Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy. It appears during the first year of life with frequent febrile seizures – fever-related seizures that, by definition, are rare beyond age 5. Later, other types of seizures typically arise, including myoclonus (involuntary muscle spasms). Status epilepticus – a state of continuous seizure requiring emergency medical care – also may occur. Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others.
In 30 to 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells. Borderline SMEI (SMEB) and another type of infant-onset epilepsy called generalized epilepsy with febrile seizures plus (GEFS+) are caused by defects in the same gene. In GEFS+, febrile seizures may persist beyond age 5.
Seizures in Dravet syndrome are difficult to control, but can be reduced by anticonvulsant drugs. A ketogenic diet, high
in fats and low in carbohydrates, also may be beneficial.
As children with Dravet syndrome get older, their decline in cognitive function stabilizes, and in many, it improves slightly.
However, most teenagers with Dravet syndrome are dependent on caregivers. The degree of cognitive impairment appears to correlate
with the frequency of seizures.
The NINDS conducts and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet
syndrome. Study of the genetic defects responsible for Dravet syndrome and related disorders is expected to lead to the development
of effective drug therapies.
Epilepsy Foundation 8301 Professional Place Landover, MD 20785-7223 postmaster@efa.org http://www.epilepsyfoundation.org Tel: 301-459-3700 800-EFA-1000 (332-1000) Fax: 301-577-2684 |
Dravet.org P.O. Box 66599 Baltimore, MD 21239-6599 info@Dravet.org http://dravet.org Tel: 866-828-1843 |
Intractable Childhood Epilepsy Alliance PO Box 365 6360 Shallowford Road Lewisville, NC 27023 info@ice-epilepsy.org http://www.ice-epilepsy.org Tel: 336-946-1570 Fax: 336-946-1571 |
Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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Last updated September 29, 2011