Natural History of MSA-Clinical Evidence for Single Disease entity.
- Author:
Jin Hwan CHO
1
;
Beom S JEON
;
Ki hyeong LEE
;
Sang Bok LEE
Author Information
1. Department of Neurology, Seoul National University College of Medicine.
- Publication Type:Original Article
- MeSH:
Age of Onset;
Autonomic Nervous System;
Cerebellar Diseases;
Female;
Follow-Up Studies;
Humans;
Medical Records;
Natural History*;
Olivopontocerebellar Atrophies;
Parkinsonian Disorders;
Pathology
- From:Journal of the Korean Neurological Association
1996;14(2):486-493
- CountryRepublic of Korea
- Language:Korean
-
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.
