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Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1
INTRODUCTION: Axial muscles are involved earlier and to a greater extent in late-onset Pompe disease (LOPD) than in myotonic muscular dystrophy type 1 (DM1). We aimed to evaluate abdominal muscles in LOPD compared in DM1 using muscle ultrasonography. METHODS: Patients with LOPD (n = 3), DM1 (n = 10)...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488967/ https://www.ncbi.nlm.nih.gov/pubmed/36147041 http://dx.doi.org/10.3389/fneur.2022.944464 |
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author | Hsieh, Pei-Chen Chang, Chun-Wei Ro, Long-Sun Huang, Chin-Chang Chi, Jia-En Kuo, Hung-Chou |
author_facet | Hsieh, Pei-Chen Chang, Chun-Wei Ro, Long-Sun Huang, Chin-Chang Chi, Jia-En Kuo, Hung-Chou |
author_sort | Hsieh, Pei-Chen |
collection | PubMed |
description | INTRODUCTION: Axial muscles are involved earlier and to a greater extent in late-onset Pompe disease (LOPD) than in myotonic muscular dystrophy type 1 (DM1). We aimed to evaluate abdominal muscles in LOPD compared in DM1 using muscle ultrasonography. METHODS: Patients with LOPD (n = 3), DM1 (n = 10), and age- and gender-matched healthy subjects (n = 34) were enrolled for muscle ultrasonography. Patients with LOPD and DM1 were 20 to 59 years of age with a disease duration ranging between 7 and 30 years. A multifrequency linear transducer was used to evaluate quality and thickness in the abdominal muscles and extremities. RESULTS: The quantitative muscle echo score revealed a higher Z score in abdominal muscles in Patients with LOPD (scores were relatively normal for the biceps and flexor digitorum groups). Patients with LOPD had significantly lower abdominal muscle thickness than patients with DM1. Abdominal muscle strength was significantly correlated with the muscle echogenicity, trunk impairment scale, and trunk control test. The extremities' sum score was correlated with the total Medical Research Council score. DISCUSSION: The increased quantitative muscle score in abdominal muscles, sparing the biceps and flexor digitorum groups, may offer differential diagnosis between LOPD and DM1. Ultrasound can easily access abdominal muscles and investigate muscle echogenicity and thickness. A quantitative approach using muscle echogenicity rather than muscle thickness may provide a greater correlation with trunk muscle function. |
format | Online Article Text |
id | pubmed-9488967 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-94889672022-09-21 Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 Hsieh, Pei-Chen Chang, Chun-Wei Ro, Long-Sun Huang, Chin-Chang Chi, Jia-En Kuo, Hung-Chou Front Neurol Neurology INTRODUCTION: Axial muscles are involved earlier and to a greater extent in late-onset Pompe disease (LOPD) than in myotonic muscular dystrophy type 1 (DM1). We aimed to evaluate abdominal muscles in LOPD compared in DM1 using muscle ultrasonography. METHODS: Patients with LOPD (n = 3), DM1 (n = 10), and age- and gender-matched healthy subjects (n = 34) were enrolled for muscle ultrasonography. Patients with LOPD and DM1 were 20 to 59 years of age with a disease duration ranging between 7 and 30 years. A multifrequency linear transducer was used to evaluate quality and thickness in the abdominal muscles and extremities. RESULTS: The quantitative muscle echo score revealed a higher Z score in abdominal muscles in Patients with LOPD (scores were relatively normal for the biceps and flexor digitorum groups). Patients with LOPD had significantly lower abdominal muscle thickness than patients with DM1. Abdominal muscle strength was significantly correlated with the muscle echogenicity, trunk impairment scale, and trunk control test. The extremities' sum score was correlated with the total Medical Research Council score. DISCUSSION: The increased quantitative muscle score in abdominal muscles, sparing the biceps and flexor digitorum groups, may offer differential diagnosis between LOPD and DM1. Ultrasound can easily access abdominal muscles and investigate muscle echogenicity and thickness. A quantitative approach using muscle echogenicity rather than muscle thickness may provide a greater correlation with trunk muscle function. Frontiers Media S.A. 2022-09-06 /pmc/articles/PMC9488967/ /pubmed/36147041 http://dx.doi.org/10.3389/fneur.2022.944464 Text en Copyright © 2022 Hsieh, Chang, Ro, Huang, Chi and Kuo. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neurology Hsieh, Pei-Chen Chang, Chun-Wei Ro, Long-Sun Huang, Chin-Chang Chi, Jia-En Kuo, Hung-Chou Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 |
title | Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 |
title_full | Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 |
title_fullStr | Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 |
title_full_unstemmed | Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 |
title_short | Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1 |
title_sort | ultrasonography of abdominal muscles: differential diagnosis of late-onset pompe disease and myotonic dystrophy type 1 |
topic | Neurology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488967/ https://www.ncbi.nlm.nih.gov/pubmed/36147041 http://dx.doi.org/10.3389/fneur.2022.944464 |
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