The Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen: A cross-sectional comparison of established screening tools in a German-Swiss population

Abstract

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has recently been developed as a fast and easy cognitive screening tool specifically designed for patients with motor impairments in routine clinical use. The German/Swiss-German version of the ECAS was validated in a German-Swiss consortium. One hundred and thirty-six non-demented ALS patients and 160 healthy controls were included in the study. In addition, the Frontal Assessment Battery (FAB), Montreal Cognitive Assessment (MoCA) and Consortium to Establish a Registry for Alzheimer's Disease plus Scale (CERAD plus) were administered to subgroups of patients. Results showed that administration of ECAS was fast (mean 24 min). Similar to the population in the UK version, ALS patients performed significantly worse in the ALS-specific functions (p < 0.001), specifically in the domain of language (p < 0.001), verbal fluency (p = 0.005) and executive functions (p = 0.02), but not for the non-ALS-specific functions. Carers reported behavioural abnormalities in about 30% and psychotic symptoms in 6% of the patients. Compared to ECAS, FAB, MoCA and CERAD were more generic and performance was not adjusted to motor speed. We conclude that the German/Swiss-German version of the ECAS is a fast and easy to administer cognitive screening instrument sensitive for ALS-specific dysfunctions and behaviour change.

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

“…The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has been recently developed to overcome this issue (14). The ECAS has been designed to measure cognitive functions, unrestricted by physical disability (15), that are commonly affected in ALS (executive functioning, language functioning, and verbal fluency) in addition to functions less commonly affected (memory and visuospatial functions). Additionally, the ECAS includes a clinical caregiver behaviour interview based on diagnostic criteria for FTD (16).…”

“…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).…”

NEK1mutations in familial amyotrophic lateral sclerosis

“…The patient is still alive. The Edinburgh Cognitive Assessment (ECAS) (Lulé et al, 2015) was performed twice with normal results.…”

Clinical and laboratory features of ALS

“…Therefore a variety of simple tools have been used to screen for cognitive impairment in ALS. These include ACE-III, an updated version of the ACE-R [83,[199][200][201] , FAB [84,202,203] , ECAS, which includes tests of social cognition [85,[204][205][206][207] and ALS CBS [86] . Table 5 shows the elements tested in the different tools.…”

“…[209] ECAS China/2016 84 36 % 27 % Ye et al [204] ECAS Italy/2016 107 37% 54% Poletti et al [207] ALS CBS USA/2015 86 62% 37% Bock et al [212] FAB Greece/2015 34 50% N/A Nidos et al [213] ALS CBS Spain/2015 50 46% 18% Turon-sans et al [214] ACE-R China/2014 145 30% N/A Wei et al [208] FAB Canada/2014 44 21% N/A Osborne et al [215] MoCA Canada/2014 39 53% N/A Osborne et al [215] FAB and FBI China/2014 126 32% 46% Wei et al [208] ECAS Germany/2014 136 22% 30% Lule et al [205] FAB USA/2009 16 50% N/A Oskarsson et al [216] FBI: Frontal behaviour inventory MoCA: Montreal cognitive assessment Current evidence suggests that some people with ALS have cognitive and behavioural impairment, and others do not. The cause of behavioural impairment in ALS is unclear.…”