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Volume 12, Number 12—December 2006

Perspective

On the Question of Sporadic or Atypical Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease

Paul Brown*Comments to Author , Lisa M. McShane†, Gianluigi Zanusso‡, and Linda Detwiler§
Author affiliations: *Bethesda, Maryland, USA; †National Institutes of Health, Bethesda, Maryland, USA; ‡University of Verona, Verona, Italy,; §Virginia-Maryland Regional College of Veterinary Medicine, College Park, Maryland, USA

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Table 2

Summary of atypical cases of bovine spongiform encephalopathy (BSE)

Country Age, y Breed Symptoms Neuropathology
Western blot pattern*
Spongiform changes Immunohistochemistry
Italy 11 Bruna Alpina None Mild Plaques L
15 Piemontese None Mild Plaques L
Denmark 14 Charolais None NR NR L
Poland 12 Black-white breed None Present Positive (no plaques) L
Japan 2 Holstein None Absent Negative L1
14 Japanese Black Dystasia Severe Positive (no plaques) H
Belgium 5.5 East-Flemish None Absent Negative L1
France 10 Cross breed None NR NR H
15 Prim Holstein None NR NR H
8 Charolais None NR NR H
The Netherlands 13 Black-white Holstein, Freisian NR Present No plaques H
Sweden 12 Mixed Charolais Recumbent NR Positive (no plaques) H1
Switzerland 19 Zebu Typical BSE Typical BSE Positive (no plaques) H
Germany 13 Angus NR Absent Positive (no plaques) H
15 Holstein-Freisian NR Absent Positive (no plaques) L
USA 12 Brahma cross Falling Absent No plaques H
10 Red crossbred Recumbent Absent No plaques H
Canada 16 Charolais Recumbent NR Positive (no plaques) H

*L, lower molecular weight; H, higher molecular weight (the 2 major Western blot PrP glycopatterns that distinguish the strains from each other and from the pattern seen in typical BSE); NR, not reported. Only the Italian cows and Swiss zebu had full neuropathologic examinations (others were limited to examination of the obex). Details are not available for additional animals with both H and L strains in France and Poland.

*L, lower molecular weight; H, higher molecular weight (the 2 major Western blot PrP glycopatterns that distinguish the strains from each other and from the pattern seen in typical BSE); NR, not reported. Only the Italian cows and Swiss zebu had full neuropathologic examinations (others were limited to examination of the obex). Details are not available for additional animals with both H and L strains in France and Poland.

*L, lower molecular weight; H, higher molecular weight (the 2 major Western blot PrP glycopatterns that distinguish the strains from each other and from the pattern seen in typical BSE); NR, not reported. Only the Italian cows and Swiss zebu had full neuropathologic examinations (others were limited to examination of the obex). Details are not available for additional animals with both H and L strains in France and Poland.

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