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Genetic and Rare Diseases Information Center (GARD)

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Wolfram syndrome

Other Names for this Disease
  • Diabetes insipidus and mellitus with optic atrophy and deafness
  • DIDMOAD syndrome
  • WFS
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What are the signs and symptoms of Wolfram syndrome?

Wolfram syndrome is characterized by diabetes insipidus, a condition in which the kidneys cannot conserve water; childhood-onset diabetes mellitus, which results from the improper control of glucose due to the lack of insulin; a gradual loss of vision caused by optic atrophy, in which the nerve that connects the eye to the brain wastes away; and deafness.[1] Other related problems include lack of muscle tone within the urinary tract, ataxia, peripheral neuropathy, dementia, psychiatric disorders (i.e. severe depression, psychosis, compulsive verbal and physical aggression), and/or seizures.[2]

The following complications have also been noted: [2] 
Last updated: 9/8/2011

  1. WFS1. Genetics Home Reference (GHR). 2006; Accessed 9/7/2011.
  2. Martinez VN. Wolfram syndrome. Orphanet. 2007; Accessed 9/7/2011.