RT Journal Article
T1 Muscle imaging in laminopathies: Synthesis study identifies meaningful muscles for follow-up.
A1 GóMez-Andrés, David
A1 Díaz-Manera, Jordi
A1 Alejaldre, Aida
A1 Pulido-Valdeolivas, Irene
A1 GonzáLez-Mera, Laura
A1 Olivé, Montse
A1 Vilchez, Juan José
A1 De Munain, Adolfo LóPez
A1 Paradas, Carmen
A1 Muelas, Nuria
A1 SáNchez-MontáÑez, Ángel
A1 Alonso-Jimenez, Alicia
A1 De la Banda, Marta Gómez García
A1 Dabaj, Ivana
A1 Bonne, Gisèle
A1 Munell, Francina
A1 Carlier, Robert Y
A1 Quijano-Roy, Susana
K1 LMNA, machine learning, magnetic resonance
K1 biomarker
K1 imaging
K1 laminopathy
AB Particular fibroadipose infiltration patterns have been recently described by muscle imaging in congenital and later onset forms of LMNA-related muscular dystrophies (LMNA-RD). Scores for fibroadipose infiltration of 23 lower limb muscles in 34 patients with LMNA-RD were collected from heat maps of 2 previous studies. Scoring systems were homogenized. Relationships between muscle infiltration and disease duration and age of onset were modeled with random forests. The pattern of infiltration differs according to disease duration but not to age of disease onset. The muscles whose progression best predicts disease duration were semitendinosus, biceps femoris long head, gluteus medius, and semimembranosus. In LMNA-RD, our synthetic analysis of lower limb muscle infiltration did not find major differences between forms with different ages of onset but allowed the identification of muscles with characteristic infiltration during disease progression. Monitoring of these specific muscles by quantitative MRI may provide useful imaging biomarkers in LMNA-RD. Muscle Nerve 58:812-817, 2018.
YR 2018
FD 2018-11-18
LK http://hdl.handle.net/10668/12785
UL http://hdl.handle.net/10668/12785
LA en
DS RISalud
RD Apr 12, 2025