Fig. 3
From: Multimodal imaging in a pedigree of X-linked Retinoschisis with a novel RS1 variant
ISCEV full-field electroretinogram [45] responses from affected grandsons (CC3 and CC4). Horizontal axis: 50Ā msec per division; Vertical axis: Dark Adapted Responses 500Ā Ī¼V per division; 30Ā Hz Flicker Responses 250Ā Ī¼V per division. The rod-isolated responses of CC3 and CC4 were slightly, but significantly reduced in amplitudes. The mixed rod and cone responses to the maximal intensity flash stimulus presented to the dark-adapted eye showed a normal a-wave amplitude in each subject, (CC3 445Ā Ī¼V, CC4 441Ā Ī¼V). The b-wave amplitudes were significantly reduced in each patient (CC3 515Ā Ī¼V, CC4 502Ā Ī¼V) and approximated the a-wave amplitudes. Ordinarily, the b-wave amplitude greatly exceeds that of the a-wave. The normal a-wave and selective reduction of the b-wave indicates preserved photo-transduction but impaired post-receptoral retinal function. This has been reported in RS1-associated X-linked retinoschisis [44]