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Creutzfeldt Jakob Disease (CJD) ICD-9 046.1; ICD-10 A81.0

What is CJD?

Creutzfeldt-Jakob 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.

What Are 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.

What Causes CJD?

In the 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 appearance.

How Do You Get CJD?

It is 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.”

Have 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 have been three 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. In 2004 BSE was identified in a Texas-born cow representing the first native case in the United States. A second native case was reported in an Alabama bovine in 2006.

Have There Been Any Cases of Variant CJD in the United States?

Yes, there have been three cases of variant CJD identified in humans in the United States. The first case-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.
The second case–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).
The third case–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 while residing in Saudi Arabia as a child.

Last Updated: Monday, September 17, 2007

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