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dc.contributor.authorHardiman, Orla
dc.contributor.authorPender, Niall
dc.date.accessioned2019-09-03T08:13:02Z
dc.date.available2019-09-03T08:13:02Z
dc.date.issued2018
dc.date.submitted2018en
dc.identifier.citationCrockford, C., Newton, J., Lonergan, K., Chiwera, T., Booth, T., Chandran, S., Colville, S., Heverin, M., Mays, I., Pal, S., Pender, N., Pinto-Grau, M., Radakovic, R., Shaw, C.E., Stephenson, L., Swingler, R., Vajda, A., Al-Chalabi, A., Hardiman, O., Abrahams, S., ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS, Neurology, 2018, 91, e1370-e1380en
dc.identifier.otherY
dc.identifier.urihttp://hdl.handle.net/2262/89402
dc.description.abstractObjective: To elucidate the relationship between disease stage in amyotrophic lateral sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and behavioral change, measured with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Methods: A large multicenter observational cohort of 161 cross-sectional patients with ALS and 80 healthy matched controls were recruited across 3 research sites (Dublin, Edinburgh, and London). Participants were administered the ECAS and categorized into independent groups based on their King's clinical disease stage at time of testing. Results: Significant differences were observed between patients and controls on all subtests of the ECAS except for visuospatial functioning. A significant cross-sectional effect was observed across disease stages for ALS-specific functions (executive, language, letter fluency) and ECAS total score but not for ALS-nonspecific functions (memory, visuospatial). Rates of ALS-specific impairment and behavioral change were also related to disease stage. The relationship between cognitive function and disease stage may be due to letter fluency impairment, whereas higher rates of all behavioral domains were seen in later King's stage. The presence of bulbar signs, but not site of onset, was significantly related to ALS-specific, ECAS total, and behavioral scores. Conclusion: ALS-specific cognitive deficits and behavioral impairment are more frequent with more severe disease stage. By end-stage disease, only a small percentage of patients are free of neuropsychological impairment. The presence of bulbar symptoms exaggerates the differences observed between disease stages. These findings suggest that cognitive and behavioral change should be incorporated into ALS diagnostic criteria and should be included in future staging systems.en
dc.description.sponsorshipThe ALS Association provided funding this study (ALS As-sociation grant 179). Further support was gained from theUniversity of Edinburgh’s Development and Alumni In-novative Initiative Grant. C.C. was funded by a scholarshipfrom the Euan MacDonald Centre for Motor Neurone Dis-ease Research. Clinical data were collected with thanks tothe MND Register, hosted by the CARE MND Register Re-search and funded by MND Scotland. The project is sup-ported through the following funding organizations under theaegis of JPND (EU Joint Programme—NeurodegenerativeDisease Research) United Kingdom: Medical ResearchCouncil (MR/L501529/1), Economic and Social ResearchCouncil (ES/L008238/1), and Irish Health Research Board(HRB-JPND/2013/1). C.E.S. and A.A.-C. receive salarysupport from the National Institute for Health ResearchBiomedical Research Centre at South London and MaudsleyNHS Foundation Trust and King’s College London.en
dc.format.extentE1370-E1380en
dc.language.isoenen
dc.relation.ispartofseriesNeurology;
dc.relation.ispartofseries91;
dc.relation.ispartofseries15;
dc.rightsYen
dc.subjectAmyotrophic lateral sclerosis (ALS)en
dc.subjectEdinburgh Cognitive andBehavioural ALS Screen (ECAS)en
dc.subjectALS Functional Rating Scale–Revised (ALSFRS-R)en
dc.titleALS-specific cognitive and behavior changes associated with advancing disease stage in ALSen
dc.typeJournal Articleen
dc.type.supercollectionscholarly_publicationsen
dc.type.supercollectionrefereed_publicationsen
dc.identifier.peoplefinderurlhttp://people.tcd.ie/hardimao
dc.identifier.peoplefinderurlhttp://people.tcd.ie/pendern
dc.identifier.rssinternalid206124
dc.identifier.doihttp://dx.doi.org/10.1212/WNL.0000000000006317
dc.rights.ecaccessrightsopenAccess
dc.subject.TCDThemeNeuroscienceen
dc.identifier.orcid_id0000-0003-2610-1291


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