Which behaviours? Identifying the most common and burdensome behaviour changes in amyotrophic lateral sclerosis

Abstract

Behaviour change is increasingly recognised as a common feature of amyotrophic lateral sclerosis (ALS), and may be similar to that seen in frontotemporal dementia (FTD). The behaviours most disturbed in ALS, and those that relate most significantly to caregiver burden, however, have not been well established. Forty ALS participants and their caregivers, and 27 age- and gender-matched healthy controls and their relatives, participated in this study. ALS participants were assessed on a disease rating scale, and caregivers and control informants completed the revised version of the Cambridge Behaviour Inventory and a measure of burden. ALS caregivers reported significantly more disturbance than healthy control informants on the functional domains of everyday skills, self-care, and sleep, and in the behavioural domains of mood and motivation. There were no differences between groups in frequency of memory and orientation difficulties, or behaviours characteristic of FTD, such as changes to eating habits or stereotypic and motor behaviour, indicating that the behavioural profile in ALS may differ from FTD. In the ALS group, the domains with the strongest relationship to caregiver burden were everyday skills, motivation and memory, likely because poor motivation, memory dysfunction and difficulties completing activities of daily living require more carer support via direct supervision, prompting or hands on care. Services to support ALS patients and caregivers need to provide targeted interventions for those functional and behavioural changes which are most burdensome in the disease.

Keywords:

• Motor Neuron Disease
• behavior
• stress
• caregivers

Acknowledgements

The authors would like thank all the participants who made this study possible. The authors would also like to acknowledge MND Victoria and the Motor Neurone Disease Research Institute of Australia for providing the funding support for this research. Thank you to Consultant Neurologists Dr Susan Mathers, Dr James Howe and Dr Katrina Reardon for assisting with the data collection; and Dr Renee Lichter for her management of the data collection on this project. We would also like to acknowledge Professor Jacinta Douglas for her helpful comments during the development of this manuscript.