CJD DISEASE: Creutzfeldt-Jakob disease or CJD is when the central nervous system dysfunctions. Before CJ After CJ It is characterized by a rapidly progressive dementia which eventually leads to death. Image Source: bestpractice.bmj.com
Normal Prion Abnormal Prion CJD = PRION DISEASE: Creutzfeldt-Jakob Disease is the human form of a prion disease A prion disease is when a protein is folded wrong and cannot function properly Image Source: http://journeyoflight79.wordpress.com/tag/spongy-bone/
THREE TYPES OF CJD: Sporadic ~Makes up the majority of CJ cases ~Occurs for no known reason ~Average age of onset is 65 Image Source: http://www.realmagick.com/creutzfeldt-jakob-disease/
THREE TYPES OF CJD: Familial ~Inherited form ~Very rare ~Located on PRNP gene (or prion protein) ~Located on 20p13 Image Source: http://www.cjdfoundation.org/
THREE TYPES OF CJD: Image Source: http://www.cjdfoundation.org/ Tonsin Biopsy: Variant CJD Prion protein immunostaining Acquired (Variant) ~Contaminated surgical instruments ~Contaminated beef ~Contaminated blood or plasma transfusion
CJD, MAD COW, & CWD Mad Cow (Bovine spongiform encephalopathy) ~Prion disease that effects bovine or cattle ~Same infection that causes variant CJD (vcjd) in humans ~vcjd accounts for less than 1% of cases (only 200world wide) No cases of vcjd in the U.S. ~Not believed to be transferable from cows to humans Image Source: http://mrsmaine.wikispaces.com/ CWD (chronic wasting disease) ~Prion disease that effects deer and elk ~No evidence that this is transferable to humans
SYMPTOMS: Blurred vision (sometimes) Changes in gait (walking) Confusion, disorientation Dementia that occurs over a few weeks or months Hallucinations Lack of coordination Muscle stiffness Muscle twitching Mycological jerks or seizures Nervous, jumpy feelings Personality changes Sleepiness Speech impairment
SIGNS OF CJD: Abnormal reflexes or increased normal reflex responses Increase in muscle tone Muscle twitching and spasms Strong startle response Weakness and loss of muscle tissue (muscle wasting) Failing Memory Blindness Image Source: encyclopedia.org
TESTS FOR CJD: Blood tests to rule out other forms of dementia and to look for markers that sometimes occur with the disease CT scan of the brain Electroencephalogram (EEG) MRI of the brain Spinal tap to test for a protein called 14-3-3 The disease can only be confirmed with a brain biopsy or autopsy. Image Source: webanswers.com
PROGNOSIS OF CJD: No known cure Proteins which cause CJD can potentially be removed Person unable to care for themselves after 6 months Usually fatal after 8 months Cause of death Infection Heat failure Respiratory failure
CONCLUSION: CJD is a disease that causes the nervous system to deteriorate. There are three types of CDJ Acquired (Variant) Sporadic Familial There are a wide variety of symptoms that are onset at a fast rate. CDJ can only be confirmed during an autopsy There are currently no treatments or cures It is important to know that currently there is no link between the human form (CDJ) and an another animal forms of this prion disease. There is no proof that humans can get it from eating an animal with a prion disease(mad cow, CWD, etc..). However there are studies happening now to find the truth. In Wisconsin there are people being monitored because they consumed a deer with CWD before they received the test results confirming the animal had the disease.
LITERATURE CITED: Belay, Ermias D., Ryan A. Maddox, Elizabeth S. Williams, Michael W. Miller, Pierluigi Gambetti, and Lawrence B. Schonberger. "Chronic Wasting Disease and Potential Transmission to Humans." Medscape. 10.6 (2004): n. page. Web. 30 Mar. 2012. <http://www.medscape.com/viewarticle/479556>. Dugdale, David C., Luc Jasmin. David Zieve. Creutzfeldt-Jakob disease PubMed Health. Web. 26 Sep. 2011. 2 Apr. 2012. <http://www.ncbi.nlm.nih.gov/pubmedhealth/pmh0001792/ Horwich, Arthur L., and Jonathan S. Weissman. "Deadly Conformations-Protein Misfolding in Prion Disease." Cell. 89. (1997): 499-510. Web. 30 Mar. 2012. <http://francissaitta.com/499.htm>.