Electrophysiology in neuro-ophthalmology

This chapter reviews common applications of visual electrophysiology relevant to neuro-ophthalmology practice. The use of standard tests and extended protocols are described including the cortical visual evoked potential and pattern and full-field electroretinogram (PERG; ERG) methods, the latter including the photopic negative response. Abnormalities of these recordings are rarely specific but provide valuable diagnostic guidance and an objective measure of visual pathway function, difficult or impossible to infer by other methods. The electrophysiological phenotypes associated with Leber hereditary optic neuropathy, OPA1- and SSBP1-associated dominant optic atrophy, and WFS1-related syndromes are described. Typical changes in retinal and optic nerve function tests associated with acquired disease are highlighted, including those related to demyelination, ischemic, compressive, nutritional and toxic, and nonorganic etiologies. The importance of complementary testing using different electrophysiological techniques is emphasized, for the purposes of differential diagnosis and in disorders that may masquerade as optic nerve pathology.