Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)

Abstract

Objective

Cognitive and behavioral impairment is observed in up to 50% of patients with amyotrophic lateral sclerosis (ALS). The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a 5-domain screening tool customized for quick cognitive screening in patients with ALS. Although the ECAS is available in Swedish at the Karolinska University Hospital (SK-ECAS), it has not yet been validated in Sweden stressing the need to assess validity and reliability of the SK-ECAS Version A.

Methods

The study included 176 patients with ALS or other motor neuron disease diagnosed between September 2017 and October 2021 at the Karolinska ALS Clinical Research Center in Stockholm, Sweden, and 35 age-matched healthy control subjects. SK-ECAS was validated against the Montreal Cognitive Assessment (MoCA) and optimal cutoffs, receiver operating characteristic (ROC) curve and area under the curve (AUC) were calculated.

Results

We identified an optimal cutoff of 108 for the SK-ECAS total score and 82 for the SK-ECAS ALS-specific score to detect cognitive impairment. The SK-ECAS showed good performance in indicating abnormal cognition with an AUC of 0.73 for SK-ECAS ALS-specific score and 0.77 for SK-ECAS total score. There was good internal consistency with a Cronbach’s alpha of 0.79.

Conclusions

This study demonstrates good validity and reliability indices for SK-ECAS Version A for the detection of cognitive impairment in newly diagnosed ALS patients.

Keywords:

• Amyotrophic lateral sclerosis
• cognition
• neuropsychology
• Sweden
• validation

Acknowledgment

The authors report no competing interests. This project was funded thanks to the Ulla-Carin Lindquists foundation for ALS research, grant number 2021:1. We would like to thank Jenny Hellqvist for her precious help during the data collection process. We would also like to thank the Umeå research team for their Swedish translation of the ECAS. And most important, we are grateful to the many patients and healthy control individuals who contributed to this study.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.