Creutzfeldt-Jakob Disease Case Study

In the early 1900’s, the Fore tribe was struck with an epidemic of Kuru, a brain eating disorder that causes shaking, and furthermore the name “Kuru” in the means shaking or trembling in a literal sense. The Fore spread this disease a fair bit, specifically due to their cannibalistic funeral practices of eating the remains of the dead, and specifically the brain (Bichell, Rae Ellen 2016). However, the presence of Prions have been around for eras, but the discovery in the Fore tribe spearheaded the movement to learn more about them. Eventually in 1982, the “Prion protein” was discovered by Stanley Prusiner, eventually earning him the Nobel Prize in 1997 for his proof of the lack of DNA or RNA in the protein, juxtaposed to bacteria and viruses (Prion Alliance, 2016). As the amount of diseases discovered increased, it became eerily known that the Prion disease in humans and animals were all caused by one original misfolded protein, PrPsc. Despite a common origin, pinning down a solution to the problems of Prions is an extremely complex issue. With an extreme resistance to everything, Prions opens the question of sanitization in hospitals. If a doctor were to be working with a Prion-contaminated sample, the tools used may be thought to be completely clean, yet Prions being highly resistant would persevere against normal

At this point, coordination is at a point where falls are often major risks, and considering many cases of the disease occur in the elderly, this can become a fatal risk. Close relatives are soon to become unrecognizable and the long term memory of the individual begins to fade as the disease worsens. It is often that at this point of the disease, the patient is put into a care center, as they may become a burden to the family members around them. Emotions swing and become entirely unpredictable and often resistance to care will occur as the patients lose awareness of their condition and become confused with their surroundings.

The third stage is deterioration or disturbance. This stage altitude is between 11400 to 20000ft.(3) In this stage obvious symptoms is begin, but not everyone can recognize the symptoms of this stage. (1) (2) And there is some of these symptoms shortness of breath/air hunger, incoordination, cyanosis, difficulty with simple tasks, drowsiness, diminished vision, headache, tingling, euphoria, numbness, aggression, hot/cold flashes, and poor judgement.(1) The arterial oxygen saturations in this phase is between 70 to 80 percent.(10)

Humans have to deal with many different diseases and the ones most disliked are the ones with no cures. Like cancer, transmissible spongiform encephalopathies have no cure, but they are more rare. These diseases are prion diseases which cause the brain to deteriorate. Prions are proteins that sometimes behave like viruses, which mean that they should have some form of nucleic acid, but since they don’t, they cause abnormalities. The nervous system contains many normal prions, but when an abnormal prion comes along, it transforms all the normal prions into abnormal ones. Bovine spongiform encephalopathy is found in cattle, but it can be transmitted to humans.

This disease gets its name from the German neurologist Hans Gerhard Creutzfeldt and Alfons Maria Jakob that first identified this disease. (Victor, 2015) VCJD is primarily found in the brain of the cattle. Abnormal proteins called prions have thought to be the cause of this disease in both cattle and in humans. These prions cause tiny holes that look like a sponge under a microscope. (Melissa Conrad Stöppler, 2015) Unlike most viruses and bacteria these prions unfortunately do not die when exposed to heat, ultraviolet light, and radiation. Disinfectants that are usually used to kill viruses and bacteria also do not work to kill prions. Even cooking the meat very well will not lower the risk of prions in the

This week may be the last week I write to you. In my last letter to you, you may have noticed that I seemed depressed and not like myself. I wasn’t completely truthful in that letter. I’m not just having a bad day, I’m diseased with an incurable, fatal disease.

Genetic instability refers to temporary or permanent unscheduled alterations within the genome occur and can occur both at chromosomal or nucleotide level. Instability at nucleotide level consists of increased frequency of base-pair mutation or amplified number of nucleotide repeat units such as trinucleotide repeats (TNR) in a gene which will show altered expression and malfunction of RNA and/or protein (Castel et al., 2010).

Acute disseminated encephalomyelitis is an immune-mediated inflammatory demyelinating condition that affects the white matter of the brain and spinal cord. ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which destroys the white matter. It is often triggered after the patient has received a viral infection or sometimes exceedingly rarely specific non-routine vaccinations. It affects children more than adults but can affect anyone. More than half of patients have an illness usually an infection two to four weeks before developing ADEM. Most of these illnesses are viral or bacterial, often no more than an upper respiratory tract infection. In children with ADEM, prolonged and severe headaches occur. In addition, the patient develops fevers during the ADEM course.

“Korsakoff syndrome is a preventable memory disorder that usually emerges (although not always) in the outcome of an episode of Wernicke 's encephalopathy, therefore the chronic disorder is sometimes known as Wernicke- Korsakoff syndrome.” Wernicke encephalopathy is an acute brain reaction to severe lack of thiamine. Wernicke encephalopathy is a medical emergency that causes life-threatening brain disruption, confusion, staggering and stumbling, lack of coordination, and abnormal involuntary eye movements.”

KD is a very rare disorder, the prevalence thought to be 2 per 100,000 people according to the Cell and Tissue Research article. It is thought that KD is often misdiagnosis as amyotrophic lateral sclerosis (ALS). The first case ever recorded of KD came from a patient who was previously diagnosis with ALS and wanted a seconded opinion. The onset for KD is usually midlife, the range being from 30-60 years of age. The onset is often preceded by non-specific

In the beginning, they thought that the practice of cannibalism was the reason for this disease. After many years of study and research they learned that was so. Michael Alper, Carlton Gajdusek, and Shirley Linderbaum were directly involved and responsible for discovering the source, mapping its progression, and finding the first victim of Kuru. Through their Study and research Linderbaum determined that the first person afflicted in this tribe was back in the early 1900. Alper figured out that it was being transmitted through the mortuary eating of the brain in particular and Gajdusek and the others discerned that Scrapie, Creutzfeldt-Jakob disease and Kuru the way they transmitted were closely related and they believe that the first person came down with Creutzfeldt-Jakob disease which is a spontaneous change in the brain, they died and were consumed. They proved that it was transmittable from humans to primates and from animals to humans. Later, Biochemist Stanley Prusiner gave the named a new pathogen in over 100 years, Prion, a protein that duplicates itself by recruiting other protein replicating itself and infecting the brain, organs and vascular system. What made this pathogen special and very different was that it has no know antibodies, it was incredibly small nearly 1000 times smaller than any infecting

CJD is a serious neurodegenerative disorder, a rare form of rapidly progressive dementia, with a 100% fatality within one to two years of onset. CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). It is believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein that becomes toxic in an abnormal form (CDC) although Dr. Campellone suggest other proteins may play a role in this disease as well. Although, it is so rare that only between one to every one million people worldwide are diagnosed.

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.

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are thought to be caused by the misfolding of prion proteins. Prions are able to replicate in the absence of nucleic acids. TSEs include: scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, kuru, Gerstmann-Straussler-Scheinker disease, and Fatal Familial Insomnia. They can affect many different animals, including humans. Currently, there are no ways to diagnose, treat, or cure TSEs, as much more research is needed before these diseases are completely understood.

Regardless of which occur first, affected individuals will generally experience most of the symptoms as the disease progresses. Specifically, symptoms that include difficulty swallowing, moving, forming words, tight muscles, spasticity, and/or exaggerated reflexes occur when the upper motor neurons have been damaged. When lower motor neurons are damaged, symptoms such as muscle weakness, atrophy, cramping, and twitching will occur. In some cases, a few of the muscle neurons that