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高嗜酸性粒细胞综合征:尽管嗜酸性粒细胞水平得到控制,但周围神经病变仍在进展。

Hypereosinophilic syndrome: progression of peripheral neuropathy despite controlled eosinophil levels.

作者信息

Cengiz B, Sucak G, Kuruoğlu R, Tan E

机构信息

Department of Neurology, Gazi University Faculty of Medicine, Ankara, Turkey.

出版信息

Acta Neurol Belg. 1999 Jun;99(2):133-7.

PMID:10427356
Abstract

Idiopathic hypereosinophilic syndrome (HES) is a disorder of the hematopoietic system, characterized by persistent elevation in the total eosinophil count (> 1500/microliter) for over 6 months, associated with organ damage and no detectable underlying cause. Treatment is centered on the reduction of total circulating eosinophils, which generally leads to remission of symptoms. We report a 68-year-old female patient with HES and peripheral neuropathy, presenting with cutaneous lesions, mental changes, cardiac and pulmonary symptoms, followed by right foot drop and eventually paraparesis, which caused an inability to ambulate. Weakness progressed to include the upper extremities despite adequate control of eosinophilia by steroids. Worsening of the peripheral neuropathy can occur despite lowering of the eosinophil levels.

摘要

特发性高嗜酸性粒细胞综合征(HES)是一种造血系统疾病,其特征为嗜酸性粒细胞总数持续升高(>1500/微升)超过6个月,伴有器官损害且无可检测到的潜在病因。治疗的核心是减少循环中的嗜酸性粒细胞总数,这通常会导致症状缓解。我们报告了一名68岁患有HES和周围神经病变的女性患者,该患者出现皮肤病变、精神改变、心脏和肺部症状,随后出现右脚下垂,最终发展为双下肢轻瘫,导致无法行走。尽管使用类固醇充分控制了嗜酸性粒细胞增多,但肌无力仍进展至累及上肢。尽管嗜酸性粒细胞水平降低,但周围神经病变仍可能恶化。

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