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Open Access 01-03-2021 | Muscular Dystrophy | Original Communication

Quantitative ¹H and ²³Na muscle MRI in Facioscapulohumeral muscular dystrophy patients

Authors: Teresa Gerhalter, Benjamin Marty, Lena V. Gast, Katharina Porzelt, Rafael Heiss, Michael Uder, Stefan Schwab, Pierre G. Carlier, Armin M. Nagel, Matthias Türk

Published in: Journal of Neurology | Issue 3/2021

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 T₂ (wT₂), water T₁ (wT₁), tissue sodium concentration (TSC), and intracellular-weighted 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 wT₂) and ion homeostasis (TSC, IR, TQF) were increased in muscles of FSHD patients. Muscle groups with FF > 10% had higher wT₂, wT₁, TSC, IR, and TQF values than muscles with FF < 10%. Muscles with FF < 10% resembled muscles of healthy controls for these MRI disease activity measures. However, wT₁ was increased in few muscles without fat replacement. Furthermore, few STIR-negative muscles (n = 11/76) exhibited increased wT₁, TSC, IR or TQF. Increased wT₁ as well as ²³Na signals were also present in muscles with normal wT₂. Muscle strength was related to the mean FF and all imaging biomarkers of tibialis anterior except wT₂ were correlated with dorsal flexion.

Conclusion

The newly evaluated imaging biomarkers related with water mobility (wT₁) and ion homeostasis (TSC, IR, TQF) showed different patterns compared to the established markers like FF in muscles of FSHD patients. These quantitative biomarkers could thus contain valuable complementary information for the early characterization of disease progression.

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Title: Quantitative 1H and 23Na muscle MRI in Facioscapulohumeral muscular dystrophy patients
Authors: Teresa Gerhalter
Benjamin Marty
Lena V. Gast
Katharina Porzelt
Rafael Heiss
Michael Uder
Stefan Schwab
Pierre G. Carlier
Armin M. Nagel
Matthias Türk
Publication date: 01-03-2021
Publisher: Springer Berlin Heidelberg
Keywords: Muscular Dystrophy
Muscular Dystrophy
Published in: Journal of Neurology / Issue 3/2021
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-020-10254-2

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Springer Medicine

Quantitative ¹H and ²³Na muscle MRI in Facioscapulohumeral muscular dystrophy patients

Abstract

Objective

Methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Objective

Methods

Results

Conclusion

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