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Objectives: The aim of this study was to develop and apply a single method for measuring cognitive change across European centres in an attempt to harmonize cognitive screening and compare cognitive phenotypes.
Methods: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has been previously shown to be sensitive to cognitive impairment in ALS (1). Here the ECAS was translated and applied to a total of 466 ALS patients (Scotland, n = 78, Ireland, n = 73, Italy-Milan n = 59, Italy-Turin n = 33, Holland, n = 40, Spain n = 40, Germany n = 73 and Switzerland, n = 35). Furthermore local normative data was collected within each country and cut-offs for abnormality determined.
Results: The ECAS was found to be sensitive to the types of impairment typically present in ALS patients across countries. The frequency of impairment within each country significantly differed ranging from 10 to 53% with a higher proportion of impaired patients from the samples in Ireland, Italy and Scotland and lower rates in Germany and the Netherlands. Furthermore the profile of impairment across cognitive domains also differed between countries with a greater proportion of patients with non-specific cognitive dysfunction (Memory, Visuospatial) in Ireland, while those in Scotland, Italy and Germany showed a more typical disproportionate impairment in ALS Specific functions (Executive, Language and Fluency).
There was a significant difference between samples in diagnostic delay and this significantly correlated with the degree of impairment in executive functions. Patients with a shorter delay indicating a more aggressive disease having more cognitive impairment. Furthermore duration of illness significantly correlated with ECAS scores, those patients with a longer duration of illness less likely to experience cognitive change. Age of onset also significantly correlated with cognitive impairment, with younger patients less likely to experience cognitive change. Frequency of impairment did not significantly differ according to symptom site of onset, which contradicts previously described associations with bulbar dysfunction, once tests which accommodate for speech and limb disability as the ECAS are used.
Genetic analyses were available from 81 patients. Those positive for the C9orf72 mutation displayed ALS-specific cognitive dysfunction but were also more likely to have visuospatial dysfunction than those found to be negative for this mutation. The findings are related to clinical profiles including severity of disease and population differences.