抗N-甲基-D-天冬氨酸受体脑炎的临床特征及一线免疫治疗的短期预后分析  

Clinical features of anti-N-methyl-D-aspartate receptor encephalitis and short-term prognostic analysis with first-line immunotherapy

作　　者：姚涛[1] 群森 程昭昭 刘天龙 鲁亚楠 余锋 YAO Tao;QUN Sen;CHENG Zhao-zhao;LIU Tian-long;LU Ya-nan;YU Feng(Department of Neurology,The First Affiliated Hospital of USTC,Division of Life Sciences and Medicine,University of Science and Technology of China,Hefei 230001,Anhui,China)

机构地区：[1]中国科学技术大学附属第一医院(安徽省立医院)神经内科,合肥230001

出　　处：《医学研究生学报》2022年第7期730-734,共5页Journal of Medical Postgraduates

基　　金：安徽省重点研究与开发计划项目(202004j07020014)。

摘　　要：目的抗N-甲基-D-天冬氨酸受体脑炎(NMDAR)患者疾病分期4个阶段。文中探讨NMDAR患者不同疾病阶段的临床特征及一线免疫治疗的短期预后。方法回顾性分析2017年3月—2021年6月期间于安徽省立医院确诊的22例抗NMDAR脑炎患者临床资料,并按临床表现分为早期阶段7例和进展阶段15例。观察患者不同阶段的临床指标(脑脊液白细胞数、蛋白浓度、氯化物浓度、糖浓度),脑电图,头颅磁共振,重症监护和一线免疫治疗6个月后随访的mRS评分,并观察一线免疫治疗6个月后的临床症状改善情况。结果①临床特征:所有患者均存在精神行为异常、言语障碍,其次以癫痫发作(63.6%)、运动障碍(54.5%)、意识障碍(50.0%)、记忆障碍(45.5%)和自主神经功能障碍(36.4%)较为常见。早期阶段以前驱症状(57.1%)、脑脊液白细胞数升高(85.7%)为最主要特征,早期阶段前驱症状与白细胞数升高情况明显多于进展阶段(P<0.05);而进展阶段则以mRS>2分(60.0%)、脑电图中-重度异常(40.0%)、头颅磁共振异常(33.3%)、重症监护(33.3%)为主要特征,进展阶段mRS>2分患者明显多于早期阶段(P<0.05)。其中,脑电图中-重异常和磁共振异常者均处于进展阶段。②短期预后:22例抗NMDAR脑炎患者一线免疫治疗6个月后随访,癫痫发作、不自主运动和意识障碍症状均消失,仅存在精神行为异常、记忆障碍各8例,言语障碍6例,除记忆障碍外,其他临床症状较急性发作期明显改善(P<0.05)。结论抗NMDAR脑炎不同疾病阶段具有不同的临床特征,早期阶段最主要症状包括前驱症状及脑脊液白细胞数升高,进展阶段脑电图中-重度异常、头颅磁共振异常较多,短期预后差;其疾病分期有助于早期诊断;早期阶段患者及时一线免疫治疗可抑制病情进展,改善短期预后。Objective To analyze the clinical characteristics of patients with anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis at different disease stages and the short-term prognosis of patients at different disease stages and with first-line immunotherapy.Methods The clinical data of 22 patients with anti-NMDAR encephalitis were collected and divided into 7 at the early stage and 15 at the progressive stage by clinical manifestations.Patients were observed in different stages of clinical outcome:Leukocyte count,protein concentration,chloride concentration and sugar concentration in cerebrospinal fluid,EEG,cranial magnetic resonance,mRS score at 6-month follow-up after intensive care and first-line immunotherapy,and for clinical improvement after 6 months of first-line immunotherapy.Results①Clinical features:psychobehavioral abnormalities and speech disturbances were present in all patients,followed by seizures(63.6%),motor disturbances(54.5%),impaired consciousness(50.0%),memory disturbances(45.5%)and autonomic dysfunction(36.4%)being more common.The early stage was dominated by prodromal symptoms(57.1%)andthe number of leukocytes in cerebrospinal fluid increased(85.7%).The increase of prodromal symptoms and leukocyte count in the early stage was significantly more than that in the progressive stage(P<0.05);However,the progressive stage was mainly characterized by mRS score>2(60.0%),Moderate to severe EEG abnormalities(40.0%),cranial magnetic resonance abnormality(33.3%),and intensive care admission(33.3%).Patients with advanced Mrs greater than 2 points were significantly more than early patients(P<0.05).Of these,those with moderate to severe EEG and abnormal cranial magnetic resonance,were in a progressive stage.②Short term outcome:at follow-up,6 months after first-line immunotherapy in 22 patients with anti-NMDAR encephalitis,symptoms including seizures,involuntary movements and impaired consciousness all disappeared,and only psychobehavioural abnormalities,memory disturbances were present in 8 patients each,a

关 键 词：抗N-甲基-D-天冬氨酸受体脑炎 免疫治疗 临床特征 短期预后 

分 类 号：R742.9[医药卫生—神经病学与精神病学]