Possible pathogenesis of congenital stationary night blindness

We reported a case of congenital stationary night blindness (CSNB) who showed Schubert-Bornschein type electroretinogram (ERG) in the right eye and nearly normal ERG in the left eye with absolute glaucoma. From the electrophysiological findings of both eyes and histological findings of the enucleated eye, we concluded that the mechanism of night blindness in CSNB is an abnormal inhibition in the bipolar cell layer. The experiments in rabbits by vincristine intravenous and intravitreous administrations did not give rise to a negative type ERG, but to a decrease of the amplitude of all ERG components. The lesion of CSNB which was reported to be responsible in the synaptic terminal by Ripps et al could not be confirmed.