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. 2019 Apr;60(5):1680–1695. doi: 10.1167/iovs.19-26672

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Copyright 2019 The Authors

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Slow rate of cone photoreceptor degeneration in CEP290-LCA. (A) Horizontal OCT from a CEP290-LCA patient (left) demonstrating the foveal ONL thickness and ONL extent measures. Near-infrared autofluorescence imaging (right) demonstrating preserved central macular region of RPE melanization. (B, C) Quantitation of foveal ONL thickness (B), and ONL extent from fovea in nasal and temporal directions (C) in a group of CEP290-LCA patients evaluated cross-sectionally at different ages. Also shown are a subset of patients with longitudinal data (connected symbols). Linear regressions (thick gray line) fit to all data. Redrawn from data in Jacobson SG, Cideciyan AV, Sumaroka A, et al. Outcome measures for clinical trials of Leber congenital amaurosis caused by the intronic mutation in the CEP290 gene. Invest Ophthalmol Vis Sci. 2017;58:2609–2622. © 2017 The Authors. Published by ARVO.