A new imaging tool to assess FSH

Facioscapulohumeral muscular dystrophy (FSH) has recently entered the era of therapeutic trials with several innovative molecules in clinical development. An international consortium of researchers has developed innovative techniques and algorithms to study the topographic and evolutionary aspects of muscle degeneration.

  • Seventeen adults with FSH participated in this study, which consisted of whole-body muscle imaging (WB-MSK-MRI) at intervals of several weeks.
  • Relevant endpoints were fat infiltration volume (MFI), muscle fat fraction (MFF), compared to clinical severity items.

This type of imaging could prove to be a very interesting biomarker for the evolutionary follow-up of patients but especially for therapeutic trials.

 

Quantitative Muscle Analysis in FSHD Using Whole-Body Fat-Referenced MRI: Composite Scores for Longitudinal and Cross-Sectional Analysis. Mellion ML, Widholm P, Karlsson M, et al. Neurology. 2022 (Juin).