The Basis of Cognitive and Behavioral Dysfunction in Amyotrophic Lateral Sclerosis.

Autor: Bampton A; Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK., McHutchison C; Division of Psychology, University of Stirling, Stirling, UK., Talbot K; Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK., Benatar M; Department of Neurology, University of Miami Miller School of Medicine, Miami, Florida, USA., Thompson AG; Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK., Turner MR; Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Jazyk: angličtina
Zdroj: Brain and behavior [Brain Behav] 2024 Nov; Vol. 14 (11), pp. e70115.
DOI: 10.1002/brb3.70115
Abstrakt: 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.
(© 2024 The Author(s). Brain and Behavior published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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