About 60 percent of people with Wolfram syndrome develop a neurological or psychiatric disorder, most commonly problems with balance and coordination (ataxia), typically beginning in early adulthood. Other neurological problems experienced by people with Wolfram syndrome include irregular breathing caused by the brain’s inability to control breathing (central apnea), loss of the sense of smell, loss of the gag reflex, muscle spasms (myoclonus), seizures, reduced sensation in the lower extremities (peripheral neuropathy), and intellectual impairment. Psychiatric disorders associated with Wolfram syndrome include psychosis, episodes of severe depression, and impulsive and aggressive behavior.

Diabetes Mellitus is typically the first symptom of Wolfram syndrome, usually diagnosed around age 6. Nearly everyone with Wolfram syndrome who develops diabetes mellitus requires insulin replacement therapy. Diabetes mellitus prevents your body from properly using the energy from the food you eat. Diabetes occurs in one of the following situations:

• The pancreas (an organ behind your stomach) produces little insulin or no insulin at all. Insulin is a naturally occurring hormone, produced by the beta cells of the pancreas, which helps the body use sugar for energy.

-Or-

• The pancreas makes insulin, but the insulin made does not work as it should. This condition is called insulin resistance.