Specchio, Nicola

Wirrell, Elaine C

Scheffer, Ingrid E

Nabbout, Rima

Riney, Kate

Samia, Pauline

Guerreiro, Marilisa

Gwer, Sam

Zuberi, Sameer M

Wilmshurst, Jo M

Yozawitz, Elissa

Pressler, Ronit

Hirsch, Edouard

Wiebe, Sam

Cross, Helen J

Perucca, Emilio

Moshé, Solomon L

Tinuper, Paolo

Auvin, Stéphane

2022-06

The 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.

Epilepsia 2022; 63(6): 1398-1442

Epilepsia

https://orcid.org/0000-0002-8120-0287

https://orcid.org/0000-0003-3015-8282

https://orcid.org/0000-0002-2311-2174

https://orcid.org/0000-0001-5877-4074

https://orcid.org/0000-0002-1122-3555

https://orcid.org/0000-0002-7427-0439

https://orcid.org/0000-0001-7328-1796

https://orcid.org/0000-0001-8230-8364

https://orcid.org/0000-0002-2905-6839

https://orcid.org/0000-0003-0833-8850

https://orcid.org/0000-0002-1061-9099

https://orcid.org/0000-0001-7345-4829

https://orcid.org/0000-0001-8703-223X

https://orcid.org/0000-0001-9427-9476

https://orcid.org/0000-0003-3874-9749

https://ahro.austin.org.au/austinjspui/handle/1/30341

Landau-Kleffner syndrome

Lennox-Gastaut syndrome

Panayiotopoulos syndrome

benign occipital epilepsy

childhood epilepsy with centrotemporal spikes

continuous spike-and-wave in sleep

eyelid myoclonia

febrile infection-related epilepsy syndrome

hemiconvulsion-hemiplegia-epilepsy syndrome

myoclonic absences

myoclonic atonic

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.

Journal Article