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Severe Loss of Tritan Color Discrimination in RPE65 Associated Leber Congenital Amaurosis

Kumaran, N; Ripamonti, C; Kalitzeos, A; Rubin, GS; Bainbridge, JWB; Michaelides, M; (2018) Severe Loss of Tritan Color Discrimination in RPE65 Associated Leber Congenital Amaurosis. Investigative Ophthalmology & Visual Science , 59 (1) pp. 85-93. 10.1167/iovs.17-22905. Green open access

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Abstract

Purpose: RPE65-associated Leber congenital amaurosis (RPE65-LCA) is a progressive severe retinal dystrophy with early profound dysfunction of rod photoreceptors followed by progressive cone photoreceptor degeneration. We aim to provide detailed information about how cone dysfunction affects color discrimination. Methods: Seven adults (aged 16-21) with RPE65-LCA underwent monocular color discrimination assessment using the Trivector and Ellipse versions of three computerized tests: Cambridge Colour Test (CCT), low vision version of the Cambridge Colour Test (lvvCCT), and the Universal Colour Discrimination Test (UCDT). For comparison, subjects were also tested using the American Optical Hardy Rand Rittler (AO-HRR) plates. Each assessment was repeated three times. Results: The Trivector version of the tests demonstrated that color discrimination along the tritan axis was undetectable in four subjects, and severely reduced in three subjects. These findings were confirmed by the Ellipse version of the tests. Color discrimination along the protan and deutan axes was evident but reduced in six of seven subjects. Four of seven subjects were unable to read any of the HRR plates. Conclusions: The computerized color vision tests adopted in this study provide detailed information about color discrimination in adult RPE65-LCA patients. The condition is associated with severe impairment of color discrimination, particularly along the tritan axis indicating possible early involvement of S-cones, with additional protan and deutan loss to a lesser extent. This psychophysical assessment strategy is likely to be valuable in measuring the impact of therapeutic intervention on cone function.

Type: Article
Title: Severe Loss of Tritan Color Discrimination in RPE65 Associated Leber Congenital Amaurosis
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1167/iovs.17-22905
Publisher version: http://dx.doi.org/10.1167/iovs.17-22905
Language: English
Additional information: This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Keywords: Color vision, retina, Leber congenital amaurosis, LCA, LCA2, clinical trials, endpoints
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10041700
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