Aaron

Aaron Jones

=What is it?=

A rare disease I am studying affects the brain and is called Jakob Creutzfield disease. This disease incurable degenerate neurological disorder or brain disease that is in the end fatal. The disease was first founded or described by a German neurologist Hans Creutzfield in the 1920's. The two years later it described by Alfons Maria Jakob and then named. It is a type of transmissible spongiform encephalopathies which are progressive conditions that affect the nervous and brain system. The transmissible spongiform encephalopathies are caused by prions which are infectious agents that are comprised entirely of progated mis-folded protein. Or in other words it can be caused by proteins such as animal meats. The CJD prion is a dangerous prion because it promotes refolding of native proteins in to the diseased state, this is the first step unfolding of alpha helices. The second step of the disease is refolding to beta pleated sheets, this causes the number of misfolded protein to grow and they increase at an exponential rate. Which then means there are large amounts of insoluble prions in affected cells. The mass of misfolded protein lead to disruption in the cell function causing cells to die. Mutations in the gene for the prion protein can cause a misfolding of the dominantly alpha helical regions into beta pleated sheets. The change in conformation disables the ability of the protein to undergo digestion. Once a prion is transmitted defective proteins invade the brain and the brain produces them in a self-sustaining feedback-loop. This lop causes an exponential spread of a prion, leading to death within a few months. Some patient have been able to live as long as 2 years. There is no explanation for this disease other than they have found a virus like particle, but have not found the nucleic acids.

=What it does to a person= In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

=What are treatments?=

There are no proven treatments.

=What is long time prognosis?=

The long time prognosis is death often with in a year with patients above 30

=Does it affect any certain cultural back ground?=

Although CJD is the most common human prion disease, it is still rare, occurring in about one out of every one million people every year. It usually affects people aged 45-75, most commonly appearing in people between the ages of 60-65. The exception to this is the more recently-recognized 'variant' CJD (vCJD), which occurs in younger people. According to the CDC:
 * CJD occurs worldwide at a rate of about 1 case per million populations per year.
 * On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million persons in the United States.
 * In the United States, CJD deaths among persons younger than 30 years of age are extremely rare (fewer than five deaths per billion per year).
 * The disease is found most frequently in patients 55-65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age.
 * In more than 85% of cases, the duration of CJD is less than 1 year (median: four months) after onset of symptoms.

= = =Comparison= With: Without:

http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm http://www.cjdfoundation.org/ http://www.healthcentral.com/ency/408/000788.html
 * Sources:**