Abstract

Congenital stationary night blindness (CNSB) is a clinically and genetic diverse group of nonprogressive retinal disorders that is characterized by stable poor vision in low light (night blindness). CNSB can be classified either functionally by electrophysiology or fundus phenotypes. ERG can distinguish a “complete” form or CNSB type 1 with absent rod function, from an “incomplete” form or CNSB type 2 with reduced rod and cone function. Phenotypically, CNSB can have a normal appearing fundus or an abnormal fundus (includes Oguchi’s disease and fundus albipunctatus).

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