Dravet syndrome is an early childhood-onset epilepsy syndrome characterized by drug-resistant seizures, frequent episodes of status epilepticus, and the development of neurocognitive impairment. Progressive myoclonic epilepsy in this context presents a particularly refractory management challenge.
The primary clinical objective is reduction in convulsive seizures.
Management involves a combination antiepileptic regimen in which stiripentol is a key component alongside other agents.
Dravet syndrome is an early childhood-onset epilepsy syndrome characterized by drug-resistant seizures, frequent episodes of status epilepticus, and the development of neurocognitive impairment.
Stiripentol administered in conjunction with clobazam and valproate reduced convulsive seizures in Dravet syndrome, but myoclonic seizures were not measured.
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