Childhood Disintegrative Disorder

This rare condition was described many years before autism (Heller, 1908) but has only recently been 'officially' recognized. With CDD children develop autism only after a relatively prolonged period (usually 3 to 4 years) of totally normal development (Volkmar, 1994). This condition differs from autism in the pattern of onset, course, and outcome (Volkmar, 1994).

Criteria and Clinical Features

Both the DSM-IV-TR and ICD-10 list criteria for this condition. Both systems describe a regression of some mixture of language, interest in the social environment, and self-care abilities. This is often accompanied by a dramatic decline in cognitive abilities. The period of acute regression may be characterized by very high levels of anxiety and usually lasts several months. The ultimate clinical picture is one of severe autism and intellectual disabilities, i.e., the clinical presentation (but not the history) is then typical of a child with autism.

History

A special educator in Vienna, Theodore Heller, proposed the term dementia infantilis to describe the condition. Relatively little is known about the condition. The assumption until recently has been that this condition is ALWAYS associated with some specific neuropathological process. However, to there is no evidence to support this. In most cases after even very extensive testing no specific medical cause for the condition is found. As with autism, children who suffer from this condition are at increased risk for seizures.

Etiology/Cause

The etiology is unknown but several lines of evidence suggest that it arises as a result of some form of central nervous system pathology.

Epidemiology

More boys than girls appear to be affected. Childhood disintegrative disorder is perhaps 10 times less common than more strictly defined autism (Volkmar, 1994) and is estimated to occur in between 1-2 children per 100,000.

Case Illustration

John's early history was totally normal. By age 2 he was speaking in sentences, and he made frequent eye contact with his parents, and his older brother. At age 3 1/2 he became extremely anxious, frequently awaking at night terrified. During the day he would cry inconsolably for long periods of time, often repeating the phrase, “it hurts, it hurts, make it go away”. After a month, his parents describe a steep decline in his communication skills. He began to flap his hands, to soil himself, and had no interest in social interaction. Extensive medical examinations were conducted at 2 pediatric hospitals, failing to reveal anything that might explain the regression. At follow-up at age 12 he still was not speaking, apart from an occasional single word, and had been placed in a school for the severely disabled.

Parent Resources

Visit our Regression and CDD Study Page

Visit our CDD Page on Facebook.

Book: When Autism Strikes

Contact

Alexander Westphal, M.D.
alexander.westphal@yale.edu