抗体阴性自身免疫脑炎:一个单中心的回顾性分析
Hana Mojžišová1, David Krýsl2, Jitka Hanzalová2
1From the Departments of Neurology (H.M., D.K., M.E., P.M.) and Immunology (J.H.), Second Faculty of Medicine Charles University and Motol University Hospital, Prague, Czech Republic; Institute of Clinical Chemistry (J.D., K.-P.W., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck; and Neuroimmunology (F.L.), Department of Neurology, University Hospital Schleswig-Holstein Kiel, Germany. hana.mojzisova@gmail.com.
Neurology(R) neuroimmunology & neuroinflammation
|October 25, 2023
概括
对抗体阴性自身免疫脑炎 (AE) 的全面测试至关重要. 许多患者从免疫治疗中受益,有些人可能有潜在的恶性瘤需要诊断和治疗.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 神经炎症是一种神经炎症.
背景情况:
- 自身免疫性脑炎 (AE) 包含异质性炎症性中枢神经系统 (CNS) 疾病.
- 没有可检测的神经自身抗体的患者被归类为抗体阴性AE.
- 了解抗体阴性AE对于准确的诊断和治疗至关重要.
研究的目的:
- 描述抗体阴性AE患者的临床特征,触发因素,治疗方法和结果.
- 在疑似抗体阴性AE病例中评估综合抗体测试的诊断产量.
- 为了强调在这个患者群体中进行彻底调查的重要性.
主要方法:
- 在2011年至2020年期间,对成年患者进行了神经抗体测试的回顾性单心研究.
- 纳入标准:可能的抗体阴性AE,确定的抗体阴性ADEM或确定的自身免疫LE.
- 使用基于细胞和基于组织的综合测试,对血清/中枢神经液样本进行重新分析.
主要成果:
- 测试患者中有1.5% (33/2250) 被归类为可能的抗体阴性AE.
- 在10例可能/确定的AE/LE/ADEM病例中,有1例患有恶性瘤;没有人接受了替代诊断.
- 80%接受免疫疗法,60%接受特定治疗 (例如,rituximab,IVIg);50%改善,30%死亡.
结论:
- 抗体阴性AE诊断需要进行全面的抗体检测.
- 由于免疫治疗的潜在益处,诊断工作是有必要的.
- 识别抗体阴性AE可以揭示潜在的恶性瘤.
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