Abstract

A patient with congenital nightblindness and white spots in the fundus was evaluated in 1985 and again in 1990. This patient appeared to represent an intermediate state between fundus albipunctatus, a congenital stationary nightblindness with the special features of white spots in the fundus and prolonged dark adaptation of several hours leading to normal absolute thresholds(1,2,3), and retinitis punctata albescens, a form of retinitis pigmentosa characterized by white spots in the retina and by the functional features of retinitis pigmentosa (ring scotomas, diminished ERGs, progressive decrease of function, etc)(5). The patient was fascinating particularly in terms of 1) her dark adaptation abnormalities and the critical importance of prior recent light exposure in interpreting her visual function, 2) her evidence of cone involvement, and 3) her ERG abnormalities. The visual function tests in this patient provide diagnostic information, are critical in following her course, and provide fascinating insight into the mechanism of her disease.

© 1992 Optical Society of America