Ferrán Ceila, Pedemonte Virginia, Turcatti Emilio, González Gabriel
Centro Hospitalario Pereira Rossell, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay.
Centro Hospitalario Pereira Rossell, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay. E-mail:
Medicina (B Aires). 2019;79 Suppl 3:60-65.
Neuromyelitis optica (NMO) is an autoimmune, inflammatory and de myelinat ing disorder of the central nervous system with a predilection for the optic nerves and spinal cord. In 2004 the association of NMO with an antibody against the water channel aquaporin 4 (anti-AQP4) was published as a different pathology from multiple sclerosis (MS). Currently the term NMO spectrum disorders (NMOSD) is proposed, because the manifestations of the disease can be more extensive, affecting in addition to the optic nerve and spinal cord, the area postrema of the dorsal medulla, brainstem, diencephalon and typical brain areas (periependymal, corpus callosum, internal capsule and subcortical white matter). NMOSD is also applied to patients who meet the NMO criteria and are negative for AQP4-IgG. Within the latter group, the presence of another antibody, anti-MOG, has been detected in 20%, with a different physiopathological mechanism, but with a similar clinic and a better prognosis. The immunosuppressive treatment in the attack, as well as the long-term treatment in the cases that are indicated, is fundamental to avoid sequelaes and recurrences. The correct diagnosis of this entity is essential since it can be aggravated with the use of drugs useful in the treatment of MS. In this publication we will review the pathophysiology, clinical and diagnostic criteria of NMOSD, and discuss the different therapeutic options.
视神经脊髓炎（NMO）是一种自身免疫性、炎症性和脱髓鞘性中枢神经系统疾病，好发于视神经和脊髓。2004年，NMO与抗水通道蛋白4抗体（抗AQP4）的关联被公布，被认为是一种与多发性硬化症（MS）不同的病理类型。目前提出了“NMO谱系障碍（NMOSD）”这一术语，因为该疾病的表现可能更广泛，除了视神经和脊髓外，还会影响延髓背侧最后区、脑干、间脑和典型脑区（室管膜周围、胼胝体、内囊和皮质下白质）。NMOSD也适用于符合NMO标准但AQP4-IgG阴性的患者。在后者中，已检测到20%的患者存在另一种抗体抗MOG，其病理生理机制不同，但临床表现相似且预后较好。发作期的免疫抑制治疗以及有指征病例的长期治疗对于避免后遗症和复发至关重要。正确诊断该疾病至关重要，因为使用对MS有效的药物可能会使其病情加重。在本出版物中，我们将回顾NMOSD的病理生理学、临床和诊断标准，并讨论不同的治疗选择。
