Analysis of Autofluorescent retinal images and measurement of atrophic lesion growth in Stargardt disease

Chen, B.; Tosha, Chinatsu; Gorin, Michael B.; Nusinowitz, Steven

doi:10.1016/j.exer.2010.03.021

Cited by 55 publications

(69 citation statements)

References 33 publications

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“…Decreased FAF is a commonly used marker of retinal degeneration in ABCA4 retinopathy. The estimated rates of enlargement of reduced FAF areas in nullizygous patients (1.4-1.5 mm 2 /y) were comparable with the higher end of the range reported by Chen et al (0.2-2.1 mm 2 /y), 27 the rates reported for similar FAF patterns (0.7-4.4 mm 2 /y), 28 and the rates reported for ERG group 3 (2.3 mm 2 /y), 29 highlighting the utility of this parameter for assessing progressive maculopathy in ABCA4-retinopathy. The estimated rates of progression for p.G1961E patients were significantly slower (0.03-0.06 mm 2 /y), highlighting the importance of considering the genotype in future clinical studies, especially involving patients with otherwise overlapping characteristics (e.g., early disease onset).…”

Section: Discussionsupporting

confidence: 74%

Phenotype and Progression of Retinal Degeneration Associated With Nullizigosity of ABCA4

Fakin

Robson

Fujinami

et al. 2016

Invest. Ophthalmol. Vis. Sci.

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Section: Discussionsupporting

confidence: 74%

Phenotype and Progression of Retinal Degeneration Associated With Nullizigosity of ABCA4

Fakin

Robson

Fujinami

et al. 2016

Invest. Ophthalmol. Vis. Sci.

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“…He reported that some cases also had macular degeneration [2,3]. Stargardt's disease and fundus flavimaculatus usually have an autosomal recessive inheritance pattern [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16] and rarely an autosomal dominant pattern [17]. …”

Section: Introductionmentioning

confidence: 99%

“…Because SFF is a complex retinal dystrophy with wide variations in phenotype, it is generally diagnosed by fundus findings and fluorescein angiograms [5]. Recently, fundus autofluorescence imaging has been reported to be helpful in evaluating the status of the photoreceptors/retinal pigment epithelium in eyes with SFF [10,11,12,13,14,15,16]. Foveal sparing, which was observed by ophthalmoscopy and/or fundus autofluorescence imaging, was reported to be present in patients with slowly progressing SFF and relatively good visual acuity [11,13,14].…”

Section: Introductionmentioning

confidence: 99%

Multifocal Electroretinograms in Stargardt's Disease/Fundus Flavimaculatus

et al. 2014

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Purpose: To determine whether the characteristics of multifocal electroretinograms (mfERGs) were correlated with the ophthalmic appearance of the fundus in patients with Stargardt's disease/fundus flavimaculatus (SFF). Methods: Full-field ERGs, mfERGs, and general ophthalmic examinations were performed on 49 eyes with SFF. Results: The SFF patients were divided into four subtypes according to the classification of Noble and Carr [Arch Ophthalmol 1979;97:1281-1285]. The patients with type 1 SFF had severely reduced mfERGs in the macular area and reduced and delayed responses in the mid-periphery. The type 2 SFF patients had reduced but recordable mfERGs from the center of the macula with more depressed responses in the paramacular area, and the type 3 SFF patients had reduced and delayed mfERGs both in the macula and periphery. The patients with type 4 SFF had normal mfERGs in the macular area and delayed responses in all outer zones. Conclusions: These mfERG findings indicate that each subtype of SFF has unique characteristics corresponding to the abnormal retinal functions.

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“…Patients in the third group have a poorer visual prognosis [8,16]. Blue light fundus autofluorescence aids in making a diagnosis, with hyperautofluorescence associated with increased lipofuscin accumulation at the level of the retinal pigment epithelium and hypo-autofluorescence indicating loss of retinal pigment epithelial cells [8,17,18,19,20]. Fujinami et al [21] re-examined the patients described by Lois et al [18], defined 3 autofluorescence subtypes and found an association between baseline subtype, genotype and atrophy progression.…”

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confidence: 99%

Colour Vision in Stargardt Disease

Vandenbroucke¹,

Buyl

Zaeytijd³

et al. 2015

Ophthalmic Res

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Purpose: To investigate the type and severity of acquired colour vision deficiencies (CVDs) in molecularly proven Stargardt disease (STD) and to establish whether a relationship exists between best-corrected visual acuity (BCVA) and full-field electroretinography (ffERG), and the degree of CVD. Methods: A retrospective, cross-sectional study of 73 patients with a molecularly proven diagnosis of STD, who underwent extensive colour vision evaluation, using pseudo-isochromatic and arrangement tests. Thirteen patients underwent Nagel anomaloscopy. Results: Normal colour vision was found in almost 20% of patients. Red/green (R/G) CVDs increased as BCVA declined. About 45% of all R/G CVDs were of the deutan type, although protan type CVDs became progressively apparent when moving from the high to the low BCVA group. An additional blue/yellow CVD was noted in about 25% of patients. In 10/13 patients, a pseudoprotanomaly was noted on anomaloscopy. Severe CVDs leading to scotopization were noted in patients with low BCVA and/or long-standing disease. No statistically significant differences in ERG results were found between groups with or without a CVD. Conclusions: The degree and type of colour vision deficiency in STD patients correlate better with BCVA than with ffERG results. The presence of specific CVDs may help to establish a diagnosis of STD. A battery of colour vision tests is required to properly evaluate CVDs in STD.

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Analysis of Autofluorescent retinal images and measurement of atrophic lesion growth in Stargardt disease

Cited by 55 publications

References 33 publications

Phenotype and Progression of Retinal Degeneration Associated With Nullizigosity of ABCA4

Phenotype and Progression of Retinal Degeneration Associated With Nullizigosity of ABCA4

Multifocal Electroretinograms in Stargardt's Disease/Fundus Flavimaculatus

Colour Vision in Stargardt Disease

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