Foveal Densitometry as a Diagnostic Technique in Stargardt's Disease

Meel, Gerard J. van; Norren, Dirk van

doi:10.1016/0002-9394(86)90011-5

Cited by 19 publications

(8 citation statements)

References 14 publications

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“…Because normal cone optical density is approximately 0.33 for a 2 ± field, 29 for our patients with hereditary macular degeneration the mean cone optical density for a 2 ± field is only approximately 60% of the normal value. This estimate is in the range found by means of a densitometric study 7 in which seven eyes of four patients with hereditary macular degeneration and acuity 20͞32 or better had optical densities ranging from 3% to 75% of the normal value.…”

Section: Discussionsupporting

confidence: 66%

“…15,16 For both hereditary macular degeneration and retinitis pigmentosa, reduction in cone optical density can precede loss of acuity. 5,7,17,18 For patients with retinitis pigmentosa, cone degeneration is often greater in the parafovea than in the fovea, 19 whereas in hereditary macular degeneration cone damage is often greatest in the fovea. 3 Therefore the pattern of optical-density reduction across the macula may be different in these two diseases, with hereditary macular degeneration causing greater losses in the fovea and retinitis pigmentosa causing greater losses in the parafovea.…”

Section: Introductionmentioning

confidence: 99%

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Color matches in diseased eyes with good acuity: detection of deficits in cone optical density and in chromatic discrimination

Swanson

Fish

1995

J. Opt. Soc. Am. A

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Reduced foveal cone optical density in diseased eyes with normal acuity can affect color matches. Using field diameters of 1 degree, 2 degrees, 4 degrees, and 8 degrees, we measured mean color-match midpoints and match widths in patients who had good acuity and who exhibited three categories of eye disease: hereditary macular degeneration (n = 12), retinitis pigmentosa (n = 19), and glaucoma (n = 18). Results were compared with those for normal observers of comparable ages. Mean color-match midpoints were abnormal only for the population with hereditary macular degeneration, indicating a reduction in cone optical density in the central 4 degrees. Mean color-match widths were enlarged for both hereditary macular degeneration and retinitis pigmentosa, a result consistent with a reduction in the number of foveal cones.

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Section: Discussionsupporting

confidence: 66%

Section: Introductionmentioning

confidence: 99%

Color matches in diseased eyes with good acuity: detection of deficits in cone optical density and in chromatic discrimination

Swanson

Fish

1995

J. Opt. Soc. Am. A

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“…Indeed a recent foveal densitometric study of patients with Stargardt's disease reported low two-way densities. 16 Another explanation for the observed changes in Stargardt's disease suggests that, as central cones are lost, temporal sensitivity may be increasingly determined by peripheral retinal elements.…”

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confidence: 99%

Losses of temporal modulation sensitivity in retinal degenerations.

Seiple

Greenstein

Carr

1989

British Journal of Ophthalmology

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Retinitis pigmentosa (RP) is a heterogenous group of retinal disorders which is associated with a progressive degeneration of the photoreceptors. Most patients with this disorder present with subjective symptoms indicative of a disorder of the rod system; however, there is ample evidence for cone system involvement as well. '4 Stargardt's disease is an autosomal recessive juvenile macular degeneration involving atrophy of the retinal pigment epithelium (RPE) and associated progressive degeneration of the photoreceptors. Whereas in RP peripheral vision is initially affected, in Stargardt's disease the macular area is primarily affected.9 "In a previous study we assessed changes in cone system temporal sensitivity in patients with these retinal diseases using a focal electroretinographic technique (FERG).6 Both RP and Stargardt's disease patients showed amplitude losses over the range of temporal frequencies tested; however, the pattern of loss differed between the two groups. RP patients with good visual acuities showed FERG amplitude losses at the higher temporal frequencies. In RP patients with decreased visual acuity their was an overall amplitude loss which was most severe at the higher temporal frequencies. In contrast, patients with Stargardt's disease had FERG amplitude losses Correspondence to W Seiple. PhD. 440at both the low and high temporal frequencies, with responses to the mid-temporal frequencies being relatively spared (see insert to Fig. 3).Various hypotheses have been proposed to account for the sensitiyity losses of the cone system in these diseases. One e (planation for RP attributes a major component of s ensitivity loss to a decrease in quantum catching ability of the photoreceptors. This could result from a decrease in photopigment density, a misalignment of photoreceptors, or a shortening of photoreceptors. Histological examination of RP retinas, psychophysical studies of RP patients by colour matching techniques, and foveal densitometric studies all lend support to this explanation.7 11-13Another explanation for the observed temporal sensitivity losses in RP patients suggests that time constants, or the dynamic adaptation properties, of the cones of the central retina are changed. This deficit would have the greatest effect on sensitivity to higher temporal frequency stimuli.61415The changes in sensitivity exhibited by patients with Stargardt's disease have also been explained by a decrease in quantum catching ability of the cone system. Indeed a recent foveal densitometric study of patients with Stargardt's disease reported low two-way densities. 16 Another explanation for the on 8 May 2018 by guest. Protected by copyright.

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“…Noble and Carr [4] reported ERG anomalies in only eight of the 50 patients tested, without saying if they were SD or FFM. Van Meel and van Norren [8] reported abnormal ERGs in two of their six SD patients, while their two FFM subjects had normal ERGs. In their study Hadden and Gass [11] reported seven of 36 patients with abnormal ERGs, but no information is given on which of the two conditions yielded the abnormal electroretinal signal.…”

Section: Discussionmentioning

confidence: 99%

“…They also reported that the age of onset was significantly lower in FFM. Foveal densitometry measurements by van Meel and van Norren [8] also indicated a difference between FFM and SD, where the former was found to be closer to normal while the latter yielded markedly abnormal results. However, most of these studies did not provide the necessary information on how the diagnosis was made and at what stage was the disease process.…”

Section: Introductionmentioning

confidence: 96%

The electroretinogram in Stargardt's disease and fundus flavimaculatus

1989

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A retrospective study was performed comparing the ERG results of 15 patients with Stargardt's disease and fundus flavimaculatus. Patients with fundus flavimaculatus had "fish-tail" lesions with or without macular changes, while the Stargardt's group had macular atrophy without fish-tail flecks. The mean visual acuity was 20/200 for the Stargardt's patients compared with a mean of 20/80 for the fundus flavimaculatus patients. The Stargardt's photopic and scotopic amplitudes were respectively 33% and 34% of normal, while the fundus flavimaculatus values were less impaired at 58% and 64% of normal.

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Foveal Densitometry as a Diagnostic Technique in Stargardt's Disease

Cited by 19 publications

References 14 publications

Color matches in diseased eyes with good acuity: detection of deficits in cone optical density and in chromatic discrimination

Color matches in diseased eyes with good acuity: detection of deficits in cone optical density and in chromatic discrimination

Losses of temporal modulation sensitivity in retinal degenerations.

The electroretinogram in Stargardt's disease and fundus flavimaculatus

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