危及生命的MOG抗体相关的出血性ADEM与CSFIL-6升高
Akash Virupakshaiah1, Carson E Moseley1, Steven Elicegui1
1From 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.
Neurology(R) neuroimmunology & neuroinflammation
|April 17, 2024
概括
成年人可以在接种疫苗后发展出严重的髓寡干细胞糖蛋白抗体相关疾病 (MOGAD),呈现为急性扩散性脑炎 (ADEM). 这种情况显示IL-6水平升高,并表明托西利祖马布可能是有效的.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
背景情况:
- 急性扩散性脑膜炎 (ADEM) 是髓寡细胞蛋白抗体相关疾病 (MOGAD) 的一个关键表现.
- 成年开始的MOGAD可能是严重和危及生命的,模仿童年ADEM.
研究的目的:
- 在接种Tdap疫苗后报告严重成人发病的MOGAD病例.
- 突出提高脑脊液 (CSF) IL-6 的作用和IL-6 阻断的潜在有效性.
主要方法:
- 一个以前健康的成年男性在接种疫苗后出现神经症状的病例报告.
- 诊断工作包括脑和脊柱的MRI,抗MOG抗体测试,以及对细胞因子的CSF分析.
- 治疗包括输液类固醇,血交换和托西利祖马布 (抗IL-6受体抗体).
主要成果:
- 患者出现了严重的神经缺陷,包括意识受损和半,尽管最初的类固醇治疗.
- 核磁共振扫描显示了广泛的脱髓化,静脉鼻血栓,出血和中线移动.
- 检测到高位的抗转基因抗体和明显升高的CSFIL-6 (>2,000x上限).
- 在血交换和随后的托西利祖马布治疗后,观察到显著的临床改善.
结论:
- 成人发病的MOGAD可以呈现出侵略性,即使在接种疫苗后.
- 脊髓液IL-6的升高是严重MOGAD的显着发现,表明它在病变发生过程中发挥了作用.
- 用托西利祖马布向IL-6可能是严重MOGAD的可行的治疗选择.
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