Characteristic ERG flicker anomaly in incomplete congenital stationary night blindness

Ten patients with the incomplete type of congenital stationary night blindness (CSNB) were examined with a 30 Hz flicker electroretinogram (ERG). After 30 min of dark adaptation, 30 Hz flicker ERG was recorded continuously for 12-15 min under white background illumination. All patients showed an exaggerated increase of amplitude and a universal characteristic change of wave shape as the light adaptation progressed. Thirty normal subjects also showed increased amplitude during light adaptation, but the increase in amplitude was significantly less than in incomplete-type CSNB, and there was little change in wave shape. The same procedure was applied to patients with complete-CSNB, retinitis pigmentosa, congenital retinoschisis, cone dystrophy, and Oguchi's disease; neither the exaggerated increase of amplitude nor the wave change was seen. Our results indicate that incomplete-type CSNB is a newly identified cone-rod dysfunction syndrome with a special functional property.