抗N-甲基-D-天冬氨酸受体脑炎患者运动障碍的临床特点分析

Clinical characteristics of movement disorders in patients with anti-N-methyl-D-aspartate acid receptor encephalitis

目的探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者运动障碍的临床特点、治疗效果和预后。方法对2012年6月至2019年10月首都医科大学宣武医院神经内科收住院治疗的前瞻性录入的163例抗NMDAR脑炎患者资料进行分析,根据是否出现运动障碍将患者分为运动障碍组(75例,46.0%)与非运动障碍组(88例,54.0%),于患者免疫治疗后6个月和12个月进行随访,比较两组患者临床表现、辅助检查、治疗和预后等指标有无差异。结果163例抗NMDAR脑炎患者中,男性91例(55.8%),女性72例(44.2%),年龄26(19,34)岁。运动障碍组75例中,口面部肌张力障碍50例(66.7%)、肢体刻板动作45例(60%)、舞蹈样动作28例(37.3%)、偏身投掷动作9例(12.0%)、运动迟缓7例(9.3%)、震颤5例(6.7%)、肌张力障碍持续状态13例(17.3%)。与非运动障碍组患者相比,运动障碍组患者合并卵巢畸胎瘤(分别为14.7%、3.4%)、免疫治疗前改良Rankin量表评分3~5分(分别为76.0%、33.0%)、脑电图异常(分别为89.3%、77.3%)、腰椎穿刺压力增高(分别为53.3%、34.1%)、脑脊液白细胞增高(分别为73.3%、51.1%)、脑脊液NMDAR抗体强阳性(分别为44.0%、25.0%)、收住重症监护病房救治(分别为60.0%、9.1%)、接受静脉滴注丙种球蛋白(分别为80.0%、40.9%)、血浆置换(分别为36.0%、3.4%)、免疫抑制剂治疗(分别为37.2%、17.0%)的比例更高,发病至开始免疫治疗天数更短[分别为20(10,33)、35(15,77)d],免疫治疗至开始好转天数更长(分别为34(20,60)、20(15,35)d),并且上述指标两组间差异有统计学意义(均P<0.05)。免疫治疗后6个月和12个月预后与复发情况两组间差异无统计学意义。结论近半数抗NMDAR脑炎患者出现表现形式多样的运动障碍,运动障碍严重程度与疾病的严重程度有一定关系,经过积极的免疫治疗和对症治疗,大多数患者运动障碍随着原发疾病治疗好转而好转。

Objective To explore the clinical characteristics,therapeutic effect and prognosis of movement disorders in anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis.Methods The prospectively collected data of hospitalized 163 patients with anti-NMDAR encephalitis admitted to Xuanwu Hospital,Capital Medical University from June 2012 to October 2019 were analyzed.According to the presence of movement disorders,the patients were divided into movement disorders group(75 cases,46.0%)and non-movement disorders group(88 cases,54.0%).Patients were followed up for six months and 12 months after immunotherapy.The clinical manifestations,auxiliary examinations,treatment and prognosis of the two groups were compared.Results Among 163 patients with anti-NMDAR encephalitis,91 patients(55.8%)were male and 72 patients(44.2%)were female,with an age of 26(19,34)years.In the 75 patients of the movement disorders group,50 patients(66.7%)presented with orofacial dyskinesia,45 patients(60%)with limb stereotypies,28 patients(37.3%)with choreoathetosis,nine patients(12.0%)with ballism,seven patients(9.3%)with bradykinesia,five patients(6.7%)with tremor,and 13 patients(17.3%)with status dystonicus.Compared with the non-movement disorders group,the movement disorders group had a higher proportion of ovarian teratoma(14.7%vs 3.4%),modified Rankin Scale score of 3-5 before immunotherapy(76.0%vs 33.0%),abnormal electroencephalogram(89.3%vs 77.3%),increased lumbar puncture pressure(53.3%vs 34.1%),cerebrospinal fluid(CSF)pleocytosis(73.3%vs 51.1%),strong positive NMDAR antibody of CSF(44.0%vs 25.0%),admitting to intensive care unit(60.0%vs 9.1%),treated with intravenous immunoglobulin(80.0%vs 40.9%),plasma exchange(36.0%vs 3.4%),and immunosuppressive therapy(37.2%vs 17.0%);had shorter days from the onset to the beginning of immunotherapy[20(10,33)d vs 35(15,77)d];had longer days from the beginning of immunotherapy to the improvement[34(20,60)d vs 20(15,35)d];and there were significant differences of above items between the two groups(P<0.05).There was no significant difference in the prognosis and relaps between the two groups at six and 12 months after immunotherapy.Conclusions Nearly a half of patients with anti-NMDAR encephalitis had movement disorders with multiple phenotypes.The severity of movement disorders was related to the severity of the disease.After active immunotherapy and symptomatic treatment,movement disorders improved with the improvement of primary disease in majority of patients.