A prospective study of CSF markers in 250 patients with possible Creutzfeldt-Jakob disease
A prospective study of CSF markers in 250 patients with possible Creutzfeldt-Jakob disease
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DOI：
10.1136/jnnp.74.9.1210
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发表时间：
2003-09-01
影响因子：
11
通讯作者：
Cras, P
中科院分区：
文献类型：
--
作者：
Van Everbroeck, B;Quoilin, S;Cras, P
目的：探讨有助于临床诊断克雅氏病（CJD）的各种脑脊液（CSF）标志物。方法：分析 250 名疑似克雅氏病患者的脑脊液样本中 14-3-3 蛋白、微管相关蛋白 tau 和 β 淀粉样蛋白的存在情况。采用光密度分析对所有患者的14-3-3水平进行定量。结果：临床数据分析显示，小脑体征或肌阵挛合并进行性痴呆是临床怀疑克雅氏病的主要特征。 14-3-3 检测的灵敏度为 100%，特异性为 92%，而使用 1300 pg/ml 阈值的 tau 测定的灵敏度为 87%，特异性为 97%。如果修改 14-3-3 分析方案（使用光密度分析），可以获得更高的特异性 (97%)，但灵敏度较低 (96%)。结合 14-3-3 和 b 淀粉样蛋白测定获得最大灵敏度、特异性和阳性预测值。病程较长（一年以上；p）的克雅氏病患者脑脊液中 14-3-3 和 tau 浓度降低
Objective: To investigate various cerebrospinal fluid (CSF) markers that could assist in the clinical diagnosis of Creutzfeldt-Jakob disease (CJD).Methods: CSF samples were analysed for the presence of 14-3-3 protein, microtubule associated protein tau, and beta amyloid in 250 patients with possible CJD. Densitometric analysis was used to quantify the level of 14-3-3 in all patients.Results: Analysis of the clinical data showed that cerebellar signs or myoclonus combined with progressive dementia were the main features leading to a clinical suspicion of CJD. While 14-3-3 detection had a sensitivity of 100% and a specificity of 92%, tau determination using a threshold of 1300 pg/ml had a sensitivity of 87% and a specificity of 97%. If the protocol for the analysis of 14-3-3 was modified (using densitometric analysis) a higher specificity (97%) could be obtained, but with a lower sensitivity (96%). Maximum sensitivity, specificity, and positive predictive value were obtained with a combination of 14-3-3 and b amyloid determinations. The concentrations of 14-3-3 and tau in the CSF were reduced in CJD patients with a long duration of disease (more than one year; p