Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace
Jennifer Niskala Apps Laura Weiss Roberts
l
Robert F. Newby
Editors
Pediatric Neuropsychology Case Studies From the Exceptional to the Commonplace
Jennifer Niskala Apps Medical College of Wisconsin Department of Psychiatry and Behavioral Medicine 9000 W. Wisconsin Ave. Milwaukee WI 53226 USA
Robert F. Newby Medical College of Wisconsin Departments of Neurology and Pediatrics 9200 W. Wisconsin Ave. Milwaukee WI 53226 USA
Laura Weiss Roberts Medical College of Wisconsin Department of Psychiatry and Behavioral Medicine 8701 Watertown Plank Road Milwaukee WI 53226 USA
ISBN 978-0-387-78964-4
e-ISBN 978-0-387-78965-1
Library of Congress Control Number: 2008930758 # 2008 Springer Science+Business Media, LLC All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 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 know 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.
Printed on acid-free paper. 987654321 springer.com
To those who have always believed and encouraged, beginning with my unconditionally loving mom and continuing with my inspirational husband. —JNA For David, Michael, Kate, and Anna: Parents learn from their children. —RFN For my beautiful, resilient, compelling children, Madeline, Helen, Willa, and Thomas. —LWR
Preface
For each one of us, life is a story. Our own story. It begins before we are born and is written slowly, painstakingly, a day at a time – or at some moments in a sudden flurry. Each person who touches our lives writes a bit of that story, and in turn we touch others and write bits of their stories. Our story is a collection of our experiences, evidence of our impact in the world, and the knowledge we have of ourselves as individuals. When a child comes into the life of parents, several stories unite. We imagine this union, growing up on the expectation that parenthood, and childhood, should somehow be uniquely perfect. So what then happens when an unexpected chapter of that story is introduced? We have all heard that children are not born with a “how to” manual. No parent raising a child doubts the truth of this statement. However, for some parents, the immensity of this truth can seem difficult to bear. Some parents face the task of writing entire chapters of their child’s life while dealing with trauma, illness, and disabilities, which were never supposed to happen. A pediatric neuropsychologist is both fascinated and frustrated by these chapters in children’s lives. For most of us, we venture into this career because of our love for children and our intense desire to help those children in need. A neuropsychologist’s career begins with training in psychology, focusing on the “normal” and aberrant development of people, behaviorally and emotionally. We then turn our focus to the cognitive functions, studying the relationship of the brain to behaviors. In pediatric neuropsychology, these diligently applied skills are used to listen to each child’s story, interpret each parent’s experience, evaluate the brain’s functional abilities, and try to find ways to guide the story’s path. The unconditional love of a parent resonates beauty. Each pediatric professional who intervenes in a child’s life helps teach a family how to understand and grow with their child. Those of us who work in these professions know, on a personal level, how moving and amazing these stories of love can be. It is with respect to the journey children take that we collected some of those stories here. Our intention in creating this book was to represent the incredible diversity of challenges that many children and their families work so hard to understand and overcome, while acknowledging the professionals witnessing and mediating this process. By bringing such a wide collection of stories to the reader, we offer insight into the field of pediatric neuropsychology but also into the lives of the children and vii
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Preface
families with whom we work every day. This book is intended to inform our colleagues not only in pediatric neuropsychology but in all fields of intervention for children who are struggling, and we have attempted to include many of their voices as authors. In addition, we hope to educate students across disciplines and bring a sense of recognition to families with similar battles. This book is divided into three sections, with individual chapters telling the personal story of a child and family seeking help. The first part presents cases of children experiencing unexpected neurological insults resulting in interruptions in their development. The second part, by contrast, acquaints the reader with children experiencing unique patterns of development. Finally, the third part presents cases that raise questions, particularly with different interpretations from various points of view and by various areas of expertise. Each chapter, or story, is intended to both stand alone and contribute to the whole of the book. Please feel free to read selectively the chapters relevant to your interests, or start at the beginning and follow our retelling as we lead you along. An appendix is provided at the end of the book compiling all the different tests, or assessment measures, used throughout with brief explanations of how to interpret various scores. Key terms presented in chapters are defined at the end of each chapter. Some of these terms are unique to a case, while others may reoccur many times. Similarly, each chapter provides recommendations for intervention, and at times these recommendations may overlap across chapters. Much like the unique needs of the children represented here, we believe each chapter has unique needs for the presentation of information. Therefore, it was decided on a case-by-case basis, with the guidance of the authors who knew these children best, what terms, tests, recommendations, and information needed to be presented. We have endeavored for symmetry across the chapters, although we also hope the individual character of each child shines through. This book has been an exciting and challenging project. The initial invitation from Springer Publishing to consider a book such as this left us literally giddy with anticipation. The chance to contribute something to our field representing the hard work we commit to children and families every day was both daunting and awe inspiring. All the contributing authors responded with enthusiasm as they completed their chapters, seeming to find joy in letting go of their clinical role for a brief moment, instead connecting individually with and sharing these children’s stories. For us as editors and authors, we are immensely grateful to each one of you who worked with us. Your passion for each child’s story is what gives this book life. “Thank you” also to the families and children whose lives will help write the chapters of other families’ stories now; to our own families, for the support and love that make our personal stories so rich; to our faithful and tireless editorial assistant, Ann Tennier, without whom this book would never have taken form: Your support and organization made reality out of creativity. Most of all, thanks to all the students, professionals, parents, and families who work every day to create a better life for a child. Jennifer Niskala Apps Robert F. Newby Laura Weiss Roberts
Contents
Part I
Dangers of Childhood: Neurological Disorders 3
1
Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus Jennifer I. Koop
2
Never, Ever Shake a Baby: Pass It On . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 Amy K. Heffelfinger
3
Grand Larceny in the First Grade: Traumatic Brain Injury in the School-Aged Years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Jonathan E. Romain
4
A Slippery Descent: Adolescent Traumatic Brain Injury . . . . . . . . . . . . . . 33 Peter L. Stavinoha
5
Steamrolled: Sports-Related Concussions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 Michael Sharland and Thomas A. Hammeke
6
Bad Signs: Sickle-Cell Disease and Stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51 Kimberly Rennie Weissgerber and Amanda Epping
7
Life Interrupted: Medulloblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 Peter L. Stavinoha
8
The Mystery of the Falling Grades: Seizure Disorder . . . . . . . . . . . . . . . . . . 69 Lynn Bennett Blackburn
9
When Half a Brain is Better than One: Recurrent Seizures . . . . . . . . . . . 77 Lynn Bennett Blackburn
10
Pathological Left-Handedness: Stroke and Seizures . . . . . . . . . . . . . . . . . . . . 87 Elizabeth N. Adams
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Contents
11
Moving Forward: Psychotherapy with a Youth After Brain Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 Mary C. Kaven
12
Family Matters: Psychosocial Factors on Neuropsychological Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109 Grace W. Fong
13
Dog Attack: Physical Trauma with Associated Brain Injury . . . . . . . . 121 Jennifer Niskala Apps
14
Back to Life: Anoxic Brain Damage in a Near Drowning . . . . . . . . . . . . 129 John M. Oestreicher
Part II
How It Can All Go Wrong: Developmental Disorders
15
The Energizer Bunny Meets Shirley Temple: Attention Deficit Hyperactivity Disorder, Combined Type . . . . . . . . . . . . . . . . . . . . . . 141 Michael J. Zaccariello
16
‘‘He is not Working up to Potential’’: Atypical Attention Deficit Hyperactivity Disorder with Executive Weaknesses . . . . . . . . . . 151 Jennifer Niskala Apps and Dawn Pflugradt
17
Sorting Sounds: Reading Disability with Phonological Awareness Deficit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159 Robert F. Newby
18
Well Compensated But Never Quite Solved: Lingering Dyslexia . . . . 167 Robert F. Newby
19
Emily Confronts Her Fiercest Bear: Word Reading Disorder with Naming Speed and Phonological Deficits . . . . . . . . . . . . . . . . . . . . . . . . 175 Molly Drake Shiffler
20
A Tale of Two Assessments: Reading Fluency . . . . . . . . . . . . . . . . . . . . . . . . 191 Kara Lindstedt and Michael J. Zaccariello
21
Lost in Space: Nonverbal Learning Disability . . . . . . . . . . . . . . . . . . . . . . . . 201 Richard J. Clark
22
Beyond Diagnosis: Applied Behavior Analysis Treatment of Moderate Autism Spectrum Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217 Christopher P. Wiebusch
Contents
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23
When Quirks and Quick Learning Create a Quandary: Mild Autism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Elizabeth N. Adams
24
On Eggshells: Pediatric Bipolar Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245 Jennifer Niskala Apps and Dawn Pflugradt
25
It Helps to Know Genetic Basis: Williams Syndrome as an Example of Cognitive Disability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 255 Bonita P. Klein-Tasman, Frank J. Gallo, Kristin D. Phillips, and Kathi M. Fine
26
Mixed Bag: Tics, Compulsions, and More . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267 Kenneth L. Grizzle
Part III
Things that Go Bump in the Night: Interesting Questions and Controversies for Our Field
27
A ‘‘Sensational’’ Way to Understand and Serve Children: Illustration of a Sensory Processing Model . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 Winnie Dunn
28
Sense and Sensibility: Relating Behavior Control Issues with Self-Regulation of Sensory Input . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295 Robert F. Newby and Winnie Dunn
29
Elusive, Inclusive, or Conclusive? (Central) Auditory Processing Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307 Susan Oliff Carneol
30
A Little of This, A Little of That: (Central) Auditory Processing Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325 Lisa R. Cox
31
A Hunt for the Elusive Neuropsychological Impairment: Conversion Disorder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335 Michael J. Zaccariello
32
A Mystery of Perplexing Symptoms: Neuropsychological Assessment in a Case of Dysautonomia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 343 Robert F. Newby and Safwan S. Jaradeh
Appendix Description of Tests and Reported Scores . . . . . . . . . . . . . . . . . . . . . 351 Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 357
Contributors
Elizabeth N. Adams Minnesota Epilepsy Group, Saint Paul, MN Jennifer Niskala Apps Department of Psychiatry and Behavioral Medicine, Medical College of Wisconsin, Milwaukee, WI Children’s Hospital of Wisconsin, Milwaukee, WI Lynn Bennett Blackburn Departments of Neurology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI Susan Oliff Carneol Children’s Hospital of Wisconsin, Milwaukee, WI Richard J. Clark St. Francis Children’s Center, Glendale, WI Wisconsin School of Professional Psychology, Milwaukee, WI Lisa R. Cox Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Winnie Dunn Department of Occupational Therapy Education, University of Kansas Medical Center, Kansas City, KS Amanda Epping Medical College of Wisconsin, Milwaukee, WI Kathi M. Fine Department of Psychology, University of Wisconsin, Milwaukee, WI Grace W. Fong Children’s Healthcare of Atlanta, Atlanta, GA
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Contributors
Frank J. Gallo Department of Psychology, University of Wisconsin, Milwaukee, WI Kenneth L. Grizzle Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI Thomas A. Hammeke Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Amy K. Heffelfinger Departments of Neurology, Neurosurgery, and Pediatrics, Medical College of Wisconsin, Milwaukee, WI Safwan S. Jaradeh Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Mary C. Kaven Department of Psychiatry, Child and Adolescent Division, University of New Mexico School of Medicine, Albuquerque, NM Bonita P. Klein-Tasman Department of Psychology, University of Wisconsin, Milwaukee, WI Jennifer I. Koop Departments of Neurology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI Kara Lindstedt Department of Clinical Psychology, Marquette University, Milwaukee, WI Robert F. Newby Departments of Neurology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI John M. Oestreicher Affinity Medical Group, Appleton, WI Dawn Pflugradt Milwaukee, WI Kristin D. Phillips Department of Psychology, University of Wisconsin, Milwaukee, WI Laura Weiss Roberts Department of Psychiatry and Behavioral Medicine, Department of Population Health, Center for the Study of Bioethics, Medical College of Wisconsin, Milwaukee, WI
Contributors
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Jonathan E. Romain Children’s Hospital of Wisconsin, Milwaukee, WI, Department of Psychiatry and Behavioral Medicine, Medical College of Wisconsin, Milwaukee, WI Michael Sharland Department of Neurology, Medical College of Wisconsin, Milwaukee, WI Molly Drake Shiffler Department of Literacy and Language Development, Cardinal Stritch University, Milwaukee, WI Peter L. Stavinoha Children’s Medical Center, University of Texas Southwestern Medical Center, Dallas, TX Kimberly Rennie Weissgerber NeuroBehavioral Health Clinic, Glendale, WI, St. Luke’s Medical Center, Milwaukee, WI Christopher P. Wiebusch Wiebusch and Nicholson Center for Autism, Inc., Pediatric Neuropsychology and Treatment Associates, S.C., Pewaukee, WI Michael J. Zaccariello Division of Neuropsychology, Department of Neurology, Medical College of Wisconsin and Froedtert Hospital, Milwaukee, WI
Part I
Dangers of Childhood: Neurological Disorders
Children are perfect in their parents’ eyes. Although parents may know their children have flaws and weaknesses, their children complete them in a way that often blinds parents to those qualities. Parents’ love for their children is so strong and absolute that they forget all else. They defend their children fiercely and protect them passionately, and when their child is injured or hurt by something outside of their control, parents can be overcome. This section presents a series of cases in which children experience unexpected deviations in their otherwise normal development. Their parents are often beset by the severity and enormity of what has happened. Each parent’s question to the pediatric neuropsychologist is slightly different. Some are not ready to hear what their child now needs, while others cannot get the information quickly enough. In every case, managing the child’s and the family’s needs is a delicate balance. This section opens with a chapter about premature birth. According to the March of Dimes Web site, http://www.marchofdimes.com/, premature birth is the number one obstetrical complication in the United States. For parents first meeting their new child, prematurity adds a stressful and frightening chapter to their story. The next four chapters describe children experiencing head injuries from different circumstances. The developing brain can be injured in various ways, with a range of short and long-term sequelae. According to the Centers for Disease Control Web site, http://www.cdc.gov/ncipc/tbi/TBI.htm, brain injuries are the leading cause of death and disability in children and adolescents. Further, one-third of all pediatric injuries involve some form of brain injury. The causes of these injuries vary greatly by age and ethnicity. Under age 5, falls are the most frequent cause. Motor vehicle crashes are another common cause of these injuries. Mild traumatic brain injuries (TBIs) can occur with concussion, either because of injuries or sportrelated activities. Of children under age 14, an estimated 475,000 TBIs occur each year, and children under age 4 are at highest risk. In addition, brain injuries in infants are a particular concern when they result from Shaken Baby Syndrome. Although it is difficult to estimate the prevalence of this trauma, the National Center on Shaken Baby Syndrome approximates that 20% of infants presenting for medical care with these injuries will die, with the majority of the survivors having permanent brain injuries (http://dontshake.com/).
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Dangers of Childhood: Neurological Disorders
The following seven chapters present children with medical illnesses disrupting their development and resulting in neuropsychological sequelae. Many acute and systemic medical illnesses can have long-standing impact on the developing brain, resulting in neurological trauma. The National Cancer Institute reports that just over 3 of every 100,000 children will be diagnosed with some form of brain cancer (http://seer.cancer.gov/publications/childhood/). Not only can the experience of cancer itself result in neurological insults, but the treatments used for cancer, including radiation and chemotherapy, have documented long-term effects on a child’s mental capacities. Seizure disorders also cause neurological insults for children. According to the Epilepsy Foundation (http://www.epilepsyfoundation.org), each year 120,000 children under age 18 years will experience a seizure and 45,000 children will develop epilepsy. At any given moment 326,000 children in elementary school and junior high school have epilepsy. The neurological impact of this illness can range from mild to severe, depending on the location and type of seizure activity, seizure frequency and severity, and the medical interventions used to treat the seizures. The last two of these seven chapters highlight the impact such difficulties can have on families. When a child experiences an illness or injury, particularly a lifethreatening one, the dynamics of the family change. Parents work to protect and conserve their family’s identity while focusing on making things ‘‘better.’’ As neuropsychologists, we often must address the family’s needs in addition to the child’s cognitive, social, and emotional needs. Chapter 11 particularly highlights the interaction between neuropsychology and therapy. Chapter 12 also touches on the complications of the family’s vs. the children’s needs, while presenting a different etiology of developmental disruption. Chapters 13 and 14 relay stories of children experiencing more unusual accidents. Although many childhood accidents result in direct brain injuries, some result in indirect neurological insult. In these two chapters, injuries to the child’s body, through physical injury in one case and anoxia, or lack of oxygen, in the other, resulted in long-term neuropsychological sequelae. For some families, making sense of why such apparently random accidents happened to their child is perhaps the hardest part. As you read each chapter, you will find several tools to assist in your understanding of the information provided. Key terms are in bold face at their first use and defined at the end of the chapter, where references for both clinicians and families are also provided for those wanting more information about the condition(s) presented. Test scores from neuropsychological evaluations are provided in tables. The appendix at the end of the book lists the tests and describes normative test data and standardized scores.
Reference Langlois, JA, Rutland-Brown, W, Thomas, KE. (2005). The incidence of traumatic brain injury among children in the United States: Differences by race. Journal of Head Trauma Rehabilitation. 20(3), 229–238.
Chapter 1
Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus Jennifer I. Koop
Lucy was a miracle child. She overcame extreme odds and significant medical complications to become a spunky, chatty, child beauty pageant contestant. When she was referred for an evaluation at age 5 years by a neurosurgeon, she was doing well academically in a regular-education kindergarten classroom. However, both her parents and teachers acknowledged that she talked ‘‘too much’’ and had ‘‘selective hearing’’ problems. Directions at home and at school often needed to be repeated several times before Lucy would comply. Lucy appeared easily distracted, especially by her own thoughts or stories, which she enjoyed creating and telling to others. Lucy was also a social butterfly who was indiscriminant in her socialization and would approach anyone, even strangers, to begin a conversation. She was described as a very physically active but clumsy young girl who didn’t enjoy down time. Overall, though, Lucy was simply a happy and delightful little girl who was immediately liked by all who met her. Lucy was born 13 weeks early, after 27 weeks of gestation. Weighing less than 2 pounds at birth, her whole body practically fit in the palm of her father’s hand. Unfortunately, Lucy’s father wasn’t able to hold her as much as he would have liked during the first 3 months of her life. Lucy stayed in the neonatal intensive care unit for 11 weeks. She experienced medical complications typically associated with premature birth, including immature lungs, necessitating use of a ventilator for several weeks; patent ductus arteriosus, a heart malformation requiring ligation surgery; retinopathy of prematurity, requiring bilateral eye surgery; and jaundice, requiring light treatment. Most significantly, on day 3 of life, Lucy sustained a Grade III intraventricular hemorrhage affecting both cerebral hemispheres (Table 1.1). She subsequently developed hydrocephalus and underwent 11 spinal taps to control her intracranial pressure, but placement of a ventriculoperitoneal shunt was not deemed necessary at the time (Table 1.2). Once Lucy was discharged home, she was a mellow infant who slept and ate well. She seemed to develop normally. She walked and talked on time. She did not demonstrate any unilateral muscle weakness, and her motor skills appeared to be developing evenly. In fact, Lucy was described as an active toddler who was
J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008
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Table 1.1 Papile (1978) grading of intraventricular hemorrhages Degree of Description Symptoms on imaging studies severity Grade I Subependymal hemorrhage Blood observed in the area surrounding the vessels of germinal matrix only Blood products reabsorbed before dispersion into the ventricles or parenchyma Grade II Intraventricular hemorrhage Minimal dispersion of blood products into without ventricular dilatation lateral ventricles No distension of ventricles Grade III Intraventricular hemorrhage with Blood products evident in ventricular system ventricular dilatation Distension of ventricles evident on ultrasound Greater risk of development of hydrocephalus Grade IV Intraventricular hemorrhage with Blood products evident in ventricles and parenchymal involvement parenchyma Parenchymal involvement most often in frontal regions Distension of ventricles evident on ultrasound Greater risk of development of hydrocephalus
Table 1.2 Common complications of premature birth Respiratory distress syndrome Bronchopulmonary dysplasia (chronic lung disease) Necrotizing enterocolitis (gastrointestinal infection) Patent ductus arteriosus (cardiac malformation) Infection Hyperbilirubinemia (significant jaundice) Apnea (episodes of breathing arrest) Retinopathy of prematurity (visual abnormalities that can cause blindness) Intraventricular hemorrhage
constantly on the go. She was referred for early intervention therapies but was discharged shortly after starting because she demonstrated adequate developmental progress. Notably, Lucy demonstrated left-hand dominance for manual-motor tasks, despite the absence of any family history of left-handedness. Because of her prematurity, Lucy was evaluated by several neurologists and neurosurgeons over the years. At age 1 year, magnetic resonance imaging (MRI) of Lucy’s brain revealed abnormalities consistent with hydrocephalus, including grossly enlarged lateral ventricles with associated atrophy of the surrounding cortical tissue. However, given the lack of evidence of increased intracranial pressure and her apparent ‘‘normal’’ development and functioning, surgical intervention was not recommended. Lucy remained healthy. She was diagnosed with asthma, which is common in children who are born prematurely, but her condition was well controlled with medications. Repeated imaging studies over the first 4 years of her life suggested stability of the structural neurological abnormalities.
1 Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus
5
In fact, Lucy was only reevaluated by the neurosurgeon that referred her for neuropsychological testing after she was hospitalized for a tonsillectomy and adenoidectomy. The neurosurgeon’s question at the time of the evaluation was whether Lucy demonstrated any cognitive dysfunction that would suggest a need for surgical intervention to alleviate her persistently enlarged ventricles but presumed compensated hydrocephalus. Compensated hydrocephalus is thought to develop when equilibrium of intracranial pressure is reestablished despite a persistently greater than normal volume of cerebral spinal fluid within the ventricular system, possibly as a result of enlarged ventricular size.
Test Results Lucy underwent her first neuropsychological evaluation at age 5 years, 4 months. She was reevaluated on two other occasions, at age 6 years, 0 months, and age 6 years, 7 months, to monitor the stability of her cognitive profile. The measures administered on each occasion assessed general intellectual capabilities (Table 1.3), fine motor skills, attention and executive functioning, and language skills, and the third evaluation assessed memory functions (Table 1.4). Ideally, specific measures of visual-spatial skills also should have been included across all evaluations. However, Lucy’s age at the time of the initial evaluation greatly restricted the number of available standardized tests with appropriate normative data, resulting in the somewhat limited battery. Across all evaluations, Lucy presented as a very sociable and happy child who smiled frequently. She often initiated conversation with the examiner and talked almost incessantly unless redirected to the task at hand. Lucy was very imaginative and went into great detail when sharing stories. Her speech was significant for some mild articulation difficulties, particularly when pronouncing R, which had resolved by the time of the third evaluation. Overall, Lucy was cooperative and appeared to be putting forth her best effort on all tasks. She responded well to praise and Table 1.3 Intellectual Differential Abilities Scale – Upper Preschool Version
5 y, 4 m Scorea Percentile General conceptual ability score 89 23rd Verbal cluster 108 70th Verbal comprehension [51] 53rd Naming vocabulary [59] 83rd Nonverbal cluster 74 4th Picture similarities [33] 5th Pattern construction [40] 16th Copying [38] 12th Early number concepts [48] 45th a Standard score, (scaled score), [T-score], {z-score}.
6 y, 0 m Score Percentile 99 47th 117 87th [53] 61st [67] 96th 87 19th [50] 50th [43] 25th [39] 14th [46] 37th
6 y, 7 m Score Percentile 94 34th 116 86th [55] 69th [64] 92nd 80 9th [28] 1st [46] 34th [47] 38th [43] 24th
6 Table 1.4 Other cognitive functions Tests 5 y, 4 m Score NEPSY Sensorimotor domain 80 Imitating hand positions (11) Preferred hand Nonpreferred hand Visual-motor precision (1) Car Time Errors Motorcycle Time Errors Fingertip tapping (8) Repetitions Sequences Preferred hand Nonpreferred Attention/executive 74 domain Tower (8) Visual attention (5) Cats Time Omissions Commissions Faces Time Omissions Commissions Auditory attention/ (8) response set Attention task (9) Omissions Commissions Response task (6) Omissions Commissions Clinical Evaluation of Language Fundamentals – Preschool Receptive language 116 Sentence structure (13) Linguistic concepts (11) Basic concepts (14) Expressive language 108 Word structure (10)
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Percentile 9th 63rd 26th–75th 26th–75th 75th 75th 26th–75th 26th–75th
>75th >75th 3rd–10th
>75th >75th 26th–75th
26th–75th >75th 3rd–10th 25th
11th–25th 26th–75th 11th–25th 5th
>75th >75th 11th–25th 75th
(5)
37th 11th–25th 26th–75th 9th 11th–25th 26th–75th
(5)
86th 84th 63rd 91st 8th 50th
112 (13) (11) (12) 114 (16)
(6)
(12)
5th 3rd–10th 26th–75th 9th >75th 3rd–10th
(12)
79th 84th 63rd 75th 82nd 82nd
Not Administered
(11)
75th 26th–75th >75th 63rd 26th–75th >75th
(continued)
1 Beating the Odds: Prematurity and Posthemorrhagic Hydrocephalus Table 1.4 (continued) Tests Formulating labels Recalling sentences Total language Wide Range Assessment of Memory and Learning 2 Story memory Recall Recognition Verbal learning Recall Recognition Design memory Recognition Visual learning Delayed recall
5 y, 4 m Score (14) (10) 112
Percentile 91st 50th 79th
Not administered
6 y, 0 m Score (13) (8) 113
Percentile 84th 25th 81st
Not administered
7
6 y, 7 m Score Percentile
(11) (11) (13) (11) (10) (13) (9) (11) (7) 89
63rd 63rd 75th 63rd 50th 75th 37th 63rd 16th 23rd
verbally acknowledged that she was doing well on certain tasks. Thus, the results obtained during the evaluation were thought to provide a valid estimate of her cognitive functioning. Review of Lucy’s evaluation results begins with the data from the initial evaluation at age 5 years, 4 months. At that time, Lucy’s overall intellectual capabilities fell within the low average range. However, the overall score reflected a significant discrepancy between solidly average verbal abilities and below average or borderline visual-spatial or nonverbal abilities. Thus, the overall score is likely not an accurate estimate of Lucy’s true ability level. Within the nonverbal domain, Lucy had particular difficulty on two tasks, one that required her to recognize similarities between simple line drawings and one that required her to copy increasingly complex geometric figures. Lucy’s difficulty on the latter task was consistent with her performance on fine motor tasks. Her fine motor speed in simple finger tapping and motor planning in copying hand positions were intact. However, her visual-motor integration and fine motor coordination in completing mazes fell in the impaired range, reflecting poor motor control. Her attention skills also were mildly to moderately impaired. On a visual attention task (NEPSY Cats) she demonstrated a rapid and careless response pattern (as suggested by her speed of completion) and made a significant number of commission errors, suggesting poor impulse control. Her receptive and expressive language abilities were average, with little variability between tasks. Results of the second and third evaluations were generally consistent with those obtained during the first evaluation. Lucy continued to demonstrate a significant discrepancy between average to high average verbal skills and borderline to low average visual-spatial skills. She also continued to demonstrate fine motor difficulties, with evidence of increasing motor planning difficulties with her dominant
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(left) hand. Her attention skills also remained mildly to moderately impaired. Interestingly, and commensurate with her profile of verbal and nonverbal abilities, the addition of memory testing in the third evaluation revealed solidly average memory for verbal material but mildly impaired memory for visual-spatial material.
Formulation and Recommendations Lucy’s pattern of performance on measures administered during the neuropsychological evaluations revealed relative weaknesses and mildly to moderately impaired visualspatial abilities, fine motor skills, attention skills, and visual memory. This pattern of performance, the information gathered through clinical interview with Lucy’s parents, and Lucy’s medical history are consistent with two neurobehavioral syndromes. Lucy clearly demonstrated behavioral symptoms and impaired performance on attention measures that were consistent with Attention Deficit Hyperactivity Disorder, Combined Type (ADHD-C). As defined in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR) of the American Psychiatric Association (2000), ADHD is characterized by a pattern of at least six of nine symptoms of inattention and impulsivity or hyperactivity that have persisted for at least 6 months and significantly impact functioning in at least two domains. As outlined in the history section, Lucy demonstrated many symptoms of inattention, hyperactivity, and impulsivity since her early toddler years that continue to impact her functioning at home and school and also were observed behaviorally in the testing environment. Additionally, and perhaps more importantly, Lucy’s pattern of strengths and weaknesses was consistent with many aspects of a nonverbal learning disability (NLD). Although NLD is not a specific learning disability as defined by federal and most state departments of education, NLD is a syndrome defined by neuropsychological deficits that significantly impact learning. In general, children with NLD demonstrate weaknesses and difficulties in visual-spatial processing, visual-motor coordination, tactile perception, attention, abstract reasoning, problem-solving, perception of emotions, and social communication. Academic problems in math, reading comprehension, and the mechanics of written language are common. In contrast, basic language processing skills, rote verbal memory, reading word recognition, and spelling are usually relatively strong. The cognitive profile of NLD is presumed to reflect impairment in nondominant hemisphere functions, which theoretically are most dependent upon white matter tract integrity. The neuropsychological profile described earlier and demonstrated by Lucy is commonly observed in children with a history of prematurity and/or hydrocephalus. Prematurity is presumed to be associated with attention and motor impairment because the normal development of these functional systems is disrupted by the trauma of preterm birth. This disrupted development is thought to be further exacerbated by the numerous medical interventions that premature infants typically experience (e.g., ventilation, repeated exposure to anesthesia for surgeries, related potential hypoxic episodes). The ventricular enlargement and increased intracranial
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pressure of hydrocephalus repeatedly have been associated with deterioration of white matter tract integrity and even delays or decreases in myelination. Perhaps the most important aspect of Lucy’s repeated neuropsychological testing is the suggestion of changes in motor, attention, and adaptive behavioral functioning. There is some evidence that neuropsychological testing is more sensitive to subtle changes in neurological status for children with unshunted, compensated hydrocephalus than other neurological techniques (e.g., computerized tomography (CT) imaging of changes in ventricular size or external monitoring of intracranial pressure). Thus correlation of the neuropsychological findings with other clinical indicators of neurological status (i.e., imaging studies) was recommended to best appreciate the significance of the observed variability and potentially clinically significant changes in cognitive performance over an 18-month period. The data from the repeated evaluations were shared with the neurosurgeon who had referred Lucy for the initial evaluation. After he had considered the neuropsychological results, especially those obtained during the third evaluation, and correlated them with neuroimaging and physiological data, the neurosurgeon recommended a surgical procedure called endoscopic third ventriculostomy, in which intracranial pressure is reduced by draining some cerebral spinal fluid from the third ventricle. Given Lucy’s cognitive profile and reported academic difficulties, her parents were encouraged to request a meeting with school personnel to develop and implement an appropriate Individualized Education Program (IEP). Once the school personnel determined that Lucy was eligible under federal criteria to receive an IEP, specific modifications and accommodations within the regular education classroom could be made. Lucy’s fine motor and visual-spatial weaknesses suggested that she would benefit from occupational therapy to strengthen her skills in these areas. Fine motor and visual-motor integration skills are particularly important during the early elementary school years because students are taught to print and to write in cursive, and an increasing number of assignments are dependent upon a student’s ability to provide written responses. Lucy’s visual-spatial difficulties impacted her ability to perform many demands of school. Although specific examples of modifications are provided, the overarching recommendation was for school personnel to utilize and emphasize Lucy’s relatively stronger verbal skills to compensate for her visual-spatial difficulties whenever possible. Copying problems or other assignments from a book or chalkboard or writing at length will be very effortful. It was suggested that the requirements for writing be reduced and that some of Lucy’s assignments and testing be oral. As she progresses in school, Lucy should begin to receive training in the use of a computer and word processor to help her prepare her assignments. Explicit directions simply given orally will be difficult for Lucy if she does not know how to proceed perceptually. She may need modeling and/or direct teacher intervention. Reading graphs, maps, and charts may be difficult. Lucy may need individual help in interpreting these educational materials, primarily though verbal explanations accompanying the visual material.
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Mathematics instruction should include practical and hands-on tasks. Lucy will need help with measuring devices and estimating time, size, and distance. Her visual-spatial impairment suggests that when she learns computational procedures, she may need to divide her sheet into columns to facilitate placement of the numbers. Lucy’s attention deficits will require additional instructional modifications to maximize her participation in and benefit from academic instruction. She will require more frequent attentional cueing and support for her relative lack of organizational skills. Redirection should be provided in a subtle positive manner, such as a tap on the shoulder or hand on the desk. Preferential seating close to the teacher or near the front of the classroom will likely help reduce distractions and position Lucy in a location that facilitates frequent redirection from or checking in with the teacher. Complex tasks should be broken down into smaller sequential steps. Instructions should be given one at a time, and longer sets of instructions should be broken down, repeated, rehearsed, or written down for Lucy. Additional instruction in organizational skills such as outlining, diagramming, or planning the sequence of steps to complete a task would be helpful. It is always difficult to predict cognitive outcomes for young children, especially in the context of a potentially progressive neurological condition. However, if Lucy’s hydrocephalus remains stable and does not progress and result in increased intracranial pressure, it would be presumed that her cognitive profile also would remain stable. That is, she will likely continue to require interventions for ADHD and NLD. Use of a stimulant or other attention-enhancing medication may be warranted at some time to improve her attention capabilities. The underlying neurological cause of Lucy’s attention difficulties, however, may make pharmacological intervention more difficult. With regard to the NLD symptoms, Lucy will likely continue to demonstrate a pattern of verbal strengths and visual-spatial weaknesses that will impact her academic performance.
Additional Resources Key Concepts and Terms Hydrocephalus Accumulation of cerebral spinal fluid within the ventricular system causing dilatation of ventricles and potentially increased intracranial pressure, which can compress brain tissue. In early childhood, hydrocephalus also often results in enlargement of the head circumference. Otherwise known as ‘‘water on the brain.’’ Intraventricular hemorrhage (IVH) A common complication of premature birth prior to 32-weeks gestation. IVH involves bleeding within the germinal matrix surrounding the lateral ventricles. Hemorrhages range in severity and are graded from I to IV, with IV being the most serious. Hydrocephalus develops following
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IVHs in 55% of cases with Grade III and up to 80% of cases with Grade IV hemorrhages. Ventriculoperitoneal shunt The most common treatment for hydrocephalus is placement of a shunting device into the ventricle. The ventriculoperitoneal shunt is attached to a catheter positioned to end into the abdomen, diverting excess cerebral spinal fluid into the abdominal cavity.
References Resources for Clinicians American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders: DSM-IV-TR. Washington, DC: American Psychiatric Association. Dykes, F. D., Dunbar, B., Lazarra, A., & Ahmann, P. A. (1989). Posthemorrhagic hydrocephalus in high-risk preterm infants: Natural history, management, and long-term outcome. The Journal of Pediatrics, 114(4 Pt 1), 611–618. Fletcher, J. M., Brookshire, B. L., Landry, S. H., & Bohan, T. P. (1996). Attentional skills and executive functions in children with early hydrocephalus. Developmental Neuropsychology, 12 (1), 53–76. Fletcher, J. M., Francis, D. J., Thompson, N. M., Davidson, K. C., & Miner, M. E. (1992). Verbal and nonverbal skill discrepancies in hydrocephalic children. Journal of Clinical and Experimental Neuropsychology : Official Journal of the International Neuropsychological Society, 14 (4), 593–609. Fletcher, J. M., Landry, S. H., Bohan, T. P., Davidson, K. C., Brookshire, B. L., Lachar, D., et al. (1997). Effects of intraventricular hemorrhage and hydrocephalus on the long-term neurobehavioral development of preterm very-low-birthweight infants. Developmental Medicine and Child Neurology, 39(9), 596–606. Papile, L. A., Burstein, J., Burstein, R., & Koffler, H. (1978). Incidence and evolution of subependymal and intraventricular hemorrhage: A study of infants with birth weights less than 1,500 gm. The Journal of Pediatrics, 92(4), 529–534. van de Bor, M., & den Ouden, L. (2004). School performance in adolescents with and without periventricular–intraventricular hemorrhage in the neonatal period. Seminars in Perinatology, 28(4), 295–303.
Resources for Families Hydrocephalus Association. http://www.hydroassoc.org/ Linden, D. W., Paroli, E. T., & Doron, M. W. (2000). Preemies: The essential guide for parents of premature babies. New York: Pocket Books. March of Dimes: Pregnancy, babies, prematurity. http://www.marchofdimes.com/ March of Dimes: Prematurity. http://www.modimes.org/prematurity/prematurity.asp
Chapter 2
Never, Ever Shake a Baby: Pass It On Amy K. Heffelfinger
At that moment, the sun stopped shining and our happy, carefree world came crumbling down. We were told our healthy 4½-month-old baby boy was in critical condition and could die. Our son, Jeremy, had just learned to smile about a week before, and now his smile was gone. I remember two doctors addressing me and my family in a small room. Their faces were without expression. They announced that my son was the victim of shaken baby syndrome (SBS) and might die. I immediately looked at my mother, a retired nurse, for some explanation. My mom began to sob; the pain in her face is still etched in my mind. I now knew how bad it was, but I still had never heard of SBS. Now Jeremy was in a drug induced coma, and we waited for the swelling in Jeremy’s brain to stop. We were told that it would stop swelling in 3 days. Four days after Jeremy was injured, his brain continued to swell, permanently destroying healthy brain cells. He was so swollen that the doctors could not open his eyes to check his pupils. Two huge vials of fluid were removed from the top of Jeremy’s head to reduce the pressure. He had two blood transfusions. A respirator kept him alive. After a week, Jeremy began to seize and screamed for 14 hours straight until he had to be sedated. This went on for several days. He was inconsolable. Jeremy was put on phenytoin, an antiseizure medicine. To measure the levels of this medicine, a daily blood culture needed to be done. Every morning he was awakened at dawn to have blood drawn from his tiny arm, a terrifying routine. On the 21st day, we went home. We were told how severe his injury was and that he was blind in both eyes due to retinal hemorrhages. We did not know what was to come, only that the road ahead would be long and hard. We only thanked God that we still had our precious baby. Jeremy escaped death by a heartbeat, thanks to the skill of professionals, modern technology, and the fact that heaven was flooded with prayers, and continues to be, on his behalf. During the first year after his injury, Jeremy had three brain surgeries and to this day endures daily physical, occupational, and speech therapies. His future, and what he will be capable of, is unknown. Our son has had to fight and work extremely hard each and every day of his short life. Time has helped our family heal and accept what happened to our precious child. His hair now covers the 7-in. scar on his head. Although he is very sensitive to light, we know that he can see. He doesn’t use his hands like he should; they hang by his side for convenience. We have to continue to tell him to walk because his brain and his feet do not have the complete system down yet. Someday. We have extremely high expectations for Jeremy because he has continued to prove his doctors wrong. We are blessed with a close and loving family; all are involved in his care and devoted to his well-being.
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A.K. Heffelfinger We travel this profound journey together as a family with Jeremy leading the way. He is carving a path for us to meander along, different from traditional roads. Still, it echoes with the sounds of joy and laughter bursting forth from our precious little boy. He is a delightful, amazing child with much to say, and he sure says it. He is Auntie’s Sweet Pea. He is Grandma’s Darling Boy. He is Dad’s Little Buddy. He is Mom’s Angel. He is our miracle.
History and Reason for Referral Jeremy, a 6-year-old boy, was referred for his third neuropsychological evaluation due to nonadherence to rules and poor attention. He was initially referred at age 4 years by his neurologist due to a history of traumatic brain injury (TBI) due to SBS. Jeremy was found unresponsive at the babysitter’s home when he was 4½months old. Initial stabilization included intubation and ventilatory support. For 3 days, he presented comatose, with upper extremities rigid and fists clenched, but was exhibiting reflex of lower extremity movements to pain. He had increased intracranial pressure, and he received a subdural peritoneal shunt, which drains from subdural space into the abdominal cavity. He was in the pediatric intensive care unit for 1 week and in the hospital for 3 weeks. Discharge diagnoses included SBS, right frontal parietal subdural hemorrhage, retinal hemorrhages, and posttraumatic seizures. As in many cases with SBS, a previous injury was evident on brain scans following the events precipitating hospitalization. Phenytoin was prescribed and discontinued 1 year after the injury. Jeremy’s retinas recovered, but cortical damage in visual areas was suspected. He received sensory integration therapy, occupational therapy, physical therapy, and speech and language therapy through the Birth to Three Program until his third birthday. He attended a 4-year-old kindergarten program for 2 years and received occupational and physical therapy services. Jeremy’s development of course had been interrupted due to the severity of the abuse. Jeremy’s prenatal development, delivery, and early development were unremarkable. He was smiling, rolling, and holding his head up before being shaken, but he lost these skills. He never crawled but sat at 13 months and walked at 14 months. His gait was abnormal, and he did not use his hands when walking. His language development was reportedly a strength, and he was speaking several words by age 1 year. Emotionally, he was often inconsolable following the injury. He was highly sensitive to textures and touch, not liking high levels of physical affection. He also was hypersensitive to sights and sounds, although his hearing, taste, and smell were believed to be acute and nondisrupted. Jeremy was afraid of animals and sleeping alone. He was able to verbalize his fears by commanding what to do in situations. In fact, Jeremy often required control in situations. He was easily disrupted by change and required routines to be followed. He was very attentive to detail and could be
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emotionally frustrated if the doors were not closed or if his clothing was not as he would like. Jeremy’s parents prepared him for transitions, using timers to indicate when change would occur. They also adapted to his routines. Socially, Jeremy had been disinterested in peers until he began school. Jeremy lived with his mother, a part-time product manager; his father, a teacher; and his 3-year-old sister. His family history was significant for some obsessive– compulsive tendencies in his father. Neuropsychological testing at ages 4 and 5 years indicated several residual neuropsychological impairments, particularly with visual perception, ataxic and apraxic motor difficulties, attention, and executive functioning. Jeremy also had trouble in areas that would require integration of different skills, such as picture naming and visuospatial construction. He had close to age-appropriate strengths in verbal knowledge. There were ongoing concerns regarding his oppositional and controlling behaviors. Jeremy had been working with a psychologist, focusing on social skills training. His parents reported that his therapy had been very successful in helping them to play with him and also with his social development. Jeremy was to attend full-day kindergarten with the help of an aide, shared among three students, as well as pullout occupational therapy services three times a week to work on fine motor skills. Physical therapy services would be available as needed. He had been attending the ‘‘Handwriting Without Tears’’ program to work on early writing skills. Jeremy’s parents’ current concerns were regarding how his behavior was hindering his advancement in many areas. Both his teachers and his parents did not know what he was capable of doing. He was constantly verbally negotiating tasks he did not want to participate in or he would procrastinate. For example, in therapy he would make deals such as ‘‘I will do it if you will do it.’’ If he were threatened with something such as ‘‘we are going to call your mom,’’ he would then immediately complete the tasks. This type of behavior occurred at home also. Jeremy’s parents’ felt that his attention had improved and that he was now able to focus while he was playing. However, although he was able to focus on things that he was interested in, he continued to be unable to focus on tasks that he did not want to do. Emotionally he was less fearful than at previous evaluations, but he continued to have a more fearful personality compared to same-age peers. His parents were very concerned with his emotional functioning. Daily he would cry for 30 to 60 minutes if he did not get his way or if someone took his toy. They had not found ways to soothe him other than to give him his way. They noted that even though his sister was only 3, she was able to verbally express how she felt, but he was unable to do this. Socially, he still had difficulties with same-age peers, but he had increased social interests and interactions with them, which was a significant improvement. Cognitively, Jeremy’s parents recognized that he continued to be delayed but reported that he was making gains. For example, he knew his alphabet and could recognize some letters, and he could count to 30 but could not recognize numbers. He continued to have a significant tendency to perseverate on things such as motors or things that have wheels.
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Test Results The threefold purpose of this assessment dictated the assessment battery. First, because Jeremy had a longstanding history of problems with emotional and behavioral regulation, it was essential to focus on whether there were actual neuropsychological impairments in attention and executive functioning to help explain his difficulties. Second, it was important to determine the magnitude and rate that he was gaining neuropsychological skills, using data from the previous evaluations for comparison. Third, because he was now 6-years old, comprehensive assessments of cognition and memory were conducted to understand more fully his neuropsychological functioning. Because of his given difficulties with basic sensory processing and attentional focus, qualitative observations were carefully made. Jeremy presented for two evaluations, and his behavior was consistent. He was unable to separate from his parents, becoming tearful when attempted. However, at one time during the testing, his father was able to leave the room, and Jeremy functioned adequately. Each week Jeremy was hesitant at first but quickly warmed up to the examiners. His behavior was notable for requiring numerous prompts and reminders to look at visual stimuli to make sure that he had seen them before responding. He also needed frequent redirection to remain on task, but he was not hyperactive, nor did he leave his seat. It was clear that he wanted to do well the majority of the time, but there were occasions where he appeared to answer incorrectly to avoid the testing situation. He was very socially engaging and enjoyable to work with. He continued to have difficulties manipulating his hands, especially on command. On the basis of these observations, the results were believed to be an accurate representation of his current level of functioning (Table 2.1). Specific tests of processing speed (e.g., coding, symbol search) were not given because of Jeremy’s significant difficulties with visual processing and motor control; therefore, a Full Scale IQ was not calculated. Overall, Jeremy’s verbal abilities were in the low average range and represented a significant strength. In contrast, his overall nonverbal/visuospatial skills were in the mildly mentally deficient range. His working memory, the ability to hold and mentally manipulate information, was in the range of borderline impairment (Table 2.2). These findings were consistent with those from his previous general cognitive testing when he was 4 years old and continued to document significantly stronger verbal abilities in comparison to weaker nonverbal/visuospatial skills. Jeremy continued to have a significantly difficult time completing the motor tasks that were administered to him. On a measure where he was to tap his fingers rapidly together and then tap his fingers in a sequence, his performance was severely affected by his attention in that he was unable to tap consistently for the time allotted. He also was unable to complete the motor program to tap his fingers in a sequence. On another task, he was to demonstrate motor planning by copying finger position movements. This task was discontinued because Jeremy was unable to do most of the hand positions. These findings suggested that Jeremy continued to exhibit constructional apraxia, that is, he had difficulty using his hands, copying
2 Never, Ever Shake a Baby: Pass It On Table 2.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Verbal Comprehension Index Perceptual Reasoning Index Working Memory Index Similarities Vocabulary Comprehension Block Design Picture Concepts Matrix Reasoning Digit Span Letter-Number Sequence Coding Symbol Search a Standard score, (scaled score), [T score], {z score}.
Table 2.2 Other cognitive functions Tests NEPSY Cats Time Omissions Commissions Attention Omissions Commissions Tower Arrows Memory for faces Kaufman Assessment Battery for Children Gestalt closure Wide Range Assessment of Memory and Learning – Second Edition Verbal learning Delayed recall Recognition Story memory Delayed recall Recognition Design memory Recognition Sentence memory
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Scorea 81 59 74 (6) (7) (7) (4) (2) (4) (5) (6) (8) (10)
Score
Percentile 10th 1st 4th
Percentile
2nd–10th 16th 91 87 93 >16th [66] [64] [62] [66] [69] [56] [50] (continued)
56 Table 6.4 (continued) Tests Organization of materials Monitor Metacognition index Global executive composite Beery-Buktenica Developmental Test of Visual-Motor Integration Visual perception Motor coordination Grooved Pegboard Test Dominant hand (left) Nondominant hand (right)
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Score [45] [59] [57] [61] 74 77 78
Percentile
85 51
developing child, with intact intellectual functioning, typically maintains his or her level of cognitive functioning over time. Moreover, silent infarctions are associated with an overall dampening of general cognitive ability and weaknesses in graphomotor ability, such as seen on Johnny’s follow-up evaluation. Thus, it is likely that the decline observed in his intellectual ability was secondary to his silent infarction. In the absence of his infarct or other injury to his brain, Johnny’s intellectual functioning would have likely remained in the average range. Additionally, the new concerns regarding Johnny’s emotional and behavioral development are likely related to his infarction. Johnny’s stroke was located in the prefrontal cortex, which is located at the front part of the brain and associated with the regulation of behaviors and emotions. Difficulties in response inhibition, task initiation, mood regulation, and behavior that Johnny’s mother reported during his follow-up evaluation were generally consistent with the difficulties faced by individuals who suffer from injuries to the prefrontal cortex. Finally, the new onset of motor difficulties noted in Johnny’s right hand was also consistent with the location of his left-sided infarction. Given Johnny’s medical history and the cognitive difficulties that arose as a result of his stroke, a number of recommendations were advanced. Specifically, it was recommended that an eligibility meeting be held to determine his qualification for an Individualized Education Plan (IEP) under the Other Health Impairment (OHI) category, because his silent infarction may have played a role in his academic failure. Because Johnny’s new onset of academic difficulties likely also stemmed from his stroke, it was recommended that he not be retained. Instead, recommendations included special education services under the OHI category to include specialized instruction in a small group setting. Because of the change in his level of intellectual ability, Johnny would likely have a more difficult time acquiring and mastering new skills and concepts. It was important to get those working with Johnny to understand that although he looked the same, expectations for him had to be modified and extra academic support would be required for him to have academic success. Given the presence of fine motor and graphomotor difficulties, recommendations were made that Johnny receive an occupational therapy evaluation at school to
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determine his need for services in this area, with a goal of increasing his writing efficiency. In the meantime, it was recommended that he be provided with classroom notes to limit the demands on his already strained graphomotor skills. Consultation with a mental health professional to address emotional and behavioral concerns was also advised. Specifically, consultation with a child psychologist with expertise in working with children who have suffered from brain injuries was suggested to address anger management and coping skills. At this point, medication was not recommended; however, if emotional and behavioral concerns did not improve over time, medication management would become an option. Finally, in light of his sickle-cell disease and silent infarction, it would be important to continue to monitor Johnny’s functioning over time. Most children with sickle-cell anemia are followed closely by their pediatric hematologists at least once a year. Those who have more severe complications, such as Johnny, are followed more closely (approximately once a month when they come in for their monthly blood transfusions). In addition to his medical needs, it would also be important to monitor his neuropsychological functioning over time. Unfortunately, as was the case with Johnny, silent infarctions are typically not noticed right away. Thus, children may suffer ‘‘silently’’ for years without anyone knowing that they have experienced a stroke. By monitoring their neuropsychological functioning, changes can be detected and accommodations can be made and/or adjusted as necessary.
Additional Resources Key Concepts and Terms Executive Functions Include self-regulatory skills that affect an individual’s planning, flexibility, generation of information, inhibition of impulses, and working memory (i.e., the ability to hold information in memory while performing some operation on it or manipulation using it). The ability to regulate emotions and attention are also key hallmarks of executive functioning.
References Resources for Clinicians Day, S., & Marion, S. B. (1996). Educator’s guide to sickle cell disease. Memphis, TN: St. Jude Children’s Research Hospital. Kirkham, F. (2007) Stroke and cardiovascular disease in childhood. Cambridge, MA: Blackwell. National Heart Lung Blood Association, www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs. html. Provides information about sickle cell disease and engages in research for sickle cell disease.
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Sickle Cell Disease Association of America, (1998). Parent/teacher guide: How parents and teachers can work together to achieve school success for children with sickle cell anemia. Baltimore, MD.
Resources for Families Children’s Hemiplegia and Stroke Association, www.chasa.org/. A non-profit support group for parents and families of children who have had a stroke. Earles, A., Lessing, S., & Vichinsky, E. (Eds.). (1993). Parents’ handbook for sickle cell disease: Part II: Six to eighteen years of age. Berkley, CA: State of California Department of Health Services. Lessing, S., & Vichinsky, E. (1998). Parents’ handbook for sickle cell disease: Part I: Birth to six years of age. Oakland, CA: State of California Department of Health Services. Sickle Cell Disease Association of America, (1998). Parent/teacher guide: How parents and teachers can work together to achieve school success for children with sickle cell anemia. Baltimore, MD: Sickle Cell Disease Association of America. Sickle Cell Disease Association of America, www.sicklecelldisease.org/. Dedicated to finding a cure for sickle cell disease. The Web site provides general information, research updates, and links to additional resources.
Chapter 7
Life Interrupted: Medulloblastoma Peter L. Stavinoha
Anthony’s parents had been planning a family trip to the coast for months. Anthony, a precocious 4-year-old with excellent language skills, had been to the beach once before and could not stop talking about the upcoming trip. On a cool Friday morning in autumn, Anthony’s mother was busy packing the family’s things for their 2-week vacation. She had decided to take the day off from work while Anthony was in day care so that she could take care of all the last-minute details. When the day-care staff called to report that Anthony did not seem right, she figured he might have come down with an illness that would make the plane ride less pleasant, but she certainly did not anticipate that the vacation would never happen. When she arrived at the day care, she learned that Anthony had been having problems with balance all morning and had fallen several times. She also noticed that his eyes were moist and that his head was tilted to one side. ‘‘Tears are falling from my eyes, Mommy,’’ said Anthony with only a slight hint of distress in his voice but still looking to his mother to somehow make it stop. Anthony’s mother felt her heart jump and quickly scooped up Anthony and shuttled him to the emergency department. Within an hour, Anthony had been sedated and was undergoing a Magnetic Resonance Imaging (MRI) scan. Within 4 hours, a neurosurgeon was describing to Anthony’s parents the lemon-sized mass that had shown up on the MRI in the posterior fossa, the area of the brain that houses the cerebellum and critical brainstem structures. The neurosurgeon was personable but very serious, and Anthony’s parents consented to the recommended treatment, which consisted of creating a hole in the back of Anthony’s skull to remove the tumor. By Sunday morning, the family’s vacation was a distant memory, and the neurosurgeon told Anthony’s parents that the surgery had gone well. They held Anthony’s hand while he was in the intensive care unit, counting their blessings that their child had survived, without realizing that the battle was not over. Samples of the tumor tissue were analyzed and identified as medulloblastoma, a malignant and aggressive type of Primitive Neuroectodermal Tumor (PNET). As Anthony recovered from neurosurgery and became increasingly alert and active, his parents were learning about the risks and benefits of the proposed treatment plan. Specifically, they learned that Anthony would need to undergo a course of radiation
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therapy in which radiation would be delivered directly to his brain and spine, and they also learned about the months of chemotherapy that Anthony would have to endure following cessation of radiation. Still in shock from their realization of how close Anthony had been to death, his parents did not really process some of the risks stated to them with regard to radiation and chemotherapy, including declines in IQ, learning problems, hair loss, and growth suppression. They also did not heed, or perhaps even hear, the recommendation that they should have Anthony evaluated by a neuropsychologist in the coming years in order to keep close tabs on his neurocognitive development in light of the tumor and its treatments. Fast-forward 8 years, and Anthony was in the middle of fifth grade and struggling significantly. For years, Anthony underwent routine MRI scans and endocrine treatments (e.g., growth hormone), and his parents were always relieved that there was no recurrence of the tumor. For kindergarten through fourth grade, Anthony’s parents had enrolled him in a small private school, where they believed that he would get any extra attention he might need. The teacher-to-student ratio was low, and Anthony had a series of kind teachers who always seemed to have a soft spot for this first child they had ever known who had experienced a brain tumor. Anthony’s parents felt that they were not being overprotective but simply providing Anthony with a school placement that would give him plenty of time to mature as they harbored lingering concerns about his ability to integrate successfully in an overcrowded public school system that they perceived as overwhelming. They always felt reinforced when his report cards said that he was making satisfactory progress and underscored what a sweet and compliant child he was. Anthony’s parents were well-educated professionals who had an inkling that he was not as strong in math as perhaps other students in his grade might be, and they also noticed that he moved a little slower and seemed a little more awkward than many other children of his age, but otherwise the consistently positive teacher comments reinforced their belief that Anthony was progressing well. At the end of fourth grade, Anthony’s father was transferred by his employer, and the family had to move. Anthony’s parents were devastated that he would no longer be able to attend the private school where he had seemed so comfortable, but circumstances dictated that they seek a new educational placement for Anthony. After exhausting all options, Anthony’s parents determined that the only placement for him would be their local public elementary school. They felt confident that Anthony had progressed well over the years, and they knew that the elementary school was highly rated. So with only a little trepidation, Anthony’s parents enrolled him in the fifth grade. Within the first few weeks, it was clear that things were not going well. Anthony’s teacher already had sent notes home about unfinished work in the classroom, and Anthony was complaining that he had no friends and that the work was very hard for him. He wanted to do well and please his teachers, so he was adamant about completing all of his homework each night, though this was taking hours, to the point of significant fatigue. Anthony’s parents were particularly distressed after receiving results of benchmark testing showing that Anthony was not keeping up with his classmates, particularly in math.
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A parent–teacher conference was called 5 weeks into the school year, and Anthony’s parents were shocked to learn how far he was behind typical fifth grade students. School staff indicated that they would like to do some testing with Anthony to see whether he needed more intensive school services, such as those offered through special education. Anthony’s parents were dismayed at the suggestion and could not even fathom that their child could be a special education student. After a few days of soul-searching and watching Anthony become more and more unhappy and even fearful of going to school, Anthony’s parents recalled the neurosurgeon’s suggestion of a thorough neuropsychological evaluation. They discussed this with the school, and it was determined that, given Anthony’s history of brain tumor, a neuropsychological evaluation would be the most appropriate starting point for determination of needs at school.
Test Results Dual priorities dictated the scope of the neuropsychological evaluation. First, Anthony had never undergone neurocognitive evaluation in the past, even though this had been recommended to his parents. Because several patterns of findings in children who have experienced medulloblastoma and subsequent radiation and chemotherapy treatments are common (such as decreases in IQ, slower processing speed, and difficulties with attention and memory), a broad group of tests was selected. Second, the practical issue of identifying Anthony’s educational needs as well as helping to elucidate the most appropriate educational placement for Anthony within the public school dictated that the evaluation includes measures that would satisfy the multidisciplinary committee that would ultimately plan Anthony’s educational placement. The initial interview with Anthony’s parents illuminated their conflicted feelings about his progress. On the one hand, they seemed sensitive to the fact that he was not as well-developed cognitively, socially, and academically as many other children of his age, but on the other hand, they were holding dearly to their pretumor picture of Anthony as a bright, energetic, and creative boy with limitless potential. Anthony was a bit wary of the testing situation at first, but he quickly settled in and seemed to enjoy the one-to-one attention. He possessed physical characteristics common to children who have undergone craniospinal radiation during early childhood, including hair loss and short stature. His movements tended to be relatively slow and cautious, but he seemed to be in a good mood. He fatigued relatively easily, and was given more breaks than is typical during the course of an evaluation. In fact, he worked rather slowly and needed so many breaks that testing had to be completed over two separate sessions. Anthony appeared to have mild difficulty with attention, though his behavior was not at all impulsive. He seemed very eager to please the examiner and to perform well on testing, and at times he even seemed mildly anxious when he was not able to complete a task within time limits.
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Parent and teacher behavioral ratings indicated social difficulties that seemed more prominent at school than home. In addition, Anthony was judged to be more withdrawn and anxious in his classroom environment than most boys of his age, though Anthony’s parents did not raise the same concern about anxiety at home except when it came to homework activities. Mild difficulties with attention and concentration were also noted at school, though again these difficulties were not reported as prominent at home. The evaluation (Tables 7.1–7.3) revealed that Anthony was experiencing a number of common late effects of treatment for medulloblastoma. In particular, his neurocognitive pattern was characterized by a significant discrepancy between verbal information processing abilities and nonverbal abilities, with his composite IQ falling below average. In contrast to average verbal abilities, Anthony exhibited significant weaknesses across tasks involving visual-spatial information processing as well as processing speed and cognitive efficiency. Auditory working memory was low average, with Anthony exhibiting deterioration of working memory with increasing complexity of information. Evaluation of learning and memory functions suggested a pattern congruent with measurement of Anthony’s overall cognitive ability. Specifically, his capacity to take in, store, and later retrieve languagebased information was much better developed than his nonverbal, visual-spatial memory functions. Visual-motor skill development was well below average, and Anthony had difficulty both with visual-perceptual functions as well as bilateral motor dexterity. His performance on tasks tapping executive functions was suggestive of difficulty with both abstract concept formation and cognitive flexibility. Anthony tended to have difficulty generating appropriate problem-solving
Table 7.1 Intellectual Wechsler Intelligence Scale for Children – Fourth Edition Full Scale IQ Verbal Comprehension Index Similarities Vocabulary Comprehension (Information) Perceptual Reasoning Index Block Design Picture Concepts Matrix Reasoning Working Memory Index Digit Span Letter-Number Sequencing Processing Speed Index Coding Symbol Search a Standard score, (scaled score), [T-score], {z-score}.
Scorea 77 96 (9) (10) (9) (11) 73 (5) (7) (5) 83 (8) (6) 73 (4) (6)
Percentile 6th 39th
4th
13th
4th
7 Life Interrupted: Medulloblastoma Table 7.2 Academic achievement Woodcock Johnson Tests of Achievement – Third Edition Academic skills Academic fluency Academic applications Broad reading Letter-word identification Reading fluency Passage comprehension Broad math Calculation Math fluency Applied problems Broad written language Spelling Writing fluency Writing samples
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Score
Percentile
Grade Equivalent
93 78 85 90 101 82 92 78 77 74 82 87 97 76 91
33rd 7th 15th 25th 52nd 12th 29th 7th 6th 4th 11th 20th 42nd 5th 26th
5.2 3.6 3.9 4.7 6.7 3.6 4.5 3.5 3.5 3.7 3.5 4.5 5.7 3.6 4.3
strategies and benefiting from direct feedback in order to modify his problemsolving approach. Academically, Anthony exhibited patterns of relative strengths and weaknesses that correlated with his underlying neurocognitive capacity. Specifically, his overall reading skill development was measured in the low end of the normal range, with his composite written expression performance falling in the low average range. Interestingly, Anthony’s strongest language-based performances were on tasks that are amenable to rote memory (e.g., sight word recognition and written spelling). In contrast, Anthony struggled significantly on math tasks such that his overall math development was well below average and more in line with his visual-spatial and nonverbal information processing weaknesses. A prominent weakness emerged when the added dimension of speed of processing intersected with academic skill performance. Congruent with his slow cognitive efficiency, which was documented in testing and informally observed during the evaluation, Anthony struggled with academic testing that required quick performance. This was in keeping with both parent and teacher reports that Anthony takes a very long time to complete classroom and homework academic activities.
Formulation and Recommendations Anthony’s test result pattern illustrated a number of issues relatively common in children treated for medulloblastoma with radiation and chemotherapy, and his treatment before 6 years of age raised his risk for neuropsychological deficits. Functional deficits commonly seen following radiation and chemotherapy treatment for medulloblastoma include difficulties with nonverbal, visual-spatial
64 Table 7.3 Other cognitive functions Tests California Verbal Learning Test List A total trials 1–5 List A trial 1 free recall List A trial 5 free recall List B free recall List A short-delay free recall List A short-delay cued recall List A long-delay free recall List A long-delay cued recall Recognition Children’s Memory Scale Dot locations Learning Total score Long delay Stories Immediate Delayed Delayed recognition Faces Immediate Delayed Wisconsin Card Sorting Test Total errors Perseverative responses Perseverative errors Nonperseverative errors Grooved Pegboard Test Preferred hand (right) Nonpreferred hand Beery-Buktenica Developmental Test of Visual-Motor Integration Benton Judgment of Line Orientation Test
P.L. Stavinoha
Score [39] {1.0} {1.0} {2.0} {1.0} {0.5} {1.0} {0.5} {0.0}
(6) (6) (5) (8) (7) (8) (5) (6) 71 79 78 73 {2.88} {3.29} 72 {2.84}
information processing, motor dexterity, executive functions, and speed/efficiency of information processing and are presumed to be due to white matter damage. Radiation by itself, and in combination with chemotherapy, is toxic and often results in damage to the protective coating of the long nerve projections that make up the white matter. Younger children appear to be particularly susceptible to this damage and associated functional deficits. Compromise to these main communication pathways that let one brain region efficiently ‘‘talk’’ to others results in a pattern of deficits that is very consistent with Anthony’s weaknesses. Anthony’s effort and reasonably well-developed rote verbal memory ability likely propped up his academic performance over the years. This was magnified by his participation in a private school setting that did not effectively document or
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communicate his slower progress compared with peers in feedback to Anthony’s family. He typically perceived himself to perform reasonably well because he did not have a clear standard to which to compare himself. Consequently, his immersion in a regular education, public school setting resulted in a mismatch between Anthony’s previously unidentified neurocognitive deficits secondary to treatment for medulloblastoma and an increasingly demanding curriculum emphasizing abstract thinking, synthesis, and information organization. Anthony wanted to perform well but found that even his best efforts produced mediocre results. This was frustrating for Anthony as he tried to assimilate into a new school and new peer group, and he started to feel anxious about his performance to the point of being somewhat fearful of going to school. Anthony’s attempts to socialize with same-age peers were not fruitful for several reasons. He showed significant physical stigmata of early radiation treatment (e.g., hair loss, short stature) that his elementary school peers had never seen before. Anthony also had difficulty perceiving social cues and responding quickly to typical middle-school social interactions secondary to his visual-spatial information processing deficits and generally slow pace of cognitive processing. During an extensive feedback conference with Anthony’s parents as well as consultation with staff at school, a number of recommendations were made to address the prominent issues with which Anthony presented, including difficulties at school, underlying neurocognitive weaknesses, and difficulties with social interactions and emotional adjustment. Because Anthony had not already been identified as a student requiring special education services, this was the top priority. He already had begun showing anxiety about his capacity to successfully perform schoolwork and difficulty fitting in with peers. Consequently, the recommendation was made to the multidisciplinary team determining special education eligibility that it swiftly act to develop an appropriate Individualized Education Plan (IEP) and supportive educational placement for Anthony on the basis of his academic and cognitive deficits secondary to brain tumor and its treatment. Because Anthony had experienced a significant health condition that clearly resulted in significant educational need, the handicapping condition of Other Health Impairment (OHI) seemed most appropriate, because it captured the link between Anthony’s deficits and corresponding medical etiology. In particular, emphasis was placed on ensuring that Anthony received modified instruction in math given that his skill development in that core subject area lagged so significantly behind that of his same-age peers. In addition, strategies for dealing with Anthony’s slow cognitive processing speed and fatigability were provided that included reduction in the amount of homework assigned to Anthony, availability of classroom support to assist with completion of assignments during the school day, and a limit on the time that Anthony would be permitted to spend on homework each evening. A number of strategies were suggested related to deficits in visual-spatial information processing. Because Anthony exhibited a relative strength in verbal memory and verbal cognitive ability overall, rehearsing information out loud in order to better digest and remember it was recommended so as to take advantage of this strength. This oral rehearsal technique included reading or reciting information
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aloud as a primary study method for remembering information later. Subsequently, oral testing procedures could be utilized judiciously given Anthony’s handwriting deficits. Compensatory strategies for organizing and dividing visual information also were recommended. For example, Anthony could receive direct instruction in using a highlighter to divide a worksheet into smaller, more manageable parts. Using spacers or place markers could also reduce complex visual stimuli such as worksheets or pages of text to more manageable amounts. An occupational therapy evaluation was recommended because of Anthony’s difficulties with fine motor dexterity and handwriting. Difficulties with speed of information processing compound problems with handwriting, so it was recommended that Anthony have access to teacher or peer notes and that he not be required to copy large amounts of information from the board. It was stressed that Anthony likely expends a great deal of energy on tedious tasks such as copying and handwriting, and this energy could be much better spent on higher level cognitive/academic activities. The lack of understanding of the full range of implications of Anthony’s brain tumor and its treatment extended far beyond the academic domain. Anthony’s peers had little to do with him because of differences in physical appearance as well as his slower speed of processing. Although Anthony was not bullied, he certainly was not included, and this magnified any normal adjustment issues that would be associated with starting a new school. As such, two methods were advised. First, a Child Life Specialist, a professional whose role includes assisting patients and families coping with medical illness and medical procedures, was asked to speak to Anthony’s class at school in order to talk more about the difficult experiences and treatments that he had endured. Second, Anthony was selected to participate in a friendship group facilitated by the school guidance counselor focusing on social interaction and social skill development. Although Anthony’s parents had come a long way in their understanding of his deficits and the long-term implications, it was clear that they had always struggled with the potential that Anthony would have certain neurocognitive limitations, and they seemed to be facing these issues at this time only out of necessity. As an extension to the feedback conference, a series of additional consultation appointments were scheduled with the neuropsychologist to help provide guidance with IEP implementation, education regarding late effects of brain tumors and their treatment, and psychosocial support for these parents who were struggling to fully acknowledge and appreciate their child’s neurocognitive assets and weaknesses.
Update As difficult as it had been for Anthony’s parents to finally seek out neuropsychological evaluation in the first place, they seemed eager to get on the neuropsychologist’s calendar for a follow-up evaluation 18 months later. Through the process of being confronted with the late effects of Anthony’s treatment for medulloblastoma
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as he integrated into a new school setting, Anthony’s parents caught a glimpse of the numerous developmental challenges that awaited Anthony in the coming years. In the short-term, with appropriate educational placement and planning, Anthony’s anxiety about school and his classroom performance had reduced significantly. He seemed happier in his new school placement, and his participation in the social group had yielded benefits in terms of peers including him in activities more often, though he still did not have a ‘‘close’’ group of friends. Even with these positive changes, Anthony’s parents began to recognize that there would be tough days ahead as Anthony transitioned into junior high, high school, and young adulthood.
Additional Resources Key Concepts and Terms Child Life Specialist A professional typically employed in a hospital setting that works to support the psychosocial development and emotional coping of children who are ill or who are undergoing medical treatment. Medulloblastoma A type of malignant brain tumor that grows in the region of the posterior fossa. Treatment for this type of aggressive brain tumor in children typically requires surgical removal of the tumor followed by treatment with radiation and chemotherapy in order to minimize risk for regrowth of the tumor. Posterior Fossa This is a region inside the skull that is low and at the rear of the skull. This vault houses important brain regions including the cerebellum and the brain stem. Primitive Neuroectodermal Tumors Sometimes referred to as PNET, these are a group of malignant and rapidly growing brain tumors that have a high rate of recurrence. Medulloblastoma is a type of PNET. White Matter Consists of long projections from nerve cells that are covered in a protective coating called myelin, which is white in color. White matter acts as the communication pathway so that one region of the brain can communicate efficiently with another.
References Resources for Clinicians Butler, R. W., & Mulhern, R. K. (2005). Neurocognitive interventions for children and adolescents surviving cancer. Journal of Pediatric Psychology, 30(1), 65–78. Mulhern, R. K., Merchant, T. E., Gajjar, A., Reddick, W. E., & Kun, L. E. (2004). Late neurocognitive sequelae in survivors of brain tumours in childhood. The Lancet Oncology, 5(7), 399–408.
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National Cancer Institute. (2006). Late effects of treatment for childhood cancer (PDQ1). Retrieved October 16, 2006 from http://www.cancer.gov/cancertopics/pdq/treatment/lateeffects/HealthProfessional/page2. Schatz, J., Kramer, J. H., Ablin, A., & Matthay, K. K. (2000). Processing speed, working memory, and IQ: A developmental model of cognitive deficits following cranial radiation therapy. Neuropsychology, 14(2), 189–200.
Resources for Families American Brain Tumor Association. Provides funding for brain tumor research and offers educational and support services for patients and families. Address: 2720 River Road Suite 146, Des Plaines, IL 60018-4110; phone: 1-800-886-2282; Web site and e-mail: http://www.abta.org;
[email protected] American Cancer Society. Dedicated to eliminating cancer through research, education, advocacy, and service. Phone: 1-800-ACS-2345; Web site: http://www.cancer.org The Brain Tumor Society. Provides information, resources, and support for people affected by brain tumors. Address: 124 Watertown Street, Suite 3H, Watertown, MA 02472; phone: 1-800770-TBTS; Web site: http://www.tbts.org The Children’s Brain Tumor Foundation. Committed to improving treatments, quality of life, and long-term outcomes for children with brain and spinal cord tumors through research, support, education, and advocacy. Address: 274 Madison Avenue, Suite 1004, New York, NY 10016; phone: (914) 238-7658; Web-site and e-mail: http://www.cbtf.org;
[email protected] National Brain Tumor Foundation. Provides information about brain tumors and their treatment. Information is available on clinical trials and medical centers specializing in brain tumors. Address: 22 Battery Street, Suite 612, San Francisco, CA 94111-5520; phone: 1-800-934CURE; Web site and e-mail: http://www.braintumor.org;
[email protected] Chapter 8
The Mystery of the Falling Grades: Seizure Disorder Lynn Bennett Blackburn
Working with children with epilepsy is like reading a good mystery. When learning or emotional problems occur, the neuropsychologist’s job is to help identify ‘‘whodunit.’’ The list of likely suspects from the epilepsy perspective include whatever is atypical about the brain that is the basis of the seizures, seizures themselves, abnormal electrical discharges between seizures (also known as subclinical seizures), and side effects of medications used to treat the seizures. These suspects may set the stage for problems with attention, learning, memory, and emotional functioning. The reactions of family and friends to the epilepsy diagnosis and/or seizures may lead to feelings of anxiety and depression. Children with epilepsy can also develop learning or emotional problems for the same reasons that other children do, such as genetics or life experiences. John was referred for neuropsychological evaluation to sort through these suspects to find the cause of his falling grades. John was attending seventh grade at the time of referral. His parents reported that he started off well in school. He began reading prior to starting kindergarten. He was placed in a program for gifted students in first grade and continued in this program throughout elementary school. John developed complex partial seizures between fourth and fifth grade. John would become confused during seizures and was sleepy after a seizure ended. Since seizures were only occurring at home, his parents informed the school nurse, but no one else knew about his epilepsy. His antiepileptic drug (AED) supported good seizure control. During fifth grade, John seemed to work harder for his grades but continued to do well. Just before John was to start sixth grade, he began to experience frequent seizures. His parents reported that sixth grade was very hard for John because of frequent changes in medication and in dose. John was often sleepy as he adjusted to each change. When seizures occurred in school, John was teased by peers. To make matters worse, his best friend throughout elementary school moved away, leaving him with no one to come to his defense. According to his parents, other friends just drifted away, failing to include John in activities. His grades dropped to Bs and Cs. An effective AED combination was identified over the summer following sixth grade, raising his parents’ hope that seventh grade would be better. However, at the J.N. Apps et al. (eds.), Pediatric Neuropsychology Case Studies: From the Exceptional to the Commonplace. # Springer Science þ Business Media, LLC 2008
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time of referral, John was in danger of failing seventh grade, with Ds and Fs in most subjects. At the time of referral, John’s parents expressed concern about his memory. John would forget what he was told to do. Even when he would begin a task, he would get distracted and fail to complete it. He needed prompts to do things that should be habit, such as brushing his teeth or taking his medication. He had trouble finding possessions he had put away. Regarding schoolwork, John’s parents reported that he often forgot to write down assignments and to turn in homework he had completed. They tried organizing John’s binder for him, providing color-coded folders for each class. They put reminder notes on his completed work. However, even with this help, he was still inconsistent in turning in work. Late assignments were one factor in his poor grades. John’s teachers noted inconsistency in memory, stating that John would appear to understand a concept one day but not the next day. Poor performances on tests were also contributing to his poor grades. However, his parents indicated that he could remember material for tests if he studied for them. John could remember upcoming events that were important to him and past events he had enjoyed. He was doing relatively well in his Spanish class, remembering new vocabulary and grammar as it was introduced. Although his seizures were once again well controlled, John did not make new friends. His school counselor reported that he sat with other students during lunch but did not talk to them. John’s parents described his interests as more ‘‘immature’’ than peers. They also indicated that John had difficulty adjusting to change, such as the move to junior high. Although his parents provided no further examples, his difficulty adjusting to change became a very important clue in understanding John. When John arrived for testing, he was like many adolescents referred for testing. He responded to the questions with one-word answers, providing little help in understanding his perspective on his problems. He knew school was hard. He complained that teachers went too fast, making it hard to take notes or write down assignments. John spent the rest of the interview staring at his shoes and offering ‘‘I don’t know’’ or shoulder shrugs as answers to the questions, all while his parents were struggling to remain quiet, barely controlling the urge to jump in and answer for him. John’s behavior during testing provided two important clues regarding his problems. First, his affect remained flat throughout testing. He showed no reaction to praise or pride in his accomplishments when successful. He appeared to have little confidence in his skills, becoming easily overwhelmed. He gave up on some tasks (saying ‘‘I don’t know’’) before he had even heard the complete question or direction. However, with encouragement to listen again and then take a guess, he often did know the correct answer. He became visibly anxious with the introduction of each new task. Second, he seemed to have problems with expressive language. He knew lots of facts but had difficulty organizing them to effectively answer test questions. He could engage in conversation if asked a series of yes/no questions but could not elaborate on answers in a way that continued the conversation. He also had trouble communicating in writing. Generation of written sentences
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was painstakingly slow. While his parents and teachers were focused on his memory, his behavior during testing suggested that emotional factors and language skills were also likely suspects in his falling grades.
Test Results Although John might have been expected to struggle with tasks involving understanding and remembering language, his achievement on these tasks fell in the average to high average range (Table 8.1). John retold stories in a somewhat disorganized fashion but captured the main theme and most details. He used effective strategies to organize his recall of items from a shopping list. Rote learning, that is, learning facts through repetition, emerged as a relative strength for John. This strength may have supported his rapid learning of early academic information. John had trouble remembering some types of visual Table 8.1 Other cognitive functions Tests Scorea Clinical Evaluation of Language Fundamentals – Fourth Edition Understanding paragraphs (9)
37th
California Verbal Learning Test – Children’s Version Total learning Long-delay free recall Recognition
115 115 108
84th 84th 70th
(13) (12) (12)
84th 74th 74th
(2) (7)
