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Multiple system atrophy: a review of 203 pathologically proven cases.
Mov Disord. 1997 Mar; 12(2):133-47.MD

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 later. 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.

Authors+Show Affiliations

University Department of Clinical Neurology, London, England.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Meta-Analysis
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

9087971

Citation

Wenning, G K., et al. "Multiple System Atrophy: a Review of 203 Pathologically Proven Cases." Movement Disorders : Official Journal of the Movement Disorder Society, vol. 12, no. 2, 1997, pp. 133-47.
Wenning GK, Tison F, Ben Shlomo Y, et al. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord. 1997;12(2):133-47.
Wenning, G. K., Tison, F., Ben Shlomo, Y., Daniel, S. E., & Quinn, N. P. (1997). Multiple system atrophy: a review of 203 pathologically proven cases. Movement Disorders : Official Journal of the Movement Disorder Society, 12(2), 133-47.
Wenning GK, et al. Multiple System Atrophy: a Review of 203 Pathologically Proven Cases. Mov Disord. 1997;12(2):133-47. PubMed PMID: 9087971.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Multiple system atrophy: a review of 203 pathologically proven cases. AU - Wenning,G K, AU - Tison,F, AU - Ben Shlomo,Y, AU - Daniel,S E, AU - Quinn,N P, PY - 1997/3/1/pubmed PY - 1997/3/1/medline PY - 1997/3/1/entrez SP - 133 EP - 47 JF - Movement disorders : official journal of the Movement Disorder Society JO - Mov Disord VL - 12 IS - 2 N2 - 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 later. 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. SN - 0885-3185 UR - https://www.unboundmedicine.com/medline/citation/9087971/Multiple_system_atrophy:_a_review_of_203_pathologically_proven_cases_ DB - PRIME DP - Unbound Medicine ER -