What is Dravet syndrome?

Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy that appears during the first year of life, with frequent febrile seizures-fever-related seizures that, by definition, are rare beyond age five. 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 five.

This information is based on source information from the National Institute of Neurological Disorders and Stroke.

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Important: This content reflects information from various individuals and organizations and may offer alternative or opposing points of view. It should not be used for medical advice, diagnosis or treatment. As always, you should consult with your healthcare provider about your specific health needs.