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Childhood disintegrative disorder


Definition:

Childhood disintegrative disorder is a condition in which children develop normally through age 3 or 4. Then, over a few months, children lose language, motor, social, and other skills that they already learned.

Alternative Names:

Disintegrative psychosis; Heller syndrome

Causes, incidence, and risk factors:

The cause of childhood disintegrative disorder is unknown, but it has been linked to brain and nervous system problems. A child who is affected loses:

  • Communication skills
  • Nonverbal behaviors
  • Skills they had already learned

The condition is similar to autistic disorder (autism).

Symptoms:
  • Delay or lack of spoken language
  • Impairment in nonverbal behaviors
  • Inability to start or maintain a conversation
  • Lack of play
  • Loss of bowel and bladder control
  • Loss of language or communication skills
  • Loss of motor skills
  • Loss of social skills
  • Problems forming relationships with other children and family members
Signs and tests:

The health care provider will determine whether the child has this disorder, or a similar condition such as childhood schizophrenia or pervasive developmental disorder (autism).

The most important sign of childhood disintegrative disorder is the loss of developmental milestones. Generally, the diagnosis is made if the child has lost function in at least two areas of development.

Treatment:

Treatment is the same as for autistic disorder (autism) because the two disorders are similar.

One experimental treatment uses steroid medications to slow the progression of the condition.

Expectations (prognosis):

The outlook for this disorder is poor. Most children with the condition have an impairment similar to that of children with severe autism by age 10.

Calling your health care provider:

Call your provider if your child has any delays in development or starts to lose developmental abilities.

References:

Raviola G, Gosselin GJ, Walter HJ, DeMaso DR. Pervasive developmental disorders and childhood psychosis. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 28.


Review Date: 6/12/2012
Reviewed By: David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc. Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine.

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