Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.

<h4>Objective</h4>Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually...

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Autores principales:	Shan Ye, Pingping Jin, Lu Chen, Nan Zhang, Dongsheng Fan
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/aacbe3f2766c4e279af49ac21098461f
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spelling	oai:doaj.org-article:aacbe3f2766c4e279af49ac21098461f2021-12-02T20:18:26ZPrognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.1932-620310.1371/journal.pone.0253279https://doaj.org/article/aacbe3f2766c4e279af49ac21098461f2021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0253279https://doaj.org/toc/1932-6203<h4>Objective</h4>Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort.<h4>Methods</h4>A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy.<h4>Results</h4>There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale-Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01).<h4>Conclusion</h4>ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.Shan YePingping JinLu ChenNan ZhangDongsheng FanPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 8, p e0253279 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Shan Ye Pingping Jin Lu Chen Nan Zhang Dongsheng Fan Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
description	<h4>Objective</h4>Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort.<h4>Methods</h4>A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy.<h4>Results</h4>There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale-Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01).<h4>Conclusion</h4>ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.
format	article
author	Shan Ye Pingping Jin Lu Chen Nan Zhang Dongsheng Fan
author_facet	Shan Ye Pingping Jin Lu Chen Nan Zhang Dongsheng Fan
author_sort	Shan Ye
title	Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
title_short	Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
title_full	Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
title_fullStr	Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
title_full_unstemmed	Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
title_sort	prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
publisher	Public Library of Science (PLoS)
publishDate	2021
url	https://doaj.org/article/aacbe3f2766c4e279af49ac21098461f
work_keys_str_mv	AT shanye prognosisofamyotrophiclateralsclerosiswithcognitiveandbehaviouralchangesbasedonasixtymonthlongitudinalfollowup AT pingpingjin prognosisofamyotrophiclateralsclerosiswithcognitiveandbehaviouralchangesbasedonasixtymonthlongitudinalfollowup AT luchen prognosisofamyotrophiclateralsclerosiswithcognitiveandbehaviouralchangesbasedonasixtymonthlongitudinalfollowup AT nanzhang prognosisofamyotrophiclateralsclerosiswithcognitiveandbehaviouralchangesbasedonasixtymonthlongitudinalfollowup AT dongshengfan prognosisofamyotrophiclateralsclerosiswithcognitiveandbehaviouralchangesbasedonasixtymonthlongitudinalfollowup
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Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.

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