Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Oct;271(10):6944-6955.
doi: 10.1007/s00415-024-12658-w. Epub 2024 Sep 5.

Prevalence and motor-functional correlates of frontotemporal-spectrum disorders in a large cohort of non-demented ALS patients

Barbara Poletti #  1   2 , Edoardo Nicolò Aiello #  3 , Monica Consonni #  4 , Barbara Iazzolino #  5 , Silvia Torre  3 , Federica Solca  3 , Veronica Faltracco  4 , Alessandra Telesca  6   7 , Francesca Palumbo  5 , Eleonora Dalla Bella  4 , Enrica Bersano  4   8 , Nilo Riva  4 , Federico Verde  3   9 , Stefano Messina  3 , Alberto Doretti  3 , Alessio Maranzano  3 , Claudia Morelli  3 , Andrea Calvo  5   10 , Vincenzo Silani #  3   9 , Giuseppe Lauria #  4   8 , Adriano Chiò #  5   10   11 , Nicola Ticozzi #  3   9
Affiliations

Prevalence and motor-functional correlates of frontotemporal-spectrum disorders in a large cohort of non-demented ALS patients

Barbara Poletti et al. J Neurol. 2024 Oct.

Abstract

Background: This study aimed at (1) delivering generalizable estimates of the prevalence of frontotemporal-spectrum disorders (FTSDs) in non-demented ALS patients and (2) exploring their motor-functional correlates.

Methods: N = 808 ALS patients without FTD were assessed for motor-functional outcomes-i.e., disease duration, severity (ALSFRS-R), progression rate (ΔFS), and stage (King's and Milano-Torino-MiToS-systems)-cognition-via the cognitive section of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)-and behaviour-via the ECAS-Carer Interview. Neuropsychological phenotypes were retrieved via Strong's revised criteria-i.e., ALS cognitively and behaviourally normal (ALScbn) or cognitively and/or behaviourally impaired (ALSci/bi/cbi).

Results: Defective ECAS-Total performances were detected in ~ 29% of patients, with the ECAS-Executive being failed by the highest number of patients (~ 30%), followed by the ECAS-Language, -Fluency, and -Memory (~ 15-17%) and -Visuospatial (~ %8). Apathy was the most frequent behavioural change (~ 28%), followed by loss of sympathy/empathy (~ 13%); remaining symptoms were reported in < 4% of patients. The distribution of Strong's classifications was as follows: ALScbn: 46.7%; ALSci/bi/cbi: 22.9%/20.0%/10.4%. Multinomial regressions on Strong's classifications revealed that lower ALSFRS-R scores were associated with a higher probability of ALSbi and ALScbi classifications (p ≤ .008). Higher King's and MiToS stages were associated with a higher probability of ALSbi classification (p ≤ .031).

Conclusions: FTSDs affect ~ 50% of non-demented ALS patients, with cognitive deficits being as frequent as behavioural changes. A higher degree of motor-functional involvement is associated with worse behavioural outcomes-with this link being weaker for cognitive deficits.

Keywords: Amyotrophic lateral sclerosis; Epidemiology; Frontotemporal degeneration; Neuropsychology.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Abrahams S (2023) Neuropsychological impairment in amyotrophic lateral sclerosis–frontotemporal-spectrum disorder. Nat Rev Neurol 19:655–667 - PubMed - DOI
    1. Strong MJ, Abrahams S, Goldstein LH et al (2017) Amyotrophic lateral sclerosis-frontotemporal-spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener 18:153–174 - PubMed - PMC - DOI
    1. Raaphorst J, De Visser M, Linssen WH, De Haan RJ, Schmand B (2010) The cognitive profile of amyotrophic lateral sclerosis: a meta-analysis. Amyotroph Lateral Scler 11:27–37 - PubMed - DOI
    1. Beeldman E, Raaphorst J, Twennaar MK, de Visser M, Schmand BA, de Haan RJ (2016) The cognitive profile of ALS: a systematic review and meta-analysis update. J Neurol Neurosurg Psychiatry 87:611–619 - PubMed - DOI
    1. Finsel J, Uttner I, Vázquez Medrano CR, Ludolph AC, Lulé D (2023) Cognition in the course of ALS—a meta-analysis. Amyotroph Lateral Scler Frontotemporal Degener 24:2–13 - PubMed - DOI

LinkOut - more resources