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 investigate the clinical characteristics of vestibular paroxysmia(VP):age distribution,triggers,accompanying symptoms,maximum frequency per day,nystagmus features during attacks,and neurovascular compression.Methods The clinical data of 70 patients with VP were collected to analyze their age distribution,triggers,accompanying symptoms,maximum frequency per day,nystagmus features during attacks,and neurovascular compres-sion by using descriptive statistical methods.Results Among the 70 patients with VP,30(42.86%)were elderly,23(32.86%)were young,and 17(24.29%)were middle-aged patients.The maximum frequency per day was 2-5 in 53 cases(75.71%),6-10 in 9 cases(12.86%),and>10 in 8 cases(11.43%).Thirty-six patients(51.43%)had triggers,including rapid walking,emotional excitement,driving,cycling,sexual intercourse,coughing or wheezing,speaking or chatting,bathing,defecation,drinking alcohol,and sound stimulation.Thirty-three patients(47.14%)had unilateral tin-nitus,bilateral tinnitus,and tinnitus with facial spasms;nystagmus during VP episodes met the characteristics of the head coordinate system,which was horizontal-torsional towards the affected side.Among 57 patients examined for the relation-ship between the vestibulocochlear nerve and vessels using magnetic resonance angiography,37 patients(64.91%)had contact between the vestibulocochlear nerve and the right anterior inferior cerebellar artery,15 patients(26.32%)had contact between the vestibulocochlear nerve and the left anterior inferior cerebellar artery,and 5 patients(8.77%)had contact between the vestibulocochlear nerve and the basilar artery.Conclusion VP is most prevalent in the elderly,then young and middle-aged people.Most patients with VP have a maximum frequency of 2-5 per day.VP can be triggered by anger,excitement,exercise,and various stimuli.VP attacks can be accompanied by tinnitus and facial spasms.Its nystag-mus is horizontal-torsional towards the affected side.The percentage of contact between the vestibulocochlear nerve and the right anterior inferior cerebellar artery is highest in patients with VP.

目的 明确小脑性共济失调患者视频眼震电图特点,为早期诊断小脑性共济失调和鉴别前庭中枢性和周围性病变提供眼动异常依据.方法 收集2019年10月至2020年5月首都医科大学宣武医院神经内科住院诊治小脑性共济失调患者20例,对其视频眼震图各个项目包括自发性眼震、视动性眼震、平稳跟踪试验、扫视试验、凝视试验、位置试验和双温试验进行分析,总结各个项目的表现特点.结果 自发性眼震试验:7例(35％)出现各种类型眼震和扫视侵扰;视动性眼震:3例(15％)出现双侧慢相角速度不对称,19例(95％)出现增益异常;平稳追踪试验:18例(90％)出现Ⅲ型和Ⅳ型曲线,10例(50％)出现增益异常;凝视试验:10例(50％)出现异常眼动;扫视试验:13例(65％)出现峰速度下降、12例(60％)出现准确度异常和1例(5％)出现潜伏期异常;静态位置试验:15例(75％)出现异常眼动;前庭双温试验:1例(5％)出现半规管功能亢进.结论 视动性眼震的增益异常、平稳追踪试验曲线波形异常提示前庭中枢异常,是早期诊断小脑性共济失调的依据之一.

目的 探讨抗SOX1抗体阳性副肿瘤性小脑变性的诊疗要素.方法 北京首都医科大学宣武医院神经内科报道1例抗SOX1抗体阳性副肿瘤性小脑变性患者,分析其诊治过程中的关键点和经验.结果 患者中年男性,急性起病,以小脑性共济失调为主要表现,肺部占位伴纵隔淋巴结肿大,PETCT代谢增高,血清副肿瘤抗体谱:抗SOX1抗体(+),左肺占位穿刺活检病理结果提示炎症性病变,发病第4个半月门诊随诊发现胃泌素释放肽前体(645.20pg·mL-1)、神经元特异性烯醇化酶(93.47 pg·mL-1)升高,超声引导下支气管镜下纵隔淋巴结组织活检,淋巴结活检病理证实为小细胞肺癌,确诊后给与阿替利珠单抗治疗过程中出现药物相关PD-L1相关脑炎,神经系统症状经类固醇联合免疫球蛋白治疗好转.结论 抗SOX1抗体阳性与小细胞肺癌关系密切,抗SOX1抗体阳性副肿瘤综合征可以以小脑变性为主要表现.肿瘤患者阿替利珠单抗等PD-L1抑制剂治疗过程中,需注意免疫相关不良反应,少数患者会出现PD-L1相关脑炎.