Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort
© 2020 the Alzheimer's Association..
INTRODUCTION: Behavioral variant frontotemporal dementia (bvFTD) may present sporadically or due to an autosomal dominant mutation. Characterization of both forms will improve understanding of the generalizability of assessments and treatments.
METHODS: A total of 135 sporadic (s-bvFTD; mean age 63.3 years; 34% female) and 99 familial (f-bvFTD; mean age 59.9; 48% female) bvFTD participants were identified. f-bvFTD cases included 43 with known or presumed chromosome 9 open reading frame 72 (C9orf72) gene expansions, 28 with known or presumed microtubule-associated protein tau (MAPT) mutations, 14 with known progranulin (GRN) mutations, and 14 with a strong family history of FTD but no identified mutation.
RESULTS: Participants with f-bvFTD were younger and had earlier age at onset. s-bvFTD had higher total Neuropsychiatric Inventory Questionnaire (NPI-Q) scores due to more frequent endorsement of depression and irritability.
DISCUSSION: f-bvFTD and s-bvFTD cases are clinically similar, suggesting the generalizability of novel biomarkers, therapies, and clinical tools developed in either form to the other.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:16 |
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| Enthalten in: |
Alzheimer's & dementia : the journal of the Alzheimer's Association - 16(2020), 1 vom: 28. Jan., Seite 60-70 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Heuer, Hilary W [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 30.10.2020 Date Revised 28.03.2024 published: Print Citation Status MEDLINE |
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| doi: |
10.1002/alz.12046 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM305135643 |
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| 245 | 1 | 0 | |a Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort |
| 264 | 1 | |c 2020 | |
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| 500 | |a Date Completed 30.10.2020 | ||
| 500 | |a Date Revised 28.03.2024 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2020 the Alzheimer's Association. | ||
| 520 | |a INTRODUCTION: Behavioral variant frontotemporal dementia (bvFTD) may present sporadically or due to an autosomal dominant mutation. Characterization of both forms will improve understanding of the generalizability of assessments and treatments | ||
| 520 | |a METHODS: A total of 135 sporadic (s-bvFTD; mean age 63.3 years; 34% female) and 99 familial (f-bvFTD; mean age 59.9; 48% female) bvFTD participants were identified. f-bvFTD cases included 43 with known or presumed chromosome 9 open reading frame 72 (C9orf72) gene expansions, 28 with known or presumed microtubule-associated protein tau (MAPT) mutations, 14 with known progranulin (GRN) mutations, and 14 with a strong family history of FTD but no identified mutation | ||
| 520 | |a RESULTS: Participants with f-bvFTD were younger and had earlier age at onset. s-bvFTD had higher total Neuropsychiatric Inventory Questionnaire (NPI-Q) scores due to more frequent endorsement of depression and irritability | ||
| 520 | |a DISCUSSION: f-bvFTD and s-bvFTD cases are clinically similar, suggesting the generalizability of novel biomarkers, therapies, and clinical tools developed in either form to the other | ||
| 650 | 4 | |a Comparative Study | |
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