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Creutzfeldt-Jakob disease

Neurophysiologic visual impairments

From the Departments of Neuro-ophthalmology (Drs. de Seze, Hache, and Arndt), Neurology (Drs. de Seze, Vermersch, Pasquier, Leys, Destée, and Petit), and Neuropathology (Drs. Maurage and Ruchoux), Hôpital R. Salengro, Lille; and the Department of Biochemistry (Dr. Laplanche), Hôpital Lariboisière, Paris, France.

Address correspondence and reprint requests to Professor J.C. Hache, Department of Neuro-ophthalmology, Hôpital R. Salengro, CHRU de Lille, 59037 Lille Cedex, France.

Objective: The predictive value of electrophysiologic visual testing in Creutzfeldt-Jakob disease (CJD) was investigated, and the retinal pathologic findings in three cases are reported.

Background: The fatal prognosis of CJD, its transmissibility, and the lack of treatment make early diagnosis essential in averting human-to-human transmission. Electroretinogram and visual evoked potentials have been studied in few cases of CJD.

Methods: A visual electrophysiologic examination was performed in 41 consecutive patients referred with suspected CJD. The disease had been diagnosed in 24 patients (CJD group; 15 were confirmed neuropathologically and 9 by clinicolaboratory methods in accordance with diagnostic criteria). The remaining 17 patients were diagnosed with other neurologic disorders, and served as a control group.

Results: Flash electroretinogram revealed a significant decrease in the amplitude of the B1 wave (<60 µV) and the B/A ratio (<2) in the CJD group compared with those in the control group. Flash visual evoked potentials revealed no significant difference in latency, but amplitude was increased (>10 µV) in the CJD group, especially in patients with myoclonus.

Conclusions: The visual electrophysiologic abnormalities provide an interesting noninvasive diagnostic tool in idiopathic CJD. The B1-wave decrease is closely correlated with the outer plexiform layer abnormalities observed on neuropathologic examination.

Received October 20, 1997. Accepted in final form June 18, 1998.

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