Creutzfeldt-Jakob Disease (Cjd)

(Subacute Spongiform Encephalopathy, Creutzfeldt-Jakob disease)

Creutzfeldt-Jakob disease (CJD) is the most common prion disease in humans. It occurs worldwide and has various forms. Among the CJD symptoms include dementia, myoclonus and other CNS deficits; Death usually occurs between the fourth month and the second year after infection, depending on the form of CJD. Treatment is symptomatic.

CJD has several forms:

Creutzfeldt-Jakob disease (CJD) is the most common prion disease in humans. It occurs worldwide and has various forms. Among the CJD symptoms include dementia, myoclonus and other CNS deficits; Death usually occurs between the fourth month and the second year after infection, depending on the form of CJD. Treatment is symptomatic. CJD has several forms: Sporadic (sCJD) Family Acquired CJD is the most common type and accounts for about 85% of cases. The sCKD typically affects people beyond the age of 40 (the median is 60 years). Familial CJD occurs in 5- 15% of cases. Inheritance occurs as an autosomal dominant and usually starts earlier than for CJD, and the illness lasts longer. Acquired CJD accounts for <1% of cases. It is occurred (in vCJD) after ingestion of contaminated beef caused by prions. Iatrogenic CJD has been, by the use of corneal or dura mater transplants, intracerebral stereotactic electrodes or the use of growth hormone, which has been obtained from human pituitary is transmitted. Variant CJD (vCJD) The vCJD is rare. Most cases have occurred in the United Kingdom (UK), where there were 177 cases in May 2015 compared to 52 cases in all other European and non-European countries. vCJD occurs after the intake of beef cattle with bovine spongiform encephalopathy (BSE), also known as mad cow disease. In vCJD, the symptoms develop at a younger average age (<30 years) than in the sCJD. In recent cases, the incubation time was (time between ingestion of contaminated beef and development of symptoms) from 12 to> 20 years. In the early 1980s, probably from scrapie-infected sheep or BSE-infected cattle, which scrapie Proin protein (PrPSc) was introduced into cattle feed due to relaxed rules for the production of animal by-products via contaminated tissue. Hundreds of thousands of cattle have developed BSE. Despite the relatively extended exposure have few people who had eaten meat from affected cattle, developed vCJD. Because the incubation period is at BSE, has been so long recognized no link between BSE and contaminated feed in the UK until BSE had become an epidemic. the BSE epidemic after massive slaughter of cattle and to process changes in the carcass disposal and recycling, whereby the contamination of meat has been dramatically reduced by nerve tissue was brought under control. In the UK, the annual number of cases of vCJD, which had in 2000 reached its peak, steadily declined and is only one case in 2013. 4 cases of vCJD have been associated with blood transfusions; they occurred in people who had received between 1996 and 1999 transfusions. In the UK, about 1/2000 people may be ill of vCJD have (based on the examination of a large number of tissue samples) but no symptoms; these people can transmit the disease when they donate blood or undergoing surgery. It is unclear whether there is a latent pool of people who have received contaminated blood transfusions and are therefore at risk of later developing vCJD. However, new referral criteria for blood donors associated with vCJD related to further reduce the risk of vCJD transmission by blood transfusion, which is already very low outside France and the UK. Although no cases have been reported vCJD originating in North America, BSE in some North American cattle (4 in the US and 19 in Canada) was reported. Symptoms and complaints About 70% of patients with CJD present with memory loss and confusion states what develops ultimately in all patients; 15-20% show incoordination and ataxia, which often arise early in the disease. An induced by noise or other sensory stimuli myoclonus (so-called. Startle myoclonus) often occurs in middle to late stages of the disease. People with vCJD tend to show psychiatric symptoms (eg. As anxiety, depression) rather than memory loss. Later symptoms are similar in both forms. Dementia, ataxia, and myoclonus are indeed the most characteristic, but there may be other neurological disorders occur (eg. As hallucinations, seizures, neuropathy, various movement disorders). Eye disorders (eg. As visual field defects, diplopia, blurred vision or flicker, visual agnosia) are common in sCJD. Diffusion-weighted MRI diagnosis Liquormarker exclusion of other diseases CJD should be considered in rapidly progressive dementia in the elderly, especially if it is accompanied by myoclonus or ataxia. However, other diseases can mimic CJD and must be considered; they include CNS vasculitis Rapide progressive Alzheimer’s disease Hashimoto encephalopathy (an autoimmune encephalopathy, which is characterized by high thyroid antibody levels and so on corticosteroids responding) intravascular lymphoma (a rare lymphoma) encephalitis that affects the limbic system, the brain stem and cerebellum , Dementia with Lewy bodies intoxication with lithium or bismuth. It consists of V. a. CJD in younger symptomatic patients if they have consumed in the United Kingdom or other countries at risk prion contaminated beef (vCJD), or a family history of CJD have (familial CJD). Rarely has a sCJD developed in young patients; in such patients but other diseases must be excluded. The diagnosis can be difficult. The best non-invasive diagnostic test for CJD is Diffusion-weighted MRI can thereby developing flick like hyperintense areas (bright areas) are detected in the cortical ribbon, which strongly support a CJD. The proteins 14-3-3, brain-specific enolase and tau are usually increased in the cerebrospinal fluid, but they are not specific for CJD. A relatively new CSF test, called RT-QUIC (real-time quaking-induced conversion), amplifies and detects only minimal amounts of prion activity (conformational change of PrPCin PrPSc) in the cerebrospinal fluid; This test will be more accurate than previous CSF tests. A similar test can be seen by the identification of prions in the urine for a reliable detection of vCJD. EEG is carried out. The results are positive in about 70% of patients with CJD; The EEG has characteristic periodic sharp waves; However, this pattern typically occurs late in the disease process and may be temporary. A brain biopsy is usually not necessary. Forecast Death usually occurs after 6-12 months, often due to pneumonia. Life expectancy at vCJD is longer (average 1.5 years). Therapy Supportive treatment CJD can not be treated. The treatment is symptomatic. Prevention Since there is no effective treatment, prevention in the transferable CJD is essential. Everyone who deals with body fluids and tissue of patients with suspected or secured CJD must wear gloves and avoid exposure of the mucous membranes. Contaminated skin can be disinfected by a 5- to 10-minute application of 4% sodium hydroxide, followed by extensive washing with water. The steam autoclaving of the materials at 132 ° C for 1 h or immersion in sodium hydroxide 1 N (normal) or 10% sodium hypochlorite for 1 hour is recommended. The standard sterilization methods (eg. B. Formal Indes infection) are ineffective. The US Department of Agriculture (USDA) is currently conducting a monitoring of BSE in 2000-5000 cattle per month. In 2004, a positive BSE case in the US has meant that the tests were expanded to an average of 1,000 cattle a day later, the tests were, however, at 40,000 per year reduced (0.1% of cattle slaughtered).

Health Life Media Team

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