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Cognition and motor phenotypes in ALS: a retrospective study by Edoardo Nicolò Aiello; Debora Pain; Alice Radici; Kalliopi Marinou Aktipi; Riccardo Sideri; Ildebrando Appollonio; Gabriele Mora is a Medicine article available to read on EtoBox.

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## Abstract ## Background Amyotrophic lateral sclerosis (ALS) is phenotypically heterogeneous in motor manifestations, and the extent of upper vs. lower motor neuron involvement is a widespread descriptor. This study aimed to examine cognition across different ALS motor phenotypes. ## Methods ALS patients (N = 124) were classified as classical (N = 66), bulbar (N = 13), predominant-upper motor neuron (PUMN; N = 19), and predominant-lower motor neuron (PLMN; N = 26) phenotypes. Cognition was assessed with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and function with the ALS Functional Rating Scale—Revised (ALSFRS-R). Revised ALS-FTD consensus criteria were applied for cognitive/behavioral phenotyping. ## Results Defective ECAS-total scores were detected in all groups — bulbar: 15.4%, classical: 30.3%, PLMN: 23.1%, and PUMN: 36.8%. Classical and PUMN ALS patients performed worse than PLMN ones on ECAS-total, ALS-specific, Fluency, and Executive measures. No other difference was detected. Worse ASLFRS-R scores correlated with poorer ECAS-total scores in classical ALS patients. ## Conclusions Frontotemporal cognitive deficits are more prevalent in PUMN and classical ALS a

Who reads Cognition and motor phenotypes in ALS: a retrospective study?

Author

Edoardo Nicolò Aiello; Debora Pain; Alice Radici; Kalliopi Marinou Aktipi; Riccardo Sideri; Ildebrando Appollonio; Gabriele Mora

Publisher

Springer Science and Business Media LLC

Published

2022

Language

EN

Field

Medicine (Health Sciences)