Spongiform Encephalopathy Case Study

This disease effects the central nervous system, mainly the brain of an adult cow. An abnormal protein called prion is thought to kill the brain cells and create small holes in the brain tissue. MCD is a disease that progresses very slowly and is fatal. (WebMD, LLC, 2015).

The U.S. Department of Agriculture (USDA) has tested hundreds of thousands of cattle for BSE. Researchers believe that the infectious agent that causes mad cow disease is an abnormal version of a protein normally found on cell surfaces, called a prion. For reasons still unknown, this protein becomes altered and destroys nervous system tissue (brain and spinal cord). There exists strong epidemiologic and laboratory evidence for a causal association between a new human prion disease called variant Creutzfeldt-Jakob disease (vCJD) that was first reported from the United Kingdom in 1996 and the BSE outbreak in cattle (http://www.cdc.gov). According to The National Creutzfeldt-Jakob Disease Surveillance Unit, by June 2014 it had killed 177 people in the United Kingdom, and 52 elsewhere. This essay will focus on the possible causes, effects, and treatment for this

Chronic Wasting Disease (CWD) is a fatal neurodegenerative transmissible spongiform encephalopathy (TSE ) found in some white tailed deer. CWD is associated with diseases such as Mad-Cow disease, Scrapie, and Creutzfeldt–Jakob disease. With well supported evidence it is hypothesized that CWD originated from Scrapie. The causation of CWD is an abnormal form of PrPcwd prion. This prion is a naturally occurring protein found in mammals. Prions lack genome and are highly resistant to degradation. Chronic Wasting Disease can be transmitted by direct contact between the animals and/or on resources such as salt licks.

Mad cow disease, also known as bovine spongiform encephalopathy (BSE), is a transmissible, slowly progressive, degenerative, and fatal disease which affects the central nervous system of adult cattle. Many researchers believe that an abnormal version of a protein normally found on cell surfaces, called a prion is the main reason to cause mad cow disease. For some still unknown reasons, this protein becomes modified and ruins the nervous system tissue, the brain and the spinal cord.

The Idea of writing about Acute Disseminated Encephalomyelitis came to me as I was watching the medical show called Mystery Diagnosis in discovery. This neurological disease caught my attention so much because the patient went to several doctors and no one could give her a clear answer as to why this symptoms were happening. Also, with rare cases like this one it only further proves that the human body is still a mystery.This neurological disease relates to our Anatomy and physiology class because of the simple fact that we are trying to learn and understand how the body as a whole is made but also how each part connect, functions, and could potentially damage each other. More Importantly, this neurological disorder further enhances our learning in possible disease that can affect the central nervous system as well as the peripheral nervous system.

2Jordanne Rice21944377As a Veterinary technician, you may encounter the many diseases that are carried by largeand small animals. Having knowledge of such diseases can prevent the spread to other animals and decrease any chance of human health endangerment. Further paragraphs will include the etiology, signs and symptoms, diagnostic tools, treatment protocols, and prevention and control programs for a handful of the many diseases that animals can encounter. Bovine Spongiform Encephalopathy (BSE) is a neurological disorder that affects cattle and that results from infection by a transmissible agent called a prion (1A). It is a contagious and infectious disease. It is also called mad cow disease. The term spongiform comes from the way the brain

Prior to 2009, 90% of people suffering from Autoimmune Encephalitis were undiagnosed, or misdiagnosed. Thanks to the help of modern day doctors, discoveries have been made to treat the victims of Autoimmune Encephalitis. Autoimmune Encephalitis, or AE, is an infection where the immune system attacks the brain, impairing function (McDow, Will). This infection results in brain inflammation; this is the swelling of blood vessels in the brains tissue caused by a malfunction in another body system (McDow, Will). Also, although this disease has been recently discovered, the disease itself has been around for many years. In 2005, Dr. Josep Dalmau discovered an antibody to fight against the disease; now there are twenty two known antibodies. Because

Prion-like proteins are normal proteins with similar prion-like domains which may also be involved in diseases of the brain or other organs. Seeded protein aggregation will lead to many more diseases of the brain. In contrast to prions being simple infection in yeast cells prion-forming proteins initiate cell process such as the central dogma. The prion-like proteins may not be widely pathogenic

The role of prions in BSE is to trigger proteins in the brain to fold abnormally. These prions are formed by abnormally folded protein that causes neurodegenerative conditions, similarly to that of Alzheimer’s disease. These misfolded proteins tend to clump together, or aggregate, because of their shape. This disease has no known cause but is generally associated with the ingestion of meat from cows who already have BSE.

Creutzfeldt - Jakob disease is a prion disease characterized by the degeneration of the cerebral cortex, basal ganglia, cerebellum, brainstem and spinal cord. CJD is classified into 4 types: sporadic, familial, iatrogenic, and contaminated meat. CJD represents approximately 85% of all human prion diseases . Although it is common in older ages (50-70), both men and women have an equal chance of acquiring the disease. Recently, there has been a new variant CJD that is thought to be from the ingestion of cattle products in Great Britain. The central characteristic that differentiates vCJD and CJD is that it occurs more commonly in younger people and also has different pathogenic findings. The second variant form is the panencephalopathic form, which is found in Japan.

Bovine Spongiform Encephalopathy “is a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion”. (Center for Disease Control & Prevention, 2015).

On Thursday, Jan. 19, 2017, DCHHS held the first Public Health Advisory Committee Meeting of the year. Lead by Commissioner John Wiley Price, DCHHS Director Zachary Thompson and Epidemiologist Sonya Hughes, this quarters meeting focused on West Nile and Zika Virus updates in Dallas County. Dallas County has identified 2016 as one of the busier mosquito’s seasons to date; however Hughes confirmed West Nile Season infection rates are still drastically lower in comparisons to the 2012 season.

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

Scientists determined that CWD transmission occurs when disease prions are shed by infected animals through saliva, urine, feces and natural decomposition after death. A prion is the agent that transmits TSE category diseases. In addition, CWD prions are extremely resistant to the environment, and transmission may occur directly between animals or indirectly between uninfected animals and the environment (Witkowski 3). Thus, the disease is highly contagious, as the prions are very hardy and can survive nearly anywhere. The prions adhere and bind to the substrate using competitive inhibition, or when the inhibitor binds to the active site on the substrate, kicking off and not allowing the enzyme that would normally bind to attach there. As a result, the infectious prion takes over and is what causes the transmission of the disease. Also, because the sickness is spread through feces and saliva, everywhere an infected deer eats or walks throughout the day may contain traces of contamination. Supplementary, CWD is an unfailingly fatal brain disease. Once a deer contracts the fatal illness, the life expectancy ranges anywhere from 18 to 20 months. Deer typically look healthy the first 16

Transmissible spongiform encephalopathies (TSEs), or Prion diseases, are a group of neurodegenerative disorders which include but is not limited to kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia in men, natural scrapie in sheep, goats and mufflons. The first records of neurodegenerative disease which would later be linked to Prions were made about two hundred years ago. It was in the 1950’s it was discovered that Prion disease could be transmitted horizontally, in addition to being inheritable and spontaneous. These findings were sought out by isolated tribes of the New Guinea that were suffering from high prevalence of a particular TST called Kuru. Some additional history of Prion disease dates back to the 1980’s when an increasing number of cows in the UK became infected with Mad Cow Disease also known as Bovine Spongiform Encephalopathy. This unfortunate epidemic was traced back to a food supplement that was fed to the cows, this supplement contained mean and bone meal from dead sheep. A speculation was then drawn that the BSE had been a result of the sheep that had suffered from scrapie, which is a sheep-specific Prion disease. As a result it was decided by the British government that the banning of animal derived feed supplements. This helped to decrease the disease of cattle which subsequently disrupted the spread of the disease amongst livestock and animals.