Cruetzfeldt Jakob Disease
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Creutzfeldt Jakob Disease is commonly known as mad cow disease. Unfortunately the terminology “mad cow disease” is incorrect and should not be used to describe this condition. When a person has Creutzfeldt Jakob Disease, their brain tissue rapidly degenerates and ends in death. This condition is not curable, and will ultimately lead to death. Historically speaking, this disease got its name from the first founder, Hans Gerhard Creutzfeldt. He examined patients with symptoms related to brain degeneration, which lead to the naming of the disease. Afterward Alfons Maria Jakob found more information regarding the disease, and thus, the “Creutzfeldt Jakob Disease” name was born.
Occurrence of Creutzfeldt Jakob Disease
This disease is considered very rare. It only happens in one out of every 1 million people, sometimes less often. The age range that is generally affected is between the ages 45-75, though those who are 60-65 get the disease more often. There are variants of the disease that occur in young people.
The variant of CJD, also known as vCJD is caused by eating beef that has been infected with mad cow disease. Both are very similar and beef contamination is more common than previously perceived. This type of disease occurs more often than regular CJD.
Transmission of the Disease
The disease is transmitted through a special protein called a prion. It is a defective protein that once ingested or taken into the body, the protein starts to disrupt the normal protein within the body. The defective protein can be transmitted by many means including human growth hormone products, Immunoglobulins, corneal grafts, dural grafts or electrode implants. The disease can also be inherited through genetics as a mutation where ten to fifteen percent of the CJD cases come from. It may also appear for the first time in the patient in sporadic form. These are the causes in depth:
Human Growth Hormone – The disease has also been shown to result from usage of HGH drawn from the pituitary glands of cadavers. Those who died from Creutzfeldt Jakob Disease could transmit the disease through the HGH extracted from their pituitary gland. The practice of removing HGH from this source has been halted in many countries while others continue it.
Immunoglobulins – the immunoglobulins are mostly known as antibodies. They are used to fight disease by identifying diseased cells. Some antibodies are affected by CJD and actually carry the harmful effects of the disease if transmitted through blood or other means.
Corneal Graft – The cornea of the eye is one of the places where sight can be compromised easily. Corneal grafts are often needed to support the reinstitution of vision in patients. Since corneal grafts usually come from cadavers, those who have died from CJD may pass the disease to a new person through the graft.
Dural Graft – patches of the dura mater make it possible for the body to communicate with the brain and spine. Many people receive grafts of the dural tissue to recover some of these functions from cadavers and this may result in CJD if the person the graft comes from was infected.
Electrode Implants – Electrode implants into the brain which have been used on other people with CJD may transmit the disease even when the equipment seems to have been sterilized. This is more likely to occur in countries where the equipment is sterilized by those who do not know how to properly sterilize the equipment properly.
Collagen Injections – Since one of the primary sources for collagen comes from cow lipids, the transmission of CJD can easily be routed through them. This is not difficult to do when the collagen injections come from an unknown source or when the practice is not done by true medical officials.
Consuming Meats – It is not known if CJD is truly derived from the consuming of beef products but it is known that consuming the prions from the meat can cause a development of classic CJD. The most common source of these prions occurred pre-1989 because the way meat was prepared has changed for ground beef and other meat mix products.
Cannibalism – Some tribes in Papua New Guinea practice cannibalism. It is known that this can cause the transmission of CJD but it only occurred in certain members of the tribe. This is because the men would usually only eat flesh meats of the deceased while women and children received organs and other unwanted materials from the remains. This made the women and children most likely to contract the disease through cannibalism.
Prions, the infectious agent of CJD, may not be inactivated by means of routine surgical instrument sterilization procedures. This is why those who have the disease and do not know who get brain probes and other procedures done to them will leave their prions on the equipment. Doctors and other medicine practitioners would then use the equipment on others, perceiving that the equipment was clean and transmit the disease to others. This is why the US Centers for Disease Control and Prevention recommends the instrumentation which are used on known patients of the disease be destroyed. Special chemical decontamination is necessary for machinery which cannot be replaced as easily but it is not guaranteed that it will prevent transmission of the disease.
Symptoms of Creutzfeldt Jakob Disease
When this disease first begins, dementia takes place. It is a rapidly increasing type of dementia that causes quite a bit of changes mentally. Memory loss, hallucinations, and personality changes all occur and cause problems. Speech impairments and twitchy begins shortly after the onset of dementia. After that happens an increased lack of coordination and seizures also begin to present themselves. Coma is likely in some individuals, and they eventually live out the rest of their lives in a comatose state.
For those that have vCJD a longer life expectancy after diagnosis is possible. Usually this variant lasts 12 months to 14 months.
Unfortunately, after these symptoms set in death is likely very near. After a few weeks the person is likely to die suddenly, or in some cases it may take a few months.
Diagnosing Creutzfeldt Jakob Disease
Diagnosing CJD is fairly easy since it is so rare and doctors know what to look for. Rapidly increasing dementia is usually the first thing that doctors look for in diagnosis. Electroencephalography can examine the spikes in the brain. Cerebrospinal fluid tests can check for a “14-3-3” protein to accurately diagnose. MRI’s are also commonly used to show the abnormalities in the brain imaging.
Facts about Creutzfeldt Jakob Disease
The median age that patients die from CJD is around 68 years old. The duration is usually 4 to 5 months of sickness before it leads to death. Dementia is noted immediately and is the outstanding symptom that can become more severe and lead to other problems.
Treatment for CJD
Unfortunately CJD is always fatal. Experimental treatments are sometimes given to help patients, but limited studies are available. One known study used the drug Pentosan Polysulphate, otherwise known as PPS. It didn’t stop the disease or even help slow dementia down. It did however show signs of lengthening survival. This is unfortunate because a lot of suffering is still involved and the patient goes through a lot of pain.
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