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Dravet Syndrome

What is Dravet syndrome?

In 1978, Charlotte Dravet first described Dravet syndrome, and it was found to have a genetic basis in 2001. Dravet syndrome is a rare genetic dysfunction of the brain (epileptic encephalopathy). It begins in the first year of life in an otherwise healthy infant, and it is lifelong. Let’s simplify it with common synonyms or search words:
  • Severe myoclonic epilepsy in infancy (SMEI)
  • Polymorphic epilepsy in infancy (PMEI)
  • Epilepsy with polymorphic seizures
  • DS

How common is Dravet syndrome?

It’s not fully known how many people are affected—estimated 1 in 15,700 to 1 in 40,000 live births. It affects males and females in equal proportions. Significant risk factors for a Dravet syndrome diagnosis are:
  • Seizures lasting more than 10 minutes
  • Seizures occurring on one side of the body
  • Seizures triggered by a warm-water bath in children under 12 months old

What are the signs and symptoms of Dravet syndrome?

Dravet syndrome has been characterized by prolonged febrile and non-febrile seizures within the first year of a child’s life. This disease progresses to other seizure types:
  • Myoclonic and partial seizures
  • Psychomotor delay
  • Ataxia
It’s also characterized by cognitive impairment, behavioral disorders, and motor deficits.

What treatment options are available for Dravet syndrome?

Diagnosing the child early is critical to proper treatment and achieving the best outcome. A child may need a multidisciplinary team to address the many ways Dravet syndrome can affect the family. Seizure treatment is aimed at finding the best combination of medicines to treat chronic seizures and prevent and treat potential seizure emergencies. Getting the best seizure control possible is the goal. This could also help improve the child’s developmental abilities and decrease mortality risk.

Where can I find more information about Dravet syndrome?

Dravet Syndrome Articles