Creutzfeldt-Jakob Syndrome is an incurable, fatal brain disease that is often mistaken for a human version of Mad Cow Disease (BSE) due to similar symptoms. Like Mad Cow Disease the symptoms start with rapidly progessive dementia which causes memory loss, hallucinations and personality changes. Creutzfeldt-Jakob Syndrom can be inherited, non-inherited or can be aquired by infection.Most victims die about 6 months after the intial symptoms appear although incubation period can be over 2 years long. Most victims die due to pneumonia because of lack of coughing reflexes. Creutzfeldt-Jakob Syndrome is a diseased caused by Prions.

Prions:

Prions are abnormal (rogue) proteins that are similar to viruses except for the fact that they do not contain Neucleic Acid or DNA or RNA. Prions target the proteins in the cell and cause them to take on the same shape as the prion making them now abnormal as well. Because the proteins cannot do their original job, the cell dies and the prions go off in search of other regular proteins. The word prion is made up of the words 'protein' and' infection'.

230px-Histology_bse.jpg

http://en.wikipedia.org/wiki/Prion

'Holes' are seen here in the brain tissue. This is caused by prions and causes the tissue to appear spongy in texture.230px-VCJD_Tonsil.jpg

Diagnosis:

CJD is normally suspected when a victim has symptoms such as rapidly progressive dementia and is confirmed either by an MRI or by a Cerebral-Spinal Fluid analysis for a particular protein called: 14-3-3. It can also be diagnosed when a Electroencephalography has characteristic triphastic spikes. Research in 2010 and 2011 enabled a possibility of identifying CJD in a blood test but unfortunatley it was unable to detect prions in patients in the early stages of CJD.

Treatment:

Unfortunatley since 2011 there is no generally accepted treatment for Creutzfeldt-Jakob Disease as it is fatal yet research continues. In 2003 an experimental treatment was given to an Irish teenager, Jonathan Simms. Unfortunatley the medicine given was unsuccessful and both brain function and tissue continued to deteriorrate.


Occurance:

CJD occurs in about one out of every million people per year yet it is still the most common of the human Prion diseases. The most common age group is 45-75 and within that group most cases appear in people ages 60-65. Of course the Variant version of CJD occurs in much younger people.

History:

The disease was first recognised by German neurologist Hans Gerhard Creutzfeldt and later by Alfons Maria Jakob which then in turn gave it the name Creutzfeldt-Jakob Syndrome.


Interesting Facts:

There is a similar Prion based disease called Kuru that was discovered amongst the natives of Paupa New Guinea that was found to have been passed on through cannibalism. It was found to be more prevelent in women and children as the men were given the best cuts of the meat whilst the women and children were given the organs and the brain. The name Kuru comes from the native word kuria/guria which means to shake due to the body tremors which are a classic symptom of the disease. It is also known as the laughing sickness because of the random bursts of laughter people would display.


Sites accessed:
http://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease#History
http://en.wikipedia.org/wiki/Kuru_(disease)
http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(10)62308-2/fulltext
http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(06)68930-7/fulltext
http://en.wikipedia.org/wiki/Prion
http://en.wikipedia.org/wiki/Electroencephalography