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Medline NLM definition: Dravet syndrome (DS), also known as severe myoclonic epilepsy of infancy (SMEI), is one of the rare early childhood intractable epileptic encephalopathies associated with pleomorphic seizure activity, cognitive decline, motor, and behavioral abnormalities. The convulsive seizure is the most common type seen in DS. After the first episode of seizure-like activity, behavioral disorders and cognitive decline are progressive and long-lasting. The most common etiology identified in patients with DS is a de-novo genetic mutation alpha-1 subunit of voltage-gated calcium channel gene (SCN1A). DS is diagnosed clinically and if unclear, genetic testing is recommended. DS treatment options include anti-epileptic drugs and cannabinoids; ketogenic diet therapy and surgical options such as the deep brain and vagal nerve stimulation. Due to drug-refractory epilepsy in DS, many more therapies are being investigated to increase the longevity of patients.
Dravet Syndrome : factsheet from NINDS, the National Institute of Neurological Disorders and Stroke (US site).
Dravet Syndrome: information from NORD, the National Organization for Rare Disorders (US site).
See also our page of links for Epilepsy.
Support Groups
Dravet Syndrome UK - Dravet Syndrome UK was created in 2008 by a group of parents who came together looking for support, resources and information relating to a rare, little known neurological condition called Dravet Syndrome. Dravet Syndrome Foundation. The mission of Dravet Syndrome Foundation (DSF) is to aggressively raise funds for Dravet syndrome and related epilepsies; to support and fund research; increase awareness; and to provide support to affected individuals and families.
Dravet Syndrome Foundation - The mission of Dravet Syndrome Foundation (DSF) is to aggressively raise funds for Dravet syndrome and related epilepsies; to support and fund research; increase awareness; and to provide support to affected individuals and families.