Paradoxical pupillary responses in congenital stationary night blindness

Three unrelated boys, ages 2, 6, and 10 years, who have congenital stationary night blindness with myopia and a Schubert-Bornschein-type electroretinogram finding, were found to show a "paradoxical" pupillary constriction in darkness. When examining room lights are turned out, the patient's pupils briskly constrict and slowly dilate. Older night blind male relatives of these boys did not show this abnormal constriction to darkness.