Sato Douglas Kazutoshi, Callegaro Dagoberto, Lana-Peixoto Marco Aurélio, Nakashima Ichiro, Fujihara Kazuo
Department of Neurology, School of Medicine, Tohoku University, Sendai, Japan.
Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
Arq Neuropsiquiatr. 2014 Jun;72(6):445-50. doi: 10.1590/0004-282x20140032.
Neuromyelitis optica spectrum disorders (NMOSD) are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4) are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.
视神经脊髓炎谱系障碍（NMOSD）的特征为严重视神经炎和/或纵向广泛横贯性脊髓炎，且一些脑部病变也是NMOSD所特有的。大多数NMOSD病例可检测到抗水通道蛋白4（AQP4）血清自身抗体。然而，一些NMOSD患者尽管在发作期间使用高灵敏度细胞检测法进行了多次检测，仍为血清阴性。NMOSD的鉴别诊断不限于多发性硬化症，还包括许多可导致纵向广泛脊髓炎和/或视神经炎的疾病。我们回顾了有助于诊断检查的临床特征、影像学和实验室检查结果，讨论了NMOSD中AQP4抗体阳性和阴性患者之间的差异，包括伴有抗髓鞘少突胶质细胞糖蛋白抗体的NMOSD的特征。
