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Background. The multisystematic character of ALS is evident. Cognitive deficits have been repeatedly described in patients, however, the clinical description of severity and frequency of deficits varies considerably between studies. The Edinburh cognitive and behavioural ALS screen (ECAS) has been developed only recently (Abrahams et al., ALSFTD 2013) as a fast and easy to administer cognitive screening tool for clinical routine. The ECAS includes an ALS-Specific score (executive functions and social cognition; fluency; language); an ALS Non-specific score (memory; visuospatial functions); and a carer behaviour screen of five domains characteristic of frontotemporal dementia (FTD).
Objective. In a German-Swiss consortium, the German/Swiss version of the ECAS was validated.
Methods. N=136 ALS patients and N=160 healthy controls were included in the study. ECAS were administered in a German version to N=56 healthy subjects from Ulm and N=55 from Hannover. A slightly modified Swiss version was administered to N=49 healthy subjects from St Gallen. Data were compared to N=62 ALS patients from Ulm, N=41 from Hannover and N=31 from St Gallen. N=53 carer behaviour screens were filled out. In addition, frontal assessment battery (FAB), Montreal cognitive assessment (MOCA) and CERAD was administered to subgroups of patients. Datasets of N=10 patients were tested on interrater-reliability.
Results. Administration of ECAS was fast (mean 22 minutes; range 17-38). Data of healthy subjects produced total ECAS cut-off score of 82/135; 59/100 for ALS specific functions and 20/36 for Non-ALS specific functions. ECAS cut-off score adjusted for age and education were determined for the German and Swiss group separately. Patients performed significatly worse in the ALS specific functions (p<0.001), in specific 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 that 30% of the patients presented with behavioural abmnormalities and 8% with psychotic symptoms. Internal consistency of ECAS with FAB and MOCA, respectively, was high for Non-ALS specific functions only (Chronbachs alpha >0.06), but not for ALS specific functions (Cronbachs alpha <0.04). Interrater-reliabilty was high.
Conclusion.
The German/Swiss version of the ECAS is a fast to administer cognitive screening instrument sensitive for ALS specific functions. It is additionally informative about behavioural changes. Cut-off scores of the German/Swiss version were far below those of the English version, highlighting the importance of validation and defining language specific cut-off scores. Age and education produced a high variance in data, asking for the need to produce age and education adjusted cutoffs, separately for the German and Swiss version.