Objective: To summarize applications of muscle magnetic resonance imaging (MRI) in cross-sectional assessment and longitudinal monitoring of motor neuron diseases and evaluate associations with clinical assessment techniques. Methods: PubMed and Scopus were searched for research published up to May 2021 relating to muscle MRI in motor neuron diseases, according to predefined inclusion and exclusion criteria. Studies were systematically appraised for bias and data were extracted for discussion. Results: Twenty-eight papers met inclusion criteria. The studies assessed muscle T1-and T2-weighted signal, diffusion, muscle volume, and fat infiltration, employing quantitative, qualitative, and semi-quantitative approaches. Various regions of interest were considered; changes in thigh and calf muscles were most frequently reported. Preliminary evidence of concordance between clinical and radiological findings and utility as an objective longitudinal biomarker is emerging. Conclusion: Muscle MRI appears a promising objective, versatile, and practical biomarker to assess motor neuron diseases.
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