Figure 3. Transient pupillary light reflex (TPLR) abnormalities in LCA5 patients. A: Change in pupil diameter is plotted as a function of time in response to short duration (0.1 s) light stimuli of two different intensities (0.4 and 1.4 log scot-cd.s.m⁻²; denoted at right end of traces) in the LCA5 patients (filled symbols). A response elicited with the brighter stimulus in a 9-year-old normal subject is shown for comparison (unfilled symbols). Stimulus monitor is shown at lower left of each of the two panels. B: TPLR response thresholds to a 0.3 mm criterion response in the LCA5 patients (filled symbols) are compared to a group of 11 LCA patients, age 1–58 years, with severely impaired vision (unfilled symbols). TPLR response amplitudes are measured at a fixed time of 0.9s (vertical dashed line in A). Thresholds in the LCA5 patients and other LCA patients show elevations in excess of 5 log units from normal. Gray hexagon denotes normal mean±2SD.