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Dravet syndrome — SCE Neurology MCQ

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HardEpilepsyDravet syndromeSCE Neurology

A 4-year-old with genetically confirmed Dravet syndrome continues to have convulsive seizures despite adequate sodium valproate. Which is the most appropriate first add-on regimen?

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Correct answer: CAdd stiripentol with clobazam while continuing sodium valproate

For Dravet syndrome in which sodium valproate alone is unsuccessful, NICE recommends stiripentol with clobazam as the first add-on treatment. Sodium-channel blockers such as carbamazepine may worsen seizures in Dravet syndrome. Ethosuximide is for absence epilepsy and does not address this treatment-resistant convulsive phenotype.

Reference: NICE NG217: treating childhood-onset epilepsies: https://www.nice.org.uk/guidance/ng217/chapter/treating-childhood-onset-epilepsies