Stiripentol | Epilepsy clinician handbook
- For use in Dravet syndrome (DS) as adjunctive therapy to at least two other anti-seizure medications (particularly clobazam with valproate).
- Available on PBS for use in patients with DS with generalised tonic-clonic seizures or generalised clonic seizures that are not adequately controlled by at least two other anti-seizure medications.
Possible side effects
- Anorexia
- Weight loss, especially when administered with sodium valproate
- Drowsiness
- Ataxi
- Hypotonicity
- Dystonia, tremor
- Insomnia
- Fatigue
- Nausea and vomiting
Other notable side effects:
- Neutropenia
- Altered behaviour: aggressiveness and irritability
- Hyperkinesia
- Raised Gamma GT notably when combined with valproate
Dangerous side effects:
- Cutaneous photosensitivity, rash, urticaria
- All anti-seizure medications are potentially teratogenic and this is often dose related
Interactions and precautions
Because of inhibitory interactions with clobazam and valproate, the daily dosage of these anticonvulsants may need to be reduced by 25-30%. The impetus for reduction in valproate dosage usually relates to gastrointestinal adverse effects, such as loss of appetite and weight loss with combined therapy.
Carbamazepine, phenytoin and phenobarbitone should generally not be used in conjunction with stiripentol for DS, due to drug interaction and the potential to worsen seizures. Approximately two-to three-fold increases in clobazam and five-fold increases in norclobazam plasma levels respectively have been reported with co-administration of stiripentol in children with DS.
Because stiripentol is a potent inhibitor of hepatic enzymes, the following drug combinations require particular caution:
- Ergot alkaloids
- Immunosuppressants (tacrolimus, cyclosporine, sirolimus): nephrotoxicity.
- Statins: rhabdomyolysis
- Theophylline, caffeine toxicity.
Potential teratogen.
History of delirium: contraindicated.
The manufacturer recommends avoiding use in renal and hepatic impairment.