Pediatric Dravet Syndrome
Dravet syndrome is a rare, severe form of epilepsy in children. Children with this genetic syndrome have frequent seizures that can last up to 45 minutes or longer.
Our experts at Children's Health provide the most advanced procedures and therapies in our region to treat children with Dravet syndrome. We have nationally recognized epilepsy centers at our Dallas and Plano locations, meaning you and your child can expect the highest quality of care.
What is Pediatric Dravet Syndrome?
Dravet syndrome appears in the first year of life in an otherwise healthy baby. Fever or infection triggers a very long seizure (a quick, uncontrollable disturbance in the brain). Children with Dravet syndrome often need to go to the emergency room because their seizures are so long (usually between 30 and 45 minutes). Most children with Dravet syndrome develop a developmental disability as they grow older. These developmental issues are related to the intense and frequent seizures and the genetic mutation, which impacts brain development.
What are the signs and symptoms of Pediatric Dravet Syndrome?
Most babies with Dravet syndrome have a seizure where only one side of their body is shaking (known as a hemiclonic seizure) or have a full-body shaking seizure (called a generalized tonic-clonic seizure). As a child gets older, they might experience other types of seizures like:
- Absence seizures may cause a child to stare into space, stop talking abruptly and then start talking again. They may also have repetitive movements like chewing their lips or moving a hand.
- Myoclonic seizures are known for their brief, shock-like muscle jerks.
A child might also experience:
- Seizures frequently triggered by a fever and an increase in body temperature
- Behavioral difficulties
- Developmental delays (such as struggles with coordination, speech and language, learning and/or playing)
- Sleep problems
- Unsteady walking
How is Pediatric Dravet Syndrome diagnosed?
During a detailed physical exam of your child, your Children’s Health℠ doctor will ask about your child’s medical history and family’s medical history. We will also try to learn as much as possible about how the seizure started and what you observed.
We might prescribe the following tests:
- Genetic testing to identify changes (also called mutations) in your child's chromosomes, genes or proteins that indicate they have Dravet syndrome.
- Electroencephalogram (EEG) to determine where in the brain the seizures are coming from. An EEG is performed by placing electrodes on the scalp and recording the electrical activity of the brain.
- MRI to identify changes in your child’s brain through a detailed image.
What are the causes of Pediatric Dravet Syndrome?
Dravet syndrome is caused by a gene mutation.
How is Pediatric Dravet Syndrome treated?
There is no cure for the disorder, but children with Dravet syndrome benefit from the following treatments and therapies:
Treatments for Dravet Syndrome
- Anti-seizure medications are the most common way to treat Dravet syndrome. These medications are in liquid form that your child can drink or a pill that your child can swallow.
- Ketogenic diet is a high-fat, low-carb diet for children who don’t respond well to medications. You and your child will work with one of our nutritionists to learn more about this diet.
- Vagus nerve stimulation uses a medical device that's placed inside your child’s chest, with a wire connecting to the vagus nerve (a cranial nerve that runs from the brainstem to the chest and abdomen). This device sends tiny electrical impulses, going through the vagus nerve to the brain that can help control the epilepsy and decrease the number of seizures.
Therapies for Dravet Syndrome
At Children’s Health, we have the expertise and resources to help your child with any issue they may experience with Dravet syndrome. For example:
- If your child experiences a sleep disorder, our Sleep Disorders Center can provide a thorough evaluation.
- If your child has limited speech, a speech-language therapist can help your child express themselves by improving speech with speech therapy, sign language, visual aids or speech technology aids.
- If your child has low muscle tone, a physical or occupational therapist can help strengthen their muscle tone or improve their walking gait.
- If your child needs orthotics, a physical therapist can create customized orthotics to help keep your child’s feet straight. The therapist can also help you find an active stroller or wheelchair for longer distances (a crouched gait makes walking difficult).
Pediatric Dravet Syndrome Doctors and Providers
We know a Dravet syndrome diagnosis can be frightening, but our world-renowned team of neurologists is here to help.
Rana Said, MD Professor of Pediatrics and Neurology
Alison Dolce, MD Pediatric Neurologist
Andrea Lowden, MD Pediatric Neurologist
Deepa Sirsi, MD Pediatric Neurologist
Frequently Asked Questions
What is the life expectancy of a child with Dravet syndrome?
Children with Dravet syndrome might have a shortened life expectancy because they are at higher risk for sudden unexpected death in epilepsy (SUDEP). This happens when a child with epilepsy dies unexpectedly after a seizure (usually at night). We don’t know what causes SUDEP. However, we recommend measures to monitor for SUDEP and aim for the best possible seizure control to decrease its risk.
Do Dravet syndrome symptoms get worse?
While Dravet syndrome does not get worse over time, your child may continue to experience epilepsy episodes throughout their life along with impairments in cognitive development, mobility and behavior.