“…These findings also may be relevant to our understanding of the paracentral rings of elevated AF in retinitis pigmentosa 64 and/or the bright AF flecks that characterize recessive Stargardt disease. 51,65 …”
When neural retina is disordered, AF from photoreceptor cells can contribute to noninvasive fundus AF images. Hyperautofluorescent puncta in fundus AF images are attributable, in at least some cases, to photoreceptor cell rosettes.
“…These findings also may be relevant to our understanding of the paracentral rings of elevated AF in retinitis pigmentosa 64 and/or the bright AF flecks that characterize recessive Stargardt disease. 51,65 …”
When neural retina is disordered, AF from photoreceptor cells can contribute to noninvasive fundus AF images. Hyperautofluorescent puncta in fundus AF images are attributable, in at least some cases, to photoreceptor cell rosettes.
“…9,10 Later, atrophy of the central retina develops, which spreads centrifugally over time. 11 Based on findings in Abca4 À/À mice, the lack of ABCA4 function results in marked accumulation of the bisretinoid Nretinylidene-N-retinylethanolamine (A2E) in the RPE. 12 A2E is a major component of RPE lipofuscin and appears to have a role in retinal disease pathophysiology.…”
Fundus AF can be used to monitor lipofuscin accumulation and melanin-related changes in vivo in mouse models of retinal disease. High RPE lipofuscin may not adversely affect retinal structure or function over prolonged time intervals, and melanin-related changes (melanolipofuscin formation) may occur before the decline in retinal function.
“…On 5 December 2011, the patient returned to the clinic with a further severe reduction in VA. BCVA test in both eyes was 0.01 this time and the area presenting flecks and fluorescent aggregates had widen considerably when compared with the previous images, reaching the mid-periphery of the retina (figure 1C,D,c,d), findings indicating the end stage of the disease 2 3…”
Section: Case Presentationmentioning
confidence: 57%
“…A characteristic feature of STGD is the presence of yellow-white flecks of lipofuscin. Initially located at the macula, these deposits expanded centrifugally over time2 as figure 1 illustrates. During fluorescence angiography lipofuscin absorbs blue excitatory light and emits a characteristic stippled hyperfluorescence 13…”
Stargardt disease is a common inherited macular degeneration characterised by a significant loss in the central vision during the first or second decade of the life. Bilateral atrophic changes in the central retina are associated with degeneration of photoreceptors and underlying retinal pigment epithelium, and yellow flecks are extending from the macula. We present a patient with Stargardt disease treated with an intravitreal injection of dobesilate, showing an improvement of visual acuity 4 weeks after treatment
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