From the UCSF Weill Institute for Neurosciences (A.V., C.E.M., C.M.S., M.S., B.A.C., E.W., S.S.Z.), University of California, San Francisco; University of Nevada Reno School of Medicine (S.E.); Renown Health (L.M.G.), Reno, NV; Department of Radiology and Biomedical Imaging (E.G.); and Program in Immunology (S.S.Z.), University of California, San Francisco.
Neurol Neuroimmunol Neuroinflamm. 2024 Jul;11(4):e200243. doi: 10.1212/NXI.0000000000200243. Epub 2024 Apr 17.
Acute disseminated encephalomyelitis (ADEM) is one characteristic manifestation of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). A previously healthy man presented with retro-orbital headache and urinary retention 14 days after Tdap vaccination. Brain and spine MRI suggested a CNS demyelinating process. Despite treatment with IV steroids, he deteriorated, manifesting hemiparesis and later impaired consciousness, requiring intubation. A repeat brain MRI demonstrated new bilateral supratentorial lesions associated with venous sinus thrombosis, hemorrhage, and midline shift. Anti-MOG antibody was present at a high titer. CSF IL-6 protein was >2,000 times above the upper limits of normal. He improved after plasma exchange, then began monthly treatment alone with anti-IL-6 receptor antibody, tocilizumab, and has remained stable. This case highlights how adult-onset MOGAD, like childhood ADEM, can rapidly become life-threatening. The markedly elevated CSF IL-6 observed here supports consideration for evaluating CSF cytokines more broadly in patients with acute MOGAD.
急性播散性脑脊髓炎（ADEM）是髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的一种特征性表现。一名既往健康的男子在接受 Tdap 疫苗接种 14 天后出现眼眶后头痛和尿潴留。脑和脊柱 MRI 提示存在中枢神经系统脱髓鞘过程。尽管接受了 IV 类固醇治疗，但他的病情恶化，出现偏瘫，随后意识障碍，需要插管。重复脑 MRI 显示新的双侧幕上病变，伴有静脉窦血栓形成、出血和中线移位。抗 MOG 抗体高滴度阳性。CSF IL-6 蛋白是正常上限的 2000 多倍。他在接受血浆置换后好转，随后开始每月单独接受抗 IL-6 受体抗体、托珠单抗治疗，病情一直稳定。本例强调了成人起病的 MOGAD 与儿童 ADEM 一样，可能迅速危及生命。此处观察到的 CSF IL-6 显著升高支持在急性 MOGAD 患者中更广泛地评估 CSF 细胞因子。
