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Creutzfeldt-jakob disease

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https://www.readbyqxmd.com/read/28240664/creutzfeldt-jakob-disease-mimicking-alzheimer-disease-and-dementia-with-lewy-bodies-findings-of-fdg-pet-with-3-dimensional-stereotactic-surface-projection
#1
Nobuhiko Miyazawa
A 78-year-old man received a diagnosis of sporadic Creutzfeldt-Jakob disease based on symptoms and findings of MRI, FDG PET, and cerebrospinal fluid markers. PET with 3-dimensional stereotactic surface projection (3D-SSP) showed that the distribution of hypometabolism mimicked that of Alzheimer disease. A 68-year-old woman was treated under a diagnosis of convulsion. Findings of MRI, PET, familial history, and cerebrospinal fluid markers revealed familial Creutzfeldt-Jakob disease. FDG PET with 3D-SSP disclosed that the hypometabolic pattern mimicked that of dementia with Lewy bodies...
February 24, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28236445/the-heidenhain-variant-of-creutzfeldt-jakob-disease-and-concomitant-tau-pathology-a-case-report
#2
Edvard Ehler, Michael Pipka, Alena Meleková, Petra Mandysová, Silvie Johanidesová, Radoslav Matěj, Robert Rusina
The Heidenhain form of Creutzfeldt-Jakob disease (CJD) is a rare CJD variant with predominantly visual symptoms in the early stages. Clinical manifestations of metamorphopsia, hemianopia and Balint's syndrome correlate with the involvement of the posterior cortical regions. A 71-year old healthy and very active man was admitted because of impaired visual acuity, hemianopia, and gait disturbance progressing over one week. MRI found typical cortical hyperintensities in the occipital regions while rhythm slowing and sharp waves were seen in the occipital regions on EEG...
February 10, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28231300/detection-and-partial-discrimination-of-atypical-and-classical-bovine-spongiform-encephalopathies-in-cattle-and-primates-using-real-time-quaking-induced-conversion-assay
#3
Etienne Levavasseur, Anne-Gaëlle Biacabe, Emmanuel Comoy, Audrey Culeux, Katarina Grznarova, Nicolas Privat, Steve Simoneau, Benoit Flan, Véronique Sazdovitch, Danielle Seilhean, Thierry Baron, Stéphane Haïk
The transmission of classical bovine spongiform encephalopathy (C-BSE) through contaminated meat product consumption is responsible for variant Creutzfeldt-Jakob disease (vCJD) in humans. More recent and atypical forms of BSE (L-BSE and H-BSE) have been identified in cattle since the C-BSE epidemic. Their low incidence and advanced age of onset are compatible with a sporadic origin, as are most cases of Creutzfeldt-Jakob disease (CJD) in humans. Transmissions studies in primates and transgenic mice expressing a human prion protein (PrP) indicated that atypical forms of BSE may be associated with a higher zoonotic potential than classical BSE, and require particular attention for public health...
2017: PloS One
https://www.readbyqxmd.com/read/28211814/are-major-dementias-triggered-by-poor-blood-flow-to-the-brain-theoretical-considerations
#4
Jack C de la Torre
There is growing evidence that chronic brain hypoperfusion plays a central role in the development of Alzheimer's disease (AD) long before dyscognitive symptoms or amyloid-β accumulation in the brain appear. This commentary proposes that dementia with Lewy bodies (DLB), frontotemporal dementia (FTD), and Creutzfeldt-Jakob disease (CJD) may also develop from chronic brain hypoperfusion following a similar but not identical neurometabolic breakdown as AD. The argument to support this conclusion is that chronic brain hypoperfusion, which is found at the early stages of the three dementias reviewed here, will reduce oxygen delivery and lower oxidative phosphorylation promoting a steady decline in the synthesis of the cell energy fuel adenosine triphosphate (ATP)...
February 15, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28205010/prion-specific-and-surrogate-csf-biomarkers-in-creutzfeldt-jakob-disease-diagnostic-accuracy-in-relation-to-molecular-subtypes-and-analysis-of-neuropathological-correlates-of-p-tau-and-a%C3%AE-42-levels
#5
Francesca Lattanzio, Samir Abu-Rumeileh, Alessia Franceschini, Hideaki Kai, Giulia Amore, Ilaria Poggiolini, Marcello Rossi, Simone Baiardi, Lynne McGuire, Anna Ladogana, Maurizio Pocchiari, Alison Green, Sabina Capellari, Piero Parchi
The differential diagnosis of Creutzfeldt-Jakob disease (CJD) from other, sometimes treatable, neurological disorders is challenging, owing to the wide phenotypic heterogeneity of the disease. Real-time quaking-induced prion conversion (RT-QuIC) is a novel ultrasensitive in vitro assay, which, at variance with surrogate neurodegenerative biomarker assays, specifically targets the pathological prion protein (PrP(Sc)). In the studies conducted to date in CJD, cerebrospinal fluid (CSF) RT-QuIC showed good diagnostic sensitivity (82-96%) and virtually full specificity...
February 15, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28193766/molecular-mechanisms-of-chronic-wasting-disease-prion-propagation
#6
Julie A Moreno, Glenn C Telling
Prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. Examples include kuru, once the leading cause of death among the Fore people in Papua New Guinea and caused by mortuary feasting; bovine spongiform encephalopathy (BSE) and its subsequent transmission to humans in the form of variant Creutzfeldt-Jakob disease (vCJD), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. The etiology of chronic wasting disease (CWD), a relatively new and burgeoning prion epidemic in deer, elk, and moose (members of the cervid family), is more enigmatic...
February 13, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28178353/cystatin-f-is-a-biomarker-of-prion-pathogenesis-in-mice
#7
Mario Nuvolone, Nicolas Schmid, Gino Miele, Silvia Sorce, Rita Moos, Christian Schori, Roger R Beerli, Monika Bauer, Philippe Saudan, Klaus Dietmeier, Ingolf Lachmann, Michael Linnebank, Roland Martin, Ulf Kallweit, Veronika Kana, Elisabeth J Rushing, Herbert Budka, Adriano Aguzzi
Misfolding of the cellular prion protein (PrPC) into the scrapie prion protein (PrPSc) results in progressive, fatal, transmissible neurodegenerative conditions termed prion diseases. Experimental and epidemiological evidence point toward a protracted, clinically silent phase in prion diseases, yet there is no diagnostic test capable of identifying asymptomatic individuals incubating prions. In an effort to identify early biomarkers of prion diseases, we have compared global transcriptional profiles in brains from pre-symptomatic prion-infected mice and controls...
2017: PloS One
https://www.readbyqxmd.com/read/28176658/a-structural-view-of-%C3%AE-b-crystallin-assembly-and-amyloid-aggregation
#8
Zhenying Liu, Shengnan Zhang, Dan Li, Cong Liu
The major len protein αB-crystallin (αB) is an intracellular chaperone. It belongs to the family of small heat shock protein (sHsps) which plays a critical role in maintaining protein homeostasis and preventing protein aggregation, especially under stress conditions. Dysfunction of αB is closely related to cataract, and many neurodegenerative diseases including Alzheimer's, Parkinson's, and Creutzfeldt-Jakob disease. Due to the extremely heterogeneous and polydispersed nature of αB, it remains unclear how αB self-assemblies and prevents its client proteins from aggregation...
February 6, 2017: Protein and Peptide Letters
https://www.readbyqxmd.com/read/28176535/creutzfeldt-jakob-disease-cerebral-amyloid-angiopathy-and-a%C3%AE-related-angiitis-with-neuropsychiatric-manifestations
#9
Gabrielle Matta, Dennis Velakoulis, Frank Gaillard, Catriona A McLean, Raju Yerra
No abstract text is available yet for this article.
February 1, 2017: Australian and New Zealand Journal of Psychiatry
https://www.readbyqxmd.com/read/28168213/extended-and-direct-evaluation-of-rt-quic-assays-for-creutzfeldt-jakob-disease-diagnosis
#10
Bradley R Groveman, Christina D Orrú, Andrew G Hughson, Matilde Bongianni, Michele Fiorini, Daniele Imperiale, Anna Ladogana, Maurizio Pocchiari, Gianluigi Zanusso, Byron Caughey
Real-Time Quaking-Induced Conversion (RT-QuIC) testing of human cerebrospinal fluid (CSF) is highly sensitive and specific in discriminating sporadic CJD patients from those without prion disease. Here, using CSF samples from 113 CJD and 64 non-prion disease patients, we provide the first direct and concurrent comparison of our improved RT-QuIC assay to our previous assay, which is similar to those commonly used internationally for CJD diagnosis. This extended comparison demonstrated a ~21% increase in diagnostic sensitivity, a 2-day reduction in average detection time, and 100% specificity...
February 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28153848/cjd-mimics-and-chameleons
#11
REVIEW
Simon Mead, Peter Rudge
Rapidly progressive dementia mimicking Creutzfeldt-Jakob disease (CJD) is a relatively rare presentation but a rewarding one to become familiar with, as the potential diagnoses range from the universally fatal to the completely reversible. Patients require urgent decisions about assessment and investigation and have quickly evolving needs for treatments and support, through symptom management and end-of-life care in most cases. We have based this pragmatic review on the experiences of a specialist prion referral centre in the UK, which, unsurprisingly, is strongly biased towards seeing patients with CJD...
February 2, 2017: Practical Neurology
https://www.readbyqxmd.com/read/28151391/variant-creutzfeldt-jakob-disease-deferral-in-canada-impact-of-stop-dates
#12
Sheila F O'brien, Wenli Fan, Qi-Long Yi, Samra Uzicanin, Lori Osmond, Mindy Goldman
BACKGROUND: To reduce the risk of variant Creutzfeldt-Jakob disease (vCJD) transmission via blood transfusion in Canada, potential donors who spent a cumulative time in the United Kingdom, Western Europe or Saudi Arabia are deferred. "Stop dates" for accumulated time were later implemented for 3 months spent in the United Kingdom or France (1980-1996) and for 5 years elsewhere in Western Europe (1980-2007); Saudi Arabia deferral was implemented with the "stop date" (1980-1996). We evaluated the long-term impact of these deferrals and "stop dates", as well as the consistency of donors' answers to post-implementation screening questions...
December 15, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28144628/de-novo-generation-of-a-unique-cervid-prion-strain-using-protein-misfolding-cyclic-amplification
#13
Crystal Meyerett-Reid, A Christy Wyckoff, Terry Spraker, Bruce Pulford, Heather Bender, Mark D Zabel
Substantial evidence supports the hypothesis that prions are misfolded, infectious, insoluble, and protease-resistant proteins (PrP(RES)) devoid of instructional nucleic acid that cause transmissible spongiform encephalopathies (TSEs). Protein misfolding cyclic amplification (PMCA) has provided additional evidence that PrPRes acts as a template that can convert the normal cellular prion protein (PrP(C)) present in uninfected normal brain homogenate (NBH) into the infectious misfolded PrP(RES) isoform. Human PrP(C) has been shown to spontaneously convert to a misfolded pathological state causing sporadic Creutzfeldt-Jakob disease (sCJD)...
January 2017: MSphere
https://www.readbyqxmd.com/read/28139079/current-evidence-on-the-transmissibility-of-chronic-wasting-disease-prions-to-humans-a-systematic-review
#14
REVIEW
L Waddell, J Greig, M Mascarenhas, A Otten, T Corrin, K Hierlihy
A number of prion diseases affect humans, including Creutzfeldt-Jakob disease; most of these are due to genetic mutations in the affected individual and occur sporadically, but some result from transmission of prion proteins from external sources. Of the known animal prion diseases, only bovine spongiform encephalopathy prions have been shown to be transmissible from animals to humans under non-experimental conditions. Chronic wasting disease (CWD) is a prion disease that affects cervids (e.g., deer and elk) in North America and isolated populations in Korea and Europe...
January 30, 2017: Transboundary and Emerging Diseases
https://www.readbyqxmd.com/read/28121634/fluorodeoxyglucose-positron-emission-tomography-fdg-pet-correlation-of-histopathology-and-mri-in-prion-disease
#15
Karin P Mente, James K O'Donnell, Stephen E Jones, Mark L Cohen, Nicolas R Thompson, Alberto Bizzi, Pierluigi Gambetti, Jiri G Safar, Brian S Appleby
Creutzfeldt-Jakob disease (CJD) and other prion diseases are rapidly progressive spongiform encephalopathies that are invariably fatal. Clinical features and magnetic resonance imaging, electroencephalogram, and cerebrospinal fluid abnormalities may suggest prion disease, but a definitive diagnosis can only be made by means of neuropathologic examination. Fluorodeoxyglucose positron emission tomography (FDG-PET) is not routinely used to evaluate patients with suspected prion disease. This study includes 11 cases of definite prion disease in which FDG-PET scans were obtained...
January 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/28116677/a-heparin-purification-process-removes-spiked-transmissible-spongiform-encephalopathy-agent
#16
Cyrus Bett, Ksenija Grgac, Dianna Long, Michael Karfunkle, David A Keire, David M Asher, Luisa Gregori
In 2000, bovine heparin was withdrawn from the US market for fear of contamination with bovine spongiform encephalopathy (BSE) agent, the cause of variant Creutzfeldt-Jakob disease in humans. Thus, US heparin is currently sourced only from pig intestines. Availability of alternative sources of crude heparin, a life-saving drug, would benefit public health. Bovine heparin is an obvious option, but BSE clearance by the bovine heparin manufacturing process should be evaluated. To this end, using hamster 263K scrapie as a surrogate for BSE agent, we applied a four-step bench-scale heparin purification protocol resembling a typical heparin manufacturing process to investigate removal of the spiked scrapie agent...
January 23, 2017: AAPS Journal
https://www.readbyqxmd.com/read/28110369/neuroradiology-of-human-prion-diseases-diagnosis-and-differential-diagnosis
#17
REVIEW
Simona Gaudino, Emma Gangemi, Raffaella Colantonio, Annibale Botto, Emanuela Ruberto, Rosalinda Calandrelli, Matia Martucci, Maria Gabriella Vita, Carlo Masullo, Alfonso Cerase, Cesare Colosimo
Human transmissible spongiform encephalopathies (TSEs), or prion diseases, are invariably fatal conditions associated with a range of clinical presentations. TSEs are classified as sporadic [e.g. sporadic Creutzfeldt-Jakob disease (sCJD), which is the most frequent form], genetic (e.g. Gerstmann-Straussler-Scheinker disease, fatal familial insomnia, and inherited CJD), and acquired or infectious (e.g. Kuru, iatrogenic CJD, and variant CJD). In the past, brain imaging played a supporting role in the diagnosis of TSEs, whereas nowadays magnetic resonance imaging (MRI) plays such a prominent role that MRI findings have been included in the diagnostic criteria for sCJD...
January 21, 2017: La Radiologia Medica
https://www.readbyqxmd.com/read/28109330/the-structure-of-mammalian-prions-and-their-aggregates
#18
E Vázquez-Fernández, H S Young, J R Requena, H Wille
Prion diseases, such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (i.e., deer, elk, moose, and reindeer), and sheep scrapie, are caused by the misfolding of the cellular prion protein (PrP(C)) into a disease-causing conformer (PrP(Sc)). PrP(C) is a normal, GPI-anchored protein that is expressed on the surface of neurons and other cell types. The structure of PrP(C) is well understood, based on studies of recombinant PrP, which closely mimics the structure of native PrP(C)...
2017: International Review of Cell and Molecular Biology
https://www.readbyqxmd.com/read/28096244/experimental-models-of-inherited-prp-prion-diseases
#19
Joel C Watts, Stanley B Prusiner
The inherited prion protein (PrP) prion disorders, which include familial Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia, constitute ∼10%-15% of all PrP prion disease cases in humans. Attempts to generate animal models of these disorders using transgenic mice expressing mutant PrP have produced variable results. Although many lines of mice develop spontaneous signs of neurological illness with accompanying prion disease-specific neuropathological changes, others do not...
January 17, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28095474/anti-prion-screening-for-acridine-dextran-and-tannic-acid-using-real-time-quaking-induced-conversion-a-comparison-with-prpsc-infected-cell-screening
#20
Jae Wook Hyeon, Su Yeon Kim, Sol Moe Lee, Jeongmin Lee, Seong Soo A An, Myung Koo Lee, Yeong Seon Lee
Prion propagation is mediated by the structural alteration of normal prion protein (PrPC) to generate pathogenic prion protein (PrPSc). To date, compounds for the inhibition of prion propagation have mainly been screened using PrPSc-infected cells. Real time-quaking-induced conversion (RT-QuIC) is one alternative screening method. In this study, we assessed the propagation inhibition effects of known anti-prion compounds using RT-QuIC and compared the results with those from a PrPSc-infected cell assay. Compounds were applied to RT-QuIC reactions at 0 h or 22 h after prion propagation to determine whether they inhibited propagation or reduced amplified aggregates...
2017: PloS One
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