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9780199738670

Myasthenia Gravis and Myasthenic Disorders

by
  • ISBN13:

    9780199738670

  • ISBN10:

    019973867X

  • Edition: 2nd
  • Format: Hardcover
  • Copyright: 2012-04-03
  • Publisher: Oxford University Press

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Summary

Myasthenia Gravis and Myasthenic Disorders,Second Editionis a thoroughly re-written and updated version of the highly successful first edition published in 1999. The current edition begins with an overview of the anatomy and molecular architecture of the neuromuscular junction and the electrophysiologic diagnosis of its disorders. The introductory chapters are followed by a detailed exposition of the pathogenesis, natural history, diagnosis and therapy of the autoimmune myasthenias, the Lambert-Eaton myasthenic syndrome, and the increasingly complex and fascinating diseases collectively referred to as congenital myasthenic syndromes. The acetylcholine receptor is a major target of both autoimmune and inherited myasthenias and a separate chapter reviews its structural and functional properties. The book also describes disorders that often target terminal nerve segment near the neuromuscular junction-- the syndrome of peripheral nerve hyperexcitability and the Guillain-Barré syndrome. Comprehensively written by leaders at the forefront of research, not to mention thoroughly referenced throughout and gorgeously illustrated, this new edition of the classic 1999 text will cement its place asthetext on Myasthenia Gravis and related disorders for years to come.

Author Biography


Dr. Andrew Engel is the McKnight-3M Professor of Neuroscience and Professor of Neurology, Mayo Clinic College of Medicine as well as the Director of the Neuromuscular Disease laboratory at the Mayo Clinic, Rochester, MN campus. He earned his medical degree at McGill University, was trained in Neurology at the NIH and the Mayo Clinic and in Neuropathology at Columbia University and has spent his entire professional carrier at the Mayo Clinic. Dr Engel has had a longstanding interest in neuromuscular diseases and especially those affecting neuromuscular transmission.

Table of Contents

Contributorsp. xv
The Anatomy and Molecular Architecture of the Neuromuscular Junctionp. 1
Basic Conceptsp. 1
The Innervation of Musclep. 3
The Presynaptic Regionp. 3
The Nerve Terminalp. 4
Giant Synaptic Vesicles
Coated Vesicles
Dense-core Vesicles
Small Clear Synaptic Vesicles
Vesicular ACh Uptake
Synaptic Vesicle Pools
Synaptic Vesicles Move to and Dock at die Active Zones
Exocytotic Machinery
SNARE Complex
Priming of Docked Synaptic Vesicles
Synaptotagmin-1
Steps in Exocytosis
Other Proteins Modulating Exocytosis
Presynaptic Cytoskeletal Components
The Active Zone and die Voltage-Gated Ca2+ Channels
Voltage-gated K+ Channels of die Presynaptic Membrane
Endocytotic Events and die Formation of New Synaptic Vesicles
The Synaptic Spacep. 15
Synaptic Basal Lamina
Asymmetric AChE
Collagen XIII
Synaptic Laminins
The Postsynaptic Regionp. 16
The Junctional Folds and Their Cytoskeletal Components
AChRs on the Junctional Folds
The Saturating Disk Model of Neuromuscular Transmission
Regulation of die Distribution and Subunit Composition of AChR
The Postsynaptic Cytoskeleton
The Junctional Sarcoplasm
AChR Syndiesis and Degradation
Signaling Mechanisms at the Neuromuscular Junctionp. 25
Agrin, MuSK, and LRP4
Dok-7 and Its Downstream Activators Crk, Crk-L, and Tidl
The Neuregulin/ARIA Signaling Pathway
Electrodiagnosis of Myasthenic Disordersp. 37
Introductionp. 37
Standard Motor Nerve Conduction Studiesp. 38
Repetitive Compound Muscle Action Potentials
Repetitive Nerve Stimulation Studiesp. 39
The Safety Margin of Neuromuscular Transmission
Pseudofacilitation vs. True Facilitation
Drug Effects
Choice of Muscles and Stimulation Sites
Troubleshooting Technical Problems
Special Considerations in Infants and Young Children
What Constitutes an Adequate Repetitive Stimulation Examination?
Needle Electromyographyp. 46
The Properties of Motor Unit Potentials
MUP Changes Observed in Myasthenic Disorders
Single-Fiber Electromyography
Electrodiagnostic Findings in Specific Myasthenic Disordersp. 47
Autoimmune Myasthenia Gravis
Lambert-Eaton Syndrome
Botulism
Congenital Myasthenic Syndromes
The Immunopathogenesis of Myasthenia Gravisp. 60
Introductionp. 60
Immunological Principlesp. 61
Basic Properties of B Cells and T Cells
The Intrathymic Differentiation of T Cells
Tolerance and Autoimmunity
Antigen Recognition by B Cells and Antibodies
Antigen Recognition by T Cells
Cooperation of B Cells and Helper T Cells
Myasthenia with Thymic Hyperplasiap. 70
Early Notions on the Role of the Thymus in MG
Intrathymic Myoid Cells and AChR
Key Tenets of "Intrathymic Pathogenesis"
Intrathymic Antigen Presentation
Thymic Autoantibody Production
Intrathymic Germinal Centers and B Cells
Human MG Thymus Grafted into SCID Mice
Two-Step Model of Intrathymic Autosensitization
"Promiscuous" Expression of Autoantigens by Medullary Thymic Epithelial Cells
Role of Regulatory T Cells (Treg)
Possible -Role of Viruses
Myasthenia Gravis with Thymomap. 74
Epidemiology
Classification of Thymomas
Thymoma Features Associated with MG
Autoantibodies in Thymoma-Associated MG
Pathogenesis of Thymoma-Associated MG
Models of Tolerance Breakdown in TAMG
B Cell Responses In Myasthenia Gravisp. 78
General Features of Anti-AChR Antibodies
Epitopes Recognized by Anti-AChR Antibodies
Anti-Striational Antibodies
"MuSK-positive" and "Seronegative" Myasthenia
T Cell Responses in Myasthenia Gravisp. 81
AChR-Specific T Cells
Antigenic Epitopes Recognized by AChR-Specific T Cells
Cooperation Between B Cells and T Cells In MGp. 82
Pathogenic Effects of the Anti-Achr Antibodiesp. 83
Summaryp. 84
Natural History of Myasthenia Gravisp. 90
The History and "Natural" History of Myasthenia Gravisp. 90
Epidemiologyp. 92
Onsetp. 92
Ocular Myastheniap. 93
Generalized Myasthenia Gravisp. 94
Time Between Onset and Generalized Manifestations
Signs and Symptoms
Exacerbation
Remission
Course
Mortality
Specific Clinical Situationsp. 99
MuSK-Myasdienia Gravis
Pregnancy
Neonatal Myasthenia Gravis
Age-Related Issues for Myasthenia Gravisp. 100
Familial Incidence of Myasthenia Gravisp. 101
Disorders Associated with Myasthenia Gravisp. 101
Thymoma
Thyroid Disorders
Odier Autoimmune Diseases
Summaryp. 102
The Diagnosis of Myasthenia Gravis and Other
Disorders of Neuromuscular Transmissionp. 108
Introduction and Historical Backgroundp. 108
Clinical Presentationp. 109
Myasthenia Gravis
MG Caused by Antibodies to Muscle-Specific Protein Kinase (MuSK-MG)
Physical Findingsp. 111
Myasthenia Gravis
MuSK-MG
Differential Diagnosisp. 113
Differential Diagnosis of Ptosis
Differential Diagnosis of Diplopia
Differential Diagnosis of Dysarthria and Dysphagia
Differential Diagnosis of Isolated Respiratory Muscle Weakness
Differential Diagnosis of Fatigue
Differential Diagnosis of Mimicking Disorders
Diagnostic Studies for Myasthenia Gravisp. 116
Edrophonium Test
Antibody Tests
Electrodiagnostic Testing
Ocular Cooling (Ice-Pack Test)
Comparison of Diagnostic Techniques
Other Diagnostic Tests
6. Therapy of Myasthenia Gravisp. 130
Introductionp. 130
Pathogensis of MGp. 130
Immune Pathogenesis of MG
Origin of MGp. 131
Treatment of MGp. 132
Prehminaries
Treatment Strategies
Treating MG in Special Situationsp. 145
Ocular Myasthenia
Management of Associated Conditions Co-morbidities
Drug Interactions
Pregnancy and Neonatal MG
Myasdienic Crisis
Future Prospects for Treatment of MGp. 148
The Paradox of Difficulty in Obtaining Approval of MG Treatments
Conclusionsp. 150
The Lambert-Eaton Myasthenic Syndromep. 156
Historyp. 156
Clinical Featuresp. 157
Symptoms
Physical findings
Cancer-Associated Lems (Ca-Lems)p. 157
Non-Cancer Associated or Autoimmune Lems (Nca-Lems)p. 158
Epidemiologyp. 159
Pathophysiology and Immunopathologyp. 159
Electrodiagnostic Findingsp. 161
Repetitive Nerve Stimulation Testing Needle Electromyography (EMG)
Single-fiber Electromyography
Electromyograpbic Quantitation of severity
Serological Testingp. 165
Voltage-gated Calcium Channel Antibodies
SOX1 Antibodies
Other Testsp. 166
Differential Diagnosisp. 166
MG/LEMS Overlapp. 167
Treatmentp. 167
Cancer
Symptomatic Treatment
Immunotherapy
Agents That May Worsen Lemsp. 168
Prognosisp. 169
Summaryp. 169
Congenital Myasthenic Syndromesp. 173
A Brief History of the Congenital Myasthenic Syndromesp. 173
Mechanisms Compromising the Safety Margin of Neuromuscular Transmissionp. 174
The Classification of Congenital Myasthenic Syndromesp. 174
The Investigation of Congenital Myasthenic Syndromesp. 174
Clinical Observations
Morphology 125I-¿-bgt Binding Sites per Endplate
In Vitro Electrophysiology Studies
Molecular Genetic Studies
Presynaptic CMSp. 181
CMS Caused by Defects in Choline Acetyltransferase (ChAT)
Paucity of Synaptic Vesicles and Reduced Quantal Release
Congenital Myasdienic Syndrome Resembling the Lambert-Eaton Syndrome
Synaptic Basal-Lamina-Associated CMSp. 186
Endplate Acetylcholinesterase Deficiency
Defects in ß2-Laminin
Postsynaptic CMS Caused by Mutations in Achrp. 194
Slow-Channel Syndromes
Fast-Channel Syndromes
AChR Deficiency Caused by Recessive
Mutations in AChR Subunits
Escobar Syndrome
CMS Caused by Defects in Rapsynp. 206
Defects in Mechanisms Governing Endplate Development and Maintenancep. 210
CMS Caused by Defects in Agrin
CMS Caused by Defects in MuSK
CMS Caused by Defects in Dok-7
CMS Caused by Defects in die Hexosamine Biosynthetic Pathway
Miscellaneous Rare Congenital Myasthenic Syndromesp. 214
Sodium-Channel Myasdienia
CMS Caused by Plectin Deficiency
CMS Associated widi Centronuclear Myopatliy (CNM)
Endplate Acetylcholine Receptor, A Target for Myasthenic Disorders: Structural and Mechanistic Essentialsp. 231
Introductionp. 231
AChR Structurep. 231
AChR Structure at die Atomic Scale
Pore Domain
Ligand-binding Domain
Interface Between Ligand-binding and Pore Domains
Mechanism of AChR Activationp. 238
Intermediate State Between Closed and Open States
Links Between Agonist Binding and Channel Opening
AChR Disease Mechanismsp. 243
Slow-channel CMS
Fast-channel CMS
Perspectivep. 248
10. Peripheral Nerve Hyperexcitability Syndromesp. 252
Backgroundp. 252
Clinical and Electrodiagnostic Features of Hyperexcitability Syndromesp. 253
Pathophysiologyp. 255
Hyperexcitability of die Motor Nerve Terminal
Hyperexcitability of Neuromuscular Junction
Inherited Myokymia (Potassium-Channel Mutations)p. 257
Autoimmune PNHp. 257
Neuromyotonia (Isaacs Syndrome)
Morvan Syndrome
Cramp-fasciculation
Syndrome
Antibodies against Voltage-gated Potassium Channel Complex Proteins
Other Antibodies
PNH Secondary to Toxinsp. 260
Hyperexcitability due to Peripheral Nerve Disordersp. 261
Other Peripheral Hyperexcitability Syndromesp. 261
Treatment Options for Hyperexcitability Syndromesp. 262
Summaryp. 262
Effects of Anti-Ganglioside Antibodies at the Neuromuscular Junctionp. 265
Neuronal Gangliosidesp. 265
Structure and Synthesis
Localization
Physiological Functions
Peripehral Nerve Disorders Associated With Anti-Ganglioside Antibodiesp. 267
Experimental Evidence for Anti-Ganglioside Antibody-Mediated Attack of NMJsp. 269
Clinical Evidence for Nmj Synaptopathy in Anti-Ganglioside Antibody-Mediated Human Neuropathyp. 271
Comparing Clinical and Experimental Electrophysiological Findingsp. 272
Therapeutic Considerationsp. 273
Indexp. 279
Table of Contents provided by Ingram. All Rights Reserved.

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