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Creutzfeldt-Jakob

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https://www.readbyqxmd.com/read/28096244/experimental-models-of-inherited-prp-prion-diseases
#1
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
#2
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
https://www.readbyqxmd.com/read/28092933/estimation-of-the-size-of-the-iatrogenic-creutzfeldt-jakob-disease-outbreak-associated-with-cadaveric-dura-mater-grafts-in-korea
#3
Byoug-Hak Jeon, Jinseob Kim, GangHyun Kim Kim, Soo Chul Park, SangYun Kim, Hae-Kwan Cheong
Purpose: This study estimated the overall magnitude of Creutzfeldt-Jakob disease (iCJD) based on dura graft cases in Korea using a mathematical model. Methods: We estimated the number of annual cases of dura grafts performed between 1980 and 1995 by applying the proportion of dura grafts recorded by the Health Insurance Review Agency claim dataset in Korea to the number of nationwide neurosurgery cases. The distribution of the incubation period was assumed to fall under a Weibull distribution with density function or a log-logistic distribution with density function...
December 19, 2016: Epidemiology and Health
https://www.readbyqxmd.com/read/28091514/novel-strain-properties-distinguishing-sporadic-prion-diseases-sharing-prion-protein-genotype-and-prion-type
#4
Laura Cracco, Silvio Notari, Ignazio Cali, Man-Sun Sy, Shu G Chen, Mark L Cohen, Bernardino Ghetti, Brian S Appleby, Wen-Quan Zou, Byron Caughey, Jiri G Safar, Pierluigi Gambetti
In most human sporadic prion diseases the phenotype is consistently associated with specific pairings of the genotype at codon 129 of the prion protein gene and conformational properties of the scrapie PrP (PrP(Sc)) grossly identified types 1 and 2. This association suggests that the 129 genotype favours the selection of a distinct strain that in turn determines the phenotype. However, this mechanism cannot play a role in the phenotype determination of sporadic fatal insomnia (sFI) and a subtype of sporadic Creutzfeldt-Jakob disease (sCJD) identified as sCJDMM2, which share 129 MM genotype and PrP(Sc) type 2 but are associated with quite distinct phenotypes...
January 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28076019/viral-and-prion-infections-of-the-central-nervous-system-radiologic-pathologic-correlation-from-the-radiologic-pathology-archives
#5
Kelly K Koeller, Robert Y Shih
Viral infections of the central nervous system (CNS) range in clinical severity, with the most severe proving fatal within a matter of days. Some of the more than 100 different viruses known to affect the brain and spinal cord are neurotropic with a predilection for producing CNS infection. The host response to viral infection of the CNS is responsible for the pathophysiology and imaging findings seen in affected patients. Viral CNS infections can take the form of meningitis, encephalitis, encephalomyelitis, or, when involving the spinal cord and nerve roots, encephalomyeloradiculitis...
January 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28076012/imaging-of-creutzfeldt-jakob-disease-imaging-patterns-and-their-differential-diagnosis
#6
Diego Cardoso Fragoso, Augusto Lio da Mota Gonçalves Filho, Felipe Torres Pacheco, Bernardo Rodi Barros, Ingrid Aguiar Littig, Renato Hoffmann Nunes, Antônio Carlos Martins Maia Júnior, Antonio J da Rocha
Diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) remains a challenge because of the large variability of the clinical scenario, especially in its early stages, which may mimic several reversible or treatable disorders. The molecular basis of prion disease, as well as its brain propagation and the pathogenesis of the illness, have become better understood in recent decades. Several reports have listed recognizable clinical features and paraclinical tests to supplement the replicable diagnostic criteria in vivo...
January 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28072756/rapid-testing-for-creutzfeldt-jakob-disease-in-donors-of-cornea
#7
Luisa Gregori, Arthur Serer, Kristy L McDowell, Juraj Cervenak, David M Asher
BACKGROUND: Creutzfeldt-Jakob disease (CJD) has been accidentally transmitted by contaminated corneal transplants. Eye donors are not ordinarily tested for CJD, in part because an easy test is not available. We propose a relatively simple postmortem procedure to collect brain samples without performing full autopsy and show that a test currently marketed for veterinary diagnosis would offer an effective screening test. METHODS: We selected 6 brains from confirmed cases of human sporadic CJD and sampled each in triplicate (18 specimens), 28 control brains of individuals with non-CJD neurodegenerative diseases and 10 normal brains...
January 9, 2017: Transplantation
https://www.readbyqxmd.com/read/28059790/combined-creutzfeldt-jakob-jacob-alzheimer-s-disease-cases-are-important-in-search-for-microbes-in-alzheimer-s-disease
#8
Frank O Bastian
The question whether Alzheimer's disease is infectious as brought up in the recent editorial published in the Journal of Alzheimer's Disease is complicated by the controversy whether the causal agent is a microbe or a misfolded host protein (amyloid). The replicating amyloid (prion) theory, based upon data from studies of Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs), has been challenged since the prion can be separated from TSE infectivity, and spiroplasma, a wall-less bacterium, has been shown to be involved in the pathogenesis of CJD...
December 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28056460/cerebrospinal-fluid-biomarker-based-diagnosis-of-sporadic-creutzfeldt-jakob-disease-a-validation-study-for-previously-established-cutoffs
#9
Franc Llorens, André Karch, Ewa Golanska, Matthias Schmitz, Peter Lange, Beata Sikorska, Pawel P Liberski, Inga Zerr
BACKGROUND: Several biomarkers have been proposed to discriminate sporadic Creutzfeldt-Jakob disease (sCJD) from other dementias and control cases. However, their clinical accuracy depends on the PRNP codon 129 genotype, leaving it unclear how well established markers behave in untested conditions. METHODS: We analyzed 14-3-3, tau, p-tau levels, and the p-tau/tau ratio in a population sample collected from Polish hospitals including nondementia, dementia, and definite sCJD cases and validated their parameters according to previously established cutoffs...
January 6, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28049644/functional-prions-in-the-brain
#10
REVIEW
Joseph B Rayman, Eric R Kandel
Prions are proteins that can adopt self-perpetuating conformations and are traditionally regarded as etiological agents of infectious neurodegenerative diseases in humans, such as Creutzfeldt-Jakob disease, kuru, and transmissible encephalopathies. More recently, a growing consensus has emerged that prion-like, self-templating mechanisms also underlie a variety of neurodegenerative disorders, including amyotrophic lateral sclerosis, Alzheimer's disease, and Huntington's disease. Perhaps most surprising, not all prion-like aggregates are associated with pathological changes...
January 3, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28028861/creutzfeldt-jakob-disease
#11
REVIEW
Yasushi Iwasaki 岩崎 靖
This review will explore the clinical and pathological findings of the various forms of Creutzfeldt-Jakob disease (CJD). Clinical findings of CJD are characterized by rapidly progressive cognitive dysfunction, diffusion-weighted magnetic resonance imaging (DWI) hyperintensity, myoclonus, periodic sharp-wave complexes on electroencephalogram and akinetic mutism state. Neuropathologic findings of CJD are characterized by spongiform changes in gray matter, gliosis-particularly hypertrophic astrocytosis-neuropil rarefaction, neuron loss and prion protein (PrP) deposition...
December 28, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28003548/detection-of-prions-in-blood-from-patients-with-variant-creutzfeldt-jakob-disease
#12
Luis Concha-Marambio, Sandra Pritzkow, Fabio Moda, Fabrizio Tagliavini, James W Ironside, Paul E Schulz, Claudio Soto
Human prion diseases are infectious and invariably fatal neurodegenerative diseases. They include sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant CJD (vCJD), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. Development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (PrP(Sc)) in the blood of patients affected by prion disease is a top medical priority to increase the safety of the blood supply...
December 21, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/28003547/detection-of-prions-in-the-plasma-of-presymptomatic-and-symptomatic-patients-with-variant-creutzfeldt-jakob-disease
#13
Daisy Bougard, Jean-Philippe Brandel, Maxime Bélondrade, Vincent Béringue, Christiane Segarra, Hervé Fleury, Jean-Louis Laplanche, Charly Mayran, Simon Nicot, Alison Green, Arlette Welaratne, David Narbey, Chantal Fournier-Wirth, Richard Knight, Robert Will, Pierre Tiberghien, Stéphane Haïk, Joliette Coste
Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease resulting from the consumption of meat products contaminated by the agent causing bovine spongiform encephalopathy. Evidence supporting the presence of a population of silent carriers that can potentially transmit the disease through blood transfusion is increasing. The development of a blood-screening assay for both symptomatic vCJD patients and asymptomatic carriers is urgently required. We show that a diagnostic assay combining plasminogen-bead capture and protein misfolding cyclic amplification (PMCA) technologies consistently detected minute amounts of abnormal prion protein from French and British vCJD cases in the required femtomolar range...
December 21, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/28003435/genetic-compendium-of-1511-human-brains-available-through-the-uk-medical-research-council-brain-banks-network-resource
#14
Michael J Keogh, Wei Wei, Ian Wilson, Jon Coxhead, Sarah Ryan, Sara Rollinson, Helen Griffin, Marzena Kurzawa-Akanbi, Mauro Santibanez-Koref, Kevin Talbot, Martin R Turner, Chris-Anne McKenzie, Claire Troakes, Johannes Attems, Colin Smith, Safa Al Sarraj, Chris M Morris, Olaf Ansorge, Stuart Pickering-Brown, James W Ironside, Patrick F Chinnery
Given the central role of genetic factors in the pathogenesis of common neurodegenerative disorders, it is critical that mechanistic studies in human tissue are interpreted in a genetically enlightened context. To address this, we performed exome sequencing and copy number variant analysis on 1511 frozen human brains with a diagnosis of Alzheimer's disease (AD, n = 289), frontotemporal dementia/amyotrophic lateral sclerosis (FTD/ALS, n = 252), Creutzfeldt-Jakob disease (CJD, n = 239), Parkinson's disease (PD, n = 39), dementia with Lewy bodies (DLB, n = 58), other neurodegenerative, vascular, or neurogenetic disorders (n = 266), and controls with no significant neuropathology (n = 368)...
January 2017: Genome Research
https://www.readbyqxmd.com/read/27997483/familial-creutzfeldt-jakob-disease-cluster-among-an-african-american-family
#15
Matthew G Johnson, Kristy K Bradley, Rebecca L Coffman, Ermias D Belay
Familial Creutzfeldt-Jakob disease (fCJD) results from inheritance of mutations in the prion protein gene. Confirming fCJD diagnosis is essential for informing persons of their potential hereditary risk and for genetic counseling to support personal decisions for genetic testing and family planning. We describe a case of fCJD that was linked to a large cluster of African Americans with fCJD identified through a public health investigation, including 8 confirmed cases and 13 suspected cases involving 7 generations in 1 family...
December 16, 2016: Journal of Public Health Management and Practice: JPHMP
https://www.readbyqxmd.com/read/27978902/-myoclonus-and-it-s-associated-factors-in-creutzfeldt-jakob-disease
#16
J Liu, H X Wang, L P Li, X Hong, A H Liu, J Ye, L Song, J Zhang, J Li, N N Hu, Z C Sun, S R Li, Y H Yang, H Q Dong, Y P Wang
Objective: To analyze features and the related factors of myoclonus of 47 patients with probable or possible Creutzfeldt-Jakob disease (CJD). Methods: All patients diagnosed with"suspected CJD" and hospitalized in Xuanwu Hospital from January 2013 to November 2015 were included, and their clinical information and myoclonus features were analyzed. Age, clinical, course and manifestation, EEG, MRI, CSF14-3-3 features between myoclonus positive group and negative group were compared, and the correlation between myoclonus features and these factors was analyzed using Spearman correlation analyses...
December 6, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27976788/-delayed-diagnosis-in-a-patient-with-creutzfeldt-jakob-disease-in-a-psychiatric-hospital
#17
S Roest, I Mestdagh, C de Grave, P Pals
A 51-year-old female teacher of dance was referred to the diagnostic unit of our psychiatric hospital with symptoms of anxiety and depression. The clinical image was suggestive of organic pathology, but this could not be determined with certainty until a late stage. We discuss the course of the patient's illness. Her symptoms appeared to be psychiatric and closely resembled those of Creutzfeldt-Jakob disease. We comment on some of the signs that could have led to an earlier diagnosis and we discuss the tools that are needed...
2016: Tijdschrift Voor Psychiatrie
https://www.readbyqxmd.com/read/27975168/molecular-alterations-in-the-cerebellum-of-sporadic-creutzfeldt-jakob-disease-subtypes-with-dj-1-as-a-key-regulator-of-oxidative-stress
#18
Waqas Tahir, Saima Zafar, Franc Llorens, Amandeep Singh Arora, Katrin Thüne, Matthias Schmitz, Nadine Gotzmann, Niels Kruse, Brit Mollenhauer, Juan Maria Torres, Olivier Andréoletti, Isidre Ferrer, Inga Zerr
Cerebellar damage and granular and Purkinje cell loss in sporadic Creutzfeldt-Jakob disease (sCJD) highlight a critical involvement of the cerebellum during symptomatic progression of the disease. In this project, global proteomic alterations in the cerebellum of brain from the two most prevalent subtypes (MM1 and VV2) of sCJD were studied. Two-dimensional gel electrophoresis (2DE) coupled mass spectrometric identification revealed 40 proteins in MM1 and 43 proteins in VV2 subtype to be differentially expressed...
December 14, 2016: Molecular Neurobiology
https://www.readbyqxmd.com/read/27942718/diagnosis-of-human-prion-disease-using-real-time-quaking-induced-conversion-testing-of-olfactory-mucosa-and-cerebrospinal-fluid-samples
#19
Matilde Bongianni, Christina Orrù, Bradley R Groveman, Luca Sacchetto, Michele Fiorini, Giovanni Tonoli, Giorgio Triva, Stefano Capaldi, Silvia Testi, Sergio Ferrari, Annachiara Cagnin, Anna Ladogana, Anna Poleggi, Elisa Colaizzo, Dorina Tiple, Luana Vaianella, Santina Castriciano, Daniele Marchioni, Andrew G Hughson, Daniele Imperiale, Tatiana Cattaruzza, Gian Maria Fabrizi, Maurizio Pocchiari, Salvatore Monaco, Byron Caughey, Gianluigi Zanusso
Importance: Early and accurate in vivo diagnosis of Creutzfeldt-Jakob disease (CJD) is necessary for quickly distinguishing treatable from untreatable rapidly progressive dementias and for future therapeutic trials. This early diagnosis is becoming possible using the real-time quaking-induced conversion (RT-QuIC) seeding assay, which detects minute amounts of the disease-specific pathologic prion protein in cerebrospinal fluid (CSF) or olfactory mucosa (OM) samples. Objective: To develop an algorithm for accurate and early diagnosis of CJD by using the RT-QuIC assay on CSF samples, OM samples, or both...
December 12, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27942712/a-new-standard-for-the-laboratory-diagnosis-of-sporadic-creutzfeldt-jakob-disease
#20
Paul Brown
No abstract text is available yet for this article.
December 12, 2016: JAMA Neurology
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