Acute encephalopathy in Dravet syndrome: case reports and literature review

Dravet syndrome is a rare and catastrophic type of epilepsy in infants. Acute encephalopathy has been sporadically reported in patients with Dravet syndromehowever, the risk factors for this serious complication have not been identified. We report two patients with a clinical diagnosis of Dravet syndrome who experienced acute encephalopathy initiated by refractory status epilepticus. SCN1A mutational analysis revealed a previously reported nonsense mutation in one patient and a novel missense mutation in the other. Analysis of our cases and previously published cases revealed that patients with Dravet syndrome who have a more severe phenotype have an increased likelihood of developing acute encephalopathy compared with patients with less severe phenotypes