Studies On Clinical Features, Neuropathological Features, Prion Protein, Prion Protein Gene And Proteome In Sporadic Creutzfeldt-Jakob Disease

We studied clinical Features, neuropathological features, prion protein, prion protein gene and proteme of brain tissues with CJD patients. Clinical Features of four subjects with confirmed CJD were different, including duration of symptoms, clinical signs at onset, symptoms, EEG and CT/MRI. All were PrPSc positive on immunohistochemistry stain. However, they had two patterns of PrPSc depositon. One was plaque-like deposits, the other were synaptic deposits. 3 were homozygous for methionine at codon 129, 1 case was methionine/valine at codon 129 of the prion protein gene. The clinical features and patterns of PrPSc depositon of the case with methionine/valine at codon 129 was different from the other cases. Though PrPSc could not be found in these cases, we still confirmed the diagnosis of CJD. The result of PrPSc negtive did not mean PrPSc was absent. Using sensitive techniques , we mgight identify depositon of PrP^Sc . Genomic DNA from 4 confirmed CJD subjects and 8 possible CJD subjects was used to amplify the coding region of PRNP in the...