ANIMAL SPONGIFORM ENCEPHALOPATHIES - AN UPDATE .1. SCRAPIE AND LESSER KNOWN ANIMAL SPONGIFORM ENCEPHALOPATHIES

The present article (part I) reviews recent developments in animal spongiform encephalopathies (SEs), with the exception of bovine spongiform encephalopathy (BSE), which is dealt with in part II. The article focuses on scrapie and describes epidemiological aspects and the prospects for a preclinical diagnosis. Up to now, confirmatory diagnosis of scrapie depended on histological examination of the brain, collected during post-mortem examination from sheep with clinical signs of the disease. An altered protein, PrPSC, can be detected in the brain of diseased animals. The demonstration of the same protein in the spleen and in peripheral lymph nodes of infected animals seems to offer interesting possibilities of arriving at a method for a preclinical diagnosis, and thus a diagnosis in the live animal. Progress has also been made in our understanding of the relationship between the genetic constitution and susceptibility of the host. Susceptibility is expressed as the survival time of sheep inoculated with scrapie. This was thought to be determined by a single genetic locus designated the Sip gene (scrapie incubation period gene). Putative markers for the two alleles of the Sip gene, sA and pA, have been discovered, consisting of restriction fragment length polymorphisms (RFLPs). In field tests, however, the link between these markers and the length of incubation time was far from consistent. These RFLPs were found to be situated outside the prion-protein-coding region of the ovine gene. In later studies, RFLPs were detected inside this region. These markers appear to be more informative, i.e. they correspond with a difference in the length of the scrapie incubation period. Finally, the article briefly describes recent developments in other, lesser known, animal spongiform encephalopathies: chronic wasting disease and other spongiform encephalopathies in exotic ungulates, transmissible mink encephalopathy, and feline spongiform encephalopathy, focusing on their possible links with scrapie or bovine spongiform encephalopathy.