Cognitive deficits have only limited influence on health-related quality of life in amyotrophic lateral sclerosis

Abstract

Objective

To explore the association between cognitive deficits and health-related quality of life in amyotrophic lateral sclerosis (ALS).

Methods

The revised ALS Functional Rating Scale (ALSFRS-R for physical impairment), the ALS Assessment Questionnaire (ALSAQ-40 for health-related quality of life) and the Edinburgh Cognitive and Behavioral ALS Screen (ECAS for cognition) were assessed in 125 patients with ALS. Correlations between ALSAQ-40 domains and ECAS functions were tested using Spearman correlation. Linear regression was used to evaluate the relationship between dysphagia, depression, hopelessness, pain (all derived from corresponding items from the ALSFRS-R or ALSAQ-40), ALSFRS-R, ECAS and the ALSAQ-40.

Results

Verbal fluency, language and executive function were disturbed in 69 (55%), 54 (43%) and 41 (33%) patients, respectively. In the ALS non-specific domains the memory and visuospatial function were impaired in 44 (35%) and 12 (10%) patients. In the non-demented group the five ECAS functions did not correlate with the ALSAQ-40 subdomains. The ALSFRS-R score, hopelessness, pain, and depression explained 65% of the ALSAQ-40 SI variance; the ECAS total score did not significantly predict ALSAQ-40 summary index. The ECAS visuospatial, executive function and fluency significantly predicted emotional well-being (adjusted R² = 0.08). When the model was controlled for depression, hopelessness and pain none of the ECAS functions (visuospatial, executive function and fluency) were significant predictors of emotional well-being.

Conclusion

Deficits in visuospatial function, executive function and fluency constrain the ability to manage activities of daily living and this might cause decline in well-being.

Keywords:

• Cognition
• mood
• emotional well-being
• depression
• ALS Assessment Questionnaire
• Edinburgh Cognitive and Behavioral ALS Screen

Acknowledgements

We would like to thank Mandy Arnold and Cindy Höpfner for patient care and assistance and Carmen Lewa, Christian Baldauf, Anna Schneider, Annalena Goebel and Carolin Nikolaus for conducting questionnaire-based surveys. We thank all our study participants for their co-operation and time.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

The present study was supported by the German Bundesministerium für Bildung und Forschung (BMBF) grant SOPHIA and OnWEBDuals to Julian Grosskreutz under the aegis of the EU Joint Programme - Neurodegenerative Disease Research (JPND) and a BMBF grant PYRAMID to Julian Grosskreutz in the framework of the ERANET E-RARE program.