Publications

prion diseases as transmissible zoonotic diseases.	prion diseases, also called transmissible spongiform encephalopathies (tses), lead to neurological dysfunction in animals and are fatal. infectious prion proteins are causative agents of many mammalian tses, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (bse; in cattle), and creutzfeldt-jakob disease (cjd; in humans). bse, better known as mad cow disease, is among the many recently discovered zoonotic diseases. bse cases were first repo ...	0	24159531
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
cerebrospinal-fluid test for new-variant creutzfeldt-jakob disease.		1996	8843819
[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
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
florid plaques and new variant creutzfeldt-jakob disease.		1997	9371186
is the neuropathology of new variant creutzfeldt-jakob disease and kuru similar?		1997	9250194
diagnosis of new variant creutzfeldt-jakob disease by tonsil biopsy.		1997	9217739
diagnosis of new variant creutzfeldt-jakob disease by tonsil biopsy.		1997	9142082
diagnosis of new variant creutzfeldt-jakob disease by tonsil biopsy.		1997	9142081
reporting of suspect new variant creutzfeldt-jakob disease.		1997	9121262
diagnosis of new variant creutzfeldt-jakob disease by tonsil biopsy.		1997	8996424
new variant creutzfeldt-jakob disease in france.		1997	8988126
creutzfeldt-jakob disease, new variant creutzfeldt-jakob disease and bovine spongiform encephalopathy - an update.		1997	22514471
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
new variant creutzfeldt-jakob disease and bovine pituitary growth hormone.		1998	9439502
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
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
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
bovine spongiform encephalopathy statement of possible relation with new variant creutzfeldt-jakob disease: effects on the welfare of united kingdom cattle.	although measures to control bovine spongiform encephalopathy (bse) had been in force in the united kingdom for many years and had resulted in a marked decline in clinical cases, the announcement by the secretary of state for health on march 20, 1996, that a new variant form of creutzfeldt-jakob disease may be linked with exposure to bse, resulted in the introduction of several new control measures. these measures included a scheme banning human consumption of meat from cattle who were more than ...	1998	16363943
new variant creutzfeldt-jakob disease and the blood supply: is it time to face the music?		1998	9780967
prion immunoreactivity in appendix before clinical onset of variant creutzfeldt-jakob disease.		1998	9728989
diagnosis of creutzfeldt-jakob disease by measurement of s100 protein in serum. tonsil biopsy helps diagnose new variant creutzfeldt-jakob disease.		1998	9758471
new variant creutzfeldt-jakob disease is more common in britain than elsewhere.		1998	9685298
new-variant creutzfeldt-jakob disease and treatment of haemophilia. executive committee uk haemophilia directors' organisation.		1998	9643779
single photon emission computed tomography in the identification of new variant creutzfeldt-jakob disease: case reports.		1998	9518911
early identification of variant creutzfeldt-jakob disease.		1998	9518890
new-variant creutzfeldt-jakob disease and treatment of haemophilia.		1998	9492811
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
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
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
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
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 ...	1999	10624339
variant creutzfeldt-jakob disease is not related to underlying iga deficiency.		1999	10583891
contaminated surgical instruments and variant creutzfeldt-jakob disease.		1999	10577671
new variant creutzfeldt-jakob disease and white cell reduction: risk assessment and decision making in the absence of data.		1999	10533815
the impact of new-variant creutzfeldt-jakob disease on blood transfusion practice.		1999	10519983
submicroscopic immunodetection of prp in the brain of a patient with a new-variant of creutzfeldt-jakob disease.	we analyzed the distribution and organization of the pathological prion protein isoform (prpsc) in the brain of new variant creutzfeldt-jakob disease using a sensitive post-embedding immunogold electron microscopy method. on methacrylate semithin sections, silver-prp staining showed florid plaques, containing microvacuoles. it also revealed scattered granular and perivacuolar deposits. at the electron microscope level, plaque prp-gold labeling was associated with filaments and flocculent amorpho ...	1999	10320013
organophosphate exposure and variant creutzfeldt-jakob disease.		1999	10227226
does variant creutzfeldt-jakob disease have an achilles heel?		1999	10217106
heightened intrathecal release of proinflammatory cytokines in creutzfeldt-jakob disease.	the authors report high intrathecal release of tumor necrosis factor alpha (tnf-alpha) and interleukin (il)-1beta in five patients with sporadic or new-variant creutzfeldt-jakob disease (cjd) without activation of the humoral or lymphocytic immune responses. increased release of tnf-alpha and il-1beta was also detected in some patients with progressive dementias. cjd is associated with a local cerebral host response that involves the release of proinflammatory cytokines.	1999	10214763
diagnosis of creutzfeldt-jakob disease. routine tonsil biopsy for diagnosis of new variant creutzfeldt-jakob disease is not justified.		1999	10024280
antemortem diagnosis of variant creutzfeldt-jakob disease.		1999	9923866
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 ...	1999	10767916
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).	2000	11693716
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