Spasticity
Spasticity
Diagnosis and Management
Allison Brashear, MD
Professor and Chair Department of Neurology Wake Forest University School of Medicine Wake Forest Baptist Medical Center Winston-Salem, North Carolina
Elie Elovic, MD
Professor and Chief Physical Medicine and Rehabilitation University of Utah School of Medicine Salt Lake City, Utah
New York
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Library of Congress Cataloging-in-Publication Data Spasticity : diagnosis and management / [edited by] Allison Brashear, Elie Elovic. â•…â•… p. ; cm. ╇ Includes bibliographical references and index. ╇ ISBN╇ 978-1-933864-51-8 ╇ 1. Spasticity. I. Brashear, Allison. II. Elovic, Elie. ╇ [DNLM: 1. Muscle Spasticity—diagnosis. 2. Muscle Spasticity—therapy. 3. Extremities—physiopathology. 4. Motor Neuron Disease. ╇ 5. Muscle Spasticity—etiology. WE 550 S7368 2011] ╇ RC935.S64S627 2011 ╇ 616.8’56—dc22 2010024464
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We dedicate this book to our families for their unconditional support, and to our professors, colleagues, students and patients who continue to humble us with their strength and challenge us to improve the care of those with spasticity.
Contents
Preface Contributors
I
xi xiii
General Overview
1. Why Is Spasticity Important?
3
Allison Brashear and Elie Elovic
2. Epidemiology of Spasticity in the Adult and Child
5
John R. McGuire
3. Spasticity and Other Signs of the Upper Motor Neuron Syndrome
17
Nathaniel H. Mayer
4. Ancillary Findings Associated With Spasticity
33
Cindy B. Ivanhoe
II Assessment Tools 5. Measurement Tools and Treatment Outcomes in Patients With Spasticity
51
Omar Gomez-Medina and Elie Elovic
6. Assessment of Spasticity in the Upper Extremity
71
Thomas Watanabe
7. Assessment of Spasticity and Other Consequences of the Upper Motor Neuron Syndrome Affecting the Lower Limb
81
Alberto Esquenazi
vii
viii
Contents
8. Setting Realistic and Meaningful Goals for Treatment
91
Elie Elovic
III Treatment of Spasticity 9. Chemoneurolysis With Phenol and Alcohol: A “Dying Art” that Merits Revival
101
Lawrence J. Horn, Gurtej Singh, and Edward R. Dabrowski
10. Botulinum Toxin in the Treatment of Lower Limb Spasticity
119
Alberto Esquenazi
11. Botulinum Toxin in the Treatment of Upper Limb Spasticity
131
Allison Brashear
12. Anatomical Correlation of Common Patterns of Spasticity
141
Mayank Pathak and Daniel Truong
13. The Role of Physical and Occupational Therapy in the Evaluation and Management of Spasticity
155
Robert Shingleton, Jonathan H. Kinzinger, and Elie Elovic
14. Emerging Technologies in the Management of Upper Motor Neuron Syndromes
183
Ira Rashbaum and Steven R. Flanagan
15. Pharmacologic Management of Spasticity: Oral Medications
199
Jay M. Meythaler and Scott Kowalski
16. Intrathecal Baclofen for Spasticity
229
Michael Saulino, Stuart A. Yablon, Elizabeth Moberg-Wolff, John W. Chow, and Dobrivoje S. Stokic
17. Surgery in the Management of Spasticity
243
David A. Fuller
18. Diagnostic Evaluation of Adult Patients With Spasticity
271
Geoffrey Sheean
IV Evaluation and Management of Diseases Involving Spasticity 19. Overview of Genetic Causes of Spasticity in Adults and Children
281
Donald McCorquordale, III and Stephan Züchner
20. Spasticity Affecting Those With Neuromuscular Diseases: Pathology, Epidemiology, and Treatment
297
Rachel M. Dolhun and Peter D. Donofrio
21. Spasticity Due to Disease of the Spinal Cord: Pathophysiology, Epidemiology, and Treatment Heather W. Walker and Steven Kirshblum
313
Contents
22. Spasticity Due to Multiple Sclerosis: Epidemiology, Pathophysiology and Treatment
ix
341
Anjali Shah and Ian Maitin
23. Spasticity Due to Stroke Pathophysiology
357
Anthony B. Ward and Surendra Bandi
24. Spasticity in Traumatic Brain Injury
371
Ross Zafonte, Stephanie Co, and Anath Srikrishnan
25. Evaluation, Treatment Planning, and Nonsurgical Treatment of Cerebral Palsy
387
Ann Tilton
26. Surgical Management of Spasticity in the Child With Cerebral Palsy
397
Kat Kolaski, John Frino, and L. A. Koman
V Basic Science of Spasticity 27. Animal Models of Spasticity
419
Patrick Harvey Kitzman Index
439
Preface Spasticity: Diagnosis and Management is the first
book solely dedicated to the diagnosis and treatment of spasticity. Arguably, this text could have been titled Upper Motor Neuron Syndrome, as spasticity is just one of many components of the constellation of conditions that make up the upper motor neuron syndrome (UMNS). Other authors use the phrase “muscle overactivity” to describe these conditions, but since the term most commonly used by physicians, therapists, patients, and caregivers is spasticity, we chose this as our title while the actual focus of the book is about the broader context of UMNS and how it leads to disability. Spasticity is only part of the UMNS as is so eloquently described by Dr Mayer in Chapter 3. UMNS occurs when damage to the brain or spinal cord results in a constellation of signs and symptoms that may include spasticity or increase in tone, rigidity, and flexor spasms superimposed on any combination of weakness, paralysis, and/or decreased dexterity. The varied clinical characteristics of UMNS must be taken into account when developing a treatment plan. In Chapter 8, Dr Elovic discusses the importance of evaluating the patient, their support systems, and the clinical manifestation of the UMNS so that clinicians can set realistic and obtainable treatment goals. The editors’ objective in the development of this book were to clearly define the process for the diagnosis of spasticity, the basic science behind its pathophysiology, the measurement tools used for evaluation, and the available treatment options.
This text is designed to bring the reader up-todate on the demographics of disorders of tone, with a detailed discussion on epidemiology by Dr McGuire in Chapter 2 and an eloquent description of pathphysiology of the UMNS by Dr Mayer in Chapter 3. In Chapter 4, Dr Ivanhoe and colleagues review concerns of the patient with spasticity that are often overlooked, including bowel and bladder function. In Chapter 5, Dr Elovic discusses the assessment tools for upper and lower extremity spasticity and Drs Watanabe and Esquenazi review the assessment of upper and lower extremity spasticity in Chapters 6 and 7. Part III of the text focuses on treatment and outlines the many tools available to physicians such as oral medication, intrathecal baclofen, and chemodenervation in Chapters 9 through 11. Chapters 10 and 11 are specifically dedicated to the use of botulinum toxin in adults, with in-depth coverage of chemodenervation treatment protocols for upper and lower extremity spasticity. Drs Truong and Pathak provide a detailed artistic rendering in Chapter 12 of the important muscles and landmarks in spasticity management. Chapter 13 focuses on nonmedical therapeutic modalities and highlights the role of therapists as an important part of the management team. Dr Flanagan’s discussion of the role of the emerging technologies in Chapter 14 highlights the continued evaluation of the treatment of spasticity for patients. In Chapters 15 and 16, Drs Meythaler and Saulino, discuss the role of oral medication and more invasive treatments such as intrathecal baclofen. In Chapter 17, Dr Fuller xi
xii
Preface
discusses the important and often overlooked option of neuro-orthopedic intervention in the management of spasticity. Part IV of the book is devoted to the evaluation and management of individual diseases involving spasticity, which reinforces the importance of disease diagnosis and management in the patient with spasticity and reemphasizes the need to establish an accurate diagnosis before embarking on treatment. Amyotrophic lateral sclerosis, multiple sclerosis, poststroke spasticity, traumatic brain injury, and spinocerebellar ataxia (discussed in Chapters 20–24) are very different conditions, even though each may present with spasticity. Accurate diagnosis is paramount in designing the appropriate treatment plan for these distinct diseases. Chapters 25 and 26 are devoted to the unique challenges involved in diagnosing and managing spasticity in children with detailed discussions of chemodenervation and surgery. Finally, Chapter 27 reviews the
basic science and the role of animal models in better understanding the underlying mechanisms of spasticity in patients with spinal cord injury. Until the advent of oral medications, intrathecal baclofen, and botulinum toxin, the treatment of disorders of tone as part of the UMNS was often frustrating for the patient, caregiver, and physician. With the development of effective therapies and a team approach to management of these disorders, we have sought to address the diagnosis and treatment of spasticity in an integrated, clinically useful text. Our charge was to provide a one-stop resource for physicians, therapists, and other healthcare providers who serve the complex needs of these patients with the goal of offering the highest level of care and improving outcomes. Allison Brashear, MD Elie Elovic, MD
Contributors
Allison Brashear, MD Professor and Chair Department of Neurology Wake Forest University School of Medicine Wake Forest Baptist Medical Center Winston-Salem, North Carolina Surendra Bandi, MS (Orthopaedics), MRCS Specialist Registrar, Rehabilitation Medicine North Staffordshire Rehabilitation Centre University Hospital of North Staffordshire Stoke-On-Trent, United Kingdom John W. Chow, PhD Methodist Rehabilitation Center Jackson, Mississippi Stephanie Co Spaulding Rehabilitation Hospital Harvard Medical School Boston, Massachusetts Edward R. Dabrowski, MD Division Chief of Physical Medicine and Rehabilitation Department of Pediatrics Medical Director of Rehabilitation Services Childrens Hospital of Michigan Assistant Professor of Pediatrics Department of Pediatrics Assistant Professor Physical Medicine and Rehabilitation Wayne State University Detroit, Michigan
Rachel M. Dolhun, MD Instructor Department of Neurology Vanderbilt University Medical Center Nashville, Tennessee Peter D. Donofrio, MD Department of Neurology Vanderbilt University Medical Center Nashville, Tennessee Elie Elovic, MD Professor and Chief Physical Medicine and Rehabilitation University of Utah School of Medicine Salt Lake City, Utah Alberto Esquenazi, MD Chairman Department of Physical Medicine and Rehabilitation Director Gait and Motion Analysis Laboratory MossRehab Elkins Park, Pennsylvania Steven R. Flanagan, MD Professor and Chairman of Rehabilitation Medicine New York University School of Medicine New York, New York
xiii
xiv
Contributors
John Frino, MD Assistant Professor Wake Forest University School of Medicine Winston-Salem, North Carolina
Kat Kolaski, MD Assistant Professor Wake Forest University School of Medicine Winston-Salem, North Carolina
David A. Fuller, MD Assistant Professor of Orthopaedic Surgery University of Medicine and Dentistry of New Jersey Camden, New Jersey
L. A. Koman, MD Professor Department of Orthopaedic Surgery Wake Forest University School of Medicine Winston-Salem, North Carolina
Omar Gomez-Medina, MD Faculty Member Department of Physical Medicine and Rehabilitation San Pablo Hospital Bayamón, Puerto Rico Lawrence J. Horn, MD, MRM Professor Department of Physical Medicine and Rehabilitation Wayne State University School of Medicine Medical Director Neuroscience Rehabilitation Institute of Michigan Detroit, Michigan Cindy B. Ivanhoe, MD Neurorehabilitation Specialists Houston, Texas Jonathan H. Kinzinger, DPT, NCS Rehabilitation Services University of Utah Health Care Salt Lake City, Utah
Scott Kowalski, DO Department of Physical Medicine and Rehabilitation Wayne State University School of Medicine Rehabilitation Institute of Michigan Detroit, Michigan Ian Maitin, MD, MBA Associate Professor and Chair Department of Physical Medicine and Rehabilitation Temple University School of Medicine Philadelphia, Pennsylvania Nathaniel H. Mayer, MD Professor Emeritus Physical Medicine and Rehabilitation Temple University School of Medicine Philadelphia, Pennsylvania Director Motor Control Analysis Laboratory MossRehab Elkins Park, Pennsylvania
Steven Kirshblum, MD Medical Director Director of Spinal Cord Injury Services Professor Kessler Institute for Rehabilitation Department of Physical Medicine and Rehabilitation New Jersey Medical School University of Medicine and Dentistry of New Jersey West Orange, New Jersey
Donald McCorquordale, III, BSc John T. Hussman Institute for Human Genomics University of Miami Miller School of Medicine Miami, Florida
Patrick Harvey Kitzman, PhD, MSPT Department of Rehabilitation Sciences University of Kentucky Lexington, Kentucky
Jay M. Meythaler, MD, JD Professor-Chair Department of Physical Medicine and Rehabilitation Wayne State University School of Medicine Detroit, Michigan
John R. McGuire, MD Associate Professor Department of Physical Medicine and Rehabilitation Medical College of Wisconsin Milwaukee, Wisconsin
contributors
Elizabeth Moberg-Wolff, M.D. Children’s Hospital of Wisconsin Medical College of Wisconsin Milwaukee, Wisconsin
Dobrivoje S. Stokic, MD, DSc Center for Neuroscience and Neurological Recovery Methodist Rehabilitation Center Jackson, Mississippi
Mayank Pathak, MD The Parkinson’s and Movement Disorder Institute Fountain Valley, California
Ann Tilton, MD Professor of Neurology and Pediatrics Louisiana State University Health and Sciences Center New Orleans, Louisianna
Ira Rashbaum, MD Clinical Professor of Rehabilitation Medicine New York University School of Medicine New York, New York Michael Saulino, MD, PhD Assistant Professor Department of Physical Medicine and Rehabilitation MossRehab Elkins Park, Pennsylvania Anjali Shah, MD Assistant Professor Department of Physical Medicine and Rehabilitation University of Texas Southwestern Medical Center Dallas, Texas Robert Shingleton, PT, DPT, MBA Physical Therapist and Spasticity Management Program Coordinator Therapy Services, Physical Medicine and Rehabilitation University Health Care Salt Lake City, Utah Geoffrey Sheean, MD Professor of Neurosciences Director of Neuromuscular Program University of California, San Diego San Diego, California Gurtej Singh, MD Physical Medicine and Rehabilitation Wayne State University School of Medicine Rehabilitation Institute of Michigan Detroit, Michigan Anath Srikrishnan Spaulding Rehabilitation Hospital Harvard Medical School Boston, Massachusetts
Daniel Truong, MD The Parkinson’s and Movement Disorder Institute Fountain Valley, California Heather W. Walker, MD Assistant Professor Department of Physical Medicine and Rehabilitation University of North Carolina at Chapel Hill Chapel Hill, North Carolina Anthony B. Ward, BSc, MD, FRCPEd, FRCP, SFEBPRM Professor of Rehabilitation Medicine North Staffordshire Rehabilitation Centre University Hospital of North Staffordshire Stoke on Tent, United Kingdom Thomas Watanabe, MD Clinical Director Drucker Brain Injury Center MossRehab at Elkins Park Elkins Park, Pennsylvania Stuart A. Yablon, MD Director of Rehabilitation Research Baylor Institute for Rehabilitation Dallas, Texas Ross Zafonte Spaulding Rehabilitation Hospital Harvard Medical School Boston, Massachusetts Stephan Züchner, MD Associate Professor for Human Genetics and Neurology University of Miami Miller School of Medicine€ Miami Institute for Human Genomics Miami, Florida
xv
I
GENERAL OVERVIEW
1
Why Is Spasticity Important?
Allison Brashear Elie Elovic
Increased tone or spasticity is the tightness that patients and/or caregivers report with passive movement of the limb. In more scientific language, spasticity is a motor disorder characterized by a velocity-dependent increase in the tonic stretch reflex. A clinical finding on the neurologic examination, spasticity together with increased tone, brisk reflexes with incoordination, and weakness represents the upper motor neuron syndrome. So why is spasticity important and why does it merit a textbook? This textbook is dedicated to the diagnosis and management of these conditions. In an age where imaging is often used more than the physical examination, diagnosis and treatment of spasticity rely solely on the physician’s examination of the patient. When many question the enduring role of history and physical examination, spasticity and the resulting impact on the patient and caregiver can only be assessed thru this means. The assessment of spasticity requires that a physician perform a neurologic assessment of the patient, including the tone, reflexes, strength, and coordination. Clinical skills are at the heart of the profession of medicine, and the diagnosis and treatment of spasticity reinforce the importance of these skills. Regardless of its cause, spasticity causes significant disability. An estimated 4 million individuals are stroke survivors in the United States, and as many as one third may have spasticity with sufficient disability to require treatment. According to the Centers
for Disease Control, 1.4 million people in the United States sustain a traumatic brain injury each year, and additional patients develop spasticity after spinal cord injury. The result of any brain or spinal cord injury is a variable pattern of increased tone with weakness and discoordination that leads to significant disability in many patients. The treatment of spasticity relies on the physician’s assessment of the individual treatment together with conversations with the caregiver. Patients’ inability to perform simple activities of daily living for themselves and the adverse effects on the caregiver drives physicians to find ways to decrease tone, build strength, and improve coordination. The team approach is a cornerstone of a successful treatment, and interaction of the patient, the caregiver, the therapist, and the physicians works best to provide a care plan that addresses functional impairment and plots a course to treat the problems. Spasticity is a clinically relevant medical problem when it interferes with function or care of the patients. The evolution of the upper motor neuron syndrome may take days to months after a central nervous system injury. Moreover, the presentation in one patient may differ from another despite them having similar central nervous system lesions. The lesion alone does not predict the amount or impact of the spasticity. Other factors such as medications, stress, medical illness, timing of therapy, and so on impact the clinical
˘
˘
Iâ•…general overview
presentation. As a result, each patient must be assessed individually with his or her caregiver, noting the concerns that impair the performance of activities of daily living or other deficits. No matter how much we learn about stroke, traumatic brain injury, multiple sclerosis, and spinal cord injury, the assessment of spasticity and the effect of tone on function will remain unique to each individual patient’s circumstance. Although neurologic examination is essential for the diagnosis of spasticity, the management of spasticity has many paths for treatment depending on the disability and goals of the patient and caregiver. One patient may benefit from a combination of tools for spasticity, including interventions such as botulinum toxin injections and intrathecal baclofen, whereas others may require a more conservative route such as splinting or oral medications. The informed physician should know how to assess the amount of spasticity,
determine the functional limitations it creates, and then be able to develop a management plan for that individual patient. How to assess the complicated picture of spasticity and when to intervene is the focus of this text. Our co-authors define for you why spasticity is important and detail the diagnosis and management options, but the goal is to provide the reader with the best options for the physician’s individual patient. As editors, we aim to explore the diagnosis and management of the many different types of patients with spasticity and to open the door to the different treatment paradigms for patients with spasticity. So why is spasticity important? The answer is because it often causes disability and impairs function in our patients. The goal of this book is to provide the foundation for excellent care of our patients facing these disabilities.
2
Epidemiology of Spasticity in the Adult and Child
John R. McGuire
Despite the extensive work done to develop improved treatments for muscle overactivity in patients with upper motor neuron (UMN) lesions, there are only a limited number of studies on the incidence and prevalence of spasticity. Most likely, this is due to the lack of consistent definitions and reliable measures of spastic hypertonia. Compounding the difficulties in the literature is that the few prevalence studies that have been performed rely primarily on patient surveys or clinical measures of spasticity, which lack sensitivity for quantifying abnormal muscle activation (1). More importantly, there are fewer studies on the prevalence of problematic or significant spasticity. A final issue is that different authors use dissimilar definitions for the condition. Some of the descriptions have included spasticity that requires medication or physiotherapy (2–4), causes pain, (5) interferes with activities of daily living (ADL) (6–10), or has an Ashworth score of 2 or higher or 3 (11, 12). The actual incidence of spasticity depends on the cause of the UMN lesion. After damage to central motor pathways above T12, there is initial paralysis followed by adaptive changes in the brain and spinal cord that develop over time, which result in a complex set of motor behaviors (13–17). Paresis, soft tissue contracture, and muscle overactivity are the 3 major mechanisms of motor impairment (18). Although spasticity is often used as an umbrella term, it is just one component of the muscle overactivity that
conÂ�tributes to the upper motor neuron syndrome (UMNS) (16, 19, 20). Reliable assessments are complicated by the fact that spasticity can vary throughout the day, change with different positions, and increase with any noxious stimulus, such as pressure sores, urinary tract infection, deep venous thrombosis, ingrown toenails, joint pain, or constipation (15, 16, 21). In a large population-based study initiated by the Christopher & Dana Reeve Foundation Paralysis Resource Center (PRC) and researchers at the University of New Mexico’s Center for Development and Disability that was performed from 2006 to 2008, more than 33,000 households across the country were surveyed for any disability (22). From this review, they estimated that nearly 1 in 50 people or approximately 6 million people in the United States are living with paralysis. The leading etiologies for this condition were stroke, spinal cord injury (SCI), multiple sclerosis (MS), and cerebral palsy (CP) (Table 2.1) (22). It should be noted that the prevalence of paralysis noted in people with SCI and MS from this survey is significantly higher than those found previously. Additional important questions that need to be answered are what are the conditions that cause problematic spasticity and what is the number of people who require treatment? Data collected from the adult spasticity management clinic at the Medical College of Wisconsin (MCW) during a 6-month period in 2008 may give some answers to these questions. The number
Iâ•…general overview
Table 2.1
Prevalence of Paralysis in the United States (n = 5,596,000) n
%
1,608,000 1,275,000 939,000 412,000 272,000 242,000 212,000 636,000
29 23 17 7 5 4 4 11
Cause Stroke SCI MS CP Postpolio syndrome TBI Neurofibromatosis Other
Paralysis Resource Center, Christopher & Dana Reeve Foundation, 2009 (22).
and diagnosis of the patients treated with intrathecal baclofen (ITB) or botulinum neurotoxin (BoNT) is shown in Table 2.2. Spinal cord injury, CP, and MS were the most common diagnoses treated with ITB, whereas stroke, CP, and SCI were the most common conditions treated with BoNT. This suggests that patients with these conditions may have the highest prevÂ� alence of problematic spasticity. When discussing the conditions in children, data from the work of Hutchison et al. (23) may shed some light. The most common causes of spasticity in 341 children seen at some of the clinics at the Royal Children’s Hospital in Melbourne, Australia, in 1998 were CP (79%), traumatic brain injury (TBI; 6%), spina bifida (5%), and SCI (2%).
Four studies evaluated the prevalence of spasticity after a stroke and are summarized in Table 2.3. They are all from Europe, with the prevalence of spasticity ranging from 17% to 38%. Each identified the arm and leg spasticity using the Modified Ashworth Scale (MAS) score (29) and used the Barthel Index (BI) (30, 31) as functional measure. In a crosssectional survey 1 year poststroke, Lundstrom et al. (2) identified 140 people with their first event from a national stroke registry. Arm and leg spasticity were measured using the MAS, and disability was measured with the modified Rankin Scale (32) and the BI. Disabling spasticity (DS) was defined as spasticity in need of an intervention, for example, intensive physiotherapy, orthosis, or pharmacologic treatment. The observed prevalence of any spasticity was 17% and of DS 4%. Patients with DS scored significantly worse on the modified Rankin Scale and the BI than those with no DS. Disabling spasticity was more frequent in the upper extremity and correlated positively with other indices of motor impairment and inversely with age. Although the prevalence of DS after a first-ever stroke from this study was low, in the context of the large number of stroke survivors, the number became more significant. In a Swedish cohort study, Sommerfeld et al. (33) evaluated 95 patients with a first-ever stroke within 1 week of their stroke (mean, 5.4 days) and 3 months after their event. The authors measured spasticity by obtaining the MAS for the arm and leg, as well as self-reported muscle stiffness, tendon reflexes, several motor impairment measures, and the BI as a disability measure. Of the 95 patients studied, 64 were hemiparetic, 18 were spastic, 6 reported muscle stiffness, and 18 had increased tendon reflexes 3 months after
STROKE Each year, approximately equal to 795,000 people in the United States experience a new (about 610,000) or recurrent stroke (185,000) (24). The prevalence of stroke in the United States is 2.6 million for men and 3.9 million for women (24), with an annual incidence rate of 183 per 100,000 (25). There are significant differences in the prevalence of stroke by race/ethnicity, education level, and state/area of residence (26). Blacks have a higher incidence of stroke than whites, especially among the young, and the rate increases with age regardless of race (24). In Europe, the annual standardized incidence for stroke is 113 per 100,000 per year (27). In Sweden, with a population of 9 million, about 30,000 patients are hospitalized every year because of stroke, of which 20,000 are a first-ever event (28).
Table 2.2
Patients Treated for Spasticity at the Medical College of Wisconsin With ITB or BoNT From January 2008 to July 2008 Diagnosis
ITB Patients
BoNT Patients
SCI CP MS TBI Stroke Anoxic encephalopathy Other
43 (30%) 34 (24%) 30 (21%) 17 (12%) 6 (4%) 3 (2%) 11 (7%)
50 (14%) 74 (20%) 23 (6%) 37 (10%) 133 (37%) 43 (12%) 3 (1%)
Total
144
363
2â•… Epidemiology of spasticity in the adå°“ult and child
Table 2.3
Prevalence of Spasticity After First Stroke Study Lundstrom et al. â•… 2008 (2) Welmer et al. 2006 â•… (34) Sommerfield et al. â•… 2004 (33) Watkins et al. â•… 2002 (10)
No. of Patients 140
Time Poststroke
Spasticity Diagnosis
Location
Prevalence Spasticity
Problematic Spasticity
of
1 year
MAS
Sweden
17%
4%a
66
18 months
MAS
Sweden
20%
NR
95
15 â•… years 1 years 3 years 5 years 12 months 12 months NR
Maynard et al. 1990 (3) Study 1 Maynard et al. 1990 (3) Study 2 Anson et al. 1996 (46)
191
Johnson et al. 1998 (44)
853
Sköld et al. 1999 (5) Noreau et al. 2000 (47) Walter et al. 2002 (45)
354 482 99
466
Spasticity Diagnosis
Location, Duration
Prevalence Spasticity
Problematic Spasticity
CS
MI, 1985–1988 USA
67% 78% NR
NR
Atlanta
62%
37%a 49% 26% 46% 12%b
CS
PSR
Colorado
NR
MAS PSRe PSRf
Sweden, 1997 Quebec Chicago Hines â•… VA
65% 40% 53%
of
35%c 32% 28% 30%d NR 40%a
CS, Clinical Scale (spasticity present if deep tendon reflexes increased, increased muscle tone during passive movements, or involuntary muscle spasms); DC, discharge from hospital; NR, not reported; PSR, patient self-report. a Spasticity that required medication. b Spasticity that interfered with ADLs. c Problematic spasticity. d Spasticity that was painful, restricting ADLs, or both. e PSR: “Over the past 12 months have you developed or suffered from spasticity?” f PSR: “Are you having a problem with spasticity?”
upper thoracic patients with incomplete injuries. The percentage of patients requiring spasticity treatment with Frankel grades B (sensory incomplete, motor complete) and C (motor incomplete, nonfunctional) was 50% and 52% (42), respectively, whereas the percentage of patients requiring spasticity treatment with Frankel grades A (sensory and motor complete) and D (motor incomplete, functional) was 27% and 29%, respectively. Little et al. (43) reported similar findings in 26 patients with SCI, where the patients with Frankel grade C had greater flexor withdrawal responses and extensor spasms, more pain, and interference with sleep than those with Frankel grades A and D. These findings suggest that increased time after injury and motor incompleteness of SCI may contribute to the increased severity of spasticity. Johnson et al. (44) investigated the frequency of both medical and nonmedical complications reported to the Colorado Spinal Cord Injury Early Notification System for patients with SCI. They interviewed each patient by telephone at 1, 3, and 5 years after injury. They noted a decrease in the prevalence of spasticity from year 1 (35%) to year 5 (28%), which may have
been due to reduced sample size at year 5 (50% of year 1). They also noted that spasticity had a variable impact on quality of life and productivity measures. They recommended that follow-up needs to be longer than 5 years (decades rather than years) to gauge the full impact of each SCI complication. Using both physical examination and patient self-report, Sköld et al. (5) found abnormal MAS in only 60% of the patients reporting significant spasticity, whereas 97% of patients with abnormal MAS report spasticity. This study underscores the importance of using both clinical measures and patient self-report when evaluating problematic spasticity. The other studies using patient self-report, which are summarized in Table 2.4, support the need for patient questionnaires to ask sufficient questions to determine the full impact of spasticity and the other components of UMNS on the patient’s daily activities (45–47). Traumatic Brain Injury In 2003, an estimated 1,565,000 people in the United States experienced TBI, a rate of 538.2 per 100,000
10
Iâ•…general overview
Table 2.5
Prevalence of Spasticity After TBI No. of Patients
Study Wedekind and â•… Lippert-Gruner â•… 2005 (52)
32
Time Post Injury
Spasticity Diagnosis
Country
PE
Germany
1 year
Prevalence Spasticity
of
34%
Problematic Spasticity NR
PE, physical examination (measure not reported); NR, not reported.
(52) investigated the 1-year outcome of 32 survivors with severe TBI. They divided the patients into 2 groups: those with brainstem injury (midbrain and pons, n = 15) and those without brainstem lesions (n = 17). At 1 year, 8 of the 15 (53%) in the brainstem injury group and 3 of the 17 (18%) in the nonbrainstem group had spasticity. The authors failed to mention how they assessed spasticity. However, patients with a brainstem lesion had lower Functional Independence Measure scores and lower disability rating scale (53) and were unable to return to work even with support. It is difficult to determine from this small study what impact, if any, spasticity had on the negative long-term outcome. Elovic and Zafonte (54) reported that 25% of the patients admitted to the Traumatic Brain Injury Model Systems had evidence of increased tone while undergoing inpatient rehabilitation. Unfortunately, the nature of the system data did not allow an identification if the spasticity was problematic. More recently, consecutive admissions with the diagnosis of TBI to the Kessler Institute for Rehabilitation were assessed for the presence of spas-
population with 230,000 hospitalizations as a result (48). Although almost 90% of all TBI are mild and cause no lasting impairment, TBI of any severity has the potential to cause significant long-term disability (49, 50). An estimated 5.3 million people are living in the United States with disability related to TBI (51). Based on these estimates, one might expect that the number of TBI patients with paralysis (242,000) reported from the PRC may be an underestimate (Table 2.5). Using the prevalence data from the PRC and based on the prevalence of problematic spasticity of 30% to 50% from other conditions (Table 2.6), there could be more than 100,000 TBI patients with problematic spasticity. This number is consistent with the number of patients treated in the spasticity clinic at MCW, where TBI is the fourth most common diagnosis treated with ITB or BoNT (Table 2.2). However, there is no study that demonstrates this number. Despite the number of patients with TBI, there has been very little work that has attempted to quantify the problem of spasticity in people with this condition. In a retrospective study, Wedekind and Lippert-Gruner
Table 2.6
Estimated Prevalence of Spasticity and Problematic Spasticity in the United States Condition Stroke TBI CP MS SCI
Estimate US Patients 6,500,000 5,300,000 764,000 400,000 259,000
NA, insufficient data from published reports. a Average percent from referenced population-based studies.
Prevalence Spasticityâ•›a
of
1,495,000 (23%) NA 649,400 (85%) 268,000 (67%) 172,040 (68%)
Prevalence of Problematic Spasticityâ•›a 448,500 (30%) NA 382,000 (50%) 152,000 (38%) 83,490 (33%)
2â•… Epidemiology of spasticity in the adå°“ult and child
ticity. In total, 161 were evaluated and 45 (27.9%) of them were noted to have increased tone on evaluation. Clearly, more research is needed on the impact and prevalence of spasticity in the TBI population. In addition, better outcome measures are needed for the assessment of spasticity in patients with TBI (55). MULTIPLE SCLEROSIS Hirtz et al. (25) estimate that in the general population the 1-year prevalence for MS is 0.9 per 1000. In the United States, there are approximately 400,000 people with MS, and 200 more people are diagnosed every week (56). This number is nearly two and a half times less than the estimated number of patients with MS with paralysis from the PRC (22). Worldwide, MS is thought to affect more than 2.5 million people. It occurs with greater frequency above the 40° latitude, is more common among whites, and is 2 to 3 times more common in women than in men (56). Although most people are diagnosed between the ages of 20 and 50, MS is also diagnosed in children and adolescents. Estimates suggest that 8000 to 10,000 children (