Dravet Syndrome

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
National charitable organization dedicated to the welfare of people with epilepsy. Works to ensure that people with seizures are able to participate in all life experiences; to improve how people with epilepsy are perceived, accepted and valued in society; and to promote research for a cure. Offers a Legal Defense Program through a fund.

8301 Professional Place East, Suite 200
Landover, MD 20785-7223
Tel: Landover
Fax: 301-577-2684

Dravet Syndrome Foundation
Dedicated to aggressively raising research funds for Dravet syndrome, a rare and catastrophic form of epilepsy beginning in childhood, and related conditions.

P.O. Box 16536
West Haven, CT 06516
Tel: West Haven

Intractable Childhood Epilepsy Alliance
The Intractable Childhood Epilepsy Alliance is a non-profit 501c3 organization dedicated to improving lives of children affected by intractable epilepsy through evidence-based information, advocacy for appropriate medical treatment including compassionate use and Orphan drug products, promotion of drug development, data collection through patient registries, and funding of research that will lead to a cure for intractable childhood epilepsies.

PO Box 365
6360 Shallowford Road
Lewisville, NC 27023
Tel: Lewisville
Fax: 336-946-1571

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NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

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