Department of Neurology, The First Affiliated Hospital of Xi'an Jiaotong University, 277 Yanta West Road, Xi'an, Shaanxi, 710061, China.
Department of Medical Imaging, The First Affiliated Hospital of Xi'an Jiaotong University, 277 Yanta West Road, Xi'an, Shaanxi, 710061, China.
Sci Rep. 2019 Dec 27;9(1):20059. doi: 10.1038/s41598-019-56665-8.
The aim of this study was to localize the anatomic distribution of upper motor neuron (UMN) loss through examining cortical thickness at the clinical onset of amyotrophic lateral sclerosis (ALS) and explore motor manifestation in functionally impaired body region attribute to impairment of lower motor neuron (LMN) or UMN or mixed LMN and UMN? The clinical features, cortical thickness of corresponding areas from different body regions in MRI and electromyography (EMG) data were collected from 108 classical ALS patients. The cortical thickness was thinner in ALS group than control group in bilateral head-face and upper-limb areas (p < 0.05). In head-face area, the cortical thickness of bulbar-onset group was significantly lower than that of control groups (p < 0.05). In upper-limb areas, the cortical thickness of cervical-onset group was significantly thinner than that of control group. Notably, the bulbar ALSFRS-R subscore was correlated with cortical thickness in bilateral head-face areas (p < 0.05). The bulbar ALSFRS-R subscore of the positive LMN damage group was lower compared to that of the negative LMN damage group (P < 0.001). The limb ALSFRS-R subscore correlated with compound muscle action potential (CMAP) amplitudes of median, ulnar, peroneal, and tibial nerves (P < 0.001), but was not related to cortical thickness. In conclusion, the UMN degeneration in ALS was derived from focal initiation, bulbar- and cervical-onset may date from head-face and upper-limb areas in motor homunculus cortex, respectively. The bulbar dysfunction was resulted from the mixed UMN and LMN impairment, while limb dysfunction derived mostly from LMN loss.
本研究旨在通过检查肌萎缩侧索硬化症（ALS）发病时皮质厚度，定位上运动神经元（UMN）损失的解剖分布，并探讨功能受损身体区域的运动表现是归因于下运动神经元（LMN）或 UMN 或混合 LMN 和 UMN 的损害。从 108 例经典 ALS 患者中收集了临床特征、MRI 和肌电图（EMG）数据中不同身体区域的皮质厚度。与对照组相比，ALS 组双侧头面部和上肢区域的皮质厚度较薄（p < 0.05）。在头面部区域，延髓起病组的皮质厚度明显低于对照组（p < 0.05）。在上肢区域，颈段起病组的皮质厚度明显变薄，与对照组相比。值得注意的是，双侧头面部区域的皮质厚度与延髓 ALSFRS-R 亚评分相关（p < 0.05）。阳性 LMN 损伤组的延髓 ALSFRS-R 亚评分低于阴性 LMN 损伤组（P < 0.001）。肢体 ALSFRS-R 亚评分与正中神经、尺神经、腓总神经和胫神经的复合肌肉动作电位（CMAP）振幅相关（P < 0.001），但与皮质厚度无关。总之，ALS 中的 UMN 退化是由局灶性起始引起的，延髓和颈段起病可能分别起源于运动同形同构皮质的头面部和上肢区域。延髓功能障碍是由混合 UMN 和 LMN 损害引起的，而肢体功能障碍主要是由 LMN 丧失引起的。
