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Creutzfeldt-Jakob

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https://www.readbyqxmd.com/read/29761923/accuracy-of-a-history-of-blood-donation-from-surrogate-witnesses-data-from-the-uk-tmer-study
#1
J M Mackenzie, M Turner, K Morris, S Field, A M Molesworth, S Pal, R G Will, C A Llewelyn, P E Hewitt
Look-back studies of blood transfusion in Creutzfeldt-Jakob disease commonly rely on reported history from surrogate witnesses. Data from the UK Transfusion Medicine Epidemiology Review have been analysed to determine the accuracy of the blood donation history provided by the relatives of cases. Our results show that only a small percentage of cases were found to be registered as donors on UK Blood Service (UKBS) databases when there was no family report of blood donation. In contrast, a history of reported donation was less accurate...
May 15, 2018: Vox Sanguinis
https://www.readbyqxmd.com/read/29755395/early-onset-creutzfeldt-jakob-disease-mimicking-immune-mediated-encephalitis
#2
Wietse A Wiels, Stephanie Du Four, Laura Seynaeve, Anja Flamez, Thomas Tousseyn, Dietmar Thal, Miguel D'Haeseleer
Objectives: The objective of this study is to explore the clinical, radiological, and pathological manifestations of a rare subtype of prion disease and their implication for differential diagnosis in case of an early onset neuropsychiatric deterioration. Methods: We discuss a patients' clinical history, as well as the string of investigations and symptomatological evolution that finally led to a pathological diagnosis. Results: Our patient had the extremely rare VV1 type sporadic Creutzfeldt-Jakob disease (sCJD)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29734943/low-mood-visual-hallucinations-and-falls-heralding-the-onset-of-rapidly-progressive-probable-sporadic-creutzfeldt-jakob-disease-in-a-73-year-old-a-case-report
#3
Daniel Martin Klotz, Rose Sarah Penfold
BACKGROUND: Creutzfeldt-Jakob disease is a rare and rapidly fatal neurodegenerative disease. Since clinicians may see only very few cases during their professional career, it is important to be familiar with the clinical presentation and progression, to perform appropriate investigations, and allow for quick diagnosis. CASE PRESENTATION: A 73-year-old British Caucasian woman presented with acute confusion of 2 weeks' duration on a background of low mood following a recent bereavement...
May 8, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29734684/comparing-the-folds-of-prions-and-other-pathogenic-amyloids
#4
REVIEW
José Miguel Flores-Fernández, Vineet Rathod, Holger Wille
Pathogenic amyloids are the main feature of several neurodegenerative disorders, such as Creutzfeldt⁻Jakob disease, Alzheimer’s disease, and Parkinson’s disease. High resolution structures of tau paired helical filaments (PHFs), amyloid-β(1-42) (Aβ(1-42)) fibrils, and α-synuclein fibrils were recently reported using cryo-electron microscopy. A high-resolution structure for the infectious prion protein, PrPSc , is not yet available due to its insolubility and its propensity to aggregate, but cryo-electron microscopy, X-ray fiber diffraction, and other approaches have defined the overall architecture of PrPSc as a 4-rung β-solenoid...
May 4, 2018: Pathogens
https://www.readbyqxmd.com/read/29710713/rapidly-progressive-alzheimer-s-disease-contributions-to-clinical-pathological-definition-and-diagnosis
#5
Samir Abu-Rumeileh, Sabina Capellari, Piero Parchi
Rapidly progressive Alzheimer's disease (rpAD) has recently been recognized as a clinical disease subtype characterized by rapidly progressive cognitive decline and/or short disease duration, and the possible occurrence of early focal neurological signs. Consistently, rpAD represents a relatively frequent alternative diagnosis among cases referred as possible or probable Creutzfeldt-Jakob disease (CJD) to surveillance centers for prion disease worldwide. Indeed, the early clinical differential diagnosis between the two disorders can be challenging given the partial overlap in clinical features and cerebrospinal fluid (CSF) levels of the protein surrogate markers 14-3-3 and total-tau...
April 25, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29704165/genetic-creutzfeldt-jakob-disease-with-an-8-year-disease-course
#6
Dimitri Renard, Jessica Orgeval, Veronique Sazdovitch, Danielle Seilhean, Eric Thouvenot
No abstract text is available yet for this article.
April 27, 2018: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29703169/case-series-of-creutzfeldt-jakob-disease-in-a-third-level-hospital-in-quito
#7
Germaine Eleanor Torres Herrán, Andrés Damián Ortega Herrera, Braulio Martinez Burbano, Marcos Serrano-Dueñas, María Angélica Ortiz Yepez, Raúl Alberto Barrera Madera, Luis Alfredo Masabanda Campaña, Guillermo David Baño Jiménez, Denny Maritza Santos Saltos, Edgar Patricio Correa Díaz
BACKGROUND: Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans. The prevalence of this disease in the United States is 0.5 to 1 per million inhabitants. So far in Ecuador, we do not know what the prevalence or incidence is, and only one case report has been written. CASE PRESENTATION: We present a case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito. The average age of symptom onset in our patients was 58...
April 27, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29699515/creutzfeldt-jakob-disease-with-alzheimer-pathology-presenting-with-status-epilepticus-following-repeated-partial-seizures-a-case-report-and-literature-review
#8
Keita Miyake, Takashi Hara, Etsuko Oshima, Kiyohiro Kawada, Hideki Ishizu, Yuko Yamauchi, Katsuya Satoh, Tetsuyuki Kitamoto, Shintaro Takenoshita, Seishi Terada, Norihito Yamada
BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease. Common first symptoms are dementia, cerebellar ataxia, visual disturbance, and psychiatric symptoms. Seizure as the first symptom of CJD is a very rare finding. CASE PRESENTATION: We experienced an elderly woman who presented initially with status epilepticus following repeated partial seizures in the course of Alzheimer disease (AD) dementia. Anti-convulsive therapy had no effect...
April 25, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29695523/thermomixer-aided-ep-quic-permits-faster-scjd-identification-than-rt-quic
#9
Keding Cheng, Robert Vendramelli, Angela Sloan, Sharon Simon, Debra Godal
We recently reported a method called end-point quaking induced conversion (EP-QuIC) (1) which is modified from real-time quaking-induced conversion (RT-QuIC) (2-4), to aid in ante-mortem diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD).….
April 25, 2018: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/29693615/observance-of-sterilization-protocol-guideline-procedures-of-critical-instruments-for-preventing-iatrogenic-transmission-of-creutzfeldt-jakob-disease-in-dental-practice-in-france-2017
#10
Denis Bourgeois, Claude Dussart, Ina Saliasi, Laurent Laforest, Paul Tramini, Florence Carrouel
Effective sterilization of reusable instruments contaminated by Creutzfeldt⁻Jakob disease in dental care is a crucial issue for public health. The present cross-sectional study investigated how the recommended procedures for sterilization were implemented by French dental practices in real-world settings. A sample of dental practices was selected in the French Rhône-Alpes region. Data were collected by a self-questionnaire in 2016. Sterilization procedures ( n = 33) were classified into 4 groups: (1) Pre-sterilization cleaning of reusable instruments; (2) Biological verification of sterilization cycles—Monitoring steam sterilization procedures; (3) Autoclave performance and practitioner knowledge of autoclave use; (4) Monitoring and documentation of sterilization procedures—Tracking and tracing the instrumentation...
April 25, 2018: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29685192/the-role-of-alu-derived-rnas-in-alzheimer-s-and-other-neurodegenerative-conditions
#11
Oksana Polesskaya, Evgeniya Kananykhina, Astrid M Roy-Engel, Olga Nazarenko, Irina Kulemzina, Ancha Baranova, Yegor Vassetsky, Max Myakishev-Rempel
Non-coding RNAs have emerged as essential contributors to neuroinflammation. The Alu element is the most abundant potential source of non-coding RNA in the human genome represented by over 1.1 million copies totaling ∼10% of the genome's mass. Accumulation of "Alu RNA" was observed in the brains of individuals with dementia and Creutzfeldt-Jakob disease - a degenerative brain disorder. "Alu RNAs" activate inflammatory pathways and apoptosis in the non-neural cells. In particular, the "Alu RNA" cytotoxicity is suggested as a mechanism in retinal pigment epithelium (RPE), a compartment damaged in the process of age-related macular degeneration...
June 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/29682384/corrigendum-to-a-case-of-sporadic-creutzfeldt-jakob-disease-presenting-as-conversion-disorder
#12
Nikhil Yegya-Raman, Rehan Aziz, Daniel Schneider, Anthony Tobia, Megan Leitch, Onyi Nwobi
[This corrects the article DOI: 10.1155/2017/2735329.].
2018: Case Reports in Psychiatry
https://www.readbyqxmd.com/read/29676187/decrease-of-ryr2-in-the-prion-infected-cell-line-and-in-the-brains-of-the-scrapie-infected-mice-models-and-the-patients-of-human-prion-diseases
#13
Qi Shi, Jian-Le Li, Yue Ma, Li-Ping Gao, Kang Xiao, Jing Wang, Wei Zhou, Cao Chen, Yan-Jun Guo, Xiao-Ping Dong
The levels of ryanodine receptors (RyRs) are usually increased in the brains of human Alzheimer disease (AD) and AD animal models. To evaluate the underlying alteration of brain RyRs in prion disease, scrapie infected cell line SMB-S15 and its infected mice were tested. RyR2 specific Western blots revealed markedly decreased RyR2 levels both in the cells and in the brains of infected mice. Assays of the brain samples of other scrapie (agents 139A and ME7) infected mice collected at different time-points during incubation period showed time-dependent decreases of RyR2...
April 20, 2018: Prion
https://www.readbyqxmd.com/read/29666711/creutzfeldt-jakob-disease-presenting-as-expressive-aphasia-and-nonconvulsive-status-epilepticus
#14
Hafiz B Mahboob, Kazi H Kaokaf, Jeremy M Gonda
Creutzfeldt-Jakob disease (CJD), the most common form of human prion diseases, is a fatal condition with a mortality rate reaching 85% within one year of clinical presentation. CJD is characterized by rapidly progressive neurological deterioration in combination with typical electroencephalography (EEG) and magnetic resonance imaging (MRI) findings and positive cerebrospinal spinal fluid (CSF) analysis for 14-3-3 proteins. Unfortunately, CJD can have atypical clinical and radiological presentation in approximately 10% of cases, thus making the diagnosis often challenging...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29665324/rapid-amplification-of-prions-from-variant-creutzfeldt-jakob-disease-cerebrospinal-fluid
#15
Marcelo A Barria, Andrew Lee, Alison Je Green, Richard Knight, Mark W Head
Human prion diseases constitute a group of infectious and invariably fatal neurodegenerative disorders associated with misfolding of the prion protein. Variant Creutzfeldt-Jakob disease (vCJD) is a zoonotic prion disease linked to oral exposure to the infectious agent that causes bovine spongiform encephalopathy (BSE) in cattle. The most recent case of definite vCJD was heterozygous (MV) at polymorphic codon 129 of the prion protein gene PRNP while all of the previous 177 definite or probable vCJD cases who underwent genetic analysis were methionine homozygous (MM)...
April 2018: Journal of Pathology. Clinical Research
https://www.readbyqxmd.com/read/29661908/teaching-neuroimages-dwi-and-eeg-findings-in-creutzfeldt-jakob-disease
#16
Aravind Ganesh, Lisa C Hoyte, Yahya Agha-Khani, Michael M C Yeung
No abstract text is available yet for this article.
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29652245/prion-disease-in-dromedary-camels-algeria
#17
Baaissa Babelhadj, Michele Angelo Di Bari, Laura Pirisinu, Barbara Chiappini, Semir Bechir Suheil Gaouar, Geraldina Riccardi, Stefano Marcon, Umberto Agrimi, Romolo Nonno, Gabriele Vaccari
Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE). After the BSE epidemic, and the associated human infections, began in 1996 in the United Kingdom, general concerns have been raised about animal prions. We detected a prion disease in dromedary camels (Camelus dromedarius) in Algeria. Symptoms suggesting prion disease occurred in 3.1% of dromedaries brought for slaughter to Ouargla abattoir in 2015-2016...
June 17, 2018: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/29621843/diagnosis-of-sporadic-creutzfeldt-jakob-disease
#18
Brad Guo, Tam Ho, Roberta Potamianos
No abstract text is available yet for this article.
March 2018: Australian journal of general practice
https://www.readbyqxmd.com/read/29617881/fdg-pet-diagnoses-of-sporadic-creutzfeldt-jakob-disease-radiology-pathology-correlation
#19
J Duignan, G M Healy, N M Hughes, C E Redmond, C McGuigan, A Beausang, R P Killeen
No abstract text is available yet for this article.
March 28, 2018: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/29580258/detection-of-creutzfeldt-jakob-disease-prions-in-skin-implications-for-healthcare
#20
Akin Nihat, Simon Mead
Evidence has recently been reported of prion seeding activity in skin tissue from patients with sporadic Creutzfeldt-Jakob disease (sCJD). This is relevant information for infection control measures during surgery. The work uses very sensitive prion assays now available for medical research, and may soon be adapted to related neurodegenerative disorders.
March 26, 2018: Genome Medicine
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