Creutzfeld-Jakob Disease (CJD)
May 2008
What is Creutzfeld-Jakob disease?
Creutzfeldt-Jakob disease (CJD) is the most common
human prion disease. Prion diseases are a group of rare brain and nervous
system diseases that affect humans and some kinds of animals. They are also
known as transmissible spongiform encephalopathies (TSEs). Prion diseases
are not caused by germs (i.e., viruses, bacteria). Instead, they are a
result of normal brain proteins changing shape and folding into abnormal
forms. In affected people, clumps of prion proteins cause brain damage and
death.
CJD occurs worldwide in about one to two persons per
million each year. In Washington State it affects about six people annually.
In all cases, the disease is fatal.
Who is at risk for being affected by CJD?
In the United States, CJD most often occurs in
people between 55 and 75 years old. CJD is not spread from person to person.
In some cases, 5-15 percent, there is a genetic cause for CJD, but in most
cases (about 85 percent), no cause can be determined. When no cause can be
determined, the disease is called “sporadic” CJD. Very rarely, CJD has been
spread during neurosurgical procedures or human-derived hormone therapies
(no longer in use).
Another form of CJD, called variant CJD or vCJD, has
affected more than 200 people. Most of those affected were 15-55 years old
and lived in or visited the United Kingdom at some time during an epidemic
of “mad cow disease” that occurred from1980-1996. Mad cow disease is a
cattle prion disease also known as bovine spongioform encephalopathy (BSE).
The cause of vCJD is probably consumption of meat products from BSE-affected
cattle. There have not been any cases of vCJD acquired in the United States.
There are many measures in place to prevent BSE in cattle and to protect the
food supply from contamination.
What are the symptoms of CJD?
People with CJD develop dementia and quickly
deteriorate mentally. Involuntary twitching, rigid or spasming muscles, lack
of coordination and balance, and visual problems are also common symptoms.
CJD always leads to death. Approximately half of affected people die within
six months of the time symptoms begin, and 90 percent succumb within twelve
months.
How is CJD diagnosed?
Symptoms, family history, a lab test, and brain
tests such as EEGs and MRIs can help healthcare providers determine that a
patient may have CJD. However, the definite diagnosis of CJD requires
testing of brain tissue most commonly collected after death in an autopsy.
When a person has an illness that could be CJD, it is important that the
person’s family talk to their physician about getting a clear diagnosis
after their loved one’s death. There is no charge for the autopsy, transport
of the body back to the family, or specialized laboratory testing. Tests are
performed at the National Prion Disease Pathology Surveillance Center
www.cjdsurveillance.com
How is CJD treated?
There is currently no treatment for CJD. The goal
of care is to make the patient comfortable before death.
To support patients’ families, the CJD Foundation
operates a national toll-free line at (800) 659-1991 and a Web site:
http://www.cjdfoundation.org/
For more information call your local
health department or Communicable Disease Epidemiology (206) 418-5500 or
toll-free 877-539-4344.
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