Visual evoked potentials in ataxia telangiectasia

Pattern reversal visual evoked potentials (VEPs) elicited in four patients with ataxia telangiectasia revealed normal results in two and absent responses in two. The pathogenesis of the VEP abnormalities is discussed. It is surmised that the VEP changes reflect progressive degeneration of the nerve fibres in the anterior visual pathway, as in Friedreich's ataxia.