Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy?
Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy?
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运动和感觉脱髓鞘性单神经病多发性（多灶性运动和感觉脱髓鞘性神经病）：慢性炎症性脱髓鞘性多发性神经病的一个单独实体或变体？
DOI：
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发表时间：
1997
影响因子：
3.8
通讯作者：
Kim Ds
中科院分区：
文献类型：
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作者：
Shin J. Oh;Claussen Gc;Kim Ds
我们报告16例多发性运动和感觉脱髓鞘单神经病(MSDMM)或多灶性运动和感觉脱髓鞘神经病(MMSDN)。临床表现为运动性和感觉性多发性神经病，电生理表现为包括传导阻滞在内的脱髓鞘，腓肠神经活检有节段性脱髓鞘。60%的患者脑脊液蛋白水平较高。80%的患者对类固醇治疗表现出良好的反应。与多灶性运动神经病(MMN)不同，MSDMM的特点是病程较短，感觉障碍和感觉神经传导异常，大多数患者检测不到GM1抗体，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)的变种，是CIDP和MMN之间的中间环节。
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.