视神经脊髓炎谱系疾病

Neuromyelitis Optica Spectrum Disorder

视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)是一种不常见抗体介导的中枢神经系统疾病。未经治疗的NMOSD患者中,大约有50%会致残和致盲,1/3会在首次发病5年内死亡,不同于多发性硬化,NMOSD是极不常见的一种临床进展病程,残疾常由疾病复发所致,因此,预防复发是至关重要的,目前一般采用长期免疫抑制治疗来实现,早期诊断和治疗对于降低长期残疾的风险和病死率极为重要。该文通过发病机制、治疗方法、免疫学等几个方面对NMOSD进行综述。

Neuromyelitis optica spectrum disorder(NMOSD)is an unusual antibody-mediated central nervous system disease.About 50%of patients with untreated NMOSD will become disabled and blind,and 1/3 will die within 5 years of the first onset.Unlike multiple sclerosis,NMOSD is an extremely rare clinically advanced course,disability is often caused by disease recurrence.Therefore,prevention of recurrence is very important.At present,long-term immunosuppressive therapy is generally adopted.Early diagnosis and treatment are extremely important to reduce the risk of long-term disability and case fatality rate.This article reviews NMOSD through several aspects such as pathogenesis,treatment methods and immunology.