Figure 3
ISCEV standard full-field flash electroretinogram (ERG). (Top traces) Right eye (RE) of proband (III.1) at age 29. Scotopic ERG: complete absence of rod specific response to a dim white flash (0.01 cd.s/m2); residual response to slightly higher intensity red flash (0,3 cd.s/m2); responses to high intensity flashes (3.0 cd.s/m2 and 10.0 cd.s/m2) show significant reduction of amplitudes and major delay in peak times. Photopic ERG: response to a transient, high-intensity flash (3.0 cd.s/m2) also shows significant reduction in amplitudes and a delay of responses. This is confirmed by response to a 30 Hz high intensity (3.0 cd.s/m2) flicker stimulus. (Middle traces) RE of the mother of the proband (II.1) at age 50. Scotopic rod specific ERG: reduction of amplitude of rod specific response to a dim white flash (0.01 cd.s/m2) to about two thirds of normal; response to slightly higher intensity red flash (0.3 cd.s/m2) shows conserved response of cone-specific part (b1), with similar decrease to two thirds of normal amplitude of rod-specific response (b2); responses to high-intensity flashes (3.0 cd.s/m2 and 10.0 cd.s/m2) show similar reduction to two thirds of normal for both a- and b-waves with only minor delay of responses. Photopic ERG: response to a transient high-intensity flash (3.0 cd.s/m2) shows mild delay of b-wave (right column top trace) and mild delay to a 30 Hz high intensity (3.0 cd.s/m2) flicker stimulus (right column bottom trace). (Bottom traces) Normal reference traces of a healthy control subject.
