Peripheral Cone Dystrophy: Expanded Clinical Spectrum, Multimodal and Ultrawide-Field Imaging, and Genomic Analysis

Figure 1

Full field electroretinography (ffERG), multifocal electroretinography (mfERG), and visual field testing of case 1. (a) Full field electroretinography demonstrated low normal a- and b-wave amplitudes and implicit times on scotopic testing. Photopic testing showed moderately reduced a- and b-wave amplitudes with mildly delayed implicit times. See Supplementary Figure 1 for normal reference and ffERG for cases 2 and 3. (b) Central amplitudes were reduced in the central and paracentral 15° with relative sparing of the fovea on multifocal electroretinography. See Supplementary Figure 2 for normal reference and mfERG testing for cases 2 and 3. (c) Humphrey Sita-Standard protocol with size III stimulus revealed bilateral dense paracentral scotomas that spared foveal sensitivities. See Supplementary Figure 3 for visual field testing of cases 2 and 3.

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