Dravet syndrome is a rare type of epilepsy. Seizures usually start in the first year of your child’s life. In addition to frequent and varied seizures, your child will experience lags in mental and physical development, with the possibility of many other symptoms. Medications help reduce the number and severity of your child’s symptoms.
Dravet syndrome is a rare type of epilepsy that starts in the first year of your baby’s life. Its first occurrence is usually a long-lasting seizure (more than five minutes) that’s triggered by a high fever.
Children with Dravet syndrome have a wide range of seizure types and severity. They also have many other symptoms, including developmental setbacks, speech and language problems, and balance and walking issues.
Dravet syndrome was previously known as severe myoclonic epilepsy of infancy, epilepsy with polymorphic seizures and polymorphic epilepsy in infancy.
Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy
Dravet syndrome can be inherited. It’s an autosomal dominant disorder, which means you only need to get the changed (mutated) gene — the SCN1A gene — from one parent. (Autosomal means the affected gene is on one of the 22 nonsex chromosomes from either parent.) If you’re a parent with the mutated (changed) gene, your child has a 50% chance of inheriting the defective gene.
But only between 4% and 10% of SCN1A mutations in children with Dravet syndrome are inherited from a parent. About 90% of SCN1A mutations aren’t found in the child’s parent and are new gene mutations only present in the child.
Genetics is tricky. There are over 6,000 places for mutations to occur on the SCN1A gene. This means that many children’s gene mutations haven’t been seen in other children. SCN1A mutations result in a range of associated conditions, from migraine to mild-to-moderate forms of epilepsy to the most severe form of epilepsy in Dravet syndrome.
Researchers estimate that between 1 in 15,700 and 1 in 40,000 infants born in the U.S. have Dravet syndrome. About 3% to 8% of children who have their first seizure by 12 months of age are likely to have Dravet syndrome.
Seizures usually start in the first year of your child’s life. The first seizure often happens with a fever. After the first seizure, additional seizures happen without a fever. The seizure may involve jerking muscle movements on one side of their body.
Other features of Dravet syndrome include:
Other seizure triggers include:
Symptoms of Dravet syndrome vary from child to child. Symptoms can range from mild to severe.
Some of the most common seizure-related symptoms include:
Other common symptoms include:
Dravet syndrome happens when there’s a change (mutation) in the SCN1A gene. Nearly 80% of children diagnosed with Dravet syndrome have the SCN1A gene mutation. However, having the mutation isn’t enough to make the diagnosis, and the absence of the mutation doesn’t exclude the diagnosis. There’s a range or family of related SCN1A gene mutation disorders. Not all children with this gene mutation will develop Dravet syndrome. The mildest condition along the range of SCN1A gene mutations is familial hemiplegic migraine. The most severe condition is Dravet syndrome.
The most serious complications include:
Your child’s healthcare provider will ask you about their medical and medication history. Diagnosis is sometimes delayed, as magnetic resonance imaging (MRI) and electroencephalogram (EEG) results are usually normal at first.
Your child’s healthcare provider will conduct a clinical exam to look for the symptoms of Dravet syndrome. Specific information you’ll be asked about your child may include:
They’ll also ask you to describe the types of seizures your child has had, what medications were prescribed (if any) and how well they controlled your child’s seizures (if medication was prescribed).
They may also suggest a blood test to check for the SCN1A gene mutation, but they can usually make the diagnosis based on your child’s symptoms.
The goal of treatment is to reduce the number and the severity of your child’s seizures. Because seizures range in type and length, no two children respond to treatment in the same way.
Medications approved specifically to treat seizures associated with Dravet syndrome are:
All three medications are approved for use in children two years of age or older.
In 2017, epilepsy experts called the North American Consensus Panel developed treatment recommendations, listing medications that should be tried first, second and third. These medications are shown in the table.
|First-line treatments||Clobazam (Onfi®, Frisium®, Urbanyl®) Valproic acid (Depakote®, Depakene®, Epilim®, Epival®)|
|Second-line treatments (added to first-line medications)||Sitripentol (Diacomit®), Topiramate (Topamax®)|
|Third-line treatments||Clonazepam (Klonopin®, Rivotril®), Levetiracatam (Keppra®), Zonisamide (Zonegran®), Ethusuximide (Zarontin®)|
|Clobazam (Onfi®, Frisium®, Urbanyl®) Valproic acid (Depakote®, Depakene®, Epilim®, Epival®)|
|Second-line treatments (added to first-line medications)|
|Sitripentol (Diacomit®), Topiramate (Topamax®)|
|Clonazepam (Klonopin®, Rivotril®), Levetiracatam (Keppra®), Zonisamide (Zonegran®), Ethusuximide (Zarontin®)|
Seizures seen in persons with Dravet syndrome are difficult to control. The goal of seizure treatment is to find the best combination of medications to treat your child’s long-term condition. Usually two or more medications are needed to treat the multiple seizure types seen with Dravet syndrome.
Some antiseizure medications can make seizures worse and shouldn’t be prescribed. These medications include phenytoin (Dilantin®), carbamazepine (Tegretol®), fosphenytoin (Cerebyx®), lamotrigine (Lamictal®), oxcarbazepine (Trileptal®), rufinamide (Banzel®) and vigabatrin (Sabril®, Sabrilan®, Sabrilex®). Talk with your child’s healthcare provider if their seizures worsen while taking any of these medications.
Your child’s healthcare provider may discuss the use of other treatments that have shown positive results in people with Dravet syndrome. These treatments include:
Work with your child’s healthcare provider to develop a seizure action plan for home or school. This plan may include rescue medications that are used as an emergency treatment for continuous seizures (status epilepticus). This medication is given to stop your child’s seizure. Rescue medications are usually benzodiazepines, which include:
Before starting school, your child should have cognitive and developmental assessments. Based on the results, your child’s school can recommend an Individualized Education Plan (IEP) or your child may qualify for a 504 program (plans and programs that some schools develop to give children with disabilities support and proper accommodations).
Ask your child’s healthcare provider about physical, occupational, speech and social/play therapies for your child, too. Your child may benefit from orthotics for foot and ankle alignment issues or may require orthopedic surgery for bones of the leg or foot due to the physical changes that can occur with Dravet syndrome.
No. In most children (80%), Dravet syndrome is caused by a genetic mutation in the SCN1A gene. Defects in this gene cause improper functioning of brain cells, which plays a role in the development of epilepsy. The defective gene can be inherited, but most of the time, the gene mutation is new in your child and not inherited.
Your child may have long-lasting and frequent seizures. The goal of treatment is to achieve the best seizure control possible. Medications help reduce the number and severity of seizures, but becoming completely seizure-free isn’t usually possible.
Your child will have other health problems and developmental setbacks that need to be caught early so they can be managed early. A crouched gait (unsteady walk/balance) is seen with increasing age (teen years and beyond). Mental decline slows and stabilizes as your child gets older. Seizures also tend to decrease in number and length as your child ages. Despite this, most children, teens and adults need ongoing care from parents or caregivers.
There isn’t a cure for Dravet syndrome right now. But research is ongoing.
About 15% to 20% of people with Dravet syndrome die before adulthood. Up to 20% of individuals with Dravet syndrome usually die from sudden unexpected death in epilepsy (SUDEP), long-lasting seizures and seizure-related accidents and injuries such as drowning and infections.
A note from Cleveland Clinic
Receiving a Dravet syndrome diagnosis isn’t easy news. Fortunately, unlike many other diseases, researchers know that the cause of this syndrome in most cases is a defective SCN1A gene. Researchers are working toward improved treatments and, one day, a cure. For now, three new medications have been specifically approved to treat Dravet syndrome. Seizures in Dravet syndrome are difficult to control. Fortunately, new medications, older, established medications and other therapies can help reduce the number and severity of your child’s seizures. Your child will need lifelong care. In addition to the care and support offered by your healthcare team, it may be helpful to have the support of other families who also have a child with this syndrome. Ask your child’s healthcare provider for names of local support groups, as well as check out the organizations listed in the reference section of this article.
Last reviewed by a Cleveland Clinic medical professional on 03/15/2022.
Learn more about our editorial process.