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- W4377011830 abstract "To evaluate the safety and efficacy of stiripentol initiated before 2 years of age in patients with Dravet syndrome.This was a 30-year, real-world retrospective study. We extracted the data of the 131 patients (59 females, 72 males) who initiated stiripentol before 2 years of age between 1991 and 2021 from the four longitudinal databases of Dravet syndrome available in France.Stiripentol was added to valproate and clobazam (93%) at 13 months and a median dose of 50 mg/kg/day. With short-term therapy (<6 months on stiripentol, median 4 months, median age 16 months), the frequency of tonic-clonic seizures (TCS) lasting longer than 5 minutes decreased (p < 0.01) and status epilepticus (>30 minutes) disappeared in 55% of patients. With long-term therapy (last visit on stiripentol <7 years of age, median stiripentol 28 months, median age 41 months), the frequency of long-lasting TCS continued to decline (p = 0.03). Emergency hospitalizations dropped from 91% to 43% and 12% with short- and long-term therapies respectively (p < 0.001). Three patients died, all from sudden unexpected death in epilepsy. Three patients discontinued stiripentol for adverse events; 55% reported at least one adverse event, mostly loss of appetite/weight (21%) and somnolence (11%). Stiripentol was used earlier, at lower doses, and was better tolerated by patients in the newest database than in the oldest (p < 0.01).Initiating stiripentol in infants with Dravet syndrome is safe and beneficial, significantly reducing long-lasting seizures including status epilepticus, hospitalizations, and mortality in the critical first years of life." @default.
- W4377011830 created "2023-05-19" @default.
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- W4377011830 date "2023-05-17" @default.
- W4377011830 modified "2023-10-01" @default.
- W4377011830 title "Initiating stiripentol before 2 years of age in patients with Dravet syndrome is safe and beneficial against status epilepticus" @default.
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- W4377011830 doi "https://doi.org/10.1111/dmcn.15638" @default.
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