Neuropathological Studies of Creutzfeldt-Jakob Disease. Two Cases, One with and One without Prominent Senile Changes
Neuropathological findings of two cases of Creutzfeldt- Jakob disease were described. The cases were a 67-year-old woman and a 74-year-old woman, both of whom showed visual disturbance, mental confusion, rigidity of the extremities, myoclonic jerks, and periodic synchronous discharges on electroencephalograms. The former died after five months of the illness and the latter after three months. Neuropathologically, marked nerve cell loss, protoplasmic astrocytosis and stromal spongiosis were observed in the cerebral cortex of both cases, especially in the occipital and temporal lobes. There were mild to moderate similar changes also in the basal ganglia, thalamus and cerebellum. In the first case, many typical senile plaques, neurofibrillary changes and Hirano bodies were found in the hippocampus, and primitive senile plaques in the cerebral cortex. Electron microscopically, spongiosis in the stroma of both cases was observed as accumulations of vacuoles of various sizes, usually with single or double limiting membranes in the neuropil. Although both cases might belong to the subacute spongiform encephalopathy type of Creutzfeldt-Jakob disease, the coexistence of prominent senile changes in the first case suggested a relation between this case and Alzheimer's disease.