Figure 5. Color fundus composite photographs of affected individuals from families 1 and 2 and Goldmann visual fields from the proband in family 2. Panel A shows a fundus composite of the right eye from 34-year-old male (IV:6) from family 1; his visual acuity (VA) was 0.1 LogMAR with an electroretinogram (ERG) that was consistent with a “mild cone–rod dystrophy.” The image shows evidence of vessel attenuation and early peripheral retinal atrophy with few bone spicules. Panel B shows a fundus composite of the right eye in 36-year-old female (IV:5) from family 1 whose VA was 0.3 LogMAR, and who had absent color vision; her ERG was consistent with a diagnosis of retinitis pigmentosa. The fundus image shows macular depigmentation, dense peripheral bone spicules, and vessel attenuation. Panel C displays the color fundus composite from the left eye of 42-year-old male (IV:3) from family 1. His VA was 1.3 LogMAR, and the image displays circular patches of RPE atrophy both at the macula and in the periphery with associated peripheral pigment migration. His ERG was described as consisted with “advanced RP.” Panel D shows a color fundus composite from the right eye of 44-year-old female (IV:2) from family 1; Her visual acuity was limited to Counting Fingers (CF) and the view of the posterior pole is obscured by dense asteroid hyalosis as seen; there is peripheral bone spicule pigmentation and circular pigment epithelial atrophy was observed in the far periphery. Panel E shows the left eye color fundus composite of 46-year-old male (II:1) from family 2. His VA was recorded as Hand Movements (HM) at his most recent examination. No color vision was ever detected; the fundus image shows an atrophic retina, with vessel attenuation, RPE atrophy around the disc, at the macula and in the periphery, and associated peripheral pigment migration. His ERG when tested at age 32 years was undetectable. Panel F displays the Goldmann visual fields for patient II:1 with the V4e isopter in green and III4e in red: there are peripheral islands of residual vision and no response centrally to the largest and brightest target (V4e) in the right eye and small areas of response in the left eye.