When first- or second-line therapy in Dravet syndrome has not prevented prolonged convulsive seizures or status epilepticus, a structured escalation pathway guides the next clinical step.
This protocol applies after clobazam, fenfluramine, or stiripentol — used as first- or second-line therapy — has not achieved the primary goal of avoiding prolonged convulsive seizures and status epilepticus.
The central target for this next-line protocol remains the avoidance of prolonged convulsive seizures and status epilepticus.
The protocol involves pharmaceutical-grade cannabidiol, which carries evidence supporting its use at specific points in the treatment sequence — the full regimen, sequencing, and eligibility criteria are detailed in the complete protocol.
DOI: 10.1111/epi.17274
Pharmaceutical-grade cannabidiol was supported either as first- or second-line treatment (caregivers: Strong; physicians: no consensus).
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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