• Dravet syndrome is a rare form of epilepsy that begins during infancy and can result in moderate to severe developmental delays.
  • Many people diagnosed with the condition have a gene mutation in the SCN1A gene.
  • Medications, therapies, and lifestyle changes can help manage the symptoms of Dravet syndrome or reduce the length and frequency of seizures.

Dravet syndrome is a rare form of epilepsy that involves frequent and prolonged seizures.

As of 2015, it affected an estimated 1 in 15,700 people in the United States. It accounts for less than 0.2 percent of all epilepsy cases, according to the National Organization for Rare Disorders.

People with Dravet syndrome usually have their first seizure within their first year of life, at the average age of 5.2 months old. Some children develop symptoms later, by 18 to 24 months old.

Dravet syndrome is caused by genetic mutations that affect how sodium ion channels work.

Sodium ion channels help control the movement of sodium ions into and out of cells. This plays an essential role in nerve signaling. Overactive nerve signaling in the brain causes seizures.

Children with Dravet syndrome have frequent and often prolonged seizures.

They experience tonic-clonic or hemiconvulsive seizures, which cause muscles to stiffen and jerk. They may also have other types of convulsive and nonconvulsive seizures, especially as they get older.

Many children with Dravet syndrome also have status epilepticus, or seizures that last for longer than 5 minutes or occur very close together. Older children and adults with Dravet syndrome may experience less frequent and shorter seizures than younger children.

By the time they’re 2 to 5 years old, children with Dravet syndrome experience developmental delays and other health challenges, such as:

  • behavioral concerns
  • cognitive delays or disabilities
  • speech delays or disabilities
  • trouble with dexterity, coordination, balance, movement
  • crouched walking posture
  • muscle weakness
  • difficulty sleeping
  • chronic infections
  • delayed growth

Dravet syndrome may also affect the autonomic nervous system. This may make it difficult for a person with the condition to regulate functions like body temperature and heart rate.

If your child has a seizure, their doctor will ask you about their symptoms and medical history.

They’ll also order one or more of the following tests:

  • electroencephalogram (EEG): to assess your child’s brain activity
  • CT or MRI scans: to create pictures of your child’s brain
  • blood tests: to check for certain disorders

These tests can help your child’s doctor confirm or rule out potential causes of the seizure.

When symptoms of Dravet syndrome first appear, the results of these tests often fail to indicate that a child has the condition. Over time, however, EEG and MRI scans may show changes in the child’s brain.

Your child’s doctor may also order genetic testing to check for gene mutations related to epilepsy.

In a 2015 Swedish study in 42 children with Dravet syndrome, researchers found that 37 of them (88 percent) had a mutation in the SCN1A gene.

Yet, not all people with SCN1A gene mutations develop Dravet syndrome.

Your child’s treatment plan for Dravet syndrome may include:

  • medication
  • vagus nerve stimulation
  • rehabilitative therapy
  • lifestyle changes

Dravet syndrome doesn’t always respond to treatment. Your child may need to try more than one medication to find a treatment plan that works for them.

Maintenance medication

Your child’s doctor will prescribe antiseizure medications to reduce the frequency of seizures. These maintenance medications are taken on a regular, ongoing basis.

The Food and Drug Administration (FDA) has approved three maintenance medications for Dravet syndrome:

  • cannabidiol (Epidiolex)
  • fenfluramine (Fintepla)
  • stiripentol (Diacomit)

Other first-line antiseizure medications include:

  • clobazam (Onfi, Frisium, Urbanyl)
  • valproic acid (Depakote, Depakene, Epilim, Epival)

If first-line treatments don’t control the frequency of your child’s seizures, their doctor may prescribe a second- or third-line medication such as:

  • topiramate (Topamax)
  • clonazepam (Klonopin, Rivotril)
  • ethosuximide (Zarontin)
  • levetiracetam (Keppra)
  • zonisamide (Zonegram)

Sodium ion channel agents are another type of antiseizure medication that’s often prescribed for other types of epilepsy. However, taking them on a regular basis can worsen seizures in Dravet syndrome.

Rescue medication

If your child has seizures that last for 5 minutes or longer, their doctor will prescribe a rescue medication to stop a seizure after it’s started. Rescue medications include:

  • clonazepam (Klonopin, Rivotril)
  • diazepam (Diastat)
  • lorazepam (Ativan)
  • midazolam (Versed)

Creating a seizure action plan

A diagnosis of Dravet syndrome means learning how to manage seizures. Preparation and education is key for anyone who may be caring for someone with Dravet syndrome.

The Dravet Syndrome Foundation encourages families to work with a neurologist to put a seizure action plan in place, with an emergency protocol for treating prolonged seizures.

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Vagus nerve stimulation

If your child continues to have frequent seizures despite taking medication, their doctor may recommend vagus nerve stimulation (VNS).

In this treatment, a surgeon implants an electrical device under the skin on your child’s chest. A wire connects this device to the vagus nerve on the left side of your child’s body. When it’s activated, the device sends electrical signals along the vagus nerve to your child’s brain.

In a 2017 review of research, VNS appeared to cut the frequency of seizures in half among people with Dravet syndrome. VNS may be more or less effective for some people.

Rehabilitative therapy

Your child’s doctor will assess their development on a regular basis and recommend therapies to manage development delays, such as:

  • occupational therapy: to help you and your child find strategies and tools to manage their condition
  • physical therapy: to improve your child’s walking abilities, balance, coordination, dexterity, or strength
  • behavioral therapy: to promote social skills, emotional regulation, and mental health
  • speech therapy: to promote speech development

Lifestyle changes

Eating a ketogenic diet may be beneficial for children with Dravet syndrome, report the authors of a 2018 review. A keto diet is high in fat and low in carbohydrates, such as grains and starchy vegetables. It may reduce the frequency of seizures, according to the review.

It’s also important to identify seizure triggers and avoid them when possible. Common seizure triggers include:

  • overheating or sudden body temperature changes, caused by fever, physical exertion, or warm baths
  • overexcitement or stress
  • flickering lights
  • visual patterns
  • illness

Your child’s doctor and occupational therapist can help you develop strategies to avoid or manage triggers.

They may also encourage you to use a baby monitor or seizure detection device to monitor your child for seizures at night. Some parents choose to share a room with their child.

Clinical trials

Scientists continue to develop and test new treatments for Dravet syndrome in both animal studies and clinical trials.

In a clinical trial, human participants receive a treatment that’s being studied to learn how safe and effective it is.

Clinical trials are currently underway to study several experimental treatments for Dravet syndrome, including the following drugs that may help reduce seizure frequency:

  • EPX-100
  • lorcaserin (Belviq)
  • soticlestat (TAK-935/OV935)
  • STK-001

Research is ongoing to learn how safe and effective these medications are.

Dravet syndrome is a lifelong condition with no known cure. However, early diagnosis and treatment may help:

  • reduce symptoms
  • limit developmental delays
  • improve quality of life

Children with Dravet syndrome usually have developmental delays by the time they’re 2 to 5 years old, according to NORD. Some people with Dravet syndrome have mild development delays, but most have moderate to severe delays and require ongoing care as adults.

Roughly 80 to 85 percent of children with Dravet syndrome live into adulthood. They have increased risk of early death due to sudden unexpected death, prolonged seizures, or seizure-related accidents.

People with Dravet syndrome often have less frequent and shorter seizures as they get older.

Dravet syndrome is a type of epilepsy that involves frequent and prolonged seizures. People with this condition also experience developmental delays and other health concerns.

Doctors may prescribe a combination of antiseizure medications, rehabilitation therapies, and other treatments for Dravet syndrome. Following a ketogenic diet may help reduce the frequency of seizures. Identifying and limiting seizure triggers is also important.

Managing Dravet syndrome can be challenging for parents and other family members.

The Dravet Syndrome Foundation runs support groups and other programs for families coping with this condition in the United States. Seeking out support and creating a seizure action plan are key steps when managing Dravet syndrome.