Evaluation of α-synuclein as a novel cerebrospinal fluid biomarker in different forms of prion diseases
Evaluation of α-synuclein as a novel cerebrospinal fluid biomarker in different forms of prion diseases
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DOI：
10.1016/j.jalz.2016.09.013
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发表时间：
2017-06-01
影响因子：
14
通讯作者：
Zerr, Inga
中科院分区：
文献类型：
--
作者：
Llorens, Franc;Kruse, Niels;Zerr, Inga
简介：朊病毒疾病的准确诊断和与替代性痴呆的区分在临床常规中获得重要性，但脑脊液（CSF）生物标志物的部分重叠阻碍了鉴别诊断背景下的绝对区分。我们建立了朊病毒病诊断的临床参数，用于定量散发性（n = 234）和遗传性（n = 56）患者的CSF α-突触核蛋白。朊病毒疾病，在认知障碍/痴呆或神经退行性疾病的情况下（n = 278），并在神经对照组（n = 111）。结果：680 pg/ mL的α-突触核蛋白的最佳截止值的结果在94%的敏感性和96%的特异性时，诊断散发性克雅氏病（CJD）。遗传性CJD病例显示CSF α-突触核蛋白值增加。没有增加的α-突触核蛋白水平在非CJD病例中检测到快速进展course.Discussion：检测疑似CJD患者的CSF中的α-突触核蛋白是一种有价值的诊断测试，几乎完全区分非朊病毒疾病病例。这些数据突出了在临床常规中在经典CSF生物标志物之前CSF α-突触核蛋白定量的效用。(C)2016年，阿尔茨海默氏症协会。爱思唯尔公司出版All rights reserved.
Introduction: Accurate diagnosis of prion diseases and discrimination from alternative dementias gain importance in the clinical routine, but partial overlap in cerebrospinal fluid (CSF) biomarkers impedes absolute discrimination in the differential diagnostic context.Methods: We established the clinical parameters for prion disease diagnosis for the quantification of CSF alpha-synuclein in patients with sporadic (n = 234) and genetic (n = 56) prion diseases, in cases with cognitive impairment/dementia or neurodegenerative disease (n = 278), and in the neurologic control group (n = 111).Results: An optimal cutoff value of 680 pg/ mL alpha-synuclein results in 94% sensitivity and 96% specificity when diagnosing sporadic Creutzfeldt-Jakob disease (CJD). Genetic CJD cases showed increased CSF alpha-synuclein values. No increased alpha-synuclein levels were detected in non-CJD cases with rapid progression course.Discussion: Detection of alpha-synuclein in the CSF of patients with suspected CJD is a valuable diagnostic test reaching almost full discrimination from non-prion disease cases. These data highlight the utility of CSF alpha-synuclein quantification in front of classical CSF biomarkers in clinical routine. (C) 2016 the Alzheimer's Association. Published by Elsevier Inc. All rights reserved.