Efficacy of Stiripentol in the Prevention and Cessation of Status Epilepticus
Abstract number :
1.224
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2023
Submission ID :
215
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Gabrielle Stires, APRN-CNP – Biocodex
Stephane Auvin, MD, PhD, FAES – Center for rare epilepsies, Pediatric Neurology Dpt Robert-Debré Hospital & INSERM NeuroDiderot; Nicola Specchio, MD, PhD – Rare and Complex Epilepsy Unit, Department of Neurosciences, Bambino Gesù Children's Hospital; Adam Strzelczyk, MD, PhD – Epilepsy Center Frankfurt Rhine-Main, Goethe-University and University Hospital Frankfurt; Francesco Brigo, MD, PhD – Department of Neurology, Hospital of Merano; Vicente Villanueva, MD, PhD – Refractory Epilepsy Unit, Neurology Service, Hospital Universitario y Politécnico La Fe; Eugen Trinka, MS, PhD – Christian Doppler University Hospital, Public Health, Health Services Research and HTA, UMIT Tirol, Karl Landsteiner Institute
Rationale: Status epilepticus (SE) is a neurologic life-threatening emergency with a high morbidity and mortality. Mechanisms of action and biological properties of stiripentol (STP), an anti-seizure medication approved for drug-resistant generalized tonic-clonic seizures in Dravet syndrome (DS), has potential therapeutical effects against SE in the acute phase, and it may also prevent repeated episodes of SE in DS. We collected evidence of the efficacy of STP in terminating or preventing SE both in DS and non-DS patients.
Methods: An electronic literature search not restricted either by year of publication nor by language was conducted with Boolean operators (i.e., stiripentol AND status epilepticus). Backward citation searching was also conducted manually. Databases used were Cochrane (Wiley) and PubMed (NLM).
Results: On 60 references, we found 17 with original data of STP impact on SE outcomes. Two preclinical studies showed that STP exhibits anticonvulsant properties, reaching suppression of SE occurrence. STP was more effective in immature brains (young animals vs. older ones) and remained effective during prolonged SE (more than 45 min), where benzodiazepines had less effect. Eleven clinical references presented STP efficacy data to prevent SE in DS patients, aged from 1.3 to 26 years. Reduction in SE occurrence was found in 22 to 100 % of patients and SE cessation in 4 to 100%. The largest study showed high efficacy of STP in short-term (< 6 months treatment) and long-term evaluation, with emergency hospitalizations dropping from 91% to 43% and 12%, respectively. Finally, four clinical articles showed STP efficacy in SE cessation in patients aged from 18 to 88 years.
Conclusions: Clinical evidence suggests that STP is an effective and well-tolerated therapy that reduces the frequency of prolonged seizures and SE (as well as its duration) in DS, and it might be of interest to introduce STP as early as possible in DS patients in order to suppress convulsive SE episodes. In addition, various observations extend the use of STP beyond DS, proved useful to treat SE in patients with drug-resistant SLC13A5-related epilepsy and with Super Refractory SE.
Funding: Biocodex
Clinical Epilepsy