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[伴有持续性传导阻滞的多灶性脱髓鞘性多发性神经病(刘易斯-萨姆纳综合征)]

[Multifocal demyelinating polyneuropathy with persistent conduction block (Lewis-Sumner syndrome)].

作者信息

Mezaki T, Kaji R, Hamano T, Kimura J, Kameyama M

机构信息

Department of Neurology, Kyoto University.

出版信息

Rinsho Shinkeigaku. 1990 Nov;30(11):1266-70.

PMID:2085935
Abstract

Multifocal demyelinating neuropathy with persistent conduction block (Lewis-Sumner syndrome) is a variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which often clinically simulates a motor neuron disease (MND). We report here three patients initially suspected to have MND, who later were diagnosed as a Lewis-Sumner syndrome. One of them showed a remarkable clinical improvement after immunoglobulin therapy. The definitive diagnosis of this syndrome rests upon nerve conduction studies, uncovering multiple sites of persistent conduction block. Technically, it is important to exclude insufficient stimulus which may lead to an erroneous impression of conduction block. Magnetic stimulation, as compared to electric current, elicited larger responses possibly because of deeper current penetration. We found this mode of stimulation useful especially in testing focal demyelination requiring full activation of a diseased nerve at a most proximal segment.

摘要

伴有持续性传导阻滞的多灶性脱髓鞘性神经病(Lewis-Sumner综合征)是慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)的一种变异型,临床上常酷似运动神经元病(MND)。我们在此报告3例最初怀疑患有MND、后来被诊断为Lewis-Sumner综合征的患者。其中1例在接受免疫球蛋白治疗后临床症状显著改善。该综合征的确诊依赖于神经传导研究,以发现多个持续性传导阻滞部位。从技术角度讲,排除可能导致传导阻滞错误印象的刺激不足很重要。与电流相比,磁刺激引发的反应更大,可能是因为电流穿透更深。我们发现这种刺激方式在检测需要使病变神经在最近端节段充分激活的局灶性脱髓鞘时特别有用。

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