The negative ERG: clinical phenotypes and disease mechanisms of inner retinal dysfunction

Inner retinal dysfunction is encountered in a number of retinal disorders, either inherited or acquired, as a primary or predominant defect. Fundus examination is rarely diagnostic in these disorders, although some show characteristic features, and careful electrophysiological assessment of retinal function is needed for accurate diagnosis. The ERG in inner retinal dysfunction typically shows a negative waveform with a preserved a-wave and a selectively reduced b-wave. Advances in retinal physiology and molecular genetics have led to a greater understanding of the pathogenesis of these disorders. This review summarizes current knowledge on normal retinal physiology, the investigative techniques used and the range of clinical disorders in which there is predominantly inner retinal dysfunction.