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a pervasive developmental disorder affecting the gray matter of the brain, occurring exclusively in females and present from birth; it is characterized by autistic behavior, ataxia, dementia, seizures, and loss of purposeful use of the hands, with cerebral atrophy, mild hyperammonemia, and decreased levels of biogenic amines.
Rett syn·drome(ret), [MIM*312750]
1. a pervasive developmental disorder characterized by the development of several specific deficits after an apparently normal prenatal and perinatal period, including deceleration in head growth, loss of purposeful hand skills with deterioration into stereotypical hand movements, impairment in expressive and receptive language, and significant psychomotor retardation;
2. a DSM diagnosis that is established when the specified criteria are met.
An X-linked dominant neurological disorder that occurs almost exclusively in girls, characterized by autism, ataxia, seizures, hypotonia, and purposeless hand movements.
Rett syn·drome(ret sin'drōm)
A progressive syndrome of autism, dementia, ataxia, and purposeless hand movements; associated with hyperammonemia, principally in girls.
Rett syndromeA brain disorder affecting only girls and, from the age of 9–36 months, causing progressive loss of recently acquired skills, such as speech and walking, repetitive writhing (athetoid) movements, epileptic seizures, AUTISM and gradual disablement. Brain examination shows a significant reduction in the profusion of the dendritic trees in layers 3 and 5 of the frontal and inferior temporal cortices. The visual cortex remains unaffected. The cause remains obscure but is thought to be the result of a fresh mutation rather than an inherited genetic defect. (First described in 1966 by the Austrian physician Andreas Rett).
Rett,Andreas, Austrian physician, 1924–.
Rett syndrome - a progressive syndrome of autism, dementia, ataxia, and purposeless hand movements.