新发难治性癫痫持续状态研究进展

Research advances in new-onset refractory status epilepticus

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.