CLINICOPATHOLOGICAL STUDY OF 35 CASES OF MULTIPLE SYSTEM ATROPHY

The clinical and pathological features of 35 cases with multiple syste m atrophy collected in the United Kingdom Parkinson's Disease Society Brain Bank (UKPDSBB) between 1985 and 1992 have been analysed. The med ian age of onset was 55 (range 33.3-75.8) years and median survival wa s 7.3 (range 2.1-11.5) years. Parkinsonism, usually asymmetric, occurr ed in all, and autonomic failure in all but one case. Cerebellar signs were noted in 34% and pyramidal features in 54% of the cases. Glial c ytoplasmic inclusions were found in all cases with adequate fixation. Lewy bodies were detected in three cases. The substantia nigra was (us ually severely) depleted of cells in all cases, With two exceptions th e putamen was atrophic; the caudate and pallidum were less commonly an d less severely affected.Overall nigrostriatal cell loss correlated wi th severity of disease at the time of death. The latest, but not the b est, recorded levodopa response tended to be inversely related to the degree of putaminal degeneration. The olivopontocerebellar system was involved in 88% of the cases, the cerebellar vermis usually being more severely affected than the hemispheres. The presence of associated ce rebellar pathology was, however, unrelated to the presence of cerebell ar signs in life.