Predicting survival rate by plasma biomarkers and clinical variables in syndromes associated with frontotemporal lobar degeneration.

INTRODUCTION

Modeling the survival rate in syndromes associated with frontotemporal lobar degeneration (FTLD) is essential to assess disease trajectories.

METHODS

In 262 patients with FTLD, we considered plasma neurofilament light chain (NfL), glial fibrillary acidic protein, brain-derived tau, phosphorylated tau217 and amyloid beta (Aβ42/Aβ40). The FTLD Survival Score (FTLD-SS) was calculated by the β coefficients of the variables independently associated with survival rate.

RESULTS

Increased plasma NfL levels (p < 0.001), older age at evaluation (p = 0.002), positive family history (p = 0.04), and motor phenotypes (p < 0.001) were associated with reduced survival. The predictive validity of FTLD-SS was 0.75 (95% confidence interval, 0.59-0.91) at 1 year.

DISCUSSION: Survival rate in FTLD is shaped by intensity of neurodegeneration (using plasma NfL as proxy) together with certain clinical variables. The FTLD-SS may serve as a simple tool for survival rate estimation and for patient stratification in clinical trials.

HIGHLIGHTS: Plasma neurofilament light chain and clinical variables can predict survival in frontotemporal lobar degeneration (FTLD)-associated syndromes. FTLD Survival Score (FTLD-SS), computed with survival predictors, may serve as a simple tool for patient stratification. FTLD-SS is associated with greater atrophy in frontal and putamen areas.