New-onset refractory status epilepticus(NORSE)is a clinical syndrome,not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue,toxic or metabolic cause. Febrile in-fection-related epilepsy syndrome(FIRES)is a subcategory of NORSE,applicable for all ages,occurring in a prior fe-brile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus( RSE)with fever or not. Proposed consensus definitions are also applicable for infantile hemiconvulsion - hemiplegia and epilepsy syn-drome,RSE,super-RSE,prolonged RSE and prolonged super-RSE. These consensus definitions will promote much more effective communication and multicenter research,and ultimately improve recognition and treatment of these condi-tions.%新发难治性癫痫持续状态( NORSE)是一个临床综合征,而不是一个具体疾病诊断,发生于非活动性癫痫或非前期神经系统相关性疾患,不伴有明确的急性或活动性结构、中毒性或代谢原因.发热感染相关联癫痫综合征(FIRES)是NORSE的一个亚类,适用于所有年龄段,始于前驱发热感染24 h~2周,然后出现伴或不伴首次发热起病的难治性癫痫持续状态( RSE).推荐共识定义也适用于婴儿偏身惊厥-偏瘫及癫痫综合征、RSE、超级RSE、持久性RSE及持久性超级RSE.这些共识定义将促进更多有效的交流和多中心研究,最终将有利于加深对这些病情的认识和治疗.
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