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In amyotrophic lateral sclerosis (ALS), it is not clear how the initial lesions develop and further expand in the upper and lower motor neuron (UMN and LMN) systems.
Among the various variants of ALS, hemiplegic ALS is characterized by significant asymmetry in the clinical symptoms of the entire disease.
This article will further discuss the pathological mechanisms that may contribute to the above-mentioned diseases.
In patient 1, LMN lesions were severe in the left lumbar spinal cord (Figure 1A-4), moderate in the left cervical spinal cord (Figure 1A-2) and mild in the right lumbar umbilicus (Figure 1A-3); right cervical spinal cord No obvious lesions were seen (Figure 1A-1).
Autopsy results of patient 1
In the current patients, the LMN and UMN lesions are on the same side, suggesting that there may be a degenerative link between the UMN and the LMN system.
The current findings in this article indicate that the pathogenesis of hemiplegic ALS may promote the continuous spread of lesions in the interconnected UMN and LMN systems.
Further research is necessary to biochemically characterize pTDP-43, which is inherent in this abnormal ALS phenotype, in which commissural transmission at both the UMN and LMN levels is reduced.
SainouchiM ,TanakaH ,ShimizuH SainouchiM Sainouchi TanakaH Tanaka ShimizuH Shimizu , et al bmj.
com/content/early/2021/04/21/jnnp-2021-326257" target="_blank" rel="noopener">Hemiplegic-type ALS: clinicopathological features of two autopsied patients Journal of Neurology, Neurosurgery & Psychiatry Published Online First: 21 April 2021.
Published Online First: doi: 10.
1136/jnnp-2021-326257 doi: leave a message here
