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What is Dravet Syndrome?

Causes of Dravet Syndrome

Dravet syndrome results from a change in the SCN1A gene(mutation). The SCN1A gene mutation is present in around 80% of children with Dravet syndrome. Although the absence of the mutation does not rule out the diagnosis, neither does the presence of the mutation suffice to make the diagnosis. A variety or family of SCN1A gene mutation syndromes exist. Dravet syndrome does not always manifest in children with this gene mutation. Familial hemiplegic migraine is the mildest condition associated with the range of SCN1A gene mutations. Dravet syndrome is the most serious condition.

Complications of Dravet Syndrome

The most serious complications include:

  • Continuous seizures (status epilepticus). This is an emergency condition that requires immediate treatment.
  • Sudden unexplained death in epilepsy (SUDEP).