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Home Eurosurveillance Weekly Release  2002: Volume 6/ Issue 43 Article 3
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Eurosurveillance, Volume 6, Issue 43, 24 October 2002
Articles

Citation style for this article: Molesworth AM. First case of variant Creutzfeldt-Jakob disease reported in a United States resident. Euro Surveill. 2002;6(43):pii=1919. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1919

First case of variant Creutzfeldt-Jakob disease reported in a United States resident

Last week, the first case of probable variant Creutzfeldt-Jakob disease (vCJD) in a United States (US) resident was documented in the Morbidity and Mortality Weekly Report (1). The patient, a 22 year old woman living in Florida, first developed neuropsychiatric symptoms in November 2001, and was initially reported in April 2002 (2).

There is no evidence that the patient acquired vCJD through medical procedures or treatments that might be associated with the transmission of prion diseases. Scientific evidence suggests that vCJD is caused by transmission of the bovine spongiform encephalopathy (BSE) agent to humans, believed to have most probably occurred through eating beef or other bovine derived products that contained the BSE agent. The patient was born in the United Kingdom (UK) in 1979, and had lived in the UK when the UK BSE outbreak was increasing, and the risk for human exposures was probably at its peak (3-5), until moving to Florida in 1992. To date, no case of BSE has been identified in cattle in the US (6). It is likely that the patient was exposed to BSE before moving to the US, and would have acquired her infection during the period 1980-1992. This would put the interval between exposure to BSE and onset of illness as 9 to 21 years.

As of early October 2002, a total of 138 cases of probable or definite vCJD have been reported worldwide. Most of the individuals who became infected had spent multiple years in the UK between 1980 and 1996. That a case should be identified in the US was not unexpected, and the report emphasizes the importance of considering vCJD in patients with relevant clinical features who have spent time in areas in which BSE has occurred. 

References :

  1. CDC. Probable variant Creutzfeldt-Jakob disease in a U.S. resident – Florida, 2002. MMWR Morb Mortal Wkly Rep 2002; 51: 927-9. (http://www.cdc.gov/mmwr//preview/mmwrhtml/mm5141a3.htm).
  2. CDC. CDC and Florida Department of Health investigate a likely cause of new variant Creutzfeldt Jakob disease in a U.K. citizen residing in the U.S. Press release. 18 April 2002. (http://www.cdc.gov/od/oc/media/pressrel/r020418.htm).
  3. Brown P, Will RG, Bradley R, Asher DM, Detwiler L. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution and current concerns. Emerg Infect Dis 2001; 7: 6-16. (http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm)
  4. Spongiform Encephalopathy Advisory Committee. Statement of 20 March 1996. (http://www.defra.gov.uk/animalh/bse/bse-publications/level-4-seacstate-20mar96.html)
  5. The BSE Inquiry. Report, evidence and supporting papers of the inquiry into the emergence and identification of Bovine Spongiform Encephalopathy (BSE) and variant Creutzfeldt-Jakob disease(vCJD) and the action taken in response to it up to 20 March 1996. Lord Phillips of Worth Matravers, Chairman. London: The Stationary Office; October 2000.
  6. United States Department of Agriculture. BSE surveillance data, to 30 September 2002. (http://www.aphis.usda.gov/oa/bse/bsesurvey.html#charts).

Reported by Anna Molesworth (amolesworth@phls.org.uk), Public Health Laboratory Service Communicable Disease Surveillance Centre, London, England.

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