Longitudinal MRI quantification of muscle degeneration in Duchenne muscular dystrophy

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Longitudinal MRI quantification of muscle degeneration in Duchenne muscular dystrophy

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Title: Longitudinal MRI quantification of muscle degeneration in Duchenne muscular dystrophy
Author: Godi, Claudia; Ambrosi, Alessandro; Nicastro, Francesca; Previtali, Stefano C.; Santarosa, Corrado; Napolitano, Sara; Iadanza, Antonella; Scarlato, Marina; Natali Sora, Maria Grazia; Tettamanti, Andrea; Gerevini, Simonetta; Cicalese, Maria Pia; Sitzia, Clementina; Venturini, Massimo; Falini, Andrea; Gatti, Roberto; Ciceri, Fabio; Cossu, Giulio; Torrente, Yvan; Politi, Letterio S.

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Citation: Godi, C., A. Ambrosi, F. Nicastro, S. C. Previtali, C. Santarosa, S. Napolitano, A. Iadanza, et al. 2016. “Longitudinal MRI quantification of muscle degeneration in Duchenne muscular dystrophy.” Annals of Clinical and Translational Neurology 3 (8): 607-622. doi:10.1002/acn3.319. http://dx.doi.org/10.1002/acn3.319.
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Abstract: Abstract Objective: The aim of this study was to evaluate the usefulness of magnetic resonance imaging (MRI) in detecting the progression of Duchenne muscular dystrophy (DMD) by quantification of fat infiltration (FI) and muscle volume index (MVI, a residual‐to‐total muscle volume ratio). Methods: Twenty‐six patients (baseline age: 5–12 years) with genetically proven DMD were longitudinally analyzed with lower limb 3T MRI, force measurements, and functional tests (Gowers, 10‐m time, North Star Ambulatory Assessment, 6‐min walking test). Five age‐matched controls were also examined, with a total of 85 MRI studies. Semiquantitative (scores) and quantitative MRI (qMRI) analyses (signal intensity ratio – SIR, lower limb MVI, and individual muscle MVI) were carried out. Permutation and regression analyses according to both age and functional test‐outcomes were calculated. Age‐related quantitative reference curves of SIRs and MVIs were generated. Results: FI was present on glutei and adductor magnus in all patients since the age of 5, with a proximal‐to‐distal progression and selective sparing of sartorius and gracilis. Patients' qMRI measures were significantly different from controls' and among age classes. qMRI were more sensitive than force measurements and functional tests in assessing disease progression, allowing quantification also after loss of ambulation. Age‐related curves with percentile values were calculated for SIRs and MVIs, to provide a reference background for future experimental therapy trials. SIRs and MVIs significantly correlated with all clinical measures, and could reliably predict functional outcomes and loss of ambulation. Interpretations qMRI‐based indexes are sensitive measures that can track the progression of DMD and represent a valuable tool for follow‐up and clinical studies.
Published Version: doi:10.1002/acn3.319
Other Sources: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4999593/pdf/
Terms of Use: This article is made available under the terms and conditions applicable to Other Posted Material, as set forth at http://nrs.harvard.edu/urn-3:HUL.InstRepos:dash.current.terms-of-use#LAA
Citable link to this page: http://nrs.harvard.edu/urn-3:HUL.InstRepos:29407681
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