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Journal of Neurology
Journal of Neurology
Volume 271, Issue 10 October 2024

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Ratko Radakovic Amy Carroll Zachary Simmons

Background Self-perceived quality of life (QoL) is important in amyotrophic lateral sclerosis (ALS). Although caregiver burden and strain have been related to cognitive and behavioural impairment, there has been no comprehensive research looking at these impairments and how they may influence self-perceived QoL subdomains. Aims To explore how cognitive and behavioural impairment are related to different areas of self-perceived QoL using disease-specific measures.

Methods This was a quantitative, cross-sectional, observational cohort study, utilising existing specialist ALS clinic data. Clinical and demographic variables were available as well as multidimensional measures, ALS-specific QoL Short Form (ALSsQoL-SF) results and the data from the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Group comparison and regression analyses were performed.

Results Data from 121 participants with ALS were analysed. 61.2% (N=74) had either cognitive and/or behavioural impairment, with 28.9% (N=35) with cognitive impairment (ALSci), 14.1% (N=17) with behavioural impairment (ALSbi) and 18.2% (N=22) with both (ALScbi). 38.8% (N=47) were classified as having no impairments (ALSni). Those with ALSbi had significantly lower QoL in the domains of negative emotions and the interaction with people and the environment compared to those with ALSci and ALSni (ps<0.05). Further, those with ALScbi had significantly lower QoL in the intimacy domains than those with ALSci and ALSni (ps<0.05). Regression analysis showed specific cognitive and behavioural (inclusive of psychosis) predictors associated with specific QoL subdomains.

Conclusions Behavioural impairments effect QoL in specific subdomains, namely relating to internalising (negative emotions) and externalising (interaction with people and the environment subdomains, intimacy).