BSE-associated Prion-Amyloid Cardiomyopathy in Primates - Vol. 19 No. 6 - June 2013 - Emerging Infectious Disease journal - CDC
Table of Contents
Volume 19, Number 6–June 2013
Volume 19, Number 6—June 2013
BSE-associated Prion-Amyloid Cardiomyopathy in Primates
Human prion diseases are progressive neurologic disorders that include sporadic, genetic, and acquired forms of Creutzfeldt-Jakob disease (CJD) (1). A key step in disease initiation is conversion of PrPC into PrPSc, which is partially resistant to proteolytic digestion and an essential part of prion infectivity. Transmission of bovine spongiform encephalopathy (BSE) to humans has led to a novel form of acquired CJD, termed variant CJD (vCJD) (2). The pathogenesis of vCJD differs substantially from sporadic CJD with remarkable colonization of non–central nervous system regions with infectious prions and PrPSc (3).
AbstractPrion 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.
Although risk reduction measures have been introduced to limit transmission from BSE-diseased cattle to humans, vCJD has occurred in several hundred instances (www.eurocjd.ed.ac.uk). Most clinically affected vCJD patients are homozygous for methionine on polymorphic codon 129 on the gene coding PrP (PRNP), and the clinical presentation of vCJD in these patients is uniform (4). The occurrence of atypical clinical features in persons with vCJD that encodes methionine and valine on PRNP codon 129 and human-to-human transmission of vCJD through blood transfusion have raised concern about atypical clinical features and alternative distribution of PrPSc in vCJD (5). We report on the novel clinicopathologic characteristics of vCJD as prion-amyloid cardiomyopathy in 1 of 3 macaques inoculated with BSE.