Childhood disintegrative disorder

Answer 1

Definition

Childhood disintegrative disorder is a condition occurring in 3- and 4-year-olds who have developed normally to age 2. Over several months, a child with this disorder will deteriorate in intellectual, social, and language functioning from previously normal behavior.

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 neurological problems. An affected child shows a loss of communication skills, has regression in nonverbal behaviors, and significant loss of previously acquired skills. The condition is similar to autistic disorder (autism).

Symptoms

• Loss of social skills
• Loss of bowel and bladder control
• Loss of expressive or receptive language
• Loss of motor skills
• Lack of play
• Failure to develop peer relationships
• Impairment in nonverbal behaviors
• Delay or lack of spoken language
• Inability to start or sustain a conversation

Signs and tests

This disorder must be differentiated from both childhood schizophrenia and pervasive developmental disorder (autism).

The most important signs of childhood disintegrative disorder are loss of developmental milestones. The child tends to have normal development through age 3 to 4, and then over a few months undergoes a gradual loss of previously established abilities (e.g., language, motor, or social skills). Generally, the diagnosis is made with a loss of functioning in at least two areas described above.

Treatment

Treatment is the same for autistic disorder (autism) because of the similarity in the two disorders.

Expectations (prognosis)

Unfortunately, the prognosis for this disorder is limited. The loss of functioning will likely be permanent. However, to some degree, behaviors can be modified.

Calling your health care provider

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

Answer 2

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

Shah PE, Dalton R, Boris NW. Pervasive developmental disorders and childhood psychosis. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 29.

Reviewed By

Review Date: 05/13/2010

Benjamin W. Van Voorhees, MD, MPH, Assistant Professor of Medicine, Pediatrics and Psychology, The University of Chicago, Chicago, IL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.