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

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


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Animals
  • Biopsy
  • Brain / metabolism
  • Brain / pathology
  • Cattle
  • Creutzfeldt-Jakob Syndrome / diagnosis*
  • Creutzfeldt-Jakob Syndrome / epidemiology*
  • Creutzfeldt-Jakob Syndrome / transmission
  • Electrocardiography
  • Female
  • Global Health
  • Humans
  • Immunohistochemistry
  • Magnetic Resonance Imaging
  • Male
  • Population Surveillance
  • Tomography, X-Ray Computed
  • United States / epidemiology