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CJD (Creutzfeldt-Jakob Disease,
Classic)
About CJD
Classic CJD is a human prion
disease. It is a neurodegenerative disorder with characteristic
clinical and diagnostic features. This disease is rapidly
progressive and always fatal. Infection with this disease leads to
death usually within 1 year of onset of illness.
Important Note: Classic CJD is not related to "mad
cow" disease. Classic CJD also is distinct from "variant CJD",
another prion disease that is related to BSE.
For information about these
diseases, see:
Occurrence and Transmission
Classic CJD has been recognized
since the early 1920s. The most common form of classic CJD is
believed to occur sporadically, caused by the spontaneous
transformation of normal prion proteins into abnormal prions. This
sporadic disease occurs worldwide, including the United States, at a
rate of approximately one case per 1 million population per year,
although rates of up to two cases per million are not unusual. The
risk of CJD increases with age, and in persons aged over 50 years of
age, the annual rate is approximately 3.4 cases per million. In
recent years, the United States has reported fewer than 300 cases of
CJD a year.
Whereas the majority of cases of
CJD (about 85%) occur as sporadic disease, a smaller proportion of
patients (5-15%) develop CJD because of inherited mutations of the
prion protein gene. These inherited forms include
Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.
Creutzfeldt-Jakob Disease Deaths and Age-Adjusted Death Rate,
United States, 1979-2004
(See graph below.)
Clinical and Pathologic
Characteristics of Classic CJD
(See table below.)
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vCJD (Variant Creutzfeldt-Jakob Disease)
About vCJD Variant CJD was
first described in 1996 in the United Kingdom. It has different
clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile
of the prion protein gene. (See
table below). The median age at death for vCJD patients is 28
years, compared with 68 years for patients with classic CJD. The
median duration of illness for vCJD is 14 months, compared to 5
months for classic CJD.
Clinical and Pathologic Characteristics
Distinguishing Classic CJD from
variant CJD
(See
table below.)
Evidence
for Relationship with BSE (Mad Cow Disease)
Since 1996,
evidence has been increasing for a causal relationship between
ongoing outbreaks in Europe of a disease in cattle, called bovine
spongiform encephalopathy (BSE, or 'mad cow' disease), and vCJD.
There is now strong scientific evidence that the agent responsible
for the outbreak of prion disease in cows, BSE, is the same agent
responsible for the outbreak of vCJD in humans. Both disorders are
invariably fatal brain diseases with unusually long incubation
periods measured in years, and are caused by an unconventional
transmissible agent. However, this evidence also suggests that the
risk is low for having vCJD, even after consumption of contaminated
product. In 1996, because of the emergence of vCJD in the United
Kingdom, CDC enhanced its surveillance for CJD in the United States.
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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
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