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抗中性粒细胞胞浆抗体阴性的嗜酸性肉芽肿性多血管炎中出现传导阻滞的神经病变

[Neuropathy presenting conduction block in ANCA-negative eosinophilic granulomatosis with polyangiitis].

作者信息

Honda Masaya, Takeshita Yukio, Koga Michiaki, Sato Ryota, Omoto Masatoshi, Kanda Takashi

机构信息

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.

出版信息

Rinsho Shinkeigaku. 2021 Sep 28;61(9):613-617. doi: 10.5692/clinicalneurol.cn-001564. Epub 2021 Aug 26.

DOI:10.5692/clinicalneurol.cn-001564
PMID:34433740
Abstract

A 74-year-old woman with a history of asthma and allergic rhinitis rapidly developed multiple mononeuropathy. Although anti-neutrophil cytoplasmic antibodies were negative, the presence of eosinophilia and eosinophilic infiltrations in the sural nerve led to a diagnosis of eosinophilic granulomatosis with polyangiitis. A motor nerve conduction study on admission revealed conduction block, which promptly disappeared after initiating immunotherapy without findings suggestive for remyelination or axonal degeneration. This electrophysiological change distinct from that of Wallerian degeneration. A biopsy of the sural nerve showed many eosinophil infiltrations and degranulation of eosinophilic cationic protein within nerve fascicles, whereas findings of necrotizing vasculitis were absent. These findings suggest that a direct effect of eosinophilic cationic protein, rather than ischemic damage due to vasculitis, was the main mechanism of transient nerve conduction failure in this patient.

摘要

一名有哮喘和过敏性鼻炎病史的74岁女性迅速发展为多发性单神经病。尽管抗中性粒细胞胞浆抗体阴性,但嗜酸性粒细胞增多以及腓肠神经中嗜酸性粒细胞浸润导致诊断为嗜酸性肉芽肿性多血管炎。入院时的运动神经传导研究显示传导阻滞,在开始免疫治疗后迅速消失,未发现提示髓鞘再生或轴索变性的表现。这种电生理变化不同于华勒变性。腓肠神经活检显示神经束内有许多嗜酸性粒细胞浸润和嗜酸性阳离子蛋白脱颗粒,而无坏死性血管炎表现。这些发现表明,嗜酸性阳离子蛋白的直接作用而非血管炎导致的缺血性损伤是该患者短暂性神经传导障碍的主要机制。

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