Alternative Names disintegrative psychosis, Heller syndrome, dementia infantilis
Definition 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 behaviour 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 behaviours, 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 behaviours
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 among males.
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. The child must have lost significant skill in 2 of the following areas: language, social, or adaptive behaviour.
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? There are no risks to others, as childhood disintegrative disorder is not contagious.
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 behaviour 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 stabilise. 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 doctor.
Author: Reviewer: eknowhow Medical Review Panel Editor: Dr John Hearne Last Updated: 9/1/2005 Contributors Potential conflict of interest information for reviewers available on request
