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Pathogenic Antibodies in Myasthenia Gravis Masakatsu Motomura 1 1First Department of Clinical Neuroscience and Neurology,Graduate School of Biomedical Sciences,Nagasaki University Keyword: myasthenia gravis , acetylcholine receptor antibodies , muscle-specific tyrosine kinase antibodies , double seronegative , neuromuscular junction pp.411-418
Published Date 2010/4/1
DOI https://doi.org/10.11477/mf.1416100669
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Abstract

 Myasthenia gravis (MG) is the most common autoimmune disorder of the neuromuscular junction and is clinically characterized by weakness and muscle fatigue. We have classified MG into 3 types on the basis of the antibody pattern. The first type associated with acetylcholine receptor (AChR) autoantibodies,which predominantly belong to IgG1 subclass and are measured by a conventional radioimmunoprecipitation assay with 125I-alpha-bungarotoxin. This subtype occurs in approximately 80% of patients with MG and leads to the loss of AChR number and function,mainly by complement-mediated destruction of the neuromuscular junction. Approximately 40% of patients with MG who have AChR antibodes have a thymoma as a paraneoplastic neurological syndrome. However,the role of antigen expression by thymomas is unclear. The second type of MG occurs in a proportion of "seronegative" patients who did not have AChR autoantibodies. These patients process IgG autoantibodies to muscle-specific tyrosine kinase (MuSK); these antibodies are predominantly of the IgG4 subclass but are not associated with complement-mediated damage to the neuromuscular junction or with the presence of thymomas. In most patients with MuSK antibodies,the symptoms of MG improve after plasma exchange; these patients show a good response to steroid and immunosuppressive drugs but a poor response to thymectomy. MG not associated with the presence of the 2 abovementioned pathogenic autoantibodies is classified as heterogeneous "double seronegative" MG. Our classification is superior to the present classifications with regard to the mechanism,treatment,and prognosis of the disease.


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電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

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