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Macular Pigment and Lutein Supplementation in Choroideremia
Journal article   Peer reviewed

Macular Pigment and Lutein Supplementation in Choroideremia

Jacque L Duncan, Tomas S Aleman, Leigh M Gardner, Elaine De Castro, Daniel A Marks, Jessica M Emmons, Michelle L Bieber, Janet D Steinberg, Jean Bennett, Edwin M Stone, …
Experimental Eye Research, Vol.74(3), pp.371-381
03/2002
DOI: 10.1006/exer.2001.1126
PMID: 12014918

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Abstract

Choroideremia is an incurable X-linked retinal degeneration caused by mutations in the gene encoding Rab escort protein-1. A group of clinically defined and genotyped patients were studied to determine: (1) the degree of rod and cone dysfunction and structural abnormality in the central retina and the level of macular pigment; and (2) the response of macular pigment and foveal vision to a 6month trial of supplementation with oral lutein (at 20mg per day). Rod and cone-mediated function was measured with dark-adapted static perimetry; in vivo retinal structure was determined with optical coherence tomography; and macular pigment optical density was measured with heterochromatic flicker photometry. In this cohort of patients (ages 15–65 years), both rod- and cone-mediated central function declined with age as did central retinal thickness. Macular pigment levels did not differ between patients and male control subjects. Supplementation of oral lutein in a subset of patients led to an increase in serum lutein and macular pigment levels; absolute foveal sensitivity did not change. It is concluded that macular pigment density can be augmented by oral intake of lutein in patients with choroideremia. There was no short-term change in the central vision of the patients on the supplement, but long-term influences of lutein supplementation on disease natural history warrant further study.
retinal degeneration carotenoids choroideremia REP-1 macular pigment rod cone lutein perimetry

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