Authors :
Presenting Author: Elena González-Alguacil, MD – Hospital Niño Jesus (Madrid)
VERONICA CANTARIN EXTREMERA, PhD – Hospital Universitario Niño Jesús
MARIA BALLARÁ PETITBÓ, MD – Hospital Universitario Niño Jesús
JUAN JOSE GARCIA PEÑAS, MD – Hospital Universitario Niño Jesús
NURIA LAMAGRANDE CASANOVA, MD – Hospital Universitario Niño Jesús
ANNA DUAT RODRIGUEZ, PhD – Hospital Universitario Niño Jesús
Victor Soto-Insuga, PhD – Hospital Niño Jesus (Madrid)
Rationale:
Super-refractory status epilepticus (SRSE) represents a critical challenge in clinical practice due to its prolonged duration, resistance to conventional therapies, and high morbidity. The need for alternative therapeutic strategies has led to the exploration of novel agents such as fenfluramine (FFA).Methods:
We conducted a retrospective, observational study to evaluate the use and efficacy of FFA in patients with SRSE and acute repetitive seizures
Results:
Seven patients were included: six with SRSE (mean duration: 5 days) and one with acute repetitive focal seizures. All had a previous epilepsy diagnosis, with a median of 14 prior antiseizure medications. Resolution of status epilepticus was achieved in 5 out of 7 patients following FFA initiation.
Among the SRSE cases, 4/6 presented with non-convulsive status. All met criteria for Lennox-Gastaut Syndrome. Etiologies included: 2 unknown, 1 structural, and 1 tuberous sclerosis complex (TSC2 mutation). FFA was initiated at a median of 6 days after SRSE onset (mean dose: 0.5 mg/kg/day). Two of these four patients achieved resolution of SE within 5 days, allowing benzodiazepine withdrawal and discharge (both with unknown etiology). The remaining two required additional therapies.
Two patients had focal SE and epilepsia partialis continua: 1 Rasmussen syndrome and 1 patient with mitochondrial disease (CARS2 mutation). Both were on midazolam infusions; one also received phenobarbital infusion. FFA was titrated to 0.7 mg/kg/day (over one month in CARS2 mutation and 10 days in Rasmussen Syndrome), with progressive seizure reduction. One patient was weaned off midazolam and discharged; the other discontinued phenobarbital infusion.
The final patient presented with acute repetitive focal seizures due to limbic encephalitis. FFA was started at 0.2 mg/kg/day and increased to 0.7 mg/kg/day over 9 days. Seizure reduction was observed by day 4, enabling hospital discharge.
Conclusions: In this small retrospective series, fenfluramine was associated with clinical improvement in seizure control in some patients with SRSE and acute repetitive seizures. It facilitated the reduction or discontinuation of anesthetic agents. Given its manageable safety profile and observed benefits, FFA may be considered as a therapeutic option in treatment-resistant SE.
Funding: No support