The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) in frontotemporal dementia

Abstract

Objectives: To examine the usefulness of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis (ALS) Screen (ECAS) as a cognitive screening tool for the detection of behavioral variant frontotemporal dementia (bvFTD). A secondary aim was to determine whether people with FTD combined with ALS (ALS-FTD) exhibit a similar ECAS profile to that of people with bvFTD alone. Methods: Patients with ALS-FTD and bvFTD and healthy controls were recruited. Participants were administered the ECAS, which comprises tests of language, verbal fluency, executive functions, memory, and visual-spatial functions. They also carried out analogous, full-length cognitive tests that examine naming, spelling, sentence completion, and social cognition skills. Results: The study cohort comprised 20 ALS-FTD patients, 23 with bvFTD, and 30 controls. Highly significant group differences were elicited for all cognitive domains, reflecting poorer performance in patients compared to controls. No significant differences in overall test scores were found between ALS-FTD and bvFTD patients, although ALS-FTD patients showed a higher frequency of impairment on verbal fluency. Correlative analyses revealed interrelationships in patients (but not controls) between scores in different domains, most marked in bvFTD. There were strong correlations between performance on ECAS subtests and analogous cognitive tasks. Conclusion: The ECAS is a sensitive and valuable tool for the assessment of FTD. Executive, language and behavioral breakdown may, however, compromise performance in other cognitive domains, reducing the specificity of the 'frontotemporal' cognitive profile. Subtle differences observed between ALS-FTD and bvFTD raise questions regarding the precise relationship between bvFTD with and without ALS.

ALS Cognitive Behavioral Screen-Phone Version (ALS-CBS™-PhV): norms, psychometrics, and diagnostics in an Italian population sample

“…By assessing executive functioning, the ALS-CBS™-PhV can furthermore be feasible for testing patients with frontotemporal dementias or other motor diseases possibly presenting with a dysexecutive profile (e.g., extra -pyramidal disorders) — as shown for another MND-specific I-level neuropsychological tool, the Edinburgh Cognitive and Behavioral ALS Screen [ 20 , 32 ].…”

“…From an experimental standpoint, the ALS-CBS™-PhV may allow large-scale epidemiological studies on FT disorders in MND patients [23] and facilitate follow-ups in clinical trials [4], specifically when combined with the available, self-administered ALS Functional Rating Scale -Revised [28]. By assessing executive functioning, the ALS-CBS™-PhV can furthermore be feasible for testing patients with frontotemporal dementias or other motor diseases possibly presenting with a dysexecutive profile (e.g., extra-pyramidal disorders) -as shown for another MND-specific I-level neuropsychological tool, the Edinburgh Cognitive and Behavioral ALS Screen [20,32].…”

Validation of The Edinburgh cognitive and behavioural ALS screen (ECAS) in behavioural variant frontotemporal dementia and Alzheimer’s disease

“…The percentage of impairment on the ECAS in the bvFTD group in our sample (94%) was similar to what has been previously reported (91%). 13 The posterior functions composite score (Memory and Visuospatial domains; sensitivity 97% and specificity 96%) was the most effective measure at detecting AD when compared to healthy controls. Although the abnormality -1581 cut-off of 88 of the ACE-III had a slightly higher sensitivity (96%) than the ECAS Total Score (sensitivity 94% and specificity 96%), it had a much lower specificity (86%) at detecting AD.…”

“…[10][11][12] A recent study demonstrated that both bvFTD with and without ALS were impaired on the ECAS against a healthy control group. 13 The study showed evidence of convergent validity with four standard neuropsychological tests of naming, spelling, cognitive inhibition (executive function) and social cognition. However, the study did not investigate the clinical utility of the test by validating performance against comprehensive clinical neuropsychology assessment or against more routinely used brief cognitive assessment/screening measures.…”

“…It has also been validated against comprehensive neuropsychological testing in ALS, showing good sensitivity and specificity 8,9 and convergent validity against other screening tests; the Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB), Consortium to Establish a Registry for Alzheimer’s disease plus Scale (CERAD plus) and Addenbrooke’s Cognitive Examination (ACE‐III) 10–12 . A recent study demonstrated that both bvFTD with and without ALS were impaired on the ECAS against a healthy control group 13 . The study showed evidence of convergent validity with four standard neuropsychological tests of naming, spelling, cognitive inhibition (executive function) and social cognition.…”