10例新发难治性癫持续状态患者的临床分析  

New-onset Refractory Status Epilepticus : Clinical Features and Outcome of 10 Cases

作　　者：武晨[1] 李力[1] 欧阳汤鹏[1] 江文[1] 

机构地区：[1]第四军医大学西京医院神经内科,710032

出　　处：《中国临床神经科学》2014年第6期634-641,共8页Chinese Journal of Clinical Neurosciences

摘　　要：目的：总结并分析新发难治性癫持续状态（NORSE）的临床特点及预后。方法采用回顾性研究分析2011至2013年收治的10例NORSE患者的临床资料，并对其预后进行随访，平均随访时间1.5年。结果9例患者发热后出现难治性癫持续状态（RSE）。急性期癫发作以部分发作为首发表现8例，继发全面性发作6例。急性期头颅MRI未见异常4例，颅内囊肿2例，余4例提示颅内信号异常。所有患者均接受正规抗癫药物治疗（3~9种），其中使用大剂量苯巴比妥4例、咪达唑仑7例、激素5例、静脉注射免疫球蛋白5例、生酮饮食治疗3例。随访期内仅1例应用3种抗癫药物14个月后无明确临床发作，7例仍有临床发作；1例处于植物状态，1例死亡。格拉斯哥预后评分5分1例，4分6例，3分1例，2分1例，1分1例。结论 NORSE是一组病因不明易发展为RSE的疾病，多种治疗常无效，预后多不良并遗留认知行为障碍。Aim To analyze new-onset refractory status epilepticus （NORSE） in a heterogenous group of patients. Methods The clinical data of 10 NORSE patients between 2011 and 2013 at neurological intensive care of Xijing Hospital were studied retrospectively. The mean duration of follow-up was 1.5 years. Results All patients presented prolonged refractory status epilepticus and nine of them exhibited preceding febrile illness before seizure. In the acute phase, eight patients had partial seizures and six of them developed secondarily generalised seizures. The initial MRI was normal in two cases, while the abnormalities found in the remaining cases were two with cysts and six with signal hyperintensity. All patients received multiple AEDs （3-9 kinds, a median of 6）, four with high dose of phenobarbital and seven cases with midazolam intravenously. Additionally, other therapies including steroids, immunoglobulin and ketogenic diet were used in ifve, ifve and three respectively. Only one patient became seizure free after 14 months with three kinds of antiepileptic drugs. One patient died and one was in vegetative state. The other seven patients still had different types of seizure. In the setting of Glasgow prognosis grade, one patients reached ifve scores and six patients reached four scores. The other three achieved three, two and one score respectively. Conclusion NORSE was used to describe patients who were declined to develop refractory status epilepticus with no identiifable causative factors, and a variety of treatment strategies were often unsuccessful. The outcome was frequently unoptimistic, with mental retardation and behavioral disturbances in chronic phase.

关 键 词：发热 癫 持续状态 难治性癫 

分 类 号：R743.1[医药卫生—神经病学与精神病学]