Dravet syndrome is a rare and severe form of early onset epileptic syndrome beginning in infancy characterized by refractory seizures and neurological development disorders. The first seizure occurs in the first year of life (usually at 5-8 months of age) in a normal developing child (Dravet et al. 2011).
Dravet Syndrome was first described in 1978 as « Severe Myoclonic Epilepsy in Infancy » (SMEI) by Dr Charlotte DRAVET but has been recognized throughout the world since then.
The first description of the clinical picture was :
Because all of these clinical signs were not present in every patient, the criteria were enlarged and in 2001, the International League Against Epilepsy changed the epilepsy syndrome name from “SMEI” to “Dravet syndrome”.
The exact frequency in the general population is unknown. In 1990 it was estimated that Dravet syndrome occurred between 1/20,000 and 1/40,000 births. This frequency was probably an underestimate because the disease was not well known at that time.
Dravet syndrome seems to affect an equal number of boys and girls and no geographic region has a particularly high or low number of cases.