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.

Keywords:

• Amyotrophic lateral sclerosis
• Edinburgh cognitive and behavioral ALS screen
• cognitive dysfunction
• normative data

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Declaration of interest

L.A. Bakker, L.A. Spreij, M. Verhaegen, J. de Vocht, Dr. C.D. Schröder, Dr. T.C.W. Nijboer, Prof. Dr. J.M.A. Visser-Meily, and Prof. P. van Damme have nothing to disclose.

Additional information

Funding

Prof. Dr. L.H. van den Berg reports grants from ALS Foundation Netherlands, grants from The Netherlands Organization for Health Research and Development (Vici scheme), grants from The Netherlands Organization for Health Research and Development (SOPHIA, STRENGTH, ALS-CarE project), funded through the EU Joint Programme – Neurodegenerative Disease Research, (JPND), personal fees from Baxalta, personal fees from Biogen, personal fees from Cytokinetics, other from Prinses Beatrix SpierFonds, other from Latran Foundation, outside the submitted work. M.A. van Es received grants from the Netherlands Organization for Health Research and Development (Veni scheme), The Thierry Latran foundation, Joint Programme – Neurodegenerative Disease Research (JPND) and the Netherlands ALS Foundation (Stichting ALS Nederland). He received travel grants from Baxalta and serves on the biomedical research advisory panel of the motor neurone disease association (MNDA).