Childhood absence epilepsy
ICD-10 G40.3 · ICD-11 8A61.21

Childhood Absence Epilepsy with Absence Seizures Only — After Prior Therapy Has Failed

This protocol applies to children with childhood absence epilepsy whose only seizure type is absence seizures, with no history of generalized tonic-clonic seizures, and who have not achieved the treatment goal on a previous regimen.

The preceding regimen used either a combination of valproic acid (VPA) and lamotrigine (LTG), or clobazam as monotherapy. Despite this treatment, freedom from absence seizures was not achieved, necessitating escalation to the next line.

Freedom from absence seizures

At this stage, certain oral antiseizure medications or a specialized dietary intervention may be considered — the complete selection criteria, sequencing, and management approach are contained in the full protocol.

References

The treatment of choice for CAE with absence seizures only is ethosuximide.

Therefore, based on the CAE trial, ETX is the drug of choice as initial monotherapy for CAE, when absence seizures are the only seizure type, but there are specific treatment considerations for each drug, which are discussed below.

Although the evidence for efficacy in CAE is scant and variably supportive, topiramate and zonisamide may be considered when other treatments fail.

The ketogenic diet has also been used successfully in children with treatment-resistant CAE.

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