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Neuromuscular Disorders: Management and Treatment E-Book

Neuromuscular Disorders: Management and Treatment E-Book

Tulio E. Bertorini | Tulio E. Bertorini

(2010)

Additional Information

Book Details

Abstract

Neuromuscular Disorders presents a multi-disciplinary approach to the management and therapeutic treatment of the full range of neuromuscular disorders and resulting complications. Dr. Tulio Bertorini and a contributing team of the world’s leading authorities in the field provide the latest tools and strategies for minimizing disability and maximizing quality of life.

  • Effectively treat your patients using the latest management tools and targeted therapeutic strategies.
  • Manage all neuromuscular disorders as well as resulting complications through comprehensive coverage of diagnosis and evaluations, treatments, and outcomes.
  • Apply the multi-disciplinary approach of an expert in clinical neuromuscular care and a team of world-renown contributors.
  • Easily refer to tools for diagnosis, treatment algorithms, and drug tables included throughout the text.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Neuromuscular Disorders: Treatment and Management iii
Copyright Page iv
Dedication v
Preface vii
Acknowledgments ix
Contributors xi
Contents xv
Part I: General Principles in the Treatment and Management of Neuromuscular Disorders 1
Chapter 1: Introduction: Evaluation of Patients with Neuromuscular Disorders 3
Medical History and Symptoms 3
Physical Examination 3
Diagnostic Tests 7
Electrophysiologic Tests 10
Histologic Tests 11
References 18
Chapter 2: Respiratory Complications in Neuromuscular Disorders 21
Management of Neuromuscular Diseases Resulting in Chronic Respiratory Failure 21
Management of Neuromuscular Diseases Resulting in Acute Respiratory Failure 30
Acute Exacerbations of Chronic Neuromuscular Respiratory Failure 31
References 31
Chapter 3: Cardiac Complications of Neuromuscular Disorders 33
Duchenne Muscular Dystrophy 33
Becker Muscular Dystrophy 33
Carriers of Duchenne Muscular Dystrophy and Becker Muscular Dystrophy 35
X-Linked Dilated Cardiomyopathy 35
Limb-Girdle Muscular Dystrophy 35
Myotonic Dystrophy 36
Emery-Dreifuss Muscular Dystrophy 36
Facioscapulohumeral Dystrophy 36
Other Neuromuscular Disorders 37
Diagnosis and Evaluation 37
Pathology 41
Treatment 41
Preoperative Assessment 45
Experimental Therapies 45
Monitoring 45
Summary 47
Conclusion 47
References 47
Chapter 4: Gastrointestinal Complications of Neuromuscular Disorders 51
Pharynx and Esophagus 51
Stomach and Duodenum 55
Small Intestine 56
Large Intestine and Anal Sphincter 57
Peripheral Neuropathy and the Gastrointestinal System 57
Conclusion 58
References 58
Chapter 5: Autonomic Dysfunction in Neuromuscular Disorders 61
Overview of the Autonomic Nervous System 61
Evaluation of the Autonomic Nervous System 63
Types of Autonomic Neuropathy 66
Symptomatic Treatment of Autonomic Disorders 71
References 75
Chapter 6: A Practical Approach to the Treatment of Painful Polyneuropathies 79
Definitions and Overview 80
Nerve Anatomy 81
Overview of Peripheral Neuropathic Pain Pathophysiology 81
Modification of Sensory and Nociceptive Afferents 84
Evidence-Based Medicine 87
Approved and \"Off-Label\" Medication Uses 89
Previous Treatment Recommendations 89
General Approach to the Patient with Painful Neuropathy 90
First-Line\" Neuropathic Pain Treatments 92
Second-Line\" Neuropathic Pain Treatments 95
Ancillary Treatments 96
Conclusion 96
References 97
Chapter 7: Principles and Guidelines of Immunotherapy in Neuromuscular Disorders 101
Basics of the Immune Response 101
Autoimmunity 102
Immunotherapy 103
Evidence-Based Medicine and Treatment of Autoimmune Neuromuscular Disorders 109
References 111
Chapter 8: Rehabilitation in Neuromuscular Disorders 115
Management of Muscle Weakness 116
Orthoses and Mobility Aids 119
Assistive Devices for Activities of Daily Living 124
Environmental Evaluations and Modifications 125
Orthopedic Considerations 126
Respiratory Failure 127
Dysarthria 127
Dysphagia 128
Pain 129
Mood Disorders 130
Cognitive Dysfunction 131
Quality of Life 132
Roles of the Caregiver and Family 133
Palliative Care 134
References 134
Chapter 9: Orthopedic Surgery in Neuromuscular Disorders 137
Muscular Dystrophy 137
Hereditary Motor and Sensory Neuropathies 144
References 151
Chapter 10: Perioperative Managementof Patients withNeuromuscular Disorders 155
Perioperative Management in Specific Neuromuscular Disorders 155
Malignant Hyperthermia Syndrome 157
Complications of Major Surgery and Organ Transplantation 159
Neuromuscular Disorders Acquired in the Intensive Care Unit 161
References 164
Part II: Treatment and Management of Specific Neuromuscular Disorders 167
Chapter 11: Treatment and Management of Adult Motor Neuron Diseases 169
Diagnosis and Evaluation 169
Treatment and Management 174
References 177
Chapter 12: Treatment and Management of Spinal Muscular Atrophy and Congenital Myopathies 179
Diagnosis and Evaluation 179
Treatment and Management 182
Novel Therapies 185
Conclusion 187
References 187
Chapter 13: Treatment and Management of Hereditary Neuropathies 191
Hereditary Motor and Sensory Neuropathies 195
Hereditary Motor Neuropathy 199
Hereditary Sensory and Autonomic Neuropathies 199
Familial Amyloidic Polyneuropathy 200
Friedreich Ataxia and Other Spinocerebellar Ataxias 202
Inherited Dyslipidemic Neuropathies 203
Lysosomal Storage Diseases: Fabry Disease 204
Lysosomal Leukodystrophies: Metachromatic Leukodystrophy and Krabbe Disease 206
Peroxisomal Disorders: Adrenomyeloneuropathy and Refsum Disease 207
Porphyric Neuropathy 208
Conclusion 211
Acknowledgments 211
References 211
Chapter 14: Treatment and Management of Autoimmune Neuropathies 215
Guillain-Barré Syndrome 215
Chronic Inflammatory Demyelinating Polyradiculoneuropathy 218
Multifocal Motor Neuropathy 220
Lewis-Sumner Syndrome 221
Paraproteinemic Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Monoclonal Gammopathy of Undetermined Significance 221
Paraproteinemic Neuropathies: Primary Systemic Amyloidosis 223
Paraproteinemic Neuropathies: POEMS Syndrome (Osteosclerotic Myeloma) 224
Vasculitis 224
Cryoglobulinemia 225
Nonvasculitic Neuropathies Associated with Connective Tissue Disease 226
Autoimmune Lumbosacral Plexopathy 226
Autoimmune Brachial Plexopathy 227
Celiac Disease 228
Paraneoplastic Sensory Neuronopathy 228
References 229
Chapter 15: Treatment and Management of Infectious, Granulomatous, and Toxic Neuromuscular Disorders 237
Infections of Nerve and Muscle 237
Granulomatous Diseases of Nerve and Muscle 246
Toxic Neuropathies and Myopathies 247
References 257
Chapter 16: Treatment and Management of Segmental Neuromuscular Disorders 261
Radiculopathy 261
Plexopathies 269
Focal Neuropathies 275
Focal Neuropathy Syndromes 276
References 282
Chapter 17: Treatment and Management of Disorders of Neuromuscular Hyperexcitability 285
Central Disorders 285
Disorders of Peripheral Nerve Hyperexcitability 290
Primary Disorders of Muscle 293
References 300
Appendix 17-1.. Exercise Tests for Periodic Paralysis 306
Chapter 18: Treatment and Management of Disorders of the Neuromuscular Junction 307
Myasthenia Gravis 307
Lambert-Eaton Myasthenic Syndrome 322
Botulism 326
Congenital Myasthenic Syndromes 331
Tick Paralysis 334
Snakebite Myasthenic Syndrome 335
Organophosphate Intoxication 337
Hypermagnesemia-Induced Paralysis 339
References 340
Chapter 19: Treatment and Management of Muscular Dystrophies 343
Dystrophinopathies 343
Limb-Girdle Muscular Dystrophies 354
Other Distal Muscular Dystrophies 356
Emery-Dreifuss Muscular Dystrophy 356
Facioscapulohumeral Muscular Dystrophy 358
Myotonic Dystrophy 359
Oculopharyngeal Muscular Dystrophy 361
Congenital Muscular Dystrophy 362
References 364
Chapter 20: Neuromuscular Manifestations of Acquired Metabolic, Endocrine, and Nutritional Disorders 373
Endocrine Disorders 373
Neuromuscular Complications of Uremia 383
Malnutrition and Vitamin Deficiencies 384
Acknowledgment 389
References 389
Chapter 21: Treatment and Management of Autoimmune Myopathies 395
Dermatomyositis 395
Polymyositis 396
Inclusion Body Myositis 397
Eosinophilic Syndromes and Fasciitis 397
Diagnosis and Evaluation 398
Treatment and Management of Inflammatory Myopathies 402
References 405
Chapter 22: Treatment and Management of Hereditary Metabolic Myopathies 409
Muscle Disorders of Glycogen Metabolism 409
Muscle Disorders of Lipid Metabolism 417
Mitochondrial Myopathies 421
Rhabdomyolysis 426
Conclusion 426
Suggested Reading 427
References 427
Index 431