Water T2 could predict functional decline in patients with dysferlinopathy

Background: Water T2 (T2) mapping is increasingly being used in muscular dystrophies to assess active muscle damage. It has been suggested as a surrogate outcome measure for clinical trials. Here, we investigated the prognostic utility of T2 to identify changes in muscle function over time in limb g...

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Detalles Bibliográficos
Autores: Moore, Ursula|||0000-0002-0643-5596, Caldas de A. Araújo, Ericky|||0000-0002-1352-0879, Reyngoudt, Harmen|||0000-0002-0777-2711, Gordish-Dressman, Heather|||0000-0002-2330-5427, Smith, Fiona E., Wilson, Ian, James, Meredith K.|||0000-0001-8078-7744, Mayhew, Anna G.|||0000-0001-7085-9011, Rufibach, Laura E.|||0000-0001-8208-0574, Day, John W.|||0000-0002-0086-9529, Jones, Kristi J., Bharucha-Goebel, Diana|||0000-0002-7437-0982, Salort-Campana, Emmanuelle|||0000-0002-1846-3017, Pestronk, Alan|||0000-0002-8991-5770, Walter, Maggie C.|||0000-0002-7611-4827, Paradas, Carmen|||0000-0002-6917-2236, Stojkovic, Tanya|||0000-0002-4054-2838, Mori-Yoshimura, Madoka|||0000-0003-4312-9114, Bravver, Elena, Pegoraro, Elena|||0000-0002-7740-4156, Mendell, Jerry R.|||0000-0002-2691-0200, Bushby, Kate|||0000-0001-8818-4008, Blamire, Andrew|||0000-0002-8749-1257, Straub, Volker|||0000-0001-9046-3540, Carlier, Pierre G.|||0000-0002-2315-6927, Diaz-Manera, Jordi|||0000-0003-2941-7988
Tipo de recurso: artículo
Fecha de publicación:2022
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:284169
Acceso en línea:https://ddd.uab.cat/record/284169
https://dx.doi.org/urn:doi:10.1002/jcsm.13063
Access Level:acceso abierto
Palabra clave:Magnetic resonance imaging
Water T2
Limb girdle muscular dystrophy
Limb girdle muscular dystrophy R2
Limb girdle muscular dystrophy 2B
Descripción
Sumario:Background: Water T2 (T2) mapping is increasingly being used in muscular dystrophies to assess active muscle damage. It has been suggested as a surrogate outcome measure for clinical trials. Here, we investigated the prognostic utility of T2 to identify changes in muscle function over time in limb girdle muscular dystrophies. Methods: Patients with genetically confirmed dysferlinopathy were assessed as part of the Jain Foundation Clinical Outcomes Study in dysferlinopathy. The cohort included 18 patients from two sites, both equipped with 3-tesla magnetic resonance imaging (MRI) systems from the same vendor. T2 value was defined as higher or lower than the median in each muscle bilaterally. The degree of deterioration on four functional tests over 3 years was assessed in a linear model against covariates of high or low T2 at baseline, age, disease duration, and baseline function. Results: A higher T2 at baseline significantly correlated with a greater decline on functional tests in 21 out of 35 muscles and was never associated with slower decline. Higher baseline T2 in adductor magnus, vastus intermedius, vastus lateralis, and vastus medialis were the most sensitive, being associated bilaterally with greater decline in multiple timed tests. Patients with a higher than median baseline T2 (.

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