Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis.

Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis.
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DOI:
10.3389/fneur.2018.00888
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发表时间:
2018
影响因子:
3.4
通讯作者:
Paul F
Paul F
中科院分区:
医学3区
文献类型:
--
作者:
Borisow N;Mori M;Kuwabara S;Scheel M;Paul F

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视神经肌萎缩症谱系障碍(NMOSD)是自身抗体介导的慢性炎症性疾病。针对水通道蛋白-4水通道的血清抗体(Ab)导致视神经炎、肌萎缩和/或脑干综合征的复发性发作。在一些具有NMOSD症状的患者中,没有检测到AQP 4-Ab,但检测到针对髓鞘少突胶质细胞糖蛋白(MOG)的Ab。这些临床综合征现在经常被称为“MOG-脑脊髓炎”(MOG-EM)。在这里,我们概述了目前关于NMOSD和MOG-EM诊断的建议。这些包括抗体和进一步的实验室测试,MR成像和光学相干断层扫描。我们讨论了急性发作的治疗选择以及长期免疫抑制治疗,包括硫唑嘌呤,利妥昔单抗和免疫球蛋白。
Neuromyelitis optica spectrum disorders (NMOSD) are autoantibody mediated chronic inflammatory diseases. Serum antibodies (Abs) against the aquaporin-4 water channel lead to recurrent attacks of optic neuritis, myelitis and/or brainstem syndromes. In some patients with symptoms of NMOSD, no AQP4-Abs but Abs against myelin-oligodendrocyte-glycoprotein (MOG) are detectable. These clinical syndromes are now frequently referred to as “MOG-encephalomyelitis” (MOG-EM). Here we give an overview on current recommendations concerning diagnosis of NMOSD and MOG-EM. These include antibody and further laboratory testing, MR imaging and optical coherence tomography. We discuss therapeutic options of acute attacks as well as longterm immunosuppressive treatment, including azathioprine, rituximab, and immunoglobulins.
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