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["枯萎的手臂”。一种多灶性运动神经病及鉴别诊断中的考量]

["The withered arm". A multifocal motor neuropathy and considerations in differential diagnosis].

作者信息

Andersen B, Lauritzen M J

机构信息

Klinisk neurofysiologisk afdeling, Amtssygehuset i Glostrup.

出版信息

Ugeskr Laeger. 1996 Jul 29;158(31):4390-4.

PMID:8759995
Abstract

Multifocal motor neuropathy (MMN) is a newly recognized disorder with a characteristic clinical picture. The diagnosis of MMN is established by the presence of a specific electrodiagnostic abnormality, conduction block confined to motor axons, and in some instances by the associated high titers of anti-GM1 antibodies. Clinical improvement may be achieved by treatment with intravenous immunoglobulin or cyclophosphamide, which underlines the importance of recognizing these patients. We present two patients with slowly progressive predominantly distal weakness and muscle wasting of an upper extremity characteristic of MMN. Extensive electrophysiological examinations are necessary to demonstrate conduction block of motor axons, which distinguishes patients with MMN from patients with motor neuron disease. Other diagnostic possibilities are discussed.

摘要

多灶性运动神经病(MMN)是一种新认识的具有特征性临床表现的疾病。MMN的诊断依据特定的电诊断异常(局限于运动轴突的传导阻滞)来确立,在某些情况下还依据相关的高滴度抗GM1抗体。静脉注射免疫球蛋白或环磷酰胺治疗可实现临床改善,这凸显了识别这些患者的重要性。我们报告了两名患者,他们表现为缓慢进展的、以上肢为主的远端无力和肌肉萎缩,具有MMN的特征。需要进行广泛的电生理检查以证实运动轴突的传导阻滞,这可将MMN患者与运动神经元病患者区分开来。还讨论了其他诊断可能性。

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