NfL reliability across laboratories, stage-dependent diagnostic performance and matrix comparability in genetic FTD: a large GENFI study

J Neurol Neurosurg Psychiatry. 2024 Aug 16;95(9):822-828. doi: 10.1136/jnnp-2023-332464.

Abstract

Background: Blood neurofilament light chain (NfL) is increasingly considered as a key trial biomarker in genetic frontotemporal dementia (gFTD). We aimed to facilitate the use of NfL in gFTD multicentre trials by testing its (1) reliability across labs; (2) reliability to stratify gFTD disease stages; (3) comparability between blood matrices and (4) stability across recruiting sites.

Methods: Comparative analysis of blood NfL levels in a large gFTD cohort (GENFI) for (1)-(4), with n=344 samples (n=148 presymptomatic, n=11 converter, n=46 symptomatic subjects, with mutations in C9orf72, GRN or MAPT; and n=139 within-family controls), each measured in three different international labs by Simoa HD-1 analyzer.

Results: NfL revealed an excellent consistency (intraclass correlation coefficient (ICC) 0.964) and high reliability across the three labs (maximal bias (pg/mL) in Bland-Altman analysis: 1.12±1.20). High concordance of NfL across laboratories was moreover reflected by high areas under the curve for discriminating conversion stage against the (non-converting) presymptomatic stage across all three labs. Serum and plasma NfL were largely comparable (ICC 0.967). The robustness of NfL across 13 recruiting sites was demonstrated by a linear mixed effect model.

Conclusions: Our results underline the suitability of blood NfL in gFTD multicentre trials, including cross-lab reliable stratification of the highly trial-relevant conversion stage, matrix comparability and cross-site robustness.

Keywords: COGNITION; FRONTOTEMPORAL DEMENTIA; NEUROPSYCHIATRY.

Publication types

  • Multicenter Study
  • Comparative Study

MeSH terms

  • Aged
  • Biomarkers* / blood
  • C9orf72 Protein / genetics
  • Cohort Studies
  • Female
  • Frontotemporal Dementia* / blood
  • Frontotemporal Dementia* / diagnosis
  • Frontotemporal Dementia* / genetics
  • Humans
  • Male
  • Middle Aged
  • Mutation
  • Neurofilament Proteins* / blood
  • Neurofilament Proteins* / genetics
  • Progranulins / genetics
  • Reproducibility of Results
  • tau Proteins / blood
  • tau Proteins / genetics

Substances

  • Neurofilament Proteins
  • neurofilament protein L
  • Biomarkers
  • tau Proteins
  • C9orf72 Protein
  • Progranulins
  • MAPT protein, human
  • C9orf72 protein, human
  • GRN protein, human