癲癇伴慢波睡眠期持續棘慢波(CSWS)患者的治療:北美治療選擇的調查_Journal of Epilepsy

Authors：

SánchezFernández I , ChapmanK , PetersJM , KlehmJ , JacksonMC , BergAT ,  LoddenkemperT

Corresponding?author：

LoddenkemperT, Email: tobias.Loddenkemper@childrens.harvard.edu

Keywords：

抗癲癇藥物; 腦電圖; 癲癇; 癲癇發作; 睡眠; 治療

DOI：

10.7507/2096-0247.20160080

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現有文獻沒能為癲癇伴慢波睡眠期持續棘慢波(Continuous spikes and waves during sleep, CSWS)患者的治療方法提供充分的證據。文章的目的在于描述北美臨床醫生在面對CSWS患者時做出的治療選擇。在美國癲癇學會(American Epilepsy Society, AES)成員中進行了關于CSWS治療選擇的問卷調查, 調查包含24項題目。問卷中描述了CSWS的治療臨床情境。題目詢問的是在該臨床情境下的治療選擇。被調查人通過在線調查網站(www.surveymonkey.com)自行填寫問卷收集。完成問卷共計232份, 81%的調查對象認為明顯的睡眠棘波增強應當治療。睡眠棘波增強在經過有效治療之后, 調查對象表示16%的患者的認知功能有>75%的改善, 52%的患者有25%~75%的改善, 20%的患者有 < 25%的改善, 還有12%的調查對象稱患者的認知功能并沒有明確的改變。減少睡眠誘發的癇樣放電增強的首選療法分別為:高劑量苯二氮類藥物(47%)、丙戊酸(26%)和皮質類固醇(15%)。次選療法分別為:丙戊酸(26%)、高劑量苯二氮卓類藥物(24%)和皮質類固醇(23%)。在高劑量苯二氮卓類藥物中, 最常用的是地西泮, 第一夜用1 mg/kg, 之后用0.5 mg/(kg·d)。丙戊酸的常用劑量為30~49 mg/(kg·d)。在皮質類固醇中, 最常用的是口服潑尼松2 mg/(kg·d)。終止治療的療效指標有(從強到弱排列):腦電圖上癲癇樣放電的相應改變、認知功能好轉、發作減少。在培訓水平及臨床經驗不同的調查對象中, 結果是一致的。在兒童和成人神經科醫生之間, 存在著概念和治療方法的差異。大部分臨床醫生認為睡眠期明顯增強的癇樣放電是應當治療的。調查對象們沒有就最佳療法的問題達成一致, 但可選療法包括了:高劑量苯二氮卓類藥物、丙戊酸、左乙拉西坦和皮質類固醇。

Citation： SánchezFernández I, ChapmanK, PetersJM, KlehmJ, JacksonMC, BergAT, LoddenkemperT. 癲癇伴慢波睡眠期持續棘慢波(CSWS)患者的治療:北美治療選擇的調查. Journal of Epilepsy, 2016, 2(5): 446-453. doi: 10.7507/2096-0247.20160080 Copy

1.	Sanchez Ferandez I, Loddenkemper T, Peters JM, et al. Electrical status epilepticus in sleep:clinical presentation and pathophysiology. PediatrNeurol, 2012, 47(10):390-410.
2.	Tassinari CA, Rubboli G, Volpi L, et al. Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia. ClinNeurophysiol, 2000, 111(Suppl. 2):94-102.
3.	Sanchez Fernandez I, Chapman KE, Peters JM, et al. The tower of Babel:survey on concepts and terminology in electrical status epilepticus in sleep and continuous spikes and waves during sleep in North America. Epilepsia, 2013, 54(6):741-750.
4.	Sanchez Fernandez I, Peters JM, Hadjiloizou S, et al. Clinical staging and electroencephalographic evolution of continuous spikes and waves during sleep. Epilepsia, 2012, 53(5):1185-1195.
5.	Nickels K, Wirrell E. Electrical status epilepticus in sleep. SeminPediatrNeurol, 2008, 15(4):50-60.
6.	Sanchez Fernandez I, Chapman KE, Peters JM, et al. Continuous spikes and waves during sleep:electroclinical presentation and suggestions for management. Epilepsy Res Treat, 2013, 20(3):583531.
7.	Kleen JK, Scott RC, Holmes GL, et al. Hippocampal interictal epileptiform activity disrupts cognition in humans. Neurology, 2013, 81(4):18-24.
8.	Diekelmann S, Born J. The memory function of sleep. Nat Rev Neurosci, 2010, 11(4):114-126.
9.	Aldenkamp AP, Arends J. Effects of epileptiform EEG discharges on cognitive function:is the concept of "transient cognitive impairment"still valid?. Epilepsy Behav, 2004, 5(Suppl. 1):25-34.
10.	Nicolai J, Ebus S, Biemans DP, et al. The cognitive effects of interictal epileptiform EEG discharges and short nonconvulsive epileptic seizures. Epilepsia, 2012, 53(11):1051-1059.
11.	Bjorn?s H, Bakke KA, Larsson PG, et al. Subclinical epileptiform activity in children with electrical status epilepticus during sleep:effects on cognition and behavior before and after treatment with levetiracetam. Epilepsy Behav, 2013, 27(2):40-48.
12.	Ortinski P, Meador KJ. Cognitive side effects of antiepileptic drugs. Epilepsy Behav, 2004, 5(Suppl. 1):60-65.
13.	Caraballo RH, Bongiorni L, Cersosimo R, et al. Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus:a study of nine cases. Epilepsia, 2008, 49(8):1520-1527.
14.	Inutsuka M, Kobayashi K, Oka M, et al. Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders. Brain Dev, 2006, 28(2):281-286.
15.	Liukkonen E, Kantola-Sorsa E, Paetau R, et al. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia, 2010, 51(6):2023-2032.
16.	Nieuwenhuis L, Nicolai J. The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep. Seizure, 2006, 15(8):249-258.
17.	Rossi PG, Parmeggiani A, Posar A, et al. Landau-Kleffner syndrome (LKS):long-term follow-up and links with electrical status epilepticus during sleep (ESES). Brain Dev, 1999, 21(9):90-98.
18.	Sinclair DB, Snyder TJ. Corticosteroids for the treatment of Landaukleffner syndrome and continuous spike-wave discharge during sleep. PediatrNeurol, 2005, 32(11):300-306.
19.	De Negri M, Baglietto MG, Battaglia FM, et al. Treatment of electrical status epilepticus by short diazepam (DZP) cycles after DZP rectal bolus test. Brain Dev, 1995, 17(5):330-333.
20.	Sanchez Fernandez I, Hadjiloizou S, Eksioglu Y, et al. Short-term response of sleep-potentiated spiking to high-dose diazepam in electric status epilepticus during sleep. PediatrNeurol, 2012, 46(10):312-318.
21.	Sanchez Fernandez I, Peters JM, An S, et al. Long-term response to high-dose diazepam treatment in continuous spikes and waves during sleep. PediatrNeurol, 2013, 49(5):163-170.
22.	Kramer U, Sagi L, Goldberg-Stern H, et al.Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES). Epilepsia, 2009, 50(7):1517-1524.
23.	Capovilla G, Beccaria F, Cagdas S, et al. Efficacy of levetiracetam in pharmacoresistant continuous spikes and waves during slow sleep. ActaNeurolScand, 2004, 110(5):144-147.
24.	Hoppen T, Sandrieser T, Rister M. Successful treatment of pharmacoresistent continuous spike wave activity during slow sleep with levetiracetam. Eur J Pediatr, 2003, 162(3):59-61.
25.	Okuyaz C, Aydin K, Gucuyener K, et al.Treatment of electrical status epilepticus during slow-wave sleep with high-dose corticosteroid. PediatrNeurol, 2005, 32(6):64-67.
26.	Scholtes FB, Hendriks MP, Renier WO. Cognitive deterioration and electrical status epilepticus during slow sleep. Epilepsy Behav, 2005, 6(2):167-173.
27.	Aeby A, Poznanski N, Verheulpen D, et al. Levetiracetam efficacy in epileptic syndromes with continuous spikes and waves during slow sleep:experience in 12 cases. Epilepsia, 2005, 46(10):1937-1942.
28.	Larsson PG, Bakke KA, Bjornaes H, et al. The effect of levetiracetam on focal nocturnal epileptiform activity during sleep-aplacebo-controlled double-blind cross-over study. Epilepsy Behav, 2012, 24(8):44-48.
29.	Wang SB, Weng WC, Fan PC, et al.Levetiracetam in continuous spike waves during slow-wave sleep syndrome. PediatrNeurol, 2008, 39(11):85-90.
30.	Buzatu M, Bulteau C, Altuzarra C, et al.Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia, 2009, 50(Suppl. 7):68-72.
31.	Arts WF, Aarsen FK, Scheltens-de Boer M, et al. Landau-Kleffner syndrome and CSWS syndrome:treatment with intravenous immunoglobulins. Epilepsia, 2009, 50(Suppl. 7):55-58.
32.	Bergqvist AG, Chee CM, Lutchka LM, et al.Treatment of acquired epileptic aphasia with the ketogenic diet. J Child Neurol, 1999, 14(2):696-701.
33.	Park YD. The effects of vagus nerve stimulation therapy on patients with intractable seizures and either Landau-Kleffner syndrome or autism. Epilepsy Behav, 2003, 4(1):286-290.
34.	Battaglia D, Veggiotti P, Lettori D, et al. Functional hemispherectomy in children with epilepsy and CSWS due to unilateral early brain injury including thalamus:sudden recovery of CSWS. Epilepsy Res, 2009, 87(10):290-298.
35.	Guzzetta F, Battaglia D, Veredice C, et al. Early thalamic injury associated with epilepsy and continuous spike-wave during slow sleep. Epilepsia, 2005, 46(8):889-900.
36.	Loddenkemper T, Cosmo G, Kotagal P, et al. Epilepsy surgery in children with electrical status epilepticus in sleep. Neurosurgery, 2009, 64(3):328-337.discussion 37.
37.	Wyllie E, Lachhwani DK, Gupta A, et al.Successful surgery for epilepsy due to early brain lesions despite generalized EEG findings. Neurology, 2007, 69(3):389-397.
38.	Yan Liu X, Wong V. Spectrum of epileptic syndromes with electrical status epilepticus during sleep in children. PediatrNeurol, 2000, 22(5):371-379.
39.	Peltola ME, Liukkonen E, Granstrom ML, et al.The effect of surgery in encephalopathy with electrical status epilepticus during sleep. Epilepsia, 2011, 52(2):602-609.

1. Sanchez Ferandez I, Loddenkemper T, Peters JM, et al. Electrical status epilepticus in sleep:clinical presentation and pathophysiology. PediatrNeurol, 2012, 47(10):390-410.
2. Tassinari CA, Rubboli G, Volpi L, et al. Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia. ClinNeurophysiol, 2000, 111(Suppl. 2):94-102.
3. Sanchez Fernandez I, Chapman KE, Peters JM, et al. The tower of Babel:survey on concepts and terminology in electrical status epilepticus in sleep and continuous spikes and waves during sleep in North America. Epilepsia, 2013, 54(6):741-750.
4. Sanchez Fernandez I, Peters JM, Hadjiloizou S, et al. Clinical staging and electroencephalographic evolution of continuous spikes and waves during sleep. Epilepsia, 2012, 53(5):1185-1195.
5. Nickels K, Wirrell E. Electrical status epilepticus in sleep. SeminPediatrNeurol, 2008, 15(4):50-60.
6. Sanchez Fernandez I, Chapman KE, Peters JM, et al. Continuous spikes and waves during sleep:electroclinical presentation and suggestions for management. Epilepsy Res Treat, 2013, 20(3):583531.
7. Kleen JK, Scott RC, Holmes GL, et al. Hippocampal interictal epileptiform activity disrupts cognition in humans. Neurology, 2013, 81(4):18-24.
8. Diekelmann S, Born J. The memory function of sleep. Nat Rev Neurosci, 2010, 11(4):114-126.
9. Aldenkamp AP, Arends J. Effects of epileptiform EEG discharges on cognitive function:is the concept of "transient cognitive impairment"still valid?. Epilepsy Behav, 2004, 5(Suppl. 1):25-34.
10. Nicolai J, Ebus S, Biemans DP, et al. The cognitive effects of interictal epileptiform EEG discharges and short nonconvulsive epileptic seizures. Epilepsia, 2012, 53(11):1051-1059.
11. Bjorn?s H, Bakke KA, Larsson PG, et al. Subclinical epileptiform activity in children with electrical status epilepticus during sleep:effects on cognition and behavior before and after treatment with levetiracetam. Epilepsy Behav, 2013, 27(2):40-48.
12. Ortinski P, Meador KJ. Cognitive side effects of antiepileptic drugs. Epilepsy Behav, 2004, 5(Suppl. 1):60-65.
13. Caraballo RH, Bongiorni L, Cersosimo R, et al. Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus:a study of nine cases. Epilepsia, 2008, 49(8):1520-1527.
14. Inutsuka M, Kobayashi K, Oka M, et al. Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders. Brain Dev, 2006, 28(2):281-286.
15. Liukkonen E, Kantola-Sorsa E, Paetau R, et al. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia, 2010, 51(6):2023-2032.
16. Nieuwenhuis L, Nicolai J. The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep. Seizure, 2006, 15(8):249-258.
17. Rossi PG, Parmeggiani A, Posar A, et al. Landau-Kleffner syndrome (LKS):long-term follow-up and links with electrical status epilepticus during sleep (ESES). Brain Dev, 1999, 21(9):90-98.
18. Sinclair DB, Snyder TJ. Corticosteroids for the treatment of Landaukleffner syndrome and continuous spike-wave discharge during sleep. PediatrNeurol, 2005, 32(11):300-306.
19. De Negri M, Baglietto MG, Battaglia FM, et al. Treatment of electrical status epilepticus by short diazepam (DZP) cycles after DZP rectal bolus test. Brain Dev, 1995, 17(5):330-333.
20. Sanchez Fernandez I, Hadjiloizou S, Eksioglu Y, et al. Short-term response of sleep-potentiated spiking to high-dose diazepam in electric status epilepticus during sleep. PediatrNeurol, 2012, 46(10):312-318.
21. Sanchez Fernandez I, Peters JM, An S, et al. Long-term response to high-dose diazepam treatment in continuous spikes and waves during sleep. PediatrNeurol, 2013, 49(5):163-170.
22. Kramer U, Sagi L, Goldberg-Stern H, et al.Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES). Epilepsia, 2009, 50(7):1517-1524.
23. Capovilla G, Beccaria F, Cagdas S, et al. Efficacy of levetiracetam in pharmacoresistant continuous spikes and waves during slow sleep. ActaNeurolScand, 2004, 110(5):144-147.
24. Hoppen T, Sandrieser T, Rister M. Successful treatment of pharmacoresistent continuous spike wave activity during slow sleep with levetiracetam. Eur J Pediatr, 2003, 162(3):59-61.
25. Okuyaz C, Aydin K, Gucuyener K, et al.Treatment of electrical status epilepticus during slow-wave sleep with high-dose corticosteroid. PediatrNeurol, 2005, 32(6):64-67.
26. Scholtes FB, Hendriks MP, Renier WO. Cognitive deterioration and electrical status epilepticus during slow sleep. Epilepsy Behav, 2005, 6(2):167-173.
27. Aeby A, Poznanski N, Verheulpen D, et al. Levetiracetam efficacy in epileptic syndromes with continuous spikes and waves during slow sleep:experience in 12 cases. Epilepsia, 2005, 46(10):1937-1942.
28. Larsson PG, Bakke KA, Bjornaes H, et al. The effect of levetiracetam on focal nocturnal epileptiform activity during sleep-aplacebo-controlled double-blind cross-over study. Epilepsy Behav, 2012, 24(8):44-48.
29. Wang SB, Weng WC, Fan PC, et al.Levetiracetam in continuous spike waves during slow-wave sleep syndrome. PediatrNeurol, 2008, 39(11):85-90.
30. Buzatu M, Bulteau C, Altuzarra C, et al.Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia, 2009, 50(Suppl. 7):68-72.
31. Arts WF, Aarsen FK, Scheltens-de Boer M, et al. Landau-Kleffner syndrome and CSWS syndrome:treatment with intravenous immunoglobulins. Epilepsia, 2009, 50(Suppl. 7):55-58.
32. Bergqvist AG, Chee CM, Lutchka LM, et al.Treatment of acquired epileptic aphasia with the ketogenic diet. J Child Neurol, 1999, 14(2):696-701.
33. Park YD. The effects of vagus nerve stimulation therapy on patients with intractable seizures and either Landau-Kleffner syndrome or autism. Epilepsy Behav, 2003, 4(1):286-290.
34. Battaglia D, Veggiotti P, Lettori D, et al. Functional hemispherectomy in children with epilepsy and CSWS due to unilateral early brain injury including thalamus:sudden recovery of CSWS. Epilepsy Res, 2009, 87(10):290-298.
35. Guzzetta F, Battaglia D, Veredice C, et al. Early thalamic injury associated with epilepsy and continuous spike-wave during slow sleep. Epilepsia, 2005, 46(8):889-900.
36. Loddenkemper T, Cosmo G, Kotagal P, et al. Epilepsy surgery in children with electrical status epilepticus in sleep. Neurosurgery, 2009, 64(3):328-337.discussion 37.
37. Wyllie E, Lachhwani DK, Gupta A, et al.Successful surgery for epilepsy due to early brain lesions despite generalized EEG findings. Neurology, 2007, 69(3):389-397.
38. Yan Liu X, Wong V. Spectrum of epileptic syndromes with electrical status epilepticus during sleep in children. PediatrNeurol, 2000, 22(5):371-379.
39. Peltola ME, Liukkonen E, Granstrom ML, et al.The effect of surgery in encephalopathy with electrical status epilepticus during sleep. Epilepsia, 2011, 52(2):602-609.

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癲癇伴慢波睡眠期持續棘慢波(CSWS)患者的治療:北美治療選擇的調查

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