摘要
目的总结抗二肽基肽酶样蛋白()抗体脑炎之临床特征。方法与结果岁男性抗DPPX抗体脑炎患者临床表现为肌阵挛、进行性记忆力减退、呼吸困难和腹泻,实验室检查血清DPPX抗体阳性,18F-FDG PET提示双侧颞叶代谢减低。为进一步了解疾病特征,文献检索获得7篇国外文献计36例病例,经分析显示抗DPPX抗体脑炎以男性、中年好发,慢性病程;肌阵挛、震颤、认知功能障碍、自主神经功能失调、小脑或脑干损害及体重下降等为其主要症状与体征,可合并B细胞淋巴瘤;脑脊液或血清DPPX抗体呈阳性;无特异性影像学表现;治疗原则以免疫调节治疗为主,预后良好。结论抗DPPX抗体脑炎临床罕见,症状复杂多样,早期诊断与鉴别诊断困难,早期予免疫调节治疗预后良好。
Objective To summarize the clinical features of anti-dipeptidyl-peptidase-like protein-6(DPPX)encephalitis.Methods and Results We presented a 36-year-old male patient with anti-DPPX encephilitis who developed with myoclonus,progressive memory loss,dyspnea and diarrhea.Antibody against DPPX was positive in his serum.18F-fluoro-2-deoxy-D-glucose(18F-FDG)PET imaging indicated the hypometabolism in the bilateral temporal lobes.Then we reviewed the literatures about anti-DPPX encephalitis.A total of 7 articles with 36 cases were enrolled.We found that anti-DPPX encephalitis was common in male middle-aged patients with a chronic disease course.The clinical features of anti-DPPX encephalitis were myoclonus,tremor,cognitive dysfunction,automatic dysfunction,injury of brain stem or cerebellum and body weight loss.A small group of patients had underlying B cell neoplasms.DPPX antibody could be detected in the cerebrospinal fluid and serum of anti-DPPX encephalitis patients.The neuro-imaging often showed non-specific changes.The immunotherapy was effective in treating anti-DPPX encephalitis.It had a good prognosis.Conclusions Anti-DPPX encephalitis is rare,and it has complicated clinical manifestations.There are difficulties in the early and differential diagnosis.But it is usually responsive to the immunotherapy.
作者
周勤明
蔡勇
倪优
孟环宇
石红琴
陈生弟
陈晟
ZHOU Qin-ming;CAI Yong;NI You;MENG Huan-yu;SHI Hong-qin;CHEN Sheng-di;CHEN Sheng(Department of Neurology,Ruijin Hospital,Shanghai Jiaotong University School of Medicine,Shanghai 200025,China;Department of Neurology,Binhai People's Hospital,Yancheng 224500,Jiangsu,China)
出处
《中国现代神经疾病杂志》
CAS
北大核心
2020年第10期862-867,共6页
Chinese Journal of Contemporary Neurology and Neurosurgery
基金
国家自然科学基金资助项目(项目编号:81671241)
关键词
脑炎
自身免疫疾病
钾通道
电压门控
血清学
脑脊髓液
正电子发射断层显像术
Encephalitis
Autoimmune diseases
Potassium channels,voltage-gated
Serology
Cerebrospinal fluid
Positron-emission tomography
作者简介
周勤明,对本文有同等贡献;蔡勇,对本文有同等贡献;通讯作者:陈晟,Email:mztcs@163.com;通讯作者:陈生弟,Email:ruijincsd@126.com
