Childhood Myasthenia Gravis: Clinical Features and Response to Steroid Treatment.
- Author:
Byung Chan LIM
1
;
Yoon Jong KANG
;
Hee HWANG
;
Jong Hee CHAE
;
Ki Joong KIM
;
Yong Seung HWANG
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. pednr@plaza.snu.ac.kr
- Publication Type:Original Article
- Keywords:
Myasthenia gravis;
Childhood;
Steroid treatment
- MeSH:
Acetylcholine;
Age of Onset;
Alopecia;
Antibodies;
Continental Population Groups;
Disease Progression;
Follow-Up Studies;
Generalization (Psychology);
Humans;
Male;
Moyamoya Disease;
Myasthenia Gravis*;
Recurrence
- From:
Journal of the Korean Child Neurology Society
2003;11(1):120-127
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: Myasthenia gravis(MG) is relatively rare in childhood and shows some distinct epidemiologic and clinical features according to ages and races. The role of immunosuppressive therapy such as steroid treatment is not well established. The objective of this study is to characterize clinical features of childhood MG and to evaluate the outcomes of steroid treatment. METHODS: We studied 23 MG patients(7 male and 16 females) with symptom onsets from 1 to 14 years of age(mean 4.1 years). RESULTS: Fifteen patients were ocular type and 6 patients were generalized type. Sero-positivity to acetylcholine receptor antibodies was found in 85% of the patients. Two patients who showed initial ocular manifestations experienced secondary generalization without steroid treatment. Alopecia totalis and moyamoya disease were associated with systemic MG in two cases. Eighteen patients were treated with both anticholine esterase and steroid while 2 patients were treated with steroid only. Complete remission was observed in 6 patients(30%) while 11 patients(55%) had one or more recurrences and only partial responses were acquired in 4 patients(20%). CONCLUSION: We found some different epidemiologic features from western countries including a relatively high proportion of ocular MG and earlier onset age especially in ocular MG. Further long-term follow up study on responses to steroid treatment will be necessary to evaluate clinical benefits in the disease progression.
