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dc.contributor.authorSteyn, Frederik J
dc.contributor.authorIoannides, Zara A
dc.contributor.authorvan Eijk, Ruben PA
dc.contributor.authorHeggie, Susan
dc.contributor.authorThorpe, Kathryn A
dc.contributor.authorCeslis, Amelia
dc.contributor.authorHeshmat, Saman
dc.contributor.authorHenders, Anjali K
dc.contributor.authorWray, Naomi R
dc.contributor.authorvan den Berg, Leonard H
dc.contributor.authorHenderson, Robert D
dc.contributor.authorMcCombe, Pamela A
dc.contributor.authorNgo, Shyuan T
dc.date.accessioned2020-01-16T06:05:07Z
dc.date.available2020-01-16T06:05:07Z
dc.date.issued2018
dc.identifier.issn0022-3050
dc.identifier.doi10.1136/jnnp-2017-317887
dc.identifier.urihttp://hdl.handle.net/10072/390546
dc.description.abstractObjective To determine the prevalence of hypermetabolism, relative to body composition, in amyotrophic lateral sclerosis (ALS) and its relationship with clinical features of disease and survival. Methods Fifty-eight patients with clinically definite or probable ALS as defined by El Escorial criteria, and 58 age and sex-matched control participants underwent assessment of energy expenditure. Our primary outcome was the prevalence of hypermetabolism in cases and controls. Longitudinal changes in clinical parameters between hypermetabolic and normometabolic patients with ALS were determined for up to 12 months following metabolic assessment. Survival was monitored over a 30-month period following metabolic assessment. Results Hypermetabolism was more prevalent in patients with ALS than controls (41% vs 12%, adjusted OR=5.4; p<0.01). Change in body weight, body mass index and fat mass (%) was similar between normometabolic and hypermetabolic patients with ALS. Mean lower motor neuron score (SD) was greater in hypermetabolic patients when compared with normometabolic patients (4 (0.3) vs 3 (0.7); p=0.04). In the 12 months following metabolic assessment, there was a greater change in Revised ALS Functional Rating Scale score in hypermetabolic patients when compared with normometabolic patients (-0.68 points/month vs-0.39 points/month; p=0.01). Hypermetabolism was inversely associated with survival. Overall, hypermetabolism increased the risk of death during follow-up to 220% (HR 3.2, 95% CI 1.1 to 9.4, p=0.03). Conclusions and relevance Hypermetabolic patients with ALS have a greater level of lower motor neuron involvement, faster rate of functional decline and shorter survival. The metabolic index could be important for informing prognosis in ALS.
dc.description.peerreviewedYes
dc.languageEnglish
dc.language.isoeng
dc.publisherBMJ Publishing Group Ltd
dc.relation.ispartofpagefrom1016
dc.relation.ispartofpageto1023
dc.relation.ispartofissue10
dc.relation.ispartofjournalJournal of Neurology Neurosurgery & Psychiatry
dc.relation.ispartofvolume89
dc.subject.fieldofresearchMedical and Health Sciences
dc.subject.fieldofresearchPsychology and Cognitive Sciences
dc.subject.fieldofresearchcode11
dc.subject.fieldofresearchcode17
dc.subject.keywordsScience & Technology
dc.subject.keywordsLife Sciences & Biomedicine
dc.subject.keywordsClinical Neurology
dc.subject.keywordsPsychiatry
dc.subject.keywordsSurgery
dc.titleHypermetabolism in ALS is associated with greater functional decline and shorter survival
dc.typeJournal article
dc.type.descriptionC1 - Articles
dcterms.bibliographicCitationSteyn, FJ; Ioannides, ZA; van Eijk, RPA; Heggie, S; Thorpe, KA; Ceslis, A; Heshmat, S; Henders, AK; Wray, NR; van den Berg, LH; Henderson, RD; McCombe, PA; Ngo, ST, Hypermetabolism in ALS is associated with greater functional decline and shorter survival, Journal of Neurology Neurosurgery & Psychiatry, 2018, 89 (10), pp. 1016-1023
dcterms.dateAccepted2018-03-24
dcterms.licensehttps://creativecommons.org/licenses/by-nc/4.0/
dc.date.updated2020-01-16T06:00:15Z
dc.description.versionVersion of Record (VoR)
gro.rights.copyright© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted. This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
gro.hasfulltextFull Text
gro.griffith.authorHeshmat, Sam


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