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.

Poletti, B., Aiello, E., Consonni, M., Iazzolino, B., Torre, S., Solca, F., et al. (2024). Prevalence and motor-functional correlates of frontotemporal-spectrum disorders in a large cohort of non-demented ALS patients. JOURNAL OF NEUROLOGY, 271(10), 6944-6955 [10.1007/s00415-024-12658-w].

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

Aiello E. N.;
2024

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.
Articolo in rivista - Articolo scientifico
Amyotrophic lateral sclerosis; Epidemiology; Frontotemporal degeneration; Neuropsychology;
English
5-set-2024
2024
271
10
6944
6955
none
Poletti, B., Aiello, E., Consonni, M., Iazzolino, B., Torre, S., Solca, F., et al. (2024). Prevalence and motor-functional correlates of frontotemporal-spectrum disorders in a large cohort of non-demented ALS patients. JOURNAL OF NEUROLOGY, 271(10), 6944-6955 [10.1007/s00415-024-12658-w].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/520822
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