Parasites, Viruses, and Diseases - A to Z - CJD

Though CJD is not Mad Cow Disease - there is a link so...

Mad Cow Giant Microbes

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder.


Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year.

Symptoms
In the early stages of disease
Failing memory
Behavioural changes
Lack of coordination
Slurred Speech 
Visual disturbances

As the illness progresses
Mental deterioration
Involuntary movements
Blindness
Weakness of extremities
Coma may occur.

NIH

Heart failure, respiratory failure, pneumonia or other infections are generally the cause of death. The disease usually runs its course in about seven months, although a few people may live up to one or two years after diagnosis.

The cause of Creutzfeldt-Jakob disease and other TSEs appears to be abnormal versions of a kind of protein called a prion.
Normally, these proteins are harmless, but when they're misshapen they become infectious and can wreak havoc on normal biological processes




Transmission

There are 3 major categories of CJD

1. Sporadic
2. Inherited
3. Acquired 

1. Sporadically. Most people with classic CJD develop the disease for no apparent reason. CJD that occurs without explanation is termed spontaneous CJD or sporadic CJD and accounts for the majority of cases.
2. By inheritance. In the United States, about 5 to 10 percent of people with CJD have a family history of the disease or test positive for a genetic mutation associated with CJD. This type is referred to as familial CJD.
3. By contamination. A small number of people have developed CJD after being exposed to infected human tissue during a medical procedure, such as a cornea or skin transplant. Also, because standard sterilization methods do not destroy abnormal prions, a few people have developed CJD after undergoing brain surgery with contaminated instruments. Cases of CJD related to medical procedures are referred to as iatrogenic CJD. Variant CJD is linked primarily to eating beef infected with bovine spongiform encephalopathy (BSE), the medical term for mad cow disease. 

There is a rare version of CJD called Variant CJD
 Variant CJD is likely to be caused by consuming meat from a cow that has been infected with a similar prion disease called bovine spongiform encephalopathy (BSE) - also known as 'mad cow disease'.
 ( that's the one you have probably heard more about due to the outbreak and mass media coverage) CDC


Risk Factors 

• Age. Sporadic CJD tends to develop later in life, usually around the age of 60. Onset of familial CJD occurs only slightly earlier. On the other hand, vCJD has affected people at a much younger age, usually in their late 20s.
• Genetics. People with familial CJD have a genetic mutation that causes the disease. The disease is inherited in an autosomal dominant fashion, which means you need to inherit only one copy of the mutated gene, from either parent, to develop the disease. If you have the mutation, the chance of passing it on to your children is 50 percent. Genetic analysis in people with iatrogenic and variant CJD suggest that inheriting identical copies of certain variants of the prion gene may predispose a person to developing CJD if exposed to contaminated tissue.
• Exposure to contaminated tissue. People who've received human growth hormone derived from human pituitary glands or who've had dura mater grafts may be at risk of iatrogenic CJD. The risk of contracting vCJD from eating contaminated beef is difficult to determine. In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent. 

 Mayo Clinic



 Only a brain biopsy or an examination of brain tissue after death (autopsy) can confirm the presence of Creutzfeldt-Jakob disease. 
 Not super helpful if you happen to be alive

 Rut Row Canada...



CJD Foundation 


Current Statistics PDF

 Canadian Statistics PDF

Feb 13 BSE reported in Alberta