Confirmed Case of Variant Creutzfeldt Jakob Disease
(vCJD) in the United
States in a Patient from the Middle East
The Virginia Department of Health and the Centers for Disease Control and
Prevention announce the recent confirmation of a vCJD case in a U.S.
resident. This is the third vCJD case identified in a U.S. resident. This
latest U.S. case occurred in a young adult who was born and raised in Saudi
Arabia and has lived in the United States since late 2005. The patient
occasionally stayed in the United States for up to 3 months at a time since
2001 and there was a shorter visit in 1989. In late November 2006, the
Clinical Prion Research Team at the University of California San Francisco
Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic
study of adenoid and brain biopsy tissues. The two previously reported vCJD
case-patients in U.S. residents were each born and raised in the United
Kingdom (U.K.), where they were believed to have been infected by the agent
responsible for their disease. There is strong scientific evidence that the
agent causing vCJD is the same agent that causes bovine spongiform
encephalopathy (BSE, commonly known as mad cow disease).
Variant CJD is a rare, degenerative, fatal brain disorder that emerged in
the United Kingdom in the mid-1990s. Although experience with this new
disease is limited, evidence to date indicates that there has never been a
case transmitted from person-to-person except through blood transfusion.
Instead, the disease is thought to result primarily from consumption of
cattle products contaminated with the BSE agent. Although no cases of BSE in
cattle have been reported in Saudi Arabia, potentially contaminated cattle
products from the United Kingdom may have been exported to Saudi Arabia for
many years during the large U.K. BSE outbreak.
The current case-patient has no history of receipt of blood, a past
neurosurgical procedure, or residing in or visiting countries of Europe.
Based on the patient's history, the occurrence of a previously reported
Saudi case of vCJD attributed to likely consumption of BSE-contaminated
cattle products in Saudi Arabia, and the expected greater than 7 year
incubation period for food-related vCJD, this U.S. case-patient was most
likely infected from contaminated cattle products consumed as a child when
living in Saudi Arabia (1). The current patient has no history of donating
blood and the public health investigation has identified no risk of
transmission to U.S. residents from this patient.
As of November 2006, 200 vCJD patients were reported world-wide, including
164 patients identified in the United Kingdom, 21 in France, 4 in the
Republic of Ireland, 3 in the United States (including the present
case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan,
Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all
except 10 of them (including the present case-patient) had resided either in
the United Kingdom (170 cases) for over 6 months during the 1980-1996 period
of the large UK BSE outbreak or alternatively in France (20 cases).
As reported in 2005 (1), the U.S. National Prion Disease Pathology
Surveillance Center at Case Western Reserve University confirmed the
diagnosis in the one previously identified case of vCJD in a Saudi resident.
He was hospitalized in Saudi Arabia and his brain biopsy specimen was
shipped to the United States for analysis. This earlier vCJD case-patient
was believed to have contracted his fatal disease in Saudi Arabia (1).
1) 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.
Date: November 29, 2006
Content source: National Center for Infectious Diseases
http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm