Dravet Syndrome

Definition: Dravet syndrome is a rare, severe, lifelong form of epilepsy that begins in infancy, usually between 6 and 12 months of age. Most cases are caused by a new change in the SCN1A gene. Seizures are often long and frequently triggered by fever or heat. Cannabidiol (Epidiolex) is one of the FDA-approved treatments for Dravet seizures.

What Are the Symptoms of Dravet Syndrome?

It often starts with a long seizure during a fever in an otherwise healthy baby. Over time, children develop several seizure types, including myoclonic (sudden jerks), tonic-clonic, and absence seizures, and some seizures can last long enough to need emergency care. According to the National Institute of Neurological Disorders and Stroke (NINDS), many children also develop developmental delays, intellectual disability, behavioral and movement problems, sleep problems, and autism spectrum disorder.

What Causes Dravet Syndrome?

Most people with Dravet syndrome have a change in the SCN1A gene, which helps control sodium channels that let brain cells fire normally. In most cases the change is new (de novo) rather than inherited from a parent. Dravet was formerly called severe myoclonic epilepsy of infancy (SMEI).

How Is Dravet Syndrome Treated?

There’s no cure, but treatment can reduce seizures. FDA-approved options include cannabidiol (Epidiolex), fenfluramine (Fintepla), and stiripentol (Diacomit). Doctors also use medicines like valproic acid, clobazam, and topiramate, along with the ketogenic diet. Some common seizure medicines that block sodium channels can make Dravet seizures worse, so diagnosis matters. Families usually keep a rescue plan for prolonged seizures.

How Does CBD Help With Dravet Syndrome?

In clinical trials, children with Dravet syndrome who took Epidiolex alongside their usual medicines had significantly fewer convulsive seizures than those on placebo. That evidence applies to pharmaceutical Epidiolex, not to retail CBD products.

Related terms: Epidiolex, Lennox-Gastaut Syndrome, Cannabidiol (CBD), FDA

Sources: NINDS: Dravet Syndrome; FDA: Epidiolex Prescribing Information.

This entry is for general education and isn’t medical advice. Talk to a neurologist about diagnosis and treatment.