X-Linked Congenital Stationary Night Blindness Associated with De-Polarizing-Bipolar System Dysfunction

Electroretinography with varying flash duration was used to examine members from three generations of a family with x-linked congenital stationary night-blindness (CSNB) and normal subjects. A 10 usee test flash produced a classic 'clinical' photopic B-wave of low-normal amplitude with loss of oscillatory potentials in the CSNB subjects. When examined with 100 msec test flashes, the 'true' photopic B-wave produced by depolarizing on-center bipolar cells was severely attenuated and a prominent D-wave due to hyperpolarizing offcenter bipolar cells was present following termination of the stimulus.