Five‐year follow‐up study on quantitative muscle magnetic resonance imaging in facioscapulohumeral muscular dystrophy: The link to clinical outcome

Autor: Sanne C.C. Vincenten, Karlien Mul, Daniël vanAs, Julia J. Jansen, Linda Heskamp, Arend Heerschap, Baziel G.M. vanEngelen, Nicol C. Voermans
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Journal of Cachexia, Sarcopenia and Muscle, Vol 14, Iss 4, Pp 1695-1706 (2023)
Druh dokumentu: article
ISSN: 2190-6009
2190-5991
DOI: 10.1002/jcsm.13250
Popis: Abstract Background It is unclear how changes in quantitative muscle magnetic resonance imaging (MRI) relate to changes in clinical outcome in facioscapulohumeral muscular dystrophy (FSHD), although this information is crucial for optimal use of MRI as imaging biomarker in trials. We therefore assessed muscle MRI and clinical outcome measures in a large longitudinal prospective cohort study. Methods All patients were assessed by MRI at baseline and at 5‐year follow‐up, employing 2pt‐Dixon and turbo inversion recovery magnitude (TIRM) sequences, after which fat fraction and TIRM positivity of 19 leg muscles were determined bilaterally. The MRI compound score (CoS) was defined as the mean fat fraction of all muscles weighted for cross‐sectional area. Clinical outcome measures included the Ricci‐score, FSHD clinical score (FSHD‐CS), MRC sumscore (MRC‐SS), and motor‐function‐measure (MFM). Results We included 105 FSHD patients [mean age 54 ± 14 years, median Ricci‐score 7 (range 0–10)]. The median change over 5 years' time in the MRI‐CoS was 2.0% (range −4.6 to +12.1; P
Databáze: Directory of Open Access Journals
Externí odkaz: