髄液によるプリオン病の診断と鑑別診断

Abstract

Hsich et al reported 14-3-3 protein in CSF supports a diagnosis of human prion disease, and 14-3-3 protein is one of supportive diagnostic criteria on WHO (1998). In the presence of 14-3-3 protein and total tau protein is widely used as a surrogate marker in the pre-mortem diagnosis of human prion disease and other rapidly progressive dementia. Most recent research report that the sensitivity of 14-3-3 protein was 43%-100%, and the specificity of 14-3-3 protein was 47%-97%. And the sensitivity of total tau protein was 43%-100%, and the specificity of total tau protein was 47%-97%. In other hand we recently developed a new in vitro amplification technology, designated "RT-QUIC assay", for the detection of PrP(Sc) in CSF of sCJD. Other group reported that CSF RT-QUIC method analysis has the potential to be a more specific diagnostic test for sCJD than current CSF tests, but their method is different from our method and their study was not enough. Now we are analyzing for the sensitivity and specificity of biomarkers and RT-QUIC method in CSF of definite cases of human prion disease, the number of definite cases of human prion disease is larger than that of other studies.