PMID: 9426875Apr 1, 1997Paper

Parkinsonism. Multiple system atrophy

Baillière's Clinical Neurology
G K Wenning, N P Quinn

Abstract

Over the last decade multiple system atrophy (MSA) has been confirmed as a distinct clinicopathological entity. For a long time, overlapping pathology in individual cases with striatonigral degeneration (SND), sporadic olivopontocerebellar atrophy (OPCA) or Shy-Drager syndrome (SDS) had often been a source of confusion. The recently discovered glial and neuronal inclusions indeed confirm that these three disorders represent manifestations of the same disease. Parkinsonism is the most frequent motor disorder of MSA. Early diagnosis of these patients is difficult but important, particularly in clinical trials of potential therapies. Patients with only parkinsonism (+/- autonomic failure) account for much of this diagnostic difficulty, particularly early on. Some features that have been associated with SND such as symmetry or absence of tremor are not helpful, and insistence on a poor levodopa response will miss a sizeable minority of patients. A number of further clinical 'red flags' may be helpful. MRI, MRS, PET and SPECT scanning, autonomic function tests and, especially, external sphincter EMG, may also help differentiate between idiopathic Parkinson's disease (IPD) and MSA. Available medical treatments are usually disappointi...Continue Reading

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