SCN1A-associated epilepsy with foudroyant course: the long-term prognosis is not necessarily disastrous!

The implications of a genetically substantiated Dravet syndrome (DS) in early childhood usually encompasses a highly active and drug-resistant epilepsy with intellectual disability and behavioral disorders along with a poor long-term prognosis; however, during the long-term course we certainly see patients with a spontaneous and sustained improvement even with freedom of seizures, a consecutively reduced drug load and a marked amelioration of cognitive and behavioral performance. Based on two such case reports we want to discuss and partially question the importance of syndromic diagnoses, such as DS, as the practicable foundation of forming a prognosis.