Evoked potentials in patients with Huntington's disease and their offspring. II. Visual evoked potentials.
Visual evoked potentials (VEPs) to full-field and half-field stimulation were recorded in a large sample of patients with Huntington's disease (n = 36) and subjects at risk (first-order offspring, n = 55) using a checkerboard pattern reversal stimulus. Whereas a clear diminution of the amplitudes found confirmed earlier findings in patients with Huntington's disease, further alterations of VEP wave forms consisted in the absence of asymmetric hemisphere lateralization to half-field stimulation (i) and severe distortion with amplification of the early NPN/PNP complexes (ii). Considering at least two different abnormal parameters necessary to represent a reliable indicator of the disease, 24 patients (67%) and 14 (25%) of the persons at risk exhibited pathological results. In contrast, only 2 among 36 patients (5%) but 30 (55%) among the offspring exhibited normal results. The diagnostic and predictive value of the investigation of VEPs in Huntington's disease is discussed.
Hennerici
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Hömberg
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Lange
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HW
D000293:Adolescent; D000328:Adult; D000368:Aged; D005074:Evoked Potentials, Visual; D005260:Female; D007839:Functional Laterality; D006801:Humans; D006816:Huntington Disease / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010775:Photic Stimulation; D011930:Reaction Time; D014794:Visual Fields