Creutzfeldt-Jakob disease. An autopsy case of the panencephalopathic type and a review of the literature.

This is a case report of a purported panencephalopathic type of Creutzfeldt-Jakob (C-J) disease in a 61-year-old Japanese farmer. He died nine months after the onset of clinical symptoms. This variety of C-J disease was named and reported in 1981 by Mizutani. The characteristic feature is extensive degeneration of cerebral white matter not related to cortical damage in addition to the spongiform changes in the cortical gray matter. The case was diagnosed clinically and at post mortem marked neural loss, astrocytic macrogliosis, and degeneration of subcortical white matter were found. Senile plaques and neurofibrillary tangles were virtually absent. Degeneration of the white matter is uncommon in C-J disease, and when it occurs is usually mild, and limited in distribution.