Table 1 of D’Souza, Mol Vis 2013; 19:2209-2216.
Clinical criteria | Patient #645 | Patient #22 |
---|---|---|
Age (yrs) when data recorded | 7 | 21 |
Age (yrs) when symptoms noticed | 7 | 5 |
BCVA-OD, OS | 20/800, 20/50 | 20/50+1, 20/40–1 |
Central visual field defect present (both OD and OS) | Yes. OD | Yes. Both OD and OS |
Foveal/macular cystic changes (both OD and OS) | Yes. Both OD and OS | Yes. Both OD and OS |
Macular atrophy (both OD and OS) | No | No |
Peripheral visual field defect present (both OD and OS) | Yes. Inferonasally OD and OS | No |
Peripheral schisis (both OD and OS) | Yes, peripheral flat schisis superiorly and inferotemporally OS>OD. | No |
Retinal detachment present or treated (both OD and OS) | No | No |
Inheritance Pattern based on family history | Only the proband is affected | Only the proband is affected |
ERG Dark-adapted combined/mixed response wave values- OD | Not performed | a wave=237, b wave=312(μV)* |
ERG Dark-adapted combined/mixed response wave values- OS | Not performed | a wave=240, b wave=318(μV)* |