Derivation of norms for the Dutch version of the Edinburgh cognitive and behavioral ALS screen

Abstract

Background: The Edinburgh cognitive and behavioral ALS screen (ECAS) was developed specifically to detect cognitive and behavioral changes in patients with amyotrophic lateral sclerosis (ALS). Differences with regard to normative data of different (language) versions of neuropsychological tests such as the ECAS exist. Objective: To derive norms for the Dutch version of the ECAS. Methods: Normative data were derived from a large sample of 690 control subjects and cognitive profiles were compared between a matched sample of 428 patients with ALS and 428 control subjects. Results: Age, level of education, and sex were significantly associated with performance on the ECAS in the normative sample. ECAS data were not normally distributed and therefore normative data were expressed as percentile ranks. The comparison of ECAS scores between patients and control subjects demonstrated that patients obtained significantly lower scores for language, executive function, verbal fluency, and memory, which is in line with the established cognitive profile of ALS. Conclusion: For an accurate interpretation of ECAS results, it is important to derive normative data in large samples with nonparametric methods. The present normative data provide healthcare professionals with an accurate estimate of how common or uncommon patients' ECAS scores are and provide a useful supplement to existing cut-off scores.

The Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen (ECAS): sensitivity in differentiating between ALS and Alzheimer’s disease in a Greek population

“…In the present study, 50% of the subjects were impaired on the ECAS Total score and 46% on the ECAS-ALS Specific score, which is in accordance with other studies [5,12,[14][15][16]. The most prominent cognitive impairments were found in language (36%), verbal fluency (36%), and executive functions (32%), which aligns with the previous studies in ALS patients [5,[13][14][15][16][17][18][19][20]38]. Moreover, the comorbidity of impairments was detected only in a small percentage of The prevalence of executive function and verbal fluency in addition to language impairment in the current study is in accordance with the English, Italian, and German validation studies of ECAS [12,13,15] and with an extended systematic review of 44 neuropsychological studies with 1130 ALS patients, where language dysfunction appeared to be the most prevalent cognitive impairment [38].…”

“…The most frequently reported behavioural changes were apathy (54%), loss of sympathy (29%), and compulsive behaviour (22%). The first two changes have been considered as highly prevalent behaviour changes in previous studies [12][13][14][15][16][17][18][19][20]. The present study and the Italian validation study of the ECAS highlighted comparably high percentages of apathetic behaviour (54% and 45%, respectively) [15].…”

“…Alternatively, the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has been developed as a more comprehensive brief assessment [13,14]. It has been adapted for Italian [15], German [16], Chinese [17], Dutch [18], and Spanish [19] populations. The ECAS is a brief screening tool (administration time approximately 20 mins) that is adjustable for patients with various motor impairments.…”

“…The ECAS is a brief screening tool (administration time approximately 20 mins) that is adjustable for patients with various motor impairments. It comprises a comprehensive assessment of a spectrum of cognitive and behavioural abnormalities in ALS [9,[13][14][15][16][17][18][19]. Furthermore, it has been validated in detecting the milder focal impairments in ALS (14,20).…”

“…Furthermore, it has been validated in detecting the milder focal impairments in ALS (14,20). The ECAS comprises an ALS-specific score (executive function, social cognition, verbal fluency, and language), an ALS-non-specific score (memory and visuospatial functions), and a carer's interview to detect the behavioural and psychotic changes typical in FTD [9,[13][14][15][16][17][18][19].…”