Weinshenker Brian G, Wingerchuk Dean M
Department of Neurology, Mayo Clinic, Rochester, MN.
Department of Neurology, Mayo Clinic, Scottsdale, AZ.
Mayo Clin Proc. 2017 Apr;92(4):663-679. doi: 10.1016/j.mayocp.2016.12.014.
The understanding of neuromyelitis optica spectrum disorder (NMOSD) has evolved substantially since its initial description over a century ago. The discovery in 2004 of a pathogenic autoantibody biomarker targeting aquaporin 4 IgG revolutionized diagnosis and therapeutic development. Although NMOSD resembles multiple sclerosis (MS), differences were identified and articulated in the late 1990s. New diagnostic criteria incorporating the biomarker as well as better understanding of the clinical and radiologic features of NMOSD now permit accurate diagnosis and differentiation from MS. Aquaporin 4 IgG-associated NMOSD is now regarded as an immune astrocytopathy with lytic and nonlytic effects on astrocytes. A second autoantibody, myelin oligodendrocyte glycoprotein IgG, which targets myelin rather than astrocytes, leads to an NMOSD syndrome with clinical and radiologic features that overlap but are distinct from those of aquaporin 4 IgG-associated NMOSD and MS. We review current understanding of the clinical aspects, pathophysiology, and treatment of NMOSD.
自一个多世纪前首次描述以来，对视神经脊髓炎谱系障碍（NMOSD）的认识已发生了重大演变。2004年发现针对水通道蛋白4 IgG的致病性自身抗体生物标志物，彻底改变了诊断和治疗方法的发展。尽管NMOSD与多发性硬化症（MS）相似，但在20世纪90年代末就已确定并阐明了两者的差异。纳入该生物标志物的新诊断标准以及对NMOSD临床和放射学特征的更好理解，现在能够实现准确诊断并与MS进行区分。水通道蛋白4 IgG相关的NMOSD现在被视为一种对星形胶质细胞具有溶解和非溶解作用的免疫性星形细胞病。第二种自身抗体，即靶向髓磷脂而非星形胶质细胞的髓鞘少突胶质细胞糖蛋白IgG，会导致一种NMOSD综合征，其临床和放射学特征与水通道蛋白4 IgG相关的NMOSD和MS重叠但又有所不同。我们综述了目前对NMOSD临床方面、病理生理学和治疗的认识。
