Abstract:
Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing neuroinflammatory autoimmune astrocytopathy, with a predilection for the optic nerves and spinal cord. Most cases are characterised by aquaporin-4-antibody positivity and have a relapsing disease course, which is associated with accrual of disability. Although the prognosis in NMOSD has improved markedly over the past few years owing to advances in diagnosis and therapeutics, it remains a severe disease. In this article, we review the evolution of our understanding of NMOSD, its pathogenesis, clinical features, disease course, treatment options and associated symptoms. We also address the gaps in knowledge and areas for future research focus.
摘要:
视神经脊髓炎谱系障碍(NMOSD)是一种罕见的复发性神经炎性自身免疫性星形细胞病,对视神经和脊髓有偏爱。大多数病例的特征是水通道蛋白-4-抗体阳性,并且具有复发性病程,这与残疾的应计有关。尽管NMOSD的预后在过去几年中由于诊断和治疗的进步而明显改善,它仍然是一种严重的疾病。在这篇文章中,我们回顾了我们对NMOSD的理解的演变,其发病机制,临床特征,病程,治疗选择和相关症状。我们还解决了未来研究重点的知识和领域的差距。