Finsterer J, Mamoli B
Neurologische Abteilung, Neurologisches Krankenhaus Rosenhügel, Wien.
Wien Klin Wochenschr. 1995;107(10):301-8.
Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory involvement, but without upper motor neuron signs. The cardinal feature and primary pathophysiological basis for the weakness is the multifocal motor conduction block which remains stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal stimulus response without any change in the negative peak duration. Nerves at the site of the conduction block show demyelination, endoneural edema, rudimentary onion bulbs and lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid antibodies, which may block the Na+ channels, produce demyelination or interfere with remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and cyclophosphamide.
多灶性运动神经病，其临床表现类似下运动神经元病，是一种罕见且特殊的脱髓鞘性神经病，其特征为在单根周围神经分布范围内出现缓慢进展的不对称肢体无力、肌肉萎缩、痉挛、肌束震颤以及罕见的感觉受累，但无锥体束征。肌无力的主要特征和主要病理生理基础是多灶性运动传导阻滞，该阻滞在同一部位多年保持稳定，且局限于运动轴突。其定义为近端刺激时复合肌肉动作电位（CMAP）和负向波峰面积较远端刺激反应降低超过50%，而负向波峰持续时间无变化。传导阻滞部位的神经显示脱髓鞘、神经内膜水肿、初级洋葱球样结构和淋巴细胞炎症。感觉神经可能显示轻度脱髓鞘、轴突丢失和淋巴细胞炎症。大多数患者抗糖脂抗体滴度升高，这些抗体可能阻断钠通道、产生脱髓鞘或干扰髓鞘再生。然而，它们在多灶性运动神经病发病机制中的作用仍不确定。多灶性运动神经病被认为是慢性炎症性脱髓鞘性多发性神经病的主要运动型变体，最佳治疗方法是使用免疫球蛋白和环磷酰胺。
