Twitch force in human Amyotrophic Lateral Sclerosis

IntroductionThis study investigated differences in muscle twitch force between slow and fast progressors of amyotrophic lateral sclerosis (ALS) to better understand disease heterogeneity and identify potential biomarkers of disease progression.MethodsForty-four ALS patients were classified as slow o...

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Main Authors: Laura Libonati, Chiara Cambieri, Marco Ceccanti, Federica Moret, Matteo Di Giulio, Eleonora Palma, Maurizio Inghilleri
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-05-01
Series:Frontiers in Neurology
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Online Access:https://www.frontiersin.org/articles/10.3389/fneur.2025.1590950/full
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author Laura Libonati
Chiara Cambieri
Marco Ceccanti
Federica Moret
Matteo Di Giulio
Eleonora Palma
Maurizio Inghilleri
Maurizio Inghilleri
author_facet Laura Libonati
Chiara Cambieri
Marco Ceccanti
Federica Moret
Matteo Di Giulio
Eleonora Palma
Maurizio Inghilleri
Maurizio Inghilleri
author_sort Laura Libonati
collection DOAJ
description IntroductionThis study investigated differences in muscle twitch force between slow and fast progressors of amyotrophic lateral sclerosis (ALS) to better understand disease heterogeneity and identify potential biomarkers of disease progression.MethodsForty-four ALS patients were classified as slow or fast progressors based on disease progression rates. Electrophysiological assessments, including compound muscle action potential (CMAP) and muscle force measurements, were conducted. Creatine kinase (CK) levels were also evaluated.ResultsSlow progressors demonstrated significantly higher muscle peak force and area under the curve (AUC) compared to fast progressors, reflecting greater muscle strength and endurance. CK levels were also elevated in slow progressors.DiscussionDespite similar CMAp values, slow progressors retained greater muscle strength, possibly due to a reduced degeneration of fast-twitch fibers and compensatory axonal sprouting. These adaptations may preserve muscle function and elevate CK levels, suggesting better muscle integrity in slow progressors.ConclusionMuscle function profiles and CK levels are promising indicators of ALS progression. These findings could enhance early detection of disease progression and lead to targeted interventions to preserve muscle function. Further research is needed to validate these results and explore the underlying functional mechanisms of disease heterogeneity.
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spelling doaj-art-c07d07f10c2f4164a8d8e292c0e3a3442025-08-20T02:16:06ZengFrontiers Media S.A.Frontiers in Neurology1664-22952025-05-011610.3389/fneur.2025.15909501590950Twitch force in human Amyotrophic Lateral SclerosisLaura Libonati0Chiara Cambieri1Marco Ceccanti2Federica Moret3Matteo Di Giulio4Eleonora Palma5Maurizio Inghilleri6Maurizio Inghilleri7Department of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell’Università, Rome, ItalyDepartment of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell’Università, Rome, ItalyDepartment of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell’Università, Rome, ItalyDepartment of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell’Università, Rome, ItalyDepartment of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell’Università, Rome, ItalyDepartment of Physiology and Pharmacology, Istituto Pasteur-Fondazione Cenci Bolognetti, University of Rome Sapienza, Rome, ItalyDepartment of Human Neurosciences, Rare Neuromuscular Diseases Center, Sapienza University of Rome, Viale Dell’Università, Rome, ItalyIRCCS Neuromed, Pozzilli, ItalyIntroductionThis study investigated differences in muscle twitch force between slow and fast progressors of amyotrophic lateral sclerosis (ALS) to better understand disease heterogeneity and identify potential biomarkers of disease progression.MethodsForty-four ALS patients were classified as slow or fast progressors based on disease progression rates. Electrophysiological assessments, including compound muscle action potential (CMAP) and muscle force measurements, were conducted. Creatine kinase (CK) levels were also evaluated.ResultsSlow progressors demonstrated significantly higher muscle peak force and area under the curve (AUC) compared to fast progressors, reflecting greater muscle strength and endurance. CK levels were also elevated in slow progressors.DiscussionDespite similar CMAp values, slow progressors retained greater muscle strength, possibly due to a reduced degeneration of fast-twitch fibers and compensatory axonal sprouting. These adaptations may preserve muscle function and elevate CK levels, suggesting better muscle integrity in slow progressors.ConclusionMuscle function profiles and CK levels are promising indicators of ALS progression. These findings could enhance early detection of disease progression and lead to targeted interventions to preserve muscle function. Further research is needed to validate these results and explore the underlying functional mechanisms of disease heterogeneity.https://www.frontiersin.org/articles/10.3389/fneur.2025.1590950/fullamyotrophic lateral sclerosismuscle functionelectromechanical couplingdisease phenotypedisease progression
spellingShingle Laura Libonati
Chiara Cambieri
Marco Ceccanti
Federica Moret
Matteo Di Giulio
Eleonora Palma
Maurizio Inghilleri
Maurizio Inghilleri
Twitch force in human Amyotrophic Lateral Sclerosis
Frontiers in Neurology
amyotrophic lateral sclerosis
muscle function
electromechanical coupling
disease phenotype
disease progression
title Twitch force in human Amyotrophic Lateral Sclerosis
title_full Twitch force in human Amyotrophic Lateral Sclerosis
title_fullStr Twitch force in human Amyotrophic Lateral Sclerosis
title_full_unstemmed Twitch force in human Amyotrophic Lateral Sclerosis
title_short Twitch force in human Amyotrophic Lateral Sclerosis
title_sort twitch force in human amyotrophic lateral sclerosis
topic amyotrophic lateral sclerosis
muscle function
electromechanical coupling
disease phenotype
disease progression
url https://www.frontiersin.org/articles/10.3389/fneur.2025.1590950/full
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