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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Detalles Bibliográficos
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
Descripción
Sumario: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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