AEDS EFFICACY IN THE DRAVET SYNDROME: A CROSS-SECTIONAL STUDY

Purpose: Dravet syndrome (DS) is a rare epilepsy with seizures’ onset during the first year of life. SCN1A gene abnormalities are detected in about 80% of patients. We aimed to evaluate the efficacy of AEDs in a large series of this pharmacoresistant epilepsy. Method: This is a cross-sectional study on all our patients with DS and SCN1A mutations who had a visit within 2010–2013. We included 54 patients (32 M and 22 F), aged from 2 to 23 years. We reviewed the characteristics of seizures (age of onset, type, duration, frequency and day/night prevalence) in relation to the AEDs used and patient0 s age. Results: Only 5 patients (9%) were seizure free for more than 1 year. Seizures were mainly fever sensitive. In the oldest patients (>10y) compared to youngers (6–10 years), seizures were most often tonic-clonic (86% vs. 36% in each group respectively), shorter (<1 min in 55% vs. 1– 5 min in 64%), sleep-related (55% vs. 36%), and rather more frequent (weekly in 55% vs. monthly in 71%). Fourty patients (74%) received a tritherapy (VPA, STP, CLB), associated with another AED in 30 (74%) of them (TPM, LEV, ZNG, CZP, ketogenic diet, bromide, canabidiol). Convulsive status epilepticus (SE) disappeared in 48% of patients after the introduction of STP. However, 5 children (9%) still experienced SE after 6 years and one after 11 years, mainly in the context of fever. Conclusion: Despite the decrease of status epileptics and long lasting seizures with tritherapy (STP, VPA, CLB), <10% of patients are seizure free and half remain with weekly seizures up to adulthood. These preliminary data suggest that there is still a need for developing and evaluating new AEDs in DS.