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The frontotemporal syndrome of ALS is associated with poor survival
Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the F...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5110703/ https://www.ncbi.nlm.nih.gov/pubmed/27671483 http://dx.doi.org/10.1007/s00415-016-8290-1 |
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author | Govaarts, Rosanne Beeldman, Emma Kampelmacher, Mike J. van Tol, Marie-Jose van den Berg, Leonard H. van der Kooi, Anneke J. Wijkstra, Peter J. Zijnen-Suyker, Marianne Cobben, Nicolle A. M. Schmand, Ben A. de Haan, Rob J. de Visser, Marianne Raaphorst, Joost |
author_facet | Govaarts, Rosanne Beeldman, Emma Kampelmacher, Mike J. van Tol, Marie-Jose van den Berg, Leonard H. van der Kooi, Anneke J. Wijkstra, Peter J. Zijnen-Suyker, Marianne Cobben, Nicolle A. M. Schmand, Ben A. de Haan, Rob J. de Visser, Marianne Raaphorst, Joost |
author_sort | Govaarts, Rosanne |
collection | PubMed |
description | Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were defined as >22 points on the ALS-Frontotemporal-Dementia-Questionnaire or ≥3 points on ≥2 items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on ≥2 tests of executive function, memory or language. Classic ALS was defined as ALS without the frontotemporal syndrome. We performed survival analyses from symptom onset and time from NIV initiation, respectively, to death. The impact of the explanatory variables on survival and NIV initiation were examined using Cox proportional hazards models. We included 110 ALS patients (76 men) with a mean age of 62 years. Median survival time was 4.3 years (95 % CI 3.53–5.13). Forty-seven patients (43 %) had an FS. Factors associated with shorter survival were FS, bulbar onset, older age at onset, short time to diagnosis and a C9orf72 repeat expansion. The adjusted hazard ratio (HR) for the FS was 2.29 (95 % CI 1.44–3.65, p < 0.001) in a multivariate model. Patients with an FS had a shorter survival after NIV initiation (adjusted HR 2.70, 95 % CI 1.04–4.67, p = 0.04). In conclusion, there is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV. |
format | Online Article Text |
id | pubmed-5110703 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-51107032016-11-29 The frontotemporal syndrome of ALS is associated with poor survival Govaarts, Rosanne Beeldman, Emma Kampelmacher, Mike J. van Tol, Marie-Jose van den Berg, Leonard H. van der Kooi, Anneke J. Wijkstra, Peter J. Zijnen-Suyker, Marianne Cobben, Nicolle A. M. Schmand, Ben A. de Haan, Rob J. de Visser, Marianne Raaphorst, Joost J Neurol Original Communication Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were defined as >22 points on the ALS-Frontotemporal-Dementia-Questionnaire or ≥3 points on ≥2 items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on ≥2 tests of executive function, memory or language. Classic ALS was defined as ALS without the frontotemporal syndrome. We performed survival analyses from symptom onset and time from NIV initiation, respectively, to death. The impact of the explanatory variables on survival and NIV initiation were examined using Cox proportional hazards models. We included 110 ALS patients (76 men) with a mean age of 62 years. Median survival time was 4.3 years (95 % CI 3.53–5.13). Forty-seven patients (43 %) had an FS. Factors associated with shorter survival were FS, bulbar onset, older age at onset, short time to diagnosis and a C9orf72 repeat expansion. The adjusted hazard ratio (HR) for the FS was 2.29 (95 % CI 1.44–3.65, p < 0.001) in a multivariate model. Patients with an FS had a shorter survival after NIV initiation (adjusted HR 2.70, 95 % CI 1.04–4.67, p = 0.04). In conclusion, there is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV. Springer Berlin Heidelberg 2016-09-26 2016 /pmc/articles/PMC5110703/ /pubmed/27671483 http://dx.doi.org/10.1007/s00415-016-8290-1 Text en © The Author(s) 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Original Communication Govaarts, Rosanne Beeldman, Emma Kampelmacher, Mike J. van Tol, Marie-Jose van den Berg, Leonard H. van der Kooi, Anneke J. Wijkstra, Peter J. Zijnen-Suyker, Marianne Cobben, Nicolle A. M. Schmand, Ben A. de Haan, Rob J. de Visser, Marianne Raaphorst, Joost The frontotemporal syndrome of ALS is associated with poor survival |
title | The frontotemporal syndrome of ALS is associated with poor survival |
title_full | The frontotemporal syndrome of ALS is associated with poor survival |
title_fullStr | The frontotemporal syndrome of ALS is associated with poor survival |
title_full_unstemmed | The frontotemporal syndrome of ALS is associated with poor survival |
title_short | The frontotemporal syndrome of ALS is associated with poor survival |
title_sort | frontotemporal syndrome of als is associated with poor survival |
topic | Original Communication |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5110703/ https://www.ncbi.nlm.nih.gov/pubmed/27671483 http://dx.doi.org/10.1007/s00415-016-8290-1 |
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