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Myasthenia Gravis and Myasthenic Disorders

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ISBN-10: 019973867X

ISBN-13: 9780199738670

Edition: 2nd 2012

Authors: Andrew G. Engel

List price: $245.00
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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…    
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Book details

List price: $245.00
Edition: 2nd
Copyright year: 2012
Publisher: Oxford University Press, Incorporated
Publication date: 4/3/2012
Binding: Hardcover
Pages: 304
Size: 10.00" wide x 7.10" long x 0.80" tall
Weight: 2.046
Language: English

Andy Engel grew up near Belvidere, NJ, in a house that his father nearly always had under construction. Woodworking and carpentry were just what you did. When other teenagers were dancing to The Bee Gees and Donna Summer, Andy was in the shop building a dulcimer inspired by a chapter in The Foxfire Book. For a few years after high school, he alternated between college, dead-end jobs, and travel. In 1982, he found his feet and hired on as a woodworker in a pre-hung door shop. From 1985 through 1996, he was self-employed as a carpenter and home builder. In 1996, Taunton's Fine Homebuilding magazine offered him an assistant editor's position. Andy left Taunton in 2005 to chase a living as…    

The Anatomy and Molecular Architecture of the Neuromuscular Junction
Basic Concepts
The Innervation of Muscle
The Presynaptic Region
The Nerve Terminal
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
Steps in Exocytosis
Other Proteins Modulating Exocytosis
Presynaptic Cytoskeletal Components
The Active Zone and die Voltage-Gated Ca<sup>2+</sup> Channels
Voltage-gated K<sup>+</sup> Channels of die Presynaptic Membrane
Endocytotic Events and die Formation of New Synaptic Vesicles
The Synaptic Space
Synaptic Basal Lamina
Asymmetric AChE
Collagen XIII
Synaptic Laminins
The Postsynaptic Region
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 Junction
Agrin, MuSK, and LRP4
Dok-7 and Its Downstream Activators Crk, Crk-L, and Tidl
The Neuregulin/ARIA Signaling Pathway
Electrodiagnosis of Myasthenic Disorders
Standard Motor Nerve Conduction Studies
Repetitive Compound Muscle Action Potentials
Repetitive Nerve Stimulation Studies
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 Electromyography
The Properties of Motor Unit Potentials
MUP Changes Observed in Myasthenic Disorders
Single-Fiber Electromyography
Electrodiagnostic Findings in Specific Myasthenic Disorders
Autoimmune Myasthenia Gravis
Lambert-Eaton Syndrome
Congenital Myasthenic Syndromes
The Immunopathogenesis of Myasthenia Gravis
Immunological Principles
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 Hyperplasia
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 Thymoma
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 Gravis
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 Gravis
AChR-Specific T Cells
Antigenic Epitopes Recognized by AChR-Specific T Cells
Cooperation Between B Cells and T Cells In MG
Pathogenic Effects of the Anti-Achr Antibodies
Natural History of Myasthenia Gravis
The History and "Natural" History of Myasthenia Gravis
Ocular Myasthenia
Generalized Myasthenia Gravis
Time Between Onset and Generalized Manifestations
Signs and Symptoms
Specific Clinical Situations
MuSK-Myasdienia Gravis
Neonatal Myasthenia Gravis
Age-Related Issues for Myasthenia Gravis
Familial Incidence of Myasthenia Gravis
Disorders Associated with Myasthenia Gravis
Thyroid Disorders
Odier Autoimmune Diseases
The Diagnosis of Myasthenia Gravis and Other
Disorders of Neuromuscular Transmission
Introduction and Historical Background
Clinical Presentation
Myasthenia Gravis
MG Caused by Antibodies to Muscle-Specific Protein Kinase (MuSK-MG)
Physical Findings
Myasthenia Gravis
Differential Diagnosis
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 Gravis
Edrophonium Test
Antibody Tests
Electrodiagnostic Testing
Ocular Cooling (Ice-Pack Test)
Comparison of Diagnostic Techniques
Other Diagnostic Tests
6. Therapy of Myasthenia Gravis
Pathogensis of MG
Immune Pathogenesis of MG
Origin of MG
Treatment of MG
Treatment Strategies
Treating MG in Special Situations
Ocular Myasthenia
Management of Associated Conditions Co-morbidities
Drug Interactions
Pregnancy and Neonatal MG
Myasdienic Crisis
Future Prospects for Treatment of MG
The Paradox of Difficulty in Obtaining Approval of MG Treatments
The Lambert-Eaton Myasthenic Syndrome
Clinical Features
Physical findings
Cancer-Associated Lems (Ca-Lems)
Non-Cancer Associated or Autoimmune Lems (Nca-Lems)
Pathophysiology and Immunopathology
Electrodiagnostic Findings
Repetitive Nerve Stimulation Testing Needle Electromyography (EMG)
Single-fiber Electromyography
Electromyograpbic Quantitation of severity
Serological Testing
Voltage-gated Calcium Channel Antibodies
SOX1 Antibodies
Other Tests
Differential Diagnosis
MG/LEMS Overlap
Symptomatic Treatment
Agents That May Worsen Lems
Congenital Myasthenic Syndromes
A Brief History of the Congenital Myasthenic Syndromes
Mechanisms Compromising the Safety Margin of Neuromuscular Transmission
The Classification of Congenital Myasthenic Syndromes
The Investigation of Congenital Myasthenic Syndromes
Clinical Observations
Morphology <sup>125</sup>I-�-bgt Binding Sites per Endplate
In Vitro Electrophysiology Studies
Molecular Genetic Studies
Presynaptic CMS
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 CMS
Endplate Acetylcholinesterase Deficiency
Defects in �2-Laminin
Postsynaptic CMS Caused by Mutations in Achr
Slow-Channel Syndromes
Fast-Channel Syndromes
AChR Deficiency Caused by Recessive
Mutations in AChR Subunits
Escobar Syndrome
CMS Caused by Defects in Rapsyn
Defects in Mechanisms Governing Endplate Development and Maintenance
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 Syndromes
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 Essentials
AChR Structure
AChR Structure at die Atomic Scale
Pore Domain
Ligand-binding Domain
Interface Between Ligand-binding and Pore Domains
Mechanism of AChR Activation
Intermediate State Between Closed and Open States
Links Between Agonist Binding and Channel Opening
AChR Disease Mechanisms
Slow-channel CMS
Fast-channel CMS
10. Peripheral Nerve Hyperexcitability Syndromes
Clinical and Electrodiagnostic Features of Hyperexcitability Syndromes
Hyperexcitability of die Motor Nerve Terminal
Hyperexcitability of Neuromuscular Junction
Inherited Myokymia (Potassium-Channel Mutations)
Autoimmune PNH
Neuromyotonia (Isaacs Syndrome)
Morvan Syndrome
Antibodies against Voltage-gated Potassium Channel Complex Proteins
Other Antibodies
PNH Secondary to Toxins
Hyperexcitability due to Peripheral Nerve Disorders
Other Peripheral Hyperexcitability Syndromes
Treatment Options for Hyperexcitability Syndromes
Effects of Anti-Ganglioside Antibodies at the Neuromuscular Junction
Neuronal Gangliosides
Structure and Synthesis
Physiological Functions
Peripehral Nerve Disorders Associated With Anti-Ganglioside Antibodies
Experimental Evidence for Anti-Ganglioside Antibody-Mediated Attack of NMJs
Clinical Evidence for Nmj Synaptopathy in Anti-Ganglioside Antibody-Mediated Human Neuropathy
Comparing Clinical and Experimental Electrophysiological Findings
Therapeutic Considerations