The cone degenerations

Krill, Alex E.; Deutman, August F.; Fishman, Marlene

doi:10.1007/bf00234530

Cited by 145 publications

(72 citation statements)

References 41 publications

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“…Bull's eye maculopathy can be seen on fundus exam often following the onset of vision changes [6]. ERG will show a decrease in cone-mediated responses with normal scotopic ERG findings [7]. As in Stargardt disease, patients with a progressive cone dystrophy tend to present in late childhood and young adulthood.…”

Section: Differential Diagnosismentioning

confidence: 99%

Macular Telangiectasia: A cause of bull’s eye maculopathy

Schultis¹,

Nguyen²

2017

New Front Ophthalmol

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A 52-year-old woman with blurred vision was found to have a bull's eye lesion in both eyes. On optical coherence tomography, she was found to have outer retinal atrophy with inner subfoveal cysts. A reduction in the normal foveal hypoautofluorescence and abnormalities on multifocal electroretinography were also observed. Visual field testing, fluorescein angiography , electrooculography, and full-field electoretinography were also conducted. Her findings are consistent with a diagnosis of Macular Telangiectasia type 2 and this case demonstrates the importance of keeping Macular Telangiectasia type 2 on the differential for patients demonstrating a bull's eye maculopathy.

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Section: Differential Diagnosismentioning

confidence: 99%

Macular Telangiectasia: A cause of bull’s eye maculopathy

Schultis¹,

Nguyen²

2017

New Front Ophthalmol

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“…The fairly rare cone dystrophy is classified into the congenital stationary form with inborn absence or functional disorder of the cones, and the progressive form [1][2][3][4][5][6][7] . Progressive cone dystrophy (PCD) is inherited autosomally dominantly or recessively, or recessively linked to the X chromosome, however its incidence is often sporadic [1][2][3][4][5][6][7][8][9][10][11][12] . Visual acuity (VA) in the affected individuals is usually normal during childhood and only gradually deteriorates, often leading to legal blindness.…”

Section: Introductionmentioning

confidence: 99%

“…Visual acuity (VA) in the affected individuals is usually normal during childhood and only gradually deteriorates, often leading to legal blindness. Impaired VA is accompanied by a defective colour sense, visual field defects, photophobia 1,2,[4][5][6][7][8][9][10][13][14][15] and in some patients with affected rods also by nyctalopia 3,6,7,9 . Visual field defects include central scotomata 1,3,6,9,12,13 , annular scotomata around the centre 1,3 , diffuse reduction in sensitivity 12 and rarely, peripheral scotomata [1][2][3]6,9 .…”

Section: Introductionmentioning

confidence: 99%

“…A finding in the macula may vary within the range of non-specific changes to the retinal pigment epithelium (RPE), to the characteristic RPE lesions of the bull's eye type [1][2][3][4][5][6][7]9,[13][14][15] . Changes in the macula are rarely accompanied by the temporal disc pallor 1,3,4,6,[12][13][14] , golden tapetal sheen 8,12 , white dot-like lesions at the level of RPE mostly in the mid-periphery 1,3 , pigment shifts in the periphery 1,3,6,9,15 and nystagmus 2,4,6,9,11,14 .…”

Section: Introductionmentioning

confidence: 99%

“…Changes in the macula are rarely accompanied by the temporal disc pallor 1,3,4,6,[12][13][14] , golden tapetal sheen 8,12 , white dot-like lesions at the level of RPE mostly in the mid-periphery 1,3 , pigment shifts in the periphery 1,3,6,9,15 and nystagmus 2,4,6,9,11,14 . Electrophysiological examinations show clear reduction in or even elimination of the cone function with normal or reduced response of the rods.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Optical coherence tomography in progressive cone dystrophy

Záhlava¹,

Lešták²,

Karel³

2014

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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Visual Field Loss in Patients With Glaucoma Who Have Asymmetric Peripapillary Focal Arteriolar Narrowing

Lam

Bunya²,

Piltz-Seymour³

2002

Arch Ophthalmol

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To evaluate the relationship between peripapillary focal arteriolar narrowing and visual field defects. Methods: From our institutional practice, we identified 31 patients with glaucoma who had peripapillary focal arteriolar narrowing in only one eye and compared visual field data between the two eyes. Mean deviation (MD) and corrected pattern standard deviation (CPSD) were recorded using Humphrey visual field testing at the time proximal narrowing was apparent on the fundus photograph. Visual field data from subsets of patients with mild and severe narrowing were also compared. Results: The MD and CPSD were significantly worse in eyes with peripapillary focal arteriolar narrowing. The eyes with narrowing exhibited a mean MD of −8.77±8.27 dB and a mean CPSD of 5.01±3.42 dB. Eyes without narrowing displayed a mean MD of −4.52 ± 6.64 dB and a mean CPSD of 3.01±2.68 dB (P =.003 for both). There was no significant difference in severity of the visual field defect between eyes with mild and severe arteriolar narrowing. Conclusion: To our knowledge, this is the first study to show that the presence of peripapillary focal arteriolar narrowing is related to the severity of visual field loss in patients with glaucoma.

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The cone degenerations

Cited by 145 publications

References 41 publications

Macular Telangiectasia: A cause of bull’s eye maculopathy

Macular Telangiectasia: A cause of bull’s eye maculopathy

Optical coherence tomography in progressive cone dystrophy

Visual Field Loss in Patients With Glaucoma Who Have Asymmetric Peripapillary Focal Arteriolar Narrowing

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