葡萄牙的一组IgG4相关疾病的神经参与
João Moura1, Maria João Malaquias1, Firmina Jorge2
1*Shared first co-authorship. Neurology Department. Centro Hospitalar Universitário de Santo António. Porto. Portugal.
Acta medica portuguesa
|April 26, 2024
概括
神经性IgG4-RD呈现出各种症状,如脑膜炎和神经病变. 诊断是具有挑战性的,强调需要更新的标准,因为各种表现和活检的困难.
科学领域:
- 神经免疫学 神经免疫学
- 类风湿病学 类风湿病学
背景情况:
- 人们越来越认识到IgG4相关疾病 (IgG4-RD) 的神经参与.
- 由于临床模仿和活检困难,诊断具有挑战性.
研究的目的:
- 描述一组神经IgG4-RD患者的临床,实验室,神经成像和组织学特征.
主要方法:
- 对15名神经免疫学三级中心患者的临床数据的回顾性审查.
- 实验室,神经成像和组织学发现的分析.
主要成果:
- 最常见的神经现象包括脑膜炎,轨道伪瘤,神经病变,外围神经病变和纵向广泛横向髓炎 (LTEM).
- 观察到血清IgG4水平升高以及脑脊液 (CSF) 异常,如细胞和蛋白质升高.
- 神经成像显示了白质病变,多变性脑膜炎和LTEM.
结论:
- 神经IgG4-RD表现出显著的表型变异性.
- 获得活检的困难强调了需要修订神经IgG4-RD的诊断标准.
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