Imaging and CSF analyses effectively distinguish CJD from its mimics.
Imaging and CSF analyses effectively distinguish CJD from its mimics.
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DOI：
10.1136/jnnp-2017-316853
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发表时间：
2018-05
期刊：
影响因子：
--
通讯作者：
Mead S
中科院分区：
文献类型：
--
作者：
Rudge P;Hyare H;Green A;Collinge J;Mead S
回顾被转诊至朊病毒专科诊所的疑似散发性克雅氏病（sCJD）患者的临床和研究结果，但发现这些患者有其他最终诊断。回顾2008年10月至2015年11月期间入组英国国家朊病毒监测队列研究的214例患者的临床结果和研究，这些患者经尸检证实为sCJD，并将这些特征与同期转诊的50例具有替代最终诊断（CJD模拟物）的患者进行比较。具有替代诊断的患者和患有sCJD的患者具有相似的年龄、性别和痴呆的频率，但是CJD模拟物具有更长的临床病史。肌阵挛，僵硬和幻觉更常见的sCJD患者，但这些功能是没有帮助的分类个别患者。阿尔茨海默病、路易体痴呆和遗传性神经退行性疾病是超过一半的CJD模拟病例的替代诊断，10%的病例有免疫介导的脑病;淋巴瘤、肝性脑病和进行性多灶性白质脑病不止一次出现。磁共振弥散加权成像是最有用的现成的测试，以正确分类的情况下（92%克雅氏病，2%克雅氏病模拟）。脑脊液细胞计数、14-3-3蛋白检测和S100 B的价值有限。在研究过程中引入的阳性CSF RT-QuIC测试在89%的测试CJD病例和0%的CJD模拟物中发现。在本研究中，弥散加权MRI分析和CSF RT-QuIC的组合允许对sCJD与其模拟物进行完美分类。
To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. Review the clinical findings and investigations in 214 patients enrolled into the UK National Prion Monitoring Cohort Study between October 2008 and November 2015 who had postmortem confirmed sCJD and compare these features with 50 patients referred over the same period who had an alternative final diagnosis (CJD mimics). Patients with an alternative diagnosis and those with sCJD were of similar age, sex and frequency of dementia but CJD mimics had a longer clinical history. Myoclonus, rigidity and hallucinations were more frequent in patients with sCJD but these features were not helpful in classifying individual patients. Alzheimer’s disease, dementia with Lewy bodies and genetic neurodegenerative disorders were alternative diagnoses in more than half of the CJD mimic cases, and 10% had an immune-mediated encephalopathy; lymphoma, hepatic encephalopathy and progressive multifocal leukoencephalopathy were seen more than once. Diffusion-weighted MRI was the most useful readily available test to classify cases correctly (92% CJD, 2% CJD mimics). The CSF cell count, 14-3-3 protein detection and S100B were of limited value. A positive CSF RT-QuIC test, introduced during the course of the study, was found in 89% of tested CJD cases and 0% CJD mimics. The combination of diffusion-weighted MRI analysis and CSF RT-QuIC allowed a perfect classification of sCJD versus its mimics in this study.
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DOI：
10.1016/j.jalz.2011.03.005
发表时间：
2011-05
期刊：
Alzheimer's & dementia : the journal of the Alzheimer's Association
影响因子：
--
作者：
McKhann GM;Knopman DS;Chertkow H;Hyman BT;Jack CR Jr;Kawas CH;Klunk WE;Koroshetz WJ;Manly JJ;Mayeux R;Mohs RC;Morris JC;Rossor MN;Scheltens P;Carrillo MC;Thies B;Weintraub S;Phelps CH
通讯作者：
Phelps CH
DOI：
10.1093/brain/awp191
发表时间：
2009-10
期刊：
Brain : a journal of neurology
影响因子：
--
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
通讯作者：
Sanchez-Juan P
影响因子：
3.5
作者：
Hyare, H.;Thornton, J.;Jaeger, H. R.
通讯作者：
Jaeger, H. R.
影响因子：
158.5
作者：
Phornphutkul, C;Introne, WJ;Gahl, WA
通讯作者：
Gahl, WA
影响因子：
17.1
作者：
Bougard, Daisy;Brandel, Jean-Philippe;Coste, Joliette
通讯作者：
Coste, Joliette