DSM-IV

Diagnostic Criteria

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 ages 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

Differential Diagnosis

Normal development

Periods of developmental regression may be observed in normal development, but these are neither as severe or as prolonged as in Rett's Disorder.

Other Pervasive Developmental Disorders

Autistic Disorder

Rett's Disorder differs from Autistic Disorder in its characteristic sex ratio and pattern of deficits. Rett's Disorder has been diagnosed only in females, whereas Autistic Disorder occurs much more frequently in males. In Rett's Disorder, there is a characteristic pattern of head growth deceleration, loss of previously acquired purposeful hand skills, and the appearance of poorly coordinated gait or trunk movements. Particularly during the preschool years, individuals with Rett's Disorder may exhibit difficulties in social interaction similar to those observed in Autistic Disorder, but these tend to be transient.

Childhood Disintegrative Disorder and Asperger's Disorder

Rett's Disorder differs from Childhood Disintegrative Disorder and Asperger's Disorder in its characteristic sex ratio, onset, and pattern of deficits. Rett's Disorder has been diagnosed only in females, whereas Childhood Disintegrative Disorder and Asperger's Disorder appear to be more common in males. The onset of symptoms in Rett's Disorder can begin as early as age 5 months, whereas in Childhood Disintegrative Disorder the period of normal development is typically more prolonged (i.e., at least until age 2 years). In Rett's Disorder, there is a characteristic pattern of head growth deceleration, loss of previously acquired purposeful hand skills, and the appearance of poorly coordinated gait or trunk movements. In contrast to Asperger's Disorder, Rett's disorder is characterized by a severe impairment in expressive and receptive language development.

DSM-5

As of DSM-5, this disorder is no longer part of the DSM.

For more information, see Autism Spectrum Disorder

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