Longitudinal assessment of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): lack of practice effect in ALS patients?

Abstract

Objective: The study objective was to assess whether controls and ALS patients show a practice effect in the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) on repeated longitudinal testing and if the ECAS detects progression of cognitive or behavioural changes over time. Methods: The ECAS was administered serially to ALS patients (n = 24 after six months, n = 10 after 12–18 months) and controls (n = 21 after six months). The ECAS was fully performed by all participants. For comparison purposes the Frontal Assessment Battery (FAB) was administered to a subgroup of 14 patients and 14 controls. Results: After six months controls showed a significantly higher overall score (p < 0.001) and significantly higher scores in all subdomains of the ECAS, except for visuospatial function and fluency. ALS patients showed no significant difference in any score of the ECAS after six months and up to18 months. Behavioural changes were increasingly, but not statistically, significant, noted by patient carers. The FAB was no longer applicable due to progressive motor deficits in 20% of ALS patients. Conclusions: In conclusion, in contrast to healthy controls, ALS patients show no practice effects. This could reflect ‘pre-symptomatic’ cognitive decline and progressive behavioural symptoms.

Keywords:

• ECAS
• longitudinal
• practice effect
• cognition
• amyotrophic lateral sclerosis
• frontotemporal dementia
• behaviour

Acknowledgements

We would particularly like to thank the patients and carers who gave of their time to participate in the study. This is an EU Joint Programme – Neurodegenerative Disease Research (JPND) project. The project is supported through the following organisations under the aegis of JPND – a supported by the Schweizerischer Nationalfonds (SNF 31ND30-141622).

Declaration of interest

Christian Burkhardt: C. Burkhardt receives honoraria from Biogen Idec for training courses and receives research support from the Swiss ALS Foundation and the EU Joint Programme-Neurodegenerative Disease Research (JPND) projects (grant number SNF 31ND30_141622 (SOPHIA) and 31ND30_151115 (STRENGTH)).

Christoph Neuwirth: C. Neuwirth receives honoraria from Biogen Idec, USA, as advisory board member tasks and training courses and support from the Swiss ALS Foundation and the EU Joint Programme-Neurodegenerative Disease Research (JPND) projects (grant number SNF 31ND30_141622 (SOPHIA) and 31ND30_151115 (STRENGTH)).

Markus Weber: M. Weber received honoraria from Merz Pharma Schweiz AG, Biogen Idec, USA, as advisory board member and support from the Swiss ALS Foundation and the EU Joint Programme-Neurodegenerative Disease Research (JPND) projects (grant number SNF 31ND30_141622 (SOPHIA) and 31ND30_151115 (STRENGTH)).

Supplementary material available online