An early description of striatonigral degeneration.

Our aim was to revisit the papers published by Scherer 1933 describing four cases of sporadic olivopontocerebellar atrophy (OPCA) thought to represent the earliest description of striatonigral degeneration. One should note that extrapyramidal rigidity associated with OPCA was then considered a type of cerebellar parkinsonism. Two of Scherer's four patients had severe parkinsonism masking cerebellar signs. Pathologically both cases displayed marked degeneration of the striatum and nigra and partially developed pontocerebellar atrophy. Cerebellar ataxia was the outstanding feature in the other two, their pathological study showing severe pontocerebellar lesions and incipient striatonigral atrophy. Scherer stated that the severity of parkinsonism in OPCA is not correlated with the degree of cerebellar degeneration but with that of striatum and nigra. We conclude that Scherer gave the first accurate description of striatonigral degeneration. Moreover, his contribution was essential in ruling out the prevalent notion of cerebellar parkinsonism in OPCA.