Screening for cognition and behaviour changes in ALS

Abstract

This study presents the Edinburgh Cognitive and Behavioural ALS Screen (ECAS), developed for ALS patients with physical disability for use by health care professionals. The screen is designed to detect the specific profile of cognition and behaviour changes in ALS and to differentiate it from other disorders. Forty-eight ALS patients (none with evident dementia), 40 healthy controls and 20 carers were recruited. The ECAS, a 15–20-min screen, includes an ALS-Specific score (executive functions and social cognition; fluency; language); an ALS Non-specific score (memory; visuospatial functions); and a carer behaviour screen of five domains characteristic of frontotemporal dementia (FTD). Data from healthy controls produced abnormality cut-offs of 77/100 ALS-Specific score; 24/36 ALS Non-specific score; 105/136 ECAS Total. Twenty-nine percent of patients showed abnormal ALS-Specific scores, and 6% also showed abnormal ALS Non-specific scores. The most prevalent deficit occurred in language functions (35%) followed by executive functions and fluency (23% each). Forty percent of carers reported behaviour change in at least one domain, while 15% met criteria for possible FTD. In conclusion, the ECAS is an effective within-clinic assessment for ALS that determines the presence, severity and type of cognitive and/or behavioural changes, an essential first step to managing these symptoms.

Key words::

• Cognition
• behaviour
• screen
• ECAS
• frontotemporal dementia

Acknowledgements

This study was funded by an award from the Motor Neurone Disease Association. The authors would like to thank all the people with MND and their carers for participating in this research. The authors also thank Shuna Colville, Siddharthan Chandran and Richard Davenport for their support and help in recruiting patients with MND.

Disclosure of interest: The authors have no known conflict of interest in relation to publication of this paper. Three of the authors (JN, EN, JF) were funded by an award from the Motor Neurone Disease Association when undertaking this research. The remaining two authors were funded by the University of Edinburgh. The authors alone are responsible for the content and writing of the paper.