Lennox-Gastaut综合征诊断治疗的中国专家共识_Journal of Epilepsy

Authors：

 中国抗癫痫协会创新与转化专委会

执笔（按作者姓氏拼音排序，排名不分前后）季涛云、姜玉武（北京大学第一医院），蒋莉（重庆医科大学附属儿童医院），梁树立（首都医科大学附属北京儿童医院），钱若兵（安徽省立医院），邵晓秋（首都医科大学附属北京天坛医院），王艺（复旦大学附属儿科医院），尹飞（中南大学湘雅医院），张玉琴（天津市儿童医院），赵国光（首都医科大学宣武医院），周东（四川大学华西医院），朱丹（广东三九脑科医院），朱遂强（华中科技大学同济医学院附属同济医院），邹丽萍（中国人民解放军总医院第一医学中心）;

Corresponding author：

中国抗癫痫协会创新与转化专委会, Email: jiangyuwu@bjmu.edu.cn

Keywords：

DOI：

10.7507/2096-0247.202202001

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Citation： 中国抗癫痫协会创新与转化专委会. Lennox-Gastaut综合征诊断治疗的中国专家共识. Journal of Epilepsy, 2022, 8(3): 187-195. doi: 10.7507/2096-0247.202202001 Copy

1.	Strzelczyk A, Schubert-Bast S. Expanding the treatment landscape for Lennox-Gastaut Syndrome: current and Future Strategies. CNS Drugs, 2021, 35(1): 61-83.
2.	中国抗癫痫协会. 临床诊疗指南(癫痫病分册). 北京: 人民卫生出版社, 2015: 21-23, 26, 47, 63, 77-78.
3.	Ostendorf AP, Ng YT. Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. Neuropsychiatr Dis Treat, 2017, 13: 1131-1140.
4.	Cross JH, Auvin S, Falip M, et al. Expert opinion on the management of Lennox-Gastaut Syndrome: treatmentalgorithms and practical considerations. Front Neurol, 2017, 8: 505.
5.	European Commission. Rare Disease Registries in Europe. September 2020. https://www.orpha.net/orphacom/cahiers/docs/GB/Registries.pdf.
6.	National Organization for Rare Disorders. Rare Disease Database.https://rarediseases.org/?s=L&post_type=rare-diseases.
7.	Montouris G, Aboumatar S, Burdette D, et al. Expert opinion: proposed diagnostic and treatment algorithms for Lennox-Gastaut syndrome in adult patients. Epilepsy Behav, 2020, 110: 107146.
8.	Kanner AM, Ashman E, Gloss D, et al. Practice guideline update summary: efficacy and tolerability of the new antiepileptic drugs II: treatment-resistant epilepsy: report of the guideline development, dissemination, and implementation subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology, 2018, 91(2): 82-90.
9.	Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci, 2018, 39(3): 403-414.
10.	Arzimanoglou A, French J, Blume WT, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol, 2009, 8(1): 82-93.
11.	Chtydodyomos P. Panayiotopoulos著. 任连坤译. 癫痫发作和综合征的诊断与治疗. 中国协和医科大学出版社, 2008: 149.
12.	Gibson PA. Lennox-Gastaut syndrome: impact on the caregivers and families of patients. J Multidiscip Healthc, 2014, 7: 441-448.
13.	Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol, 2003, 20(6): 426-441.
14.	Arzimanoglou A, Resnick T. All children who experience epileptic falls do not necessarily have Lennox-Gastaut syndrome. . . but many do. Epileptic Disord, 2011, 13 Suppl 1: S3-13.
15.	Douglass LM, Salpekar J. Surgical options for patients with Lennox-Gastaut syndrome. Epilepsia, 2014, 55(Suppl 4): 21-28.
16.	Hancock EC, Cross JH. Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev, 2013, 2013(2): CD003277.
17.	Tomson T, Battino D, Perucca E. Valproic acid after five decades of use in epilepsy: time to reconsider the indications of a time-honoured drug. Lancet Neurol, 2016, 15(2): 210-218.
18.	Michoulas A, Farrell K. Medical management of Lennox-Gastaut syndrome. CNS Drugs, 2010, 24(5): 363-374.
19.	Covanis A, Gupta AK, Jeavons PM. Sodium valproate: monotherapy and polytherapy. Epilepsia, 1982, 23(6): 693-720.
20.	NICE. Epilepsies: diagnosis and management. 2021.
21.	SIGN. Epilepsies in children and young people: investigative procedures and management. May 2021.
22.	Motte J, Trevathan E, Arvidsson JF, et al. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group. N Engl J Med, 1997, 337(25): 1807-1812.
23.	Eriksson AS, Nergårdh A, Hoppu K. The efficacy of lamotrigine in children and adolescents with refractory generalized epilepsy: a randomized, double-blind, crossover study. Epilepsia, 1998, 39(5): 495-501.
24.	Egunsola O, Choonara I, Sammons HM. Safety of lamotrigine in paediatrics: a systematic review. BMJ Open, 2015, 5(6): e007711.
25.	Glauser T, Kluger G, Sachdeo R, et al. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurology, 2008, 70(21): 1950-1958.
26.	Ohtsuka Y, Yoshinaga H, Shirasaka Y, et al. Rufinamide as an adjunctive therapy for Lennox-Gastaut syndrome: a randomized double-blind placebo-controlled trial in Japan. Epilepsy Res, 2014, 108(9): 1627-1636.
27.	Sachdeo RC, Glauser TA, Ritter F, et al. A double-blind, randomized trial of topiramate in Lennox-Gastaut syndrome. Topiramate YL Study Group. Neurology, 1999, 52(9): 1882-1887.
28.	Glauser TA, Levisohn PM, Ritter F, et al. Topiramate in Lennox-Gastaut syndrome: open-label treatment of patients completing a randomized controlled trial. Topiramate YL Study Group. Epilepsia, 2000, 41(S1): 86-90.
29.	王艺, 孙道开, 陈天兰, 等. 托吡酯治疗Lennox-Gastaut综合征47例临床分析. 临床神经电生理学杂志, 2001, 10(4): 224-225.
30.	邱鹏玲, 周水珍, 孙道开. 托吡酯单药治疗40例小儿癫痫疗效分析. 临床神经电生理学杂志, 2001, 10(4): 203-205.
31.	孙道开, 王艺, 陈天兰, 等. 托吡酯治疗小儿癫痫66例临床分析. 中国实用儿科杂志, 2001, 16(6): 356-358.
32.	Devinsky O, Patel AD, Cross JH, et al. Effect of Cannabidiol on drop seizures in the Lennox-Gastaut Syndrome. N Engl J Med, 2018, 378(20): 1888-1897.
33.	Thiele EA, Marsh ED, French JA, et al. Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet, 2018, 391(10125): 1085-1096.
34.	Felbamate Study Group in Lennox-Gastaut Syndrome. Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). N Engl J Med, 1993, 328(1): 29-33.
35.	Ng YT, Conry JA, Drummond R, et al. Randomized, phase III study results of clobazam in Lennox-Gastaut syndrome. Neurology, 2011, 77: 1473-1481.
36.	Kim HJ, Kim SH, Kang HC, et al. Adjunctive levetiracetam treatment in pediatric Lennox-Gastaut syndrome. Pediatr Neurol, 2014, 51(4): 527-531.
37.	You SJ, Kang HC, Kim HD, et al. Clinical efficacy of zonisamide in Lennox-Gastaut syndrome: Korean multicentric experience. Brain Dev, 2008, 30(4): 287-290.
38.	Auvin S, Dozieres B, Ilea A, et al. Use of perampanel in children and adolescents with Lennox-Gastaut Syndrome. Epilepsy Behav, 2017, 74: 59-63.
39.	Biró A, Stephani U, Tarallo T, et al. Effectiveness and tolerability of perampanel in children and adolescents with refractory epilepsies: first experiences. Neuropediatrics, 2015, 46(2): 110-116.
40.	Zhang Y, Wang Y, Zhou Y, et al. Therapeutic effects of the ketogenic diet in children with Lennox-Gastaut syndrome. Epilepsy Res, 2016, 128: 176-180.
41.	Caraballo RH, Fortini S, Fresler S, et al. Ketogenic diet in patients with Lennox-Gastaut syndrome. Seizure, 2014, 23(9): 751-755.
42.	Lemmon ME, Terao NN, Ng YT, et al. Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature. Dev Med Child Neurol, 2012, 54(5): 464-468.
43.	Archer JS, Warren AE, Stagnitti MR, et al. Lennox-Gastaut syndrome and phenotype: secondary network epilepsies. Epilepsia, 2014, 55(8): 1245-1254.
44.	Kang JW, Eom S, Hong W, et al. Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome. Pediatrics, 2018, 142(4): e20180449.
45.	安宁, 刘仕勇, 杨梅华, 等. Lennox-Gastaut综合征的外科治疗初探. 中华神经外科疾病研究杂志, 2007, 6(4): 352-355.
46.	丁平, 于晓曼, 张军臣, 等. 切除性手术治疗学龄儿童病灶性Lennox-Gastaut 综合征. 临床神经外科杂志, 2015, 12(3): 181-184,188.
47.	Lee YJ, Kang HC, Lee JS, et al. Resective pediatric epilepsy surgery in Lennox-Gastaut syndrome. Pediatrics, 2010, 125(1): e58-66.
48.	Tao JX, Satzer D, Issa NP, et al. Stereotactic laser anterior corpus callosotomy for Lennox-Gastaut syndrome. Epilepsia, 2020, 61(6): 1190-1200.
49.	Liang S, Zhang S, Hu X, et al. Anterior corpus callosotomy in school-aged children with Lennox-Gastaut syndrome: a prospective study. Eur J Paediatr Neurol, 2014, 18(6): 670-676.
50.	Sharawat IK, Panda PK, Sihag RK, et al. Efficacy and safety of corpus callosotomy and ketogenic diet in children with Lennox Gastaut syndrome: a systematic review and meta-analysis. Childs Nerv Syst, 2021, 37(8): 2557-2566.
51.	Dibué M, Greco T, Spoor JKH, et al. Vagus nerve stimulation in patients with Lennox-Gastaut syndrome: A meta-analysis. Acta Neurol Scand, 2021, 143(5): 497-508.

1. Strzelczyk A, Schubert-Bast S. Expanding the treatment landscape for Lennox-Gastaut Syndrome: current and Future Strategies. CNS Drugs, 2021, 35(1): 61-83.
2. 中国抗癫痫协会. 临床诊疗指南(癫痫病分册). 北京: 人民卫生出版社, 2015: 21-23, 26, 47, 63, 77-78.
3. Ostendorf AP, Ng YT. Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. Neuropsychiatr Dis Treat, 2017, 13: 1131-1140.
4. Cross JH, Auvin S, Falip M, et al. Expert opinion on the management of Lennox-Gastaut Syndrome: treatmentalgorithms and practical considerations. Front Neurol, 2017, 8: 505.
5. European Commission. Rare Disease Registries in Europe. September 2020. https://www.orpha.net/orphacom/cahiers/docs/GB/Registries.pdf.
6. National Organization for Rare Disorders. Rare Disease Database.https://rarediseases.org/?s=L&post_type=rare-diseases.
7. Montouris G, Aboumatar S, Burdette D, et al. Expert opinion: proposed diagnostic and treatment algorithms for Lennox-Gastaut syndrome in adult patients. Epilepsy Behav, 2020, 110: 107146.
8. Kanner AM, Ashman E, Gloss D, et al. Practice guideline update summary: efficacy and tolerability of the new antiepileptic drugs II: treatment-resistant epilepsy: report of the guideline development, dissemination, and implementation subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology, 2018, 91(2): 82-90.
9. Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci, 2018, 39(3): 403-414.
10. Arzimanoglou A, French J, Blume WT, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol, 2009, 8(1): 82-93.
11. Chtydodyomos P. Panayiotopoulos著. 任连坤译. 癫痫发作和综合征的诊断与治疗. 中国协和医科大学出版社, 2008: 149.
12. Gibson PA. Lennox-Gastaut syndrome: impact on the caregivers and families of patients. J Multidiscip Healthc, 2014, 7: 441-448.
13. Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol, 2003, 20(6): 426-441.
14. Arzimanoglou A, Resnick T. All children who experience epileptic falls do not necessarily have Lennox-Gastaut syndrome. . . but many do. Epileptic Disord, 2011, 13 Suppl 1: S3-13.
15. Douglass LM, Salpekar J. Surgical options for patients with Lennox-Gastaut syndrome. Epilepsia, 2014, 55(Suppl 4): 21-28.
16. Hancock EC, Cross JH. Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev, 2013, 2013(2): CD003277.
17. Tomson T, Battino D, Perucca E. Valproic acid after five decades of use in epilepsy: time to reconsider the indications of a time-honoured drug. Lancet Neurol, 2016, 15(2): 210-218.
18. Michoulas A, Farrell K. Medical management of Lennox-Gastaut syndrome. CNS Drugs, 2010, 24(5): 363-374.
19. Covanis A, Gupta AK, Jeavons PM. Sodium valproate: monotherapy and polytherapy. Epilepsia, 1982, 23(6): 693-720.
20. NICE. Epilepsies: diagnosis and management. 2021.
21. SIGN. Epilepsies in children and young people: investigative procedures and management. May 2021.
22. Motte J, Trevathan E, Arvidsson JF, et al. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group. N Engl J Med, 1997, 337(25): 1807-1812.
23. Eriksson AS, Nergårdh A, Hoppu K. The efficacy of lamotrigine in children and adolescents with refractory generalized epilepsy: a randomized, double-blind, crossover study. Epilepsia, 1998, 39(5): 495-501.
24. Egunsola O, Choonara I, Sammons HM. Safety of lamotrigine in paediatrics: a systematic review. BMJ Open, 2015, 5(6): e007711.
25. Glauser T, Kluger G, Sachdeo R, et al. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurology, 2008, 70(21): 1950-1958.
26. Ohtsuka Y, Yoshinaga H, Shirasaka Y, et al. Rufinamide as an adjunctive therapy for Lennox-Gastaut syndrome: a randomized double-blind placebo-controlled trial in Japan. Epilepsy Res, 2014, 108(9): 1627-1636.
27. Sachdeo RC, Glauser TA, Ritter F, et al. A double-blind, randomized trial of topiramate in Lennox-Gastaut syndrome. Topiramate YL Study Group. Neurology, 1999, 52(9): 1882-1887.
28. Glauser TA, Levisohn PM, Ritter F, et al. Topiramate in Lennox-Gastaut syndrome: open-label treatment of patients completing a randomized controlled trial. Topiramate YL Study Group. Epilepsia, 2000, 41(S1): 86-90.
29. 王艺, 孙道开, 陈天兰, 等. 托吡酯治疗Lennox-Gastaut综合征47例临床分析. 临床神经电生理学杂志, 2001, 10(4): 224-225.
30. 邱鹏玲, 周水珍, 孙道开. 托吡酯单药治疗40例小儿癫痫疗效分析. 临床神经电生理学杂志, 2001, 10(4): 203-205.
31. 孙道开, 王艺, 陈天兰, 等. 托吡酯治疗小儿癫痫66例临床分析. 中国实用儿科杂志, 2001, 16(6): 356-358.
32. Devinsky O, Patel AD, Cross JH, et al. Effect of Cannabidiol on drop seizures in the Lennox-Gastaut Syndrome. N Engl J Med, 2018, 378(20): 1888-1897.
33. Thiele EA, Marsh ED, French JA, et al. Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet, 2018, 391(10125): 1085-1096.
34. Felbamate Study Group in Lennox-Gastaut Syndrome. Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). N Engl J Med, 1993, 328(1): 29-33.
35. Ng YT, Conry JA, Drummond R, et al. Randomized, phase III study results of clobazam in Lennox-Gastaut syndrome. Neurology, 2011, 77: 1473-1481.
36. Kim HJ, Kim SH, Kang HC, et al. Adjunctive levetiracetam treatment in pediatric Lennox-Gastaut syndrome. Pediatr Neurol, 2014, 51(4): 527-531.
37. You SJ, Kang HC, Kim HD, et al. Clinical efficacy of zonisamide in Lennox-Gastaut syndrome: Korean multicentric experience. Brain Dev, 2008, 30(4): 287-290.
38. Auvin S, Dozieres B, Ilea A, et al. Use of perampanel in children and adolescents with Lennox-Gastaut Syndrome. Epilepsy Behav, 2017, 74: 59-63.
39. Biró A, Stephani U, Tarallo T, et al. Effectiveness and tolerability of perampanel in children and adolescents with refractory epilepsies: first experiences. Neuropediatrics, 2015, 46(2): 110-116.
40. Zhang Y, Wang Y, Zhou Y, et al. Therapeutic effects of the ketogenic diet in children with Lennox-Gastaut syndrome. Epilepsy Res, 2016, 128: 176-180.
41. Caraballo RH, Fortini S, Fresler S, et al. Ketogenic diet in patients with Lennox-Gastaut syndrome. Seizure, 2014, 23(9): 751-755.
42. Lemmon ME, Terao NN, Ng YT, et al. Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature. Dev Med Child Neurol, 2012, 54(5): 464-468.
43. Archer JS, Warren AE, Stagnitti MR, et al. Lennox-Gastaut syndrome and phenotype: secondary network epilepsies. Epilepsia, 2014, 55(8): 1245-1254.
44. Kang JW, Eom S, Hong W, et al. Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome. Pediatrics, 2018, 142(4): e20180449.
45. 安宁, 刘仕勇, 杨梅华, 等. Lennox-Gastaut综合征的外科治疗初探. 中华神经外科疾病研究杂志, 2007, 6(4): 352-355.
46. 丁平, 于晓曼, 张军臣, 等. 切除性手术治疗学龄儿童病灶性Lennox-Gastaut 综合征. 临床神经外科杂志, 2015, 12(3): 181-184,188.
47. Lee YJ, Kang HC, Lee JS, et al. Resective pediatric epilepsy surgery in Lennox-Gastaut syndrome. Pediatrics, 2010, 125(1): e58-66.
48. Tao JX, Satzer D, Issa NP, et al. Stereotactic laser anterior corpus callosotomy for Lennox-Gastaut syndrome. Epilepsia, 2020, 61(6): 1190-1200.
49. Liang S, Zhang S, Hu X, et al. Anterior corpus callosotomy in school-aged children with Lennox-Gastaut syndrome: a prospective study. Eur J Paediatr Neurol, 2014, 18(6): 670-676.
50. Sharawat IK, Panda PK, Sihag RK, et al. Efficacy and safety of corpus callosotomy and ketogenic diet in children with Lennox Gastaut syndrome: a systematic review and meta-analysis. Childs Nerv Syst, 2021, 37(8): 2557-2566.
51. Dibué M, Greco T, Spoor JKH, et al. Vagus nerve stimulation in patients with Lennox-Gastaut syndrome: A meta-analysis. Acta Neurol Scand, 2021, 143(5): 497-508.

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Lennox-Gastaut综合征诊断治疗的中国专家共识

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