重症及难治性抗N-甲基-D-天冬氨酸受体脑炎的接续治疗及预后  

Clinical approach and prognosis of continuous neurological care after intensive care for the patients with severe and refractory anti-N-methyl-D-aspartate receptor encephalitis

作　　者：周世梅[1] 关鸿志[2] 赵忙所[1] 黄方杰[1] 魏妍 王静[1] 刘建平[1] 赵秀丽 任海涛[2] 范思远[2] 彭斌[2] 乔立艳[1] Zhou Shimei;Guan Hongzhi;Zhao Mangsuo;Huang Fangjie;Wei Yan;Wang Jing;Liu Jianping;Zhao Xiuli;Ren Haitao;Fan Siyuan;Peng Bin;Qiao Liyan(Department of Neurology,Yuquan Hospital,Tsinghua University,Beijing 100040,China;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]清华大学玉泉医院神经科,北京100040 [2]中国医学科学院北京协和医学院北京协和医院神经科,北京100730

出　　处：《中华医学杂志》2020年第25期1937-1941,共5页National Medical Journal of China

基　　金：国家重点研发计划(2016YFC0901500);首都临床特色应用研究与推广项目(Z161100000516094)。

摘　　要：目的分析总结重症及难治性抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎出重症监护病房(ICU)后接续治疗阶段的临床特点、治疗方案及预后,提出接续治疗流程建议。方法回顾性分析2015年12月至2019年10月在清华大学玉泉医院神经科普通病房收治的重症及难治性抗NMDAR脑炎,重点关注转出ICU至出院的接续治疗,并进行随访。结果重症及难治性抗NMDAR脑炎20例,男女比例为9∶11,转入普通病房时病程为4.4(2.0,6.0)个月。6例神志清楚,6例为昏迷状态,5例为恢复早期,3例为恢复后期。并发症包括重度营养不良、肺炎、尿路感染、褥疮、白细胞减少等。7/18患者脑脊液抗NMDAR抗体高滴度阳性(≥1∶100)。10例接受静脉用丙种球蛋白(IVIg)治疗,少数患者使用激素冲击(1例)、利妥昔单抗(2例)、鞘内注射甲氨蝶呤(1例)和环磷酰胺(1例)治疗,所有患者加用长程免疫制剂吗替麦考酚酯(1.0~3.0 g/d)。随访17.0(8.0,27.0)个月,16例(80%)患者预后良好(mRS≤2分),病死率10%。结论接续治疗阶段患者神经功能障碍突出、合并症多,治疗周期较长。全面评估神经功能与并发症情况,采取个体化的免疫治疗,联合支持治疗和对症治疗,多数患者远期预后较好。Objective To study the clinical features,continuous care and prognosis of the patients with severe and refractory anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis after intensive care unit(ICU).Methods Clinical data of patients with severe and refractory anti-NMDAR encephalitis,who were transferred from ICU to general ward of neurology between December 2015 and October 2019,were retrospectively reviewed and analyzed in the study.Results Twenty patients(11 females and 9 males)were enrolled in the study.The median course of disease when patients were transferred to general ward was 4.4(2.0,6.0)months.Six cases were alert,6 cases were in a coma,5 were in the early recovery phase and 3 were in the late recovery phase.Severe malnutrition,pneumonia,urinary tract infections,bedsores and leukocytopenia were common complications.Seven out of 18 patients were tested positive for cerebrospinal fluid anti-NMDAR antibodies with high titers(≥1∶100).During this continuous therapy stage,10 patients were treated with intravenous immunoglobulin(IVIg),1 with methylprednisolone,2 with rituximab,1 with intrathecal methotrexate and 1 received intravenous cyclophosphamide.All Patients were prescribed a long-term immunotherapy(mycophenolate mofetil 1.5-3.0 g/d).Sixteen patients(80%)had good prognosis(modified Rankin Scale(mRS)≤2),and the mortality was 10%,with follow-up time of 17.0(8.0,27.0)months.Conclusions Patients with anti-NMDAR encephalitis,who are transferred from ICU,have severely impaired neurologic function.These patients need long-term individualized immunotherapy and continuous neurological care.Good outcomes can be achieved in most patients.

关 键 词：受体 N-甲基-D-天冬氨酸 脑炎 重症监护 治疗学 

分 类 号：R742.9[医药卫生—神经病学与精神病学]