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Multiple system atrophy: a review of 203 pathologically proven cases
G K Wenning1, F Tison, Y Ben Shlomo
1University Department of Clinical Neurology, London, England.
Summary
Multiple System Atrophy (MSA) typically affects men in their early fifties, presenting with autonomic failure and parkinsonism. Pathological hallmarks include cell loss in specific brain regions and characteristic cytoplasmic inclusions.
Area of Science:
- Neurology
- Pathology
Background:
- Multiple System Atrophy (MSA) is a rare neurodegenerative disorder.
- Understanding its clinicopathological features is crucial for diagnosis and management.
Purpose of the Study:
- To summarize the clinicopathological features of 203 pathologically proven Multiple System Atrophy cases.
- To correlate clinical symptoms with pathological findings.
Main Methods:
- Systematic review of 108 publications up to February 1995.
- Analysis of 203 pathologically confirmed Multiple System Atrophy cases.
- Correlation of clinical manifestations with neuropathological changes.
Main Results:
- The majority of patients presented in their early fifties, with a male predominance (1.3:1).
- Autonomic failure (74%) and parkinsonism (87%) were the most common clinical features.
- Pathological findings included cell loss and gliosis in the putamen, substantia nigra, and cerebellar regions, with characteristic cytoplasmic inclusions.
Conclusions:
- Multiple System Atrophy exhibits diverse clinicopathological features.
- Specific pathological changes correlate with distinct clinical symptoms, such as akinesia with nigral/putaminal cell loss and ataxia with olivopontocerebellar atrophy.
- The presence of glial and/or neuronal cytoplasmic inclusions is a key diagnostic marker.