Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia
Phenocopy frontotemporal dementia (phFTD) shares core characteristics with behavioral variant frontotemporal dementia (bvFTD), yet without associated cognitive deficits and brain abnormalities on conventional magnetic resonance imaging (MRI), and without progression. Using advanced MRI techniques, w...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2019-11-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fneur.2019.01207/full |
| _version_ | 1811193005250445312 |
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| author | Rozanna Meijboom Rozanna Meijboom Rebecca M. E. Steketee Lize C. Jiskoot Lize C. Jiskoot Esther E. Bron Aad van der Lugt John C. van Swieten Marion Smits |
| author_facet | Rozanna Meijboom Rozanna Meijboom Rebecca M. E. Steketee Lize C. Jiskoot Lize C. Jiskoot Esther E. Bron Aad van der Lugt John C. van Swieten Marion Smits |
| author_sort | Rozanna Meijboom |
| collection | DOAJ |
| description | Phenocopy frontotemporal dementia (phFTD) shares core characteristics with behavioral variant frontotemporal dementia (bvFTD), yet without associated cognitive deficits and brain abnormalities on conventional magnetic resonance imaging (MRI), and without progression. Using advanced MRI techniques, we previously observed subtle structural and functional brain changes in phFTD similar to bvFTD. The aim of the current study was to follow these as well as cognition in phFTD over time, by means of a descriptive case series. Cognition, gray matter (GM) volume and white matter (WM) microstructure, and perfusion of 6 phFTD patients were qualitatively compared longitudinally (3-years follow-up), and cross-sectionally with baseline data from 9 bvFTD patients and 17 controls. For functional brain changes, arterial spin labeling (ASL) was performed to assess GM perfusion. For structural brain changes, diffusion tensor imaging was performed to assess WM microstructure and T1w imaging to assess GM volume. MRI acquisition was performed at 3T (General Electric, US). Clinical profiles of phFTD cases at follow-up are described. At follow-up phFTD patients showed clinical symptomatology similar to bvFTD, but had a relatively stable clinical profile. Longitudinal qualitative comparisons in phFTD showed some deterioration of language and memory function, a stable pattern of structural brain abnormalities and increased perfusion over time. Additionally, both baseline and follow-up cognitive scores and structural values in phFTD were generally in between those of controls and bvFTD. Although a descriptive case series does not allow for strong conclusions, these observations in a unique longitudinal phFTD patient cohort are suggestive of the notion that phFTD and bvFTD may belong to the same disease spectrum. They may also provide a basis for further longitudinal studies in phFTD, specifically exploring the structural vs. functional brain changes. Such studies are essential for improved insight, accurate diagnosis, and appropriate treatment of phFTD. |
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| format | Article |
| id | doaj.art-eefc834e61714769b4393115f50a233b |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-12T00:01:10Z |
| publishDate | 2019-11-01 |
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| series | Frontiers in Neurology |
| spelling | doaj.art-eefc834e61714769b4393115f50a233b2022-12-22T03:56:14ZengFrontiers Media S.A.Frontiers in Neurology1664-22952019-11-011010.3389/fneur.2019.01207480034Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal DementiaRozanna Meijboom0Rozanna Meijboom1Rebecca M. E. Steketee2Lize C. Jiskoot3Lize C. Jiskoot4Esther E. Bron5Aad van der Lugt6John C. van Swieten7Marion Smits8Department of Radiology and Nuclear Medicine, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsCenter for Clinical Brain Sciences, University of Edinburgh, Edinburgh, United KingdomDepartment of Radiology and Nuclear Medicine, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsDepartment of Neurology, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsDepartment of Radiology, Leiden University Medical Center, Leiden, NetherlandsBiomedical Imaging Group Rotterdam, Departments of Medical Informatics and Radiology, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsDepartment of Radiology and Nuclear Medicine, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsDepartment of Neurology, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsDepartment of Radiology and Nuclear Medicine, Erasmus MC–University Medical Center Rotterdam, Rotterdam, NetherlandsPhenocopy frontotemporal dementia (phFTD) shares core characteristics with behavioral variant frontotemporal dementia (bvFTD), yet without associated cognitive deficits and brain abnormalities on conventional magnetic resonance imaging (MRI), and without progression. Using advanced MRI techniques, we previously observed subtle structural and functional brain changes in phFTD similar to bvFTD. The aim of the current study was to follow these as well as cognition in phFTD over time, by means of a descriptive case series. Cognition, gray matter (GM) volume and white matter (WM) microstructure, and perfusion of 6 phFTD patients were qualitatively compared longitudinally (3-years follow-up), and cross-sectionally with baseline data from 9 bvFTD patients and 17 controls. For functional brain changes, arterial spin labeling (ASL) was performed to assess GM perfusion. For structural brain changes, diffusion tensor imaging was performed to assess WM microstructure and T1w imaging to assess GM volume. MRI acquisition was performed at 3T (General Electric, US). Clinical profiles of phFTD cases at follow-up are described. At follow-up phFTD patients showed clinical symptomatology similar to bvFTD, but had a relatively stable clinical profile. Longitudinal qualitative comparisons in phFTD showed some deterioration of language and memory function, a stable pattern of structural brain abnormalities and increased perfusion over time. Additionally, both baseline and follow-up cognitive scores and structural values in phFTD were generally in between those of controls and bvFTD. Although a descriptive case series does not allow for strong conclusions, these observations in a unique longitudinal phFTD patient cohort are suggestive of the notion that phFTD and bvFTD may belong to the same disease spectrum. They may also provide a basis for further longitudinal studies in phFTD, specifically exploring the structural vs. functional brain changes. Such studies are essential for improved insight, accurate diagnosis, and appropriate treatment of phFTD.https://www.frontiersin.org/article/10.3389/fneur.2019.01207/fullfrontotemporal dementiadiffusion tensor imagingwhite mattercognitionperfusiongray matter |
| spellingShingle | Rozanna Meijboom Rozanna Meijboom Rebecca M. E. Steketee Lize C. Jiskoot Lize C. Jiskoot Esther E. Bron Aad van der Lugt John C. van Swieten Marion Smits Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia Frontiers in Neurology frontotemporal dementia diffusion tensor imaging white matter cognition perfusion gray matter |
| title | Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia |
| title_full | Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia |
| title_fullStr | Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia |
| title_full_unstemmed | Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia |
| title_short | Qualitative Assessment of Longitudinal Changes in Phenocopy Frontotemporal Dementia |
| title_sort | qualitative assessment of longitudinal changes in phenocopy frontotemporal dementia |
| topic | frontotemporal dementia diffusion tensor imaging white matter cognition perfusion gray matter |
| url | https://www.frontiersin.org/article/10.3389/fneur.2019.01207/full |
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