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Emerg Infect Dis. 2015 May;21(5):750-9. doi: 10.3201/eid2105.142017.

Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications.

Abstract

Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. We describe the clinical presentation of a recent case of vCJD in the United States and provide an update on diagnostic testing. The location of this patient's exposure is less clear than those in the 3 previously reported US cases, but strong evidence indicates that exposure to contaminated beef occurred outside the United States more than a decade before illness onset. This case exemplifies the persistent risk for vCJD acquired in unsuspected geographic locations and highlights the need for continued global surveillance and awareness to prevent further dissemination of vCJD.

KEYWORDS:

Creutzfeldt-Jakob disease; PMCA; Prions and related diseases; United Kingdom; United States; bovine spongiform encephalopathy; cortical ribbon sign; global health; mad cow disease; pulvinar sign

PMID:
25897712
PMCID:
PMC4412247
DOI:
10.3201/eid2105.142017
[Indexed for MEDLINE]
Free PMC Article

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