Friedreich's ataxia: clinical involvement and evoked potentials

Abstract

In 15 patients definitely affected by Friedreich's ataxia, precocious SEP abnormalities were constantly recorded independent of the duration of symptoms and the severity of clinical involvement; VEPs were frequently involved, but neither VEP abnormalities, nor visual impairment could be correlated with the severity or duration of Friedreich's ataxia clinical involvement; BAEPs were, to different extents, abnormal. These were completely dissociated from hearing disorders and significantly correlated (P less than 0.001) with Friedreich's ataxia clinical disability. Our observation of increased latency of the VEP P100 component and the early disappearance of BAEP Wave V with persistence of Wave I is in contrast with the hypothesis that changes are related to primary axonal degeneration. For practical purposes SEPs could help in the early diagnosis of Friedreich's ataxia, and BAEPs could be used for the electrophysiological monitoring of its clinical progression.