The answer may contain complex scientific concepts. For a better understanding do not hesitate to ask your child’s doctor.
Despite the growing knowledge on Dravet syndrome and new therapeutic options introduced over the last decade, there are still unanswered questions. Research is still needed to understand the influencing factors of Dravet syndrome regarding the clinical course and outcome: why do some seizures remain untreated by antiepileptic medicines, are there other genes involved, what is the life expectancy, etc.
Poor developmental and mental outcome was first thought to be the result of high frequency, intensity and uncontrolled seizures. But in our current knowledge this has not been proven. It seems that the genetic origin of Dravet syndrome is the most important cause of developmental difficulties.
Dravet syndrome seizures severity may depend on the type of mutation affecting the SCN1A gene. Nevertheless, current understanding suggests that other factors may also play a role.
Dravet syndrome is a life-threatening disorder. However, dying from a seizure remains exceptional. And yet, the mortality rate is higher than in the general population. This over-mortality rate results from factors such as infections, prolonged status epilepticus, accidents or remain unexplained. These unexplained deaths are a topic of active research in all types of epilepsies. They are medically defined as “SUDEP” (which stands for “sudden unexpected death in epilepsy”). However, a majority of children with Dravet syndrome live well into adulthood.