Disease (CJD) is a brain disorder that usually occurs in people over the age of
60. It is sometimes called a “spongiform” disease because the brain may develop
holes in it like a sponge. There are four types of CJD: sporadic, variant,
familial and iatrogenic. Sporadic CJD occurs spontaneously and is the most
common type of CJD in the United States. Variant CJD is associated with eating
beef in the United Kingdom during the “mad cow” epidemic. Familial CJD is
inherited through a genetic mutation. Iatrogenic CJD is transmitted by
contaminated surgical instruments in high risk surgeries involving transplants
of brain and spinal cord tissue. Every year about one person out of every
million is diagnosed with CJD, primarily sporadic. There is no known treatment;
most people die within 3 to 12 months of onset of symptoms.
Signs And Symptoms of CJD?
At first, CJD can seem a lot like other diseases of the elderly.
Symptoms include behavioral changes, confusion, difficulty remembering recent
events, and loss of feeling in the arms, legs, or face. Patients may lose their
balance or seem uncoordinated; they may have difficulty walking or have muscle
jerks and spasms. A brain wave test (EEG) is often helpful in the diagnosis.
Other special testing includes cerebrospinal fluid 14–3–3 protein and blood
tests for genetic mutation. To be sure the person has died of CJD an autopsy
must be done. The National Prion Disease
Pathology Surveillance Center provides
free cerebrospinal fluid 14–3–3 protein tests, blood tests for genetic testing,
autopsy arrangement and tissue examination.
1980’s scientists discovered a new type of agent: an infectious protein called
a prion. Humans and other animals have normal prions which are found throughout
the body with larger concentrations in the brain. Scientists are still not sure
how prions work. Current theory is the abnormal prion converts the normal prion
to the abnormal form destroying brain tissue and giving it a sponge like
You Get CJD?
not known how most people get this rare disease, although we do know you do not
get it from being around someone with CJD. Approximately 10–15 percent of cases
are inherited (familial CJD) and a few cases have been related to transplants
of tissues from the nervous system (iatrogenic CJD). In 1995, the first case of
variant CJD was recognized in the United Kingdom and has been linked to
ingestion of foods from cattle infected with “mad cow disease.”
There Been Any Cases of “Mad Cow Disease” in the United States?
Bovine spongiform encephalopathy (BSE) or
“mad cow disease” is a disease specific to cattle. There are different strains
of BSE categorized as Typical BSE strain (responsible for the outbreak in the
United Kingdom) and Atypical BSE Strains (H an L strains). The Atypical strains,
H and L strains, are thought to represent sporadic disease (spontaneously occurring
or through environmental factors yet to be identified). The typical strain has
not been identified in any U.S.-born cattle.
There have been four cases of BSE in cattle
identified in the United States. The first case of BSE was identified in
Washington State (2002) and the cow was found to have been imported from Canada
(non-U.S. born). Most of the BSE cases in Canada are associated with the
“classical” or typical strain seen in the UK outbreak and causally linked to
vCJD in humans. In 2004 the second case
of BSE was identified in a Texas-born cow representing the first native case in
the United States. A second native case (3rd U.S. case) was reported
in an Alabama bovine in 2006. In 2012, the 3rd native case (4th U.S. case) of BSE
was reported in California. All native (U.S. born) cases were of the atypical
BSE Strain, which has not yet been linked to human disease.
There Been Any Cases of Variant CJD in the United States?
there have been four cases of variant CJD identified in humans in the
case-The patient was born in the United Kingdom in the late 1970s and lived
there until relocating to Florida in 1995. Onset of symptoms began in 2001 and
the patient died in 2004.
Second case–The patient was born and raised in England before moving to Texas
in 2001. Symptoms began in 2005 and were confirmed neuropathologically (by
autopsy) in 2006 by experts in the United Kingdom. Both the first and second
case-patients are believed to have been exposed to the BSE agent while residing
in the United Kingdom during the defined period of risk (1980–1996).
case–The patient was born and raised in Saudi Arabia and resided in the United
States since 2005. Variant CJD was neuropathologically confirmed by biopsy in
2006. Investigators believe that exposure most likely occurred from consumption
of contaminated cattle imported from the UK while residing in Saudi Arabia as a
The Texas Department of State Health Services (DSHS) in
conjunction with the CDC completed the investigation of the fourth vCJD case in
the United States. It confirmed that the affected individual was born outside
the Americas and indicated exposure to the BSE agent (infective agent linked to
human vCJD) most likely occurred before he moved to the United States; the
individual previously resided in Kuwait, Russia and Lebanon. Our investigation
did not support frequent travel or travel of any significant duration to
European countries. In absence of a definite travel link to a country where
other known vCJD cases were likely infected, it is less clear as to which
specific overseas country the individual’s exposure occurred. The
neuropathological analysis performed by the National Prion Disease Pathology
Surveillance Center (NPDPSC) at Case Western Reserve University (http://case.edu/med/pathology/centers/npdpsc/) confirmed the clinical diagnosis of vCJD.