Rett’s Disorder - Past and Present
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Introduction
Pervasive Developmental Disorder (PDD)
Key Features:
Delay or loss of appropriate social skills, language, and behavior
Affects many developmental areas, starting early and persisting throughout life
Examples:
Rett’s Disorder, Autistic Disorder, Childhood Disintegrative Disorder, Asperger’s Disorder, Pervasive Developmental Disorder Not Otherwise Specified
History
1966 - Dr. Andreas Rett of Austria observed two females with unusual hand-wringing motions
1983 - Dr. Bengt Hagberg of Sweden published comprehensive review of Rett’s Disorder in an English neurology journal
1984 - First International Rett Syndrome Conference in Vienna
1985 - Dr. Hugo Moser organized first North American International Rett Syndrome Conference in Baltimore, MD; International Rett Syndrome Association established
DSM-IV: Diagnosis of Rett’s Disorder
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
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