Abnormal fundus autofluorescence in relation to retinal function in patients with retinitis pigmentosa

Popović, Petra; Jarc-Vidmar, Martina; Hawlina, Marko

doi:10.1007/s00417-005-1186-x

Cited by 108 publications

(103 citation statements)

References 21 publications

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“…2,51 On the basis of these histological findings, we assume that the increased NIA reflects the presence of melanin and melanolysosomes in areas with preserved cone function. The functional findings in our patients and earlier studies on FAF and retinal function 11,13,14 are in agreement with the histological findings. The area within the rings of increased FAF and NIA are identical with the area of preserved cone function.…”

Section: Discussionsupporting

confidence: 92%

“…41 This ring of increased FAF demarcates the area of preserved cone function, 11,13,14 whereas rod function can be severely affected in areas within the ring. 12 This is puzzling, as FAF intensity at both sides of the ring of increased FAF appears to be similar, and thus FAF intensity does not correspond to photoreceptor function.…”

Section: Discussionmentioning

confidence: 99%

“…10 Characteristic patterns of FAF abnormalities have been reported in RP, and their association with retinal function has been demonstrated. [11][12][13][14] In contrast, melanin is the major protective agent in the RPE cells sheltering the RPE from exposure to light scattering, radiation, oxidative stress, and light damage. 9,15,16 Melanin granules accumulate in the apical part of the RPE cells undergoing a moderate decrease in numbers with age.…”

Section: Introductionmentioning

confidence: 99%

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Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Kellner

Weber

et al. 2008

Eye

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Aims To compare melanin-related nearinfrared fundus autofluorescence (FAF; NIA, excitation 787 nm, emission 4800 nm) with lipofuscin-related FAF (excitation 488 nm, emission 4500 nm) in retinitis pigmentosa (RP). Methods Thirty-three consecutive RP patients with different modes of inheritance were diagnosed clinically, with full-field ERG, and if possible with molecular genetic methods. FAF and NIA imaging were performed with a confocal scanning laser ophthalmoscope (Heidelberg Retina Angiograph 2). Results Rings of increased FAF were present within an area of preserved retinal pigment epithelium (RPE) at the posterior pole (31/33). Rings of increased NIA were located in the same region as rings of increased FAF. In contrast to FAF, NIA showed a precipitous decline of NIA peripheral to the ring. In larger areas of preserved NIA (11/31), pericentral and foveal NIA were of similar intensity with an area of lower NIA in between. In smaller areas of preserved NIA (20/31), NIA was homogeneous from the perifovea to the fovea. In one patient without a ring of increased FAF, NIA distribution was normal. In the remaining patient with severely advanced RP, no residual RPE as well as no FAF and NIA were detectable. Conclusion Characteristic features for FAF and NIA alterations in a heterogeneous group of RP patients indicate a common pathway of RPE degeneration. Patterns of NIA and FAF indicate different pathophysiologic processes involving melanin and lipofuscin. Combined NIA and FAF imaging will provide further insight into the pathogenesis of RP and noninvasive monitoring of future therapeutic interventions.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Kellner

Weber

et al. 2008

Eye

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“…In all cases the central visual field is preserved, being lost only at the end stage of the disease. Studies by Escher et al (2012); Lima et al (2009Lima et al ( , 2012; Murakami et al (2008); Popović et al (2005); Robson et al (2003Robson et al ( , 2004Robson et al ( , 2006Robson et al ( , 2008Robson et al ( , 2011 describe similar patterns of degeneration.…”

Section: Introductionmentioning

confidence: 88%

Mathematical models of retinitis pigmentosa: The oxygen toxicity hypothesis

Roberts

Gaffney

Luthert

et al. 2017

Journal of Theoretical Biology

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The group of genetically mediated diseases, known collectively as retinitis pigmentosa (RP), cause retinal degeneration and, hence, loss of vision. The most common inherited retinal degeneration, RP is currently untreatable. The retina detects light using cells known as photoreceptors, of which there are two types: rods and cones. In RP, genetic mutations cause patches of photoreceptors to degenerate and typically directly affect either rods or cones, but not both. During disease progression, degenerate patches spread and the unaffected photoreceptor type also begins to degenerate. The cause underlying these phenomena is currently unknown.The oxygen toxicity hypothesis proposes that secondary photoreceptor loss is due to hyperoxia (toxically high oxygen levels), which results from the decrease in oxygen uptake following the initial loss of photoreceptors. In this paper, we construct mathematical models, formulated as 1D systems of partial differential equations, to investigate this hypothesis. Using a combination of numerical simulations, asymptotic analysis and travelling wave analysis, we find that degeneration may spread due to hyperoxia, and generate spatiotemporal patterns of degeneration similar to those seen in vivo. We determine the conditions under which a degenerate patch will spread and show that the wave speed of degeneration is a monotone decreasing function of the local photoreceptor density. Lastly, the effects of treatment with antioxidants and trophic factors, and of capillary loss, upon the dynamics of photoreceptor loss and recovery are considered.

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“…[33][34][35]37,39,40 Based on available data, a double hyperautofluorescent ring has not been reported in recessive RP patients so far.…”

Section: Discussionmentioning

confidence: 99%

Double Concentric Autofluorescence Ring inNR2E3-p.G56R-Linked Autosomal Dominant Retinitis Pigmentosa

Escher

Tran

Vaclavik

et al. 2012

Invest. Ophthalmol. Vis. Sci.

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Abnormal fundus autofluorescence in relation to retinal function in patients with retinitis pigmentosa

Cited by 108 publications

References 21 publications

Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Lipofuscin- and melanin-related fundus autofluorescence visualize different retinal pigment epithelial alterations in patients with retinitis pigmentosa

Mathematical models of retinitis pigmentosa: The oxygen toxicity hypothesis

Double Concentric Autofluorescence Ring inNR2E3-p.G56R-Linked Autosomal Dominant Retinitis Pigmentosa

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