29例视神经脊髓炎谱系疾病临床分析

【目的】通过总结视神经脊髓炎谱系疾病（NMOSD）的临床特点，提高对该病的认识。【方法】回顾性分析29例NMOSD患者的临床表现、辅助检查和诊治情况。【结果】29例患者，女性26例，男性3例，发病年龄9～61（36±13．77）岁；1例合并甲亢，1例合并未分化结缔组织病；2例在怀孕时发病，1例发病时伴桥本甲状腺炎；21例患者行靶抗原水通道蛋白4（aquaporin 4，AQP4）抗体检测，19例呈阳性；19例有一项或多项免疫指标异常；脑MRI提示颅内侵犯13例，脊髓MRI提示脊髓受累25例；6例目前表现为单相病程，其余23例为缓解复发型。【结论】NMOSD是以体液免疫为主的中枢神经系统炎性脱髓鞘疾病，可合并多种自身免疫性疾病，存在多种自身抗体。疾病缓解期，建议应用免疫抑制剂，以减少复发。

Clinical Characteristics of Neuromyelitis Optica Spectrum Disorders:An Analysis of 2 9 Cases

Objective]To investigate the clinical features of neuromyelitis optica spectrum disorders (NMOSD)by summarizing the clinical features .[Methods]The clinical manifestations,assistant examina-tions,diagnosis and treatment of 29 patients with NMOSD were analyzed retrospectively.[Results]Among the 29 patients suffered from NMOSD ,3 were male and 5 were female,9~61 years old(36±13.77),1 case com-bined with hyperthyroidism,2 cases diseased in pregnancy,1 case with Hashimoto's thyroiditis;21 cases were tested with target antigens aquaporin (AQP4)antibody detection,1 9 cases with one or several immune indices showed abnormal;brain MRI showed intracranial invasion in 1 3 cases,spinal cord MRI showed spinal cord in-volvement in 25 cases;6 cases now appeared to be monophasic and the rest of the 23 cases belonged to eased relapsing.[Conclusion]NMOSD is characterized by inflammatory demyelinating disease of the central nervous system,which is dominated by humoral immunity.NMOSD coexist with many kinds of autoimmune diseases and multiple autoantibodies.Pregnancy may aggravate the severity of NMOSD.Immunosuppressive therapy is recommended to reduce recurrence.