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Abstract

Reports of secondary bloodborne transmission of vCJD add to the uncertainty about the future of the vCJD outbreak.

Abstract

The only variant Creutzfeldt-Jakob disease (vCJD) patient identified in the United States died in 2004, and the diagnosis was confirmed by analysis of autopsy tissue. The patient likely acquired the disease while growing up in Great Britain before immigrating to the United States in 1992. Additional vCJD patients continue to be identified outside the United Kingdom, including 2 more patients in Ireland, and 1 patient each in Japan, Portugal, Saudi Arabia, Spain, and the Netherlands. The reports of bloodborne transmission of vCJD in 2 patients, 1 of whom was heterozygous for methionine and valine at polymorphic codon 129, add to the uncertainty about the future of the vCJD outbreak.

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A new variant of Creutzfeldt-Jakob disease in the UK.

M. Pocchiari, S N Cousens, R Will … (1996)

Epidemiological surveillance of Creutzfeldt-Jakob disease (CJD) was reinstituted in the UK in 1990 to identify any changes in the occurrence of this disease after the epidemic of bovine spongiform encephalopathy (BSE) in cattle. Case ascertainment of CJD was mostly by direct referral from neurologists and neuropathologists. Death certificates on which CJD was mentioned were also obtained. Clinical details were obtained for all referred cases, and information on potential risk factors for CJD was obtained by a standard questionnaire administered to patients' relatives. Neuropathological examination was carried out on approximately 70% of suspect cases. Epidemiological studies of CJD using similar methodology to the UK study have been carried out in France, Germany, Italy, and the Netherlands between 1993 and 1995. Ten cases of CJD have been identified in the UK in recent months with a new neuropathological profile. Other consistent features that are unusual include the young age of the cases, clinical findings, and the absence of the electroencephalogram features typical for CJD. Similar cases have not been identified in other countries in the European surveillance system. These cases appear to represent a new variant of CJD, which may be unique to the UK. This raises the possibility that they are causally linked to BSE. Although this may be the most plausible explanation for this cluster of cases, a link with BSE cannot be confirmed on the basis of this evidence alone. It is essential to obtain further information on the current and past clinical and neuropathological profiles of CJD in the UK and elsewhere.

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The public health impact of prion diseases.

Ermias D. Belay, Lawrence B. Schonberger (2004)

Several prion disease-related human health risks from an exogenous source can be identified in the United States, including the iatrogenic transmission of Creutzfeldt-Jakob disease (CJD), the possible occurrence of variant CJD (vCJD), and potential zoonotic transmission of chronic wasting disease (CWD). Although cross-species transmission of prion diseases seems to be limited by an apparent "species barrier," the occurrence of bovine spongiform encephalopathy (BSE) and its transmission to humans indicate that animal prion diseases can pose a significant public health risk. Recent reports of secondary person-to-person spread of vCJD via blood products and detection of vCJD transmission in a patient heterozygous at codon 129 further illustrate the potential public health impacts of BSE.

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Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States.

Pierluigi Gambetti, Ryan Maddox, Ermias D. Belay … (2003)

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Author and article information

Journal

Journal ID (nlm-ta): Emerg Infect Dis

Journal ID (iso-abbrev): Emerging Infect. Dis

Journal ID (publisher-id): EID

Title: Emerging Infectious Diseases

Publisher: Centers for Disease Control and Prevention

ISSN (Print): 1080-6040

ISSN (Electronic): 1080-6059

Publication date (Print): September 2005

Volume: 11

Issue: 9

Pages: 1351-1354

Affiliations

[* ]Centers for Disease Control and Prevention, Atlanta, Georgia, USA;

[† ]Florida Department of Health, Tallahassee, Florida, USA;

[‡ ]Case Western Reserve University, Cleveland, Ohio, USA

Author notes

Address for correspondence: Ermias D. Belay, Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, 1600 Clifton Road, Mailstop A39, Atlanta, GA 30333, USA; fax: 404-639-3838; email: ebelay@ 123456cdc.gov

Article

Publisher ID: 05-0371

DOI: 10.3201/eid1109.050371

PMC ID: 3310634

PubMed ID: 16229761

SO-VID: ba5bbe4f-9f51-475c-8af6-884a9d7afb62

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Variant Creutzfeldt-Jakob Disease Death, United States

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Most cited references 4

A new variant of Creutzfeldt-Jakob disease in the UK.

The public health impact of prion diseases.

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States.

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