Managing Dravet syndrome can feel like solving a puzzle, but there are many tools that can help. Dravet syndrome is a rare, severe form of epilepsy that begins in infancy. It’s characterized by frequent and prolonged seizures, which are often triggered by fever. While there’s no cure, a mix of treatments — including medications, diet changes, and even nerve stimulation — can make a big difference in reducing seizure frequency and severity.

Finding the right approach takes time, since what works for one person may not work for another. Some treatments are used daily to prevent seizures, while others are there for emergencies. In this article, we’ll explore the different options available and how they can help improve life for those with Dravet syndrome.

A Word About Off-Label Treatments

Some of the treatments mentioned in this article are specifically approved by the U.S. Food and Drug Administration (FDA) for treating Dravet syndrome, while others may be used “off-label.” Off-label use refers to a doctor prescribing a medication or dosage for a condition that isn’t specially listed on the drug’s FDA-approved label. This is common for rare diseases like Dravet syndrome, where research may be limited. This doesn’t mean the drug has never been successfully used to treat Dravet, but rather that it hasn’t gone through the formal FDA approval process for Dravet syndrome. While off-label use is legal and often necessary, it should be based on sound medical evidence.

Because Dravet syndrome is a rare and complex condition, treatment often involves a mix of FDA-approved options and off-label approaches to find the best seizure control. New treatments are being studied all the time, giving families more hope for better outcomes. Always talk to your doctor before starting or changing any treatment to make sure it’s safe and appropriate for your or your loved one’s specific needs.

Dravet Syndrome Treatments

People with Dravet syndrome experience a wide range of seizure types and severity. For this reason, treatment options vary. Although there is no cure for Dravet syndrome, treatment is aimed at finding the best combination of antiepileptic drugs to treat chronic seizures. Usually multiple seizure medications are needed to treat the variety of seizure types that can happen with this syndrome.​

First-Line Treatments for Dravet Syndrome

Anti-seizure medications are generally the first therapies prescribed for people with Dravet syndrome. Valproic acid is often used as a first-line treatment, particularly for the generalized tonic-clonic seizures and focal seizures that can be associated with fever in Dravet syndrome. Clobazam (sold as Onfi and Sympazan) is also effective for a broad range of seizure types. Valproic acid and clobazam are often used in combination.

While not a replacement for preventive treatment, rescue medicines can be used to treat clusters of seizures or seizures that last longer than normal and avoid emergencies.

Second-Line Treatments for Dravet Syndrome

If first-line treatments are not effective, a neurologist may prescribe second-line medications, including topiramate (Eprontia, Qudexy XR, Topamax, Trokendi XR). It works through multiple mechanisms to reduce seizure activity.

In 2018, the U.S. Food and Drug Administration (FDA) approved the anti-seizure drug stiripentol (Diacomit) for use with clobazam in children 2 years and older who have drug-resistant Dravet seizures. As many as 71 percent of children treated with stiripentol in a research study had a greater than 50 percent decrease in seizures, compared to 5 percent treated with placebo (a substance with no effect).

The ketogenic diet is considered a second-line treatment for Dravet syndrome. This high-fat, low-carbohydrate diet shifts the body’s energy source from glucose (sugar) to ketones (molecules produced by the liver when the body burns fat for energy). Some studies have shown that 63 percent of children with Dravet syndrome achieve at least a 50 percent reduction in seizures after three months, dropping to 60 percent at six months and 47 percent at 12 months. Studies have found that 10 percent to 15 percent of children become seizure-free on the diet. There is also some evidence that a ketogenic diet is linked to improvements in behavior and thinking for children with Dravet syndrome.

It’s important to know that the ketogenic diet is a highly restrictive diet that requires meticulous planning and adherence. It’s often challenging for families to maintain long term. If you are considering a ketogenic diet to treat Dravet syndrome, work closely with your neurologist, a dietitian, and others on your healthcare team.

Third-Line Treatments for Dravet Syndrome

Anti-seizure and anticonvulsant medications may be prescribed when seizures remain uncontrolled after trying first- and second-line treatments. These medications may include:

• Clonazepam (Klonopin)
• Ethosuximide (Zarontin)
• Levetiracetam (Keppra, Elepsia XR, Roweepra, Spritam)
• Zonisamide (Zonegran, Zonisade)

Vagus Nerve Stimulation

Vagus nerve stimulation (VNS) is FDA-approved as an add-on therapy for adults and children aged 4 and up. Nerve stimulation is used to treat focal or partial seizures that do not respond to medications. (Dravet is primarily characterized by generalized seizures, but focal seizures can occur as well.) A device implanted in the chest sends electrical signals through the vagus nerve to help control seizures.

A 2017 review of 13 studies including 68 people with Dravet syndrome reported that 53 percent of people treated with nerve stimulation showed a minimum 50 percent reduction in seizures.

Cannabidiol

An oral form of purified cannabidiol (CBD), Epidiolex is FDA-approved to control Dravet-related seizures. In a 2017 clinical trial of 120 young adults and children with Dravet syndrome, 43 percent of those treated with cannabidiol had their seizures reduced by more than half — compared to 27 percent of those treated with placebo.

Learn more about how CBD is used to treat severe epilepsy.

Fenfluramine

Fenfluramine (Fintepla) is an oral medication approved by the FDA in 2020 for treating Dravet syndrome seizures. The drug’s effectiveness was determined following two clinical trials that measured the number of convulsive seizures experienced by trial participants with Dravet syndrome between ages 2 and 18. The studies demonstrated a 66.8 percent median reduction in convulsive seizure frequency among study participants taking fenfluramine.

Rescue Medications for Dravet Syndrome

Rescue medicines can be used to treat clusters of seizures or seizures that last longer than normal. These are not a replacement for preventive treatment, which is taken on a regular schedule. Rescue treatments work quickly in the brain to end seizures and avoid emergencies.

The most commonly prescribed rescue medicines are fast-acting benzodiazepines such as diazepam (Valium), lorazepam (Ativan), and midazolam. These rescue medications are given orally (by mouth), sublingually (under the tongue), buccally (between the cheek and gum), or nasally (sprayed into a nostril). Diastat, the rectal form of diazepam, is most often prescribed for children.

It’s essential to make a detailed seizure action plan with your neurologist, outlining when and how to administer rescue medications. The Epilepsy Foundation offers information and tools for creating a seizure response plan.

Treatments To Avoid With Dravet Syndrome

Certain common anticonvulsant drugs can worsen seizures for some people with Dravet syndrome. They include sodium channel blockers such as:

• Carbamazepine (Tegretol)
• Eslicarbazepine (Aptiom)
• Lamotrigine (Lamictal)
• Phenytoin (Dilantin)
• Oxcarbazepine (Trileptal)
• Vigabatrin (Sabril)

Ask Your Doctor About Your Options

While managing Dravet syndrome can be challenging, ongoing research and new treatment options continue to bring hope. With the right combination of therapies and a strong support system, many families find ways to improve seizure control and enhance quality of life. Be sure to work closely with your doctor to explore the best treatment plan for you or your loved one — you’re not alone in this journey.

Talk To Others Who Understand

On MyEpilepsyTeam, the social network for people living with epilepsy and their loved ones, more than 124,000 members come together to offer each other support and advice.

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