{
  "abstract": "Background and objectives Quantitative and operator-independent biomarkers of disease progression are urgently needed in amyotrophic lateral sclerosis (ALS) research. We assess the potential of skeletal muscle MRI as a sensitive and reliable outcome measure for future ALS clinical trials.Methods In this longitudinal cohort study, muscle MRI of head-neck, upper and lower limb regions, alongside clinical and functional assessments, were acquired at three time points over the individual maximum observation period (iMOP) of 1 year in 20 patients with ALS and 16 healthy controls. Quantitative MRI parameters cross-sectional area (CSA), volume (VOL), fat fraction, functional rest muscle area and water T2 (T 2m) were correlated with changes in clinical disease severity (functional rating scales and myometry).Results Among 20 patients with ALS, 17 completed follow-up. Progressive muscle atrophy (CSA, VOL) was observed at hand (rs=0.66), head-neck (partial η²=0.47) and lower-limb level (thighs: η²=0.56, calves: η²=0.54) over iMOP. MRI changes correlated with leg muscle strength (knee extension: r=0.77; plantar flexion: r=0.78), hand grip strength (r=0.71) and functional rating scales (r=0.68).Interpretation Our findings demonstrate the effectiveness of muscle MRI as a sensitive neuroimaging biomarker of disease progression in ALS, highlighting its potential application in clinical trials.",
  "authors": [
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK",
        "Department of Radiology, University Hospital Tulln, Tulln, Austria"
      ],
      "name": "Uros Klickovic"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Luca Zampedri"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK",
        "Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Nick Zafeiropoulos"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Oliver J Ziff"
    },
    {
      "affiliations": [
        "Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Christopher DJ Sinclair"
    },
    {
      "affiliations": [
        "Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Stephen Wastling"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Magdalena Dudziec"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Jodie Allen"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK",
        "Department of Neurology, Medical University of Vienna, Vienna, Austria"
      ],
      "name": "Karin Trimmel"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Robin S Howard"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Andrea Malaspina"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Nikhil Sharma"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Katie CL Sidle"
    },
    {
      "affiliations": [
        "Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Sachit Shah"
    },
    {
      "affiliations": [
        "Department of Radiology, University Hospital Tulln, Tulln, Austria"
      ],
      "name": "Christian Nasel"
    },
    {
      "affiliations": [
        "Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Tarek A Yousry"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Linda Greensmith"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Jasper M Morrow"
    },
    {
      "affiliations": [
        "Neuroradiological Academic Unit, UCL Queen Square Institute of Neurology, London, UK",
        "Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK"
      ],
      "name": "John S Thornton"
    },
    {
      "affiliations": [
        "MND Centre, UCL Queen Square Institute of Neurology, London, UK"
      ],
      "name": "Pietro Fratta"
    }
  ],
  "title": "Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis",
  "uid": "e58214a5-62d3-58e0-a34a-520d36f70ee1"
}