Prion data suggest BSE link to sporadic CJD

Bse and Cjd Update

An earlier POST report1 described what was known of the origin of BSE in cattle, and the basis for concern that ‘new variant’ forms of a human transmissible spongiform encaphalopathy (TSE) Creutzfeldt-Jacob Disease (CJD) had a BSE origin. A second report reviewed mathematical models which had helped understand the spread of BSE in cattle and predict what would happen to the future course of the...

CJD and prion disease.indd

The neuropsychology of variant CJD: a comparative study with inherited and sporadic forms of prion disease.

OBJECTIVE To assess cognitive function in variant Creutzfeldt-Jakob disease (vCJD). We describe the neuropsychological profiles of 10 cases and compare these data with cross sectional data obtained from patients with histologically confirmed sporadic CJD and cases with inherited prion disease with confirmed mutations in the prion protein gene. METHODS Patients referred to the Specialist Cogni...

Preclinical Detection of Variant CJD and BSE Prions in Blood

The emergence of variant Creutzfeldt Jakob Disease (vCJD) is considered a likely consequence of human dietary exposure to Bovine Spongiform Encephalopathy (BSE) agent. More recently, secondary vCJD cases were identified in patients transfused with blood products prepared from apparently healthy donors who later went on to develop the disease. As there is no validated assay for detection of vCJD...

BSE-associated Prion-Amyloid Cardiomyopathy in Primates

Prion amyloidosis occurred in the heart of 1 of 3 macaques intraperitoneally inoculated with bovine spongiform encephalopathy prions. This macaque had a remarkably long duration of disease and signs of cardiac distress. Variant Creutzfeldt-Jakob disease, caused by transmission of bovine spongiform encephalopathy to humans, may manifest with cardiac symptoms from prion-amyloid cardiomyopathy.