Stiripentol in the Management of Epilepsy

  title={Stiripentol in the Management of Epilepsy},
  author={Katherine C. Nickels and Elaine C. Wirrell},
  journal={CNS Drugs},
Stiripentol is a structurally unique antiepileptic drug that has several possible mechanisms of action, including diverse effects on the gamma-aminobutyric acid (GABA)-A receptor and novel inhibition of lactate dehydrogenase. Because of its inhibition of several cytochrome P450 enzymes, it has extensive pharmacokinetic interactions, which often necessitates reduction in doses of certain co-therapies, particularly clobazam. Stiripentol also has a neuroprotective action, by reducing calcium… 

Stiripentol: A Review in Dravet Syndrome

Based on the available evidence, stiripentol, as an adjunct to clobazam and valproate, is a demonstrably beneficial and generally well-tolerated second-line treatment for patients with DS.

Stiripentol for the treatment of seizures associated with Dravet syndrome

  • C. Chiron
  • Psychology, Medicine
    Expert review of neurotherapeutics
  • 2019
Stiripentol could be especially useful in two critical conditions of the disease, very young age (<2 years) and convulsive status epilepticus, and seizure freedom seems occasionally be obtained with fenfluramine.

Lorcaserin for Dravet Syndrome: A Potential Advance Over Fenfluramine?

Preliminary uncontrolled studies in patients with Dravet syndrome have yielded promising results, and a phase III, double-blind, placebo-controlled, parallel group trial is currently ongoing to assess its efficacy and safety in children and adults with Draket syndrome.

Antiepileptic Drugs in Pediatrics.

The history and evolution of AED development, features of specific AEDs with a focus on the newest generation, and examples of Aeds in development are reviewed.

Pharmacokinetic considerations for anti-epileptic drugs in children

The main pharmacokinetic characteristics of AEDs used in children are reviewed, focusing on age-related factors which are of relevance when treating this patient population, especially in children.

Pharmacokinetics and Drug Interaction of Antiepileptic Drugs in Children and Adolescents

The mechanism of action, pharmacokinetics, drug–drug interactions, and safety/tolerability profiles of the main AEDs currently used in children and adolescents are reviewed, paying particular regard to issues of relevance when treating this patient population.

Efficacy and Safety of Long-Term Treatment with Stiripentol in Children and Adults with Drug-Resistant Epilepsies: A Retrospective Cohort Study of 196 Patients

It is suggested that stiripentol is an effective and well-tolerated therapeutic option not only in Dravet syndrome but also in other epilepsy syndromes with or without an established genetic aetiology.

Pharmacokinetic Drug–Drug Interactions among Antiepileptic Drugs, Including CBD, Drugs Used to Treat COVID-19 and Nutrients

The purpose of this review is to present data from the last few years on drug–drug interactions among of Aeds, as well as AEDs with other drugs, nutrients and food, and to pay a special attention to the new potential interactions of the applied first-generation AEDS.

A Practical Guide to the Treatment of Dravet Syndrome with Anti-Seizure Medication

This practical guide provides an overview of three adjunct ASMs for the treatment of seizures in patients with Dravet syndrome including their indications/contraindications, mechanism of action, efficacy, safety and tolerability profile, dosage requirements, and laboratory and clinical parameters to be evaluated.

The role of new medical treatments for the management of developmental and epileptic encephalopathies: Novel concepts and results

An overview of new medical treatment options for developmental and epileptic encephalopathies is provided and the clinical implications of these results for improved treatment are discussed.



The effects of stiripentol on GABAA receptors

Positive modulation by stiripentol was most effective at GABAA receptors containing an α3 subunit, which is developmentally regulated, with highest levels in the immature brain, and may explain the greater clinical efficacy of stiripENTol in childhood‐onset epilepsies, including Dravet syndrome.

Stiripentol. A novel antiepileptic drug.

STP potentiates central gamma-aminobutyric acid (GABA) transmission and is characterized by nonlinear pharmacokinetics and inhibition of liver microsomal enzymes, and seems a good candidate for adjunctive therapy in intractable epilepsy.

The efficacy of bromides, stiripentol and levetiracetam in two patients with malignant migrating partial seizures in infancy

Antiepileptic drugs, previously reported to be beneficial in case reports, when given concomitantly, may substantially reduce the number and severity of seizures, without influence on psychomotor development.

Stiripentol, a Putative Antiepileptic Drug, Enhances the Duration of Opening of GABAA‐Receptor Channels

Stiripentol is currently an efficient drug for add‐on therapy in infantile epilepsies because it improves the efficacy of antiepileptic drugs (AEDs) through its potent inhibition of liver cytochromes P450.


  • C. Chiron
  • Medicine, Psychology
  • 2011

Stiripentol in refractory status epilepticus

It is suggested that STP, either alone or as add‐on therapy, may prove useful in treating established and BZD‐resistant status epilepticus and may be particularly effective in terminating SE in children when SE is most prevalent.

Stiripentol in Atypical Absence Seizures in Children: An Open Trial

Stiripentol (STP) was added to the antiepileptic drug (AED) regimen of 10 patients with uncontrolled atypical absence seizures (more than one seizure a day) and it is concluded that STP shows promise in treatment of atypicals absence seizures in children.