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Studies have shown a balanced diet, regular exercise and at least six to eight hours of sleep can help lead a healthy and productive life. Removing rest from this formula can greatly diminish one’s health. Some of the many repercussions of lack of sleep can include stroke, loss of memory, diabetes, or even a heart attack. (http://www.webmd.com/sleep-disorders/excessive-sleepiness-10/10-results-sleep-loss). Now imagine that the ability to sleep isn’t even a possibility, that one is stuck between a never ending ability to stay awake and death. This condition is known as Fatal Familial Insomnia. Fatal Familial Insomnia (FFI) is an extremely rare, hereditary prion disease that carries a death sentence. The average life expectancy from showing symptoms of the disease to death is on average 18 months, but can be anywhere from 7 – 36 months (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1781306/).
The brain naturally has cellular prion protein (PrPc) that have normal turnover – they are regularly destroyed by the host cell proteases, and new ones are synthesized. The infectious prion proteins (PrPsc) are less susceptible to the host cell’s proteases, so the number of infectious prions builds up in the brain. Although it has not been proven, it has been hypothesized that PrPsc interacts directly with PrPc and converts the normal prion’s properties into an infectious prion (Nestor, et al). Prion proteins tend to accumulate in neural tissue – causing the brain tissue to develop transmissible spongiform encephalopathies. These are basically holes that develop in brain tissue that creates a “spongelike appearance” (Nestor et al). Examples of prion diseases include Mad Cow Disease, Creutzfeldt - Jakob disease (CJD) and Fatal Familial Inso...
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...nt was alert and responded to questions. The continued use of this medication worked well for two weeks, but in the third week the patient developed fevers and restlessness and then eventually died (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1781306/).
There are several studies happening at sleep clinics all over the world trying to find appropriate treatment and the potential eradication of FFI, but so far there is no known cure. There of course are blood tests that can determine if a person has the gene, but since there is no treatment for this disease ultimately the patient must decide if they want to know their estimated and possibly reduced life expectancy.
As time progresses, the cure for FFI and other prion diseases are still to be determined. Until science truly understands prions and their effects on the human body, a cure for these may not be in reach.
Another feature of the prion is its ability to remain stable in extreme conditions. Because prions do not have any DNA or RNA like other infectious agents, they are very hard to deal with. Prions are extremely resistant to conventional procedures to inactivate them including irradiation, boiling, dry heat, and chemicals such asformalin, betapropiolactone, and alcohols.
Mad Cow Disease, scientifically referred to as (BSE) Bovine Spongiform Encephalopathy, is a disease that affects those humans who eat the meat from infected cows. Mad Cow Disease is one of several fatal brain diseases called (TSE) Transmissible Spongiform Encephalopathy. (USDA) There was evidence of a new illness resembling the sheep disease scrapie. It was technically named BSE but quickly acquired the mad cow tag because of the way infected cattle behave. (CNN) In 1997, there was an award given to Stanley Prusiner, for concluding that a distorted protein called a prion was responsible for Mad Cow Disease, noted the long incubation period made it difficult to distinguish (Bryant). Another name for Mad Cow Disease is the new variant Cruetzfeldt-Jakob Disease (vCJD), similar to the Creutzfeldt-Jakob Disease, which is a deadly brain illness that strikes about one per million per year (USDA) due to genetic or unknown causes while the vCJD is contracted from eating infected cows (USDA). Both CJD and vCJD are so similarly named because of the similar effects from the illness.
Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however
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...
TSEs or more commonly prion diseases are a group of invariably fatal neurodegenerative diseases that occur in humans and animals . This disease is caused by a protease –resistant protein (PrPsc) after misfolding of a host-encoded prion protein (PrP). TSEs can exist as genetic, infectious or sporadic forms. The diseases are characterized by dementia, ataxia and neuropathlogically due to loss of specific neurons in the brain. Other clinical features include persistent painful stimuli, dystonia, visual or cerebellar problems and gliosis (1).
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.
Chronic sleep loss is becoming more common in modern culture and less restricted to sleep-deprived diseases such as insomnia. Suggested to be the result of a number car, industrial, medical, and other occupational accidents, sleep deprivation is beginning to be recognized as a public concern. As a result, the Centers for Disease Control
Sleep is one of our basic needs to survive and to function in day to day operations, but not everyone needs the same amount of sleep. Some people can survive on very little sleep, i.e. five hours a night, and some people need a lot of sleep, to the extend that they are sleeping up to 10 to sometimes 15 hours a night (Nature, 2005). According to Wilson (2005) the general rule states that most people need from seven to eight hours of sleep. The deprivation of sleep in our society in continually increasing with the demands in society increasing work loads, the myth that a few hours of sleep is only necessary to function properly and that sleep is sometimes considered as killing time (Nature, 2005). Sometimes sleep deprivation is also caused by other situations like sleep disorders, i.e. sleep apnea, chronic insomnia or medical conditions such as stress (Wilson, 2005).
Sleep deprivation is often associated with the development of medical conditions, especially cardiovascular disease (CVD). As disturbance in sleep could cause deprivation and shorten one’s sleep duration, it is often taken into account when carrying out related studies. Short-term sleep deprivation not only raises blood pressure and produces stress hormones, but also leads to irregular heartbeats [1]. These major risk factors are precursors to coronary disease. In this essay, I will summarize the findings of five studies in relation to sleep deprivation and the increased risk of CVD.
Because CJD is such a rapid progressing disease most patients die within one year of onset. Some of the symptoms associated with CJD are much like Alzheimer’s. “Some common symptoms include depression, agitation, apathy and mood swings, rapidly worsening confusion, disorientation and problems with memory, thinking, planning and judgement, difficulty walking, and muscle stiffness, twitches, and involuntary movements.” (Alzheimer's Association, 2016). Diagnosis of CJD is a controversial issue. The only way to diagnose CJD is by brain biopsy or autopsy. In a brain biopsy, a small piece of tissue is removed and examined by a neuropathologist. This is of course dangerous and not always effective because it is hard to know if the tissue obtained is from the affected part of the brain. Biopsies are usually not recommended, not only because of the risk, but because there is no treatment available that will cure CJD. “There is no treatment that can slow or stop the underlying brain cell destruction caused by Creutzfeldt-Jakob disease and other prion diseases. Various drugs have been tested but have not shown any benefit. Clinical studies of potential CJD treatments are complicated by the rarity of the disease and its rapid progression.” (Alzheimer's Association, 2016). Treatment for CJD is aimed at alleviating symptoms and making the patient as comfortable as possible. “Doctors may prescribe painkillers such as opiates to treat pain if it occurs. Muscle stiffness and twitching may be treated with muscle-relaxing medications or antiseizure drugs. In the later stages of the disease, individuals with CJD become completely dependent on others for their daily needs and comfort.” (Alzheimer's Association,
Kales, A. (1972). The evaluation and treatment of sleep disorders : Pharmacological and psychological studies. In M. Chase (ed.)The Sleeping Brain. Los Angeles : Brain Information Service.
...tressed out that we can't sleep at night. Insomnia can affect a person really fast and it's hard to get rid of this disorder without a lot of effort. If we follow the advice of the doctor’s and begin to live a structured life all could be well.
Sleep is a very important factor in the human function. Our body and brain is able to reset itself and rejuvenate while we sleep. When we do not get the required amount of sleep, we start to feel lethargic and foggy minded, because our mind and body wasn’t able to replenish itself. Sleep is imperative that an insignificant rest deficiency or lack of sleep can affect our ability to remember things; decisions and can affect our temperament. Chronic sleep deficiency can get the body to feel agitated and it could lead to serious health problems such as, heart problems, stress, acne, and obesity.