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Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes

BACKGROUND: Frontotemporal dementia (FTD) syndromes, mimics, phenocopy (phFTD), and slowly progressive behavioral variant FTD (bvFTD) can be difficult to distinguish clinically. Biomarkers such as neurofilament light chain (NfL) may be helpful. OBJECTIVE: To study plasma NfL levels in people with FT...

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Autores principales: Ooi, Suyi, Patel, Sheila K., Eratne, Dhamidhu, Kyndt, Christopher, Reidy, Natalie, Lewis, Courtney, Lee, Sarah C.M., Darby, David, Brodtmann, Amy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: IOS Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9661326/
https://www.ncbi.nlm.nih.gov/pubmed/35988220
http://dx.doi.org/10.3233/JAD-220272
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author Ooi, Suyi
Patel, Sheila K.
Eratne, Dhamidhu
Kyndt, Christopher
Reidy, Natalie
Lewis, Courtney
Lee, Sarah C.M.
Darby, David
Brodtmann, Amy
author_facet Ooi, Suyi
Patel, Sheila K.
Eratne, Dhamidhu
Kyndt, Christopher
Reidy, Natalie
Lewis, Courtney
Lee, Sarah C.M.
Darby, David
Brodtmann, Amy
author_sort Ooi, Suyi
collection PubMed
description BACKGROUND: Frontotemporal dementia (FTD) syndromes, mimics, phenocopy (phFTD), and slowly progressive behavioral variant FTD (bvFTD) can be difficult to distinguish clinically. Biomarkers such as neurofilament light chain (NfL) may be helpful. OBJECTIVE: To study plasma NfL levels in people with FTD syndromes and determine if plasma NfL can distinguish between FTD syndromes and phFTD. METHODS: Plasma NfL levels were estimated using both Simoa(®) Quanterix HD-X™ and SR-X™ machines grouped via final diagnosis after investigation and review. RESULTS: Fifty participants were studied: bvFTD = 20, semantic variant FTD (svFTD) = 11, non-fluent variant FTD (nfvFTD) = 9, FTD with motor neuron disease (MND) = 4, phFTD = 2, slow progressors = 3, FTD mimic = 1, mean age 67.2 (SD 8.4) years. NfL levels were significantly higher in the FTD group compared to phenocopy group (p = 0.003). Median NfL (IQR) pg/mL was comparable in the FTD syndromes: bvFTD 41.10 (50.72), svFTD 44.38 (16.61), and nfvFTD 42.61 (22.93), highest in FTD with MND 79.67 (45.32) and lowest in both phFTD 13.99 (0.79) and slow progressors 17.97 (3.62). CONCLUSION: Plasma NfL appears to differentiate FTD syndromes and mimics. However, a lower NfL may predict a slower, but not necessarily absence of neurodegeneration, and therefore appears limited in distinguishing slow progressors from FTD phenocopies. Larger numbers of patients from all clinical groups are required to strengthen diagnostic utility.
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spelling pubmed-96613262022-11-28 Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes Ooi, Suyi Patel, Sheila K. Eratne, Dhamidhu Kyndt, Christopher Reidy, Natalie Lewis, Courtney Lee, Sarah C.M. Darby, David Brodtmann, Amy J Alzheimers Dis Research Article BACKGROUND: Frontotemporal dementia (FTD) syndromes, mimics, phenocopy (phFTD), and slowly progressive behavioral variant FTD (bvFTD) can be difficult to distinguish clinically. Biomarkers such as neurofilament light chain (NfL) may be helpful. OBJECTIVE: To study plasma NfL levels in people with FTD syndromes and determine if plasma NfL can distinguish between FTD syndromes and phFTD. METHODS: Plasma NfL levels were estimated using both Simoa(®) Quanterix HD-X™ and SR-X™ machines grouped via final diagnosis after investigation and review. RESULTS: Fifty participants were studied: bvFTD = 20, semantic variant FTD (svFTD) = 11, non-fluent variant FTD (nfvFTD) = 9, FTD with motor neuron disease (MND) = 4, phFTD = 2, slow progressors = 3, FTD mimic = 1, mean age 67.2 (SD 8.4) years. NfL levels were significantly higher in the FTD group compared to phenocopy group (p = 0.003). Median NfL (IQR) pg/mL was comparable in the FTD syndromes: bvFTD 41.10 (50.72), svFTD 44.38 (16.61), and nfvFTD 42.61 (22.93), highest in FTD with MND 79.67 (45.32) and lowest in both phFTD 13.99 (0.79) and slow progressors 17.97 (3.62). CONCLUSION: Plasma NfL appears to differentiate FTD syndromes and mimics. However, a lower NfL may predict a slower, but not necessarily absence of neurodegeneration, and therefore appears limited in distinguishing slow progressors from FTD phenocopies. Larger numbers of patients from all clinical groups are required to strengthen diagnostic utility. IOS Press 2022-10-11 /pmc/articles/PMC9661326/ /pubmed/35988220 http://dx.doi.org/10.3233/JAD-220272 Text en © 2022 – The authors. Published by IOS Press https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial (CC BY-NC 4.0) License (https://creativecommons.org/licenses/by-nc/4.0/) , which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Ooi, Suyi
Patel, Sheila K.
Eratne, Dhamidhu
Kyndt, Christopher
Reidy, Natalie
Lewis, Courtney
Lee, Sarah C.M.
Darby, David
Brodtmann, Amy
Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes
title Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes
title_full Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes
title_fullStr Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes
title_full_unstemmed Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes
title_short Plasma Neurofilament Light Chain and Clinical Diagnosis in Frontotemporal Dementia Syndromes
title_sort plasma neurofilament light chain and clinical diagnosis in frontotemporal dementia syndromes
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9661326/
https://www.ncbi.nlm.nih.gov/pubmed/35988220
http://dx.doi.org/10.3233/JAD-220272
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