Creutzfeldt-Jakob Disease

The word encephalitis is derived from two Greek words. Enkephalos, which means brain, and itis, which means inflammation. Encephalitis was defined by Sir John Burton Cleland, a pathologist and naturalist. When he defined it, it was named ‘Australian X disease’.

Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition falling under the category of Transmissible Spongiform Encephalopathy (TSE) because of having characteristic of spongy degeneration of the brain that it causes and its ability to be transmitted. First it was described in the United Kingdom in March 1996 and it has been connected with exposure to a TSE of cattle known as Bovine Spongiform Encephalopathy (BSE) sometimes called Classical BSE, having been reported first in 1986 in the United Kingdom.

It is a rare, degenerative but fatal brain disorder affecting very a small fraction of persons. The symptoms usually arise at the age of 60 and the person dies within a year. Many researchers believe that this disorder is the result of an abnormal protein known as prion. About 5-10% cases reported in the United States share a genetic basis where this form of dementia is caused by a mutation in the gene for the prion protein. Patients with Creutzfeldt-Jakob disease suffer from the problems associated with muscle coordination, personality changes, impaired memory, judgment making, thinking disability and impaired vision. Other possible symptoms include insomnia and depression. In later stages the persons

This letter is my version of a fail-safe, in the event that I don’t get a chance to say goodbye, or hello again, because something went wrong. This is not my goodbye letter, and please don’t assume I’m dead because you’re reading it. I have every intention to return to school after break as my normal, oddball self, minus one medical problem. I just really hate loose ends, and maybe part of me, even though I tell myself I’m not, is afraid of this. I don’t want to leave the people I’ve grown so close to over these past few years, at least, not without some kind of goodbye, thank you, warning, or p.s. the treasure is hidden in blank. That would just be rude. So here goes nothing.

Author Beata Sikorska writes a chapter on this disease in the book Neurodegenerative Diseases, she discusses how this rare disease is caused by an abnormal piece of protein (PrP) called a prion (Sikorska, 2012). A prion is an infectious particle made of proteins and the specific protein PrP is the human gene encoding expressed mostly in the nervous system. Proteins are made up by a string of amino acids that fold and form a 3-D shape; the shape the amino acids take gives these proteins it’s function. When a protein does not fold properly it does not function or can become destructive. The human body has specific mechanisms to protect itself from many different types of infections; one of these mechanisms is the blood brain barrier. This barrier blocks harmful substances from entering the brain while allowing necessary nutrients in this includes allowing PrP proteins to enter. Marie-Clare Porter describes the process of CJD in the British Journal of Anesthesia as a prion which is also known as transmissible spongiform encephalopathies, are a group of neurodegenerative conditions that are transmissible, progressive and uniformly fatal (Porter, 2013). Once a prion enters the brain it can infect other normal proteins causing them to lose function and become destructive. As the affected brain cells continue to die they leave tiny sponge-like holes; these holes eventually lead to the signs and symptoms of CJD and are

Creutzfeldt-Jakob disease is a fatal neurodegenerative disease that causes rapid degeneration of the cerebral cortex (the outer layer of tissue surrounding the brain). It is closely related to bovine spongiform encephalopathy (BSE) or “mad cow disease” in its pathology. Creutzfeldt-Jakob disease is often called the human version of BSE and is therefore assumed that it has crossed the species barrier from cattle to man. In the early stages of Creutzfeldt-Jakob disease, it is found that patients have a failing memory, behaviour changes, impaired coordination and vision problems. As the disease progresses into the later stages, the mental deterioration becomes severe. The patients experience uncontrollable movements, blindness, and weakness and

This is all caused by a deadly little prion, which can be referred to as Spongiform Encephalies (Kuru: The Dynamics of a Prion Disease: Dr. Bindon), causes the brain to become spongy and have holes while dterteating the neurological functions along with the body (Kuru: The Dynamics of a Prion Disease: Dr. Bindon). Prions are able to come in many deadly forms for example some of the more well known are Creutzfelt- Jakob Disease, Fatal Familial Insomnia, and Kuru which all occur in humans (Kuru: The Dynamics of a Prion Disease: Dr. Bindon). Then there are the ones that occur in animals such as Scrapie, Bovine Spongiform Encephalopathy, Feline Spongiform Encephalopathy, Transmittable Spongiform Encephalopathy, Chronic Wasting Disease, and Transmittable Mink Encephalopathy (Kuru: The Dynamics of a Prion Disease: Dr. Bindon). All of these have direct relation between humans and animals on how the prions destroy the brain.

Generally, a sponge is equated with the soaking or consumption of liquids. Nevertheless, this is not representative of this disease or its effects on the human brain. This progressive and debilitating disease maintains many unknowns, and patient outlook is equivalent to lethal injection. Creutzfeldt-Jakob’s Disease (CJD) is a rare, degenerative, invariably fatal brain disorder, derived from transmissible spongiform encephalopathy caused by prions. Prions occur in a normal state, which are harmless proteins found in the body’s cells, and also in an infectious form that causes disease. Harmless forms of prion proteins have the same sequence of amino acids, but the infectious forms of protein have a different folded shape than normal proteins.

To conceive an understanding behind the neurological science of brain function, assessing the possibilities of how and why come into question. Research conducted during post mortem investigations suggests the presence of neurofibrillary triangles (insoluble protein) and senile plaque (hard, wax-like, deposit surrounding brain protein) in the cerebral cortex resulting in degeneration of the tissue. Noted by Munoz and Feldman (2000) of the Canadian Medical Association, the presence of neurofibrillary triangles “signifies the failure of the neuron to properly maintain its cytoskeleton, which is required to support the extraordinarily complex branching shape of its numerous processes” (p.66) if overproduction and imbalanced dispersal of triangles occurs. Senile plaques, however, prove to be significantly harder to detect as the process for plaque build-up starts to take affect well into middle age adults. Components of senile plaques are

I even wished that something bad would happen to you. When I found out about your accident, my world turned upside down. I did not expect this to happen to anyone in my life. At first, somehow, I thought that this was my fault. When I visited you at the hospital, guilt struck me because of how bad the condition you were in. This guilt put such a huge weight on my shoulders. Because of this, I feel like I am overprotective of my other friends. There are days that I wish that I can put this down. However, if I put it down, I would be lying to myself. How can I face your family and not think of all of the terrible things that I have said about you? There are days where I wonder what if you did not suffer from a massive heart attack. What would it be like when you were well? Would we still be friends? It is sad to say that I wish I was not carrying this guilt because if I was not carrying this guilt, you would be well today. I would not be worrying about his. If the event did not happen, we probably would not be friends like we are today. I would rather have you being well and no friends with me than you being sick and still friends with me. If you understand what I am writing to you, I

Creutzfedlt-Jakob Disease(CJD) is a rare, non-treatable, and fatal brain disorder caused by a prion, a pathogen that is smaller than a virus. The causative agent of Creutzfedlt-Jakob Disease is very difficult, almost impossible to destory. The casusative agent is a clear watery fluid that fills the space between the arachnoid membrane and the pia mater. Per year, Creutzfedlt-Jakob Disease effects one in every one million people worldwide, in the United States there are around 300 cases. There are 3 catagories of Creutzfedlt-Jakob Disease; The most common being sporadic CJD(the disease appears even though the person has no common risk factors for CJD), Hereditary CJD, and the least common acquired CJD(caused by exposure to the brain or nervous

Morrie a great dancer, and loved his job as a sociology professor at Brandeis University, Was diagnosed with (amyotrophic lateral sclerosis (ALS), Lou Gehrig's disease,) (page 6 Morrie). This disease is a brutal, unforgiving illness of the neurological system that slowly takes over your body. However most people would just look at the negative side but not Morrie, he did not see the negative only the positive. He says (Now that I am dying, I’ve become much interested in people) (page 27 Morrie).

Neurodegeneration simply refers to the progressive death of neurons. As the condition progresses, the neurons will lose both structure and function. Diseases that are neurodegenerative in nature are incurable and results in the death of neurons; the transmitting and receiving of nerve impulses is accomplished by neurons. The loss of neurons occurs in a plethora of diseases such as: amyotrophic lateral sclerosis (ALS), Parkinson's, Alzheimer's, and Huntington's. ALS specifically refers to the death of motor neurons (i.e. neurons responsible for controlling muscle movement) and ability to control your muscles gradually worsens over time (National Institute of Neurological Disorders and

It has been considered to be one of the most mysterious diseases scientists are fighting so far. With no effective treatment or research on the disease, it has a 100% fatality rate, however fortunately for us, the disease is extremely rare with only 2 cases and deaths since 2008. The disease is caused by an abnormal and infectious protein in the brain called a prion. Normally, prions are used to help transmit messages between certain brain cells. They use “protein folding” which is beginning as a string of amino acids and folding themselves into a 3-D shape. However, CJD causes mistakes in the process, which causes misfolded prion proteins to spread, whereas a healthy person’s body easily recycles them. As more and more misfolded prion

I hope your well, as you may of guessed I myself am not doing very well, as a lot has happened to me in these long ten years. By the time you receive this letter, I would of been dead for some time now, I hope my attorney delivered this letter safely to you, and that you are doing well. I often wondered where that rebellious teen who tried to steal from me that night had ended up? I sure hope no grave robbers come to try and steal my pocket book, but lord knows I would kick them right in the “blue jean sitter” if a fool was ever to try. I’m sure you remember that encounter that we had in the middle of the night on the street corner ten years ago, If not, I did not do my job. Remember when I pulled you up and dragged you to my house around