Childhood disintegrative disorder

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Causes

By Mayo Clinic staff

There's no known cause of childhood disintegrative disorder, also known as Heller's syndrome. Most experts agree that there's likely a genetic basis for autism spectrum disorders. The theory is that an abnormal gene is switched on in the early stages of development, before birth, and that this gene affects other genes that coordinate a child's brain development. Environmental exposures, such as to a toxin or infection, may contribute to these effects.

It's also possible that an autoimmune response may play a role in the development of childhood disintegrative disorder. In an autoimmune response, your body's immune system perceives normal body components as foreign and attacks them.

Comprehensive medical and neurological examinations in children diagnosed with childhood disintegrative disorder seldom uncover an underlying medical or neurological cause. Although the occurrence of epilepsy is higher in children with childhood disintegrative disorder, experts don't know whether epilepsy plays a role in causing the disorder.

Childhood disintegrative disorder has also been associated with:

  • Tuberous sclerosis. In this condition, noncancerous (benign) tumors grow in the brain.
  • Lipid storage diseases. In this rare group of inherited metabolic disorders, a toxic buildup of excess fats (lipids) occurs in the brain and nervous system.
  • Subacute sclerosing panencephalitis. This chronic infection of the brain is caused by a form of the measles virus that results in brain inflammation and the death of nerve cells.

Further studies are needed to investigate the causes of childhood disintegrative disorder, but this is difficult due to the limited number of children diagnosed with the disorder.

DS00801

Sept. 16, 2008

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