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Distinct disease trajectories in frontotemporal dementia–motor neuron disease and behavioural variant frontotemporal dementia: A longitudinal study.

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Title: Distinct disease trajectories in frontotemporal dementia–motor neuron disease and behavioural variant frontotemporal dementia: A longitudinal study.
Authors: Long, Zhe (AUTHOR), Irish, Muireann (AUTHOR), Hodges, John R. (AUTHOR), Piguet, Olivier (AUTHOR), Burrell, James R. (AUTHOR)
Source: European Journal of Neurology. Nov2022, Vol. 29 Issue 11, p3158-3169. 12p.
Subjects: Frontotemporal dementia, Cerebral atrophy, Voxel-based morphometry, Longitudinal method, Neurons, Frontotemporal lobar degeneration
Abstract: Background and purpose: The heterogeneity of cognitive and behavioural disturbances in frontotemporal dementia–motor neuron disease (FTD‐MND), and clinical differences between FTD‐MND and FTD subtypes, have been illustrated cross‐sectionally. This study aimed to examine the FTD‐MND disease trajectory by comparing clinical features of FTD‐MND and the behavioural variant FTD (bvFTD) longitudinally. Methods: Neuropsychological and disease severity assessments were conducted in a cohort of FTD‐MND (baseline, n = 42; follow‐up, n = 18) and bvFTD (baseline, n = 116; follow‐up, n = 111) using a longitudinal, case–control design. Age‐, sex‐, and education‐matched controls (n = 52) were recruited. Predictors of clinical progression were analyzed. Voxel‐based morphometry analysis was undertaken to investigate the progression of brain atrophy. Results: At baseline, FTD‐MND was characterized by semantic and general cognition deficits, whereas bvFTD had greater behavioural disturbances. General cognition and language deteriorated in FTD‐MND when followed longitudinally. Language deficits at baseline predicted cognitive deterioration and disease progression and correlated with progressive atrophy of language regions. Further deterioration in behaviour was evident in bvFTD over time. The rate of disease progression (i.e., general cognition, semantic association, and disease severity) was significantly faster in FTD‐MND than in bvFTD. Conclusions: FTD‐MND and bvFTD appear to have distinct disease trajectories, with more rapid progression in FTD‐MND. Language impairments should be closely monitored in FTD‐MND as potential predictors of cognitive deterioration and disease progression. [ABSTRACT FROM AUTHOR]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Label: Title
  Group: Ti
  Data: Distinct disease trajectories in frontotemporal dementia–motor neuron disease and behavioural variant frontotemporal dementia: A longitudinal study.
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  Data: <searchLink fieldCode="AR" term="%22Long%2C+Zhe%22">Long, Zhe</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Irish%2C+Muireann%22">Irish, Muireann</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Hodges%2C+John+R%2E%22">Hodges, John R.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Piguet%2C+Olivier%22">Piguet, Olivier</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Burrell%2C+James+R%2E%22">Burrell, James R.</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22European+Journal+of+Neurology%22">European Journal of Neurology</searchLink>. Nov2022, Vol. 29 Issue 11, p3158-3169. 12p.
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  Data: <searchLink fieldCode="DE" term="%22Frontotemporal+dementia%22">Frontotemporal dementia</searchLink><br /><searchLink fieldCode="DE" term="%22Cerebral+atrophy%22">Cerebral atrophy</searchLink><br /><searchLink fieldCode="DE" term="%22Voxel-based+morphometry%22">Voxel-based morphometry</searchLink><br /><searchLink fieldCode="DE" term="%22Longitudinal+method%22">Longitudinal method</searchLink><br /><searchLink fieldCode="DE" term="%22Neurons%22">Neurons</searchLink><br /><searchLink fieldCode="DE" term="%22Frontotemporal+lobar+degeneration%22">Frontotemporal lobar degeneration</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background and purpose: The heterogeneity of cognitive and behavioural disturbances in frontotemporal dementia–motor neuron disease (FTD‐MND), and clinical differences between FTD‐MND and FTD subtypes, have been illustrated cross‐sectionally. This study aimed to examine the FTD‐MND disease trajectory by comparing clinical features of FTD‐MND and the behavioural variant FTD (bvFTD) longitudinally. Methods: Neuropsychological and disease severity assessments were conducted in a cohort of FTD‐MND (baseline, n = 42; follow‐up, n = 18) and bvFTD (baseline, n = 116; follow‐up, n = 111) using a longitudinal, case–control design. Age‐, sex‐, and education‐matched controls (n = 52) were recruited. Predictors of clinical progression were analyzed. Voxel‐based morphometry analysis was undertaken to investigate the progression of brain atrophy. Results: At baseline, FTD‐MND was characterized by semantic and general cognition deficits, whereas bvFTD had greater behavioural disturbances. General cognition and language deteriorated in FTD‐MND when followed longitudinally. Language deficits at baseline predicted cognitive deterioration and disease progression and correlated with progressive atrophy of language regions. Further deterioration in behaviour was evident in bvFTD over time. The rate of disease progression (i.e., general cognition, semantic association, and disease severity) was significantly faster in FTD‐MND than in bvFTD. Conclusions: FTD‐MND and bvFTD appear to have distinct disease trajectories, with more rapid progression in FTD‐MND. Language impairments should be closely monitored in FTD‐MND as potential predictors of cognitive deterioration and disease progression. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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              Text: Nov2022
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