Online edition:ISSN 2758-089X

Development of Changes in VEP and Associate Neuropathology on Creutzfeldt-Jakob Disease

A case, 74-year-old female, of Creutzfeldt-Jakob disease (CJD) was reported with a chronological changes of visual evoked potentials (VEPs) and neuropathological findings. The disease started with a disturbance of visual integration and developed to blindness, myoclonus, mental deterioration and akinetic mutism. A clinical diagnosis was made by a periodic synchronous discharge of EEG and other neurological specificity. Autopsy findings showed a peculiar spongy degeneration of the cerebral cortex. The flash VEP showed a loss of w-shaped wave and a marked delay of the peak latencies in the early stage. Subsequently, the once delayed latency was shortened with the advance of the illness in the middle stage and the N70-P100 amplitude became a huge triphasic wave like inversed ERG. Topographic distribution of the focus of huge component appeared on the left parieto-occipital region. At the terminal stage, the amplitude of huge component reduced. The mechanism of specific VEP changes in this case was interpreted to be due to the loss of generator due to spongy degeneration and the existence of cell fusion in the occipital cortex.

Tsutsui J, et al