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Dravet Syndrome (SMEI)

Dravet Syndrome was first described in 1978 by Dr. Charlotte Dravet, a French epileptologist mentored by Dr. Henri Gastaut at the Centre St. Paul in Marseilles.  In 1981 the syndrome was termed Severe Myoclonic Epilepsy of Infancy (SMEI) and clinical diagnostic criteria were established in 1984.  The International League Against Epilepsy officially recognized SMEI as an epileptic syndrome in 1989, and in 2001, after atypical cases without myoclonic seizures were documented, they adopted the eponym, Dravet Syndrome.  Since that time a mutation on the SCN1A gene has been identified as a genetic marker for the spectrum of genetic, febrile, sodium channel epilepsies, with Dravet Syndrome being the most severe and malignant form.

SMEI is a progressive childhood neurodevelopmental disorder characterized by severe epilepsy that does not respond well to treatment.  Estimates of the prevalence of this rare disorder have ranged from 1:20,000 to 1:40,000 births, though incidence may be far greater as new genetic evidence is discovered.  It occurs more frequently in boys than in girls, but knows no geographic or ethnic boundaries.

* Idea-League Foundation
* Dravet Syndrome Foundation

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(MedPage Today)