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Can Dravet Syndrome Be Cured?

Reviewed on 10/14/2020

Ask a Doctor

Please help! My little boy is only one year old and he can’t stop having seizures. After a month or two of increasing jerking and shaking movements the doctors couldn’t figure out, the electroencephalogram showed what the neurologist called a hallmark of Dravet – some electrical pattern of brainwaves she used to diagnose my baby with myoclonic seizures.

Even though we put him, on Klonopin and Depakene, he’s gone from having about five seizures a day to nine or 10 a day this week. My heart just breaks for my little guy.

Is there anything more we can do? Is maybe a brand new medication or procedure for Dravet around the corner? Is there a cure for Dravet Syndrome?

Doctor’s Response

Dravet syndrome is an infantile myoclonic seizure disorder. “Myoclonic” describes a jerking seizure caused by spasming muscle groups during which the child stay awake. Your child may experience other types of seizures too, including generalized ones. A pediatric neurologist may be consulted.

First, here are some lifestyle changes and safety tips:

  • The ketogenic diet: A diet extremely low in carbohydrates and high in fats and protein may be useful in children with epilepsy
    • Consult with your pediatrician and maybe a nutritionist before starting your child on this diet.
    • Pay particular attention to the possibility of dehydration.
  • Caution should be used in children with drop attacks, as they may fall and injure themselves. A helmet can be protective. Routine seizure precautions are applicable.

What Are the Medications for Dravet Syndrome?

  • The mainstays of medical therapy for myoclonic epilepsy are valproic acid (sodium valproate), ethosuximide, or benzodiazepines (clonazepam or clobazam).
  • Phenobarbital, lamotrigine, vigabatrin, and carbamazepine may worsen the seizures in some cases, so they shouldn’t be used continuously. 
  • Combination therapy with valproic acid and benzodiazepines is often helpful.
  • Stiripentol is indicated for treatment of seizures associated with Dravet syndrome in patients aged 2 years or older who are taking clobazam, but isn’t useful on its own.

Two drugs first approved in 2018 and 2020 specifically for Dravet Syndrome have helped improve the prognosis, however[CD1] .

  • A purified form of cannabidiol derived from the cannabis plant called Epidiolex is FDA-approved for seizures associated Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), or tuberous sclerosis complex in patients aged 1 year or older. 
    • Doctors don’t know how it works, but cannabidiol consistently showed a 35% to 40% drop in the number of seizures for the Epidiolex groups in several international controlled clinical trials.
  • Fenfluramine (Fintepla) is indicated for treatment of seizures associated with Dravet syndrome in patients two years and older. 

Sadly, there is no cure for Dravet, and up to 20% of children with Dravet do not survive into adulthood. Adults with Dravet usually are permanently reliant on caretakers, although the severity of symptoms may vary, according to the U.S. National Institutes of Health.

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Reviewed on 10/14/2020
References
Medscape Medical Reference





Dravet syndrome : Clinical presentation, genetic investigation and anti-seizure medication
Bradley Osterman MD, FRCPC, CSCN
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