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[多灶性运动神经病:诊断与鉴别诊断]

[Multifocal motor neuropathy: diagnosis and differential diagnosis].

作者信息

Fischer D, Reimann J, Schröder R, Schmidt St

机构信息

Neurologische Klinik und Poliklinik, Rheinische Friedrich-Wilhelms-Universität, Sigmund-Freud-Str.25, 53105 Bonn.

出版信息

Nervenarzt. 2002 Dec;73(12):1153-9. doi: 10.1007/s00115-002-1440-y.

Abstract

Multifocal motor neuropathy (MMN) is an acquired immune mediated motor neuropathy with characteristic clinical and neurographic features.Clinically, MMN is characterized by progressive, predominantly distal and asymmetrical limb weakness. Neurographic recordings demonstrate features of multifocal demyelination with or without conduction block. Sensory nerves are not affected. Due to strict diagnostic criteria,MMN may be underdiagnosed in patients with motor neuropathies. Since intravenous immunglobulins are an efficient therapy for MMN,clinical and electrophysiological differentiation from other neuromuscular disorders is mandatory to prevent progressive impairment of motor function. We present a patient with MMN and review the clinical, electrophysiological, and histological features. In addition,pathogenesis, differential diagnosis and treatment of MMN are discussed.

摘要

多灶性运动神经病(MMN)是一种获得性免疫介导的运动神经病,具有特征性的临床和神经电生理表现。临床上,MMN的特点是进行性、主要为远端和不对称的肢体无力。神经电生理记录显示多灶性脱髓鞘的特征,伴或不伴有传导阻滞。感觉神经不受影响。由于诊断标准严格,MMN在运动神经病患者中可能诊断不足。鉴于静脉注射免疫球蛋白是治疗MMN的有效方法,因此必须从临床和电生理方面将其与其他神经肌肉疾病进行鉴别,以防止运动功能的进行性损害。我们报告一例MMN患者,并回顾其临床、电生理和组织学特征。此外,还讨论了MMN的发病机制、鉴别诊断和治疗。

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