Figure 2.
(A) Patient III:2 (48 years old) Fundus photographs showing bilateral macular RPE disturbance with a well defined area of atrophy at the left macula. (B) Patient III:2 (48 years old) Fundus autofluorescence imaging. RE: Concentric rings of increased and decreased AF in a BEM-like pattern. LE: Decreased AF corresponding to atrophy seen on ophthalmoscopy with a surrounding ring of relative increased AF. (C) Patient IV:3 (31 years old). Fundus photography showing extensive macular RPE atrophy and areas of pigmentation, worse in the left than right eye. (D) Patient IV:3 (31 years old). Fundus autofluorescence imaging showing bilateral decreased AF centrally with a surrounding ring of relative increased AF. (E) Patient II:2 (81 years old). Fundus photographs showing bilateral symmetrical well circumscribed macular atrophy and attenuated retinal vessels.