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ToolsCrockford, Christopher, Newton, Judith, Lonergan, Katie, Chiwera, Theresa, Booth, Tom, Chandran, Siddharthan, Colville, Shuna, Heverin, Mark, Mays, Iain, Pal, Suvankar, Pender, Niall, Pinto-Grau, Marta, Radakovic, Ratko, Shaw, Christopher E., Stephenson, Laura, Swingler, Robert, Vajda, Alice, Al-Chalabi, Ammar, Hardiman, Orla and Abrahams, Sharon (2018) ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS. Neurology, 91 (15). E1370-E1380. ISSN 0028-3878
Full text not available from this repository. (Request a copy)Abstract
Objective 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.
| Item Type: | Article |
|---|---|
| Additional Information: | Funding Information: The ALS Association provided funding this study (ALS Association grant 179). Further support was gained from the University of Edinburgh’s Development and Alumni Innovative Initiative Grant. C.C. was funded by a scholarship from the Euan MacDonald Centre for Motor Neurone Disease Research. Clinical data were collected with thanks to the MND Register, hosted by the CARE MND Register Research and funded by MND Scotland. The project is supported through the following funding organizations under the aegis of JPND (EU Joint Programme—Neurodegenerative Disease Research) United Kingdom: Medical Research Council (MR/L501529/1), Economic and Social Research Council (ES/L008238/1), and Irish Health Research Board (HRB-JPND/2013/1). C.E.S. and A.A.-C. receive salary support from the National Institute for Health Research Biomedical Research Centre at South London and Maudsley NHS Foundation Trust and King’s College London. The work leading up to this publication was funded by the European Community’s Health Seventh Framework Programme (FP7/2007–2013; grant agreement 259867) and Horizon 2020 Programme (H2020-PHC-2014-two-stage; grant agreement 633413). The Article Processing Charge was funded by UKRI. |
| Uncontrolled Keywords: | clinical neurology ,/dk/atira/pure/subjectarea/asjc/2700/2728 |
| Faculty \ School: | Faculty of Medicine and Health Sciences > Norwich Medical School |
| Related URLs: | |
| Depositing User: | LivePure Connector |
| Date Deposited: | 13 Aug 2024 09:31 |
| Last Modified: | 25 Sep 2024 18:01 |
| URI: | https://ueaeprints.uea.ac.uk/id/eprint/96220 |
| DOI: | 10.1212/WNL.0000000000006317 |
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