International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions
hemiconvulsion-hemiplegia-epilepsy syndrome
Molecular Mechanisms of Synaptic Plasticity and Neurological Disorders
Lennox-Gastaut syndrome
Epilepsies, Myoclonic
febrile infection-related epilepsy syndrome
Pediatrics
Childhood absence epilepsy
618
Semiology
Cellular and Molecular Neuroscience
03 medical and health sciences
0302 clinical medicine
Seizures
Epilepsy and Seizures
Biochemistry, Genetics and Molecular Biology
616
Health Sciences
Genetics
Humans
Psychology
Child
continuous spike-and-wave in sleep
myoclonic absences
myoclonic atonic
Psychiatry
childhood epilepsy with centrotemporal spikes
Epilepsy
Generalized epilepsy
Life Sciences
Epilepsy syndromes
Electroencephalography
Landau-Kleffner syndrome
Panayiotopoulos syndrome
3. Good health
benign occipital epilepsy
FOS: Psychology
Psychiatry and Mental health
Epilepsy, Absence
FOS: Biological sciences
eyelid myoclonia
Medicine
Molecular Basis of Rett Syndrome and Related Disorders
Epilepsies, Partial
Neuroscience
DOI:
10.1111/epi.17241
Publication Date:
2022-05-03T16:50:06Z
AUTHORS (19)
ABSTRACT
AbstractThe 2017 International League Against Epilepsy classification has defined a three‐tier system with epilepsy syndrome identification at the third level. Although a syndrome cannot be determined in all children with epilepsy, identification of a specific syndrome provides guidance on management and prognosis. In this paper, we describe the childhood onset epilepsy syndromes, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features. Based on the 2017 Classification of Seizures and Epilepsies, some syndrome names have been updated using terms directly describing the seizure semiology. Epilepsy syndromes beginning in childhood have been divided into three categories: (1) self‐limited focal epilepsies, comprising four syndromes: self‐limited epilepsy with centrotemporal spikes, self‐limited epilepsy with autonomic seizures, childhood occipital visual epilepsy, and photosensitive occipital lobe epilepsy; (2) generalized epilepsies, comprising three syndromes: childhood absence epilepsy, epilepsy with myoclonic absence, and epilepsy with eyelid myoclonia; and (3) developmental and/or epileptic encephalopathies, comprising five syndromes: epilepsy with myoclonic–atonic seizures, Lennox–Gastaut syndrome, developmental and/or epileptic encephalopathy with spike‐and‐wave activation in sleep, hemiconvulsion–hemiplegia–epilepsy syndrome, and febrile infection‐related epilepsy syndrome. We define each, highlighting the mandatory seizure(s), EEG features, phenotypic variations, and findings from key investigations.
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