Seyhmus Ari, Uğur Keklíkçí, Ihsan Caça, Kaan Unlü, Hasan Kayabaşi
{"title":"Wolfram syndrome: case report and review of the literature.","authors":"Seyhmus Ari, Uğur Keklíkçí, Ihsan Caça, Kaan Unlü, Hasan Kayabaşi","doi":"10.1007/s12019-007-0007-z","DOIUrl":null,"url":null,"abstract":"<p><p>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 a sufficient criterion 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.</p>","PeriodicalId":75729,"journal":{"name":"Comprehensive therapy","volume":"33 1","pages":"18-20"},"PeriodicalIF":0.0000,"publicationDate":"2007-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s12019-007-0007-z","citationCount":"19","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Comprehensive therapy","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/s12019-007-0007-z","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 19
Abstract
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 a sufficient criterion 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.