{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T18:32:19Z","timestamp":1791052339623,"version":"4.1.0"},"reference-count":40,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2024,9,29]],"date-time":"2024-09-29T00:00:00Z","timestamp":1727568000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/501100022572","name":"NIHR Sheffield Biomedical Research Centre","doi-asserted-by":"publisher","award":["NIHR 203321"],"award-info":[{"award-number":["NIHR 203321"]}],"id":[{"id":"10.13039\/501100022572","id-type":"DOI","asserted-by":"publisher"}]},{"name":"AMBRoSIA Biosampling Programme","award":["972-797"],"award-info":[{"award-number":["972-797"]}]},{"name":"MNDA (Sheffield Care and Research Centre for Motor Neuron Disorders","award":["764-780"],"award-info":[{"award-number":["764-780"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,12,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Introduction<\/jats:title>\n                  <jats:p>Motor neuron disease (MND) is a devastating neurodegenerative disease characterized by progressive muscle weakness.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Sources of data<\/jats:title>\n                  <jats:p>PubMed, MEDLINE, and Cochrane databases were searched for articles to March 2024. Searches involved the terms \u2018motor neuron disease\u2019 or \u2018amyotrophic lateral sclerosis\u2019 and \u2018epidemiology\u2019, \u2018diagnosis\u2019, \u2018clinical\u2019, \u2018genetic\u2019, \u2018management\u2019, \u2018treatment\u2019, or \u2018trial\u2019.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Areas of agreement<\/jats:title>\n                  <jats:p>Evidence-based management involves riluzole, multidisciplinary care, provision of noninvasive ventilation and gastrostomy, and symptomatic treatments. Tofersen should be offered to treat SOD1-MND.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Areas of controversy<\/jats:title>\n                  <jats:p>Edaravone and Relyvrio are approved treatments in the USA, but insufficient evidence was found to support approval in the UK and Europe.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Growing points<\/jats:title>\n                  <jats:p>The discovery of neurofilaments as MND biomarkers, growth of platform trials and development of novel therapies provide optimism for more powerful neuroprotective therapies.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Areas timely for developing research<\/jats:title>\n                  <jats:p>Further work should focus on the elucidation of environmental causes of MND, gene\u2013environment interactions, and advanced cellular models of disease.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/bmb\/ldae010","type":"journal-article","created":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T00:40:58Z","timestamp":1727656858000},"page":"4-15","source":"Crossref","is-referenced-by-count":9,"title":["New developments in the diagnosis and management of motor neuron disease"],"prefix":"10.1093","volume":"152","author":[{"given":"David","family":"O\u2019Brien","sequence":"first","affiliation":[{"name":"Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield , 285 Glossop Road, Sheffield S10 2HQ,","place":["United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8925-2567","authenticated-orcid":false,"given":"Pamela J","family":"Shaw","sequence":"additional","affiliation":[{"name":"Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield , 285 Glossop Road, Sheffield S10 2HQ,","place":["United 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