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What is Heller Syndrome? Print E-mail
Written by Gary Presant, MD   
Tuesday, 20 October 2009
What is  Heller Syndrome?
A child with childhood disintegrative disorder develops normally for the first 2 years. At 3-4 years of age, the child shows marked deterioration of his or her intellectual, social, and language skills. Childhood disintegrative disorder is a type of pervasive developmental disorder.

What is going on in the body?
The typical child with childhood disintegrative disorder develops normally for the first 2 years. He or she has age-appropriate verbal and nonverbal communication and social relationships. The child's play and adaptive behavior are normal. At about age 3 or 4, the parents may notice that something is different about their child. The child begins to show a loss of previously attained abilities.

What are the causes and risks of the condition?
The cause of childhood disintegrative disorder is unknown. It has, however, been associated with seizure disorders. It appears likely that this condition is the result of some insult to the developing central nervous system. It is more common among males.

What are the treatments for the condition?
Treatment of childhood disintegrative disorder follows that of most other types of pervasive developmental disorder. Parents, teachers, and therapists work together in coordinated efforts to encourage social adjustment and speech development in the child. Positive reinforcement techniques such as offering food for appropriate behavior or language responses have been successful in promoting skill development.

What are the side effects of the treatments?
Side effects vary, depending upon the treatment given.

What happens after treatment for the condition?
Usually the loss of skills reaches a plateau, and the child's abilities stablize. There may be limited improvement, although this is rare.

How is the condition monitored?
A person with childhood disintegrative disorder may require long-term assistance educationally and help establishing independent living in adulthood. Any new or worsening symptoms should be reported to the healthcare provider.


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