新发难治性癫痫持续状态研究进展  

Research advances in new-onset refractory status epilepticus

作　　者：刁海燕(综述) 王燕宏(审校)[1] DIAO Haiyan;WANG Yanhong(Department of Neurology,Third Hospital of Shanxi Medical University,Shanxi Bethune Hospital,Shanxi Academy of Medical Sciences,Tongji Shanxi Hospital,Taiyuan 030032,China)

机构地区：[1]山西医科大学第三医院(山西白求恩医院,山西医学科学院,同济山西医院)神经内科,山西太原030032

出　　处：《中风与神经疾病杂志》2024年第9期859-864,共6页Journal of Apoplexy and Nervous Diseases

摘　　要：新发难治性癫痫持续状态(NORSE),包括其亚型发热性感染相关癫痫综合征(FIRES)是最严重的癫痫持续状态形式之一。NORSE的患者均需接受全面的辅助检查来揭示其可能的病因,但仍有约半数患者无法明确病因(即隐源性NORSE)。目前发现的病因包括自身免疫、传染性、遗传、毒性等,自身免疫性脑炎仍是其主要原因。NORSE的急性期临床表现常为难治性或超难治性的癫痫发作,许多患者转为慢性期会出现耐药性癫痫。NORSE死亡率高,而且常遗留神经认知障碍相关后遗症。目前暂无NORSE的诊疗指南,本文对目前NORSE的定义、病因、发病机制、临床表现、治疗及预后作一综述。New-onset refractory status epilepticus(NORSE),including the subtype febrile infection-related epi-lepsy syndrome(FIRES),is one of the most severe forms of status epilepticus.Comprehensive examinations can help re-veal the possible etiology of NORSE,but the causes remain unexplained in approximately a half of the patients—crypto-genic NORSE.Autoimmune encephalitis is the main cause among autoimmunity,infectivity,genetics,toxicity,and other known reasons.NORSE typically manifests as refractory or super-refractory seizures in the acute phase,and can become drug-resistant when patients enter the chronic phase.The outcome of NORSE is unfavorable,with a high mortality rate and a high risk of neurocognitive deficit-related sequelae.Guidelines for the diagnosis and treatment of NORSE are lack-ing.This article reviews the definition,etiology,pathogenesis,clinical manifestations,treatment,and prognosis of NORSE.

关 键 词：新发难治性癫痫持续状态 发热感染相关癫痫综合征 超难治性癫痫持续状态 

分 类 号：R742.1[医药卫生—神经病学与精神病学]