O’Brien, D. and Shaw, P.J. orcid.org/0000-0002-8925-2567 (2024) New developments in the diagnosis and management of motor neuron disease. British Medical Bulletin, 152 (1). pp. 4-15. ISSN 0007-1420
Abstract
Introduction
Motor neuron disease (MND) is a devastating neurodegenerative disease characterized by progressive muscle weakness.
Sources of data
PubMed, MEDLINE, and Cochrane databases were searched for articles to March 2024. Searches involved the terms ‘motor neuron disease’ or ‘amyotrophic lateral sclerosis’ and ‘epidemiology’, ‘diagnosis’, ‘clinical’, ‘genetic’, ‘management’, ‘treatment’, or ‘trial’.
Areas of agreement
Evidence-based management involves riluzole, multidisciplinary care, provision of noninvasive ventilation and gastrostomy, and symptomatic treatments. Tofersen should be offered to treat SOD1-MND.
Areas of controversy
Edaravone and Relyvrio are approved treatments in the USA, but insufficient evidence was found to support approval in the UK and Europe.
Growing points
The discovery of neurofilaments as MND biomarkers, growth of platform trials and development of novel therapies provide optimism for more powerful neuroprotective therapies.
Areas timely for developing research
Further work should focus on the elucidation of environmental causes of MND, gene–environment interactions, and advanced cellular models of disease.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2024 The Authors. Except as otherwise noted, this author-accepted version of a journal article published in British Medical Bulletin is made available via the University of Sheffield Research Publications and Copyright Policy under the terms of the Creative Commons Attribution 4.0 International License (CC-BY 4.0), which permits unrestricted use, distribution and reproduction in any medium, provided the original work is properly cited. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ |
Keywords: | diagnosis; management; motor neuron disease |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Medicine, Dentistry and Health (Sheffield) > School of Medicine and Population Health |
Funding Information: | Funder Grant number National Institute for Health and Care Research NIHR203321 |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 30 Oct 2024 16:27 |
Last Modified: | 27 Feb 2025 09:13 |
Status: | Published |
Publisher: | Oxford University Press (OUP) |
Refereed: | Yes |
Identification Number: | 10.1093/bmb/ldae010 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:219089 |