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

被引:0
作者
Cotelli, Maria Sofia [1 ]
Tarantino, Barbara [2 ]
Tan, Kuebra [3 ]
Huber, Hanna [3 ]
Cantoni, Valentina [1 ]
Bracca, Valeria [4 ]
Gasparotti, Roberto [5 ]
Premi, Enrico [6 ]
Logroscino, Giancarlo [7 ]
Benedet, Andrea L. [3 ]
Blennow, Kaj [3 ,8 ,9 ,10 ,11 ,12 ]
Zetterberg, Henrik [3 ,13 ,14 ,15 ,16 ]
Grassi, Mario [2 ]
Ashton, Nicholas J. [3 ,17 ,18 ]
Borroni, Barbara [4 ,19 ]
机构
[1] ASST Spedali Civili, Dept Continu Care & Frailty, Brescia, Italy
[2] Univ Pavia, Dept Brain & Behav Sci, Med & Genom Stat Unit, Pavia, Italy
[3] Univ Gothenburg, Sahlgrenska Acad, Inst Neurosci & Physiol, Dept Psychiat & Neurochem, Gothenburg, Sweden
[4] Univ Brescia, Dept Clin & Expt Sci, Piazzale Spedali Civili 1, I-25123 Brescia, Italy
[5] Univ Brescia, Neuroradiol Unit, Brescia, Italy
[6] ASST Spedali Civili, Stroke Unit, Brescia, Italy
[7] Univ Bari Aldo Moro, Pia Fdn Cardinale Giovanni Panico, Ctr Neurodegenerat Dis & Aging Brain, Bari, Italy
[8] Sahlgrens Univ Hosp, Clin Neurochem Lab, Gothenburg, Sweden
[9] Sorbonne Univ, Pitie Salpetriere Hosp, Paris Brain Inst, ICM, Paris, France
[10] Univ Sci & Technol China, Neurodegenerat Disorder Res Ctr, Div Life Sci & Med, Hefei, Peoples R China
[11] Univ Sci & Technol China, Inst Aging & Brain Disorders, Dept Neurol, Hefei, Peoples R China
[12] USTC, Affiliated Hosp 1, Hefei, Peoples R China
[13] UCL, Inst Neurol, Dept Neurodegenerat Dis, London, England
[14] UCL, UK Dementia Res Inst, London, England
[15] Hong Kong Ctr Neurodegenerat Dis, Clear Water Bay, Hong Kong, Peoples R China
[16] Univ Wisconsin Madison, Wisconsin Alzheimers Dis Res Ctr, Sch Med & Publ Hlth, Madison, WI USA
[17] Banner Sun Hlth Res Inst, Sun City, AZ USA
[18] Univ Arizona, Translat Genom Res Inst, Phoenix, AZ 85006 USA
[19] IRCCS Ist Ctr San Giovanni di Dio Fatebenefratelli, Mol Markers Lab, Brescia, Italy
基金
瑞典研究理事会;
关键词
biological markers; corticobasal syndrome; frontotemporal dementia; frontotemporal lobar degeneration; motor neuron disease; primary progressive aphasia; progressive supranuclear palsy; survival; BEHAVIORAL VARIANT; DEMENTIA; SENSITIVITY; PREVALENCE; DIAGNOSIS; CRITERIA; GFAP;
D O I
10.1002/alz.14558
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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 (A beta 42/A beta 40). The FTLD Survival Score (FTLD-SS) was calculated by the beta 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.
引用
收藏
页数:9
相关论文
共 40 条
  • [1] Borroni B., Padovani A., Dementia: a new algorithm for molecular diagnstics in FTLD, Nat Rev Neurol, pp. 241-242, (2013)
  • [2] Gorno-Tempini M.L., Hillis A.E., Weintraub S., Et al., Classification of primary progressive aphasia and its variants, Neurology, 76, pp. 1006-1014, (2011)
  • [3] Rascovsky K., Hodges J.R., Knopman D., Et al., Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia, Brain, 134, pp. 2456-2477, (2011)
  • [4] Hoglinger G.U., Respondek G., Stamelou M., Et al., Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov Disord, 32, pp. 853-864, (2017)
  • [5] Armstrong M.J., Litvan I., Lang A.E., Et al., Criteria for the diagnosis of corticobasal degeneration, Neurology, 80, pp. 496-503, (2013)
  • [6] Strong M.J., Abrahams S., Goldstein L.H., Et al., Amyotrophic lateral sclerosis—frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria, Amyotroph Lateral Scler Front Degener, 18, pp. 153-174, (2017)
  • [7] Panza F., Lozupone M., Seripa D., Et al., Development of disease-modifying drugs for frontotemporal dementia spectrum disorders, Nat Rev Neurol, (2020)
  • [8] Benussi A., Borroni B., Advances in the treatment and management of frontotemporal dementia, Expert Rev Neurother, 23, 7, pp. 621-639, (2023)
  • [9] Coyle-Gilchrist I.T.S., Dick K.M., Patterson K., Et al., Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes, Neurology, 86, 18, pp. 1736-1743, (2016)
  • [10] El-Wahsh S., Finger E., Piguet O., Et al., Predictors of survival in frontotemporal lobar degeneration syndromes, J Neurol Neurosurg Psychiatry, 92, pp. 425-433, (2021)