Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease.
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease.
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DOI：
10.1093/brain/awp191
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发表时间：
2009-10
期刊：
影响因子：
--
通讯作者：
Sanchez-Juan P
中科院分区：
文献类型：
--
作者：
Zerr I;Kallenberg K;Summers DM;Romero C;Taratuto A;Heinemann U;Breithaupt M;Varges D;Meissner B;Ladogana A;Schuur M;Haik S;Collins SJ;Jansen GH;Stokin GB;Pimentel J;Hewer E;Collie D;Smith P;Roberts H;Brandel JP;van Duijn C;Pocchiari M;Begue C;Cras P;Will RG;Sanchez-Juan P
散发性Creutzfeldt-Jakob病的几种分子亚型已被确定，脑电图和脑脊液生物标志物已被报道用于支持临床诊断，但根据亚型的不同，其效用不同。近年来，一系列的出版物已经证明了一个潜在的重要作用，磁共振成像在生前诊断散发性克雅氏病。磁共振成像信号改变与不同的散发性克雅氏病分子亚型相关，因此可能有助于早期识别散发性克雅氏病病例的整个谱。这项多中心国际研究旨在为修订散发性克雅氏病的临床诊断标准提供依据。从12个国家招募了散发性Creutzfeldt-Jakob病和液体衰减反转恢复或弥散加权成像患者。被称为“疑似散发性克雅氏病”但在彻底随访后有替代诊断的患者作为对照进行分析。根据涵盖七个皮质区域、基底神经节、丘脑和小脑的标准协议，评估所有磁共振成像扫描的信号变化。在436例散发性Creutzfeldt-Jakob病患者和141例对照者中评估了磁共振成像扫描。在散发性Creutzfeldt-Jakob病的鉴别诊断中，高信号模式具有最佳的敏感性和特异性。最佳的诊断准确性在快速进展性痴呆的鉴别诊断时，至少有两个皮质区域（颞，顶叶或枕叶）或尾状核和壳核显示高信号的液体衰减反转恢复或扩散加权成像磁共振成像。根据我们的分析，磁共振成像在83%的病例中呈阳性。在所有确诊个案中，经修订的标准将涵盖绝大部分怀疑个案，其中98%为阳性。磁共振成像中大脑皮质信号增加，尾状核和壳核液体衰减反转恢复或弥散加权成像高信号对散发性Creutzfeldt-Jakob病的诊断有帮助。我们建议对散发性克雅氏病的临床诊断标准进行修订，以纳入磁共振成像扫描的结果。
Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt–Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease to include findings from magnetic resonance imaging scans.
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影响因子：
2.4
作者：
Satoh, Katsuya;Shirabe, Susumu;Eguchi, Katsumi
通讯作者：
Eguchi, Katsumi
影响因子：
--
作者：
Josephs, Keith A.;Ahlskog, J. Eric;Petersen, Ronald C.
通讯作者：
Petersen, Ronald C.
影响因子：
5.1
作者：
Krasnianski, A.;Kallenberg, K.;Zerr, I.
通讯作者：
Zerr, I.
影响因子：
2.1
作者：
Geschwind MD;Potter CA;Sattavat M;Garcia PA;Rosen HJ;Miller BL;DeArmond SJ
通讯作者：
DeArmond SJ
影响因子：
9.9
作者：
Meissner, B;Westner, IM;Zerr, I
通讯作者：
Zerr, I