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Leeds Research Finds New Piece Of BSE PuzzleEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
New research funded mainly through the Wellcome Trust with additional support from the Medical Research Council shows that a new treatment route for bovine spongiform encephalopathy (BSE) and its human form Creutzfeldt Jakob disease (CJD) could be a step closer. The research carried out by scientists at the University of Leeds was published today (November 20) in PLoS Pathogens. (Source: Health News from Medical News Today)
Source: Health News from Medical News Today - November 20, 2009 Category: Consumer Health News Tags: CJD / vCJD / mad cow disease Source Type: news

Leeds Research Finds New Piece Of BSE PuzzleEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
New research funded mainly through the Wellcome Trust with additional support from the Medical Research Council shows that a new treatment route for bovine spongiform encephalopathy (BSE) and its human form Creutzfeldt Jakob disease (CJD) could be a step closer. The research carried out by scientists at the University of Leeds was published today (November 20) in PLoS Pathogens. (Source: CJD / vCJD / Mad Cow Disease News From Medical News Today)
Source: CJD / vCJD / Mad Cow Disease News From Medical News Today - November 20, 2009 Category: Infectious Diseases Tags: CJD / vCJD / mad cow disease Source Type: news

Brain Disease "Resistance Gene" Could Offer Insights Into CJDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A community in Papua New Guinea that suffered a major epidemic of a CJD-like fatal brain disease called kuru has developed strong genetic resistance to the disease, according to new research by Medical Research Council (MRC) scientists. Kuru is a fatal prion disease, similar to CJD in humans and BSE in animals, and is geographically unique to an area in Papua New Guinea. (Source: Health News from Medical News Today)
Source: Health News from Medical News Today - November 20, 2009 Category: Consumer Health News Tags: CJD / vCJD / mad cow disease Source Type: news

Brain Disease "Resistance Gene" Could Offer Insights Into CJDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A community in Papua New Guinea that suffered a major epidemic of a CJD-like fatal brain disease called kuru has developed strong genetic resistance to the disease, according to new research by Medical Research Council (MRC) scientists. Kuru is a fatal prion disease, similar to CJD in humans and BSE in animals, and is geographically unique to an area in Papua New Guinea. (Source: CJD / vCJD / Mad Cow Disease News From Medical News Today)
Source: CJD / vCJD / Mad Cow Disease News From Medical News Today - November 20, 2009 Category: Infectious Diseases Tags: CJD / vCJD / mad cow disease Source Type: news

Cannibalism clue to brain diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion This study has shown that variations in two particular regions of the gene that codes for the prion protein are more common in those people who were exposed to the kuru disease but who had not become infected. The researchers note that they cannot rule out the possibility that one of the mutations is responsible for kuru disease, but discuss several reasons why this is highly unlikely. The study demonstrates that the kuru disease resulted in a strong selection pressure in this population. This means that any individuals with characteristics that would make them less susceptible to the disease would be more lik...
Source: NHS News Feed - November 20, 2009 Category: Consumer Health News Tags: Neurology Genetics/stem cells Source Type: news

ProMetic Life Sciences Inc.: SaBTO Recommends Use of P-Capt(R) Prion Reduction Filter to Protect Children From vCJD Blood TransmissionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
MONTREAL, QUEBEC, CANADA and LILLE, FRANCE (MARKET WIRE) ProMetic Life Sciences Inc. (TSX: PLI) ("ProMetic") and MacoPharma SA ("MacoPharma") announce that the Advisory Committee on the Safety of Blood, Tissues and Organs ("SaBTO"), an independent Committee that advises the UK Department of Health ("DoH"), has recommended the adoption of the P-Capt(R) prion reduction filter to pre-treat red blood cells destined for children born since 1 January 1996. The filter, which 'cleans' blood prior to use, removes the prion responsible for variant Creutzfeldt-Jakob disease ("vCJD"). The Committee also indicated that the requirement ...
Source: Market Wire - Pharmaceuticals and Biotech - November 20, 2009 Category: Pharmaceuticals Tags: Pharmaceuticals and Biotech PLI Source Type: news

Scientists make mad cow discoveryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Researchers at the University of Leeds discover a type of protein which may play a part in causing "mad cow disease". (Source: BBC News | Health | UK Edition)
Source: BBC News | Health | UK Edition - November 20, 2009 Category: Consumer Health News Tags: West Yorkshire Source Type: news

The Protein Srebp2 Drives Cholesterol Formation In Prion-Infected Neuronal Cells Which May Promote Prion-Dependent DiseasesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Prions are causing fatal and infectious diseases of the nervous system, such as the mad cow disease (BSE), scrapie in sheep or Creutzfeldt-Jakob disease in humans. Scientists of Helmholtz Zentrum MÃ¼nchen and Technische UniversitÃ¤t MÃ¼nchen have now succeeded in elucidating another disease mechanism of prion diseases: The prion-infected cell changes its gene expression and produces increased quantities of cholesterol. Prions need this for their propagation. (Source: Neurology / Neuroscience News From Medical News Today)
Source: Neurology / Neuroscience News From Medical News Today - November 19, 2009 Category: Neuroscience Tags: Neurology / Neuroscience Source Type: news

Immune tribe 'indicates CJD hope'Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Darwinian natural selection could help halt human "mad cow disease", experts say after finding a tribe impervious to a related fatal brain disorder. (Source: BBC News | Health | UK Edition)
Source: BBC News | Health | UK Edition - November 19, 2009 Category: Consumer Health News Tags: Health Source Type: news

Tribes resistance could help CJDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Darwinian natural selection could help halt human "mad cow disease", experts say after finding a tribe impervious to a related fatal brain disorder. (Source: BBC News | Health | UK Edition)
Source: BBC News | Health | UK Edition - November 19, 2009 Category: Consumer Health News Tags: Health Source Type: news

Inherited Creutzfeldt-Jakob disease in a Dutch patient with a novel five octapeptide repeat insertion and unusual cerebellar morphologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
An atypical case of inherited Creutzfeldt–Jakob disease (CJD) is described in a 35-year-old Dutch woman, homozygous for methionine at codon 129 of the prion protein gene (PRNP). The clinical phenotype was characterised by slowly progressive cognitive decline and parkinsonism. Neuropathological findings consisted of scanty spongiosis and only faint to absent immunohistochemical staining for the abnormal prion protein, PrPSc, with patchy deposits in the cerebellar cortex. Purkinje cells were abnormally located in the molecular layer of the cerebellum. Western blot analysis showed the co-occurrence of PrPSc types 1 and ...
Source: Journal of Neurology, Neurosurgery & Psychiatry - November 16, 2009 Category: Neurosurgery Authors: Jansen, C, van Swieten, J C, Capellari, S, Strammiello, R, Parchi, P, Rozemuller, A J M Tags: Genetics, Immunology (including allergy), Brain stem / cerebellum, Memory disorders (neurology), Infection (neurology), Parkinson's disease, Neuropathology, Memory disorders (psychiatry) Short reports Source Type: journals

Genetic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Journal of Neurology, Neurosurgery & Psychiatry)
Source: Journal of Neurology, Neurosurgery & Psychiatry - November 16, 2009 Category: Neurosurgery Authors: Kovacs, G G, Horvath, S, Strobel, T, Puskas, M, Bakos, A, Summers, D M, Will, R G, Budka, H Tags: PostScript Source Type: journals

Farmers, Ranchers Fighting Back Against FDA Tyranny Over Animal FarmsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(NaturalNews) A bill that would grant the FDA expanded authority to inspect farms has come under fire from ranchers and farmers concerned about increased government interference in their operations.In response to a recent series of food-borne illness outbreaks, a bill has been approved by the House Energy and Commerce Committee that would allocate more money and authority to the FDA to fulfill its current food safety duties. Although technically the bill would not expand the FDA's authority to foods currently supervised by the Department of Agriculture (USDA) , meat, poultry and some egg products , many farmers and their a...
Source: NaturalNews.com - November 16, 2009 Category: Consumer Health Advice Source Type: news

Identification of polymorphisms in the ovine Shadow of prion protein (SPRN) gene and assessment of their effect on promoter activity and susceptibility for classical scrapie.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Shadow of prion protein (SPRN) is an interesting candidate gene thought to be involved in prion pathogenesis. In humans, an association has already been discovered between mutations in SPRN and the incidence of variant and sporadic Creutzfeldt-Jakob disease. However, in sheep, the effect of mutations in SPRN is largely unknown. Therefore, we analysed the presence of mutations in the entire ovine SPRN gene, their association with scrapie susceptibility and their effect on SPRN promoter activity. In total, 26 mutations were found: seven in the promoter region, four in intron 1, seven in the coding sequence and eight in t...
Source: Animal Genetics - November 16, 2009 Category: Genetics & Stem Cells Authors: Lampo E, Duchateau L, Schepens B, Van Poucke M, Saelens X, Erkens T, Van Zeveren A, Peelman LJ Tags: Anim Genet Source Type: journals

2010 ASN AbstractsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: VBD can enlarge to significant dimensions, producing compressive symptoms in the brainstem, as well as thrombosis and ischemic strokes. SACE of the dolichoectasia can be effective for the vessel reconstruction, to prevent progression of the dilatation, and other potential clinical events. 3. Three Case Reports of Multiple Carotid and/or Vertebral Artery Dissections in Female Mei Lu, Heather Gornik, Esteban Cheng Ching, Vikram Kashyap, Rishi Gupta Cleveland Clinic Foundation, Cleveland, OH 44195, United States Introduction: Multiple carotid and/or vertebral artery dissections are rare. However, patients with mul...
Source: Journal of Neuroimaging - November 11, 2009 Category: Radiology Source Type: journals

Are Neurology Residents Prepared to Deal With Dying Patients? [Research Letters]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Archives of Neurology)
Source: Archives of Neurology - November 9, 2009 Category: Neurology Authors: Creutzfeldt, C. J., Gooley, T., Walker, M. Tags: Medical Practice, Medical Education, Neurology, Neurology, Other, Pain, Patient-Physician Relationship/ Care, End-of-life Care/ Palliative Medicine, Patient-Physician Relationship, Other Research Letters Source Type: journals

Vomiting due to intravenous levetiracetam in a case of Creutzfeldt-Jakob disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19906565 [PubMed - as supplied by publisher] (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - November 9, 2009 Category: Neurology Authors: Belcastro V, Mattucci E, Rossi A, Calabresi P, Tambasco N Tags: Epilepsy Behav Source Type: journals

What's really in that burger? E.coli and chicken feces both allowed by USDAEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(NaturalNews) There are 14 billion hamburgers consumed each year in the United States alone. The people who eat those burgers, though, have little knowledge of what's actually in them. Current USDA regulations, for example, openly allow beef contaminated with E. coli to be repackaged, cooked and sold as ready-to-eat hamburgers.This simple fact would shock most consumers if they knew about it. People assume that beef found to be contaminated with E. coli must be thrown out or destroyed (or even recalled), but in reality, it's often just pressed into hamburger patties, cooked, and sold to consumers. This practice is openly e...
Source: NaturalNews.com - November 6, 2009 Category: Consumer Health Advice Source Type: news

H1N1 swine flu infects commercial swine in USA, reports USDAEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(NaturalNews) The pork industry desperately wants you to believe "the Big Lie" about swine flu: That it can't infect pigs, and therefore it's perfectly safe to buy and eat lots and lots of pork products.It's a merry little tale, and it would be a nice little piece of information to pass along if only it were true.But it isn't.H1N1 swine flu can and does infect pigs. And the safety margin for eating pork products from H1N1-infected pigs is not well known.In fact, the USDA just confirmed H1N1 infections in commercial pigs (the kind used to make those pork chops you ate for breakfast). This is the first time that a commercial...
Source: NaturalNews.com - November 5, 2009 Category: Consumer Health Advice Source Type: news

Annual CJD surveillance report publishedEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Department of Health (DH) Area: News The seventeenth annual report of the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) has been published, looking back over the period from May 1990 (when the Unit was set up) to 31 December 2008. It outlines the Unit's work in the clinical surveillance of variant (vCJD), sporadic and iatrogenic CJD. Key figures on the incidence of CJD are as follows: . A total of 167 cases of definite or probable vCJD had been identified in the UK between 1995 and 31 December 2008 . Only one new probable or definite case of vCJD was reported in 2008...
Source: NeLM - News - November 3, 2009 Category: Drugs & Pharmacology Source Type: organizations

Cellular prion protein in blood platelets associates with both lipid rafts and the cytoskeleton.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The recently shown transmissibility of variant Creutzfeldt-Jakob disease (vCJD) by blood transfusion emphasises the need for better understanding of the cellular prion protein (PrPc) in blood. A substantial amount of cell-associated PrPc in blood resides in platelets. Platelet activation leads to up-regulation of PrPc on the platelet surface and its release on exosomes and microparticles. The sub-cellular localisation and function of platelet PrPc, however, is poorly understood. In the present study, we investigated the association of PrPc with platelet lipid rafts and the platelet cytoskeleton. Immuno-fluorescence mic...
Source: Thrombosis and Haemostasis - November 1, 2009 Category: Hematology Authors: Brouckova A, Holada K Tags: Thromb Haemost Source Type: journals

Organic transplant horrors: Diseased organs routinely implanted into donation recipientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(NaturalNews) Matthew Millington, 31, was an Iraq war veteran who served in the British army. Suffering from an unspecified "serious long condition", doctors told him he would be dead in two years unless he underwent a lung transplant. With tens of thousands of people world-wide awaiting organ transplants, the young man was one of the "lucky" patients who soon received his lungs from a donor. The problem was he was given lungs riddled with a fast growing cancer -- and Millington died less than 10 months after his operation. This is just a horrible, rare, mistake right? Not necessarily, according to a warning just issued by...
Source: NaturalNews.com - October 29, 2009 Category: Consumer Health Advice Source Type: news

Italy reports likely case of human form of mad cowEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
ROME (Reuters) - The Italian Health Ministry reported on Friday a "likely" case of Creutzfeldt-Jakob Disease, the human form of mad cow disease. (Source: Reuters: Health)
Source: Reuters: Health - October 23, 2009 Category: Consumer Health News Tags: healthNews Source Type: news

Italy reports likely case of human form of mad cowEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
ROME (Reuters) - The Italian Health Ministry reported Friday a "likely" case of Creutzfeldt-Jakob Disease, the human form of mad cow disease. (Source: Reuters: Health)
Source: Reuters: Health - October 23, 2009 Category: Consumer Health News Tags: healthNews Source Type: news

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Transmissible spongiform encephalopathies such as scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and bovine sporadic encephalopathy in cattle are characterized by the accumulation of a misfolded protein: the pathological prion protein. Ever since bovine sporadic encephalopathy was discovered as the likely cause of the new variant of CJD in humans, parallels between human and animal transmissible spongiform encephalopathies must be viewed under the aspect of a disease risk for humans. In our study we have compared prion characteristics of different forms of sheep scrapie with those of different phenotypes ...
Source: The American Journal of Pathology - October 21, 2009 Category: Pathology Authors: Wemheuer WM, Benestad SL, Wrede A, Schulze-Sturm U, Wemheuer WE, Hahmann U, Gawinecka J, Schütz E, Zerr I, Brenig B, Bratberg B, Andréoletti O, Schulz-Schaeffer WJ Tags: Am J Pathol Source Type: journals

Prion protein detection in serum using micromechanical resonator arrays.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, we have continued development of a micromechanical resonator array that is capable of detecting PrP(c) in bovine blood serum. The sensitivity of the resonators for the detection of PrP(c) is further enhanced by the use of secondary mass labels. A pair of antibodies is used in a sandwich immunoassay format to immobilize PrP(c) on the surface of resonators and attach nanoparticles as secondary mass labels to PrP(c). Secondary mass labeling is optimized in terms of incubation time to maximize the frequency shifts that correspond to the presence of PrP(c) on the surface of resonators. Our results show that a min...
Source: Talanta - October 21, 2009 Category: Chemistry Authors: Varshney M, Waggoner PS, Montagna RA, Craighead HG Tags: Talanta Source Type: journals

Involvement of Dab1 in APP processing and beta-amyloid deposition in sporadic Creutzfeldt-Jakob patients.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alzheimer's disease and prion pathologies (e.g., Creutzfeldt-Jakob disease (CJD)) display profound neural lesions associated with aberrant protein processing and extracellular amyloid deposits. Dab1 has been implicated in the regulation of Amyloid Precursor Protein (APP), but a direct link between human prion diseases and Dab1/APP interactions has not been published. Here we examined this putative relationship in seventeen cases of sporadic CJD (sCJD) post mortem. Biochemical analyses of brain tissue revealed two groups, which also correlated with PrP(sc) types 1 and 2. One group, with PrP(sc) type 1 showed increased D...
Source: Neurobiology of Disease - October 20, 2009 Category: Neurology Authors: Gavín R, Ferrer I, Del Río JA Tags: Neurobiol Dis Source Type: journals

ANA: No Benefit for Quinacrine in CJD (CME/CE)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
BALTIMORE (MedPage Today) -- The first U.S. treatment trial for sporadic Creutzfeldt-Jakob disease (CJD) has shown that quinacrine does not extend survival compared with placebo. (Source: MedPage Today Meeting Coverage)
Source: MedPage Today Meeting Coverage - October 15, 2009 Category: Journals (General) Source Type: info

Why Younger People Are More At Risk Of vCJDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Specific cells within the immune system could help explain why younger people are more susceptible to variant CJD, scientists believe. Patients diagnosed with variant CJD are, on average, 28 years old but it has been unclear why older people are not as affected by the disease. (Source: Biology / Biochemistry News From Medical News Today)
Source: Biology / Biochemistry News From Medical News Today - October 15, 2009 Category: Biochemistry Tags: CJD / vCJD / mad cow disease Source Type: news

Cell Study Explains Why Younger People More At Risk Of Variant Creutzfeldt-Jakob Disease (vCJD)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Specific cells within the immune system could help explain why younger people are more susceptible to variant Creutzfeldt-Jakob disease -- a rare, degenerative, fatal brain disorder. Patients diagnosed with variant CJD are, on average, 28 years old but it has been unclear why older people are not as affected by the disease. (Source: ScienceDaily Headlines)
Source: ScienceDaily Headlines - October 15, 2009 Category: Science Source Type: news

New research shows why young people are more prone to CJDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
CELLS in the immune system could hold the key to why younger people are more susceptible to the human form of mad cow disease, Scottish scientists said yesterday. (Source: Scotsman.com News - Health)
Source: Scotsman.com News - Health - October 15, 2009 Category: Consumer Health News Source Type: news

Breakthrough on vCJD risksEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
YOUNG people's immune systems put them at a higher risk of catching vCJD, an Edinburgh University study has found. (Source: Scotsman.com News - Health)
Source: Scotsman.com News - Health - October 15, 2009 Category: Consumer Health News Source Type: news

Increase in CD230 (cellular prion protein) fluorescence on blood lymphocytes in bovine spongiform encephalopathy-infected nonhuman primates.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
CONCLUSION: Blood lymphocytes from asymptomatically infected as well as diseased macaques were characterized by increased CD230 fluorescence, and phosphatidylinositol-phospholipase C-resistant PrP molecules contributed at least partially to this increase. Conformational changes within PrP(c) molecules may be the underlying mechanism for the increased PrP(c) fluorescence. This cell-associated phenomenon contributed at least partially to an increase in soluble plasma-derived PrP(c) levels. It is not yet known whether these changes reflect infectivity. PMID: 19843289 [PubMed - as supplied by publisher] (Source: Transfusion)
Source: Transfusion - October 14, 2009 Category: Hematology Authors: Holznagel E, Yutzy B, Schulz-Schaeffer W, Hanschman KM, Stuke A, Hahmann U, Törner M, Coulibaly C, Hoffmann A, Hunsmann G, Löwer J Tags: Transfusion Source Type: journals

Cell study explains why younger people more at risk of vCJDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(University of Edinburgh) Specific cells within the immune system could help explain why younger people are more susceptible to variant CJD, scientists believe.Patients diagnosed with variant CJD are, on average, 28 years old but it has been unclear why older people are not as affected by the disease. (Source: EurekAlert! - Medicine and Health)
Source: EurekAlert! - Medicine and Health - October 14, 2009 Category: Global & Universal Source Type: news

Transfusion-transmitted vCJD and its consequences for the Asia-Pacific regionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: ISBT Science Series)
Source: ISBT Science Series - October 13, 2009 Category: Hematology Authors: A. Keller Tags: Papers Source Type: journals

Management and prevention of human prion diseasesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract Prion diseases are a group of fatal neurologic disorders that affect humans and animals and for which there is no available therapy. The basic pathogenic mechanism is linked to posttranslational changes of the host cellular prion protein (PrPc) into a pathologic conformer (PrPTSE) that has a strong tendency to aggregate and form amyloid fibrils. In humans, the most common form of the disease is sporadic Creutzfeldt-Jakob disease (CJD), which equally affects females and males of all ages and all ethnic groups. Sporadic CJD has an overall mortality rate of approximately one to two cases per million peo...
Source: Current Neurology and Neuroscience Reports - October 13, 2009 Category: Neuroscience Tags: Current Neurology and Neuroscience Reports Source Type: journals

Genetic Association of a Cathepsin D Polymorphism and Sporadic Creutzfeldt-Jakob DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Dement Geriatr Cogn Disord 2009;28:302-306 (DOI:10.1159/000246343) (Source: Dementia and Geriatric Cognitive Disorders)
Source: Dementia and Geriatric Cognitive Disorders - October 12, 2009 Category: Geriatrics Source Type: journals

ORIGINAL CONTRIBUTION: Codistribution of Amyloid {beta} Plaques and Spongiform Degeneration in Familial Creutzfeldt-Jakob Disease With the E200K-129M HaplotypeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions To our knowledge, this is the first description of Aβ plaques in familial CJD with the E200K mutation. The codistribution of plaques and CJD-associated changes suggests that PrP plays a central role in Aβ formation and that Aβ pathology and prion disease likely in fluence each other. The kindred described herein provides support that PrPE200K may result in increased Aβ deposition. (Source: Archives of Neurology)
Source: Archives of Neurology - October 12, 2009 Category: Neurology Authors: Ghoshal, N., Cali, I., Perrin, R. J., Josephson, S. A., Sun, N., Gambetti, P., Morris, J. C. Tags: Neurology, Neurogenetics, Prion Diseases, Genetics, Genetic Disorders Original Contribution Source Type: journals

Codistribution of Amyloid {beta} Plaques and Spongiform Degeneration in Familial Creutzfeldt-Jakob Disease With the E200K-129M Haplotype [Original Contribution]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions To our knowledge, this is the first description of Aβ plaques in familial CJD with the E200K mutation. The codistribution of plaques and CJD-associated changes suggests that PrP plays a central role in Aβ formation and that Aβ pathology and prion disease likely in fluence each other. The kindred described herein provides support that PrPE200K may result in increased Aβ deposition. (Source: Archives of Neurology)
Source: Archives of Neurology - October 12, 2009 Category: Neurology Authors: Ghoshal, N., Cali, I., Perrin, R. J., Josephson, S. A., Sun, N., Gambetti, P., Morris, J. C. Tags: Neurology, Neurogenetics, Prion Diseases, Genetics, Genetic Disorders Original Contribution Source Type: journals

Kufs' disease: diagnostic difficulties in the examination of extracerebral biopsies.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report the case of a 45-year-old woman referred to the Neurological Department with suspicion of Creutzfeldt-Jakob disease (CJD). CJD as well as infectious, autoimmune and some lysosomal diseases were excluded. Since clinical symptoms, i.e. psychotic, auditory and visual hallucinations as well as behavioural disturbances, still suggested metabolic or neurodegenerative disease, a skin and muscle biopsy was performed. On ultrastructural examination the muscle biopsy revealed the subsarcolemmal accumulation of lipofuscin, lipofuscinlike and granular osmiophilic deposits (GRODs). The most unique fingerprint deposits (FP) an...
Source: Folia Neuropathologica - October 9, 2009 Category: Pathology Authors: Lewandowska E, Lipczyńska-Łojkowska W, Modzelewska J, Wierzba-Bobrowicz T, Mierzewska H, Szpak GM, Passenik E, Jachińska K Tags: Folia Neuropathol Source Type: journals

Collar and rest best for recent-onset cervical radiculopathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Neck disorder pain reduced more than with "wait-and-see" policyRelated items from OnMedicaMRI can predict brain activity Better treatment has reduced deaths from aneurysmsCelebrities back brain donation campaignFull impact of vCJD may have yet to hit Myasthenia gravis – an overview (Source: OnMedica Latest News)
Source: OnMedica Latest News - October 8, 2009 Category: UK Health Source Type: news

Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(Source: Brain)
Source: Brain - October 8, 2009 Category: Neurology Authors: Head, M. W., Ironside, J. W. Tags: Scientific Commentary Source Type: journals

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristicsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Five phenotypically distinct subtypes have been identified in sporadic Creutzfeldt–Jakob disease (sCJD), based on the methionine/valine polymorphic genotype of codon 129 of the prion protein (PrP) gene and the presence of either one of the two protease K-resistant scrapie prion protein (PrPSc) types identified as 1 and 2. The infrequent co-existence of both PrPSc types in the same case has been known for a long time. Recently, it has been reported, using type-specific antibodies, that the PrPSc type 1 is present in all cases of sCJD carrying PrPSc type 2. The consistent co-occurrence of both PrPSc types complicates t...
Source: Brain - October 8, 2009 Category: Neurology Authors: Cali, I., Castellani, R., Alshekhlee, A., Cohen, Y., Blevins, J., Yuan, J., Langeveld, J. P. M., Parchi, P., Safar, J. G., Zou, W.-Q., Gambetti, P. Tags: Original Articles Source Type: journals

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of...
Source: Brain - October 8, 2009 Category: Neurology Authors: Zerr, I., Kallenberg, K., Summers, D. M., Romero, C., Taratuto, A., Heinemann, U., Breithaupt, M., Varges, D., Meissner, B., Ladogana, A., Schuur, M., Haik, S., Collins, S. J., Jansen, G. H., Stokin, G. B., Pimentel, J., Hewer, E., Collie, D., Smith, P., Tags: Original Articles Source Type: journals

Thalamo-striatal diffusion reductions precede disease onset in prion mutation carriersEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Human prion diseases present substantial scientific and public health challenges. They are unique in being sporadic, infectious and inherited, and their pathogen is distinct from all other pathogens in lacking nucleic acids. Despite progress in understanding the molecular structure of prions, their initial cerebral pathophysiology and the loci of cerebral injury are poorly understood. As part of a large prospective study, we analysed early diffusion MRI scans of 14 patients with the E200K genetic form of Creutzfeldt–Jakob Disease, 20 healthy carriers of this mutation that causes the disease and 20 controls without th...
Source: Brain - October 8, 2009 Category: Neurology Authors: Lee, H., Rosenmann, H., Chapman, J., Kingsley, P. B., Hoffmann, C., Cohen, O. S., Kahana, E., Korczyn, A. D., Prohovnik, I. Tags: Original Articles Source Type: journals

Spatial Correlations between the Vacuolation, Prion Protein (PrP(sc)) Deposits and The Cerebral Blood Vessels In Sporadic Creutzfeldt-Jakob Disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of the present study was to determine whether the pathology was spatially related to blood vessels in cases of sporadic CJD (sCJD), a disease without an iatrogenic aetiology, and therefore less likely to be caused by haematogenous spread. Hence, the spatial correlations between the vacuolation ('spongiform change'), PrP(sc) deposits, and the blood vessels were studied in immunolabelled sections of the cerebral cortex and cerebellum in eleven cases of the common M/M1 subtype of sCJD. Both the vacuolation and the PrP(sc) deposits were spatially correlated with the blood vessels; the PrP(sc) deposits being more ...
Source: Current Neurovascular Research - October 6, 2009 Category: Neurology Authors: Armstrong RA Tags: Curr Neurovasc Res Source Type: journals

First Direct Information About The Prion's Molecular Structure ReportedEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A collaboration between scientists at Vanderbilt University and the University of California, San Francisco has led to the first direct information about the molecular structure of prions. In addition, the study has revealed surprisingly large structural differences between natural prions and the closest synthetic analogs that scientists have created in the lab. (Source: Health News from Medical News Today)
Source: Health News from Medical News Today - October 6, 2009 Category: Consumer Health News Tags: CJD / vCJD / mad cow disease Source Type: news

Less protease-resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphateEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a 68-year-old woman with sporadic Creutzfeldt[ndash]Jakob disease who received continuous intraventricular PPS infusion (1[ndash]120 [mu]g/kg/day) for 17 months starting 10 months after the onset of clinical symptoms. Treatment with PPS was well tolerated but was associated with a minor, transient intraventricular hemorrhage and a non-progressive collection of subdural fluid. The patient's overall survival time was well above the mean time expected for the illness but still within the normal range. Post-mortem examination revealed that the level of abnormal protease-resistant prion protein in the brain was marked...
Source: Acta Neurologica Scandinavica - October 4, 2009 Category: Neurology Authors: T. Terada, Y. Tsuboi, T. Obi, K. Doh-ura, S. Murayama, T. Kitamoto, T. Yamada, K. Mizoguchi Source Type: journals

Evolutionary Origins Of Prion Disease Gene UncoveredEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Scientists have uncovered the evolutionary ancestry of the prion gene, which may reveal new understandings of how the prion protein causes diseases such as bovine spongiform encephalopathy, also known as "mad cow disease." (Source: ScienceDaily Headlines)
Source: ScienceDaily Headlines - September 29, 2009 Category: Science Source Type: news