Spasticity Diagnosis and Management

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

Acquisitions Editor: Beth Barry Cover Design: Joe Tenerelli Compositor: The Manila Typesetting Company Printer: Bang Printing Visit our website at www.demosmedpub.com © 2011 Demos Medical Publishing, LLC. All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher. Medicine is an ever-changing science. Research and clinical experience are continually expanding our knowledge, in particular our understand- ing of proper treatment and drug therapy. The authors, editors, and publisher have made every effort to ensure that all information in this book is in accordance with the state of knowledge at the time of production of the book. Nevertheless, the authors, editors, and pubÂ

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 xi Contributors xiii I General Overview 3 5 1. Why Is Spasticity Important? 17 33 Allison Brashear and Elie Elovic 51 2. Epidemiology of Spasticity in the Adult and Child 71 John R. McGuire 81 3. Spasticity and Other Signs of the Upper Motor Neuron Syndrome vii Nathaniel H. Mayer 4. Ancillary Findings Associated With Spasticity Cindy B. Ivanhoe II Assessment Tools 5. Measurement Tools and Treatment Outcomes in Patients With Spasticity Omar Gomez-Medina and Elie Elovic 6. Assessment of Spasticity in the Upper Extremity Thomas Watanabe 7. Assessment of Spasticity and Other Consequences of the Upper Motor Neuron Syndrome Affecting the Lower Limb Alberto Esquenazi

viii Contents 8. Setting Realistic and Meaningful Goals for Treatment 91 Elie Elovic III Treatment of Spasticity 101 119 9. Chemoneurolysis With Phenol and Alcohol: A “Dying Art” that Merits Revival 131 141 Lawrence J. Horn, Gurtej Singh, and Edward R. Dabrowski 155 10. Botulinum Toxin in the Treatment of Lower Limb Spasticity 183 199 Alberto Esquenazi 229 11. Botulinum Toxin in the Treatment of Upper Limb Spasticity 243 271 Allison Brashear 12. Anatomical Correlation of Common Patterns of Spasticity Mayank Pathak and Daniel Truong 13. The Role of Physical and Occupational Therapy in the Evaluation and Management of Spasticity Robert Shingleton, Jonathan H. Kinzinger, and Elie Elovic 14. Emerging Technologies in the Management of Upper Motor Neuron Syndromes Ira Rashbaum and Steven R. Flanagan 15. Pharmacologic Management of Spasticity: Oral Medications Jay M. Meythaler and Scott Kowalski 16. Intrathecal Baclofen for Spasticity Michael Saulino, Stuart A. Yablon, Elizabeth Moberg-Wolff, John W. Chow,  and Dobrivoje S. Stokic 17. Surgery in the Management of Spasticity David A. Fuller 18. Diagnostic Evaluation of Adult Patients With Spasticity Geoffrey Sheean IV Evaluation and Management of Diseases Involving Spasticity 281 297 19. Overview of Genetic Causes of Spasticity in Adults and Children 313 Donald McCorquordale, III and Stephan Züchner 20. Spasticity Affecting Those With Neuromuscular Diseases: Pathology, Epidemiology, and Treatment 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

Contents ix 22. Spasticity Due to Multiple Sclerosis: Epidemiology, Pathophysiology 341 and Treatment 357 371 Anjali Shah and Ian Maitin 387 397 23. Spasticity Due to Stroke Pathophysiology 419 Anthony B. Ward and Surendra Bandi 439 24. Spasticity in Traumatic Brain Injury Ross Zafonte, Stephanie Co, and Anath Srikrishnan 25. Evaluation, Treatment Planning, and Nonsurgical Treatment of Cerebral Palsy Ann Tilton 26. Surgical Management of Spasticity in the Child With Cerebral Palsy Kat Kolaski, John Frino, and L. A. Koman V Basic Science of Spasticity 27. Animal Models of Spasticity Patrick Harvey Kitzman Index



Preface Spasticity: Diagnosis and Management is the first This text is designed to bring the reader up-to- date on the demographics of disorders of tone, with a book solely dedicated to the diagnosis and treatment detailed discussion on epidemiology by Dr McGuire in of spasticity. Arguably, this text could have been titled Chapter 2 and an eloquent description of pathphysiol- Upper Motor Neuron Syndrome, as spasticity is just ogy of the UMNS by Dr Mayer in Chapter 3. In Chap- one of many components of the constellation of condi- ter 4, Dr Ivanhoe and colleagues review concerns of tions that make up the upper motor neuron syndrome the patient with spasticity that are often overlooked, (UMNS). Other authors use the phrase “muscle over- including bowel and bladder function. In Chapter 5, activity” to describe these conditions, but since the Dr Elovic discusses the assessment tools for upper and term most commonly used by physicians, therapists, lower extremity spasticity and Drs Watanabe and Es- patients, and caregivers is spasticity, we chose this as quenazi review the assessment of upper and lower ex- our title while the actual focus of the book is about tremity spasticity in Chapters 6 and 7. the broader context of UMNS and how it leads to dis- ability. Part III of the text focuses on treatment and out- lines the many tools available to physicians such as Spasticity is only part of the UMNS as is so elo- oral medication, intrathecal baclofen, and chemode- quently described by Dr Mayer in Chapter 3. UMNS nervation in Chapters 9 through 11. Chapters 10 and occurs when damage to the brain or spinal cord re- 11 are specifically dedicated to the use of botulinum sults in a constellation of signs and symptoms that toxin in adults, with in-depth coverage of chemode- may include spasticity or increase in tone, rigidity, nervation treatment protocols for upper and lower and flexor spasms superimposed on any combination extremity spasticity. Drs Truong and Pathak provide of weakness, paralysis, and/or decreased dexterity. a detailed artistic rendering in Chapter 12 of the im- The varied clinical characteristics of UMNS must be portant muscles and landmarks in spasticity manage- taken into account when developing a treatment plan. ment. Chapter 13 focuses on nonmedical therapeutic In Chapter 8, Dr Elovic discusses the importance of modalities and highlights the role of therapists as an evaluating the patient, their support systems, and the important part of the management team. Dr Flana- clinical manifestation of the UMNS so that clinicians gan’s discussion of the role of the emerging technolo- can set realistic and obtainable treatment goals. The gies in Chapter 14 highlights the continued evaluation editors’ objective in the development of this book of the treatment of spasticity for patients. In Chapters were to clearly define the process for the diagnosis of 15 and 16, Drs Meythaler and Saulino, discuss the spasticity, the basic science behind its pathophysiol- role of oral medication and more invasive treatments ogy, the measurement tools used for evaluation, and such as intrathecal baclofen. In Chapter 17, Dr Fuller the available treatment options. xi

xii Preface discusses the important and often overlooked option basic science and the role of animal models in better of neuro-orthopedic intervention in the management understanding the underlying mechanisms of spastic- of spasticity. ity in patients with spinal cord injury. Part IV of the book is devoted to the evaluation Until the advent of oral medications, intrathe- and management of individual diseases involving spas- cal baclofen, and botulinum toxin, the treatment of ticity, which reinforces the importance of disease diag- disorders of tone as part of the UMNS was often nosis and management in the patient with spasticity frustrating for the patient, caregiver, and physician. and reemphasizes the need to establish an accurate di- With the development of effective therapies and a agnosis before embarking on treatment. Amyotrophic team approach to management of these disorders, we lateral sclerosis, multiple sclerosis, poststroke spastic- have sought to address the diagnosis and treatment of ity, traumatic brain injury, and spinocerebellar ataxia spasticity in an integrated, clinically useful text. Our (discussed in Chapters 20–24) are very different con- charge was to provide a one-stop resource for physi- ditions, even though each may present with spastic- cians, therapists, and other healthcare providers who ity. Accurate diagnosis is paramount in designing the serve the complex needs of these patients with the goal appropriate treatment plan for these distinct diseases. of offering the highest level of care and improving Chapters 25 and 26 are devoted to the unique chal- outcomes. lenges involved in diagnosing and managing spasticity in children with detailed discussions of chemodener- Allison Brashear, MD vation and surgery. Finally, Chapter 27 reviews the Elie Elovic, MD

Contributors Allison Brashear, MD Rachel M. Dolhun, MD Professor and Chair Instructor Department of Neurology Department of Neurology Wake Forest University School of Medicine Vanderbilt University Medical Center Wake Forest Baptist Medical Center Nashville, Tennessee Winston-Salem, North Carolina Peter D. Donofrio, MD Surendra Bandi, MS (Orthopaedics), MRCS Department of Neurology Specialist Registrar, Rehabilitation Medicine Vanderbilt University Medical Center North Staffordshire Rehabilitation Centre Nashville, Tennessee University Hospital of North Staffordshire Stoke-On-Trent, United Kingdom Elie Elovic, MD Professor and Chief John W. Chow, PhD Physical Medicine and Rehabilitation Methodist Rehabilitation Center University of Utah School of Medicine Jackson, Mississippi Salt Lake City, Utah Stephanie Co Alberto Esquenazi, MD Spaulding Rehabilitation Hospital Chairman Harvard Medical School Department of Physical Medicine and Boston, Massachusetts Rehabilitation Edward R. Dabrowski, MD Director Division Chief of Physical Medicine and Gait and Motion Analysis Laboratory MossRehab Rehabilitation Elkins Park, Pennsylvania Department of Pediatrics Medical Director of Rehabilitation Services Steven R. Flanagan, MD Childrens Hospital of Michigan Professor and Chairman of Rehabilitation Medicine Assistant Professor of Pediatrics New York University School of Medicine Department of Pediatrics New York, New York Assistant Professor Physical Medicine and xiii Rehabilitation Wayne State University Detroit, Michigan

xiv Contributors John Frino, MD Kat Kolaski, MD Assistant Professor Assistant Professor Wake Forest University School of Medicine Wake Forest University School of Medicine Winston-Salem, North Carolina Winston-Salem, North Carolina David A. Fuller, MD L. A. Koman, MD Assistant Professor of Orthopaedic Surgery Professor University of Medicine and Dentistry of New Jersey Department of Orthopaedic Surgery Camden, New Jersey Wake Forest University School of Medicine Winston-Salem, North Carolina Omar Gomez-Medina, MD Faculty Member Scott Kowalski, DO Department of Physical Medicine and Department of Physical Medicine and Rehabilitation Rehabilitation San Pablo Hospital Wayne State University School of Medicine Bayamón, Puerto Rico Rehabilitation Institute of Michigan Detroit, Michigan Lawrence J. Horn, MD, MRM Professor Ian Maitin, MD, MBA Department of Physical Medicine and Associate Professor and Chair Department of Physical Medicine and Rehabilitation Wayne State University School of Medicine Rehabilitation Medical Director Neuroscience Temple University School of Medicine Rehabilitation Institute of Michigan Philadelphia, Pennsylvania Detroit, Michigan Nathaniel H. Mayer, MD Cindy B. Ivanhoe, MD Professor Emeritus Neurorehabilitation Specialists Physical Medicine and Rehabilitation Houston, Texas Temple University School of Medicine Philadelphia, Pennsylvania Jonathan H. Kinzinger, DPT, NCS Director Rehabilitation Services Motor Control Analysis Laboratory University of Utah Health Care MossRehab Salt Lake City, Utah Elkins Park, Pennsylvania Steven Kirshblum, MD Donald McCorquordale, III, BSc Medical Director John T. Hussman Institute for Human Genomics Director of Spinal Cord Injury Services University of Miami Miller School of Medicine Professor Miami, Florida Kessler Institute for Rehabilitation Department of Physical Medicine and John R. McGuire, MD Associate Professor Rehabilitation Department of Physical Medicine and New Jersey Medical School University of Medicine and Dentistry of Rehabilitation Medical College of Wisconsin New Jersey Milwaukee, Wisconsin West Orange, New Jersey Jay M. Meythaler, MD, JD Patrick Harvey Kitzman, PhD, MSPT Professor-Chair Department of Rehabilitation Sciences Department of Physical Medicine and University of Kentucky Lexington, Kentucky Rehabilitation Wayne State University School of Medicine Detroit, Michigan

contributors xv Elizabeth Moberg-Wolff, M.D. Dobrivoje S. Stokic, MD, DSc Children’s Hospital of Wisconsin Center for Neuroscience and Neurological Recovery Medical College of Wisconsin Methodist Rehabilitation Center Milwaukee, Wisconsin Jackson, Mississippi Mayank Pathak, MD Ann Tilton, MD The Parkinson’s and Movement Disorder Institute Professor of Neurology and Pediatrics Fountain Valley, California Louisiana State University Health and Sciences Ira Rashbaum, MD Center Clinical Professor of Rehabilitation Medicine New Orleans, Louisianna New York University School of Medicine New York, New York Daniel Truong, MD The Parkinson’s and Movement Disorder Institute Michael Saulino, MD, PhD Fountain Valley, California Assistant Professor Department of Physical Medicine and Heather W. Walker, MD Assistant Professor Rehabilitation Department of Physical Medicine and MossRehab Elkins Park, Pennsylvania Rehabilitation University of North Carolina at Chapel Hill Anjali Shah, MD Chapel Hill, North Carolina Assistant Professor Department of Physical Medicine and Anthony B. Ward, BSc, MD, FRCPEd, FRCP, SFEBPRM Rehabilitation University of Texas Southwestern Medical Center Professor of Rehabilitation Medicine Dallas, Texas North Staffordshire Rehabilitation Centre University Hospital of North Staffordshire Robert Shingleton, PT, DPT, MBA Stoke on Tent, United Kingdom Physical Therapist and Spasticity Management Thomas Watanabe, MD Program Coordinator Clinical Director Therapy Services, Physical Medicine and Drucker Brain Injury Center MossRehab at Elkins Park Rehabilitation Elkins Park, Pennsylvania University Health Care Salt Lake City, Utah Stuart A. Yablon, MD Director of Rehabilitation Research Geoffrey Sheean, MD Baylor Institute for Rehabilitation Professor of Neurosciences Dallas, Texas Director of Neuromuscular Program University of California, San Diego Ross Zafonte San Diego, California Spaulding Rehabilitation Hospital Harvard Medical School Gurtej Singh, MD Boston, Massachusetts Physical Medicine and Rehabilitation Wayne State University School of Medicine Stephan Züchner, MD Rehabilitation Institute of Michigan Associate Professor for Human Genetics and Detroit, Michigan Neurology Anath Srikrishnan University of Miami Miller School of Medicine€ Spaulding Rehabilitation Hospital Miami Institute for Human Genomics Harvard Medical School Miami, Florida Boston, Massachusetts



I GENERAL OVERVIEW



Why Is Spasticity 1 Important? Allison Brashear Elie Elovic Increased tone or spasticity is the tightness that pa- for Disease Control, 1.4 million people in the United tients and/or caregivers report with passive movement States sustain a traumatic brain injury each year, and of the limb. In more scientific language, spasticity is a additional patients develop spasticity after spinal cord motor disorder characterized by a velocity-dependent injury. The result of any brain or spinal cord injury increase in the tonic stretch reflex. A clinical find- is a variable pattern of increased tone with weakness ing on the neurologic examination, spasticity together and discoordination that leads to significant disability with increased tone, brisk reflexes with incoordination, in many patients. and weakness represents the upper motor neuron syn- drome. So why is spasticity important and why does it The treatment of spasticity relies on the physi- merit a textbook? cian’s assessment of the individual treatment together with conversations with the caregiver. Patients’ inabil- This textbook is dedicated to the diagnosis and ity to perform simple activities of daily living for them- management of these conditions. In an age where im- selves and the adverse effects on the caregiver drives aging is often used more than the physical examina- physicians to find ways to decrease tone, build strength, tion, diagnosis and treatment of spasticity rely solely and improve coordination. The team approach is a cor- on the physician’s examination of the patient. When nerstone of a successful treatment, and interaction of many question the enduring role of history and physi- the patient, the caregiver, the therapist, and the physi- cal examination, spasticity and the resulting impact cians works best to provide a care plan that addresses on the patient and caregiver can only be assessed thru functional impairment and plots a course to treat the this means. The assessment of spasticity requires that problems. a physician perform a neurologic assessment of the patient, including the tone, reflexes, strength, and co- Spasticity is a clinically relevant medical problem ordination. Clinical skills are at the heart of the pro- when it interferes with function or care of the patients. fession of medicine, and the diagnosis and treatment The evolution of the upper motor neuron syndrome of spasticity reinforce the importance of these skills. may take days to months after a central nervous sys- tem injury. Moreover, the presentation in one patient Regardless of its cause, spasticity causes signifi- may differ from another despite them having similar cant disability. An estimated 4 million individuals are central nervous system lesions. The lesion alone does stroke survivors in the United States, and as many not predict the amount or impact of the spasticity. as one third may have spasticity with sufficient dis- Other factors such as medications, stress, medical ill- ability to require treatment. According to the Centers ness, timing of therapy, and so on impact the clinical ˘

˘ Iâ•…general overview presentation. As a result, each patient must be assessed determine the functional limitations it creates, and individually with his or her caregiver, noting the con- then be able to develop a management plan for that cerns that impair the performance of activities of daily individual patient. living or other deficits. No matter how much we learn about stroke, traumatic brain injury, multiple sclero- How to assess the complicated picture of spastic- sis, and spinal cord injury, the assessment of spasticity ity and when to intervene is the focus of this text. Our and the effect of tone on function will remain unique co-authors define for you why spasticity is important to each individual patient’s circumstance. and detail the diagnosis and management options, but the goal is to provide the reader with the best options Although neurologic examination is essential for for the physician’s individual patient. As editors, we the diagnosis of spasticity, the management of spastic- aim to explore the diagnosis and management of the ity has many paths for treatment depending on the many different types of patients with spasticity and to disability and goals of the patient and caregiver. One open the door to the different treatment paradigms patient may benefit from a combination of tools for for patients with spasticity. spasticity, including interventions such as botulinum toxin injections and intrathecal baclofen, whereas So why is spasticity important? The answer is be- others may require a more conservative route such as cause it often causes disability and impairs function in splinting or oral medications. The informed physician our patients. The goal of this book is to provide the should know how to assess the amount of spasticity, foundation for excellent care of our patients facing these disabilities.

Epidemiology of Spasticity in the 2 Adult and Child John R. McGuire Despite the extensive work done to develop improved conÂ

Iâ•…general overview Table 2.1 Four studies evaluated the prevalence of spas- Prevalence of Paralysis in the United States ticity after a stroke and are summarized in Table 2.3. They are all from Europe, with the prevalence (n = 5,596,000) of spasticity ranging from 17% to 38%. Each iden- tified the arm and leg spasticity using the Modified Cause n% Ashworth Scale (MAS) score (29) and used the Barthel Index (BI) (30, 31) as functional measure. In a cross- Stroke 1,608,000 29 sectional survey 1 year poststroke, Lundstrom et al. SCI 1,275,000 23 (2) identified 140 people with their first event from a MS 17 national stroke registry. Arm and leg spasticity were CP 939,000 measured using the MAS, and disability was mea- Postpolio syndrome 412,000 7 sured with the modified Rankin Scale (32) and the BI. TBI 272,000 5 Disabling spasticity (DS) was defined as spasticity in Neurofibromatosis 242,000 4 need of an intervention, for example, intensive phys- Other 212,000 4 iotherapy, orthosis, or pharmacologic treatment. The 636,000 11 observed prevalence of any spasticity was 17% and of DS 4%. Patients with DS scored significantly worse Paralysis Resource Center, Christopher & Dana Reeve on the modified Rankin Scale and the BI than those Foundation, 2009 (22). with no DS. Disabling spasticity was more frequent in the upper extremity and correlated positively with and diagnosis of the patients treated with intrathecal other indices of motor impairment and inversely with baclofen (ITB) or botulinum neurotoxin (BoNT) is age. Although the prevalence of DS after a first-ever shown in Table 2.2. Spinal cord injury, CP, and MS stroke from this study was low, in the context of the were the most common diagnoses treated with ITB, large number of stroke survivors, the number became whereas stroke, CP, and SCI were the most common more significant. conditions treated with BoNT. This suggests that pa- tients with these conditions may have the highest prevÂ

2â•… Epidemiology of spasticity in the adå°“ult and child  Table 2.3 Prevalence of Spasticity After First Stroke Study No. of Time Spasticity Location Prevalence Problematic Patients Poststroke Diagnosis of Spasticity Spasticity Lundstrom et al. 140 1 year MAS Sweden 17% 4%a â•… 2008 (2) 20% NR Welmer et al. 2006 66 18 months MAS Sweden 21% NR 19% â•… (34) 27% 67%b 36% Sommerfield et al. 95 <1 week MAS Sweden 38% â•… 2004 (33) 3 months Watkins et al. 106 12 months MAS-elbow UK â•… 2002 (10) TAS Combined NR, not reported; Combined, MAS and TAS. aSpasticity that requires an intervention, for example, physiotherapy, orthosis, pharmacologic. bPatients with arm and leg spasticity (67%) had 50% lower Barthel score than patients with no spasticity. stroke. Nonspastic patients (77) had statistically sig- on functional improvement in patients who have had nificantly better motor and activity scores than spas- stroke in the long term. tic patients (18). However, the correlations between muscle tone and disability scores were low, and severe Watkins et al. (10) evaluated 106 consecutive disabilities were seen in almost the same number of community-dwelling stroke survivors in Liverpool, nonspastic and spastic patients. They concluded that UK, who were 12 months poststroke. They measured severe disabilities were seen in almost the same num- spasticity at the elbow using the MAS and at several ber of nonspastic and spastic patients and suggested joints and in the arms and legs using the Tone As- that the importance of spasticity may be overstated. sessment Scale (TAS) (36); they also assessed disabil- There were several limitations to this study including ity using the BI. The prevalence of spasticity in their the small number of participants and the investiga- study depended on the metric used. Using the MAS, tors’ reliance on the use of the MAS as the only means 29 (27%) of the 106 patients had spasticity, whereas of identifying if a person has spasticity. As a result, 38 (36%) were identified as spastic using the TAS. they may have missed patients with spasticity or other Forty (38%) was spastic when including those who components of the UMN syndrome (10). In addition, were identified as having tone by either metric. Those the sample of patients only had a limited amount with spasticity had significantly lower BI scores at of motor deficits because 67% were hemiparetic at 12 months, whereas those with arm and leg involve- 3 months. Of this group, 28% had spasticity (33). In ment had a BI 50% of those without spasticity. an 18-month follow-up study with the same cohort of patients, Welmer et al. (34) evaluated the frequency Of the 4 studies that addressed the prevalence of spasticity and its association with functioning and of spasticity in stroke survivors, 3 suggest that it is health-related quality of life (HRQL) (35). Of the associated with greater motor impairments and has 66 patients studied, 38 were hemiparetic; of these, a negative impact on functional capabilities. The low 13 displayed spasticity, 12 had increased tendon re- prevalence of spasticity in these reports is most likely flexes, and 7 reported muscle stiffness 18 months due to the lack of sensitivity of the measures used to after stroke. Although there was a weak correlation be- assess it and the mild motor impairments of the sam- tween spasticity and HRQL, the hemiparetic patients ples studied. The study of more involved patients can without spasticity had significantly better BI function- be undertaken by looking at prevalence of spasticity ing scores and significantly better HRQL health scales from an inpatient rehabilitation unit. Francisco (4) than patients with spasticity. This follow-up study performed this type of study when he presented a ret- suggests that spasticity may have a negative impact rospective review of 204 stroke admissions to a free- standing rehabilitation hospital in 2002. The mean duration of stroke to admission was 5.76 days (range,

Iâ•…general overview 1.2–48 months), and 78% of the patients had hemor- persons (40). The PRC reports a much higher estimate rhagic strokes. Seventy percent had spastic hypertonia of SCI prevalence of approximately 1,275,000 people (MAS ≥1), and 50% had clinically significant spasticity in the United States, with the most common cause of that required treatment. The larger prevalence of SCI being motor vehicle accidents followed by falls problematic spasticity in this group supports the no- and acts of violence (22). Sports-related spinal cord tion that more severe spasticity is associated with injuries occur more commonly in children and teenag- greater impairments, as many of the patients included ers, whereas work-related injuries are more common in this investigation also had severe motor, language, in adults. Most people with SCI are in their teens or and cognitive impairments. twenties, and 78% are male (41). The male prepon- derance of SCI decreases after age 65, at which point, Two studies used electrophysiologic measures to the most common mechanism of SCI is falls. More evaluate the prevalence of spasticity after a stroke. than half of all SCI occur at the cervical level, almost O’Dwyer et al. (37) evaluated 24 hemiparetic stroke a third in the thoracic level, and the remainder in the patients 1 to 13 months (mean, 5.3 months) after their lumbar area (41). event for upper limb spasticity and contracture. The motor impairment was graded mild to severe based Table 2.4 summarizes the studies that assessed on item 6 of the Motor Assessment Scale (38). They the prevalence of spasticity in patients with SCI. Of studied stretch-induced electromyographic activity of the 7 studies reviewed, 3 of the studies used clinical as- the biceps muscle at different velocities of stretch and sessments to identify patients with spasticity, whereas found tonic stretch reflexes in 5 patients (21%). Of 3 used patient questionnaires. The prevalence of spas- the 24 patients, 13 had a flexion contracture from 2° ticity ranged from 40% to 78% (average, 68%), with to 22°, suggesting that contracture may be more im- the higher prevalence noted in the studies that used a portant than spasticity in this population. Although clinical scale. The prevalence of problematic spasticity this study had similar prevalence data to the studies was addressed in 5 of the studies. The criteria used in Table 2.3, there were a limited number of patients to define it was if the patient required medication for in this study and they only tested 1 muscle for spas- treatment and if their spasticity interfered with ADL, ticity. In a larger study, Malhotra et al. (1) evaluated was painful, or both. Using these measures, the preva- wrist spasticity in 100 patients 1 to 6 weeks (mean, lence of problematic spasticity ranged from 12% to 3 weeks) after their first stroke with severe weakness 49%, with an average of 33%. (scored 0 in the grasp section of the Action Research Arm Test) (39). Spasticity was evaluated using the In the first of 2 epidemiologic studies, Maynard MAS and biomechanical and neurophysiological mea- et al. (3) evaluated the occurrence of spasticity and its sures. The MAS was abnormal in 44 patients, and 87 severity in 96 patients at one SCI center. Spasticity was patients had abnormal involuntary muscle activation considered present if the patient had increased deep ten- usinganovelportabledevicewithanelectrogoniometer, don reflexes, muscle tone during passive movements, force transducer, and surface bipolar electromyo- or involuntary muscle spasms. Severity of spastic- graphic electrodes. This suggests that neurophysi- ity was determined if they were taking antispasticity ological measures for spasticity are more sensitive medication and if they had satisfactory treatment. than clinical ones and that assessing prevalence with Treatment was indicated if the spasticity was interfer- clinical metrics may result in an underestimate. Ad- ing with ADL and sleep or caused pain that prevented ditional studies with more objective measures of or interfered with activities. By this definition, 67% spasticity are needed to more accurately determine of the patients had spasticity at the time of their dis- the prevalence of spasticity in patients who have had charge (average, 118 days) and 37% were taking anti- stroke. spasticity medication. The incidence of spasticity was higher among groups with cervical and upper thoracic SPINAL CORD INJURY levels of injury compared with groups with other lev- els of injury. At their 1-year follow-up, the percent of The estimated annual incidence of SCI in the United patients with spasticity increased to 78% and 49% States, not including those who die at the scene of the of them required medication. The second part of the accident, is approximately 40 cases per million or ap- study analyzed the presence of spasticity severe enough proximately 12,000 new cases each year (40). The to require treatment in 466 subjects with SCI from 13 estimated prevalence of SCI in the United States for different SCI centers. From this patient population, 2008 was approximately 259,000 persons, with stud- 26% of the patients received antispasticity treatment ies reporting within a range of 229,000 to 306,000 at the time of discharge (average, 105 days), and the percentage increased to 46% at their 1-year follow-up. Spasticity treatment was more common in cervical and

2â•… Epidemiology of spasticity in the adå°“ult and child ˘ Table 2.4 Prevalence of Spasticity After SCI Study No. of Time Post Spasticity Location, Prevalence Problematic Patients Injury Diagnosis Duration of Spasticity Spasticity Maynard et al. 1990 (3) 96 DC, CS MI, 1985–1988 67% 37%a 78% 49% Study 1 1 year USA NR 26% 46% Maynard et al. 1990 (3) 466 DC, CS Atlanta 62% 12%b Study 2 1 year Colorado NR 35%c 32% Anson et al. 1996 (46) 191 1 to >15 NR Sweden, 1997 65% 28% Quebec 40% 30%d â•… years Chicago Hines 53% NR â•… VA 40%a Johnson et al. 1998 (44) 853 1 years PSR 3 years 5 years Sköld et al. 1999 (5) 354 12 months MAS Noreau et al. 2000 (47) 482 12 months PSRe Walter et al. 2002 (45) 99 NR PSRf 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. aSpasticity that required medication. bSpasticity that interfered with ADLs. cProblematic spasticity. dSpasticity that was painful, restricting ADLs, or both. ePSR: “Over the past 12 months have you developed or suffered from spasticity?” fPSR: “Are you having a problem with spasticity?” upper thoracic patients with incomplete injuries. The been due to reduced sample size at year 5 (50% of percentage of patients requiring spasticity treatment year 1). They also noted that spasticity had a vari- with Frankel grades B (sensory incomplete, motor able impact on quality of life and productivity mea- complete) and C (motor incomplete, nonfunctional) sures. They recommended that follow-up needs to was 50% and 52% (42), respectively, whereas the per- be longer than 5 years (decades rather than years) to centage of patients requiring spasticity treatment with gauge the full impact of each SCI complication. Us- Frankel grades A (sensory and motor complete) and ing both physical examination and patient self-report, D (motor incomplete, functional) was 27% and 29%, Sköld et al. (5) found abnormal MAS in only 60% of respectively. Little et al. (43) reported similar findings the patients reporting significant spasticity, whereas in 26 patients with SCI, where the patients with Fran- 97% of patients with abnormal MAS report spastic- kel grade C had greater flexor withdrawal responses ity. This study underscores the importance of using and extensor spasms, more pain, and interference both clinical measures and patient self-report when with sleep than those with Frankel grades A and D. evaluating problematic spasticity. The other studies These findings suggest that increased time after injury using patient self-report, which are summarized in and motor incompleteness of SCI may contribute to Table 2.4, support the need for patient questionnaires the increased severity of spasticity. to ask sufficient questions to determine the full impact of spasticity and the other components of UMNS on Johnson et al. (44) investigated the frequency of the patient’s daily activities (45–47). both medical and nonmedical complications reported to the Colorado Spinal Cord Injury Early Notification Traumatic Brain Injury System for patients with SCI. They interviewed each patient by telephone at 1, 3, and 5 years after injury. In 2003, an estimated 1,565,000 people in the United They noted a decrease in the prevalence of spasticity States experienced TBI, a rate of 538.2 per 100,000 from year 1 (35%) to year 5 (28%), which may have

10 Iâ•…general overview Table 2.5 Prevalence of Spasticity After TBI Study No. of Time Post Spasticity Country Prevalence Problematic Patients Injury Diagnosis of Spasticity Spasticity Wedekind and 32 1 year PE Germany 34% NR â•… Lippert-Gruner â•… 2005 (52) PE, physical examination (measure not reported); NR, not reported. population with 230,000 hospitalizations as a result (52) investigated the 1-year outcome of 32 survi- (48). Although almost 90% of all TBI are mild and vors with severe TBI. They divided the patients into cause no lasting impairment, TBI of any severity has 2 groups: those with brainstem injury (midbrain and the potential to cause significant long-term disability pons, n = 15) and those without brainstem lesions (49, 50). An estimated 5.3 million people are living in (n = 17). At 1 year, 8 of the 15 (53%) in the brain- the United States with disability related to TBI (51). stem injury group and 3 of the 17 (18%) in the non- Based on these estimates, one might expect that the brainstem group had spasticity. The authors failed to number of TBI patients with paralysis (242,000) re- mention how they assessed spasticity. However, pa- ported from the PRC may be an underestimate (Table tients with a brainstem lesion had lower Functional 2.5). Using the prevalence data from the PRC and Independence Measure scores and lower disability based on the prevalence of problematic spasticity of rating scale (53) and were unable to return to work 30% to 50% from other conditions (Table 2.6), there even with support. It is difficult to determine from could be more than 100,000 TBI patients with prob- this small study what impact, if any, spasticity had on lematic spasticity. This number is consistent with the the negative long-term outcome. Elovic and Zafonte number of patients treated in the spasticity clinic at (54) reported that 25% of the patients admitted to the MCW, where TBI is the fourth most common diagno- Traumatic Brain Injury Model Systems had evidence sis treated with ITB or BoNT (Table 2.2). However, of increased tone while undergoing inpatient rehabili- there is no study that demonstrates this number. tation. Unfortunately, the nature of the system data did not allow an identification if the spasticity was Despite the number of patients with TBI, there problematic. More recently, consecutive admissions has been very little work that has attempted to quantify with the diagnosis of TBI to the Kessler Institute for the problem of spasticity in people with this condition. Rehabilitation were assessed for the presence of spas- In a retrospective study, Wedekind and Lippert-Gruner Table 2.6 Estimated Prevalence of Spasticity and Problematic Spasticity in the United States Condition Estimate Prevalence Prevalence of Problematic US Patients of Spasticityâ•a› Spasticityâ•a› Stroke 6,500,000 1,495,000 (23%) 448,500 (30%) TBI 5,300,000 NA NA CP MS 764,000 649,400 (85%) 382,000 (50%) SCI 400,000 268,000 (67%) 152,000 (38%) 259,000 172,040 (68%) 83,490 (33%) NA, insufficient data from published reports. aAverage percent from referenced population-based studies.

2â•… Epidemiology of spasticity in the adå°“ult and child 11 ticity. In total, 161 were evaluated and 45 (27.9%) relied primarily on patient self-report for determining of them were noted to have increased tone on evalu- severity of spasticity, and one study used the MAS. ation. Clearly, more research is needed on the impact Two studies (6, 12) suggest that MS-related spastic- and prevalence of spasticity in the TBI population. In ity was not adequately treated. Barnes et al. (12) con- addition, better outcome measures are needed for the ducted a random sample of 100 people with MS (from assessment of spasticity in patients with TBI (55). a total of 260 patients) in the city of Newcastle upon Tyne in the north of England. From a total of 68 pa- MULTIPLE SCLEROSIS tients who participated in the study, 45 (67%) were women with a mean age of 49 years (range, 28–73). Hirtz et al. (25) estimate that in the general popula- The mean time from diagnosis was 10.2 years (range, tion the 1-year prevalence for MS is 0.9 per 1000. In 0–48 years). Spasticity for each limb was assessed us- the United States, there are approximately 400,000 ing the MAS, and the worst joint score was recorded. people with MS, and 200 more people are diagnosed Ninety-seven percent of the patients had detectable every week (56). This number is nearly two and a half leg spasticity (MAS ≥1), and 50% had arm spastic- times less than the estimated number of patients with ity. The 32 (47%) patients with significant spasticity MS with paralysis from the PRC (22). Worldwide, MS (MAS ≥2) had more severe disability as measured by is thought to affect more than 2.5 million people. It the Kurtzke Functional Systems Scale (57), the New- occurs with greater frequency above the 40° latitude, castle Independence Assessment Form (58), and the is more common among whites, and is 2 to 3 times motor subscale of the Functional Independence Mea- more common in women than in men (56). Although sure (59), suggesting that spasticity is one factor that most people are diagnosed between the ages of 20 and may play an important role in the overall disability in 50, MS is also diagnosed in children and adolescents. MS and effective intervention may reduce disability. Estimates suggest that 8000 to 10,000 children (<18 Unfortunately, 50% of the patients from this study years old) in the United States have MS, and another needed oral medications, which were either not pre- 10,000 to 15,000 have experienced at least one symp- scribed or were dosed suboptimally. tom suggestive of MS (56). In a survey of 493 patients with MS from the Table 2.7 summarizes the 3 studies on the preva- Northern California Chapter of the National Multiple lence of spasticity in patients with MS. These reports Sclerosis Society, 168 patients (34%) returned com- pleted questionnaires (6). Fifty-eight percent rated Table 2.7 Prevalence of Spasticity in Patients With MS Study No. of Duration of Spasticity Country Prevalence/Severity of Patients Survey Diagnosis Spasticity, No. (%) Goodin, 1999 (6) 168 1997 PSRa USA None: 50 (30) Mild: 64 (38) Barnes et al. 2003 68 1998–1999 MAS UK Moderate/severe: 54 (32) (12) 20,380 1996–2003 PSRc USA MAS <2: 36 (53) Rizzo et al. 2004 (7) MAS ≥2: 32 (47)b None: 3196 (16) Minimal/mild: 10,248 (50) Moderate: 3494 (17) Severe/total: 3440 (17) MAS, Modified Ashworth Scale; PSR, patient self-report. aPSR: none, mild, moderate, or severe spasticity and/or spasms for each arm and leg. bFifty percent needed spasticity medication. cPSR: 0 = normal (no symptoms of spasticity), 1= minimal (some problems with spasticity, but does not interfere with activities), 2 = mild (spasticity occasionally forces me to change some of my activities, for example, once a week or less), 3 = moderate (spasticity frequently affects some of my activities, for example, several times a week), 4 = severe (every day, spasticity problems force me to modify my daily activities), 5 = total (every day, spasticity problems prevent me from doing many of my daily activities).

12 Iâ•…general overview themselves as partially or totally disabled, and 65% to alter some of their daily activities. Patients with felt that their disability was in part or in whole due more severe spasticity were more likely to be older, to fatigue. Seventy percent of the patients had mild, male, disabled, or unemployed; had a longer dura- moderate, or severe spasticity. Spasticity was rated for tion of disease; and had more relapses and worsening each arm and leg as none, mild, moderate, or severe MS symptoms in the months before the survey. Only spasticity and/or spasms (60). Unfortunately, the sur- 1.1% of the respondents had the ITB pump, and 78% vey did not correlate spasticity with their measures of of the patients with severe/total spasticity were using disability. Only 67% of the patients with moderate or any medication for it. This suggests that poor toler- severe spasticity and 44% of those with mild spastic- ance or undertreatment may be a problem. A weak- ity had received any treatment. The obvious limita- ness of this study was the use of a subjective patient tions of this study are the subjective metrics used to self-report measure of spasticity, which may have re- assess spasticity and the limited (34%) response rate sulted in an overestimation of the prevalence of spas- that suggests that this may not have had a representa- ticity (5). Nevertheless, the patient self-report may be tive sample of the population sampled. a more accurate reflection of problematic spasticity for the patient. In a much larger cross-sectional study from the Patient Registry of the North American Research CEREBRAL PALSY Committee on Multiple Sclerosis, Rizzo et al. (7) published the most extensive review of the prevalence Cerebral palsy is the most common cause of motor and severity of spasticity in patients with MS. More disability in childhood (61–64). It is an umbrella term than 20,000 patients were enrolled for the study be- covering a group of nonprogressive but often chang- tween 1996 and 2003. In the survey, spasticity was ing motor impairment syndromes secondary to lesions described for the patients as “unusual tightening of or anomalies of the brain, arising at any time during muscles that feels like leg stiffness, jumping of legs, brain development (61). Population-based studies have a repetitive bouncing of the foot, muscle cramping reported the prevalence of CP to range from 1.5 to in legs or arms, legs going out tight and straight or 3.0 cases per 1000 children (25, 62–64). The number drawing up.” A 0-to-5 (0 = normal, 5 = total) spas- of people with CP in the United States is estimated at ticity scale that reflected severity and frequency of 764,000 (64). The estimated number of CP patients spasticity on the patients’ daily activities was used. with paralysis is more than 400,000 according to Eighty-four percent of the respondents had some the PRC (Table 2.1). It is the second most common degree of spasticity, of which 63% had problem- atic spasticity that at least occasionally forced them Table 2.8 Prevalence of Spasticity in Children With CP Study No. of CP Age (years) Diagnosis Country Prevalence of Disabling Spasticity Spasticity Yeargin-Alisop 416 8 CR USA, 2002 All: 80.6% NR â•… et al., 2008 127 6–19 â•… (66) Di: 93 (22%) Wichers et al., Tri: 5 (1%) â•… 2005 (8) Tet: 104 (25%) Hem: 94 (22%) PE Netherlands, All: 93.7% â•… 1977–1988 Di: 30 (25%) 40%a Tri: 12 (9%) 58% Tet: 29 (24%) 93% Hem: 48 (38%) 8% CR, Clinician review and International Classification of Disease codes; NR, not reported; PE, physical examination; Di, spastic diplegia; Tri, spastic triplegia; Tet, spastic tetraplegia (quadriplegia); Hem, spastic hemiplegia. aPercentage of severe motor disability: not able to walk independently by the age of 5.

2â•… Epidemiology of spasticity in the adå°“ult and child 13 condition treated with ITB or BoNT at the MCW (TaÂ

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Spasticity and Other Signs of the Upper 3 Motor Neuron Syndrome Nathaniel H. Mayer The noted 19th century neurologist John Hughlings in nature and potentially explained by withdrawal of Jackson was one of the first to recognize that a lesion inhibition from neural tissue normally mediating that of the central nervous system could simultaneously re- behavior. A loss of inhibition resulted in positive signs, sult in the development of positive and negative signs, manifested clinically by stereotypic movements and although he did not believe that the lesion directly postures that were generated by stereotypic linkages of caused the observed signs. In humans, a lesion of the overactive muscle groups. Positive and negative signs descending corticospinal motor system is capable of interact often at the same time. producing the negative sign of muscle weakness dur- ing voluntary effort and, at the same time and in the Inequalities of muscle weakness and muscle over- same muscle, the positive sign of increased resistance activity within and across muscle groups often lead to to passive stretch. The combination is the key feature a net balance of muscle torques acting across joints of muscle spasticity, although it is important to recog- shared in common by these groups. For example, a nize that spasticity is only one of a number of positive positive behavior such as an associated reaction may signs that materialize after an upper motor neuron promote involuntary elbow and finger flexion in a (UMN) lesion. The aggregate of positive and negative hemiÂ

18 iâ•…general overview example, persistent posturing often creates overlap- regarding the value and validity of the testing of mus- ping skin folds, underneath which maceration, ery- cle strength in this population. On further reflection, thema, and malodor flourish. Fixed, excessive postures weakness does not seem to be the best description can cause stretch injury of nerves. Fingernails can dig of impaired volition in UMNS. Two features stand into the palm, causing pressure, pain, and laceration. out. The first is a loss of selective control, that is, a loss Moreover, clinical experience reveals that UMN pat- of independent joint movement and inability to per- terns are more diverse than perhaps commonly noted. form separate movements of individual joints or to For example, a flexed elbow is very common, but an operate and control several joints selectively as a unit extended elbow is also found. A flexed wrist is com- at will. Instead of autonomous control, the patient mon, but an extended wrist is also seen. No matter with UMNS, attempting to make selective joint move- the diversity of pattern, it still reflects an underlying ment, makes multijoint obligatory patterns of move- net balance of torques brought on by positive sign ste- ment that reflect obligatory muscle activations within reotypy and deficient voluntary reversal of negative and across joints. A patient of mine with UMNS after signs. 7 years following a head injury reported to me the re- covery of selective control of thumb, index, and long In summary, a lesion of the descending motor€sysÂ

3â•… Spasticity and Other Signs of the Upper Motor Neuron Syndrome 19 FIGURE 3.2 FIGURE 3.3 Effort by a hemiparetic patient to reach forward and grasp Effort by the same hemiparetic patient to touch her chin, a a door handle does not produce the intended movement totally different task from reaching forward to grasp a door pattern but only components of flexor synergy (shoulder handle, produces a similar meaningless stereotypy of move- retraction, abduction, elbow flexion). ment as in Figure 3.2. grasp an object (see Figure 3.2), the patient dominated by flexor synergy pattern seems unable to generate an by flexor synergy reveals many, if not all, of these joint appropriate set of task-related instructions for trans- movements. When asked to perform a totally differ- mission to muscles of the upper limb in order to pro- ent task (see Figure 3.3), the flexion “synergy-bound” duce meaningful motor behaviors that reflect different patient produces a similar stereotyped behavior. The intentions. Extrapolating from Jackson’s hierarchical patient is locked into an obligatory movement pattern model, high-level neural entities that assemble and or- that is initiated (and terminated) by volitional effort. ganize the movements comprising an action task are It is beyond the scope of this chapter to discuss the seemingly absent in the synergy-bound patient. Lack- nature of voluntary movement, but for now, we note ing higher-level, task-related neural function—a nega- that a number of voluntary features are retained by tive consequence of UMNS—what becomes manifest a patient with UMNS during synergy production, in- clinically with volitional effort is released positive cluding initiation, termination, and varying the speed lower-level neural activity, neural circuits with fixed of synergy pattern movement. The key characteristic muscle linkages across limb segments, resulting clini- of an obligatory flexor or extensor synergy pattern is cally in the stereotypic behavior of obligatory synergy that it is meaningless with respect to the task at hand. patterns. The behavior starts and stops by volitional Upon seeing repeated flexion synergy patterns of move- effort, but its content is not meaningfully related to ment, for example, a remote observer would become an intention-driven task, its instructional set is empty, puzzled about the patient’s intended actions since the and the resulting stereotypic behavior ends up as a movement pattern would be very similar across dif- kind of released default pattern. In the synergy-bound ferently intended tasks. No intent can be discerned, patient, an attempt to produce meaningful action ends no outcome of the movement effort makes sense, up initiating the same repetitive default pattern, time and analogous to aphasia in the language system, the after time. Chronic unidirectional joint movements obligatory flexion synergy pattern seems to produce with little, if any, redirection or reversal of joint move- an inscrutable “aphasic” response or meaningless be- ment are a source of recognizable UMNS patterns, havior. The stereotypic behavior of a flexor synergy generated by persistence of stereotypic, positive or re- pattern is without apparent meaning from the point lease behaviors resulting from higher-level dissolution of view of intentional action. The patient dominated of nervous control, Jackson’s negative sign.

20 iâ•…general overview STRETCH SENSITIVE POSITIVE SIGNS FIGURE 3.5 Stretch Reflexes This patient with UMNS shows different EMG thresholds Spasticity is a term linked to sensitivity of a muscle to (ie, onset of EMG activity) at different wrist angles and dif- stretch. Clinically, stretch sensitivity results in exagger- ferent amounts of EMG activity as a function of the initial ated stretch reflexes. Spasticity as a phenomenon has a velocity of stretch (slowest rate in the left record, fastest specific definition, but it is often used (confusingly) as rate in the middle record). a collective term for all positive signs of UMNS, many of which are not based on sensitivity of a muscle to the examiner performs several trials of stretch at dif- stretch. Strictly speaking, spasticity is a clinical behav- ferent rates of stretch for each trial. The target muscle ior based on increased excitability of phasic and tonic group is stretched through the full (available) range of stretch reflexes that are present in many patients with joint motion at different rates of stretch, for example, a UMN lesion. In normal individuals, passive stretch, slow, moderate, fast, and very fast rates of limb seg- including rapid passive stretch of a muscle group, does ment motion. If the patient is spastic, resistance to not produce noteworthy resistance. Electromyographi- stretch will be felt by the examiner beginning at some cally, little, if any, activity is generated (see Figure 3.4), joint angle during the stretch maneuver, and the inten- and when present, the electromyographic (EMG) ac- sity of resistance will increase the faster the examiner tivity is brief, reminiscent of a tendon jerk response, stretches the muscle group in each subsequent trial. and typically produced only by the most rapid of An increased tendon jerk reflex is also considered a stretches. manifestation of muscle stretch sensitivity and is dis- cussed below. In a spastic patient, however, once the threshold of the EMG activity is triggered at a given degree of The character of the stretch reflex was first identi- muscle stretch, the EMG activity persists until stretch fied by Sherrington’s seminal studies of the cat’s myo- is relinquished (see Figure 3.5). The examiner experi- tatic reflex (5, 6). Before his studies, clinicians were ences increased resistance shortly after threshold activ- well aware of tendon jerk responses, but they thought ity begins, and resistance persists and usually contin- the jerk phenomenon was a local response generated ues to increase until stretch is released. Starting from by muscle. Sherrington’s studies demonstrated that a position of maximal muscle shortening and “rest” stretch responses could be abolished by cutting the (ie, the patient eschews voluntary effort), the defining relevant dorsal roots. By doing so, he established the characteristic of spasticity as experienced by an exam- afferent-efferent nature of the stretch reflex, a circuit iner doing the stretching is a velocity sensitive increase that required transmission through the central nervous in resistance. Velocity sensitivity means the following: system, and he established a basis for understanding later descriptions of clinical reflex phenomena. One FIGURE 3.4 of my favorites is the succinct description of the clini- cal characteristics of spastic stretch reflexes by Peter In normal individuals, passive stretch generates little, if Nathan (7): “Spasticity is a condition in which stretch any, EMG activity. When EMG is present, the activity is of reflexes that are normally latent become obvious. In short duration, reminiscent of a tendon jerk response, and spasticity, the tendon reflexes have a lowered thresh- typically only produced by the most rapid of stretches. old to tap, the response of the tapped muscles is in- creased, and additional muscles besides the tapped one respond; tonic stretch reflexes are affected in the same way” (7). Spasticity and muscle tone are often confused. Tonus or tone of a system generally refers to baseline physiologic activity of a system. For example, normal bowel sounds reflect baseline peristalsis in the intesti-

3â•… Spasticity and Other Signs of the Upper Motor Neuron Syndrome 21 nal tract. Historically, muscle tonus, like baseline ac- resistance that now has a nonneural basis (10). Such tivity in other bodily systems, was originally thought nonneural stiffness may be associated with reduced to be an active phenomenon, the result of a small de- tonic stretch reflex and tendon jerk activity. Under€cirÂ

22 iâ•…general overview was stretched at different velocities. In our view, the 2 bedside ways of assessing phasic and tonic stretch re- flexes are tendon taps and passive muscle lengthening at different rates of stretch, respectively. From a Jack- sonian perspective, phasic and tonic stretch reflexes are stereotypic positive signs. Patients with a stable UMNS have phasic and tonic stretch reflexes that do not vary much from day to day at the bedside. Phasic Stretch Reflexes and Clonus FIGURE 3.6 A phasic jerk response is produced by briskly tap- Clonus at a frequency of 7 Hz developed in both prona- ping a tendon held taut by the examiner. In this way, tors during voluntary underhand reaching performed by a stretch of the tendon-muscle system occurs virtually at patient with UMNS. the instant of tap. Stretch of extrafusal muscle fibers is detected by the muscle spindle and transmitted to that occur at typical frequencies of 6 to 8 Hz (see the central nervous system by Ia afferents that project Figure 3.6). Figure 3.6 shows forearm pronator clo- through the dorsal roots and make a number of con- nus in a patient with stroke and left hemiparesis of nections in the spinal cord. These include monosyn- 4 years duration. Clonic bursts of EMG develop in aptic excitatory connections with homonymous alpha both pronators at a rate of about 8 Hz. Clonus can be motor neurons that innervate the tapped muscle group sustained or unsustained, and it can be stopped by re- and from which afferent outflow originates, along positioning clonic muscles to a shorter length. Clonus with monosynaptic, excitatory connections to heter- is usually associated with other hyperexcitable phasic onymous synergists. The clinical observation of reflex stretch reflexes. In addition to rapid stretch, various radiation to muscles other than the one whose tendon cutaneous stimuli, especially cold or noxious stimula- has been tapped is explained by Burke (14) as the re- tion, may give rise to ipsilateral or even contralateral sult of muscle spindles excited by spreading vibrations clonus (15). Clonus may represent self reexcitation of originating from the site of tap. Monosynaptic con- stretch reflexes in a hyperexcitable stretch reflex loop nections are also made to the Ia inhibitory interneu- (16). Rack et al. (17) viewed clonus as a self-sustaining ron that projects to alpha motor neurons of antagonist oscillation of the stretch reflex pathway, with the€freÂ

3â•… Spasticity and Other Signs of the Upper Motor Neuron Syndrome 23 FIGURE 3.7 played. Note an increase of EMG in the faster stretch record on the right. Differential muscle responses to Using root mean square EMG activity as a quantitative stretch can occur within a group as illustrated in Fig- measure, an increase in root mean square EMG activity is ure 3.9 and likely signifies a lack of homogeneity in observed with increasing rates of stretch, from slow to very the central lesion or in its reorganization. When using fast stretch, applied by a clinical examiner to a patient focal treatments delivered to individual muscles, it is with spastic wrist flexors. important to identify whether all or only some mus- cles of a group are spastic. When a spastic muscle is constant velocity of stretch do not simulate what clini- stretched, reflex activity, once triggered, commonly cians experience because clinicians, unlike machines, continues until the examiner stops stretching and im- cannot maintain a constant stretch velocity when mediately releases the muscle group. In some patients, spastic tension runs high. Figure 3.8 illustrates spastic- reflex activity persists if the examiner continues to hold ity of wrist flexors in an adult with spinal cord injury the muscle statically in a stretched state after dynamic of 2 years’ duration. The EMG responses to stretch stretch has ended. Such activity has been referred to of wrist flexors at 2 different rates of stretch are dis- as a static stretch reflex. The static stretch reflex has been attributed to secondary muscle spindle endings; primary endings of the muscle spindle are known to have dynamic or velocity sensitivity. It remains unclear what operational mechanisms underlie exaggerated stretch reflex activity in the UMNS. Proprioceptive afferent activity generated by muscle stretch is not increased above baseline, and so far, evidence for the theory of increased fusimotor drive has been lacking (20). Evidence seems to favor a reduction in the threshold of the tonic stretch reflex, namely, less afferent input is necessary to trigger stretch FIGURE 3.8 FIGURE 3.9 Passive stretch of wrist flexors in a patient with spinal Differential muscle responses can occur within a muscle cord injury. Note that a constant velocity of stretch is not group as illustrated in this record of passive stretch of el- maintained by the examiner. As the amplitude of stretch bow flexors in a patient with clinical spasticity. Note con- increases, stretch reflex activity superimposed on the ten- siderable stretch-induced activity in brachioradialis with sion length curve of muscle creates increasing resistance minimal responses in brachialis and pronator teres and that counters the examiner’s stretching force, causing the no response in biceps. When using focal treatments for rate of stretch to slow down. spasticity, it would be important to identify which muscles in a group are actually spastic.

24 iâ•…general overview reflex activity in the presence of a UMN lesion. In- not occur (26). However, antagonist muscles are being creased stretch reflex activity resulting from increased stretched during isotonic movements driven by ago- gain in the system, that is, more output reflex activity nists. Therefore, stretch-sensitive antagonist muscles for the same amount of afferent input (“more bang may be subject to stretch reflex activity superimposed for the buck”) is a less favored view. Mechanisms of on the supraspinal drive of cocontraction, and hence, reduced presynaptic and postsynaptic inhibition and the term spastic cocontraction has been applied to impaired recurrent inhibition in the Renshaw system such antagonist activity. Essentially, the antagonists are likely to contribute to the handling of afferent in- have 2 sources of efferent input: supraspinal motor put by a reorganized spinal cord (21). Herman (22) drive and segmental stretch driven reflex activity. It is expressed the view that enhanced reciprocal inhibi- difficult to make practical clinical distinction between tion is characteristic of spastic hemiplegia, whereas supraspinal and segmental stretch reflex drives go- reduced reciprocal inhibition is more characteristic of ing to an antagonist muscle. Such a distinction is not spastic paraplegia. vital to medications with a peripheral mechanism of action, but theoretically, drugs that have relevant cen- Stretch Sensitive Cocontraction tral mechanisms of action could have a differentiating Cocontraction in the UMNS is seen during voluntary effect. Clinically, patients with spastic cocontraction effort. Cocontraction is the simultaneous activation often move with slowness and with great effort. Prob- of agonist and antagonist muscles. The key feature of lems arise when patients experience a restraining or cocontraction is that it occurs during voluntary effort braking action produced by antagonist cocontraction and that it is generated by simultaneous supraspinal during voluntary isotonic movements. When cocon- motor drive to agonist and antagonists (23, 24) (see traction is present, the performance of alternating vol- Figure 3.10). untary movements about a joint will reveal temporal asymmetry. For example, during alternating flexion Physiologically, Humphrey and Reed (25) found and extension movements about the elbow, elbow cocontraction to be activated and deactivated at a cor- flexion is typically quicker, whereas elbow extension tical level. Although cocontraction can be a normal is typically slower because flexors cocontract during mechanism to provide joint stability under particular extension phase. The cocontracting elbow flexors re- circumstances, cocontraction in the UMNS refers to strain elbow extension, causing temporal asymmetry. inappropriate antagonist activation that blunts or even The amplitude of elbow extension may also be altered. reverses agonist-driven movement. Cocontraction, because it originates supraspinally, may occur during Stretch Sensitive Dystonia isometric effort when antagonist muscle stretch does Denny-Brown (27) used the term dystonia to describe FIGURE 3.10 a variety of postural reactions developed by monkeys after ablations of the cerebral cortex were performed. Activity at the onset of movement is seen in both pronator Lesions were independent of, or in addition to, dam- muscles in this record of a voluntary effort to supinate the age to the pyramidal tract. Holding the monkeys in forearm, made by a patient with UMNS. Note how prona- different spatial positions led to a variety of different tor cocontraction eventually limits, even reverses, forearm postural reactions of the limbs, and these limb postures movement. remained persistent in their attitude. Any attempt by an examiner to pull a limb away from its persistent position was met by an increased resistance of springy quality. The limb would fly back to its original posture when released. Denny-Brown called this fixed attitude dystonia, a term signifying a persistent posture main- tained by muscular contraction. He revealed the active nature of dystonia in these monkeys by demonstrating continuous EMG activity in relevant limb muscles. In addition, he showed the dystonia to be efferent. It did not depend on afferent input from the limb because it persisted even after the dorsal roots were cut. Like Jackson’s thinking regarding the effects of brain lesions, Denny-Brown thought that dystonia afÂ

3â•… Spasticity and Other Signs of the Upper Motor Neuron Syndrome 25 havior generated by parts of the motor mechanism FIGURE 3.12 that had direct access to alpha motor neurons (28). Of interest in the present context, he pointed out that At rest, persistent dystonic EMG activity was recorded from monkeys who underwent various cortical ablations the pectoralis major, and a lesser level of activity was also did not have spastic features, such as tendon jerk present in long head of triceps. The patient was asked to sit hyperreflexia. Efferent drive at rest was supraspinal quietly and relax. Pectoralis major is an adductor and inter- in origin and seemingly was unrelated to afferent- nal rotator of the shoulder (correlate with Figure 3.11). efferent stretch reflex arcs. However, Denny-Brown also indicated that the dystonia of his monkeys was stretching eased her clinical complaint of shoulder affected by the degree of stretch placed on a muscle, stiffness, but passive stretching had to be repeated fre- and in humans with UMNS, dystonic (at rest) activ- quently. Dystonia “sensitive to stretch” can result in ity in a muscle can be modified by prolonged stretch a lessening of the dystonic activity as a result of sus- (29, 30). The presence of muscle activity at rest (ie, tained stretch. Phasic or brief duration stretch can elicit without obvious source of afferent input, phasic a spastic (resistive) reaction. Some are of the€opinion stretch, or volitional effort), sensitive to prolonged that delayed relaxation after voluntary contraction (tonic) stretch, has been called spastic dystonia. For ex- characterized by continuous firing of motor units is ample, Figures 3.11 and 3.12 illustrate a patient with also a form of spastic dystonia (31). right hemiparesis secondary to a stroke sitting quietly at rest. If available, EMG equipment can help identify persistent muscle activity that is potentially consistent Persistent EMG activity was recorded from the with the dystonic form of muscle overactivity. How- pectoralis major, and a lesser level of activity is also ever, without using this confirmation method, it may noted in long head of triceps. The clinical posture of be risky to assume that all patients with persistent limb an adducted/internally rotated shoulder is noted in the postures have underlying muscle activity that sustains figure. The shoulder adductors were spastic on pas- the posture. Passive tissue stiffness alone may be suffi- sive stretch. Range of motion exercises with sustained cient to hold a posture. Stretching a muscle group may elicit a spastic response that brings the stretched mus- FIGURE 3.11 cle back to its equilibrium position at which it will re- Note an adducted, internally rotated shoulder at rest in main in equilibrium, balanced by the passive rheologic this hemiparetic patient due to stroke. properties of muscles and other soft tissues surround- ing the joint. Muscle contracture, that is, fixed shorten- ing, holds a limb in a fixed posture as does heterotopic ossification. Activity generated by an unnoticed as- sociated reaction could mimic dystonic posturing, for example, standing “quietly at rest” while actually weightbearing on a cane with the contralateral limb could promote ipsilateral elbow flexor activity and a flexed elbow posture. Associated reactions in UMNS are described in the next section. Their essential feature is that voluntary activity in one part of the body accom- panies involuntary activity in another.

26 iâ•…general overview POSITIVE SIGNS THAT ARE NOT muscles and more joints are recruited. Flexor spasms STRETCH-SENSITIVE are more common in patients with lesions of the spi- nal cord than patients with supraspinal hemiplegia. Flexor and Extensor Spasms The studies of Herman (33) indicated that the manner in which afferent activity was transmitted through the A characteristic feature of the UMNS, according to spinal cord was diffferent for paraplegic and hemiple- Lance (32), is the release of flexor reflex afferent ac- gic patients. tivity. The flexor reflex is a polysynaptic reflex that results in contraction of flexor muscles across several Associated Reactions limb segments. It is generated by afferent stimuli col- lectively known as flexor reflex afferents. These affer- An associated reaction is another form of involuntary ents may include cutaneous receptors responding to muscle overactivity seen in the UMNS. An associated touch, temperature, and pressure and nociceptors re- reaction refers to involuntary activity in one limb that sponding to painful stimuli, secondary endings from is associated with a voluntary movement effort made in muscle spindles (group II afferents), and free nerve another limb. Figure 3.13(A)-(C) shows the sequence of endings scattered diffusely in skeletal muscles. The right elbow motion in a right hemiplegic patient mov- polysynaptic flexor reflex has a long latency (twice the ing from a sitting position to a standing position. Body latency of a monosynaptic tendon jerk) due to slow motion including voluntary arm and leg movements afferent conduction into the cord and to central delay. on the uninvolved left side was most active during the Flexor reflex afferent activity ascends and descends stand up phase in the middle photo. Note how an as- in the cord, synapsing in the internuncial pool, a sys- sociated reaction of the right elbow flexors developed tem of spinal interneurons influenced by inputs from on the hemiparetic side and produced an increase in peripheral as well as central sources. Compared with right elbow flexion in the middle photo compared with segmental stretch reflexes, the time course of polysyn- elbow flexion during sitting (left photo) and during the aptic flexor reflexes is slower, and unlike segmental subsequent standing equilibrium phase (right photo). stretch reflexes, flexor reflexes represent coordinated Associated reactions were first described by Walshe in activity of groups of motor neurons spanning many 1923 (34). segments. Polysynaptic reflexes result in muscle con- traction across multiple joints, sometimes bilaterally. Following Jackson’s release phenomena formula- Recruitment of flexor muscles across a number of tion, Walsh referred to associated reactions as “released joints is an example of an interjoint reflex that can pro- postural reactions deprived of voluntary control.” tect tissues subject to noxious stimulation. Extensor Associated reactions may be due to disinhibited reflexes are also polysynaptic and may contribute to spread of voluntary motor activity into a limb affected body support functions. Flexor and extensor reflexes by the UMN syndrome. Figure 3.14 shows a patient may be the substrate for more complex coordinative with right hemiparesis throwing a ball with the left patterns, such as locomotor stepping generators. upper limb. The patient did not involve the right up- per limb with the throw, having no volitional control After a UMN lesion, particularly after a spinal of this limb. Figure 3.14 reveals the presence of EMG cord lesion, disinhibition of the flexor reflex becomes activity in the right-sided shoulder muscles associated more prominent clinically. Flexor reflexes can range with the patient’s effort to throw the ball with her from the familiar Babinski sign to a mass triple flex- left hand. The intensity of an associated reaction in ion reflex involving the hip, knee, and ankle. A pa- the limb with UMN may depend on how much effort tient may call the flexor reflex a muscle “spasm.” but is made by the voluntary limb. Dewald and Rymer a careful history will reveal that the patient refers to (35) thought that impaired descending supraspinal multijoint activity rather than focal spasm of a single commands were involved in generating an associated muscle group crossing one joint. Flexor reflex afferents reaction. They hypothesized that unaffected bulbospi- from limbs and enteroceptors from bladder and bowel nal motor pathways may have taken over the role of can singly or in combination promote polysegmental transmitting descending voluntary commands when reflex activity with contraction of muscles about the the other UMN tracts were damaged. ankle, knee, hip, abdominals, even paraspinals. A large, sudden rise in intra-abdominal pressure caused Although clinicians often seem more concerned by contraction of the rectus abdominis can result in about spasticity than other positive phenomena of the urinary incontinence as part of the phenomenon. In UMNS, the clinical impact of spasticity may be less UMN lesions, the onset threshold of the flexor reflex is than advertised when one ponders how often patients reduced, contraction intensity is increased, and more and caregivers might actually stretch spastic muscles at rates that would elicit intense resistance. We have

3â•… Spasticity and Other Signs of the Upper Motor Neuron Syndrome 27 AC B FIGURE 3.13 Note changes in elbow angle as the patient moves through the sequence: (A) sitting, (B) standing up, (C) maintaining a standing position. The photos illustrate an associated re- action of involuntary right elbow flexion during the stand- ing up phase (B) as voluntary movement efforts in other limbs are taking place. observed patients and caregivers performing limb might be expected to provide ample opportunity for manipulations at slow rates of stretch to avoid or min- the development of muscle overactivity generated by imize rate-sensitive spastic resistance. We also note associated reactions. that what often passes for spastic resistance is, in large measure, decreased tissue compliance due to changes Muscle Rheology in the intrinsic rheologic properties of muscle. Associ- ated reactions, on the other hand, may be unavoid- Although Jackson conceived of positive and negative able because voluntary movement efforts of a patient signs of the UMNS with respect to nervous system pro- with UMNS must necessarily go on throughout the duction of motor behavior, others subsequently became day (36). cognizant of how changes in peripheral soft tissue could also affect extremity movements. Changes in the rheo- High-intensity voluntary efforts required dur- logical properties of soft tissues, especially muscle, are ing transfers, gait, and many activities of daily living

28 iâ•…general overview FIGURE 3.14 A patient with right hemiparesis throwing a ball with her left hand. The patient did not involve the right upper limb with the throw, having no volitional control of this limb. The figure reveals EMG activity in right-sided shoulder muscles as an associated reaction to the patient’s voluntary ball throw with her left upper limb. Note that these right-sided muscles, pec- toralis major, teres major, and latissimus dorsi, are adductors and internal rotators. The patient presented clinically with the UMN pattern of an adducted/internally rotated shoulder. important clinically (37). Muscle contracture, a physi- guishing between dynamic tension of muscle over- cal shortening of muscle length, limits the operating activity and static rheologic tension can be difficult. range of joint motion and, as such, has a broad influ- O’Dwyer et al. (38) suggested that what appears clini- ence on the performance of activities of daily living and cally as spasticity after stroke is really increased mus- mobility. Muscle contracture is often accompanied by cle stiffness and muscle contracture. They suggested physical shortening of other soft tissues such as fascia, that mechanical and biological changes in soft tissues nerves, blood vessels, and skin. (Muscle contracture, played a major role in resistance to both passive and an invariant physical state of fixed tissue shortening, active movements. Muscles immobilized in a short- is not to be confused with muscle contraction, a dy- ened position for long periods became shorter and namic, variable state of internal shortening produced stiffer. When muscle overactivity developed in these by the sliding action of actin and myosin filaments shortened muscles, tension was generated at shorter within a muscle fiber. Contracture implies that muscle lengths. A lack of voluntary contraction in the antago- length remains the same even if one were to block all nists of these shortened muscles prevented their natural muscle contraction by local or general anesthesia.) reextension, leading to a continuation of the process of stiffness and fixation. In the upper limb, muscles The development of contracture is promoted by that typically shorten include shoulder adductors/ a number of processes that start when an acute UMN internal rotators, forearm pronators, and elbow, lesion occurs: (1) paresis impairs cycles of shortening wrist, and finger flexors. In the lower limb, muscles and lengthening of agonist and antagonist muscles that typically shorten include ankle plantar flexors, caused by everyday muscle use; (2) the force of grav- toe flexors, and hip and knee flexors. The position of ity generates positional effects on limb segments and any given joint results from a net balance of static and joints; (3) positional effects are also created by a net dynamic torques of muscles acting across the joint, as balance of static soft tissue forces traversing joints; well as rheologic properties of related soft tissues. Vari- and (4) the various involuntary UMN motor behav- ability in central lesion recovery and reorganization iors described above lead to a net balance of dynamic can lead to other types of UMN patterns such as the limb torques acting across joints, resulting in the de- intrinsic plus hand and the hyperextended wrist. velopment of chronic 1-way positioning of joints that typically leads to stiffness and contracture. Because Based on studies of patients with cerebral palsy muscle relaxant medications affect dynamic muscle (CP), contractures are thought to arise from muscle contraction only, a clinical picture dominated by€con- fibers that have fewer sarcomeres in series and, there- tracture will not respond to such drugs. Physical fore, are shorter than normal (39). Sarcomere lengths methods are necessary to undo contractures. Distin- are greater than normal when muscle fibers with fewer

3â•… Spasticity and Other Signs of the Upper Motor Neuron Syndrome 29 sarcomeres in series are stretched during normal move- therapeutic exercise or surgical lengthening. Current ment. These longer sarcomeres are thought to be the theory believes that sarcomeres operating over a more main reason that muscles of patients with CP have normal range will result in a reduction in passive ten- excessive passive tension. Similar processes might con- sion along with better force generation if the muscle- ceivably account for elevated passive tension in adults tendon length is adjusted appropriately. The study of with UMNS. However, Friden and Lieber (40), reexam- Friden and Lieber suggests that this view may have to be ining the issue of excessive passive tension in CP, have altered. described the mechanical properties of isolated muscle fiber segments obtained from spastic patients with CP MALADAPTIVE CONSEQUENCES undergoing surgical correction of flexion contractures. OF THE UMNS They found that single fibers developed passive tension at significantly shorter sarcomere lengths than fibers We have offered the argument that the interaction be- taken from subjects without spasticity. Muscle fibers tween stereotypic positive and negative signs of the of patients were almost twice as stiff as controls, and UMNS results in chronic 1-way joint attitudes giving resting sarcomere lengths were shorter. Friden and rise to common patterns of UMN dysfunction. Me- Lieber also found that the cross-sectional area of spas- chanical and biological changes in soft tissues, such tic fibers was less than one third of normal fibers. This as muscle, play an important role in resistance to pas- resulted in greater stress forces when spastic fibers were sive and active movements. Generated by the various passively stretched. Their study suggested that sarco- forms of positive signs, muscle overactivity, superim- meres in CP do not have to be stretched beyond normal posed on emerging soft tissue changes, contributes to lengths to develop excessive passive tension. From this a net balance of torques that promotes and maintains perspective, their study challenges the assumption that tissue shortening and chronic 1-way positioning of up- tendon lengthenings or even stretching exercises that per and lower limb joints (see Figure 3.15). The nega- aim to allow a muscle to achieve normal sarcomere tive sign of impaired limb usage and weak voluntary lengths will lessen passive tension to normal levels. The contraction of the antagonists of shortened muscles study of Friden and Lieber points to a process of con- prevents range of motion in the opposite direction, siderable structural remodeling of spastic muscle tissue contributing to the continuation of the process of components. In that regard, their study challenges the postural fixation and its maladaptive consequences current theoretical framework suggesting that muscle- for patient care. Stereotypic movement patterns and tendon lengths need to be appropriately adjusted by FIGURE 3.15 FIGURE 3.16 This figure illustrates the concept that UMN postural and Clenched fist and flexed wrist deformities in this hemipa- movement patterns reflect a net balance of torques gener- retic patient illustrate the potential for maceration, organ- ated by combinatorial interactions of various positive and ism overgrowth, and malodor as moisture accumulates negative UMN signs. and persists between fingers in contact with palm and in redundant tissue folds at the wrist.

30 iâ•…general overview postures, generated by positive signs and not reversed CONCLUSIONS because of negative signs, promote maladaptive UMNS consequences (41) under the following personal health Skeletal muscle is a key effector organ of the nervous categories exemplified for the upper limb: system. Skeletal muscle develops torque about a joint in response to efferent neural activity coming from the Skin Integrity: Clenched fist (see Figure 3.16) central nervous system. Normally,€everyÂ

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Ancillary Findings Associated With 4 Spasticity Cindy B. Ivanhoe It is challenging to describe the “ancillary features and incorporating these other features creates an artificial associated findings” connected with spasticity and the divide (1). upper motor neuron syndrome (UMNS). Aside from the usual problems we think about in terms of spas- Another consideration is how spasticity affects ticity, muscle overactivity also has the potential to the body system being discussed, that is, does it di- affect a wide range of body systems and functions. rectly affect the end organ or is the muscle overac- How these interactions, directly and indirectly, will af- tivity reducing function in the body system? Both fect an individual is determined by a range of factors. options must be considered. Detrusor-sphincter dys- These factors include diagnosis, duration, age of on- synergia would be an illustration of the direct effects set, and chronicity. Inherent in this discussion is how of spasticity on an end organ, whereas difficulty with the UMNS affects function across organ systems. For toileting may occur from impaired posture, balance, example, skin is affected by positioning and external and motor control. Drooling may be the result of de- interventions, such as orthotics and casts. Skin lesions, creased oromotor control from spasticity, weakness, such as pressure sores, will further affect the presenta- incoordination, or dystonia (Figure 4.1). Sedation due tion of tone because they present a noxious stimulus. to medications may increase these baseline impair- Swallowing and breathing are affected by positioning, ments even if they reduce tone. weakness, tone, and coordination. Cognition and so- cialization can be affected by postures and dysarthria. Lastly, in some situations, the combination of The integrated effects of the UMNS coalesce to create spasticity effects on multiple body systems impairs individual functional issues. Treatment approaches to higher-order functional states. Sexuality may be af- one feature of the presentation consequently affect fected by restricted muscle movement or contracture, other aspects of overall functioning. aside from cognitive concerns and psychological is- sues. Depression, altered body image, change in role, Although the term spasticity is often used to de- and self-esteem significantly affect the overall function scribe a symptom complex that includes but is not lim- level in this realm. ited to spasticity, it is important to untangle the effects of spasticity from other components of the UMNS, The following chapter covers a variety of symp- for example, negative features (weakness, decreased toms, complexes, and body systems that are affected dexterity, etc.) and sensory cognitive and behavioral by and are associated with UMNS. Oral medications changes. Separating the effects of spasticity without have their place in the management of UMNS; how- ever, they have limitations due to their side-effect pro- files, including effects on endurance and cognition. 33