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Congenital Stationary Night Blindness Presenting as Leber's Congenital Amaurosis
Richard G. Weleber, MD;
Andrea Cibis Tongue, MD
Arch Ophthalmol. 1987;105(3):360-365.
Abstract
Two siblings with autosomal-recessive congenital stationary night blindness were clinically blind in infancy. Both had markedly abnormal electroretinograms that, in the first child, led consultants at two university centers to make the diagnosis of Leber's congenital amaurosis. The patients had intermittent nystagmus and esotropia, but good photopic vision developed eventually. Scotopic vision was clearly defective in each child. Refractive error in both patients was close to emetropic in early infancy but became myopic by 1 year of age. Congenital stationary night blindness must be considered in the differential diagnosis of the blind infant.
Author Affiliations
From the Departments of Ophthalmology (Drs Weleber and Tongue) and Medical Genetics (Dr Weleber), Oregon Health Sciences University, Portland.
Footnotes
Accepted for publication Sept 4, 1986.
Reprint requests to Department of Ophthalmology, Oregon Health Sciences University, 3181 SW Sam Jackson Park Rd, Portland, OR 97201 (Dr Weleber).
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