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Genetic and Rare Diseases Information Center (GARD)

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Dravet syndrome


Other Names for this Disease

  • Myoclonic epilepsy, severe, of infancy
  • Severe Myoclonic Epilepsy of Infancy
  • SME
  • SMEI
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Your Question

What is the life expectancy for a child with Dravet syndrome? Other main characteristics?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Dravet syndrome?

Dravet syndrome is a severe form of epilepsy. The condition appears during the first year of life as frequent fever-related (febrile) seizures.[1] As the condition progresses, other types of seizures typically occur, including myoclonus and status epilepticus.[1] A family history of either epilepsy or febrile seizures exists in 15 percent to 25 percent of cases.[2] Intellectual development begins to deteriorate around age 2, and affected individuals often have a lack of coordination, poor development of language, hyperactivity, and difficulty relating to others.[1][2] In 30 to 80 percent of cases, Dravet syndrome is caused by changes in the SCN1A gene, which is required for the proper function of brain cells.[3]
Last updated: 6/21/2012

What is the life expectancy for a child with Dravet syndrome?

Little information is available on the long-term prognosis and life expectancy for children with Dravet syndrome.[4] The degree of intellectual impairment appears to correlate with the frequency of seizures.[1] The decline in cognitive function tends to stabilize after age 4.[4] An individual with Dravet syndrome has an 85 percent chance of surviving into adulthood.[4]
Last updated: 12/21/2009

References
Other Names for this Disease
  • Myoclonic epilepsy, severe, of infancy
  • Severe Myoclonic Epilepsy of Infancy
  • SME
  • SMEI
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.