Human spongiform encephalopathie transmission

Brown P. Transmissible human spongiform encephalopathy (infectious cerebral amyloidosis) Creutzfeldt-Jakob disease, Gerstman-Straussler-Scheinker syndrome and Kuru. Caine DB, editor. Neurodegenerative Diseases. Philadelphia W.B. Saunders, 1994 839-76. 

The transmissible spongiform encephalopathies, or prion diseases, are fatal neurodegenerative diseases characterized by spongiform changes, astrocytic gliomas, and neuronal loss resulting from the deposition of insoluble protein aggregates in neural cells. They include Creutzfeldt-Jakob disease in humans, scrapie in... 

Minghetti,L., Greco,A., Cardone, F., Puopolo,M.,Ladogana, A. and Almonti, S. Increased brain synthesis of prostaglandin E2 and F2-isoprostane in human and experimental transmissible spongiform encephalopathies.. Neuropathol. Exp. Neurol. 59 866-871, 2000. 

Pocchiari, M., Puopolo, M., Croes, E. A. et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 127 2348-2359,2004. 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

Creutzfeldt-Jacob disease—Human form of transmissible spongiform encephalopathy (TSE), a brain disease in which nerve cells of the brain are destroyed. Seen under a microscope, the brain tissue of people with TSE resembles a sponge. 

Transmissible spongiform encephalopathies (TSEs)—Brain diseases transmitted from one animal to another. Under a microscope, the brain tissue of animals and people with TSEs resembles a sponge. TSEs include variant Creutzfeldt-Jacob disease (vCJD) in humans, scrapie in sheep and goats, and bovine spongiform encephalopathy (BSE) in cows (mad cow disease). These diseases are spread by consumption of brain tissue and are thought to be caused by prions, a kind of protein. 

Because they are derived from cattle, there is a concern that gelatins might be vehicles for the transmission of the prion agent responsible for bovine spongiform encephalopathy (BSE) in cattle and variant Creutzfeldt-Jakob disease (vGD) in humans. There is at present no evidence that these products have contributed to the transmission of BSE or vCJD. However, the incubation period may be up to several years, and due prudence is warranted when such products are used. 

WHO (1997), Medicinal and other products and human and animal transmissible spongiform encephalopathies Memorandum from a WHO meeting, Bull. World Health Org., 75(6), 505-513. 

The inescapable variability of the source material and potential threat to safety due to unrecognized infectious agents [transmission ofAIDS by blood products and the undefined potential for either human or bovine source materials to spread Creutzfeld-Jakob disease (CJD)/ transmissible or bovine spongiform encephalopathy (TSE, BSE)]... 

In human populations, exposure to the BSE agent (probably in contaminated bovine-based food products) has been strongly linked to the 1996 appearance of a new transmissible spongiform encephalopathy of humans called variant Creutzfeldt-Jakob Disease (vCJD). 

A new human transmissible spongiform encephalopathy, variant CJD or vCJD, is identified and distinguished from classic CJD. The UK bans the use of mammalian MBM in feed for all farm animals. The UK introduces slaughter scheme to keep cattle older than 30 months out of food and feed chains. Cattle passports are made mandatory for all cattle bom beginning July 1, 1996. The United States FDA imposes a ban on feeding MBM protein to mminants (36). 

A few cases of Creutzfeldt-Jakob disease have been transmitted by corneal transplantation, dural transplantation, surgical equipment, and human growth hormone extracted from human pituitary glands (195). This has of course raised concern that Creutzfeldt-Jakob disease might prove to be transmissible by blood and blood products (196). The concern has been further accentuated by recent fears of transmission bovine spongiform encephalopathy through food consumption by humans. Some manufacturers of plasma products have reacted by recalling products from the market (197). 

Belay, E. (1999) Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol, 53,283-314. 

Prions (small proteinaceous infectious particles) are a unique class of infectious agent causing spongiform encephalopathies such as bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans. There is considerable concern about the transmission of these agents from infected animals or patients. Risk of infec-tivity is highest in brain, spinal cord and eye tissues. There are still many unknown factors regarding de-... 

Certain brain diseases of humans and animals known as proteinopathies are associated with the accumulation of misfolded proteins both inside and around neurons [1], Alzheimer s disease in humans is by far the most common member of this group. The prion diseases, exemplified by Creutzfeldt-Jakob disease (CJD) in man, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE) in cattle, constitute a peculiar sub-group within proteinopathies by being experimentally transmissible [2], Because of this feature, together with the sponge-like appearance of vacuoles in affected brain areas, prion diseases are also known as Transmissible Spongiform Encephalopathies (TSEs). 

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