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https://www.readbyqxmd.com/read/28987186/prion-diseases
#1
James W Ironside, Diane L Ritchie, Mark W Head
The human prion diseases comprise Creutzfeldt-Jakob disease, variably protease-sensitive prionopathy, Gerstmann-Sträussler-Scheinker disease, fatal familial insomnia, and kuru. Each is a uniformly fatal rare neurodegenerative disease in which conformational changes in the prion protein are thought to be the central pathophysiologic event. The majority of cases of human prion diseases occur worldwide in the form of sporadic Creutzfeldt-Jakob disease and a minority of around 10-15% are associated with mutations of the prion protein gene, termed PRNP, in the forms of genetic Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28838665/infectious-and-sporadic-prion-diseases
#2
Richard Knight
Prion diseases are progressive fatal encephalopathies characterized by a neurodegenerative pathology, the tissue deposition of abnormally folded prion protein and, in general, potential transmissibility. Creutzfeldt-Jakob disease (CJD) is the commonest human prion disease and occurs in three principal forms: sporadic (idiopathic), acquired (infectious), and inherited (genetic). This chapter concerns the sporadic and acquired forms. Sporadic CJD occurs worldwide and affects mainly the middle aged and elderly...
2017: Progress in Molecular Biology and Translational Science
https://www.readbyqxmd.com/read/28803271/brain-biopsies-requiring-creutzfeldt-jakob-disease-precautions-in-the-republic-of-ireland-2005-2016
#3
F M Brett, S Looby, A Chalissery, D Chen, C Heaney, J Heffernan, F Cunningham, R Howley, T Loftus, H Kearney, M A Farrell
AIMS: Creutzfeldt-Jakob disease (CJD) risk precautions are required when performing brain biopsies on patients with a dementing illness and in 'risk' groups. The impact on a diagnostic neuropathology service is considerable. We sought to determine if better case selection might reduce the necessity for application of CJD risk precautions. METHODS: We reviewed the clinical information, contributory investigations and final neuropathologic diagnosis in a cohort of patients (n = 21), referred to the National CJD Surveillance Centre between January 1, 2005, and December 31, 2016...
August 12, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28791720/infectivity-in-bone-marrow-from-sporadic-cjd-patients
#4
Alvina Huor, Jean Yves Douet, Caroline Lacroux, Séverine Lugan, Cécile Tillier, Naima Aron, Hervé Cassard, Mark Arnold, Juan Maria Torres, James W Ironside, Olivier Andréoletti
Prion infectivity was recently identified in the blood of both sporadic and variant Creutzfeldt-Jakob disease (CJD) patients. In variant CJD (vCJD), the widespread distribution of prions in peripheral tissues of both asymptomatic and symptomatic patients is likely to explain the occurrence of the observed prionaemia. However, in sporadic CJD (sCJD), prion infectivity is described to be located principally in the central nervous system. In this study, we investigated the presence of prion infectivity in bone marrow collected after death in patients affected with different sCJD agents...
August 9, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28518033/distribution-and-quantitative-estimates-of-variant-creutzfeldt-jakob-disease-prions-in-tissues-of-clinical-and-asymptomatic-patients
#5
Jean Y Douet, Caroline Lacroux, Naima Aron, Mark W Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W Ironside, Olivier Andréoletti
In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD. PMCA identified major levels of vCJD prions in a range of tissues, including liver, salivary gland, kidney, lung, and bone marrow...
June 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28444687/creutzfeldt-jakob-disease-lookback-study-21-years-of-surveillance-for-transfusion-transmission-risk
#6
Lauren A Crowder, Lawrence B Schonberger, Roger Y Dodd, Whitney R Steele
BACKGROUND: Transfusion transmission of human prion diseases has been observed for variant Creutzfeldt-Jakob disease (vCJD), but not for the classic forms of prion disease (CJD: sporadic, genetic, and iatrogenic). Although the presence of prions or misfolded prion proteins in blood has been documented in some patients with the most common form of CJD, sporadic CJD, no transfusion-transmitted cases of CJD have been recognized. Since 1995, the American Red Cross has conducted a lookback study of the recipients of blood products from donors who develop CJD to assess the risk of blood-borne CJD transmission in the United States...
August 2017: Transfusion
https://www.readbyqxmd.com/read/28349199/amyloid-%C3%AE-accumulation-in-the-cns-in-human-growth-hormone-recipients-in-the-uk
#7
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...
August 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28332471/cjd-surveillance-in-the-republic-of-ireland-from-2005-to-2015-a-suggested-algorithm-for-referrals
#8
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/28110369/neuroradiology-of-human-prion-diseases-diagnosis-and-differential-diagnosis
#9
REVIEW
Simona Gaudino, Emma Gangemi, Raffaella Colantonio, Annibale Botto, Emanuela Ruberto, Rosalinda Calandrelli, Matia Martucci, Maria Gabriella Vita, Carlo Masullo, Alfonso Cerase, Cesare Colosimo
Human transmissible spongiform encephalopathies (TSEs), or prion diseases, are invariably fatal conditions associated with a range of clinical presentations. TSEs are classified as sporadic [e.g. sporadic Creutzfeldt-Jakob disease (sCJD), which is the most frequent form], genetic (e.g. Gerstmann-Straussler-Scheinker disease, fatal familial insomnia, and inherited CJD), and acquired or infectious (e.g. Kuru, iatrogenic CJD, and variant CJD). In the past, brain imaging played a supporting role in the diagnosis of TSEs, whereas nowadays magnetic resonance imaging (MRI) plays such a prominent role that MRI findings have been included in the diagnostic criteria for sCJD...
May 2017: La Radiologia Medica
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
#10
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/27942451/neuroanatomical-correlates-of-prion-disease-progression-a-3t-longitudinal-voxel-based-morphometry-study
#11
Enrico De Vita, Gerard R Ridgway, Mark J White, Marie-Claire Porter, Diana Caine, Peter Rudge, John Collinge, Tarek A Yousry, Hans Rolf Jager, Simon Mead, John S Thornton, Harpreet Hyare
PURPOSE: MRI has become an essential tool for prion disease diagnosis. However there exist only a few serial MRI studies of prion patients, and these mostly used whole brain summary measures or region of interest based approaches. We present here the first longitudinal voxel-based morphometry (VBM) study in prion disease. The aim of this study was to systematically characterise progressive atrophy in patients with prion disease and identify whether atrophy in specific brain structures correlates with clinical assessment...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27812793/uk-iatrogenic-creutzfeldt-jakob-disease-investigating-human-prion-transmission-across-genotypic-barriers-using-human-tissue-based-and-molecular-approaches
#12
Diane L Ritchie, Marcelo A Barria, Alexander H Peden, Helen M Yull, James Kirkpatrick, Peter Adlard, James W Ironside, Mark W Head
Creutzfeldt-Jakob disease (CJD) is the prototypic human prion disease that occurs most commonly in sporadic and genetic forms, but it is also transmissible and can be acquired through medical procedures, resulting in iatrogenic CJD (iCJD). The largest numbers of iCJD cases that have occurred worldwide have resulted from contaminated cadaveric pituitary-derived human growth hormone (hGH) and its use to treat primary and secondary growth hormone deficiency. We report a comprehensive, tissue-based and molecular genetic analysis of the largest series of UK hGH-iCJD cases reported to date, including in vitro kinetic molecular modelling of genotypic factors influencing prion transmission...
April 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/27649940/can-creutzfeldt-jakob-disease-unravel-the-mysteries-of-alzheimer
#13
Gabor G Kovacs
Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. Observations of recent studies can be summarized as follows: 1) The frequency of iatrogenic CJD cases with parencyhmal and vascular Aβ deposits is statistically higher than expected; 2) The morphology and distribution of Aβ deposition may show distinct features; 3) The pituitary and the dura mater themselves may serve as potential sources of Aβ seeds; 4) Cadaveric dura mater from 2 examined cases shows Aβ deposition; and 5) There is a lack of evidence that the clinical phenotype of AD appears following the application of cadaveric pituitary hormone or dura mater transplantation...
September 2, 2016: Prion
https://www.readbyqxmd.com/read/27597180/relative-abundance-of-apoe-and-a%C3%AE-1-42-associated-with-abnormal-prion-protein-differs-between-creutzfeldt-jakob-disease-subtypes
#14
Roger A Moore, Young Pyo Choi, Mark W Head, James W Ironside, Robert Faris, Diane L Ritchie, Gianluigi Zanusso, Suzette A Priola
Aggregated and protease-resistant mammalian prion protein (PrP(Sc)) is the primary protein component of infectious prions. Enriched PrP(Sc) preparations are often used to study the mechanisms that underly prion disease. However, most enrichment procedures are relatively nonspecific and tend to yield significant amounts of non-PrP(Sc) components including various proteins that could confound functional and structural studies. It is thus important to identify these proteins and assess their potential relevance to prion pathogenesis...
December 2, 2016: Journal of Proteome Research
https://www.readbyqxmd.com/read/27529062/prion-diseases-immunotargets-and-therapy
#15
REVIEW
Jennifer T Burchell, Peter K Panegyres
Transmissible spongiform encephathalopathies or prion diseases are a group of neurological disorders characterized by neuronal loss, spongiform degeneration, and activation of astrocytes or microglia. These diseases affect humans and animals with an extremely high prevalence in some species such as deer and elk in North America. Although rare in humans, they result in a devastatingly swift neurological progression with dementia and ataxia. Patients usually die within a year of diagnosis. Prion diseases are familial, sporadic, iatrogenic, or transmissible...
2016: ImmunoTargets and Therapy
https://www.readbyqxmd.com/read/27364252/human-prion-diseases-surgical-lessons-learned-from-iatrogenic-prion-transmission
#16
REVIEW
David J Bonda, Sunil Manjila, Prachi Mehndiratta, Fahd Khan, Benjamin R Miller, Kaine Onwuzulike, Gianfranco Puoti, Mark L Cohen, Lawrence B Schonberger, Ignazio Cali
The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood "infectious protein" has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts...
July 2016: Neurosurgical Focus
https://www.readbyqxmd.com/read/27214308/growth-hormone-and-cerebral-amyloidosis
#17
S Benvenga, F Guarneri
Great interest has recently been focused on a paper reporting characteristic deposits of amyloid-β protein associated with Alzheimer's disease in brains of adults who died of Creutzfeldt-Jakob disease. As they had contracted such disease after treatment with prion-contaminated human growth hormone extracted from cadaver-derived pituitaries, the authors have suggested that interhuman transmission of Alzheimer's disease had occurred. Our previous research led us to find that amyloid-forming peptides share amino acid sequence homology, summarized by a motif...
August 2016: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/27174240/advanced-tests-for-early-and-accurate-diagnosis-of-creutzfeldt-jakob-disease
#18
REVIEW
Gianluigi Zanusso, Salvatore Monaco, Maurizio Pocchiari, Byron Caughey
Early and accurate diagnosis of Creutzfeldt-Jakob disease (CJD) is a necessary to distinguish this untreatable disease from treatable rapidly progressive dementias, and to prevent iatrogenic transmission. Currently, definitive diagnosis of CJD requires detection of the abnormally folded, CJD-specific form of protease-resistant prion protein (PrP(CJD)) in brain tissue obtained postmortem or via biopsy; therefore, diagnosis of sporadic CJD in clinical practice is often challenging. Supporting investigations, including MRI, EEG and conventional analyses of cerebrospinal fluid (CSF) biomarkers, are helpful in the diagnostic work-up, but do not allow definitive diagnosis...
June 2016: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/27016065/dura-mater-is-a-potential-source-of-a%C3%AE-seeds
#19
Gabor G Kovacs, Mirjam I Lutz, Gerda Ricken, Thomas Ströbel, Romana Höftberger, Matthias Preusser, Günther Regelsberger, Selma Hönigschnabl, Angelika Reiner, Peter Fischer, Herbert Budka, Johannes A Hainfellner
Deposition of amyloid-β (Aβ) in the brain parenchyma and vessels is one of the hallmarks of Alzheimer disease (AD). Recent observations of Aβ deposition in iatrogenic Creutzfeldt-Jakob disease (iCJD) after dural grafting or treatment with pituitary extracts raised concerns whether Aβ is capable of transmitting disease as seen in prion diseases by the disease-associated prion protein. To address this issue, we re-sampled and re-evaluated archival material, including the grafted dura mater of two cases with iCJD (28 and 33-years-old) without mutations in the AβPP, PSEN1 and PSEN2 genes, and carrying ε3/ε3 alleles of the APOE gene...
June 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/26954665/correction-the-distribution-of-prion-protein-allotypes-differs-between-sporadic-and-iatrogenic-creutzfeldt-jakob-disease-patients
#20
Roger A Moore, Mark W Head, James W Ironside, Diane L Ritchie, Gianluigi Zanusso, Young Pyo Choi, Suzette A Priola
No abstract text is available yet for this article.
March 2016: PLoS Pathogens
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