Stiripentol combined with two other antiepileptic medicines seems to be an effective strategy to improve long-term seizure frequency in Dravet syndrome patients.
The study that contains that finding, “Stiripentol efficacy and safety in Dravet syndrome: a 12-year observational study” was published in the journal Developmental Medicine & Child Neurology.
In a prospective, open-label observational study from 2003 to 2015, researchers evaluated the long-term safety and effectiveness of stiripentol (Diacomit) as an antiepileptic medication for Dravet syndrome patients with ongoing seizures.
A total of 41 patients, recruited in Australia or the United Kingdom, with a median age of 5, who underwent treatment with stiripentol for a median duration of three years, were evaluated.
All participants underwent treatment with two additional antiepileptic medications, most frequently clobazam (Onfi ) and sodium valproate (Depakote).
Patients were seen at the clinic three months after starting therapy with stiripentol to determine its initial therapeutic effect and assess potential adverse events. Following the first evaluation, all participants were regularly monitored and the dose was gradually increased according to the degree of seizure and adverse events.
“Throughout the period of observation, frequency of focal seizures, generalized tonic-clonic seizures, status epilepticus, and adverse events were recorded,” researchers wrote.
Responders were defined as those having a 50 percent or higher improvement in seizure frequency for a defined seizure type or status epilepticus (a state defined by seizures lasting longer than five minutes, or so close together that the person doesn’t recover between them).
At the three-month clinical visit, 39 patients were taking stiripentol and 29 were still on therapy at the time of their most recent follow-up. The remaining patients discontinued the treatment due to either adverse events and/ or lack of effectiveness after seven months of treatment on average.
Out of the initial 41 patients, 23 were classified as responders within only three months of treatment with stiripentol, showing at least 50 percent reduction in seizure frequency.
At the point of final data collection, 12 of the 23 patients remained responders, and an additional eight were classified as responders. Overall, 20 of 41 patients were considered long-term responders.
Of the 27 patients with status epilepticus before study enrollment, 11 responded to the therapy and most had a 90 percent reduction in status events. Seven patients reported no further status epilepticus after stiripentol treatment.
The authors believe “this study demonstrates that stiripentol is an effective antiepileptic medication in Dravet syndrome, when used as part of unrestricted polytherapy,” and advocate “earlier institution of stiripentol in an infant or child experiencing frequent episodes of life-threatening status epilepticus.”