Diagnosis of Creutzfeldt-Jakob disease -: Effect of clinical criteria on incidence estimates
Diagnosis of Creutzfeldt-Jakob disease -: Effect of clinical criteria on incidence estimates
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DOI：
10.1212/wnl.54.5.1095
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发表时间：
2000-03-14
期刊：
影响因子：
9.9
通讯作者：
Alpérovitch, A
中科院分区：
文献类型：
--
作者：
Brandel, JP;Delasnerie-Lauprêtre, N;Alpérovitch, A
目标：目的：评价3种不同克雅氏病（Creutzfeldt-Jakob disease，CJD）诊断标准对CJD发病率的影响。方法：对1991 ~ 1997年收治的428例疑似散发性CJD患者按不同诊断标准进行分类，并进行分析比较。在尸检患者亚组中，计算每组标准的特异性、敏感性、阳性和阴性预测值。临床诊断的阳性和阴性预测值适用于未经尸检的病例。随后，对转介病例中克雅二氏症的实际病例数进行了估计。结果如下：与法国和欧洲研究标准相比，Masters标准显示出较高的敏感性，但特异性和阳性预测值较低。与CJD病例真实总数的估计值相比，马斯特斯标准高估了7%的发病率，而法国和欧洲研究标准则低估了12%。CSF中14-3-3蛋白的检测被认为是临床上可能的CJD的额外诊断标准，当应用法国或欧洲研究标准时，估计发病率略有增加。结论：不同的诊断标准可能导致低估或高估CJD的真实发病率。因此，不同国家CJD发病率的比较应依赖于使用相同标准的诊断分类，考虑到14-3-3蛋白检测作为可能的CJD的标准将导致估计的CJD发病率略有增加。
Objective: To assess the effect of usage of three different versions of Creutzfeldt-Jakob disease (CJD) diagnostic criteria on estimates of CJD incidence, Methods: A total of 428 patients referred for suspected sporadic CJD between 1991 and 1997 were classified according to different criteria to be compared after analysis of medical records. Specificity, sensitivity, and positive and negative predictive values were calculated for each set of criteria in the subgroup of patients with a postmortem examination. Positive and negative predictive values of the clinical diagnosis were applied to cases without postmortem examination. Subsequently, the true number of cases of CJD among the referred cases was estimated. Results: By comparison with the French and European study criteria, the Masters' criteria showed higher sensitivity but lower specificity and positive predictive value. Comparison with an estimate of the true total number of CJD cases showed that Masters' criteria overestimated the incidence by 7%, whereas the French and the European study criteria led to an underestimate of 12%. Detection of the 14-3-3 protein in CSF, considered as an additional diagnostic criterion for clinically probable CJD, resulted in a slight increase in the estimated incidence when the French or European study criteria were applied. Conclusions: Different diagnostic criteria could lead to an under- or overestimation of the true incidence of CJD. Therefore, comparisons of CJD incidence in different countries should rely on diagnostic classifications using identical criteria, Taking into account 14-3-3 protein detection as a criterion for probable CJD will result in a small increase in the estimated CJD incidence.