RT Journal Article
T1 Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy.
A1 Moore, Ursula
A1 Jacobs, Marni
A1 Fernandez-Torron, Roberto
A1 LLauger Rossello, Jaume
A1 Smith, Fiona E
A1 James, Meredith
A1 Mayhew, Anna
A1 Rufibach, Laura
A1 Carlier, Pierre G
A1 Blamire, Andrew M
A1 Day, John W
A1 Jones, Kristi J
A1 Bharucha-Goebel, Diana X
A1 Salort-Campana, Emmanuelle
A1 Pestronk, Alan
A1 Walter, Maggie C
A1 Paradas, Carmen
A1 Stojkovic, Tanya
A1 Mori-Yoshimura, Madoka
A1 Bravver, Elena
A1 Pegoraro, Elena
A1 Mendell, Jerry R
A1 Bushby, Kate
A1 Straub, Volker
A1 Diaz-Manera, Jordi
K1 LGMDR2
K1 Magnetic Resonace Imaging (MRI)
K1 Miyoshi myopathy
K1 dysferlinopathy
K1 exercise
K1 limb girdle muscle dystrophy
AB 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
SN 1664-2295
YR 2020
FD 2020-12-16
LK https://hdl.handle.net/10668/27384
UL https://hdl.handle.net/10668/27384
LA en
DS RISalud
RD Apr 8, 2025