Neuromyelitis optica
Neuromyelitis optica
复制标题
DOI：
10.1055/s-0028-1090039
复制
发表时间：
2008-11
影响因子：
2
通讯作者：
D. Wingerchuk;B. Weinshenker
中科院分区：
文献类型：
--
作者：
D. Wingerchuk;B. Weinshenker
视神经肌炎（NMO，Devic综合征）的特征是视神经炎和横肌炎的并发症，通常与脊髓中延伸到三个或更多个椎体节段的病变有关。它是一种炎性、脱髓鞘性中枢神经系统疾病，虽然它最常复发，但它与多发性硬化症的不同之处在于它更严重，倾向于保留大脑，并与脊髓MRI上的纵向广泛病变有关。此外，NMO与高度特异性的血清自身抗体标记物NMO-IgG相关，其靶向水通道水通道蛋白-4。这种疾病在90%以上的患者中有复发过程。与多发性硬化症相比，由于继发性进展过程不常见，因此复发几乎是所有与疾病相关的残疾的原因。我们建议静脉注射皮质类固醇治疗急性肌病和视神经炎复发，随后迅速进行抢救性血浆置换治疗严重的、进行性的、类固醇难治性事件。大量证据支持NMO发病机制中的体液自身免疫机制，并且大多数现有数据表明需要全身免疫抑制来预防攻击。我们建议长期口服药物，如硫唑嘌呤或霉酚酸酯的患者相对较轻的疾病和利妥昔单抗治疗那些更严重的，最近的攻击或治疗难治性疾病。我们还建议对首次出现纵向广泛横贯性脊髓炎发作的NMO-IgG血清阳性患者进行至少5年的免疫抑制，因为他们复发或转化为NMO的风险很高。
Opinion statementNeuromyelitis optica (NMO, Devic’s syndrome) is characterized by concurrence of optic neuritis and transverse myelitis, typically associated with a lesion in the spinal cord extending over three or more vertebral segments. It is an inflammatory, demyelinating central nervous system disorder, and although it is most commonly relapsing, it is distinct from multiple sclerosis in that it is more severe, tends to spare the brain, and is associated with a longitudinally extensive lesion on spinal cord MRI. Furthermore, NMO is associated with a highly specific serum autoantibody marker, NMO-IgG, which targets the water channel aquaporin-4. The disease follows a relapsing course in more than 90% of patients. The relapses account for almost all disability associated with the disease because a secondary progressive course is uncommon, in contrast to multiple sclerosis. We recommend intravenous corticosteroids for acute myelitis and optic neuritis relapses, followed quickly by rescue plasmapheresis for severe, progressive, steroid-refractory events. Overwhelming evidence supports humoral autoimmune mechanisms in the pathogenesis of NMO, and most available data suggest that systemic immunosuppression is required to prevent attacks. We recommend long-term treatment with oral agents such as azathioprine or mycophenolate mofetil for patients with relatively mild disease and rituximab for those with more severe, recent attacks or treatment-refractory disease. We also recommend immunosuppression for at least 5 years in NMO-IgG seropositive patients presenting with a first-ever attack of longitudinally extensive transverse myelitis because they are at high risk for relapse or conversion to NMO.