Computed tomography of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD), one of the transmissible spongiform encephalopathy, affects gray matter primarily. Degeneration of the white matter is uncommon and when it occurs is usually mild, and limited in distribution. Mizutani separated the "panencephalopathic type" of CJD from the other type, because this panencephalopathic type has extensive white matter changes, that could not be explained simply as secondary to cortical degeneration. This communication discusses six necropsy cases of CJD. In four cases of panencephalopathic type, out of our cases, there were extensive changes of cerebral white matter in addition to severe loss of neurons and hypertrophic astrogliosis in cortex and basal ganglia. Computed tomography demonstrated diffuse low attenuation areas in the white matter with marked atrophy of the cortex, brainstem and cerebellum.