Publications
| Title | Abstract | Year(sorted ascending) Filter | PMID Filter |
|---|
| bovine spongiform encephalopathy and variant creutzfeldt-jakob disease: how safe is eating beef? | cases of bovine spongiform encephalopathy (bse, mad cow disease) have been found in north american cattle. its human counterpart, called variant creutzfeldt-jakob disease (variant cjd), is rare but seems to be linked to eating diseased beef. many questions remain about these diseases, such as why young people seem at greater risk of variant cjd. also, are some people more genetically at risk for acquiring variant cjd than others? | 2005 | 15825799 |
| [bovine spongiform encephalopathy]. | the identification of variant creutzfeldt-jakob disease (vcjd) in human strongly reinforced the perception of risks associated with the infectious agent involved in bovine spongiform encephalopathy (bse). the development of rapid tests for the diagnosis of bse by the detection of the abnormal prion protein allowed a huge increase in surveillance of the cattle disease. this first revealed a higher prevalence of the infection than previously believed. however, food safety measures, mainly based on ... | 2005 | 15850957 |
| prions and the blood and immune systems. | prion diseases take a number of forms in animals and humans. they are caused by conformational change in widely expressed prion protein leading to the formation of intracellular aggregates. although the main focus of disease is the central nervous system, it is known that involvement of the immune system occurs in peripherally transmitted disease in particular. animal experiments suggest that in some prion diseases follicular dendritic cells in the germinal centers are a major site of initial ac ... | 2005 | 15820951 |
| protein conformation significantly influences immune responses to prion protein. | in prion diseases, such as variant creutzfeldt-jakob disease normal cellular prion protein (prpc), a largely alpha-helical structure is converted to an abnormal conformational isoform (prpsc) that shows an increase in beta-sheet content. similarly, the recombinant form of prpc (ralpha-prp) can be converted to a conformation dominated by beta-sheet (rbeta-prp) by reduction and mild acidification in vitro, a process that may mimic in vivo conversion following prpc internalization during recycling. ... | 2005 | 15749856 |
| ultrastructural pathology of prion diseases revisited: brain biopsy studies. | we report here a detailed ultrastructural comparison of brain biopsies from 13 cases of creutzfeldt-jakob disease (cjd) and from one case of fatal familial insomnia (ffi). the latter disease has not heretofore benefited from ultrastructural study. in particular, we searched for tubulovesicular structures (tvs), 35-nm particles regarded as the only disease-specific structures at the level of thin-section electron microscopy. our material consisted of brain biopsies obtained by open surgery from o ... | 2005 | 15634235 |
| care management of creutzfeldt-jakob disease within the united kingdom. | the purpose of this article is to review the management of health and social care provision for creutzfeldt-jakob disease patients within the united kingdom. the link between the epidemic of bovine spongiform encephalopathy (bse) in cattle and the subsequent emergence of variant creutzfeldt-jakob disease (vcjd) in humans during the mid 1990s created new mechanisms for the organization of health and social care for creutzfeldt-jakob disease patients. this article draws on the experiences of two n ... | 2005 | 15720480 |
| type 1 and type 2 human prpsc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant creutzfeldt-jakob disease. | in creutzfeldt-jakob disease (cjd), the type (type 1 or 2) of abnormal isoform of the prion protein (prp(sc)) in the brain and the genotype at codon 129 of the prp gene are major determinants of clinicopathological phenotype. little is known about the difference in biochemical properties between the two types of prp(sc), except for the different proteinase k cleavage sites. to investigate the size of aggregates formed by prp(sc) types 1 and 2, brain homogenates from various cases of cjd with the ... | 2005 | 15604452 |
| biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type. | the phenotype of human prion diseases is influenced by the prion protein (prp) genotype as determined by the methionine (m)/valine (v) polymorphism at codon 129, the scrapie prp (prpsc) type and the etiology. to gain further insight into the mechanisms of phenotype determination, we compared two-dimensional immunoblot profiles of detergent insoluble and proteinase k-resistant prp species in a type of sporadic creutzfeldt-jakob disease (scjdmm2), variant cjd (vcjd) and sporadic fatal insomnia (sf ... | 2005 | 15606903 |
| new ratios for the detection and classification of cjd in multisequence mri of the brain. | we present a method for the analysis of deep grey brain nuclei for accurate detection of human spongiform encephalopathy in multisequence mri of the brain. we employ t1, t2 and flair-t2 mr sequences for the detection of intensity deviations in the internal nuclei. the mr data are registered to a probabilistic atlas and normalised in intensity prior to the segmentation of hyperintensities using a foveal model. anatomical data from a segmented atlas are employed to refine the registration and remo ... | 2005 | 16685996 |
| dementia associated with infectious diseases. | at the turn of the last century, infectious diseases represented an important cause of health morbidity and behavioral changes. neurosyphilis, for example, was relatively common at the time and often led to the development of cognitive impairment and dementia. with the advent of effective antibiotic treatment, the association between infectious diseases and dementia became increasingly less frequent, although a resurgence of interest in this area has taken place during the past 15 years with the ... | 2005 | 16240484 |
| molecular evolution of the sheep prion protein gene. | transmissible spongiform encephalopathies (tses) are infectious, fatal neurodegenerative diseases characterized by aggregates of modified forms of the prion protein (prp) in the central nervous system. well known examples include variant creutzfeldt-jakob disease (vcjd) in humans, bse in cattle, chronic wasting disease in deer and scrapie in sheep and goats. in humans, sheep and deer, disease susceptibility is determined by host genotype at the prion protein gene (prnp). here i examine the molec ... | 2005 | 16243700 |
| uncertainty due to model choice in variant creutzfeldt-jakob disease projections. | for some statistical applications, uncertainty due to unverifiable assumptions can be much greater than that arising from the random variability that is quantified by conventional confidence intervals. the case of projecting possible maximum numbers of eventual deaths due to variant creutzfeldt-jakob disease in the united kingdom provides an extreme example of this phenomenon. the need for parametric extrapolation of the incubation distribution, along with non-identifiability of the number of in ... | 2005 | 15515116 |
| variant creutzfeldt-jakob disease transmission by plasma products: assessing and communicating risk in an era of scientific uncertainty. | a substantial body of animal data indicates that transmissible spongiform encephalopathies (tses) are transmitted through blood. these data have been augmented in the past year by reports that two recipients of red cells from donors with variant creutzfeldt-jakob disease (vcjd) in the united kingdom have acquired this infection. most of the blood donations collected in countries affected by bovine spongiform encephalopathy (bse) and vcjd also contribute plasma to fractionation pools. thus, a num ... | 2005 | 16262750 |
| prion biology in transfusion medicine: implications for lab testing. | although eerily silent for many years after the recognition of scrapie in 1759, tses remained present within the genome of some mammals. not since the mid-1950s when dr. carleton gadjusek visited the fore indians of new guinea to study kuru, however, has there been a more frenetic interest by governmental investigators. certainly, the u.k. experience has heralded a renewed interest in tses due to the notoriety associated with younger subjects succumbing to a variant cjd traced to the ingestion o ... | 2005 | 16265819 |
| ethical considerations in presymptomatic testing for variant cjd. | variant creutzfeldt-jakob disease (vcjd) is a fatal, transmissible, neurodegenerative disorder for which there is currently no effective treatment. vcjd arose from the zoonotic spread of bovine spongiform encephalopathy. there is now compelling evidence for human to human transmission through blood transfusions from presymptomatic carriers and experts are warning that the real epidemic may be yet to come. imperatives exist for the development of reliable, non-invasive presymptomatic diagnostic t ... | 2005 | 16269554 |
| risk assessment of variant creutzfeldt-jakob disease in cosmetics. | 2005 | 16276156 | |
| transmission barriers for bovine, ovine, and human prions in transgenic mice. | transgenic (tg) mice expressing full-length bovine prion protein (boprp) serially propagate bovine spongiform encephalopathy (bse) prions without posing a transmission barrier. these mice also posed no transmission barrier for suffolk sheep scrapie prions, suggesting that cattle may be highly susceptible to some sheep scrapie strains. tg(boprp) mice were also found to be susceptible to prions from humans with variant creutzfeldt-jakob disease (cjd); on second passage in tg(boprp) mice, the incub ... | 2005 | 15827140 |
| [prion diseases as zoonosis]. | prion diseases such as bovine spongiform encephalopathy (bse) have been recognized as zoonosis since the existence of variant creutzfeldt-jakob disease (vcjd) was reported in 1996. bse became a serious social problem even in japan after the first bse case was found in 2001. the incidence of bse in eu and uk appears declining, and the vcjd incidence also shows a tendency to decrease. on the contrary, fears for the spread of bse became actual problems: bse occurrence outside of eu, transmission of ... | 2005 | 16308529 |
| survey of zoonoses recorded in scotland between 1993 and 2002. | all the human and animal laboratory reports of zoonoses sent to health protection scotland between 1993 and 2002 were identified. there were 24,946 reports from veterinary laboratories, and 94,718 (20 per cent) of the 468,214 reports from medical laboratories were considered to be zoonotic. the most common reports of zoonoses from people were campylobacter, salmonella, cryptosporidium and giardia species and escherichia coli o157. the most common reports of zoonoses from animals were salmonella, ... | 2005 | 16311383 |
| risks of transmission of variant creutzfeldt-jakob disease by blood transfusion. | variant creutzfeldt-jakob disease (vcjd) was first identified in 1996 in the uk, and results from human exposure to the bovine spongiform encephalopathy (bse) agent. vcjd has subsequently been identified in 10 additional countries, and numbers continue to increase in the uk. unlike other human prion diseases, infectivity and the disease-associated form of the prion protein are readily detected in lymphoid tissues in vcjd. in experimental bse infection in a sheep model, infectivity has been trans ... | 2005 | 16416391 |
| prptse distribution in a primate model of variant, sporadic, and iatrogenic creutzfeldt-jakob disease. | human prion diseases, such as creutzfeldt-jakob disease (cjd), are neurodegenerative and fatal. sporadic cjd (scjd) can be transmitted between humans through medical procedures involving highly infected organs, such as the central nervous system. however, in variant cjd (vcjd), which is due to human contamination with the bovine spongiform encephalopathy (bse) agent, lymphoreticular tissue also harbors the transmissible spongiform encephalopathy-associated prion protein (prp(tse)), which poses a ... | 2005 | 16254368 |
| projections of the future course of the primary vcjd epidemic in the uk: inclusion of subclinical infection and the possibility of wider genetic susceptibility. | the incidence of variant creutzfeldt-jakob disease (vcjd) in the united kingdom appears to be in decline, with only four deaths reported this year (to 6 september 2004). however, results of a survey of lymphoreticular tissues have suggested a substantially higher prevalence of vcjd than expected from the clinical data alone. there are two plausible explanations for this discrepancy: first, a proportion of those infected will not develop clinical disease (subclinical infection); and second, the g ... | 2005 | 16849160 |
| bse safety standards: an evaluation of public health policies of japan, europe, and usa. | since the advent of bovine spongiform encephalopathy (bse) in the united kingdom in 1986, new bse cases have recently become rare. however, in japan and the united states, positive cases have started to be seen recently. the rise in bse cases paved the way for the human form of this disease, the variant creutzfeldt-jakob disease (vcjd). the observed trends in the uk may be attributed to effective implementation of public health policies coupled with increased vigilance through advancement in sci ... | 2005 | 21432135 |
| [prion disease as infectious disease transmissible from animals to human]. | prion diseases such as bovine spongiform encephalopathy (bse) have been recognized as zoonosis since the existence of variant creutzfeldt-jakob disease (vcjd) was reported in 1996. after then, bse became a serious social problem all over the world. the incidence of bse in eu and uk appears declining, and the vcjd incidence also shows a tendency to decrease. on the contrary, fears for the spread of bse became actual problems: bse occurrence outside of eu, introduction of bse to other ruminants, a ... | 2005 | 16363697 |
| community blood supply model: development of a new model to assess the safety, sufficiency, and cost of the blood supply. | through a combination of predonation donor screening and donated unit testing, the blood supply is safer than ever. however, as a result of increasingly stringent screening measures, one of the greatest threats may be an insufficient supply. the balance between safety and adequacy of the blood supply has not received enough attention. | 2005 | 16160212 |
| australian sporadic cjd analysis supports endogenous determinants of molecular-clinical profiles. | to define the protease-resistant prion protein (prpres) types and associated clinical profiles in australian patients with sporadic creutzfeldt-jakob disease (cjd) to allow comparison with those reported from other continents and concomitantly reaffirm absence of variant cjd (vcjd). | 2005 | 16009895 |
| cerebrospinal fluid biomarkers in creutzfeldt-jakob disease. | creutzfeldt-jakob disease (cjd) is a rare neurodegenerative disorder. since the emergence of variant cjd (vcjd) vigilance concerning the disease's incidence has increased and the interest in accurate in vivo diagnosis has augmented. so far, a large number of biomarkers has been investigated as aid in the differential diagnosis of sporadic creutzfeldt-jakob disease (scjd) and vcjd. these include, among others, neuron-specific enolase (nse), microtubuli associated protein tau, s-100beta, amyloid-b ... | 2005 | 16023527 |
| variant creutzfeldt-jakob disease: a cause for concern. review of the evidence for risk of transmission through abdominal lymphoreticular tissue surgery. | concern has long existed regarding the possible iatrogenic spread of variant creutzfeldt-jakob disease (v-cjd) through surgery. this had been fueled by recent reports of bovine spongiform encephalopathy in u.s. cattle and the first probable case of blood transmission of v-cjd in the uk. | 2005 | 15868249 |
| [variant creutzfeldt-jakob disease in france: estimating the number of cases related to travel to the united kingdom between 1980 and 1995]. | the outbreak of variant creutzfeldt-jakob disease (vcjd) cases rose serious concerns about secondary transmission of the disease, particularly through blood transfusion. protective measures leading to the exclusion of potentially infectious blood donors were settled: in france, donors who had stayed more than one year in the uk were excluded. in this work, which was part of a larger study aiming to estimate the french epidemic of vcjd, the number of vcjd cases who were infected during a trip to ... | 2005 | 15888987 |
| cerebroventricular infusion of pentosan polysulphate in human variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (cjd) is a transmissible spongiform encephalopathy believed to be caused by the bovine spongiform encephalopathy agent, an abnormal isoform of the prion protein (prp(sc)). at present there is no specific or effective treatment available for any form of cjd. pentosan polysulphate (pps), a large polyglycoside molecule with weak heparin-like activity, has been shown to prolong the incubation period of the intracerebral infection when administered to the cerebral ve ... | 2005 | 15907546 |
| tse clearance during plasma products separation process by gradiflow(tm). | recent experimental evidence from rodent models suggests a potential risk for transmissible spongiform encephalopathy (tse) transmission by blood. the emergence of a new variant creutzfeldt-jakob disease (vcjd) has raised increased concerns about the safety of blood components and plasma products derived from vcjd-infected donors. recent risk-minimisation strategies have included a ban on the use of uk-sourced plasma for the preparation of licensed blood products and leukodepletion of blood dona ... | 2005 | 15939286 |
| novel antibody-lectin enzyme-linked immunosorbent assay that distinguishes prion proteins in sporadic and variant cases of creutzfeldt-jakob disease. | we used different anti-prion protein (anti-prp) monoclonal antibodies to capture either full-length or truncated prp species and then used biotinylated lectin to compare the nature of the glycans on bound prp species present in control, sporadic creutzfeldt-jakob disease (scjd), or variant cjd (vcjd) brains. when full-length prp species in these three groups were compared, no significant difference in the binding of concanavalin a or aleuria aurantia lectin was detected. however, the binding of ... | 2005 | 15750071 |
| the public health impact of prion diseases. | several prion disease-related human health risks from an exogenous source can be identified in the united states, including the iatrogenic transmission of creutzfeldt-jakob disease (cjd), the possible occurrence of variant cjd (vcjd), and potential zoonotic transmission of chronic wasting disease (cwd). although cross-species transmission of prion diseases seems to be limited by an apparent "species barrier," the occurrence of bovine spongiform encephalopathy (bse) and its transmission to humans ... | 2005 | 15760286 |
| annulling a dangerous liaison: vaccination strategies against aids and tuberculosis. | human immunodeficiency virus (hiv) and mycobacterium tuberculosis annually cause 3 million and 2 million deaths, respectively. last year, 600,000 individuals, doubly infected with hiv and m. tuberculosis, died. since world war i, approximately 150 million people have succumbed to these two infections--more total deaths than in all wars in the last 2,000 years. although the perceived threats of new infections such as sars, new variant creutzfeldt-jakob disease and anthrax are real, these outbreak ... | 2005 | 15812488 |
| commentary: the risk of variant creutzfeldt-jakob disease: reassurance and uncertainty. | 2005 | 15649957 | |
| risk of variant creutzfeldt-jakob disease in france. | france has the second highest number of variant creutzfeldt-jakob disease (vcjd) cases worldwide. imports of bovine carcasses from the uk probably constituted the main source of exposure of the french population to the bovine spongiform encephalopathy (bse) agent. meat products consumed whilst visiting the uk have also been considered as a possible source of exposure. | 2005 | 15649960 |
| blood transfusion and autologous donation: a survey of post-surgical patients, interest group members and the public. | before planned surgery, patients may choose autologous donation in order to avoid the small, but potential, risks of receiving an allogeneic blood transfusion. this study examined the perceived risks of allogeneic blood transfusions, preferences and willingness to pay for autologous donation and the desired role in the decision-making process in three populations: post-surgical patients, special interest group members and the general public. quantitative and qualitative data were collected from ... | 2005 | 15713125 |
| the neuropsychology of variant cjd: a comparative study with inherited and sporadic forms of prion disease. | to assess cognitive function in variant creutzfeldt-jakob disease (vcjd). we describe the neuropsychological profiles of 10 cases and compare these data with cross sectional data obtained from patients with histologically confirmed sporadic cjd and cases with inherited prion disease with confirmed mutations in the prion protein gene. | 2005 | 15716521 |
| rational targeting for prion therapeutics. | prions--pathogens that are lethal to humans and other animals--are thought to be conformational isomers of the cellular prion protein. their unique biology, and the potential for a wider pathobiological significance of prion-like mechanisms, has motivated much research into understanding prion neurodegeneration. moreover, concerns that extensive dietary exposure to bovine spongiform encephalopathy (bse) prions might have infected many individuals--who might eventually develop its human counterpa ... | 2005 | 15611724 |
| risk of oral infection with bovine spongiform encephalopathy agent in primates. | the uncertain extent of human exposure to bovine spongiform encephalopathy (bse)--which can lead to variant creutzfeldt-jakob disease (vcjd)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. we therefore investigated oral transmission of bse to non-human primates. we gave two macaques a 5 g oral dose of brain homogenate from a bse-infected cow. one macaque developed vcjd-like neurological dis ... | 2005 | 15733719 |
| phenotype of disease-associated prp accumulation in the brain of bovine spongiform encephalopathy experimentally infected sheep. | in view of the established link between bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease and of the susceptibility of sheep to experimental bse, the detection of potential cases of naturally occurring bse in sheep has become of great importance. in this study, the immunohistochemical (ihc) phenotype of disease-associated prion protein (prp(d)) accumulation has been determined in the brain of 64 sheep, of various breeds and prp genotypes, that had developed neurologica ... | 2005 | 15722546 |
| human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993-2002. | the objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries. | 2006 | 17096829 |
| differentiation of scjd and vcjd forms by automated analysis of basal ganglia intensity distribution in multisequence mri of the brain--definition and evaluation of new mri-based ratios. | we present a method for the analysis of basal ganglia (including the thalamus) for accurate detection of human spongiform encephalopathy in multisequence magnetic resonance imaging (mri) of the brain. one common feature of most forms of prion protein diseases is the appearance of hyperintensities in the deep grey matter area of the brain in t2-weighted magnetic resonance (mr) images. we employ t1, t2, and flair-t2 mr sequences for the detection of intensity deviations in the internal nuclei. fir ... | 2006 | 16894998 |
| quantitative evaluation of prion inactivation comparing steam sterilization and chemical sterilants: proposed method for test standardization. | prions are notoriously resistant to inactivation. to prevent accidental transmission of variant creutzfeldt-jakob disease (vcjd), various decontamination procedures have been adopted for re-usable medical devices by the authorities of countries at risk. as the vcjd agent in humans has a wide tissue distribution, practical methods of prion decontamination urgently need to be standardized, as do other sterilization and disinfection procedures (european committee for standardization). this article ... | 2006 | 16895739 |
| variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 2. | up until february 2006, variant cjd (vcjd), the human disease associated with transmission of bse from cattle, has been confirmed in 160 patients resident in the uk and 28 elsewhere, some of whom have never visited the uk. cases have been reported in france (16 cases), ireland (3), usa (2), canada, italy, japan, the netherlands, portugal, saudi arabia and spain (1 each). the presumed main period of hazard for ingestion of the bse agent in bovine products in the uk is 1984-89, or perhaps up to 19 ... | 2006 | 16823692 |
| cerebral amyloidoses: molecular pathways and therapeutic challenges. | alzheimer disease (ad) and creutzfeldt-jakob disease (cjd) are sporadic and genetic neurodegenerative conditions characterized by brain accumulation and deposition of protein aggregates. in ad, the key pathogenic event is linked to the formation of a 4-kda amyloid beta (abeta) peptide, generated by sequential cleavages of the amyloid precursor protein (app). in cjd and other prion diseases, the process is initiated by conformational changes of the cellular prion protein, or prp(c), into a beta-s ... | 2006 | 16842201 |
| trends in scientific activity addressing transmissible spongiform encephalopathies: a bibliometric study covering the period 1973-2002. | the purpose of this study is to analyse the trends in scientific research on transmissible spongiform encephalopathies by applying bibliometric tools to the scientific literature published between 1973 and 2002. | 2006 | 17026743 |
| isolation from cattle of a prion strain distinct from that causing bovine spongiform encephalopathy. | to date, bovine spongiform encephalopathy (bse) and its human counterpart, variant creutzfeldt-jakob disease, have been associated with a single prion strain. this strain is characterised by a unique and remarkably stable biochemical profile of abnormal protease-resistant prion protein (prp(res)) isolated from brains of affected animals or humans. however, alternate prp(res) signatures in cattle have recently been discovered through large-scale screening. to test whether these also represent sep ... | 2006 | 17054396 |
| insights into the management of emerging infections: regulating variant creutzfeldt-jakob disease transfusion risk in the uk and the us. | variant creutzfeldt-jakob disease (vcjd) is a human prion disease caused by infection with the agent of bovine spongiform encephalopathy. after the recognition of vcjd in the uk in 1996, many nations implemented policies intended to reduce the hypothetical risk of transfusion transmission of vcjd. this was despite the fact that no cases of transfusion transmission had yet been identified. in december 2003, however, the first case of vcjd in a recipient of blood from a vcjd-infected donor was ann ... | 2006 | 17076547 |
| cjd: update for dental staff. | it is almost a decade since the recognition of the emergence of a new infectious disease termed variant creutzfeldt-jakob disease (vcjd) caused by prions (prptse), abnormal variants of a normal human cell surface protein (prp). this disease has a number of similarities to other forms of cjd--lethal disorders characterized by a prolonged incubation period, and progressive mental deterioration. in relation to oral tissues, prptse have been found in neural, gingival, pulpal, lingual, lymphoreticula ... | 2006 | 17087448 |
| [creutzfeldt-jakob disease--the past or the future]. | some recent views on ethiopathogenesis and epidemiology of four main forms of cjd, based on up to-day experiences and expectations for the future, are presented. the sporadic form of the disease (scjd) displays a stable morbidity--ca. 1 case/1 million population yearly. the reasons of its so constant appearance remain still unknown. the familial forms of cjd (fcjd) depending upon more than 40 mutations in prnp gene known today are inherited as autosomal dominant train. the clinical and neuropath ... | 2006 | 16909780 |
| methods to minimize the risks of creutzfeldt-jakob disease transmission by surgical procedures: where to set the standard? | new prion-related disorders have emerged over the past 20 years, of which the most notable in the human context is variant creutzfeldt-jakob disease (cjd). this disorder is a challenge to medical and public health professionals seeking early detection and diagnosis, provision of therapy, and support for persons affected and a better understanding of transmission risks. the risk of iatrogenic transmission of the disease remains a significant threat, given the well documented cases of cjd transmis ... | 2006 | 16912952 |
| a novel copper-hydrogen peroxide formulation for prion decontamination. | with the appearance of variant creutzfeldt-jakob disease (cjd) and the detection of infectious prions in the peripheral organs of persons with sporadic cjd, the development of decontamination methods that are compatible with medical equipment has become a major issue. here, we show that a formulation of copper metal ions in combination with hydrogen peroxide dramatically reduces the level of prion protein (prp)(sc) (the scrapie isoform of prp) present in homogenates of samples from prion-infecte ... | 2006 | 16941355 |
| creutzfeldt-jakob disease and blood transfusion: results of the uk transfusion medicine epidemiological review study. | this paper reports the results to 1 march 2006 of an ongoing uk study, the transfusion medicine epidemiological review (tmer), by the national cjd surveillance unit (ncjdsu) and the uk blood services (ukbs) to determine whether there is any evidence that creutzfeldt-jakob disease (cjd), including sporadic cjd (scjd), familial cjd (fcjd), and variant cjd (vcjd) is transmissible via blood transfusion. | 2006 | 16958834 |
| application of an immunocapillary electrophoresis assay to the detection of abnormal prion protein in brain, spleen and blood specimens from patients with variant creutzfeldt-jakob disease. | sensitive and specific detection of abnormal prion protein in blood could provide a diagnostic test or screening assay for animal and human prion diseases. here, the application of an immunocapillary electrophoresis (ice) method developed for sheep scrapie to brain, spleen and blood from patients with creutzfeldt-jakob disease (cjd) is described. the assay involves organic-solvent extraction, a competitive immunoassay using fluorescently labelled synthetic prion protein peptides and polyclonal a ... | 2006 | 16963772 |
| balancing evidence and public opinion in health technology assessments: the case of leukoreduction. | leukoreduction, filtering white blood cells from transfusion blood, effectively avoids leukocyte-related complications of blood transfusion. the technology has proven its relative cost-effectiveness for specific patient populations. with the advent of variant creutzfeldt-jakob disease, a transmittable spongiform encephalopathy caused by mad cow disease (bovine spongiform encephalopathy), the hard hit united kingdom introduced universal leukoreduction for all patients as a precaution for transmis ... | 2006 | 16984672 |
| prion diseases in humans: an update. | the year 2006 marks 20 years from the first identified bovine spongiform encephalitis in cows and 10 years from the first description of variant creutzfeldt-jakob disease in humans. the threatened epidemic in humans now appears unlikely, but psychiatrists need to be aware of recent developments in prion diseases. | 2006 | 17012651 |
| [neuroimaging in the diagnosis of human transmissible spongiform encephalopathies]. | the role of neuroimaging in the diagnosis of the human transmissible spongiform encephalopathies (htse) has been changing from the exclusion of other conditions to the contribution of diagnostic data having incalculable utility, such as basal ganglia and cerebral cortex in different magnetic resonance imaging (mri) sequences. | 2006 | 17013788 |
| [atypical clinicoradiological course in a case panancephalic variant of creutzfeldt-jakob]. | creutzfeldt-jakob disease (cjd) is the most frequent of the human transmissible spongiform encephalopathies. pathogenic mechanisms of cjd are still unknown. sporadic cjd, the most habitual, is clinically characterized by rapidly progressive dementia, myoclonia and ataxia. panencephalic variant cjd, typically from japan, is characterized by extensive involvement of the cerebral white and gray matter. international interest has grown from more than one decade ago in relation to the diagnosis of ne ... | 2006 | 17013790 |
| clinical presentation and pre-mortem diagnosis of variant creutzfeldt-jakob disease associated with blood transfusion: a case report. | concerns have been raised that variant creutzfeldt-jakob disease (vcjd) might be transmissible by blood transfusion. two cases of prion infection in a group of known recipients of transfusion from donors who subsequently developed vcjd were identified post-mortem and reported in 2004. another patient from this at-risk group developed neurological signs and was referred to the national prion clinic. | 2006 | 17161728 |
| is there a real risk of transmitting variant creutzfeldt-jakob disease by donor sperm insemination? | although >99% of cases of creutzfeldt-jakob disease (cjd) are caused by spontaneous or inherited mutations in the prion protein, 'variant' cjd (vcjd) arose from dietary exposure to meat products infected with the bovine spongiform encephalopathy prion. while european and canadian sperm donor candidates are rejected for significant cjd risk factors, american sperm donors are managed like blood donors (excluding all men who spent > or =3 months in the uk during 1980-1996 or > or =5 years in europe ... | 2006 | 17169195 |
| [transfusion risk analysis with regard to vcjd in france]. | the latest updates (february 2004 and february 2005) of the analysis of the risk of transmission of the agent of creutzfeldt-jakob disease (cjd) by blood and blood products in france firstly reported in 2000, were triggered by the two cases of probable transmission of variant cjd (vcjd) by transfusion reported in the uk, and the notification of two french cases of vcjd who had been blood donors on several occasions before clinical onset. even though some figures of the quantitative assumption us ... | 2006 | 17188540 |
| vcjd and blood transfusion in the united kingdom. | a study was set up in the uk in 1997 to examine whether there is any evidence that variant creutzfeldt-jakob disease (vcjd) is transmitted by blood transfusion. to date, the study has identified three probable cases of vcjd transmission by blood transfusion, including two clinical cases and one pre- (or sub-) clinical case. | 2006 | 17188541 |
| [vcjd epidemiology in france]. | variant creutzfeldt-jakob disease (vcjd) is the only form of prion diseases linked to bovine spongiform encephalopathy (bse). the disease was first described in the united-kingdom (uk) and france is the second affected country with 21 cases. clinical, genetic and neuropathological features are the same in both countries. comparison of the total number of cases in france and in the uk, according to dates of onset, shows that, in france, the maximum incidence seems to be five years delayed and tha ... | 2006 | 17188544 |
| creutzfeldt-jakob disease and vision. | this review describes a group of diseases known as the transmissible spongiform encephalopathies (tses), which affect animals and humans. examination of affected brain tissue suggests that these diseases are caused by the acquisition and deposition of prion protein (prp). creutzfeldt-jakob disease (cjd) is the most important form of tse in humans with at least four different varieties of the disease. variant cjd (vcjd), a new form of the disease found in the uk, has several features that differ ... | 2006 | 16430434 |
| prions in skeletal muscles of deer with chronic wasting disease. | the emergence of chronic wasting disease (cwd) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant creutzfeldt jakob disease, have raised concerns about the zoonotic potential of cwd. because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. here bioassays in transgenic mice exp ... | 2006 | 16439622 |
| variant creutzfeldt-jakob disease: risk of transmission by blood transfusion and blood therapies. | in the last decade, a new variant of the human prion disease creutzfeldt-jakob disease (now known as variant cjd or vcjd) was identified and causally linked to dietary exposure to bovine spongiform encephalopathy (bse) during the 1980s and early 1990s. preliminary studies in animal models suggest that prions can be transmitted by blood. based on two recent reports of iatrogenic vcjd transmission by blood transfusion in humans, a department of health-sponsored risk assessment warned that recipien ... | 2006 | 16445812 |
| clinical implications of emerging pathogens in haemophilia: the variant creutzfeldt-jakob disease experience. | the impact of variant creutzfeldt-jakob disease (vcjd) on the clinical practice of haemophilia in the uk is coloured by the haemophilia community's experience of hepatitis c virus and human immunodeficiency virus (hiv) transmission via plasma-derived therapies in the 1980s, when the delay in recognizing and acting on the potential risks cost many patients their lives and left others to manage another chronic disease. this crisis prompted organisations such as the united kingdom haemophilia centr ... | 2006 | 16445813 |
| prions in dentistry--what are they, should we be concerned, and what can we do? | to briefly review the characteristics of prions, the risk of transmission and implications for infection control in dentistry. | 2006 | 16480606 |
| vcjd prion acquires altered virulence through trans-species infection. | variant creutzfeldt-jakob disease (vcjd) appears to be caused by infection with the bovine spongiform encephalopathy (bse) agent. to date, all patients with vcjd are homozygous for methionine at codon 129 of the prp gene. to investigate the relationship between polymorphism at codon 129 and susceptibility to bse or vcjd prions, we performed splenic follicular dendritic cell assay with humanized knock-in mice through peripheral infection. all humanized knock-in mice showed little or no susceptibi ... | 2006 | 16480953 |
| new thoughts on cjd, surgical instruments and disease transmission. | for most people, the threat of widespread transmission of variant creutzfeldt-jakob disease in humans as a result of bovine spongiform encephalopathy has passed into the history books. but concerns about transmission of the disease during certain medical procedures still exist. | 2006 | 16483102 |
| detection and localization of prpsc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) differs from other human prion diseases in that the pathogenic prion protein prp(sc) can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. however, a recent study using a high-sensitivity western blotting technique revealed low levels of prp(sc) in skeletal muscle from a quarter of swiss patients with sporadic cjd (scjd). this posed the question of whether prp(sc) in muscle could also be detec ... | 2006 | 16507908 |
| the first japanese case of variant creutzfeldt-jakob disease showing periodic electroencephalogram. | 2006 | 16530582 | |
| diagnostics for tse agents. | bovine spongiform encephalopathy (bse) is a fatal acquired neuro-degenerative disease in cattle, belonging to the group of transmissible spongiform encephalopathies (tses) or prion diseases. since its first recognition in the u.k. in 1986, bse has raised great public health concerns because the bse agent has been shown to cause variant creutzfeldt jakob disease (vcjd) in humans. with the introduction of mandatory active surveillance programmes in the european union the need to develop rapid test ... | 2006 | 16566455 |
| the neuropathologic phenotype of experimental ovine bse is maintained after blood transfusion. | iatrogenic transmission by blood transfusion has been described in cases of human variant creutzfeldt-jakob disease (vcjd), experimental ovine bovine spongiform encephalopathy (bse), and natural sheep scrapie, demonstrating that blood in these prion diseases is infectious. however, the possible effect of the transfusion, derived from differences in the inoculum (blood) and the route of infection (intravenous), on the pathologic phenotype of the disease in the recipients is not known. this study ... | 2006 | 16569766 |
| what can we learn from the oral intake of prions by sheep? | the central nervous system is the ultimate target of prions, the agents responsible for fatal neurodegenerative diseases known as transmissible spongiform encephalopathies (tses). the neuro-invasive phase and its related clinical signs take place after a long incubation period. during this asymptomatic phase, however, active transport and replication of the infectious agent take place in peripheral sites. the oral infection route has been extensively studied because of its implication in the rec ... | 2006 | 16575798 |
| preparation of soluble infectious samples from scrapie-infected brain: a new tool to study the clearance of transmissible spongiform encephalopathy agents during plasma fractionation. | concern about the safety of blood, blood components, and plasma-derived products with respect to prions has increased since the report of two blood-related infections of variant creutzfeldt-jakob disease in the united kingdom. efforts were directed toward the development of procedures able to remove or inactivate prions from blood components or plasma-derived products with brain fractions of transmissible spongiform encephalopathy (tse)-infected rodents as spiking materials. these spiking materi ... | 2006 | 16584444 |
| unsuccessful intraventricular pentosan polysulphate treatment of variant creutzfeldt-jakob disease. | pentosan polysulphate, delivered by chronic intraventricular infusion, has been proposed as a potential therapy for human prion disease. the first treated patient is still alive several years after treatment started. here we describe in detail a case of variant creutzfeldt-jakob disease in which this treatment was started at a relatively early stage but had no definite clinical benefit. the patient died from disease progression 16 months after diagnosis and 5 months after pentosan polysulphate t ... | 2006 | 16598408 |
| the expanding universe of prion diseases. | prions cause fatal and transmissible neurodegenerative disease. these etiological infectious agents are formed in greater part from a misfolded cell-surface protein called prp(c). several mammalian species are affected by the diseases, and in the case of "mad cow disease" (bse) the agent has a tropism for humans, with negative consequences for agribusiness and public health. unfortunately, the known universe of prion diseases is expanding. at least four novel prion diseases--including human dise ... | 2006 | 16609731 |
| predicting susceptibility and incubation time of human-to-human transmission of vcjd. | identification of possible transmission of variant creutzfeldt-jakob disease (vcjd) via blood transfusion has caused concern over spread of the disease within the human population. we aimed to model iatrogenic spread to enable a comparison of transmission efficiencies of vcjd and bovine spongiform encephalopathy (bse) and an assessment of the effect of the codon-129 polymorphism on human susceptibility. | 2006 | 16632309 |
| pathological prion protein in muscles of hamsters and mice infected with rodent-adapted bse or vcjd. | recently, pathological prion protein (prp(tse)) was detected in muscle from sheep infected with scrapie, the archetype of transmissible spongiform encephalopathies (tses). this finding has highlighted the question of whether mammalian muscle may potentially also provide a reservoir for tse agents related to bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd). here, results are reported from studies in hamsters and mice that provide direct experimental evidence, fo ... | 2006 | 16361438 |
| pathogenesis and prevalence of variant creutzfeldt-jakob disease. | in the late 1980s and early 1990s, there was widespread exposure of the uk population to bovine spongiform encephalopathy (bse)-contaminated food products, which has led to over 150 deaths from variant creutzfeldt-jakob disease (vcjd). although the pathogenesis in humans is not fully understood, data from animal models and, to a lesser extent, patients with vcjd suggest that oral exposure to bse is rapidly followed by accumulation of prp(res) in gut-associated lymphoid tissue, then, after haemat ... | 2006 | 16362983 |
| infection and disease: cause and cure. | much can be learnt about the mechanisms by which micro-organisms cause disease from the ways that they interact with cells and tissues. this issue of the journal of pathology contains articles that address the roles that cell and tissue biology and pathology are playing in the elucidation of these mechanisms. a review of variant creutzfeldt-jakob disease is followed by a discussion of severe acute respiratory syndrome (sars). two articles on human papillomavirus (hpv) infection address the assoc ... | 2006 | 16362991 |
| managing the risk of transmission of variant creutzfeldt jakob disease by blood products. | whereas plasma-derived clotting factor concentrates now have a very good safety record for not being infectious for lipid enveloped viruses, concern has arisen about the possibility that prion diseases might be transmitted by blood products. there is epidemiological evidence that classical sporadic creutzfeld jakob disease (cjd) is not transmitted by blood transfusion. there is now good evidence that the abnormal prion associated with variant cjd can be transmitted by transfusion of fresh blood ... | 2006 | 16371015 |
| risk factors for variant creutzfeldt-jakob disease: a case-control study. | to investigate the potential risk factors for variant creutzfeldt-jakob disease (vcjd) in the united kingdom. | 2006 | 16287153 |
| molecular aspects of disease pathogenesis in the transmissible spongiform encephalopathies. | the transmissible spongiform encephalopathies (tse), or prion diseases, are a group of rare, fatal, and transmissible neurodegenerative diseases of mammals for which there are no known viral or bacterial etiological agents. the bovine form of these diseases, bovine spongiform encephalopathy (bse), has crossed over into humans to cause variant creutzfeldt-jakob disease. as a result, bse and the tse diseases are now considered a significant threat to human health. understanding the basic mechanism ... | 2006 | 16691009 |
| endoplasmic reticulum stress features are prominent in alzheimer disease but not in prion diseases in vivo. | prion diseases and alzheimer disease (ad) share a variety of clinical and neuropathologic features (e.g. progressive dementia, accumulation of abnormally folded proteins in diseased tissue, and pronounced neuronal loss) as well as pathogenic mechanisms like generation of oxidative stress molecules and complement activation. recently, it was suggested that neuronal death in ad may have its origin in the endoplasmic reticulum (er). cellular stress conditions can interfere with protein folding and ... | 2006 | 16691116 |
| protease-resistant prion protein in lymphoreticular tumors of variant creutzfeldt-jakob disease mice. | we report protease-resistant prion protein (prpres) in spontaneous lymphoreticular tumors of mice infected with the agent of variant creutzfeldt-jakob disease (vcjd). prpres may accumulate in lymphoreticular system tumors of asymptomatic persons with vcjd. the statistical power of estimates of vcjd prevalence might be increased by expanding screening to include samples of lymphoreticular neoplasms. | 2006 | 16704797 |
| a new human genotype prone to variant creutzfeldt-jakob disease. | 2006 | 16709965 | |
| two cases of variant creutzfeldt-jakob disease (vcjd) referred to the department of community mental health, aldershot garrison in 2003. | in the year 2003 the department of community mental health (dcmh) at aldershot garrison received referrals of two soldiers, a sergeant and a lance corporal, who presented with a complex picture of neurological and psychiatric symptoms. both had been investigated very thoroughly by neurologists who, owing to the mainly negative results of their investigations, were unable to make a diagnosis. of the two patients one had also been assessed as a psychiatric in-patient in a civilian hospital and had ... | 2006 | 16749468 |
| detection of type 1 prion protein in variant creutzfeldt-jakob disease. | molecular typing of the abnormal form of the prion protein (prp(sc)) has come to be regarded as a powerful tool in the investigation of the prion diseases. all evidence thus far presented indicates a single prp(sc) molecular type in variant creutzfeldt-jakob disease (termed type 2b), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). here we show for the first time that the prp(sc) that accumulates in the brain in variant creutzfeldt-jakob d ... | 2006 | 16400018 |
| comparative evidence for a link between peyer's patch development and susceptibility to transmissible spongiform encephalopathies. | epidemiological analyses indicate that the age distribution of natural cases of transmissible spongiform encephalopathies (tses) reflect age-related risk of infection, however, the underlying mechanisms remain poorly understood. using a comparative approach, we tested the hypothesis that, there is a significant correlation between risk of infection for scrapie, bovine spongiform encephalopathy (bse) and variant cjd (vcjd), and the development of lymphoid tissue in the gut. | 2006 | 16405727 |
| distinct glycoform ratios of protease resistant prion protein associated with prnp point mutations. | inherited prion diseases are neurodegenerative disorders caused by autosomal dominant mutations in the human prion protein gene (prnp). kindred with inherited prion disease can show remarkable phenotypic variability that has yet to be explained. here we report analysis of protease resistant disease-related prion protein (prp(sc)) isoforms from a range of inherited prion disease cases (point mutations p102l, d178n, e200k and 2-, 4- and 6-octapeptide repeat insertions) and show that the glycoform ... | 2006 | 16415305 |
| clusterin expression in follicular dendritic cells associated with prion protein accumulation. | peripheral accumulation of abnormal prion protein (prp) in variant creutzfeldt-jakob disease and some animal models of transmissible spongiform encephalopathies (tses) may occur in the lymphoreticular system. within the lymphoid tissues, abnormal prp accumulation occurs on follicular dendritic cells (fdcs). clusterin (apolipoprotein j) has been recognized as one of the molecules associated with prp in tses, and clusterin expression is increased in the central nervous system where abnormal prp de ... | 2006 | 16767691 |
| an overview of prion biology and the role of blood filtration in reducing the risk of transfusion-transmitted variant creutzfeldt-jakob disease. | prions are infectious proteins believed to be responsible for a variety of progressive and fatal neurodegenerative diseases, collectively referred to as transmissible spongiform encephalopathies (tse). by 1996, it was recognized that ingestion of beef from cattle afflicted with a tse known as bovine spongiform encephalopathy, could result in a devastating human tse known as variant creutzfeldt-jakob disease (vcjd). two recent reports of probable transfusion-transmitted vcjd have raised concerns ... | 2006 | 16787827 |
| kuru in the 21st century--an acquired human prion disease with very long incubation periods. | kuru provides the principal experience of epidemic human prion disease. its incidence has steadily fallen after the abrupt cessation of its route of transmission (endocannibalism) in papua new guinea in the 1950s. the onset of variant creutzfeldt-jakob disease (vcjd), and the unknown prevalence of infection after the extensive dietary exposure to bovine spongiform encephalopathy (bse) prions in the uk, has led to renewed interest in kuru. we investigated possible incubation periods, pathogenesis ... | 2006 | 16798390 |
| dissociation of pathological and molecular phenotype of variant creutzfeldt-jakob disease in transgenic human prion protein 129 heterozygous mice. | all neuropathologically confirmed cases of variant creutzfeldt-jakob disease (vcjd), characterized by abundant florid plaques and type 4 disease-related prion protein (prp(sc)) in the brain, have been homozygous for methionine at polymorphic residue 129 of prnp. the distinctive neuropathological and molecular phenotype of vcjd can be faithfully recapitulated in prnp-null transgenic mice homozygous for human prp m129 but not v129, where a distinct prion strain is propagated. here we model suscept ... | 2006 | 16809423 |
| variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 1. | from 1986 more than 184,000 cattle in the uk and islands (of which >1,880 have been detected by active surveillance using rapid tests) and approaching 5,500 elsewhere have been confirmed with bse. the original 1988 ban on the use of ruminant-derived protein in ruminant feed has been upgraded and now prohibits the use of any processed animal protein in feed for any farmed food animal. as a result of rigorous enforcement this reinforced ban is now regarded as fully effective from 1 aug. 1996. reas ... | 2006 | 16823691 |
| prion disease genetics. | prion diseases have stimulated intense scientific scrutiny since it was proposed that the infectious agent was devoid of nucleic acid. despite this finding, genetics has played a key role in understanding the pathobiology and clinical aspects of prion disease through the effects of a series of polymorphisms and mutations in the prion protein gene (prnp). the advent of variant creutzfeldt-jakob disease has confirmed one of the most powerful human genetic susceptibility factors, as all tested pati ... | 2006 | 16391566 |
| an immunoassay for the pathological form of the prion protein based on denaturation and time resolved fluorometry. | concern about the possible secondary spread of variant creutzfeldt-jakob disease (vcjd) through blood transfusion and blood products has increased the need for a sensitive and rapid test for the identification of prp(sc) in specimens collected non-invasively from living persons. furthermore, an accurate estimate of the prevalence of pre-clinical vcjd in the british population would be possible if there were such a test that could be applied to specimens available readily (e.g. blood and urine). ... | 2006 | 16219367 |