Childhood Disintegrative Disorder

Alternate Names
disintegrative psychosis
Heller syndrome
dementia infantilis

Definition of Childhood Disintegrative Disorder

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 signs and symptoms of the condition?

As a child with childhood disintegrative disorder has alterations in thinking skills, he or she usually loses communication skills. The child returns to using nonverbal behaviors, or experiences a significant loss of previously acquired skills in some other area. Examples include:

• loss of social skills
• loss of bowel or bladder control
• loss of expressive language, which is the ability to communicate to others
• loss of receptive language, which is the ability to understand what others are communicating
• loss of motor skills
• lack of play
• failure to develop peer relationships
• impairment in nonverbal behaviors
• delay or lack of spoken language
• inability to initiate or sustain a conversation

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 in boys than girls.

What can be done to prevent the condition?

There is no known prevention for childhood disintegrative disorder.

How is the condition diagnosed?

Childhood disintegrative disorder can only be diagnosed if the symptoms are preceded by at least 2 years of normal development. The disintegration of skills begins before age 10.

Diagnosis is made on the basis of the symptoms the child is having. To meet the definition for this disorder, the child must have lost significant skill in 2 of the following 3 areas: language, social, or adaptive behavior.

What are the long-term effects of the condition?

Childhood disintegrative disorder results in significant impairment in the person's ability to function. Most children who receive this diagnosis will need lifelong assistance of some kind, depending on the severity of the symptoms.

What are the risks to others?

Because childhood disintegrative disorder is not contagious, there are no risks to others.

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 stabilize. Occasionally, limited improvement may be seen.

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 professional.