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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Frontiers Media S.A.
2020-12-01
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Series: | Frontiers in Neurology |
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Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2020.613446/full |
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doaj-945d00c7ea94451cb594a35eeef06617 |
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record_format |
Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ursula Moore Marni Jacobs Marni Jacobs Roberto Fernandez-Torron Roberto Fernandez-Torron Jaume LLauger Rossello Fiona E. Smith Meredith James Anna Mayhew Laura Rufibach Pierre G. Carlier Andrew M. Blamire John W. Day Kristi J. Jones Diana X. Bharucha-Goebel Diana X. Bharucha-Goebel Emmanuelle Salort-Campana Alan Pestronk Maggie C. Walter Carmen Paradas Carmen Paradas Tanya Stojkovic Madoka Mori-Yoshimura Elena Bravver Elena Pegoraro Jerry R. Mendell Kate Bushby Volker Straub Jordi Diaz-Manera Jordi Diaz-Manera Jordi Diaz-Manera |
spellingShingle |
Ursula Moore Marni Jacobs Marni Jacobs Roberto Fernandez-Torron Roberto Fernandez-Torron Jaume LLauger Rossello Fiona E. Smith Meredith James Anna Mayhew Laura Rufibach Pierre G. Carlier Andrew M. Blamire John W. Day Kristi J. Jones Diana X. Bharucha-Goebel Diana X. Bharucha-Goebel Emmanuelle Salort-Campana Alan Pestronk Maggie C. Walter Carmen Paradas Carmen Paradas Tanya Stojkovic Madoka Mori-Yoshimura Elena Bravver Elena Pegoraro Jerry R. Mendell Kate Bushby Volker Straub Jordi Diaz-Manera Jordi Diaz-Manera Jordi Diaz-Manera Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy Frontiers in Neurology dysferlinopathy Magnetic Resonace Imaging (MRI) Miyoshi myopathy LGMDR2 limb girdle muscle dystrophy exercise |
author_facet |
Ursula Moore Marni Jacobs Marni Jacobs Roberto Fernandez-Torron Roberto Fernandez-Torron Jaume LLauger Rossello Fiona E. Smith Meredith James Anna Mayhew Laura Rufibach Pierre G. Carlier Andrew M. Blamire John W. Day Kristi J. Jones Diana X. Bharucha-Goebel Diana X. Bharucha-Goebel Emmanuelle Salort-Campana Alan Pestronk Maggie C. Walter Carmen Paradas Carmen Paradas Tanya Stojkovic Madoka Mori-Yoshimura Elena Bravver Elena Pegoraro Jerry R. Mendell Kate Bushby Volker Straub Jordi Diaz-Manera Jordi Diaz-Manera Jordi Diaz-Manera |
author_sort |
Ursula Moore |
title |
Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy |
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 |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Neurology |
issn |
1664-2295 |
publishDate |
2020-12-01 |
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. |
topic |
dysferlinopathy Magnetic Resonace Imaging (MRI) Miyoshi myopathy LGMDR2 limb girdle muscle dystrophy exercise |
url |
https://www.frontiersin.org/articles/10.3389/fneur.2020.613446/full |
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doaj-945d00c7ea94451cb594a35eeef066172020-12-16T15:31:14ZengFrontiers Media S.A.Frontiers in Neurology1664-22952020-12-011110.3389/fneur.2020.613446613446Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With DysferlinopathyUrsula Moore0Marni Jacobs1Marni Jacobs2Roberto Fernandez-Torron3Roberto Fernandez-Torron4Jaume LLauger Rossello5Fiona E. Smith6Meredith James7Anna Mayhew8Laura Rufibach9Pierre G. Carlier10Andrew M. Blamire11John W. Day12Kristi J. Jones13Diana X. Bharucha-Goebel14Diana X. Bharucha-Goebel15Emmanuelle Salort-Campana16Alan Pestronk17Maggie C. Walter18Carmen Paradas19Carmen Paradas20Tanya Stojkovic21Madoka Mori-Yoshimura22Elena Bravver23Elena Pegoraro24Jerry R. Mendell25Kate Bushby26Volker Straub27Jordi Diaz-Manera28Jordi Diaz-Manera29Jordi Diaz-Manera30The John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United KingdomDivision of Biostatistics and Study Methodology, Center for Translational Science, Children's National Health System, Washington, DC, United StatesPediatrics, Epidemiology and Biostatistics, George Washington University, Washington, DC, United StatesThe John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United KingdomNeuromuscular Area, Biodonostia Health Research Institute, Neurology Service, Donostia University Hospital, Donostia-San Sebastian, SpainRadiology Department, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, SpainMagnetic Resonance Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United KingdomThe John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United KingdomThe John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United KingdomJain Foundation, Newcastle upon Tyne, Seattle, WA, United StatesUniversité Paris-Saclay, CEA, DRF, Service Hospitalier Frederic Joliot, Orsay, FranceMagnetic Resonance Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United KingdomDepartment of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA, United States0The Children's Hospital at Westmead, The University of Sydney, Sydney, NSW, Australia1Department of Neurology Children's National Health System, Washington, DC, United States2National Institutes of Health (NINDS), Bethesda, MD, United States3Service des maladies neuromusculaire et de la SLA, Hôpital de La Timone, Marseille, France4Department of Neurology Washington University School of Medicine, St. Louis, MO, United States5Department of Neurology, Friedrich-Baur-Institute, Ludwig-Maximilians-University of Munich, Munich, Germany6Center for Biomedical Network Research on Eurodegenerative Diseases, Instituto de Salud Carlos III, Madrid, Spain7Neuromuscular Unit, Department of Neurology, Hospital U. Virgen del Rocío/Instituto de Biomedicina de Sevilla, Sevilla, Spain8Centre de référence des maladies neuromusculaires, Institut de Myologie, AP-HP, Sorbonne Université, Hôpital Pitié-Salpêtrière, Paris, France9Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry Tokyo, Tokyo, Japan0Carolinas Neuromuscular/ALS-MDA Center, Neuroscience Institute, Carolinas HealthCare System, Charlotte, NC, United States1Department of Neuroscience, University of Padova, Padova, Italy2The Abigail Wexner Research Institute at Nationwide Children's Hospital, Columbus, OH, United StatesThe John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United KingdomThe John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United KingdomThe John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom3Neuromuscular disorders Unit, Department of Neurology, Hospital de la Santa Creu I Sant Pau, Barcelona, Spain4Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER), Valencia, SpainPractice 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.https://www.frontiersin.org/articles/10.3389/fneur.2020.613446/fulldysferlinopathyMagnetic Resonace Imaging (MRI)Miyoshi myopathyLGMDR2limb girdle muscle dystrophyexercise |