不常见的儿童免疫媒介性:疾病过程,诊断和结果 - - 一系列三例病例
Aakash Mahesan1, Aradhana Rohil1, Prashant Jauhari1
1Centre of Excellence & Advanced Research for Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Annals of Indian Academy of Neurology
|October 8, 2024
概括
儿童免疫媒介性 (IME) 可能对标准治疗不耐药. 及时诊断和免疫抑制针对CASPR2,GAD65和PNMA2等抗体可以显著改善控制.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 儿科 儿科 儿科
背景情况:
- 免疫媒介性 (IME) 是药物耐药性的重要原因之一.
- 早期诊断和免疫抑制疗法对于管理IME至关重要.
研究的目的:
- 报告与特定自身抗体相关的三例儿科IME病例.
- 在这些情况下评估免疫抑制的有效性.
主要方法:
- 病例系列包括三个患有发烧,脑病变和耐火性的儿童.
- 诊断工作包括抗体测试 (CASPR2,GAD65,PNMA2),MRI,EEG和FDG-PET.
- 治疗包括抗发作药物 (ASM) 和免疫抑制.
主要成果:
- 所有患者的检测结果为CASPR2,GAD65或PNMA2抗体呈阳性.
- 大脑MRI和EEG的发现是不特定的.
- FDG-PET揭示了特征性的超能代谢模式,暗示着自身免疫性脑炎.
- 在GAD65抗体IME中实现了完全的发作自由;在CASPR2和PNMA2抗体IME中,发作减少了50%以上.
- 在所有患者中都注意到持续的行为问题.
结论:
- 针对CASPR2,GAD65和PNMA2的抗体与儿科IME有关.
- FDG-PET可以帮助诊断中的自身免疫性脑炎.
- 早期免疫抑制对于IME的控制至关重要,尽管完全的反应并不普遍.
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