Stiripentol for seizures with Dravet syndrome

The US Food and Drug Administration (FDA) has approved the anticonvulsant drug stiripentol (Diacomit, Biocodex) for the treatment of seizures associated with Dravet syndrome, the agency has announced.

Stiripentol is indicated for patients aged 2 or older who are taking clobazam. No clinical data support its use as monotherapy in Dravet syndrome, the FDA said. 

The most common side effects reported with stiripentol are somnolence, decreased appetite, agitation, ataxia, weight loss, hypotonia, nausea, tremor, dysarthria, and insomnia.

Stiripentol must be dispensed with a patient Medication Guide that describes important information about the drug's uses and risks. As is true for many other antiepileptic agents, the most serious risks include suicidality, agitation, new or worsening depression, aggression, and panic attacks, the FDA said.

The dosage of stiripentol is 50 mg/kg/day, administered by mouth in two or three divided doses. Full prescribing information for stiripentol is available online.  

In June, as reported by Medscape Medical News, the FDA approved the first cannabis drug for Dravet syndrome and another rare form of epilepsy, Lennox-Gastaut syndrome.

Dravet syndrome is a rare genetic condition that usually appears in the first year of life with prolonged febrile seizures, followed often by other types of seizures, including myoclonic seizures. Patients with Dravet syndrome may also develop status epilepticus, a potentially life-threatening state of continuous seizure activity requiring emergency medical care.

https://www.medscape.com/viewarticle/901000

See:  http://childnervoussystem.blogspot.com/2017/05/stiripentol-in-dravet-syndrome.html