The International League Against Epilepsy Classification and Definitions of Epilepsy Syndromes with Onset in Neonates and Infants

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The International League Against Epilepsy (ILAE) Task Force proposed a classification system containing definitions of epilepsy syndromes with onset in neonates and infants up to 2 years of age. Concordant with the 2017 ILAE Framework for Classification of the Epilepsies, this new classification emphasized the importance of classifying epilepsy both by electroclinical features and etiology. This classification was developed to clearly define a uniform group of each epilepsy syndrome and to support precision therapies for specific genetic factors. The ILAE Task Force classified epilepsy syndromes into two major categories; self-limited epilepsies and developmental and epileptic encephalopathies. Self-limited epilepsies include syndromes that are likely to show spontaneous remission, while developmental and epileptic encephalopathies are characterized by developmental impairment related to both the underlying etiology independent of epileptiform discharges and the epileptic encephalopathy. Etiology-specific epilepsy syndromes include disorders due to specific genetic, structural, and metabolic etiologies. The ILAE Task Force presented mandatory features, alerts, and exclusionary features for each epilepsy syndrome. Clinicians need to consider both the electroclinical features and the etiology when defining epilepsy syndromes and developing a management plan for each individual. Key words: Epilepsy syndromes; Benign neonatal epilepsies; Infantile spasms; Severe myoclonic epilepsy in infancy.