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Visual Improvement With the Use of Idebenone in the Treatment of Wolfram Syndrome

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Title Journal of Neuro-Ophthalmology, December 2012, Volume 32, Issue 4
Date 2012-12
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s6x383kt
Setname ehsl_novel_jno
ID 227367
Reference URL https://collections.lib.utah.edu/ark:/87278/s6x383kt

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Title Visual Improvement With the Use of Idebenone in the Treatment of Wolfram Syndrome
Creator Bababeygy, Simon R; Wang, Michelle Y; Khaderi, Khizer R; Sadun, Alfredo A
Affiliation Doheny Eye Institute and Department of Ophthalmology, Keck School of Medicine, University of Southern California, Los Angeles, California
Abstract Recently in the Journal of Neuro-Ophthalmology, we discussed the potential efficacy of idebenone, a coen-zyme Q derivative that acts as a carrier in the mitochon-drial electron transport chain, in treating patients with Leber hereditary optic neuropathy (LHON) (1). Within the past 2 decades, Wolfram syndrome (WS) has been mapped to chromosome 4p16.1 (2) and is thought to harbor a mitochondrial genome deletion (3,4) or complex III deficiency (5). The only current treatment for this devastating disease is limited to blood sugar control with lower doses of insulin compared with patients with diabe-tes mellitus (DM). We had the opportunity to treat a patient with WS with idebenone, hoping for improve-ment in visual function. A 21-year-old Romanian man was initially evaluated 5 years previously with progressive bilateral visual and hearing loss and a neurogenic bladder. Surgical history was significant for bilateral cataract extractions with intraocular lens implants at 11 years of age. Medications included insulin and effexor. Family history was significant for DM in both parents, without a history of hearing or visual loss. Genetic testing confirmed the diagnosis of WS with the mutation of the WFS1 Wolframin gene.
Subject Antioxidants; Humans; Male; Optic Atrophy; Optic Disk; Ubiquinone; Visual Fields; Wolfram Syndrome; Young Adult
Format application/pdf
Publication Type Journal Article
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
Setname ehsl_novel_jno
ID 227365
Reference URL https://collections.lib.utah.edu/ark:/87278/s6x383kt/227365
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