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variant creutzfeldt jakob disease. 200011023754
transmission of bse by blood transfusion in sheep.we have shown that it is possible to transmit bovine spongiform encephalopathy (bse) to a sheep by transfusion with whole blood taken from another sheep during the symptom-free phase of an experimental bse infection. bse and variant creutzfeldt-jakob disease (vcjd) in human beings are caused by the same infectious agent, and the sheep-bse experimental model has a similar pathogenesis to that of human vcjd. although uk blood transfusions are leucodepleted--a possible protective measure against an ...200011041403
vcjd - predicting the future?the recent emergence of variant creutzfeldt-jakob disease (vcjd) in the uk, and demonstration that vcjd is caused by the same prion strain that causes bovine spongiform encephalopathy, have led to concerns about the possibility of a human epidemic. although only 79 cases of vcjd have occurred to date, it is likely that hundreds of thousands of infected cattle entered the human food chain in the late 1980s and early 1990s, and the average incubation period of vcjd is unknown. mathematical models ...200011054179
prevalence of detectable abnormal prion protein in persons incubating vcjd: plausible incubation periods and cautious inference.both small and large variant creutzfeldt jakob disease (vcjd) epidemics are consistent with the current observed incidence. uncertainty in vcjd projections could potentially be reduced by incorporating information on the prevalence of the infectious agent in persons incubating vcjd. the prospect of vcjd prevalence studies has been raised by detection of abnormal prion protein, thought to be the infectious agent, in appendices and tonsils removed from vcjd patients. although unlinked anonymous te ...200011055271
american academy of pediatrics. technical report: transmissible spongiform encephalopathies: a review for pediatricians. committee on infectious diseases.transmissible spongiform encephalopathies (tses) are a family of rare, slowly progressive, and universally fatal neurodegenerative syndromes affecting animals and humans. until recently, tses were of little interest to pediatricians. however, since the outbreak in adolescents and the association of tses with new-variant creutzfeldt-jakob disease (nvcjd), interest among pediatricians and the general public has increased. even before bovine spongiform encephalopathy and nvcjd were linked, the reco ...200011061795
human prion diseases.the term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions, pathogenic agents with novel modes of replication and transmission. prion diseases are characterized by long incubation periods ranging from months to years and are invariably fatal once clinical symptoms have appeared. they are also called transmissible spongiform encephalopathies (tse), on account of the predominant neuropathological change observed in the central nervous system. the most ...200011087170
prions and blood products.the transmission of creutzfeldt-jakob disease (cjd) by human pituitary-derived growth hormone has led to concerns that blood products might also provide a route for the iatrogenic transmission of cjd. a number of actions have been implemented by regulatory authorities to address such concerns, and numerous studies have been undertaken to determine whether or not there is a risk of cjd being transmitted in this manner. to date, no excess risk has been identified, leading to a growing consensus th ...200011087171
[confusion surrounding bovine spongiform encephalopathy (bse) and the risk of new variant creutzfeldt jakob disease].there is a lot of confusing news regarding the risks of consuming beef for contracting variant creutzfeldt jakob disease. bureaucratic inertia and political expediency are fueled by the lack of pathogenetic and epidemiologic understanding of the mode of transmission. consumers discard beef from their diet, which may be the least contaminated tissue, but other meat products, of which the risks are probably much higher, continue to enjoy free international trade and may be used in the human diet. ...200011143292
the new variant of creutzfeldt-jakob disease.new variant creutzfeldt-jakob disease (nvcjd) is a novel human transmissible spongiform encephalopathy which was first identified in 1996 in the united kingdom (uk). subsequent scientific studies have revealed that the strain of the transmissible agent responsible for nvcjd is identical to that of the bovine spongiform encephalopathy (bse) agent, and the disease has been considered as 'human bse'. by 31 december 1999, 52 cases of nvcjd had been reported (49 cases in the uk, two cases in france a ...200011189730
public health service recommendations for the use of vaccines manufactured with bovine-derived materials.the center for biologics evaluation and research (cber), u.s. food and drug administration (fda) learned earlier this year that some vaccines were manufactured with bovine-derived materials obtained from countries in which bovine spongiform encephalopathy (bse) or a substantial risk for bse exists. a list of these countries is published by the u.s. department of agriculture (usda). this information was of concern because cases of variant creutzfeldt-jakob disease (vcjd) have been attributed to, ...200011190118
transgenic models of prion disease.there is growing concern that bovine spongiform encephalopathy (bse) may have passed from cattle to humans, resulting in approximately 70 cases of an atypical, variant cjd (vcjd) in teenagers and young adults. we report here that transgenic (tg) mice expressing full-length bovine (bo) prp serially propagate bse prions and that there is no species barrier for transmission from cattle to tg(boprp) mice. surprisingly, these same mice were also highly susceptible to vcjd and natural sheep scrapie. t ...200011214913
pathology of variant creutzfeldt-jakob disease.variant creutzfeldt-jakob disease (vcjd) is a novel prion disease in man which was first described in 1996 in the uk. there is substantial evidence to indicate that vcjd represents the effects of the bovine spongiform encephalopathy (bse) agent in man. the neuropathology of vcjd is characterised by the florid plaque, composed of a central amyloid core with a fibrillary periphery, surrounded by a rim of spongiform change in an intact neuropil. unique patterns of prp accumulation in vcjd are revea ...200011214917
clinical and differential diagnosis of creutzfeldt-jakob disease.until recently, the clinical diagnosis of cjd relied mainly on three criteria. these include patient history (rapidly progressive dementia), neurological findings (ataxia, pyramidal/extrapyramidal signs, myoclonus, akinetic mutism) and typical electroencephalographic (eeg) findings. these criteria are fulfilled in typical cases. the occurrence or increase of certain proteins in cerebrospinal fluid (csf; 14-3-3, neuron-specific enolase) now provide important adjuncts in recognizing variant forms. ...200011214918
mad cow disease: new recruits for french prion research.as panic over "mad cow disease" engulfs france and threatens to spread to other countries in western europe, french research minister roger-gérard schwartzenberg last week unveiled detailed plans for spending $27 million the government has earmarked for prion disease research in 2001. next year's budget for studying prions--infectious, abnormal proteins linked to bovine spongiform encephalopathy and its human form, variant creutzfeldt-jakob disease--will triple france's current prion research sp ...200017798203
spongiform disease: experts downplay new vcjd fears.the chilling scenario of getting "mad cow disease" from eating products of other animals besides just cows was splashed across the mainstream british press last week based on a report from a leading u.k. lab that prions--abnormal proteins linked to bovine spongiform encephalopathy and its human form, variant creutzfeldt-jakob disease--can jump from one species to another more easily than previously believed. although some experts say the findings raise concern about a threat to human health, oth ...200017811144
new variant creutzfeldt-jakob disease: three case reports from leicestershire.since a report in 1996 of 10 cases of creutzfeldt-jakob disease (cjd) with onset in a younger than usual age, a pattern of the disease has emerged. this includes early neuropsychiatric features and sensory symptoms and neurological signs such as ataxia and involuntary movements later in the course of the disease. three patients with varied clinical presentations and disease course seen at a single neurology unit are described. the first patient was characterised by cognitive and psychiatric symp ...200010675225
extent of misclassification of death from creutzfeldt-jakob disease in england 1979-96: retrospective examination of clinical records.to investigate the extent to which deaths from creutzfeldt-jakob disease were misclassified during 1979-96.200010634732
developments in variant creutzfeldt jakob disease.the announcement on 17 july 2000 that the rate of increase in the incidence of variant creutzfeldt jakob disease (vcjd) in the united kingdom (uk) had reached statistical significance makes the current issue of eurosurveillance particularly timely. a clus200012631967
ultrastructural analysis of the florid plaque in variant creutzfeldt-jakob disease.we report here the first description of florid plaques--the hallmark of variant creutzfeldt-jakob disease (vcjd). these plaques are composed of broad bundles of amyloid, are highly neuritic and exhibited astrocytes and microglial cells. collectively, they are more similar to neuritic plaques of alzheimer's disease than to kuru plaques of kuru--creutzfeldt-jakob disease--gerstmann-sträussler-sheinker disease.200011693720
variant creutzfeldt-jakob disease (vcjd). precautionary measures against the risk of transmission of the agent of vcjd by blood transfusion. 200011144617
variant creutzfeldt-jakob disease in uk children: a national surveillance study.variant creutzfeldt-jakob disease (vcjd) was first reported in 1996; the youngest patient developed symptoms at 16 years of age. we have done 3 years of prospective active surveillance for progressive intellectual and neurological deterioration (pind) in uk children, and have searched for vcjd among the children who were reported.200011072940
universal leukocyte reduction.leukocyte reduction of blood components, in the united states, is generally reserved for conditions in which a clinical indication has been documented. there is no evidence that either creutzfeldt-jakob disease or variant creutzfeldt-jakob disease are transmitted by transfusion in humans or that leukocyte reduction of blood components could reduce their transmission. a number of adverse outcomes following transfusion are alleged to be the result of white blood cells. at this point in time, there ...200011055514
universal leucodepletion and new-variant creutzfeldt-jakob disease 200010997992
universal leucodepletion to reduce the risk of transmission of new-variant creutzfeldt-jakob disease. 200010991551
leucocyte depletion of blood components.universal leucocyte depletion has been implemented in the uk and several other european countries as a precautionary measure against the potential risk of transmission of variant creutzfeldt-jakob disease by blood transfusion. leucocyte depletion had previously only been recommended for a relatively small proportion of transfusion recipients based on clinical and experimental evidence showing clinical benefit. however there is now increasing evidence to support its value in preventing transfusio ...200010986150
variant creutzfeldt-jakob disease in leicestershire. 200010934815
the "pulvinar" sign in variant creutzfeldt-jakob disease. 200010915718
what is a natural cause of death? a survey of how coroners in england and wales approach borderline cases.many deaths fall in the "grey" area between those that are clearly natural and those that are unnatural. there are no guidelines to help doctors in dealing with such cases and death certification is often arbitrary and inconsistent. in an attempt to initiate debate on these difficult areas, and with the ultimate aim of achieving national consensus, the views of coroners in england and wales were sought.200010889819
the first case of new variant creutzfeldt-jakob disease in france: clinical data and neuropathological findings.clinical data and autopsy findings in a case of new variant creutzfeldt-jakob disease (vcjd) are reported. this case, the first histologically confirmed case described outside the united kingdom, very much resembles the cases described by will et al. [(1996) lancet 347:921-925] and zeidler et al. [(1997) lancet 350:903-908, 908-910]. neuropathological studies failed to reveal any conspicuous clues that could be relevant for understanding the pathophysiology of the disease. for epidemiological su ...200010867807
electron microsocopy of brain amyloid plaques from a patient with new variant creutzfeldt-jakob disease.cerebral cortex biopsy from a patient with new variant creutzfeldt-jakob disease (nvcjd) has been examined at the electron microscope level. spongiform changes corresponded mostly to distended neurites scattered in the neuropil or surrounding amyloid plaques. these latter exhibited heterogeneous submicroscopic morphology including variable amount of loosely interwoven amyloid fibrils admixed in a cellular-rich environment constituted essentially by abnormal neuronal processes. by immunoelectron ...200010867797
new variant creutzfeldt-jakob disease presenting as localization-related epilepsy. 200010851396
update on variant creutzfeldt-jakob disease. 200010842719
diagnosis of new variant creutzfeldt-jakob disease.as of december 31, 1998, 35 deaths had been attributed to new variant creutzfeldt-jakob disease (nvcjd) in the united kingdom, of which 33 cases had been neuropathologically confirmed and 2 classified as probable nvcjd. fifteen cases were male and 20 female. the median illness duration was 14 months (range, 8-38 months) and the median age at death was 29 years (range, 18-53 years). the dinical features were consistent with previous descriptions. in nearly all cases, there were early psychiatric ...200010805327
pulvinar sign on mri images in variant creutzfeldt-jakob disease. 200010791518
the pulvinar sign on magnetic resonance imaging in variant creutzfeldt-jakob disease.there is a need for an accurate non-invasive diagnostic test for variant creutzfeldt-jakob disease (vcjd). we investigated the sensitivity and specificity of bilateral pulvinar high signal on magnetic resonance imaging (mri) for the diagnosis of vcjd.200010791525
variant creutzfeldt-jakob disease--information from the world health organization. 200111605332
variant creutzfeldt-jakob disease in hong kong.a 34-year-old chinese woman who had lived in the united kingdom in the 1980s was admitted to hospital in hong kong because of a 7-month history of progressive neurological deterioration. initially, she complained of heartburn and paraesthesia of the hands and feet. she then developed slowness of speech and gait, and was noted to be forgetful and irritable. in january 2001, she was brought back to hong kong for treatment. on admission in may she was dysarthric, ataxic, and dystonic. magnetic reso ...200111590272
new variant creutzfeldt-jakob disease presenting with loss of taste and smell. 200111534516
old drugs to treat new variant creutzfeldt-jakob disease. 200111520533
detection of variant creutzfeldt-jakob disease infectivity in extraneural tissues.abnormal accumulations of prion protein (prp) can be detected in the spleen, lymph nodes, and tonsils of patients with variant creutzfeldt-jakob disease (vcjd). therefore, it has been assumed, but not shown, that these tissues harbour infectivity, which in turn presents the potential for iatrogenic spread through surgery. here, we show and measure levels of infectivity in spleen and tonsil from two patients with vcjd, by bioassay in intracerebrally inoculated riii mice. similar bioassays failed ...200111476840
a neuropsychological-neuropathological case study of variant creutzfeldt-jakob disease.we report the first neuropsychological-neuropathological case study of a patient with variant creutzfeldt-jakob disease (vcjd) who was seen at the early stages of the disease, and whose cognitive functioning was monitored in the following months until his death. at presentation, his neuropsychological profile included impaired ability to retain new episodic information, deficits on tests of retrieval from semantic memory, and impairments on tests of memory for public knowledge, such as famous pe ...200111459921
edrf transcripts and diagnosis of variant creutzfeldt-jakob disease. 200111445093
safety issues affecting hemophilia products.clotting factor concentrates (cfcs) have evolved substantially toward both safety from pathogens and overall final purity of the products. the array of product types for both factor viii and factor ix cfcs ranges from so-called intermediate purity (containing multiple plasma proteins), very high purity (containing chiefly the respective purified clotting protein plus an albumin stabilizer), and recombinant cfcs (with or without albumin stabilizers). each is discussed in the context of theoretic ...200111441416
new variant creutzfeldt-jakob disease--is our practice safe? 200111412169
[the impact of the implementation of staying in the united kingdom for six months or more as donor exclusion criteria on the donor base of the basque country].to reduce the risk of new variant creutzfeldt-jakob disease by blood products some countries exclude persons who have spent six months or more cumulatively in the united kingdom as blood donors.200111333688
unexamined assumptions in explorations of upper limit for cases of variant creutzfeldt-jakob disease. 200111197358
classical and variant creutzfeldt-jakob diseases and their potential impact on the practice of clinical dentistry in australia.following recent published evidence regarding the experimental transmission of prion diseases via blood transfusion, dental practitioners have expressed their concern about the potential impact of these transmissible spongiform encephalopathies on dental care provision. this review provides updated information on creutzfeldt-jakob disease and related disorders and highlights their potential significance for the practice of clinical dentistry. the current guidelines in australia relating to infec ...200111838871
variant creutzfeldt-jakob disease (vcjd). 200111797239
this is really a case of new variant creutzfeldt-jakob. response to c.j.g. lang et al. concerning our article acta neuropathol (2000) 99:704-708. 200111761727
evaluation of performance of white blood cell reduction filters: an original flow cytometric method for detection and quantification of cell-derived membrane fragments.contamination of blood products by white blood cells leads to a risk of transmission of infectious agents, particularly abnormal prion protein, the probable causative agent of new-variant creutzfeldt-jakob disease. blood product filtration could reduce this risk, but the filtration systems might generate potentially infectious membrane fragments. we developed an original flow cytometric method that allows the detection and quantification of membrane fragments in filtered products and the evaluat ...200111746097
has the variant creutzfeldt-jakob disease epidemic hit its peak? 200111705496
conformation as therapeutic target in the prionoses and other neurodegenerative conditions.neurodegenerative conditions are increasing in prevalence as the average human life expectancy rises. alzheimer's disease (ad) is the fourth commonest cause of death in the united states; the recent outbreak of new variant creutzfeldt-jakob disease (nvcjd) has raised the specter of a large population being at risk to develop this prionosis. the pathogenesis of many neurodegenerative diseases is now recognized to be associated with abnormalities of protein conformation. a common theme in these di ...200121374507
mapping the early steps in the ph-induced conformational conversion of the prion protein.under certain conditions, the prion protein (prp) undergoes a conformational change from the normal cellular isoform, prp(c), to prp(sc), an infectious isoform capable of causing neurodegenerative diseases in many mammals. conversion can be triggered by low ph, and in vivo this appears to take place in an endocytic pathway and/or caveolae-like domains. it has thus far been impossible to characterize the conformational change at high resolution by experimental methods. therefore, to investigate t ...200111248018
adaptation of the bovine spongiform encephalopathy agent to primates and comparison with creutzfeldt-- jakob disease: implications for human health.there is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (bse) has contaminated human beings, causing variant creutzfeldt-jakob disease (vcjd). this disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted bse agent are unknown. we show that (i) bse can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic tran ...200111259641
bovine spongiform encephalopathy and variant creutzfeldt-jakob disease: background, evolution, and current concerns.the epidemic of bovine spongiform encephalopathy (bse) in the united kingdom, which began in 1986 and has affected nearly 200,000 cattle, is waning to a conclusion, but leaves in its wake an outbreak of human creutzfeldt-jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue. although averaging only 10-15 cases a year since its first appearance in 1994, its future magnitude and geographic distribution (in countries that have imp ...200111266289
[confusion surrounding bovine spongiform encephalopathy (bse) and the risk of new variant creutzfeldt-jakob disease]. 200111268917
the emerging european epidemic of variant creutzfeldt-jakob disease and bovine spongiform encephalopathy: lessons for australia. 200111270752
temporary depletion of complement component c3 or genetic deficiency of c1q significantly delays onset of scrapie.following peripheral exposure to transmissible spongiform encephalopathies (tses), infectivity usually accumulates in lymphoid tissues before neuroinvasion. the host prion protein (prpc) is critical for tse agent replication and accumulates as an abnormal, detergent insoluble, relatively proteinase-resistant isoform (prpsc) in diseased tissues. early prpsc accumulation takes place on follicular dendritic cells (fdcs) within germinal centers in lymphoid tissues of patients with variant creutzfeld ...200111283677
complement facilitates early prion pathogenesis.new-variant creutzfeldt-jakob disease and scrapie are typically initiated by extracerebral exposure to the causative agent, and exhibit early prion replication in lymphoid organs. in mouse scrapie, depletion of b-lymphocytes prevents neuropathogenesis after intraperitoneal inoculation, probably due to impaired lymphotoxin-dependent maturation of follicular dendritic cells (fdcs), which are a major extracerebral prion reservoir. fdcs trap immune complexes with fc-gamma receptors and c3d/c4b-opson ...200111283678
bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. 200111290640
geographical distribution of variant creutzfeldt-jakob disease in great britain, 1994-2000.geographical variation in the distribution of variant creutzfeldt-jakob disease (vcjd) might indicate the transmission route of the infectious agent to man. we investigated whether regional incidences of vcjd were correlated with regional dietary data.200111293592
[epidemics of bovine spongiform encephalopathy and new variant of creutzfeldt-jakob disease in humans. most recent findings on prion disease].prion diseases have been popularized by extensive media coverage of bovine spongiform encephalopathy (bse) or "mad cow disease" epidemic, observed in great britain since 1986, and new variant creutzfeldt-jakob disease (nvcjd), reported for the first time in 1996. in contrast to the classical form of the disease, nvcjd affects younger patients, presents a relatively longer duration of illness and is caused by the same agent as bse. evidence from laboratory studies now strongly supports the hypoth ...200111294108
increased expression of the normal cellular isoform of prion protein in inclusion-body myositis, inflammatory myopathies and denervation atrophy.the cellular isoform of the prion protein (prpc) is a glycosylphosphatidylinositol-anchored glycoprotein, normally expressed in neural and non-neural tissues, including skeletal muscle. in transmissible spongiform encephalopathies, or prion diseases, prpc, which is soluble in nondenaturing detergent and sensitive to proteinase k (pk)-treatment, represents the molecular substrate for the production of a detergent-insoluble and pk-resistant isoform, termed prp(sc). in human prion diseases, prp(sc) ...200111303793
prion protein and developments in its detection.the theoretical risk of transmission of variant creutzfeldt-jakob disease (vcjd) via blood transfusions has led to replacement of uk-derived plasma for fractionation by plasma sourced outwith the uk and the introduction of leucodepletion of donated blood and its components. prion protein in an abnormal conformation (prpsc) has been identified as inextricably linked with the infectivity of transmissible spongiform encephalopathies such as vcjd and in this review some of its properties relevant to ...200111328566
absence of protease-resistant prion protein in the cerebrospinal fluid of creutzfeldt-jakob disease.creutzfeldt-jakob disease (cjd), believed to be caused by a protease-resistant isoform of prion protein (prp(sc)), usually manifests itself as a clinically distinctive age-related dementia because of its rapid progression, occasionally accompanied by cerebellar ataxia. recently, a variant cjd (vcjd) has been described, which has prominent early psychiatric symptoms and an earlier age of death. although cerebrospinal fluid (csf) is part of the extracellular fluid of the central nervous system (cn ...200111329135
variant creutzfeldt-jakob disease in an elderly patient.we report a case of variant creutzfeldt-jakob disease(vcjd) in a 74-year old man in whom diagnosis was made at necropsy. the occurrence of vcjd in an individual in this age group is unlikely to be an isolated event. doctors need to be aware that vcjd can arise in elderly patients so that appropriate investigations (including magnetic resonance imaging) can be done, and permission for neuropathological necropsy requested, in suspected cases. this case could also have important implications for pu ...200111343744
identification of multiple quantitative trait loci linked to prion disease incubation period in mice.polymorphisms in the prion protein gene are known to affect prion disease incubation times and susceptibility in humans and mice. however, studies with inbred lines of mice show that large differences in incubation times occur even with the same amino acid sequence of the prion protein, suggesting that other genes may contribute to the observed variation. to identify these loci we analyzed 1,009 animals from an f2 intercross between two strains of mice, cast/ei and nzw/olahsd, with significantly ...200111353827
use of 14-3-3 and other brain-specific proteins in csf in the diagnosis of variant creutzfeldt-jakob disease.the detection of the protein 14-3-3 in the csf has been shown to be a reliable and sensitive marker for sporadic creutzfeldt-jakob disease (cjd). other brain-specific proteins such as neuron specific enolase (nse), s-100b, and tau protein have also been reported to be increased in the csf of patients with sporadic cjd. in 1996 a variant of cjd (vcjd) was described which is likely to be causally linked to the bovine spongiform encephalopathy agent. this study reports and compares the findings of ...200111385008
genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice.the incubation period (ip) and the neuropathology of transmissible spongiform encephalopathies (tses) have been extensively used to distinguish prion isolates (or strains) inoculated into panels of inbred mouse strains. such studies have shown that the bovine spongiform encephalopathy (bse) agent is indistinguishable from the agent causing variant creutzfeldt-jakob disease (vcjd), but differs from isolates of sporadic cjd, reinforcing the idea that the vcjd epidemic in britain results from consu ...200111404459
the leeuwenhoek lecture 2001. animal origins of human infectious disease.since time immemorial animals have been a major source of human infectious disease. certain infections like rabies are recognized as zoonoses caused in each case by direct animal-to-human transmission. others like measles became independently sustained with the human population so that the causative virus has diverged from its animal progenitor. recent examples of direct zoonoses are variant creutzfeldt-jakob disease arising from bovine spongiform encephalopathy, and the h5n1 avian influenza out ...200111405946
scrapie strains maintain biological phenotypes on propagation in a cell line in culture.bovine spongiform encephalopathy (bse) and its human equivalent, variant creutzfeldt-jakob disease (vcjd), are caused by the same strain of infectious agent, which is similar to, but distinct from, >20 strains of their sheep scrapie homologue. a better understanding of the molecular strain determinants could be obtained from cells in monoculture than from whole animal studies where different cell targeting is commonly a strain-related feature. although a few cell types can be infected with diffe ...200111432823
the impact of creutzfeldt-jakob disease and variant creutzfeldt-jakob disease on plasma safety.although the true risk of transmitting (classical) creutzfeld-jakob disease (cjd) and variant cjd (vcjd) via transfusion is likely very minimal, a review of prions and the impact of these associated prion diseases is timely because of their current effect on safety policies in the blood-plasma industry. various types of human and animal prion diseases are outlined and reviewed, with emphasis on the importance of cross-species transmission as is relevant for vcjd. review of the prion theory focus ...200111441420
variant creutzfeldt-jakob disease: a summary of current scientific knowledge in relation to public health.the prion diseases pose unique scientific, medical, veterinary and regulatory challenges. here, we summarize current information bearing on the natural history, pathobiology and epidemiology of these disorders and public policy responses to the potential threats to public health posed, particularly, by bovine spongiform encephalopathy and variant creutzfeldt-jakob disease (vcjd). six years after the first case reports of vcjd, there is still no clear indication of the magnitude of the primary ep ...200111468957
neuroinvasion by a creutzfeldt-jakob disease agent in the absence of b cells and follicular dendritic cells.with the potential spread of bovine spongiform encephalopathy to people as a variant creutzfeldt-jakob disease (cjd), it becomes critical to identify cells in the periphery that carry infection. initial work with scrapie agents suggested that b cells were central vectors for neuroinvasion. subsequent studies indicated that b cells played an indirect role by promoting the development of follicular dendritic cells (fdcs) that accumulate abnormal prion protein (prp). the mechanism for the role of f ...200111470899
tissue distribution of protease resistant prion protein in variant creutzfeldt-jakob disease using a highly sensitive immunoblotting assay.variant creutzfeldt-jakob disease (vcjd) has a pathogenesis distinct from other forms of human prion disease: disease-related prion protein (prp(sc)) is readily detectable in lymphoreticular tissues. quantitation of risk of secondary transmission, and targeting of risk reduction strategies, is limited by lack of knowledge about relative prion titres in these and other peripheral tissues, the unknown prevalence of preclinical vcjd, and a transmission barrier which limits the sensitivity of bioass ...200111476832
afterthoughts about bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. 200111485682
bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. 200111485685
sporadic creutzfeldt-jakob disease in a young dutch valine homozygote: atypical molecular phenotype.a case of sporadic creutzfeldt-jakob disease (scjd) is described in a young dutch protein prion gene (prnp) codon 129 valine homozygote. certain clinical and molecular features of this case overlap those of variant cjd. the case highlights possible difficulties in the differential diagnosis of vcjd and the more rare scjd subtypes based on molecular features alone.200111506411
variant creutzfeldt-jakob disease in australian blood donors: estimation of risk and the impact of deferral strategies.in australia, a policy of deferring donors who have lived in the uk for longer than 6 months between 1980 and 1996 has been instituted to reduce the theoretical risk of transmitting variant creutzfeldt-jakob disease (vcjd) through the blood supply. the objective of this report was to refine estimates of the possible risks and benefits of donor-deferral strategies that are aimed at avoiding transmission of vcjd.200111520409
brain in human nutrition and variant creutzfeldt-jakob disease risk (vcjd): detection of brain in retail liver sausages using cholesterol and neuron specific enolase (nse) as markers.no information is available about the consumption of brain via meat products. with respect to the new variant of creutzfeldt-jakob disease (vcjd) and the presumed food-borne transmission of bovine spongiform encephalopathy (bse) to humans, a preliminary survey for brain and/or spinal cord (tissues of the central nervous system, cns) was conducted. we applied a previously developed integrated procedure using cholesterol and neuron specific enolase (nse) as markers. quantification of cholesterol h ...200111520429
prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies.variant creutzfeldt-jakob disease and bovine spongiform encephalopathy are initiated by extracerebral exposure to prions. although prion transmission from extracerebral sites to the brain represents a potential target for prophylaxis, attempts at vaccination have been limited by the poor immunogenicity of prion proteins. to circumvent this, we expressed an anti-prion protein (anti-prp) mu chain in prnp(o/o) mice. transgenic mice developed sustained anti-prp titers, which were not suppressed by i ...200111546838
bovine spongiform encephalopathy and variant creutzfeldt-jakob disease: implications for australia.the bovine spongiform encephalopathy (bse) epizootic developed in the united kingdom in the mid-1980s. feeding practices in the cattle industry amplified the causative prion, and meat contaminated with bse entered the market. human consumption of prion-contaminated meat led to the new zoonosis--variant creutzfeldt-jakob disease (vcjd). the uk bse inquiry published its report in october 2000; while praising policy decisions, it also documented failures in the execution of these policies, specific ...200111548083
cna42 monoclonal antibody identifies fdc as prpsc accumulating cells in the spleen of scrapie affected sheep.natural scrapie, new variant creutzfeldt-jakob disease and murine experimental transmissible spongiform encephalopathies (tse) are fatal neurodegenerative disorders. the agent responsible for these diseases is closely related to prpsc, an abnormal isoform of the cellular prion protein. before reaching the brain, it invades and replicates in lymphoid organs such as spleen, tonsils and lymph nodes. follicular dendritic cells (fdc) may support the prion replication in lymphoid tissues of sheep as s ...200111557290
variant creutzfeldt-jakob disease is not associated with individual abilities to metabolise organophosphates. 200111561051
florid plaques in ovine prp transgenic mice infected with an experimental ovine bse.the occurrence of the variant creutzfeldt-jakob disease (vcjd), related to bovine spongiform encephalopathy (bse), raises the important question of the sources of human contamination. the possibility that sheep may have been fed with bse-contaminated foodstuff raises the serious concern that bse may now be present in sheep without being distinguishable from scrapie. sensitive models are urgently needed given the dramatic consequences of such a possible contamination on animal and human health. w ...200111571272
long-term subclinical carrier state precedes scrapie replication and adaptation in a resistant species: analogies to bovine spongiform encephalopathy and variant creutzfeldt-jakob disease in humans.cattle infected with bovine spongiform encephalopathy (bse) appear to be a reservoir for transmission of variant creutzfeldt-jakob disease (vcjd) to humans. although just over 100 people have developed clinical vcjd, millions have probably been exposed to the infectivity by consumption of bse-infected beef. it is currently not known whether some of these individuals will develop disease themselves or act as asymptomatic carriers of infectivity which might infect others in the future. we have stu ...200111581378
mri of creutzfeldt-jakob disease: imaging features and recommended mri protocol.creutzfeldt-jakob disease (cjd) is a rare, progressive and invariably fatal neurodegenerative disease characterized by specific histopathological features. of the four subtypes of cjd described, the commonest is sporadic cjd (scjd). more recently, a new clinically distinct form of the disease affecting younger patients, known as variant cjd (vcjd), has been identified, and this has been causally linked to the bovine spongiform encephalopathy (bse) agent in cattle. characteristic appearances on m ...200111585394
editorial: development of australia's response to bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. 200111596725
new variant creutzfeldt-jakob disease: the epidemic that never was. 200111597973
variant creutzfeldt-jakob disease and blood transfusion.variant creutzfeldt-jakob disease (vcjd) was first described in the united kingdom in 1996 and is thought to have been transmitted from cattle infected with bovine spongiform encephalopathy probably via the food chain. thus far just over 100 definite or probable clinical cases have been described, though the number of people currently infected and the eventual size and geographic distribution of any future clinical epidemic remain uncertain. there is little evidence that sporadic cjd is transmit ...200111604578
[what do medical students know about cause and epidemiology of the creutzfeldt-jakob disease in germany?].due to the increase of patients with the variant creutzfeldt-jakob disease (vcjd) in great britain and the first cases of autochthonous bse cases in germany, the study tried to investigate the knowledge of medical students about the epidemiology of cjd in germany and how they assess the influence of different factors on the etiology of cjd. altogether 63 first year medical students, 96 third year medical students and 50 nurses were included in an anonymous questionnaire survey. they were asked t ...200111607873
predictability of the uk variant creutzfeldt-jakob disease epidemic.back-calculation analysis of the variant creutzfeldt-jakob disease epidemic in the united kingdom is used to estimate the number of infected individuals and future disease incidence. the model assumes a hazard of infection proportional to the incidence of bovine spongiform encephalopathy in the united kingdom and accounts for precautionary control measures and very wide ranges of incubation periods. the model indicates that current case data are compatible with numbers of infections ranging from ...200111679631
the role of mri in the diagnosis of sporadic and variant creutzfeldt-jakob disease.creutzfeldt-jakob disease (cjd) is a rare but important fatal, dementing illness. a number of types of cjd are identified, each with distinct clinical features. characteristic mri changes have been described recently. sporadic cjd, the commonest type, is found worldwide, and causes hyperintensity of the putamen and caudate nuclei. in the recently described variant cjd, which affects younger patients and has been linked to bovine spongiform encephalopathy (bse) in cattle, a highly characteristic ...200111688725
the shifting biology of prions.transmissible spongiform encephalopathies (tses), or prion diseases, are rare fatal neurodegenerative diseases of humans and animals. although some tses, like scrapie in sheep, have been known to exist for centuries, bovine spongiform encephalopathy (bse) was recognized only 15 years ago. new variant creutzfeldt-jakob disease (nvcjd) of humans is probably caused by consumption of bse-infected materials. the nature of the infectious agent is not fully elucidated, but substantial evidence suggests ...200111690621
increased susceptibility to kuru of carriers of the prnp 129 methionine/methionine genotype.kuru reached epidemic proportions by the mid-twentieth century among the fore people of new guinea and disappeared after the abolition of cannibalistic rituals. to determine susceptibility to kuru and its role in the spread and elimination of the epidemic, we analyzed the prnp gene coding sequences in 5 kuru patients; no germline mutations were found. analysis of the prnp 129 methionine (m)/valine (v) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic pref ...200111120925
deposition patterns of disease-associated prion protein in captive mule deer brains with chronic wasting disease.chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) in captive and free-ranging cervids in the usa; its origin is obscure. archival formalin-fixed and paraffin-embedded specimens of 16 captive mule deer brains with cwd were analyzed using immunocytochemistry for the disease-associated prion protein (prp). the most prominent pattern of prp deposition were plaque-like structures, a substantial proportion of which were florid plaques surrounded by a rim of spongiform va ...200111699564
sporadic--but not variant--creutzfeldt-jakob disease is associated with polymorphisms upstream of prnp exon 1.human prion diseases have inherited, sporadic, and acquired etiologies. the appearance of the novel acquired prion disease, variant creutzfeldt-jakob disease (vcjd), and the demonstration that it is caused by the same prion strain as that causing bovine spongiform encephalopathy, has led to fears of a major human epidemic. the etiology of classical (sporadic) cjd, which has a worldwide incidence, remains obscure. a common human prion-protein-gene (prnp) polymorphism (encoding either methionine o ...200111704923
estimation of epidemic size and incubation time based on age characteristics of vcjd in the united kingdom.the size of the variant creutzfeldt-jakob disease (vcjd) epidemic in the united kingdom is a major public health concern and a subject of speculation. the cases are young (mean age = 28). assuming that the risk of developing the disease in susceptible exposed subjects decreases exponentially with age after age 15, that all infections occurred between 1980 and 1989, and that the distribution of the incubation period is lognormal, we estimate that the mean duration of the incubation period is 16.7 ...200111721058
the molecular pathology of cjd: old and new variants.the study of prion disease has become an area of intense interest since experimental evidence emerged for the transmission of phenotypic variation without the involvement of a nucleic acid component. additional impetus has come from the widespread concern that exposure to bovine spongiform encephalopathy contaminated material poses a distinct and, conceivably, a severe threat to public health in the uk and other countries. the occurrence of new variant creutzfeldt-jakob disease has dramatically ...200111724914
bse did not cause variant cjd: an alternative cause related to post-industrial environmental contamination.the new prion diseases that have emerged in the last 15 years are bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (variant cjd). although initially confined to the uk, these diseases have recently emerged in other european countries. the accepted cause of the human disease is that bse spread from cattle to humans by the consumption of infected beef. however, the evidence that supports this is very thin. this article describes this evidence and lists a series of hypot ...200111735310
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