Cerebellar changes in 50 cases of Creutzfeldt-Jakob disease with emphasis on granule cell atrophy variant.

Cerebellar changes have been found in 41/50 cases of Creutzfeldt-Jakob disease. They were severe in 9 cases. We did not find significant correlation between the cerebellar symptoms and signs pointed out in clinical records and prominent cerebellar changes. The only exception consisted in dentate nucleus involvement which was more frequently related to those symptoms and signs. 5 of the cases with severe changes were characterized by predominant granule cell atrophy without kuru plaques. The mean age of death (56.0) was significantly lower in this variant than that of patients with other cerebellar changes (64.4) (p less than 0.01). The granule cell atrophy seems a distinct variant on the basis of age of death and pathological changes. However, it is not characterized by the presence of kuru plaques.