CREUTZFELDT-JAKOB-DISEASE - NEUROPHYSIOLOGIC VISUAL IMPAIRMENTS

Objective: The predictive value of electrophysiologic visual testing i n Creutzfeldt-Jakob disease (CJD) was investigated, and the retinal pa thologic findings in three cases are reported. Background: The fatal p rognosis of CJD, its transmissibility, and the lack of treatment make early diagnosis essential in averting human-to-human transmission. Ele ctroretinogram and visual evoked potentials have been studied in few c ases of CJD. Methods: A visual electrophysiologic examination was perf ormed in 41 consecutive patients referred with suspected CJD. The dise ase had been diagnosed in 24 patients (CJD group; 15 were confirmed ne uropathologically and 9 by clinicolaboratory methods in accordance wit h diagnostic criteria). The remaining 17 patients were diagnosed with other neurologic disorders, and served as a control group. Results: Fl ash electroretinogram revealed a significant decrease in the amplitude of the B1 wave (<60 mu V) and the B/A ratio (<2) in the CJD group com pared with those in the control group. Flash visual evoked potentials revealed no significant difference in latency, but amplitude was incre ased (>10 mu V) in the CJD group, especially in patients with myoclonu s. Conclusions: The visual electrophysiologic abnormalities provide an interesting noninvasive diagnostic tool in idiopathic CJD. The B1-wav e decrease is closely correlated with the outer plexiform layer abnorm alities observed on neuropathologic examination.