Childhood disintegrative disorder

Introduction
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By Eve Spratt MD and Mari Asper MD

Childhood disintegrative disorder is also known as or subsumes Dementia infantalis, Disintegrative psychosis of childhood, and Childhood onset pervasive developmental disorder. -ed.

Childhood disintegrative disorder is a rare condition that mimics autism but develops after 2 years of age. The ICD-9, ICD-10, and DSM-IV definitions are essentially the same and include apparent normal development up to the age of at least 2 years followed by very marked loss of age-appropriate verbal and nonverbal communication, social relationships, play, and adaptive behavior. Under the proposed revisions to the upcoming DSM-V, the diagnosis of childhood disintegrative disorder will be removed and patients will instead be classified under autism spectrum disorder.

Key Points

  • Childhood disintegrative disorder is a lifelong pervasive developmental disorder that closely resembles autistic disorder.
  • It is characterized by normal development until at least 2 years of age followed by severe regression of previously acquired skills.
  • Loss of language skills is a salient feature, but functional decline is also observed in social skills, bowel or bladder control, play, and motor skills.
  • Etiology is unknown.
  • Prognosis is usually poor.
  • Mental retardation and seizures are common comorbidities.