Epilepsy syndromes: the 2022 ILAE definition and classification
https://doi.org/10.17749/2077-8333/epi.par.con.2022.123
Abstract
Until recently, no classification of epileptic syndromes officially approved by the International League Against Epilepsy (ILAE) was available. In 2022, owing to many year-persistent efforts made by experts and the public community, the studies providing the definition and classification of epileptic syndromes were published. ILAE has approved the following definition of the epileptic syndrome: “a characteristic cluster of clinical and EEG features, often supported by specific etiological findings (structural, genetic, metabolic, immune, and infectious)”. The classification of epileptic syndromes is proposed according to the age principle: onset in neonates and infants, onset in childhood, onset at a variable age. Separately, the syndromes of idiopathic generalized epilepsy were presented. Clinical data for each epileptic syndrome are presented in a single pattern: epidemiology, clinical context, natural history, type(s) of seizures, electroencephalography, neuroimaging findings, genetic findings, other laboratory findings (when informative), differential diagnosis. The criteria for diagnosis, including mandatory criteria, warning signs and exclusion criteria, are given. This classification should be a starting point for further improvement in the organizing work of practitioners who deal with epilepsy.
About the Author
D. V. Blinov
Institute for Preventive and Social Medicine; Lapino Clinical Hospital, Medical Group “Mother and Child”
Russian Federation
Russian Federation
Dmitry V. Blinov – MD, PhD, MBA, Head of Medical and Scientific Affairs; Neurologist
WoS ResearcherID: J-4946-2017;
Scopus Author ID: 7003589812;
RSCI SPIN-code: 6317-98334/10
Sadovaya-Triumfalnaya Str., Moscow 127006
111 1st Uspenskoye Hwy, Moscow Region, Odintsovo District, Lapino 143081
References
1. Karlov V.A. Epilepsy in children and adult women and men. A guide for doctors. 2nd ed. Moscow: BINOM; 2019: 806 pp. (in Russ.).
2. Hauser W.A., Annegers J.F., Kurland L.T. Incidence of epilepsy and unprovoked seizures in Rochester, Minnesota: 1935–1984. Epilepsia. 1993; 34 (3): 453–68. https://doi.org/10.1111/j.1528-1157.1993.tb02586.x.
3. Epilepsy: a public health imperatives. Geneva: World Health Organization, 2019. Available at: https://www.who.int/publications-detail-redirect/epilepsy-a-public-health-imperative (accessed 07.06.2022).
4. Karlov V.A. Russian League Against Epilepsy: the challenge of time. Epilepsia i paroksizmalʹnye sostoania / Epilepsy and Paroxysmal Conditions. 2020; 12 (1): 5 (in Russ.).
5. Karlov V.A. What has been achieved and what has not been achieved in 2020. Epilepsia i paroksizmalʹnye sostoania / Epilepsy and Paroxysmal Conditions. 2021; 13 (1): 5 (in Russ.).
6. Karlov V.A. Russian League Against Epilepcy: the answer to the present challenge. Epilepsia i paroksizmalʹnye sostoania / Epilepsy and Paroxysmal Conditions. 2021; 13 (1S): S88–90 (in Russ.). https://doi.org/10.17749/2077-8333/epi.par.con.2021.085.
7. Political declaration of the third high-level meeting of the General Assembly on the prevention and control of noncommunicable diseases. Geneva: World Health Organization; 2022. Available at: https://apps.who.int/gb/ebwha/pdf_files/EB150/B150_7-en.pdf (accessed 07.06.2022).
8. Draft Intersectoral global action plan on epilepsy and other neurological disorders 2022–2031. World Health Organization; 2021. Available at: https://www.who.int/news/item/28-04-2022-draft-intersectoral-global-action-plan-on-epilepsy-and-other-neurological-disorders-2022-2031 (accessed 07.06.2022).
9. Fisher R.S., Cross J.H., French J.A., et al. Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58 (4): 522–30. https://doi.org/10.1111/epi.13670.
10. Scheffer I.E., Berkovic S., Capovilla G., et al. ILAE classification of the epilepsies: position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58 (4): 512–21. https://doi.org/10.1111/epi.13709.
11. Avakyan G.N., Blinov D.V., Lebedeva A.V., et al. ILAE Classification of the Epilepsies: the 2017 revision and update. Epilepsia i paroksizmalʹnye sostoania / Epilepsy and Paroxysmal Conditions. 2017; 9 (1): 6–25 (in Russ.). https://doi.org/10.17749/2077-8333.2017.9.1.006-025.
12. Wirrell E., Tinuper P., Perucca E., et al. Introduction to the epilepsy syndrome papers. Epilepsia. 2022; 63 (6): 1330–2. https://doi.org/10.1111/epi.17262.
13. Wirrell E.C., Nabbout R., Scheffer I.E., et al. Methodology for classification and definition of epilepsy syndromes with list of syndromes: report of the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63 (6): 1333–48. https://doi.org/10.1111/epi.17237.
14. Zuberi S.M., Wirrell E., Yozawitz E., et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63 (6): 1349–97. https://doi.org/10.1111/epi.17239.
15. Riney K., Bogacz A., Somerville E., et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63 (6): 1443–74. https://doi.org/10.1111/epi.17240.
16. Linstone H.A., Turoff M. (Eds.) The Delphi method: techniques and applications. Available at: https://web.njit.edu/~turoff/pubs/delphibook/delphibook.pdf (accessed 07.06.2022).
17. Symonds J.D., Elliott K.S., Shetty J., et al. Early childhood epilepsies: epidemiology, classification, aetiology, and socio-economic determinants. Brain. 2021; 144 (9): 2879–91. https://doi.org/10.1093/brain/awab162.
18. Wirrell E., Wong-Kisiel L., Mandrekar J., Nickels K. Predictors and course of medically intractable epilepsy in young children presenting before 36 months of age: a retrospective, population-based study. Epilepsia. 2012; 53 (9): 1563–9. https://doi.org/10.1111/j.1528-1167.2012.03562.x
19. Moseley B.D., Wirrell E.C., Wong-Kisiel L.C., Nickels K. Early onset epilepsy is associated with increased mortality: a population-based study. Epilepsy Res. 2013; 105 (3): 410–14. https://doi.org/10.1016/j.eplepsyres.2013.03.002.
20. Berg A.T., Langfitt J.T., Testa F.M., et al. Global cognitive function in children with epilepsy: a community-based study. Epilepsia. 2008; 49 (4): 608–14. https://doi.org/10.1111/j.1528-1167.2007.01461.x.
21. Berg A.T., Zelko F.A., Levy S.R., Testa F.M. Age at onset of epilepsy, pharmacoresistance, and cognitive outcomes: a prospective cohort study. Neurology. 2012; 79 (13): 1384–91. https://doi.org/10.1212/WNL.0b013e31826c1b55.
22. Wilson S.J., Micallef S., Henderson A., et al. Developmental outcomes of childhood-onset temporal lobe epilepsy: a community-based study. Epilepsia. 2012; 53 (9): 1587–96. https://doi.org/10.1111/j.1528-1167.2012.03632.x.
23. Sharkov A.A. Genetic epilepsy with febrile seizures plus (GEFS+). Epilepsia i paroksizmalʹnye sostoania / Epilepsy and Paroxysmal Conditions. 2020; 12 (1S): S50–6 (in Russ.). https://doi.org/10.17749/2077-8333.2020.12.1S.S50-S56.
24. Camfield P., Camfield C. Incidence, prevalence and aetiology of seizures and epilepsy in children. Epileptic Disord. 2015; 17 (2) 117–23. https://doi.org/10.1684/epd.2015.0736.
25. Wirrell E.C., Grossardt B.R., Wong-Kisiel L.C., Nickels K.C. Incidence and classification of new-onset epilepsy and epilepsy syndromes in children in Olmsted County, Minnesota from 1980 to 2004: a population-based study. Epilepsy Res. 2011; 95 (1–2): 110–8. https://doi.org/10.1016/j.eplepsyres.2011.03.009.
26. Specchio N., Wirrell E.C., Scheffer I.E., et al. 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. Epilepsia. 2022; 63 (6): 1398–442. https://doi.org/10.1111/epi.17241.
27. Verrotti A., D’Alonzo R., Rinaldi V.E., et al. Childhood absence epilepsy and benign epilepsy with centro-temporal spikes: a narrative review analysis. World J Pediatr. 2017; 13 (2): 106–11. https://doi.org/10.1007/s12519-017-0006-9.
28. Kelley S.A., Kossoff E.H. Doose syndrome (myoclonic–astatic epilepsy): 40 years of progress. Dev Med Child Neurol. 2010; 52 (11): 988–93. https://doi.org/10.1111/j.1469-8749.2010.03744.x.
29. Doose H. Myoclonic-astatic epilepsy. Epilepsy Res Suppl. 1992; 6: 163–8.
30. Hirsch L.J., Gaspard N., van Baalen A., et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018; 59 (4): 739–44. https://doi.org/10.1111/epi.14016.
31. Payne E.T., Koh S., Wirrell E.C. Extinguishing febrile infection-related epilepsy syndrome: pipe dream or reality? Semin Neurol. 2020; 40 (2): 263–72. https://doi.org/10.1055/s-0040-1708503.
32. Specchio N., Pietrafusa N. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. Dev Med Child Neurol. 2020; 62 (8): 897–905. https://doi.org/10.1111/dmcn.14553.
33. Gastaut H., Poirier F., Payan H., et al. H.H.E. syndrome; hemiconvulsions, hemiplegia, epilepsy. Epilepsia. 1960; 1: 418–47. https://doi.org/10.1111/j.1528-1157.1959.tb04278.x.
34. Ayvazyan S.O. Comorbidity in hemiconvulsion-hemiplegia-epilepsy syndrome in children. Epilepsia i paroksizmalʹnye sostoania / Epilepsy and Paroxysmal Conditions. 2018; 10 (1): 73–9 (in Russ.). https://doi.org/10.17749/2077-8333.2018.10.1.073-079.
35. Hirsch E., French J., Scheffer I.E., et al. ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022; 63 (6): 1475–99. https://doi.org/10.1111/epi.17236.
36. Cendes F., Lopes-Cendes I., Andermann E., Andermann F. Familial temporal lobe epilepsy: a clinically heterogeneous syndrome. Neurology. 1998; 50 (2): 554–7. https://doi.org/10.1212/WNL.50.2.554.
37. Kobayashi E., Lopes-Cendes I., Guerreiro C.A., et al. Seizure outcome and hippocampal atrophy in familial mesial temporal lobe epilepsy. Neurology. 2001; 56 (2): 166–72. https://doi.org/10.1212/wnl.56.2.166.
38. Berkovic S.F., Serratosa J.M., Phillips H.A., et al. Familial partial epilepsy with variable foci: clinical features and linkage to chromosome 22q12. Epilepsia. 2004; 45 (9): 1054–60. https://doi.org/10.1111/j.0013-9580.2004.30502.x.
39. Bureau M., Genton P., Dravet C., et al. Epileptic syndromes in infancy, childhood and adolescence. 5th ed. John Libbey Eurotext; 2012: 600 pp.
40. Proposal for classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1985; 26 (3): 268–78.
41. Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1989; 30 (4): 389–99. https://doi.org/10.1111/j.1528-1157.1989.tb05316.x.
42. Angione K., Eschbach K., Smith G., et al. Genetic testing in a cohort of patients with potential epilepsy with myoclonic-atonic seizures. Epilepsy Res. 2019; 150: 70–7. https://doi.org/10.1016/j.eplepsyres.2019.01.008.
43. Zhang Y.H., Burgess R., Malone J.P., et al. Genetic epilepsy with febrile seizures plus: refining the spectrum. Neurology. 2017; 89 (12): 1210–9. https://doi.org/10.1212/WNL.0000000000004384.
44. Alwadei A.H. Childhood absence epilepsy: electro-clinical manifestations, treatment options, and outcome in a tertiary educational center. Int J Pediatr Adolesc Med. 2022; 9 (2): 131–5. https://doi.org/10.1016/j.ijpam.2021.11.003.
45. Elmali A.D., Auvin S., Bast T., et al. How to diagnose and classify idiopathic (genetic) generalized epilepsies. Epileptic Disord. 2020; 22 (4): 399–420. https://doi.org/10.1684/epd.2020.1192.
46. Turco F., Bonanni E., Milano C., et al. Prolonged epileptic discharges predict seizure recurrence in JME: Insights from prolonged ambulatory EEG. Epilepsia. 2021; 62 (5): 1184–92. https://doi.org/10.1111/epi.16875.
47. Pietrafusa N., La Neve A., de Palma L., et al. Juvenile myoclonic epilepsy: long-term prognosis and risk factors. Brain Dev. 2021; 43 (6): 688–97. https://doi.org/10.1016/j.braindev.2021.02.005.
48. Amrutkar C., Riel-Romero R.M. Juvenile myoclonic epilepsy. Treasure Island (FL): StatPearls Publishing; 2021.
Supplementary files
|
1. Приложение A1. Эпилептические синдромы с началом в неонатальном периоде и младенчестве | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(1MB)
|
Indexing metadata ▾ | |
|
|
2. Приложение А2. Диагностические критерии эпилептических синдромов с началом в неонатальном периоде и младенчестве | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(1MB)
|
Indexing metadata ▾ | |
|
|
3. Приложение В1. Эпилептические синдромы с началом в детском возрасте | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(1MB)
|
Indexing metadata ▾ | |
|
|
4. Приложение В2. Диагностические критерии эпилептических синдромов с началом в детском возрасте | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(1004KB)
|
Indexing metadata ▾ | |
|
|
5. Приложение В3. Эпилептические синдромы с началом в детском возрасте: сравнение названий | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(755KB)
|
Indexing metadata ▾ | |
|
|
6. Приложение С1. Эпилептические синдромы с началом в разном возрасте | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(986KB)
|
Indexing metadata ▾ | |
|
|
7. Приложение С2. Диагностические критерии эпилептических синдромов с началом в разном возрасте | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(916KB)
|
Indexing metadata ▾ | |
|
|
8. Приложение С3. Отличительные особенности связанной со сном гипермоторной (гиперкинетической) эпилепсии, семейной мезиальной височной эпилепсии, семейной фокальной эпилепсии с вариабельными очагами и эпилепсии со слуховыми приступами | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(769KB)
|
Indexing metadata ▾ | |
|
|
9. Приложение D1. Синдромы идиопатической генерализованной эпилепсии | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(946KB)
|
Indexing metadata ▾ | |
|
|
10. Приложение D2. Диагностические критерии синдромов идиопатической генерализованной эпилепсии | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(855KB)
|
Indexing metadata ▾ | |
|
|
11. Приложение D3. Отличительные особенности детской абсансной эпилепсии, юношеской абсансной эпилепсии, юношеской миоклонической эпилепсии и эпилепсии с изолированными генерализованными тонико-клоническими приступами | |
| Subject | ||
| Type | Исследовательские инструменты | |
Download
(798KB)
|
Indexing metadata ▾ | |
Review
For citations:
Blinov D.V. Epilepsy syndromes: the 2022 ILAE definition and classification. Epilepsy and paroxysmal conditions. 2022;14(2):101-182. (In Russ.) https://doi.org/10.17749/2077-8333/epi.par.con.2022.123
Views:
33138
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Email this article 
