Wolfram syndrome: case report and review of the literature.
Wolfram syndrome (WFS) is a rare diffuse neurodegenerative disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, deafness, and a wide variety of central nervous system abnormalities. Insulin-dependent diabetes mellitus with optic nerve atrophy is sufficient criteria for the diagnosis. WFS is a devastating disease for the patients and their families. This study emphasizes the need for careful evaluation of cases having insulin-dependent diabetes mellitus and optic atrophy.
Ari
Seyhmus
S
Department of Opthalmology, Diyarbakir State Hospital, Diyarbakir, Turkey. sari@dicle.edu.tr
Keklíkçí
Uğur
U
Caça
Ihsan
I
Unlü
Kaan
K
Kayabaşi
Hasan
H
D000293:Adolescent; D003638:Deafness / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D003920:Diabetes Mellitus / Q000175:diagnosis / Q000473:pathology; D005260:Female; D006801:Humans; D009896:Optic Atrophy / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D014929:Wolfram Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology