The Effect of Light Deprivation in Patients With Stargardt Disease

MM Teussink, MD Lee, RT Smith, RAC van Huet, Caroline Klaver, BJ Klevering, T Theelen, C Hoyng

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21 Citations (Scopus)

Abstract

PURPOSE: To investigate whether long-term protection from light exposure affects the rate of disease progression in patients with autosomal recessive Stargardt disease (STGD1), measured using fundus autofluorescence imaging. DESIGN: Longitudinal, retrospective, interventional case series. METHODS: Five patients with Stargardt disease protected 1 eye from light exposure by applying a black contact lens during waking hours for 12 months. Disease progression was followed by performing autofluorescence imaging at semi-regular intervals. Longitudinal changes in autofluorescence were studied by evaluating areas of decreased autofluorescence and areas of increased autofluorescence as a measure of retinal pigment epithelium damage and lipofuscin accumulation, respectively. RESULTS: We observed less progression of decreased autofluorescence in 4 out of 5 light-protected eyes relative to their respective nonprotected eyes. The progression of increased autofluorescence, on the other hand, was highly variable and did not respond consistently to treatment. CONCLUSIONS: Areas of decreased autofluorescence may serve as a useful biomarker for measuring the progression of Stargardt disease. The reduced progression of decreased autofluorescence in the light-protected eyes suggests that light deprivation might be beneficial in patients with Stargardt disease. (C) 2015 by Elsevier Inc. All rights reserved.
Original languageUndefined/Unknown
Pages (from-to)964-972
Number of pages9
JournalAmerican Journal of Ophthalmology
Volume159
Issue number5
DOIs
Publication statusPublished - 2015

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