WorldWideScience

Sample records for bovine spongiform encephalopathy

  1. Bovine Spongiform Encephalopathy

    Science.gov (United States)

    Bovine spongiform encephalopathy (BSE), also referred to as “mad cow disease” is a chronic, non-febrile, neuro-degenerative disease affecting the central nervous system. The transmissible spongiform encephalopathies (TSEs) of domestic animals, of which BSE is a member includes scrapie of sheep...

  2. Frequently Asked Questions on BSE (Bovine Spongiform Encephalopathy or Mad Cow Disease)

    Science.gov (United States)

    ... BSE / FAQ on BSE (Bovine Spongiform Encephalopathy or Mad Cow Disease) Programs Beginning Farmer and Rancher Development Farm Storage ... Asked Questions on BSE (Bovine Spongiform Encephalopathy or Mad Cow Disease) Q. What is Bovine Spongiform Encephalopathy? A. Bovine ...

  3. 77 FR 29914 - Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products

    Science.gov (United States)

    2012-05-21

    ... RIN 0579-AC68 Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products AGENCY... live bovines and products derived from bovines with regard to bovine spongiform encephalopathy. This.... SUPPLEMENTARY INFORMATION: On March 16, 2012, we published in the Federal Register (77 FR 15848-15913, Docket...

  4. Bovine Spongiform Encephalopathy (BSE, Mad Cow Disease

    Directory of Open Access Journals (Sweden)

    G. K. Bruckner

    1997-07-01

    Full Text Available Mad Cow Disease or BSE (Bovine Spongiform Encephalopathy became a household name internationally and also in South Africa. International hysteria resulted following reports of a possible link between a disease diagnosed in cattle in Britain and a variant of the disease diagnosed in humans after the presumed ingestion or contact with meat from infected cattle. The European Union instituted a ban on the importation of beef from the United Kingdom during March 1996 that had a severe effect on the beef industry in the UK and also resulted in a world wide consumer resistance against beef consumption.

  5. Bovine Spongiform Encephalopathy (BSE), or Mad Cow Disease

    Science.gov (United States)

    ... Search The CDC Bovine Spongiform Encephalopathy (BSE), or Mad Cow Disease Note: Javascript is disabled or is not supported ... damages the central nervous system of cattle. More Mad Cow Disease is a neurological disorder of cattle. About BSE ...

  6. Prion biology relevant to bovine spongiform encephalopathy.

    Science.gov (United States)

    Novakofski, J; Brewer, M S; Mateus-Pinilla, N; Killefer, J; McCusker, R H

    2005-06-01

    Bovine spongiform encephalopathy (BSE) and chronic wasting disease (CWD) of deer and elk are a threat to agriculture and natural resources, as well as a human health concern. Both diseases are transmissible spongiform encephalopathies (TSE), or prion diseases, caused by autocatalytic conversion of endogenously encoded prion protein (PrP) to an abnormal, neurotoxic conformation designated PrPsc. Most mammalian species are susceptible to TSE, which, despite a range of species-linked names, is caused by a single highly conserved protein, with no apparent normal function. In the simplest sense, TSE transmission can occur because PrPsc is resistant to both endogenous and environmental proteinases, although many details remain unclear. Questions about the transmission of TSE are central to practical issues such as livestock testing, access to international livestock markets, and wildlife management strategies, as well as intangible issues such as consumer confidence in the safety of the meat supply. The majority of BSE cases seem to have been transmitted by feed containing meat and bone meal from infected animals. In the United Kingdom, there was a dramatic decrease in BSE cases after neural tissue and, later, all ruminant tissues were banned from ruminant feed. However, probably because of heightened awareness and widespread testing, there is growing evidence that new variants of BSE are arising "spontaneously," suggesting ongoing surveillance will continue to find infected animals. Interspecies transmission is inefficient and depends on exposure, sequence homology, TSE donor strain, genetic polymorphism of the host, and architecture of the visceral nerves if exposure is by an oral route. Considering the low probability of interspecies transmission, the low efficiency of oral transmission, and the low prion levels in nonnervous tissues, consumption of conventional animal products represents minimal risk. However, detection of rare events is challenging, and TSE

  7. Quantitative Risk Assessment of Bovine Spongiform Encephalopathy

    Science.gov (United States)

    Tsutsui, Toshiyuki; Kasuga, Fumiko

    Bovine spongiform encephalopathy (BSE) is a progressive neurological disease of cattle affecting the central nervous system and was first diagnosed in the United Kingdom (UK) in 1986 (Wells et al., 1987). This disease is one of the transmissible spongiform encephalopathy (TSE) which includes Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep. The causative agent of TSE is considered to be an abnormal form of prion protein. However, the details of its pathogenic mechanism have not been fully identified. Scrapie, which causes neurological symptoms in sheep and goats, has existed in the UK for 200 years (Hoinville, 1996) and spread across the rest of the world in the 1900s (Detwiler & Baylis, 2003). There has been no report so far that scrapie can be transmitted to humans. Initially, BSE was also considered as a disease affecting only animals. However, a variant type of Creutzfeldt-Jakob disease (vCJD) was first reported in the UK, and exposure to a BSE agent was suspected (Collinge, Sidle, Meads, Ironside, & Hill, 1996). vCJD is clinically and pathologically different from the sporadic type of CJD, and age at clinical onset of vCJD is younger than sporadic type (Will et al., 1996). Since the UK government announced the possible association between BSE and vCJD in 1996, BSE has become a huge public health concern all over the world. Of particular concern about vCJD, the fatal disease in younger age, distorted consumer confidence in beef safety, and as a result reduced beef consumption has been seen in many BSE-affected countries.

  8. 77 FR 20319 - Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products

    Science.gov (United States)

    2012-04-04

    ...; ] DEPARTMENT OF AGRICULTURE Animal and Plant Health Inspection Service 9 CFR Part 93 RIN 0579-AC68 Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Correction In proposed rule...

  9. 78 FR 73993 - Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products

    Science.gov (United States)

    2013-12-10

    ... Health Inspection Service 9 CFR Parts 92, 93, 94, 95, 96, and 98 RIN 0579-AC68 Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Corrections In rule document 2013-28228 appearing...

  10. Cattle traceability system in Japan for bovine spongiform encephalopathy

    OpenAIRE

    Katsuaki Sugiura; Takashi Onodera

    2008-01-01

    To promote consumer confidence in the safety of beef and to ensure the proper implementation of eradication measures against bovine spongiform encephalopathy (BSE), the Cattle Traceability Law was approved by the Diet in June 2003 and a cattle traceability system has been in operation in Japan since December 2003. The system enables tracing the cohort and offspring animals of a BSE case within 24 h of its detection. The traceability database system also provides distributors, restaurants and ...

  11. Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie.

    Science.gov (United States)

    Jacobs, J G; Sauer, M; van Keulen, L J M; Tang, Y; Bossers, A; Langeveld, J P M

    2011-01-01

    With increased awareness of the diversity of transmissible spongiform encephalopathy (TSE) strains in the ruminant population, comes an appreciation of the need for improved methods of differential diagnosis. Exposure to bovine spongiform encephalopathy (BSE) has been associated with the human TSE, variant Creutzfeldt-Jakob disease, emphasizing the necessity in distinguishing low-risk TSE types from BSE. TSE type discrimination in ruminants such as cattle, sheep, goats and deer, requires the application of several prion protein (PrP)-specific antibodies in parallel immunochemical tests on brain homogenates or tissue sections from infected animals. This study uses in a single incubation step, three PrP-specific antibodies and fluorescent Alexa dye-labelled anti-mouse Fabs on a Western blot. The usual amount of brain tissue needed is 0.5 mg. This multiplex application of antibodies directed towards three different PrP epitopes enabled differential diagnosis of all established main features of classical scrapie, BSE and Nor98-like scrapie in sheep and goats, as well as the currently known BSE types C, H and L in cattle. Moreover, due to an antibody-dependent dual PrP-banding pattern, for the first time CH1641 scrapie of sheep can be reliably discriminated from the other TSE isolate types in sheep. PMID:20943889

  12. A collaborative Canadian-United Kingdom evaluation of an immunohistochemistry protocol to diagnose bovine spongiform encephalopathy

    Science.gov (United States)

    Bovine spongiform encephalopathy is a transmissible spongiform encephalopathy of domestic cattle. The disorder was reported in the United Kingdom in the late 1980s and was associated with recycling of ruminant byproducts in cattle feed. In 1996, the bovine disease was reported to be the cause of a...

  13. Cattle traceability system in Japan for bovine spongiform encephalopathy

    Directory of Open Access Journals (Sweden)

    Katsuaki Sugiura

    2008-09-01

    Full Text Available To promote consumer confidence in the safety of beef and to ensure the proper implementation of eradication measures against bovine spongiform encephalopathy (BSE, the Cattle Traceability Law was approved by the Diet in June 2003 and a cattle traceability system has been in operation in Japan since December 2003. The system enables tracing the cohort and offspring animals of a BSE case within 24 h of its detection. The traceability database system also provides distributors, restaurants and consumers with information on the cattle from which the beef that they sell, serve and consume originate.

  14. Bovine Spongiform Encephalopathy: The Past Present and Future of Mad Cow Disease in the United States

    OpenAIRE

    Schlotthauer, Brent G.

    1998-01-01

    In an attempt to provide an introductory, yet thorough, discussion of Bovine Spongiform Encephalopathy and its ramifications in the United States, this paper shall: discuss the history of and explain the disease known as Bovine Spongiform Encephalopathy; explain Creutzfeldt-Jakob Disease; outline the history, responsibility and structure of the Food and Drug Administration Center for Veterinary Medicine; provide a comparative analysis of the steps that the United States and other countries ha...

  15. Transcriptional analysis implicates endoplasmic reticulum stress in bovine spongiform encephalopathy.

    Directory of Open Access Journals (Sweden)

    Yue Tang

    Full Text Available Bovine spongiform encephalopathy (BSE is a fatal, transmissible, neurodegenerative disease of cattle. To date, the disease process is still poorly understood. In this study, brain tissue samples from animals naturally infected with BSE were analysed to identify differentially regulated genes using Affymetrix GeneChip Bovine Genome Arrays. A total of 230 genes were shown to be differentially regulated and many of these genes encode proteins involved in immune response, apoptosis, cell adhesion, stress response and transcription. Seventeen genes are associated with the endoplasmic reticulum (ER and 10 of these 17 genes are involved in stress related responses including ER chaperones, Grp94 and Grp170. Western blotting analysis showed that another ER chaperone, Grp78, was up-regulated in BSE. Up-regulation of these three chaperones strongly suggests the presence of ER stress and the activation of the unfolded protein response (UPR in BSE. The occurrence of ER stress was also supported by changes in gene expression for cytosolic proteins, such as the chaperone pair of Hsp70 and DnaJ. Many genes associated with the ubiquitin-proteasome pathway and the autophagy-lysosome system were differentially regulated, indicating that both pathways might be activated in response to ER stress. A model is presented to explain the mechanisms of prion neurotoxicity using these ER stress related responses. Clustering analysis showed that the differently regulated genes found from the naturally infected BSE cases could be used to predict the infectious status of the samples experimentally infected with BSE from the previous study and vice versa. Proof-of-principle gene expression biomarkers were found to represent BSE using 10 genes with 94% sensitivity and 87% specificity.

  16. Review on prion diseases in animals with emphasis to Bovine Spongiform Encephalopathy

    Directory of Open Access Journals (Sweden)

    Rajender P. Gupta

    Full Text Available Prion diseases are known as Transmissible Spongiform Encephalopathies (TSE. These are degenerative brain disorders characterized by tiny microscopic holes that give the brain 'spongy' appearance. The causative agent is proteinaceous infective particle called prion. Prion diseases affect a variety of mammals including humans. The disease is transmitted by contaminated food or feed containing prion protein. In animals the diseases caused by prions are Scrapie, Bovine Spongiform Encephalopathy (BSE, Transmissible Mink Encephalopathy (TME, Chronic Wasting Disease (CWD, Feline Spongiform Encephalopathy (FSE and exotic Engulate Encephalopathy (EUE. Currently the only reliable test is histo-pathological examination of tissues. Control measures are surveillance, culling sick animals and banning specified risk materials. In India no case of BSE has been reported so far but the disease warrants constant monitoring and surveillance if once introduced or imported would be a herculean task to eradicate it. [Vet. World 2012; 5(7.000: 443-448

  17. Susceptibility of European red deer (Cervus elaphus elaphus to alimentary challenge with bovine spongiform encephalopathy.

    Directory of Open Access Journals (Sweden)

    Mark P Dagleish

    Full Text Available European red deer (Cervus elaphus elaphus are susceptible to the agent of bovine spongiform encephalopathy, one of the transmissible spongiform encephalopathies, when challenged intracerebrally but their susceptibility to alimentary challenge, the presumed natural route of transmission, is unknown. To determine this, eighteen deer were challenged via stomach tube with a large dose of the bovine spongiform encephalopathy agent and clinical signs, gross and histological lesions, presence and distribution of abnormal prion protein and the attack rate recorded. Only a single animal developed clinical disease, and this was acute with both neurological and respiratory signs, at 1726 days post challenge although there was significant (27.6% weight loss in the preceding 141 days. The clinically affected animal had histological lesions of vacuolation in the neuronal perikaryon and neuropil, typical of transmissible spongiform encephalopathies. Abnormal prion protein, the diagnostic marker of transmissible encephalopathies, was primarily restricted to the central and peripheral nervous systems although a very small amount was present in tingible body macrophages in the lymphoid patches of the caecum and colon. Serial protein misfolding cyclical amplification, an in vitro ultra-sensitive diagnostic technique, was positive for neurological tissue from the single clinically diseased deer. All other alimentary challenged deer failed to develop clinical disease and were negative for all other investigations. These findings show that transmission of bovine spongiform encephalopathy to European red deer via the alimentary route is possible but the transmission rate is low. Additionally, when deer carcases are subjected to the same regulations that ruminants in Europe with respect to the removal of specified offal from the human food chain, the zoonotic risk of bovine spongiform encephalopathy, the cause of variant Creutzfeldt-Jakob disease, from consumption of

  18. Bovine Spongiform Encephalopathy (BSE, Mad Cow Disease)

    OpenAIRE

    G.K. Bruckner

    1997-01-01

    Mad Cow Disease or BSE (Bovine Spongiform Encephalopathy) became a household name internationally and also in South Africa. International hysteria resulted following reports of a possible link between a disease diagnosed in cattle in Britain and a variant of the disease diagnosed in humans after the presumed ingestion or contact with meat from infected cattle. The European Union instituted a ban on the importation of beef from the United Kingdom during March 1996 that had a severe effect o...

  19. Cows for fear: is BSE a threat to human health? Bovine spongiform encephalopathy.

    OpenAIRE

    Josephson, J

    1998-01-01

    In 1996, a new variant of Creutzfeldt-Jakob disease (vCJD)-a disease that causes lack of coordination, muscle twitching or jerking, dementia, and, eventually, death-suddenly appeared in Great Britain. It is believed that the victims contracted the disease from eating the beef of cattle stricken with bovine spongiform encephalopathy (BSE), or mad cow disease. As of December 1997, at least 25 people in the United Kingdom and France have contracted vCJD.

  20. Modeling of Bovine Spongiform Encephalopathy in a Two-Species Feedback Loop

    OpenAIRE

    Barnes, Richard; Lehman, Clarence

    2015-01-01

    Bovine spongiform encephalopathy, otherwise known as mad cow disease, can spread when an individual cow consumes feed containing the infected tissues of another individual, forming a one-species feedback loop. Such feedback is the primary means of transmission for BSE during epidemic conditions. Following outbreaks in the European Union and elsewhere, many governments enacted legislation designed to limit the spread of such diseases via elimination or reduction of one-species feedback loops i...

  1. Transmissible Spongiform Encephalopathies (Prion Diseases)

    Science.gov (United States)

    ... called bovine spongiform encephalopathy (BSE), also known as "mad cow disease." Other TSEs found in animals include scrapie, which ... and Worldwide NINDS Clinical Trials Organizations Column1 Column2 Creutzfeldt-Jakob Disease (CJD) Foundation Inc. 341 W. 38th Street, Suite ...

  2. 9 CFR 96.2 - Prohibition of casings due to African swine fever and bovine spongiform encephalopathy.

    Science.gov (United States)

    2010-01-01

    ... Drug Administration at 21 CFR 589.2000 may be imported. (2) Casings that are derived from bovines that... swine fever and bovine spongiform encephalopathy. 96.2 Section 96.2 Animals and Animal Products ANIMAL... ENTRY INTO THE UNITED STATES § 96.2 Prohibition of casings due to African swine fever and...

  3. Bovine spongiform encephalopathy in Japan: consumers’ food safety perceptions and willingness to pay for tested beef

    OpenAIRE

    McCluskey, Jill J.; Kristine M. Grimsrud; Ouchi, Hiromi; Wahl, Thomas I.

    2005-01-01

    The discovery of bovine spongiform encephalopathy (BSE), commonly known as ‘mad cow disease’, in Japan caused anxiety about consuming beef and beef products. As a result, there was a sudden fall in sales of beef that hurt the Japanese beef industry as well as major beef exporters to Japan. We analyse factors that affect Japanese consumers’ willingness to pay (WTP) price premiums for BSE-tested beef and estimate the mean WTP for BSE-tested beef using data obtained from a consumer survey in Jap...

  4. Monitoring and analysis of bovine spongiform encephalopathy (BSE) testing in Denmark using statistical models

    DEFF Research Database (Denmark)

    Paisley, Larry

    2002-01-01

    The evolution of monitoring and surveillance for bovine spongiform encephalopathy (BSE) from the phase of passive surveillance that began in the United Kingdom in 1988 until the present is described. Currently, surveillance for BSE in Europe consists of mass testing of cattle slaughtered for human....... The analyses were intended to provide information for decision-makers, the media and the public as well as to provide inputs for future BSE surveillance models. The results to date suggest that the total number of BSE cases that will be found in Denmark in 2001 will not exceed 16....

  5. 76 FR 35185 - Notice of Request for Extension of Approval of an Information Collection; Bovine Spongiform...

    Science.gov (United States)

    2011-06-16

    ... Collection; Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products AGENCY: Animal and... byproducts to protect against the introduction of bovine spongiform encephalopathy into the United States... animal products and byproducts to prevent the introduction of bovine spongiform encephalopathy into...

  6. Central nervous system gene expression changes in a transgenic mouse model for bovine spongiform encephalopathy

    Directory of Open Access Journals (Sweden)

    Tortosa Raül

    2011-10-01

    Full Text Available Abstract Gene expression analysis has proven to be a very useful tool to gain knowledge of the factors involved in the pathogenesis of diseases, particularly in the initial or preclinical stages. With the aim of finding new data on the events occurring in the Central Nervous System in animals affected with Bovine Spongiform Encephalopathy, a comprehensive genome wide gene expression study was conducted at different time points of the disease on mice genetically modified to model the bovine species brain in terms of cellular prion protein. An accurate analysis of the information generated by microarray technique was the key point to assess the biological relevance of the data obtained in terms of Transmissible Spongiform Encephalopathy pathogenesis. Validation of the microarray technique was achieved by RT-PCR confirming the RNA change and immunohistochemistry techniques that verified that expression changes were translated into variable levels of protein for selected genes. Our study reveals changes in the expression of genes, some of them not previously associated with prion diseases, at early stages of the disease previous to the detection of the pathological prion protein, that might have a role in neuronal degeneration and several transcriptional changes showing an important imbalance in the Central Nervous System homeostasis in advanced stages of the disease. Genes whose expression is altered at early stages of the disease should be considered as possible therapeutic targets and potential disease markers in preclinical diagnostic tool development. Genes non-previously related to prion diseases should be taken into consideration for further investigations.

  7. Control of bovine spongiform encephalopathy by genetic engineering: possible approaches and regulatory considerations

    International Nuclear Information System (INIS)

    Transmissible spongiform encephalopathies (TSE) include bovine spongiform encephalopathy (BSE), scrapie in sheep and Creutzfeldt-Jakob disease (CJD) in humans. A new CJD variant (nvCJD) is believed to be related to consumption of meat from BSE cattle. In TSE individuals, prion proteins (PrP) with approximately 250 amino acids convert to the pathogenic prion PrPSc, leading to a dysfunction of the central neural system. Research elsewhere with mice has indicated a possible genetic engineering approach to the introduction of BSE resistance: individuals with amino acid substitutions at positions 167 or 218, inoculated with a pathogenic prion protein, did not support PrPSc replication. This raises the possibility of producing prion-resistant cattle with a single PrP amino acid substitution. Since prion-resistant animals might still harbour acquired prion infectivity, regulatory assessment of the engineered animals would need to ascertain that such possible 'carriers' do not result in a threat to animal and human health. (author)

  8. Bovine Spongiform Encephalopathy (BSE – Infectious, Contagious, Zoonotic or Production Disease?

    Directory of Open Access Journals (Sweden)

    Doherr Marcus G

    2003-03-01

    Full Text Available In 1986, a new progressive neurological condition similar to scrapie of sheep and goats was recognised in cattle in the United Kingdom (UK, and was named bovine spongiform encephalopathy (BSE. There is an ongoing discussion whether BSE should be classified as infectious, contagious, or zoonotic, and if it fits the definition of a production disease. The objective of this work is to briefly describe the main characteristics of transmissible spongiform encephalopathies (TSE, to review the epidemiology of BSE, and to address the question of how to classify BSE. TSEs are characterised as chronic wasting diseases with spongiform vacuolation and the accumulation of infectious prion protein (PrPSc in the central nervous system. TSE infectivity is very difficult to inactivate. Cattle BSE most likely originated from sheep scrapie, although this will remain to be an issue for debate. The disease can be transmitted from cattle to a range of species, and has resulted in smaller TSE epidemics in domestic cats, zoo cats and zoo ruminants, and in humans. Transmission in the field occurred through feed containing ruminant-derived protein, and measures to prevent the recycling of infectivity have proven effective to reduce the number of new infections. Mandatory reporting of clinical suspects combined with targeted screening of risk populations is needed to assess the BSE status of a country. Infection studies and the transmissibility to other species classify BSE as infectious and zoonotic. Absence of excretion of the agent, and therefor of horizontal transmission, categorise BSE as non-contagious. However, BSE is a multifactorial infectious disease that is dependent on management factors (mainly feeding, and therefore fits into the broader definition of production diseases.

  9. Reflexive modernization at the source: local media coverage of bovine spongiform encephalopathy in rural Alberta.

    Science.gov (United States)

    Davidson, Debra J; Bogdan, Eva

    2010-11-01

    The potential for reflexive modernization is defined by multiple factors, but the acknowledgment of risk is crucial, particularly among social groups that play a key role in risk minimization. This study offers an examination of the role of local media in response to the outbreak of bovine spongiform encephalopathy (BSE) in beef-producing communities in rural Alberta. BSE is one of several global risk issues that reflexive modernization theorists argue have the potential to trigger a transformation toward a critically reflexive society in which such risks are minimized. Content analysis of newspapers in beef-producing regions in Alberta, however, shows how local media framed BSE in a manner that maximized community cohesion and protection of local culture. This selective coverage of BSE in rural Alberta is quite likely to have contributed to, or at least reinforced, support for the current institutional structure of Canadian agriculture in beef-producing regions, through the constriction of discourse. PMID:21553633

  10. Animal feed controls implemented in Japan for the eradication of bovine spongiform encephalopathy

    Directory of Open Access Journals (Sweden)

    Katsuaki Sugiura

    2009-06-01

    Full Text Available After the detection of the first case of bovine spongiform encephalopathy (BSE in Japan in September 2001, the Japanese government introduced a series of animal feed control measures to reduce the risk of the spread of the disease from a feed source. To ensure the proper implementation of these measures, the Food and Agricultural Materials Inspection Centre conducted audit inspections of feed importers, producers, distributors and end-users. The audit inspections include on-site inspection of the feed plants, warehouses, farms and other related premises and the laboratory analysis of feed samples taken from these premises to check for the presence of animal protein. The results of inspections conducted in recent years indicate good compliance with the feed control measures.

  11. Control methods for cattle feedstuffs aimed at prevention of Bovine spongiform encephalopathy (BSE

    Directory of Open Access Journals (Sweden)

    Nešić Ksenija

    2006-01-01

    Full Text Available In the course of the last decades of the twentieth century, more than 30 new diseases were determined for the first time in history. Bovine spongiform encephalopathy (BSE, or "mad cow disease" is one of them. The disease implies the subacute neurodegenerative transmission of spongiform encephalopathy and it was diagnosed and described for the first time in Great Britain in 1986. A theory has been established that BSE is spread through feedstuffs, more precisely, meat-bone flour which contains infective proteins of ruminants, and legislature has been passed throughout the world with the objective of preventing the entry of meat-bone flour into the food chain. The complete ban of the use of meat-bone flour for all farm animals (with the exception of fish flour for non-ruminants and an adequate thermal treatment in the production of meat-bone flour (133ºC, 3 bar, 20 min are the elements on which the European Union (EU legislature is based. The regulations in our country include a ban on the use of meat-bone flour in cattle feedstuffs and a ban on imports of beef proteins. The implementation of this legislature throughout the world requires the corresponding analytical means. At the present time, there are several available possibilities: optic microscopy, PCR, immunoprobes, spectroscopic methods, and several others which are still being examined for use for this purpose. All the analytical methods are being applied with the objective of controlling the implementation of the current regulations, but also in order to discover possible cross contamination that could take place in factories of animal feedstuffs, during transportation, storage, or on farms, in particular when there are no separate lines for feedstuffs that contains meat-bone flour and others in which even its traces are banned. In order to secure the successful control and prevention of bovine spongiform encephalopathy in our country, as well as to secure the unhindered continuation of

  12. Experimental transmission of bovine spongiform encephalopathy (BSE) to cynomolgus macaques, a non-human primate.

    Science.gov (United States)

    Ono, Fumiko; Terao, Keiji; Tase, Naomi; Hiyaoka, Akio; Ohyama, Atsushi; Tezuka, Yukio; Wada, Naomi; Kurosawa, Asuka; Sato, Yuko; Tobiume, Minoru; Hagiwara, Ken'ichi; Yamakawa, Yoshio; Sata, Tetsutaro

    2011-01-01

    Bovine spongiform encephalopathy (BSE) was transmitted to three macaques by intracerebral inoculation of a brain homogenate from affected cattle detected in Japan. All monkeys developed abnormal behavioral signs, such as intermittent anorexia and hyperekplexia, around 24 months after inoculation. Neuronal symptoms, such as tremor, myoclonic jerking, and paralysis, appeared 27-44 months after inoculation. These symptoms worsened and total paralysis ensued within a year after onset. The disease duration was approximately 8-12 months. Both the incubation period and the duration of disease were shortened in the secondary transmission experiment to macaques. Heavy accumulation of disease-causing conformer(s) of prion protein (PrP(Sc)), with a similar glycoform profile to the PrP(Sc) contained in the inoculum, and severe spongiform changes in the histology of the brain, confirmed the successful transmission of BSE to monkeys. Florid plaques, a characteristic histological hallmark of variant Creutzfeldt-Jakob disease, were prominent in the cerebral cortex, in which a prion antigen was detected by immunohistochemistry (IHC). PrP(Sc) was mostly confined to the central nervous system, although small amounts of PrP(Sc) accumulated in the peripheral nerves of monkeys, as detected by Western blotting (WB). Neither IHC nor WB detected PrP(Sc) in the lymphatic organs/tissues, such as the tonsils, spleen, and appendix. PMID:21266755

  13. Emergence of a novel bovine spongiform encephalopathy (BSE) prion from an atypical H-type BSE.

    Science.gov (United States)

    Masujin, Kentaro; Okada, Hiroyuki; Miyazawa, Kohtaro; Matsuura, Yuichi; Imamura, Morikazu; Iwamaru, Yoshifumi; Murayama, Yuichi; Yokoyama, Takashi

    2016-01-01

    The H-type of atypical bovine spongiform encephalopathy (H-BSE) was serially passaged in bovinized transgenic (TgBoPrP) mice. At the fourth passage, most challenged mice showed a typical H-BSE phenotype with incubation periods of 223 ± 7.8 days. However, a different phenotype of BSE prion with shorter incubation periods of 109 ± 4 days emerged in a minor subset of the inoculated mice. The latter showed distinct clinical signs, brain pathology, and abnormal prion protein profiles as compared to H-BSE and other known BSE strains in mice. This novel prion was transmitted intracerebrally to cattle, with incubation periods of 14.8 ± 1.5 months, with phenotypes that differed from those of other bovine prion strains. These data suggest that intraspecies transmission of H-BSE in cattle allows the emergence of a novel BSE strain. Therefore, the continuation of feed ban programs may be necessary to exclude the recycling of H-BSE prions, which appear to arise spontaneously, in livestock. Such measures should help to reduce the risks from both novel and known strains of BSE. PMID:26948374

  14. Microarray analysis in caudal medulla of cattle orally challenged with bovine spongiform encephalopathy.

    Science.gov (United States)

    Almeida, L M; Basu, U; Williams, J L; Moore, S S; Guan, L L

    2011-01-01

    Bovine spongiform encephalopathy (BSE) is a fatal disorder in cattle characterized by progressive neurodegeneration of the central nervous system. We investigated the molecular mechanisms involved in neurodegeneration during prion infection through the identification of genes that are differentially expressed (DE) between experimentally infected and non-challenged cattle. Gene expression of caudal medulla from control and orally infected animals was compared by microarray analysis using 24,000 bovine oligonucleotides representing 16,846 different genes to identify DE genes associated with BSE disease. In total, 182 DE genes were identified between normal and BSE-infected tissues (>2.0-fold change, P apoptosis, and cytoskeleton organization; 13 of these genes were found to be involved in 26 different Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways. The expression of five DE genes associated with synapse function (tachykinin, synuclein, neuropeptide Y, cocaine, amphetamine-responsive transcript, and synaptosomal-associated protein 25 kDa) and three DE genes associated with calcium ion regulation (parvalbumin, visinin-like, and cadherin) was further validated in the medulla tissue of cattle at different infection times (6, 12, 42, and 45 months post-infection) by qRT-PCR. These data will contribute to a better understanding of the molecular mechanisms of neuropathology in bovine species. PMID:22033911

  15. Emergence of a novel bovine spongiform encephalopathy (BSE) prion from an atypical H-type BSE

    Science.gov (United States)

    Masujin, Kentaro; Okada, Hiroyuki; Miyazawa, Kohtaro; Matsuura, Yuichi; Imamura, Morikazu; Iwamaru, Yoshifumi; Murayama, Yuichi; Yokoyama, Takashi

    2016-01-01

    The H-type of atypical bovine spongiform encephalopathy (H-BSE) was serially passaged in bovinized transgenic (TgBoPrP) mice. At the fourth passage, most challenged mice showed a typical H-BSE phenotype with incubation periods of 223 ± 7.8 days. However, a different phenotype of BSE prion with shorter incubation periods of 109 ± 4 days emerged in a minor subset of the inoculated mice. The latter showed distinct clinical signs, brain pathology, and abnormal prion protein profiles as compared to H-BSE and other known BSE strains in mice. This novel prion was transmitted intracerebrally to cattle, with incubation periods of 14.8 ± 1.5 months, with phenotypes that differed from those of other bovine prion strains. These data suggest that intraspecies transmission of H-BSE in cattle allows the emergence of a novel BSE strain. Therefore, the continuation of feed ban programs may be necessary to exclude the recycling of H-BSE prions, which appear to arise spontaneously, in livestock. Such measures should help to reduce the risks from both novel and known strains of BSE. PMID:26948374

  16. Experimental transmission of bovine spongiform encephalopathy to European red deer (Cervus elaphus elaphus

    Directory of Open Access Journals (Sweden)

    Reid Hugh W

    2008-05-01

    Full Text Available Abstract Background Bovine spongiform encephalopathy (BSE, a member of the transmissible spongiform encephalopathies (TSE, primarily affects cattle. Transmission is via concentrate feed rations contaminated with infected meat and bone meal (MBM. In addition to cattle, other food animal species are susceptible to BSE and also pose a potential threat to human health as consumption of infected meat products is the cause of variant Creutzfeldt-Jakob disease in humans, which is invariably fatal. In the UK, farmed and free ranging deer were almost certainly exposed to BSE infected MBM in proprietary feeds prior to legislation banning its inclusion. Therefore, although BSE has never been diagnosed in any deer species, a possible risk to human health remains via ingestion of cervine products. Chronic wasting disease (CWD, also a TSE, naturally infects several cervid species in North America and is spreading rapidly in both captive and free-ranging populations. Results Here we show that European red deer (Cervus elaphus elaphus are susceptible to intra-cerebral (i/c challenge with BSE positive cattle brain pool material resulting in clinical neurological disease and weight loss by 794–1290 days and the clinical signs are indistinguishable to those reported in deer with CWD. Spongiform changes typical of TSE infections were present in brain and accumulation of the disease-associated abnormal prion protein (PrPd was present in the central and peripheral nervous systems, but not in lymphoid or other tissues. Western immunoblot analysis of brain material showed a similar glycosylation pattern to that of BSE derived from infected cattle and experimentally infected sheep with respect to protease-resistant PrP isoforms. However, the di-, mono- and unglycosylated bands migrated significantly (p Conclusion This study shows that deer are susceptible to BSE by intra-cerebral inoculation and display clinical signs and vacuolar pathology that are similar to those

  17. Pathogenesis of experimental bovine spongiform encephalopathy (BSE): estimation of tissue infectivity according to incubation period§

    OpenAIRE

    Arnold, Mark Edward; Hawkins, Stephen Anthony Charles; Green, Robert; Dexter, Ian; Wells, Gerald Arthur Henry

    2009-01-01

    International audience This paper reports the results of tissue infectivity assays of bovine spongiform encephalopathy (BSE) agent in orally exposed cattle at stages during the incubation period. Estimations of the titre of infectivity in central nervous system (CNS), certain peripheral nerve ganglia and distal ileum tissue were made according to time post exposure from the relationship between incubation period and dose for RIII mice and C57bl mice using data from titrations of brain mate...

  18. Effects of wind farms and food scarcity on a large scavenging bird species following an epidemic of bovine spongiform encephalopathy

    OpenAIRE

    Martínez-Abraín, Alejandro; Tavecchia, Giacomo; Regan, H. M.; Jiménez, Juan (O.F.M.), trad; Surroca, M.; Oro, Daniel

    2012-01-01

    Wind farms are emerging as a major cause of mortality of large scavenging bird species, which may be catastrophic when they operate in concert with other threats. As a study model, we examine the impact of wind turbines on the population dynamics of a soaring bird species, when acting in conjunction with a sudden decrease in food availability following the European bovine spongiform encephalopathy (BSE) epidemic. In Spain, vultures have been provided with supplementary food at traditional vul...

  19. 78 FR 72979 - Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products

    Science.gov (United States)

    2013-12-04

    ... risks of other livestock diseases, such as bovine viral diarrhea, foot-and-mouth disease, infectious... Products Derived from Bovines,'' published in the Federal Register on September 18, 2007 (72 FR 53314-53379..., 2012, we published in the Federal Register (77 FR 15848-15913, Docket No. APHIS-2008-0010) a...

  20. A stochastic model of the bovine spongiform encephalopathy epidemic in Canada.

    Science.gov (United States)

    Oraby, Tamer; Al-Zoughool, Mustafa; Elsaadany, Susie; Krewski, Daniel

    2016-01-01

    Bovine spongiform encephalopathy (BSE) appeared in the United Kingdom in the mid 1980s, and has been attributed to the use of meat and bone meal (MBM) in cattle feed contaminated with a scrapie-like agent. Import of infectious materials from a country where BSE has occurred is believed to be the major factor underlying the spread of the BSE epidemic to other countries. This study presents a new stochastic model developed to estimate risk of BSE from importation of cattle infected with the BSE agent. The model describes the propagation of the BSE agent through the Canadian cattle herd through rendering and feeding processes, following importation of cattle with infectious prions. This model was used estimate the annual number of newly infected animals each year over the period 1980-2019. Model predictions suggested that the number of BSE infections in Canada might have been approximately 40-fold greater than the actual number of clinically diagnosed cases. Under complete compliance with the 2007 ban on feeding MBM, this model further predicts that BSE is disappearing from the Canadian cattle system. A series of sensitivity analyses was also conducted to test the robustness of model predictions to alternative assumptions about factors affecting the evolution of the Canadian BSE epidemic. PMID:27556562

  1. The Canadian Management of Bovine Spongiform Encephalopathy in Historical and Scientific Perspective, 1990-2014.

    Science.gov (United States)

    Quimby, Alexandra E; Shamy, Michel C F

    2015-11-01

    On February 11, 2015, the Canadian Food Inspection Agency announced that a cow born and raised in Alberta had tested positive for bovine spongiform encephalopathy (BSE), commonly known as mad cow disease. BSE is a prion disease of cattle that, when transmitted to humans, produces a fatal neurodegenerative disease known as variant Creutzfeldt-Jakob disease. We believe that this latest case of BSE in Canadian cattle suggests the timeliness of a review of the management of BSE in Canada from a historically and scientifically informed perspective. In this article, we ask: how did the Canadian management of BSE between 1990 and 2014 engage with the contemporary understanding of BSE's human health implications? We propose that Canadian policies largely ignored the implicit medical nature of BSE, treating it as a purely agricultural and veterinary issue. In this way, policies to protect Canadians were often delayed and incomplete, in a manner disturbingly reminiscent of Britain's failed management of BSE. Despite assurances to the contrary, it is premature to conclude that BSE (and with it the risk of variant Creutzfeldt-Jakob disease) is a thing of Canada's past: BSE remains very much an issue in Canada's present. PMID:26357946

  2. Impacts of bovine spongiform encephalopathy and avian inlfuenza on U.S. meat demand

    Institute of Scientific and Technical Information of China (English)

    Jianhong E Mu; Bruce A McCarl; Amy Hagerman; David Bessler

    2015-01-01

    This paper examines the U.S. meat demand impacts of the announced outbreaks of bovine spongiform encephalopathy (BSE) and avian inlfuenza (AI). Findings indicate that beef and chicken demand was negatively affected by BSE and AI disease outbreaks. Speciifcal y, in the short run, U.S. consumers shift demand due to both outbreaks but more so due to domestic disease outbreaks than for outbreaks occurring overseas-the impact of U.S. AI outbreaks is about 0.5%for beef and the impact of U.S. BSE cases is around–0.42%for beef and 0.4%for pork, respectively. Regarding the BSE shock on meat demand, there is a high rate of beef demand adjusted from disturbance to the long-run equilibrium and a lower adjustment rate for chicken demand because of the repeated outbreaks of AI worldwide. In the long run, information related to severe, persistently recurring overseas animal disease outbreaks changes U.S. consumers’ meat consumption patterns. Although effects of animal diseases on U.S. meat demand were statistical y signiifcant, the magnitudes were smal-the impact of WHO reported human death numbers for AI is 0.005%for beef,–0.002%for pork, and–0.006%for chicken and the impact of U.S. BSE cases is 1.1%for pork and–0.7%for chicken.

  3. Surveillance and simulation of bovine spongiform encephalopathy and scrapie in small ruminants in Switzerland

    Directory of Open Access Journals (Sweden)

    Zurbriggen Andreas

    2010-04-01

    Full Text Available Abstract Background After bovine spongiform encephalopathy (BSE emerged in European cattle livestock in 1986 a fundamental question was whether the agent established also in the small ruminants' population. In Switzerland transmissible spongiform encephalopathies (TSEs in small ruminants have been monitored since 1990. While in the most recent TSE cases a BSE infection could be excluded, for historical cases techniques to discriminate scrapie from BSE had not been available at the time of diagnosis and thus their status remained unclear. We herein applied state-of-the-art techniques to retrospectively classify these animals and to re-analyze the affected flocks for secondary cases. These results were the basis for models, simulating the course of TSEs over a period of 70 years. The aim was to come to a statistically based overall assessment of the TSE situation in the domestic small ruminant population in Switzerland. Results In sum 16 TSE cases were identified in small ruminants in Switzerland since 1981, of which eight were atypical and six were classical scrapie. In two animals retrospective analysis did not allow any further classification due to the lack of appropriate tissue samples. We found no evidence for an infection with the BSE agent in the cases under investigation. In none of the affected flocks, secondary cases were identified. A Bayesian prevalence calculation resulted in most likely estimates of one case of BSE, five cases of classical scrapie and 21 cases of atypical scrapie per 100'000 small ruminants. According to our models none of the TSEs is considered to cause a broader epidemic in Switzerland. In a closed population, they are rather expected to fade out in the next decades or, in case of a sporadic origin, may remain at a very low level. Conclusions In summary, these data indicate that despite a significant epidemic of BSE in cattle, there is no evidence that BSE established in the small ruminant population in

  4. 77 FR 15847 - Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products

    Science.gov (United States)

    2012-03-16

    ... been fed ruminant protein, other than milk protein, during their lifetime; The bovines from which the... from animals that are not known to have been fed ruminant protein, other than milk protein, during... March 16, 2012 Part II Department of Agriculture Animal and Plant Health Inspection Service 9 CFR...

  5. Survey of laboratory findings in suspected cases of bovine spongiform encephalopathy in Denmark from 1990 to 2000

    DEFF Research Database (Denmark)

    Agerholm, J.S.; Tegtmeier, C.L.; Nielsen, T.K.

    2002-01-01

    A survey of the laboratory findings in suspected cases of bovine spongiform encephalopathy (BSE) in Denmark from I June 1990 to '31 December 2000 is presented. During this period BSE was a notifiable disease, and the heads of suspected cases were submitted according to the legislation on BSE. A...... total of 176 submissions were made, mostly from bovines with neurological disorders and mainly during the last 3 years of this period. Lesions or other laboratory findings consistent with severe neurological disorders were found in 115 cases. The most frequent diagnosis was encephalic 41 p listeriosis...

  6. Infectivity in skeletal muscle of cattle with atypical bovine spongiform encephalopathy.

    Science.gov (United States)

    Suardi, Silvia; Vimercati, Chiara; Casalone, Cristina; Gelmetti, Daniela; Corona, Cristiano; Iulini, Barbara; Mazza, Maria; Lombardi, Guerino; Moda, Fabio; Ruggerone, Margherita; Campagnani, Ilaria; Piccoli, Elena; Catania, Marcella; Groschup, Martin H; Balkema-Buschmann, Anne; Caramelli, Maria; Monaco, Salvatore; Zanusso, Gianluigi; Tagliavini, Fabrizio

    2012-01-01

    The amyloidotic form of bovine spongiform encephalopathy (BSE) termed BASE is caused by a prion strain whose biological properties differ from those of typical BSE, resulting in a clinically and pathologically distinct phenotype. Whether peripheral tissues of BASE-affected cattle contain infectivity is unknown. This is a critical issue since the BASE prion is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible. We carried out bioassays in transgenic mice overexpressing bovine PrP (Tgbov XV) and found infectivity in a variety of skeletal muscles from cattle with natural and experimental BASE. Noteworthy, all BASE muscles used for inoculation transmitted disease, although the attack rate differed between experimental and natural cases (∼70% versus ∼10%, respectively). This difference was likely related to different prion titers, possibly due to different stages of disease in the two conditions, i.e. terminal stage in experimental BASE and pre-symptomatic stage in natural BASE. The neuropathological phenotype and PrP(res) type were consistent in all affected mice and matched those of Tgbov XV mice infected with brain homogenate from natural BASE. The immunohistochemical analysis of skeletal muscles from cattle with natural and experimental BASE showed the presence of abnormal prion protein deposits within muscle fibers. Conversely, Tgbov XV mice challenged with lymphoid tissue and kidney from natural and experimental BASE did not develop disease. The novel information on the neuromuscular tropism of the BASE strain, efficiently overcoming species barriers, underlines the relevance of maintaining an active surveillance. PMID:22363650

  7. Studies of the transmissibility of the agent of bovine spongiform encephalopathy to the domestic chicken

    Directory of Open Access Journals (Sweden)

    Moore Jo

    2011-11-01

    Full Text Available Abstract Background Transmission of the prion disease bovine spongiform encephalopathy (BSE occurred accidentally to cattle and several other mammalian species via feed supplemented with meat and bone meal contaminated with infected bovine tissue. Prior to United Kingdom controls in 1996 on the feeding of mammalian meat and bone meal to farmed animals, the domestic chicken was potentially exposed to feed contaminated with the causal agent of BSE. Although confirmed prion diseases are unrecorded in avian species a study was undertaken to transmit BSE to the domestic chicken by parenteral and oral inoculations. Transmissibility was assessed by clinical monitoring, histopathological examinations, detection of a putative disease form of an avian prion protein (PrP in recipient tissues and by mouse bioassay of tissues. Occurrence of a progressive neurological syndrome in the primary transmission study was investigated by sub-passage experiments. Results No clinical, pathological or bioassay evidence of transmission of BSE to the chicken was obtained in the primary or sub-passage experiments. Survival data showed no significant differences between control and treatment groups. Neurological signs observed, not previously described in the domestic chicken, were not associated with significant pathology. The diagnostic techniques applied failed to detect a disease associated form of PrP. Conclusion Important from a risk assessment perspective, the present study has established that the domestic chicken does not develop a prion disease after large parenteral exposures to the BSE agent or after oral exposures equivalent to previous exposures via commercial diets. Future investigations into the potential susceptibility of avian species to mammalian prion diseases require species-specific immunochemical techniques and more refined experimental models.

  8. Effect of autolysis on the specificity of bovine spongiform encephalopathy rapid tests

    Directory of Open Access Journals (Sweden)

    Caramelli Maria

    2010-07-01

    Full Text Available Abstract Background Routine rapid testing for Bovine Spongiform Encephalopathy (BSE has highlighted some problems with BSE rapid test performance, the most significant being the number of initially reactive samples and the false positive results on autolyzed tissue. This point is important for BSE active surveillance in risk populations, because tissue autolysis is often unavoidable in routine cases. A robust test suitable for use on field material is therefore needed. To date, very limited information regarding the effect of autolysis on the robustness of rapid tests has been documented; therefore, the National Reference Centre for Animal Encephalopathies (CEA rapid test laboratory selected 450 autolyzed and negative brain stem samples from fallen stock bovines older than 24 months to assess the specificity of four tests approved for BSE active surveillance: Biorad TeSeE, Enfer TSE version 2.0, Prionics® Check LIA, and IDEXX Herd Check BSE Antigen Kit EIA. The samples were graded according to the degree of autolysis and then dissected into five portions, four of which randomly assigned to processing by rapid tests and one to be available for confirmatory Western blot analysis. Findings The specificity of the four systems was 100% for all three grades of autolysis, while the percentage of initially reactive results was 0.00 (95%CI 0.00-0.82, 0.22 (95%CI 0.006-1.23, 0.44 (95%CI 0.05-1.60, and 0.89 (95%CI 0.24-2.26 for the Biorad TeSeE, the Prionics® Check LIA, the IDEXX Herd Check BSE and the Enfer TSE tests, respectively. No association with the degree of autolysis could be drawn. Conclusions The present study demonstrates that the four rapid tests can be considered well-running diagnostic tools regardless of tissue quality; nevertheless, the number of initial reactive samples reported for some systems must not be underestimated in routine testing. Furthermore the compliance with the reported performance can be guaranteed only when an ongoing

  9. Quantitative analysis of wet-heat inactivation in bovine spongiform encephalopathy

    International Nuclear Information System (INIS)

    Highlights: ► We quantitatively analyzed wet-heat inactivation of the BSE agent. ► Infectivity of the BSE macerate did not survive 155 °C wet-heat treatment. ► Once the sample was dehydrated, infectivity was observed even at 170 °C. ► A quantitative PMCA assay was used to evaluate the degree of BSE inactivation. - Abstract: The bovine spongiform encephalopathy (BSE) agent is resistant to conventional microbial inactivation procedures and thus threatens the safety of cattle products and by-products. To obtain information necessary to assess BSE inactivation, we performed quantitative analysis of wet-heat inactivation of infectivity in BSE-infected cattle spinal cords. Using a highly sensitive bioassay, we found that infectivity in BSE cattle macerates fell with increase in temperatures from 133 °C to 150 °C and was not detected in the samples subjected to temperatures above 155 °C. In dry cattle tissues, infectivity was detected even at 170 °C. Thus, BSE infectivity reduces with increase in wet-heat temperatures but is less affected when tissues are dehydrated prior to the wet-heat treatment. The results of the quantitative protein misfolding cyclic amplification assay also demonstrated that the level of the protease-resistant prion protein fell below the bioassay detection limit by wet-heat at 155 °C and higher and could help assess BSE inactivation. Our results show that BSE infectivity is strongly resistant to wet-heat inactivation and that it is necessary to pay attention to BSE decontamination in recycled cattle by-products

  10. A pilot study for targeted surveillance of bovine spongiform encephalopathy in Nigeria.

    Science.gov (United States)

    Nwankiti, O O; Ikeh, E I; Asala, O; Seuberlich, T

    2013-06-01

    Bovine spongiform encephalopathy (BSE), popularly known as 'mad cow disease', led to an epidemic in Europe that peaked in the mid-1990s. Its impact on developing countries, such as Nigeria, has not been fully established as information on livestock and surveillance has eluded those in charge of this task. The BSE risk to Nigeria's cattle population currently remains undetermined, which has resulted in international trade restrictions on commodities from the cattle population. This is mainly because of a lack of updated BSE risk assessments and disease surveillance data. To evaluate the feasibility of BSE surveillance in Nigeria, we carried out a pilot study targeting cattle that were presented for emergency or casualty slaughter. In total, 1551 cattle of local breeds, aged 24 months and above were clinically examined. Ataxia, recumbency and other neurological signs were topmost on our list of criteria. A total of 96 cattle, which correspond to 6.2%, presented clinical signs that supported a suspect of BSE. The caudal brainstem tissues of these animals were collected post-mortem and analysed for the disease-specific form of the prion protein using a rapid test approved by the International Animal Health Organization (OIE). None of the samples were positive for BSE. Although our findings do not exclude the presence of BSE in Nigeria, they do demonstrate that targeted sampling of clinically suspected cases of BSE is feasible in developing countries. In addition, these findings point to the possibility of implementing clinical monitoring schemes for BSE and potentially other diseases with grave economic and public health consequences. PMID:22594841

  11. Modeling of bovine spongiform encephalopathy in a two-species feedback loop.

    Science.gov (United States)

    Barnes, Richard; Lehman, Clarence

    2013-06-01

    Bovine spongiform encephalopathy, otherwise known as mad cow disease, can spread when an individual cow consumes feed containing the infected tissues of another individual, forming a one-species feedback loop. Such feedback is the primary means of transmission for BSE during epidemic conditions. Following outbreaks in the European Union and elsewhere, many governments enacted legislation designed to limit the spread of such diseases via elimination or reduction of one-species feedback loops in agricultural systems. However, two-species feedback loops-those in which infectious material from one-species is consumed by a secondary species whose tissue is then consumed by the first species-were not universally prohibited and have not been studied before. Here we present a basic ecological disease model which examines the rôle feedback loops may play in the spread of BSE and related diseases. Our model shows that there are critical thresholds between the infection's expansion and decrease related to the lifespan of the hosts, the growth rate of the prions, and the amount of prions circulating between hosts. The ecological disease dynamics can be intrinsically oscillatory, having outbreaks as well as refractory periods which can make it appear that the disease is under control while it is still increasing. We show that non-susceptible species that have been intentionally inserted into a feedback loop to stop the spread of disease do not, strictly by themselves, guarantee its control, though they may give that appearance by increasing the refractory period of an epidemic's oscillations. We suggest ways in which age-related dynamics and cross-species coupling should be considered in continuing evaluations aimed at maintaining a safe food supply. PMID:23746801

  12. Whole Blood Gene Expression Profiling in Preclinical and Clinical Cattle Infected with Atypical Bovine Spongiform Encephalopathy

    Science.gov (United States)

    Xerxa, Elena; Barbisin, Maura; Chieppa, Maria Novella; Krmac, Helena; Vallino Costassa, Elena; Vatta, Paolo; Simmons, Marion; Caramelli, Maria; Casalone, Cristina; Corona, Cristiano

    2016-01-01

    Prion diseases, such as bovine spongiform encephalopathies (BSE), are transmissible neurodegenerative disorders affecting humans and a wide variety of mammals. Variant Creutzfeldt-Jakob disease (vCJD), a prion disease in humans, has been linked to exposure to BSE prions. This classical BSE (cBSE) is now rapidly disappearing as a result of appropriate measures to control animal feeding. Besides cBSE, two atypical forms (named H- and L-type BSE) have recently been described in Europe, Japan, and North America. Here we describe the first wide-spectrum microarray analysis in whole blood of atypical BSE-infected cattle. Transcriptome changes in infected animals were analyzed prior to and after the onset of clinical signs. The microarray analysis revealed gene expression changes in blood prior to the appearance of the clinical signs and during the progression of the disease. A set of 32 differentially expressed genes was found to be in common between clinical and preclinical stages and showed a very similar expression pattern in the two phases. A 22-gene signature showed an oscillating pattern of expression, being differentially expressed in the preclinical stage and then going back to control levels in the symptomatic phase. One gene, SEL1L3, was downregulated during the progression of the disease. Most of the studies performed up to date utilized various tissues, which are not suitable for a rapid analysis of infected animals and patients. Our findings suggest the intriguing possibility to take advantage of whole blood RNA transcriptional profiling for the preclinical identification of prion infection. Further, this study highlighted several pathways, such as immune response and metabolism that may play an important role in peripheral prion pathogenesis. Finally, the gene expression changes identified in the present study may be further investigated as a fingerprint for monitoring the progression of disease and for developing targeted therapeutic interventions. PMID

  13. Quantitative analysis of wet-heat inactivation in bovine spongiform encephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Matsuura, Yuichi; Ishikawa, Yukiko; Bo, Xiao; Murayama, Yuichi; Yokoyama, Takashi [Prion Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba, Ibaraki 305-0856 (Japan); Somerville, Robert A. [The Roslin Institute and Royal (Dick) School of Veterinary Studies, Roslin, Midlothian, EH25 9PS (United Kingdom); Kitamoto, Tetsuyuki [Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, 2-1 Seiryo, Aoba, Sendai 980-8575 (Japan); Mohri, Shirou, E-mail: shirou@affrc.go.jp [Prion Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba, Ibaraki 305-0856 (Japan)

    2013-03-01

    Highlights: ► We quantitatively analyzed wet-heat inactivation of the BSE agent. ► Infectivity of the BSE macerate did not survive 155 °C wet-heat treatment. ► Once the sample was dehydrated, infectivity was observed even at 170 °C. ► A quantitative PMCA assay was used to evaluate the degree of BSE inactivation. - Abstract: The bovine spongiform encephalopathy (BSE) agent is resistant to conventional microbial inactivation procedures and thus threatens the safety of cattle products and by-products. To obtain information necessary to assess BSE inactivation, we performed quantitative analysis of wet-heat inactivation of infectivity in BSE-infected cattle spinal cords. Using a highly sensitive bioassay, we found that infectivity in BSE cattle macerates fell with increase in temperatures from 133 °C to 150 °C and was not detected in the samples subjected to temperatures above 155 °C. In dry cattle tissues, infectivity was detected even at 170 °C. Thus, BSE infectivity reduces with increase in wet-heat temperatures but is less affected when tissues are dehydrated prior to the wet-heat treatment. The results of the quantitative protein misfolding cyclic amplification assay also demonstrated that the level of the protease-resistant prion protein fell below the bioassay detection limit by wet-heat at 155 °C and higher and could help assess BSE inactivation. Our results show that BSE infectivity is strongly resistant to wet-heat inactivation and that it is necessary to pay attention to BSE decontamination in recycled cattle by-products.

  14. The spongiform encephalopathies: prion diseases.

    Science.gov (United States)

    Mocsny, N

    1998-10-01

    The spongiform encephalopathies may be caused by prions, infectious pathogens that differ from all other infectious agents in that they do not have deoxyribonucleic acid (DNA) or ribonucleic acid (RNA). Very difficult to inactivate, they are composed of an abnormal protein. It is believed by many that prions cause sporatic and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy in animals and kuru, fatal familial insomnia, Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler-Scheinker disease in humans. Another, the new variant CJD in humans in England, is an example of a breech in the species barrier between humans and animals. Transmitted primarily via exposure to infected brain or spinal cord tissue or blood, there have been numerous iatrogenic cases from contaminated pituitary hormones, surgical equipment, dural grafts, corneal transplants and others. All facets of blood product manufacturing have been affected. Nurses should be aware of the latest developments, and able to practice infection control while providing the best patient/family information possible. PMID:9816561

  15. Control of bovine spongiform encephalopathy by genetic engineering: Possible approaches and regulatory considerations

    International Nuclear Information System (INIS)

    Full text: Transmissible spongiform encephalopathies (TSE), including bovine spongiform encephalopathy (BSE) and scrapie in sheep, are diseases in which affected animals exhibit abnormal neurological behavior associated with accumulation of prion protein in nervous system tissues. TSEs also occur in humans and include sporadic Creutzfeldt-Jakob disease (CJD), as well as its new variant (nvCJD) believed to be related to consumption of meat from BSE cattle. Since the emergence of BSE, also known as 'mad cow disease' in 1986, significant numbers of BSE cattle have been diagnosed in several countries and there have been more than 100 deaths from definite or probable nvCJD in humans. This paper examines a possible genetic modification that may render cattle resistant to BSE. Prion proteins (PrP), present in both animals and humans, consist of approximately 250 amino acids and play a role in copper transport and regulation in tissues. The conversion of a normal, wild-type PrPC into the pathogenic prion PrPSc, involves a change in protein folding. Presence of aberrantly folded PrPSc stimulates the conversion of PrPC to PrPSc and the accumulation of PrPSc leads to a dysfunction of the central neural system. The aberrant PrPSc is virtually indestructible as it is not digested by proteases, unaffected by sterilization techniques, high doses of gamma irradiation, or incineration. Naturally occurring scrapie resistant sheep that do not convert PrPC to PrPSc, have the 'ARR' genotype, i.e. amino acids alanine, arginine and arginine in PrP positions 136, 154 and 171. Sheep selection for scrapie resistance based on DNA analyses is now in progress. In humans, the presence of lysine instead of glutamine at position 219 of the PrP amino acid chain results in resistance to sporadic CJD. Any publication on existence of a similar resistance to BSE has not been found in available literature. Research with laboratory mice indicated a possible genetic engineering approach to the

  16. Individual factors associated with L- and H-type Bovine Spongiform Encephalopathy in France

    Directory of Open Access Journals (Sweden)

    Sala Carole

    2012-05-01

    Full Text Available Abstract Background Cattle with L-type (L-BSE and H-type (H-BSE atypical Bovine Spongiform encephalopathy (BSE were identified in 2003 in Italy and France respectively before being identified in other countries worldwide. As of December 2011, around 60 atypical BSE cases have currently been reported in 13 countries, with over one third in France. While the epidemiology of classical BSE (C-BSE has been widely described, atypical BSEs are still poorly documented, but appear to differ from C-BSE. We analysed the epidemiological characteristics of the 12 cases of L-BSE and 11 cases of H-BSE detected in France from January 2001 to late 2009 and looked for individual risk factors. As L-BSE cases did not appear to be homogeneously distributed throughout the country, two complementary methods were used: spatial analysis and regression modelling. L-BSE and H-BSE were studied separately as both the biochemical properties of their pathological prion protein and their features differ in animal models. Results The median age at detection for L-BSE and H-BSE cases was 12.4 (range 8.4-18.7 and 12.5 (8.3-18.2 years respectively, with no significant difference between the two distributions. However, this median age differed significantly from that of classical BSE (7.0 (range 3.5-15.4 years. A significant geographical cluster was detected for L-BSE. Among animals over eight years of age, we showed that the risk of being detected as a L-BSE case increased with age at death. This was not the case for H-BSE. Conclusion To the best of our knowledge this is the first study to describe the epidemiology of the two types of atypical BSE. The geographical cluster detected for L-BSE could be partly due to the age structure of the background-tested bovine population. Our regression analyses, which adjusted for the effect of age and birth cohort showed an age effect for L-BSE and the descriptive analysis showed a particular age structure in the area where the cluster was

  17. Time and frequency domain analysis of heart rate variability in cattle affected by bovine spongiform encephalopathy

    Directory of Open Access Journals (Sweden)

    Konold Timm

    2011-07-01

    Full Text Available Abstract Background Heart rate variability (HRV analysis is a method to assess the function of the autonomic nervous system. Brainstem nuclei that influence HRV are affected by vacuolar changes and accumulation of disease-associated prion protein (PrPd in bovine spongiform encephalopathy (BSE resulting in clinical signs suggestive of an increased parasympathetic tone. It was hypothesised that BSE in cattle causes changes in the autonomic nervous system; this was tested by comparing HRV indices derived from 1048 electrocardiograms, which were recorded from 51 naturally or experimentally infected cattle with BSE confirmed by postmortem tests, 321 clinical suspect cases or cattle inoculated with potentially infectious tissue without disease confirmation and 78 BSE-free control cattle. Findings Statistically significant differences were found for low or high frequency power, their normalised values and ratio when the last recording prior to cull or repeated recordings were compared but only between male and female cattle of the three groups and not between groups of the same gender, even though BSE cases of each gender appeared to be more nervous during the recording. The same findings were made for heart rate, deviation from the mean RR interval and vasovagal tonus index when repeated recordings were compared. BSE cases with severe vacuolar changes in the parasympathetic nucleus of the vagus nerve had a significantly lower low:high frequency power ratio but not a lower heart rate than BSE cases with mild vacuolation, whereas severity of vacuolar changes in the solitary tract nucleus or intensity of PrPd accumulation in both nuclei did not appear to have any affect on either index. Abnormalities in the electrocardiogram were detected in 3% of the recordings irrespective of the BSE status; sinus arrhythmia was present in 93% of the remaining recordings. Conclusions HRV analysis was not useful to distinguish BSE-positive from BSE-negative cattle

  18. Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

    Directory of Open Access Journals (Sweden)

    Okada Hiroyuki

    2011-06-01

    Full Text Available Abstract Atypical bovine spongiform encephalopathy (BSE has recently been identified in Europe, North America, and Japan. It is classified as H-type and L-type BSE according to the molecular mass of the disease-associated prion protein (PrPSc. To investigate the topographical distribution and deposition patterns of immunolabeled PrPSc, H-type BSE isolate was inoculated intracerebrally into cattle. H-type BSE was successfully transmitted to 3 calves, with incubation periods between 500 and 600 days. Moderate to severe spongiform changes were detected in the cerebral and cerebellar cortices, basal ganglia, thalamus, and brainstem. H-type BSE was characterized by the presence of PrP-immunopositive amyloid plaques in the white matter of the cerebrum, basal ganglia, and thalamus. Moreover, intraglial-type immunolabeled PrPSc was prominent throughout the brain. Stellate-type immunolabeled PrPSc was conspicuous in the gray matter of the cerebral cortex, basal ganglia, and thalamus, but not in the brainstem. In addition, PrPSc accumulation was detected in the peripheral nervous tissues, such as trigeminal ganglia, dorsal root ganglia, optic nerve, retina, and neurohypophysis. Cattle are susceptible to H-type BSE with a shorter incubation period, showing distinct and distinguishable phenotypes of PrPSc accumulation.

  19. Ultra-sensitive detection of prion protein fibrils by flow cytometry in blood from cattle affected with bovine spongiform encephalopathy

    Directory of Open Access Journals (Sweden)

    Maas Elke

    2005-10-01

    Full Text Available Abstract Background The definite diagnosis of prion diseases such as Creutzfeldt-Jakob disease (CJD in humans or bovine spongiform encephalopathy (BSE in cattle currently relies on the post mortem detection of the pathological form of the prion protein (PrPSc in brain tissue. Infectivity studies indicate that PrPSc may also be present in body fluids, even at presymptomatic stages of the disease, albeit at concentrations well below the detection limits of currently available analytical methods. Results We developed a highly sensitive method for detecting prion protein aggregates that takes advantage of kinetic differences between seeded and unseeded polymerization of prion protein monomers. Detection of the aggregates was carried out by flow cytometry. In the presence of prion seeds, the association of labelled recombinant PrP monomers in plasma and serum proceeds much more efficiently than in the absence of seeds. In a diagnostic model system, synthetic PrP aggregates were detected down to a concentration of approximately 10-8 nM [0.24 fg/ml]. A specific signal was detected in six out of six available serum samples from BSE-positive cattle. Conclusion We have developed a method based on seed-dependent PrP fibril formation that shows promising results in differentiating a small number of BSE-positive serum samples from healthy controls. This method may provide the basis for an ante mortem diagnostic test for prion diseases.

  20. Long-term impacts of bovine spongiform encephalopathy on beef risk perceptions and risk attitudes in Canada.

    Science.gov (United States)

    Muringai, Violet; Goddard, Ellen

    2016-01-01

    In this study, the objective was to examine whether or not changes in bovine spongiform encephalopathy (BSE) concerns exert an effect on people's risk perceptions and risk attitudes regarding consuming beef in Canada, 8 years after finding the first domestic animal with BSE. Data were collected from two surveys (2071 respondents) conducted with the same respondents in 2008 and 2011 in Canada. Data on meat consumption for the same households were also available from the Nielsen Homescan panel over the period 2002 to 2009. Based on census data, the current sample is generally not representative of the Canadian population, but the sample is unique in that the same individuals responded to two surveys and there is an ability to track their evolving household purchases of beef before the first survey and between the two surveys. In essence, alterations in beef risk perceptions are significantly influenced by changes in concerns regarding (1) feed given to livestock, (2) animal diseases and BSE, (3) trust in manufacturers, the government, and farmers, and (4) demographic characteristics. There were significant differences in beef purchases across households, with alterations to their risk perceptions and risk attitudes. In conclusion, although the first domestic incident of BSE was in 2003, concerns regarding BSE are still contributing to consumers' risk perceptions but not to their risk attitudes with respect to consumption of beef in 2011. PMID:27556567

  1. Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein.

    Science.gov (United States)

    Wilson, Rona; Plinston, Chris; Hunter, Nora; Casalone, Cristina; Corona, Cristiano; Tagliavini, Fabrizio; Suardi, Silvia; Ruggerone, Margherita; Moda, Fabio; Graziano, Silvia; Sbriccoli, Marco; Cardone, Franco; Pocchiari, Maurizio; Ingrosso, Loredana; Baron, Thierry; Richt, Juergen; Andreoletti, Olivier; Simmons, Marion; Lockey, Richard; Manson, Jean C; Barron, Rona M

    2012-07-01

    The association between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health and raises the possibility that other ruminant TSEs may be transmissible to humans. In recent years, several novel TSEs in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). Upon challenge with these ruminant TSEs, gene-targeted Tg mice expressing human PrP did not show any signs of disease pathology. These data strongly suggest the presence of a substantial transmission barrier between these recently identified ruminant TSEs and humans. PMID:22495232

  2. 9 CFR 94.18 - Restrictions on importation of meat and edible products from ruminants due to bovine spongiform...

    Science.gov (United States)

    2010-01-01

    ... and edible products from ruminants due to bovine spongiform encephalopathy. 94.18 Section 94.18..., AND BOVINE SPONGIFORM ENCEPHALOPATHY: PROHIBITED AND RESTRICTED IMPORTATIONS § 94.18 Restrictions on importation of meat and edible products from ruminants due to bovine spongiform encephalopathy. (a)(1)...

  3. Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle – an update

    Directory of Open Access Journals (Sweden)

    Konold Timm

    2012-12-01

    Full Text Available Abstract Background To provide information on dose–response and aid in modelling the exposure dynamics of the BSE epidemic in the United Kingdom groups of cattle were exposed orally to a range of different doses of brainstem homogenate of known infectious titre from clinical cases of classical bovine spongiform encephalopathy (BSE. Interim data from this study was published in 2007. This communication documents additional BSE cases, which occurred subsequently, examines possible influence of the bovine prion protein gene on disease incidence and revises estimates of effective oral exposure. Findings Following interim published results, two further cattle, one dosed with 100 mg and culled at 127 months post exposure and the other dosed with 10 mg and culled at 110 months post exposure, developed BSE. Both had a similar pathological phenotype to previous cases. Based on attack rate and incubation period distribution according to dose, the dose estimate at which 50% of confirmed cases would be clinically affected was revised to 0.15 g of the brain homogenate used in the experiment, with a 95% confidence interval of 0.03–0.79 g. Neither the full open reading frame nor the promoter region of the prion protein gene of dosed cattle appeared to influence susceptibility to BSE, but this may be due to the sample size. Conclusions Oral exposure of cattle to a large range of doses of a BSE brainstem homogenate produced disease in all dose groups. The pathological presentation resembled natural disease. The attack rate and incubation period were dependent on the dose.

  4. The analysis of costs related to bovine spongiform encephalopathy disease occurrence in the Czech Republic in 2001–2014

    Directory of Open Access Journals (Sweden)

    Richard POSPÍŠIL

    2015-12-01

    Full Text Available This paper pays attention to analysis of the economic impacts of the bovine spongiform encephalopathy (BSE occurrence in the Czech Republic, namely the financial compensations to the farmers whose herds had been affected and the costs of animal killing and carcass disposal in the rendering plant. Between February 2001 and the end of 2014, a total of 1 879 749 cows were examined and 30 cases of the BSE were detected. Consequently, 4 243 cows in cohorts were killed and their carcasses were safely disposed of. The farmers whose herds had been affected were provided compensations for the losses suffered. The total of the compensations in this period reached EUR 7 752 000. Of these, 83.3 % (EUR 6 458 000 were compensations for the value of the killed animals, 9.7 % (EUR 752 000 for the related costs, i.e., killing, safe disposal of carcasses and the examination for the BSE, and 6.9 % (EUR 535 000 for the losses due to non-materialised production. The average costs per 1 BSE-positive animal were EUR 258 400 and the average costs per 1 cohort animal were EUR 1 827. In the rendering plant responsible for killing the infected and cohort animals and safely disposing of their carcasses, the total of 2 342 tons of raw material was processed between March 2003 and 2009, and this cost EUR 363 777. The fact that there were only two last cases of the BSE in 2009 suggests a trend towards the disease eradication, which is in agreement with the situation in the other EU countries.

  5. Relationship between clinical signs and postmortem test status in cattle experimentally infected with the bovine spongiform encephalopathy agent

    Directory of Open Access Journals (Sweden)

    Stack Michael J

    2010-12-01

    Full Text Available Abstract Background Various clinical protocols have been developed to aid in the clinical diagnosis of classical bovine spongiform encephalopathy (BSE, which is confirmed by postmortem examinations based on vacuolation and accumulation of disease-associated prion protein (PrPd in the brain. The present study investigated the occurrence and progression of sixty selected clinical signs and behaviour combinations in 513 experimentally exposed cattle subsequently categorised postmortem as confirmed or unconfirmed BSE cases. Appropriate undosed or saline inoculated controls were examined similarly and the data analysed to explore the possible occurrence of BSE-specific clinical expression in animals unconfirmed by postmortem examinations. Results Based on the display of selected behavioural, sensory and locomotor changes, 20 (67% orally dosed and 17 (77% intracerebrally inoculated pathologically confirmed BSE cases and 21 (13% orally dosed and 18 (6% intracerebrally inoculated but unconfirmed cases were considered clinical BSE suspects. None of 103 controls showed significant signs and were all negative on diagnostic postmortem examinations. Signs indicative of BSE suspects, particularly over-reactivity and ataxia, were more frequently displayed in confirmed cases with vacuolar changes in the brain. The display of several BSE-associated signs over time, including repeated startle responses and nervousness, was significantly more frequent in confirmed BSE cases compared to controls, but these two signs were also significantly more frequent in orally dosed cattle unconfirmed by postmortem examinations. Conclusions The findings confirm that in experimentally infected cattle clinical abnormalities indicative of BSE are accompanied by vacuolar changes and PrPd accumulation in the brainstem. The presence of more frequently expressed signs in cases with vacuolar changes is consistent with this pathology representing a more advanced stage of disease. That

  6. Bovine spongiform encephalopathy infection alters endogenous retrovirus expression in distinct brain regions of cynomolgus macaques (Macaca fascicularis

    Directory of Open Access Journals (Sweden)

    Montag Judith

    2011-06-01

    Full Text Available Abstract Background Prion diseases such as bovine spongiform encephalopathies (BSE are transmissible neurodegenerative diseases which are presumably caused by an infectious conformational isoform of the cellular prion protein. Previous work has provided evidence that in murine prion disease the endogenous retrovirus (ERV expression is altered in the brain. To determine if prion-induced changes in ERV expression are a general phenomenon we used a non-human primate model for prion disease. Results Cynomolgus macaques (Macaca fasicularis were infected intracerebrally with BSE-positive brain stem material from cattle and allowed to develop prion disease. Brain tissue from the basis pontis and vermis cerebelli of the six animals and the same regions from four healthy controls were subjected to ERV expression profiling using a retrovirus-specific microarray and quantitative real-time PCR. We could show that Class I gammaretroviruses HERV-E4-1, ERV-9, and MacERV-4 increase expression in BSE-infected macaques. In a second approach, we analysed ERV-K-(HML-2 RNA and protein expression in extracts from the same cynomolgus macaques. Here we found a significant downregulation of both, the macaque ERV-K-(HML-2 Gag protein and RNA in the frontal/parietal cortex of BSE-infected macaques. Conclusions We provide evidence that dysregulation of ERVs in response to BSE-infection can be detected on both, the RNA and the protein level. To our knowledge, this is the first report on the differential expression of ERV-derived structural proteins in prion disorders. Our findings suggest that endogenous retroviruses may induce or exacerbate the pathological consequences of prion-associated neurodegeneration.

  7. Prion biology and bovine spongiform encephalopathy Biología del prion y encefalopatía espongiforme bovina

    Directory of Open Access Journals (Sweden)

    OA Peralta

    2011-01-01

    Full Text Available The complex nature of prions has intrigued the scientific community during the last 70 years. Since the first indication of scrapie infectivity and the experimental transmission of the scrapie agent in 1937, prions and their associated transmissible spongiform encephalopathies (TSEs have been under constant investigation. TSEs are neurodegenerative and fatal diseases with no early diagnosis, treatment or cure. Despite their diverse presentations, all TSEs stem from the infectious, spontaneous or hereditary conversion of the host-encoded cellular prion protein PrP C into the pathogenic isoform PrP Sc. Based on the prion hypothesis, PrP C has the autocatalytic or induced capacity to change its secondary configuration from a mainly α-helix structure into predominant β-sheet configuration. Another enigmatic aspect of the prion biology is the potential physiological function of PrP C, a protein that is widely distributed in mammalian tissues and intensely expressed in the nervous system. PrP C has been associated to several biological roles including cellular adhesion, protection and differentiation. The unpredictable properties of the PrP Sc and the complex presentation of TSEs have opened many questions yet to be answered. The potential zoonotic transmission of the bovine spongiform encephalopathy (BSE has generated intense concern in the international community over animal product biosecurity. During the last years, research in prion biology has mainly focused on determination of the pathogenesis of TSEs and the development of diagnostic and therapeutic methods. However, further research in prion biology is required in order to understand the complex nature of TSEs and how these diseases can be controlled.La compleja naturaleza de los priones ha intrigado a la comunidad científica durante los últimos 70 años. Desde el primer hallazgo de la infectividad del scrapie y la primera transmisión experimental de este agente en 1937, los priones y

  8. Laboratory Examinations of Transmissible Spongiform Encephalopathies in Denmark during 2012

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    The aim of this report is to give detailed information on the diagnostic examination on trans-missible spongiform encephalopathies (TSE) performed in Denmark during 2012. The present annual report is the 17th on this topic published by the National Veterinary Institute, Technical University of......, Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations. The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report as...

  9. Laboratory Examinations of Transmissible Spongiform Encephalopathies in Denmark during 2014

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    , Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations. The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report as......The aim of this report is to give detailed information on the diagnostic examination on transmissible spongiform encephalopathies (TSE) performed in Denmark during 2014. The present annual report is the 19th on this topic published by the National Veterinary Institute, Technical University of...

  10. Laboratory Examinations of Transmissible Spongiform Encephalopathies in Denmark during 2013

    DEFF Research Database (Denmark)

    Jensen, Tim Kåre

    , Chapter 2.4.6 and Chapter 2.7.13) regarding diagnostic examinations. The DTU-VET is the national reference laboratory of bovine spongiform encephalopathy (BSE) and TSE/Scrapie, and therefore the results of all neuropathological examinations on BSE and Scrapie in Denmark are given in the present report as......The aim of this report is to give detailed information on the diagnostic examination on trans-missible spongiform encephalopathies (TSE) performed in Denmark during 2013. The present annual report is the 18th on this topic published by the National Veterinary Institute, Technical University of...

  11. IMMUNOLOGICAL STUDY OF SPONGIFORM ENCEPHALOPATHIES

    Directory of Open Access Journals (Sweden)

    J. Meenupriya

    2013-04-01

    Full Text Available Spongiform encephalopathies, categorized as a subclass of neuro-degenerative diseases and commonly known as prion diseases, are a group of progressive conditions that affect the brain and nervous system of many animals, including humans. Prion diseases are common among cannibalistic communities; further research has revealed that the infected or malformed prion protein (named PrPsc spreads its virulence to the normal, healthy prion protein (named PrPc when people consume infected tissues. Knowing that a small interaction between normal and infected prion protein creates virulence, this relationship can be studied as a simple antigen-antibody interaction to understand the series of events that transform a normal prion protein into a virulent misfolded protein. Thoroughly modeled and validated structures of both PrPsc and PrPc can be effectively used to map the epitopes and thereby screen the antigen-antibody interaction using docking studies for a particular organism of concern. This simple immunological approach is used to understand the vital interaction between the normal and malformed proteins that is involved in the disease-spreading mechanism. Clarification of this mechanism could be used in various immune- and bioinformatics algorithms to map the interaction epitopes, furthering an understanding of these pathologies.

  12. Coexistence of two forms of disease-associated prion protein in extracerebral tissues of cattle infected with H-type bovine spongiform encephalopathy.

    Science.gov (United States)

    Okada, Hiroyuki; Miyazawa, Kohtaro; Masujin, Kentaro; Yokoyama, Takashi

    2016-08-01

    H-type bovine spongiform encephalopathy (H-BSE) is an atypical form of BSE in aged cattle. H-BSE is characterized by the presence of two proteinase K-resistant forms of disease-associated prion protein (PrP(Sc)), identified as PrP(Sc) #1 and PrP(Sc) #2, in the brain. To investigate the coexistence of different PrP(Sc) forms in the extracerebral tissues of cattle experimentally infected with H-BSE, immunohistochemical and molecular analyses were performed by using N-terminal-, core-region- and C-terminal-specific anti-prion protein antibodies. Our results demonstrated that two distinct forms of PrP(Sc) coexisted in the various extracerebral tissues. PMID:27010466

  13. Current status of transmissible spongiform encephalopathies in ruminants

    Directory of Open Access Journals (Sweden)

    Penders J.

    2004-01-01

    Full Text Available Transmissible spongiform encephalopathies (TSE encompass subacute neurological degenerative diseases for which the prototypes are scrapie in sheep and some forms of Creutzfeldt-Jakob disease in man. The emergence of a new form of TSE in cattle in United Kingdom (UK since 1986, namely bovine spongiform encephalopathy (BSE, sharply increased the interest for these diseases, especially because of the epidemic nature of BSE in UK, its subsequent spread in continental Europe and the later discovery of its zoonotic character. The number of measures of veterinary public health taken to control the disease and to prevent its spread to animals and human beings increased in time and culminated by the total feed ban. Indeed, since the beginning of 2001, feed containing proteins of animal origin is prohibited for the feeding of production animals, including ruminants and monogastric species. The effect of this total ban of mammalian meat and bone meal needs to be evaluated. The incidence of BSE has a trend to decrease in UK and in most of the other European member states. However, as BSE is a rare event distributed in a large bovine population, it is difficult to state unambiguously whether this trend is significant. Furthermore, the evaluation of this measure will be only effective at least five years after its introduction, since this period is the mean incubation time of BSE. The main concern is currently the eradication of BSE in the infected countries. Additionally, the control of scrapie is also carried out due to the possible contamination of sheep with the BSE agent. These actions must take into account several new facts: the recent discovery of BSE cases in countries with a low geographical BSE risk level as Japan, Canada and the United States of America (USA; the growing incidence of chronic wasting disease, a spongiform encephalopathy observed in deer in USA; the characterization of a new pattern of bovine amyloidotic spongiform encephalopathy in

  14. Experimental H-type and L-type bovine spongiform encephalopathy in cattle: observation of two clinical syndromes and diagnostic challenges

    Directory of Open Access Journals (Sweden)

    Konold Timm

    2012-03-01

    Full Text Available Abstract Background The majority of atypical bovine spongiform encephalopathy (BSE cases so far identified worldwide have been detected by active surveillance. Consequently the volume and quality of material available for detailed characterisation is very limiting. Here we report on a small transmission study of both atypical forms, H- and L-type BSE, in cattle to provide tissue for test evaluation and research, and to generate clinical, molecular and pathological data in a standardised way to enable more robust comparison of the two variants with particular reference to those aspects most relevant to case ascertainment and confirmatory diagnosis within existing regulated surveillance programmes. Results Two groups of four cattle, intracerebrally inoculated with L-type or H-type BSE, all presented with a nervous disease form with some similarities to classical BSE, which progressed to a more dull form in one animal from each group. Difficulty rising was a consistent feature of both disease forms and not seen in two BSE-free, non-inoculated cattle that served as controls. The pathology and molecular characteristics were distinct from classical BSE, and broadly consistent with published data, but with some variation in the pathological characteristics. Both atypical BSE types were readily detectable as BSE by current confirmatory methods using the medulla brain region at the obex, but making a clear diagnostic distinction between the forms was not consistently straightforward in this brain region. Cerebellum proved a more reliable sample for discrimination when using immunohistochemistry. Conclusions The prominent feature of difficulty rising in atypical BSE cases may explain the detection of naturally occurring cases in emergency slaughter cattle and fallen stock. Current confirmatory diagnostic methods are effective for the detection of such atypical cases, but consistently and correctly identifying the variant forms may require modifications to

  15. No H- and L-type cases in Belgium in cattle diagnosed with bovine spongiform encephalopathy (1999-2008 aging seven years and older

    Directory of Open Access Journals (Sweden)

    Van Muylem Patrick

    2010-05-01

    Full Text Available Abstract Background The bovine spongiform encephalopathy (BSE epidemic presented homogeneity of the phenotype. This classical BSE (called C-type was probably due to the contamination of the food chain by a single prion strain. However, due to the active surveillance and better techniques, two rare variants of BSE have been recently reported in different continents without a clear correlation to the BSE epidemic. These emerging types behave as different strains of BSE and were named H-type and L-type according to the high and low molecular mass of the unglycosylated fragment of their proteinase K resistant prion protein (PrPres. In these types, the proportion of the un-, mono- and di-glycosylated fragments of PrP (glycoprofile is also atypical and represents an effective diagnostic parameter. This study evaluated the presence of such types in bovine of 7 years and older in Belgium. Results The Belgian BSE archive contained 41 bovines of at least 7 years of age. The biochemical features of their PrPres were analyzed by Western blot with five antibodies recognising different regions of PrPres, from N- to C-terminus: 12B2, 9A2, Sha31, SAF84 and 94B4. All antibodies clearly detected PrPres except 12B2 antibody, which is specific for N-terminal region 101-105, a PrP region that is only retained in H-types. The glycoprofiles did correspond to that of C-type (with more than 55% of diglycosylated PrPres using antibody 94B4. Therefore, all cases have the features of C-type BSE. Conclusions This study supports that, among the BSE cases of 7 years and older identified in Belgium, none was apparently of the H- or L- type. This is consistent with the very rare occurrence of atypical BSE and the restricted dimension of Belgium. These results shed some light on the worldwide prevalence of atypical BSE.

  16. Removal of transmissible spongiform encephalopathy prion from large volumes of cell culture media supplemented with fetal bovine serum by using hollow fiber anion-exchange membrane chromatography.

    Directory of Open Access Journals (Sweden)

    Ming Li Chou

    Full Text Available Cases of variant Creutzfeldt-Jakob disease in people who had consumed contaminated meat products from cattle with bovine spongiform encephalopathy emphasize the need for measures aimed at preventing the transmission of the pathogenic prion protein (PrPSc from materials derived from cattle. Highly stringent scrutiny is required for fetal bovine serum (FBS, a growth-medium supplement used in the production of parenteral vaccines and therapeutic recombinant proteins and in the ex vivo expansion of stem cells for transplantation. One such approach is the implementation of manufacturing steps dedicated to removing PrPSc from materials containing FBS. We evaluated the use of the QyuSpeed D (QSD adsorbent hollow-fiber anion-exchange chromatographic column (Asahi Kasei Medical, Tokyo, Japan for the removal of PrPSc from cell culture media supplemented with FBS. We first established that QSD filtration had no adverse effect on the chemical composition of various types of culture media supplemented with 10% FBS or the growth and viability characteristics of human embryonic kidney (HEK293 cells, baby hamster kidney (BHK-21 cells, African green monkey kidney (Vero cells, and Chinese hamster ovary (CHO-k1 cells propagated in the various culture-medium filtrates. We used a 0.6-mL QSD column for removing PrPSc from up to 1000 mL of Dulbecco's modified Eagle's medium containing 10% FBS previously spiked with the 263K strain of hamster-adapted scrapie. The Western blot analysis, validated alongside an infectivity assay, revealed that the level of PrPSc in the initial 200mL flow-through was reduced by 2.5 to > 3 log10, compared with that of the starting material. These results indicate that QSD filtration removes PrPSc from cell culture media containing 10% FBS, and demonstrate the ease with which QSD filtration can be implemented in at industrial-scale to improve the safety of vaccines, therapeutic recombinant proteins, and ex vivo expanded stem cells produced

  17. Encefalopatías espongiformes transmisibles Transmissible spongiform encephalopathies

    Directory of Open Access Journals (Sweden)

    Jorge E Delgado-Hachmeister

    2002-01-01

    Full Text Available Las encefalopatías espongiformes transmisibles (EET han cobrado gran importancia en los últimos años. Principalmente por el surgimiento de la encefalopatía espongiforme del bovino (EEB y la nueva variante de la ermedad de Creutzfeldt-Jakob (nvECJ, esta última probablemente adquirida por la ingesta de carne de bovino contaminada. Hasta la fecha se ha informado de 109 casos de la nvECJ en el humano y la gran mayoría de los casos ha ocurrido en el Reino Unido. No se sabe la magnitud real que podrán tener las EET en el humano, sin embargo algunos piensan que nos encontramos en el principio de una pandemia de la nvECJ. En el presente artículo se discuten varios aspectos de las EET y métodos para la prevención de la transmisión de estas enfermedades, tanto en rumiantes como en el humano.Transmissible spongiform encephalopathies (TSE are a group of diseases which have received a lot of attention in recent years. The interest on these diseases has been stimulated by the appearance of bovine spongiform encephalopathy (BSE and the new variant of Creutzfeldt-Jakob disease (nvCJD; the latter is likely to be acquired by ingesting contaminated beef. Until now 109 cases of nvCJD have been reported, most of them occurring in the United Kingdom. Some experts think that this is the beginning of a nvCJD pandemic. Deep knowledge of the mechanisms of transmission of TSE is needed to prevent the emergence of a TSE pandemic in humans.We address various aspects of TSE and discuss prevention methods of TSE in ruminants and humans.

  18. 疯牛病发生与防制新进展%A Newly Occurrence Prevention and cure Introduction of Bovine Spongiform Encephalopathy

    Institute of Scientific and Technical Information of China (English)

    王红宁; 王平章; 吴琦

    2002-01-01

    疯牛病(Mad-cow disease)是牛海绵状脑病(Bovine Spongiform Encephalopa山y,BSE)的俗称,是一种慢性、传染性、致死性的中枢神经系统疾病.该病自1985年4月首次在英国发现以来,至今已在许多国家都有发现.目前,世界上有100多个过国家面临着严重疯牛病的威胁.本文从病原、流行病学、发生与防制等方面对疯牛病进行了综述.

  19. PRIONS AND THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

    Science.gov (United States)

    This book chapter is an invited, scholarly review of the mechanism(s) of TSEs for the 2nd edition of Metabolic Encephalopathies. Each chapter in the book assumes a professional knowledge of neuroscience and biochemistry, and the focus of the book is on the metabolic basis of dise...

  20. The new variant of the Creutzfeldt-Jakob disease accounts for no relative increase of the Creutzfeldt-Jakob disease mortality rate in the United Kingdom; this fits ill with the new variant being the consequence of consumption of food infected with the agent of Bovine Spongiform Encephalopathy

    Directory of Open Access Journals (Sweden)

    Hénaut Alain

    2003-08-01

    Full Text Available Abstract Background A new variant of Creutzfeldt-Jakob disease was described in the United Kingdom. It is often claimed that it is caused by consumption of food infected with the agent of bovine spongiform encephalopathy. However, this remains open to question because the number of cases of the variant is, at the present time, less than would be expected from a major food-borne source. Discussion The EUROCJD cooperative study presents currently available epidemiological data of Creutzfeldt-Jakob disease and its new variant, for nine European countries plus Australia and Canada. Unexpectedly, for the United Kingdom where all but a few cases of the new variant have been reported, these cases have to be included in the incidence curve of the sporadic forms of the disease in order to obtain the best fit with the median curve from all the countries. This variant could be merely a rare clinical phenotype within the sporadic disease. The published clinical and experimental data which suggest that it is linked with bovine spongiform encephalopathy, lead us to propose that this link could be a common etiological origin other than consumption of bovine infected food. In any case, public health recommendations hold and further investigation is required. Summary The lack of a relative increase of the Creutzfeldt-Jakob-disease mortality rate in the United Kingdom, does not fit well with the new variant being the consequence of consumption of food infected with the agent of bovine spongiform encephalopthy.

  1. Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles.

    Science.gov (United States)

    Kirkwood, J K; Cunningham, A A

    1994-09-24

    Since 1986, scrapie-like spongiform encephalopathy has been diagnosed in 19 captive wild animals of eight species at or from eight zoological collections in the British Isles. The affected animals have comprised members of the family Bovidae: one nyala (Tragelaphus angasi), four eland (Taurotragus oryx), and six greater kudu (Tragelaphus strepsiceros), one gemsbok (Oryx gazella), one Arabian oryx (Oryx leucoryx), and one scimitar-horned oryx (Oryx dammah), and members of the family Felidae: four cheetah (Acinonyx jubatus) and one puma (Felis concolor). In addition, three cases of a spongiform encephalopathy of unknown aetiology have been reported in ostriches (Struthio camellus) from two zoos in north west Germany. Three features suggest that some of these cases may have been caused by the agent of bovine spongiform encephalopathy (BSE). First, they have been temporally and geographically coincident with the BSE epidemic. Secondly, in all the ungulates for which details are available, it is possible that either the affected animal itself, or the herd into which it was born or moved, had been exposed to proprietary feeds containing ruminant-derived protein or other potentially contaminated material, and all the carnivores had been fed parts of cattle carcases judged unfit for human consumption. Thirdly, the pathological results of inoculating mice with a homogenate of fixed brain tissue from the nyala and from one greater kudu were similar to the results of inoculating mice with BSE brain tissue. PMID:7817514

  2. Risk analysis of Transmissible Spongiform Encephalopathies in animals: state-of-the-art

    DEFF Research Database (Denmark)

    Paisley, Larry; de Koeijer, Aline; Hagenaars, Thomas J.;

    2008-01-01

    ). Nevertheless, the decline in the BSE epidemic in the UK and most European countries demonstrates that management has been, for the most part, sucessful. Literature pertaining to the three inter-related facets of risk analysis: risk assessment, risk management and risk communication of TSE's of animal origin...... was reviewed and used to describe the state-of-the-art of risk analysis for TSEs.......The Bovine Spongiform Encephalopathy (BSE) crisis of the last two decades has shown that proper interaction of risk assessment, risk management and risk communication is essential. Mathematical models and risk assessments have been used as a basis for BSE risk management options and much of the...

  3. Retrovirus antigens in brains of mice with scrapie- and murine leukemia virus-induced spongiform encephalopathy.

    OpenAIRE

    Hoffman, P M; Pitts, O M; Rohwer, R. G.; Gajdusek, D C; Ruscetti, S K

    1982-01-01

    Wild mouse ecotropic virus-induced spongiform encephalomyelopathy pathologically similar to scrapie was associated with the expression of retrovirus antigens in mouse brains. However, scrapie-infected mice with spongiform encephalopathy showed no increased expression of retrovirus antigens in brain. Thus, the pathogenesis of the scrapie spongiform lesion does not appear to involve activation of endogenous retrovirus.

  4. Heart rate variability analysis in sheep affected by transmissible spongiform encephalopathies

    Directory of Open Access Journals (Sweden)

    Konold Timm

    2011-12-01

    Full Text Available Abstract Background The function of the autonomic nervous system can be assessed by determining heart rate variability (HRV, which is impaired in some brainstem diseases in humans. Transmissible spongiform encephalopathies (TSEs in sheep are diseases characterised by accumulation of disease-associated prion protein in the brainstem, including nuclei of the parasympathetic nervous system. This study was undertaken to assess whether analysis of HRV can be used as an aid in the diagnosis of TSEs in clinically affected, naturally or experimentally infected sheep. Findings When HRV indices were compared between 41 clinical TSE cases (18 sheep infected with scrapie and 23 sheep infected with bovine spongiform encephalopathy, 11 control sheep and six sheep reported as scrapie suspects or dosed with BSE brain homogenate, which were not confirmed as TSE cases by postmortem tests, no significant differences were found between the groups. Median heart rate was significantly different but only when sheep were grouped by gender: it was higher in female TSE cases than in control sheep and higher in female than castrated male ovine classical BSE cases. Conclusions HRV analysis was not useful as a diagnostic aid for TSEs of sheep.

  5. Contribution du modèle Age-Période-Cohorte à l'étude de l'épizootie d'Encéphalopathie Spongiforme Bovine en France et en Europe

    OpenAIRE

    Carole Sala, Carole

    2009-01-01

    Contribution Model Age-Period-Cohort to study the outbreak of Bovine Spongiform Encephalopathy in France and Europe Contribution du modèle Age-Période-Cohorte à l'étude de l'épizootie d'Encéphalopathie Spongiforme Bovine en France et en Europe

  6. Three Essays on the Economic Impact of Bovin Spngiform Encephalopathy in the United States

    OpenAIRE

    Gollamudi, Rachna

    2011-01-01

    The first native-born case of Bovine Spongiform Encephalopathy (BSE or commonly known as Mad Cow Disease) in North American continent was reported on 20 May 2003 in central Alberta, Canada. The first case of BSE in the United States was announced on 23 December 2003. My dissertation is divided into three essays on the economic impact of the outbreak of Bovine Spongiform Encephalopathy (BSE) in the United States in December 2003. The first essay focuses on quantifying the impact of the outb...

  7. Bovine Spongiform Encephalopathy: Production and Inspection

    Science.gov (United States)

    ... Forms Skip Navigation Z7_0Q0619C0JGR010IFST1G5B10H1 Web Content Viewer (JSR 286) Actions ${title} Loading... / Topics / Food Safety Education / ... Protections Against BSE Z7_0Q0619C0JGR010IFST1G5B10H3 Web Content Viewer (JSR 286) Actions ${title} Loading... Z7_0Q0619C0JGR010IFST1G5B1090 Web Content ...

  8. Comparative evidence for a link between Peyer's patch development and susceptibility to transmissible spongiform encephalopathies

    Directory of Open Access Journals (Sweden)

    Rhind Susan M

    2006-01-01

    Full Text Available Abstract Background Epidemiological analyses indicate that the age distribution of natural cases of transmissible spongiform encephalopathies (TSEs reflect age-related risk of infection, however, the underlying mechanisms remain poorly understood. Using a comparative approach, we tested the hypothesis that, there is a significant correlation between risk of infection for scrapie, bovine spongiform encephalopathy (BSE and variant CJD (vCJD, and the development of lymphoid tissue in the gut. Methods Using anatomical data and estimates of risk of infection in mathematical models (which included results from previously published studies for sheep, cattle and humans, we calculated the Spearman's rank correlation coefficient, rs, between available measures of Peyer's patch (PP development and the estimated risk of infection for an individual of the corresponding age. Results There was a significant correlation between the measures of PP development and the estimated risk of TSE infection; the two age-related distributions peaked in the same age groups. This result was obtained for each of the three host species: for sheep, surface area of ileal PP tissue vs risk of infection, rs = 0.913 (n = 19, P s = 0.933 (n = 19, P s = 0.693 (n = 94, P s = 0.384 (n = 46, P = 0.008. In addition, when changes in exposure associated with BSE-contaminated meat were accounted for, the two age-related patterns for humans remained concordant: rs = 0.360 (n = 46, P = 0.014. Conclusion Our findings suggest that, for sheep, cattle and humans alike there is an association between PP development (or a correlate of PP development and susceptibility to natural TSE infection. This association may explain changes in susceptibility with host age, and differences in the age-susceptibility relationship between host species.

  9. Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases).

    Science.gov (United States)

    Budka, H; Aguzzi, A; Brown, P; Brucher, J M; Bugiani, O; Gullotta, F; Haltia, M; Hauw, J J; Ironside, J W; Jellinger, K

    1995-10-01

    Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human transmissible spongiform encephalopathies (prion diseases) are proposed for the following disease entities: CJD--sporadic, iatrogenic (recognised risk) or familial (same disease in 1st degree relative): spongiform encephalopathy in cerebral and/or cerebellar cortex and/or subcortical grey matter; or encephalopathy with prion protein (PrP) immunoreactivity (plaque and/or diffuse synaptic and/or patchy/perivacuolar types). Gerstmann-Sträussler-Scheinker disease (GSS) (in family with dominantly inherited progressive ataxia and/or dementia): encephalo(myelo)pathy with multicentric PrP plaques. Familial fatal insomnia (FFI) (in member of a family with PRNP178 mutation): thalamic degeneration, variable spongiform change in cerebrum. Kuru (in the Fore population). Without PrP data, the crucial feature is the spongiform change accompanied by neuronal loss and gliosis. This spongiform change is characterised by diffuse or focally clustered small round or oval vacuoles in the neuropil of the deep cortical layers, cerebellar cortex or subcortical grey matter, which might become confluent. Spongiform change should not be confused with non-specific spongiosis. This includes status spongiosus ("spongiform state"), comprising irregular cavities in gliotic neuropil following extensive neuronal loss (including also lesions of "burnt-out" CJD), "spongy" changes in brain oedema and metabolic encephalopathies, and artefacts such as superficial cortical, perineuronal, or perivascular vacuolation; focal changes indistinguishable from spongiform change may occur in some cases of Alzheimer's and diffuse Lewy body diseases. Very rare cases might not be diagnosed by these criteria. Then confirmation must be sought by additional techniques such as PrP immunoblotting, preparations for electron microscopic examination of scrapie associated fibrils (SAF), molecular biologic studies, or experimental

  10. Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities

    Science.gov (United States)

    Transmissible spongiform encephalopathies (TSEs) are fatal neurologic diseases that can affect several animal species and human beings. There are four animal TSE agents found in the United States: scrapie of sheep and goats; chronic wasting disease (CWD) of deer, elk, and moose; transmissible mink ...

  11. Differential expression of interferon responsive genes in rodent models of transmissible spongiform encephalopathy disease

    Directory of Open Access Journals (Sweden)

    Lazar Jozef

    2007-03-01

    Full Text Available Abstract Background The pathological hallmarks of transmissible spongiform encephalopathy (TSE diseases are the deposition of a misfolded form of a host-encoded protein (PrPres, marked astrocytosis, microglial activation and spongiosis. The development of powerful gene based technologies has permitted increased levels of pro-inflammatory cytokines to be demonstrated. However, due to the use of assays of differing sensitivities and typically the analysis of a single model system it remained unclear whether this was a general feature of these diseases or to what extent different model systems and routes of infection influenced the relative levels of expression. Similarly, it was not clear whether the elevated levels of cytokines observed in the brain were accompanied by similar increases in other tissues that accumulate PrPres, such as the spleen. Results The level of expression of the three interferon responsive genes, Eif2ak2, 2'5'-OAS, and Mx2, was measured in the brains of Syrian hamsters infected with scrapie 263K, VM mice infected with bovine spongiform encephalopathy and C57BL/6 mice infected with the scrapie strain ME7. Glial fibrillary acidic expression confirmed the occurrence of astrocytosis in all models. When infected intracranially all three models showed a similar pattern of increased expression of the interferon responsive genes at the onset of clinical symptoms. At the terminal stage of the disease the level and pattern of expression of the three genes was mostly unchanged in the mouse models. In contrast, in hamsters infected by either the intracranial or intraperitoneal routes, both the level of expression and the expression of the three genes relative to one another was altered. Increased interferon responsive gene expression was not observed in a transgenic mouse model of Alzheimer's disease or the spleens of C57BL/6 mice infected with ME7. Concurrent increases in TNFα, TNFR1, Fas/ApoI receptor, and caspase 8 expression in ME

  12. Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies

    Institute of Scientific and Technical Information of China (English)

    Zhiqi Song; Deming Zhao; Lifeng Yang

    2013-01-01

    Transmissible spongiform encephalopathies refer to a group of infectious neurodegenerative diseases with an entirely novel mechanism of transmission and pathophysiology including synaptic damage,dendritic atrophy,vacuolization,and microglial activation.Extensive neuronal loss is the main cause of chronic brain deterioration and fatal outcome of prion diseases.As the final outcome of pathological alterations,neuronal death is a prominent feature of all prion diseases.The mechanisms responsible for prion diseases are not well understood.A more comprehensive understanding of the molecular basis of neuronal damage is essential for the development of an effective therapy for transmissible spongiform encephalopathies and other neurodegenerative diseases sharing similar features.Here,we review the molecular mechanisms of mitochondrial dysfunction and endoplasmic reticulum stress-mediated neuronal death,which play crucial roles in the pathogenisis of prion diseases.

  13. Trends in scientific activity addressing transmissible spongiform encephalopathies: a bibliometric study covering the period 1973–2002

    Directory of Open Access Journals (Sweden)

    Iribarren-Maestro Isabel

    2006-10-01

    Full Text Available Abstract Background The purpose of this study is to analyse the trends in scientific research on transmissible spongiform encephalopathies by applying bibliometric tools to the scientific literature published between 1973 and 2002. Methods The data for the study were obtained from Medline database, in order to determine the volume of scientific output in the above period, the countries involved, the type of document and the trends in the subject matters addressed. The period 1973–2002 was divided in three sub-periods. Results We observed a significant growth in scientific production. The percentage of increase is 871.7 from 1973 to 2002. This is more evident since 1991 and particularly in the 1996–2001 period. The countries found to have the highest output were the United States, the United Kingdom, Japan, France and Germany. The evolution in the subject matters was almost constant in the three sub-periods in which the study was divided. In the first and second sub-periods, the subject matters of greatest interest were more general, i.e Nervous system or Nervous system diseases, Creutzfeldt-Jakob disease, Scrapie, and Chemicals and Drugs, but in the last sub-period, some changes were observed because the Prion-related matters had the greatest presence. Collaboration among authors is small from 1973 to 1992, but increases notably in the third sub-period, and also the number of authors and clusters formed. Some of the authors, like Gajdusek or Prusiner, appear in the whole period. Conclusion The study reveals a very high increase in scientific production. It is related also with the beginnings of research on bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease, with the establishment of progressive collaboration relationships and a reflection of public health concerns about this problem.

  14. Familial spongiform encephalopathy associated with a novel prion protein gene mutation.

    Science.gov (United States)

    Nitrini, R; Rosemberg, S; Passos-Bueno, M R; da Silva, L S; Iughetti, P; Papadopoulos, M; Carrilho, P M; Caramelli, P; Albrecht, S; Zatz, M; LeBlanc, A

    1997-08-01

    Human prion diseases include Creutzfeldt-Jakob disease, Gerstmann-Stráussler-Scheinker disease, fatal familial insomnia, and kuru. Each of these diseases has a specific clinical presentation while spongiform encephalopathy, neuronal loss, and gliosis are their neuropathological hallmarks. We studied a Brazilian family with an autosomal dominant form of dementia. Nine members of the family were affected by a dementia with frontotemporal clinical features, with a mean age at onset of 44.8 +/- 3.8 years and a mean duration of symptoms of 4.2 +/- 2.4 years. Neuropathological examination of 3 patients showed severe spongiform change and neuronal loss in the deep cortical layers and in the putamen, but minimal gliosis in the most severely affected areas. The putamen and cerebellum, but not other areas of the affected brain, displayed prion protein immunoreactivity. A novel prion protein gene mutation causing a nonconservative substitution at codon 183 was identified in 2 neuropathologically confirmed affected individuals (mother and son). The mutation was transmitted in a mendelian fashion to 12 members of the family. Therefore, we identified a novel prion disease variant characterized by an early onset and long duration of the symptoms, severe spongiform change with minimal gliosis, associated with a prion protein gene mutation at codon 183. PMID:9266722

  15. Prion Diseases: Update on Mad Cow Disease, Variant Creutzfeldt-Jakob Disease, and the Transmissible Spongiform Encephalopathies.

    Science.gov (United States)

    Janka, Jacqueline; Maldarelli, Frank

    2004-08-01

    Transmissible spongiform encephalopathies (TSEs) are a group of progressive, fatal neurodegenerative disorders that share a common spongiform histopathology. TSEs may be transmitted in a sporadic, familial, iatrogenic, or zoonotic fashion. The putative infectious agent of TSE, the prion, represents a novel paradigm of infectious disease with disease transmission in the absence of nucleic acid. Several small but spectacular epidemics of TSEs in man have prompted widespread public health and food safety concerns. Although TSEs affect a comparatively small number of individuals, prion research has revealed fascinating insights of direct relevance to common illnesses. This paper reviews recent advances that have shed new light on the nature of prions and TSEs. PMID:15265460

  16. Resistance of Bovine Spongiform Encephalopathy (BSE) Prions to Inactivation

    OpenAIRE

    Kurt Giles; Glidden, David V.; Robyn Beckwith; Rose Seoanes; David Peretz; Stephen J DeArmond; Prusiner, Stanley B.

    2008-01-01

    Distinct prion strains often exhibit different incubation periods and patterns of neuropathological lesions. Strain characteristics are generally retained upon intraspecies transmission, but may change on transmission to another species. We investigated the inactivation of two related prions strains: BSE prions from cattle and mouse-passaged BSE prions, termed 301V. Inactivation was manipulated by exposure to sodium dodecyl sulfate (SDS), variations in pH, and different temperatures. Infectiv...

  17. Toward unfolding the prion misfolding mystery: protein free radical chemistry in transmissible spongiform encephalopathies

    International Nuclear Information System (INIS)

    Owing to the high oxygen-respiration in the brain of mammals, oxidative damage to prion protein has been suggested to be an additional factor. A large body of intriguing features of scrapie and prion diseases have provided multiple lines of indirect chemistry evidence, suggesting that the infectious agents may be putative forms of sequence-specific prion radicals (SSPR) and/or their immediate precursors in the transmissible spongiform encephalopathies (TSE). Here a molecular mechanism corresponding to the self-replication of scrapie protein mediated by prion free-radical processes, consonant with 'protein-only' hypotheses is proposed. This new theory may not only aid our understanding of the occurrence of prions, but also provides new insight into the possible chemistry principles underlying the neutrodegenerative disorders. It is anticipated that future studies based on this suggestion and chemistry principles of genetic diseases may allow us to determine an effective approach to stop mad cow disease and its human version, new variant of Creutzfeldt-Jakob disease (v CJD)

  18. Defining and Assessing Analytical Performance Criteria for Transmissible Spongiform Encephalopathy-Detecting Amyloid Seeding Assays.

    Science.gov (United States)

    Gray, John G; Graham, Catherine; Dudas, Sandor; Paxman, Eric; Vuong, Ben; Czub, Stefanie

    2016-05-01

    Transmissible spongiform encephalopathies (TSEs) are infectious, fatal neurodegenerative diseases that affect production animal health, and thus human food safety. Enhanced TSE detection methods mimic the conjectured basis for prion replication, in vitro; biological matrices can be tested for prion activity via their ability to convert recombinant cellular prion protein (PrP) into amyloid fibrils; fluorescent spectra changes of amyloid-binding fluorophores in the reaction vessel detect fibril formation. In vitro PrP conversion techniques have high analytical sensitivity for prions, comparable with that of bioassays, yet no such protocol has gained regulatory approval for use in animal TSE surveillance programs. This study describes a timed in vitro PrP conversion protocol with accurate, well-defined analytical criteria based on probability density and mass functions of TSE(+) and TSE(-) associated thioflavin T signal times, a new approach within this field. The prion detection model used is elk chronic wasting disease (CWD) in brain tissues. The protocol and analytical criteria proved as sensitive for elk CWD as two bioassay models, and upward of approximately 1.2 log10 more sensitive than the most sensitive TSE rapid test we assessed. Furthermore, we substantiate that timing in vitro PrP conversion may be used to titrate TSE infectivity, and, as a result, provide a comprehensive extrapolation of analytical sensitivity differences between bioassay, TSE rapid tests, and in vitro PrP conversion for elk CWD. PMID:27068712

  19. Towards unfolding the prion misfolding mystery: Protein free radical chemistry in transmissible spongiform encephalopathies

    Institute of Scientific and Technical Information of China (English)

    YANG Chi-Ming

    2003-01-01

    Owing to the high oxygen-respiration in the brain of mammals, oxidative damage to prion protein hasbeen suggested to be an additional factor. A large body of intriguing features of scrapie and prion diseases haveprovided multiple lines of indirect chemistry evidence, suggesting that the infectious agents may be putative forms ofsequence-specific prion radicals (SSPR) and/or their immediate precursors in the transmissible spongiform encepha-lopathies (TSE). Here a molecular mechanism corresponding to the self-replication of scrapie protein mediated byprion free-radical processes, consonant with "protein-only" hypotheses is proposed. This new theory may not onlyaid our understanding of the occurrence of prions, but also provides new insight into the possible chemistry principlesunderlying the neurodegenerative disorders. It is anticipated that future studies based on this suggestion and chem-istry principles of genetic diseases may allow us to determine an effective approach to stop mad cow disease and itshuman version, new variant of Creutzfeldt-Jakob disease (v CJD).

  20. Assessing transmissible spongiform encephalopathy species barriers with an in vitro prion protein conversion assay

    Science.gov (United States)

    Johnson, Christopher J.; Carlson, Christina M.; Morawski, Aaron R.; Manthei, Alyson; Cashman, Neil R.

    2015-01-01

    Studies to understanding interspecies transmission of transmissible spongiform encephalopathies (TSEs, prion diseases) are challenging in that they typically rely upon lengthy and costly in vivo animal challenge studies. A number of in vitro assays have been developed to aid in measuring prion species barriers, thereby reducing animal use and providing quicker results than animal bioassays. Here, we present the protocol for a rapid in vitroprion conversion assay called the conversion efficiency ratio (CER) assay. In this assay cellular prion protein (PrPC) from an uninfected host brain is denatured at both pH 7.4 and 3.5 to produce two substrates. When the pH 7.4 substrate is incubated with TSE agent, the amount of PrPC that converts to a proteinase K (PK)-resistant state is modulated by the original host’s species barrier to the TSE agent. In contrast, PrPC in the pH 3.5 substrate is misfolded by any TSE agent. By comparing the amount of PK-resistant prion protein in the two substrates, an assessment of the host’s species barrier can be made. We show that the CER assay correctly predicts known prion species barriers of laboratory mice and, as an example, show some preliminary results suggesting that bobcats (Lynx rufus) may be susceptible to white-tailed deer (Odocoileus virginianus) chronic wasting disease agent.

  1. In vitro prion protein conversion suggests risk of bighorn sheep (Ovis canadensis) to transmissible spongiform encephalopathies

    Science.gov (United States)

    Johnson, Christopher J.; Morawski, A.R.; Carlson, C.M.; Chang, H.

    2013-01-01

    Background: Transmissible spongiform encephalopathies (TSEs) affect both domestic sheep (scrapie) and captive and free-ranging cervids (chronic wasting disease; CWD). The geographical range of bighorn sheep (Ovis canadensis; BHS) overlaps with states or provinces that have contained scrapie-positive sheep or goats and areas with present epizootics of CWD in cervids. No TSEs have been documented in BHS, but the susceptibility of this species to TSEs remains unknown. Results: We acquired a library of BHS tissues and found no evidence of preexisting TSEs in these animals. The prion protein gene (Prnp) in all BHS in our library was identical to scrapie-susceptible domestic sheep (A136R 154Q171). Using an in vitro prion protein conversion assay, which has been previously used to assess TSE species barriers and, in our study appears to recollect known species barriers in mice, we assessed the potential transmissibility of TSEs to BHS. As expected based upon Prnp genotype, we observed BHS prion protein conversion by classical scrapie agent and evidence for a species barrier between transmissible mink encephalopathy (TME) and BHS. Interestingly, our data suggest that the species barrier of BHS to white-tailed deer or wapiti CWD agents is likely low. We also used protein misfolding cyclic amplification to confirm that CWD, but not TME, can template prion protein misfolding in A136R 154Q171genotype sheep. Conclusions: Our results indicate the in vitro conversion assay used in our study does mimic the species barrier of mice to the TSE agents that we tested. Based on Prnp genotype and results from conversion assays, BHS are likely to be susceptible to infection by classical scrapie. Despite mismatches in amino acids thought to modulate prion protein conversion, our data indicate that A136R154Q171 genotype sheep prion protein is misfolded by CWD agent, suggesting that these animals could be susceptible to CWD. Further investigation of TSE transmissibility to BHS, including

  2. Influence of the immune system on peripherally acquired transmissible spongiform encephalopathy infection with special reference to the role of the follicular dendritic cell

    OpenAIRE

    Brown, Karen L.

    2009-01-01

    The Transmissible Spongiform Encephalopathies (TSEs) or “prion” diseases are a group of fatal neurodegenerative diseases the aetiology of which is not fully understood. These diseases are characterised by a number of pathological changes in the central nervous system (CNS) including; vacuolation of the neuropil, gliosis and deposition of PrPSc; the abnormal form of the host glycoprotein PrP. Although the major pathology in these diseases is associated with the CNS the immune system is central...

  3. Elimination of transmissible spongiform encephalopathy infectivity and decontamination of surgical instruments by using radio-frequency gas-plasma treatment.

    Science.gov (United States)

    Baxter, H C; Campbell, G A; Whittaker, A G; Jones, A C; Aitken, A; Simpson, A H; Casey, M; Bountiff, L; Gibbard, L; Baxter, R L

    2005-08-01

    It has now been established that transmissible spongiform encephalopathy (TSE) infectivity, which is highly resistant to conventional methods of deactivation, can be transmitted iatrogenically by contaminated stainless steel. It is important that new methods are evaluated for effective removal of protein residues from surgical instruments. Here, radio-frequency (RF) gas-plasma treatment was investigated as a method of removing both the protein debris and TSE infectivity. Stainless-steel spheres contaminated with the 263K strain of scrapie and a variety of used surgical instruments, which had been cleaned by a hospital sterile-services department, were examined both before and after treatment by RF gas plasma, using scanning electron microscopy and energy-dispersive X-ray spectroscopic analysis. Transmission of scrapie from the contaminated spheres was examined in hamsters by the peripheral route of infection. RF gas-plasma treatment effectively removed residual organic residues on reprocessed surgical instruments and gross contamination both from orthopaedic blades and from the experimentally contaminated spheres. In vivo testing showed that RF gas-plasma treatment of scrapie-infected spheres eliminated transmission of infectivity. The infectivity of the TSE agent adsorbed on metal spheres could be removed effectively by gas-plasma cleaning with argon/oxygen mixtures. This treatment can effectively remove 'stubborn' residual contamination on surgical instruments. PMID:16033987

  4. Spongiform encephalopathy in transgenic mice expressing a point mutation in the β2–α2 loop of the prion protein

    OpenAIRE

    C. J. Sigurdson; Joshi-Barr, S; Bett, C.; Winson, O; Manco, G; Schwarz, P.; Rülicke, T.; Nilsson, K P R; Margalith, I.; Raeber, A; Peretz, D; Hornemann, S; Wüthrich, K; Aguzzi, A

    2011-01-01

    Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases attributed to misfolding of the cellular prion protein, PrPC, into a β-sheet-rich, aggregated isoform, PrPSc. We previously found that expression of mouse PrP with the two amino acid substitutions S170N and N174T, which result in high structural order of the β2–α2 loop in the NMR structure at pH 4.5 and 20 °C, caused transmissible de novo prion disease in transgenic mice. Here we report that expression of mo...

  5. EFSA BIOHAZ Panel (EFSA Panel on Biological Hazards), 2014. Scientific Opinion on BSE risk in bovine intestines and mesentery

    DEFF Research Database (Denmark)

    Hald, Tine; Baggesen, Dorte Lau

    Bovine intestines and mesenteries in the European Union (EU) have to be removed from the food and feed chain. The opinion provides a quantitative assessment of the Bovine Spongiform Encephalopathy (BSE) infectious load that might enter the food and feed chain yearly if bovine intestine and...

  6. BOVINE SPONGIFORM ENCEPHALOPATHY (BSE): RISKS AND IMPLICATIONS FOR THE UNITED STATES

    OpenAIRE

    Fox, John A.; Peterson, Hikaru Hanawa

    2002-01-01

    Practioner's Abstract: Mad cow disease has caused two disruptions in European beef markets--first in the U.K. in 1996 following the announcement of a link to new variant Creutzfeldt-Jacob Disease in humans, and the second in late 2000 following the discovery of "homegrown" cases of the disease in Germany and Spain. In September 2001 the disease was discovered in Japan where it also resulted in an immediate and substantial reduction in beef demand. The disease has not been found in the U.S. bu...

  7. 78 FR 72859 - Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy

    Science.gov (United States)

    2013-12-04

    ... countries can be viewed at: Austria: http://www.oie.int/fileadmin/Home/eng/Internationa_Standard_Setting/docs/pdf/SCAD/A_SCAD_Feb2012.pdf (page 46). ] Belgium: http://www.oie.int/fileadmin/Home/eng.../Home/eng/Internationa_Standard_Setting/docs/pdf/SCAD/A_SCAD_Feb2012.pdf (page 48). Bulgaria:...

  8. In-situ spectroscopic investigation of transmissible spongiform encephalopathies: application of Fourier-transform infrared spectroscopy to a scrapie-hamster model

    Science.gov (United States)

    Kneipp, Janina; Lasch, Peter; Beekes, Michael; Naumann, Dieter

    2002-03-01

    Transmissible spongiform encephalopathies (TSE), such as BSE in cattle, scrapie in sheep and goats, and Creutzfeldt-Jakob disease in man are a group of fatal infectious diseases of the central nervous system that are far from being fully understood. Presuming the pathological changes to originate from small disease-specific compositional and structural modifications at the molecular level, Fourier-transform infrared (FTIR) spectroscopy can be used to achieve insight into biochemical parameters underlying pathogenesis. We have developed an FTIR microspectroscopy-based strategy which, as a combination of image reconstruction and multivariate pattern recognition methods, permitted the comparison of identical substructures in the cerebellum of healthy and TSE-infected Syrian hamsters in the terminal stage of the disease. Here we present FTIR data about the pathological changes of scrapie-infected and normal tissue of the gray matter structures stratum granulosum and stratum moleculare. IR spectroscopy was also applied to tissue pieces of the medulla oblongata of infected and control Syrian hamsters. Mapping data were analyzed with cluster analysis and imaging methods. We found variations in the spectra of the infected tissue, which are due to changes in carbohydrates, nucleic acids, phospholipids, and proteins.

  9. EU-Approved Rapid Tests for Bovine Spongform Encephalopathy Detect Atypical Forms: A Study for Their Sensitivities

    NARCIS (Netherlands)

    Meloni, D.; Davidse, A.; Langeveld, J.P.M.; varello, K.; Casalone, C.; Corona, C.; Balkema-Buschmann, A.; Groschup, M.; Ingravalle, F.; Bozzetta, E.

    2012-01-01

    Since 2004 it become clear that atypical bovine spongiform encephalopthies (BSEs) exist in cattle. Whenever their detection has relied on active surveillance plans implemented in Europe since 2001 by rapid tests, the overall and inter-laboratory performance of these diagnostic systems in the detecti

  10. Ultrastructural characteristics (or evaluation) of Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies or prion diseases.

    Science.gov (United States)

    Liberski, Paweł P; Sikorska, Beata; Hauw, Jean-Jacques; Kopp, Nicolas; Streichenberger, Nathalie; Giraud, Pierrie; Boellaard, Jan; Budka, Herbert; Kovacs, Gabor G; Ironside, James; Brown, Paul

    2010-12-01

    The authors report on a large series of human prion diseases to establish ultrastructural characteristics that may be useful for their diagnosis. For Creutzfeldt-Jakob disease (CJD and its variant, vCJD) and fatal familial insomnia (FFI) only vacuolation (spongiform change) and the presence of tubulovesicular structures are consistent findings. Other changes, such as the presence of myelinated vacuoles, branching cisternae, neuroaxonal dystrophy, and autophagic vacuoles, were present in different proportions in either CJD or FFI, but they are nonspecific ultrastructural findings that can also occur in other neurodegenerative conditions. The hallmark of Gerstmann-Sträussler-Scheinker disease (GSS) and vCJD is the amyloid plaque, but plaques of GSS and kuru are different than those of vCJD. Whereas the former are typical unicentric kuru type or multicentric plaques, the latter are unicentric florid plaques. Also, kuru plaques are nonneuritic, whereas GSS florid plaques are usually neuritic; however, a proportion of plaques from GSS was also found to have nonneuritic characteristics. Thus, the presence or absence of dystrophic neurites is not a discriminatory factor for GSS and vCJD. Furthermore, plaques from GSS with different mutations were also slightly different. In GSS with mutations P102L, 232T, and A117V plaques were stellate while in 1 case with 144 base-pair insertion and in GSS-A117V, round plaques were also observed, and typical primitive neuritic plaques, i.e., composed of dystrophic neurites with little or no amyloid, were found only in a P102L case from the original Austrian family. In 2 cases of sporadic CJD, the kuru stellate plaque predominated. PMID:21070167

  11. Transmission of Atypical Bovine Prions to Mice Transgenic for Human Prion Protein

    OpenAIRE

    Béringue, Vincent; Herzog, Laëtitia; Reine, Fabienne; Le Dur, Annick; Casalone, Cristina; Vilotte, Jean-Luc; Laude, Hubert

    2008-01-01

    To assess risk for cattle-to-human transmission of prions that cause uncommon forms of bovine spongiform encephalopathy (BSE), we inoculated mice expressing human PrP Met129 with field isolates. Unlike classical BSE agent, L-type prions appeared to propagate in these mice with no obvious transmission barrier. H-type prions failed to infect the mice.

  12. Accumulation of L-type Bovine Prions in Peripheral Nerve Tissues

    OpenAIRE

    Iwamaru, Yoshifumi; Imamura, Morikazu; Matsuura, Yuichi; Masujin, Kentaro; Shimizu, Yoshihisa; Shu, Yujing; Kurachi, Megumi; Kasai, Kazuo; Murayama, Yuichi; Fukuda, Shigeo; Onoe, Sadao; Hagiwara, Ken’ichi; Yamakawa, Yoshio; Sata, Tetsutaro; Mohri, Shirou

    2010-01-01

    We recently reported the intraspecies transmission of L-type atypical bovine spongiform encephalopathy (BSE). To clarify the peripheral pathogenesis of L-type BSE, we studied prion distribution in nerve and lymphoid tissues obtained from experimentally challenged cattle. As with classical BSE prions, L-type BSE prions accumulated in central and peripheral nerve tissues.

  13. A quantitative assessment of the risk of transmission of bovine spongiform encephalopathy by tallow-based calf milk-replacer

    DEFF Research Database (Denmark)

    Paisley, Larry; Hostrup-Pedersen, J.

    A Monte Carlo simulation model was constructed to assess the risk of BSE transmission to calves by calf milk-replacer (CMR). We assumed that any BSE infectivity in the CMR would be associated with the allowable levels of impurities in tallow used to manufacture the milk-replacer. Simulations used...

  14. A quantitative assessment of the risk of transmission of bovine spongiform encephalopathy by tallow-based calf milk-replacer

    DEFF Research Database (Denmark)

    Paisley, Larry; Hostrup-Pedersen, J.

    A Monte Carlo simulation model was constructed to assess the risk of BSE transmission to calves by calf milk-replacer (CMR). We assumed that any BSE infectivity in the CMR would be associated with the allowable levels of impurities in tallow used to manufacture the milk-replacer. Simulations used...... three different levels of impurities, six different distributions of the BSE infectivity titers of CNS tissues and with and without inclusion of specified risk material (SRM). Our results suggest that tallow-based CMR could have been responsible for some BSE infections in nearly all simulations. The...... reduction in the allowable impurities in tallow and the exclusion of SRM have greatly reduced-but have not eliminated-the risk of BSE transmission by CMR The results of the simulations are associated with much uncertainty....

  15. Establishment of bovine prion peptide-based monoclonal antibodies for identifying bovine prion

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    To obtain high titer monoclonal antibodies(McAbs) which can react with mammalian prion protein(PrP),Balb/C mice were immunized with bovine(Bo) PrP peptide(BoPrP 209-228 aa) coupled to keyhole limpet hemocyanin(KLH).The hybridoma cell lines secreting monoclonal antibodies against the peptide were established by cell fusion and cloning.The obtained McAbs were applied to detect recombinant human,bovine and hamster PrP,cellular prion protein(PrPc) in normal bovine brain and pathogenic scrapie prion protein(PrPSc) accumulated in the medulla oblongata of bovine spongiform encephalopathy(BSE)specimen with Western blot and immunohistochemical detection,respectively.The current procedure might offer a simple,feasible method to raise high titer antibodies for studying biological features of PrP in mammals,as well as detection of transmissible spongiform encephalopathy(TSE) and diagnosis of BSE,in particular.

  16. Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy

    Directory of Open Access Journals (Sweden)

    Paul Brown

    2013-07-01

    Full Text Available Successful transmission of Transmissible Mink Encephalopathy (TME to cattle supports the bovine hypothesis for the still controversial origin of TME outbreaks. Human and primate susceptibility to classical Bovine Spongiform Encephalopathy (c-BSE and the transmissibility of L-type BSE to macaques indicate a low cattle-to-primate species barrier. We therefore evaluated the zoonotic potential of cattle-adapted TME. In less than two years, this strain induced in cynomolgus macaques a neurological disease similar to L-BSE but distinct from c-BSE. TME derived from another donor species (raccoon induced a similar disease with even shorter incubation periods. L-BSE and cattle-adapted TME were also transmissible to transgenic mice expressing human prion protein (PrP. Secondary transmissions to transgenic mice expressing bovine PrP maintained the features of the three tested bovine strains (cattle TME, c-BSE and L-BSE regardless of intermediate host. Thus, TME is the third animal prion strain transmissible to both macaques and humanized transgenic mice, suggesting zoonotic potentials that should be considered in the risk analysis of animal prion diseases for human health. Moreover, the similarities between TME and L-BSE are highly suggestive of a link between these strains, and therefore the possible presence of L-BSE for many decades prior to its identification in USA and Europe.

  17. 75 FR 57737 - Notice of Request for Approval of an Information Collection; National Veterinary Services...

    Science.gov (United States)

    2010-09-22

    ...; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Documents... associated with National Veterinary Services Laboratories diagnostic support for the bovine spongiform... Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Documents. OMB...

  18. Retinal function and morphology are altered in cattle infected with the prion disease transmissible mink encephalopathy.

    Science.gov (United States)

    Smith, J D; Greenlee, J J; Hamir, A N; Richt, J A; Greenlee, M H West

    2009-09-01

    Transmissible spongiform encephalopathies (TSEs) are a group of diseases that result in progressive and invariably fatal neurologic disease in both animals and humans. TSEs are characterized by the accumulation of an abnormal protease-resistant form of the prion protein in the central nervous system. Transmission of infectious TSEs is believed to occur via ingestion of prion protein-contaminated material. This material is also involved in the transmission of bovine spongiform encephalopathy ("mad cow disease") to humans, which resulted in the variant form of Creutzfeldt-Jakob disease. Abnormal prion protein has been reported in the retina of TSE-affected cattle, but despite these observations, the specific effect of abnormal prion protein on retinal morphology and function has not been assessed. The objective of this study was to identify and characterize potential functional and morphologic abnormalities in the retinas of cattle infected with a bovine-adapted isolate of transmissible mink encephalopathy. We used electroretinography and immunohistochemistry to examine retinas from 10 noninoculated and 5 transmissible mink encephalopathy-inoculated adult Holstein steers. Here we show altered retinal function, as evidenced by prolonged implicit time of the electroretinogram b-wave, in transmissible mink encephalopathy-infected cattle before the onset of clinical illness. We also demonstrate disruption of rod bipolar cell synaptic terminals, indicated by decreased immunoreactivity for the alpha isoform of protein kinase C and vesicular glutamate transporter 1, and activation of Müller glia, as evidenced by increased glial fibrillary acidic protein and glutamine synthetase expression, in the retinas of these cattle at the time of euthanasia due to clinical deterioration. This is the first study to identify both functional and morphologic alterations in the retinas of TSE-infected cattle. Our results support future efforts to focus on the retina for the development of

  19. Transmission barriers for bovine ovine, and human prions in transgenic mice

    OpenAIRE

    Van Scott, Michael R.; Peretz, David; Nguyen, Hoang-Oanh B.; Stephen J DeArmond; Prusiner, Stanley B.

    2005-01-01

    Transgenic (Tg) mice expressing full-length bovine prion protein (BoPrP) serially propagate bovine spongiform encephalopathy (BSE) prions without posing a transmission barrier. These mice also posed no transmission barrier for Suffolk sheep scrapie prions, suggesting that cattle may be highly susceptible to some sheep scrapie strains. Tg(BoPrP) mice were also found to be susceptible to prions from humans with variant Creutzfeldt-Jakob disease (CJD); on second passage in Tg(BoPrP) mice, the in...

  20. Time trends in exposure of cattle to bovine spongiform encephalopathy and cohort effect in France and Italy: value of the classical Age-Period-Cohort approach

    Directory of Open Access Journals (Sweden)

    Ru Giuseppe

    2009-09-01

    Full Text Available Abstract Background The Age-Period-Cohort (APC analysis is routinely used for time trend analysis of cancer incidence or mortality rates, but in veterinary epidemiology, there are still only a few examples of this application. APC models were recently used to model the French epidemic assuming that the time trend for BSE was mainly due to a cohort effect in relation to the control measures that may have modified the BSE exposure of cohorts over time. We used a categorical APC analysis which did not require any functional form for the effect of the variables, and examined second differences to estimate the variation of the BSE trend. We also reanalysed the French epidemic and performed a simultaneous analysis of Italian data using more appropriate birth cohort categories for comparison. Results We used data from the exhaustive surveillance carried out in France and Italy between 2001 and 2007, and comparatively described the trend of the epidemic in both countries. At the end, the shape and irregularities of the trends were discussed in light of the main control measures adopted to control the disease. In Italy a decrease in the epidemic became apparent from 1996, following the application of rendering standards for the processing of specific risk material (SRM. For the French epidemic, the pattern of second differences in the birth cohorts confirmed the beginning of the decrease from 1995, just after the implementation of the meat and bone meal (MBM ban for all ruminants (1994. Conclusion The APC analysis proved to be highly suitable for the study of the trend in BSE epidemics and was helpful in understanding the effects of management and control of the disease. Additionally, such an approach may help in the implementation of changes in BSE regulations.

  1. 疯牛病--牛海绵状脑病(BSE)研究进展%Review on mad cow disease--Bovine Spongiform Encephalopathy(BSE)

    Institute of Scientific and Technical Information of China (English)

    李海阔

    2001-01-01

    疯牛病是一种由朊病毒感染引起的、非炎性的、致死性的脑退化性疾病,并可传染给人.本文从流行病学、病原学、病理学等方面论述了疯牛病的研究进展情况,详细讨论了朊病毒的生物学特性、致病机理和增殖模式.

  2. Fu ruya et al / J . Livestock Sci . ( 2012 ) 3: 7 - 26 7 Vaccines containing organic mercury compound and implications in relation to BSE (bovine spongiform encephalopathy)

    OpenAIRE

    Furuya, A; Furuya, M; S. K atagiri; M. K agoshima

    2012-01-01

    The present study was undertaken to investigate the effect of thimerosal (an organic mercury compound), a preservative incorporated into vaccines, on living organisms. A case is presented where BSE-like pathological conditions in brain tissue is artificially created artificially created. Possible mechanisms and causes are discussed. Implications are commented on. In this study ourexperiments revealed production of similar BSE-like pathological conditions through in vivo staining of a chemical...

  3. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Stages of Hepatic Encephalopathy? What Triggers or Can Cause HE to Get Worse? How is HE Diagnosed? ... portosystemic encephalopathy or PSE, is a condition that causes temporary worsening of brain function in people with ...

  4. Characterization of melanoma associated spongiform scleropathy

    DEFF Research Database (Denmark)

    Alyahya, Ghassan Ayish Jabur; Heegaard, Steffen; Prause, J.U.

    ophthalmology, melanoma associated spongiform scleropathy (MASS), MASS, malignant uveal melanoma, sclera, ciliary body, choroid, histopathology......ophthalmology, melanoma associated spongiform scleropathy (MASS), MASS, malignant uveal melanoma, sclera, ciliary body, choroid, histopathology...

  5. Diabetic encephalopathy

    OpenAIRE

    I. A. Strokov; V. V. Zakharov; K. I. Strokov

    2012-01-01

    The epidemiology, clinical presentation, morphology, and pathogenesis of central nervous system lesion in types 1 and 2 diabetes mellitus (DM) are considered, by using the results of experimental and clinical studies. The definition of diabetic encephalopathy is given. Whether there is a relationship between diabetic encephalopathy and diabetic polyneuropathy is considered. It is concluded that it is expedient to identify diabetic encephalopathy as a complication of DM. The capacities of path...

  6. Diabetic encephalopathy

    Directory of Open Access Journals (Sweden)

    I. A. Strokov

    2012-01-01

    Full Text Available The epidemiology, clinical presentation, morphology, and pathogenesis of central nervous system lesion in types 1 and 2 diabetes mellitus (DM are considered, by using the results of experimental and clinical studies. The definition of diabetic encephalopathy is given. Whether there is a relationship between diabetic encephalopathy and diabetic polyneuropathy is considered. It is concluded that it is expedient to identify diabetic encephalopathy as a complication of DM. The capacities of pathogenetic treatment for diabetic encephalopathy are shown.

  7. Instability of familial spongiform encephalopathy-related prion mutants

    International Nuclear Information System (INIS)

    We examined the influence of D177N (D178N in humans) mutation on the conformational stability of the S2 region of moPrPC with varying pHs by using the SDSL-ESR technique. The ESR spectrum of D177N at pH 7.5 was narrower than that of Y161R1, referred to as WT*. The ESR spectrum of D177N did not change when pH in the solution decreased to pH 4.0. Our results suggested that the disappearance of a salt bridge (D177-R163) induced the increase in the instability of S2 region. Moreover, the line shape of the ESR spectrum obtained from H176S neighboring the salt bridge linked to the S2 region was similar to D177N. These results indicate that the protonation of H176 is strongly associated with the stability of S2 region. These findings are important for understanding the mechanism by which the disruption of the salt bridge in the S2 region forms the pathogenic PrPSc structure in hereditary prion disease

  8. 9 CFR 121.3 - VS select agents and toxins.

    Science.gov (United States)

    2010-01-01

    ... encephalopathy agent; Camel pox virus; Classical swine fever virus; Ehrlichia ruminantium (Heartwater); Foot-and... pathogenic), bovine spongiform encephalopathy agent, classical swine fever virus, foot-and-mouth...

  9. Disease-associated prion protein in neural and lymphoid tissues of mink (Mustela vison) inoculated with transmissible mink encephalopathy

    Science.gov (United States)

    Transmissible mink encephalopathy (TME) is a prion disorder of farmed raised mink. As with the other transmissible spongiform encephalopathies, the disorder is associated with accumulation of the misfolded prion protein in the brain and an invariably fatal outcome. TME outbreaks have been rare but...

  10. Hepatic encephalopathy

    Science.gov (United States)

    ... mild and include: Breath with a musty or sweet odor Change in sleep patterns Changes in thinking ... 24411831 . Nevah MI, Fallon MB. Hepatic encephalopathy, hepatorenal syndrome, hepatopumonary syndrome, and systemic complications of liver disease. ...

  11. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Symptoms to look for Caregiver Support Caregiver Stories Home › What is Hepatic Encephalopathy? Why Your Liver is ... questions about HE, one step at a time. Home About Us Ways to Give Contact Us Privacy ...

  12. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Get Worse? How is HE Diagnosed? Prior to Treatment Who treats HE? Preparing for your Medical Appointment Hepatic Encephalopathy Treatment Options Treatment Basics Treatment Medications Importance of Adhering ...

  13. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Hepatic Encephalopathy Treatment Options Treatment Basics Treatment Medications Importance of Adhering to Your Treatment Plan Long-Term Considerations Patient Support Finding Support Services Peer Support Groups Financial Assistance Support for My Loved Ones Resources Find ...

  14. Hepatic Encephalopathy

    Science.gov (United States)

    ... Hepatic Encephalopathy Treatment Options Treatment Basics Treatment Medications Importance of Adhering to Your Treatment Plan Long-Term Considerations Patient Support Finding Support Services Peer Support Groups Financial Assistance Support for My Loved Ones Resources Find ...

  15. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Hepatic Encephalopathy so you can tell your doctor right away if you think you may have it. ... American Liver Foundation © 2016 American Liver Foundation. All rights reserved. Funding for the HE123 - Diagnosis, Treatment and ...

  16. Limited amplification of chronic wasting disease prions in the peripheral tissues of intracerebrally inoculated cattle

    Science.gov (United States)

    Chronic wasting disease (CWD) is a fatal neurodegenerative disease, classified as a prion disease or transmissible spongiform encephalopathy (TSE) similar to bovine spongiform encephalopathy (BSE). Cervids affected by CWD accumulate an abnormal protease resistant prion protein throughout the central...

  17. Protein Hydrolysates from Non-bovine and Plant Sources Replaces Tryptone in Microbiological Media

    Science.gov (United States)

    Ranganathan, Yamini; Patel, Shifa; Pasupuleti, Vijai K.; Meganathan, R.

    Tryptone (pancreatic digest of casein) is a common ingredient in laboratory and fermentation media for growing wild-type and genetically modified microorganisms. Many of the commercially manufactured products such as human growth hormone, antibiotics, insulin, etc. are produced by recombinant strains grown on materials derived from bovine sources. With the emergence of Bovine Spongiform Encephalopathy (BSE) and the consequent increase in Food and Drug Administration (FDA) regulations, elimination of materials of bovine origin from fermentation media is of paramount importance. To achieve this objective, a number of protein hydrolysates derived from non-bovine animal and plant sources were evaluated. Tryptone in Luria-Bertani (LB) broth was replaced with an equal quantity of alternate protein hydrolysates. Four of the six hydrolysates (one animal and three from plants) were found to efficiently replace the tryptone present in LB-medium as measured by growth rate and growth yield of a recombinant Escherichia coli strain. In addition, we have determined plasmid stability, inducibility and activity of the plasmid encoded β-galactosidase in the recombinant strain grown in the presence of various protein hydrolysates.

  18. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... of brain function in people with advanced liver disease. When your liver is damaged it can no longer remove toxic substances from your blood. These toxins build up and can travel through your body until they reach your brain, causing mental and physical symptoms of HE. Hepatic Encephalopathy often ...

  19. [Hepatic encephalopathy].

    Science.gov (United States)

    Jacques, Jérémie; Carrier, Paul; Debette-Gratien, Marilyne; Sobesky, Rodolphe; Loustaud-Ratti, Véronique

    2016-01-01

    Hepatic encephalopathy is a severe complication of liver cirrhosis and is an important therapeutic challenge, with a social and economic issue. If, now, the pathophysiology is not totally understood (main role of ammonia, but a better understanding of cerebral mechanisms), the clinical presentation is well-known. Some treatments are useful (disaccharides, treatment of the trigger) but their efficiency is limited. Nevertheless, the emergence of new treatments, such as non-absorbable antibiotics (rifaximin essentially), is an interesting therapeutic tool. PMID:26597584

  20. Thinking the unthinkable: Alzheimer's, Creutzfeldt-Jakob and Mad Cow disease: the age-related reemergence of virulent, foodborne, bovine tuberculosis or losing your mind for the sake of a shake or burger.

    Science.gov (United States)

    Broxmeyer, Lawrence

    2005-01-01

    The possibility of the age-related reemergence of foodborne Mycobacterium bovis (bovine tuberculosis) as a vector for Creutzfeldt-Jakob Disease (CJD or human Mad Cow Disease) and Mad Cow disease itself is real. The CDC reported last May of an outbreak of CJD linked to the consumption of meat contaminated "with the agent causing" bovine spongiform encephalopathy (BSE) in a New Jersey racetrack between the time frame 1995-2004. In the opinion of experts, ample justification exists for considering a similar pathogenesis for Alzheimer's, Creutzfeldt-Jakob and the other spongiform encephalopathies such as Mad Cow disease. In fact, Creutzfeldt-Jakob and Alzheimer's often coexist and at this point are thought to differ merely by time-dependent physical changes. A recent study links up to 13% of all "Alzheimer's" victims as really having Creutzfeldt-Jakob disease. Bovine tuberculosis, which includes Mycobacterium bovis and M. avium-intracellulare or paratuberculosis, is and has always been the most prevalent threat to the cattle industry, and the USDA reports that between 20% and 40% of US dairy herds are infected with paratuberculosis alone. The health risk for milk tainted with M. bovis has been known for decades and there was a time not so long ago when "tuberculin-tested" was printed on every milk container. Schliesser stated that meat from tuberculous animals may also constitute a significant risk of infection. At the turn of the 20th century 25% of the many US deaths from TB in adults were caused by M. bovis. Dairy products aside, when past and present meat consumption are factored in, there is three times the risk of developing Alzheimer's in meat eaters as opposed to vegetarians. The investigation into the causal trail for Creutzfeldt-Jakob, indistinguishable from Alzheimer's except for its shorter, lethal course might have grown cold where it not for Roel's and others who linked mad cow in cattle with M. bovis and related paratuberculosis on clinical, pathologic

  1. Comparative study of the effectiveness and safety of porcine and bovine atelocollagen in Asian nasolabial fold correction.

    Science.gov (United States)

    Moon, Suk-Ho; Lee, Yoon-Jae; Rhie, Jong-Won; Suh, Dong-Sam; Oh, Deuk-Young; Lee, Joong-Ho; Kim, Young-Jin; Kim, Sue-Min; Jun, Young-Joon

    2015-06-01

    Bovine-derived collagen has been used for soft-tissue augmentation since 1977. However, there are issues regarding the possibility of bovine spongiform encephalopathy (BSE). Researchers discovered that the histologic structure of porcine-derived collagen is similar to that of human dermal collagen and that it is free from the risk of BSE. This study was conducted to establish the effectiveness and safety of porcine-derived collagen compared to bovine-derived collagen. The 73 patients included in this study were healthy volunteers who responded to an advertisement approved by the Institutional Review Board (IRB). They had visited the authors' hospital complaining of wrinkles on their nasolabial fold. Either porcine (TheraFill®) or bovine atelocollagen was randomly injected into each side of their nasolabial folds, and the five-grade Wrinkle Severity Rating Scale (WSRS) was used to evaluate the wrinkles before and after the injection. The average age of the 73 study patients was 46.18 years. The WSRS scores of the porcine and bovine atelocollagen-injected patients were 2.90 ± 0.71 and 2.85 ± 0.72 at the baseline and 2.15 ± 0.70 and 2.21 ± 0.67 after 6 months. There were no statistically significant differences between the two groups. Adverse effects of the porcine atelocollagen injection were seen in 12 patients, with the most common symptom being redness. This study showed that porcine atelocollagen can be used easily and without the need for the skin testing which is necessary before bovine atelocollagen injection. The efficacy of porcine atelocollagen is also similar to that of bovine atelocollagen. PMID:25272190

  2. Scientific Opinion on BSE risk in bovine intestines and mesentery

    Directory of Open Access Journals (Sweden)

    EFSA Panel on Biological Hazards (BIOHAZ

    2014-02-01

    Full Text Available Bovine intestines and mesenteries in the European Union (EU have to be removed from the food and feed chain. The opinion provides a quantitative assessment of the Bovine Spongiform Encephalopathy (BSE infectious load that might enter the food and feed chain yearly if bovine intestine and mesentery from animals born and raised in the EU would be re-allowed for consumption. Data on the evolution of the BSE infectious titre; and of the weight of histological structures accumulating BSE infectivity, were collected. The Cattle TSE Monitoring Model (C-TSEMM was used to estimate the number of BSE infected cattle entering undetected in the food and feed chain yearly. A model named TSEi was developed to estimates the BSE infectious load in tissues from infected animals at different ages and the total yearly infectious load that could enter the food and feed chain in the EU27. In BSE infected cattle, the infectivity associated with intestine and mesentery reaches its maximum in animals younger than 18 months and then progressively declines to a minimum value in animals older than 60 months. Due to the decline of the BSE prevalence in the EU, between 2007 and 2012, the yearly amount of BSE infectivity associated with intestine and mesentery (sent to destruction from animals entering the food and feed chain was reduced by a factor of 10. However, over this period, the maximum level of exposure to the BSE agent for individuals that would have consumed these tissues remained stable. Finally, the TSEi model indicated that the removal of the last four metres of the small intestine and of the caecum from the food and feed chain would result in a major reduction of the BSE exposure risk associated with intestine and mesentery in cattle.

  3. Hypertensive Encephalopathy

    Directory of Open Access Journals (Sweden)

    Mostafa SHARIFIAN

    2012-09-01

    Full Text Available How to cite this article: Sharifian M. Hypertensive Encephalopathy. Iran J Child Neurol 2012; 6(3:1-7.Hypertension is called the silent killer and vital organs such as the brain, eyes,kidneys and the heart are the targets. Seizure, central nervous system (CNShemorrhage, and cerebrovascular accident (CVA, blindness and heart attacksare the end points.The prevalence of hypertension in children is much less than adults, but evidencereveals that the source of hypertension in adulthood goes back to childhood. In70-80% of cases hypertension is due to renal diseases. In children, hypertensiveencephalopathy (HE may be the first manifestation of renal diseases. Seizure isone of the most common manifestations of HE.In this article, definitions, etiology, pathophysiology and finally the acute andchronic managements of HE will be discussed.ReferencesSawicka K, Szczyrek M, Jastrzębska I, Prasal M, ZwolakA, Jadwiga D. Hypertension – The silent killer. J Pre-Clin Clin Res 2011;5(2:43-6.Croix B, Feig DI. Childhood hypertension is not a silent disease. Pediatr Nephrol 2006 Apr;21(4:527-32.Wong TY, Mitchell P. Hypertensive retinopathy. N Engl J Med 2004 Nov;351(22:2310-7.Krzesinski JM, Cohen EP.Hypertension and the kidney.Acta Clin Belg 2007 Jan-Feb;62(1:5-14.Report of the Second Task Force on Blood Pressure Control in Children – 1987. Task Force on Blood Pressure Control in Children. National Heart, Lung, and Blood Institute, Bethesda, Maryland. Pediatrics 1987Jan;79(1:1-25.Update on the 1987 Task Force Report on High Blood Pressure in Children and Adolescents: a working group report from the National High Blood Pressure Education Program. National High Blood Pressure Education Program Working Group on Hypertension Control in Children and Adolescents. Pediatrics 1996 Oct;98(4 Pt1:649-58.Ataei N, Aghamohammadi A, Yousefi E, Hosseini M, Nourijelyani K, Tayebi M, et al. Blood pressure nomograms for school children in Iran. Pediatr Nephrol 2004 Feb;19

  4. Neuroimmune connections in jejunal and ileal Peyer's patches at various bovine ages: potential sites for prion neuroinvasion.

    Science.gov (United States)

    Defaweux, Valérie; Dorban, Gauthier; Antoine, Nadine; Piret, Joëlle; Gabriel, Annick; Jacqmot, Olivier; Falisse-Poirier, Nandini; Flandroy, Sylvain; Zorzi, Danièle; Heinen, Ernst

    2007-07-01

    During preclinical stages of cattle orally infected with bovine spongiform encephalopathy (BSE), the responsible agent is confined to ileal Peyer's patches (IPP), namely in nerve fibers and in lymph follicles, before reaching the peripheral and central nervous systems. No infectivity has been reported in other bovine lymphoid organs, including jejunal Peyer's patches (JPP). To determine the potential sites for prion neuroinvasion in IPP, we analyzed the mucosal innervation and the interface between nerve fibers and follicular dendritic cells (FDC), two dramatic influences on neuroinvasion. Bovine IPP were studied at three ages, viz., newborn calves, calves less than 12 months old, and bovines older than 24 months, and the parameters obtained were compared with those of JPP. No differences in innervation patterns between IPP and JPP were found. The major difference observed was that, in calves of less than 12 months, IPP were the major mucosal-associated lymphoid organ that possessed a large number of follicles with extended FDC networks. Using a panel of antibodies, we showed that PP in 24-month-old bovines were highly innervated at various strategic sites assumed to be involved in the invasion and replication of the BSE pathogen: the suprafollicular dome, T cell area, and germinal centers. In PP in calves of less than 12 months old, no nerve fibers positive for the neurofilament markers NF-L (70 kDa) and NF-H (200 kDa) were observed in contact with FDC. Thus, in view of the proportion of these protein subunits present in neurofilaments, the innervation of the germinal centers can be said to be an age-dependent dynamic process. This variation in innervation might influence the path of neuroinvasion and, thus, the susceptibility of bovines to the BSE agent. PMID:17406903

  5. Toxic spongiform leucoencephalopathy after inhaling heroin vapour

    International Nuclear Information System (INIS)

    This is a report of clinical, CT and MRI findings in a patient with toxic spongiform leucoencephalopathy after heroin ingestion. The disease is observed in drug addicts who inhale pre-heated heroin. The clinical onset, which usually occurs some days or even longer after the last heroin consumption, is characterized by a cerebellar syndrome. The cerebellar hemispheres, the cerebellar and cerebral peduncles and the pyramidal tract may be affected. Spongiform demyelination is the morphological substrate of the lesions, which are not contrast enhancing, hypodense on CT and hyperintense on T2-weighted MRI. The frequently perfect symmetry of the affection of functional systems points to a toxic and/or metabolic pathophysiological mechanism. (orig.)

  6. Toxic spongiform leucoencephalopathy after inhaling heroin vapour

    Energy Technology Data Exchange (ETDEWEB)

    Weber, W.; Henkes, H.; Kuehne, D. [Klinik fuer Allgemeine Roentgendiagnostik und Neuroradiologie, Alfried-Krupp-Krankenhaus, Alfried Krupp Strasse 21, D-45117, Essen (Germany); Moeller, P.; Bade, K. [Neurologische Klinik, Knappschafts-Krankenhaus, D-45657 Recklinghausen (Germany)

    1998-06-02

    This is a report of clinical, CT and MRI findings in a patient with toxic spongiform leucoencephalopathy after heroin ingestion. The disease is observed in drug addicts who inhale pre-heated heroin. The clinical onset, which usually occurs some days or even longer after the last heroin consumption, is characterized by a cerebellar syndrome. The cerebellar hemispheres, the cerebellar and cerebral peduncles and the pyramidal tract may be affected. Spongiform demyelination is the morphological substrate of the lesions, which are not contrast enhancing, hypodense on CT and hyperintense on T2-weighted MRI. The frequently perfect symmetry of the affection of functional systems points to a toxic and/or metabolic pathophysiological mechanism. (orig.) With 2 figs., 2 tabs., 26 refs.

  7. Molecular dynamics simulations capture the misfolding of the bovine prion protein at acidic pH.

    Science.gov (United States)

    Cheng, Chin Jung; Daggett, Valerie

    2014-01-01

    Bovine spongiform encephalopathy (BSE), or mad cow disease, is a fatal neurodegenerative disease that is transmissible to humans and that is currently incurable. BSE is caused by the prion protein (PrP), which adopts two conformers; PrPC is the native innocuous form, which is α-helix rich; and PrPSc is the β-sheet rich misfolded form, which is infectious and forms neurotoxic species. Acidic pH induces the conversion of PrPC to PrPSc. We have performed molecular dynamics simulations of bovine PrP at various pH regimes. An acidic pH environment induced conformational changes that were not observed in neutral pH simulations. Putative misfolded structures, with nonnative β-strands formed in the flexible N-terminal domain, were found in acidic pH simulations. Two distinct pathways were observed for the formation of nonnative β-strands: at low pH, hydrophobic contacts with M129 nucleated the nonnative β-strand; at mid-pH, polar contacts involving Q168 and D178 facilitated the formation of a hairpin at the flexible N-terminus. These mid- and low pH simulations capture the process of nonnative β-strand formation, thereby improving our understanding of how PrPC misfolds into the β-sheet rich PrPSc and how pH factors into the process. PMID:24970211

  8. Molecular Dynamics Simulations Capture the Misfolding of the Bovine Prion Protein at Acidic pH

    Directory of Open Access Journals (Sweden)

    Chin Jung Cheng

    2014-02-01

    Full Text Available Bovine spongiform encephalopathy (BSE, or mad cow disease, is a fatal neurodegenerative disease that is transmissible to humans and that is currently incurable. BSE is caused by the prion protein (PrP, which adopts two conformers; PrPC is the native innocuous form, which is α-helix rich; and PrPSc is the β-sheet rich misfolded form, which is infectious and forms neurotoxic species. Acidic pH induces the conversion of PrPC to PrPSc. We have performed molecular dynamics simulations of bovine PrP at various pH regimes. An acidic pH environment induced conformational changes that were not observed in neutral pH simulations. Putative misfolded structures, with nonnative β-strands formed in the flexible N-terminal domain, were found in acidic pH simulations. Two distinct pathways were observed for the formation of nonnative β-strands: at low pH, hydrophobic contacts with M129 nucleated the nonnative β-strand; at mid-pH, polar contacts involving Q168 and D178 facilitated the formation of a hairpin at the flexible N-terminus. These mid- and low pH simulations capture the process of nonnative β-strand formation, thereby improving our understanding of how PrPC misfolds into the β-sheet rich PrPSc and how pH factors into the process.

  9. Pathogenesis of Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Irena Ciećko-Michalska

    2012-01-01

    Full Text Available Hepatic encephalopathy can be a serious complication of acute liver failure and chronic liver diseases, predominantly liver cirrhosis. Hyperammonemia plays the most important role in the pathogenesis of hepatic encephalopathy. The brain-blood barrier disturbances, changes in neurotransmission, neuroinflammation, oxidative stress, GABA-ergic or benzodiazepine pathway abnormalities, manganese neurotoxicity, brain energetic disturbances, and brain blood flow abnormalities are considered to be involved in the development of hepatic encephalopathy. The influence of small intestine bacterial overgrowth (SIBO on the induction of minimal hepatic encephalopathy is recently emphasized. The aim of this paper is to present the current views on the pathogenesis of hepatic encephalopathy.

  10. Posterior Reversible Encephalopathy Syndrome

    OpenAIRE

    J Gordon Millichap

    2013-01-01

    Investigators at Children's Hospital of Montefiore, Albert Einstein College of Medicine, NY, determined the incidence of posterior reversible encephalopathy syndrome (PRES) in a pediatric critical care unit.

  11. Detection of Bovine Central Nervous System Tissue as Bovine Spongiform Encephalopathy Specified Risk Material in Beef by Real Time RT-PCR%牛肉中疯牛病特殊风险物质荧光RT-PCR检测方法的建立

    Institute of Scientific and Technical Information of China (English)

    史喜菊; 马贵平; 李冰玲; 杨金良; 王宇; 刘旭辉; 李炎鑫; 刘全国

    2007-01-01

    建立了检测牛肉中疯牛病特殊风险物质(主要是中枢神经组织)标记物胶原纤维酸性蛋白(GFAP)mRNA的荧光RT-PCR检测技术.结果表明,该技术具有良好的种属特异性和组织特异性,只能从牛源和羊源的中枢神经组织中检测到GFAP,猪源和禽源检测结果为阴性,而且只有脑和脊髓等中枢神经组织产生阳性反应,其它内脏组织以及不同部位牛肉检测结果均为阴性;敏感性检测结果表明,该方法最低检测限达到0.001%以下;稳定性试验结果表明,100℃加热处理30min对检测结果无明显影响,中枢神经组织在30℃以上室温可以稳定存放4 d,在4℃可以稳定冷藏2周,检测结果仍然为阳性.所建立的荧光RT-PCR技术用于牛肉中疯牛病特殊风险物质的检测具有特异性强、敏感性高、稳定性好、快速方便等优点,适合于在日常检测工作中推广使用.

  12. 76 FR 72159 - Submission for OMB Review; Comment Request

    Science.gov (United States)

    2011-11-22

    ...: Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products. OMB Control Number: 0579..., poultry, meat, other animal products and byproducts, hay, and straw into the United States in order to prevent the introduction of diseases, such as bovine spongiform encephalopathy (BSE), a...

  13. Dopaminergic agonists for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Gluud, L L; Gluud, C

    2004-01-01

    Hepatic encephalopathy may be associated with an impairment of the dopaminergic neurotransmission. Dopaminergic agonists may therefore have a beneficial effect on patients with hepatic encephalopathy.......Hepatic encephalopathy may be associated with an impairment of the dopaminergic neurotransmission. Dopaminergic agonists may therefore have a beneficial effect on patients with hepatic encephalopathy....

  14. Identification of bovine material in porcine spray-dried blood derivatives using the Polymerase Chain Reaction technique

    Directory of Open Access Journals (Sweden)

    Sánchez A.

    2004-01-01

    Full Text Available Due to the widely supported theory of bovine spongiform encephalopathy (BSE spread in cattle by contaminated animal feeds, screening of feed products has become essential. For many years, manufacturers have used blood and plasma proteins as high quality ingredients of foods for both pets and farm animals. However, in Europe, the Commission Regulation 1234/2003/EC temporally bans the use of processed animal proteins, including blood-derivative products, in feedstuffs for all farm animals which are fattened or bred for the production of food. This regulation has some exceptions, such as the use of non ruminant blood products into the feed of farm fish. Authorization of the re-introduction of these proteins into animal feed formulations, especially non ruminant proteins into the feed for non ruminant farm animals, is expected when adequate control methods to discriminate ruminant proteins exist. Currently, the number of validated methods to differentiate the species of origin for most of the animal by-products is limited. Here we report the development of a rapid and sensitive polymerase chain reaction (PCR-based assay, which allows detection of bovine or porcine specific mitochondrial DNAfrom spray-dried blood derivate products (plasma, whole blood and red cells, as a marker for bovine contamination in porcine products. Sample extracts, suitable for PCR, were easily and quickly obtained with the commercial PrepManTM Ultra reagent (Applied Biosystems. To confirm the porcine origin of the samples, primers targeting a specific region of 134 bp of the porcine cytochrome b coding sequence were designed (cytbporc1-F and cytbporc2-R. Previously published PCR primers (L8129 and H8357, specific for a 271 bp fragment of the bovine mitochondrial ATPase 8-ATPase 6 genes, were chosen to accomplish amplification of bovine DNA. The limit of detection (LOD of the bovine PCR assay was at least of 0.05% (v/v of bovine inclusion in spray-dried porcine plasma or red

  15. South-East Asia bovine populations and the Japanese cattle breeds do not harbour the E211K variant of the PRNP

    Directory of Open Access Journals (Sweden)

    George Msalya

    2014-02-01

    Full Text Available An important outcome of intensive worldwide Bovine spongiform encephalopathy (BSE obtained with the surveillance by The National Creutzfeldt-Jakob Disease Surveillance Unit (http://www.cjd.ed.ac.uk/figures. htm, has been the detection of atypical BSE in cattle. The discovery of a prion protein gene (PRNP E211K variant in an atypical BSE case is particularly remarkable because it is analogous to the most common pathogenic mutation in humans (E200K, which causes hereditary Creutzfeldt-Jakob disease (CJD. Knowledge of the distribution and frequency of PRNP E211K variants in cattle populations is critical for understanding and managing atypical BSE. This study was carried out to investigate the prevalence of the E211K variant in the South-East Asia bovine populations and in the Japanese cattle breeds. It was discovered that E211K variant was monomorphic for a G allele and the GG genotype in the 745 animals analyzed in this study. Therefore, neither the Bos indicus nor the Bos taurus animals analyzed are presently known to harbor the 211K variant predicting that the number of carriers for this variant will also be vanishingly low.

  16. An overview of animal prion diseases

    OpenAIRE

    Imran Muhammad; Mahmood Saqib

    2011-01-01

    Abstract Prion diseases are transmissible neurodegenerative conditions affecting human and a wide range of animal species. The pathogenesis of prion diseases is associated with the accumulation of aggregates of misfolded conformers of host-encoded cellular prion protein (PrPC). Animal prion diseases include scrapie of sheep and goats, bovine spongiform encephalopathy (BSE) or mad cow disease, transmissible mink encephalopathy, feline spongiform encephalopathy, exotic ungulate spongiform encep...

  17. 76 FR 38667 - Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting

    Science.gov (United States)

    2011-07-01

    ... 20877. For those unable to attend in person, the meeting will also be Web cast. The Web cast will be....yorkcast.com/webcast/Viewer/?peid=8477143b2da5442a8192731eccde3b7a1d . Contact Person: Bryan Emery or... check the Agency's Web site and call the appropriate advisory committee hot line/phone line to...

  18. Overcoming RNA inhibition in the fluorescent polymerase chain reaction assay to enhance detection of bovine DNA in cattle feeds.

    Science.gov (United States)

    Sawyer, Mary; Rensen, Gabriel; Smith, Wayne; Yee, Melanie; Wong, Alice; Osburn, Bennie; Cullor, James

    2004-01-01

    The practice of incorporating mammalian protein in ruminant feeds was banned in the United States in 1997 as a measure to avoid transmission of bovine spongiform encephalopathy (BSE). A sensitive means of identifying the banned additives in feeds would be by detection of species-specific DNA using the polymerase chain reaction (PCR). However, problems may arise in the PCR due to the presence of inhibitory substances. Using human DNA as an internal PCR control, inhibitory substances were evident in the DNA extraction products of cattle feeds. The results of heating experiments excluded enzymes as a cause of inhibition, and spectrophotometric calculations suggested the possibility of RNA contamination. Co-electrophoresis of untreated and RNAse digested extracts confirmed the presence of RNA in the undigested product. Seven cattle feeds were spiked with predetermined amounts of bovine meat and bone meal (BMBM). The DNA extracted products were treated with RNAse and the bovine specific mitochondrial DNA (B-mtDNA) was amplified by PCR. The minimum level of detection of B-mtDNA was influenced by RNAse treatment and feed composition. RNAse treatment decreased false-negative results overall by 75%. False-negative results were decreased 100% in the higher BMBM concentrations and 50% in the lower BMBM concentrations. Also, each cattle feed was spiked to attain a 2% wt/wt concentration with each swine, fish, sheep, or poultry product, or cattle dried blood. Amplification of B-mtDNA occurred only with the cattle dried blood and only in three feeds in which B-mtDNA was detected at the only level tested (2%). A commercial immunochromotographic assay (Neogen) detected the spiked BMBM in only one of the seven feeds and only at the upper concentration (1%). PMID:15992269

  19. Spatial correlation between the prevalence of transmissible spongiform diseases and British soil geochemistry.

    Science.gov (United States)

    Imrie, C E; Korre, A; Munoz-Melendez, G

    2009-02-01

    Transmissible spongiform encephalopathies (TSEs) are a group of fatal neurological conditions affecting a number of mammals, including sheep and goats (scrapie), cows (BSE), and humans (Creutzfeldt-Jakob disease). The diseases are widely believed to be caused by the misfolding of the normal prion protein to a pathological isoform, which is thought to act as an infectious agent. Outbreaks of the disease are commonly attributed to contaminated feed and genetic susceptibility. However, the implication of copper and manganese in the pathology of the disease, and its apparent geographical clustering, have prompted suggestions of a link with trace elements in the environment. Nevertheless, studies of soils at regional scales have failed to provide evidence of an environmental risk factor. This study uses geostatistical techniques to investigate the correlations between the distribution of TSE prevalence and soil geochemical variables across the UK according to different spatial scales. A similar spatial pattern in scrapie and BSE occurrence is identified, which may be linked with increasing pH and total organic carbon, and decreasing iodine concentration. However, the pattern also resembles that of the density of dairy farming. Nevertheless, despite the low spatial resolution of the TSE data available for this study, the fact that significant correlations are detected indicates there is a possibility of a link between soil geochemistry, scrapie, and BSE. It is suggested that further investigations of the prevalence of TSE and environmental exposure to trace metals should take into account the factors affecting their bioavailability. PMID:18427934

  20. Management of Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    G. Wright

    2011-01-01

    Full Text Available Hepatic encephalopathy (HE, the neuropsychiatric presentation of liver disease, is associated with high morbidity and mortality. Reduction of plasma ammonia remains the central therapeutic strategy, but there is a need for newer novel therapies. We discuss current evidence supporting the use of interventions for both the general management of chronic HE and that necessary for more acute and advanced disease.

  1. Diabetic encephalopathy: Pathogenesis, clinical manifestations, therapy approaches

    Directory of Open Access Journals (Sweden)

    I. Kh. Khairullin

    2014-07-01

    Full Text Available The paper considers the epidemiology, morphology, and clinical manifestations of diabetic encephalopathy. It shows the differences of diabetic encephalopathy in types 1 and 2 diabetes mellitus. Pathogenetic treatment options for diabetic encephalopathy are given.

  2. Diabetic encephalopathy: Pathogenesis, clinical manifestations, therapy approaches

    OpenAIRE

    I. Kh. Khairullin; S. T. Zyangirova; Yu. N. Isayeva; O. R. Esin

    2014-01-01

    The paper considers the epidemiology, morphology, and clinical manifestations of diabetic encephalopathy. It shows the differences of diabetic encephalopathy in types 1 and 2 diabetes mellitus. Pathogenetic treatment options for diabetic encephalopathy are given.

  3. Diabetic encephalopathy: Pathogenesis, clinical manifestations, therapy approaches

    Directory of Open Access Journals (Sweden)

    I. Kh. Khairullin

    2012-01-01

    Full Text Available The paper considers the epidemiology, morphology, and clinical manifestations of diabetic encephalopathy. It shows the differences of diabetic encephalopathy in types 1 and 2 diabetes mellitus. Pathogenetic treatment options for diabetic encephalopathy are given.

  4. La qualité différenciée de la viande bovine. La nécessaire stratégie d'innovation

    Directory of Open Access Journals (Sweden)

    Sans P.

    2003-01-01

    Full Text Available Differentiation of beef meat by quality. The necessary strategy of innovation. The beef meat supply chain faces with a very delicate situation: on the long term, red meat consumption in the Western countries is declining to benefit of meats of monogastrics (poultry and pork. Moreover, since the middle of the 1990s in France, a major sanitary crisis, the bovine spongiform encephalopathy, leads many consumers to stop or reduce their consumption of beef. Vis-a-vis this situation, the stakeholders have to react strongly. The purpose of this contribution is to show how the innovation, in all its forms, constitutes one of the means to restore the lost confidence. First of all, the author specially focuses on the various possible strategies while insisting on the innovations about products (technical and marketing and organisation. A historical analysis of the development of the bovine meat processing industry makes it possible to highlight its current forces and weaknesses. It leads to the acknowledgement of the recent development of pre-packaged units of sale made by the industry and of a larger range of processed products that allow communication on private marks. On the organisational level, these changes are accompanied by deep evolutions on the definition of the processes and by the emergence of standardisation (in order to ensure a complete traceability for example by tracing and tracking. In a second time, the author shows how the recent changes in the processing sector of the bovine meat lead to an acceleration of industrialisation: the implementation of standards, the diffusion of schedules coming from quality insurance systems (like guide of good practices for example as well as the requirements of the purchasers of supermarkets strongly boost the rationalisation of the production.

  5. Epithelial and endothelial expression of the green fluorescent protein reporter gene under the control of bovine prion protein (PrP) gene regulatory sequences in transgenic mice

    Science.gov (United States)

    Lemaire-Vieille, Catherine; Schulze, Tobias; Podevin-Dimster, Valérie; Follet, Jérome; Bailly, Yannick; Blanquet-Grossard, Françoise; Decavel, Jean-Pierre; Heinen, Ernst; Cesbron, Jean-Yves

    2000-05-01

    The expression of the cellular form of the prion protein (PrPc) gene is required for prion replication and neuroinvasion in transmissible spongiform encephalopathies. The identification of the cell types expressing PrPc is necessary to understanding how the agent replicates and spreads from peripheral sites to the central nervous system. To determine the nature of the cell types expressing PrPc, a green fluorescent protein reporter gene was expressed in transgenic mice under the control of 6.9 kb of the bovine PrP gene regulatory sequences. It was shown that the bovine PrP gene is expressed as two populations of mRNA differing by alternative splicing of one 115-bp 5' untranslated exon in 17 different bovine tissues. The analysis of transgenic mice showed reporter gene expression in some cells that have been identified as expressing PrP, such as cerebellar Purkinje cells, lymphocytes, and keratinocytes. In addition, expression of green fluorescent protein was observed in the plexus of the enteric nervous system and in a restricted subset of cells not yet clearly identified as expressing PrP: the epithelial cells of the thymic medullary and the endothelial cells of both the mucosal capillaries of the intestine and the renal capillaries. These data provide valuable information on the distribution of PrPc at the cellular level and argue for roles of the epithelial and endothelial cells in the spread of infection from the periphery to the brain. Moreover, the transgenic mice described in this paper provide a model that will allow for the study of the transcriptional activity of the PrP gene promoter in response to scrapie infection.

  6. Lead encephalopathy in adults

    Directory of Open Access Journals (Sweden)

    Janapareddy Vijaya Bhaskara Rao

    2014-01-01

    Full Text Available Lead poisoning is a common occupational health hazard in developing countries. We report the varied clinical presentation, diagnostic and management issues in two adult patients with lead encephalopathy. Both patients worked in a battery manufacturing unit. Both patients presented with seizures and one patient also complained of abdominal colic and vomiting. Both were anemic and a lead line was present. Blood lead level in both the patients was greater than 25 µg/dl. Magnetic resonance imaging of brain revealed bilateral symmetric involvement of the thalamus, lentiform nucleus in both patients and also the external capsules, sub-cortical white matter in one patient. All these changes, seen as hyperintensities in T2-weighted images suggested demyelination. They were advised avoidance of further exposure to lead and were treated with anti-epileptics; one patient also received D-penicillamine. They improved well on follow-up. Lead encephalopathy is an uncommon but important manifestation of lead toxicity in adults.

  7. Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder. Symptoms usually start around age 60. Memory problems, behavior changes, vision ... during a medical procedure Cattle can get a disease related to CJD called bovine spongiform encephalopathy (BSE) ...

  8. Variant Creutzfeldt-Jakob Disease (vCJD)

    Science.gov (United States)

    ... disease in cows, bovine spongiform encephalopathy (BSE or 'mad cow' disease), is the same agent responsible for the outbreak ... US Clinical and Pathologic Characteristics Relationship with BSE (Mad Cow Disease) Surveillance for vCJD Resources News and Highlights Updated: ...

  9. Relationship with BSE (Mad Cow Disease)

    Science.gov (United States)

    ... Wasting Disease (CWD) Prion Diseases Relationship with BSE (Mad Cow Disease) Evidence Recommend on Facebook Tweet Share Compartir There ... Jakob Disease (CJD) Bovine Spongiform Encephalopathy (BSE), or Mad Cow Disease Chronic Wasting Disease (CWD) File Formats Help: How ...

  10. Genetics Home Reference: prion disease

    Science.gov (United States)

    ... as bovine spongiform encephalopathy (BSE) or, more commonly, "mad cow disease." Another example of an acquired human prion disease ... forms of prion disease , including kuru and variant Creutzfeldt-Jakob disease, are not inherited. Related Information What does it ...

  11. Isolation of protein-associated circular DNA from healthy cattle serum.

    Science.gov (United States)

    Funk, Mathis; Gunst, Karin; Lucansky, Vincent; Müller, Hermann; Zur Hausen, Harald; de Villiers, Ethel-Michele

    2014-01-01

    Three replication-competent single-stranded DNA molecules sharing nucleotide similarity to transmissible spongiform encephalopathy (TSE)-associated isolate Sphinx 2.36 were isolated from healthy bovine serum. PMID:25169856

  12. 78 FR 1824 - Notice of Request for Approval of an Information Collection; National Veterinary Services...

    Science.gov (United States)

    2013-01-09

    ... Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy... information collection associated with National Veterinary Services Laboratories diagnostic support for the... Staff Veterinarian, Veterinary Services, APHIS, 4700 River Road Unit 43, Riverdale, MD 20737; (301)...

  13. Encephalopathy for prions

    International Nuclear Information System (INIS)

    The encephalopathy spongyform for prions are neuro degenerative illness that can be sporadic or transferable, for infectious or hereditary mechanisms. Their investigation has outlined enormous challenges and in the historical journey in search of its cause two doctors have received the Nobel prize of medicine Carleton Gajdusek, for its works in New Guinea where it described the infectious transmission for cannibalistic rites that it took to studies of experimental transmission in chimpanzees and to its theory of the slow virus; later on, Stanley Prusiner developed its experimental works in hamsters, throwing to the neurobiology the prion concept (particles infectious proteinaceous not viral). The paper narrates the history of a patient that died in the San Juan de Dios of Bogota Hospital by cause of this prionic illness and clinical and pathological aspects are discussed

  14. Hepatic encephalopathy: historical remarks.

    Science.gov (United States)

    Amodio, Piero

    2015-03-01

    The history of hepatic encephalopathy (HE) is briefly reviewed since the beginning of western medicine by Hippocrates. For about 2000 years the main evidence was the mere association between jaundice, fever and delirium. A clear link between delirium and cirrhosis was proven in the 17th century by Morgagni. In subsequent times the focus was manly the descriptions of symptoms and the only pathophysiological improvement was the evidence that jaundice, per se, does not alter brain function. Only at the end of the 19th century Hann et al proved the role of portal-systemic shunt and pf nitrogenous derivates in the pathophysiology of the syndrome. A terrific development of knowledge occurred in the last 60 years, after the works of Sherlock in London. Nowadays some consensus about HE was reached, so that new developments will likely occur. PMID:26041956

  15. Diabetic encephalopathy: a cerebrovascular disorder?

    NARCIS (Netherlands)

    Manschot, S.M.

    2006-01-01

    Animal study: The aim was to investigate the role of vascular disturbances in the development of experimental diabetic encephalopathy. We describe the effects of treatment with the Angiotensin Converting Enzyme(ACE)-inhibitor enalapril (treatment aimed at the vasculature)

  16. Spongiform leucoencephalopathy following intravenous heroin abuse: Radiological and histopathological findings

    International Nuclear Information System (INIS)

    A case of spongiform leucoencephalopathy in a known intravenous heroin abuser is presented. To our knowledge, this is the only case of heroin-related spongiform leucoencephalopathy reported in Australia. The relationship to intravenous rather than inhaled heroin is particularly unusual with only one other possible case documented in the literature. The imaging and histopathological findings are described. Neurological examination revealed disorientation in time and place, memory loss and cognitive impairment but no focal signs. Biochemical and haematological profiles were normal. Viral serology was positive for hepatitis C but negative for hepatitis B and human immunodeficiency virus (HIV). Cerebral CT revealed diffuse symmetrical hypodensity of the cerebral white matter. The ventricles and subarachnoid spaces were of normal size. Magnetic resonance imaging showed diffuse symmetrical signal abnormality in the cerebral white matter. These changes were hyperintense on proton density, T2-weighted, modified T2-weighted (FLAIR) and diffusion-weighted images. T1 -weighted scans showed corresponding hypointensity. There was no enhancement after intravenous gadolinium. Cerebral spinal fluid (CSF) specimens were negative for a variety of virological, immunological and bacteriological markers. No viral or bacterial growth was demonstrated. Oligoclonal bands for multiple sclerosis and Protein 134 for Wilson's disease were negative. Right frontal brain biopsy showed spongiform white matter and degenerative change with prominent fibrous gliosis. In severely affected areas, loss of normal myelin staining and axonal loss were present, accompanied by scattered foamy macrophages. Loss of oligodendroglial nuclei was also present. There was no evidence of inflammation or progressive multifocal leucoencephalopathy. No bacteria or virus particles were seen on electron microscopic examination of the brain tissue. Following the biopsy, the patient discharged himself from hospital and the

  17. Benzodiazepine receptor antagonists for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Gluud, L L; Gluud, C

    2004-01-01

    Hepatic encephalopathy may be associated with accumulation of substances that bind to a receptor-complex in the brain resulting in neural inhibition. Benzodiazepine receptor antagonists may have a beneficial effect on patients with hepatic encephalopathy.......Hepatic encephalopathy may be associated with accumulation of substances that bind to a receptor-complex in the brain resulting in neural inhibition. Benzodiazepine receptor antagonists may have a beneficial effect on patients with hepatic encephalopathy....

  18. Non-Hyperammonemic valproate encephalopathy.

    Science.gov (United States)

    Farooq, Omar; Zunga, Pervaiz M; Dar, Mohd I; Rather, Abdul Q; Rashid, Samia; Basu, Javid; Dar, Ishrat H; Ashraf, Mohd

    2014-04-01

    A 21-year-old male known case of primary hypothyroidism, Seizure disorder sequelae of an old trauma receiving sodium valproate, clobazam and phenobarbitone for control of Generalized tonic clonic seizures reported to neurology OPD with history of altered sensorium and gait unsteadiness for 1 week with history of hike in valproate dose 2 weeks before. On examination he was drowsy. Neurological examination was unremarkable except for gait unsteadiness and ataxia. Patient was admitted and evaluated for acute worsening. All (the) biochemical parameters including complete blood count, liver function tests, kidney function tests, routine urine examination, arterial blood gas analysis, blood and urine culture tests were normal. CSF analysis was also normal. Repeat MRI brain was also done which depicted all old changes with no fresh changes which will account for worsening of his sensorium. EEG was suggestive of diffuse encephalopathy. Thyroid function tests were also normal. Valproate encephalopathy was suspected and Valproate was empirically stopped and he was put on levetiracetam and phenytoin. His sensorium improved rapidly after stoppage of valproate with normalization of EEG. Serum valproate Levels were high with serum ammonia levels were in the normal range. We made the inference of nonhyperammoneamic valproate encephalopathy. This case highlights the existence of non-hyperammonemic valproate induced encephalopathy, suggesting mechanisms other than hyperammonemia responsible for this encephalopathy. PMID:25206067

  19. Dopamine agents for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Junker, Anders Ellekær; Als-Nielsen, Bodil; Gluud, Christian;

    2014-01-01

    BACKGROUND: Patients with hepatic encephalopathy may present with extrapyramidal symptoms and changes in basal ganglia. These changes are similar to those seen in patients with Parkinson's disease. Dopamine agents (such as bromocriptine and levodopa, used for patients with Parkinson's disease) have...... therefore been assessed as a potential treatment for patients with hepatic encephalopathy. OBJECTIVES: To evaluate the beneficial and harmful effects of dopamine agents versus placebo or no intervention for patients with hepatic encephalopathy. SEARCH METHODS: Trials were identified through the Cochrane...... the trials followed participants after the end of treatment. Only one trial reported adequate bias control; the remaining four trials were considered to have high risk of bias. Random-effects model meta-analyses showed that dopamine agents had no beneficial or detrimental effect on hepatic...

  20. Spongiform leucoencephalopathy after inhaling heroin vapor: CT and MRI findings

    International Nuclear Information System (INIS)

    Objective: To explore CT and MRI features of spongiform leucoencephalopathy after inhaling 'heroin' pyrolysate, and to improve the diagnostic ability of the disease. Methods: Four patients, which inhaled heroin vapor, received the CT or MRI pre- and post-contrast scanning. MR sequences included conventional SE T1WI, T2WI, fluid attenuation inverse recover (FLAIR), and MRS. Results: All 4 cases had similar symmetrical lesions involving the cerebellum, lateral brainstem, cerebral peduncles, posterior limbs of internal capsule, splenium of corpus callosum, medial lemniscuses, and posterior cerebral white mattes without enhancement. The lesions showed hypodense on CT and hypointense on T1WI, hyperintense on T2WI and FLAIR. Cerebellar lesions were more serious than cerebral one, and the parieto-occipital lesions were serious than frontal one. MRS showed that the abnormalities in the white matter were degenerative changes, not necrosis. Conclusion: CT and MRI findings of this diseases are characteristic. Combined with the history, the disease can be diagnosed

  1. Psychopathology and Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    João Gama Marques

    2013-12-01

    Full Text Available Since Hippocrates that neuropsychiatric illness secondary to liver disease fascinates physicians, but only in the XIX century Marcel Nencki and Ivan Pavlov suggested the relation between high concentrations of ammonia and Hepatic Encephalopathy (HE. The reaction of ammonia and glutamate (origins glutamine, “the Trojan Horse of neurotoxicity of ammonia continues to be the main responsible for the neurologic lesions, recently confirmed by neurochemistry and neuroimagiology studies. Glutamine starts the inflammatory reaction at the central nervous sys- tem but other important actors seem to be manganese and the neurotransmitters systems of GABA and endocanabinoids. Nowadays there are three different etiologic big groups for HE: type A associated with acute liver failure; type B associated with portosystemic bypass; and type C associated with cirrhosis of the liver. The staging of HE is still based on classic West Haven system, but a latent Grade 0 was introduced (the so called minimal HE; remaining the aggra- vating HE from Grade 1 (subtle changes at clinical examination to Grade 4 (coma. In this work a bibliographic review was made on 30 of the most pertinent and recent papers, focusing in psychopathology, physiopathology, etiology and staging of this clinical entity transversal to Psychiatry and Gastroenterology. Alterations are described in vigility and conscience like temporal, spatial and personal disorientation. Attention, concentration and memory are impaired very early, on latent phase and can be accessed through neuropsychological tests. Mood oscillates between euphoric and depressive. Personality changes begin obviously and abruptly or in a subtle and insidious way. There can be changes in perception like visual hallucinations or even of acoustic-verbal. The thought disorders can be of delusional type, paranoid, systematized or not, but also monothematic ala Capgras Syndrome. Speech can be accelerated, slowed down or completely in

  2. Cerebral Palsy and Neonatal Encephalopathy

    OpenAIRE

    J Gordon Millichap

    2005-01-01

    The type and severity of cerebral palsy (CP) and pattern of associated disability in children with or without preceding neonatal encephalopathy (NE) were compared in a population-based case-control study of patients followed for 6 years at the Children’s Hospital, Westmead, Sydney, Australia.

  3. Prussian blue caged in spongiform adsorbents using diatomite and carbon nanotubes for elimination of cesium.

    Science.gov (United States)

    Hu, Baiyang; Fugetsu, Bunshi; Yu, Hongwen; Abe, Yoshiteru

    2012-05-30

    We developed a spongiform adsorbent that contains Prussian blue, which showed a high capacity for eliminating cesium. An in situ synthesizing approach was used to synthesize Prussian blue inside diatomite cavities. Highly dispersed carbon nanotubes (CNTs) were used to form CNT networks that coated the diatomite to seal in the Prussian blue particles. These ternary (CNT/diatomite/Prussian-blue) composites were mixed with polyurethane (PU) prepolymers to produce a quaternary (PU/CNT/diatomite/Prussian-blue), spongiform adsorbent with an in situ foaming procedure. Prussian blue was permanently immobilized in the cell walls of the spongiform matrix and preferentially adsorbed cesium with a theoretical capacity of 167 mg/g cesium. Cesium was absorbed primarily by an ion-exchange mechanism, and the absorption was accomplished by self-uptake of radioactive water by the quaternary spongiform adsorbent. PMID:22464752

  4. Rifaximin in the treatment of hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Iadevaia MD

    2011-12-01

    Full Text Available Maddalena Diana Iadevaia, Anna Del Prete, Claudia Cesaro, Laura Gaeta, Claudio Zulli, Carmelina LoguercioDepartment of Internistica Clinica e Sperimentale, F Magrassi e A Lanzara, Hepatogastroenterology Unit, Second University of Naples, Naples, ItalyAbstract: Hepatic encephalopathy is a challenging complication in patients with advanced liver disease. It can be defined as a neuropsychiatric syndrome caused by portosystemic venous shunting, ranging from minimal to overt hepatic encephalopathy or coma. Its pathophysiology is still unclear, although increased levels of ammonia play a key role. Diagnosis of hepatic encephalopathy is currently based on specific tests evaluating the neuropsychiatric state of patients and their quality of life; the severity of hepatic encephalopathy is measured by the West Haven criteria. Treatment of hepatic encephalopathy consists of pharmacological and corrective measures, as well as nutritional interventions. Rifaximin received approval for the treatment of hepatic encephalopathy in 2010 because of its few side effects and pharmacological benefits. The aim of this work is to review the use and efficacy of rifaximin both in acute and long-term management of hepatic encephalopathy. Treatment of overt hepatic encephalopathy involves management of the acute episode as well as maintenance of remission in those patients who have previously experienced an episode, in order to improve their quality of life. The positive effect of rifaximin in reducing health care costs is also discussed.Keywords: acute hepatic encephalopathy, recurrent hepatic encephalopathy, rifaximin, lactulose, cost, health-related quality of life

  5. Pharmacotherapy for hepatic encephalopathy.

    Science.gov (United States)

    Phongsamran, Paula V; Kim, Jiwon W; Cupo Abbott, Jennifer; Rosenblatt, Angela

    2010-06-18

    Hepatic encephalopathy (HE) is a challenging clinical complication of liver dysfunction with a wide spectrum of neuropsychiatric abnormalities that range from mild disturbances in cognitive function and consciousness to coma and death. The pathogenesis of HE in cirrhosis is complex and multifactorial, but a key role is thought to be played by circulating gut-derived toxins of the nitrogenous compounds, most notably ammonia. Therapeutic treatment options for HE are currently limited and have appreciable risks and benefits associated with their use. Management of HE primarily involves avoidance of precipitating factors, limitation of dietary protein intake, and administration of various ammonia-lowering therapies such as non-absorbable disaccharides and select antimicrobial agents. Non-absorbable disaccharides, such as lactulose, have traditionally been regarded as first-line pharmacotherapy for patients with HE. However, multiple adverse events have been associated with their use. In addition, recent literature has questioned the true efficacy of the disaccharides for this indication. Neomycin, metronidazole and vancomycin may be used as alternative treatments for patients intolerant or unresponsive to non-absorbable disaccharides. Antimicrobials reduce bacterial production of ammonia and other bacteria-derived toxins through suppression of intestinal flora. Neomycin has been reported to be as effective as lactulose, and similar efficacy has been reported with vancomycin and metronidazole for the management of HE. However, the adverse effects frequently associated with these antimicrobials limit their use as first-line pharmacological agents. Neomycin is the most commonly used antimicrobial for HE and, although poorly absorbed, systemic exposure to the drug in sufficient amounts causes hearing loss and renal toxicity. Long-term neomycin therapy requires annual auditory testing and continuous monitoring of renal function. Long-term use of metronidazole has been

  6. Hepatic encephalopathy: clinical and experimental studies

    OpenAIRE

    Rijt, Carin

    1991-01-01

    textabstractThe pathogenesis of hepatic encephalopathy is still unsolved. Therapy, therefore, is often insufficient. For the development of effective, new therapies insight into the disease-inducing substrates and the mechanisms of its toxic actions in the central nervous system ·are required. For both studies on pathogenesis and therapy of hepatic encephalopathy, methods for the quantitation of its severity are needed. For the measurement of hepatic encephalopathy clinical grading, conventio...

  7. Management of Overt Hepatic Encephalopathy

    OpenAIRE

    Sharma, Praveen; Sharma, Barjesh C.

    2014-01-01

    Hepatic encephalopathy (HE) is an important complication of cirrhosis with significant morbidity and mortality. Management of HE primarily involves avoidance of precipitating factors and administration of various ammonia-lowering therapies such as non-absorbable disaccharides, antimicrobial agents like rifaximin and l-ornithine l-aspartate. The non-absorbable disaccharides which include lactulose and lactitol are considered the first-line therapy for the treatment of HE and in primary and sec...

  8. Cerebral palsy and neonatal encephalopathy.

    OpenAIRE

    Gaffney, G; Flavell, V; Johnson, A; Squier, M.; Sellers, S

    1994-01-01

    A retrospective cohort study was carried out to test the hypothesis that children born at term with cerebral palsy with signs of neurological dysfunction preceded by depression at birth (termed neonatal encephalopathy) differ from those without such signs in the frequency of antenatal and perinatal factors, and in the severity and characteristics of their impairment and disability. The study was carried out in the area covered by Oxford Regional Health Authority. Antenatal, intrapartum, neona...

  9. Perinatal Hypoxic-Ischemic Encephalopathy

    OpenAIRE

    Ming-Chi Lai; San-Nan Yang

    2010-01-01

    Perinatal hypoxic-ischemic encephalopathy (HIE) is an important cause of brain injury in the newborn and can result in long-term devastating consequences. Perinatal hypoxia is a vital cause of long-term neurologic complications varying from mild behavioural deficits to severe seizure, mental retardation, and/or cerebral palsy in the newborn. In the mammalian developing brain, ongoing research into pathophysiological mechanism of neuronal injury and therapeutic strategy after perinatal hypoxia...

  10. An overview of animal prion diseases.

    Science.gov (United States)

    Imran, Muhammad; Mahmood, Saqib

    2011-01-01

    Prion diseases are transmissible neurodegenerative conditions affecting human and a wide range of animal species. The pathogenesis of prion diseases is associated with the accumulation of aggregates of misfolded conformers of host-encoded cellular prion protein (PrPC). Animal prion diseases include scrapie of sheep and goats, bovine spongiform encephalopathy (BSE) or mad cow disease, transmissible mink encephalopathy, feline spongiform encephalopathy, exotic ungulate spongiform encephalopathy, chronic wasting disease of cervids and spongiform encephalopathy of primates. Although some cases of sporadic atypical scrapie and BSE have also been reported, animal prion diseases have basically occurred via the acquisition of infection from contaminated feed or via the exposure to contaminated environment. Scrapie and chronic wasting disease are naturally sustaining epidemics. The transmission of BSE to human has caused more than 200 cases of variant Cruetzfeldt-Jacob disease and has raised serious public health concerns. The present review discusses the epidemiology, clinical neuropathology, transmissibility and genetics of animal prion diseases. PMID:22044871

  11. Valproic Acid Induced Hyperammonaemic Encephalopathy

    International Nuclear Information System (INIS)

    Objective: To observe clinical and laboratory features of valproic acid-induced hyperammonaemic encephalopathy in patients taking valproic acid. Methods: Observational study was conducted at the Neurology Department, Dow University of Health Sciences, Civil Hospital, Karachi, from February 26, 2010 to March 20, 2011. Ten patients on valproic acid therapy of any age group with idiopathic or secondary epilepsy, who presented with encephalopathic symptoms, were registered and followed up during the study. Serum ammonia level, serum valproic acid level, liver function test, cerebrospinal fluid examination, electroencephalogram and brain imaging of all the patients were done. Other causes of encephalopathy were excluded after clinical and appropriate laboratory investigations. Microsoft Excel 2007 was used for statistical analysis. Results: Hyperammonaemia was found in all patients with encephalopathic symptoms. Rise in serum ammonia was independent of dose and serum level of valproic acid. Liver function was also found to be normal in 80% (n=8) of the patients. Valproic acid was withdrawn in all patients. Three (30%) patients improved only after the withdrawal of valproic acid. Six (60%) patients improved after L-Carnitine replacement, one (10%) after sodium benzoate. On followup, serum ammonia had reduced to normal in five (50%) patients and to more than half of the baseline level in two (20%) patients. Three (30%) patients were lost to followup after complete clinical improvement. Conclusion: Within therapeutic dose and serum levels, valproic acid can cause symptomatic hyperammonaemia resulting in encephalopathy. All patients taking valproic acid presenting with encephalopathic symptoms must be monitored for the condition. (author)

  12. Prion protein NMR structure and species barrier for prion diseases

    OpenAIRE

    Billeter, Martin; Riek, Roland; Wider, Gerhard; Hornemann, Simone; Glockshuber, Rudi; Wüthrich, Kurt

    1997-01-01

    The structural basis of species specificity of transmissible spongiform encephalopathies, such as bovine spongiform encephalopathy or “mad cow disease” and Creutzfeldt–Jakob disease in humans, has been investigated using the refined NMR structure of the C-terminal domain of the mouse prion protein with residues 121–231. A database search for mammalian prion proteins yielded 23 different sequences for the fragment 124–226, which display a high degree of sequence identity and show relevant amin...

  13. Prion Diseases as Transmissible Zoonotic Diseases

    OpenAIRE

    Lee, Jeongmin; Kim, Su Yeon; Hwang, Kyu Jam; Ju, Young Ran; Woo, Hee-Jong

    2013-01-01

    Prion diseases, also called transmissible spongiform encephalopathies (TSEs), lead to neurological dysfunction in animals and are fatal. Infectious prion proteins are causative agents of many mammalian TSEs, including scrapie (in sheep), chronic wasting disease (in deer and elk), bovine spongiform encephalopathy (BSE; in cattle), and Creutzfeldt–Jakob disease (CJD; in humans). BSE, better known as mad cow disease, is among the many recently discovered zoonotic diseases. BSE cases were first r...

  14. Prion Diseases and the Gastrointestinal Tract

    OpenAIRE

    Davies, Gwynivere A; Bryant, Adam R; John D. Reynolds; Jirik, Frank R.; Keith A Sharkey

    2006-01-01

    The gastrointestinal (GI) tract plays a central role in the pathogenesis of transmissible spongiform encephalopathies. These are human and animal diseases that include bovine spongiform encephalopathy, scrapie and Creutzfeldt-Jakob disease. They are uniformly fatal neurological diseases, which are characterized by ataxia and vacuolation in the central nervous system. Alhough they are known to be caused by the conversion of normal cellular prion protein to its infectious conformational isoform...

  15. CT and MR imaging of spongiform leukoencephalopathy after heroin vapor inhalation

    International Nuclear Information System (INIS)

    Objective: To summarize the CT and MRI features of spongiform leucoencephalopathy after heroin vapor inhalation. Methods: The clinical characteristic, CT and MRI of 5 cases and biopsy of 1 case with heroin vapor inhalation induced spongiform leukoencephalopathy were analyzed. Results: CT and MRI appearances of spongiform leucoencephalopathy after heroin vapor inhalation were as follow: (1) Brain CT and MRI showed extensive symmetrical white matter lesions within bilateral cerebral and cerebellar hemispheres, the brainstem, and posterior limbs of the internal capsule. The cerebellum was heavily impaired. The most outstanding characteristics of the lesion was ring-like or butterfly-like in shape with clear margin in cerebellar hemispheres. (2) The lesion was bilateral and symmetrical. The margin of the lesion was clear. (3) A computed tomographic scan showed hypointense lesions. T1-weighted magnetic resonance imaging (MRI) showed hypointense lesions and T2-weighted MRI showed hyperintense lesions. (4) No enhancement with Gd-DTPA was revealed. (5) Spongiform vacuoles degeneration of white matter was the main morphological change by biopsy. Conclusion: Spongiform leukoencephalopathy should be considered if a patient showed acute cerebellar signs who had a history of inhaling heroin pyrolysate. The imaging manifestation of this disease is typical and the diagnosis can be made by head CT or MRI

  16. Prussian blue caged in spongiform adsorbents using diatomite and carbon nanotubes for elimination of cesium

    International Nuclear Information System (INIS)

    Highlights: ► Prussian blue was sealed in cavities of diatomite using carbon nanotubes. ► The caged Prussian blue after being permanently immobilized in polyurethane spongy showed a 167 mg/g capability for absorbing cesium. ► Cesium elimination was accomplished by simply adding the Prussian-blue based spongiform adsorbent to radioactive water. - Abstract: We developed a spongiform adsorbent that contains Prussian blue, which showed a high capacity for eliminating cesium. An in situ synthesizing approach was used to synthesize Prussian blue inside diatomite cavities. Highly dispersed carbon nanotubes (CNTs) were used to form CNT networks that coated the diatomite to seal in the Prussian blue particles. These ternary (CNT/diatomite/Prussian-blue) composites were mixed with polyurethane (PU) prepolymers to produce a quaternary (PU/CNT/diatomite/Prussian-blue), spongiform adsorbent with an in situ foaming procedure. Prussian blue was permanently immobilized in the cell walls of the spongiform matrix and preferentially adsorbed cesium with a theoretical capacity of 167 mg/g cesium. Cesium was absorbed primarily by an ion-exchange mechanism, and the absorption was accomplished by self-uptake of radioactive water by the quaternary spongiform adsorbent.

  17. Prussian blue caged in spongiform adsorbents using diatomite and carbon nanotubes for elimination of cesium

    Energy Technology Data Exchange (ETDEWEB)

    Hu, Baiyang [Graduate School of Environmental Science, Hokkaido University, Sapporo 060-0810 (Japan); Fugetsu, Bunshi, E-mail: hu@ees.hokudai.ac.jp [Graduate School of Environmental Science, Hokkaido University, Sapporo 060-0810 (Japan); Yu, Hongwen [Graduate School of Environmental Science, Hokkaido University, Sapporo 060-0810 (Japan); Abe, Yoshiteru [Kyoei Engineering Corporation, Niigata 959-1961 (Japan)

    2012-05-30

    Highlights: Black-Right-Pointing-Pointer Prussian blue was sealed in cavities of diatomite using carbon nanotubes. Black-Right-Pointing-Pointer The caged Prussian blue after being permanently immobilized in polyurethane spongy showed a 167 mg/g capability for absorbing cesium. Black-Right-Pointing-Pointer Cesium elimination was accomplished by simply adding the Prussian-blue based spongiform adsorbent to radioactive water. - Abstract: We developed a spongiform adsorbent that contains Prussian blue, which showed a high capacity for eliminating cesium. An in situ synthesizing approach was used to synthesize Prussian blue inside diatomite cavities. Highly dispersed carbon nanotubes (CNTs) were used to form CNT networks that coated the diatomite to seal in the Prussian blue particles. These ternary (CNT/diatomite/Prussian-blue) composites were mixed with polyurethane (PU) prepolymers to produce a quaternary (PU/CNT/diatomite/Prussian-blue), spongiform adsorbent with an in situ foaming procedure. Prussian blue was permanently immobilized in the cell walls of the spongiform matrix and preferentially adsorbed cesium with a theoretical capacity of 167 mg/g cesium. Cesium was absorbed primarily by an ion-exchange mechanism, and the absorption was accomplished by self-uptake of radioactive water by the quaternary spongiform adsorbent.

  18. Delayed Encephalopathy Associated with Carbon Monoxide Intoxication

    Directory of Open Access Journals (Sweden)

    Huseyin Aydin

    2011-01-01

    Full Text Available Acute carbon monoxide intoxication may cause coma and death. Patients usually recovier within days after prompt therapy. However, in a small number of patients, severe clinical deterioration may develop after a period with no apparent abnormality. This is called delayed type encephalopathy. We present MR imaging findings of a case of delayed encephalopathy due to carbon monoxide intoxication.

  19. Delayed Encephalopathy Associated with Carbon Monoxide Intoxication

    OpenAIRE

    Hüseyin Aydın; Mürüvet Akın; Emel Boyraz; Murat Çolakkaya

    2011-01-01

    Acute carbon monoxide intoxication may cause coma and death. Patients usually recovier within days after prompt therapy. However, in a small number of patients, severe clinical deterioration may develop after a period with no apparent abnormality. This is called delayed type encephalopathy. We present MR imaging findings of a case of delayed encephalopathy due to carbon monoxide intoxication.

  20. MR findings of wernicke encephalopathy

    International Nuclear Information System (INIS)

    Seven patients (33 to 58 years old) with clinical diagnoses of Wernicke encephalopathy were examined with MR on either a 2.0T (5 cases) or a 0.5T scanner (2 cases) using spin-echo pulse sequences. In 2 patients, follow-up MR studies were performed 1 and 5 weeks after thiamine (vitamine B1) treatment. Five patients (4 chronic alcoholics and 1 with hyperemesis gravidarum) showed atrophy of both mamillary bodies, along with patchy lesions around the third ventricle, medial thalami, tectum of the midbrain, and periaqueductal gray matter. Another patient with hyperemesis of gravidrum demonstrated only slightly atrophic mamillary bodies, and the last patient with severe vomiting after gastrojejunostomy showed only diencephaic/mesencephalic lesions with apparently normal mamillary bodies. A follow-up MR showed a decrease in previously-noted diencephalic/-/mesencephalic lesions but no change in the size of the mamillary bodies. Diencephalic/mesencephalic lesions were well seen as a high-signal intensity on proton-and T2-weighted axial images, while atrophy of the mamillary bodies was seen best on T1-weighted sagittal images. MR imaging is very useful in demonstrating the characteristic lesions of Wernicke encephalopathy and in evaluating the result of treatment on follow-up study

  1. Current understanding and neurobiology of epileptic encephalopathies.

    Science.gov (United States)

    Auvin, Stéphane; Cilio, Maria Roberta; Vezzani, Annamaria

    2016-08-01

    Epileptic encephalopathies are a group of diseases in which epileptic activity itself contributes to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone. These impairments can worsen over time. This concept has been continually redefined since its introduction. A few syndromes are considered epileptic encephalopathies: early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, epilepsy of infancy with migrating focal seizures, West syndrome or infantile spasms, Dravet syndrome during infancy, Lennox-Gastaut syndrome, epileptic encephalopathy with continuous spikes-and-waves during sleep, and Landau-Kleffner syndrome during childhood. The inappropriate use of this term to refer to all severe epilepsy syndromes with intractable seizures and severe cognitive dysfunction has led to confusion regarding the concept of epileptic encephalopathy. Here, we review our current understanding of those epilepsy syndromes considered to be epileptic encephalopathies. Genetic studies have provided a better knowledge of neonatal and infantile epilepsy syndromes, while neuroimaging studies have shed light on the underlying causes of childhood-onset epileptic encephalopathies such as Lennox-Gastaut syndrome. Apart from infantile spasm models, we lack animal models to explain the neurobiological mechanisms at work in these conditions. Experimental studies suggest that neuroinflammation may be a common neurobiological pathway that contributes to seizure refractoriness and cognitive involvement in the developing brain. PMID:26992889

  2. Comparison of MRI, CT, TCD and SPECT in patients with spongiform leukoencephalopathy after inhaling heroin vapor

    International Nuclear Information System (INIS)

    Objective: To compare the characteristics of MRI, CT, single photon emission computed tomography (SPECT) and transcranial Doppler sonography (TCD) in patients with spongiform leukoencephalopathy after inhaling heroin vapor. Methods: Seventeen patients were investigated. MRI or CT was scanned in 17 patients, SPECT in 9 patients' and TCD in 11 patients. Results: MRI or CT: Brain MRI and CT revealed extensive symmetric white matter involvement of bilateral cerebral and cerebellar hemispheres and the brainstem. The lesions, which showed no contrast enhancement, were hypodense on CT and hypointense on T1-weighted and hyperintense on T2-weighted MRI. SPECT: The regional cerebral blood flows (rCBF) of white matter involvement on bilateral cerebral and cerebellar hemispheres was reduced obviously. The rCBF of temporal lobes, parietal lobes, occipital lobes, cerebellar hemispheres, and basal ganglion was reduced in varying degrees. TCD: The systolic peak became sharpened, and end-diastolic flow velocity and mean flow velocity were reduced obviously and pulsatile index was increased markedly in patients with spongiform leukoencephalopathy after inhaling heroin vapor. Conclusion: The characteristic manifestations of MRI and CT imaging may be regarded as the main foundation of diagnosing spongiform leukoencephalopathy after inhaling heroin vapor; SPECT and TCD can help comprehend the changes of hemodynamics of cerebral vessels and the degree of cerebral ischemia in patients with spongiform leukoencephalopathy after inhaling heroin vapor

  3. CT and MR imaging of Spongiform leukoencephalopathy after heroin vapor inhalation: analysis of 13 cases

    Institute of Scientific and Technical Information of China (English)

    ZHOU Liang; WU Yong-ming; LIU Ji-yuan; LU Bing-xun; YIN Jia; PAN Su-yue; JI Zhong; LUO Yi-feng; LIU Xiao-jia; WANG Qun; GUAN Yu-qing

    2001-01-01

    To study the CT and MR imaging features of spongiform leukoencephalopathy after heroin vapor inhalation. Methods: The CT and MR imaging features and pathologic findings of 13 patients with heroin-induced spongiform leukoencephalopathy were analyzed. Results: CT scanning and MRI of all the patients showed diffuse,symmetric lesion in the cerebellar and cerebral white matter, and the cerebellum was invariably involved in all cases.Symmetric round or butterfly-like lesions lateral to the midline of the cerebellum with clear border was the most distinct feature in CT and MRI examination. The lesions were not found in the anterior limbs of the internal capsules. CT scanning showed low-density changes while MRI T1WI imaging presented low-signal and T2WI high-signal lesions without space-occupying mass. The pathologic findings showed spongiform degeneration of the white matter in the central nervous system, but necrotic lesions were not observed. Conclusions: Spongiform leukoencephalopathy should be considered when acute. Cerebellar signs are present in patients who admitted a history of heroin inhalation. The CT and MRI manifestation of this disease is typical and the diagnosis can thus be made.

  4. Influenza-Associated Encephalitis/Encephalopathy

    OpenAIRE

    J Gordon Millichap

    2008-01-01

    The role of influenza A and influenza B in acute childhood encephalitis and encephalopathy (ACE) was evaluated prospectively in all children admitted to the Hospital for Sick Children, Toronto, Canada, during an 11-year period from Jan 1994- Dec 2004.

  5. Wernicke's encephalopathy following ‘hunger strike’

    OpenAIRE

    Pentland, Brian; Mawdsley, Clifford

    1982-01-01

    A 41-year-old doctor who voluntarily starved himself and developed Wernicke's encephalopathy after intravenus rehydration is described. The need to be aware of this complication of dextrose infusion in the undernourished non-alcoholic patient is emphasized.

  6. Rifaximin in the treatment of hepatic encephalopathy

    OpenAIRE

    Iadevaia MD; Del Prete A; Cesaro C; Gaeta L; Zulli C; Loguercio C

    2011-01-01

    Maddalena Diana Iadevaia, Anna Del Prete, Claudia Cesaro, Laura Gaeta, Claudio Zulli, Carmelina LoguercioDepartment of Internistica Clinica e Sperimentale, F Magrassi e A Lanzara, Hepatogastroenterology Unit, Second University of Naples, Naples, ItalyAbstract: Hepatic encephalopathy is a challenging complication in patients with advanced liver disease. It can be defined as a neuropsychiatric syndrome caused by portosystemic venous shunting, ranging from minimal to overt hepatic encephalopathy...

  7. Management of Hepatic Encephalopathy in the Hospital

    OpenAIRE

    Leise, Michael D; Poterucha, John J.; Kamath, Patrick S; KIM, W. RAY

    2014-01-01

    Hepatic encephalopathy (HE) develops in about 50% of patients with cirrhosis and is one of the features of decompensated cirrhosis. The inpatient incidence of HE is approximately 23,000/year and management of these patients is common for internists and subspecialists. Treatment of the hospitalized patient with HE has changed in recent years. Treatment entails two phases, induction and maintenance of remission. Most cases of significant hepatic encephalopathy are precipitated by infection, gas...

  8. 9 CFR 94.22 - Restrictions on importation of beef from Uruguay.

    Science.gov (United States)

    2010-01-01

    ... FEVER, SWINE VESICULAR DISEASE, AND BOVINE SPONGIFORM ENCEPHALOPATHY: PROHIBITED AND RESTRICTED... following conditions: (a) The meat is beef from bovines that have been born, raised, and slaughtered in... slaughtering establishment, with no evidence found of vesicular disease. (f) The beef consists only of...

  9. Prion-Specific Antibodies Produced in Wild-Type Mice

    DEFF Research Database (Denmark)

    Heegaard, Peter M. H.; Bergström, Ann-Louise; Andersen, Heidi Gertz;

    2015-01-01

    method for production of mouse monoclonal antibodies (MAbs) against peptides representing two sites of interest in the bovine prion protein (boPrP), the causative agent of bovine spongiform encephalopathy ("mad cow disease") and new variant Creutzfeldt-Jakob's disease (CJD) in humans, as well as a...

  10. Genetics Home Reference: familial encephalopathy with neuroserpin inclusion bodies

    Science.gov (United States)

    ... Home Health Conditions FENIB familial encephalopathy with neuroserpin inclusion bodies Enable Javascript to view the expand/collapse ... All Close All Description Familial encephalopathy with neuroserpin inclusion bodies ( FENIB ) is a disorder that causes progressive ...

  11. Chronic Traumatic Encephalopathy: A Review

    Directory of Open Access Journals (Sweden)

    Michael Saulle

    2012-01-01

    Full Text Available Chronic traumatic encephalopathy (CTE is a progressive neurodegenerative disease that is a long-term consequence of single or repetitive closed head injuries for which there is no treatment and no definitive pre-mortem diagnosis. It has been closely tied to athletes who participate in contact sports like boxing, American football, soccer, professional wrestling and hockey. Risk factors include head trauma, presence of ApoE3 or ApoE4 allele, military service, and old age. It is histologically identified by the presence of tau-immunoreactive NFTs and NTs with some cases having a TDP-43 proteinopathy or beta-amyloid plaques. It has an insidious clinical presentation that begins with cognitive and emotional disturbances and can progress to Parkinsonian symptoms. The exact mechanism for CTE has not been precisely defined however, research suggest it is due to an ongoing metabolic and immunologic cascade called immunoexcitiotoxicity. Prevention and education are currently the most compelling way to combat CTE and will be an emphasis of both physicians and athletes. Further research is needed to aid in pre-mortem diagnosis, therapies, and support for individuals and their families living with CTE.

  12. Branched-chain amino acids for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Koretz, R L; Kjaergard, L L;

    2003-01-01

    Hepatic encephalopathy may be caused by a decreased plasma ratio of branched-chain amino acids (BCAA) to aromatic amino acids. Treatment with BCAA may therefore have a beneficial effect on patients with hepatic encephalopathy.......Hepatic encephalopathy may be caused by a decreased plasma ratio of branched-chain amino acids (BCAA) to aromatic amino acids. Treatment with BCAA may therefore have a beneficial effect on patients with hepatic encephalopathy....

  13. Prussian blue caged in spongiform adsorbents using diatomite and carbon nanotubes for elimination of cesium

    OpenAIRE

    Hu, Baiyang; Fugetsu, Bunshi; Yu, Hongwen; Abe, Yoshiteru

    2012-01-01

    We developed a spongiform adsorbent that contains Prussian blue, which showed a high capacity for eliminating cesium. An in situ synthesizing approach was used to synthesize Prussian blue inside diatomite cavities. Highly dispersed carbon nanotubes (CNTs) were used to form CNT networks that coated the diatomite to seal in the Prussian blue particles. These ternary (CNT/diatomite/Prussian-blue) composites were mixed with polyurethane (PU) prepolymers to produce a quaternary (PU/CNT/diatomite/P...

  14. Surgical Treatment of Pediatric Epileptic Encephalopathies

    Directory of Open Access Journals (Sweden)

    J. Fridley

    2013-01-01

    Full Text Available Pediatric epileptiform encephalopathies are a group of neurologically devastating disorders related to uncontrolled ictal and interictal epileptic activity, with a poor prognosis. Despite the number of pharmacological options for treatment of epilepsy, many of these patients are drug resistant. For these patients with uncontrolled epilepsy, motor and/or neuropsychological deterioration is common. To prevent these secondary consequences, surgery is often considered as either a curative or a palliative option. Magnetic resonance imaging to look for epileptic lesions that may be surgically treated is an essential part of the workup for these patients. Many surgical procedures for the treatment of epileptiform encephalopathies have been reported in the literature. In this paper the evidence for these procedures for the treatment of pediatric epileptiform encephalopathies is reviewed.

  15. Antiviral effects of bovine interferons on bovine respiratory tract viruses.

    OpenAIRE

    Fulton, R W; Downing, M M; Cummins, J M

    1984-01-01

    The antiviral effects of bovine interferons on the replication of bovine respiratory tract viruses were studied. Bovine turbinate monolayer cultures were treated with bovine interferons and challenged with several bovine herpesvirus 1 strains, bovine viral diarrhea virus, parainfluenza type 3 virus, goat respiratory syncytial virus, bovine respiratory syncytial virus, bovine adenovirus type 7, or vesicular stomatitis virus. Treatment with bovine interferons reduced viral yield for each of the...

  16. Delayed encephalopathy after acute carbon monoxide poisoning

    Directory of Open Access Journals (Sweden)

    Mehmet İbrahim Turan

    2014-03-01

    Full Text Available Carbon monoxide poisoning is a major cause of death following attempted suicide and accidental exposures. Although clinical presentation depends on the duration and the intensity of exposure, the assessment of the severity of intoxication is difficult. A small percentage of patients who show complete initial recovery may develop delayed neurological deficits. Delayed encephalopathy after acute carbon monoxide poisoning is a rare and poor prognosis neurologic disorders and there is no specific treatment. We present a case with early onset of delayed encephalopathy after acute carbon monoxide poisoning with typical cranial imaging findings in a child with atypical history and clinical presentation.

  17. Hypertensive encephalopathy complicating transplant renal artery stenosis.

    OpenAIRE

    McGonigle, R J; Bewick, M.; Trafford, J. A.; Parsons, V

    1984-01-01

    A 26-year-old female diabetic patient developed hypertensive encephalopathy with gross neurological abnormalities complicating renal artery stenosis of her transplant kidney. The elevated blood pressure was unresponsive to medical treatment. Surgical correction of the stenoses in the renal artery cured the hypertension and renal failure and led to the patient's complete recovery.

  18. Non-Absorbable Disaccharides for Hepatic Encephalopathy

    DEFF Research Database (Denmark)

    Gluud, Lise L; Vilstrup, Hendrik; Morgan, Marsha Y

    2016-01-01

    Non-absorbable disaccharides (NADs) have been used to treat hepatic encephalopathy (HE) since 1966. However, a Cochrane review, published in 2004, found insufficient evidence to recommend their use in this context. This updated systematic review evaluates the effects of the NADs, lactulose and...

  19. Hepatic encephalopathy: clinical and experimental studies

    NARCIS (Netherlands)

    C.C.D. van der Rijt (Carin)

    1991-01-01

    textabstractThe pathogenesis of hepatic encephalopathy is still unsolved. Therapy, therefore, is often insufficient. For the development of effective, new therapies insight into the disease-inducing substrates and the mechanisms of its toxic actions in the central nervous system ·are required. For b

  20. Wernicke encephalopathy in children and adolescents

    Institute of Scientific and Technical Information of China (English)

    Matt Lallas; Jay Desai

    2014-01-01

    Background: Wernicke encephalopathy is caused by thiamine (vitamin B1) defi ciency. It is generally considered to be a disease of adult alcoholics. However, it is known to occur in the pediatric population and in non-alcoholic conditions. Data sources: We searched PubMed with the key words Wernicke, thiamine, pediatric, children and adolescents and selected publications that were deemed appropriate. Results: The global prevalence rates of hunger, poverty and resultant nutrient deprivation have decreased in the 21st century. However, several scenarios which may predispose to Wernicke encephalopathy may be increasingly prevalent in children and adolescents such as malignancies, intensive care unit stays and surgical procedures for the treatment of obesity. Other predisposing conditions include magnesium defi ciency and defects in the SLC19A3 gene causing thiamine transporter-2 deficiency. The classic triad consists of encephalopathy, oculomotor dysfunction and gait ataxia but is not seen in a majority of patients. Treatment should be instituted immediately when the diagnosis is suspected clinically without waiting for laboratory confi rmation. Common magnetic resonance findings include symmetric T2 hyperintensities in dorsal medial thalamus, mammillary bodies, periaqueductal gray matter, and tectal plate. Conclusions: Wernicke encephalopathy is a medical emergency. Delay in its recognition and treatment may lead to significant morbidity, irreversible neurological damage or even death. This article aims to raise the awareness of this condition among pediatricians.

  1. STXBP1 encephalopathy

    DEFF Research Database (Denmark)

    Stamberger, Hannah; Nikanorova, Marina; Willemsen, Marjolein H;

    2016-01-01

    OBJECTIVE: To give a comprehensive overview of the phenotypic and genetic spectrum of STXBP1 encephalopathy (STXBP1-E) by systematically reviewing newly diagnosed and previously reported patients. METHODS: We recruited newly diagnosed patients with STXBP1 mutations through an international networ...

  2. Association between Helicobacter pylori seropositivity and Hepatic Encephalopathy

    International Nuclear Information System (INIS)

    Objective: The knowledge on Helicobacter pylori (H. pylori) contribution in the pathology of the liver and biliary tract diseases in human is very limited. The aim of this study was to assess the probable association between H. pylori seropositivity and hepatic encephalopathy. Methodology: This is a case control study conducted through three groups, cirrhotics with hepatic encephalopathy (HE), cirrhotics without HE and healthy controls. All subjects were examined serologically for determination of IgG class antibodies to H. pylori based on ELISA technique. Results: H. pylori seropositivity was present in 88% cirrhotic patients with hepatic encephalopathy, 86% cirrhotics without hepatic encephalopathy and 66% healthy controls. Conclusion: According to our results, H. pylori seropositivity rate in cirrhotic patients with or without hepatic encephalopathy was higher than healthy controls. But H. pylori seropositivity rate was not significantly different among cirrhotics with hepatic encephalopathy and those without it.

  3. Hashimoto's encephalopathy: A rare proteiform disorder.

    Science.gov (United States)

    Montagna, Giacomo; Imperiali, Mauro; Agazzi, Pamela; D'Aurizio, Federica; Tozzoli, Renato; Feldt-Rasmussen, Ulla; Giovanella, Luca

    2016-05-01

    Hashimoto's encephalopathy (HE) is a rare not well understood, progressive and relapsing multiform disease, characterized by seizures, movement disorders, subacute cognitive dysfunction, psychiatric symptoms and responsiveness to steroid therapy. The disorder is generally associated with thyroid diseases and the most common feature is the presence of anti-thyroperoxidase antibodies (TPOAb). Patients are usually euthyroid or mildly hypothyroid at presentation. All age groups can be affected. The pathophysiology is still unclear, especially the link between elevated serum TPOAb and the encephalopathy. Most reported cases occurred in women and girls. Unspecific symptoms, non-pathognomonic laboratory neurophysiology and neuroimaging features make its diagnosis a real challenge for clinicians. The case of a 16year old boy, with a clinical picture of HE associated with hypothyroidism, demonstrating an excellent response to high dose steroids is presented together with a systematic review of the literature. PMID:26849953

  4. Encephalopathy of infection and systemic inflammation.

    Science.gov (United States)

    Young, G Bryan

    2013-10-01

    This review will discuss several intracranial infections and sepsis-associated encephalopathy. Intracranial infections and inflammation of interest to the neurologist and EEG technicians include viral and autoimmune encephalitides; bacterial, fungal, and other meningitides; cerebritis; and brain abscess and subdural empyema. Sepsis-associated encephalopathy refers to a diffuse brain dysfunction secondary to infection that is principally located outside of the central nervous system. It is much more common than all of the intracranial infections put together, at least for adults in Western society. It probably involves a number of mechanisms that are not mutually exclusive and likely vary from patient to patient. Morbidity and mortality are directly related to the severity of SAE. The earliest features of SAE are delirium and mild EEG slowing; it is crucial to recognize these early features and to search for and treat the underlying infection promptly to reduce mortality and morbidity. PMID:24084178

  5. Research progress on transmissible spongiform encephalopathies%传染性海绵样脑病病原的研究进展

    Institute of Scientific and Technical Information of China (English)

    陈悦青; 毛子安

    2000-01-01

    传染性海绵样脑病因疯牛病的爆发和新型克雅氏病的出现而引起人们的关注.研究发现,疯牛病的流行可能是由羊痒病的病原引起的,这种病原被Prusiner命名为"Prion"(1982).从80年代开始研究以来,越来越多的关于Prion的特性被人们所了解,比如Prion蛋白和PrP基因.本文综述了近几年来研究者们用不同手段从各个角度对病原进行研究的新进展.

  6. Retrovirus-induced spongiform encephalopathy: the 3'-end long terminal repeat-containing viral sequences influence the incidence of the disease and the specificity of the neurological syndrome.

    OpenAIRE

    DesGroseillers, L; Rassart, E; Robitaille, Y; Jolicoeur, P

    1985-01-01

    Using chimeric murine leukemia viruses (MuLVs) constructed in vitro with parental viral genomes from the neurotropic Cas-BR-E MuLV and the nonneurotropic amphotropic 4070-A MuLV, we previously mapped the paralysis-inducing determinant of Cas-BR-E MuLV within a pol-env region. To assess the role of the long terminal repeats (LTRs) in influencing the neurological disease, we constructed another chimeric MuLV (pNEMO-1)m harboring the gag-pol-env from Cas-BR-E MuLV and the LTR region from the str...

  7. Effects on instruments of the World Health Organization--recommended protocols for decontamination after possible exposure to transmissible spongiform encephalopathy-contaminated tissue.

    Science.gov (United States)

    Brown, Stanley A; Merritt, Katharine; Woods, Terry O; Busick, Deanna N

    2005-01-15

    It has been recommended by the World Health Organization (WHO) and Centers for Disease Control and Prevention (CDC) that rigorous decontamination protocols be used on surgical instruments that have been exposed to tissue possibly contaminated with Creutzfeldt-Jakob disease (CJD). This study was designed to examine the effects of these protocols on various types of surgical instruments. The most important conclusions are: (1) autoclaving in 1N NaOH will cause darkening of some instruments; (2) soaking in 1N NaOH at room temperature damages carbon steel but not stainless steel or titanium; (3) soaking in chlorine bleach will badly corrode gold-plated instruments and will damage some, but not all, stainless-steel instruments, especially welded and soldered joints. Damage became apparent after the first exposure and therefore long tests are not necessary to establish which instruments will be damaged. PMID:15449256

  8. Pyridoxal phosphate-dependent neonatal epileptic encephalopathy

    OpenAIRE

    Bagci, S.; Zschocke, J.; Hoffmann, G F; Bast, T.; Klepper, J; Müller, A.; Heep, A; Bartmann, P.; Franz, A R

    2009-01-01

    Pyridox(am)ine-5′-phosphate oxidase converts pyridoxine phosphate and pyridoxamine phosphate to pyridoxal phosphate, a cofactor in many metabolic reactions, including neurotransmitter synthesis. A family with a mutation in the pyridox(am)ine-5′-phosphate oxidase gene presenting with neonatal seizures unresponsive to pyridoxine and anticonvulsant treatment but responsive to pyridoxal phosphate is described. Pyridoxal phosphate should be considered in neonatal epileptic encephalopathy unrespons...

  9. Hemorrhagic Encephalopathy from Acute Baking Soda Ingestion

    OpenAIRE

    Hughes, Adrienne; Brown, Alisha Emily Cutler; Valento, Matthew

    2016-01-01

    Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities.  We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion.  Healthcare providers should be aware of the dangers baking soda misuse and the associated adverse effects.

  10. Antibiotics for the Treatment of Hepatic Encephalopathy

    OpenAIRE

    Patidar, Kavish R.; Bajaj, Jasmohan S.

    2013-01-01

    The treatment of hepatic encephalopathy (HE) is complex and therapeutic regimens vary according to the acuity of presentation and the goals of therapy. Most treatments for HE rely on manipulating the intestinal milieu and therefore antibiotics that act on the gut form a key treatment strategy. Prominent antibiotics studied in HE are neomycin, metronidazole, vancomycin and rifaximin. For the management of the acute episode, all antibiotics have been tested. However the limited numbers studied,...

  11. Delayed encephalopathy after acute carbon monoxide poisoning

    OpenAIRE

    Mehmet İbrahim Turan; Atilla Çayır; Haşim Olgun

    2014-01-01

    Carbon monoxide poisoning is a major cause of death following attempted suicide and accidental exposures. Although clinical presentation depends on the duration and the intensity of exposure, the assessment of the severity of intoxication is difficult. A small percentage of patients who show complete initial recovery may develop delayed neurological deficits. Delayed encephalopathy after acute carbon monoxide poisoning is a rare and poor prognosis neurologic disorders and there is no specific...

  12. Inflammatory responses in hypoxic ischemic encephalopathy

    OpenAIRE

    Liu, Fudong; McCullough, Louise D.

    2013-01-01

    Inflammation plays a critical role in mediating brain injury induced by neonatal hypoxic ischemic encephalopathy (HIE). The mechanisms underlying inflammatory responses to ischemia may be shared by neonatal and adult brains; however, HIE exhibits a unique inflammation phenotype that results from the immaturity of the neonatal immune system. This review will discuss the current knowledge concerning systemic and local inflammatory responses in the acute and subacute stages of HIE. The key compo...

  13. Preclinical Models of Encephalopathy of Prematurity

    OpenAIRE

    Jantzie, Lauren L.; Robinson, Shenandoah

    2015-01-01

    Encephalopathy of prematurity (EoP) encompasses the central nervous system (CNS) abnormalities associated with injury from preterm birth. Although rapid progress is being made, limited understanding exists of how the cellular and molecular CNS injury from early birth manifests as the myriad of neurological deficits in children who are born preterm. More importantly, this lack of direct insight into the pathogenesis of these deficits hinders both our ability to diagnose those infants who are a...

  14. Gut Microbiota: Its Role in Hepatic Encephalopathy

    OpenAIRE

    Rai, Rahul; Saraswat, Vivek A.; Dhiman, Radha K.

    2014-01-01

    Ammonia, a key factor in the pathogenesis of hepatic encephalopathy (HE), is predominantly derived from urea breakdown by urease producing large intestinal bacteria and from small intestine and kidneys, where the enzyme glutaminases releases ammonia from circulating glutamine. Non-culture techniques like pyrosequencing of bacterial 16S ribosomal ribonucleic acid are used to characterize fecal microbiota. Fecal microbiota in patients with cirrhosis have been shown to alter with increasing Chil...

  15. The role of microbiota in hepatic encephalopathy

    OpenAIRE

    Jasmohan S. Bajaj

    2014-01-01

    Hepatic encephalopathy (HE), which consists of minimal (MHE) and overt (OHE) stages, is a model for impaired gut-liver-brain axis in cirrhosis. Microbiota changes in both stages have been associated with impaired cognition, endotoxemia, and inflammation. There is dysbiosis (reduced autochthonous taxa [Lachnospiraceae, Ruminococcaceae, and Clostridiales XIV] and increased Enterobacteriaceae and Streptococcaceae) with disease progression. In MHE, there is an increased abundance of Streptococcus...

  16. Fetal lead exposure: Encephalopathy in a child

    OpenAIRE

    Dsouza, Herman S.; Menezes, Geraldine; Venkatesh, T

    2002-01-01

    It is well known that lead exposure in the early period of pre pregnancy and antenatal life leads to serious health complications. In this case report, a five month old child who was suffering from encephalopathy was finally confirmed a victim of lead exposure, the source being the environment and the family. We report this case with complete clinical investigation including blood lead analysis. This case report highlights the various ways in which lead may accumulate in the body. It is also ...

  17. Transcranial electrostimulation in patients with alcoholic encephalopathy

    OpenAIRE

    Barylnik Yu.B.; Lim V.G.; Shchetinin S.G.

    2010-01-01

    The method of transcranial electrostimulation (TES) was used for treating patients with alcoholic encephalopathy against the background of the basic treatment, which includes nootropics, normotimics, soporifics, over-all strengthening therapy and other devices. The course of treatment consisted of 10 daily procedures lasting for 30 minutes. The TES influence was evaluated according to the clinical state, the neurologic status, including EEG (electroencephalogram), the psychometric scales were...

  18. Hemorrhagic Encephalopathy From Acute Baking Soda Ingestion

    Directory of Open Access Journals (Sweden)

    Adrienne Hughes

    2016-09-01

    Full Text Available Baking soda is a readily available household product composed of sodium bicarbonate. It can be used as a home remedy to treat dyspepsia. If used in excessive amounts, baking soda has the potential to cause a variety of serious metabolic abnormalities. We believe this is the first reported case of hemorrhagic encephalopathy induced by baking soda ingestion. Healthcare providers should be aware of the dangers of baking soda misuse and the associated adverse effects.

  19. No oxygen delivery limitation in hepatic encephalopathy

    DEFF Research Database (Denmark)

    Gjedde, Albert; Keiding, Susanne; Vilstrup, Hendrik;

    2010-01-01

    Hepatic encephalopathy is a condition of reduced brain functioning in which both blood flow and brain energy metabolism declined. It is not known whether blood flow or metabolism is the primary limiting factor of brain function in this condition. We used calculations of mitochondrial oxygen tensi...... oxygen tensions that patients with HE were unable to utilize. We ascribe the inability to use the delivered oxygen of patients with HE to a specific inhibition associated with oxidative metabolism in mitochondria....

  20. Сhanges of brain bioelectric activity by diabetic encephalopathy

    OpenAIRE

    Vitaly P. Omelchenko; Elena A. Timoshenko

    2011-01-01

    This article focuses on the analysis of brain bioelectric activity by diabetic encephalopathy. Paid attention to the establishment of EEG parameters and patients psychological characteristics interrelation.

  1. Genetics Home Reference: MECP2-related severe neonatal encephalopathy

    Science.gov (United States)

    ... Hospital: Seizures Centers for Disease Control and Prevention: Intellectual Disability Cleveland Clinic: Microcephaly in Children Cleveland Clinic: Sleep Apnea Disease InfoSearch: Encephalopathy, neonatal ...

  2. Effect of Helicobacter Pylori Eradication on Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Ahamd Shavakhi

    2008-05-01

    Full Text Available Background and Aims: Ammonia has still essential role in pathogenesis of encephalopathy. It is probable that helicobacter pylori (H. pylori infection leads to hyperammonemia and aggravates the hepatic encephalopathy via urease activity or its effect on zinc level. This study was aimed to assess the effect of H pylori eradication on hepatic encephalopathy. Methods: In a cross-sectional study, 42 cirrhotic patients with encephalopathy were enrolled the study. For evaluation the encephalopathy, flapping tremor, number connection test (NCT and grade of encephalopathy was determined for all of the patients. The prevalence of H pylori infection was evaluated based on positive serology by ELAISA method (Genesis Kit, UK, and/or rapid urease test (RUT during endoscopic evaluation. One month later, after treatment, grading of encephalopathy, NCT and flapping tremor was evaluated again and data were analyzed by software SPSS.Results: Serologic evaluation or RUT showed that 30 patients of 42 (71.42% were positive for H pylori. In comparison between pre and post treatment, grade of encephalopathy and NCT were reduced significantly but alteration in flapping tremor was not statistically significant.Conclusions: Our results indicate that eradication of H pylori may lead to improve the grade of hepatic encephalopathy and NCT.

  3. C-peptide and Diabetic Encephalopathy

    Institute of Scientific and Technical Information of China (English)

    Xiao-jun Cai; Hui-qin Xu; Yi Lu

    2011-01-01

    With the changes of life style, diabetes and its complications have become a major cause of morbidity and mortality. It is reasonable to anticipate a continued rise in the incidence of diabetes and its complications along with the aging of the population, increase in adult obesity rate, and other risk factors. Diabetic encephalopathy is one of the severe microvascular complications of diabetes, characterized by impaired cognitive functions, and electrophysiological, neurochemical, and structural abnormalities. It may involve direct neuronal damage caused by intracellular glucose. However, the pathogenesis of this disease is complex and its diagnosis is not very clear. Previous researches have suggested that chronic metabolic alterations, vascular changes, and neuronal apoptosis may play important roles in neuronal loss and damaged cognitive fimctions.Multiple factors are responsible for neuronal apoptosis, such as disturbed insulin growth factor (IGF) system,hyperglycemia, and the aging process. Recent data suggest that insulin/C-peptide defidency may exert a primary and key effect in diabetic encephalopathy. Administration of C-peptide partially improves the condition of the IGF system in the brain and prevents neuronal apoptosis in the hippocampus of diabetic patients.Those Findings provide a basis for application of C-peptide as a potentially effective therapy for diabetes and diabetic encephalopathy.

  4. Inflammatory Macrophages Promotes Development of Diabetic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Beiyun Wang

    2015-06-01

    Full Text Available Background/Aims: Diabetes and Alzheimer's disease are often associated with each other, whereas the relationship between two diseases is ill-defined. Although hyperglycemia during diabetes is a major cause of encephalopathy, diabetes may also cause chronic inflammatory complications including peripheral neuropathy. Hence the role and the characteristics of inflammatory macrophages in the development of diabetic encephalopathy need to be clarified. Methods: Diabetes were induced in mice by i.p. injection of streptozotocin (STZ. Two weeks after STZ injection and confirmation of development of diabetes, inflammatory macrophages were eliminated by i.p. injection of 20µg saporin-conjugated antibody against a macrophage surface marker CD11b (saporin-CD11b twice per week, while a STZ-treated group received injection of rat IgG of same frequency as a control. The effects of macrophage depletion on brain degradation markers, brain malondialdehyde (MDA, catalase, superoxidase anion-positive cells and nitric oxide (NO were measured. Results: Saporin-CD11b significantly reduced inflammatory macrophages in brain, without affecting mouse blood glucose, serum insulin, glucose responses and beta cell mass. However, reduced brain macrophages significantly inhibited the STZ-induced decreases in brain MDA, catalase and superoxidase anion-positive cells, and the STZ-induced decreases in brain NO. Conclusion: Inflammatory macrophages may promote development of diabetic encephalopathy.

  5. Blood Biomarkers for Evaluation of Perinatal Encephalopathy

    Science.gov (United States)

    Graham, Ernest M.; Burd, Irina; Everett, Allen D.; Northington, Frances J.

    2016-01-01

    Recent research in identification of brain injury after trauma shows many possible blood biomarkers that may help identify the fetus and neonate with encephalopathy. Traumatic brain injury shares many common features with perinatal hypoxic-ischemic encephalopathy. Trauma has a hypoxic component, and one of the 1st physiologic consequences of moderate-severe traumatic brain injury is apnea. Trauma and hypoxia-ischemia initiate an excitotoxic cascade and free radical injury followed by the inflammatory cascade, producing injury in neurons, glial cells and white matter. Increased excitatory amino acids, lipid peroxidation products, and alteration in microRNAs and inflammatory markers are common to both traumatic brain injury and perinatal encephalopathy. The blood-brain barrier is disrupted in both leading to egress of substances normally only found in the central nervous system. Brain exosomes may represent ideal biomarker containers, as RNA and protein transported within the vesicles are protected from enzymatic degradation. Evaluation of fetal or neonatal brain derived exosomes that cross the blood-brain barrier and circulate peripherally has been referred to as the “liquid brain biopsy.” A multiplex of serum biomarkers could improve upon the current imprecise methods of identifying fetal and neonatal brain injury such as fetal heart rate abnormalities, meconium, cord gases at delivery, and Apgar scores. Quantitative biomarker measurements of perinatal brain injury and recovery could lead to operative delivery only in the presence of significant fetal risk, triage to appropriate therapy after birth and measure the effectiveness of treatment. PMID:27468268

  6. Using saccades to diagnose covert hepatic encephalopathy.

    Science.gov (United States)

    Cunniffe, Nicholas; Munby, Henry; Chan, Shona; Saatci, Defne; Edison, Eric; Carpenter, R H S; Massey, Dunecan

    2015-06-01

    Covert Hepatic Encephalopathy (CHE), previously known as Minimal Hepatic Encephalopathy, is a subtle cognitive defect found in 30-70 % of cirrhosis patients. It has been linked to poor quality of life, impaired fitness to drive, and increased mortality: treatment is possible. Despite its clinical significance, diagnosis relies on psychometric tests that have proved unsuitable for use in a clinical setting. We investigated whether measurement of saccadic latency distributions might be a viable alternative. We collected data on 35 cirrhosis patients at Addenbrooke's Hospital, Cambridge, with no evidence of clinically overt encephalopathy, and 36 age-matched healthy controls. Performance on standard psychometric tests was evaluated to determine those patients with CHE as defined by the World Congress of Gastroenterology. We then compared visually-evoked saccades between those with CHE and those without, as well as reviewing blood test results and correlating saccadic latencies with biochemical parameters and prognostic scores. Cirrhosis patients have significantly longer median saccadic latencies than healthy controls. Those with CHE had significantly prolonged saccadic latencies when compared with those without CHE. Analysis of a cirrhosis patient's saccades can diagnose CHE with a sensitivity of 75 % and a specificity of 75 %. We concluded that analysis of a cirrhosis patient's saccadic latency distributions is a fast and objective measure that can be used as a diagnostic tool for CHE. This improved early diagnosis could direct avoidance of high-risk activities such as driving, and better inform treatment strategies. PMID:25586511

  7. Minimal hepatic encephalopathy matters in daily life

    Institute of Scientific and Technical Information of China (English)

    Jasmohan S Bajaj

    2008-01-01

    Minimal hepatic encephalopathy is a neuro-cognitive dysfunction which occurs in an epidemic proportion of cirrhotic patients, estimated as high as 80% of the population tested. It is characterized by a specific, complex cognitive dysfunction which is independent of sleep dysfunction or problems with overall intelligence. Although named "minimal", minimal hepatic encephalopathy (MHE) can have a far-reaching impact on quality of life, ability to function in daily life and progression to overt hepatic encephalopathy. Importantly, MHE has a profound negative impact on the ability to drive a car and may be a significant factor behind motor vehicle accidents. A crucial aspect of the clinical care of MHE patients is their driving history, which is often ignored in routine care and can add a vital dimension to the overall disease assessment. Driving history should be an integral part of care in patients with MHE. The lack of specific signs and symptoms, the preserved communication skills and lack of insight make MHE a difficult condition to diagnose. Diagnostic strategies for MHE abound, but are usually limited by financial, normative or time constraints. Recent studies into the inhibitory control and critical flicker frequency tests are encouraging since these tests can increase the rates of MHE diagnosis without requiring a psychologist. Although testing for MHE and subsequent therapy is not standard of care at this time, it is important to consider this in cirrhotics in order to improve their ability to live their life to the fullest.

  8. NMDA receptors in hyperammonemia and hepatic encephalopathy.

    Science.gov (United States)

    Llansola, Marta; Rodrigo, Regina; Monfort, Pilar; Montoliu, Carmina; Kosenko, Elena; Cauli, Omar; Piedrafita, Blanca; El Mlili, Nisrin; Felipo, Vicente

    2007-12-01

    The NMDA type of glutamate receptors modulates learning and memory. Excessive activation of NMDA receptors leads to neuronal degeneration and death. Hyperammonemia and liver failure alter the function of NMDA receptors and of some associated signal transduction pathways. The alterations are different in acute and chronic hyperammonemia and liver failure. Acute intoxication with large doses of ammonia (and probably acute liver failure) leads to excessive NMDA receptors activation, which is responsible for ammonia-induced death. In contrast, chronic hyperammonemia induces adaptive responses resulting in impairment of signal transduction associated to NMDA receptors. The function of the glutamate-nitric oxide-cGMP pathway is impaired in brain in vivo in animal models of chronic liver failure or hyperammonemia and in homogenates from brains of patients died in hepatic encephalopathy. The impairment of this pathway leads to reduced cGMP and contributes to impaired cognitive function in hepatic encephalopathy. Learning ability is reduced in animal models of chronic liver failure and hyperammonemia and is restored by pharmacological manipulation of brain cGMP by administering phosphodiesterase inhibitors (zaprinast or sildenafil) or cGMP itself. NMDA receptors are therefore involved both in death induced by acute ammonia toxicity (and likely by acute liver failure) and in cognitive impairment in hepatic encephalopathy. PMID:17701332

  9. De novo mutations in epileptic encephalopathies.

    Science.gov (United States)

    Allen, Andrew S; Berkovic, Samuel F; Cossette, Patrick; Delanty, Norman; Dlugos, Dennis; Eichler, Evan E; Epstein, Michael P; Glauser, Tracy; Goldstein, David B; Han, Yujun; Heinzen, Erin L; Hitomi, Yuki; Howell, Katherine B; Johnson, Michael R; Kuzniecky, Ruben; Lowenstein, Daniel H; Lu, Yi-Fan; Madou, Maura R Z; Marson, Anthony G; Mefford, Heather C; Esmaeeli Nieh, Sahar; O'Brien, Terence J; Ottman, Ruth; Petrovski, Slavé; Poduri, Annapurna; Ruzzo, Elizabeth K; Scheffer, Ingrid E; Sherr, Elliott H; Yuskaitis, Christopher J; Abou-Khalil, Bassel; Alldredge, Brian K; Bautista, Jocelyn F; Berkovic, Samuel F; Boro, Alex; Cascino, Gregory D; Consalvo, Damian; Crumrine, Patricia; Devinsky, Orrin; Dlugos, Dennis; Epstein, Michael P; Fiol, Miguel; Fountain, Nathan B; French, Jacqueline; Friedman, Daniel; Geller, Eric B; Glauser, Tracy; Glynn, Simon; Haut, Sheryl R; Hayward, Jean; Helmers, Sandra L; Joshi, Sucheta; Kanner, Andres; Kirsch, Heidi E; Knowlton, Robert C; Kossoff, Eric H; Kuperman, Rachel; Kuzniecky, Ruben; Lowenstein, Daniel H; McGuire, Shannon M; Motika, Paul V; Novotny, Edward J; Ottman, Ruth; Paolicchi, Juliann M; Parent, Jack M; Park, Kristen; Poduri, Annapurna; Scheffer, Ingrid E; Shellhaas, Renée A; Sherr, Elliott H; Shih, Jerry J; Singh, Rani; Sirven, Joseph; Smith, Michael C; Sullivan, Joseph; Lin Thio, Liu; Venkat, Anu; Vining, Eileen P G; Von Allmen, Gretchen K; Weisenberg, Judith L; Widdess-Walsh, Peter; Winawer, Melodie R

    2013-09-12

    Epileptic encephalopathies are a devastating group of severe childhood epilepsy disorders for which the cause is often unknown. Here we report a screen for de novo mutations in patients with two classical epileptic encephalopathies: infantile spasms (n = 149) and Lennox-Gastaut syndrome (n = 115). We sequenced the exomes of 264 probands, and their parents, and confirmed 329 de novo mutations. A likelihood analysis showed a significant excess of de novo mutations in the ∼4,000 genes that are the most intolerant to functional genetic variation in the human population (P = 2.9 × 10(-3)). Among these are GABRB3, with de novo mutations in four patients, and ALG13, with the same de novo mutation in two patients; both genes show clear statistical evidence of association with epileptic encephalopathy. Given the relevant site-specific mutation rates, the probabilities of these outcomes occurring by chance are P = 4.1 × 10(-10) and P = 7.8 × 10(-12), respectively. Other genes with de novo mutations in this cohort include CACNA1A, CHD2, FLNA, GABRA1, GRIN1, GRIN2B, HNRNPU, IQSEC2, MTOR and NEDD4L. Finally, we show that the de novo mutations observed are enriched in specific gene sets including genes regulated by the fragile X protein (P < 10(-8)), as has been reported previously for autism spectrum disorders. PMID:23934111

  10. Use of a Marker Organism To Model the Spread of Central Nervous System Tissue in Cattle and the Abattoir Environment during Commercial Stunning and Carcass Dressing

    OpenAIRE

    D.J. Daly; Prendergast, D M; Sheridan, J. J.; Blair, I. S.; McDowell, D. A.

    2002-01-01

    Due to concerns about a link between variant Creutzfeldt-Jakob disease in humans and similar prion protein-induced disease in cattle, i.e., bovine spongiform encephalopathy (BSE), strict controls are in place to exclude BSE-positive animals and/or specified risk materials including bovine central nervous system (CNS) tissue from the human food chain. However, current slaughter practice, using captive bolt guns, may induce disruption of brain tissues and mobilize CNS tissues into the bovine ci...

  11. Irreversible Encephalopathy After Treatment With High-Dose Intravenous Metronidazole

    NARCIS (Netherlands)

    Groothoff, Miriam V. R.; Hofmeijer, Jannette; Sikma, Maaike A.; Meulenbelt, Jan

    2010-01-01

    Background: Encephalopathy associated with metronidazole is rare and, in most cases, reversible following discontinuation. Objective: We describe a case of fatal encephalopathy after treatment with high-dose intravenous metronidazole and the potential causes of the irreversibility. Case summary: A 3

  12. Branched-chain amino acids for people with hepatic encephalopathy

    DEFF Research Database (Denmark)

    Gluud, Lise Lotte; Dam, Gitte; Les, Iñigo;

    2015-01-01

    -chain amino acids (BCAA) versus control interventions has evaluated if BCAA may benefit people with hepatic encephalopathy. OBJECTIVES: To evaluate the beneficial and harmful effects of BCAA versus any control intervention for people with hepatic encephalopathy. SEARCH METHODS: We identified trials through...

  13. Minimal hepatic encephalopathy characterized by parallel use of the continuous reaction time and portosystemic encephalopathy tests

    DEFF Research Database (Denmark)

    Lauridsen, M M; Schaffalitzky de Muckadell, O B; Vilstrup, H

    2015-01-01

    Minimal hepatic encephalopathy (MHE) is a frequent complication to liver cirrhosis that causes poor quality of life, a great burden to caregivers, and can be treated. For diagnosis and grading the international guidelines recommend the use of psychometric tests of different modalities (computer...

  14. New developments in classical microscopy; what can be expected for the official control?

    NARCIS (Netherlands)

    Raamsdonk, van L.W.D.; Pinotti, L.; Veys, P.; Bremer, M.G.E.G.; Hekman, W.E.; Kemmers-Voncken, A.E.M.; Campagnoli, A.; Paltanin, C.; Crespo, C.; Vliege, J.J.M.; Pinckaers, V.G.Z.; Jorgensen, J.S.

    2011-01-01

    The official control of animal proteins in feed is focused on the prevention of Bovine Spongiform Encephalopathy (mad cow disease). The current legislation of the European Union is planned to avoid the feeding of animal by-products to the same species as its origin (ban of cannibalism, or species-to

  15. A 2cM Genome-Wide Scan of European Holstein Cattle Affected by Classical BSE

    Science.gov (United States)

    Background Classical bovine spongiform encephalopathy (BSE) is an acquired prion disease that is invariably fatal in cattle and has been implicated as a significant human health risk. Polymorphisms that alter the prion protein of sheep or humans have been associated with variations in transmissibl...

  16. Food risks and consumer trust : European governance of Avian influenza

    NARCIS (Netherlands)

    Krom, de M.P.M.M.

    2010-01-01

    During the 1990s, many European countries faced one or more food crises, such as bovine spongiform encephalopathy (BSE), E. coli, dioxin residues, and foot-and-mouth disease. These crises were marked by a growing public recognition of food-related risks and the changing nature of these risks, and te

  17. 77 FR 44107 - Information From Foreign Regions Applying for Recognition of Animal Health Status

    Science.gov (United States)

    2012-07-27

    ... Federal Register (76 FR 81404-81408, Docket No. APHIS-2007-0158) a proposal \\1\\ to amend the regulations... where the risk of bovine spongiform encephalopathy is neither negligible nor controlled. He stated that... that foreign country, stating that multinational meat packers might lobby APHIS to conduct...

  18. 76 FR 1592 - Submission for OMB Review; Comment Request

    Science.gov (United States)

    2011-01-11

    ... meat products produced by the use of AMR systems is free from Bovine Spongiform Encephalopathy (BSE... Federal Meat Inspection Act (FMIA) (21 U.S.C. 601 et seq.) This statutes mandate that FSIS protect the public by ensuring that meat products are safe, wholesome, not adulterated, and properly labeled...

  19. 9 CFR 94.15 - Animal products and materials; movement and handling.

    Science.gov (United States)

    2010-01-01

    ... FEVER, SWINE VESICULAR DISEASE, AND BOVINE SPONGIFORM ENCEPHALOPATHY: PROHIBITED AND RESTRICTED... Act (7 U.S.C. 8301 et seq.). (d) Meat and other products of ruminants or swine from regions listed in... part are met. (e) Any meat or other animal products not otherwise eligible for entry into the...

  20. 9 CFR 95.1 - Definitions.

    Science.gov (United States)

    2010-01-01

    ... in the FSIS regulations at 9 CFR 310.22(a). Suspect for a transmissible spongiform encephalopathy. (1..., hoof meal, horn meal, blood meal, meat meal, tankage, glands, organs, or other parts or products of... animals. Bone meal means ground animal bones and hoof meal and horn meal. Bovine. Bos taurus, Bos...

  1. BSE : the European regulatory context.

    Science.gov (United States)

    Chalus, T; Peutz, I

    2000-10-01

    The Bovine Spongiform Encephalopathy crisis provoked a fundamental re-appraisal of the way in which the European Community approaches matters of food safety. Between 28 July 1989, when restrictions on the dispatch of certain live cattle from the UK starte PMID:12631966

  2. Evaluation of the cumulative evidence for freedom from BSE in birth cohorts

    DEFF Research Database (Denmark)

    Böhning, Dankmar; Greiner, Matthias

    2006-01-01

    Substantial resources are used for surveillance of bovine spongiform encephalopathy (BSE) despite an extremely low detection rate, especially in healthy slaughtered cattle. We have developed a method based on the geometric waiting time distribution to establish and update the statistical evidence...

  3. Epidemiological models to support animal disease surveillance activities

    DEFF Research Database (Denmark)

    Willeberg, Preben; Paisley, Larry; Lind, Peter

    2011-01-01

    the risk of future cases, detected and undetected, of a waning infection of bovine spongiform encephalopathy. Both studies were successful in advancing European policy changes to reduce the cost of surveillance to appropriate levels given the magnitude of the respective hazards....

  4. Recent production of candidate reference materials at IRMM

    Energy Technology Data Exchange (ETDEWEB)

    Kramer, G.N.; Pauwels, J.; Le Guern, L.; Schimmel, H.; Trapmann, S. [Commission of the European Communities, Geel (Belgium). Joint Research Centre

    2001-06-01

    In the execution of its mission to promote a common European measurement system in support of EU policies, IRMM's Reference Materials Unit is currently involved in preparation of proficiency-testing samples and candidate reference materials. Recent work related to bovine spongiform encephalopathy in cows, genetically modified organisms, and a variety of environmental materials is described. (orig.)

  5. Practical experiences with animal fat as added fuel in the Ingolstadt power station; Praktische Erfahrungen bei der versuchsweisen Mitverbrennung von Tierfett im Kraftwerk Ingolstadt

    Energy Technology Data Exchange (ETDEWEB)

    Gerischer, C.N. [E.ON Kraftwerke GmbH, Kraftwerksgruppe Ingolstadt/Irsching, Grossmehring (Germany)

    2004-07-01

    In 2001 the market was overflowing with animal fat due to the discussion about the bovine spongiform encephalopathy disease. One solution was the use as additional fuel in power plants. The fundamental technical requirements existed in Ingolstadt power station. Economical considerations led to the decision to test animal fat as added fuel. The main experiences are presented and discussed. (orig.)

  6. Creutzfeldt-Jakob Disease Fact Sheet for Healthcare Workers and Morticians

    Science.gov (United States)

    ... Career Awards Enhancing Diversity Find People About NINDS Creutzfeldt-Jakob Disease Fact Sheet for Healthcare Workers and Morticians Description ... form of CJD (vCJD), largely in Britain, to mad cow disease (bovine spongiform encephalopathy or BSE), a deadly brain ...

  7. 76 FR 4602 - Declaration of Prion as a Pest Under FIFRA and Amendment of EPA's Regulatory Definition of Pests...

    Science.gov (United States)

    2011-01-26

    ... commonly designated by the type of diseases they produce, such as PrP\\Sc\\ (prions associated with scrapie) and PrP\\BSE\\ (prions associated with bovine spongiform encephalopathy--mad cow disease). In the disease process, prions (such as PrP\\sc\\) recruit normal prion proteins (PrP\\c\\) and convert them...

  8. BSE v Sloveniji

    OpenAIRE

    Batagelj, Zenel; Hohkraut, Tomaž

    2014-01-01

    Namen raziskave je ugotoviti stališče anketiranih do aktualnega vprašanja pojava BSE, Bovine Spongiform Encephalopathy imenovane tudi "bolezen norih krav". Predvsem ali še vedno uživajo goveje meso, o nakupu govedine neposredno na kmetiji in mnenje t.i. črnem zakolu.

  9. 76 FR 71294 - Prions; Proposed Amendment To Clarify Product Performance Data for Products With Prion-Related...

    Science.gov (United States)

    2011-11-17

    ... types of prions are commonly designated by the type of diseases they produce, such as PrP\\Sc\\ (prions associated with scrapie) and PrP\\BSE\\ (prions associated with bovine spongiform encephalopathy--mad cow disease). In the disease process, prions (such as PrP\\sc\\) recruit normal prion proteins (PrP\\c\\)...

  10. 78 FR 13501 - Declaration of Prion as a Pest Under FIFRA; Related Amendments; and Availability of Final Test...

    Science.gov (United States)

    2013-02-28

    ... types of prions are commonly designated by the type of diseases they produce, such as PrP\\Sc\\ (prions associated with scrapie) and PrP\\BSE\\ (prions associated with bovine spongiform encephalopathy--mad cow disease). In the disease process, prions (such as PrP\\sc\\) recruit normal prion proteins (PrP\\c\\)...

  11. Cow Disease in America

    Institute of Scientific and Technical Information of China (English)

    邹国如

    2004-01-01

    Last Wednesday,Agriculture Secretary Ann Veneman announced the first case of mad cow disease in the United States.A test seemed to show bovine spongiform encephalopathy,or B-S-E.in a cow from Washington state.The next day,a laboratory in Waybridge,England,Confirmed(证实) the case。

  12. Cost to Benefit - Fish Skin Yields Unique Gelatin Products

    Science.gov (United States)

    Gelatin is traditionally produced by hydrolysis of bones and skins from cattle and pigs. This can create problems for people with kosher and halal dietary restrictions, and also carries the potential of health hazards associated with outbreaks of bovine spongiform encephalopathy. The gelatins of col...

  13. 9 CFR 94.16 - Milk and milk products.

    Science.gov (United States)

    2010-01-01

    ... are not limited to condensed milk, long-life milks such as sterilized milk, casein and caseinates... 9 Animals and Animal Products 1 2010-01-01 2010-01-01 false Milk and milk products. 94.16 Section... VESICULAR DISEASE, AND BOVINE SPONGIFORM ENCEPHALOPATHY: PROHIBITED AND RESTRICTED IMPORTATIONS § 94.16...

  14. Prion infected Meat-and-Bone Meal is still infectious after biodiesel production

    Science.gov (United States)

    The epidemic of bovine spongiform encephalopathy (BSE) has led to world-wide drop in the market for beef by-products, such as Meat-and-Bone Meal (MBM), a fat-containing product traditionally used as an animal feed supplement. While normal rendering is insufficient, the production of biodiesel from M...

  15. 77 FR 1388 - Lists of Regions Classified With Respect to Certain Animal Diseases and States Approved To...

    Science.gov (United States)

    2012-01-10

    ... FEVER, CLASSICAL SWINE FEVER, SWINE VESICULAR DISEASE, AND BOVINE SPONGIFORM ENCEPHALOPATHY: PROHIBITED.... A single region of Europe recognized by APHIS as low risk for classical swine fever. (1) A list of... equivalent with respect to classical swine fever to the risk profile for the region it is...

  16. 9 CFR 94.20 - Gelatin derived from horses or swine, or from ruminants that have not been in any region where...

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 1 2010-01-01 2010-01-01 false Gelatin derived from horses or swine... § 94.20 Gelatin derived from horses or swine, or from ruminants that have not been in any region where bovine spongiform encephalopathy exists. Gelatin derived from horses or swine, or from ruminants...

  17. New developments in the detection and identification of processed animal proteins in feeds

    NARCIS (Netherlands)

    Raamsdonk, van L.W.D.; Holst, von C.; Baeten, V.; Berben, G.; Boix, A.; Jong, de J.

    2007-01-01

    It is generally accepted that the most likely route of infection of cattle with bovine spongiform encephalopathy (BSE) is by consumption of feeds containing low levels of processed animal proteins (PAPs). This likely route of infection resulted in feed bans, which were primarily aimed at ruminant fe

  18. Histopathological and imaging modifications in chronic ethanolic encephalopathy.

    Science.gov (United States)

    Folescu, Roxana; Zamfir, Carmen Lăcrămioara; Sişu, Alina Maria; Motoc, Andrei Gheorghe Marius; Ilie, Adrian Cosmin; Moise, Marius

    2014-01-01

    Chronic abuse of alcohol triggers different types of brain damage. The Wernicke-Korsakoff syndrome gets together Wernicke's encephalopathy and Korsakoff's syndrome. Another type of encephalopathy associated with chronic ethanol consumption is represented by the Marchiafava-Bignami malady or syndrome, an extremely rare neurological disorder, which is characterized by a demielinization of corpus callosum, extending as far as a necrosis. Because the frequency of ethanolic encephalopathy is increased and plays a major role in the sudden death of ethanolic patients, we have studied the chronic ethanolic encephalopathy both in deceased and in living patients, presenting different pathologies related to the chronic ethanol consumption. The present study investigated the effects of chronic ethanolic encephalopathy on the central nervous system based both on the histopathological exam of the tissular samples and the imaging investigation, such as MRI and CT. PMID:25329105

  19. Extraneural Prion Neuroinvasion without Lymphoreticular System Infection

    OpenAIRE

    Bartz, Jason C.; DeJoia, Crista; Tucker, Tammy; Kincaid, Anthony E.; Bessen, Richard A.

    2005-01-01

    While prion infection of the lymphoreticular system (LRS) is necessary for neuroinvasion in many prion diseases, in bovine spongiform encephalopathy and atypical cases of sheep scrapie there is evidence to challenge that LRS infection is required for neuroinvasion. Here we investigated the role of prion infection of LRS tissues in neuroinvasion following extraneural inoculation with the HY and DY strains of the transmissible mink encephalopathy (TME) agent. DY TME agent infectivity was not de...

  20. Post-dengue encephalopathy and Parkinsonism.

    Science.gov (United States)

    Fong, Choong Yi; Hlaing, Chaw Su; Tay, Chee Geap; Ong, Lai Choo

    2014-10-01

    Parkinsonism as a neurologic manifestation of dengue infection is rare with only 1 reported case in an adult patient. We report a case of a 6-year-old child with self-limiting post-dengue encephalopathy and Parkinsonism. This is the first reported pediatric case of post-dengue Parkinsonism and expands the neurologic manifestations associated with dengue infection in children. Clinicians should consider the possibility of post-dengue Parkinsonism in children with a history of pyrexia from endemic areas of dengue. PMID:24776518

  1. Thalamic hemorrhage in ischemic anoxic encephalopathy

    International Nuclear Information System (INIS)

    The authors have performed MR imaging, CT, and US examinations in eight infants with ischemic anoxic encephalopathy (IAE). The patients were delivered at 39-42 weeks. IAE was diagnosed by reduced Apgar score, presence of respiratory difficulty, and convulsive episodes. High-signal-intensity lesions were seen in the thalamus with inversion recovery sequences and low-signal-intensity areas were seen with PS1660/193 sequences in six of the eight infants. CT and US were positive for hemorrhage in only three of the infants. Of MR imaging, CT, and US, MR imaging was the most sensitive technique for detecting thalamic hemorrhage in IAE

  2. Chronic Traumatic Encephalopathy and Movement Disorders: Update.

    Science.gov (United States)

    Tarazi, Apameh; Tator, Charles H; Tartaglia, Maria Carmela

    2016-05-01

    Association of repetitive brain trauma with progressive neurological deterioration has been described since the 1920s. Punch drunk syndrome and dementia pugilistica (DP) were introduced first to explain symptoms in boxers, and more recently, chronic traumatic encephalopathy (CTE) has been used to describe a neurodegenerative disease in athletes and military personal with a history of multiple concussions. Although there are many similarities between DP and CTE, a number of key differences are apparent especially when comparing movement impairments. The aim of this review is to compare clinical and pathological aspects of DP and CTE with a focus on disorders of movement. PMID:27021775

  3. Qualifying and quantifying minimal hepatic encephalopathy

    DEFF Research Database (Denmark)

    Morgan, Marsha Y; Amodio, Piero; Cook, Nicola A;

    2016-01-01

    gold standard for the diagnosis of this syndrome. As these patients have, by definition, no recognizable clinical features of brain dysfunction, the primary prerequisite for the diagnosis is careful exclusion of clinical symptoms and signs. A large number of psychometric tests/test systems have been...... evaluated in this patient group. Of these the best known and validated is the Portal Systemic Hepatic Encephalopathy Score (PHES) derived from a test battery of five paper and pencil tests; normative reference data are available in several countries. The electroencephalogram (EEG) has been used to diagnose...

  4. Cardiovascular dysfunction in infants with neonatal encephalopathy.

    LENUS (Irish Health Repository)

    Armstrong, Katey

    2012-04-01

    Severe perinatal asphyxia with hypoxic ischaemic encephalopathy occurs in approximately 1-2\\/1000 live births and is an important cause of cerebral palsy and associated neurological disabilities in children. Multiorgan dysfunction commonly occurs as part of the asphyxial episode, with cardiovascular dysfunction occurring in up to a third of infants. This narrative paper attempts to review the literature on the importance of early recognition of cardiac dysfunction using echocardiography and biomarkers such as troponin and brain type natriuretic peptide. These tools may allow accurate assessment of cardiac dysfunction and guide therapy to improve outcome.

  5. Post-partum posterior reversible encephalopathy syndrome

    DEFF Research Database (Denmark)

    Aaen, Anne Albers; Jeppesen, Jørgen; Obaid, Hayder;

    2015-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a complex clinical condition with vasogenic subcortical oedema caused by hypertension. Oedema is often seen on magnetic resonance imaging. The wide clinical spectrum ranges from headaches to vision loss and even death. Early diagnosis and...... treatment is important for the reversibility of the condition. In this case report we emphasize the importance of blood pressure control in a post-partum woman, who had a rather complicated pregnancy. The symptoms of PRES were not recognized immediately because of failure to use and acknowledge a blood...

  6. Kuru: A Journey Back in Time from Papua New Guinea to the Neanderthals’ Extinction

    Directory of Open Access Journals (Sweden)

    Pawel P. Liberski

    2013-07-01

    Full Text Available Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008. In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century.

  7. Doenças priônicas: avaliação dos riscos envolvidos na utilização de produtos de origem bovina Prionic disease: evaluation of the risks involved in using products of bovine origin

    Directory of Open Access Journals (Sweden)

    Omar Lupi

    2003-02-01

    Full Text Available Os príons são proteínas que se mostram capazes de auto-replicação apesar de, para isso, alterar o metabolismo celular. São responsáveis por inúmeras doenças em animais e no ser humano (doenças priônicas, todas elas fatais. Essas moléstias apresentam enorme variabilidade quanto ao período de incubação, de alguns meses a 40 anos. Os príons acumulam-se e destroem os neurônios, provocando quadros conhecidos como encefalopatias espongiosiformes. Discute-se a apresentação clínica, epidemiológica e histórica das doenças priônicas. O foco maior de discussão recai, no entanto, na possibilidade teórica da transmissão iatrogênica dos príons por meio das formulações tópicas que utilizam ceramidas (cerebrosídeos ou placenta de origem bovina, assim como pelo risco representado por alguns procedimentos dermatológicos, como transplantes da pele e implantes de colágeno.A prion is a protein that is capable of self replication, thereby altering a cell's metabolism. It is responsible for a number of human and animal diseases (prionic diseases, all of which are always lethal. These diseases have enormous variability in their incubation periods, ranging from a few months to forty years. Prions accumulate and destroy nerve cells, causing spongiform encephalopathy. We discuss the clinical picture, epidemiology, and historical background of prionic diseases. The major focus of the discussion lies, however, on the theoretical possibility of iatrogenic transmission of prion infection due to topical formulations using ceramides (cerebrosides or placenta of bovine origin, as well as the risk represented by some dermatological procedures such as skin grafts and collagen implants.

  8. Ohtahara syndrome: Early infantile epileptic encephalopathy

    Directory of Open Access Journals (Sweden)

    Knežević-Pogančev Marija

    2008-01-01

    Full Text Available DEFINITION Ohtahara syndrome (early infantile epileptic encephalopathy with suppression bursts, is the earliest developing form of epileptic encephalopathy. ETHIOLOGY It considered to be a result of static structural developing brain damage. CLINICAL PICTURE Variable seizures develop mostly within the first 10 days of life, but may occur during the first hour after delivery. The most frequently observed seizure type are epileptic spasms, which may be either generalized and symmetrical or lateralized. The tonic spasms may occur in clusters or singly, while awake and during sleep alike. The duration of spasms is up to 10 seconds, and the interval between spasms within cluster ranges from 9 to 15 seconds. In one third of cases, other seizure types include partial motor seizures or hemiconvulsions The disorder takes a progressively deteriorating course with increasing frequency of seizures and severe retardation of psychomotor development. DIAGNOSTIC WORKUP In the initial stage of Ohtahara syndrome, interictal EEG shows a pattern of suppression-burst with high-voltage paroxysmal discharges separated by prolonged periods of nearly flat tracing that last for up to 18 seconds. PROGNOSIS AND THREATMENT Half of the reported children having Ohtahara syndrome die in infancy. Anticonvulsant helps little in controlling the seizures and halting the deterioration of psychomotor development. Severe psychomotor retardation is the rule. With time, the disorder may evolve into West syndrome or partial epilepsy. Psychomotor development may be slightly better if the infants do not develop West and later Lennox-Gastaut syndrome.

  9. The why and wherefore of hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Grover VPB

    2015-12-01

    Full Text Available Vijay PB Grover, Joshua M Tognarelli, Nicolas Massie, Mary ME Crossey, Nicola A Cook, Simon D Taylor-Robinson Liver Unit, Division of Diabetes, Endocrinology and Metabolism, Department of Medicine, Imperial College London, London, UK Abstract: Hepatic encephalopathy is a common neuropsychiatric abnormality, which complicates the course of patients with liver disease. It was probably first described by Hippocrates over 2000 years ago, who said that "those whose madness arises from phlegm are quiet and neither shout nor make a disturbance, while those whose madness arises from bile shout, play tricks and will not keep still, but are always up to some mischief". He was presumably describing the differences between patients with pneumonia and acute liver failure. Despite the fact that the syndrome was probably first recognized thousands of years ago, the exact pathogenesis still remains unclear. Furthermore, a precise definition of the syndrome is lacking, as are definitive methods of diagnosing this condition. It is important as both patients with cirrhosis and the general population with whom they interact may be affected as a consequence. At a minimum, the individual may be affected by impaired quality of life, impaired ability to work, and slowed reaction times, which are relevant to the population at large if affected individuals operate heavy machinery or drive a car. Pathogenic mechanisms, diagnostic tools, and treatment options are discussed. Keywords: hepatic encephalopathy, cirrhosis, ammonia, pathology, treatment, rifaximin, lactulose

  10. Experimental models of hepatic encephalopathy: ISHEN guidelines.

    Science.gov (United States)

    Butterworth, Roger F; Norenberg, Michael D; Felipo, Vicente; Ferenci, Peter; Albrecht, Jan; Blei, Andres T

    2009-07-01

    Objectives of the International Society for Hepatic Encephalopathy and Nitrogen Metabolism Commission were to identify well-characterized animal models of hepatic encephalopathy (HE) and to highlight areas of animal modelling of the disorder that are in need of development. Features essential to HE modelling were identified. The best-characterized animal models of HE in acute liver failure, the so-called Type A HE, were found to be the hepatic devascularized rat and the rat with thioacetamide-induced toxic liver injury. In case of chronic liver failure, surgical models in the rat involving end-to-side portacaval anastomosis or bile duct ligation were considered to best model minimal/mild (Type B) HE. Unfortunately, at this time, there are no satisfactory animal models of Type C HE resulting from end-stage alcoholic liver disease or viral hepatitis, the most common aetiologies encountered in patients. The commission highlighted the urgent need for such models and of improved models of HE in chronic liver failure in general as well as a need for models of post-transplant neuropsychiatric disorders. Studies of HE pathophysiology at the cellular and molecular level continue to benefit from in vitro and or ex vivo models involving brain slices or exposure of cultured cells (principally cultured astrocytes) to toxins such as ammonia, manganese and pro-inflammatory cytokines. More attention could be paid in the future to in vitro models involving the neurovascular unit, microglia and neuronal co-cultures in relation to HE pathogenesis. PMID:19638106

  11. Creutzfeldt-Jakob disease: an emergency department presentation of a rare disease.

    Science.gov (United States)

    Prince, Louise A; Mann, Deborah; Reilly, Tracey

    2006-07-01

    Creutzfeldt-Jakob Disease (CJD) is one of a group of neurodegenerative disorders causing spongiform encephalopathies. CJD is the most common human transmissible spongiform encephalopathy, or prion disease, but has an annual incidence of only 0.4-1.8 cases per million population worldwide. The prognosis for this disease is very poor and there is currently no cure. Patients typically present with non-specific neurological or psychiatric complaints and often have multiple physician visits before diagnosis, which requires histological examination of brain tissue. This patient had serial presentations to our Emergency Department, with progressive symptoms and multiple laboratory and radiological tests as well as consults, but her diagnosis remained unclear until her disease rapidly progressed and a brain biopsy was performed. With increasing concerns about prion diseases such as bovine spongiform encephalopathy (BSE)-or mad cow disease-and CJD, awareness of the symptoms and diagnostic challenges associated with these diseases will be helpful to emergency physicians. PMID:16798153

  12. Unlocking the bovine genome

    Science.gov (United States)

    The draft genome sequence of cattle (Bos taurus) has now been analyzed by the Bovine Genome Sequencing and Analysis Consortium and the Bovine HapMap Consortium, which together represent an extensive collaboration involving more than 300 scientists from 25 different countries. ...

  13. Current concepts in the assessment and treatment of hepatic encephalopathy.

    LENUS (Irish Health Repository)

    Cash, W J

    2012-02-01

    Hepatic encephalopathy (HE) is defined as a metabolically induced, potentially reversible, functional disturbance of the brain that may occur in acute or chronic liver disease. Standardized nomenclature has been proposed but a standardized approach to the treatment, particularly of persistent, episodic and recurrent encephalopathy associated with liver cirrhosis has not been proposed. This review focuses on the pathogenesis and treatment of HE in patients with cirrhosis. The pathogenesis and treatment of hepatic encephalopathy in fulminant hepatic failure is quite different and is reviewed elsewhere.

  14. Dengue viral infections as a cause of encephalopathy

    Directory of Open Access Journals (Sweden)

    Malavige G

    2007-01-01

    Full Text Available The aim of this study was to determine the clinical characteristics and poor prognostic factors associated with high mortality in dengue encephalopathy. Fifteen patients with confirmed dengue infections, who developed encephalopathy, were recruited from two tertiary care hospitals in Colombo, Sri Lanka. Among the factors that contributed to encephalopathy were: Acute liver failure (73%, electrolyte imbalances (80% and shock (40%. Five (33.3% patients developed seizures. Disseminated intravascular coagulation was seen in five (33.3%. Secondary bacterial infections were observed in 8 (53.3% of our patients. The overall mortality rate was 47%.

  15. Camel and bovine chymosin

    DEFF Research Database (Denmark)

    Langholm Jensen, Jesper; Mølgaard, Anne; Navarro Poulsen, Jens Christian;

    2013-01-01

    Bovine and camel chymosin are aspartic peptidases that are used industrially in cheese production. They cleave the Phe105-Met106 bond of the milk protein κ-casein, releasing its predominantly negatively charged C-terminus, which leads to the separation of the milk into curds and whey. Despite...... having 85% sequence identity, camel chymosin shows a 70% higher milk-clotting activity than bovine chymosin towards bovine milk. The activities, structures, thermal stabilities and glycosylation patterns of bovine and camel chymosin obtained by fermentation in Aspergillus niger have been examined...... interactions arising from variation in the surface charges and the greater malleability both in domain movements and substrate binding contribute to the better milk-clotting activity of camel chymosin towards bovine milk....

  16. Leucine metabolism in patients with Hepatic Encephalopathy

    International Nuclear Information System (INIS)

    A primed continuous infusion of [15N, 1-13C]leucine was used to determine whether increased oxidation and/or protein synthesis of leucine occurs in patients with cirrhosis. Five controls and patients were equilibrated on a metabolic balance diet [0.6 g protein per kg ideal body weight (IBW)]. An additional four patients were equilibrated in the same manner with the same type of diet with a protein level of 0.75 g per kg IBW. Plasma leucine and breath CO2 enrichments were measured by mass spectrometry. Protein synthesis and leucine metabolism were identical in controls and patients when both were fed a diet with 0.6 g protein/kg IBW. Results indicate that systemic derangements of leucine metabolism are not the cause of Hepatic Encephalopathy

  17. Brain MRI findings in Wernicke encephalopathy.

    Science.gov (United States)

    Wicklund, Meredith R; Knopman, David S

    2013-08-01

    A 71-year-old woman with myelofibrosis on chemotherapy experienced an acute illness with nausea, vomiting, and diarrhea. Two weeks later, she developed an acute confusional state characterized by disorientation and fluctuating alertness with normal speech and language. Her neurologic examination demonstrated an upper motor neuron pattern of right hemiparesis. She reported double vision though ophthalmoparesis was not appreciated. Her gait was normal. While hospitalized, she developed generalized tonic-clonic seizures. Brain MRI revealed a small area of restricted diffusion of the left precentral gyrus (figure). She was diagnosed with a stroke with secondary seizures; however, as the confusional state resolved, she developed profound retrograde and anterograde amnesia. Review of the brain MRI showed high T2 signal in the medial thalamus and contrast enhancement of the mamillary bodies; a diagnosis of Wernicke-Korsakoff syndrome was entertained and she was started on thiamine replacement. The encephalopathy and hemiparesis resolved though she remains severely amnestic. PMID:24195023

  18. Transcranial electrostimulation in patients with alcoholic encephalopathy

    Directory of Open Access Journals (Sweden)

    Barylnik Yu.B.

    2010-09-01

    Full Text Available The method of transcranial electrostimulation (TES was used for treating patients with alcoholic encephalopathy against the background of the basic treatment, which includes nootropics, normotimics, soporifics, over-all strengthening therapy and other devices. The course of treatment consisted of 10 daily procedures lasting for 30 minutes. The TES influence was evaluated according to the clinical state, the neurologic status, including EEG (electroencephalogram, the psychometric scales were also used for evaluating the manifestation of depression, anxiety and working memory in comparison with appropriate indices in the control group of patients, who were being treated by the traditional method. TES led to normalization of health state, neurologic status and vegetative innervation, the reduction in pathologic inclination, which corresponded to general improvement of the state of patients, EEG indices and psychometric scales

  19. Posterior reversible encephalopathy syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Kostić Dejan

    2015-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is characterized by the following symptoms: seizures, impaired consciousness and/or vision, vomiting, nausea, and focal neurological signs. Diagnostic imaging includes examination by magnetic resonance (MR and computed tomography (CT, where brain edema is visualized bi-laterally and symmetrically, predominantly posteriorly, parietally, and occipitally. Case report. We presented a 73-year-old patient with the years-long medical history of hipertension and renal insufficiency, who developed PRES with the symptomatology of the rear cranium. CT and MR verified changes in the white matter involving all lobes on both sides of the brain. After a two-week treatment (antihypertensive, hypolipemic and rehydration therapy clinical improvement with no complications occurred, with complete resolution of changes in the white matter observed on CT and MR. Conclusion. PRES is a reversible syndrome in which the symptoms withdraw after several days to several weeks if early diagnosis is made and appropriate treatment started without delay.

  20. Сhanges of brain bioelectric activity by diabetic encephalopathy

    Directory of Open Access Journals (Sweden)

    Vitaly P. Omelchenko

    2011-05-01

    Full Text Available This article focuses on the analysis of brain bioelectric activity by diabetic encephalopathy. Paid attention to the establishment of EEG parameters and patients psychological characteristics interrelation.

  1. Posterior encephalopathy subsequent to cyclosporin A presenting as irreversible abulia.

    Science.gov (United States)

    Nishie, Makoto; Kurahashi, Kozo; Ogawa, Masaya; Yoshida, Yasuji; Midorikawa, Hiroshi

    2003-08-01

    A case of cyclosporin A (Cys A)-induced posterior encephalopathy developed into persistent abulia despite rapid and marked improvement of abnormal T2- and FLAIR MRI hyperintense regions. Diffusion-weighted MRI signal intensity was also high at the onset. This change is atypical in Cys A-induced encephalopathy and was thought to predict poor recovery from the encephalopathy. Persistent abulia was probably due to marked hypoperfusion in the whole cortex including bilateral frontal lobes and basal ganglia as detected by SPECT. Apart from the breakdown of the blood-brain barrier, direct toxicity of Cys A to the brain may play a role in the pathogenesis of chronic, irreversible encephalopathy. PMID:12924507

  2. Acute Necrotizing Encephalopathy of Childhood; A Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Reza SALEHIOMRAN

    2013-06-01

    Full Text Available How to Cite this Article: Salehi Omran MR, Nooreddini H, Baghdadi F. Acute Necrotizing Encephalopathy of Childhood; A Case Report. Iran J Child Neurol. 2013 Spring;7(2:51-54. AbstractAcute Necrotizing Encephalopathy of Childhood (ANEC is an atypical disease followed by respiratory or gastrointestinal infection, high fever, which is accompanied with rapid alteration of consciousness and seizures. This disease is seen nearly exclusively in East Asian infants and children who had previously a good health. Serial MRI examinations demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. This disease has a poor prognosis, often culminating in profound morbidity and mortality. A 22-month infant with ANEC hospitalized in Amirkola Children Hospital has been evaluated. References1. Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997 Mar;19(2:81-92. Review.2. Wang HS, Huang SC. Acute necrotizing encephalopathy of childhood. Chang Gung Med J 2001 Jan;24(1:1-10.3. Campistol J, Gassió R, Pineda M, Fernandez-Alvarez E. Acute necrotizing encephalopathy of childhood (infantile bilateral  thalamic necrosis: two non-Japanese cases. Dev Med Child Neurol 1998 Nov;40(11:771-4.4. Ito Y, Ichiyama T, Kimura H, Shibata M, Ishiwada N, Kuroki H, Furukawa S, Morishima T. Detection of influenza virus RNA by reverse transcription-PCR and proinflammatory cytokines in influenza-virus-associated encephalopathy. J Med Virol 1999 Aug;58(4:420-5.5. Sugaya N. Influenza-associated encephalopathy in Japan. Semin Pediatr Infect Dis 2002 Apr;13(2:79-84. Review.6. Skelton BW, Hollingshead MC, Sledd AT, Phillips CD, Castillo M. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease. Pediatr Radiol 2008 Jul; 38(7:810-3.7. Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH. Acute necrotizing encephalopathy of childhood

  3. Safety, efficacy, and patient acceptability of rifaximin for hepatic encephalopathy.

    Science.gov (United States)

    Kimer, Nina; Krag, Aleksander; Gluud, Lise L

    2014-01-01

    Hepatic encephalopathy is a complex disease entity ranging from mild cognitive dysfunction to deep coma. Traditionally, treatment has focused on a reduction of ammonia through a reduced production, absorption, or clearance. Rifaximin is a nonabsorbable antibiotic, which reduces the production of ammonia by gut bacteria and, to some extent, other toxic derivatives from the gut. Clinical trials show that these effects improve episodes of hepatic encephalopathy. A large randomized trial found that rifaximin prevents recurrent episodes of hepatic encephalopathy. Most patients were treated concurrently with lactulose. Trials have varied greatly in design, outcomes, and duration of treatment regimes. Although a number of retrospective studies have indicated that long-term treatment with rifaximin is safe and possibly beneficial, high quality trials are needed to further clarify efficacy and safety of long-term treatment with rifaximin and evaluate effects of combination therapy with lactulose and branched-chain amino acids for patients with liver cirrhosis and hepatic encephalopathy. PMID:24672227

  4. Genetics Home Reference: acute necrotizing encephalopathy type 1

    Science.gov (United States)

    ... are known to trigger this condition include human herpesvirus 6, coxsackie virus, and enteroviruses. In rare cases, ... Registry (1 link) Encephalopathy, acute, infection-induced Scientific articles on PubMed (1 link) PubMed OMIM (1 link) ...

  5. Frontal Lobe MRI Abnormalities in HHV-6 Encephalopathy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-01-01

    Full Text Available Magnetic resonance imaging and SPECT findings in 10 children with a diagnosis of human herpesvirus 6 (HHV-6 encephalopathy are reported from Jikei University School of Medicine, Tokyo; and Saitama Children's Medical Center, Japan.

  6. Neuroprotection of aucubin in primary diabetic encephalopathy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Hippocampal neuronal apoptosis accompanied by impairment of cognitive function occurs in primary diabetic encephalopathy. In this study, we investigated the neuroprotective mechanism of the iridoid glycoside, aucubin, using rats (n=8). Diabetes mellitus was induced in the rats by intraperitoneal (i.p.) injection of streptozotocin (60 mg/kg body weight). After 65 d, half of the DM rats were administered aucubin (5 mg/kg; i.p.) for 15 d, yielding treatment DM+A. A third group of rats received no strepto- zotocin or aucibin, and served as controls (CON). Encephalopathy was assessed using Y-maze be- havioral testing. Rats were euthanized on Day 87, and hippocampi were excised for visual (light and transmission electron microscopic) and immunochemical (Western blot; immunohistochemical) as- sessments of the CA1 subfield for apoptosis and expression of regulatory proteins Bcl-2 and Bax. Treatment responses to all the parameters examined (body weight, plasma glucose, Y-maze error rates, pyramidal cell ultrastructure, proportions of apoptotic cells, levels of expression of Bcl-2 and Bax, and survivability of neuronal cells) were identical: there were highly significant differences between DM and CON groups (P<0.001), but the effects were significantly moderated (P<0.01) in DM+A compared with DM. These findings confirm the association of apoptosis with the encephalopathic effects of diabetes mellitus, and suggest a major role of the expression levels of Bcl-2 and Bax in the regulation of apop- totic cell death. All of the results suggest that aucubin could effectively inhibit apoptosis by modulating the expressions of Bcl-2 and Bax genes.

  7. Neuroprotection of aucubin in primary diabetic encephalopathy

    Institute of Scientific and Technical Information of China (English)

    XUE HongYu; JIN LiJi; JIN Lei; ZHANG Peng; LI DanQing; XIA YanQiu; LU YaNan; XU YongPing

    2008-01-01

    Hippocampal neuronal apoptosis accompanied by impairment of cognitive function occurs in primary diabetic encephalopathy. In this study, we investigated the neuroprotective mechanism of the iridoid glycoside, aucubin, using rats (n=8). Diabetes mellitus was induced in the rats by intraperitoneal (i.p.) injection of streptozotocin (60 mg/kg body weight). After 65 d, half of the DM rats were administered aucubin (5 mg/kg;i.p.) for 15 d, yielding treatment DM+A. A third group of rats received no strepto-zotocin or aucibin, and served as controls (CON). Encephalopathy was assessed using Y-maze be-havioral testing. Rats were euthanized on Day 87, and hippocampi were excised for visual (light and transmission electron microscopic) and immunochemical (Western blot;immunohistochemical) as-sessments of the CA1 subfield for apoptosis and expression of regulatory proteins Bcl-2 and Bax. Treatment responses to all the parameters examined (body weight, plasma glucose, Y-maze error rates, pyramidal cell ultrastructure, proportions of apoptotic cells, levels of expression of Bcl-2 and Bax, and survivability of neuronal cells) were identical: there were highly significant differences between DM and CON groups (P<0.001), but the effects were significantly moderated (P<0.01) in DM+A compared with DM. These findings confirm the association of apoptosis with the encephalopathic effects of diabetes mellitus, and suggest a major role of the expression levels of Bcl-2 and Bax in the regulation of apop-totic cell death. All of the results suggesf that aucubin could effectively inhibit apoptosis by modulating the expressions of Bcl-2 and Bax genes.

  8. Posterior Reversible Encephalopathy Syndrome in Our Setup

    International Nuclear Information System (INIS)

    Objectives: To assess the clinical presentation and neuroimaging abnormalities in a series of patients diagnosed as posterior reversible encephalopathy syndrome at Military Hospital Rawalpindi. Study Design: Case series study. Place and Duration: Study was carried out at Military Hospital Rawalpindi form December 01st, 2011 to May 31st, 2012. Patients and Methods: Study included all the cases of the Posterior reversible encephalopathy syndrome (PRES) admitted in the wards and intensive care unit (ITC). Neuroimaging was done and all the studies were reviewed by independent neuroradiologist. Different clinical and laboratory variables were also studied and correlated with neuroimaging. Follow up ws done to look for the prognosis. Results: Of the seven patients labelled as PRES two were male and five were female. Two patients were over 50 years of age, out of them one was male and one was female. One patient had end stage renal disease (ESRD) secondary to diabetes mellitus (DM) and hypertension (HTN), one had eclampsia, one had pregnancy-induced hypertension (PIH) and one had just uncontrolled HTN. Peak spontaneous bacterial peritonitis (SBP) in 5 cases was 210 mm of Hg, four of which had seizures. Rest two had spontaneous bacterial peritonitis (SBP) of 160 out of which one developed seizures. Total out of 7, 5 experienced seizures and altered conscious state, rest two only had confusion. One patient had papilloedema. Follow up was done after 06 weeks, 02 patients died, 05 remained alive and symptoms of PRES had vanished. Conclusion: PRES is a neurological emergency, presents with a variety of symptoms and has a specific neuroimaging pattern. Early recognition and prompt treatment result in a good neurological outcome. (author)

  9. Pancreatic encephalopathy- a rare complication of severe acute biliary pancreatitis

    OpenAIRE

    Vlad Denis Constantin; Alexandru Carȃp; Bogdan Socea; Simona Bobic

    2014-01-01

    Background. Pancreatic encephalopathy is a rare complication of severe acute pancreatitis, with high mortality, being difficult to diagnose and treat, thus requiring continuous research regarding its management. Materials and Methods. Of 20 patients diagnosed with severe acute pancreatitis on admission at Department of Emergency and Admission (DEA), from January 1st 2010 to March 31st 2014, 5 cases complicated by pancreatic encephalopathy were analyzed using a descriptive observational...

  10. A case of Hashimoto's encephalopathy presenting with seizures and psychosis

    OpenAIRE

    Min-Joo Lee; Hae-Sang Lee; Jin-Soon Hwang; Da-Eun Jung

    2012-01-01

    Hashimoto’s encephalopathy (HE) is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic...

  11. Erythropoietin and hypothermia for hypoxic-ischemic encephalopathy

    OpenAIRE

    Rogers, EE; Bonifacio, SL; Glass, HC; Juul, SE; Chang, T.; Mayock, DE; Durand, DJ; Song, D.; Barkovich, AJ; Ballard, RA; Wu, YW

    2014-01-01

    © 2014 Elsevier Inc. All rights reserved. Background Erythropoietin is neuroprotective in animal models of neonatal hypoxic-ischemic encephalopathy. We previously reported a phase I safety and pharmacokinetic study of erythropoietin in neonates. This article presents the neurodevelopmental follow-up of infants who were enrolled in the phase I clinical trial. Methods We enrolled 24 newborns with hypoxic-ischemic encephalopathy in a dose-escalation study. Patients received up to six doses of er...

  12. Event-related evoked potentials in chronic respiratory encephalopathy

    OpenAIRE

    Tahan, A R Al; Zaidan, R.; Jones, S; Husain, A.; Mobeireek, A; Bahammam, A

    2010-01-01

    Background: Cognitive event-related potential (P300) is an index of cognitive processing time. It was found to be prolonged in dementia, renal, and hepatic encephalopathies, but was not extensively assessed in respiratory failure. Objective: To evaluate P300 changes in patients with respiratory failure, and especially those with mild or subclinical hypoxic–hypercapnic encephalopathy. Methods: Auditory event-related evoked potential P300 latency was measured using an oddball paradigm in patien...

  13. Bovine Herpesvirus 4 infections and bovine mastitis

    NARCIS (Netherlands)

    Wellenberg, Gerardus Johannus

    2002-01-01

    Mastitis is an often occurring disease in dairy cattle with an enormous economic impact for milk producers worldwide. Despite intensive research, which is historically based on the detection of bacterial udder pathogens, still around 20-35% of clinical cases of bovine mastitis have an unknown aetiol

  14. MRI features of patients with heroin spongiform leukoencephalopathy of different clinical stages

    International Nuclear Information System (INIS)

    Objective: To investigate radiological features of patients with heroin spongiform leukoencephalopathy (HSLE) of different clinical stages and discuss the evolutional characteristics of the disease. Methods: Thirty two patients with HSLE underwent precontrast MRI and postcontrast MRI. The history of addiction, clinical presentations, and brain MRI were analyzed and summarized according to the patient's clinical staging. There are 6 cases in I stage, 21 cases in II stage, 5 cases in III stage. Results: All patients had history of heroin vapor inhalation. Most of the cases developed subacute cerebellar impairment in earlier period. Brain MRI revealed symmetrical lesion within bilateral cerebellum in all patients. Splenium of the corpus callosum, posterior limb of the internal capsule, deep white matter of the occipital and parietal lobes, were gradually involved with progressive deterioration of HSLE. The brain stem and deep white matter of the frontal and temporal lobes were involved in some cases. Conclusions: The history of heated heroin vapor inhalation was the prerequisite for the diagnosis of HSLE. Brain MRI presented the characteristic lesion and its evolution of HSLE. Brain MRI was very important for accurate diagnosis and helpful to judge the clinical stages according to the involved brain region. (authors)

  15. Frequency of helicobacter pylori antibodies in porto-systemic encephalopathy,

    International Nuclear Information System (INIS)

    Objective: To study the frequency of Helicobacter pylori antibodies in patients presenting with porto-systemic encephalopathy due to liver disease. Patients and Methods: During the study period, seventy-six patients of porto-systemic encephalopathy due to liver diseases was selected. These subjects were evaluated for hepatic encephalopathy grade, modified Child-Pugh classification and were managed according to the standard practices. These patients were evaluated for Helicobacter (H. pylori) antibody status by ELlSA (Abbott Laboratories) method. Results: Out of 76 patients studied and tested for H. pylori antibodies, 48(63.2%) were males and 28(36.8%) were females with age ranging between 17 and 85 years. Out of 76 patients who presented with porto-systemic encephalopathy, 59(77.6%) had a positive H. pylori antibody test. Thirty-five of these were males and 24 were females. A significant number of patients who presented with higher grade of encephalopathy were H. pylori antibody positive (p<0.001). Conclusion: In this study, frequency of H. pylori antibodies was significantly high in patients of porto-systematic encephalopathy. (author)

  16. Developmental outcomes of newborn encephalopathy in the term infant.

    Science.gov (United States)

    Badawi, N; Keogh, J M; Dixon, G; Kurinczuk, J J

    2001-06-01

    Newborn encephalopathy is a clinically defined condition of abnormal neurological behaviours in the newborn period. Though most cases have their origin in the preconceptional and antepartum period, newborn encephalopathy represents a crucial link between intrapartum events and permanent neurological problems in the child. The birth prevalence of newborn encephalopathy ranges from 1.8 to 7.7 per 1000 term live births according to the definition used and the population to which it is applied. Few studies have investigated the outcomes of newborn encephalopathy other than for cases solely attributed to intrapartum hypoxia. These adverse outcomes range from death to cerebral palsy, intellectual disability, and less severe neurological disabilities such as learning and behavioural problems. Outcomes following newborn encephalopathy may vary from country to country with 9.1% of affected babies dying in the newborn period in Western Australia and 10.1% manifesting cerebral palsy by the age of two. These compare to a case fatality of 30.5% in Kathmandu and a cerebral palsy rate of 14.5% by one year of age. The study by Robertson et al which followed children with hypoxic ischaemic encephalopathy found an incidence of impairment of 16% among survivors assessed at 8 years with 42% requiring school resource room help or special classes. This review emphasises the great need for comprehensive clinical and educational assessment as these infants approach school entry to enable appropriate educational provisions to be made. PMID:11450384

  17. Efficacy and Safety of Porcine Collagen Filler for Nasolabial Fold Correction in Asians: A Prospective Multicenter, 12 Months Follow-up Study

    OpenAIRE

    Lee, Jung-Ho; Choi, Yong Sung; Kim, Sue-Min; Kim, Young-Jin; Rhie, Jong Won; Jun, Young-Joon

    2014-01-01

    Recently, injectable dermal fillers have become important alternatives to surgical procedures for the correction of facial wrinkles. Bovine collagen is the first approved material for filler injection, and several studies have shown its efficacy. However, the risk of developing an allergic reaction and xenogenic transmission of bovine spongiform encephalopathy remain among its disadvantages. In this randomized, double-blinded, split-face study, we compared the efficacy and safety of a porcine...

  18. Galvijų spongiforminės encefalopatijos ir virusinių ligų paplitimo, diagnostikos ir prevencijos retrospektyvi analizė Lietuvoje

    OpenAIRE

    Milius, Jonas

    2006-01-01

    Assessment of occurrence and diagnostic methods of viral diseases in cattle – viral diarrhoea (BVD), infectious bovine rhinotracheitis (IBR), rabies, enzootic bovine leukosis (EBL), and spongiform encephalopathy (BSE) – was carried out for the first time in Lithuania. It was established that viruses of rabies, infectious rhinotraheitis and viral diarrhoea are most widespread in the country. It was determined that occurrence of rabies in cattle is parallel with the infection of wildlife with r...

  19. Bovine Herpesvirus 4 infections and bovine mastitis

    OpenAIRE

    Wellenberg, Gerardus Johannus

    2002-01-01

    Mastitis is an often occurring disease in dairy cattle with an enormous economic impact for milk producers worldwide. Despite intensive research, which is historically based on the detection of bacterial udder pathogens, still around 20-35% of clinical cases of bovine mastitis have an unknown aetiology. Due to the high number of unknown causes of clinical mastitis, studies were undertaken to gain more insight into the role of viruses in this important disease. For the first time, we found tha...

  20. Risk factors for sepsis-associated encephalopathy

    Institute of Scientific and Technical Information of China (English)

    Jian Li; Ang Li; Yibing Weng; Shuwen Zhang; Meili Duan

    2011-01-01

    Sepsis-associated encephalopathy (SAE) is a diffuse and acute cerebral dysfunction caused by sepsis. Many sepsis patients exhibit acute deterioration in mental status during the early stage of disease, and central nervous system dysfunction has been shown to increase patient mortality. The present study selected 284 sepsis patients who were admitted to the Intensive Care Unit of Beijing Friendship Hospital, Capital Medical University, from January to December 2009. The patients were assigned to SAE and non-SAE patient groups according to SAE occurrence. SAE incidence was 37.68%, and mortality was significantly greater in SAE patients compared with non-SAE patients (41.12% vs. 17.51%, P < 0.01). Univariate analysis and multivariate logistic regression analysis indicated lower arterial partial pressure of oxygen and greater alanine aminotransferase and Acute Physiology and Chronic Health Evaluation II scores in the SAE group compared with the non-SAE group. Arterial partial pressure of oxygen, alanine aminotransferase, and Acute Physiology and Chronic Health Evaluation II scores were determined to be potential risk factors for SAE.

  1. Encephalopathy Associated With Autoimmune Thyroid Disease

    Directory of Open Access Journals (Sweden)

    li A. Raouf

    2014-07-01

    Full Text Available Autoimmune thyroid diseases (ATDs are immune-endocrine disorders affecting the thyroid gland and, eventually, also a number of other systemic targets, including the brain and the nervous system. Encephalopathy associated with autoimmune thyroid disease (EAATD is a rare, heterogeneous condition arising from the background of an ATD. It is characterised by neurological and/or psychiatric symptoms with acute or sub-acute onset, and virtually any neurological or psychiatric symptom can appear. However, EAATD often presents with confusion, altered consciousness, seizures, or myoclonus. The majority of cases are associated with Hashimoto’s thyroiditis, but a number of patients with Graves’ disease have also been described. EAATD is likely an immune-mediated disorder. Its exact prevalence has not been precisely elucidated, with an increasing number of cases reported in the last few years. Most EAATD patients respond in a dramatic manner to corticosteroids. However, the immunosuppressive treatment may require a long course (up to 12 months. The increasing number of EAATD cases reported in the literature demonstrates a growing interest of the scientific community about this condition, which still requires a better definition of its pathophysiology, the diagnostic criteria, and the most appropriate management, including the long-term follow-up of patients. The current clinical evidence about EAATD is mostly based on the report of single cases or small cohort studies. In this review, we present the current knowledge about EAATD, with a dedicated focus to the clinical management of the patients from a diagnostic and therapeutic perspective.

  2. The mechanisms and treatment of asphyxial encephalopathy

    Directory of Open Access Journals (Sweden)

    Guido eWassink

    2014-02-01

    Full Text Available Acute post-asphyxial encephalopathy occurring around the time of birth remains a major cause of death and disability. The recent seminal insight that allows active neuroprotective treatment is that even after profound asphyxia (the primary phase, many brain cells show initial recovery from the insult during a short latent phase, typically lasting approximately 6 h, only to die hours to days later after a secondary deterioration characterized by seizures, cytotoxic edema, and progressive failure of cerebral oxidative metabolism. Although many of these secondary processes are potentially injurious, they appear to be primarily epiphenomena of the ‘execution’ phase of cell death. Animal and human studies designed around this conceptual framework have shown that moderate cerebral hypothermia initiated as early as possible but before the onset of secondary deterioration, and continued for a sufficient duration to allow the secondary deterioration to resolve, has been associated with potent, long-lasting neuroprotection. Recent clinical trials show that while therapeutic hypothermia significantly reduces morbidity and mortality, many babies still die or survive with disabilities. The challenge for the future is to find ways of improving the effectiveness of treatment. In this review, we will dissect the known mechanisms of hypoxic-ischemic brain injury in relation to the known effects of hypothermic neuroprotection.

  3. Antibody-Mediated Autoimmune Encephalopathies and Immunotherapies.

    Science.gov (United States)

    Gastaldi, Matteo; Thouin, Anaïs; Vincent, Angela

    2016-01-01

    Over the last 15 years it has become clear that rare but highly recognizable diseases of the central nervous system (CNS), including newly identified forms of limbic encephalitis and other encephalopathies, are likely to be mediated by antibodies (Abs) to CNS proteins. The Abs are directed against membrane receptors and ion channel-associated proteins that are expressed on the surface of neurons in the CNS, such as N-methyl D-aspartate receptors and leucine-rich, glioma inactivated 1 protein and contactin-associated protein like 2, that are associated with voltage-gated potassium channels. The diseases are not invariably cancer-related and are therefore different from the classical paraneoplastic neurological diseases that are associated with, but not caused by, Abs to intracellular proteins. Most importantly, the new antibody-associated diseases almost invariably respond to immunotherapies with considerable and sometimes complete recovery, and there is convincing evidence of their pathogenicity in the relatively limited studies performed so far. Treatments include first-line steroids, intravenous immunoglobulins, and plasma exchange, and second-line rituximab and cyclophosphamide, followed in many cases by steroid-sparing agents in the long-term. This review focuses mainly on N-methyl D-aspartate receptor- and voltage-gated potassium channel complex-related Abs in adults, the clinical phenotypes, and treatment responses. Pediatric cases are referred to but not reviewed in detail. As there have been very few prospective studies, the conclusions regarding immunotherapies are based on retrospective studies. PMID:26692392

  4. The why and wherefore of hepatic encephalopathy.

    Science.gov (United States)

    Grover, Vijay Pb; Tognarelli, Joshua M; Massie, Nicolas; Crossey, Mary Me; Cook, Nicola A; Taylor-Robinson, Simon D

    2015-01-01

    Hepatic encephalopathy is a common neuropsychiatric abnormality, which complicates the course of patients with liver disease. It was probably first described by Hippocrates over 2000 years ago, who said that "those whose madness arises from phlegm are quiet and neither shout nor make a disturbance, while those whose madness arises from bile shout, play tricks and will not keep still, but are always up to some mischief ". He was presumably describing the differences between patients with pneumonia and acute liver failure. Despite the fact that the syndrome was probably first recognized thousands of years ago, the exact pathogenesis still remains unclear. Furthermore, a precise definition of the syndrome is lacking, as are definitive methods of diagnosing this condition. It is important as both patients with cirrhosis and the general population with whom they interact may be affected as a consequence. At a minimum, the individual may be affected by impaired quality of life, impaired ability to work, and slowed reaction times, which are relevant to the population at large if affected individuals operate heavy machinery or drive a car. Pathogenic mechanisms, diagnostic tools, and treatment options are discussed. PMID:26719720

  5. Pathogenetic aspects of alcoholic encephalopathy treatment

    Directory of Open Access Journals (Sweden)

    Shchetinin S.G.

    2010-12-01

    Full Text Available Alcohol is considered to be the most common exogenous toxins, causing encephalopathy. The defeat of almost all parts of the nervous system should be assigned to the special features of ethanol. Neurophysiological mechanisms of development of substance dependence are based in the stem and limbic structures of the brain that are involved in ensuring the regulation of emotional state, mood, motivation sphere, psychophysical tone of human behavior in general and its adaptation to the environment. Stress or disruption of the normal functioning of these structures can lead to the formation of abstinence syndrome, affective disorders in remission and craving for alcohol. Dopaminergic and opioid (endorphin system play an important role in the genesis of various mental and motor disorders. In some way alcohol dependence can be regarded as an endorfinodefitsitnoe disease with a pathogenetic point of view. Activating of opioidereal system by trans-cranial electrical stimulation promotes the restoration of disturbed emotional, cognitive and autonomic functions, reduces craving for alcohol and in that way increases the effectiveness of rehabilitation treatment

  6. Reversible splenial abnormality in hypoglycemic encephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hyun; Choi, Jeong Yoon; Koh, Seong-Beom [Korea University School of Medicine, Department of Neurology, Guro Hospital, Seoul (Korea); Lee, Younghen [Korea University School of Medicine, Department of Radiology, Ansan Hospital, Ansan City (Korea)

    2007-03-15

    Lesions involving the splenium of the corpus callosum (SCC) have been rarely reported in cases of hypoglycemic brain injury. We identified signal abnormalities in the SCC in three adult patients with hypoglycemic encephalopathy by using diffusion-weighted imaging (DWI) on a 1.5-T MR scanner. Repeat DWI was performed in all patients following a marked clinical improvement, and MR angiography and routine MRI were also performed. We examined each patient's detailed medical history and blood laboratory tests in order to exclude other conditions causing similar SCC abnormalities. Initial DWI was performed during which each patient showed altered mental status that was attributed to profound hypoglycemia. We observed an identical pattern of DWI abnormality characterized by high signals in the SCC with apparent diffusion coefficient reductions that were reversed completely within several days following appropriate correction of hypoglycemia. T2-weighted or FLAIR images also showed no residual lesion in the SCC and MR angiography was normal in all patients. These case reports suggest that the SCC should be added to the list of selective vulnerability to hypoglycemia and that hypoglycemia, in turn, be included in the differential diagnosis of reversible SCC abnormalities. (orig.)

  7. Reversible splenial abnormality in hypoglycemic encephalopathy

    International Nuclear Information System (INIS)

    Lesions involving the splenium of the corpus callosum (SCC) have been rarely reported in cases of hypoglycemic brain injury. We identified signal abnormalities in the SCC in three adult patients with hypoglycemic encephalopathy by using diffusion-weighted imaging (DWI) on a 1.5-T MR scanner. Repeat DWI was performed in all patients following a marked clinical improvement, and MR angiography and routine MRI were also performed. We examined each patient's detailed medical history and blood laboratory tests in order to exclude other conditions causing similar SCC abnormalities. Initial DWI was performed during which each patient showed altered mental status that was attributed to profound hypoglycemia. We observed an identical pattern of DWI abnormality characterized by high signals in the SCC with apparent diffusion coefficient reductions that were reversed completely within several days following appropriate correction of hypoglycemia. T2-weighted or FLAIR images also showed no residual lesion in the SCC and MR angiography was normal in all patients. These case reports suggest that the SCC should be added to the list of selective vulnerability to hypoglycemia and that hypoglycemia, in turn, be included in the differential diagnosis of reversible SCC abnormalities. (orig.)

  8. Hashimoto’s Encephalopathy Presenting with Acute Cognitive Dysfunction and Convulsion

    OpenAIRE

    Kang, Woo-Hyuk; Na, Ju-Young; Kim, Meyung-Kug; Yoo, Bong-Goo

    2013-01-01

    Hashimoto’s encephalopathy is an immune-mediated disorder characterized by acute or subacute encephalopathy related to increased anti-thyroid antibodies. Clinical manifestations of Hashimoto’s encephalopathy may include stroke-like episodes, altered consciousness, psychosis, myoclonus, abnormal movements, seizures, and cognitive dysfunction. Acute cognitive dysfunction with convulsion as initial clinical manifestations of Hashimoto’s encephalopathy is very rare. We report a 65-year-old man wh...

  9. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis

    Directory of Open Access Journals (Sweden)

    Naghi DARA

    2013-12-01

    Full Text Available Abstract How to Cite This Article: Dara N, Sayyari AA, Imanzadeh F. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis. Iran J Child Neurol. 2014 Winter; 8(1:1-11. Objective As acute liver failure (ALF and chronic liver disease (cirrhosis continue to increase in prevalence, we will see more cases of hepatic encephalopathy. Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma, change of personality, intellectual and behavioral deterioration, speech and motor dysfunction. Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression. “Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.

  10. Cartilage (Bovine and Shark) (PDQ)

    Science.gov (United States)

    ... Ask about Your Treatment Research Cartilage (Bovine and Shark) (PDQ®)–Patient Version Overview Go to Health Professional ... 8 ). Questions and Answers About Cartilage (Bovine and Shark) What is cartilage? Cartilage is a type of ...

  11. [Posttraumatic cerebrasthenia and posttraumatic encephalopathy--difficulties in opinionating].

    Science.gov (United States)

    Ptaszyńska-Sarosiek, Iwona; Niemcunowicz-Janica, Anna; Filimoniuk, Marcin; Wardaszka, Zofia; Okłota, Magdalena; Szeremeta, Michał; Sackiewicz, Adam

    2010-01-01

    Post-traumatic cerebrasthenia and post-traumatic encephalopathy are the effects of a head trauma. Most physicians overuse these diagnoses. They do not perform differential diagnostic management, what leads to issuing erroneous opinions. The aim of this report is to show the symptoms of posttraumatic cerebrasthenia and posttraumatic encephalopathy and diagnostic investigations, which could be helpful for diagnosing these conditions. Cerebrasthenia (the subjective posttraumatic syndrome, subjective syndrome, posttraumatic neurosis) is usually a functional--"unorganic" disorder. It happens in the cases of many people who had brain concussion. The most common symptoms are: headache, vertigo, irritability, insomnia, attention deficits, fatigability. Posttraumatic encephalopathy is a persistent brain tissues injury which could be caused by more severe trauma (brain contusion, endocranial bleeding). The clinical picture of this disease entity is not unambiguous. The most frequently encountered form is a simple (common) form, which is similar to cerebrasthenia, but with differences in the neurological examination (focal signs) and with persistent changes in EEG. PMID:21520540

  12. EPILEPTIC ENCEPHALOPATHY WITH CONTINUOUS SPIKES-WAVES ACTIVITY DURING SLEEP

    Directory of Open Access Journals (Sweden)

    E. D. Belousova

    2012-01-01

    Full Text Available The author represents the review and discussion of current scientific literature devoted to epileptic encephalopathy with continuous spikes-waves activity during sleep — the special form of partly reversible age-dependent epileptic encephalopathy, characterized by triad of symptoms: continuous prolonged epileptiform (spike-wave activity on EEG in sleep, epileptic seizures and cognitive disorders. The author describes the aspects of classification, pathogenesis and etiology, prevalence, clinical picture and diagnostics of this disorder, including the peculiar anomalies on EEG. The especial attention is given to approaches to the treatment of epileptic encephalopathy with continuous spikeswaves activity during sleep. Efficacy of valproates, corticosteroid hormones and antiepileptic drugs of other groups is considered. The author represents own experience of treatment this disorder with corticosteroids, scheme of therapy and assessment of efficacy.

  13. Wernicke Encephalopathy Presenting in a Patient with Severe Acute Pancreatitis

    Directory of Open Access Journals (Sweden)

    Ana Cecilia Arana-Guajardo

    2012-01-01

    Full Text Available Context Acute pancreatitis can lead to prolonged fasting and malnutrition. Many metabolic changes, including thiamine deficiency, may lead to the well know pancreatic encephalopathy. In this condition however the thiamine deficiency is rarely suspected. Case report We report the case of a 17-year-old woman with severe acute pancreatitis who developed mental status changes and ophthalmoplegia. A magnetic resonance image showed hyperintensive signals in periventricular areas, medial thalamus, and mammillary bodies, findings consistent with the diagnosis of Wernicke encephalopathy. Thiamine treatment reversed neurological complications. Conclusion Wernicke encephalopathy secondary to thiamine deficiency should be considered as a possible cause of acute mental status changes in patients with acute pancreatitis and malnutrition. Prophylactic doses of thiamine could be considered in susceptible patients.

  14. Wernicke’s Encephalopathy Complicating Hyperemesis during Pregnancy

    Directory of Open Access Journals (Sweden)

    Mohamed Adnane Berdai

    2016-01-01

    Full Text Available Wernicke’s encephalopathy is caused by severe thiamine deficiency; it is mostly observed in alcoholic patients. We report the case of a 28-year-old woman, at 17 weeks of gestational age, with severe hyperemesis gravidarum. She presented with disturbance of consciousness, nystagmus, ophthalmoplegia, and ataxia. The resonance magnetic imagery showed bilaterally symmetrical hyperintensities of thalamus and periaqueductal area. The case was managed with very large doses of thiamine. The diagnosis of Wernicke’s encephalopathy was confirmed later by a low thiamine serum level. The patient was discharged home on day 46 with mild ataxia and persistent nystagmus. Wernicke’s encephalopathy is a rare complication of hyperemesis gravidarum. It should be diagnosed as early as possible to prevent long-term neurological sequela or death. Thiamine supplementation in pregnant women with prolonged vomiting should be initiated, especially before parenteral dextrose infusion. Early thiamine replacement will reduce maternal morbidity and fetal loss rate.

  15. Magnetic resonance imaging in a case of Wernicke's encephalopathy

    International Nuclear Information System (INIS)

    Wernicke's encephalopathy is an uncommon disorder caused by a thiamine deficiency which is clinically characterized by the triad of ophthalmoplegia, ataxia and disturbances of consciousness, each finding being variably present. The disease is caused by malnutrition or malabsorption, and is often associated with prolonged alcohol intake, neoplasm and extensive inflammatory processes of the digestive tract and parenteral hyperalimentation-induced gastrointestinal mucosal atrophy. Clinical diagnosis can be elusive and MRI may be the only imaging technique able to detect the cerebral lesions, whose type and distribution are characteristic of the Wernicke's encephalopathy, whereas CT is positive only in exceptional cases. We report a case of a 56-year-old woman who developed a Wernicke's encephalopathy 1 month after a colonic resection with signal intensity changes located in the mammillary bodies and in the medial thalamic nuclei. (orig.)

  16. An overview of animal prion diseases

    Directory of Open Access Journals (Sweden)

    Imran Muhammad

    2011-11-01

    Full Text Available Abstract Prion diseases are transmissible neurodegenerative conditions affecting human and a wide range of animal species. The pathogenesis of prion diseases is associated with the accumulation of aggregates of misfolded conformers of host-encoded cellular prion protein (PrPC. Animal prion diseases include scrapie of sheep and goats, bovine spongiform encephalopathy (BSE or mad cow disease, transmissible mink encephalopathy, feline spongiform encephalopathy, exotic ungulate spongiform encephalopathy, chronic wasting disease of cervids and spongiform encephalopathy of primates. Although some cases of sporadic atypical scrapie and BSE have also been reported, animal prion diseases have basically occurred via the acquisition of infection from contaminated feed or via the exposure to contaminated environment. Scrapie and chronic wasting disease are naturally sustaining epidemics. The transmission of BSE to human has caused more than 200 cases of variant Cruetzfeldt-Jacob disease and has raised serious public health concerns. The present review discusses the epidemiology, clinical neuropathology, transmissibility and genetics of animal prion diseases.

  17. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis

    Directory of Open Access Journals (Sweden)

    Naghi DARA*

    2014-01-01

    Full Text Available How to Cite This Article: Dara N, Sayyari AA, Imanzadeh F. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis. Iran J Child Neurol. 2014 Winter; 8(1:1-11.ObjectiveAs acute liver failure (ALF and chronic liver disease (cirrhosis continue to increase in prevalence, we will see more cases of hepatic encephalopathy.Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma, change of personality, intellectualand behavioral deterioration, speech and motor dysfunction.Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression.“Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.References:Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei AT. Hepatic encephalopathy definition, nomenclature, diagnosis, and quantification: final report of the working party at the 11th World Congresses of Gastroenterology, Vienna, 1998.Hepatology 2002;35:716-21.BleiAT,Cordoba J. Hepatic encephalopathy. AmJ Gastroenterol 2001;96:1968–76.Vaquero J,Chung C, Cahill ME, BleiAT. Pathogenesis of hepatic encephalopathy in acute liver failure. Semin Liver Dis 2003;23:259-69.Bajaj JS, Wade JB, Sanyal AJ. Spectrum of neurocognitive impairment in cirrhosis: Implications for the assessment of hepatic encephalopathy

  18. Gut microbiota: its role in hepatic encephalopathy.

    Science.gov (United States)

    Rai, Rahul; Saraswat, Vivek A; Dhiman, Radha K

    2015-03-01

    Ammonia, a key factor in the pathogenesis of hepatic encephalopathy (HE), is predominantly derived from urea breakdown by urease producing large intestinal bacteria and from small intestine and kidneys, where the enzyme glutaminases releases ammonia from circulating glutamine. Non-culture techniques like pyrosequencing of bacterial 16S ribosomal ribonucleic acid are used to characterize fecal microbiota. Fecal microbiota in patients with cirrhosis have been shown to alter with increasing Child-Turcotte-Pugh (CTP) and Model for End stage Liver Disease (MELD) scores, and with development of covert or overt HE. Cirrhosis dysbiosis ratio (CDR), the ratio of autochthonous/good bacteria (e.g. Lachnospiraceae, Ruminococcaceae and Clostridiales) to non-autochthonous/pathogenic bacteria (e.g. Enterobacteriaceae and Streptococcaceae), is significantly higher in controls and patients with compensated cirrhosis than patients with decompensated cirrhosis. Although their stool microbiota do not differ, sigmoid colonic mucosal microbiota in liver cirrhosis patients with and without HE, are different. Linkage of pathogenic colonic mucosal bacteria with poor cognition and inflammation suggests that important processes at the mucosal interface, such as bacterial translocation and immune dysfunction, are involved in the pathogenesis of HE. Fecal microbiome composition does not change significantly when HE is treated with lactulose or when HE recurs after lactulose withdrawal. Despite improving cognition and endotoxemia as well as shifting positive correlation of pathogenic bacteria with metabolites, linked to ammonia, aromatic amino acids and oxidative stress, to a negative correlation, rifaximin changes gut microbiome composition only modestly. These observations suggest that the beneficial effects of lactulose and rifaximin could be associated with a change in microbial metabolic function as well as an improvement in dysbiosis. PMID:26041954

  19. Wernicke encephalopathy: MR findings and clinical presentation

    Energy Technology Data Exchange (ETDEWEB)

    Weidauer, Stefan; Lanfermann, Heinrich; Zanella, Friedhelm E. [Institute of Neuroradiology, University of Frankfurt, Frankfurt (Germany); Nichtweiss, Michael [Department of Neurology, Municipal Hospital of Wismar, Wismar (Germany)

    2003-05-01

    Wernicke encephalopathy (WE) is a severe neurological disorder caused by vitamin B1 deficiency. The aim of the study was to analyse MRI findings typical for this disease and to evaluate the significance of their correlations with clinical symptoms. Magnetic resonance images and clinical features of 12 patients with WE were analysed. The patients underwent MR imaging within 3-14 days after onset of clinical symptoms. In 7 of 12 patients MR imaging showed symmetrical diencephalic and midbrain lesions. Postcontrast T1-weighted images from 5 of 9 patients examined during the initial 6 days of acute WE showed a subtle enhancement of the mamillary bodies, the tectal plate, the periaqueductal area and the periventricular region of the third ventricle including the paramedian thalamic nuclei. In addition, T2-weighted and fluid-attenuated inversion recovery (FLAIR) images revealed hyperintense signals in these regions (except for 2 patients where the mamillary bodies were normal). Hyperintense lesions on T2-weighted images without any enhancement on postcontrast T1-weighted images were detected in 2 patients by MR imaging performed 11 or 14 days after onset of WE. Patients with hyperintensities on T2-weighted images of the periventricular region of the third ventricle and the paramedian thalamic nuclei had poor recovery from their mental dysfunction. The MR examination in case of WE shows a typical pattern of lesions in 58% of cases. Enhancement of the mamillary bodies, the periventricular region of the third ventricle including the paramedian thalamic nuclei, and the periaqueductal area on postcontrast T1-weighted images can be observed in the initial period after clinical onset of symptoms and are characteristic signs of the acute stage of WE. Hyperintense lesions in the periventricular region and the paramedian thalamic nuclei on T2-weighted and FLAIR images in the subacute stage of WE and enhancement on postcontrast T1-weighted images of the mamillary bodies and the

  20. Toxic encephalopathy due to colchicine--Gloriosa superba poisoning.

    Science.gov (United States)

    Gooneratne, Inuka Kishara; Weeratunga, Praveen; Caldera, Manjula; Gamage, Ranjanie

    2014-10-01

    Gloriosa superba, a flowering plant widespread in South and Southeast Asia, is implicated in many cases of self-poisoning. Colchicine is concentrated in the seeds and tubers and this mediates its toxicity. We describe a 28-year-old woman who developed delayed encephalopathy after eating G superba tubers. MR scan of brain showed bilateral symmetrical T2 basal ganglia hyperintensities in the caudate and lentiform nuclei. The delay in onset of encephalopathy is attributable to a direct-effect colchicine, probably mediated through its effect on microtubular transport. PMID:24591648

  1. Hyperammonemic Encephalopathy due to Valproic Acid and Topiramate Interaction

    Directory of Open Access Journals (Sweden)

    Jennifer D. Twilla

    2014-01-01

    Full Text Available Valproic acid-induced hyperammonemic encephalopathy is a rare yet serious adverse drug reaction. Medication interactions such a valproic acid and topiramate can precipitate an event. We present the case of a 52-year-old female that presented with acute mental status change and hypersomnolence due to hyperammonemia caused by a valproic acid derivative. The patient improved after withdrawal of the offending medications and treatment with lactulose. Clinicians should remain hypervigilant in monitoring for valproic acid-induced hyperammonemic encephalopathy and risk factors such as polypharmacy.

  2. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis

    OpenAIRE

    Dara, Naghi; Ali-Akbar SAYYARI; Imanzadeh, Farid

    2014-01-01

    How to Cite This Article: Dara N, Sayyari AA, Imanzadeh F. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis. Iran J Child Neurol. 2014 Winter; 8(1):1-11.ObjectiveAs acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy.Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complic...

  3. Isolated Brainstem Involvement in Posterior Reversible Encephalopathy Syndrome

    Directory of Open Access Journals (Sweden)

    Tarkan Ergün

    2013-09-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a clinical and radiologic entity characterized by headache, variable mental status, epilepsy, visual disturbances, and typical transient changes in the posterior cerebral perfusion. Parieto-occipital region the most commonly involved site. Less commonly, brainstem, basal ganglia, and cerebellum are involved besides the supratentorial white matter areas. However, isolated brainstem involvement is very rare. We here present a case of isolated brainstem involvement in posterior reversible encephalopathy syndrome which was diagnosed by diffusion-weighted MR imaging.

  4. Pancreatic encephalopathy- a rare complication of severe acute biliary pancreatitis

    Directory of Open Access Journals (Sweden)

    Vlad Denis Constantin

    2014-10-01

    Full Text Available Background. Pancreatic encephalopathy is a rare complication of severe acute pancreatitis, with high mortality, being difficult to diagnose and treat, thus requiring continuous research regarding its management. Materials and Methods. Of 20 patients diagnosed with severe acute pancreatitis on admission at Department of Emergency and Admission (DEA, from January 1st 2010 to March 31st 2014, 5 cases complicated by pancreatic encephalopathy were analyzed using a descriptive observational, retrospective, single-center study. Results. The study shows different types of diagnostic algorithm and therapeutical approaches, in correlation with morbidity and mortality rates. Conclusions. Our study highlighted the fact that speed is critical, early management being the key to outcome.

  5. Acute hyperammonemic encephalopathy with features on diffusion-weighted images: Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ja Young; Yu, In Kyu [Dept. of Radiology, Eulji University Hospital, Daejeon (Korea, Republic of)

    2015-02-15

    Acute hyperammonemic encephalopathy is a rare toxic encephalopathy caused by accumulated plasma ammonia. A few literatures are reported about MRI findings of acute hyperammonemic encephalopathy. It is different from the well-known chronic hepatic encephalopathy. The clinical symptom and MRI findings of acute hyperammonemic encephalopathy can be reversible with proper treatment. Acute hepatic encephalopathy involves the cingulate cortex, diffuse cerebral cortices, insula, bilateral thalami on diffusion-weighted imaging (DWI), and fluid-attenuated inversion-recovery. Acute hepatic encephalopathy might mimic hypoxic-ischemic encephalopathy because of their similar predominant involving sites. We experienced 2 cases of acute hyperammonemic encephalopathy consecutively. They showed restricted diffusion at the cingulate cortex, cerebral cortices, insula, and bilateral dorsomedial thalami on DWI. One patient underwent acute fulminant hepatitis A, the other patient with underlying chronic liver disease had acute liver failure due to hepatotoxicity of tuberculosis medication. In this report, we presented the characteristic features of DWI in acute hyperammonemic encephalopathy. In addition, we reviewed articles on MRI findings of acute hyperammonemic encephalopathy.

  6. Acute hyperammonemic encephalopathy with features on diffusion-weighted images: Report of two cases

    International Nuclear Information System (INIS)

    Acute hyperammonemic encephalopathy is a rare toxic encephalopathy caused by accumulated plasma ammonia. A few literatures are reported about MRI findings of acute hyperammonemic encephalopathy. It is different from the well-known chronic hepatic encephalopathy. The clinical symptom and MRI findings of acute hyperammonemic encephalopathy can be reversible with proper treatment. Acute hepatic encephalopathy involves the cingulate cortex, diffuse cerebral cortices, insula, bilateral thalami on diffusion-weighted imaging (DWI), and fluid-attenuated inversion-recovery. Acute hepatic encephalopathy might mimic hypoxic-ischemic encephalopathy because of their similar predominant involving sites. We experienced 2 cases of acute hyperammonemic encephalopathy consecutively. They showed restricted diffusion at the cingulate cortex, cerebral cortices, insula, and bilateral dorsomedial thalami on DWI. One patient underwent acute fulminant hepatitis A, the other patient with underlying chronic liver disease had acute liver failure due to hepatotoxicity of tuberculosis medication. In this report, we presented the characteristic features of DWI in acute hyperammonemic encephalopathy. In addition, we reviewed articles on MRI findings of acute hyperammonemic encephalopathy.

  7. Bovine milk exosome proteome

    Science.gov (United States)

    Exosomes are 40-100 nm membrane vesicles of endocytic origin and are found in blood, urine, amniotic fluid, bronchoalveolar lavage (BAL) fluid, as well as human and bovine milk. Exosomes are extracellular organelles important in intracellular communication/signaling, immune function, and biomarkers ...

  8. BOVINE VIRAL DIARRHEA VIRUSES

    Science.gov (United States)

    Bovine viral diarrhea virus (BVDV) is an umbrella term for two species of viruses, BVDV1 and BVDV2, within the Pestivirus genus of the Flavivirus family. BVDV viruses are further subclassified as cytopathic and noncytopathic based on their activity in cultured epithelial cells. Noncytopathic BVDV p...

  9. Genotyping bovine coronaviruses.

    Science.gov (United States)

    Bovine coronaviruses (BoCV) are enveloped, single-stranded, positive-sense RNA viruses of the Coronaviridae family. Infection is associated with enteritis and pneumonia in calves and Winter Dysentery in adult cattle. Strains, isolated more than 50 years ago, are used in vaccines and as laboratory ...

  10. Toxic encephalopathy from chlorobenzilate poisoning: report of a case.

    Science.gov (United States)

    Ravindran, M

    1978-10-01

    Chlorobenzilate is an organochlorine insecticide with toxicity like those of Dichlorodiphenyltrichloroethane (DDT). A patient who developed toxic encephalopathy after exposure to chlorobenzilate mist, with associated clinical and EEG abnormalities, is briefly reported. Some unusual features of his clinical picture are pointed out. PMID:737852

  11. Hepatic Encephalopathy Associated With Cancer or Anticancer Therapy

    OpenAIRE

    Willson, Kaspar J.; Nott, Louise M.; Broadbridge, Vy T.; Price, Timothy

    2013-01-01

    Hepatic encephalopathy is an uncommon cause of neurologic deterioration associated with hyperammonemia, which results from hepatic dysfunction or altered ammonia metabolism. Often overlooked, hyperammonemia may occur via any of several pathophysiological processes, and in the setting of malignancy, it is a potentially reversible cause of confusion and coma. Hepatic dysfunction as a result of malignant infiltration, chemotherapeutic toxicities, targeted anticancer therapies, reactivation hepat...

  12. Safety, efficacy, and patient acceptability of rifaximin for hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Kimer N

    2014-03-01

    Full Text Available Nina Kimer,1 Aleksander Krag,2 Lise L Gluud1 1Gastrounit, Medical Division, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark; 2Department of Gastroenterology, Odense University Hospital, University of Southern Denmark, Odense, Denmark Abstract: Hepatic encephalopathy is a complex disease entity ranging from mild cognitive dysfunction to deep coma. Traditionally, treatment has focused on a reduction of ammonia through a reduced production, absorption, or clearance. Rifaximin is a nonabsorbable antibiotic, which reduces the production of ammonia by gut bacteria and, to some extent, other toxic derivatives from the gut. Clinical trials show that these effects improve episodes of hepatic encephalopathy. A large randomized trial found that rifaximin prevents recurrent episodes of hepatic encephalopathy. Most patients were treated concurrently with lactulose. Trials have varied greatly in design, outcomes, and duration of treatment regimes. Although a number of retrospective studies have indicated that long-term treatment with rifaximin is safe and possibly beneficial, high quality trials are needed to further clarify efficacy and safety of long-term treatment with rifaximin and evaluate effects of combination therapy with lactulose and branched-chain amino acids for patients with liver cirrhosis and hepatic encephalopathy. Keywords: prevention, lactulose, liver cirrhosis

  13. About pathognomonic images: an infrequent case of acute encephalopathy

    Directory of Open Access Journals (Sweden)

    Alessandro Grasso

    2013-05-01

    Full Text Available BACKGROUND The occurrence of acute encephalopathy is a dramatic clinical dilemma when usual diagnostic techniques (blood tests, cerebral CT and cerebrospinal fluid analysis show no abnormalities. CLINICAL CASE We describe a case of a 73 years old man admitted in our Internal Medicine Unit for acute diarrhoea with vomiting and fever who developed a prolonged gastrointestinal dysmotility syndrome with poor nutritional intake. Although a parenteral support was provided, he developed acute encephalopathy followed by hypotension and lactic acidosis without evidence of renal and hepatic disease or glycemic alterations. Likewise, no cerebral CT and cerebrospinal fluid alterations were found. Conversely, cerebral MRI showed marked and diffuse DP-2 and FLAIR hyperintensity of the mesencephalic tectal plate, of the periaqueductal area, and of the periventricular region of the third ventricle including the median thalamic area. These MRI descriptions were considered pathognomonic of Wernicke encephalopathy. Thus, the immediate use of ev thiamine was followed by a prompt and complete recovery of neurological, hemodinamic and metabolic conditions. CONCLUSIONS Non-alcoholic Wernicke encephalopathy is a rare and dramatic clinical event with high mortality. In this context, brain MRI is the best diagnostic tool providing a typical picture.

  14. Safety, efficacy, and patient acceptability of rifaximin for hepatic encephalopathy

    DEFF Research Database (Denmark)

    Kimer, Nina; Krag, Aleksander; Gluud, Lise L

    2014-01-01

    Hepatic encephalopathy is a complex disease entity ranging from mild cognitive dysfunction to deep coma. Traditionally, treatment has focused on a reduction of ammonia through a reduced production, absorption, or clearance. Rifaximin is a nonabsorbable antibiotic, which reduces the production of ...

  15. Branched-chain amino acids for people with hepatic encephalopathy

    DEFF Research Database (Denmark)

    Gluud, Lise Lotte; Dam, Gitte; Les, Iñigo;

    2015-01-01

    BACKGROUND: Hepatic encephalopathy is a brain dysfunction with neurological and psychiatric changes associated with liver insufficiency or portal-systemic shunting. The severity ranges from minor symptoms to coma. A Cochrane systematic review including 11 randomised clinical trials on branched-ch...

  16. Neuropsychiatric manifestation of Hashimoto's encephalopathy in an adolescent and treatment

    Directory of Open Access Journals (Sweden)

    Ramdas Sarjerao Ransing

    2016-01-01

    Full Text Available Hashimoto's encephalopathy is usually underdiagnosed and untreated because of complex neuropsychiatric manifestation. We report a case of an adolescent female with Hashimoto's encephalopathy who responded well to a combination of aspirin and levothyroxine. A 16-year-old girl presented at psychiatric emergency services with a depressive episode, menstrual irregularities, and a 5-month past history of thyroid swelling. On clinical examination, she was in a euthyroid state with insignificant neurological history. However, her previous investigation revealed a hypothyroid state. Her magnetic resonance imaging findings demonstrated infarcts in the bilateral gangliocapsular region and left frontal periventricular deep white matter lesion. Ultrasonography of the thyroid and fine needle aspiration cytology confirmed lymphocytic thyroiditis. Anti-thyroid peroxidase (289 IU/ml antibody titer was elevated (289 IU/mL. Her depressive symptoms responded well to antidepressants, mood stabilizers, nonsteroidal anti-inflammatory drugs, and levothyroxine. She remained in the euthyroid state and then in the euthymic state for 3 years. Hashimoto's encephalopathy is steroid-responsive encephalopathy. Most researchers have observed a dramatic response to steroids with or without levothyroxine. A clinician may consider aspirin as an alternative to a steroid in long-term management to avoid steroid-related side effects and contraindications.

  17. Benzodiazepine receptor antagonists for acute and chronic hepatic encephalopathy

    DEFF Research Database (Denmark)

    Als-Nielsen, B; Kjaergard, L L; Gluud, C

    2001-01-01

    The pathogenesis of hepatic encephalopathy is unknown. It has been suggested that liver failure leads to the accumulation of substances that bind to a receptor-complex in the brain resulting in neural inhibition which may progress to coma. Several trials have assessed benzodiazepine receptor...

  18. GRIN1 Mutations in Early-Onset Epileptic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Wenjuan Chen

    2015-06-01

    Full Text Available Investigators from Yokohama City University and other medical centers in Israel and Japan reported mutations on N-methyl-D-aspartate (NMDA receptors subunit GRIN1 (GluN1 identified in patients with nonsyndromic intellectual disability and early-onset epileptic encephalopathy.

  19. Coagulopathy and encephalopathy in a dog with acute hepatic necrosis.

    Science.gov (United States)

    Strombeck, D R; Krum, S; Rogers, Q

    1976-10-15

    Disseminated intravascular coagulation developed secondary to hepatic necrosis in a 5-year-old Saint Bernard. Although the coagulopathy responded to treatment with heparin, the dog died from the combined effects of gastric hemorrhage and encephalopathy, both of which are complications of hepatic necrosis. PMID:977448

  20. Acute necrotizing encephalopathy of childhood: report of a Spanish case.

    Science.gov (United States)

    San Millan, Beatriz; Teijeira, Susana; Penin, Carmen; Garcia, Jose L; Navarro, Carmen

    2007-12-01

    Acute necrotizing encephalopathy of childhood is a rare disease with a broad clinical, radiologic, and biochemical spectrum. In the few postmortem studies published to date, the neuropathologic findings involved symmetric, necrotic brain lesions as the hallmark. Here we report on the clinical and neuropathologic findings of a Spanish child with the most severe form of the disease. PMID:18021928

  1. Subclinical hepatic encephalopathy: diagnosis, clinical implications, and intervention

    NARCIS (Netherlands)

    J.C. Guero Guillen

    1997-01-01

    textabstractHepatic encephalopathy (HE) is traditionally graded into four clinical stages of severity, ranging from lethargy, sleep and memory disturbances (grade 1) to coma (grade 4). In addition to the clinical grading of HE, a subclinical stage has been described. In subclinical hepatic encephalo

  2. Pathophysiological aspects of acute hepatic encephalopathy in the rat

    International Nuclear Information System (INIS)

    The aim of the present thesis is to elucidate the pathogenesis of acute hepatic encephalopathy (HE). In order to study acute HE, plasma and brain concentrations were measured of ammonia, aminoacids, lactate and polyamines as well as brain energy rich phosphates. In addition new techniques of brain research were developed and applied. 277 refs.; 29 figs.; 18 tabs

  3. MRI reveals reversible lesions resembling posterior reversible encephalopathy in porphyria

    International Nuclear Information System (INIS)

    We report a 20-year-old woman who had an attack of acute intermittent porphyria with seizures, hallucinations, autonomic and somatic neuropathy. T2-weighted MRI revealed multiple lesions which were no longer visible 3 months later. We suggest a similar mechanism to posterior reversible encephalopathy underlying cerebral symptoms in porphyria. (orig.)

  4. MRI reveals reversible lesions resembling posterior reversible encephalopathy in porphyria

    Energy Technology Data Exchange (ETDEWEB)

    Celik, M. [Huesrev Gerede c, 128/4 Tesvikiye, 80690 Istanbul (Turkey); Department of Neurology, Sisli Etfal Education and Research Hospital, Sisli Etfal S., Sisli, Istanbul (Turkey); Forta, H.; Babacan, G. [Department of Neurology, Sisli Etfal Education and Research Hospital, Sisli Etfal S., Sisli, Istanbul (Turkey); Dalkilic, Tuerker [Department of Neurosurgery, Sisli Etfal Education and Research Hospital, Sisli Etfal S., Sisli, Istanbul (Turkey)

    2002-10-01

    We report a 20-year-old woman who had an attack of acute intermittent porphyria with seizures, hallucinations, autonomic and somatic neuropathy. T2-weighted MRI revealed multiple lesions which were no longer visible 3 months later. We suggest a similar mechanism to posterior reversible encephalopathy underlying cerebral symptoms in porphyria. (orig.)

  5. Hyperammonemic encephalopathy induced by a combination of valproate and pivmecillinam.

    Science.gov (United States)

    Lokrantz, C-M; Eriksson, B; Rosén, I; Asztely, F

    2004-04-01

    We describe the clinical and neurophysiological findings in a case of hyperammonemic encephalopathy. A 72-year-old woman taking valproate (VPA), as monotherapy for her partial epilepsy developed urinary tract infection. She was treated with pivmecillinam 600 mg daily. The following days she deteriorated and became stuporous. At admission her serum ammonia level was increased (113 mmol/l) but the liver function appeared normal. EEG showed bilateral triphasic waves and continuous high-amplitude delta-theta wave. The patient recovered rapidly after discontinuation of VPA and i.v. treatment with cefuroxime for her urinary tract infection. VPA-induced hyperammonemic encephalopathy in adults is a rare phenomenon, especially when VPA is used as monotherapy. It has been suggested that the VPA-induced hyperammonemic encephalopathy is due to reduced serum carnitine concentration. Pivmecillinam, a widely used antibiotic for treatment of urinary tract infections, is also known to decrease the serum carnitine concentration. Our case shows that caution is required when treatment with VPA is combined with pivmecillinam due to the risk of developing hyperammonemic encephalopathy. PMID:15016014

  6. Hypoxic-Ischemic-Encephalopathy Studied by EEG and Mr Spectroscopy

    OpenAIRE

    J Gordon Millichap

    2011-01-01

    Amplitude-integrated EEG (a-EEG) time course during the first 24 hrs of life was related to brain metabolism changes detected by proton MR spectroscopy (H-MRS) at 7-10 days of post-natal life in non-cooled term newborns with hypoxic-ischemic-encephalopathy (HIE).

  7. Wernicke's encephalopathy in a child: case report and MR findings

    International Nuclear Information System (INIS)

    We report a child affected by Wernicke's encephalopathy (WE), which was unsuspected clinically. MRI suggested the correct diagnosis and prompted appropriate thiamine replacement. WE is a difficult condition to recognise, especially in children, and MRI may be useful in the diagnosis of the disease. (orig.)

  8. Metformin inhibits glutaminase activity and protects against hepatic encephalopathy.

    Directory of Open Access Journals (Sweden)

    Javier Ampuero

    Full Text Available AIM: To investigate the influence of metformin use on liver dysfunction and hepatic encephalopathy in a retrospective cohort of diabetic cirrhotic patients. To analyze the impact of metformin on glutaminase activity and ammonia production in vitro. METHODS: Eighty-two cirrhotic patients with type 2 diabetes were included. Forty-one patients were classified as insulin sensitizers experienced (metformin and 41 as controls (cirrhotic patients with type 2 diabetes mellitus without metformin treatment. Baseline analysis included: insulin, glucose, glucagon, leptin, adiponectin, TNFr2, AST, ALT. HOMA-IR was calculated. Baseline HE risk was calculated according to minimal hepatic encephalopathy, oral glutamine challenge and mutations in glutaminase gene. We performed an experimental study in vitro including an enzymatic activity assay where glutaminase inhibition was measured according to different metformin concentrations. In Caco2 cells, glutaminase activity inhibition was evaluated by ammonia production at 24, 48 and 72 hours after metformina treatment. RESULTS: Hepatic encephalopathy was diagnosed during follow-up in 23.2% (19/82: 4.9% (2/41 in patients receiving metformin and 41.5% (17/41 in patients without metformin treatment (logRank 9.81; p=0.002. In multivariate analysis, metformin use [H.R.11.4 (95% CI: 1.2-108.8; p=0.034], age at diagnosis [H.R.1.12 (95% CI: 1.04-1.2; p=0.002], female sex [H.R.10.4 (95% CI: 1.5-71.6; p=0.017] and HE risk [H.R.21.3 (95% CI: 2.8-163.4; p=0.003] were found independently associated with hepatic encephalopathy. In the enzymatic assay, glutaminase activity inhibition reached 68% with metformin 100 mM. In Caco2 cells, metformin (20 mM decreased glutaminase activity up to 24% at 72 hours post-treatment (p<0.05. CONCLUSIONS: Metformin was found independently related to overt hepatic encephalopathy in patients with type 2 diabetes mellitus and high risk of hepatic encephalopathy. Metformin inhibits glutaminase

  9. SCN8A encephalopathy: Research progress and prospects.

    Science.gov (United States)

    Meisler, Miriam H; Helman, Guy; Hammer, Michael F; Fureman, Brandy E; Gaillard, William D; Goldin, Alan L; Hirose, Shinichi; Ishii, Atsushi; Kroner, Barbara L; Lossin, Christoph; Mefford, Heather C; Parent, Jack M; Patel, Manoj; Schreiber, John; Stewart, Randall; Whittemore, Vicky; Wilcox, Karen; Wagnon, Jacy L; Pearl, Phillip L; Vanderver, Adeline; Scheffer, Ingrid E

    2016-07-01

    On April 21, 2015, the first SCN8A Encephalopathy Research Group convened in Washington, DC, to assess current research into clinical and pathogenic features of the disorder and prepare an agenda for future research collaborations. The group comprised clinical and basic scientists and representatives of patient advocacy groups. SCN8A encephalopathy is a rare disorder caused by de novo missense mutations of the sodium channel gene SCN8A, which encodes the neuronal sodium channel Nav 1.6. Since the initial description in 2012, approximately 140 affected individuals have been reported in publications or by SCN8A family groups. As a result, an understanding of the severe impact of SCN8A mutations is beginning to emerge. Defining a genetic epilepsy syndrome goes beyond identification of molecular etiology. Topics discussed at this meeting included (1) comparison between mutations of SCN8A and the SCN1A mutations in Dravet syndrome, (2) biophysical properties of the Nav 1.6 channel, (3) electrophysiologic effects of patient mutations on channel properties, (4) cell and animal models of SCN8A encephalopathy, (5) drug screening strategies, (6) the phenotypic spectrum of SCN8A encephalopathy, and (7) efforts to develop a bioregistry. A panel discussion of gaps in bioregistry, biobanking, and clinical outcomes data was followed by a planning session for improved integration of clinical and basic science research. Although SCN8A encephalopathy was identified only recently, there has been rapid progress in functional analysis and phenotypic classification. The focus is now shifting from identification of the underlying molecular cause to the development of strategies for drug screening and prioritized patient care. PMID:27270488

  10. Le complexe respiratoire bovin

    OpenAIRE

    Lekeux, Pierre

    1996-01-01

    Les maladies respiratoires des bovins sont, partout dans le monde, la cause principale de mortalité chez les jeunes bovins. Plusieurs facteurs favorisent l'apparition de ce syndrome : des facteurs propres à l'animal, comme l'âge, l'état général et le statut immunitaire; d'autres relatifs à l'environnement, comme les stress engendrés par les changements de régime alimentaire, de température et d'humidité; d'autres encore, liés à la présence d'agents infectieux, comme des bactéries, des virus e...

  11. Genetics of bovine vaccination

    OpenAIRE

    Leach, Richard Jonathan

    2011-01-01

    Infectious disease is an important issue for animal breeders, farmers and governments. Solutions to control infectious disease are needed and research focused on the genetic loci determining variation in immune-related traits has the potential to deliver solutions. The primary aim of this thesis is to discover regions of the bovine genome which influence the immune response post immunisation. To accomplish this two types of immunising agents, a Foot-and-Mouth Disease Virus (FMD...

  12. Vitrification of Bovine Oocytes

    OpenAIRE

    Anchamparuthy, Vahida Muhammed Ismail

    2007-01-01

    Cryopreservation of oocytes is a challenge. Studies were conducted to vitrify mouse zygotes and cumulus-intact bovine oocytes from follicles of different diameters, small (â ¤ 4 mm) and medium (4 to 10 mm), using nylon mesh. The specific goals were to assess changes in apoptotic gene expression (Fas-FasL, Bax, Bcl-2, and survivin) in conjunction with terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) and caspase assays. Mouse zygotes were exposed to increasing concentrations...

  13. Pathogenèse des encéphalopathies spongiformes transmissibles : apports du modèle ovin

    OpenAIRE

    Schelcher, Francois; Andreoletti, Olivier; Tabouret, Guillaume; Lacroux, Caroline; Foucras, Gilles; Eychenne, Francis; Berthon, Patricia; Sarradin, Pierre; Lantier, Frédéric; Elsen, Jean Michel

    2004-01-01

    Parmi les Encéphalopathies Spongiformes Transmissibles (EST), la tremblante du mouton est un modèle d’infection naturelle, caractérisé par la diversité des souches d’agent de transmission, par un rôle majeur des facteurs génétiques de réceptivité/sensibilité et par une transmission interindividuelle fréquente. La voie de contamination naturelle semble être orale. Chez des ovins génétiquement sensibles (PrPVRQ/VRQ) (premiers symptômes observés vers 20 à 24 mois), la PrPsc a été détectée dans l...

  14. Subclinical hepatic encephalopathy: the diagnostic value of evoked potentials.

    Science.gov (United States)

    Kullmann, F; Hollerbach, S; Holstege, A; Schölmerich, J

    1995-01-01

    Brainstem auditory (BAEPs) and somatosensory evoked potentials (SEPs) have been shown to be useful in detecting brainstem or cortical dysfunction in neurological diseases and in combination with other methods to diagnose brain death (37,38). These neurophysiological methods are simple and easy to perform. BAEPs and SEPs can even be easily recorded in intensive care units and guarantee a standardized examination. Moreover, these methods require no extensive patient cooperation and are not heavily influenced by learning effects. The role of BAEPs in the evaluation and diagnosis of hepatic encephalopathy is not clear. BAEPs are obviously strongly influenced by the etiology of liver disease and are normal in viral hepatitis, but prolonged in alcoholic liver disease, Wilson's disease or in hepatic coma (8,12). Unfortunately, BAEPs were not compared to psychometric tests. There was no clear-cut differentiation between various hepatic encephalopathy-gradings. At present, the use of BAEPs in the detection of subclinical hepatic encephalopathy cannot be recommended, whereas in comatose patients BAEPs can be useful as a prognostic marker and for follow-up examinations (12). Recently, Pozessere et al. (12) examined 13 comatose patients with advanced coma stages (Glasgow coma scale 5-10) and recorded unspecific changes in their EEG tracings. In all cases of hepatic coma and in one intoxicated patient they found prolongation of interpeak latencies. In addition, in this small study the interpeak latencies correlated well with the clinical outcome of the patients. Only two studies were performed using SEPs to detect neurophysiological alterations in hepatic encephalopathy (32,33). The design as well as the results of these studies are quite different. Despite the small number of patients (n = 10), the prolongation of late components in 50% of patients with hepatic encephalopathy stage 0 could be a promising result (32). The value of SEPs in detecting subclinical hepatic

  15. [Role of rifaximin in the treatment of hepatic encephalopathy].

    Science.gov (United States)

    Sanchez-Delgado, Jordi; Miquel, Mireia

    2016-04-01

    Hepatic encephalopathy (HE) is a frequent and serious complication of liver cirrhosis. In addition to correction of the precipitating factors, the most commonly used treatments are non-absorbable disaccharides and rifaximin. Many of the recommendations are based on current clinical practice and there are few randomized controlled trials. Currently, rifaximin should be initiated during an episode of EH if, after 24-48 hours of non-absorbable disaccharide therapy, there is no clinical improvement. In recurrent EH, it is advisable to add rifaximin in patients under non-absorbable disaccharide therapy who develop a new episode. Currently, standard treatment with rifaximin for minimal EH is not recommended. Rifaximin is effective in the acute treatment of overt encephalopathy and in preventing recurrence. PMID:26545947

  16. Concentric structure of thalamic lesions in acute necrotizing encephalopathy

    International Nuclear Information System (INIS)

    Acute necrotizing encephalopathy of childhood (ANE) is characterized by multiple, symmetrical brain lesions affecting the bilateral thalami, putamina and cerebral white matter, which often show a concentric structure on CT and MRI. To reveal the pathological substrate of this finding, comparison was made between CT and necropsy findings of three fatal cases of ANE. Cranial CT demonstrated a concentric structure of the thalamocerebral lesions in one patient who died 3.5 days after the onset of encephalopathy, but not in the other two patients who died within 30 h. Neuropathological examination of postmortem brains revealed laminar changes of vascular and parenchymal pathology in all the cases. Excessive permeability of blood vessels and resultant vasogenic edema became more prominent with increasing depth from the cerebral surface. The deep portion of the lesions showed severe perivascular hemorrhage, accounting for the central high density on the CT images of one patient. (orig.)

  17. Chinese Consensus on Diagnosis and Treatment of Hepatic Encephalopathy

    Institute of Scientific and Technical Information of China (English)

    Xin-yue; Chen; Jun; Cheng; Fang-ling; Duan; Jian-gao; Fan; Xiao-ling; Fan; Li-min; Guo; Ming-zhou; Guo; Tao; Han; Jing-yuan; Liu; Xiong; Ma; Wei; Shen; Shi-guang; Feng; Xian-bo; Wang; Yun; Wu; Wen; Xie; Yao; Xie; Hui-chun; Xing; Ping-geYuan; Yong-ping; Yang; Fu-kui; Zhang

    2012-01-01

    Hepatic encephalopathy(HE)is a complex,neuropsychiatric abnormality that occurs as a consequence of metabolic disorders in patients with hepatic insufficiency.The pathogenesis is complex with a strong prognosticator of death.To standardize the clinical management of HE,relevant new data were reviewed and assessed by Chinese Committee of Experts on Hepatic Encephalopathy in China and was discussed and debated extensively.Then the consensus on the management of HE was developed.The final recommendations are based on the data available at the time of production of the document and may be updated with pertinent scientific developments at a later time.All the discussion was organized by the editorial board of Chinese Journal of Experimental and Clinical Infectious Diseases(Electronic Edition),Chinese Journal of Liver Diseases(Electronic Edition)and Infection International(Electronic Edition).The evidence gradings in the consensus are listed in Table1.

  18. Contributions of Microdialysis to New Alternative Therapeutics for Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Liliana Carmona-Aparicio

    2013-08-01

    Full Text Available Hepatic encephalopathy (HE is a common complication of cirrhosis, of largely reversible impairment of brain function occurring in patients with acute or chronic liver failure or when the liver is bypassed by portosystemic shunts. The mechanisms causing this brain dysfunction are still largely unclear. The need to avoid complications caused by late diagnosis has attracted interest to understand the mechanisms underlying neuronal damage in order to find markers that will allow timely diagnosis and to propose new therapeutic alternatives to improve the care of patients. One of the experimental approaches to study HE is microdialysis; this technique allows evaluation of different chemical substances in several organs through the recollection of samples in specific places by semi-permeable membranes. In this review we will discuss the contributions of microdialysis in the understanding of the physiological alterations in human hepatic encephalopathy and experimental models and the studies to find novel alternative therapies for this disease.

  19. Therapeutic effect of gangliosides on patients with radiation encephalopathy

    International Nuclear Information System (INIS)

    To study the therapeutic effect of gangliosides injection on radiation encephalopathy(REP). Methods: Forty patients with REP after radiotherapy for nasopharyngeal carcinoma were divided into experimental (conventional therapy combined with gangliosides) and the controls (conventional therapy only) groups randomly. The clinical therapeutic outcomes were observed and compared in the 2 groups. Results: The therapeutic outcomes were better in the experimental group than that between the control group obviously (P<0.05). Conclusion: Gangliosides has an obvious therapeutic effect on REP. (authors)

  20. Language in Children with Neonatal Hypoxic-Ischemic Encephalopathy

    OpenAIRE

    Martinez, Chenia; Carneiro, Luciana; Vernier, Luíza; Cesa, Carla; Guardiola, Ana; Vidor, Deisi

    2014-01-01

    Introduction Neonatal hypoxic-ischemic encephalopathy (NHIE) is a common neurologic injury, and it may compromise the child's language and cognition. Understanding the process of language acquisition becomes possible with concise knowledge about children's global development. Objective The aim of this study was to observe if language acquisition and development are impaired in children with NHIE. Methods Seventy children with NHIE from 1 to 24 months old were analyzed in a Pediatric Neurology...

  1. Rifaximin therapy and hepatic encephalopathy: Pros and cons

    OpenAIRE

    2012-01-01

    Hepatic encephalopathy (HE) is the second most common major complication in cirrhotics and it significantly impacts quality of life. Therapeutic approaches for HE treatment and prevention mainly continue to rely on ammonia-lowering strategies and non-absorbable disaccharides are currently considered the cornerstone therapy. Non-absorbable antibiotics, such as neomycin and paramomycin, are effective in treatment of acute HE episodes but their prolonged use for recurrence prevention is hampered...

  2. Safety, efficacy, and patient acceptability of rifaximin for hepatic encephalopathy

    OpenAIRE

    Kimer N; Krag A; Gluud LL

    2014-01-01

    Nina Kimer,1 Aleksander Krag,2 Lise L Gluud1 1Gastrounit, Medical Division, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark; 2Department of Gastroenterology, Odense University Hospital, University of Southern Denmark, Odense, Denmark Abstract: Hepatic encephalopathy is a complex disease entity ranging from mild cognitive dysfunction to deep coma. Traditionally, treatment has focused on a reduction of ammonia through a reduced production, absorption, or clearance. Rifaximin is a no...

  3. [Ultrastructural changes of the astroglia in experimental postresuscitation encephalopathy

    OpenAIRE

    Tertishniy S.I.

    2015-01-01

    Background. Astroglia plays crucial role in the functioning of the central nervous system both in normal and in pathological conditions. Data concerning changes of the astroglia during postresuscitational brain pathology are reare and fragmentary. Objective. To study morphogenesis of the ultrastructural changes in astrocytes in postresuscitational encephalopathy after experimental clinical death. Methods. Clinical death lasting 6-8 minutes was modeled on 17 domestic cats with subsequent resus...

  4. Comparative Neuroprotective Effects of Dexamethasone and Minocycline during Hepatic Encephalopathy

    OpenAIRE

    Maha Gamal; Zainab Abdel Wahab; Mohamed Eshra; Laila Rashed; Nivin Sharawy

    2014-01-01

    Objective. Encephalopathy and brain edema are serious complications of acute liver injury and may lead to rapid death of patients. The present study was designed to investigate the role of the inflammatory mediators and oxidative stress in the cytotoxic brain oedema and the neuroprotective effects of both minocycline and dexamethasone. Methods. 48 male albino rats were divided into 4 groups: control group, acute liver injury (ALI) group, minocycline pretreated ALI group, and dexamethasone...

  5. Baclofen-Induced Encephalopathy in End Stage Renal Disease

    Directory of Open Access Journals (Sweden)

    Andrew Meillier

    2015-01-01

    Full Text Available Baclofen is a highly used centrally acting GABA agonist that continues to be an effective therapy for spasticity and chronic hiccups. The renally dependent excretion determines the circulating concentrations and guides effective dosing to decrease adverse reactions. Caution should be considered in administering baclofen to patients with decreased renal function. We present a patient with end stage renal disease on hemodialysis with recent baclofen ingestion who presented with toxic encephalopathy that was resolved with additional dialysis sessions.

  6. Recurrent encephalopathy? No I’m a sleeping beauty!

    OpenAIRE

    Mehtab Iqbal; Manish Prasad; Christopher Ritey

    2014-01-01

    To describe the clinical presentation of ′Kleine-Levin (sleeping beauty) syndrome′ in a child, who presented with recurrent episodes consistent with encephalopathy, associated with excessive sleepiness, cognitive and behavioural disturbance and hyper sexuality. 14 years old boy presented acutely with excessive tiredness, sleeping excessively, abnormal behaviour and hypersexuality following a viral throat infection. On examination he was sleepy but easily arousable. His GCS (15/15) and rest of...

  7. Fatal encephalopathy after an isolated overdose of cocaine

    OpenAIRE

    Kondziella, D.; Danielsen, E R; Arlien-Soeborg, P

    2007-01-01

    Cocaine induced brain damage can be divided into primary neurotoxic effects causing toxic encephalopathy, secondary effects of compromised cerebral blood flow in ischaemic and haemorrhagic stroke, cerebral vasculitis and vasospasm, and tertiary effects due to hypoxia as a result of cardiopulmonary collapse. Toxic leucoencephalopathy mainly affects white matter (WM) tracts serving higher cerebral function, thereby leading to altered personality, attention deficits and memory impairment in mild...

  8. Event-related evoked potentials in chronic respiratory encephalopathy

    OpenAIRE

    Zaidan, Radwan

    2010-01-01

    A R Al Tahan1, R Zaidan1, S Jones2, A Husain3, A Mobeireek1, A Bahammam11Department of Medicine, 3Department of Physiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia; 2Department of Neurophysiology, Institute of Neurology, London, UKBackground: Cognitive event-related potential (P300) is an index of cognitive processing time. It was found to be prolonged in dementia, renal, and hepatic encephalopathies, but was not extensively assessed in respiratory failure.Objective: T...

  9. Clinical, imagiological and etiological spectrum of posterior reversible encephalopathy syndrome

    OpenAIRE

    Pereira, PR; Pinho, J; Rodrigues, M; Sousa, F; Amorim, J; Ribeiro, M.; Rocha, J.; Ferreira, C.

    2015-01-01

    Objective Analyze the cases of posterior reversible encephalopathy syndrome (PRES) admitted in a Neurology Department during an 8-year period. Method Retrospective observational study in a central hospital in the north of Portugal. Results 14 patients were identified, mean age 52.3 years. Precipitating factors included: eclampsia, isolated arterial hypertension, spinal trauma and autonomic dysreflexia, Guillain-Barré syndrome, sepsis, sarcoidosis and pulmonary cryptococcosis and drugs. Most p...

  10. Linkage of gut microbiome with cognition in hepatic encephalopathy

    OpenAIRE

    Jasmohan S. Bajaj; Ridlon, Jason M.; Hylemon, Phillip B; Thacker, Leroy R.; Heuman, Douglas M; Smith, Sean; Sikaroodi, Masoumeh; Gillevet, Patrick M

    2011-01-01

    Hepatic encephalopathy (HE) has been related to gut bacteria and inflammation in the setting of intestinal barrier dysfunction. We aimed to link the gut microbiome with cognition and inflammation in HE using a systems biology approach. Multitag pyrosequencing (MTPS) was performed on stool of cirrhotics and age-matched controls. Cirrhotics with/without HE underwent cognitive testing, inflammatory cytokines, and endotoxin analysis. Patients with HE were compared with those without HE using a co...

  11. Long-term neurodevelopmental outcome with hypoxic-ischemic encephalopathy

    OpenAIRE

    Perez, Anna; Ritter, Susanne; Brotschi, Barbara; Werner, Helen; Caflisch, Jon; Martin, Ernst; Latal, Beatrice

    2013-01-01

    OBJECTIVES: To determine the long-term neurodevelopmental outcome for children after hypoxic-ischemic encephalopathy (HIE) without major disability, and to examine neonatal injury patterns detected on cerebral magnetic resonance imaging (MRI) in relation to later deficits. STUDY DESIGN: Prospectively enrolled children with HIE and neonatal cerebral MRI data (n = 68) were examined at a mean age of 11.2 years (range, 8.2-15.7 years). Eleven children had a major disability (ie, cerebral palsy or...

  12. Impact of Pretransplant Hepatic Encephalopathy on Liver Posttransplantation Outcomes

    OpenAIRE

    Teperman, Lewis W.

    2013-01-01

    Patients with cirrhosis commonly experience hepatic encephalopathy (HE), a condition associated with alterations in behavior, cognitive function, consciousness, and neuromuscular function of varying severity. HE occurring before liver transplant can have a substantial negative impact on posttransplant outcomes, and preoperative history of HE may be a predictor of posttransplant neurologic complications. Even with resolution of previous episodes of overt or minimal HE, some patients continue t...

  13. Contemporary Understanding and Management of Overt and Covert Hepatic Encephalopathy

    OpenAIRE

    NeSmith, Meghan; Ahn, Joseph; Flamm, Steven L.

    2016-01-01

    Hepatic encephalopathy (HE) is a major complication of liver disease that leads to significant morbidity and mortality. Caring for hospitalized patients with HE is becoming more complex, and the economic burden of HE continues to rise. Defining and diagnosing HE, particularly covert HE (CHE), remain challenging. In this article, we review new tools and those currently under development for the diagnosis of CHE and the latest advances in the acute and long-term management of overt HE (OHE) and...

  14. Approach to Clinical Syndrome of Jaundice and Encephalopathy in Tropics

    OpenAIRE

    Anand, Anil C; Garg, Hitendra K.

    2014-01-01

    A large number of patients present with jaundice and encephalopathy in tropical country like India and acute liver failure is the usual cause. Clinical presentation like ALF is also a complication of many tropical infections, and these conditions may mimic ALF but may have subtle differences from ALF. Moreover, what hepatologists see as acute liver failure in tropics is different from what is commonly described in Western Textbooks. Paracetamol overdose, which is possibly the commonest cause ...

  15. Approach to clinical syndrome of jaundice and encephalopathy in tropics.

    Science.gov (United States)

    Anand, Anil C; Garg, Hitendra K

    2015-03-01

    A large number of patients present with jaundice and encephalopathy in tropical country like India and acute liver failure is the usual cause. Clinical presentation like ALF is also a complication of many tropical infections, and these conditions may mimic ALF but may have subtle differences from ALF. Moreover, what hepatologists see as acute liver failure in tropics is different from what is commonly described in Western Textbooks. Paracetamol overdose, which is possibly the commonest cause of ALF in UK and USA, is hardly ever seen in India. Most common etiology here is viral hepatitis (hepatitis E > hepatitis B> hepatitis A). Apart from ALF, one may also come across subacute hepatic failure (SAHF) as well as acute-on-chronic liver failure (ACLF) due to viral hepatitis. Interestingly, a host of other conditions can mimic ALF because clinical presentation in these conditions can be dominated by jaundice and encephalopathy. Malarial hepatopathy is possibly the best-known condition out of these and is not an uncommon manifestation of severe malaria. A similar presentation can also be seen in other common infections in tropics such as dengue fever, typhoid fever, leptospirosis, scrub typhus, amoebic liver abscesses, tuberculosis and other bacterial and fungal infections with or without human immunodeficiency virus (HIV) related disease. In many of these conditions, liver failure may not be underlying pathophysiology. Some pregnancy related liver diseases could also present with jaundice and encephalopathy. This review summarizes the commonly seen presentations in tropical country like India, where jaundice and encephalopathy dominate the clinical picture. PMID:26041951

  16. Morphological changes of cortical pyramidal neurons in hepatic encephalopathy

    OpenAIRE

    Chen, Jeng-Rung; Wang, Bing-Ning; Tseng, Guo-Fang; Wang, Yueh-Jan; Huang, Yong-San; Wang, Tsyr-Jiuan

    2014-01-01

    Background Hepatic encephalopathy (HE) is a reversible neuropsychiatric syndrome associated with acute and chronic liver diseases. It includes a number of neuropsychiatric disturbances including impaired motor activity and coordination, intellectual and cognitive function. Results In the present study, we used a chronic rat HE model by ligation of the bile duct (BDL) for 4 weeks. These rats showed increased plasma ammonia level, bile duct hyperplasia and impaired spatial learning memory and m...

  17. Guillain-Barre syndrome with posterior reversible encephalopathy syndrome

    OpenAIRE

    Basavaraj F Banakar; Pujar, Guruprasad S.; Amita Bhargava; Shubhkaran Khichar

    2014-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity commonly associated with eclampsia, septicemia, chemotherapeutic drugs etc. Concurrent occurrence of Guillain-Barre syndrome (GBS) with PRES is a rare entity. Dysautonomia is a proposed mechanism for such occurrence. Here we present a non-diabetic, non-hypertensive 63-year-old male patient, who came with acute onset flaccid quadriparesis, developing generalized seizures, altered sensorium and raised blood pressu...

  18. Wernicke's encephalopathy in a patient with masticator and parapharyngeal space abscess: a case report

    Science.gov (United States)

    2016-01-01

    Wernicke's encephalopathy is a fatal neurological disease caused by thiamine deficiency. Many reports indicate that Wernicke's encephalopathy is caused by malnutrition. We report the case of a 79-year-old female patient who had a left masticator space and parapharyngeal space abscess who was diagnosed with Wernicke's encephalopathy. She reported problems while eating due to the presence of the abscess, but the true quantities of food she was ingesting were never assessed. Clinicians have a responsibility to provide adequate nutritional support by ensuring that patients receive adequate nutrition. Clinicians should also keep in mind that Wernicke's encephalopathy may occur in patients who experienced prolonged periods of malnutrition. PMID:27162754

  19. Study of Posterior Reversible Encephalopathy Syndrome in Children With Acute Lymphoblastic Leukemia After Induction Chemotherapy.

    Science.gov (United States)

    Tang, Ji-Hong; Tian, Jian-Mei; Sheng, Mao; Hu, Shao-Yan; Li, Yan; Zhang, Li-Ya; Gu, Qing; Wang, Qi

    2016-03-01

    Increasing occurrence of posterior reversible encephalopathy syndrome has been reported in children with acute lymphoblastic leukemia. However, the etiology of posterior reversible encephalopathy syndrome is not clear. To study the possible pathogenetic mechanisms and treatment of this complication, we reported 11 cases of pediatric acute lymphoblastic leukemia who developed posterior reversible encephalopathy syndrome after induction chemotherapy. After appropriate treatment, the clinical symptoms of posterior reversible encephalopathy syndrome in most cases disappeared even though induction chemotherapy continued. During the 1-year follow-up, no recurrence of posterior reversible encephalopathy syndrome was observed. Although the clinical and imaging features of posterior reversible encephalopathy syndrome may be diverse, posterior reversible encephalopathy syndrome should be recognized as a possible important complication of acute lymphoblastic leukemia when neurologic symptoms appear. In line with previous reports, our study also indicated that posterior reversible encephalopathy syndrome was reversible when diagnosed and treated at an early stage. Thus, the occurrence of posterior reversible encephalopathy syndrome should be considered and investigated to optimize the early induction scheme of acute lymphoblastic leukemia treatment. PMID:26060305

  20. Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders.

    Science.gov (United States)

    Latt, N; Dore, G

    2014-09-01

    Wernicke encephalopathy is an acute, reversible neuropsychiatric emergency due to thiamine deficiency. Urgent and adequate thiamine replacement is necessary to avoid death or progression to Korsakoff syndrome with largely irreversible brain damage. Wernicke Korsakoff syndrome refers to a condition where features of Wernicke encephalopathy are mixed with those of Korsakoff syndrome. Although thiamine is the cornerstone of treatment of Wernicke encephalopathy, there are no universally accepted guidelines with regard to its optimal dose, mode of administration, frequency of administration or duration of treatment. Currently, different dose recommendations are being made. We present recommendations for the assessment and treatment of Wernicke encephalopathy based on literature review and our clinical experience. PMID:25201422

  1. Research progress of BOLD-fMRI in minimal hepatic encephalopathy

    International Nuclear Information System (INIS)

    The minimal hepatic encephalopathy is the early stage of hepatic encephalopathy. It has few apparent clinical symptoms and specific manifestations, and is difficult to diagnose. In the recent years, BOLD-fMRI has been used to study hepatic encephalopathy gradually. Through detection of the brain neuron activities in different states, it can not only locate the abnormal activity of brain functional areas, but also can find the changes of brain functional connectivity. BOLD- fMRI combining with other MR technologies can explore the pathology and pathogenesis of minimal hepatic encephalopathy from micro to macro and from structure to function. (authors)

  2. Guillain-Barre syndrome with posterior reversible encephalopathy syndrome

    Directory of Open Access Journals (Sweden)

    Basavaraj F Banakar

    2014-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a clinicoradiologic entity commonly associated with eclampsia, septicemia, chemotherapeutic drugs etc. Concurrent occurrence of Guillain-Barre syndrome (GBS with PRES is a rare entity. Dysautonomia is a proposed mechanism for such occurrence. Here we present a non-diabetic, non-hypertensive 63-year-old male patient, who came with acute onset flaccid quadriparesis, developing generalized seizures, altered sensorium and raised blood pressure on fifth day of illness. Magnetic resonance imaging (MRI of brain showed altered signal intensities involving the parieto-occipital areas suggestive of posterior reversible encephalopathy. Cerebrospinal fluid analysis showed albuminocytological dissociation, nerve conduction studies revealed demyelinating type of polyneuropathy. The patient was treated with antihypertensives and antiepileptics. After resolution of the encephalopathy, intravenous immunoglobulin (IVIg was given. The patient recovered gradually over few months. Our case concludes GBS as independent risk factor, for PRES may be secondary to dysautonomia and physicians should be aware of such rare coexistence so that early treatment can be done to reduce the mortality and morbidity.

  3. Current trends in the treatment of hepatic encephalopathy

    Directory of Open Access Journals (Sweden)

    Mohamad Rasm Al Sibae

    2009-07-01

    Full Text Available Mohamad Rasm Al Sibae, Brendan M McGuireDepartment of Medicine, Division of Gastroenterology and Hepatology, University of Alabama at Birmingham, Birmingham, AL, USAAbstract: Hepatic encephalopathy (HE is a common reversible neuropsychiatric syndrome associated with chronic and acute liver dysfunction and significant morbidity and mortality. Although a clear pathogenesis is yet to be determined, elevated ammonia in the serum and central nervous system are the mainstay for pathogenesis and treatment. Management includes early diagnosis and prompt treatment of precipitating factors (infection, gastrointestinal bleeding, electrolyte disturbances, hepatocellular carcinoma, dehydration, hypotension, and use of benzodiazepines, psychoactive drugs, and/or alcohol. Clinical trials have established the efficacy of lactulose and lactitol enemas in the treatment of acute hepatic encephalopathy. Extensive clinical experience has demonstrated the efficacy of oral lactulose and lactitol with the goal of two to three soft bowel movements a day for the treatment of chronic HE. However, lactulose and lactitol have significant gastrointestinal side effects. For patients unable to tolerate lactulose or lactitol or who still have persistent chronic HE with lactulose or lactitol, neomycin, metronidazole and rifaximin are second-line agents. More recent data supports the benefits of rifaximin used solely and as an additional agent with fewer side effects than neomycin or metronidazole. Newer therapies being investigated in humans with clinical promise include nitazoxanide, the molecular adsorbent recirculating system (MARS, L-ornithine phenylacetate, sodium benzoate, and/or sodium phenylacetate and Kremezin® (AST-120.Keywords: hepatic encephalopathy, liver dysfunction, lactulose, lactitol

  4. Comparative Neuroprotective Effects of Dexamethasone and Minocycline during Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Maha Gamal

    2014-01-01

    Full Text Available Objective. Encephalopathy and brain edema are serious complications of acute liver injury and may lead to rapid death of patients. The present study was designed to investigate the role of the inflammatory mediators and oxidative stress in the cytotoxic brain oedema and the neuroprotective effects of both minocycline and dexamethasone. Methods. 48 male albino rats were divided into 4 groups: control group, acute liver injury (ALI group, minocycline pretreated ALI group, and dexamethasone pretreated ALI group. 24 hours after acute liver injury serum ammonia, liver enzymes, brain levels of heme oxygenase-1 gene, iNOS gene expression, nitrite/nitrate, and cytokines were measured. In addition, the grades of encephalopathy and brain water content were assessed. Results. ALI was associated with significant increases in all measured inflammatory mediators, oxidative stress, iNOS gene expression, and nitrite/nitrate. Both minocycline and dexamethasone significantly modulated the inflammatory changes and the oxidative/nitrosative stress associated with ALI. However, only minocycline but not dexamethasone significantly reduced the cytotoxic brain oedema. Conclusion. Both minocycline and dexamethasone could modulate inflammatory and oxidative changes observed in brain after ALI and could be novel preventative therapy for hepatic encephalopathy episodes.

  5. Evaluation of two experimental models of hepatic encephalopathy in rats

    Directory of Open Access Journals (Sweden)

    García-Moreno L.M.

    2005-01-01

    Full Text Available The serious neuropsychological repercussions of hepatic encephalopathy have led to the creation of several experimental models in order to better understand the pathogenesis of the disease. In the present investigation, two possible causes of hepatic encephalopathy, cholestasis and portal hypertension, were chosen to study the behavioral impairments caused by the disease using an object recognition task. This working memory test is based on a paradigm of spontaneous delayed non-matching to sample and was performed 60 days after surgery. Male Wistar rats (225-250 g were divided into three groups: two experimental groups, microsurgical cholestasis (N = 20 and extrahepatic portal hypertension (N = 20, and a control group (N = 20. A mild alteration of the recognition memory occurred in rats with cholestasis compared to control rats and portal hypertensive rats. The latter group showed the poorest performance on the basis of the behavioral indexes tested. In particular, only the control group spent significantly more time exploring novel objects compared to familiar ones (P < 0.001. In addition, the portal hypertension group spent the shortest time exploring both the novel and familiar objects (P < 0.001. These results suggest that the existence of portosystemic collateral circulation per se may be responsible for subclinical encephalopathy.

  6. Bovine Virus Diarrhea (BVD)

    OpenAIRE

    Hoar, Bruce R.

    2004-01-01

    Bovine virus diarrhea (BVD) is a complicated disease to discuss as it can result in a wide variety of disease problems from very mild to very severe. BVD can be one of the most devastating diseases cattle encounter and one of the hardest to get rid of when it attacks a herd. The viruses that cause BVD have been grouped into two genotypes, Type I and Type II. The disease syndrome caused by the two genotypes is basically the same, however disease caused by Type II infection is often more severe...

  7. [Posterior reversible encephalopathy syndrome of the midbrain and hypothalamus - a case report of uremic encephalopathy presenting with hypersomnia].

    Science.gov (United States)

    Shiga, Yuji; Kanaya, Yuhei; Kono, Ryuhei; Takeshima, Shinichi; Shimoe, Yutaka; Kuriyama, Masaru

    2016-01-01

    We report the case of a 73-year-old woman presenting with hypersomnia and loss of appetite. She suffered from diabetic nephropathy without receiving dialysis, in addition to hypertension, which was well controlled without marked fluctuation. There were no objective neurological findings. Her laboratory findings showed renal failure with 3.7 mg/dl of serum creatinine and decreased serum sodium and potassium. Brain magnetic resonance imaging (MRI) showed posterior reversible encephalopathy syndrome (PRES) with vasogenic edema, which was distributed in the dorsal midbrain, medial thalamus, and hypothalamus. After we addressed the electrolyte imbalance and dehydration, her symptoms and MRI findings gradually improved, but faint high signals on MRI were still present 3 months later. Orexin in the cerebrospinal fluid was decreased on admission, but improved 6 months later. We diagnosed uremic encephalopathy with atypical form PRES showing functional disturbance of the hypothalamus. PMID:26640128

  8. Proteomic Analysis of Bovine Nucleolus

    Institute of Scientific and Technical Information of China (English)

    Amrutlal K.Patel; Doug Olson; Suresh K. Tikoo

    2010-01-01

    Nucleolus is the most prominent subnuclear structure, which performs a wide variety of functions in the eu-karyotic cellular processes. In order to understand the structural and functional role of the nucleoli in bovine cells,we analyzed the proteomie composition of the bovine nueleoli. The nucleoli were isolated from Madin Darby bo-vine kidney cells and subjected to proteomie analysis by LC-MS/MS after fractionation by SDS-PAGE and strongcation exchange chromatography. Analysis of the data using the Mascot database search and the GPM databasesearch identified 311 proteins in the bovine nucleoli, which contained 22 proteins previously not identified in theproteomic analysis of human nucleoli. Analysis of the identified proteins using the GoMiner software suggestedthat the bovine nueleoli contained proteins involved in ribosomal biogenesis, cell cycle control, transcriptional,translational and post-translational regulation, transport, and structural organization.

  9. Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein

    OpenAIRE

    Wadsworth, Jonathan D. F.; Joiner, Susan; Linehan, Jacqueline M; Balkema-Buschmann, Anne; Spiropoulos, John; Simmons, Marion M; Griffiths, Peter C; Martin H Groschup; Hope, James; Brandner, Sebastian; Asante, Emmanuel A.; Collinge, John

    2013-01-01

    Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. One example is atypical/Nor98 scrapie, which evaded statutory diagnostic methods worldwide until the early 2000s. To investigate whether sheep infected with scrapie prions could be another source of infection, we inoculated transgenic mice that overexpressed human prion protein with brain tissue...

  10. Prions in skeletal muscle

    OpenAIRE

    Patrick J Bosque; Ryou, Chongsuk; Telling, Glenn; Peretz, David; Legname, Giuseppe; Stephen J DeArmond; Prusiner, Stanley B.

    2002-01-01

    Considerable evidence argues that consumption of beef products from cattle infected with bovine spongiform encephalopathy (BSE) prions causes new variant Creutzfeldt–Jakob disease. In an effort to prevent new variant Creutzfeldt–Jakob disease, certain “specified offals,” including neural and lymphatic tissues, thought to contain high titers of prions have been excluded from foods destined for human consumption [Phillips, N. A., Bridgeman, J. & Ferguson-Smith, M. (2000) in The BSE Inquiry (Sta...

  11. A prion primer

    OpenAIRE

    Cashman, N R

    1997-01-01

    By biological and medical criteria, prions are infectious agents; however, many of their properties differ profoundly from those of conventional microbes. Prions are "encoded" by alterations in protein conformation rather than in nucleic acid or amino acid sequence. New epidemic prion diseases (bovine spongiform encephalopathy and new variant Creutzfeldt-Jakob disease) have recently emerged under the active surveillance of the modern world. The risk of contracting prion disease from blood pro...

  12. Highly sensitive, quantitative cell-based assay for prions adsorbed to solid surfaces

    OpenAIRE

    Edgeworth, J. A.; Jackson, G. S.; Clarke, A R; Weissmann, C; Collinge, J.

    2009-01-01

    Prions are comprised principally of aggregates of a misfolded host protein and cause fatal transmissible neurodegenerative disorders of humans and animals, such as variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Prions pose significant public health concerns, including contamination of blood products and surgical instruments; require laborious and often insensitive animal bioassay to detect; and resist conventional hospital sterilization methods. A major experimental a...

  13. Influenza - flu

    OpenAIRE

    Valčić Miroslav A.; Radojičić Sonja

    2010-01-01

    In epidemiology or in epizootiology, there are some infectious diseases that have potential for significant reduction of the susceptible species population. Over the past few decades, epidemiologists were concentrated on diseases that were 'modern' and made front-page news in tabloids. One should recall diseases like bovine spongiform encephalopathy, SARS and AIDS syndromes. However, we should always be aware of the most dangerous diseases such as our old friend, inf...

  14. Food risks and consumer trust : European governance of Avian influenza

    OpenAIRE

    Krom, de, WHC Wilbert

    2010-01-01

    During the 1990s, many European countries faced one or more food crises, such as bovine spongiform encephalopathy (BSE), E. coli, dioxin residues, and foot-and-mouth disease. These crises were marked by a growing public recognition of food-related risks and the changing nature of these risks, and tended to undermine citizen-consumer trust in the practices and institutions that managed food safety. To restore and retain trust in food throughout Europe, the European food policy framework was su...

  15. Consumer's behaviour with respect to meat demand in the presence of animal disease concerns: the special case of consumers who eat bison, elk, and venison

    OpenAIRE

    Myae, Aye C.; Goddard, Ellen W.

    2010-01-01

    Prion diseases have raised concerns in consumer’s minds about food safety associated with meat world-wide. Bovine Spongiform Encephalopathy (BSE) and Chronic Wasting Disease (CWD) both exist in Canada and consumer markets for beef, bison, elk and deer may have been affected by the diseases. While numerous studies have examined Canadian consumer beef purchasing behavior in the presence of BSE (Lomeli (2005), John(2007)), no examination of the impact of the animal diseases on consumer behavior ...

  16. ПРИОННЫЕ БОЛЕЗНИ ДИКИХ И СОДЕРЖАЩИХСЯ В НЕВОЛЕ ЖИВОТНЫХ

    OpenAIRE

    С.С. Рыбаков; К.Н. Груздев

    2004-01-01

    The symptoms of human and animal transmissible spongiform encephalopathies (TSEs) are described. The differences between a pathogenic form of a prion protein, a new class of infectious agents, and a normal one are specified. A brief description of some prion diseases affecting wild and captive animals (chronic wasting disease of deer and elks, captive bovine TSEs, feline TSEs, TSE of mink) is given. The possibility of chronic wasting disease transmission to man is discussed. The results of TS...

  17. How do U.S. and Canadian consumers value credence attributes associated with beef labels after the North American BSE crisis of 2003?

    OpenAIRE

    Steiner, Bodo E.; Yang, Jun

    2010-01-01

    A consumer survey conducted in 2006 (n = 419), and therefore after the first confirmed bovine spongiform encephalopathy (BSE) cases in North America in 2003, employs attribute-based choice experiments for a cross-country comparison of consumers' valuation of credence attributes associated with beef steak labels; specifically a guarantee that beef was tested for BSE, a guarantee that the steaks were produced without genetically modified organisms (GMOs) and a guarantee that beef steaks were pr...

  18. Fabrication and Characteristics of Chitosan Sponge as a Tissue Engineering Scaffold

    OpenAIRE

    Ikeda, Takeshi; Ikeda, Kahori; Yamamoto, Kouhei; Ishizaki, Hidetaka; Yoshizawa, Yuu; Yanagiguchi, Kajiro; Yamada, Shizuka; Hayashi, Yoshihiko

    2014-01-01

    Cells, growth factors, and scaffolds are the three main factors required to create a tissue-engineered construct. After the appearance of bovine spongiform encephalopathy (BSE), considerable attention has therefore been focused on nonbovine materials. In this study, we examined the properties of a chitosan porous scaffold. A porous chitosan sponge was prepared by the controlled freezing and lyophilization of different concentrations of chitosan solutions. The materials were examined by scanni...

  19. Meat meals. Industrialists go to the rescue; Farines animales. Les industriels a la rescousse

    Energy Technology Data Exchange (ETDEWEB)

    Arnaud, J.F.; Desfilhes, Ph.; Rouaud, P.O.

    2000-12-01

    With the bovine spongiform encephalopathy crisis, the French public authorities are exploring all possible ways to eliminate the meat meals that accumulate in storage sites. These meals can be considered as fuel substitutes and cement companies, big energy consuming industries (Usinor, Saint-Gobain, Pechiney, paper industries etc..), and incinerating facilities for district heating are experimenting some solutions to their elimination. However, the incineration of meat meals can become an additional source of pollution, in particular of dioxins. (J.S.)

  20. Representations of mad cow disease

    OpenAIRE

    Washer, P.

    2006-01-01

    This paper examines the reporting of the story of Bovine Spongiform Encephalopathy (BSE) and its human derivative variant Creutzfeld-Jacob Disease (vCJD) in the British newspapers. Three ‘snapshots’ of newspaper coverage are sampled and analysed between the period 1986 and 1996 focusing on how representations of the disease evolved over the 10-year period. Social representations theory is used to elucidate how this new disease threat was conceptualised in the newspaper reporting a...

  1. Discriminant Analysis of Undaria pinnatifida Production Areas Using Trace Elemental Analysis

    OpenAIRE

    Mikio Kaihara

    2010-01-01

    Increasingly, attention is being paid to declaring the origin of agricultural and marine products after the advent of the bovine spongiform encephalopathy (BSE; commonly known as mad-cow disease). The display of the production centers on U. pinnatifida has been required in Japan since 2006. As an example of testing in another marine product, near-infrared spectra (NIR) and trace elemental analysis of U. pinnatifida are proven effective methods for discriminating production centers...

  2. A survey and a molecular dynamics study on the (central) hydrophobic region of prion proteins

    OpenAIRE

    Zhang, Jiapu; Wang, Feng

    2014-01-01

    Prion diseases are invariably fatal neurodegenerative diseases that affect humans and animals. Unlike most other amyloid forming neurodegenerative diseases, these can be highly infectious. Prion diseases occur in a variety of species. They include the fatal human neurodegenerative diseases Creutzfeldt-Jakob Disease (CJD), Fatal Familial Insomnia (FFI), Gerstmann-Straussler-Scheinker syndrome (GSS), Kuru, the bovine spongiform encephalopathy (BSE or 'mad-cow' disease) in cattle, the chronic wa...

  3. 疯牛病防制研究新进展

    Institute of Scientific and Technical Information of China (English)

    周祖华

    2004-01-01

    @@ 疯牛病(Mad-cow disease)又称牛海绵状脑病(Bovine SpongiformEncephalopathy,BSE),是一种慢性、传染性、致死性的中枢神经系统疾病.该病自1985年首次在英国发现以来,至今已在许多国家都有发现.

  4. Mad Cows and Ailing Hens: The Transatlantic Relationship and Livestock Diseases

    OpenAIRE

    O'Neill, Katherine

    2006-01-01

    This paper examines how the emergence and spread of animal diseases such as bovine spongiform encephalopathy (BSE, or "mad cow disease") or avian influenza have shaped the dynamics of transatlantic trade in live animals and meat products. It then compares the responses of the US and the EU, respectively, to looming, potentially long-term threats of epidemics to human and animal health, focusing particularly on recent outbreaks BSE and avian flu. It documents what appears to be a shift away fr...

  5. Progress and limits of TSE diagnostic tools

    OpenAIRE

    Grassi, Jacques; Maillet, Séverine; Simon, Stéphanie; Morel, Nathalie

    2008-01-01

    International audience Following the two "mad cow" crises of 1996 and 2000, there was an urgent need for rapid and sensitive diagnostic methods to identify animals infected with the bovine spongiform encephalopathy (BSE) agent. This stimulated research in the field of prion diagnosis and led to the establishment of numerous so-called "rapid tests" which have been in use in Europe since 2001 for monitoring at-risk populations (rendering plants) and animals slaughtered for human consumption ...

  6. The Effects of Mad Cow Disease on U.S. Live Cattle Futures Price

    OpenAIRE

    Paiva, Newton N.

    2003-01-01

    Due to red meat consumption, bovine spongiform encephalopathy (BSE) disease has been a major human health concern since its discovery in 1986. An event study approach was applied to determine the impact of BSE official events that occurred in the United Kingdom on U.S. live cattle futures prices. When abnormal returns were aggregated during the course of the events, the price series were adversely affected, mainly after the event day. This suggests that market reaction was dissipated quickly ...

  7. ARIES: an expert system supporting legislative tasks. Identifying animal materials using the Linnaeus II software

    OpenAIRE

    van Raamsdonk, Leo W.D.

    2010-01-01

    Bovine Spongiform Encephalopathy (BSE, Mad cow disease) is generally considered to be caused by recycling animal by-products as ingredient in animal, especially ruminant, feed. Feed bans were enforced to minimize the risk on infections, and monitoring programs are effectuated for controlling the ban. The only official detection method is visual (microscopic) examination of the presence of primarily bone fragments, but muscle fibres, hairs, feather filaments, and fish bones a...

  8. Proteínas de prión : de la patogénesis a la función

    OpenAIRE

    Málaga Trillo, Edward; Solis Padilla, Gonzalo

    2006-01-01

    The prion protein (PrP) is a membrane-anchored glycoprotein normally present in all vertebrates. Under unusual circumstances, it can undergo structural transformation and become the sole constituent of prions, a unique class of infectious agent devoid of nucleic acid. Prions are the cause of a group of lethal neurodegenerative disorders that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) or “mad cow disease”. Much is known about PrP’s pathogenic p...

  9. 令全世界恐慌的疯牛病

    Institute of Scientific and Technical Information of China (English)

    刘卓宝

    2001-01-01

    @@ 疯牛病(mad cow disease,MCD),又称牛海绵状脑病(bovine spongiform encephalopathy,BSE).是指成年牛脑灰白质发生海绵状空洞变性,而呈惊恐、疯癫状表现,最终导致死亡的牛中枢神经系统疾病.

  10. A Trade Regime for Sub-National Exports Under the Agreement on the Application of Sanitary and Phytosanitary Measures

    OpenAIRE

    Loppacher, Laura J.; Kerr, William A.; Barichello, Richard R.

    2006-01-01

    Regulations relating to disease management have traditionally been an important component of the overall environment in which international trade in agriculture products occurs. The World Trade Organization (WTO) Agreement on the Application of Sanitary and Phytosanitary Measures (SPS Agreement) allows members to restrict or prohibit imports from a country when imported products present a risk to human, animal or plant health or life. As the Bovine Spongiform Encephalopathy (BSE, also commonl...

  11. Consumer Responses to Recent BSE Events

    OpenAIRE

    Pritchett, James G.; Johnson, Kamina K.; Thilmany, Dawn D.; Hahn, William F.

    2007-01-01

    Recent bovine spongiform encephalopathy (BSE, a.k.a. mad cow disease) discoveries in Canadian and U.S. beef cattle have garnered significant media attention, which may have changed consumers’ meat-purchasing behavior. Consumer response is hypothesized and tested within a meat demand system in which response is measured using single-period dummy variables, longer-term dummy variables, and media indices that count positive and negative meat-industry articles. Parameters are estimated using re...

  12. Population trends of grassland birds in North America are linked to the prevalence of an agricultural epizootic in Europe

    OpenAIRE

    Nocera, Joseph J.; Koslowsky, Hannah M.

    2011-01-01

    Globalization of trade has dramatic socioeconomic effects, and, intuitively, significant ecological effects should follow. However, few quantitative examples exist of the interrelationship of globalization, socioeconomics, and ecological patterns. We present a striking illustration of a cascade in which bovine spongiform encephalopathy (BSE; “mad cow disease”) outbreaks in Europe exerted pressure on global beef markets, subsequently affecting North American hayfields and grassland bird popula...

  13. Follicular dendritic cells related to nerve fibres and cellular prion protein expression in ileal and jejunal Peyer’s patches of cows and calves

    OpenAIRE

    Defaweux, Valérie; Antoine, Nadine; Dorban, G.; C Demonceau; Piret, Joëlle; Heinen, Ernst

    2005-01-01

    Prion pathogenesis following oral exposure is thought to involve gut-associated lymphoid tissue, which includes Peyer’s patches (PP). Before neuroinvasion, early accumulation of infectious prion protein (PrPsc) takes place on follicular dendritic cells (FDC) which are resident cells in germinal centres. The strain, the infection pathway and the lesions in the central nervous system are similar between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt Jakob diseases. But in BSE, t...

  14. Neuroinvasion by a Creutzfeldt–Jakob disease agent in the absence of B cells and follicular dendritic cells

    OpenAIRE

    Shlomchik, Mark J.; Radebold, Klaus; Duclos, Nicole; Manuelidis, Laura

    2001-01-01

    With the potential spread of bovine spongiform encephalopathy to people as a variant Creutzfeldt–Jakob disease (CJD), it becomes critical to identify cells in the periphery that carry infection. Initial work with scrapie agents suggested that B cells were central vectors for neuroinvasion. Subsequent studies indicated that B cells played an indirect role by promoting the development of follicular dendritic cells (FDCs) that accumulate abnormal prion protein (PrP). The mechanism for the role o...

  15. Computational Studies of the Structural Stability of Rabbit Prion Protein Compared to Human and Mouse Prion Proteins

    OpenAIRE

    Zhang, Jiapu

    2011-01-01

    Prion diseases are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. The neurodegenerative diseases such as Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob diseases, Gerstmann-Str$\\ddot{a}$ussler-Scheinker syndrome, Fatal Familial Insomnia, Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (or 'mad-cow' disease) and chronic wasting disease in cattle belong to prion diseases. By now there have not been some effective therapeut...

  16. Comparison of strategies for substantiating freedom from scrapie in a sheep flock

    OpenAIRE

    Ducrot Christian; Calavas Didier; Martinez Marie-José; Durand Benoit

    2009-01-01

    Abstract Background The public health threat represented by a potential circulation of bovine spongiform encephalopathy agent in sheep population has led European animal health authorities to launch large screening and genetic selection programmes. If demonstrated, such a circulation would have dramatic economic consequences for sheep breeding sector. In this context, it is important to evaluate the feasibility of qualification procedures that would allow sheep breeders demonstrating their fl...

  17. Getting Access Into the EU Market: A Coordination Challenge for the Brazilian Beef Chain

    OpenAIRE

    Soares, Manoel Rossi; Miranda, Silvia Helena Galvao de; Zuurbier, Peter J.P.

    2005-01-01

    Brazil is the world beef export leader since 2004, when he Bovine Spongiform Encephalopathy (BSE) crisis in the United States and the severe draught in Australia dropped those countries beef exports and gave Brazilian beef chain a unique opportunity to enter in new markets. Brazil is facing now a major challenge in terms of aggregating value, since its export volume is already stabilizing. The European Union (EU) represents the most demanding market in terms of quality and one in which Brazil...

  18. Transmission of scrapie prions to primate after an extended silent incubation period

    OpenAIRE

    Emmanuel E Comoy; Mikol, Jacqueline; Luccantoni-Freire, Sophie; Correia, Evelyne; Lescoutra-Etchegaray, Nathalie; Durand, Valérie; Dehen, Capucine; Andreoletti, Olivier; Casalone, Cristina; Richt, Juergen A.; Greenlee, Justin J.; Baron, Thierry; Benestad, Sylvie L.; Brown, Paul; Deslys, Jean-Philippe

    2015-01-01

    Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly ...

  19. Assessment of Alternative Phosphorus Fertilizers for Organic Farming: Meat and Bone Meal

    OpenAIRE

    Möller, Kurt (Prof. Dr. phil. habil.)

    2015-01-01

    In the past meat and bone meal was a major source of nutrients for recycling back to agricultural land, either as animal feed or organic nitrogen and phosphorus fertilizer. Nowadays - since the Bovine Spongiform Encephalopathy (BSE) crisis in 1999 - it is mainly used as fertilizer. Although meat and bone meals are allowed by EU regulation in organic farming, several growers’ organisations prohibited them since the BSE crisis. Incineration or melting in a cupola furnace are alternative treatme...

  20. Feed-borne transmission and case clustering of BSE.

    OpenAIRE

    Hagenaars, T J; Ferguson, N. M.; Donnelly, C A; Ghani, A.C.; Anderson, R M

    2000-01-01

    An unresolved issue in the epidemiology of bovine spongiform encephalopathy (BSE) in the UK is what precisely determines the degree to which cases of disease in cattle are clustered within herds throughout the course of the epidemic. This paper presents an analysis of feed-borne transmission at the herd level and tests various models of case-clustering mechanisms, associated with heterogeneity in exposure to infectious feed, against observed epidemic pattern. We use an age-structured metapopu...

  1. Traceability and the new CAP

    OpenAIRE

    Maraveyas, Napoleon N.; Doukas, Yannis El.

    2009-01-01

    The Common Agricultural Policy (CAP) was strongly criticized for the food safety crises of the 1990s which included Bovine Spongiform Encephalopathy (BSE), dioxin, foot and mouth disease and swine fever to name a few. Even though the first rules on food safety date from the very early days of the EU, a need was recognized to replace a number of these rules accumulated through the years, whose implementation was difficult to monitor, with a simpler and more comprehensive approach. The result w...

  2. Small-Molecule Theranostic Probes: A Promising Future in Neurodegenerative Diseases

    OpenAIRE

    Suzana Aulić; Maria Laura Bolognesi; Giuseppe Legname

    2013-01-01

    Prion diseases are fatal neurodegenerative illnesses, which include Creutzfeldt-Jakob disease in humans and scrapie, chronic wasting disease, and bovine spongiform encephalopathy in animals. They are caused by unconventional infectious agents consisting primarily of misfolded, aggregated, β -sheet-rich isoforms, denoted prions, of the physiological cellular prion protein (PrPC). Many lines of evidence suggest that prions (PrPSc) act both as a template for this conversion and as a neurotoxic a...

  3. Supplanting ecosystem services provided by scavengers raises greenhouse gas emissions

    OpenAIRE

    Morales-Reyes, Z.; Pérez-García, Juan M.; Moleón, Marcos; Botella, Francisco; Carrete, Martina; Lazcano, Carolina; Moreno-Opo, Rubén; Margalida, Antoni; Donázar, José A.; Sánchez-Zapata, José A.

    2015-01-01

    Global warming due to human-induced increments in atmospheric concentrations of greenhouse gases (GHG) is one of the most debated topics among environmentalists and politicians worldwide. In this paper we assess a novel source of GHG emissions emerged following a controversial policy decision. After the outbreak of bovine spongiform encephalopathy in Europe, the sanitary regulation required that livestock carcasses were collected from farms and transformed or destroyed in auth...

  4. Viral infections and bovine mastitis: a review

    NARCIS (Netherlands)

    Wellenberg, G.J.; Poel, van der W.H.M.; Oirschot, van J.T.

    2002-01-01

    This review deals with the role of viruses in the aetiology of bovine mastitis. Bovine herpesvirus 1, bovine herpesvirus 4, foot-and-mouth disease virus, and parainfluenza 3 virus have been isolated from milk from cows with clinical mastitis. Intramammary inoculations of bovine herpesvirus 1 or para

  5. Effect of antibiotics, prebiotics and probiotics in treatment for hepatic encephalopathy.

    NARCIS (Netherlands)

    Bongaerts, G.P.A.; Severijnen, R.S.V.M.; Timmerman, H.

    2005-01-01

    In order to reduce ammonia production by urease-positive bacteria Solga recently hypothesised (S.F. Solga, Probiotics can treat hepatic encephalopathy, Medical Hypotheses 2003; 61: 307-13), that probiotics are new therapeutics for hepatic encephalopathy (HE), and that they may replace antibiotics an

  6. The Detection of Bovine - Derived Material in Import Meat and Bone Meal by PCR and Microwell Plate Hybridization%进口肉骨粉及动物饲料中牛源性成分的PCR-微孔板杂交检测方法

    Institute of Scientific and Technical Information of China (English)

    王静; 徐宝梁; 王丙武; 王曙光; 杨瑞馥; 张敏丽

    2001-01-01

    @@ 疯牛病(Mad-Cow Disease)即牛海绵状脑病(Bovine Spongiform Encephlopathy,BSE),已被证实与人的克雅氏病(Creutzfeldt Jakob Disease,CJD)有直接关联.人如果食用感染了疯牛病病原的牛肉,就会患上克雅氏病,造成永久性的脑部损伤,脑组织形成海绵状空洞,导致精神错乱、痴呆,最终死亡.

  7. Bovine Rhinitis Viruses Are Common in U.S. Cattle with Bovine Respiratory Disease

    OpenAIRE

    Hause, Ben M.; Collin, Emily A.; Anderson, Joe; Hesse, Richard A.; Anderson, Gary

    2015-01-01

    Bovine rhinitis viruses (BRV) are established etiological agents of bovine respiratory disease complex however little research into their epidemiology and ecology has been published for several decades. In the U.S., only bovine rhinitis A virus 1 (BRAV1) has been identified while bovine rhinitis A virus 2 (BRAV2) and bovine rhinitis B virus (BRBV) were previously only identified in England and Japan, respectively. Metagenomic sequencing of a nasal swab from a bovine respiratory disease (BRD) ...

  8. Event-related evoked potentials in chronic respiratory encephalopathy

    Directory of Open Access Journals (Sweden)

    A R Al Tahan

    2010-02-01

    Full Text Available A R Al Tahan1, R Zaidan1, S Jones2, A Husain3, A Mobeireek1, A Bahammam11Department of Medicine, 3Department of Physiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia; 2Department of Neurophysiology, Institute of Neurology, London, UKBackground: Cognitive event-related potential (P300 is an index of cognitive processing time. It was found to be prolonged in dementia, renal, and hepatic encephalopathies, but was not extensively assessed in respiratory failure.Objective: To evaluate P300 changes in patients with respiratory failure, and especially those with mild or subclinical hypoxic–hypercapnic encephalopathy.Methods: Auditory event-related evoked potential P300 latency was measured using an oddball paradigm in patients with respiratory failure due to any cause (partial pressure of oxygen in arterial blood (PO2 should be 75 mm/Hg or less. Apart from blood gases measurement, patients underwent the Mini-Mental State Examination (MMSE. Patient performances were compared with that of matched normal control. Patients were admitted into the study from outpatient clinics and wards at King Khalid University Hospital and Sahara Hospital.Results: Thirty-four patients (12 women, 22 men were admitted to the study. Ages ranged from 19–67 years with a mean of 46.1 years. Respiratory failure was severe or very severe in 11 patients (33%, and mild or moderate in the rest (66%. Mean value for PO2 and partial pressure of carbon dioxide in arterial blood (PCO2 were 63.7 and 45.2 mm/Hg, respectively. pH mean was 7.4 and O2 saturation was 90.7%. P300 latency ranged from 218 to 393 milliseconds, with a mean of 338.4 milliseconds. In comparison with control (309.9 milliseconds, there was a significant difference (P = 0.007. P300 amplitude differences were not significant. No significant difference in MMSE was noted between mild and severe respiratory failure. Results of detailed neuropsychological assessment were clearly abnormal but were

  9. Risk factor for hypoxic ischemic encephalopathy in children

    International Nuclear Information System (INIS)

    To determine underlying risk factors in neonates with hypoxic ischemic encephalopathy. All neonates (153) with the diagnosis of Hypoxic Ischemic Encephalopathy (HIE) were included in the study. Controls (187) were selected from admissions on the same day. Possible risk factors such as maternal age, parity, antenatal monitoring, place of delivery, prolonged second stage of labour, type of delivery, type of attendant at delivery and the gestational age were noted and compared. Sixty one (39.9%) mothers of asphyxiated babies reported no antenatal visits compared to 24.1% in the control group (OR 2.1, 95% CI 1.3-3.2; p=0.002). Only 6.5% of cases were born in government hospitals (teaching and district) in comparison to 20.9% of controls (OR 3.8, 95% CI 1.9-7.6; p=0.001). In 28.1% of cases, mothers had history of prolonged 2nd stage of labour in comparison to 5.9% of controls (OR 6.3, 95% CI 3.3-11.9; p<0.001). Fifty five cases (35.9%) were delivered by unskilled birth attendants compared to 28 (14.9%) controls (OR 3.2, 95% CI 1.9-5.3; p<0.001). No significant difference was found in maternal age, maternal parity, gestational age and the mode of delivery between the two groups. Delivery by unskilled birth attendant, prolonged second stage of labour, birth in a non-government hospital setup and absence of antenatal care were significant risk factors for hypoxic ischemic encephalopathy in neonates. Improvement in antenatal and intrapartum care may be helpful in decreasing the frequency of this problem. (author)

  10. Posterior reversible encephalopathy syndrome in a patient of organophosphate poisoning

    Directory of Open Access Journals (Sweden)

    Rajesh Phatake

    2014-01-01

    Full Text Available A 32-year-old male presented with a history of consuming some organophosphorous compound with suicidal intention.He was treated with atropine, pralidoxime, ventilator support. During stay patient had persistent irritability, tachycardiaand hypertension despite sedation and labetalol infusion. He developed headache, visual blurring hemiparesis and focal seizures. Magnetic resonance imaging of the brain revealed multifocal hyperintensities mainly in subcortical areas of parietal and occipital regions in T2-weighted images, with increased values of Apparent Diffusion Coefficient, suggesting posterior reversible encephalopathy syndrome (PRES. The possibilities of PRES caused by organophosphorous poisoning either due to hypertension caused by autonomic deregulation or direct neurological toxicity has been discussed.

  11. Wernicke encephalopathy and pellagra in an alcoholic and malnourished patient.

    Science.gov (United States)

    Terada, Norihiko; Kinoshita, Kensuke; Taguchi, Shijima; Tokuda, Yasuharu

    2015-01-01

    Deficiency of multiple vitamins can be identified in alcoholic and malnourished patients. We report a patient with Wernicke encephalopathy, a B1 deficiency and pellagra, a niacin deficiency. A 61-year-old Japanese man presented with generalised weakness. He had drunk alcohol heavily for more than a year without eating adequate meals. Physical examination showed disorientation, eye movement impairment, muscle wasting and a rash over the limbs. Multivitamin supplementations improved all the symptoms. Pellagra causes dementia, diarrhoea, or dermatitis, and can mimic non-specific erythaema in alcoholics. The differential diagnosis between pellagra and non-specific erythaema is important because of the treatability of pellagra by niacin supplementation. PMID:26490997

  12. Posterior reversible encephalopathy syndrome due to seronegative systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Sawan Verma

    2014-09-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a neurotoxic state coupled with a unique computed tomography or magnetic resonance imaging (MRI appearance. Recognized in the setting of a number of complex conditions (preeclampsia/eclampsia, allogeneic bone marrow transplantation, organ transplantation, autoimmune disease and high-dose chemotherapy in the imaging, clinical and laboratory features of this toxic state are becoming better elucidated. We are presenting a case of PRES due to seronegative systemic lupus erythematosus, with MRI findings of diffuse vasogenic edema.

  13. De novo mutations in the classic epileptic encephalopathies

    OpenAIRE

    ,

    2013-01-01

    Epileptic encephalopathies (EE) are a devastating group of severe childhood epilepsy disorders for which the cause is often unknown. Here, we report a screen for de novo mutations in patients with two classical EE: infantile spasms (IS, n=149) and Lennox-Gastaut Syndrome (LGS, n=115). We sequenced the exomes of 264 probands, and their parents, and confirmed 329 de novo mutations. A likelihood analysis showed a significant excess of de novo mutations in the ~4,000 genes that are the most intol...

  14. Depression as a mask for dyscirculatory encephalopathy and its therapy

    Directory of Open Access Journals (Sweden)

    D.V. Neverovskiy

    2014-05-01

    Full Text Available In outpatient practice, neurologists often face patients diagnosed with dyscirculatory encephalopathy (DEP. Sleep disorders, headaches, rapid and unexplained fatigability, and irritability are predominating, which poses a number of complex medical issues. A case report of a 69 year-old patient, who was being followed up for a long time because of DEP diagnosis is reported. However, examination of the neu-ropsychic status revealed the depressive disorder with somatoform complaints, which was verified by a psychiatrist. Psychotherapy and antidepressant therapy led to significant improvement. The differential diagnosis and treatment of depressive disorders in outpatient practice are discussed. 

  15. Evaluation of 80 Term Neonates with Hypoxic Ischemic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Selahattin Katar

    2007-01-01

    Full Text Available This study aimed to review the etiology, clinical - laboratory features and mortality rate of term 80 neonates with perinatal asphyxia admitted to our neonatal unit between January 2005-April 2006. The sex distribution was 24 (%30 female and 56 (% 70 male. The mean gestational age was 38.6±1.3 weeks and weight 3156±561 gram. Of the patients % 46.25 were delivered with a cesarean section and % 53.75 with spontaneous vaginal delivery. The etiologic factors for hypoxic ischemic encephalopathy were % 31.25 force delivery, meconium aspiration, and % 66.25 preeclampsia, eclampsia and diabetic mother’s infant. The distribution of patients according to HIE statging system (Sarnat&Sarnat were as follows: 33 patients (% 41.25 in stage 1, 20 (% 25 in stage 2 and 27 (% 33.75 in stage 3. Seizures were observed in % 33.75 of patients. The mean duration of hospital stay was 10.6±7.7 days for the surviving patients and 4.2±3.4 days for patients who died. Except from central nervous system, liver and kidney were the most involved organs.Perinatal asphyxia remains to be leading cause of neonatal mortality. Hypoxic ischemic encephalopathy is a common newborn problem and cause important mortality and morbidity where low-social –cultural –education conditions with in regions.

  16. Pathogenesis of pancreatic encephalopathy in severe acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Xi-Ping Zhang; Hua Tian

    2007-01-01

    BACKGROUND:Pancreatic encephalopathy (PE) is a serious complication of severe acute pancreatitis (SAP). In recent years, more and more PE cases have been reported worldwide, and the onset PE in the early stage was regarded as a poor prognosis sign of SAP, but the pathogenesis of PE in SAP still has not been clariifed in the past decade. The purpose of this review is to elucidate the possible pathogenesis of PE in SAP. DATA SOURCES:The English-language literature concern-ing PE in this review came from the Database of MEDLINE (period of 1991-2005), and the keywords of severe acute pancreatitis and pancreatic encephalopathy were used in the searching. RESULTS:Many factors were involved in the pathogenesis of PE in SAP. Pancreatin activation, excessive release of cytokines and oxygen free radicals, microcirculation abnormalities of hemodynamic disturbance, ET-1/NO ratio, hypoxemia, bacterial infection, water and electrolyte imbalance, and vitamin B1 deifciency participated in the development of PE in SAP. CONCLUSIONS:The pathogenesis of PE in SAP has not yet been fully understood. The development of PE in SAP may be a multi-factor process. To ifnd out the possible inducing factor is essential to the clinical management of PE in SAP.

  17. Subacute fatal aluminum encephalopathy after reconstructive otoneurosurgery: a case report.

    Science.gov (United States)

    Reusche, E; Pilz, P; Oberascher, G; Lindner, B; Egensperger, R; Gloeckner, K; Trinka, E; Iglseder, B

    2001-10-01

    We report a 52-year-old woman who underwent otoneurosurgery to resect acoustic neurinoma. Bone reconstruction was performed with an aluminium (Al)-containing cement. Six weeks later the patient suffered from loss of consciousness, myoclonic jerks, and persistent grand mal seizures, clinical symptoms that resembled those of lethal dialysis encephalopathy of the 1960s and 1970s. She died 6 months later because of septic complications. Light- and electron-microscopic investigation of the central nervous system (CNS) showed pathognomonic Al-containing intracytoplasmic argyrophilic inclusions in choroid plexus epithelia, neurons, and cortical glia. These changes are characteristics of dialysis-associated encephalopathy (DAE), induced nowadays by long-term ingestion of Al-containing drugs (and with benign clinical courses). Atomic absorption spectrometry showed an increase of mean bulk Al concentration of the cortex and subcortex up to 9.3 microg/g (normal range <2 microg/g); laser microprobe showed the increase of Al in subcellular structures. This unique case again shows the extraordinary neurotoxicity of Al, which was, in our patient, initiated by an amount of about 30 mg Al and apparently caused by direct Al access to the brain parenchyma via a cerebrospinal fluid leakage. PMID:11679949

  18. CT and MR manifestations of acute methyl alcohol toxic encephalopathy

    International Nuclear Information System (INIS)

    Objective: To analyze the CT and MR manifestations of methyl alcohol toxic encephalopathy and to improve the diagnosing value of CT and MRI. Methods: 40 patients with methyl alcohol intoxication were collected in this study, in which CT scan was performed on 40 cases and MRI on 4 cases. All CT and MRI radiological data of brain were retrospectively studied. Results: 13 of 40 cases showed abnormal findings on brain CT and MRI. The most common manifestation (6/13, 46%)was hypodensity in frontal parietal white matter and external capsule-putamen on CT, which showed long or short T1 and long T2 on MR. Hemorrhage in right putamen was found only in 1 patient (1/13,7%). CT showed low density inbilateral external capsule in 4 cases (4/13,31%), in which MR showed long or short T1 and long T2. Low density lesions in subcortical white matter of bilateral frontal and parietal lobes, cingulate gyms and insular lobes were found in 2 patients (2/13,15%). The more severe clinic manifestation, the more obvious brain lesion CT and MRI showed. Conclusion: Brain CT and MR manifestations have great diagnostic value of acute methyl alcohol toxic encephalopathy. MRI was more sensitive and better than CT in finding early brain damage caused by methanol intoxication. (authors)

  19. Is Encephalopathy a Mechanism to Renew Sulfate in Autism?

    Directory of Open Access Journals (Sweden)

    Laurie Lentz-Marino

    2013-01-01

    Full Text Available This paper makes two claims: (1 autism can be characterized as a chronic low-grade encephalopathy, associated with excess exposure to nitric oxide, ammonia and glutamate in the central nervous system, which leads to hippocampal pathologies and resulting cognitive impairment, and (2, encephalitis is provoked by a systemic deficiency in sulfate, but associated seizures and fever support sulfate restoration. We argue that impaired synthesis of cholesterol sulfate in the skin and red blood cells, catalyzed by sunlight and nitric oxide synthase enzymes, creates a state of colloidal instability in the blood manifested as a low zeta potential and increased interfacial stress. Encephalitis, while life-threatening, can result in partial renewal of sulfate supply, promoting neuronal survival. Research is cited showing how taurine may not only help protect neurons from hypochlorite exposure, but also provide a source for sulfate renewal. Several environmental factors can synergistically promote the encephalopathy of autism, including the herbicide, glyphosate, aluminum, mercury, lead, nutritional deficiencies in thiamine and zinc, and yeast overgrowth due to excess dietary sugar. Given these facts, dietary and lifestyle changes, including increased sulfur ingestion, organic whole foods, increased sun exposure, and avoidance of toxins such as aluminum, mercury, and lead, may help to alleviate symptoms or, in some instances, to prevent autism altogether.

  20. A case of tacrolimus-induced encephalopathy after kidney transplantation

    Directory of Open Access Journals (Sweden)

    Myoung Uk Kim

    2011-01-01

    Full Text Available We present a case of tacrolimus-induced encephalopathy after successful kidney transplantation. An 11-year-old girl presented with sudden onset of neurologic symptoms, hypertension, and psychiatric symptoms, with normal kidney function, after kidney transplantation. The symptoms improved after cessation of tacrolimus. Magnetic resonance imaging (MRI showed acute infarction of the middle cerebral artery (MCA territory in the right frontal lobe. Three days later, she had normal mental function and maintained normal blood pressure with left hemiparesis. Follow-up MRI was performed on D19, showing new infarct lesions at both cerebral hemispheres. Ten days later, MRI showed further improvement, but brain single photon emission computed tomography (SPECT showed mild reduction of uptake in both the anterior cingulate gyrus and the left thalamus. One month after onset of symptoms, angiography showed complete resolution of stenosis. However, presenting as a mild fine motor disability of both hands and mild dysarthria, what had been atrophy at both centrum semiovale at 4 months now showed progression to encephalomalacia. There are two points of interest in this case. First, encephalopathy occurred after administration of tacrolimus and improved after discontinuation of the drug. Second, the development of right-side hemiplegia could not be explained by conventional MRI; but through diffusion tensor imaging (DTI and diffusion tensor tractography (DTT of white matter tract, visualization was possible.

  1. Wernicke encephalopathy in a patient with liver failure: Clinical case report.

    Science.gov (United States)

    Zhao, Pan; Zhao, Yanling; Wei, Zhenman; Chen, Jing; Yan, Lilong

    2016-07-01

    Early recognition and diagnosis of Wernicke encephalopathy is pivotal for the prognosis of this medical emergency, especially in patients with liver failure which predisposes individuals to develop hepatic encephalopathy. For these patients, distinguishing between hepatic encephalopathy and Wernicke encephalopathy is a challenge in real-world clinical practice.A male patient with 21-year medical history of liver cirrhosis presented diarrhea and ascites. One month before this visit, he was noted to have poor appetite and progressive fatigue. After admission, although several major symptoms, including diarrhea, ascites, hyponatremia, and hypoproteinemia, were greatly improved through appropriate treatments, his laboratory indicators were not changed much. His appetite was not reversed at discharge. On the 5th day after discharge, the patient suddenly became reluctant to speak and did not remember the recent happenings. Simultaneously, unsteady gait and strabismus occurred. On the basis of clinical manifestations and brain magnetic resonance imaging scan results, the patient was diagnosed as Wernicke encephalopathy and these relative symptoms were resolved after intravenous vitamin B1.To our knowledge, this is the second case report of Wernicke encephalopathy developing in a critically ill cirrhotic patient without hepatocellular carcinoma or operative intervention. Wernicke encephalopathy may be underdiagnosed in these patients and this case raises physicians' awareness of its possible onset. PMID:27399058

  2. Need for early diagnosis of mental and mobility changes in Wernicke encephalopathy.

    Science.gov (United States)

    Wijnia, Jan W; Oudman, Erik; Bresser, Esmay L; Gerridzen, Ineke J; van de Wiel, Albert; Beuman, Carla; Mulder, Cornelis L

    2014-12-01

    Korsakoff syndrome is a chronic form of amnesia resulting from thiamine deficiency. The syndrome can develop from unrecognized or undertreated Wernicke encephalopathy. The intra-individual course of Wernicke-Korsakoff syndrome has not been studied extensively, nor has the temporal progression of gait disturbances and other symptoms of Wernicke encephalopathy. Here we present the detailed history of a patient whose acute symptoms of Wernicke encephalopathy were far from stable. We follow his mobility changes and the shifts in his mental status from global confusion and impaired consciousness to more selective cognitive deficits. His Wernicke encephalopathy was missed and left untreated, being labeled as "probable" Korsakoff syndrome. Patients with a history of self-neglect and alcohol abuse, at risk of or suffering with Wernicke encephalopathy, should receive immediate and adequate vitamin replacement. Self-neglecting alcoholics who are bedridden may have severe illness and probably active Wernicke encephalopathy. In these patients, mobility changes, delirium, or impaired consciousness can be an expression of Wernicke encephalopathy, and should be treated to prevent further damage from the neurologic complications of thiamine deficiency. PMID:25539041

  3. Bovine Tuberculosis, A Zoonotic Disease

    Directory of Open Access Journals (Sweden)

    Tarmudji

    2008-12-01

    Full Text Available Bovine tuberculosis is caused by the infection of Mycobacterium tuberculosis var. bovis (M. bovis. This species is one of Mycobacterium tuberculosis complex, can infect wide range of hosts: cattle and other domesticated animals, wild mammals and humans (zoonotic. M. bovis bacterium from infected hosts can be transmitted to other susceptible animals and humans through respiratory excretes and secretion materials. Humans can be infected with M. bovis by ingested M. bovis contaminated animal products, unpasteurised milk from tuberculosis cows or through respiratory route of contaminated aerosol. Bovine tuberculosis at the first stage does not show any clinical sign but as the disease progress in the next stage which may take several months or years, clinical signs may arise, suh as: fluctuative body temperature, anorexia, lost body weight, coughing, oedema of lymph nodes, increased respiratory frequencies. Pathological lesion of bovine tuberculosis is characterised by the formation of granulomas (tubercles, in which bacterial cells have been localised, most in lymph nodes and pulmonum, but can occur in other organs. The granulomas usually arise in small nodules or tubercles appear yellowish either caseus, caseo-calcareus or calcified. In Indonesia, bovine tuberculosis occurred in dairy cattle since 1905 through the imported dairy cows from Holland and Australian. It was unfortunate that until recently, there were not many research and surveilances of bovine tuberculosis conducted in this country, so the distribution of bovine tuberculosis is unknown. Early serological diagnosis can be done on live cattle by means of tuberculin tests under field conditions. Confirmation can be done by isolation and identification of excreted and secreted samples from the slaughter house. Antibiotic treatment and vaccination were uneffective, therefore the effective control of bovine tuberculosis is suggested by tuberculin tests and by slaughtering the selected

  4. Uremic Encephalopathy with Atypical Magnetic Resonance Features on Diffusion-Weighted Images

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eu Gene; Jeon, Se Jeong; Choi, See Sung [Dept. of Radiology, Wonkwang University School of Medicine and Hospital, Iksan (Korea, Republic of)

    2012-11-15

    Uremic encephalopathy is a well-known disease with typical MR findings including bilateral vasogenic or cytotoxic edema at the cerebral cortex or basal ganglia. Involvement of the basal ganglia has been very rarely reported, typically occurring in uremic-diabetic patients. We recently treated a patient who had non-diabetic uremic encephalopathy with an atypical lesion distribution involving the supratentorial white matter, without cortical or basal ganglia involvement. To the best of our knowledge, this is only the second reported case of non-diabetic uremic encephalopathy with atypical MR findings.

  5. A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke’s Encephalopathy

    Science.gov (United States)

    Solmaz, Soner; Gereklioğlu, Çiğdem; Tan, Meliha; Demir, Şenay; Yeral, Mahmut; Korur, Aslı; Boğa, Can; Özdoğu, Hakan

    2015-01-01

    Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke’s encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke’s encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke’s encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature. PMID:25912759

  6. A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke’s Encephalopathy

    Directory of Open Access Journals (Sweden)

    Soner Solmaz

    2015-12-01

    Full Text Available Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke’s encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke’s encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke’s encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature.

  7. Bovine respiratory disease model based on dual infections with infection with bovine viral diarrhea virus and bovine corona virus

    Science.gov (United States)

    Bovine respiratory disease complex (BRDC) is the leading cause of economic loss in the U.S. cattle industry. BRDC likely results from simultaneous or sequential infections with multiple pathogens including both viruses and bacteria. Bovine viral diarrhea virus (BVDV) and bovine corona virus (BoCV...

  8. Prevalence of subclinical hepatic encephalopathy in cirrhotic patients in China

    Institute of Scientific and Technical Information of China (English)

    Yu-Yuan Li; Yu-Qiang Nie; Wei-Hong Sha; Zheng Zeng; Fu-Ying Yang; Li Ping; Lin Jia

    2004-01-01

    AIM: Subclinical hepatic encephalopathy (SHE) is a common complication of liver diseases. The aim of this study was to find out the normal value of psychometric test and to investigate the prevalence of SHE in Chinese patients with stabilized hepatic cirrhosis.METHODS: Four hundred and nine consecutive cirrhotic patients without overt clinical encephalopathy were screened for SHE by using number connection test part A (NCT-A) and symbol digit test (SDT). SHE was defined as presence of at least one abnormal psychometric test. The age-corrected normal values were defined as the mean±2times standard deviation (2SD), and developed in 356 healthy persons as normal controls. Four hundred and sixteen patients with chronic viral hepatitis were tested as negative controls to assess the diagnostic validity of this test battery.RESULTS: There was no significant difference in NCT scores and SDT quotients between healthy controls and chronic hepatitis group (P>0.05). In all age subgroups,the NCT and SDT measurements of cirrhotic patients differed significantly from those of the controls (P<0.05).When mean±2SD of SDT and NCT measurements from healthy control group was set as the normal range, 119cirrhotic patients (29.1%) were found to have abnormal NCT-A and SDT tests, 53 (13.0%) were abnormal only in SDT and 36 (8.8%) only in NCT-A. Taken together, SHE was diagnosed in 208 (50.9%) cirrhotic patients by this test battery. The prevalence of SHE increased from 39.9%and 55.2% in Child-Pugh's grade A and B groups to 71.8%in Child-Pugh's grade C group (P<0.05). After the adjustment of age and residential areas required from the tests, no correlation was found in the rate of SHE and causes of cirrhosis, education level and smoking habit.CONCLUSION: Psychometric tests are simple and reliable indicators for screening SHE among Chinese cirrhotic patients. By using a NCT and SDT battery, SHE could be found in 50.9% of cirrhotic patients without overt clinical encephalopathy. The

  9. Association of Bovine Viral Diarrhea Virus with Multiple Viral Infections in Bovine Respiratory Disease Outbreaks

    OpenAIRE

    Richer, Lisette; Marois, Paul; Lamontagne, Lucie

    1988-01-01

    We investigated eleven outbreaks of naturally occurring bovine respiratory diseases in calves and adult animals in the St-Hyacinthe area of Quebec. Specific antibodies to bovine herpesvirus-1, bovine viral diarrhea virus, respiratory syncytial virus, parainfluenza type 3 virus, reovirus type 3, and serotypes 1 to 7 of bovine adenovirus were found in paired sera from diseased animals. Several bovine viruses with respiratory tropism were involved concomitantly in herds during an outbreak of bov...

  10. Historical overview of prion diseases: a view from afar.

    Science.gov (United States)

    Liberski, Pawel P

    2012-01-01

    The transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of neurodegenerative disorders which include kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia in men, natural scrapie in sheep, goats and mufflons, transmissible mink encephalopathy in ranch-reared mink, chronic wasting disease of mule deer and elk, bovine spongiform encephalopathy or "mad cow disease" and its analogues in several exotic species of antelopes and wild felids in zoological gardens, and feline spongiform encephalopathy in domestic cats. This short review summarizes the history of the research to find the nature of the scrapie agent, especially as I have witnessed it unfolding before my eyes. I review the historical background of TSEs starting from the first description of scrapie in 1732. In 1957, the first prion disease in humans, kuru was described and its transmissibility was demonstrated in 1965 by seminal work of Gajdusek, Gibbs and colleagues, followed by transmission of CJD and then, GSS. In 1982, Stanley B. Prusiner formulated "prion hypothesis" which has dominated the field for the last 30 years. This theory had been recently extended to cover other neurodegenerations which are caused by misfolded proteins; these disease are called prionoids. PMID:22505359

  11. Posterior reversible encephalopathy syndrome: An atypical postpartum complication

    Directory of Open Access Journals (Sweden)

    Debashish Paul

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema. Data suggest that most cases occur in young middle-aged with marked female preponderance, hypertension being the most common cause. In this case, it was diagnosed in a normotensive patient in the postnatal period that underwent cesarean section. The initial symptoms had misled toward a diagnosis of postdural puncture headache. Symptomatic treatment was started immediately in the ICU. This is an interesting case as the patient was a normotensive one without any other contributory factors and there was unanticipated delay in diagnosing the case until the time we could get a magnetic resonance imaging report.

  12. Severe hypoglycemic encephalopathy due to hypoallergenic formula in an infant.

    Science.gov (United States)

    Ogawa, Erika; Ishige, Mika; Takahashi, Yuno; Kodama, Hiroko; Fuchigami, Tatsuo; Takahashi, Shori

    2016-08-01

    A 7-month-old girl was brought to hospital due to vomiting. Upon admission, she was in a convulsive state and stupor with extremely low blood glucose. Head computed tomography showed brain edema, and comprehensive treatment for acute encephalopathy was initiated immediately. Severe hypoglycemia, metabolic acidosis, elevation of ammonia and serum transaminases and creatine kinase suggested metabolic decompensation. Infusion of a high-glucose solution containing vitamins, biotin, and l-carnitine resolved the metabolic crisis quickly, but brain damage was irreversible. She was found to have been fed exclusively on a hypoallergenic formula (HF) for 7 months, although she was found later to be non-allergic. Evidence of inborn metabolic diseases was absent, therefore biotin deficiency and carnitine deficiency were concluded to be a consequence of reliance on a HF for a prolonged period. Health-care professionals should warn parents of the consequences of using HF. PMID:27324861

  13. Bruxism Associated with Anoxic Encephalopathy: Successful Treatment with Baclofen

    Directory of Open Access Journals (Sweden)

    A. Bruce Janati

    2013-01-01

    Full Text Available Introduction. Bruxism is a movement disorder characterized by grinding and clenching of the teeth. Etiology of bruxism can be divided into three groups: psychosocial factors, peripheral factors, and pathophysiological factors. Methods. The clinical investigation was conducted at King Khaled Hospital in Hail, Saudi Arabia, in 2012. Results. A 16-year-old Saudi female was brought to the hospital in a comatose state and with generalized convulsive seizures secondary to acute anoxic encephalopathy. In the third week of hospitalization, while still in a state of akinetic mutism, she developed incessant bruxism which responded favorably to a GABA receptor agonist (baclofen. Conclusion. Our data support the hypothesis that bruxism emanates from imbalance or dysregulation of the neurotransmitter system. Larger scale studies will be needed to confirm this hypothesis.

  14. Mapping Metabolic Brain Activity in Three Models of Hepatic Encephalopathy

    Directory of Open Access Journals (Sweden)

    Natalia Arias

    2013-01-01

    Full Text Available Cirrhosis is a common disease in Western countries. Liver failure, hyperammonemia, and portal hypertension are the main factors that contribute to human cirrhosis that frequently leads to a neuropsychiatric disorder known as hepatic encephalopathy (HE. In this study, we examined the differential contribution of these leading factors to the oxidative metabolism of diverse brain limbic system regions frequently involved in memory process by histochemical labelling of cytochrome oxidase (COx. We have analyzed cortical structures such as the infralimbic and prelimbic cotices, subcortical structures such as hippocampus and ventral striatum, at thalamic level like the anterodorsal, anteroventral, and mediodorsal thalamus, and, finally, the hypothalamus, where the mammillary nuclei (medial and lateral were measured. The severest alteration is found in the model that mimics intoxication by ammonia, followed by the thioacetamide-treated group and the portal hypertension group. No changes were found at the mammillary bodies for any of the experimental groups.

  15. Chronic Traumatic Encephalopathy: The Neuropathological Legacy of Traumatic Brain Injury.

    Science.gov (United States)

    Hay, Jennifer; Johnson, Victoria E; Smith, Douglas H; Stewart, William

    2016-05-23

    Almost a century ago, the first clinical account of the punch-drunk syndrome emerged, describing chronic neurological and neuropsychiatric sequelae occurring in former boxers. Thereafter, throughout the twentieth century, further reports added to our understanding of the neuropathological consequences of a career in boxing, leading to descriptions of a distinct neurodegenerative pathology, termed dementia pugilistica. During the past decade, growing recognition of this pathology in autopsy studies of nonboxers who were exposed to repetitive, mild traumatic brain injury, or to a single, moderate or severe traumatic brain injury, has led to an awareness that it is exposure to traumatic brain injury that carries with it a risk of this neurodegenerative disease, not the sport or the circumstance in which the injury is sustained. Furthermore, the neuropathology of the neurodegeneration that occurs after traumatic brain injury, now termed chronic traumatic encephalopathy, is acknowledged as being a complex, mixed, but distinctive pathology, the detail of which is reviewed in this article. PMID:26772317

  16. Posterior Reversible Encephalopathy Syndrome (PRES After Acute Pancreatitis

    Directory of Open Access Journals (Sweden)

    Tara Murphy

    2015-12-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is an unusual condition typified by acute visual impairment caused by sudden, marked parieto-occipital vasogenic edema. Thought to be inflammatory in origin, it has been described in patients undergoing chemotherapy, with autoimmune disease, and in some infections. We report a case of PRES that occurred one week after an episode of acute pancreatitis in an otherwise healthy 40-year-old female. There was progressive visual impairment over a 24-hour period with almost complete visual loss, with characteristic findings on magnetic resonance imaging. After treatment with steroids, the visual loss recovered. Clinicians should retain an index of suspicion of this rare condition in patients with visual impairment after acute pancreatitis.

  17. Quantitative EEG evaluation in patients with acute encephalopathy

    Directory of Open Access Journals (Sweden)

    Aline Souza Marques da Silva Braga

    2013-12-01

    Full Text Available Objective To investigate the use of quantitative EEG (qEEG in patients with acute encephalopathies (AEs and EEG background abnormalities. Method Patients were divided into favorable outcome (group A, 43 patients and an unfavorable outcome (group B, 5 patients. EEGLAB software was used for the qEEG analysis. A graphic of the spectral power from all channels was generated for each participant. Statistical comparisons between the groups were performed. Results In group A, spectral analysis revealed spectral peaks (theta and alpha frequency bands in 84% (38/45 of the patients. In group B, a spectral peak in the delta frequency range was detected in one patient. The remainder of the patients in both groups did not present spectral peaks. Statistical analysis showed lower frequencies recorded from the posterior electrodes in group B patients. Conclusion qEEG may be useful in the evaluations of patients with AEs by assisting with the prognostic determination.

  18. A Case of Chronic Wernicke's Encephalopathy: A Neuropsychological Study.

    Science.gov (United States)

    Oudman, Erik; Van der Stigchel, Stefan; Postma, Albert; Wijnia, Jan W; Nijboer, Tanja C W

    2014-01-01

    A 54-year-old woman was referred to our Korsakoff Center because of extensive cognitive problems following acute Wernicke's encephalopathy (WE). She had a relatively short history of alcohol abuse and was found lying on the floor in her home by her son. After 5 days without treatment, she was diagnosed with WE in a general hospital. During the course of the disease, minimal change to the acute situation occurred, with chronic confusion, attention deficits, and incoherent behavior symptoms most notable unlike classical Korsakoff's syndrome. Neuropsychological assessment after 4 and 16 months after admission to the hospital revealed global cognitive decline, with striking impairments in attentional, executive, and memory functions. The present case study suggests that the state of confusion and the neuropsychological symptoms in WE can become chronic in case of very late treatment. We therefore recommend that confused alcoholics should receive appropriate parenteral thiamine according to the current clinical standards. PMID:24904442

  19. Contemporary Understanding and Management of Overt and Covert Hepatic Encephalopathy.

    Science.gov (United States)

    NeSmith, Meghan; Ahn, Joseph; Flamm, Steven L

    2016-02-01

    Hepatic encephalopathy (HE) is a major complication of liver disease that leads to significant morbidity and mortality. Caring for hospitalized patients with HE is becoming more complex, and the economic burden of HE continues to rise. Defining and diagnosing HE, particularly covert HE (CHE), remain challenging. In this article, we review new tools and those currently under development for the diagnosis of CHE and the latest advances in the acute and long-term management of overt HE (OHE) and CHE. In particular, we review the latest data on the use of lactulose and rifaximin for treatment of OHE and summarize the data on adjunctive agents such as sodium benzoate and probiotics. PMID:27182210

  20. Hepatic encephalopathy: effects of liver failure on brain function.

    Science.gov (United States)

    Felipo, Vicente

    2013-12-01

    Liver failure affects brain function, leading to neurological and psychiatric alterations; such alterations are referred to as hepatic encephalopathy (HE). Early diagnosis of minimal HE reveals an unexpectedly high incidence of mild cognitive impairment and psychomotor slowing in patients with liver cirrhosis - conditions that have serious health, social and economic consequences. The mechanisms responsible for the neurological alterations in HE are beginning to emerge. New therapeutic strategies acting on specific targets in the brain (phosphodiesterase 5, type A GABA receptors, cyclooxygenase and mitogen-activated protein kinase p38) have been shown to restore cognitive and motor function in animal models of chronic HE, and NMDA receptor antagonists have been shown to increase survival in acute liver failure. This article reviews the latest studies aimed at understanding how liver failure affects brain function and potential ways to ameliorate these effects. PMID:24149188

  1. Cyclosporin-A induced Posterior Reversible Encephalopathy Syndrome

    Directory of Open Access Journals (Sweden)

    Saeed Bassam

    2008-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a recently proposed clinico-neuroradiological entity observed in a variety of clinical settings such as cyclosporin A (CsA neurotoxicity. We report a 3.5-year-old Syrian boy in whom steroid-resistant focal segmental glomerulosclerosis (FSGS was recently diagnosed. The patient remitted his nephrotic syndrome after 10 days of CsA administration. However, he shortly developed altered mental status, visual impairment, focal neurological deficits and seizures. We discontinued CsA that resulted in complete reversal of the patient′s encephalopathical condition over a period of 4 months. We conclude that PRES should be suspected in immunosuppresed patients with kidney disease if they have a sudden episode of neurological symptoms.

  2. Hashimoto encephalopathy: a case report with proton MR spectroscopic findings

    Institute of Scientific and Technical Information of China (English)

    SU Tian-hao; JIN Er-hu; HE Wen

    2011-01-01

    A 52-year-old female patient with Hashimoto encephalopathy was admitted to hospital for clinical treatment,and the findings on MR spectroscopy (MRS) and MR imaging (MRI) in the brain were reported.MRS revealed the decreases in N-acetylaspartate (NAA/Cr=1.19) and myo-inositol peaks,and the elevations in lipid,lactate,glutamate/glutamine multiplet and choline (Cho/Cr=1.21) peaks which supported a cerebral inflammatory change,in addition to multifocal hyperintensities on T2WI and fluid-attenuated inversion recovery (FLAIR) images,slight hyperintensities on diffusion weighted imaging (DWI),hypointensities on T1WI.The atrophy of the brain was revealed on follow-up MRI two years later.

  3. CT diagnosis and clinical findings of subcortical arteriosclerotic encephalopathy

    International Nuclear Information System (INIS)

    Objective: A study of the pathology, clinical characteristics and major CT manifestations, in order to obtain a better understanding of the SAE. Methods: In total 94 cases of SAE were enrolled in the study in Jun-an hospital, included 58 males and 36 females, aged from 62-92 years (averagely 77 years). The clinical presentations of SAE included hyper- tension, diabetics, arteriosclerotisc eyeground, and stupor, characterized by progressive decline of intellectual function, different-level of paralysis, aphasia, swirl, dazzle, naupathia, extremity numbness. Results: A predominance was found in the patients aged more than 60 years. CT scan showed symmetrical hypodense shadows with poorly-defined border, complicated with the sign of lacunar infarction, cerebral atrophy, parenchymal hemorrhage and/or subarachnoid hemorrhage in some cases. Conclusion: A good understanding of the clinical and CT features of subcortical arteriosclerotic encephalopathy is valuable for CT diagnosis and differentiated diagnosis. (authors)

  4. Oxidative metabolism of astrocytes is not reduced in hepatic encephalopathy

    DEFF Research Database (Denmark)

    Iversen, Peter; Mouridsen, Kim; Hansen, Mikkel Bo;

    2014-01-01

    In patients with impaired liver function and hepatic encephalopathy (HE), consistent elevations of blood ammonia concentration suggest a crucial role in the pathogenesis of HE. Ammonia and acetate are metabolized in brain both primarily in astrocytes. Here, we used dynamic [(11)C]acetate PET of the...... brain to measure the contribution of astrocytes to the previously observed reduction of brain oxidative metabolism in patients with liver cirrhosis and HE, compared to patients with cirrhosis without HE, and to healthy subjects. We used a new kinetic model to estimate uptake from blood to astrocytes and...... astrocyte metabolism of [(11)C]acetate. No significant differences of the rate constant of oxidation of [(11)C]acetate (k 3) were found among the three groups of subjects. The net metabolic clearance of [(11)C]acetate from blood was lower in the group of patients with cirrhosis and HE than in the group of...

  5. Encephalopathy with status epilepticus during sleep (ESES) induced by oxcarbazepine in idiopathic focal epilepsy in childhood

    DEFF Research Database (Denmark)

    Pavlidis, Elena; Rubboli, Guido; Nikanorova, Marina;

    2015-01-01

    Encephalopathy with status epilepticus during sleep (ESES) is an age-related disorder characterized by neuropsychological regression, epilepsy and a typical EEG pattern of continuous epileptiform activity (> 85%) during NREM sleep. Cases of worsening or induction of ESES with phenytoin...

  6. Rifaximin vs conventional oral therapy for hepatic encephalopathy: A meta-analysis

    OpenAIRE

    2012-01-01

    AIM: To characterize the efficacy of rifaximin in the management of hepatic encephalopathy (HE) as several randomized controlled studies have shown contradictory results on its effectiveness in comparison to other oral agents.

  7. Valproate-induced hyperammonemic encephalopathy enhanced by topiramate and phenobarbitone: A case report and an update

    Directory of Open Access Journals (Sweden)

    Vivekanandan S

    2010-01-01

    Full Text Available Although sodium valproate (VPA-induced hepatic encephalopathy is a well-recognized entity, VPA can occasionally produce encephalopathy secondary to hyperammonemia in the presence of normal hepatic function, namely valproate-induced non-hepatic hyperammonemic encephalopathy (VNHE. Known risk factors include therapy with multiple antiepileptic drugs, especially when topiramate is one of the drugs; presence of underlying inborn errors of metabolism; febrile states; and insufficient nutritional intake. We describe a 5-year-old male child who developed VNHE while on polypharmacy with topiramate and phenobarbitone; the child also had poor nutritional intake. The encephalopathy reversed with withdrawal of VPA and treatment with L-carnitine. We emphasize the need for early recognition, investigation, and treatment of this potentially life-threatening condition. We also recommend that VPA, topiramate, and phenobarbitone should not be given in combination.

  8. A case of recurrent acute encephalopathy with febrile convulsive status epilepticus with carnitine palmitoyltransferase II variation.

    Science.gov (United States)

    Sakai, Eiko; Yamanaka, Gaku; Kawashima, Hisashi; Morishima, Yasuyuki; Ishida, Yu; Oana, Shingo; Miyajima, Tasuku; Shinohara, Mayu; Saitoh, Makiko; Mizuguchi, Masashi

    2013-08-01

    Acute encephalopathy with febrile convulsive status epilepticus (AEFCSE) is the most common type of acute encephalopathy in childhood in Japan, which develops with prolonged febrile convulsion, followed by mild unconsciousness. It is generally sporadic and nonrecurrent. In this report, a 1-year-old girl showed signs of AEFCSE triggered by respiratory syncytial virus infection. Two years later, she presented with AEFCSE triggered by influenza virus infection, resulting in severe neurologic sequelae. The patient had a thermolabile genotype of carnitine palmitoyltransferase II (CPT II) variations consisting of three single nucleotide polymorphisms in exons 4 [1055T > G/F352C and 1102G > A/V368I] and 5 [1939A > G/M647V]. The polymorphism has been identified as a genetic predisposition for acute encephalopathy. This report presents the first case of recurrent encephalopathy with CPT II variations that may partially associate with pathogenesis of recurrent AEFCSE. PMID:23450341

  9. Lactulose enhances neuroplasticity to improve cognitive function in early hepatic encephalopathy

    Institute of Scientific and Technical Information of China (English)

    Nan Yang; He Liu; Yao Jiang; Ji Zheng; Dong-mei Li; Chao Ji; Yan-yong Liu; Ping-ping Zuo

    2015-01-01

    Lactulose is known to improve cognitive function in patients with early hepatic encephalopa-thy; however, the underlying mechanism remains poorly understood. In the present study, we investigated the behavioral and neurochemical effects of lactulose in a rat model of early hepatic encephalopathy induced by carbon tetrachloride. Immunohistochemistry showed that lactulose treatment promoted neurogenesis and increased the number of neurons and astrocytes in the hippocampus. Moreover, lactulose-treated rats showed shorter escape latencies than model rats in the Morris water maze, indicating that lactulose improved the cognitive impairments caused by hepatic encephalopathy. The present ifndings suggest that lactulose effectively improves cog-nitive function by enhancing neuroplasticity in a rat model of early hepatic encephalopathy.

  10. De novo loss- or gain-of-function mutations in KCNA2 cause epileptic encephalopathy

    DEFF Research Database (Denmark)

    Syrbe, Steffen; Hedrich, Ulrike B S; Riesch, Erik;

    2015-01-01

    Epileptic encephalopathies are a phenotypically and genetically heterogeneous group of severe epilepsies accompanied by intellectual disability and other neurodevelopmental features. Using next-generation sequencing, we identified four different de novo mutations in KCNA2, encoding the potassium ...

  11. Prognostic Value of Cytochrome C and Cytokines in Acute Viral Encephalopathy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-06-01

    Full Text Available Serum cytochrome c and cytokines were evaluated as prognostic predictors in 29 children (ages 9 mos to 9 yrs 11 mos with viral acute encephalopathies and multiple organ failure at Fukushima Medical University School of Medicine, Japan.

  12. Recurrent encephalopathy? No I’m a sleeping beauty!

    Science.gov (United States)

    Iqbal, Mehtab; Prasad, Manish; Ritey, Christopher

    2014-01-01

    To describe the clinical presentation of ‘Kleine-Levin (sleeping beauty) syndrome’ in a child, who presented with recurrent episodes consistent with encephalopathy, associated with excessive sleepiness, cognitive and behavioural disturbance and hyper sexuality. 14 years old boy presented acutely with excessive tiredness, sleeping excessively, abnormal behaviour and hypersexuality following a viral throat infection. On examination he was sleepy but easily arousable. His GCS (15/15) and rest of the neurological examination including fundoscopy and other systemic examination was completely unremarkable. All his initial investigations including electrolytes, LFTs, CSF, virology screen and MRI brain scan were normal. Detailed autoimmune screening was also negative. EEG showed non-specific diffuse slowing consistent with encephalopathy. His excessive sleepiness gradually improved together with his altered behaviour in about two weeks after presentation. Hyper sexuality became more overt during this phase. All these symptoms completely disappeared three weeks after his presentation and he attended school as before. He was readmitted six weeks later with exactly similar presentation and again only positive result being diffuse non-specific slowing of EEG. His recovery was also similar and he was completely back to his normal self in three weeks time. His recurrent symptoms were consistent with ‘Kleine-Levin syndrome (KLS)’ or ‘sleeping beauty syndrome’. KLS is a rare disorder which mainly affects adolescent males. Common symptoms include hypersomnia (100%), cognitive changes (96%), eating disturbances (80%), hypersexuality, compulsions, and depressed mood. The syndrome usually lasts for 8 years, with on an average seven episode of 10 days each recurring every 3.5 months. It is most frequently precipitated by infections and somnolence decreases using stimulants in nearly 40% of cases. PMID:24891916

  13. Recurrent encephalopathy? No I'm a sleeping beauty!

    Science.gov (United States)

    Iqbal, Mehtab; Prasad, Manish; Ritey, Christopher

    2014-01-01

    To describe the clinical presentation of 'Kleine-Levin (sleeping beauty) syndrome' in a child, who presented with recurrent episodes consistent with encephalopathy, associated with excessive sleepiness, cognitive and behavioural disturbance and hyper sexuality. 14 years old boy presented acutely with excessive tiredness, sleeping excessively, abnormal behaviour and hypersexuality following a viral throat infection. On examination he was sleepy but easily arousable. His GCS (15/15) and rest of the neurological examination including fundoscopy and other systemic examination was completely unremarkable. All his initial investigations including electrolytes, LFTs, CSF, virology screen and MRI brain scan were normal. Detailed autoimmune screening was also negative. EEG showed non-specific diffuse slowing consistent with encephalopathy. His excessive sleepiness gradually improved together with his altered behaviour in about two weeks after presentation. Hyper sexuality became more overt during this phase. All these symptoms completely disappeared three weeks after his presentation and he attended school as before. He was readmitted six weeks later with exactly similar presentation and again only positive result being diffuse non-specific slowing of EEG. His recovery was also similar and he was completely back to his normal self in three weeks time. His recurrent symptoms were consistent with 'Kleine-Levin syndrome (KLS)' or 'sleeping beauty syndrome'. KLS is a rare disorder which mainly affects adolescent males. Common symptoms include hypersomnia (100%), cognitive changes (96%), eating disturbances (80%), hypersexuality, compulsions, and depressed mood. The syndrome usually lasts for 8 years, with on an average seven episode of 10 days each recurring every 3.5 months. It is most frequently precipitated by infections and somnolence decreases using stimulants in nearly 40% of cases. PMID:24891916

  14. Recurrent encephalopathy? No I′m a sleeping beauty!

    Directory of Open Access Journals (Sweden)

    Mehtab Iqbal

    2014-01-01

    Full Text Available To describe the clinical presentation of ′Kleine-Levin (sleeping beauty syndrome′ in a child, who presented with recurrent episodes consistent with encephalopathy, associated with excessive sleepiness, cognitive and behavioural disturbance and hyper sexuality. 14 years old boy presented acutely with excessive tiredness, sleeping excessively, abnormal behaviour and hypersexuality following a viral throat infection. On examination he was sleepy but easily arousable. His GCS (15/15 and rest of the neurological examination including fundoscopy and other systemic examination was completely unremarkable. All his initial investigations including electrolytes, LFTs, CSF, virology screen and MRI brain scan were normal. Detailed autoimmune screening was also negative. EEG showed non-specific diffuse slowing consistent with encephalopathy. His excessive sleepiness gradually improved together with his altered behaviour in about two weeks after presentation. Hyper sexuality became more overt during this phase. All these symptoms completely disappeared three weeks after his presentation and he attended school as before. He was readmitted six weeks later with exactly similar presentation and again only positive result being diffuse non-specific slowing of EEG. His recovery was also similar and he was completely back to his normal self in three weeks time. His recurrent symptoms were consistent with ′Kleine-Levin syndrome (KLS′ or ′sleeping beauty syndrome′. KLS is a rare disorder which mainly affects adolescent males. Common symptoms include hypersomnia (100%, cognitive changes (96%, eating disturbances (80%, hypersexuality, compulsions, and depressed mood. The syndrome usually lasts for 8 years, with on an average seven episode of 10 days each recurring every 3.5 months. It is most frequently precipitated by infections and somnolence decreases using stimulants in nearly 40% of cases.

  15. Hypertensive Encephalopathy: Isolated Pons Involvement Mimicking Central Pontine Myelinolysis

    International Nuclear Information System (INIS)

    MRI of the brain was performed, and it demonstrated an isolated high signal on the T2 weighted and fluid attenuated inversion recovery sequences that involved only the central pons with sparing the periphery. There was no restricted diffusion on diffusion weighted imaging. The differential diagnosis included posterior reversible syndrome and central pontine myelinolysis; however, the blood sodium on admission was normal. The pathogenesis of HE is that the auto-regulatory mechanisms that control the cerebral blood flow are exceeded, resulting in hyper-perfusion. The consequent over-distension of the cerebral vessels, the breakdown of the blood brain barrier and ultimately, the extravasation of fluid into the interstitium all cause vasogenic edema. In most cases, the changes of hypertensive encephalopathy represent reversible vasogenic edema, which can be seen on T2-weighted images, and restricted diffusion is not seen on the diffusion-weighted imaging (DWI) and the apparent diffusion coefficient (ADC) maps. Hypertensive encephalopathy that manifests as a reversible increased signal isolated to the pons on T2-weighted images is extremely uncommon. The differential diagnosis for such pontine T2 hyperintensity includes pontine glioma, ischemic and radiation changes (generally irreversible conditions), as well as central pontine myelinolysis (CPM) and demyelinating disorders such as multiple sclerosis, acute disseminated encephalomyelitis and rhomb-encephalitis. In CPM electrolyte imbalances provide a clue for the diagnosis, where as for glioma, there will be an expansion and mass effect. In conclusion, clinical recognition of brainstem HE may be difficult. The features of a lack of correlation between the severity of the radiological abnormality and the clinical status, combined with the rapid resolution following antihypertensive treatment, should suggest the diagnosis. It is important for the radiologist to be familiar with the imaging abnormalities of this life

  16. Prion remains infectious after passage through digestive system of American crows (Corvus brachyrhynchos.

    Directory of Open Access Journals (Sweden)

    Kurt C VerCauteren

    Full Text Available Avian scavengers, such as American crows (Corvus brachyrhynchos, have potential to translocate infectious agents (prions of transmissible spongiform encephalopathy (TSE diseases including chronic wasting disease, scrapie, and bovine spongiform encephalopathy. We inoculated mice with fecal extracts obtained from 20 American crows that were force-fed material infected with RML-strain scrapie prions. These mice all evinced severe neurological dysfunction 196-231 d postinoculation (x =198; 95% CI: 210-216 and tested positive for prion disease. Our results suggest a large proportion of crows that consume prion-positive tissue are capable of passing infectious prions in their feces (ˆp=1.0; 95% CI: 0.8-1.0. Therefore, this common, migratory North American scavenger could play a role in the geographic spread of TSE diseases.

  17. Uremic Encephalopathy with Atypical Magnetic Resonance Features on Diffusion-Weighted Images

    OpenAIRE

    Kang, Eugene; Jeon, Se Jeong; Choi, See-Sung

    2012-01-01

    Uremic encephalopathy is a well-known disease with typical MR findings including bilateral vasogenic or cytotoxic edema at the cerebral cortex or basal ganglia. Involvement of the basal ganglia has been very rarely reported, typically occurring in uremic-diabetic patients. We recently treated a patient who had non-diabetic uremic encephalopathy with an atypical lesion distribution involving the supratentorial white matter, without cortical or basal ganglia involvement. To the best of our know...

  18. Wernicke’s Encephalopathy in a Patient with Nasopharyngeal Carcinoma: Magnetic Resonance Imaging Findings

    OpenAIRE

    Law, Huong Ling; Tan, Suzet; Sedi, Rosleena

    2011-01-01

    We report a case of Wernicke’s encephalopathy in a patient with nasopharyngeal carcinoma with a 3-month history of poor oral intake related to nausea and vomiting due to chemotherapy. The patient later developed deep coma while receiving in-patient therapy. Magnetic resonance imaging of the brain revealed typical findings of Wernicke’s encephalopathy. The patient was treated with thiamine injections, which resulted in subsequent partial recovery of neurological function. This paper stresses t...

  19. Comparison of Rifaximin and Lactulose for the Treatment of Hepatic Encephalopathy: A Prospective Randomized Study

    OpenAIRE

    Paik, Yong-Han; Lee, Kwan Sik; Han, Kwang-Hyub; Song, Kun Hoon; Kim, Myoung Hwan; Moon, Byung Soo; Ahn, Sang Hoon; Lee, Se Joon; Park, Hyo Jin; Lee, Dong Ki; Chon, Chae Yoon; Lee, Sang In; Moon, Young Myoung

    2005-01-01

    Rifaximin has been reported to be effective for the treatment of hepatic encephalopathy (HE) in Europe. However, it is unknown whether Rifaximin is effective for the treatment of HE in Koreans, therefore we conducted a open-label prospective randomized study to evaluate the efficacy of rifaximin versus lactulose in Korean patients. Fifty-four patients with liver cirrhosis and hepatic encephalopathy were enrolled. Thirty-two patients were randomized to receive rifaximin and 22 to receive lactu...

  20. Prodominant hypertensive brainstem encephalopathy with supratentorial involvement: Case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hee; Park, Sung Tae; Lim, Hyun Kyung [Dept. of Radiology, Soonchunhyang University Hospital, Soonchunhyang University School of Medicine, Seoul (Korea, Republic of); Kim, Sung Tae; Cha, Ji Hoon [Dept. of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2014-12-15

    Hypertensive encephalopathy typically presents with bilateral parietooccipital vasogenic edema. Brainstem and cerebellar edema are uncommon in association with typical supratentorial changes. We experienced three cases of atypical hypertensive encephalopathy involving brainstem and cerebellum as well as cerebral white matter, which showed characteristic alternating linear bright and low signals in the pons, the so-called 'stripe sign'. We report these cases here with a brief literature review.