Quantitative 1 H and 23 Na muscle MRI in Facioscapulohumeral muscular dystrophy patients
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ORIGINAL COMMUNICATION
Quantitative 1H and 23Na muscle MRI in Facioscapulohumeral muscular dystrophy patients Teresa Gerhalter1 · Benjamin Marty2,3 · Lena V. Gast1,6 · Katharina Porzelt4 · Rafael Heiss1 · Michael Uder1 · Stefan Schwab4 · Pierre G. Carlier2,3 · Armin M. Nagel1,5,6 · Matthias Türk4 Received: 19 August 2020 / Revised: 29 September 2020 / Accepted: 1 October 2020 © The Author(s) 2020
Abstract Objective Our aim was to assess the role of quantitative 1H and 23Na MRI methods in providing imaging biomarkers of disease activity and severity in patients with Facioscapulohumeral muscular dystrophy (FSHD). Methods We imaged the lower leg muscles of 19 FSHD patients and 12 controls with a multimodal MRI protocol to obtain STIR-T2w images, fat fraction (FF), water T 2 (wT2), water T1 (wT1), tissue sodium concentration (TSC), and intracellularweighted sodium signal (inversion recovery (IR) and triple quantum filter (TQF) sequence). In addition, the FSHD patients underwent muscle strength testing. Results Imaging biomarkers related with water mobility (wT1 and w T2) and ion homeostasis (TSC, IR, TQF) were increased in muscles of FSHD patients. Muscle groups with FF > 10% had higher w T2, wT1, TSC, IR, and TQF values than muscles with FF
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