Creutzfeldt-Jacob Disease


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Creutzfeldt-Jacob Disease

  1. 1. Creutzfeldt-Jacob Disease (aka Mad Cow) Konstantine Adamopoulos and Emma Gillam
  2. 2. What is Mad Cow Disease? <ul><ul><li>Mad cow is a “fatal neurodegenerative disease in cattle.” </li></ul></ul><ul><ul><li>It can be transmitted to humans through ingesting of infected beef. </li></ul></ul>
  3. 3. Symptoms of Creutzfeldt-Jacob <ul><ul><li>The disease can have a dormant period of up to 4-5 years. </li></ul></ul><ul><ul><li>It starts out with symptoms such as insomnia, mild depression, confusions, and behavioral changes. </li></ul></ul><ul><ul><li>As the disease progresses, dementia and myclonus (involuntary jerking movements) occur. </li></ul></ul>
  4. 4. More Symptoms and Signs of Mad Cow <ul><ul><li>The progression of the disease, around its finality, takes about a year to induce fatal results.  </li></ul></ul><ul><ul><li>The final stage of CJ causes the victim to lose all mental and physical function-- </li></ul></ul><ul><ul><ul><li>This leads to a coma, and the victim will eventually die.  </li></ul></ul></ul><ul><ul><li>*There is no way to diagnose Mad Cow, however, as it can be commonly misdiagnosed as other types of brain disorders. </li></ul></ul>
  5. 5. More About Symptoms <ul><ul><li>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.  </li></ul></ul><ul><li>  </li></ul><ul><ul><li>(Fill in handout) </li></ul></ul><ul><li>  </li></ul>
  6. 6. The PATHOGEN <ul><ul><li>The pathogen is neither a virus nor a bacteria.  </li></ul></ul><ul><ul><li>In CJ, the patient contracts the disease by eating food previously contaminated with bovine spongiform encephalopathy (BSE).  </li></ul></ul><ul><ul><li>The cause of this disease is a protein agent called a prion . </li></ul></ul><ul><li>  </li></ul><ul><li>  </li></ul>
  7. 7. The Prion <ul><li>     The prion takes a normal functioning protein and transforms it into infectious, deadly proteins.  </li></ul><ul><li>    These proteins can cause Mad Cow by causing the brain to deteriorate.  </li></ul><ul><li>   </li></ul>
  8. 8. Prions, Continued <ul><li>     Prions are very  </li></ul><ul><li>special in that they  </li></ul><ul><li>are not able to be  </li></ul><ul><li>broken down in the  </li></ul><ul><li>body.  </li></ul><ul><li>    They fold in such a  </li></ul><ul><li>way that is unknown to </li></ul><ul><li>the body's immune  </li></ul><ul><li>system. </li></ul><ul><li>     These tangled foreign proteins are what cause spongiform encephalopathy. </li></ul>
  9. 9. Specific Pathogenesis <ul><ul><li>Prions are not made up of DNA or RNA </li></ul></ul><ul><ul><li>They have over 250 amino acids </li></ul></ul><ul><ul><li>The body cannot identify them because of their abnormal structure </li></ul></ul><ul><ul><li>The prion, which most closely resembles a neurological protein, induces other normal proteins to become a prion. </li></ul></ul><ul><ul><li>Here, abnormal proteins &quot;direct the folding of normal proteins just by direct contact&quot; (MAD COW DISEASE, The BSE...) </li></ul></ul>
  10. 10. Diagnostics <ul><ul><li>There is no way to certainly confirm a person with Creutzfeldt-Jacob Disease.  </li></ul></ul><ul><ul><li>Doctors can confirm that a person had CJ Disease by performing an after-death brain biopsy (tissue examination).   </li></ul></ul><ul><ul><li>However, doctors can make an accurate diagnosis by evaluating a patients' history, neurological exams, and diagnostic tests. </li></ul></ul>
  11. 11. Transmission <ul><ul><li>An &quot;easy&quot; way of transmitting Mad Cow disease is through blood transfusion. </li></ul></ul><ul><ul><ul><li>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.  </li></ul></ul></ul><ul><ul><ul><li>So, people with the disease who may not show any signs or symptoms may be passing on the disease through transfusing blood.  </li></ul></ul></ul>
  12. 12.   Transmission, Continued <ul><ul><li>Transmission of CJ can also occur when a person comes into contact with infected brain tissue. </li></ul></ul><ul><ul><ul><li>  Cases of the disease have been caused by people eating contaminated beef products. </li></ul></ul></ul>
  13. 13. Prevention <ul><ul><li>To prevent mad cow disease, the Food and Drug administration banned brains and spinal cord from older cows in all animal feed. </li></ul></ul><ul><ul><li>  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.) </li></ul></ul><ul><ul><li>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. </li></ul></ul>
  14. 14. Epidemiology <ul><ul><li>Mad cow most likely originated in England, where cows were fed sheep carcasses as part of their diet. </li></ul></ul><ul><ul><li>  In order to save money in the 1970s, farmers stopped cooking the sheep meat and fed the cows raw carcasses and brain matter. </li></ul></ul><ul><ul><li>The cows ate this, and the mutation occured. </li></ul></ul><ul><ul><li>Most cases of Mad Cow occur in England, but it has spread to other countries, including the United States. </li></ul></ul><ul><ul><li>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) </li></ul></ul>
  15. 15. Works Cited <ul><li>Kugler, Mary. &quot;Mad Cow Disease and Humans.&quot; Rare Diseases . 2009. 18 Dec. 2009 < >. </li></ul><ul><li>  </li></ul><ul><li>McNeil Jr., Donald. &quot;To Prevent Mad Cow Disease, F.D.A Proposes New Restrictions.&quot; New York Times . 2009. New York Times. 9 Jan. 2010 < >. </li></ul><ul><li>  </li></ul><ul><li>&quot;Mad Cow Facts.&quot; Center for Global Food Issues. 2009. Center for Global Food Issues. 18 Dec. 2009 < >. </li></ul><ul><li>  </li></ul><ul><li>Medicues. &quot;Mad Cow Disease: Where Did it Come From and Am I at Risk?.&quot; Medicues . 2008. Medicues. 1 Dec. 2009 < >. </li></ul><ul><li>    </li></ul><ul><li> </li></ul><ul><li> </li></ul><ul><li> </li></ul><ul><li> </li></ul><ul><li> </li></ul><ul><li> </li></ul>