新发难治性癫痫持续状态研究进展_Journal of Epilepsy

Authors：

董军 , 张鹏 ,  陈阳美

重庆医科大学附属第二医院神经内科 (重庆 400010);

Corresponding author：

陈阳美, Email: chenym1997@sina.com

Keywords：

癫痫持续状态; 新发难治性癫痫持续状态; 病因; 免疫治疗

DOI：

10.7507/2096-0247.20190006

Video：

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新发难治性癫痫持续状态 (New-onset refractory status epilepticus, NORSE) 是一种临床表现，而非特异性诊断，是指在无活动性癫痫或其他现存相关神经系统疾病的患者中，首次出现与急性中毒、急性代谢紊乱或急性脑结构性病变无关的难治性癫痫持续状态。NORSE 患者脑电图、神经影像学、常规脑脊液检查等缺乏特异性改变。约 50% 的 NORSE 患者可找到明确病因，其中自身免疫性脑炎约占 2/3。目前，隐源性 NORSE 尚无有效治疗方法，传统抗癫痫药物及镇静剂对其疗效差。近年来有研究发现免疫治疗对隐源性 NORSE 患者癫痫持续状态的控制效果优于传统抗癫痫药物及镇静剂，但其免疫治疗方案尚未达成共识，亟待进一步多中心临床研究。现将 NORSE 的流行病学、发病机制、临床特征及治疗等方面作一综述。

Citation： 董军, 张鹏, 陈阳美. 新发难治性癫痫持续状态研究进展. Journal of Epilepsy, 2019, 5(1): 30-33. doi: 10.7507/2096-0247.20190006 Copy

1.	Wilder-Smith EP, Lim EC, Teoh HL, et al. The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity. Ann Acad Med Singapore, 2005, 34(7): 417-420.
2.	Baxter P, Clarke A, Cross H, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure, 2003, 12(6): 379-387.
3.	van Baalen A, Häusler M, Boor R, et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia, 2010, 51(7): 1323-1328.
4.	Sakuma H, Awaya Y, Shiomi M, et al. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand, 2010, 121(4): 251-256.
5.	Mikaeloff Y, Jambaque I, Hertz-Pannier L, et al. Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis. Epilepsy Res, 2006, 69(1): 67-79.
6.	Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia, 2018, 59(4): 739-744.
7.	Nabbout R, Vezzani A, Dulac O, Chiron C. Acute encephalopathy with inflammation-mediated status epilepticus. Lancet Neurol, 2011, 10(1): 99-108.
8.	Sakuma H, Tanuma N, Kuki I, et al. Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry, 2015, 86(7): 820-822.
9.	Juhász C, Buth A, Chugani DC, et al. Successful surgical treatment of an inflammatory lesion associated with new-onset refractory status epilepticus. Neurosurg Focus, 2013, 34(6): E5.
10.	Vezzani A, Balosso S, Ravizza T. The role of cytokines in the pathophysiology of epilepsy. Brain Behav Immun, 2008, 22(6): 797-803.
11.	Gall CR, Jumma O, Mohanraj R. Five cases of new onset refractory status epilepticus (NORSE) syndrome: outcomes with early immunotherapy. Seizure, 2013, 22(3): 217-220.
12.	Khawaja AM, DeWolfe JL, Miller DW, et al. New-onset refractory status epilepticus (NORSE)−The potential role for immunotherapy. Epilepsy Behav, 2015, 47: 17-23.
13.	Gaspard N, Foreman BP, Alvarez V, et al. New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology, 2015, 85(18): 1604-1613.
14.	Iizuka T, Kanazawa N, Kaneko J, et al. Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy. Neurol Neuroimmunol Neuroinflamm, 2017, 4(6): e396.
15.	Costello DJ, Kilbride RD, Cole AJ. Cryptogenic new onset refractory status epilepticus (NORSE) in adults-infectious or not. J Neurol Sci, 2009, 277(1-2): 26-31.
16.	Meletti S, Giovannini G, d’Orsi G, et al. New-onset refractory status epilepticus with claustrum damage: definition of the clinical and neuroimaging features. Front Neurol, 2017, 8: 111.
17.	De Keyser J. Status epilepticus with neuron-reactive serum antibodies: response to plasma exchange. Neurology, 2000, 55(9): 1421.
18.	王学峰, 王康, 肖波. 成人全面性惊厥性癫痫持续状态治疗中国专家共识. 国际神经病学神经外科学杂志, 2018, 45(01): 1-4.
19.	中华医学会神经病学分会脑电图与癫痫学组. 非惊厥性癫痫持续状态的治疗专家共识. 中华神经科杂志, 2013, 46(2): 133-137.
20.	Trinka E, Cock H, Hesdorffer D, et al. A definition and classification of status epilepticus−Report of the ILAE Task Force on Classification of Status Epilepticus. Epilepsia, 2015, 56(10): 1515-1523.
21.	Van Lierde I, Van Paesschen W, Dupont P, et al. De novo cryptogenic refractory multifocal febrile status epilepticus in the young adult: a review of six cases. Acta Neurol Belg, 2003, 103(2): 88-94.
22.	Li J, Saldivar C, Maganti RK. Plasma exchange in cryptogenic new onset refractory status epilepticus. Seizure, 2013, 22(1): 70-73.
23.	Gaspard N, Hirsch LJ, Sculier C, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia, 2018, 59(4): 745-752.

1. Wilder-Smith EP, Lim EC, Teoh HL, et al. The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity. Ann Acad Med Singapore, 2005, 34(7): 417-420.
2. Baxter P, Clarke A, Cross H, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure, 2003, 12(6): 379-387.
3. van Baalen A, Häusler M, Boor R, et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia, 2010, 51(7): 1323-1328.
4. Sakuma H, Awaya Y, Shiomi M, et al. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand, 2010, 121(4): 251-256.
5. Mikaeloff Y, Jambaque I, Hertz-Pannier L, et al. Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis. Epilepsy Res, 2006, 69(1): 67-79.
6. Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia, 2018, 59(4): 739-744.
7. Nabbout R, Vezzani A, Dulac O, Chiron C. Acute encephalopathy with inflammation-mediated status epilepticus. Lancet Neurol, 2011, 10(1): 99-108.
8. Sakuma H, Tanuma N, Kuki I, et al. Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry, 2015, 86(7): 820-822.
9. Juhász C, Buth A, Chugani DC, et al. Successful surgical treatment of an inflammatory lesion associated with new-onset refractory status epilepticus. Neurosurg Focus, 2013, 34(6): E5.
10. Vezzani A, Balosso S, Ravizza T. The role of cytokines in the pathophysiology of epilepsy. Brain Behav Immun, 2008, 22(6): 797-803.
11. Gall CR, Jumma O, Mohanraj R. Five cases of new onset refractory status epilepticus (NORSE) syndrome: outcomes with early immunotherapy. Seizure, 2013, 22(3): 217-220.
12. Khawaja AM, DeWolfe JL, Miller DW, et al. New-onset refractory status epilepticus (NORSE)−The potential role for immunotherapy. Epilepsy Behav, 2015, 47: 17-23.
13. Gaspard N, Foreman BP, Alvarez V, et al. New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology, 2015, 85(18): 1604-1613.
14. Iizuka T, Kanazawa N, Kaneko J, et al. Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy. Neurol Neuroimmunol Neuroinflamm, 2017, 4(6): e396.
15. Costello DJ, Kilbride RD, Cole AJ. Cryptogenic new onset refractory status epilepticus (NORSE) in adults-infectious or not. J Neurol Sci, 2009, 277(1-2): 26-31.
16. Meletti S, Giovannini G, d’Orsi G, et al. New-onset refractory status epilepticus with claustrum damage: definition of the clinical and neuroimaging features. Front Neurol, 2017, 8: 111.
17. De Keyser J. Status epilepticus with neuron-reactive serum antibodies: response to plasma exchange. Neurology, 2000, 55(9): 1421.
18. 王学峰, 王康, 肖波. 成人全面性惊厥性癫痫持续状态治疗中国专家共识. 国际神经病学神经外科学杂志, 2018, 45(01): 1-4.
19. 中华医学会神经病学分会脑电图与癫痫学组. 非惊厥性癫痫持续状态的治疗专家共识. 中华神经科杂志, 2013, 46(2): 133-137.
20. Trinka E, Cock H, Hesdorffer D, et al. A definition and classification of status epilepticus−Report of the ILAE Task Force on Classification of Status Epilepticus. Epilepsia, 2015, 56(10): 1515-1523.
21. Van Lierde I, Van Paesschen W, Dupont P, et al. De novo cryptogenic refractory multifocal febrile status epilepticus in the young adult: a review of six cases. Acta Neurol Belg, 2003, 103(2): 88-94.
22. Li J, Saldivar C, Maganti RK. Plasma exchange in cryptogenic new onset refractory status epilepticus. Seizure, 2013, 22(1): 70-73.
23. Gaspard N, Hirsch LJ, Sculier C, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia, 2018, 59(4): 745-752.

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