Natural History of MSA-Clinical Evidence for Single Disease entity
Journal of the Korean Neurological Association
;
: 486-493, 1996.
Article
in Korean
| WPRIM
| ID: wpr-203665
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Autonomic Nervous System
/
Olivopontocerebellar Atrophies
/
Cerebellar Diseases
/
Medical Records
/
Follow-Up Studies
/
Age of Onset
/
Natural History
/
Parkinsonian Disorders
Type of study:
Observational study
/
Prognostic study
Limits:
Female
/
Humans
Language:
Korean
Journal:
Journal of the Korean Neurological Association
Year:
1996
Type:
Article
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