Abstract
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.
Highlights
Schuster et al reported that bulbar-onset patients showed cortical thinning in the bilateral bulbar-segment of motor homunculus
Motor neuron degeneration in upper motor neuron (UMN) and lower motor neuron (LMN) levels was detected through examining cortical thickness and EMG in amyotrophic lateral sclerosis (ALS) patients, respectively
We found that bulbar-onset patients manifested cortex thinning of head-face areas and bulbar denervation in EMG, both of them were related to bulbar ALSFRS-R subscore
Summary
Schuster et al reported that bulbar-onset patients showed cortical thinning in the bilateral bulbar-segment of motor homunculus. Patients with lower limb-onset demonstrated thinning in the bilateral segments of the motor homunculus representing arms and legs[3]. The different region onset was an aspect of clinical heterogeneity in ALS1. It has been suggested that different body onsets correspond to different motor homunculus partitions[3]. When localizing the anatomic distribution of UMN loss, more detailed motor partitions are bound to help characterize UMN damages. Jiang et al proposed a more detailed and refined division of the motor homunculus cortex, which comprised of head-face, tongue-larynx, upper-limb, trunk, and lower-limb areas[7]
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