PDF(Pubmed)
Abstract：
The term \'neuromyelitis optica spectrum disorders\' (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and its formes frustes and to a number of closely related clinical syndromes without AQP4-IgG. NMOSD were originally considered subvariants of multiple sclerosis (MS) but are now widely recognized as disorders in their own right that are distinct from MS with regard to immunopathogenesis, clinical presentation, optimum treatment, and prognosis. In part 1 of this two-part article series, which ties in with our 2014 recommendations, the neuromyelitis optica study group (NEMOS) gives updated recommendations on the diagnosis and differential diagnosis of NMOSD. A key focus is on differentiating NMOSD from MS and from myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD), which shares significant similarity with NMOSD with regard to clinical and, partly, radiological presentation, but is a pathogenetically distinct disease. In part 2, we provide updated recommendations on the treatment of NMOSD, covering all newly approved drugs as well as established treatment options.
摘要：
术语“视神经脊髓炎谱系障碍”（NMOSD）用作总称，指的是水通道蛋白4免疫球蛋白G（AQP4-IgG）阳性的视神经脊髓炎（NMO）及其形式，以及许多与AQP4-IgG密切相关的临床综合征。NMOSD最初被认为是多发性硬化症（MS）的亚变体，但现在被广泛认为是与MS在免疫发病机制方面不同的疾病。临床表现,最佳处理,和预后。在这个由两部分组成的文章系列的第1部分中，这与我们2014年的建议有关，视神经脊髓炎研究组(NEMOS)对NMOSD的诊断和鉴别诊断提出了最新建议.重点是将NMOSD与MS和髓鞘少突胶质细胞糖蛋白抗体相关脑脊髓炎（MOG-EM；也称为MOG抗体相关疾病，MOGAD)，与NMOSD在临床和临床方面具有显著相似性，部分,放射学表现，而是一种发病机制不同的疾病.在第2部分中，我们提供了关于NMOSD治疗的最新建议，涵盖所有新批准的药物以及既定的治疗方案。
