Visual involvement in Friedreich's ataxia: PERG and VEP study

Neuro-ophthalmological assessment, including red-free light retinography, in conjunction with simultaneous visual evoked potential (VEP) and pattern electroretinogram (PERG) recordings were performed in 10 Friedreich's ataxia patients: 9 patients showed marked VEP abnormalities. Moderate PERG amplitude reduction, with normal latencies, was related to mild and scattered fiber loss revealed by red-free light retinography. The initial part of the visual pathways does not seem to be the main site of electrophysiological abnormalities as demonstrated by the greater extent and relative independence of VEP versus PERG alterations. Primary axonal degeneration of the optic nerve and tracts cannot account for all features of VEP abnormalities, thus implying some dysfunction in succeeding visual structures as well.