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J Autism Dev Disord. 1991 Dec;21(4):381-406.

Rett syndrome: a review of current knowledge.

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University of Illinois, College of Education, Chicago 60680.


Rett syndrome was first described in 1966 by Andreas Rett. To date, this syndrome has been reported only to afflict females. The disorder is characterized by a progressive loss of cognitive and motor skills as well as the development of stereotypic hand movements, occurring after an apparently normal 6 to 18 months of development. Although Rett syndrome is thought to afflict as many as 10,000 girls in the United States, fewer than 1,200 have been identified thus far. A lack of awareness of this disorder is thought to play a critical role in the failure to differentially diagnose this syndrome. The present article presents a review of our current knowledge concerning this disorder. Information is provided related to the clinical manifestations, etiology, prevalence, pathogenesis, and treatment of the Rett syndrome.

[Indexed for MEDLINE]

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