Table 2. Clinical data are listed for probands from 13 families.
Family Number | Sex | Age (years) at | Visual Acuity | Fundus Features | ||
---|---|---|---|---|---|---|
Exam | Onset | R eye | L eye | |||
RP98 | M | 51 | 25 | 0.2 | HM | Bilateral retinal vascular attenuation, bone spicule like pigmentation throughout the fundus, chorioretinal degeneration, pale optic disc |
RP154 | M | 38 | 28 | 0.5 | 0.4 | Bilateral attenuation of retinal arterioles, bone spicule like pigmentation in the mid-periphery retina, RPE degeneration, pale optic disc |
RP152 | M | 36 | 24 | 0.4 | 0.3 | Bilateral attenuation of retinal arterioles, bone spicule like pigmentation in the mid-periphery retina, RPE degeneration, pale optic disc |
RP173 | M | 23 | 17 | 0.8 | 0.8 | Bilateral attenuation of retinal arterioles, bone spicule like pigmentation in the periphery retina |
RP137 | M | 43 | 12 | 0.4 | 0.2 | Bilateral attenuation of retinal arterioles, bone spicule like pigmentation in the mid-periphery retina, RPE degeneration, pale optic disc |
RP128 | F | 23 | 3 | 0.8 | 0.8 | Bilateral attenuation of retinal arterioles, widespread RPE degeneration, macular epimembrane |
RP214 | F | 32 | Childhood | 0.3 | 0.4 | Bilateral attenuation of retinal arterioles, widespread RPE degeneration, the degenerated RPE area confused in the macular. |
RP88 | M | 48 | Childhood | FC | FC | Bilateral retinal vascular attenuation, bone spicule like pigmentation throughout the fundus, chorioretinal degeneration, pale optic disc |
RP135 | M | 36 | 5 | 0.3 | 0.2 | Bilateral attenuation of retinal arterioles, bone spicule like pigmentation in the mid-periphery retina, RPE degeneration, pale optic disc |
RP352 | F | 42 | Childhood | 0.05 | 0.05 | Bilateral retinal vascular attenuation, bone spicule like pigmentation in the mid-periphery retina, RPE degeneration, pale optic disc |
RP89 | M | 45 | Childhood | LP | LP | Bilateral retinal vascular attenuation, bone spicule like pigmentation throughout the fundus, chorioretinal degeneration, pale optic disc |
RP71 | F | 37 | 18 | 0.6 | 0.5 | Bilateral attenuation of retinal arterioles, bone spicule like pigmentation in the mid-periphery retina |
RP110 | M | 65 | 25 | 0.1 | 0.2 | Bilateral retinal vascular attenuation, bone spicule like pigmentation throughout the fundus, RPE degeneration, pale optic disc |
FC, finger counting; HM, hand movement; LP, light perception; F, female; M, male.