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Cognition and motor phenotypes in ALS: a retrospective study

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)...

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Autores principales: Aiello, Edoardo Nicolò, Pain, Debora, Radici, Alice, Aktipi, Kalliopi Marinou, Sideri, Riccardo, Appollonio, Ildebrando, Mora, Gabriele
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9385798/
https://www.ncbi.nlm.nih.gov/pubmed/35608739
http://dx.doi.org/10.1007/s10072-022-06157-x
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author Aiello, Edoardo Nicolò
Pain, Debora
Radici, Alice
Aktipi, Kalliopi Marinou
Sideri, Riccardo
Appollonio, Ildebrando
Mora, Gabriele
author_facet Aiello, Edoardo Nicolò
Pain, Debora
Radici, Alice
Aktipi, Kalliopi Marinou
Sideri, Riccardo
Appollonio, Ildebrando
Mora, Gabriele
author_sort Aiello, Edoardo Nicolò
collection PubMed
description 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 and linked to disease severity in the latter, but occur also in PLMN phenotypes. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-022-06157-x
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spelling pubmed-93857982022-08-19 Cognition and motor phenotypes in ALS: a retrospective study Aiello, Edoardo Nicolò Pain, Debora Radici, Alice Aktipi, Kalliopi Marinou Sideri, Riccardo Appollonio, Ildebrando Mora, Gabriele Neurol Sci Original Article 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 and linked to disease severity in the latter, but occur also in PLMN phenotypes. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-022-06157-x Springer International Publishing 2022-05-24 2022 /pmc/articles/PMC9385798/ /pubmed/35608739 http://dx.doi.org/10.1007/s10072-022-06157-x Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Aiello, Edoardo Nicolò
Pain, Debora
Radici, Alice
Aktipi, Kalliopi Marinou
Sideri, Riccardo
Appollonio, Ildebrando
Mora, Gabriele
Cognition and motor phenotypes in ALS: a retrospective study
title Cognition and motor phenotypes in ALS: a retrospective study
title_full Cognition and motor phenotypes in ALS: a retrospective study
title_fullStr Cognition and motor phenotypes in ALS: a retrospective study
title_full_unstemmed Cognition and motor phenotypes in ALS: a retrospective study
title_short Cognition and motor phenotypes in ALS: a retrospective study
title_sort cognition and motor phenotypes in als: a retrospective study
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9385798/
https://www.ncbi.nlm.nih.gov/pubmed/35608739
http://dx.doi.org/10.1007/s10072-022-06157-x
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