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慢性炎症性脱髓鞘性多发性神经病。免疫球蛋白或血浆治疗前及治疗期间的传导阻滞。

Chronic inflammatory demyelinating polyneuropathy. Conduction failure before and during immunoglobulin or plasma therapy.

作者信息

van der Meché F G, Vermeulen M, Busch H F

机构信息

Department of Neurology, University Hospital Dijkzigt, Rotterdam, The Netherlands.

出版信息

Brain. 1989 Dec;112 ( Pt 6):1563-71. doi: 10.1093/brain/112.6.1563.

DOI:10.1093/brain/112.6.1563
PMID:2597997
Abstract

An earlier study has shown that patients with chronic inflammatory demyelinating polyneuropathy may improve after the infusion of fresh frozen plasma or high dose intravenous immunoglobulin. We studied 8 such responding patients with neurophysiological techniques. Before treatment all patients had decreased compound muscle action potentials (CMAP) and slowing of conduction. During several courses of treatment a progressive increase of the CMAPs occurred and in 5 patients a correlation between the CMAPs of the abductor pollicis brevis muscle and grasp force could be demonstrated. In 2 patients, increase in strength was correlated with shortening of the distal motor latency; no significant relationships were found between strength and conduction velocity. Different patterns in the distribution of conduction failure were observed. In 2 patients, lesions were distributed along the length of the motor fibres, whereas the sensory system was spared. In another 2 patients the lesions, most likely demyelinating, were localized distally and included the sensory system. The 4 remaining patients were more difficult to classify, but individually showed greater resemblance to one or other of the two described patterns. Different patterns of conduction failure may reflect different pathogenetic mechanisms.

摘要

一项较早的研究表明,慢性炎症性脱髓鞘性多发性神经病患者在输注新鲜冷冻血浆或大剂量静脉注射免疫球蛋白后可能会有所改善。我们使用神经生理学技术对8名有反应的此类患者进行了研究。治疗前,所有患者的复合肌肉动作电位(CMAP)均降低且传导减慢。在几个疗程的治疗过程中,CMAP逐渐增加,并且在5名患者中,拇短展肌的CMAP与握力之间存在相关性。在2名患者中,力量的增加与远端运动潜伏期的缩短相关;未发现力量与传导速度之间存在显著关系。观察到传导阻滞分布的不同模式。在2名患者中,病变沿运动纤维的长度分布,而感觉系统未受影响。在另外2名患者中,病变最可能是脱髓鞘性的,位于远端且累及感觉系统。其余4名患者更难分类,但个体上与上述两种模式中的一种或另一种更相似。不同的传导阻滞模式可能反映了不同的发病机制。

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