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

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was developed to assess cognitive and behavioural changes in an anterior frontotemporal syndrome (executive functions, language, fluency and behaviour), common in Amyotrophic Lateral Sclerosis (ALS) and also assesses posterior cerebral dysfunction (memory and visuospatial abilities). OBJECTIVES: To validate the ECAS in behavioural variant Frontotemporal Dementia (bvFTD) without ALS, as compared with Alzheimer's disease (AD), against comprehensive neuropsychological assessment. Compare its sensitivity to that of the Addenbrooke's Cognitive Examination (ACE-III) and investigate behavioural changes in both types of dementia. METHODS: Retrospective study of 16 people with bvFTD (without ALS), 32 with AD, and 48 healthy controls completed the ECAS, ACE-III and extensive neuropsychological assessment. RESULTS: The ECAS showed higher sensitivity (94%) and marginally lower specificity (96%) than the ACE-III for both the bvFTD and AD groups. The anterior composite subscore was sensitive for bvFTD (94%), and slightly less so for AD (84%), while the posterior composite subscore was sensitive for AD (97%), and less so for bvFTD (75%). All people with bvFTD that were impaired on the ECAS total and anterior composite scores were also impaired on the anterior function's tests of the neuropsychological assessment. A cut-off of four or more behavioural domains affected differentiated well between the bvFTD and AD groups, while a qualitative analysis of the behavioural interview found different themes between groups. CONCLUSIONS: The ECAS is a valid and sensitive assessment for bvFTD without ALS and for AD. The carer behavioural interview makes it particularly suitable to detect behavioural abnormalities related to frontal lobe disorders.

The Edinburgh Cognitive and Behavioral ALS screen: relationship to age, education, IQ and the Addenbrooke's Cognitive Examination-III.

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was developed to assess cognitive and behavioral changes common in Amyotrophic Lateral Sclerosis and other diseases affecting motor functions. It focuses on domains typically affected by the frontotemporal syndrome (executive and language functions, fluency and behavior), but assesses also memory and visuospatial functions. OBJECTIVES: (A) To investigate the relationship between the ECAS and the Addenbrooke's Cognitive Examination (ACE-III). (B) To investigate the effects of age, education, and IQ on the ECAS and create appropriate cutoff scores to determine abnormality. METHODS: (A) 57 healthy participants (aged 35-80) were assessed with the ECAS, the Wechsler Abbreviated Scale of Intelligence (WASI-II), and the ACE-III. (B) 80 healthy participants (aged 51-80) were divided into four groups according to age and education; and were tested with the ECAS and the WASI-II. RESULTS: The ECAS and the ACE-III have a good convergent validity with a significant correlation. Regression analysis revealed that IQ, followed by age, were the strongest predictors of the total ECAS score. IQ predicted 24% of the ECAS and 46% of the ACE-III variance. Education was not a significant predictor over and above IQ for both the ECAS and the ACE-III. Abnormality cutoff scores adjusted for age and education are presented. CONCLUSIONS: The ECAS shows good convergent validity with the ACE-III, but is less influenced by intelligence and presents less ceiling effects. The inclusion of an executive function assessment and behavioral interview in the ECAS makes it particularly useful for the assessment of frontal lobe disorders.