Journal Article

Bulbar involvement and cognitive features in amyotrophic lateral sclerosis: a retrospective study on 347 patients

Treddenti, Mauro, Poletti, Barbara, De Lorenzo, Alberto, Patisso, Valerio, Aiello, Edoardo Nicolò, Colombo, Eleonora, Morelli, Claudia, Doretti, Alberto, Maranzano, Alessio, Verde, Federico, Solca, Federica, Silani, Vincenzo, Ticozzi, Nicola, Torre, Silvia

2023

Overview

This study aimed at clarifying the role of bulbar involvement (BI) as a risk factor for cognitive impairment (CI) in non-demented amyotrophic lateral sclerosis (ALS) patients. Data on = 347 patients were retrospectively collected. Cognition was assessed via the Edinburgh Cognitive and Behavioral ALS Screen (ECAS). On the basis of clinical records and ALS Functional Rating Scale-Revised (ALSFRS-R) scores, BI was characterized as follows: (1) BI at onset-from medical history; (2) BI at testing (an ALSFRS-R-Bulbar score ≤11); (3) dysarthria (a score ≤3 on item 1 of the ALSFRS-R); (4) severity of BI (the total score on the ALSFRS-R-Bulbar); and (5) progression rate of BI (computed as 12-ALSFRS-R-Bulbar/disease duration in months). Logistic regressions were run to predict a below- vs. above-cutoff performance on each ECAS measure based on BI-related features while accounting for sex, disease duration, severity and progression rate of respiratory and spinal involvement and ECAS response modality. No predictors yielded significance either on the ECAS-Total and -ALS-non-specific or on ECAS-Language/-Fluency or -Visuospatial subscales. BI at testing predicted a higher probability of an abnormal performance on the ECAS-ALS-specific ( = 0.035) and ECAS-Executive Functioning ( = 0.018). Lower ALSFRS-R-Bulbar scores were associated with a defective performance on the ECAS-Memory ( = 0.025). No other BI-related features affected other ECAS performances. In ALS, the occurrence of BI itself, while neither its specific features nor its presence at onset, might selectively represent a risk factor for executive impairment, whilst its severity might be associated with memory deficits.

• Publisher: Frontiers Media SA,Frontiers Media S.A
• Subject: Amyotrophic lateral sclerosis / bulbar / cognition / Cognition & reasoning / Cognitive ability / Dysarthria / Executive function / Frontotemporal Degeneration / Memory / neuropsychology / Neuroscience / Patients / Regression analysis / Respiration / Risk factors