Spongiform Encephalopathy Creutzfeldt SubagudaJakob, Devaca Disease Mad, Mad lasVacas Disease, Evil lasJakob,
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Creutzfeldt Jakob disease, first described in 1920, belongs to a family of diseases in humans and animals known as encephalopathy transmissible spongiform. The term Spongiform refers to the characteristic appearance of infected brains, which are filled with holes or holes until they resemble sponges under a microscope. Creutzfeldt Jakob disease is the most common known human transmissible spongiform encephalopathies, which include Kuru, people identified in an isolated tribe in Papua New Guinea and has almost disappeared, fatal familial insomnia and Gerstmann Straussler Scheinker, the latter two are extremely rare hereditary diseases, found in only a few families around the world. There are transmissible spongiform encephalopathies in specific types of animals, such as spongiform encephalopathy cattle, found in cows and disease is often called the "mad cow" scrapie, which affects sheep and mink encephalopathy. Similar diseases have also been reported in elk, deer and exotic animals in zoos. Although transmissible spongiform encephalopathies, are considered viral encephalitis slow virus, currently the scientific literature maintains that Creutzfeldt Jakob disease and other transmissible spongiform encephalopathies are caused not by known organisms such as viruses and bacteria, but a type of protein called a prion (protein substances, different viruses and bacteria are difficult kill, do not appear to contain genetic information in the form of nucleic acids, DNA or RNA and generally have a long incubation period before symptoms appear). In some cases, the incubation period can be up to 40 years. Prions are found in normal cells in the body in a safe manner, but also occur in an infectious form and then cause disease. Both forms of prion protein are very similar, by which a person's normal prions spontaneously change to the infectious form of the protein and then a chain reaction will alter the prions in other cells. A occurrence, the abnormal prion proteins unite to form fibers or clumps called plaques that are visible with powerful microscopes that can start to accumulate years before symptoms begin to appear of the disease. It is unclear what role the abnormal prion protein and the appearance of the disease or development of clinical manifestations. Initially, the symptoms of Creutzfeldt Jakob disease can include confusion, memory failure, depression, behavioral changes, lack of coordination and visual disturbances. As the disease progresses, patients experience rapid dementia (loss progresiva de capacidades intelectuales), alteraciones neuromuscular tales como hipotonía (tono anormalmente disminuido del músculo) y atrofia (pérdida de masa del músculo) y debilidad de las extremidades, sacudidas mioclónicas (contracciones musculares involuntarias) y atetosis (movimientos anormales involuntarios, de brazos y piernas). En fases más avanzadas de la enfermedad incluyen la pérdida adicional de funciones físicas e intelectuales, ceguera y coma. La muerte sobreviene a consecuencia de las infecciones, generalmente pulmonares, que surgen como complicación de la enfermedad. Se conocen tres formas clínicas de la enfermedad de Creutzfeldt Jakob: 1.- La forma esporádica, en la que la enfermedad appears even if the person has no risk factors for the disease known. It is the most common type of Creutzfeldt Jakob disease, manifested by at least 85% of cases. 2 .- The hereditary form, in which the person has a history of the disease in family history, or positive evidence of genetic mutation associated with the disease. It is estimated that 5-10% of cases of Creutzfeldt Jakob disease is hereditary and arise from a mutation, or change in the gene that controls formation of normal prion protein. If the prion gene is altered in the sperm or egg cells of a person, the mutation can be transmitted to children. Have identified several different mutations in the prion gene. The specific mutation found in each family affects the frequency of occurrence of the disease and what symptoms are most noticeable. However, not all people with mutations in the gene acquire prions Creutzfeldt Jakob disease. This indicates that mutations affecting the disease susceptibility and that other still unknown factors also play a role in disease. 3 .- The acquired form, in which the disease is spread by exposure to brain or nervous system, usually through certain medical procedures. There is no evidence that this disease can spread through casual contact with a patient with Creutzfeldt Jakob disease. Less than 1% of cases of Creutzfeldt Jakob disease are acquired. Have described several variants of the disease, which differ somewhat in the symptoms and course of it. The principal is called new variant Creutzfeldt Jakob disease or "mad cow", described mainly in Britain and other European countries, which affects younger patients. Begins primarily with psychiatric symptoms and has a longer evolution. In Spain to date, according to the Center of Reference for Bovine Spongiform Encephalopathy (BSE), there have been 60 cases BSE. Has been related to the fact that BSE can be transmitted to humans through consumption of contaminated beef. Another variant, called a panencefalopática, occurs mainly in Japan and has a relatively long course of the disease, with symptoms often progressing over several years. Some symptoms of Creutzfeldt Jakob disease can mimic symptoms of other progressive neurological disorders such as Alzheimer's or Huntington's disease. However, Creutzfeldt Jakob disease causes characteristic changes in brain tissue that can be seen at autopsy. It also tends to cause a deterioration faster than the capabilities of a person Alzheimer's disease or most other types of dementia. At present, there is evidence of diagnostic orientation, including an extraction cord to rule out more common causes of dementia and detects a protein marker indicating neuronal degeneration; EEG to record the unique electrical pattern of brain MRI scanner, which can also highlight characteristic patterns of brain degeneration that can help diagnose the disease. However, the only way to confirm the diagnosis is by biopsy (operation consisting in removing the living individual a piece of organ or tumor in order to subject it to microscopic examination) or brain autopsy, which reveals a series of characteristic lesions of which the fundamental is at spongiosis of the cerebral cortex, thalamus , basal ganglia and cerebellum, neuronal loss occurs gradually as the disease progresses intense glial proliferation, all of these lesions are known as spongiform status. May also occur at times of amyloid deposits, which may be diffuse or in plates and in which substances can be identified prion, the plate-shaped deposits called plaques of Kuru, as they were described for the first time in the Kuru, the pathological lesion progresses from the cortex to the thalamus, unlike what happens in fatal familial insomnia in which the progression of the lesion is cortical structures to the thalamus. Several drugs have been tried such as amantadine, steroids, interferon, acyclovir, antiviral agents and antibiotics, however, none of these treatments has shown a profit, so that therapeutic measures are symptomatic, aimed at controlling myoclonus pain, opioid drugs can help reduce pain. There is currently no treatment that can cure or control the disease. However, an experimental treatment trials are being initiated with quinacrine, a drug used for years to combat malaria and with chlorpromazine, a medication for psychiatric disorders. Although Creutzfeldt Jakob disease can be transmitted to others, the risk of this occurring is extremely low. The disease is not transmitted through air or touch another person or by most forms of casual contact. Spouses and other family members of patients with sporadic Creutzfeldt-Jakob disease are not subject to a higher risk of contracting the disease than the general population. However, the direct or indirect contact with brain tissue and the spinal cord fluid from infected patients should be avoided to prevent disease transmission through these materials. 2007
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