Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy

Practice of sports during childhood or adolescence correlates with an earlier onset and more rapidly progressing phenotype in dysferlinopathies. To determine if this correlation relates to greater muscle pathology that persists into adulthood, we investigated the effect of exercise on the degree of...

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Autores: Moore, U, Jacobs, M, Fernandez-Torron, R, Rossello, JL, Smith, FE, James, M, Mayhew, A, Rufibach, L, Carlier, PG, Blamire, AM, Day, JW, Jones, KJ, Bharucha-Goebel, DX, Salort-Campana, E, Pestronk, A, Walter, MC, Paradas, C, Stojkovic, T, Mori-Yoshimura, M, Bravver, E, Pegoraro, E, Mendell, JR, Bushby, K, Straub, V, Diaz-Manera, J
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2020
País:España
Institución:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repositorio:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:oai:iibsantpau.fundanetsuite.com:p11743
Acceso en línea:https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=11743
https://ddd.uab.cat/record/252276
Access Level:acceso abierto
Palabra clave:dysferlinopathy
Magnetic Resonace Imaging (MRI)
Miyoshi myopathy
LGMDR2
limb girdle muscle dystrophy
exercise
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spelling Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With DysferlinopathyMoore, UJacobs, MFernandez-Torron, RRossello, JLSmith, FEJames, MMayhew, ARufibach, LCarlier, PGBlamire, AMDay, JWJones, KJBharucha-Goebel, DXSalort-Campana, EPestronk, AWalter, MCParadas, CStojkovic, TMori-Yoshimura, MBravver, EPegoraro, EMendell, JRBushby, KStraub, VDiaz-Manera, JdysferlinopathyMagnetic Resonace Imaging (MRI)Miyoshi myopathyLGMDR2limb girdle muscle dystrophyexercisePractice of sports during childhood or adolescence correlates with an earlier onset and more rapidly progressing phenotype in dysferlinopathies. To determine if this correlation relates to greater muscle pathology that persists into adulthood, we investigated the effect of exercise on the degree of muscle fatty replacement measured using muscle MRI. We reviewed pelvic, thigh and leg T1W MRI scans from 160 patients with genetically confirmed dysferlinopathy from the Jain Foundation International clinical outcomes study in dysferlinopathy. Two independent assessors used the Lamminen-Mercuri visual scale to score degree of fat replacement in each muscle. Exercise intensity for each individual was defined as no activity, minimal, moderate, or intensive activity by using metabolic equivalents and patient reported frequency of sports undertaken between the ages of 10 and 18. We used ANCOVA and linear modeling to compare the mean Lamminen-Mercuri score for the pelvis, thigh, and leg between exercise groups, controlling for age at assessment and symptom duration. Intensive exercisers showed greater fatty replacement in the muscles of the pelvis than moderate exercisers, but no significant differences of the thigh or leg. Within the pelvis, Psoas was the muscle most strongly associated with this exercise effect. In patients with a short symptom duration of <15 years there was a trend toward greater fatty replacement in the muscles of the thigh. These findings define key muscles involved in the exercise-phenotype effect that has previously been observed only clinically in dysferlinopathy and support recommendations that pre-symptomatic patients should avoid very intensive exercise.FRONTIERS MEDIA SA2020info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=11743https://ddd.uab.cat/record/252276Frontiers in NeurologyISSN: 16642295reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pauinstname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)Inglésinfo:eu-repo/semantics/openAccessoai:iibsantpau.fundanetsuite.com:p117432026-06-14T12:41:47Z
dc.title.none.fl_str_mv Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
title Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
spellingShingle Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
Moore, U
dysferlinopathy
Magnetic Resonace Imaging (MRI)
Miyoshi myopathy
LGMDR2
limb girdle muscle dystrophy
exercise
title_short Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
title_full Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
title_fullStr Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
title_full_unstemmed Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
title_sort Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
dc.creator.none.fl_str_mv Moore, U
Jacobs, M
Fernandez-Torron, R
Rossello, JL
Smith, FE
James, M
Mayhew, A
Rufibach, L
Carlier, PG
Blamire, AM
Day, JW
Jones, KJ
Bharucha-Goebel, DX
Salort-Campana, E
Pestronk, A
Walter, MC
Paradas, C
Stojkovic, T
Mori-Yoshimura, M
Bravver, E
Pegoraro, E
Mendell, JR
Bushby, K
Straub, V
Diaz-Manera, J
author Moore, U
author_facet Moore, U
Jacobs, M
Fernandez-Torron, R
Rossello, JL
Smith, FE
James, M
Mayhew, A
Rufibach, L
Carlier, PG
Blamire, AM
Day, JW
Jones, KJ
Bharucha-Goebel, DX
Salort-Campana, E
Pestronk, A
Walter, MC
Paradas, C
Stojkovic, T
Mori-Yoshimura, M
Bravver, E
Pegoraro, E
Mendell, JR
Bushby, K
Straub, V
Diaz-Manera, J
author_role author
author2 Jacobs, M
Fernandez-Torron, R
Rossello, JL
Smith, FE
James, M
Mayhew, A
Rufibach, L
Carlier, PG
Blamire, AM
Day, JW
Jones, KJ
Bharucha-Goebel, DX
Salort-Campana, E
Pestronk, A
Walter, MC
Paradas, C
Stojkovic, T
Mori-Yoshimura, M
Bravver, E
Pegoraro, E
Mendell, JR
Bushby, K
Straub, V
Diaz-Manera, J
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv dysferlinopathy
Magnetic Resonace Imaging (MRI)
Miyoshi myopathy
LGMDR2
limb girdle muscle dystrophy
exercise
topic dysferlinopathy
Magnetic Resonace Imaging (MRI)
Miyoshi myopathy
LGMDR2
limb girdle muscle dystrophy
exercise
description Practice of sports during childhood or adolescence correlates with an earlier onset and more rapidly progressing phenotype in dysferlinopathies. To determine if this correlation relates to greater muscle pathology that persists into adulthood, we investigated the effect of exercise on the degree of muscle fatty replacement measured using muscle MRI. We reviewed pelvic, thigh and leg T1W MRI scans from 160 patients with genetically confirmed dysferlinopathy from the Jain Foundation International clinical outcomes study in dysferlinopathy. Two independent assessors used the Lamminen-Mercuri visual scale to score degree of fat replacement in each muscle. Exercise intensity for each individual was defined as no activity, minimal, moderate, or intensive activity by using metabolic equivalents and patient reported frequency of sports undertaken between the ages of 10 and 18. We used ANCOVA and linear modeling to compare the mean Lamminen-Mercuri score for the pelvis, thigh, and leg between exercise groups, controlling for age at assessment and symptom duration. Intensive exercisers showed greater fatty replacement in the muscles of the pelvis than moderate exercisers, but no significant differences of the thigh or leg. Within the pelvis, Psoas was the muscle most strongly associated with this exercise effect. In patients with a short symptom duration of <15 years there was a trend toward greater fatty replacement in the muscles of the thigh. These findings define key muscles involved in the exercise-phenotype effect that has previously been observed only clinically in dysferlinopathy and support recommendations that pre-symptomatic patients should avoid very intensive exercise.
publishDate 2020
dc.date.none.fl_str_mv 2020
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=11743
https://ddd.uab.cat/record/252276
url https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=11743
https://ddd.uab.cat/record/252276
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv FRONTIERS MEDIA SA
publisher.none.fl_str_mv FRONTIERS MEDIA SA
dc.source.none.fl_str_mv Frontiers in Neurology
ISSN: 16642295
reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
instname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
instname_str Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
reponame_str r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
collection r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
repository.name.fl_str_mv
repository.mail.fl_str_mv
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