Stiripentol

The mechanism by which DIACOMIT exerts its anticonvulsant effect in humans is unknown. Possible mechanisms of action include direct effects mediated through the gamma-aminobutyric acid (GABA) A receptor and indirect effects involving inhibition of cytochrome P450 activity with resulting increase in blood levels of clobazam and its active metabolite.

• DIACOMIT is indicated for the treatment of seizures associated with Dravet syndrome (DS) in patients taking clobazam who are 6 months of age and older and weighing 7 kg or more. There are no clinical data to support the use of DIACOMIT as monotherapy in Dravet syndrome.ICD-10: G40.909

• None.contraindicated

The dosage of DIACOMIT is 50 mg/kg/day, administered by mouth in 2 or 3 divided doses, depending on age and weight. ( 2.2 ) Capsules must be swallowed whole with a glass of water during a meal. Capsules should not be broken or opened. ( 2.3 ) Powder for suspension should be mixed in a glass of water and should be taken immediately after mixing during a meal. ( 2.3 ) Reduce dose or discontinue dose gradually. ( 2.5 ) 2.1 Laboratory Tests Prior to First Dose of DIACOMIT Hematologic testing should be obtained prior to starting treatment with DIACOMIT [see Warnings and Precautions (5.3) ]. 2.2 Dosing Information The recommended oral dosage of DIACOMIT is 50 mg/kg/day, administered in 2 or 3 divided doses (i.e., 16.67 mg/kg three times daily or 25 mg/kg twice daily), depending on the patient’s age and body weight as shown in Table 1. If the exact dosage is not achievable given the available strengths, round to the nearest possible dosage, which is usually within 50 mg to 150 mg of the recommended 50 mg/kg/day. A combination of the two DIACOMIT strengths can be used to achieve this dosage. The maximum recommended total dosage is 3,000 mg/day. Table 1.

Somnolence: Monitor for somnolence, particularly when DIACOMIT is used concomitantly with other CNS depressants; If somnolence occurs during co-administration with clobazam, consider an initial reduction of clobazam by 25%. ( 5.1 ) Decreased Appetite and Decreased Weight: the weight of patients and the growth rate of pediatric patients should be carefully monitored. ( 5.2 ) Neutropenia and Thrombocytopenia: Blood counts should be obtained prior to starting treatment with DIACOMIT and then every 6 months. ( 5.3 ) Withdrawal : DIACOMIT should be gradually withdrawn to minimize the risk of increased seizure frequency and status epilepticus. ( 5.4 ) Risks in Patients with Phenylketonuria (PKU) : DIACOMIT for oral suspension contains phenylalanine; consider total daily intake before prescribing to patients with PKU. ( 5.5 ) Suicidal Behavior and Ideation : Monitor for suicidal thoughts or behaviors. ( 5.6 ) 5.1 Somnolence DIACOMIT can cause somnolence. In controlled studies in patients with Dravet syndrome, the incidence of somnolence was 67% in DIACOMIT-treated patients, compared to 23% in patients on placebo. All patients in both groups were on concomitant clobazam, which is also known to cause somnolence. Co-administration of DIACOMIT with clobazam results in increased levels of clobazam and its active metabolite [see Drug Interactions (7.1) ]. Other central nervous system CNS depressants, including alcohol, could potentiate the somnolence effect of DIACOMIT. Prescribers should monitor patients for somnolence.

The following serious or otherwise clinically significant adverse reactions are described elsewhere in the labeling: Somnolence [see Warnings and Precautions (5.1) ] Decreased Appetite and Decreased Weight [see Warnings and Precautions (5.2) ] Neutropenia and Thrombocytopenia [see Warnings and Precautions (5.3) ] Withdrawal Symptoms [see Warnings and Precautions (5.4) ] Risks in Patients with Phenylketonuria [see Warnings and Precautions (5.5) ] Suicidal Behavior and Ideation [see Warnings and Precautions (5.6) ] Adverse reactions that occurred in at least 10% of DIACOMIT-treated patients and more frequently than on placebo were somnolence, decreased appetite, agitation, ataxia, weight decreased, hypotonia, nausea, tremor, dysarthria, and insomnia. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact BIOCODEX at 1-866-330-3050 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. 6.1 Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in clinical trials of another drug, and may not reflect the rates observed in practice. During its development for the treatment of seizures associated with Dravet syndrome, DIACOMIT was administered to 55 healthy male volunteers and 438 patients with Dravet syndrome, including 310 patients treated for 12 months or more.

Pregnancy: Based on animal data, may cause fetal harm. ( 8.1 ) 8.1 Pregnancy Pregnancy Exposure Registry There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to AEDs, such as DIACOMIT, during pregnancy. Physicians are advised to recommend that pregnant patients taking DIACOMIT enroll in the North American Antiepileptic Drug (NAAED) Pregnancy Registry. This can be done by calling the toll free number 1-888-233-2334, and must be done by patients themselves or their caregiver. Information on the registry can also be found at the website http://www.aedpregnancyregistry.org/. Risk Summary There are no adequate data on the developmental risks associated with the use of DIACOMIT in pregnant women. Administration of stiripentol to pregnant animals produced evidence of developmental toxicity, including increased incidences of fetal malformations, increased embryofetal and pup mortality, and decreased embryofetal and pup growth, at maternal doses lower than the recommended clinical dose [see Animal Data ] . The background risk of major birth defects and miscarriage in Dravet syndrome is unknown. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively.

Metabolism

The following pharmacokinetic properties of stiripentol have been found in studies in adult healthy volunteers and adult patients. Systemic exposure of stiripentol increases in a greater than dose proportional manner from 500 mg to 2000 mg.

There are no data concerning overdose in humans. In mice treated with high doses of stiripentol (600 to 1800 mg/kg i.p.), decreased motor activity and decreased respiration were observed. Treatment of an overdose should be supportive (symptomatic measures in intensive care units). For management of a suspected drug overdose, contact your regional Poison Control Center.

• Aug 20, 2018NDANDA206709Biocodex Sa
• Aug 20, 2018NDANDA207223Biocodex Sa

1. 1Seizure79339%
2. 2Product Use In Unapproved Indication34817%
3. 3Off Label Use31215%
4. 4Drug Ineffective26013%
5. 5Decreased Appetite24312%
6. 6Somnolence22011%
7. 7Product Dose Omission Issue1648.1%
8. 8Product Preparation Issue1547.6%
9. 9Fatigue1195.9%
10. 10Pyrexia984.8%
11. 11Weight Decreased944.6%
12. 12Hospitalisation814.0%
13. 13Epilepsy793.9%
14. 14Status Epilepticus763.7%
15. 15Drug Interaction753.7%

The 10 most recently updated of 21 ClinicalTrials.gov registrations naming Stiripentol as an intervention. Registration is not evidence of efficacy or safety — reference crosswalk only.

• Assessment of Safety of the Use of Fenfluramine in Children With Dravet Syndrome Under 24 Months of Age
  Recruiting · Phase 4 · Interventional · 5 enrolled · University of Colorado, Denver
  NCT06598449updated 2026-05-15
• Fenfluramine for Adult Dravet Patients
  Terminated · Phase 3 · Interventional · 7 enrolled · University Health Network, Toronto
  NCT05560282updated 2026-05-05
• A Phase 3 Study to Examine the Efficacy and Safety of ZX008 in Subjects With CDKL5 Deficiency Disorder.
  Active not recruiting · Phase 3 · Interventional · 87 enrolled · Zogenix, Inc.
  NCT05064878updated 2026-04-29
• Treatment Plan to Provide Expanded Access to Stiripentol for Patients With Dravet Syndrome
  Approved for marketing · Expanded access · Children's Hospital Medical Center, Cincinnati
  NCT01983722updated 2026-01-23
• Population Pharmacokinetics of Antiepileptic in Pediatrics
  Completed · Observational · 753 enrolled · Assistance Publique - Hôpitaux de Paris
  NCT03196466updated 2025-11-20
• Bioavailability of Stiripentol After Single Oral Dose of Capsule vs Suspension in Healthy Subjects (STILIQ)
  Completed · Phase 1 · Interventional · 24 enrolled · Biocodex
  NCT07176832updated 2025-09-16
• Pharmacokinetic Parameters of Stiripentol in Renal Impaired Patients and Matching Controls With Normal Renal Function
  Completed · Phase 1 · Interventional · 39 enrolled · Biocodex
  NCT05735951updated 2025-09-05
• Stiripentol for the Treatment of Refractory Status Epilepticus
  Completed · Observational · 25 enrolled · University Hospital Marburg
  NCT06540378updated 2024-08-06
• Retrospective Non-interventional Study of Stiripentol Use in Dravet Patients in the USA
  Completed · Observational · 100 enrolled · Biocodex
  NCT05544058updated 2024-08-02
• Study of Interest of Stiripentol and Carbamazepine in the Treatment of Patients With Pharmacoresistant Focal Epilepsies
  Completed · Phase 4 · Interventional · 52 enrolled · Biocodex
  NCT05419180updated 2024-07-24

Frequently asked questions

How does Stiripentol work?

The mechanism by which DIACOMIT exerts its anticonvulsant effect in humans is unknown. Possible mechanisms of action include direct effects mediated through the gamma-aminobutyric acid (GABA) A receptor and indirect effects involving inhibition of cytochrome P450 activity with resulting increase in blood levels of clobazam and its active metabolite.

What is Stiripentol used for?

According to FDA labeling, Stiripentol carries indications including: DIACOMIT is indicated for the treatment of seizures associated with Dravet syndrome (DS) in patients taking clobazam who are 6 months of age and older and weighing 7 kg or more. There are no clinical data to support the use of DIACOMIT as monotherapy in Dravet syndrome.. This is a reference summary of labeled uses, not medical advice or a treatment recommendation.

What class of drug is Stiripentol?

Stiripentol is classified as Other antiepileptics, GABA A Agonists, Increased Central Nervous System GABA Activity.

What are the brand names for Stiripentol?

Stiripentol is marketed under brand names including Diacomit.

What are the contraindications for Stiripentol?

Stiripentol labeling lists contraindications including: None.. Always consult the full prescribing information and a clinician.