Retinal Function in Stargardt's Disease and Fundus Flavimaculatus

Moloney, Jane B.M.; Mooney, David J.; O'Connor, M B

doi:10.1016/0002-9394(83)90455-5

Cited by 39 publications

(29 citation statements)

References 4 publications

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“…Results of previous studies were comparable and showed slight R/G CVDs after disease onset, progressively evolving towards stronger red defects, often associated with blue defects, eventually leading to scotopization [23,38]. The Farnsworth-Munsell 100-hue test also showed an axis that was predominantly R/G [14,50]. However, others using the same test found that acquired dyschromatopsias in the B/Y axis predominated in STD patients [7].…”

Section: Discussionmentioning

confidence: 53%

“…Just 16% of the 50 cases seen by Noble and Carr [13] had reduced amplitudes on ganzfeld ERG, and 70% of the ERG recordings reported by Gelisken and De Laey [47] were within the normal limits. In contrast, others identified abnormalities in all of the STD patients [14]. Again, this high variability can be partially explained by the heterogeneous characteristics of the patients included, as well as the diagnostic uncertainty in the premolecular era.…”

Section: Discussionmentioning

confidence: 92%

“…Two other series tested patients with the Ishihara plates, HRR plates or Farnsworth PD-15 and demonstrated normal colour vision in over 60% of cases [13,53]. According to some, the rate of abnormal colour vision is a lot higher in patients with FFM compared to Stargardt macular dystrophy [14,50] whereas others found normal colour vision in almost every patient with FFM [15]. More sophisticated analysis with anomaloscopy, the Farnsworth-Munsell 100-hue test or colour contrast sensitivity documented a nearly 100% rate of colour vision abnormality [5,14,23].…”

Section: Discussionmentioning

confidence: 99%

“…These represent accumulations of lipofuscin within retinal pigment epithelial cells [5,9,10,11]. Different fundus findings have been used to subclassify STD [7,12,13,14]. However, since fundus appearance changes over time in patients with STD [7,15], and since the fundus appearance does not always correlate with retinal function [7,12], a classification based on funduscopy is not clinically useful.…”

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confidence: 99%

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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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Section: Discussionmentioning

confidence: 53%

Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Colour Vision in Stargardt Disease

Vandenbroucke¹,

Buyl

Zaeytijd³

et al. 2015

Ophthalmic Res

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“…Peripheral vision is useful for moving about with premises. The disease is diagnosed by teenage [7,20]. The progression of disease in the retina as well as in area of central vision tends to be extensive over time.…”

Section: Rehabilitation Plan Imentioning

confidence: 99%

Low Vision Management in a Case of Stargardt's Disease

Dhungel¹

2015

AOVS

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Phenotypic Subtypes of Stargardt Macular Dystrophy–Fundus Flavimaculatus

et al. 2001

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To determine if phenotypic subtypes exist in Stargardt macular dystrophy-fundus flavimaculatus (SMD-FFM). Methods: A cross-sectional study of 63 patients with autosomal recessive SMD-FFM was undertaken. The age of onset, duration of symptoms, visual acuity, and clinical features on fundus examination, color fundus photographs, and fundus autofluorescence images were recorded. Electrophysiological tests, including pattern, focal, and fullfield electroretinogram (ERG), electro-oculogram, and colorcontrast sensitivity measurement, were also performed. Results: Based on electrophysiological attributes (ERG), patients with SMD-FFM could be classified into 3 groups. In group 1, there was severe pattern ERG abnormality with normal scotopic and full-field ERGs. In group 2, there was additional loss of photopic function, and in group 3, there was loss of both photopic and scotopic function. Differences in scotopic or photopic function among groups were not explained on the basis of differences in age of onset or duration of disease. Conclusions: Patients with SMD-FFM can be classified into 3 groups based on the absence or presence of generalized loss of either photopic or photopic and scotopic function. It appears that these 3 groups may represent distinct phenotypic subtypes in SMD-FFM.

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Retinal Function in Stargardt's Disease and Fundus Flavimaculatus

Cited by 39 publications

References 4 publications

Colour Vision in Stargardt Disease

Colour Vision in Stargardt Disease

Low Vision Management in a Case of Stargardt's Disease

Phenotypic Subtypes of Stargardt Macular Dystrophy–Fundus Flavimaculatus

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