Muscle MRI in motor neuron diseases: a systematic review

Kriss, Alexander; Jenkins, Thomas M.

doi:10.1080/21678421.2021.1936062

Cited by 12 publications

(10 citation statements)

References 49 publications

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“…The precise and efficient evaluation and tracking of changes related to the evolution of the disease, beyond the traditional clinical measures (i.e., the revised ALS functional rating scale, ALSFRS-R), is a major difficulty in ALS. Quantitative muscle MRI has been proposed and successfully used to provide biomarkers for tracking disease evolution and, when available, assess response to treatment in neuromuscular disorders (8)(9)(10)(11) and for ALS specifically (12). Longitudinal studies investigating the role of muscle MRI as a biomarker of ALS progression are few and heterogeneous in terms of evaluated MRI studies (1,3,4,13).…”

Section: Introductionmentioning

confidence: 99%

Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis

et al. 2021

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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.

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Section: Introductionmentioning

confidence: 99%

Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis

et al. 2021

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“…To evaluate these new therapeutic options, the development of non-invasive surrogate biomarkers is an important complement to patient-specific outcomes, clinical evaluation, and electrophysiology. Quantitative MRI (qMRI) is a promising non-invasive tool in the evaluation of neuromuscular diseases (NMD) 9 , 10 . Advanced qMRI techniques like Dixon fat-fraction (FF) imaging, quantitative T2 measures or diffusion tensor imaging (DTI) can provide quantitative variables which have shown to be more precise and reliable than semiquantitative rating by visual inspection 11 .…”

Section: Introductionmentioning

confidence: 99%

Quantitative muscle MRI captures early muscle degeneration in calpainopathy

Forsting

Rohm

Froeling

et al. 2022

Sci Rep

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To evaluate differences in qMRI parameters of muscle diffusion tensor imaging (mDTI), fat-fraction (FF) and water T2 time in leg muscles of calpainopathy patients (LGMD R1/D4) compared to healthy controls, to correlate those findings to clinical parameters and to evaluate if qMRI parameters show muscle degeneration in not-yet fatty infiltrated muscles. We evaluated eight thigh and seven calf muscles of 19 calpainopathy patients and 19 healthy matched controls. MRI scans were performed on a 3T MRI including a mDTI, T2 mapping and mDixonquant sequence. Clinical assessment was done with manual muscle testing, patient questionnaires (ACTIVLIM, NSS) as well as gait analysis. Average FF was significantly different in all muscles compared to controls (p < 0.001). In muscles with less than 8% FF a significant increase of FA (p < 0.005) and decrease of RD (p < 0.004) was found in high-risk muscles of calpainopathy patients. Water T2 times were increased within the low- and intermediate-risk muscles (p ≤ 0.045) but not in high-risk muscles (p = 0.062). Clinical assessments correlated significantly with qMRI values: QMFM vs. FF: r = − 0.881, p < 0.001; QMFM versus FA: r = − 0.747, p < 0.001; QMFM versus MD: r = 0.942, p < 0.001. A good correlation of FF and diffusion metrics to clinical assessments was found. Diffusion metrics and T2 values are promising candidates to serve as sensitive early and non-invasive methods to capture early muscle degeneration in non-fat-infiltrated muscles in calpainopathies.

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“…Muscle edema and fat deposition are not unique to IIM and can be seen in denervation changes (including disorders of the motor neurons), neoplasm, infection, and muscle injury (20,(39)(40)(41)(42)(43)(44). Differentiating neurogenic changes from IIMs can sometimes be difficult.…”

Section: Mri Changes In Iimsmentioning

confidence: 99%

“…Differentiating neurogenic changes from IIMs can sometimes be difficult. The overall pattern of muscle involvement and comparing it to “classic” forms of IIMs can be helpful ( 42 ). Muscle MRI can be useful as a clinical tool to identify a pattern (with a sensitivity to detect selective patterns in the rigidity of the spine in relation to the genetic diagnosis reported as 0.9) ( 45 ).…”

Section: Magnetic Resonance Imagingmentioning

confidence: 99%

“…Muscle MRI can be useful as a clinical tool to identify a pattern (with a sensitivity to detect selective patterns in the rigidity of the spine in relation to the genetic diagnosis reported as 0.9) ( 45 ). Furthermore, the identification of muscle “islands” or small areas of muscle tissue with normal signal intensity surrounded by areas with intensity similar to subcutaneous fat on imaging is usually associated with neurogenic etiology ( 42 , 46 ).…”

Section: Magnetic Resonance Imagingmentioning

confidence: 99%

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Imaging biomarkers in the idiopathic inflammatory myopathies

et al. 2023

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Idiopathic inflammatory myopathies (IIMs) are a group of acquired muscle diseases with muscle inflammation, weakness, and other extra-muscular manifestations. IIMs can significantly impact the quality of life, and management of IIMs often requires a multi-disciplinary approach. Imaging biomarkers have become an integral part of the management of IIMs. Magnetic resonance imaging (MRI), muscle ultrasound, electrical impedance myography (EIM), and positron emission tomography (PET) are the most widely used imaging technologies in IIMs. They can help make the diagnosis and assess the burden of muscle damage and treatment response. MRI is the most widely used imaging biomarker of IIMs and can assess a large volume of muscle tissue but is limited by availability and cost. Muscle ultrasound and EIM are easy to administer and can even be performed in the clinical setting, but they need further validation. These technologies may complement muscle strength testing and laboratory studies and provide an objective assessment of muscle health in IIMs. Furthermore, this is a rapidly progressing field, and new advances are going to equip care providers with a better objective assessment of IIMS and eventually improve patient management. This review discusses the current state and future direction of imaging biomarkers in IIMs.

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Muscle MRI in motor neuron diseases: a systematic review

Cited by 12 publications

References 49 publications

Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis

Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis

Quantitative muscle MRI captures early muscle degeneration in calpainopathy

Imaging biomarkers in the idiopathic inflammatory myopathies

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