Dravet Syndrome
Treatment

Because Dravet syndrome is so rare, it can be difficult to find a pediatric neurologist who has experience in treating it. The Dravet Syndrome Foundation maintains a searchable listing of physicians who have treated patients with Dravet syndrome and related epilepsies. To search the site, visit www.dravetfoundation.org.

When caring for children with Dravet syndrome, our main goals are to achieve the best possible control of the seizures, prevent a dangerous type of seizure called status epilepticus, and improve the child's and family's quality of life.

To reduce the frequency and severity of the seizures, patients must avoid seizure triggers and take medications. A ketogenic diet — low in carbohydrates and high in fats — may also help. Surgery is generally not recommended, but an implantable device called a vagal nerve stimulator may reduce seizures in some cases.

Working with experts such as a speech therapist, physical therapist and occupational therapist can help improve patients' motor and cognitive development. Psychological and social support for the whole family is also important.

Seizure Trigger Avoidance

People with Dravet syndrome tend to be more sensitive to seizure triggers than those with other types of epilepsy, so it's important to avoid these common triggers:

  • Overheating (usually from hot baths or exercise)
  • Sudden temperature changes in the environment (hot or cold)
  • Fevers (should be treated with fever reducers such as ibuprofen or acetaminophen)
  • Flashing lights
  • Visual patterns such as stripes and other contrasting patterns
  • Stress or overexcitement

Vaccines may sometimes trigger a seizure. Your child's doctor may recommend giving your child a fever reducer before a vaccination and for 24 hours afterward to prevent vaccine-related fever.

Medications

Every Dravet patient is different, and medications that work for one child may not work for another. Generally, we introduce medications in a systematic order, beginning with those that have helped the largest number of Dravet syndrome patients, to determine what treatment regimen works best for each individual child.

The following medications are first-line treatments for Dravet syndrome, meaning they've benefitted more patients:

  • Clobazam (Onfi, Frisium, Urbanyl)
  • Valproic acid (Depakote, Depakene, Epilim, Epival)
  • Topiramate (Topamax)

If first-line treatments fail, your child's neurologist may try second-line medications:

  • Stiripentol (Diacomit)
  • Levetiracetam (Keppra)

Some common epilepsy drugs can worsen seizures in people with Dravet and should be avoided. These include:

  • Carbamazepine (Tegretol, Carbatrol)
  • Oxcarbazepine (Trileptal)
  • Lamotrigine (Lamictal)
  • Phenytoin (Dilantin, Epanutin)
  • Vigabatrin (Sabril, Sabrilan, Sabrilex)

Children who experience status epilepticus — life-threatening seizures that last longer than five minutes or that continue one after the other — must be treated with so-called rescue medications to try to stop the seizure. Your child's doctor may prescribe a rescue medication — typically a benzodiazepine, such as diazepam (Diastat), midazolam or lorazepam — to have on hand in case of status epilepticus.

Ketogenic Diet

The high-fat, low-carbohydrate ketogenic diet can be helpful for some children with Dravet syndrome, especially if their seizures aren't responding to medication. Studies have shown that 60 percent to 71 percent of Dravet patients have less frequent seizures while on the diet. Some research also suggests that a ketogenic diet is linked to improvements in behavior and cognition.

Ketogenic diets are designed by the patient's neurologist and a dietitian experienced in Dravet syndrome.

Vagal Nerve Stimulation

Vagal nerve stimulation may be considered if medications aren't working. Vagal nerve stimulation involves implanting a small device in the child's neck, around the vagus nerve. The device sends regular, mild electric signals from the vagus nerve up to the brain, which may interrupt a seizure or prevent one from starting.

Experimental Treatments

Researchers at UCSF and other institutions are actively seeking new treatments for Dravet syndrome. If standard treatment options fail, your child's doctors may discuss trying an experimental drug.

Currently, UCSF is leading a clinical trial of two doses of fenfluramine hydrochloride (ZX008). An earlier study found that the higher dose reduced patients' monthly seizures by 64 percent on average. For more information, please visit http://clinicaltrials.ucsf.edu/trial/NCT02682927.

Reviewed by health care specialists at UCSF Benioff Children's Hospital.

Related Information

UCSF Clinics & Centers

Pediatric Epilepsy Center of Excellence
1825 Fourth St., Fifth Floor
San Francisco, CA 94158
Phone: (415) 353-2437
Fax: (415) 353-2400
Appointment information

Neurosurgery Clinic
1825 Fourth St., Fifth Floor, 5A
San Francisco, CA 94158
Phone: (415) 353-7500
Fax: (415) 353-2889
Appointment information

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