Wolfram syndrome with childhood glaucoma: A rare case report with review of literature

Wolfram syndrome (WFS) is a rare neurodegenerative disorder characterized by young-onset diabetes mellitus, central diabetes insipidus, optic nerve atrophy, and hearing loss. The ophthalmic association of this order has been limited to optic nerve atrophy. We report here a case of WFS and its diseas...

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Main Authors: Divya Kesarwani, Gunjan Jain, Sarswati, Rachna Agarwal, Kumudini Sharma, Vaibhav Kumar Jain
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2020-01-01
Series:Journal of Clinical Ophthalmology and Research
Subjects:
Online Access:http://www.jcor.in/article.asp?issn=2320-3897;year=2020;volume=8;issue=3;spage=122;epage=124;aulast=Kesarwani
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author Divya Kesarwani
Gunjan Jain
Sarswati
Rachna Agarwal
Kumudini Sharma
Vaibhav Kumar Jain
author_facet Divya Kesarwani
Gunjan Jain
Sarswati
Rachna Agarwal
Kumudini Sharma
Vaibhav Kumar Jain
author_sort Divya Kesarwani
collection DOAJ
description Wolfram syndrome (WFS) is a rare neurodegenerative disorder characterized by young-onset diabetes mellitus, central diabetes insipidus, optic nerve atrophy, and hearing loss. The ophthalmic association of this order has been limited to optic nerve atrophy. We report here a case of WFS and its disease course with childhood glaucoma as a rare ophthalmic manifestation which has been reported just once earlier. The patient had young-onset diabetes mellitus (insulin dependent), diabetes insipidus, and optic-disc pallor suggestive of partial optic atrophy. However, hearing assessment was normal at the time of presentation and in due course of disease. Glaucoma is one of the rare possible ocular manifestations of WFS which should be highly considered while evaluating patient with this disorder.
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spelling doaj.art-f9b003aa5ca943d5acf4ef98c7bc10912022-12-21T17:14:55ZengWolters Kluwer Medknow PublicationsJournal of Clinical Ophthalmology and Research2320-38972020-01-018312212410.4103/jcor.jcor_26_20Wolfram syndrome with childhood glaucoma: A rare case report with review of literatureDivya KesarwaniGunjan JainSarswatiRachna AgarwalKumudini SharmaVaibhav Kumar JainWolfram syndrome (WFS) is a rare neurodegenerative disorder characterized by young-onset diabetes mellitus, central diabetes insipidus, optic nerve atrophy, and hearing loss. The ophthalmic association of this order has been limited to optic nerve atrophy. We report here a case of WFS and its disease course with childhood glaucoma as a rare ophthalmic manifestation which has been reported just once earlier. The patient had young-onset diabetes mellitus (insulin dependent), diabetes insipidus, and optic-disc pallor suggestive of partial optic atrophy. However, hearing assessment was normal at the time of presentation and in due course of disease. Glaucoma is one of the rare possible ocular manifestations of WFS which should be highly considered while evaluating patient with this disorder.http://www.jcor.in/article.asp?issn=2320-3897;year=2020;volume=8;issue=3;spage=122;epage=124;aulast=Kesarwanidiabetes insipidusdiabetes mellitusdidmoad syndromeglaucomaoptic atrophywolfram syndrome
spellingShingle Divya Kesarwani
Gunjan Jain
Sarswati
Rachna Agarwal
Kumudini Sharma
Vaibhav Kumar Jain
Wolfram syndrome with childhood glaucoma: A rare case report with review of literature
Journal of Clinical Ophthalmology and Research
diabetes insipidus
diabetes mellitus
didmoad syndrome
glaucoma
optic atrophy
wolfram syndrome
title Wolfram syndrome with childhood glaucoma: A rare case report with review of literature
title_full Wolfram syndrome with childhood glaucoma: A rare case report with review of literature
title_fullStr Wolfram syndrome with childhood glaucoma: A rare case report with review of literature
title_full_unstemmed Wolfram syndrome with childhood glaucoma: A rare case report with review of literature
title_short Wolfram syndrome with childhood glaucoma: A rare case report with review of literature
title_sort wolfram syndrome with childhood glaucoma a rare case report with review of literature
topic diabetes insipidus
diabetes mellitus
didmoad syndrome
glaucoma
optic atrophy
wolfram syndrome
url http://www.jcor.in/article.asp?issn=2320-3897;year=2020;volume=8;issue=3;spage=122;epage=124;aulast=Kesarwani
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