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Open Access 09-06-2024 | Amyotrophic Lateral Sclerosis | Original Communication

Repeated cognitive assessments show stable function over time in patients with ALS

Authors: Linn Öijerstedt, Juliette Foucher, Anikó Lovik, Solmaz Yazdani, Alexander Juto, Ulf Kläppe, Fang Fang, Caroline Ingre

Published in: Journal of Neurology

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Abstract

Background

Amyotrophic lateral sclerosis (ALS) is a multisystem disorder with not only motor symptoms but also extra-motor features including cognitive impairment. The most common cognitive profile observed in patients with ALS includes deficits in executive function, language, and social cognition. However, longitudinal studies on cognitive changes over time in ALS are sparse. We aimed to investigate the presence and nature of cognitive impairment at the time of ALS diagnosis and its association with survival as well as explore longitudinal cognitive change.

Method

Patients (n = 216) were recruited at the Karolinska University Hospital in Stockholm, Sweden. Follow-up visits (n = 307 in total) were performed every 6 months. Cognitive impairment was assessed using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and/or Montreal Cognitive Assessment (MoCA).

Results

Cognitive impairment was observed in 38% of the patients at the time of ALS diagnosis, and the majority of these patients had deficits in executive function and/or language. Patients with cognitive impairment at the time of diagnosis had a more rapid decline in ALSFRS-R at 12- and 18-months follow-up, and a shorter survival. Cognitive function was stable during the first 2 years after diagnosis, and did not follow the trajectories of decline in motor functions.

Conclusion

Cognitive impairment in ALS was associated with a faster decline of motor functions, and shorter survival. However, cognitive function did not deteriorate over time. Cognitive assessment is essential for the patients and caregivers to understand the phenotypic expression of ALS.
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Metadata
Title
Repeated cognitive assessments show stable function over time in patients with ALS
Authors
Linn Öijerstedt
Juliette Foucher
Anikó Lovik
Solmaz Yazdani
Alexander Juto
Ulf Kläppe
Fang Fang
Caroline Ingre
Publication date
09-06-2024
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-024-12479-x