Publications

variant creutzfeldt-jakob disease (vcjd) and gastrointestinal endoscopy.	variant creutzfeldt-jakob disease (vcjd) is a transmissible form of spongiform encephalopathy believed to be contracted from the consumption of bovine spongiform encephalopathy (bse) infected beef products. to date over 100 individuals have developed this incurable disease. there have been no documented cases of iatrogenic infection, but there is a theoretical risk that surgical procedures could transmit the disease. this review describes the background of the disease and assesses the possible r ...	2001	11740649
transmissible spongiform encephalopathies: vaccine issues.	the recent emergence of bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) suggests that transmissible spongiform encephalopathies (tses) pose an ongoing threat to human and animal health. to avoid iatrogenic transmission of tses in vaccines, strategies must be developed to obviate tse agent infectivity in cellular substrates, cell culture media components and enzymes, and excipients, and to validate the safety of these components and field vaccines efficiently	2001	11761262
species differences in the blood content of the normal cellular isoform of prion protein, prp(c), measured by time-resolved fluoroimmunoassay.	the concern that variant creutzfeldt-jakob disease could be transmitted via blood transfusion has prompted studies of blood infectivity in animal models. as normal prion protein acts as a substrate for conversion to the abnormal form associated with infectivity, we have quantified its distribution in mice and hamsters, the most commonly used animal models.	2001	11903999
[prions, epidemic of creutzfeldt-jakob variant disease and global emergency].	we present here the current understanding of "prion" theory and global risk for epidemics of variant creutzfeldt-jakob disease (vcjd). prion is the infectious agent of all transmissible spongiform encephalopaties (tses). it is regarded as an aggregate of a pathological conformer (prpsc) of a normal cellular glycoprotein (prpc) encoded by a gene, in humans on chromosome 20. the differences between prpsc and prpc are largely if not exclusively conformational; prpc is mostly alpha-helical while prp ...	2001	11873615
the transmissible spongiform encephalopathies: pathogenic mechanisms and strategies for therapeutic intervention.	primary neurodegenerative diseases tend to be intractable and largely affect the elderly. there is rarely the opportunity to identify individuals at risk and the appearance of clinical symptoms usually signifies the occurrence of irreversible neurological damage. this situation describes sporadic creutzfeldt-jakob disease which occurs world-wide, affecting one person per million per annum. the epidemic of bovine spongiform encephalopathy in the uk in the 1980s and the subsequent causal appearanc ...	2001	12540284
differential diagnosis of infections with the bovine spongiform encephalopathy (bse) and scrapie agents in sheep.	scrapie, bovine spongiform encephalopathy (bse), and variant creutzfeldt-jakob disease belong to the group of disorders called transmissible spongiform encephalopathies or prion diseases. the possibility that some sheep may be infected with the bse agent is of human and animal health concern. immunohistochemical methods were used to identify specific prion protein (prp) peptide sequences in specific cell types of the brain and lymphoreticular system (lrs) of sheep with natural scrapie and suffol ...	2001	11798244
pediatric adenoidectomy under vision using suction-diathermy ablation.	to compare adenoidectomy using suction-diathermy ablation with adenoidectomy by way of curettage in a pediatric tertiary care setting.	2001	11802020
transmissible spongiform encephalopathies in australia.	the australian national creutzfeldt-jakob disease registry (ancjdr) commenced surveillance in september 1993 as part of the commonwealth's response to 4 cases of pituitary hormone (gonadotrophin)-associated creutzfeldt-jakob disease (cjd). with the passage of time, the registry has become responsible for ascertaining all human transmissible spongiform encephalopathies (tse; also known as prion diseases) within australia since 1970. included in the spectrum of diseases monitored are classical (sp ...	2001	11806657
odyssey toward a healthier world.	the 22nd international congress of chemotherapy was held in amsterdam, the netherlands, july 14, 2001. the congress attracted participants from around the world and covered a broad spectrum of work on microbial infections and cancer, their treatment by antiinfective drugs and their prevention by vaccination. a theme of the congress was "compassion and science," and this was picked up in a fascinating albeit slightly controversial symposium on "health, human rights and infection." there were seve ...	2001	12813588
use of a marker organism to model the spread of central nervous system tissue in cattle and the abattoir environment during commercial stunning and carcass dressing.	due to concerns about a link between variant creutzfeldt-jakob disease in humans and similar prion protein-induced disease in cattle, i.e., bovine spongiform encephalopathy (bse), strict controls are in place to exclude bse-positive animals and/or specified risk materials including bovine central nervous system (cns) tissue from the human food chain. however, current slaughter practice, using captive bolt guns, may induce disruption of brain tissues and mobilize cns tissues into the bovine circu ...	2002	11823220
rapid typing of the codon 129 polymorphism of the human prion protein gene by combined real-time pcr and melting curve analysis.	homozygosity of methionine (m/m) at amino acid residue 129 (codon 129) of the human prion protein (prp) has been reported for all so far analyzed cases of the new variant creutzfeldt-jakob disease (vcjd). this contrasts with its general distribution in the healthy caucasian population of only about 43%. for this reason a predisposition for carriers of the corresponding genotype to develop vcjd after infection with the bovine spongiform encephalopathy (bse) agent is assumed, and pcr based methods ...	2002	11833672
[new variant creutzfeldt-jakob disease].		2002	11845390
new variant cjd-bse (mad cow disease). the need for disposable ent instruments.	this paper outlines the development of bovine spongiform encephalopathy (bse) in the united kingdom. the relationship between bse and new variant creutzfeldt-jakob disease (vcjd) is considered and the risks of iatrogenic spread reviewed. the rationale for disposable surgical instruments in adenotonsillectomy to prevent iatrogenic spread is discussed.	2002	11852121
possible underascertainment of variant creutzfeldt-jakob disease: a systematic study.	to predict the size of the vcjd epidemic it is important to know whether the description of cases of vcjd in 1996 represent the first cases of a new disease entity or whether detection was due to increased surveillance of cjd in humans. detection of earlier cases would suggest a shorter incubation period and might lead to predictions of epidemic size being revised.	2002	11861685
neuropathology of variant creutzfeldt-jakob disease.	the neuropathological features human prion diseases comprise spongiform change, neuronal loss, astrocytic and microglial proliferation and the accumulation of the abnormal isoform of prion protein (prpres) in the central nervous system. variant creutzfeldt-jakob disease (cjd) is a novel human prion disease which appears to result from infection by the bovine spongiform encephalopathy (bse) agent. the neuropathology of variant cjd shows morphological and immunocytochemical characteristics distinc ...	2002	11862618
predicting the size of the vcjd epidemic in france.	more than 5 years after the description of the first cases of variant creutzfeldt-jakob disease (vcjd), there is still great uncertainty about the size of the vcjd epidemic in the united kingdom (uk), although the most recent predictions based on statistical modelling are more optimistic than the previous ones. the number of vcjd cases in france is far too small to attempt any direct modelling of the vcjd epidemic in the french population. comparative assessment of the level of exposure to the b ...	2002	11862620
implications of prion-induced diseases for animal-derived pharmaceutical products.	the implications of prion-induced diseases for the use of medications that theoretically could harbor the infectious pathogens are discussed. prions have been identified as protein particles that lack nucleic acids. there is evidence that prions cause the transmissible neurodegenerative diseases known as transmissible spongiform encephalopathies. of these diseases, bovine spongiform encephalopathy (bse) and the human spongiform encephalopathy to which it has been linked, new variant creutzfeldt- ...	2002	11862637
risks of bovine spongiform encephalopathy and variant creutzfeldt-jakob disease in the united states.		2002	11898487
[prions as the driving force in transmissible spongiform encephalopathies].	transmissible spongiform encephalopathies are degenerative disorders affecting the central nervous system (cns) occurring in a variety of species. the causative agent is thought to be composed of an abnormal form of the host encoded prion protein (prpc), termed prpsc. the conformational change of prpc into prpsc can occur spontaneously, however, it can also be induced by prpsc. prion diseases such as bovine spongiform encephalopathy (bse), scrapie and variant creutzfeldt-jakob-disease (vcjd) are ...	2002	12585203
prions in skeletal muscle.	considerable evidence argues that consumption of beef products from cattle infected with bovine spongiform encephalopathy (bse) prions causes new variant creutzfeldt-jakob disease. in an effort to prevent new variant creutzfeldt-jakob disease, certain "specified offals," including neural and lymphatic tissues, thought to contain high titers of prions have been excluded from foods destined for human consumption [phillips, n. a., bridgeman, j. & ferguson-smith, m. (2000) in the bse inquiry (statio ...	2002	11904434
the epidemiology of variant creutzfeldt-jakob disease in europe.	variant creutzfeldt-jakob disease is one of a family of neurodegenerative diseases, first diagnosed in 1996. scientific evidence strongly supports the hypothesis that it is acquired through consumption of bovine spongiform encephalopathy-infected meat. the majority of cases have been diagnosed in the uk in young individuals, with an excess of cases in the north and a significant cluster of cases in leicestershire. many uncertainties in its biology and epidemiology, in particular the length of th ...	2002	11909749
alpha-synuclein-immunoreactive deposits in human and animal prion diseases.	prion related disorders are associated with the accumulation of a misfolded isoform (prpsc) of the host-encoded prion protein, prp. there is strong evidence for the involvement of unidentified co-factors in the prp to prpsc conversion process. in this study, we show alpha-synuclein-immunoreactive deposits in the central nervous system of various prion diseases (sporadic, iatrogenic and new variant creutzfeldt-jakob diseases, and experimental scrapie of hamsters). alpha-synuclein accumulated clos ...	2002	11935269
profound sex-specific effects on incubation times for transmission of bovine spongiform encephalopathy to mice.	four strains of mice were inoculated intracerebrally with a primary isolate of bovine spongiform encephalopathy (bse) and the cloned mouse-adapted scrapie strain me7. clinical prion disease diagnosis was made at the appearance of three or more neurological symptoms and their persistence for 3 consecutive weeks and confirmed by neuropathological criteria. for bse, incubation periods were profoundly different between the sexes in all four mouse strains, being longer in the females. in contrast, me ...	2002	11937772
chronic wasting disease in deer and elk in north america.	chronic wasting disease (cwd) has emerged as an important disease of wildlife in north america. the disease is a unique member of the transmissible spongiform encephalopathies (tses) or prion diseases, which naturally affect only a few species. of the tses, cwd is the only one found in free-ranging species. however, interest in cwd has recently grown, by association with the better-known tses such as variant creutzfeldt-jakob disease of humans and bovine spongiform encephalopathy. knowledge of t ...	2002	11974617
14-3-3 in the cerebrospinal fluid of patients with variant and sporadic creutzfeldt-jakob disease measured using capture assay able to detect low levels of 14-3-3 protein.	a protein capture assay was used to measure 14-3-3 (-isoform) in the cerebrospinal fluid (csf) of patients with either variant or sporadic creutzfeldt-jakob disease (cjd). the results were compared with those obtained using western blotting. elevated levels of 14-3-3 were found in 58% of variant cjd (vcjd) patients and 82% of sporadic cjd (spcjd) patients using the protein capture assay. using a western blotting technique, the presence of csf 14-3-3 was detected in 58% of vcjd patients and in 89 ...	2002	11983294
food safety: bovine spongiform encephalopathy (mad cow disease).	bovine spongiform encephalopathy is just one of a group of diseases known as transmissible spongiform encephalopathies. only recently has it become recognized that transmissible spongiform encephalopathies are likely due to proteins known as prions. although it has been recognized that transmissible spongiform encephalopathies may readily spread within species, the recent observations that bovine spongiform encephalopathy in cattle may have originated from another transmissible spongiform enceph ...	2002	11984426
[the risk of variant creutzfeldt-jakob disease in the netherlands and the effect of preventive measures].	variant creutzfeldt-jakob disease (vcjd) is a fatal and untreatable neurological disease, in which pathogenic prions (prpsc) are involved. there is convincing epidemiological and experimental evidence that vcjd is a human expression of bovine spongiform encephalopathy (bse). the risk of transmission of pathogenic prions which cause vcjd to humans is influenced by the species barrier, genetic susceptibility of the host, dose of infection and route of exposure. transmission of pathogenic prions fr ...	2002	11998352
haemophilia 2002: emerging risks of treatment.	haemophilia care and treatment products have greatly improved over the past 2 decades. transitions in treatment produced by these changes were accompanied by the emergence of unexpected risks and new complications. in order to provide the best comprehensive care to patients with haemophilia, healthcare providers periodically need to re-evaluate and adjust their management and therapeutic products to prevent or minimize the effects produced by the emerging issues. for example, reducing the effect ...	2002	12010415
risk of variant creuzfeldt-jakob disease from factor concentrates: current perspectives.	the demonstration of iatrogenic transmission of creuzfeldt-jakob disease (cjd) through therapeutic interventions led to substantial concerns in communities requiring blood products in the 1980s and 1990s. these concerns led some regulatory authorities to adopt a very precautionary approach and require recall of plasma products, including factor concentrates, which included donors at risk of cjd. the fda's approach on recall contributed to a substantial lack of plasma products on the world market ...	2002	12010416
bse and variant creutzfeldt-jakob disease: never say never.		2002	12012095
bovine spongiform encephalopathy update.	bovine spongiform encephalopathy (bse) is a zoonosis being the origin of variant creutzfeldt-jakob disease and an important cattle disease in its own right. countries have been slow to learn the importance of protecting, not only their cattle populations, but also their human populations. since 2000, several additional european countries have reported bse in native-born stock and this has led to a concern about the bse status of countries that have imported cattle and catlle products from infect ...	2002	12014225
prnp contains both intronic and upstream regulatory regions that may influence susceptibility to creutzfeldt-jakob disease.	the prion protein (prp) plays a central role in creutzfeldt-jakob disease (cjd) and other transmissible spongiform encephalopathies (tses). mutations in the protein coding region of the human prp gene (prnp), which have been proposed to alter the stability of the prp protein, have been linked to a number of forms of tse. however, the majority of cjd cases are not associated with mutations in the prnp coding region and alternative mechanisms must therefore underlie susceptibility to these forms o ...	2002	12034503
neurological adverse events associated with vaccination.	public tolerance to adverse reactions is minimal. several reporting systems have been established to monitor adverse events following immunization. the present review summarizes data on neurologic complications following vaccination, and provides evidence that indicates whether they were directly associated with the vaccines. these complications include autism (measles vaccine), multiple sclerosis (hepatitis b vaccine), meningoencephalitis (japanese encephalitis vaccine), guillain-barré syndrome ...	2002	12045734
follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions.	infectious prion diseases initiate infection within lymphoid organs where prion infectivity accumulates during the early stages of peripheral infection. in a mouse-adapted prion infection, an abnormal isoform (prp(sc)) of prion protein (prp) accumulates in follicular dendritic cells within lymphoid organs. human prions, however, did not cause an accumulation of prp(sc) in the wild type mice. here, we report that knock-in mouse expressing humanized chimeric prp demonstrated prp(sc) accumulations ...	2002	12051707
[transmission of spongiform encephalopathies (prion diseases)].	the transmissible spongiform encephalopathies (tse), or prion diseases, constitute a form of degenerative disorders of the central nervous system, which are characterized by a typical spongiform histological pattern and a fatal course. according to prusiner's theory, its agent consists of a protein without any nucleic acid, the "proteinaceous infectious agent", or prion. this is a pathologically folded form of the normal prion protein (prpc), and then called prpsc. tse are observed in different ...	2002	12063689
transmission of prion disease.	the transmission of bovine spongiform encephalopathy to humans as variant creutzfeldt-jakob disease (vcjd) has focused public attention on how prion diseases are transmitted and how prions reach the brain after exposure. prion diseases are characterised by transmissibility and neuropathological features of gliosis, neuronal loss and microscopic vacuoles, termed spongiosis. the principal component of prions is the glycoprotein prp(sc), which is a conformational modified isoform of the normal memb ...	2002	12064258
pathological diagnosis of variant creutzfeldt-jakob disease.	the neuropathological and biochemical features of the 89 histologically confirmed cases of variant creutzfeldt-jakob disease (vcjd) diagnosed up to the end of october 2001 in the uk are reviewed. histology of the central nervous system, lymphoid tissues and other organs was accompanied by immunocytochemistry and western blot analysis of the disease-associated form of the prion protein (prp(res)). all patients with vcjd were methionine homozygotes at codon 129 of the prp gene. the pathology of vc ...	2002	12064259
clinical diagnosis and differential diagnosis of cjd and vcjd. with special emphasis on laboratory tests.	the most widely distributed form of transmissible spongiform encephalopathy, sporadic creutzfeldt-jakob disease, typically affects patients in their sixties. rapidly progressive dementia is usually followed by focal neurological signs and typically myoclonus. the disease duration in sporadic cjd is shorter than in variant cjd (6 months and 14 months, respectively). the clinical diagnosis in sporadic cjd is supported by the detection of periodic sharp and slow wave complexes in the electroencepha ...	2002	12064260
current perspectives on bovine spongiform encephalopathy and variant creutzfeldt-jakob disease.	bovine spongiform encephalopathy (bse) clearly originated in the uk, where there have now been more than 180 000 cases. however, through the exportation of cattle and cattle-feed additives from the uk, bse also became established to a lesser extent in other european countries. there is current concern that bse might have been distributed more widely as a result of the exportation of cattle or bse-infected feed or foodstuff not only from the uk but also from other european countries that later be ...	2002	12084100
immunization delays the onset of prion disease in mice.	the outbreak of new variant creutzfeldt-jakob disease has raised the specter of a potentially large population being at risk to develop this prionosis. none of the prionoses currently have an effective treatment. recently, vaccination has been shown to be effective in mouse models of another neurodegenerative condition, namely alzheimer's disease. here we report that vaccination with recombinant mouse prion protein delays the onset of prion disease in mice. vaccination was performed both before ...	2002	12107084
molecular advances in understanding inherited prion diseases.	the prion diseases are neurodegenerative disorders that have attracted great interest because of the possible link between bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (ctd) in humans. possible transmission of these diseases has been linked to a single protein termed the prion protein. this protein is an abnormal isoform of a normal synaptic glycoprotein. the majority of prion diseases does not appear to be caused by transmission of an infectious agent but occur s ...	2002	12109876
comparative epidemiology of scrapie outbreaks in individual sheep flocks.	data recording the course of scrapie outbreaks in 4 sheep flocks (2 in cheviot sheep and 2 in suffolks) are compared. for each outbreak the data on scrapie incidence and sheep demography and pedigrees cover periods of years or decades. a key finding is that the incidence of clinical cases peaks in sheep 2-3 years old, despite very different forces-of-infection. this is consistent with age-specific susceptibility of sheep to scrapie, as has been reported for cattle to bovine spongiform encephalop ...	2002	12113497
vcjd: the epidemic that never was. possibility of bse being cause of variant cjd is indeed biologically plausible.		2002	12125684
implications of prion diseases for neurosurgery.	prion diseases comprise a group of diseases characterised by transmissibility, spongiosis, gliosis, neuronal loss, and accumulation of an abnormally folded membrane protein, prp(sc). infectivity resists almost all chemical and physical processes that inactivate conventional viruses, whereas protein extraction abolishes infectivity. this fact is of great importance to surgery, especially neurosurgery, since conventional cleaning of surgical instruments does not abolish infectivity. as a matter of ...	2002	12172722
[human prion diseases].	prion diseases are rare neurodegenerative transmissible fatal diseases affecting humans and mammals. the causative agent is a novel pathogen termed the prion. unlike classical infectious agents such as bacteria or viruses, prions lack an independent genome and consist largely of an abnormal form of the host-encoded prion protein. creutzfeldt-jakob disease (cjd) is the main representative of human prion diseases that may be sporadic in most cases, hereditary, or acquired. clinical examination yie ...	2002	12190050
apolipoprotein e and other cerebrospinal fluid proteins differentiate ante mortem variant creutzfeldt-jakob disease from ante mortem sporadic creutzfeldt-jakob disease.	the ability to perform an ante mortem differential diagnosis of creutzfeld-jakob disease (cjd) is aided by several clinical and molecular tests. there is a need for molecular tests which can reliably distinguish ante mortem variant cjd (vcjd) from ante mortem sporadic cjd (spcjd). a proteomics approach employing two-dimensional protein electrophoresis is applied to the study of ante mortem csf samples obtained in collaboration with the cjd surveillance unit and the national hospital for neurolog ...	2002	12210228
disease transmission by blood products: past, present and future.	transfusion of blood and blood products has been associated with transmission of infectious agents. however, it is probable that blood products are currently very safe and that pooled virus-inactivated products from remunerated donors are now safer than untreated single voluntary donor components. although the transmission events of the past and the present are reasonably well understood, reliance on a linear approach to predict safety in the future is open to criticism. indeed, it was not possi ...	2002	12214137
epidemiology of variant cjd.	there are 100 confirmed cases of variant cjd (vcjd) in the u.k., with four cases in other countries (france and the republic of ireland). in the u.k., the mean age of onset is 28 years (range 12-74) with a median duration of 13 months (range 6-39). there are reported regional variations in incidence in the u.k., with a north/south difference and a 'cluster' of cases in one county, leicestershire. the incidence of cases in the u.k. is rising. there are concerns about the possibility of secondary, ...	2002	12220145
variant creutzfeldt-jakob disease--a problem for general dental practitioners?	over a hundred deaths from variant creutzfeldt-jakob disease (vcjd) have now been recorded. the incubation period for vcjd may be up to 40 years and the number of asymptomatic carriers in the population could be as many as 100,000. confirmed iatrogenic transmission of other human transmissible spongiform encephalopathies raises the possibility of cross-infection from apparently healthy persons who are incubating vcjd. decontamination techniques routinely used in general dental practice are incap ...	2002	12221758
prions, bse and food.	biochemical and biophysical properties of prions including possible inactivation methods are reviewed. possible molecular markers of transmissible spongiform encephalopathy (tse) and mechanisms behind infectivity and correlation with clinical symptoms are discussed. the risk of bovine spongiform encephalopathy (bse) for humans i.e. variant creutzfeldt-jakob disease (ccjd) is addressed in detail. the consequences of the emergence of the new ccjd and the lack of information on the infectivity of c ...	2002	12222633
analysis of the prion protein in primates reveals a new polymorphism in codon 226 (y226f).	bovine spongiform encephalopathy has been epizootic in cows for the last two decades, and most probably causes variant creutzfeldt-jakob disease in humans. a thorough understanding of prion pathogenesis relies on suitable animal models. modeling the transmission of bse to primates is a crucial public health priority, necessary for determining the tissue distribution of the agent and for devising therapies. susceptibility of humans to bse is partly determined by polymorphism within the gene encod ...	2002	12222676
[basic research on bse transmission to people].	prion diseases of animal and man belong to neurological diseases with amyloidal deposition of the respective proteins. as to prion disease, the cellular prionprotein is in its abnormal isoform(s) an essential component of prionprotein aggregates found in affected tissue. in contrast to all neurodegenerative diseases like morbus alzheimer or huntington's disease, prion diseases are transmissible. therefore, prion diseases were designated transmissible spongiform encephalopathies (tse). the diseas ...	2002	12224460
bacterial contamination of animal feed and its relationship to human foodborne illness.	animal feed is at the beginning of the food safety chain in the "farm-to-fork" model. the emergence of variant creutzfeldt-jakob disease has raised awareness of the importance of contaminated animal feed, but less attention has been paid to the role of bacterial contamination of animal feed in human foodborne illness. in the united states, animal feed is frequently contaminated with non-typhi serotypes of salmonella enterica and may lead to infection or colonization of food animals. these bacter ...	2002	12228823
prp(cwd) lymphoid cell targets in early and advanced chronic wasting disease of mule deer.	up to 15% of free-ranging mule deer in northeastern colorado and southeastern wyoming, usa, are afflicted with a prion disease, or transmissible spongiform encephalopathy (tse), known as chronic wasting disease (cwd). cwd is similar to a subset of tses including scrapie and variant creutzfeldt-jakob disease in which the abnormal prion protein isoform, prp(cwd), accumulates in lymphoid tissue. experimental scrapie studies have indicated that this early lymphoid phase is an important constituent o ...	2002	12237446
quinacrine does not prolong survival in a murine creutzfeldt-jakob disease model.	paramount among issues relating to the transmissible spongiform encephalopathies (also known as prion diseases) is the absence of any effective therapy. this need has been heightened by the substantial european and emerging global problem of bovine spongiform encephalopathy and consequent variant creutzfeldt-jakob disease. stimulated by the recent reports of a potent antiprion effect in cell culture-based clearance assays, we studied the utility of quinacrine in a well-characterized in vivo mode ...	2002	12325081
nursing patients with variant creutzfeldt-jakob disease at home.	variant creutzfeldt-jakob disease (vcjd) is a rare variant of a rare neurodegenerative disease, with a rapid and fatal course. the emergence of vcjd in humans in 1996 is believed to have resulted from the consumption of bovine spongiform encephalopathy (bse)-infected meat. by july 2002, the number of vcjd cases in the uk had increased to over 120 and it is not yet known how many more people will be affected. the majority of affected individuals are cared for within their own homes with the suppo ...	2002	12362140
variant creutzfeldt-jakob disease in an italian woman.	as of may, 2002, 128 cases of variant creutzfeldt-jakob (vcjd) disease have been identified in the uk, france, and ireland. we report the first case of vcjd in italy. the patient was a young italian woman who had never travelled to a country with bovine spongiform encephalopathy (bse). she was diagnosed by cerebral mri and western blot analysis of tonsil biopsy samples. the results of these analyses suggest that vcjd in continental europe and the uk share genetic, clinical, and neuroimaging feat ...	2002	12383671
measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice.	there is increasing concern over the extent to which bovine spongiform encephalopathy (bse) prions have been transmitted to humans, as a result of the rising number of variant creutzfeldt-jakob disease (vcjd) cases. toward preventing new transmissions, diagnostic tests for prions in livestock have been developed using the conformation-dependent immunoassay (cdi), which simultaneously measures specific antibody binding to denatured and native forms of the prion protein (prp). we employed high-aff ...	2002	12389035
probable variant creutzfeldt-jakob disease in a u.s. resident--florida, 2002.	on april 18, 2002, the florida department of health and cdc announced the occurrence of a likely case of variant creutzfeldt-jakob disease (vcjd) in a florida resident aged 22 years. this report documents the investigation of this case and underscores the importance of physicians increasing their suspicion for vcjd in patients presenting with clinical features described in this report who have spent time in areas in which bovine spongiform encephalopathy (bse) is endemic.	2002	12403409
[prion diseases].	creutzfeldt-jakob disease, kuru, gerstmann sträussler scheinker syndrome and fatal familial insomnia in humans, as well as scrapie and bovine spongiform encephalopathy, in animals, are fatal disorders of the central nervous system that are part of the group of transmissible spongiform encephalopathies, (tse) or prion diseases. neuronal intracellular spongiosis and the accumulation of abnormal, protease resistant prion protein in the nervous central system characterize tse. the conformational cha ...	2002	12407310
variant creutzfeldt-jakob disease: an unfolding epidemic of misfolded proteins.	variant creutzfeldt-jakob disease (vcjd) is an emerging infectious disease believed to be the human manifestation of bovine spongiform encephalopathy (bse). variant cjd belongs to a family of human and animal diseases called transmissible spongiform encephalopathies (tse). the pathogenesis of tse is not fully understood, but a modified form of a normal cellular protein plays a central role. current measures to control vcjd aim to prevent transmission of the infectious agent from animals to human ...	2002	12410862
variant creutzfeldt-jakob disease.	variant creutzfeldt-jakob disease is caused by the transmission of bovine spongiform encephalopathy to humans. the clinical and investigative features of variant cjd are relatively distinct from sporadic cjd. the number of cases of vcjd are increasing with time in the uk, but the total future number of cases of vcjd is uncertain.	2002	12416394
neuropathology of variant creutzfeldt-jakob disease.	the clinical, neuropathological genetic and biochemical features of variant creutzfeldt-jakob disease (vcjd) are compared to the 926 other cases of suspected cjd referred to the national cjd surveillance unit laboratory from 1990-2001. histological studies of the central nervous system, lymphoid tissues and other organs were accompanied by immunocytochemistry for prion protein (prp); western blot analysis of prpres was performed on frozen brain tissue. the pathology of vcjd showed relatively uni ...	2002	12416395
bovine spongiform encephalopathy. update.	bovine spongiform encephalopathy (bse) is a zoonosis being the origin of variant creutzfeldt-jakob disease and an important cattle disease in its own right. countries have been slow to learn the importance of protecting, not only their cattle populations, but also their human populations. since 2000, several additional european countries have reported bse in native-born stock and this has led to a concern about the bse status of countries that have imported cattle and cattle products from infect ...	2002	12416396
induction of antibodies against murine full-length prion protein in wild-type mice.	the causative and infectious agent of the transmissible spongiform encephalopathies, e.g. bovine spongiform encephalopathy in cattle or variant creutzfeldt-jakob disease in humans, is a pathogenic form of the scrapie prion protein (prp(sc)) generated by a conformational rearrangement in the normal cellular prion protein (prp(c)). anti-prp antibodies have been shown to exert a protective effect against infection with prp(sc). however, the generation of anti-prp antibodies has proven quite difficu ...	2002	12417440
partitioning of human and sheep forms of the pathogenic prion protein during the purification of therapeutic proteins from human plasma.	therapeutic proteins derived from human plasma and other biologic sources have demonstrated an excellent safety record relative to the potential threat of transmissible spongiform encephalopathy (tse) transmission. previously, hamster-adapted scrapie was used as a model agent to assess tse clearance in purification steps leading to the isolation of biopharmaceutical proteins. the current study investigated the validity of hamster scrapie as a model for human tse clearance studies. the partitioni ...	2002	12421224
implications of bse infection screening data for the scale of the british bse epidemic and current european infection levels.	the incidence of confirmed clinical cases of bovine spongiform encephalopathy (bse) in great britain continues to decline, but the recent discovery of cases in previously unaffected countries (including israel, japan, poland, slovenia and spain) has heightened concerns that bse transmission was more intense and widespread than previously thought. we use back-calculation methods to undertake an integrated analysis of data on infection prevalence in apparently healthy cattle and the incidence of c ...	2002	12427310
bse prions propagate as either variant cjd-like or sporadic cjd-like prion strains in transgenic mice expressing human prion protein.	variant creutzfeldt-jakob disease (vcjd) has been recognized to date only in individuals homozygous for methionine at prnp codon 129. here we show that transgenic mice expressing human prp methionine 129, inoculated with either bovine spongiform encephalopathy (bse) or variant cjd prions, may develop the neuropathological and molecular phenotype of vcjd, consistent with these diseases being caused by the same prion strain. surprisingly, however, bse transmission to these transgenic mice, in addi ...	2002	12456643
disease-associated prion protein in vessel walls.	human prion diseases like creutzfeldt-jakob disease are infectious, inherited, or sporadic neurodegenerative disorders, characterized by the accumulation of an abnormal isoform of the host-encoded prion protein. this affects nervous tissue in sporadic creutzfeldt-jakob disease and, additionally, in lymphoid tissue in bovine spongiform encephalopathy-linked variant creutzfeldt-jakob disease. experimental studies have established the involvement of cells of the lymphoid and peripheral nervous syst ...	2002	12466112
identification of genetic loci affecting mouse-adapted bovine spongiform encephalopathy incubation time in mice.	prion diseases are fatal neurodegenerative disorders of humans and animals, which include bovine spongiform encephalopathy (bse) and its human form, variant creutzfeldt-jakob disease (vcjd). they are characterized by a prolonged incubation period, which is known to be influenced by polymorphisms in the prion protein gene. previous studies of inbred mice have demonstrated that additional genetic loci also contribute to the observed variation in incubation period. however, a substantial transmissi ...	2002	12481985
variant creutzfeldt-jakob disease and bovine spongiform encephalopathy.	strong epidemiologic and laboratory evidence indicate that a novel, variant form of creutzfeldt-jakob disease (vcjd) first reported in the united kingdom in 1996 is causally linked with bovine spongiform encephalopathy (bse). bse was first identified in the early 1980s in the united kingdom, and has since spread to other european countries and recently to japan and israel. although the united kingdom bse epizootic is on the decline, widespread exposure of humans to infected cattle products may h ...	2002	12489284
immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes.	demonstration of the abnormal form of the prion protein (prp) in the brain confirms the diagnosis of human prion disease (prd). using immunohistochemistry, we have compared ten monoclonal antibodies in prd subtypes including sporadic and variant creutzfeldt-jakob disease (cjd), fatal familial insomnia, alzheimer's disease (ad), and control brains. cjd subgroups were determined using western blot analysis for the protease-resistant prp type in combination with sequencing to determine the genotype ...	2002	11770893
increased csf levels of prostaglandin e(2) in variant creutzfeldt-jakob disease.	the concentration of the cyclooxygenase product prostaglandin e(2) was sixfold higher in csf samples from 18 cases of variant creutzfeldt-jakob disease (cjd) than in a group of eight subjects with other noninflammatory neurologic diseases, and comparable to those found in a group of six patients affected by diseases with a known inflammatory component. this finding suggests that cyclooxygenase activity may have a role in variant cjd pathogenesis, as previously reported in sporadic cjd.	2002	11781418
estimating the human health risk from possible bse infection of the british sheep flock.	following the controversial failure of a recent study and the small numbers of animals yet screened for infection, it remains uncertain whether bovine spongiform encephalopathy (bse) was transmitted to sheep in the past via feed supplements and whether it is still present. well grounded mathematical and statistical models are therefore essential to integrate the limited and disparate data, to explore uncertainty, and to define data-collection priorities. we analysed the implications of different ...	2002	11786878
lymph nodal prion replication and neuroinvasion in mice devoid of follicular dendritic cells.	variant creutzfeldt-jakob disease and scrapie are typically initiated by extracerebral exposure to prions, and exhibit early prion accumulation in germinal centers. follicular dendritic cells (fdcs), whose development and maintenance in germinal centers depends on tumor necrosis factor (tnf) and lymphotoxin (lt) signaling, are thought to be indispensable for extraneural prion pathogenesis. here, we administered prions intraperitoneally to mice deficient for tnf and lt signaling components. lt al ...	2002	11792852
migrating intestinal dendritic cells transport prp(sc) from the gut.	bovine spongiform encephalopathy, variant creutzfeldt-jakob disease (vcjd) and possibly also sheep scrapie are orally acquired transmissible spongiform encephalopathies (tses). tse agents usually replicate in lymphoid tissues before they spread into the central nervous system. in mouse tse models prp(c)-expressing follicular dendritic cells (fdcs) resident in lymphoid germinal centres are essential for replication, and in their absence neuroinvasion is impaired. disease-associated forms of prp ( ...	2002	11752724
intestinal entry of prions.	variant creutzfeldt-jakob disease is thought to be caused by infected prion protein via ingestion of contaminated beef. after ingestion of infected prion proteins, uptake by small intestine may be by either m-cell dependent or m-cell independent routes. a receptor for prion protein, laminin receptor precursor is expressed on the brush border of small intestinal epithelium in 40 % of subjects. the cellular prion protein expressed on the enteric nervous system might serve as the target for convers ...	2002	11803499
laminar distribution of the pathological changes in the cerebral cortex in variant creutzfeldt-jakob disease (vcjd).	to determine the pattern of cortical degeneration in cases of variant creutzfeldt-jakob disease (vcjd), the laminar distribution of the vacuolation ("spongiform change"), surviving neurones, glial cell nuclei, and prion protein (prp) deposits was studied in the frontal, parietal and temporal lobes. the vacuolation exhibited two common patterns of distribution: either the vacuoles were present throughout the cortex or a bimodal distribution was present with peaks of density in the upper and lower ...	2002	12572772
mr spectroscopic pulvinar sign in a case of variant creutzfeldt-jakob disease.	we report mr spectroscopic findings in a patient hospitalized with biopsy-proven variant creutzfeldt-jakob (vcjd) disease. n-acetyl aspartate was markedly decreased in the postero-medial part of the thalami (pulvinar) but was not diminished in the parieto-occipital white matter and cortical grey matter. these observations, which are in accordance with the pathological findings in this disease, suggest that mr spectroscopy, a highly sensitive method for the detection of subtle brain metabolic dys ...	2002	12538948
from the centers for disease control and prevention. probable variant creutzfeldt-jakob disease in a us resident--florida, 2002.		2002	12492096
quantification of vacuolation ("spongiform change"), surviving neurones and prion protein deposition in eleven cases of variant creutzfeldt-jakob disease.	vacuolation ("spongiform change") and prion protein (prp) deposition were quantified in the cerebral cortex, hippocampus, dentate gyrus and molecular layer of the cerebellum in 11 cases of variant creutzfeldt-jakob disease (vcjd). the density of vacuoles was greater in the cerebral cortex compared to the hippocampus, dentate gyrus and cerebellum. within the cortex, vacuole density was significantly greater in the occipital compared to the temporal lobe and the density of surviving neurones was g ...	2002	11972799
[considerations of the robert koch institute and its task force on minimizing a) known and b) theoretical risk for transmission of variant creutzfeldt-jakob syndrome by potentially contaminated medical products].		2002	11968175
tonsillectomy and variant creutzfeldt-jakob disease.		2002	11902091
distribution of infectivity in variant creutzfeldt-jakob disease.		2002	11888628
evaluation of the validity and reliability of a-scan ultrasound biometry with a single use disposable cover.	the uk medical devices agency has suggested that ophthalmic practitioners should, where practicable and not compromising clinical outcome, restrict corneal contact devices to single patient use to minimise a remote theoretical risk of transmission of new variant creutzfeldt-jakob disease (vcjd). this study reports on a modified technique of ultrasound a-scan biometry that complies with the mda recommendations.	2002	11864896
variant creutzfeldt-jakob disease.		2002	11861680
the use of antioxidants in transmissible spongiform encephalopathies: a case report.	transmissible spongiform encephalopathies (tse), which include creutzfeldt-jakob disease and new variant creutzfeldt-jakob disease, are diseases characterized by progressive deterioration in the central nervous system with neuronal degeneration, vacuolatization of the neuropil, and gliosis. little is known about the pathogenic mechanisms of infection, and controversy exits around the inciting infective agent. it has been shown that an important factor in pathogenesis is the immune system.	2002	11838883
future uncertain for variant creutzfeldt-jakob disease.		2002	12849414
creutzfeldt-jakob disease in australia 1970-1999.	to ascertain all persons who developed a transmissible spongiform encephalopathy (tse) within australia during the 30-year period 1970 to 1999 through a comprehensive national surveillance program and subject the group to detailed epidemiologic analysis.	2002	12427885
the spatial patterns of prion protein deposits in cases of variant creutzfeldt-jakob disease.	the spatial patterns of the prion protein (prp) deposits were studied in immunostained sections of areas of the cerebral cortex, hippocampus, dentate gyrus, and the molecular layer of the cerebellum in 11 cases of variant creutzfeldt-jakob disease (vcjd). clustering of prp deposits, with a regular distribution of the clusters parallel to the tissue boundary, was the most common spatial pattern observed. two morphological types of prp deposit were recognised, those consisting of a condensed core ...	2002	12410388
irregular presence of abnormal prion protein in appendix in variant creutzfeldt-jakob disease.		2002	12397162
inline-filtration.	routine leukocyte-depletion (ld) of cellular blood products, and even plasma, is currently being implemented in most european countries, as a result of the fear that the variant creutzfeldt-jakob-disease (vcjd) might be transmissible by transfusion. however, not only is the scientific evidence supporting such a notion scarce, but the benefits of applying this procedure to all patients also remain unfounded.	2002	12350049
kuru: the old epidemic in a new mirror.	the kuru epidemic lasted almost a century; it started in 1901-1902, reached epidemic proportions in the mid-1950s, and disappeared in the 1990s. kuru is the prototype member of a group of disorders known as transmissible spongiform encephalopathies (tses) or prion diseases. recent data on the genetics and pathogenesis of tses contribute to a better understanding of the documented kuru phenomena, and vice versa, observations made during the kuru epidemic are immensely helpful in understanding the ...	2002	12270735
purity of spiking agent affects partitioning of prions in plasma protein purification.	prions are not detectable in the blood or plasma of persons afflicted with classical or variant creutzfeldt-jakob disease, and they have never been shown to be transmitted by blood or plasma products. despite the uncertainty as to the presence and biophysical properties of prions in plasma, prion removal studies have been conducted using brain homogenate or microsomes prepared from prion-infected rodent brains as model prions. in this study, we compare the partitioning of different prion spiking ...	2002	12217343
variant creutzfeldt-jakob disease: costs borne by families.	the objectives of this study were: (1) to estimate the costs borne by families caring for patients with variant creutzfeldt-jakob disease (vcjd); (2) to contextualise results to recent policy initiatives, and (3) to consider the methodological problems of estimating costs of care. semi-structured interviews and a follow-up postal questionnaire, eliciting costs to families both before and after the patient's death, were carried out. participants included 19 families of patients with vcjd. cost pr ...	2002	12121267
vcjd: the epidemic that never was. new variant creutzfeldt-jakob disease: the critique that never was.		2002	12114248
sensory features of variant creutzfeldt-jakob disease.	sensory symptoms are a prominent feature of variant creutzfeldt-jakob disease (vcjd), occurring at an early stage of the illness. they are persistent and can be troublesome. here, they are described in detail and a possible anatomical basis is discussed.	2002	12111303
quantitative variations in the pathology of 11 cases of variant creutzfeldt-jakob disease (vcjd).	quantitative variations in the density and distribution of the vacuolation ('spongiform change'), surviving neurons, and prion protein (prp) deposits were studied in eight brain regions from 11 cases of variant creutzfeldt-jakob disease (vcjd). principal components analysis (pca) was used to study the similarities and differences between cases and to identify the neuropathological variables which could best account for these variations. two principal components (pc) were extracted from the data ...	2002	12100967
the incubation period of kuru.	kuru is a transmissible spongiform encephalopathy that was identified in papua new guinea in the late 1950s. several thousand cases of the disease occurred during a period of several decades. epidemiologic investigations implicated ritual endocannibalistic funeral feasts as the likely route through which the infectious agent was spread.	2002	12094094
first hundred cases of variant creutzfeldt-jakob disease: retrospective case note review of early psychiatric and neurological features.	to describe the early psychiatric and neurological features of variant creutzfeldt-jakob disease.	2002	12077031