状态综合征变得容易:儿科观点
Kam Lun Ellis Hon1,2, Alexander K C Leung3, Karen K Y Leung2
1Department of Paediatrics, CUHK Medical Centre, The Chinese University of Hong Kong, Shatin, Hong Kong.
新发性耐火状态 (NORSE) 和发烧性感染相关综合征 (FIRES) 是具有挑战性的疾病. 我们为这些难治性发作提出了一个新的统一术语,NOSES (新发病状态综合征).
科学领域:
- 神经学 神经学
- 儿科神经学 儿科神经学
- 发病学 (Epileptology) 是一个专业的学科.
背景情况:
- 耐火状态综合征包括诸如新发性耐火状态 (NORSE) 和发烧性感染相关综合征 (FIRES) 等疾病.
- 这些综合征的现有命名通常是相互矛盾和令人困惑的.
- NORSE影响以前健康的儿童,而FIRES被认为是先前发烧事件的变体;这两种情况通常都暗示自身免疫或炎症起源.
研究的目的:
- 提供对NORSE/FIRES的假设,病因学,病理生理学,临床特征,诊断,治疗和未来前景的最新审查.
- 为了澄清这些复杂的神经疾病的理解和管理.
主要方法:
- 使用PubMed临床查询,使用NORSE和FIRES的关键词进行了全面的文献搜索.
- 搜索重点关注到2025年5月的人类受试者,包括审查,系统审查,病例系列和病例报告.
主要成果:
- 在NORSE/FIRES中发作通常是反抗性的,需要治疗,例如抗发作药物 (ASM),性饮食和免疫治疗.
- 预后往往很差,幸存者经常经历耐药性和认知障碍.
- 新的指导方针和共识改善了临床管理,一旦确定病因 (自身免疫,副瘤) 后,就会作出具体的诊断.
结论:
- 护士和火焰有相似之处,尽管迷走神经刺激在护士中可能更有效.
- 一个新的统一术语,NOSES (新发病状态综合征),是针对新发病状态持续超过24小时的特征,尽管有两个标准的ASM.
- 自身免疫性脑炎,神经瘤性疾病和传染性脑炎在确定病因时,代表了NOSES内的特定诊断.
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