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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

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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