External Ocular Manifestation of Wolfram Syndrome

AUTHORS

Morteza Mehdizadeh 1 , * , M Mosallaei 2 , A Borhani Haghghi 2

1 Assistant professor of Department of Ophthalmology, Khalili Hospital, School of Medicine, Shiraz University of Medical Sciences, [email protected], Fars, Iran

2 Department of Ophthalmology, Khalili Hospital, School of Medicine, Shiraz University of Medical Sciences, Fars, Iran

How to Cite: Mehdizadeh M, Mosallaei M, Borhani Haghghi A. External Ocular Manifestation of Wolfram Syndrome, Iran Red Crescent Med J. Online ahead of Print ; 11(2):210-212.

ARTICLE INFORMATION

Iranian Red Crescent Medical Journal: 11 (2); 210-212
Article Type: Case Report
Received: May 17, 2008
Accepted: November 9, 2008

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Abstract

Wolfram syndrome is a neurodegenerative disorder usually diagnosed in childhood. The ocular manifestation of wolfram syndrome is mainly characterized by bilateral optic atrophy; however, pigmentary retinopathy has been reported in some cases. We present a case with new external ocular manifestation of wolfram syndrome not previously reported in the literature. A 22-year-old retarded man clinically diagnosed as having wolfram syndrome presented with ocular discomfort in his both eyes for a 4-year period, being aggravated recently. On examination, he was emmetrope with best corrected visual acuity of about two meter count finger in each eye. Pupillary response was sluggish but equal in both eyes. Ocular motility was normal and there was no considerable sign of nystagmus; however, there were disturbed blinking reflex, lagophthalmus, and poor bell's phenomenon. Slit lamp examination showed bilateral symmetrical saucer-like depression of the corneal surface adjacent to the limbos that is known to be deled formation. The result of Schemer test was out of the normal limit, in favor of dry eye. So, dry eye due to blinking disturbances in patients with wolfram syndrome as a neurodegenerative disorder may lead to external ocular manifestations, requiring conservative treatments.

Keywords

Wolfram syndrome Neurodegenerative disorder Dry eye Dellen formation

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