Visual evoked potentials in spinocerebellar degenerations

Pattern reversal visual evoked potentials were studied in 21 patients with spinocerebellar ataxias among whom 6 had Friedreich's ataxia, 10 had hereditary spastic ataxia and 5 had spinocerebellar degeneration with slow eye movements (olivopontocerebellar degeneration). The VEP abnormalities found in 4 cases of Friedreich's ataxia and one with spinocerebellar degeneration with slow eye movements, consisted of, bilaterally absent VEP in 3 patients and bilaterally abnormal responses with asymmetry in two. All the patients with spastic ataxia had normal VEP latencies. The N 70 - P 100 amplitudes, in patients with hereditary ataxias were significantly reduced compared to controls (P less than 0.001). The VEP abnormalities correlated best with neuroophthalmic findings, but had no relation to age, sex, inheritance or duration of illness. The VEP findings are probably suggestive of progressive nerve fibre loss in the visual pathways with associated slowing of conduction. The higher incidence of visual pathway involvement in Friedreich's ataxia compared to other hereditary ataxias as reported in recent studies is confirmed.