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Am J Ophthalmol. 1990 Jan 15;109(1):44-8.

Autosomal dominant congenital stationary night blindness and normal fundus with an electronegative electroretinogram.

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  • 1Department of Ophthalmology, New York University Medical Center, NY 10016.


We studied three members of three successive generations of a family with autosomal dominant congenital stationary night blindness and normal fundi. Psychophysical studies on two members showed normal final cone thresholds and mildly increased rod thresholds. Full-field electroretinograms on all three members showed normal photopic b-wave amplitudes and implicit times. Under scotopic conditions, the rod response was absent, and with a bright flash stimulus, there was a normal a-wave with no b-wave. This electronegative dark-adapted electroretinogram resembled the Schubert-Bornschein type seen in congenital stationary blindness, which has been seen only in autosomal and X-linked recessive pedigrees.

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