Pineda Torres Gerardo Mariano Advanced Grammar
Inlingua English Center
Have you ever heard about mad cow disease (MCD)? If you had, you may want to know that humans can
also get infected. The human version of the MCD is called Creutzfeldt-Jakob Disease (CJD). This rare
disease affects one person in every one million people per year and just in the USA 200 cases are reported
every year. Once infected and after a certain period of time, failing memory, blindness, coma and
involuntary movements appear in patients with CJD. The responsible of this brain disease is a prion. A
prion is a protein that is capable of fold another in order to generate an infectious protein. Usually the fold
or deformation of the protein changes radically their biological roll or even its function as a molecule.
We can distinguish 3 different types of CJD: sporadic (85 percent of people without even been directly
exposed acquired it), hereditary (5-10 percent if there was any case of CJD in the family) and acquired (1
percent of the population that is believed to get infected by direct contact with infected tissue). Nowadays
there is not a standard procedure to diagnosed CJD. Normal medical routine include electroencephalograms
or magnetic resonance imaging for scanning the brain tissue.
Prion is a term that stands for proteinaceous infectious agent. A protein can be understood as a long
aggregate of small repetitive molecules, aminoacids. Hemoglobin and DNA are some examples of proteins.
Since these prions can’t replicate or metabolize they are not considered living organisms. However, prions
are considered a high level treat because of its complex diagnosis and no efficient treatments available.
After a period of time the infected proteins will begin to polymerize into an aggregate consisting of tightly
packed sheets. This configuration is very stable, also resistant to chemical and physical degradation, and
once accumulate in the tissue or cells the damage is irreversible.
CJD symptoms usually begin at the age of 60, and 90 percent of the patients die after 1 year. Other
symptoms of the CJD are: confusion, dementia, hallucinations, muscle stiffness, personality changes and
sleepiness. As it was mention there is no cure or effective treatment for this condition. Some medication is
recommend to control de aggressive behaviors. At the present time, scientists are working to develop tests
for CJD while the only and certain procedure to find CJD consist in a complete brain biopsy. One of the
treatments develop at NINDS detects the neuronal degeneration using fluids coming from the brain,
obviously this diagnosis is safer than the brain biopsy. The false positive rate reported for this test is about
5 to 10 percent.
CJD is a very rare brain infection cause by a prion witch triggers a very fast degeneration of the brain tissue
leading to hallucinations, involuntary movements, dementia and finally death. Nowadays there are not
efficient treatments and standard procedures to detect it, but scientist are working on a safer detection of
the disease base on tests results obtained from the brain fluids of the patient. The prion is responsible for
the fold of other proteins leading to structural changes and finally adding layers (polymerizing) of these
proteins to build tightly packed sheets. Scientists are beginning to investigate prion related diseases like
CJD and other related. We have to continue the support for prion researches in order to find better and
more efficient treatments.