Dravet syndrome is a rare and progressive epileptic
encephalopathy of infancy.
Stiripentol reduces the seizure frequency in patients with
Dravet syndrome. We evaluated the clinical characteristics of patients with
Dravet syndrome and their response to
stiripentol. We retrospectively collected the data of 21 patients (11 females; mean age, 8.2 years, range: 5.4-15 years) with
Dravet syndrome who were treated with
stiripentol in our outpatient clinic between June 2016 and June 2017. Patients with seizure reduction ≥50% were considered responders. Most of our patients had severe (47%) or moderate (33%) cognitive disabilities, although 14% had mild cognitive disability. There was a significant difference in both
status epilepticus and age between the groups with normal/mild versus severe/moderate neurocognitive prognoses. Of the patients, 85.7% were using
stiripentol. The mean duration of
stiripentol use was 41.2 months (range: 24-64 months). In 12 patients (57%), the seizure frequency decreased by more than 50%, and 2 of them were seizure-free.
Status epilepticus was not recorded after
stiripentol treatment in 8 of 11 patients with
status epilepticus. Despite the small sample size, our results suggest that
stiripentol has a favorable efficacy. In addition, considering the absence of
status epilepticus after treatment and the negative effects of
status epilepticus on cognitive development, early treatment should be initiated in SD patients, for whom disease control is difficult.