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Dravet Syndrome — RACP Paediatrics MCQ

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HardNeurology & DevelopmentDravet SyndromeRACP Paediatrics

A 3-year-old boy with severe refractory epilepsy beginning at age 6 months has fever-sensitive seizures, developmental regression, and an SCN1A mutation. What is the diagnosis?

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Correct answer: B — Dravet syndrome

Dravet syndrome (severe myoclonic epilepsy of infancy) is caused by SCN1A mutations (usually de novo). It presents with prolonged febrile seizures in the first year, then develops multiple seizure types with developmental regression. Sodium channel blockers (carbamazepine, lamotrigine, phenytoin) are contraindicated as they worsen seizures.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Epileptic Encephalopathies