癫痫的 ILAE 分类：ILAE 委员会关于分类和术语的意见书_Journal of Epilepsy

Authors：

 Ingrid EScheffer , SamuelBerkovic , GiuseppeCapovilla , 张静　张月华　译 , 刘晓燕　吴逊　审

;

Corresponding author：

Ingrid EScheffer, Email: scheffer@unimelb.edu.au

Keywords：

分类; 癫痫综合征; 术语; 病因

DOI：

10.7507/2096-0247.20180084

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自从 1989 年国际抗癫痫联盟（ILAE）最后批准的分类以来，ILAE 已经更新了癫痫的分类，以反映我们在获得巨大科学进步后对癫痫及其基本机制理解方面的收获。作为执业医师的重要工具，在我们的思想中癫痫分类必须有恰当和动态的变化，并且应全面的翻译到全球所有地区。癫痫分类最初的目的是对患者进行诊断，但是对于癫痫研究、抗癫痫治疗的发展和全世界的交流也非常重要。这次新分类起源于 2013 年提交以供公开评论的一份草拟文件，该草拟文件在经过国际癫痫界几轮讨论广泛反馈后得以修订。此分类呈现了 3 个层次，首先是发作类型，假设患者已经有最新的 2017 年 ILAE 癫痫发作分类所确定的发作类型；诊断了发作类型后，下一步就是诊断癫痫类型，包括局灶性癫痫、全面性癫痫、全面性及局灶性癫痫两者兼有，以及分类不明的癫痫；第三层次是癫痫综合征，此处可以做出特定综合征的诊断。这一新分类在每一层次均包含了病因，强调在每一步诊断时都要考虑病因，因为病因将会对治疗产生重要影响。选择将病因分为 6 个亚组是基于其潜在的治疗因果关系。该分类引入了新的术语如“发育性和癫痫性脑病”等。“自限性”和“药物有效性”取代“良性”一词。希望这一新的框架将有助于提高 21 世纪癫痫的关爱和研究。

Citation： Ingrid EScheffer, SamuelBerkovic, GiuseppeCapovilla, 张静　张月华　译, 刘晓燕　吴逊　审. 癫痫的 ILAE 分类：ILAE 委员会关于分类和术语的意见书. Journal of Epilepsy, 2018, 4(6): 517-525. doi: 10.7507/2096-0247.20180084 Copy

1.	Gastaut H, Caveness WF, Landolt W, et al. A proposed international classification of epileptic seizures. Epilepsia, 1964, 5(3): 297-306.
2.	Gastaut H. Clinical and electroencephalographical classification of epileptic seizures. Epilepsia, 1969, 10(Suppl 1): 2-13.
3.	Gastaut H. Classification of the epilepsies. Proposal for an international classification. Epilepsia, 1969, 10(Suppl 1): 14-21.
4.	Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for classification of epilepsies and epileptic syndromes. Epilepsia, 1985, 26(3): 268-278.
5.	Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia, 1989, 30(4): 389-399.
6.	Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia, 1981, 22(4): 489-501.
7.	Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia, 2001, 42(6): 796-803.
8.	Engel J. Report of the ILAE classification core group. Epilepsia, 2006, 47(9): 1558-1568.
9.	Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology 2005-2009. Epilepsia, 2010, 51(4): 676-685.
10.	Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy. Epilepsia, 2017, 58(4): 522-530.
11.	Epilepsy ILa. Guidelines for publications from league commissions and task forces 2014. Available at: http://www.ilae.org/visitors/centre/guidelines.cfm. Accessed July 2 2015.
12.	Avanzini G. A sound conceptual framework for an epilepsy classification is still lacking. Epilepsia, 2010, 51(4): 720-722.
13.	Beghi E. New classification proposals for epilepsy: a real advancement in the nosography of the disease? Epilepsia, 2011, 52(6): 1197-1198, discussion 1205-1199.
14.	Berg AT, Scheffer IE. New concepts in classification of the epilepsies: entering the 21st century. Epilepsia, 2011, 52(6): 1058-1062.
15.	Berg AT, Scheffer IE. What is at stake in a classification? Epilepsia, 2011, 52(6): 1205-1208.
16.	Camfield P. Issues in epilepsy classification for population studies. Epilepsia, 2012, 53(Suppl 2): 10-13.
17.	Duncan JS. The evolving classification of seizures and epilepsies. Epilepsia, 2011, 52(6): 1204-1205, discussion 1205-1209.
18.	Engel J Jr. The etiologic classification of epilepsy. Epilepsia, 2011, 52(6): 1195-1197, discussion 1205-1209.
19.	Ferrie CD. Terminology and organization of seizures and epilepsies: radical changes not justified by new evidence. Epilepsia, 2010, 51(4): 713-714.
20.	Fisher RS. What is a classification essay? Epilepsia, 2010, 51(4): 714-715.
21.	Guerrini R. Classification concepts and terminology: is clinical description assertive and laboratory testing objective? Epilepsia, 2010, 51(4): 718-720.
22.	Jackson G. Classification of the epilepsies 2011. Epilepsia, 2011, 52(6): 1203-1204.
23.	Moshe SL. In support of the ILAE Commission classification proposal. Epilepsia, 2011, 52(6): 1200-1201, discussion 1205-1209.
24.	Luders HO, Amina S, Baumgartner C, et al. Modern technology calls for a modern approach to classification of epileptic seizures and the epilepsies. Epilepsia, 2012, 53(3): 405-411.
25.	Panayiotopoulos CP. The new ILAE report on terminology and concepts for the organization of epilepsies: critical review and contribution. Epilepsia, 2012, 53(3): 399-404.
26.	Shinnar S. The new ILAE classification. Epilepsia, 2010, 51(4): 715-717.
27.	Shorvon SD. The etiologic classification of epilepsy. Epilepsia, 2011, 52(6): 1052-1057.
28.	Wolf P. Much ado about nothing? Epilepsia, 2010, 51(4): 717-718.
29.	Wong M. Epilepsy is both a symptom and a disease: a proposal for a two-tiered classification system. Epilepsia, 2011, 52(6): 1201-1203, discussion 1205-1209.
30.	Scheffer I, French J, Hirsch E, et al. Clasification of the epilepsies: new concepts for discussion and debate-special report of the ILAE Classification Task Force of the Commission for Classification and Terminology. Epilepsia Open, 2016, 1(1-2): 37-44.
31.	Fisher RS, Acevedo C, Arzimanoglou A, et al. ILAE official report: a practical clinical definition of epilepsy. Epilepsia, 2014, 55(4): 475-482.
32.	McTague A, Howell KB, Cross JH, et al. The genetic landscape of the epileptic encephalopathies of infancy and childhood. Lancet Neurol, 2016, 15(3): 304-316.
33.	Guerrini R, Pellacani S. Benign childhood focal epilepsies. Epilepsia, 2012, 53(Suppl 4): 9-18.
34.	Beaumanoir A, Nahory A. Benign partial epilepsies: 11 cases of frontal partial epilepsy with favorable prognosis. Rev Electroencephalogr Neurophysiol Clin, 1983, 13(3): 207-211.
35.	Berkovic SF, McIntosh A, Howell RA, et al. Familial temporal lobe epilepsy: a common disorder identified in twins. Ann Neurol, 1996, 40(2): 227-235.
36.	Sveinbjornsdottir S, Duncan JS. Parietal and occipital lobe epilepsy: a review. Epilepsia, 1993, 34(3): 493-521.
37.	Gaillard WD, Chiron C, Cross JH, et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia, 2009, 50(9): 2147-2153.
38.	Guerrini R, Dobyns WB. Malformations of cortical development: clinical features and genetic causes. Lancet Neurol, 2014, 13(7): 710-726.
39.	Grinton BE, Heron SE, Pelekanos JT, et al. Familial neonatal seizures in 36 families: clinical and genetic features correlate with outcome. Epilepsia, 2015, 56(7): 1071-1080.
40.	Tinuper P, Bisulli F, Cross JH, et al. Definition and diagnostic criteria of sleep-related hypermotor epilepsy. Neurology, 2016, 86(19): 1834-1842.
41.	Lennox WG. The genetics of epilepsy. Am J Psychiatry, 1947, 103(4): 457-462.
42.	Lennox WG. The heredity of epilepsy as told by relatives and twins. J Am Med Assoc, 1951, 146(6): 529-536.
43.	Brunklaus A, Dorris L, Ellis R, et al. The clinical utility of an SCN1A genetic diagnosis in infantile-onset epilepsy. Dev Med Child Neurol, 2013, 55(2): 154-161.
44.	Mullen SA, Scheffer IE. Translational research in epilepsy genetics: sodium channels in man to interneuronopathy in mouse. Arch Neurol, 2009, 66(1): 21-26.
45.	Helbig I, Mefford H, Sharp A, et al. 15q13.3 microdeletions increase risk of idiopathic generalized epilepsy. Nat Genet, 2009, 41(2): 160-162.
46.	Dibbens LM, Mullen S, Helbig I, et al. Familial and sporadic 15q13.3 microdeletions in idiopathic generalized epilepsy: precedent for disorders with complex inheritance. Hum Mol Genet, 2009, 18(19): 3626-3631.
47.	Claes L, Del-Favero J, Ceulemans B, et al. De novo mutations in the sodium-channel gene SCN1A cause severe myoclonic epilepsy of infancy. Am J Hum Genet, 2001, 68(6): 1327-1332.
48.	Weckhuysen S, Mandelstam S, Suls A, et al. KCNQ2 encephalopathy: emerging phenotype of a neonatal epileptic encephalopathy. Ann Neurol, 2012, 71(1): 15-25.
49.	Depienne C, Bouteiller D, Keren B, et al. Sporadic infantile epileptic encephalopathy caused by mutations in PCDH19 resembles Dravet syndrome but mainly affects females. PLoS Genet, 2009, 5(2): e1000381.
50.	Arsov T, Mullen SA, Rogers S, et al. Glucose transporter 1 deficiency in the idiopathic generalized epilepsies. Ann Neurol, 2012, 72(5): 807-815.
51.	Scheffer IE, Grinton BE, Heron SE, et al. PRRT2 phenotypic spectrum includes sporadic and fever-related infantile seizures. Neurology, 2012, 79(21): 2104-2108.
52.	Carvill GL, Heavin SB, Yendle SC, et al. Targeted resequencing in epileptic encephalopathies identifies de novo mutations in CHD2 and SYNGAP1. Nat Genet, 2013, 45(7): 825-830.
53.	Depienne C, Trouillard O, Gourfinkel-An I, et al. Mechanisms for variable expressivity of inherited SCN1A mutations causing dravet syndrome. J Med Genet, 2010, 47(6): 404-410.
54.	Vezzani A, Fujinami RS, White HS, et al. Infections, inflammation and epilepsy. Acta Neuropathol, 2016, 131(2): 211-234.
55.	Lancaster E, Dalmau J. Neuronal autoantigens-pathogenesis, associated disorders and antibody testing. Nat Rev Neurol, 2012, 19(8): 380-390.
56.	Staden UE, Isaacs E, Boyd SG, et al. Language dysfunction in children with rolandic epilepsy. Neuropediatrics, 1998, 29(5): 242-248.
57.	Lillywhite LM, Saling MM, Harvey AS, et al. Neuropsychological and functional MRI studies provide converging evidence of anterior language dysfunction in BECTS. Epilepsia, 2009, 50(10): 2276-2284.
58.	Wirrell EC, Camfield CS, Camfield PR, et al. Long-term psychosocial outcome in typical absence epilepsy. Sometimes a wolf in sheeps’ clothing. Arch Pediatr Adolesc Med, 1997, 151(2): 152-158.

1. Gastaut H, Caveness WF, Landolt W, et al. A proposed international classification of epileptic seizures. Epilepsia, 1964, 5(3): 297-306.
2. Gastaut H. Clinical and electroencephalographical classification of epileptic seizures. Epilepsia, 1969, 10(Suppl 1): 2-13.
3. Gastaut H. Classification of the epilepsies. Proposal for an international classification. Epilepsia, 1969, 10(Suppl 1): 14-21.
4. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for classification of epilepsies and epileptic syndromes. Epilepsia, 1985, 26(3): 268-278.
5. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia, 1989, 30(4): 389-399.
6. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia, 1981, 22(4): 489-501.
7. Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia, 2001, 42(6): 796-803.
8. Engel J. Report of the ILAE classification core group. Epilepsia, 2006, 47(9): 1558-1568.
9. Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology 2005-2009. Epilepsia, 2010, 51(4): 676-685.
10. Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy. Epilepsia, 2017, 58(4): 522-530.
11. Epilepsy ILa. Guidelines for publications from league commissions and task forces 2014. Available at: http://www.ilae.org/visitors/centre/guidelines.cfm. Accessed July 2 2015.
12. Avanzini G. A sound conceptual framework for an epilepsy classification is still lacking. Epilepsia, 2010, 51(4): 720-722.
13. Beghi E. New classification proposals for epilepsy: a real advancement in the nosography of the disease? Epilepsia, 2011, 52(6): 1197-1198, discussion 1205-1199.
14. Berg AT, Scheffer IE. New concepts in classification of the epilepsies: entering the 21st century. Epilepsia, 2011, 52(6): 1058-1062.
15. Berg AT, Scheffer IE. What is at stake in a classification? Epilepsia, 2011, 52(6): 1205-1208.
16. Camfield P. Issues in epilepsy classification for population studies. Epilepsia, 2012, 53(Suppl 2): 10-13.
17. Duncan JS. The evolving classification of seizures and epilepsies. Epilepsia, 2011, 52(6): 1204-1205, discussion 1205-1209.
18. Engel J Jr. The etiologic classification of epilepsy. Epilepsia, 2011, 52(6): 1195-1197, discussion 1205-1209.
19. Ferrie CD. Terminology and organization of seizures and epilepsies: radical changes not justified by new evidence. Epilepsia, 2010, 51(4): 713-714.
20. Fisher RS. What is a classification essay? Epilepsia, 2010, 51(4): 714-715.
21. Guerrini R. Classification concepts and terminology: is clinical description assertive and laboratory testing objective? Epilepsia, 2010, 51(4): 718-720.
22. Jackson G. Classification of the epilepsies 2011. Epilepsia, 2011, 52(6): 1203-1204.
23. Moshe SL. In support of the ILAE Commission classification proposal. Epilepsia, 2011, 52(6): 1200-1201, discussion 1205-1209.
24. Luders HO, Amina S, Baumgartner C, et al. Modern technology calls for a modern approach to classification of epileptic seizures and the epilepsies. Epilepsia, 2012, 53(3): 405-411.
25. Panayiotopoulos CP. The new ILAE report on terminology and concepts for the organization of epilepsies: critical review and contribution. Epilepsia, 2012, 53(3): 399-404.
26. Shinnar S. The new ILAE classification. Epilepsia, 2010, 51(4): 715-717.
27. Shorvon SD. The etiologic classification of epilepsy. Epilepsia, 2011, 52(6): 1052-1057.
28. Wolf P. Much ado about nothing? Epilepsia, 2010, 51(4): 717-718.
29. Wong M. Epilepsy is both a symptom and a disease: a proposal for a two-tiered classification system. Epilepsia, 2011, 52(6): 1201-1203, discussion 1205-1209.
30. Scheffer I, French J, Hirsch E, et al. Clasification of the epilepsies: new concepts for discussion and debate-special report of the ILAE Classification Task Force of the Commission for Classification and Terminology. Epilepsia Open, 2016, 1(1-2): 37-44.
31. Fisher RS, Acevedo C, Arzimanoglou A, et al. ILAE official report: a practical clinical definition of epilepsy. Epilepsia, 2014, 55(4): 475-482.
32. McTague A, Howell KB, Cross JH, et al. The genetic landscape of the epileptic encephalopathies of infancy and childhood. Lancet Neurol, 2016, 15(3): 304-316.
33. Guerrini R, Pellacani S. Benign childhood focal epilepsies. Epilepsia, 2012, 53(Suppl 4): 9-18.
34. Beaumanoir A, Nahory A. Benign partial epilepsies: 11 cases of frontal partial epilepsy with favorable prognosis. Rev Electroencephalogr Neurophysiol Clin, 1983, 13(3): 207-211.
35. Berkovic SF, McIntosh A, Howell RA, et al. Familial temporal lobe epilepsy: a common disorder identified in twins. Ann Neurol, 1996, 40(2): 227-235.
36. Sveinbjornsdottir S, Duncan JS. Parietal and occipital lobe epilepsy: a review. Epilepsia, 1993, 34(3): 493-521.
37. Gaillard WD, Chiron C, Cross JH, et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia, 2009, 50(9): 2147-2153.
38. Guerrini R, Dobyns WB. Malformations of cortical development: clinical features and genetic causes. Lancet Neurol, 2014, 13(7): 710-726.
39. Grinton BE, Heron SE, Pelekanos JT, et al. Familial neonatal seizures in 36 families: clinical and genetic features correlate with outcome. Epilepsia, 2015, 56(7): 1071-1080.
40. Tinuper P, Bisulli F, Cross JH, et al. Definition and diagnostic criteria of sleep-related hypermotor epilepsy. Neurology, 2016, 86(19): 1834-1842.
41. Lennox WG. The genetics of epilepsy. Am J Psychiatry, 1947, 103(4): 457-462.
42. Lennox WG. The heredity of epilepsy as told by relatives and twins. J Am Med Assoc, 1951, 146(6): 529-536.
43. Brunklaus A, Dorris L, Ellis R, et al. The clinical utility of an SCN1A genetic diagnosis in infantile-onset epilepsy. Dev Med Child Neurol, 2013, 55(2): 154-161.
44. Mullen SA, Scheffer IE. Translational research in epilepsy genetics: sodium channels in man to interneuronopathy in mouse. Arch Neurol, 2009, 66(1): 21-26.
45. Helbig I, Mefford H, Sharp A, et al. 15q13.3 microdeletions increase risk of idiopathic generalized epilepsy. Nat Genet, 2009, 41(2): 160-162.
46. Dibbens LM, Mullen S, Helbig I, et al. Familial and sporadic 15q13.3 microdeletions in idiopathic generalized epilepsy: precedent for disorders with complex inheritance. Hum Mol Genet, 2009, 18(19): 3626-3631.
47. Claes L, Del-Favero J, Ceulemans B, et al. De novo mutations in the sodium-channel gene SCN1A cause severe myoclonic epilepsy of infancy. Am J Hum Genet, 2001, 68(6): 1327-1332.
48. Weckhuysen S, Mandelstam S, Suls A, et al. KCNQ2 encephalopathy: emerging phenotype of a neonatal epileptic encephalopathy. Ann Neurol, 2012, 71(1): 15-25.
49. Depienne C, Bouteiller D, Keren B, et al. Sporadic infantile epileptic encephalopathy caused by mutations in PCDH19 resembles Dravet syndrome but mainly affects females. PLoS Genet, 2009, 5(2): e1000381.
50. Arsov T, Mullen SA, Rogers S, et al. Glucose transporter 1 deficiency in the idiopathic generalized epilepsies. Ann Neurol, 2012, 72(5): 807-815.
51. Scheffer IE, Grinton BE, Heron SE, et al. PRRT2 phenotypic spectrum includes sporadic and fever-related infantile seizures. Neurology, 2012, 79(21): 2104-2108.
52. Carvill GL, Heavin SB, Yendle SC, et al. Targeted resequencing in epileptic encephalopathies identifies de novo mutations in CHD2 and SYNGAP1. Nat Genet, 2013, 45(7): 825-830.
53. Depienne C, Trouillard O, Gourfinkel-An I, et al. Mechanisms for variable expressivity of inherited SCN1A mutations causing dravet syndrome. J Med Genet, 2010, 47(6): 404-410.
54. Vezzani A, Fujinami RS, White HS, et al. Infections, inflammation and epilepsy. Acta Neuropathol, 2016, 131(2): 211-234.
55. Lancaster E, Dalmau J. Neuronal autoantigens-pathogenesis, associated disorders and antibody testing. Nat Rev Neurol, 2012, 19(8): 380-390.
56. Staden UE, Isaacs E, Boyd SG, et al. Language dysfunction in children with rolandic epilepsy. Neuropediatrics, 1998, 29(5): 242-248.
57. Lillywhite LM, Saling MM, Harvey AS, et al. Neuropsychological and functional MRI studies provide converging evidence of anterior language dysfunction in BECTS. Epilepsia, 2009, 50(10): 2276-2284.
58. Wirrell EC, Camfield CS, Camfield PR, et al. Long-term psychosocial outcome in typical absence epilepsy. Sometimes a wolf in sheeps’ clothing. Arch Pediatr Adolesc Med, 1997, 151(2): 152-158.

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