Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage

Objective: This study aimed to establish (1) the pattern and severity of neuropsychiatric symptoms and other non-motor symptoms of sleep and mood, across ALS phenotypes in comparison to bvFTD and (2) the contribution of non-modifiable factors including age, sex and disease state to the severity of s...

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Autores principales: Emma M. Devenney, Kate McErlean, Nga Yan Tse, Jashelle Caga, Thanuja Dharmadasa, William Huynh, Colin J. Mahoney, Margaret Zoing, Srestha Mazumder, Carol Dobson-Stone, John B. Kwok, Glenda M. Halliday, John R. Hodges, Olivier Piguet, Rebekah M. Ahmed, Matthew C. Kiernan
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Publicado: Frontiers Media S.A. 2021
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spelling oai:doaj.org-article:699516c0614b4b02a87827e30181fbbd2021-12-01T02:15:08ZFactors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage1664-229510.3389/fneur.2021.743688https://doaj.org/article/699516c0614b4b02a87827e30181fbbd2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fneur.2021.743688/fullhttps://doaj.org/toc/1664-2295Objective: This study aimed to establish (1) the pattern and severity of neuropsychiatric symptoms and other non-motor symptoms of sleep and mood, across ALS phenotypes in comparison to bvFTD and (2) the contribution of non-modifiable factors including age, sex and disease state to the severity of symptoms experienced by ALS patients.Methods: Consecutive participants were recruited to the study and underwent a detailed clinical, cognitive, behavioral and neuroimaging assessment. Neuropsychiatric and other non-motor symptoms were determined using the Cambridge Behavioral Inventory, the CBI-R. The scores were converted to define impairment in terms of mild, moderate and severe symptoms for each subscale. Rate, severity and contribution of King's staging and modifiable factors were also determined and a regression model identified predictors of symptom severity.Results: In total, 250 participants (115 ALS, 98 bvFTD, and 37 ALS-FTD patients) were recruited. A similar pattern of neuropsychiatric symptom severity was identified (apathy, disinhibition and stereotypic behavior) for all behavioral phenotypes of ALS compared to bvFTD (all p > 0.05). Neuropsychiatric symptoms were also present in cases defined as ALSpure and the cognitive phenotype of ALS (ALSci) although they occurred less frequently and were at the milder end of the spectrum. Disordered sleep and disrupted mood were common across all phenotypes (all p < 0.05). The severity of sleep dysfunction was influenced by both sex and age (all p < 0.05). Neuropsychiatric symptoms, sleep and mood disorders were common early in the disease process and deteriorated in line with progression on the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R; all p < 0.05). Diagnostic phenotype, disease duration and global cognition scores were the strongest predictors of non-motor and neuropsychiatric impairments.Conclusion: The current findings reveal strikingly similar patterns of changes across the subgroups of ALS and bvFTD, supporting the concept of the ALS-FTD spectrum. The findings further highlight the impact of non-motor and neuropsychiatric symptoms in patients with ALS, that are often as severe as that seen in ALS-FTD and bvFTD. This study advances understanding across the ALS-FTD spectrum that may accelerate the early identification of patient needs, to ensure prompt recognition of symptoms and thereby to improve clinical awareness, patient care and management.Emma M. DevenneyEmma M. DevenneyKate McErleanNga Yan TseNga Yan TseJashelle CagaJashelle CagaThanuja DharmadasaThanuja DharmadasaThanuja DharmadasaWilliam HuynhWilliam HuynhWilliam HuynhColin J. MahoneyColin J. MahoneyMargaret ZoingMargaret ZoingSrestha MazumderSrestha MazumderCarol Dobson-StoneCarol Dobson-StoneJohn B. KwokJohn B. KwokGlenda M. HallidayGlenda M. HallidayJohn R. HodgesJohn R. HodgesOlivier PiguetOlivier PiguetRebekah M. AhmedRebekah M. AhmedRebekah M. AhmedMatthew C. KiernanMatthew C. KiernanMatthew C. KiernanFrontiers Media S.A.articleALS (amyotrophic lateral sclerosis)behavioral impairmentnon-motor deficitsneuropsychiatric symptomsfrontotemporal dementiaNeurology. Diseases of the nervous systemRC346-429ENFrontiers in Neurology, Vol 12 (2021)
institution DOAJ
collection DOAJ
language EN
topic ALS (amyotrophic lateral sclerosis)
behavioral impairment
non-motor deficits
neuropsychiatric symptoms
frontotemporal dementia
Neurology. Diseases of the nervous system
RC346-429
spellingShingle ALS (amyotrophic lateral sclerosis)
behavioral impairment
non-motor deficits
neuropsychiatric symptoms
frontotemporal dementia
Neurology. Diseases of the nervous system
RC346-429
Emma M. Devenney
Emma M. Devenney
Kate McErlean
Nga Yan Tse
Nga Yan Tse
Jashelle Caga
Jashelle Caga
Thanuja Dharmadasa
Thanuja Dharmadasa
Thanuja Dharmadasa
William Huynh
William Huynh
William Huynh
Colin J. Mahoney
Colin J. Mahoney
Margaret Zoing
Margaret Zoing
Srestha Mazumder
Srestha Mazumder
Carol Dobson-Stone
Carol Dobson-Stone
John B. Kwok
John B. Kwok
Glenda M. Halliday
Glenda M. Halliday
John R. Hodges
John R. Hodges
Olivier Piguet
Olivier Piguet
Rebekah M. Ahmed
Rebekah M. Ahmed
Rebekah M. Ahmed
Matthew C. Kiernan
Matthew C. Kiernan
Matthew C. Kiernan
Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage
description Objective: This study aimed to establish (1) the pattern and severity of neuropsychiatric symptoms and other non-motor symptoms of sleep and mood, across ALS phenotypes in comparison to bvFTD and (2) the contribution of non-modifiable factors including age, sex and disease state to the severity of symptoms experienced by ALS patients.Methods: Consecutive participants were recruited to the study and underwent a detailed clinical, cognitive, behavioral and neuroimaging assessment. Neuropsychiatric and other non-motor symptoms were determined using the Cambridge Behavioral Inventory, the CBI-R. The scores were converted to define impairment in terms of mild, moderate and severe symptoms for each subscale. Rate, severity and contribution of King's staging and modifiable factors were also determined and a regression model identified predictors of symptom severity.Results: In total, 250 participants (115 ALS, 98 bvFTD, and 37 ALS-FTD patients) were recruited. A similar pattern of neuropsychiatric symptom severity was identified (apathy, disinhibition and stereotypic behavior) for all behavioral phenotypes of ALS compared to bvFTD (all p > 0.05). Neuropsychiatric symptoms were also present in cases defined as ALSpure and the cognitive phenotype of ALS (ALSci) although they occurred less frequently and were at the milder end of the spectrum. Disordered sleep and disrupted mood were common across all phenotypes (all p < 0.05). The severity of sleep dysfunction was influenced by both sex and age (all p < 0.05). Neuropsychiatric symptoms, sleep and mood disorders were common early in the disease process and deteriorated in line with progression on the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R; all p < 0.05). Diagnostic phenotype, disease duration and global cognition scores were the strongest predictors of non-motor and neuropsychiatric impairments.Conclusion: The current findings reveal strikingly similar patterns of changes across the subgroups of ALS and bvFTD, supporting the concept of the ALS-FTD spectrum. The findings further highlight the impact of non-motor and neuropsychiatric symptoms in patients with ALS, that are often as severe as that seen in ALS-FTD and bvFTD. This study advances understanding across the ALS-FTD spectrum that may accelerate the early identification of patient needs, to ensure prompt recognition of symptoms and thereby to improve clinical awareness, patient care and management.
format article
author Emma M. Devenney
Emma M. Devenney
Kate McErlean
Nga Yan Tse
Nga Yan Tse
Jashelle Caga
Jashelle Caga
Thanuja Dharmadasa
Thanuja Dharmadasa
Thanuja Dharmadasa
William Huynh
William Huynh
William Huynh
Colin J. Mahoney
Colin J. Mahoney
Margaret Zoing
Margaret Zoing
Srestha Mazumder
Srestha Mazumder
Carol Dobson-Stone
Carol Dobson-Stone
John B. Kwok
John B. Kwok
Glenda M. Halliday
Glenda M. Halliday
John R. Hodges
John R. Hodges
Olivier Piguet
Olivier Piguet
Rebekah M. Ahmed
Rebekah M. Ahmed
Rebekah M. Ahmed
Matthew C. Kiernan
Matthew C. Kiernan
Matthew C. Kiernan
author_facet Emma M. Devenney
Emma M. Devenney
Kate McErlean
Nga Yan Tse
Nga Yan Tse
Jashelle Caga
Jashelle Caga
Thanuja Dharmadasa
Thanuja Dharmadasa
Thanuja Dharmadasa
William Huynh
William Huynh
William Huynh
Colin J. Mahoney
Colin J. Mahoney
Margaret Zoing
Margaret Zoing
Srestha Mazumder
Srestha Mazumder
Carol Dobson-Stone
Carol Dobson-Stone
John B. Kwok
John B. Kwok
Glenda M. Halliday
Glenda M. Halliday
John R. Hodges
John R. Hodges
Olivier Piguet
Olivier Piguet
Rebekah M. Ahmed
Rebekah M. Ahmed
Rebekah M. Ahmed
Matthew C. Kiernan
Matthew C. Kiernan
Matthew C. Kiernan
author_sort Emma M. Devenney
title Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage
title_short Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage
title_full Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage
title_fullStr Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage
title_full_unstemmed Factors That Influence Non-Motor Impairment Across the ALS-FTD Spectrum: Impact of Phenotype, Sex, Age, Onset and Disease Stage
title_sort factors that influence non-motor impairment across the als-ftd spectrum: impact of phenotype, sex, age, onset and disease stage
publisher Frontiers Media S.A.
publishDate 2021
url https://doaj.org/article/699516c0614b4b02a87827e30181fbbd
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