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  1. Creutzfeldt-Jakob Disease

    Science.gov (United States)

    ... National Institute of Neurological Disorders and Stroke (NINDS). Enfermedad de Creutzfeldt-Jakob Dementia: Hope Through Research Information booklet about Alzheimer's disease, vascular dementia, and other types of dementia ...

  2. Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    LIU Jian-rong

    2013-01-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a degenerative central nervous system (CNS disease caused by infection of prion protein (PrP, with clinical features including short course, rapid development and 100% mortality. This article aims to discuss the pathogenesis, histopathological features, clinical manifestations, electroencephalogram (EEG findings, imaging data and treatment progress of this disease based on literature review. Cerebrospinal fluid 14-3-3 protein detection, EEG and MRI are three important methods to make an early diagnosis on patients with suspected CJD, such as elderly patients with rapidly progressive dementia (RPD and young patients with mental symptoms involving multiple systems (MS.

  3. Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Iwasaki, Yasushi

    2017-04-01

    This review will explore the clinical and pathological findings of the various forms of Creutzfeldt-Jakob disease (CJD). Clinical findings of CJD are characterized by rapidly progressive cognitive dysfunction, diffusion-weighted magnetic resonance imaging (DWI) hyperintensity, myoclonus, periodic sharp-wave complexes on electroencephalogram and akinetic mutism state. Neuropathologic findings of CJD are characterized by spongiform changes in gray matter, gliosis-particularly hypertrophic astrocytosis-neuropil rarefaction, neuron loss and prion protein (PrP) deposition. The earliest pathological symptom observed by HE staining in the cerebral cortex is spongiform change. This spongiform change begins several months before clinical onset, and is followed by gliosis. Subsequently, neuropil rarefaction appears, followed by neuron loss. Regions showing fine vacuole-type spongiform change reflect synaptic-type PrP deposition and type 1 PrP(Sc) deposition, whereas regions showing large confluent vacuole-type spongiform changes reflect perivacuolar-type PrP deposition and type 2 PrP(Sc) deposition. Hyperintensities of the cerebral gray matter observed in DWI indicate the pathology of the spongiform change in CJD. The cerebral cortical lesions with large confluent vacuoles and type 2 PrP(Sc) show higher brightness and more continuous hyperintensity on DWI than those with fine vacuoles and type 1 PrP(Sc) . CJD cases showing diffuse myelin pallor of cerebral white matter have been described as panencephalopathic-type, and this white matter pathology is mainly due to secondary degeneration caused by cerebral cortical involvement, particularly in regard to neuron loss. In conclusion, clinical and neuroimaging findings and neuropathologic observations are well matched in both typical and atypical cases in CJD. The clinical diagnosis of CJD is relatively easy for typical CJD cases such as the MM1-type. However, even in atypical cases it seems that clinical findings can be used for

  4. Variant Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    E.A. Croes (Esther); C.M. van Duijn (Cock)

    2003-01-01

    textabstractA variant form of Creutzfeldt-Jakob disease (vCJD) has had major impact in Europe during the last decade. In this article, we review the aetiology of vCJD and its relation with bovine spongiform encephalopathy. Further, treatment of the disease, the strategies focusing on prevention of t

  5. CREUTZFELDT - JAKOB DISEASE : A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Theophilus Premkumar

    2015-06-01

    Full Text Available Prion diseases are a group of fatal neurodegenerative diseases caused by the trans - formation of an endogenous protein, PrP (prion - related protein, into an abnormal conformation, the most common of which in humans is Creutzfeldt - Jakob disease. We report t he case of a 40 year old lady who presented with rapidly progressive dementia with pyramidal, extra - pyramidal, cerebellar symptoms, myoclonus and akinetic mutism. Her EEG showed typical periodic sharp wave complexes and MRI Brain revealed DWI>FLAIR intensi ty in bilateral caudate nuclei, putamen & bilateral subcortical frontal lobes . The clinico - radiological correlation was consistent with the diagnosis of Creutzfeldt - Jakob disease.

  6. Radiological assessment of Creutzfeldt-Jakob disease

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    Tschampa, Henriette J.; Urbach, Horst [University of Bonn, Department of Radiology, Bonn (Germany); Zerr, Inga [University of Goettingen, National Reference Center for TSE Surveillance at the Department of Neurology, Goettingen (Germany)

    2007-05-15

    Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The ''pulvinar sign'', defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD. (orig.)

  7. Sleep Pathology in Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Kang, Peter; de Bruin, Gabriela S.; Wang, Leo H.; Ward, Beth A.; Ances, Beau M.; Lim, Miranda M.; Bucelli, Robert C.

    2016-01-01

    Study Objectives: Associations between sleep and neurodegenerative diseases have become increasingly evident. This study aims to characterize the prevalence and type of sleep pathology in Creutzfeldt-Jakob disease (CJD), a rapidly progressive, fatal neurodegenerative disease. Methods: In this observational cross-sectional cohort study, we performed a retrospective analysis of sleep signs and symptoms in a consecutive group of patients with definite CJD at a tertiary care medical center (n = 28). Polysomnography was performed in 14 patients. Results: Although only 5 of 28 patients carried a premorbid sleep diagnosis, signs/symptoms of sleep pathology were present in 25 patients. Eleven reported hypersomnia whereas 13 reported insomnia. Seven had restless legs symptoms and/or periodic limb movements of sleep, and nine reported parasomnias. Of the 14 patients who underwent polysomnography, 1 did not show sleep, 9 (69%) had poorly formed or absent sleep spindles and/or K-complexes, and 10 (77%) had sleep-disordered breathing. Of the 8 patients who experienced rapid eye movement (REM) sleep during the polysomnography, 3 (38%) showed REM sleep without atonia, and 2 patients met criteria for REM sleep behavior disorder. Median total sleep time was 226 (interquartile range [IQR] = 195–282) min. Median sleep efficiency was 58.5% (IQR = 41–65.5 %). Median REM time was 0.35% (IQR = 0–7.125%). Five patients (38%) demonstrated periodic limb movements during polysomnography. One case is presented. Conclusions: Sleep pathology is common in CJD, and screening for sleep pathology is indicated in the evaluation of patients with rapidly progressive dementias. Early identification and treatment of sleep pathology may provide an intervenable target for CJD. Citation: Kang P, de Bruin GS, Wang LH, Ward BA, Ances BM, Lim MM, Bucelli RC. Sleep pathology in Creutzfeldt-Jakob disease. J Clin Sleep Med 2016;12(7):1033–1039. PMID:27250807

  8. Creutzfeldt-Jakob Disease and Infection Control

    Directory of Open Access Journals (Sweden)

    Lynn Johnston

    2001-01-01

    Full Text Available Over the past year, several situations have occurred in Canada in which patients who had recently undergone a surgical procedure were subsequently diagnosed with confirmed or suspected Creutzfeldt-Jakob disease (CJD. This raised concerns over contamination of surgical instruments: which instruments might have been contaminated from direct exposure to tissues; can instruments become cross-contaminated by exposure to other contaminated instruments; what assessment is necessary to determine cross-contamination; and what should be done with instruments that have been contaminated. Additionally, should there be a patient traceback in the face of potential but unproven exposure? Unfortunately, there are no easy answers to most of the above questions. Australia, the United Kingdom and the World Health Organization have developed guidelines for the infection control management of patients with CJD, as well as instruments and devices that come into contact with them and their tissues (1-3. Health Canada's draft CJD infection control guidelines, withdrawn from the Health Canada Web site until safety concerns regarding sodium hydroxide can be addressed, closely mirrored recommendations made in those documents. The Centers for Disease Control and Prevention guidelines for CJD are under revision. However, a recent American publication made recommendations on what procedures should be used for reprocessing items that have been in contact with the prion protein (PrP (4. These recommendations differ substantially from the draft Canadian guidelines. This article reviews current knowledge about CJD, and highlights some of the infection control concerns and controversies.

  9. Sporadic Creutzfeldt-Jakob Disease (sCJD)

    Centers for Disease Control (CDC) Podcasts

    2009-02-03

    In this podcast, Dr. Lynne Sehulster discusses Creutzfeldt-Jakob disease, a rare neurodegenerative disease. This disease is caused by a pathological accumulation in the brain of an abnormal protein known as prions.  Created: 2/3/2009 by Emerging Infectious Diseases.   Date Released: 2/3/2009.

  10. Creutzfeldt-Jakob disease: the value of MRI; Creutzfeldt-Jakob-Krankheit: Stellenwert der MRT

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    Urbach, H.; Tschampa, H.J.; Keller, E.; Schild, H.H. [Radiologische Klinik, Neuroradiologie, Rheinische Friedrich-Wilhelms-Univ. Bonn (Germany); Paus, S. [Neurologische Klinik, Rheinische Friedrich-Wilhelms-Univ. Bonn (Germany)

    2001-06-01

    To define the role of MRI in the diagnosis of Creutzfeldt-Jakob disease (CJD). Methods: 14 patients with suspected CJD were studied within 3 years. MRI findings were correlated with WHO established diagnostic criteria (clinical findings, EEG, CSF with 14-3-3 protein assay). Results: 12 patients had CJD. One patient each suffered from Hashimoto's encephalitis and ALS dementia complex, respectively. Nine of 12 CJD patients had increased signal intensity of the striatum (n = 8), pulvinar thalami (n = 5) and/or cerebellar and cerebral cortex (n = 3), respectively. Signal intensity was most pronounced on FLAIR sequences; six patients were studied with diffusion-weighted MRI and showed impaired diffusion in these areas. Both patients without CJD did not show the abovementioned signal changes (sensitivity 75%, specificity and positive predictive value 100%, respectively). Conclusion: If patients with suspected CJD are studied with FLAIR and diffusion-weighted sequences, this disorder can reliably be proven or ruled out. Typical MRI findings narrow down the differential diagnosis and should be included in the WHO diagnostic criteria. (orig.) [German] Bestimmung des Stellenwerts der MRT in der Diagnostik der Creutzfeldt-Jakob-Krankheit (CJK). Methoden: Analyse der MRTs von 14 innerhalb von drei Jahren mit Verdacht auf CJK zugewiesenen Patienten. Korrelation der MRTs mit den entsprechend den WHO-Diagnosekriterien etablierten Untersuchungsverfahren (Klinik, EEG, Liquor mit 14-3-3 Protein-Nachweis). Ergebnisse: 12 Patienten hatten eine CJK, jeweils ein Patient hatte eine Hashimoto-Enzephalitis bzw. einen ALS-Demenz-Komplex. Bei 9 der 12 CJK-Patienten fanden sich beidseits Signalerhoehungen des Striatum (n = 8), des Pulvinar thalami (n = 5) und/oder des Kleinhirn- bzw. Grosshirnkortex (n = 3). Die Signalerhoehungen waren am deutlichsten auf FLAIR-Aufnahmen erkennbar; 6 mit diffusionsgewichteter MRT untersuchte Patienten wiesen eine eingeschraenkte Diffusion dieser Areale

  11. Creutzfeldt-Jakob disease presenting as bulbar palsy.

    Science.gov (United States)

    Mittal, Manoj; Hammond, Nancy; Husmann, Kathrin; Lele, Abhijit; Pasnoor, Mamatha

    2010-11-01

    Creutzfeldt-Jakob disease (CJD) is a degenerative neurological disorder caused by a prion protein. The diagnosis may be challenging in unusual early presentations. A bulbar symptom as the initial complaint is a rare presentation for CJD, with only a few reports so far. These patients can be misdiagnosed with motor neuron disease or the Miller Fisher variant of Guillain-Barré syndrome. We describe a 69-year-old woman with CJD who presented with bulbar symptoms at the onset.

  12. Diffusion-weighted magnetic resonance imaging - a new instrument in the diagnosis of Creutzfeldt-Jacob's disease; Diffusjonsvektet magnetisk resonanstomografi - nytt i diagnostikken av Creutzfeldt-Jakobs sykdom

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    Romi, Fredrik; Smivoll, Alf Inge; Moerk, Sverre; Tysnes, Ole-Bjoern

    2000-07-01

    Creutzfeldt-Jacob's disease (CID) is characterised by rapidly progressive dementia, ataxia, myoclonus and several other neurological deficits. It generally affects older adults and occurs in sporadic, genetic and iatrogenic forms. Death occurs usually within one year after onset of the disease. The diagnosis is based on clinical criteria, neuro physiological and radiological findings and confirmed by post mortal histopathology. During the last two years several cases of CID have been reported with diffusion-weighted magnetic resonance imaging (MR) abnormalities represented by increased signal intensity indicating reduced diffusion in basal ganglia and/or cortex cerebric. These abnormalities seem to be characteristic of CID. We report a case of CID in a 54 year old woman who developed vertigo, nystagmus, ataxia, myoclonus and dementia over a period of eight months. Diffusion-weighted magnetic resonance imaging showed increased signal intensity in corpus striatum and gyrus conguli. The diagnosis was post mortally confirmed with histopathology. (Author) 7 figs., 15 refs.

  13. Health professions and risk of sporadic Creutzfeldt- Jakob disease, 1965 to 2010

    NARCIS (Netherlands)

    E. Alcalde-Cabero; J. Almazán-Isla; J-P. Brandel (Jean-Philippe); M. Breithaupt; J. Catarino; S.J. Collins (Steven); J. Haybäck; R. Höftberger (Romana); E. Kahana; G.G. Kovacs (Gabor); A. Ladogana (Anna); E. Mitrová (Eva); A. Molesworth; Y. Nakamura; M. Pocchiari (Maurizio); M. Popovic; M. Ruiz-Tovar; A. Taratuto; C. van Duin; M. Yamada; R.G. Will (Robert); I. Zerr (Inga); J. de Pedro-Cuesta (Jesús)

    2012-01-01

    textabstractIn 2009, a pathologist with sporadic Creutzfeldt- Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt Jakob Disease Surveillance network

  14. Diffusion MR imaging in sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    Burcak Cakir Pekoz

    2014-08-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare dementing disease and is thought to caused by a prion. It is characterized by rapidly progressive dementia, ataxia, myoclonus, akinetic mutism and eventual death. Brain biopsy or autopsy is required for a definitive diagnosis of CJD. Diffusion-weighted imaging became an important tool for early diagnosis of CJD because of the high sensitivity. We present 59-year-old female patient diagnosed as sporadic CJD with typical MR imagings. [Cukurova Med J 2014; 39(4.000: 880-883

  15. A novel phenotype of sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Giaccone, G; Di Fede, Giuseppe; Mangieri, Michela; Limido, Lucia; Capobianco, Raffaella; Suardi, Silvia; Grisoli, Marina; Binelli, Simona; Fociani, Paolo; Bugiani, Orso; Tagliavini, Fabrizio

    2009-01-01

    An atypical case of sporadic Creutzfeldt-Jakob disease (CJD) is described in a 78-year-old woman homozygous for methionine at codon 129 of the prion protein (PrP) gene. The neuropathological signature was the presence of PrP immunoreactive plaque-like deposits in the cerebral cortex, striatum and thalamus. Western blot analysis showed a profile of the pathological form of PrP (PrP(Sc)) previously unrecognised in sporadic CJD, marked by the absence of diglycosylated protease resistant species. These features define a novel neuropathological and molecular CJD phenotype.

  16. Imaging and clinical characteristics of sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    HAN Shun-chang

    2013-04-01

    Full Text Available Five patients with sporadic Creutzfeldt-Jakob disease (sCJD presented rapidly progressive dementia which were subacute onset from 1 to 4 months. Among these cases, periodic synchronous discharge (PSD of electroencephalography (EEG was seen in 2 patients. Besides, 4 patients obtained positive results in cerebrospinal fluid (CSF analysis for 14-3-3 protein. The cranial MRI examination showed symmetrical or asymmetrical colored-ribbon-shaped high signals in cerebral cortex or basal ganglia by diffusion weighted imaging (DWI, suggesting that DWI had high sensitivity and specificity for the diagnosis of sCJD as a preferred method in the clinical examination of sCJD.

  17. Enfermedad de Creutzfeldt-Jakob: Reporte de dos casos.

    OpenAIRE

    Carla Brevis C.; Eduardo López A.; Catherine Navarrete G.

    2011-01-01

    INTRODUCCIÓN: La enfermedad de Creutzfeldt-Jakob (ECJ) es una enfermedad priónica neurodegenerativa que afecta el Sistema Nervioso Central (SNC), invariablemente mortal. Clasificada en esporádica, familiar e iatrogénica, se manifiesta por cuadro demencial subagudo, síntomas motores, visuales, y mioclonías. Presenta electroencefalograma (EEG) con actividad espicular pseudoperiódica; resonancia agnética (RM) con hiperintesidad de núcleos estriados y áreas de corteza cerebral; y líquido céfalo r...

  18. Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease

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    Talbott, Sabrina D.; Sattenberg, Ronald J.; Heidenreich, Jens O. (Dept. of Radiology, Univ. of Louisville, Louisville (United States)), e-mail: sdtalb02@gwise.louisville.edu; Plato, Brian M (Dept. of Neurology, Univ. of Louisville, Louisville (United States)); Parker, John (Dept. of Pathology and Laboratory Medicine, Univ. of Louisville, Louisville (United States))

    2011-04-15

    Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three patients with sporadic Creutzfeldt-Jakob disease confirmed by brain biopsy or autopsy and review the literature of imaging manifestations of this disease

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

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  20. Visual art therapy in sporadic Creutzfeldt-Jakob disease: a case study.

    Science.gov (United States)

    Shrestha, Rajeet; Trauger-Querry, Barbara; Loughrin, Athena; Appleby, Brian S

    2016-01-01

    This paper describes the diagnostic and treatment utility of visual art therapy in a case of sporadic Creutzfeldt-Jakob disease. Visual art therapy was compared longitudinally with clinical and neuroimaging data over five-month period in an autopsy-confirmed case of sporadic Creutzfeldt-Jakob disease of MM2-cortical subtype. Art therapy sessions and content were useful in ascertaining neuropsychiatric symptoms during the course of her illness. Art therapy offered a unique emotional and cognitive outlet as illness progressed. Patients and families affected by sporadic Creutzfeldt-Jakob disease may benefit from art therapy despite the rapidly progressive nature of the illness. Art therapy can also be useful for assessment of patients with sporadic Creutzfeldt-Jakob disease by healthcare professionals.

  1. A case of pure autotopagnosia following Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Tamura, Itaru; Hamada, Shinsuke; Soma, Hiroyuki; Moriwaka, Fumio; Tashiro, Kunio

    2016-12-02

    A 69-year-old male (N.A.) with Creutzfeldt-Jakob disease showed pure autotopagnosia. We administered tests evaluating his ability to name his own body parts, to point to body parts (his own and examiner's), and to recognize positional relationships between his body parts by verbal questions and responses. We found impaired localization of the patient's own body parts by pointing and impaired recognition of positional relationships between his body parts. However, there was no impairment in naming his own body parts or in localizing the examiner's body parts. The results suggest a pure autotopagnosia in N.A. leading to an impairment of recognition of the spatial position of his body parts in a three-dimensional body representation within the egocentric reference frame. We were able to rule out the possibility that his pattern of performance could have been due to a disability in programming reaching movements of the arm.

  2. An unusual case of sporadic Creutzfeldt-Jakob disease (CJD).

    Science.gov (United States)

    Javed, Qaiser; Alam, Faouzi; Krishna, Sowmya; Jaganathan, Geetha

    2010-01-01

    A 49-year-old healthy white British female, not previously known to psychiatric services, presented with an acute onset of florid psychotic symptoms. Her symptoms included visual, auditory and tactile hallucinations as well as persecutory delusions. She was started on antipsychotic medication; however, her psychotic symptoms did not improve significantly in the first 3 months. Her blood tests were normal. Lumbar puncture was performed which was positive for protein 14-3-3. A computed tomography scan of the brain showed generalised atrophic changes. The history of early visual hallucinations, rapid cognitive decline and positive 14-3-3 result was in keeping with the Heidenhain variant of sporadic Creutzfeldt-Jakob disease (sCJD). Despite a short life expectancy as reported in literature, our patient, who was diagnosed with sCJD more than two-and-a-half years ago, is still alive. We therefore believe this is an important finding to report.

  3. Asymmetric neuroimaging in Creutzfeldt-Jakob disease: a ruse.

    Science.gov (United States)

    Bavis, James; Reynolds, Patrick; Tegeler, Charles; Clark, Paige

    2003-10-01

    Creutzfeldt-Jakob disease (CJD) causes diffuse neurological symptoms, but asymmetric lesions have been found on conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI). Less often, position emission tomography (PET) scanning can also reveal asymmetric lesions in patients with CJD. Such imaging may mislead clinicians. The authors present a case of a woman with CJD who was diagnosed as having suffered a stroke because she had asymmetric T2-weighted imaging (T2WI) MRI abnormalities that were interpreted as a stroke. It was noted that the patient had clinical features consistent with CJD, including rapidly progressive dementia, myoclonus, cerebellar dysfunction, and pyramidal and extrapyramidal signs. This diagnosis was supported by periodic epileptiform discharges on the electroencephalogram (EEG) and by elevated 14-3-3 protein in the cerebrospinal fluid. MRI T2WI and DWI showed dramatically asymmetric abnormalities involving the left cortex. A PET study found decreased metabolism in the left cerebral and right cerebellar hemispheres. The patient's clinical, EEG, and laboratory data were all consistent with CJD, not other diseases, but the MRI and PET had atypical, asymmetric findings. This case demonstrates that CJD should be considered in the differential diagnosis of patients with rapidly progressive neurological decline, even if they have asymmetric imaging findings.

  4. "Preclinical" MSA in definite Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Rodriguez-Diehl, Roberta; Rey, Maria Jesus; Gironell, Alexandre; Martinez-Saez, Elena; Ferrer, Isidre; Sánchez-Valle, Raquel; Jagüe, Jordi; Nos, Carlos; Gelpi, Ellen

    2012-04-01

    Multiple system atrophy (MSA) is a sporadic alpha-synucleinopathy clinically characterized by variable degrees of parkinsonism, cerebellar ataxia and autonomic dysfunction. The histopathological hallmark of MSA is glial cytoplasmic inclusion (GCI). It is considered to represent the earliest stage of the degenerative process in MSA and to precede neuronal degeneration. Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal, rapidly progressive dementia generally associated with ataxia, pyramidal and extrapyramidal symptoms and myoclonus. Definite diagnosis needs neuropathological demonstration of variable degrees of spongiform degeneration of neuropil, neuronal loss, astro- and microgliosis, and the presence of abnormal deposits of the misfolded prion protein PrP(res) . Both diseases, CJD and MSA are infrequent among neurodegenerative diseases. In the present report we describe clinical and neuropathological findings of a previously healthy 64-year-old woman who developed symptoms of classical CJD. At post mortem examination, the brain showed in addition to classical methionine/methionine PrP(res) type 1 (MM1) sCJD changes and moderate Alzheimer-type pathology, features of "preclinical" MSA with minimal histopathological changes. These were characterized by discrete amounts of alpha-synuclein immunoreacive glial cytoplasmic inclusions in the striato-nigral system, isolated intraneuronal inclusions in pigmented neurons of the substantia nigra, as well as some vermiform intranuclear inclusions. To our knowledge, this is the first report on the coexistence of definite sCJD and "minimal changes" MSA in the same patient.

  5. Cerebrospinal Fluid Markers in Sporadic Creutzfeldt-Jakob Disease

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    Andrea Galassi

    2011-09-01

    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD is the commonest form of human prion diseases, accounting for about 85% of all cases. Current criteria for intra vitam diagnosis include a distinct phenotype, periodic sharp and slow-wave complexes at electroencephalography (EEG, and a positive 14-3-3-protein assay in the cerebrospinal fluid (CSF. In sCJD, the disease phenotype may vary, depending upon the genotype at codon 129 of the prion protein gene (PRNP, a site of a common methionine/valine polymorphism, and two distinct conformers of the pathological prion protein. Based on the combination of these molecular determinants, six different sCJD subtypes are recognized, each with distinctive clinical and pathologic phenotypes. We analyzed CSF samples from 127 subjects with definite sCJD to assess the diagnostic value of 14-3-3 protein, total tau protein, phosphorylated181 tau, and amyloid beta (Aβ peptide 1-42, either alone or in combination. While the 14-3-3 assay and tau protein levels were the most sensitive indicators of sCJD, the highest sensitivity, specificity and positive predictive value were obtained when all the above markers were combined. The latter approach also allowed a reliable differential diagnosis with other neurodegenerative dementias.

  6. Enfermedad de Creutzfeldt-Jakob: Reporte de dos casos.

    Directory of Open Access Journals (Sweden)

    Carla Brevis C.

    2011-12-01

    Full Text Available INTRODUCCIÓN: La enfermedad de Creutzfeldt-Jakob (ECJ es una enfermedad priónica neurodegenerativa que afecta el Sistema Nervioso Central (SNC, invariablemente mortal. Clasificada en esporádica, familiar e iatrogénica, se manifiesta por cuadro demencial subagudo, síntomas motores, visuales, y mioclonías. Presenta electroencefalograma (EEG con actividad espicular pseudoperiódica; resonancia agnética (RM con hiperintesidad de núcleos estriados y áreas de corteza cerebral; y líquido céfalo raquídeo (LCR con aumento de proteína 14-3-3. Finalmente, un estudio histopatológico del cerebro establece la encefalopatía espongiforme. PRESENTACIÓN DEL CASO: Se exponen dos casos de ECJ. Ambas mujeres, 44 y 67 años, con alteración de la marcha, deterioro cognitivo y mioclonías, cuadro rápidamente progresivo hasta la dependencia absoluta. EEG en ambos casos compatible con ECJ, además de imágenes que revelan hiperintensidad en núcleos estriados y/o zonas de la corteza. Ambas cursaron con infecciones durante su hospitalización y se les realizó gastrostomía por presentar trastorno deglutorio severo. DISCUSIÓN: El diagnóstico de ECJ se sospecha con la clínica, y se fundamenta con hallazgos característicos en RM, EEG y análisis de LCR. Ante la sospecha de la forma familiar se sugiere estudio genético, sin embargo, en el Hospital Clínico Herminda Martin (HCHM no se realiza. Al ser una enfermedad invariablemente mortal y sin tratamiento, dificulta la decisión entre realización de estudios e intervenciones, contra el manejo expectante.

  7. Creutzfeldt-Jakob Disease: Analysis of Four Cases

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    Ali Al Balushi

    2016-08-01

    Full Text Available Background: Creutzfeldt-Jakob disease (CJD is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over two-year period. Cases: The first patient is an 82-year-old woman who presented with depression, cognitive decline and word-finding difficulty over 4 weeks. The patient deteriorated neurologically to akinetic mutism and death within 6 weeks of presentation. The second patient is a 54-year-old woman with liver cirrhosis who presented with confusion, ataxia and multiple falls over 4 weeks. She was treated initially for hepatic encephalopathy, but continued to progress to mutism, startle myoclonus and obtundation. Death occurred within 4 weeks of presentation. The third patient is a 58-year-old woman who presented with an 8-week history of confusion, urinary incontinence, Parkinsonism, ataxia and myoclonus. Death occurred within 2 months from presentation. The fourth patient is a 67-year-old man who presented with a 6-week history of headache, blurred vision, ataxia and personality change and progressed to confusion, myoclonus, akinetic mutism and obtundation. Death occurred within 3 weeks from presentation. Conclusion: These 4 cases highlight the varied possible clinical presentations of CJD and demonstrate the importance of considering CJD in patients with atypical presentations of rapidly progressive cognitive decline. To diagnose CJD, brain biopsy remains the gold standard. However, the presence of CSF protein 14-3-3, typical MRI findings and suggestive EEG abnormalities all support the diagnosis.

  8. CSF Neurofilament Proteins Levels are Elevated in Sporadic Creutzfeldt-Jakob Disease

    NARCIS (Netherlands)

    van Eijk, Jeroen J. J.; van Everbroeck, Bart; Abdo, W. Farid; Kremer, Berry P. H.; Verbeek, Marcel M.

    2010-01-01

    In this study we investigated the cerebrospinal fluid (CSF) levels of neurofilament light (NFL) and heavy chain (NFHp35), total tau (t-tau), and glial fibrillary acidic protein (GFAP) to detect disease specific profiles in sporadic Creutzfeldt Jakob disease (sCJD) patients and Alzheimer's disease

  9. CSF neurofilament proteins levels are elevated in sporadic Creutzfeldt-Jakob disease.

    NARCIS (Netherlands)

    Eijk, J.J.J. van; Everbroeck, B. van; Abdo, W.; Kremer, H.P.H.; Verbeek, M.M.

    2010-01-01

    In this study we investigated the cerebrospinal fluid (CSF) levels of neurofilament light (NFL) and heavy chain (NFHp35), total tau (t-tau), and glial fibrillary acidic protein (GFAP) to detect disease specific profiles in sporadic Creutzfeldt Jakob disease (sCJD) patients and Alzheimer's disease

  10. Risk factors for Creutzfeldt-Jakob disease: a reanalysis of case-control studies.

    NARCIS (Netherlands)

    D.P.W.M. Wientjens (Dorothee); Z. Davanipour; K. Kondo; W.B. Matthews; R.G. Will (Robert); C.M. van Duijn (Cock); A. Hofman (Albert)

    1996-01-01

    textabstractTo review the evidence for risk factors of Creutzfeldt-Jakob disease (CJD), we pooled and reanalyzed the raw data of three case-control studies. The pooled data set comprised 178 patients and 333 control subjects. The strength of association between CJD and putative risk factors was asse

  11. CSF Neurofilament Proteins Levels are Elevated in Sporadic Creutzfeldt-Jakob Disease

    NARCIS (Netherlands)

    van Eijk, Jeroen J. J.; van Everbroeck, Bart; Abdo, W. Farid; Kremer, Berry P. H.; Verbeek, Marcel M.

    2010-01-01

    In this study we investigated the cerebrospinal fluid (CSF) levels of neurofilament light (NFL) and heavy chain (NFHp35), total tau (t-tau), and glial fibrillary acidic protein (GFAP) to detect disease specific profiles in sporadic Creutzfeldt Jakob disease (sCJD) patients and Alzheimer's disease (A

  12. Influence of timing on CSF tests value for Creutzfeldt-Jakob disease diagnosis

    NARCIS (Netherlands)

    P. Sanchez-Juan (Pascual); R. Sánchez-Valle (Raquel); A. Green (Alison); A. Ladogana (Anna); N. Cuadrado-Corrales (Natividad); E. Mitrová (Eva); K. Stoeck (Katharina); T. Sklaviadis (Theodoros); J. Kulczycki (Jerzy); K. Hess; A. Krasnianski (Anna); M. Equestre; D. Slivarichová; A. Saiz (Albert Abe); M. Calero (Miguel); M. Pocchiari (Maurizio); R.S.G. Knight (Richard); P. Tikka-Kleemola (Päivi); I. Zerr (Inga)

    2007-01-01

    textabstractBackground: The analysis of markers in the cerebrospinal fluid (CSF) is useful in the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD). However, the time at which the study of these markers is most sensitive remains controversal. Objective: To assess the influence of time of sampli

  13. Differentiation of prions from L-type BSE versus sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Nicot, Simon; Bencsik, Anna; Morignat, Eric; Mestre-Francés, Nadine; Perret-Liaudet, Armand; Baron, Thierry

    2012-12-01

    We compared transmission characteristics for prions from L-type bovine spongiform encephalopathy and MM2-cortical sporadic Creutzfeldt-Jakob disease in the Syrian golden hamster and an ovine prion protein-transgenic mouse line and isolated distinct prion strains. Our findings suggest the absence of a causal relationship between these diseases, but further investigation is warranted.

  14. Creutzfeldt-Jakob disease 38 years after diagnostic use of human growth hormone

    NARCIS (Netherlands)

    E.A. Croes (Esther); G. Roks (Gerwin); G.H. Jansen; P.C. Nijssen; C.M. van Duijn (Cock)

    2002-01-01

    textabstractA 47 year old man is described who developed pathology proven Creutzfeldt-Jakob disease (CJD) 38 years after receiving a low dose of human derived growth hormone (hGH) as part of a diagnostic procedure. The patient presented with a cerebellar syndrome, which is compatible with iatrogenic

  15. Determination of neuronal antibodies in suspected and definite Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    O. Grau-Rivera (Oriol); R. Sánchez-Valle (Raquel); A. Saiz (Albert Abe); J.L. Molinuevo (José Luis); R. Bernabé (Reyes); E. Munteis (Elvira); F. Pujadas (Francesc); A. Salvador (Antoni); J. Saura (Júlia); A. Ugarte (Antonio); M.J. Titulaer (Maarten); J. Dalmau (Josep); F. Graus (Francesc)

    2014-01-01

    textabstractIMPORTANCE: Creutzfeldt-Jakob disease (CJD) and autoimmune encephalitis with antibodies against neuronal surface antigens (NSA-abs) may present with similar clinical features. Establishing the correct diagnosis has practical implications in the management of care for these patients.

  16. A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk

    NARCIS (Netherlands)

    P. Sanchez-Juan (Pascual); M.T. Bishop (Matthew); G.G. Kovacs (Gabor); M. Calero (Miguel); Y.S. Aulchenko (Yurii); A. Ladogana (Anna); A. Boyd (Alison); V. Lewis (Victoria); C. Ponto (Claudia); Calero, O. (Olga); A. Poleggi (Anna); A. Carracedo (Angel); S. van der Lee (Sven); T. Ströbel (Thomas); F. Rivadeneira Ramirez (Fernando); A. Hofman (Albert); S. Haik; O. Combarros (Onofre); J. Berciano (José); A.G. Uitterlinden (André); S.J. Collins (Steven); H. Budka (Herbert); J-P. Brandel (Jean-Philippe); J.-L. Laplanche (Jean-Louis); M. Pocchiari (Maurizio); I. Zerr (Inga); R. Knight (Richard); R.G. Will (Robert); C.M. van Duijn (Cock)

    2015-01-01

    textabstractWe performed a genome-wide association (GWA) study in 434 sporadic Creutzfeldt-Jakob disease (sCJD) patients and 1939 controls from the United Kingdom, Germany and The Netherlands. The findings were replicated in an independent sample of 1109 sCJD and 2264 controls provided by a

  17. A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk

    NARCIS (Netherlands)

    P. Sanchez-Juan (Pascual); M.T. Bishop (Matthew); G.G. Kovacs (Gabor); M. Calero (Miguel); Y.S. Aulchenko (Yurii); A. Ladogana (Anna); A. Boyd (Alison); V. Lewis (Victoria); C. Ponto (Claudia); Calero, O. (Olga); A. Poleggi (Anna); A. Carracedo (Angel); S. van der Lee (Sven); T. Ströbel (Thomas); F. Rivadeneira Ramirez (Fernando); A. Hofman (Albert); S. Haik; O. Combarros (Onofre); J. Berciano (José); A.G. Uitterlinden (André); S.J. Collins (Steven); H. Budka (Herbert); J-P. Brandel (Jean-Philippe); J.-L. Laplanche (Jean-Louis); M. Pocchiari (Maurizio); I. Zerr (Inga); R. Knight (Richard); R.G. Will (Robert); C.M. van Duijn (Cock)

    2015-01-01

    textabstractWe performed a genome-wide association (GWA) study in 434 sporadic Creutzfeldt-Jakob disease (sCJD) patients and 1939 controls from the United Kingdom, Germany and The Netherlands. The findings were replicated in an independent sample of 1109 sCJD and 2264 controls provided by a multinat

  18. A Case of Sporadic Creutzfeldt-Jakob Disease Presenting as Conversion Disorder.

    Science.gov (United States)

    Yegya-Raman, Nikhil; Aziz, Rehan; Schneider, Daniel; Tobia, Anthony; Leitch, Megan; Nwobi, Onyi

    2017-01-01

    Background. Creutzfeldt-Jakob disease is a rare disorder of the central nervous system. Its initial diagnosis may be obscured by its variable presentation. This case report illustrates the complexity of diagnosing this disease early in the clinical course, especially when the initial symptoms may be psychiatric. It offers a brief review of the literature and reinforces a role for consultation psychiatry services. Methods. PUBMED/MEDLINE was searched using the terms "Creutzfeldt-Jakob disease", "psychiatric symptoms", "conversion disorder", "somatic symptom disorder", "functional movement disorder", and "functional neurologic disorder". Case. The patient was a 64-year-old woman with no prior psychiatric history who was initially diagnosed with conversion disorder and unspecified anxiety disorder but soon thereafter was discovered to have Creutzfeldt-Jakob disease. Discussion. This case highlights the central role of psychiatric symptoms in early presentations of Creutzfeldt-Jakob disease. Still, few other cases in the literature report functional neurological symptoms as an initial sign. The consultation psychiatrist must remain alert to changing clinical symptoms, especially with uncharacteristic disease presentations.

  19. Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    Yull, H.M.; Ritchie, D.L.; Langeveld, J.P.M.; Zijderveld, van F.G.; Bruce, M.E.; Ironside, J.W.; Head, M.W.

    2006-01-01

    Molecular typing of the abnormal form of the prion protein (PrPSc) has come to be regarded as a powerful tool in the investigation of the prion diseases. All evidence thus far presented indicates a single PrPSc molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resultin

  20. Prions in the urine of patients with variant Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Moda, Fabio; Gambetti, Pierluigi; Notari, Silvio; Concha-Marambio, Luis; Catania, Marcella; Park, Kyung-Won; Maderna, Emanuela; Suardi, Silvia; Haïk, Stéphane; Brandel, Jean-Philippe; Ironside, James; Knight, Richard; Tagliavini, Fabrizio; Soto, Claudio

    2014-08-07

    Prions, the infectious agents responsible for transmissible spongiform encephalopathies, consist mainly of the misfolded prion protein (PrP(Sc)). The unique mechanism of transmission and the appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to consumption of prion-contaminated cattle meat, have raised concerns about public health. Evidence suggests that variant Creutzfeldt-Jakob disease prions circulate in body fluids from people in whom the disease is silently incubating. To investigate whether PrP(Sc) can be detected in the urine of patients with variant Creutzfeldt-Jakob disease, we used the protein misfolding cyclic amplification (PMCA) technique to amplify minute quantities of PrP(Sc), enabling highly sensitive detection of the protein. We analyzed urine samples from several patients with various transmissible spongiform encephalopathies (variant and sporadic Creutzfeldt-Jakob disease and genetic forms of prion disease), patients with other degenerative or nondegenerative neurologic disorders, and healthy persons. PrP(Sc) was detectable only in the urine of patients with variant Creutzfeldt-Jakob disease and had the typical electrophoretic profile associated with this disease. PrP(Sc) was detected in 13 of 14 urine samples obtained from patients with variant Creutzfeldt-Jakob disease and in none of the 224 urine samples obtained from patients with other neurologic diseases and from healthy controls, resulting in an estimated sensitivity of 92.9% (95% confidence interval [CI], 66.1 to 99.8) and a specificity of 100.0% (95% CI, 98.4 to 100.0). The PrP(Sc) concentration in urine calculated by means of quantitative PMCA was estimated at 1×10(-16) g per milliliter, or 3×10(-21) mol per milliliter, which extrapolates to approximately 40 to 100 oligomeric particles of PrP(Sc) per milliliter of urine. Urine samples obtained from patients with variant Creutzfeldt-Jakob disease contained minute quantities of PrP(Sc). (Funded by the

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

    Science.gov (United States)

    Taillefer, Marguerite S; Tangarorang, Glendo L; Kuchel, George A; Menkes, Daniel L

    2011-09-01

    We report an atypical presentation of sporadic Creutzfeldt-Jakob disease (CJD) in a 74-year-old woman that illustrates the difficulty in diagnosing this rare, but important, cause of rapidly progressive dementia. Despite well-established criteria, this diagnosis is often missed or substantially delayed (Table 1). In this case, a precipitous cognitive decline associated with a urinary tract infection initiallysuggested delirium. Although atypical CJD was considered as a cause when symptoms persisted, a definitive diagnosis was established postmortem when the cerebrospinal fluid (CSF) prion protein 14-3-3 tested positive. Creutzfeldt-Jakob disease must be considered in the differential diagnosis of rapidly progressive dementia as Connecticut accounts for approximately three of the more than 200 cases diagnosed nationally.

  2. Involvement of the endosomal-lysosomal system correlates with regional pathology in Creutzfeldt-Jakob disease

    DEFF Research Database (Denmark)

    Kovács, Gábor G; Gelpi, Ellen; Ströbel, Thomas

    2007-01-01

    The endosomal-lysosomal system (ELS) has been suggested to play a role in the pathogenesis of prion diseases. The purpose of this study was to examine how experimental observations can be translated to human neuropathology and whether alterations of the ELS relate to neuropathologic changes....... Combined with stereologic techniques, we examined components of the ELS in human sporadic Creutzfeldt-Jakob disease brains. We immunostained for the early endosomal marker Rab5 and lysosomal enzymes cathepsin D and B. We determined neuron-specific changes in their expression and correlated......-immunoreactive lysosomes. The intraneuronal distribution of cathepsin D and B diverges between Purkinje cells and frontal cortical neurons in sporadic Creutzfeldt-Jakob disease brains. We demonstrated focal intra- and perineuronal colocalization of cathepsin D and PrP. Our results indicate that effects in the ELS...

  3. Preliminary risk analysis applied to the transmission of Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Bertrand, E; Schlatter, J

    2011-01-01

    Transmissible spongiform encephalopathy (TSE) is a degenerative disease of the central nervous system. As yet, there is no human screening test and no effective treatment. This disease is invariably fatal. General preventive measures are therefore essential. The objective of this study is to analyze and address on a prioritized basis the risks relating to the transmission of Creutzfeldt-Jakob disease during surgical operations by means of a preliminary risk analysis (PRA). The PRA produces 63 scenarios with maximum risk relating to operational and legal dangers. The study recommends a number of courses of action, such as training and internal controls, in order to reduce the risks identified. A procedure has been drawn up and assessed for each action. This PRA makes it possible to target and significantly reduce the potential dangers for transmission of Creutzfeldt-Jakob disease through the use of medical instruments.

  4. Unusual Phenotype of the Brownell-Oppenheimer Variant of Sporadic Creutzfeldt-Jakob Disease

    Directory of Open Access Journals (Sweden)

    Dronacharya Lamichhane

    2016-03-01

    Full Text Available Creutzfeldt-Jakob disease is a rare, transmissible, neurodegenerative disease caused by conformationally changed abnormal prion protein. Most patients present with cognitive impairment, myoclonus, ataxia, visual impairment alone or in combination. Patients who present with ataxia only at the onset are said to have Brownell-Oppenheimer variant of the disease. However, here we present a case where visual symptoms preceded the clinical presentation and hallucinations accompanied the ataxia at the onset of the disease.

  5. A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis?

    OpenAIRE

    Tan, Bowei; Morales Mangual, Carlos; Mahmud, Iftekhar; Tongo, Nosakhare D; Mararenko, Larisa; Kay, Arthur

    2017-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present?a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3....

  6. Unsuccessful intraventricular pentosan polysulphate treatment of variant Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Whittle, I R; Knight, R S G; Will, R G

    2006-06-01

    Pentosan polysulphate, delivered by chronic intraventricular infusion, has been proposed as a potential therapy for human prion disease. The first treated patient is still alive several years after treatment started. Here we describe in detail a case of variant Creutzfeldt-Jakob disease in which this treatment was started at a relatively early stage but had no definite clinical benefit. The patient died from disease progression 16 months after diagnosis and 5 months after pentosan polysulphate treatment was commenced.

  7. Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia

    OpenAIRE

    S. E. McPherson; J. D. Kuratani; Cummings, J L.; J. Shih; Mischel, P. S.; Vinters, H V

    1994-01-01

    Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD), and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia. We report an autopsy-confirmed case of CJD in which the presenting symptom was change in language abilities. The patient ultimately evidenced mixed transcortical aphasia (MTA) with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the s...

  8. Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles.

    Directory of Open Access Journals (Sweden)

    Romolo Nonno

    2006-02-01

    Full Text Available Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model. We report that the efficiency of primary transmission of prions from Creutzfeldt-Jakob disease patients to a wild rodent species, the bank vole (Clethrionomys glareolus, is comparable to that reported in transgenic mice carrying human prion protein, in spite of a low prion protein-sequence homology between man and vole. Voles infected with sporadic and genetic Creutzfeldt-Jakob disease isolates show strain-specific patterns of spongiform degeneration and pathological prion protein-deposition, and accumulate protease-resistant prion protein with biochemical properties similar to the human counterpart. Adaptation of genetic Creutzfeldt-Jakob disease isolates to voles shows little or no evidence of a transmission barrier, in contrast to the striking barriers observed during transmission of mouse, hamster, and sheep prions to voles. Our results imply that in voles there is no clear relationship between the degree of homology of the prion protein of the donor and recipient species and susceptibility, consistent with the view that the prion strain gives a major contribution to the species barrier. The vole is therefore a valuable model to study human prion diversity and, being susceptible to a range of animal prions, represents a unique tool for comparing isolates from different species.

  9. Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles.

    Directory of Open Access Journals (Sweden)

    2006-02-01

    Full Text Available Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model. We report that the efficiency of primary transmission of prions from Creutzfeldt-Jakob disease patients to a wild rodent species, the bank vole (Clethrionomys glareolus, is comparable to that reported in transgenic mice carrying human prion protein, in spite of a low prion protein-sequence homology between man and vole. Voles infected with sporadic and genetic Creutzfeldt-Jakob disease isolates show strain-specific patterns of spongiform degeneration and pathological prion protein-deposition, and accumulate protease-resistant prion protein with biochemical properties similar to the human counterpart. Adaptation of genetic Creutzfeldt-Jakob disease isolates to voles shows little or no evidence of a transmission barrier, in contrast to the striking barriers observed during transmission of mouse, hamster, and sheep prions to voles. Our results imply that in voles there is no clear relationship between the degree of homology of the prion protein of the donor and recipient species and susceptibility, consistent with the view that the prion strain gives a major contribution to the species barrier. The vole is therefore a valuable model to study human prion diversity and, being susceptible to a range of animal prions, represents a unique tool for comparing isolates from different species.

  10. Symptomatic aggravation after corticosteroid pulse therapy in definite sporadic Creutzfeldt-Jakob disease with the feature of Hashimoto's encephalopathy.

    Science.gov (United States)

    Jang, Jae-Won; Park, So Young; Park, Young Ho; Kim, Jung E; Kim, SangYun

    2014-09-08

    Creutzfeldt-Jakob disease and Hashimoto's encephalopathy often show similar clinical presentation. Among Creutzfeldt-Jakob disease mimics, Hashimoto's encephalopathy is particularly important as it is treatable with corticosteroids. Thus, in cases of middle-aged woman diagnosed with probable Creutzfeldt-Jakob disease and who exhibit high titers of antithyroid antibodies, corticosteroid pulse therapy is typically performed with expectations of near complete recovery from Hashimoto's encephalopathy. Herein, we provide the first case report that exhibited a negative effect of corticosteroid pulse therapy for a patient with Creutzfeldt-Jakob disease with features of Hashimoto's encephalopathy. We report a case of 59-year-old Asian woman with blurred vision, dysarthria, myoclonus, and rapidly progressive dementia. Cerebrospinal fluid showed 14-3-3 protein positive. Electroencephalogram showed periodic sharp waves (1.5 Hz) at the bilateral frontal or occipital areas. Magnetic resonance imaging showed high signal intensities at the bilateral cerebral cortex, caudate nucleus, and putamen. The patient was diagnosed with probable Creutzfeldt-Jakob disease. However, serum analysis showed a high titer of antithyroid antibodies. We started corticosteroid pulse therapy with subsequent aggravation of seizure activity including generalized myoclonus, epilepsia parialis continua, and ballistic dyskinesia, which was effectively treated with clonazepam. We provide evidence of a case of Creutzfeldt-Jakob disease that exhibited clinical deterioration after corticosteroid therapy. Although histopathological confirmation with brain biopsy is not easily available in Creutzfeldt-Jakob disease patients, selective initiation of corticosteroid pulse therapy should be considered in cases of uncertain diagnosis for differentiation with Hashimoto's encephalopathy.

  11. The diagnostic efficiency of biomarkers in sporadic Creutzfeldt-Jakob disease compared to Alzheimer's disease

    DEFF Research Database (Denmark)

    Bahl, J.M.; Heegaard, N.H.; Falkenhorst, G.

    2009-01-01

    ) together with the prion protein gene genotype to discriminate patients with sCJD (n=21) from neurological controls (n=164) and Alzheimer's disease (AD) patients (n=49). Low p-tau/t-tau ratio was the best single marker for sCJD with 90% specificity against neurological controls at 86% sensitivity whilst NSE......Laboratory markers have a prominent place among the diagnostic criteria for sporadic Creutzfeldt-Jakob disease (sCJD). Here we investigate the capability of protein 14-3-3, total-tau (t-tau), threonin-181-phosphorylated tau (p-tau), and neuron-specific enolase (NSE) in cerebrospinal fluid (CSF...

  12. [Delayed diagnosis in a patient with Creutzfeldt-Jakob disease in a psychiatric hospital].

    Science.gov (United States)

    Roest, S; Mestdagh, I; de Grave, C; Pals, P

    2016-01-01

    A 51-year-old female teacher of dance was referred to the diagnostic unit of our psychiatric hospital with symptoms of anxiety and depression. The clinical image was suggestive of organic pathology, but this could not be determined with certainty until a late stage. We discuss the course of the patient's illness. Her symptoms appeared to be psychiatric and closely resembled those of Creutzfeldt-Jakob disease. We comment on some of the signs that could have led to an earlier diagnosis and we discuss the tools that are needed.

  13. Sporadic Creutzfeldt-Jakob Disease With Unilateral Symptoms in the Setting of Metastatic Renal Cell Carcinoma.

    Science.gov (United States)

    Rossi, Kyle C; Stahl, Christine M; Zhang, Pengfei; Liang, John W; Marcuse, Lara V; Lublin, Fred

    2017-07-01

    Although it is not rare for magnetic resonance imaging findings in Creutzfeldt-Jakob disease to be asymmetric, unilateral clinical syndromes are uncommonly reported and may confound diagnosis. In addition, neurological paraneoplastic syndromes are not common in renal cell carcinoma, though there are cases reported, often without an offending antibody isolated. A 66-year-old man was admitted with 1 month of left-sided numbness and "loss of control" of the left arm. Examination revealed action-induced irregular jerking movements of the left arm. Mental status testing was normal. Magnetic resonance imaging brain revealed patchy areas of restricted diffusion along the cerebral cortices. Screening computed tomographic scans revealed innumerable lung nodules compatible with metastases, as well as a renal mass consistent with renal cell carcinoma. Lumbar puncture was performed and cerebrospinal fluid was sent for paraneoplastic autoantibody evaluation and protein 14-3-3. Over the next week the patient developed dystonic posturing of the left arm, left leg jerking movements, a right arm action tremor, and cognitive impairment. Paraneoplastic autoantibodies were negative. Protein 14-3-3 was elevated and brain biopsy revealed spongiform encephalopathy with positive immunoblotting. The patient died about 2 months from symptom onset. Creutzfeldt-Jakob disease can present with entirely unilateral myoclonus and numbness, without specific complaints of cognitive impairment. Not every difficult or unclear neurological syndrome in a patient with metastatic cancer is a paraneoplastic syndrome.

  14. Polymorphisms in the prion protein gene and in the doppel gene increase susceptibility for Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    E.A. Croes (Esther); B.Z. Alizadeh (Behrooz); A.M. Bertoli Avella (Aida); T.A.M. Rademaker (Tessa); J. Vergeer-Drop (Jeannette); B. Dermaut (Bart); J.J. Houwing-Duistermaat (Jeanine); D.P.W.M. Wientjens (Dorothee); A. Hofman (Albert); C. van Broeckhoven (Christine); C.M. van Duijn (Cock)

    2004-01-01

    textabstractThe prion protein gene (PRNP) plays a central role in the origin of Creutzfeldt-Jakob disease (CJD), but there is growing interest in other polymorphisms that may be involved in CJD. Polymorphisms upstream of PRNP that may modulate the prion protein production as well as polymorphisms in

  15. Variant Creutzfeldt-Jakob Disease in France and the United Kingdom: Evidence for the Same Agent Strain

    NARCIS (Netherlands)

    Brandel, J.P.; Heath, C.A.; Head, M.W.; Levavasseur, E.; Knight, R.; Laplanche, J.L.; Langeveld, J.P.M.; Ironside, J.W.; Hauw, J.J.; Mackenzie, J.; Alperovitch, A.; Will, R.G.; Haik, S.

    2009-01-01

    Objective: Variant Creutzfeldt-Jakob disease (vCJD) was first reported in the United Kingdom in 1996. Since then, the majority of cases have been observed in the United Kingdom where there was a major epidemic of bovine spongiform encephalopathy. France was the second country affected. To address th

  16. Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions

    DEFF Research Database (Denmark)

    de Pedro-Cuesta, Jesús; Mahillo-Fernández, Ignacio; Rábano, Alberto

    2011-01-01

    Evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains debatable in part due to misclassification of exposure levels. In a registry-based case-control study, the authors applied a risk-based classification of surgical interventions to determine the association betw...

  17. A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia.

    Science.gov (United States)

    Coulthart, Michael B; Geschwind, Michael D; Qureshi, Shireen; Phielipp, Nicolas; Demarsh, Alex; Abrams, Joseph Y; Belay, Ermias; Gambetti, Pierluigi; Jansen, Gerard H; Lang, Anthony E; Schonberger, Lawrence B

    2016-10-01

    As of mid-2016, 231 cases of variant Creutzfeldt-Jakob disease-the human form of a prion disease of cattle, bovine spongiform encephalopathy-have been reported from 12 countries. With few exceptions, the affected individuals had histories of extended residence in the UK or other Western European countries during the period (1980-96) of maximum global risk for human exposure to bovine spongiform encephalopathy. However, the possibility remains that other geographic foci of human infection exist, identification of which may help to foreshadow the future of the epidemic. We report results of a quantitative analysis of country-specific relative risks of infection for three individuals diagnosed with variant Creutzfeldt-Jakob disease in the USA and Canada. All were born and raised in Saudi Arabia, but had histories of residence and travel in other countries. To calculate country-specific relative probabilities of infection, we aligned each patient's life history with published estimates of probability distributions of incubation period and age at infection parameters from a UK cohort of 171 variant Creutzfeldt-Jakob disease cases. The distributions were then partitioned into probability density fractions according to time intervals of the patient's residence and travel history, and the density fractions were combined by country. This calculation was performed for incubation period alone, age at infection alone, and jointly for incubation and age at infection. Country-specific fractions were normalized either to the total density between the individual's dates of birth and symptom onset ('lifetime'), or to that between 1980 and 1996, for a total of six combinations of parameter and interval. The country-specific relative probability of infection for Saudi Arabia clearly ranked highest under each of the six combinations of parameter × interval for Patients 1 and 2, with values ranging from 0.572 to 0.998, respectively, for Patient 2 (age at infection × lifetime) and

  18. The diagnostic efficiency of biomarkers in sporadic Creutzfeldt-Jakob disease compared to Alzheimer's disease

    DEFF Research Database (Denmark)

    Bahl, Justyna Maria Czarna; Heegaard, Niels Henrik Helweg; Falkenhorst, Gerhard;

    2009-01-01

    Laboratory markers have a prominent place among the diagnostic criteria for sporadic Creutzfeldt-Jakob disease (sCJD). Here we investigate the capability of protein 14-3-3, total-tau (t-tau), threonin-181-phosphorylated tau (p-tau), and neuron-specific enolase (NSE) in cerebrospinal fluid (CSF......) together with the prion protein gene genotype to discriminate patients with sCJD (n=21) from neurological controls (n=164) and Alzheimer's disease (AD) patients (n=49). Low p-tau/t-tau ratio was the best single marker for sCJD with 90% specificity against neurological controls at 86% sensitivity whilst NSE...... with the presence of 14-3-3 protein in CSF gave the best test specificity of 96% at 84% sensitivity. We conclude that the combination of more than one CSF marker for neurodegeneration can improve the diagnostic test accuracy for sCJD against neurological controls including patients with other dementias....

  19. Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia

    Directory of Open Access Journals (Sweden)

    S. E. McPherson

    1994-01-01

    Full Text Available Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD, and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia. We report an autopsy-confirmed case of CJD in which the presenting symptom was change in language abilities. The patient ultimately evidenced mixed transcortical aphasia (MTA with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the symptoms in MTA, including intact repetition and echolalia. Observation in this patient and a review of the literature suggest that frontal-subcortical circuit dysfunction may contribute to the syndrome of echolalia. This hypothesis offers an alternative explanation to “isolation” of the speech area as the cause of MTA.

  20. Heidenhain variant of Creutzfeldt-Jakob disease: An autopsy study from India

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    Kher Monica

    2009-01-01

    Full Text Available Prion diseases are rare, progressive and fatal neurodegenerative diseases characterized by long incubation period and short clinical course. We present a rare case of Heidenhain variant of Creutzfeldt-Jakob disease, occurring in a 55-year-old lady presenting with dementia, cortical blindness, and myoclonic jerks. She succumbed to the disease within 8 weeks of onset of symptoms. MRI revealed hyperintense signals on T2WI and fluid attenuated inversion recovery (FLAIR images in basal ganglia and fronto-temporal and parietal cortex, sparing thalamus, striate cortex and globus pallidum. Abundant abnormal prion protein deposits (PrP sc were detected in caudate, putamen, thalamus, cingulate and striate cortex, in comparison to frontal and parietal cortex while no deposits were found in globus pallidum. MRI changes did not correlate with degree of spongy change, gliosis or prion protein deposition. The cause for abnormal signal changes in MRI and FLAIR images remains unclear.

  1. Unique inflammatory RNA profiles of microglia in Creutzfeldt-Jakob disease

    Science.gov (United States)

    Baker, Christopher A.; Manuelidis, Laura

    2003-01-01

    Previous studies in Creutzfeldt-Jakob disease (CJD) have shown that myeloid cells in the periphery as well as derivative microglial cells in the brain are infectious. Microglia can show an activated phenotype before prion protein (PrP) pathology is detectable in brain, and isolated infectious microglia contain very little PrP. To find whether a set of inflammatory genes are significantly induced or suppressed with infection, we analyzed RNA from isolated microglia with relevant cDNA arrays, and identified 30 transcripts not previously examined in any transmissible spongiform encephalopathy. This CJD expression profile contrasted with that of uninfected microglia exposed to prototypic inflammatory stimuli such as lipopolysaccharide and IFN-, as well as PrP amyloid. These findings underscore inflammatory pathways evoked by the infectious agent in brain. Transcript profiles unique for CJD microglia and other myeloid cells provide opportunities for more sensitive preclinical diagnoses of infectious and noninfectious neurodegenerative diseases.

  2. Creutzfeldt-Jakob disease: A great masquerade in neurology, a rare case report from South India

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    Sivaprakash Varadan

    2015-01-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare, fatal neurodegenerative disease caused by an infectious protein called prion and is characterized by spongiform changes, neuronal loss, reactive astrocytic proliferation, and accumulation of pathologic cellular protein. Clinical presentation of CJD is characterized by rapidly progressive dementia, neurologic symptoms and visual impairment, and the development of akinetic mutism, which can mimic many neurological conditions. The diagnosis is based on clinical presentation, electroencephalogram, and typical cerebrospinal fluid and magnetic resonance imaging (MRI findings. Literature on the incidence and prevalence of CJD is lacking in South India. We report the case of a 57-year-old woman with progressive dementia and typical neurologic symptoms, myoclonic jerks, and MRI findings of CJD. This case highlights the need for a high index of suspicion to diagnose CJD.

  3. A Case Report of Probable Sporadic Creutzfeldt-Jakob Disease: How to Approach Early Diagnosis?

    Science.gov (United States)

    Tan, Bowei; Morales Mangual, Carlos; Mahmud, Iftekhar; Tongo, Nosakhare D; Mararenko, Larisa; Kay, Arthur

    2017-05-30

    Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral cortex, basal ganglia, and thalami in diffusion-weighted imaging (DWI). Electroencephalogram (EEG) showed bihemispheric periodic lateralizing epileptiform discharges. The probable diagnosis of sCJD was reached based on the clinical features, characteristic findings in her MRI, the EEG, and a positive 14-3-3 CSF assay. The literature was also reviewed for early diagnosis of sCJD.

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

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    Demet Özbabalık Adapınar

    2011-12-01

    Full Text Available Variant Creutzfeldt-Jakob disease (vCJD was first reported in the UK in 1996. Here, we report the first Turkish case of vCJD. A 47-year-old man, who has never lived outside of Turkey and had had no transfusion, was admitted to the University Hospital with speech disorder, cognitive decline and ataxia following depression, irritability, and personality change. The immunoassay of the 14-3-3 protein in the cerebrospinal fluid was negative. Brain magnetic resonance imaging revealed high-signal lesions involving the bilateral caudate and lentiform nucleus on T2- and diffusion-weighted imaging. The patient developed akinetic mutism 10 months after disease onset. The clinical presentation and neuroimaging findings were compatible with the vCJD cases reported since 1996 and met the World Health Organization’s case definition for probable vCJD.

  5. Iatrogenic Creutzfeldt-Jakob disease following human growth hormone therapy: case report

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    Caboclo Luís Otávio Sales Ferreira

    2002-01-01

    Full Text Available We report the case of a 41-year-old man with iatrogenic Creutzfeldt-Jakob disease (CJD acquired after the use of growth hormone (GH obtained from a number of pituitary glands sourced from autopsy material. The incubation period of the disease (from the midpoint of treatment to the onset of clinical symptoms was rather long (28 years. Besides the remarkable cerebellar and mental signs, the patient exhibited sleep disturbance (excessive somnolence from the onset of the symptoms, with striking alteration of the sleep architecture documented by polysomnography. 14-3-3 protein was detected in the CSF, and MRI revealed increased signal intensity bilaterally in the striatum, being most evident in diffusion-weighted (DW-MRI sequences. This is the second case of iatrogenic CJD associated with the use of GH reported in Brazil.

  6. Creutzfeldt-Jakob disease in Ireland: epidemiological aspects 1980-2002.

    LENUS (Irish Health Repository)

    Horan, Gail

    2012-02-03

    Surveillance for Creutzfeldt-Jakob disease (CJD) has been carried out in the Republic of Ireland since 1980. Initial surveillance was passive and based on consented autopsy confirmation of CJD in patients in whom there was a high index of clinical suspicion. Since 1999, an active surveillance programme involving formal notification of all suspect CJD cases has been in place. The annual mortality rate has increased from 0.34 cases\\/million in 1980 to 1.27 cases\\/million in 2001. In all, 29 cases have been pathologically confirmed: 1 had variant CJD (vCJD), 1 had iatrogenic human growth hormone-induced CJD and 1 had fatal insomnia. Sporadic CJD (sCJD) accounted for the remainder. This paper details the change in incidence over 22 years as the surveillance programme in Ireland got under way; the increased incidence is attributed to better case ascertainment, as has occurred in other countries where active surveillance programmes have been established.

  7. Creutzfeldt-Jakob disease with mixed transcortical aphasia: insights into echolalia.

    Science.gov (United States)

    McPherson, S E; Kuratani, J D; Cummings, J L; Shih, J; Mischel, P S; Vinters, H V

    1994-01-01

    Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD), and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia. We report an autopsy-confirmed case of CJD in which the presenting symptom was change in language abilities. The patient ultimately evidenced mixed transcortical aphasia (MTA) with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the symptoms in MTA, including intact repetition and echolalia. Observation in this patient and a review of the literature suggest that frontal-subcortical circuit dysfunction may contribute to the syndrome of echolalia. This hypothesis offers an alternative explanation to "isolation" of the speech area as the cause of MTA.

  8. The Heidenhain variant of Creutzfeldt-Jakob disease and concomitant tau pathology: A case report.

    Science.gov (United States)

    Ehler, Edvard; Pipka, Michael; Meleková, Alena; Mandysová, Petra; Johanidesová, Silvie; Matěj, Radoslav; Rusina, Robert

    2017-02-10

    The Heidenhain form of Creutzfeldt-Jakob disease (CJD) is a rare CJD variant with predominantly visual symptoms in the early stages. Clinical manifestations of metamorphopsia, hemianopia and Balint's syndrome correlate with the involvement of the posterior cortical regions. A 71-year old healthy and very active man was admitted because of impaired visual acuity, hemianopia, and gait disturbance progressing over one week. MRI found typical cortical hyperintensities in the occipital regions while rhythm slowing and sharp waves were seen in the occipital regions on EEG. Protein 14-3-3 was detected in the cerebrospinal fluid. Postmortem neuropathology revealed typical histopathological changes consistent with CJD. Moreover, we found deposits of phosphorylated tau protein in the limbic regions that met the criteria for primary age-related tauopathy (PART); representing an additional and interesting finding in our case.

  9. Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients

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    Hanae Takatsuki, PhD

    2016-10-01

    Full Text Available Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 106/g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 106/g SD50 did not exist the infectivity.

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

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    Iria Grande

    2011-01-01

    Full Text Available We describe a case report of an 80-year-old woman who presented with symptomatology compatible with an episode of major depression with catatonia. After psychiatric admission, electroconvulsive therapy (ECT was applied, but symptoms progressed with cognitive impairment, bradykinesia, widespread stiffness, postural tremor, and gait disturbance. After compatible magnetic resonance imaging (MRI, diffusion changes, and electroencephalogram (EEG findings the case was reoriented to Creutzfeldt-Jakob disease (CJD. The genetic study found a methionine/valine heterozygosity at codon 129 of the prion protein gene PrPSc. On followup, a significant clinical recovery turned out. For this reason, EEG and MRI were repeated and confirmed the findings. The patient subsequently demonstrated progressive clinical deterioration and died 21 months later. The diagnosis was verified postmortem by neuropathology. The vCJD subtype MV2 is indeed characterized by early and prominent psychiatric symptoms and a prolonged disease duration however no frank clinical recovery has before been reported.

  11. Magnetic resonance spectroscopic abnormalities in sporadic and variant Creutzfeldt-Jakob disease

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    Pandya, H.G.; Coley, S.C.; Wilkinson, I.D.; Griffiths, P.D

    2003-02-01

    AIM: To study the proton MR spectroscopic findings in Creutzfeldt-Jakob disease (CJD) (sporadic and variant). MATERIALS AND METHODS: MR imaging and proton MR spectra were acquired in two patients with sporadic CJD (biopsy proven) and one patient with variant CJD. RESULTS: The two patients with sporadic CJD demonstrated MR signal change within the basal ganglia and thalami and reduced N-acetylaspartate (NAA):creatine ratios. The patient with variant CJD showed characteristic signal change within the pulvinar of the thalami and a markedly reduced N-acetylaspartate:creatine ratio. CONCLUSION: All three patients with CJD demonstrated evidence of reduced N-acetylaspartate: creatine ratios on MR spectroscopy. These changes imply that neuronal loss and/or dysfunction is a consistent finding in established CJD. Pandya H. G., et al (2003) Clinical Radiology58, 148--153.

  12. MRI of Creutzfeldt-Jakob disease: Imaging features and recommended MRI protocol

    Energy Technology Data Exchange (ETDEWEB)

    Collie, D.A.; Sellar, R.J.; Zeidler, M.; Colchester, A.C.F.; Knight, R.; Will, R.G

    2001-09-01

    Creutzfeldt-Jakob Disease (CJD) is a rare, progressive and invariably fatal neurodegenerative disease characterized by specific histopathological features. Of the four subtypes of CJD described, the commonest is sporadic CJD (sCJD). More recently, a new clinically distinct form of the disease affecting younger patients, known as variant CJD (vCJD), has been identified, and this has been causally linked to the bovine spongiform encephalopathy (BSE) agent in cattle. Characteristic appearances on magnetic resonance imaging (MRI) have been identified in several forms of CJD; sCJD may be associated with high signal changes in the putamen and caudate head and vCJD is usually associated with hyperintensity of the pulvinar (posterior nuclei) of the thalamus. These appearances and other imaging features are described in this article. Using appropriate clinical and radiological criteria and tailored imaging protocols, MRI plays an important part in the in vivodiagnosis of this disease. Collie, D.A. et al. (2001)

  13. A patient with a 'typical presentation' of Wernicke encephalopathy was found to have sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Goossens, K; van Bruchem-Visser, R L

    2017-06-01

    Creutzfeldt-Jakob disease (CJD) has a significant degree of clinical heterogeneity that is especially found in the features at onset. Here we present a patient with the sporadic form of CJD mimicking Wernicke encephalopathy. We first treated him with a high dose of thiamine; however, the vitamin B1 levels proved to be normal, which ruled out Wernicke encephalopathy. Meanwhile, his clinical condition progressively worsened and he developed a rapidly progressive cognitive disorder, mutism and myoclonus of the muscles. At this point, the diagnosis of CJD was most likely. The patient died two months after the first symptoms. Autopsy showed prion-protein depositions in several regions. Genetic analysis was negative for familial CJD. Those findings confirmed the diagnosis of 'sporadic Creutzfeldt-Jakob disease'. CJD presents in a wide range of sequences and clinical symptoms. Therefore, recognition in the early stage can be difficult.

  14. Development of Dose-Response Models of Creutzfeldt-Jakob Disease Infection in Nonhuman Primates for Assessing the Risk of Transfusion-Transmitted Variant Creutzfeldt-Jakob Disease

    Science.gov (United States)

    Gregori, Luisa; Anderson, Steven A.; Asher, David M.

    2014-01-01

    ABSTRACT Estimates for the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion have relied largely on data from rodent experiments, but the relationship between dose (amount of infected blood) and response (vCJD infection) has never been well quantified. The goal of this study was to develop a dose-response model based on nonhuman primate data to better estimate the likelihood of transfusion-transmitted vCJD (TTvCJD) in humans. Our model used dose-response data from nonhuman primates inoculated intracerebrally (i.c.) with brain tissues of patients with sporadic and familial CJD. We analyzed the data statistically by using a beta-Poisson dose-response model. We further adjusted model parameters to account for the differences in infectivity between blood and brain tissue and in transmission efficiency between intravenous (i.v.) and i.c. routes to estimate dose-dependent TTvCJD infection. The model estimates a mean infection rate of 76% among recipients who receive one unit of whole blood collected from an infected donor near the end of the incubation period. The nonhuman primate model provides estimates that are more consistent with those derived from a risk analysis of transfused nonleukoreduced red blood cells in the United Kingdom than prior estimates based on rodent models. IMPORTANCE TTvCJD was recently identified as one of three emerging infectious diseases posing the greatest immediate threat to the safety of the blood supply. Cases of TTvCJD were reported in recipients of nonleukoreduced red blood cells and coagulation factor VIII manufactured from blood of United Kingdom donors. As the quantity of abnormal prions (the causative agent of TTvCJD) varies significantly in different blood components and products, it is necessary to quantify the dose-response relationship for a wide range of doses for the vCJD agent in transfused blood and plasma derivatives. In this paper, we suggest the first mechanistic dose-response model for

  15. A case of Creutzfeldt-Jakob disease: diagnostic dilemmas of a rapidly fatal disease

    Directory of Open Access Journals (Sweden)

    Mirza M. Baig

    2013-10-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a rapidly progressive and ultimately fatal disorder of the central nervous system. It occurs worldwide with an incidence of 0.5-1 new case per million population per year. No specific treatment is available and management is limited to supportive care. Autopsy or biopsy provides a definitive diagnosis. Because of the transmissible nature of the disease and hesitancy of patients/family members to give consent for biopsy, numerous challenges in confirming the clinical diagnosis are faced by healthcare professionals. We report a case of 66-year-old male who was hospitalized due to hip fracture following a fall. Acute mental status changes followed the surgical fixation of hip fracture which triggered neurologic work up. This finally revealed suspicion and confirmation of CJD. Patient had progressive cognitive decline with akinetic mutism during further hospital stay and was later discharged home with hospice. Shorter thereafter he died at home. This case demonstrates the importance of keeping an open mind towards possibility of CJD when faced with esoteric neurologic presentations. Also this case provides insight into challenges in quarantine and sterilization of surgical instruments when these patients go through major surgeries.

  16. [A case of Creutzfeldt-Jakob disease presenting with arm levitation as an initial symptom].

    Science.gov (United States)

    Kamogawa, Kenji; Ninomiya, Satoko; Okuda, Shinya; Matsumoto, Yushi; Tomita, Hitomi; Okamoto, Kensho; Okuda, Bungo

    2014-01-01

    A 74-year-old, right handed man, developed insidiously with levitation and clumsiness of the right upper limb. His right arm tended to levitate spontaneously, when he was examined. He could put the elevated arm down on command, while the arm resumed to antigravity posture when his attention was diverted. His right arm also exhibited unwilled elevation when performing complex finger movements on the right side. He had a feeling of strangeness of the elevated limb, especially with the eyes closed. In addition to asymmetric limb-kinetic apraxia, combined sensations such as stereognosis were disturbed on the right side. Brain MRI showed high signal lesions predominantly in the left cerebral cortices and basal ganglia. SPECT with (123)I-IMP revealed asymmetric hypoperfusion, predominantly in the left medial frontal and parietal regions. Two months after the onset, levitation of the arm gradually disappeared, with the development of rapidly progressive dementia, frontal signs, dystonia and generalized myoclonus. The diagnosis of Creutzfeldt-Jakob disease (CJD) was made based on the clinical features and cerebrospinal fluid biomarkers. The early manifestation of the patient mimicked corticobasal degeneration which presents with arm levitation or alien hand syndrome. It is suggested that CJD can represent involuntary movements with higher brain dysfunction resembling corticobasal degeneration at the early stage of the illness. Although the underlying mechanism of arm levitation is still unknown, frontal disinhibition and parietal cortical sensory disturbance may contribute to the development of involuntary arm levitation in our patient.

  17. Creutzfeldt-Jakob disease masked by head trauma and features of Wilson's disease.

    Science.gov (United States)

    Scontrini, Alessandra; Di Bonaventura, Carlo; Fiorelli, Marco; Tiple, Dorina; Colaizzo, Elisa; Ladogana, Anna; Parchi, Piero; Pocchiari, Maurizio

    2015-04-01

    Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder typically characterized by progressive dementia associated with myoclonus, cerebellar and other focal neurological signs. Electroencephalogram, brain MRI and cerebrospinal fluid (CSF) analyses are helpful diagnostic tools, but diagnosis in patients with atypical presenting neurological signs is often difficult to make. A 55-year-old woman developed disorientation, drowsiness and focal motor signs after a traumatic brain injury due to an accidental fall. In two weeks, her symptoms worsened in spite of a brain MRI showed an improvement of traumatic lesions, but the presence of bilateral hyperintensity in the basal nuclei was suggestive of a metabolic or prion encephalopathy. The high 24-h urinary copper level and reduction of ceruloplasmin initially supported the diagnosis of Wilson's disease, but the absence of Kayser-Fleischer rings, and the positivity of 14-3-3 protein test and elevated tau concentrations in the CSF oriented toward a diagnosis of CJD. She died 5 months after the onset, and the postmortem examination of the brain revealed immunochemical features of CJD. This case exemplifies the difficulty of a timely diagnosis when rapid progressive dementia is masked by concomitant factors (i.e., head trauma) and neurological signs are associated with unclear laboratory findings.

  18. Three sporadic cases of Creutzfeldt-Jakob disease in China and their clinical analysis.

    Science.gov (United States)

    Wang, Xingbang; Li, Na; Liu, Aifen; Ma, Lin; Shan, Peiyan; Jiang, Wenjing; Zhang, Qun

    2017-09-01

    The present study described the characteristics of three cases of Creutzfeldt-Jakob disease (CJD) in China and analyzed their clinical presentations. The clinical information of the three cases was collected and analyzed. Blood and cerebrospinal fluid (CSF) specimens of the patients were collected for detection of the prion protein (PRNP) gene and 14-3-3 protein levels. Dynamic changes of electroencephalograms (EEGs) and brain magnetic resonance images (MRIs) were also observed. All the three cases were sporadic CJD cases. They presented with symptoms including hyposthenia, progressive memory loss, truncal and limb ataxia, dysarthria, lowered vision acuity, bucking, language disorders, myoclonia and akinetic mutism state. One of the three cases was associated with a prolonged duration of >6 years. The EEG of two cases showed slow biphasic waves. The diffusion-weighted MRI sequence revealed abnormal hyperintensity and bilateral ribboning in the cortex. Two patients tested positive for the 14-3-3 protein in the CSF. All patients were of methionine homozygosity at codon 129 in the gene encoding PRNP protein and one patient had a mutation. The CJD cases showed differences in terms of symptoms and disease duration. Subacute onset was common and with attentive nursing and supportive treatments, one of the patients had a prolonged survival time of >6 years.

  19. Cerebroventricular infusion of pentosan polysulphate in human variant Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Todd, N V; Morrow, J; Doh-ura, K; Dealler, S; O'Hare, S; Farling, P; Duddy, M; Rainov, N G

    2005-06-01

    Variant Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy believed to be caused by the bovine spongiform encephalopathy agent, an abnormal isoform of the prion protein (PrP(sc)). At present there is no specific or effective treatment available for any form of CJD. Pentosan polysulphate (PPS), a large polyglycoside molecule with weak heparin-like activity, has been shown to prolong the incubation period of the intracerebral infection when administered to the cerebral ventricles in a rodent scrapie model. PPS also prevents the production of further PrP(sc) in cell culture models. These properties of PPS prompted its cerebroventricular administration in a young man with vCJD. Long-term continuous infusion of PPS at a dose of 11 microg/kg/day for 18 months did not cause drug-related side effects. Follow-up CT scans demonstrated progressive brain atrophy during PPS administration. Further basic and clinical research is needed in order to address the issue of efficacy of PPS in vCJD and in other prion diseases.

  20. Doença de Creutzfeldt-Jakob: registro de caso

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    João Aris Kouyoumdjian

    1987-03-01

    Full Text Available É apresentado um caso da doença de Creutzfeldt-Jakob (DCJ com estudo neuropatológico. O paciente, de 76 anos de idade e do sexo masculino, apresentou quadro de demência rapidamente progressiva associada a ataxia, afasia, mioclonias e síndrome motora piramidal com evolução de aproximadamente 4 meses até o óbito. Havia antecedente de trauma ocular à direita causado por substância química de origem vegetal há cerca de 12 a 18 meses. O eletrencefalograma revelou lentificação difusa do traçado. Os achados neuropatológicos foram característicos. O encontro de partícula proteinácea infectante no "scrapie", descrita como "prion" ou PrP27-30 cujo antissoro reagiu com proteínas do cérebro de pacientes com DCJ abriu nova perspectiva na conceituação do agente etiológico, descrito anteriormente como vírus não convencional. A descrição de alguns casos de DCJ em jovens que faziam uso de hormônio de crescimento preparado de hipófises de cadáveres é preocupante em termos de aparecimento de novos casos.

  1. Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries

    Science.gov (United States)

    Diack, Abigail B.; Ritchie, Diane; Bishop, Matthew; Pinion, Victoria; Brandel, Jean-Philippe; Haik, Stephane; Tagliavini, Fabrizio; Van Duijn, Cornelia; Belay, Ermias D.; Gambetti, Pierluigi; Schonberger, Lawrence B.; Piccardo, Pedro; Will, Robert G.

    2012-01-01

    Variant Creutzfeldt-Jakob disease (vCJD) has been reported in 12 countries. We hypothesized that a common strain of agent is responsible for all vCJD cases, regardless of geographic origin. To test this hypothesis, we inoculated strain-typing panels of wild-type mice with brain material from human vCJD case-patients from France, the Netherlands, Italy, and the United States. Mice were assessed for clinical disease, neuropathologic changes, and glycoform profile; results were compared with those for 2 reference vCJD cases from the United Kingdom. Transmission to mice occurred from each sample tested, and data were similar between non-UK and UK cases, with the exception of the ranking of mean clinical incubation times of mouse lines. These findings support the hypothesis that a single strain of infectious agent is responsible for all vCJD infections. However, differences in incubation times require further subpassage in mice to establish any true differences in strain properties between cases. PMID:23017202

  2. Regulation of human cerebrospinal fluid malate dehydrogenase 1 in sporadic Creutzfeldt-Jakob disease patients

    Science.gov (United States)

    Schmitz, Matthias; Llorens, Franc; Pracht, Alexander; Thom, Tobias; Correia, Ângela; Zafar, Saima; Ferrer, Isidre; Zerr, Inga

    2016-01-01

    The identification of reliable diagnostic biomarkers in differential diagnosis of neurodegenerative diseases is an ongoing topic. A previous two-dimensional proteomic study on cerebrospinal fluid (CSF) revealed an elevated level of an enzyme, mitochondrial malate dehydrogenase 1 (MDH1), in sporadic Creutzfeldt-Jakob disease (sCJD) patients. Here, we could demonstrate the expression of MDH1 in neurons as well as in the neuropil. Its levels are lower in sCJD brains than in control brains. An examination of CSF-MDH1 in sCJD patients by ELISA revealed a significant elevation of CSF-MDH1 levels in sCJD patients (independently from the PRNP codon 129 MV genotype or the prion protein scrapie (PrPSc) type) in comparison to controls. In combination with total tau (tau), CSF-MDH1 detection exhibited a high diagnostic accuracy for sCJD diagnosis with a sensitivity of 97.5% and a specificity of 95.6%. A correlation study of MDH1 level in CSF with other neurodegenerative marker proteins revealed a significant positive correlation between MDH1 concentration with tau, 14-3-3 and neuron specific enolase level. In conclusion, our study indicated the potential of MDH1 in combination with tau as an additional biomarker in sCJD improving diagnostic accuracy of tau markedly. PMID:27852982

  3. Cathepsin D (C224T) polymorphism in sporadic and genetic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Kovacs, Gabor G; Sanchez-Juan, Pascual; Ströbel, Thomas; Schuur, Maaike; Poleggi, Anna; Nocentini, Sara; Giannattasio, Claudia; Belay, Girma; Bishop, Matthew; Capellari, Sabina; Parchi, Piero; Gelpi, Ellen; Gal, Aniko; Bakos, Agnes; Molnar, Maria J; Heinemann, Uta; Zerr, Inga; Knight, Richard S G; Mitrova, Eva; van Duijn, Cornelia; Budka, Herbert

    2010-01-01

    Accumulation of cathepsin D immunoreactive lysosomes correlates with tissue pathology in sporadic Creutzfeldt-Jakob disease (CJD) brains. The C-to-T transition within exon 2 of the cathepsin D (CTSD) gene is associated with altered enzymatic activity. Possession of the TT genotype is a risk factor for variant CJD. To verify the association between the CTSD position 224T allele and the risk for and survival in sporadic and genetic CJD, we genotyped 540 sporadic, 101 genetic CJD, and 723 control individuals. Genotype data and duration of illness were compared using multiple logistic regression and Kruskal-Wallis test. Multivariate survival analysis was performed using Cox's regression model. The distribution of CTSD position 224 alleles was approximately the same in all groups. We observed a trend for shorter survival in sporadic CJD patients harboring the T allele at position 224 of the CTSD gene in particular in sporadic CJD patients with the prion protein gene position 129 MM genotype. We conclude that the CTSD position 224 polymorphism alone is not a significant risk or disease-modifying factor in sporadic or genetic CJD.

  4. Subtype and regional-specific neuroinflammation in sporadic creutzfeldt-jakob disease.

    Science.gov (United States)

    Llorens, Franc; López-González, Irene; Thüne, Katrin; Carmona, Margarita; Zafar, Saima; Andréoletti, Olivier; Zerr, Inga; Ferrer, Isidre

    2014-01-01

    The present study identifies deregulated cytokines and mediators of the immune response in the frontal cortex and cerebellum of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 and VV2 subtypes compared to age-matched controls. Deregulated genes include pro- and anti-inflammatory cytokines, toll-like receptors, colony stimulating factors, cathepsins, members of the complement system, and members of the integrin and CTL/CTLD family with particular regional and sCJD subtype patterns. Analysis of cytokines and mediators at protein level shows expression of selected molecules and receptors in neurons, in astrocytes, and/or in microglia, thus suggesting interactions between neurons and glial cells, mainly microglia, in the neuroinflammatory response in sCJD. Similar inflammatory responses have been shown in the tg340 sCJD MM1 mice, revealing a progressive deregulation of inflammatory mediators with disease progression. Yet, inflammatory molecules involved are subjected to species differences in humans and mice. Moreover, inflammatory-related cell signaling pathways NFκB/IKK and JAK/STAT are activated in sCJD and sCJD MM1 mice. Together, the present observations show a self-sustained complex inflammatory and inflammatory-related responses occurring already at early clinical stages in animal model and dramatically progressing at advanced stages of sCJD. Considering this scenario, measures tailored to modulate (activate or inhibit) specific molecules could be therapeutic options in CJD.

  5. Neuronal phosphorylated RNA-dependent protein kinase in Creutzfeldt-Jakob disease.

    LENUS (Irish Health Repository)

    Paquet, Claire

    2009-02-01

    The mechanisms of neuronal apoptosis in Creutzfeldt-Jakob disease (CJD) and their relationship to accumulated prion protein (PrP) are unclear. A recent cell culture study showed that intracytoplasmic PrP may induce phosphorylated RNA-dependent protein kinase (PKR(p))-mediated cell stress. The double-stranded RNA protein kinase PKR is a proapoptotic and stress kinase that accumulates in degenerating neurons in Alzheimer disease. To determine whether neuronal apoptosis in human CJD is associated with activation of the PKR(p) signaling pathway, we assessed in situ end labeling and immunocytochemistry for PrP, glial fibrillary acidic protein, CD68, activated caspase 3, and phosphorylated PKR (Thr451) in samples of frontal, occipital, and temporal cortex, striatum, and cerebellum from 6 patients with sporadic CJD and 5 controls. Neuronal immunostaining for activated PKR was found in all CJD cases. The most staining was in nuclei and, in contrast to findings in Alzheimer disease, cytoplasmic labeling was not detected. Both the number and distribution of PKR(p)-positive neurons correlated closely with the extent of neuronal apoptosis, spongiosis, astrocytosis, and microglial activation and with the phenotype and disease severity. There was no correlation with the type, topography, or amount of extracellular PrP deposits. These findings suggest that neuronal apoptosis in human CJD may result from PKR(p)-mediated cell stress and are consistent with recent studies supporting a pathogenic role for intracellular or transmembrane PrP.

  6. Creutzfeldt-Jakob disease. Report of 10 neuropathologically-verified cases in Argentina.

    Science.gov (United States)

    Taratuto, A L; Piccardo, P; Leiguarda, R; Granillo, R; Monti, A; Scarlatti, A; Leits, A; Morasso, C; Marquez Vigo, C; Vila, J

    1989-01-01

    We describe 10 neuropathologically verified patients with Creutzfeldt-Jakob disease who died in Argentina between 1980 and 1987. Two of the ten cases were Chilean by birth. Another case visited Chile several times. Two cases (one Argentinian and one Chilean) regularly consumed sheep brain. Ages ranged from 42 to 63 years and the male to female ratio was 7:3. Disease duration ranged from 3.5 to 24 months. Prodromal symptoms presented as behavioral changes in 5 patients, lasting from one year to several weeks, and as neurological impairment in the other 5. Patients developed pyramidal, extrapyramidal and cerebellar disturbances, as well as movement disorders and progressive dementia. Visual alterations were found in 5 cases and periodic EEG activity in 7. Unequivocal cortical spongiform changes, together with varying degrees of neuronal depletion and astroglial hyperplasia were constant findings. No white matter involvement was apparent either from CT brain scans or on histopathological study of biopsied and autopsied material. Increasing awareness of this disease as well as possibilities of transmission is necessary in order to provide better information on its true incidence in Argentina.

  7. Enfermedad de creutzfeldt-jakob en el Perú: reporte de once casos

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    Luis Torres-Ramírez

    2014-04-01

    Full Text Available La enfermedad de Creutzfeldt-Jakob (ECJ es una enfermedad neurológica fatal producida por la isoforma patológica de la proteína priónica humana. Se reporta las características clínicas de seis casos de la forma esporádica de ECJ con diagnóstico definitivo por histopatología, y cinco casos con diagnóstico probable, en pacientes atendidos en el Instituto Nacional de Ciencias Neurológicas del Perú. La edad de inicio en los casos definitivos fue de 55,8 años y, en los probables, de 59,6 años, con predominio del sexo masculino. El tiempo de enfermedad fue de 8,8 meses. Se encontró un EEG típico en 50% de los casos definitivos y 80% de los probables. La proteína 14-3-3 en líquido cefalorraquídeo fue positiva en un caso probable y los hallazgos típicos en resonancia magnética se observaron en dos casos probables. Todos los casos cursaron con una evolución clínica típica de la enfermedad, y se considera el primer reporte de ECJ en el Perú

  8. Catatonia as Presentation of Creutzfeldt-Jakob Disease: a Case Report

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    Inês Silva Fernandes

    2017-03-01

    Full Text Available Background: Catatonia is a neuropsychiatric syndrome, classically related to schizophrenia,  but  more  often  associated  with other psychiatric, neurological and/or metabolic causes. Case Report: A 61-year-old man was admitted  in  the  Psychiatric  Department  with catatonia of unknown etiology. He was submitted to a detailed investigation including electroencephalogram that revealed triphasic periodic activity and cranial magnetic resonance imaging that revealed brain cortical and subcortical atrophy of frontal and medial  temporal  predominance.  The  patient was  then  transferred  to  the  Neurology  Department.  An increase  of  14.3.3  protein  in the cerebrospinal fluid, was detected and a presumptive diagnosis of spongiform encephalopathy was made. The clinical picture worsened with plurisegmental myoclonus, episodes of ocular deviation and dystonia. The patient died after 5 weeks. Anatomopathological examination confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease. Conclusions: This case report reflects the difficulty in the differential diagnosis of diseases with neuropsychiatric symptoms, particularly of catatonia, and the importance of coordination and multidisciplinary synergy in medicine.

  9. Codon 219 polymorphism of PRNP in healthy caucasians and Creutzfeldt-Jakob disease patients

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    Petraroli, R.; Pocchiari, M. [Instituto Superiore di Sanita, Rome (Italy)

    1996-04-01

    A number of point and insert mutations of the PrP gene (PRNP) have been linked to familial Creutzfeldt-Jakob disease (CJD) and Gerstmann-Straussler-Scheinker disease (GSS). Moreover, the methionine/valine homozygosity at the polymorphic codon 129 of PRNP may cause a predisposition to sporadic and iatrogenic CJD or may control the age at onset of familial cases carrying either the 144-bp insertion or codon 178, codon 198, and codon 210 pathogenic mutations in PRNP. In addition, the association of methionine or valine at codon 129 and the point mutation at codon 178 on the same allele seem to play an important role in determining either fatal familial insomnia or CJD. However, it is noteworthy that a relationship between codon 129 polymorphism and accelerated pathogenesis (early age at onset or shorter duration of the disease) has not been seen in familial CJD patients with codon 200 mutation or in GSS patients with codon 102 mutation, arguing that other, as yet unidentified, gene products or environmental factors, or both, may influence the clinical expression of these diseases. 17 refs.

  10. "Creutzfeldt-Jakob disease associated with non sterile phlebotomy (case report "

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    Ghorbani A

    2007-07-01

    Full Text Available Background: Creutzfeldt-Jakob disease (C-JD is a rare disorder characterized with rapidly progressive mental decline, myoclonic jerk and finally death. The transmissible pathogen for this disease is a proteinaceous infectious particle termed prion. The prion protein is encoded by a gene (designated as PRNP on the short arm chromosome 20.This disorder is diagnosed based on clinical findings, course of disease, EEG, MRI and confirmed with brain biopsy. Case report: A 56- year- old woman presented with confusion, disorientation, hyper somnolence, psychiatric problems such as hallucination, progressive mental deterioration and myoclonic jerks. She had history of several times phlebotomy with traditional and non sterile methods in two past years. She had no past history of other disease. Her illness was diagnosed based on clinical findings, course of her illness, typical MRI, EEG and rule out other dementing disease. She died after one month. Conclusion: in any patients with psychiatric disorders, rapidly progressive mental deterioration and myoclonic jerks C-JD should be considered as an important diagnosis. Treatable dementing disease should be considered and ruled out at first. The significance of phlebotomy in C-JD has yet to be determined.

  11. Creutzfeldt-Jakob disease (CJD) in a case of suspected chronic heavy metal poisoning.

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    Oehmichen, M; Schulz-Schaeffer, W; Kretzschmar, H; Theuerkauf, I; Gerling, I; Windl, O; Meissner, C

    2001-05-01

    We describe a patient who died of suspected heavy metal poisoning after a nine-month history of rapidly worsening dementia. Autopsy at a forensic-pathological institute established the postmortem diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) based on demonstration of the proteinase-resistant prion protein (PrPsSc) in Western-Blot on native brain tissue. Microscopic examination of the macroscopically largely inconspicuous brain revealed marked spongiform changes in the gray matter--mainly affecting the cerebral cortex, nucleus caudatus, and putamen--with confluent vacuoles. Patchy or perivacuolar deposits of PrPSc were found as well as granular PrPsc deposits. The cerebellum contained focal PrPsc deposits. There was an astrogliosis in the white matter and a proliferation of microglia in the gray matter with a simultaneous clear reduction in neuronal elements. The differential diagnosis is discussed, as is the potential risk to those performing autopsy on forensic cases with a clinical picture of rapidly progressing dementia, especially in cases where a prion disease is not initially suspected.

  12. Cathepsin D SNP associated with increased risk of variant Creutzfeldt-Jakob disease

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    Sanchez-Juan Pascual

    2008-04-01

    Full Text Available Abstract Background Variant Creutzfeldt-Jakob disease (vCJD originally resulted from the consumption of foodstuffs contaminated by bovine spongiform encephalopathy (BSE material, with 163 confirmed cases in the UK to date. Many thousands are likely to have been exposed to dietary infection and so it is important (for surveillance, epidemic modelling, public health and understanding pathogenesis to identify genetic factors that may affect individual susceptibility to infection. This study looked at a polymorphism in the cathepsin D gene (refSNP ID: rs17571 previously examined in Alzheimer's disease (AD. Methods Blood samples taken from 110 vCJD patients were tested for the C-T base change, and genotype data were compared with published frequencies for a control population using multiple logistic regression. Results There was a significant excess of the cathepsin D polymorphism TT genotype in the vCJD cohort compared to controls. The TT genotype was found to have a 9.75 fold increase in risk of vCJD compared to the CT genotype and a 10.92 fold increase compared to the CC genotype. Conclusion This mutation event has been observed to alter the protease activity of the cathepsin D protein and has been linked to an increase in amyloid beta plaque formation in AD. vCJD neuropathology is characterised by the presence of amyloid plaques, formed from the prion protein, and therefore alterations in the amyloid processing activity of cathepsin D may affect the neuropathogenesis of this disease.

  13. A common BACE1 polymorphism is a risk factor for sporadic Creutzfeldt-Jakob disease.

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    Olga Calero

    Full Text Available The β site APP cleaving enzyme 1 (BACE1 is the rate-limiting β-secretase enzyme in the amyloidogenic processing of APP and Aβ formation, and therefore it has a prominent role in Alzheimer's disease (AD pathology. Recent evidence suggests that the prion protein (PrP interacts directly with BACE1 regulating its β-secretase activity. Moreover, PrP has been proposed as the cellular receptor involved in the impairment of synaptic plasticity and toxicity caused by Aβ oligomers. Provided that common pathophysiologic mechanisms are shared by Alzheimer's and Creutzfeldt-Jakob (CJD diseases, we investigated for the first time to the best of our knowledge a possible association of a common synonymous BACE1 polymorphism (rs638405 with sporadic CJD (sCJD. Our results indicate that BACE1 C-allele is associated with an increased risk for developing sCJD, mainly in PRNP M129M homozygous subjects with early onset. These results extend the very short list of genes (other than PRNP involved in the development of human prion diseases; and support the notion that similar to AD, in sCJD several loci may contribute with modest overall effects to disease risk. These findings underscore the interplay in both pathologies of APP, Aβ oligomers, ApoE, PrP and BACE1, and suggest that aging and perhaps vascular risk factors may modulate disease pathologies in part through these key players.

  14. Enfermedad de creutzfeldt-jakob en el Perú: reporte de once casos

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    Luis Torres-Ramírez

    Full Text Available La enfermedad de Creutzfeldt-Jakob (ECJ es una enfermedad neurológica fatal producida por la isoforma patológica de la proteína priónica humana. Se reporta las características clínicas de seis casos de la forma esporádica de ECJ con diagnóstico definitivo por histopatología, y cinco casos con diagnóstico probable, en pacientes atendidos en el Instituto Nacional de Ciencias Neurológicas del Perú. La edad de inicio en los casos definitivos fue de 55,8 años y, en los probables, de 59,6 años, con predominio del sexo masculino. El tiempo de enfermedad fue de 8,8 meses. Se encontró un EEG típico en 50% de los casos definitivos y 80% de los probables. La proteína 14-3-3 en líquido cefalorraquídeo fue positiva en un caso probable y los hallazgos típicos en resonancia magnética se observaron en dos casos probables. Todos los casos cursaron con una evolución clínica típica de la enfermedad, y se considera el primer reporte de ECJ en el Perú

  15. Diversidad sindromatica de la enfermedad de Creutzfeldt-Jakob: correlato neurofisiologico e histopatologico

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    Sergio Ferrer D

    1982-03-01

    Full Text Available Se estudian dos casos de enfermedad de Creutzfeldt-Jakob comprobados por examen necrópsico. Uno de ellos presentaba típicas descargas hipersincrónicas, periódicas en el electroencefalograma; el otro caso no mostraba estas descargas epileptiformes y evolucionó con una lentitud difusa progresiva. La espongiosis, gliosis y pérdida neuronal fué intensa en la corteza del caso con espigas y muy moderada en el caso sin espigas. El compromiso subcortical era similar en ambos casos. Se postula que la diferente expresión topográfica de su histopatología explicaria tambien la diferencia en el comportamiento de los potenciales evocados somatosensoriales y el diferente modelo electroencefalográfico del sueno penthotálico. La independencia de las descargas epileptiformes en cada hemisferio se prueba con el test de Wada. Se discute el origen de las espigas y su periodicidad. Se postula teóricamente que el predominio de las lesiones de las capas II a IV privaria a las células piramidales remanentes, de influencias inhibitorias, lo que explicaria la génesis de las espigas.

  16. Doença de Creutzfeldt-Jakob: a propósito de um caso com comprometimento medular Creutzfeldt-Jakob disease: case report with spinal cord involvement

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    Marlos Fábio Alves de Azevedo

    2001-12-01

    Full Text Available A doença de Creutzfeldt-Jakob (DCJ é a encefalopatia espongiforme subaguda transmissível mais frequente nos seres humanos. Aproximadamente 85% dos casos pertencem à forma esporádica da doença. Os outros 15% consistem na forma genética e iatrogênica. Relatamos o caso de uma paciente com a forma esporádica da doença de Creutzfeldt-Jakob, com comprometimento medular e apresentação clínica caracterizada por síndrome demencial e cerebelar, miofasciculação com arreflexia difusa e crises convulsivas do tipo tônico-clônico generalizada. É rara a associação das duas últimas manifestações clínicas. O caso foi considerado como provável DCJ até confirmação por autópsia e imunohistoquímica. Concluímos que se deve sempre pensar na DCJ em pacientes que apresentam demência rapidamente progressiva e, na ausência de sinais piramidais ou extrapiramidais, pensar em acometimento periférico e/ou medular.Creutzfeldt-Jakob disease (CJD is the most common subacute transmissible spongiform encephalopathy. Approximately 85% of the cases are sporadic. The remaining 15% consist of genetic and iatrogenic forms. We report a sporadic form of CJD with spinal cord involvement and a clinical manifestation characterized by dementia and cerebellar syndrome, myofasciculation with absent reflexes and seizures. The two last manifestations are rare. The clinical hypothesis was probable CJD which was confirmed with autopsy and immunohistochemistry. We conclude that CJD should always be suspected when rapidly progressive dementia occurs and the absence of pyramidal or extrapyramidal signs suggest a spinal cord and/or peripheral nerve involvement.

  17. Enfermedad de Creutzfeldt-Jakob por RMI: alteración cortical como signo temprano de la enfermedad Creutzfeldt-Jakob disease by MRI: Cortical alteration as early sign disease

    OpenAIRE

    María Fernanda Markarian; Adriana Ojeda; Ana María Uriarte

    2008-01-01

    Se estudió por RMI un paciente de 59 años con diagnóstico probable de Enfermedad de Creutzfeldt-Jakob desde el inicio de sus síntomas. El paciente comienza con un cuadro de leve deterioro cognitivo. En una primera resonancia en secuencias FLAIR se visualiza hiperintensidad cortical a predomino de hemisferio izquierdo, no observándose en FSE T 2. Se hace más significativa en nueva resonancia en FLAIR y Difusión, con aparición de hiperintensidad en cabeza de ambos caudados y rápido deterioro co...

  18. Brain Dopamine Transporter Binding and Glucose Metabolism in Progressive Supranuclear Palsy-Like Creutzfeldt-Jakob Disease

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    Eero Rissanen

    2014-01-01

    Full Text Available Here, we present a patient with Creutzfeldt-Jakob disease (CJD who developed initial symptoms mimicking progressive supranuclear palsy (PSP. Before the development of typical CJD symptoms, functional imaging supported a diagnosis of PSP when [123I]-FP-CIT-SPECT showed a defect in striatal dopamine transporter binding, while [18F]-fluorodeoxyglucose PET showed cortical hypometabolism suggestive of Lewy body dementia. However, the postmortem neuropathological examination was indicative of CJD only, without tau protein or Lewy body findings. This case demonstrates that CJD should be taken into account in rapidly progressing atypical cases of parkinsonism, even when functional imaging supports a diagnosis of a movement disorder.

  19. Sporadic Creutzfeldt-Jakob disease with focal findings: caveats to current diagnostic criteria

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    Edward C. Mader

    2013-02-01

    Full Text Available The clinical diagnosis of Creutzfeldt-Jakob disease (CJD is largely based on the 1998 World Health Organization diagnostic criteria. Unfortunately, rigid compliance with these criteria may result in failure to recognize sporadic CJD (sCJD, especially early in its course when focal findings predominate and traditional red flags are not yet present. A 61-year-old man presented with a 3-week history of epilepsia partialis continua (jerking of the left upper extremity and a 2-week history of forgetfulness and left hemiparesis; left hemisensory neglect was also detected on admission. Repeated brain magnetic resonance imaging (MRI showed areas of restricted diffusion in the cerebral cortex, initially on the right but later spreading to the left. Electroencephalography (EEG on hospital days 7, 10, and 14 showed right-sided periodic lateralized epileptiform discharges. On day 20, the EEG showed periodic sharp wave complexes leading to a diagnosis of probable sCJD and subsequently to definite sCJD with brain biopsy. Neurological decline was relatively fast with generalized myoclonus and akinetic mutism developing within 7 weeks from the onset of illness. CJD was not immediately recognized because of the patient’s focal/lateralized manifestations. Focal/lateralized clinical, EEG, and MRI findings are not uncommon in sCJD and EEG/MRI results may not be diagnostic in the early stages of sCJD. Familiarity with these caveats and with the most current criteria for diagnosing probable sCJD (University of California San Francisco 2007, MRI-CJD Consortium 2009 will enhance the ability to recognize sCJD and implement early safety measures.

  20. Isolated language impairment as the primary presentation of sporadic Creutzfeldt Jakob Disease.

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    El Tawil, S; Chohan, G; Mackenzie, J; Rowe, A; Weller, B; Will, R G; Knight, R

    2017-03-01

    Sporadic Creutzfeldt Jakob Disease (sCJD) is a neurodegenerative disorder that typically presents as a rapidly progressive encephalopathy associated with various neurological features, culminating in akinetic mutism and death. Atypical cases, presenting with an isolated focal may cause diagnostic confusion. We described a series of patients with sCJD presenting with isolated language impairment. We report a patient with sCJD referred to the NCJDRSU, who presented with isolated language impairment and subsequently identified all cases of sporadic CJD on the NCJDRSU database (covering the years 1990-2012) with an isolated language impairment presentation. Nineteen patients (11 females) with sCJD (1.19% of all patients) had an isolated language disorder of at least 2 weeks duration as the first neurological symptom pattern. Mean age at onset was 68.28 years. No specific pattern of language affection was seen in these patients. Further progression usually affected more than one neurological domain, with all patients eventually developing cognitive decline and myoclonic jerks. The median duration of illness was 4 months. CSF 14.3.3 was positive and S100b level was elevated in all patients in whom it was performed. EEG and MRI showed typical features of sCJD in six patients each. Most patients showed MM genotype of PRNP codon 129. This study highlights the fact that isolated aphasia can be the first neurological symptom approximately in 1% of patients with sCJD. The diagnosis is usually made with appearance of other clinical features and investigation results, but in a small minority, these may not be apparent for relatively long periods. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Bioassay studies support the potential for iatrogenic transmission of variant Creutzfeldt Jakob Disease through dental procedures.

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    Elizabeth Kirby

    Full Text Available BACKGROUND: Evidence is required to quantify the potential risks of transmission of variant Creutzfeldt Jakob (vCJD through dental procedures. Studies, using animal models relevant to vCJD, were performed to address two questions. Firstly, whether oral tissues could become infectious following dietary exposure to BSE? Secondly, would a vCJD-contaminated dental instrument be able to transmit disease to another patient? METHODS: BSE-301V was used as a clinically relevant model for vCJD. VM-mice were challenged by injection of infected brain homogenate into the small intestine (Q1 or by five minute contact between a deliberately-contaminated dental file and the gingival margin (Q2. Ten tissues were collected from groups of challenged mice at three or four weekly intervals, respectively. Each tissue was pooled, homogenised and bioassayed in indicator mice. FINDINGS: Challenge via the small intestine gave a transmission rate of 100% (mean incubation 157±17 days. Infectivity was found in both dental pulp and the gingival margin within 3 weeks of challenge and was observed in all tissues tested within the oral cavity before the appearance of clinical symptoms. Following exposure to deliberately contaminated dental files, 97% of mice developed clinical disease (mean incubation 234±33 days. INTERPRETATION: Infectivity was higher than expected, in a wider range of oral tissues, than was allowed for in previous risk assessments. Disease was transmitted following transient exposure of the gingiva to a contaminated dental file. These observations provide evidence that dental procedures could be a route of cross-infection for vCJD and support the enforcement of single-use for certain dental instruments.

  2. Genetic and Transcriptomic Profiles of Inflammation in Neurodegenerative Diseases: Alzheimer, Parkinson, Creutzfeldt-Jakob and Tauopathies.

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    López González, Irene; Garcia-Esparcia, Paula; Llorens, Franc; Ferrer, Isidre

    2016-02-04

    Polymorphisms in certain inflammatory-related genes have been identified as putative differential risk factors of neurodegenerative diseases with abnormal protein aggregates, such as sporadic Alzheimer's disease (AD) and sporadic Parkinson's disease (sPD). Gene expression studies of cytokines and mediators of the immune response have been made in post-mortem human brain samples in AD, sPD, sporadic Creutzfeldt-Jakob disease (sCJD) subtypes MM1 and VV2, Pick's disease (PiD), progressive supranuclear palsy (PSP) and frontotemporal lobar degeneration linked to mutation P301L in MAPT Frontotemporal lobar degeneration-tau (FTLD-tau). The studies have disclosed variable gene regulation which is: (1) disease-dependent in the frontal cortex area 8 in AD, sPD, sCJD MM1 and VV2, PiD, PSP and FTLD-tau; (2) region-dependent as seen when comparing the entorhinal cortex, orbitofrontal cortex, and frontal cortex area 8 (FC) in AD; the substantia nigra, putamen, FC, and angular gyrus in PD, as well as the FC and cerebellum in sCJD; (3) genotype-dependent as seen considering sCJD MM1 and VV2; and (4) stage-dependent as seen in AD at different stages of disease progression. These observations show that regulation of inflammation is much more complicated and diverse than currently understood, and that new therapeutic approaches must be designed in order to selectively act on specific targets in particular diseases and at different time points of disease progression.

  3. Creutzfeldt-jakob, Parkinson, lewy body dementia and Alzheimer diseases: from diagnosis to therapy.

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    Dupiereux, Ingrid; Zorzi, Willy; Quadrio, Isabelle; Perret-Liaudet, Armand; Kovacs, Gabor G; Heinen, Ernst; Elmoualij, Benaïssa

    2009-03-01

    Depositions of proteins in form of amyloid and non-amyloid plaques are common pathogenic signs of more than 20 degenerative diseases affecting the central nervous system or a variety of peripheral tissues. Among the neuropathological conditions, Alzheimer's, Parkinson's and the prion diseases, such as Creutzfeldt-Jakob disease (CJD), present ambiguities as regarding their differential diagnosis. At present, their diagnosis must be confirmed by post-mortem examination of the brain. Currently the ante-mortem diagnosis is still based on the integration of multiple data (clinical, paraclinical and biological analyses) because no unique marker exists for such diseases. The detection of specific biomarkers would be useful to develop a differential diagnostic, distinguishing not only different neurodegenerative diseases but also the disease from the non-pathological effects of aging. Several neurodegenerative biomarkers are present at very low levels during the early stages of the disease development and their ultra-low detection is needed for early diagnosis, which should permit more effective therapeutic interventions, before the disease concerned can progress to a stage where considerable damage to the brain has already occurred. In the case of prion diseases, there are concerns regarding not only patient care, but the wider community too, with regard to the risk of transmission of prions, especially during blood transfusion, for which, four cases of variant CJD infection associated with transfusion of non-leukocyte-depleted blood components have been confirmed. Therefore the development of techniques with high sensitivity and specificity represent the major challenge in the field of the protein misfolding diseases. In this paper we review the current analytical and/or biochemical diagnostic technologies used mainly in prion, but also in Alzheimer and Parkinson diseases and emphasizing work on the protein detection as a surrogates and specific biomarker in the body

  4. Creutzfeldt-Jakob disease : report of 10 cases from North India.

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    Mehndiratta M

    2001-10-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is increasingly being reported over the last three decades as a result of heightened awareness of the disease. Various studies have reported annual incidence of 0.5-1.5 cases of CJD per million of general population. In India, the disease is still under reported. Over the period spanning from 1968-1997, National Institute of Mental Health and Neurosciences (NIMHANS, Bangalore recorded 69 cases of CJD from different parts of India in the CJD registry. This paper describes the clinical experience with cases of CJD managed at the Department of Neurology, G.B. Pant Hospital, New Delhi from 1990-1998. In this series, the mean age of the patients was 53.80 (+/- 7.32 years and there were 5 females and 5 males. Myoclonus was present in all the cases and abnormal behaviour with or without other features was the presenting complaint in 7 of the 10 patients, while one patient of CJD had cerebellar ataxia as the presenting feature. One patient with occipital variant of CJD presented with acute onset cortical blindness and myoclonic jerks. One of the patients had acute psychosis precipitated by emotional stress at the onset. Extrapyramidal features were noted in 7 of the 10 patients before death. The mean duration of symptoms from the onset of disease to death was 6.6 (+/- 6.11 months. Classical EEG changes were observed in all the patients, except in one possible case of occipital variant of CJD, where we did not have access to EEG record. Brain biopsy could be undertaken in 3 patients, and in 2 patients the features of subacute spongiform encephalopathy (SSE were noted.

  5. Myoclonus in Creutzfeldt-Jakob disease: polygraphic and video-electroencephalography assessment of 109 patients.

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    Binelli, Simona; Agazzi, Pamela; Canafoglia, Laura; Scaioli, Vidmer; Panzica, Ferruccio; Visani, Elisa; Di Fede, Giuseppe; Giaccone, Giorgio; Bizzi, Alberto; Bugiani, Orso; Avanzini, Guiliano; Tagliavini, Fabrizio; Franceschetti, Silvana

    2010-12-15

    We used electroencephalography (EEG)-polygraphic recordings to classify myoclonus in 109 patients with Creutzfeldt-Jakob disease (CJD) on the basis of its electromyography (EMG) pattern, time course, distribution, and EEG correlates. We recorded myoclonic jerks in 55 patients (50.4%), and we classified them as periodic myoclonus in 28, rhythmic in 13, and irregular in 20 (6 patients showed two types of myoclonus). Myoclonus occurred as a prominently negative event (interrupting the EMG discharge) in 10. Periodic sharp-wave complexes (PSWCs) were present in all but one patient with myoclonic jerks but were time-locked with EMG-bursts only in case of periodic myoclonus. Jerk-locked back averaging revealed a variable EEG-EMG transfer-time commonly exceeding that characterizing cortical myoclonus. Myoclonus was frequently associated with Met/Met polymorphism at codon 129 of the prion protein gene, but it was also observed in association with Met/Val or Val/Val polymorphisms provided that the EEG showed the presence of the PSWC pattern. The presence of enlarged somatosensory evoked potentials significantly correlated with the myoclonic presentation, as did MR signal hyperintensity involving the cortical mantle. Our observations on the basis of standard polygraphic criteria suggest that CJD associates with a remarkable variety of myoclonic jerks, and therefore different brain structures are probably involved as generators. The significant association between the presence of all myoclonus types with PSWCs suggests that hyperexcitable corticosubcortical loops are always required to generate (or allow) both myoclonus and the EEG complexes, either they are time locked or not.

  6. Detection of prions in blood from patients with variant Creutzfeldt-Jakob disease.

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    Concha-Marambio, Luis; Pritzkow, Sandra; Moda, Fabio; Tagliavini, Fabrizio; Ironside, James W; Schulz, Paul E; Soto, Claudio

    2016-12-21

    Human prion diseases are infectious and invariably fatal neurodegenerative diseases. They include sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant CJD (vCJD), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. Development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (PrP(Sc)) in the blood of patients affected by prion disease is a top medical priority to increase the safety of the blood supply. vCJD has already been transmitted from human to human by blood transfusion, and the number of asymptomatic carriers of vCJD in the U.K. alone is estimated to be 1 in 2000 people. We used the protein misfolding cyclic amplification (PMCA) technique to analyze blood samples from 14 cases of vCJD and 153 controls, including patients affected by sCJD and other neurodegenerative or neurological disorders as well as healthy subjects. Our results showed that PrP(Sc) could be detected with 100% sensitivity and specificity in blood samples from vCJD patients. Detection was possible in any of the blood fractions analyzed and could be done with as little as a few microliters of sample volume. The PrP(Sc) concentration in blood was estimated to be ~0.5 pg/ml. Our findings suggest that PMCA may be useful for premortem noninvasive diagnosis of vCJD and to identify prion contamination of the blood supply. Further studies are needed to fully validate the technology. Copyright © 2016, American Association for the Advancement of Science.

  7. Genetic cross-interaction between APOE and PRNP in sporadic Alzheimer's and Creutzfeldt-Jakob diseases.

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    Olga Calero

    Full Text Available Alzheimer's disease (AD and Creutzfeldt-Jakob disease (CJD represent two distinct clinical entities belonging to a wider group, generically named as conformational disorders that share common pathophysiologic mechanisms. It is well-established that the APOE ε4 allele and homozygosity at polymorphic codon 129 in the PRNP gene are the major genetic risk factors for AD and human prion diseases, respectively. However, the roles of PRNP in AD, and APOE in CJD are controversial. In this work, we investigated for the first time, APOE and PRNP genotypes simultaneously in 474 AD and 175 sporadic CJD (sCJD patients compared to a common control population of 335 subjects. Differences in genotype distribution between patients and control subjects were studied by logistic regression analysis using age and gender as covariates. The effect size of risk association and synergy factors were calculated using the logistic odds ratio estimates. Our data confirmed that the presence of APOE ε4 allele is associated with a higher risk of developing AD, while homozygosity at PRNP gene constitutes a risk for sCJD. Opposite, we found no association for PRNP with AD, nor for APOE with sCJD. Interestingly, when AD and sCJD patients were stratified according to their respective main risk genes (APOE for AD, and PRNP for sCJD, we found statistically significant associations for the other gene in those strata at higher previous risk. Synergy factor analysis showed a synergistic age-dependent interaction between APOE and PRNP in both AD (SF = 3.59, p = 0.027, and sCJD (SF = 7.26, p = 0.005. We propose that this statistical epistasis can partially explain divergent data from different association studies. Moreover, these results suggest that the genetic interaction between APOE and PRNP may have a biological correlate that is indicative of shared neurodegenerative pathways involved in AD and sCJD.

  8. Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics

    NARCIS (Netherlands)

    Cali, I.; Castellani, R.; Alshekhlee, A.; Cohen, Y.; Blevins, J.; Yuan, J.; Langeveld, J.P.M.; Parchi, P.; Safar, J.G.; Zou, W.Q.; Gambetti, P.

    2009-01-01

    Five phenotypically distinct subtypes have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionine/valine polymorphic genotype of codon 129 of the prion protein (PrP) gene and the presence of either one of the two protease K-resistant scrapie prion protein (PrPSc) types

  9. Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

    NARCIS (Netherlands)

    Parchi, P.; Strammiello, R.; Notari, S.; Giese, A.; Langeveld, J.P.M.; Ladogana, A.; Zerr, I.; Roncaroli, F.; Cras, P.; Ghetti, B.; Pocchiari, M.; Kretzschmar, H.; Capellari, S.

    2009-01-01

    Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects sho

  10. Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.

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    Wei Zhao

    Full Text Available OBJECTIVE: Sporadic Creutzfeldt-Jakob disease (sCJD is a fatal and transmissible neurodegenerative disorder. However, no studies have reported Chinese specific characteristics of sCJD. We aimed to identify differences in sCJD between Chinese patients and patients from other countries. METHODS: The data from 57 Chinese sCJD patients were retrospectively analyzed, including demographic data, clinical manifestations, laboratory examinations, electroencephalograms (EEGs, diffusion-weighted imaging (DWI scans, positron emission tomography (PET scans, and pathological results. RESULT: The disease was pathologically confirmed in 11 patients. 39 cases were diagnosed as probable sCJD, and 7 were possible. Of the total cases, 33 were male, and 24 were female. The onset age ranged from 36 to 75 years (mean: 55.5, median: 57. Disease onset before the age of 60 occurred in 57.9% of patients. The disease duration from onset to death ranged 5-22 months (mean: 11.6, median: 11, and 51.9% of patients died 7 to 12 months after disease onset. The majority of patients presented with sub-acute onset with progressive dementia. 3 of the 9 patients who took 14-3-3 protein analysis had positive results (33.3%. The sensitivity of EEG was 79.6% (43/54. For DWI and PET examinations, the sensitivities were 94% (47/50 and 94.1% (16/17, respectively. In seven patients who did not show typical hyper-intensities on the first DWI examination, abnormalities of hypo-metabolism in the cerebral cortex were clearly detected by PET. In 13 out of the 17 patients, PET detected extra abnormal regions in addition to the hyper-intense areas observed in DWI. CONCLUSION: This is the first study to indicate that Chinese sCJD patients have a much earlier onset age and a longer disease duration than other populations, which is most likely related to racial differences. The longer disease duration may also be a probable characteristic of Asian populations. PET had high sensitivity for the

  11. A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk.

    Science.gov (United States)

    Sanchez-Juan, Pascual; Bishop, Matthew T; Kovacs, Gabor G; Calero, Miguel; Aulchenko, Yurii S; Ladogana, Anna; Boyd, Alison; Lewis, Victoria; Ponto, Claudia; Calero, Olga; Poleggi, Anna; Carracedo, Ángel; van der Lee, Sven J; Ströbel, Thomas; Rivadeneira, Fernando; Hofman, Albert; Haïk, Stéphane; Combarros, Onofre; Berciano, José; Uitterlinden, Andre G; Collins, Steven J; Budka, Herbert; Brandel, Jean-Philippe; Laplanche, Jean Louis; Pocchiari, Maurizio; Zerr, Inga; Knight, Richard S G; Will, Robert G; van Duijn, Cornelia M

    2014-01-01

    We performed a genome-wide association (GWA) study in 434 sporadic Creutzfeldt-Jakob disease (sCJD) patients and 1939 controls from the United Kingdom, Germany and The Netherlands. The findings were replicated in an independent sample of 1109 sCJD and 2264 controls provided by a multinational consortium. From the initial GWA analysis we selected 23 SNPs for further genotyping in 1109 sCJD cases from seven different countries. Five SNPs were significantly associated with sCJD after correction for multiple testing. Subsequently these five SNPs were genotyped in 2264 controls. The pooled analysis, including 1543 sCJD cases and 4203 controls, yielded two genome wide significant results: rs6107516 (p-value=7.62x10-9) a variant tagging the prion protein gene (PRNP); and rs6951643 (p-value=1.66x10-8) tagging the Glutamate Receptor Metabotropic 8 gene (GRM8). Next we analysed the data stratifying by country of origin combining samples from the pooled analysis with genotypes from the 1000 Genomes Project and imputed genotypes from the Rotterdam Study (Total n=12967). The meta-analysis of the results showed that rs6107516 (p-value=3.00x10-8) and rs6951643 (p-value=3.91x10-5) remained as the two most significantly associated SNPs. Rs6951643 is located in an intronic region of GRM8, a gene that was additionally tagged by a cluster of 12 SNPs within our top100 ranked results. GRM8 encodes for mGluR8, a protein which belongs to the metabotropic glutamate receptor family, recently shown to be involved in the transduction of cellular signals triggered by the prion protein. Pathway enrichment analyses performed with both Ingenuity Pathway Analysis and ALIGATOR postulates glutamate receptor signalling as one of the main pathways associated with sCJD. In summary, we have detected GRM8 as a novel, non-PRNP, genome-wide significant marker associated with heightened disease risk, providing additional evidence supporting a role of glutamate receptors in sCJD pathogenesis.

  12. A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk.

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    Pascual Sanchez-Juan

    Full Text Available We performed a genome-wide association (GWA study in 434 sporadic Creutzfeldt-Jakob disease (sCJD patients and 1939 controls from the United Kingdom, Germany and The Netherlands. The findings were replicated in an independent sample of 1109 sCJD and 2264 controls provided by a multinational consortium. From the initial GWA analysis we selected 23 SNPs for further genotyping in 1109 sCJD cases from seven different countries. Five SNPs were significantly associated with sCJD after correction for multiple testing. Subsequently these five SNPs were genotyped in 2264 controls. The pooled analysis, including 1543 sCJD cases and 4203 controls, yielded two genome wide significant results: rs6107516 (p-value=7.62x10-9 a variant tagging the prion protein gene (PRNP; and rs6951643 (p-value=1.66x10-8 tagging the Glutamate Receptor Metabotropic 8 gene (GRM8. Next we analysed the data stratifying by country of origin combining samples from the pooled analysis with genotypes from the 1000 Genomes Project and imputed genotypes from the Rotterdam Study (Total n=12967. The meta-analysis of the results showed that rs6107516 (p-value=3.00x10-8 and rs6951643 (p-value=3.91x10-5 remained as the two most significantly associated SNPs. Rs6951643 is located in an intronic region of GRM8, a gene that was additionally tagged by a cluster of 12 SNPs within our top100 ranked results. GRM8 encodes for mGluR8, a protein which belongs to the metabotropic glutamate receptor family, recently shown to be involved in the transduction of cellular signals triggered by the prion protein. Pathway enrichment analyses performed with both Ingenuity Pathway Analysis and ALIGATOR postulates glutamate receptor signalling as one of the main pathways associated with sCJD. In summary, we have detected GRM8 as a novel, non-PRNP, genome-wide significant marker associated with heightened disease risk, providing additional evidence supporting a role of glutamate receptors in sCJD pathogenesis.

  13. Enfermedad de Creutzfeldt-Jakob por RMI: alteración cortical como signo temprano de la enfermedad Creutzfeldt-Jakob disease by MRI: Cortical alteration as early sign disease

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    María Fernanda Markarian

    2008-12-01

    Full Text Available Se estudió por RMI un paciente de 59 años con diagnóstico probable de Enfermedad de Creutzfeldt-Jakob desde el inicio de sus síntomas. El paciente comienza con un cuadro de leve deterioro cognitivo. En una primera resonancia en secuencias FLAIR se visualiza hiperintensidad cortical a predomino de hemisferio izquierdo, no observándose en FSE T 2. Se hace más significativa en nueva resonancia en FLAIR y Difusión, con aparición de hiperintensidad en cabeza de ambos caudados y rápido deterioro cognitivo, alteraciones visuales, aparición de signos piramidales y extrapiramidales, convulsiones y mioclonias y mutismo. Con la acentuación de las alteraciones corticales -y en los ganglios de la base en una tercera resonancia-, el paciente trasforma su ECG de ritmo lento a un ritmo de punta-onda bifásico y trifásico. A 3 meses de la primera resonancia, nuevas imágenes muestran atrofia e importante hiperintensidad cortical y en ganglios de la base. En conclusión, las secuencias FLAIR y Difusión serían más sensibles que las secuencias T2 en la detección del aumento de intensidad de señal en la corteza cerebral, siendo un indicio diagnóstico temprano de la enfermedad de Creutzfeld-Jakob.A 59-year-old man with probable Creutzfeldt-Jakob disease was studied from early symptoms. The patients manifested mild cognitive impairment. The first magnetic resonance showed hiperintense signal cortical abnormalities in FLAIR sequence predominantly in left hemisphere, FSE T2 no showed abnormalities. In other resonance those abnormities were more significative and appeared head of the caudate nucleus abnormalities in FLAIR and Diffusion-weighted, the patients began with rapidly progressing impairment, visual disturbance, pyramidal and extrapyramidal signs, seizures, myoclonus and mutism. The third resonance revealed cortical and basal ganglia high signal intensity abnormalities and the patient transformed slowing EEG to biphasic and triphasic sharp

  14. Doença de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de ressonância magnética e DWI Creutzfeldt-Jakob disease, Heidenhain variant: case report with MRI (DWI) findings

    OpenAIRE

    Walter Oleschko Arruda; Kelly C. Bordignon; Jerônimo B. Milano; Ricardo Ramina

    2004-01-01

    A doença de Creutzfeldt-Jakob (CJD) é uma forma de demência pré-senil de rápida evolução, geralmente fatal em um ano. Casos autóctones no Brasil têm sido raramente descritos assim como achados de ressonância magnética. Mulher, natural de Ponta Grossa PR, branca , 54 anos , foi admitida no serviço em outubro de 2001 com quadro de amaurose bilateral cortical progressiva desde há 1 mês do internamento. Nunca viajou ao exterior e foi somente submetida a uma cirurgia de redução do estômago, para o...

  15. New variant of Creutzfeldt-Jakob (vCJD disease and other human prion diseases under epidemiological surveillance in Brazil

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    Vera Lúcia Gattás

    Full Text Available Abstract To increase the timeliness of detection of human cases of the new variant of Creutzfeldt-Jakob disease (vCJD and to reduce the risk of transmission, the Brazilian Ministry of Health has established and standardized rules and control measures. These include the definition of criteria for suspect cases, reporting, monitoring, and control measures for illness prevention and transmission. Guidelines to be used by the team of health care staff were published and distributed to health workers. A detailed proposal for a simplified system of surveillance for prion diseases was developed and mandatory reporting introduced. Additional effort is necessary to increase vCJD case detection, thus making it necessary to establish a partnership with health care services for best identification of suspected cases and dissemination of information to all involved in the service dealing with vCJD investigation.

  16. Long term survival in a patient with variant Creutzfeldt-Jakob disease treated with intraventricular pentosan polysulphate.

    Science.gov (United States)

    Parry, A; Baker, I; Stacey, R; Wimalaratna, S

    2007-07-01

    Variant Creutzfeldt-Jakob disease (vCJD) is a neurodegenerative disease that principally affects young people and has a median duration of illness of 13 (range 6-39) months. vCJD is incurable and there are currently no treatments that conclusively slow the rate of disease progression. However, recent animal studies and isolated case reports have suggested that treatment with intraventricular pentosan polysulphate (PPS) may be beneficial in the treatment of patients with vCJD. We report a case of a 22-year-old male with vCJD treated 19 months after the onset of clinical symptoms with continuous intraventricular PPS (32 microg/kg/day) over a period of 31 months. Treatment with PPS appeared to be safe and well tolerated and was associated with prolonged survival (51 months) when compared to natural history studies. However, PPS treatment did not appear to arrest the progression of the disease.

  17. Creutzfeldt-Jakob disease in Japan: an epidemiological study done in a select prefecture between 1976 and 1986.

    Science.gov (United States)

    Akai, J; Ishihara, O; Higuchi, S

    1989-01-01

    An epidemiological study was performed with respect to Creutzfeldt-Jakob disease in a designated area in Japan. The subjects were observed in a small rural prefecture where the population generally remains within a limited radius throughout their lives. The patients' life-styles in each area were investigated in detail. Nine cases appeared in 11 years; 6 were definitive and 3 probable. They were all of the subacute type; there were no noteworthy sexual differences, age of onset, course and/or past histories. Three of the nine cases came from two families; the relationship between familial and isolated cases was examined. Revealed facts and time-space clustering were investigated statistically, but no indication of natural transmission was observable.

  18. Bitemporal hypometabolism in Creutzfeldt-Jakob disease measured by positron emission tomography with (/sup 18/F)-2-fluorodeoxyglucose

    Energy Technology Data Exchange (ETDEWEB)

    Friedland, R.P.; Prusiner, S.B.; Jagust, W.J.; Budinger, T.F.; Davis, R.L.

    1984-10-01

    It is well established that Creutzfeldt-Jakob disease (CJD) is caused by a slow infectious agent similar to the scrapie prion. However, the pathogenesis of this infection is poorly understood. Positron emission tomography (PET) was performed on a 54-year-old man with autopsy confirmed CJD using (18F)-2-fluorodeoxyglucose (FDG) and the Donner 280-crystal tomograph. Temporal lobe hypometabolism with hemispheric asymmetry was observed. These findings are similar to those previously obtained in PET-FDG studies of patients with clinically defined Alzheimer disease (AD). The similarities in the regional metabolic alterations between CJD and AD provide additional evidence for the possibility that AD may be caused by a slow infectious prion.

  19. Clinical analysis of early-stage misdiagnosis of Creutzfeldt-Jakob disease%克雅病早期误诊临床分析

    Institute of Scientific and Technical Information of China (English)

    吴杰贤; 梁颖茵; 姚晓黎

    2011-01-01

    目的 提高散发性克雅病临床诊断水平,降低其误诊率.方法 回顾性分析11例克雅病患者临床表现及早期误诊情况.结果 11例克雅病患者早期误诊为阿尔茨海默病4例,病毒性脑炎3例,脑梗死2例,路易体痴呆2例,脑脊液14-3-3蛋白阳性6例.结论 克雅病的早期临床表现极不典型,较易误诊;多次复查脑电图、磁共振弥散加权成像、脑脊液14-3-3蛋白是诊断本病的重要手段.%Objective To improve the clinical practical diagnosis of sporadic Creutzfeldt-Jakob disease and decrease the probability of misdiagnosis.Methods Clinical manifestations of 11 cases of Creutzfeldt-Jakob disease misdiagnosed in the early stage were reviewed.Results Eleven cases of Creutzfeldt-Jakob disease were misdiagnosed as Alzheimer' s disease (4 cases), viral encephalitis (3 cases),cerebral infarction (2 cases) and dementia with Lewy bodies (2 cases) in the early stage.Cerebrospinal fluid 14-3-3 protein was positive in 6 cases.Conclusions The clinical manifestations of Creutzfeldt- Jakob disease in the early stage are atypical and tend to be misdiagnosed;repetitive EEG,diffusion weighted imaging and cerebrospinal fluid 14-3-3 protein are essential to diagnosis.

  20. Stereotypic Movements in Case of Sporadic Creutzfeldt-Jakob Disease: Possible Role of Anti-NMDA Receptor Antibodies

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    Michelle Molina

    2012-12-01

    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD and anti-NMDA receptor antibody encephalitis (NMDAE can both produce a rapidly progressive dementia with resulting state of catatonia or akinetic mutism. Both are associated with movement disorders. In published case series, myoclonus appears to be the most frequent movement disorder in sCJD, while stereotypic, synchronized, one-cycle-per-second movements such as arm or leg elevation, jaw opening, grimacing, head turning, and eye deviation are seen in NMDAE. We report a case of a 59-year-old woman with rapidly worsening cognitive disturbance leading to a nearly catatonic state interrupted by stereotypic movements. sCJD was diagnosed via periodic sharp wave complexes on EEG as well as cerebrospinal fluid (CSF 14-3-3 and tau protein elevation. Characteristic movement disorder of NMDAE was present in absence of ovarian mass or CSF pleiocytosis. Given prior case reports of presence of anti-NMDA receptor antibodies in sCJD, we propose that the movement disorder in this case was caused by anti-NMDA receptor antibodies whose formation was secondary to neuronal damage from prion disease. It is important to consider sCJD even in cases that have some clinical features suggestive of NMDAE.

  1. Spreading brain lesions in a familial Creutzfeldt-Jakob disease with V180I mutation over 4 years

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    Deguchi Kentaro

    2012-11-01

    Full Text Available Abstract Background We report a female patient with familial Creutzfeldt-Jakob disease with V180I mutation (fCJD with V180I, who was serially followed up with magnetic resonance imaging (MRI and electroencephalogram (EEG for up to four years. Case presentation At 6 months after the onset, diffusion-weighted images (DWI and fluid-attenuated inversion recovery (FLAIR of brain MRI revealed an increased signal intensity in the bilateral frontal, temporal, and parietal cerebral cortex with left dominancy except for the occipital lobe. However, her follow-up MRI at four years showed the high-signal regions spreading to the occipital cerebral cortex in DWI and FLAIR images, and bilateral frontal cerebral white matter in FLAIR images. EEG showed a progressive and general slow high-voltage rhythm from 7–8 to 3–5 c/s over four years, without evidence of periodic synchronous discharge. These findings correspond to the symptom progression even after akinetic mutism at 18 months. Conclusion We suggest that serial MRI and EEG examinations are useful for early diagnosis of fCJD with V180I and for monitoring disease progression.

  2. Racial and ethnic differences in individuals with sporadic Creutzfeldt-jakob disease in the United States of America.

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    Brian S Appleby

    Full Text Available BACKGROUND: Little is known about racial and ethnic differences in individuals with sporadic Creutzfeldt-Jakob disease (sCJD. The authors sought to examine potential clinical, diagnostic, genetic, and neuropathological differences in sCJD patients of different races/ethnicities. METHODOLOGY/PRINCIPAL FINDINGS: A retrospective study of 116 definite and probable sCJD cases from Johns Hopkins and the Department of Veterans Affairs Healthcare Systems was conducted that examined differences in demographic, clinical, diagnostic, genetic, and neuropathological characteristics among racial/ethnic groups. Age at disease onset differed among racial/ethnic groups. Non-Hispanic Whites had a significantly older age at disease onset compared to the other groups (65 vs. 60, p = 0.036. Non-Whites were accurately diagnosed more rapidly than Whites (p = 0.008 and non-Hispanic Whites were more likely to have normal appearing basal ganglia on brain magnetic resonance imaging (MRI compared to minorities (p = 0.02. Whites were also more likely to undergo post-mortem evaluation compared to non-Whites (p = 0.02. CONCLUSIONS/SIGNIFICANCE: Racial/ethnic groups affected by sCJD demonstrated differences in age at disease onset, time to correct diagnosis, clinical presentation, and diagnostic test results. Whites were more likely to undergo autopsy compared to non-Whites. These results have implications in regards to case ascertainment, diagnosis, and surveillance of sCJD and possibly other human prion diseases.

  3. Sporadic Creutzfeldt-Jakob disease: the extent of microglia activation is dependent on the biochemical type of PrPSc.

    Science.gov (United States)

    Puoti, Gianfranco; Giaccone, Giorgio; Mangieri, Michela; Limido, Lucia; Fociani, Paolo; Zerbi, Pietro; Suardi, Silvia; Rossi, Giacomina; Iussich, Selina; Capobianco, Raffaella; Di Fede, Giuseppe; Marcon, Gabriella; Cotrufo, Roberto; Filippini, Graziella; Bugiani, Orso; Tagliavini, Fabrizio

    2005-10-01

    In prion-related encephalopathies, microglial activation occurs early and is dependent on accumulation of disease-specific forms of the prion protein (PrPSc) and may play a role in nerve cell death. Previously, we found that different types of PrPSc (i.e. type 1 and type 2) coexisted in approximately 25% of patients with sporadic Creutzfeldt-Jakob disease (CJD); and a close relationship was detected between PrPSc type, the pattern of PrP immunoreactivity, and extent of spongiform degeneration. To investigate whether microglial reaction is related to the biochemical type and deposition pattern of PrPSc, we carried out a neuropathologic and biochemical study on 26 patients with sporadic CJD, including all possible genotypes at codon 129 of the prion protein gene. By quantitative analysis, we demonstrated that strong microglial activation was associated with type 1 PrPSc and diffuse PrP immunoreactivity, whereas type 2 PrPSc and focal PrP deposits were accompanied by mild microglia reaction. These findings support the view that the phenotypic heterogeneity of sporadic CJD is largely determined by the physicochemical properties of distinct PrPSc conformers.

  4. Bitemporal hypometabolism in Creutzfeldt-Jakob Disease measured by positron emission tomography with (F-18)2-fluorodeoxyglucose

    Energy Technology Data Exchange (ETDEWEB)

    Friedland, R.P.; Budinger, T.F.; Prusiner, S.B.; Jagust, W.J.

    1984-01-01

    It is well established that Creutzfeldt-Jakob Disease (CJD) is caused by a slow infectious agent similar to the scrapie prion. However, the pathogenesis of this infection is poorly understood. Positron emission tomography (PET) was performed on a 54 year old male subject with autopsy confirmed CJD using (F-18)2-fluorodeoxyglucose (FDG) and the Donner 280-crystal tomograph. An x-ray computed tomographic study of the brain performed 4 days prior to PET was normal. In the PET study the frontal to temporal cortex difference of activity densities was 30% on the left and 12% on the right, reflecting temporal hypometabolism. The left-right temporal cortex difference of activity density was 25%, documenting marked hemispheric asymmetry. These findings are similar to those previously obtained in PET-FDG studies of patients with clinically defined Alzheimer's Disease (AD) and are distinctly different from PET-FDG finding in patients with other dementing illnesses or in healthy aged subjects. Recent work has demonstrated extensive biological similarities between CJD, scrapie and AD. The similarities in the regional metabolic alterations between CJD and AD provide additional evidence for the hypothesis that AD is caused by a slow infectious (prion-like) pathogen.

  5. Diffusion-Weighted MRI in Creutzfeldt-Jakob Disease: Focus on the Cerebral Cortex and Chronologic Change

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong Eun; Song, Chang Joon; Lee, In Ho [Chungnam National University, Daejeon (Korea, Republic of); Yu, In Kyu [Eulji University Hospital, Seoul (Korea, Republic of); Choi, See Sung [Wonkwang University Hospital, Iksan (Korea, Republic of)

    2010-08-15

    To evaluate high cortical signal intensity and chronologic changes for diffusion-weighted MR imaging (DWI) in sporadic Creutzfeldt-Jakob disease. We retrospectively analyzed the DWI results of 16 patients with probable CJD (according to WHO criteria) and evaluated the distribution, extent and bilaterality of the lesions in the cortex, basal ganglia and thalamus. We also reviewed the chronologic changes of the lesions by evaluating the followup MR examination results in 8 of 16 patients. Cortical abnormalities were present in 15 (94%) of 16 patients. Isolated cortical involvement was present in 6 patients (40%), while the combined involvement of the cortex and basal ganglia was present in 9 patients (60%). The distribution of the lesions was bilateral in 12 patients and predominantly on the right side in 8 patients. Upon follow-up MR imaging, the cortical lesions showed progress in terms of extent and signal intensity. Basal ganglia abnormalities were present in 9 of 15 patients. Moreover, 4 of 6 patients who had no abnormal signal intensity in the basal ganglia on the initial MR imaging results, showed abnormally high signal intensity upon follow-up MR imaging. The characteristically high cortical signal intensities on DWI in an elderly patient with rapidly progressive dementia should point to the diagnosis of early phase CJD and might be useful for the differential diagnosis.

  6. Sporadic Creutzfeldt-Jakob Disease: Prion Pathology in Medulla Oblongata—Possible Routes of Infection and Host Susceptibility

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    Diego Iacono

    2015-01-01

    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD, the most frequent human prion disorder, is characterized by remarkable phenotypic variability, which is influenced by the conformation of the pathologic prion protein and the methionine/valine polymorphic codon 129 of the prion protein gene. While the etiology of sCJD remains unknown, it has been hypothesized that environmental exposure to prions might occur through conjunctival/mucosal contact, oral ingestion, inhalation, or simultaneous involvement of the olfactory and enteric systems. We studied 21 subjects with definite sCJD to assess neuropathological involvement of the dorsal motor nucleus of the vagus and other medullary nuclei and to evaluate possible associations with codon 129 genotype and prion protein conformation. The present data show that prion protein deposition was detected in medullary nuclei of distinct sCJD subtypes, either valine homozygous or heterozygous at codon 129. These findings suggest that an “environmental exposure” might occur, supporting the hypothesis that external sources of contamination could contribute to sCJD in susceptible hosts. Furthermore, these novel data could shed the light on possible causes of sCJD through a “triple match” hypothesis that identify environmental exposure, host genotype, and direct exposure of specific anatomical regions as possible pathogenetic factors.

  7. [Perioperative considerations for performing a brain biopsy on a patient with subtype VV2 sporadic Creutzfeldt-Jakob disease].

    Science.gov (United States)

    Guerrero-Domínguez, R; Rubio-Romero, R; González-González, G; Jiménez, I

    2015-04-01

    Creutzfeldt-Jakob disease (CJD) is the most common transmissible spongiform encephalopathy. It is an infectious, progressive, degenerative neurological disorder, with a presumably long incubation period, but a rapid fatal course. CJD is transmitted by a proteinaceous infectious agent, or «prion». Because the prions are difficult to eradicate and are resistant to the currently used sterilization methods, special precautions must be taken with all surgical instruments. It is recommended the single-use equipment, destruction of contaminated equipment, decontamination of reusable instruments, use of protective clothing, and storing and quarantining surgical instruments. The single-use equipment and some tissues and body fluids from the patient with CJD are highly infectious and must be incinerated. We report a case of a patient who had undergone brain biopsy for suspected of CJD, being confirmed to have sporadic CJD. Specific preventive measures were taken to reduce the risk of transmission to healthcare workers. Copyright © 2014 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Does Poor Dental Health Have a Role in the Emergence of Variant Creutzfeldt Jakob Disease in the United Kingdom?

    Directory of Open Access Journals (Sweden)

    Robert Burnie

    2011-05-01

    Full Text Available Introduction: Variant creutzfeldt jakob disease (vCJD is the hu-man neurological disease known to be caused by the same proteinaceous infectious agent (“prion” that causes Bovine Spongiform Encephalopathy or "Mad Cow Disease". Two un-usual and unexplained characteristics of the vCJD epidemic are its geographical distribution within the UK (about twice as frequent in Scotland and Northern England and its median age of onset of 26 years that has remained unchanged over the fifteen years of the epidemic.The hypothesis: Infection via the dental route as a consequence of poor dental health, most probably the presence of untreated decay may account for the geographical distribution of vCJD in the UK and offer an explanation for the constant median age of onset of the dis-ease by representing a fixed stage in development.Evaluation of the hypothesis: Analysis of existing data indicates that vCJD incidence by region and an index of dental health by region are positively correlated (r=0.737, p= 0.015. The hypothesis that infection via the dental route may explain the constant median age of onset and geographical distribution of vCJD could be investigated further with a case control study based on individual dental records and by further animal experiments to confirm the biological plausibility of this route.

  9. MM2-thalamic Creutzfeldt-Jakob disease: neuropathological, biochemical and transmission studies identify a distinctive prion strain.

    Science.gov (United States)

    Moda, Fabio; Suardi, Silvia; Di Fede, Giuseppe; Indaco, Antonio; Limido, Lucia; Vimercati, Chiara; Ruggerone, Margherita; Campagnani, Ilaria; Langeveld, Jan; Terruzzi, Alessandro; Brambilla, Antonio; Zerbi, Pietro; Fociani, Paolo; Bishop, Matthew T; Will, Robert G; Manson, Jean C; Giaccone, Giorgio; Tagliavini, Fabrizio

    2012-09-01

    In Creutzfeldt-Jakob disease (CJD), molecular typing based on the size of the protease resistant core of the disease-associated prion protein (PrP(Sc) ) and the M/V polymorphism at codon 129 of the PRNP gene correlates with the clinico-pathologic subtypes. Approximately 95% of the sporadic 129MM CJD patients are characterized by cerebral deposition of type 1 PrP(Sc) and correspond to the classic clinical CJD phenotype. The rare 129MM CJD patients with type 2 PrP(Sc) are further subdivided in a cortical and a thalamic form also indicated as sporadic fatal insomnia. We observed two young patients with MM2-thalamic CJD. Main neuropathological features were diffuse, synaptic PrP immunoreactivity in the cerebral cortex and severe neuronal loss and gliosis in the thalamus and olivary nucleus. Western blot analysis showed the presence of type 2A PrP(Sc) . Challenge of transgenic mice expressing 129MM human PrP showed that MM2-thalamic sporadic CJD (sCJD) was able to transmit the disease, at variance with MM2-cortical sCJD. The affected mice showed deposition of type 2A PrP(Sc) , a scenario that is unprecedented in this mouse line. These data indicate that MM2-thalamic sCJD is caused by a prion strain distinct from the other sCJD subtypes including the MM2-cortical form. © 2012 The Authors; Brain Pathology © 2012 International Society of Neuropathology.

  10. Creutzfeldt-Jakob disease, Heidenhain variant: case report with MRI (DWI) findings; Doenca de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de ressonancia magnetica e DWI

    Energy Technology Data Exchange (ETDEWEB)

    Arruda, Walter Oleschko; Bordignon, Kelly C.; Milano, Jeronimo B.; Ramina, Ricardo [Instituto de Neurologia de Curitiba, PR (Brazil)]. E-mail: warruda@speednet.com.br

    2004-06-01

    Creutzfeldt-Jakob disease (CJD) is a pre senile dementia characterized by rapidly progressive mental deterioration, myoclonic jerking, and other less common neurological signs. Few accentuates cases have been described in Brazil. A 54-year-old white woman, was admitted in our service with a month history of progressive, bilateral cortical blindness. After admission, she developed right partial motor seizures (right facial, upper and lower limbs), she became progressively aphasic (mixed aphasia). Seizures were controlled with phenytoine, but she developed choreoathetotic movements on her right dimidium, with partial control after introduction of chlorpromazine 25 mg q/d. She could no longer stand up or walk due to severe ataxia. The first EEG (October, 2001) showed left hemisphere severe seizure activity (status epilepticus partial is). She was delivered home with enteral nutrition, phenytoine, chlorpromazine and mepacrine 100 mg q d. The following laboratory tests were negative or normal: blood series, platelets, ESR, kidney and liver function, copper, ceruloplasmin, Vedril, HIV, HTLV-1, lactate, and cerebral Dsa (performed in other service). A spinal tap with normal opening pressure was perform and CSFR examination was normal. CSFR 14-3-3 protein was positive, CSF specific neuronal enolase 7.5 ng/ml(normal). Genetic study of PRNP gene did not disclosed any known mutation. A MRI (October, 2001) showed areas of hyperintense signal (T 2 and FLAIR) without Gd-enhancement on T1, in the left temporal lobe and in both occipital lobes; basal ganglia have a normal appearance. DWI imaging showed bright areas at the same sites. An EEG (March, 2002) disclosed a periodical sharp triphasic waves pattern, suggestive of CJD. A second MRI (April, 2002) showed mild generalized atrophy, no ventricular dilatation, and the hyperintense sites disappeared. She remained clinically stable and under use of chlorpromazine and mepacrine until she died due to pulmonary complications on April

  11. Cerebrospinal fluid real-time quaking-induced conversion is a robust and reliable test for sporadic creutzfeldt-jakob disease: An international study.

    Science.gov (United States)

    McGuire, Lynne I; Poleggi, Anna; Poggiolini, Ilaria; Suardi, Silvia; Grznarova, Katarina; Shi, Song; de Vil, Bart; Sarros, Shannon; Satoh, Katsuya; Cheng, Keding; Cramm, Maria; Fairfoul, Graham; Schmitz, Matthias; Zerr, Inga; Cras, Patrick; Equestre, Michele; Tagliavini, Fabrizio; Atarashi, Ryuichiro; Knox, David; Collins, Steven; Haïk, Stéphane; Parchi, Piero; Pocchiari, Maurizio; Green, Alison

    2016-07-01

    Real-time quaking-induced conversion (RT-QuIC) has been proposed as a sensitive diagnostic test for sporadic Creutzfeldt-Jakob disease; however, before this assay can be introduced into clinical practice, its reliability and reproducibility need to be demonstrated. Two international ring trials were undertaken in which a set of 25 cerebrospinal fluid samples were analyzed by a total of 11 different centers using a range of recombinant prion protein substrates and instrumentation. The results show almost complete concordance between the centers and demonstrate that RT-QuIC is a suitably reliable and robust technique for clinical practice. Ann Neurol 2016;80:160-165.

  12. Diffusion-weighted imaging and magnetic resonance spectroscopy of sporadic Creutzfeldt-Jakob disease: correlation with clinical course

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Hyoung; Choi, Byung Se; Jung, Cheolkyu [Seoul National University Bundang Hospital, Department of Radiology, Seoul National University College of Medicine, Seongnam-si (Korea, Republic of); Chang, YoungHee; Kim, SangYun [Seoul National University Bundang Hospital, Department of Neurology, Seoul National University College of Medicine, Seongnam-si (Korea, Republic of)

    2011-12-15

    Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal disease with variable clinical courses. The presence or absence of basal ganglia (BG) involvement has been reported to be associated with clinical course. We investigated the association of clinical course of sCJD with diffusion-weighted imaging (DWI) and MR spectroscopy (MRS) as well as BG involvement at early stage. DWI and single voxel proton MRS were performed in 14 patients with sCJD during the initial diagnostic workup. Apparent diffusion coefficient (ADC) and metabolites were measured in medial occipitoparietal cortices where large hyperintense DWI lesions were found in all patients. The presence or absence of BG involvement, ADC, N-acetylaspartate (NAA)/creatine (Cr) ratios, and choline (Cho)/Cr ratios were correlated with disease duration (i.e., the time from the symptom onset to death). The disease duration ranged from 2 to 31 months (median, 16). Hyperintense DWI lesions were observed bilaterally in both cortices and basal ganglia in eight patients and in cortices alone in six patients. Patients with BG involvement had shorter disease duration (median, 6.8 versus 20.5; p = 0.039) than those without and lower NAA/Cr ratios (median, 1.41 versus 2.03; p = 0.001). ADC and Cho/Cr ratios were not significantly different between the patients with BG involvement and those without. By multiple regression analysis, NAA/Cr ratios had the greatest correlation with the disease duration (p = 0.029). The disease duration of sCJD was variable. NAA/Cr ratios of the affected brain at the early stage of sCJD can be used as a useful parameter in predicting the clinical course. (orig.)

  13. Genomic Characteristics of Genetic Creutzfeldt-Jakob Disease Patients with V180I Mutation and Associations with Other Neurodegenerative Disorders.

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    Sol Moe Lee

    Full Text Available Inherited prion diseases (IPDs, including genetic Creutzfeldt-Jakob disease (gCJD, account for 10-15% of cases of prion diseases and are associated with several pathogenic mutations, including P102L, V180I, and E200K, in the prion protein gene (PRNP. The valine to isoleucine substitution at codon 180 (V180I of PRNP is the most common pathogenic mutation causing gCJD in East Asian patients. In this study, we conducted follow-up analyses to identify candidate factors and their associations with disease onset. Whole-genome sequencing (WGS data of five gCJD patients with V180I mutation and 145 healthy individuals were used to identify genomic differences. A total of 18,648,850 candidate variants were observed in only the patient group, 29 of them were validated as variants. Four of these validated variants were nonsense mutations, six were observed in genes directly or indirectly related to neurodegenerative disorders (NDs, such as LPA, LRRK2, and FGF20. More than half of validated variants were categorized in Gene Ontology (GO terms of binding and/or catalytic activity. Moreover, we found differential genome variants in gCJD patients with V180I mutation, including one uniquely surviving 10 years after diagnosis of the disease. Elucidation of the relationships between gCJD and Alzheimer's disease or Parkinson's disease at the genomic level will facilitate further advances in our understanding of the specific mechanisms mediating the pathogenesis of NDs and gold standard therapies for NDs.

  14. Consumption of bovine spongiform encephalopathy (BSE) contaminated beef and the risk of variant Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Chen, Chu-Chih; Wang, Yin-Han; Wu, Kuen-Yuh

    2013-11-01

    To date, the variant Creutzfeldt-Jakob disease (vCJD) risk assessments that have been performed have primarily focused on predicting future vCJD cases in the United Kingdom, which underwent a bovine spongiform encephalopathy (BSE) epidemic between 1980 and 1996. Surveillance of potential BSE cases was also used to assess vCJD risk, especially in other BSE-prevalent EU countries. However, little is known about the vCJD risk for uninfected individuals who accidentally consume BSE-contaminated meat products in or imported from a country with prevalent BSE. In this article, taking into account the biological mechanism of abnormal prion PrP(res) aggregation in the brain, the probability of exposure, and the expected amount of ingested infectivity, we establish a stochastic mean exponential growth model of lifetime exposure through dietary intake. Given the findings that BSE agents behave similarly in humans and macaques, we obtained parameter estimates from experimental macaque data. We then estimated the accumulation of abnormal prions to assess lifetime risk of developing clinical signs of vCJD. Based on the observed number of vCJD cases and the estimated number of exposed individuals during the BSE epidemic period from 1980 to 1996 in the United Kingdom, an exposure threshold hypothesis is proposed. Given the age-specific risk of infection, the hypothesis explains the observations very well from an extreme-value distribution fitting of the estimated BSE infectivity exposure. The current BSE statistics in the United Kingdom are provided as an example.

  15. Characterization of variant Creutzfeldt-Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes.

    Science.gov (United States)

    Takeuchi, Atsuko; Kobayashi, Atsushi; Ironside, James W; Mohri, Shirou; Kitamoto, Tetsuyuki

    2013-07-26

    To date, all clinical variant Creutzfeldt-Jakob disease (vCJD) patients are homozygous for methionine at polymorphic codon 129 (129M/M) of the prion protein (PrP) gene. However, the appearance of asymptomatic secondary vCJD infection in individuals with a PRNP codon 129 genotype other than M/M and transmission studies using animal models have raised the concern that all humans might be susceptible to vCJD prions, especially via secondary infection. To reevaluate this possibility and to analyze in detail the transmission properties of vCJD prions to transgenic animals carrying distinct codon 129 genotype, we performed intracerebral inoculation of vCJD prions to humanized knock-in mice carrying all possible codon 129 genotypes (129M/M, 129M/V, or 129V/V). All humanized knock-in mouse lines were susceptible to vCJD infection, although the attack rate gradually decreased from 129M/M to 129M/V and to 129V/V. The amount of PrP deposition including florid/amyloid plaques in the brain also gradually decreased from 129M/M to 129M/V and to 129V/V. The biochemical properties of protease-resistant abnormal PrP in the brain and transmissibility of these humanized mouse-passaged vCJD prions upon subpassage into knock-in mice expressing bovine PrP were not affected by the codon 129 genotype. These results indicate that individuals with the 129V/V genotype may be more susceptible to secondary vCJD infection than expected and may lack the neuropathological characteristics observed in vCJD patients with the 129M/M genotype. Besides the molecular typing of protease-resistant PrP in the brain, transmission studies using knock-in mice carrying bovine PrP may aid the differential diagnosis of secondary vCJD infection, especially in individuals with the 129V/V genotype.

  16. Doença de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de ressonância magnética e DWI Creutzfeldt-Jakob disease, Heidenhain variant: case report with MRI (DWI findings

    Directory of Open Access Journals (Sweden)

    Walter Oleschko Arruda

    2004-06-01

    Full Text Available A doença de Creutzfeldt-Jakob (CJD é uma forma de demência pré-senil de rápida evolução, geralmente fatal em um ano. Casos autóctones no Brasil têm sido raramente descritos assim como achados de ressonância magnética. Mulher, natural de Ponta Grossa PR, branca , 54 anos , foi admitida no serviço em outubro de 2001 com quadro de amaurose bilateral cortical progressiva desde há 1 mês do internamento. Nunca viajou ao exterior e foi somente submetida a uma cirurgia de redução do estômago, para obesidade. História familial sem relato de casos semelhantes. Logo após o internamento a paciente desenvolveu quadro de disfasia mista, hemiparesia flácida direita, com movimentos coreoatetóticos e crises parciais motoras. Paciente evoluiu com quadro demencial progressivo; atualmente, acamada, torporosa, dependente de alimentação enteral, recebendo mepacrina, fenitoína e clorpromazina , estabilizando o quadro até final de maio de 2002. Exames laboratoriais negativos ou normais. Pesquisa de proteína 14-3-3 no líquor foi positiva; enolase-neurônio-específica no líquor foi normal. Estudo genético do gen PRNP não revelou mutação descrita anteriormente. EEG (23/10/2001 revelou intensa atividade irritativa hemisfério cerebral esquerdo. Estudo de ressonância magnética revelou áreas de hipersinal em T2 e FLAIR em regiões temporal esquerda e bioccipital; gânglios da base normal. Imagens de DWI mostraram hipersinal nas mesmas áreas.Outro EEG (15/03/2002 revelou padrão periódico de ondas trifásicas sugestivos de CJD. A paciente fez uso de mepacrina associado a clorpromazina com aparente estabilização do quadro, até seu óbito por complicações infecciosas pulmonares em abril de 2003.Creutzfeldt-Jakob disease (CJD is a presenile dementia characterized by rapidly progressive mental deterioration, myoclonic jerking, and other less common neurological signs. Few autoctonous cases have been described in Brazil. A 54-year

  17. Alien hand and leg as the presenting feature of probable sporadic Creutzfeldt-Jakob disease: A rare presentation of a rare disease

    Directory of Open Access Journals (Sweden)

    Banshi Lal Kumawat

    2015-01-01

    Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD can have varied clinical presentation depending upon the genotype at codon 129. The common presenting clinical features of sCJD are rapid onset cognitive impairment, ataxia, psychosis and visual signs (field defects, distortion, cortical blindness. Alien limb sign was first described in patients with corpus callosal tumors and later with other neurodegenerative conditions like corticobasal degeneration. Alien hand complaints as the presenting feature of sCJD has been described in literature, but simultaneous alien hand and leg has been rarely described as presenting feature of sCJD. We describe here a case of a 55-year-old man who presented with progressive left alien hand and leg as the sole clinical manifestation of probable sCJD.

  18. Doença de Creutzfeldt-Jakob forma Heidenhain: relato de caso com achados de ressonância magnética e DWI

    OpenAIRE

    Arruda Walter Oleschko; Bordignon Kelly C.; Milano Jerônimo B.; Ramina Ricardo

    2004-01-01

    A doença de Creutzfeldt-Jakob (CJD) é uma forma de demência pré-senil de rápida evolução, geralmente fatal em um ano. Casos autóctones no Brasil têm sido raramente descritos assim como achados de ressonância magnética. Mulher, natural de Ponta Grossa PR, branca , 54 anos , foi admitida no serviço em outubro de 2001 com quadro de amaurose bilateral cortical progressiva desde há 1 mês do internamento. Nunca viajou ao exterior e foi somente submetida a uma cirurgia de redução do estômago, para o...

  19. Alien hand and leg as the presenting feature of probable sporadic Creutzfeldt-Jakob disease: A rare presentation of a rare disease

    Science.gov (United States)

    Kumawat, Banshi Lal; Sharma, Chandra Mohan; Nath, Kunal; Acharya, Mihir; Khandelwal, Dinesh; Jain, Deepak

    2015-01-01

    Sporadic Creutzfeldt-Jakob disease (sCJD) can have varied clinical presentation depending upon the genotype at codon 129. The common presenting clinical features of sCJD are rapid onset cognitive impairment, ataxia, psychosis and visual signs (field defects, distortion, cortical blindness). Alien limb sign was first described in patients with corpus callosal tumors and later with other neurodegenerative conditions like corticobasal degeneration. Alien hand complaints as the presenting feature of sCJD has been described in literature, but simultaneous alien hand and leg has been rarely described as presenting feature of sCJD. We describe here a case of a 55-year-old man who presented with progressive left alien hand and leg as the sole clinical manifestation of probable sCJD. PMID:25745324

  20. Creutzfeldt-Jakob disease

    Science.gov (United States)

    ... part of a funeral ritual) Scrapie (found in sheep) Other very rare inherited human diseases, such as ... markers that sometimes occur with the disease CT scan of the brain Electroencephalogram (EEG) MRI of the ...

  1. Creutzfeldt-Jakob Disease

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    ... newsletter Stay up-to-date on the latest advances in Alzheimer's and dementia treatments, care and research. Subscribe now ... up-to-date on the latest news and advances in Alzheimer's treatments, care and research. Get tips for living ...

  2. Creutzfeldt-Jakob Disease

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    ... to as “mad cow” disease; scrapie, which affects sheep and goats; mink encephalopathy; and feline encephalopathy. Similar ... to move and speak and enter a coma. Pneumonia and other infections often occur in these individuals ...

  3. Doença de Creutzfeldt-Jakob: considerações clínicas, eletrencefalográficas e anatomopatológicas a propósito de uma caso Creutzfeldt-Jakob disease: a case report with clinical, electroencephalographic and neuropathological aspects

    Directory of Open Access Journals (Sweden)

    Wilson Luiz Sanvito

    1971-03-01

    Full Text Available É relatado um caso da doença de Creutzfeldt-Jakob cujo diagnóstico foi comprovado mediante estudo anátomo-patológico. São analisadas as diversas formas clínicas da doença e particular ênfase é dada aos aspectos eletrencefalográficos. No que respeita aos aspectos neuropatológicos é ressaltada a importância, para o diagnóstico, da presença de degeneração neuronal ao lado de hipertrofia da astroglia; o estado espongioso pode ocorrer em elevado número de casos. O paciente do presente registro, do sexo masculino, apresentou aos 52 anos de idade um quadro rapidamente evolutivo, caracterizado por instabilidade à marcha, mutismo, mioclonias generalizadas e coma vigil, vindo a falecer 5 meses após o início da doença. O estudo anátomo-patológico evidenciou lesões difusas nas regiões corticais, sub-corticais, no tronco do encéfalo e na medula espinhal, caracterizadas por degeneração neuronal, hipertrofia da astroglia e presença do estado espongioso.A case of peculiar form of Creutzfeldt-Jakob disease — the subacute disseminated encephalo-myelopathy one — is reported. The diagnosis was ascertained by necroscopic study. The clinical and electroencephalographic aspects are analysed. The patient here concerned, a man aged fifty two, during the clinical course of the disease showed stupor, decorticate posture, myoclonic jerks, epileptic seizures, muscular wasting in the left leg, exaggerated tendon reflexes in the face, tendon reflexes not elicitable in the legs. The electroencephalographic findings, during the downhill course of the disease, showed a pattern of irregularly depressed background rhythm with the periodic synchronous high voltage wave. The post-mortem findings revealed mild atrophy of the brain and the histological study revealed neuronal degeneration, astroglial hypertrophies and status spongiosus. The microscopic examination showed that the areas most affected were the frontal and occipital lobes, the basal

  4. Polymorphisms at codons 56 and 174 of the prion-like protein gene (PRND) are not associated with sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Jeong, Byung-Hoon; Kim, Nam-Ho; Kim, Jae-Il; Carp, Richard I; Kim, Yong-Sun

    2005-01-01

    Association between sporadic Creutzfeldt-Jakob disease (CJD) and the prion-like protein gene (PRND) has been reported in the German population. To investigate whether the PRND polymorphisms are associated with an increased risk for developing sporadic CJD in the Korean population, we compared the genotype and allele frequencies of PRND polymorphisms in 110 sporadic CJD patients with those in 102 healthy Koreans. Two polymorphisms (P56L, T174 M) in Koreans were found in the open reading frame (ORF) of PRND. One heterozygote of P56L was observed in normal controls but not in sporadic CJD patients. A strong significant difference of PRND genotype frequency at codon 174 was found between the normal Korean population and various European populations. In contrast to results in the German population, our study did not show a significant difference in PRND genotype or allele frequency at codon 174 between sporadic CJD and normal controls. This was the first genetic association study of the ORF of PRND in an Asian CJD population.

  5. An autopsy case of Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting with pathological laughing and an exaggerated startle reaction.

    Science.gov (United States)

    Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi; Akagi, Akio; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

    2017-07-13

    A 78-year-old Japanese woman presented with slow progressive disorientation and memory disturbances. Pathological laughing was observed at an early disease stage and continued for several months. Around the same time, the patient began to exhibit an exaggerated startle reaction and mild myoclonus. The pathological laughing and startle reaction disappeared before the patient reached an akinetic mutism state approximately 16 months after symptom onset. MRI showed extensive hyperintensity of the cerebral cortex and striatum on diffusion-weighted images, and swelling in the cerebral cortex on T2-weighted and fluid attenuated inversion recovery images. A prion protein (PrP) gene analysis revealed a V180I mutation with methionine homozygosity at codon 129. Neuropathological examination showed extensive spongiform changes with characteristic various-sized and non-confluent (VaSNoC) vacuoles in the cerebral neocortex and striatum. Gliosis and hypertrophic astrocytosis were generally mild in character. Neurons were relatively preserved in number. We believe that pathological laughing and an exaggerated startle reaction are possible pathognomonic findings of V180I genetic Creutzfeldt-Jakob disease. Based on the pathological findings of the present case, the presence of the VaSNoC-type spongiform changes with relative preservation of the neurons in the cerebral cortex and a lack of apparent brainstem involvement are associated at least in part with the pathological laughing and startle reaction. © 2017 Japanese Society of Neuropathology.

  6. Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate.

    Directory of Open Access Journals (Sweden)

    Chae Kim

    2011-09-01

    Full Text Available The origin, range, and structure of prions causing the most common human prion disease, sporadic Creutzfeldt-Jakob disease (sCJD, are largely unknown. To investigate the molecular mechanism responsible for the broad phenotypic variability of sCJD, we analyzed the conformational characteristics of protease-sensitive and protease-resistant fractions of the pathogenic prion protein (PrP(Sc using novel conformational methods derived from a conformation-dependent immunoassay (CDI. In 46 brains of patients homozygous for polymorphisms in the PRNP gene and exhibiting either Type 1 or Type 2 western blot pattern of the PrP(Sc, we identified an extensive array of PrP(Sc structures that differ in protease sensitivity, display of critical domains, and conformational stability. Surprisingly, in sCJD cases homozygous for methionine or valine at codon 129 of the PRNP gene, the concentration and stability of protease-sensitive conformers of PrP(Sc correlated with progression rate of the disease. These data indicate that sCJD brains exhibit a wide spectrum of PrP(Sc structural states, and accordingly argue for a broad spectrum of prion strains coding for different phenotypes. The link between disease duration, levels, and stability of protease-sensitive conformers of PrP(Sc suggests that these conformers play an important role in the pathogenesis of sCJD.

  7. Deep Learning Representation from Electroencephalography of Early-Stage Creutzfeldt-Jakob Disease and Features for Differentiation from Rapidly Progressive Dementia.

    Science.gov (United States)

    Morabito, Francesco Carlo; Campolo, Maurizio; Mammone, Nadia; Versaci, Mario; Franceschetti, Silvana; Tagliavini, Fabrizio; Sofia, Vito; Fatuzzo, Daniela; Gambardella, Antonio; Labate, Angelo; Mumoli, Laura; Tripodi, Giovanbattista Gaspare; Gasparini, Sara; Cianci, Vittoria; Sueri, Chiara; Ferlazzo, Edoardo; Aguglia, Umberto

    2017-03-01

    A novel technique of quantitative EEG for differentiating patients with early-stage Creutzfeldt-Jakob disease (CJD) from other forms of rapidly progressive dementia (RPD) is proposed. The discrimination is based on the extraction of suitable features from the time-frequency representation of the EEG signals through continuous wavelet transform (CWT). An average measure of complexity of the EEG signal obtained by permutation entropy (PE) is also included. The dimensionality of the feature space is reduced through a multilayer processing system based on the recently emerged deep learning (DL) concept. The DL processor includes a stacked auto-encoder, trained by unsupervised learning techniques, and a classifier whose parameters are determined in a supervised way by associating the known category labels to the reduced vector of high-level features generated by the previous processing blocks. The supervised learning step is carried out by using either support vector machines (SVM) or multilayer neural networks (MLP-NN). A subset of EEG from patients suffering from Alzheimer's Disease (AD) and healthy controls (HC) is considered for differentiating CJD patients. When fine-tuning the parameters of the global processing system by a supervised learning procedure, the proposed system is able to achieve an average accuracy of 89%, an average sensitivity of 92%, and an average specificity of 89% in differentiating CJD from RPD. Similar results are obtained for CJD versus AD and CJD versus HC.

  8. A patient with progressive cognitive decline and periodic abnormal waves in EEG: PLEDs of neurosyphilis or PSDs of Creutzfeldt-Jakob disease?

    Science.gov (United States)

    Lv, Yudan; Chu, Fengna; Meng, Hongmei; Wang, Zan; Cui, Li

    2014-07-01

    We report one Chinese patient with neurosyphilis exhibiting periodic lateralized epileptiform discharges (PLEDs) in the electroencephalogram (EEG). The patient (male, 59 years old) manifested with progressive cognitive decline and abnormal behavior. After several days, he gradually lost contact with others, and fell into a coma. EEG revealed periodic abnormal waves, predominantly located in the right anterior frontal region. The serum and cerebrospinal fluid Venereal Disease Research Laboratory test and Treponema pallidum hemagglutination assay were positive. Magnetic resonance imaging (MRI) of brain showed focal atrophy in the right frontal and temporal region. Diffusion-weighted MRI showed "lace sign" in cortex, which could be seen as an early and special feature in Creutzfeldt-Jakob disease (CJD). To differentiate "PLEDs of neurosyphilis on EEG" from "periodic synchronous discharges (PSD) of CJD on EEG," we treated this patient with diazepam 20 mg intravenously. After 10 minutes, periodic abnormal waves on EEG disappeared, with improved mental status, which confirmed the diagnosis of PLEDs of neurosyphilis. Then, after the treatment with penicillin, the patient improved and returned to work.

  9. Variant Creutzfeldt-Jakob Disease-Human Manifestation of Bovine Spongiform Encephalopathy%新型克-雅氏病是人的牛海绵状脑病

    Institute of Scientific and Technical Information of China (English)

    方元

    2001-01-01

    本文在概述人朊病毒的基础上,重点阐述新型克-雅氏病与早先已知的各种人朊病毒病的区别和它是由牛海绵状脑病朊病毒引起的证据,并对牛海绵状脑病并非源于痒病而是原于牛散发性朊病毒病、影响新型克-雅氏病流行规模的主要因素和该病的主要预防措施等问题进行了讨论。%Variant Creutzfeldt-Jakob disease (vCJD) is a new disease firstly described in April, 1996 in the United Kingdom. On the basis of a brief account of human prion diseases, this paper elucidates the differences between vCJD and bovine spongiform encephalopathy (BSE) and presents the evidence conforming that vCJD is caused by the BSE agent. In addition, the origin of BSE (it was not derived from scrapie), some important factors determining epidemic scale of vCJD and key preventive measures of vCJD are discussed.

  10. Human variant Creutzfeldt-Jakob disease and sheep scrapie PrP(res) detection using seeded conversion of recombinant prion protein.

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    Orrú, Christina D; Wilham, Jason M; Hughson, Andrew G; Raymond, Lynne D; McNally, Kristin L; Bossers, Alex; Ligios, Ciriaco; Caughey, Byron

    2009-08-01

    The pathological isoform of the prion protein (PrP(res)) can serve as a marker for prion diseases, but more practical tests are needed for preclinical diagnosis and sensitive detection of many prion infections. Previously we showed that the quaking-induced conversion (QuIC) assay can detect sub-femtogram levels of PrP(res) in scrapie-infected hamster brain tissue and distinguish cerebral spinal fluid (CSF) samples from normal and scrapie-infected hamsters. We now report the adaptation of the QuIC reaction to prion diseases of medical and agricultural interest: human variant Creutzfeldt-Jakob disease (vCJD) and sheep scrapie. PrP(res)-positive and -negative brain homogenates from humans and sheep were discriminated within 1-2 days with a sensitivity of 10-100 fg PrP(res). More importantly, in as little as 22 h we were able to distinguish CSF samples from scrapie-infected and uninfected sheep. These results suggest the presence of prions in CSF from scrapie-infected sheep. This new method enables the relatively rapid and sensitive detection of human CJD and sheep scrapie PrP(res) and may facilitate the development of practical preclinical diagnostic and high-throughput interference tests.

  11. Age at Death of Creutzfeldt-Jakob disease in subsequent family generation carrying the E200K mutation of the prion protein gene.

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    Maurizio Pocchiari

    Full Text Available BACKGROUND: The E200K mutation of the prion protein gene (PRNP is the most frequent amino acid substitution in genetic Creutzfeldt-Jakob disease and is the only one responsible for the appearance of clustered cases in the world. In the Israel and Slovakian clusters, age of disease onset was reduced in successive generations but the absence of a clear molecular basis raised the possibility that this event was an observational bias. The aim of the present study was to investigate possible selection biases or confounding factors related to anticipation in E200K CJD patients belonging to a cluster in Southern Italy. METHODS: Clinical and demographical data of 41 parent-offspring pairs from 19 pedigrees of the Italian cluster of E200K patients were collected. Age at death of parents was compared with age at death of E200K CJD offspring. Subgroup analyses were performed for controlling possible selection biases, confounding factors, or both. RESULTS: The mean age at death/last follow-up of the parent generation was 71.4 years while that of CJD offspring was 59.3 years with an estimated anticipation of 12.1 years. When the same analysis was performed including only parents with CJD or carrying the E200K mutation (n = 26, the difference between offspring and parents increased to 14.8 years. CONCLUSIONS: These results show that early age at death occurs in offspring of families carrying the E200K PRNP mutation and that this event is not linked to observational biases. Although molecular or environmental bases for this occurrence remain unsettled, this information is important for improving the accuracy of information to give to mutated carriers.

  12. CSF concentrations of cAMP and cGMP are lower in patients with Creutzfeldt-Jakob disease but not Parkinson's disease and amyotrophic lateral sclerosis.

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    Patrick Oeckl

    Full Text Available BACKGROUND: The cyclic nucleotides cyclic adenosine-3',5'-monophosphate (cAMP and cyclic guanosine-3',5'-monophosphate (cGMP are important second messengers and are potential biomarkers for Parkinson's disease (PD, amyotrophic lateral sclerosis (ALS and Creutzfeldt-Jakob disease (CJD. METHODOLOGY/PRINCIPAL FINDINGS: Here, we investigated by liquid chromatography/tandem mass spectrometry (LC-MS/MS the cerebrospinal fluid (CSF concentrations of cAMP and cGMP of 82 patients and evaluated their diagnostic potency as biomarkers. For comparison with a well-accepted biomarker, we measured tau concentrations in CSF of CJD and control patients. CJD patients (n = 15 had lower cAMP (-70% and cGMP (-55% concentrations in CSF compared with controls (n = 11. There was no difference in PD, PD dementia (PDD and ALS cases. Receiver operating characteristic (ROC curve analyses confirmed cAMP and cGMP as valuable diagnostic markers for CJD indicated by the area under the curve (AUC of 0.86 (cAMP and 0.85 (cGMP. We calculated a sensitivity of 100% and specificity of 64% for cAMP and a sensitivity of 67% and specificity of 100% for cGMP. The combination of both nucleotides increased the sensitivity to 80% and specificity to 91% for the term cAMPxcGMP (AUC 0.92 and to 93% and 100% for the ratio tau/cAMP (AUC 0.99. CONCLUSIONS/SIGNIFICANCE: We conclude that the CSF determination of cAMP and cGMP may easily be included in the diagnosis of CJD and could be helpful in monitoring disease progression as well as in therapy control.

  13. Rapid and Highly Sensitive Detection of Variant Creutzfeldt-Jakob Disease Abnormal Prion Protein on Steel Surfaces by Protein Misfolding Cyclic Amplification: Application to Prion Decontamination Studies.

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    Maxime Belondrade

    Full Text Available The prevalence of variant Creutzfeldt-Jakob disease (vCJD in the population remains uncertain, although it has been estimated that 1 in 2000 people in the United Kingdom are positive for abnormal prion protein (PrPTSE by a recent survey of archived appendix tissues. The prominent lymphotropism of vCJD prions raises the possibility that some surgical procedures may be at risk of iatrogenic vCJD transmission in healthcare facilities. It is therefore vital that decontamination procedures applied to medical devices before their reprocessing are thoroughly validated. A current limitation is the lack of a rapid model permissive to human prions. Here, we developed a prion detection assay based on protein misfolding cyclic amplification (PMCA technology combined with stainless-steel wire surfaces as carriers of prions (Surf-PMCA. This assay allowed the specific detection of minute quantities (10-8 brain dilution of either human vCJD or ovine scrapie PrPTSE adsorbed onto a single steel wire, within a two week timeframe. Using Surf-PMCA we evaluated the performance of several reference and commercially available prion-specific decontamination procedures. Surprisingly, we found the efficiency of several marketed reagents to remove human vCJD PrPTSE was lower than expected. Overall, our results demonstrate that Surf-PMCA can be used as a rapid and ultrasensitive assay for the detection of human vCJD PrPTSE adsorbed onto a metallic surface, therefore facilitating the development and validation of decontamination procedures against human prions.

  14. Comparison of the neuropathological characteristics of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) in mice.

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    Brown, D A; Bruce, M E; Fraser, J R

    2003-06-01

    Bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) belong to a group of diseases called the transmissible spongiform encephalopathies (TSEs). Transmission studies in inbred mice (strain typing) provided overwhelming evidence that vCJD arose from BSE. In this study, we compare the patterns of neuropathology in a panel of three inbred mouse strains (RIII, C57BL and VM) and one cross (C57BL x VM) infected with either vCJD or BSE. For each mouse strain, patterns of abnormal prion protein (PrPres) deposition, astrocytosis and vacuolation were similar in the vCJD- and BSE-challenged mice. Prion protein (PrP)-positive plaques were prominent in the VM and C57BL x VM mice in addition to diffuse PrPres accumulation, whereas only diffuse PrPres labelling was observed in the RIII and C57BL mice. The hippocampus was targeted in all mouse strains, as was the cochlear nucleus in the medulla, both showing consistent severe vacuolation and heavy PrPres deposition. Although the targeting of PrPres was similar in the BSE- and vCJD-infected brains, the amount and intensity of PrPres observed in the brains treated with formic acid during fixation was reduced considerably. The distribution of astrocytosis was similar to the targeting of PrPres deposition in the brain, although some differences were observed in the hippocampi of mice challenged with vCJD. We conclude that there are no significant differences in the targeting of neuropathological changes observed in the BSE- and vCJD-infected mice, consistent with the previous evidence of a link between BSE and vCJD.

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

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    Jeong Byung-Hoon

    2011-08-01

    Full Text Available Abstract Background Although polymorphisms of PRNP, the gene encoding prion protein, are known as a determinant affecting prion disease susceptibility, other genes also influence prion incubation time. This finding offers the opportunity to identify other genetic or environmental factor (s modulating susceptibility to prion disease. Ribosomal protein SA (RPSA, also called 37 kDa laminin receptor precursor (LRP/67 kDa laminin receptor (LR, acts as a receptor for laminin, viruses and prion proteins. The binding/internalization of prion protein is dependent for LRP/LR. Methods To identify other susceptibility genes involved in prion disease, we performed genetic analysis of RPSA. For this case-control study, we included 180 sporadic Creutzfeldt-Jakob disease (CJD patients and 189 healthy Koreans. We investigated genotype and allele frequencies of polymorphism on RPSA by direct sequencing or restriction fragment length polymorphism (RFLP analysis. Results We observed four single nucleotide polymorphisms (SNPs, including -8T>C (rs1803893 in the 5'-untranslated region (UTR of exon 2, 134-32C>T (rs3772138 in the intron, 519G>A (rs2269350 in the intron and 793+58C>T (rs2723 in the intron on the RPSA. The 519G>A (at codon 173 is located in the direct PrP binding site. The genotypes and allele frequencies of the RPSA polymorphisms showed no significant differences between the controls and sporadic CJD patients. Conclusion These results suggest that these RPSA polymorphisms have no direct influence on the susceptibility to sporadic CJD. This was the first genetic association study of the polymorphisms of RPSA gene with sporadic CJD.

  16. Study on clinical features in general paresis of insane, HIV-associated dementia and Creutzfeldt-Jakob disease behaved as dementia

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    LIU Wen-yan

    2012-06-01

    Full Text Available Objective To investigate the clinical features of general paresis of insane (GPI, HIV-associated dementia (HAD and Creutzfeldt -Jakob disease (CJD. Methods The clinical features, laboratory examination, electroencephalography (EEG, magnetic resonance imaging (MRI, treatment and prognosis of 19 patients (GPI, n = 8; HAD, n = 6; CJD, n = 5 were analyzed retrospectively. Results The cases of three groups had cognitive impairment. At the same time, multiple systems (pyramidal system, extrapyramidal system and cerebellar and multiple cranial nerves were involved. In GPI patients, the results of rapid plasma regain circle card test (RPR and treponema pallidum particle agglutination assay (TPPA were all positive in 8 cases, venereal disease research laboratory tests (VDRL of CSF were positive in 4 cases, and Head MRI showed encephalatrophy in 6 cases. In HAD patients, serologic tests for HIV of all cases were positive, the average protein of CSF was increased significantly and Pandy's test was positive in 2 cases, Head MRI were characterized with multiple space occupying lesions or diffuse abnormal density image. In CJD patients, CSF 14-3-3 protein showed positive in 4 patients, EEG showed diffuse slow waves in 5 case, in which 1 case showed typical periodic triphasic wave, Head MRI in 3 sporadic CJD patients (sCJD showed swelling like changes in sulus of cortex area and 1 varaint CJD (vCJD patient showed hockey like change in thalamencephalon. Conclusion The clinical characteristics of dementia caused by GPI, HAD or CJD are varied, and the diagnosis mainly depends on clinical features, positive findings in serum, CSF, EEG and MRI detections.

  17. Rapid and Highly Sensitive Detection of Variant Creutzfeldt-Jakob Disease Abnormal Prion Protein on Steel Surfaces by Protein Misfolding Cyclic Amplification: Application to Prion Decontamination Studies.

    Science.gov (United States)

    Belondrade, Maxime; Nicot, Simon; Béringue, Vincent; Coste, Joliette; Lehmann, Sylvain; Bougard, Daisy

    2016-01-01

    The prevalence of variant Creutzfeldt-Jakob disease (vCJD) in the population remains uncertain, although it has been estimated that 1 in 2000 people in the United Kingdom are positive for abnormal prion protein (PrPTSE) by a recent survey of archived appendix tissues. The prominent lymphotropism of vCJD prions raises the possibility that some surgical procedures may be at risk of iatrogenic vCJD transmission in healthcare facilities. It is therefore vital that decontamination procedures applied to medical devices before their reprocessing are thoroughly validated. A current limitation is the lack of a rapid model permissive to human prions. Here, we developed a prion detection assay based on protein misfolding cyclic amplification (PMCA) technology combined with stainless-steel wire surfaces as carriers of prions (Surf-PMCA). This assay allowed the specific detection of minute quantities (10-8 brain dilution) of either human vCJD or ovine scrapie PrPTSE adsorbed onto a single steel wire, within a two week timeframe. Using Surf-PMCA we evaluated the performance of several reference and commercially available prion-specific decontamination procedures. Surprisingly, we found the efficiency of several marketed reagents to remove human vCJD PrPTSE was lower than expected. Overall, our results demonstrate that Surf-PMCA can be used as a rapid and ultrasensitive assay for the detection of human vCJD PrPTSE adsorbed onto a metallic surface, therefore facilitating the development and validation of decontamination procedures against human prions.

  18. Is brain copper deficiency in Alzheimer's, Lewy body, and Creutzfeldt Jakob diseases the common key for a free radical mechanism and oxidative stress-induced damage?

    Science.gov (United States)

    Deloncle, Roger; Guillard, Olivier

    2015-01-01

    In Alzheimer's (AD), Lewy body (LBD), and Creutzfeldt Jakob (CJD) diseases, similar pathological hallmarks have been described, one of which is brain deposition of abnormal protease-resistant proteins. For these pathologies, copper bound to proteins is able to protect against free radicals by reduction from cupric Cu++ to cupreous Cu+. We have previously demonstrated in bovine brain homogenate that free radicals produce proteinase K-resistant prion after manganese is substituted for copper. Since low brain copper levels have been described in transmissible spongiform encephalopathies, in substantia nigra in Parkinson's disease, and in various brain regions in AD, LBD, and CJD, a mechanism has been proposed that may underlie the neurodegenerative processes that occur when copper protection against free radicals is impaired. In peptide sequences, the alpha acid proton near the peptide bond is highly mobile and can be pulled out by free radicals. It will produce a trivalent α-carbon radical and induce a free radical chain process that will generate a D-amino acid configuration in the peptide sequence. Since only L-amino acids are physiologically present in mammalian (human) proteins, it may be supposed that only physiological L-peptides can be recycled by physiological enzymes such as proteases. If a D-amino acid is found in the peptide sequence subsequent to deficient copper protection against free radicals, it will not be recognized and might alter the proteasome L-amino acid recycling from brain peptides. In the brain, there will result an accumulation of abnormal protease-resistant proteins such as those observed in AD, LBD, and CJD.

  19. Asymmetric cortical high signal on diffusion weighted-MRI in a case of Creutzfeldt-Jakob disease Hipersinal cortical assimétrico na ressonância magnética na imagem em difusão em caso de doença de Creutzfeldt-Jakob

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    Ricardo Nitrini

    2005-06-01

    Full Text Available High signal in the cerebral cortex and/or basal ganglia on diffusion-weighted magnetic resonance imaging (DW-MRI has been described as a good diagnostic marker for sporadic Creutzfeldt-Jakob disease (sCJD. We report a case of sCJD with atypical clinical evolution and unusual DW-MRI findings. A 53-year-old man was seen with a 2-year history of a rapidly progressive dementia and cerebellar ataxia. Cerebrospinal fluid analysis, including the test for 14-3-3 protein, was normal. EEG did not show periodic activity. However, DW-MRI showed gyriform hyperintensity involving practically the entire cortical ribbon of the left hemisphere, whilst being limited to the posterior cingulate gyrus in the right hemisphere. DNA analysis showed no mutations or insertions in the prion protein gene, and homozigozity for methionine in codon 129. A subsequent brain biopsy confirmed the diagnosis of CJD. Thus, high signal on DW-MRI may be limited to the cerebral cortex and may present a very asymmetric distribution in sCJD.Hipersinal no cortex cerebral e/ou nos gânglios da base observado com a técnica de difusão da ressonância magnética (RM-DIF tem sido descrito como bom marcador diagnóstico da doença de Creutzfeldt-Jakob esporádica (DCJe. Relatamos caso de DCJe com evolução clínica atípica e achados incomuns na RM-DIF. Homem de 53 anos foi examinado com história de dois anos de demência rapidamente progressiva e ataxia cerebelar. Exame do líquido cefalorraqueano, incluindo pesquisa da proteína 14-3-3, foi normal; EEG não revelou atividade periódica; RM-DIF mostrou hiperintensidade nos giros que afetava quase inteiramente o manto cortical do hemisfério cerebral esquerdo e que no hemisfério direito se limitava à parte posterior do giro cíngulo. Análise do DNA revelou ausência de mutação ou de inserção no gene da proteína priônica e a presença de homozigose para metionina no códon 129. Biópsia cerebral confirmou o diagnóstico de DCJ

  20. Sporadic Creutzfeldt-Jakob disease: a clinico-neuropathological analysis of nine definite cases Doença de Creutzfeldt-Jakob do tipo esporádico: análise clínico-neuropatológica de nove casos da forma definida

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    CARLOS M. DE CASTRO COSTA

    1998-09-01

    Full Text Available The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD. All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100% of the patients. In 5 (56% of these 9 cases, prion protein (PrP amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people.Os autores analisaram, do ponto de vista clínico e neuropatológico, nove casos da forma esporádica definida da doença de Creutzfeldt-Jakob (DCJ. Todos eles eram mulheres, com idade média de 62,7 anos. Oitenta e nove por cento dos pacientes exibiram sintomas psiquiátricos prodrômicos e iniciais; sinais típicos de demência e mioclonias estavam presentes em 100% deles. O EEG foi anormal em todos os casos e apresentou paroxismos pseudoperiódicos em 56% dos pacientes. O tempo de evolução da doença variou de 3 a 19 meses. Do ponto de vista neuropatológico, atrofia cerebral e cerebelar, espongiose, astrocitose e perda neuronal estavam presentes em 100% dos pacientes. Em 5 (56% dos 9 casos, foi evidenciada, por microscopia óptica e eletrônica, a presença de placas amilóides de proteína prion (PrP no cerebelo. Havia correlação positiva entre o número de placas e o tempo de evolução da doença. Os autores salientam as semelhanças desses seus casos de pacientes idosos com a nova

  1. Panencephalopathic Creutzfeldt-Jakob disease with distinct pattern of prion protein deposition in a patient with D178N mutation and homozygosity for valine at codon 129 of the prion protein Gene.

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    Marcon, Gabriella; Indaco, Antonio; Di Fede, Giuseppe; Suardi, Silvia; Finato, Nicoletta; Moretti, Valentino; Micoli, Sandro; Fociani, Paolo; Zerbi, Pietro; Pincherle, Alessandro; Redaelli, Veronica; Tagliavini, Fabrizio; Giaccone, Giorgio

    2014-03-01

    Prion diseases include sporadic, acquired and genetic forms linked to mutations of the prion protein (PrP) gene (PRNP). In subjects carrying the D178N PRNP mutation, distinct phenotypes can be observed, depending on the methionine/valine codon 129 polymorphism. We present here a 53-year-old woman with D178N mutation in the PRNP gene and homozygosity for valine at codon 129. The disease started at age 47 with memory deficits, progressive cognitive impairment and ataxia. The clinical picture slowly worsened to a state of akinetic mutism in about 2 years and the disease course was 6 years. The neuropathologic examination demonstrated severe diffuse cerebral atrophy with neuronal loss, spongiosis and marked myelin loss and tissue rarefaction in the hemispheric white matter, configuring panencephalopathic Creutzfeldt-Jakob disease. PrP deposition was present in the cerebral cortex, basal ganglia and cerebellum with diffuse synaptic-type pattern of immunoreactivity and clusters of countless, small PrP deposits, particularly evident in the lower cortical layers, in the striatum and in the molecular layer of the cerebellum. Western blot analysis showed the presence of type 1 PrP(Sc) (Parchi classification). These findings underline the clear-cut distinction between the neuropathological features of Creutzfeldt-Jakob disease associated with D178N PRNP mutation and those of fatal familial insomnia.

  2. Red-backed vole brain promotes highly efficient in vitro amplification of abnormal prion protein from macaque and human brains infected with variant Creutzfeldt-Jakob disease agent.

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    Nemecek, Julie; Nag, Nabanita; Carlson, Christina M.; Schneider, Jay R.; Heisey, Dennis M.; Johnson, Christopher J.; Asher, David M.; Gregori, Luisa

    2013-01-01

    Rapid antemortem tests to detect individuals with transmissible spongiform encephalopathies (TSE) would contribute to public health. We investigated a technique known as protein misfolding cyclic amplification (PMCA) to amplify abnormal prion protein (PrPTSE) from highly diluted variant Creutzfeldt-Jakob disease (vCJD)-infected human and macaque brain homogenates, seeking to improve the rapid detection of PrPTSE in tissues and blood. Macaque vCJD PrPTSE did not amplify using normal macaque brain homogenate as substrate (intraspecies PMCA). Next, we tested interspecies PMCA with normal brain homogenate of the southern red-backed vole (RBV), a close relative of the bank vole, seeded with macaque vCJD PrPTSE. The RBV has a natural polymorphism at residue 170 of the PrP-encoding gene (N/N, S/S, and S/N). We investigated the effect of this polymorphism on amplification of human and macaque vCJD PrPTSE. Meadow vole brain (170N/N PrP genotype) was also included in the panel of substrates tested. Both humans and macaques have the same 170S/S PrP genotype. Macaque PrPTSE was best amplified with RBV 170S/S brain, although 170N/N and 170S/N were also competent substrates, while meadow vole brain was a poor substrate. In contrast, human PrPTSE demonstrated a striking narrow selectivity for PMCA substrate and was successfully amplified only with RBV 170S/S brain. These observations suggest that macaque PrPTSE was more permissive than human PrPTSE in selecting the competent RBV substrate. RBV 170S/S brain was used to assess the sensitivity of PMCA with PrPTSE from brains of humans and macaques with vCJD. PrPTSE signals were reproducibly detected by Western blot in dilutions through 10-12 of vCJD-infected 10% brain homogenates. This is the first report showing PrPTSE from vCJD-infected human and macaque brains efficiently amplified with RBV brain as the substrate. Based on our estimates, PMCA showed a sensitivity that might be sufficient to detect PrPTSE in v

  3. Red-backed vole brain promotes highly efficient in vitro amplification of abnormal prion protein from macaque and human brains infected with variant Creutzfeldt-Jakob disease agent.

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    Julie Nemecek

    Full Text Available Rapid antemortem tests to detect individuals with transmissible spongiform encephalopathies (TSE would contribute to public health. We investigated a technique known as protein misfolding cyclic amplification (PMCA to amplify abnormal prion protein (PrP(TSE from highly diluted variant Creutzfeldt-Jakob disease (vCJD-infected human and macaque brain homogenates, seeking to improve the rapid detection of PrP(TSE in tissues and blood. Macaque vCJD PrP(TSE did not amplify using normal macaque brain homogenate as substrate (intraspecies PMCA. Next, we tested interspecies PMCA with normal brain homogenate of the southern red-backed vole (RBV, a close relative of the bank vole, seeded with macaque vCJD PrP(TSE. The RBV has a natural polymorphism at residue 170 of the PrP-encoding gene (N/N, S/S, and S/N. We investigated the effect of this polymorphism on amplification of human and macaque vCJD PrP(TSE. Meadow vole brain (170N/N PrP genotype was also included in the panel of substrates tested. Both humans and macaques have the same 170S/S PrP genotype. Macaque PrP(TSE was best amplified with RBV 170S/S brain, although 170N/N and 170S/N were also competent substrates, while meadow vole brain was a poor substrate. In contrast, human PrP(TSE demonstrated a striking narrow selectivity for PMCA substrate and was successfully amplified only with RBV 170S/S brain. These observations suggest that macaque PrP(TSE was more permissive than human PrP(TSE in selecting the competent RBV substrate. RBV 170S/S brain was used to assess the sensitivity of PMCA with PrP(TSE from brains of humans and macaques with vCJD. PrP(TSE signals were reproducibly detected by Western blot in dilutions through 10⁻¹² of vCJD-infected 10% brain homogenates. This is the first report showing PrP(TSE from vCJD-infected human and macaque brains efficiently amplified with RBV brain as the substrate. Based on our estimates, PMCA showed a sensitivity that might be sufficient to detect Pr

  4. Follow-up of a pedigree of familial Creutzfeldt-Jakob disease%遗传性CJD一家系随访分析

    Institute of Scientific and Technical Information of China (English)

    刘峥; 叶静; 李存江; 贾建平

    2011-01-01

    Objective To detect the PRNP gene mutations in a pedigree of familial Creutzfeldt- Jakob disease and evaluate the mutation rate of PRNP gene and the phenotype of the affected members. Methods The study group consisted of 28 members of a familial dementia pedigree and 310 healthy subjects. Genomic DNA was extracted from peripheral blood leucocytes of all subjects followed by in vitro amplification using polymerase chain reaction ( PCR ). The PCR products were directly sequenced by Sanger method. Result We detected missense mutations of PRNP gene in 15 family members, in which 3 were patients and 12 were healthy carriers, resulting in G114V mutation in the prion protein. A new patient demonstrated progressive dementia, myoclonus and Parkinsonism, whose cerebral MRI showed mild atrophy of left temporal lobe. EEG of the new patient indicated periodic sharp wave complexes ( PSWCs ). Conclusion Patients in this pedigree demonstrate the phenotype of CJD. The pedigree is autosomal dominant with incomplete penetrance.%目的 随访一个遗传性朊蛋白病的家系,对全部家系成员进行朊蛋白基因(PRNP)突变的筛查,探讨患病者的表型和突变发生率.方法 研究对象包括28例家系成员和310例健康对照.对研究对象的PRNP基因的开放阅读框架进行PCR扩增,产物直接测序,异常者重复测序,并与对照组对比.收集新发病例的影像和神经电生理资料.结果 共发现15例G114V基因突变者,其中3例发病,12例为携带者.1例新发病的患者表现为进行性痴呆、肌阵挛、帕金森综合征,头颅MRI示左侧颞叶轻度萎缩,脑电图有典型的周期性放电.结论 本家系为常染色体显性遗传的家族性CJD,新发病例的出现进一步明确了这一表型诊断,部分携带者不发病提示存在不完全外显.

  5. 中国2010年克-雅病监测病例特征分析%Surveillance of Creutzfeldt-Jakob disease in China,2010

    Institute of Scientific and Technical Information of China (English)

    陈操; 石琦; 周伟; 张宝云; 高晨; 田婵; 韩俊; 董小平

    2011-01-01

    Objective To understand the incidence, epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in China. Methods The analysis was conducted on the clinical and epidemiological data of suspected CJD cases collected through the surveillance in China in 2010. Cerebrospinal fluid (CSF) and blood samples were taken from the patients to detect PrPSc in brain tissue and 14-3-3 protein in CSF by using Western blot assay and analyze the polymorphism of 129 amino acid and mutation of PRNP gene extracted from blood samples by using PCR and sequencing assay. Results Totally 1 laboratory confirmed cases, 46 clinical diagnosed and 25 suspected cases of CJD, 3 fatal familial insomnia cases (FFI) and 5 genetic CJD cases were detected, including 1 R208H, 1 P102L and 3 T188K mutation cases No seasonal, geographic and population clustering of the disease were observed. The mean age of clinical diagnosed patients was 62 years old, the male to female ratio was 1. 30:1, the mean age of suspected patients was 57 years old, the male to female ratio was 2. 14: 1. Rapidly progressive dementia was the mostly common symptom, occurring firstly in 54. 93% of the CJD patients. More typical clinical manifestations were presented in clinical diagnosed patients than in suspected patients. Conclusion CJD occurred sporadically in China in 2010, which was demonstrated by the time, geographic, population, gender and age distributions of the cases.%目的 了解中国(未包括香港、澳门和台湾地区,下同)克-雅病(Creutzfeldt-Jakob disease,CJD)的发病情况、流行病学及临床特征.方法 对2010年中国CJD监测网络获得的可疑CJD病例的临床及流行病学资料进行分析,收集患者脑脊液及血液样品,利用Western blot方法检测脑组织中PrPSc蛋白及脑脊液中14 -3 -3蛋白,提取全血基因组DNA并利用PCR及测序方法对PRNP基因进行129位多态性及基因突变的分析.结果 共发现散发型CJD确定诊断病例1

  6. Gerstmann-Sträussler-Scheinker syndrome with the P102L pathogenic mutation presenting as familial Creutzfeldt-Jakob disease: a case report and review of the literature.

    Science.gov (United States)

    Rusina, Robert; Fiala, Jindřich; Holada, Karel; Matějčková, Milada; Nováková, Jana; Ampapa, Radek; Koukolík, František; Matěj, Radoslav

    2013-01-01

    Gerstmann-Sträussler-Scheinker syndrome is a rare autosomal dominant disease caused by a mutation in the prion gene, usually manifesting as progressive ataxia with late cognitive decline. A 44-year-old woman with a positive family history developed early personality and behavior changes, followed by paresthesias and ataxia, later associated with memory problems, pyramidal signs, anosognosia and very late myoclonus, spasticity, and severe dysexecutive impairment. Magnetic resonance showed caudate, mesio-frontal, and insular hyper-intensities, electroencephalography revealed generalized triphasic periodic complexes. A pathogenic P102L mutation in the prion gene was detected. Our case differed from classical Gerstmann-Sträussler-Scheinker syndrome by rapid progression, severe dementia, abnormal electroencephalography and magnetic resonance findings, which were highly suggestive of familial Creutzfeldt-Jakob disease.

  7. Creutzfeldt-Jakob disease versus anti-LGI1 limbic encephalitis in a patient with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal electroencephalogram: a case report

    Directory of Open Access Journals (Sweden)

    Sun L

    2015-06-01

    Full Text Available Li Sun, Jie Cao, Chang Liu, Yudan LvDepartment of Neurology, The First Hospital of JiLin University, ChangChun, People’s Republic of ChinaAbstract: Diagnosis of Creutzfeldt-Jakob disease (CJD is often challenging in elderly individuals, not only because of its variable clinical features but also because of nonspecific changes on the electroencephalogram (EEG in the early stages of the disease. Here we report on a patient who presented with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal EEG. We provide a detailed analysis and differential diagnosis between anti-leucine-rich glioma inactivated 1 (LGI1 limbic encephalitis versus CJD, in the hope of providing a new understanding of CJD. A 65-year-old Chinese man presented with slowly progressive cognitive decline with psychiatric symptoms. On admission, he presented with facial grimacing and brief left upper limb dystonic posturing lasting 1–2 seconds, with hyponatremia that was difficult to rectify. Neurological examination showed increased muscle tension in the left limb but without pathological reflexes. His early EEG showed focal periodic wave complexes. Diffusion-weighted magnetic resonance imaging showed a suspected “lace sign” in the occipital cortex. His cerebrospinal fluid was negative for LGI1 antibodies and positive for 14-3-3 brain protein. Therefore, we made a presumptive diagnosis of CJD. At the following visit, a second EEG showed paroxysmal sharp wave complexes, but the patient had a poor prognosis. Atypical facio-brachio-crural movement and nonspecific EEG changes may occasionally be found in patients with CJD or anti-LGI1 encephalitis. Clinicians should not be dissuaded from a diagnosis of CJD where the EEG does not show paroxysmal sharp wave complexes in the early stages but abnormal facio-brachio-crural movement is present.Keywords: abnormal facio-brachio-crural movement, hyponatremia, Creutzfeldt-Jakob

  8. Creutzfeldt-Jakob病43例患者的临床分析%The clinical features of 43 patients with Creutzfeldt-Jakob disease

    Institute of Scientific and Technical Information of China (English)

    刘静; 王玉平; 王红星; 李莉萍; 刘爱华; 叶静; 杨延辉

    2016-01-01

    Objective To analyze the clinical features of 43 patients with clinically possible or probable Creutzfeldt-Jakob disease (CJD) to provide references for the early clinical evaluation and diagnosis of CJD. Method All patients who were diagnosed with “suspected CJD” and hospitalized in our hospital between January 2013 to January 2016 were collected, and their clinical features and laboratory data were analyzed retrospectively. Results In general, the onset of CJD occurred at about 60 years old, with a mean course of disease of 5.70±5.08 months(median:four months). The first symptoms are changeable, mainly presenting with rapidly progressive dementia. In addition, typical clinical manifestations included 6 types: rapidly progressivedementia, damage in the locomotor system(fibrae pyramidales, extrapyramidal and cerebellar symptoms), myoclonus, akinetic mutism, sleep and vision disorders.Conclusion The early diagnosis of CJD should pay attention to its clinical features, and highly alarming and periodic review should be given to the patient with 2 typical features or above manifestations to avoid misdiagnosis, although 14-3-3 protein, EEG and MRI show atypical findings.%目的:分析43例临床可能或很可能克雅氏病(CJD)患者的临床特征,为CJD早期诊断提供一些参考。方法搜集2013年1月至2016年1月以“可疑CJD”诊断在首都医科大学宣武医院住院的患者,对其临床特点及实验室资料进行分析。结果 CJD通常在60岁左右发病,平均病程5.70±5.08个月;首发症状多变,以迅速进展性痴呆为主。典型临床表现有6种:迅速进展的痴呆、运动系统损害(锥体束、锥体外系及小脑症状)、肌阵挛、无动性缄默、睡眠障碍和视力障碍。结论 CJD的早期诊断应重视其临床特征,当一个患者具有典型特征中的两项或以上表现时,即使14-3-3蛋白、脑电图(EEG)、磁共振(MRI)均不典型,也要高度警惕CJD

  9. Brain in human nutrition and variant Creutzfeldt-Jakob disease risk (vCJD): detection of brain in retail liver sausages using cholesterol and neuron specific enolase (NSE) as markers.

    Science.gov (United States)

    Lücker, E; Horlacher, S; Eigenbrodt, E

    2001-08-01

    No information is available about the consumption of brain via meat products. With respect to the new variant of Creutzfeldt-Jakob disease (vCJD) and the presumed food-borne transmission of bovine spongiform encephalopathy (BSE) to humans, a preliminary survey for brain and/or spinal cord (tissues of the central nervous system, CNS) was conducted. We applied a previously developed integrated procedure using cholesterol and neuron specific enolase (NSE) as markers. Quantification of cholesterol had to be backed up by NSE immunochemistry in order to account for low specificity and relatively high variances. Out of 126 high-quality finely graded liver sausages, five samples (4 %) showed positive NSE immunoresponses. In four of these samples a transgression of the normal maximum cholesterol content was obtained. The identification of such a considerable number of CNS-positive sausages indicates that brain consumption is not as rare as previously assumed. Overall, the present integrated method could be successfully applied for the detection of CNS in heat-treated meat products. Its routine application in official food control would deter illegal practice and thus help to control transmissible spongiform encephalopathies.

  10. Estudo retrospectivo da doença de Creutzfeldt-Jakob diagnosticada no norte de Portugal entre 1993-2002: características demográficas, clínicas e neuropatológicas

    Directory of Open Access Journals (Sweden)

    Silva Ana Martins

    2003-01-01

    Full Text Available INTRODUÇÃO E OBJETIVO: Descrição das características demográficas, clinicas e neuropatológicas de 11 doentes com doença de Creutzfeldt-Jakob (DCJ. MÉTODO: Revisão clínica e neuropatológica de doentes com DCJ diagnosticados entre 1993 e 2002 em hospitais do Norte de Portugal. RESULTADOS: Foram identificados 11 doentes (4 do sexo feminino; idade média de início dos sintomas, 64 anos; média de duração da doença, 8 meses. Todos apresentaram síndrome demencial progressiva associada a mioclonias, sendo a síndrome cerebelar a forma de apresentação inicial em quatro deles. O estudo neuropatológico revelou sempre espongiose e gliose reativa associada a perda neuronal. O estudo imunocitoquímico para proteína priônica (PrP foi positivo nos oito casos em que foi executado. CONCLUSÃO: O grupo de doentes descritos constitui uma série clinica representativa da heterogeneidade de fenótipos possíveis da DCJ esporádica. O estudo neuropatológico é ainda indispensável para o diagnóstico definitivo da doença.

  11. An autopsy-verified case of FTLD-TDP type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Hayashi, Yuichi; Iwasaki, Yasushi; Takekoshi, Akira; Yoshikura, Nobuaki; Asano, Takahiko; Mimuro, Maya; Kimura, Akio; Satoh, Katsuya; Kitamoto, Tetsuyuki; Yoshida, Mari; Inuzuka, Takashi

    2016-11-01

    Here we report an autopsy-verified case of frontotemporal lobar degeneration (FTLD)-transactivation responsive region (TAR) DNA binding protein (TDP) type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD). A 69-year-old woman presented with an 11-month history of progressive dementia, irritability, insomnia, and gait disturbance without a family history of dementia or prion disease. Neurological examination revealed severe dementia, frontal signs, and exaggerated bilateral tendon reflexes. Periodic sharp-wave complexes were not observed on the electroencephalogram. Brain diffusion MRI did not reveal abnormal changes. An easy Z score (eZIS) analysis for (99m)Tc-ECD-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed a bilateral decrease in thalamic regional cerebral blood flow (rCBF). PRNP gene analysis demonstrated methionine homozygosity at codon 129 without mutation. Cerebrospinal fluid (CSF) analysis showed normal levels of both 14-3-3 and total tau proteins. Conversely, prion protein was slowly amplified in the CSF by a real-time quaking-induced conversion assay. Her symptoms deteriorated to a state of akinetic mutism, and she died of sudden cardiac arrest, one year after symptom onset.  Despite the SPECT results supporting a clinical diagnosis of MM2-thalamic-type sCJD, a postmortem assessment revealed that this was a case of FTLD-TDP type A, and excluded prion disease. Thus, this case indicates that whereas a bilateral decreasing thalamic rCBF detected by (99m)Tc-ECD-SPECT can be useful for diagnosing MM2-thalamic-type sCJD, it is not sufficiently specific. Postmortem diagnosis remains the gold standard for the diagnosis of this condition.

  12. Creutzfeldt-Jakob disease a case report, with special attention to the electroencephalogram in this disorder and to its possible relationships to kuru, scrapie and «mad cow disease»

    Directory of Open Access Journals (Sweden)

    A.H. Chapman

    1993-06-01

    Full Text Available A case of Creutzfeldt-Jakob disease in a 58-year-old Brazillian cattle rancher and businessman is presented. The EEG was normal, which is consistent with the fact that it was made during the first half of his illness; in a later stage suppression of normal rhythms by slow moderate voltage waves would be expected. The resemblances of kuru, scrapie and "mad cow disease» to C-J disease are discussed. In each of these 4 illnesses the patient or affected animal (scrapie and «mad cow disease" (a has a widespread spongiform encephalopathy and consequent dementia, myoclonic epilepsy and cerebellar and corticospinal symptoms, (b Each illness is caused by a virus (or virus-like organism called a PrP or prion which is unusually resistant to heat and entirely resistant to ultraviolet light and x-rays, (c This causative agent can be transmitted to other mammals by intracerebral injection or, in the proved cases of 3 of them, by the oral route. Unresolved questions about C-J disease include the following: Are C-J disease, kuru, scrapie and "mad cow disease" essentially similar illnesses caused by the same virus or by subtle variants of it? What is the incubation period of C-J disease, and does its virus exist for long periods of time in some asymptomatic persons, some of whom may never become neurologically ill? How does this virus enter the bodies of most persons with C-J disease, and why does the clinical disease characteristically occur only in middle age?

  13. Analyses of the similarity and difference of global gene expression profiles in cortex regions of three neurodegenerative diseases: sporadic Creutzfeldt-Jakob disease (sCJD), fatal familial insomnia (FFI), and Alzheimer's disease (AD).

    Science.gov (United States)

    Tian, Chan; Liu, Di; Xiang, Wei; Kretzschmar, Hans A; Sun, Qing-Lan; Gao, Chen; Xu, Yin; Wang, Hui; Fan, Xue-Yu; Meng, Ge; Li, Wei; Dong, Xiao-Ping

    2014-10-01

    Neurodegenerative disease is a general designation for the disorders that are progressive loss of structure or function and final death of neurons, including Alzheimer's, Parkinson's, Huntington's, prion diseases, etc. In this study, we comparatively analyzed 21 individual microarray data sets of the cortex tissues from 11 sporadic Creutzfeldt-Jakob disease (sCJD), 3 fatal familial insomnia (FFI), 3 Alzheimer's disease (AD), and 4 normal controls. After normalization, a collection of 730 differently expressed sets (DESets) were obtained by comparison of the data of three diseases with their original controls. Principal component analysis (PCA) showed a background-related distribution within the groups of FFI, AD, and normal control, but two apparently different subgroups within the group of sCJD were observed. Review of the clinical materials of 11 sCJD patients identified the difference in brain PrP(Sc) deposits between two subgroups. Hierarchical cluster analysis illustrated the relatively independent clusters of normal controls, FFIs, six sCJD cases (subgroup 1) with more PrP(Sc) deposits, respectively, while an overlapped cluster of five cases of sCJD2 (subgroup 2) with less PrP(Sc) deposits and AD patients. Despite of the presence of special gene expressions, many common features were found among those neurodegenerative diseases. The most commonly changed biological processes (BPs) were signal transduction, synaptic transmission, and neuropeptide signaling pathway. The most commonly changed pathways were MAPK signaling pathway, Parkinson's disease, and oxidative phosphorylation. Our data here provide the similarity and difference in global gene expressions among the patients with sCJD, FFI, and AD, which may help to understand the common mechanism of neurodegenerative diseases.

  14. An autopsied case of MM1 + MM2-cortical with thalamic-type sporadic Creutzfeldt-Jakob disease presenting with hyperintensities on diffusion-weighted MRI before clinical onset.

    Science.gov (United States)

    Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

    2017-02-01

    + MM2-cortical with thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD), which suggests a broader spectrum of sCJD clinicopathological findings. © 2016 Japanese Society of Neuropathology.

  15. Dynamic change of MRI in Creutzfeldt-Jakob disease (report of 1 case)%Creutzfeldt-Jakob病的MRI动态改变(附1例报告)

    Institute of Scientific and Technical Information of China (English)

    黄承芳; 姜丹; 金红花; 张智燕; 杨卫; 吴银侠

    2015-01-01

    目的:探讨Creutzfeldt-Jakob病( CJD)的MRI动态改变。方法回顾性分析1例CJD患者的临床资料。结果本例患者以进行性痴呆为特征,伴有共济失调、肌阵挛,发病3个月后达晚期。 DWI示早期双侧额、颞、顶、枕叶皮质、海马异常稍高信号,颞叶明显;随疾病进展脑皮质及海马异常信号变强,范围变大;晚期随着脑萎缩出现,脑皮质及海马异常高信号逐渐消失。患者因反复肺部感染死亡。结论散发型CJD患者DWI表现为早期病灶高信号,最常见于大脑皮质。%Objective To investigate the dynamic change of MRI in Creutzfeldt-Jakob disease ( CJD ) . Methods The clinical data of 1 sporadic CJD patient were analyzed retrospectively.Results The clinical features were progressive dementia, accompanied with ataxia and myoclonus in this case, and she reached terminal stage at 3 months after the onset of the disease.DWI showed the mildly hyperintensities in both side of cortex of frontal, temporal, parietal, occipital lobe and hippocampus, and especially of temporal lobe. With the progressive exacerbation of disease, the hyperintensities in cortex and hippocampus became higher and gradually distributed wider region.Late as brain atrophy, DWI displayed abnormally hyperintensities gradually decreased in both side cortexes of cerebra and hippocampus.The patient was died of pulmonary infection.Conclusion The DWI of sporadic CJD shows hyperintensities in the early stage, mostly in cerebral cortex.

  16. Hashimoto's encephalopathy mimicking Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Gauthier, Angela C; Baehring, Joachim M

    2017-01-01

    Hashimoto's encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto's thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966U/mL. After one month of 60mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved. Physicians should strongly consider this uncommon diagnosis in patients with rapid cognitive decline and no other clear etiology. Copyright © 2016 Elsevier Ltd. All rights reserved.

  17. Røyking og Alzheimers sykdom – en kort oversikt

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    Espen Bjertness

    2009-10-01

    Full Text Available SAMMENDRAGEn rekke epidemiologiske studier har påvist en invers sammenheng mellom røyking og Alzheimerssykdom (AD. Den inverse assosiasjonen er sterkere jo høyere forbruk av sigaretter og jo lengervarighet av røyking. Hos AD pasienter ser man dessuten at røykere har et senere debut-tidspunkt forsykdommen enn ikke-røykere. En mulig biologisk forklaring er at nikotin kompenserer for tap avnikotinreseptorer i hjernen, som er et vanlig funn ved AD, og opprettholder derved den kognitivefunksjonen i større grad enn hos ikke-røykere. Alternative forklaringer er konfounding med genereller ukjente faktorer. Selektiv død av røykerelaterte sykdommer kan medføre at gjenlevenderøykere har en lavere genetisk disposisjon for AD enn gjenlevende ikke-røykere.Bjertness E. Smoking and Alzheimer’s disease - a brief overview.Nor J Epidemiol ENGLISH SUMMARYSeveral case-control studies have demonstrated an inverse association between smoking andAlzheimer’s disease (AD. It has been speculated if nicotine from cigarettes compensates for loss ofnicotine receptors, which is a common finding in the brain in AD. Low-dose subcutaneous administrationof nicotine has been shown to improve cognitive function in AD. However, there are reportsindicating that the inverse association is due to confounding with genes or unknown risk factors.Apolipoprotein E4 is associated both with AD and death from coronary heart disease. Smoking isalso associated with death from coronary heart disease. Therefore cases with ApoE4-associated ADwill be removed from the pool of smokers due to selective death from coronary heart disease,leading to a higher prevalence of cases with the ApoE4 genotype in the pool of non-smokers.1995; 5 (2: 151-153.

  18. Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of (99m)Tc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Hayashi, Yuichi; Iwasaki, Yasushi; Yoshikura, Nobuaki; Asano, Takahiko; Hatano, Taku; Tatsumi, Shinsui; Satoh, Katsuya; Kimura, Akio; Kitamoto, Tetsuyuki; Yoshida, Mari; Inuzuka, Takashi

    2015-11-15

    We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14-3-3 protein, yielded normal results. An easy Z-score (eZIS) analysis for (99m)Tc-ethyl cysteinate dimer-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. Neuropathological examinations revealed severe neuronal loss, gliosis, and hypertrophic astrocytosis in the medial thalamus and inferior olivary nucleus. A slight depletion of Purkinje cells was observed. PrP immunostaining showed no obvious PrP deposits in the basal ganglia, thalamus, cerebellum, or brainstem; however, mild synaptic-type PrP deposits with some smaller plaque-like structures were only partially observed in the localized region of the frontal lobe with the spongiform change. Western blot analyses of protease-resistant PrP showed a type 2 pattern. In conclusion, eZIS analysis of (99m)Tc-ECD-SPECT images is useful for detecting both thalamic and medullary lesions. This is the first case of medullary lesions detected in a live patient with MM2-thalamic-type sCJD using SPECT.

  19. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

    Science.gov (United States)

    Kallenberg, K.; Summers, D. M.; Romero, C.; Taratuto, A.; Heinemann, U.; Breithaupt, M.; Varges, D.; Meissner, B.; Ladogana, A.; Schuur, M.; Haik, S.; Collins, S. J.; Jansen, Gerard H.; Stokin, G. B.; Pimentel, J.; Hewer, E.; Collie, D.; Smith, P.; Roberts, H.; Brandel, J. P.; van Duijn, C.; Pocchiari, M.; Begue, C.; Cras, P.; Will, R. G.; Sanchez-Juan, P.

    2009-01-01

    Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt–Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease to include findings from magnetic resonance imaging scans. PMID:19773352

  20. Subjektivt velvære, sosiale interaksjoner og aktivitet hos eldre med demens på dagsenter : en kvantitativ undersøkelse av atferdsendring ved dyreassisterte intervensjoner

    OpenAIRE

    Brusletto, Birgit

    2014-01-01

    Bakgrunn: Demens er en sykdom som stadig flere rammes av. Forskning har vist at dyreassisterte aktiviteter (DAA) og dyreassisterte terapier (DAT) overfor eldre med demens kan bidra til å bremse en utvikling av symptomer på negativ atferd, gi reduksjon i ensomhetsfølelse og stimulere til økt sosialt miljø blant deltakerne. Mål: Hovedmålet med denne masteroppgaven har vært å undersøke om intervensjoner med hund gir atferdsendringer som tyder på bedre helse ved større grad av velvære og økt ...

  1. Osteoporose – risikofaktor eller sykdom? Definisjon, utbredelse, årsaker, diagnostisering og forebyggende tiltak

    Directory of Open Access Journals (Sweden)

    Anne Johanne Søgaard

    2009-11-01

    Full Text Available  SAMMENDRAGVi vet foreløpig lite om utbredelsen av osteoporose i Norge – dvs. bentetthet (BMD minst 2,5 standardavvikunder gjennomsnittet for unge kvinner. Vi vet heller ikke om det har vært noen økning de siste 10-årene.Denne artikkelen gir en oversikten over emnet osteoporose – med vekt på risikofaktorer og forebyggendetiltak, og drøfter WHOs definisjon av osteoporose. I henhold til denne er osteoporose både en risikofaktor forbrudd – og en betegnelse på en tilstand som forutsetter minst ett brudd (etablert osteoporose. Definisjoneninnebærer at diagnosen osteoporose avhenger av det normalmaterialet man sammenliker med, og medfører aten meget stor andel av norske kvinner over 70 år vil få diagnosen osteoporose. En slik medikalisering vilkunne føre til hyppige legekontroller, angst og lavere fysisk aktivitet av redsel for å falle. Osteoporose erasymptomatisk før man har fått brudd, og har ingen automatiske behandlingsmessige konsekvenser. Man kanlikevel frykte at WHOs definisjon vil tvinge frem krav om medikamentell forebyggende behandling. Dette erproblematisk fordi det er stor overlapping i bentetthet mellom de som får brudd og de som ikke får. Måling avBMD er imidlertid den beste metoden vi i dag har for å forutsi brudd – og er like god som blodtrykksmåling ertil å forutsi slag. For å vurdere individuell risiko, bør imidlertid flere risikofaktorer for brudd kombineres. Deviktigste risikofaktorene for lav bentetthet er: lav kroppsvekt/lav relativ vekt, vektreduksjon, lavt inntak avkalsium/D-vitamin, røyking, tidlig menopause, inaktivitet, høyt alkoholkonsum og genetiske faktorer. Tiltakmed fokus på noen av disse har vist positiv effekt på BMD.Søgaard AJ. Osteoporosis – risk factor or disease? Definition, distribution, aetiology, diagnosis and  ENGLISH SUMMARYAt present we do not know the distribution of osteoporosis in the Norwegian population – i.e. bone mineraldensity (BMD below 2.5 standard

  2. Arvelig hemokromatose - nytten av screening

    Directory of Open Access Journals (Sweden)

    Arne Åsberg

    2009-10-01

    Full Text Available Arvelig hemokromatose fører til jernopphopning i kroppen, men gir sjelden alvorlig helseskade. Nesten alle hemokromatosepasienter i vårt land er homozygote for C282Y-mutasjonen i HFE-genet. Omtrent 7 per 1000 innbyggere har denne genotypen. Alvorlig syke blir bare omkring 5-15% av homozygote menn og nesten ingen kvinner. Likevel er det holdepunkter for at screening for hemokromatose blant friske, yngre menn kan være kostnadseffektivt. Det er relativt lett å påvise om en person er disponert for sykdommen, i god tid før den bryter ut, og forebyggende behandling er billig og effektiv. Imidlertid kan vi ikke forutsi hvilke screeningpositive personer som ubehandlet får alvorlig sykdom. Et kontrollert forsøk med screening bør gjennomføresHereditary hemochromatosis – benefits of screening. Hereditary hemochromatosis leads to iron accumulation in the body; however, serious illness due to hemochromatosis is rare. In Norway, almost all patients with hemochromatosis are homozygous for the C282Ymutation in the HFE-gene, a genotype carried by about 7 per 1000 inhabitants. Serious complications are seen in only about 5-15% of homozygous men and in very few women. Nevertheless, screening young men for hemochromatosis may be cost-effective. Detecting predisposed men is relatively straightforward, and prophylactic treatment is cheap and effective. However, we can not predict, among screen-positive men, the few who untreated will become seriously ill. A controlled screening trial should be conducted.

  3. Unusual resistance to ionizing radiation of the viruses of kuru, Creutzfeldt-Jakob disease, and scrapie.

    Science.gov (United States)

    Gibbs, C J; Gajdusek, D C; Latarjet, R

    1978-01-01

    The titers of several preparations of kuru. Creutzfeldt-Jacob disease, and scrapie viruses were reduced by only 1/10th or less by high doses of gamma radiation of 50 kGy and by only 1/10th-1/1000th or less for 200 kGy. This unusual radiation resistance of the two human viruses further links them with the scrapie virus and suggests that the genetic information of all three viruses is considerably smaller than that of any other known viruses of mammals. PMID:104301

  4. Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia

    National Research Council Canada - National Science Library

    S. E. McPherson; J. D. Kuratani; J. L. Cummings; J. Shih; P. S. Mischel; H. V. Vinters

    1994-01-01

    .... The patient ultimately evidenced mixed transcortical aphasia (MTA) with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the symptoms in MTA, including intact repetition and echolalia...

  5. Creutzfeldt-Jakob disease with mixed transcortical aphasia: insights into echolalia

    National Research Council Canada - National Science Library

    McPherson, S E; Kuratani, J D; Cummings, J L; Shih, J; Mischel, P S; Vinters, H V

    1994-01-01

    .... The patient ultimately evidenced mixed transcortical aphasia (MTA) with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the symptoms in MTA, including intact repetition and echolalia...

  6. An experiential learning model applied to nurses working with patients with Creutzfeldt-Jakob disease.

    Science.gov (United States)

    D'Amour, Rolande; Guimond, Pierrette

    2010-01-01

    Creutzfeldt-Jacob disease (C/D) is a rare neurological disease, transmissible, incurable and always fatal affecting humans, as well as animals. In the 1980s, the "mad cow disease" (MCD) epidemic in the United Kingdom popularized prion diseases worldwide. However, this contributed to the proliferation of disinformation, causing confusion between C/D and MCD in the public, as well as in some health care providers. The purpose of this article is to describe the process utilized to develop, implement, and evaluate a workshop on CJD for nurses and other health care providers. Kolb's experiential teaching/learning model was used as a framework for this workshop. A workbook was developed to complement the participants' learning. Fifteen health care providers from the Alzheimer Society of Canada's Dementia Network agreed to participate in this educational project. The results indicated that the participants had limited knowledge about C/D. They felt ill prepared and uncomfortable in providing quality care to this patient population. The workshop generated new insights and knowledge about the disease and the needs of the patients and their families. Participants exchanged ideas for tailored interventions. An experiential teaching/learning model is a highly effective approach to increase knowledge and skills, as well as fostering reflective practice.

  7. Subtype and regional-specific neuroinflammation in sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    Franc eLlorens

    2014-08-01

    Full Text Available The present study identifies deregulated cytokines and mediators of the immune response in the frontal cortex and cerebellum of sCJD MM1 and VV2 subtypes compared to age-matched controls. Deregulated genes include pro- and anti-inflammatory cytokines, toll-like receptors, colony stimulating factors, cathepsins, members of the complement system, and members of the integrin and CTL/CTLD family with particular regional and sCJD subtype patterns. Analysis of cytokines and mediators at protein level shows expression of selected molecules and receptors in neurons and/or in astrocytes and/or in microglia thus suggesting interactions between neurons and glial cells, mainly microglia, in the neuroinflammatory response in sCJD. Similar inflammatory responses have been shown in the tg340 sCJD MM1 mice, revealing a progressive deregulation of inflammatory mediators with disease progression. Yet inflammatory molecules involved are subjected to species differences in humans and mice. Moreover, inflammatory-related cell signalling pathways NFκB/IKK and JAK/STAT are activated in sCJD and sCJD MM1 mice. Together, the present observations show a self-sustained complex inflammatory and inflammatory-related responses occurring already at early clinical stages in animal model and dramatically progressing at advanced stages of sCJD. Considering this scenario, measures tailored to modulate (activate or inhibit specific molecules could be therapeutic options in CJD.

  8. Genetic and Transcriptomic Profiles of Inflammation in Neurodegenerative Diseases: Alzheimer, Parkinson, Creutzfeldt-Jakob and Tauopathies

    National Research Council Canada - National Science Library

    López González, Irene; Garcia-Esparcia, Paula; Llorens, Franc; Ferrer, Isidre

    2016-01-01

    Polymorphisms in certain inflammatory-related genes have been identified as putative differential risk factors of neurodegenerative diseases with abnormal protein aggregates, such as sporadic Alzheimer's disease (AD...

  9. 医源性Creutzfeldt-Jakob病%Introgenic Creutzfeldt-Jakob Disease

    Institute of Scientific and Technical Information of China (English)

    林世和

    2002-01-01

    @@ 1999年7月19~21日在世界卫生组织官员Maura Ricketts的组织领导下,奥地利著名神经病学家Herbert Budka、英国Martin Zeidler、MR Bradley教授等专程来到北京,由中国卫生部和世界卫生组织联合举办,中国预防医学科学院具体承办的Creutzfeldt-Jakob病(CJD)监测讲习班,旨在我国尽快成立国家CJD监测中心,同世界发达国家与世界卫生组织取得紧密联系,共同努力尽快发现CJD的危险因素与致病因子,为减少和预防CJD的发生与扩延做出自己的贡献.会议期间吉林大学第一医院CJD课题组报告了国人CJD的临床、病理、免疫组化、基因检测、14-3-3蛋白和动物传递的研究.董小平研究员报告了人朊病毒病的实验室研究,郭玉璞教授对CJD的病理改变做了补充发言.

  10. Cleanability of dental instruments--implications of residual protein and risks from Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Walker, J T; Dickinson, J; Sutton, J M; Raven, N D H; Marsh, P D

    2007-10-13

    Cleaning of dental instruments is the first line of control in reducing the adherent bioburden. The threat of vCJD and the difficulty in removing prion protein has provided a new challenge for cleaning surgical and dental instruments. Prion proteins are also more resistant to many disinfection and sterilisation techniques. A number of different methods are currently available in primary care for cleaning instruments including manual washing, ultrasonic cleaners and washer disinfectors. Manual cleaning of dental instruments is time-consuming, introduces operator error and the risk of puncture wounds, is not reproducible and does not completely remove debris from instruments. Ultrasonic baths are significantly more effective than hand cleaning alone and are currently used by the majority of dental surgeries (often as an adjunct to manual cleaning). Automated washer-disinfectors appear to provide a validated, reliable and reproducible procedure for disinfection and sterilisation of dental instruments to ensure both the safety of patients and dental staff. Dental instruments that are difficult to clean are frequently contaminated with tissue debris after routine reprocessing and cannot be excluded as a potential transmission risk for infectious agents, including prions. The transmission of vCJD via dentistry is considered to be low risk, however, the Department of Health (DoH) has recently advised dentists to ensure that endodontic reamers and files are treated as single-use as a precautionary basis in order to further reduce any risk of vCJD transmission.

  11. Viktigheten av fysisk aktivitet og trening blant eldre

    Directory of Open Access Journals (Sweden)

    Hilde Lohne-Seiler

    2012-11-01

    Full Text Available Regelmessig fysisk aktivitet er viktig for eldres helse. Sammenhengen mellom en fysisk aktiv livsstil og helsegevinster som forebygging av sykdom, opprettholdelse av uavhengighet og økt livskvalitet er godt dokumentert (ACSM's Position Stand 1998; Spirduso & Cronin, 2001; Taylor et al., 2004. Hensikten med denne oversiktsartikkelen er derfor å redegjøre for viktigheten av regelmessig fysisk aktivitet og trening i aldringsprosessen, samt utdype sammenhengen mellom en fysisk aktiv livsstil og fysisk funksjon blant eldre. Basert på en nasjonal multisenterstudie (Anderssen et al., 2009, tilfredsstilte 20% voksne og eldre (20-85 år de nasjonale anbefalinger om 30 minutter med daglig fysisk aktivitet. Kun 12% i den eldste aldersgruppen (80-85 år tilfredsstilte anbefalingene. Totalt fysisk aktivitetsnivå, målt ved hjelp av akselerometer, ble redusert med økende alder, hvor de eldste (80-85 år hadde 50% lavere aktivitetsnivå sammenlignet med de yngste (65-69 år (Lohne-Seiler et al., 2012. Fra 25-års alder reduseres utholdenhet og muskelstyrke med 10% per 10 år dersom individet er inaktiv (Heath et al., 1981; Wilmore, 1991. Systematisk utholdenhets- og styrketrening ser derimot ut til å redusere tapet med inntil halvparten, det vil si en reduksjon tilsvarende 5% per 10 år hos fysisk aktive individer (Heath et al., 1981. Eldre individer ser ut til å ha de samme treningsinduserte effekter som yngre, det vil si at man får effekt av økt aktivitet uansett alder, og da også lik prosentvis fremgang (Hagberg et al., 1989. Studier har vist at det er mulig å øke maksimalt oksygenopptak og maksimal muskelstyrke med inntil 10-20% i løpet av en 12 ukers intervensjon (Hagberg et al., 1989; Reeves et al., 2003; Lohne-Seiler et al, in press. Da muskelstyrke ser ut til å ha en sterk sammenheng med individets funksjonsevne/mobilitet, er det viktig å kunne tilrettelegge for systematisk styrketrening for eldre for på den måten å oppnå

  12. Katodisk beskyttelse av umbilicals

    OpenAIRE

    Mjølhus, Bjarte

    2008-01-01

    Ulike sider ved bruk av sinkbånd til korrosjonsbeskyttelse av karbonstål umbilical er evaluert. Det er utført litteraturstudie ved gjennomgang av tidligere rapporter, sammenligning av standarder og artikkelsøk. Langtidsforsøk med egne korrosjonsceller ble utført over 2 måneder for å undersøke korrosjonsbeskyttelse ved 4 ulike temperaturer. Det ble også gjort undersøkelser av korrosjonsbelegg i SEM for å undersøke og sammenligne med publiserte data. Åpen krets potensialet viste seg å ligge ...

  13. AVS on satellite

    Science.gov (United States)

    Zhao, Haiwu; Wang, Guozhong; Hou, Gang

    2005-07-01

    AVS is a new digital audio-video coding standard established by China. AVS will be used in digital TV broadcasting and next general optical disk. AVS adopted many digital audio-video coding techniques developed by Chinese company and universities in recent years, it has very low complexity compared to H.264, and AVS will charge very low royalty fee through one-step license including all AVS tools. So AVS is a good and competitive candidate for Chinese DTV and next generation optical disk. In addition, Chinese government has published a plan for satellite TV signal directly to home(DTH) and a telecommunication satellite named as SINO 2 will be launched in 2006. AVS will be also one of the best hopeful candidates of audio-video coding standard on satellite signal transmission.

  14. DESIGN AV FREMTIDENS KUMLOKK

    OpenAIRE

    Breien, William Topper

    2014-01-01

    Denne oppgavens mål har vært å utvikle et nytt design for kumlokk. Det nye designet skal være lettere enn utgangspunktet. Dette har blitt gjort ved hjelp av en iterativ designprosess, styrkeberegninger i Siemens NX 8, simuleringer av støp i Magmasofts MAGMA5, prøvestøping og testing ved støperiet til Ulefos Jernværk i Ulefoss. Denne rapporten inneholder teoretisk bakgrunn og beskrivelse av metoder tatt i bruk ved utvikling av nytt design, simulerin...

  15. Verdsetting av brukermedvirkning ved utforming av skoleanlegg

    Directory of Open Access Journals (Sweden)

    Else Margrethe Lefdal

    2015-03-01

    Full Text Available Ved planlegging av offentlige bygninger i Norge forutsettes det at brukerne medvirker i prosessen. Dette er forankret i lov- og avtaleverk og motiveres ved at brukerne innehar en kompetanse som er av betydning for byggenes funksjonalitet. I denne artikkelen vil jeg belyse brukermedvirkningsproblematikk i planleggingen av et skoleanlegg for videregående opplæring. Tilgjengelige plan- og byggesaksdokumenter i den aktuelle fylkeskommunens digitale og fysiske arkiver er blitt analysert. I referater fra brukermøtene fremmes det for eksempel ønsker fra brukerne om begrenset bruk av glass. Det ferdige skole-anlegget har imidlertid utstrakt bruk av innvendige glassvegger, men det er ikke dokumentert diskusjoner knyttet til bestemmelsen av dette. Et profesjons- og maktperspektiv er relevant for å forstå hva som blir verdsatt, og hvilke strukt-urer og mekanismer som har påvirket planleggings­prosessen. De sentrale aktørgruppene er skoleeier og representantene for lærer- og arkitekt-profesjonen. Omfattende bruk av innvendig glass i skoleanlegget tolker jeg som et kompromiss mellom skoleeiers ønske om åpenhet og kontroll, lærernes ønske om mindre støy og arkitektens ønske om å skape romfølelse og visuelle siktlinjer.AbstractWhen planning public buildings in Norway, it is expected that users are involved in the process. This is grounded in legislation and agreements, and motivated by the idea that users have an expertise which is significant for the planning of the building’s functionality. The aim of this article is to investigate user participation in planning the school facilities for a secondary school. I have analyzed planning and building documents available in government records, digital and physical. In the reports from meetings with user groups, it is for example stated that users want to limit the use of glass. However, the completed school building has extensive use of glass dividing walls, but any discussions leading to this

  16. RARB and STMN2 polymorphisms are not associated with sporadic Creutzfeldt-Jakob disease (CJD) in the Korean population.

    Science.gov (United States)

    Jeong, Byung-Hoon; Kim, Hae-Jung; Lee, Kyung-Hee; Carp, Richard I; Kim, Yong-Sun

    2014-01-01

    Polymorphisms in the prion protein gene (PRNP) can affect the susceptibility of humans to prion diseases. Recently, aside from PRNP, single nucleotide polymorphisms (SNPs) of two candidate genes for susceptibility to human prion diseases have been identified by human genome-wide association studies (GWAS) in the British population. One SNP of retinoic acid receptor beta (RARB), which is correlated with prion disease incubation time in mice, was associated with human prion diseases such as variant and iatrogenic CJD in the British population. The other SNP of the gene that encodes SCG10 (STMN2), which is related to clinical onset of sporadic CJD, was also associated with variant CJD and kuru. In order to investigate whether two polymorphisms located in upstream of RARB and STMN2 are associated with sporadic CJD in the Korean population, we compared genotype and allele frequencies of these polymorphisms in 217 sporadic CJD patients and 216 healthy Koreans. The genotype distribution and allele frequencies in upstream of the RARB and STMN2 polymorphisms were not significantly different between healthy controls and Korean sporadic CJD patients. This finding indicates that the two SNPs are not correlated with genetic susceptibility to sporadic CJD in the Korean population. This is the first genetic association study of RARB and STMN2 with sporadic CJD in an Asian population.

  17. Generalized cerebral atrophy seen on MRI in a naturally exposed animal model for creutzfeldt-jakob disease

    Directory of Open Access Journals (Sweden)

    Dasanu Constantin A

    2010-11-01

    Full Text Available Abstract Background Magnetic resonance imaging has been used in the diagnosis of human prion diseases such as sCJD and vCJD, but patients are scanned only when clinical signs appear, often at the late stage of disease. This study attempts to answer the questions "Could MRI detect prion diseases before clinical symptoms appear?, and if so, with what confidence?" Methods Scrapie, the prion disease of sheep, was chosen for the study because sheep can fit into a human sized MRI scanner (and there were no large animal MRI scanners at the time of this study, and because the USDA had, at the time of the study, a sizeable sample of scrapie exposed sheep, which we were able to use for this purpose. 111 genetically susceptible sheep that were naturally exposed to scrapie were used in this study. Results Our MRI findings revealed no clear, consistent hyperintense or hypointense signal changes in the brain on either clinically affected or asymptomatic positive animals on any sequence. However, in all 37 PrPSc positive sheep (28 asymptomatic and 9 symptomatic, there was a greater ventricle to cerebrum area ratio on MRI compared to 74 PrPSc negative sheep from the scrapie exposed flock and 6 control sheep from certified scrapie free flocks as defined by immunohistochemistry (IHC. Conclusions Our findings indicate that MRI imaging can detect diffuse cerebral atrophy in asymptomatic and symptomatic sheep infected with scrapie. Nine of these 37 positive sheep, including 2 one-year old animals, were PrPSc positive only in lymph tissues but PrPSc negative in the brain. This suggests either 1 that the cerebral atrophy/neuronal loss is not directly related to the accumulation of PrPSc within the brain or 2 that the amount of PrPSc in the brain is below the detectable limits of the utilized immunohistochemistry assay. The significance of these findings remains to be confirmed in human subjects with CJD.

  18. MM1-type sporadic Creutzfeldt-Jakob disease with 1-month total disease duration and early pathologic indicators.

    Science.gov (United States)

    Iwasaki, Yasushi; Kato, Hiroko; Ando, Tetsuo; Mimuro, Maya; Kitamoto, Tetsuyuki; Yoshida, Mari

    2017-10-01

    A 62-year-old man presented with abnormal behavior and cognitive impairment. Diffusion-weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent. Neuropil rarefaction and neuron loss were not apparent. Mild proliferation of anti- GFAP-positive astrocytes was observed in the cerebral cortex, but unaffected regions were noted. Regions without spongiform changes and GFAP-positive astrocytes included the hippocampal formation and subiculum. PrP immunostaining showed extensive diffuse synaptic-type PrP deposition in the gray matter, including the hippocampal region, but it was also mild. PrP gene analysis revealed no mutation with methionine homozygosity at polymorphic codon 129. Western blot analysis of proteinase K-resistant PrP indicated type 1 PrP(Sc) . The clinicopathological findings of the present case confirm several hypotheses: (i) the earliest pathologic evidence observed by HE staining in CJD are spongiform changes; (ii) DWI hyperintense regions indicate these spongiform changes; and (iii) regions without spongiform changes, gliosis and proliferation of GFAP-positive astrocytes, but with PrP deposition, exist in the early disease stage. © 2017 Japanese Society of Neuropathology.

  19. Description and analysis of 12 years of surveillance for Creutzfeldt-Jakob disease in Denmark, 1997 to 2008

    DEFF Research Database (Denmark)

    Gubbels, S; Bacci, S; Laursen, H;

    2012-01-01

    Prospective surveillance of Creutzfeldt–Jakob disease (CJD) was initiated in Denmark in 1997, following the observation of variant CJD in the United Kingdom. Demographic, clinical and diagnostic information was collected for each patient with clinical suspicion of CJD. Here we describe the method...

  20. [A case of Creutzfeldt-Jakob in the Mexican north-east and review of current concepts on prion disease].

    Science.gov (United States)

    Calderón-Garcidueñas, A L; Sagastegui-Rodríguez, J A; Canales-Ibarra, C; Farías-García, R

    2001-01-01

    The case reported here is that of a 50-year-old man from Saltillo, Coahuila, Mexico, who during the previous 15 months developed a demential syndrome and myoclonia. The brain biopsy led to establish a diagnosis of spongiform encephalopathy. The EEG showed periodic sharp wave complexes over the right hemisphere. A review on about prion diseases is included.

  1. R3-R4 deletion in the PRNP gene is associated with Creutzfeldt-Jakob disease (CJD)

    Energy Technology Data Exchange (ETDEWEB)

    Cervenakova, L.; Brown, P.; Nagle, J. [and others

    1994-09-01

    There are conflicting reports on the association of deletions in the PRNP gene on chromosome 20 with CJD, a rapidly progressive fatal spongiform encephalopathy. We accumulated data suggesting that a deletion of R3-R4 type (parts of the third and fourth repeats are deleted from the area of four repeating 24 bp sequences in the 5{prime} region of the gene) is causing CJD. Screening of 129 unaffected control individuals demonstrated presence of a deletion of R2 type in four (1.55% of the studied chromosomes), but none of them had the R3-R4 type. Of 181 screened patients with spongiform encephalopathies, two had a deletion of R3-R4 type with no other mutations in the coding sequence. Both patients had a classical rapidly progressive dementing disease and diffuse spongiform degeneration, and both cases were apparently sporadic. The same R3-R4 type of deletion was detected in three additional neuropathologically confirmed spongiform encephalopathy patients, of which two had other known pathogenic mutations in the PRNP gene: at codon 178 on the methionine allele exhibiting the phenotype of fatal familial insomnia, and codon 200 causing CJD with severe dementia; the third was a patient with iatrogenic CJD who developed the disease after treatment with growth hormone extracted from cadaveric human pituitary glands. In all cases the deletion coincided with a variant sequence at position 129 coding for methionine.

  2. AV dissociation, an inevitable response.

    Science.gov (United States)

    Wang, Kyuhyun; Benditt, David G

    2011-07-01

    The independent activation of the atria and ventricles, AV dissociation, is a common phenomenon that occurs during a wide variety of electrophysiologic circumstances. The clinical significance of AV dissociation is often misunderstood. This article examines the basis and clinical implications of AV dissociation. AV dissociation is often an obligatory, secondary phenomenon, and should not be construed as the primary disorder; it may be due to either the AV conduction system being completely blocked (3° AV block) or the P wave and the QRS complex being generated from separate sources (usually, the AV junction or ventricle) but occurring close together during the physiologic refractory period of each other. The latter may happen in junctional or ventricular arrhythmias including escape or accelerated rhythm, tachycardia, or premature beats. The crucial clinical point is not the AV dissociation itself, but that an underlying triggering primary disorder is present and should be identified. ©2011, Wiley Periodicals, Inc.

  3. Summer Student Report - AV Workflow

    CERN Document Server

    Abramson, Jessie

    2014-01-01

    The AV Workflow is web application which allows cern users to publish, update and delete videos from cds. During my summer internship I implemented the backend of the new version of the AV Worklow in python using the django framework.

  4. Skyggedom av enkepensjonsdommen

    DEFF Research Database (Denmark)

    Rognlien, Ida Gundersby

    2014-01-01

    Masteroppgaven undersøker om bortfall av ektefellepensjon ved lovendring med tilbakevirkende kraft er i strid med retten til eiendom etter EMK TP 1 art 1. Skyggedommetoden/ Feminist Judgments -metoden brukes for å gjenskrive enkepensjonsdommen Rt.2006.262 i et feministisk perspektiv. Da mannen døde.......1975.220. I spørsmålet om inngrepet er proporsjonalt ble vektlagt at det ikke foreligger en transparent avveining av de relevante interessene. Videre er hensynene bak og behovene for den opprinnelige ektefellepensjonen fortsatt tilstede, og de er ikke tilstrekkelig avveid mot formålene med inngrepet...

  5. Skyggedom av enkepensjonsdommen

    DEFF Research Database (Denmark)

    Rognlien, Ida Gundersby

    2014-01-01

    Masteroppgaven undersøker om bortfall av ektefellepensjon ved lovendring med tilbakevirkende kraft er i strid med retten til eiendom etter EMK TP 1 art 1. Skyggedommetoden/ Feminist Judgments -metoden brukes for å gjenskrive enkepensjonsdommen Rt.2006.262 i et feministisk perspektiv. Da mannen døde.......1975.220. I spørsmålet om inngrepet er proporsjonalt ble vektlagt at det ikke foreligger en transparent avveining av de relevante interessene. Videre er hensynene bak og behovene for den opprinnelige ektefellepensjonen fortsatt tilstede, og de er ikke tilstrekkelig avveid mot formålene med inngrepet...

  6. En av gutta

    DEFF Research Database (Denmark)

    Neumann, Cecilie Basberg; Rysst, Mari; Bjerck, Mari

    2012-01-01

    Hvilken betydning har kjønn og klær for kvinner som arbeider i mannsdominerte arbeiderklasseyrker? Forfatterne av denne artikkelen finner at kvinnene må nedtone sitt kjønn og sin seksualitet gjennom å dekke til kroppen, i klær laget for menn, for å signalisere at de er på jobb for å arbeide...

  7. En av gutta

    DEFF Research Database (Denmark)

    Neumann, Cecilie Basberg; Rysst, Mari; Bjerck, Mari

    2012-01-01

    Hvilken betydning har kjønn og klær for kvinner som arbeider i mannsdominerte arbeiderklasseyrker? Forfatterne av denne artikkelen finner at kvinnene må nedtone sitt kjønn og sin seksualitet gjennom å dekke til kroppen, i klær laget for menn, for å signalisere at de er på jobb for å arbeide...

  8. Introduction to AVS Audio

    Institute of Scientific and Technical Information of China (English)

    Hao-Jun Ai; Shui-Xian Chen; Rui-Min Hu

    2006-01-01

    This paper describes a general audio coding algorithm which has been recently standardized by AVS, China.The algorithm is based on a perceptual coding technique. The codec delivers near CD-quality audio at 128kb/s. This paper describes the coder structure in detail and discusses the reasons for specific design methods. A summary of the subjective test results are presented for the prototype codec. Comparison Mean Opinion Score (CMOS) test indicates that the quality of the AVS audio coder is comparable with MPEG Layer-3 audio coder. A real-time decoder was used for the characterization test,which is based on a 16-bit fixed-point DSP. The performance of the DSP solution was demonstrated, including computational complexity and storage characteristics.

  9. Negotiation Mechanism of AVS Standardization

    Institute of Scientific and Technical Information of China (English)

    Su Junbin

    2007-01-01

    @@ 1. Introduction The mission of the Audio Video Coding Standard (AVS) Working Group of China is to develop AVS standards drafts and deliver them to the national standardization administration authorities. Since its establishment in 2002, the AVS Working Group has developed rapidly, growing from 33 members in 2003 to 153 in 2006. By the end of 2006, it had put forward for approval nine standards drafts (Table 1) with Part 2 of the AVS 1.0 draft approved as a national standard in February 2006.

  10. Læring av erfaring?

    DEFF Research Database (Denmark)

    Ramvi, Ellen

    Hva skal til for å lære av erfaring? Denne avhandlingen er en empirisk baseret undersøkelse av ungdosmskolelæreres muligheter og betingelser for å "lære av erfaring" i den særlige betydning av begrepet  W. Bion gir i sin psykoanalytiske teori. Undersøgelsen baserer seg på et feltarbeid som strekker...... seg over ett skoleår. Datamaterialet ble samlet inn via observerasjoner og mer eller mindre strukturerte samtaler med lærere ved to forskellige norske ungdsomsskoler. Analysen av materialet foregår i to trinn: først en fenomenologisk analyse, tett på lærernes egne beskrivelser og refleksjoner av...... lærerarbeidet, dets udfordringer og vanskeligheter, og deretter en psykoanalytisk perspektivering av de fenomenologiske analyseresultatene. Analysen viser en almindelig lærerhverdag og en skoleorganisation, hvor lærernes "læring av erfaring" i stor utstrekning blokkeres i et vekselspill mellem lærernes flukt...

  11. Ultralydfremstilling av fostervekst

    Directory of Open Access Journals (Sweden)

    Synnøve Lian Johnsen

    2009-09-01

    Full Text Available Ultralyd billeddanning er i dag den beste undersøkelsesmetoden vi har til å overvåke fosterets utvikling og vekst. Ultralydmåling av fosterets størrelse og biometriske referansetabeller for svangerskapet brukes til å fastsette svangerskapets alder, beregne termin og vurdere fosterets vekst. Optimal studiedesign er ulik for hvert av disse formålene. Det er stor variasjon i fysiologisk vekst og en utfordring å identifisere fostre som har dårlig tilvekst. En enkeltmåling av fosteret kan fortelle oss om det er lite eller stort i forhold til andre fostre, men ikke om størrelsen er passelig i forhold til forventet fysiologisk vekst for dette fosteret. Tilpassede referansemodeller ("customised" justerer forventet størrelse i relasjon til føtale og maternelle faktorer og har vist seg å bedre identifisere sanne veksthemmede fostre enn bare å klassifisere i henhold til 2,5, 5 eller 10 persentilen. Men et foster som i utgangspunktet er stort for alderen, men etter hvert vokser langsomt vil ikke nødvendigvis bli fanget opp ved en slik enkeltmåling. Serielle målinger vil hjelpe, men en slik longitudinell metode blir ikke fullt ut utnyttet før det appliseres betinget ("conditional" vekstberegning. Betingelsesleddene for variasjon i vekst og målemetode kalkuleres ut fra longitudinelle data og kan så anvendes for det enkelte foster. En første måling brukes til å beregne forventet vekst og variasjon for en neste måling. Denne metoden forventes å skjerpe diagnostikken for vekst-avvik i alle vektklasser og kan sammen med tilpasningen av andre faktorer bedre overvåkningen av risikosvangerskapUltrasonographic imaging is today the best method for assessing fetal size and monitoring fetal growth. Ultrasound measurements of fetal size are used for age, size and growth assessment, but the statistical design and analysis are different for each of these purposes. Physiological ranges for fetal growth are wide and the identification of fetal

  12. Teratogene effekter av antiepileptika

    Directory of Open Access Journals (Sweden)

    Bernt A. Engelsen

    2009-10-01

    Full Text Available  SAMMENDRAGCa. 1 av 200 gravide har epilepsi. Gravide med epilepsi har økt risiko for visse obstetriske komplikasjonerog for å føde barn med medfødte misdannelser. Risikoen for misdannelser synes koblet til bruk avantiepileptika under svangerskapet, og ikke til selve epilepsien. Alle typer misdannelser er økt, men leppeganespalteog nevralrørsdefekter utgjør særlig viktige misdannelser. Årsakene til misdannelsene er multifaktorielle.Bruk av antiepileptika i monoterapi kan sies å gi en individuell risiko for større misdannelser påca. 4-6%. Karbamazepin og natriumvalproat gir hhv. 0,5-1% og 2-3% risiko for nevralrørsdefekt. Samletrisiko for større og mindre anomalier inkludert dysmorfe ansiktstrekk synes ikke å overstige 10%. ENGLISH SUMMARYEngelsen BA. Teratogenic effects of antiepileptic drugs. Nor J Epidemiol 1997; 7 (1: 23-28.Approximately 1 in 200 pregnant women have epilepsy, and 1 in 250 births are to children of mothers whouse antiepleptic drugs (AED. Pregnant women with epilepsy have increased risk for certain obstetricalcomplications, and for giving birth to children with congenital malformations. The increased risk forcongenital malformations seems connected to the use of AED, not to the epileptic syndromes. The etiologyof congenital malformations are multifactorial. Use of AED in monotherapy is associated with anindividual risk of giving birth to a child with a major malformation of 4-6%. The specific risk of spinabifida is 0,5 to 1% for carbamazepine and 2-3% for sodium valproate.

  13. Helseeffekter av byluftpartikler

    Directory of Open Access Journals (Sweden)

    Magne Refsnes

    2009-10-01

    Full Text Available Svevestøv i byluft består av forbrenningspartikler og mineralpartikler med svært forskjellige størrelser og kjemiske egenskaper. Svevestøvet kan deles inn i størrelsesfraksjoner som PMAmbient particulate matter (PM comprises particles from different combustion processes and a variety of mineral particles. The particles vary widely in size distribution and chemical/physical characteristics. PM is often divided into size fractions with different aerodynamic diameters: PM10 (PM ! 10 mm, PM2.5 (PM ! 2.5 mm and PM0.1 (PM ! 0.1 mm. Recent population studies have found an association between an increase in mortality and morbidity due to lung and/or cardiovascular disease and short-term increases in PM. The relative risk (RR was approximately 1.005 for an increase in 10 mg/m3 PM10, without an observed threshold even at concentrations below 10 mg/m3. Chronic exposure has been investigated to a lesser extent, but longterm exposure to PM2.5 has been found to be associated with an approximately 10-fold greater increase in RR than short-term exposures. Experimental studies with volunteers in chamber and field studies show mild lung or cardiovascular responses at concentrations of ambient particles (PM2.5/PM10: 100-200 mg/m3 that may occur during episodes of air pollution. Animal studies at higher concentrations have shown stronger responses. The experimental studies support the epidemiological evidence for an adverse health effect of PM. Both population- and experimental studies indicate the existence of vulnerable individuals. At low to average ambient concentrations there seems to exist a discrepancy between the results of population- and experimental studies that might be due to the absence of the most vulnerable individuals in the experimental studies. Together with cell culture experiments, human and animal studies indicate the importance of physical and chemical properties of the particles (size, content of metals, organics, endotoxins, etc. for

  14. Lokalisering av arbeidsplasser og boliger

    DEFF Research Database (Denmark)

    Næss, Petter

    2011-01-01

    En miljømessig effektiv lokalisering av arbeidsplasser og boliger sikter mot å begrense - og helst redusere - biltrafikk og miljøproblemer knyttet til bytransport, sammenliknet med alternative lokaliseringer. En effektiv transportmessig lokalisering kan også bidra til å redusere tap av verdifulle...... arealer (f eks jordbruksareal eller natur- og friluftsområder) utenfor dagens tettstedsgrense. Tiltaket må samtidig utformes slik at en kan bevare viktige bomiljøkvaliteter og så mye som mulig av de grønne arealene innenfor tettbebyggelsen....

  15. Lokalisering av arbeidsplasser og boliger

    DEFF Research Database (Denmark)

    Næss, Petter

    2011-01-01

    En miljømessig effektiv lokalisering av arbeidsplasser og boliger sikter mot å begrense - og helst redusere - biltrafikk og miljøproblemer knyttet til bytransport, sammenliknet med alternative lokaliseringer. En effektiv transportmessig lokalisering kan også bidra til å redusere tap av verdifulle...... arealer (f eks jordbruksareal eller natur- og friluftsområder) utenfor dagens tettstedsgrense. Tiltaket må samtidig utformes slik at en kan bevare viktige bomiljøkvaliteter og så mye som mulig av de grønne arealene innenfor tettbebyggelsen....

  16. Store Stokkavatn. Beskrivelse av forurensningssituasjonen

    OpenAIRE

    Holtan, H.

    1985-01-01

    Rapporten, som er et ledd i arbeidet med en vannbruksplan for Store Stokkavatn ved Stavanger, sammenstiller og diskuterer data som er samlet inn fra innsjøen og dens tilløp av Byveterinæretaten i Stavanger. Store Stokkavatnet er reservevannkilde for Stavanger og dessuten er innsjøen sentral i rekreasjonsmessig sammenheng. På bakgrunn av de foreliggende data, er innsjøens forurensningstilstand på grensen av det akseptable hva algeproduksjon (eutrofiering) og bakteriologiske forhold angår. Fler...

  17. Optimering av bergtransporter i underjordsgruva

    OpenAIRE

    Abrahamsson, Magnus

    2015-01-01

    Detta examensarbete utfördes på LKAB, en mineralkoncern som tillverkar och levererar järnmalmsprodukter. Arbetet utfördes för företagets underjordsgruva i Malmberget och behandlar tillredningens bergtransporter. Tillredningen är den process som förbereder nya delar av gruvan för järnmalmsbrytning. Målsättningen med arbetet har varit att kartlägga hur transporterna av tillredningsberg styrs samt att kvantifiera en besparingspotential för dessa transporter. Om möjligt skulle även förbättringsfö...

  18. Institusjonelle felt og forståelser av evaluering – en analyse av evalueringen av NAV-reformen

    DEFF Research Database (Denmark)

    Halvorsen, Anne; Breidahl, Karen Nielsen; Hansen, Morten Balle

    2016-01-01

    I Norge ble det i årene 2006-2009 gjennomført en omfattende reform med etablering av en ny arbeids- og velferdstjenestene (NAV). Reformen ble gjort til gjenstand for en omfattende evaluering, EVANAV, over flere år. I evalueringens siste fase ble det iverksatt en evaluering av evalueringen av...... interessene som var involvert i evalueringen. I EVANAV ble dette blant annet ivaretatt i formuleringen av fire mål som tilgodeser ulike behov. Basert på evalueringsteori og teori om institusjonelle felt bidrar analysen til en bedre forståelse av hva evaluering er, og hvordan ulike utfordringer knyttet til...

  19. PAH i forurenset sediment: Utredning av egnethet av PAHkomponenter/ grupperinger for vurdering av tiltaksbehov

    OpenAIRE

    Ruus, Anders; Arp, Hans Petter Heinrich; Oen, Amy M.P.; Grung, Merete; Breedveld, Gijs D.

    2015-01-01

    I Norge er det utarbeidet klassegrenser for til sammen 16 PAH-forbindelser i sediment og vann. Grenseverdiene baserer seg på kunnskap om toksisitet av de ulike stoffene og hva som er akseptabel eksponering for miljøet. I tillegg til grenseverdiene for enkeltkomponentene av PAH opererer norske miljømyndigheter med klassegrenser for PAH-grupperingen SUM PAH-16 i sediment. Disse klassegrensene bygger på overkonsentrasjoner i forhold til bakgrunnsnivå, og ikke økotoksikologiske dat...

  20. Bruk av kartleggingsresultater i forbedringsarbeid

    DEFF Research Database (Denmark)

    Nordahl, Thomas; Hansen, Line Skov

    . Barnehagen har gjerne tilgang på informasjon fra kartlegginger, men det er ikke alltid denne informasjonen blir systematisk analysert og aktivt brukt i forbedringsarbeid. Forfatterne viser hvordan slike analyser kan gjennomføres på forskjellige nivåer i den enkelte barnehage, og vektlegger viktigheten av...

  1. Analyse av klimatilpasningstiltak : en casestudie av overvannsnettet på Bogafjell i Sandnes kommune

    OpenAIRE

    Bekkum, Ina

    2012-01-01

    For å simulere klimaendringenes effekt på avløpssystemet og klimatilpasningstiltakenes avbøtende virkning kan man benyttes seg av urbanhydrologiske avløpsmodeller. I denne oppgaven er det gjennomført et casestudie av overvannsnettet på Bogafjell i Sandnes kommune. Det er gjort simuleringer av enkeltregn i form av symmetriske nedbørshyetogram, med og uten klimatillegg – i Rosie/ArcGIS med MOUSE beregningsmotor – for å teste tiltaksprinsipper som frakopling av overvann, fordrøyning av ...

  2. Transmembrane potentials of canine AV junctional tissues.

    Science.gov (United States)

    Tse, W W

    1986-06-01

    The atrioventricular (AV) junction comprises the AV node, His bundle (HB), and specialized tissues proximal to the node called paranodal fibers (PNF). In the present study, an in vitro, dissection-exposed canine right atrial (RA), transitional fiber (TF), AV junctional preparation was used. The TF and PNF formed a pathway running along the base of the septal cusp of the tricuspid valve (SCTV). In the first experiment, impulses elicited at the RA were monitored to propagate sequentially through the TF, PNF, AV node, and then the HB. This functional evidence supports the concept that a conduction pathway connecting the RA and the AV node exists along the base of the SCTV. This internodal pathway is referred to as the septal cusp pathway. In another experiment, transmembrane potentials and Vmax were determined on each of the AV junctional tissues. Results showed that PNF had the lowest Vmax (2.5 V/sec), followed by AV node (7.0 V/sec) and HB (33 V/sec). This finding showed that PNF, and not the AV node, has the lowest Vmax, suggesting that the PNF has the lowest conductivity among the AV junctional tissues, and this study advances our understanding on the mechanism of AV conduction delay in dog hearts.

  3. A New Approach for Detection Improvement of the Creutzfeldt-Jakob Disorder through a Specific Surface Chemistry Applied onto Titration Well

    Directory of Open Access Journals (Sweden)

    Dominique Debarnot

    2012-10-01

    Full Text Available This work illustrates the enhancement of the sensitivity of the ELISA titration for recombinant human and native prion proteins, while reducing other non-specific adsorptions that could increase the background signal and lead to a low sensitivity and false positives. It is achieved thanks to the association of plasma chemistry and coating with different amphiphilic molecules bearing either ionic charges and/or long hydrocarbon chains. The treated support by 3-butenylamine hydrochloride improves the signal detection of recombinant protein, while surface modification with the 3,7-dimethylocta-2,6-dien-1-diamine (geranylamine enhances the sensitivity of the native protein. Beside the surface chemistry effect, these different results are associated with protein conformation.

  4. Clinical findings of a probable case of MM2-cortical-type sporadic Creutzfeldt-Jakob disease with antibodies to anti-N-terminus of α-enolase.

    Science.gov (United States)

    Hayashi, Yuichi; Yamada, Megumi; Kimura, Akio; Asano, Takahiko; Satoh, Katsuya; Kitamoto, Tetsuyuki; Yoneda, Maokoto; Inuzuka, Takashi

    2017-10-02

    We report the case of a 76-year-old woman presenting with 47-month history of progressive dementia and cortical blindness with no family history. Antibodies against thyroid glands and the N-terminus of α-enolase (NAE) were detected in her serum. Neurological examination revealed progressive dementia, frontal signs, visual disturbance, and exaggerated bilateral tendon reflexes in both legs. Diffusion MRI showed cortical hyper-intensities in the bilateral occipital and parietal, and the left frontal and temporal cortices. (99m)Tc-ethyl cysteinate dimer-single photon emission computed tomography indicated decreased regional cerebral blood flow throughout the bilateral parietal lobes and partially in the left frontal and temporal lobes. PRNP gene analysis showed no mutations with methionine homozygosity at codon 129 in peripheral blood. Cerebrospinal fluid examination, including 14-3-3 and total tau protein detection, revealed normal levels; however, prion proteins were amplified by the real-time quaking-induced conversion method. Hashimoto's encephalopathy was excluded on the basis of unresponsiveness to corticosteroids. The symptoms progressed slowly. Periodic sharp-wave complexes were observed on electroencephalogram 36 months after the onset of symptoms; the patient reached a state of akinetic mutism at 47 months. This was a probable case of MM2-cortical-type sCJD with anti-NAE antibodies based on the World Health Organization (WHO) diagnostic criteria for sCJD, genetic information, and the slowly progressive course. However, this case did not meet with the probable WHO diagnostic criteria until 3 years after symptom onset, highlighting the difficulty of diagnosing a living case of the MM2-type of sCJD. Therefore, establishment of clinical diagnostic criteria for MM2-type of sCJD is required.

  5. Human Variant Creutzfeldt-Jakob disease and sheep scrapie PrP (res) detection using seeded conversion of recombinant prion protein.

    NARCIS (Netherlands)

    Orrú, C.D.; Wilham, J.M.; Hughson, A.G.; Raymond, L.D.; McNally, K.L.; Bossers, A.; Ligios, C.; Caughey, B.

    2009-01-01

    The pathological isoform of the prion protein (PrPres) can serve as a marker for prion diseases, but more practical tests are needed for preclinical diagnosis and sensitive detection of many prion infections. Previously we showed that the quaking-induced conversion (QuIC) assay can detect sub-femtog

  6. Enfermedad de Creutzfeldt-Jakob: hallazgos clínicos, electroencefalográficos, imagenológicos y de patología

    OpenAIRE

    2010-01-01

    Éste artículo hace parte de: Acta Neurológica Colombianana Vol. 24 No. 3 SEPTIEMBRE 2008 La enfermedad de Creutzfeld-Jakob (ECJ) hace parte de un grupo de enfermedades transmisibles que se caracterizan por la presencia de encefalopatía espongiforme, donde también se encuentran el kuru, el síndrome Gerstmann-Straussler-Scheinker, y el insomnio fatal familiar. De ellas, la más común es la ECJ (representando aproximadamente el 85 por ciento de casos de encefalopatías espongiformes), con una i...

  7. Evaluering av ABW-filter

    OpenAIRE

    Ohren, J.A.

    1986-01-01

    Det er foretatt en evaluering av ABW-filteret basert på forskningsresultater og driftserfaringer fra USA. ABW-filteret er godt egnet med sand eller sand-antrasitt i konvensjonelle fullrenseanlegg med flokkulering, sedimentering og filtrering. Etter disse prosessene er filteret også egnet med aktivt kull. ABW-filteret er også interessant for etterbehandling i kommunale og industrielle avløpsrenseanlegg.

  8. Multimedia for læring av tallsystemer : utvikling av programvare for læring av og om tallsystemer

    OpenAIRE

    Idland, Terje

    2006-01-01

    Oppgaven har en teoretisk forankring innenfor Piagets konstruktivisme, Vygotskys sosialkonstruktivisme, Dienes teori for læring av posisjonssystemet og Mayer & Morenos teori for læring med multimedia. Primært har jeg brukt eksisterende teori som grunnlag for utvikling av pedagogisk programvare for læring av tallsystemer. Denne programvaren er todelt. En del er en interaktiv applett som er et utforskingsmiljø for posisjonssystemet. Den andre delen er en interaktiv multimediep...

  9. Ny versjon av standarden ISO 15489

    Directory of Open Access Journals (Sweden)

    Herbjørn Andresen

    2016-09-01

    Full Text Available Standarden NS-ISO 15489-1:2016 er gjeldende norsk standard fra 1. juli 2016. Dette er andre versjon av ISO 15489, den første versjonen fra 2001 er ikke lenger gyldig. 2001-utgaven av ISO 15489 besto av to deler. Del 2, SN-ISO/TR 15489-2:2001 er trukket, slik at den nye ISO 15489 kun består av del 1. ISO 15489 var den første internasjonale Records Management-standarden, og har en funksjon som overordnet referansestandard for en voksende portefølje av senere standarder på tilgrensende områder. Denne artikkelen gjennomgår endringene i hovedtrekk, både i standarden ISO 15489 som sådan og i noen av de standardene som bygger videre på den.

  10. Effekten av karbohydrat- og proteininntak på restitusjon av utholdenhetskapasiteten etter et utmattende arbeid

    OpenAIRE

    Rustad, Per Inge

    2011-01-01

    Innledning: Dietten i etterkant av trening og konkurranser er av stor betydning for restitusjonsprosessen. Få studier har imidlertid undersøkt restitusjonseffekten av karbohydratog proteininntak etter et utmattende arbeid på utholdenhetskapasiteten dagen etter. I vår studie ble det gjennomført totalt tre diettintervensjoner for å undersøke følgende problemstillinger; 1) undersøke utholdenhetskapasiteten dagen etter et utmattende utholdenhetsarbeid etter inntak av en høy karbohy...

  11. AVS-M: From Standards to Applications

    Institute of Scientific and Technical Information of China (English)

    Ye-Kui Wang

    2006-01-01

    AVS stands for the Audio Video coding Standard Workgroup of China, who develops audio/video coding standards as well as system and digital right management standards. AVS-M is the AVS video coding standard targeting for mobile multimedia applications. Besides the coding specification, AVS also developed the file format and Real-time Transport Protocol (RTP) payload format specifications to enable the application of AVS-M video in various services. This paper reviews the high-level coding tools and features of the AVS-M coding standard as well as the file format and payload format standards.In particular, sixteen AVS-M high-level coding tools and features, which cover most of the high-level topics during AVS-M standardization, are discussed in some detail. After that, the error resilience tools are briefly reviewed before the file format and RTP payload format discussions. The coding efficiency and error resiliency performances of AVS-M are provided finally.H.264/AVC has been extensively used as a comparison in many of the discussions and the simulation results.

  12. The AV-8B Decision

    Science.gov (United States)

    1982-06-01

    isa csestudy of the debate overthdeionfte United States Gover mn to prc--u the McDonnell Douglas AV-8B Advanced Hrrier V/STOL jet aircraft for the U.S...Sciences 3 ABSTRACT This thesis is a case study of the debate over the decision of the United States Government to procure the McDonnell Douglas IT-8...a egrate4 dur nq the 1ive To r perIoo by the introduction of the moderNtely prcod F1-18 aircraft. The F/1 8 proqrts will, 0euce to*n uber and tpes of

  13. Normal Pressure Hydrocephalus

    Science.gov (United States)

    ... similar to those of other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, the disorder is ... similar to those of other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, the disorder is ...

  14. Gerstmann-Straussler-Scheinker Disease

    Science.gov (United States)

    ... include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia. Treatment There is no cure for GSS, nor are ... include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia. Treatment There is no cure for GSS, nor are ...

  15. PREPARATION FOR RETIREMENT - AVS SEMINAR

    CERN Multimedia

    Social Service

    2001-01-01

    The 500 or so participants in the fifth Preparation for Retirement seminar held at the end of March were unfortunately deprived of the planned session on the AVS due to the unavailability of the Director of the Caisse Cantonale Genevoise de Compensation (CCGC). We have since had formal confirmation that because of an extra workload due to important changes in the Swiss tax and social legislation and the implementation this summer of the maternity insurance in Geneva, the CCGC has suspended its participation in preparation for retirement seminars in the international organisations for the time being. Conscious of the necessity of offering a session dedicated to the AVS, it is with pleasure that we can inform you that one of our legal advisers, Mr Lorenz Stampfli, has accepted to lead this session. In order to allow for adequate preparation we have reserved the following date: Wednesday 26 September from 14.00 to 16.00 in the Main Amphitheatre The session will be open to all people already registered and any o...

  16. Syncope and Idiopathic (Paroxysmal) AV Block.

    Science.gov (United States)

    Brignole, Michele; Deharo, Jean-Claude; Guieu, Regis

    2015-08-01

    Syncope due to idiopathic AV block is characterized by: 1) ECG documentation (usually by means of prolonged ECG monitoring) of paroxysmal complete AV block with one or multiple consecutive pauses, without P-P cycle lengthening or PR interval prolongation, not triggered by atrial or ventricular premature beats nor by rate variations; 2) long history of recurrent syncope without prodromes; 3) absence of cardiac and ECG abnormalities; 4) absence of progression to persistent forms of AV block; 5) efficacy of cardiac pacing therapy. The patients affected by idiopathic AV block have low baseline adenosine plasma level values and show an increased susceptibility to exogenous adenosine. The APL value of the patients with idiopathic AV block is much lower than patients affected by vasovagal syncope who have high adenosine values. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Review of AVS Audio Coding Standard

    Institute of Scientific and Technical Information of China (English)

    ZHANG Tao; ZHANG Caixia; ZHAO Xin

    2016-01-01

    Audio Video Coding Standard (AVS) is a second⁃generation source coding standard and the first standard for audio and video coding in China with independent intellectual property rights. Its performance has reached the international standard. Its coding efficiency is 2 to 3 times greater than that of MPEG⁃2. This technical solution is more simple, and it can greatly save channel resource. After more than ten years ’develop⁃ment, AVS has achieved great success. The latest version of the AVS audio coding standard is ongoing and mainly aims at the increasing demand for low bitrate and high quality audio services. The paper reviews the history and recent develop⁃ment of AVS audio coding standard in terms of basic fea⁃tures, key techniques and performance. Finally, the future de⁃velopment of AVS audio coding standard is discussed.

  18. Tolkning av det verbala och det visuella

    Directory of Open Access Journals (Sweden)

    Barbro Sjöberg

    2011-12-01

    Full Text Available I en tidigare publicerad artikel i Techne serien (Sjöberg, 2005 gjordes en genre-beskrivning av fenomenet skissbok. Genrebeskrivningen baserade sig på en analys av sex formgivarstuderandes skissböcker. I den nu aktuella artikeln redogörs för hur genren tillämpats i en slöjdpedagogisk kontext inom två kurser i Konsthand-arbete under åren 2002 respektive 2004 inom de didaktikbaserade ämnesteknolo-giska studierna i slöjdpedagogik vid Åbo Akademi, Vasa. En av målsättningarna för kurserna var att testa skissboken i en slöjdpedagogisk kontext. Under kursens gång hade de studerande som uppgift att utforska och dokumentera ett givet tema i sina skissböcker. Efter avslutad kurs skannades skissböckerna in för att utgöra empiriskt material för tolkning och analys av hur de studerande utformat dokumen-tationen i sina skissböcker på basen av de instruktioner de fått under kursens gång. I föreliggande studie redogörs för bearbetning, tolkning och analys av det empiris-ka materialet med hjälp av NVivo 9, en version av NVivo som underlättar bearbet-ning och den vetenskapliga tolkningen av visuellt material. Resultaten visar att uppmuntran till horisontell mediering i samband med dokumentation i skissbok kan leda till en mångsidig och personlig utforskning av givet tema.Sökord: metod, slöjd, NVivo, skissbok, lärarutbildningURN:NBN:no-29968

  19. Påvising av aminoglykosidresistens ved hjelp av massespektrometri. Utvikling av metode for deteksjon, stabilitet av aminoglykosid og molekylær karakterisering av teststammer

    OpenAIRE

    Kvarekvål, Torunn

    2014-01-01

    Aminoglykosid er potente antibiotika som vert brukt til behandling av alvorlege infeksjonar med aerobe bakteriar. Dei er baktericide og verkar ved å binda til ribosoma i bakteriane og på denne måten forstyrra den normale peptidsyntesen. Aminoglykosida er verdifulle medikament, men som for mange andre antibiotika er resistens mot desse midla eit aukande problem. Den viktigaste resistensmekanismen er aminoglykosidmodifiserande enzym (AME). Desse enzyma inaktiverer ...

  20. Uptake of AV-1451 in meningiomas.

    Science.gov (United States)

    Bruinsma, Tyler J; Johnson, Derek R; Fang, Ping; Senjem, Matthew; Josephs, Keith A; Whitwell, Jennifer L; Boeve, Bradley F; Pandey, Mukesh K; Kantarci, Kejal; Jones, David T; Vemuri, Prashanthi; Murray, Melissa; Graff-Radford, Jonathan; Schwarz, Christopher G; Knopman, David S; Petersen, Ronald C; Jack, Clifford R; Lowe, Val J

    2017-09-08

    AV-1451 is an imaging agent labeled with the positron-emitting radiolabel Fluorine-18. 18F-AV-1451 binds paired helical filament tau (PHF-tau), a pathology related to Alzheimer's disease. In our study of AV-1451 uptake in the brains of cognitively normal subjects, we noted a case of a meningioma with visually significant uptake of AV-1451. We initiated the present retrospective study to further examine cases of meningioma that underwent AV-1451 imaging. We searched the patient records of 650 patients who had undergone AV-1451 at our institution for the keyword "meningioma" to identify potential cases. PET/CT and MRI results were visually reviewed and semi-quantitative analysis of PET was performed. A paired student's t test was run between background and tumor standard uptake values. Fisher's exact test was used to examine the association between AV-1451 uptake and presence of calcifications on CT. We identified 12 cases of meningioma, 58% (7/12) of which demonstrated uptake greater than background using both visual analysis and tumor-to-normal cortex ratios (T/N + 1.90 ± 0.83). The paired student's t test revealed no statistically significant difference between background and tumor standard uptake values (p = 0.09); however, cases with a T/N ratio greater than one showed statistically higher uptake in tumor tissue (p = 0.01). A significant association was noted between AV-1451 uptake and presence of calcifications (p = 0.01). AV-1451 PET imaging should be reviewed concurrently with anatomic imaging to prevent misleading interpretations of PHF-tau distribution due to meningiomas.

  1. Portering av programvara – metodik och fallstudie

    OpenAIRE

    Ståhl, Nils

    2012-01-01

    Carasoft AB är ett företag som specialiserat sig på utveckling av dokumenthanteringssystem. Man erbjuder bland annat ett Windowsbaserat dokumenthanteringssystem vid namn Caradoc. Systemet består av flertalet DLL:er skrivna i Delphi och har inte ändrats eller kompilerats sedan 2004. Det här examensarbetet har i syfte att utreda om det finns möjlighet att inom rimlig tid portera Caradoc till Windows 7, med hjälp av den nya Delphi-versionen XE2. Efter en förstudie i ämnet portering, programspråk...

  2. Basic Considerations on AVS DRM Architecture

    Institute of Scientific and Technical Information of China (English)

    Tie-Jun Huang; Yong-Liang Liu

    2006-01-01

    Digital Rights Management (DRM) is an important infrastructure for the digital media age. It is a part of the AVS (Audio and Video coding Standard) of China. AVS Trusted Decoder (ATD) that plays back digital media program according to rights conditions is the core of AVS DRM architecture. Adaptation layers are responsible for translating or negotiating between ATD and peripheral systems. The Packaging Adaptation Layer (PAL), Licensing Adaptation Layer (LAL) and Rendering Adaptation Layer (RAL) will help ATD to gain the interoperability in various DRM environments.

  3. Outcomes of AV Fistulas and AV Grafts after Interventional Stent-Graft Deployment in Haemodialysis Patients.

    Science.gov (United States)

    Schmelter, Christopher; Raab, Udo; Lazarus, Friedrich; Ruppert, Volker; Vorwerk, Dierk

    2015-08-01

    The study was designed to assess outcomes of arteriovenous (AV) accesses after interventional stent-graft deployment in haemodialysis patients. 63 haemodialysis patients with 66 AV fistulas and AV grafts were treated by interventional stent-graft deployment from 2006 to 2012 at our hospital. Data of these patients were retrospectively analysed for location of deployed stent-grafts, occurrence and location of (re-)stenosis and (re-)thrombosis. Complex stenosis was the most frequent indication for stent-graft deployment (45.5%), followed by complications of angioplasty with vessel rupture or dissection (31.8%). A high rate of procedural success was achieved (98.5%). The most frequent location of the deployed stent-graft was the draining vein (66.7%). Stent-graft deployment was more frequent in AV grafts than in AV fistulas. Primary patency was 45.5% at 6 month, 31.3% at 12 month and 19.2% at 24 month. Primary patency was significantly better for AV fistulas than for AV grafts with deployed stent-grafts. Patency of the deployed stent-graft was much better than overall AV access primary patency with deployed stent-graft. Re-stenosis with thrombosis was the most frequent indication for re-intervention. Most frequent location of re-stenosis was the draining vein (37.1%), followed by stenosis at the AV access (29.5%) and the deployed stent-graft (23.5%). Re-stenosis and re-thrombosis remain frequent in AV fistulas and AV grafts in haemodialysis patients despite stent-graft deployment. Re-stenosis of the deployed stent-graft is, only in the minority of the cases, responsible for AV access dysfunction.

  4. Outcomes of AV Fistulas and AV Grafts after Interventional Stent-Graft Deployment in Haemodialysis Patients

    Energy Technology Data Exchange (ETDEWEB)

    Schmelter, Christopher, E-mail: christopher.schmelter@klinikum-ingolstadt.de; Raab, Udo, E-mail: udo.raab@klinikum-ingolstadt.de [Klinikum Ingolstadt, Department of Diagnostic and Interventional Radiology (Germany); Lazarus, Friedrich, E-mail: friedrich.lazarus@klinikum-ingolstadt.de [Klinikum Ingolstadt, Department of Nephrology (Germany); Ruppert, Volker, E-mail: volker.ruppert@klinikum-ingolstadt.de [Klinikum Ingolstadt, Department of Vascular Surgery (Germany); Vorwerk, Dierk, E-mail: dierk.vorwerk@klinikum-ingolstadt.de [Klinikum Ingolstadt, Department of Diagnostic and Interventional Radiology (Germany)

    2015-08-15

    PurposeThe study was designed to assess outcomes of arteriovenous (AV) accesses after interventional stent-graft deployment in haemodialysis patients.Materials and Methods63 haemodialysis patients with 66 AV fistulas and AV grafts were treated by interventional stent-graft deployment from 2006 to 2012 at our hospital. Data of these patients were retrospectively analysed for location of deployed stent-grafts, occurrence and location of (re-)stenosis and (re-)thrombosis. Complex stenosis was the most frequent indication for stent-graft deployment (45.5 %), followed by complications of angioplasty with vessel rupture or dissection (31.8 %).ResultsA high rate of procedural success was achieved (98.5 %). The most frequent location of the deployed stent-graft was the draining vein (66.7 %). Stent-graft deployment was more frequent in AV grafts than in AV fistulas. Primary patency was 45.5 % at 6 month, 31.3 % at 12 month and 19.2 % at 24 month. Primary patency was significantly better for AV fistulas than for AV grafts with deployed stent-grafts. Patency of the deployed stent-graft was much better than overall AV access primary patency with deployed stent-graft. Re-stenosis with thrombosis was the most frequent indication for re-intervention. Most frequent location of re-stenosis was the draining vein (37.1 %), followed by stenosis at the AV access (29.5 %) and the deployed stent-graft (23.5 %).ConclusionRe-stenosis and re-thrombosis remain frequent in AV fistulas and AV grafts in haemodialysis patients despite stent-graft deployment. Re-stenosis of the deployed stent-graft is, only in the minority of the cases, responsible for AV access dysfunction.

  5. Integrasjon av vindkraft i energisystemet : effekten av variabilitet på markedsverdien

    OpenAIRE

    Brekken, Tormod Ween

    2014-01-01

    Klimapolitikken bidrar til å fase ut termisk kraftproduksjon basert på fossile kilder. Dette kan føre til at en mindre andel av kraftproduksjonen kan respondere på prissignaler. Det er planlagt å øke andelen vindkraft i flere kraftmarkeder. Vindkraft er avhengig av været, og er karakterisert av varierende produksjon. Dette påvirker inntekten til hva produsenter av vindkraft vil tjene i kraftmarkedet. Studier har funnet at markedsverdien til vindkraft reduseres ved økte andeler vindkraft i ma...

  6. Forandring av selvoppfatning blant studenter i løpet av en bachelorutdanning i sykepleie

    Directory of Open Access Journals (Sweden)

    John Olav Bjørnestad

    2010-01-01

    Full Text Available Selvoppfatning er av betydning for faglig framgang og kreativitet. Hensikten med denne studien var å beskrive forandring av selvoppfatning hos en gruppe sykepleierstudenter i løpet av sin treårige bachelorutdanning. En studiegruppe med 127 sykepleierstudenter ved en norsk høgskole besvarte et spørreskjema i første og siste studieår. Skalaer for måling av generell selvoppfatning og regelbevissthet samt spørsmål om bakgrunnsvariabler inngikk i spørreskjemaet. Resultatene viste at selvoppfatningen økte signifikant i løpet av utdanningen i den totale studiegruppen, og viktige faktorer var barn og opplevelse av tilfredshet. For ca 45% av deltakerne var selvoppfatningen enten lavere eller uforandret. Regelbevissthet minket i løpet av utdanningen og var ikke assosiert til studentenes selvoppfatning. Fortsatt forskning behøves for å klarlegge mulige påvirkningsfaktorer og for å finne tiltak som kan fremme en positiv utvikling av selvoppfatning hos studenter.

  7. En norsk pilotstudie av helsesøstres oppfatninger av pasienters health literacy: helsefremmende allmenndannelse

    OpenAIRE

    Hanne Søberg Finbråten; Kjell Sverre Pettersen

    2012-01-01

    Bakgrunn Uttrykket “ansvar for egen helse” innebærer også evnen til kritisk evaluering av helseinformasjon. Slike kunnskaper og ferdigheter reflekterer health literacy (HL) – på norsk; helsefremmende allmenndannelse, som handler om å forstå, kunne skaffe seg tilgang på, kritisk vurdere og adekvat anvende helseinformasjon for ervervelse av god helse. Hensikt Formålet var å kartlegge helsesøstres oppfatninger av brukeres tre hierarkiske nivåer av HL (Nutbeam 2000): functional HL (FHL), inte...

  8. Dicty_cDB: FC-AV24 [Dicty_cDB

    Lifescience Database Archive (English)

    Full Text Available FC (Link to library) FC-AV24 (Link to dictyBase) - - - Contig-U16482-1 FC-AV24E (Li...nk to Original site) - - - - - - FC-AV24E 591 Show FC-AV24 Library FC (Link to library) Clone ID FC-AV24 (Li.../dictycdb.biol.tsukuba.ac.jp/CSM/FC/FC-AV/FC-AV24Q.Seq.d/ Representative seq. ID FC-AV...24E (Link to Original site) Representative DNA sequence >FC-AV24 (FC-AV24Q) /CSM/FC/FC-AV/FC-AV24Q.Seq....RFWYFLSKIVKMKKSTGEIL NVTEIFEDKPQKVKNFGVFIRYNSRSGTHNIYKEYRDLTRCGAVSQMYDEMASRHSARES SIHIIDIKEIAASLTRRANTKQFHDS

  9. Kartlegging av PCB i sedimenter fra Indre Sørfjord

    OpenAIRE

    Skei, J.; Klungsøyr, J

    1990-01-01

    Som følge av forhøyede nivåer av PCB i fiskelever innerst i Sørfjorden er det gjennomført en sedimentundersøkelse for om mulig å finne kilden til PCB. Det ble ikke registrert høye nivåer av PCB i sedimentene. Høyeste konsentrasjoner ble målt i munningen av Eitrheimsvågen. Analyser av trafooljer brukt i Tyssedalsområdet viste spor av PCB.

  10. Utvärdering av event marketing

    OpenAIRE

    Fransson, Erik; Törnqvist, Johan

    2001-01-01

    Event marketing är en marknadsföringsform som har växt ordentligt under senare delen av 90-talet och det börjar bli ett naturligt inslag i företagens marknadskommunikation. Trots denna tillväxt har event marketing under en längre tid sammankopplats med brist på utvärderingsmetoder. En avsaknad av utvärderingsmetoder leder till svårigheter att avgöra vilka effekter en event marketing-satsning resulterar i, hur genomförandet kan förbättras samt huruvida event marketing är en lämplig form av mar...

  11. Eksakte metodar for analyse av tovegstabellar

    OpenAIRE

    Aaberge, Rolf

    1980-01-01

    Dei fleste matematisk-statistiske metodane som er utvikla til analyse av tabellar, byggjer på føresetnader om at talet på observasjonar i tabellcellene er "stort. Haldorsen (1977a) og (1977b) omtalar metodar som kviler på dette kravet. I denne rapporten skal vi presentere eksakte metodar for analyse av to-vegstabel lar, dvs. metodar som er gyldige sjølv om vi har småe observasjonstal i tabellcellene. I mange undersøkingar vil observasjonane ofte gi uttrykk for kva slags ...

  12. Grafisk presentasjon av GPenSim-simulering

    OpenAIRE

    2009-01-01

    GPenSim er et verktøy for modellering og simulering av diskret hendelsesystemer (DES). GPenSim er integrert i Matlab-plattformen, og har dermed tilgang til innebygde Matlabfunksjoner som plot etc. I GPenSim blir Petri net-grafen definert i Petri netdefinisjonsfiler. Resultatet av en simulering blir vist i tekst. Oppgaven gikk ut på å utvikle et verktøy som skulle presentere både Petri net-grafen og simuleringsresultatet grafisk. En grafisk presentasjon viser tydeligere sammenhe...

  13. AVS Intellectual Property Rights (IPR) Policy

    Institute of Scientific and Technical Information of China (English)

    Cliff Reader

    2006-01-01

    The AVS Workgroup has developed an IPR Policy to facilitate the adoption of standards in the marketplace.The policy is based on consideration of IPR issues in parallel with the technical work for drafting the standard. The paper describes the relationship between IPR and the standard, and how the goals for the standard must be complemented by goals for the IPR. The existing IPR policies of the ITU and ISO are outlined, and then the AVS IPR policy is described,organized by its three main components: commitment to license on declared basic terms, disclosure of intellectual property,and protection of IPR.

  14. Bruk av landstrøm for reduksjon av NOX og CO2 i norske havner

    OpenAIRE

    Frengstad, Åsmund Møll

    2014-01-01

    Bruk av Smart Grid og landstrøm for reduksjon av NOX- og CO2- utslipp i norske havner. I dag slippes det ut mye avgasser fra marine næringer i havner. Disse gassene bidrar både til internasjonale miljøutslipp i form av CO2, men også til lokal luftforurensing. Dette kan føre til redusert livskvalitet for befolkningen og besøkende til havnebyene. For å belyse problemet er Hurtigruten ASA benyttet som eksempel med de havnene den besøker på sin rute opp langs norskekysten. Rapporten gir en...

  15. Kunstpunkalliansen : En alternativ estetisk kontinuitet fra slutten av 1960-tallet til begynnelsen av 1990-tallet

    OpenAIRE

    Storsve, Gaute

    2007-01-01

    I denne oppgaven har jeg forsøkt å vise at det finnes en alternativ estetisk kontinuitet innenfor populærmusikken fra slutten av 1960-tallet til begynnelsen av 1990-tallet. Det som kjennetegner denne kontinuiteten er ulike former for krysninger av punkelementer med en sterk kunstbevissthet. Jeg valgte å kalle dette for kunstpunkalliansen. For å illustrere hva kunstpunkalliansen er har jeg analysert tre låter fra tre forskjellige band fra tre forskjellige tiår: The Velvet Undergrounds «Ven...

  16. Ishockeykjelke - Innfesting av spilleren: Utvikling av ny kjelke til det norske kjelkehockeylandslaget

    OpenAIRE

    2013-01-01

    Kjelkehockey er basert på de samme prinsippene som ishockey, men er rettet mot utøvere med funksjonsnedsettelser i nedre del av kroppen. Utøverne sitter i en tilpasset kjelke med skøytestål på undersiden av bakre del av kjelken. De har en tilpasset hockeykølle i hver hånd, som har to funksjoner, i den ene enden er det pigger til å stake seg frem med på isen, og den andre enden ligner en vanlig hockeykølle og brukes til å håndter...

  17. Instrumentering av Bergsøysundbrua og Gjemnesundbrua

    OpenAIRE

    Dahlen, Adrian; Lystad, Tor Martin

    2013-01-01

    Gjemnessundbrua og Bergsøysundbrua på Europavei E39 i Møre og Romsdal, skal instrumenteres for identifikasjon av strukturelle og dynamiske egenskaper. Denne rapporten omhandler dokumentasjon og beskrivelse av det utførte arbeidet ved monteringen av måleutstyret. Det er også gjort analyser av de ulike konstruksjonsdelene akselerometersensorer og anemometre er montert på. Til slutt er de initielle responssignalene fra systemet behandlet, der egenfrekvenser og ...

  18. Dicty_cDB: FC-AV04 [Dicty_cDB

    Lifescience Database Archive (English)

    Full Text Available FC (Link to library) FC-AV04 (Link to dictyBase) - - - Contig-U15991-1 FC-AV04P (Li...nk to Original site) FC-AV04F 307 FC-AV04Z 363 FC-AV04P 670 - - Show FC-AV04 Library FC (Link to library) Clone ID FC-AV...al site URL http://dictycdb.biol.tsukuba.ac.jp/CSM/FC/FC-AV/FC-AV04Q.Seq.d/ Representative seq. ID FC-AV...04P (Link to Original site) Representative DNA sequence >FC-AV04 (FC-AV04Q) /CSM/FC/FC-AV/FC-AV...B: ifnftskekkk*nnsfmfsvvtlffnfilfyffifnyffnyffisfffpiqiiiifnyfl fylffls*ipk*lki*av*dyfsnf*l**c*cslrnrkit**--

  19. REMINDER Preparation for Retirement Programme - AVS Seminar

    CERN Multimedia

    Social Service

    2001-01-01

    The AVS seminar, which had to be cancelled in March this year, will be held on: Wednesday 26 September from 14:00 to 16:00 in the Main Amphitheatre The session will be led by Mr. Lorenz STAMPFLI. The session is open to all people already registered and any other person who is interested, without the need to register.

  20. REMINDER PREPARATION FOR RETIREMENT PROGRAMME - AVS SEMINAR

    CERN Multimedia

    Social Service

    2001-01-01

    The AVS seminar, which had to be cancelled in March this year, will be held on: Wednesday 26 September from 14:00 to 16:00 in the Main Amphitheatre The session will be led by Mr. Lorenz STAMPFLI. The session is open to all people already registered and any other person who is interested, without the need to register.

  1. Acid volatile sulfide (AVS)- a comment

    NARCIS (Netherlands)

    Meysman, F.J.R.; Middelburg, J.J.

    2005-01-01

    The review by Rickard and Morse (this volume) adequately summarizes our current understanding with respect to acid-volatile sulfides (AVS). At the same time, this review addresses some of the misunderstandings with regard to measurements and dynamics of this important sedimentary sulfur pool. In

  2. Risikostyring av jetfuelprisen med futureskontrakter : en studie av hedgingstrategier for flyselskaper

    OpenAIRE

    Marker, Sebastian

    2013-01-01

    Denne oppgaven undersøker risikostyring av jetfuelprisen gjennom hedgingstrategier med WTI- og fyringsoljefutures som sikringsinstrument. Analysen tar sikte på å estimere minimum varians hedgingrater for ulike hedgingstrategier. Formålet er å finne hedgingstrategier som gir høyest hedgingeffektivitet, og dermed gir størst reduksjon i jetfuelprisrisiko. Første del av analysen vil estimere hedgingrater med utgangspunkt i flere analysemetoder under en klassisk lineær regresjonsmodell (CLRM). Hed...

  3. Aktivitetsbasert pedagogikk; en studie av bachelorstudenters utvikling av handlingskompetanse gjennom aktivitetsbasert pedagogikk

    Directory of Open Access Journals (Sweden)

    Kari Margrete Hjelle

    2012-06-01

    Full Text Available Målet med artikkelen er å synliggjøre hvordan bachelorstudenter i ergoterapi kan utvikle profesjonell handlingskompetanse gjennom aktivitetsbasert pedagogikk. På bakgrunn av dette gjennomførte vi kvalitative fokusgruppeintervju med 12 ergoterapeutstudenter som hadde deltatt i undervisningsopplegget “Aktivitetsgrupper-lede og delta”. Datamaterialet ble analysert etter retningslinjer for fenomenologisk basert meningskondensering. Studentene diskuterte sine erfaringer og det kom tydelig fram at undervisningsopplegget ga dem muligheter til å bruke sitt engasjement og sin kreativitet i valg av aktiviteter for sin simulerte aktivitetsgruppe. Videre fikk de muligheter til å oppdage sider ved anvendelse av aktiviteter for ulike pasienter, som de ikke kunne forstå uten integrering av teori i praktisk ferdighetstrening. Ved planlegging, gjennomføring og refleksjon over å lede og delta i aktivitetsgrupper, oppøvet de evnen til handlingsrefleksjon. Aktivitetsbasert pedagogikk kan bidra til utvikling av profesjonell handlingskompetanse og det er behov for både teoretiske og praktiske læringsformer. Funnene ble diskutert i lys av pedagogisk handlingsteori.

  4. Low-Complexity Tools in AVS Part 7

    Institute of Scientific and Technical Information of China (English)

    Feng Yi; Qi-Chao Sun; Jie Dong; Lu Yu

    2006-01-01

    Audio Video coding Standard (AVS) is established by the AVS Working Group of China. The main goal of AVS part 7 is to provide high compression performance with relatively low complexity for mobility applications. There are 3 main low-complexity tools: deblocking filter, context-based adaptive 2D-VLC and direct intra prediction. These tools are presented and analyzed respectively. Finally, we compare the performance and the decoding speed of AVS part 7 and H.264 baseline profile. The analysis and results indicate that AVS part 7 achieves similar performance with lower cost.

  5. Overview of AVS-video: tools, performance and complexity

    Science.gov (United States)

    Yu, Lu; Yi, Feng; Dong, Jie; Zhang, Cixun

    2005-07-01

    Audio Video coding Standard (AVS) is established by the Working Group of China in the same name. AVS-video is an application driven coding standard. AVS Part 2 targets to high-definition digital video broadcasting and high-density storage media and AVS Part 7 targets to low complexity, low picture resolution mobility applications. Integer transform, intra and inter-picture prediction, in-loop deblocking filter and context-based two dimensional variable length coding are the major compression tools in AVS-video, which are well-tuned for target applications. It achieves similar performance to H.264/AVC with lower cost.

  6. Varmepumper i hushald. Analyse av endringar i effektforbruket hos hushald med panelomnar og vedfyring ved installasjon av ulike typar varmepumper

    OpenAIRE

    Øvrebotten, Kristian

    2015-01-01

    Sidan oljekrisa på 1970-talet som førte til ein gradvis overgang frå bruk av oljekjelar og parafinkaminar til panelomnar og varmepumper, har andelen elektrisitet stadig auka mens andelen av olje har vore redusert. Andelen av elektrisitet hos hushalda utgjer i dag om lag 79 % av den samla energibruken hos hushalda i Noreg. Dette høge forbruket av elektrisitet hos hushalda bidreg til å skape problem med høge effekttoppar i kraftnettet som igjen skapar problem for kraftdistributørar og er med...

  7. A Novel MBAFF Scheme of AVS

    Institute of Scientific and Technical Information of China (English)

    Jian-Wen Chen; Guo-Ping Li; Yun He

    2006-01-01

    Adaptive frame/field coding techniques have been adopted in many international video standards for interlaced sequence coding. When the frame/field adaptation is applied on the picture level, the coding efficiency is improved greatly,compared with the pure frame coding or the pure field coding. The picture-level adaptive frame/field coding (PAFF) selects frame coding or field coding once for one picture. If this frame/field adaptation is extended to Macro Block (MB) level, the coding efficiency will be further increased. In this paper, a novel MB-level adaptive frame/field (MBAFF) coding scheme is proposed. In the proposed MBAFF scheme, the top field of the current picture is used as a reference. The experiments are implemented on the platforms of Audio Video coding Standard (AVS) base profile and H.264/AVC, respectively. On the AVS platform, 0.35dB gain can be achieved averagely, compared with AVS1.0 anchor. On the H.264/AVC platform, 0.16dB gain can be achieved averagely, compared with MBAFF scheme of H.264/AVC. Additionally, an extensive subjective quality enhancement can be achieved by the proposed scheme.

  8. Utveckling av ett webbforum med innehållshanteringssystemet Drupal

    OpenAIRE

    Lindqvist, Christoffer

    2010-01-01

    Detta examensarbete består av två huvuddelar. Den första delen är en teoretisk analys av innehållshanteringssystem i allmänhet och en forskning i hur det populära innehållshanteringsverktyget Drupal är uppbyggt. Den andra delen av arbetet är en praktisk genomgång av hur funktionalitet för ett webbforum kan byggas upp med hjälp av Drupals ramverk. Den teoretiska analysen undersöker vad ett innehållshanteringsverktyg är, vad det används till och vilka huvuddrag det i allmänhet består av. Sed...

  9. Teknologisk fravalg og tilvalg av fremtidig syke og funksjonshemmede

    Directory of Open Access Journals (Sweden)

    Berge Solberg

    2008-05-01

    Full Text Available I dette nummeret av Etikk i praksis ønsker vi å fokusere på et betent problemområde, nemlig etikken rundt teknologisk fravalg og tilvalg av fremtidige syke og funksjonshemmede barn. Det handler om abort, medisinsk teknologi og valg av barn. Det kan synes som om vi står overfor nye muligheter for fravalg og tilvalg som ikke bare setter en ny debatt, men som samtidig krever en tilbakevending og nyåpning av tidligere gjennomdiskuterte spørsmål og tema. Debattene rundt «valg av barn» berører dype, eksistensielle dimensjoner ved menneskelivet. I tillegg berører det store samfunnsmessige temaer som inklusjon og menneskeverd, selvbestemmelse, abort, eugenikk og samfunnets forståelse av funksjonshemming.http://dx.doi.org/10.5324/eip.v2i1.1686

  10. Kvinnors upplevelser av komplicerad förlossning

    OpenAIRE

    Samuelsson, Magdalena; Wahlberg, Christina

    2006-01-01

    Att vänta och föda barn beskrivs som en av de viktiga övergångsperioderna i livet och innebär ofta en personlig utveckling för den födande kvinnan. Då graviditeten och förlossningen av någon anledning kompliceras, kan denna process störas, och kvinnan kan istället få både fysiska och psykiska men i efterförloppet. Syftet med denna studie var att belysa kvinnors upplevelser av en komplicerad förlossning. Metoden var en granskning i form av en innehållsanalys av sju artiklar som utgjordes av fy...

  11. AVS 3D Video Coding Technology and System

    Institute of Scientific and Technical Information of China (English)

    Siwei Ma; Shiqi Wang; Wen Gao

    2012-01-01

    Following the success of the audio video standard (AVS) for 2D video coding, in 2008, the China AVS workgroup started developing 3D video (3DV) coding techniques. In this paper, we discuss the background, technical features, and applications of AVS 3DV coding technology. We introduce two core techniques used in AVS 3DV coding: inter-view prediction and enhanced stereo packing coding. We elaborate on these techniques, which are used in the AVS real-time 3DV encoder. An application of the AVS 3DV coding system is presented to show the great practical value of this system. Simulation results show that the advanced techniques used in AVS 3DV coding provide remarkable coding gain compared with techniques used in a simulcast scheme.

  12. Analyse av klimatilpassningstiltak : en casestudie av avløpsnettet i Veumdalen, Fredrikstad kommune

    OpenAIRE

    Myking, Jakob Severin Eide

    2012-01-01

    Økt forståelse av fortidens klimaendringer og klimasystemet i sin helhet, har gitt forskerne mulighet til å anta fremtidens klimaendringer. Denne økte forståelsen har gitt sikre tegn på at klimaendringene er knyttet til en stor grad av usikkerhet. Det eneste som er sikkert, er at klimaendringer kommer til å skje, og at det er umulig å si med sikkerhet når eller med hvilken kraft de vil inntreffe. Det som derimot kan sies med sikkerhet, er at befolkningen kommer til å øke. Dette vil føre til ø...

  13. Optimering av FISH- teknik för detektion av Laktobaciller

    OpenAIRE

    Hamidi, Helaleh

    2008-01-01

    Sammanfattning Syftet med den här studien var att utveckla och optimera FISH (Fluorescense In Situ Hybridisation) tekniken som en snabb och ganska billig metod för detektion av laktobaciller. Det vill säga att kunna på objektsglas använda FISH tekniken för att identifiera laktobaciller på artnivå med fluorescensmärkta prober mot 16S och 23S RNA. FISH är en allmän och användbar metod för att detektera och lokalisera mikroorganismer eller en specifik grupp av mikroorganismer i provet (1). Metod...

  14. Kunnskapsutvikling i et profesjonsperspektiv - En kvantitativ studie av sosialarbeideres bruk av kunnskapsressurser i arbeidslivet

    OpenAIRE

    2014-01-01

    Problemområde Profesjoner betraktes gjerne som grupper som rår over store deler av samfunnets ressurser. Som velferdsstatens portvakter anses de gjerne som grupper med definisjonsmakt og forvaltere av kunnskap (Molander og Smeby, 2013). Det moderne kunnskapssamfunnet byr imidlertid på nye utfordringer for profesjonsfeltet. Det stilles i økende grad krav til at profesjonsutøvere skal være faglig oppdaterte, utøve en kunnskapsbasert praksis og til en hver tid inneha nødvendig kompetanse. Sosial...

  15. Functional mathematical model of dual pathway AV nodal conduction.

    Science.gov (United States)

    Climent, A M; Guillem, M S; Zhang, Y; Millet, J; Mazgalev, T N

    2011-04-01

    Dual atrioventricular (AV) nodal pathway physiology is described as two different wave fronts that propagate from the atria to the His bundle: one with a longer effective refractory period [fast pathway (FP)] and a second with a shorter effective refractory period [slow pathway (SP)]. By using His electrogram alternance, we have developed a mathematical model of AV conduction that incorporates dual AV nodal pathway physiology. Experiments were performed on five rabbit atrial-AV nodal preparations to develop and test the presented model. His electrogram alternances from the inferior margin of the His bundle were used to identify fast and slow wave front propagations. The ability to predict AV conduction time and the interaction between FP and SP wave fronts have been analyzed during regular and irregular atrial rhythms (e.g., atrial fibrillation). In addition, the role of dual AV nodal pathway wave fronts in the generation of Wenckebach periodicities has been illustrated. Finally, AV node ablative modifications have been evaluated. The model accurately reproduced interactions between FP and SP during regular and irregular atrial pacing protocols. In all experiments, specificity and sensitivity higher than 85% were obtained in the prediction of the pathway responsible for conduction. It has been shown that, during atrial fibrillation, the SP ablation significantly increased the mean HH interval (204 ± 39 vs. 274 ± 50 ms, P AV node mechanisms and should be considered as a step forward in the studies of AV nodal conduction.

  16. AV nodal dual pathway electrophysiology and Wenckebach periodicity.

    Science.gov (United States)

    Zhang, Youhua; Mazgalev, Todor N

    2011-11-01

    The precise mechanism(s) governing the phenomenon of AV nodal Wenckebach periodicity is not fully elucidated. Currently 2 hypotheses, the decremental conduction and the Rosenbluethian step-delay, are most frequently used. We have provided new evidence that, in addition, dual pathway (DPW) electrophysiology is directly involved in the manifestation of AV nodal Wenckebach phenomenon. AV nodal cellular action potentials (APs) were recorded from 6 rabbit AV node preparations during standard A1A2 and incremental pacing protocols. His electrogram alternans, a validated index of DPW electrophysiology, was used to monitor fast (FP) and slow (SP) pathway conduction. The data were collected in intact AV nodes, as well as after SP ablation. In all studied hearts the Wenckebach cycle started with FP propagation, followed by transition to SP until its ultimate block. During this process complex cellular APs were observed, with decremental foot formations reflecting the fading FP and second depolarizations produced by the SP. In addition, the AV node cells exhibited a progressive loss in maximal diastolic membrane potential (MDP) due to incomplete repolarization. The pause created with the blocked Wenckebach beat was associated with restoration of MDP and reinitiation of the conduction cycle via the FP wavefront. DPW electrophysiology is dynamically involved in the development of AV nodal Wenckebach periodicity. In the intact AV node, the cycle starts with FP that is progressively weakened and then replaced by SP propagation, until block occurs. AV nodal SP modification did not eliminate Wenckebach periodicity but strongly affected its paradigm. © 2011 Wiley Periodicals, Inc.

  17. Legemiddelbivirkninger – forekomst og vurdering av årsakssammenhenger.

    Directory of Open Access Journals (Sweden)

    Tone Westergren

    2009-09-01

    Full Text Available En gjennomgang av spontanrapportene fra den norske bivirkningsdatabasen viser en betydelig geografisk variasjon når det gjelder innrapportering av bivirkninger. I Norge blir disse meldingene evaluert ved RELIS (Regionale legemiddelinformasjonssentre av tverrfaglige team bestående av leger og farmasøyter. Bivirkningsarbeidet ved RELIS gjøres på oppdrag fra Statens legemiddelverk, som har det overordnede ansvar for legemiddelovervåkningen i Norge. Vurderingene baseres på kjent risikoprofil for mistenkte legemidler, indikasjon, dosering, tidsmessig sammenheng mellom reaksjon og medikamentinntak, effekt av seponering og eventuelle andre risikofaktorer. 3345 legemiddel/bivirkningskombinasjoner ble vurdert på bakgrunn av 1408 rapporter i 2006. De fleste mistenkte bivirkningene ble klassifisert som mulige, ettersom andre faktorer også kunne ha spilt en rolle. Få bivirkninger ble klassifisert som sikre. Informasjon om bivirkninger i litteratur og databaser er i flere tilfeller preget av manglende homogenitet, tilfeldige sammentreff i tid, manglende data og lite vurdering av sammenhengen mellom reaksjonen og medisinbruken. Kliniske studier har begrenset mulighet til å påvise annet enn de vanligste bivirkningene. Det kan ta år etter markedsføring å fastslå sammenhengen med mer sjeldne bivirkninger. Frekvenstabeller for bivirkninger er anslag basert på et begrenset antall pasienter, spesielt for nye legemidler. Evaluering av bivirkningsrapporter fra helsepersonell kan avdekke mangel på lett tilgjengelig informasjon og bidra til ny kunnskap om et legemiddels bivirkningsprofil

  18. Russisk oljetransport gjennom Barentshavet : En tilfellestudie av den norske responsen

    OpenAIRE

    Aasen, Kari

    2010-01-01

    Oppgaven er en tilfellestudie av den norske responsen på økningen av russisk oljetransport gjennom Barentshavet. Oljetransporten fra Nordvest-Russland tok seg plutselig dramatisk opp i 2002, noe som kom svært overraskende på norske myndigheter. Hensikten med oppgaven har derfor vært å kartlegge den norske responsen, for å finne ut hva som har preget den. På bakgrunn av kjennetegnene ved responsen har jeg deretter forsøkt å sannsynliggjøre hvilke oppfatninger av Russland som vært dominerende. ...

  19. Konsum av risiko-matvarer - Beskrivelse av en undersøkelse som skal kartlegge konsum av matvarer med betydning for inntaket av miljøgifter

    Directory of Open Access Journals (Sweden)

    Helle Margrete Meltzer

    2009-11-01

    Full Text Available  SAMMENDRAGI perioden 1999-2001 vil det bli gjennomført tre kostholdsundersøkelser som til sammen skal kartlegge konsum avmatvarer med betydning for inntaket av miljøgifter i Norge, fortrinnsvis kadmium, kvikksølv, PCB og dioksiner.Disse giftene finnes hovedsakelig i krabbe, flatfisk, skjell, gjedde og abbor, lever eller nyre fra vilt og viltvoksendesopp. Fordi gjennomsnittskonsumet er lavt, spørres det ikke spesifikt om disse matvarene i landsdekkende kostholdsundersøkelsersom 'Norkost'. Hensikten med studien er å kunne gi en bedre beskrivelse av eksponeringsfordelingeni befolkningen med tanke på miljøgifter. Fordelingen er antakelig skjev, dvs. et stort antall personerventes å ha et lavt inntak og noen få personer ventes å ha et relativt høyt inntak av de undersøkte stoffene. Eventuelleukjente risikogrupper vil kunne avdekkes, og det er av stor interesse å undersøke hvor stor eksponeringen i deutsatte gruppene er.Del A av undersøkelsen er landsdekkende og omfatter en postal frekvensundersøkelse til 10 000 tilfeldig valgtepersoner mellom 18 og 79 år. Del B omfatter en postal frekvensundersøkelse til 6000 tilfeldig valgte personer i sekskommuner, der tre er kystkommuner og tre er innlandskommuner. Kommunene blir valgt ut fra kjennskap til godtilgang på de aktuelle matvarene. Vi antar at der tilgangen på matvarene er god, er konsumet høyere. Del C avundersøkelsen vil omfatte høykonsumenter av aktuelle matvarer, valgt fra del B av undersøkelsen. Dette vil være endybdestudie der analyser av miljøgifter i blod, hår og urin også skal inngå.Undersøkelsen er den første i sitt slag her til lands og vi kjenner ikke til at tilsvarende studier er gjort i andreland. Hensikten med artikkelen er å gi en beskrivelse av undersøkelsen i en tidlig fase av gjennomføringen.Meltzer HM, Bergsten C, Stene LC, Stigum H, Wiborg ML, Lund-Larsen K, Alexander J. Consumption ofcontaminated foods – Description of a dietary survey

  20. Dicty_cDB: FC-AV01 [Dicty_cDB

    Lifescience Database Archive (English)

    Full Text Available FC (Link to library) FC-AV01 (Link to dictyBase) - - - Contig-U16311-1 FC-AV01Z (Li...nk to Original site) - - FC-AV01Z 643 - - - - Show FC-AV01 Library FC (Link to library) Clone ID FC-AV01 (Li.../dictycdb.biol.tsukuba.ac.jp/CSM/FC/FC-AV/FC-AV01Q.Seq.d/ Representative seq. ID FC-AV...01Z (Link to Original site) Representative DNA sequence >FC-AV01 (FC-AV01Q) /CSM/FC/FC-AV/FC-AV01Q.Seq....ed Amino Acid sequence ---SGSHGGSQSQSAGSDSQSAGSESSQSESGSQSQSESGSQSQSQSGSQSFSGSLYSGS YSGSQSGSQSGNSGAAVKQTGAGS

  1. Pollen transmission of asparagus virus 2 (AV-2) may facilitate mixed infection by two AV-2 isolates in asparagus plants.

    Science.gov (United States)

    Kawamura, Ryusuke; Shimura, Hanako; Mochizuki, Tomofumi; Ohki, Satoshi T; Masuta, Chikara

    2014-09-01

    Asparagus virus 2 (AV-2) is a member of the genus Ilarvirus and thought to induce the asparagus decline syndrome. AV-2 is known to be transmitted by seed, and the possibility of pollen transmission was proposed 25 years ago but not verified. In AV-2 sequence analyses, we have unexpectedly found mixed infection by two distinct AV-2 isolates in two asparagus plants. Because mixed infections by two related viruses are normally prevented by cross protection, we suspected that pollen transmission of AV-2 is involved in mixed infection. Immunohistochemical analyses and in situ hybridization using AV-2-infected tobacco plants revealed that AV-2 was localized in the meristem and associated with pollen grains. To experimentally produce a mixed infection via pollen transmission, two Nicotiana benthamiana plants that were infected with each of two AV-2 isolates were crossed. Derived cleaved-amplified polymorphic sequence analysis identified each AV-2 isolate in the progeny seedlings, suggesting that pollen transmission could indeed result in a mixed infection, at least in N. benthamiana.

  2. EST Table: AV399953 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399953 br--0128 11/12/09 GO hit GO:0016491(oxidoreductase activity)|GO:0055114(ox...idation reduction) 10/09/28 43 %/114 aa ref|XP_397141.3| PREDICTED: similar to NADPH dependent diflavin oxid...imilar to NADPH-dependent FMN and FAD containing oxidoreductase-like protein [Tribolium castaneum] AV399953 br-- ...

  3. EST Table: AV404903 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404903 prgv0575 11/12/09 GO hit GO:0004174(electron-transferring-flavoprotein deh...w homology 10/09/10 low homology 10/09/10 low homology 10/09/10 low homology AV404903 prgv ...

  4. Bochdalek Hernia As A Cause Of Intermittent AV Block

    OpenAIRE

    Etsadashvili, Kakhaber; Rashid, Haroon Mohammed; Jalabadze, Khatuna; Melia, Anzor

    2011-01-01

    Congenital diaphragmatic hernia is very rare cause of AV block. We report such a patient with sick sinus node syndrome and previous AAIR pacemaker implantation, in which intermittent AV block was diagnosed by 24-hours ECG monitoring and upgrade of pacing system to DDDR was suggested.

  5. Introduction to AVS2 Scene Video Coding Techniques

    Institute of Scientific and Technical Information of China (English)

    Jiaying Yan; Siwei Dong; Yonghong Tian; Tiejun Huang

    2016-01-01

    The second generation Audio Video Coding Standard (AVS2) is the most recent video coding standard. By introducing several new coding techniques, AVS2 can provide more efficient compression for scene videos such as surveillance videos, conference videos, etc. Due to the limited scenes, scene videos have great redundancy especially in background region. The new scene video coding techniques applied in AVS2 mainly focus on reducing redundancy in order to achieve higher compression. This paper introduces several important AVS2 scene video coding techniques. Experimental results show that with scene video coding tools, AVS2 can save nearly 40%BD⁃rate (Bjøntegaard⁃Delta bit⁃rate) on scene videos.

  6. Implementeringsforskning: vitenskap for forbedring av praksis

    Directory of Open Access Journals (Sweden)

    Signe Flottorp

    2013-11-01

    Full Text Available Medisinsk forskning har ført til store framskritt de siste tiårene. Det er investert mye mer ressurser på basalforskning og klinisk forskning enn på å utvikle og evaluere metoder for å sikre at pasientene får nytte av forskningen. Formålet med implementeringsforskning er å redusere gapet mellom forskning og praksis, ved å utvikle og evaluere tiltak som kan sikre at behandlingen som pasientene mottar er kunnskapsbasert, at den er omsorgsfull og av god kvalitet.I denne artikkelen gjør vi rede for hva implementering og implementeringsforskning er. Vi belyser historikken til denne unge vitenskapen, og illustrerer mangfoldet i de faglige tilnærmingene og begrepene som brukes om det å få forskning brukt i praksis. Det finnes en rekke teorier om endring av atferd, både på individnivå og på organisatorisk nivå. Teoriene er imidlertid i liten grad testet empirisk, særlig når det gjelder å endre atferd i helsetjenesten.Systematiske oversikter over metodisk gode studier er den beste kilden til informasjon om effekt av implementeringstiltak. The Cochrane Effective Practice and Organisation of Care Group (EPOC er en viktig kilde for slike oversikter. De systematiske oversiktene som er utarbeidet på dette feltet viser at passive dissemineringstiltak har begrenset effekt, mens mer aktive tiltak kan ha liten til moderat effekt. Det er ofte betydelig variasjon i effekt på tvers av studiene. Det er derfor viktig å få bedre kunnskap om hvilke faktorer som kan forklare slike forskjeller i effekt.Vi gir eksempler på norske implementeringsstudier, og refererer bidrag fra forskere ved Kunnskapssenteret. Implementeringsforskningen kan, hvis den lykkes, sikre pasientene bedre behandling.Flottorp S, Aakhus E. Implementation research: science for improving practice. Nor J Epidemiol 201 3; 23 (2: 187-196.ENGLISH SUMMARY Medical research has led to major advances in recent decades. More resources have been invested in basic and clinical research

  7. Cofactor requirement of HpyAV restriction endonuclease.

    Directory of Open Access Journals (Sweden)

    Siu-Hong Chan

    Full Text Available BACKGROUND: Helicobacter pylori is the etiologic agent of common gastritis and a risk factor for gastric cancer. It is also one of the richest sources of Type II restriction-modification (R-M systems in microorganisms. PRINCIPAL FINDINGS: We have cloned, expressed and purified a new restriction endonuclease HpyAV from H. pylori strain 26695. We determined the HpyAV DNA recognition sequence and cleavage site as CCTTC 6/5. In addition, we found that HpyAV has a unique metal ion requirement: its cleavage activity is higher with transition metal ions than in Mg(++. The special metal ion requirement of HpyAV can be attributed to the presence of a HNH catalytic site similar to ColE9 nuclease instead of the canonical PD-X-D/EXK catalytic site found in many other REases. Site-directed mutagenesis was carried out to verify the catalytic residues of HpyAV. Mutation of the conserved metal-binding Asn311 and His320 to alanine eliminated cleavage activity. HpyAV variant H295A displayed approximately 1% of wt activity. CONCLUSIONS/SIGNIFICANCE: Some HNH-type endonucleases have unique metal ion cofactor requirement for optimal activities. Homology modeling and site-directed mutagenesis confirmed that HpyAV is a member of the HNH nuclease family. The identification of catalytic residues in HpyAV paved the way for further engineering of the metal binding site. A survey of sequenced microbial genomes uncovered 10 putative R-M systems that show high sequence similarity to the HpyAV system, suggesting lateral transfer of a prototypic HpyAV-like R-M system among these microorganisms.

  8. Bioseguridad en granjas avícolas

    Directory of Open Access Journals (Sweden)

    Sandra L. Ricaurte Galindo

    2005-02-01

    Full Text Available La bioseguridad es el conjunto de prácticas de manejo diseñadas para prevenir la entrada y transmisión de agentes patógenos que puedan afectar la sanidad en las granjas avícolas. La bioseguridad es una parte fundamental de cualquier empresa avícola ya que proporciona un aumento de la productividad de la parvada y un aumento en el rendimiento económico. En líneas generales, se debe contemplar la localización de la granja, características constructivas de los galpones, control de parvadas extraños a la granja, limpieza y desinfección de los galpones, control de visitas, evitar el stress en las aves encasetadas, evitar la contaminación del pienso, control de vacunaciones y medicaciones y control de deyecciones, cadáveres, etc.The biosecurity is the group of practical of handling designed to prevent the entrance and agents' pathologies transmission that can affect the sanity in the poultry farms. The biosecurity is since a fundamental part of any poultry company it provides an increase of the productivity of the chickens and hens and an increase in the economic yield. In general lines, the localization of the farm should be contemplated, characteristic constructive of the ship, control of strange chickens and hens to the farm, cleaning and disinfection of the ship, control of visits, to avoid the stress in the birds housed, to avoid the contamination of the I think, control of vaccinations and medications and dejections control, cadavers, etc.

  9. Coexistence of mixed phenotype Creutzfeldt-Jakob disease, Lewy body disease and argyrophilic grain disease plus histological features of possible Alzheimer's disease: a multi-protein disorder in an autopsy case.

    Science.gov (United States)

    Fernández-Vega, Iván; Ruiz-Ojeda, Javier; Juste, Ramon A; Geijo, Maria; Zarranz, Juan Jose; Sánchez Menoyo, Jose Luis; Vicente-Etxenausia, Ikerne; Mediavilla-García, Jennifer; Guerra-Merino, Isabel

    2015-02-01

    We report hereby an autopsy case of sporadic mixed phenotype CJD without hereditary burden and a long-term clinical course. An 80-year old man was diagnosed with mild cognitive impairment 27 months before death, caused by bronchopneumonia and severe respiratory impairment. During this time, the patient developed gradual mental deterioration, some sleeping problems and myoclonus. Other clinical manifestations were progressive gait problems, language deterioration, presence of primitive reflexes and irritability. In keeping with those symptoms, a rapidly evolving dementia was clinically suspected. Cerebrospinal fluid test for 14-3-3 protein was negative. However, an abnormal EEG and MRI at end-stage of disease were finally consistent with CJD. Post-mortem examination revealed a massive cortical neuronal loss with associated reactive astrocytosis, also evident in the white matter. Diffuse spongiform changes involving some basal ganglia, especially medial thalamus, some troncoencephalic nuclei, mainly inferior olivary nucleus and the molecular layer of the cerebellum were seen. Immunorreactive deposits for anti-prion protein antibody were present at different areas of the CNS. Additionally, Lewy bodies were observed at the brainstem and amygdala. Furthermore, argirophilic grains together with oligodendroglial coiled bodies and pre-tangle inclusions in the neurons from the limbic system containing hyperphosphorylated 4R tau were noted. To the best of our knowledge, this is the first case of CJD combined with Lewy body disease and argirophilic grain disease. Furthermore, we believe this case is an extremely rare combination of MM2-cortical-type and MM2-thalamic-type sporadic CJD (sCJD), which explains the broad spectrum of MM2-type sCJD findings and symptoms. Moreover, histological features of possible Alzheimer's disease were also reported.

  10. Investigation of prion protein gene in 10 sporadic Creutzfeldt-Jakob disease patients: a new novel mutation of prion protein gene%散发性Creutzfeldt-Jakob病患者10例prion基因研究

    Institute of Scientific and Technical Information of China (English)

    南善姬; 赵节绪; 林世和; 江新梅; 宋晓南

    2006-01-01

    目的 检测10例Creutzfeldt-Jakob病(CJD)患者prion基因(PRNP)外显子突变情况.方法 抽取患者外周静脉血,提取DNA,PCR法扩增PRNP外显子后直接测序,并用限制性内切酶Nsp Ⅰ检测PRNP 129位点密码子基因型.结果 2例肯定CJD患者中,1例PRNP检测未见异常,另1例PRNP第729碱基G被C取代(729G→C),使编码prion第211个氨基酸的密码子GAG变成了GAC,翻译后第211个氨基酸由谷氨酸变为天冬氨酸(E211D).8例很可能CJD患者中,2例PRNP第751碱基G被A取代(751G→A),使编码prion第219个氨基酸的密码子GAG变成了AAG,翻译后第219个氨基酸由谷氨酸变为赖氨酸(E219K).10例CJD患者PRNP 129位点密码子基因型都是甲硫氨酸纯合型.结论 1例肯定CJD患者的prion基因外显子存在一种新的点突变E211D,这很可能是导致遗传prion病发生的原因.2例很可能CJD患者的prion基因突变E219K,与M129V同属于基因多态性,而不是致病原因.prion基因检测有助于prion病的诊断.

  11. Phase 4 paroxysmal AV block in a patient with scleroderma.

    Science.gov (United States)

    Butschek, Ross; Powell, Brian D; Littmann, Laszlo

    2013-01-01

    A 72-year-old man with limited cutaneous systemic scleroderma was hospitalized for two episodes of witnessed syncope. The baseline 12-lead electrocardiogram was normal but on telemetry there were numerous episodes of paroxysmal AV block with asystolic periods of up to 7.5 s duration. Analysis of the rhythm strips revealed phase 4 intra-His bundle block characterized by critical P-P intervals that triggered the AV block, and a narrow range of junctional escape to subsequent P wave intervals that were required to release the AV block. A dual chamber pacemaker was implanted. Copyright © 2013 Elsevier Inc. All rights reserved.

  12. Ishockeykjelke - Understell: Utvikling av ny kjelke til det norske kjelkehockeylandslaget

    OpenAIRE

    2013-01-01

    Kjelkehockey er basert på de samme prinsippene som ishockey, men er rettet mot utøvere med funksjonsnedsettelser i nedre del av kroppen. Utøverne sitter i en tilpasset kjelke med skøytestål på undersiden av bakre del av kjelken. De har en tilpasset hockeykølle i hver hånd, som har to funksjoner, i den ene enden er det pigger til å stake seg frem med på isen, og den andre enden ligner en vanlig hockeykølle og brukes til å håndter...

  13. Highly Sensitive, Quantitative Cell-Based Assay for Prions Adsorbed to Solid Surfaces

    National Research Council Canada - National Science Library

    Julie Ann Edgeworth; Graham S. Jackson; Anthony R. Clarke; Charles Weissmann; John Collinge

    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...

  14. "Oktober i lederstolen" og "Harlekins Alle hjerters dag" : to noveller av Neil Gaiman : oversatt og kommentert

    OpenAIRE

    Eilertsen, Helle Elisabet Jæger

    2011-01-01

    Oppgaven består av oversettelser av to noveller skrevet av Neil Gaiman, og en kommentardel. Novellene er innenfor sjangeren fantastisk litteratur. Kommentardelen tar for seg relevante problemstillinger, blant annet oversettelse av navn, særegne uttrykk og dobbeltbetydning. Den tar også for seg hjemliggjøring/fremmedgjøring og valg av stil. Dette er knyttet til oversettelsesteoretikere som Schleiermacher, Nida, Koller og Berman.

  15. Liv og lerret Fire portretter og et "kryptoportrett" av Gustav KLimt

    OpenAIRE

    Gamborg, Siri Angela

    2009-01-01

    Studie av fire portretter og et "kryptoportrett" av Gustav Klimt. Analysen fokuserer på Klimts avbildning av kvinner og menn på forskjellig måte og ved hjelp av ulike stilistiske virkemidler. Kjønnsaspektet i studien er belyst utfra samtidskonteksten og kvinnenes spesielle situasjon i Wien rundt århundreskiftet 1800/1900 og Klimts fremstilling av disse elementene i sine portretter.

  16. Open Source AV solution supporting In Situ Simulation

    DEFF Research Database (Denmark)

    Krogh, Kristian; Pociunas, Gintas; Dahl, Mads Ronald

    the software to meet our expectations for a portable AV system for VAD. The system would make use of “off the shelf” hardware components which are widely available and easily replaced or expanded. The developed AV software and coding is contracted to be available as Copyleft Open Source to ensure low cost...... a stable AV software that has be developed and implemented for an in situ simulation initiative. This version (1.3) is the first on released as Open Source (Copyleft) software (see QR tag). We have found that it is possible to deliver multi-camera video assisted debriefing in a mobile, in situ simulation...... environment using an AV system constructed from “off the shelf” components and Open Source software....

  17. EST Table: AV403854 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV403854 pg--0133 10/09/28 98 %/123 aa ref|ZP_03045664.1| tail length tape measure ...protein [Escherichia coli E22] gb|EDV82450.1| tail length tape measure protein [Escherichia coli E22] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV404025 pg-- ...

  18. EST Table: AV399431 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399431 NV120218 10/09/28 91 %/131 aa ref|NP_047488.1| CG30 [Bombyx mori NPV] gb|A...AC63757.1| CG30 [Bombyx mori NPV] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV399298 NV12 ...

  19. EST Table: AV405409 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405409 wdV10576 11/12/09 n.h 10/09/28 33 %/246 aa ref|XP_001866937.1| scavenger r...ef|XP_967476.1| PREDICTED: similar to scavenger receptor cysteine-rich protein isoform 1 [Tribolium castaneum] AV405409 wdV1 ... ...eceptor cysteine-rich protein [Culex quinquefasciatus] gb|EDS44206.1| scavenger receptor cysteine-rich prote

  20. EST Table: AV404175 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404175 pg--0560 10/09/28 100 %/209 aa ref|NP_040587.1| capsid component [Enteroba...EFF06860.1| phage major capsid protein E [Escherichia coli B185] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV404434 pg-- ...

  1. EST Table: AV399451 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399451 NV120246 10/09/28 94 %/223 aa ref|NP_047521.1| AcMNPV orf124 [Bombyx mori ...NPV] gb|AAC63790.1| AcMNPV orf124 [Bombyx mori NPV] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV399564 NV12 ...

  2. EST Table: AV398238 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398238 NV021277 11/12/09 GO hit GO:0004871(signal transducer activity)|GO:0005515...zinc ion binding) 10/09/28 91 %/106 aa ref|XP_002007246.1| GI12833 [Drosophila mojavensis] gb|EDW17722.1| GI12833 [Drosophila mojav...1.1| hypothetical protein TcasGA2_TC003318 [Tribolium castaneum] AV398238 NV02 ...

  3. AV-95 Sun Devil: High-Speed Military Rotorcraft

    Science.gov (United States)

    1996-01-01

    The AV-95 Sun Devil must combine helicopter capabilities, such as vertical takeoff and landings (VTOL) and rotor-powered flight, along with long-duration cruise and high-speed dash capabilities unobtainable by conventional helicopters. To be able to perform both tasks, and perform them well, the AV-95 Sun Devil design incorporates several unconventional devices; the AV-95 uses two convertible turbofan engines, able to provide both shaft power for the main rotor and tall fan as well as jet thrust either separately or simultaneously. Other devices used for the AV-95 include a variable diameter main rotor and a blown flap. In helicopter mode, the AV-95 Sun Devil performs like a winged helicopter. The addition of wings to an attack helicopter results in two significant advantages. First, the addition of wings makes a helicopter more maneuverable than a wingless, but otherwise similar helicopter. Second, since the wings produce lift, rotor stall and compressibility effects can be significantly delayed at high tip velocities. In fixed-wing mode, the main rotor is completely off-loaded but slightly powered, and the rotor diameter has been minimized. The AV-95 Sun Devil has many advantages over other VTOL aircraft. The conversion process is simple and fast; conversion does not make the AV-95 vulnerable to enemy attack during conversion such as a tilt-wing or a tilt-rotor. Stop-rotor aircraft and a stowed rotor aircraft require heavy breaking of the rotor for conversion; this adds time for conversion and weight to the aircraft. Because the AV-95 never stops the rotor in flight, much weight is spared, and conversion is much simpler and faster.

  4. EST Table: AV404360 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404360 pg--0892 11/12/09 n.h 10/09/28 61 %/132 aa sp|P03764.2|STF_LAMBD RecName: ...Full=Side tail fiber protein 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV404360 pg-- ...

  5. EST Table: AV399747 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399747 NV120691 10/09/28 89 %/128 aa ref|YP_950793.1| gp41 [Maruca vitrata MNPV] ...gb|ABL76015.1| gp41 [Maruca vitrata MNPV] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV399524 NV12 ...

  6. Atle Næss: Roten av minus en

    Directory of Open Access Journals (Sweden)

    Linda Hamrin Nesby

    2006-06-01

    Full Text Available Atle Næss har skrevet flere romaner, og to biografier om hhv Edevard Munch og Galileo Galilei. Med Roten av minus en kombinerer han roman- og biografigenren, og skriver både en kjærlighetshistorie, et utkast til en biografi og et riss av en selvbiografi som i sum blir til en roman om hvorvidt livet har den orden og nødvendighet som biografien er satt til å formidle

  7. Gradering av kvaliteten på dokumentasjonen

    Directory of Open Access Journals (Sweden)

    Gunn E. Vist

    2013-11-01

    Full Text Available Hva menes med at noe er ”godt dokumentert”? I hvilken grad kan vi stole på forskningsresultater? Den mest brukte metoden for å vurdere kvalitet på dokumentasjon og styrke på anbefalinger er GRADE (Grading of Recommendations Assessment, Development and Evaluation. GRADE brukes i systematiske oversikter og i retningslinjer der man vurderer effekten av og gir anbefalinger om ulike behandlingsalternativer. I GRADE gjøres et klart skille mellom kvaliteten på dokumentasjonen og styrken på anbefalingene. For å vurdere kvaliteten på dokumentasjonen tar GRADE utgangspunkt i en godt utført systematisk oversikt som omfatter den aktuelle problemstillingen. Både randomiserte forsøk og observasjonsstuder kan inngå. GRADE benytter fem kriterier som vurderes for eventuell nedgradering av kvaliteten på dokumentasjonen: studiekvalitet, konsistens mellom studiene, direkthet, presisjon og rapporteringsskjevheter. I tillegg kan observasjonsstudier vurderes for oppgradering ved følgende tre kriterier: sterke assosiasjoner, dose-responseffekter og forvekslingsfaktorer. Alle vurderingene noteres og presenteres sammen med resultatene i oppsummeringstabeller.Vist GE, Sæterdal I, Vandvik PO, Flottorp SA. Grading the quality of the evidence. Nor J Epidemiol 2013; 23 (2: 151-156.ENGLISH SUMMARY What is meant by the claim that something is “well documented”? How much confidence can we have in the results? The most commonly used method to grade the quality of the evidence is GRADE (Grading of Recommendations Assessment, Development and Evaluation. GRADE is used in systematic reviews, where the effects of different treatments or options are being compared, and in guidelines with recommendations about the competing options. GRADE makes a clear distinction between the quality of the evidence and the strength of recommendation. When grading the quality of the evidence with GRADE, the starting point is a well conducted systematic review of research on the

  8. Problemstillingar rundt framføring av transkribert musikk - Ei analyse av Bach/Busoni: Chaconne i d-moll

    OpenAIRE

    Flem, Kato Nogva

    2014-01-01

    Ei samanlikning og analyse av Busoni sin transkripsjon av Bach sin Chaconne frå partita nr. 2 for solofiolin. Partita for Violin No. 2 was written by Johann Sebastian Bach during 1717-1723 and consists of five movements; Allemande, Courante, Sarabande, Gigue and Chaconne. The chaconne is longer than the rest of the piece combined, and is frequently played separately. Yehudi Menuhin calls the Chaconne "the greatest structure for solo violin that exists." (Menuhin, 1997, p. 236) Violinist...

  9. Enhanced A-V nodal conduction (Lown-Ganong-Levine syndrome) by congenitally hypoplastic A-V node.

    Science.gov (United States)

    Ometto, R; Thiene, G; Corrado, D; Vincenzi, M; Rossi, L

    1992-11-01

    The basic anatomical substrate of enhanced A-V nodal conduction, manifesting or not as Lown-Ganong-Levine syndrome, is still a controversial issue. We describe the case of a 34-year-old man who presented episodes of ventricular fibrillation. Electrophysiological studies showed that the AH interval was 55 ms, and increased by only 20 ms at paced cycle lengths of 300 ms; atrial pacing induced atrial fibrillation, with a shortest RR interval of 240 ms. Despite verapamil therapy, this patient died suddenly at home. Histological study disclosed a severe A-V node hypoplasia that was evidently congenital in nature; the rest of the conduction system was normal, and no accessory A-V pathways were present. We suggest that enhanced A-V nodal conduction in this patient was due to the developmental defect in the A-V node; this abnormality caused a loss of specific impulse-delaying function, and thus allowed rapid, unfiltered atrial impulses to reach the lower A-V junction and ventricles.

  10. Spectral analysis of the quadrangles Av-13 and Av-14 on Vesta

    Science.gov (United States)

    Zambon, F.; Frigeri, A.; Combe, J.-Ph.; Tosi, F.; Longobardo, A.; Ammannito, E.; De Sanctis, M. C.; Blewett, D. T.; Scully, J.; Palomba, E.; Denevi, B.; Yingst, A.; Russell, C. T.; Raymond, C. A.

    2015-10-01

    The Av-13 (Tuccia) and Av-14 (Urbinia) quadrangles are located in the south-west region of Vesta. They are characterized by a large topographic variability, from the highest (Vestalia terra highlands) to the lowest (Rheasilvia basin). Many geological units in these quadrangles are not associated with mineralogical variability, as shown by the color-composite maps. Maps of mafic absorption band-center position reveal that the principal lithology is eucrite-rich howardite, but diogenite-rich howardite areas are also present, corresponding to particular features such as Antonia and Justina craters, which are characterized by strong mafic absorptions. These quadrangles, especially Urbinia, contain many bright ejecta, such as those of Tuccia crater, which are the highest reflectance materials on Vesta (Zambon et al., 2014). Dark areas are present and correspond to regions with deeper OH-signature. The two quadrangles contain many vertical ridge crests associated with the Rheasilvia impact. These ridges do not show mineralogical differences with respect to their surroundings, but have a distinctive appearance in color-ratio composite images.

  11. 快速 AVS 帧内预测算法%FAST AVS INTRA PREDICTION ALGORITHM

    Institute of Scientific and Technical Information of China (English)

    王双龙

    2015-01-01

    As to the complexity process of choosing best probable encoding mode in AVS intra prediction,we proposed a fast intra predic-tion encoding algorithm.With the analysis of AVS intra prediction encoding theory and its implementation in reference software GDM2.1,we derived the threshold of zero coefficient block determination.The fast algorithm determines whether or not the current block is a zero coeffi-cient block according to its threshold discriminant condition.For zero coefficient block,it encodes cbp element directly and terminates intra prediction encoding in advance,which avoids the consequent complex process.As for the non-zero coefficient blocks,the fast selection algo-rithm associating the time domain SAD and the frequency domain SATD is used,which excludes 40% to 80% impossible prediction modes, and the rate distortion function is applied to the remained prediction modes to select the best prediction mode.It was verified through experi-ment that when PSNR (peak signal-to-noise ratio)decreased within 0.25db and bits rate increased within 2%,the time of AVS encoding de-creased over 40%,and the encoding efficiency was largely improved as well.%针对 AVS(Audio Video code Standard)帧内预测中选择最优编码模式流程复杂的问题,提出一种快速帧内预测编码的算法。通过分析 AVS 帧内预测编码的原理及其在参考软件 GDM2.1中的代码实现,推导出判定零系数块的阈值。该快速算法根据零系数块的判定阈值条件判定当前块,对于零系数块,直接编码 cbp 元素,提前中止帧内预测编码,避免了后续的复杂流程;对于非零系数块,采用时域 SAD(Sum of Absolute Difference)与频域 SATD(Sum of Absolute Transformed Difference)相联合的快速选择算法,排除了40%~80%的不可能预测模式,对余下的模式采用率失真代价函数的方法选取最佳预测模式。经实验验证,在 PSNR(Peak Sig-nal to Noise Ratio)降低0

  12. Kortisonskepsis : En studie om holdninger til kortisonkrem blant foreldre av barn med atopisk eksem

    OpenAIRE

    Gustavsen, Hanne Engstrøm

    2010-01-01

    Bakgrunn: Kortisonkremer er en viktig del av behandlingen mot atopisk eksem. Mange foreldre er skeptiske til bruken av kortisonkremer, fordi de har lest eller hørt om bivirkninger. Pasienters og pårørendes holdninger til bruk av kortisonkrem kan påvirke gjennomføringen av behandlingen. Formål: Vi ønsket å undersøke holdninger til bruk av kortisonkrem blant foreldre og pårørende av barn med atopisk eksem. Vi ville også undersøke om det er en sammenheng mellom kunnskapsnivå om kortisonkreme...

  13. Utviklingen av Noark-standarden 1984 – 2008

    Directory of Open Access Journals (Sweden)

    Trond Sirevåg

    2014-11-01

    Full Text Available Artikkelen behandler utviklingen av Noark – standarden for elektronisk arkivdanning i norsk offentlig forvaltning – fra versjon 1 (1984 til versjon 5 (2008. Den beskriver omstendighetene som medførte at standarden ble til i 1984, og søker å forklare hvorfor og hvordan det lyktes å utbre standarden slik at Noark-systemer ble enerådende i offentlig forvaltning i løpet av 1990-årene. Riksarkivarens strategier i Noark-utviklingen behandles, – de som fra først av fikk Riksarkivaren til å engasjere seg i standardiseringsarbeidet, de som deretter fikk Riksarkivaren til å overta forvalteransvaret for Noark, og de som senere er endret eller justert som resultat av erfaringer med Noark-baserte systemer og av nyåpnede teknologiske muligheter. Særlig vekt legges på å belyse Riksarkivarens motiver for å overta ansvaret for Noark-standarden fra 1990. Noark plasseres også i det internasjonale bildet, og sammenholdes med andre standarder som ISO 15489 og EU-standarden MoReq. Da Noark-utviklingen startet i 1984, pågikk tilsvarende utviklingsprosjekter både i Danmark og i Sverige. Artikkelen søker forklaringer på at en journal- og sakarkivstandard bare ble en realitet i Norge.

  14. Kartläggning av byggprocessen hos Peab med hjälp av SCOR

    OpenAIRE

    Johansson, Robin

    2012-01-01

    Detta är ett examensarbete utfört på Institutionen för teknik och naturvetenskap (ITN) i Norrköping som är en del av Linköpings Universitet i samarbete med Peab som intressent. Historiskt sett har byggbranschens intresse för logistik varit svalt vilket har lett till att logistiken inom denna bransch har halkat efter i förhållande till övriga branscher. Syftet med detta examensarbete är att utifrån ett logistiskt synsätt tillhandahålla anpassningsförslag till SCOR-processerna ”Make” och ”Retur...

  15. The role of AV and VV optimization for CRT

    Directory of Open Access Journals (Sweden)

    William W. Brabham, M.D.

    2013-06-01

    Full Text Available Cardiac resynchronization therapy is an effective therapy for patients with left ventricular systolic dysfunction and a ventricular conduction delay; however, approximately 30% of patients do not experience significant clinical improvement with this treatment. Modern devices allow individualized programming of the AV delay and VV offset, which offer the possibility of improving clinical response rates with optimized programming. AV and VV delay optimization techniques have included echocardiography, device-based algorithms, and several other novel noninvasive techniques. While an acute improvement in hemodynamic function has been clearly demonstrated with optimized device settings, long-term clinical benefit is limited. In the majority of cases, an empiric AV delay with simultaneous biventricular or left ventricular pacing is adequate. The value of optimization of these intervals in “non-responders” still requires further investigation.

  16. Solmoduler på balkongfronter : Renovering av flerbostadshus i miljonprogrammet

    OpenAIRE

    Persson, Maria

    2015-01-01

    Under det senaste seklet har en intensiv energianvändning orsakat stora utsläpp av koldioxid och andra växthusgaser i atmosfären med negativa miljöeffekter som följd. Den alltjämt ökade efterfrågan på elektricitet runtom i världen måste mötas av förnybara energikällor i kombination med energieffektiviseringar för att inte riskera ytterligare miljöförstöring. I Sverige är bostads- och servicesektorn en intensiv energianvändare och några av de byggnader som förbrukar mest energi finns i miljonp...

  17. En kartläggning av stereoskopisk 3D

    OpenAIRE

    Holmström, Benny

    2011-01-01

    Det här lärdomsprovet är en genomgång av ett av den moderna tidens största modeord: stereoskopisk 3D och syskontermen autostereoskopisk 3D. Kort sagt de tekniker som går ut på att leverera en bild till vardera av människans två ögon och därmed simulera djup i tvådimensionella bilder. Det här kan göras på en mängd olika sätt, och i detta examensarbete går jag igenom de vanligaste teknikerna och förklarar dem kortfattat, en och en. Utöver tekniken analyserar jag även dagens marknad, teknikernas...

  18. Brachial artery aneurysms following brachio-cephalic AV fistula ligation.

    Science.gov (United States)

    Khalid, Usman; Parkinson, Frances; Mohiuddin, Kamran; Davies, Paula; Woolgar, Justin

    2014-01-01

    Peripheral artery aneurysms proximal to a long-standing arteriovenous (AV) fistula can be a serious complication. It is important to be aware of this and manage it appropriately. Vascular access nurses input all data regarding patients undergoing dialysis access procedures into a securely held database prospectively. This was retrospectively reviewed to identify cases of brachial artery aneurysms over the last 3 years. In Morriston Hospital, around 200 forearm and arm AV fistulas are performed annually for vascular access in renal dialysis patients. Of these, approximately 15 (7.5%) are ligated. Three patients who had developed brachial artery aneurysms following AV fistula ligation were identified. All 3 patients had developed brachial artery aneurysms following ligation of a long-standing brachio-cephalic AV fistula. Two patients presented with pain and a pulsatile mass in the arm, and one presented with pins and needles and discoloration of fingertips. Two were managed with resection of the aneurysm and reconstruction with a reversed long saphenous vein interposition graft, the third simply required ligation of a feeding arterial branch. True aneurysm formation proximal to an AV fistula that has been ligated is a rare complication. There are several reasons for why these aneurysms develop in such patients, the most plausible one being the increase in blood flow and resistance following ligation of the AV fistula. Of note, all the patients in this study were on immunosuppressive therapy following successful renal transplantation. Vigilance by the vascular access team and nephrologists is paramount to identify those patients who may warrant further evaluation and investigation by the vascular surgeon.

  19. Lesing av delvis motstridende tekster i syvende klasse

    Directory of Open Access Journals (Sweden)

    Wenke Mork Rogne

    2013-06-01

    Full Text Available I denne artikkelen ser vi på hvilken type informasjon elever i 7. klasse vektlegger for å konstruere en sammenhengende oppsummering av fire til dels motstridende tekster. Elever møter ofte flere tekster om samme tema – multiple tekster – og vi trenger økt kunnskap om hvordan de leser slike tekster. Elevene leste fire ulike tekster om en sykkelulykke. Tekstene presenterte informasjon som var delvis sammenfallende på tvers av alle tekstene, og delvis motstridende informasjon som innebar at enkelte tekster ga ulike beskrivelser av hendelsesforløpet. Vi testet elevenes ordavkodingsferdigheter, fikk dem til å fylle ut et spørreskjema om lesevaner og vi innhentet elevenes resultater på Nasjonal leseprøve. Etter lesing ba vi elevene gi en oppsummering av sykkelulykken. Elevene gjenga relativt mer sammenfallende informasjon enn motstridende informasjon. Gode resultater på Nasjonal leseprøve ser ut til å ha en sammenheng med elevenes ferdigheter i å konstruere en sammenhengende forståelse av de fire tekstene. Elever som oppga å lese mye på Internett hadde imidlertid fått med mindre av informasjonen som var felles for de fire tekstene i sine oppsummeringer. Vi fant også en negativ sammenheng mellom fritidslesing på Internett og resultatene fra Nasjonal leseprøve. Samlet sett indikerer resultatene at når elevene skal skape sammenheng mellom flere delvis motstridende tekster, så blir sammenfallende informasjon vektlagt mer enn motstridende informasjon. Dessuten ser det ut til at de elevene som bruker mest fritid til å lese på Internett, sliter mer med å sammenfatte innholdet i de fire tekstene enn de andre elevene.

  20. InterProScan Result: AV398312 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398312 AV398312_3_ORF2 E26799A422BE33BE PANTHER PTHR10795:SF30 SITE-1 PROTEASE (STEROL...-REGULATED, CLEAVES STEROL REGULATORY ELEMENT BINDING PROTEINS) NA ? IPR015500 unintegrated ...

  1. InterProScan Result: AV404435 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404435 AV404435_2_ORF1 15076070D471749D PROFILE PS50110 RESPONSE_REGULATORY 17.91...4 T IPR001789 Signal transduction response regulator, receiver domain Molecular Function: two-component resp

  2. Modellering og dimensjonering av kaianlegg etter Eurokode 2

    OpenAIRE

    Lilleaker, Tore; Tvervåg, Henrik

    2010-01-01

    Dette er sluttrapporten for masteroppgaven på en kaikonstruksjon utført ved NTNU, institutt for konstruksjonsteknikk. Det er i oppgaven gjennomført en prosjektering og dimensjonering av en betongkai etter NS-EN 1992-1-1. Det er også gjennomført et litteraturstudie med en tilhørende teoridel om bestandighet for betongkonstruksjoner i et marint miljø, samt at det er gjennomført en sannsynlighetsbasert bestandighetsanalyse av denne konstruksjonen. Avslutningsvis er det drøftet litt rundt teori, ...

  3. EST Table: AV403981 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available terminase large subunit (DNA packaging protein A) from bacteriophage origin [Escherichia coli UMN026] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV403981 pg-- ... ...AV403981 pg--0297 11/12/09 n.h 10/09/28 100 %/265 aa ref|YP_002411376.1| terminase large subunit (DNA packag...ing protein A) from bacteriophage origin [Escherichia coli UMN026] emb|CAR11828.1|

  4. Modellering av nedslagsfeltet- Veumdalen i Fredrikstad med programmet SWMM

    OpenAIRE

    2015-01-01

    Fredrikstad kommune har hatt store prosjekter pågang i de siste årene i forbindelse med opprustning av ledningsnettet. Oppfylte kjellere har vært en viktig tema for kommunen, og de har en intensjon om å redusere faren for oversvømmelser i utsatte steder. En av de utsatte stedene i Fredrikstad er Veumdalen som har problemer med oversvømmelser nesten hvert år. Som en forebyggende tiltak har kommunen i samarbeid med COWI bygget en tunnel fra Veumveien/Veumbekken til Seutelva. Det ...

  5. EST Table: AV399012 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399012 NV060536 11/12/09 n.h 10/09/28 65 %/135 aa ref|XP_396932.2| PREDICTED: similar to vav...ene:AGAP010292 10/09/10 65 %/135 aa gnl|Amel|GB13170-PA 10/09/10 57 %/138 aa gi|189241833|ref|XP_972772.2| PREDICTED: similar to vav1 [Tribolium castaneum] AV399012 NV06 ... ...7 %/132 aa C35B8.2b#CE39333#WBGene00006887#locus:vav- 1#status:Partially_confirmed#UniProt:Q45FX5#protein_id

  6. EST Table: AV398785 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398785 NV060280 11/12/09 n.h 10/09/28 40 %/193 aa ref|XP_002005659.1| GI20589 [Drosophila mojav...ensis] gb|EDW09594.1| GI20589 [Drosophila mojavensis] 10/08/28 40 %/193 aa FBpp0169806|DmojGI2.../10 n.h 10/09/10 35 %/184 aa gi|189234823|ref|XP_001809803.1| PREDICTED: similar to AGAP010960-PA [Tribolium castaneum] AV398785 NV06 ...

  7. EST Table: AV399741 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399741 NV120677 11/12/09 GO hit GO:0000213(tRNA-intron endonuclease activity)|GO:...0006388(tRNA splicing, via endonucleolytic cleavage and ligation) 10/09/28 42 %/119 aa ref|NP_001127799.2| t...0/08/27 n.h 10/09/10 n.h 10/09/10 35 %/145 aa gnl|Amel|GB15173-PB 10/09/10 n.h AV399741 NV12 ...

  8. EST Table: AV399757 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399757 NV120719 11/12/09 n.h 10/09/28 83 %/234 aa ref|NP_047435.1| AcMNPV orf30 [...Bombyx mori NPV] gb|AAC63704.1| AcMNPV orf30 [Bombyx mori NPV] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h AV399757 NV12 ...

  9. Prosjektering og analyse av Nordøyvegen bru 3

    OpenAIRE

    Lauknes, Andreas; Arild, Audun

    2016-01-01

    Rapporten tar for seg prosjektering av en etteroppspent betongbru, der beregninger og dimensjoneringen er i henhold til det Europeiske regelverket, nasjonale tillegg og Håndbøker fra Statens vegvesen. Brua som skal dimensjoneres og bli gjort kapasitetskontroll for er Nordøyvegen bru 3, som skal bygges som en del av et større fastlandsprosjekt for nordøyene i Haram kommune. Brua skal bygges som en bjelke-/platebru, og konstruksjonsmetoden er fritt frambygg med totalt syv byggefaser. Den består...

  10. Fysisk aktivitet inom rehabiliteringen av anorexi : - En systematisk litteraturstudie

    OpenAIRE

    Högnäs, Maria

    2011-01-01

    Studiens syfte var att undersöka hur fysisk aktivitet påverkar unga flickor (12-24 år) med anorexi, i samband med rehabilitering. Skribenten vill dessutom ytterligare få specificerat vilken typ av fysisk aktivitet som kan vara lämplig för denna målgrupp. Frågeställningarna besvaras genom en systematisk litteraturstudie. Genom databassökning och frisökning hittades 14 relevanta artiklar som genomgick en kvalitetsgranskning, enligt en modifierad metod för kvalitetsgranskning av Forsberg & Wengs...

  11. Norsk passivhusstandard og Passivhauskonzept: en sammenlignende analyse av krav og kriterier

    DEFF Research Database (Denmark)

    Anton, Karin; Vestergaard, Inge

    kriterier som omfattes av passivhuskonseptet med tilhørende sertifisering og kriterier i Norsk Standard 3701. Gjennom analysen ønsker vi å systematisere og sammenlikne de forskjellige kriteriene i standarden: optimalisering, kuldebroer, lufttetthet og trykktesting, utnyttelse av passiv solenergi, bruk av...

  12. Ungdommers opplevelser med overdreven bruk av online-rollespillet World of Warcraft

    DEFF Research Database (Denmark)

    Flaaten, Øivind; Torp, Steffen; Aarseth, Espen

    2010-01-01

    Ungdommers bruk av online-rollespill blir ofte problematisert (Falkner 2007). På bakgrunn av kvalitative intervjuer med ti ungdommer og fem pårørendekontakter beskrives i denne artikkelen ungdommenes opplevelser av online-spillet World of Warcraft (WoW). Denne pilotstudien gir en nyansert...

  13. Etableringen av Tesla Motors på det norske bilmarkedet : en empirisk studie av salgsdrivere og konkurranseeffekter

    OpenAIRE

    Asperheim, Hanne Henjum; Vedum, Anders

    2015-01-01

    Denne masterutredningen er en empirisk studie av Tesla Motors’ etablering på det norske bilmarkedet. Vi gjennomfører to separate analyser: Først fokuseres det på geografiske salgsforskjeller, deretter på effekten Tesla Motors har hatt på det norske nybilmarkedet. I vår første analyse utvikler vi en modell for å identifisere Teslas salgsdrivere, i form av geografiske og sosioøkonomiske variabler. Resultatene viser at et fylkes inntektsnivå, utdanningsnivå og miljøbevissthet har en positiv s...

  14. EST Table: AV404171 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404171 pg--0555 10/09/28 85 %/178 aa ref|ZP_05092873.1| phage tail tape measure p...rotein, lambda family [Carboxydibrachium pacificum DSM 12653] gb|EEB75267.1| phage tail tape measure protein

  15. EST Table: AV404301 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404301 pg--0808 10/09/28 81 %/199 aa ref|ZP_05092873.1| phage tail tape measure p...rotein, lambda family [Carboxydibrachium pacificum DSM 12653] gb|EEB75267.1| phage tail tape measure protein

  16. EST Table: AV404025 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404025 pg--0351 11/12/09 n.h 10/09/28 84 %/167 aa ref|ZP_05092873.1| phage tail tape measure... protein, lambda family [Carboxydibrachium pacificum DSM 12653] gb|EEB75267.1| phage tail tape measure

  17. EST Table: AV404116 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404116 pg--0495X 10/09/28 87 %/148 aa ref|YP_311293.1| putative tail length tape measure... protein [Shigella sonnei Ss046] gb|AAZ89058.1| putative tail length tape measure protein [Shigella s

  18. EST Table: AV400897 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400897 br--1948 10/09/28 47 %/180 aa ref|XP_967144.2| PREDICTED: similar to corneal wound healing...TED: similar to corneal wound healing-related protein [Tribolium castaneum] CK493011 br-- ...

  19. EST Table: AV405806 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405806 wdV30260 10/09/28 65 %/199 aa ref|NP_001119709.1| transformer 2 isoform E ...[Bombyx mori] gb|AAX47003.1| transformer-2 protein E [Bombyx mori] 10/08/28 low homology 10/08/27 low homolo

  20. EST Table: AV400604 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400604 br--1517 10/09/28 48 %/208 aa ref|XP_001863389.1| wolfram syndrome 1 [Cule...x quinquefasciatus] gb|EDS38516.1| wolfram syndrome 1 [Culex quinquefasciatus] 10/08/28 50 %/173 aa FBpp0291

  1. EST Table: AV398407 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398407 NV021774 10/09/28 100 %/186 aa ref|NP_001040215.1| stathmin [Bombyx mori] gb|ABD36259.1| stathmin...507-PA 10/09/10 48 %/236 aa gi|91083957|ref|XP_975021.1| PREDICTED: similar to stathmin [Tribolium castaneum] FS915193 NV02 ...

  2. EST Table: AV398495 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398495 NV021874 10/09/28 91 %/209 aa ref|NP_001040215.1| stathmin [Bombyx mori] gb|ABD36259.1| stathmin...07-PA 10/09/10 54 %/213 aa gi|91083957|ref|XP_975021.1| PREDICTED: similar to stathmin [Tribolium castaneum] FS915193 NV02 ...

  3. EST Table: AV401468 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401468 e96h0771 10/09/28 87 %/171 aa ref|NP_001040215.1| stathmin [Bombyx mori] gb|ABD36259.1| stathmin...07-PA 10/09/10 55 %/166 aa gi|91083957|ref|XP_975021.1| PREDICTED: similar to stathmin [Tribolium castaneum] FS915193 e96h ...

  4. EST Table: AV403752 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV403752 pg--0009 10/09/28 100 %/257 aa ref|YP_002411376.1| terminase large subunit (DNA packaging... protein A) from bacteriophage origin [Escherichia coli UMN026] emb|CAR11828.1| terminase large subunit (DNA packaging

  5. EST Table: AV404329 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404329 pg--0843 10/09/28 100 %/173 aa ref|NP_001136443.1| clathrin heavy chain [B...61 %/169 aa T20G5.1#CE00480#WBGene00011867#locus:chc-1#Clathrin heavy chain#status:Partially_confirmed#UniPr

  6. EST Table: AV405172 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405172 prgv0893 10/09/28 85 %/198 aa gb|ABF55966.2| cleavage stimulation factor 6...ref|XP_970762.1| PREDICTED: similar to cleavage stimulation factor 64-kDa subunit [Tribolium castaneum] FS920483 prgv ...

  7. EST Table: AV400944 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400944 e96h0016 10/09/28 73 %/153 aa ref|XP_002005200.1| GI20361 [Drosophila mojav...ensis] gb|EDW09135.1| GI20361 [Drosophila mojavensis] 10/08/28 73 %/153 aa FBpp0169578|DmojGI20361-PA 10/08

  8. EST Table: AV398908 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398908 NV060415 10/09/28 95 %/160 aa ref|XP_002004473.1| GI19595 [Drosophila mojav...ensis] ref|XP_002059335.1| GJ18395 [Drosophila virilis] gb|EDW08408.1| GI19595 [Drosophila mojavensis] gb|E

  9. EST Table: AV400707 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400707 br--1674X 10/09/28 54 %/135 aa ref|XP_002008152.1| GI11978 [Drosophila mojav...ensis] gb|EDW18628.1| GI11978 [Drosophila mojavensis] 10/08/28 54 %/135 aa FBpp0161195|DmojGI11978-PA 10/0

  10. EST Table: AV401018 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401018 e96h0121 10/09/28 81 %/103 aa ref|XP_002007281.1| GI12464 [Drosophila mojav...ensis] gb|EDW17757.1| GI12464 [Drosophila mojavensis] 10/08/28 81 %/103 aa FBpp0161681|DmojGI12464-PA 10/08

  11. EST Table: AV399925 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399925 br--0031 10/09/28 87 %/140 aa ref|XP_002008796.1| GI13691 [Drosophila mojav...ensis] gb|EDW19272.1| GI13691 [Drosophila mojavensis] 10/08/28 87 %/140 aa FBpp0162908|DmojGI13691-PA 10/08

  12. EST Table: AV401292 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401292 e96h0497 10/09/28 99 %/102 aa ref|XP_002004473.1| GI19595 [Drosophila mojav...ensis] ref|XP_002059335.1| GJ18395 [Drosophila virilis] gb|EDW08408.1| GI19595 [Drosophila mojavensis] gb|E

  13. EST Table: AV405991 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405991 wdV30476 10/09/28 81 %/103 aa ref|XP_002007281.1| GI12464 [Drosophila mojav...ensis] gb|EDW17757.1| GI12464 [Drosophila mojavensis] 10/08/28 81 %/103 aa FBpp0161681|DmojGI12464-PA 10/08

  14. EST Table: AV399270 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399270 NV060991 10/09/28 41 %/162 aa ref|XP_002007640.1| GI13052 [Drosophila mojav...ensis] gb|EDW18116.1| GI13052 [Drosophila mojavensis] 10/08/28 41 %/162 aa FBpp0162269|DmojGI13052-PA 10/08

  15. EST Table: AV402088 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402088 heS00552 10/09/28 57 %/120 aa ref|XP_002003339.1| GI17860 [Drosophila mojav...ensis] gb|EDW12781.1| GI17860 [Drosophila mojavensis] 10/08/28 57 %/120 aa FBpp0167077|DmojGI17860-PA 10/08

  16. EST Table: AV404032 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404032 pg--0359 10/09/28 39 %/179 aa ref|XP_002008018.1| GI12064 [Drosophila mojav...ensis] gb|EDW18494.1| GI12064 [Drosophila mojavensis] 10/08/28 39 %/179 aa FBpp0161281|DmojGI12064-PA 10/08

  17. EST Table: AV398210 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398210 NV021247 10/09/28 77 %/206 aa ref|XP_001998997.1| GI23318 [Drosophila mojav...ensis] gb|EDW14458.1| GI23318 [Drosophila mojavensis] 10/08/28 77 %/206 aa FBpp0172535|DmojGI23318-PA 10/08

  18. EST Table: AV398468 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398468 NV021845 10/09/28 40 %/208 aa ref|XP_001998750.1| GI23457 [Drosophila mojav...ensis] gb|EDW14211.1| GI23457 [Drosophila mojavensis] 10/08/28 40 %/208 aa FBpp0172674|DmojGI23457-PA 10/08

  19. EST Table: AV398578 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398578 NV021976 10/09/28 61 %/149 aa ref|XP_968620.1| PREDICTED: similar to AGAP0.../08/28 55 %/161 aa FBpp0085738|SdhA-PC 10/08/27 60 %/131 aa C03G5.1#CE03917#WBGene00015391#locus:sdha-1#succinate dehydrogenase flav

  20. EST Table: AV401286 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401286 e96h0482 10/09/28 55 %/188 aa ref|XP_002004616.1| GI19509 [Drosophila mojav...ensis] gb|EDW08551.1| GI19509 [Drosophila mojavensis] 10/08/28 55 %/188 aa FBpp0168726|DmojGI19509-PA 10/08

  1. EST Table: AV401287 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401287 e96h0483 10/09/28 93 %/160 aa ref|XP_002004473.1| GI19595 [Drosophila mojav...ensis] ref|XP_002059335.1| GJ18395 [Drosophila virilis] gb|EDW08408.1| GI19595 [Drosophila mojavensis] gb|E

  2. EST Table: AV406016 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV406016 wdV30506 10/09/28 81 %/103 aa ref|XP_002007281.1| GI12464 [Drosophila mojav...ensis] gb|EDW17757.1| GI12464 [Drosophila mojavensis] 10/08/28 81 %/103 aa FBpp0161681|DmojGI12464-PA 10/08

  3. EST Table: AV406029 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV406029 wdV30520 10/09/28 70 %/125 aa ref|XP_002007572.1| GI12310 [Drosophila mojav...ensis] gb|EDW18048.1| GI12310 [Drosophila mojavensis] 10/08/28 71 %/125 aa FBpp0127559|DanaGF24367-PA 10/08

  4. EST Table: AV400537 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400537 br--1414 10/09/28 41 %/216 aa ref|XP_002000083.1| GI10045 [Drosophila mojav...ensis] gb|EDW15544.1| GI10045 [Drosophila mojavensis] 10/08/28 41 %/216 aa FBpp0159262|DmojGI10045-PA 10/08

  5. EST Table: AV400979 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400979 e96h0061 10/09/28 57 %/143 aa ref|XP_002004493.1| GI19964 [Drosophila mojav...ensis] gb|EDW08428.1| GI19964 [Drosophila mojavensis] 10/08/28 57 %/143 aa FBpp0169181|DmojGI19964-PA 10/08

  6. EST Table: AV398644 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398644 NV060124 10/09/28 96 %/174 aa ref|XP_002004473.1| GI19595 [Drosophila mojav...ensis] ref|XP_002059335.1| GJ18395 [Drosophila virilis] gb|EDW08408.1| GI19595 [Drosophila mojavensis] gb|E

  7. EST Table: AV404441 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404441 pg--0982 10/09/28 79 %/229 aa ref|XP_001998398.1| GI23651 [Drosophila mojav...ensis] gb|EDW13859.1| GI23651 [Drosophila mojavensis] 10/08/28 79 %/229 aa FBpp0172868|DmojGI23651-PA 10/08

  8. EST Table: AV398478 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398478 NV021855 10/09/28 99 %/204 aa ref|NP_001136443.1| clathrin heavy chain [Bo...6 %/204 aa T20G5.1#CE00480#WBGene00011867#locus:chc-1#Clathrin heavy chain#status:Partially_confirmed#UniPro

  9. EST Table: AV402568 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402568 heS30139 10/09/28 48 %/151 aa ref|XP_002006691.1| GI18446 [Drosophila mojav...ensis] gb|EDW10626.1| GI18446 [Drosophila mojavensis] 10/08/28 48 %/151 aa FBpp0167663|DmojGI18446-PA 10/08

  10. EST Table: AV404233 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404233 pg--0732 10/09/28 70 %/129 aa ref|XP_002003556.1| GI21941 [Drosophila mojav...ensis] gb|EDW12998.1| GI21941 [Drosophila mojavensis] 10/08/28 70 %/129 aa FBpp0171158|DmojGI21941-PA 10/08

  11. EST Table: AV399704 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399704 NV120582 10/09/28 80 %/230 aa ref|XP_001998398.1| GI23651 [Drosophila mojav...ensis] gb|EDW13859.1| GI23651 [Drosophila mojavensis] 10/08/28 80 %/230 aa FBpp0172868|DmojGI23651-PA 10/08

  12. EST Table: AV404790 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404790 prgv0452 10/09/28 65 %/113 aa ref|XP_001979237.1| GG24851 [Drosophila erec...10/09/10 59 %/106 aa gi|91086741|ref|XP_971589.1| PREDICTED: similar to riboflavin kinase [Tribolium castaneum] FS912700 prgv ...

  13. EST Table: AV400760 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400760 br--1758 10/09/28 64 %/214 aa ref|XP_307835.4| AGAP009424-PA [Anopheles ga...9241497|ref|XP_967205.2| PREDICTED: similar to neuron navigator 2 [Tribolium castaneum] BY916808 br-- ...

  14. EST Table: AV405194 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405194 prgv0919 10/09/28 65 %/160 aa ref|XP_002000766.1| GI22341 [Drosophila mojav...ensis] gb|EDW16227.1| GI22341 [Drosophila mojavensis] 10/08/28 65 %/160 aa FBpp0171558|DmojGI22341-PA 10/08

  15. EST Table: AV403131 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV403131 msgV0077 10/09/28 92 %/200 aa ref|XP_002004473.1| GI19595 [Drosophila mojav...ensis] ref|XP_002059335.1| GJ18395 [Drosophila virilis] gb|EDW08408.1| GI19595 [Drosophila mojavensis] gb|E

  16. EST Table: AV398368 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398368 NV021731 10/09/28 95 %/237 aa ref|NP_001136443.1| clathrin heavy chain [Bo...2 %/237 aa T20G5.1#CE00480#WBGene00011867#locus:chc-1#Clathrin heavy chain#status:Partially_confirmed#UniPro

  17. EST Table: AV399507 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399507 NV120319 10/09/28 70 %/144 aa ref|YP_803401.1| baculovirus repeated ORF-b [Anticars...ia gemmatalis nucleopolyhedrovirus] gb|ABI13791.1| baculovirus repeated ORF-b [Anticarsia gemmatali

  18. EST Table: AV403922 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV403922 pg--0214 10/09/28 91 %/142 aa ref|ZP_06118881.2| DNA packaging protein FI ...[Clostridium hathewayi DSM 13479] gb|EFC94454.1| DNA packaging protein FI [Clostridium hathewayi DSM 13479

  19. EST Table: AV404246 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404246 pg--0747 10/09/28 91 %/130 aa ref|ZP_06118881.2| DNA packaging protein FI ...[Clostridium hathewayi DSM 13479] gb|EFC94454.1| DNA packaging protein FI [Clostridium hathewayi DSM 13479

  20. EST Table: AV403894 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available ref|XP_001605691.1| PREDICTED: similar to xaa-pro dipeptidase app(e.coli) [Nasonia vitripennis] 10/08/28 52...%/206 aa gi|189241712|ref|XP_968082.2| PREDICTED: similar to xaa-pro dipeptidase app(e.coli) [Tribolium castaneum] AV403894 pg-- ...

  1. Centralized Library Services for Audiovisual Media. AV in Action 4.

    Science.gov (United States)

    Nederlands Bibliotheek en Lektuur Centrum, The Hague (Netherlands).

    Designed to provide assistance to countries in developing centralized services to their libraries for nonbook materials, this pamphlet contains examples from five countries that have succeeded in establishing such services. Those examples include: (1) "The Central Library Service for AV-Materials in Denmark" (Suzanne Hemmeth…

  2. EST Table: AV400204 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400204 br--0896 10/09/28 36 %/111 aa ref|XP_967620.1| PREDICTED: similar to anopheles stephen...093471|ref|XP_967620.1| PREDICTED: similar to anopheles stephensi ubiquitin, putative [Tribolium castaneum] FS914988 br-- ...

  3. EST Table: AV400925 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400925 br--1989 10/09/28 35 %/122 aa ref|XP_967620.1| PREDICTED: similar to anopheles stephen...nl|Amel|GB19565-PA 10/09/10 35 %/122 aa gi|91093471|ref|XP_967620.1| PREDICTED: similar to anopheles stephensi ubiquitin, putative [Tribolium castaneum] FS914988 br-- ...

  4. EST Table: AV400917 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400917 br--1979 10/09/28 46 %/148 aa ref|XP_975194.1| PREDICTED: similar to something about...|Amel|GB19080-PA 10/09/10 46 %/148 aa gi|91081571|ref|XP_975194.1| PREDICTED: similar to something about silencing protein 10 [Tribolium castaneum] FS937283 br-- ...

  5. EST Table: AV398194 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398194 NV021231 10/09/28 38 %/170 aa ref|NP_724426.2| vulcan, isoform D [Drosophi...la melanogaster] gb|AAM68355.2| vulcan, isoform D [Drosophila melanogaster] 10/08/28 38 %/170 aa FBpp0289730

  6. EST Table: AV406060 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV406060 wdV30561X 10/09/28 99 %/122 aa gb|ACN86367.1| troponin T transcript varian...006588#locus:tnt- 3#status:Partially_confirmed#UniProt:B9WRS6#protein_id:AC M46022.1 10/09/10 81 %/122 aa AG

  7. EST Table: AV405690 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405690 wdV30118 10/09/28 93 %/225 aa ref|NP_001040124.1| exuperantia [Bombyx mori] gb|ABD36117.1| exuperan...9360-PA 10/09/10 53 %/220 aa gi|91085815|ref|XP_974770.1| PREDICTED: similar to exuperantia [Tribolium castaneum] FS919769 wdV3 ...

  8. EST Table: AV401231 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401231 e96h0406 10/09/28 100 %/169 aa ref|NP_001040343.1| peripheral-type benzodiazepine... receptor [Bombyx mori] gb|ABF51223.1| peripheral-type benzodiazepine receptor [Bombyx mori] 10/08/28

  9. EST Table: AV401871 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401871 heS00267 11/12/09 GO hit GO:0046872(metal ion binding) 10/09/28 65 %/194 aa gb|ACR61720.1| triglyce...ride lipase [Manduca sexta] 10/08/28 48 %/206 aa FBpp0147001|DgriGH13095-PA 10/08/2

  10. EST Table: AV398786 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398786 NV060281 10/09/28 96 %/126 aa gb|ACZ13341.1| actin-4 [Bursaphelenchus xylo...philus] dbj|BAI52957.1| actin [Bursaphelenchus xylophilus] 10/08/28 96 %/126 aa FBpp0278212|DpseGA24908-PA 1

  11. EST Table: AV401011 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401011 e96h0108 10/09/28 100 %/105 aa gb|ACZ13341.1| actin-4 [Bursaphelenchus xyl...ophilus] dbj|BAI52957.1| actin [Bursaphelenchus xylophilus] 10/08/28 100 %/105 aa FBpp0278212|DpseGA24908-PA

  12. EST Table: AV400057 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400057 br--0482 10/09/28 100 %/104 aa gb|ACZ13341.1| actin-4 [Bursaphelenchus xyl...ophilus] dbj|BAI52957.1| actin [Bursaphelenchus xylophilus] 10/08/28 100 %/104 aa FBpp0278212|DpseGA24908-PA

  13. EST Table: AV398858 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398858 NV060362 10/09/28 100 %/102 aa gb|ACZ13341.1| actin-4 [Bursaphelenchus xyl...ophilus] dbj|BAI52957.1| actin [Bursaphelenchus xylophilus] 10/08/28 100 %/102 aa FBpp0278212|DpseGA24908-PA

  14. EST Table: AV402944 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402944 heS30768 10/09/28 76 %/117 aa gb|ACO12033.1| 40S ribosomal protein S8 [Lepeo...phtheirus salmonis] gb|ACO12251.1| 40S ribosomal protein S8 [Lepeophtheirus salmonis] 10/08/28 67 %/122 aa

  15. Rektorers forståelse av mobbing i skolen

    DEFF Research Database (Denmark)

    Hansen, Helle Rabøl

    2008-01-01

    PÅ opfordring fra redaktionen gives en kommentar med dansk vinkel til den norske artikel 'Rektorers forståelse av mobbning i skolen'.Den omtalte artikel er at betragte som første trin i indførelse af en nordisk mobbeforståelse med undgangspunkt i Dan Olweus begrebsafgrænsning.Der savnes også en i...

  16. EST Table: AV405516 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405516 wdV10864 10/09/28 33 %/180 aa ref|XP_969213.1| PREDICTED: similar to artemis...0768|ref|XP_969213.1| PREDICTED: similar to artemis protein [Tribolium castaneum] FS920671 wdV1 ...

  17. EST Table: AV401629 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401629 e96h0968 10/09/28 70 %/161 aa ref|XP_002431431.1| Karyogamy protein KAR4, ...putative [Pediculus humanus corporis] gb|EEB18693.1| Karyogamy protein KAR4, putative [Pediculus humanus cor

  18. Forskerskoler som modeller for forskerutdanning: Progresjon og utvikling av ekspertise

    Directory of Open Access Journals (Sweden)

    Sten Ludvigsen

    2013-12-01

    Full Text Available I de senere årene har forskerskoler blitt fremhevet som en mulig løsning på ulike utfordringer innenfor forskerutdanningen. På bakgrunn av erfaringer med to forskerskoler innenfor utdanningsvitenskap, vil vi i denne artikkelen belyse to problemstillinger: Hvordan kan man organisere læringsforløpet til ph.d.-kandidatene slik at det støtter deres progresjon? Og hva må forskerutdanningen inneholde for at ph.d.-kandidatene skal utvikle tilstrekkelig ekspertise innenfor et avgrenset kunnskapsområde?For å skape progresjon, bør utdanningsprogrammet bidra til å gi struktur og angi faglig retning. Videre må utdanningselementene være relevante for avhandlingsarbeidet. I tillegg kan progresjon fremmes gjennom seminarer, der utviklingen av tekster og artikler følges opp. Argumentet om at forskerskoler bør bygge på gode forskningsmiljøer har stått sentralt i utredningen av ulike modeller for forskerutdanning i Norge. Ett aspekt som har fått økende oppmerksomhet er betydningen av å oppnå en tilstrekkelig grad av spesialisering. Uten utvikling av en slik ekspertise, vil ph.d.-kandidatene ikke kunne publisere internasjonalt og delta i forskningsfellesskap.Ved Det utdanningsvitenskapelige fakultet har man de siste 10 årene utviklet flere typer forskerskoler. I denne artikkelen tar vi utgangspunkt i den UiO-finansierte, tematisk avgrensede forskerskolen «Læring, kommunikasjon og IKT» (2004–2008 og den NFR-finansierte nasjonale forskerskolen i utdanningsvitenskap, NATED (2008–2016, hvor ph.d.-kandidatene deltar i faglige aktiviteter organisert i fire spor.Metodene som brukes for å besvare problemstillingene omfatter intervjuer, spørreskjema og refleksjoner vedrørende de to ulike forskerskolene.Resultatene viser at man kan oppnå nødvendig spesialisering, høy kvalitet, høy tilfredshet og høy relevans ved bruk av forskerskoler som virkemiddel i forskerutdanningen. Begge forskerskolene har tilrettelagt for internasjonal

  19. Minimizing ventricular pacing by a novel atrioventricular (AV) delay hysteresis algorithm in patients with intact or compromised intrinsic AV conduction and different atrial and ventricular lead locations.

    Science.gov (United States)

    Pakarinen, Sami; Toivonen, Lauri

    2013-09-01

    To investigate if an advanced AV search hysteresis (AVSH) algorithm, Ventricular Intrinsic Preference (VIP(™)), reduces the incidence of ventricular pacing (VP) in sinus node dysfunction (SND) with both intact and compromised AV conduction and with intermittent AV block regardless of the lead positions in the right atria and the ventricle. Patients were classified as having intact AV (AVi) conduction if the PR interval was ≤ 210 ms on ECG and 1:1 AV conduction during atrial pacing up to 120 bpm with PR interval ≤ 350 ms. Otherwise the AV conduction was classified as compromised (AVc). Both AVi and AVc patients were randomized to VIP ON or OFF. VIP performed an intrinsic AV conduction search every 30 s for three consecutive atrial cycles with the extension of the sensed and paced AV (SAV/PAV) delays from basic values of 150/200 ms to 300/350 ms. Extended AV intervals were allowed for three cycles when VP occurred before returning to basic AV delays. The primary end-point was %VP at 12 months. Among 389 patients, 30.1% had intact and 69.9% had compromised AV conduction. The mean %VP at 12 months was 9.6% by VIP compared to 51.8% with standard AV settings in patients with AVi (P < 0.0001) and 28.0% versus 78.9% (P < 0.0001) with AVc. With VIP, excessive %VP among most used lead positions was not seen. Conversely, when VIP was off %VP was low only in patients who had leads in the RA septal-RV septal position (23.0%). VIP feature reduces VP both in patients with SND and with intermittent heart block regardless of the lead positions in the right atria and the ventricle.

  20. En obekväm sanning eller en stor bluff? : En analys av den visuella gestaltningen av fenomenet "global uppvärmning" i filmmediet

    OpenAIRE

    Eriksson, Anton; Oretoft, Mikael

    2010-01-01

    En uppsats som kritiskt granskar och analyserar den visuella gestaltningen i två dokumentärfilmer. Huvudtemat i filmerna är global uppvärmning och konsekvenserna av detta. Uppsatsens syfte är att undersöka den visuella gestaltningen av den miljödebatt som filmerna är en del i och genom detta belysa frågor som berör visuell kultur och popularisering av vetenskap.

  1. Fra strømfordeling til pengestrøm? : Statnett fra forvaltningsbedrift til statsforetak : en konsekvensanalyse av omorganiseringen av Statnett seks år etter

    OpenAIRE

    Knudsen, Halvor Gillund

    1998-01-01

    Fra strømfordeling til pengestrøm? Statnett fra forvaltningsbedrift til statsforetak en konsekvensanalyse av omorganiseringen av Statnett seks år etter. Tema for oppgaven er konsekvenser av endrete tilknytningsformer i staten. Temaet er kommet frem gjennom tilknytning til et samarbeidsprosjekt mellom Universitetet i Oslo ved Vitenskapsbutikken og Arbeids- og administrasjonsdepartementet. Årsaken til at man ønsker å se på slike forhold er den stadige kritikken mot en etter sigende alt for...

  2. En påle genom Dracula-filmernas hjärta? : En komparativ analys av adaptationer av Bram Stokers Dracula (1897) från åren 2000-2014

    OpenAIRE

    Bahrman, Alexander

    2016-01-01

    En komparativ analys av adaptationer av Bram Stokers Dracula (1897) från åren 2000-2014 med syfte att undersöka resultatet av nästan 100 år av adaptationer och ett mål för att verkställa om en filmkanon har skapats kring karaktären.

  3. En påle genom Dracula-filmernas hjärta? : En komparativ analys av adaptationer av Bram Stokers Dracula (1897) från åren 2000-2014

    OpenAIRE

    Bahrman, Alexander

    2016-01-01

    En komparativ analys av adaptationer av Bram Stokers Dracula (1897) från åren 2000-2014 med syfte att undersöka resultatet av nästan 100 år av adaptationer och ett mål för att verkställa om en filmkanon har skapats kring karaktären.

  4. Analysis of AVS-penogram in 3000 impotent patients

    Energy Technology Data Exchange (ETDEWEB)

    Minn, Young Guy; Choi, Hyung Ki [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1994-09-01

    Audio visual simulation penogram(AVS-penogram) is used as a screening method for evaluation of erectile dysfunction. In this study, 3000 patients were evaluated from Dec. 1986 to Dec. 1992 and finally diagnosed by comprehensive diagnostic methods. The patients were classified according to AVS-penogram curves into 4 types and correlated with the patient`s final diagnosis. For 800 patients who were evaluated with combined AVS-penogram and NPT monitoring, compatibility ratios between psychogenic and organic group were compared. After correlating each of the curve type with the patients and comparing erotic and nocturnal erection, following results were obtained. 1. The mean age of the patients was 41.25 years. 2. The overall rate of final diagnosis were 57.5%, 14.3%, 8.9% in psychogenic, arteriogenic and veogenic impotence. 3. Type I group was proved as psychogenic impotence in 87% of the patients. 4. Type IIA group was proved as organic impotence in 79% of the patients and most of them had neurogenic and arteriogenic impotence. 5. Type IIB group was proved as psychogenic impotence in 51%, organic impotence in 49% of the patients. Among the organic impotence group, most of them had arteriogenic, venogenic and neurogenic impotence. The difference of both group was statically insignificant. 6. Type IIC group was proved as psychogenic impotence in 39%, organic impotence in 61% of the patients. Further studies are needed for sub-classification of both groups. 7. The compatibility ratio of the AVS-penogram and NPTM was 85.9% in psychogenic group with normal finding in both tests and 44.7% in organic group with abnormal finding in both tests. In conclusion, AVS-penogram is a primary diagnostic method in screening impotent patients and type I is regarded as psychogenic impotence. In Type IIB and IIC, sub-classification of curve type is needed for differential diagnosis between psychogenic and organic impotence. (Abstract Truncated)

  5. Forbuden frukt smaker best en studie av nordmenns spise- og drikkemønster av sjokolade, søtsaker, salt snacks, sukkerholdige leskedrikker og lignende

    OpenAIRE

    Bugge, Annechen Bahr

    2010-01-01

    Denne studien er en del av forskningsprosjektene Young Food og HealthMeal. Begge prosjektene er finansiert av Norges forskningsråd. Temaet i rapporten er nordmenns spise- og drikkemønster av en type mat og drikke som helsemyndighetene ønsker at befolkningen skal redusere forbruket sitt av – slik som sjokolade, søtsaker, søte bakervarer, salt snacks og sukkerholdige drikker. Selv om forbruksutviklingen de seneste par årene har vist positive tendenser, har altså nordmenn fremdeles et langt høye...

  6. En handlingsmodell för förebyggandet av psykologiska komplikationer hos kvinnor som genomgår avbrytande av havandeskap

    OpenAIRE

    2014-01-01

    Abstrakt Utvecklingsarbetet ”En handlingsmodell för förebyggandet av psykologiska komplikationer hos kvinnor som genomgår avbrytande av havandeskap”, är en fortsättning på examensarbetet ”Abort - En systematisk översikt av kvinnornas upplevelser och av vårdpersonalens betydelse” som publicerades och presenterades våren 2013. Syftet med utvecklingsarbetet är att producera en handlingsmodell för hälsovårdspersonalen. Handlingsmodellen kommer att ge råd och tips på hur man som hälsovårdspe...

  7. Cloning and characterization of a novel apolipoprotein gene, apolipoprotein AV, in tree shrews.

    Science.gov (United States)

    Li, Guoping; Luo, Huairong; Sun, Guotao; Wu, Guisheng; Wu, Gang; Wang, Yan; Man, Yong; Wang, Shu; Li, Jian; Chen, Baosheng

    2013-09-01

    Apolipoprotein AV (apoAV) modulates plasma triglyceride levels, which is an independent risk factor for cardiovascular disease. ApoAV is also involved in atherosclerosis lesion formation. In order to systematically evaluate the apolipoprotein-related gene profile in tree shrew, a model for its insusceptibility to atherosclerosis, we performed apoAV cloning and characterization. The full-length cDNA of apoAV was identified using SMART-RACE. ApoAV cDNA sequence revealed two transcripts, 1,948 and 1,397 base pairs, due to alternative polyadenylation. These two transcripts share the same open reading frame (ORF), which encodes a 369-amino acid protein with high identity to human apoAV (75 %), including a 23-amino acid N-terminal signal peptide. ApoAV is expressed exclusively in the liver. Mature apoAV was expressed in E. coli BL21(DE3) and purified by Ni-chelated resin. Lipoprotein lipase activity was significantly stimulated by this recombinant protein. The full-length ORF of apoAV was cloned into pDsRed-monomer-N1 vector with a red fluorescent protein tag and was primarily localized in cytoplasm of hepG2 cells. The successful cloning, expression and localization of apoAV in tree shrew has laid down the foundation for further investigation on its structure and functions.

  8. Kartlegging av læreres kunnskap er ikke enkelt

    Directory of Open Access Journals (Sweden)

    Reidar Mosvold

    2015-05-01

    Full Text Available Læreres kunnskap har betydning for elevenes læring, og som et ledd i prosessen for styrking av kvaliteten i norsk skole har politikere i senere tid ytret ønske om å kartlegge læreres kunnskap. Kartlegging av læreres kunnskap er imidlertid ikke enkelt, og i denne artikkelen undersøker vi hvordan de samme lærernes undervisningskunnskap i matematikk kan se ut til å være forskjellig når den måles ved hjelp av oppgaver med ulikt format. Deltakerne i studien var 30 praktiserende lærere, og instrumentet besto av 28 flervalgsoppgaver med tilhørende åpne oppgaver. Sammenhengen mellom lærernes kunnskap slik den måles ved hjelp av flervalgsoppgavene og de åpne oppgavene er ikke alltid klar. Det er derfor nødvendig å tilnærme seg måling av læreres undervisnings-kunnskap i matematikk med stor varsomhet. Nærmere undersøkelser er nødvendig for å finne ut hva slags type kunnskap som kan måles med ulike typer oppgaver og hva slags måleinstrumenter som kan brukes til hvilke formål.Nøkkelord: Undervisningskunnskap i matematikk, kartlegging, måleinstrument-er, flervalgsoppgaver AbstractTeachers’ knowledge influence students’ learning, and as part of their attempts to increase the quality of Norwegian schools, politicians have announced their intentions to measure the knowledge of teachers. Measuring teacher knowledge is not straightforward, however, and this article investigates how a group of mathematics teachers’ knowledge might appear different when measured by items of different formats. The participants in this study were 30 in-service teachers, and the instrument consisted of 28 multiple-choice items with associated open-response items. The connection between the teachers’ know-ledge as measured by multiple-choice items and open-response items is not always straightforward. As an implication, it is necessary to approach measure-ment of teachers’ mathematical knowledge for teaching with great care. Further studies are

  9. Avsluttende studentevaluering i Bachelorutdanningen : avgangsstudenters vurdering av undervisning og utdanning ved Politihøgskolen, Kull 05–08

    OpenAIRE

    Hove, Kjersti

    2010-01-01

    Denne rapporten inneholder en oppsummering og enkle analyser av studenters evaluering av utdanningen ved slutten av studiet. Evalueringen bygger delvis på spørsmål hentet fra StudData, og en tilsvarende rapport fra HiO ( Hatlevik, Ida Katrine Riksaasen: Avgangsstudenten – Studentenes vurdering av undervisning, praksis, studieforhold, tilegnet kompetanse, studieatferd og fremtidig utdanning. HiO-notat nr 2 -2009) hvor analyser av flere studentgruppers evalueringer er sammenstilt. Rapporten fra...

  10. Wirevagn : Utvecklingen av en utrullare för hisslinor

    OpenAIRE

    Rehnsfeldt, Patrik

    2014-01-01

    Kandidatexamensarbetet som utgick från en förfrågan från företaget Irongrip AB hade som mål att utveckla ett produktförslag på en utlindare av stålvajer för hissmontörer. Irongrip AB som tillverkar och säljer verktyg för hantering av stålvajer hade uppmärksammat att en efterfrågan på en sådan produkt fanns på marknaden då befintliga lösningar var bristfälliga och inte hade alla de funktioner som är önskvärda hos en sådan produkt.Arbetet inleddes med en förstudie där studiebesök vid lindragnin...

  11. EST Table: AV400562 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400562 br--1452X 10/09/28 59 %/160 aa ref|XP_001843664.1| neuroendocrine protein ...7b2 [Culex quinquefasciatus] gb|EDS33875.1| neuroendocrine protein 7b2 [Culex quinquefasciatus] 10/08/28 41 ...47 %/159 aa gi|91081787|ref|XP_973692.1| PREDICTED: similar to neuroendocrine protein 7b2 [Tribolium castaneum] BP115163 br-- ...

  12. EST Table: AV400244 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400244 br--1010 10/09/28 53 %/100 aa ref|XP_001654557.1| neuroendocrine protein 7...b2 [Aedes aegypti] gb|EAT46754.1| neuroendocrine protein 7b2 [Aedes aegypti] 10/08/28 n.h 10/08/27 n.h 10/09/10 low homology 10/09/10 n.h 10/09/10 n.h BP115163 br-- ...

  13. EST Table: AV398380 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398380 NV021745 10/09/28 70 %/209 aa ref|XP_001648944.1| dendritic cell protein [...hort=eIF3m gb|EAT44291.1| dendritic cell protein [Aedes aegypti] 10/08/28 66 %/206 aa FBpp0276999|DpseGA2097...P_968265.1| PREDICTED: similar to dendritic cell protein [Tribolium castaneum] FS916297 NV02 ...

  14. EST Table: AV399091 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399091 NV060650 10/09/28 71 %/209 aa ref|XP_001648944.1| dendritic cell protein [...hort=eIF3m gb|EAT44291.1| dendritic cell protein [Aedes aegypti] 10/08/28 66 %/206 aa FBpp0276999|DpseGA2097...P_968265.1| PREDICTED: similar to dendritic cell protein [Tribolium castaneum] FS916297 NV06 ...

  15. EST Table: AV399252 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399252 NV060970 10/09/28 62 %/104 aa ref|NP_001119705.1| transformer 2 isoform A ...[Bombyx mori] gb|AAT42220.2| transformer-2 protein A [Bombyx mori] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 low homology 10/09/10 low homology NM_001126237 NV06 ...

  16. EST Table: AV400450 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400450 br--1258 10/09/28 98 %/184 aa ref|NP_001040380.1| F-box only protein 21 [B...ombyx mori] gb|ABF51283.1| F-box only protein 21 [Bombyx mori] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h FS910521 br-- ...

  17. EST Table: AV402699 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402699 heS30352 10/09/28 96 %/136 aa ref|NP_001128387.1| scavenger receptor type ...C [Bombyx mori] dbj|BAG70411.1| scavenger receptor type C [Bombyx mori] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h FS765704 heS3 ...

  18. EST Table: AV402471 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402471 heS01053 10/09/28 100 %/233 aa ref|NP_001128387.1| scavenger receptor type... C [Bombyx mori] dbj|BAG70411.1| scavenger receptor type C [Bombyx mori] 10/08/28 35 %/207 aa FBpp0123883|Da...12043.1| PREDICTED: similar to scavenger receptor SR-C-like protein [Tribolium castaneum] FS765704 heS0 ...

  19. EST Table: AV405279 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405279 wdV10395 10/09/28 66 %/172 aa ref|XP_002062541.1| GK17594 [Drosophila will.../27 33 %/169 aa T21E12.4#CE23997#WBGene00000962#locus:dhc-1#dynein heavy chain#status:Partially_confirmed#Un...17|ref|XP_975584.1| PREDICTED: similar to dynein heavy chain [Tribolium castaneum] DC550560 wdV1 ...

  20. EST Table: AV404429 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404429 pg--0968X 10/09/28 88 %/186 aa ref|NP_001116822.1| kinesin heavy chain [Bo...5-PA 10/08/27 44 %/109 aa R05D3.7#CE26945#WBGene00006840#locus:unc-116#Kinesin heavy chain#status:Confirmed#...56|ref|XP_973415.2| PREDICTED: similar to Kinesin heavy chain CG7765-PA [Tribolium castaneum] FS934567 pg-- ...

  1. EST Table: AV402302 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402302 heS00852 10/09/28 98 %/239 aa ref|NP_001128387.1| scavenger receptor type ...C [Bombyx mori] dbj|BAG70411.1| scavenger receptor type C [Bombyx mori] 10/08/28 31 %/247 aa FBpp0259854|Dya...043.1| PREDICTED: similar to scavenger receptor SR-C-like protein [Tribolium castaneum] FS765704 heS0 ...

  2. EST Table: AV398139 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398139 NV021172 10/09/28 46 %/243 aa ref|XP_001661901.1| DNA mismatch repair protein muts... [Aedes aegypti] gb|EAT36110.1| DNA mismatch repair protein muts [Aedes aegypti] 10/08/28 43 %/238 a...el|GB10970-PA 10/09/10 42 %/247 aa gi|91082687|ref|XP_971522.1| PREDICTED: similar to DNA mismatch repair protein muts [Tribolium castaneum] BY922838 NV02 ...

  3. Effekter av endringer i finansieringsansvaret for TNF-hemmere

    OpenAIRE

    Hagen, Terje P.; Bjarkum, Irina; Hobbel, Silje; Orderdalen, Karianne

    2009-01-01

    Tumor necreosis factor alfa (TNF-a) - hemmere, og andre biologiske inflammatoriske modifiserende legemidler blir brukt innen reumatologi, gastroenterologi og dermatologi. I denne rapporten analyseres effekter av endringer i finansieringssystemet for seks legemidler som faller innenfor disse legemiddelgruppene og som langt på vei er substitutter: Humira, Enbrel og Remicade som er TNF-hemmere, Raptiva og Orencia som er registrert under kategorien selektive immunsuppressiver og MabThera som er r...

  4. EST Table: AV401797 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401797 heS00172 10/09/28 94 %/235 aa ref|NP_001036831.1| saposin-related [Bombyx ...9/10 low homology 10/09/10 low homology 10/09/10 41 %/191 aa gi|91077504|ref|XP_966852.1| PREDICTED: similar to saposin isoform 1 [Tribolium castaneum] FS791050 heS0 ...

  5. EST Table: AV401078 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401078 e96h0200 10/09/28 42 %/112 aa ref|XP_001648294.1| Juvenile hormone-inducib...le protein, putative [Aedes aegypti] gb|EAT44634.1| Juvenile hormone-inducible protein, putative [Aedes aegy... 10/09/10 36 %/106 aa gi|91080979|ref|XP_974925.1| PREDICTED: similar to Juvenile hormone-inducible protein, putative [Tribolium castaneum] FS808716 e96h ...

  6. Kritik av riskbegreppet - berättigad eller ej?

    DEFF Research Database (Denmark)

    Nexøe, Jørgen; Halvorsen, Peder; Kristiansen, Ivar Sønbø

    2009-01-01

    skull". Trots stora framsteg inom medicinen finns det skäl att stanna upp och fråga: Vad håller vi på med egentligen? Redaktörerna och författarna till denna bok lyfter fram behovet av att reflektera över medicinsk praktik och att kritiskt granska de teorier som ligger till grund för vad vi gör. I boken...

  7. Inkludering av ungdom med minoritetsbakgrunn i NIF-organisert idrett

    Directory of Open Access Journals (Sweden)

    Mari Kristin Sisjord

    2014-10-01

    Full Text Available Norges Idrettsforbund og Olympiske og Paralympiske Komité (NIF har en uttalt målsetting om en åpen og inkluderende idrett. Hvordan kommer dette til uttrykk i den praktiske virksomheten? Denne artikkelen, som bygger på data fra en undersøkelse om likestilling og mangfold i den organiserte idretten (NIF, retter søkelyset mot arbeid med inklusjon av ungdom med minoritetsbakgrunn på ulike organisasjonsnivå i NIF: særforbund, idrettskretser og idrettslag. Datamaterialet er kvalitative intervju med representanter fra ulike organisasjonsnivå. Resultatene viser at NIFs overordnede politikk i varierende grad nedfelles i særforbundenes virksomhet, i idrettskretsene og i idrettslagene. Av særforbundene skiller Fotballforbundet og Klatreforbundet seg ut som aktive pådrivere i arbeidet med inkludering. Mange idrettslag oppfattet slike spørsmål som lite aktuelle i sitt rekrutteringsområde. Representanter for lag som hadde erfaring med inkludering og rekruttering av minoritetsungdom, tilkjennega varierte erfaringer og synspunkter.

  8. Toward robust AV conferencing on next-generation networks

    Science.gov (United States)

    Liu, Haining; Cheng, Liang; El Zarki, Magda

    2005-01-01

    In order to enable a truly pervasive computing environment, next generation networks (including B3G and 4G) will merge the broadband wireless and wireline networking infrastructure. However, due to the tremendous complexity in administration and the unreliability of the wireless channel, provision of hard-guarantees for services on such networks will not happen in the foreseeable future. This consequently makes it particularly challenging to offer viable AV conferencing services due to their stringent synchronization, delay and data fidelity requirements. We propose in this paper a robust application-level solution for wireless mobile AV conferencing on B3G/4G networks. Expecting no special treatment from the network, we apply a novel adaptive delay and synchronization control mechanism to maintain the synchronization and reduce the latency as much as possible. We also employ a robust video coding technique that has better error-resilience capability. We investigate the performance of the proposed solution through simulations using a three-state hidden Markov chain as the generic end-to-end transport channel model. The results show that our scheme yields tight synchronization performance, relatively low end-to-end latency and satisfactory presentation quality. The scheme successfully provides a fairly robust AV conferencing service.

  9. Synliggörande av tyst kunskap i gymnasial yrkesutbildning

    Directory of Open Access Journals (Sweden)

    Mats Lundgren

    2012-04-01

    Full Text Available Yrkesutbildning skiljer sig från andra former av gymnasieutbildning genom att den innehåller praktisk kunskap som kan vara svårfångad, ofta benämnd som tyst kunskap. Tyst kunskap utgör en förutsättning för att yrkeselever ska kunna tillägna sig ett yrkes kultur, praxis och kunskapstradition. Med utgångspunkt i yrkeslärares berättelser beskrivs i artikeln med stöd av tre definierade kompetensområden, 1 yrkesfältet, 2 handlag i yrket samt 3 interaktion och social kompetens, hur yrkeslärare arbetar för att möjliggöra att ett yrkes tysta kunskaper kan läras i en skolmiljö. Resultatet visar betydelsen av att yrkeslärares tysta kunskaper blottläggs för eleverna genom att de i sin undervisning, berättar, visar och ställer frågor. Det är i dessa möten som den tysta kunskapens betydelse i yrkesutövning kan bli synlig för eleverna.

  10. Bruk av ordlæringsstrategier blant norske tyskelever

    Directory of Open Access Journals (Sweden)

    Anna Gausland

    2011-05-01

    Full Text Available Interessen for forskning på læringsstrategier er økende i Norge. Det finnes likevel ennå ingen publiserte empiriske studier som fokuserer på læringsstrategier i andre fremmedspråk. Hensikten med den foreliggende artikkelen er å gi et første innblikk i 54 norske tyskelevers bruk av ordlæringsstrategier. Studien er gjennomført som en skriftlig spørreundersøkelse hvor elevene beskrev hvordan de hadde arbeidet for å lære en liste med tysk-norske ordpar. Samlet sett ser vi at elevene behersker ordlæringsstrategier av ulik art, men at noen få strategier er hyppig forekomne hos de fleste elevene. Det viser seg at elevene i hovedsak bruker ordlæringsstrategier som inneholder element av repetisjon, altså strategier som krever lite kognitiv bearbeiding. Dernest blir metakognitive strategier som organiserer og planlegger læringen mest brukt. Artikkelens siste del sammenligner funn fra undersøkelsen med internasjonale studier og skisserer videre forskningsbehov innen feltet.

  11. Särhållning av gruvavfall med avseende på syrabildande potential : en studie av svenska sulfidmalmsgruvor

    OpenAIRE

    Sellin, Jessica

    2016-01-01

    Gruvavfall utgör över 80 % av den totala mängden avfall i Sverige. Avfallet från gruvindustrin kan påverka våra ekosystem negativt om tungmetaller och försurande ämnen lämnar materialet. I Sverige utgör avfall från sulfidmalmer störst risk för miljön eftersom det innehåller mineral som genom kontakt med luft vittrar och producerar surt och metallhaltigt lakvatten. Efterbehandling av dessa material är viktigt för att förhindra läckage till omgivningen. Eftersom efterbehandli...

  12. Mellom bibel og menneskerettigheter : en studie av Vårt Land og Dagens vinkling av Palestina/Israel-konflikten

    OpenAIRE

    2005-01-01

    Sammendrag Tittel: Israel og Palestina mellom bibel og menneskerettigheter. - Vinkling av Palestina/Israelkonflikten i avisene Vårt Land og Dagens lederartikler Spørsmålsstilling: Hvilke holdninger til Palestina/Israel-konflikten reflekterer lederartikler i de to kristne dagsavisene Dagen og Vårt Land? Er det mulig å spore en endring i vinklingen over tid? Hvordan kan disse lederartiklene gi oss innblikk i en debatt om Palestina/Israel-konflikten blant kristne i Norge? Metode...

  13. Å LESE MEDIETEKSTER PÅ IPADEN. Observasjoner av en medievant fireårings bruk av iPad i hjemmet

    Directory of Open Access Journals (Sweden)

    Henriette Jæger

    2016-06-01

    Full Text Available Artikkelen tar utgangspunkt i tre observasjoner av en gutt på fire år som bruker iPad på fritiden. Den søker å synliggjøre de strategiene dette barnet velger for å skaffe seg tilgang til ulike medietekster, hvordan han leser og tolker dem, og den undersøker eventuelle spor av kritisk refleksjon hos barnet over disse tekstene. Teoretisk sett hviler den på en forståelse av mediekompetanse som en parallell prosess til utvikling av lese- og skrivekyndighet (literacy, og medieopplevelsene omtales som viktige møter med tekst. Artikkelen undersøker hvordan å lese medietekster kan legge et grunnlag for å utvikle en utvidet tekstkompetanse eller mediekompetanse. Konklusjonen legger vekt på motivasjon og lekenhet som barns primære inngang til medietekstene og som et grunnlag for å utvikle mediekompetanse (media literacy. The article is based on three observations of a four-year-old boy who uses the iPad in his spare time. It demonstrates the strategies that this boy applies to gain access to various media texts, and how he reads and interprets them. It also seeks to examine traces of critical reflection that he may demonstrate in relation to these texts. Theoretically, it rests on an understanding of media literacy as a parallel process to the development of literacy, and it seeks to demonstrate how reading media texts can create an important starting point for the development of media literacy. The Kindergarten practitioner’s role and opportunities to develop children's media literacy within a kindergarten context is a focus towards the end of the article. In conclusion, I argue that motivation and playfulness are important approaches to media texts and create an important foundation for the development of media literacy

  14. Har forhold under svangerskapet betydning for utviklingen av otitis media?

    Directory of Open Access Journals (Sweden)

    Kari J. Kværner

    2009-10-01

    Full Text Available  SAMMENDRAGMed utgangspunkt i data fra et populasjonsbasert utvalg av 7 992 norske tvillinger ble sammenhengenmellom gjentatte ørebetennelser og forhold under svangerskapet studert. Som parametre på intrauterinevekstforhold ble informasjon om fødselsvekt og gestasjonsalder fra Medisinsk Fødselsregister benyttet.Informasjon om rapporterte øreinfeksjoner ble basert på spørreskjemaer som ble besvart av tvillingene ialderen 18–25 år. Først studerte vi individuelle effekter av fødselsvekt og gestasjonsalder på forekomsten avotitis media (OM. Rapportert fødselsvekt hos individer med OM var signifikant lavere enn hos kontrollene,med en gjennomsnittsforskjell på 86 gram. Hos de som rapporterte OM var gjennomsnittlig gestasjonsalder3 dager kortere enn i kontrollmaterialet. Deretter beregnet vi effekten av fødselsvekt ved bruk av co-twinkontroll metoden. Blant monozygote (MZ tvillingpar gjenspeiler fødselsvektforskjeller innenfor tvillingparetintra-uterine miljøforskjeller. Blant dizygote (DZ par kan vektforskjellen være både genetiske ogmiljøbetinget. Ved sammenligning av fødselsvektforskjeller innenfor tvillingparene er tvillingen som ikkehar gjentatte øreinfeksjoner en matchet kontroll blant OM diskordante par. Resultatene antyder at lavfødselsvekt er en risikofaktor for otitis media. Gjennomsnittlig vektforskjell blant par diskordante for OMvar 48 gram, og den av tvillingen som ikke rapporterte OM hadde signifikant høyere fødselsvekt.Kværner KJ, Tambs K, Harris J, Magnus P. Do pregnancy-related factors influence the development ofotitis media? Nor J Epidemiol 1997; 7 (1: 55-58.  ENGLISH SUMMARYThe association between intrauterine growth, as measured by weight and gestational age, and the occurrenceof recurrent ear infections was studied using data from a population based sample of 7992 Norwegian twins.Perinatal measures were collected from the Medical Birth Registry, and reports of recurrent ear infectionswere based on

  15. Overvåking av norsk kosthold - metoder og resultater

    Directory of Open Access Journals (Sweden)

    Lars Johansson

    2009-11-01

    Full Text Available  SAMMENDRAGKunnskap om endringer i matvarenes sammensetning og befolkningens kosthold er grunnleggende for utformingog oppfølging av en helsefremmende mat- og ernæringspolitikk. Dagens system for overvåking avkostholdet bygger først og fremst på en matvaredatabase og opplysninger om forbruket av matvarer fra tretyper datasett; matforsyningsstatistikk, forbruksundersøkelser og kostholdsundersøkelser. Det avgis årlig envurdering av utviklingen i norsk kosthold i forhold til mat- og ernæringspolitiske målsettinger. Det norskesystemet for overvåking av kostholdet er blitt betydelig styrket i løpet av 1990-årene ved innføringen avregelmessige kostholdsundersøkelser blant landsrepresentative utvalg av befolkningen i ulike aldersgrupperog økte ressurser til analyse av næringsinnholdet i matvarer. Det er imidlertid beskjedent sammenlignet meddatainnsamlingen i land som USA og Storbritannia. Det norske overvåkingssystemet er forsatt i enutviklingsfase, og det må justeres og kompletteres etter hvert. Dessuten vil det arbeides med å kvalitetssikredatainnsamlingen.Johansson L. Surveillance of the diet in Norway – methods and results.Nor J Epidemiol ENGLISH SUMMARYKnowledge about changes in composition and intake of foods, as well as changes in the prevalence of dietrelatedhealth problems is fundamental for an effective food and nutrition policy. The National Council onNutrition and Physical Activity is responsible for evaluating changes in the diet. Annually the Council publishesreports about trends in the Norwegian diet, and suggests measures to improve the diet. The Council, incooperation with the National Food Authority and the Institute for Nutrition Research, University of Oslo,has established a national food and dietary surveillance system. This system is mainly based on a fooddatabase and food consumption data. The food consumption data include three principally different datasets: food supplies, household consumption surveys

  16. Diffusjon av influensa i Norge under spanskesyken 1918-19

    Directory of Open Access Journals (Sweden)

    Svenn-Erik Mamelund

    2009-10-01

    Full Text Available  SAMMENDRAGSpanskesyken var en verdensomspennende influensaepidemi som tok livet av minst 30 millioner mennesker,de fleste i løpet av noen få høstmåneder i 1918, men den strakk seg også inn i 1919. En siste svakbølge fant sted vinteren 1920. De første sporadiske tilfellene av spanskesyken i Norge oppsto i militærleirei begynnelsen av april 1918. Over tre måneder senere kom de første spredte tilfellene av det som skullevise seg å være starten på en ny stor influensapandemi. De første av disse oppsto i Kristiania, mest sannsynligetter smitteimport fra Skottland over Nordsjøen. Sommeren 1918 spredte spanskesyken seg raskt frasør til nord, fra kysten til innlandet innover fjordene, og fra de største byene og industrisentra via mindrebyer og andre tettsteder til landsbygda langs de viktigste ferdselsveiene. Da spanskesyken blusset opp igjenhøsten 1918 spredte den seg langsommere og trolig fra nord til sør, fortsatt fra kysten innover fjordene,men spredning fra byene langs kommunikasjonsveiene var mindre fremtredende. Vinteren 1918/19opptrådte influensaen spredt og sporadisk, tilsynelatende uten bestemt spredningsmønster og rekkefølge.Jernbanen, hurtigruten og automobilrutene spilte trolig en viktig rolle i spredningsprosessen, spesielt andrehalvår 1918. Ulik sosial interaksjon, for eksempel et marked som samlet store folkemengder, kan bidra til åforklare eksplosive utbrudd av influensa og diffusjon på lavt geografisk nivå.Mamelund S-E. The diffusion of influenza in Norway during the 1918-19 Pandemic.Nor J Epidemiol ENGLISH SUMMARYIn 1918-1919, a world-wide pandemic, the Spanish Influenza, swept over the entire globe, killing at least30 million people, most of them during the fall of 1918. A last, but small and less defined wave occurred inthe winter of 1920. The first sporadic cases of Spanish Influenza in Norway are known from army campsin the early days of April 1918. More than three months later, the first scattered

  17. Implementering av nye GOLD-stadier hos pasienter med KOLS i allmennpraksis

    OpenAIRE

    Storaker, Marit Aarvaag; Nerwey, Ibrahim; Kierulf-Strømme, Kirsten; Tengesdal, Randi Hauge; Wyller, Nora Guttormsgaard; Skårn, Elling Skeide; Cagrici, Ufuk

    2014-01-01

    Bakgrunn: GOLD (Global Initiative for Chronic Obstructive Lung Disease) lanserte i 2011 nye kriterier for gradering av alvorlighetsgrad av KOLS, der man i tillegg til FEV1 ved spirometri også tar hensyn til antall eksaserbasjoner og pasientens symptombyrde. Mange pasienter havner i en lavere risikogruppe etter den nye inndelingen, og er følgelig overbehandlet med f.eks. inhalasjonssteroider. Andre kan identifiseres tidligere som høyrisikopasienter for hyppige eksaserbasjoner og raskt tap av l...

  18. STUDY ON EFFICIENT COMPUTATION AND PERFORMANCE OF AV-BASED REDUCED-RANK FILTERING

    Institute of Scientific and Technical Information of China (English)

    Xu Bin; Yang Chenyang; Mao Shiyi

    2005-01-01

    In this paper, the complexity and performance of the Auxiliary Vector (AV) based reduced-rank filtering are addressed. The AV filters presented in the previous papers have the general form of the sum of the signature vector of the desired signal and a set of weighted AVs,which can be classified as three categories according to the orthogonality of their AVs and the optimality of the weight coefficients of the AVs. The AV filter with orthogonal AVs and optimal weight coefficients has the best performance, but requires considerable computational complexity and suffers from the numerical unstable operation. In order to reduce its computational load while keeping the superior performance, several low complexity algorithms are proposed to efficiently calculate the AVs and their weight coefficients. The diagonal loading technique is also introduced to solve the numerical unstability problem without complexity increase. The performance of the three types of AV filters is also compared through their application to Direct Sequence Code Division Multiple Access (DS-CDM A) systems for interference suppression.

  19. Utveckling av mjukglassomrörare : En studie för ökad luftinblandning

    OpenAIRE

    2010-01-01

    Den här rapporten är ämnad som underlag till ett fortsatt utvecklingsarbete av en omrörare för mjukglassmaskiner. I en mjukglassmaskin kyls glassen under kontinuerlig omrörning, på så vis blandas luft in och ger glassen fluffighet. Omrörarens huvudfunktioner är luftinblandning, avskrapning av iskristaller längs innerhöljet av kylcylindern samt utmatning av mjukglass mot öppningsdörr. Med en ny utformning ska omröraren bidra till en mer kostnadseffektiv mjukglass-maskin samt ge en godare glass...

  20. Handlingsplan för ägare av transformatorer med korrosiv olja

    OpenAIRE

    Weidemann, Stephan

    2016-01-01

    Sedan början av 2000-talet har ett stort antal transformatorer havererat av en initialt, okänd anledning. Problemet visade sig vara att en antioxidationstillsats i oljan, dibensyl disulfid (DBDS), reagerat med kopparen i lindningarna och vilket gett upphov till avlagringar på isolationspapperet i form av kopparsulfid. Kopparsulfiden i sin tur försvagade papperets isolationsförmåga och allvarliga driftstörningar eller driftstopp på grund av kortslutningar kan ske. Ett stort antal studier har g...

  1. NATO i krigen mot terror : En analyse av NATOs rolle i Afghanistan etter 11. september

    OpenAIRE

    Berg-Hansen, Susanne

    2007-01-01

    Denne oppgaven studerer NATOs rolle i den USA-ledede Operation Enduring Freedom i Afghanistan. Noen timer etter terrorangrepet mot USA 11. september 2001, påkalte NATO for første gang i historien sin grunnpilar, Artikkel 5 i NATO-traktaten, som fastslår at et angrep på ett av medlemmene er et angrep på alle NATO-landene. Til tross for alliansens påkallelse av Artikkel 5, ble NATO stående på sidelinjen i hovedfasen av "Operation Enduring Freedom". USA valgte å gjennomføre mesteparten av kamp...

  2. Tibetanisering : religiøs og etnisk utdanning av tibetanske flyktningbarn i Nord-India

    OpenAIRE

    Lind, Trude

    2007-01-01

    Religiøs sosialisering og nasjonal identitet blant tibetanske flyktningbarn i Nord-India er tema for denne oppgaven. Mye av materialet bygger på feltarbeidet jeg utførte ved Tibetan Children’s Village (TCV) Dharamsala høsten 2003. TCV regnes som en av de viktigste sekulære utdanningsinstitusjonene for tibetanerne i eksil. Siden den kinesiske invasjonen av Tibet i 1950 har mer en 100 000 tibetanere flyktet over fjellene i Himalaya til India, Nepal og Bhutan. Et av Dalai Lamas og eksilregje...

  3. Prevalence of E/A wave fusion and A wave truncation in DDD pacemaker patients with complete AV block under nominal AV intervals.

    Directory of Open Access Journals (Sweden)

    Wolfram C Poller

    Full Text Available Optimization of the AV-interval (AVI in DDD pacemakers improves cardiac hemodynamics and reduces pacemaker syndromes. Manual optimization is typically not performed in clinical routine. In the present study we analyze the prevalence of E/A wave fusion and A wave truncation under resting conditions in 160 patients with complete AV block (AVB under the pre-programmed AVI. We manually optimized sub-optimal AVI.We analyzed 160 pacemaker patients with complete AVB, both in sinus rhythm (AV-sense; n = 129 and under atrial pacing (AV-pace; n = 31. Using Doppler analyses of the transmitral inflow we classified the nominal AVI as: a normal, b too long (E/A wave fusion or c too short (A wave truncation. In patients with a sub-optimal AVI, we performed manual optimization according to the recommendations of the American Society of Echocardiography.All AVB patients with atrial pacing exhibited a normal transmitral inflow under the nominal AV-pace intervals (100%. In contrast, 25 AVB patients in sinus rhythm showed E/A wave fusion under the pre-programmed AV-sense intervals (19.4%; 95% confidence interval (CI: 12.6-26.2%. A wave truncations were not observed in any patient. All patients with a complete E/A wave fusion achieved a normal transmitral inflow after AV-sense interval reduction (mean optimized AVI: 79.4 ± 13.6 ms.Given the rate of 19.4% (CI 12.6-26.2% of patients with a too long nominal AV-sense interval, automatic algorithms may prove useful in improving cardiac hemodynamics, especially in the subgroup of atrially triggered pacemaker patients with AV node diseases.

  4. Prevalence of E/A wave fusion and A wave truncation in DDD pacemaker patients with complete AV block under nominal AV intervals.

    Science.gov (United States)

    Poller, Wolfram C; Dreger, Henryk; Schwerg, Marius; Melzer, Christoph

    2015-01-01

    Optimization of the AV-interval (AVI) in DDD pacemakers improves cardiac hemodynamics and reduces pacemaker syndromes. Manual optimization is typically not performed in clinical routine. In the present study we analyze the prevalence of E/A wave fusion and A wave truncation under resting conditions in 160 patients with complete AV block (AVB) under the pre-programmed AVI. We manually optimized sub-optimal AVI. We analyzed 160 pacemaker patients with complete AVB, both in sinus rhythm (AV-sense; n = 129) and under atrial pacing (AV-pace; n = 31). Using Doppler analyses of the transmitral inflow we classified the nominal AVI as: a) normal, b) too long (E/A wave fusion) or c) too short (A wave truncation). In patients with a sub-optimal AVI, we performed manual optimization according to the recommendations of the American Society of Echocardiography. All AVB patients with atrial pacing exhibited a normal transmitral inflow under the nominal AV-pace intervals (100%). In contrast, 25 AVB patients in sinus rhythm showed E/A wave fusion under the pre-programmed AV-sense intervals (19.4%; 95% confidence interval (CI): 12.6-26.2%). A wave truncations were not observed in any patient. All patients with a complete E/A wave fusion achieved a normal transmitral inflow after AV-sense interval reduction (mean optimized AVI: 79.4 ± 13.6 ms). Given the rate of 19.4% (CI 12.6-26.2%) of patients with a too long nominal AV-sense interval, automatic algorithms may prove useful in improving cardiac hemodynamics, especially in the subgroup of atrially triggered pacemaker patients with AV node diseases.

  5. Overvåking av vassdrag i Ringsaker - Undersøkelser av innsjøer og bekker i 2012

    OpenAIRE

    Løvik, J.; Brettum, Pål

    2013-01-01

    Rapporten omhandler vannkvalitet og miljøtilstand i fire innsjøer og fem mindre vassdrag i Ringsaker kommune i 2012. Hovedvekten er lagt på effekter av næringsstoffer, dvs. overgjødsling. Basert på mengder og sammensetning av planteplankton, sammensetning av dyreplankton samt fysisk/kjemiske støtteparametre vurderes innsjøenes økologiske tilstand mht. overgjødsling som følger: Ljøsvatnet: moderat, Sjusjøen: moderat, men nær grensen til god og Næra og Botsenden: god. Vurderingene bør betraktes...

  6. An uncommon case of spontaneous conversion from AV re-entry tachycardia to AV nodal re-entry tachycardia in a patient with dual tachycardia.

    Science.gov (United States)

    Zeljković, Ivan; Benko, Ivica; Manola, Šime; Radeljić, Vjekoslav; Pavlović, Nikola

    2015-01-01

    We report the case of a 46-year old patient in whom an electrophysiology study (EP) was performed due to paroxysmal supraventricular tachycardia documented in 12-lead ECG. During the EP study, supraventricular tachycardia was induced easily and it corresponded to orthodromic AV reentry tachycardia (AVRT) using a concealed left free wall accessory pathway. However, during the study AVRT spontaneously and repeatedly converted to the typical slow-fast AV node reentry tachycardia (AVNRT). Both accessory and AV nodal slow pathways were ablated, due to the finding that both AVRT and AVNRT were independently inducible during the EP study.

  7. Karakterisering av vannkjemisk status i bekker og innsjøer før utbygging av E 18 Grimstad-Kristiansand

    OpenAIRE

    Hindar, A.

    2005-01-01

    Førtilstanden er kartlagt for i alt 25 bekke- og innsjølokaliteter som blir krysset av planlagt E 18 fra Grimstad til Kristiansand. Forsuring har stått i fokus fordi deler av vegstrekningen vil gå gjennom sulfidgneis, og fordi det er avdekket omfattende problemer med avrenningsvann ved utsprengning i slik berggrunn. Det har vært et mål å forsøke å skille effekten av sur nedbør fra den påvirkning ueksponert og eksponert sulfidgneis gir. Naturtilstand i kombinasjon med sur nedbør gir sulfatkons...

  8. Harmonikk for det indre øret : En studie av harmonikk som assosiasjonsgiver i Bernard Herrmanns musikk til filmen Vertigo av Alfred Hitchcock

    OpenAIRE

    Bøe, Brynjar

    2012-01-01

    I denne oppgaven tar jeg for meg Bernard Herrmanns musikk til filmen Vertigo fra 1958, regissert av Alfred Hitchcock. Jeg vil forsøke å finne sammenhenger mellom komponistens bruk av harmonikk og filmfortellingen for øvrig. Jeg vil se hvilke hvordan filmkomponisten være med på å fortelle historien i filmen, og hvilke musikalske virkemidler, med vekt på harmonikkrelaterte sådan, han/hun har til rådighet for å gjøre dette. Jeg vil se på noen av de musikalske valgene Herrmann har tatt...

  9. Sensorisk realisme i Carlos Reygadas' Post Tenebras Lux (2012) - En undersøkelse av tilskuerhenvendelsen i lys av nyere realismeteori og filmfenomenologi

    OpenAIRE

    Johnson, Silja Espolin

    2015-01-01

    I dette prosjektet undersøker den meksikanske filmregissøren Carlos Reygadas fjerde og nyeste spillefilm Post Tenebras Lux (2012) i lys av den nærværsbaserte og "fysiske" tilskueropplevelsen den produserer. Prosjektet består av fire kapitler. I det første kapittelet presenterer jeg filmen i konteksten av en tendens som har markert seg på filmfestivaler verden over de siste to tiårene som går under flere navn, blant andre: slow cinema, hybridfilm og, det begrepet jeg har funnet mest fruktbart...

  10. Artificial vision support system (AVS(2)) for improved prosthetic vision.

    Science.gov (United States)

    Fink, Wolfgang; Tarbell, Mark A

    2014-11-01

    State-of-the-art and upcoming camera-driven, implanted artificial vision systems provide only tens to hundreds of electrodes, affording only limited visual perception for blind subjects. Therefore, real time image processing is crucial to enhance and optimize this limited perception. Since tens or hundreds of pixels/electrodes allow only for a very crude approximation of the typically megapixel optical resolution of the external camera image feed, the preservation and enhancement of contrast differences and transitions, such as edges, are especially important compared to picture details such as object texture. An Artificial Vision Support System (AVS(2)) is devised that displays the captured video stream in a pixelation conforming to the dimension of the epi-retinal implant electrode array. AVS(2), using efficient image processing modules, modifies the captured video stream in real time, enhancing 'present but hidden' objects to overcome inadequacies or extremes in the camera imagery. As a result, visual prosthesis carriers may now be able to discern such objects in their 'field-of-view', thus enabling mobility in environments that would otherwise be too hazardous to navigate. The image processing modules can be engaged repeatedly in a user-defined order, which is a unique capability. AVS(2) is directly applicable to any artificial vision system that is based on an imaging modality (video, infrared, sound, ultrasound, microwave, radar, etc.) as the first step in the stimulation/processing cascade, such as: retinal implants (i.e. epi-retinal, sub-retinal, suprachoroidal), optic nerve implants, cortical implants, electric tongue stimulators, or tactile stimulators.

  11. Rettferdige minutter: Fordeling av spilletid i fotballkamper for aldersbestemte klasser

    Directory of Open Access Journals (Sweden)

    Trine Anker

    2010-01-01

    Full Text Available På et fotballag i overgangen mellom barne- og ungdomsfotballen er spilletid på fotballbanen under kamp både en knapp ressurs og en kilde til diskusjoner om fordeling. Spørsmålet som er utgangspunkt for denne artikkelen, er hvilke hensyn som bør tas når trener og lagleder skal fordele samlet spilletid på enkeltspillere. Er det viktigst å vinne fotballkampen, eller har det størst betydning å fordele spilletiden mest mulig likt mellom spillerne? Skal det tas hensyn til guttenes evner, motivasjon og preferanser? Er deres familiebakgrunn og personlighet av betydning? Diskusjonen baserer seg på en gjennomgang av ulike politiske rettferdighetsteorier for fordeling av knappe goder.Nøkkelord: rettferdighet, practice, fordelingsrettferdighet, diskursetikk, idrettsetikkEnglish summary: Minutes of justice: Distribution of time during football matches in a boys' teamDuring a football match, time for playing can be considered as a limited good. In this article the point of departure is the redistribution of time in a boys' football team in the transition phase between children and youth football. The questions are how and under which conditions time for playing should be distributed among the players. Is winning the game more important than trying to let every player play for an equal amount of time? Should the boys' talents, motivation, and preferences be considered while distributing time, or are family background and personality of importance? Different theories of political justice for the distribution of limited goods underpin the discussion of how to distribute the minutes of playing time during a football match.http://dx.doi.org/10.5324/eip.v4i1.1737

  12. Effects of AV-delay optimization on hemodynamic parameters in patients with VDD pacemakers.

    Science.gov (United States)

    Krychtiuk, Konstantin A; Nürnberg, Michael; Volker, Romana; Pachinger, Linda; Jarai, Rudolf; Freynhofer, Matthias K; Wojta, Johann; Huber, Kurt; Weiss, Thomas W

    2014-05-01

    Atrioventricular (AV) delay optimization improves hemodynamics and clinical parameters in patients treated with cardiac resynchronization therapy and dual-chamber-pacemakers (PM). However, data on optimizing AV delay in patients treated with VDD-PMs are scarce. We, therefore, investigated the acute and chronic effects of AV delay optimization on hemodynamics in patients treated with VDD-PMs due to AV-conduction disturbances. In this prospective, single-center interventional trial, we included 64 patients (38 men, 26 women, median age: 77 (70-82) years) with implanted VDD-PM. AV-delay optimization was performed using a formula based on the surface electrocardiogram (ECG). Hemodynamic parameters (stroke volume (SV), cardiac output (CO), heart rate (HR), and blood pressure (BP)) were measured at baseline and follow-up after 3 months using impedance cardiography. Using an ECG formula for AV-delay optimization, the AV interval was decreased from 180 (180-180) to 75 (75-100) ms. At baseline, AV-delay optimization led to a significant increase of both SV (71.3 ± 15.8 vs. 55.3 ± 12.7 ml, p AV delay vs. nominal AV interval, respectively) and CO (5.1 ± 1.4 vs. 3.9 ± 1.0 l/min, p AV-delay optimization in patients treated with VDD-PMs exhibits immediate beneficial effects on hemodynamic parameters that are sustained for 3 months.

  13. Posthumanisme /nymaterialisme og nomadisme - affektive brytninger av barnehagens observasjonspraksiser

    Directory of Open Access Journals (Sweden)

    Ann Merete Otterstad

    2015-09-01

    Full Text Available This article challenges observation as a method in early childhood, justified as a mapping tool for creating knowledge about children. Observation as data material is about writing down already known categorisation about children and their development. Categories and categorisation make a foundation for correspondence and coherence   - connections that might create generalising knowledge about children in early years. The article is about a research project conducted in a child-centre over a two-year period. We had an on-going conversation with the personnel around theories about ‘child development’. In the article we experiment with observation based on posthuman/newmaterial theories. Our specific interests are to explore the complexities around observation by asking; why observations, what do we expect through observation, and what might observation as datamaterial be/become? We are inspired by the Norwegian film “Kitchen Stories” (Salmer fra Kjøkkenet, Hamer, 2003 both as affect/provocation and desire (Koro-Ljungberg & MacLure, 2013. We search for affective bending and messiness (Lather, 2007; Law, 2004 to disturb and challenge observation as dominating paradigm in the field of early years, to break some patterns around the positioning of data material. Artikkelen utfordrer observasjon i barnehagen ut fra at metoden brukes som verktøy for å kartlegge og danne grunnlaget for utvikling av kunnskap om barn.  Observasjon som datamateriale innebærer iakttagelser og nedtegnelser av allerede gitte kategoriseringer om barn og barns utvikling.  Kategorier og kategorisering legger grunnlag for mønster som har i seg ideer om korrespondanse og koherens – sammenhenger som kan bidra til generaliserende kunnskap om barna i barnehagen. Omrisset av artikkelen dreies rundt et forskningsarbeid gjort i en barnehage over en toårs-periode, der vi sammen med de ansatte diskuterer teorier om barn og barns ’utvikling’. Vi bruker posthumane

  14. EST Table: AV401818 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401818 heS00196 10/09/28 77 %/219 aa ref|NP_001103813.1| P-element somatic inhibi...tor [Bombyx mori] dbj|BAF91871.1| Bombyx homolog of P-element somatic inhibitor [Bombyx mori] 10/08/28 35 %/...irmed#UniProt: Q9BLA0#protein_id:AAK09071.1 10/09/10 33 %/182 aa AGAP011446-PA Pr...otein|3L:24763465:24770148:-1|gene:AGAP011446 10/09/10 47 %/174 aa gnl|Amel|GB18145-PA 10/09/10 46 %/184 aa gi|18

  15. EST Table: AV400561 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400561 br--1451 10/09/28 62 %/198 aa ref|XP_001843664.1| neuroendocrine protein 7...b2 [Culex quinquefasciatus] gb|EDS33875.1| neuroendocrine protein 7b2 [Culex quinquefasciatus] 10/08/28 42 %.../196 aa FBpp0240992|DwilGK11849-PA 10/08/27 54 %/104 aa T03D8.3#CE18921#WBGene00011392#locus:sbt-1#neuroendocrine...45-PA 10/09/10 51 %/199 aa gi|91081787|ref|XP_973692.1| PREDICTED: similar to neuroendocrine protein 7b2 [Tribolium castaneum] BP115163 br-- ...

  16. EST Table: AV405781 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405781 wdV30227 10/09/28 88 %/272 aa ref|NP_001106136.1| Annexin IX isoform B [Bo...mbyx mori] dbj|BAA92810.1| annexin IX-B [Bombyx mori] 10/08/28 71 %/270 aa FBpp0280989|DpseGA19090-PA 10/08/...9 10/09/10 62 %/270 aa gnl|Amel|GB16448-PA 10/09/10 73 %/271 aa gi|91092420|ref|XP_967931.1| PREDICTED: similar to Annexin IX CG5730-PC [Tribolium castaneum] NM_001043376 wdV3 ...

  17. EST Table: AV402401 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402401 heS00969 10/09/28 85 %/225 aa dbj|BAB16697.1| annexin [Bombyx mori] dbj|BAB16698.1| anne...a#CE23956#WBGene00003589#locus:nex- 2#Annexin#status:Confirmed#UniProt:Q27512#protein_id:CAA 82571.2 10/09/1... %/220 aa gnl|Amel|GB16448-PA 10/09/10 73 %/220 aa gi|91092420|ref|XP_967931.1| PREDICTED: similar to Annexin IX CG5730-PC [Tribolium castaneum] NM_001043376 heS0 ...

  18. EST Table: AV404085 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404085 pg--0423X 10/09/28 100 %/117 aa gb|ACH69160.1| myosin heavy chain [Bombyx ...mori] 10/08/28 84 %/116 aa FBpp0291042|Mhc-PO 10/08/27 60 %/116 aa F11C3.3#CE09349#WBGene00006789#locus:unc-54#myosin heav... gnl|Amel|GB30329-PA 10/09/10 83 %/116 aa gi|189239929|ref|XP_001813306.1| PREDICTED: similar to Myosin heavy chain CG17927-PF isoform 1 [Tribolium castaneum] FJ029109 pg-- ...

  19. EST Table: AV404226 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404226 pg--0725 10/09/28 81 %/264 aa gb|ACQ72825.1| myosin heavy chain [Bombyx ma...ndarina] 10/08/28 78 %/264 aa FBpp0291043|Mhc-PP 10/08/27 51 %/264 aa F11C3.3#CE09349#WBGene00006789#locus:unc-54#myosin heav... aa gnl|Amel|GB30329-PE 10/09/10 78 %/264 aa gi|189239927|ref|XP_001814139.1| PREDICTED: similar to Myosin heavy chain CG17927-PF isoform 7 [Tribolium castaneum] FJ029109 pg-- ...

  20. EST Table: AV403042 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV403042 heS30974 10/09/28 62 %/228 aa ref|XP_321040.4| AGAP002015-PA [Anopheles ga...|DwilGK17594-PA 10/08/27 39 %/210 aa T21E12.4#CE23997#WBGene00000962#locus:dhc-1#dynein heavy chain#status:P...62 %/230 aa gi|91087317|ref|XP_975584.1| PREDICTED: similar to dynein heavy chain [Tribolium castaneum] DC550560 heS3 ...

  1. Framväxten av koncernfackligt samarbete inom metallindustrin

    OpenAIRE

    Kjellberg, Anders

    2006-01-01

    The chapter "Framväxten av koncernfackligt samarbete inom metallindustrin" (pp. 177-239) by Anders Kjellberg analyzes the rise and development of trade union cooperation within groups in Swedish metal industry. Special attention is paid to the two large metal enterprises in Finspång, situated in the northern part of the province of Östergötland: (1) the turbine company (for a long period of time called STAL), which were owned by the following groups: Asea, ABB, Alstom and today ...

  2. Gulliver's Travels av Jonathan Swift : fra politisk satire til barnebok

    OpenAIRE

    Krog, Kira Katarina

    2004-01-01

    Denne oppgaven tar for seg Jonathan Swifts bok Gulliver’s Travels’ (1727) utvikling fra politisk satire til barnebok. Med utgangspunkt i opplysningstidens tro på menneskets suverene fornuft og tradisjonen fra antikkens greske satire har oppgaven en historisk problemstilling. Den følger satirens og barnebokens utvikling gjennom tidene og ender i en lesning av en norsk barnebokversjon, utgitt nærmere 300 år etter førsteutgaven. Oppgaven har tre hoveddeler. Den første tar for seg hvordan bok...

  3. EST Table: AV401507 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401507 e96h0814 10/09/28 95 %/238 aa dbj|BAG15923.1| transposase [Bombyx mandarin...a] dbj|BAG15924.1| transposase [Bombyx mandarina] dbj|BAG15925.1| transposase [Bombyx mandarina] 10/08/28 n.h 10/08/27 n.h 10/09/10 n.h 10/09/10 n.h 10/09/10 n.h FS797048 e96h ...

  4. EST Table: AV398425 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398425 NV021796 10/09/28 92 %/176 aa ref|NP_001040254.1| beadex/dLMO protein [Bom...byx mori] gb|ABD36315.1| beadex/dLMO protein [Bombyx mori] 10/08/28 87 %/123 aa FBpp0262745|DyakGE17735-PA 1...a gnl|Amel|GB11268-PA 10/09/10 76 %/156 aa gi|91080717|ref|XP_975367.1| PREDICTED: similar to beadex/dLMO protein [Tribolium castaneum] FS794536 NV02 ...

  5. EST Table: AV398143 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398143 NV021176 10/09/28 100 %/213 aa ref|NP_001040254.1| beadex/dLMO protein [Bo...mbyx mori] gb|ABD36315.1| beadex/dLMO protein [Bombyx mori] 10/08/28 74 %/175 aa FBpp0262745|DyakGE17735-PA ... gnl|Amel|GB11268-PA 10/09/10 81 %/180 aa gi|91080717|ref|XP_975367.1| PREDICTED: similar to beadex/dLMO protein [Tribolium castaneum] FS794536 NV02 ...

  6. Fysiologiske effekter av delvis okklusjon under ettbeins utholdenhetsarbeid

    OpenAIRE

    Henriksen, Bjørnar Sandland

    2009-01-01

    Unilateral perifer aterosklerose i beinet har tidligere vist å gi en fysiologisk adaptasjon til sykdommen ved å endre den oksidative kapasiteten i muskelen (Jansson, 1988; Henriksson et al., 1980). Denne observasjonen er blitt fulgt opp med treningsstudier som har undersøkt effekten av ischemi ved sykkelarbeid hos friske (Sundberg, 1994), samt intervalltrening på tredemølle med okklusjon på begge beina (Abe et al., 2006). Det er imidlertid ikke utført noen treningsstudier med ett – beins knee...

  7. Endogenous RGS proteins modulate SA and AV nodal functions in isolated heart: implications for sick sinus syndrome and AV block.

    Science.gov (United States)

    Fu, Ying; Huang, Xinyan; Piao, Lin; Lopatin, Anatoli N; Neubig, Richard R

    2007-05-01

    G protein-coupled receptors play a pivotal role in regulating cardiac automaticity. Their function is controlled by regulator of G protein signaling (RGS) proteins acting as GTPase-activating proteins for Galpha subunits to suppress Galpha(i) and Galpha(q) signaling. Using knock-in mice in which Galpha(i2)-RGS binding and negative regulation are disrupted by a genomic Galpha(i2)G184S (GS) point mutation, we recently (Fu Y, Huang X, Zhong H, Mortensen RM, D'Alecy LG, Neubig RR. Circ Res 98: 659-666, 2006) showed that endogenous RGS proteins suppress muscarinic receptor-mediated bradycardia. To determine whether this was due to direct regulation of cardiac pacemakers or to alterations in the central nervous system or vascular responses, we examined isolated, perfused hearts. Isoproterenol-stimulated beating rates of heterozygote (+/GS) and homozygote (GS/GS) hearts were significantly more sensitive to inhibition by carbachol than were those of wild type (+/+). Even greater effects were seen in the absence of isoproterenol; the potency of muscarinic-mediated bradycardia was enhanced fivefold in GS/GS and twofold in +/GS hearts compared with +/+. A(1)-adenosine receptor-mediated bradycardia was unaffected. In addition to effects on the sinoatrial node, +/GS and GS/GS hearts show significantly increased carbachol-induced third-degree atrioventricular (AV) block. Atrial pacing studies demonstrated an increased PR interval and AV effective refractory period in GS/GS hearts compared with +/+. Thus loss of the inhibitory action of endogenous RGS proteins on Galpha(i2) potentiates muscarinic inhibition of cardiac automaticity and conduction. The severe carbachol-induced sinus bradycardia in Galpha(i2)G184S mice suggests a possible role for alterations of Galpha(i2) or RGS proteins in sick sinus syndrome and pathological AV block.

  8. InterProScan Result: AV398327 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398327 AV398327_4_ORF1 65C8DA7FC6C4D303 PANTHER PTHR10590 SODIUM/NUCLEOSIDE COTRA...NSPORTER 1.3e-109 T IPR008276 Concentrative nucleoside transporter Molecular Function: nucleoside:sodium sym

  9. InterProScan Result: AV400452 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400452 AV400452_2_ORF2 5E62868E5F66D93B PANTHER PTHR11523:SF15 SODIUM/POTASSIUM-D...EPENDENT ATPASE BETA SUBUNIT, INSECT NA ? IPR000402 unintegrated Molecular Function: sodium:potassium-exchan...ss: potassium ion transport (GO:0006813)|Biological Process: sodium ion transport (GO:0006814)|Cellular Component: membrane (GO:0016020) ...

  10. InterProScan Result: AV400452 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV400452 AV400452_2_ORF2 5E62868E5F66D93B PANTHER PTHR11523 SODIUM/POTASSIUM-DEPEND...ENT ATPASE BETA SUBUNIT 1.1e-19 T IPR000402 ATPase, P-type cation exchange, beta subunit Molecular Function: sodiu...006754)|Biological Process: potassium ion transport (GO:0006813)|Biological Process: sodiu

  11. AVS/Express (application visualization system) user's guide

    Energy Technology Data Exchange (ETDEWEB)

    Kato, Katsumi [Research Organization for Information Science Technology, Tokai, Ibaraki (Japan); Kume, Etsuo [Japan Atomic Energy Research Inst., Tokai, Ibaraki (Japan). Tokai Research Establishment

    2002-09-01

    Computer and network environment for image processing has been developed and maintained under the course of establishing a distributed processing environment by the information system operating division. We introduced a server for image processing, AVS/Express for image processing software and Stereo viewing system. This report summarizes the information to use AVS/Express efficiently in the computer environment for image processing. (author)

  12. InterProScan Result: AV399953 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399953 AV399953_3_ORF1 920D8122A6A51D76 PFAM PF00175 NAD_binding_1 2.5e-08 T IPR001433 Oxido...reductase FAD/NAD(P)-binding Molecular Function: oxidoreductase activity (GO:0016491)|Biological Process: oxidation reduction (GO:0055114) ...

  13. InterProScan Result: AV398312 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398312 AV398312_1_ORF2 384AC61A6604E3FD PANTHER PTHR10795:SF30 SITE-1 PROTEASE (STEROL...-REGULATED, CLEAVES STEROL REGULATORY ELEMENT BINDING PROTEINS) 1.8e-17 T IPR015500 unintegrated ...

  14. InterProScan Result: AV405377 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405377 AV405377_1_ORF1 5614E1377E837F70 SUPERFAMILY SSF103473 MFS general substra...te transporter 2.1e-06 T IPR016196 Major facilitator superfamily, general substrate transporter ...

  15. InterProScan Result: AV399408 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399408 AV399408_2_ORF1 5D6D9219C673BB29 PFAM PF05274 Baculo_E25 1.3e-78 T IPR007938 unintegrated Cellular... Component: viral envelope (GO:0019031)|Cellular Component: host cell nucleus (GO:0042025) ...

  16. Pilot Implementation and Preliminary Evaluation of START:AV Assessments in Secure Juvenile Correctional Facilities.

    Science.gov (United States)

    Desmarais, Sarah L; Sellers, Brian G; Viljoen, Jodi L; Cruise, Keith R; Nicholls, Tonia L; Dvoskin, Joel A

    2012-01-01

    The Short-Term Assessment of Risk and Treatability: Adolescent Version (START:AV) is a new structured professional judgment guide for assessing short-term risks in adolescents. The scheme may be distinguished from other youth risk assessment and treatment planning instruments by its inclusion of 23 dynamic factors that are each rated for both vulnerability and strength. In addition, START:AV is also unique in that it focuses on multiple adverse outcomes-namely, violence, self-harm, suicide, unauthorized leave, substance abuse, self-neglect, victimization, and general offending-over the short-term (i.e., weeks to months) rather than long-term (i.e., years). This paper describes a pilot implementation and preliminary evaluation of START:AV in three secure juvenile correctional facilities in the southern United States. Specifically, we examined the descriptive characteristics and psychometric properties of START:AV assessments completed by 21 case managers on 291 adolescent offenders (250 boys and 41 girls) at the time of admission. Results provide preliminary support for the feasibility of completing START:AV assessments as part of routine practice. Findings also highlight differences in the characteristics of START:AV assessments for boys and girls and differential associations between the eight START:AV risk domains. Though results are promising, further research is needed to establish the reliability and validity of START:AV assessments completed in the field.

  17. InterProScan Result: AV401371 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401371 AV401371_2_ORF2 4E899180012BEB2A GENE3D G3DSA:3.30.465.10 no description 7...e-07 T IPR016169 CO dehydrogenase flavoprotein-like, FAD-binding, subdomain 2 Molecular Function: catalytic

  18. Third-degree AV block sensitive to prednisolone 72 hours post AVNRT ablation.

    Science.gov (United States)

    Parwani, Abdul S; Schröder, Anna I; Blaschke, Daniela; Blaschke, Florian; Huemer, Martin; Attanasio, Philipp; Pieske, Burkert; Boldt, Leif-Hendrik; Haverkamp, Wilhelm

    2017-05-01

    A patient developed a transient first-degree AV block during a radiofrequency ablation of an atrioventricular nodal reentrant tachycardia. Three days later the patient presented with a third-degree AV block. It resolved within 24 h under antiphlogistic therapy. Patient was asymptomatic without necessity for pacemaker implantation at 12 months follow-up.

  19. InterProScan Result: AV404903 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404903 AV404903_2_ORF1 4CB4FF124EAAC1C5 PFAM PF05187 ETF_QO 1.7e-14 T IPR007859 Electron transfer flav...oprotein-ubiquinone oxidoreductase Molecular Function: electron-transferring-flavoprote

  20. Association of temporary complete AV block and junctional ectopic tachycardia after surgery for congenital heart disease.

    Science.gov (United States)

    Paech, Christian; Dähnert, Ingo; Kostelka, Martin; Mende, Meinhardt; Gebauer, Roman

    2015-01-01

    Junctional ectopic tachycardia (JET) is a postoperative complication with a mortality rate of up to 14% after surgery for congenital heart disease. This study evaluated the risk factors of JET and explored the association of postoperative temporary third degree atrioventricular (AV) block and the occurrence of JET. Data were collected retrospectively from 1158 patients who underwent surgery for congenital heart disease. The overall incidence of JET was 2.8%. Temporary third degree AV block occurred in 1.6% of cases. Permanent third degree AV block requiring pacemaker implantation occurred in 1% of cases. In all, 56% of patients with JET had temporary AV block (P AV block (P = 0.56). temporary third degree AV block did not suffer from JET. A correlation between temporary third degree AV block and postoperative JET could be observed. The risk factors identified for JET include younger age groups at the time of surgery, longer aortic cross clamping time and surgical procedures in proximity to the AV node.

  1. InterProScan Result: AV399740 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399740 AV399740_3_ORF2 7A42A41D4F3F6388 PFAM PF00067 p450 1.2e-19 T IPR001128 Cytochrome P450 Molecular... Function: monooxygenase activity (GO:0004497)|Molecular Function: iron ion binding (GO:0005506)|Molecular... Function: electron carrier activity (GO:0009055)|Molecular Function: heme binding (GO:0020037) ...

  2. InterProScan Result: AV404187 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404187 AV404187_4_ORF1 AB84B56694D3FC58 PFAM PF00267 Porin_1 3.5e-13 T IPR001702 Por...in, Gram-negative type Molecular Function: transporter activity (GO:0005215)|Biological Process: transport (GO:0006810)|Cellular Component: membrane (GO:0016020) ...

  3. InterProScan Result: AV404181 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404181 AV404181_1_ORF1 F6474B68BA8D3768 PANTHER PTHR10783:SF4 XENOTROPIC AND POLY...TROPIC MURINE LEUKEMIA VIRUS RECEPTOR XPR1 NA ? IPR004342 unintegrated Cellular Component: integral to membrane (GO:0016021) ...

  4. InterProScan Result: AV404181 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV404181 AV404181_1_ORF1 F6474B68BA8D3768 PANTHER PTHR10783:SF4 XENOTROPIC AND POLY...TROPIC MURINE LEUKEMIA VIRUS RECEPTOR XPR1 1.4e-114 T IPR004342 unintegrated Cellular Component: integral to membrane (GO:0016021) ...

  5. InterProScan Result: AV399344 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399344 AV399344_1_ORF2 5604F45CA0D42E5E PFAM PF05314 Baculo_ODV-E27 1.4e-47 T IPR007978 Baculovirus occlus...ion-derived virus envelope EC27 Cellular Component: viral envelope (GO:0019031) ...

  6. InterProScan Result: AV399741 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV399741 AV399741_2_ORF2 F87D90D9B5BA6C11 PFAM PF02778 tRNA_int_endo_N 5.4e-08 T IPR006678 tRNA intro...n endonuclease, N-terminal Molecular Function: tRNA-intron endonuclease activity (GO:0000213) ...

  7. InterProScan Result: AV405061 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV405061 AV405061_3_ORF2 400BDC036077591E PANTHER PTHR10590 SODIUM/NUCLEOSIDE COTRA...NSPORTER 1.8e-12 T IPR008276 Concentrative nucleoside transporter Molecular Function: nucleoside:sodium symp

  8. InterProScan Result: AV398727 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV398727 AV398727_2_ORF2 9953252ABA69E49B PFAM PF00762 Ferrochelatase 8.4e-28 T IPR001015 Ferroche...latase Molecular Function: ferrochelatase activity (GO:0004325)|Biological Process: heme biosynthetic process (GO:0006783) ...

  9. InterProScan Result: AV399409 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available 429 Baculovirus LEF-11 Biological Process: viral infectious cycle (GO:0019058)|Biological Process: regulation of transcription (GO:0045449) ... ...AV399409 AV399409_1_ORF2 07DC81A7C7B2FA42 PFAM PF06385 Baculo_LEF-11 3e-33 T IPR009

  10. InterProScan Result: AV403947 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV403947 AV403947_3_ORF2 9625723E9868C46D PANTHER PTHR13768:SF2 GAMMA-SOLUBLE NSF A...TTACHMENT PROTEIN (SNAP-GAMMA) 8.7e-75 T IPR011990 unintegrated Molecular Function: binding (GO:0005488) ...

  11. InterProScan Result: AV401295 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401295 AV401295_2_ORF2 87FC32DE014A3D18 PROFILE PS50141 A_DEAMIN_EDITASE 15.862 T IPR002466 Adenosine deam...inase/editase Molecular Function: RNA binding (GO:0003723)|Molecular Function: adenosine deam

  12. InterProScan Result: AV401829 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401829 AV401829_3_ORF1 B72A6A008C2EBEA4 PANTHER PTHR11359 AMP DEAMINASE NA ? IPR0...01365 unintegrated Biological Process: purine ribonucleoside monophosphate biosynthetic process (GO:0009168)|Molecular Function: deaminase activity (GO:0019239) ...

  13. InterProScan Result: AV401295 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401295 AV401295_2_ORF2 87FC32DE014A3D18 PANTHER PTHR10910:SF17 ADENOSINE DEAMINAS...ular Function: adenosine deaminase activity (GO:0004000)|Biological Process: RNA processing (GO:0006396) ...

  14. InterProScan Result: AV401829 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401829 AV401829_3_ORF1 B72A6A008C2EBEA4 PANTHER PTHR11359 AMP DEAMINASE 6e-56 T I...PR001365 unintegrated Biological Process: purine ribonucleoside monophosphate biosynthetic process (GO:0009168)|Molecular Function: deaminase activity (GO:0019239) ...

  15. InterProScan Result: AV401888 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV401888 AV401888_3_ORF1 2F04E8AA567F7BCF PANTHER PTHR11511 INSECT HEMOCYANIN-RELAT...ED 1.5e-119 T IPR013788 Arthropod hemocyanin/insect LSP Molecular Function: oxygen transporter activity (GO:0005344)|Biological Process: transport (GO:0006810) ...

  16. InterProScan Result: AV402282 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available AV402282 AV402282_2_ORF1 D1F0DCC6A8296985 PANTHER PTHR11511 INSECT HEMOCYANIN-RELAT...ED 5e-97 T IPR013788 Arthropod hemocyanin/insect LSP Molecular Function: oxygen transporter activity (GO:0005344)|Biological Process: transport (GO:0006810) ...

  17. InterProScan Result: AV399297 [KAIKOcDNA[Archive

    Lifescience Database Archive (English)

    Full Text Available ulovirus p35, apoptosis preventing protein Biological Process: anti-apoptosis (GO:0006916)|Molecular Function: caspase inhibitor activity (GO:0043027) ... ...AV399297 AV399297_3_ORF2 F5C6B0DD046B3A9D PFAM PF02331 P35 5.2e-107 T IPR003429 Bac

  18. Selvbestemmelse og oppdragelse : en teoretisk analyse av oppdragelsens påvirkning på utviklingen av selvbestemmelse

    OpenAIRE

    2007-01-01

    1) Problemstilling Hensikten med oppgaven er å rette fokus mot oppdragelse som en påvirkningsfaktor på utviklingen av selvbestemmelsen, og hvilken betydning behovstilfredsstillelse har for individets subjektive velvære. Oppdragelse er et populært tema som stadig blir debattert i mediene. Det diskuteres blant annet hvorfor oppdragelse er viktig, hvordan det er mulig å oppdra barn på best mulig måte og hvem som er viktige oppdragere i dagens samfunn. Vår oppgave handler om individets selvbes...

  19. 中国新一代图像编码格式"AVS"%Chinese new Video coding format AVS

    Institute of Scientific and Technical Information of China (English)

    2004-01-01

    @@ 中国在光盘、无线LAN、家庭内部网络等各种领域不断推出自己的规格.其中,包括中国计划2004年实现标准化的新一代图像编码格式"AVS".中国科学院有关负责人将在NAB2004技术论坛"Leading Edge Tech for TV"上,就定位相当于"MPEG"的AVS规格发表演讲.

  20. Endrer motivasjonen seg i løpet av de fire første ukene av livsstilsendringskurset EasyLife?

    OpenAIRE

    Strand, Hanne Embretsen; Nordengen, Solveig

    2011-01-01

    Vi har undersøkt motivasjonsregulering hos deltagere i et livsstilsendringsprogram. Teoretisk har vi benyttet oss av Helseoppfatningsmodellen (The Health Belief Model; Rosenstock, 1966), Den transteoretiske modellen (The Transtheoretical Model; DiClemente & Prochaska, 1982; Marcus & Simkin, 1994), Selvbestemmelsesteorien (Self-Determination Theory; Deci & Ryan, 2000) og Rosenstocks fire modeller for ansvar. For å kartlegge motivasjonsregulering har vi benyttet oss av BREQ-2 (Markland & Tobin ...