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  Rett Syndrome 
  
Reviewed  07-01-2001  

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What is Rett Syndrome?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations
Related NINDS Publications and Information

What is Rett Syndrome?
Rett syndrome is a progressive neurological disorder in which individuals exhibit reduced muscle tone, autistic-like behavior, hand movements consisting mainly of wringing and waving, loss of purposeful use of the hands, diminished ability to express feelings, avoidance of eye contact, a lag in brain and head growth, gait abnormalities, and seizures. Hypotonia (loss of muscle tone) is usually the first symptom. The syndrome affects approximately 1 in every 10,000-15,000 live female births, with symptoms usually appearing in early childhood — between ages 6 and 18 months. Rett syndrome is caused by mutations (structural alterations or defects) in the MECP2 (pronounced meck-pea-two) gene, which is found on the X chromosome. Scientists identified the gene — which is believed to control the functions of several other genes — in 1999.

Is there any treatment?
There is no cure for Rett syndrome; however, there are several treatments options. These include treatments for the orthopedic and learning disabilities and seizures that may occur in individuals with Rett syndrome. Some children may require special nutritional programs to maintain adequate weight.

What is the prognosis?
In spite of the severe impairments that characterize this disorder, the majority of individuals with Rett can be expected to reach adulthood, surviving at least into their 40s. However, the risk of death is increased. Sudden, unexplained death — possibly from brainstem dysfunction with respiratory arrest — often occurs.

What research is being done?
Basic research on Rett syndrome includes studies in the areas of neuropathology, neurochemistry, neuroimaging, nutrition, and genetics. The National Institute of Child Health and Human Development (NICHD) funds research on Rett syndrome.

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 Organizations

International Rett Syndrome Association (IRSA)
9121 Piscataway Road
Suite 2B
Clinton, MD 20735
irsa@rettsyndrome.org
http://www.rettsyndrome.org
Tel: 301-856-3334 800-818-RETT (7388)
Fax: 301-856-3336

National Institute of Child Health and Human Development (NICHD)
National Institutes of Health
Bldg. 31, Rm. 2A32
Bethesda, MD 20892-2425
NICHDClearinghouse@mail.nih.gov
http://www.nichd.nih.gov
Tel: 301-496-5133 800-370-2943

Rett Syndrome Research Foundation (RSRF)
4600 Devitt Drive
Cincinnati, OH 45246
monica@rsrf.org
http://www.rsrf.org
Tel: 513-874-3020
Fax: 513-874-2520

Related NINDS Publications and Information

  • Rett Syndrome Fact Sheet

  • Rett Syndrome fact sheet prepared by the National Institute of Neurological Disorders and Stroke (NINDS).


    NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

    All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.


    Provided by:
    The National Institute of Neurological Disorders and Stroke
    National Institutes of Health
    Bethesda, MD 20892




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