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

<p><i>Objective</i>: 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. <i>Methods</i>: The ECAS was administered serially to ALS patients (<i>n</i> = 24 after six months, <i>n</i> = 10 after 12–18 months) and controls (<i>n</i> = 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. <i>Results</i>: After six months controls showed a significantly higher overall score (<i>p</i> < 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 The FAB was no longer applicable due to progressive motor deficits in 20% of ALS patients. <i>Conclusions</i>: In conclusion, in contrast to healthy controls, ALS patients show no practice effects. This could reflect ‘pre-symptomatic’ cognitive decline and progressive behavioural symptoms.</p

Characteristic of the autopsy cohort.

<p>Characteristic of the autopsy cohort.</p

Risk of bronchopneumonia under NIV.

<p>Risk of bronchopneumonia under NIV.</p

Autopsy confirmed causes of death in %.

<p>Autopsy confirmed causes of death in %.</p

Impact of NIV and PEG on survival^*.

<p>Impact of NIV and PEG on survival^{<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0177555#t002fn002" target="_blank">*</a>}.</p

Age and education-matched cut-off scores for the revised German/Swiss-German version of ECAS

<p>The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has been developed to assess cognition and behaviour in patients with amyotrophic lateral sclerosis (ALS). Cognitive impairments of ALS-specific and ALS-non-specific functions can be determined using cut-off scores based on performance of healthy subjects. However, detailed analyses show that older healthy subjects perform worse than younger ones, whereas highly-educated individuals perform better than those with lower education levels. As a consequence, this study presents new age and education matched cut-off scores for the revised German/Swiss-German version of the ECAS based on the performance of 86 healthy subjects.</p

Correlation between duration of measurements and maximum stimulus intensity for all 6 muscles.

<p>Correlation between duration of measurements and maximum stimulus intensity for all 6 muscles.</p

Coefficient of variation (COV) and variability () for MUNIX measurements in individual muscles in raters.

<p>Coefficient of variation (COV) and variability () for MUNIX measurements in individual muscles in raters.</p

Inter-rater variability (COV) in individual muscles for the first and second measurement and mean of both values.

<p>Inter-rater variability (COV) in individual muscles for the first and second measurement and mean of both values.</p

Relative mean and standard deviation of MUNIX and CMAP measurements in individual muscles of the experienced group (filled circles) and less-experienced group (empty circles) compared to the hypothetical reference values, expressed as accuracy (%).

<p>Relative mean and standard deviation of MUNIX and CMAP measurements in individual muscles of the experienced group (filled circles) and less-experienced group (empty circles) compared to the hypothetical reference values, expressed as accuracy (%).</p