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Creutzfeldt-Jakob

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https://www.readbyqxmd.com/read/28421536/lysosomal-quality-control-in-prion-diseases
#1
REVIEW
Priyanka Majumder, Oishee Chakrabarti
Prion diseases are transmissible, familial or sporadic. The prion protein (PrP), a normal cell surface glycoprotein, is ubiquitously expressed throughout the body. While loss of function of PrP does not elicit apparent phenotypes, generation of misfolded forms of the protein or its aberrant metabolic isoforms has been implicated in a number of neurodegenerative disorders such as scrapie, kuru, Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Sträussler-Scheinker and bovine spongiform encephalopathy...
April 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28413741/serial-magnetic-resonance-imaging-in-creutzfeldt-jakob-disease-a-case-report-and-literature-review
#2
Ahmed H Qavi, Tasnim F Imran, Zachariah Hasan, Fariha Ilyas, Usman Ghani, Salman Assad, Shabih Hasan
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. CJD usually appears in later life and runs a rapid course. Typically, the onset of symptoms occurs about age 60 and about 90% of individuals die within one year. We report a case of 67-year-old male presented with progressive aphasia, confusion, dysphagia and inability to carry out activities of daily life (ADLs) over a period of three to four weeks. The patient had past medical history of chronic atrial fibrillation and hypertension...
March 14, 2017: Curēus
https://www.readbyqxmd.com/read/28402042/mm1-type-sporadic-creutzfeldt-jakob-disease-with-1-month-total-disease-duration-and-early-pathologic-indicators
#3
Yasushi Iwasaki, Hiroko Kato, Tetsuo Ando, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida
A 62-year-old man presented with abnormal behavior and cognitive impairment. Diffusion-weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent...
April 12, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28358272/evaluation-of-rapid-post-mortem-test-kits-for-bovine-spongiform-encephalopathy-bse-screening-in-japan-their-analytical-sensitivity-to-atypical-bse-prions
#4
Ken'ichi Hagiwara, Yoshifumi Iwamaru, Naoko Tabeta, Takashi Yokoyama, Minoru Tobiume
A classical type of bovine spongiform encephalopathy (C-BSE), recognized in 1987, had a large impact on public health due to its zoonotic link to variant Creutzfeldt-Jakob disease by the human consumption of dietary products contaminated with the C-BSE prion. Thus, a number of countries implemented BSE surveillance using rapid post-mortem test kits that were approved for detection of the C-BSE prion in the cattle brain. However, as atypical BSE (L- and H-BSE) cases emerged in subsequent years, the efficacy of the kits for the detection of atypical BSE prions became a matter of concern...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28349199/amyloid-%C3%AE-accumulation-in-the-cns-in-human-growth-hormone-recipients-in-the-uk
#5
Diane L Ritchie, Peter Adlard, Alexander H Peden, Suzanne Lowrie, Margaret Le Grice, Kimberley Burns, Rosemary J Jackson, Helen Yull, Michael J Keogh, Wei Wei, Patrick F Chinnery, Mark W Head, James W Ironside
Human-to-human transmission of Creutzfeldt-Jakob disease (CJD) has occurred through medical procedures resulting in iatrogenic CJD (iCJD). One of the commonest causes of iCJD was the use of human pituitary-derived growth hormone (hGH) to treat primary or secondary growth hormone deficiency. As part of a comprehensive tissue-based analysis of the largest cohort yet collected (35 cases) of UK hGH-iCJD cases, we describe the clinicopathological phenotype of hGH-iCJD in the UK. In the 33/35 hGH-iCJD cases with sufficient paraffin-embedded tissue for full pathological examination, we report the accumulation of the amyloid beta (Aβ) protein associated with Alzheimer's disease (AD) in the brains and cerebral blood vessels in 18/33 hGH-iCJD patients and for the first time in 5/12 hGH recipients who died from causes other than CJD...
March 27, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28335586/can-characteristic-mri-brain-substitute-invasive-lumbar-puncture-search-for-14-3-3-immunoassay-for-creutzfeldt-jakob-disease-suggestions-from-a-case-study
#6
Saira Saad, Mazhar Badshah, Muhammad Irshad
No abstract text is available yet for this article.
February 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28332471/cjd-surveillance-in-the-republic-of-ireland-from-2005-to-2015-a-suggested-algorithm-for-referrals
#7
Teresa Loftus, Daphne Chen, Seamus Looby, Albi Chalissery, Rachel Howley, Ciara Heaney, Josephine Heffernan, Michael Farrell, Francesca Brett
Definitive diagnosis of Creutzfeldt Jakob disease (CJD) remains tissue-based. Possible and probable CJD are useful clinical terms but may be used indiscriminately. The aim of this study was to assess the effectiveness of the Irish surveillance system and to ascertain how diagnostic accuracy in identifying clinically "definite" cases might be improved. We reviewed the clinical information, relevant investigations, and samples n = 100; (autopsy n = 87; biopsy n = 13) in 96 patients between January 1, 2005 and December 31, 2015...
March 23, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28321768/validation-of-%C3%AE-synuclein-as-a-csf-biomarker-for-sporadic-creutzfeldt-jakob-disease
#8
Franc Llorens, Niels Kruse, André Karch, Matthias Schmitz, Saima Zafar, Nadine Gotzmann, Ting Sun, Silja Köchy, Tobias Knipper, Maria Cramm, Ewa Golanska, Beata Sikorska, Pawel P Liberski, Raquel Sánchez-Valle, Andre Fischer, Brit Mollenhauer, Inga Zerr
The analysis of cerebrospinal fluid (CSF) biomarkers gains importance in the differential diagnosis of prion diseases. However, no single diagnostic tool or combination of them can unequivocally confirm prion disease diagnosis. Electrochemiluminescence (ECL)-based immunoassays have demonstrated to achieve high diagnostic accuracy in a variety of sample types due to their high sensitivity and dynamic range. Quantification of CSF α-synuclein (a-syn) by an in-house ECL-based ELISA assay has been recently reported as an excellent approach for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), the most prevalent form of human prion disease...
March 21, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28319876/redefining-periodic-patterns-on-electroencephalograms-of-patients-with-sporadic-creutzfeldt-jakob-disease
#9
Jung-Won Shin, Byeongsoo Yim, Seung Hun Oh, Nam Keun Kim, Sang Kun Lee, Ok-Joon Kim
OBJECTIVE: We aimed to redefine various periodic patterns (PPs) observed on electroencephalography (EEG) in patients with sporadic Creutzfeldt-Jakob disease (sCJD) using the American Clinical Neurophysiology Society's (ACNS) Criteria. METHODS: We analyzed EEG data of 23 patients with sCJD were admitted to two university hospitals between August 2005 and September 2015. RESULTS: We classified PPs on EEG data into three types: irregular periodic discharges (PDs) with superimposed rhythmic activities, appearing at a median of 8weeks after onset (w...
May 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28298604/prion-strain-characterization-of-a-novel-subtype-of-creutzfeldt-jakob-disease
#10
Roberta Galeno, Michele Angelo Di Bari, Romolo Nonno, Franco Cardone, Marco Sbriccoli, Silvia Graziano, Loredana Ingrosso, Michele Fiorini, Angelina Valanzano, Giulia Pasini, Anna Poleggi, Ramona Vinci, Anna Ladogana, Maria Puopolo, Salvatore Monaco, Umberto Agrimi, Gianluigi Zanusso, Maurizio Pocchiari
In 2007, we reported a patient with an atypical form of Creutzfeldt-Jakob disease (CJD), heterozygous for methionine-valine (MV) at codon 129, who showed a novel pathological prion protein (PrP(TSE)) conformation with an atypical glycoform (AG) profile and an intraneuronal PrP deposition. In the present study, we further characterized the conformational properties of this pathological prion protein (PrP(TSE) MV(AG)) showing that PrP(TSE) MV(AG) is composed by multiple conformers with biochemical properties distinct from PrP(TSE) type 1 and type 2 of MV sporadic CJD (sCJD)...
March 15, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28290426/rapidly-progressive-dementia-with-myoclonus-not-creutzfeldt-jakob-disease
#11
M Saini, N M Varghese, Khin Hnin Su Wai, N K Loh
No abstract text is available yet for this article.
March 2017: Neurology India
https://www.readbyqxmd.com/read/28289166/hans-jacob-and-brain-research-on-hamburg-euthanasia-victims-awaiting-further-brains
#12
Lawrence A Zeidman
Several neuropathologists conducted brain research on victims of so-called euthanasia programs carried out by the National Socialist (Nazi) regime in Germany from 1940 to 1945. Some published their results in German journals or books during and after the war. One of these neuropathologists was Hans Jacob of Hamburg, a former Nazi party member and the leader of the same laboratory previously run by Alfons Jakob (Creutzfeldt-Jakob disease). Though much has been published on the unethical actions of Jacob's fellow neuropathologist from Berlin, Julius Hallervorden, Jacob's actions were remarkably similar and have not been previously analyzed in the neuroscience literature...
March 14, 2017: Neurology
https://www.readbyqxmd.com/read/28278413/creutzfeldt-jakob-disease-surveillance-in-australia-update-to-december-2015
#13
Genevieve M Klug, Alison Boyd, Shannon Sarros, Christiane Stehmann, Marion Simpson, Catriona A McLean, Colin L Masters, Steven J Collins
Nation-wide surveillance of human transmissible spongiform encephalopathies (also known as prion diseases), the most common being Creutzfeldt-Jakob disease, is performed by the Australian National Creutzfeldt-Jakob Disease Registry, based at the University of Melbourne. Prospective surveillance has been undertaken since 1993 and over this dynamic period in transmissible spongiform encephalopathy research and understanding, the unit has evolved and adapted to changes in surveillance practices and requirements concomitant with the delineation of new disease subtypes, improvements in diagnostic capabilities and the overall heightened awareness of prion diseases in the health care setting...
September 30, 2016: Communicable Diseases Intelligence Quarterly Report
https://www.readbyqxmd.com/read/28278113/clinical-and-laboratory-features-of-14-young-chinese-probable-scjd-patients
#14
Qi Shi, Kang Xiao, Cao Chen, Wei Zhou, Chen Gao, Jing Wang, Bao-Yun Zhang, Yuan Wang, Xiao-Ping Dong
Sporadic Creutzfeldt-Jakob disease (sCJD) occurs frequently in the relatively older population, mainly in the groups of 60-69 and 70-79 year-old. Since 2006 when China performed national CJD surveillance, 14 young probable sCJD patients below 40 year-old were identified, counting for 1.93% of all probable sCJD cases. The clinical features of young probable sCJD cases, including the onset feature, the presence of sCJD-associated signs and the clinical duration, are indistinguishable from those of older patients...
March 4, 2017: Prion
https://www.readbyqxmd.com/read/28261810/geographic-exposure-risk-of-variant-creutzfeldt-jakob-disease-in-us-blood-donors-a-risk-ranking-model-to-evaluate-alternative-donor-deferral-policies
#15
Hong Yang, Yin Huang, Luisa Gregori, David M Asher, Travis Bui, Richard A Forshee, Steven A Anderson
BACKGROUND: Variant Creutzfeldt-Jakob disease (vCJD) has been transmitted by blood transfusion (TTvCJD). The US Food and Drug Administration (FDA) recommends deferring blood donors who resided in or traveled to 30 European countries where they may have been exposed to bovine spongiform encephalopathy (BSE) through beef consumption. Those recommendations warrant re-evaluation, because new cases of BSE and vCJD have markedly abated. STUDY DESIGN AND METHODS: The FDA developed a risk-ranking model to calculate the geographic vCJD risk using country-specific case rates and person-years of exposure of US blood donors...
March 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28258683/-patient-with-creutzfeld-jakob-disease-a-case-report
#16
Żanna Pastuszak, Kazimierz Tomczykiewicz, Adam Stępień, Renata Piusińska-Macoch, Joanna Klimczuk, Agnieszka Rolewska, Dariusz Galbarczyk
Creutzfeldt-Jakob disease (CJD) is a rare syndrome of central nervous system caused by infectious protein called prion. There are four types of CJD: sporadic (sCJD), familial (fCJD), jatrogenic (jCJD) and variant (vCJD). The most frequent symptoms are rapidly progressing dementia, mioclonias, akinetic mutism and signs of cerebellum dysfunction. In sCJD, MRI often shows high signal intensity in the putamen and caudate nucleus on T2-weighted images while in vCJD pulvinar sign is often observed. 70% patients with CJD often has characteristic generalized periodic sharp wave pattern in electroencephalography...
February 20, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28256219/mapping-the-broad-structural-and-mechanical-properties-of-amyloid-fibrils
#17
Guillaume Lamour, Roy Nassar, Patrick H W Chan, Gunes Bozkurt, Jixi Li, Jennifer M Bui, Calvin K Yip, Thibault Mayor, Hongbin Li, Hao Wu, Jörg A Gsponer
Amyloids are fibrillar nanostructures of proteins that are assembled in several physiological processes in human cells (e.g., hormone storage) but also during the course of infectious (prion) and noninfectious (nonprion) diseases such as Creutzfeldt-Jakob and Alzheimer's diseases, respectively. How the amyloid state, a state accessible to all proteins and peptides, can be exploited for functional purposes but also have detrimental effects remains to be determined. Here, we measure the nanomechanical properties of different amyloids and link them to features found in their structure models...
February 28, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28248914/creutzfeldt-jakob-disease-presenting-as-stroke-a-case-report-and-systematic-literature-review
#18
REVIEW
Divya K Sharma, Mike Boggild, Annemarie W van Heuven, Richard P White
BACKGROUND: Creutzfeldt-Jacob disease (CJD) is a human prion disease generally characterized by subacute changes in behavior and intellectual function, often followed by ataxia, vision changes, and myoclonus. Ten percent of cases may present atypically, both symptomatically and in respect to initial investigations. METHODS: We report a case of CJD mimicking acute stroke and review all similar cases in the magnetic resonance imaging era reported in English, identified through a PubMed and SCOPUS search...
March 2017: Neurologist
https://www.readbyqxmd.com/read/28244340/lack-of-germline-mutation-at-codon-211-of-the-prion-protein-gene-prnp-in-korean-native-cattle-short-communication
#19
Yong-Chan Kim, Byung-Hoon Jeong
Bovine prion diseases are composed of two types of bovine spongiform encephalopathy (BSE), classical BSE and atypical BSE. Recent studies have identified one case of atypical BSE with an E211K mutation. E211K is homologous to the human E200K mutation, which is related to familial Creutzfeldt-Jakob disease (CJD), one of the familial forms of human prion diseases. To date, familial forms of prion diseases have not been reported in non-human animals. Because the familial forms of human prion diseases account for more than 10% of all human prion disease cases, the detection of the E211K mutation in healthy cattle is very important for verifying the role of this mutation as a familial form of BSE...
March 2017: Acta Veterinaria Hungarica
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
#20
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...
May 2017: Clinical Nuclear Medicine
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