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伴有传导阻滞的多灶性运动神经病:诊断与治疗中的当前问题

Multifocal motor neuropathy with conduction block: current issues in diagnosis and treatment.

作者信息

Nagale Smriti V, Bosch E Peter

机构信息

Department of Neurology, Mayo Clinic Scottsdale, Scottsdale, Arizona 85259, USA.

出版信息

Semin Neurol. 2003 Sep;23(3):325-34. doi: 10.1055/s-2003-814745.

Abstract

Multifocal motor neuropathy (MMN) with conduction block is an acquired, autoimmune-mediated neuropathy that is responsive to treatment. The clinical history is one of slowly, progressive distal weakness, which more commonly involves the upper extremities, and it affects mainly young adults. Physical examination reveals weakness without sensory loss in the distribution of individual nerves. Atrophy may be present, but hyperreflexia and spasticity are not seen. Electrophysiological studies reveal motor conduction blocks at sites not prone to compression with normal sensory responses. Immunoglobulin M anti-GM1 titers may be elevated. Treatment with human immunoglobulin or cyclophosphamide has been shown to improve strength in the majority of patients with MMN in the short term. However, motor strength and function may gradually decline over years in spite of long-term therapy.

摘要

伴有传导阻滞的多灶性运动神经病(MMN)是一种后天获得性、自身免疫介导的神经病,对治疗有反应。临床病史表现为缓慢进展的远端肌无力,更常见于上肢,主要影响年轻成年人。体格检查发现个别神经分布区域肌无力但无感觉丧失。可能存在萎缩,但未见反射亢进和痉挛。电生理研究显示在不易受压部位存在运动传导阻滞,感觉反应正常。免疫球蛋白M抗GM1滴度可能升高。已证明用人免疫球蛋白或环磷酰胺治疗可在短期内改善大多数MMN患者的肌力。然而,尽管进行了长期治疗,运动肌力和功能可能在数年内逐渐下降。

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