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在区域后期发病的NMO频谱障碍中复发的特征 - - 一个回顾性队列研究

Xianxing Zhang^1,2,3, Jin Wu⁴, Jingyu Lin^1,2

¹Department of Neurology, the First Affiliated Hospital of Fujian Medical University, Fuzhou, Fujian, 350005, China.

|May 21, 2024

概括

脑神经炎光学谱系障碍 (NMOSD) 患有区域后热发病的患者年轻,复发率更高. 早期识别区域前膜干扰对于管理NMOSD复发风险至关重要.

科学领域:

神经免疫学神经免疫学
神经学神经学
自免疫性疾病自免疫性疾病

背景情况:

神经omyelitis光谱障碍 (NMOSD) 是一种严重的自身免疫性炎症状况,其特点是复发和残疾的高风险.
在NMOSD中,前膜区域 (AP) 经常受到影响,可能会影响疾病的发病和临床表现.
了解AP开始的NMOSD的特定特征对于优化治疗策略至关重要,旨在实现无复发状态.

研究的目的:

调查NMOSD患者的临床和复发性特征,这些患者最初的发作是在区域前膜 (AP) 中.
为了比较AP开始的NMOSD (APO-NMOSD) 和非AP开始的NMOSD (NAPO-NMOSD) 患者之间的临床特征和复发模式.

主要方法:

对166名AQP4-IgG血清阳性NMOSD患者进行了回顾性研究,这些患者的诊断使用了2015年国际NMOSD诊断小组 (IPND) 标准.
根据最初神经病发作的位置,患者被分为APO-NMOSD和NAPO-NMOSD组.
临床特征,包括扩展残疾状态量表 (EDSS) 评分,病变分布和复发率,在两组之间进行了比较.

主要成果:

与NAPO-NMOSD患者相比,APO-NMOSD患者更年轻 (34.6岁和42.3岁),在初始呈现和最后的随访时EDSS得分较低.
在APO-NMOSD患者中,更高比例的患者在第一次发作时表现出多核病变 (33.3%对9.2%),并且每年复发率更高 (0.4对0.19).
APO-NMOSD与第一次复发的时间更短,自然过程中的复发率更高,并在第一次复发时增加脑干参与的风险有关.

结论:

具有AQP4-IgG血清阳性NMOSD患者的AP发作通常较年轻,并且面临明显更高的疾病复发风险.
这些发现强调了承认AP参与NMOSD的重要性,因为它标志着复发发作的风险增加.
临床医生应保持对NMOSD中AP损伤的高怀疑指数,以主动管理患者并减轻复发风险.

关键词:

年度复发率年度复发率.面积 Postrema 区域极端神经炎视觉谱系障碍视觉谱系障碍

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