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Their onset and progression, however, is poorly understood. We explored how cognition and behaviour change over time, and whether demographic, clinical and genetic factors impact these changes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants with MND were recruited through the Phenotype-Genotype-Biomarker study. Every 3\u20136\u2009months, the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was used to assess amyotrophic lateral sclerosis (ALS) specific (executive functioning, verbal fluency, language) and ALS non-specific (memory, visuospatial) functions. Informants reported on behaviour symptoms via semi-structured interview.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Participants with neuropsychological data at \u22653 visits were included (n=237, mean age=59, 60% male), of which 18 (8%) were<jats:italic>C9ORF72<\/jats:italic>positive. Baseline cognitive impairment was apparent in 18 (8%), typically in ALS specific domains, and associated with lower education, but not<jats:italic>C9ORF72<\/jats:italic>status. Cognition, on average, remained stable over time, with two exceptions: (1)<jats:italic>C9ORF72<\/jats:italic>carriers declined in all ECAS domains, (2) 8%\u20139% of participants with baseline cognitive impairment further declined, primarily in the ALS non-specific domain, which was associated with less education. Behavioural symptoms were uncommon.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In this study, cognitive dysfunction was less common than previously reported and remained stable over time for most. However, cognition declines longitudinally in a small subset, which is not entirely related to<jats:italic>C9ORF72<\/jats:italic>status. Our findings raise questions about the timing of cognitive impairment in MND, and whether it arises during early clinically manifest disease or even prior to motor manifestations.<\/jats:p><\/jats:sec>","DOI":"10.1136\/jnnp-2023-331697","type":"journal-article","created":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T17:21:46Z","timestamp":1697131306000},"page":"jnnp-2023-331697","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Temporal course of cognitive and behavioural changes in motor neuron diseases"],"prefix":"10.1136","clinical-trial-number":[{"clinical-trial-number":"nct02327845","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0103-8580","authenticated-orcid":false,"given":"Caroline 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