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Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy
INTRODUCTION/AIMS: Oculopharyngeal muscular dystrophy (OPMD) is a late‐onset, progressive muscle disease. Quantitative muscle ultrasound (QMUS) assesses structural changes in muscles and is a sensitive biomarker in neuromuscular disorders. Our aim of this study was to determine whether QMUS can dete...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley & Sons, Inc.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9804746/ https://www.ncbi.nlm.nih.gov/pubmed/35859342 http://dx.doi.org/10.1002/mus.27679 |
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author | Kroon, Rosemarie H. M. J. M. Kalf, Johanna G. Meijers, Rutger L. de Swart, Bert J. M. Cameron, Ian G. M. Doorduin, Jonne van Alfen, Nens van Engelen, Baziel G. M. Horlings, Corinne G. C. |
author_facet | Kroon, Rosemarie H. M. J. M. Kalf, Johanna G. Meijers, Rutger L. de Swart, Bert J. M. Cameron, Ian G. M. Doorduin, Jonne van Alfen, Nens van Engelen, Baziel G. M. Horlings, Corinne G. C. |
author_sort | Kroon, Rosemarie H. M. J. M. |
collection | PubMed |
description | INTRODUCTION/AIMS: Oculopharyngeal muscular dystrophy (OPMD) is a late‐onset, progressive muscle disease. Quantitative muscle ultrasound (QMUS) assesses structural changes in muscles and is a sensitive biomarker in neuromuscular disorders. Our aim of this study was to determine whether QMUS can detect muscle pathology and can be used as longitudinal imaging biomarker in OPMD. METHODS: Genetically confirmed OPMD patients, recruited by their treating physicians or from the national neuromuscular database, were examined twice, 20 months apart, using QMUS of orofacial and limb muscles, and measurements of functional capacity and muscle strength. Absolute echo intensity (AEI) and muscle thickness of all muscles were analyzed and correlated with clinical data. RESULTS: The tongue, deltoid, iliopsoas, rectus femoris, and soleus muscles showed increased AEI at baseline compared with normal values in 43 OPMD patients, with the rectus femoris being most often affected (51%).The AEI and muscle thickness of 9 of 11 muscles correlated significantly with the motor function measure, 10‐step stair test, swallowing capacity, dynamometry, Medical Research Council grade, tongue strength, and bite force (r = 0.302 to −0.711). Between baseline and follow‐up, deterioration in AEI was found for the temporalis, tongue, and deltoid muscles, and decreased muscle thickness was detected for the temporalis, masseter, digastric, tongue, deltoid, iliopsoas, and soleus muscles (P < .05). No relation was found between the change in AEI and repeat length or disease duration. DISCUSSION: QMUS detected muscle pathology and disease progression in OPMD over 20 months. We conclude that QMUS should be considered as a biomarker in treatment trials. |
format | Online Article Text |
id | pubmed-9804746 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | John Wiley & Sons, Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-98047462023-01-06 Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy Kroon, Rosemarie H. M. J. M. Kalf, Johanna G. Meijers, Rutger L. de Swart, Bert J. M. Cameron, Ian G. M. Doorduin, Jonne van Alfen, Nens van Engelen, Baziel G. M. Horlings, Corinne G. C. Muscle Nerve Clinical Research Articles INTRODUCTION/AIMS: Oculopharyngeal muscular dystrophy (OPMD) is a late‐onset, progressive muscle disease. Quantitative muscle ultrasound (QMUS) assesses structural changes in muscles and is a sensitive biomarker in neuromuscular disorders. Our aim of this study was to determine whether QMUS can detect muscle pathology and can be used as longitudinal imaging biomarker in OPMD. METHODS: Genetically confirmed OPMD patients, recruited by their treating physicians or from the national neuromuscular database, were examined twice, 20 months apart, using QMUS of orofacial and limb muscles, and measurements of functional capacity and muscle strength. Absolute echo intensity (AEI) and muscle thickness of all muscles were analyzed and correlated with clinical data. RESULTS: The tongue, deltoid, iliopsoas, rectus femoris, and soleus muscles showed increased AEI at baseline compared with normal values in 43 OPMD patients, with the rectus femoris being most often affected (51%).The AEI and muscle thickness of 9 of 11 muscles correlated significantly with the motor function measure, 10‐step stair test, swallowing capacity, dynamometry, Medical Research Council grade, tongue strength, and bite force (r = 0.302 to −0.711). Between baseline and follow‐up, deterioration in AEI was found for the temporalis, tongue, and deltoid muscles, and decreased muscle thickness was detected for the temporalis, masseter, digastric, tongue, deltoid, iliopsoas, and soleus muscles (P < .05). No relation was found between the change in AEI and repeat length or disease duration. DISCUSSION: QMUS detected muscle pathology and disease progression in OPMD over 20 months. We conclude that QMUS should be considered as a biomarker in treatment trials. John Wiley & Sons, Inc. 2022-08-12 2022-10 /pmc/articles/PMC9804746/ /pubmed/35859342 http://dx.doi.org/10.1002/mus.27679 Text en © 2022 The Authors. Muscle & Nerve published by Wiley Periodicals LLC. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Clinical Research Articles Kroon, Rosemarie H. M. J. M. Kalf, Johanna G. Meijers, Rutger L. de Swart, Bert J. M. Cameron, Ian G. M. Doorduin, Jonne van Alfen, Nens van Engelen, Baziel G. M. Horlings, Corinne G. C. Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
title | Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
title_full | Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
title_fullStr | Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
title_full_unstemmed | Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
title_short | Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
title_sort | muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy |
topic | Clinical Research Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9804746/ https://www.ncbi.nlm.nih.gov/pubmed/35859342 http://dx.doi.org/10.1002/mus.27679 |
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