Stiripentol add-on therapy for focal refractory epilepsy

Brigo, Francesco; Igwe, Stanley C.; Bragazzi, Nicola Luigi

doi:10.1002/14651858.cd009887.pub4

Cited by 11 publications

(9 citation statements)

References 21 publications

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“…30 Although preliminary studies suggest that stiripentol might also be of benefit in refractory focal epilepsy, the lack of prospective controlled trials prevents the development of recommendations regarding its use in these conditions unless standard therapies have failed or are not tolerated. 31…”

Section: Clinical Trialsmentioning

confidence: 99%

Stiripentol: A Novel Antiseizure Medication for the Management of Dravet Syndrome

Buck

Goodkin

2019

Ann Pharmacother

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Objective: To describe the pharmacology, efficacy, and safety of stiripentol in the treatment of refractory seizures in patients with Dravet syndrome. Data Sources: A search of the English language literature was conducted using PubMed and MEDLINE (1978 to April 2019) with the search terms stiripentol, Dravet syndrome, and refractory epilepsy. Other resources included article bibliographies, prescribing information, and relevant trials at https://clinicaltrials.gov/ . Study Selection and Data Extraction: All phase 1, 2, or 3 trials; observational studies; and retrospective studies were analyzed. Data Synthesis: In controlled studies, stiripentol has been shown to reduce seizure frequency by 50% or more in 40% to 70% of patients with Dravet syndrome. Reductions in seizure duration and episodes of status epilepticus have also been documented. Common adverse effects include somnolence and anorexia. Stiripentol inhibits the metabolism of clobazam and valproate, often requiring dose adjustment. Relevance to Patient Care and Clinical Practice: Stiripentol, a direct allosteric modulator of GABAA receptors, offers a novel approach to treatment in patients with Dravet syndrome, both with and without pathogenic variants of the sodium channel α-1 subunit gene, and potentially other refractory seizures. Although available outside the United States for a decade, it was only recently approved by the Food and Drug Administration for patients 2 years of age and older with Dravet syndrome taking clobazam. Conclusions: Stiripentol is an effective adjunctive therapy for reducing the frequency and duration of refractory seizures in patients with Dravet syndrome. Its role in the treatment of other refractory epilepsies requires further study.

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Section: Clinical Trialsmentioning

confidence: 99%

Stiripentol: A Novel Antiseizure Medication for the Management of Dravet Syndrome

Buck

Goodkin

2019

Ann Pharmacother

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“…Perampanel (PER) and stiripentol (STP) are two recently approved third-generation antiepileptic drugs both presenting unique chemical structures ( Figure 1). STP is an orphan drug that has been specifically approved in children aging >1 year old as adjunctive therapy in the Dravet syndrome [1][2][3][4], being its use as add-on therapy in focal refractory epilepsy also studied over the past years [5].…”

Section: Introductionmentioning

confidence: 99%

“…Perampanel (PER) and stiripentol (STP) are two recently approved third‐generation antiepileptic drugs both presenting unique chemical structures (Figure 1). STP is an orphan drug that has been specifically approved in children aging >1 year old as adjunctive therapy in the Dravet syndrome [1–4], being its use as add‐on therapy in focal refractory epilepsy also studied over the past years [5]. On the other hand, PER is the first drug that specifically modulates AMPA (α‐amino‐3‐hydroxy‐5‐methylisoxazole‐4‐propionic acid) receptors of glutamate (i.e., the most abundant excitatory neurotransmitter), thus being a new hope for epilepsy treatment, particularly for the patients that are resistant to other antiepileptic drugs.…”

Section: Introductionmentioning

confidence: 99%

Salting‐out assisted liquid–liquid extraction method optimized by design of experiments for the simultaneous high‐performance liquid chromatography analysis of perampanel and stiripentol in mouse matrices

Meirinho

Campos

Rodrigues

et al. 2020

J of Separation Science

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We report a high-performance liquid chromatography method development able to simultaneously determine perampanel and stiripentol, two third-generation antiepileptics whose therapeutic spectrum can potentially be extended, in several mouse matrices. A salting-out assisted liquid-liquid extraction optimized by a design of experiments approach was adopted for sample preparations. Isopropanol and magnesium sulfate were the extraction solvent and salting-out agent, respectively. Both drugs and internal standard (terbinafine) were separated using a LiChroCART R Purospher Star column (C 18 , 55 × 4 mm; 3 μm) isocratically pumped with mobile phase [1% triethylamine in water (pH 2.5) and acetonitrile (53:47, v/v)] at 1 mL/min. Stiripentol and terbinafine were detected by fluorescence at 254/372 nm and perampanel at 275/430 nm. Good linearity was demonstrated for perampanel at 1-500 ng/mL range in brain, 2-2000 ng/mL in liver and 1-2000 ng/mL in plasma and kidney (r 2 ≥ 0.9922), and for stiripentol between 10 and 2000 ng/mL in brain and 10 and 20 000 ng/mL in the remaining matrices (r 2 ≥ 0.9917). Precision (CV ≤ 15%) and accuracy (bias ±15%) were also verified, with obtained recovery values consistent with those predicted by the experimental design. This method was applied in preliminary pharmacokinetic studies to quantify perampanel or stiripentol after oral administration to mice, showing to be a promising bioanalytical tool to support future nonclinical in vivo pharmacokinetic studies.

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“…Although stiripentol levels were in therapeutic range, Pox did only decrease as expected with dialysis and pyridoxine ( Figure 2 ). Thus, we stopped stiripentol medication to avoid side effects as described for other indication [ 4 ].…”

mentioning

confidence: 99%

Is stiripentol truly effective for treating primary hyperoxaluria?

Martín-Higueras

Feldkötter

Höppe

2020

Clinical Kidney Journal

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Stiripentol add-on therapy for focal refractory epilepsy

Abstract: Editorial group: Cochrane Epilepsy Group. Publication status and date: New search for studies and content updated (no change to conclusions), published in Issue 5, 2018.

Cited by 11 publications

References 21 publications

Stiripentol: A Novel Antiseizure Medication for the Management of Dravet Syndrome

Stiripentol: A Novel Antiseizure Medication for the Management of Dravet Syndrome

Salting‐out assisted liquid–liquid extraction method optimized by design of experiments for the simultaneous high‐performance liquid chromatography analysis of perampanel and stiripentol in mouse matrices

Is stiripentol truly effective for treating primary hyperoxaluria?

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