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J Korean Neurol Assoc. 1996;14(2):486-493.
- Natural History of MSA-Clinical Evidence for Single Disease entity
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Jin Hwan Cho, M.D., Beom S. Jeon, M.D., Ki hyeong Lee, M.D., Sang Bok Lee, M.D.
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Department of Neurology, Seoul National University College of Medicine
- Multiple System Atrophy : 44예의 임상경과에 대한 고찰
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조진찬, 전범석, 이기형, 이상복
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서울대학교 의과대학 신경과학교실
- Abstract
- BACKGROUND
& OBJECT10NS : Multiple system atrophy(MSA) is a heterogenous system disorder affecting extrapyramidal, cerebellar and autonomic nervous system. Clinical spectrum is broad, and depending on the system affected, patients are classified into striato-nigral degeneration (SND), olivo-ponto-cerebellar atrophy (OPCA) and Shy-Draper syndrome (SDS). However, evolution of symptoms during follow-up usually occurs, stirring up a debate between "lumpers" and "splitters". Recent pathological documentation of intracytoplasmic inclusions support "lumpers" that MSA is a specific disease entity with specific pathology. The study was done to analyze the natural course of MSA, and examine whether they are separate or part of the same disease. METHOD: We obtained the clinical data of patients with clinically probable MSA by the criteria of Quinn (1994) . In addition to review of medical records, all patients were phone-interviewed or examined personally. RESULTS : Forty four patients were included in the study (male 23, female 21). Mean onset age 52.9 years, and mean follow-up period 19.7 months. Nine patients died during follow-up (mean disease duration 5.2 years). The initial predominant features were parkinsonism in 40% (14/35) , cerebellar dysfunction in 25.7% (9/35), autonomic dysfunction in 17.1% (6/35) and others in 17.1%. At the latest follow-up, parkinsonism were noted in 77.1%, cerebellar dysfunction in 88.6% and autonomic dysfunction in 80%. With progression, all the patients showed mixed clinical manifestations, the most common being combination of all 3(60%). CONCLUS10N: The data supports that SND, OPCA and SDS are part of the same disease process.
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- 초록
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