Abstracts

Rationale: Dravet syndrome is a severe infantile onset epilepsy syndrome that often presents with recurrent episodes of status epilepticus (SE). The most important cause of morbidity and mortality in Dravet syndrome is SE. The usual initial management of SE in all patients with SE involves intravenous (IV) benzodiazepines followed by an IV fosphenytoin loading dose. However in patients with Dravet syndrome caused by mutations in SCN1A, medications that block voltage-gated sodium channels, like fosphenytoin, may exacerbate seizures. Thus a better understanding of management of SE in children with Dravet syndrome may improve outcomes. Methods: Thirteen children with confirmed SCN1A mutations and clinical diagnosis of Dravet syndrome were included in the study. We retrospectively reviewed all the episodes of SE, defined as continuous seizure >10 minutes, in these patients. We investigated triggers, length of seizure, medications utilized, and comorbidities of SE in these patients. Results: A total of 102 episodes of SE were reviewed. Status epilepticus occurred over the range of 2 months to 7 years. The mean age at which episodes of SE occurred is 1.63 ± 1.27 years. About half (53/102) of the episodes were associated with elevated temperature, many of which occurred with intercurrent illnesses. Respiratory failure requiring intubation was the most common complication associated with treatment of SE occurring in about one third of patients (30/102) of the episodes. Benzodiazepines were the most common medication administered particularly diazepam alone in 29 events (28%) or diazepam in combination with other medications in 54 episodes (53%). After benzodiazepines, fosphenytoin was the next most common treatment in 28 episodes (27%). As seizure duration increased, number of medication doses, numbers of different types of medication, and use of continuous IV drip medications increased. Conclusions: SE is common in children with Dravet syndrome. A more thorough understanding of triggers, complications, and response to treatment of SE in Dravet syndrome will lead to improved outcomes. We recommend that SE in patients with Dravet syndrome should be treated with a modified protocol rather than the routinely used SE algorithm.