Serum neurofilament light chain in behavioral variant frontotemporal dementia
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Abstract
Objective 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.
Glossary
- Aβ42=
- β-amyloid 1–42;
- AD=
- Alzheimer disease;
- ALS=
- amyotrophic lateral sclerosis;
- AUC=
- area under the curve;
- bvFTD=
- behavioral variant frontotemporal dementia;
- CDR-SOB=
- Clinical Dementia Rating scale Sum of Boxes;
- CI=
- confidence interval;
- FTD=
- frontotemporal dementia;
- FTLD=
- frontotemporal lobar degeneration;
- ICV=
- intracranial volume;
- MCI=
- mild cognitive impairment;
- MMSE=
- Mini-Mental State Examination;
- NfL=
- neurofilament light chain;
- p-tau=
- phosphorylated tau;
- pNfH=
- phosphorylated neurofilament heavy chain;
- ROC=
- receiver operating characteristic;
- v1=
- visit 1;
- v2=
- visit 2
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Coinvestigators are listed at links.lww.com/WNL/A693.
Class of Evidence: NPub.org/coe
- Received January 12, 2018.
- Accepted in final form July 10, 2018.
- © 2018 American Academy of Neurology
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