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Lookup NU author(s): Dr Ursula Moore, Roberto Fernandez-Torron, Meredith JamesORCiD, Dr Anna Mayhew, Professor Andrew BlamireORCiD, Emerita Professor Katherine Bushby, Professor Volker StraubORCiD, Professor Jordi Diaz ManeraORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© Copyright © 2020 Moore, Jacobs, Fernandez-Torron, LLauger Rossello, Smith, James, Mayhew, Rufibach, Carlier, Blamire, Day, Jones, Bharucha-Goebel, Salort-Campana, Pestronk, Walter, Paradas, Stojkovic, Mori-Yoshimura, Bravver, Pegoraro, Mendell, Bushby, Straub and Diaz-Manera.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.
Author(s): Moore U, Jacobs M, Fernandez-Torron R, LLauger Rossello J, 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, The Jain COS Consortium, Bushby K, Straub V, Diaz-Manera J
Publication type: Article
Publication status: Published
Journal: Frontiers in Neurology
Year: 2020
Volume: 11
Online publication date: 16/12/2020
Acceptance date: 23/11/2020
Date deposited: 09/03/2021
ISSN (electronic): 1664-2295
Publisher: Frontiers Media S.A.
URL: https://doi.org/10.3389/fneur.2020.613446
DOI: 10.3389/fneur.2020.613446
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