Childhood Disintegrative Disorderhildhood disintegrative disorder (CDD) is classified as a pervasive developmental disorder and is characterized by at least 2 years of normal early development followed by profound loss of previously acquired skills in the areas of cognition, communication, motor control, and bowel and bladder control. Once established, behaviors manifested as a result of CDD are indistinguishable from those of autism. Previously, CDD has been referred to as Heller’s syndrome, dementia infantilis, and disintegrative psychosis (Filipek et al.,).
Etiology of the disorder is unknown; however, marked disintegration of functioning after a normal period of development suggests that the underlying mechanism is organic (e.g., neurobiological disorder or medical condition). Neurologic conditions such as tuberous sclerosis, neurolipidoses, and metachromatic leucodystrophyas have been implicated with the disorder, as have pertussis and measles (Volkmar,). Children with CDD have been shown to have abnormal epileptiform electroencephalograms; however, if seizures occur, they are rarely the first sign. More often than not, seizures occur after the onset of CDD (Malhotra & Gupta,).
CDD is a rare condition with prevalence rates reported to be about 1 per 100,000 children (Malhotra & Gupta,). Because CDD shares a number of features with other pervasive developmental disorders, it is often indistinguishable. Children with CDD have impaired communication (verbal and nonverbal), poor social interactions, and stereotypic behaviors and interests, like children with autism. There is some indication that CDD may be more common in males than females; given how rare the disorder is, however, this remains unclear. Critical to the diagnosis of CDD is a history of normal development up to the age of 2 years followed by a marked decline. This “normal” period of development distinguishes CDD from infantile autism.