Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review
Abstract Status epilepticus (SE) is a life‐threatening emergency with high morbidity and mortality. In people with epilepsy, the management of SE is focused on early medical treatment. Stiripentol is a third‐generation antiseizure medication (ASM) approved for refractory generalized tonic–clonic sei...
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Wiley
2024-12-01
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| Series: | Epilepsia Open |
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| Online Access: | https://doi.org/10.1002/epi4.13036 |
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| author | Nicola Specchio Stéphane Auvin Adam Strzelczyk Francesco Brigo Vicente Villanueva Eugen Trinka |
| author_facet | Nicola Specchio Stéphane Auvin Adam Strzelczyk Francesco Brigo Vicente Villanueva Eugen Trinka |
| author_sort | Nicola Specchio |
| collection | DOAJ |
| description | Abstract Status epilepticus (SE) is a life‐threatening emergency with high morbidity and mortality. In people with epilepsy, the management of SE is focused on early medical treatment. Stiripentol is a third‐generation antiseizure medication (ASM) approved for refractory generalized tonic–clonic seizures in Dravet syndrome. The aim of this systematic review was to evaluate the effectiveness and safety of stiripentol in reducing the incidence of SE in patients with Dravet syndrome or any epilepsy characterized by recurrent SE. The PubMed and Cochrane databases were systematically searched, and gray literature was hand‐searched. Search results were screened by title and abstract; studies with data on the effect of stiripentol on SE outcomes, including the cessation of SE, reduction in number of SE episodes, or reduction in hospitalizations, were included. Of 66 records identified, 17 studies were eligible for inclusion, of which 15 were human studies (n = 474; aged 1.1–78 years), and two were animal experiments. Results of retrospective or prospective observational studies showed that stiripentol as add‐on therapy to ASMs such as clobazam or valproate reduced the incidence of SE in patients with Dravet syndrome or other developmental and epileptic encephalopathies (DEEs). A mean of 68% of patients (range 41%–100%) had a ≥50% reduction in SE episodes from baseline, and 26%–100% of patients (mean 77%) became SE‐free after stiripentol initiation. Moreover, this review found stiripentol, used as acute treatment, may also be effective for the cessation of super‐refractory SE, but data are limited to three retrospective case series. Stiripentol was generally well‐tolerated. In conclusion, stiripentol reduces the incidence of SE episodes in patients with Dravet syndrome and potentially other DEEs, and it promotes cessation of super‐refractory SE in patients with and without a history of seizures. Plain Language Summary Status epilepticus (SE) is a life‐threatening, long‐lasting seizure occurring in patients with/without epilepsy. This article analyzed 15 published studies that investigated the effects and safety of the anti‐seizure medication stiripentol for preventing SE in epilepsy patients (prevention) or stopping an SE episode (cessation), and two animal studies that investigated how stiripentol works. In epilepsy patients, stiripentol halved the number of SE episodes in 41–100% of patients, 26–100% of patients became SE‐free, and stiripentol was considered to be well tolerated. In patients with/without epilepsy, stiripentol may stop the SE episode after other drugs like anesthetics have not worked. |
| format | Article |
| id | doaj-art-a50466d248b041bebcfa0b2ad5688a5d |
| institution | DOAJ |
| issn | 2470-9239 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wiley |
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| series | Epilepsia Open |
| spelling | doaj-art-a50466d248b041bebcfa0b2ad5688a5d2025-08-20T02:50:04ZengWileyEpilepsia Open2470-92392024-12-01962017203610.1002/epi4.13036Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic reviewNicola Specchio0Stéphane Auvin1Adam Strzelczyk2Francesco Brigo3Vicente Villanueva4Eugen Trinka5Neurology, Epilepsy and Movement Disorders Unit, Bambino Gesù Children's Hospital IRCCS, Member of ERN EpiCARE Rome ItalyUniversité Paris Cité, INSERM NeuroDiderot Paris FranceEpilepsy Center Frankfurt Rhine‐Main, Department of Neurology University Hospital Frankfurt, Goethe‐University Frankfurt Frankfurt am Main GermanyDepartment of Neurology Hospital of Merano (SABES‐ASDAA) Merano ItalyRefractory Epilepsy Unit, Neurology Service Hospital Universitario y Politécnico La Fe, Member of ERN EpiCARE Valencia SpainDepartment of Neurology, Christian‐Doppler University Hospital, Centre for Cognitive Neuroscience Paracelsus Medical University, Member of ERN EpiCARE Salzburg AustriaAbstract Status epilepticus (SE) is a life‐threatening emergency with high morbidity and mortality. In people with epilepsy, the management of SE is focused on early medical treatment. Stiripentol is a third‐generation antiseizure medication (ASM) approved for refractory generalized tonic–clonic seizures in Dravet syndrome. The aim of this systematic review was to evaluate the effectiveness and safety of stiripentol in reducing the incidence of SE in patients with Dravet syndrome or any epilepsy characterized by recurrent SE. The PubMed and Cochrane databases were systematically searched, and gray literature was hand‐searched. Search results were screened by title and abstract; studies with data on the effect of stiripentol on SE outcomes, including the cessation of SE, reduction in number of SE episodes, or reduction in hospitalizations, were included. Of 66 records identified, 17 studies were eligible for inclusion, of which 15 were human studies (n = 474; aged 1.1–78 years), and two were animal experiments. Results of retrospective or prospective observational studies showed that stiripentol as add‐on therapy to ASMs such as clobazam or valproate reduced the incidence of SE in patients with Dravet syndrome or other developmental and epileptic encephalopathies (DEEs). A mean of 68% of patients (range 41%–100%) had a ≥50% reduction in SE episodes from baseline, and 26%–100% of patients (mean 77%) became SE‐free after stiripentol initiation. Moreover, this review found stiripentol, used as acute treatment, may also be effective for the cessation of super‐refractory SE, but data are limited to three retrospective case series. Stiripentol was generally well‐tolerated. In conclusion, stiripentol reduces the incidence of SE episodes in patients with Dravet syndrome and potentially other DEEs, and it promotes cessation of super‐refractory SE in patients with and without a history of seizures. Plain Language Summary Status epilepticus (SE) is a life‐threatening, long‐lasting seizure occurring in patients with/without epilepsy. This article analyzed 15 published studies that investigated the effects and safety of the anti‐seizure medication stiripentol for preventing SE in epilepsy patients (prevention) or stopping an SE episode (cessation), and two animal studies that investigated how stiripentol works. In epilepsy patients, stiripentol halved the number of SE episodes in 41–100% of patients, 26–100% of patients became SE‐free, and stiripentol was considered to be well tolerated. In patients with/without epilepsy, stiripentol may stop the SE episode after other drugs like anesthetics have not worked.https://doi.org/10.1002/epi4.13036acute seizuresantiseizure medicationdevelopmental and epileptic encephalopathyDravet syndromehospitalizationmorbidity |
| spellingShingle | Nicola Specchio Stéphane Auvin Adam Strzelczyk Francesco Brigo Vicente Villanueva Eugen Trinka Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review Epilepsia Open acute seizures antiseizure medication developmental and epileptic encephalopathy Dravet syndrome hospitalization morbidity |
| title | Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review |
| title_full | Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review |
| title_fullStr | Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review |
| title_full_unstemmed | Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review |
| title_short | Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review |
| title_sort | efficacy and safety of stiripentol in the prevention and cessation of status epilepticus a systematic review |
| topic | acute seizures antiseizure medication developmental and epileptic encephalopathy Dravet syndrome hospitalization morbidity |
| url | https://doi.org/10.1002/epi4.13036 |
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