[Visual evoked potentials in Creutzfeldt-Jakob disease]

Neuropathological studies show frequent and extensive effects on the visual system in Creutzfeldt-Jakob disease (CJD), but deterioration of vision is not reported by all patients. We examined the function of the visual system by means of visual evoked potentials (VEP). We recorded monocular pattern-reversal VEP in six patients with sporadic CJD 1-13 months after first symptoms occurred. Three patients had normal vision, and in a further three, vision was impaired. All patients had pathological VEP with a delayed P100 component (six eyes) or loss of cortical response (five eyes). The patients with visual impairment vs those without were not different concerning VEP findings. The VEP are already pathological in initial CJD stages and point to an early effect on the visual system in CJD, irrespective of clinical visual deficits.