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Rett's Disorder

Diagnostic Criteria for 299.80 Rett's Disorder

The essential feature of Rett's Disorder is the development of multiple specific deficits following a period of normal functioning after birth.  There is a loss of previously acquired purposeful hand skills before subsequent development of characteristic hand movement resembling hand wringing or hand washing.  Interest in the social environment diminishes in the first few years after the onset of the disorder.  There is also significant impairment in the expressive and receptive language development withn severe psychomotor retardation. 

A.  All of the following:

  1. apparently normal prenatal and perinatal development

  2.  apparently normal psychomotor development through the
       first 5 months after birth

  3.  normal head circumference at birth

B. Onset of all of the following after the period of normal
    development:

  1. deceleration of head growth between ages 5 and 48 months

  2. loss of previously acquired purposeful hand skills between 5
      and 30 months with the subsequent development of
      stereotyped hand movements (e.g., hand-wringing or hand
      washing)

  3. loss of social engagement early in the course ( although often
      social interaction develops later)

  4. appearance of poorly coordinated gait or trunk movements

  5. severely impaired expressive and receptive language
     development with severe psychomotor retardation