MedWorm: Mad Cow Disease

'Mad Cow' and Other Prion Diseases Hide Out in Spleen

Scientific American - Official RSS Feed — Thu, 26 Jan 2012 20:00:00 +0100

By Jo Marchant of Nature magazinePrion diseases such as bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) are able to jump species much more easily than previously thought. [More] (Source: Scientific American - Official RSS Feed)

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Creutzfeldt-Jakob disease and mad cows: lessons learnt from yeast cells.

Swiss Medical Weekly — Thu, 26 Jan 2012 09:24:02 +0100

Authors: Hofmann J, Wolf H, Grassmann A, Arndt V, Graham J, Vorberg I Abstract Transmissible spongiform encephalopathies are fatal neurodegenerative diseases that affect mammals including humans. The proteinaceous nature of the infectious agent, the prion, and its propagation, challenge established dogmas in biology. It is now widely accepted that prion diseases are caused by unconventional agents principally composed of a misfolded host-encoded protein, PrP. Surprisingly, major break-throughs in prion research came from studies on functionally unrelated proteins in yeast and filamentous fungi. Aggregates composed of these proteins act as epigenetic elements of inheritance that can propagate their alternative states by a conformational switch into an ordered ß-sheet rich polymer j...

Symptomatic and Palliative Care for Stroke Survivors

Journal of General Internal Medicine — Thu, 19 Jan 2012 06:51:08 +0100

We present the current and established data to aid health care providers in symptomatic and palliative management of stroke survivors. Content Type Journal ArticleCategory ReviewsPages 1-8DOI 10.1007/s11606-011-1966-4Authors Claire J. Creutzfeldt, Department of Neurology, University of Washington Harborview Medical Center, Box 359775, 325 Ninth Ave, Seattle, WA 98104-2499, USARobert G. Holloway, Department of Neurology, University of Rochester Medical Center, Rochester, NY, USAMelanie Walker, Department of Neurology, University of Washington Harborview Medical Center, Box 359775, 325 Ninth Ave, Seattle, WA 98104-2499, USA Journal Journal of General Internal MedicineOnline ISSN 1525-1497Print ISSN 0884-8734 (Source: Journal of General Internal Medicine)

Adverse effects of growth hormone replacement therapy in children

Arquivos Brasileiros de Endocrinologia e Metabologia — Sun, 08 Jan 2012 19:33:01 +0100

Human growth hormone (hGH) replacement therapy has been widely available for clinical purposes for more than fifty years. Starting in 1958, hGH was obtained from cadaveric pituitaries, but in 1985 the association between hGH therapy and Creutzfeldt-Jakob disease was reported. In the same year, the use of recombinant hGH (rhGH) was approved. Side effects of rhGH replacement therapy in children and adolescents include rash and pain at injection site, transient fever, prepubertal gynecomastia, arthralgia, edema, benign intracranial hypertension, insulin resistance, progression of scoliosis, and slipped capital femoral epiphysis. Since GH stimulates cell multiplication, development of neoplasms is a concern. We will review the side effects reported in all rhGH indications.A terapia de reposiç...

Variant Creutzfeldt-Jakob disease occurring in mother and son

Journal of Neurology, Neurosurgery and Psychiatry — Fri, 06 Jan 2012 05:00:00 +0100

Introduction Variant Creutzfeldt–Jakob disease (vCJD) is distinguished from sporadic CJD by a number of characteristics, including a causal association with bovine spongiform encephalopathy (BSE) prions mainly through ingestion, lower sensitivity for 14-3-3 protein in cerebrospinal fluid (CSF), absence of triphasic periodic EEG and death at younger age (mean 28 years vs 65 years).1 Although millions of people have been potentially exposed to the infection, the number of recorded cases of vCJD to date has been small (~200). One explanation for the low number of cases is that, apart from the homozygosity for methionine at codon 129 of the prion protein gene (PRNP), additional genetic factors might influence the susceptibility to infection.2 3 If there are genetic factors that...

Assessing prion infectivity of human urine in sporadic creutzfeldt-jakob disease.

Emerging Infectious Diseases — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Notari S, Qing L, Pocchiari M, Dagdanova A, Hatcher K, Dogterom A, Groisman JF, Lumholtz IB, Puopolo M, Lasmezas C, Chen SG, Kong Q, Gambetti P Abstract Prion diseases are neurodegenerative conditions associated with a misfolded and infectious protein, scrapie prion protein (PrP(Sc)). PrP(Sc) propagate prion diseases within and between species and thus pose risks to public health. Prion infectivity or PrP(Sc) presence has been demonstrated in urine of experimentally infected animals, but there are no recent studies of urine from patients with Creutzfeldt-Jakob disease (CJD). We performed bioassays in transgenic mice expressing human PrP to assess prion infectivity in urine from patients affected by a common subtype of sporadic CJD, sCJDMM1. We tested raw urine and 100-fold...

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Desmoplakin as a Potential Candidate for Cerebrospinal Fluid Marker to Rule Out 14-3-3 False Positive Rates in Sporadic Creutzfeldt-Jakob Disease Differential Diagnosis.

Neuro-Degenerative Diseases — Sat, 31 Dec 2011 05:00:00 +0100

Conclusion: Desmoplakin showed a low positive rate accompanied by a very low false positive rate. Thus, we conclude that desmoplakin is a promising candidate for supportive CSF marker to rule out 14-3-3 false positive cases in sCJD differential diagnosis. PMID: 22213780 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)

Desmoplakin as a Potential Candidate for Cerebrospinal Fluid Marker to Rule Out 14-3-3 False Positive Rates in Sporadic Creutzfeldt-Jakob Disease Differential Diagnosis

Karger Publishers — Fri, 30 Dec 2011 23:00:00 +0100

Neurodegenerative Dis (DOI:10.1159/000334499) (Source: Karger Publishers)

Cerebrospinal fluid markers for differential dementia diagnosis in a large memory clinic cohort

Neurology — Mon, 26 Dec 2011 05:00:00 +0100

Conclusion: CSF Aβ42, t-tau, and p-tau are useful in differential dementia diagnosis. However, in DLB, FTLD, VaD, and CBD, a substantial group exhibit a CSF AD biomarker profile, which requires more autopsy confirmation in the future. (Source: Neurology)

The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey

Karger Publishers — Fri, 23 Dec 2011 23:00:00 +0100

Dement Geriatr Cogn Disord Extra 2011;1:429–432 (DOI:10.1159/000332024) (Source: Karger Publishers)

Risk of Prion Disease Transmission through Bovine‐Derived Bone Substitutes: A Systematic Review

Clinical Implant Dentistry and Related Research — Thu, 15 Dec 2011 05:00:00 +0100

Conclusion: This review indicates that bovine‐derived graft biomaterials may carry a risk of prion transmission to patients. (Source: Clinical Implant Dentistry and Related Research)

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Creutzfeldt-Jakob Disease as a Cause of Cognitive Decline and Seizures in the Elderly: Diagnostic Pointers and Strategy for Investigation

Infectious Diseases in Obstetrics and Gynecology — Tue, 13 Dec 2011 16:29:07 +0100

Cognitive decline affects one in twenty people over the age of 65. There is often a paucity of clues as to the underlying pathology, and while the diagnosis will usually prove to be either Alzheimer’s disease or vascular dementia, there may be clinical features suggesting rarer alternatives. This case of a 71-year-old lady with a 3-month history of progressive cognitive decline illustrates clinical features suggestive of Creutzfeltd-Jakob disease such as rapid decline in conscious level and myoclonic jerking. Diagnosis was confirmed by 3 means: (1) Electroencephalogram demonstrating periodic sharp wave complexes, (2) MRI brain showing cortical ribboning and high signal in the caudate nucleus, and (3) presence of protein S100 and protein14-3-3 in the cerebrospinal fluid. Postmortem brain ...

Change in the characteristics of ferritin induces iron imbalance in prion disease affected brains.

Neurobiology of Disease — Sun, 11 Dec 2011 05:00:00 +0100

Authors: Singh A, Qing L, Kong Q, Singh N Abstract Prion disease associated neurotoxicity is mainly attributed to PrP-scrapie (PrP(Sc)), the disease associated isoform of a normal protein, the prion protein (PrP(C)). Participation of other proteins and processes is suspected, but their identity and contribution to the pathogenic process is unclear. Emerging evidence implicates imbalance of brain iron homeostasis as a significant cause of prion disease-associated neurotoxicity. The underlying cause of this change, however, remains unclear. We demonstrate that iron is sequestered in heat and SDS-stable protein complexes in sporadic-Creutzfeldt-Jakob-disease (sCJD) brains, creating a phenotype of iron deficiency. The underlying cause is change in the characteristics of ferritin, an ir...

Protein insight into vCJD spread

BBC News | Health | UK Edition — Fri, 02 Dec 2011 07:47:22 +0100

Scientists discover blocking protein production in the immune system could prevent the spread of a disease that destroys nerve cells. (Source: BBC News | Health | UK Edition)

Hidden Side Of Prion Diseases Discovered By Medical Researchers In Canada And The US

Health News from Medical News Today — Thu, 01 Dec 2011 10:00:00 +0100

Medical researchers in Canada and the United States recently published their joint findings that fatal prion diseases, which include BSE or "mad cow disease," have a hidden signature. Findings published this month in the peer-reviewed journal, Public Library of Science (PLoS) Pathogens, demonstrate that up to seven months before an animal shows physical signs of having a prion infection, a particular prion protein in the brain was being eradicated. This member of the prion family is known as shadoo protein... (Source: Health News from Medical News Today)

Comparison of candidate vCJD in vitro diagnostic assays using identical sample sets

Vox Sanguinis — Thu, 01 Dec 2011 05:00:00 +0100

Conclusion The approach outlined has successfully compared in vitro diagnostics assays for their sensitivity and reproducibility and is a first step toward the evaluation of an assay suitable for blood donor screening/diagnosis of vCJD. (Source: Vox Sanguinis)

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Candidate cell substrates, vaccine production, and transmissible spongiform encephalopathies.

Emerging Infectious Diseases — Thu, 01 Dec 2011 05:00:00 +0100

The objective of this study was to determine the potential susceptibility to infection of 5 cell lines used or proposed for manufacture of biological products, as well as other lines. Cell lines were exposed to the infectious agents of sporadic and variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy (BSE). Exposed cultures were tested for TSE-associated prion protein (PrP(TSE)) and TSE infectivity by assay in rodents and nonhuman primates. No PrP(TSE) or infectivity has been detected in any exposed cell line under study so far. Animals inoculated with BSE brain homogenate developed typical spongiform encephalopathy. In contrast, animals inoculated with cells exposed to the BSE agent remained asymptomatic. All cell lines we studied resisted infection with 3 TSE agents, in...

Isolation of Prion with BSE Properties from Farmed Goat.

Emerging Infectious Diseases — Thu, 01 Dec 2011 05:00:00 +0100

Authors: Spiropoulos J, Lockey R, Sallis RE, Terry LA, Thorne L, Holder TM, Beck KE, Simmons MM Abstract Transmissible spongiform encephalopathies are fatal neurodegenerative diseases that include variant Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE) in cattle. Scrapie is not considered a public health risk, but BSE has been linked to variant Creutzfeldt-Jakob disease. Small ruminants are susceptible to BSE, and in 2005 BSE was identified in a farmed goat in France. We confirm another BSE case in a goat in which scrapie was originally diagnosed and retrospectively identified as suspected BSE. The prion strain in this case was further characterized by mouse bioassay after extraction from formaldehyde-fixed brain tissue em...

Preclinical sporadic Creutzfeldt-Jakob disease in French blood donors: an epidemiologic model-based study.

Transfusion — Thu, 01 Dec 2011 05:00:00 +0100

CONCLUSION: Few donors are expected to be in the late preclinical stage of sCJD at the time of blood donation. This result and that of the worldwide absence of any epidemic increase in sCJD over the years indicate that this risk of transfusion-transmitted sCJD, if any, is likely to be very low. PMID: 22128904 [PubMed - as supplied by publisher] (Source: Transfusion)

Medical researchers in Canada and the US discover hidden side of prion diseases

EurekAlert! - Medicine and Health — Wed, 30 Nov 2011 05:00:00 +0100

(University of Alberta Faculty of Medicine & Dentistry) Medical researchers recently discovered that fatal prion diseases, which include BSE or "mad cow disease," have a hidden signature.Researchers say the discovery tells them that prion diseases may not be unstoppable as once thought, because brain cells are in fact trying to get rid of a prion protein -- something the medical research community was previously unaware of. (Source: EurekAlert! - Medicine and Health)

Genome-wide study links MTMR7 gene to variant Creutzfeldt-Jakob risk.

Neurobiology of Aging — Wed, 30 Nov 2011 05:00:00 +0100

Authors: Sanchez-Juan P, Bishop MT, Aulchenko YS, Brandel JP, Rivadeneira F, Struchalin M, Lambert JC, Amouyel P, Combarros O, Sainz J, Carracedo A, Uitterlinden AG, Hofman A, Zerr I, Kretzschmar HA, Laplanche JL, Knight RS, Will RG, van Duijn CM Abstract The aim of our study was to discover genomic variations related to variant Creutzfeldt-Jakob disease (vCJD) susceptibility. A genome-wide association analysis with most vCJD samples available in the world was performed. A series of 93 vCJD UK patients and 1504 UK controls were included in the discovery stage. Our best findings were replicated in an independent population of 22 UK and 20 French vCJD cases. Post hoc analysis to assess our main results included 5711 French controls, 445 Dutch controls, and 446 sporadic Creutzfeldt-Ja...

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Creutzfeldt-jakob disease with paralysis of the unilateral vocal cord and soft palate.

The Tohoku Journal of Experimental Medicine — Tue, 22 Nov 2011 09:00:02 +0100

Authors: Hasegawa J, Okumura Y, Osumi E, Tago H, Katori Y, Kobayashi T Abstract Creutzfeldt-Jakob disease (CJD) is a progressive disease that is characterized by the accumulation of abnormal prion-like proteins in the central nervous system. The cerebral cortex is primarily affected in CJD, leading to spongiform changes and dementia. To date, there have been no reported cases of CJD, with local neuroparalysis discovered at an early stage of the disease. Here, we describe a patient who presented unilateral vocal cord and soft palate paralysis before the progression of CJD. After developing forgetfulness 6 months ago, a 76-year-old woman was presented at department of Otorhinolaryngology in a general hospital for recently developed hoarseness and dysphagia. In the oral and laryngeal ...

Comparison of nanofiltration efficacy in reducing infectivity of centrifuged versus ultracentrifuged 263K scrapie-infected brain homogenates in "spiked" albumin solutions.

Transfusion — Mon, 14 Nov 2011 05:00:00 +0100

CONCLUSION: These findings confirm the utility of nanofiltration in removing infectivity from plasma (or other products) spiked with scrapie brain homogenate supernatants. However, efficiency is diminished using supernatants that have been ultracentrifuged to reduce aggregated forms of the infectious agent. Thus, filtration removal data based on experiments using "standard" low-speed centrifugation supernatants might overestimate the amount of prion removal in plasma or urine-derived therapeutic products. PMID: 22082124 [PubMed - as supplied by publisher] (Source: Transfusion)

Creutzfeldt-Jakob Disease Surveillance in Argentina, 1997–2008

Neuroepidemiology — Fri, 04 Nov 2011 13:26:59 +0100

Neuroepidemiology 2011;37:193–202 (DOI:10.1159/000331907) (Source: Neuroepidemiology)

Rapidly Progressive Dementia: Experience in a Tertiary Care Medical Center

Alzheimer Disease and Associated Disorders — Wed, 02 Nov 2011 05:00:00 +0100

In conclusion, nonprion neurodegenerative diseases are the most common cause of RPD in our center. Our results suggest that although CJD is often suspected as a cause of RPD, its frequency depends on the referral differences across specialized centers (C) 2011 Lippincott Williams & Wilkins, Inc. (Source: Alzheimer Disease and Associated Disorders)

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance A Study in France from 2000 to 2008

Neuroepidemiology — Tue, 01 Nov 2011 23:00:00 +0100

Neuroepidemiology 2011;37:188–192 (DOI:10.1159/000332764) (Source: Neuroepidemiology)

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Could we face the return of CJD? Experts fear it may lie dormant in thousands

the Mail online | Health — Tue, 01 Nov 2011 08:09:04 +0100

Holly Mills is one of just three people still clinging to life after developing the human form of mad cow disease. As many as 15,000 people nationwide could be affected. (Source: the Mail online | Health)

Neuroscience in Nazi Europe Part II: Resistance against the Third Reich.

The Canadian Journal of Neurological Sciences — Tue, 01 Nov 2011 04:00:00 +0100

Authors: Zeidman LA Abstract Previously, I mentioned that not all neuroscientists collaborated with the Nazis, who from 1933 to 1945 tried to eliminate neurologic and psychiatric disease from the gene pool. Oskar and Cécile Vogt openly resisted and courageously protested against the Nazi regime and its policies, and have been discussed previously in the neurology literature. Here I discuss Alexander Mitscherlich, Haakon Saethre, Walther Spielmeyer, Jules Tinel, and Johannes Pompe. Other neuroscientists had ambivalent roles, including Hans Creutzfeldt, who has been discussed previously. Here, I discuss Max Nonne, Karl Bonhoeffer, and Oswald Bumke. The neuroscientists who resisted had different backgrounds and motivations that likely influenced their behavior, but this group undoubt...

Dura mater graft-associated creutzfeldt-jakob disease: the first case in Korea.

J Korean Med Sci — Tue, 01 Nov 2011 04:00:00 +0100

We report the first case of dCJD in Korea. A 54-yr-old woman, who underwent resection of the meningioma in the left frontal region and received a dura mater graft 23 yr ago presented with dysesthesia followed by psychiatric symptoms and ataxia. Her neurological symptoms rapidly progressed to such an extent that she exhibited myoclonus, dementia, and pyramidal and extrapyramidal signs within 8 weeks. The 14-3-3 protein was detected in her cerebrospinal fluid; however, an electroencephalogram did not reveal characteristic positive sharp wave complexes. Diffusion-weighted magnetic resonance images, obtained serially over 64 days, revealed the rapid progression of areas of high signal intensity in the caudate nucleus and cingulate gyrus to widespread areas of high signal intensity in the corte...

Mechanisms of prion disease progression: a chemical reaction network approach

IET Systems Biology — Tue, 01 Nov 2011 04:00:00 +0100

Fatal neurodegenerative diseases such as bovine spongiform encephalopathy in cattle, scrapie in sheep and Creutzfeldt-Jakob disease in humans are caused by prions. Prion is a protein encoded by a normal cellular gene. The cellular form of the prion, namely PrPC, is benign but can be converted into a disease-causing form (named scrapie), PrPSc, by a conformational change from α-helix to β-sheets. Prions replicate by this conformational change; that is, PrPSc interacts with PrPC producing a new molecule of PrPSc. This kind of replication is modelled in this contribution as an autocatalytic process. The kinetic model accounts for two of the three epidemiological manifestations: sporadic and infectious. By assuming irreversibility of the PrPSc replication and describing a first...

Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic Creutzfeldt-Jakob disease in Canada: a 6-year prospective study

BMC Neurology — Thu, 27 Oct 2011 04:00:00 +0100

Conclusions: CSF 14-3-3, tau and S100B proteins are useful diagnostic markers of sCJD even in a low-prevalence clinical population. CSF tau showed better overall diagnostic accuracy than 14-3-3 or S100B. Reporting of quantitative assay results and combining tau with S100B could enhance case definitions used in diagnosis and surveillance of sCJD. (Source: BMC Neurology)

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Alzheimer's 'may be infectious like mad cow disease'

the Mail online | Health — Wed, 05 Oct 2011 16:09:24 +0100

Alzheimer's disease could be the result of an infection in some cases, researchers at the University of Texas Health Science Center have found. (Source: the Mail online | Health)

Could Alzheimer's Be Infectious, Like Mad Cow, CJD?

Health News from Medical News Today — Wed, 05 Oct 2011 09:00:00 +0100

The brain damage seen in some cases of Alzheimer's disease could have its roots in an infectious prion-like disease, such as that seen in bovine spongiform encephalopathy (mad cow) and its human form Creutzfeldt-Jakob disease (CJD), according to an international study published this week in the journal Molecular Psychiatry that was led by the University of Texas Medical School at Houston in the US... (Source: Health News from Medical News Today)

Is Alzheimer's contagious? What new study says

Health News: CBSNews.com — Tue, 04 Oct 2011 19:53:06 +0100

Preliminary study suggests that Alzheimer's can spread from person to person much like "mad cow" disease spreads (Source: Health News: CBSNews.com)

University of Texas Health Science Center: Alzheimer's might be transmissible in similar way as infectious prion diseases

EurekAlert! - Social and Behavioral Science — Tue, 04 Oct 2011 04:00:00 +0100

(University of Texas Health Science Center at Houston) The brain damage that characterizes Alzheimer's disease may originate in a form similar to that of infectious prion diseases such as bovine spongiform encephalopathy (mad cow) and Creutzfeldt-Jakob, according to newly published research by The University of Texas Health Science Center at Houston. (Source: EurekAlert! - Social and Behavioral Science)

High sensitivity of an ELISA kit for detection of the gamma-isoform of 14-3-3 proteins: usefulness in laboratory diagnosis of human prion disease

BMC Neurology — Tue, 04 Oct 2011 04:00:00 +0100

Conclusion: The 14-3-3 gamma ELISA was more sensitive than conventional WB, and was useful for laboratory diagnosis of CJD, similar to the ELISA for the tau protein. Using DWI-MRI and these ELISA tests on CSF, diagnosis of CJD will be possible even at early stages of the disease. (Source: BMC Neurology)

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Lower risk of creutzfeldt-jakob disease in pituitary growth hormone recipients initiating treatment after 1977.

The Journal of Clinical Endocrinology and Metabolism — Sat, 01 Oct 2011 04:00:00 +0100

Conclusions: Risk of acquiring CJD was significantly lower for post-1977 NHPP hGH recipients than for pre-1977 recipients, suggesting that the new purification procedure in 1977 may have greatly reduced or eliminated CJD agent in hGH. PMID: 21816775 [PubMed - in process] (Source: The Journal of Clinical Endocrinology and Metabolism)

Sporadic Creutzfeldt-Jakob disease presenting as a stroke mimic590.

British Journal of Hospital Medicine — Sat, 01 Oct 2011 04:00:00 +0100

This article describes a 75-year-old man with a Heidenhain variant of sporadic Creutzfeldt-Jakob disease who presented with a sudden onset of homonymous hemianopia mimicking a stroke. PMID: 22041731 [PubMed - in process] (Source: British Journal of Hospital Medicine)

Clinical effects of leucoreduction of blood transfusions.

The Netherlands Journal of Medicine — Sat, 01 Oct 2011 04:00:00 +0100

Authors: Bilgin YM, van de Watering LM, Brand A Abstract For many years filtration for removal of leucocytes from red blood cell (RBC) and platelet transfusions was applied for selected patients to prevent cytomegalovirus (CMV) (re)activation, HLA immunisation and recurrent febrile nonhaemolytic transfusion reactions (FNHTR ). Since the 1980s, there was also growing concern about cancer recurrence and postoperative infections. In this review we discuss the studies on possible benefits of leucoreduction. In 2001 the Dutch Health Council decided that all blood products should undergo leucoreduction by filtration, as a precautionary measure to reduce possible transmission of variant Creutzfeld-Jacob disease (vCJD). The incidences of transfusion-transmitted CMV infection, HLA immunisat...

Demographics of successful, unsuccessful and deferral visits at six blood centers over a 4-year period.

Transfusion — Mon, 26 Sep 2011 04:00:00 +0100

CONCLUSION: Successful donation visits according to demographic characteristics need to be placed within the context of all donor visits. Deferral rates indicate that the burden of donor deferral is high. Efforts to expand the diversity of the donor base through recruitment of minority donors may bring additional challenges because certain deferral reasons were proportionally much higher in these groups. PMID: 21950621 [PubMed - as supplied by publisher] (Source: Transfusion)

De novo generation of prion strains

Nature Reviews Microbiology — Mon, 26 Sep 2011 04:00:00 +0100

Authors: David W. Colby & Stanley B. Prusiner Prions are self-replicating proteins that can cause neurodegenerative disorders such as bovine spongiform encephalopathy (also known as mad cow disease). Aberrant conformations of prion proteins accumulate in the central nervous system, causing spongiform changes in the brain and eventually death. Since the inception of the (Source: Nature Reviews Microbiology)

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Fibril formation of the rabbit/human/bovine prion proteins.

Biophysical Journal — Wed, 21 Sep 2011 04:00:00 +0100

Authors: Zhou Z, Yan X, Pan K, Chen J, Xie ZS, Xiao GF, Yang FQ, Liang Y Abstract Prion diseases are infectious fatal neurodegenerative diseases including Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in cattle. The misfolding and conversion of cellular PrP in such mammals into pathogenic PrP is believed to be the key procedure. Rabbits are among the few mammalian species that exhibit resistance to prion diseases, but little is known about the molecular mechanism underlying such resistance. Here, we report that the crowding agents Ficoll 70 and dextran 70 have different effects on fibrillization of the recombinant full-length PrPs from different species: although these agents dramatically promote fibril formation of the proteins from human and cow, they s...

We need to know the truth about vCJD numbers | Frank Dobson

Guardian Unlimited Science — Mon, 19 Sep 2011 10:06:47 +0100

It's estimated that one in 4,000 people are unknowingly infected. The government must authorise trials of a new blood testThe government's expert advisers assume that as many as 15,000 people in this country are infected with the prion infection agents that cause the lethal brain disease, variant Creutzfeldt-Jakob (vCJD), the human form of BSE or "mad cow disease". The experts don't know if this is the right figure. It could be a lot higher but ministers are refusing to fund trials of a new test to find out.So far just 200 of our fellow citizens have developed the disease. But the prions that cause the disease can lie dormant for decades and people who are infected pose a risk to others if they are blood or organ donors, or if surgical instruments used on them are then reused on other pati...

Leu‐138 in the bovine prion peptide fibrils is involved in the seeding discrimination related to codon‐129 M/V polymorphism in the prion peptide seeding experiment

FEBS Journal — Thu, 15 Sep 2011 04:00:00 +0100

SummaryThe risk of acquiring vCJD is closely related to polymorphism at codon 129 of the human prion gene, as almost all vCJD patients are Met/Met homozygotes. Although animal transmission experiments corroborated this seeding discrimination, the origin of the differential seeding efficiency of the bovine prion seed for human codon‐129 polymorphism remained elusive. Here we used a short PrP peptide as a model system to test whether seeding discrimination can be found in this simple system. We used previously developed “seed titration method” and time‐resolved circular dichroism spectroscopy to compare the sequence‐dependent seeding efficiency regarding codon‐129 polymorphism. Our results showed that the Met→Val substitution on the human PrP peptide ten times decreased the see...

Congress members seek to block approval of GM salmon

Guardian Unlimited Science — Mon, 12 Sep 2011 11:03:56 +0100

Opposition to the engineered fish led by Alaska delegation that says modified salmon is a threat to the state's wild salmon industryMembers of Congress are pushing to stop the Food and Drug Administration (FDA) from approving genetically engineered salmon, saying not enough is known about a fish they say could harm fishery businesses in coastal states.It appeared last year that the FDA might approve the engineered salmon quickly. But the congressional pushback and a lack of action by the FDA could mean the fish won't be on the nation's dinner tables any time soon.The fish, which grows twice as fast as the conventional variety, is engineered by AquaBounty, a Massachusetts-based company, but not yet allowed on the market. The company's application has been pending for more than 15 years. If ...

The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships

Brain — Sat, 10 Sep 2011 04:00:00 +0100

In this study, we examine diagnostic accuracy in a consecutive series of 228 patients referred to a specialist early-onset dementia clinic, whose brains were subsequently examined at post-mortem. Diagnosis was based on structured history, neurological examination and neuropsychological assessment, with emphasis on qualitative as well as quantitative aspects of performance. Neuroimaging provided support for but did not alter the clinical diagnosis. We set out the principles that guided diagnosis: (i) time course of illness; (ii) weighting of physical, behavioural and cognitive symptoms and signs; (iii) ‘anterior’ versus ‘posterior’ hemisphere character of cognitive change; and (iv) specificity of deficit, paying attention to the differentiation between syndromes of f...

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RNA integrity in post mortem human variant Creutzfeldt–Jakob disease (vCJD) and control brain tissue

Neuropathology and Applied Neurobiology — Sat, 03 Sep 2011 17:30:42 +0100

Conclusion: Reasonable‐quality RNA can be isolated from samples dissected from archived frozen human half brains but repeated sampling results in RNA degradation. Better‐quality RNA is obtained from samples placed in RNALater than from snap frozen samples. The quality and yield of RNA are not affected by age at death, gender, post mortem interval of >6 h or freezer storage time. (Source: Neuropathology and Applied Neurobiology)

Blood Testing For Creutzfeldt-Jakob Disease

Clinical Neurology News — Thu, 01 Sep 2011 04:00:00 +0100

Brain MRI has improved the diagnosis of Creutzfeldt-Jakob disease, which is often supported by the pulvinar sign (seen in the posteromedial thalamus of C and D in these images of a patient with variant CJD transmitted through a blood transfusion). However, encouraging reports of blood tests for abnormal prion proteins in development suggest that they have the potential to serve the dual roles of screening blood and tissue donors as well as diagnosing patients with prion diseases. See story on page 8. (Source: Clinical Neurology News)

Is a Blood Test for Variant CJD on the Horizon?

Clinical Neurology News — Thu, 01 Sep 2011 04:00:00 +0100

The prospects for a practical test that quickly detects the presence of prions in blood, organs, and tissues became more encouraging with the results of two studies published earlier this year. (Source: Clinical Neurology News)

Atypical presentation of Creutzfeldt-Jakob disease: a rare but important cause of rapidly progressive dementia.

Connecticut Medicine — Thu, 01 Sep 2011 04:00:00 +0100

We report an atypical presentation of sporadic Creutzfeldt-Jakob disease (CJD) in a 74-year-old woman that illustrates the difficulty in diagnosing this rare, but important, cause of rapidly progressive dementia. Despite well-established criteria, this diagnosis is often missed or substantially delayed (Table 1). In this case, a precipitous cognitive decline associated with a urinary tract infection initiallysuggested delirium. Although atypical CJD was considered as a cause when symptoms persisted, a definitive diagnosis was established postmortem when the cerebrospinal fluid (CSF) prion protein 14-3-3 tested positive. Creutzfeldt-Jakob disease must be considered in the differential diagnosis of rapidly progressive dementia as Connecticut accounts for approximately three of the more than ...

Rapid emergence of temporal and pulvinar lesions in MELAS mimicking Creutzfeldt-Jakob disease

Neurology — Sun, 28 Aug 2011 23:00:00 +0100

(Source: Neurology)

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Purified Growth Hormone Not Tied to Creutzfeldt-Jakob DiseasePurified Growth Hormone Not Tied to Creutzfeldt-Jakob Disease

Medscape Medical News Headlines — Mon, 22 Aug 2011 23:02:46 +0100

Short kids who were treated with human growth hormone after 1977 -- when scientists started purifying the hormone -- aren't as likely to develop Creutzfeldt-Jakob disease as a result, researchers say. Reuters Health Information (Source: Medscape Medical News Headlines)

Purified growth hormone not tied to Creutzfeldt-Jakob disease

Modern Medicine — Fri, 19 Aug 2011 23:00:00 +0100

NEW YORK (Reuters Health) - Short kids who were treated with human growth hormone after 1977 -- when scientists started purifying the hormone -- aren't as likely to develop Creutzfeldt-Jakob disease as a result, researchers say. (Source: Modern Medicine)

Beef producers still recovering from mad cow disease

CTV Health — Tue, 16 Aug 2011 12:47:14 +0100

Canada's beef producers are still feeling the economic impact eight years after mad cow disease was discovered in Alberta. (Source: CTV Health)

The first report of RPSA polymorphisms, also called 37/67 kDa LRP/LR gene, in sporadic Creutzfeldt-Jakob disease (CJD)

BMC Medical Genetics - Latest articles — Fri, 12 Aug 2011 23:00:00 +0100

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[Two new cases of sporadic Creutzfeldt-Jakob disease in Madrid, Spain].

Revista de Neurologia — Fri, 12 Aug 2011 07:00:39 +0100

CONCLUSIONS. Such a high incidence over such a short period of time and in a health district of barely 170,000 inhabitants seems rather surprising. PMID: 21796606 [PubMed - in process] (Source: Revista de Neurologia)

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Timeline of FDA raids on raw milk farmers, dietary supplement makers and natural medicine practitioners

NaturalNews.com — Wed, 10 Aug 2011 07:00:00 +0100

(NaturalNews) The US Food and Drug Administration has a long history of conducting armed, SWAT-style raids on farmers, cancer treatment pioneers and dietary supplement manufacturers. This list, compiled by the editors of NaturalNews, reveals only some of the hundreds of armed FDA raids that have been conducted in the last twenty-five years.What you see from this is a pattern of government-sponsored terrorism against innocent Americans and small business people; all done in the name of "protecting" the public from milk, walnuts, vitamins, plants or fruit extracts. The real reason behind all this, of course, is that the FDA has long waged a campaign of fear and intimidation against natural product providers for the sole purpose of destroying the natural products industry and thereby handing ...

Budget cuts mean no CJD screening of donated blood

The Irish Times - Health — Mon, 01 Aug 2011 23:19:42 +0100

THE MINISTER FOR Health has been advised not to introduce a new technology to screen donated blood for Creutzfeldt-Jakob disease because it would not be cost effective to do so. (Source: The Irish Times - Health)

Biochemical and strain properties of CJD prions: complexity versus simplicity

Journal of Neurochemistry — Sun, 24 Jul 2011 23:00:00 +0100

Prions, the agents responsible for transmissible spongiform encephalopathies, are infectious proteins consisting primarily of scrapie prion protein (PrPSc), a misfolded, beta‐sheet enriched and aggregated form of the host‐encoded cellular prion protein (PrPC). Their propagation is based on an autocatalytic PrP conversion process. Despite the lack of a nucleic acid genome, different prion strains have been isolated from animal diseases. Increasing evidence supports the view that strain‐specific properties may be enciphered within conformational variations of PrPSc. In humans, sporadic Creutzfeldt‐Jakob disease (sCJD) is the most frequent form of prion diseases and has demonstrated a wide phenotypic and molecular spectrum. In contrast, variant Creutzfeldt‐Jakob disease (vCJD), whic...

Study Of Prion Diseases And Alzheimer's To Benefit From $600,000 Research Grants

Health News from Medical News Today — Sun, 24 Jul 2011 07:00:00 +0100

The University of Western Ontario is one of nine universities which will share 2.9 million dollars in research grants announced by PrioNet Canada to study Prion diseases and neurodegenerative disorders including Alzheimer's. Prion diseases are fatal, infectious and transmissible neurodegenerative diseases affecting both humans and animals including mad cow disease or bovine spongiform encephalopathy (BSE), Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. The goal of the funding which supports 11 projects is two-fold, explains Dr... (Source: Health News from Medical News Today)

Western researchers receive $600,000 to study Prion diseases and Alzheimer's

EurekAlert! - Medicine and Health — Wed, 20 Jul 2011 04:00:00 +0100

(University of Western Ontario) A team of researchers from The University of Western Ontario has received $600,000 from PrioNet Canada to study Prion diseases and Alzheimer's. Prion diseases are fatal, infectious and transmissible neurodegenerative diseases affecting both humans and animals including mad cow disease or bovine spongiform encephalopathy, Creutzfeldt-Jakob disease in humans, and chronic wasting disease in deer and elk. The team led by Marco Prado will investigate whether prion protein could be a therapeutic target in Alzheimer disease. (Source: EurekAlert! - Medicine and Health)

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Impact of Being Placed at Risk of Creutzfeldt-Jakob Disease: A Qualitative Study of Blood Donors to Variant CJD Cases and Patients Potentially Surgically Exposed to CJD

Neuroepidemiology — Mon, 11 Jul 2011 23:00:00 +0100

Neuroepidemiology 2011;36:274–281 (DOI:10.1159/000328646) (Source: Neuroepidemiology)

Diphenyl-pyrazole derived compounds increase survival time of mice after prion infection.

Antimicrobial Agents and Chemotherapy — Sun, 10 Jul 2011 23:00:00 +0100

Authors: Leidel F, Eiden M, Geissen M, Kretzschmar HA, Giese A, Hirschberger T, Tavan P, Schätzl HM, Groschup MH Transmissible spongiform encephalopathies (TSEs) represent a group of fatal neurodegenerative disorders which can be transmitted by natural infection or inoculation. TSEs include scrapie in sheep, BSE in cattle, and Creutzfeldt-Jakob disease (CJD) in humans. The emergence of a variant form of CJD (vCJD) which has been associated to BSE, produced strong pressure to search for effective treatments with new drugs. Up to now, however, TSEs are incurable, although many efforts have been made in vitro as well as in vivo for the search for potent therapeutic and prophylactic compounds. For this purpose we analyzed a compound library consisting of 10.000 compounds with a cell-based...

Early detection of sporadic CJD by diffusion-weighted MRI before the onset of symptoms

Journal of Neurology, Neurosurgery and Psychiatry — Wed, 06 Jul 2011 23:00:00 +0100

According to the WHO diagnostic criteria,1 sporadic Creutzfeldt–Jakob disease (sCJD) is diagnosed by characteristic electroencephalographic (EEG) findings, the presence of 14-3-3 protein in the cerebrospinal fluid (CSF) and appropriate clinical symptoms.1 Recent studies have made progress towards establishing a diagnosis of sCJD from the combination of diffusion-weighed MR images and 14-3-3 protein detected in the CSF; however, the earliest markers of this disease are not known. We evaluated a patient with sCJD who exhibited abnormal high-intensity signals on diffusion-weighed MR imaging (DWI) during an incidental medical check-up that included an MRI. Here, we report the chronological changes in clinical and MRI findings beginning 2 months before disease onset to end-stage sCJD...

Conserved properties of human and bovine prion strains on transmission to guinea pigs

Laboratory Investigation AOP — Sun, 03 Jul 2011 23:00:00 +0100

Authors: Jiri G Safar, Kurt Giles, Pierre Lessard, Frederic Letessier, Smita Patel, Ana Serban, Stephen J DeArmond & Stanley B Prusiner (Source: Laboratory Investigation AOP)

Atypical Creutzfeldt-Jakob Disease Evolution after Electroconvulsive Therapy for Catatonic Depression

Clinical and Developmental Immunology — Sun, 03 Jul 2011 14:03:27 +0100

We describe a case report of an 80-year-old woman who presented with symptomatology compatible with an episode of major depression with catatonia. After psychiatric admission, electroconvulsive therapy (ECT) was applied, but symptoms progressed with cognitive impairment, bradykinesia, widespread stiffness, postural tremor, and gait disturbance. After compatible magnetic resonance imaging (MRI), diffusion changes, and electroencephalogram (EEG) findings the case was reoriented to Creutzfeldt-Jakob disease (CJD). The genetic study found a methionine/valine heterozygosity at codon 129 of the prion protein gene PrPSc. On followup, a significant clinical recovery turned out. For this reason, EEG and MRI were repeated and confirmed the findings. The patient subsequently demonstrated progressive ...

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Tracking Down BSE And Creutzfeldt-Jakob Disease

Health News from Medical News Today — Fri, 01 Jul 2011 07:00:00 +0100

Prion diseases such as Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy (BSE) in cattle are transmissible neurodegenerative diseases linked to the aggregation of the prion protein in the central nervous system. It is known that the aggregation of prion proteins promotes neuronal decay with fatal consequences for the infected individual. However, there is only a limited understanding of how neurons are lost and which molecules are involved... (Source: Health News from Medical News Today)

Tumefactive demyelination and glioblastoma: a rare collision lesion.

Clinical Neuropathology — Thu, 30 Jun 2011 23:00:00 +0100

Conclusion: Our experience underscores the importance of adequate tissue sampling during biopsy for suspected glioma, and confirms the fact that active inflammatory demyelination may coexist with a high-grade glioma. Despite detailed study, the basis for the association remains elusive. PMID: 21726504 [PubMed - in process] (Source: Clinical Neuropathology)

Frequency of Met129Val allele associated with predisposition to variant Creutzfeldt - Jakob disease in the Middle ages

Central European Journal of Medicine — Thu, 30 Jun 2011 17:47:54 +0100

We examined the frequency of the alleles encoding methionine and valine at codon 129 in DNA isolated from 100 skeletal remains of individuals who lived between 10th and 13th century. Our results confirmed significant alteration in previously studied alleles frequency between the populations of medieval Polish Lands and contemporaries. The calculated frequency of the alleles in medieval Poland (51% as compared to contemporary 65% for 129Met, and appropriately 49% vs. 35% for 129Val) implies a selection process that shaped 129 Met-Val distribution profiles in the Middle Ages. We suggest that the study of the genetic relationship between past and present-day populations could be a useful tool to follow allelic composition of particular genes (here: of the PRNP) over a span of time which ...

Rinderpest Is Dead; Second Disease In History Declared Eradicated

Health News from Medical News Today — Tue, 28 Jun 2011 17:00:00 +0100

The Romans couldn't beat it when they ruled the world, but today in Rome the UN has declared the eradication of rinderpest, the second disease in all of human history to be successfully wiped out after smallpox. Scientists are celebrating victory over a deadly animal disease that cattle herders around the world have dreaded for millennia. We are still very reliant on livestock to feed our bellies and economy, but way back when if your animals fell to rinderpest your family had a good chance of starving and succumbing to a similar fate... (Source: Health News from Medical News Today)

South Korea lifts 8-year ban on Canadian beef imports

CTV Health — Tue, 28 Jun 2011 13:32:52 +0100

South Korea says it has decided to resume imports of Canadian beef, lifting an eight-year ban imposed over fears of mad cow disease. (Source: CTV Health)

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Breaking the chain: 'Molecular cap' blocks processes that lead to Alzheimer's, HIV

UCLA Newsroom: Health Sciences — Wed, 22 Jun 2011 21:40:00 +0100

This study was published June 14 in PLoS Biology, an online journal of the Public Library of Science. "These are the first small molecules visualized as they bind to amyloid-like fibers," Eisenberg said. "These small molecules are less likely to be broken up in the body and can potentially be modified to force apart amyloid fibers or serve as diagnostic tools to identify infected areas of the body." Eisenberg and his research team found that orange-G was uniquely able to pierce the impenetrable "steric zippers" that seal the water-tight amyloid fibers of the amyloid-beta protein that is responsible for forming senile plaques in Alzheimer's disease. "In 10 years we have gotten to the point where we are starting to understand the structural biology of amyloid fibers and ...

Pros and cons of a prion-like pathogenesis in Parkinson's disease

BMC Neurology — Sun, 19 Jun 2011 23:00:00 +0100

DiscussionIt has recently been found that alpha-synuclein may behave similarly to the prion precursor and propagate between cells. The post-mortem proof of alpha-synuclein containing Lewy bodies in embryonic dopamine cells transplants in PD patient suggests that the misfolded protein might be transmitted from the diseased host to donor neurons reminiscent of prion behavior. The involvement of the basal ganglia and brainstem in the degenerative process are other congruencies between Parkinson's and Creutzfeldt-Jakob disease. However, a number of issues advise caution before categorizing Parkinson's disease as a prion disorder, because clinical appearance, brain imaging, cerebrospinal fluid and neuropathological findings exhibit fundamental differences between both disease entities. Most of ...

Drugs Being Developed To Tackle CJD Could Also Help Prevent Alzheimer's

Health News from Medical News Today — Thu, 09 Jun 2011 09:00:00 +0100

Scientists funded by the Medical Research Council (MRC) and Science Foundation Ireland (SFI) have identified two antibodies which could help block the onset of Alzheimer's disease in the brain. The antibodies, ICSM-18 and ICSM-35, were already known to play a crucial role in preventing 'protein misfolding', the main cause of Creutzfeldt-Jakob disease (CJD), the human form of mad cow disease... (Source: Health News from Medical News Today)

Is there evidence of vertical transmission of variant Creutzfeldt-Jakob disease?

Journal of Neurology, Neurosurgery and Psychiatry — Wed, 08 Jun 2011 23:00:00 +0100

Objectives The possibility of vertical transmission of variant Creutzfeldt–Jakob disease (vCJD) has been raised because of the widespread distribution of infectivity in vCJD and the demonstration that this condition can be transmitted through blood transfusion. The aim of this study is to search for evidence of this type of transmission of vCJD. Methods A national surveillance system for CJD has been established in the UK since 1990. Through this register, details were extracted of all children born to vCJD cases up to March 2009. This list was checked against the CJD register and cases identified through the UK study of Progressive Intellectual and Neurological Deterioration in children to determine whether any of the children of vCJD cases had themselves developed a progressive ne...

CJD protein sheds light on Alzheimer's

NHS News Feed — Wed, 08 Jun 2011 17:30:00 +0100

Conclusion This animal research supports the theory that the prion protein plays a role in the effects that the amyloid beta protein has on neurones. It also suggests that using antibodies can prevent at least one effect of amyloid protein on the nerve cells. It is important to note that the study looked at only one effect of amyloid beta on nerve cells: the effect on one aspect of neurone signalling called long-term potentiation, which is involved in learning and memory. Alzheimer’s is a complex disease and is largely caused by the death of neurones in certain areas of the brain. What causes the death of neurones in this disease is still not fully understood. Blocking the effects of amyloid beta on long-term potentiation may not be sufficient to stop neurones dying and, therefore, to af...

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Discovery that mad cow disease drugs block onset of Alzheimer's is 'significant step forward' in fight against condition

the Mail online | Health — Wed, 08 Jun 2011 11:56:14 +0100

While developing treatments for Creutzfeldt-Jakob Disease it was found that two of the antibodies unexpectedly also worked to treat Alzheimer's. (Source: the Mail online | Health)

Surprise discovery allows scientists to block Alzheimer's

The Independent - Science — Tue, 07 Jun 2011 23:00:01 +0100

Scientists developing treatments for the devastating brain disorder Creutzfeldt-Jakob Disease (CJD) have unexpectedly blocked the onset of Alzheimer's disease, the most common cause of dementia. (Source: The Independent - Science)

CJD drugs could help Alzheimer's patients

Telegraph Health — Tue, 07 Jun 2011 15:15:04 +0100

Drugs being developed to tackle the human form of mad cow disease could also help patients with dementia, it is hoped. (Source: Telegraph Health)

Estimation of variant Creutzfeldt-Jakob disease infectivity titers in human blood.

Transfusion — Thu, 02 Jun 2011 23:00:00 +0100

CONCLUSION: Our analysis suggested that, while less than one ID(iv ) is likely to be present in a given unit of NLR-RBCs collected from a donor incubating vCJD, there is a high probability of TT infection among recipients of vCJD-implicated blood components. The analysis supports continuing measures currently recommended to reduce the risk of TTvCJD. PMID: 21645006 [PubMed - as supplied by publisher] (Source: Transfusion)

Call for papers for a special issue on Creutzfeldt-Jakob Disease.

Euro Surveill — Tue, 31 May 2011 04:00:03 +0100

Authors: PMID: 21586263 [PubMed - in process] (Source: Euro Surveill)

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Two Gene Classes Linked To New Prion Formation

Health News from Medical News Today — Fri, 27 May 2011 07:00:00 +0100

Unlocking the mechanisms that cause neurodegenerative prion diseases may require a genetic key, suggest new findings reported by University of Illinois at Chicago distinguished professor of biological sciences Susan Liebman. Prions can turn a normal protein into a misfolded form. One prion in mammals promotes progressive neurodegenerative disorders like "mad cow" disease that often prove fatal. But how this process happens remains an open question for scientists. Prions have been found to exist in a wide range of organisms... (Source: Health News from Medical News Today)

Inherited prion disease with 4-octapeptide repeat insertion: disease requires the interaction of multiple genetic risk factors

Brain — Wed, 25 May 2011 23:00:00 +0100

Genetic factors are implicated in the aetiology of sporadic late-onset neurodegenerative diseases. Whether these genetic variants are predominantly common or rare, and how multiple genetic factors interact with each other to cause disease is poorly understood. Inherited prion diseases are highly heterogeneous and may be clinically mistaken for sporadic Creutzfeldt–Jakob disease because of a negative family history. Here we report our investigation of patients from the UK with four extra octapeptide repeats, which suggest that the risk of clinical disease is increased by a combination of the mutation and a susceptibilty haplotype on the wild-type chromosome. The predominant clinical syndrome is a progressive cortical dementia with pyramidal signs, myoclonus and cerebellar abnormalitie...

CDC Assesses Potential Human Exposure to Prion Diseases

MedlinePlus Health News — Wed, 25 May 2011 18:56:11 +0100

Source: American Dietetic Association Related MedlinePlus Pages: Creutzfeldt-Jakob Disease, Degenerative Nerve Diseases (Source: MedlinePlus Health News)

CDC Warns Against Exposure to 'Mad Cow'-Like Brain Diseases

MedlinePlus Health News — Mon, 23 May 2011 16:00:00 +0100

Travel to certain countries, hunting, eating wild deer meat raises risks, experts say Source: HealthDay Related MedlinePlus Pages: Creutzfeldt-Jakob Disease, Nutrition (Source: MedlinePlus Health News)

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CDC assesses potential human exposure to prion diseases

EurekAlert! - Medicine and Health — Mon, 23 May 2011 04:00:00 +0100

(Elsevier Health Sciences) Researchers from the Centers for Disease Control and Prevention have examined the potential for human exposure to prion diseases, looking at hunting, venison consumption, and travel to areas in which prion diseases have been reported in animals. Three prion diseases in particular -- bovine spongiform encephalopathy, variant Creutzfeldt-Jakob disease and chronic wasting disease -- were specified in the investigation. The results of this investigation are published in the June issue of the Journal of the American Dietetic Association. (Source: EurekAlert! - Medicine and Health)

CDC Assesses Potential Human Exposure To Prion Diseases Study Results Reported In The Journal Of The American Dietetic Association

Health News from Medical News Today — Mon, 23 May 2011 04:00:00 +0100

Researchers from the Centers for Disease Control and Prevention (CDC) have examined the potential for human exposure to prion diseases, looking at hunting, venison consumption, and travel to areas in which prion diseases have been reported in animals. Three prion diseases in particular - bovine spongiform encephalopathy (BSE or "Mad Cow Disease"), variant Creutzfeldt-Jakob disease (vCJD), and chronic wasting disease (CWD) - were specified in the investigation. The results of this investigation are published in the June issue of the Journal of the American Dietetic Association... (Source: Health News from Medical News Today)

A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt-Jakob disease

BMC Medical Genetics - Latest articles — Sat, 21 May 2011 23:00:00 +0100

Conclusions: Our findings support the hypothesis that genetic variations in the PRNP promoter may have a role in the pathogenesis of sCJD. (Source: BMC Medical Genetics - Latest articles)

Natural Born Prion Killers: Lichens Degrade 'Mad Cow' Related Brain Pathogen

Scientific American - Official RSS Feed — Thu, 19 May 2011 18:30:00 +0100

Remember mad cow disease? In the 1980s, cattle in the U.K. had begun contracting a fatal brain ailment triggered by an infectious protein called a prion. The pathogen could spread to humans who ate contaminated beef. Officials brought the bovine epidemic under control with major changes in agricultural practices.Unfortunately, the same cannot be said for a cousin of the sickness, one that targets deer, elk and moose in the U.S. Called chronic wasting disease (CWD), or "mad deer" disease, the ailment poses an unusual challenge in that it has spread among wild populations, not among herded animals. Wild animals go where they may, so you cannot institute controls the way you can for livestock. So it comes as good news that a naturally occurring disinfectant exists within common lich...

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Natural Born Prion Killers: Lichens Degrade "Mad Cow" Related Brain Pathogen

Scientific American - Official RSS Feed — Thu, 19 May 2011 18:30:00 +0100

Rapid screening and confirmatory methods for biochemical diagnosis of human prion disease.

Journal of Virological Methods — Mon, 16 May 2011 23:00:00 +0100

In conclusion, these rapid and robust in vitro tools were suitable for routine human TSE diagnosis and characterisation. CJD could also be diagnosed during the patient's lifetime by detection of PrPsc in the tonsil. A diagnostic strategy associating TeSeE™ CJD ELISA screening to biochemical confirmation by TeSeE™ Western blot is proposed. PMID: 21619894 [PubMed - as supplied by publisher] (Source: Journal of Virological Methods)

Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias

Neurology — Sun, 15 May 2011 23:00:00 +0100

Conclusions: The pattern of FLAIR/DWI hyperintensity and restricted diffusion can differentiate sCJD from other RPDs with a high sensitivity and specificity. MRI with DWI and ADC should be included in sCJD diagnostic criteria. New sCJD MRI criteria are proposed. (Source: Neurology)

When Coroners Said No To Post-Mortem Tissue Collection They Were Wrong, Academics Argue

Health News from Medical News Today — Tue, 10 May 2011 10:00:00 +0100

The Coroner's Society "failed" in its duty to protect public health by refusing to take part in vCJD study. The creation of a post-mortem tissue archive for a study of the human form of mad cow disease failed because of a "misguided" refusal by coroners to participate. The Coroners' Society of England and Wales (CSEW) did not recognise its "moral obligation to protect public health" from potential new risks associated with variant Creutzfeldt-Jakob disease (vCJD) when it decided not to allow the collection of tissue from autopsies... (Source: Health News from Medical News Today)

Fast, Sensitive Blood Test For Human Prion Disease

Health News from Medical News Today — Tue, 10 May 2011 09:00:00 +0100

WHAT: Scientists from the National Institute of Allergy and Infectious Diseases (NIAID), part of the National Institutes of Health (NIH), report that they have developed a method -10,000 times more sensitive than other methods - to detect variant Creutzfeldt-Jacob disease (vCJD) in blood plasma. vCJD is a type of prion disease in humans that leads to brain damage and death. The NIAID researchers also used the test to rapidly detect scrapie, a prion disease of sheep, in infected hamsters, some pre-symptomatic... (Source: Health News from Medical News Today)

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Creutzfeldt-Jakob disease: Coroners wrong to say no to post-mortem tissue collection, UK academics argue

ScienceDaily Headlines — Mon, 09 May 2011 10:57:57 +0100

The creation of a post-mortem tissue archive for a study of the human form of mad cow disease failed because of a 'misguided' refusal by coroners to participate, academics in the UK argue in a new article. (Source: ScienceDaily Headlines)

Coroners wrong to say no to post-mortem tissue collection, academics argue

EurekAlert! - Social and Behavioral Science — Mon, 09 May 2011 04:00:00 +0100

(London School of Hygiene & Tropical Medicine) The creation of a post-mortem tissue archive for a study of the human form of mad cow disease failed because of a "misguided" refusal by coroners to participate. (Source: EurekAlert! - Social and Behavioral Science)

NIH study describes fast, sensitive blood test for human prion disease

EurekAlert! - Infectious and Emerging Diseases — Mon, 09 May 2011 04:00:00 +0100

(NIH/National Institute of Allergy and Infectious Diseases) Scientists from NIAID report that they have developed a method -- 10,000 times more sensitive than other methods -- to detect variant Creutzfeldt-Jacob disease (vCJD) in blood plasma. vCJD is a type of prion disease in humans that leads to brain damage and death. The NIAID researchers also used the test to rapidly detect scrapie, a prion disease of sheep, in infected hamsters, some pre-symptomatic. (Source: EurekAlert! - Infectious and Emerging Diseases)

Diagnosing variant Creutzfeldt-Jakob disease: a retrospective analysis of the first 150 cases in the UK

Journal of Neurology, Neurosurgery and Psychiatry — Thu, 05 May 2011 23:00:00 +0100

Conclusions Early clinical diagnosis in vCJD is not possible in the great majority of cases because of non-specific initial symptoms. Once neurological signs develop, a diagnosis is usually made promptly but this is often at a relatively advanced stage of illness. The inherent delays in the diagnosis of vCJD have implications for those involved in both public health and therapeutics. (Source: Journal of Neurology, Neurosurgery and Psychiatry)

Call for papers for a special issue on Creutzfeldt-Jakob Disease

Eurosurveillance latest news — Thu, 05 May 2011 00:00:00 +0100

(Source: Eurosurveillance latest news)

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Clinical relevance of the HLA system in blood transfusion

Vox Sanguinis — Mon, 02 May 2011 23:00:00 +0100

HLA alloimmunization induced by pregnancy, multiple transfusions or transplantation is responsible for some of the serious complications seen in patients receiving blood and blood products. These complications are primarily the result of antibody and antigen triggering an acute immunological reaction, which in some cases can be fatal e.g. TRALI. Some adverse reactions are triggered by HLA antibodies present in the patient whereas others are initiated by antibodies or HLA reactive cells present in the transfused product. The introduction of universal leucodepletion for the prevention of vCJD transmission has resulted in a significant reduction in these reactions by eliminating the main source of alloimmunization, but residual cellular components or platelets are still able to activate the i...

Knockdown of prion protein (PrP) by RNA interference weakens the protective activity of wild-type PrP against copper ion and antagonizes the cytotoxicity of fCJD-associated PrP mutants in cultured cells.

International Journal of Molecular Medicine — Sun, 01 May 2011 23:00:00 +0100

In this report, based on analysis of the known sequences of human PrP, we constructed two small interfering RNA (siRNA) duplexes targeting the segments of amino acids (aa) 108-114 (Ri2) and aa 171-177 (Ri3). Western blot analysis results revealed that these PrP-specific siRNAs could effectively knock down the levels of either endogenous PrP in human neuroblastoma SHSY-5Y cells or recombinant PrP transfected with the plasmid expressing the full-length human PrP in human embryonic kidney (HEK) 293T cells. Meanwhile, the two siRNAs also showed a significant effect on the reduction of the expression of the PrP-PG9 and PrP-PG12 familial Creutzfeldt-Jakob disease (CJD)-associated PrP mutants with four and seven extra octarepeats, in the cells transfected with the respective expression plasmids. ...

Creutzfeldt-Jacob disease mimics, or how to sort out the subacute encephalopathy patient

Postgraduate Medical Journal — Thu, 21 Apr 2011 23:00:00 +0100

Sporadic Creutzfeldt–Jacob disease (CJD) is a rare untreatable neurodegenerative disease which every neurologist will occasionally encounter during their career. However, it is likely to appear on their differential diagnosis list significantly more frequently. Numerous conditions can present with subacute encephalopathy which might be sporadic CJD and this article explores these diagnoses. It includes the commonest sporadic CJD mimics which are neurodegenerative, and highlights the relatively rare treatable mimics which must not be missed. It discusses relevant investigations, including serum antibodies, CSF, electroencephalography and MR brain imaging, and strategies when preliminary investigations fail to support sporadic CJD but no alternative diagnosis is readily apparent. (Sour...

Prion infection begins after one minute of exposure

Medical Research Council Press Releases — Tue, 19 Apr 2011 12:00:00 +0100

Scientists funded by the Medical Research Council (MRC) have shown that one minute of exposure to infecting prions is enough to begin the chain reaction of events which lead to prion disease in the brain, causing Creutzfeldt-Jakob disease (Source: Medical Research Council Press Releases)

Infusion of P-Capt prion-filtered red blood cell products demonstrate acceptable in vivo viability and no evidence of neoantigen formation.

Transfusion — Wed, 13 Apr 2011 23:00:00 +0100

CONCLUSIONS: P-Capt-filtered, stored RBCs demonstrated acceptable viability and no detectable neoantigen expression, immunogenic responses. or safety issues after infusion of a complete unit. The additional filtration time and modest reduction in RBC content are within acceptable levels for implementation in countries with transfusion transmission of vCJD. PMID: 21492178 [PubMed - as supplied by publisher] (Source: Transfusion)

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Prions and protein folding diseases

Journal of Internal Medicine — Sun, 10 Apr 2011 23:00:00 +0100

Abstract Prions represent a group of proteins with a unique capacity to fold into different conformations. One isoform is rich in beta‐pleated sheets and can aggregate into amyloid that may be pathogenic. This abnormal form propagates itself by imposing its confirmation on the homologous normal host cell protein. Pathogenic prions have been shown to cause lethal neurodegenerative diseases in humans and animals. These diseases are sometimes infectious and hence referred to as transmissible spongiform encephalopathies. In the present review, the remarkable evolution of the heterodox prion concept is summarized. The origin of this phenomenon is based on information transfer between homologous proteins, without the involvement of nucleic acid‐encoded mechanisms. Historically kuru and Creut...

Prions and protein‐folding diseases

Journal of Internal Medicine — Sun, 10 Apr 2011 23:00:00 +0100

Abstract. Norrby E (Royal Swedish Academy of Sciences, Stockholm, Sweden). Prions and protein‐folding diseases (Review). J Intern Med 2011; doi: 10.1111/j.1365‐2796.2011.02387.xPrions represent a group of proteins with a unique capacity to fold into different conformations. One isoform is rich in beta‐pleated sheets and can aggregate into amyloid that may be pathogenic. This abnormal form propagates itself by imposing its confirmation on the homologous normal host cell protein. Pathogenic prions have been shown to cause lethal neurodegenerative diseases in humans and animals. These diseases are sometimes infectious and hence referred to as transmissible spongiform encephalopathies. In the present review, the remarkable evolution of the heterodox prion concept is summarized. The or...

STERIS Pioneers Prion Decontamination Technologies

Health News from Medical News Today — Thu, 07 Apr 2011 12:00:00 +0100

Following recent patient safety alerts, prion diseases - such as Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD) - are of increasing concern within the healthcare environment, as their causative agents can be extremely resistant to the cleaning, disinfection and sterilization methods currently used in many hospitals. STERIS has been at the forefront of research in this area, developing novel technologies that reduce the risk of surface prion contamination... (Source: Health News from Medical News Today)

Canada restricts blood donors from Saudi Arabia

CTV Health — Wed, 30 Mar 2011 18:09:50 +0100

Former residents of Saudi Arabia, or Canadians who spent more than six months in Saudi Arabia during the 1980s or early '90s, will no longer be allowed to donate blood because of fears of mad cow disease. (Source: CTV Health)

Canadian Blood Services To Expand Deferral Policy For vCJD

Health News from Medical News Today — Wed, 30 Mar 2011 08:00:00 +0100

Canadian Blood Services is expanding its deferral policy for vCJD (variant Creutzfeldt -Jakob disease) policy to include Saudi Arabia. A new question will be added to the Record of Donation donor questionnaire asking each donor if he or she has spent a cumulative total of six months or more in Saudi Arabia between 1980 and 1996. Answering yes will result in that person not being eligible to donate blood for transfusion... (Source: Health News from Medical News Today)

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Two-year wait to hear of CJD risk

Scotsman.com News - Health — Tue, 29 Mar 2011 23:41:10 +0100

Health chiefs have admitted they waited two years to contact 38 patients who are at risk of contracting Creutzfeldt-Jakob disease after surgery. (Source: Scotsman.com News - Health)

Second hospital reports CJD risk

BBC News | Health | UK Edition — Tue, 29 Mar 2011 17:05:44 +0100

Patients in Wales and Essex may have been put at risk of contracting Creutzfeldt-Jakob Disease (CJD) during surgery, it has been revealed. (Source: BBC News | Health | UK Edition)

Blood Donation Deferral Policy Expanded Beyond the United Kingdom and Western Europe

Government of Canada News - Health Canada — Tue, 29 Mar 2011 15:30:00 +0100

Health Canada, in consultation with Canadian Blood Services and Héma-Québec, is announcing new protective measures regarding blood donation. This follows the Public Health Agency of Canada report (http://www.phac-aspc.gc.ca/ccdrw-rmtch/2011/ccdrw-rmtcs1011r-eng.php) of a new case of laboratory-diagnosed probable vCJD (Variant Creuzfeldt-Jacob Disease) in a Canadian resident. (Source: Government of Canada News - Health Canada)

CJD: 38 patients at risk of fatal brain disease Creutzfeldt-Jakob Disease after surgery at UK hospital

the Mail online | Health — Tue, 29 Mar 2011 13:09:56 +0100

Letters have been sent to affected patients after it was founds that a person who had an operation in a Welsh hospital in 2007 was at 'high risk' of CJD. (Source: the Mail online | Health)

38 hospital patients in CJD risk

BBC News | Health | UK Edition — Tue, 29 Mar 2011 11:10:02 +0100

Thirty eight patients may have been put at risk of contracting Creutzfeldt-Jakob Disease (CJD) during surgery in south Wales, it is revealed. (Source: BBC News | Health | UK Edition)

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Cancer is a p53 protein aggregation disease

EurekAlert! - Medicine and Health — Tue, 29 Mar 2011 04:00:00 +0100

(VIB (the Flanders Institute for Biotechnology)) Protein aggregation, generally associated with Alzheimer's and mad cow disease, turns out to play a significant role in cancer. Certain mutations of p53 cause the protein to aggregate, disrupting its protective function. (Source: EurekAlert! - Medicine and Health)

38 Welsh patients at risk of ‘mad cow’s disease’

OnMedica Latest News — Mon, 28 Mar 2011 23:00:00 +0100

Surgery in south Wales puts 38 patients at risk of Creutzfeldt-Jakob Disease Related items from OnMedicaDH to publish surgery mortality figuresOne small step on a complicated journeyMaidstone makes 'huge strides' in infection controlChange to flu vaccine ordering system mootedHealth Bill “is a massive gamble” (Source: OnMedica Latest News)

Coroners and the obligation to protect public health: The case of the failed UK vCJD study

Public Health — Sun, 27 Mar 2011 23:00:00 +0100

Summary: The Health Protection Agency has recently attempted to create a postmortem tissue archive to determine the prevalence of abnormal prion protein. The success of this archive was prevented because the Health Protection Agency could not convince coroners to support the study’s methodology and participate on that basis. The findings of this paper detail and support the view that the Coroners’ Society of England and Wales’s refusal to participate was misguided and failed to appreciate that coroners have a moral obligation to protect public health. Measures to assist coroners in fulfilling this role are proposed. (Source: Public Health)

Some fertility drugs carry theoretical risk of deadly disease

CTV Health — Fri, 25 Mar 2011 12:29:39 +0100

Women injected with urine-derived fertility drugs could be at risk of developing the human form of mad cow disease, although no cases have yet been linked to the drugs, researchers say. (Source: CTV Health)

Psychoses as the presenting manifestation of Creutzfeldt-Jakob disease in an elderly male

General Hospital Psychiatry — Fri, 25 Mar 2011 00:00:00 +0100

We report the case of a healthy elderly man who presents with acute psychosis. We describe our diagnostic evaluation based on the patient's history and physical examination, which identified Creutzfeldt-Jakob disease. Avoiding diagnostic error in patients who face such a rapidly progressive and catastrophic illness is paramount. (Source: General Hospital Psychiatry)

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Sporadic Creutzfeldt-Jakob disease mimicking nonconvulsive status epilepticus

Neurology — Mon, 21 Mar 2011 00:00:00 +0100

(Source: Neurology)

Notification and support for people exposed to the risk of Creutzfeldt-Jakob disease (CJD) (or other prion diseases) through medical treatment (iatrogenically).

Cochrane Database of Systematic Reviews — Sat, 19 Mar 2011 21:00:08 +0100

Authors: Ryan R, Hill S, Lowe D, Allen K, Taylor M, Mead C Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD) are rare and always-fatal diseases transmissible via certain medical procedures. If a person is exposed to the disease risk through medical treatment, they may need to be notified of this to prevent them passing the risk to others in healthcare settings and to enable additional infection control measures to be put in place for certain procedures. As CJD is incurable, and unable to be screened for or effectively treated, communicating this risk information after an exposure incident may have significant implications for the person at risk, their families/ carers and healthcare professionals. The best ways to notify people of their exposure to the risk of CJD or vCJD, and to ...

Focus on Prion Diseases

Medicineworld.org: New Article Alert — Tue, 15 Mar 2011 12:40:37 +0100

New research by Chongsuk Ryou, researcher at the UK Sanders-Brown Center on Aging and professor of microbiology, immunology and molecular genetics in the UK College of Medicine, may shed light on possible therapys for prion diseases. Prion diseases, which include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy ("mad cow" disease) in cattle, are caused by prions - unconventional pathogens composed of infectious protein particles and resistant to conventional sterilization procedures. Presently there is no known agent or procedure that can halt or reverse damage caused by prion disease........ (Source: Medicineworld.org: New Article Alert)

New Research Focuses On Prion Diseases

Health News from Medical News Today — Tue, 15 Mar 2011 10:00:00 +0100

New research by Chongsuk Ryou, researcher at the UK Sanders-Brown Center on Aging and professor of microbiology, immunology and molecular genetics in the UK College of Medicine, may shed light on possible treatments for prion diseases. Prion diseases, which include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy ("mad cow" disease) in cattle, are caused by prions - unconventional pathogens composed of infectious protein particles and resistant to conventional sterilization procedures... (Source: Health News from Medical News Today)

[Emergent viral threats in blood transfusion.]

Transfusion Clinique et Biologique — Tue, 15 Mar 2011 00:00:00 +0100

Authors: Pozzetto B, Garraud O During the last 20 years, the safety of blood products increased dramatically with regard to the infectious risk and notably to that represented by retroviruses (HIV and HTLV) and hepatitis B and C viruses. The aim of this review is to identify the residual and emergent viral threats that could be responsible for the occurring of new contaminations in the receivers of blood products. Beside many other viruses (HHV-8, erythrovirus B19, hepatitis A and E viruses…), a special attention has been paid to emerging arbovirus diseases (West Nile virus infection, dengue, chikungunya) that threaten to occur in the French metropolitan area following the implantation in Europe of the mosquito Aedes albopictus, the main vector of dengue and chikungunya in temperate...

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Tau and p-tau as CSF biomarkers in dementia: a meta-analysis

Clinical Chemistry and Laboratory Medicine — Fri, 11 Mar 2011 03:52:45 +0100

Conclusions: CSF tau concentrations in DLB, FTLD and VaD are intermediate between controls and AD patients. Overlap with both controls and AD patients results in insufficient diagnostic accuracy, and the development of more specific biomarkers for these disorders is needed. CJD is characterized by extremely increased tau values, resulting in a sensitivity and specificity that exceeds 90%. (Source: Clinical Chemistry and Laboratory Medicine)

Researchers Identify Biomarker For Creutzfeldt-Jakob Disease

Health News from Medical News Today — Thu, 10 Mar 2011 10:00:00 +0100

Neena Singh, MD, PhD and colleagues at Case Western Reserve University School of Medicine have identified the first disease-specific biomarker for sporadic Creutzfeldt-Jakob disease (sCJD), a universally fatal, degenerative brain disease for which there is no cure. sCJD is one of the causes of dementia and typically leads to death within a year of disease onset. The finding, published in the March 9th issue of PLoS ONE, a scientific journal produced by the Public Library of Science, provides a basis for developing a test to diagnosis sCJD while patients are still alive... (Source: Health News from Medical News Today)

New biomarker for Creutzfeldt-Jakob disease identified

ScienceDaily Headlines — Wed, 09 Mar 2011 23:27:27 +0100

Researchers have identified the first disease-specific biomarker for sporadic Creutzfeldt-Jakob disease (sCJD), a universally fatal, degenerative brain disease for which there is no cure. sCJD is one of the causes of dementia and typically leads to death within a year of disease onset. (Source: ScienceDaily Headlines)

Researchers identify new biomarker for Creutzfeldt-Jakob disease, the human form of mad cow disease

EurekAlert! - Medicine and Health — Wed, 09 Mar 2011 05:00:00 +0100

(Case Western Reserve University) Neena Singh, MD, PhD and colleagues at Case Western Reserve University School of Medicine have identified the first disease-specific biomarker for sporadic Creutzfeldt-Jakob disease (sCJD), a universally fatal, degenerative brain disease for which there is no cure. sCJD is one of the causes of dementia and typically leads to death within a year of disease onset. (Source: EurekAlert! - Medicine and Health)

VIDEO: Man loses his 10-year vCJD battle

BBC News | Health | UK Edition — Mon, 07 Mar 2011 18:30:16 +0100

A Belfast man who suffered variant CJD - the human form of mad cow disease - has died, 10 years after he first became ill. (Source: BBC News | Health | UK Edition)

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Man loses his 10-year vCJD battle

BBC News | Health | UK Edition — Mon, 07 Mar 2011 17:16:28 +0100

A Belfast man who was one of the world's longest survivors of vCJD - the human form of mad cow disease - has died, nearly ten years after he first became ill. (Source: BBC News | Health | UK Edition)

FDA Approves Revised Alphanate Labeling to Include Reduced Infectivity of an Experimental Model Agent for vCJD

Drugs.com - Pharma News — Mon, 07 Mar 2011 14:23:04 +0100

Alphanate® is the first plasma-derived blood clotting factor to include labeling statements on manufacturing processes that provide a reasonable assurance of removal for low levels of a variant Creutzfeldt-Jakob disease (vCJD) model... (Source: Drugs.com - Pharma News)

What kills cows, saves birds

The Scientist — Mon, 07 Mar 2011 00:00:00 +0100

Mad cow disease outbreaks in Europe may be favorable to bird populations across the pond (Source: The Scientist)

Co-Occurrence of Types 1 and 2 PrP(res) in Sporadic Creutzfeldt-Jakob Disease MM1.

The American Journal of Pathology — Tue, 01 Mar 2011 00:00:00 +0100

Authors: Kobayashi A, Mizukoshi K, Iwasaki Y, Miyata H, Yoshida Y, Kitamoto T The genotype (M/M, M/V, or V/V) at polymorphic codon 129 of the human prion protein (PrP) gene and the type (1 or 2) of protease-resistant PrP (PrP(res)) in the brain are major determinants of the clinicopathological phenotypes of sporadic Creutzfeldt-Jakob disease (sCJD). According to this molecular typing system, sCJD has been classified into six subgroups (MM1, MM2, MV1, MV2, VV1, and VV2). Besides these pure subgroups, mixed cases presenting mixed neuropathological phenotypes and more than one PrP(res) type have been found in sCJD. To investigate the frequency of the co-occurrence of types 1 and 2 PrP(res) in sCJD patients classified as MM1, we produced type 2 PrP(res)-specific antibody Tohoku 2 (T2) that...

Serum tau protein as a marker for the diagnosis of Creutzfeldt-Jakob disease

Journal of Neurology — Mon, 28 Feb 2011 17:14:23 +0100

Abstract Total tau protein (t-tau) levels in cerebrospinal fluid (CSF) (CSF-tau) are markedly elevated in patients with Creutzfeldt-Jakob disease (CJD). Some CSF-tau may leak into the blood. We evaluated t-tau levels in serum (serum-tau) as a possible marker for the differential diagnosis of CJD from Alzheimer’s disease (AD) and other rapidly progressive dementias (RPD). Serum- and CSF-tau levels were determined in patients with sporadic CJD (n = 12), AD (n = 10) and RPD but no CJD (non-CJD–RPD; n = 9) who showed RPD fulfilling the World Health Organization (WHO) criteria for possible CJD at onset and had a final diagnosis other than CJD. We also analyzed serum-tau levels in healthy volunteers as a control group (n = 10). Serum- as...

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Potential Treatment For Prion Diseases

Health News from Medical News Today — Fri, 25 Feb 2011 09:00:00 +0100

Scientists who examined more than 10,000 chemical compounds during the last year in search of potential new drugs for a group of untreatable brain diseases, are reporting that one substance shows unusual promise. The early positive signs for so-called prion diseases come from research in laboratory mice and cell cultures, they say in a report in ACS' Journal of Medicinal Chemistry. Adam Renslo and colleagues, who include Nobel Laureate Stanley B... (Source: Health News from Medical News Today)

Universal White Blood Cell Reduction in Europe: Has Transmission of Variant Creutzfeldt-Jakob Disease Been Prevented?

Transfusion Medicine Reviews — Thu, 24 Feb 2011 00:00:00 +0100

Universal white blood cell (WBC) reduction was introduced in Europe to prevent transmission of variant Creutzfeldt-Jakob disease (vCJD) by transfusion. Findings from rodent models indicate that WBC reduction should not prevent vCJD transmission because the residual plasma infectivity suffices to infect transfusion recipients even under optimistic infectivity assumptions. Although infectivity in human blood may not partition in the manner in which it is distributed in rodents, prion-reduction filters remove the residual plasma infectivity in rodent models. Precautionary introduction of prion filtration in the UK—for patients without dietary exposure to bovine spongiform encephalopathy and in the absence of a reported case of vCJD transmission attributable to infectivity residing in plasma...

The risk of variant Creutzfeldt‐Jakob disease among UK patients with bleeding disorders, known to have received potentially contaminated plasma products

Haemophilia — Wed, 23 Feb 2011 00:00:00 +0100

Summary. The risk of variant Creutzfeldt‐Jakob disease (vCJD) from potentially infected plasma products remains unquantified. This risk has been assessed for 787 UK patients with an inherited bleeding disorder prospectively followed‐up for 10–20 years through the UK Haemophilia Centre Doctors’ Organisation (UKHCDO) Surveillance Study. These patients had been treated with any of 25 ‘implicated’ clotting factor batches from 1987 to 1999, which included in their manufacture, plasma from eight donors who subsequently developed clinical vCJD. Variant CJD infectivity of these batches was estimated using plasma fraction infectivity estimates and batch‐manufacturing data. Total potential vCJD infectivity received by each patient has been estimated by cumulating estimated infecti...

Prion propagation and toxicity in vivo occur in two distinct mechanistic phases

Nature — Wed, 23 Feb 2011 00:00:00 +0100

Authors: Malin K. Sandberg, Huda Al-Doujaily, Bernadette Sharps, Anthony R. Clarke & John Collinge Mammalian prions cause fatal neurodegenerative conditions including Creutzfeldt–Jakob disease in humans and scrapie and bovine spongiform encephalopathy in animals. Prion infections are typically associated with remarkably prolonged but highly consistent incubation periods followed by a rapid clinical phase. The relationship between prion propagation, generation of neurotoxic species and clinical onset has remained obscure. Prion incubation periods in experimental animals are known to vary inversely with expression level of cellular prion protein. Here we demonstrate that prion propagation in brain proceeds via two distinct phases: a clinically silent exponential phase not rate...

Iatrogenic Creutzfeldt-Jakob disease in Australia: time to amend infection control measures for pituitary hormone recipients?

Med J Aust — Mon, 21 Feb 2011 00:00:00 +0100

Authors: Solvyns SL, Ralston DW PMID: 21401477 [PubMed - in process] (Source: Med J Aust)

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Improving Understanding Of The Spread Of Infectious Prions

Health News from Medical News Today — Sat, 19 Feb 2011 08:00:00 +0100

Researchers at the University of California, San Diego School of Medicine have identified the motors that move non-infectious prion proteins (PrPC) - found within many mammalian cells - up and down long, neuronal transport pathways. Identifying normal movement mechanisms of PrPC may help researchers understand the spread of infectious prions within and between neurons to reach the brain, and aid in development of therapies to halt the transport. Their study is published in the February 18 edition of the journal Cell. The small prion protein is found in the cell membrane of brain neurons... (Source: Health News from Medical News Today)

Absence of Association between Two HECTD2 Polymorphisms and Sporadic Creutzfeldt-Jakob Disease

Dementia and Geriatric Cognitive Disorders — Wed, 16 Feb 2011 23:00:00 +0100

Dement Geriatr Cogn Disord 2011;31:146–151 (DOI:10.1159/000324133) (Source: Dementia and Geriatric Cognitive Disorders)

Factors affecting the accuracy of urine-based biomarkers of BSE

Proteome Science — Mon, 07 Feb 2011 00:00:00 +0100

Conclusion: Despite the presence of confounding factors, the disease specific changes in abundance exhibited by a panel of urine proteins permitted the creation of classifiers able to discriminate between control and infected cattle with a high degree of accuracy. (Source: Proteome Science)

[Articles] Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay

LANCET — Fri, 04 Feb 2011 18:04:00 +0100

These initial studies provide a prototype blood test for diagnosis of vCJD in symptomatic individuals, which could allow development of large-scale screening tests for asymptomatic vCJD prion infection. (Source: LANCET)

[Comment] A prototype assay to detect vCJD-infected blood

LANCET — Fri, 04 Feb 2011 18:03:55 +0100

Variant Creutzfeldt-Jakob disease (vCJD) is a human infection in the family of transmissible spongiform encephalopathies (TSEs) or prion diseases. Most cases of vCJD have resulted from consumption of food infected with the bovine spongiform encephalopathy agent, but infection has also been transmitted by blood transfusions and plasma products. Although the disease is very rare, with only 171 cases of dietary vCJD (220 cases worldwide) and five blood-related vCJD infections reported in the UK, its effect on transfusion medicine has been enormous. (Source: LANCET)

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Prototype Blood Test for Variant Creutzfeldt-Jakob Disease Developed

Medscape Infectious Diseases Headlines — Fri, 04 Feb 2011 15:32:57 +0100

Initial studies show the assay can detect vCJD-infected human blood and could help researchers on the road to developing a screening test for blood donors. Medscape Medical News (Source: Medscape Infectious Diseases Headlines)

Human mad cow blood test developed

CBC | Health — Fri, 04 Feb 2011 01:45:41 +0100

British scientists have developed a preliminary blood test that could one day be used to detect the proteins that cause the human form of mad cow disease, according to a new study. (Source: CBC | Health)

Blood Test Spots Variant CJD (CME/CE)

MedPage Today Infectious Disease — Thu, 03 Feb 2011 20:17:18 +0100

(MedPage Today) -- A blood test to detect the abnormal prion proteins responsible for variant Creutzfeldt-Jakob disease (vCJD) -- aka mad cow disease -- showed good sensitivity and specificity in an early-stage study, researchers said. (Source: MedPage Today Infectious Disease)

U.K. scientists creating mad cow disease blood test

CTV Health — Thu, 03 Feb 2011 13:27:34 +0100