Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy
Por:
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
Publicada:
16 dic 2020
Resumen:
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.
Filiaciones:
Moore, U:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Jacobs, M:
Childrens Natl Hlth Syst, Div Biostat & Study Methodol, Ctr Translat Sci, Washington, DC USA
George Washington Univ, Pediat Epidemiol & Biostat, Washington, DC USA
Fernandez-Torron, R:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Donostia Univ Hosp, Neuromuscular Area, Biodonostia Hlth Res Inst, Neurol Serv, Donostia San Sebastian, Spain
Rossello, JL:
Univ Autonoma Barcelona, Dept Radiol, Hosp Santa Creu & St Pau, Barcelona, Spain
Smith, FE:
Newcastle Univ, Magnet Resonance Ctr, Translat & Clin Res Inst, Newcastle Upon Tyne, Tyne & Wear, England
James, M:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Mayhew, A:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Rufibach, L:
Jain Fdn, Seattle, WA USA
Carlier, PG:
Univ Paris Saclay, CEA, DRF, Serv Hosp Freder Joliot, Orsay, France
Blamire, AM:
Newcastle Univ, Magnet Resonance Ctr, Translat & Clin Res Inst, Newcastle Upon Tyne, Tyne & Wear, England
Day, JW:
Stanford Univ, Dept Neurol & Neurol Sci, Sch Med, Stanford, CA 94305 USA
Jones, KJ:
Univ Sydney, Childrens Hosp Westmead, Sydney, NSW, Australia
Bharucha-Goebel, DX:
Childrens Natl Hlth Syst, Dept Neurol, Washington, DC USA
NINDS, NIH, Bldg 36,Rm 4D04, Bethesda, MD 20892 USA
Salort-Campana, E:
Hop La Timone, Serv Malad Neuromusculaire, Marseille, France
Hop La Timone, SLA, Marseille, France
Pestronk, A:
Washington Univ, Sch Med, Dept Neurol, St Louis, MO 63110 USA
Walter, MC:
Ludwig Maximilians Univ Munchen, Dept Neurol, Friedrich Baur Inst, Munich, Germany
Paradas, C:
Inst Salud Carlos III, Ctr Biomed Network Res Eurodegenerat Dis, Madrid, Spain
Hosp Univ Virgen del Rocio, Neuromuscular Unit, Dept Neurol, Inst Biomed Sevilla, Seville, Spain
Stojkovic, T:
Sorbonne Univ, Hop Pitie Salpetriere, AP HP, Ctr Reference Malad Neuromusculaires,Inst Myol, Paris, France
Mori-Yoshimura, M:
Natl Ctr Neurol & Psychiat Tokyo, Dept Neurol, Natl Ctr Hosp, Tokyo, Japan
Bravver, E:
Carolinas HealthCare Syst, Carolinas Neuromuscular ALS MDA Ctr, Neurosci Inst, Charlotte, NC USA
Pegoraro, E:
Univ Padua, Dept Neurosci, Padua, Italy
Mendell, JR:
Nationwide Childrens Hosp, Abigail Wexner Res Inst, Columbus, OH USA
Bushby, K:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Straub, V:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Diaz-Manera, J:
Newcastle Univ, John Walton Muscular Dystrophy Res Ctr, Translat & Clin Res Inst, Newcastle Hosp NHS Fdn Trust, Newcastle Upon Tyne, Tyne & Wear, England
Hosp Santa Creu & Sant Pau, Neuromuscular Disorders Unit, Dept Neurol, Barcelona, Spain
Ctr Invest Biomed Red Enfermedades Raras CIBERER, Valencia, Spain
Green Accepted, gold, Green Published
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