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运动和感觉性脱髓鞘性多灶性单神经病(多灶性运动和感觉性脱髓鞘性神经病):是一种独立的疾病实体还是慢性炎症性脱髓鞘性多发性神经病的一种变异型?

Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy?

作者信息

Oh S J, Claussen G C, Kim D S

机构信息

Department of Neurology, The University of Alabama at Birmingham, and The Department of Veterans Affairs Medical Center, 35294, USA.

出版信息

J Peripher Nerv Syst. 1997;2(4):362-9.

PMID:10975745
Abstract

We report 16 patients with motor and sensory demyelinating mononeuropathy multiplex (MSDMM) or multifocal motor and sensory demyelinating neuropathy (MMSDN). These patients had the clinical pattern of motor and sensory mononeuropathy multiplex, electrophysiological evidence of demyelination including conduction block, and segmental demyelination in the sural nerve biopsy. Sixty per cent of patients had high CSF protein. Eighty per cent of patients showed good responsiveness to steroid treatment. Unlike multifocal motor neuropathy (MMN), MSDMM is characterized by a shorter course, sensory deficits and sensory nerve conduction abnormalities, absence of GM1 antibody in most patients tested, and a good response to steroid therapy. We believe that MSDMM represents a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) and an intermediate link between CIDP and MMN.

摘要

我们报告了16例运动和感觉性脱髓鞘性多灶性单神经病(MSDMM)或多灶性运动和感觉性脱髓鞘性神经病(MMSDN)患者。这些患者具有运动和感觉性多灶性单神经病的临床模式、包括传导阻滞在内的脱髓鞘电生理证据,以及腓肠神经活检中的节段性脱髓鞘。60%的患者脑脊液蛋白升高。80%的患者对类固醇治疗反应良好。与多灶性运动神经病(MMN)不同,MSDMM的特点是病程较短、存在感觉缺陷和感觉神经传导异常、大多数检测患者不存在GM1抗体,以及对类固醇治疗反应良好。我们认为MSDMM代表慢性炎症性脱髓鞘性多发性神经病(CIDP)的一种变异型,以及CIDP和MMN之间的中间环节。

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