Congenital stationary night blindness is an inherited retinal disorder that predominates on rods. It is a rare disease present at birth. Three types of transmission can be found: autosomal dominant, recessive, and X-linked recessive. The affection is heterogenous. The only symptom is hemeralopia with a moderate loss of visual acuity. Both the funduscopy and visual field are normal. In recessive forms, the b wave on the electroretinogram is not found in the scotoscopic study, while the a wave is normal and increases with light intensity. In dominant forms, the b wave is seen. Levels of rhodopsine are normal and regenerate normally. Signal transmission may be the affected function. There is no specific treatment.
Last update: July 2005