Creutzfeldt-Jacob Disease (aka Mad Cow)

Konstantine Adamopoulosand

Emma Gillam

What is Mad Cow Disease?

• Mad cow is a “fatal neurodegenerative disease in cattle.”

• It can be transmitted to humans through ingesting of infected beef.

Symptoms ofCreutzfeldt-Jacob

• The disease can have a dormant period of up to 4-5 years.

• It starts out with symptoms such as insomnia, mild depression, confusions, and behavioral changes.

• As the disease progresses, dementia and myclonus (involuntary jerking movements) occur.

More Symptoms and Signs of Mad Cow• The progression of the disease, around its finality, takes

about a year to induce fatal results. • The final stage of CJ causes the victim to lose all mental

and physical function--o This leads to a coma, and the victim will eventually die. 

• *There is no way to diagnose Mad Cow, however, as it can be commonly misdiagnosed as other types of brain disorders.

More About Symptoms

• Mad Cow induces a incubation period (4-5 years), but once it is activated, the symptoms lead to rapid fatal onset, which means that the symptoms are acute. 

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The PATHOGEN

• The pathogen is neither a virus nor a bacteria. • In CJ, the patient contracts the disease by eating food

previously contaminated with bovine spongiform encephalopathy (BSE). 

• The cause of this disease is a protein agent called a prion.  

The Prion

    The prion takes a normal functioning protein and transforms it into infectious, deadly proteins.     These proteins can cause Mad Cow by causing the brain to deteriorate.   

Prions, Continued

    Prions are very special in that they are not able to be broken down in the body.     They fold in such a way that is unknown tothe body's immune system.

    These tangled foreign proteins are what cause spongiform encephalopathy.

Specific Pathogenesis

1. Prions are not made up of DNA or RNA2. They have over 250 amino acids3. The body cannot identify them because of their

abnormal structure4. The prion, which most closely resembles a neurological

protein, induces other normal proteins to become a prion.

5. Here, abnormal proteins "direct the folding of normal proteins just by direct contact" (MAD COW DISEASE, The BSE...)

Diagnostics

• There is no way to certainly confirm a person with Creutzfeldt-Jacob Disease. 

• Doctors can confirm that a person had CJ Disease by performing an after-death brain biopsy (tissue examination).  

• However, doctors can make an accurate diagnosis by evaluating a patients' history, neurological exams, and diagnostic tests.

Transmission

• An "easy" way of transmitting Mad Cow disease is through blood transfusion.o Because MC/BSE has a very long incubation time (from

4-5 years and up), it is extremely difficult to tell if people have the disease or not. 

o So, people with the disease who may not show any signs or symptoms may be passing on the disease through transfusing blood. 

  Transmission, Continued• Transmission of CJ can also occur when a person

comes into contact with infected brain tissue.o  Cases of the disease have been caused by people

eating contaminated beef products.

Prevention

• To prevent mad cow disease, the Food and Drug administration banned brains and spinal cord from older cows in all animal feed.

•  However, other animals are still allowed to eat materials, like brain and spinal cords, that could put them in contact with the disease (such as chickens, pigs, etc.)

• If a cow is given feed containing chicken waste or plate waste and eat the tissue of another organism that was infected with mad cow, it could become infected as well.

Epidemiology• Mad cow most likely originated in England, where cows

were fed sheep carcasses as part of their diet.•  In order to save money in the 1970s, farmers stopped

cooking the sheep meat and fed the cows raw carcasses and brain matter.

• The cows ate this, and the mutation occured.• Most cases of Mad Cow occur in England, but it has

spread to other countries, including the United States.• In November 2006 there were 200 cases of Mad Cow

diagnosed around the world (164 in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the US, 2 in the Netherlands, and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia, and Spain)

Works CitedKugler, Mary. "Mad Cow Disease and Humans." Rare Diseases. 2009. About.com. 18 Dec. 2009 <http://rarediseases.about.com/od/rarediseases1/a/vcjd.htm>.  McNeil Jr., Donald. "To Prevent Mad Cow Disease, F.D.A Proposes New Restrictions." New York Times. 2009. New York Times. 9 Jan. 2010 <http://www.nytimes.com/2005/10/05/health/05cow.html>. "Mad Cow Facts." Center for Global Food Issues. 2009. Center for Global Food Issues. 18 Dec. 2009 <http://www.mad-cow-facts.com/about-mad-cow/>. Medicues. "Mad Cow Disease: Where Did it Come From and Am I at Risk?." Medicues. 2008. Medicues. 1 Dec. 2009 <http://www.medicues.com/news/mad-cow-disease-where-did-it-come-from-and-am-i-at-risk>.  http://www.youtube.com/watch?v=VOu7dxIKj7Ihttp://www.bachbloesemadvies.be/cms/cms/grafisch/tekst/95_199_Insomnia.jpghttp://inplacenews.files.wordpress.com/2008/06/20080409125636_mad_cow.jpg http://www.phas.ubc.ca/ais/images/abstractImages/Cashman.jpeghttp://scrapetv.com/News/News%20Pages/Health/Images/alzheimers-brain.jpghttp://www.heartsandminds.org/fotos/MSCAmanEatingAHamburger.jpg