Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome

We report a 39-year-old with Alport's syndrome. The patient presented with anterior lenticonus, cataract, and a corrected distance visual acuity of 20/25 and 20/60 in the right and left eyes, respectively. Fundus examination revealed generalized retinal flecks sparing the fovea in both eyes. Op...

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Autores principales: Al Akeely, Adel, Schatz, Patrik, Alhazimi, Amro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9198527/
https://www.ncbi.nlm.nih.gov/pubmed/35719281
http://dx.doi.org/10.4103/meajo.meajo_7_21
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author Al Akeely, Adel
Schatz, Patrik
Alhazimi, Amro
author_facet Al Akeely, Adel
Schatz, Patrik
Alhazimi, Amro
author_sort Al Akeely, Adel
collection PubMed
description We report a 39-year-old with Alport's syndrome. The patient presented with anterior lenticonus, cataract, and a corrected distance visual acuity of 20/25 and 20/60 in the right and left eyes, respectively. Fundus examination revealed generalized retinal flecks sparing the fovea in both eyes. Optical coherence topography showed temporal macular thinning. Normal fundus autofluorescence was observed in both eyes. Full-field electroretinography (ERG) demonstrated normal photopic and scotopic responses, while multifocal ERG showed no reduction of amplitudes generated from the temporal thinned macula, compared to the nasal macula, indicating preserved functional integrity of the retina.
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spelling pubmed-91985272022-06-16 Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome Al Akeely, Adel Schatz, Patrik Alhazimi, Amro Middle East Afr J Ophthalmol Case Report We report a 39-year-old with Alport's syndrome. The patient presented with anterior lenticonus, cataract, and a corrected distance visual acuity of 20/25 and 20/60 in the right and left eyes, respectively. Fundus examination revealed generalized retinal flecks sparing the fovea in both eyes. Optical coherence topography showed temporal macular thinning. Normal fundus autofluorescence was observed in both eyes. Full-field electroretinography (ERG) demonstrated normal photopic and scotopic responses, while multifocal ERG showed no reduction of amplitudes generated from the temporal thinned macula, compared to the nasal macula, indicating preserved functional integrity of the retina. Wolters Kluwer - Medknow 2022-04-30 /pmc/articles/PMC9198527/ /pubmed/35719281 http://dx.doi.org/10.4103/meajo.meajo_7_21 Text en Copyright: © 2022 Middle East African Journal of Ophthalmology https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Al Akeely, Adel
Schatz, Patrik
Alhazimi, Amro
Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome
title Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome
title_full Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome
title_fullStr Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome
title_full_unstemmed Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome
title_short Electrophysiological Evaluation of Fleck Retina and Temporal Macular Thinning in X-Linked Alport’s Syndrome
title_sort electrophysiological evaluation of fleck retina and temporal macular thinning in x-linked alport’s syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9198527/
https://www.ncbi.nlm.nih.gov/pubmed/35719281
http://dx.doi.org/10.4103/meajo.meajo_7_21
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AT alhazimiamro electrophysiologicalevaluationoffleckretinaandtemporalmacularthinninginxlinkedalportssyndrome

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