AN AUTOPSY CASE OF CREUTZFELDT‐JAKOB DISEASE ACCOMPANYING PROMINENT STATUS SPONGIOSUS

An autopsy case of Creutzfeldt‐Jakob disease is reported in a 60‐year‐old man who developed an abrupt onset of delusion, intellectural deterioration and rapidly progressing dementia terminating in decorticated rigidity. Autopsy revealed a moderate gyral atrophy of the cerebrum, particularly in the frontal lobe (1320 g). Microscopically, diffuse and prominent status spongiosue was observed throughout the cerebral cortices, mostly accompanied by atrophy and occasional loss of neuronal cells. Similar changes were seen in the basal ganglia. Lipid pigments accumulated in most of the atrophic neuronal cells. Histochemical studies were made on vacuoles of the spongious state and lipid pigments stored in the neuronal cells. Clinico‐pathological subjects of the Creutzfeldt‐Jakob disease and its subtypes, especially its relationship to subacute spongiform encephalopathy, are briefly discussed.