Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis
Background: Biomarkers of disease progression and outcome measures are still lacking for patients with amyotrophic lateral sclerosis (ALS). Muscle MRI can be a promising candidate to track longitudinal changes and to predict response to the therapy in clinical trials.Objective: Our aim is to apply q...
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2021
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oai:doaj.org-article:90fbf184fe63401a8dc5d30ffe9147262021-11-30T15:38:45ZLongitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis1664-229510.3389/fneur.2021.749736https://doaj.org/article/90fbf184fe63401a8dc5d30ffe9147262021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fneur.2021.749736/fullhttps://doaj.org/toc/1664-2295Background: Biomarkers of disease progression and outcome measures are still lacking for patients with amyotrophic lateral sclerosis (ALS). Muscle MRI can be a promising candidate to track longitudinal changes and to predict response to the therapy in clinical trials.Objective: Our aim is to apply quantitative muscle MRI in the evaluation of disease progression, focusing on thigh and leg muscles of patients with ALS, and to explore the correlation between radiological and clinical scores.Methods: We enrolled newly diagnosed patients with ALS, longitudinally scored using the ALS Functional Rating Scale-Revised (ALSFRS-R), who underwent a 3T muscle MRI protocol including a 6-point Dixon gradient-echo sequence and multi-echo turbo spin echo (TSE) T2-weighted sequence for quantification of fat fraction (FF), cross-sectional area (CSA), and water T2 (wT2). A total of 12 muscles of the thigh and six muscles of the leg were assessed by the manual drawing of 18 regions of interest (ROIs), for each side. A group of 11 age-matched healthy controls (HCs) was enrolled for comparison.Results: 15 patients (M/F 8/7; mean age 62.2 years old, range 29–79) diagnosed with possible (n = 2), probable (n = 12), or definite (n = 1) ALS were enrolled. Eleven patients presented spinal onset, whereas four of them had initial bulbar involvement. All patients performed MRI at T0, nine of them at T1, and seven of them at T2. At baseline, wT2 was significantly elevated in ALS subjects compared to HCs for several muscles of the thigh and mainly for leg muscles. By contrast, FF was elevated in few muscles, and mainly at the level of the thigh. The applied mixed effects model showed that FF increased significantly in the leg muscles over time (mainly in the triceps surae) and that wT2 decreased significantly in line with worsening in the leg subscore of ALSFRS-R, mainly at the leg level and in the anterior and medial compartment of the thigh.Conclusions: Quantitative MRI represents a non-invasive tool that is able to outline the trajectory of pathogenic modifications at the muscle level in ALS. In particular, wT2 was found to be increased early in the clinical history of ALS and also tended to decrease over time, also showing a positive correlation with leg subscore of ALSFRS-R.Matteo PaolettiLuca DiamantiShaun I. MuzicShaun I. MuzicElena BallanteElena BallanteFrancesca SolazzoLia FoppoliXeni DeligianniXeni DeligianniFrancesco SantiniFrancesco SantiniSilvia FiginiNiels BergslandNiels BergslandAnna PichiecchioAnna PichiecchioFrontiers Media S.A.articleALSmuscle MRIqMRIfat fractionedemawT2Neurology. Diseases of the nervous systemRC346-429ENFrontiers in Neurology, Vol 12 (2021) |
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ALS muscle MRI qMRI fat fraction edema wT2 Neurology. Diseases of the nervous system RC346-429 |
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ALS muscle MRI qMRI fat fraction edema wT2 Neurology. Diseases of the nervous system RC346-429 Matteo Paoletti Luca Diamanti Shaun I. Muzic Shaun I. Muzic Elena Ballante Elena Ballante Francesca Solazzo Lia Foppoli Xeni Deligianni Xeni Deligianni Francesco Santini Francesco Santini Silvia Figini Niels Bergsland Niels Bergsland Anna Pichiecchio Anna Pichiecchio Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis |
| description |
Background: Biomarkers of disease progression and outcome measures are still lacking for patients with amyotrophic lateral sclerosis (ALS). Muscle MRI can be a promising candidate to track longitudinal changes and to predict response to the therapy in clinical trials.Objective: Our aim is to apply quantitative muscle MRI in the evaluation of disease progression, focusing on thigh and leg muscles of patients with ALS, and to explore the correlation between radiological and clinical scores.Methods: We enrolled newly diagnosed patients with ALS, longitudinally scored using the ALS Functional Rating Scale-Revised (ALSFRS-R), who underwent a 3T muscle MRI protocol including a 6-point Dixon gradient-echo sequence and multi-echo turbo spin echo (TSE) T2-weighted sequence for quantification of fat fraction (FF), cross-sectional area (CSA), and water T2 (wT2). A total of 12 muscles of the thigh and six muscles of the leg were assessed by the manual drawing of 18 regions of interest (ROIs), for each side. A group of 11 age-matched healthy controls (HCs) was enrolled for comparison.Results: 15 patients (M/F 8/7; mean age 62.2 years old, range 29–79) diagnosed with possible (n = 2), probable (n = 12), or definite (n = 1) ALS were enrolled. Eleven patients presented spinal onset, whereas four of them had initial bulbar involvement. All patients performed MRI at T0, nine of them at T1, and seven of them at T2. At baseline, wT2 was significantly elevated in ALS subjects compared to HCs for several muscles of the thigh and mainly for leg muscles. By contrast, FF was elevated in few muscles, and mainly at the level of the thigh. The applied mixed effects model showed that FF increased significantly in the leg muscles over time (mainly in the triceps surae) and that wT2 decreased significantly in line with worsening in the leg subscore of ALSFRS-R, mainly at the leg level and in the anterior and medial compartment of the thigh.Conclusions: Quantitative MRI represents a non-invasive tool that is able to outline the trajectory of pathogenic modifications at the muscle level in ALS. In particular, wT2 was found to be increased early in the clinical history of ALS and also tended to decrease over time, also showing a positive correlation with leg subscore of ALSFRS-R. |
| format |
article |
| author |
Matteo Paoletti Luca Diamanti Shaun I. Muzic Shaun I. Muzic Elena Ballante Elena Ballante Francesca Solazzo Lia Foppoli Xeni Deligianni Xeni Deligianni Francesco Santini Francesco Santini Silvia Figini Niels Bergsland Niels Bergsland Anna Pichiecchio Anna Pichiecchio |
| author_facet |
Matteo Paoletti Luca Diamanti Shaun I. Muzic Shaun I. Muzic Elena Ballante Elena Ballante Francesca Solazzo Lia Foppoli Xeni Deligianni Xeni Deligianni Francesco Santini Francesco Santini Silvia Figini Niels Bergsland Niels Bergsland Anna Pichiecchio Anna Pichiecchio |
| author_sort |
Matteo Paoletti |
| title |
Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis |
| title_short |
Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis |
| title_full |
Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis |
| title_fullStr |
Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis |
| title_full_unstemmed |
Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis |
| title_sort |
longitudinal quantitative mri evaluation of muscle involvement in amyotrophic lateral sclerosis |
| publisher |
Frontiers Media S.A. |
| publishDate |
2021 |
| url |
https://doaj.org/article/90fbf184fe63401a8dc5d30ffe914726 |
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