Validation of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): A cognitive tool for motor disorders

Abstract

Our objective was to assess the validity of the Edinburgh Cognitive and Behaviour ALS Screen (ECAS), a multi-domain screen designed to detect cognitive deficits in patients with motor disorders. Forty ALS patients (without pre-diagnosed dementia) and 40, age-, gender- and education-matched healthy controls were recruited. All participants underwent extensive neuropsychological assessment and the ECAS. Performance at neuropsychological assessment across five domains (fluency, executive function, language, memory and visuospatial function) was compared to the ECAS ALS-Specific (fluency, executive functions and social cognition, language), ALS Non-specific (memory, visuospatial functions), and Total scores. Data from the healthy controls produced population-based abnormality cut-offs: composite score performance ≤ 2 SD in any domain classified impairment at neuropsychological assessment. Thirty-three percent of patients were impaired, most commonly in a single domain (executive or language dysfunction). Receiver Operator Curve (ROC) analyses using ECAS Total scores and ALS-Specific scores revealed 85% sensitivity and 85% specificity in the detection of cognitive impairment characteristic of ALS (fluency, executive function, language). A five-point borderline range produced optimal values (ALS-Specific Score 77-82, and ECAS-Total Score 105-110). In conclusion, validation against gold standard extensive neuropsychology demonstrated that the ECAS is a screening tool with high sensitivity and specificity to impairment characteristic of ALS.

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

“…Cut-off scores, based on 2 standard deviations (SD) below the mean, for abnormality have previously been reported for the ECAS-A (14) and validated against a full neuropsychological battery (17). The present study demonstrated that the newly presented alternate versions are highly equivalent to the original ECAS.…”

“…Not only has the ECAS shown high sensitivity and specificity to cognitive impairment against a full neuropsychological battery (17)(18)(19)(20)(21), it has high clinical utility in describing the nature of these impairments. Monitoring progression of cognitive and behavioural symptoms may have important implications for patient management, treatment, prognosis, end-oflife decision making, and caregiver burden (22)(23)(24)(25)(26)(27)(28).…”

“…The ECAS is a short screening tool designed with high clinical utility and is administrable by non-neuropsychological health care professionals. It has been validated against a comprehensive neuropsychological battery in Scottish (17), German/Swiss-German (15,18), Italian (19), Chinese (20), and Irish populations (21).…”

Screening for cognitive and behavioural impairment in patients with amyotrophic lateral sclerosis: frequency of abnormality and effect on survival

“…Therefore a variety of simple tools have been used to screen for cognitive impairment in ALS. These include Addenbrooke's cognitive examination-III (ACE-III), an updated version of the ACE-R [5][6][7][8], the Frontal assessment battery (FAB) [9][10][11], the Edinburgh cognitive and behavioral ALS screen (ECAS), which includes tests of social cognition, [12][13][14][15][16] and the ALS cognitive behavioral screen (ALS CBS) [17]. Table 1 shows the elements tested in the different tools.…”

Correlation between Cognitive Aspects and Dysphagia in Patients with Amyotrophic Lateral Sclerosis: Preliminary Report

“…Moreover, studies have shown that individuals with ALS present cognitive impairment, regardless of the type of neuropsychological assessment performed [22] [23] [24] [25]. Understanding cognitive impairment in ALS could provide more information about the pathogenesis of neurodegeneration and inform patient care [3] [26] [27] [28].…”