Volume 12, Issue 2 p. 133-147
Article

Multiple system atrophy: A review of 203 pathologically proven cases

Dr. G. K. Wenning

Corresponding Author

Dr. G. K. Wenning

University Department of Clinical Neurology, Institute of Neurology, London

Universitätsklinik für Neurologie, Innsbruck, Austria

Universitütsklinik für Neurologie, Anichstrasse 35, 6020 Innsbruck, AustriaSearch for more papers by this author
F. Tison

F. Tison

University Department of Clinical Neurology, Institute of Neurology, London

Search for more papers by this author
Y. ben Shlomo

Y. ben Shlomo

Department of Epidemiology and Public Health, University College Medical School, London, England

Search for more papers by this author
S. E. Daniel

S. E. Daniel

Parkinson's Disease Society Brain Bank, Institute of Neurology, London

Search for more papers by this author
N. P. Quinn

N. P. Quinn

University Department of Clinical Neurology, Institute of Neurology, London

Search for more papers by this author
First published: 04 November 2004
Citations: 606

Abstract

We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the latter. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.