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

IF 11.1 1区医学 Q1 CLINICAL NEUROLOGY Alzheimer's & Dementia Pub Date : 2025-02-12 DOI:10.1002/alz.14558

Maria Sofia Cotelli, Barbara Tarantino, Kübra Tan, Hanna Huber, Valentina Cantoni, Valeria Bracca, Roberto Gasparotti, Enrico Premi, Giancarlo Logroscino, Andrea L. Benedet, Kaj Blennow, Henrik Zetterberg, Mario Grassi, Nicholas J. Ashton, Barbara Borroni

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Abstract

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.

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通过血浆生物标志物和临床变量预测与额颞叶变性相关综合征的生存率

建立与额颞叶变性（FTLD）相关综合征的生存率模型对于评估疾病轨迹至关重要。方法在262例FTLD患者中，我们考虑血浆神经丝轻链（NfL）、胶质纤维酸性蛋白、脑源性tau、磷酸化tau217和β淀粉样蛋白（a - β42/ a - β40）。FTLD生存评分（FTLD- ss）由与存活率独立相关变量的β系数计算。血浆NfL水平升高(p <；0.001)、评估时年龄较大（p = 0.002）、阳性家族史（p = 0.04）和运动表型(p <；0.001)与生存率降低相关。1年时，FTLD-SS的预测效度为0.75（95%可信区间为0.59-0.91）。FTLD的生存率由神经退行性变的强度（以血浆NfL为代表）以及某些临床变量决定。在临床试验中，FTLD-SS可作为生存率估计和患者分层的简单工具。血浆神经丝轻链和临床变量可以预测额颞叶变性（FTLD）相关综合征的生存率。FTLD生存评分（FTLD- ss）与生存预测因子一起计算，可以作为患者分层的简单工具。FTLD-SS与额叶和壳核区域更大的萎缩有关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Alzheimer's & Dementia 医学-临床神经学

CiteScore

14.50

自引率

5.00%

发文量

299

审稿时长

3 months

期刊介绍： Alzheimer's & Dementia is a peer-reviewed journal that aims to bridge knowledge gaps in dementia research by covering the entire spectrum, from basic science to clinical trials to social and behavioral investigations. It provides a platform for rapid communication of new findings and ideas, optimal translation of research into practical applications, increasing knowledge across diverse disciplines for early detection, diagnosis, and intervention, and identifying promising new research directions. In July 2008, Alzheimer's & Dementia was accepted for indexing by MEDLINE, recognizing its scientific merit and contribution to Alzheimer's research.