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 th at extrapyramidal rigidity associated with OPCA was then considered a type of cerebellar parkinsonism. Two of Scherer's four patients had severe parki nsonism masking cerebellar signs. Pathologically both cases displayed marke d degeneration of the striatum and nigra and partially developed pontocereb ellar atrophy. Cerebellar ataxia was the outstanding feature in the other t wo, their pathological study showing severe pontocerebellar lesions and inc ipient striatonigral atrophy. Scherer stated that the severity of parkinson ism in OPCA is not correlated with the degree of cerebellar degeneration bu t with that of striatum and nigra. We conclude that Scherer gave the first accurate description of striatonigral degeneration. Moreover, his contribut ion was essential in ruling out die prevalent notion of cerebellar parkinso nism in OPCA.