Michael W Nicolle1. 1. Department of Clinical Neurological Sciences, London Health Sciences Center, The University of Western Ontario, London, Ontario, Canada. mnicolle@uwo.ca
Abstract
BACKGROUND: In myasthenia gravis (MG), the prototypic autoimmune disease, antibodies against acetylcholine receptors impair neuromuscular transmission and produce weakness. Although recognized for several hundred years, it has only been over the last three decades that effective treatments have become available for MG. REVIEW SUMMARY: This review summarizes the principles of normal neuromuscular transmission, the clinical features of MG, and the tests available for its diagnosis. The current treatments for MG are discussed, including possible mechanisms of action and a discussion of potential adverse effects. When available, evidence-based justification for individual treatment options is given, and areas of controversy identified. CONCLUSIONS: Significant improvements in the diagnosis and management of MG have been made over the last several decades. The available treatments either improve neuromuscular transmission directly, or suppress or modulate the pathogenic immune response in MG. Treatment is highly individualized and must take into account the severity of disease, the presence of other diseases, and the kinetics of response for the available treatments. This requires detailed knowledge of the mechanisms of action and possible adverse effects for each treatment. However, despite an optimistic outlook with modern treatment, the management of MG continues to be plagued by lack of efficacy in some, and significant adverse effects in most MG patients.
BACKGROUND: In myasthenia gravis (MG), the prototypic autoimmune disease, antibodies against acetylcholine receptors impair neuromuscular transmission and produce weakness. Although recognized for several hundred years, it has only been over the last three decades that effective treatments have become available for MG. REVIEW SUMMARY: This review summarizes the principles of normal neuromuscular transmission, the clinical features of MG, and the tests available for its diagnosis. The current treatments for MG are discussed, including possible mechanisms of action and a discussion of potential adverse effects. When available, evidence-based justification for individual treatment options is given, and areas of controversy identified. CONCLUSIONS: Significant improvements in the diagnosis and management of MG have been made over the last several decades. The available treatments either improve neuromuscular transmission directly, or suppress or modulate the pathogenic immune response in MG. Treatment is highly individualized and must take into account the severity of disease, the presence of other diseases, and the kinetics of response for the available treatments. This requires detailed knowledge of the mechanisms of action and possible adverse effects for each treatment. However, despite an optimistic outlook with modern treatment, the management of MG continues to be plagued by lack of efficacy in some, and significant adverse effects in most MG patients.