Article
Details
Citation
Bampton A, McHutchison C, Talbot K, Benatar M, Thompson AG & Turner MR (2024) The Basis of Cognitive and Behavioral Dysfunction in Amyotrophic Lateral Sclerosis. Brain and Behavior, 14 (11), Art. No.: e70115. https://doi.org/10.1002/brb3.70115
Abstract
ABSTRACT
Objective
To summarize and evaluate evidence pertaining to the clinical, genetic, histopathological, and neuroimaging correlates of cognitive and behavioral dysfunction in amyotrophic lateral sclerosis (ALS).
Methodology
We comprehensively reviewed the literature on cognitive and behavioral manifestations of ALS, narrating findings from both cross-sectional and longitudinal studies. We discussed knowledge gaps in the evidence base and key limitations affecting studies to date, before formulating a framework for future research paradigms aimed at investigating clinicopathological correlates of neuropsychological dysfunction in ALS.
Results
Studies have demonstrated clinical associations with cognitive dysfunction in ALS e.g., bulbar-onset of symptoms, pathological associations (extramotor TDP-43 deposition), and imaging associations (frontotemporal involvement). The most common behavioral deficit, apathy, is highly associated with verbal fluency, but longitudinal studies assessing behavioral dysfunction in ALS are comparatively lacking.
Conclusion
Longitudinal studies have been helpful in identifying several potential correlates of cognitive and behavioral dysfunction but have frequently been confounded by selection bias and inappropriate testing platforms. This review provides a framework for more robust assessment of clinicopathological associations of neuropsychological abnormalities in ALS in the future, advocating for greater utilization of pre-symptomatic C9orf72 repeat expansion-carrying cohorts.
Keywords
amyotrophic lateral sclerosis; behavioral; C9orf72; cognitive; frontotemporal dementia; motor neurone disease; TDP-43
Journal
Brain and Behavior: Volume 14, Issue 11
Status | Published |
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Funders | Medical Research Council |
Publication date | 30/11/2024 |
Publication date online | 05/11/2024 |
Date accepted by journal | 05/10/2024 |
URL | http://hdl.handle.net/1893/36494 |
Publisher | Wiley |
ISSN | 2162-3279 |
eISSN | 2157-9032 |
People (1)
Lecturer in Psychology, Psychology