한국 소아중증근무력증 환아 55례의 임상적 고찰 및 재발요인 분석에 대한 연구

A Clinical Study on Characteristics and Relapsing Factors of Juvenile Myasthenia Gravis in Korea

Juvenile myasthenia gravis(JMG) is quite well known to be caused by the autoimmune mechanism. In addition, there are some opinions that MG is different from Caucasians and Oriental races. 55 cases of Juvenile MG under the age at onset of 16 years who were admitted to the Department of Pediatrics of Yonsei Medical School during 18 years from Jan.1976 to Dec. 1993 were analysed clinically. The results were summarized as follows : The male to female ratio was 1:1.4. In 39(71%) cases of patients. The age at onset was between 2 and 6 years(mean 4.9 years) and 20(38%) cases were seen in spring time. All patients were initially treated with anticholinesterase but 46(84%) cases also required immunosuppressive therapies. Complete remission occurred in 4 patients(6%). Phamarcologic remission in 22(40%). Marked improvement in 11(20%). Mild improvement in 9(17%), No change or Worsening in 9(17%). The mean interval between intial therapy and symptomatic improvement was 12.1 days. 26 cases were relapsd and the mean interval between the pharmacologic remission and onset of symptoms was 10.3 months. The major factors to relapse were reducing dosages and infections. All of these findings indicate that Juvenile myasthenia gravis has some differences between Caucasians and Koreans and special attentions are needed for the proper management.