| regulation of microglial proliferation during chronic neurodegeneration. | an important component of chronic neurodegenerative diseases is the generation of an innate inflammatory response within the cns. microglial and astroglial cells play a key role in the development and maintenance of this inflammatory response, showing enhanced proliferation and activation. we studied the time course and regulation of microglial proliferation, using a mouse model of prion disease. our results show that the proliferation of resident microglial cells accounts for the expansion of t ... | 2013 | 23392676 |
| spontaneous obesity-linked type 2 diabetes in the absence of islet amyloid in a cynomolgus monkey infected with bovine spongiform encephalopathy. | a 9-year-old cynomolgus monkey (macaca fascicularis) infected orally with bovine spongiform encephalopathy (bse) was presented for necropsy following euthanasia 4 years post infection (p.i.). this macaque r984 was exposed to a bse dose that causes a simian form of variant creutzfeldt-jakob disease (vcjd) within 5 years p.i. in other macaques. all orally bse-infected macaques developed a significant weight gain within the first 2 years p.i. compared with non-bse-infected age- and sex-matched cont ... | 2013 | 23381923 |
| a closer look at prion strains: characterization and important implications. | prions are infectious proteins that are responsible for transmissible spongiform encephalopathies (tses) and consist primarily of scrapie prion protein (prp (sc) ), a pathogenic isoform of the host-encoded cellular prion protein (prp (c) ). the absence of nucleic acids as essential components of the infectious prions is the most striking feature associated to these diseases. additionally, different prion strains have been isolated from animal diseases despite the lack of dna or rna molecules. mo ... | 2017 | 23357828 |
| bse and variant cjd: emerging science, public pressure and the vagaries of policy-making. | classical bovine spongiform encephalopathy (bse) was first recognized in 1987 in the united kingdom and ultimately spread to cattle across europe and to the middle east, north america and japan through the movement of infected animals and contaminated meat and bone meal. the human expression of bse, variant creutzfeldt-jakob disease (vcjd), likewise was first identified in the uk and now has been observed in many countries due to human exposure to bse contaminated products or to vcjd contaminate ... | 2013 | 23287715 |
| prion removal capacity of plasma protein manufacturing processes: a data collection from ppta member companies. | the variant creutzfeldt-jakob disease incidence peaked a decade ago and has since declined. based on epidemiologic evidence, the causative agent, pathogenic prion, has not constituted a tangible contamination threat to large-scale manufacturing of human plasma-derived proteins. nonetheless, manufacturers have studied the prion removal capabilities of various manufacturing steps to better understand product safety. collectively analyzing the results could reveal experimental reproducibility and d ... | 2013 | 23252676 |
| immunotherapy in prion disease. | transmissible spongiform encephalopathies (tses), also known as prion diseases, describe a group of fatal neurodegenerative disorders affecting both humans and animals. accumulation of misfolded prion proteins is the pathological hallmark of these disorders; such accumulation occurs in lymphoreticular tissue prior to cns involvement in scrapie, experimental models and human variant creutzfeldt-jakob disease. lymphoreticular accumulation of misfolded prion protein has not been demonstrated in hum ... | 2013 | 23247613 |
| bioassay studies support the potential for iatrogenic transmission of variant creutzfeldt jakob disease through dental procedures. | evidence is required to quantify the potential risks of transmission of variant creutzfeldt jakob (vcjd) through dental procedures. studies, using animal models relevant to vcjd, were performed to address two questions. firstly, whether oral tissues could become infectious following dietary exposure to bse? secondly, would a vcjd-contaminated dental instrument be able to transmit disease to another patient? | 2012 | 23226225 |
| human prion diseases: from kuru to variant creutzfeldt-jakob disease. | transmissible spongiform encephalopathies (tses) or prion diseases are the names given to the group of fatal neurodegenerative disorders that includes kuru, creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker disease (gss), fatal and sporadic familial insomnia and the novel prion disease variable protease-sensitive prionopathy (pspr) in humans. kuru was restricted to natives of the foré linguistic group in papua new guinea and spread by ritualistic endocannibalism. cjd appears as spo ... | 2012 | 23225013 |
| risk factors for variant creutzfeldt-jakob disease in dental practice: a case-control study. | to assess the risk of variant creutzfeldt-jakob disease (vcjd) associated with dental treatment. | 2012 | 23222357 |
| summary of: risk factors for variant creutzfeldt-jakob disease in dental practice: a case-control study. | | 2012 | 23222331 |
| constant transmission properties of variant creutzfeldt-jakob disease in 5 countries. | variant creutzfeldt-jakob disease (vcjd) has been reported in 12 countries. we hypothesized that a common strain of agent is responsible for all vcjd cases, regardless of geographic origin. to test this hypothesis, we inoculated strain-typing panels of wild-type mice with brain material from human vcjd case-patients from france, the netherlands, italy, and the united states. mice were assessed for clinical disease, neuropathologic changes, and glycoform profile; results were compared with those ... | 2012 | 23017202 |
| shadoo/prp (sprn(0/0) /prnp(0/0) ) double knockout mice: more than zeroes. | shadoo (sho) is a brain glycoprotein with similarities to the unstructured region of prp (c) . frameshift alleles of the sho gene, sprn, are reported in variant creutzfeldt-jakob disease (vcjd) patients while sprn mrna knockdown in prp-null (prnp(0/0) ) embryos produces lethality, advancing sho as the hypothetical prp-like "pi" protein. also, sho levels are reduced as misfolded prp accumulates during prion infections. to penetrate these issues we created sprn null alleles (daude et al., proc. na ... | 2016 | 22929230 |
| dealing with the uncertain risk of variant creutzfeldt-jakob disease transmission by coagulation replacement products. | the identification of variant creutzfeldt-jakob disease (vcjd) in the uk in 1996 led to significant concerns about the possibility of secondary transmission, however the prevalence of subclinical vcjd and risks of vcjd transmission by plasma are not known. in the uk, public health precautions have been implemented in all recipients of coagulation factor concentrates manufactured from uk plasma pools between 1980 and 2001. the recent demonstration of abnormal prion protein in a spleen sample at a ... | 2012 | 22775486 |
| [variant creutzfeldt-jakob disease (vcjd)]. | | 2011 | 22755226 |
| consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in europe and usa. | the current classification of human sporadic prion diseases recognizes six major phenotypic subtypes with distinctive clinicopathological features, which largely correlate at the molecular level with the genotype at the polymorphic codon 129 (methionine, m, or valine, v) in the prion protein gene and with the size of the protease-resistant core of the abnormal prion protein, prp(sc) (i.e. type 1 migrating at 21 kda and type 2 at 19 kda). we previously demonstrated that prp(sc) typing by western ... | 2012 | 22744790 |
| response to the article by adanipar et al.: the first report of a patient with probable variant creutzfeldt-jakob disease in turkey, dement geriatr cogn disord extra 2011;1:429-432. | | 2012 | 22619663 |
| a comparative analysis to study editing of small noncoding bc200- and alu transcripts in brain of prion-inoculated rhesus monkeys (m. mulatta). | small retroelements (short interspersed elements, abbreviated sines) are abundant in vertebrate genomes. using rna isolated from rhesus monkey cerebellum and buffy coat, reverse-transcription polymerase chain reaction (rt pcr) was applied to clone cdna of bc200 and alu rnas. transcripts containing alu-sine sequences may be subjected to extensive rna editing by adar (adenosine deaminases that act on rna) deamination. abundance of alu transcripts was determined with real-time rt pcr and was signif ... | 2012 | 22524594 |
| cost-effectiveness of prion filtration of red blood cells to reduce the risk of transfusion-transmitted variant creutzfeldt-jakob disease in the republic of ireland. | variant creutzfeldt-jakob disease (vcjd) is a rare, progressive fatal noninflammatory neurodegenerative disease. ireland has the second highest rate of vcjd in the world with an ongoing risk of vcjd transmission through blood transfusion. prion-removing filters have been developed to reduce the risk of vcjd transmission. this study aimed to evaluate the cost-effectiveness of implementing a policy of prion filtration of red blood cells (rbcs) in the republic of ireland. | 2012 | 22486387 |
| neuroinflammation and common mechanism in alzheimer's disease and prion amyloidosis: amyloid-associated proteins, neuroinflammation and neurofibrillary degeneration. | in cases with a long (>1 year) clinical duration of prion disease, the prion protein can form amyloid deposits. these cases do not show accumulation of 4-kda β-amyloid, which is observed in amyloid deposits in alzheimer's disease (ad). in ad, amyloid is associated with inflammation and neurofibrillary degeneration, and it is elusive whether prion amyloid is associated with these changes as well. | 2012 | 22398730 |
| prion infected rhesus monkeys to study differential transcription of alu dna elements and editing of alu transcripts in neuronal cells and blood cells. | rhesus monkeys were used as a non-human primate model to study small non-coding rna after infection with human sporadic and variant creutzfeldt-jakob prions. | 2012 | 22380733 |
| geographical bse risk assessment and its impact on disease detection and dissemination. | bovine spongiform encephalopathy (bse) rapidly evolved into an issue of major public concern particularly when, in 1996, evidence was provided that this disease had crossed the species barrier and infected humans in the uk with what has become known as "variant creutzfeldt jakob disease" (vcjd). the aim of this paper is to describe the european geographical bse risk assessment (gbr) that was successfully used for assessing the qualitative likelihood that bse could be present in a country where i ... | 2012 | 22305879 |
| the first report of a patient with probable variant creutzfeldt-jakob disease in turkey. | variant creutzfeldt-jakob disease (vcjd) was first reported in the uk in 1996. here, we report the first turkish case of vcjd. a 47-year-old man, who has never lived outside of turkey and had had no transfusion, was admitted to the university hospital with speech disorder, cognitive decline and ataxia following depression, irritability, and personality change. the immunoassay of the 14-3-3 protein in the cerebrospinal fluid was negative. brain magnetic resonance imaging revealed high-signal lesi ... | 2011 | 22279448 |
| kuru: genes, cannibals and neuropathology. | kuru was the first human transmissible spongiform encephalopathy (tse) or prion disease identified, occurring in the fore linguistic group of papua new guinea. kuru was a uniformly fatal cerebellar ataxic syndrome, usually followed by choreiform and athetoid movements. kuru imposed a strong balancing selection on the fore population, with individuals homozygous for the 129 met allele of the gene (prnp) encoding for prion protein (prp) being the most susceptible. the decline in the incidence of k ... | 2012 | 22249461 |
| genome-wide study links mtmr7 gene to variant creutzfeldt-jakob risk. | the aim of our study was to discover genomic variations related to variant creutzfeldt-jakob disease (vcjd) susceptibility. a genome-wide association analysis with most vcjd samples available in the world was performed. a series of 93 vcjd uk patients and 1504 uk controls were included in the discovery stage. our best findings were replicated in an independent population of 22 uk and 20 french vcjd cases. post hoc analysis to assess our main results included 5711 french controls, 445 dutch contr ... | 2012 | 22137330 |
| unusual dopaminergic depletion in variant creutzfeldt-jakob disease with early and rapid cognitive decline. | | 2011 | 21625143 |
| 11c-pib pet does not detect prp-amyloid in prion disease patients including variant creutzfeldt-jakob disease. | | 2012 | 21478204 |
| risk reduction strategies for variant creutzfeldt-jakob disease transmission by uk plasma products. | | 2011 | 21362112 |
| comment on validation of diagnostic criteria for variant creutzfeldt-jakob disease. | | 2011 | 21280094 |
| variant creutzfeldt-jakob disease occurring in mother and son. | | 2012 | 21257982 |
| transmission of sporadic creutzfeldt-jakob disease by blood transfusion: risk factor or possible biases. | the occurrence of transfusion transmissions of variant creutzfeldt-jakob disease (cjd) cases has reawakened attention to the possible similar risk posed by other forms of cjd. | 2011 | 21214582 |
| diagnosing variant creutzfeldt-jakob disease: a retrospective analysis of the first 150 cases in the uk. | establishing an early clinical diagnosis in variant creutzfeldt-jakob disease (vcjd) can be difficult, resulting in extended periods of uncertainty for many families and sometimes a view that patients have been subjected to unnecessary investigations. this issue is accentuated by the progressive nature of vcjd and by the difficulty in achieving a confident clinical diagnosis before an advanced stage of illness. although diagnostic delay may be a result of the non-specific early clinical features ... | 2011 | 21172857 |
| a quantitative study of the pathological changes in the cortical white matter in variant creutzfeldt-jakob disease (vcjd). | to quantify cortical white matter pathology in variant creutzfeldt-jakob disease (vcjd) and to correlate white and grey matter pathologies. | 2010 | 21073844 |
| strategies to reduce transfusion acquired vcjd. | variant creutzfeldt-jakob disease (vcjd) can be transmitted by transfusion. the risk depends on the number of infected donors in the community. an estimate of these numbers in a less genetically susceptible population, based on the epidemic seen so far, suggests a maximum of 300 more cases. from this, it is possible to predict a maximum of one transfusion acquired case in 3 years from plasma transfusion. importation of plasma from outside the uk has been advocated to prevent these cases and woul ... | 2011 | 21070399 |
| a pilot to examine the logistical and feasibility issues in testing deceased tissue donors for vcjd using tonsil as the analyte. | transplanted tissues have transmitted transmissible spongiform encephalopathies and in the uk there have been more cases of variant creutzfeldt-jakob disease (vcjd) than elsewhere in the world. a pilot study was undertaken to look at the feasibility of testing for vcjd in deceased donors using tonsillar tissue. this pilot showed that obtaining consent for removal and testing tonsil tissue was feasible. donor eligibility for inclusion in the pilot was limited to tissue donors from the national he ... | 2012 | 21046259 |
| phase i/ii safety study of transfusion of prion-filtered red cell concentrates in transfusion-dependent patients. | variant creutzfeldt-jakob (vcjd) is a fatal transfusion transmissible prion infection. no test for vcjd in the donor population is currently available. therefore, prion removal by filtration of red cell concentrate (rcc) is an attractive option for prevention. | 2010 | 20345513 |
| feasibility study of a screening assay that identifies the abnormal prion protein prptse in plasma: initial results with 20,000 samples. | it is likely that transmission of variant creutzfeldt-jakob disease (vcjd) occurs by transfusion and that the candidate infectious agent (prp(tse)) is present in small concentrations in the blood of infected donors in the asymptomatic phase of the disease. a new blood screening assay has been developed to detect prp(tse) in citrated plasma samples. | 2010 | 20088835 |
| variant creutzfeldt-jakob disease: the first confirmed case from portugal shows early onset, long duration and unusual pathology. | we present clinical and autopsy findings in the first case of variant creutzfeldt-jakob disease diagnosed and confirmed in portugal. onset was at 11 years, the earliest onset reported, and the course (32 months) relatively long. western blot showed protease resistant prion protein, mainly of type 4 (2b) isoform. the cerebral cortex revealed severe spongiform change with numerous amyloid plaques, which did not fit the definition of florid plaques. in the striatum, spongiform change was limited bu ... | 2010 | 20019229 |
| the epidemiology of progressive intellectual and neurological deterioration in childhood. | to study the epidemiology of diseases that cause progressive intellectual and neurological deterioration (pind) in uk children. | 2010 | 19948513 |
| genetic creutzfeldt-jakob disease mimicking variant creutzfeldt-jakob disease. | | 2009 | 19917826 |
| cellular prion protein in blood platelets associates with both lipid rafts and the cytoskeleton. | the recently shown transmissibility of variant creutzfeldt-jakob disease (vcjd) by blood transfusion emphasises the need for better understanding of the cellular prion protein (prpc) in blood. a substantial amount of cell-associated prpc in blood resides in platelets. platelet activation leads to up-regulation of prpc on the platelet surface and its release on exosomes and microparticles. the sub-cellular localisation and function of platelet prpc, however, is poorly understood. in the present s ... | 2009 | 19888536 |
| variant creutzfeldt-jakob disease in the united kingdom: a countrywide or local risk? | the aim of this study was to identify factors that may have augmented local risks for variant creutzfeldt-jakob disease (vcjd). | 2010 | 19692715 |
| a retrospective case note review of deceased recipients of vcjd-implicated blood transfusions. | to date, four instances of probable transfusion-transmission of variant creutzfeldt-jakob disease (vcjd) infection have been described, and surviving recipients of vcjd-implicated blood components have been informed that they may be 'at risk' of vcjd. nearly two-thirds of all recipients of vcjd-implicated blood components are deceased, and many died before the vcjd risk was known. the primary aim of this study was to determine retrospectively whether there was evidence that any of the other dece ... | 2009 | 19671123 |
| variant creutzfeldt-jakob disease: neuropsychological profile in an extended series of cases. | neuropsychological data on an extended series of cases of variant creutzfeldt-jakob disease (vcjd) are presented, complementing earlier findings from smaller sample studies of this condition. distinct neuropsychological features in this extended series included relatively preserved verbal knowledge, immediate verbal memory span, and elementary visual processing. this sparing contrasted with ubiquitous impairment in every vcjd patient on timed tests of verbal fluency and digit-symbol substitution ... | 2009 | 19570313 |
| variant creutzfeldt-jakob disease and exposure to fractionated plasma products. | the risk to public health of onward transmission of variant creutzfeldt-jakob disease (vcjd) via blood transfusion and plasma product administration is of on-going concern, particularly with the recent reported detection of abnormal prion protein in a person with haemophilia. | 2009 | 19538514 |
| pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant creutzfeldt-jakob disease transmission. | | 2009 | 19490428 |
| [bilateral hyperintensity of the pulvinar in sporadic creutzfeldt-jakob disease]. | definitive diagnosis of creutzfeldt-jakob disease (cjd) is made by pathological study. the premortem differentiation of variant forms is important in public health. bilateral hyperintensity of the thalamic pulvinar nucleus in t2, flair and dw sequences ("pulvinar sign") in magnetic resonance imaging (mri) is related to the variant creutzfeldt-jakob disease (vcjd) and is a diagnostic criterion of probable vcjd. a case of sporadic cjd with bilateral hyperintensity of pulvinar nucleus is described. | 2009 | 19418298 |
| is there evidence of vertical transmission of variant creutzfeldt-jakob disease? | the possibility of vertical transmission of variant creutzfeldt-jakob disease (vcjd) has been raised because of the widespread distribution of infectivity in vcjd and the demonstration that this condition can be transmitted through blood transfusion. the aim of this study is to search for evidence of this type of transmission of vcjd. | 2011 | 19401289 |
| a rapid dual staining procedure for the quantitative discrimination of prion amyloid from tissues reveals how interactions between amyloid and lipids in tissue homogenates may hinder the detection of prions. | transmissible spongiform encephalopathies (tses) are fatal neurodegenerative diseases with no cure to this day, and are often associated with the accumulation of amyloid plaques in the brain and other tissues in affected individuals. the emergence of new variant creutzfeldt-jakob disease, an acquired tse with a relatively long asymptomatic incubation period and unknown prevalence or incidence, which could potentially be iatrogenically transmitted, has prompted the need for sensitive and rapid me ... | 2009 | 19318058 |
| survival and re-operation rates after neurosurgical procedures in scotland: implications for targeted surveillance of sub-clinical variant creutzfeldt-jakob disease. | to assess the feasibility of post-mortem surveillance for subclinical variant creutzfeldt-jakob disease (vcjd) at least 5 years after neurosurgical procedures. | 2009 | 19299901 |
| "hot cross bun" sign in variant creutzfeldt-jakob disease. | | 2009 | 19279286 |
| further characterisation of the prion protein molecular types detectable in the nibsc creutzfeldt-jakob disease brain reference materials. | sporadic and variant creutzfeldt-jakob disease brain reference materials available from the uk national institute for biological standards and control have been subjected to further characterisation by western blot analysis, with particular reference to the co-occurrence of different abnormal disease-associated prion protein (prp(sc)) types. the results confirm the presence of genuine type 1 and type 2 protease-resistant prp (prp(res)) in each of the three sporadic creutzfeldt-jakob disease reag ... | 2009 | 19264507 |
| amyloidophilic compounds for prion diseases. | recent outbreaks of variant creutzfeldt-jakob disease and iatrogenic creutzfeldt-jakob disease have aroused great concern in many countries and have necessitated the development of suitable therapies. we have demonstrated that sulfated glycans such as pentosan polysulfate and fucoidan, and amyloidophilic compounds such as amyloid dye derivatives, styrylbenzoazole derivatives, and phenylhydrazine derivatives have efficacies in prion-infected animals. amyloidophilic compounds present potentialitie ... | 2009 | 19200011 |
| a quantitative study of the pathological changes in the cerebellum in 15 cases of variant creutzfeldt-jakob disease (vcjd). | to determine in the cerebellum in variant creutzfeldt-jakob disease (vcjd): (i) whether the pathology affected all laminae; (ii) the spatial topography of the pathology along the folia; (iii) spatial correlations between the pathological changes; and (iv) whether the pathology was similar to that of the common methionine/methionine type 1 subtype of sporadic cjd. | 2009 | 19187059 |
| lack of evidence of transfusion transmission of creutzfeldt-jakob disease in a us surveillance study. | since 2004, several reported transfusion transmissions of variant creutzfeldt-jakob disease (vcjd) in the united kingdom have reawakened concerns about the possible risk of similar transmissions of nonvariant or classic forms of cjd. | 2009 | 19170987 |
| report of the working group 'overall blood supply strategy with regard to variant creutzfeldt-jakob disease (vcjd)': statement on the development and implementation of test systems suitable for the screening of blood donors for vcjd - dated september 17, 2008. | | 2009 | 21048823 |
| an update on the assessment and management of the risk of transmission of variant creutzfeldt-jakob disease by blood and plasma products. | there have been four highly probable instances of variant creutzfeldt-jakob disease (vcjd) transmission by non-leucocyte depleted red cell concentrates and it is now clear that the infectious agent is transmissible by blood components. to date there in no reported evidence that the infectious agent has been transmitted by fractionated plasma products, e.g. factor viii concentrate. this review outlines current and potential risk management strategies including donor deferral criteria, the potenti ... | 2009 | 18950452 |
| review. the neuropathology of kuru and variant creutzfeldt-jakob disease. | a comparison of the pathological profiles of two spongiform encephalopathies with a similar presumptive route of infection was performed. archival kuru and recent variant creutzfeldt-jakob disease (vcjd) cases reveal distinct lesional differences, particularly with respect to prion protein, suggesting that the strain of agent is important in determining the phenotype. genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that al ... | 2008 | 18849282 |
| ten-year mortality from creutzfeldt-jakob disease in cyprus. | we report the mortality from sporadic creutzfeldt-jakob disease in cyprus for a 10-year surveillance period (1995-2004). in that time, 5 cases were identified out of a population of 749,000, giving an incidence of 0.7 cases per million population per year. our sporadic incidence matches that expected according to global epidemiological surveillance. no cases of variant creutzfeldt-jakob disease were found but 1 familial case was diagnosed. | 2014 | 18720636 |
| clinical aspects of variant creutzfeldt-jakob disease. | | 2008 | 18631793 |
| ultrastructural study of florid plaques in variant creutzfeldt-jakob disease: a comparison with amyloid plaques in kuru, sporadic creutzfeldt-jakob disease and gerstmann-sträussler-scheinker disease. | although the histological features of the amyloid plaques in variant creutzfeldt-jakob disease (vcjd) are distinct from those in other forms of prion disease [kuru, sporadic creutzfeldt-jakob disease (scjd) and gerstmann-sträussler-scheinker disease (gss)], their ultrastructural features have only been described in a single case report. | 2009 | 18513219 |
| towards modeling of amyloid fibril structures. | amyloid fibrils are associated with a number of debilitating diseases, including alzheimer's disease and variant creutzfeldt-jakob disease. the elucidation of the structure of amyloid fibrils is an important step toward understanding the mechanism of amyloid formation and developing therapeutic agents for amyloid diseases. despite great interests and substantial efforts from various research communities, deriving high-resolution structures of amyloid fibrils remains a challenging problem, due to ... | 2008 | 18508498 |
| two cases of variant creutzfeldt-jakob disease reported in spain in 2007 and 2008. | | 2008 | 18445462 |
| ian mcewan--novels about neurological and psychiatric patients. | ian mcewan, a respected contemporary british writer, sometimes uses neurological and psychiatric patients as main characters of his stories. in his recent novels one can find beautiful descriptions of patients with huntington's disease, variant creutzfeldt-jakob disease, de clerambault syndrome and also details about some neurosurgical procedures. | 2008 | 18437042 |
| advances in the development of a screening test for variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is a transmissible neurodegenerative prion disease that continues to present a unique problem for medical diagnostics. uncertainties remain over the prevalence of vcjd in the uk population and its incubation period in individuals of different genotypes. although the infectious agent that causes vcjd is widely distributed in the peripheral tissues of patients and those carrying the disease, it does not provoke any host immune response that would be amenabl ... | 2008 | 23485140 |
| vision loss due to coincident ocular and central causes in a patient with heidenhain variant creutzfeldt-jakob disease. | creutzfeldt-jakob disease (cjd) is a degenerative disease of the brain associated with a rapidly progressive spongiform encephalopathy. visual symptoms and neuro-ophthalmological signs are not infrequent, and presentation to an ophthalmologist may result. a case is reported of an 89-years-old gentleman who presented with a short history of isolated deterioration in vision. he underwent ocular intervention but subsequently developed progressive dementia, asterixis, myoclonus, cerebellar and extra ... | 2008 | 18065777 |
| modeling red cell procurement with both double-red-cell and whole-blood collection and the impact of european travel deferral on units available for transfusion. | in 1997 the fda approved the first double-red-blood-cell (2rbc) collection device. soon after, travel deferral for variant creutzfeldt-jakob disease (vcjd) risk was adopted. to show the importance of including 2rbcs in predictive models of the blood supply, an existing whole-blood (wb) model was updated to include 2rbc collection and then run to simulate the effect of vcjd deferral on total rbc availability. | 2007 | 17958531 |
| size frequency distributions of abnormal protein deposits in alzheimer's disease and variant creutzfeldt-jakob disease. | the size frequency distributions of beta-amyloid (a beta) and prion protein (prpsc) deposits were studied in alzheimer's disease (ad) and the variant form of creutzfeldt-jakob disease (vcjd) respectively. all size distributions were unimodal and positively skewed. a beta deposits reached a greater maximum size and their distributions were significantly less skewed than the prpsc deposits. all distributions were approximately log-normal in shape but only the diffuse prpsc deposits did not deviate ... | 2007 | 17849360 |
| application of atomic dielectric resonance spectroscopy for the screening of blood samples from patients with clinical variant and sporadic cjd. | sub-clinical variant creutzfeldt-jakob disease (vcjd) infection and reports of vcjd transmission through blood transfusion emphasise the need for blood screening assays to ensure the safety of blood and transplanted tissues. most assays aim to detect abnormal prion protein (prpsc), although achieving required sensitivity is a challenge. | 2007 | 17760958 |
| airway management for tonsillectomy: a national survey of uk practice. | the emergence of variant creutzfeldt-jakob disease (vcjd) prompted guidelines from the department of health that stress the use of disposable and protective equipment. this survey explores current methods of airway management for tonsillectomy in the uk and ascertains anaesthetists' current knowledge and opinions of the guidelines and of vcjd. | 2007 | 17596593 |
| measurement of cd83 mrna by real-time polymerase chain reaction to determine removal of dendritic cells by leucoreduction of whole blood. | information is lacking regarding efficiency of removal of circulating dendritic cells (dcs) by leucoreduction (lr) of blood. this is important since dcs may play a role in transporting abnormal prion, the likely infectious agent of variant creutzfeldt-jakob disease. in this study, we report development of a real-time polymerase chain reaction (rt-pcr) assay to quantify residual dcs in lr whole blood via measurement of selected messenger rna (mrna) markers. taqman-based rt-pcr assays were set up ... | 2007 | 17561858 |
| manufacture of plasma-derived products in france and measures to prevent the risk of vcjd transmission: precautionary measures and efficacy of manufacturing processes in prion removal. | the emergence of the variant creutzfeldt-jakob disease in the mid 1990s soon raised concerns about its possible transmission through the use of blood and plasma-derived medicinal products. a risk analysis approach was initiated by health authorities, based on updated scientific knowledge and precautionary measures were implemented in france and other countries for the management of this new possible risk. assessment of the vcjd risk is based on epidemiology and estimates of the number of potenti ... | 2007 | 17540602 |
| neuropathological changes in striate and extrastriate visual cortex in variant creutzfeldt-jakob disease (vcjd). | pathological changes in striate (b17, v1) and extrastriate (b18, v2) visual cortex were studied in variant creutzfeldt-jakob disease (vcjd). no differences in densities of vacuoles or surviving neurons were observed in b17 and b18 but densities of glial cell nuclei and deposits of prion protein (prp) were greater in b18. prp deposit densities in b17 and b18 were positively correlated. diffuse deposit density in b17 was negatively correlated with the density of surviving neurons in b18. the vacuo ... | 2007 | 19668500 |
| blood conservation techniques in obstetrics: a uk perspective. | in the uk, maternal mortality due to haemorrhage appears to be rising, with obstetric haemorrhage accounting for 3-4% of the red cells transfused. allogeneic blood transfusion carries risks such as administration errors, transmitted infections and immunological reactions. the supply of blood is decreasing, partly due to the exclusion of donors who have themselves received a blood transfusion since 1980, in order to stop transmission of variant-creutzfeldt-jakob disease. the cost of blood is sign ... | 2007 | 17509870 |
| the use of single-use devices in anaesthesia: balancing the risks to patient safety. | single-use devices are designed, manufactured and sold to be used once and then discarded. this paper addresses growing concerns about the quality of some devices. single-use devices, manufactured at a lower cost to justify their disposal, are perceived to have a lesser efficacy, which may threaten patient safety through iatrogenic harm. there is, in addition, growing scepticism about the actual risk of contracting variant creutzfeldt-jakob disease and other blood-borne diseases from reused surg ... | 2007 | 17506734 |
| [plasma fractionation in the world: current status]. | from 22 to 25 million liters of plasma are fractionated yearly in about 70 fractionation plants, either private or government-owned, mainly located in industrialized countries, and with a capacity ranging from 50000 to three million liters. in an increasingly global environment, the plasma industry has recently gone through a major consolidation phase that has seen mergers and acquisitions, and has led to the closure of a number of small plants in europe. currently, some fifteen countries are in ... | 2007 | 17499539 |
| blood transfusion and spread of variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) may be transmissible by blood. to prevent secondary transmission through blood components, several countries have started to exclude as donors persons who have received a blood transfusion. we investigated the effectiveness of this measure by using a dynamic age-structured model. it is the first such model based on epidemiologic data: 1) blood donor activities, 2) a case-control study on cjd, 3) age distribution of recipients, and 4) death of recipients o ... | 2007 | 17370520 |
| blood-transmitted prions and variant creutzfeldt-jakob disease. | | 2007 | 17317213 |
| long term survival in a patient with variant creutzfeldt-jakob disease treated with intraventricular pentosan polysulphate. | variant creutzfeldt-jakob disease (vcjd) is a neurodegenerative disease that principally affects young people and has a median duration of illness of 13 (range 6-39) months. vcjd is incurable and there are currently no treatments that conclusively slow the rate of disease progression. however, recent animal studies and isolated case reports have suggested that treatment with intraventricular pentosan polysulphate (pps) may be beneficial in the treatment of patients with vcjd. we report a case of ... | 2007 | 17314188 |
| changing indications for red cell transfusion from 2000 to 2004 in the north of england. | falling donor numbers and the threat of transfusion-transmitted variant creutzfeldt-jakob disease may lead to shortages in the national blood supply. knowledge of current patterns of transfusion and trends in usage will help predict future change in blood use. our previous survey identified medical indications as the major reason for transfusion, but detailed information within this category was limited. we performed prospective surveys of indications for red cell transfusion for two 14-day peri ... | 2006 | 17163872 |
| three reported cases of variant creutzfeldt-jakob disease transmission following transfusion of labile blood components. | | 2006 | 17105612 |
| anaesthesia in patients with dementia. | the next couple of decades will be characterized by an increase in life expectancy, leading to an older population. as the incidence of alzheimer's dementia and vascular dementia is rising with age, the future anaesthesiologist will be increasingly confronted with perioperative care of patients with impaired cognitive function. this paper tries to highlight some topics specifically related to demented patients. | 2004 | 17021564 |
| factors determining the potential for onward transmission of variant creutzfeldt-jakob disease via surgical instruments. | while the number of variant creutzfeldt-jakob disease (vcjd) cases continues to decline, concern has been raised that transmission could occur directly from one person to another through routes including the transfer of blood and shared use of surgical instruments. here we firstly present data on the surgical procedures undertaken on vcjd patients prior to onset of clinical symptoms, which supports the hypothesis that cases via this route are possible. we then apply a mathematical framework to a ... | 2006 | 17015298 |
| does the surface property of a disposable applanation tonometer account for its underestimation of intraocular pressure when compared with the goldmann tonometer? | disposable tonometers are increasingly being adopted partly because of concerns over the transmission of variant creutzfeldt-jakob disease and partly for convenience. recently, we have found one such tonometer (tonojet by luneau ophthalmologie, france) underestimated the intraocular pressure (iop). | 2007 | 16912886 |
| an outline of the neuropathology of transmissible spongiform encephalopathies (prion diseases). | we review here the basic neuropathology of transmissible spongiform encephalopathies (tse) or prion diseases. the classic hallmark of tse neuropathology is a combination (in different proportions in different diseases) of spongiform change, astrocytosis, neuronal loss and amyloid plaques. immunohistochemically, accumulation of the abnormal isoform of prion protein (prp(sc) or prp(d)) is regarded as a diagnostic for tse. we also review the peculiarities of kuru, variant creutzfeldt-jakob disease ... | 2004 | 16903141 |
| clinical findings and diagnostic tests in creutzfeldt-jakob disease and variant creutzfeldt-jakob disease. | sporadic creutzfeldt-jakob disease (scjd) is a rare transmissible disease caused by accumulation of pathological prion protein in the cns. scjd typically affects patients in their sixties. the median disease duration in scjd (6 months) is shorter than in variant creutzfeldt-jakob disease (vcjd) (14 months). the clinical diagnosis in scjd is supported by the detection of periodic sharp and slow wave complexes (pswc) in the electroencephalogram, 14-3-3 proteins in the cerebrospinal fluid (csf) and ... | 2004 | 16903140 |
| can a second wave of new variant of the cjd be discarded in absence of observation of clinical non met-met cases? | presently, all patients with clinical variant creutzfeldt-jakob disease in the united kingdom have been met-met at codon 129 of the prp gene. there is much worry about the possibility of a second wave of the epidemic in the 60% of the united kingdom population which are not met-met. | 2006 | 16830965 |
| size frequency distributions of the florid prion protein aggregates in variant creutzfeldt-jakob disease follow a power-law function. | the objective was to test the hypothesis that the size frequency distributions of the prion protein (prp) plaques in cases of variant creutzfeldt-jakob disease (vcjd) follow a power-law function. the design was a retrospective neuropathological study. the patients were 11 cases of clinically and neuropathologically verified vcjd. size distributions of the diffuse and florid-type plaques were measured in several areas of the cerebral cortex and hippocampus from each case and a power-law function ... | 2006 | 16816906 |
| variant creutzfeldt-jakob disease in the united kingdom and elsewhere: situation at the end of 2005. | | 2006 | 16801715 |
| surface decontamination of surgical instruments: an ongoing dilemma. | the issues of cross-infection and the survival of variant creutzfeldt jakob disease (vcjd) on surgical instruments have highlighted the importance of cleanliness of multiple-use surgical instruments. the aim of this study was to assess the levels of total protein contamination on a wide range of surgical instruments as an indication of the effectiveness of routine cleaning and disinfection in hospitals. anonymized trays of wrapped and autoclaved instruments were supplied to two laboratories for ... | 2006 | 16759745 |
| blood audit evidenced-based cross-match requesting for lumbar spine surgery. | complete audit cycle. | 2006 | 16721282 |
| a note on parameter estimation for variant creutzfeldt-jakob disease epidemic models. | a recent series of papers has raised issues regarding estimation of the key epidemiological parameters of variant creutzfeldt-jakob disease (vcjd) from fitting survival models to case data. in particular, it was stated that the scale of the epidemic cannot be estimated and must be fixed in any analysis. we show that this problem is an artefact of the approximate likelihood used in these papers to facilitate model-fitting, and is not a concern if estimation is based on the full likelihood. we als ... | 2007 | 16612835 |
| variant creutzfeldt-jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. | to perform prion protein gene (prnp) codon 129 analysis in dna extracted from appendix tissue samples that had tested positive for disease associated prion protein. | 2006 | 16606639 |
| new variant creutzfeldt-jakob disease: a controversial but potential blood transfusion risk. | | 2003 | 16583978 |
| donor selection criteria to maximize double platelet products (dpp) by platelet apheresis. | variant creutzfeldt-jakob disease brought us to perform a study to diminish donor exposure from transfusion of platelet concentrates. the current study aimed to develop donor selection criteria that maximize the likelihood of deriving single donor platelets and producing double platelet products (dpp). donors were recruited among plasmapheresis donors and among other donors when the selected donors did not show up. donor precount and body weight and haematocrit were examined as determinants of h ... | 2006 | 16574489 |
| predictive ability of sequential surveys in determining donor loss from increasingly stringent variant creutzfeldt-jakob disease deferral policies. | predonation screening questions about travel increase the safety of the blood supply from diseases such as variant creutzfeldt-jakob disease (vcjd) and malaria. this study examines the ability of sequential surveys to predict actual travel deferrals and the operational validity of travel questions. | 2006 | 16533291 |
| the effects of leukodepletion on the generation and removal of microvesicles and prion protein in blood components. | universal leukodepletion (ld) has been implemented in the united kingdom to reduce the risk of transfusion-transmitted variant creutzfeldt-jakob disease. if ld causes microvesiculation of blood cells, however, potentially infectious membrane-associated prion could reach the final products. | 2006 | 16533284 |
| the alternative role of 14-3-3 zeta as a sweeper of misfolded proteins in disease conditions. | here, we propose a novel hypothesis that 14-3-3 zeta might act as a sweeper of misfolded proteins by facilitating the formation of aggregates, which are referred to as inclusion bodies. studies on the localization of the 14-3-3 proteins in different types of inclusion bodies in the brain including neurofibrillary tangle in alzheimer's disease, pick bodies in pick's disease, lewy body-like hyaline inclusions in sporadic amyotrophic lateral sclerosis, prion/florid plaques in sporadic/variant creut ... | 2006 | 16516399 |
| [variant creutzfeldt-jakob disease, 10 years later]. | | 2006 | 16462658 |
| periodic electroencephalogram complexes in a patient with variant creutzfeldt-jakob disease. | based on the current criteria, the diagnosis of "possible" or "probable" variant creutzfeldt-jakob disease (vcjd) implies the absence of periodic sharp wave complexes (pswcs) in the electroencephalogram (eeg). to verify this point, we investigated the development of the eeg changes along the course of the disease in a pateint with vcjd. | 2006 | 16437565 |
| short te quantitative proton magnetic resonance spectroscopy in variant creutzfeldt-jakob disease. | variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative disorder. clinical diagnosis is difficult in the early stages as the disease often presents with non-specific psychiatric and neurological symptoms. to investigate the diagnostic potential of quantitative short te in vivo mrs, and the nature and anatomical distribution of biochemical abnormalities in vcjd, localised single-voxel spectra (te/tr 30 ms/2,000 ms) were acquired from three brain regions: thalami, caudate nuclei and ... | 2006 | 16408201 |