Stiripentol
Mechanism :
Exact mechanism is not known. May enhance GABAergic inhibitory neurotransmission by weak partial agonism and/or positive allosteric modulation of gamma-aminobutyric acid (GABA)-A receptors. It also inhibits multiple cytochrome P450 isoenzymes involved in the metabolism of other anticonvulsants
Indication :
- Dravet syndrome: Adjunctive treatment in conjunction with clobazam and valproic acid in patients with severe myoclonic epilepsy in infancy.
Contraindications :
Hypersensitivity to stiripentol or any component of the formulation.
Dosing :
Children <3 years:
Use under close monitoring; no specific dosing.
Children >3 years:
Oral:
Initial: Titrate dose upward over 3 days to 50 mg/kg daily given in 2 or 3 divided doses. Maximum daily dose: 50 mg/kg.
Use only in conjunction with
clobazam and valproate.
Adverse Effect :
Drowsiness, agitation, ataxia, hypotonia, dysarthria, insomnia, weight loss/gain, decreased appetite, nausea, thrombocytopenia, neutropenia, tremor, fatigue, vomiting, sialorrhea, fever.
Interaction :
Caffeine and Caffeine Containing Products: Stiripentol may increase the serum concentration of Caffeine and Caffeine Containing Products.
Carbamazepine: Stiripentol may increase the serum concentration of Carbamazepine.
CNS Depressants: May enhance the adverse/toxic effect of other CNS Depressants.
CYP1A2 Inducers: May decrease the serum concentration of CYP1A2 Substrates
CYP1A2 Inhibitors: May decrease the metabolism of CYP1A2 Substrates
CYP1A2 Substrates: CYP1A2 Inhibitors may decrease the metabolism of CYP1A2 Substrates.
CYP2C19 Inducers: May increase the metabolism of CYP2C19 Substrates
CYP2C19 Inhibitors: May decrease the metabolism of CYP2C19 Substrates
CYP3A4 Inducers: May increase the metabolism of CYP3A4 Substrates
CYP3A4 Inhibitors: May decrease the metabolism of CYP3A4 Substrates
CYP3A4 Substrates: Stiripentol may increase the serum concentration of CYP3A4 Substrates
Opioid Analgesics: CNS Depressants may enhance the CNS depressant effect of Opioid Analgesics.
Selective Serotonin Reuptake Inhibitors: CNS Depressants may enhance the adverse/toxic effect of Selective Serotonin Reuptake Inhibitors.
Hepatic Dose :
Children =3 years and Adolescents: Initial dose: No dosage adjustment necessary. Use with caution and titrate dose per seizure control and tolerability