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May 19, 2022Muscle & Nerve

Quantitative muscle analysis in facioscapulohumeral muscular dystrophy usingwhole‐body fat‐referenced MRI: Protocol development, multicenter feasibility, and repeatability

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Authors

PWPer WidholmAAAndré AhlgrenMKMarkus Karlsson

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Overview

Multicenter study demonstrates high repeatability of fat-referenced MRI muscle quantification in facioscapulohumeral muscular dystrophy, highlighting its utility for clinical trials.

Key Points

  • To develop and validate an objective, quantitative whole-body fat-referenced MRI protocol to evaluate skeletal muscle volume and fat composition for multisite clinical trials in facioscapulohumeral muscular dystrophy.
  • Enrolled N=17 patients aged 18 to 65 years with genetically confirmed FSHD1 across six clinical sites.
  • Performed T1-weighted two-point Dixon MRI covering the torso and extremities at two visits 4 to 12 weeks apart.
  • Quantified muscle fat fraction (MFF), muscle fat infiltration (MFI), and lean muscle volume (LMV) across 36 muscles using semi-automatic multi-atlas-based segmentation.
  • Individual muscle within-patient standard deviations ranged from 1.00% to 3.51% for MFF and 0.40% to 1.48% for MFI, with coefficients of variation between 2.7% and 11.7% for LMV.
  • Composite scores across all muscles demonstrated high repeatability, with observed standard deviations of 0.70% for MFF and 0.32% for MFI, and a composite LMV coefficient of variation of 2.0%.

Cite This Study

Widholm et al. (2022) studied this question.

synapsesocial.com/papers/6a83a5e58dcee831255bcaachttps://doi.org/10.1002/mus.27638
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