抗体相关自身免疫性小脑性共济失调15例分析  

Analysis of 15 cases anti-neuronal antibodies related autoimmune cerebellar ataxia

作　　者：刘静[1] 樊春秋[1] 刘爱华[1] 杨冬菊[1] 陈芳[1] 户宁宁 Liu Jing;Fan Chunqiu;Liu Aihua;Yang Dongju;Chen Fang;Hu Ningning(Department of Neurology,the Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经内科,北京100053

出　　处：《脑与神经疾病杂志》2023年第6期336-342,共7页Journal of Brain and Nervous Diseases

摘　　要：目的总结抗体相关自身免疫性小脑性共济失调(ACA)15例的特点及潜在意义。方法收集2015年1月至2021年12月在首都医科大学宣武医院神经内科患者住院治疗、自身免疫抗体阳性且以小脑性共济失调为主要临床表现的患者,整理统计患者的临床资料。结果15例患者均以头晕、走路不稳伴或不伴构音障碍的神经系统症状为首发和主要表现,4例患者有眼震,有8例患者在之后的筛查中发现肿瘤。15例患者,男性3例,女性12例,年龄34~69岁,平均年龄(55.5±3.79)岁。抗Yo抗体阳性3例,抗Hu抗体阳性2例,抗Tr抗体阳性2例,抗Ri抗体阳性、抗Amphiphysin抗体阳性、抗GAD抗体阳性、抗SOX1抗体阳性、抗PNMA2抗体阳性各1例,有3例为多重抗神经元抗体阳性。15例抗体阳性中有9例脑脊液相应抗体阳性,15例血清抗神经元抗体全部阳性。15例脑脊液常规、生化均未见明显异常,7例脑脊液特异性寡克隆区带阳性(46.67%)。7例寡克隆区带阳性中有5例脑脊液抗体阳性(阳性率71.42%)。8例寡克隆区带阴性中只有4例脑脊液抗体阳性(阳性率50%)。5例头颅MRI有小脑萎缩,均为双侧萎缩(阳性率33.33%)。糖皮质激素或静脉注射免疫球蛋白治疗后MRS评分显示神经系统症状均有改善。结论自身ACA调相关抗体以细胞内抗体为主。自身ACA以头晕、走路不稳、构音障碍、中枢性眼震等前庭小脑系统症状为首发和主要表现,眼震电图等前庭功能检查对前庭小脑系统功能的评估有重要意义。血清中检测抗神经抗体被认为足以诊断自身免疫性小脑性共济失调。脑脊液化验呈非特异性炎性表现,脑脊液寡克隆区带阳性与脑脊液神经元抗体阳性率的关系有待进一步扩大样本量研究确定。治疗应尽早开始,糖皮质激素或静脉注射免疫球蛋白治疗后MRS评分显示神经系统症状均有改善。Objective To summarize the characteristics and potential significance of 15 cases of antineurona associated autoimmune cerebellar ataxia.Method The clinical data of patients with positive autoimmune antibodies and the main manifestation of cerebellar ataxia who were hospitalized in the Neurology Department of Xuanwu Hospital,Capital Medical University from January 2015 to December 2021 were collected.Results All the 15 patients presented with neurological symptoms of dizziness and unsteady walking with or without articulation disorder as the first and main presentation,4 patients had nystagmus and the form of nystagmus was consistent with the characteristics of central nystagmus,and 8 patients were found to have tumors during subsequent screening.Among the 15 patients,there were 3 males and 12 females,aged 34-69 years,with an average age of(55.5±3.79)years.There were 3 positive cases of anti-Yo antibody,2 positive cases of anti-Hu antibody,2 positive cases of anti-Tr antibody,1 positive case of anti-Ri antibody,anti-Amphiphysin antibody,anti-GAD antibody,anti-SOX1 antibody,and anti-PNMA2 antibody,and 3 positive cases of multiple anti-neuron antibodies.Nine of the 15 patients with positive antibodies were positive in CSF and all of them were positive in serum antineuronal antibodies.There were no obvious abnormalities in routine and biochemical tests of cerebrospinal fluid in 15 patients,and 7 patients(46.67%)had positive cerebrospinal fluid specific oligoclonal zone.CSF antibody was positive in 5 of 7 patients(71.42%)with positive oligoclonal zone.CSF antibody was positive in 4 of 8 patients(50%)with negative oligoclonal zone.Cerebellar atrophy was found in 5 patients(positive rate 33.33%).MRS scores showed improvement in neurological symptoms after glucocorticoid or steroids intravenous immunoglobulin treatment.Conclusion Autoimmune cerebellar ataxia(ACA)related antibodies were mainly intracellular antibodies.ACA is the first and main manifestation of vestibular cerebellar system symptoms such as dizziness,unst

关 键 词：抗神经元抗体 自身免疫机制介导的小脑性共济失调 副肿瘤性小脑变性 眼球震颤 脑脊液寡克隆区带 免疫治疗 

分 类 号：R512.3[医药卫生—内科学]