title: 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.
authors.forename: Atul
authors.initials: A
authors.lastname: Maheshwari
authors.forename: Michael
authors.initials: M
authors.lastname: Fischer
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Gambetti
authors.forename: Alicia
authors.initials: A
authors.lastname: Parker
authors.forename: Aarthi
authors.initials: A
authors.lastname: Ram
authors.forename: Claudio
authors.initials: C
authors.lastname: Soto
authors.forename: Luis
authors.initials: L
authors.lastname: Concha-Marambio
authors.forename: Yvonne
authors.initials: Y
authors.lastname: Cohen
authors.forename: Ermias D
authors.initials: ED
authors.lastname: Belay
authors.forename: Ryan A
authors.initials: RA
authors.lastname: Maddox
authors.forename: Simon
authors.initials: S
authors.lastname: Mead
authors.forename: Clay
authors.initials: C
authors.lastname: Goodman
authors.forename: Joseph S
authors.initials: JS
authors.lastname: Kass
authors.forename: Lawrence B
authors.initials: LB
authors.lastname: Schonberger
authors.forename: Haitham M
authors.initials: HM
authors.lastname: Hussein
mesh_terms: D000328:Adult; D000818:Animals; D001706:Biopsy; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000453:epidemiology* / Q000635:transmission; D004562:Electrocardiography; D005260:Female; D014943:Global Health; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D011159:Population Surveillance; D014057:Tomography, X-Ray Computed; D014481:United States / Q000453:epidemiology
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