| iatrogenic and zoonotic creutzfeldt-jakob disease: the australian perspective. | the transmissible brain diseases of humans and animals, the spongiform encephalopathies, continue to stimulate interest, and the announcement that exposure to "mad cow disease" (bovine spongiform encephalopathy [bse]) is a possible explanation for more than 10 cases of a variant creutzfeldt-jakob disease in humans in the united kingdom is a recent example. cases of iatrogenic creutzfeldt-jakob disease (from previous use of human cadaveric tissues for pituitary hormone therapy and neurosurgical g ... | 1996 | 8637463 |
| bovine spongiform encephalopathy and creutzfeldt-jakob disease: implications for physicians. | the appearance of bovine spongiform encephalopathy (bse) followed by new spongiform encephalopathies and variant creutzfeldt-jakob disease (cjd) in the united kingdom indicates that these diseases may be linked. to give an understanding of this risk, the authors review the literature on the pathogenesis of cjd and bse and the current findings on how these diseases are transmitted. they also discuss the implications for canada's food and blood supply and outline previously published recommendatio ... | 1996 | 8804258 |
| [the prion diseases]. | prions (proteinaceous infectious particles) are responsible to subacute spongiform encephalopathies (sse) in man and animals. recent outbreak of bovine sse (bse), or mad cow disease in uk provoked concerns on its possible human hazards. a statement of the british government in march 1996 upset the world, which was based on 10 cases of "new variant" form of creutzfeldt-jakob disease (cjd). prion diseases in animals are often epizootic and may be spread to different species through various routes ... | 1997 | 9086360 |
| [epidemiology of human prion diseases]. | prions(proteinaceous infectious particles) are responsible to subacute spongiform encephalopathies(sse) in man and animals. recent outbreak of bovine sse(bse), or mad cow disease in uk provoked concerns on its possible human hazards. a statement of the british government in march 1996 upset the world, which was based on 10 cases of "new variant" form of creutzfelds-jakob disease(cjd). prion diseases in animals are often epizootic and may be spread to different species through various routes incl ... | 1997 | 9103905 |
| [creutzfeldt-jakob disease; one year later]. | during the last year the knowledge of the transmission of prion diseases has increased. new diagnostic methods were developed: investigation of cerebrospinal fluid for the 14-3-3 protein and tonsillar biopsy to detect protease resistant prion protein. indirect evidence of a causal relation between new variant creutzfeldt-jakob disease (nvcj) and bovine spongiform encephalopathy (bse) is accumulating, although epidemiological data do not indicate that the incidence of cj is increasing. | 1997 | 9190535 |
| emerging and reemerging infections. progress and challenges in the subspecialty of infectious disease pathology. | emerging and reemerging infections are attracting greater attention from the public health and medical communities. pathologists and other physicians are increasingly aware of the importance of the subspecialty of infectious disease pathology as a tool for diagnosis, surveillance, and research of emerging infections. in this communication, we describe the role that infectious disease pathologists have played during the last 2 years in broadening our understanding of selected emerging infections, ... | 1997 | 9278604 |
| human prion diseases and bovine spongiform encephalopathy (bse). | prion diseases are transmissible neurodegenerative disorders which affect a range of mammalian species. in humans they can be inherited and sporadic as well as acquired by exposure to human prions. prions appear to be composed principally of a conformational isomer of host-encoded prion protein and propagate by recruitment of cellular prion protein. recent evidence argues that prion protein can also encode disease phenotypes by differences in its conformation and glycosylation. such molecular an ... | 1997 | 9300662 |
| new variant creutzfeldt-jakob disease: neurological features and diagnostic tests. | in april, 1996, ten cases of creutzfeldt-jakob disease (cjd) with an apparently new clinicopathological phenotype were published and it was suggested that these new variant cases (nvcjd) might be causally linked to bovine spongiform encephalopathy (bse). there have now been 21 cases of nvcjd in the uk and one case in france. we report clinical features and diagnostic test results of the first 14 cases of nvcjd in the uk. | 1997 | 9314867 |
| new variant creutzfeldt-jakob disease: psychiatric features. | an apparently new variant of creutzfeldt-jakob disease (cjd), new variant cjd (nvcjd), was identified in the uk in 1996. there have now been 21 cases of nvcjd in the uk and one in france. psychiatric symptoms are prominent in the initial presentation in these cases. | 1997 | 9314868 |
| a prion primer. | by biological and medical criteria, prions are infectious agents; however, many of their properties differ profoundly from those of conventional microbes. prions are "encoded" by alterations in protein conformation rather than in nucleic acid or amino acid sequence. new epidemic prion diseases (bovine spongiform encephalopathy and new variant creutzfeldt-jakob disease) have recently emerged under the active surveillance of the modern world. the risk of contracting prion disease from blood produc ... | 1997 | 9371069 |
| bovine spongiform encephalopathy and early onset variant creutzfeldt-jakob disease. | transmissible spongiform encephalopathies affect a variety of vertebrates, including humans. while scrapie has been enzootic in sheep for centuries, bovine spongiform encephalopathy (bse) appeared only some 12 years ago but rapidly became epizootic. it is not clear whether bse originated in cattle as a rare spontaneous event or whether it stems from sheep, but its spread is clearly due to feeding of cattle-derived contaminated bone and meat meal. recent evidence links the appearance of new varia ... | 1997 | 9384548 |
| new-variant creutzfeldt-jakob disease and treatment of haemophilia. executive committee of the ukhcdo. united kingdom haemophilia centre directors' organisation. | | 1997 | 9400534 |
| new variant creutzfeldt-jakob disease and bovine pituitary growth hormone. | | 1998 | 9439502 |
| the spectrum of transmissible spongiform encephalopathies. | since the first description by a.m. jakob and h.g. creutzfeldt, five human diseases have been identified as transmissible spongiform encephalopathies (tse). the disease bearing these authors' name, creutzfeldt-jakob disease (cjd) occurs sporadically, may be transmitted and has a genetic basis in 10-15% of all cases. genetic diseases are the gerstmann-sträussler-scheinker syndrome and fatal familial insomnia. the latest form of cjd in humans, variant cjd (vcjd), was first described in 1996 and ma ... | 1997 | 9450236 |
| new variant creutzfeldt-jakob disease and bovine spongiform encephalopathy. | new variant creutzfeldt-jakob disease (cjd) and bovine spongiform encephalopathy (bse) are invariably fatal, subacute degenerative diseases of the brain that are classified as transmissible spongiform encephalopathies. bse was first diagnosed in 1986 as part of an ongoing epizootic in the united kingdom that was amplified by the feeding of rendered bovine meat-and-bone meal to young calves. as of june 1997, a total of 17 cases of new variant cjd have been reported among residents of the united k ... | 1998 | 9494833 |
| use of competing conceptions of risk in animal agriculture. | this study considers a theory of risk as a means of coping with risk and uncertainty that have become a growing reality for animal agriculture. microbial contaminations of food, waste management, animal products in the human diet, and transmissible spongiform encephalopathies (tse) incorporate different conceptions of risk and require different approaches to handling the uncertainty involved. a dichotomous schema is suggested to assist understanding risk that may be adapted to recognizing and ha ... | 1998 | 9535327 |
| new variant creutzfeldt-jakob disease. | since the report of new variant creutzfeldt-jakob disease (nvcjd) in humans last year, the search was on for direct evidence to link the condition to bovine spongiform encephalopathy (bse). the first case nvcjd was noted 10 years after the recognition of bse in uk cattle. a direct link is now established. there are, however, some 'protective' mechanisms, the most important of which are the inefficiency of the gastric route of introducing the infected material, the species barrier and genetic 'pr ... | 1998 | 9562257 |
| new variant creutzfeldt-jakob disease. | new variant creutzfeldt-jakob disease is a novel human prion disorder with characteristic clinical and neuropathological features, which results from exposure to the bovine spongiform encephalopathy agent. the probably lengthy incubation period makes it difficult to predict future new variant creutzfeldt-jakob disease case numbers; further studies are required to clarify risk factors and the potential for human spread. | 1998 | 9642546 |
| neuropathological findings in new variant cjd and experimental transmission of bse. | the diagnosis of new variant creutzfeldt-jakob disease is dependent on the neuropathological examination of brain tissue following brain biopsy or autopsy. the characteristic neuropathological features are multiple 'florid' plaques in the cerebral and cerebellar cortex, spongiform change most marked in the basal ganglia, severe thalamic gliosis and marked accumulation of the disease-associated prion protein in diffuse or pericellular deposits in the cerebrum and cerebellum. these features allow ... | 1998 | 9684997 |
| the new variant form of creutzfeldt-jakob disease. | a new phenotype of creutzfeldt-jakob disease termed new variant creutzfeldt-jakob disease (nvcjd) was first described in march 1996. this differs from other forms of cjd in terms of its epidemiology, clinical features and neuropathology. to date 24 cases of this new form of cjd have been described, 23 within the uk. this article describes nvcjd discussing clinical and epidemiological features and discusses possible links with the bovine spongiform encephalopathy epidemic in cattle in the uk. | 1998 | 9684998 |
| 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. | 1998 | 9737381 |
| phenotype-genotype studies in kuru: implications for new variant creutzfeldt-jakob disease. | the prnp polymorphic (methionine/valine) codon 129 genotype influences the phenotypic features of transmissible spongiform encephalopathy. all tested cases of new variant creutzfeldt-jakob disease (nvcjd) have been homozygous for methionine, and it is conjectural whether different genotypes, if they appear, might have distinctive phenotypes and implications for the future "epidemic curve" of nvcjd. genotype-phenotype studies of kuru, the only other orally transmitted transmissible spongiform enc ... | 1998 | 9789072 |
| [prionoses--neurodegenerative diseases caused by prions, offectious proteinaceous molecules]. | prionoses are a group of human and animal neurodegenerative diseases caused by prions, infectious pathogens that differ from bacteria, fungi, parasites, viroids, and viruses. despite intensive searches over the past three decades, no nucleic acid has been found within prions and considerable experimental data argue that prions are composed exclusively of proteins (glycoproteins). normal prion protein (prpc) is encoded by a gene present in all nuclear cells of humans and other mammals but is cons ... | 1998 | 9810774 |
| prp expression in b lymphocytes is not required for prion neuroinvasion. | prion diseases are typically initiated by infection of peripheral sites, as in the case of bovine spongiform encephalopathy, new variant creutzfeldt-jakob disease, kuru and most cases of iatrogenic creutzfeldt-jakob disease. in mouse scrapie, prion infectivity accumulates in lymphoid organs, and the absence of mature b lymphocytes prevents peripherally administered prions from inducing central nervous system disease. we have now assessed whether expression of the cellular prion protein, prpc, is ... | 1998 | 9846583 |
| epidemiological determinants of the pattern and magnitude of the vcjd epidemic in great britain. | understanding the epidemiology and aetiology of new-variant creutzfeldt-jakob (vcjd) disease in humans has become increasingly important given the scientific evidence linking it to bovine spongiform encephalopathy (bse) in cattle and hence the wide exposure of the population of great britain (gb) to potentially infectious tissue. the recent analysis undertaken to determine the risk to the population from dorsal route ganglia illustrated the danger in presenting point estimates rather than ranges ... | 1998 | 9921684 |
| 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 ... | 1999 | 9923873 |
| 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 ... | 1999 | 9934675 |
| new-variant creutzfeldt-jakob disease: the risk of transmission by blood transfusion. | new-variant creutzfeldt-jakob disease (nvcjd) was first described in the uk in 1996 and is thought to be related to the consumption of cattle suffering from bovine spongiform encephalopathy. although only 29 cases have been confirmed to date, it is too early to predict the number of people who may currently be incubating the disease. past experience suggests that sporadic cjd is rarely, if ever, spread by blood transfusion. however, it is unclear whether nvcjd may be transmissible by this route ... | 1998 | 9950096 |
| 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. | 1999 | 10023945 |
| prion diseases in man. | prion diseases are uncommon fatal neurodegenerative disorders which have gained scientific and public importance as a result of major advances in the understanding of the nature of the causative agent, and the emergence of new forms of these diseases in both animals and man. the transmissible agent in prion diseases is unique and is closely associated with an abnormal isoform of a widely distributed cell-surface glycoprotein, prion protein. the precise mechanisms of conversion to the abnormal is ... | 1998 | 10211109 |
| new variant creutzfeldt-jakob disease (nvcjd): the risk of transmission by blood transfusion and the potential benefit of leukocyte-reduction of blood components. | | 1999 | 10218230 |
| 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. | 1999 | 10221162 |
| 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 ... | 1999 | 10318733 |
| bovine spongiform encephalopathy and new variant creutzfeldt-jakob disease. | bovine spongiform encephalopathy (bse) and creutzfeldt-jakob disease (cjd) belong to a group of degenerative neurological disorders collectively known as the transmissible spongiform encephalopathies (tses). the group also includes scrapie of sheep and goats, kuru of humans, chronic wasting disease of mule deer and elk and transmissible encephalopathy of mink. these fatal diseases cause behavioural changes, alterations of sensation, changes in mental state and ataxia. the typical pathology is no ... | 1998 | 10326298 |
| 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 ... | 1999 | 10354380 |
| 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 ... | 1999 | 10377292 |
| 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. | 1999 | 10386559 |
| 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 ... | 1999 | 10394138 |
| 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 ... | 1999 | 10427633 |
| 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. ... | 1999 | 10440324 |
| tragedy of variant creutzfeldt-jakob disease. | | 1999 | 10459895 |
| deaths from variant creutzfeldt-jakob disease. | | 1999 | 10459909 |
| 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 ... | 1999 | 10462888 |
| 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 ... | 1999 | 10517931 |
| contaminated surgical instruments and variant creutzfeldt-jakob disease. | | 1999 | 10577672 |
| 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 ... | 1999 | 10580067 |
| [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 ... | 1999 | 10596282 |
| [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 ... | 1999 | 10615684 |
| 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 ... | 1999 | 10622074 |
| 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 ... | 1999 | 10626542 |
| 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. | 2000 | 10627667 |
| 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). | 2000 | 10675119 |
| variant creutzfeldt-jakob disease: an update. | | 2000 | 10748782 |
| 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 ... | 2000 | 10823860 |
| 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. | 2000 | 10870113 |
| 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 ... | 2000 | 10871542 |
| 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 ... | 2000 | 10878555 |
| variant creutzfeldt-jakob disease. | | 2000 | 10914416 |
| 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 ... | 2000 | 10931212 |
| 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 ... | 2000 | 10936691 |
| 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). | 2000 | 10938959 |
| [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 ... | 2000 | 10951677 |
| 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. | 2000 | 10953096 |
| 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 ... | 2000 | 10953401 |
| variant creutzfeldt-jakob disease and the quebec blood supply. | | 2000 | 10976257 |
| 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 ... | 2000 | 10981894 |
| 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 ... | 2000 | 10991726 |
| variant creutzfeldt jakob disease. | | 2000 | 11023754 |
| 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 ... | 2000 | 11041403 |
| 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 ... | 2000 | 11054179 |
| 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 ... | 2000 | 11055271 |
| 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 ... | 2000 | 11061795 |
| 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 ... | 2000 | 11087170 |
| 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 ... | 2000 | 11087171 |
| 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 ... | 2001 | 11120925 |
| [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. ... | 2000 | 11143292 |
| 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 ... | 2000 | 11189730 |
| 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, ... | 2000 | 11190118 |
| 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 ... | 2000 | 11214913 |
| 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 ... | 2000 | 11214917 |
| 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. ... | 2000 | 11214918 |
| 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 ... | 2001 | 11248018 |
| 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 ... | 2001 | 11259641 |
| 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 ... | 2001 | 11266289 |
| [confusion surrounding bovine spongiform encephalopathy (bse) and the risk of new variant creutzfeldt-jakob disease]. | | 2001 | 11268917 |
| the emerging european epidemic of variant creutzfeldt-jakob disease and bovine spongiform encephalopathy: lessons for australia. | | 2001 | 11270752 |
| 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 ... | 2001 | 11283677 |
| 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 ... | 2001 | 11283678 |
| bovine spongiform encephalopathy and variant creutzfeldt-jakob disease. | | 2001 | 11290640 |
| 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. | 2001 | 11293592 |
| [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 ... | 2001 | 11294108 |
| 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) ... | 2001 | 11303793 |
| 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 ... | 2001 | 11328566 |
| 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 ... | 2001 | 11329135 |
| 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 ... | 2001 | 11343744 |
| 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 ... | 2001 | 11353827 |
| 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 ... | 2001 | 11385008 |
| 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 ... | 2001 | 11404459 |
| 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 ... | 2001 | 11405946 |
| 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 ... | 2001 | 11432823 |