Development and validation of the Korean Version of Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis Screen (ECAS)

Introduction: Cognitive and behavioral changes are common in amyotrophic lateral sclerosis (ALS) with about 15% of patients presenting with overt frontotemporal dementia (FTD) and approximately 30-50% of patients showing varying degree of impairments. We aimed to develop and validate the Korean version of Edinburgh Cognitive and Behavioral ALS Screen (ECAS-K), a brief multi-domain assessment tool developed for ALS patients with physical disability.

Methods: We developed the ECAS-K according to the guidelines for translation, and administered to 38 ALS patients and 26 age- and education-matched healthy controls. We also administered the Montreal Cognitive Assessment (MoCA) and Frontal Assessment Battery (FAB) to investigate convergent validity, and the Center for Neurologic Study-Liability Scale (CNS-LS) to assess the association between pseudobulbar affect and cognitive/behavioral changes.

Results: Internal consistency was found to be high between ECAS-K test items with Cronbach’s alpha of 0.87. Significant differences were noted between ALS patients and controls in language, fluency, and memory functions (p < 0.05). Abnormal performance was noted in 39.4% of patients on the ECAS total score, and 66.6% of patients showed behavioral changes across at least one domain. Significant correlations were observed between the ECAS-K and other cognitive screens (MoCA and FAB, correlation efficient of 0.69 and 0.55, respectively, p < 0.01).

Conclusion: We developed and validated the ECAS-K which could be used as an effective tool to screen the cognitive and behavioral impairments in Korean ALS patients.