Shy-Drager syndrome: the transitional variant.

The clinicopathological features of a patient with the transitional variant of the Shy-Drager syndrome is described. The only previously reported case of the transitional variant was reexamined and pathological similarities to the present case are reviewed. Both patients exhibited features of Parkinson's disease with Lewy bodies in the substantia nigra and locus ceruleus. Striato-nigral degeneration and olivo-ponto-cerebellar atrophy were evident in both cases. They can thus be considered as transitional forms of the Shy-Drager syndrome with feature of both Parkinson's disease and multiple system atrophy.