Creutzfeldt Jakob Disease: Causes, Symptoms and Treatment

One of the diseases that has greatly impacted the lives of folks nowadays is Creutzfeldt-Jakob disease (CJD) which is a condition that is fatal and unusual which has impact on the mind of individual. CJD causes harm to the mind that gets worsened with the duration of time. The patients identified as having the disease perish within twelve months as people become immobile (Solassol et al. , 2006). CJD is usually triggered by an unusual health proteins known as prion. Before prion is realized, proteins' function for body must be clarified. For each and every human being, proteins are important for life and can be found in body of most living things. All of human's body has good composition of proteins such as muscles, scalp, fingernails, bones, skin, blood, body organs and eyes. After water, protein is the next most significant constituent of body (Belay et al. , 2003).

According to LiveScience Personnel (2012), protein is mainly used for building, maintenance and fixing of body cells; it is present in various varieties which perform many jobs in body and the structure of each necessary protein determine its function. The inspiration of proteins are long strings of amino acids which get folded and curled into intricate three dimensional styles which allow protein to perform their job. Dr. Stanley B. Prusiner from College or university of California i. e. San Francisco purified an agent which was infectious made of unique kind of necessary protein and referred to it as "prion" in 1982; Nobel Award in Physiology or Medication was honored to him in 1997 for breakthrough of Creutzfeldt-Jakob Disease (CJD) that was mainly triggered by prions.

Prions get gathered in brain at higher levels that cause permanent damage to the nerve skin cells which cause various neurological symptoms. These infectious debris will vary in action from conventional bacteria and viruses which cannot be destroyed by exposing to heat and radiation. Even antiviral and antibacterial medications have no effects on prions; hence, presently, there is insufficient valuable treatment for CJD (Belay et al. , 2003).

Understanding Creutzfeldt-Jakob Disease (CJD)

Basically, CJD is an extremely unusual form of dangerous form of dementia which can propagate fastly in the body from brain. Prions are mainly found in brain and are harmless; when they are not given proper forms, they could have effects that can be devastating which can even attack brain, wipe out its cells and create slots or spaces in structure of brain (Ironside, 2009). Prion diseases have been found in both family pets and humans; such diseases were in news of mid 1980s along with Bovine Spongiform Encephalopathy (BSE) epidemic that was known as Mad Cow disease, this can be a cattle disease. In humans, it is known as CJD. The disease is found in about one to two people among million people worldwide.

Types of CJD

There are in essence two types of CJD i. e. Vintage CJD and Variant CJD (vCJD). The three types of common CJD are Sporadic CJD, Familial or hereditary CJD and Iatrogenic or Received CJD.

Sporadic CJD

More than 90% of men and women identified as having CJD suffer from this type of CJD which is mainly found in people aged in between 45 to 75. There are no specific symptoms because of this CJD (Shah et al. , 2009); however, some early symptoms such as major depression may be there but it can quickly progress into distress and problems of memory space which is often seen in dementia, accompanied by lack of balance and coordination along with intensifying blindness (Piouti et al. , 2012).

Familial or genetic CJD

Familial or genetic CJD is one of the exceptional genetic condition where the person's genes are inherited in one parent who triggers the protein to get mutated into a prion in later levels of life that result in the symptoms of CJD. In other words, it seems in families which have abnormal gene. About 7% of circumstances have this type of CJD. To be able to diagnose it, blood test is performed; person having this unusual gene has 50% potential for transmitting it to children. The other rare form of this CJD are Fatal Familial Insomnia (FFI) and Gerstmann-Strussler-Scheinker (GSS); both these are rarely within people (Brand et al. , 2006). It occurs in people who have age group of 50 years.

Iatrogenic or Acquired CJD

This type of CJD is seldom found in people as it is found in those individuals who get CJD while unintentional transmission that occurs during a surgical procedure like corneal grafts, devices in neurosurgery, individual pituitary hormone remedy or human being dura-mater grafts (Mayo Medical clinic Personnel, 2010).

Variant CJD

People having average age group of 28 years are located to be suffering from Variant CJD. It really is usually developed from eating of beef that was attacked with BSE. It can even happen with blood transfusion in which variant CJD is passed from one person to other. Once the symptoms are recognized, the condition spreads quickly in the torso (Tattum et al. , 2010).

Signs and Symptoms of CJD

In many of the cases, common CJD looks a lot like many other dementias; the disease comes on fastly and then declines the ability of considering as symptoms start showing up. Some of the most commonly found symptoms are that the individual may have swings in mood, problems related to storage area, interest absence and not behaving like oneself, having difficulty while walking such as keeping balance, dementia quick progressions such as lack of recollection and other thinking features, problems in eye-sight such as blindness, stiffening of limbs, twitching or jerks in muscles, feeling of clumsiness, speaking problem such as slurred conversation, difficulty in swallowing and akinetic mutism in which person can move eyes and appearance to be alert but can neither move voluntarily or speak (Espinosa, Bensalem-Owen, & Cost, 2010; Sikorska et al. , 2004).

However, folks who are in CJD's later periods face problems such as dropping awareness as it can be seen in various neurological examinations. As the disease in the first phases, CJD patients are terrified and it can be a distressing situation for the coffee lover; it is almost always associated with hallucinations that are aesthetic which means viewing things that aren't present there which might develop uncomfortable sense in them (Andrews, 2012).

People who suffer from sporadic CJD usually live than twelve months after the appearance of signs and symptoms; the utmost life is 2 yrs. Before death, a lot of people fall under condition of coma; the key reasons of fatality are heart failure, pneumonia or respiratory system failing such as breathing problem (Rossetti & Dunand, 2007). Among the most typical symptoms within Variant CJD people are changes in patterns, depression, drawback from social gatherings, difficulty in walking, dementia that is intensifying, struggling to do motion or speak and pain and odd kind of discomfort in limbs or face (LiveScience Staff, 2012).

Causes of CJD

According to studies of Ironside (2009) and Tattum et al. (2010), CJD is brought on by slow trojan or other small organisms. The agent that causes this disease has several characteristics which are different from trojans and bacteria's. The organisms are difficult to wipe out as they don't have genetic information on them in the form Nucleic acids such as DNA or RNA and has long period of incubation before symptoms are made visible. However, it's been found that it's mostly induced by prion protein that can occur in both normal and infectious form. The standard varieties have same patterns as that of amino acids but infectious ones change in their figures from normal proteins. Once they seem, abnormal ones start to get aggregated which start influencing the brain. Only 5-10% instances are inherited ones that arise either from mutation or changes in gene that happen to be responsible for handling the forming of normal prion proteins.

Diagnosis of CJD

CJD is difficult to diagnose specially when it is within its initial levels; there is absolutely no specific test available for diagnosing in living person. Only way to make certain that person got CJD is by analyzing the muscle of brain after his death. However, there are some other tests and methods can be used for analysis of the condition such as medical history examination as it will help doctors in learning the individuals symptoms and signs when they begin as CJD gets multiply quickly; Magnetic Resonance Imaging (MRI) where brain's picture is taken up to identify the difference between numerous kinds of CJD; Computerized Tomography (CT) check in which brain's picture will be studied to diagnose the condition; Puncture of Lumbar this means taking fluid form the vertebrae of a person by using syringe and a needle, it is performed to identify the microbe infections of brain; blood tests to recognize when there is any chance of genetic CJD; Electroencephalogram (ECG) in which electronic activity of brain is measured and brain autopsy where structure of brain is evaluated after person's death (Puoti et al. , 2012; Shah et al. , 2009).

Treatment for CJD

Up till now, there's been no success in growing right treatment for CJD. The researchers did numerous tests numerous drugs such as steroids, antiviral real estate agents, antibiotics, amantadine, acyclovir and so many more but they have never had the opportunity to find perfect treatment for this disease (Puoti et al. , 2012). Some studies remain in progression to build up appropriate treatment for patients but do not require have been successful in benefiting the humans.

The treatment for CJD that is being followed by various doctors is targeted at alleviation of symptoms and trying to make the individuals as comfortable as the experts can do in order that they have the perseverence of struggling against the condition. Some of the opiate drugs can relieve pain when people suffer from it however the drugs such as Sodium Valproate and Clonzaepam definitely assist in relieving of myoclonus. When the disease is in later levels, position of person is altered frequently so that he gets comfortable and bedsores can be avoided. For draining urine, a catheter can be utilized as it can help in handling the function of bladder and manufactured feeding can also be used.

Precautions for CJD

Although there are chances that CJD gets sent when you are careless during medical procedures but still people need to care for various things that will help them in continue to be protected from this disease. CJD is not a contagious disease that can be sent either by interpersonal or intimate contact or via air or feeding, touching or even taking care of person experiencing CJD at home. a few of the basic protective measures that require to be followed are cleansing hands either before eating or drinking alcohol, covering wounds or slices with bandages that are waterproof, guarding face and hands from being exposes to blood or essential fluids of body of person who is affected by this disease and taking special care and attention in blood vessels transfusions so that CJD infected person will not give his blood.

In order to avoid variant CJD, beef from selected countries should be consumed; countries which may have risky of TSE are more vulnerable to associated risk. The only options available for avoiding this type of CJD are either to eat beef from countries that highlight on strict laws on its quality and prevent eating parts of cattle which have high hazards such as spinal-cord, intestines, brain and eye.

Conclusion

Creutzfeldt - Jakob disease (CJD) is one of the fastest growing disease that has been experienced by many people worldwide. It has become important for research workers to recognize right treatment for this disease so that patients can get full restoration. In order to ensure that people who've genetic CJD receive appropriate treatment before symptoms learn to become visible, it is important that folks get their bloodstream tests done at regular intervals. When any of the signs or symptoms is observed in people, it is advised that they contact a doctor immediately who is able to ensure that right testing are done for identification of this disease.

Still, the researchers want to do extensive improve identifying the primary causes of CJD along with creating a remedial solution. Among all the available choices, the best some may be to get hold of any well-known doctors who are specialist in neurology who'll take the patient in right procedure. Hence, everyone needs to make sure they have complete information relating to this disease so that they can face it with persistence and strong will power.

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