Sludge Watch ==> United States: Third Case of vCJD

[Maureen Reilly]

Third case of vCJD reported in the United States
07.dec.06
Eurosurveillance (Volume 11, Issue 12)
Eurosurveillance Editorial team
http://www.eurosurveillance.org/ew/2006/061207.asp

A clinical diagnosis of variant Creutzfeldt Jakob Disease (vCJD) was 
confirmed after brain biopsy investigations in a United States (US) resident 
and reported in November [1]. The patient is a young man who grew up in 
Saudi Arabia and lived in the US since late 2005. Before that he visited the 
US once in 1989 and several times after 2001. He has never visited any 
country in Europe or received a blood transfusion nor has he undergone any 
neurosurgical procedure. This vCJD case is the third in a US resident. The 
previous two patients both grew up in the United Kingdom (UK), and this is 
where they were believed to have been infected [2].
In Saudi Arabia, the first and only previous case of vCJD was reported in 
2005. This was suspected to be related to consumption of meat contaminated 
with the prion agent which causes bovine spongiform encephalitis in cattle 
(BSE). The European Food Safety Authority (http://www.efsa.org) has not 
published a geographical BSE risk assessment for Saudi Arabia [3] and there 
have been no cases of BSE in cattle reported by Saudi Arabia to the World 
Organisation for Animal Health (http://www.oie.int). Although the UK is not 
the only potential beef exporter to have had a BSE epidemic, it remains 
plausible, subject to Saudi Arabia's import policy, that contaminated beef 
was inadvertently imported from the UK to Saudi Arabia in the period before 
1996 (when the EU banned the export of UK beef and cattle).
Based on this patient's history, the occurrence of a previously reported 
case of vCJD in Saudi Arabia, and the expected length of the incubation 
period for food-related vCJD, the most likely source of infection is thought 
to be contaminated meat products the patient consumed as a child when living 
in Saudi Arabia. The patient has no known history of donating blood, and 
investigations have identified no risk of onwards transmission within the 
US.
Variant Creutzfeldt-Jakob disease was first identified in the United Kingdom 
in the mid-1990s. As of November 2006, worldwide there have been 200 vCJD 
cases: 164 patients in the United Kingdom, 21 in France, four in Ireland, 
three in the US (including the present case), two in the Netherlands and one 
each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain [4]. All 
patients, except 10 (including the present case) had lived either in the 
United Kingdom (170 cases) or in France (20 cases). Evidence so far 
indicates that the most probable source of infection in most cases was 
consumption of meat products contaminated with the prion agent causing BSE.
References:
1. Centers for Disease Control and Prevention. Confirmed Case of Variant 
Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the 
Middle East. (http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm)
2. Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter 
S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob 
disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354.
3. European Food Safety Authority . Geographical BSE Risk (GBR) assessments 
covering 2000-2006. List of countries and their GBR level of risk as 
assessed by the Scientific Steering Committee and the (EFSA). 1 August 2006. 
(http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/summary_list_countries.Par.0001.File.dat/GBR_assessments_table_Overview_assessed_countries_2002-2006.pdf)
4. Variant Creuzfeldt-Jakob disease. Current data - December 2006. 
(http://www.cjd.ed.ac.uk/vcjdworld.htm)