Human spongiform encephalopathie

Amouyel P, Vidal O, Launay JM, La-pianche JL. The apolipoprotein E alleles as major susceptibility factors for Creutz-feldt-Jakob disease. The French Research Group on Epidemiology of Human Spongiform Encephalopathies. Lancet 1994 344[8933] 1315—1318. 

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. 

Brown P, Gibbs CJ Jr, Rodgers-Johnson P, Asher DM, Sulima MP, Bacote A, Goldfarb LG, Gajdusek DC (1994) Human spongiform encephalopathy the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 35 513-529... 

Riek R, Wider G, Billeter M, Homemann S, Glockshuber R, Wiithrich K (1998) Prion protein NMR structure and familial human spongiform encephalopathies. Proc Natl Acad Sci USA 95 11667... 

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... 

If the source of the infechon persists, after onset, then the incidence of new cases is maintained at a level which is commensurate with the infechvity of the pathogen and the ffequeney of exposure of individuals. In this manner, if cases of the variant Creutzfeldt-Jacob disease (vCJD), first recognized in the mid-1990s, relates to human exposure to bovine spongiform encephalopathy-infected beef in the early 1980s, then... 

Western blot A method to detect protein in a given sample of tissue homogenate or extract. It uses gel electrophoresis to separate denatured proteins by mass. Some diagnostic applications for the Western blot include Lyme disease, bovine spongiform encephalopathy, and human immunodeficiency virus (HIV) (it is considered the gold standard for HIV diagnostic testing). 

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... 

Fig. 3. Classification of human prion diseases. Sporadic the transformation from PrPc (circle) to PrPSc (square) occurs without apparent cause. Familial a point mutation ( ) is thought to facilitate the transformation. Infectious the transformation arises via PrPSc which acts as a template. The kinetic equations are defined by Eigen (1996). The infectious form includes kuru, iatrogenic CJD (iCJD), variant CJD (vCJD first reported in 1996), bovine spongiform encephalopathy (BSE first reported in 1985), and scrapie. In the nucleation-dependent model, monomeric PrPc and PrPSc are in chemical equilibrium.

Bovine spongiform encephalopathy (BSE or mad cow disease) is a progressive neurological degenerative disease in cattle. It is caused by a mutated protein called a prion. BSE was first reported in the United Kingdom in 1986. Creutzfeldt-Jakob disease (CJD) is a rare disease that occurs in humans. Evidence to date indicates it is possible for humans to acquire CJD after consuming BSE-contaminated cattle products. 

With the background of the mad cow crisis in Europe, questions relating to the prion diseases have attracted intensive interest. It is now widely accepted that prion diseases, such as Creutzfeldt-Jakob disease (CJd) in humans and bovine spongiform encephalopathy (BSE) are caused by a conformational change of the prion protein (PrP) from a normally folded cellular form, PrP ", to an alternate, aggregation-prone, pathogenic scrapie form,... 

Two human forms of spongiform encephalopathies are kura and Creutzfeldt-Jacob disease (CJD). 

Besides the direct and indirect risks associated with the consumption of agricultural products by humans in general, several risks are specific to the consumption of animal produce from modem agriculture. These have received considerable public attention in the past, i.e. antibiotic and hormone residuals, or just recently, such as Bovine Spongiform Encephalopathy (BSE). 

Scientific procedures for risk assessment include assessment of risk for human health as well as risk for the environment. A substantial part of the EU risk assessment work was in 1997 delegated to the DG SANCO, in relation to the scandal surrounding BSE (bovine spongiform encephalopathy or mad cow disease ). Risk assessment work not under DG SANCO includes pharmaceuticals, working environment, and health effects caused by lifestyle factors such as diet, smoking, and alcohol consumption (EU 2006f). 

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. 

European Union (2004), Note for guidance on minimising the risk of transmitting animal spongiform encephalopathy agents via human and veterinary medicinal products (EMEA/410/01 Rev. 2—October 2003) adopted by the Committee for Proprietary Medicinal Products (CPMP) and by the Committee for Veterinary Medicinal Products (CVMP), Off. J. Europ. Union, C24/26-C24/19. 

There is a perceived need to develop effective vaccines against bovine spongiform encephalopathy (BSE) and the human form, new variant Creutzfeldt-Jacob (nvCJ) disease. Organ transplant vaccines would obviously be beneficial and tetanus toxoid has been shown to lower cholesterol in animals. Tumor-specific antigens have also been explored and heat stress proteins have been evaluated as adjuvants in this case. 

Excipients of human or animal origin should be identified. The genus, species, country of origin, source (e.g., pancreas), and manufacturer or supplier should be clearly indicated. Furthermore, for excipients derived from ruminant materials, the application should state whether the materials are from bovine spongiform encephalopathy countries as defined by the U.S. Department of Agriculture (9 CFR 94.11). 

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