Macular pigment distribution in Stargardt macular disease.

نویسندگان

  • M-B Rougier
  • M-N Delyfer
  • J-F Korobelnik
چکیده

PURPOSE To investigate macular pigment optical density (MPOD) and distribution in patients with Stargardt disease. METHODS Prospective observational case series. The study included 13 eyes of 13 consecutive patients. A modified confocal scanning laser ophthalmoscope (SLO, Heidelberg, Germany) was used for MPOD measurement. It calculated the MPOD at 0.5° of the center of the fovea, and MPOD in the 0.5° and 2° areas. RESULTS Two different MPOD profile patterns were observed: group 1 was composed of patients with a flat profile, i.e., with very low MPOD in all locations, and group 2 presented a normal profile. In group 2, all eyes but one had good visual acuity (VA); in group 1, some displayed poor VA, but others had good VA. All patients in group 1 displayed a thinning of the macular area on OCT. CONCLUSIONS These results suggest that the flat MPOD profile, even if the visual acuity is good, could be associated with poor prognosis. The two different patterns of MPOD distribution described could reflect two different stages in the course of Stargardt disease. No strong correlations were found between MPOD profiles and visual acuity or macular thickness, but a straight trend was brought out that may indicate that the flat MPOD profile has a poor functional prognosis. Long follow-up is required to confirm this hypothesis.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Correlation of Macular Focal Electroretinogram with Ellipsoid Zone Extension in Stargardt Disease

Stargardt disease (STGD1) is the most common cause of inherited juvenile macular degeneration. This disease is characterized by a progressive accumulation of lipofuscin in the outer retina and subsequent loss of photoreceptors and retinal pigment epithelium. The aim of this study was to evaluate the relationship between cone photoreceptor function and structure in STGD1. Macular function was as...

متن کامل

Towards Treatment of Stargardt Disease: Workshop Organized and Sponsored by the Foundation Fighting Blindness

Accumulation of fluorescent metabolic byproducts of the visual (retinoid) cycle is associated with photoreceptor and retinal pigment epithelial cell death in both Stargardt disease and atrophic (nonneovascular) age-related macular degeneration (AMD). As a consequence of this observation, small molecular inhibitors of enzymes in the visual cycle were recently tested in clinical trials as a strat...

متن کامل

Complement system dysregulation and inflammation in the retinal pigment epithelium of a mouse model for Stargardt macular degeneration.

Accumulation of vitamin A-derived lipofuscin fluorophores in the retinal pigment epithelium (RPE) is a pathologic feature of recessive Stargardt macular dystrophy, a blinding disease caused by dysfunction or loss of the ABCA4 transporter in rods and cones. Age-related macular degeneration, a prevalent blinding disease of the elderly, is strongly associated with mutations in the genes for comple...

متن کامل

C20-D3-vitamin A slows lipofuscin accumulation and electrophysiological retinal degeneration in a mouse model of Stargardt disease.

Stargardt disease, also known as juvenile macular degeneration, occurs in approximately one in 10,000 people and results from genetic defects in the ABCA4 gene. The disease is characterized by premature accumulation of lipofuscin in the retinal pigment epithelium (RPE) of the eye and by vision loss. No cure or treatment is available. Although lipofuscin is considered a hallmark of Stargardt dis...

متن کامل

Resonance Raman measurement of macular carotenoids in retinal, choroidal, and macular dystrophies.

BACKGROUND It has been hypothesized that the macular carotenoid pigments lutein and zeaxanthin may protect against macular and retinal degenerations and dystrophies. OBJECTIVE To test this hypothesis by objectively measuring lutein and zeaxanthin levels in a noninvasive manner in patients who have retinitis pigmentosa (RP), choroideremia (CHM), and Stargardt macular dystrophy and comparing th...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Journal francais d'ophtalmologie

دوره 34 5  شماره 

صفحات  -

تاریخ انتشار 2011