Assessment of the upper motor neuron in amyotrophic lateral sclerosis

Clinical signs of upper motor neuron (UMN) involvement are an important component in supporting the diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is concurrently affected by muscle wasting and lower motor neuron degeneration, particularly in th...

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मुख्य लेखकों: Huynh, W, Simon, N, Grosskreutz, J, Turner, M, Vucic, S, Kiernan, M
स्वरूप: Journal article
प्रकाशित: Elsevier 2016
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author Huynh, W
Simon, N
Grosskreutz, J
Turner, M
Vucic, S
Kiernan, M
author_facet Huynh, W
Simon, N
Grosskreutz, J
Turner, M
Vucic, S
Kiernan, M
author_sort Huynh, W
collection OXFORD
description Clinical signs of upper motor neuron (UMN) involvement are an important component in supporting the diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is concurrently affected by muscle wasting and lower motor neuron degeneration, particularly in the early symptomatic stages of ALS. Whilst recent criteria have been proposed to facilitate improved detection of lower motor neuron impairment through electrophysiological features that have improved diagnostic sensitivity, assessment of upper motor neuron involvement remains essentially clinical. As a result, there is often a significant diagnostic delay that in turn may impact institution of disease-modifying therapy and access to other optimal patient management. Biomarkers of pathological UMN involvement are also required to ensure patients with suspected ALS have timely access to appropriate therapeutic trials. We provide an overview of current and recently developed assessments, including novel imaging and electrophysiological techniques used to study corticomotoneuronal pathology in ALS.
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spelling oxford-uuid:74fdc1bb-d6ce-4e3d-b7b1-40c947484d5f2022-03-26T20:06:34ZAssessment of the upper motor neuron in amyotrophic lateral sclerosisJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:74fdc1bb-d6ce-4e3d-b7b1-40c947484d5fSymplectic Elements at OxfordElsevier2016Huynh, WSimon, NGrosskreutz, JTurner, MVucic, SKiernan, MClinical signs of upper motor neuron (UMN) involvement are an important component in supporting the diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is concurrently affected by muscle wasting and lower motor neuron degeneration, particularly in the early symptomatic stages of ALS. Whilst recent criteria have been proposed to facilitate improved detection of lower motor neuron impairment through electrophysiological features that have improved diagnostic sensitivity, assessment of upper motor neuron involvement remains essentially clinical. As a result, there is often a significant diagnostic delay that in turn may impact institution of disease-modifying therapy and access to other optimal patient management. Biomarkers of pathological UMN involvement are also required to ensure patients with suspected ALS have timely access to appropriate therapeutic trials. We provide an overview of current and recently developed assessments, including novel imaging and electrophysiological techniques used to study corticomotoneuronal pathology in ALS.
spellingShingle Huynh, W
Simon, N
Grosskreutz, J
Turner, M
Vucic, S
Kiernan, M
Assessment of the upper motor neuron in amyotrophic lateral sclerosis
title Assessment of the upper motor neuron in amyotrophic lateral sclerosis
title_full Assessment of the upper motor neuron in amyotrophic lateral sclerosis
title_fullStr Assessment of the upper motor neuron in amyotrophic lateral sclerosis
title_full_unstemmed Assessment of the upper motor neuron in amyotrophic lateral sclerosis
title_short Assessment of the upper motor neuron in amyotrophic lateral sclerosis
title_sort assessment of the upper motor neuron in amyotrophic lateral sclerosis
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AT turnerm assessmentoftheuppermotorneuroninamyotrophiclateralsclerosis
AT vucics assessmentoftheuppermotorneuroninamyotrophiclateralsclerosis
AT kiernanm assessmentoftheuppermotorneuroninamyotrophiclateralsclerosis