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伴有持续性传导阻滞的多灶性脱髓鞘性神经病

Multifocal demyelinating neuropathy with persistent conduction block.

作者信息

Lewis R A, Sumner A J, Brown M J, Asbury A K

出版信息

Neurology. 1982 Sep;32(9):958-64. doi: 10.1212/wnl.32.9.958.

Abstract

We describe five patients with a chronic asymmetric sensorimotor neuropathy most pronounced in the upper extremities with focal involvement of individual nerves. Diagnosis was established by electrophysiologic evidence of persistent multifocal conduction block. Sural nerve biopsy in three patients showed primarily demyelinating-remyelinating changes with varying degrees of fiber loss. Two patients had acute optic neuritis, indicating that the disorder was not always restricted to the peripheral nervous system. Two patients treated with corticosteroids improved, whereas three untreated patients had static deficits or steady progression of symptoms. Chronic multifocal demyelinating neuropathy with persistent conduction block seems to be a variant of chronic acquired demyelinating polyneuropathy and may be immunologically mediated.

摘要

我们描述了5例慢性非对称性感觉运动性神经病患者,这种神经病在上肢最为明显,个别神经有局灶性受累。通过持续性多灶性传导阻滞的电生理证据确诊。3例患者的腓肠神经活检主要显示脱髓鞘-再髓鞘化改变,并伴有不同程度的纤维丢失。2例患者发生急性视神经炎,表明该疾病并不总是局限于周围神经系统。2例接受皮质类固醇治疗的患者病情改善,而3例未治疗的患者存在静止性缺陷或症状稳步进展。伴有持续性传导阻滞的慢性多灶性脱髓鞘性神经病似乎是慢性获得性脱髓鞘性多发性神经病的一种变体,可能由免疫介导。

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