Abstract
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.
Highlights
Prion amyloidosis occurred in the heart of 1 of 3 macaques intraperitoneally inoculated with bovine spongiform encephalopathy prions
We report on the novel clinicopathologic characteristics of variant CJD (vCJD) as prion-amyloid cardiomyopathy in 1 of 3 macaques inoculated with bovine spongiform encephalopathy (BSE)
Conclusions the vCJD epidemic is declining, considerable concern exists that clinical characterastics of vCJD will shift
Summary
All procedures involving rhesus macaques were performed at the Institute of Neuropathology, University Medical Center Hamburg-Eppendorf (Hamburg, Germany), in accordance with the German Animal Welfare Act and the Council Directive 86/609/EEC (Permit 33.42502/08–08.02 LAVES, Lower Saxony, Germany). Examination of brain by using hematoxylin and eosin staining showed typical neuropathologic features of vCJD (data not shown) and abundant deposits of PrPSc in the cortex, basal ganglia, and cerebellum in paraffin-embedded tissue blots performed as described by using 12F10 monclonal antiprion antibody [6] (Figure 1, panel A). Paraffin-embedded tissue blot analysis of this heart showed PrPSc as amyloid, occupying considerable stretches of heart tissue, mainly in the septum (Figure 2, panel C), whereas no PrPSc could be seen in hearts of other macaques (data not shown). The primate with cardiac PrPSc showed the longest disease duration (4 months, compared with 4 weeks for other BSE-infected monkeys), signs of cardiac affection
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