In epilepsy with myoclonic atonic seizures, treatment is escalated stepwise. When a second-line regimen has been trialled adequately and the patient has not reached the expected seizure reduction or seizure freedom, a defined third-line approach is indicated.
This third-line protocol is indicated after second-line therapy — including ethosuximide, the combination of valproate and ethosuximide, or the ketogenic diet — has not achieved a greater than 50% reduction in seizures or seizure freedom. The failure of these second-line options, whether used individually or in combination, is the trigger for escalation.
The Delphi consensus recommended valproate and benzodiazepines (either alone or in combination) and the ketogenic diet for treatment during the stormy phase; steroids can also be considered.
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