儿童期全身性癫痫发作

• How do antiepileptic drugs compare for people with generalized tonic-clonic seizures?
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• What are the effects of rufinamide as an adjunct to conventional antiepileptic drug (AED) therapy for people with refractory epilepsy?
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• What are the benefits and harms of treatment for prevention of sudden unexpected death in epilepsy (SUDEP)?
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• How do early and late antiepileptic drug withdrawal compare in children with epilepsy in remission?
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证据评分

证据 A 癫痫发作控制：有良好的证据证明，经颊黏膜/经鼻咪达唑仑与直肠地西泮相比，在急性全身性癫痫发作中更为有效。[49]McIntyre J, Robertson S, Norris E, et al. Safety and efficacy of buccal midazolam versus rectal diazepam for emergency treatment of seizures in children: a randomised controlled trial. Lancet. 2005 Jul 16-22;366(9481):205-10.http://www.ncbi.nlm.nih.gov/pubmed/16023510?tool=bestpractice.com[50]Holsti M, Sill BL, Firth SD, et al. Prehospital intranasal midazolam for the treatment of pediatric seizures. Pediatr Emerg Care. 2007 Mar;23(3):148-53.http://www.ncbi.nlm.nih.gov/pubmed/17413428?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有良好的证据证明，经颊黏膜/经鼻咪达唑仑与直肠地西泮相比，在急性全身性癫痫发作中更为有效。[49]McIntyre J, Robertson S, Norris E, et al. Safety and efficacy of buccal midazolam versus rectal diazepam for emergency treatment of seizures in children: a randomised controlled trial. Lancet. 2005 Jul 16-22;366(9481):205-10.http://www.ncbi.nlm.nih.gov/pubmed/16023510?tool=bestpractice.com[50]Holsti M, Sill BL, Firth SD, et al. Prehospital intranasal midazolam for the treatment of pediatric seizures. Pediatr Emerg Care. 2007 Mar;23(3):148-53.http://www.ncbi.nlm.nih.gov/pubmed/17413428?tool=bestpractice.com

证据 A 癫痫发作控制：有良好的证据证明，在未分类的全身性癫痫患者中，丙戊酸盐的耐受性优于托吡酯，且较拉莫三嗪更为有效。[99]Marson AG, Al-Kharusi AM, Alwaidh M, et al; SANAD Study group. The SANAD study of effectiveness of valproate, lamotrigine, or topiramate for generalised and unclassifiable epilepsy: an unblinded randomised controlled trial. Lancet. 2007 Mar 24;369(9566):1016-26.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2039891/http://www.ncbi.nlm.nih.gov/pubmed/17382828?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有良好的证据证明，在未分类的全身性癫痫患者中，丙戊酸盐的耐受性优于托吡酯，且较拉莫三嗪更为有效。[99]Marson AG, Al-Kharusi AM, Alwaidh M, et al; SANAD Study group. The SANAD study of effectiveness of valproate, lamotrigine, or topiramate for generalised and unclassifiable epilepsy: an unblinded randomised controlled trial. Lancet. 2007 Mar 24;369(9566):1016-26.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2039891/http://www.ncbi.nlm.nih.gov/pubmed/17382828?tool=bestpractice.com

证据 B 癫痫发作控制：有中等质量的证据证明，托吡酯辅助治疗对婴儿期患有婴儿痉挛、Lennox-Gastaut 综合征和重度肌阵挛性癫痫的小儿原发性全身性癫痫有效。[62]Zou LP, Ding CH, Fang F, et al. Prospective study of first-choice topiramate therapy in newly diagnosed infantile spasms. Clin Neuropharmacol. 2006 Nov-Dec;29(6):343-9.http://www.ncbi.nlm.nih.gov/pubmed/17095898?tool=bestpractice.com[63]Korinthenberg R, Schreiner A. Topiramate in children with West syndrome: a retrospective multicenter evaluation of 100 patients. J Child Neurol. 2007 Mar;22(3):302-6.http://www.ncbi.nlm.nih.gov/pubmed/17621500?tool=bestpractice.com[74]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 Jun 10;52(9):1882-7.http://www.ncbi.nlm.nih.gov/pubmed/10371538?tool=bestpractice.com[73]Nieto-Barrera M, Candau R, Nieto-Jimenez M, et al. Topiramate in the treatment of severe myoclonic epilepsy in infancy. Seizure. 2000 Dec;9(8):590-4.http://www.ncbi.nlm.nih.gov/pubmed/11162758?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有中等质量的证据证明，托吡酯辅助治疗对婴儿期患有婴儿痉挛、Lennox-Gastaut 综合征和重度肌阵挛性癫痫的小儿原发性全身性癫痫有效。[62]Zou LP, Ding CH, Fang F, et al. Prospective study of first-choice topiramate therapy in newly diagnosed infantile spasms. Clin Neuropharmacol. 2006 Nov-Dec;29(6):343-9.http://www.ncbi.nlm.nih.gov/pubmed/17095898?tool=bestpractice.com[63]Korinthenberg R, Schreiner A. Topiramate in children with West syndrome: a retrospective multicenter evaluation of 100 patients. J Child Neurol. 2007 Mar;22(3):302-6.http://www.ncbi.nlm.nih.gov/pubmed/17621500?tool=bestpractice.com[74]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 Jun 10;52(9):1882-7.http://www.ncbi.nlm.nih.gov/pubmed/10371538?tool=bestpractice.com[73]Nieto-Barrera M, Candau R, Nieto-Jimenez M, et al. Topiramate in the treatment of severe myoclonic epilepsy in infancy. Seizure. 2000 Dec;9(8):590-4.http://www.ncbi.nlm.nih.gov/pubmed/11162758?tool=bestpractice.com

证据 B 癫痫发作控制：有中等质量的证据证明，拉莫三嗪辅助治疗与安慰剂相比，在控制儿童和青少年原发性全身性癫痫中更为有效。[81]Motte J, Trevathan E, Arvidsson JF, et al; Lamictal Lennox-Gastaut Study Group. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. N Engl J Med. 1997 Dec 18;337(25):1807-12.http://www.nejm.org/doi/full/10.1056/NEJM199712183372504#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/9400037?tool=bestpractice.com[82]Trevathan E, Kerls SP, Hammer AE, et al. Lamotrigine adjunctive therapy among children and adolescents with primary generalized tonic-clonic seizures. Pediatrics. 2006 Aug;118(2):e371-8.http://www.ncbi.nlm.nih.gov/pubmed/16847080?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有中等质量的证据证明，拉莫三嗪辅助治疗与安慰剂相比，在控制儿童和青少年原发性全身性癫痫中更为有效。[81]Motte J, Trevathan E, Arvidsson JF, et al; Lamictal Lennox-Gastaut Study Group. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. N Engl J Med. 1997 Dec 18;337(25):1807-12.http://www.nejm.org/doi/full/10.1056/NEJM199712183372504#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/9400037?tool=bestpractice.com[82]Trevathan E, Kerls SP, Hammer AE, et al. Lamotrigine adjunctive therapy among children and adolescents with primary generalized tonic-clonic seizures. Pediatrics. 2006 Aug;118(2):e371-8.http://www.ncbi.nlm.nih.gov/pubmed/16847080?tool=bestpractice.com

证据 B 癫痫发作控制：有中等质量的证据证明，非尔氨酯与安慰剂相比，对 Lennox-Gastaut 综合征患者更为有效。[83]The Felbamate Study Group in Lennox-Gastaut Syndrome. Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). N Engl J Med. 1993 Jan 7;328(1):29-33.http://www.nejm.org/doi/full/10.1056/NEJM199301073280105#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/8347179?tool=bestpractice.com[84]Dodson WE. Felbamate in the treatment of Lennox-Gastaut syndrome: results of a 12-month open-label study following a randomized clinical trial. Epilepsia. 1993;34(suppl 7):S18-24.http://www.ncbi.nlm.nih.gov/pubmed/8243374?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有中等质量的证据证明，非尔氨酯与安慰剂相比，对 Lennox-Gastaut 综合征患者更为有效。[83]The Felbamate Study Group in Lennox-Gastaut Syndrome. Efficacy of felbamate in childhood epileptic encephalopathy (Lennox-Gastaut syndrome). N Engl J Med. 1993 Jan 7;328(1):29-33.http://www.nejm.org/doi/full/10.1056/NEJM199301073280105#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/8347179?tool=bestpractice.com[84]Dodson WE. Felbamate in the treatment of Lennox-Gastaut syndrome: results of a 12-month open-label study following a randomized clinical trial. Epilepsia. 1993;34(suppl 7):S18-24.http://www.ncbi.nlm.nih.gov/pubmed/8243374?tool=bestpractice.com

证据 B 癫痫发作控制：有中等质量的证据证明，在新确诊的混合型癫痫患者中，托吡酯单药治疗与其他抗惊厥药同样有效。[100]Wirrell E, Camfield C, Camfield P, et al. Prognostic significance of failure of the initial antiepileptic drug in children with absence epilepsy. Epilepsia. 2001 Jun;42(6):760-3.http://www.ncbi.nlm.nih.gov/pubmed/11422332?tool=bestpractice.com[103]Wheless JW, Neto W, Wang S; EPMN-105 Study Group. Topiramate, carbamazepine, and valproate monotherapy: double-blind comparison in children with newly diagnosed epilepsy. J Child Neurol. 2004 Feb;19(2):135-41.http://www.ncbi.nlm.nih.gov/pubmed/15072107?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有中等质量的证据证明，在新确诊的混合型癫痫患者中，托吡酯单药治疗与其他抗惊厥药同样有效。[100]Wirrell E, Camfield C, Camfield P, et al. Prognostic significance of failure of the initial antiepileptic drug in children with absence epilepsy. Epilepsia. 2001 Jun;42(6):760-3.http://www.ncbi.nlm.nih.gov/pubmed/11422332?tool=bestpractice.com[103]Wheless JW, Neto W, Wang S; EPMN-105 Study Group. Topiramate, carbamazepine, and valproate monotherapy: double-blind comparison in children with newly diagnosed epilepsy. J Child Neurol. 2004 Feb;19(2):135-41.http://www.ncbi.nlm.nih.gov/pubmed/15072107?tool=bestpractice.com

证据 C 癫痫发作控制：有质量较差的证据证明，迷走神经刺激对顽固性癫痫发作有效。[107]Morris GL 3rd, Gloss D, Buchhalter J, et al. Evidence-based guideline update: vagus nerve stimulation for the treatment of epilepsy. Neurology. 2013 Oct 15;81(16):1453-9.http://www.neurology.org/content/81/16/1453.longhttp://www.ncbi.nlm.nih.gov/pubmed/23986299?tool=bestpractice.com 系统评价或者受试者＞200名的随机对照临床试验（RCT）。

癫痫发作控制：有质量较差的证据证明，迷走神经刺激对顽固性癫痫发作有效。[107]Morris GL 3rd, Gloss D, Buchhalter J, et al. Evidence-based guideline update: vagus nerve stimulation for the treatment of epilepsy. Neurology. 2013 Oct 15;81(16):1453-9.http://www.neurology.org/content/81/16/1453.longhttp://www.ncbi.nlm.nih.gov/pubmed/23986299?tool=bestpractice.com