Disorders A - Z:   A    B   C    D    E    F    G    H    I    J    K    L    M    N    O    P    Q    R    S    T    U    V    W    X    Y    Z

Skip secondary menu

NINDS Dravet Syndrome Information Page

Synonym(s):   Severe Myoclonic Epilepsy of Infancy (SMEI)

Table of Contents (click to jump to sections)


Listen to this page using ReadSpeaker


What is 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.

Is there any treatment?

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. 

What is the prognosis?

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.

What research is being done?

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.

NIH Patient Recruitment for Dravet Syndrome Clinical Trials

Organizations

Column1 Column2
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



NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last updated September 29, 2011