CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease
CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease
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DOI：
10.1212/01.wnl.0000230159.67128.00
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发表时间：
2006-08-22
期刊：
影响因子：
9.9
通讯作者：
Zerr, I.
中科院分区：
文献类型：
--
作者：
Sanchez-Juan, P.;Green, A.;Zerr, I.
目的：分析脑脊液中不同脑源性蛋白(14-3-3、Tau、神经元特异性烯醇化酶[NSE]和S100B)对克雅病(CJD)诊断的敏感性和特异性，并分析影响这些参数的生物因素。方法：对1859例散发性、遗传性、医源性和变异型CJD患者和1117例正常对照进行脑脊液14-3-3、Tau、NSE和S100B检测。结果：14-3-3和Tau对散发性CJD的敏感性最高(85%和86%)，14-3-3和Tau、S100B或NSE联合检测的敏感性可达93%以上。多变量分析显示，病程最短、发病年龄<40岁、PrP基因第129位密码子纯合的患者所有检测的敏感性最高。在一组反复进行腰椎穿刺术的患者中，第二次测试也提高了诊断的敏感性。结论：检测疑似克雅氏病患者脑脊液中脑源性蛋白水平的升高是一项有价值的诊断试验。第二次腰椎穿刺术可能对第一次检查为阴性的非典型临床病程的患者有价值。
Objectives: To analyze the diagnostic sensitivity and specificity of various brain-derived proteins (14-3-3, Tau, neuron specific enolase [NSE], and S100b) in the CSF of patients with Creutzfeldt-Jakob disease (CJD) and to analyze biologic factors that modify these parameters. Methods: CSF was tested for 14-3-3, Tau, NSE, and S100b in 1,859 patients with sporadic, genetic, iatrogenic, and variant CJD, and in 1,117 controls. Results: The highest sensitivity was achieved for 14-3-3 and Tau in sporadic CJD (85% and 86%), and a combined determination of 14-3-3 and Tau, S100b, or NSE increased the sensitivity to over 93%. A multivariate analysis showed that the sensitivity of all tests was highest in patients with the shortest disease duration, age at onset > 40 years, and homozygosity at codon 129 of the prion protein gene. In a group of patients with repeated lumbar punctures, a second test also increased the diagnostic sensitivity. Conclusions: The detection of elevated levels of brain-derived proteins in the CSF in patients with suspected CreutzfeldtJakob disease is a valuable diagnostic test. A second lumbar puncture may be of value in patients with atypical clinical course in whom the first test was negative.