摘要
目的分析原发性自身免疫性小脑性共济失调(PACA)的临床特征,为对该类疾病的诊断及治疗提供经验。方法收集2018年1月至2023年1月中南大学湘雅三医院收治的PACA患者,回顾性分析患者的临床表现、实验室检查、影像学资料等。结果共纳入6例患者,其中男3例,女3例;中位年龄54岁。6例患者急性或亚急性起病,以步态不稳为主要症状,无前驱感染史;脑脊液常规、生化、细胞学和颅脑磁共振成像(MRI)检查基本正常;脑脊液自身免疫性小脑性共济失调抗体和血副肿瘤综合征抗体均为阴性;4例结缔组织病相关指标异常。6例患者中,4例患者接受免疫球蛋白治疗,其中2例合并激素治疗,1例合并激素及环磷酰胺治疗。6例患者经治疗后,步态不稳均有不同程度缓解,接受免疫或激素治疗患者的症状缓解明显。结论PACA是一种免疫介导的,但未发现明确病因或特定神经元抗体的自身免疫性疾病;临床表现以共济失调为主;脑脊液及影像学检查大致正常;免疫治疗对多数患者是有效的。
Objective To investigate the clinical features of primary autoimmune cerebellar ataxia(PACA),and to provide experience for the diagnosis and treatment of this disease.Methods Related data were collected from the patients with PACA who were admitted to The Third Xiangya Hospital of Central South University from January 2018 to January 2023,and a retrospective analysis was performed for their clinical manifestations,laboratory examination,and radiological examination.Results A total of six patients were enrolled,among whom there were three male patients and three female patients,with a median age of 54 years.All six patients had acute or subacute onset with ataxia as the main symptom,and there was no history of prodromal infection.Basically normal results were obtained from routine cerebrospinal fluid test,biochemical examination,cytology,and cranial magnetic resonance imaging(MRI).All patients tested negative for autoimmune cerebellar ataxia antibody in cerebrospinal fluid and paraneoplastic syndrome antibody in blood.Four patients were found to have abnormal indicators associated with connective tissue disease.Of all six patients,four received immunoglobulin therapy,among whom 2 received hormone therapy and 1 received hormone and cyclophosphamide.After treatment,all six patients had varying degrees of improvement in ataxia,and the patients receiving immunotherapy or hormone therapy had significant relief of symptoms.Conclusions PACA is an immunemediated disease with no clear etiology or specific neuronal antibody.Ataxia is the main clinical manifestation,and cerebrospinal fluid test and radiological examination often have basically normal results.Immunotherapy is effective in most patients.[Journal of International Neurology and Neurosurgery,2023,50(1):25-28]
作者
王晨
袁名扬
侯德仁
WANG Chen;YUAN Mingyang;HOU Deren(The Third Xiangya Hospital of Central South University,Changsha,Hunan 410000,China)
出处
《国际神经病学神经外科学杂志》
2023年第1期25-28,共4页
Journal of International Neurology and Neurosurgery
基金
湖南省科技厅社会发展项目(2020SK53613)
湖南省自然科学基金(2021JJ31006)
湖南省研究生科研创新项目(CX20220375)。
关键词
小脑性共济失调
自身免疫
免疫介导
cerebellar ataxia
autoimmunity
immunemediated cerebellar ataxias
作者简介
王晨(1997-),女,硕士研究生,主要从事阿尔茨海默病相关研究,Email:1344198105@qq.com;通信作者:侯德仁(1966-),男,主任医师,主要从事阿尔茨海默病相关研究,Email:hou0718@126.com。
