Paradoxic pupillary constriction in a patient with congenital stationary night blindness

Paradoxic pupillary constriction to an "off-step" of light was studied in a 35-year-old woman with congenital stationary night blindness. ERG was of the Schubert-Bornschein type (scotopic B-wave absent); fundi were normal for a high myope. Paradoxic constrictions were larger in response to full field and peripheral off-steps from low photopic levels than from higher photopic levels. The patient's "on response" was smaller in magnitude and slower in both latency and dynamics than the on-response of normal subjects. Pupillary hippus was larger in magnitude and more peaked in the patient than in normal subjects. Steady-state (tonic) pupil size increased paradoxically with increased light level over the range 1-2 log fL.