| MuSK 항체 양성을 보이는 혈청음성 중증근무력증 환자의 임상적 특징 |
| 이지영, 성정준a 오동훈a 민주홍a 김현정b 박종하b 최영철c 김성훈d A. Vincente 이광우a |
| 서울대학교 의과대학 신경과학교실, 서울대학교병원 신경과a, 서울대학교병원 임상의학연구소b,
영동세브란스병원 신경과, 연세대학교 의과대학 신경과학교실c, 강원대학교 의과대학 신경과학교실d,
Honorary Consultant Immunologist, Neurosciences Group, Institute o |
| Clinical Features of MuSK Antibody Positive Seronegative Myasthenia Gravis |
| Jee-Young Lee |
| Department of Neurology, Seoul National University Hospital, College of Medicine, Seoul National Universitya, Seoul; Clinical Research Institute, Seoul National University Hospitalb Seoull; Department of Neurology, Young dong Severance Hospital, College of Medicine, Yonsei Universityc, Seoul; Department of Neurology, College of Medicine, Kangwon National Universityd, Seoul, Korea; Honorary Consultant Immunologist, Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospitale, United Kingdom |
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| Abstract |
Background: A variable proportion of seronegative myasthenia gravis (SNMG) patients have antibodies to the muscle-specific tyrosine kinase (MuSK). Although several reports from Western countries suggest differences in the clinical features of MuSK antibody-positive and -negative SNMG patients, there have been no reports about these patients in Korea.
Methods: We performed the first survey of MuSK antibodies in Korea, measuring MuSK antibodies by commercial preparations (RSR Ltd) in the serum of SNMG patients who registered at the Seoul National University Hospital from October 2003 to January 2004, and identified clinical features and treatment responses prospectively until October 2004 using double blind method.
Results: Twenty-three (15 generalized and eight ocular MG, 15 men and eight women) SNMG patients with the ages from 1 to 60 years, (mean 36.24±16.82 years), were included. None of 8 ocular SNMG had MuSK antibody, whereas MuSK antibody was present in four (26.7%) of 15 generalized SNMG. All four MuSK positive patients were females, with pharyngeal and respiratory muscle weakness, and required immunosuppressive treatment in addition to acetylcholine esterase inhibitors. However, the overall disease severity and the age of onset did not show significant differences between MuSK antibody-positive and -negative SNMG patients and the responses to treatment were equally favorable.
Conclusions: Our study showed the lower rate of MuSK antibodies in SNMG than the previous reports. However it seems to require large multicenter survey to confirm the possibilities of geographical or ethnical differences in the future. KeyWords:Seronegative myasthenia gravis, MuSK antibodies |
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