Dravet Syndrome – Cannabidiol and SCN1A — SCE Neurology MCQ

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HardEpilepsy & Seizure DisordersDravet Syndrome – Cannabidiol and SCN1ASCE Neurology

A 30-year-old man with Dravet syndrome (severe myoclonic epilepsy of infancy) confirmed by SCN1A mutation has drug-resistant seizures. What medication is specifically approved for Dravet syndrome?

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Correct answer: C — Cannabidiol (Epidyolex) — approved as adjunctive therapy for seizures associated with Dravet syndrome

Cannabidiol (CBD, Epidyolex) has been approved by NICE (TA614) as adjunctive therapy for seizures in Dravet syndrome and Lennox-Gastaut syndrome. The CARE trial demonstrated significant reduction in convulsive seizure frequency. Importantly, sodium channel blockers (carbamazepine, phenytoin, lamotrigine, oxcarbazepine) are CONTRAINDICATED in Dravet syndrome as they can worsen seizures — Dravet is caused by loss-of-function SCN1A mutations, and further sodium channel blockade exacerbates the deficit. Standard treatments include valproate, clobazam, stiripentol, and fenfluramine. A: Carbamazepine worsens Dravet seizures. C: Phenytoin worsens Dravet. D: Gabapentin is ineffective. E: Vigabatrin is for infantile spasms/TSC.

Reference: NICE TA614 Cannabidiol for Dravet (2019); BNF