Figure 4

A,E) Fundus photographs of patient 55 show an atrophic maculopathy involving the fovea in the OD with clumps of pigmentation in the macular area. The OS shows an atrophic maculopathy sparing the inferior half of fovea. Retinal vessels and optic discs are within normal limits. B,F) Fundus auto-fluorescence images show macular atrophy appears dark black involving the fovea in the OD and in the OS inferior half of fovea is spared. C,G) Goldmann Visual Fields show an absolute central scotoma OU. D,H) OCT shows bilateral loss of IS/OS junctions and decreased retinal thickness. Accumulation of auto-fluorescent photoreceptor debris in the foveal area is seen in the OD. I) Cone mediated ERG a-waves are within normal amplitude limits (OU) and peak times are delayed (0D) and within normal limits (OS). The b-waves are within the lower limits of normal amplitude (OU) with peak times that are delayed (0D) and within normal limits (OS). J) The rod-mediated ERG b-wave is within normal amplitude and peak time limits (OU). K) mfERGs are attenuated in amplitude at all eccentricities.