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Structural and Functional Characteristics of Color Vision Changes in Choroideremia

Jolly, Jasleen K; Simunovic, Matthew P; Dubis, Adam M; Josan, Amandeep S; Robson, Anthony G; Bellini, Marco P; Bloch, Edward; ... MacLaren, Robert E; + view all (2021) Structural and Functional Characteristics of Color Vision Changes in Choroideremia. Frontiers in Neuroscience , 15 , Article 729807. 10.3389/fnins.2021.729807. Green open access

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Abstract

Color vision is considered a marker of cone function and its assessment in patients with retinal pathology is complementary to the assessments of spatial vision [best-corrected visual acuity (BCVA)] and contrast detection (perimetry). Rod-cone and chorioretinal dystrophies—such as choroideremia—typically cause alterations to color vision, making its assessment a potential outcome measure in clinical trials. However, clinical evaluation of color vision may be compromised by pathological changes to spatial vision and the visual field. The low vision Cambridge Color Test (lvCCT) was developed specifically to address these latter issues. We used the trivector version of the lvCCT to quantify color discrimination in a cohort of 53 patients with choroideremia. This test enables rapid and precise characterization of color discrimination along protan, deutan, and tritan axes more reliably than the historically preferred test for clinical trials, namely the Farnsworth Munsell 100 Hue test. The lvCCT demonstrates that color vision defects—particularly along the tritan axis—are seen early in choroideremia, and that this occurs independent of changes in visual acuity, pattern electroretinography and ellipsoid zone area on optical coherence tomography (OCT). We argue that the selective loss of tritan color discrimination can be explained by our current understanding of the machinery of color vision and the pathophysiology of choroideremia.

Type: Article
Title: Structural and Functional Characteristics of Color Vision Changes in Choroideremia
Location: Switzerland
Open access status: An open access version is available from UCL Discovery
DOI: 10.3389/fnins.2021.729807
Publisher version: https://doi.org/10.3389/fnins.2021.729807
Language: English
Additional information: © 2021 Jolly, Simunovic, Dubis, Josan, Robson, Bellini, Bloch, Georgiadis, da Cruz, Bridge and Maclaren. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY).
Keywords: color vision, choroideremia, cones, retinal degeneration, ellipsoid zone, pattern electroretinogram, Cambridge color test
UCL classification: 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
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
URI: https://discovery.ucl.ac.uk/id/eprint/10149166
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