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

Nicola Specchio¹, Elaine C Wirrell², Ingrid E Scheffer³, Rima Nabbout⁴, Kate Riney^{5

6}, Pauline Samia⁷, Marilisa Guerreiro⁸, Sam Gwer⁹, Sameer M Zuberi¹⁰, Jo M Wilmshurst¹¹, Elissa Yozawitz¹², Ronit Pressler¹³, Edouard Hirsch¹⁴, Sam Wiebe¹⁵, Helen J Cross¹⁶, Emilio Perucca^{17

18}, Solomon L Moshé¹⁹, Paolo Tinuper^{20

21}, Stéphane Auvin²²

Affiliations

¹ Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, Scientific Institute for Research and Health Care, Full Member of EpiCARE, Rome, Italy.
² Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
³ Austin Health and Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Victoria, Australia.
⁴ Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Sick Children Hospital, Public Hospital Network of Paris, member of EpiCARE, Imagine Institute, National Institute of Health and Medical Research, Mixed Unit of Research 1163, University of Paris, Paris, France.
⁵ Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
⁶ Faculty of Medicine, University of Queensland, South Brisbane, Queensland, Australia.
⁷ Department of Pediatrics and Child Health, Aga Khan University, Nairobi, Kenya.
⁸ Department of Neurology, University of Campinas, Campinas, Brazil.
⁹ School of Medicine, Kenyatta University, and Afya Research Africa, Nairobi, Kenya.
¹⁰ Paediatric Neurosciences Research Group, Royal Hospital for Children and Institute of Health & Wellbeing, member of EpiCARE, University of Glasgow, Glasgow, UK.
¹¹ Department of Paediatric Neurology, Red Cross War Memorial Children's Hospital, Neuroscience Institute, University of Cape Town, Cape Town, South Africa.
¹² Isabelle Rapin Division of Child Neurology of the Saul R. Korey Department of Neurology, Montefiore Medical Center, Bronx, New York, USA.
¹³ Programme of Developmental Neurosciences, University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health, Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children, London, UK.
¹⁴ Neurology Epilepsy Units "Francis Rohmer", INSERM 1258, FMTS, Strasbourg University, Strasbourg, France.
¹⁵ Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
¹⁶ Programme of Developmental Neurosciences, University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children, and Young Epilepsy Lingfield, London, UK.
¹⁷ Department of Neuroscience, Monash University, Melbourne, Victoria, Australia.
¹⁸ Department of Medicine, Austin Health, University of Melbourne, Heidelberg, Victoria, Australia.
¹⁹ Isabelle Rapin Division of Child Neurology, Saul R. Korey Department of Neurology, and Departments of Neuroscience and Pediatrics, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA.
²⁰ Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
²¹ Institute of Neurological Sciences, Scientific Institute for Research and Health Care, Bologna, Italy.
²² Robert Debré Hospital, Public Hospital Network of Paris, NeuroDiderot, National Institute of Health and Medical Research, Department Medico-Universitaire Innovation Robert-Debré, Pediatric Neurology, University of Paris, Paris, France.

PMID: 35503717
DOI: 10.1111/epi.17241

Free article

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

Nicola Specchio et al. Epilepsia. 2022 Jun.

Free article

. 2022 Jun;63(6):1398-1442.

doi: 10.1111/epi.17241. Epub 2022 May 3.

Authors

Affiliations

¹ Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, Scientific Institute for Research and Health Care, Full Member of EpiCARE, Rome, Italy.
² Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
³ Austin Health and Royal Children's Hospital, Florey Institute, Murdoch Children's Research Institute, University of Melbourne, Melbourne, Victoria, Australia.
⁴ Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Sick Children Hospital, Public Hospital Network of Paris, member of EpiCARE, Imagine Institute, National Institute of Health and Medical Research, Mixed Unit of Research 1163, University of Paris, Paris, France.
⁵ Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
⁶ Faculty of Medicine, University of Queensland, South Brisbane, Queensland, Australia.
⁷ Department of Pediatrics and Child Health, Aga Khan University, Nairobi, Kenya.
⁸ Department of Neurology, University of Campinas, Campinas, Brazil.
⁹ School of Medicine, Kenyatta University, and Afya Research Africa, Nairobi, Kenya.
¹⁰ Paediatric Neurosciences Research Group, Royal Hospital for Children and Institute of Health & Wellbeing, member of EpiCARE, University of Glasgow, Glasgow, UK.
¹¹ Department of Paediatric Neurology, Red Cross War Memorial Children's Hospital, Neuroscience Institute, University of Cape Town, Cape Town, South Africa.
¹² Isabelle Rapin Division of Child Neurology of the Saul R. Korey Department of Neurology, Montefiore Medical Center, Bronx, New York, USA.
¹³ Programme of Developmental Neurosciences, University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health, Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children, London, UK.
¹⁴ Neurology Epilepsy Units "Francis Rohmer", INSERM 1258, FMTS, Strasbourg University, Strasbourg, France.
¹⁵ Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
¹⁶ Programme of Developmental Neurosciences, University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children, and Young Epilepsy Lingfield, London, UK.
¹⁷ Department of Neuroscience, Monash University, Melbourne, Victoria, Australia.
¹⁸ Department of Medicine, Austin Health, University of Melbourne, Heidelberg, Victoria, Australia.
¹⁹ Isabelle Rapin Division of Child Neurology, Saul R. Korey Department of Neurology, and Departments of Neuroscience and Pediatrics, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA.
²⁰ Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
²¹ Institute of Neurological Sciences, Scientific Institute for Research and Health Care, Bologna, Italy.
²² Robert Debré Hospital, Public Hospital Network of Paris, NeuroDiderot, National Institute of Health and Medical Research, Department Medico-Universitaire Innovation Robert-Debré, Pediatric Neurology, University of Paris, Paris, France.

PMID: 35503717
DOI: 10.1111/epi.17241

Abstract

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.

Keywords: 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.

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References

REFERENCES

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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

Affiliations

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

Authors

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical

Molecular Biology Databases