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https://www.readbyqxmd.com/read/29225787/creutzfeldt-jakob-disease-recent-developments
#1
REVIEW
Graeme Mackenzie, Robert Will
Creutzfeldt-Jakob disease (CJD) is a rare prion disorder that has been the subject of both professional and public interest following the identification of variant CJD as a zoonotic disorder. There have been recent advances in diagnostic techniques, including real-time quaking-induced conversion and magnetic resonance imaging brain scan, that have allowed more accurate case recognition in all forms of CJD. Although the epidemic of variant CJD is clearly in decline, prevalence studies suggest that it may be premature to be complacent about concerns for public health...
2017: F1000Research
https://www.readbyqxmd.com/read/29190276/public-health-risks-from-subclinical-variant-cjd
#2
Abigail B Diack, Robert G Will, Jean C Manson
No abstract text is available yet for this article.
November 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/29169405/atypical-creutzfeldt-jakob-disease-with-prp-amyloid-plaques-in-white-matter-molecular-characterization-and-transmission-to-bank-voles-show-the-m1-strain-signature
#3
Marcello Rossi, Daniela Saverioni, Michele Di Bari, Simone Baiardi, Afina Willemina Lemstra, Laura Pirisinu, Sabina Capellari, Annemieke Rozemuller, Romolo Nonno, Piero Parchi
Amyloid plaques formed by abnormal prion protein (PrPSc) aggregates occur with low frequency in Creutzfeldt-Jakob disease, but represent a pathological hallmark of three relatively rare disease histotypes, namely variant CJD, sporadic CJDMV2K (methionine/valine at PRNP codon 129, PrPSc type 2 and kuru-type amyloid plaques) and iatrogenic CJDMMiK (MM at codon 129, PrPSc of intermediate type and kuru plaques). According to recent studies, however, PrP-amyloid plaques involving the subcortical and deep nuclei white matter may also rarely occur in CJDMM1 (MM at codon 129 and PrPSc type 1), the most common CJD histotype...
November 23, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29167394/prion-seeding-activity-and-infectivity-in-skin-samples-from-patients-with-sporadic-creutzfeldt-jakob-disease
#4
Christina D Orrú, Jue Yuan, Brian S Appleby, Baiya Li, Yu Li, Dane Winner, Zerui Wang, Yi-An Zhan, Mark Rodgers, Jason Rarick, Robert E Wyza, Tripti Joshi, Gong-Xian Wang, Mark L Cohen, Shulin Zhang, Bradley R Groveman, Robert B Petersen, James W Ironside, Miguel E Quiñones-Mateu, Jiri G Safar, Qingzhong Kong, Byron Caughey, Wen-Quan Zou
Sporadic Creutzfeldt-Jakob disease (sCJD), the most common human prion disease, is transmissible through iatrogenic routes due to abundant infectious prions [misfolded forms of the prion protein (PrPSc)] in the central nervous system (CNS). Some epidemiological studies have associated sCJD risk with non-CNS surgeries. We explored the potential prion seeding activity and infectivity of skin from sCJD patients. Autopsy or biopsy skin samples from 38 patients [21 sCJD, 2 variant CJD (vCJD), and 15 non-CJD] were analyzed by Western blotting and real-time quaking-induced conversion (RT-QuIC) for PrPSc Skin samples from two patients were further examined for prion infectivity by bioassay using two lines of humanized transgenic mice...
November 22, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29162644/a-role-for-astroglia-in-prion-diseases
#5
Adriano Aguzzi, Yingjun Liu
In this issue of JEM, Krejciova et al. (https://doi.org/10.1084/jem.20161547) report that astrocytes derived from human iPSCs can replicate human CJD prions. These observations provide a new, potentially very valuable model for studying human prions in cellula and for identifying antiprion compounds that might serve as clinical candidates. Furthermore, they add to the evidence that astrocytes may not be just innocent bystanders in prion diseases.
December 4, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29142239/differential-overexpression-of-serpina3-in-human-prion-diseases
#6
S Vanni, F Moda, M Zattoni, E Bistaffa, E De Cecco, M Rossi, G Giaccone, F Tagliavini, S Haïk, J P Deslys, G Zanusso, J W Ironside, I Ferrer, G G Kovacs, G Legname
Prion diseases are fatal neurodegenerative disorders with sporadic, genetic or acquired etiologies. The molecular alterations leading to the onset and the spreading of these diseases are still unknown. In a previous work we identified a five-gene signature able to distinguish intracranially BSE-infected macaques from healthy ones, with SERPINA3 showing the most prominent dysregulation. We analyzed 128 suitable frontal cortex samples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal familial insomnia (FFI) n = 9, Gerstmann-Sträussler-Scheinker syndrome (GSS)) n = 4), patients with Alzheimer disease (AD, n = 14) and age-matched controls (n = 30)...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29142140/imaging-and-csf-analyses-effectively-distinguish-cjd-from-its-mimics
#7
Peter Rudge, Harpreet Hyare, Alison Green, John Collinge, Simon Mead
OBJECTIVE: To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. METHODS: Review the clinical findings and investigations in 214 patients enrolled into the UK National Prion Monitoring Cohort Study between October 2008 and November 2015 who had postmortem confirmed sCJD and compare these features with 50 patients referred over the same period who had an alternative final diagnosis (CJD mimics)...
November 15, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29142138/chitotriosidase-chit1-is-increased-in-microglia-and-macrophages-in-spinal-cord-of-amyotrophic-lateral-sclerosis-and-cerebrospinal-fluid-levels-correlate-with-disease-severity-and-progression
#8
Petra Steinacker, Federico Verde, Lubin Fang, Emily Feneberg, Patrick Oeckl, Sigrun Roeber, Sarah Anderl-Straub, Adrian Danek, Janine Diehl-Schmid, Klaus Fassbender, Klaus Fliessbach, Hans Foerstl, Armin Giese, Holger Jahn, Jan Kassubek, Johannes Kornhuber, G Bernhard Landwehrmeyer, Martin Lauer, Elmar Hans Pinkhardt, Johannes Prudlo, Angela Rosenbohm, Anja Schneider, Matthias L Schroeter, Hayrettin Tumani, Christine A F von Arnim, Jochen Weishaupt, Patrick Weydt, Albert C Ludolph, Deniz Yilmazer Hanke, Markus Otto
OBJECTIVES: Neurochemical markers of amyotrophic lateral sclerosis (ALS) that reflect underlying disease mechanisms might help in diagnosis, staging and prediction of outcome. We aimed at determining the origin and differential diagnostic and prognostic potential of the putative marker of microglial activation chitotriosidase (CHIT1). METHODS: Altogether 316 patients were included, comprising patients with sporadic ALS, ALS mimics (disease controls (DCo)), frontotemporal lobar degeneration (FTLD), Creutzfeldt-Jakob disease (CJD), Alzheimer's disease (AD), Parkinson's disease (PD) and healthy controls (Con)...
November 15, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29142106/soluble-a%C3%AE-aggregates-can-inhibit-prion-propagation
#9
Claire J Sarell, Emma Quarterman, Daniel C-M Yip, Cassandra Terry, Andrew J Nicoll, Jonathan D F Wadsworth, Mark A Farrow, Dominic M Walsh, John Collinge
Mammalian prions cause lethal neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD) and consist of multi-chain assemblies of misfolded cellular prion protein (PrP(C)). Ligands that bind to PrP(C) can inhibit prion propagation and neurotoxicity. Extensive prior work established that certain soluble assemblies of the Alzheimer's disease (AD)-associated amyloid β-protein (Aβ) can tightly bind to PrP(C), and that this interaction may be relevant to their toxicity in AD. Here, we investigated whether such soluble Aβ assemblies might, conversely, have an inhibitory effect on prion propagation...
November 2017: Open Biology
https://www.readbyqxmd.com/read/29141869/human-stem-cell-derived-astrocytes-replicate-human-prions-in-a-prnp-genotype-dependent-manner
#10
Zuzana Krejciova, James Alibhai, Chen Zhao, Robert Krencik, Nina M Rzechorzek, Erik M Ullian, Jean Manson, James W Ironside, Mark W Head, Siddharthan Chandran
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades. In this paper, we show that astrocytes derived from human induced pluripotent stem cells (iPSCs) support the replication of prions from brain samples of CJD patients. For experimental exposure of astrocytes to variant CJD (vCJD), the kinetics of prion replication occur in a prion protein codon 129 genotype-dependent manner, reflecting the genotype-dependent susceptibility to clinical vCJD found in patients...
November 15, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29099286/fdg-pet-in-creutzfeldt-jakob-disease-analysis-of-clinical-pet-correlation
#11
Dimitri Renard, Giovanni Castelnovo, Laurent Collombier, Eric Thouvenot, Vincent Boudousq
OBJECTIVE: To assess the relationship between clinical pattern and cerebral glucose metabolism on [18F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) in Creutzfeldt-Jakob disease (CJD). METHODS: Predefined clinical signs (ataxia, visual, pyramidal, myoclonus, limb apraxia, limb dystonia, sensory, parkinsonism, and corticobasal syndrome [CBS]) and FDG-PET data were assessed in consecutive CJD patients. Two types of statistical parametric mapping (SPM) analyses, using stringent level of significance p<0...
November 3, 2017: Prion
https://www.readbyqxmd.com/read/29097653/experimental-transfusion-of-variant-cjd-infected-blood-reveals-previously-uncharacterised-prion-disorder-in-mice-and-macaque
#12
Emmanuel E Comoy, Jacqueline Mikol, Nina Jaffré, Vincent Lebon, Etienne Levavasseur, Nathalie Streichenberger, Chryslain Sumian, Armand Perret-Liaudet, Marc Eloit, Olivier Andreoletti, Stéphane Haïk, Philippe Hantraye, Jean-Philippe Deslys
Exposure of human populations to bovine spongiform encephalopathy through contaminated food has resulted in <250 cases of variant Creutzfeldt-Jakob disease (vCJD). However, more than 99% of vCJD infections could have remained silent suggesting a long-term risk of secondary transmission particularly through blood. Here, we present experimental evidence that transfusion in mice and non-human primates of blood products from symptomatic and non-symptomatic infected donors induces not only vCJD, but also a different class of neurological impairments...
November 2, 2017: Nature Communications
https://www.readbyqxmd.com/read/29097599/brains-with-sporadic-creutzfeldt-jakob-disease-and-copathology-showed-a-prolonged-end-stage-of-disease
#13
Aitzol Miguelez-Rodriguez, Jorge Santos-Juanes, Ikerne Vicente-Etxenausia, Katty Perez de Heredia-Goñi, Beatriz Garcia, Luis M Quiros, Laura Lorente-Gea, Isabel Guerra-Merino, Jose J Aguirre, Ivan Fernandez-Vega
AIMS: To investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD). MATERIALS AND METHODS: Thirty consecutive cases of confirmed CJD during the period 2010-2015 at Basque Brain bank were retrospectively reviewed. Moreover, major neurodegenerative-associated proteins (phosphorylated Tau, 4R tau, 3R tau, alpha-synuclein, TDP43, amyloid beta) were tested...
November 2, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29043964/accuracy-of-diagnosis-criteria-in-patients-with-suspected-diagnosis-of-sporadic-creutzfeldt-jakob-disease-and-detection-of-14-3-3-protein-france-1992-to-2009
#14
Laurene Peckeu, Nicole Delasnerie-Lauprètre, Jean-Philippe Brandel, Dominique Salomon, Véronique Sazdovitch, Jean-Louis Laplanche, Charles Duyckaerts, Danielle Seilhean, Stéphane Haïk, Jean-Jacques Hauw
Diagnostic criteria of Creutzfeldt-Jakob disease (CJD), a rare and fatal transmissible nervous system disease with public health implications, are determined by clinical data, electroencephalogram (EEG), detection of 14-3-3 protein in cerebrospinal fluid (CSF), brain magnetic resonance imaging and prion protein gene examination. The specificity of protein 14-3-3 has been questioned. We reviewed data from 1,572 autopsied patients collected over an 18-year period (1992-2009) and assessed whether and how 14-3-3 detection impacted the diagnosis of sporadic CJD in France, and whether this led to the misdiagnosis of treatable disorders...
October 2017: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/28962210/three-sporadic-cases-of-creutzfeldt-jakob-disease-in-china-and-their-clinical-analysis
#15
Xingbang Wang, Na Li, Aifen Liu, Lin Ma, Peiyan Shan, Wenjing Jiang, Qun Zhang
The present study described the characteristics of three cases of Creutzfeldt-Jakob disease (CJD) in China and analyzed their clinical presentations. The clinical information of the three cases was collected and analyzed. Blood and cerebrospinal fluid (CSF) specimens of the patients were collected for detection of the prion protein (PRNP) gene and 14-3-3 protein levels. Dynamic changes of electroencephalograms (EEGs) and brain magnetic resonance images (MRIs) were also observed. All the three cases were sporadic CJD cases...
September 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28924012/inhibition-of-il-1%C3%AE-signaling-normalizes-nmda-dependent-neurotransmission-and-reduces-seizure-susceptibility-in-a-mouse-model-of-creutzfeldt-jakob-disease
#16
Ilaria Bertani, Valentina Iori, Massimo Trusel, Mattia Maroso, Claudia Foray, Susanna Mantovani, Raffaella Tonini, Annamaria Vezzani, Roberto Chiesa
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder caused by prion protein (PrP) misfolding, clinically recognized by cognitive and motor deficits, electroencephalographic abnormalities, and seizures. Its neurophysiological bases are not known. To assess the potential involvement of NMDA receptor (NMDAR) dysfunction, we analyzed NMDA-dependent synaptic plasticity in hippocampal slices from Tg(CJD) mice, which model a genetic form of CJD. Because PrP depletion may result in functional upregulation of NMDARs, we also analyzed PrP knock-out (KO) mice...
October 25, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28898175/an-unusual-presentation-of-creutzfeldt-jakob-disease-and-an-example-of-how-hickam-s-dictum-and-ockham-s-razor-can-both-be-right
#17
Eli S Neiman, Amtul Farheen, Nancy Gadallah, Thomas Steineke, Peter Parsells, Zev A Kizelnik, Michael Rosenberg
Patients can have more than one neurological problem, and sorting out acute from chronic disease can be challenging. The authors report a middle-aged patient who presented with ataxia, right hemiparesis, and abnormal nystagmus. Magnetic resonance imaging (MRI) showed a Chiari and an arachnoid cyst with brainstem compression that appeared to explain his abnormal examination. Shortly after admission, he was noted to have intermittent abnormal behavior and confusion. History from family revealed significant acute and chronic psychiatric problems that appeared to explain his abnormal mental status; this delayed the diagnosis of intermittent complex partial seizures...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28878311/high-diagnostic-value-of-second-generation-csf-rt-quic-across-the-wide-spectrum-of-cjd-prions
#18
Alessia Franceschini, Simone Baiardi, Andrew G Hughson, Neil McKenzie, Fabio Moda, Marcello Rossi, Sabina Capellari, Alison Green, Giorgio Giaccone, Byron Caughey, Piero Parchi
An early and accurate in vivo diagnosis of rapidly progressive dementia remains challenging, despite its critical importance for the outcome of treatable forms, and the formulation of prognosis. Real-Time Quaking-Induced Conversion (RT-QuIC) is an in vitro assay that, for the first time, specifically discriminates patients with prion disease. Here, using cerebrospinal fluid (CSF) samples from 239 patients with definite or probable prion disease and 100 patients with a definite alternative diagnosis, we compared the performance of the first (PQ-CSF) and second generation (IQ-CSF) RT-QuIC assays, and investigated the diagnostic value of IQ-CSF across the broad spectrum of human prions...
September 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28866189/comparison-of-thermal-properties-of-fish-collagen-and-bovine-collagen-in-the-temperature-range-298-670k
#19
Marlena Gauza-Włodarczyk, Leszek Kubisz, Sławomir Mielcarek, Dariusz Włodarczyk
The increased interest in fish collagen is a consequence of the risk of exposure to Creutzfeld-Jacob disease (CJD) and the bovine spongiform encephalopathy (BSE), whose occurrence is associated with prions carried by bovine collagen. Collagen is the main biopolymer in living organisms and the main component of the skin and bones. Until the discovery of the BSE, bovine collagen had been widely used. The BSE epidemic increased the interest in new sources of collagen such as fish skin collagen (FSC) and its properties...
November 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/28861800/molecular-subtyping-of-prp-res-in-human-sporadic-cjd-brain-tissue
#20
G M Klug, V Lewis, S J Collins
Across the spectrum of sporadic human prion diseases (also known as transmissible spongiform encephalopathies: TSE), there is considerable phenotypic diversity. Cumulative scientific evidence supports that prions, the infectious agents of prion diseases, are constituted predominantly, if not exclusively, by misfolded, typically protease-resistant, disease-associated isoforms of the prion protein (PrP(res)). Consequently, tissue deposition of PrP(res) is considered a hallmark of prion disease pathology, and this can be visualized by Western blotting after tissue homogenization and treatment with proteinases, particularly proteinase K (PK)...
2017: Methods in Molecular Biology
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