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高嗜酸性粒细胞综合征中的神经病变

Neuropathy in the hypereosinophilic syndrome.

作者信息

Dorfman L J, Ransom B R, Forno L S, Kelts A

出版信息

Muscle Nerve. 1983 May;6(4):291-8. doi: 10.1002/mus.880060408.

Abstract

Peripheral nerve dysfunction was a prominent clinical feature in each of three patients with the hypereosinophilic syndrome (HES). The neuropathy, occurring at the onset of marked eosinophilia or at a time of its worsening, had a painful onset, evolved over 1-4 weeks, and affected both sensory and motor function. Electrodiagnostic studies demonstrated both multifocal and generalized nerve involvement, with the former predominating (multiple mononeuropathy). Nerve and muscle biopsies from two patients revealed severe axonal degeneration with neurogenic atrophy of muscle. The eosinophilia decreased dramatically with corticosteroid treatment and the neuropathy gradually improved, but all three patients manifested residual neurologic disability 17-22 months after onset. The neuropathy may be on an ischemic basis or may be related to neurotoxicity of eosinophil products.

摘要

外周神经功能障碍是三例高嗜酸性粒细胞综合征(HES)患者的显著临床特征。神经病变在明显嗜酸性粒细胞增多症发作时或病情加重时出现,起病时有疼痛,病程为1 - 4周,影响感觉和运动功能。电诊断研究显示多灶性和全身性神经受累,以前者为主(多灶性单神经病)。两名患者的神经和肌肉活检显示严重的轴索性变性伴神经性肌肉萎缩。使用皮质类固醇治疗后嗜酸性粒细胞增多症显著减轻,神经病变逐渐改善,但所有三名患者在发病后17 - 22个月均表现出残留的神经功能残疾。这种神经病变可能基于缺血,也可能与嗜酸性粒细胞产物的神经毒性有关。

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