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KMID : 0391520050130020210
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Journal of the Korean Child Neurology Society
2005 Volume.13 No. 2 p.210 ~ p.219
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Acetylcholine Receptor Antibody and Clinical Features in Childhood Onset Myasthenia Gravis
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Lee Kye-Hyang
Kim Su-Jin
Lee Jee-Hun
Lee Mun-Hyang
Kim Hyun-Sook
Kim Jhin-Gook
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Abstract
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PURPOSE: We studied 32 children with myasthenia gravis to evaluate clinical features and outcomes. Also, we tried to compare between seropositive and seronegative juvenile myasthenia gravis, and validate the clinical correlation between acetylcholine receptor antibody titers and clinical severity in childhood myasthenia gravis.
METHODS: The childhood myasthenia gravis patients were diagnosed and treated in Samsung Medical Center from Oct. 1994 to Aug. 2004.
RESULTS: The overall clinical features, responses to treatment and outcomes were nearly same as those of other previous reports in Korea as well as the other countries. The mean age at onset was 5.3+/-3.4 years, and the ratio of male to female was 1:1.3. Ocular types were 78.1%, and generalized types were 21.9%. There were significantly lower mean acetylcholine receptor antibody titers in the ocular groups. There were no significant differences in clinical features and outcomes between seropositive and seronegative groups. However, there was a significant correlation between clinical severity and acetylcholine receptor antibody titers. Steroid add-on treatment was required in 78.6% of the patients. Thymectomy was done in 4 patients, all of whom were in partial remission. Overall, the complete remission rate was 37.5%.
CONCLUSION: There were no significant differences in clinical features between seropositve & seronegative groups. However, there was a significant correlation between clinical severity and acetylcholine receptor antibody titers.
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KEYWORD
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Myasthenia Gravis, Children, Acetylcholine Receptor
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