Rehabilitation of neuromuscular disease

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Veröffentlicht: Philadelphia [u.a.] Saunders 1998
Schriftenreihe:Physical medicine and rehabilitation clinics of North America 9,1
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adam_text REHABILITATION OF NEUROMUSCULAR DISEASE CONTENTS Foreword xi George H. Kraft Preface xiii Gregory T. Carter The Role of Physiatry in the Management of Neuromuscular Disease 1 William M. Fowler, Jr, Gregory T. Carter, and George H. Kraft Patients with neuromuscular disease (NMD) are complex and pose a myriad of clinical problems. Management is best carried out by a team consisting of physicians, physical, occupational, and speech therapists, social workers, vocational counselors, and psychologists, among others. Physiatrists are well suited to this type of multi disciplinary care and can play a significant role in the care of these patients. Clinical Approach to the Diagnostic Evaluation of Progressive Neuromuscular Diseases 9 Craig M. McDonald This article reviews the clinical approach to the diagnostic evalua¬ tion of progressive neuromuscular diseases with an emphasis on relevant neuromuscular history, family history, clinical examina¬ tion findings, laboratory studies, and the role of muscle biopsy. In some instances, a precise diagnosis is not medically possible; however, the accurate characterization of an individual patient within the most appropriate NMD clinical syndrome often allows PHYSICAL MEDICINE AND REHABILITATION CLINICS OF NORTH AMERICA VOLUME 9 • NUMBER 1 • FEBRUARY 1998 V the clinician to provide the patient and family with accurate prognostic information and anticipatory guidance for the future. Molecular Basis of Neuromuscular Disease 49 Phillip F. Chance, Tetsuo Ashizawa, Eric P. Hoffman, and Thomas O. Crawford For many neuromuscular disorders, the chromosomal location is known, the causal gene has been identified, and direct application of this knowledge may be made in a clinical setting. The benefits resulting from molecular based methods include improved diag¬ nostic accuracy and genetic counseling for patients and other at risk family members. This chapter discusses in detail four of the most frequently encountered neuromuscular disorders. These diseases include spinal muscular atrophy, Charcot Marie Tooth neuropathy, Duchenne/Becker type muscular dystrophy, and myotonic dystrophy. Electrodiagnosis in Neuromuscular Disease 83 Lisa S. Krivickas This article provides a general approach to the electrodiagnostic evaluation of the patient with neuromuscular disease. The elec¬ trodiagnostic findings in patients with various forms of periph¬ eral neuropathy, motor neuron disease, neuromuscular junction disorders, and myopathy are presented. The pediatric electrodiag¬ nostic evaluation is also discussed. The Role of Exercise in Neuromuscular Disease 115 David D. Kilmer Persons with hereditary neuromuscular diseases generally lead a sedentary lifestyle, placing them at risk for diseases associated with inactivity and causing weakness, which compounds their primary disease. Although research is lacking in this area, evi¬ dence to date suggests that this population may benefit from both strengthening and aerobic fitness training programs. Overwork weakness has not been demonstrated in controlled trials of exer¬ cise, but clinicians need to be prudent in their recommendations, encouraging alternating periods of physical activity with sched¬ uled rest. Future investigations should focus on the primary symptom of fatigue, and quantify changes in the ability to work and participate in physical activities as outcome measures of an exercise program. Nutritional Aspects of Neuromuscular Disease 127 Megan A. McCrory, Nancy C. Wright, and David D. Kilmer Both the rapidly progressive Duchenne muscular dystrophy (DMD) and the slowly progressive neuromuscular diseases (SP NMDs) are associated with loss of skeletal muscle, gain of excess body vi CONTENTS fat, and changes in energy metabolism and physical activity over time. This article reviews several nutritional techniques and their adequacy in monitoring changes in the nutrtional status of these individuals as the diseases progress. In addition, nutritional inter¬ ventions which aim to counteract the effects of the disease process and increase the quality of life in individuals with DMD are reviewed. Finally, new research on physical activity and body composition, and the potential for development of secondary chronic diseases in individuals with SP NMD are discussed. Management of Cardiac Complications in Neuromuscular Disease 145 William R. Lewis and Sanjay Yadlapalli Cardiovascular complications are common and varied in neuro¬ muscular diseases. Knowledge of the complications specific to each disease is essential for appropriate screening for cardiovas¬ cular disease. Appropriate treatment of complications varies be¬ tween neuromuscular diseases and draws primarily on experi¬ ence from patients without neuromuscular disease. This article details the known cardiovascular complications and treatments for some of the major neuromuscular diseases. Management of Pulmonary Complications in Neuromuscular Disease 167 Joshua O. Benditt Neuromuscular diseases frequently affect the respiratory system by impairing function of the mechanical respiratory pump that can lead to progressive hypoventilation, atelectasis, pneumonia, respiratory failure, and even death. Recognition of respiratory impairment can be difficult due to subtle signs and symptoms; therefore, the clinician must maintain a high index of suspicion in all patients with neuromuscular disease. Invasive and noninva sive mechanical ventilation, as well as a variety of respiratory therapy techniques, can be used to prevent these complications. Limb Contractures in Progressive Neuromuscular Disease and the Role of Stretching, Orthotics, and Surgery 187 Craig M. McDonald Limb contractures are common impairments in selected progres¬ sive neuromuscular disease (NMD) conditions and can lead to such increased disabilities as worsening motor performance, de¬ creased mobility, loss of ADL skills, and pain. This article reviews the pathogenesis of joint contractures in NMD, the prevalence of specific contractures among many common NMD conditions, and the role of optimal positioning, stretching, upright ambulation, orthotics, and splinting, as well as surgery in the prevention and management of contractures. CONTENTS Vli Spinal Deformity in Neuromuscular Disease: Natural History and Management 213 Dennis A. Hart and Craig M. McDonald Severe spinal deformity in progressive neuromuscular disease (NMD) leads to multiple problems, including poor sitting bal¬ ance, difficulty with upright seating and positioning, pain, diffi¬ culty in attendant care, and potential exacerbation of underlying restrictive respiratory compromise. Severe scoliosis and pelvic obliquity can in some instances completely preclude upright sit¬ ting in a wheelchair. This article reviews the prevalence, natural history, and management of scoliosis in neuromuscular diseases at greatest risk for progressive spinal deformity. Quality of Life: Issues for Persons with Neuromuscular Disease 233 R. Ted Abresch, Nancy K. Seyden, and Mark A. Wineinger Increasingly, assessment of quality of life is being used to deter¬ mine appropriate medical care, evaluate the effectiveness of reha¬ bilitative treatments, and ascertain the impact of disability. This article documents the subjective assessment of quality of life by individuals with neuromuscular diseases and their health care providers; the article also demonstrates how the attitudes and expectations of health care professionals may affect health out¬ comes. The effect of impairments, disability, and handicap are examined in relationship to life satisfaction. Factors that signifi¬ cantly increase or decrease quality of life for individuals with neuromuscular diseases are identified and discussed. Cognitive, Psychosocial, and Educational Issues in Neuromuscular Disease 249 Barbara J. Sigford and Richard A. Lanharn, Jr Research on the cognitive function of persons with neuromuscu¬ lar diseases is critically reviewed. Emphasis is placed on Du chenne muscular dystrophy and myotonic muscular dystrophy, as these groups have received the majority of attention by re¬ searchers. Implications for psychosocial and educational function are discussed. A general critique of research methodology is provided to assist in interpreting often conflicting findings. Comprehensive Management of Amyotrophic Lateral Sclerosis 271 Gregory T. Carter and Robert G. Miller Amyotrophic lateral sclerosis (ALS) is a rapidly progressive mo¬ tor neuron disease that poses a myriad of clinical problems. Patients with ALS are best treated in a multidisciplinary setting involving physicians, clinical nursing specialists, and physical, occupational, speech, and respiratory therapists, as well as psy viii CONTENTS chologists and social workers. Palliative and rehabilitative strate¬ gies may ease suffering, while new treatments provide hope for effective treatment of this disease. Animal Models of Neuromuscular Diseases: Pathophysiology and Implications for Rehabilitation 285 R. Ted Abresch, Sandra A. Walsh, and Mark A. Wineinger This article explains how animal models can be utilized to de¬ velop rehabilitation strategies to help treat the pathophysiology of neuromuscular diseases (NMDs) and illustrates how these models can be used to test the effects of physical interventions, such as exercise, stretching, and immobilization, in this setting. Index 301 Subscription Information Inside back cover CONTENTS IX
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series Physical medicine and rehabilitation clinics of North America
series2 Physical medicine and rehabilitation clinics of North America
spellingShingle Rehabilitation of neuromuscular disease
Physical medicine and rehabilitation clinics of North America
Maladies neuromusculaires - Rééducation et réadaptation
Neuromusculaire aandoeningen gtt
Revalidatie gtt
Neuromuscular Diseases rehabilitation
Neuromuskuläre Krankheit (DE-588)4075332-3 gnd
Rehabilitation (DE-588)4049078-6 gnd
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(DE-588)4049078-6
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title Rehabilitation of neuromuscular disease
title_auth Rehabilitation of neuromuscular disease
title_exact_search Rehabilitation of neuromuscular disease
title_full Rehabilitation of neuromuscular disease guest ed.: Gregory T. Carter
title_fullStr Rehabilitation of neuromuscular disease guest ed.: Gregory T. Carter
title_full_unstemmed Rehabilitation of neuromuscular disease guest ed.: Gregory T. Carter
title_short Rehabilitation of neuromuscular disease
title_sort rehabilitation of neuromuscular disease
topic Maladies neuromusculaires - Rééducation et réadaptation
Neuromusculaire aandoeningen gtt
Revalidatie gtt
Neuromuscular Diseases rehabilitation
Neuromuskuläre Krankheit (DE-588)4075332-3 gnd
Rehabilitation (DE-588)4049078-6 gnd
topic_facet Maladies neuromusculaires - Rééducation et réadaptation
Neuromusculaire aandoeningen
Revalidatie
Neuromuscular Diseases rehabilitation
Neuromuskuläre Krankheit
Rehabilitation
Aufsatzsammlung
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