Natural History of MSA-Clinical Evidence for Single Disease entity

J Korean Neurol Assoc > Volume 14(2); 1996 > Article

Journal of the Korean Neurological Association 1996;14(2): 486-493.

Multiple System Atrophy : 44예의 임상경과에 대한 고찰

조진찬, 전범석, 이기형, 이상복

서울대학교 의과대학 신경과학교실

Natural History of MSA-Clinical Evidence for Single Disease entity

Jin Hwan Cho, M.D., Beom S. Jeon, M.D., Ki hyeong Lee, M.D., Sang Bok Lee, M.D.

Department of Neurology, Seoul National University College of Medicine

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.