KCNQ2-epileptic encephalopathy (EE) usually manifests as a neonatal epilepsy syndrome characterized by tonic episodes, focal clonic activity, and autonomic changes [1]. Early EEG recordings typically show a burst-suppression pattern or a multifocal epileptiform activity [1]. Basal ganglia hyperintensities may be found at early brain MRI. A large majority of children with KCNQ2-EE become seizure-free before three years of age, but developmental impairment remains severe in two thirds of the patients.
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