{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:20:52Z","timestamp":1787026852725,"version":"build-2736575974"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T00:00:00Z","timestamp":1769040000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Alport syndrome (AS) is an inherited disease caused by the absence of type IV collagen from glomerular, cochlear, and ocular basement membranes. Ocular manifestations of AS include corneal opacity, peripheral and central fleck retinopathy, and temporal thinning on OCT. These findings in AS usually do not cause any vision loss. We report a case of bilateral vision loss with optic disc edema, macular edema, and macular star figure in AS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case description<\/jats:title>\n                    <jats:p>A 27-year-old woman presented with progressive, painless bilateral blurry vision over 2\u202fweeks. She had genetically confirmed X-linked COL4A5 Alport syndrome and uncontrolled hypertension despite four antihypertensive medications. Visual acuity was 20\/50 OU. Ishihara color testing was 6\/14 OD and 2\/14 OS. Fundoscopy showed moderate optic disc edema, a complete macular star, and tortuous vessel OU. Humphrey visual fields revealed severe constriction (mean deviation was \u221217.65\u202fdB OS and \u221221.98\u202fdB OD). Blood pressure was 163\/93\u202fmm Hg. OCT showed a peripapillary RNFL of 245\u202f\u03bcm OD and 204\u202f\u03bcm OS. MRI indicated an enlarged, partially empty sella and bilateral patulous optic nerve sheath. Lumbar puncture opening pressure was 13\u202fcm H\u2082O with mildly elevated CSF protein and no pleocytosis. Infectious and inflammatory neuroretinitis workup was negative. She was started on acetazolamide 250\u202fmg twice daily and transitioned to hemodialysis. After 9\u202fmonths, vision improved to 20\/30 OU, color plates to 10\/14 OD and 8\/14 OS, and OCT RNFL to 139\u202f\u03bcm OD and 128\u202f\u03bcm OS. Visual fields improved (mean deviation \u22123.13\u202fdB OS, \u22123.8\u202fdB OD).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>We consider the possibility of hypertensive retinopathy or idiopathic neuroretinitis in this patient with Alport syndrome. Given the disproportionate degree of macular edema and prominent macular star, in the absence of common manifestations of hypertensive retinopathy such as retinal hemorrhages or hard exudates, we suspect a possible superimposed neuroretinitis-like exudative mechanism driven by the absence of type IV collagen, providing structural support in Alport syndrome patients. This complex symptom presentation, to our knowledge, has never been described in the context of Alport syndrome. Further research is needed to better understand the relationship between Alport-related renal disease, hypertensive retinopathy, and neuroretinitis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fneur.2026.1745094","type":"journal-article","created":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T07:59:07Z","timestamp":1769068747000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: Neuroretinitis versus hypertensive retinopathy secondary to Alport syndrome"],"prefix":"10.3389","volume":"17","author":[{"given":"Rohini","family":"Chahal","sequence":"first","affiliation":[{"name":"The University of Texas Health Science Center at Houston, McGovern Medical School","place":["Houston, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amna M.","family":"Ali","sequence":"additional","affiliation":[{"name":"The University of Texas Health Science Center at Houston, McGovern Medical School","place":["Houston, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Safa","family":"Ibrahim","sequence":"additional","affiliation":[{"name":"Blanton Eye Institute, Houston Methodist Hospital","place":["Houston, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dina","family":"Abdelsalam","sequence":"additional","affiliation":[{"name":"Blanton Eye Institute, Houston Methodist Hospital","place":["Houston, TX, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew G.","family":"Lee","sequence":"additional","affiliation":[{"name":"Blanton Eye Institute, Houston Methodist Hospital","place":["Houston, TX, United States"]},{"name":"Departments of Ophthalmology, Neurology, and Neurosurgery, Weill Cornell Medicine","place":["New York, NY, United States"]},{"name":"Department of Ophthalmology, University of Texas Medical Branch","place":["Galveston, TX, United States"]},{"name":"Department of Ophthalmology, The University of Iowa Hospitals and Clinics","place":["Iowa City, IA, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,1,22]]},"reference":[{"key":"ref1","article-title":"Alport Syndrome","volume-title":"StatPearls","author":"Watson","year":"2023"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"161","DOI":"10.3109\/1381681970904143","article-title":"Alport syndrome. 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