抗体介导的鼻病和鼻病
Valérie Quinot1, Kevin Rostasy2, Romana Höftberger1
1Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, Austria.
Journal of clinical medicine
|October 16, 2024
概括
针对兰维尔节点的致病性抗体定义了新的自身免疫神经病变. 由于认识较低和诊断有限,尽管有明显的临床特征,儿童病例的诊断不足.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 新发现的致病性抗体向Ranvier节点的细胞粘附分子.
- 这导致了针对特定抗体介导的外围神经病变的新分类的发展,称为自身免疫性nodo-和paranodopathies.
研究的目的:
- 审查最近关于节点和偏节点神经病的发现.
- 为了突出这些疾病在儿科患者的特征,一个未经探索的领域.
主要方法:
- 诊断包括转染细胞的测试和ELISA以确定特定的抗原和抗体子类.
- 对成人和儿科病例的现有文献的审查.
主要成果:
- 这些神经病变涉及诸如神经素155/186,接触素-1和CASPR1之类的表位,具有其他标的潜力.
- 儿科病例很少见,但通常存在非典型的慢性炎症性脱髓化多神经病变 (CIDP) 现型.
- 临床过程通常是快速的,对标准疗法反应不佳,病理发现表明轴突损伤而不是脱髓化.
结论:
- 自身免疫性nodo-和paranodopathies代表了外围神经病变的一个独特的子集.
- 提高意识和改善诊断对于识别儿科病例至关重要.
- 了解病理生理学是开发向治疗的关键.
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