The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders

Upper motor neuron (UMN) is a term traditionally used for the corticospinal or pyramidal tract neuron synapsing with the lower motor neuron (LMN) in the anterior horns of the spinal cord. The upper motor neuron controls resting muscle tone and helps initiate voluntary movement of the musculoskeletal...

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Main Authors: Parvathi Menon, Steve Vucic
Format: Article
Language:English
Published: MDPI AG 2021-07-01
Series:Brain Sciences
Subjects:
Online Access:https://www.mdpi.com/2076-3425/11/8/958
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author Parvathi Menon
Steve Vucic
author_facet Parvathi Menon
Steve Vucic
author_sort Parvathi Menon
collection DOAJ
description Upper motor neuron (UMN) is a term traditionally used for the corticospinal or pyramidal tract neuron synapsing with the lower motor neuron (LMN) in the anterior horns of the spinal cord. The upper motor neuron controls resting muscle tone and helps initiate voluntary movement of the musculoskeletal system by pathways which are not completely understood. Dysfunction of the upper motor neuron causes the classical clinical signs of spasticity, weakness, brisk tendon reflexes and extensor plantar response, which are associated with clinically well-recognised, inherited and acquired disorders of the nervous system. Understanding the pathophysiology of motor system dysfunction in neurological disease has helped promote a greater understanding of the motor system and its complex cortical connections. This review will focus on the pathophysiology underlying progressive dysfunction of the UMN in amyotrophic lateral sclerosis and three other related adult-onset, progressive neurological disorders with prominent UMN signs, namely, primary lateral sclerosis, hereditary spastic paraplegia and primary progressive multiple sclerosis, to help promote better understanding of the human motor system and, by extension, related cortical systems.
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spelling doaj.art-fe02e4ac9ccc459b9cf6ae1b58c62fdd2023-11-22T06:57:58ZengMDPI AGBrain Sciences2076-34252021-07-0111895810.3390/brainsci11080958The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical DisordersParvathi Menon0Steve Vucic1Brain and Nerve Research Centre, University of Sydney, Sydney 2139, AustraliaBrain and Nerve Research Centre, University of Sydney, Sydney 2139, AustraliaUpper motor neuron (UMN) is a term traditionally used for the corticospinal or pyramidal tract neuron synapsing with the lower motor neuron (LMN) in the anterior horns of the spinal cord. The upper motor neuron controls resting muscle tone and helps initiate voluntary movement of the musculoskeletal system by pathways which are not completely understood. Dysfunction of the upper motor neuron causes the classical clinical signs of spasticity, weakness, brisk tendon reflexes and extensor plantar response, which are associated with clinically well-recognised, inherited and acquired disorders of the nervous system. Understanding the pathophysiology of motor system dysfunction in neurological disease has helped promote a greater understanding of the motor system and its complex cortical connections. This review will focus on the pathophysiology underlying progressive dysfunction of the UMN in amyotrophic lateral sclerosis and three other related adult-onset, progressive neurological disorders with prominent UMN signs, namely, primary lateral sclerosis, hereditary spastic paraplegia and primary progressive multiple sclerosis, to help promote better understanding of the human motor system and, by extension, related cortical systems.https://www.mdpi.com/2076-3425/11/8/958upper motor neuronALSPLSHSPPPMS
spellingShingle Parvathi Menon
Steve Vucic
The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders
Brain Sciences
upper motor neuron
ALS
PLS
HSP
PPMS
title The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders
title_full The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders
title_fullStr The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders
title_full_unstemmed The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders
title_short The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders
title_sort upper motor neuron improved knowledge from als and related clinical disorders
topic upper motor neuron
ALS
PLS
HSP
PPMS
url https://www.mdpi.com/2076-3425/11/8/958
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