Bouchard J P, Barbeau A, Bouchard R, Bouchard R W
Can J Neurol Sci. 1979 May;6(2):185-9. doi: 10.1017/s0317167100119614.
Twenty four ataxic patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction. The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct from Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies.
对24例共济失调患者进行了肌电图和神经传导研究。根据患者的来源地区、疾病进展情况和临床体征，将他们分为两组。第一组来自里穆斯基地区的患者表现出弗里德赖希共济失调的所有临床和电生理体征。第二组包括患有一种名为夏尔勒沃 - 萨格奈常染色体隐性痉挛性共济失调（ARSACS）的新综合征的患者。尽管后者的临床进展较好，但有更多的失神经肌电图体征，且运动传导速度较慢。两组在感觉神经传导方面均表现出相同且明显的异常。据我们所知，尚未有关于痉挛性共济失调电生理研究结果的报道。这些结果强调了痉挛性共济失调与弗里德赖希共济失调、痉挛性截瘫以及已知的家族性神经病变不同的地位。
