The specimens of blood, CSF and brain tissues of the suspected patient were collected by the sentinel hospital. All samples were transported to the national reference laboratory for human prion disease in CCDC. The peripheral blood leukocytes were used for sequencing analysis of PRNP and polymorphism of codon 129, with an automatic genetic analyzer (ABI3130XL). CSF samples were obtained by routine lumbar puncture and analysed by Western immunoblot to detect the 14-3-3 protein. Brain tissues obtained from autopsy or biopsy were applied into neuropathologic assays and/or PrPSc detections with immunohistochemistry and/or Western blot. The standard operation procedure (SOP) of each test was well documented in the CJD surveillance program, which was described previously [4] .
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