Creutzfeldt-Jakob Disease Imagine losing control of body movement, losing memory, and forgetting family members. Imagine not remembering anything because of a disease. Creutzfeldt-Jakob Disease (CJD) is a brain disease similar to alzheimer's, but CJD is highly infectious and causes a much fast deterioration of the nervous system (Sick!). CJD affects between two hundred and fifty and three hundred Americans each year and is most common in people between the age of fifty and seventy-five years (World of Health). Since CJD is not very common, not many people know what it is, what causes it, and what treatment there is. Creutzfeldt-Jakob Disease was first described by a Alfons Maria Jakob, a German psychiatrist, in 1921. Jakob gave credit to another German psychiatrist, Hans Creutzfeldt, and that is how the disease got its name (Blaser). Even though the disease was found in 1921, very little is known to this day. Prior to 1995, almost no one outside the medical field knew about CJD and most doctors had never seen a case. It was not until a new variation of CJD was found that more research had been done (Sick!). The …show more content…
Prusiner’s theory stated that spongiform encephalopathies might be caused by certain proteins (Sick!). Many researchers thought that Prusiner’s theory had no logic because no form of protein had ever been found to cause any infectious disease. More than fifteen years of research confirmed Prusiner’s theory that a protein was the cause. The research had discovered an unusual type of protein in the brains of animals affected by various kinds of spongiform encephalopathies. Scientists now believe, because of Prusiner, that Kuru, bovine spongiform encephalopathy, vCJD, and other related diseases, like CJD, are caused by the transmission of prions from an infected person to a healthy person
Tay-Sachs disease is a rare and fatal genetic disorder that destroys neurons in the brain and spinal cord. The disease appears in three forms, Juvenile Onset, Late Onset (known as LOTS), and the most common form, Infantile (also known as Classic). The differences between the three forms of the disease are related to the age at which the symptoms of the disease begin to form. Tay-Sachs results from a deficiency of the enzyme hexosaminidase A, which plays a vital role in removing a fatty substance, called GM2 gangliosides, from neurons.
Abstract: Bovine spongiform encephalopathy is caused by a prion, which is an infectious agent comprised solely of protein. The prion is a degenerate form of a normal cellular protein found in the brain and in nervous tissue. It targets the normal protein and causes the normal protein to change its shape. When enough of the prion is produced, the cell dies and symptoms of the disease are expressed.
Walton, Sir John. Brain's Diseases of the Nervous System. Oxford University Press. New York, 1955, pp. 365.
Mad cow disease is caused by prions, "weird mutant proteins that are found in brain and spinal tissue"1. Another name for mad cow disease is called bovine spongiform encephalopathy (BSE) and the definition is "a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion"2. It started from what is called a prion protein then it turned into a pathogenic, and then it starts to damaged the brain of a cattle. There's another name for this disease and it's called Creutzfeldt-Jakob Disease "a form of progressive dementia characterized by loss of nerve cells and degeneration of nerve cell membranes leading to the production of small holes in the brain. It is rare, degenerative, and invariably fatal"3. This disease happen in human causing lapses in the memory, mood swings similar to depression, lack of interest and social withdrawal3. It is said that this disease has no tr...
Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however
In sporadic CJD, the disease occurs even though the affected does not have any known risk factors that would cause an occurrence of the disease. This sudden occurring CJD is indisputably the most frequently diagnosed type of Creutzfeldt - Jakob disease. This statistically accounts for at least 85 percent of CJD cases. Due to that there are some fifty to sixty deaths per year due to sporadic CJD in the United States alone. Similar figures are seen in other countries such as Australia, Canada and the United Kingdom.
The origin of CWD has yet to be determined (Sigurdson & Aguzzi, 2007). The infection was first noted in 1967 at a captive mule deer research facility. In 1978 pathologists recognized the TSE type brain lesions, also that CWD presented as a prion disease by the neuronal perikaryonic vacuoles, the accumulation of aggregated prion protein and prion infectivity in the brain. In the late 1970s and early 1980s the infection w...
PrP can occur in two forms- a normal cellular prion protein known as PrPc and a pathogenic misfolded conformer known as PrPsc. The abnormal PrPsc differs from the normal prion protein PrPc in both secondary and tertiary structure. PrPsc is principally rich in Beta sheet contents but PrPc is principally rich in alpha helical contents. Due to this difference of between the isoforms, prions are extremely resistant to certain decontamination systems. The Two tables below outline both human and animal diseases (2).
James Parkinson. It’s not certain how long the disease has existed but its probably been around
Autopsies of affected cattle reveal holes in the brain tissue that give it a spongy, or spongiform, texture. Similar spongiform diseases have been recognized in humans (for example, Creutzfeldt-Jakob disease or CJD) for over a century and in sheep (scrapie) for over 200 years. The cause of BSE is unproven, although there is strong evidence that prions, which may be infective proteins, are the agent. Other hypotheses suggest that prions work with an as yet undetected virus to cause the infection.
The prion diseases that Chronic Wasting Disease is related to are Creutzfeldt-Jakobs disease found in humans, bovine spongiform encephalopathy (BSE) in cattle, and scrapies in sheep (3,4). These diseases are grouped together because they share certain characteristics such as long incubation periods, spongiform changes that are associated with neural loss, and cause failure to induce inflammatory responses (Chronic Wasting Disease Alliance).
Prions are pathogens, and cause infections, like viruses. Prions cause many neurodegenerative diseases, but are made up of harmless proteins found in mammals and birds. The proteins are not in their normal form though, and once they enter the human brain, can cause severe brain infections. One thing that makes them different from viruses, is the lack of nucleic acids, which means they have no genetic code. Once in the brain, they make normal proteins turn into abnormal ones, which then multiply, causing severe infection. Soon, holes appear in the brain that can only be treated by incineration. An example of a disease caused by a prion would be the Mad Cow Disease, or the human equivalent Creutzfeldt–Jakob disease. Prions are very dangerous. While some people can confuse prions and viruses, there are some ways to tell the difference.
Alzheimer’s disease is a complex illness that affects the brain tissue directly and undergoes gradual memory and behavioral changes which makes it difficult to diagnose. It is known to be the most common form of dementia and is irreversible. Over four million older Americans have Alzheimer’s, and that number is expected to triple in the next twenty years as more people live into their eighties and nineties. (Johnson, 1989). There is still no cure for Alzheimer’s but throughout the past few years a lot of progress has been made.
The first discovery was made in 1952, in the developing field of virology. Virology is the study of viruses and how they behave. To develop the vaccines for the viruses, researchers infected the HeLa cells with many types of infections, such as measles, mumps, and the infamous poliomyelitis virus, also known as Polio. According to the Centers for Disease Control and Prevention (CDC), whose mission is to save lives and protect people’s health security, Polio is a "crippling and potentially deadly infectious disease caused by a virus that spreads from person to person invading the brain and spinal cord and causing paralysis" (Freeman). Jonas Salk, who was a virologist at the National Foundation for Infantile Paralysis (NFIP), used inactivated viruses (virus particles grown in culture and then killed by a form of heat) to create a polio vaccine. Salk drew blood from about two million children, which the NFIP checked for immunization.Through the collection of many HeLa cells and trial and error, the polio vaccine wa...
Creutzfeldt-Jakob Disease is an uncommon, deteriorating, consistently fatal brain disorder that is caused by prions. The symptoms of CJD are similar of Alzheimer’s but progress much faster. There are three variations of CJD, sporadic, familial, and acquired. All variations affect the brain the same way and have the same result of death. CJD is an untreatable and incurable disease.