期刊
JOURNAL OF CLINICAL NEUROLOGY
卷 12, 期 1, 页码 101-106出版社
KOREAN NEUROLOGICAL ASSOC
DOI: 10.3988/jcn.2016.12.1.101
关键词
Creutzfeldt-Jakob disease; cerebrospinal fluid; RT-QuIC; 14-3-3; total tau protein
资金
- National Research Foundation of Korea Grant - Korean Government [NRF-2011K3A1A 1003362]
- Hallym University Specialization Fund [HRF-S-41]
Background and Purpose The level of 14-3-3 protein in the cerebrospinal fluid (CSF) is increased in Creutzfeldt-Jakob disease (CJD) patients, which has led to it being used as a clinical biomarker for the ante-mortem diagnosis of human prion diseases. However, the specificity of the 14-3-3 protein is less reliable for CJD diagnosis. Newly developed assays including real-time quaking-induced conversion (RT-QuIC) have made it possible to detect the PrPSc-like abnormal prion isoform with a high sensitivity in animal and human specimens that might contain a minute amount of PrPSc due to in vitro prion replication. Methods This study applied a highly sensitive RT-QuIC assay using recombinant human PrP to detect PrPSc in the CSF of 81 patients with sporadic CJD (sCJD) in Korea. Results RT-QuIC analysis of the CSF samples based on the expression levels of 14-3-3 and total tau proteins revealed positivity in 62 of 81 sCJD patients (sensitivity of 76.5%) but no positive results in the 100 non-CJD patients. Conclusions The sensitivity of the RT-QuIC in this study was similar to that in some previous reports, and the specificity of RT-QuIC was higher than that of 14-3-3 in CSF, suggesting that RT-QuIC analysis can complement the weakness of the specificity of 14-3-3 for the diagnosis of sCJD. These results indicate that RT-QuIC might be very useful for the rapid and specific diagnosis of sCJD and provide a practical novel method for the ante-mortem diagnosis of human prion diseases.
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