Valproic acid is the established first-line antiseizure medication in Dravet syndrome. When it fails to prevent prolonged convulsive seizures or status epilepticus, a defined next step is indicated. The evidence-based protocol for this situation is outlined below.
Initial therapy with valproic acid — with or without clobazam as an initial add-on — has not achieved the primary goals: avoidance of prolonged convulsive seizures and status epilepticus. This unmet target is the recognised trigger for escalation to the next protocol.
The evidence-based next step calls for adding or switching to one of a small set of antiseizure agents that carry specific evidence for use in Dravet syndrome at the first- or second-line stage. The complete protocol — covering agent selection, sequencing, and conditions for use — is available via the link below.
The primary aim remains avoidance of prolonged convulsive seizures and status epilepticus. Infrequent, brief convulsive seizures may represent an acceptable outcome during optimisation.
There was consensus for use of valproic acid as first-line therapy, and for use of clobazam, fenfluramine, or stiripentol as first-or second-line therapy.
Further consensus for other first-line therapies included fenfluramine (physicians: Strong; caregivers: Moderate), and stiripentol (physicians: Moderate; caregivers: Strong).
In DS, it is appropriate to accept infrequent, brief convulsive seizures with the main goal focused on avoiding prolonged convulsive seizures and status epilepticus (physicians: n = 19, 79%; caregivers: n = 9, 56%).
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