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From: TSS ()
Subject: Atypical scrapie in a swiss goat and implications for transmissible spongiform encephalopathy surveillance
Date: May 1, 2007 at 2:02 pm PST

Full Scientific Reports

Atypical scrapie in a swiss goat and implications for transmissible spongiform encephalopathy surveillance

Torsten Seuberlich1, Catherine Botteron, Sylvie L. Benestad, Hervé Brünisholz, Reto Wyss, Ulrich Kihm, Heinzpeter Schwermer, Martina Friess, Alexandra Nicolier, Dagmar Heim and Andreas Zurbriggen
Correspondence: 1 Corresponding Author: Torsten Seuberlich, NeuroCenter, Department of Clinical Veterinary Medicine, Vetsuisse Faculty, Bremgartenstrasse 109a, CH-3001 Berne, Switzerland, e-mail:

Different types of transmissible spongiform encephalopathies (TSEs) affect sheep and goats. In addition to the classical form of scrapie, both species are susceptible to experimental infections with the bovine spongiform encephalopathy (BSE) agent, and in recent years atypical scrapie cases have been reported in sheep from different European countries. Atypical scrapie in sheep is characterized by distinct histopathologic lesions and molecular characteristics of the abnormal scrapie prion protein (PrPsc). Characteristics of atypical scrapie have not yet been described in detail in goats. A goat presenting features of atypical scrapie was identified in Switzerland. Although there was no difference between the molecular characteristics of PrPsc in this animal and those of atypical scrapie in sheep, differences in the distribution of histopathologic lesions and PrPsc deposition were observed. In particular the cerebellar cortex, a major site of PrPsc deposition in atypical scrapie in sheep, was found to be virtually unaffected in this goat. In contrast, severe lesions and PrPsc deposition were detected in more rostral brain structures, such as thalamus and midbrain. Two TSE screening tests and PrPsc immunohistochemistry were either negative or barely positive when applied to cerebellum and obex tissues, the target samples for TSE surveillance in sheep and goats. These findings suggest that such cases may have been missed in the past and could be overlooked in the future if sampling and testing procedures are not adapted. The epidemiological and veterinary public health implications of these atypical cases, however, are not yet known.


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