Figure 1.

Visual function in representative patients with CRB1-RD. (A) Kinetic perimetry results using two targets (V-4e, I-4e) illustrate preserved central and temporal peripheral islands of vision. (B) Dark-adapted (top) and light-adapted (bottom) static threshold perimetry results displayed as grayscale maps of rod and cone sensitivity loss. The scale has 16 levels of gray, representing 0- to 30-dB losses. N, nasal; T, temporal; I, inferior; S, superior visual field. (C) Dark-adapted, two-color (500 and 650 nm) sensitivity profiles across the horizontal meridian (central 60°) in the patients (symbols connected by lines) compared with normal for rod-mediated sensitivity to the 500-nm stimulus (shaded band) and for cone-mediated sensitivity to the 650 nm stimulus at the cone plateau (dashed lines). The photoreceptor mediation at loci with function, based on the sensitivity difference between the two colors, is given: C, cone-mediated.