Creutzfeldt-Jakob disease
Creutzfeldt-Jakob is a fatal, progrossive and incurable neurologic disorder which affects the brain in a destructive way. Globally, it attacks one person in every one million people. Specifically, at United State there are more than 300 cases per year. However, it is extremely complicated to diagnose, so the number of cases might get higher in the future. CJD (Creutzfeldt-Jakob disease) symptoms usually occur in later life for women and men whose ages are between 60 and 70. This essay displays a definition of Creutzfeldt-Jakob disease based on its identity, causes, symptoms, risk factors and treatment.
Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however
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.
Walton, Sir John. Brain's Diseases of the Nervous System. Oxford University Press. New York, 1955, pp. 365.
Creutzfeldt-Jakob disease (CJD) is an unusual, degenerative, consistently terminal brain disorder, typically onset of symptoms occurs at about age 60. This disease has been categorized into three major categories: 1) sporadic CJD, 2) hereditary CJD, 3) acquired CJD.
Neurodegeneration is used mainly for diseases that are characterised by progressive loss of structure and function of neurons. There are many neurodegenerative diseases including amyotrophic lateral sclerosis that...
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).
Canavan Disease is a fatal neurological disease where there is significant damage to the nerve cells in the brain. There is a defect in the myelin sheath that causes many problems for the nervous system. The major problem is caused when the enzyme aspartoacyle is not present. This missing enzyme causes a chemical imbalance that causes this defect in the myelin sheath. The myelin in the brain destructs which makes it a spongy tissue. This causes overall muscle weakening and slower movements, leading to severe mental retardation. A recent study has shown that the cells in the brain that are responsible for making myelin sheaths (oligodendrocytes), cannot complete the task. When babies are born they may not show any signs at all until the first few months. This disease is only inherited and categorized under a group of diseases called leukodystrophies. Leukodystrophies gets its name because it means there is a degeneration of myelin, which is a fatty cushioning that shields nerve fibers. This makes the nerve signals very difficult to transmit. People with Canavan Disease life span can range from a couple days, months, or maybe even until their twenties (Genetics Home Reference, n.d.); (Canavan Foundation, n.d.).
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.
With the advent of more effective highly active antiretroviral therapy (HAART) and thus increased life span of people with AIDS, neurological disorders are becoming a hot topic in AIDS research. In the early days of the epidemic, those infected with the virus could only hope to live for a short time before developing the symptoms of full blown AIDS, and death ensued shortly afterwards. The progress made in treatment in the past two decades has prolonged the lives of people with AIDS, to the point where diagnosis is no longer a sign of imminent debilitation and death, but rather an acknowledgement of a possible long road ahead with the aid of drug cocktails. There is also a strong possibility that the HIV infected person may develop HIV associated dementia after years of living with the disease (1).
Walton, Sir John. Brain’s Diseases of the Nervous System. 9th ed. Oxford University Press. Oxford: 1985.
Imagine if you loss control of your body but your mind stayed unaffected. You would be a prisoner in your own body, all leading up to your death sentence. That is the sad fate for the people diagnosed with Amyotrophic lateral sclerosis (ALS). “Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder was first described by Ran in 1850. This description was then expanded in 1873 by Charcot, who emphasized the involvement of the corticospinal tracts. In the United States, ALS is often referred to as Lou Gehrig's disease, after the famous ball player who was stricken by the disease in the midst of his career. (Yale School of Medicine, 2014)” In this paper will go through the definition, the process, the signs, the risk factors, etiology, and discus the known people that have suffered with this terminal disease.
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.
Leigh syndrome is a fatal disorder that causes progressive neurodegeneration in mostly young kids. It was discovered in 1951 by Denis Leigh who originally named it Necrotizing Encephalomyelopathy. Leigh originally classified it based on phenotypes found in a boy who had normal development until the age of 6 months. After this the boy showed various phenotypes including optic atrophy, deafness, and bilateral spasticity. The neurological phenotypes displayed in the boy were: neuron degeneration, gliosis of thalamus, midbrain, medulla, pons, and spinal cord. Leigh believed that the disorder was caused by a lack of thiamine in the boys system (Leigh, 1951).
With motor neurone disease it attacks the nerves, in the brain and spinal cord. This means messages gradually stop reaching muscles, which leads to weakness and wasting. In the case study the