Urban P P, Vogt T, Hopf H C
Department of Neurology, University of Mainz, Germany.
Brain. 1998 Jun;121 ( Pt 6):1099-108. doi: 10.1093/brain/121.6.1099.
We investigated corticobulbar tract function by recording from the tongue and orofacial muscles and using transcranial magnetic stimulation (TMS) in 30 patients with amyotrophic lateral sclerosis (ALS) in order to improve the diagnostic yield in the detection of subclinical upper motor neuron (UMN) dysfunction. A UMN lesion was assumed when the peripheral conduction time and amplitude of the M-wave were within normal range and either the response to cortical stimulation was absent, or the central conduction time was delayed (> mean + 2.5 SD). Only two patients showed clinical evidence of UMN involvement in the cranial nerves, while TMS demonstrated corticobulbar tract dysfunction in the orofacial muscles in 17 patients (57%) and to the tongue in 15 patients (50%). Following recording at both sites, corticobulbar tract dysfunction was confirmed in 21 patients (70%). Twelve out of 13 patients with bulbar symptoms had evidence of additional subclinical corticobulbar tract involvement using TMS. In seven out of 15 patients with isolated limb involvement, subclinical corticobulbar tract dysfunction was demonstrated. Our results indicate the presence of early and, in most cases, subclinical corticobulbar tract involvement in the pathways to the orofacial muscles and tongue in ALS.
我们对30例肌萎缩侧索硬化症（ALS）患者的舌肌和口面部肌肉进行记录，并使用经颅磁刺激（TMS）来研究皮质延髓束功能，以提高亚临床性上运动神经元（UMN）功能障碍检测中的诊断率。当M波的外周传导时间和波幅在正常范围内，且对皮质刺激无反应或中枢传导时间延迟（>平均值+2.5标准差）时，则假定存在UMN病变。只有2例患者表现出临床证据支持UMN累及脑神经，而TMS显示17例患者（57%）的口面部肌肉存在皮质延髓束功能障碍，15例患者（50%）的舌肌存在皮质延髓束功能障碍。在两个部位进行记录后，21例患者（70%）被证实存在皮质延髓束功能障碍。13例有延髓症状的患者中，有12例通过TMS显示有额外的亚临床皮质延髓束受累证据。在15例仅有肢体受累的患者中，有7例显示存在亚临床皮质延髓束功能障碍。我们的结果表明，在ALS患者中，通往口面部肌肉和舌肌的通路存在早期且在大多数情况下为亚临床的皮质延髓束受累情况。
