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  Serum neurofilament light chain in behavioral variant frontotemporal dementia

Steinacker, P., Anderl-Straub, S., Diehl-Schmid, J., Semler, E., Uttner, I., von Arnim, C. A., et al. (2018). Serum neurofilament light chain in behavioral variant frontotemporal dementia. Neurology, 91(15), e1390-e1401. doi:10.1212/WNL.0000000000006318.

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Steinacker, Petra 1, Author
Anderl-Straub, Sarah 1, Author
Diehl-Schmid, Janine 1, Author
Semler, Elisa 1, Author
Uttner, Ingo 1, Author
von Arnim, Christine A.F. 1, Author
Barthel, Henryk 1, Author
Danek, Adrian 1, Author
Fassbender, Klaus 1, Author
Fliessbach, Klaus 1, Author
Foerstl, Hans 1, Author
Grimmer, Timo 1, Author
Huppertz, Hans-Jürgen 1, Author
Jahn, Holger 1, Author
Kassubek, Jan 1, Author
Kornhuber, Johannes 1, Author
Landwehrmeyer, Bernhard 1, Author
Lauer, Martin 1, Author
Maler, Juan Manuel 1, Author
Mayer, Benjamin 1, Author
Oeckl, Patrick 1, AuthorPrudlo, Johannes 1, AuthorSchneider, Anja 1, AuthorVolk, Alexander E. 1, AuthorWiltfang, Jens 1, AuthorSchroeter, Matthias L.1, 2, Author           Ludolph, Albert C. 1, AuthorOtto, Markus 1, AuthorFTLDc study group, Author               more..
1External Organizations, ou_persistent22              
2Department Neurology, MPI for Human Cognitive and Brain Sciences, Max Planck Society, ou_634549              


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 Abstract: BJECTIVE: To determine the association of serum neurofilament light chain (NfL) with functional deterioration and brain atrophy during follow-up of patients with behavioral variant frontotemporal dementia (bvFTD). METHODS: Blood NfL levels from 74 patients with bvFTD, 26 with Alzheimer disease (AD), 17 with mild cognitive impairment (MCI), and 15 healthy controls (Con) at baseline and follow-up were determined and analyzed for the diagnostic potential in relation to functional assessment (Clinical Dementia Rating Scale Sum of Boxes [CDR-SOB], frontotemporal lobar degeneration-related CDR-SOB, Mini-Mental State Examination [MMSE]) and brain volumetry. RESULTS: At baseline, serum NfL level correlated with CSF NfL (bvFTD r = 0.706, p < 0.0001; AD/MCI r = 0.666, p = 0.0003). Highest serum levels were observed in bvFTD (p <0 0.0001 vs Con and MCI, p = 0.0078 vs AD, respectively). Discrimination of bvFTD from Con/MCI/AD was possible with 91%/74%/74% sensitivity and 79%/74%/58% specificity. At follow-up, serum NfL increased in bvFTD and AD (p = 0.0039 and p = 0.0006, respectively). At baseline and follow-up, NfL correlated with functional scores of patients with bvFTD (e.g., CDR-SOB [baseline] r = 0.4157, p = 0.0006; [follow-up] r = 0.5629, p < 0.0001) and with atrophy in the gray and white matter of many brain regions including frontal and subcortical areas (e.g., frontal lobe: r = -0.5857, p < 0.0001; 95% confidence interval -0.7415 to -0.3701). For patients with AD/MCI, NfL correlated with the functional performance as well (e.g., CDR-SOB [baseline] r = 0.6624, p < 0.0001; [follow-up] r = 0.5659, p = 0.0003) but not with regional brain volumes. CONCLUSIONS: As serum NfL correlates with functional impairment and brain atrophy in bvFTD at different disease stages, we propose it as marker of disease severity, paving the way for its future use as outcome measure for clinical trials. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that for patients with cognitive problems, serum NfL concentration discriminates bvFTD from other forms of dementia.


Language(s): eng - English
 Dates: 2018-01-122018-07-102018-09-122018-10-09
 Publication Status: Published in print
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Type: Peer
 Identifiers: DOI: 10.1212/WNL.0000000000006318
PMID: 30209235
Other: Epub 2018
 Degree: -



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Project information

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Project name : German Consortium for Frontotemporal Lobar Degeneration
Grant ID : 01GI1007A
Funding program : -
Funding organization : German Federal Ministry of Education and Research (BMBF)
Project name : PreFrontAls
Grant ID : 01ED1512
Funding program : -
Funding organization : Joint Programme – Neurodegenerative Disease Research (JPND)
Project name : -
Grant ID : D.3830
Funding program : -
Funding organization : Foundation of the State of Baden-Wurttemberg
Project name : -
Grant ID : -
Funding program : -
Funding organization : Thierry Latran Foundation
Project name : Pathophysiological and diagnostic approaches toward Parkinson´s disease and Parkinson`s disease dementia
Grant ID : D.5009
Funding program : -
Funding organization : Boehringer Ingelheim Ulm University BioCenter (BIU)
Project name : -
Grant ID : -
Funding program : Ilidio Pinho professorship
Funding organization : Fundação Ilídio Pinho
Project name : -
Grant ID : UID/BIM/04501/2013
Funding program : -
Funding organization : iBiMED - Institute of Biomedicine

Source 1

Title: Neurology
Source Genre: Journal
Publ. Info: Cleveland, Ohio [etc.] : Advanstar Communications [etc.]
Pages: - Volume / Issue: 91 (15) Sequence Number: - Start / End Page: e1390 - e1401 Identifier: ISSN: 0028-3878
CoNE: https://pure.mpg.de/cone/journals/resource/954925246073