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. 2016 Jul 7;99(1):236–245. doi: 10.1016/j.ajhg.2016.05.026

Figure 4.

Figure 4

Pigmentary Retinopathy and Loss of Visual Acuity in CS_258

(A) Fundus photography taken by digital Fundus Camera (Carl Zeiss Meditec, VISUCAM 500) showed retinal pigmentary changes, and an abnormal appearance of the macula with attenuated arteriolar vessels (the arrow indicates the fovea centralis).

(B and C) Left (B) and right (C) eye electrophysiological examination done to assess visual function of the proband: sVEP (sweep visual evoked potential) was performed 1 m from the optoelectronic stimulator (Metrovision-Vision Monitor). Potential for mean and maximum visual acuity in both eyes was obtained from sVEP recordings. A VEP produced by a pattern stimulus that is altered at a high temporal frequency rate between 5 and 15 Hz was obtained. Recorded amplitudes were consistent with an equivalent Snellen visual acuity of 0.11 (mean) and 0.28 (maximal) for the left eye and 0.29 (mean) and 0.41 (maximal) for the right eye. The sVEP shows maximal amplitude values in four phases corresponding to Snellen equivalent. Maximal visual acuity and mean visual acuity for the left eye was consistent with 0.28 and 0.11, respectively (B). Maximum visual acuity and mean visual acuity for the right eye was consistent with 0.41 and 0.29, respectively (C).