Electrophysiological and pathological studies on Creutzfeldt-Jakob disease with retinal involvement

In a case of Creutzfeldt-Jakob disease in a 59-year-old female, multifocal degeneration from the cerebral cortex through the visual pathway to the retina was detected clinically, electrophysiologically, and pathologically. Visual evoked cortical potentials (VECPs) showed a peculiar huge negative wave in the early stage but the amplitude reduced gradually. The a- and b-waves of the ERG were detectable in the final stage. Dynamic topography of VECPs revealed a delay of excitation in the visual cortex in the early stage, but a complete defect of the cortical potential and diminished reactivity of the brain stem were apparent in the later stage. Pathological findings were the spongy degeneration of the cerebral cortex, demyelination of the white matter and the optic pathway, and the degeneration of the nerve fiber layer and ganglion cell layer of the retina.