Creutzfeldt-Jakob Disease Research Paper

Creutzfeldt-Jakob disease Or CJD is a degenerative brain disorder that causes dementia and eventually death. This disease affects the person's brain by causing it to be unstable. Some early symptoms of CJD are; failing memory, behavioral changes, impaired coordination and visual disturbances. Because this disease affects the neurological balance of the brain, a person who is suffering from it eventually loses control of their brain and will fall into a coma. Some other symptoms are insomnia, depression, unusual sensations and muscle jerks. Some cases have shown that people may go blind and lose the ability to speak or move. The overall outcome for all cases have resulted in death. Typically the symptoms appear around the age of sixty and around ninety percent of individuals die within one year. Some cases have had people live for longer than one year after the symptoms progressed but eventually the individuals passed away.
There are three different types of CJD. The first is sporadic, which accounts for around eighty five percent of cases. Sporadic is when the disease appears randomly, usually the cause is unknown or there is none.This can affect anyone, even if they do not eat meat. The second type is hereditary. This type is genetic which accounts for five to ten percent of cases in the United States. The
individual may get the disease although their parents never showed symptoms of it in their lives or inherit it from a gene mutation. The third type is acquired, this is usually accidentally brought on. A person gets this from coming into contact with infected brain tissue or fluids. Acquired makes up around one percent of cases. The most common way for it to be acquired is when infected surgical instruments or transplant tissues come in contact with a person. CJD is not passed on like a cold or the flu, sneezing or coughing will not transfer or spread the disease to another individual. It is controversial whether you can get CJD through blood. It is most common among people of the age of sixty or older but it can affect anyone of any age.
There are also two different names to CJD, when brought on from infected meat it is called variant CJD. According to VeryWell, in Europe CJD was linked to people eating contaminated beef. This is quite similar to Kuru. Kuru is brought on by cannibalism. Usually the person has consumed infected brain tissue. Kuru shows many of the same symptoms, although a difference is that Kuru has an incubation period. This means a person can be infected but not show signs or symptoms for many years. Normally the incubation period consists of ten to thirteen years but according to National Library of Medicine some cases have reported an incubation period of fifty or longer years. This happened mostly among the people of New Guinea. Although the practice ended around nineteen-sixty, cases still appeared after. The difference between Kuru and CJD is that CJD is caused by a prion.
The prion has usually mutated and causes normal proteins to fold abnormally.
At first the people assumed that all cases of CJD were a result of eating contaminated meat but due to further investigation that theory was disproven. In the nineteen eighties the first major outbreak of Bovine Spongiform Encephalopathy (mad cow disease or BSE) happened in the United Kingdom, it reached its peak around nineteen ninety three with almost one thousand cases per week of infected cattle. Because there is no way to detect BSE in the blood, according to emedicinehealth, people who lived in the area a of BSE outbreak for a long period of time are not allowed to donate
blood.
The gene affected is PRNP which is the prion protein gene. More than thirty mutations can be found affecting the PRNP gene. Some mutations can cause changes in the PrP, which is an amino acid. Some outcomes to changes that occur in the PrP are cognitive disorders and neurodegenerative diseases. Some diseases associated with genetic mutation are Wilson disease, Huntington disease and Creutzfeldt-Jakob disease. Normally the mutations affect the nervous system. PrP is active in the brain and other tissues, although the function of this protein is unknown, but is said to help with the junction between nerve cells.
There is no cure for Creutzfeldt-Jakob disease although some medication may help with the symptoms. Some drugs have been tested in hopes of stopping the disease or slowing it down. A few are listed here; acyclovir, amantadine,antidepressants, antibiotics, antiviral agents, interferon and steroids. Because no medication has shown significance in stopping or slowing down the disease, care for the person becomes important. Usually the individual will have to be cared for by medical professionals. This is because the individual may show aggressive behavior or need help controlling themselves as well as need assistance with walking and eating. When symptoms begin to appear, tests will normally be administered.
Early in the process mental analysis and nervous system tests will be done. The results show memory and thinking problems. Later on a motor skills test will be done. Results show abnormal reflexes, increase in muscle tone, spasms or twitches in muscles and muscle wasting (weakness or loss of muscle tissue). Coordination tests will be done as well as eye exams. The coordination test will show the patient to be losing control of their ability to walk or stand. The eye exams will result in blind spots the patient can or may not notice. Blood tests can be done to rule out any other form of dementia or abuse of narcotics, CT scans of the brain will show shrinkage and sometimes a spinal tap will be done to test the proteins. A spinal tap is when a doctor sticks a large needle into the spine of a patient and takes out spinal fluid. The spinal fluid is important in testing because it can show abnormal protein activity or infections within the brain.
Because CJD has no cure at the moment, current research that is being done involves advancing tests to help with the diagnosis of the disease in its earlier stages. Scientists have also concluded that low amounts of cerebrospinal fluid can be a symptom for patients with various prion diseases. A current project idea by the Creutzfeld-Jakob Disease Foundation is bringing awareness to the disease but because it affects such a small amount of people, funding is difficult. Right now the project for CJD is small. Medical professionals also want to attempt to try new medications.
Scientist have concluded that the only way to be one hundred percent sure if a patient has CJD is to do a brain biopsy after the patient has passed away. Because this disease is very rare and only affects around one in every one million people worldwide there is not much that can be done. Scientist are currently also investing time in research on the nature of prions, as well as creating a test to show when CJD will appear in someone’s lifetime, the use of antibodies to combat prion diseases and the possible development of a vaccine to treat CJD. The National Institute of Neurological Disorders and Stroke (NINDS) is hoping to seek out more information on the brain and nervous system. They plan to use that knowledge to reduce the burden of neurological diseases. CJD is currently untreatable. When symptoms appear it is best to see a medical professional as soon as possible. With all the current research being done, there is hope in finding a cure. Anyone can help out by donating to the Creutzfeld-Jakob Disease Foundation. Overall CJD is a rare but malicious disease. It is important to be caution when eating meat, and to look out for symptoms at their earlier stages. CJD consists of three types; sporadic, hereditary, and acquired. When dealing with this disease it is better to be helped by medical professionals. Ultimately this aggressive disease results in coma and death. Current research for CJD is still ongoing and hopefully one day there will be a cure.