中国科学院心理研究所机构知识库

摘要： 目的:分析总结合并小脑共济失调的抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎患者的临床特点。方法:回顾分析北京协和医院2011—2019年诊治的抗NMDAR脑炎患者中合并小脑共济失调患者的临床表现、实验室检查结果、治疗与预后。结果:347例抗NMDAR脑炎患者中共有15例（4.3%）合并小脑共济失调，其中1例患者合并卵巢畸胎瘤，男性7例，女性8例，中位年龄28岁，从脑炎发病到出现小脑症状平均病程30.8 d，极期改良Rankin量表（mRS）评分平均3.73分，脑脊液白细胞中位数28×10 6/L。15例患者均接受了一线免疫治疗，1例患者接受了利妥昔单抗二线免疫治疗，9例患者接受了吗替麦考酚酯长程免疫治疗。随访时间7~66个月，末次mRS评分平均2.73分，预后良好者（mRS评分≤2分）6例（6/15） 。结论:抗NMDAR脑炎合并小脑共济失调者相对少见，此类患者在神经功能方面预后较差，应及时识别抗NMDAR脑炎患者的小脑共济失调症状，采用规范的免疫治疗方案和长程免疫治疗，以期改善预后。

Objective:To analyze and summarize the clinical characteristics of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis patients with cerebellar ataxia.Methods:The clinical manifestations, laboratory examinations, treatment and prognosis of anti-NMDAR encephalitis patients with cerebellar ataxia diagnosed and treated in Peking Union Medical College Hospital from 2011 to 2019 were retrospectively analyzed.Results:About 4.3% (15 cases) of a total of 347 anti-NMDAR encephalitis patients were complicated with cerebellar ataxia, of which one patient had ovarian teratoma. There were seven male cases and eight female cases, with a median age of 28 years. The average duration from the onset of encephalitis to the onset of cerebellar symptoms was 30.8 days.The average modified Rankin Scale (mRS) score was 3.73. In the acute phase, the median cerebrospinal fluid leukocyte count was 28×10 6/L. All patients received first-line immunotherapy, of which one case additionally received second-line immunotherapy with rituximab and nine patients received long-term immunotherapy with mycophenolate mofetil. The follow-up time ranged from seven to 66 months. The average mRS score of the last time was 2.73, and only six patients (6/15) had good prognosis (mRS score≤2). Conclusions:Patients with anti-NMDAR encephalitis and cerebellar ataxia are rare, and have relatively poor prognosis in terms of neurological function. Symptoms of cerebellar ataxia in anti-NMDAR encephalitis patients should be recognized in time, and standardized immunotherapy regimens and long-term immunotherapy should be adopted to improve the prognosis.