Neuromyelitis optica spectrum disorders are neuroinflammatory demyelinating disorders that lead to permanent visual loss and motor dysfunction.To date,no effective treatment exists as the exact causative mechanism rem...Neuromyelitis optica spectrum disorders are neuroinflammatory demyelinating disorders that lead to permanent visual loss and motor dysfunction.To date,no effective treatment exists as the exact causative mechanism remains unknown.Therefore,experimental models of neuromyelitis optica spectrum disorders are essential for exploring its pathogenesis and in screening for therapeutic targets.Since most patients with neuromyelitis optica spectrum disorders are seropositive for IgG autoantibodies against aquaporin-4,which is highly expressed on the membrane of astrocyte endfeet,most current experimental models are based on aquaporin-4-IgG that initially targets astrocytes.These experimental models have successfully simulated many pathological features of neuromyelitis optica spectrum disorders,such as aquaporin-4 loss,astrocytopathy,granulocyte and macrophage infiltration,complement activation,demyelination,and neuronal loss;however,they do not fully capture the pathological process of human neuromyelitis optica spectrum disorders.In this review,we summarize the currently known pathogenic mechanisms and the development of associated experimental models in vitro,ex vivo,and in vivo for neuromyelitis optica spectrum disorders,suggest potential pathogenic mechanisms for further investigation,and provide guidance on experimental model choices.In addition,this review summarizes the latest information on pathologies and therapies for neuromyelitis optica spectrum disorders based on experimental models of aquaporin-4-IgG-seropositive neuromyelitis optica spectrum disorders,offering further therapeutic targets and a theoretical basis for clinical trials.展开更多
目的观察维持性泼尼松治疗视神经脊髓炎(neuromyelitis optica,NMO)的疗效及安全性。方法对17例复发型NMO患者进行随访,观察在维持性泼尼松治疗前后,患者的扩展残疾状态量表(expanded disability status scale,EDSS)评分、年复发率的变...目的观察维持性泼尼松治疗视神经脊髓炎(neuromyelitis optica,NMO)的疗效及安全性。方法对17例复发型NMO患者进行随访,观察在维持性泼尼松治疗前后,患者的扩展残疾状态量表(expanded disability status scale,EDSS)评分、年复发率的变化以及治疗期间出现的不良反应。结果经维持性泼尼松治疗后,患者的EDSS评分较治疗前改善[(3.09±0.85)vs.(4.06±0.80)],差异有统计学意义(P<0.05);年复发率较治疗前明显下降[(0.34±0.31)次/年vs.(1.51±0.57)次/年],差异有统计学意义(P<0.005);但不同剂量泼尼松对于年复发率降低水平的影响差异无统计学意义。随访期间无严重不良反应出现。结论维持性泼尼松治疗能有效降低NMO患者的年复发率,并改善其神经功能缺失情况,有较好的耐受性。展开更多
文摘Neuromyelitis optica spectrum disorders are neuroinflammatory demyelinating disorders that lead to permanent visual loss and motor dysfunction.To date,no effective treatment exists as the exact causative mechanism remains unknown.Therefore,experimental models of neuromyelitis optica spectrum disorders are essential for exploring its pathogenesis and in screening for therapeutic targets.Since most patients with neuromyelitis optica spectrum disorders are seropositive for IgG autoantibodies against aquaporin-4,which is highly expressed on the membrane of astrocyte endfeet,most current experimental models are based on aquaporin-4-IgG that initially targets astrocytes.These experimental models have successfully simulated many pathological features of neuromyelitis optica spectrum disorders,such as aquaporin-4 loss,astrocytopathy,granulocyte and macrophage infiltration,complement activation,demyelination,and neuronal loss;however,they do not fully capture the pathological process of human neuromyelitis optica spectrum disorders.In this review,we summarize the currently known pathogenic mechanisms and the development of associated experimental models in vitro,ex vivo,and in vivo for neuromyelitis optica spectrum disorders,suggest potential pathogenic mechanisms for further investigation,and provide guidance on experimental model choices.In addition,this review summarizes the latest information on pathologies and therapies for neuromyelitis optica spectrum disorders based on experimental models of aquaporin-4-IgG-seropositive neuromyelitis optica spectrum disorders,offering further therapeutic targets and a theoretical basis for clinical trials.
文摘目的观察维持性泼尼松治疗视神经脊髓炎(neuromyelitis optica,NMO)的疗效及安全性。方法对17例复发型NMO患者进行随访,观察在维持性泼尼松治疗前后,患者的扩展残疾状态量表(expanded disability status scale,EDSS)评分、年复发率的变化以及治疗期间出现的不良反应。结果经维持性泼尼松治疗后,患者的EDSS评分较治疗前改善[(3.09±0.85)vs.(4.06±0.80)],差异有统计学意义(P<0.05);年复发率较治疗前明显下降[(0.34±0.31)次/年vs.(1.51±0.57)次/年],差异有统计学意义(P<0.005);但不同剂量泼尼松对于年复发率降低水平的影响差异无统计学意义。随访期间无严重不良反应出现。结论维持性泼尼松治疗能有效降低NMO患者的年复发率,并改善其神经功能缺失情况,有较好的耐受性。
