Cognitive-behavioral changes in amyotrophic lateral sclerosis: Screening prevalence and impact on patients and caregivers

Abstract

Our objective was to evaluate the association between cognitive-behavioral deficits and patient quality of life (QoL), caregiver burden, and disease stage in a population of patients with amyotrophic lateral sclerosis (ALS). We administered the ALS Cognitive-Behavioral Screen™ to 86 patients with ALS. Multiple regression was used to evaluate the association between cognitive or behavioral deficits and disease stage, patient QoL, and caregiver burden while controlling for clinically important variables. Of 86 participants enrolled, 53 (62%) had some degree of cognitive impairment, 32 (37%) were behaviorally impaired and four met both cognitive and behavioral screening criteria for frontotemporal dementia (FTD). The severity of cognitive-behavioral deficits was not associated with patient QoL. More pronounced cognitive deficits (beta = –1.4, p = 0.04) and behavioral symptoms (–0.69, p < 0.001) predicted higher caregiver burden. Self-reported QoL was lower in patients with more depressive symptoms (beta = –0.32, p < 0.001) and more advanced disease (beta =0.10, p = 0.01). In conclusion, general QoL for patients with ALS is not associated with cognitive or behavioral deficits. More severe cognitive deficits and caregiver-reported behavioral symptoms predict higher caregiver burden. Routine cognitive-behavioral screening can identify patients who require full neuropsychological examination, inform patient counseling, and identify caregivers in need of early, targeted interventions.

Key words:

• Amyotrophic lateral sclerosis
• frontotemporal dementia
• quality of life
• caregiver burden

Acknowledgements

This publication was supported by the National Center for Advancing Translational Sciences, National Institutes of Health. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIH. We thank Susan Woolley-Levine for use of the ALS Cognitive-Behavioral screen. We gratefully acknowledge Betty Smoot, Jennifer Creasman, and the UCSF ALS Center multidisciplinary team for their thoughtful contributions to the project design and Danny Yagan for assistance with statistical analysis.

Declaration of interest

A. Kim reports research grants from SanBio, Inc. and Biogen Idec. He provides expert witness consulting on topics unrelated to this work. M. Bock, Y-N. Duong, I. Allen, J. Murphy, and C. Lomen-Hoerth report no disclosures.