• ALS
• clinical phenotype
• cognitive profile
• ECAS
• Neuropsychological evaluation

Although Amyotrophic Lateral Sclerosis (ALS) is traditionally considered a neurodegenerative disorder affecting motor neurons, growing evidence highlights its multisystem nature, often involving cognitive and behavioral dysfunctions. This study aimed to evaluate the cognitive profile of a selected italian cohort of ALS patients, investigating potential cognitive impairments at symptom onset using both the ECAS (Edinburgh Cognitive and Behavioral ALS Screen) and II level neuropsychological assessments.
Our findings revealed heterogeneity in cognitive profiles. Impaired scores in ALS-specific tests were strongly associated with lower ECAS Total Scores, while ALS-non-specific test scores showed a weaker relationship with the Total Score. Similarly, the limited overlap between ALS-specific and ALS-non-specific test performances suggests these subdomains assess distinct cognitive alterations that do not always coexist, potentially reflecting a degree of independence among cognitive domains.
Screening tests identified cognitive impairments in 14% of the cohort, whereas advanced neuropsychological evaluations detected them in 41%, indicating differing sensitivities between methods. Additionally, a weak positive correlation between ECAS scores and ALS-FRS-r scores suggests that as functional impairments progress, cognitive performance may mildly decline.
These findings underscore the importance of comprehensive cognitive assessments in ALS, both to ensure early detection of impairments and to fully capture the complexity of the cognitive profiles characterizing patients with this disease.