Logo image
Associations between prognostic index scores and plasma neurofilament light in Huntington's disease
Journal article   Open access   Peer reviewed

Associations between prognostic index scores and plasma neurofilament light in Huntington's disease

Georgia M. Parkin, Jody Corey-Bloom, Jeffrey D. Long, Chase Snell, Haileigh Smith and Elizabeth A. Thomas
Parkinsonism & related disorders, Vol.97, pp.25-28
04/2022
DOI: 10.1016/j.parkreldis.2022.02.023
PMCID: PMC9127124
PMID: 35276585
url
https://doi.org/10.1016/j.parkreldis.2022.02.023View
Published (Version of record) Open Access

Abstract

The inclusion of premanifest Huntington's Disease (Pre-HD) subjects in clinical trials necessitates selecting those who are near transition to manifest Huntington's disease (Man-HD). We previously determined that plasma neurofilament light (NfL) levels are significantly correlated with predicted years to Man-HD onset, using established formulae. Recently, a new normalized prognostic index (PIN) score for predicting Pre-HD disease progression has been validated. Our objective was to determine whether plasma NfL levels are similarly associated with PIN score and PIN score-derived years to Man-HD onset (PIN-YTO). 112 individuals (46 Pre-HD, 66 Man-HD) underwent blood sample collection and clinical assessment, inclusive of the Symbol Digit Modalities Test and Unified Huntington's Disease Rating Scale Total Motor Score. Plasma NfL levels were measured using a Meso Scale Discovery assay. Pre-HD and Man-HD cohorts differed by age (p < .0001), and CAG repeat number (p = .004), but not education level or gender. Plasma NfL levels were significantly correlated with PIN scores (r = 0.69, p < .0001) and PIN-YTO (r = −0.69, p < .0001). Plasma NfL levels were similarly correlated with predicted years to onset scores determined using Langbehn and colleague's formula (r = −0.68, p < .0001). All significant correlations endured corrections for age and CAG repeat number. A plasma NfL cut-point of <45.0 pg/ml distinguished Pre-HD participants >10 predicted years from Man-HD onset, compared to those ≤10 predicted years. We have extensively shown that plasma NfL levels are associated with predicted years to manifest HD onset in Pre-HD participants, and present a plasma NfL cut-point that may help exclude far-from-onset Pre-HD patients from clinical trials. •Plasma NfL is correlated with the normalized prognostic index (PIN) score.•Plasma NfL correlated with PIN-derived predicted years to manifest HD onset (YTO).•A plasma NfL cut-point of <45.0 pg/ml identifies premanifest HD far from HD onset.•Associations mirrored those previously observed using Langbehn et al.’s YTO formula.•Plasma NfL is associated with YTO but not factors unique to the PIN.
Biomarker Blood Huntington's disease Neurofilament light Plasma

Details

Logo image