Twenty years ago, the discovery of a deficit of acetylcholinereceptors at the neuromuscular junctions of patients with myastheniagravis1 and the development of an animal model of the disease2shed new light on a disorder that was first described clinically300 years earlier by the great physiologist Thomas Willis3.During the past two decades, remarkable progress has been madein our understanding of myasthenia gravis, and the new knowledgehas been applied directly to the clinical diagnosis and treatmentof this formerly grave disease. Myasthenia gravis is undoubtedlythe most thoroughly understood of all human autoimmune diseasesand . . . [Full Text of this Article]
Clinical Features
The Neuromuscular Junction in Myasthenia Gravis
The Acetylcholine Receptor
Immunopathogenesis of Myasthenia Gravis
Antibody-Mediated Mechanisms
Accelerated Degradation of Acetylcholine Receptors
Blockade of Acetylcholine Receptors
Damage to Neuromuscular Junctions
Functional Properties of Anti-Acetylcholine-Receptor Antibodies and the Severity of Disease
Properties of Acetylcholine-Receptor Antibodies
"Antibody-Negative" Myasthenia Gravis
Role of Lymphocytes in Myasthenia Gravis
Origin of the Autoimmune Response in Myasthenia Gravis
Diagnosis
Diagnostic Testing
Differential Diagnosis
Search for Associated Conditions
Treatment
Anticholinesterase Agents
Thymectomy
Immunosuppressive Treatment
Corticosteroids
Azathioprine
Cyclosporine
Short-Term Immunotherapies: Plasma Exchange and Intravenous Immune Globulin
Specific Immunotherapy: The Future of Treatment
B-Cell-Directed Approaches
T-Cell-Directed Approaches
Conclusions
Source Information
From the Department of Neurology, Johns Hopkins University School of Medicine, 600 N. Wolfe St., Baltimore, MD 21287-7519, where reprint requests should be addressed to Dr. Drachman.
References
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