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Creutzfeldt-jakob disease

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https://www.readbyqxmd.com/read/29652245/prion-disease-in-dromedary-camels-algeria
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/29555421/neurodegenerative-diseases-infectious-pathologies-and-liquid-crystals-hypothesis-of-a-common-information-vector-involving-a-multidisciplinary-approach
#5
C Binot, C-H Chouard
The existence of an information vector common to very different pathologies is the hypothesis of one of us, the argumentation and discussion of which we present here. It is a mesomorphic state of material called liquid crystal. The liquid-ordered (Lo) phase, made up of membrane rafts mediated by cholesterol, lies at the center of our concept. This mesophase is either preexistent and then modified by the pathogenic process, or initiated by the latter. The most notable disorders involved are Alzheimer's, Parkinson's, Charcot and Creutzfeldt-Jakob diseases, flu-like illnesses and acquired immunodeficiency syndrome (AIDS), although this list may well be extended to include other anisotropic, birefringent amyloid proteinopathies, which have properties compatible with those of liquid crystals...
March 16, 2018: Revue Neurologique
https://www.readbyqxmd.com/read/29525731/atypical-presentation-of-probable-creutzfeldt-jakob-disease-associated-with-anti-zic4-antibody-literature-review-of-neuronal-antibodies-in-creutzfeldt-jakob-disease
#6
Richard Salazar
INTRODUCTION: Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. CASE REPORT: A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia...
May 2018: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29518068/update-dura-mater-graft-associated-creutzfeldt-jakob-disease-japan-1975-2017
#7
Ryusuke Ae, Tsuyoshi Hamaguchi, Yosikazu Nakamura, Masahito Yamada, Tadashi Tsukamoto, Hidehiro Mizusawa, Ermias D Belay, Lawrence B Schonberger
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in medical/surgical settings. Cadaveric dura mater graft-associated CJD (dCJD) accounts for a common form of iatrogenic CJD...
March 9, 2018: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/29515622/high-incidence-of-sporadic-creutzfeldt-jakob-disease-in-slovenia-in-2015-a-case-series
#8
Tomaž Rus, Bogdan Lorber, Maja Trošt, Srečko Dobrecovič, Nuška Čakš Jager, Mara Popović, Milica G Kramberger
Background: Creutzfeldt-Jakob disease (CJD) is a rare fatal neurodegenerative disorder presenting with rapid cognitive decline and additional signs. The clinical characteristics of an increasing number of sporadic CJD (sCJD) patients admitted to the Ljubljana University Medical Centre are presented as well as the incidence of sCJD in Slovenia in 2015 compared to previous years. Methods: We investigated patients presenting with rapidly progressive dementia and at least one additional sign...
January 2018: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/29511772/-neurodegenerative-diseases
#9
Wolfgang Reith
Neurodegenerative diseases are sporadic and rare hereditary disorders of the central nervous system, which cause a slowly progressive loss of function of specific neuron populations and their connections. Severe impairments and care dependency can be the sequelae. Neurodegenerative disorders are diseases of older people; therefore, the demographic shift leads to an increase in the number of affected patients. Radiologists will also become more involved. For this reason important neurodegenerative diseases are presented in this article...
March 2018: Der Radiologe
https://www.readbyqxmd.com/read/29505163/generation-of-novel-neuroinvasive-prions-following-intravenous-challenge
#10
Patricia Aguilar-Calvo, Cyrus Bett, Alejandro M Sevillano, Timothy D Kurt, Jessica Lawrence, Katrin Soldau, Per Hammarström, K Peter R Nilsson, Christina J Sigurdson
Prion aggregates typically spread into the central nervous system (CNS), likely via peripheral nerves. Yet prion conformers differ in their capacity to penetrate the CNS; certain fibrillar prions replicate persistently in lymphoid tissues with no CNS entry, leading to chronic silent carriers. Subclinical carriers of variant Creutzfeldt-Jakob (vCJD) prions in the United Kingdom have been estimated at 1:2000, and vCJD prions have been transmitted through blood transfusion, however the circulating prion conformers that neuroinvade remain unclear...
March 5, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29487167/neurofilament-light-chain-and-tau-concentrations-are-markedly-increased-in-the-serum-of-patients-with-sporadic-creutzfeldt-jakob-disease-and-tau-correlates-with-rate-of-disease-progression
#11
Andrew Geoffrey Bourne Thompson, Connie Luk, Amanda J Heslegrave, Henrik Zetterberg, Simon H Mead, John Collinge, Graham S Jackson
OBJECTIVES: A blood-based biomarker of neuronal damage in sporadic Creutzfeldt-Jakob disease (sCJD) will be extremely valuable for both clinical practice and research aiming to develop effective therapies. METHODS: We used an ultrasensitive immunoassay to measure two candidate biomarkers, tau and neurofilament light (NfL), in serum from patients with sCJD and healthy controls. We tested longitudinal sample sets from six patients to investigate changes over time, and examined correlations with rate of disease progression and associations with known phenotype modifiers...
February 27, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29486073/creutzfeldt-jakob-disease-considerations-for-perioperative-care-2-2-www-aornjournal-org-content-cme
#12
Deborah Anderson
No abstract text is available yet for this article.
March 2018: AORN Journal
https://www.readbyqxmd.com/read/29478593/prion-disease
#13
Leonel T Takada, Mee-Ohk Kim, Stacy Metcalf, Ignacio Illán Gala, Michael D Geschwind
Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion protein gene (PRNP). Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs historically have been classified into three forms: familial Jakob-Creutzfeldt disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29458529/both-murine-host-and-inoculum-modulate-expression-of-experimental-variant-creutzfeldt-jakob-disease
#14
Cyrus Bett, Pedro Piccardo, Juraj Cervenak, Juan-Maria Torres, David M Asher, Luisa Gregori
Transmissible spongiform encephalopathies (TSEs) are infections that are experimentally transmissible to laboratory animals. TSE agents (prions) can be serially passaged in the same animal species. The susceptibility of mice to infection with specific TSE agents can be unpredictable and must be established empirically. We challenged wild-type C57BL/6 and RIIIS/J mice and transgenic mice overexpressing bovine prion protein (TgBo110) with a human brain infected with variant Creutzfeldt-Jakob disease (vCJD) agent and pooled brains of macaques experimentally infected with human vCJD agent (first-passage macaque vCJD)...
February 1, 2018: Journal of General Virology
https://www.readbyqxmd.com/read/29436943/heterozygous-genotype-at-codon-129-correlates-with-prolonged-disease-course-in-heidenhain-variant-sporadic-cjd-case-report
#15
Ryan A Townley, Elliot T Dawson, Daniel A Drubach
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapid and fatal neurodegenerative disease defined by misfolded prion proteins accumulating in the brain. A minority of cases initially present with posterior cortical atrophy (PCA) phenotype, also known as Heidenhain variant or visual variant CJD. This case provides further evidence of sCJD presenting as PCA. The case also provides evidence for early DWI changes and cortical atrophy over 30 months before neurologic decline and subsequent death. The prolonged disease course correlates with prion protein codon 129 heterozygosity and coexistence of multiple prion strains...
February 2018: Neurocase
https://www.readbyqxmd.com/read/29434023/cerebellar-ataxia-as-a-first-manifestation-of-creutzfeldt-jakob-disease-in-two-cousins
#16
Marta Waliszewska-Prosół, Krystian Obara, Paweł Szewczyk, Małgorzata Śniatowska, Sławomir Budrewicz
No abstract text is available yet for this article.
February 6, 2018: Postgraduate Medical Journal
https://www.readbyqxmd.com/read/29432500/large-animals-as-potential-models-of-human-mental-and-behavioral-disorders
#17
REVIEW
Michał Danek, Janusz Danek, Aleksander Araszkiewicz
Many animal models in different species have been developed for mental and behavioral disorders. This review presents large animals (dog, ovine, swine, horse) as potential models of this disorders. The article was based on the researches that were published in the peer-reviewed journals. Aliterature research was carried out using the PubMed database. The above issues were discussed in the several problem groups in accordance with the WHO International Statistical Classification of Diseases and Related Health Problems 10thRevision (ICD-10), in particular regarding: organic, including symptomatic, disorders; mental disorders (Alzheimer's disease and Huntington's disease, pernicious anemia and hepatic encephalopathy, epilepsy, Parkinson's disease, Creutzfeldt-Jakob disease); behavioral disorders due to psychoactive substance use (alcoholic intoxication, abuse of morphine); schizophrenia and other schizotypal disorders (puerperal psychosis); mood (affective) disorders (depressive episode); neurotic, stress-related and somatoform disorders (posttraumatic stress disorder, obsessive-compulsive disorder); behavioral syndromes associated with physiological disturbances and physical factors (anxiety disorders, anorexia nervosa, narcolepsy); mental retardation (Cohen syndrome, Down syndrome, Hunter syndrome); behavioral and emotional disorders (attention deficit hyperactivity disorder)...
December 30, 2017: Psychiatria Polska
https://www.readbyqxmd.com/read/29429784/rapid-progressive-visual-decline-and-visual-field-defects-in-two-patients-with-the-heidenhain-variant-of-creutzfeld-jakob-disease
#18
Janine Lenk, Kay Engellandt, Naim Terai, Antonia Bottesi, Egbert Matthé
BACKGROUND: Heidenhain variant of Creutzfeldt-Jakob (HvCJD) is a rare disease, patients presenting with loss of visual acuity and a decline in visual fields. CASE PRESENTATION: Two patients with rapid loss of visual acuity and declining visual fields presented with homonymic hemianopsia over several weeks. Cranial MRI showed neither stroke nor other morphological changes explaining the severe visual field defects. Neurological examination revealed no pathologies...
April 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29422367/csf-tau-supplements-14-3-3-protein-detection-for-sporadic-creutzfeldt-jakob-disease-diagnosis-while-transitioning-to-next-generation-diagnostics
#19
Qiao-Xin Li, Shiji Varghese, Shannon Sarros, Christiane Stehmann, James D Doecke, Christopher J Fowler, Colin L Masters, Steven J Collins
The pre-mortem clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is supported by biomarkers, especially cerebrospinal fluid (CSF) 14-3-3 and total tau (Tau) protein levels. These CSF biomarkers have proven the most useful prior to transitioning to powerful next generation diagnostics employing protein amplification techniques such as the real time quaking-induced conversion (RT-QuIC) assay. To enhance national diagnostic capacity while transitioning to RT-QuIC assays an optimized CSF Tau cutoff was determined and shown to usefully supplement 14-3-3 protein detection...
February 5, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29414853/the-structure-of-prpsc-prions
#20
REVIEW
Holger Wille, Jesús R Requena
PrPSc (scrapie isoform of the prion protein) prions are the infectious agent behind diseases such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (deer, elk, moose, and reindeer), as well as goat and sheep scrapie. PrPSc is an alternatively folded variant of the cellular prion protein, PrPC, which is a regular, GPI-anchored protein that is present on the cell surface of neurons and other cell types. While the structure of PrPC is well studied, the structure of PrPSc resisted high-resolution determination due to its general insolubility and propensity to aggregate...
February 7, 2018: Pathogens
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