Relationship between evoked potential and neuropsychological findings in persons "at risk" for Huntington's disease.

Scalp-recorded evoked potentials (EPs) elicited by left and right median nerve stimulation (LSEP and RSEP), checkerboard pattern flash (VEP), and acoustic click (AEP) were obtained in 40 individuals “at risk” (AR) for Huntington's disease (HD) and 40 nonpatients matched for age, sex, and intelligence. EPs of AR subjects were generally similar to those of nonpatient's overall configuration; however, mean amplitudes were significantly lower for ARs. The lower amplitudes tended to occur primarily among a subgroup of ARs who manifested some degree of cognitive change, as defined by neuropsychological test criteria.