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1 : New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases

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New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases, Page 1 of 2

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Abstract:

This chapter updates an article written in June 1997 and is based on the accumulating data available in late November 1997. There is an increasing evidence for an etiologic link between the bovine spongiform encephalopathy (BSE) epizootic in the United Kingdom and the emergence of a new variant form of Creutzfeldt-Jakob disease (nvCJD). Both CJD in humans and BSE in cattle are invariably fatal, subacute, degenerative diseases of the brain that are classified as transmissible spongiform encephalopathies (TSEs). The source of the BSE epizootic is uncertain. The leading hypothesis is that it resulted from feeding cattle rendered protein, such as meat-and-bone meal, produced from the carcasses of scrapie-infected sheep in the early 1980s, after most rendering plants abandoned the use of organic solvents in the preparation of this feed. This hypothesis best explains the occurrence of the epizootic predominantly in the United Kingdom, where the ratio of the numbers of sheep to cattle and the rate of endemic scrapie are both high. The currently available pathological, clinical, and epidemiologic information on the newly described variant of CJD provides convincing evidence that it is, in fact, a novel variant. The accumulating experimental and epidemiologic data provide increasingly strong support for a causal link between BSE and nvCJD.

Citation: Schonberger L. 1998. New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases, p 1-15. In Scheld W, Craig W, Hughes J (ed), Emerging Infections 2. ASM Press, Washington, DC. doi: 10.1128/9781555816957.ch1

Key Concept Ranking

Bovine Spongiform Encephalopathy
0.8362233
Infectious Diseases
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Prion Proteins
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Transmissible Spongiform Encephalopathies
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0.8362233
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Figures

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Figure 1

Confirmed BSE, by quarter of year of onset in Great Britain from 1 September 1986 to 30 September 1997 (reported through 28 November 1997). Adapted from references by Wilesmith et al. ( ) and Smith ( ).

Citation: Schonberger L. 1998. New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases, p 1-15. In Scheld W, Craig W, Hughes J (ed), Emerging Infections 2. ASM Press, Washington, DC. doi: 10.1128/9781555816957.ch1
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Image of Figure 2
Figure 2

Kuru–type plaque surrounded by a zone of spongiform change in the frontal lobe of a patient with nvCJD (arrow). The sample was stained with hematoxylin and cosin. Photomicrograph courtesy of J. W. Ironside.

Citation: Schonberger L. 1998. New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases, p 1-15. In Scheld W, Craig W, Hughes J (ed), Emerging Infections 2. ASM Press, Washington, DC. doi: 10.1128/9781555816957.ch1
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Image of Figure 3
Figure 3

nvCJD cases, by year of onset and year of death. 1994 to September 1997. Data are from the Department of Health of the United Kingdom ( ), Cousens et al. ( ), and Will ( ). One patient was still alive.

Citation: Schonberger L. 1998. New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases, p 1-15. In Scheld W, Craig W, Hughes J (ed), Emerging Infections 2. ASM Press, Washington, DC. doi: 10.1128/9781555816957.ch1
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Image of Figure 4
Figure 4

CJD deaths and death rates by age group in the United Slales, 1979 to 1995.

Citation: Schonberger L. 1998. New-Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: the Strengthening Etiologic Link between Two Emerging Diseases, p 1-15. In Scheld W, Craig W, Hughes J (ed), Emerging Infections 2. ASM Press, Washington, DC. doi: 10.1128/9781555816957.ch1
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References

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