Decline of cognitive and behavioral functions in amyotrophic lateral sclerosis : a longitudinal study
Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30-50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5-9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression.Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation.Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment.Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:21 |
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| Enthalten in: |
Amyotrophic lateral sclerosis & frontotemporal degeneration - 21(2020), 5-6 vom: 01. Aug., Seite 373-379 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bersano, Enrica [VerfasserIn] |
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| Links: |
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| Themen: |
Amyotrophic Lateral Sclerosis |
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| Anmerkungen: |
Date Completed 31.08.2021 Date Revised 29.01.2022 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1080/21678421.2020.1771732 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM310649854 |
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| 520 | |a Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30-50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5-9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression.Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation.Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment.Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a Amyotrophic Lateral Sclerosis | |
| 650 | 4 | |a cognitive impairment | |
| 650 | 4 | |a fronto-temporal dementia | |
| 650 | 4 | |a longitudinal study | |
| 700 | 1 | |a Sarnelli, Maria Francesca |e verfasserin |4 aut | |
| 700 | 1 | |a Solara, Valentina |e verfasserin |4 aut | |
| 700 | 1 | |a Iazzolino, Barbara |e verfasserin |4 aut | |
| 700 | 1 | |a Peotta, Laura |e verfasserin |4 aut | |
| 700 | 1 | |a De Marchi, Fabiola |e verfasserin |4 aut | |
| 700 | 1 | |a Facchin, Alessio |e verfasserin |4 aut | |
| 700 | 1 | |a Moglia, Cristina |e verfasserin |4 aut | |
| 700 | 1 | |a Canosa, Antonio |e verfasserin |4 aut | |
| 700 | 1 | |a Calvo, Andrea |e verfasserin |4 aut | |
| 700 | 1 | |a Chiò, Adriano |e verfasserin |4 aut | |
| 700 | 1 | |a Mazzini, Letizia |e verfasserin |4 aut | |
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