Molecular Vision



Appendix 3: Summary of clinical dataPatientSexDxOnset (y)Initial symptom(s)Age,First / last examVAOD first; lastOS first; lastKinetic visual fields (V4e)cFull field ERGdMEE1aMMD30Nyctalopia (30), vision (32)38 / --20/100 ; --CF ; --Not performedRods: Near-normalCones: Near-normal, normal timingMEE2aMRP25 dark adaptation37 /--20/20 ; --20/30 ; --Temporal scotomasRods: 50% normalCones: <15% normalMEE3MRP22Nyctalopia25 /--20/100 ; --20/500 ; --20°; temporal crescentsRods: NDCones: SDMEE4FCRD20 vision30 /--20/300 ; --20/300 ; --Not performedRods: ~50% normalCones: ~50% normalMEE5FRP30Nyctalopia46 / 4820/60 ; 20/6020/20 ; 20/60Central & pericentral ring scotomas Rods: NDCones: 20-35% normalMEE7MRP4Nyctalopia, peripheral vision10 /--20/40 ; --20/30 ; --20°; temporal crescentsRods (OD only): NDCones (OD): SD MEE8MRP7Nyctalopia22 /--20/60 ; --20/70 ; --~25° Rods: NDCones: SDMEE9MRP4Nyctalopia, peripheral vision9 / 4720/400b ; LP20/50 ; LP13y: V4e < 8° Rods and cones: NDMEE10MRP5Nyctalopia13 / 5620/30 ; LP20/30 ; LP31y: ~18°; temporal crescentsRods and cones: NDMEE11FRP5Nyctalopia, peripheral vision22 / 2720/200 ; 20/15020/200 ; 20/150~30°; temporal crescentsRods: NDCones: SDMEE12FRP4Nyctalopia13 / 1520/30 ; 20/4020/40 ; 20/40 20°Not performedMEE13FRP28Nyctalopia28 / 3620/20 ; 20/2020/20 ; 20/20Superior constrictionRods: NDCones: 25% normalCEI23745FRP27Nyctalopia30 / 45n/a ; 20/1000n/a ; 20/3035y: ~15°; inferior crescentsRods: NDCones: SD CEI26396FLCA6Nyctalopia10 / 1420/40 ; 20/5020/50 ; 20/80I4e 5-10°; V4e fullRods: NDCones: SD CEI26528aFCRD12Nyctalopia18 / 2220/25 ; 20/2520/30 ; 20/25Central scotoma through I3e OU; V4e fullRods: NDCones: SD CEI26529aMCRD13 vision15 / 1920/20 ; 20/4020/20 ; 20/3016y: Central scotoma through I3e; V4e fullRods: 50-60% normalCones: < 40% normalCEI29023MEOSRD10Nyctalopia13 / --20/20 ; --20/20 ; --I4e ~ 10°; V4e fullRods: NDCones: SDCEI24459FRP15Not available20 / 2720/30 ; n/a20/30 ; n/aNot availableRods: NDCones: SDCEI29345MRP16Nyctalopia37n/aCentral scotomas contiguous with superior peripheral lossRods: NDCones: SDResults are from the first exam unless indicated. All ages are in years.Abbreviations: Dx – diagnosis; MD – macular dystrophy; RP – retinitis pigmentosa; CRD – cone-rod dystrophy; EOSRD – early-onset severe retinal dystrophy; LCA – Leber congenital amaurosis; VA – visual acuity; OD – right eye; OS – left eye; OU – both eyes; LP – light perception; HM – hand motion; ND – nondetectable; SD – severely depressed. a Siblings (Pairs at CEI and MEI) b Anisometropic amblyopia diagnosed as a child.c Goldmann perimetry was used at MEE; Octopus semi-automatic kinetic perimetry was used at CEI. Degrees refer to central horizontal visual field diameter. All data refers to V4e unless indicated.d Classification of cone dysfunction based upon 30 Hz flicker amplitudes; severe depression judged as <1 ?V at MEE (normal range 50-125 ?V) and <5 ?V at CEI (normal range 82-186 ?V). Cone timing delayed unless specified otherwise. ................
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