{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T09:24:32Z","timestamp":1789982672497,"version":"4.0.1"},"reference-count":36,"publisher":"BMJ","issue":"3","funder":[{"name":"Ryder Briggs\/Neurocare Charitable Trust"},{"DOI":"10.13039\/501100000374","name":"British Medical Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000374","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To assess clinical, electrophysiological and whole-body muscle MRI measurements of progression in patients with motor neuron disease (MND), as tools for future clinical trials, and to probe pathophysiological mechanisms in vivo.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A prospective, longitudinal, observational, clinicoelectrophysiological and radiological cohort study was performed. Twenty-nine patients with MND and 22 age-matched and gender-matched healthy controls were assessed with clinical measures, electrophysiological motor unit number index (MUNIX) and T2-weighted whole-body muscle MRI, at first clinical presentation and 4\u2009months later. Between-group differences and associations were assessed using age-adjusted and gender-adjusted multivariable regression models. Within-subject longitudinal changes were assessed using paired t-tests. Patterns of disease spread were modelled using mixed-effects multivariable regression, assessing associations between muscle relative T2 signal and anatomical adjacency to site of clinical onset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with MND had 30% higher relative T2 muscle signal than controls at baseline (all regions mean, 95%\u2009CI 15% to 45%, p&lt;0.001). Higher T2 signal was associated with greater overall disability (coefficient \u22120.009, 95%\u2009CI \u22120.017 to \u20130.001, p=0.023) and with clinical weakness and lower MUNIX in multiple individual muscles. Relative T2 signal in bilateral tibialis anterior increased over 4\u2009months in patients with MND (right: 10.2%, 95%\u2009CI 2.0% to 18.4%, p=0.017; left: 14.1%, 95%\u2009CI 3.4% to 24.9%, p=0.013). Anatomically, contiguous disease spread on MRI was not apparent in this model.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Whole-body muscle MRI offers a new approach to objective assessment of denervation over short timescales in MND and enables investigation of patterns of disease spread in vivo. Muscles inaccessible to conventional clinical and electrophysiological assessment may be investigated using this methodology.<\/jats:p><\/jats:sec>","DOI":"10.1136\/jnnp-2017-316744","type":"journal-article","created":{"date-parts":[[2017,10,31]],"date-time":"2017-10-31T16:16:29Z","timestamp":1509466589000},"page":"248-255","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":49,"title":["Imaging muscle as a potential biomarker of denervation in motor neuron disease"],"prefix":"10.1136","volume":"89","author":[{"given":"Thomas M","family":"Jenkins","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James J P","family":"Alix","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"David","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eilish","family":"Pearson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D Ganesh","family":"Rao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nigel","family":"Hoggard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eoghan","family":"O\u2019Brien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen","family":"Baster","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Bradburn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Bigley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher J","family":"McDermott","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iain D","family":"Wilkinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela J","family":"Shaw","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2017,10,31]]},"reference":[{"key":"2020050722291856000_89.3.248.1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70293-X"},{"key":"2020050722291856000_89.3.248.2","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1186\/s12883-016-0672-6","article-title":"The value of magnetic resonance imaging as a biomarker for amyotrophic lateral sclerosis: a systematic review","volume":"16","author":"Grolez","year":"2016","journal-title":"BMC Neurol"},{"key":"2020050722291856000_89.3.248.3","doi-asserted-by":"publisher","DOI":"10.1007\/s00256-007-0409-0"},{"key":"2020050722291856000_89.3.248.4","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410250508"},{"key":"2020050722291856000_89.3.248.5","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1212\/WNL.51.1.110","article-title":"Magnetic resonance imaging of muscle in amyotrophic lateral sclerosis","volume":"51","author":"Bryan","year":"1998","journal-title":"Neurology"},{"key":"2020050722291856000_89.3.248.6","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1002\/mus.24630","article-title":"Magnetic resonance imaging abnormalities of peripheral nerve and muscle are common in amyotrophic lateral sclerosis and share features with multifocal motor neuropathy","volume":"52","author":"Staff","year":"2015","journal-title":"Muscle Nerve"},{"key":"2020050722291856000_89.3.248.7","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1148\/radiol.2015150559","article-title":"MR imaging of brachial plexus and limb-girdle muscles in patients with amyotrophic lateral sclerosis","volume":"279","author":"Gerevini","year":"2016","journal-title":"Radiology"},{"key":"2020050722291856000_89.3.248.8","doi-asserted-by":"publisher","DOI":"10.1080\/146608200300079536"},{"key":"2020050722291856000_89.3.248.9","first-page":"1071","article-title":"Regression models for prognostic prediction: advantages, problems, and suggested solutions","volume":"69","author":"Harrell","year":"1985","journal-title":"Cancer Treat Rep"},{"key":"2020050722291856000_89.3.248.10","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(99)00210-5"},{"key":"2020050722291856000_89.3.248.11","unstructured":"Medical research council. 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