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Wolfram syndrome

Disease definition

Wolfram syndrome (WS) also known as DIDMOAD, is a neurodegenerative disorder characterized by type I diabetes mellitus (DM), diabetes insipidus (DI), sensorineural deafness (D), bilateral optical atrophy (OA) and neurological signs. Other related problems are urinary tract atony, ataxia, peripheral neuropathy, psychiatric disorders and/or seizures. 2 types of WS may be distinguished: type 1 and type 2 (WS1 and WS2).

ORPHA:3463

  • Synonym(s):
    • DIDMOAD syndrome
    • Diabetes insipidus-diabetes mellitus-optic atrophy-deafness syndrome
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood, Adolescent, Adult
  • ICD-10: E13.8
  • OMIM: 222300  598500  604928
  • UMLS: C0043207
  • MeSH: D014929
  • GARD: 7898
  • MedDRA: -

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