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February 1, 1997

Survival of patients with pathologically proven multiple system atrophy
A meta-analysis

February 1997 issue
48 (2) 384-393


Article abstract-A systematic review of the neurologic literature identified 433 cases of pathologically proven multiple system atrophy over a 100-year period. Earlier case reports included patients younger in age with more frequent cerebellar involvement. Mean age of onset was 54.2 years (range 31 to 78) and survival was 6.2 years (range 0.5 to 24). Survival analysis showed a secular trend from a median duration of 4.9 years for publications between 1887 and 1970 to 6.8 years between 1991 and 1994. Older age of onset was associated with shorter survival; the hazard ratio for patients with onset after 60 years was 1.8 (95% CI 1.4 to 2.3) compared with patients between 31 and 49 years. Cerebellar features were associated with marginally increased survival (6.1 years versus 5.4 years; p = 0.04). There were no differences in survival according to gender, parkinsonian, or pyramidal features or whether the patient was classified as striatonigral degeneration or olivopontocerebellar atrophy type. These results demonstrate the poor prognosis for patients with multiple system atrophy but may be biased toward the worst cases. Future research needs to recruit more representative samples.
NEUROLOGY 1997;48: 384-393

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Published In

Volume 48Number 2February 1997
Pages: 384-393

Publication History

Published online: February 1, 1997
Published in print: February 1997


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Affiliations & Disclosures

G. K. Wenning, MD, PhD
From the Department of Social Medicine (Dr. Ben-Shlomo), Bristol University, UK; the Department of Neurology (Dr. Wenning), University Hospital Innsbruck, Austria; the Department of Neurology (Dr. Tison), Hopital Pellegrin, Bordeaux, France; and the University Department of Neurology (Dr. Quinn), Institute of Neurology, London, UK.
Supported by the UK Parkinson's Disease Society (G.K.W.) and by a grant from Produits Roche, France (F.T.).
Presented in part at the 46th Annual Meeting of the American Academy of Neurology, Washington, DC, May 1994.
Received April 26, 1996. Accepted in final form July 16, 1996.
Address correspondence and reprint requests to Dr. Y. Ben-Shlomo, Senior Lecturer, Department of Social Medicine, University of Bristol, Canynge Hall, Whiteladies Road, Bristol BS8 2PR, UK.

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