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Objective : The goal of this meta-analysis is to improve insight into the development of cognition over the course of ALS and to assess predictors of cognitive performance. Method : A literature search was conducted in Pubmed and Web of Science on 29 July 2019 and 16 March 2021. Data were screened in Endnote ®  Version X9 (London, UK). Meta-analyses and meta-regressions were calculated for cross-sectional data using Rstudio ® . Studies were assigned to temporal and physical categories and Hedges’ g was calculated for the respective categories to provide an estimate of a cognitive course based on cross-sectional data. Due to low numbers and heterogeneity in reporting, longitudinal studies were analyzed descriptively. Results : A total of  N  = 45 cross-sectional and  N  = 13 longitudinal studies were included. Impairments in all cognitive domains, except verbal IQ, were found in ALS patients (PALS). PALS showed stable cognitive performances in cross-sectional and in most longitudinal studies. PALS with symptoms for 18–24 months and PALS who had an ALSFRS-R score of 40–36 were the most frequently reported subgroup regarding neuropsychology. Age was related to visuospatial functioning, and depressiveness to attention. In longitudinal studies, impact of site of onset and cognitive status at baseline on cognitive course was found. Conclusion : Despite vast evidence for cognitive impairment at disease onset in different domains, evidence for evolution of these deficits is rather limited, suggesting that PALS present with cognitive impairment early in the course possibly in a sense of disease trait.

Cognition in the course of ALS—a meta-analysis