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[散发性克雅氏病和变异型克雅氏病的临床与实验室诊断]

[The clinical and laboratory diagnosis of sCJD and vCJD].

作者信息

Kulczycki Jerzy, Łojkowska Wanda

机构信息

I Kliniki Neurologicznej Instytutu Psychiatrii i Neurologii w Warszawie.

出版信息

Neurol Neurochir Pol. 2003;37 Suppl 2:7-14.

PMID:14560703
Abstract

Clinical diagnosis of sporadic CJD is usually confirmed by a typical EEG pattern and an increased level of protein 14-3-3 in the CSF, with specificity of 74% and 84%, respectively. However, both these tests are often negative in vCJD patients. Recently MR imaging has substantially improved the diagnosis of sCJD and vCJD. In sCJD patients hyperintense signal in T2-weighted sequences was found to be present in the neostriatum (the caudate nucleus and putamen) with specificity of 93%, while in vCJD cases signal hyperintensity was found in pulvinar thalami. The "pulvinar sign" was evidenced to be highly sensitive (79%) and specific (100%) for the diagnosis of the latter form of CJD.

摘要

散发性克雅氏病(sCJD)的临床诊断通常通过典型的脑电图模式和脑脊液中14-3-3蛋白水平升高来确诊,其特异性分别为74%和84%。然而,这两项检测在变异型克雅氏病(vCJD)患者中往往呈阴性。最近,磁共振成像(MR)显著改善了sCJD和vCJD的诊断。在sCJD患者中,T2加权序列上的高信号出现在新纹状体(尾状核和壳核),特异性为93%,而在vCJD病例中,丘脑枕出现信号高增强。“丘脑枕征”被证明对后一种形式的克雅氏病诊断具有高度敏感性(79%)和特异性(100%)。

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