Figure 2.

Intra-familial phenotypic variation in family #18. The proband (A) had pericentral RP, with near-peripheral C-shaped scotomas with preserved peripheral I4e responses, and relatively preserved full-field ERG responses. His children, in contrast, had either atypically mild RP without pericentral features (B) or typical RP (C,D). Note the constricted peripheral I4e stimulus responses and lower ERG response amplitudes in the children (B–D) in comparison to the proband. undet = undetectable.