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Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy

(1) Background: Duchenne (DMD) is a rare neuromuscular disease that progressively weakens muscles, which severely impairs gait capacity. The Six Minute-Walk Test (6MWT), which is commonly used to evaluate and monitor the disease’s evolution, presents significant variability due to extrinsic factors...

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Autores principales: Migliorelli, Carolina, Gómez-Martinez, Meritxell, Subías-Beltrán, Paula, Claramunt-Molet, Mireia, Idelsohn-Zielonka, Sebastian, Mas-Hurtado, Eudald, Miralles, Felip, Montolio, Marisol, Roselló-Ruano, Marina, Medina-Cantillo, Julita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9861677/
https://www.ncbi.nlm.nih.gov/pubmed/36679627
http://dx.doi.org/10.3390/s23020831
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author Migliorelli, Carolina
Gómez-Martinez, Meritxell
Subías-Beltrán, Paula
Claramunt-Molet, Mireia
Idelsohn-Zielonka, Sebastian
Mas-Hurtado, Eudald
Miralles, Felip
Montolio, Marisol
Roselló-Ruano, Marina
Medina-Cantillo, Julita
author_facet Migliorelli, Carolina
Gómez-Martinez, Meritxell
Subías-Beltrán, Paula
Claramunt-Molet, Mireia
Idelsohn-Zielonka, Sebastian
Mas-Hurtado, Eudald
Miralles, Felip
Montolio, Marisol
Roselló-Ruano, Marina
Medina-Cantillo, Julita
author_sort Migliorelli, Carolina
collection PubMed
description (1) Background: Duchenne (DMD) is a rare neuromuscular disease that progressively weakens muscles, which severely impairs gait capacity. The Six Minute-Walk Test (6MWT), which is commonly used to evaluate and monitor the disease’s evolution, presents significant variability due to extrinsic factors such as patient motivation, fatigue, and learning effects. Therefore, there is a clear need for the establishment of precise clinical endpoints to measure patient mobility. (2) Methods: A novel score (6M+ and 2M+) is proposed, which is derived from the use of a new portable monitoring system capable of carrying out a complete gait analysis. The system includes several biomechanical sensors: a heart rate band, inertial measurement units, electromyography shorts, and plantar pressure insoles. The scores were obtained by processing the sensor signals and via gaussian-mixture clustering. (3) Results: The 6M+ and 2M+ scores were evaluated against the North Star Ambulatory Assessment (NSAA), the gold-standard for measuring DMD, and six- and two-minute distances. The 6M+ and 2M+ tests led to superior distances when tested against the NSAA. The 6M+ test and the 2M+ test in particular were the most correlated with age, suggesting that these scores better characterize the gait regressions in DMD. Additionally, the 2M+ test demonstrated an accuracy and stability similar to the 6M+ test. (4) Conclusions: The novel monitoring system described herein exhibited good usability with respect to functional testing in a clinical environment and demonstrated an improvement in the objectivity and reliability of monitoring the evolution of neuromuscular diseases.
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spelling pubmed-98616772023-01-22 Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy Migliorelli, Carolina Gómez-Martinez, Meritxell Subías-Beltrán, Paula Claramunt-Molet, Mireia Idelsohn-Zielonka, Sebastian Mas-Hurtado, Eudald Miralles, Felip Montolio, Marisol Roselló-Ruano, Marina Medina-Cantillo, Julita Sensors (Basel) Article (1) Background: Duchenne (DMD) is a rare neuromuscular disease that progressively weakens muscles, which severely impairs gait capacity. The Six Minute-Walk Test (6MWT), which is commonly used to evaluate and monitor the disease’s evolution, presents significant variability due to extrinsic factors such as patient motivation, fatigue, and learning effects. Therefore, there is a clear need for the establishment of precise clinical endpoints to measure patient mobility. (2) Methods: A novel score (6M+ and 2M+) is proposed, which is derived from the use of a new portable monitoring system capable of carrying out a complete gait analysis. The system includes several biomechanical sensors: a heart rate band, inertial measurement units, electromyography shorts, and plantar pressure insoles. The scores were obtained by processing the sensor signals and via gaussian-mixture clustering. (3) Results: The 6M+ and 2M+ scores were evaluated against the North Star Ambulatory Assessment (NSAA), the gold-standard for measuring DMD, and six- and two-minute distances. The 6M+ and 2M+ tests led to superior distances when tested against the NSAA. The 6M+ test and the 2M+ test in particular were the most correlated with age, suggesting that these scores better characterize the gait regressions in DMD. Additionally, the 2M+ test demonstrated an accuracy and stability similar to the 6M+ test. (4) Conclusions: The novel monitoring system described herein exhibited good usability with respect to functional testing in a clinical environment and demonstrated an improvement in the objectivity and reliability of monitoring the evolution of neuromuscular diseases. MDPI 2023-01-11 /pmc/articles/PMC9861677/ /pubmed/36679627 http://dx.doi.org/10.3390/s23020831 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Migliorelli, Carolina
Gómez-Martinez, Meritxell
Subías-Beltrán, Paula
Claramunt-Molet, Mireia
Idelsohn-Zielonka, Sebastian
Mas-Hurtado, Eudald
Miralles, Felip
Montolio, Marisol
Roselló-Ruano, Marina
Medina-Cantillo, Julita
Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy
title Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy
title_full Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy
title_fullStr Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy
title_full_unstemmed Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy
title_short Multidimensional Biomechanics-Based Score to Assess Disease Progression in Duchenne Muscular Dystrophy
title_sort multidimensional biomechanics-based score to assess disease progression in duchenne muscular dystrophy
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9861677/
https://www.ncbi.nlm.nih.gov/pubmed/36679627
http://dx.doi.org/10.3390/s23020831
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