Other Names for this Disease
- Myoclonic epilepsy, severe, of infancy
- Severe Myoclonic Epilepsy of Infancy
- Severe myoclonus epilepsy of infancy
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Your QuestionWhat is the life expectancy for a child with Dravet syndrome? Other main characteristics?
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Dravet syndrome is a severe form of epilepsy. The condition appears during the first year of life as frequent fever-related (febrile) seizures. As the condition progresses, other types of seizures typically occur, including myoclonus and status epilepticus. A family history of either epilepsy or febrile seizures exists in 15 percent to 25 percent of cases. 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. 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.
Last updated: 6/21/2012
Little information is available on the long-term prognosis and life expectancy for children with Dravet syndrome. The degree of intellectual impairment appears to correlate with the frequency of seizures. The decline in cognitive function tends to stabilize after age 4. An individual with Dravet syndrome has an 85 percent chance of surviving into adulthood.
Last updated: 12/21/2009
- NINDS Dravet Syndrome Information Page. National Institute of Neurological Disorders and Stroke. September 29, 2011 ; http://www.ninds.nih.gov/disorders/dravet_syndrome/dravet_syndrome.htm. Accessed 6/20/2012.
- Infants and Epilepsy. Epilepsy Foundation. 2012; http://www.epilepsyfoundation.org/aboutepilepsy/syndromes/rareepilepsysyndromes/severe-myoclonic-epilepsy-of-infancy.cfm. Accessed 6/20/2012.
- Severe Myoclonic Epilepsy of Infancy. Online Mendelian Inheritance in Man (OMIM). February 2, 2012 ; http://omim.org/entry/607208. Accessed 6/20/2012.
- Dravet syndrome. IDEA League Web site. http://www.idea-league.org/dravet-syndrome. Accessed 12/21/2009.