Dravet syndrome treatment
Fact-checked by Dravet syndrome is a rare form of epilepsy usually marked by infantile-onset seizures, along with a range of cognitive, behavioral, and motor symptoms. Each person responds differently to Dravet syndrome treatment, so individualized care is essential.
A Dravet syndrome treatment plan may include medications, non-drug approaches, supportive therapies, and preparation for emergencies.
Early use of Dravet syndrome medications may improve seizure control, long-term outcomes, and quality of life, making prompt and accurate diagnosis critical.
Goals of treatment
Even with treatment, people with Dravet syndrome rarely become entirely seizure-free. Instead, the main goals of Dravet syndrome management include:
- reducing how often seizures occur and how severe they are
- preventing prolonged seizures
- managing symptoms that affect thinking, behavior, and movement
- minimizing medication side effects and improving quality of life
Effective Dravet syndrome seizure control can help reduce the risk of life-threatening complications, including sudden unexpected death in epilepsy (SUDEP), a leading cause of early mortality in people with Dravet syndrome.
Medications
Daily anti-seizure medications are a cornerstone of Dravet syndrome treatment, helping reduce how often seizures occur. These medications are usually taken by mouth as tablets, capsules, or liquids.
There are several Dravet syndrome therapy options, and each works in a different way to help regulate the abnormal brain activity that causes seizures. The specific medications depend on seizure type, response to previous treatments, and other individual needs.
Recommended medications
According to international treatment guidelines, valproate is typically recommended as the first-line medication for Dravet syndrome. In the U.S., it is available in several forms, including divalproex sodium (Depakote and generics) and valproic acid.
If seizures are not adequately controlled, doctors may recommend adding or switching to other anti-seizure medications for Dravet syndrome, such as:
- clobazam (sold as Onfi and generics)
- Diacomit (stiripentol)
- fenfluramine (sold as Fintepla and generics)
- Epidiolex (pharmaceutical-grade cannabidiol)
- topiramate (sold as Topamax and others)
Clobazam, Diacomit, and fenfluramine are commonly used as second-line treatments. Epidiolex may be used as a third-line option, and topiramate is sometimes used as a fourth-line treatment. Other anti-seizure medications may be used as a fourth-line option in certain situations, including:
- clonazepam (sold as Klonopin and generics)
- ethosuximide (sold as Zarontin and generics)
- levetiracetam (sold as Keppra and others)
- zonisamide (sold as Zonegran and others)
While these medications all have U.S. approval for treating seizures, only Diacomit, fenfluramine, and Epidiolex have specific U.S. approval for treating Dravet syndrome. Certain medications may still be prescribed based on individual seizure type and patient needs.
Medications to avoid
Sodium channel blockers, a class of epilepsy medications, can worsen seizures in many people with Dravet syndrome. Doctors typically recommend avoiding these medications in Dravet syndrome treatment, including:
- carbamazepine (sold as Tegretol and others)
- lamotrigine (sold as Lamictal and others)
- oxcarbazepine (sold as Trileptal and others)
- phenytoin (sold as Dilantin and others)
Experimental medications
Ongoing Dravet syndrome clinical trials are evaluating experimental medications that may offer new treatment options. Some in the more advanced stages of development include:
- bexicaserin (LP352)
- EPX-100
- relutrigine (PRAX-562)
- zorevunersen (STK-001)
Some investigational therapies — including bexicaserin, EPX-100, and relutrigine — are anti-seizure medications designed to control abnormal brain activity through new mechanisms. Zorevunersen and other early-stage therapies are genetic treatments designed to address the underlying causes of Dravet syndrome.
Non-medication approaches
Non-drug treatments for Dravet syndrome may supplement medication for some patients, particularly when seizures are not fully controlled with standard treatments. These can include:
- ketogenic diet: a high-fat, low-carbohydrate, limited protein diet
- vagus nerve stimulation: a device implanted in the chest that sends electrical signals through the vagus nerve, which runs from the brain to the chest and torso
Research suggests that the ketogenic diet for Dravet syndrome may help reduce seizures in some patients by altering how the brain uses energy. However, it isn’t typically recommended early in the disease course.
Vagus nerve stimulation in Dravet syndrome is typically used as an add-on therapy when medications do not provide adequate seizure control. It is believed to help regulate the abnormal brain signaling patterns that cause seizures.
Patients are also encouraged to avoid known seizure triggers whenever possible.
Emergency management
Despite routine treatment, many people with Dravet syndrome continue to experience seizures, including status epilepticus, a medical emergency involving seizures lasting longer than five minutes or repeated seizures without full recovery between them.
Caregivers should immediately administer treatment and seek emergency care for Dravet seizures that are severe, prolonged, or do not stop on their own. Families should always have rescue medication available to help stop seizures as quickly as possible.
First-line rescue medications for Dravet syndrome belong to a class called benzodiazepines and may include:
- clonazepam
- diazepam (sold as Diastat and others)
- lorazepam (sold as Ativan and generics)
- midazolam (sold as Nayzilam and generics)
These medications may be given at home as nasal sprays, dissolving tablets, or rectal gels. In hospital settings, they may be given intravenously (into a vein).
If the seizure continues after benzodiazepines, doctors may give additional intravenous anti-seizure medications, such as:
- valproic acid
- levetiracetam
- phenytoin
Although phenytoin is not recommended for routine treatment of Dravet syndrome, it may sometimes be used in emergency situations.
Supportive care
Supportive care for Dravet syndrome helps manage symptoms that affect development, movement, and daily functioning, and may include:
- physical therapy: to improve strength, balance, and walking ability
- occupational therapy: to improve daily living skills and independence
- speech therapy: to improve communication and address feeding or swallowing challenges
- behavioral or play therapy: to help children understand their world, manage emotions, and address behavioral issues
Many patients require additional forms of developmental support throughout their lives, such as specialized education plans.
Organizations such as the Dravet Syndrome Foundation offer Dravet syndrome caregiver resources to support families and help guide treatment decisions.
Planning for the future
Patients and caregivers should expect treatment plans to change over time as symptoms evolve. Regular follow-up care helps ensure treatment remains effective and appropriate.
Other future considerations that may make living with Dravet syndrome easier include:
- working with healthcare providers to develop an emergency seizure management plan
- implementing seizure safety precautions at home and school
- requesting accommodations to make school or other daily activities more accessible
- creating strategies to cope with behavioral challenges
- developing social and emotional support networks
- planning for long-term care during adulthood
Families might also consider participating in Dravet syndrome clinical trials, which can give individuals access to new therapies that are not yet widely available.
Dravet Syndrome News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
