From the Division of Neuroradiology, Serviço de Diagnóstico por Imagem, Santa Casa de Misericórdia de São Paulo, Rua Dr. Cesário Motta Jr. 112, Vila Buarque, São Paulo-SP 01221-020, Brazil; Division of Neuroradiology, Grupo DASA, São Paulo, Brazil (B.G.D., A.J.d.R., R.H.N.); and Division of Neuroradiology, Fleury Medicina e Saúde, São Paulo, Brazil (B.G.D, A.C.M.M.J.).
Radiographics. 2018 Jan-Feb;38(1):169-193. doi: 10.1148/rg.2018170141.
Neuromyelitis optica (NMO) is an autoimmune demyelinating disorder for which the aquaporin-4 (AQP4) water channels are the major target antigens. Advances in the understanding of NMO have clarified several points of its pathogenesis, clinical manifestations, and imaging patterns. A major advance was the discovery of the AQP4 antibody, which is highly specific for this disorder. Descriptions of new clinical and radiologic features in seropositive patients have expanded the spectrum of NMO, and the term NMO spectrum disorder (NMOSD) has been adopted. NMOSD is now included in a widening list of differential diagnoses. Acknowledgment of NMOSD imaging patterns and their mimicry of disorders has been crucial in supporting early NMOSD diagnosis, especially for unusual clinical manifestations of this demyelinating disease. This pictorial review summarizes the wide imaging spectrum of NMOSD and its differential diagnosis, as well as its historical evolution, pathophysiology, and clinical manifestations. RSNA, 2018.
视神经脊髓炎(NMO)是一种自身免疫性脱髓鞘疾病,水通道蛋白 4(AQP4)是其主要的靶抗原。对 NMO 的认识的进步阐明了其发病机制、临床表现和影像学模式的几个要点。一个主要的进展是发现了 AQP4 抗体,该抗体对这种疾病具有高度特异性。在血清阳性患者中描述新的临床和影像学特征,扩大了 NMO 的范围,因此采用了 NMO 谱障碍(NMOSD)这一术语。NMOSD 现在已被列入越来越多的鉴别诊断中。承认 NMOSD 的影像学模式及其对其他疾病的模拟,对于支持 NMOSD 的早期诊断至关重要,尤其是对这种脱髓鞘疾病不常见的临床表现。本影像学综述总结了 NMOSD 的广泛影像学谱及其鉴别诊断,以及其历史演变、病理生理学和临床表现。放射学学会,2018 年。