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ALS Specific cognitive and behaviour changes associated with advancing disease stage in ALS

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Original languageEnglish
Article numbere1370
Issue number15
Early online date12 Sep 2018
Publication statusE-pub ahead of print - 12 Sep 2018


Objective: The purpose of this study is to elucidate the relationship between disease stage in ALS, as measured using the King’s Clinical Staging System, and cognitive and behavioural change using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS).

Methods: A large multicentre observational cohort of 161 cross-sectional patients with ALS and 80 healthy matched controls were recruited across three research sites (Dublin, Edinburgh, London). Participants were administered the ECAS and categorised 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 Non-Specific functions (memory, visuospatial). Rates of ALS-Specific impairment and behavioural change 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 behavioural domains were seen in later King’s stage. The presence of bulbar signs, but not site of onset, significantly related to ALS Specific, ECAS Total, and behavioural scores.

Conclusions: ALS specific cognitive deficits and behavioural 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 behavioural change should be incorporated into ALS diagnostic criteria, and should be included in future staging systems.

    Research areas

  • Amyotrophic Lateral Sclerosis, assessment of cognitive disorders/dementia, behaviour, staging, all neuropsychology/behavior, frontotemporal dementia

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