Publications
| Title | Abstract | Year(sorted descending) Filter | PMID Filter |
|---|
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| [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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| an enzyme-detergent method for effective prion decontamination of surgical steel. | prions, transmissible agents that cause creutzfeldt-jakob disease (cjd) and other prion diseases, are known to resist conventional sterilization procedures. iatrogenic transmission of classical cjd via neurosurgical instruments is well documented and the involvement of lymphoreticular tissues in variant cjd (vcjd), together with the unknown population prevalence of asymptomatic vcjd infection, has led to concerns about transmission from a wide range of surgical procedures. to address this proble ... | 2005 | 15722550 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| 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 |
| [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 |
| 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 |
| 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 |
| accumulation of prion protein in the peripheral nervous system in human prion diseases. | after the finding that anti-prion antibodies stain sensory and sympathetic ganglia in variant creutzfeldt-jakob disease (vcjd), it was suggested that this localization supported the oral route of entry. however, prion accumulation subsequently also appeared in the peripheral nervous system (pns) in sporadic cases. this study aims at evaluating the extent of prion protein accumulation in the pns in all clinicopathologic subgroups of the disorder, with the exception of the familial and sporadic fo ... | 2005 | 16106220 |
| [update on transmissible spongiform subacute encephalopathies (tsse)]. | this update concerns human and ruminant transmissible spongiform subacute encephalopathies (tsse). the latest data on variant creutzfeldt-jakob disease confirm that new cases are less frequent than feared some years ago, but subclinical carriers could be a source of iatrogenic infection. the macaque is a good model of human oral transmission of bovine spongiform encephalopathy (bse). the latest data on bse in europe confirm the effectiveness of precautionary measures taken in 1996 and 2000. conc ... | 2005 | 16114866 |
| the use of non-prion biomarkers for the diagnosis of transmissible spongiform encephalopathies in the live animal. | scrapie and bovine spongiform encephalopathy (bse) are major global concerns and the emergence of variant creutzfeldt-jakob disease (vcjd) has caused turmoil for blood transfusion services and hospitals worldwide. recent reports of iatrogenic cjd (icjd) cases following blood transfusions from transmissible spongiform encephalopathies (tse)-infected donors have fuelled this concern. major diagnostic tests for bse and scrapie are conducted post-mortem from animals in late stages of the disease. al ... | 2005 | 16120244 |
| bovine prion protein gene (prnp) promoter polymorphisms modulate prnp expression and may be responsible for differences in bovine spongiform encephalopathy susceptibility. | the susceptibility of humans to the variant creutzfeldt-jakob disease is greatly influenced by polymorphisms within the human prion protein gene (prnp). similar genetic differences exist in sheep, in which prnp polymorphisms modify the susceptibility to scrapie. however, the known coding polymorphisms within the bovine prnp gene have little or no effect on bovine spongiform encephalopathy (bse) susceptibility in cattle. we have recently found a tentative association between prnp promoter polymor ... | 2005 | 16141216 |
| inactivation of the bse agent by the heat and pressure process for manufacturing gelatine. | dietary exposure to the bovine spongiform encephalopathy (bse) agent is the probable cause of variant creutzfeldt-jakob disease in people. the industrial manufacturing process for the production of gelatine and colloidal protein by the heat and pressure process was downscaled accurately and its capacity to remove or inactivate bse infectivity was investigated. gelatine was made from bones experimentally contaminated with mouse brain infected with the 301v strain of mouse-passaged bse agent in wh ... | 2005 | 16157568 |
| 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 |
| variant creutzfeldt-jakob disease: implications for the health care system. | the recognition of the first cases of variant creutzfeldt-jakob disease in the united kingdom (uk) in 1996 and the realisation that this new disease represented the human form of the cattle disease bse has prompted a considerable investment in research, particularly in the uk, europe and the united states (us). much has been learnt about this disease but much is still unknown. infectivity is not destroyed by conventional sterilisation and disinfection treatment methods. this, combined with the w ... | 2005 | 16222925 |
| 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 |
| 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 |
| 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 | |
| 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 |
| [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 |
| 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 |
| 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 |
| 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 |
| variant creutzfeldt-jakob disease death, united states. | the only variant creutzfeldt-jakob disease (vcjd) patient identified in the united states died in 2004, and the diagnosis was confirmed by analysis of autopsy tissue. the patient likely acquired the disease while growing up in great britain before immigrating to the united states in 1992. additional vcjd patients continue to be identified outside the united kingdom, including 2 more patients in ireland, and 1 patient each in japan, portugal, saudi arabia, spain, and the netherlands. the reports ... | 2005 | 16229761 |
| a response to 'lymphocyte contamination of laryngoscope blades--a possible vector for transmission of variant creutzfeldt-jakob disease'. | 2005 | 16229718 | |
| american fresh frozen plasma for neonates and children. | from the spring of 2004 the united kingdom blood services have been importing fresh frozen plasma from united states donors for all neonates and children born after 1 january 1996. the decision to mandate the use of american plasma in this age group was taken by the department of health in 2002 as part of its precautionary approach to the risk of transfusion transmitted variant creutzfeldt-jakob disease. in this article we explain the background to this decision and explore some of the implicati ... | 2005 | 15613525 |
| variant creutzfeldt-jakob disease: update. | 2005 | 15591527 | |
| high levels of disease related prion protein in the ileum in variant creutzfeldt-jakob disease. | 2005 | 16162963 | |
| genotype frequencies at codon 129 of the prion protein gene in brazil: implications in susceptibility to variant creutzfeldt-jakob disease compared to european and asian populations. | a polymorphism at codon 129 of the prion protein gene has been shown to confer genetic susceptibility to prion diseases, and to influence the epidemic course of variant creutzfeldt-jakob disease. we employed a pcr-endonuclease digestion-based assay to investigate this genetic trait in brazil, and then compared our results to previously published data from several european and asian countries. | 2005 | 16119432 |
| abnormal prion protein in the retina of the most commonly occurring subtype of sporadic creutzfeldt-jakob disease. | involvement of the eye has been reported in patients with variant creutzfeldt-jakob disease (vcjd), but there is disagreement on whether retinal involvement occurs in sporadic creutzfeldt-jakob disease (scjd). | 2005 | 16113366 |
| leucoreduction and variant creutzfeldt-jakob disease. | 2005 | 16101813 | |
| lymphocyte contamination of laryngoscope blades--a possible vector for transmission of variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is associated with extensive prion infection of lymphoreticular tissues during the prolonged asymptomatic incubation period. instruments exposed to infected tissues of preclinically infected individuals during medical or surgical procedures represent a potential risk of iatrogenic transmission of vcjd prions. we assessed the frequency of contamination with lymphoid tissue of single-use laryngoscope blades used for tracheal intubation for general anaesthes ... | 2005 | 15960716 |
| the "pulvinar sign" in a case of paraneoplastic limbic encephalitis associated with non-hodgkin's lymphoma. | this paper reports a 59 year old woman with paraneoplastic limbic encephalitis associated with diffuse large b cell lymphoma. her brain magnetic resonance imaging scan showed bilateral posterior thalamic hyperintensities, similar to the "pulvinar sign". her symptoms included progressive psychiatric disturbance and resembled the initial symptoms of variant creutzfeldt-jakob disease (vcjd). clinicians should consider this treatable disorder in the differential diagnosis of vcjd. | 2005 | 15897519 |
| variation in concentration of prion protein in the peripheral blood of patients with variant and sporadic creutzfeldt-jakob disease detected by dissociation enhanced lanthanide fluoroimmunoassay and flow cytometry. | a highly sensitive dissociation-enhanced lanthanide fluoroimmunoassay (delfia) and flow cytometry techniques have previously been developed and employed to characterize soluble cellular prion protein (prp(c)) expression in whole blood and separated components from healthy adult blood donors. no previous studies with these techniques have evaluated the concentration and expression of prp in the blood of patients with variant creutzfeldt-jakob disease (vcjd). | 2005 | 15819670 |
| size frequency distribution of prion protein (prp) aggregates in variant creutzfeldt-jakob disease (vcjd). | the frequency distribution of aggregate size of the diffuse and florid-type prion protein (prp) plaques was studied in various brain regions in cases of variant creutzfeldt-jakob disease (vcjd). the size distributions were unimodal and positively skewed and resembled those of beta-amyloid (a beta) deposits in alzheimer's disease (ad) and down's syndrome (ds). the frequency distributions of the prp aggregates were log-normal in shape, but there were deviations from the expected number of plaques ... | 2005 | 15785857 |
| 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 |
| improved infection control in the prevention of variant creutzfeldt-jakob disease in australia: costs and benefits. | to evaluate the costs and benefits of infection control strategies to prevent the transmission of variant creutzfeldt-jakob disease (vcjd) in ophthalmic surgery in australia. | 2004 | 15707207 |
| [informing the transfused patient of the possible transmission of variant creutzfeldt-jakob disease by blood transfusion]. | true risks and theoretical risks: the texts ruling the obligation of the physician to inform the patient appears not to include theoretical risks in their application. in france however, the field of blood transfusions extends this obligation to the theoretical risk of potential transmission through the blood of the infectious agent responsible for creutzfeldt-jakob's disease. | 2004 | 15637795 |
| joint ash and aabb educational session. | in the vein-to-vein flow of blood from donor to patient, the role of the transfusion medicine specialist has become increasingly centered at the bedside. three clinically centered issues in blood safety and in blood conservation are presented in this chapter. in section i, dr. patricia hewitt presents the epidemiologic and clinical evidence regarding new variant creutzfeldt-jakob disease (nvcjd) in the uk and its relevance to transfusion medicine. lessons learned from the responses by the nation ... | 2004 | 15561698 |
| variant creutzfeldt-jakob disease and prions in the blood supply. | 2004 | 16163166 | |
| the success of precaution? managing the risk of transfusion transmission of variant creutzfeldt-jakob disease. | the precautionary principle has emerged as an important new paradigm influencing decision making in the blood system. the principle has influenced decision making in several nations leading to the institution of policies to protect their blood supplies form variant creutzfeldt-jakob disease (vcjd). increasingly evidence has emerged to support the institution of these policies, which were introduced in advance of clear evidence of risk. these vcjd decisions serve as an example of the successful a ... | 2004 | 15383021 |
| quantifying losses to the donated blood supply due to donor deferral and miscollection. | donors are deferred for multiple reasons. losses related to disease marker rates are well established. donor and donation losses for other reasons, however, have not been extensively quantified. | 2004 | 15383013 |
| surveillance for progressive intellectual and neurological deterioration in the canadian paediatric population. | to conduct active surveillance of the canadian paediatric population for children who have a progressive intellectual and neurological deterioration to detect the occurrence of cases of creutzfeldt-jakob disease or variant creutzfeldt-jakob disease. | 2004 | 15198447 |
| does a perception of increased blood safety mean increased blood transfusion? an assessment of the risk compensation theory in canada. | the risk compensation theory is a widely used concept in transport economics to analyze driver risk behaviour. this article explores the feasibility of applying the theory in blood transfusion to raise important questions regarding the increased blood safety measures and their possible effects on blood usage (e.g., the appropriateness in transfusion). further, it presents the findings of a pilot survey of physicians in canada. | 2004 | 15182381 |
| is variant creutzfeldt-jakob disease in young children misdiagnosed as alpers' syndrome? an analysis of a national surveillance study. | there has been concern that children with variant creutzfeldt-jakob disease (vcjd) might be misdiagnosed as cases of alpers' syndrome, as a spongiform degeneration of the brain is seen in both conditions. | 2004 | 15146014 |
| mechanism of intestinal entry of infectious prion protein in the pathogenesis of variant creutzfeldt-jakob disease. | the pathogenesis of variant creutzfeldt-jakob disease (vcjd) is most likely to be dependent on intestinal entry of orally ingested infectious prion proteins, though tonsils or other oral portals of entry are possible. the exact route of entry of infectious prion proteins is uncertain but receptors for prion proteins such as laminin receptor precursor (lrp) may be expressed on intestinal brush border. cellular prion protein (prp(c)) is expressed on intestinal enteric nervous system and is separat ... | 2004 | 15063598 |
| variant creutzfeldt-jakob disease: between lymphoid organs and brain. | prion diseases are often caused by peripheral uptake of the infectious agent. to reach their ultimate target, the central nervous system (cns), prions enter their host, replicate in lymphoid organs and spread via peripheral nerves. once the agent has reached the cns disease progression is rapid, resulting in neurodegeneration and death. many of these mechanisms have been uncovered using genetically modified mice. a recently published study demonstrated the presence of pathological prion protein ... | 2004 | 15040321 |
| the pulvinar sign in variant creutzfeldt-jakob disease. | 2004 | 15023827 | |
| mimicry of variant creutzfeldt-jakob disease by sporadic creutzfeldt-jakob disease: importance of the pulvinar sign. | 2004 | 15023826 | |
| variations in neurodegenerative disease across the uk: findings from the national study of progressive intellectual and neurological deterioration (pind). | to identify any uk children with variant creutzfeldt-jakob disease (vcjd) and obtain information about the causes of progressive intellectual and neurological deterioration (pind) and the geographical distribution of cases. | 2004 | 14709491 |
| anaesthesia in patients with dementia. | the next couple of decades will be characterized by an increase in life expectancy, leading to an older population. as the incidence of alzheimer's dementia and vascular dementia is rising with age, the future anaesthesiologist will be increasingly confronted with perioperative care of patients with impaired cognitive function. this paper tries to highlight some topics specifically related to demented patients. | 2004 | 17021564 |
| an outline of the neuropathology of transmissible spongiform encephalopathies (prion diseases). | we review here the basic neuropathology of transmissible spongiform encephalopathies (tse) or prion diseases. the classic hallmark of tse neuropathology is a combination (in different proportions in different diseases) of spongiform change, astrocytosis, neuronal loss and amyloid plaques. immunohistochemically, accumulation of the abnormal isoform of prion protein (prp(sc) or prp(d)) is regarded as a diagnostic for tse. we also review the peculiarities of kuru, variant creutzfeldt-jakob disease ... | 2004 | 16903141 |
| clinical findings and diagnostic tests in creutzfeldt-jakob disease and variant creutzfeldt-jakob disease. | sporadic creutzfeldt-jakob disease (scjd) is a rare transmissible disease caused by accumulation of pathological prion protein in the cns. scjd typically affects patients in their sixties. the median disease duration in scjd (6 months) is shorter than in variant creutzfeldt-jakob disease (vcjd) (14 months). the clinical diagnosis in scjd is supported by the detection of periodic sharp and slow wave complexes (pswc) in the electroencephalogram, 14-3-3 proteins in the cerebrospinal fluid (csf) and ... | 2004 | 16903140 |
| current safety of the blood supply in the united states. | in common with other developed countries, the united states has placed a great deal of emphasis on blood safety. as a result of careful donor selection and the use of advanced tests, including nucleic acid testing (nat), the risk of transmission of human immunodeficiency virus and hepatitis c virus has been reduced to about 1 in 1.5 million donations. nat for hepatitis b virus has not been introduced, but nevertheless the risk is low. attention recently has been focused on emerging infections. n ... | 2004 | 15615252 |
| cd163 identifies a unique population of ramified microglia in hiv encephalitis (hive). | the idea that cns ramified microglia are quiescent has been challenged by studies that show that microglia without the classic signs of activation can be phagocytic and appear with shorter, thicker ramifications. these semi-activated cells may constitute a form of microglia that has not been previously recognized in neuropathological conditions and may contribute to the pathology and dysfunction in these disorders. this study investigated the expression of cd 163, a cell surface marker whose nor ... | 2004 | 15624762 |
| [comments on present-day spread and epidemiology of bse and prion diseases]. | prion diseases of animals and man are neurological diseases with amyloidal deposition of the respective proteins. as to prion disease, the cellular prion protein is in its abnormal isoform(s) an essential component of prion protein aggregates found in affected tissue. in contrast to all neurodegenerative diseases like morbus alzheimer or huntington's disease, prion diseases are transmissible. therefore, prion diseases were designated transmissible spongiform encephalopathies (tse). the diseases ... | 2004 | 14770333 |
| possible transmission of variant creutzfeldt-jakob disease by blood transfusion. | variant creutzfeldt-jakob disease (vcjd) is a novel human prion disease caused by infection with the agent of bovine spongiform encephalopathy (bse). epidemiological evidence does not suggest that sporadic cjd is transmitted from person to person via blood transfusion, but this evidence may not apply to vcjd. we aimed to identify whether vcjd is transmissible through blood transfusion. | 2004 | 14962520 |
| tissue distribution of bovine spongiform encephalopathy agent in primates after intravenous or oral infection. | the disease-associated form of prion protein (prp(res)) has been noted in lymphoreticular tissues in patients with variant creutzfeldt-jakob disease (vcjd). thus, the disease could be transmitted iatrogenically by surgery or use of blood products. we aimed to assess transmissibility of the bovine spongiform encephalopathy (bse) agent to primates by the intravenous route and study its tissue distribution compared with infection by the oral route. | 2004 | 14962521 |
| identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic creutzfeldt-jakob disease. | transmissible spongiform encephalopathies (tses), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (prp(sc)) of the host-encoded cellular prion protein (prp(c)). human and animal tse agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant prp(sc) fragments and the degree of glycosylation. ... | 2004 | 14970340 |
| small de novo duplication in the repeat region of the tata-box-binding protein gene manifest with a phenotype similar to variant creutzfeldt-jakob disease. | a 20-year-old north american patient developed rapidly progressive cognitive decline and pronounced ataxia, a phenotype compatible with prion disease. no structural changes were found in the prnp gene, which excludes genetic prion disease, but the patient's prnp codon 129 met/met genotype is known to predispose to variant creutzfeldt-jakob disease (vcjd). further studies identified an expanded allele with 55 cag/caa repeats in the tbp gene. the increase of trinucleotide repeat number in the codi ... | 2004 | 15521976 |
| human prion protein with valine 129 prevents expression of variant cjd phenotype. | variant creutzfeldt-jakob disease (vcjd) is a unique and highly distinctive clinicopathological and molecular phenotype of human prion disease associated with infection with bovine spongiform encephalopathy (bse)-like prions. here, we found that generation of this phenotype in transgenic mice required expression of human prion protein (prp) with methionine 129. expression of human prp with valine 129 resulted in a distinct phenotype and, remarkably, persistence of a barrier to transmission of bs ... | 2004 | 15539564 |
| update on creutzfeldt-jakob disease. | prion diseases are transmissible fatal neurodegenerative disorders in which infectivity is associated with the accumulation of prp(sc), a disease-related isoform of normal cellular prion protein. the recent emergence of variant creutzfeldt-jakob disease has led to major public health concerns, and the need for the development of effective treatments. as prp(sc) is associated both with pathology and infectivity, therapeutic approaches to date have largely aimed at preventing its accumulation, but ... | 2004 | 15542971 |