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

期刊论文
发表日期：

2024年9月5日

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Barbara Poletti,
Edoardo Nicolò Aiello,
Monica Consonni,
Barbara Iazzolino,
Silvia Torre,
Federica Solca,
Veronica Faltracco,
Alessandra Telesca,
Francesca Palumbo,
Eleonora Dalla Bella,
Enrica Bersano,
Nilo Riva,
Federico Verde,
Stefano Messina,
Alberto Doretti,
Alessio Maranzano,
Claudia Morelli,
Andrea Calvo,
Vincenzo Silani,
Giuseppe Lauria,
Adriano Chiò,
Nicola Ticozzi

摘要

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.