LuciStir (Stiripentol)

About Stiripentol

Stiripentol is an anticonvulsant medication used for the treatment of Dravet syndrome - a serious genetic brain disorder.

In the European Union, stiripentol is indicated for use in conjunction with clobazam and valproate as adjunctive therapy of refractory generalized tonic-clonic seizures in people with severe myoclonic epilepsy in infancy (SMEI, Dravet's syndrome) whose seizures are not adequately controlled with clobazam and valproate.

In the United States, stiripentol is indicated for the treatment of seizures associated with Dravet syndrome in people two years of age and older taking clobazam. There are no clinical data to support the use of stiripentol as monotherapy in Dravet syndrome.

It is used in some countries as an add-on therapy with sodium valproate and clobazam for treating children with Dravet syndrome whose seizures are not adequately controlled. As of 2017, it was not known whether stiripentol remains useful as children become adolescents or adults.

Seizures

Indicated for seizures associated with Dravet syndrome in patients taking clobazam

There are no clinical data to support the use of stiripentol as monotherapy in Dravet syndrome

50 mg/kg/day PO administered in 2 or 3 divided doses (ie, 16.67 mg/kg TID or 25 mg/kg BID); not to exceed 3000 mg/day

Round to the nearest possible dosage (usually within 50-150 mg of the recommended 50 mg/kg/day)

If the exact dosage is not achievable given the available strengths, a combination of the 2 strengths can be used to achieve this dosage

Dosage Modifications

Renal and hepatic impairment

• There is no formal study of the pharmacokinetics and metabolism in patients with renal or hepatic impairment
• Since metabolites are eliminated mainly through the kidney and metabolized by the liver, administration to patients with moderate or severe renal or hepatic impairment is not recommended

Dosing Considerations

Prior to starting treatment, obtain a hematologic test

Gradual withdrawal

• As advised for most antiepileptic drugs (AEDs), if treatment is discontinued, gradually withdraw stiripentol to minimize the risk of increased seizure frequency and status epilepticus
• In situations in which rapid withdrawal is medically required, appropriate monitoring is recommended