Wolfram syndrome is a rare but serious genetic disorder that progressively destroys the nervous system and leads to other abnormalities in the body. It occurs when a genetic mutation interferes with the production of wolframin, a protein required for nerve cells to function normally.

Wolfram syndrome is also called DIDMOAD, an acronym for its primary symptoms:

• Diabetes insipidus (a hormonal disorder causing excess urination)
  
  
• Onset of insulin-dependent diabetes mellitus (an impaired ability to use glucose) before age 15
  
  
• Optic atrophy (nerve damage causing vision problems)
  
  
• Deafness

Urinary tract, respiratory and seizure disorders and psychiatric problems, such as depression, are some of the secondary symptoms that often complicate the syndrome and its treatment. These symptoms are usually first detected in childhood beginning with insulin-dependent diabetes mellitus, although the sequence of symptoms varies among individuals. Eventually, the symptoms can become life-threatening.

Physicians first look for other possible explanations for symptoms. However, Wolfram syndrome is suggested if insulin-dependent diabetes mellitus and optic nerve damage leading to blindness occur by the age of 15. Genetic testing can confirm the presence of the gene mutation but not the condition.

There is no known prevention or cure for Wolfram syndrome. Treatment revolves around relieving symptoms and may include:

• Insulin therapy for diabetes mellitus
• Antidiuretic medication for diabetes insipidus
• Antidepressants for depression
• Anticonvulsant medication for seizures

The gene for Wolfram syndrome is recessive, which means a child with this disorder received the gene from each parent. The parents of patients have a 25 percent chance of having another child with Wolfram syndrome with each consecutive pregnancy. Wolfram syndrome affects males and females equally.