Lennox-Gastaut综合征患者的非药物治疗——生酮饮食和迷走神经刺激_Journal of Epilepsy

Authors：

 KossoffEH , ShieldsWD , 李东红 , 杨小枫

Corresponding author：

KossoffEH, Email: ekossoff@jhmi.edu

Keywords：

充足蛋白质; 辅助疗法; 中枢神经系统; 电刺激; 高脂

DOI：

10.7507/2096-0247.20160063

Video：

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Abstract Full text Figures/Tables Video References Cited by

Lennox-Gastaut综合征（Lennox-Gastaut syndrome，LGS）的患者常常对于药物治疗无效或耐药。对于这些药物难治性患者，生酮饮食（Ketogenic diets，KD）和迷走神经刺激（Vagus nerve stimulation，VNS）可作为非药物治疗的选择。传统的KD用于癫痫治疗已经有90余年的历史了，它是一种高脂、低碳水化合物饮食，其中90%的热量来自于脂肪。KD治疗对于约半数的LGS患者有效，癫痫发作可减少50%以上，有些患者癫痫发作甚至可减少90%以上。VNS疗法，需要手术植入一个刺激发生器，它可以通过缠绕在左侧迷走神经上的电极对大脑进行间断性电刺激。VNS作为辅助疗法可用于不宜手术治疗的药物难治性癫痫患者（包括LGS）。与KD相似，VNS对约半数的LGS患者有效，癫痫发作将减少50%以上，而且随时间的推移，疗效会逐渐增强。KD和VNS均可作为LGS患者的治疗选择。

Citation： KossoffEH, ShieldsWD, 李东红, 杨小枫. Lennox-Gastaut综合征患者的非药物治疗——生酮饮食和迷走神经刺激. Journal of Epilepsy, 2016, 2(4): 355-359. doi: 10.7507/2096-0247.20160063 Copy

1.	Kossoff EH, Zupec-Kania BA, Amark PE, et al. Optimal clinical management of children receiving the ketogenic diet:recommendations of the International Ketogenic Diet Study Group. Epilepsia, 2009, 50(2):304-317.
2.	Ferrie CD, Patel A. Treatment of Lennox-Gastaut syndrome (LGS). Eur J Paediatr Neurol, 2009, 13(8):493-504.
3.	Farasat S, Kossoff EH, Pillas DJ, et al. The importance of parental expectations of cognitive improvement for their children with epilepsy prior to starting the ketogenic diet. Epilepsy Behav, 2006, 8(2):406-410.
4.	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(8):464-468.
5.	Kossoff EH. International consensus statement on clinical implementation of the ketogenic diet:agreement, flexibility, and controversy. Epilepsia, 2008, 49(Suppl. 8):11-13.
6.	Neal EG, Chaffe H, Schwartz RH, et al. The ketogenic diet for the treatment of childhood epilepsy:a randomised controlled trial. Lancet Neurol, 2008, 7(1):500-506.
7.	Henderson CB, Filloux FM, Alder SC, et al. Efficacy of the ketogenic diet as a treatment option for epilepsy:meta-analysis. J Child Neurol, 2006, 21(2):193-198.
8.	Kossoff EH, Hartman AL. Ketogenic diets:new advances for metabolism-based therapies. Curr Opin Neurol, 2012, 25(8):173-178.
9.	McNally MA, Pyzik PL, Rubenstein JE, et al. Empiric use of potassium citrate reduces kidney-stone incidence with the ketogenic diet. Pediatrics, 2009, 124(8):e300-e304.
10.	Bergqvist AG, Schall JI, Gallagher PR, et al. Fasting versus gradual initiation of the ketogenic diet:a prospective, randomized clinical trial of efficacy. Epilepsia, 2005, 46(5):1810-1819.
11.	Freeman JM, Vining EP, Kossoff EH, et al. A blinded, crossover study of the efficacy of the ketogenic diet. Epilepsia, 2009, 50(11):322-325.
12.	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(5):82-93.
13.	Crumrine PK. Management of seizures in Lennox-Gastaut syndrome. Paediatr Drugs, 2011, 13(1):107-118.
14.	Hong AM, Turner Z, Hamdy RF, et al. Infantile spasms treated with the ketogenic diet:prospective single-center experience in 104 consecutive infants. Epilepsia, 2010, 51(2):1403-1407.
15.	Shahwan A, Bailey C, Maxiner W, et al. Vagus nerve stimulation for refractory epilepsy in children:more to VNS than seizure frequency reduction. Epilepsia, 2009, 50(11):1220-1228.
16.	Hajnsek S, Petelin Z, Poljakovic Z, et al. Vagus nerve stimulation in the treatment of patients with pharmacoresistant epilepsy:our experiences. Coll Antropol, 2011, 35(6):755-760.
17.	Karceski S. Vagus nerve stimulation and Lennox-Gastaut syndrome:a review of the literature and data from the VNS patient registry. CNS Spectr, 2001, 6(1):766-770.
18.	Frost M, Gates J, Helmers SL, et al. Vagus nerve stimulation in children with refractory seizures associated with Lennox-Gastaut syndrome. Epilepsia, 2001, 42(5):1148-1152.
19.	Cersosimo RO, Bartuluchi M, Fortini S, et al. Vagus nerve stimulation:effectiveness and tolerability in 64 paediatric patients with refractory epilepsies. Epileptic Disord, 2011, 13(4):382-388.
20.	Aldenkamp AP, Majoie HJ, Berfelo MW, et al. Long-term effects of 24-month treatment with vagus nerve stimulation on behaviour in children with Lennox-Gastaut syndrome. Epilepsy Behav, 2002, 3(1):475-479.
21.	Majoie HJ, Berfelo MW, Aldenkamp AP, et al. Vagus nerve stimulation in children with therapy-resistant epilepsy diagnosed as Lennox-Gastaut syndrome:clinical results, neuropsychological effects, and cost-effectiveness. J Clin Neurophysiol, 2001, 18(5):419-428.
22.	Kostov K, Kostov H, Tauboll E. Long-term vagus nerve stimulation in the treatment of Lennox-Gastaut syndrome. Epilepsy Behav, 2009, 16(3):321-324.
23.	Rychlicki F, Zamponi N, Trignani R, et al. Vagus nerve stimulation:clinical experience in drug-resistant pediatric epileptic patients. Seizure, 2006, 15(9):483-490.

1. Kossoff EH, Zupec-Kania BA, Amark PE, et al. Optimal clinical management of children receiving the ketogenic diet:recommendations of the International Ketogenic Diet Study Group. Epilepsia, 2009, 50(2):304-317.
2. Ferrie CD, Patel A. Treatment of Lennox-Gastaut syndrome (LGS). Eur J Paediatr Neurol, 2009, 13(8):493-504.
3. Farasat S, Kossoff EH, Pillas DJ, et al. The importance of parental expectations of cognitive improvement for their children with epilepsy prior to starting the ketogenic diet. Epilepsy Behav, 2006, 8(2):406-410.
4. 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(8):464-468.
5. Kossoff EH. International consensus statement on clinical implementation of the ketogenic diet:agreement, flexibility, and controversy. Epilepsia, 2008, 49(Suppl. 8):11-13.
6. Neal EG, Chaffe H, Schwartz RH, et al. The ketogenic diet for the treatment of childhood epilepsy:a randomised controlled trial. Lancet Neurol, 2008, 7(1):500-506.
7. Henderson CB, Filloux FM, Alder SC, et al. Efficacy of the ketogenic diet as a treatment option for epilepsy:meta-analysis. J Child Neurol, 2006, 21(2):193-198.
8. Kossoff EH, Hartman AL. Ketogenic diets:new advances for metabolism-based therapies. Curr Opin Neurol, 2012, 25(8):173-178.
9. McNally MA, Pyzik PL, Rubenstein JE, et al. Empiric use of potassium citrate reduces kidney-stone incidence with the ketogenic diet. Pediatrics, 2009, 124(8):e300-e304.
10. Bergqvist AG, Schall JI, Gallagher PR, et al. Fasting versus gradual initiation of the ketogenic diet:a prospective, randomized clinical trial of efficacy. Epilepsia, 2005, 46(5):1810-1819.
11. Freeman JM, Vining EP, Kossoff EH, et al. A blinded, crossover study of the efficacy of the ketogenic diet. Epilepsia, 2009, 50(11):322-325.
12. 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(5):82-93.
13. Crumrine PK. Management of seizures in Lennox-Gastaut syndrome. Paediatr Drugs, 2011, 13(1):107-118.
14. Hong AM, Turner Z, Hamdy RF, et al. Infantile spasms treated with the ketogenic diet:prospective single-center experience in 104 consecutive infants. Epilepsia, 2010, 51(2):1403-1407.
15. Shahwan A, Bailey C, Maxiner W, et al. Vagus nerve stimulation for refractory epilepsy in children:more to VNS than seizure frequency reduction. Epilepsia, 2009, 50(11):1220-1228.
16. Hajnsek S, Petelin Z, Poljakovic Z, et al. Vagus nerve stimulation in the treatment of patients with pharmacoresistant epilepsy:our experiences. Coll Antropol, 2011, 35(6):755-760.
17. Karceski S. Vagus nerve stimulation and Lennox-Gastaut syndrome:a review of the literature and data from the VNS patient registry. CNS Spectr, 2001, 6(1):766-770.
18. Frost M, Gates J, Helmers SL, et al. Vagus nerve stimulation in children with refractory seizures associated with Lennox-Gastaut syndrome. Epilepsia, 2001, 42(5):1148-1152.
19. Cersosimo RO, Bartuluchi M, Fortini S, et al. Vagus nerve stimulation:effectiveness and tolerability in 64 paediatric patients with refractory epilepsies. Epileptic Disord, 2011, 13(4):382-388.
20. Aldenkamp AP, Majoie HJ, Berfelo MW, et al. Long-term effects of 24-month treatment with vagus nerve stimulation on behaviour in children with Lennox-Gastaut syndrome. Epilepsy Behav, 2002, 3(1):475-479.
21. Majoie HJ, Berfelo MW, Aldenkamp AP, et al. Vagus nerve stimulation in children with therapy-resistant epilepsy diagnosed as Lennox-Gastaut syndrome:clinical results, neuropsychological effects, and cost-effectiveness. J Clin Neurophysiol, 2001, 18(5):419-428.
22. Kostov K, Kostov H, Tauboll E. Long-term vagus nerve stimulation in the treatment of Lennox-Gastaut syndrome. Epilepsy Behav, 2009, 16(3):321-324.
23. Rychlicki F, Zamponi N, Trignani R, et al. Vagus nerve stimulation:clinical experience in drug-resistant pediatric epileptic patients. Seizure, 2006, 15(9):483-490.

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