视神经脊髓炎谱系疾病临床特点分析及发病机制探讨  

The analysis of clinical features and pathogenesis of neuromyelitis optica spectrum disorders

作　　者：张洋洋 黄丽琴 焦雯钰 张倩[1] 张兆辉[1] Zhang Yangyang;Huang Liqin;Jiao Wenyu(Department of Neurology,Renmin Hospital of Wuhan University,Wuhan Hubei 430060)

机构地区：[1]武汉大学人民医院神经内科,430060

出　　处：《卒中与神经疾病》2021年第3期316-320,337,共6页Stroke and Nervous Diseases

摘　　要：目的分析视神经脊髓炎谱系疾病(Neuromyelitis optica spectrum disorders,NMOSDs)的临床特点,并探讨相关发病机制及诊治方案。方法回顾性收集2018年6月-2019年12月入住本院神经内科并诊断为NMOSDs的患者56例,分析患者的一般人口学特征、临床表现、实验室及影像学检查表现、诊疗及预后等。结果共纳入56例患者,男女比例为1∶10.2,首次发病年龄20~77(48.36±14.52)岁,73.2%患者表现为急性-亚急性起病,62.5%患者以急性脊髓炎起病,21.4%患者以急性视神经炎起病,16.1%患者表现为最后区综合征。30例患者中22例脑脊液水通道蛋白-4(Aquaporin-4,AQP4)-IgG抗体阳性,2例髓鞘少突胶质细胞糖蛋白(Myelinoligodendrocyte glycoprotein,MOG)-IgG抗体阳性,颈髓多与延髓、胸髓合并受累,也可出现颅内病灶。结论NMOSDs临床表现复杂多样,好发于中青年女性,复发率及致残率较高,首发症状以急性脊髓炎多见,以最后区综合征起病多易误诊。多数患者在发作急性期经过大剂量激素冲击治疗后预后良好,硫唑嘌呤、他克莫司、利妥昔单抗等免疫抑制剂仍是预防复发的一线药物。Objective To analyze the clinical features of neuromyelitis optica spectrum disorders(NMOSDs),and to explore the pathogenesis,diagnosis and treatment plans.Methods In this retrospective study,56 patients with NMOSDs who were admitted to our hospital from June 2018 to December 2019 were collected.The general demographic characteristics,clinical manifestations,laboratory and imaging findings,diagnosis and treatment,and prognosis of the patients were analyzed.Results A total of 56 patients were enrolled,the male-to-female ratio was 1∶10.2,the age of the first onset was 20~77(48.36±14.52)years,and 73.2% of patients showed acute-subacute onset.62.5% of patients had acute myelitis,21.4% of patients had acute optic neuritis,and 16.1% of patients had last zone syndrome.Of the 30 patients,22 cases were positive for AQP4-IgG in cerebrospinal fluid,and 2 cases were positive for MOG-IgG.Cervical spinal cord lesions often occur together with the medulla oblongata and thoracic spinal cord lesions.Intracranial lesions are also found in some patients with NMOSDs.Conclusion The clinical manifestations of NMOSDs are complex and diverse,and tend to occur in young and middle-aged women.The recurrence rate and disability rate are high.Acute myelitis is the most common first symptom.The area postrema syndrome is often misdiagnosed.Most patients have a good prognosis after high-dose hormone shock therapy in the acute stage of the attack.Immunosuppressants such as azathioprine,tacrolimus,and rituximab are the first-line drugs to prevent the recurrence.

关 键 词：视神经脊髓炎谱系疾病 发病机制 临床特点 诊疗 

分 类 号：R744.52[医药卫生—神经病学与精神病学]