Shimizu Y, Yokota T, Saito Y, Yuasa T, Miyatake T
Department of Neurology, Tokyo Medical and Dental University.
Rinsho Shinkeigaku. 1994 Aug;34(8):812-6.
In a 64-year-old woman with a clinical diagnosis of corticobasal degeneration, a hyperexcitability of central motor tract was demonstrated. She had akinesia, regidity, myoclonic movement, hyperreflexia, forced grasping and clumusiness on the right extremities. The asymmetry of these symptoms was striking. Dopa therapy was not effective. On single photon emission computed tomography (SPECT), blood perfusion was reduced especially on the left side in the frontoparietal area. She had large C-reflex after the median nerve stimulation only on the affected side. The intensity threshold of motor evoked potential by stimulation on the left motor cortex was lower than that on the right motor cortex. Conditioning stimulation of the median nerve on the affected side, greatly increased MEP size as compared to those of normal subjects, at conditioning-test (C-T) intervals of 40-80 ms; whereas, stimulation on the contralateral side did not. On the other hand, SEP did not have a marked increase or asymmetry in size and SEP recovery curve showed no hyperexcitability. These results indicate there is a hyperexcitability of the central motor tract on the affected side.
在一名临床诊断为皮质基底节变性的64岁女性患者中,证实存在中枢运动传导束的兴奋性增高。她有运动不能、强直、肌阵挛运动、反射亢进、右侧肢体强迫抓握和笨拙。这些症状的不对称性很明显。多巴治疗无效。在单光子发射计算机断层扫描(SPECT)中,尤其是额顶叶区域左侧的血流灌注减少。仅在患侧正中神经刺激后出现大的C反射。左侧运动皮层刺激诱发运动诱发电位的强度阈值低于右侧运动皮层。在40 - 80毫秒的条件刺激 - 测试(C - T)间隔下,患侧正中神经的条件刺激与正常受试者相比,显著增加了运动诱发电位的大小;而对侧刺激则没有。另一方面,体感诱发电位(SEP)在大小上没有明显增加或不对称,并且SEP恢复曲线未显示兴奋性增高。这些结果表明患侧中枢运动传导束存在兴奋性增高。