Transmissible virus dementia. II. Neurohistology of three, geographically clustered cases of Creutzfeldt-Jakob disease.

Histopathological findings in three temporo-spatially linked cases of Creutzfeldt-Jakob disease (CJD) are reported. The patients were males, unrelated and without positive family history. Their mean age at the onset of the disease was 52.3 years. The mean duration of the illness was 6.6 months; the clinical symptomatology differed considerably. Neurohistology revealed in all patients a neuronal loss, intracyoplasmic vacuolation, spongiosis and astrocytosis. Besides the cortical regions, basal ganglia and cerebella were severely affected. All cases were pathologically suggestive of the cortico-striato-cerebella variant of CJD. Attention is drawn to this apparent topical similarity of most pronounced lesions. The possible significance of the pathoanatomical consistency, observed in the cases studied, for the unusual pattern of natural occurrence of CJD is considered.