Dataset Information

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

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?1 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.

SUBMITTER: Gerhalter T

PROVIDER: S-EPMC7914168 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature

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Publications

Quantitative <sup>1</sup>H and <sup>23</sup>Na muscle MRI in Facioscapulohumeral muscular dystrophy patients.

Gerhalter Teresa T Marty Benjamin B Gast Lena V LV Porzelt Katharina K Heiss Rafael R Uder Michael M Schwab Stefan S Carlier Pierre G PG Nagel Armin M AM Türk Matthias M

Journal of neurology 20201012 3

<h4>Objective</h4>Our aim was to assess the role of quantitative <sup>1</sup>H and <sup>23</sup>Na MRI methods in providing imaging biomarkers of disease activity and severity in patients with Facioscapulohumeral muscular dystrophy (FSHD).<h4>Methods</h4>We imaged the lower leg muscles of 19 FSHD patients and 12 controls with a multimodal MRI protocol to obtain STIR-T<sub>2</sub>w images, fat fraction (FF), water T<sub>2</sub> (wT<sub>2</sub>), water T<sub>1</sub> (wT<sub>1</sub>), tissue sodium ...[more]

PMID: 33047224

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Project description:BackgroundFacioscapulohumeral muscular dystrophy (FSHD) is a patchy and slowly progressive disease of skeletal muscle. For MRI to be a useful biomarker in an FSHD clinical trial, it should reliably detect changes over relatively short time-intervals (~ 1 year). We hypothesized that fatty change over the study course would be most likely in muscles already demonstrating disease progression, and that the degree of MRI burden would be correlated with function.MethodsWe studied 36 patients with FSHD and lower-extremity weakness at baseline. Thirty-two patients returned in our 12-month longitudinal observational study. We analyzed DIXON MRI images of 16 lower-extremity muscles in each patient and compared them to quantitative strength measurement and ambulatory functional outcome measures.ResultsThere was a small shift to higher fat fractions in the summed muscle data for each patient, however individual muscles demonstrated much larger magnitudes of change. The greatest increase in fat fraction was observed in muscles having an intermediate fat replacement at baseline, with minimally (baseline fat fraction < 0.10) or severely (> 0.70) affected muscles less likely to progress. Functional outcome measures did not demonstrate marked change over the interval; however, overall MRI disease burden was correlated with functional outcome measures. Direct comparison of the tibialis anterior (TA) fat fraction and quantitative strength measurement showed a sigmoidal relationship, with steepest drop being when the muscle gets more than ~ 20% fatty replaced.ConclusionsAssessing MRI changes in 16 lower-extremity muscles across 1 year demonstrated that those muscles having an intermediate baseline fat fraction were more likely to progress. Ambulatory functional outcome measures are generally related to overall muscle MRI burden but remain unchanged in the short term. Quantitative strength measurement of the TA showed a steep loss of strength when more fatty infiltration is present suggesting that MRI may be preferable for following incremental change or modulation with drug therapy.

Dataset Information

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

Objective

Methods

Results

Conclusion

Publications

Quantitative <sup>1</sup>H and <sup>23</sup>Na muscle MRI in Facioscapulohumeral muscular dystrophy patients.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets

Dataset Information

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

Objective

Methods

Results

Conclusion

Publications

Quantitative <sup>1</sup>H and <sup>23</sup>Na muscle MRI in Facioscapulohumeral muscular dystrophy patients.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets

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