NR ADCK
AU de Seze,J.; Hache,J.C.; Vermersch,P.; Arndt,C.F.; Maurage,C.A.; Pasquier,F.; Laplanche,J.L.; Ruchoux,M.M.; Leys,D.; Destee,A.; Petit,H.
TI Creutzfeldt-Jakob disease: neurophysiologic visual impairments.
QU Neurology 1998 Oct; 51(4): 962-7
PT journal article
AB 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 microV) 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 microV) 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.
MH Adult; *Creutzfeldt-Jakob Syndrome/complications/diagnosis/physiopathology; Electroretinography; *Evoked Potentials, Visual; Female; Human; Male; Middle Age; Predictive Value of Tests; Retina/pathology/physiopathology; Sensitivity and Specificity; Vision, Low/diagnosis/*etiology/pathology
AD Department of Neuro-ophthalmology, Hopital R. Salengro, Lille, France.
SP englisch
PO USA