[Multiple system atrophy (MSA)].
The clinical and pathological features of 47 cases of multiple system atrophy (MSA) were analyzed. The mean age of onset was 56.7 (range 40 to approximately 76) years and mean survival was 5.7 (range 1.5 to approximately 12) years. Parkinsonism occurred in 31 cases. Helpful early points to the diagnosis included rigid-akinetic type, rapid progression, failure to respond to L-DOPA and other signs such as autoromic dysfunction, cerebellar ataxia and pyramidal signs, but these were not always present. T2 weighted MR images demonstrated decreased signal in the putamen and/or slit-hyperintensity in the outer margin of the putamen. These clinical and MRI findings are useful in the differential diagnosis between Parkinson's disease and MSA. Glial cytoplasmic inclusions (GCIs) were found in all 21 cases, but not in control brains. Several types of neuroral inclusion were also observed; large neuroral cytoplasmic, small neuroral cytoplasmic and nuclear inclusions. GCIs were labeled by antiubiquitin, anti-alpha and beta-tubulin and antitau antibodies. Large neuronal cytoplasmic inclusions (NCIs) observed in the pontine nuclei were positive with only antiubiquitin antibody.
Riku
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Department of Neurology, Social Insurance Chukyo Hospital.
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009847:Olivary Nucleus / Q000473:pathology; D010300:Parkinson Disease / Q000473:pathology*; D011149:Pons / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology