References and Readings Brennan and Prediger (1981). Coefficient kappa: Some uses, misuses, and alternatives. Educational and Psychological Measurement, 41, 687–699.

KAS-R ▶ Katz Adjustment Scale

KAS-RR ▶ Katz Adjustment Scale

Katz Adjustment Scale M ARK A. S ANDBERG Independent Practice Smithtown, NY, USA

Synonyms KAS-R; KAS-RR; Katz adjustment scales-relative report form

Description The Katz adjustment scales-relative report form (KAS-RR) is a 178-item, self-report instrument with inputs from close relatives of the patient principally used to assess the community adjustment of individuals following receipt of psychiatric care. Persons closest to patients, typically family members, rate behavior during the previous several weeks in three dimensions: (a) general behavior (100 items), (b) socially expected activities (32 items), and (c) use of leisure time (46 items). The scales were originally available in two forms: self-report and relative-report. Over time the relative report form demonstrated greater predictive capacity as a measure of community adjustment and potential for requiring

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Katz Adjustment Scale

rehospitalization. Administration time for the KAS-R can vary from 10 to 45 min depending on whether all three parts of the measure are completed and whether the examiner chooses the long (100-item) or short (48-item) form of Part I.

Historical Background With the advent of powerful psychotropic medications in the late 1950s, whose purpose was to treat the acute and more flagrant psychiatric symptoms of those with serious mental illnesses, the need arose to objectively assess the degree to which community reintegration was accomplished. Recognizing this need, the KASs were developed in the early 1960s for the purpose of evaluating long-term community adjustment following psychotropic intervention. The intent of the KAS was to provide a psychometrically sound means to assess not only the presence and severity of psychopathology but also to ascertain social functioning within real-world community settings, or as the manual states, ‘‘the degree to which he or she was adjusted’’ (Katz & Warren, 1998: 37). The original version (KAS) consisted of both a selfreport and a relative report component, but appraisals from significant others were viewed as having greater utility in research and clinical settings. The current measure (KAS-R) reflects that evolution. In its current state of development, the KAS-R continues to serve as a measure of how pathology affects patients’ adjustment and social behavior in community settings and the extent to which rehabilitation services might be required. Katz and Warren (1998) noted that the KAS-R is not designed as a screening test for medical or psychiatric syndromes.

Current Knowledge As stated in the KAS-R manual (Katz & Warren, 1998), ‘‘individuals are rated by a significant other on items describing aspects of their general behaviour, how ably they fulfil their social roles, and how productively they use their leisure time’’ (p. 3). The general behaviors portion of the KAS-R (Part I) yields a total of 22 scores and indices. Using a 4-point Likert scale, scores include a General Psychopathology score, a Stability score, and scores in four specific index areas: Social Aggression (belligerence, negativism, and verbal expansiveness), Emotionality (anxiousness and nervousness comprise the Anxiety Index, and depression and helplessness make up the

Depression Index), Disorientation/Withdrawal (confusion, expressive deficit, and withdrawal/retardation), and Severe Psychopathology (bizarreness, hyperactivity, and suspiciousness). In addition, a score for inconsistent responding is also calculated to address questions of symptom validity. Part II of the KAS-R assesses socially expected activities of the patients and yields two scores: Performance of Socially Expected Activities and Expectations for Performance of Socially Expected Activities. Part III comprises 23 items that examine patients’ Use of leisure time and is described in the manual as a good prognostic measure concerning the likelihood of rehospitalization. The KAS-R manual provides detailed information to assist with the interpretation of patient scores, including the meaning of scale elevations (i.e., T score of 60). The authors stressed that information from the KAS-R should be combined with other sources of information when making treatment decisions. The KAS-R can be handscored or scored through several computerized scoring options (i.e., mail-in or PC-based) offered through Western Psychological Services Test Report Services. Internal consistency estimates of the KAS-R in clinical and nonclinical samples range from 0.61 to 0.87 and 0.62 to 0.98, respectively. Scores from the KAS-R short form were judged to be ‘‘equivalent to their long form counterparts’’ (Katz & Warren, 1998: 52). The median correlation coefficient between parents’ ratings was 0.72. The manual provides ‘‘modest’’ test–retest reliability coefficients which range from 0.50 to 0.79 depending upon the study, but the test authors noted that modest correlations between ratings is concordant with the expectation that greater adjustment over time will result in most cases as a result of treatment and community reentry. Validity estimates concerning the KAS-R’s construct, predictive, and discriminant validity is provided in the manual. The instrument correlates well with other measures of psychopathology, including the Minnesota Multiphasic Personality Inventory (MMPI), Brief Psychiatric Rating Scale, and other relevant measures of psychopathology and personality, thus supporting its construct validity. Parents’ KAS-R ratings also correlate highly with clinician and staff ratings of adjustment, although the level of agreement varies depending upon the patients’ psychiatric disorder. Studies are also referred to in the manual which support the KAS-R predictive validity. For example, KAS-R scores obtained on the individuals admitted into day treatment programs were found to be significantly higher among those who had relapsed within a year after treatment than for those who did not require that level of care. The KAS-R has also been studied as a measure in medical rehabilitation, including with patients who sustained head injuries. For example, Dodwell (1988)

Katz Index of ADLs

reported a correlation of 0.78 between KAS-R P-Role scores (Socially Expected Activities) and patient ratings on similar measures, and viewed ratings on this particular scale to be a highly useful measure of social recovery. Greene and Clopton (2004) provide a comprehensive review of the KAS-R in the book edited by Mark Maruish titled The Use of Psychological Testing for Treatment Planning and Outcomes Assessment: Volume 3: Instruments for Adults (3rd ed.). Included is discussion of the instrument’s usefulness in planning and monitoring treatment and in assessing treatment outcome in the rehabilitation of patients with traumatic brain injury.

References and Readings Dodwell, D. (1988). The heterogeneity of social outcome following head injury. Journal of Neurology, Neurosurgery and Psychiatry, 51, 833–838. Greene, R. L., & Clopton, J. R. (2004). Minnesota multiphasic personality inventory-2 (MMPI-2). In M. E. Maruish (Ed.), The use of psychological testing for treatment planning and outcomes assessment: Volume 3: Instruments for adults (3rd ed., pp. 719–747). Mahwah, NJ: Lawrence Erlbaum Associates Publishers. Katz, M. M., & Warren, W. L. (1998). Katz adjustment scales relative report form manual. Los Angeles: Western Psychological Services.

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Description The Katz Index of Activities of Daily Living (Katz ADL) is designed to assess functional status, specifically, the client’s ability to perform activities of daily living (ADLs) independently. Clinicians will typically use the tool to detect problems in performing activities of daily living to either predict the future function and level of care or for treatment planning purposes. The index ranks adequacy of performance in the following six functions: bathing, dressing, toileting, transferring, continence, and feeding. If no supervision, direction, or personal assistance is required, then 1 point is given to that functional activity. If the client requires supervision, direction, personal assistance, or total care, then a 0 is assigned to that functional activity. A total score of 6 indicates full function, 4 indicates moderate impairment, and 2 or less indicates severe functional impairment. Although the index was developed from observations of older adults after hip fractures, it has been used with adults with musculoskeletal and neurological impairments and with community-dwelling older adults. Administration time varies depending on the individual’s disability; however, a 2-week observation is recommended prior to completion.


Katz Adjustment Scales-Relative Report Form ▶ Katz Adjustment Scale

Katz ADL ▶ Katz Index of ADLs

Katz Index of ADLs M ICHELLE M ARIE T IPTON -B URTON Santa Clara Valley Medical Center San Jose, CA, USA

Synonyms Katz ADL

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The Katz index is one of the oldest (1963) indices for assessing activities of daily living. The original form included six items, with each item rated on a 3-point scale (independence, receives assistance, and dependent), which is converted to an independent/dependent rating (with the ‘‘receives assistance’’ falling under independent for some items and dependent for others). Although the Katz ADL index is sensitive to changes in declining health status, the tool is limited in its ability to measure small increments of functional change seen in the rehabilitation of older adults. The Katz inventory is very useful in creating a common language about patient function and assists all practitioners in overall care planning and discharge planning.

Psychometric Data Internal consistency: Based on the random sample of South Carolina residents (n = 6.472) using a five-item telephone instrument, Cronbach’s alpha was 0.87 (Ciesla et al., 1993); in a group of frail elderly persons (n = 83) with the urinary continence item deleted, Cronbach’s alpha was 0.56 (Reuben et al., 1995).

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Kaufman Adult Intelligence Test

Inter-rater reliability: Examined based on a number of differences between raters, and, in all cases, the interobserver variability was low (Brossom & Asberg, 1984). Content validity: The Guttman scaling was originally based on the development and anthropological hierarchies. A very high percentage (96%) of subjects could be classified by the index (Katz, Ford, Moskowitz, Jackson, & Jaffe, 1963). Brorsson and Asberg (1984) report coefficients of scalability ranging from 0.74 to 0.88. Construct: Katz ratings were found to predict the length of stay in hospital, type of discharge, actual residence 1 year post-op, and mortality in clients in acute care (Brorsson & Asberg, 1984). Scores were also predictive of discharge location, length of stay in rehabilitation, and mortality (Asberg & Nydevik, 1991; Hermodsson & Ekdahl, 1991).

Clinical Uses The Katz ADL tool was designed to assess the basic activities of daily living with the older adult, but it can easily be utilized with adults of other ages with various neurological and orthopedic deficits to establish an ADL baseline. Although it is suggested that the assessment be over a 2-week observation, it could be administered within a couple of treatment sessions to establish a baseline.

Cross References ▶ Functional Assessment Measure

References and Readings Asberg, K. H., & Sonn, U. (1988). The cumulative structure of personal and instrumental ADL: A study of elderly people in a health service district. Scandinavian Journal of Rehabilitation Medicine, 21, 171–177. Brosson, B., & Asberg, K. H. (1984). Katz index of independence in ADL: Reliability and validity in short-term care. Scandinavian Journal of Rehabilitation Medicine, 16, 125–177. Hartigan, I., & McAuley, C. (2009). A comparative review of the Katz ADL and the Barthel Index in assessing the activities of daily living of older people. International Journal of Older People Nursing, 2(3), 204–212. JOURNALS. Wiley InterScience, 16 August 2007. Web. July 29, 2009. <www.interscience.wiley.com/journal/118500995/abstract>. Katz, S. (1983). Assessing self maintenance: Activities of daily living, mobility and instruments of daily living. JAGS, 31(12), 721–726. Katz, S., Ford, A. B., Moskowitz, R. W., Jackson, B. A., & Jaffe M. W. (1963). Studies of illness in the aged: The index of ADL: A standardized measure of biological and psychosocial function. JAMA, 185(12), 94–99.

Kaufman Adult Intelligence Test N OELLE E. C ARLOZZI Kessler Foundation Research Center West Orange, NJ, USA

Synonyms KAIT

Description The Kaufman Adult Intelligence Test (KAIT; Kaufman & Kaufman, 1993) is a theoretically driven intelligence test designed to measure both adolescent and adult intelligence and provide clinical and neuropsychological data for individuals 11–85 plus years of age. The KAIT consists of a Core Battery (60 min administration time) and an Expanded Battery (requiring an additional 26 min to complete; Table 1). The Core Battery consists of six subtests; three focus on crystallized intelligence, or verbal and school-related skills (Definitions, Auditory Comprehension, and Double Meanings), and three on fluid intelligence, or nonverbal skills and the ability to solve new problems (Rebus Learning, Logical Steps, and Mystery Codes). The Expanded battery includes an additional four subtests: Memory for Block Designs (can be used for an invalidated Fluid subtest), Famous Faces (can be used for an invalidated Crystallized subtest), Rebus Delayed Recall, and Auditory Delayed Recall. All subtests were standardized to have a mean of 10 and a standard deviation of 3. Scoring is objective for all subtests except for Auditory Comprehension and Auditory Delayed Recall which require the examiner to match participant responses to responses listed in the manual. Standardization consisted of 2,000 individuals stratified by age, gender, geographic region, socioeconomic status, and race/ethnic group.

Historical Background Theoretically, the KAIT is based on Horn and Catell’s (1966, 1967) model of fluid and crystallized intelligence which posits that intelligence consists of two different


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Kaufman Adult Intelligence Test. Table 1 The Kaufman Adult Intelligence Test (KAIT) core and expanded test battery KAIT subtest

Battery

Scale

Description

Assessing

Definitions

Core

Crystallized This task involves the presentation of incomplete words, as well as clues about meaning (30 s time limit).

Word knowledge and verbal concept formation

Auditory Core comprehension

Crystallized This task requires the participant to listen to six stories Attention span, memory, of increasing complexity and respond by answering inferential logic both literal and inferential questions.

Double meanings

Crystallized This task requires the participant to determine a word Verbal concept formation that is semantically appropriate for two sets of word and semantic flexibility pairs.

Core

Rebusa learning Core

Fluid

In this task, the participant is taught a series of symbols that are then combined into sentences that the participant must translate.

Logical steps

Core

Fluid

This task includes the presentation of one or more Deductive and syllogistic facts needed for the participant to logically figure out reasoning a problem (30 s time limit).

Mystery codes

Core

Fluid

In this task, the participant is presented with pictorial Complex problem solving (as defined by Piaget) stimuli and associated codes that are expressed as symbols; the participant must figure out the relationship of the pictures with the codes to determine the code of a novel pictoral stimulus.

Famous faces

Expanded Crystallized In this task, the participant is shown pictures of famous people and provided with verbal clue regarding their identities.

Memory for block designs

Expanded Fluid

Rebusa delayed Expanded Fluid recall Auditory delayed recall

Paired associate learning, visual memory, and visual sequencing

General factual knowledge

This task involves the presentation of six blocks Nonverbal abstract containing six faces for 5 s; the participant is asked to concept formation and reproduce this design presentation within 45 s. visuo-spatial memory This task requires long-term memory for the visual information presented in Rebus Learning.

Delayed recall

Expanded Crystallized This task requires long-term memory for the auditory Delayed recall information presented in Rebus Learning.

a

A Rebus is a pictogram that represents a syllabic sound

components: the ability to use reasoning to solve problems (fluid intelligence) and the ability to use previously acquired skills to solve problems (crystallized intelligence). Further, the KAIT has theoretical underpinnings from Piaget’s (1972) developmental framework with an emphasis on deductive reasoning, and from Luria’s (1966, 1980) premise that emphasizes the attention, planning, decision-making, impulse control and emotional development as it relates to frontal lobe development. During the development of the KAIT, 1,850 items across 28 subtests were refined. After item analysis, reliability and factor analysis, the test was refined to include the ten subtests in the 1993 published version explained earlier.

Psychometric Data The KAIT has demonstrated both reliability and validity. Using the Spearman–Brown formula, split-half reliability values range from 0.79 to 0.93 for the individual subtests 0.95 for Crystallized IQ, 0.95 for Fluid IQ, and 0.97 for Composite IQ (Kaufman & Kaufman, 1993). Test-retest reliability coefficients, with intervals ranging between 6 and 99 days, range from 0.72 to 0.95, with Logical Steps, Mystery Codes, Memory for Blocks, and Auditory Delayed Recall exhibiting the lowest coefficients (Kaufman, & Kaufman, 1993). Consistent with theory, crystallized abilities (as measured by the KAIT) appear to increase with age until age 50, and

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then decrease after age 75, while Fluid abilities (as measured by the KAIT) appear to peak in the mid-1920s, plateau through the mid-1950s, and then decline after age 55 (Kaufman & Horn, 1996). Further, males appear to perform better than females on Memory for Block Designs, Logical Steps, and Famous Faces, whereas females perform better than males on Double Meanings (Kaufman & Horn, 1996). There is also evidence that African Americans and Hispanics perform more poorly than Caucasians on most subtests of the KAIT, even when education is included as a covariate, and that Hispanics outperform African Americans on tests of Fluid ability (Kaufman, Chen, & Kaufman, 1995; Kaufman, McLean, Kaufman, & Kaufman, 1994; Kaufman, McLean, & Kaufman, 1995). The KAIT has demonstrated good construct validity and concurrent validity. Most studies have found support through exploratory and confirmatory factor analyses suggesting a two-factor solution paralleling Crystallized and Fluid Intelligence (Caruso & Jacob-Timm, 2001; Kaufman, 1993; Kaufman & Kaufman, 1993; Kaufman et al., 1995). One study found support for a more contemporarymodel of Horn and Cattell’s theory (Horn, 1994) which includes nine factors (as opposed to the fluid/crystallized dichotomy; Flanagan & McGrew, 1998). The KAIT has also demonstrated concurrent validity with the WAIS-R (Wechsler, 1981), Stanford Binet Intelligence Scale-fourth edition (Thorndike, Hagen, & Sattler, 1986), K-ABC (Kaufman & Kaufman, 1993), General Ability Measure for Adults (Lassiter, Matthews, Bell, & Maher, 2002), WISC-R (Wechsler, 1974), and WISC-III (Vo, Weisenberger, Becker, & Jacob-Timm, 1999; Wechsler, 1991; Woodrich & Kush, 1998).

neurological impairment (Kaufman & Kaufman, 1993) and between controls and individuals with Alzheimer’s disease (Kaufman & Kaufman, 1993). Further, Rebus Learning, Rebus Delayed Recall, Famous Faces and Memory for Block Designs discriminate between patients with right versus left hemisphere damage (Kaufman & Kaufman, 1993). In addition, depressed participants have significantly lower, delayed than immediate memory scores relative to matched controls (Grossman, Kaufman, Mednitsky, Scharff, & Dennis, 1993). Finally, when the relationship between IQ (KAIT) and personality (Meyers-Briggs) was examined, individuals that are Intuitive (individuals who perceive possibilities and relationships via insights) have higher IQ scores than those who are Sensing (individuals who emphasize immediate experiences and practicality; Kaufman, McLean, & Lincoln, 1996).

Cross References ▶ Abstract Reasoning ▶ Academic Skills ▶ Intelligence ▶ Kaufman Assessment Battery for Children ▶ Kaufman Brief Intelligence Test ▶ Problem Solving ▶ Reasoning ▶ Standardized Tests ▶ Stanford–Binet Intelligence Scales and Revised Versions ▶ Wechsler Adult Intelligence Scale (All Versions) ▶ Wechsler Memory Scale (All Versions) ▶ Woodcock–Johnson Cognitive-Achievement Battery

References Clinical Uses The KAIT has been criticized for including instructions that are long, complicated, and require visual and auditory stimulation, making it difficult to administer people with auditory, visual, or short-term memory difficulties (Brown, 1994). Regardless, there is some evidence that the KAIT is comparable to the WAIS-R in identifying college students with learning disabilities (LD), although neither test yielded significant differences in scores between LD and controls (Morgan, Sullivan, Darden, & Gregg, 1997). The KAIT can also discriminate between children with scholastic difficulties and children with central nervous system disorders (Woodrich & Kush, 1998), between controls and individuals with

Brown, D. T. (1994). Reviews and critiques of school psychology materials: Review of the Kaufman Adolescent and Adult Intelligence Test (KAIT). Journal of School Psychology, 32(1), 85–99. Caruso, J. C., & Jacob-Timm, S. (2001). Confirmatory factor analysis of the Kaufman Adolescent and Adult Intelligence Test with young adolescents. Assessment, 8(1), 11–17. Flanagan, D. P., & McGrew, K. S. (1998). Interpreting intelligence tests from contemporary Gf-Gc theory: Joint confirmatory factor analysis of the WJ-R and KAIT in a non-white sample. Journal of School Psychology, 36(2), 151–182. Grossman, I., Kaufman, A. S., Mednitsky, S., Scharff, L., & Dennis, B. (1993). Neurocognitive abilities for a clinically depressed sample versus a matched control group for normal individuals. Psychiatry Research, 51, 231–244. Horn, J. L. (1994). Theory of fluid and crystallized intelligence. In R. J. Sternberg (Ed.), Encyclopedia of human intelligence (pp. 443–451). New York: Macmillan.

Kaufman Assessment Battery for Children Horn, J. L., & Catell, R. B. (1966). Refinement and test of theory of fluid and crystallized intelligence. Journal of Educational Psychology, 57, 253–270. Horn, J. L., & Catell, R. B. (1967). Age differences in fluid and crystallized intelligence. Acta Psychologia, 26, 107–129. Kaufman, A. S. (1993). Joint exploratory factor analysis of the Kaufman Assessment Battery for Children and the Kaufman Adolescent and Adult Intelligence Test for 11–12 year olds. Journal of Clinical Child Psychology, 22, 355–364. Kaufman, J. C., Chen, T. -H., & Kaufman, A. S. (1995). Ethnic group, education, and gender differences on six Horn abilities for adolescents and adults. Journal of Psychoeducational Assessment, 12, 49–65. Kaufman, A. S., & Horn, J. L. (1996). Age changes on tests of fluid and crystallized ability for women and men on the Kaufman Adolescent and Adult Intelligence Test (KAIT) at ages 17 to 94 years. Archives of Clinical Neuropsychology, 11, 97–121. Kaufman, A. S., & Kaufman, N. L. (1993). Manual: Kaufman Adolescent and Adult Intelligence Test. Circle Pines, MN: American Guidance Service. Kaufman, A. S., Kaufman, J. C., & McLean, J. E. (1995). Factor structure of the Kaufman Adolescent and Adult Intelligence Test (KAIT) for whites, African Americans, and Hispanics. Educational and Psychological Measurement, 55, 365–376. Kaufman, A. S., McLean, J. E., & Kaufman, J. C. (1995). The fluid and crystallized abilities of white, black, and Hispanic adolescents and adults, both with and without an education covariate. Journal of Clinical Psychology, 51, 636–647. Kaufman, J. C., McLean, J. E., Kaufman, A. S., & Kaufman, N. L. (1994). White-black and white-Hispanic differences on fluid and crystallized abilities by age across the 11- to 94-year range. Psychological Reports, 75, 1279–1288. Kaufman, A. S., McLean, J. E., & Lincoln, A. (1996). The relationship of the Myers-Briggs Type Indicator to IQ level and fluid-crystallized discrepancy on the Kaufman Adolescent and Adult Intelligence Test. Assessment, 3, 225–239. Lassiter, K. S., Matthews, D., Bell, N. L., & Maher, C. M. (2002). Comparison of the General Ability Measure for adults and the Kaufman Adolescent and Adult Intelligence Test with college students. Psychology in the Schools, 39(5), 497–506. Luria, A. R. (1966). Human brain and psychological processes. New York: Harper & Row. Luria, A. R. (1980). The working brain: An introduction to neuropsychology. New York: Basic Books. Morgan, A. W., Sullivan, S. A., Darden, C., & Gregg, N. (1997). Measuring the intelligence of college students with learning disabilities: A comparison of results obtained on the WAIS-R and the KAIT. Journal of Learning Disabilities, 30, 560–565. Piaget, J. (1972). Intellectual evolution from adolescence to adulthood. Human Development, 15, 1–12. Thorndike, R. M., Hagen, E. P., & Sattler, J. M. (1986). Guide for administering and scoring the Stanford-Binet Intelligence Scale (4th ed.). Chicago: Riverside. Vo, D. H., Weisenberger, J. L., Becker, R., & Jacob-Timm, S. (1999). Concurrent validity of the KAIT for students in grade 6 and 8. Journal of Psychoeducational Assessment, 17, 152–162. Wechsler, D. (1974). Manual for Wechsler Intelligence Scale for Children-Revised (WISC-R). San Antonio, TX: The Psychological Corporation. Wechsler, D. (1981). Wechsler Adult Intelligence Scale-Revised. San Antonio, TX: The Psychological Corporation.

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Wechsler, D. (1991). Manual for the Wechsler Intelligence Scale for Children-third edition. San Antonio, TX: The Psychological Corporation. Woodrich, D. L., & Kush, J. C. (1998). Kaufman Adolescent and Adult Intelligence Test (KAIT): Concurrent validity of fluid ability for preadolescents and adolescents with central nervous system disorders and scholastic concerns. Journal of Psychoeducational Assessment, 16, 215–225.

Kaufman Assessment Battery for Children S HAHAL R OZENBLATT Advanced Psychological Assessment, P.C. Smithtown, NY, USA

Synonyms KABC-II

K Description Like its predecessor, the K-ABC, the KABC-II is designed to assess the intellectual functioning of children, pre-school through adolescent, with a focus on determining areas of processing strengths and weaknesses. While retaining some of the theoretical roots and subtests of the original measure, the KABC-II represents a major revision. First, the age for which the measure is applicable was increased at the upper limit and now ranges from 3 years to 18 years, 11 months (The K-ABC was originally normed for children ranging in age from 2 years, 6 months to 12 years, 6 months). Although the KABC-II maintains the theoretical ties to the Lurian process approach (Luria, 1970), the second edition offers the user the option to make use of the Cattell–Horn–Carroll hierarchical model of intelligence (CHC; Carroll, 1997). The components of the KABC-II have also been updated and/or changed (Bain & Gray, 2008). The test consists of 18 subtests, but the type and number of subtests that are administered depend on the approach taken (i.e., Luria vs. CHC) and on the child’s age. When using the Lurian approach, five subtests are administered for age 3 years; and 9 subtests, for ages 4 through 6 years and 7 through 18 years. When the CHC model is used, two additional subtests are administered, contributing to a scale associated with crystallized intelligence. The names of the subtests stay the same regardless of the theoretical model used; however, the scale names differ.

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CHC Scales For the CHC model, the scales are Short-Term Memory (Gsm; taking in and holding information, then using it within a few seconds), Visual Processing (Gv; perceiving, storing, & manipulating visual patterns), Long-Term Storage & Retrieval (Glr; storing & efficiently retrieving newly learned information), Fluid Reasoning (Gf; the ability to solve novel problems using reasoning), Crystallized Ability (Gc; knowledge-based ability that is based on education and acculturation), and a global score – Fluid-Crystallized Index (FCI) (Kaufman, Kaufman, Kaufman-Singer & Kaufman, 2005; Kaufman, Lichtenberger, Fletcher-Janzen & Kaufman, 2005).

Luria Scales With the exception of Gc, the CHC-based scales have parallel factor groupings in the Lurian model. Gsm is labelled Sequential Processing; Gv is labelled Simultaneous Processing; Glr is labelled Learning, and Gf is labelled Planning. Gc is referred to as the Knowledge Scale when Luria’s model is used, but it does not contribute to the global score, the Mental Processing Index (MPI) (Bain and Gray, 2008). One of the strengths of the KABC-II is the fact that it was developed, in part, to be used with children for whom cultural and/or linguistic issues may impact test results. For this purpose, a nonverbal scale, the Nonverbal Index (NVI), was developed (Baron, 2004). The KABC-II subtests and the scales to which they belong are as follows: Gsm/Sequential Processing: Word Order, Number Recall, & Hand Movements; Gv/Simultaneous Processing: Rover, Triangles, Conceptual Thinking, Face Recognition, Gestalt Closure & Block Counting; Gf/Planning: Pattern Reasoning & Story Completion; Glr/Learning: Atlantis, Atlantis Delayed, Rebus & Rebus Delayed; Gc/Knowledge: Riddles, Expressive Vocabulary & Verbal Knowledge. The KABC-II has the advantage of the addition of discontinued rules (e.g., four scores of 0 on five consecutive items), rather than stopping points based on chronological age, as was the case with the K-ABC. As is common to other intelligence scales, the KABC-II scales have a mean of 100 and standard deviation of 15, while the subtests have a mean of 10 and standard deviation of 3. In addition, scores can be reported in percentiles,

age equivalents, or as descriptive categories (e.g., Above Average, Average, and Below Average). Administration time ranges from 25 min for younger children to 75 min for older children.

Historical Background The K-ABC was originally developed by Alan and Nadine Kaufman in 1983 (Kaufman & Kaufman, 1983), in part, as a reaction to the Binet and Wechsler scales, which the authors believed tended to ignore developments in clinical psychology, neuropsychology, intelligence, and learning (Kaufman, Lichtenberger, et al., 2005). According to Kaufman, Lichtenberger, et al. (2005), the K-ABC was rooted in Sperry’s cerebral specialization approach and Luria’s successive-simultaneous processing dichotomy. Another major purpose of the K-ABC was to make it a more culturally fair test, such that the large differences between Caucasians and test-takers of other racial/cultural/ethnic backgrounds were reduced. It was normed on 2,000 children ranging in age from 2 years, 6 months to 12 years, 6 months. The sample was based on census data to include an equal number of males and females in each of the age groups and was stratified based on race/ethnicity, four levels of parent education, and population distribution (e.g., city, suburban, and rural) (Baron, 2004). Criticisms of the K-ABC (Kamphaus, 2003) have included insufficient floor and ceiling on various subtests for evaluating children at the lower and upper ends of the intelligence continuum, validity of the ability–achievement dichotomy of the test, and questions about whether the Mental Processing scale measures what it is purported to as opposed to other factors (e.g., semantic memory or nonverbal reasoning). The KABC-II was developed with these criticisms in mind.

Psychometric Data Bain and Gray (2008) reviewed research on the psychometric properties of the KABC-II, the results of which are summarized here. Standardization was based on 3,025 examinees ranging in age from 3 through 18 years, who were randomly selected from a larger pool based on 2001 U.S. census data. Samples were stratified based on sex, ethnic group, parental education, geographic region, and special education or gifted placement.

Kaufman Assessment Battery for Children

Reliability Internal consistency reliabilities using split-half analysis for the core subtests ranged from 0.60 to 0.95 and from 0.57 to 0.92 for the supplementary subtests across the age-groups. Reliability coefficients for the factor indices ranged from 0.81 to 0.95, while the MPI and FCI produced coefficients that ranged from 0.94 to 0.97 across age groups, with the exception of the 3-year-old group in which a coefficient of 0.90 was obtained. The NVI produced a lower range of coefficients (i.e., 0.85 to 0.95), which was lowest for the 3-year-olds. The standard error of the measure (SEM) for the FCI ranged from 2.60 to 3.69 with the largest number attributed to the 3-year-old group. The SEM for the MPI ranged from 2.82 to 3.61 across age groups, with the exception of the 3-year-olds wherein the SEM was 4.69. SEMs for the NVI were larger across age groups, ranging from 3.34 to 5.95. Test–retest reliability over approximately a 4-week period was presented for 205 children by Kaufman and Kaufman (in Bain & Gray, 2008). The 3- to 5-year-old group had the lowest subtest reliability coefficients, ranging from 0.50 for Hand Movements to 0.86 for Expressive Vocabulary. Coefficients for the 7- to 12-year-old group ranged from 0.53 for Block Counting to 0.88 for Expressive Vocabulary and for the 13- to 18-year-old group from 0.60 for Hand Movement to 0.92 for Gestalt Closure. Reliability coefficients for the MPI and FCI ranged from 0.74 to 0.95 across the three age groups and from 0.72 to 0.87 for the NVI. Kaufman and Kaufman also provided data on practice effects with the largest gains found for Learning/Glr scale (11.5 points overall) and 7 to 10 points across age-groups for the Simultaneous/Gv and Planning/Gf scales. Three to 4 point gains were noted for the Knowledge/Gc scale and what was referred to as a negligible amount for the Sequential/Gsm scale. Sex differences were notable at the younger age ranges (3–6 years) on the KABC-II, with girls performing significantly better on the global scales, Sequential/Gsm, Simultaneous/Gv, Learning/Glr, and Planning/Gf scales, with differences ranging from 2.5 to 3.9. At the older age level (7–18 years), boys performed significantly better (mean difference = 4.1) on the Simultaneous/Gv Scale. The MPI and FCI scores were not significantly different at the older age ranges. Assessment of the relationship between KABC-II performance and level of parental education indicated that the strongest relationship occurred at the preschool age range, accounting for 10.3% of the variance for the Sequential/Gsm and 22.5% for the FCI. The variance

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associated with parental education was lower for the older age group (7–18 years), with 5.1% for the Simultaneous/Gv scale to 17.6% for the Knowledge/Gc scale. Variance accounted for by race/ethnicity was largest for the Knowledge/Gc scale, ranging from 6% to 7%. Adjusted FCI means (based on parental education) for the 7- to 18-year-old-group ranged from 94.5 for the African American group to 103.9 for the Asian American group. For the adjusted MPI, means ranged from 95.2 for the African American group to 104.6 for the Asian American group. Comparisons by ethnic group were not calculated for younger children because of small sample sizes.

Validity The KABC-II and K-ABC were compared using a sample of 74 children who were 3–5 years old and 48 children who were 8–12 years old. Mean differences of about 7 points were found for the younger children and 6 points for the older children, with higher scores on the K-ABC. This finding is consistent with the Flynn effect (Flynn, 1987), which shows that mean performance on an IQ measure increases over time. When a test is renormed, the IQ is reset to 100, resulting in higher outcomes on the older version when compared to the new version. Correlations between the KABC-II and the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV; Wechsler, 2003) were 0.88, 0.89, and 0.79 for the WISC-IV Full Scale IQ (FSIQ) and the KABC-II MPI, FCI, and NVI, respectively. Comparisons with the Wechsler Preschool & Primary Scale of Intelligence, Third Edition (WPPSI-III; Wechsler, 2002) were 0.73, 0.81, and 0.81 between the FSIQ and MPI, FCI, and NVI. Adjusted mean correlations with the Kaufman Adolescent and Adult Intelligence Test (KAIT; Kaufman & Kaufman, 1993) were 0.85 and 0.91 for the MPI and FCI, respectively. The KABC-II was also compared with measures of academic functioning. The Peabody Individual Achievement Test, Revised (PIAT-R; Markwardt, 1998) Total Test Score, and KABC-II FCI had a correlation of 0.67 for the younger age group. A correlation of 0.87 was obtained between the FCI and Wechsler Individual Achievement Test, Second Edition (WIAT-II; Wechsler, 2001) for the older group.

Clinical Uses The clinical validity of the KABC-II has been assessed through multiple studies, summarized by Bain and Gray

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(2008), showing the measure’s ability to discriminate clinical from nonclinical samples. Children with specific learning disabilities in reading, math, and writing produced mean scores on the MPI and FCI that were consistently 16 points or lower than the nonclinical group. Two groups of children diagnosed with mild mental retardation and with autistic disorder had results that were 30 points or lower than the reference group, while children with attention-deficit/hyperactivity disorder had scores that were 8–10 points lower on the MCI and FCI than the reference group. Children with hearing loss obtained mean differences 11 to 16 points lower on the MPI and FCI and 9 points on the NVI. Gifted students had scores that were approximately 15 points higher than the reference sample on the MPI and FCI.

Wechsler, D. (2001). Wechsler individual achievement test – second edition. San Antonio, TX: The Psychological Corporation. Wechsler, D. (2002). Wechsler preschool & primary scale of intelligence – third edition. San Antonio, TX: The Psychological Corporation. Wechsler, D. (2003). Wechsler intelligence scale for children– fourth edition. San Antonio, TX: The Psychological Corporation.

Cross References

K-BIT

▶ Crystallized Intelligence ▶ Kaufman Adolescent & Adult Intelligence Test

Description

References and Readings Bain, S. K., & Gray, R. (2008). Test reviews: Kaufman, A. S., & Kaufman, N. L. (2004). Kaufman Assessment Battery for children (2nd edn). Journal of Psychoeducational Assessment, 26, 92–101. Baron, I. S. (2004). Neuropsychological evaluation of the child. New York: Oxford University Press. Carroll, J. B. (1997). The three-stratum theory of cognitive abilities. In D. P. Flannigan, & P. L. Harrison (Eds.), Contemporary intellectual assessment (pp. 69–76). New York: The Guilford Press. Flynn, J. R. (1987). Massive IQ gains in 14 nations: What IQ tests really measure. Psychological Bulletin, 101, 171–191. Kamphaus, R. W. (2003). Clinical assessment practice with the Kaufman Assessment Battery for Children. In C. R. W. Reynolds (Ed.), Handbook of psychological and educational assessment of children (pp. 204–216). New York: The Guilford Press. Kaufman, A. S., & Kaufman, N. L. (1983). Kaufman assessment battery for children: interpretive manual. Circle Pines, MN: American Guidance Serve, Inc. Kaufman, A. S., & Kaufman, N. L. (1993). Kaufman adolescent & adult intelligence test. Circle Pines, MN: American Guidance Serve, Inc. Kaufman, A. S., Lichtenberger, E. O., Fletcher-Janzen, E., & Kaufman, N. L. (2005). Essentials of KABC-II assessment. Hoboken, NJ: Wiley. Kaufman, J. C., Kaufman, A. S., Kaufman-Singer, J., & Kaufman, N. L. (2005). Kaufman Assessment Battery for Children – Second Edition and the Kaufman Adolescent & Adult Intelligence Test. In D. P. Flannigan & P. L. Harrison (Eds.), Contemporary intellectual assessment (pp. 344–370). New York: The Guilford Press. Luria, A. R. (1970). The functional organization of the brain. Scientific American, 222, 66–78. Markwardt, F. C. Jr. (1998). Peabody individual achievement test, revised. Circle Pines, MN: American Guidance Serve, Inc.

Kaufman Brief Intelligence Test N OELLE E. C ARLOZZI Kessler Foundation Research Center West Orange, NJ, USA

Synonyms

The Kaufman Brief Intelligence Test-second edition (K-BIT-2; Kaufman & Kaufman, 2004) is a brief intelligence test designed as a screening measure for verbal and nonverbal abilities for individuals 4–90 years of age. The K-BIT-2 contains three subtests: Verbal Knowledge, Matrices, and Riddles. The Verbal Knowledge subtest consists of items where the examiner says a word or asks a question and the participant responds by pointing to the picture that best answers the question; the Matrices subtest requires the participant to analyze a series or pictures/ patterns and point to the response that corresponds with that picture/pattern; and for the Riddles subtest, the examiner says a verbal riddle and the participant responds by pointing to a picture or saying a word that answers the riddle. The Verbal Knowledge and Riddles subtests comprise the Verbal Standard Score, while Matrices makes up the Nonverbal Standard Score. Administration time is approximately 15–30 min for ages 20–90. The K-BIT-2 utilized a standardization sample of 2,120 individuals (aged 4–90) stratified according to 2001 Current Population Survey; it was stratified according to gender, geographic region, socioeconomic status, race or ethnicity, and education. The two subtests provide raw scores that can be converted into standard scores, percentile ranks, normal curve equivalents, and stanines (M = 100, SD = 15), as well as an IQ composite score. Although the Verbal Knowledge and Riddles subtest are presented in English, the Matrices subtest can be administered in either Spanish or English.

Kaufman Brief Intelligence Test

Historical Background The K-BIT-2 is a revision of the K-BIT, and is a relatively new test that was developed as a brief screening measure for intellectual impairment in children, screening of job candidates, and brief intellectual assessment(Kaufman & Kaufman, 2004). The K-BIT-2 was designed to build on the strengths of the K-BIT, and to address its primary weakness, a reliance on verbal ability.

Psychometric Data While the original version of the K-BIT has a large body of literature to support its reliability and validity, psychometric support for the K-BIT-2 is less available. More specifically, the average reliability coefficients for the K-BIT IQ Composite score was 0.93 across all age ranges. Utilizing the Spearman-Brown formula, the K-BIT demonstrated split-half reliability values of 0.97 for Vocabulary and 0.94 for Matrices for adults ages 20–90 (Kaufman & Kaufman, 1990), and the K-BIT-2 demonstrated split-half reliability values of 0.90 for the Verbal Score, and 0.91 for the Nonverbal Score for ages 19–90 (Kaufman & Kaufman, 2004). Using the Guilford’s Formula, the K-BIT has demonstrated good internal consistency (0.97 for adults aged 20–90; Kaufman & Kaufman, 1990). Test-retest reliability coefficients, with a mean interval between tests of 21 days, was 0.97 for Vocabulary, 0.86 for Matrices, and 0.95 for the K-BIT IQ composite for participants aged 20–54, and 0.95 for Vocabulary, 0.92 for Matrices, and 0.95 for the K-BIT IQ composite for participants aged 55–89 (Kaufman & Kaufman, 1990). For the K-BIT-2, test-retest reliability coefficients, with a mean interval between tests of 28 days, was 0.88 for Verbal, 0.76 for Nonverbal, and 0.88 for the IQ composite for participants aged 4–12, 0.93 for Verbal, 0.80 for Nonverbal, and 0.89 for the IQ composite for participants aged 13–21, 0.89 for Verbal, 0.89 for Nonverbal, and 0.92 for the IQ composite for participants aged 22–59, and 0.92 for Verbal, 0.85 for Nonverbal, and 0.92 for the IQ composite for participants aged 60–89 (Kaufman & Kaufman, 2004). Standard error of measurement of the K-BIT was also good (2.7 for the Vocabulary subtest, 3.8 for the Matrices subtest, and 2.7 for the K-BIT IQ composite for participants aged 20–90; Kaufman & Kaufman, 1990). Further, the K-BIT has also demonstrated moderate intercorrelations for the Vocabulary and Matrices subtests (age 20–90 r = 0.72; Kaufman & Kaufman, 1990). Although K-BIT scores tend to increase

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with education (Hays, Reas, & Shaw, 2002), there is no consistent evidence for gender differences on the K-BIT (Webber & McGillivray, 1998). Further, the K-BIT has demonstrated good construct validity and external validity (construct and concurrent validity; Kaufman & Kaufman, 1990). In order to demonstrate construct validity on the Vocabulary subtest, both K-BIT raw scores (Kaufman & Kaufman, 1990) and K-BIT-2 raw scores (Kaufman and Kaufman, 2004) increased steadily throughout early adulthood, peaking during middle age, and then gradually declining over the rest of the lifespan; this pattern of growth is consistent with theories of crystallized intelligence (Horn, 1985; Wang & Kaufman, 1993). Kaufman and Kaufman (1990) were also able to demonstrate construct validity for the Matrices subtest with raw scores peaking in late adolescence or early adulthood and undergoing a steady decline throughout the rest of adulthood – also consistent with theories of fluid intelligence (Horn, 1985; Wang & Kaufman, 1993). The K-BIT has also demonstrated concurrent validity: K-BIT scores are significantly correlated with the K-BIT-2 (Kaufman & Kaufman, 2004), Kaufman Assessment Battery for Children (K-ABC; Kaufman & Kaufman, 1990), Matrix Analogies Test-Short Form (Hayes, 1999), Peabody Picture Vocabulary Test-Revised (Childers, Durham, & Wilson, 1994), Peabody Picture Vocabulary Test-third edition (Powell, Plamondon, & Retzlaff, 2002), Stanford-Binet Test Composite score (Prewett & McCaffery, 1993), Wechsler Adult Intelligence Scale-Revised (Kaufman & Kaufman, 1990; Naugle, Chelune, & Tucker, 1993), Wechsler Intelligence Scale for Children-third edition (Canivez, 1995, 1996; Canivez, Neitzel, & Martin, 2005; Chin, Ledesma, Cirino, Sevcik, Morris, Fritjers, et al., 2001; Grados & Russo-Garcia, 1999; Prewett, 1995), Wechsler Abbreviated Scale of Intelligence (Hays et al., 2002), Woodcock-Johnson Reading Comprehension subtest (Klinge & Dorsey, 1993), Wide Range Achievement Test-Revised (Bowers & Pantle, 1998), Wide Range Achievement Test-third edition (Powell et al., 2002), Shipley Institute of Living Scale (Bowers & Pantle, 1998) and Vineland Adaptive Behavior Scales (Hayes & Farnill, 2003). The K-BIT2 has also demonstrated significant concurrent validity with the Wechsler Abbreviated Scale of Intelligence (WASI; Kaufman & Kaufman, 2004). Finally, there is evidence for the discriminant validity of the K-BIT, which was demonstrated by a lack of a significant relationships between the Adjustment Scales for Children and Adolescents (measuring child psychopathology) and the K-BIT (Canivez et al., 2005).

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Clinical Uses There is evidence to suggest that the K-BIT is not particularly sensitive to the effects of traumatic brain injury (Donders, 1995; Donovick, Burright, Burg, Gronendyke, Klimczak, Mathews, et al., 1996), although it does appear to differentiate neurosurgical patients (in acute recovery phases) and psychiatric patients from controls (Donovick et al., 1996). In summary, the K-BIT appears to be a good intellectual screening instrument when a more comprehensive assessment is not warranted (Canivez, 1995; Naugle et al., 1993), but should not be used exclusively for placement and diagnostic purposes (Chin et al., 2001).

Cross References ▶ Intelligence ▶ Intelligence Quotient ▶ Performance IQ ▶ Shipley Institute of Living Scale ▶ Verbal IQ ▶ Wechsler Abbreviated Scale of Intelligence

References and Readings Bowers, T. L., & Pantle, M. L. (1998). Shipley Institute of Living Scale and the Kaufman Brief Intelligence Test as screening instruments for intelligence. Assessment, 5, 187–195. Canivez, G. L. (1995). Validity of the Kaufman Brief Intelligence Test: Comparisons with the Wechsler Intelligence Scale for Children-third edition. Canivez, G. L. (1996). Validity and diagnostic efficiency of the Kaufman Brief Intelligence Test in reevaluating students with Learning Disability. Journal of Psychoeducational Assessment, 14, 4–19. Canivez, G. L., Neitzel, R., & Martin, B. E. (2005). Construct validity of the Kaufman Brief Intelligence Test, Wechsler Intelligence Scale for Children-third edition, and adjustment scales for children and adolescents. Journal of Psychoeducational Assessment, 23, 15–34. Childers, J. S., Durham, T., & Wilson, S. (1994). Relation of performance on the Kaufman Brief Intelligence Test with the Peabody Picture Vocabulary Test-Revised among preschool children. Perceptual and Motor Skills, 79, 1195–1199. Chin, C. E., Ledesma, H. M. L., Cirino, P. T., Sevcik, R. A., Morris, R. D., Fritjers, J. C., et al. (2001). Relation between Kaufman Brief Intelligence Test and WISC-III scores of children with RD. Journal of Learning Disabilities, 34(1), 2–8. Donders, J. (1995). Validity of the Kaufman Brief Intelligence Test (K-BIT) in children with traumatic brain injury. Assessment, 2, 219–224. Donovick, P. J., Burright, R. G., Burg, J. S., Gronendyke, S. J., Klimczak, N., Mathews, A., et al. (1996). The K-BIT: A screen for IQ in six diverse populations. Journal of Clinical Psychology in Medical Settings, 3, 131–139.

Grados, J. J., & Russo-Garcia, K. A. (1999). Comparison of the Kaufman Brief Intelligence Test and the Wechsler Intelligence Scale for Children-third edition in economically disadvantaged African American youth. Journal of Clinical Psychology, 55(9), 1063–1071. Hayes, S. C. (1999). Comparison of the Kaufman Brief Intelligence Test and the Matrix Analogies Test-short form in an adolescent forensic population. Psychological Assessment, 11(1), 108–110. Hayes, S. C., & Farnill, D. (2003). Correlations with the Vineland Adaptive Behavior Scales with Kaufman Brief Intelligence Test in a forensic sample. Psychological Reports, 92, 573–580. Hays, J. R., Reas, D. L., & Shaw, B. (2002). Concurrent validity of the Wechsler Abbreviated Scale of Intelligence and the Kaufman Brief Intelligence Test among psychiatric inpatients. Psychological Reports, 90, 355–359. Homack, S. R., & Reynolds, C. R. (2007). Kaufman Brief Intelligence Testsecond edition (K-BIT-2). In A. S. Kaufman, & N. L. Kaufman (Eds.), Essentials of assessment with Brief Intelligence Tests (pp. 19–51). Hoboken, NJ: Wiley. Horn, J. L. (1985). Remodeling old models of intelligence. In B. B. Wolman (Ed.), Handbook of intelligence. New York: Wiley. Kaufman, A. S., & Kaufman, N. L. (1990). Kaufman Brief Intelligence Test. Circle Pines, MN: American Guidance Service. Kaufman, A. S., & Kaufman, N. L. (2004). Kaufman Brief Intelligence Test (2nd ed.). Circle Pines, MN: American Guidance Service. Klinge, V., & Dorsey, J. (1993). Correlates of the Woodcock-Johnson Reading Comprehension and Kaufman Brief Intelligence Test in a forensic psychiatric population. Journal of Clinical Psychology, 49(4), 593–598. Naugle, R. I., Chelune, G. J., & Tucker, G. D. (1993). Validity of the Kaufman Brief Intelligence Test. Psychological Assessment, 5(2), 182–186. Powell, S., Plamondon, R., & Retzlaff, P. (2002). Screening cognitive abilities in adults with developmental disabilities: Correlations of the K-BIT, PPVT-3, WRAT-3, and CVLT. Journal of Developmental and Physical Disabilities, 14(3), 239–246. Prewett, P. N. (1995). A comparison of two screening tests (the Matrix Analogies test-Short Form and the Kaufman Brief Intelligence Test) with the WISC-III. Psychological Assessment, 7(1), 69–72. Prewett, P. N., & McCaffery, L. K. (1993). A comparison of the Kaufman Brief Intelligence Test (K-BIT), the Stanford-Binet Intelligence Scale (4th ed.), a 2-subtest short form, and the Kaufman test of Educational Achievement (K-TEA). Psychology in the Schools, 30, 299–304. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of neuropsychological tests: Administration, norms, and commentary (3rd ed.). New York: Oxford University Press. Wang, J., & Kaufman, A. S. (1993). Changes in fluid and crystallized intelligence across the 20- to 90-year age range on the K-BIT. Journal of Psychoeducational Assessment, 11, 29–37. Webber, L. S., & McGillivray, J. A. (1998). An Australian validation of the Kaufman Brief Intelligence Test (K-BIT) with adolescents with an intellectual disability. The Australian Psychologist, 33, 234–237.

K-BIT ▶ Kaufman Brief Intelligence Test

Kindling

KBS ▶ Klu¨ver–Bucy Syndrome

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Cross References ▶ Developmental milestones ▶ Pediatric TBI


KELS ▶ Kohlman Evaluation of Living Skills

Duval, J., Braun, C. M. J., Montour-Prouix, I., Daigneault, S., Roulea, I., & Begin, J. (2008). Brain lesions and IQ: Recovery versus decline depends on age of onset. Journal of Chile Neurology, 23, 663–668. Webb, C., Rose, F. D., Johnson, D. A., & Attree, E. A. (1996). Age and recovery from brain injury: Clinical opinions and experimental evidence. Brain Injury, 10, 303–310.

Kennard Principle B RADLEY A XELROD, C HRISTIAN S CHUTTE John D. Dingell VA Medical Center Detroit, Michigan, USA

Definition The recently disproven assumption is that children recover more rapidly than adults suffering from the same type of brain lesion.

Current Knowledge Initially proposed by Margaret Kennard in 1936 when studying primates, it was found that motor impairment from unilateral lesions to the motor cortex was less severe in infants than in adults. This theory was generalized to humans in claiming that children would sustain less impairment and would recover more rapidly than adults if both sustained brain injury. The initially widely accepted principle lost credibility, as it was discovered that children with diffuse impairment did not recover more rapidly than their adult counterparts. Similarly, very young children did not recover as quickly as elderly adults did. In fact, younger children have greater difficulty with recovery of functions and have more developmental delays than older children. Prognosis for recovery is associated with the existing cognitive skills as a foundation. The abilities that have not yet been achieved will be delayed in acquisition following neurological injury. The theory continues to be supported by clinicians, despite significant research, which has demonstrated that age is unrelated to prediction of recovery from brain injury.

Kindling C HRISTINE J. W EBER-M IHAILA Northeast Regional Epilepsy Group New York, NY, USA

Definition Originally identified accidentally in 1967 by Graham Goddard, Ph.D., the animal model in which repeated electrical or chemical stimulation generates seizures of increasing behavioral involvement and duration. Behaviorally, the development of the seizure begins with a limited number of neural circuits involved, but additional neural circuits are increasingly engaged until the seizure advances to convulsions. The increasing duration of the seizures indicates that the brain’s ability to resist seizure activity becomes weakened, and the threshold for the incitement of future seizures is reduced. Changes to the brain through kindling are considered enduring and can potentially lead to spontaneous seizures.

Cross References ▶ Epilepsy ▶ Plasticity ▶ Seizure

References and Readings Abel, M. S., & McCandless, D. W. (1992). The kindling model of epilepsy. In R. N. Adams, G. B. Baker, J. M. Baker, A. N. Bateson,

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D. P. J. Boisvert, A. A. Boulton, et al. Neuromethods: Animal models of neurological disease (pp. 153–155). Totowa, NJ: Humana Press. Bertram, E. (2007). The relevance of kindling for human epilepsy. Epilepsia, 48(2), 65–74. Goddard, G. V. (1967). Development of epileptic seizures through brain stimulation at low intensity. Nature, 214, 1020–1021.

Kinesthesia K ERRY D ONNELLY University at Buffalo/SUNY Buffalo, NY, USA

Synonyms Dynamic proprioception

Definition Kinesthesia is the perception of the range, extent, direction, force, and momentum of movement. Also known as dynamic proprioception, this information is supplied primarily by specialized visceroceptors in the muscles, tendons, and joints, mediated by the posterior columns and lemniscal system, and cortically processed by the parietal lobes.

Kinsbourne, Marcel (1931– ) K ATHLEEN O’T OOLE Children’s Healthcare of Atlanta Atlanta, GA, USA


Although he has made significant contributions to the understanding of hemispheric specialization, aging, memory, learning disabilities, and consciousness, Dr. Kinsbourne is perhaps best known for his groundbreaking work in the domain of attention. Beginning in 1970, he published experiments on which he based his well known and still widely cited attentional model of hemispheric asymmetries. This model constituted a radical deviation from the then-prevalent static anatomical ‘‘switchboard’’ model (centers and connections) of the cerebrum that purported to explain neuropsychological deficits as ‘‘disconnection syndromes.’’ He noted that there is little evidence in neuroanatomy for hierarchical one-way information flow from way station to way station along designated channels in the cerebrum. His alternative, physiologically more realistic, model acknowledged the brain as a highly reciprocally interconnected neural network, characterized by a constantly shifting pattern of locally higher and lower activation levels, a constantly variable cortical activation manifold. Structural damage was no longer seen as the only cause of neuropsychological deficits, as he added pathologies

Cross References ▶ Proprioception

References and Readings Guyton, A. C., & Hall, J. E. (2000). Somatic sensations. Textbook of medical physiology (pp. 540–551). Philadelphia: W.B. Saunders.

Kinetra® ▶ Deep Brain Stimulator (Parkinsons)

Kinsbourne, Marcel (1931– ). Figure 1

Kinsbourne, Marcel (1931– )

of activation level to the explanatory repertoire in neuropsychology (a concept now thoroughly validated by neuroimaging). He recognized that neurologically intact people shift their attention from one side to the other by a changing balance of activation between reciprocally inhibitory lateralized opponent processors. The right hemisphere harbors the processor for shifting attention leftward, and vice versa. He could now explain the symptomatology of unilateral neglect of space and person as the result of unilateral brain damage depressing the activity one of these processors. The lesion would cause imbalance of activation levels between right and left hemisphere opponent processors, biasing attention to the ipsilesional side. Maneuvers that temporarily correct this imbalance eliminate the attentional bias and abolish the neglect. This model for unilateral neglect has stood the test of time and remains the standard view. His model of shifting balance of hemispheric activation also enabled him to demonstrate that in intact individuals, loading one hemisphere with a task that induces a mental set for which it is specialized (e.g., verbal or spatial) results in observable gaze and attention shifts in the opposite direction. This was an early demonstration of embodied cognition, and it offered a new explanation for perceptual asymmetries. The hemisphere organization of the brain could be inferred, in part, by observing the differential embodiment of different categories of thought processes. In 1978, he published his Functional Cerebral Distance Principle, which holds that concurrent cerebral processes that are compatible function optimally when their neural substrates are closely interconnected, whereas concurrent orthogonal processes are least liable to mutual interference (cross talk) when they are only indirectly interconnected. In 1971, Dr. Kinsbourne developed the first laterality test for motor processes. By having subjects concurrently perform a motor task (e.g., finger tapping) and a cognitive task (e.g., oral naming) that engages the specialization of either the same or the opposite hemisphere, he could show that when the same hemisphere was in control of both tasks, the performance of one or both was compromised. One could thereby infer which hemisphere harbors the cognitive process being evaluated. By use of intracarotid sodium amytal temporarily to anesthetize one and then the other hemisphere in the conscious aphasic patient, Dr. Kinsbourne also

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discovered that many aphasic patients owe their language recovery to cross-hemisphere plasticity, the assumption of language by the intact right hemisphere. With Frank Wood, he demonstrated that the amnesic syndrome found in Korsakoff psychosis involves a deficit in episodic remembering, and with Robert Hicks, he showed that the amnesic syndrome compromises the estimation of time passing as well as of autobiographical episodes. In a series of articles beginning in 1975, he marshaled evidence from his own research and that of others (especially Merrill Hiscock), that the rudiments of lateralization are present at a very early age. He argued that laterality is invariant, counter to the then-current view that lateralization emerges progressively during the childhood years. In 1974, Dr. Kinsbourne and his colleagues undertook an intensive study of attention-deficit/hyperactivity disorder (ADHD) and its response to stimulant medication. He found that ADHD involves a diminished dopaminergic substrate for incentive motivation. With Dr. James Swanson, he designed a controlled behavioral laboratory methodology for acquiring stimulant dose response and time response curves for patients with ADHD. This methodology had been adapted for commercial drug development. He and his colleagues demonstrated experimentally that many split-brain phenomena could be explained by the interruption of the transmission of activation (rather than information) across the corpus callosum. Also, he developed the ‘‘somatic twist’’ model for the evolutionary origin of decussation during the invertebrate–vertebrate transition. Decussation is the anatomical arrangement by which sensorimotor areas on one side of the brain connect with sense receptors and motor neurons on the other side of the body. Such contralateral crossing is only found in the vertebrate phylum. In 1985, he was the first to demonstrate that in the course of aging, lateralized brain use tends to expand to involve the brain bilaterally. In 1988, he developed his integrative cortical field model of consciousness, which he further elaborated in publications extending to 2006. With Professor Daniel Dennett, he coauthored an influential ‘‘target article’’ entitled ‘‘Time and the Observer’’ (1992), which demonstrated that when the brain encodes the duration of a stimulus, it does not need to replay that duration. The discussion advocated a decentralized view of the organization of conscious processes in the brain, and critiqued the

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view that the human brain contains a specialized ‘‘conscious awareness module.’’ Dr. Kinsbourne formulated a novel theory of cognitive and emotional hemisphere lateralization, attributing approach tendencies (positive emotions) to the left hemisphere and withdrawal tendencies (negative emotions) to the right hemisphere. This construct has been validated with respect to positive and negative emotions by an extensive research program directed by Dr. Richard Davidson. In studies with Drs. Peter Bick and Michael Green, he also demonstrated that schizophrenic auditory hallucinations derive from the subvocal inner speech of the patient himself/herself. Dr. Kinsbourne studied overarousal as a basis for autistic behavior, and a hyperglutamatergic brain state as its possible neural basis. He has offered evidence that the characteristic stereotypic movement routines of autistic children are compensatory, and serve to lower the child’s excessive arousal levels. He described a previously unrecognized subtype of ADHD, which he called ‘‘overfocusing,’’ and which appears to be a high level disorder on the autistic spectrum, which can easily be mistaken for ADHD. In 1996, Drs. Kinsbourne and Vadim Deglin reported findings from patients recovering from electroshock, administered daily to alternating hemispheres, for the treatment of drug-resistant depression. They discovered that the patients used contrasting strategies to solve syllogisms, depending on which cerebral hemisphere remained active while the other hemisphere was recovering. The same patient would offer decontextualized solutions when using the left hemisphere and contextualized solutions when using the right hemisphere. This finding supports the controversial concept of ‘‘hemisphericity.’’ With Dr. James Root, Dr. Kinsbourne reported a novel laterality paradigm, which involves central stimulus presentation and lateralized manual response. It probes the laterality of response preparation, depending on the nature of the decision. He also developed the view that imitation in infants is embodied perception, and that human dyadic interaction is speciesspecific intrinsically motivated interpersonal entrainment. Entrainment is seen as essential to the development of language and of unified dyadic and group processes. Entrainment of multiple individuals is conducive to sharing the same unified goal or point of view.

Education and Training Marcel Kinsbourne won a state scholarship to study Medicine at Oxford University, where he graduated in 1955 with the B.M., B.Ch. (the Oxford title for the M.D.). He was admitted to the Membership of the Royal College of Physicians in 1957. In 1963, he was awarded the D.M. (Oxon), a higher doctorate in the Oxford University Medical Faculty, for his research in neuropsychology. In 1964, after 7 years of postgraduate training, divided between the National Hospital, Queen Square and the Hospital for Sick Children, Great Ormond Street, both in London, New York University at Bellevue Hospital (with a Fulbright scholarship) and Oxford University Medical School, he qualified as a pediatric neurologist.

Major Appointments

Dr. Kinsbourne was appointed University Lecturer in Experimental Psychology at Oxford in 1964, and subsequently Fellow of New College, Oxford. In 1967, he relocated to Duke University Medical Center, Durham, NC, as Chief of the Division of Child Neurology, with a joint appointment in the Psychology Department. He continued his academic activities in parallel both in Neurology and in Cognitive Neuroscience. He moved to Canada in 1974 as Professor of Pediatrics (Neurology) at the University of Toronto with a joint appointment in Psychology. In 1980, he was appointed Director of the Division of Behavioral Neurology at the Eunice Kennedy Shriver Center for Developmental Disabilities, Waltham, MA with a joint appointment to Harvard Medical School, further pursuing his research in ADHD. He assumed professorship in Psychology at the New School, New York, in 1995, a position he holds as of this writing.


In 1949, Dr. Kinsbourne was awarded a state scholarship at Oxford University. The following year, he received the Henderson Scholarship in Anatomy, Christ Church, Oxford University. In 1952, he received the University Entrance Scholarship, Guy’s Hospital, London, England. This was followed by the Beaney Prize in Pathology at the same hospital. For the 1958–1959 years, he was granted a Fulbright traveling scholarship. In 1961, he received the Queen

Klu¨ver–Bucy Syndrome

Square Prize in Neurology. In 1975, he was honored with the James Arthur Lectureship on Evolution of the Brain: The American Museum of Natural History, New York. In 1990, he was a visiting resident scholar, Rockefeller Foundation, Bellagio Study Center. In 2006, the International Neuropsychological Society commemorated his contributions to the science and profession of neuropsychology with a symposium in his honor. His talent and dedication for teaching was acknowledged in 2008 by the New School’s University Award for Excellence in Teaching.

Short Biography Dr. Marcel Kinsbourne was born into a Viennese Jewish family in 1931. His father and two of his father’s brothers were attorneys, and a maternal uncle and great-uncle were physicians. After the Nazi takeover of Austria in 1938, against worldwide closed doors, his mother managed to enlist the assistance of a relative in England to enable the family to escape, with a few days to spare before World War II began. The family assumed British citizenship and lived in London throughout the war. He has seven children and nine grandchildren. He lives with his wife, Caroline, and his three youngest daughters in Winchester, MA.

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Kinsbourne, M. (2003). The corpus callosum equilibrates the cerebral hemispheres. In E. Zaidel & M. Iacoboni (Eds.), The Parallel Brain: The Cognitive Neuroscience of the Corpus Callosum (pp. 271–278). New York: Academic Press. Kinsbourne, M. (2006). From unilateral neglect to the brain basis of consciousness. Cortex, 42, 869–874. Kinsbourne, M., & Hicks, R. E. (1978). Mapping cerebral functional space: competition and collaboration in human performance. In M. Kinsbourne (Ed.), The Asymmetrical Function of the Brain (pp. 267–273). New York: Cambridge University Press. Kinsbourne, M., & Warrington, E. K. (1962). A disorder of simultaneous form perception. Brain, 85, 461–468. Kinsbourne, M. (1977). Hemi-neglect and hemisphere rivalry. In E. A. Weinstein & R. P. Friedland (Eds.), Hemi-inattention and hemisphere specialization: Advances in Neurology. (Vol. 18., pp. 41–49). New York: Raven Press. Kinsbourne, M. (Ed.). (1978). Asymmetrical function of the brain. New York: Cambridge University Press. Kinsbourne, M. (1988). Integrated field theory of consciousness. In A. J. Marcel & E. Bisiach (Eds.), Consciousness in contemporary science. Oxford: Clarendon. Kinsbourne, M. (1993). Development of attention and metacognition. In I. Rapin & S. Segalowitz (Eds.), Handbook of neuropsychology (Vol. 7., pp. 261–278). Amsterdam: Elsevier Biomedical. Kinsbourne, M. (2008). Development of cerebral lateralization in children. In C. R. Reynolds & E. Fletcher-Jansen (Eds.), Handbook of clinical child neuropsychology (3rd ed.). New York: Springer. Swanson, J. M., & Kinsbourne, M. (1976). Stimulant related statedependent learning in hyperactive children. Science, 192, 1354–1356.

Klonopin (Clonazepam) Cross References ▶ Attention ▶ Consciousness ▶ Handedness ▶ Hemispheric Specialization ▶ Lateral Asymmetry ▶ Learning Disabilities ▶ Neglect and Hemi-Attention ▶ Normal Aging

References and Readings Kinsbourne, M. (1970). The cerebral basis of lateral asymmetries in attention. Acta Psychologica, 33, 193–201. Kinsbourne, M. (1972). Eye and head turning indicate cerebral lateralization. Science, 176, 539–541. Kinsbourne, M. (1989). A model of adaptive behavior related to cerebral participation in emotional control. In G. Gainotti & C. Caltagirone (Eds.), Emotions and the Dual Brain (pp. 248–260). New York: Springer.

▶ Clonazepam

Klu¨ver–Bucy Syndrome K ELLY DAVIS G ARRETT 1, FARZIN I RANI 2, DAVID J. L IBON 3, R OD S WENSON 4, D ENENE M. WAMBACH 3 1 University of Utah Salt Lake City, UT, USA 2 Hospital of the University of Pennsylvania Philadelphia, PA, USA 3 Drexel University, College of Medicine Philadelphia, PA, USA 4 University of North Dakota Medical School Fargo, ND, USA

Synonyms KBS; Temporal lobe syndrome

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Short Description or Definition Klu¨ver–Bucy syndrome (KBS) is a disorder classically associated with bilateral extirpation of the temporal lobe, including most of the hippocampus and amygdala, resulting in six core symptoms: (1) visual agnosia; (2) hyperorality; (3) hypermetamorphosis; (4) alterations in emotional behavior; (5) changes in sexual behavior; and (6) changes in dietary habits. The initial work of Klu¨ver and Bucy (1937, 1938, 1939) was important in furthering understanding of the function of the temporal lobes and associated structures, Papez’s neuroanatomic circuit of emotion (Papez, 1937), as well as neurobehavioral phenomena including amnesia and agnosia. Though comparatively scarce, case descriptions of KBS are uniformly rich.

Categorization Both partial and complete KBS involving animals and humans have been reported. Human KBS syndrome has been described in children, adolescents, and adults. Following the suggestion of Lilly, Cummings, Benson, and Frankel (1983) and Poeck (1985) partial KBS is sometimes diagnosed when at least three of the six core KBS symptoms are present.

Epidemiology Elements of KBS are most often associated with certain Frontotemporal Lobar Degeneration (FTLD) phenotypes, i.e., a social compartment/dysexecutive disorder (Libon et al., 2007; Rosen et al., 2005). However, symptoms of KBS have been reported with a wide number of medical/ neurological illnesses including herpes simplex encephalitis, epilepsy, Huntington’s chorea, Alzheimer’s disease, brain injury, and a host of metabolic conditions.

The Klu¨ver–Bucy Syndrome: History, Description, and Significance The KBS and the extensive study of patient HM constitute two of the most important neurobehavioral discoveries of the twentieth century. Tangentially, both series of experiments have much in common. First, the initial description of both phenomena was the result of neurosurgical intervention. Second, the results or core presentation resulting from the neurosurgical intervention was serendipitous.

Indeed according to Bucy ‘‘the syndrome of bilateral destruction of the temporal lobes came by chance without prior planning’’ (Bucy, 1985). Third, both phenomena continue to raise important questions about brain–behavior relationships in general, and the functions of the temporal lobes in particular. Heinrick Klu¨ver (1897–1979) was born in Germany. After World War I he studied Gestalt psychology in Hamburg and Bonn. In 1923 he attended Stanford University, earning a Ph.D. in physiological psychology after 1 year (Nahm, 1997). After Stanford, Klu¨ver went to the University of Minnesota. However, Klu¨ver had no patience for the ‘‘pencil-and-paper psychologists who were so enamored of psychometric testing’’ (Bucy, 1985). Nonetheless, at the University of Minnesota Klu¨ver met a man whom he referred to ‘‘as the greatest of all neuropsychologists’’ – Karl Spencer Lashley (Bucy, 1985). Ultimately, Klu¨ver followed Lashley to the University of Chicago where he was to remain for the rest of his career. In 1963, Klu¨ver was honored with the Samuel W. Hamilton Award by the American Psychopathological Association. Klu¨ver (1965) used this occasion to deliver an address of considerable scope and breadth about the nature of the temporal lobe syndrome discovered by Bucy and himself. Early in his career Klu¨ver was very interested in eidetic imagery and the use of mescaline to study hallucinatorylike visual phenomena. Like Freud did with cocaine, Klu¨ver engaged in some self-experimentation with mescaline (Nahm, 1997). In this regard, Bucy lightheartedly referred to his longtime friend as ‘‘one of the first hippies’’ (Bucy, 1985). It was Klu¨ver’s desire to assess the hallucinatory properties of mescaline that led him to use this drug with monkeys. Klu¨ver noted that administering mescaline to monkeys produced chewing and lip-smacking behavior(s) as well as seizures. Klu¨ver’s interest in the effects of mescaline on the temporal lobes was stimulated, in part, by the hypothesis that the oral behavior seen in mescaline-treated monkeys was caused by ‘‘uncinated fits’’ (Nahm, 1997). Klu¨ver was aware of the work of John Hughlings Jackson (Jackson & Colman, 1888), who described similar behavior in an epileptic patient found to have a temporal lobe tumor (Nahm, 1997), and hypothesized that the behavior could be caused by any insult to the temporal lobes. Upon arriving at the University of Chicago, Klu¨ver was introduced to the ablation technique by his previous mentor, Karl Lashley. However, it was at the University of Chicago that Klu¨ver began a productive relationship with neurosurgeon Paul C. Bucy (1904–1992). Early observations of the temporal lobe syndrome in monkeys (1937)


were based on a single case study. Subsequent work (1939) included the description of a series of 16 animals. In general, surgical intervention involved bilateral removal of the temporal lobes (Broadman’s area 20, 21, and most of 22), including the uncus, most of the amygdala and hippocampus, and the tail of the caudate. There was some variation in the species of monkey, sex, surgical techniques, including the timing of the surgeries (left first, right first, or bilateral), amount and specialization of brain tissue (temporal lobe first, or second and third convolutions of temporal lobe, or connections between temporal lobe and occipital or prefrontal areas). These variations, and subsequent surgeries, yielded the following conclusions – KBS occurs with bilateral extirpation of the temporal lobes not by merely disconnecting the temporal lobes from the frontal or occipital lobes, and the six core symptoms of the temporal lobe syndrome (described below) remain stable over time (Klu¨ver, 1965; Klu¨ver & Bucy, 1938, 1939). 1. Psychic Blindness (visual agnosia) – Though primary visual and manual sensory abilities were unharmed, the ability to recognize or attach meaning to objects, by sight or with manual examination, was uniformly impaired. Animate and inanimate objects were approached and re-approached without hesitation or premorbidly displayed emotional response (e.g., avoidance, excitement). For example, ‘‘the monkey seems just as eager to examine the tongue of a hissing snake, the mouth of a cat, a wire cage, or a wagon as a piece of food’’ (1939). People and their clothing were approached and examined like other objects. Any object’s ‘‘acquired meaning’’ that was obtained through pre-surgical training was lost. Objects were examined repeatedly, as if there has been no recollection of the previous examination, representing, according to Klu¨ver and Bucy, a failure to ‘‘generalize when responding to visual stimuli’’ (1939, p. 995). All edible objects were consumed, but the animals did not always choose to examine edible objects first. Unlike the animal’s premorbid behavior, objects were not necessarily approached and examined in an orderly fashion. 2. Oral Tendencies – With rare exception, the monkeys examined all objects by mouth, i.e., biting gently, chewing, licking, touching with lips. This oral exploration was made without using hands. In situations where oral manipulation would be facilitated by first picking up objects with the hands and bringing them to the mouth (such as an object lying just outside the cage door), the monkeys were observed to first attempt to ‘‘reach’’ the objects with their tongues.

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3. Hypermetamorphosis – Using Heinrich Wilhelm Neumann’s description (1859, Danek, 2007) of hypermetamorphosis, KBS was further described: ‘‘as if the animal were acting under the influence of some compulsory or irresistible impulse’’ (1939, p. 987). Objects were quickly examined, especially if presented alone, and for comparable amounts of time. 4. Emotional Changes – Klu¨ver and Bucy described their subjects as having an ‘‘absence of emotional reactions’’ (1939, p. 991). Notably, anger and fear were not expressed, including typical avoidance behaviors. There was a characteristic aggressiveness that was also lacking, as evidenced by no attempts to destroy, break, or carry objects. Occasionally, ‘‘emotional behavior’’ did occur, but it was attributed to interference with the monkeys’ attempts to make contact with an object. Facial expressions were generally lost for several months, though their reappearance was among the rare post-surgery changes observed over time. 5. Changes in Sexual Behavior – Marked changes in sexual behavior made the monkeys ‘‘appear hypersexed’’ (1939, p. 992). Specifically, increased frequency of arousal, and frequent manipulation of genitalia with mouth or hands were observed. Masturbatory, as well as heterosexual and homosexual, behavior emerged after surgery. 6. Changes in Dietary Habits – After surgery animals would readily eat meat, which is uncustomary. Animals often ate excessively and gained weight. In general, sensory and motor abilities were unchanged. Specifically, there were no changes in visual fields, lateral dominance, auditory function, and tactile and motor abilities. However, animals did not vocalize, even when attacked or when the colony was shouting at feeding times. Detailed autopsy and anatomical material were made available in subsequent publications (Bucy & Klu¨ver, 1940, 1955). The initial reports of Klu¨ver and Bucy stimulated much activity with researchers essentially interested in two broad questions: whether a full or partial temporal lobe syndrome could be produced with more restricted or isolated lesions; and, more importantly, how the temporal lobe syndrome could be used to understand larger issues regarding brain– behavior relationships. With respect to the first question many researchers were able to replicate the behavioral abnormalities reported by Klu¨ver and Bucy (1938, 1939; Akert, Gruessen, Woolsey, & Meyer, 1961; Horel, Keating, & Misantone, 1975). However, some researchers noted greater alterations in social behavior when ablations and/or lesions were restricted to the amygdala (Hayman,

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Rexer, Pavol, Strite, & Meyers, 1998; Rosvold, Mirsky, & Pribram, 1954), and greater disturbances in cognitive operations when lesions were confined to specific regions within the temporal lobes (Mishkin, 1954; Mishkin & Pribram, 1954; Pribram & Bagshaw, 1953). Nonetheless, Klu¨ver (1965) was skeptical that the temporal lobe syndrome could be fractionated into single parts, likely reflecting his early training in Gestalt psychology. Regarding the nature of the KBS within the greater context of brain–behavior relationships, Klu¨ver (1965) speculated on the evolutionary significance of the rhinencephalon or limbic areas of the brain. Klu¨ver noted that rhinencephalon/limbic behaviors are largely internally generated. Moreover, the function of these behaviors (e.g., drives, instincts, emotion, and procreation) is geared to both self-perseveration and the propagation of the species. This is contrasted with visual/auditory behaviors which are externally based and rich in semantic representation. Klu¨ver believed that the temporal lobe syndrome could be viewed as an interaction between deficits in modulating internally generated, drive-based behavior (hyperorality, hypersexuality) with deficits in higher-order cognitive functions (visual agnosia). For Norman Geschwind, the KBS was a paradigmatic example of a disconnection syndrome, i.e., a disorder due to the ‘‘the effects of lesions of association pathways’’ (Geschwind, 1965). Geschwind noted that there are many pathways connecting limbic structures (including the hippocampus and amygdala) to visual association cortex in the lateral and medial posterior temporal lobe. Geschwind believed that many of the symptoms of the KBS could be explained by a disconnection between visual association pathways and limbic areas of the brain. Klu¨ver and Bucy (1939) considered a disconnection as a possible mechanism to produce temporal lobe syndrome, but reported that severing the temporal lobe from either the frontal lobes or the occipital lobes was not sufficient to elicit the syndrome. Olson, Plotzker, and Ezzyat (2007) noted intimate connections between the temporal pole (TP) (including the amygdala) and auditory, visual, and olfactory sensory areas. They also noted that in the macaque, the dorsolateral TP receives projections from auditory association cortex; the ventral TP receives projections from visual inferior temporal cortex; and the medial TP receives projections from prepiriform olfactory cortex and the insula. Insult to these pathways produced elements of a KBS, particularly aberrations in social behavior and maternal behavior in female monkeys.

Human Klu¨ver–Bucy Syndrome The first identification of a partial KBS in humans was made in 1955 in the context of a bilateral temporal lobectomy to treat seizures and troubling behavioral disturbance (Terzian & Ore, 1955). The patient did not exhibit hyperorality but showed all of the other symptoms associated with a KBS, including dulling of emotions. Encephalitis due to herpes in young children can result in human KBS. Behavioral changes include hyperorality, hypersexuality, and emotional indifference. Though the emotional disturbances for these children varied (e.g., irritability, excessive cheerfulness, placidity), all were reportedly indifferent to family and caregivers in the context of premorbidly secure attachments (Pradhan, Singh, & Pandey, 1998). These observations were striking in the case of Terzian and Ore (1955) who referred to the parents as ‘‘mother and father’’ but ‘‘manifested no particular affection for them’’ (p. 375). A complete human KBS was first described by Marlowe, Mancall, and Thomas (1975), likely due to viral herpes encephalitis. Their patient showed all of the features of KBS including severe Wernicke’s aphasia and profound memory impairment. Lilly and colleagues (1983) described a series of 12 patients with KBS, each with aphasia and amnesia. These human cases also afforded a more thorough exploration of higher-order cognitive disorders manifested in humans. New onset or ongoing seizure activity and new onset of homosexual behaviors are commonly described features of KBS due to idiopathic disease, injury, or surgical intervention (Lilly et al.). Traumatic injury of the temporal lobes can produce KBS as the result of either a single event (i.e., motor vehicle accident) and/or recurrent mild TBI without loss of consciousness (e.g., boxing; Yoneoka, Takeda, Inoue, Ibuchi, Kumagie Sugai, et al., 2004). Many TBI cases involve hematoma, with evacuation. Some cases improve with time, pharmacotherapy, and rehabilitation (Goscinski, Kwiatkowski, Polak, Orlowiejska, & Partyk, 1997; Lilly et al.; Slaughter, Bobo, & Childers, 1999), with infectious and traumatic etiologies affording the best prognosis.

Evaluation Most cases of KBS appear almost immediately after either insult to the brain or temporal lobe surgery. Symptoms are often extraordinarily striking. Pilleri (1967) described the hyperorality of one patient: ‘‘he would crawl around the yard on all fours and graze like an animal picking up


objects from the ground and dropping them again.’’ Researchers (e.g., Marlowe et al., 1975; Terzian & Ore, 1955) comment on the inaccessibility of their patients to formal neuropsychological testing. The patient of Conlon, Kertesz, and Mount (1988) was found to have an IQ in the normal range, but presented with striking anterograde amnesia. To the extent to which patients with KBS are amenable to testing, dysexecutive, amnesic, and languagerelated comprehension problems will likely dominate the neuropsychological picture. Yet, the richness in the behavior produced by these patients often trumps psychometric testing.

Treatment and Prognosis Slaughter et al. (1999) reported that the aggression, hyperorality, and hypersexual behaviors associated with a KBS due to traumatic temporal lobe injury resolved following treatment with selective serotonin re-uptake inhibitors (SSRIs). Similar results were found in the treatment of a KBS associated with Huntington’s chorea with moderate doses of haloperidol (Janati, 1985). For these cases, remission of troubling KBS behaviors afforded improved engagement in rehabilitation therapies. Thus, SSRIs, such as sertraline, (Menhekar & Duggal, 2005) may represent a promising option. Some success in treating KBS has been reported using carbamazepine (Hooshmand, Sepdham, & Vries, 1974; Stewart, 1985). Carbamazepine has also been associated with ‘‘rapid regression of KBS’’ in four cases of TBI with persistence of ‘‘psychological disorders’’ (Goscinski et al., 1997, p. 271). Nonetheless, there are no treatment/control studies reporting resolution of Klu¨ver–Bucy symptoms. Prognosis for most humans with KBS is usually poor. Some exceptions include encephalitis due to herpes seen in children. Pradhan et al. (1998) reported global improvement in seven patients after 1-year follow-up. Similarly, hypersexuality and bulimia remitted after 1 year in all three adult cases described by Hart, Kwentus, Frazier, and Hormel (1986). Further, they assert that ‘‘early aggressive antiviral therapy is not only life saving, but is important for limiting severity of chronic postencephalitic behavioral and cognitive disturbance’’ (p. 1377). Environmental structuring and other behavioral modification strategies have an important role in ameliorating KBS behaviors and maintaining safety. Resources for healthcare providers and caregivers are provided by the US National Institutes of Health (NINDS) and the National Organization for Rare Disorders (http://www. rarediseases.org).

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Cross References ▶ Frontotemporal Dementia ▶ Limbic System ▶ Papez Circuit ▶ Visual Agnosia

References and Readings Akert, K., Gruessen, R. A., Woolsey, C. N., & Meyer, D. R. (1961). Klu¨ver–Bucy syndrome in monkeys with neocortical ablations of temporal lobe. Brain, 84, 480–498. Bucy, P. (1985). Neurosurgical giants: Feet of clay and iron (pp. 350–353). New York: Elsevier. Bucy, P., & Klu¨ver, H. (1940). Anatomic changes secondary to temporal lobectomy. Archives of Neurology and Psychiatry, 44, 1142–1146. Bucy, P., & Klu¨ver, H. (1955). An anatomical investigation of the temporal lobe in the monkey. Journal of Comparative Neurology, 103, 151–251. Conlon, P., Kertesz, A., & Mount, J. (1988). Kluver–Bucy syndrome with severe amnesia secondary to herpes encephalitis. Canadian Journal of Psychiatry, 33, 754–756. Cummings, J. L., & Duchen, L. W. (1981). Kluver–Bucy syndrome in Pick disease: Clinical and pathologic correlations. Neurology, 31, 1415–1422. Danek, A. (2007). ‘‘Hypermetamorphosis’’ Heinrich Neumann’s (1814–1884) legacy: Eine Hinterlassenschaft des Breslauer Psychiaters Heinrich Neumann. Der Nervenarzt, 78, 342–348. Geschwind, N. (1965). Disconnection syndromes in animals and man – Part 1. Brain, 88, 237–294. Goscinski, I., Kwiatkowski, S., Polak, J., Orlowiejska, M., & Partyk, A. (1997). The Kluver–Bucy syndrome. Journal of Neurosurgical Sciences, 41, 269–272. Hart, R. P., Kwentus, J. A., Frazier, R. B., & Hormel, T. L. (1986). Natural history of Kluver–Bucy syndrome after treated herpes encephalitis. Southern Medical Journal, 79, 1376–1378. Hayman, L. A., Rexer, J. L., Pavol, M. A., Strite, D., & Meyers, C. A. (1998). Kluver–Bucy syndrome after bilateral selective damage of amygdala and its cortical connections. Clinical and Research Reports, 10, 354–358. Hooshmand, H., Sepdham, T., & Vries, J. K. (1974). Klu¨ver–Bucy syndrome successfully treated with carbamazepine. JAMA, 229, 1782. Horel, J. A., Keating, E. G., & Misantone, L. J. (1975). Partial Klu¨ver–Bucy syndrome produced by destroying temporal neocortex or amygdala. Brain Research, 94, 347–359. Jackson, J. H., & Colman, W. S. (1888). Case of epilepsy with tasting movements and dreamy state? – Very small patch of softening in the left uncinate gyrus. Brain, 21, 580–590. Janati, A. (1985). Kluver–Bucy syndrome in Huntington’s chorea. Journal of Nervous and Mental Disease, 173, 632–635. Klu¨ver, H. (1965). Neurobiology of normal and abnormal perception. In P. H. Hoch & J. Zubin (Eds.), Psychopathology of perception (pp. 1–40). New York: Grune & Stratton. Klu¨ver, H., & Bucy, P. (1937). ‘‘Psychic blindness’’ and other symptoms following bilateral temporal lobectomy in Rhesus monkeys. American Journal of Physiology, 119, 352–353. Klu¨ver, H., & Bucy, P. (1938). An analysis of certain effects of bilateral temporal lobectomy in the rhesus monkey, with special reference to ‘‘psychic blindness’’. The Journal of Psychology, 5, 33–54.

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Knowledge System

Klu¨ver, H., & Bucy, P. (1939). Preliminary analysis of functions of the temporal lobes in monkeys. Archives of Neurology and Psychiatry, 42(6), 979–1000. Libon, D. J., Xie, S., Moore, P., Farmer, J., Antani, S., McCawley, G., et al. (2007). Patterns of neuropsychological impairment associated with frontotemporal dementia: A factor analytic study. Neurology, 68, 368–375. Lilly, R., Cummings, J. L., Benson, D. F., & Frankel, M. (1983). The human Klu¨ver–Bucy syndrome. Neurology, 33, 1141–1411. Marlowe, W. B., Mancall, E. L., & Thomas, J. J. (1975). Complete Klu¨ver– Bucy syndrome in man. Cortex, 11, 53–59. Menhekar, D. N., & Duggal, H. S. (2005). Sertraline for Kluver–Bucy syndrome in an adolescent. Letter to the Editor/European Psychiatry, 20, 355–356. Mishkin, M. (1954). Visual discrimination performance following partial ablations of the temporal lobe II: Ventral surface vs. hippocampus. Journal of Comparative Physiological Psychology, 47, 187–193. Mishkin, M., & Pribram, P. H. (1954). Visual discrimination performance following partial ablations of the temporal lobe I: Ventral vs. lateral. Journal of Comparative Physiological Psychology, 47, 14–20. Nahm, F. K. D. (1997). Heinrick Klu¨ver and the temporal lobe syndrome. Journal of the History of Neuroscience, 6, 193–208. National Institute of Neurological Diseases and Stroke Klu¨ver–Bucy Syndrome Information Page. http://www.ninds.nih.gov/disorders/ kluver_bucy/kluver_bucy.htm. December 1, 2008. Olson, I. R., Plozker, A., & Ezzyat, Y. (2007). The enigmatic temporal pole: A review of findings on social and emotional processing. Brain, 130, 1718–1730. Papez, J. W. (1937). A proposed mechanism of human emotion. Archives of Neurology and Psychiatry, 38, 725–743. Pilleri, G. (1967). The Klu¨ver–Bucy syndrome in man. A clinicoanatomical contribution to the function of the medial temporal lobe structures. Psychiatria et Neurologia (Basel), 152, 65–103. Poeck, K. (1985). The Kluver–Bucy syndrome in man. Handbook of Clinical Neurology, 45, 257–263. Pradhan, S., Singh, M. N., & Pandey, N. (1998). Kluver Bucy syndrome in young children. Clinical Neurology and Neurosurgery, 100, 254–258. Pribram, P. H., & Bagshaw, M. H. (1953). Further analysis of the temporal lobe syndrome using fronto-temporal ablations. Journal Comparative Neurology, 99, 347–375. Rosen, H. J., Allison, S. C., Schauer, G. F., Gorno-Tempini, M. L., Weiner, M. W., & Miller, B. L. (2005). Neuroanatomical correlates of behavioural disorders in dementia. Brain, 128, 2612–2625. Rosvold, E., Mirsky, A., & Pribram, P. (1954). Influence of amydalectomy on social behaviour in monkeys. Journal Comparative Physiological Psychology, 47, 173–178. Slaughter, J., Bobo, W., & Childers, M. K. (1999). Selective serotonin reuptake inhibitor treatment of post-traumatic Klu¨ver–Bucy syndrome. Brain Injury, 13, 59–62. Stewart, J. T. (1985). Carbamazepine treatment of a patient with Klu¨ver– Bucy syndrome. Journal of Clinical Psychiatry, 46, 496–497. Terzian, H., & Ore, G. D. (1955). Syndrome of Klu¨ver and Bucy; reproduced in man by bilateral removal of the temporal lobes. Neurology, 5, 373–380. Yoneoka, Y., Takeda, N., Inoue, A., Ibuchi, Y., Kumagai, T., Sugai, T., et al. (2004). Human Kluver–Bucy syndrome following acute subdural haematoma. Acta Neurochirurgica, 146, 1267–1270.

Knowledge System ▶ Semantic Memory

Kohlman Evaluation of Living Skills K ELLI W ILLIAMS G ARY Virginia Commonwealth University Richmond, VA, USA

Synonyms KELS

Description The Kohlman Evaluation of Living Skills (KELS) is an interview and task performance test initially developed for adolescents and adults in short-term psychiatric settings. Later, the KELS was used with the geriatric population as well as persons with mental retardation, brain injury, and other cognitive impairment. It is designed to be a rapidly and easily administered standardized tool to evaluate both basic and instrumental activities of daily living. The KELS assesses 17 living skills grouped into five major categories of self-care, safety and health, money management, transportation and telephone, and work and leisure (Thomson, 1992). Time required for evaluation is 30–45 min. The first item of the KELS is based on observation and the other 16 are either performance- or interview-based. If clients are unable to perform a task, an interview can be substituted for some but not all items. Specific instructions and questions asked by the evaluator are to be stated as given in manual to maintain reliability and validity. The evaluator is allowed, however, to ask a structured set of additional probing questions. For some of the items, the client has to demonstrate specific tasks with equipment provided by the evaluator; however, most of the items are assessed through an interview to keep the administration and scoring brief. Scoring for self-care items is based on the clients’ physical appearance in street clothes (e.g. unkempt or

Kohs Blocks

dirty hair, torn clothes, unfastened clothing, unpleasant body order, dirty fingernails, visible dirt on body, dirty clothes, dental decay, and open untreated sores) and frequency of self-care activities (e.g. bathing, dressing, and grooming). The section on safety and health requires the awareness of dangerous household situations, identification of appropriate action for sickness, knowledge of emergency numbers, and location of medical facilities. Money management assesses knowledge of the use of money when purchasing, obtaining sources of income, budgeting for food, banking, and payment of bills. The section on transportation and telephone has items related to mobility within the community, basic knowledge of transit systems, and use of phone book and telephone. The section on work and leisure asks about plans for future employment and involvement in leisure activity. Most items are simply scored in a binary fashion; the categories of ‘‘independent’’ and ‘‘needs assistance’’ are assigned scores of 0 and 1, respectively. However, for the section on work and leisure ‘‘needs assistance’’ is counted as only 1/2 point. A total score of 6 or more indicates that the person needs assistance to live in the community while a lower score means the person should be able to live independently.

Historical Background In 1978, the KELS was developed for use on a locked inpatient unit at Harborview Medical Center in Seattle, Washington (McGourty, 1979). As validity studies were conducted, the KELS was adopted by occupational therapists and occupational therapy schools throughout the United States. The third edition was published by the American Occupational Therapy Association, Inc. in 1992. Currently, it is used worldwide in a variety of settings.

Psychometric Data An early study reported interrater reliability between 0.74 and 0.94 (Ilika, J. & Hoffman, N. G., 1981, unpublished document). While the study was not published, it provided the basis for slight procedural changes. Later studies reported high agreement between 0.84 and 1.0 (Brown, Moore, Hemman, & Yunek, 1996; McGourty, L. K., 1987, unpublished document). The KELS showed predictive validity when compared with other ADL assessments with correlations ranging from 0.70 and 0.895 (Zimnavoda, Weinblatt, & Katz, 2002).

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Clinical Uses The most important use of the KELS is to evaluate clients’ capacity to perform skills of daily living required for successful integration into the community. Since its development, the KELS has been applicable to a variety of settings and populations and its use has expanded in the acute care setting with geriatric patients, adolescents, and those with developmental disabilities. However, it has been difficult to confirm the true applicability of the KELS within these other settings and with other populations owing to the lack of published research (Moore, Palmer, Patterson, & Jeste, 2007). The examiner is encouraged to gather additional information about the client’s skills and abilities during the interview portion of the assessment by asking additional questions. Therefore, variation in judgments about partial answers could affect scoring. There is no formalized training and certification of the KELS, so education and periodic re-education of staff is left to the discretion of individual departments.

Cross References ▶ Activities of Daily Living ▶ Instrumental Activities of Daily Living ▶ Occupational Therapy

References and Readings Brown, C., Moore, W. P., Hemman, D., & Yunek, A. (1996). Influence of instrumental activities of daily living assessment method on judgement of independence. American Journal of Occupational Therapy, 50, 202–206. McGourty, L. K. (1979). Kohlman evaluation of living skills. Seattle, Washington, DC: KELS Research. Moore, D. J., Palmer, B. W., Patterson, T. L., & Jeste, D. V. (2007). A review of performance based measures of functional living skills. Journal of Psychiatry Research, 41, 97–118. Thomson, L. K. (1992). The Kohlman evaluation of living skills (3rd ed.). Rockville, MD: American Occupational Therapy Association. Zimnavoda, T., Weinblatt, N., & Katz, N. (2002). Validity of the Kohlman Evaluation of Living Skills (KELS) with Israeli individuals living in the community. Occupational Therapy International, 9, 312–325.

Kohs Blocks ▶ Block Design

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Korsakoff Amnestic Syndrome

Korsakoff Amnestic Syndrome ▶ Wernicke-Korsakoff Syndrome

Korsakoff (Korsakov) Psychosis ▶ Korsakoff ’s Syndrome ▶ Wernicke-Korsakoff Syndrome

Korsakoff (Korsakov), Sergei Sergeievich (1854–1900) B ENJAMIN M. H AMPSTEAD Emory University/Rehabilitation Medicine Atlanta, GA, USA

Major Appointments

Physician, Preobrazhenskii Mental Hospital, Moscow, Russia. Chair of Psychiatry, Preobrazhenskii Mental Hospital, Moscow, Russia. Superintendent, Psychiatric Clinic, Moscow University, Moscow, Russia.


1890 Founded the Moscow Society of Neuropathologists and Psychiatrists.


Korsakoff is best known for his work detailing the cognitive and physical effects of chronic alcoholism. His initial contribution to this area was his 1887 doctoral thesis, which found that many alcoholics demonstrated evidence of polyneuritis (e.g., muscular weakness and pain, staggering gait) as well as profound memory impairments and confabulation.

Although others (e.g., James Jackson in 1822; Robert Lawson in 1879) had previously described some of these same symptoms, Korsakoff realized the cognitive and physical symptoms were characteristic of a single disease. In fact, Korsakoff initially named the disorder psychosis polyneuritica in order to fully capture both the cognitive (psychosis) and physical (polyneuritica) aspects of the condition. In his subsequent work, Korsakoff hypothesized a causal role for a toxin and began referring to it as cerebropathia psychica toxemica. Over the next few years, however, other researchers began questioning the relationship between polyneuritis and amnesia and, at the 1897 Moscow Medical Congress, Friedrich Jolly first used the terms Korsakoff ’s psychosis and Korsakoff ’s syndrome to describe only the cognitive sequelae of the disease. Although Wernicke had also described some cognitive consequences of alcoholism, neither Korsakoff nor Wernicke realized the similarities between their respective discoveries. It was not until the early 1900s, when Karl Bonhoeffer documented that Korsakoff ’s syndrome was often preceded by Wernicke’s encephalopathy, that the term Wernicke–Korsakoff syndrome was used to describe these patients.

Short Biography Korsakoff was born on the Gus Estate in the Vladimir Province of Russia. Interestingly, there are discrepancies about both his birthday (January 22 or June 22) and the year of his birth as some authors claim he was born in 1853 (Finger, 1994), while others have cited 1854 (Beighton & Beighton, 1997). What is clear, however, is that Korsakoff was highly regarded for both his intellectual contributions and the compassion with which he cared for his patients. In fact, Korsakoff refused to restrain his mentally ill patients and even provided interventions that involved the entire family. He was equally sympathetic to his students and implemented financial aid programs that allowed them to focus solely on their studies. Professionally, Korsakoff authored a textbook of psychiatry and founded the Moscow Society of Neuropathologists and Psychiatrists. He organized the 12th International Medical Congress that was held in Moscow in 1897. At the time of his death, on May 1, 1900, Korsakoff had been developing a constitution for the Russian Association of Psychiatrists and Neurologists, which was officially founded after his death.

Korsakoff’s Syndrome

Cross References ▶ Karl Wernicke ▶ Korsakoff ’s Syndrome ▶ Wernicke–Korsakoff Syndrome

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confabulation. However, KP is often distinguished from dementia associated with alcoholism due to the preservation of major intellectual ability, which is clouded in the latter syndrome.

Categorization References and Readings Finger, S. (1994). Origins of neuroscience: A history of explorations into brain function (pp. 357–362). New York: Oxford University Press. Beighton, P., & Beighton, G. (1997). The person behind the syndrome. New York: Springer. Korsakoff, S. S. (1887). Disturbance of psychic function in alcoholic paralysis and its relation to the disturbance of the psychic sphere in multiple neuritis of non-alcoholic origin. Quoted by M. Victor, R. D. Adams, G. H. Collins (1971) The Wernicke–Korsakoff syndrome. Oxford: Blackwell. Korsakoff, S. S. (1889a). Psychic disorder in conjunction with peripheral neuritis. (trans: Victor, M., Yakovlev, P. I., 1955) Neurology, 5, 394–406. Korsakoff, S. S. (1889b). Etude medico-psychologique sur une forme des maladies de la me moire. Revue Philosophie, 20, 501–530.

Korsakoff’s Syndrome S EVERN B. C HURN , J ONATHAN C AMPBELL Virginia Commonwealth University Richmond, VA, USA

Synonyms Alcohol amnesic disorder; Alcoholic polyneuropathy; Alcoholic psychosis; Korsakoff (korsakov) psychosis; Wernicke’s encephalitis; Wernicke–Korsakoff encephalopathy

Short Description or Definition Korsakoff ’s Psychosis (KP) is an abnormal mental condition that is usually a sequela of chronic alcoholism. Generally considered a later stage of Wernicke’s Encephalopathy (WE), KP is associated with polyneuritis, and is characterized by an impaired ability to acquire new information and by a substantial, but irregular memory loss for which the patient often attempts to compensate through

Korsakoff ’s Psychosis is usually associated with the late, irreversible stage of Wernicke’s Encephalitis. WE is an acute neuropsychiatric condition resulting from an initially reversible dysfunction caused by a depletion of vitamin B1. It can be characterized by nystagmus, ophthalmoplegia, altered mental status and an unsteady gait, and other balance disturbances (Sechi & Serra, 2007). Typically, the syndrome is recognized by the ‘‘triad’’ of ataxia, confusion, and ophthamolplegia (McIntosh & Chick, 2006, 2004). However, the presentation with many comorbidities, especially head traumas, often results in a lower incidence of diagnoses of WE (see below). The dysfunction typically results from the imbalance between energy demands and inefficient energy utilization. This leads to an energy deficit, local acidosis, increased glutamate release, and, if not treated, neuronal cell death (Thomson & Marshall, 2006).

Epidemiology The prevalence of KP in industrialized countries varies from 0.0% to 2.8% of the population. However, this incidence may be grossly underestimated. Since there exists a dearth of tests or imaging that can identify WE or KP, the diagnosis remains clinical. Unfortunately, autopsy records estimate that up to 80% of KP cases may be misdiagnosed (Sechi & Serra, 2007). Therefore, suspected WE cases should be treated aggressively. In industrialized countries, almost 90% of cases are associated with excessive alcohol consumption (Thomson, 2000). However, gastric bypass surgeries, bilateral head trauma, and thiamine-deficient feeding solutions (especially in infants) have also caused similar imbalances in metabolism. Particularly in the case of alcoholism, if the patients are not treated and continue to abuse alcohol, the vast majority will develop Korsakoff’s Psychoses. Of these, a quarter will develop such severe mental deficiencies that they will require long-term care (Thomson & Marshall, 2006), a condition sometimes referred to as dementia associated with alcoholism.

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Natural History, Prognostic Factors, and Outcomes There exists a high correlation between decreased thiamine (B1) and the progression of KP, but excitotoxicity due to excessive release of excitatory amino acids may also play a role in progression to KP (Thomson & Marshall, 2006). Thiamine is utilized as a cofactor in multiple cellular processes, particularly those involved with energy production, glucose utilization, production of reducing enzymes, and gene transcription. The daily requirement for thiamine is approximately 1–2 mg/day. The daily requirements are dependent upon carbohydrate consumption, which is estimated to replenish the approximately 1 mg/day turnover of the vitamin. The body has a limited storage capacity of approximately 30 mg for thiamine. Thus, thiamine deficiencies will be readily evident within 3–4 weeks of lack of thiamine intake. However, diseases that increase metabolic stress (e.g., AIDS), impair thiamine intake (malnutrition, alcohol abuse), or thiamine metabolism (liver disease) will increase the dietary need for thiamine. The brain has a high metabolic demand and is especially sensitive to factors that disrupt energy production. In fact, the brain, while constituting 2% of the total body mass, requires over 20% of the body’s energy needs (Raichle & Gusnard, 2002). Surprisingly, this energy demand remains relatively constant, despite widely varying mental challenges and motor activity. To fuel this constant energy need, the brain primarily utilizes carbohydrates. Therefore, the brain is especially sensitive to thiamine deficits. Cellular utilization of thiamine involves absorption of the vitamin across multiple cellular membranes, and the phosphorylation of the vitamin into the useable form by cellular kinases. Thiamine is a charged entity; therefore, crossing cellular membranes typically occurs through an active process (Manzardo & Penick, 2006). For supply to the cells of the body, thiamine must cross at least four membrane structures: the brush border of the gut, the basolateral membrane to allow transport into the blood, the blood brain barrier, and the cellular membranes of neurons and glia. An additional step necessary for the production of the phosphorylated form of the vitamin may involve transport into cellular organelles such as the mitochondria, but this is less well understood. Once inside the cell, thiamine is phosphorylated to a diphosphate form of the vitamin by thiamine diphosphokinase. The phosphorylation of thiamine may help to drive the cellular absorption of thiamine; however, the exact mechanism has yet to be elucidated (Manzardo & Penick, 2006). The diphosphate

form is the active cofactor for glucose metabolism and other cellular functions. In addition, a triphosphate form has been identified that may modulate ion channels in neurons. Thiamine-diphosphate is an important cofactor for two rate-limiting enzymes in glucose metabolism – typically for the production of ATP. Of note, these enzymes are also important for neurotransmitter synthesis, myelin production (important for production of the myelin sheath to insulate axonal conduction), and protein synthesis (Manzardo & Penick, 2006). Thus, prolonged thiamine deficiencies would disrupt multiple cellular processes. In glucose metabolism, both pyruvate dehydrogenase (PDH) and a-ketoglutarate dehydrogenase (aKGDH) require thiamine-diphosphate as a cofactor. PDH catalyzes the conversion of pyruvate to acetyl coenzyme A (acetyl-CoA). Acetyl-CoA either enters the citric acid cycle, serves as a substrate for acetylcholine production, or serves as a substrate for myelin production. Once in the citric acid cycle, aKGDH regulates the conversion of a-ketoglutarate to succinyl-CoA. In addition to being an important step in the citric acid cycle, a-ketoglutarate is also a substrate for the production of the neurotransmitters glutamate, aspartate, and g-aminobutyric acid (GABA). Thiamine-diphosphate is also important in the regulation of the pentose-phosphate pathway. The pentose phosphate pathway is involved in the coordination between the citric acid cycle, the pentose phosphate shunt, as well as the maintenance of cellular reducing factors, nucleic acid synthesis, and neurotransmitter synthesis. Thiamine-diphosphate acts as a cofactor for transketolase, which catalyzes the conversion of glucose-6-phosphate into ribose-5-phosphate and reduced nicotinamide adenine dinucleotide (NADPH). Ribose5-phosphate is required for the synthesis of nucleic acids and other important cellular compounds. NADPH acts as a hydrogen source for the synthesis of fatty acids, amino acids, steroids, and other cellular molecules. It is also an essential component for the synthesis of cellular compounds to reduce reactive oxygen species, including glutathione. Of note, transketolase activity is especially sensitive to reduced levels of thiamine (Manzardo & Penick, 2006). In addition, there exists a population of people who express transketolase isoforms that exhibit a reduced binding affinity for thiamine. Under non-limiting conditions (such as those seen in a normal western diet), the reduced binding affinity would not be significant. However, in the presence of prolonged thiamine deficiency, the reduced affinity would result in reduced energy production and increased oxidative stress. The subpopulation of individuals with


increased sensitivity to thiamine deficiency has been proffered as a mechanism whereby only a subset of chronic alcohol abusers develop overt Korsakoff ’ psychosis (Manzardo & Penick, 2006). The overall effect of inhibition of these enzymes will be altered mitochondrial function. This will result in decreased energy production, which if severe will result in neuronal necrosis. Prolonged mitochondrial dysfunction may also trigger neuronal cell death by apoptotic mechanisms. In addition, altered carbohydrate metabolisms will set up a condition creating oxidative stress. This will also occur at a time when protective mechanisms (i.e., decreased radical scavenger systems) are depleted. All of these effects have been described in both animal models and culture systems.

Neuropsychology and Psychology of Korsakoff Psychosis In his studies, Sergeie Sergeievich Korsakoff (1853–1900), described a distinctive mental disorder that ‘‘appears at times in the form of sharply delineated irritable weakness of the mental sphere, at times in the form of confusion with characteristic mistakes in orientation for space, time, and situation and at times as an almost pure form of acute amnesia, where recent memory is most severely involved, while the remote memory is well preserved.’’ The loss of learning and memory could become very severe: ‘‘some have suffered so widespread memory loss that they literally forget everything immediately.’’ The label Korsakoff Pyschosis has been attributed to Friedrich Jolly and the most common associations are Korsakoff Psychosis for the mental disturbances that are associated with alcoholic polyneuropathy and Korsakoff Syndrome (KS) to describe the nonalcoholic expression of the disease. Historically, confabulatory behavior was the hallmark symptom of KS. However, this has come into question. Typically, there are five major symptoms of KS: (1) Dense and profound anterograde amnesia, despite relatively intact intellectual functioning, (2) variable retrograde amnesia, sometimes with severe memory loss, (3) temporaspatial disorientation, with or without confabulatory behavior, (4) disturbances of affective processing and lack of insight, and (5) apathy and lack of initiative. With regard to memory, working or primary memory will be variably intact. However, secondary memory, including episodic, semantic, and implicit, can be greatly affected. Episodic memory, referring to events that occurred in one’s past, is characteristically severely

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affected in KS while semantic memory, that is, the knowledge of facts, is variably affected. Implicit memory, response to priming or procedural memory can be relatively intact.

Evaluation Once the patient has been treated for the acute confessional state, a careful neurologic exam should be completed to elucidate the core mental deficits. While a MiniMental State Evaluation can help screen for global confusion, it is unable to elucidate memory function. Supplemental questioning regarding personal and general semantic memory function, episodic memory, and distracter episodic memory can help to confirm KS.

Treatment Recommended treatment protocols vary, but 500 mg of thiamine HCl given i.v. or intramuscular over 30 min, three times/day for 3 days is generally considered adequate intervention. If no improvement in mentation is observed, the course can be discontinued. However, if improvement is noted, the treatment is usually continued at a dose of 250 mg/day for an additional 3–5 days. Failure to identify and treat acute WE may be associated with a 20% mortality rate. In addition, even if treated early, approximately 20% of WE cases progress to KP. Prophylactic treatment of 250 mg/ day i.m. for 3–5 days is recommended for all people suspected of severe alcohol withdrawal, poor nutrition, and that present with signs of malnutrition. Thiamine treatment if mandatory if i.v. glucose is administered as this may precipitate a WE event. Thiamine treatment is generally well tolerated, but a flush may result from a rapid administration. That is why a diluted form, given over 30 min, is recommended.

Cross References ▶ Alcoholic Brain Syndrome ▶ Amnestic Disorder ▶ Amnestic Syndrome ▶ Confabulation ▶ Episodic Memory ▶ Mammillary Bodies ▶ Memory Impairment ▶ Posttraumatic Amnesia ▶ Traumatic Brain Injury ▶ Wernicke–Korsakoff Syndrome

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Kumho Tire v. Carmichael

References and Readings Manzardo, A. M., & Penick, E. C. (2006). A theoretical argument for inherited thiamine insensitivity as one possible biological cause of familial alcoholism. Alcoholism: Clinical and Experimental Research, 30(9), 1545–1550. McIntosh, C., & Chick, J. (2006). Managing neurological problems in heavy drinkers. Practitioner, 250(1685), 22, 25, 27–29 passim. McIntosh, C., & Chick, J. (2004). Alcohol and the nervous system. Journal of Neurology Neurosurgery & Psychiatry, 75(Suppl 3), iii16–21. Raichle, M. E., & Gusnard, D. A. (2002). Appraising the brain’s energy budget. Proceedings of the National Academy of Sciences USA, 99(16), 10237–10239. Sechi, G., & Serra, A. (2007). Wernicke’s encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet Neurology, 6(5), 442–455. Thomson, A. D., & Marshall, E. J. (2006). The natural history and pathophysiology of Wernicke’s Encephalopathy and Korsakoff ’s Psychosis. Alcohol and Alcoholism, 41(2), 151–158. Thomson, A. D. (2000). Mechanisms of vitamin deficiency in chronic alcohol misusers and the development of the Wernicke-Korsakoff syndrome. Alcohol and Alcoholism (Suppl), 35(1), 2–7. Thomson, A. D., & Marshall, E. J. (2006). The treatment of patients at risk of developing Wernicke’s encephalopathy in the community. Alcohol and Alcoholism, 41(2), 159–167.

Kumho Tire v. Carmichael R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition The issue in Kumho Tire v. Carmichael (1999) was whether the Daubert decision applies only to scientific ideas. On July 6, 1993, the right rear tire of a minivan driven by Patrick Carmichael blew out, which led to an accident resulting in the death of one passenger and severe injuries to others. The Carmichaels sued the tire maker claiming that the tire was defective. The Carmichaels had a tire expert testify that the tire was indeed defective and linked to the accident. However, the district court applied the Daubert Standards and said that the methods used by the expert witness were not reliable and thus ruled in favor of Kumho. The Carmichaels appealed to the Eleventh Circuit court, claiming that Duabert was limited to scientific expert testimony and thus not applicable to ‘‘skill’’ or ‘‘experiencebased observation.’’ The Eleventh Circuit court reversed the decision in favor of the Carmichaels, holding that a Daubert analysis was restricted to purely scientific testimony mentioned in FRE 702. Kumho Tires subsequently asked the US

Supreme Court to review whether Daubert was indeed limited to scientific evidence. The Court reasoned that the trial judge’s gatekeeper function extended to all forms of expert witnesses including technical and experiential testimony and determined that the Duabert standard should be applied.

Cross References ▶ Admissibility ▶ Daubert v. Merrell Dow ▶ Joiner v. General Electric (1997)

References and Readings Daubert v. Merrell Dow, 509 US. 579 (1993). Frye v. U.S., D.C. Cir., 293 F. 1013 (1923). Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: Principles of productive attorney-neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. Joiner v. General Electric, 522 U.S. 136 (1997). Kumho Tire v. Carmichael, 526 U.S. 137 (1999).

Kurtzke Expanded Disability Status Scale ▶ Expanded Disability Status Scale

Kuru B RUCE J. D IAMOND 1, A MY C. M OORS 2 A MANDA FAULHABER 1 1 William Paterson University Wayne, NJ, USA 2 Villanova University Villanova, PA, USA

Synonyms Human prion disease; Prion; Slow virus infection; Spongiform encephalitis

Kuru

Short Description or Definition Kuru is a chronic, progressive, and potentially fatal disorder of the nervous system caused by a virus, and like other prion diseases is transmissible to a range of mammalian species including humans. There is renewed interest in Kuru because it provides an example of an acquired human prion disease. Kuru reached epidemic proportions among the Fore people of the Okapa District of the Eastern Highlands Provence, Papua New Guinea, and it may provide an opportunity to model incubation periods, pathogenesis, and genetic susceptibility factors applicable to a variety of other prion diseases including variant Creutzfeldt–Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE) (Collinge et al., 2006). Prion diseases are neurodegenerative disorders that affect a range of mammalian species including humans with an incidence of approximately one person per million worldwide per annum. BSE could pose a threat to public health as a result of dietary exposure to infected tissues. Consequently, Kuru and other prion diseases have received heightened research and clinical attention (Collinge, 1997). In the Fore language, Kuru means to be afraid or to tremble. Symptoms include trembling and muscle weakness that increases steadily over time until the person can no longer swallow and eventually dies of starvation. Another associated symptom is memory loss, but it does not usually occur until the latter stages of the disease (Jansen, 2005).

Categorization Human prion diseases or transmissible spongiform encephalopathies have generally been classified into Creutzfeldt– Jakob disease (CJD), Gerstmann–Straüssler–Scheinker disease (GSS) and Kuru. CJD may present as a sporadic, genetic, or infectious illness (Prusiner, 1997).

Epidemiology The incidence of the Kuru has declined dramatically since endocannabalistic practices ended among the Fore people where ritualistic endocannibalism was part of the funeral ceremony. Transmission occurred in two ways: infected tissue was digested and it also came into contact with the skin of the victim’s maternal kin (women and young children) allowing transmission through conjunctivae, mucous membranes, and skin abrasions (Roper, Brown, Adams, & Victor, 2005).

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Natural History, Prognostic Factors, Symptoms Kuru was first identified in the medical literature in the 1950s and it was the first slow infection documented in human beings. Recent research has identified 11 patients with Kuru living in the South Fore, all of whom were born before the cessation of cannibalism. Findings suggest that incubation periods of infection with human prion disease can exceed 50 years. Among the Fore group, most patients who survived Kuru were heterozygous at polymorphic codon 129. This genotype is associated with extended incubation periods and resistance to prion disease (Collinge et al., 1996). The clinical course is characterized by a rapidly progressive cerebellar ataxia, abnormal extraoccular movements, weakness progressing to immobility, incontinence in the latter stages, and death within 3–6 months of onset. Symptoms can also include tremor, dysarthria, and emotional liability (i.e., laughing) (Chusid & McDonald, 1973).

Treatment Quinacrine and chlorpromazine, which pass the blood– brain barrier, have been identified as candidates for treatment of CJD and other prion diseases (Korth, May, Cohen & Prusiner, 2001). However, the use of quinacrine for the treatment of CJD has been characterized as questionable, at least when used as a monotherapy (Barrett et al., 2003). A novel generation of Heparan sulfate mimetics (HMs) show anti-prion potential and could with further development and refinement treat prion diseases (Adjou et al., 2003).

Cross References ▶ Creutzfeldt–Jakob Disease (vCJD) ▶ Prion Disease ▶ Spongiform Encephalopathies

References and Readings Adjou, K. T., Simoneau, S., Sales, N., Lamoury, F., Dormont, D., Papy-Garcia, D., et al. (2003). A novel generation of heparan sulfate mimetics for the treatment of prion disease. Journal of General Virology, 84, 2595–2603. Barrett, A., Tagliavini, F., Forloni, G., Bate, C., Salmona, M., Colombo, L., et al. (2003). Evaluation of quinacrine treatment for prion diseases. Journal of Virology, 77, 8462–8469.

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Chusid, J. G., & McDonald, J. J. (1973). Correlative neuroanatamy and functional neurology. San Francisco, CA: Lange Medical Publications. Collinge, J. (1997). Human prion diseases and bovine spongiform encephalopathy (BSE). Human Molecular Genetics, 6, 1699–1705. Collinge, J., Whitfield, J., McKintosh, E., Beck, J., Mead, S., Thomas, D. J., & Alpers, M. (2006). Kuru in the 21st centuryAn acquired human prion disease with very long incubation periods. The Lancet, 367, 2068–2074. Jansen, P. A. (2005). Kuru. The Medscape Journal Online, retrieved from http://www.emedicine.com/med/topic1248.htm

Korth, C., May, B. C. H., Cohen, F. E., & Prusiner, S. B. (2001). Acridine and phenothiazine derivatives as pharmacotheraputics for prion disease. Proceedings of the national Academy of Science PNAS, 98, 9836–9841. Prusiner, S. B. (1997). Prion biology and diseases: Sporadic, inherited, and infectious degenerative illnesses of humans and animals. In L. L. Heston (Ed.), Progress in Alzheimer’s disease and similar conditions (pp. 69–100). Washington, DC: American Psychiatric Association. Ropper, A. H., Brown, R. H., Adams, R. D., & Victor, M. (2005). Adams & Victor’s principles of neurology. New York: McGraw-Hill Medical.

L L Scale R ICHARD T EMPLE CORE Health Care Dripping Springs, TX, USA

▶ MMPI ▶ True Response Inconsistency Scale (TRIN, MMPI) ▶ Validity Scales (MMPI) ▶ Variable Response Inconsistency Scale (VRIN, MMPI)

References and Readings Synonyms Lie scale

Definition One of the original validity scales on the Minnesota Multiphasic Personality Inventory (MMPI) and its revisions, the L scale consists of 15 items designed to detect attempts to avoid responding honestly. The items reflect patterns of behavior that are socially desirable but only found in the most conscientious individuals. ‘‘True’’ is the non-elevating response for all items, making the scale also sensitive to systematic response bias. There is good face validity to these items; however, because of their transparency, the scale may not detect more sophisticated attempts to respond dishonestly. Elevations on this scale have been associated with denial and lack of psychological sophistication. In addition to its value in the validation of MMPI data, the clinical neuropsychologist may find value in the L scale in validating information obtained in the clinical interview. Readers are referred to the MMPI entry for a discussion of limitations of this self-report measure when used with neuropsychological populations (see also Gass, 2006 and Lezak, Howieson, & Loring, 2004).

Gass, C. (2006). Use of the MMPI-2 in neuropsychological evaluations. In J. Butcher (Ed.). MMPI-2: A practitioner’s guide (pp. 301–326). Washington, DC: American Psychological Association. Graham, J. R. (2005). MMPI-2: Assessing personality and psychopathology (pp. 22–25). New York: Oxford University Press. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.

L’e´tat De Mal E´pileptique ▶ Status Epilepticus

L’etat Lacunaire E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms Lacunar state

Cross References ▶ F Minus K Index ▶ F Scale ▶ Fake Bad Scale ▶ Faking Good, Bad ▶ K Scale

Definition In L’etat lacunaire, or lacunar state, the brain contains multiple small infarcts or lacunes, usually in the deep structures, especially the basal ganglia.


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La Belle Indifference

Current Knowledge Similar to lacunar strokes, the lacunar state is associated with hypertension and atherosclerosis, the latter being present especially in the small deep vessels in the brain. Many small infarcts can result in dementia, characterized by loss of recent memory, alterations in orientation to time and space, and occasionally paranoia. Lacunar state is a common cause of vascular dementia. There also can be headache, vertigo, and light-headedness. Sensory loss and motor deficits such as ataxia, hemiparesis, and dysarthria, may occur, presenting similarly to lacunar stroke syndromes. Although the diagnosis can be made on clinical grounds, CT scanning and magnetic resonance imaging usually clearly demonstrate the multiple small cerebral infarctions.

Cross References

symptom and is listed as an associated descriptive feature of conversion disorder (APA, 2000). Extant neurobiological models converge on the conceptualization of conversion symptoms as involuntary responses to threat reflecting errors in body state information processing and representation (Kozlowska, 2005). Theories suggesting frontal lobe hypoactivation as an explanation for apathy and indifference have been proposed (Spence et al., 2000) as well as theories suggesting a link to right hemispheric dysfunction (Stone et al., 2006), but existing research is inconclusive.

Cross References ▶ Anosodiaphoria ▶ Anosognosia ▶ Apathy

▶ Cerebrovascular Disease ▶ Lacunar Infarction

References and Readings References and Readings Debette, S., Bombois, S., Bruandet, A., Delbeuck, X., Lepoittevin, S., Delmaire, C., et al. (2007). Subcortical hyperintensities are associated with cognitive decline in patients with mild cognitive impairment. Stroke, 38, 2924–2930. Erkinjuntti, T. (2002). Subcortical vascular dementia. Cerebrovascular Diseases, 13, 58–60.

La Belle Indifference

American Psychiatric Association. (2000). Somatoform disorders. Conversion disorder. In Diagnostic and statistical manual of mental disorders, (4th ed., text revision) (DSM-IV-TR). Washington, DC: American Psychiatric Press, 492–498. Kozlowska, K. (2005). Healing the disembodied mind: Contemporary models of conversion disorder. Harvard Review of Psychiatry, 13, 1–13. Spence, S. A., Crimlisk, H. L., Cope, H., et al. (2000). Discrete neurophysiological correlates in prefrontal cortex during hysterical and feigned disorder of movement. Lancet, 355, 1243–1244. Stone, J., Smyth, R., Carson, A., Warlow C., & Sharpe, M. (2006). La belle indifference in conversion symptoms and hysteria. British Journal of Psychiatry, 188, 204–209.

N ATALIE C. B LEVINS Indiana University School of Medicine Indianapolis, IN, USA

LACI Synonyms

▶ Lacunar Infarction

Belle indifference

Definition La belle indiffe´rence is defined in DSM-IV-TR as a relative lack of concern about the nature or implications of the

Lack of Neural Tube Closure ▶ Anencephaly

Lacunar Stroke Syndrome (LACS)

Lacunar Infarction E LLIOT J. R OTH Northwestern University Chicago, IL, USA

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Mixed sensorimotor stroke causes hemiparesis and ipsilateral sensory loss. Lacunar strokes can be treated with thrombolysis if diagnosed sufficiently early. Alternatively, antiplatelet therapy or anticoagulation can be used. Outcome is generally good, with mortality less than 5%, and complete recovery of independent function in up to 80% at 1 year.

Synonyms LACI; Lacune; Lacunar stroke; Lacunar stroke syndrome (LACS)

Definition A lacunar infarction is a small (less than 15 mm) deep subcortical area of brain damage, usually resulting from occlusion of the tiny (200–800 m) deep penetrating lenticulostriate arteries that normally provide blood supply to the deeper subcortical brain tissues, such as the basal ganglia, internal capsule, thalamus, and brain stem.

Current Knowledge It is thought that prolonged exposure to hypertension, diabetes, smoking, and other factors induces the processes of lipohyalinosis (small vessel disease of the brain) and microatheroma (tiny accumulations and swelling of artery walls that are comprised of cell debris, lipids, calcium, and fibrous tissue) in the small blood vessels that feed the subcortical areas, ultimately causing their occlusion. It is estimated that lacunar infarcts account for 25% of all ischemic strokes, with an incidence of approximately 15 per 100,000 per year. They are more frequent in men and in African Americans, Hispanics, and Asians. There are five clinical stroke syndromes associated with lacunar infarctions, each with a distinct presentation. Pure motor hemiplegia is most common, causing about one-third to one-half of all lacunar strokes. It involves only complete or partial paralysis of the arm, leg, and/or face. Ataxic hemiparesis is associated with a combination of cerebellar and motor symptoms, including weakness and clumsiness, on the ipsilateral side of the body, affecting the leg more than the arm; it is also called hemiataxia – hemiparesis. Dysarthria-clumsy hand syndrome is associated with weakness of the hand and facial muscles. In pure sensory stroke, the patient has a transient or persistent contralateral numbness or dysesthetic pain.

Cross References ▶ Anticoagulation ▶ Antiplatelet Therapy ▶ Atherosclerosis ▶ Cerebrovascular Disease ▶ Ischemic Stroke ▶ Pure Motor Stroke ▶ Small Vessel Ischemic Disease ▶ Thrombolysis

References and Readings Baumgartner, R. W., Sidler, C., Mosso, M., & Georgiadis, D. (2003). Ischemic lacunar stroke in patients with and without potential mechanism other than small-artery disease. Stroke, 34, 653–659. Mok, V. C. T., Wong, A., Lam, W. W. M., Fan, Y. H., Tang, W. K., Kwok, T., et al. (2004). Cognitive impairment and functional outcome after stroke associated with small vessel disease. Journal of Neurology, Neurosurgery & Psychiatry, 75, 560–566. Wardlaw, J. M. (2005). What causes lacunar stroke? Journal of Neurology, Neurosurgery & Psychiatry, 76, 617–619.

Lacunar State ▶ L’etat Lacunaire

Lacunar Stroke ▶ Lacunar Infarction

Lacunar Stroke Syndrome (LACS) ▶ Lacunar Infarction

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Lacune

Lacune ▶ Lacunar Infarction

Lamotrigine J OHN C. C OURTNEY 1, C RISTY A KINS 2 1 Children’s Hospital of New Orleans New Orleans, LA, USA 2 Mercy Family Center Metarie, LA, USA

Common Skin rash, ataxia, headache, tremor, fatigue, sedation, nausea, vomiting, constipation, flu syndrome, pharyngitis, and asthenia.


Additional Information Generic Name Lamotrigine

Brand Name

Drug Interaction Effects: http://www.drugs.com/drug_interactions.html Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Free Drug Online and PDA Software: www.epocrates.com Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.com: 8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill Identification: http://www.drugs.com/pill_identification.html

Lamictal, Labileno, and Lamictin

Class Anticonvulsant, mood stabilizer

Proposed Mechanism(s) of Action Blocks voltage-sensitive sodium channels, inhibits glutamate release.

Indication

Landau–Kleffner Syndrome J EFFREY B. T ITUS 1,2 , R EBECCA K ANIVE 1, M ICHAEL M ORRISSEY 1 1 St. Louis Children’s Hospital St. Louis, MO, USA 2 Washington University School of Medicine St. Louis, MO, USA

Synonyms

Bipolar I disorder (maintenance), partial seizures, and seizures associated with Lennox–Gastaut syndrome.

Acquired epileptic aphasia

Off Label Use

Definition

Bipolar depression, bipolar mania, schizophrenia (psychosis), neuropathic pain, and chronic pain.

Landau–Kleffner Syndrome (LKS) is an epileptic encephalopathy marked by sudden and relatively rapid onset of aphasia in a child with normal or near-normal language development. The acquired aphasia classically begins with verbal auditory agnosia (word deafness) and often progresses to expressive language impairment. Sleepactivated epileptiform abnormalities maximally over the temporal regions are a defining feature. Clinical seizures are evident in most cases, but they are not necessary for the diagnosis.

Side Effects Serious Stevens–Johnson rash, organ failure, epidermal necrolysis, drug hypersensitivity, and sudden unexplained death in epileptic patients.

Landau–Kleffner Syndrome

Epidemiology LKS is an especially rare epilepsy syndrome, constituting about 0.2% of childhood epilepsy syndromes. Its prevalence, however, has increased in recent years. LKS is believed to be almost twice as common in males. A genetic predisposition for LKS has not been identified. Rather, some have suggested that an underlying autoimmune condition may be responsible for at least a subset of cases. The cognitive changes associated with LKS are believed to be at least partially secondary to the continuously abnormal epileptiform activity during sleep, which is thought to create structural changes and functional impairments that often do not abate following normalization of the EEG. Nevertheless, while functional imaging studies demonstrate abnormalities in the temporal lobes, structural studies are typically normal in patients with LKS. The majority of cases are believed to be idiopathic.

Natural History, Prognostic Factors, and Outcomes The typical age of onset for LKS is between 3 and 10 years, with about 70% of cases presenting with sudden language impairment prior to the age of 6 years. LKS typically first presents as verbal auditory agnosia (word deafness), characterized by progressive problems with speech comprehension. Some children with LKS also exhibit an inability to respond to nonspeech sounds, such as environmental stimuli (e.g., doorbell, knock on the door, telephone, etc.). The loss of auditory response is precipitous, often occurring over a matter of days, and it can be easily mistaken for hearing impairment. Differential diagnosis of peripheral hearing loss is important. While impairment in receptive language is the defining feature of LKS, subsequent impairment in expressive language is common. Functional outcome in LKS is variable. Despite the good response of clinical seizures and EEG abnormalities to medication intervention, 75–86% of patients may experience residual language problems of varying severity. Later age of onset appears correlated with better functional outcomes (after 6 years of age). The majority of patients with LKS experience clinical seizures (about 70%), but they are typically infrequent and often disappear prior to the age of 15. Seizures typically begin around the onset of language impairment, and only about 20% of patients experience seizures beyond the age of 10. Partial motor, atypical absence, and generalized

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tonic–clonic seizures are most common, with about 80% of children exhibiting convulsive seizures. Prognosis does not appear to be affected by seizure frequency or type.

Neuropsychology and Psychology of LKS All children with LKS should undergo a comprehensive neuropsychological assessment to track developmental changes during the course of the condition. This can help with differential diagnosis and treatment planning. Because children with LKS typically do not exhibit further regression in language functioning after normalization of their EEG, monitoring of cognitive functioning can help track the presence of continued epileptiform abnormalities and potentially maximize functional outcome by helping to inform the need for treatment modifications. Children with LKS typically have a history of normal or near-normal language development and exhibit severe impairments in receptive language. The core deficit in receptive language appears to be impairment in phonologic decoding or discrimination. The cognitive profile in LKS can be variable, though relatively better developed expressive language as compared with receptive language is often observed. Korkman, Granstro¨m, Appelqvist, and Liukkonen (1998) examined five children with LKS and found variable intellectual ability. On the Performance scale of the Wechsler Intelligence Scale for Children-Revised (WISC-R), one child performed in the average range (PIQ = 91), one performed in the borderline range (PIQ = 74), and one performed in the mildly impaired range (PIQ = 60). Two children could not complete the WISC-R and were administered the Leiter International Performance Scale, scoring in the mildly impaired range. All children had severely impaired ability to understand everyday speech, with only minimal language competence observed in the child with average intelligence. This was limited to comprehension of selected single words and the ability to repeat four numbers on the digit span subtest of the WISC-R. He was also able to understand some contextually dependent expressions with the help of lipreading. Verbal expression in the sample of children with LKS was variable. Two of the five children were mute, and two could only use some short phrases with poor phonological production. The child with average intelligence spoke fluently, though his production was difficult to understand and marked by phonological distortions. The four children with lower functioning also exhibited impaired performance on nonverbal neuropsychological measures (e.g., visualmotor integration).

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Further investigation by Korkman et al. of the receptive language impairment in LKS concluded that children with LKS demonstrate selectivity in their auditory processing impairment. Specifically, children with LKS have problems discriminating phonological stimuli, with relatively preserved ability to discriminate environmental stimuli. This suggests a specific impairment of their ability to decode speech-related auditory stimuli. Such a specific impairment in linguistic processing may implicate dysfunction in brain regions associated with processing more complex auditory stimuli, or it may suggest impairment in brain regions specifically devoted to speech processing. There is some suggestion that the definition of LKS should be expanded to include acquired impairment of any higher-order cognitive functioning in association with sleep-activated epileptiform abnormalities. This is based upon the finding that patients with LKS do not only demonstrate language impairments, but also deficits in visual processing, visual-motor integration, and executive functioning. LKS can also be associated with emotional and behavioral disturbance. It is not uncommon for children to exhibit elevated anxiety and/or depression in response to the sudden loss of the ability to comprehend speech. This can contribute to behavioral difficulties related to confusion and frustration. Attention problems and behavioral dysregulation are common in children with LKS and occur in about two-thirds of cases. Sleep disruption may contribute to these problems.

Evaluation LKS is distinguished by its characteristic clinical course and features. It can be difficult to make a clinical distinction between LKS and continuous spikes and waves during slow-wave sleep (CSWS), which is associated with more global neuropsychological deterioration. Progressive deterioration of language functioning, especially receptive language, distinguishes LKS from CSWS. The EEG abnormalities in LKS are also more focal than in CSWS. Children with autistic spectrum disorders (ASDs) are often referred for consideration of LKS following speech regression. These conditions are distinguishable in their clinical progression by an earlier onset of language regression in children with ASD and a wider range of cognitive impairment (i.e., both verbal and nonverbal deficits). Children with ASD also do not typically demonstrate an association between developmental regression and the onset of epileptiform abnormalities. When present, epileptiform abnormalities are often more diffuse than in LKS.

The characteristic awake EEG profile in LKS typically includes normal for age background activity. Focal or multifocal interictal epileptiform spikes and sharp waves can be seen during the awake EEG. Interictal epileptiform activity is markedly activated during non-REM (NREM) sleep and are most predominant over the bilateral temporal regions. The term “electrical status epilepticus of sleep” (ESES) is often used to describe the EEG if at least 80% of NREM sleep is occupied by epileptiform discharges. The classic presentation of ESES includes a steady increase in spikes and sharp waves during the transition into sleep, marked activation specifically during slow wave sleep (SWS), and a marked reduction in spikes and sharp waves during rapid eye movement (REM) sleep. LKS variants have been described to account for clinical presentations that include sleep-activated epileptiform abnormalities without the traditional features of LKS, particularly the classic acquired verbal auditory agnosia. Variants of LKS have been proposed to describe children with regression of more anterior language abilities (anterior LKS), often manifesting as oral-motor apraxia. Children with preexisting language impairment who may or may not exhibit language regression are sometimes described as developmental LKS.

Treatment As part of treatment planning, all children with LKS should undergo a comprehensive neuropsychological assessment. This can help in clarifying the diagnosis, monitoring the effectiveness of treatment, and planning appropriate educational and therapeutic services. Intensive speech-language therapy is of critical importance for children with LKS, and participation should be maximized to promote the best functional outcomes. Because learning can be variable in LKS, school services should be designed based upon the needs of each individual patient. In general, however, efforts to circumvent deficits in auditory processing are typically helpful. This includes the use of visual mediation during learning and the application of alternative means of communication (e.g., sign language, picture-based communication systems, etc.). Academic services can be enhanced with the assignment of a one-on-one aide, particularly when attention and concentration are also impaired. Corticosteroid therapy is generally considered to be the treatment of choice in LKS, and can help decrease epileptiform activity, reduce seizure frequency, and

Language

improve language outcomes. Early and long-term treatment are recommended; however, persistent language dysfunction following normalization of the EEG is common. Treatment with antiepileptic drugs (AEDs) in LKS is traditionally believed to be effective for managing seizures, but the benefits for language development are minimal. Valproic acid and carbamazepine are commonly used in LKS, but no specific AED regimen has been associated with better efficacy. Initial treatment with AEDs and follow-up corticosteroid therapy for nonresponders is a common treatment approach. Effectiveness of the ketogenic diet has been demonstrated in some patients with LKS, and multiple subpial transection (MST) has been found to be of benefit in some patients who fail to respond to medication intervention.

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Landry–Guillain–Barre´ Syndrome ▶ Guillain–Barre´ Syndrome

Landry–Guillain–Barre´–Strohl Syndrome ▶ Guillain–Barre´ Syndrome

Language Cross References ▶ Aphasia ▶ Auditory Agnosia ▶ Autistic Disorder ▶ Carbamazepine ▶ Electroencephalography ▶ Epilepsy ▶ Leiter International Performance Scale, Revised ▶ Seizure ▶ Steroids ▶ Valproate ▶ Wechsler Intelligence Scale for Children

References and Readings Aicardi, J. (1998). Diseases of the nervous system in childhood. London: Mac Keith. Korkman, M., Granstro¨m, M., Appelqvist, K., & Liukkonen, E. (1998). Neuropsychological characteristics of five children with the LandauKleffner Syndrome: Dissociation of auditory and phonological discrimination. Journal of the International Neuropsychological Society, 4, 566–575. Riviello, J. J., & Hadjiloizou, S. (2008). The Landau-Kleffner Syndrome and epilepsy with continuous spike-waves during sleep. In J. M. Pellock, B. F. D. Bourgeois, & W. E. Dodson (Eds.), Pediatric epilepsy: Diagnosis and therapy (3rd ed., pp. 351–358). New York, NY: Demos. Tatum, W., Genton, P., Bureau, M., Dravet, C., & Roger, J. (2001). Less common epilepsy syndromes. In E. Wyllie (Ed.), The treatment of epilepsy: Principles and practice (3rd ed., pp. 551–575). Philadelphia, PA: Lippincott Williams & Wilkins. Van Slyke, P. A. (2002). Classroom instruction for children with LandauKleffner Syndrome. Child Language Teaching and Therapy, 18, 23–42.

A IMEE D IETZ University of Cincinnati Hastings and Williams French Building Cincinnati, OH, USA

Definition One avenue for the communication of thoughts, feelings, and/or ideas. Language is comprised of symbols (i.e., vocabulary/words), which are socially agreed upon by members of a given community/culture (e.g., American English, French, Spanish, etc.), and includes various regional dialects within those communities/cultures. Language requires the rule-governed use of these symbols (e.g., use of grammar, putting words together to form complete sentences, etc.) and includes four domains: (1) verbal expression (e.g., speaking aloud), (2) written expression (e.g., composing a letter, an e-mail, an essay, etc.), (3) auditory comprehension (e.g., the understanding of what others say aloud), and (4) reading comprehension (e.g., the understanding of written text). Language does not include the motoric act of moving the articulators (e.g., the lips, tongue, cheeks, vocal folds, etc.) to pronounce the words (i.e., speech) or the motoric act of constructing the letters with a writing instrument.

Cross References ▶ Aphasia ▶ Articulation

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Language Analysis

▶ Speech ▶ Written Language Disorders


Large Occipito-Frontal Head Circumference ▶ Megalencephaly

Brown, C. M., & Hagoort, P. H. (Eds.). (2000). The neurocognition of language. Oxford: Oxford University Press. Owens, R. E. (2008). Language development: An introduction (7th ed.). Upper Saddle River, NJ: Allyn & Bacon. Tanner, D. C. (2006). Case studies in communication sciences and disorders. Upper Saddle River, NJ: Pearson.

Language Analysis ▶ Discourse Assessment

Lashley, Karl Spencer (1890–1958) J ULIA RUTENBERG , B ENJAMIN M. H AMPSTEAD Emory University/Rehabilitation Medicine Atlanta VAMC RR&D CoE Atlanta, GA, USA

Major Appointments

Language of Confusion ▶ Cognitive-Communication Disorder

Language of Generalized Intellectual Impairment ▶ Cognitive-Communication Disorder

Language Sampling ▶ Discourse Assessment

Laplace–Gauss Distribution

1910–1911 Teaching fellow, University of Pittsburgh, Pittsburgh, PA, USA 1917–1918 Instructor of Psychology, University of Minnesota, Minneapolis, MN, USA 1920–1926 Full Professor, University of Minnesota, Minneapolis, MN, USA 1926–1929 Research Psychologist, Behavioral Research Fund, Institute for Juvenile Research, Chicago, IL, USA 1929–1935 Professor of Psychology, University of Chicago, Chicago, IL, USA 1929 President of the American Psychological Association 1935–1937 Professor of Psychology at Harvard, Cambridge, MA, USA 1937 President of the Eastern Psychological Association 1937–1955 Research Professor of Neuropsychology at Harvard, Cambridge, MA, USA 1947 President of the American Society of Naturalists 1942–1955 Director at the Yerkes Laboratories of Primate Biology, Orange Park, Florida, USA

▶ Normal Curve


Large Brain ▶ Megalencephaly

1927–1930 Lashley served on the National Research Council. 1937 Howard Crosby Warren Medal by the Society of Experimental Psychologists

Lashley, Karl Spencer (1890–1958)

1943 Daniel Giraud Medal for Zoology by the National Academy of Sciences 1951 Named Foreign Member of the Royal Society 1953 William Baly Medal for Physiology by the Royal College of Physicians 1953 LL.D. honorary degree from Johns Hopkins University. Professional Memberships: American Society of Zoologists, American Physiological Society, Society of Experimental Psychologists, American Society of Human Genetics, American Philosophical Society, American Academy of Arts and Sciences, New York Academy of Sciences (Honorary Member), Florida Psychological Association, Florida Psychological Association, National Academy of Science, British Institute for the Study of Animal Behaviour (Honorary Member), American Neurological Association, Harvey Society (Honorary Member), British Psychological Association (Honorary Fellow), Foreign Member of the Royal Society.


Perhaps Lashley’s primary contribution to the field of Neuropsychology was the manner in which he methodologically examined brain–behavior relations. To this end, he supported the equipotentiality of cortical tissue, a theory initially posited by Pierre Florens, which claimed that behaviors (or cognitive abilities) are dependent on the brain functioning as a whole. This theory also claims that the amount of neural tissue damaged is more important than its location because the remaining cerebral tissue has the potential to take over the functions of the lesioned area(s). This former notion became known as the principle of mass action, which states that the severity of the behavioral deficit is directly proportional to the mass of the lesioned tissue. However, Lashley believed that sensory and motor abilities were, to some extent, ‘‘localized’’ within the cortex. In 1929, Lashley published Brain Mechanisms and Intelligence: A Quantitative Study of Injuries to the Brain, work that introduced the concepts of mass action and equipotentiality. Using rats, his research examined the effects of cerebral lesions on acquisition and retention of information. This work used a variety of tasks including mazes of varying difficulty, brightness discriminations, and inclined-plane problems (which requires discrimination of the direction of slope of a surface). The major finding was that

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maze learning performance was negatively affected by brain damage, but this effect depended on the extent of the damage rather than the location. Since the location of the cerebral lesion did not dramatically affect performance, he concluded that ‘‘intellect’’ was not localized in any single region of the brain. This distinction did not necessarily hold true for simpler behaviors. For example, large occipital lesions caused deficits in the brightness discrimination task, but the animals rapidly relearned these discriminations at the same rate as they had initially. He addressed this discrepancy by suggesting that more complex tasks such as the maze learning requires more cortical tissue than the simpler brightness discrimination or inclined-plane tasks. Lashley’s address as president of the American Psychological Association, entitled ‘‘Basic Neural Mechanism in Behavior,’’ was given to the International Congress of Psychology at Yale University and published in 1930. He discussed two theories of brain functioning: that of localization and reflex theory. Both of these theories propose the specialization of specific brain regions (localization) or single cells (reflex theory) for specific and limited functions. Lashley refuted both hypotheses and proposed that, rather than focusing on where psychological functions are localized, psychologists should address the means through which complex systems are able to exert an influence on each other. Lashley’s 1938 presidential address to the Eastern Psychological Association (‘‘The Experimental Analysis of Instinctive Behaviour’’) was regarded by some as one of his greatest contributions to comparative psychology. He reasoned that determinants of behavior can be instinctual as well as learned. For example, animal behaviors such as the honey dance of bees, kin recognition, and reproduction activity can all be considered instinctive. He suggested that at a neural level, all instincts are the result of an activation of specific sensorimotor mechanisms. In his subsequent research, he claimed that just as genetic determinants can be used to describe differences between species, similar determinants can be used to describe differences within species. He also proposed that individual variation within the brain was the cause of variable mental capacity and behavior. For this reason, he opposed attempts at developing standardized cortical maps, including those of Brodmann. In his 1949 presidential address to the American Society of Naturalists, Lashley emphasized the role of materialism, behaviorism, and genetics as determinants of behavior

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and suggested that, from an evolutionary standpoint, increased capacity and abilities are primary factors that differentiate animals.

Short Biography Karl Spencer Lashley, the only son of Charles Gilpen Lashley and Margaret Blanche Spencer, was born on June 7, 1890 in Davis, West Virginia. Starting when Lashley was 4, the family began a series of moves that included stays in California, Seattle, and finally Alaska during the 1898 gold rush, before they finally returned to West Virginia in 1899. Despite the lack of regular and formal schooling, Lashley graduated from Davis High School when he was 14 years old, during which time he developed his interest in the natural behavior of animals and steadfast love of nature. In 1905, Lashley matriculated to the University of West Virginia, where he enrolled in a zoology course taught by neurologist John Black Johnston. This course had a profound effect on Lashley and not only helped determine his major course of study, but formed a basis for his career in the life sciences. From 1906 to 1909, Lashley was a research assistant to Albert M. Reese and worked with the Golgi series of frog brains. He graduated from the University of West Virginia with an A.B. degree in 1910 and was subsequently awarded a teaching fellowship in biology at the University of Pittsburgh. The following year, he completed his thesis on the bacteriology of rotten eggs and earned his Master of Science from the University of Pittsburgh. Lashley spent the summer of 1911 at Cold Spring Harbor Biological Laboratory on Long Island conducting genetics research on Stylonychia, a member of the ciliate family. The following fall, Lashley enrolled in a Ph.D. program in zoology at Johns Hopkins University. After completing his Ph.D. in 1914, Lashley remained at Hopkins as a Bruce Fellow in zoology, conducting field experiments on the behavior of sea birds with John B. Watson. In the fall of 1917, he accepted his first teaching position as instructor of psychology at the University of Minnesota, but took a leave of absence after his first year in order to work alongside John B. Watson at the U.S. Interdepartmental Hygiene Board in Baltimore, educating the public about the dangers of venereal disease. In 1920, he returned to the University of Minnesota, where he became a full professor in psychology. In 1926, he moved to Chicago, accepting a position as a research psychologist with the Behaviour Research Fund at the Institute for Juvenile Research. In 1929, he was elected president of

the American Psychological Association and also became Professor of Psychology at the University of Chicago, where he remained until 1935. Between the years 1920 and 1929, Lashley continued to research the functions of the central nervous system, with specific focus on sensory discrimination and the formation and retention of habits in animals. His contributions to the mechanisms of learning during this time were particularly noteworthy. In 1935, he became Professor of Psychology at Harvard, which involved more administrative work than he preferred, and in 1937 after threatening to resign, he was appointed Research Professor of Neuropsychology, which gave him freedom to pursue research outside Harvard. In 1942, he accepted the Directorship position at the Yerkes Laboratories of Primate Biology in Orange Park, Florida. He retired from this position in 1955, as he dealt with a series of prolonged and difficult medical treatments for hemolytic anemia. After traveling to England to sign the Charter Book of Royal Society, he unexpectedly died in France on August 7, 1958 at the age of 68.

Cross References ▶ Equipotentiality ▶ Localization

References and Readings Bartlett, F. C. (1960). Karl Spencer Lashley: 1890–1958. Biographical Memoirs of Fellows of the Royal Society, 5, 107–118. Beach, F. A. (1961). Karl Spencer Lashley 1890–1958, A biographical memoir. http://books.nap.edu/html/biomems/klashley.pdf Beach, F. (1960). The neuropsychology of Lashley. Selected papers of K. S. Lashley. New York: McGraw-Hill. Carmichael, L. (1959). Karl Spencer Lashley, experimental psychologist. Science, 129(3360), 1410–1412. Darrow, C., Landis, C., Heath, C., Stone, L., & Lashley, K. (1932). Studies in the dynamics of behavior. Chicago: University of Chicago Press. Hebb, D. O. (1959). Karl Spencer Lashley: 1890–1958. The American Journal of Psychology, 72(1), 142–150. Kimble, G., Wertheimer, M., & White, C. (2006). Portraits of pioneers in psychology (Portraits of pioneers in psychology (Hardcover APA)). Washington: American Psychological Association (APA). Lashley, K. (1929). Brain mechanisms & intelligence 1st edition. Chicago: University of Chicago Press. Mook, D. (2004). Classic experiments in psychology. New York: Greenwood Press. Weidman, N. (2006). Constructing scientific psychology: Karl Lashley’s mind-brain debate (Cambridge studies in the history of psychology). New York: Cambridge University Press.

Late-Delayed Effects of Radiation Therapy

Late-Delayed Effects of Radiation Therapy C AROL L. A RMSTRONG The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Synonyms

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often attributed to degeneration of vascular tissue. There are few prospective studies of the late-delayed time points in patients with nonmalignant disease. Results in patients with lymphoma, leukemia, small-cell lung cancer, and other malignant histologies have demonstrated that groups of patients with malignant cancer are often neuropsychologically impaired at baseline even when there are no CNS tumors. Because radiation is most often given to higher-grade neoplasms, cognitive decline may also be associated with disease progression versus radiation effects (Archibald et al., 1994; Armstrong et al., 2005).

Late effects

Current Knowledge Definition The late-delayed radiation symptoms are most commonly seen in the neurological, neurocognitive, and endocrine systems. Late-delayed radiation effects on the central nervous (CNS) system are iatrogenic injuries that are irreversible and possibly progressive. Neural substrates include vasculopathies and capillary damage, stroke, demyelination, disruption of cellular mitosis, radiation necrosis, diaschisis, destruction of the hypothalamic– pituitary axis, and gliosis. Late-delayed effects often present as leukoencephalopathy within the tumor field; this gross change is more commonly seen on MRI or CT at higher total radiation doses.

Historical Background Changes have been retrospectively described several months to many years posttreatment (for review, see Armstrong, Gyato, Awadalla, Lustig, & Tochner, 2004; Crossen, Garwood, Glatstein, & Neuwelt, 1994; DeAngelis, Delattre, & Posner, 1989). Declines have been reported using PET and [11C] methionine, an amino acid marker, in gray matter remote from the tumor during several months to over 1 year after irradiation (Sato, Kameyama, Kayama, Yoshimoto, Ishiwata, & Ito, 1995; Shishido, Uemura, Inugami, Tomura, Higano, & Fujita, 1990). Neural disruption can also occur; in a case study, a patient with a low-grade mixed glioma showed a gradual decrease following RT in amino acid metabolism in the gray matter contralateral to the tumor (Sato et al., 1995), whereas both CT and MR were unrevealing. The temporal pattern of the onset of late-delayed symptoms is uncertain and likely influenced by other factors. Onset of clinically apparent symptoms has been reported as early as 2 years posttreatment, and as late as 10–20 years posttreatment,

Cognitive effects in adults: While retrospective reports and individual case studies demonstrate the severity that late-delayed damage from RT can cause, there are few prospective studies on the actual frequency of such severe damage in both children and adults. Vigliani and colleagues’ prospective study provided outcome at 4 years (Vigliani, Sichez, Poisson, & Delattre, 1996). One patient demonstrated a decline in cognitive function, two patients improved, and one patient was unchanged. Armstrong and colleagues’ prospective longitudinal study of 26 adult patients with low-grade, supratentorial, brain tumors extending 6 years from treatment found selective cognitive decline emerging only at the 5-year endpoint, limited to visual memory, in half of the patients who were treated with moderately high, part field radiation (mean of 5400 cGy, fractions of 180 cGy). Ratings of clinical MRIs (T2-weighted and FLAIR images) showed, also in half of the patients, mild accumulation of hyperintensities with onset from 6 months to 3 years and with no further progression to 6 years. White matter atrophy and total hyperintensities demonstrated the effect, and subcortical and deep white matter, corpus callosum, cerebellar structures, and pons accounted for these changes over time. Klein and colleagues reported that radiation-related memory impairment in their adult patients with lowgrade tumors only occurred for those receiving dose fractions that exceeded 200 cGy (Klein et al., 2002). Early-delayed radiation effects are not predictive of latedelayed effects (Armstrong, Corn, Ruffer, Pruitt, Mollman, & Phillips, 2000), and those who develop somnolence during the acute phase of radiotherapy acquire no extra risk for late-delayed neuropsychological deficits (Berg, Ch’ien, Lancaster, Williams, & Cummins, 1983). Cognitive effects in children: A consensus has been reached from overwhelming evidence that RT compromises the developing brain, and that younger children

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have worse outcomes than older children (Armstrong et al., 2004; Mulhern, Hancock, Fairclough, & Kun, 1992). Younger children may be more vulnerable to decline prior to RT due to the interaction of their tumor and treatment with their brain development, which is thought to be due to the iatrogenic effects on their more actively myelinating brain regions, though developmental effects from injury to hippocampal neuroblast progenitors and immune-mediated processes are also implicated. Radcliffe and colleagues reported on prospective findings in children treated with radiation therapy for medulloblastomas, and found an initial decline in IQ at 2 years posttreatment, but no evidence of further decline at the 5-year follow-up (Radcliffe, Bunin, Sutton, Goldwein, & Phillips, 1994). Medulloblastoma is a malignant tumor type and is treated with craniospinal radiation, and thus cognitive impairment from the tumor, dose burden, and malignancy effects are likely comorbid factors. Nevertheless, children with medulloblastoma and late-delayed radiation effects are invariably in need of special supports for learning. In other prospective studies of children with leukemia or medulloblastomas, both of whom received craniospinal doses (doses to whole brain and the spine, in lower doses than those given to targeted tumors, but with a boost to a high dose to the tumor area), declines in IQ were not limited to radiation effects and were also related to underlying disease (Longeway et al., 1990; Mulhern, Fairclough, & Ochs, 1991; Ochs et al., 1991; Rubenstein, Varni, & Katz, 1990).

Future Directions The differential diagnosis of progressive, irreversible latedelayed radiation effects includes the need to differentiate (1) cognitive impairment from tumors versus vascular injury; (2) effects of other clinical factors as effects of the tumor, nonmass neoplasm (such as leukemia), malignancy, and age, for example, which are risk factors for iatrogenic damage; (3) diffuse radiation effects from other disease-related changes in the brain such as from cytokines, inflammation, and chronic pulmonary disease; and (4) changes in imaging studies of radiotherapy effects versus surgical intervention and white matter risks of aging. Thus, it is critical to understand the clinical factors that interact with radiotherapy effects, and their impact on cognitive and radiographic outcomes. The introduction of proton beam therapy for treatment of many brain tumors, which significantly decreases the distribution and therefore total dose of radiation to healthy brain tissue, is hoped to bring decreases in the

functional morbidity of radiotherapy, without loss of, and possibly benefits to, survival rates. The hippocampus neural milieu may be particularly damaged by therapeutic levels of radiotherapy (▶ Radiation Injury), and other studies are needed to understand its temporal patterns of change, following radiotherapy and its recovery capacity.

Cross References ▶ Early-Delayed Effects of Radiation ▶ Involved Field Radiotherapy ▶ Late Effects of Radiation Therapy ▶ Proton Beam Therapy ▶ Radiation Injury ▶ Radiation Necrosis

References and Readings Archibald, Y., Lunn, D., Ruttan, L., Macdonald, D., Del Maestro, R., Barr, H., et al. (1994). Cognitive functioning in long-term survivors of high-grade glioma. Journal of Neurosurgery, 80, 247–253. Armstrong, C., Corn, B., Ruffer, J., Pruitt, A., Mollman, J., & Phillips, P. (2000). Radiotherapeutic effects on brain function: Double dissociation of memory systems. Neuropsychiatry Neuropsychology and Behavior Neurology, 13, 101–111. Armstrong, C., Gyato, K., Awadalla, A., Lustig, R., & Tochner, Z. (2004). A critical review of the clinical effects of therapeutic irradiation damage to the brain: The roots of controversy. Neuropsychology Review, 14(1), 65–86. Armstrong, C. L., Hunter, J. V., Hackney, D., Shabbout, M., Lustig, R., Goldstein, B., et al. (2005). MRI changes during the early-delayed phase of radiotherapy effects. International Journal of Radiation Oncology Biology and Physics, 63(1), 56–63. Berg, R., Ch’ien, L., Lancaster, W., Williams, S., & Cummins, J. (1983). Neuropsychological sequelae of postradiation somnolence syndrome. Developmental Behavioral Pediatrics, 4, 103–107. Crossen, J., Garwood, D., Glatstein, E., & Neuwelt, E. (1994). Neurobehavioral sequelae of cranial irradiation in adults: A review of radiation-induced encephalopathy. Journal of Clinical Oncology, 12, 627–642. DeAngelis, L., Delattre, J., & Posner, J. (1989). Radiation-induced dementia in patients cured of brain metastases. Neurology, 39, 789–796. Klein, M., Heimans, J., Aaronson, N., van der Ploeg, H., Grit, J., Muller, M., et al. (2002). Effect of radiotherapy and other treatmentrelated factors on mid-term to long-term cognitive sequelae in low-grade gliomas: A comparative study. The Lancet, 360, 1361–1368. Longeway, K., Mulhern, R., Crisco, J., Kun, L., Lauer, S., Casper, J., et al. (1990). Treatment of meningeal relapse in childhood acute lymphoblastic leukemia: II. A prospective study of intellectual loss specific to CNS relapse and therapy. American Journal of Pediatric Hematology/ Oncology, 12, 45–50.

Late Effects of Radiation Therapy Mulhern, R., Fairclough, D., & Ochs, D. (1991). A prospective comparison of neuropsychological performance of children surviving leukemia who received 18-Gy, 24-Gy, or no cranial irradiation. Journal of Clinical Oncology, 9, 1348–1356. Mulhern, R., Hancock, J., Fairclough, D., & Kun, L. (1992). Neuropsychological status of children treated for brain tumors: A critical review and integrative analysis. Medical and Pediatric Oncology, 20, 181–191. Ochs, J., Mulhern, R., Fairclough, D., Parvey, L., Whitaker, J., Ch’ien, L., et al. (1991). Comparison of neuropsychologic functioning and clinical indicators of neurotoxicity in long-term survivors of childhood leukemia given cranial radiation or parenteral methotrexate: A prospective study. Journal of Clinical Oncology, 9, 145–151. Radcliffe, J., Bunin, G., Sutton, L., Goldwein, J., & Phillips, P. (1994). Cognitive deficits in long-term survivors of childhood medulloblastoma and other noncortical tumors: Age-dependent effects of whole brain radiation. International Journal of Developmental Neuroscience, 12, 327–334. Rubenstein, C., Varni, J., & Katz, E. (1990). Cognitive functioning in long-term survivors of childhood leukemia: A prospective analysis. Journal of Developmental and Behavioral Pediatrics, 11, 301–305. Sato, K., Kameyama, M., Kayama, T., Yoshimoto, T., Ishiwata, K., & Ito, M. (1995). Serial positron emission tomography imaging of changes in amino acid metabolism in low grade astrocytoma after radio- and chemotherapy. Neurologia Medico-Chirurfica (Tokyo), 35, 808–812. Shishido, F., Uemura, K., Inugami, A., Tomura, N., Higano, S., Fujita, H., et al. (1990). Value of 11C-methionine and PET in the diagnosis of low grade gliomas. Kaku Igaku, 27, 293–302. Vigliani, M., Sichez, N., Poisson, M., & Delattre, J. (1996). A prospective study of cognitive functions following conventional radiotherapy for supratentorial gliomas in young adults: 4-year results. International Journal of Radiation Oncology, Biology, Physics, 35, 527–533.

Late Effects ▶ Late-Delayed Effects of Radiation Therapy ▶ Radiation Injury

Late Effects of Radiation Therapy C AROL L. A RMSTRONG The Children’s Hospital of Philadelphia Philadelphia, PA, USA

Definition Radiation therapy is considered a necessary treatment for many types of tumor, and is frequently used in the CNS

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for the treatment of primary brain tumors, arteriovenous malformations, small-cell lung cancer, high-risk leukemia, brain metastases, and other neoplasms. However, there are severe iatrogenic late effects of radiotherapy (XRT), which are very significant when they occur in the context of either partial brain fields or whole brain field. Late effects are defined as those occurring after the complete dose of XRT has been administered. XRT is used as an adjunct to surgical resection of the mass, which provides the best cure or prevention of recurrence. The curative effects are not always the goal, and it is also used as prophylaxis, and as palliation, that is, to prolong life expectancy.

Current Knowledge Neurological effects: The late effects of XRT include risks of leukoencephalopathy, radiation necrosis (which is a rare but progressive mass lesion), and functional impairment. Fig. 1a and 1b of the late effects of radiotherapy within the radiation portals of a 43-year-old woman treated for a right parietal glioma at 6 months posttreatment (earlydelayed phase), and at 8 years posttreatment (late-delayed phase). There are also neurological effects when critical brain structures are unavoidably included in the radiation fields, such as vasculitis or stroke that can occur when the circle of Willis is involved, blindness when the optic tracts and chiasm are involved, and ototoxicity when the auditory canals are involved. Today, every effort is made to avoid these structures. Late effects often injure multiple systems, including but not limited to widespread endocrine system dysfunction, diabetes type 2, chronic renal insufficiency, asthma, cardiac enlargement, and bilateral cataracts. Cognitive effects: The most severe late effects of XRT have become less frequent with improvements in treatment regimens, however, patients remain at very high risk for neurocognitive effects. The late cognitive effects depend on a number of factors, including the area treated, dose burden, patient age, and the time posttreatment (Armstrong, Gyato, Awadalla, Lustig, & Tochner, 2004). Late effects are not a matter initially of widespread cognitive dementia, but are specific and related to the underlying neurophysiological mechanisms (▶ Early-Delayed Effects of Radiation and ▶ Late-Delayed Effects of Radiation Therapy). The most well-documented clinical risks for injury that must be differentiated from radiation effects are those of cancer type, radiation regimen, concurrent chemotherapy, and age (Vigliani, Duyckaerts, & Delattre, 1997).

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Late Effects of Radiation Therapy. Figure 1 (a) Adult with resected right parietal glioma, with focal field photon radiotherapy to the tumor region, six months post completion of radiotherapy. The effects on bilateral posterior parietal lobe are visible in the white matter. (b) Same adult with resected right parietal glioma, without tumor recurrence, eighth years after radiotherapy, with portal fields still visible in the posterior brain

However, the risk of eventually developing dementia or mental retardation from large dose burden to the brain may occur, though is not usually seen until many years, if not decades, later.

Cross References ▶ Early-Delayed Effects of Radiation ▶ Late-Delayed Effects of Radiation Therapy ▶ Radiation Injury

Late Life Function and Disability Index TAMARA B USHNIK Rusk Institute for Rehabilitation Medicine – NYU Langone Medical Center New York, NY, USA

Synonyms LL-FDI

References and Readings Armstrong, C., Gyato, K., Awadalla, A., Lustig, R., & Tochner, Z. (2004). A critical review of the clinical effects of therapeutic irradiation damage to the brain: The roots of controversy. Neuropsychology Review, 14(1), 65–86. Vigliani, M.-C., Duyckaerts, C., & Delattre, J.-Y. (1997). Radiationinduced cognitive dysfunction in adults. In C. J. Vecht (Ed.), Handbook of clinical neurology, Vol. 23(67), Neuro-oncology (pp. 371–388). New York: Elsevier.

Description Late life function and disability index (LL-FDI) was designed in 1992 to assess and demonstrate change over time or after an intervention in two outcome domains: disability and function (Haley et al., 2002; Jette et al., 2002). The disability component contains 16 items in two dimensions: frequency of performance and

Late-Life Forgetfulness

limitation in performance of life tasks. Each dimension contains two disability domains; within frequency, there are personal and social role domains, and within limitation there are instrumental and management role domains (Jette et al., 2002). The function component contains 32 items in three dimensions: upper extremity, basic lower extremity, and advance lower extremity functions. The three dimensions did not subdivide into different domains.

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and the physical functioning scale of the SF-36 and the London Handicap Scale, the LL-FDI demonstrated less ceiling effects and better precision across the range of function and disability (Dubuc, Haley, Ni, Kooyoomjian, & Jette, 2004).

Clinical Uses This is a fairly new outcome measure which is still being developed. As such, it is primarily used in research

Historical Background The LL-FDI is based upon Nagi’s disablement framework for assessing functional limitations and disability components (Nagi, 1991). Functional limitations are conceptualized as limitations of the ability to perform actions or activities. Disability comprises performance of expected life tasks within a typical sociocultural and physical environment.

Psychometric Data Scaling: The frequency and limitation dimensions of the LL-FDI disability component exhibited good item separation along a hierarchy from easy to difficult items to endorse; this is supportive of a scale with good scaling (Jette et al., 2002). Similar findings were noted for the function component (Jette et al., 2002). Reliability: With an average interval of 12 days, testretest intraclass correlations ranged between 0.68–0.82 for the disability component and between 0.91 and 0.98 for the functional limitation component (Haley et al., 2002; Jette et al., 2002). Validity: In the original articles describing the development of the LL-FDI, the sample was divided into 4 groups: severe, moderate, slight, and no functional limitations based upon scores on the physical function scale of the SF-36. Both the functional limitation and disability components discriminated between the 4 groups (Haley et al., 2002; Jette et al., 2002). In a comparison of the LLFDI and scores on a short physical performance battery (SPPB) and a self-paced walk test, correlations between the two lower extremity functional limitation components and the SPPB and walk test were significantly stronger than with the upper extremity functional limitation component (Sayers, Jette, Haley, Heeren, Guralnik, & Fielding, 2004). In a comparison between the LL-FDI

Cross References ▶ Disability ▶ Functional Status ▶ SF-36/SF-12

References and Readings Dubuc, N., Haley, S. M., Ni, P., Kooyoomjian, J. T., & Jette, A. M. (2004). Function and disability in late life: Comparison of the Late-Life Function and Disability Instrument to the Short-Form-36 and the London Handicap Scale. Disability & Rehabilitation, 6, 362–370. Haley, S. M., Jette, A. M., Coster, W. J., Kooyoomjian, J. T., Levenson, S., Heeren, T., & Ashba, J. (2002). Late Life Function and Disability Instrument: II. Development and evaluation of the function component. Journals of Gerontology Series A: Biological Sciences and Medical Sciences, 57(4), M217–M222. Jette, A. M., Haley, S. M., Coster, W. J., Kooyoomjian, J. T., Levenson, S., Heeren, T., et al. (2002). Late Life Function and Disability Instrument: I. Development and evaluation of the disability component. Journal of Gerontology, 57A, M209–M216. Nagi, S. Z. (1991). Disability concepts revisited: Implications for prevention. In A. M. Pope & A. R. Tarlov (Eds.), Disability in America: Toward a national agenda for prevention. (pp. 309–327). Washington, DC: National Academy Press. Sayers, S. P., Jette, A. M., Haley, S. M., Heeren, T. C., Guralnik, J. M., & Fielding, R. A. (2004). Validation of the Late-Life Function and Disability Instrument. Journal of American Geriatric Society, 52, 1554–1559.

Late-Life Forgetfulness ▶ Benign Senescent Forgetfulness

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Latent Schizophrenia

Latent Schizophrenia ▶ Schizotypal Personality Disorder

Latent Variable M ICHAEL D. F RANZEN Allegheny General Hospital Pittsburgh, PA, USA

Definition Latent variables are those that cannot be directly observed or measured and are estimated on the basis of other observations. This is true for many variables in the social sciences and the cognitive sciences. Latent variables may be hypothetical abstract constructs or factors derived from a factor analysis. The statistical relations among the observed variables are interpreted to be due to the relation of the observed variables to the latent variable.

Lateral Corticospinal Tract ▶ Pyramidal System

Lateral Cuneate Nucleus ▶ Accessory Cuneate Nucleus

Lateral Dominance ▶ Hemispheric Specialization

Lateral Gaze Palsy D OUGLAS I. K ATZ Boston University School of Medicine Braintree Rehabilitation Hospital Braintree, MA, USA

Current Knowledge In clinical neuropsychological terms, the construct of immediate recall verbal memory is a latent variable whose component-observed variables include accuracy scores for recall of a brief narrative text, and total correct paired associates of words across four learning trials. These scores are combined in a way that reflects the relation between them in comprising the latent factor of verbal immediate recall. In this way, a larger set of observations can be combined into a simpler structure.

Cross References ▶ Factor Analysis

References and Readings Borsboom, D., Mellenbergh, D. J., van Heerden, J. (2003). The theoretical status of latent variables. Psychological Review, 110, 203–219. Finch, H., Davis, A., Dean, R. S. (2010). Factor invariance assessment of the Dean–Woodcock Sensory–Motor Battery for patients with ADHD versus nonclinical subjects. Educational and Psychological Measurement, 70, 161–173.

Synonyms Horizontal gaze palsy

Definition Lateral gaze palsy is an inability to produce horizontal, conjugate eye movements in one or both directions. Lesions of the cranial nerve VI (abducens) nucleus in the pons cause ipsilateral, horizontal gaze palsy by disrupting motoneurons that innervate the ipsilateral lateral rectus muscle by way of cranial nerve VI, and interneurons that connect to the contralateral cranial nerve III nucleus in the midbrain, via the medial longitudinal fasciculus, to stimulate the medial rectus of the opposite eye. Lesions of the paramedian pontine reticular formation, adjacent to the abducens nucleus, may cause lateral gaze palsy, particularly involving ipsilateral saccadic eye movements. Lesions of the frontal or parietal cortical eye fields may also cause weakness of horizontal gaze (contralateral to frontal lesions and ipsilateral to parietal lesions) that becomes more subtle over time.

Lateral Geniculate Nucleus of Thalamus

Cross References ▶ Internuclear Ophthalmoplegia ▶ Oculomotor Nerve ▶ Pons

References and Readings Leigh, R. J., & Zee, D. S. (2006). The neurology of eye movements (4th ed.). New York: Oxford University Press.

Lateral Geniculate Nucleus of Thalamus A NDREW P RESTON 1, A LLISON S. E VANS 2 1 Brown University Medical School Providence, RI, USA 2 Chapel Hill Pediatric Psychology Chapel Hill, NC, USA

Synonyms LGN

Structure Anatomy & Location: The lateral geniculate nucleus (LGN) is a bilateral nucleus located on the caudal, inferior surface of the thalamus, lying lateral to the medical geniculate nucleus, inferior to the pulvinar and superior to the pretectal area. The LGN comprises six layers that relay information to cells in the visual cortex. The magnocellular layers (layers 1–2) send information to the area of the cortex that processes large features and motion. The parvocellular layers (layers 3–6) send information to the area of the visual cortex that processes finer details and color. Input pathways: Visual input from retinal ganglion cells in both eyes send information to the optic nerve, whose fibers cross at the optic chiasm and then continue in the optic tract. Fibers cross so that information from

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the right visual field from both eyes is sent to the left optic tract and information from the left visual field of both eyes is sent to the right optic tract. Optic tract fibers project around the midbrain to reach the LGN. Output pathways: From the LGN, third-order thalamocortical neurons project through the retrolenticular part of the internal capsule and form broadly spreading fibers called optic radiations, which terminate in the primary visual cortex of the occipital lobe. As the thalamocortical fibers leave the LGN, they are divided into inferior and superior radiations and pass around the lateral ventricle (Fig. 1). Inferior projections (Meyer’s loop) follow through the temporal lobe, synapse on inferior areas of the primary visual cortex, and supply information about the superior visual field. Superior projections travel through the parietal cortex, synapse on superior regions of the primary visual cortex, and supply information about the inferior visual field.

Function The primary function of the LGN is to relay visual information from the retina to the visual cortex.

Illness Lesions Prior to LGN: (a) Disease of the eyeball (e.g., cataract) and disease of the optic nerve (e.g., multiple sclerosis) lead to loss of vision in the affected eye, monocular blindness. (b) Damage to the medial aspect of the optic chiasm/compression of the optic chiasm (e.g., adjacent pituitary tumor) leads to bitemporal hemianopsia, the loss of peripheral vision in both eyes. (c) Damage to the lateral aspect of the optic chiasm (e.g., aneurysm of the internal carotid artery) will affect the fibers of the ipsilateral temporal hemiretina (nasal visual field) (d) Damage to optic tract leads to loss of vision in contralateral visual field in both eyes (homonymous hemianopsia). Lesions At or After LGN: (a) Meyer’s Loop cut: lesion to temporal optic radiation leads to contralateral superior quadrantanopsia. (b) Lesion to parietal portion of optic radiation leads to contralateral inferior quadrantanopsia. (c) Lesion to occipital cortex leads to contralateral homonymous hemianopsia with macular sparing (intact foveal vision).

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Lateral Inhibition

Lateral Geniculate Nucleus of Thalamus. Figure 1 Schematic of visual system, including LGN (Mendoza and Foundas, 2008)

Cross References ▶ Magnocellular Neurons ▶ Meyer’s Loop ▶ Optic Radiations ▶ Optic Tract ▶ Parvocellular Neurons ▶ Thalamus ▶ Visual Field Deficit

Mendoza, J., & Foundas, A. L. (2008). Clinical neuroanatomy: A neurobehavioral approach. New York: Springer. Purves, D., Augustine, G. J., Fitzpatrick, D., Katz, L. C., LaMantia, A., McNamara, J. O., & Williams, S. M. (2001). Neuroscience (2nd ed.). Sunderland, MA: Sinauer Associates.

Lateral Inhibition


R ONALD A. C OHEN Brown University Providence, RI, USA

Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sunderland, MA: Sinauer Associates. Crossman, A. R., & Neary, D. (2000). Neuroanatomy (2nd ed.). Churchill Livingston. Haines, D. E. (2000). Neuroanatomy: An atlas of structures, sections, and systems (5th ed.). Baltimore, MD: Lippincott Williams & Wilkins.

Synonyms Sensory inhibition; Spatial inhibition

Lateralization

Definition Lateral inhibition refers to the capacity of excited neurons to reduce the activity of their neighbors. Neurons that are firing inhibit the stimulation of surrounding. Accordingly, only the neurons that are most stimulated and least inhibited respond. Lateral inhibition plays an important role in visual perception by increasing the contrast and resolution of visual stimuli. This occurs at various levels of the visual system. For example, when a small light is presented in a dark environment, receptors on the retina central to the stimulus are activated and transduce the visual information to the brain, while receptors that are peripheral to the stimulus send inhibitory signals that enhance the perception of darkness in the surrounding. This process has the effect of creating greater dark-light contrast and is responsible for the Mach band visual effect. Similar inhibitory processes occur cortically and contribute to both object and spatial perception.

Cross References ▶ Cortical Magnification ▶ Enhancement ▶ Visual Perception


References and Readings Haines, D. E. (Ed.). (2002). Fundamental neuroscience. Philadelphia, PA: Churchill Livingstone/Elsevier Science. Wilson-Pauwek, L., Akesson, E. J., Stewart, P. A., & Spacey, S. D. (2002). Cranial nerves in health and disease. Hamilton, Ontario, Canada: B.C. Decker.

L Lateral Medullary Syndrome


Lateral Spinothalamic Tract ▶ Spinothalamic Tract

Lateral Sulcus Lateral Lemniscus J OHN E. M ENDOZA Tulane University Medical Center New Orleans, LA, USA

Definition A pontine–mesencephalic pathway that carries auditory information. Lemniscus means ‘‘ribbon’’ and anatomically is used to refer to a fiber pathway or tract. The fibers in the lateral lemniscus originate in the cochlear and superior olivary nuclei of the pons and carry both ipsilateral and

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contralateral auditory information. It terminates in the nuclei of the inferior colliculi in the mesencephalon. The lateral lemniscus should be differentiated from the medial lemniscus, another brainstem fiber pathway which carries somatosensory information. Because of the bilateral nature of the auditory pathways, unilateral lesions of the lateral lemniscus are not normally associated with clinically notable hearing losses.

▶ Wallenberg’s Syndrome

Ratliff, F., Knight, B. W., Toyoda, J., & Hartline, H. K. (1967). Enhancement of Flicker by lateral inhibition. Science, 158(3799), 392. Von Be´ke´sy, G. (1967). Sensory inhibition. Princeton, NJ: Princeton University Press.

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▶ Sylvian Fissure

Laterality ▶ Handedness ▶ Hemispheric Specialization

Lateralization ▶ Hemispheric Specialization

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Lawton iADL Scale

Lawton iADL Scale ▶ Lawton-Brody Instrumental Activities of Daily Living Scale

Lawton-Brody iADL Scale ▶ Lawton-Brody Instrumental Activities of Daily Living Scale

Lawton-Brody Instrumental Activities of Daily Living Scale J ESSICA F ISH Medical Research Council Cognition & Brain Sciences Unit Cambridge, UK

Synonyms Lawton-Brody iADL scale; Lawton iADL scale

Description The Lawton-Brody Instrumental Activities of Daily Living (iADL) scale covers eight functional domains: using the telephone, shopping, food preparation, housekeeping, laundry, transport, medication, and finances. Competence is rated according to descriptions of the person’s level of involvement/ability in each activity (e.g., for using the telephone, four levels of competence are described: operates telephone on own initiative, looks up and dials numbers, etc.; dials a few well-known numbers; answers telephone but does not dial; does not use telephone at all). It should be administered by a researcher or practitioner with relevant qualifications, with ratings derived from interview, which should take between 10 and 15 min. Activities in which a person has never participated are excluded (e.g., males are not assessed on laundry, meal preparation, and housekeeping if their wife has always taken responsibility for these things), but can also be rated regarding whether a person could do them. There are several ways of scoring responses, but Graf (2008) states that common practice is to score each item either on a simple independent/dependent basis, or on a 3-point

scale (dependent/needs assistance/independent). Vittengl, White, McGovern, and Morton (2006) reviewed a variety of scoring procedures and found no advantages for more complex over simple scoring systems, and thus advocate the use of simple methods.

Historical Background The Lawton-Brody iADL scale was developed in 1969 (Lawton & Brody, 1969). Despite the lack of good evidence regarding its psychometric properties, it is a very widely used measure.

Psychometric Data Sikkes, de Lange-de Klerk, Pijnenburg, Scheltens, and Uitdehaag (2009) provided a review of ADL scales for people with dementia. Each scale was examined in terms of nine indices of psychometric quality. The LawtonBrody iADL scale was found to have a positive result in only one index, as there was evidence of good internal consistency for two subscales (Cronbach’s alpha between 0.78 and 0.91). However, it should be noted that only 2 of the 12 scales examined achieved two positive results (the Disability Assessment for Dementia and the Bristol Activities of Daily Living Scale). Sikkes et al. also report that there is an apparent ceiling effect; in that 20% of people with dementia in one study achieved the highest possible score. They report that data for determining construct validity, reliability, and responsiveness were not available. Vittengl et al. (2006) examined the value of different scoring methods, including whether the scale’s predictive validity differed according to the type of scoring procedure used. Their sample comprised 231 people aged on average 78.3 years (SD 11.4), who lived in rural areas of the midwestern USA. Although no significant differences were found between different scoring methods, iADL scale scores were found to be strong predictors of mental status, psychosocial functioning and living situation, moderate predictors of outpatient medical care, and weak (but statistically significant) predictors of depression and inpatient medical care during the preceding month, suggesting the measure is a valid one to use.

Clinical Uses The scale is useful in determining an individual’s level of competence in everyday activities, and consequently, the

Lead Exposure

degree of assistance that may be necessary for the individual to ensure the attainment of an optimum level of functioning. It may also be useful in measuring change over time or with intervention, but the lack of evidence regarding its sensitivity to change may mean that a different or an additional outcome measure should be used for this purpose.

Cross References ▶ Independent Living Scales® ▶ Katz Index of ADLs

References and Readings Graf, C. (2008). How to try this: The Lawton Instrumental Activities of Daily Living Scale. American Journal of Nursing, 108, 52–62. Lawton, M. P., & Brody, E. M. (1969). Assessment of older people: Selfmaintaining and instrumental activities of daily living. The Gerontologist, 9, 179–186. Sikkes, S. A. M., de Lange-de Klerk, E. S. M., Pijnenburg, Y. A. L., Scheltens, P., & Uitdehaag, B. M. J. (2009). A systematic review of Instrumental Activities of Daily Living scales in dementia: Room for improvement. Journal of Neurology, Neurosurgery and Psychiatry, 80, 7–12. Vittengl, J. R., White, C. N., McGovern, R. J., & Morton, B. J. (2006). Comparative validity of seven scoring systems for the instrumental activities of daily living scale in rural elders. Aging and Mental Health, 10, 40–47.

Lead Exposure B RADLEY J. H UFFORD Rehabilitation Hospital of Indiana Indianapolis, IN, USA

Synonyms Lead toxicity; Plumbism

Definition Direct or indirect contact with organic or inorganic lead resulting in a harmful alteration of body structure and/or function, including illness or death.

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Current Knowledge Lead is a virtually omnipresent metal that has been reported to negatively affect all organ systems. The neurological effects of lead are particularly pernicious with architectural changes to or death of neurons in a variety of cerebral areas reported (e.g., hippocampal areas and the amygdala may be targets of organic lead; the cerebellum and hippocampus may be favored by inorganic lead). Acute lead poisoning is much less common in the United States, as use of lead in plumbing, fuels, food storage, and paint has been reduced and enhanced regulations have lowered the risk of occupational lead exposure. However, persons with low socioeconomic status continue to be differentially exposed to lead. Research indicates that chronic low-level lead exposure is of concern in its own right, particularly with respect to its effect on the neurological development of children. Children are more sensitive to lead exposure than adults, as they absorb and store lead more readily and tend to ingest greater quantities. Cognitive and neurobehavioral deficits attributed to lead exposure in childhood have been noted to be quite variable. This variability is likely dependent upon when in the developmental/brain maturation process the child is exposed to lead but may also be exacerbated by environmental stressors. High blood lead levels are routinely found to be associated with more severe attentional, intellectual, executive, and externalizing problems in emotional/social adjustment than lower blood levels. However, even very low blood lead levels (less than 5 micrograms of lead per deciliter) have been correlated with lowered intelligence quotients, cognitive inflexibility, slower psychomotor reaction speed, and aspects of attentional difficulties. A reduction in blood lead levels over time has not been consistently shown to result in cognitive/ behavioral improvement. This may be due to the fact that lead remains in bone, brain, and other tissues for much longer periods of time than it does in the circulation. Cognitive and behavioral impairments caused by childhood lead exposure appear to remain present to some degree into adulthood and may ultimately lead to dementing conditions. These and similar findings have resulted in the conclusion that any exposure to lead, no matter how seemingly minimal, is unacceptably dangerous. Adult exposure to lead has been documented to result in a wide variety of neurocognitive deficits. Psychomotor speed, memory, and executive/attentional abilities are common cognitive functions affected, particularly at higher lead levels, and may present as similar to a dementia. Irritability, dysphoria, sleep disturbances, and other affective symptoms are commonly reported. Psychotic

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symptoms have been observed as well. Some of the clinical symptom picture may vary with the type of lead (organic versus inorganic), degree of cognitive reserve (i.e., the brain’s resistance to cognitive decline), psychosocial stressors, and other factors. Acute lead exposure leads to acute cognitive difficulties, but chronic lead exposure or cumulative lead build-up may be associated with progressive cognitive deterioration. Older adults with a past history of lead exposure are at particular risk for delayed lead-related effects (e.g., due to osteoporosis causing stored bone lead to be re-released into the circulation).

Cross References ▶ Mercury Exposure ▶ Toxic Exposure

References and Readings Chiodo, L., Covington, C., Sokol, R., Hannigan, J., Jannise, J., Ager, J., et al. (2007). Blood lead levels and specific attention effects in young children. Neurotoxicology and Teratology, 29(5), 538–546. Hartman, D. (1995). Neuropsychological toxicology. New York: Plenum Press. Niesink, R., Jaspers, R., Kornet, L., van Ree, J., & Tilson, H. (1999). Introduction to neurobehavioral toxicology: Food and environment. New York: CRC Press. Toxicological Profile for Lead (Update). (August, 2007). Atlanta, GA: United States Department of Health and Human Services, Agency for Toxic Substances and Disease Registry. White, L., Cory-Slechta, D., Gilbert, M., Tiffany-Castiglioni, E., Zawia, N., Virolini, M., et al. (2007). New and evolving concepts in the neurotoxicology of lead. Toxicology and Applied Pharmacology, 225(1), 1–27.

Definition A learned treatise is authoritative text, which is considered evidence that is provided to support claims. Learned treatise is admissible as evidence in court. Learned treatise can be text that an expert witness used that text to reach his conclusions. Under the Federal Rules of Evidence, either party can introduce a learned treatise as evidence.

Cross References ▶ Federal Rules of Evidence

References and Readings Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology. New York: Taylor & Francis. Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: principles of productive attorney neuropsychologist relations. In G. Larrabee (Ed.), Forensic neuropsychology: a scientific approach. New York: Oxford University Press. Kaufmann, P. M. (2008). Admissibility of neuropsychological evidence in criminal cases: competency, insanity, culpability, and mitigation. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press. Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts (3rd ed.). New York: Guilford Press.

Learning Lead Toxicity ▶ Lead Exposure

Learned Treatise N ATHALIE D E FABRIQUE Cook County Department of Corrections Chicago, IL, USA

R ICK PARENTE Towson University Towson, Maryland, USA

Synonyms Acquisition of knowledge; Erudition

Definition Synonyms Authoritative reference

Learning is the cognitive process of acquiring a skill or knowledge. It can also be defined as a relatively permanent change in performance that results from practice.

Learning

Historical Background Learning is not the same as performance. It is how much change in performance occurs over successive trials. The question of what goes on between learning trials has generated many different theories. The discussion begins with the classical theories, which provide the backdrop to the more recent theoretical discussions of this elusive concept. Table 1 is a summary of these theoretical orientations: Classical conditioning – Pavlov’s classical conditioning paradigm is central to all theories of human learning. Although Pavlov studied dogs salivating to meat powder, in Western European cultures, the same phenomenon has been studied with eyeblink conditioning. In this paradigm, a light brightens just before a puff of air strikes the eye, and a person blinks in response to the air puff. Soon, the person learns to blink as soon as the light changes but before the puff occurs. The puff is the unconditioned stimulus, and the light is the conditioned stimulus. The conditioned response is blinking to the light before the air puff (unconditioned stimulus) occurs. Classical conditioning usually does not involve learning any new response or goal-directed problem-solving behavior. Most studies involve conditioning existing physiological reactions. Nevertheless, many theorists regard classical conditioning to be the fundamental principle of learning. Contiguity–behaviorist theories – John Watson was perhaps the first American to study learning theory. Watson rejected the then popular German notion of studying unconscious experience, and he advocated studying

Learning. Table 1 Summary of classical learning theorist’s concepts of learning Reward What is learned? necessary

Theorist

Tradition

Pavlov

Connectionist

CS-CR connections

No

Watson

Behaviorism

S-R connections

No

Guthrie

Behaviorism

S-R connections

No

Thorndike

Reinforcement S-R connections

Yes

Hull

Reinforcement S-R connections

Yes

Skinner

Reinforcement S-R reflexes and R-S operants

Yes

Wertheimer Gestalt

Gestalt whole

No

Tolman

Sign-gestalt expectancies

No

Purposive behaviorism

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only measurable, objective, and practical behavior. He formalized his thinking in his now-famous article Psychology as the Behaviorist Views It, in 1913. Watson also rejected the study of other concepts such as motivation and instinct. He believed that behavior resulted from conditioned reflexes, and he denied the possibility that people are born with special mental abilities or traits or predispositions to behave in a certain way. He was therefore a strong proponent of the notion that environment shapes behavior, and he minimized the role of hereditary factors in the development of learned behaviors. Watson felt that human learning resulted from classical conditioning. People are born with a repertoire of stimulus–response connections called reflexes. However, it is possible to modify these and to develop new ones with age. Through classical conditioning, a variety of stimulating sources come to elicit the repertoire of reflexes with which one is born. Watson, however, felt that this was only part of the learning process. Complex habits are learned by forming sequences of reflexes. Learning sequences of skilled reflexes accounted for the uniquely human ability to learn complex behaviors. Watson also proposed two basic principles of learning: frequency and recency. The frequency principle means that the more frequently one has made a given response, the more likely one is to do it again. The recency principle means that the more recently one has made the response, the more likely it is to occur again. Other theorists like Edwin Guthrie (1886–1959) were contemporaries of Watson and were highly influenced by his behaviorism. Guthrie’s basic principle of learning was ‘‘a combination of stimuli which has accompanied a movement will on a future occurrence, tend to be followed by that movement’’ (Guthrie, 1952). In essence, if a response follows a stimulus once, it will probably follow it again when the stimulus returns. Guthrie felt that the last response a person makes in a situation will be the first one he or she makes in the same situation when it recurs. However, he felt that learning would occur strictly by contiguity, that is, through making the response in the presence of the stimulus even though no reward was present. According to him, reward simply preserved the last response. Guthrie specified several ways of breaking bad habits. In general, to break a bad habit, one must first discover a stimulus that evoked the undesirable response and then make a different response in the presence of that stimulus. To accomplish this, he proposed three methods. The first was the threshold method. This involved presenting the eliciting stimulus so faintly and weekly that the response based on the bad habit did not occur. The next step was to

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gradually increase the intensity of the eliciting stimulus only slightly but always just below the threshold necessary to elicit the undesired response. This method is especially useful for eliminating emotional responses. The second method involves inducing fatigue. A person would emit the undesirable response repeatedly until he or she tired and stopped making the response. At that point, the person did something else, which became the last response associated to the stimulus. The third method involved creating a variety of different stimuli that became associated with the undesired response. The original stimulus therefore no longer induced the response. Reinforcement theories – A hallmark of reinforcement theories is that pain and pleasure are consequences of one’s actions (Bentham, 1789). Edward Thorndike (1898) was perhaps the first American psychologist to modify the connectionist views described above to include the notion of reinforcement as a necessary and sufficient condition for learning. Thorndike’s study of cats led him to conclude that an animal’s ability to learn stimulus and response connections defined what is called intelligent behavior. Thorndike, like Guthrie and Watson, believed that the bonds of learning strengthened with practice. He called this principle the law of exercise. However, he also proposed a more important law, which he called the Law of effect. The law of effect stated that behavior depended on the effect that it produced. If the effect was satisfying, then learning strengthened; if the effect was annoying, then the stimulus–response connection weakened (Thorndike 1911). Like Guthrie and Watson, Thorndike said nothing about feelings. He simply described a connectionist system that would either enhance or diminish behavior with reinforcement. Skinner (1938) defined two types of learning. Respondent behavior occurred automatically to a specific type of stimuli. Respondent behavior involves specific types of stimulus and response connections that Skinner called reflexes. It followed the laws of classical conditioning. However, Skinner felt that most behavior is not respondent, but operant. An organism emits operant behavior in response to the environment; it is not elicited by any particular stimulus. For Skinner, once operant behavior occurred and was closely followed by reinforcement, the probability that it would occur again increased. Skinner also distinguished between positive and negative reinforcers. A positive reinforcer was something pleasurable that followed the occurrence of an operant, whereas a negative reinforcer involved the removal of something aversive following the operant response. Punishment involved the application of something aversive

following a response. Skinner’s research indicated that punishment did not permanently eliminate behavior. Punishment was ineffective because it was temporary. Skinner found that punished behaviors often would reoccur with even greater frequency later on. Skinner also developed the notion of schedules of reinforcement, which referred to the pattern by which reinforcers followed responses. The simplest schedule involved continuous reinforcement, that is, where the reward followed the operant response every time an individual emits the operant. With intermittent reinforcement, the reward followed the response with less frequency. Skinner’s research indicated that responding would actually increase with intermittent reinforcement relative to continuous schedules. Moreover, when learning to respond with intermittent reward, the response was even more persistent once the reward was eliminated entirely relative to conditions in which the person initially received continuous reward. Skinner also posited the notion of shaping which was the mechanism by which people learn complex behaviors. He considered learning a larger, more complex behavior to involve shaping smaller behaviors he called successive approximations. Reinforcement theory – Clark Hull proposed perhaps the most well-developed connectionist-learning theory of the mid-twentieth century. Like Watson, Guthrie, and Thorndike, his concept of behavior involved the association between stimulus and response. Unlike Skinner, Hull believed that animals and humans never simply emitted a response. The response always resulted from some eliciting stimulus. Hull proposed a four-stage analysis of learning. This analysis involved a series of ‘‘intervening variables,’’ that is, unobservable processes that mediated behavior. Hull believed that knowing the values, amounts, and characteristics of the environment, would allow him to determine their effect on the intervening variables, which, in turn, would allow him to predict behavior. In the second stage, Hull defined several intervening variables like habit strength, which was the current strength of the learning connection between the independent variables and the desired response. Drive was the person’s current motivational state. Reward provided drive reduction. In addition, inhibition was the extent to which a person could keep from making a response. For Hull, reward was necessary for learning to occur. He was also the first theorist of his day to describe the concept of incentive motivation. For Hull, learning involved not only the establishment of an association between a stimulus and a response; it also involved learning that a particular reward was

Learning

associated with a particular response. Incentive motivation learning was necessary for behavior to occur. Hull’s model of learning can be summarized according to the following multiplicative relationship among his intervening variables: Behavior ¼ Habit Drive Incentive=Motivation Inhibition: Kenneth Spence was one of Hull’s students. Spence developed Hull’s theory, especially the notion of incentive. Unlike Hull, Spence assumed that habit strength did not depend on reinforcement. Habit strength would increase whenever a person made the response together with the stimulus that evoked it, however, a person could also learn that greater responding produced greater rewards.

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good configurations. One learns that certain signals or signs come to elicit these gestalt expectations. Tolman defined six kinds of learning. Cathexis was a tendency to seek out specific goals when experiencing specific drives. Equivalent believes involved the expectation that the situation will either be rewarding or punishing. For example, giving an employee a pat on the back eventually becomes associated with feelings of acceptance and a job well done. Field expectancies are essentially the same thing as sign-gestalt expectations. Field expectancies are called knowledge or mental maps of the environment. Field cognition modes are ways that one chooses to learn or one’s desire to learn certain things rather than others. Drive discrimination refers to the ability to discriminate or distinguish different drives. Motor patterns included sequences of smaller behaviors that formed larger molar behavior.

Current Knowledge Future Directions Cognitive interpretations of learning – Max Wertheimer started the cognitive revolution in Germany and in the USA. Wertheimer chose the word gestalt, which literally means pattern or configuration, to describe human perception and learning. The gestalt psychologists described several different principles of perception. For example, the law of proximity described how items that are close together as a unit are perceived. The law of closure indicated that human mind seeks conclusion and that one seems to perceive partially closed areas as holes. The law good configuration indicated that an event is typically perceived in its simplest and most complete form. Unlike the reinforcement theorists, Gestalt theorists did not ask what has a person learned to do in terms of stimulus–response associations; they asked how has the person learned to do it. The gestalt psychologist studied memory traces but considered them to be cognitive structures that determined human perception of novel situations. Edward Tolman was another cognitive psychologist who was heavily influenced by the gestalt movement. His book Purposive Behavior in Animals and Man presented the view that behavior was a response to goals and that an individual is capable of shifting behavior in response to the environment. He also believed that behavior was molar, that is, it included large behavioral units that were organized into purposeful goal-seeking strategies. Cognitions involved the amalgamation of several different learning experiences that formed what Tolman called sign-gestalt expectations. In other words, a person comes to expect an organized world that conforms to

Out of this combination of theories developed several other theories of verbal development. Skinner’s original book Verbal Behavior (1957) applied his conditioning theory to the acquisition of language. His work precursed a long tradition of verbal learning research that studied the association of verbal paired associations and later, the free recall and recognition of verbal materials. This tradition was eventually challenged by other theorists like Chomsky who felt that Skinner’s account emphasized the environment and its relation to language development at the expense of the individual’s own active part in the process of learning language. Chomsky argued that children are born with an innate set of linguistic principles, which can be modified as they learn the structure of any language. Chomsky felt that these structures are vastly more complex and complicated than could be accounted for by Skinnerian learning principles. Concept learning focuses on the ability of humans to abstract the latent structure of the environment, to visualize organization or to see ‘‘the big picture.’’ Human Information Processing models describe learning as a computer analogy that involves encoding, consolidation, and retrieval of newly learning information. These models assume that the ability to access information in memory is limited by how well the information can be encoded and consolidated. For example, just as one cannot retrieve a file on a computer hard drive without the file name, one cannot retrieve information stored in one’s long-term memory without appropriate cues encoded with it at the time it is learned. Just as the central

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Learning

Learning. Table 2 Summary of modern explanations of learning Explanation

What is learned?

Verbal learning

Associations between words and sound patterns

Psycholinguistics

Modification of innate linguistic skills

Concept learning

Prototypes, schemata, and exemplars

Human information processing

Mental programs for storing and retrieving information

Mathematical learning

Probabilities for predicting a response

Physiological learning

Neural loops and networks

Style of learning

Predispositions to respond

processing unit can defragment/disorganize information on the hard drive to speed up retrieval, the human executive monitor consolidates newly learned information in memory to create efficient behavior. Mathematical learning theory is not a theory per se, but a group of algorithms that are capable of predicting certain types of behavior. Physiological learning theory studies the various cellular and biochemical changes in learning that occur within the brain. What structures are involved and how do they operate? This view of learning assumes that different areas of the brain mediate different aspects of learning. For example, the hippocampus, which is part of the limbic system, plays a role in creation of long-term memories and ability to navigate spatial environments. The hippocampus is part of a larger memory system located in the medial temporal lobe that creates memories for what one verbalizes and for one’s ability to remember facts. The parahippocampal gyrus, which is next to the hippocampus, mediates one’s ability to recognize objects. Damage to both hippocampal areas results in severe amnesia. The hypothalamus mediates learning of sleep and wakefulness patterns, eating and drinking behavior, and the release of hormones. The thalamus controls the transfer of information between the cerebral hemispheres and partially controls one’s motivation. It also serves as a relay station between the limbic system and prefrontal cortex to the association cortex and other prefrontal areas of the brain. It therefore partially controls the ability to attend, organize, remember, and multitask. The cerebellum mediates learning of coordinated motor activity. The cortex consolidates spatial and olfactory memories.

The basal ganglia are a group of structures in the forebrain that controls the ability to excite or to inhibit learned behaviors. Rewards and punishments affect learning via the basal ganglia by conditioning its action-selection process. What the learning theorists call habits are stored in the striatum, which is a primitive cortical structure that mediates learning of motoric behavior. Learning style refers to behaviors that define ways a person goes about learning new information. For example, many people are convergent thinkers meaning that they use a set of rules to converge upon a solution to a problem. Divergent thinkers solve problems with a holistic style in which they try to see the ‘‘big picture.’’ Concept learning is the study of how humans learn categories that divide the environment and domain into examples and non-examples of things understood. Behaviorist’s explanations suggest that concepts are learned through experience and the association of stimuli with responses. The cognitive psychologists define concepts as collections of rules that generalized in different ways. These types of rule structures are often referred to as prototypes or schemata, or the amalgamation of different examples. Obviously, this account of learning cannot completely survey all of the important aspects of research in the field. However, this summary of learning has focused on the basic principles and theories that have shaped the development of the field. These theories are important because all of the more recent theories of learning are based on some form of connectionist, gestaltist, cognitive, or physiological underpinnings.

Cross References ▶ Association Areas ▶ Association Pathways ▶ Auditory Verbal Learning ▶ Basal Forebrain ▶ Basal Ganglia ▶ Behaviorism ▶ Cognitive Processing ▶ Extinction ▶ Forebrain ▶ Hippocampus ▶ Hypothalamus ▶ Learning Disability ▶ Limbic System ▶ Neocortex ▶ Striatum ▶ Thalamus

Learning Disability

References and Readings Bentham, J. (1789). The principles of morals and legislation. Theory of legislation. London: Paul, Trench, Trubner. Chomsky, N. (1967). Review of skinner’s verbal behavior. In L. A. Jakobovits, & M. S. Miron (Eds.), Readings in the philosophy of language. Englewood Cliffs, NJ: Prentice-Hall. Guthrie, E. R. (1952). The psychology of learning (Rev. Ed.). New York: Harper & Row. Logan, F. (1981). Fundamentals of learning and motivation. Dubuque, IA: W.C. Brown Co. Publishers. Pavlov, I. (1927). Conditioned reflexes. London: Clarendon. Skinner, B. F. (1938). The behavior of organisms: An experimental analysis. New York: Appleton-Century-Crofts. Thorndike, E. L. (1898). Review of Evans’ Evolution, ethics and animal psychology. Psychological Review, 5, 229–230. Thorndike, E. L. (1911). Animal intelligence. New York: Macmillan. Tolman, E. C. (1932). Purposive behavior in animals and men. New York: Appleton-Century-Crofts. Watson, J. (1919). Psychology from the standpoint of a behaviorist. Philadelphia: Lippincott. Wertheimer, M. (1923). Laws of organization in perceptual forms. In W. D. Ellis (Ed.), A sourcebook of gestalt psychology. New York: Harcourt, Brace and World.

Learning Aid ▶ Mnemonic Techniques

Learning Disability K ATHLEEN K EELY M C C ANN D EIDRICK , E LENA H ARLAN D REWEL Thompson Center for Autism and Neurodevelopmental Disorders, University of Missouri-Columbia Columbia, MO, USA

Synonyms Dyscalculia; Dysgraphia; Dyslexia; Dysphasia; Learning Disorders

Short Description or Definition The definition of learning disability has changed over time, but all definitions focus on the idea of academic difficulties that are not expected. According to the DSM-IV, a diagnosis of learning disability is assigned when a child does not make progress in a particular area of learning that would be

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expected on the basis of that child’s intellectual skills, grade level, and/or age. The child’s learning difficulties have a clear negative impact on their academic achievement or other daily living skills, and the learning difficulties are not due to a primary sensory deficit (APA, 1994). Newer definitions include the inability to make progress, despite the provision of high-quality academic instruction (Response to Intervention; Fletcher, Lyon, Fuchs, & Barnes, 2007).

Categorization Areas of learning difficulty include reading skills, reading comprehension, reading fluency, written expression, mathematics calculation, mathematics problem solving, listening comprehension, and oral expression (Fletcher et al., 2007).

Epidemiology The number of children with a learning disability in the USA varies based on the definition. Approximately 9% of boys and 6% of girls in the second and third grades have a reading disability (i.e., reading achievement is 1.5 standard deviations below what would be expected given IQ; Peterson & Pennington, 2009). Estimated incidence of children with mathematics disabilities ranges from 6% to 14% depending on the definition (i.e., math achievement lower than expected given IQ vs. math achievement lower than expected for grade or age level). Some studies show that math disabilities are more prevalent among girls but other studies find no gender differences (Barnes, Fuchs, & EwingCobbs, 2009). Approximately 46.4% of children between the ages of 6 and 21 who receive special education services in the USA are receiving services for a specific learning disability (U.S. Department of Education, 2009).

Natural History, Prognostic Factors, and Outcomes The etiology of learning disorders is complex, including neurobiological and genetic factors as well as the impacts of home environment and educational instruction (Pennington, 2009). Child outcomes may be affected by comorbidity. For example, comorbid mathematics and reading disorders, speech-language disorders, and ADHD may affect child functioning. In addition, children with learning disorders may be vulnerable to internalizing problems that develop in response to the impact of academic failure (Fletcher et al., 2007). Research suggests that

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Learning Disability

learning disorders are persistent, with most persons diagnosed during childhood continuing to exhibit the core components of learning disorders in adulthood.

Neuropsychology and Psychology of Learning Disorders Children with learning disorders are a heterogeneous group, exhibiting weaknesses in underlying cognitive skills that may vary depending on whether the child is diagnosed with a reading disability (e.g., phonological processing, rapid naming, verbal memory, and language), a mathematics disability (e.g., attention and semantic memory), or a disorder of written expression (e.g., fine motor skill and language) (Fletcher et al., 2007). Learning disorders, such as reading disabilities, are associated with externalizing behaviors, especially in boys. Internalizing behaviors, such as dysthymia, low self-esteem, and depressed mood are associated with reading disabilities in girls (Peterson & Pennington, 2009). Children with learning disabilities often have difficulty with attention, motivation, and self-regulation (Barnes et al., 2009).

Evaluation The process of evaluation for learning disabilities is complicated by frequent changes in how learning disabilities are defined, and the evaluation approach taken depends on the definition subscribed to by the evaluator. Historically, evaluation for learning disability involved identification of children who performed more poorly on tests of academic achievement than was expected on the basis of their measured intellectual ability. However, research suggests that these children do not differ in core academic skills, response to treatment, or outcome from children who exhibit low academic achievement that is consistent with their intellectual functioning in core academic skill deficits, response to treatment, and prognosis (Barnes et al., 2009; Peterson & Pennington, 2009). One option for addressing this concern is to diagnose both children who exhibit low achievement and those with a significant discrepancy between IQ and academic achievement with a learning disability (Peterson & Pennington, 2009). Alternatively, some authors suggest a response to intervention (RTI) approach, where unexpected academic underachievement is defined by a lack of response to empirically supported classroom instruction (Fletcher et al., 2007). However, there is not a consensus for this approach and it continues to be hotly debated (Reynolds & Shaywitz, 2009). In addition,

some authors advocate evaluation of underlying neuropsychological processes in order to individualize treatment (e.g., Naglieri, Conway, & Goldstein, 2009). Regardless of definition, there is an emerging consensus about the core academic and cognitive competencies associated with learning disabilities that can be used to guide evaluations, and the reader is referred to entries on dyslexia and nonverbal learning disabilities for more information.

Treatment Empirically supported interventions continue to emerge in the area of learning disabilities, with interventions in dyslexia leading the way, followed by interventions in math and written expression (NICHD, 2000; What Works Clearinghouse at http://ies.ed.gov/ncee/wwc/). Based on an overview of these interventions, Fletcher et al. (2007) offer ten overarching principles for the treatment of learning disorders that emphasize academically focused interventions that increase the time spent on academic work using systematic educational programs that teach content and meta-cognitive skills (e.g., self-monitoring).

Cross References ▶ Dyscalculia ▶ Dysgraphia ▶ Dyslexia ▶ Dysphasia ▶ Nonverbal Learning Disorder

References and Readings American Psychiatric Association. (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington DC: APA. Barnes, M. A., Fuchs, L. S., & Ewing-Cobbs, L. (2009). Math disabilities. In K. O. Yeates, M. D. Ris, H. G. Taylor, & B. F. Pennington (Eds.), Pediatric neuropsychology: Research, theory, and practice (pp. 297–323). New York: Guilford Press. Fletcher, J. M., Lyon, G. R., Fuchs, L. S., & Barnes, M. A. (2007). Learning disabilities: From identification to intervention. New York: Guilford Press. Naglieri, J. A., Conway, C., & Goldstein, S. (2009). Using the planning, attention, simultaneous, successive (PASS) theory within a neuropsychological context. In C. R. Reynolds & E. Fletcher-Janzen (Eds.), Handbook of clinical child neuropsychology (3rd ed., pp. 783–800). New York: Springer ScienceþBusiness Media. National Institute of Child Health and Human Development (2000). Report of the national reading panel. Teaching children to read: An evidence-based assessment of the scientific research literature on reading and its implications for reading instruction (NIH Publication No. 00-4769) Washington, DC: U.S. Government Printing Office.

Legal Competency Pennington, B. F. (2009). Diagnosing learning disorders: A neuropsychological framework, second edition. New York: Guilford Press. Peterson, R. L., & Pennington, B. F. (2009). Reading disability. In K. O. Yeates, M. D. Ris, H. G. Taylor, & B. F. Pennington (Eds.), Pediatric neuropsychology: Research, theory, and practice (pp. 324–362). New York: Guilford Press. Reynolds, C. R., & Shaywitz, S. E. (2009). Response to intervention: Ready or not? Or, from wait-to-fail to watch-them-fail. School Psychology Quarterly, 24, 130–145. U.S. Department of Education, Office of Special Education and Rehabilitative Services, Office of Special Education Programs (2009). 28th Annual report to congress on the implementation of the individuals with disabilities education act, 2006, Vol. 1. Washington, DC.

Learning Disorders ▶ Dyslexia ▶ Learning Disability ▶ Mathematics Disability

Left (or Right) Neglect ▶ Hemiinattention ▶ Hemispatial Neglect ▶ Neglect Syndrome ▶ Visual Neglect ▶ Neglect

Legal Competency R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Synonyms Competency to proceed

Definition Otherwise referred to as ‘‘competency to proceed,’’ this term refers to a criminal defendant’s capacity to participate effectively and make decisions on his or her own legal behalf while being prosecuted. It is to be distinguished from legal sanity because it has a very different legal

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standard. Furthermore, competency most commonly deals with current mental states (at the time of the proceedings) whereas insanity deals with the mental state (mens rea) at the time the offense(s) were committed. Competency to proceed is relevant to any portion of the process, from talking to police (i.e., Miranda rights to remain silent) to plead guilty, receive a sentence, waive one’s right to counsel, and even being executed. The rationale for questioning/examining a defendant’s competency can be traced back to English common law and the seventeenth century. In Dusky v United States (1960), the US Supreme Court upheld the right of a criminal defendant to be deemed competent through the criminal proceedings. Therefore, it would be considered a denial of due process when an incompetent person is tried for a crime, because, by definition, an incompetent person is one who cannot assist counsel and cannot adequately participate in the legal process. Dusky set the standard for competency in criminal matters in a two-pronged fashion: (1) the defendant must have the capacity to understand the criminal process he or she is facing (which includes an understanding of the roles of the various participants, such as the judge, jury, and prosecutor), and (2) the defendant must by capable to participate in that process through consulting with counsel in the preparation of his or her defense. Both of these prongs require a rational as well as a factual level of understanding. The following represent the specific competencies to be addressed during the criminal justice process: Competency to: (1) confess (or to waive rights at pretrial investigations); (2) plead guilty; (3) waive right to counsel; (4) stand trial; (5) be sentenced; (6) waive further appeal (when facing execution); and (7) be executed. Forensic psychiatrists and forensic psychologists (more often than neuropsychologists) can play a critical role in the assessment of each type of specific competency by conducting a comprehensive interview and via the administration of several empirically validated instruments specifically designed to assess various aspects competency. Neuropsychologists are more likely to become involved in competency assessments when a defendant claims amnesia, when there is a suspicion of dementia or a recent head injury, or when other neurological illness (es) that could impair the defendant’s cognitive abilities underlying competence are at issue. Neuropsychologists bring their specialized knowledge of the science of brainbehavior relationships and the impact of cognitive impairment on functional abilities and their expertise in detecting non-credible self-presentations (e.g., feigning, malingering). In this way, the neuropsychological examination may be a very valuable addition to the

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Legal Insanity

assessments provided by other professionals in assisting the trier of fact in criminal forensic settings.

Cross References ▶ Capacity ▶ Criminal Responsibility ▶ Diminished Responsibility ▶ Dusky v. United States (1960) ▶ Estelle v. Smith (1981) ▶ Insanity ▶ Insanity Defense

References and Readings Dusky v. U.S. (1960). 362 US 402. Estelle v. Smith, 451 U.S. 454 (1981). Marcopulos, B. A., Morgan, J. E., Denney, R. L. (2008). Neuropsychological evaluation of competency to proceed. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press. Poythress, N. G., Nicholson, R., Otto, R. K., et al. (1999). The MacArthur competence assessment tool-criminal adjudication (MacCat-CA). FL: Psychological Assessment Resources. Zapf, P. A., & Roesch, R. (2009). Evaluation of competence to stand trial. New York: Oxford University Press.

intellectual functioning that consists of both perceptual and conceptual tasks designed to measure aspects of attention, cognition, and memory. It is administered without vocal instructions and does not require verbal responses, although examinees may employ verbal mediation on some items. The Leiter-R has two primary domains, a Visualization and Reasoning battery made up of ten subtests, and a ten-subtest Attention and Memory battery. The latter battery is a relatively new addition intended to aid in differential diagnosis for individuals suspected of attentional difficulties, learning disorder, or who have experienced a traumatic brain injury. For individuals with severe cognitive impairments, the measure also allows for the calculation of a Growth Score to compare results and small changes in scores over successive administrations. Each of the two primary domains takes approximately 90 min to complete if all subtests are administered. However, there is an option to administer a brief screening version of four subtests that takes approximately 25 min. Subtests within each battery differ based on the individual’s age (e.g., 2–3, 4–4, 6–10, or 11–20).


Legal Insanity ▶ Guilty But Mentally III

Leiter International Performance Scale, Revised S TEPHEN M. K ANNE , M ICAH O. M AZUREK Thompson Center for Autism and Neurodevelopmental Disorders Columbia, MO, USA

Synonyms Leiter-R

Description The Leiter International Performance Scale, Revised (Leiter-R) is a widely used measure of nonverbal

The roots of the Leiter-R are found in the work of Russell Graydon Leiter who, in 1927, began developing a measure of intelligence that would have applicability across cultures. The forerunner of the original Leiter International Performance Scale was published in 1929 as a part of his Master’s Thesis. In 1940, the first commercial edition was published with 68 subtests that covered the ages of 2–18. The Leiter was revised in 1997 to its current incarnation, being standardized on over 2,000 children and adolescents covering the ages 2–20 years and 11 months. In addition to changes in test materials (e.g., colored cards instead of blocks, easels), the new version utilized more sophisticated psychometrics such as item response theory and linear structural relations (LISREL) in its development. The theoretical underpinnings of the Leiter-R lie in hierarchical models of cognitive skills hypothesizing an overall general ability termed g (e.g., Carroll, 1993; Gustafsson, 1984), and the Cattell–Horn–Carroll theory of cognitive abilities (CHC theory; cf. McGrew & Flanagan, 1998). The Leiter-R bases assess several specific factors of the CHC theory, which posits g at the highest hierarchical level above a range of other cognitive abilities, including fluid, crystallized, quantitative, short- and

Lemniscal System

long-term memory, auditory, processing speed, decision speed, and language development.

Psychometric Data The Leiter-R results can be reported as standard scores (such as traditional IQ scores), scaled scores, percentiles, in addition to age and grade equivalencies. Using a standardization plan based on the 1993 US Census statistics, the Leiter-R was standardized in 1,719 typical children and 692 children from nine different clinical groups. The Leiter-R manual reports internal consistency reliability ranging from 0.88 to 0.93 and concurrent validity with strong correlations with the Wechsler Intelligence Scale for Children – Third Edition ranging from r = 0.86 with the full scale IQ, to r = 0.80 with the verbal IQ index. Confirmatory factor analyses support the Leiter-R fitting, a hierarchical g model of cognitive abilities.

Clinical Uses Proponents of the Leiter-R suggest that its format and development emphasize the measurement of ‘‘fluid intelligence,’’ considered a more reasonable measure of innate intellectual ability, given its lack of vulnerability to educational, social, and other influences. Other advantages include the covered age range (2 years 0 months to 20 years 11 months) and the large range of standardized IQ scores, from 30 to 170. The Leiter-R has another advantage in its nonverbal administration and lack of dependence on verbal responses, allowing for the assessment of intellectual functioning when difficulties with language or hearing could be a potential source of confound. The authors of the measure suggest its appropriateness for individuals with hearing impairments, expressive or receptive language disorders, learning disabilities, cognitive impairment, traumatic brain injury, English as a second language, attentional problems, and autism spectrum disorders. Researchers have demonstrated its utility in assessing cognitive abilities of children with Fragile X (Hooper, Hatton, Baranek, Roberts, & Bailey, 2000), language impairment (Farrell & Phelps, 2000), and autism (Tsatsanis, Dartnall, Cicchetti, Sparro, Klin, & Volmar, 2003). Some researchers have voiced a concern regarding Leiter-R administration, noting that a number of children found it unusual for the examiner to remain silent

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throughout the administration. Standardized administration allows for some conversation before and in-between subtests, primarily to establish and maintain rapport. Tsatsanis et al. (2003) found very brief vocalizations (‘‘touch,’’ ‘‘point’’) to be effective for the population they assessed, which were children with low-functioning autism.

Cross References ▶ Stanford–Binet Intelligence Scales and Revised Versions ▶ WISC-IV ▶ Woodcock–Johnson Cognitive-Achievement Battery

References and Readings Carroll, J. B. (1993). Human cognitive abilities: A survey of factor-analytic studies. Cambridge; New York: Cambridge University Press. Farrell, M. M., & Phelps, L. (2000). A Comparison of the Leiter-R and the Universal Nonverbal Intelligence Test (UNIT) with children classified as language impaired. Journal of Psychoeducational Assessment, 18(3), 268–274. Gustafsson, J. E. (1984). A unifying model for the structure of intellectual abilities. Intelligence, 8, 179–203. Hooper, S. R., Hatton, D. D., Baranek, G. T., Roberts, J. P., & Bailey, D. B. (2000). Nonverbal assessment of IQ, attention, and memory abilities in children with Fragile-X syndrome using the Leiter-R. Journal of Psychoeducational Assessment, 18(3), 255–267. McGrew, K. S., & Flanagan, D. P. (1998). The intelligence test desk reference (ITDR):Cf-Gc cross battery assessment. Boston: Allyn & Bacon. Tsatsanis, K. D., Dartnall, N., Cicchetti, D., Sparrow, S. S., Klin, A., & Volkmar, F. R. (2003). Concurrent validity and classification accuracy of the Leiter and Leiter-R in low-functioning children with autism. Journal of Autism and Developmental Disorders, 33(1), 23–30.

Leiter-R ▶ Leiter International Performance Scale, Revised

Lemniscal System ▶ Posterior Columns

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Lennox–Gastaut Syndrome

Lennox–Gastaut Syndrome J EFFREY B. T ITUS 1,2 , R EBECCA K ANIVE 1, M ICHAEL M ORRISSEY 1 1 St. Louis Children’s Hospital St. Louis, MO, USA 2 Washington University School of Medicine St. Louis, MO, USA

Synonyms Childhood epileptic encephalopathy with diffuse slow spike-and-waves

Definition The Lennox–Gastaut syndrome (LGS) is characterized by generalized seizures of multiple types that emerge in children between the ages of 1 and 8 years and are accompanied by diffuse, interictal slow spike-and-wave discharges that occur at less than 3 Hz frequency. Prognosis is poor and mental retardation is traditionally considered a defining feature.

Epidemiology Approximately 1–4% of childhood epilepsy cases are believed to meet criteria for LGS. This accounts for about 10% of epilepsy syndromes that begin during the first 5 years of life. The prevalence rate in western countries ranges from 0.1 to 0.28 per 1,000, and about two in every 100,000 children receive a diagnosis of LGS each year. Representation among children with mental retardation is higher and is estimated to be about 7%. Among institutionalized patients with mental retardation, the percentage is estimated to be as high as 16.3%. Males are believed to be at higher risk for LGS, but this is not consistently supported in the literature. There do not appear to be differences in the prevalence of LGS among geographic regions or ethnicities.

Natural History, Prognostic Factors, Outcomes The typical age of onset in LGS is between the ages of 1 and 8 years; however, onset in early adulthood has been noted. Peak incidence occurs between 3 and 5 years of age. LGS can

be either idiopathic or symptomatic, with symptomatic cases being much more common (70–78%). Among these, brain malformations, hypoxic-ischemic insult, encephalitis, meningitis, and tuberous sclerosis are the most common etiologies. Patients with brain lesions are more likely to have abnormalities in the frontal lobes than any other brain region. LGS secondary to brain tumor or metabolic disorder is rare. Up to 44% of patients with LGS have been described as being cryptogenic (i.e., cause is suspected but cannot be determined). Family history of epilepsy is reported in 3– 27% of cases, and it is more common in the subset of patients with cryptogenic LGS (48%). Multiple seizure types develop in children with LGS and most frequently include tonic, atonic, myoclonic, and atypical absence seizures. Daily seizures are typical and are persistent in 60–80% of cases. While the course of the condition can vary, prognosis in LGS is poor. Mental retardation is evident in 20–60% of cases prior to diagnosis, and cognitive stagnation and/or deterioration is observed in the majority of cases. Within 5 years of diagnosis, an estimated 90% of cases meet diagnostic criteria for mental retardation. Risk factors for severe mental retardation include the following: younger age of onset ( 10 years, none had a DNR order. Pain is correlated with suicidal thoughts, and adequate pain management is important in maintaining quality of life.

Evaluation

Locked-In Syndrome. Figure 2 The infarction seen in Figure 1 was caused by a complete occlusion of the basilar artery. The vertebral arteries are smaller than normal (V: right vertebral artery). The carotid arteries are of normal size (C). The white line (open arrowhead) indicates the normal position of the basilar artery, which ends by forming the two posterior cerebral arteries (P: right posterior cerebral artery). A small remnant of the top of the basilar artery (B) remains

after onset. He communicated by eye blinks for ‘‘yes’’ and ‘‘no.’’ He was cognitively intact, and participated in decision making regarding his care. Over several months, he recovered the ability to move one index finger.

Neuropsychology and Psychology of Locked-In Syndrome Neuropsychology: Cognition is normal on neuropsychological testing in patients with locked-in syndrome, if there are no other additional brain lesions. In the first few months after injury, attention may be impaired. Fatigue may be a factor in neuropsychological evaluation, and several shorter evaluations will yield more reliable results than a full evaluation in a single session. The neuropsychologist evaluating a patient with locked-in syndrome should prepare all the testing in advance in a yes/no format, and before any evaluation begins, establish a mutually agreed upon response (i.e.,

Initial evaluation is the same for all patients with acute onset of coma, or impaired level of consciousness: history, physical exam, labwork (kidney function, liver function, blood counts, evaluation for infection, drug testing), and CT scan. CT scans are done first because they are more widely available, and faster, but less sensitive to brainstem lesions than MRI. EEG may be indicated if there is suspicion of nonconvulsive status epilepticus. If a pontine infarction is found, all involved clinicians need to be aware that there is a possibility of consciousness, and attempt to communicate through whatever voluntary movement is present. Impaired level of consciousness may be present in the initial weeks, but attempts at communication should be done frequently.

Treatment Initial management includes maintenance of respiratory function (mechanical ventilation, tracheotomy), treatment of infections (pneumonia, urinary tract infections), prevention of deep vein thrombosis and pulmonary embolism, adequate nutrition/hydration (gastrostomy tube), and prevention of skin breakdown (pressure sores/decubitus ulcers). All staff should be educated about the possibility of consciousness, and methods to establish communication. Rehabilitation should begin as soon as possible in a facility with experience in dealing with patients with impaired levels of alertness. Initial communication with a yes/ no strategy can be augmented with the use of an alphabet board. Infrared eye movement sensors can be used to control computers, and expand the communication abilities of patients with locked-in syndrome. The recovery of any movement that could control a call light, or activate a computer could be facilitated by the use of functional electrical stimulation (FES) or robotic rehabilitation

Lockett v. Ohio (1978)

devices. Electrode grids implanted onto the cortex can control computers, and direct the movement of powered wheelchairs, but at present this is experimental.

Cross References ▶ Guillain–Barre´ Syndrome ▶ Minimally Conscious State

References and Readings Bauby, J. -D. (1996). The diving bell and the butterfly: A memoir of life in death. New York: Vintage. Devuysy, G., et al. (2002). Stroke or transient ischemic attacks with basilar artery stenosis or occlusion: Clinical patterns and outcome. Archives of Neurology, 59, 567–573. Jorgenson, H. S., et al. (1999). What determines good recovery in patients with the most severe strokes? The Copenhagen stroke study. Stroke, 30, 2008–2012. Posner, J. B., Saper, C. B., Schiff, N. D., & Plum, F. (2007). Plum and Posner’s diagnosis of stupor and coma. (4th ed.). New York: Oxford University Press. Schnakers, C., et al. (2008). Cognitive function in the locked-in syndrome. Journal of Neurology, 255(3), 323–330 Smith, E., & Delargy, M. (2005). Locked-in syndrome. BMJ, 330, 406–409.

Lockett v. Ohio (1978) R OBERT L. H EILBRONNER Chicago Neuropsychology Group Chicago, IL, USA

Definition The US Supreme Court ruled in Lockett v. Ohio (1978) that in the case of a defendant facing the death penalty, one is allowed to present any aspect of character or record or any circumstance related to the offense that could create a basis for a lesser sentence than the death penalty. The 8th and 14th Amendments of the Constitution required, in all but the rarest capital cases, that the triers of fact be allowed to consider a number of mitigating factors, both statutory and nonstatutory, before imposing the death penalty. The Court held that the Ohio statute did not permit the type of individualized consideration of mitigating factors required by the Constitution. Despite this decision in favor of presenting mitigating factors, certain arguments have not been allowed to be presented by the

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defendant. Specifically, issues related to the morality of the death penalty and issues related to the execution process, have not been allowed in any court.

Historical Background Sandra Lockett was found guilty of capital murder in Ohio, a state in which she was limited in her ability to introduce mitigating evidence during the sentencing phase of her trial. She challenged the constitutionality of this limitation, in part, arguing that she was deprived the opportunity to fully inform the jury as to those factors that they may have considered that would have returned a penalty other than death. In Lockett v. Ohio (1978), the Court held (in a 6 to 2 vote) that the sentencer could ‘‘. . .not be precluded form considering as a mitigating factor, any aspect of the defendant’s character or record and any circumstances of the offense that the defendant offers as a basis for a sentence of less than death.’’ As a result of the ruling, a defendant in a capital case cannot be limited in the type of mitigation he or she offers during the penalty phase of the trial. Any information regarding the defendant’s background, as a child or as an adult, could be considered relevant. Thus, factors such as a history of childhood trauma (e.g., physical or sexual abuse), verbal abuse, exposure to drugs and alcohol, neglect and abandonment, undiagnosed or misdiagnosed conditions (e.g., mental retardation, emotional disturbance, learning disability, ADHD), gang or cult membership, or witnessing the death of a family member or friend could be considered nonstatutory mitigation. In addition, any circumstances related to the crime could be considered mitigating. Lockett requires defense attorneys and forensic psychological and psychiatric experts to explore all avenues of mitigation because the factors that can be introduced are not limited to those specifically delineated by statute.

Cross References ▶ Atkins v. Virginia ▶ Death Penalty ▶ Mitigating Factors

References and Readings Cunningham, M. D., & Goldstein, A. M. (2003). Sentencing determinations in death penalty cases. In A. Goldstein (Ed.), Handbook of psychology (Vol. 11). Forensic psychology. New Jersey: Wiley. Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press.

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Heilbronner, R. L., & Waller, D. (2008). Neuropsychological consultation in the sentencing phase of capital Cases. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford Press. Lockett v. Ohio, 438 U.S. 586 (1978). Reynolds, C., Price, J. R., & Niland, J. (2003). Applications of neuropsychology in capital felony (death penalty) defense. Journal of Forensic Neuropsychology, 3, 89–123.

Locus Ceruleus G ALYA A BDRAKHMANOVA Virginia Commonwealth University Richmond, VA, USA

Synonyms Blue spot; Nucleus pigmentosus pontis

Definition Locus ceruleus (Latin, ‘‘place, dark blue’’) is a pigmented noradrenergic nucleus located in the dorsorostral pons.

Current Knowledge Locus ceruleus (also spelled locus coeruleus) contains 30,000–35,000 neurons and is made up of four subnuclei: central (largest), anterior, ventral, and posterior dorsal. The locus ceruleus is dark blue in sections, melanin granules inside the pigmented cells of locus ceruleus contribute to its blue color. The neurons of locus ceruleus provide noradrenergic innervations to most regions of the central nervous system. The projections of the locus ceruleus include spinal cord, brain stem, cerebellum, thalamic relay nuclei, amygdala, basal telencephalon, and cortex. The norepinephrine from the locus ceruleus has an excitatory effect on most of the brain, mediating arousal and attention processing. The locus ceruleus is targeted by several endogenous opioid peptides, e.g. enkephalin, excitatory aminoacids, and stress-related peptides including corticotropin-releasing hormone. The locus ceruleus receives afferents from the hypothalamus including recently discovered orexin/hypocretin inputs, cerebellum, raphe nuclei, medial prefrontal cortex, nucleus paragigantocellularis and nucleus prepositus

hypoglossi. The cingulate gyrus and the amygdala also innervate the locus ceruleus, allowing emotional pain and stressors to trigger noradrenergic responses. Serotonin and histamine fibers innervations of locus ceruleus have been also described, the latter originates in the tuberomammilary nucleus. Locus ceruleus is responsible for physiological responses to stress and panic, and is involved in the mechanisms of clinical depression and mood disorders, panic disorder, and anxiety. Locus ceruleus is activated by stress, and responds by increasing norepinephrine secretion, which in turn increases cognitive function (through the prefrontal cortex), increases motivation (through the nucleus accumbens), activates the hypothalamicpituitary-adrenal axis, and increases the sympathetic discharge/inhibits parasympathetic tone (through the brainstem). Enhanced noradrenergic postsynaptic responsiveness in the neuronal pathway that originates in the locus ceruleus and ends in the basolateral nuclei of the amygdala is a major factor in the pathophysiology of most stress-induced fear-circuitry disorders and especially of posttraumatic stress disorder (PTSD). Implications of locus ceruleus in cognition, attention, and memory appear to be of particular interest for understanding such psychiatric disorders as attention deficit hyperactivity disorder (ADHD), Parkinson’s and Alzheimer’s diseases, schizophrenia, and dementia. Locus ceruleus is also involved in the rapid eye movement (REM) stage of sleep and is studied in relation to sleep disorders with a special attention given to regulation of locus ceruleus neurons by hypothalamic neuropeptide hypocretin. A number of behavioral studies indicated a critical role of noradrenergic neurons of the locus ceruleus in relapse and vulnerability to opiate abuse.

Cross References ▶ Arousal ▶ Noradrenaline ▶ Stress

References and Readings Aston-Jones, G., & Cohen, J. D. (2005). An integrative theory of locus coeruleus-norepinephrine function: adaptive gain and optimal performance. Annu Rev Neurosci, 28, 403–450. Berridge, C. W. & Waterhouse, B. D. (2003). The locus coeruleusnoradrenergic system: Modulation of behavioural and statedependent cognitive processes. Brain Research: Brain Research Reviews, 42, 33–84.

Loewenstein Occupational Therapy Cognitive Assessment Ordway, G. A., Schwartz, M. A., & Frazer, A. (2007). Brain norepinephrine: Neurobiology and therapeutics. Cambridge: Cambridge University Press. Van Bockstaele, E. J., Reyes, B. A., & Valentino, R. J. (2010). The locus coeruleus: A key nucleus where stress and opioids intersect to mediate vulnerability to opiate abuse. Brain Research, 1314, 162–174.

Loewenstein Occupational Therapy Cognitive Assessment M ICHELLE M. T IPTON -B URTON Santa Clara Valley Medical Center San Jose, CA, USA

Synonyms LOTCA

Description The Loewenstein Occupational Therapy Cognitive Assessment (LOTCA) battery – second edition provides an initial profile of the cognitive abilities of individuals with traumatic brain injury (TBI), stroke, or other conditions causing cognitive impairment. The tool was developed in Israel; however, it is frequently used in the USA. The battery takes 30–45 min to administer. The current edition (second) is divided into six main areas: (1) orientation, (2) visual perception, (3) spatial perception, (4) motor praxis, (5) visuomotor organization, and (6) thinking operations. The battery contains 26 subtests including the Risk Object Classification (ROC) (Williams Riska & Allen, 1985), which was added to enhance the evaluation of the categorization operation. This revised edition includes four major changes from the original version that were based on clinical experience and research results. Changes and additions were made to the following categories: orientation, spatial perception and motor praxis, categorization, and logic questions.

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injury or dysfunction. Since its introduction in 1989, it has been widely used by occupational therapists with varied populations. Although the LOTCA was originally developed for clients with brain damage (TBI, stroke, and brain tumors), it is also suitable for assessment in other populations where cognitive status has to be established (spinal cord injured, degenerative and psychiatric diseases, and underachieving students).

Psychometric Data Research data on reliability and validity of the LOTCA battery, which was established on adults with brain damage compared to normals, also provided data on the performance of children in the age range of 6–12 years that verifies the hierarchical order of acquiring the cognitive competencies tested in the battery. Inter-rater reliability was assessed in two ways prior to data collection. Spearman’s rank correlation coefficient showed interrater reliability ranging from 0.82 to 0.97 for the various subtests. Second, a video recording of the assessment of one client was viewed and scored by six therapists. Agreement reached 100% for 14 subtests, 86% for four subtests, and 72% for one subtest, equaling an 86% level of agreement. Internal consistency reliability – Cronbach’s alpha coefficient was calculated for three areas included in the battery. An alpha coefficient of 0.87 was found for ‘‘perception’’ consisting of five subtests (object identification, shape identification, overlapping figures, object constancy, and spatial perception). An alpha coefficient of 0.95 was calculated for ‘‘visuomotor organization’’ consisting of eight subtests (praxis, copying geometric forms, reproduction of models, constructing a pegboard design, constructing a colored block design, constructing a plain block design, reproduction of a puzzle, and drawing a clock). The third area, ‘‘thinking operations,’’ consisting of five subtests (categorization, structured and unstructured ROC classifications, pictorial sequence, and geometrical sequence) showed an alpha coefficient of 0.85. (At the time of study, only the first pictorial sequence was included.) Correlation coefficients range from 0.40 to 0.80 among the subtests suggest that they are not all equivalent, and it is therefore expedient to retain all parts of the battery.

Historical Background Clinical Uses The LOTCA was developed by the staff members at the Department of Occupational Therapy at Loewenstein Rehabilitation Hospital (LRH) in Israel for the purpose of evaluating basic cognitive abilities in persons with brain

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The LOTCA offers a quick, relatively simple battery that is useful in identifying cognitive dysfunction and also provides a profile of the client’s cognitive status useful for establishing

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a baseline for treatment, planning treatment goals, and monitoring cognitive/functional changes during treatment. It can provide some groundwork for the neuropsychologist’s assessment. The test battery is based on Loewenstein’s occupational therapists’ clinical experience as well as on cognitive neuropsychology and developmental theories (Luria, 1980; Golden, 1984; Inhelder & Piaget, 1964).

Cross References ▶ Occupational Therapy

References and Readings Cermak, S. A., Katz, N., McGuire, E., Greenbaum, S., Peralta, C., & Maser-Flanagan, V. (1995). Performance of Americans and Israelis with cerebrovascular accident on the Lowenstein Occupational Therapy Cognitive Assessment (LOTCA). American Journal of Occupational Therapy, 49(6), 500–506. Katz, N., Itzkovich, M., Averbuch, S., & Elazar, B. (1989). Lowenstein Occupational Therapy Cognitive Assessment (LOTCA) battery for brain-injured patients: reliability and validity. American Journal of Occupational Therapy, 43(3), 184–192. Su, C. Y., Chen, W. L., Tsai, P. C., Tsai, C. X., & Su, W. L. (2007). Psychometric properties of the Loewenstein Occupational Therapy Cognitive Assessment –2nd Edition in Taiwanese persons with schizophrenia. American Journal of Occupational Therapy, 61(1), 108–118.

Logical Reasoning ▶ Abstract Reasoning

rate of verbal output, grammatically simple but correct speech, and preserved comprehension. Patients with logopenic aphasia differ from those characterized as ‘‘nonfluent’’ by better conversational speech, and from those characterized as ‘‘semantic’’ by preserved comprehension.

Categorization The term logopenic aphasia has been in the literature for many years, but was first operationalized by GornoTempini (Gorno-Tempini et al., 2004), who proposed a classification system based on presenting language symptoms, distinctive patterns of brain atrophy and genetic factors. The logopenic subtype is defined as having the following characteristics: 1. Lowest score on tests of syntactic comprehension 2. Impaired naming performance (first percentile), but with preserved recognition 3. Impaired repetition 4. Impaired single word reading 5. Intermediate performance on fluency tests 6. Intact single word comprehension 7. Intact semantic associations This term was later refined and described as a subtype of primary progressive aphasia (PPA) (Rogalski and Mesulam, 2007) with the following clinical presentation: word-finding pauses (patient may be nonfluent when asked to describe specific information); fluent speech during small talk (often involving circumlocutions); impaired object naming (usually); and relatively preserved speech syntax and single word comprehension. As with the other subtypes of PPA, additional diagnostic criteria include:

Primary progressive aphasia

1. Presence of a language disorder as characterized by one or more of the following: word-finding deficits that cannot be attributed to dysarthria; object naming impairments; poor syntax in spoken or written language; and spelling errors. 2. Evidence of indolent progression. 3. Relative preservation of memory for recent events, familiar face and object recognition, behavior and basic personality for approximately the first 2 years. 4. Imaging evidence that the cause is not a spaceoccupying or cerebrovascular lesion.


Epidemiology

The term logopenic aphasia refers to a type of progressive aphasia characterized by word-finding problems and slowed

Of all the progressive aphasia subtypes, the logopenic variant is the most likely to be associated with Alzheimer’s

Logopenic Aphasia N ANCY J OHNSON , B ETH B OROSH Northwestern Feinberg School of Medicine Chicago, IL, USA

Synonyms

Logopenic Aphasia

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Logopenic Aphasia. Table 1 Common tests used to evaluate language areas Skill tested

Description of LPA

Comparison

Fluency

Slowed speech, correct syntax, frequent word finding pauses

NFPA < LPA < SD

BDAE-31 Conversational and Expository Speech: 7 different discourse elements are scored. WAB-R2 Spontaneous Speech: Information content and fluency each scored on a 0–10 point scale. Motor

Dysarthria uncommon; if apraxia is present usually mild

NFPA < LPA < SD

Motor Speech Evaluation (Wertz et al., 1984): Dysarthria and apraxia of speech scored on a 1–7 points scale on different tasks. Apraxia Battery for Adults-2 (Dabul, 2000): Six subtests measuring both limb and oral apraxia, and articulation. Auditory Comprehension Word

SD < LPA < NFPA

Generally intact

BDAE-3 Word Comprehension: 16 items (short forms) or 37 items (standard form) pictured on cards. Extended testing includes 10 items in each of the following groups: foods, tools, and animals. WAB-R Auditory Word Recognition: 60 items (real and pictured) broken into 10 categories. Sentence

Impaired for syntactically complex information

LPA < NPFA < SD

BDAE-3 Complex Ideational Material: Yes/No questions using syntactically complex sentence structure. WAB-R Commands: 11 items of progressively longer and more complex grammatical structure. Reading

Impaired oral reading of both regular and irregular words

LPA = NPFA = SD

3

PALPA Regularity and Reading subtest: Reading aloud of regular, exceptional, and non-words. BDAE-3 Oral Reading: Standard form is 10 regular words; extended battery includes 12 mixed morphological type words (e.g., noisier) and 12 ‘‘semantic paralexia-prone’’ words (e.g., loyalty). Naming

Impaired naming, but recognition generally intact

SD < LPA < NPFA

Boston Naming Test (Kaplan et al., 2001): 15 items (short form) or 60 items (standard) ranging from easiest to most difficult; second edition includes a multiple choice recognition. NPFA < LPA < SD

Repetition WAB-R Repetition: 15 items ranging from words to complex sentences. BDAE-3 Repetition: 3 subtests including single words, nonsense words, and sentences. 1

Boston Diagnostic Aphasia Examination (BDAE-3; (Goodglass et al., 2001)) Western Aphasia Battery Revised (WAB-R; (Kertesz, 2007)) 3 Psycholinguistic Assessments of Language Processing in Aphasia (PALPA; (Kay et al., 1992)) 2

disease pathology (Josephs et al., 2008; Kertesz et al., 2005; Mesulam et al., 2008), although the next most common pathology is frontotemporal lobar degeneration (FTLD) with tau-negative pathology but ubiquitin containing inclusions. Genetically, this variant has the highest rate of APOE e4 allele (a known risk factor for Alzheimer’s disease) compared to nonfluent and semantic types (Gorno-Tempini et al., 2004).

course of logopenic progressive aphasia (LPA) and Table 1 lists common language tests by domain as well as the expected performance of LPA patients compared to that of patients with other forms of progressive aphasia (i.e., semantic dementia [SD] and nonfluent progressive aphasia [NFPA]).

Evaluation Neuropsychology and Psychology of Logopenic Aphasia A thorough evaluation of all facets of speech and language will help to differentiate the logopenic subtype from other forms of progressive aphasia. Motor speech, grammar, and single word comprehension are spared early in the

In general, patients with any subtype of progressive aphasia are most likely to show anatomical involvement of the left hemisphere. Using voxel-based morphometry, logopenic patients showed significant atrophy in the posterior portion of the left middle and superior temporal gyrus and the inferior parietal compared to control subjects (Gorno-Tempini et al., 2008). In another study

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comparing LPA to other aphasia subtypes, additional areas of relatively greater atrophy were also found in the left anterior hippocampus and precuneus regions (Gorno-Tempini et al., 2004). Compared to other subtypes of progressive aphasia and the behavioral variant of frontotemporal dementia, the logopenic subtype has been shown to have slightly greater annualized brain volume loss (Knopman et al., 2009) and greater annualized decline on the mini mental state examination (Knopman et al., 2008).

Treatment Patients with logopenic aphasia are more likely to be treated with cholinesterase inhibitors than other aphasia subtypes (Gorno-Tempini et al., 2008), possibly because of the increased finding of Alzheimer’s disease pathology. However, there are no medications specifically approved for treating any type of progressive aphasia, and there have been no large-scale clinical trials using any known pharmacologic agents. Of the two small clinical trials conducted, neither one characterized the progressive aphasia patients by subtype. A trial of bromocriptine was negative (Reed et al., 2004), but a trend for a significant treatment effect was found in PPA subjects treated with galantamine for 8-weeks compared to placebo (Kertesz et al., 2008).

Cross References ▶ Frontotemporal Lobar Degenerations ▶ Progressive Aphasia

References and Readings Dabul, B. (2000). Apraxia Battery for Adults. Shreveport: Dysphasia Plus. Goodglass, H., Kaplan, E., & Barresi, B. (2001). The Boston diagnostic aphasia examination-third edition. Baltimore: Lippincott Williams & Wilkins. Gorno-Tempini, M., Dronkers, N., Rankin, K., Ogar, J., La Phengrasamy, B., Rosen, H., et al. (2004). Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology, 55, 335–346. Gorno-Tempini, M. L., Brambati, S. M., Ginex, V., Ogar, J., Dronkers, N. F., Marcone, A., et al. (2008). The logopenic/phonological variant of primary progressive aphasia. Neurology, 71(16), 1227–1234. Josephs, K. A., Whitwell, J. L., Duffy, J. R., Vanvoorst, W. A., Strand, E. A., Hu, W. T., et al. (2008). Progressive aphasia secondary to Alzheimer disease vs FTLD pathology. Neurology, 70(1), 25–34. Kaplan, E., Goodglass, H., & Weintraub, S. (2001). Boston naming test second edition. Philadelphia: Lippincott Williams & Wilkins.

Kay, J., Lesser, R., & Coltheart, M. (1992). PALPA: Psycholinguistic Assessment of Language Processing in Aphasia. Hove, UK: Psychology Press. Kertesz, A. (2007). Western aphasia battery – revised. Bloomington, MN: Pearson. Kertesz, A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. (2005). The evolution and pathology of frontotemporal dementia. Brain, 128, 1996–2005. Kertesz, A., Morlog, D., Light, M., Blair, M., Davidson, W., Jesso, S., et al. (2008). Galantamine in frontotemporal dementia and primary progressive aphasia. Dementia and Geriatric Cognitive Disorders, 25(2), 178–185. Knopman, D. S., Jack, C. R., Jr., Kramer, J. H., Boeve, B. F., Caselli, R. J., Graff-Radford, N. R., et al. (2009). Brain and ventricular volumetric changes in frontotemporal lobar degeneration over 1 year. Neurology, 72(21), 1843–1849. Knopman, D. S., Kramer, J. H., Boeve, B. F., Caselli, R. J., Graff-Radford, N. R., Mendez, M. F., et al. (2008). Development of methodology for conducting clinical trials in frontotemporal lobar degeneration. Brain, 131(Pt 11), 2957–2968. Mesulam, M., Wicklund, A., Johnson, N., Rogalski, E., Leger, G. C., Rademaker, A., et al. (2008). Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Annals of Neurology, 63(6), 709–719. Reed, D., Johnson, N., Thompson, C., Weintraub, S., & Mesulam, M. (2004). A clinical trial of bromocriptine for treatment of Primary Progressive Aphasia. Annals of Neurology, 56(5), 750. Rogalski, E., & Mesulam, M. (2007). An update on primary progressive aphasia. Current Neurology and Neuroscience Reports, 7(5), 388–392. Wertz, R., LaPointe, L., & Rosenbek, J. (1984). Apraxia of speech: The disorders and its management. New York: Grune and Stratton.

Logorrhea S ARAH B UCKINGHAM The University of Oklahoma Health Sciences Center Oklahoma, OK, USA

Synonyms Press of speech

Definition Logorrhea means excessive verbal production; it is manifested as an unusual verbosity that may suggest the presence of neurological or psychiatric pathologies. Logorrhea is frequently reported as a symptom of Wernicke’s aphasia, where damage to the posterior language cortex yields reduced verbal self-monitoring and a press for speech despite anomia and the consequent absence of meaningful

Lorazepam

linguistic content in spoken utterances (Christman & Buckingham, 1989). Logorrhea in aphasia may be produced with normal prosody and a normal or slightly fast speech rate, and it may co-occur with neologistic jargon (Hallowell & Chapey, 2008). Logorrhea has also been reported as a symptom of mania in bipolar disorder and as a symptom of chronic speech catatonia syndrome (Lee, 2004).

Cross References ▶ Catatonic Behavior ▶ Jargon ▶ Wernicke’s Aphasia

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Brand Name Ativan

Class Anxiolytic

Proposed Mechanism(s) of Action Binds to benzodiazepine receptors at the GABA-A ligandgated channel, thus allowing for neuronal hyperpolarization. Benzodiazepines enhance the inhibitory action of GABA via boosted chloride conductance. This inhibition in the cortex may provide lorazepam’s therapeutic benefits in seizure disorders.

References and Readings Christman, S. S. & Buckingham, H. W. (1989). Jargonaphasia. In C. Code (Ed.), The characteristics of aphasia (pp. 111–130). London: Taylor & Francis. Hallowell, B. & Chapey, R. (2008). Introduction to language intervention strategies in adult aphasia. In R. Chapey (Ed.), Language intervention strategies in aphasia and related neurogenic communication disorders (5th ed., pp. 3–19). Baltimore, MD: Lippincott Williams & Wilkins. Lee, J. W. Y. (2004). Chronic ‘speech catatonia’ with constant logorrhea, verbigeration and echolalia successfully treated with lorazepam: A case report. Psychiatry and Clinical Neurosciences, 58(6), 666–668.

Indication Anxiety, anxiety associated with depression, status epilepticus, pre-surgical pre-anesthetic.

Off Label Use Insomnia, muscle spasm, alcohol-induced psychosis, headache, panic disorder, acute mania, acute psychosis, delirium.

Side Effects

Long Tract Sign ▶ Babinksi Reflex

Serious Respiratory depression, hepatic dysfunction (rare), renal dysfunction and blood dyscrasias, grand mal seizures.

Common

Lorazepam J OHN C. C OURTNEY Children’s Hospital of New Orleans New Orleans, LA, USA

Generic Name Lorazepam

Sedation, fatigue, depression, dizziness, memory problems, disinhibition, confusion, ataxia, slurred speech.


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Loss of Consciousness

Additional Information Drug interaction effects: http://www.drugs.com/drug_interactions.html Drug molecule images: http://www.worldofmolecules.com/drugs/ Free drug online and PDA software: www.epocrates.com Gene-based estimate of drug interactions: http://mhc.daytondcs. com:8080/cgi bin/ddiD4?ver=4&task=getDrugList Pill identification: http://www.drugs.com/pill_identification.html

Loss of Consciousness S AMANTHA B ACKHAUS Rehabilitation Hospital of Indiana Indianapolis, Indiana, USA

drug and alcohol overdoses, severe fatigue, central nervous system diseases, diabetic coma, and electrolyte imbalances, among others.

Cross References ▶ Acceleration/Deceleration Injury ▶ Coma ▶ Concussion ▶ Mild Brain Injury ▶ Post-Concussion Disorder (Syndrome) ▶ Sports-Related Concussion ▶ Traumatic Brain Injury

Synonyms


Coma; Unconscious

Freeman, J. R., Barth, J. T., Broshek, D. K., & Plehn, K. (2005). Sports injuries. In J. M. Silver, T. W. McAllister, & S. C. Yudofsky (Eds.), Textbook of traumatic brain injury (pp. 453–476). Arlington, VA: American Psychiatric Publishing, Inc. Kantor, D. (2007). Consciousness-decreased. Retrieved December 26, 2007 from http://www.nlm.nih.gov/medlineplus/ency/article/003202.htm Lucas, J. A. (1998). Traumatic brain injury and postconcussive syndrome. In P. J. Snyder & P. D. Nussbaum (Eds.), Clinical neuropsychology: A pocket handbook for assessment (pp. 243–303). Washington, DC: American Psychological Association. MedicineNet.com (2005). Temporary loss of consciousness: Fainting or syncope. Retrieved December 26, 2007 from http://www.medicinenet. com/fainting/article.htm

Definition Loss of consciousness (LOC) is defined as a significant alteration of mental status involving a lack of responsiveness to people and other environmental stimuli. It results in an interruption of one’s self and surroundings. Examples include being in a coma or comatose state or a temporary loss of consciousness such as a syncope episode or fainting. Prolonged coma is called a vegetative state or persistent vegetative state. The amount of time it takes a person to regain consciousness and awareness of one’s surroundings is often used as an indication of brain injury severity. Individuals who experience LOC may not always be able to state the amount of time they were unconscious. LOC information is often collected in research studies and has been shown to be a good predictor of outcome. When a person cannot remember the scene or may have post-traumatic amnesia for the event, it is sometimes advisable to obtain information from eyewitnesses of the accident or injury. However, this too can be disputable at times if witnesses to trauma arrive late on the scene.

Loss of Psychic Self-activation ▶ Abulia

LOTCA ▶ Loewenstein Assessment

Occupational

Therapy

Current Knowledge Causes Causes can include traumatic brain injury, hypoxia, stroke, cardiac arrest, severe poisoning secondary to

Lou Gehrig’s Disease ▶ Amyotrophic Lateral Sclerosis

Cognitive

L-Tryptophan

Low-Grade Astrocytoma (LGA) ▶ Fibrillary Astrocytoma

Low-Grade Glioma ▶ Fibrillary Astrocytoma

L-Tryptophan M ARLA S ANZONE Independent Practice Annapolis, MD, USA

Synonyms 5-HTP; 5-hydroxytryptophan; Trp; Tryptophan; W

Indications and Definition The term is sometimes used interchangeably with the term ‘‘tryptophan’’ and abbreviated ‘‘Trp’’ or ‘‘W.’’ L-Tryptophan is one of nine essential amino acids available in most protein-based foods. Essential amino acids must be derived from food or supplements. Nonessential amino acids can be synthesized from essential or other nonessential amino acids. During digestion, L-Tryptophan is broken down by intestinal bacteria that cause the odor associated with fecal material. The L-isomer from tryptophan contains the organic structural heterocyclic, indole, distinguishing it from tryptophan’s D-isomer. The L-isomer comprises the structure of the protein (IUPAC-IUB-JCBN, 1983; L-Tryptophan, 2009). L-Tryptophan can be synthetically and organically derived. Synthetically, it is formulated from the bacterium, Escherichia coli, and the fermentation of serine, a nonessential organic polar amino acid, and indole. Tryptophan-synthase then converts this to tryptophan.

Mechanisms of Action L-Tryptophan

is the biochemical precursor for many compounds, including serotonin, melatonin, and niacin. It is converted to 5-HTP by the enzyme, tryptophan

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hydroxylase. 5-HTP (oxitriptan), the intermediary between tryptophan and serotonin, is decarboxylated to 5-hydroxytryptamine (5-HT) or serotonin via aromatic-L-amino acid decarboxylase. The vitamin B6 assists this conversion. 5-HTP is psychoactive and readily crosses the blood brain barrier (BBB), whereas 5-HT (serotonin) does not. 5-HTP and five other amino acids (tyrosine, phenylalanine, valine, leucine, and isoleucine) bind to transport molecules to cross the BBB. High levels of the 5-HTP are believed to increase the synthesis of serotonin in the central nervous system. This increases the release and bioavailability of serotonin from the raphe nuclei (Sandyk, 1992; Turner et al., 2006; Van Praag, 1983). Melatonin is released from the pineal gland as a function of the degradation of serotonin. The enzyme, N-acetyl-transferase, metabolizes serotonin into N-acetyl5-HT, which is then methylated by 5-hydroxyindoleO-methyltransferase into melatonin. L-tryptophan is also a precursor of niacin synthesis in the kynurenine pathway. N-formyl-kynurenine is converted to kynurenine via formamidase. Kynurenine pathway metabolism produces 2-amino-3-(3-oxoprop-1enyl)-fumaric acid, which undergoes nonenzymatic cyclization, resulting in the production of niacin via the quinolinate pathway (L-Tryptophan, 2009; Tryptophan, 2003).

Specific Compounds and Properties The chemical formula of L-tryptophan is C11H12N2O2. Its chemical weight is 204.2252 (Metabolomics Toolbox, 2009).

Clinical Use The metabolite of tryptophan, 5-Hydroxytryptophan (5-HTP), is occasionally used in the prescription form, Tryptan, to augment antidepressant medication with treatment-resistant affective disorders. The non-pharmaceutical form of 5-HTP is sold as a dietary supplement in health food stores. It is marketed for the treatment of numerous conditions from suppressing appetite and inducing sleep, to premenstrual dysphoric disorder, epilepsy, dementia, and depression. A 2001 meta-analysis suggested that its utility as an antidepressant, sleep aid, and appetite suppression was not sufficiently demonstrated by adequate research methodologies to be considered efficacious. Research is lacking regarding the efficacy

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of tryptophan to treat epilepsy or dementia. The studies that have been completed have been inconclusive (5-Hydroxytryptophan, 2009; IUPAC-IUB-JCBN, 1983; L-Tryptophan, 2009). Research investigating the use of tryptophan for the symptoms of other conditions associated with low serotonin levels such as depression, seasonal affective disorder, and premenstrual dysphoric disorders has shown some promise, but more data are needed (L-Tryptophan, 2009; Sandyk, 1992; Turner et al., 2006; Van Praag, 1983). Tryptophan or 5-HTP is not regulated by the Food and Drug Administration (FDA), rendering standardization and research matters left to individual makers of the products. The quality and labeling accuracy of dietary and nutritional supplements vary considerably as a result (L-Tryptophan, 2009).

suggested it may decrease irritability, aggression, and movement disturbances associated with Parkinson’s disease and dementia. However, other investigations raised concern that this combination may increase the risk of symptoms associated with the autoimmune disorder, scleroderma (5-Hydroxytryptophan, 2009; South, 2009). Tryptophan ingested via typical dietary routes does not cause side effects. Tryptophan supplementation has been associated with difficulty breathing, hives, itching, rash, swelling of the mouth or throat, and wheezing. Potentially serious adverse reactions include serotonin syndrome and eosinophilia-myalgia syndrome (EMS), particularly if individuals are taking other serotonin enhancing products (Monson and Schoenstadt, 2008).

Dietary Sources Tryptophan Deficiency In developed countries, tryptophan deficiency is not common, but can occur among individuals with poor overall protein intake. A deficiency of tryptophan can lead to Vitamin B3 deficiency and has been suggested in depression, insomnia, schizophrenia, suicidal thoughts, and carbohydrate craving. It has also been implicated as a possible factor when magnesium is also deficient, in cases involving heart artery spasms (Vitamin B3, 2006). Tryptophan is rapidly metabolized into the toxic metabolites hydroxykynurenine, xanthurenic acid, and hydroxyanthranilic acid in individuals deficient in Vitamin B6, reducing to less than 1% the bioavailability of ingested tryptophan to the brain. This can have considerable negative effects on energy, mood, sleep, and other functions thought to be mediated at least in part by tryptophan (L-Tryptophan, 2009). Insufficient levels of tryptophan are also seen in Fructose Malabsorption, also called Dietary Fructose Intolerance. Fructose malabsorption is a digestive disorder of the small intestine whereby tryptophan cannot be adequately absorbed by intestinal tissue, resulting in excess levels of intestinal fructose. It has been estimated that 30–40% of the population in parts of central Europe are affected by Fructose malabsorption (Fructose malabsorption, 2009; L-Tryptophan, 2009).

The most abundant sources of dietary tryptophan, a common constituent of most protein-based foods, are bananas, chick peas, chocolate, cottage cheese, dates, eggs, fish, mangoes, red meat, milk casein, oats, peanuts, poultry, pumpkin seeds, sunflower seeds, and yogurt (L-Tryptophan, 2009). However, of all 22 amino acids, tryptophan is the least available. In a typical proteinbased animal or vegetarian diet, only about 1–1.5 g of tryptophan per day is ingested. Turkey is not a significantly better source of tryptophan than other poultry. However, it is commonly perceived to be the source of drowsiness post the typical American Thanksgiving dinner. The turkey dinner– tryptophan connection has its origins in the science of carbohydrate metabolism. When foods rich in tryptophan are consumed as part of a high-carbohydrate meal, the insulin released stimulates muscle tissue cells to uptake branched-chain large neutral amino acids (LNAA). This increases the ratio of tryptophan to LNAA which decreases competition for binding to the LNAA transporter. Relatively higher levels of tryptophan bind to the transporter molecule and cross the BBB where the conversion to serotonin then melatonin occurs. Higher levels of serotonin and melatonin induce the sleepiness attributed to tryptophan in turkey (L-Tryptophan, 2009).

History Side Effects Research looking at the efficacy of 5-HTP to increase plasma serotonin levels when coadministered with a peripheral decarboxylase inhibitor such as carbidopa

Sir Frederick Hopkins in 1901 is credited with isolating the tryptophan molecule via casein hydrolysis. The USA banned the use of tryptophan as a dietary supplement in 1991 following a 1989 outbreak of the autoimmune

Lumbar Puncture

condition, eosinophilia myalgia syndrome (EMS). At least 37 died, and 1,500 were disabled following ingestion of L-tryptophan hypothesized to have contained trace impurities. The impurities were initially thought to have resulted from the methods of genetic engineering used by the Japanese supplier to synthesize the bacteria that produces L-tryptophan. Other epidemiological studies suggested that L-tryptophan metabolites produced during digestion as a function of normal histamine metabolism were inhibited. As a result, the ingestion of large doses of tryptophan was thought to cause excessive levels of histamine, increasing the susceptibility to EMS in vulnerable individuals (L-Tryptophan, 2009). Despite remediation of purification procedures, the FDA maintained the ban on the sale of L-tryptophan, until 2001. In 2002, the dietary supplement, L-tryptophan, was sold again in the USA as a result of reduced marketing restrictions. The FDA maintained importation bans based on concerns that the causal factors for the EMS outbreak were not yet fully understood. The prescription form of tryptophan, Tryptan, remained on the market (L-Tryptophan, 2009).

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South, J. (2009). L-Tryptophan, nature’s answer to Prozac. In Smart Nutrition - International Antiaging Systems. Retrieved February 2, 2009, from http://www.smartnutrition.info/JamesSouth-tryptophan.htm. Turner, E. H., Loftis, J. M., & Blackwell, A. D. (2006). Serotonin a la carte: Supplementation with the serotonin precursor 5-hydroxytryptophan. Pharmacology & Therapeutics, 109(3), 325–338. Tryptophan. (2003). In MedicineNet. Retrieved February 2, 2009, from http://www.medterms.com/tryptophan. Van Praag, H. M. (1983). In search of the action of antidepressants, 5HTP, tyrosine mixtures in depression. Neuropharmacology, 22, 433–440. Vitamin B3. (2006). Vitamin B3 (niacin, nicotinic acid, nicotinamide) deficiency. In Vitamins & Health Supplements Guide. Retrieved February 3, 2009, from http://www.vitamins-supplements.org/niacindeficiency.php.

Lumbar Puncture PAUL E. K APLAN Capitol Clinical Neuroscience Folsom, CA, USA

Synonyms Cross References ▶ 5-HTP ▶ 5-Hydroxytryptophan ▶ Serotonin ▶ Serotonin Syndrome ▶ Tryptophan

References and Readings 5-Hydroxytryptophan. (2009). In Wikipedia. Retrieved February 3, 2009, from http://en.wikipedia.org/wiki/5-HTP. Fructose malabsorption. (2009). In Wikipedia. Retrieved February 3, 2009, from http://en.wikipedia.org/wiki/fructosemalabsorption. (IUPAC-IUB) (JCBN) International Union of Pure & Applied Chemistry & International Union of Biochemistry & Molecular (JCBN) Joint Commission on Biochemical Nomenclature. (1983). Nomenclature and symbolism for amino acids and peptides. In Recommendations on Organic & Biochemical Nomenclature, Symbols & Terminology. Retrieved February 3, 2009, from http://www.chem.qmul.ac.uk/iupac/AminoAcid. L-Tryptophan. (2009). In Wikipedia. Retrieved February 2, 2009, from http://en.wikipedia.org/wiki/Tryptophan. Metabolomics Toolbox. (2009). In Human Metabolome Database, Retrieved January 19, 2009, from http://hmdb.ca/scripts/show_card. cgi?METABOCARD = HMDB00216.txt. Monson, K., & Schoenstadt, A. (2008). In eMedTV. Retrieved February 2, 2009, from http://insomnia.emedtv.com/tryptophan/tryptophanside-effects.html. Sandyk, R. (1992). L-Tryptophan in neuro-psychiatric disorders: A review. International Journal of Neuroscience, 67, 24–144.

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Spinal puncture; Spinal tap

Definition Lumbar usually refers to that part of the back or spine between the ribs and the pelvis. Lumbar puncture used to be called a spinal puncture or in common parlance a ‘‘spinal tap.’’ Before CT scans were invented, the majority of the ‘‘Neurological’’ exam would have consisted of a two or three hour history and physical, appropriate X-rays, and a lumbar puncture. Lumbar punctures could generate headaches, infections, or worse. With the advent of the much less invasive CT and MRI brain scans, the importance of the lumbar puncture as a diagnostic or treatment tool has greatly declined. It is now especially used for the evaluation of infectious or immunologic agents. It also has been used to evaluate hydrocephalus, but its use needs to be with extreme caution in that case. In multiple sclerosis, leukocytosis, and abnormal immunoglobulin, production will be noted.

Current Knowledge Spinal taps used to be a standard portion of the neurological evaluation. They are not performed nearly as frequently as in the past and only for special reasons.

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Lumboperitoneal Shunt (LP Shunt)

Cross References

Definition

▶ Encephalopathy ▶ Hydrocephalus ▶ Multiple Sclerosis

Lupus cerebritis is an important complication of systemic lupus erythematosus, characterized by cerebral vasculitis or inflammation of the blood vessels in the brain. This may result in stroke or other symptoms including headaches, seizures, psychosis, dementia, or peripheral neuropathy. Stroke occurs in 5–20% of all patients with systemic lupus. It is treated by the control of systemic lupus and by the use of anti-inflammatory medications such as steroids.

References and Readings Freeman Multiple Sclerosis, Thompson, E. J., Deisenhammer, F., et al. (2005). Recommended standard of cerebrospinal fluid analysis in the diagnosis of multiple sclerosis: A consensus statement. Archives of Neurology, 62, 865–870. Quality Standards Subcommittee of the American Academy of Neurology. (1992). Practice parameter: Lumbar puncture. American Academy of Neurology, 149–155.

Cross References ▶ Cerebral Angiitis ▶ Collagen Vascular Disease ▶ Vasculitis

Lumboperitoneal Shunt (LP Shunt)


▶ Shunts

Luminal Sodium

Jennekens, F. G., & Kater, L. (2002). The central nervous system in systemic lupus erythematosus. Part 1. Clinical syndromes: A literature investigation. Rheumatology (Oxford), 41(6), 605–618. Muscal, E., & Brey, R. L. (2010). Neurologic manifestations of systemic lupus erythematosus in children and adults. Neurologic Clinics, 28, 61–73.

▶ Phenobarbital

Luria, Alexander Romanovich (1902–1977)

Lupus ▶ Systemic Lupus Eythematosus (SLE)

B ENJAMIN H AMPSTEAD, A KSHAY A MARANENI Emory University/Rehabilitation Medicine Atlanta, GA, USA

Lupus Cerebritis Major Appointments E LLIOT J. R OTH Northwestern University Chicago, IL, USA

Synonyms

CNS lupus

1921–1923: Laboratory Assistant, Kazan Institute for the Scientific Organization of Labor, Kazan, Russia. 1923–1930: Staff professor and researcher, Institute of Psychology in Moscow, Moscow, Russia. 1930–1934: Head of Psychology department, Psychoneurological Institute at Kharkov University, Kharkov, Ukraine.


1934–1936: Research, Moscow Institute of Genetics; Department head, Department of Clinical Psychology, Institute of Experimental Medicine, Moscow, Russia. 1939–1941: Head of the Laboratory of Experimental Psychology, Neurological Clinic of the Institute of Medicine (later called the Neurological Institute of the Academy of Medical Sciences), Moscow, Russia. 1941–1945: Commissioned to organize and run a hospital for soldiers during World War II, Cheliabensk, Russia. 1952–1958: Research Staff, Institute of Defectology, Moscow, Russia. 1945–1977: Professor of Psychology, Moscow State University, Moscow, Russia. 1945–1977: Head of Department of Neuropsychology, Moscow State University, Moscow, Russia. 1945–1950, 1958–1977: Burdenko Institute of Neurosurgery, Director of Neurosurgery Lab, Moscow, Russia. 1945–1977: Head of the Aphasiological Laboratory, Moscow Medical Institute, Moscow Russia.


Ranked No. 69 on the list of top-100 psychologists of the twentieth century by Review of General Psychology.


The works of prominent psychiatrists such as Freud and Jung largely fueled Alexander Luria’s initial interests in psychological theory. However, Luria was ultimately unsatisfied with the prevailing psychological theories of the time and set out to discover, ‘‘a psychology that would overcome [insufficiencies in knowing the complexity of people], that would simultaneously describe the concrete facts of the mental life of individuals and generate general explanatory laws’’ (Cole, 23). In combination with friend and mentor Lev Semionovitch Vygotsky, Luria ultimately studied psychological processes and cognitive abilities in diverse populations such as children with and without mental retardation and poorly educated rural women. They also performed some of the first studies investigating the role of genetics in cognitive functioning using monozygotic and dizygotic twins. After earning his medical degree, Luria began applying his knowledge of cognitive processes to those who

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had sustained neurological injury, work that was highly influenced by his care of World War II soldiers (see below). Luria’s revolutionary theory of brain–behavior relations utilized many of the strengths of both localization and equipotentiality while also addressing many of their shortcomings. Luria posited the existence of three functional units within the brain, each responsible for a variety of functions necessary for both simple and complex behaviors (or cognitive abilities). The first functional unit encompasses the brain stem and diencephalon and is generally responsible for modulating arousal and filtering sensory input. The second functional unit utilizes cortical areas posterior to the central sulcus (i.e., the parietal, temporal, and occipital cortices) to perceive, integrate, and analyze sensory information. The frontal/ prefrontal cortex comprises the third functional unit and is involved in a variety of cognitive abilities, generally termed executive functions, including planning, decision making, impulse control, and analyzing behaviors. The third unit can also modulate the level of cortical arousal (i.e., the first functional unit) through both direct and indirect processes. Luria believed that all behavior requires the brain to function as a whole, but that each functional unit (or brain region) contributes in a unique way to a given behavior. Obviously, more complex behaviors require a larger functional network. Thus, the role of brain regions can be classified as pluripotential, given the limited or multifaceted contribution of each region to behavior. An especially compelling aspect of Luria’s theory is that it addresses recovery of function after injury. He believed that deficits caused by damage to one unit (or brain region) can, at least in part, be ameliorated by the spontaneous compensation of another area or through specific retraining. His idea of neural-plasticity is profoundly insightful and has been supported through recent functional neuroimaging studies that have shown altered patterns of activation, following various forms of cognitive and physical rehabilitation. These and Luria’s other contributions to the field of Neuropsychology are detailed in his works including: The Nature of Human Conflicts (1932), Higher Cortical Functions in Man (1962/1966), Restoration of Function After Brain Injury (1963), The Mind of a Mnemonist (1968), The Man With a Shattered World (1972), The Working Brain: An Introduction to Neuropsychology (1973), The Neuropsychology of Memory (1976), and The Making of Mind: A Personal Account of Soviet Psychology (1979).

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Short Biography Alexander Romanovich Luria was born in Kazan, Russia on July 16, 1902. His father was a gastroenterologist and his mother was a dentist. Luria moved to Moscow at the age of 15 when his father was appointed vice-director of the Central Institute for Advanced Medical Studies. Luria excelled in school and used his knowledge of Latin as a springboard for learning English, French, and German. He earned a high school diploma in 1918 and entered Kazan University where he participated in many studentrun discussion groups and became interested in man’s role in society. Building on this interest in psychological processes, he read the works of Dilthey, Freud, and Jung. He also read, and translated into Russian, L. Brentano’s Theory of Human Drives. These works were highly influential and increased Luria’s desire to develop a more sound theory of psychological processes. Luria graduated from Kazan University in 1921 and enrolled at both Kazan Psychiatric Hospital and the Pedagogical Institute to study medicine and psychology, respectively. In 1922, Luria formed a small psychoanalytic circle that he named the Kazan Psychoanalytic Association. One of the first things he did as head of the organization was send a letter to Freud, who gave Luria permission to translate one of his books into Russian. After further studying the available literature, Luria became critical of psychoanalysis and felt that it ignored the importance of social experience in cognition. In 1927, he resigned from the Psychoanalytic Society after his colleagues published harsh criticisms of Freud’s work. Despite the fact that his own work conflicted with Freud, Luria refrained from denouncing Freud. At the age of only 19, Luria accepted a position as a laboratory assistant at the Kazan Institute for the Scientific Organization of Labor. Later, he founded a journal called ‘‘Problems of the Psychophysiology of Labor and Reflexology.’’ He later cited these events as significant in his development as a psychologist. Two years later, he secured his first staff appointment at the Moscow Institute of Psychology, where he and his colleagues studied emotional reactions in students preparing to take examinations, work that laid the foundation for an early model of the polygraph. In 1924, Luria’s career would forever change when he met Lev Semionovitch Vygotsky at the Second Psychoneurological Congress in Leningrad. Vygotsky was invited to join the staff at the Institute of Psychology, where Luria, Vygotsky, and Alexei Leontiv would essentially develop the field of cognitive psychology. In 1930, Luria and his colleagues founded a new Department of Psychology at the Psychoneurological Institute of Kharkov University.

During the 1930s, the group began investigating the genetic basis and environmental influences on behavior (and cognitive functioning) by studying monozygotic and dizygotic twins. Using a series of cognitive tests, the group investigated the processes by which information is learned and remembered. Their results suggested that individuals increased the use of various cognitive strategies with age and that both the genetics and the environment contributed to cognitive development. Luria returned to medical school in 1936, and a year later, began a 2-year internship in neurology. After completing this training in 1939, he became head of the Laboratory of Experimental Psychology at the Neurological Clinic of the Institute of Medicine where he studied aphasia. In 1941, the Neurological Clinic in the Institute of Experimental Medicine commissioned him to organize a 400-bed hospital for soldiers injured during World War II. He had two major duties: (1) providing medical treatment for those who had sustained brain damage and diagnosing the effects of such injury and (2) developing a scientifically based rehabilitation program. During these experiences, Luria documented several types of aphasia and also implemented specific rehabilitation techniques using preserved abilities to compensate for damaged functions. His experiences during this time contributed significantly toward his theory of cortical functioning, which is described above. In 1950, Luria was forced to leave his appointment at the Institute of Neurosurgery in Moscow when Genetics was labeled a pseudoscience and banned from the Soviet Union. With the threat of arrest, he spent much of the next 8 years at the Institute of Defectology, also in Moscow, where he was essentially demoted and started as part of the research staff. After Stalin died in 1953, accusations against Luria were dropped and he eventually returned to the Institute of Neurosurgery in 1958 where he spent most of his remaining life, continuing to hold appointments at Moscow State University. During this time, he published two prominent case reports: the first documented a patient who seemed to have limitless memory (The Mind of a Mnemonist, 1968) and the second examined the cognitive deficits and gradual rehabilitation of a patient who sustained a bomb-induced lesion of the left parietal lobe (The Man with a Shattered World, 1972). One of Luria’s most complete works was Higher Cortical Functions in Man (1962), in which he presented a series of neuropsychological tests and discussed the process by which he determined cognitive deficits and lesion location. Later in life, Luria followed a routine that involved writing in the morning, lab work in the afternoon and

Luria Nebraska Neuropsychological Battery

evenings with a nap in between. In 1977, his wife, Lana Pimenovna, was diagnosed with an inoperable tumor. Luria dedicated the rest of his time to caregiving and, for this reason, the couple moved to a sanatorium. He died of heart failure while on the telephone trying to obtain a new medication for his wife. She lived another 5 months and, during this time, was able to archive and organize Luria’s memorial office at Moscow State University, where she also donated his personal library. He profoundly influenced many prominent neuropsychologists including Anna-Lise Christensen and Elkohnen Goldberg, who played a large role in introducing Luria to the West.

Cross References ▶ Christensen, Anna-Lise ▶ Equipotentiality

References and Readings Christensen, A. L., Goldberg, E., & Bougakov, D. (2009). Luria’s legacy in the 21st century. New York: Oxford University Press. Cole, M., Levitin, K., & Luria, A. R. (2005). New Jersey: Lawrence Erlbaum Associates, Inc. Publishers. Homskaya, E. D. (2001). Alexander Romanovich Luria: A scientific biography. David Tupper (Ed.). New York: Springer. Luria, A. R. (1966). Higher cortical functions in man. (Basil Haigh, Trans.). (Originally published by Moscow University Press, 1962.) New York: Basic Books. Luria, A. R. (1973). The working brain: An introduction to neuropsychology. New York: Basic Books. Moskovich, L., Bougakov, D., DeFina, P., & Goldberg, E. (2002). A. R. Luria: Pursuing neuropsychology in a swiftly changing society. In A. Stringer, E. Cooley, & A.-L. Christensen (Eds.), Pathways to prominence. New York: Psychology Press. Tupper, D. (1999). Introduction: Alexander Luria’s continuing influence on worldwide neuropsychology. Neuropsychology Review, 9, 1–7.

Luria Nebraska Neuropsychological Battery S HAHAL R OZENBLATT Advanced Psychological Assessment, P.C. Smithtown, NY, USA

Synonyms LNNB; Luria–Nebraska battery

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Description The LNNB, developed by Golden and his colleagues (Golden, Purisch, & Hammeke, 1991), was designed to discriminate patients with neurological impairments from normal individuals. It had a further goal of allowing for the lateralization and localization of neurological impairment. The LNNB consists of two forms: Form I contains 11 scales, and Form II contains 12 scales. The two forms are supposed to serve as parallel versions, with Form II containing a measure that assesses delayed recall of some of the previously administered shortterm memory items. Each item is scored from 0 (no impairment) to 2 (severe impairment). The scales are now relabelled C1–C12, replacing the scale names (e. g., Motor Functions or Rhythm), because the scales are multidetermined, which makes the domain labels less meaningful. Five summary scales have been created using items from the clinical scales. The Pathognomic scale consists of items designed to discriminate patients with neurological impairment from healthy individuals. The Right Hemisphere and Left Hemisphere scales consist of tactile and motor functions designed to lateralize dysfunction. The Profile Elevation and Impairment scales are designed to determine the examinee’s level of functioning and extent of impairment (Lezak, Howieson, & Loring, 2004). Additional scales have been added to the LNNB since it was first developed. These newer scales include eight localization scales, four for each side of the brain (e.g., Frontal & Parietal-Occipital), and 28 factor scales (e.g., four for reading). Another addition to the LNNB is a 66-item list of qualitative aspects of test performance designed to aid the examiner in evaluating the nature of the examinee’s failure on a particular task, not merely to document its occurrence (Lezak, 2004). A short form of the LNNB was developed by McCue et al. (McCue, Shelly, & Goldstein, 1985). The short form was proposed for use with elderly patients. It retained the complete Memory, Intellectual Processes, and Pathognomic scales, dropped the Rhythm scale, and reduced the number of items on all other scales, leaving 141 total items. Differences between the standard and short forms of the LNNB were found with the short form showing lower (i.e., better) scores on the Expressive Language scale and higher (i.e., worse) scores on Reading. The short form was able to correctly identify approximately 75% of Alzheimer patients and 90% of depressed elderly patients, when LNNB age and education corrections were entered into scale calculations.

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Luria Nebraska Neuropsychological Battery

Historical Background The LNNB is based on the work of A. L. Christensen (1979). She took Luria’s examination techniques and test materials, as presented in works such as Higher Cortical Functions in Man (Luria, 1966), and organized them under a single battery. The materials and techniques incorporated into the battery spanned the range of methods that Luria incorporated into his investigations (e.g., higher kinaesthetic and coetaneous functions; 1966). In keeping with the spirit of Lurian investigation, Christensen stressed the need to understand how the patient arrived at a response, while downplaying the importance of standard scores. As with other approaches based on Luria’s theories (e.g., Boston Process Approach; Kaplan, 1990), Christensen emphasized the importance of tailoring the procedures in a manner that will challenge, but not overly frustrate, patients. The main advantage of Christensen’s approach is that it enables actual observation of behavior rather than inferred cognitive processes. The LNNB is inexpensive, flexible, and brief to administer (Lezak, 2004). A number of drawbacks limit the utility of Christensen’s battery. First, the battery lacks tests of attention, concentration, and mental tracking (Lezak et al., 2004). Moreover, there is a dearth of techniques available to assess nonverbal memory and concept formation, and there are no methods for evaluating fund of information. A further limitation is that the techniques incorporated into the battery, while useful in discriminating patients with severe impairments, are not useful in detecting most mild or diffuse deficits. The LNNB was developed, in part, as a way to standardize the items in Christensen’s battery (Golden et al., 1991).

Psychometric Data The norms for Form I of the LNNB were based on 50 subjects (26 women and 24 men) hospitalized for a range of medical conditions (e.g., back injuries, infectious diseases, and chronic pain), with an age range of 42 14.8 years, and 12.2 2.9 years of education. Form II was standardized on 51 normal individuals (Golden et al., 1991). The critical level, which provides the cutting score for the clinical scales, is derived by multiplying the patient’s age by 0.214 for every year between 25 and 70 years.

Correction for education is done by multiplying the years of education (0–20) by 1.47 and subtracting this number from the critical level. A criticism of this approach is that age and education are treated as linear variables, with no accommodation for variation in the impact that these variables can have on different tasks. Furthermore, gender is not accounted for, and research has shown that women tend to underperform men on the Motor and Visual scales (Vannieuwkirk & Galbraith, 1985). Split-half reliabilities are reported to range from 0.89 to 0.95, but as Lezak et al. (2004) commented, ‘‘it seems logically improbable to perform split-half reliability studies on a test in which each item differs from its neighbour. . .’’. Internal consistency is generally in the 0.80 range (Golden, Purisch, & Hammeke, 1991).

Clinical Uses Lezak et al. (2004) summarized the research on the clinical utility of the LNNB. The battery has shown a high level of accuracy in its ability to separate persons who sustained brain injuries from normal control subjects. It is also able to discriminate between chronic psychotic patients and those with neurologic disease, with hit rates of 73–74%. The LNNB does not fair as well when mild conditions are involved (a problem also noted on Christensen’s original battery), and does not identify lesion laterality to a satisfactory degree. Research has demonstrated that the LNNB is not able to differentiate patients with focal lesions from those with diffuse damage. For example, research on patients with multiple sclerosis (MS; Stanley & Howe, 1983) indicated that the patient group performed as well as normal controls on items that were supposed to reveal greater impairment, while the MS group did not perform significantly differently from persons with brain injuries on items on which better performance was expected. Aphasic patients were also studied (Ryan, Farage, Mittenberg, & Kasprisin, 1988), and the LNNB was unable to discriminate between types of aphasic conditions. A survey of battery choice in neuropsychological assessment (Retzlaff, Butler, & Vanderploeg, 1992) indicated that only about 3% of neuropsychologists use the LNNB. This limited use is due in part to the limitations noted above. Purisch (2001) wrote a paper in response to what

Luxury Perfusion

he referred to as misconceptions about the psychometric properties and clinical utility of the LNNB. In his concluding remarks, Purisch wrote, ‘‘Application of the Lurian model to the data permits a deeper understanding for the primary neuropsychological functions that are critical to formulating rational treatments, counselling and guidance, and analyzing components of behavioral functioning within the real world context’’.

Cross References ▶ Boston Process Approach ▶ Halstead–Reitan Neuropsychological Test Battery

References and Readings Christensen, A. L. (1979). Luria’s neuropsychological investigation (2nd ed.). Copenhagen: Munksgaard. Golden, C. J., Purisch, A. D., & Hammeke, T. A. (1991). Luria-Nebraska neuropsychological battery: forms I and II. Los Angeles: Western Psychological Services. Kaplan, E. (1990). The process approach to neuropsychological assessment of psychiatric patients. Journal of Neuropsychiatry, 2, 72–87. Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press. Luria, A. R. (1966). Higher cortical function in man. New York: Basic Books. McCue, M., Shelly, C., & Goldstein, G. (1985). A proposed short form of the Luria-Nebraska Neuropsychological Battery oriented toward assessment of the elderly. International Journal of Clinical Neuropsychology, 7, 96–101. Purisch, A. D. (2001). Misconceptions about the Luria-Nebraska Neuropsychological Battery. NeuroRehabilitation, 16, 275–280. Retzlaff, P., Butler, M., & Vanderploeg, R. D. (1992). Neuropsychological battery choice and theoretical orientation: A multivariate analysis. Journal of Clinical Psychology, 48, 666–672. Ryan, J. J., Farage, C. M., Mittenberg, W., & Kasprisin, A. (1988). Validity of the Luria-Nebraska Language Scales in aphasia. International Journal of Neuroscience, 43, 75–80. Vannieuwkirk, R. R., & Galbraith, G. G. (1985). The relationship of age to performance on the Luria-Nebraska Neuropsychological Battery. Journal of Clinical Psychology, 41, 527–532.

Luria–Nebraska Battery ▶ Luria Nebraska Neuropsychological Battery

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Lurking Variable ▶ Confounding Variables

Luxury Perfusion S USAN L ADLEY-O’B RIEN Denver Health Medical Center Denver, CO, USA

Definition Luxury perfusion is a misnomer used to describe the dilation of numerous vascular channels observed within in the relatively avascular infarcted area of the brain 24–48 h after an ischemic stroke. These are predominantly venous channels, but arterial channels open up as well. At surgery, the infarcted area thus appears to contain an increase in the number of blood vessels. These are not actually functioning to supply the infarcted brain; however, so do not represent true ‘‘perfusion.’’ On a contrasted CT scan, this phenomenon is perceived as an area of enhancement (increased density or whiteness) at the margin of the infarct. This is visible 1 day to several days after a stroke. The term luxury perfusion may also refer to a hyperperfusion syndrome occurring in 0.2% of patients undergoing cerebral endarterectomy. This condition is characterized by ipsilateral headaches, hypertension, seizures, and focal neurologic deficits. The mechanism of ‘‘luxury perfusion syndrome’’ is a severe impairment of cerebral autoregulation and postoperative hypertension, with increased cerebral blood flow to a previously hypoperfused hemisphere. The associated intractable headache usually improves with upright posture. Transcranial Doppler study may demonstrate abrupt increase flow velocities in the middle cerebral artery, and diffuse hyperintensity may be demonstrated transiently on diffusion-weighted MRI in the distribution of the involved arterial territory. This syndrome, if not recognized and treated appropriately, may lead to the development of cerebral edema, with severe morbidity or death.

Cross References ▶ Carotid Endarterectomy

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Lyme Disease

References and Readings Dafer, R. M. (2006). Risk estimates of stroke after coronary artery bypass graft and carotid endarterectomy. Neurologic Clinics, 24(4), 795–806. Mettler, F. A. (2005). Mettler: Essentials of radiology (2nd ed.). Philadelphia: Saunders. Ramsey, R. (1994). Neuroradiology (3rd ed.). Philadelphia: WB Saunders.

Lyme Disease L AUREN B. K RUPP, PATRICIA M ELVILLE SUNY Stony Brook Stony Brook, NY, USA

Synonyms Borreliosis

cerebral spinal fluid (CSF). Syndromes suggestive of persistent infection in treatment naı¨ve patients include recurrent attacks of arthritis, the skin condition acrodermatitis, encephalomyelitis, encephalopathy, or chronic neuropathy. Neurologic Lyme disease is often referred to as neuroborreliosis. The first reported case of neuroborreliosis was in 1922 (Garin & Bujacoux, 1922). Common neurologic manifestations include aseptic meningitis, facial nerve palsy, and radiculopathy. In the past two decades, encephalitis, encephalopathy, and neuropsychological impairments have become a focus of investigation (Broderick, Sandock, & Mertz, 1987; Logigian, Kaplan, & Steere, 1990; Krup et al., 1991a; Kaplan, Meadows, Vincent, Logigian, & Steere, 1992; Fallon et al., 2008). Abnormal cognitive functioning can often improve after antibiotic therapy (Logigian et al., 1990). However, in some patients cognitive deficits may persist for variable durations post treatment (Fallon et al., 2008; Krup et al., 1991b; Benke, Gasse, Hittmair-Delazer, & Schmutzhard, 1995; Bujak, Weinstein, & Dornbush, 1996).

Short Description or Definition Epidemiology Lyme disease, caused by the spirochete Borrelia burgdorferi, is an infection, which in the USA is transmitted via the deer tick, ixodes scapularis. Other tick species transmit the disease in Europe. Typically, 3–30 days after the tick bite, there is an expanding rash, known as erythema migrans (EM). This rash is flat and frequently has a central clear area giving it a ‘‘bull’s eye’’ appearance. The rash constitutes the localized phase of the infection. Unfortunately, some individuals never get the rash or the rash can be missed and the infection disseminates causing late manifestations of multisystem involvement. The case definition of Lyme disease established by the Centers for Disease Control (CDC) is a person with EM or with at least one late manifestation along with laboratory confirmation of the infection.

Categorization of Systemic and Neurological Complications During the localized phase the rash may be accompanied by fatigue and malaise. When the dissemination phase of the disease develops the skin, joints, heart, liver, or neurological system can be involved. If no treatment is given persistent infection can ensue, a complication occurring in approximately 60% of cases. During persistent infection spirochetes can become sequestered in joint fluid or

Lyme disease is the most common tick borne illness in Europe and the USA. Since surveillance began with the CDC there have been an increasing number of reported cases. Over 18,000 cases were reported in the year 2000. In the past several decades, the infection has spread throughout the northeast and now also includes many areas in the mid Atlantic seaboard. Connecticut, the state with the highest frequency, has an estimated incidence of 1/1000.

Natural History Following antibiotic therapy the majority of patients fully recover. Management of persistent infection is more difficult depending on the degree of end-organ damage. In some patients a Post Lyme syndrome (PLS) can develop. PLS is defined as persistent symptoms despite appropriate treatment. A meta-analysis of three studies on these patients concluded that the most common symptoms consist of fatigue, musculoskeletal pain, and memory problems. These symptoms can persist years following antibiotic treatment (Cairns & Godwin, 2005). Many of the symptoms in PLS overlap with those of chronic fatigue syndrome (CFS) (Gaudino, Coyle, & Krupp, 1997).

Lyme Disease

Neuropsychological Findings in Lyme Disease and PLS Memory and other cognitive deficits have been documented in untreated and treated Lyme patients with cognitive symptoms. In one study, untreated Lyme patients with neurological problems were compared to patients with fibromyalgia or depression. A total of 14 of the 27 patients tested had impairments on either the Wechsler Memory scale (WMS), California Verbal Learning Test (CVLT), or Rey Osterrieth Figure test (Logigian et al., 1990; Kaplan et al. 1992). Among 15 PLS patients evaluated a mean of 6.7 months after completing antibiotic therapy, deficits relative to healthy controls were noted on all the subtests of the Selective Reminding Test with the exception of recognition and recall. Deficits were also present on the WMS-revised (WMS-R) and the Controlled Oral Word Association Test (COWA) (Krup et al., 1991b). The cognitive differences between patients and healthy controls remained significant even after controlling for differences in depressed mood (Krup et al., 1991b). Other impairments in PLS include poor performance on attention and verbal memory from the WMS-R (Bujak et al., 1996), poor mental flexibility, impaired verbal fluency, and slowed cognitive processing speed (Benke et al., 1995; Pollina, Elkins, Squires, Scheffer, & Krupp, 1999). Children with untreated Lyme disease can also have neurocognitive difficulties consisting of mild to moderate deficits on auditory and visual sequential processing (Bloom, Wyckoff, Meissner, & Steere, 1998). In contrast, among 41 pediatric posttreatment cases, no differences in cognitive functioning compared to controls were identified (Adams, Rose, Eppes, & Klein, 1994).

Evaluation The assessment of Lyme disease requires a careful history and physical examination. Antibody testing in the blood or CSF consists of IgM and IgG ELISA and IgM and IgG Western blot. However, within the first 2 weeks of the infection these tests can be negative. A complete blood cell count and electrolytes will screen for abnormalities associated with coinfection from other agents. In some cases, joint fluid or CSF should be tested for antibodies or cultured. Conventional magnetic resonance imaging (MRI) shows nonspecific the lesions that tend to be small (2–3 mm), lack mass effect, and do not correlate with disease duration (Fernandez, Rothberg, Ferencz, & Wujack,

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1990). Studies examining cerebral blood flow have demonstrated hypoperfusion of the cerebral white matter (Logigian et al., 1990; Fallon et al., 2008; Fallon, Keilp, Prohovnik, Heertum, & Mann, 2003) but the findings are nonspecific (Logigian et al., 1990). Neuropsychological testing is indicated for patients with symptoms of memory or other cognitive problems.

Treatment of Localized, Disseminated, and Persistent Lyme Disease Antibiotic treatment is the mainstay of therapy. For localized infection oral treatment is sufficient, usually with doxycyline 100 mg twice a day for 10 days. In later stages of infection with manifestations such as facial palsy, conduction block, or arthritis, oral therapy should be given for longer periods (30 days). For CNS complications or other signs of persistent infection, IV ceftriaxone is the treatment of choice.

Management of PLS How to manage patients with PLS has been the subject of much debate and varying opinions can be found in the literature (Hurley & Taber, 2008). The majority of infectious disease and neurology experts have concluded that repeated courses of antibiotic therapy are not effective for PLS (Halperin et al., 2007). Several randomized placebo-controlled clinical trials support this opinion. PLS subjects reporting reduced quality of life were enrolled in a clinical trial to determine whether additional antibiotic therapy using a combination of IV and oral antibiotics would improve their symptoms. There was no difference in treatment effect between the antibiotic and placebo groups (Klempner et al., 2001). Further, at baseline no cognitive impairments were identified in these PLS patients (Kaplan et al., 2003). Another randomized controlled trial was conducted for PLS subjects with persistent and severe fatigue (Krupp et al., 2003). Treatment groups received either 1 month of IV ceftriaxone or 1 month of IV placebo. After 1 month of therapy, the ceftriaxone group showed improved fatigue scores compared to the placebo group. However, by 6 months posttreatment fatigue levels had risen in the treated group and were not different from the placebo group (Krupp et al., 2003). A very comprehensive study examined the response of cognitive deficits in PLS to additional antibiotics.

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The study focused exclusively on PLS patients with documented cognitive impairment. Patients had to be seropositive for B. burgdorferi, and had to have completed of IV antibiotic therapy for Lyme disease prior to study entry (Fallon et al., 2008). Patients were randomized to receive either IV ceftriaxone or IV placebo for 10 weeks. A treatment benefit on memory performance was observed at week 12 but the benefit was not sustained at 24 weeks. Further, adverse events were associated with IV therapy. Taken together, these clinical trials of do not support treating PLS with additional antibiotics (Fallon et al., 2008; Klempner et al., 2001; Kaplan et al., 2003; Krupp et al., 2003). On the other hand, symptomatic therapies for pain or depression should be considered. In summary, most patients with Lyme disease respond well to antibiotic therapy. Some patients experience cognitive impairments that usually improve following therapy. A subgroup of patients develop PLS and experience persistent cognitive symptoms, fatigue, pain, and reduced quality of life. These problems do not respond to additional antibiotic treatment. Alternative therapeutic approaches for persistent symptoms need to be developed.

References and Readings Adams, W. V., Rose, C. D., Eppes, S. C., & Klein, J. D. (1994). Cognitive effects of Lyme disease in children. Pediatrics, 94, 185–189. Benke, T., Gasse, T., Hittmair-Delazer, M., & Schmutzhard, E. (1995). Lyme encephalopathy: Long-term neuropsychological deficits years after acute neuroborreliosis. Acta Neurologica Scandinavica, 91, 353–357. Bloom, B. J., Wyckoff, P. M., Meissner, H. C., & Steere, A. C. (1998). Neurocognitive abnormalities in children after classic manifestations of Lyme disease. The Pediatric Infectious Disease Journal, 17, 189–196. Broderick, J. P., Sandock, B. A., & Mertz, L. E. (1987). Focal encephalitis in a young woman 6 years after the onset of Lyme disease: Tertiary lyme disease? Mayo Clinic Proceedings, 62, 313–316. Bujak, D. I., Weinstein, A., & Dornbush, R. (1996). Clinical and neurocognitive features of the post Lyme syndrome. The Journal of Rheumatology, 23, 1932–1937. Cairns, V., & Godwin, J. (2005). Post-Lyme borreliosis syndrome: A meta-analysis of reported symptoms. International Journal of Epidemiology, 34, 1340–1345. Fallon, B. A., Keilp, J., Prohovnik, I., Heertum, R. V., & Mann, J. J. (2003). Regional cerebral blood flow and cognitive deficits in chronic lyme disease. The Journal of Neuropsychiatry and Clinical Neurosciences, 15, 326–332. Fallon, B. A., Kelip, J. G., Corbera, K. M., et al. (2008). A randomized, placebo-controlled trial of repeated IV antibiotic therapy for Lyme encephalopathy. Neurology, 70, 992–1003. Fernandez, R. E., Rothberg, M., Ferencz, G., & Wujack, D. (1990). Lyme disease of the CNS: MR imaging findings in 14 Cases. AJNR. American Journal of Neuroradiology, 11, 479–481.

Garin, C. H., & Bujacoux, C. (1922). Paralysie par les Tiques. Journal de me´decine de Lyon, 71, 765–767. Gaudino, E. A., Coyle, P. K., & Krupp, L. B. (1997). Post-Lyme syndrome and chronic fatigue syndrome. Neuropsychiatric similarities and differences. Archives of Neurology, 54, 1372–1376. Halperin, J. J., Shapiro, E. D., Logigian, E., et al. (2007). Practice parameter: treatment of nervous system Lyme disease (an evidence-based review): Report of the quality standards subcommittee of the American Academy of Neurology. Neurology, 69, 91–102. Hurley, R. A., & Taber, K. H. (2008). Acute and chronic Lyme disease: Controversies for Neuropsychiatry. The Journal of Neuropsychiatry and Clinical Neurosciences, 20, iv–6. Kaplan, R. F., Meadows, M.-E., Vincent, L. C., Logigian, E. L., & Steere, A. C. (1992). Memory impairment and depression in patients with Lyme encephalopathy: Comparison with fibromyalgia and nonpsychotically depressed patients. Neurology, 42, 1263–1267. Kaplan, R. F., Trevino, R. P., Johnson, G. M., et al. (2003). Cognitive function in post-treatment Lyme disease: Do additional antibiotics help. Neurology, 60, 1916–1922. Klempner, M. S., Hu, L. T., Evans, J., et al. (2001). Two controlled trials of antibiotic treatment in patients with persistent symptoms and a history of Lyme disease. The New England Journal of Medicine, 345, 85–92. Krupp, L. B., Hyman, L. G., Grimson, R., et al. (2003). Study and treatment of post Lyme disease (STOP-LD): A randomized double masked clinical trial. Neurology, 60, 1923–1930. Krupp, L. B., Masur, D., Joseph, S., et al. (1991a). Cognitive functioning in late Lyme Borreliosis. Archives of Neurology, 48, 1125–1129. Krupp, L. B., Masur, D., Schwartz, J., et al. (1991b). Cognitive functioning in late Lyme borreliosis. Archives of Neurology, 48, 1125–1129. Logigian, E. L., Kaplan, R. F., & Steere, A. C. (1990). Chronic neurologic manifestations of Lyme disease. The New England Journal of Medicine, 323, 1438–1444. Pollina, D. A., Elkins, L. E., Squires, N. K., Scheffer, S. R., & Krupp, L. B. (1999). Does process-specific slowing account for cognitive deficits in Lyme disease? Applied Neuropsychology, 6, 27–32.

Lymphoplasmacyte Rich Tumor ▶ Lymphoplasmacytic Tumor

Lymphoplasmacytic Tumor J ENNIFER T INKER Drexel University Philadelphia, PA, USA

Synonyms Lymphoplasmacyte rich tumor

Lymphoplasmacytic Tumor

Definition

Cross References

Lymphoplasmacytic tumors are composed of lymphocytes and plasma cells. Within the CNS, lymphoplasmacytic tumor presents as a lymphoplasmacyte-rich meningioma (LRM) (previously designated inflammatory meningioma). Being an extremely rare variant of meningioma, LRM demonstrates inconsistent biological behavior and clinical course. The LRM is composed primarily of inflammatory cells, and subsequently presents as clinically similar to hematological abnormalities rather than typical meningiomas (Yamaki, Ikeda, Sakamoto, Ohtaki & Hashi, 1997). Mean age of onset is 34 years of age. LMRs are unresponsive to anti-inflammatory therapy, and are primarily treated by means of surgical debulking, when feasible. Radiation therapy serves as a secondary treatment (Bruno et al., 2004).

▶ Meningiomas

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References and Readings Bruno, M. C., Ginguene, C., Santangelo, M., Panagiotopoulos, K., Piscopo, G. A., Tortora, F. et al. (2004). Lymphoplasmacyte rich meningioma. A case report and review of the literature. Journal of Neurological Sciences, 48(3), 117–124. Yakami, T., Ikeda, T., Sakamoto, Y., Ohtaki, M., & Hashi, K. (1997). Lymphoplasmacyte-rich meningioma with clinical resemblance to inflammatory pseudotumor. Journal of Neurosurgery, 86, 898–904.

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M M’Naghten Rule N ATHALIE D E FABRIQUE Cook County Department of Corrections Chicago, IL, USA

Synonyms McNaughton test

Denney, R. L. (2005). Criminal responsibility and other criminal forensic issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. New York: Oxford University Press. M’Naughten’s Case, 10 Clark & Finnelly 200 (1843). Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psychological evaluations for the courts (3rd ed.). New York: Guilford. Rogers, R., & Shuman, D. (2000). Conducting insanity evaluations (2nd ed.). New York: Guilford. Shapiro, D. L. (1999). Criminal responsibility evaluations: A manual for practice. Sarasota, FL: Professional Resource Press. Yates, K. F., & Denney, R. L. (2008). Neuropsychology in the assessment of mental state at the time of the offense. In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the criminal forensic setting. New York: Guilford.

Definition Historically, insanity was not considered a defense, but was a way to be acquitted from a crime as a result of a mental illness. The test was named after Daniel M’Naghten, a British man who was a defendant in a murder case in 1843. He was acquitted due to being found insane. The M’Naghten Test or M’Naghten Rule states that a person may be insane if at the time of committing the crime, the accused person was under the effect of mental illness and did not know the nature and quality of the act he was doing. If the person accused did know what he was doing, the M’Naghten Test assesses whether the person knows what he did was wrong.

Cross References ▶ Actus Reus ▶ Insanity ▶ Insanity Defense ▶ Mens Rea

References and Readings American Law Institute (1955). Model penal code. Philadelphia: American Law Institute. Dalby, J. T. (2006). The case of Daniel McNaughton: Let’s get the story straight’’. American Journal of Forensic Psychiatry, 27, 17–32.

MacArthur Competence Assessment Tool for Clinical Research (MacCAT-CR) ▶ MacArthur Competence Assessment Tools

MacArthur Competence Assessment Tool for Treatment (MacCAT-T) ▶ MacArthur Competence Assessment Tools

MacArthur Competence Assessment Tool-Criminal Adjudication (MacCAT-CA) ▶ MacArthur Competence Assessment Tools


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MacArthur Competence Assessment Tools

MacArthur Competence Assessment Tools LYNN A. S CHAEFER Nassau University Medical Center East Meadow, NY, USA

Synonyms MacArthur competence assessment tool for clinical research (MacCAT-CR); MacArthur competence assessment tool for treatment (MacCAT-T); MacArthur competence assessment tool-criminal adjudication (MacCAT-CA)

Description The MacArthur Competence Assessment Tools (MacCAT) consist of the MacArthur Competence Assessment Tool for Treatment (MacCAT-T), the MacArthur Competence Assessment Tool for Clinical Research (MacCAT-CR), and the MacArthur Competence Assessment Tool-Criminal Adjudication (MacCAT-CA). Each of these tools measures a different form of decisional capacity; separate instruments have been developed based on the premise that the capacity to make decisions is situation-specific. All three instruments are commercially available through Professional Resource Press (www.prpress.com). The MacCAT-T (Grisso & Appelbaum, 1998b) measures capacity to consent to medical treatment with a semi-structured interview tailored to the patient’s specific disorder and treatment decision. Patients are provided information about their disorder and are asked to reiterate this in their own words; follow-up questions ensure patients’ understanding of their condition. Patients are then asked if they have doubts about the information and the reasons for their beliefs are explored. The process is repeated for treatment options. Finally, the patient is asked to make a choice; questions are asked to ascertain their reasoning process. The order of the interview is: understanding disorder, appreciation disorder, understanding treatment, understanding risks and benefits, appreciation treatment, alternative treatments, first choice and reasoning, generating consequences, and final choice. Responses are rated by the clinician as 2, 1, or 0 (adequate, questionable, and inadequate), using the manual as a guide. What is considered ‘‘adequate’’ for each response

will depend on the specific situation or condition and the clinician’s judgment that the patient’s response was or was not accurate. Summary ratings are generated for four constructs: understanding (rating range 0–6), appreciation (0–4), reasoning (0–8), and expressing a choice (0 or 2). There is no total score; each summary rating can be discussed separately. There are also no cutoffs for the individual summary scores. An inadequate rating on one scale may result in the patient being considered incompetent to consent to treatment. The construct considered the most important in determining competence varies somewhat by state, with most states recognizing understanding, but with variability on use of the other constructs. The MacCAT-Twas normed on 40 hospitalized patients with schizophrenia or schizoaffective disorder and 40 controls (matched for age, gender, race, and socioeconomic status). Administration requires 20–25 min. Both a DVD and a VHS videotape are available demonstrating the administration of the MacCAT-T. This tool has the advantages of ease of use in clinical settings and of being tailored to the individual patient’s medical situation. The MacCAT-CR (Appelbaum & Grisso, 2001) measures capacity to consent to participation in research. Questions are tailored to the specific research study in which the subject is asked to participate. As in the MacCAT-T, four summary ratings are generated: understanding, appreciation, reasoning, and expressing a choice. Responses are rated by the clinician as 2, 1, or 0, using the manual as a guide. Understanding (rating range 0–26) is based on answers to 13 questions that are in reference to five disclosures about the research study. Appreciation (0–6) is based on answers to three questions assessing: (a) the subject’s recognition of the study as not meant for their personal medical benefit, (b) the possibility of their reduced benefit, and (c) their ability to withdraw from the study. Reasoning (0–8) ascertains the subject’s reasoning of their expressed choice (0–2). Administration requires 20–25 min. Norms for the MacCAT-CR are not provided. Like the MacCAT-T, the MacCAT-CR has the advantage of utilizing specific details of the individual’s situation in order to make decisions about competency. The MacCAT-CA (Poythress et al., 1999) measures a defendant’s competency to stand trial. It is a structured interview of 22 questions, organized into understanding, reasoning, and appreciation constructs. Responses are again rated by the clinician as 2, 1, or 0, using the manual as a guide. Unlike the previous MacArthur tools, the MacCAT-CA features a hypothetical vignette, from which questions for the understanding and reasoning scores are derived. Understanding score questions determine defendants’ comprehension of the roles of attorneys,


judge, and jury; the charged offense and any lesser offense; consequences of conviction and pleading guilty and rights waived when pleading guilty. Reasoning score questions assess judgments by the defendants of the relative importance of facts, as well as their decision process about two pleading choices. The appreciation score comes from questions about the defendants’ legal situation, and specifically by the explanations of their choices (to rule out implausible or delusional thinking). Summary scores are generated for understanding (rating range 0–16), reasoning (0–16), and appreciation (0–12); there is no total score. Cutoff scores, while provided, are intended to be used comparatively and not as absolutes. Norms were derived from 283 hospitalized defendants whom were deemed incompetent, 249 jailed defendants whom were treated for psychiatric reasons but deemed competent, and 197 jailed inmates presumed competent. Subjects were between 18 and 65 years of age, and 90% were male. This tool has the advantages of potential use in both clinical and research settings and of having a large normative sample. However, it has the disadvantage of employing a vignette and not being completely tailored to the individual defendant’s actual situation. The applicability to females has also been questioned (Grisso, 2003).

Historical Background Neuropsychologists are occasionally asked to provide information regarding an individual’s decision-making capabilities, which can be used in judgments of competency. Competence is a legal construct involving one’s right to make decisions for oneself; adults are deemed to be competent unless proven otherwise. Simply having a mental or cognitive disorder or diagnosis is not enough to be considered ‘‘incompetent.’’ There must also be evidence that individuals are unable to understand relevant information, engage in reasoning based on provided information, and make and express decisions regarding their wishes. Determinations of competence are not all-or-nothing: there are different types of competencies; each involving different skill sets (see Grisso, 2003). While executive functioning skills, and decision-making ability in particular, are important for demonstrating competence, evidence of competency is derived from the performance of various functional tasks (e.g., managing finances and taking medication). An important distinction is made between decisional capacity (the ability to decide) and executional capacity (the ability to carry out the decision) (Collopy, 1988). Individuals with only decisional capacity

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may still be deemed competent, as they may be able to carry out tasks with assistance and/or they can instruct others to perform tasks in accordance with their decisions. Moberg and Kniele (2006) discuss the ethical evaluation of competency by neuropsychologists, and advocate integrating specific competency measures into interview and neuropsychological testing data, when available. The MacCAT are one such example of competency measures, each evaluating a different type of decision-making competency. All three measures were created through funding from the MacArthur Research Network on Mental Health and Law. The MacCAT-T was derived from three instruments that were part of the initial research initiative in the 1990s, the MacArthur Treatment Competence Study: the Understanding Treatment Disclosures (UTD), Perceptions of Disorder (POD), and Thinking Rationally about Treatment (TRAT) instruments (see Grisso, 2003). These three instruments assessed decision-making ability (understanding, appreciation/acknowledgment, and reasoning, respectively), but were lengthier (60–90 min each), used hypothetical vignettes rather than patients’ actual situations, and had detailed scoring criteria. A shorter, more flexible, and more relevant instrument was sought for use by clinicians. The MacCAT-CR was derived from the MacCAT-T. The MacCAT-CA is derived from the longer (47-item) MacArthur Structured Assessment of Competencies of Criminal Defendants (MacSAC-CD), which was a research tool as part of the MacArthur Research Network on Mental Health and Law to develop measures of competence to stand trial.

Psychometric Data Each of the three MacArthur tools is standardized with regard to procedure (i.e., structure of interview questions), but not with regard to content, as each utilizes at least some, if not all, of the subject’s individualized situation in the questions. The MacCAT-CA, utilizing a vignette and a highly structured interview, has the best standardization of the three. For the MacCAT-T, interrater reliability was 0.99 for understanding, 0.87 for appreciation, and 0.91 for reasoning (Grisso and Appelbaum, 1998b). This was determined by correlations among three raters on 20 patient and 20 community control protocols. Retest reliability was not performed. Given the purpose of the instrument, a review of test items revealed that the measure has good face validity. Tests of convergent validity with physician ratings have been mixed (Sturman, 2005). For example, in

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a German study (Vollman et al., 2003), there were moderate associations between MacCAT-T scores and physician ratings for the schizophrenia and dementia groups, but not the depression group. The MacCAT-CR’s interrater reliability in two studies of persons with schizophrenia and Alzheimer’s disease (Carpenter et al., 2000; Kim et al., 2001) was 0.98 and 0.94 for understanding, 0.84 and 0.90 for appreciation, and 0.84 and 0.80 for reasoning, respectively. Retest reliability in patients with depression (Appelbaum et al., 1999) was 0.26 for understanding, 0.36 for appreciation, and 0.15 for reasoning. For the MacCAT-CA, interrater reliability was very good to excellent, with intraclass correlations of 0.90 for understanding, 0.85 for reasoning, and 0.75 for appreciation (Otto et al., 1998). Regarding internal consistency, Cronbach’s alpha was 0.85 for understanding, 0.81 for reasoning, and 0.88 for appreciation. Within each scale, inter-item correlations were 0.42 for understanding, 0.36 for reasoning, and 0.54 for appreciation. Significant correlations were also found between MacCAT-CA scales and WAIS-R Full Scale IQ (particularly between understanding and reasoning) and psychoticism (as measured by the Brief Psychiatric Rating Scale (BPRS) and the Minnesota Multiphasic Personality Inventory-2 (MMPI-2)) in the original study (Otto et al., 1998). A more recent study examining the factor structure of the MacCAT-CA using the original normative dataset (Zapf et al., 2005) found the instrument to be normatively sound and fitting best with a three-factor model (understanding, reasoning, and appreciation). Correlations between MacCAT-CA scores and tests of intelligence (estimated WAIS-R Full Scale IQ) and psychopathology (BPRS) were 0.42 and -0.36, respectively, indicating moderate to strong covariation.

Clinical Uses In a review of the measure, Grisso (2003) stated, ‘‘A strength of the MacCAT-T is its use of constructs that are based on legal analysis of competence and that have proved useful in studies with the parallel research instruments that influenced the development of the MacCAT-T (p. 425).’’ All three MacArthur tools utilize the constructs of understanding, appreciation, and reasoning. The MacCAT-T and MacCAT-CR offer clinical portability and situationspecificity at the expense of exactness and standardization. The MacCAT-CA offers better standardization, but loses some situation-specificity. The lack of a total score on these instruments underlies the concept that competence is multifaceted; the absence of cutoff scores (on the

MacCAT-Tand MacCAT-CR) indicates that competence is not ‘‘all-or-nothing.’’ Even though the MacCAT-CA provides cutoff scores, the manual cautions against using these as absolutes. Rather, they should be used as part of the evaluation for competence. The MacCAT-T has been used to assess competence to consent to treatment in persons with dementia, schizophrenia, and depression (Vollman et al., 2003). Patients with dementia were found more impaired than those with schizophrenia, who in turn were more impaired than those with depression. In medical inpatients, Raymont and others (2004) utilized the MacCAT-T to examine decision-making capacity in acutely ill medical patients of various diagnoses. Regarding the MacCAT-CR, studies have shown that cognitively impaired individuals with Alzheimer’s disease (Kim et al., 2001) and mild cognitive impairment (Jefferson et al., 2008) have more decision-making impairment compared to controls, and therefore would have impaired capacity to provide informed consent for research. The MacCAT-CR has also been used to assess capacity of patients with schizophrenic and HIV to consent to clinical drug trials (Moser et al., 2002). According to the manual, the MacCAT-CA can be used for felony and misdemeanor defendants in inpatient, outpatient, or forensic settings and can be used to assess treatment. The MacCAT-CA is not recommended for individuals who are cognitively impaired (e.g., those with limited intellectual abilities). It also does not include an effort measure (explicit or embedded) to rule out feigned incompetence or retardation (Rogers et al., 2002). For a screening tool with an embedded measure for feigned incompetence, the Evaluation of Competency to Stand Trial-Revised (ECST-R; Rogers et al., 2004) is favored (see Marcopulos et al., 2008). Finally, the MacCAT-CA has limitations in non-English speaking individuals as well as those suffering from delusions (Pinals et al., 2006). The MacCAT-CA has recently been tested on an adolescent population (Grisso et al., 2003). It has also been adapted to legal requirements for competence to stand trial in England and Wales (Akinkunmi, 2002). This measure, the MacArthur Competence Assessment Tool-Fitness to Plead (MacCAT-FP) evidenced good internal consistency and interrater reliability, and significantly differentiated a prison group from a hospital group.

Cross References ▶ Brief Psychiatric Rating Scale ▶ Competency

Macrocephaly

▶ Criminal Forensics ▶ Diminished Capacity ▶ Full Scale IQ ▶ Functional Capacity Evaluations ▶ Informed Consent ▶ Legal Competency ▶ Mild Cognitive Impairment ▶ Minnesota Multiphasic Personality Inventory ▶ Patient Competency Rating Scale

References and Readings Akinkunmi, A. (2002). The MacArthur competence assessment tool— fitness to plead: a preliminary evaluation of a research instrument for assessing fitness to plead in England and Wales. Journal of the American Academy of Psychiatry and the Law, 30, 476–482. American Bar Association Commission on Law and Aging - American Psychological Association (2008). Assessment of older adults with diminished capacity: a handbook for psychologists. Washington, DC: American Psychological Association. Appelbaum, P. S., & Grisso, T. (2001). MacArthur competence assessment tool for clinical research (MacCAT-CR). Sarasota, FL: Professional Resource. Appelbaum, P. S., Grisso, T., Frank, L., O’Donnell, S., & Kupfer, D. (1999). Competence of depressed patients for consent to research. American Journal of Psychiatry, 156, 1380–1384. Carpenter, W., Gold, J., Lahti, A., Queern, C., Conley, R., Bartko, J., et al. (2000). Decisional capacity for informed consent in schizophrenia research. Archives of General Psychiatry, 57, 533–538. Collopy, B. J. (1988). Autonomy in long term care: Some crucial distinctions. The Gerontologist, 28(Suppl), 10–17. Dunn, L. B., Nowrangi, M. A., Palmer, B. W., Jeste, D. V., & Saks, E. R. (2006). Assessing decisional capacity for clinical research or treatment: a review of instruments. American Journal of Psychiatry, 163, 1323–1334. Grisso, T. (2003). Evaluating competencies: forensic assessments and instruments (2nd ed.). New York: Klumer/Plenum. Grisso, T., & Appelbaum, P. S. (1998a). Assessing competence to consent to treatment: A guide for physicians and other health professionals. New York: Oxford University Press. Grisso, T., & Appelbaum, P. S. (1998b). MacArthur competence assessment tool for treatment (MacCAT-T). Sarasota, FL: Professional Resource. Grisso, T., Steinberg, L., Woolard, J., Cauffman, E., Scott, E., Graham, S., et al. (2003). Juveniles’ competence to stand trial: a comparison of adolescents’ and adults’ capacities as trial defendants. Law and Human Behavior, 27, 333–364. Jefferson, A. L., Lambe, S., Moser, D. J., Byerly, L. K., Ozonoff, A., & Karlawish, J. H. (2008). Decisional capacity for research participation among individuals with mild cognitive impairment. Journal of the American Geriatrics Society, 56(7), 1236–1243. Kim, S., Caine, E., Currier, G., Leibovici, A., & Ryan, J. (2001). Assessing the competence of persons with Alzheimer’s disease in providing informed consent for participation in research. American Journal of Psychiatry, 158, 712–717. Marcopulos, B., Morgan, J., & Denney, R. L. (2008). Neuropsychological evaluation of competency to proceed. In R. L. Denney & J. P. Sullivan

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(Eds.), Clinical neuropsychology in the criminal forensic setting (pp. 176–203). New York: Guilford. Moberg, P. J., & Kniele, K. (2006). Evaluation of competency: ethical considerations for neuropsychologists. Applied Neuropsychology, 13 (2), 101–114. Moser, D. J., Schultz, S. K., Arndt, S., Benjamin, M. L., Fleming, F. W., Brems, C. S., et al. (2002). Capacity to provide informed consent for participation in schizophrenia and HIV research. American Journal of Psychiatry, 159, 1201–1207. Otto, R., Poythress, N., Edens, J., Nicholson, R., Monahan, J., Bonnie, R., et al. (1998). Psychometric properties of the MacArthur competence assessment tool – criminal adjudication. Psychological Assessment, 10, 435–443. Pinals, D. A., Tillbrook, C. E., & Mumley, D. L. (2006). Practical application of the MacArthur competence assessment tool – criminal adjudication (MacCAT-CA) in a public sector forensic setting. Journal of the American Academy of Psychiatry and the Law, 34(2), 179–188. Poythress, N., Nicholson, R., Otto, R., Eden, J., Bonnie, R., Monahan, J., et al. (1999). The MacArthur competence assessment tool – criminal adjudication: professional manual. Odessa, FL: Psychological Assessment Resources. Raymont, V., Bingley, W., Buchanan, A., David, A. S., Hayward, P., Wessely, S., et al. (2004). Prevalence of mental incapacity in medical inpatients and associated risk factors: cross-sectional study. Lancet, 364, 1421–1427. Rogers, R., Sewell, K. W., Grandjean, N. R., & Vitacco, M. (2002). The detection of feigned mental disorders on specific competency measures. Psychological Assessment, 14, 177–183. Rogers, R., Tillbrook, C. E., & Sewell, K. W. (2004). Evaluation of competency to stand trial – revised professional manual. Lutz, FL: Psychological Assessment Resources. Sturman, E. D. (2005). The capacity to consent to treatment and research: a review of standardized assessment tools. Clinical Psychology Review, 25, 954–974. Vollmann, J., Bauer, A., Danker-Hopfe, H., & Helmchen, H. (2003). Competence of mentally ill patients: a comparative empirical study. Psychological Medicine, 33, 1463–1471. Zapf, P. A., Skeem, J., & Golding, S. L. (2005). Factor structure and validity of the MacArthur competence assessment tool – adjudication. Psychological Assessment, 17(4), 433–445.

MACI ▶ Millon Adolescent Clinical Inventory

Macrencephaly ▶ Megalencephaly

Macrocephaly ▶ Megalencephaly

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Macropsia

Macropsia T HOMAS M. L AUDATE , A ARON P. N ELSON Brigham and Women’s Hospital Boston, MA, USA

References and Readings Park, M. G., Choi, K. D., Kim, J. S., Park, K. P., Kim, D. S., Kim, H. J., & Jung, D. S. (2007). Hemimacropsia after medial temporo-occipital infarction. Journal of Neurology, Neurosurgery, and Psychiatry, 78(5), 546–548.

Synonyms Megalopsia

Mad Hatter Syndrome ▶ Mercury Exposure

Definition Macropsia is a condition in which visual objects are perceived to be larger than they are objectively sized. Macropsia can be a clinical feature of migraine, stroke, or temporal, parietal, or occipital lobe epilepsy. Macropsia can also be caused by disorders in areas throughout the visual system. For instance, retinal rod and cone cells can become spaced closely together (e.g., due to macular scarring, tumor). With increased density of photoreceptors, an observed object is perceived as being larger than usual. Macropsia is also thought to occur as a result of impairment of ocular accommodation (i.e., focus), caused by spasm or natural functional variation. The impaired accommodation causes a close object to be judged to be at a distance, but since the actual retinal image is large, macropsia occurs. Also, macropsia can be induced by recently prescribed presbyopic correction or by drug effects. Macropsia, together with micropsia and distortions of perceived self-size of body or body part, can be symptoms of the so-called Alice in Wonderland Syndrome. This syndrome has been reported in cases of Epstein–Barr virus encephalitis, migraines, and frontal lobe epilepsy. The syndrome is named after the Lewis Carroll book, Alice’s Adventures in Wonderland, in which Alice, after falling down a rabbit hole, drinks from a bottle that causes her to shrink to 10 in. height and then eats a small cake that makes her grow to more than 9 ft tall.

Cross References ▶ Epilepsy ▶ Micropsia ▶ Visuoperceptual

MAE ▶ Multilingual Aphasia Examination

Magnetic Apraxia ▶ Alien Hand Syndrome

Magnetic Gait A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 1 Boston University Medical Center Boston, MA, USA 2 William Beaumont Army Medical Center El Paso, TX, USA

Definition A gait abnormality marked by an inability to lift the feet off the floor. It results in a decrease in mobility and an increase in falls. It is most commonly associated as part of the triad of symptoms of normal pressure hydrocephalus. As such, it can be associated with dementia and urinary incontinence. Normal pressure hydrocephalus is often considered in the differential diagnosis for Parkinson’s disease.

Cross References ▶ Parkinsonism

Magnetic Resonance Imaging

References and Readings Sudarsky, L. (2004). Gait disorders. In R. L. Watts, & W. C. Koller (Eds.), Movement disorders (2nd ed., pp. 813–824). New York: McGraw-Hill.

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evaluation of the carotid arteries reducing the need for conventional angiography. In traumatic brain injury, MRA can be used to screen for vascular injuries in the head and neck; however, conventional angiography still remains superior to MRA for evaluation and also treatment of traumatic vascular injuries (Barkley, Morales, Hayman, & Diaz-Marchan, 2007).

Magnetic Resonance (MR) DSA ▶ Digital Subtraction Angiography

Cross References ▶ Magnetic Resonance Imaging ▶ Transcranial Doppler Ultrasonography

Magnetic Resonance Angiography J ACINTA M C E LLIGOTT National Rehabilitation Hospital Dun Laoghaire Co., Dublin, Ireland

Definition Magnetic resonance angiography (MRA) is a magnetic resonance imaging (MRI) technique that provides cross sectional or projectional images of normal and diseased arterial tissue (Yucel et al., 1999).

References and Readings Barkley, J. M., Morales, D., Hayman, L. A., & Diaz-Marchan, P. J. (2007). Static neuroimaging in the evaluation of TBI. In N. D. Zasler, D. L. Katz, R. D. Zafonte (Eds.), Brain injury medicine: Principles and practice.New York: Demos. Donnelly, R., David, H., & London, N. J. M. (2000). ABC of arterial and venous disease: Non invasive methods of arterial and venous assessment. Student BMJ, Retrieved from http://student.bmj.com/issues/ 00/08/education/270.php Yucel, K. E., Anderson, C. M., Edelmann, R. R., Grist, T. M., Baum, R. A., Manning, W. J., et al. (1999). Magnetic resonance angiography: Update on applications for extracranial arteries. American Heart Association Scientific Statement, 100(22), 2284–2301.

Current Knowledge New advances in MRI and MRA offer potentially noninvasive or minimally invasive imaging of cerebral blood vessels (Yucel et al., 1999). While cerebral angiography remains the gold standard in the evaluation of cerebrovascular disease, this procedure requires the insertion of a catheter into an artery, usually the femoral artery in the groin, and then threading the catheter into the carotid artery in the neck to inject a contrast medium containing iodine. Cerebral angiography is therefore invasive, costly, and potentially hazardous. New advances in MRA technique allow the imaging of a moving column of blood and do not require ionizing radiation or iodinated contrast (Donnelly, David, & London, 2000). Advances in MRI technology and the use of contrast agents may potentially expand the capability and clinical applications of MRA (Yucel et al., 1999). For example, MRA has been found to complement Doppler ultrasonography for preoperative

Magnetic Resonance Imaging E DUARDO L OPEZ Johnson Rehabilitation Institute Edison, NJ, USA

Synonyms MRI

Definition MRI is the computer generated cross-sectional images of the body through the placement of the body part in a large

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Magnetic Resonance Sprectroscopy

static magnetic field gradient pulsed to allow the resonance of hydrogen to be detected.

Current Knowledge MRI became available in the early 1980s and developed into a method comparable to existing techniques, such as ultrasound and computed tomography (CT). MRI, however, is more sensitive to pathology than X-ray attenuation. As bone has a very low signal on MRI, extracerebral blood collections and pathology in the posterior fossa, inferior frontal, and temporal regions are better detected. Furthermore, imaging can be conducted in all three planes without requiring patient repositioning and the better contrast between gray and white matter reveals anatomic details more readily, i.e. sulci can be seen even if they are compressed by extradural collections. MRI has a clear advantage in characterizing lesions of all types in the subacute and chronic recovery period. Gradient echo imaging sequences can help detect postacute lesions, emphasizing magnetic susceptibility effects of old blood (hemosiderin). However, MRI does not demonstrate acute blood as clearly as CT does, takes significantly longer than CT and is more expensive. Additionally, MRI and requires a ventilator with nonferromagnetic components for intubated patients, making it impractical for initial evaluation of patients with severe head injury. Superiority of MRI over CT has been unequivocal for brainstem lesions, deep lesions such as petechial hemorrhages seen in patients with diffuse axonal injury (DAI), and in imaging the corpus callosum which may be the site of small hemorrhages. MRI with its higher sensitivity, better spatial resolution, excellent soft tissue contrast, multiplanar imaging capability, and lack of ionizing radiation – emerged as primary modality of choice in the evaluation of patients with traumatic brain injury (TBI) and epilepsy. MR imaging plays a crucial role not only in identifying the anatomical location or a substrate but also in depicting the relationship of the substrate to the eloquent regions of the brain (cortices involved with motor, speech, or memory function). MR imaging can clearly demonstrate traumatic hematomas, especially those that are subacute and appear isodense on CT. MRI scanning offers several advantages over CT in the brain injury population, especially for long-term prognostication. The relationship of imaging with neuropsychological outcome has centered on correlation of test measures, a patient’s particular impairment, and pathology reported on MRI (Katz 1999).

Cross References ▶ Anencephaly ▶ Computed Tomography ▶ Neuroimaging

References and Readings Haaga, J. R. (1994). In Haaga, J. R., et al. (Ed.), Computed tomography and magnetic resonance imaging of the whole body (3rd ed.). St. Louis: Mosby-Year Book, Inc. Katz, D. I., & Black, S. E., (1999). Neurological and neuroradiological evaluation. In M. Rosenthal et al. (Ed.), Rehabilitation of the adult and child with traumatic brain injury (3rd ed., pp. 89–116). Philadelphia: FA Davis

Magnetic Resonance Sprectroscopy C INDY B. I VANHOE , N ATASHA K. E ADDY Neurorehabilitation Specialists Houston, TX, USA

Synonyms MRS; Proton magnetic resonance spectroscopy

Definition Magnetic resonance spectroscopy is a noninvasive study used to characterize tissues of the body. It evaluates the concentration of metabolites in the areas studied. Like magnetic resonance imaging, MRS is based on the principle of nuclear magnetic resonance.

Current Knowledge MRS can use signals from a variety of nuclei such as carbon, nitrogen, fluorine, sodium, phosphorus, and hydrogen to study the concentration of metabolites (i.e., N-acetyl apartate, choline, creatine, glutamate, glutamine, lipids, amino acids, and lactate) in the tissue examined. The study is most notably used to evaluate disorders of the central nervous system. MRS is not always specific, but in combination with clinical information and MRI,

Magnocellular Neurons

it has been shown to be helpful in diagnosing certain diseases. The results of MRS are displayed in a graph with a spectrum of resonances (peaks) displayed on the x-axis using units of parts per million (ppm) and the amplitude of the resonances measured on the y-axis using an arbitrary scale. The clinical applications of this study continue to be debated and defined, but currently it may be used to evaluate brain neoplasms, acute and chronic multiple sclerosis lesions, neuropsychiatric systemic lupus erythematosus, measure neuronal loss in patients with HIV disease, differentiation of brain abscess from other cystic lesions, metabolic disorders in children, evaluate areas of acute ischemia after stroke, locate an area of seizure focus in epileptic patients, diagnose neurodegenerative disorders such as Alzheimer disease, and evaluate diffuse axonal injury or metabolic depression in patients with traumatic brain injury.

Cross References ▶ Magnetic Resonance Imaging ▶ Neuroimaging

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Definition Magnetoencephalography (MEG) is a noninvasive, functional imaging technique that measures the magnetic fields produced by electrical currents in the brain.

Current Knowledge Skull and soft tissue interfere with magnetic currents less than electrical currents, providing MEG greater accuracy than electroencephalography (EEG). MEG may be particularly helpful in localizing areas of normal brain function, as well as brain dysfunction for clinical and research purposes. Information gained from MEG, which produces functional mapping, can be complementary to the structural findings obtained by magnetic resonance imaging (MRI) or computed tomography (CT) scan. Through a process referred to as magnetic source imaging (MSI), MEG may be combined with MRI to superimpose functional MEG data on structural MRI data (Paetau, 2002). MEG may be useful for: identifying seizure source, confirming that seizure activity is originating from a lesion found on structural imaging, presurgical mapping of brain function, and learning more about how the brain functions.

References and Readings Gujar, S. K., Maheshwari, S., Bjo¨rkman-Burtscher, I., & Sundgren, P. C. (2005). Magnetic resonance spectroscopy. Journal of NeuroOphthalmology, 25(3), 217–226. Smith, J. K., Londono, A., Castillo, M., & Kwock, L. (2002). Proton magnetic resonance spectroscopy of brain-stem lesions. Neuroradiology, 44, 825–829. Uzan, M., Albayram, S., Dashti, S. G. R., Aydin, S., Hanci, M., & Kuday, C. (2003). Thalamic proton magnetic resonance spectroscopy in vegetative state induced by traumatic brain injury. Journal of Neurology Neurosurgery and Psychiatry, 74, 33–38.

Magnetoencephalography F LORA H AMMOND 1, LORI G RAFTON 2 1 Indiana University Indianapolis, IN, USA 2 Carolinas Rehabilitation Charlotte, NC, USA

Synonyms MEG

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References and Readings Paetau, R. (2002). Magnetoencephalography in pediatric neuroimaging. Developmental Science, 5(3), 361–370.

Magnocellular Neurons U RAINA C LARK The Warren Alpert Medical School of Brown University The Miriam Hospital Providence RI, USA

Definition The magnocellular pathway is one of the three primary subcortical pathways (magnocellular, parvocellular, and koniocellular pathways) leading from the retina to visual cortex via the lateral geniculate nucleus (LGN). Cells in the magnocellular pathway (M pathway) are specialized for detecting contrast sensitivity, course features, and movement. In some ways, the M pathway can be considered the

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Mainstreaming

origin of the parietal or dorsal visual stream, as it has been shown that the M pathway dominates in the route leading from V1 to the parietal cortex. Experimental ablation of the M pathway results in reduced spatial contrast sensitivity and impairments in detecting rapidly moving or flickering stimuli, while visual acuity and color contrast sensitivity appear to be spared.

Current Knowledge Of the three major visual streams in the primate visual system, much is known about the magnocellular and parvocellular (P) pathways, whereas less is known about the more recently discovered koniocellular (K) pathway. For the most part, the M and P pathways are thought to function as separate systems, each serving distinct visual processes (e.g., color, motion, or luminance perception), working in parallel from the retina to visual cortex. However, findings suggest that some aspects of motion detection could be supported by combining information from the M and P pathways in primary visual cortex (De Valois & Cottaris, 1998). In the M pathway, retinal ganglion cells called parasol cells, which are sensitive to movement, flicker, and contrast, project onto the magnocellular layers (layers 1 and 2) of the LGN. Layer 1 receives input from the contralateral eye and is called the contralateral layer; layer 2 is ipsilateral. Only about 10% of the cells in the LGN are in the magnocellular layers (the remaining 90% are divided between the P and K layers). Investigations of the monkey visual system have revealed that projections from the magnocellular LGN layers terminate primarily in V1 layer 4Ca; cells from this region then synapse with cells in V1 layer 4B. Magnocellular LGN cells also project to V1 layer 6, which then projects back to the LGN. From layer 4B, projections are sent to several regions that are primarily involved in motion processing, low spatial frequency detection, and low contrast analysis, including V2’s thick CO stripe regions, and to dorsal V3 and the middle temporal area (MT, also referred to as V5). Projections from MT travel predominantly to the parietal cortex. Motion-perception deficits, or akinetopsia, have been reported in patients with V5 lesions as well as in neurologically healthy individuals who received transcranial magnetic stimulation to cause a temporary disruption of MT processing.

▶ Parvocellular Neurons ▶ Ventral Visual Pathway ▶ Visual Cortex ▶ Visual System

References and Readings Beckers, G., & Ho¨mberg, V. (1992). Cerebral visual motion blindness: transitory akinetopsia induced by transcranial magnetic stimulation of human area V5. Proceedings of the Royal Society of London, 24, 173–178. De Valois, R. L., & Cottaris, N. P. (1998). Inputs to directionally selective simple cells in macaque striate cortex. Proceedings of the National Academy of Sciences of the United States of America, 95(24), 14488–14493. Kaplan, E., Lee, B. B., & Shapley, R. M. (1990). New views of primate retinal function. In N. N. Osborne & G. J. Chader (Eds.), Progress in retinal research (Vol. 9, pp. 273–336). New York: Pergamon Press. Merigan, W. H., & Maunsell, J. H. R. (1993). How parallel are the primate visual pathways? Annual Review of Neuroscience, 16, 369–402. Van Essen, D. C., & Maunsell, J. H. R. (1983). Hierarchical organization and functional streams in the visual cortex. Trends in Neuroscience, 6, 270–275. White, A. J. R., Solomon, S. G., & Martin, P. R. (2001). Spatial properties of koniocellular cells in the lateral geniculate nucleus of the marmoset Calithrix jacchus. The Journal of Physiology, 533, 519–535. Xu, X., Ichida, M. J., Allison, J. D., Boyd, J. D., Bonds, A. B., & Casagrande, V. A. (2001). A comparison of koniocellular, magnocellular and parvocellular receptive field properties in the lateral geniculate nucleus of the owl monkey (Aotus trivirgatus). The Journal of Physiology, 531, 203–218. Zihl, J., von Cramon, D., & Mai, N. (1983). Selective disturbance of movement vision after bilateral brain damage. Brain, 106, 313–340.

Mainstreaming K ATHRINE H AK University of Northern Colorado Greeley, CO, USA

Synonyms Inclusion

Cross References

Definition

▶ Dorsal Visual Pathway ▶ Lateral Geniculate Nucleus of Thalamus

Mainstreaming refers to the practice of maximizing the time that students, who are receiving special education

Major Depression

services, spend in general education classrooms and with general education peers. It incorporates the provision of free and appropriate education (FAPE) in the least restrictive environment (LRE). It aims to provide as much time as possible for special education students with their same-aged peers; it balances the individual needs of these students with their inclusion in the instructional and social environment of their peers. Specialized services are only provided in a pullout model when a student’s individual needs cannot be satisfactorily met in an inclusive environment.

References and Readings Kavale, K. A. (2002). Mainstreaming to full inclusion: From orthogenesis to pathogenesis of an idea. International Journal of Disability, Development and Education, 49(2), 201–214. Knight, B. A. (1999). Towards inclusion of students with special needs in the regular classroom. Support for Learning, 14(1), 3–7. Powell-Smith, K. A., Stoner, G., Bilter, K. J., & Sansosti, F. J. (2008). Best practices in supporting the education of students with severe and low-incidence disabilities. In A. Thomas & J. Grimes (Eds.), Best practices in school psychology V (Vol. 4, pp. 1233–1248). Bethesda, MD: The National Association of School Psychologists.

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significant distress. In children or adolescents, irritability may be the primary criteria, instead of depressed mood.

Categorization Major depression is a mood disorder, and is categorized as either major depressive disorder, single episode or major depressive disorder, recurrent. Major depressive disorder, single episode consists of at least one major depressive episode of at least 2 weeks, while major depressive disorder, recurrent consists of at least two major depressive episodes of at least 2 weeks. In the Diagnostic and Statistical Manual of Mental Disorders, Text Revision (DSM IV-TR; APA, 2000), major depression can be further specified as mild, moderate, and severe with or without psychotic features. Other specifiers include its course: chronic (where criteria is met continuously for at least the past 2 years) and by the following features: catatonic features, melancholic features, atypical features, postpartum onset, or with seasonal features. There is some evidence that atypical features are more common in younger people and that melancholic features are more common in older depressed people (APA, 2000).

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