Kvistad Silje Agnethe Stokke, Wergeland Stig, Torkildsen Øivind, Myhr Kjell-Morten, Vedeler Christian A
Tidsskr Nor Laegeforen. 2013 Oct 15;133(19):2057-61. doi: 10.4045/tidsskr.13.0608.
Neuromyelitis optica (NMO) is a rare autoimmune inflammatory disease of the central nervous system that is characterized mainly by recurrent optic neuritis and longitudinally extensive transverse myelitis. The aim of this article is to present current knowledge on the clinical features, diagnosis, pathogenesis and treatment of the condition.
The article is based on a discretionary selection of English-language original articles, meta-analyses and review articles found in PubMed, and on the authors' own experience with the patient group.
Neuromyelitis optica was previously assumed to be a variant of multiple sclerosis (MS), but the discovery of aquaporin-4 antibodies in patients with neuromyelitis optica has led to this view being revised. The cause of the condition is still unknown, but it has been shown that the antibodies bind selectively to a water channel expressed mainly on astrocytes at the blood-brain-barrier, which has an important role in the regulation of brain volume and ion homeostasis. Clinically, the condition presents as optic neuritis and/or transverse myelitis. A diagnosis is made on the basis of case history, clinical examination, MRI of the brain and spinal cord, analysis of cerebrospinal fluid, visual evoked potentials and a blood test with analysis of aquaporin-4 antibodies. Once a diagnosis has been made, rapid treatment is important. In the acute phase, intravenous methylprednisolone is recommended. There are several options for preventative treatment, but the primary recommendations are oral prednisolone and azathioprine or intravenous infusion of rituximab. Treatment is distinct from the treatment of MS and some of the immunomodulatory drugs commonly used in MS can lead to worsening of neuromyelitis optica.
The condition is an important differential diagnosis of MS, but differs from MS in terms of clinical features, prognosis and treatment. Patients have a high risk of sequelae following relapses, and therefore early diagnosis and treatment is important.
视神经脊髓炎（NMO）是一种罕见的中枢神经系统自身免疫性炎症性疾病，主要特征为复发性视神经炎和纵向广泛横贯性脊髓炎。本文旨在介绍有关该疾病临床特征、诊断、发病机制及治疗的当前知识。
本文基于从PubMed中酌情选取的英文原创文章、荟萃分析及综述文章，以及作者对患者群体的自身经验。
视神经脊髓炎以前被认为是多发性硬化（MS）的一种变体，但在视神经脊髓炎患者中发现水通道蛋白4抗体后，这一观点已被修正。该疾病的病因仍不清楚，但已表明这些抗体选择性地结合主要在血脑屏障处星形胶质细胞上表达的一种水通道，这在脑容量调节和离子稳态中起重要作用。临床上，该疾病表现为视神经炎和/或横贯性脊髓炎。根据病史、临床检查、脑和脊髓的MRI、脑脊液分析、视觉诱发电位以及检测水通道蛋白4抗体的血液检测来进行诊断。一旦确诊，快速治疗很重要。在急性期，推荐静脉注射甲基泼尼松龙。有几种预防治疗方案，但主要推荐是口服泼尼松龙和硫唑嘌呤或静脉输注利妥昔单抗。其治疗与MS的治疗不同，MS中常用的一些免疫调节药物可导致视神经脊髓炎病情恶化。
该疾病是MS的重要鉴别诊断，但在临床特征、预后和治疗方面与MS不同。患者复发后有很高的后遗症风险，因此早期诊断和治疗很重要。
