Department of Neurosciences, Hospital de Santa Maria, Avenue Prof. Egas Moniz, Lisbon 1649-028, Portugal.
J Clin Neurosci. 2012 Feb;19(2):318-21. doi: 10.1016/j.jocn.2011.02.048. Epub 2011 Dec 5.
Flail-leg syndrome or lower limb diplegia is a form of motor neuron disease characterized by a slower progression rate. The differential diagnosis with motor neuropathy is important. We present two patients with a previous diagnosis of amyotrophic lateral sclerosis (ALS)-flail-leg syndrome, in whom neurophysiological studies suggested proximal conduction block. Both patients responded to immunomodulatory therapy, which suggested an immunologically mediated, treatable flail-leg syndrome phenotype. We stress the importance of fasciculations in the diagnosis of ALS, and the study of nerve root conduction in the differential diagnosis.
摆腿综合征或下肢弛缓性瘫痪是一种运动神经元病，其特征为进展速度较慢。与运动神经病的鉴别诊断很重要。我们介绍了两名先前诊断为肌萎缩侧索硬化症（ALS）-摆腿综合征的患者，神经生理学研究提示存在近端传导阻滞。两名患者均对免疫调节治疗有反应，提示存在免疫介导的、可治疗的摆腿综合征表型。我们强调了肌束震颤在 ALS 诊断中的重要性，以及神经根传导研究在鉴别诊断中的重要性。
