Contributors |
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xv | |
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1 The Anatomy And Molecular Architecture Of The Neuromuscular Junction |
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1 | (36) |
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1 | (2) |
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The Innervation Of Muscle |
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3 | (1) |
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3 | (1) |
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4 | (11) |
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Small Clear Synaptic Vesicles |
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Synaptic Vesicles Move to and Dock at the Active Zones |
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Priming of Docked Synaptic Vesicles |
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Other Proteins Modulating Exocytosis |
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Presynaptic Cytoskeletal Components |
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The Active Zone and the Voltage-Gated Ca2' Channels |
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Voltage-gated K' Channels of the Presynaptic Membrane |
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Endocytotic Events and the Formation of New Synaptic Vesicles |
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15 | (1) |
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16 | (9) |
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The Junctional Folds and Their Cytoskeletal Components |
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AChRs on the Junctional Folds |
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The Saturating Disk Model of Neuromuscular Transmission |
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Regulation of the Distribution and Subunit Composition of AChR |
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The Postsynaptic Cytoskeleton |
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The Junctional Sarcoplasm |
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AChR Synthesis and Degradation |
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Signaling Mechanisms At The Neuromuscular Junction |
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25 | (12) |
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Dok-7 and Its Downstream Activators Crk, Crk-L, and Tid 1 |
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The Neuregulin/ARIA Signaling Pathway |
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2 Electrodiagnosis Of Myasthenic Disorders |
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37 | (23) |
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37 | (1) |
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Standard Motor Nerve Conduction Studies |
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38 | (1) |
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Repetitive Compound Muscle Action Potentials |
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Repetitive Nerve Stimulation Studies |
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39 | (7) |
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The Safety Margin of Neuromuscular Transmission |
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Pseudofacilitation vs. True Facilitation |
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Choice of Muscles and Stimulation Sites |
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Troubleshooting Technical Problems |
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Special Considerations in Infants and Young Children |
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What Constitutes an Adequate Repetitive Stimulation Examination? |
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46 | (1) |
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The Properties of Motor Unit Potentials |
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MUP Changes Observed in Myasthenic Disorders |
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Single-Fiber Electromyography |
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Electrodiagnostic Findings In Specific Myasthenic Disorders |
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47 | (13) |
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Autoimmune Myasthenia Gravis |
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Congenital Myasthenic Syndromes |
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3 The Immunopathogenesis Of Myasthenia Gravis |
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60 | (30) |
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60 | (1) |
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61 | (9) |
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Basic Properties of B Cells and T Cells |
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The Intrathymic Differentiation of T Cells |
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Tolerance and Autoimmunity |
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Antigen Recognition by B Cells and Antibodies |
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Antigen Recognition by T Cells |
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Cooperation of B Cells and Helper T Cells |
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Myasthenia With Thymic Hyperplasia |
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70 | (4) |
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Early Notions on the Role of the Thymus in MG |
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Intrathymic Myoid Cells and AChR |
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Key Tenets of "Intrathymic Pathogenesis" |
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Intrathymic Antigen Presentation |
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Thymic Autoantibody Production |
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Intrathymic Germinal Centers and B Cells |
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Human MG Thymus Grafted into SCID Mice |
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Two-Step Model of Intrathymic Autosensitization |
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"Promiscuous" Expression of Autoantigens by Medullary Thymic Epithelial Cells |
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Role of Regulatory T Cells (Treg) |
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Myasthenia Gravis With Thymoma |
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74 | (4) |
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Classification of Thymomas |
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Thymoma Features Associated with MG |
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Autoantibodies in Thymoma-Associated MG |
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Pathogenesis of Thymoma-Associated MG |
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Models of Tolerance Breakdown in TAMG |
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B Cell Responses In Myasthenia Gravis |
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78 | (3) |
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General Features of Anti-AChr Antibodies |
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Epitopes Recognized by Anti-AChR Antibodies |
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Anti-Striational Antibodies |
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"MuSK-positive" and "Seronegative" Myasthenia |
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T Cell Responses In Myasthenia Gravis |
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81 | (1) |
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Antigenic Epitopes Recognized by AChR-Specific T Cells |
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Cooperation Between B Cells And T Cells In Mg |
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82 | (1) |
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Pathogenic Effects Of The Anti-Achr Antibodies |
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83 | (1) |
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84 | (6) |
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4 Natural History Of Myasthenia Gravis |
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90 | (18) |
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The History And "Natural" History Of Myasthenia Gravis |
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90 | (2) |
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92 | (1) |
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92 | (1) |
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93 | (1) |
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Generalized Myasthenia Gravis |
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94 | (5) |
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Time Between Onset and Generalized Manifestations |
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Specific Clinical Situations |
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99 | (1) |
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Pregnancy Neonatal Myasthenia Gravis |
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Age-Related Issues For Myasthenia Gravis |
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100 | (1) |
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Familial Incidence Of Myasthenia Gravis |
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101 | (1) |
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Disorders Associated With Myasthenia Gravis |
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101 | (1) |
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Other Autoimmune Diseases |
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102 | (6) |
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5 The Diagnosis Of Myasthenia Gravis And Other Disorders Of Neuromuscular Transmission |
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108 | (22) |
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Introduction And Historical Background |
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108 | (1) |
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109 | (2) |
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MG Caused by Antibodies to Muscle-Specific Protein Kinase (MuSK-MG) |
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111 | (2) |
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113 | (3) |
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Differential Diagnosis of Ptosis |
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Differential Diagnosis of Diplopia |
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Differential Diagnosis of Dysarthria and Dysphagia |
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Differential Diagnosis of Isolated Respiratory Muscle Weakness |
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Differential Diagnosis of Fatigue |
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Differential Diagnosis of Mimicking Disorders |
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Diagnostic Studies For Myasthenia Gravis |
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116 | (14) |
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Electrodiagnostic Testing |
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Ocular Cooling (Ice-Pack Test) |
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Comparison of Diagnostic Techniques |
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6 Therapy Of Myasthenia Gravis |
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130 | (26) |
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130 | (1) |
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130 | (1) |
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Immune Pathogenesis of MG |
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131 | (1) |
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132 | (13) |
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Treating Mg In Special Situations |
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145 | (3) |
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Management of Associated Conditions Co-morbidities |
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Drug Interactions Pregnancy and Neonatal MG |
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Future Prospects For Treatment Of Mg |
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148 | (2) |
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The Paradox of Difficulty in Obtaining Approval of MG Treatments |
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150 | (6) |
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7 The Lambert--Eaton Myasthenic Syndrome |
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156 | (17) |
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156 | (1) |
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157 | (1) |
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Cancer-Associated Lems (Ca-Lems) |
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157 | (1) |
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Non-Cancer Associated Or Autoimmune Lems (Nca-Lems) |
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158 | (1) |
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159 | (1) |
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Pathophysiology And Immunopathology |
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159 | (2) |
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Electrodiagnostic Findings |
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161 | (4) |
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Repetitive Nerve Stimulation Testing Needle Electromyography (EMG) |
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Single-fiber Electromyography |
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Electromyographic Quantitation of severity |
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165 | (1) |
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Voltage-gated Calcium Channel Antibodies |
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166 | (1) |
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166 | (1) |
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167 | (1) |
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167 | (1) |
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Agents That May Worsen Lems |
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168 | (1) |
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169 | (1) |
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169 | (4) |
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8 Congenital Myasthenic Syndromes |
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173 | (58) |
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A Brief History Of The Congenital Myasthenic Syndromes |
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173 | (1) |
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Mechanisms Compromising The Safety Margin Of Neuromuscular Transmission |
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174 | (1) |
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The Classification Of Congenital Myasthenic Syndromes |
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174 | (1) |
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The Investigation Of Congenital Myasthenic Syndromes |
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174 | (7) |
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125I-α-bgt Binding Sites per Endplate |
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In Vitro Electrophysiology Studies |
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Molecular Genetic Studies |
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181 | (5) |
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CMS Caused by Defects in Choline Acetyltransferase (ChAT) |
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Paucity of Synaptic Vesicles and Reduced Quantal Release |
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Congenital Myasthenic Syndrome Resembling the Lambert-Eaton Syndrome |
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Synaptic Basal-Lamina-Associated Cms |
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186 | (8) |
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Endplate Acetylcholinesterase Deficiency |
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Postsynaptic Cms Caused By Mutations In Achr |
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194 | (12) |
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AChR Deficiency Caused by Recessive Mutations in AChR Subunits |
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Cms Caused By Defects In Rapsyn |
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206 | (4) |
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Defects In Mechanisms Governing Endplate Development And Maintenance |
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210 | (4) |
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CMS Caused by Defects in Agrin |
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CMS Caused by Defects in MuSK |
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CMS Caused by Defects in Dok-7 |
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CMS Caused by Defects in the Hexosamine Biosynthetic Pathway |
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Miscellaneous Rare Congenital Myasthenic Syndromes |
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214 | (17) |
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Sodium-Channel Myasthenia |
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CMS Caused by Plectin Deficiency |
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CMS Associated with Centronuclear Myopathy (CNM) |
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9 Endplate Acetylcholine Receptor, A Target For Myasthenic Disorders: Structural And Mechanistic Essentials |
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231 | (21) |
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231 | (1) |
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231 | (7) |
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AChR Structure at the Atomic Scale |
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Interface Between Ligand-binding and Pore Domains |
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Mechanism Of Achr Activation |
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238 | (5) |
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Intermediate State Between Closed and Open States |
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Links Between Agonist Binding and Channel Opening |
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243 | (5) |
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248 | (4) |
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10 Peripheral Nerve Hyperexcitability Syndromes |
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252 | (13) |
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252 | (1) |
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Clinical And Electrodiagnostic Features Of Hyperexcitability Syndromes |
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253 | (2) |
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255 | (2) |
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Hyperexcitability of the Motor Nerve Terminal |
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Hyperexcitability of Neuromuscular Junction |
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Inherited Myokymia (Potassium-Channel Mutations) |
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257 | (1) |
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257 | (3) |
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Neuromyotonia (Isaacs Syndrome) |
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Cramp-fasciculation Syndrome |
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Antibodies against Voltage-gated Potassium Channel Complex Proteins |
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260 | (1) |
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Hyperexcitability Due To Peripheral Nerve Disorders |
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261 | (1) |
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Other Peripheral Hyperexcitability Syndromes |
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261 | (1) |
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Treatment Options For Hyperexcitability Syndromes |
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262 | (1) |
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262 | (3) |
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11 Effects Of Anti-Ganglioside Antibodies At The Neuromuscular Junction |
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265 | (14) |
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265 | (2) |
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Peripehral Nerve Disorders Associated With Anti-Ganglioside Antibodies |
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267 | (2) |
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Experimental Evidence For Anti-Ganglioside Antibody-Mediated Attack Of Nmjs |
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269 | (2) |
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Clinical Evidence For Nmj Synaptopathy In Anti-Ganglioside Antibody-Mediated Human Neuropathy |
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271 | (1) |
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Comparing Clinical And Experimental Electrophysiological Findings |
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272 | (1) |
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Therapeutic Considerations |
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273 | (6) |
Index |
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279 | |