在被认为是神经退行性痴呆症患者中,与自身免疫性脑炎相关的抗体
Anna E M Bastiaansen1, Robin W van Steenhoven1, Esmee S Te Vaarwerk1
1From the Department of Neurology (A.E.M.B., R.W.S., M.M.P.N., M.P., S.C.F., P.A.E.S., J.M.V., H.S., J.S., F.J.J., M.J.T.), Erasmus University Medical Center, Rotterdam; Department of Neurology (R.W.S.), Amsterdam UMC Location VUmc; Clinical Neuropsychology (E.S.V.), Leiden University; Alzheimer Center Amsterdam (W.M.F.), Neurology, Amsterdam UMC Location VUmc; Amsterdam Neuroscience (W.M.F., C.T., Y.A.L.P.), Neurodegeneration; Neurochemistry Laboratory (C.T.), Department of Chemistry, Amsterdam UMC Location VUmc; Department of Biology (E.G.), Faculty of Science, Utrecht University; Department of Immunology (M.W.J.S.), Erasmus University Medical Center, Rotterdam, The Netherlands; Institute of Clinical Chemistry (F.L.), University Hospital Schleswig-Holstein, Germany; Alzheimer Center Erasmus MC (H.S., J.S., F.J.J.), Erasmus MC University Medical Center, Rotterdam; and Alzheimer Center Amsterdam (Y.A.L.P.), Neurology, Vrije Universiteit Amsterdam, Amsterdam UMC Location VUmc, The Netherlands.
一小部分疑似神经退行性痴呆症患者有神经元抗体,表明自身免疫性脑炎 (AIE). 早期发现这些病例对于有效的免疫疗法治疗至关重要.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
背景情况:
- 自身免疫性脑炎 (AIE) 可以模仿神经退行性痴呆症的认知问题,但没有明确的MRI/CSF炎症.
- 识别AIE至关重要,因为它经常对免疫疗法做出反应.
研究的目的:
- 为了确定诊断为神经退行性痴呆症的患者的神经抗体的频率.
- 描述检测到神经元抗体的患者的临床特征.
主要方法:
- 对920名神经退行性痴呆症患者进行了回顾性队列研究.
- 使用IHC,CBA和活细胞培养物测试了1398个样本 (血清/CSF).
- 需要至少两种技术的阳性结果来确认.
主要成果:
- 在0.8% (7/920) 的患者中发现了神经抗体 (抗IgLON5,抗LGI1,抗DPPX,抗NMDAR).
- 所有7名患者都表现出非典型的症状,如亚急性恶化,肌细胞结核或自身免疫病史.
- 非典型的临床症状在抗体阳性患者中更频繁 (100%对21%).
结论:
- 在疑似神经退行性痴呆病例中,很少但很大一部分患有神经元抗体,这表明AIE.
- 考虑在非典型痴呆症患者中进行神经元抗体测试,以了解潜在的免疫疗法益处.
- 谨慎的临床相关性和确认是必要的,以避免误诊和不适当的治疗.
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