Creutzfeldt-Jakob disease is a rare but fatal brain disorder that is believed to affect 250 to 300 people in the United States every year. Since it is hard to diagnose, the actual number of cases may be higher.
Creutzfeldt-Jakob disease (CJD) is thought to be caused by abnormal prions, a type of protein that causes other proteins to malfunction, and kills brain cells (neurons).
This creates holes in brain tissue, so that the brain has the appearance of a sponge when viewed under a microscope.
Prion diseases can attack humans and animals. The origin of prions is unknown.
CJD can make its appearance in people who are anywhere from 20 years to 70 years old. Usually this happens around age 60.
Sporadic, or classical Creutzfeldt-Jakob disease is the most common form in 85% of all cases. It is not contagious. It usually hits suddenly in the late 50s or 60s.
Death comes in a matter of months. The cause is not known.
Genetic, or familial Creutzfeldt-Jakob disease is inherited. It will usually develop at a younger age, and develops more slowly than sporadic CJD. Genetic CJD is very rare.
Iatrogenic Creutzfeldt-Jakob disease is the result of medical procedures where there is contact with infected tissue. This can be from blood transfusions, surgical instruments, or via the use of human growth hormone.
Variant Creutzfeldt-Jakob disease happens after exposure to bovine spongiform encephalopathy (BSE). It hits at a young age, and will take a longer time, about 14 months, from onset to death, causing mental, neurological and physical dysfunction.
New variant Creutzfeldt-Jakob (nvCJD) is infectious, and is related to mad cow disease. It is thought that the infection in cows is the same one that can infect humans.
No cases of nvCJD has been found in the United States.
In the early stages, CJD causes loss of memory and apathy, and personality changes. Ultimately the individual will become delirious having hallucinations, or develop dementia.
After a few weeks, clumsiness and confusion begin to appear. Walking is more difficult, speech may become slurred, and vision may blur. As more time elapses, limbs become stiff and shaky.
In a short time the individual becomes unable to take care of themselves, unable to speak, or to move. Ultimately they will lose all awareness of the world around them.
CJD usually runs its course within six months. Rarely the disease may last for up to two years. There is no known cure for Creutzfeldt-Jakob disease.
Resources:
CJD INFORMATION
http://www.cjdfoundation.org
CJD Support Network: Creutzfeldt-Jakob disease
http://www.cjdsupport.net/index.php?sid=5
Creutzfeldt-Jakob disease - Overview
http://www.umm.edu/ency/article/000788.htm
Creutzfeldt-Jakob Disease Fact Sheet
http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm
Creutzfeldt-Jakob disease
http://www.nlm.nih.gov/medlineplus/ency/article/000788.htm
Creutzfeldt-Jakob disease
http://www.mayoclinic.com/health/creutzfeldt-jakob-disease/DS00531
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