NMOSD:以前的血清阳性患者现在患有血清阴性脑干发作 (P11-14.017)
Andrew Weber1, John Rose2, L DeWitt3
1University of Utah.
Neurology
|February 20, 2025
概括
一位先前患有aquaporin-4 IgG (AQP4-IgG) 阳性神经脊髓炎光学谱障碍的患者经历了复发,尽管他对AQP4-IgG是血清阴性. 这一案例凸显了NMOSD中血清阴性复发的可能性,并提出了关于抗体波动和细胞免疫力的问题.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 神经免疫学 神经免疫学
背景情况:
- 神经omyelitis optica光谱障碍 (NMOSD) 是一种自身免疫性疾病,其特征是对水-4 (AQP4-IgG) 的抗体.
- NMOSD发作可以表现为视觉神经炎,横向髓炎或区域后风综合征,症状可能独立或同时发生.
研究的目的:
- 描述一个病例,患者有AQP4-IgG血清阳性NMOSD病史,在AQP4-IgG血清阴性时经历了新的脑干发作.
- 探索NMOSD中血清阴性复发的含义.
主要方法:
- 使用了回顾性图表审查,实验室测试 (ELISA,CBA-IFA,CBA-FACS) 和大脑MRI.
- 对 AQP4-IgG 标位随时间推移和复发期间进行了纵向分析.
主要成果:
- 一名43岁的女性患有NMOSD,最初为AQP4-IgG阳性,在停止治疗七年后,在AQP4-IgG测试呈阴性后,经历了脑干发作 (区域后综合征).
- 核磁共振扫描显示了一种新的增强后脑干病变,并通过IV甲基prednisolone和血交换解决了症状.
- 追溯分析表明,研究队列中的11%的阳性血清患者变得血清阴性,通常具有较低的初始标位.
结论:
- 这种病例表明NMOSD的血清阴性复发,挑战了疾病活动中血清状况的传统理解.
- 这些发现促使进一步调查天然抗体波动,抗原传播以及细胞免疫在血清阴性NMOSD攻击中的作用.
- 该患者在2012年满足了血清阳性NMOSD和2021年满足了血清阴性NMOSD的标准.
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