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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
odyssey toward a healthier world.the 22nd international congress of chemotherapy was held in amsterdam, the netherlands, july 14, 2001. the congress attracted participants from around the world and covered a broad spectrum of work on microbial infections and cancer, their treatment by antiinfective drugs and their prevention by vaccination. a theme of the congress was "compassion and science," and this was picked up in a fascinating albeit slightly controversial symposium on "health, human rights and infection." there were seve ...200112813588
the transmissible spongiform encephalopathies: pathogenic mechanisms and strategies for therapeutic intervention.primary neurodegenerative diseases tend to be intractable and largely affect the elderly. there is rarely the opportunity to identify individuals at risk and the appearance of clinical symptoms usually signifies the occurrence of irreversible neurological damage. this situation describes sporadic creutzfeldt-jakob disease which occurs world-wide, affecting one person per million per annum. the epidemic of bovine spongiform encephalopathy in the uk in the 1980s and the subsequent causal appearanc ...200112540284
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
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
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
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
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
edrf transcripts and diagnosis of variant creutzfeldt-jakob disease. 200111445093
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
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
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
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
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
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
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
bovine spongiform encephalopathy and variant creutzfeldt-jakob disease: a risk analysis.there is doubt that variant creutzfeldt-jakob disease (vcjd) resulted from bovine spongiform encephalopathy (bse) transmission from cattle to human. what is uncertain is the total number of vcjd cases (currently about 80). in this review i covered recent data on the vcjd and bse epidemic, the mode of bse spreading to humans and, finally, the data on the prnp analogue--the doppel gene (prnd).200011693716
[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
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
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
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
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
prion diseases, blood and the immune system: concerns and reality.there is a great amount of uncertainty about the nature of the agent which causes spongiform encephalopathies. in recent years the occurrence of bovine spongiform encephalopathy and of new variant-creutzfeldt jakob disease, has raised concerns that prions may, under certain circumstances, contaminate the blood supply. this review article illustrates the problems with which research in this field is fraught, and presents some of the arguments which are controversially discussed in the field.200010627667
reversion of prion protein conformational changes by synthetic beta-sheet breaker peptides.transmissible spongiform encephalopathies are associated with a structural transition in the prion protein that results in the conversion of the physiological prpc to pathological prp(sc). we investigated whether this conformational transition can be inhibited and reversed by peptides homologous to the prp fragments implicated in the abnormal folding, which contain specific residues acting as beta-sheet blockers (beta-sheet breaker peptides).200010675119
adaptation and selection of prion protein strain conformations following interspecies transmission of transmissible mink encephalopathy.interspecies transmission of the transmissible spongiform encephalopathies (tses), or prion diseases, can result in the adaptation and selection of tse strains with an expanded host range and increased virulence such as in the case of bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. to investigate tse strain adaptation, we serially passaged a biological clone of transmissible mink encephalopathy (tme) into syrian golden hamsters and examined the selection of distinct strai ...200010823860
age-related expression of the cellular prion protein in human peripheral blood leukocytes.creutzfeldt-jakob disease typically affects older patients, yet victims of new-variant creutzfeldt-jakob disease (nvcjd) are unusually young. because the cellular prion protein prp(c) is required for disease development, we investigated age-dependent variability in cell surface prp(c) expression on various subclasses of human peripheral blood leukocytes (pbl) as a possible susceptibility factor.200010870113
variant creutzfeldt-jakob disease: immunocytochemical studies and image analysis.variant creutzfeldt-jakob disease (vcjd) is a recently identified human prion disease that appears to arise from exposure to the bovine spongiform encephalopathy agent. the clinical features and neuropathology of vcjd are distinctive, particularly the patterns of prp(sc) accumulation in the brain. prp immunocytochemistry has also demonstrated the accumulation of prp(sc) in tissues outside the central nervous system, including sensory ganglia and lymphoid tissues. these observations have allowed ...200010871542
diverse patterns of expression of the 67-kd laminin receptor in human small intestinal mucosa: potential binding sites for prion proteins?it has been shown that the 67-kd laminin receptor (lr) may function as a receptor for sindbis and tick-born encephalitis viruses. recent data indicate that the 37-kd precursor (lrp) for this molecule acts as a receptor for prion proteins (prp), self-proteins implicated in the pathogenesis of transmissible spongiform encephalopathies including new variant creutzfeldt-jakob disease (nvcjd). laminin and prp share the same binding site on lrp, which is incorporated into the mature lr as a functional ...200010878555
variant creutzfeldt-jakob disease. 200010914416
laboratory diagnosis of variant creutzfeldt-jakob disease.the neuropathological and biochemical features of 33 cases of variant creutzfeldt-jakob disease (vcjd) diagnosed up to the end of 1998 are analysed in relation to the 646 cases of suspected cjd referred to the cjd surveillance unit laboratory from 1990 to 1998. morphological studies of the central nervous system, lymphoid tissues and other organs were accompanied by immunocytochemistry; western blot analysis of prpres was performed on frozen brain tissue. the findings were analysed in relation t ...200010931212
examination of the human prion protein-like gene doppel for genetic susceptibility to sporadic and variant creutzfeldt-jakob disease.a novel human gene named doppel (dpl) that has homology to the prion protein gene (prnp) has recently been identified on chromosome 20p. by automated sequencing we have found a common (m174t, 48%) and an uncommon coding polymorphism. the polymorphic frequency of the m174t allele was examined in cases of variant and sporadic creutzfeldt-jakob disease and compared with the frequency in the normal uk population. in sharp distinction to the m129v polymorphism of prnp we have not found any evidence o ...200010936691
prions and transfusion medicine.there is growing concern at national and international levels that blood supplies might be contaminated with creutzfeldt-jakob disease (cjd) agents (prions).200010938959
[the future and problems of vigilance in creutzfeldt-jakob disease].introduction: the epidemiological surveillance of human transmissible spongiform encephalopathies, is heavily dependent on diagnostic quality, requires therefore a dynamic health care system able to incorporate new diagnostic tools, and rests on activities within three major observation fields: study of age-specific incidences of the disorder, identification of possible changes in clinico-pathological profile, particularly, of variant creutzfeldt-jakob disease, and analysis of incidences among t ...200010951677
follicular dendritic cells in tse pathogenesis.the pathogenesis of transmissible spongiform encephalopathies (tses) often includes a replication phase in lymphoid tissues before infection spreads to the central nervous system. recent studies show that the follicular dendritic cells of the germinal centres are critical for this replication. these cells are therefore potential targets for therapy or prophylaxis in natural tses, such as variant creutzfeldt-jakob disease.200010953096
the human transmissible spongiform encephalopathies (tses): implications for dental practitioners.transmissible spongiform encephalopathies (tses) are rare, fatal degenerative brain diseases which affect humans and certain animals, and are caused by inheritance or acquisition of prions (prps). inherited tses include fatal familial insomnia (ffi), gerstmann-straussler-scheinker syndrome (gss) and other less well clinically characterised disorders, while the human infective tses include sporadic, iatrogenic and variant creutzfeldt-jakob disease (vcjd). the causative prions are found especially ...200010953401
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
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
variant creutzfeldt-jakob disease and the quebec blood supply. 200010976257
incidence of variant creutzfeldt-jakob disease in the uk.the number of deaths from variant cjd (vcjd) in the uk increased in the last quarter of 1998, although numbers were lower in subsequent quarters. we analysed the numbers of definite and probable (living and dead) vcjd cases since 1994 to assess trends in incidence. we estimated that the number of onsets increased by 23% per year for 1994-2000 (p=0.004), and that deaths increased by 33% for 1995-2000 (p=0.005). the absolute number of cases in the uk is still low, but such an increase should be a ...200010981894
epidemiology. tracking the human fallout from 'mad cow disease'.a task force here has been studying cases of variant creutzfeldt-jakob disease (vcjd), an incurable malady of the brain and nervous system that has been linked to eating beef or other products from cattle infected with bovine spongiform encephalopathy or "mad cow disease." the team's goal is to find out just how the patients got infected and how many of them there may ultimately be. the number of confirmed or probable vcjd cases in the united kingdom is still relatively small--a total of 80 as s ...200010991726
variant creutzfeldt jakob disease. 200011023754
variant creutzfeldt-jakob disease: an update. 200010748782
investigation of variant creutzfeldt-jakob disease and other human prion diseases with tonsil biopsy samples.prion diseases are associated with the accumulation of an abnormal isoform of cellular prion protein (prpsc), which is the principal constituent of prions. prions replicate in lymphoreticular tissues before neuroinvasion, suggesting that lymphoreticular biopsy samples may allow early diagnosis by detection of prpsc. variant creutzfeldt-jakob disease (variant cjd) is difficult to distinguish from common psychiatric disorders in its early stages and definitive diagnosis has relied on neuropatholog ...19999923873
molecular analysis of ovine prion protein identifies similarities between bse and an experimental isolate of natural scrapie, ch1641.new variant creutzfeldt-jakob disease (vcjd) and bovine spongiform encephalopathy (bse) are caused by the same strain of pathogen and, as sheep can develop experimental bse, this has raised concern that humans may be at risk from eating mutton if bse has naturally transmitted to sheep. biochemical typing of abnormal prion proteins (prpsc) has been suggested to detect bse in sheep. although this approach is ingenuous, we can now report biochemical evidence of strain variation in contemporary and ...19999934675
geographical distribution of variant cjd in the uk (excluding northern ireland).the agent that causes variant creutzfeldt-jakob disease (variant cjd) is indistinguishable from the causative agent of bovine spongiform encephalopathy (bse). the transmission route by which human beings are infected has not been established. one hypothesis is that cases of variant cjd have resulted from exposure to the bse agent via rendering plants involved in the production of meat and bone meal, the main vehicle of the bse epidemic.199910023945
new variant creutzfeldt-jakob disease.new variant creutzfeldt-jakob disease is a novel human spongiform encephalopathy with a consistent clinico-pathological phenotype. epidemiological evidence indicates that this disease is occurring almost exclusively in the uk, where there has been an epidemic of spongiform encephalopathy in the cattle population. current evidence strongly supports the hypothesis that there is a causal link between bovine spongiform encephalopathy and new variant creutzfeldt-jakob disease.199910221162
new variant creutzfeldt-jakob disease (nvcjd): the risk of transmission by blood transfusion and the potential benefit of leukocyte-reduction of blood components. 199910218230
emerging infectious diseases and pathogens.emerging infectious diseases are caused by old, new, and mutant microorganisms. emergence of these pathogens can be attributed to changes in the characteristics and risk factors of patients, the widespread use of antibiotics, changes in the environment, the role of xenotransplantation, and international travel. in the united states, the incidences of c. difficile, cyclosporiasis, enterohemorrhagic e. coli gastroenteritis, hantavirus, hepatitis c virus infection, and lyme disease have increased s ...199910318733
risk of transmission of bovine spongiform encephalopathy to humans in the united states: report of the council on scientific affairs. american medical association.the risk of possible transmission of bovine spongiform encephalopathy (bse) in the united states is a substantial public health concern.199910386559
the relationship between new variant creutzfeldt-jakob disease and bovine spongiform encephalopathy.creutzfeldt-jakob disease (cjd) has been transmitted in the laboratory and also by iatrogenic accident. however, research has failed to find evidence that its most common form (sporadic cjd) is a natural infection and, in particular, that there is a causal link with scrapie. bovine spongiform encephalopathy (bse) probably resulted from scrapie infection in cattle food. in the wake of the bse epidemic, a novel clinico-pathological form of cjd has been recognized: new variant cjd (nvcjd). this pap ...199910394138
tragedy of variant creutzfeldt-jakob disease. 199910459895
deaths from variant creutzfeldt-jakob disease. 199910459909
bovine spongiform encephalopathy and new variant creutzfeldt-jakob disease: an overview.about 10 years after bovine spongiform encephalopathy (bse) appeared in british cattle, a new variant of creutzfeldt-jakob disease (nv-cjd) was described in the united kingdom. this new disease is distinguishable from classical cjd in its aetiology, epidemiology, clinical profile, and neuropathology. the emergence of nv-cjd raised fears of a causal relationship between bse and nv-cjd and of a human epidemic of indeterminate size. this paper reviews our knowledge of this group of diseases, and ex ...199910462888
variant creutzfeldt-jakob disease.it is clear that the prion strain causing bovine spongiform encephalopathy (bse) in cattle has infected human beings, manifesting itself as a novel human prion disease, variant creutzfeldt-jakob disease (cjd). studies of the incubation periods seen in previous epidemics of human prion disease and of the effect of transmission barriers limiting spread of these diseases between species, suggest that the early variant cjd cases may have been exposed during the preclinical phase of the bse epidemic. ...199910440324
contaminated surgical instruments and variant creutzfeldt-jakob disease. 199910577672
big decisions based on small numbers: lessons from bse.the epidemic of bovine spongiform encephalopathy (bse) has been the most expensive disaster ever to have befallen farming in the uk. it is believed to have led to a new form of spongiform encephalopathy in humans and as yet there is no way of knowing how many people will die of this disease. in order to curtail the bse epidemic major decisions had to be made, often on the basis of inadequate scientific data. these data may have been derived from experiments using small sample numbers. here we re ...199910427633
molecular biology of prion propagation.the occurrence of new variant creutzfeldt-jakob disease and the experimental confirmation that it is caused by the same prion strain as bse has dramatically highlighted the need for a precise understanding of the molecular basis of prion propagation. the molecular basis of prion-strain diversity, previously a major challenge to the protein-only model, is now becoming clearer. the conformational change thought to be central to prion propagation, from a predominantly alpha-helical fold to one pred ...199910377292
prevention of transfusion-transmitted cytomegalovirus infection.cytomegalovirus (cmv) is a double-stranded dna virus which can be transmitted by blood transfusion. its seroprevalence in adults ranges from 40% to 100% depending on geographical and socioeconomic conditions. seropositive individuals have latent cmv infection with viral dna present in peripheral blood leucocytes. cmv can be associated with considerable morbidity and mortality in susceptible individuals, e.g. cmv-seronegative bone marrow allograft patients. evidence, from a number of reports, sug ...199910354380
[prion biology: update].the word "prion" was created in 1982 to name the etiological agent of the transmissible spongiform encephalopathies (tse), a group of degenerative diseases affecting central nervous system of man and animals, including bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd). prions present two isoforms: prpc, cellular or normal, which exists in all vertebrates and is sensitive to detergents and proteases, and prpsc, disease associated, partially resistant. the molecul ...199910615684
new variant creutzfeldt-jakob disease.it is now recognised that new variant creutzfeldt-jakob disease (cjd) can present during adolescence, so it may be within the experience of any paediatrician. some observations on prion proteins are made, and some of the features of classical cjd are reviewed. reports of patients with new variant cjd are given. the possible links between this condition and bovine spongiform encephalopathy (bse) are considered, especially the finding that certain people who are homozygous for methionine at codon ...199910622074
the transmission of prions to humans.the identification of new-variant creutzfeldt-jakob disease (nvcjd) in 1996 led to the proposal that this new disease was caused by the transmission of bovine spongiform encephalopathy (bse) to the human population. the ramifications of such a proposal have been extensive and profound, both politically and on the general public in the uk and other countries. patients with nvcjd exhibit a consistent set of clinicopathological features, and cases of nvcjd continue to be reported almost exclusively ...199910626542
creutzfeldt-jakob disease, new variant creutzfeldt-jakob disease, and bovine spongiform encephalopathy.creutzfeldt-jakob disease (cjd) is a subacute spongiform encephalopathy (sse) that is manifested by a variety of neurologic signs that usually include dementia, myoclonus, and an abnormal electroencephalogram (eeg). in 1996, a new variant of cjd (nvcjd) with a somewhat distinctive clinical presentation and neuropathology was reported in adolescents and young adults, a cohort of patients not normally affected with cjd. the appearance of nvcjd coincided temporally and geographically with the emerg ...199910517931
pathogenesis of the oral route of infection of mice with scrapie and bovine spongiform encephalopathy agents.transmissible spongiform encephalopathies can be transmitted via the oral route. the understanding of this mode of contamination has become a major issue since it is responsible for the appearance of bovine spongiform encephalopathy (bse) and is probably implicated in new variant creutzfeldt-jakob disease. in this study, we addressed the questions of the propagation pathway and the strain specificity of the pathogenesis of oral contamination of mice with the c506m3 scrapie strain and the 6pb1 bs ...199910580067
[human prion diseases].the interest in prion diseases, particularly the creutzfeldt-jakob type (cjd), rose dramatically in the last years for two reasons. 1) the general public wants to know whether eating beef may cause cjd. discovering the new variant creutzfeldt-jakob disease (nvcjd) and experimental evidence that nvcjd and bovine spongiforme encephalopathy (bse) are caused by the same prion strain make this idea probable. 2) infectiologists and neuroscientists recognise a model disease for a new infectious princip ...199910596282
quantitative analysis of mri signal intensity in new variant creutzfeldt-jakob disease.high signal intensity within the posterior thalamus (pulvinar nucleus) has been noted on mri in patients with new variant creutzfeldt-jakob disease (nvcjd). in this study mri examinations from three patients with proven nvcjd were compared with mri examinations from a control group of 14 age-matched subjects with no neurological abnormalities. mean signal intensity from seven target areas (periaqueductal tissue, posterior thalamus, dorsomedial thalamus, anterior thalamus, putamen, caudate head a ...199910624339
prions and the ent surgeon.recent developments in our understanding of prion diseases have raised concerns for the public health. there is now compelling evidence that the transmissible agent for variant creutzfeldt-jakob disease (vcjd) in affected individuals is accumulated in lymphoreticular tissues such as the appendix and tonsils. this agent demonstrates a remarkable resistance to standard methods of sterilisation used in hospital sterile services departments. the possible implications this has on the safety of surgic ...199910767916
antemortem diagnosis of variant creutzfeldt-jakob disease. 19999923866
variant creutzfeldt-jakob disease is not related to underlying iga deficiency. 199910583891
contaminated surgical instruments and variant creutzfeldt-jakob disease. 199910577671
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