Variant Creutzfeldt-Jakob Disease Death, United States

• Main Authors: Ermias D. Belay, James J. Sejvar, Wun-Ju Shieh, Steven T. Wiersma, Wen-Quan Zou, Pierluigi Gambetti, Stephen Hunter, Ryan A. Maddox, Landis Crockett, Sherif R. Zaki, Lawrence B. Schonberger
• Format: Article
• Language: English
• Published: Centers for Disease Control and Prevention 2005-09-01
• Series: Emerging Infectious Diseases
• Subjects: Creutzfeldt-Jakob disease; variant Creutzfeldt-Jakob disease; prion disease; transmissible spongiform encephalopathy; epidemiology; surveillance
• Online Access: https://wwwnc.cdc.gov/eid/article/11/9/05-0371_article

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.