慢性炎症性脱叶林性多神经病与可逆性严重认知障碍和胃肠道功能障碍
Madison M Patrick1, Rachel Bielling1, Galen Postma2
1Clinical Sciences, Florida State University College of Medicine, Tallahassee, USA.
Cureus
|December 25, 2023
概括
一名患有认知能力下降,胃肠道问题和神经病变的患者经历了静脉注射免疫球蛋白治疗的快速改善,这表明了慢性炎症性脱髓化多神经病变 (CIDP) 的新变种.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
背景情况:
- 一名患者出现了快速进展的认知障碍,胃肠道功能障碍和普遍的神经肌病.
- 症状包括认知能力下降,肌肉衰弱,视力丧失,体重减轻和慢性腹.
研究的目的:
- 描述一种异常的可逆神经和胃肠道症状.
- 调查慢性炎症性脱髓化多神经病变 (CIDP) 的新表型变异的潜力.
主要方法:
- 诊断工作包括神经检查,认知评估 (SLUMS),眼科评估 (OCT),胃肠道研究,血清学测试,便测试,成像,神经传导研究和肌肉活检.
- 治疗包括静脉注射免疫球蛋白 (IVIg) 治疗,根据初始反应调整剂量.
主要成果:
- 神经传导研究证实了脱髓化的感觉运动外围神经病变.
- 在四个月内,IVIg治疗导致认知功能显著改善 (SLUMS得分正常化),肌肉强度和反射.
- 胃肠道症状消失了,但视力敏度和神经传导延迟保持不变.
结论:
- 患者对IVIg的剧烈反应表明一种常见的免疫机制.
- 这种病例可能代表一种新的CIDP表型变体,呈现出认知,胃肠道和神经肌肉症状.
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